• Female
• Humans
• Pregnancy
• Acute Disease
• Hypertriglyceridemia/therapy
• Hypertriglyceridemia/complications
• Pancreatitis/therapy
• Pancreatitis/etiology
• Plasmapheresis/methods
• Pregnancy Complications/therapy

• dyslipidaemias
• pancreatitis
• acute pancreatitis

• Humans
• Pancreatitis/etiology
• Pancreatitis/therapy
• Retrospective Studies
• Acute Disease
• Multiple Organ Failure/epidemiology
• Hyperlipidemias
• Hypertriglyceridemia/complications
• Hypertriglyceridemia/therapy
• Blood Component Removal

• Scientific Research Project of Zhejiang Provincial Department of Education (Natural Science)
• “Ten Thousand Plan” Innovation Leader of Zhejiang Province

• curative effect
• hypertriglyceridaemia
• insulin
• severe
• third trimester

Glycogen storage disease type Ia (GSDIa) is an autosomal recessive inborn error of carbohydrate metabolism that is caused by deficiency of the enzyme glucose-6-phosphatase (G6Pase), leading to disturbed glycogenolysis and gluconeogenesis. Patients with GSDIa show severe fasting hypoglycemia, hyperlipidemia, hyperlactacidemia, and hyperuricemia, which are associated with fatal outcomes in pregnant women and fetuses.

Herein, we report the case of a 24-year-old female who on her first visit to the hospital, presented with pregnancy combined with extremely high hyperlipidemia and hyperlactic acidosis with anemia, and frequent hypoglycemia occurred during the treatment. Genetic tests revealed a mutation in the G6Pase gene (G6PC) at 17q21, the patient was finally diagnosed with glycogen storage disease type Ia for the first time after 22 years of inaccurate treatment. She has been treated with a continuous double filtration plasmapheresis (DFPP) strategy to remove blood lipids, and a cornstarch diet therapy. The patient did not develop pancreatitis during the course of the disease and a healthy baby girl weighing 3 kg was delivered.

Patients with GSDIa may be misdiagnosed as epilepsy. DFPP can be used to control hyperlipidemia in GSDIa patients during pregnancy.

• Glycogen storage disease type Ia
• Pregnancy
• Hyperlipidemia
• Double filtration plasmapheresis
• Case report

• blood purification
• hypertriglyceridemia
• metabolic disorders
• pancreatitis
• stroke

• Female
• Humans
• Adult
• Pancreatitis/complications
• Pancreatitis/therapy
• Acute Disease
• Hypertriglyceridemia/complications
• Hypertriglyceridemia/therapy
• Hyperlipidemias/complications
• Triglycerides
• Stroke/complications
• Stroke/therapy

• National Natural Science Foundation of China
• National Key Research and Development Program of China

Protocols for regional citrate anticoagulation with the hypertonic 4% trisodium citrate solution have been recently described as an anticoagulation strategy during membrane therapeutic plasma exchange (mTPE). The effect of citrate in the patient’s systemic hemostasis is negligible, thus regional citrate anticoagulation application is advantageous in circumstances in which heparin-based protocols are deemed unsafe for patients with a high risk of bleeding. The downsides of using hypertonic citrate solutions are mainly hypocalcemia and hypernatremia that ultimately can cause adverse clinical events.

(1) A 57-year-old Caucasian female with a history of active vaginal bleeding secondary to endometrial hyperplasia. She had a history of antiphospholipid syndrome, and systemic lupus erythematosus with marked refractory autoimmune thrombocytopenia. Her platelet count was persistently below 4,000/mm³ even after different immunosuppressive regimens and daily platelet transfusions. (1) A 70-year-old Caucasian female was hospitalized presenting acute kidney injury stage 3 due to rapidly progressive antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, however without the need for renal replacement therapy. At admission, serum creatinine (sCr) was 3.56 mg/dL (normal range: 0.53-1.00 mg/dL). Her baseline sCr was 0.8 mg/dL obtained 6 months earlier. Chest tomography revealed bilateral masses compatible with granulomatous lesions and no signs of alveolar bleeding. Since severe cases of ANCA vasculitis involving the lungs may evolve with alveolar hemorrhage, heparin was avoided.

(1) Systemic lupus erythematosus-associated autoimmune thrombocytopenia and (2) ANCA-associated vasculitis with kidney and lung involvement.

Herein, we describe a case series of 12 consecutive mTPE treatments in 2 different patients using regional 4% trisodium citrate anticoagulation.

All the sessions were uneventful, presented only minor electrolyte imbalances, and were effectively completed without early interruptions due to clotting of the plasmafilter.

In our 2 cases, extracorporeal regional citrate anticoagulation was successful in optimizing plasmafilter patency without bleeding events in 2 high-risk patients using established protocols for the citrate and calcium infusions.

• autoimmune diseases
• plasmapheresis
• regional citrate anticoagulation
• therapeutic plasma exchange