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Metabolism, Diabetes, and Cancer / Cancer, Hypoxia, and Metabolism · Journal article
Jimd Reports · August 13, 2026
Early or partial results. Treat as a signal, not a conclusion.
This case series describes the clinical management and outcomes of three pregnant women with fructose-1,6-bisphosphatase deficiency, a rare metabolic disorder predisposing to hypoglycemia and lactic acidosis. All three had successful deliveries, but two developed lactic acidosis postpartum and one experienced a severe life-threatening hypoglycemic episode with lactic acidosis during pregnancy despite multidisciplinary management. The report highlights diagnostic and management challenges specific to pregnancy in this rare condition but provides no evidence base for the efficacy of any particular intervention.
Single-centre case series. Three pregnant women with confirmed fructose-1,6-bisphosphatase deficiency managed at a single centre; all were obese at presentation.. Intervention: Multidisciplinary management by obstetricians, maternal-fetal medicine specialists, metabolic physicians, and metabolic dieticians; dietary intervention attempted in all patients.. n = 3. Single centre (not specified in source text).
Three patients with fructose-1,6-bisphosphatase deficiency had successful pregnancies and deliveries with multidisciplinary management. Two of three patients developed gestational diabetes mellitus during pregnancy. One patient with morbid obesity experienced severe life-threatening hypoglycemia with lactic acidosis requiring intensive care during pregnancy; she recovered fully with no adverse maternal or fetal effects.
One patient with morbid obesity experienced severe life-threatening hypoglycemia with lactic acidosis requiring intensive care during pregnancy; she recovered fully with no adverse maternal or fetal effects.
Clinicians managing pregnancy in patients with fructose-1,6-bisphosphatase deficiency should be aware that multidisciplinary care can support successful delivery, but the disorder poses specific challenges in managing comorbid gestational or type 2 diabetes because it predisposes to hypoglycemia. The postpartum period appears to carry particular risk of lactic acidosis even with stable glucose levels.
A single-centre case series of three patients with a rare metabolic disorder; descriptive report of clinical management and outcomes without a comparator or control group.
As stated by the source record.
Quoted from the source exactly as published.
Clinicians managing pregnancy in patients with fructose-1,6-bisphosphatase deficiency should be aware that multidisciplinary care can support successful delivery, but the disorder poses specific challenges in managing comorbid gestational or type 2 diabetes because it predisposes to hypoglycemia. The postpartum period appears to carry particular risk of lactic acidosis even with stable glucose levels.
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ABSTRACT Fructose‐1,6‐bisphosphatase deficiency is a rare inherited metabolic disorder of gluconeogenesis characterized by recurrent hypoglycemia and lactic acidosis, typically triggered by inadequate glucose intake or increased consumption of fructose, sucrose, or sorbitol. During pregnancy, the risk of hypoglycemia and lactic acidosis increases because endogenous glucose production is limited while fetal glucose demand rises. Here we report three patients with fructose‐1,6‐bisphosphatase deficiency who had successful pregnancies and deliveries. These patients were managed by a multidisciplinary team of obstetricians, maternal‐fetal medicine specialists, metabolic physicians, and metabolic dieticians. All patients were obese; one had morbid obesity. Two patients developed gestational diabetes mellitus. One responded to dietary intervention, whereas the patient with morbid obesity had poor dietary adherence, suboptimal glycemic control, and experienced an episode of severe life‐threatening hypoglycemia with lactic acidosis requiring intensive care. She recovered fully, with no adverse maternal or fetal effects. All patients had normal vaginal deliveries and were closely monitored during labor, delivery, and the postpartum period. Both patients with gestational diabetes developed lactic acidosis immediately after delivery despite stable blood glucose levels. Managing gestational or type 2 diabetes in patients with fructose‐1,6‐bisphosphatase deficiency is particularly challenging because the disorder predisposes them to hypoglycemia. This case series contributes to the limited literature on pregnancy outcomes and highlights key management challenges in this rare condition.
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