RESUMO
CONTEXT: Congenital hyperinsulinism (HI) results in severe, persistent hypoglycemia and is associated with high risk of neurodevelopmental deficits. Sixty percent of HI cases are unresponsive to diazoxide, the only Food and Drug Administration-approved drug. Somatostatin analogs are used off-label as second-line treatment; the long-acting somatostatin analogue, lanreotide, has been used to treat HI over the past decade. Existing reports are limited to small case series. OBJECTIVE: To assess the effectiveness and safety of lanreotide in individuals with HI. DESIGN: Retrospective cohort study of individuals with HI treated with lanreotide between 2015 and 2020. SETTING: The Congenital Hyperinsulinism Center at The Children's Hospital of Philadelphia. PATIENTS: Fifty-four individuals with hyperinsulinism treated with lanreotide. MAIN OUTCOME MEASURES: Fasting duration with plasma glucoseâ >â 70 mg/dL; frequency of lanreotide-associated side effects. RESULTS: The median duration of lanreotide therapy was 28.7 (2.8-64.5) months. Thirty-four patients (63%) had HI due to inactivating mutations of the adenosine 5'-triphosphate (ATP) sensitive potassium channel (KATP-HI), and 39% had undergone a pancreatectomy. Of 52 patients receiving other HI therapies, 22 (42%) were able to discontinue other treatments and were managed on lanreotide alone. Fasting duration with plasma glucoseâ >â 70 mg/dL was significantly longer during therapy with lanreotide compared to prior to lanreotide initiation (8.6â ±â 6.5 vs 5.1â ±â 4.7 hours, Pâ =â 0.001). The most common side effects were subcutaneous nodules (26%) and gallstones (11%). CONCLUSIONS: Lanreotide is a well-tolerated treatment for patients with HI. It results in a longer duration of fasting and a simplification of treatment regimens.
Assuntos
Hiperinsulinismo Congênito , Hiperinsulinismo , Glicemia , Criança , Hiperinsulinismo Congênito/genética , Humanos , Hiperinsulinismo/complicações , Peptídeos Cíclicos/efeitos adversos , Peptídeos Cíclicos/uso terapêutico , Estudos Retrospectivos , Somatostatina/efeitos adversos , Somatostatina/análogos & derivados , Somatostatina/uso terapêuticoRESUMO
PURPOSE OF REVIEW: Women's weight status affects their reproductive functioning, and, likewise, women's lifetime weight trajectories are influenced by reproductive events. We examine the relationship between polycystic ovary syndrome (PCOS), pregnancy, the postpartum period, and gynecological cancers with weight, body image, and other psychiatric issues. RECENT FINDINGS: Women with overweight or obesity are at higher risk for PCOS, and the mood and anxiety symptoms often comorbid with PCOS are linked to weight as well as the core symptoms of the disorder. Excessive gestational weight gain can influence one's body image and mood, and it predicts lasting effects on postpartum weight retention. Finally, overweight and obesity are related to several gynecological cancers. These diseases also impact mood, anxiety, and poor body image. Weight management interventions may be of some benefit in improving the disease states and pregnancy outcomes discussed here, but feasibility, logistics, and costs are issues that remain in delivering such interventions.