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1.
Diabetes Technol Ther ; 26(4): 279-282, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38194228

RESUMEN

Introduction: Obesity in patients with type 1 diabetes (T1D) may worsen their prognosis. Bariatric surgery in these patients can be associated with complications such as diabetic ketoacidosis and severe hypoglycemic episodes. Closed-loop insulin delivery could be a solution to avoid them. Case Report: A 45-year-old woman with T1D and obesity (body mass index of 38.4 kg/m2) was included in our preoperative course of bariatric surgery. Three months before surgery, a closed-loop insulin delivery was instituted due to one prior severe hypoglycemia. Patient did not have immediate or late postoperative hypoglycemia despite consuming a weak amount of carbohydrate. Three months after surgery glycemic control was on target with 86% of time in range 70-180 mg/dL and no time below 70 mg/dL. Conclusion: This case report shows that the use of a closed-loop insulin delivery made it possible to perform bariatric surgery in complete safety for our patient.


Asunto(s)
Cirugía Bariátrica , Diabetes Mellitus Tipo 1 , Hipoglucemia , Femenino , Humanos , Persona de Mediana Edad , Insulina/uso terapéutico , Diabetes Mellitus Tipo 1/complicaciones , Diabetes Mellitus Tipo 1/tratamiento farmacológico , Diabetes Mellitus Tipo 1/cirugía , Glucemia/metabolismo , Hipoglucemiantes/uso terapéutico , Sistemas de Infusión de Insulina , Hipoglucemia/etiología , Hipoglucemia/prevención & control , Insulina Regular Humana/uso terapéutico , Cirugía Bariátrica/efectos adversos , Obesidad
2.
Front Endocrinol (Lausanne) ; 13: 855082, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35432221

RESUMEN

Background: NR0B1 pathogenic variants can cause congenital adrenal hypoplasia or primary adrenal insufficiency in early childhood usually associated with hypogonadotropic hypogonadism. NR0B1 is necessary for organogenesis of the adrenal cortex and to maintain normal spermatogenesis. In humans, restoration of fertility in patients carrying NR0B1 pathogenic variants is challenging. Objective: The aim of the study was to investigate the clinical, hormonal, histological, spermiological, and molecular genetic characteristics of a cohort of patients with NR0B1 pathogenic variants, monitored for fertility preservation. Patients: We included five patients, including four teenagers, with NR0B1 pathogenic or likely pathogenic variants. They all had primary adrenal insufficiency and were receiving replacement therapy with glucocorticoids and mineralocorticoids. Patients received recombinant follicle-stimulating hormone and recombinant human chorionic gonadotropin in order to induce spermatogenesis. Combined gonadotropin treatment was initiated between 13 years and 15 years and 6 months for the four teenagers and at 31 years and 2 months for the only adult. Physical and hormonal assessments were performed just before starting gonadotropin treatment. After 12 months of gonadotropin treatment, physical examination and hormonal assessments were repeated, and semen analyses were performed. If no sperm cells were observed in at least 2 semen collections at 3-month interval, testicular biopsy for testicular sperm extraction was proposed. Results: Bilateral testicular volume increased from 8 ml (interquartile range, 6-9) to 12 ml (10-16) after gonadotropin treatment. Inhibin B levels were relatively stable: 110 ng/L (46-139) before and 91 ng/L (20-120) at the end of gonadotropin treatment. Azoospermia was observed in all semen analyses for all cases during gonadotropin treatment. Three patients agreed to testicular biopsy; no mature sperm cells could be retrieved in any. Conclusion: We characterized a cohort of patients with NR0B1 pathogenic or likely pathogenic variants for fertility preservation by recombinant gonadotropin treatment, which began either at puberty or in adulthood. No sperm cells could be retrieved in semen samples or testicular biopsy even after gonadotropin treatment, indicating that gonadotropin treatment, even when started at puberty, is ineffective for restoring fertility.


Asunto(s)
Enfermedad de Addison , Hipogonadismo , Enfermedad de Addison/tratamiento farmacológico , Adolescente , Adulto , Preescolar , Gonadotropina Coriónica/uso terapéutico , Receptor Nuclear Huérfano DAX-1/genética , Humanos , Hipogonadismo/tratamiento farmacológico , Masculino , Sustancias para el Control de la Reproducción , Espermatozoides , Testículo
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