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Tuna K, Ilkun O, Dziegielewski PT, Sharma A. Uremic Leontiasis Ossea due to Resistant Secondary Hyperparathyroidism. AACE Clin Case Rep 2025; 11:5-9. [PMID: 39896948 PMCID: PMC11784621 DOI: 10.1016/j.aace.2024.09.001] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/06/2024] [Revised: 08/28/2024] [Accepted: 09/03/2024] [Indexed: 02/04/2025] Open
Abstract
Background/Objective Severe progressive overgrowth of the facial bones known as uremic leontiasis ossea (ULO) is a rare complication of resistant hyperparathyroidism in end-stage renal disease (ESRD). The objective of this report is to describe the presentation and treatment of ULO. Case Report A 48-year-old woman with a history of hypertension, coronary artery disease, and ESRD on hemodialysis presented with severe secondary hyperparathyroidism and calciphylaxis. She had significant changes to her face in the last 3 months leading to oropharyngeal dysphagia and difficulty articulating. Physical examination revealed bony overgrowth in her upper jaw and hard palate, widely spaced teeth, and calcinosis cutis. Her parathyroid hormone (PTH), calcium, and phosphorus levels were 5066 pg/mL (normal range, 12-88 pg/mL); 10.0 mg/dL (8.4-10.2 mg/dL); and 5.4 mg/dL (2.7-4.5 mg/dL); respectively. Using a multidisciplinary approach, she successfully underwent a 3.5-gland parathyroidectomy (immediate postoperative PTH level, 600 pg/mL). She was discharged without complication. Pathology showed hypercellular parathyroid glands with reactive changes. Discussion ULO, the most severe form of renal osteodystrophy, results in hypertrophy of the craniofacial skeleton. It carries the risk of significant comorbidities due to cranial nerve compression, respiratory compromise, dysarthria, and dysphagia. Conclusion With prolonged, uncontrolled PTH stimulation in ESRD, significant facial disfiguration with disabilities can occur. It is of utmost importance to adhere to guideline-specified PTH targets in persons with ESRD to prevent patient harm from permanent physical deformities.
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Affiliation(s)
- Kubra Tuna
- Division of Endocrinology, Diabetes and Metabolism, University of Florida College of Medicine, Gainesville, Florida
| | - Olesya Ilkun
- Division of Nephrology, University of Florida College of Medicine, Gainesville, Florida
| | - Peter T. Dziegielewski
- Department of Otolaryngology, University of Florida College of Medicine, Gainesville, Florida
| | - Anu Sharma
- Division of Endocrinology, Diabetes and Metabolism, University of Florida College of Medicine, Gainesville, Florida
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Liao Z, Ye Z, Zhang X, Zhang J, He P, Hong H, Mi J. Impact of Surgical Parathyroidectomy on Craniofacial Morphology in Patients With Renal Failure. Am Surg 2025; 91:86-93. [PMID: 39116316 DOI: 10.1177/00031348241272425] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 08/10/2024]
Abstract
PURPOSE Parathyroidectomy is beneficial in tertiary hyperparathyroidism (THPT) consequent to chronic renal failure. The craniofacial morphology of patients who undergo total parathyroidectomy and autologous transplantation (tPTX + AT) has not been widely studied. This study assessed the efficacy of tPTX + AT in THPT and evaluated possible improvements in craniofacial features. METHODS This retrospective analysis included patients who were diagnosed with medically refractory THPT and had undergone tPTX + AT between September 2013 and May 2021. The VAS was used to evaluate improvements in various symptoms including bone pain and pruritus. Changes in serum calcium, phosphorus, alkaline phosphatase, and intact parathyroid hormone (iPTH) levels were also assessed. The impact of the procedure was assessed by comparing two-photon X-ray bone mineral density measurements obtained 1 year before and after surgery. RESULTS The VAS of pain and pruritus decreased significantly on the first postoperative day (P < 0.05). Calcium levels changed significantly (from 2.50 ± 0.22 mmol/L to 2.10 ± 0.26 mmol/L) on postoperative day 1 (P = 0.0000); iPTH levels also declined substantially on this day, reducing from 211.00 (122.10, 252.80) to 5.04 (2.96, 9.40) pmol/L. Bone mineral density increased significantly across various regions including the greater trochanter of the femur, intertrochanteric area, total hip, and third lumbar vertebra (P < 0.05). The angles between the upper incisor and mandibular plane and the lower lip and Ricketts E line (drawn from the tip of the nose to the soft tissue area) also improved (P = 0.043, P = 0.001). CONCLUSION Total parathyroidectomy and autologous transplantation can rapidly alleviate bone pain and skin itching in THPT. It may also improve bone density and facial soft tissue.
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Affiliation(s)
- Zhenpeng Liao
- Department of Otolaryngology Head and Neck Surgery, The Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, People's Republic of China
| | - Zhongkang Ye
- Department of Otolaryngology Head and Neck Surgery, The Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, People's Republic of China
| | - Xu Zhang
- Department of Otolaryngology Head and Neck Surgery, The Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, People's Republic of China
| | - Jun Zhang
- Department of Otolaryngology Head and Neck Surgery, The Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, People's Republic of China
| | - Peng He
- Department of Otolaryngology Head and Neck Surgery, The Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, People's Republic of China
| | - Haiyu Hong
- Department of Otolaryngology Head and Neck Surgery, The Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, People's Republic of China
| | - Jiaoping Mi
- Department of Otolaryngology Head and Neck Surgery, The Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, People's Republic of China
- Department of Otolaryngology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou Key Laboratory of Otorhinolaryngology, Otorhinolaryngology Institute of Sun Yat-sen University, Guangzhou, China
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García Romero JM, Guerrero Morales PH, Rico Razo MF, Córdova Argueta JM, Olaya Niebla E. Sagliker Syndrome: A Case Report of Facial Deformities and Renal Osteodystrophy Secondary to Hyperparathyroidism in End-Stage Renal Disease. Cureus 2024; 16:e64399. [PMID: 39130819 PMCID: PMC11317026 DOI: 10.7759/cureus.64399] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/12/2024] [Indexed: 08/13/2024] Open
Abstract
Sagliker syndrome (SS) is a rare but distinctive form of renal osteodystrophy associated with poorly managed secondary hyperparathyroidism (SHPT) in patients with chronic kidney disease (CKD). We present a case of a 28-year-old male with end-stage CKD on hemodialysis for 10 years, who exhibited progressive facial deformities and maxillofacial bone pain. Physical examination revealed bilateral expansion of the maxillary and mandibular bones and facial asymmetry. Radiological findings included diffuse bone thickening and multilocular cysts in the maxillofacial bones, while laboratory tests showed decreased levels of calcium and elevated parathyroid hormone, confirming SHPT. Despite multidisciplinary management involving nephrology, endocrinology, and maxillofacial surgery, the patient's condition deteriorated and he manifested community-acquired pneumonia leading to cardiopulmonary arrest and death. This case underscores the challenges in managing severe HPT in CKD and emphasizes the importance of early assessment and comprehensive multidisciplinary care to prevent irreversible complications.
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Affiliation(s)
- José Manuel García Romero
- Transplant and Donation Department, Regional General Hospital 1 of the Mexican Social Security Institute, Querétaro, MEX
| | - Pedro Hugo Guerrero Morales
- Transplant and Donation Department, Regional General Hospital 1 of the Mexican Social Security Institute, Querétaro, MEX
| | - Maria Fernanda Rico Razo
- Transplant and Donation Department, Regional General Hospital 1 of the Mexican Social Security Institute, Querétaro, MEX
| | - José Macario Córdova Argueta
- Transplant and Donation Department, Regional General Hospital 1 of the Mexican Social Security Institute, Querétaro, MEX
| | - Erick Olaya Niebla
- Transplant and Donation Department, Regional General Hospital 1 of the Mexican Social Security Institute, Querétaro, MEX
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Erol Ç, Şahin Ö, Şen AE, Aydın Z. Bone and Parathyroid Scintigraphy Findings in Sagliker Syndrome. Mol Imaging Radionucl Ther 2024; 33:40-42. [PMID: 38390778 PMCID: PMC10899744 DOI: 10.4274/mirt.galenos.2023.24382] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/24/2024] Open
Abstract
Sagliker syndrome (SS) is a rare, exaggerated form of chronic kidney disease (CKD)-mineral and bone disorder resulting from untreated secondary hyperparathyroidism due to CKD. Herein, we describe a 34-year-old male patient whose Tc-99m-methylene diphosphonate bone scintigraphy and Tc-99m-sestamibi parathyroid scintigraphy revealed hints of SS and exhibited its defining characteristics.
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Affiliation(s)
- Çağlagül Erol
- Necmettin Erbakan University Meram Faculty of Medicine, Department of Nuclear Medicine, Konya, Türkiye
| | - Özlem Şahin
- Necmettin Erbakan University Meram Faculty of Medicine, Department of Nuclear Medicine, Konya, Türkiye
| | - Ahmet Eren Şen
- Kastamonu Training and Research Hospital, Clinic of Nuclear Medicine, Kastamonu, Türkiye
| | - Zeynep Aydın
- Konya City Hospital, Clinic of Nuclear Medicine, Konya, Türkiye
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Salimkhanov R, Bondarenko E, Eremkina A, Bibik E, Kim E, Begova K, Kim I, Kuznetsov S, Mokrysheva N. Case report: Sagliker syndrome in the patient with recurrent tertiary hyperparathyroidism due to intrathyroidal parathyroid carcinoma. Front Endocrinol (Lausanne) 2024; 14:1292993. [PMID: 38250739 PMCID: PMC10796468 DOI: 10.3389/fendo.2023.1292993] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/13/2023] [Accepted: 12/13/2023] [Indexed: 01/23/2024] Open
Abstract
Sagliker syndrome (SS) is an extremely rare disorder that manifests in patients with advanced chronic kidney disease (CKD) undergoing programmed hemodialysis as a renal replacement therapy. Treatment of secondary hyperparathyroidism (SHPT) in these patients is still challenging. The main clinical manifestations of SS include craniofacial and fingertip deformities, dental anomalies, gingival hyperplasia, short stature, hearing loss, neurological and psychiatric impairment. The etiology and pathogenesis of SS in patients with SHPT require further clarification. However, mutations in the GNAS1, FGF23, and FGFR3 genes were described in some patients, suggesting a possible role of genetic predisposition to the syndrome. The preferred therapeutic approach for SS is surgery, but the volume of the operation is debated. The main surgical strategies include total, subtotal parathyroidectomy, or total parathyroidectomy with autotransplantation of the parathyroid gland (PG). Unfortunately, parathyroidectomy does not contribute to the regression of significant skeletal deformities. We present a unique clinical case of a patient with classical features of SS, recurrent tertiary hyperparathyroidism (THPT) after total parathyroidectomy due to intrathyroidal parathyroid carcinoma (PC).
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Affiliation(s)
- Rustam Salimkhanov
- Department of Parathyroid Pathology and Mineral Disorders, Endocrinology Research Center, Moscow, Russia
| | | | - Anna Eremkina
- Department of Parathyroid Pathology and Mineral Disorders, Endocrinology Research Center, Moscow, Russia
| | - Ekaterina Bibik
- Department of Parathyroid Pathology and Mineral Disorders, Endocrinology Research Center, Moscow, Russia
| | - Ekaterina Kim
- Department of Parathyroid Pathology and Mineral Disorders, Endocrinology Research Center, Moscow, Russia
| | - Kamila Begova
- Department of Parathyroid Pathology and Mineral Disorders, Endocrinology Research Center, Moscow, Russia
| | - Ilya Kim
- Department of Endocrine Surgery, Endocrinology Research Center, Moscow, Russia
| | - Sergey Kuznetsov
- Department of Endocrine Surgery, Endocrinology Research Center, Moscow, Russia
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Ahmad I, Alkomi S, Sharaha R, Manasrah S, Dukmak ON. Sagliker Syndrome in a Patient With Secondary Hyperparathyroidism and Chronic Kidney Disease: A Case Report From Palestine. Cureus 2024; 16:e51956. [PMID: 38333487 PMCID: PMC10852203 DOI: 10.7759/cureus.51956] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/09/2024] [Indexed: 02/10/2024] Open
Abstract
Sagliker syndrome (SS) is a rare complication in patients with chronic kidney disease (CKD) on prolonged dialysis due to uncontrolled secondary hyperparathyroidism (SHPT). SS manifests with a constellation of clinical manifestations, including short stature, craniomaxillofacial abnormalities, hearing loss, and neuropsychiatric disorders. This article reports a 33-year-old male patient with CKD who complained of progressive disfiguring facial changes, multiple recurrent fractures, and shortened height. The condition affects his quality of life. On workup, his lab results showed highly elevated serum levels of parathyroid hormone, alkaline phosphatase (ALP), calcium, and phosphate. His comorbidities and poor health status limit his ability to do parathyroidectomy (Ptx). A reliable diagnostic approach must be considered, enabling physicians to make earlier interventions and get better outcomes.
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Affiliation(s)
- Ibtihal Ahmad
- Nephrology, Internal Medicine, School of Medicine, Palestine Polytechnic University, Hebron, PSE
| | - Saja Alkomi
- Nephrology, Internal Medicine, School of Medicine, Palestine Polytechnic University, Hebron, PSE
| | - Rula Sharaha
- Nephrology, Internal Medicine, School of Medicine, Palestine Polytechnic University, Hebron, PSE
| | - Shaheera Manasrah
- Nephrology, Internal Medicine, School of Medicine, Palestine Polytechnic University, Hebron, PSE
| | - Osama N Dukmak
- Nephrology, Internal Medicine, School of Medicine, Al-Quds University, Jerusalem, PSE
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Hu TL, Chen J, Shao SQ, Li LL, Lai C, Gao WN, Xu RF, Meng Y. Biomechanical and histomorphological analysis of the mandible in rats with chronic kidney disease. Sci Rep 2023; 13:21886. [PMID: 38081976 PMCID: PMC10713524 DOI: 10.1038/s41598-023-49152-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/17/2023] [Accepted: 12/05/2023] [Indexed: 12/18/2023] Open
Abstract
The present study aimed to investigate the biomechanical and histomorphological features of mandibles in an adenine-induced chronic kidney disease-mineral and bone disorder (CKD-MBD) rat model of CKD. A total of 14 Sprague-Dawley rats were randomized into the following two groups: control group and CKD group. At the end of the sixth week, all rats were euthanized, and serum was collected for biochemical marker tests. Macroscopic bone growth and biomechanical parameters were measured in the right hemimandible, while the left hemimandible was used for bone histomorphometric analysis. Compared to the control group, the CKD group showed a significant increase in serum creatinine, blood urea nitrogen, and serum parathyroid hormone at the end of the sixth week. The biomechanical structural properties significantly decreased in the CKD group compared to the control group. Bone histomorphometric analysis indicated that the trabecular bone volume of rats in the CKD group was significantly lower than that of the control group. In the CKD groups, the bone formation parameters of the trabecular bone were significantly increased, while the bone mineralization apposition rates of both the trabecular bone and periosteal cortical bone were significantly increased. The rat CKD model showed deteriorated structural mechanics, low trabecular bone volume, high trabecular bone formation, increased trabecular bone mineralization apposition rate, and increased cortical bone mineralization apposition rate, which met the characteristics of osteitis fibrosa, indicating that this model is a useful tool for the study of mandible diseases in CKD patients.
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Affiliation(s)
- Ta-la Hu
- Department of Nephrology, The Affiliated Hospital of Inner Mongolia Medical University, Hohhot, 010050, Inner Mongolia, China
| | - Jun Chen
- School of Life Sciences and Biopharmaceutics, Guangdong Pharmaceutical University, Guangzhou, 510006, Guangdong, China
| | - Shen-Quan Shao
- School of Life Sciences and Biopharmaceutics, Guangdong Pharmaceutical University, Guangzhou, 510006, Guangdong, China
| | - Le-le Li
- Department of Nephrology, The Affiliated Hospital of Inner Mongolia Medical University, Hohhot, 010050, Inner Mongolia, China
| | - Can Lai
- Graduate School, Inner Mongolia Medical University, Hohhot, 010110, Inner Mongolia, China
| | - Wu-Niri Gao
- Department of Nephrology, The Affiliated Hospital of Inner Mongolia Medical University, Hohhot, 010050, Inner Mongolia, China
| | - Rui-Feng Xu
- Department of Nephrology, The Affiliated Hospital of Inner Mongolia Medical University, Hohhot, 010050, Inner Mongolia, China
| | - Yan Meng
- Department of Nephrology, The Affiliated Hospital of Inner Mongolia Medical University, Hohhot, 010050, Inner Mongolia, China.
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Xu Y, Hong Y, Liu X, Zhou L, Jiang C. Anaesthetic considerations in Shrinking Man syndrome: two case reports. BMC Anesthesiol 2023; 23:20. [PMID: 36631742 PMCID: PMC9835252 DOI: 10.1186/s12871-023-01978-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/12/2022] [Accepted: 01/05/2023] [Indexed: 01/13/2023] Open
Abstract
BACKGROUND Shrinking Man syndrome (SMS) is a rare but often serious complication of dialysis-dependent end-stage renal disease, characterized by significant loss of height, bone pain, bone deformity, and skin itching. Patients with SMS always have abnormal facial changes and cardiovascular system damage (manifested by hypertension, hypotension, cardiovascular calcification, and valvular heart disease), which pose a great challenge to anaesthesiologists. The purpose of this report is to describe our anaesthetic experience regarding two patients with SMS combined with alterations of the airway and cardiovascular system. CASE PRESENTATION We describe two cases of SMS treated at West China Hospital, a tertiary care centre in Chengdu, China. All cases met the diagnostic criteria, which comprised 1) dialysis-dependent end-stage renal disease, 2) loss of height, and 3) bone pain and bone deformity. One patient had an anticipated difficult airway and moderate-to-severe mitral stenosis. The other patient presented with significant hypotension. Anaesthetic considerations included awake fibreoptic bronchoscopy-assisted tracheal intubation, real-time transoesophageal echocardiogram monitoring and individualized blood pressure management strategies. CONCLUSION This case series highlights the importance of adequate preoperative assessment and preparation, as well as individualized anaesthetic management, in patients with SMS.
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Affiliation(s)
- Yan Xu
- grid.412901.f0000 0004 1770 1022Department of Anesthesiology, West China Hospital, Sichuan University & The Research Units of West China (2018RU012), Chinese Academy of Medical Sciences, Chengdu, 610041 China
| | - Ying Hong
- grid.412901.f0000 0004 1770 1022Department of Anesthesiology, West China Hospital, Sichuan University & The Research Units of West China (2018RU012), Chinese Academy of Medical Sciences, Chengdu, 610041 China
| | - Xin Liu
- grid.412901.f0000 0004 1770 1022Department of Anesthesiology, West China Hospital, Sichuan University & The Research Units of West China (2018RU012), Chinese Academy of Medical Sciences, Chengdu, 610041 China
| | - Li Zhou
- grid.412901.f0000 0004 1770 1022Department of Anesthesiology, West China Hospital, Sichuan University & The Research Units of West China (2018RU012), Chinese Academy of Medical Sciences, Chengdu, 610041 China
| | - Chunling Jiang
- grid.412901.f0000 0004 1770 1022Department of Anesthesiology, West China Hospital, Sichuan University & The Research Units of West China (2018RU012), Chinese Academy of Medical Sciences, Chengdu, 610041 China ,grid.13291.380000 0001 0807 1581Department of Anesthesiology and Translational Neuroscience Center, West China Hospital, Sichuan University, 610041 Chengdu, China
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Sabanis N, Paschou E, Drylli A, Papanikolaou P, Zagkotsis G. Uremic Leontiasis Ossea: Theoretical Concepts and Practical Considerations. SAUDI JOURNAL OF KIDNEY DISEASES AND TRANSPLANTATION 2022; 33:702-715. [PMID: 37955462 DOI: 10.4103/1319-2442.389430] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/14/2023] Open
Abstract
Leontiasis ossea (LO) in chronic kidney disease patients, also known as Sagliker syndrome, is an exceptionally uncommon uremic complication of long-lasting and severe secondary hyperparathyroidism. The prominent features of uremic LO (ULO) encompass the characteristic clinical trial of massive thickening of maxillary and mandibular bones, widening of interdental spaces, and flattening of nasal bridges and nares. Moreover, during the transformation of craniofacial architecture, significant structural and functional consequences may appear, including upper airway patency, visual and hearing acuity, oral phase of swallowing as well as various neurological and psychiatric disorders. Only few cases of ULO have been reported in the literature until now, making challenging not only the traditional diagnostic procedures but also the optimal therapeutic approach. In this narrative review, we aim to explore the underlying pathophysiological mechanisms, summarize the evidence for adverse outcomes, and highlight the current therapeutic strategies for ULO prevention and treatment, given that precise genetic determinants remain elusive.
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Affiliation(s)
- Nikolaos Sabanis
- Department of Nephrology, General Hospital of Livadia, Livadia, Greece
| | - Eleni Paschou
- Department of General Practice and Family Medicine, Medical Unit of St George, Livadia, Greece
| | - Aikaterini Drylli
- Department of Otorhinolaryngology, National and Kapodistrian University of Athens, Athens, Greece
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Guimarães LM, Valeriano AT, Rebelo Pontes HA, Gomez RS, Gomes CC. Manifestations of hyperparathyroidism in the jaws: Concepts, mechanisms, and clinical aspects. Oral Surg Oral Med Oral Pathol Oral Radiol 2022; 133:547-555. [PMID: 35181256 DOI: 10.1016/j.oooo.2021.08.020] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/24/2021] [Revised: 07/07/2021] [Accepted: 08/22/2021] [Indexed: 11/18/2022]
Abstract
Hyperparathyroidism is one of the most common endocrine disorders worldwide. In countries where routine biochemical screening is not common, symptomatic hyperparathyroidism predominates. Its manifestations include skeletal alterations, calcification of soft tissues, kidney stones, and functional alterations in other systems. Notably, jaw alterations can be the first clinical sign of hyperparathyroidism, including brown tumor, renal osteodystrophy, osteitis fibrosa, and leontiasis ossea, and knowing such conditions is of core importance for the multidisciplinary diagnosis and management of hyperparathyroidism. We aimed to perform a concise review, systematizing the concepts and mechanisms underlying hyperparathyroidism and associated gnathic alterations. In addition, a detailed description of the clinical aspects of the jaw manifestations is presented.
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Affiliation(s)
- Letícia Martins Guimarães
- Department of Pathology, Biological Sciences Institute, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil
| | - Alline Teixeira Valeriano
- Department of Pathology, Biological Sciences Institute, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil
| | - Hélder Antônio Rebelo Pontes
- Service of Oral Pathology, Hospital Universitário João de Barros Barreto, Universidade Federal do Pará, Belém, Brazil
| | - Ricardo Santiago Gomez
- Department of Oral Surgery and Pathology, Faculty of Dentistry, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil
| | - Carolina Cavalieri Gomes
- Department of Pathology, Biological Sciences Institute, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.
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Uremic leontiasis ossea: distinctive imaging features allow differentiation from other clinical causes of leontiasis ossea. Radiol Case Rep 2022; 17:553-557. [PMID: 34987683 PMCID: PMC8693403 DOI: 10.1016/j.radcr.2021.11.061] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/25/2021] [Revised: 11/21/2021] [Accepted: 11/21/2021] [Indexed: 11/23/2022] Open
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Mi JP, He P, Shi K, Feng SY, Chen XZ, He QQ, Zhao MY, Ge PJ, Fan YP. Cephalometric craniofacial features of patients with Sagliker syndrome: a primary analysis of our experience. ANNALS OF TRANSLATIONAL MEDICINE 2021; 9:963. [PMID: 34277763 PMCID: PMC8267326 DOI: 10.21037/atm-21-1544] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/23/2021] [Accepted: 05/27/2021] [Indexed: 11/06/2022]
Abstract
Background Sagliker syndrome (SS) is characterized by a severe uglifying facial appearance resulting from untreated or inadequately treated secondary hyperparathyroidism (SHPT). To date, the craniofacial morphology of patients with SS has yet to be analyzed. The present research sought to cephalometrically evaluate the craniofacial features of patients with SS and to perform an in-depth analysis of their serum biochemical parameters, with the aim of furthering the theoretical basis for the early diagnosis and prevention of SS. Methods A retrospective chart review of 9 patients who fulfilled the diagnostic criteria for SS were included in this study, and their serum biochemical parameters were collected. After subjecting standard lateral cephalometric radiographic images to correction for distortions caused by magnification followed by digitization, we conducted a cephalometric analysis. Student's two-tailed t tests or Mann-Whitney U tests were used to analyze the data. Thirty-three patients with patients with SHPT alone were also included as controls. Results The lower anterior facial height (ANS-ME) and total anterior facial height (N-Me) measurements of patients with SS were significantly increased compared to those of the controls. The angles between the Frankfort horizontal, palatal, and occlusal planes and the mandibular plane, were greater in the SS group than in the control group, as was the gonial angle. Patients with SS also exhibited a significantly larger maxillary protrusion angle and relative position of the maxilla to the mandible. Most patients with SS had class II malocclusion, whereas most of the controls exhibited normal occlusion. Soft tissue largely followed the same pattern as craniofacial changes. Our investigation also showed that among patients with SHPT, female sex, longer duration of dialysis, and higher serum levels of alkaline phosphatase and intact parathyroid hormone were associated with development to SS. Conclusions Patients with SS show facial and biochemical differences compared to patients with SHPT. Female sex, long dialysis duration, and high serum levels of intact parathyroid hormone and alkaline phosphatase may be potential risk factors for SS.
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Affiliation(s)
- Jiao-Ping Mi
- Department of Otolaryngology Head and Neck Surgery, The Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, China
| | - Peng He
- Department of Otolaryngology Head and Neck Surgery, The Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, China
| | - Kai Shi
- Department of Stomatology, The Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, China
| | - Shao-Yan Feng
- Department of Otolaryngology Head and Neck Surgery, The Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, China
| | - Xian-Zhen Chen
- Department of Otolaryngology Head and Neck Surgery, The Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, China
| | - Qing-Qing He
- Department of Otolaryngology Head and Neck Surgery, Zhuhai Maternal and Child Health Hospital, Zhuhai, China
| | - Ming-Yue Zhao
- The Graduate School of Zunyi Medical University, Zunyi, China
| | - Ping-Jiang Ge
- Department of Otolaryngology Head and Neck Surgery, Guangdong Provincial General Hospital, Guangzhou, China
| | - Yun-Ping Fan
- Department of Otolaryngology Head and Neck Surgery, The Fifth Affiliated Hospital of Sun Yat-sen University, Zhuhai, China.,Department of Otolaryngology Head and Neck Surgery, The Seventh Affiliated Hospital of Sun Yat-sen University, Shenzhen, China
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Aravindhan R, Magesh KT, Vivek N, Saravanan C. Maxillary brown tumor due to secondary hyperparathyroidism in a Hemodialysis patient: A case report and literature review. J Oral Maxillofac Pathol 2021; 25:527-532. [PMID: 35281174 PMCID: PMC8859602 DOI: 10.4103/jomfp.jomfp_157_21] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2021] [Revised: 07/07/2021] [Accepted: 09/05/2021] [Indexed: 11/08/2022] Open
Abstract
Hemodialysis is one of the commonly used renal replacement therapies in treating end-stage renal failure patients. Patients with long-term dialysis may develop frequently complications such as secondary hyperparathyroidism (SHPT), bone diseases, amyloidosis, endocrinal disturbances, cardiovascular complications and infections. Brown tumors (BTs) are erosive giant cell bony lesions that arise in some patients as a result of primary or SHPT. About 2% of all the reported cases showed involvement of facial skeleton, of which the mandible is the favorite site. A complete clinical, biochemical, radiological and histopathological correlation is required for definitive diagnosis. We report here a case of BT in 37-year-old female hemodialysis patient with SHPT.
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Abstract
In this report, we present a case of a patient with a history of complex airway anatomy secondary to Sagliker syndrome (SS) who presented with acute exacerbation of chronic respiratory failure. The patient's difficult airway, complicated medical comorbidities, and poor psychosocial status posed a unique challenge for providing safe care during an emergency. The perioperative anesthesia service (PAS), led by critical care anesthesiologists, coordinated a multidisciplinary airway management plan. The PAS team also assisted this medically complex patient with her decision-making process. A 37-year-old female with SS, which is characterized by irreversible disfiguring of head and neck anatomy secondary to end-stage renal disease (ESRD) and poorly controlled hyperparathyroidism, presented with acute exacerbation of chronic respiratory failure due to hypervolemia. The patient's respiratory status rapidly deteriorated despite aggressive hemodialysis, requiring transfer to the ICU. Given the challenging anatomy and poor respiratory reserve in this patient, the PAS team helped coordinate a comprehensive airway plan that involved transnasal fiberoptic intubation, and in case of emergency, extracorporeal membrane oxygenation (ECMO) as a bridge to a surgical airway. During the decision-making process, the patient was found to be in psychological distress and had limited insights into her condition. The PAS team helped facilitated multidisciplinary goals-of-care discussions for the patient and her family. Fortunately, the patient's oxygenation improved with noninvasive oxygen support and aggressive hemodialysis without the need for intubation. She was discharged with outpatient follow-up appointments arranged to discuss long-term management. This is the first reported case of SS in the United States. The early involvement by the PAS team helped coordinate a multidisciplinary care plan for this patient with a difficult airway and complex comorbidities. This report highlights an innovative airway algorithm for a potentially "cannot-intubate, cannot ventilate" complex airway, and the PAS team's role in providing support for the patient's physical and psychological needs, suggesting that a comprehensive perioperative service can improve the quality and safety of care, not only for surgical patients but also for medically complex patients as well.
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Affiliation(s)
- QiLiang Chen
- Department of Anesthesiology, Stanford University, Stanford, USA
| | - Javier Lorenzo
- Department of Anesthesiology, Stanford University, Stanford, USA
| | - Amy Lu
- Department of Anesthesiology, Stanford University, Stanford, USA
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15
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Yu Y, Zhu CF, Fu X, Xu H. Sagliker syndrome: A case report of a rare manifestation of uncontrolled secondary hyperparathyroidism in chronic renal failure. World J Clin Cases 2019; 7:3792-3799. [PMID: 31799306 PMCID: PMC6887613 DOI: 10.12998/wjcc.v7.i22.3792] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/12/2019] [Revised: 10/03/2019] [Accepted: 10/15/2019] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Sagliker syndrome (SS) resulting from uncontrolled secondary hyperparathyroidism (SHPT) in chronic renal failure (CRF) is seldom reported.
CASE SUMMARY A 24-year-old woman presented with asymmetric facial deformity and stature shortening. She was diagnosed with SS, SHPT, CRF, and thyroid cancer. The patient underwent a total parathyroidectomy and thyroidectomy with central lymph node dissection. The patient’s condition was stable and was discharged from the hospital.
CONCLUSION Undergoing dialysis vintage, presenting high serum phosphate levels, and female gender may be risk factors for SS. Intramembranous ossification in the craniomaxillofacial region is possibly activated in this special pathophysiological condition. What’s more, the choice of surgery mainly depends on the treatment goal and the experience of the individual surgeon.
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Affiliation(s)
- Yu Yu
- Department of General Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Discipline Construction Research Center of China Hospital Development Institute, Shanghai Jiao Tong University, Shanghai 200011, China
| | - Chen-Fang Zhu
- Department of General Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Discipline Construction Research Center of China Hospital Development Institute, Shanghai Jiao Tong University, Shanghai 200011, China
| | - Xiao Fu
- Department of General Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Discipline Construction Research Center of China Hospital Development Institute, Shanghai Jiao Tong University, Shanghai 200011, China
| | - Hua Xu
- Department of General Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Discipline Construction Research Center of China Hospital Development Institute, Shanghai Jiao Tong University, Shanghai 200011, China
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Uremic leontiasis ossea due to secondary hyperparathyroidism complicated by vitamin C deficiency in a non-adherent chronic hemodialysis patient: A case report. Clin Nephrol Case Stud 2019; 7:54-59. [PMID: 31508269 PMCID: PMC6734544 DOI: 10.5414/cncs109788] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/20/2019] [Accepted: 07/26/2019] [Indexed: 12/05/2022] Open
Abstract
Non-adherence to medical therapy in patients with end-stage kidney disease (ESKD) can lead to severe metabolic derangements rarely seen in the current medical era. Such complications may take the form of secondary hyperparathyroidism (HPT) leading to rare manifestations of bone mineral disease, and profound vitamin C deficiency from poor nutrition combined with removal of water-soluble vitamins during dialysis. Secondary HPT causes renal osteodystrophy which can lead to diffuse enlargement of the facial skeleton and morphological changes suggestive of leontiasis ossea. We report a 36-year-old, non-adherent woman on chronic dialysis for over 10 years who developed progressive, diffuse facial bone enlargement in the context of years of extreme HPT and newly diagnosed severe vitamin C deficiency. Imaging revealed diffuse hypertrophy of the maxillary and mandibular bones. Histopathology showed extensive fibro-osseous proliferation without evidence of Brown tumor, suggestive of uremic leontiasis ossea. In this report, we discuss the orofacial manifestations of secondary HPT and the possible potentiating role of vitamin C deficiency on the development of renal osteodystrophy through altered vitamin D metabolism. Non-adherent patients on chronic dialysis should be evaluated for vitamin C deficiency, and the development of uremic leontiasis ossea should be considered when such patients present with distortion of facial features in the context of severe secondary HPT.
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Panezai MA, Ahmed S, Colbert GB. Sagliker syndrome in a patient with end-stage renal disease with secondary hyperparathyroidism. Proc (Bayl Univ Med Cent) 2019; 32:624-626. [PMID: 31656444 DOI: 10.1080/08998280.2019.1624092] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/13/2019] [Revised: 05/17/2019] [Accepted: 05/22/2019] [Indexed: 10/26/2022] Open
Abstract
Sagliker syndrome (SS), also known as an "uglifying human face syndrome," is one of the severe manifestations of chronic kidney disease in patients with uncontrolled secondary hyperparathyroidism. Patients with SS develop short stature, maxillary and mandibular bone overgrowth, nasal bone and cartilage destruction, widely spaced teeth with anterior positioning, soft tissue tumors in the oral cavity, auditory loss, and neurological and psychological features. SS can possibly be prevented with proper treatment of secondary hyperparathyroidism using disease-modifying medication and surgical therapies. We report a case of SS in a patient in the USA with end-stage renal disease achieving adequate hemodialysis.
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Affiliation(s)
| | - Sana Ahmed
- Division of Nephrology, Department of Internal Medicine, Baylor University Medical Center at DallasDallasTexas
| | - Gates B Colbert
- Division of Nephrology, Department of Internal Medicine, Baylor University Medical Center at DallasDallasTexas
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Radiological manifestations of renal osteodystrophy in the orofacial region: a case report and literature review. Oral Radiol 2018; 34:262-266. [PMID: 30484027 DOI: 10.1007/s11282-017-0291-y] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2017] [Accepted: 05/15/2017] [Indexed: 02/05/2023]
Abstract
Renal osteodystrophy (ROD) is a metabolic skeletal pathology that frequently affects the orofacial region and presents with characteristic radiological manifestations. This paper reports a case of ROD causing diffused orofacial bone alterations in a female patient with end-stage chronic kidney disease. The radiological features on panoramic, posteroanterior and lateral skull and computed tomography radiographs are described for this case. Previous reports in the literature are reviewed and discussed with a focus on radiological characteristics and differential diagnoses.
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Pontes FSC, Lopes MA, de Souza LL, dos Santos da Mata Rezende D, Santos-Silva AR, Jorge J, da Silva WG, Pires FR, Rocha AC, de Campos WG, Caldato MCF, Martin RM, Fonseca FP, Pontes HAR. Oral and maxillofacial manifestations of chronic kidney disease–mineral and bone disorder: a multicenter retrospective study. Oral Surg Oral Med Oral Pathol Oral Radiol 2018; 125:31-43. [DOI: 10.1016/j.oooo.2017.09.011] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/28/2017] [Revised: 09/21/2017] [Accepted: 09/21/2017] [Indexed: 12/14/2022]
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Systematic review of oral manifestations related to hyperparathyroidism. Clin Oral Investig 2017; 22:1-27. [DOI: 10.1007/s00784-017-2124-0] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/05/2016] [Accepted: 05/10/2017] [Indexed: 12/29/2022]
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Tunç E, Demirhan O, Sağliker Y, Yildiz İ, Paylar N, Güzel Aİ. Chromosomal findings and sequence analysis of target exons of calcium-sensingreceptor (CaSR) gene in patients with Sagliker syndrome. Turk J Med Sci 2017; 47:13-21. [PMID: 28263480 DOI: 10.3906/sag-1507-102] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/13/2015] [Accepted: 01/28/2016] [Indexed: 12/29/2022] Open
Abstract
BACKGROUND/AIM Sagliker syndrome (SS) develops as a continuation of chronic kidney disease and secondary hyperparathyroidism conditions. It was thought that there are some genetic predisposition factors leading to SS. The calcium-sensing receptor (CaSR) is essential for calcium homeostasis in the body. We aimed to examine SS patients for chromosome aberrations (CAs) and CaSR gene abnormalities in exons 2 and 3. MATERIALS AND METHODS Twenty-three patients and 23 control subjects were admitted to Balcalı Hospital of the Medical Faculty of Çukurova University in Turkey between 2009 and 2011. Chromosomal analysis was performed according to standard cytogenetic methods. Full sequencing of exons 2 and 3 of the CaSR gene was done. RESULTS We found base alterations and deletions in exons 2 and 3 of the CaSR gene. We also found a statistically significant increase in the rate of CAs in patients compared to controls. In total we evaluated 639 metaphase plaques in 23 patients and found 241 CAs, of which 88% were structural and 12% were numerical abnormalities. CONCLUSION There is no relation between the etiology of SS and nucleotide alterations that we could find in exons 2 and 3 of the CaSR gene. Our data suggest that there may be a correlation between CAs and the progression of SS.
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Affiliation(s)
- Erdal Tunç
- Department of Medical Biology and Genetics, Faculty of Medicine, Çukurova University, Adana, Turkey
| | - Osman Demirhan
- Department of Medical Biology and Genetics, Faculty of Medicine, Çukurova University, Adana, Turkey
| | - Yahya Sağliker
- Department of Internal Medicine, Faculty of Medicine, Çukurova University, Adana, Turkey
| | | | - Nuray Paylar
- Department of Internal Medicine, Faculty of Medicine, Çukurova University, Adana, Turkey
| | - Ali İrfan Güzel
- Department of Medical Biology and Genetics, Faculty of Medicine, Recep Tayyip Erdoğan University, Rize, Turkey
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Demirhan O, Arslan A, Sagliker Y, Akbal E, Ergun S, Bayraktar R, Sagliker HS, Dogan E, Gunesacar R, Ozkaynak PS. Gene mutations in chronic kidney disease patients with secondary hyperparathyroidism and Sagliker syndrome. J Ren Nutr 2015; 25:176-86. [PMID: 25701941 DOI: 10.1053/j.jrn.2014.12.008] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/16/2014] [Revised: 12/22/2014] [Accepted: 12/22/2014] [Indexed: 12/26/2022] Open
Abstract
Sagliker syndrome (SS) develops particularly before puberty while chronic kidney disease (CKD) reaches stage 3 with overt secondary hyperparathyroidism. We conducted screening for mutations in all the 13 exons of GNAS1 gene, all 3 exons of FGF23, and all 18 exons in FGFR3 genes in 23 patients. In 73.9% (17 of 23) patients, 17 genetic abnormalities in GNAS1 were detected. Seven (58.3%) of 12 nucleotide alterations comprised novel missense mutations and 3 nonsense. Mismutations were in different manner. There were also 6 heterozygous transversion polymorphisms in exons. Six were introngenic mutations (introns 65626, 70387, 70817). We found 10 mutations with different manner in FGF23 gene. Two were defined previously but remaining 8 were novel mutations. Three were in intronic region near exon 2. We sequenced all exons and intronic regions near exon-exon junction regions of FGFR3 gene. We found 22 mutations with different manner. Six were defined previously and remaining 16 were novel mutations. Eight of them were in intronic region near exon 11 and the last 2 were in noncoding exonic region of exons. One was in the exon-exon junction region between exon 11 and 12, therefore this mutation might be preventing splicing of this intron. Because the incidence of CKD late stage 3 is around 8% but the incidence of SS is around 0.5% in CKD, these gene mismutations might be responsible for bone deformities such as McCune-Albright syndrome and achondroplasias. Although our patients were not resembling any of them, they could be in between, and SS might be a combination-compulsion of bone dysplasias-hereditary osteodystrophies and CKD.
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Affiliation(s)
| | | | - Yahya Sagliker
- Sagliker Hypertension and Nephrology Unit, Adana, Turkey.
| | | | | | | | | | - Ekrem Dogan
- Sutcu Imam University, Kahramanmaras, Turkey
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24
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Lopes MLDDS, Albuquerque AFM, Germano AR, Queiroz LMG, Miguel MCDC, da Silveira ÉJD. Severe maxillofacial renal osteodystrophy in two patients with chronic kidney disease. Oral Maxillofac Surg 2015; 19:321-327. [PMID: 25784153 DOI: 10.1007/s10006-015-0490-9] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2014] [Accepted: 03/09/2015] [Indexed: 06/04/2023]
Abstract
Renal osteodystrophy (ROD) is the bone pathology that occurs as an uncommon complication related to the several alterations in mineral metabolism present in patients with chronic kidney disease (CKD). This paper describes two cases of severe ROD affecting the maxilla and mandible and causing facial disfigurement of a young and a middle-aged female patient with CKD. Both patients had a history of secondary hyperparathyroidism, previously treated by surgery. The pathogenesis of the disease, as well as its clinical, imaging, and histopathological features, and management of the patient are discussed.
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Affiliation(s)
- Maria Luiza Diniz de Sousa Lopes
- Postgraduate Program in Oral Pathology, Dentistry Department, Federal University of Rio Grande do Norte, Avenida Salgado Filho, 1787-Lagoa Nova, Natal, RN, CEP 59.056-000, Brazil,
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25
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Baracaldo RM, Bao D, Iampornpipopchai P, Fogel J, Rubinstein S. Facial disfigurement due to osteitis fibrosa cystica or brown tumor from secondary hyperparathyroidism in patients on dialysis: A systematic review and an illustrative case report. Hemodial Int 2015; 19:583-92. [DOI: 10.1111/hdi.12298] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022]
Affiliation(s)
- Rafael M. Baracaldo
- Division of Nephrology and Hypertension; Nassau University Medical Center; East Meadow NY USA
| | - Dashi Bao
- Division of Nephrology and Hypertension; Nassau University Medical Center; East Meadow NY USA
| | - Pichet Iampornpipopchai
- Division of Nephrology and Hypertension; Nassau University Medical Center; East Meadow NY USA
| | - Joshua Fogel
- Department of Finance and Business Management; Brooklyn College; Brooklyn NY USA
| | - Sofia Rubinstein
- Division of Nephrology and Hypertension; Nassau University Medical Center; East Meadow NY USA
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26
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Mejía Pineda A, Aguilera ML, Meléndez HJ, Lemus JA, Peñalonzo MA. Sagliker syndrome in patients with secondary hyperparathyroidism and chronic renal failure: Case report. Int J Surg Case Rep 2015; 8C:127-30. [PMID: 25661637 PMCID: PMC4353958 DOI: 10.1016/j.ijscr.2015.01.044] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/04/2014] [Revised: 01/22/2015] [Accepted: 01/23/2015] [Indexed: 12/28/2022] Open
Abstract
Sagliker syndrome is assumed to result from insufficient treatment of secondary hyperparathyroidism in early stages of chronic renal failure. Sagliker syndrome should be added to current surgical indications for parathyroidectomy. Introduction Sagliker described craniofacial, skeletal, neurologic and soft tissue abnormalities in patients with secondary hyperparathyroidism with end-stage chronic renal disease. This unique entity has been attributed to delay in treatment of renal insufficiency. As of 2012 sixty cases have been reported. The aim of the study is to report this entity in five patients and to propose this condition as an indication for parathyroidectomy. Methods We report five patients. All patients had chronic renal failure (CRF) and secondary hyperparathyroidism that fulfilled the criteria for Sagliker syndrome and underwent parathyroid surgery. We collected detailed information, including clinical history, laboratory data, and old/current photographs for comparison. Results The five patients, four women and one man mean age 21 years, had severe secondary hyperparathyroidism (mean serum preoperative PTH = 3779.6 pg/ml, mean preoperative Ca2+ = 8.83 mg/dl and P3– = 5.66 mg/dl); maxillary and mandibular bone changes; teeth/dental abnormalities; and soft and benign tumors in the mouth. Three had severe uglifying facial appearance. All patients underwent total parathyroidectomy without postoperative complications. After surgery mean serum PTH was 17.16 pg/ml (p = 0.006), P3− = 3.38 mg/dl (p = 0.0068) and Ca2+ = 6.97 mg/dl (p = 0.345). At surgery, average height was 149.8 cm, a mean of 6 cm (p = 0.003) less than at the beginning of CRF. Preoperative Beck Depression Inventory test mean score was 37 (severe depression); 6 months after surgery mean score was 15.8 (mild mood disturbance)(p = 0.0001). Clinical follow-up was satisfactory with a mean follow up time of 14 months. Conclusion We consider that Sagliker syndrome should be added to current surgical indications for parathyroidectomy.
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Affiliation(s)
- Ana Mejía Pineda
- Fellow Endocrine Surgery, Universidad Francisco Marroquín, Guatemala.
| | | | | | - José A Lemus
- Medical Doctor, Universidad Francisco Marroquín, Guatemala.
| | - Marco A Peñalonzo
- Director Endocrine Surgery Fellowship, Universidad, Francisco Marroquín, Guatemala.
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Shiue I. Hypertension, diabetes and poor mental health are associated with dissatisfaction of teeth appearance: Scottish Health Survey, 2012. Int J Cardiol 2014; 179:52-4. [PMID: 25464410 DOI: 10.1016/j.ijcard.2014.10.079] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/06/2014] [Accepted: 10/19/2014] [Indexed: 11/26/2022]
Affiliation(s)
- Ivy Shiue
- School of Energy, Geoscience, Infrastructure & Society, Heriot-Watt University, UK; Owens Institute for Behavioral Research, University of GA, USA.
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28
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James BC, Hwang JL, Grogan RH, Kaplan EL, Sarne D, Angelos P. Leontiasis ossea caused by long-standing hyperparathyroidism secondary to chronic renal failure. Surgery 2014; 156:1644-6. [PMID: 25244968 DOI: 10.1016/j.surg.2014.08.082] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/05/2014] [Accepted: 08/22/2014] [Indexed: 11/16/2022]
Affiliation(s)
- Benjamin C James
- Department of Surgery, Endocrine Surgery Research Program, The University of Chicago, Chicago, IL.
| | - Jessica L Hwang
- Section of Adult and Pediatric Endocrinology, Diabetes, and Metabolism, The University of Chicago, Chicago, IL
| | - Raymon H Grogan
- Department of Surgery, Endocrine Surgery Research Program, The University of Chicago, Chicago, IL
| | - Edwin L Kaplan
- Department of Surgery, Endocrine Surgery Research Program, The University of Chicago, Chicago, IL
| | - David Sarne
- Section of Adult and Pediatric Endocrinology, Diabetes, and Metabolism, The University of Chicago, Chicago, IL
| | - Peter Angelos
- Department of Surgery, Endocrine Surgery Research Program, The University of Chicago, Chicago, IL
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Yang G, Zhang B, Zha XM, Wang NN, Xing CY. Total parathyroidectomy with autotransplantation for a rare disease derived from uremic secondary hyperparathyroidism, the uremic leontiasis ossea. Osteoporos Int 2014; 25:1115-21. [PMID: 23989901 DOI: 10.1007/s00198-013-2488-1] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/02/2013] [Accepted: 08/02/2013] [Indexed: 12/21/2022]
Abstract
SUMMARY We described six uremic leontiasis ossea (ULO) patients who underwent total parathyroidectomy with autotransplantation. ULO demonstrated more a systemic disease than a simple craniofacial deformation. The surgery seemed an effective treatment to alleviate secondary hyperparathyroidism and to improve patients' quality of life. ULO may have a high postoperative recurrence tendency. INTRODUCTION ULO is a rare disease derived from uremic secondary hyperparathyroidism (SHPT). Previous studies mostly focused on the craniofacial deformations. This study aims to investigate the systemic features of the disease and the surgical outcomes. METHODS The present study retrospectively assessed six ULO patients who underwent total parathyroidectomy (TPTX) with autotransplantation (AT). Follow-up data were recorded. The follow-up status was considered as "effectiveness" if serum intact parathyroid hormone (iPTH) levels were <150 pg/mL in the first 3 days after surgery, or as "recurrence" if serum iPTH gradually increased >300 pg/mL during follow-up in patients whose status was initially considered as "effectiveness". RESULTS Craniofacial deformations, short stature, thoracocyllosis, spine malformations, osteodynia, and muscle weakness were observed in all patients. Abnormal pulmonary functions were observed in five patients. After surgery, one patient died from respiratory failure. Surgery was effective in the remaining five patients with relieved osteodynia and stopped craniofacial deformation. A mean follow-up of 7.6 (4 to 12) months was available. Three patients suffered from recurrence of hyperparathyroidism originating from autografts. CONCLUSIONS Our data suggests that ULO is not only a simple disease with craniofacial malformations but is a severe systemic disease leading to increased surgical risks. TPTX with AT seems an effective treatment to relieve SHPT and to improve quality of life. ULO may have a high postoperative recurrence tendency.
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Affiliation(s)
- G Yang
- Department of Nephrology, First Affiliated Hospital of Nanjing Medical University, 300# Guangzhou Road, Nanjing, Jiangsu, 210029, China
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Chen XH, Shen B, Zou J, Ding X, Liu Z, Lv W, Cao X, Nie Y. Clinical status of Sagliker syndrome: a case report and literature review. Ren Fail 2014; 36:800-3. [PMID: 24575953 DOI: 10.3109/0886022x.2014.890110] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022] Open
Abstract
In a 53-year-old woman, Sagliker syndrome developed during 22 years of treatment with intermittent hemodialysis as a result of severe secondary hyperparathyroidism (SHPT) complicating end-stage renal disease. She failed medical managements and lost her renal graft just after the kidney transplantation due to acute rejection. Although surgical parathyroidectomy was effective, the parathyroid hormone level became extremely high again due to recurrent hyperparathyroidism. It is possible that such patient could survive long-term with dialysis, but prevention of severe SHPT is the most important.
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Affiliation(s)
- Xiao Hong Chen
- Blood Purification Center, Zhongshan Hospital of Fudan University , Shanghai , P.R. China and
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31
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Yavascan O, Kose E, Alparslan C, Sirin Kose S, Bal A, Kanik A, Aksu N. Severe Renal Osteodystrophy in a Pediatric Patient With End-Stage Renal Disease: Sagliker Syndrome? J Ren Nutr 2013; 23:326-30. [DOI: 10.1053/j.jrn.2012.07.002] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/11/2012] [Revised: 06/11/2012] [Accepted: 07/11/2012] [Indexed: 12/26/2022] Open
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Yildiz I, Sagliker Y, Demirhan O, Tunc E, Inandiklioglu N, Tasdemir D, Acharya V, Zhang L, Golea O, Sabry A, Ookalkar DS, Capusa C, Radulescu D, Garneata L, Mircescu G, Ben Maiz H, Chen CH, Prado Rome J, Benzegoutta M, Paylar N, Eyuboglu K, Karatepe E, Esenturk M, Yavascan O, Grzegorzevska A, Shilo V, Mazdeh MM, Francesco RC, Gouda Z, Adam SM, Emir I, Ocal F, Usta E, Kiralp N, Sagliker C, Ozkaynak PS, Sagliker HS, Bassuoni M, Sekin O. International evaluation of unrecognizably uglifying human faces in late and severe secondary hyperparathyroidism in chronic kidney disease. Sagliker syndrome. A unique catastrophic entity, cytogenetic studies for chromosomal abnormalities, calcium-sensing receptor gene and GNAS1 mutations. Striking and promising missense mutations on the GNAS1 gene exons 1, 4, 10, 4. J Ren Nutr 2012; 22:157-61. [PMID: 22200434 DOI: 10.1053/j.jrn.2011.10.030] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2011] [Accepted: 10/13/2011] [Indexed: 12/21/2022] Open
Abstract
Hypotheses explaining pathogenesis of secondary hyperparathyroidism (SH) in late and severe CKD as a unique entity called Sagliker syndrome (SS) are still unclear. This international study contains 60 patients from Turkey, India, Malaysia, China, Romania, Egypt, Tunisia, Taiwan, Mexico, Algeria, Poland, Russia, and Iran. We examined patients and first degree relatives for cytogenetic chromosomal abnormalities, calcium sensing receptor (Ca SR) genes in exons 2 and 3 abnormalities and GNAS1 genes mutations in exons 1, 4, 5, 7, 10, 13. Our syndrome could be a new syndrome in between SH, CKD, and hereditary bone dystrophies. We could not find chromosomal abnormalities in cytogenetics and on Ca SR gene exons 2 and 3. Interestingly, we did find promising missense mutations on the GNAS1 gene exons 1, 4, 10, 4. We finally thought that those catastrophic bone diseases were severe SH and its late treatments due to monetary deficiencies and iatrogenic mistreatments not started as early as possible. This was a sine qua non humanity task. Those brand new striking GNAS1 genes missense mutations have to be considered from now on for the genesis of SS.
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Hamrahian M, Pitman KT, Csongrádi É, Bain JH, Kanyicska B, Fülöp T. Symmetrical craniofacial hypertrophy in patients with tertiary hyperparathyroidism and high-dose cinacalcet exposure. Hemodial Int 2012; 16:571-6. [PMID: 23228066 DOI: 10.1111/j.1542-4758.2012.00670.x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Abstract
We are reporting on a series of two patients with end-stage renal disease on hemodialysis, presented for surgical parathyroidectomy secondary refractory hyperparathyroidism. Both patients had failed maximized medical managements, including higher-than-usual doses of the calcimimetic cinacalcet (270 and 180 mg/day, respectively). On physical exam, both patients had marked symmetrical craniofacial hypertrophy with coarse distortion of facial features, similar in appearance to past reports of Sagliker syndrome. On X-ray and computed tomographic exam, they had peculiar areas of bone absorption on the skull, imitating the radiologic appearance of multiple myeloma. Bone biopsy of the maxilla, however, did not show the expected brown tumor, but rather described only fibrosis and reactive bone formations. This phenotype developed while being on cinacalcet, progressed despite escalation of therapy, and improved only after parathyroidectomy. Both patients developed massive "hungry bone syndrome" after parathyroidectomy necessitating prolonged i.v. calcium infusion. This pattern of severe facial distortion likely represented an adverse consequence of severe tertiary hyperparathyroidism, along with supraphysiologic dose of cinacalcet administration and 25-hydroxy vitamin D deficiency in sensitive individuals. The genetic base of this observation remained unexplained.
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Affiliation(s)
- Mehrdad Hamrahian
- Department of Medicine, Division of Nephrology, University of Mississippi Health Care and University of Mississippi Medical Center, Jackson, Mississippi 39216-4505, USA
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Erkan AN, Sagliker Y, Yildiz I, Ozluoglu L. Audiological findings in chronic kidney disease patients with Sagliker syndrome. J Ren Nutr 2011; 20:S56-8. [PMID: 20797572 DOI: 10.1053/j.jrn.2010.06.002] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022] Open
Abstract
Potential hearing loss was found to be high in a 10 patients with chronic kidney disease and Sagliker syndrome. The cause of hearing loss in these subjects remains unknown. We do not know whether those are the results of preexisting renal disease, hemodialysis, or other factors. Thus, future studies will include more subjects with Sagliker syndrome to determine the frequency of hearing loss and to investigate the etiologic factors that cause loss of hearing.
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Affiliation(s)
- Alper N Erkan
- Department of Otorhinolaryngology, Baskent University Faculty of Medicine, Ankara, Turkey
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Ozenli Y, Giray S, Sagliker Y, Adam SM. A controlled study of psychiatric manifestations and electroencephalography findings in chronic kidney disease patients with Sagliker syndrome. J Ren Nutr 2011; 20:S51-5. [PMID: 20797571 DOI: 10.1053/j.jrn.2010.06.001] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
Abstract
Sagliker syndrome (SS) is a novel syndrome that was described in 2004 in patients with chronic kidney disease (CKD). The aim of this study was to assess psychiatric evaluations and electroencephalography (EEG) findings of patients with CKD and SS to compare them with patients with CKD having characteristics similar to that of the study group, in terms of age and gender. The study group comprised 13 patients with CKD and SS. The control group included 13 patients with CKD. Psychiatric diseases were diagnosed using the Structure Clinical Interview. Beck Depression Inventory, Beck Anxiety Inventory, Social Comparison Scale, Hopelessness Scale, and Mini Mental State Examination (MMSE) were administered to the groups. Moreover, EEG recording for all the patients was performed. According to the results obtained from the Structure Clinical Interview, 69.2% of patients with CKD and SS were diagnosed with a mental disease, as compared with only 3 (23.1%) patients with CKD. There was a significant difference between the study and the control group (P < .001). As compared with the control group, patients with CKD and SS had significantly higher scores on the Beck Depression Inventory, the Beck Anxiety Inventory, and the Hopelessness Scale. However, patients with CKD and SS had significantly lower scores on the Social Comparison Scale. The MMSE scores were not significantly different between the 2 groups. When the 2 groups were evaluated separately, no significant differences were found between the EEG abnormalities and psychiatric diagnosis of both the groups. However, an evaluation of EEG abnormalities in all cases with CKD suggested a statistically significant difference between them. In the EEG recordings, electrical seizures activity was not enrolled in any of the cases. In the present study, psychiatric morbidity for patients with CKD and SS was worse than for patients with only CKD. These results indicate a need to develop an effective psychologic strategy for dealing with psychiatric disorders among patients with CKD and SS.
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Affiliation(s)
- Yarkin Ozenli
- Department of Psychiatry, Faculty of Medicine, Baskent University, Ankara, Turkey
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Eversole R, Su L, ElMofty S. Benign fibro-osseous lesions of the craniofacial complex. A review. Head Neck Pathol 2008; 2:177-202. [PMID: 20614314 PMCID: PMC2807558 DOI: 10.1007/s12105-008-0057-2] [Citation(s) in RCA: 160] [Impact Index Per Article: 9.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/21/2008] [Accepted: 04/22/2008] [Indexed: 10/22/2022]
Abstract
Benign fibro-osseous lesions of the craniofacial complex are represented by a variety of disease processes that are characterized by pathologic ossifications and calcifications in association with a hypercellular fibroblastic marrow element. The current classification includes neoplasms, developmental dysplastic lesions and inflammatory/reactive processes. The definitive diagnosis can rarely be rendered on the basis of histopathologic features alone; rather, procurement of a final diagnosis is usually dependent upon assessment of microscopic, clinical and imaging features together. Fibrous dysplasia and osteitis deformans constitute two dysplastic lesions in which mutations have been uncovered. Other dysplastic bone diseases of the craniofacial complex include florid osseous dysplasia, focal cemento-osseous dysplasia and periapical cemental dysplasia, all showing a predilection for African descent individuals; although no specific genetic alterations in DNA coding have yet to be uncovered and most studies have been derived from predominant high African descent populations. Ossifying fibromas are neoplastic lesions with four subtypes varying with regard to behavior and propensity for recurrence after surgical excision. The clinicopathologic and molecular features of this unique yet heterogeneous group of diseases are reviewed.
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Affiliation(s)
- Roy Eversole
- Department of Pathology and Medicine, Arthur Dugoni School of Dentistry, University of the Pacific, San Francisco, CA, USA.
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Sagliker Y, Acharya V, Ling Z, Golea O, Sabry A, Eyupoglu K, Ookalkar DS, Tapiawala S, Durugkar S, Khetan P, Capusa C, Univar R, Yildiz I, Cengiz K, Bali M, Ozkaynak PS, Sagliker HS, Paylar N, Adam SM, Balal M, Paydas S, Demirhan O, Tasdemir D, Maiz HB, Redulescu D, Garneata L, Mircescu G, Hong-Liang R, Lun L, Yildizer K, Emir I, Yuksekgonul M, Yenicerioglu Y, Akar H, Sagliker C, Esenturk M, Kiralp N. International Study on Sagliker Syndrome and Uglifying Human Face Appearence in Severe and Late Secondary Hyperparathyroidism in Chronic Kidney Disease Patients. J Ren Nutr 2008; 18:114-7. [DOI: 10.1053/j.jrn.2007.10.023] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022] Open
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Uzel A, Uzel I, Sagliker Y, Yildiz I, Halvaci I, Paylar N, Ocal F, Balal M, Ozkaynak PS, Paydas S, Sagliker C, Sagliker HS, Kiralp N, Adam SM, Esenturk M, Gocmez E, Taskapan H, Yuksekgonul M, Emir I, Guler T, Yakar H, Sekin O, Kayali E, Caliskan S, Eskiocak AF, Ogruk B, Guzelyurt T, Kurt C. Cephalometric evaluation of patients with Sagliker syndrome: uglifying human face appearance in severe and late secondary hyperparathyroidism in chronic renal failure patients. J Ren Nutr 2006; 16:229-32. [PMID: 16825025 DOI: 10.1053/j.jrn.2006.04.019] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022] Open
Abstract
It is well known that secondary hyperparathyroidism may be an extremely severe condition in chronic renal failure, and almost all patients with chronic kidney disease, even in the well-developed countries, encounter every kind of bone abnormalities if they are not treated properly. Although some sporadic cases have been reported of unique facial bone changes, the largest collection of this phenomenon has been reported by Sagliker et al. We also have found 6 of 9 patients who have these changes (Sagliker syndrome) to manifest class II malocclusion of the upper and lower jaws according to dental universally accepted criteria by performing cephalometric studies, x-ray plain films, tomographic procedures, and drawing technology.
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Affiliation(s)
- Asli Uzel
- Cukurova University Dentistry Faculty, Adana, Turkey
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Giray S, Sagliker Y, Yildiz I, Halvaci I, Paylar N, Ocal F, Balal M, Ozkaynak PS, Paydas S, Sagliker C, Sagliker HS, Kiralp N, Adam SM, Esenturk M, Gocmez E, Taskapan H, Yuksekgonul M, Emir I, Guler T, Yakar H, Sekin O, Kayali E, Caliskan S, Eskiocak AF, Ogruk B, Guzelyurt T, Kurt C. Neurologic manifestations in Sagliker syndrome: uglifying human face appearance in severe and late secondary hyperparathyroidism in chronic renal failure patients. J Ren Nutr 2006; 16:233-6. [PMID: 16825026 DOI: 10.1053/j.jrn.2006.04.018] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022] Open
Abstract
Patients with chronic renal failure (CRF) often have signs and symptoms related to fluid and electrolyte disturbances, anemia, malnutrition, bone disease, and gastrointestinal problems. Vascular and neurologic impairment in particular remain an important source of morbidity and mortality in this vulnerable patient population. Sagliker syndrome is a novel syndrome that was recently described in 2004 in patients with CRF and severe and late secondary hyperparathyroidism who suffered from severe skull and facial bone changes, particularly from uglifying human face appearances and neuropsychiatric disorders. The goal of this study was to assess neuropsychiatric manifestations occurring in CRF patients with Sagliker syndrome. Four female and 8 male patients with CRF on regular dialysis at the hemodialysis units of the Internal Medicine Departments around southern Turkey participated in the study. All patients underwent a clinical neurologic examination performed by the same neurologist. Neuropsychiatric signs and symptoms were found in all cases. The results showed that the most frequent neurologic manifestations in CRF patients with Sagliker syndrome were headache, polyneuropathy, cranial neuropathy, fatigue, and psychiatric disorders.
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Affiliation(s)
- Semih Giray
- Baskent University Adana Teaching and Research Hospital Neurology Department, Adana, Turkey
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