下垂体前葉機能低下症

Envenomation by Russell's viper is a major cause of hemotoxic snakebite in South Asia, associated with venom-induced consumptive coagulopathy (VICC), leading to end-organ damage, including the kidneys, adrenal glands, and pituitary gland. Hypopituitarism is a rare but increasingly recognized delayed complication, reported primarily from South India, Sri Lanka, and Myanmar, but not previously reported from Bangladesh. We conducted a prospective longitudinal cohort study at a tertiary referral hospital in Rajshahi, Bangladesh (2019-2025). A total of 203 male patients with Russell's viper envenomation, among them 153 patients were confirmed (43 presented with a brought snake, 110 patients had a photograph of actual bitten snake taken by mobile by the patient or attendant, which was verified by a herpetologist), and 50 patients were suspected (diagnosed using a clinical syndrome-of-snakebite approach) admitted with Russell's viper envenomation were followed up after discharge. During follow-up, those patients presented with persistent fatigue, hypotension, sexual dysfunction, and other suggestive features underwent structured endocrine evaluation and pituitary MRI. Four male patients (two with confirmed identification and two with syndromic approach-based suspected) (1.97%), aged 35-60 years, developed hypopituitarism 3-22 months after severe systemic envenomation complicated by coagulopathy and AKI. All demonstrated secondary adrenal insufficiency (basal cortisol 0.31-3.68 μg/dL) and hypogonadotropic hypogonadism. Two had central hypothyroidism, and one developed central diabetes insipidus. MRI revealed pituitary atrophy with complete or partial empty sella in all cases. All patients showed marked clinical improvement following hormone replacement therapy. Hypopituitarism is an underrecognized but treatable delayed complication of severe Russell's viper envenomation in Bangladesh. Prospective follow-up of high-risk survivors is necessary to assess hormonal deficiency.

OBJECTIVE: Pituitary apoplexy (PA) is an acute hemorrhagic event within the pituitary gland, most often occurring in pre-existing adenomas. In elderly patients, management is challenging due to frailty, comorbidities, and variable presentation. This study aimed to evaluate clinical features, management, and outcomes of PA in the elderly. METHODS: We performed a retrospective analysis of elderly patients (≥65 years) treated for PA at a tertiary referral center in Italy between 2011 and 2022. Data included demographics, clinical presentation, endocrine status, tumor characteristics, frailty (mFI-5), management, and outcomes. RESULTS: Twenty-eight patients (median age 71 years; 79% male) were included. Visual disturbances occurred in 61%, cranial nerve palsy in 71%, and hypopituitarism in 43%. Median mFI-5 was 1. Steroids were administered in 61% of cases. At follow-up, pituitary function did not recover in patients with preoperative hypopituitarism and worsened in 11%, with 10% developing panhypopituitarism. Postoperative hypothyroidism occurred in 54%. Higher frailty showed a non-significant trend toward worse cranial nerve and endocrine outcomes. Steroid therapy was significantly associated with visual improvement (OR 47.1, p = 0.04). CONCLUSIONS: PA in the elderly shows heterogeneous presentation and significant endocrine morbidity. Early diagnosis and prompt steroid therapy are crucial. The endoscopic endonasal approach is safe and effective, but careful patient selection remains essential.

Familial hemophagocytic lymphohistiocytosis type 5 is caused by biallelic pathogenic variants in STXBP2, which encodes syntaxin-binding protein, a key regulator of vesicle trafficking. In addition to immune dysregulation, patients with familial hemophagocytic lymphohistiocytosis type 5 may present with severe, persistent diarrhea associated with microvillus inclusion disease. However, the long-term clinical course of microvillus inclusion disease in this context remains poorly characterized due to limited survival beyond early childhood. We report a 22-year-old male with familial hemophagocytic lymphohistiocytosis type 5 who developed chronic diarrhea in infancy that persisted despite following bone marrow transplantation. The diagnosis of microvillus inclusion disease was established 16 years after transplantation based on characteristic histopathological findings. The patient also developed Fanconi syndrome, a rare complication in this setting, which improved following intensification of parenteral nutrition. Long-term follow-up revealed that the patient was dependent on parenteral nutrition not only for caloric intake but also for the correction of electrolyte imbalance. Growth retardation and multiple endocrine abnormalities improved with nutritional optimization, highlighting the systemic impact of chronic undernutrition. This patient expands the long-term extrahematopoietic phenotype of STXBP2-related familial hemophagocytic lymphohistiocytosis type 5 and underscores the importance of individualized nutritional management.

Pituitary stalk interruption syndrome (PSIS) is a rare congenital anomaly associated with multiple pituitary hormone deficiencies. It is characterized by an absent or thin pituitary stalk, an ectopic posterior pituitary, and a hypoplastic anterior pituitary and is typically diagnosed via MRI. The syndrome presents variably depending on age, with neonatal cases often manifesting as recurrent hypoglycemia. Early diagnosis is critical to prevent severe complications. We report a case of a preterm male neonate born via emergency cesarean section due to non-reassuring cardiotocography and vaginal bleeding in the setting of breech presentation. The neonate developed recurrent hypoglycemia despite glucose therapy. Endocrine evaluations indicated hypopituitarism, with low cortisol, growth hormone, and adrenocorticotropic hormone (ACTH) levels. MRI confirmed PSIS with a hypoplastic anterior pituitary, absent pituitary stalk, and ectopic posterior pituitary. Hormonal replacement therapy with hydrocortisone, levothyroxine, and growth hormone led to clinical stabilization. PSIS should be considered in neonates with unexplained hypoglycemia. Early MRI evaluation and prompt hormonal replacement therapy are vital for preventing complications and improving outcomes. Increasing awareness among clinicians can enhance diagnostic accuracy and optimize patient care.

OBJECTIVE: Cigarette smoking and nicotine dependence are prevalent in the general population and can negatively impact surgical outcomes. However, the effect of preoperative smoking status on the outcomes of endonasal transsphenoidal surgery (ETS) for pituitary adenomas (PA) remains unknown. This study aims to investigate the impact of preoperative smoking status on the outcomes following endonasal transsphenoidal surgery. METHODS: A retrospective, international, multi-center, propensity score matched (PSM), cohort study was conducted using the TriNetX database. Adult patients diagnosed with PA who underwent endoscopic transsphenoidal excision between 2005 and 2024 were identified. Patients with documented cigarette smoking or nicotine dependence within six months prior to ETS were classified as the smoker cohort, while those without either were classified as the non-smoker (control) cohort. Propensity score matching was performed based on key covariates, including age, sex, race, comorbidities, and preoperative neuroendocrine function. RESULTS: The crude cohort included a total of 11,472 patients. After PSM, 1623 patients were included in both the smoker and non-smoker groups. The mean age at ETS was 50.9 years. Compared to the non-smoker group, smoker patients were associated with a higher incidence of postoperative cerebrospinal fluid (CSF) leakage (OR:1.31; 95%CI:1.05-1.62; p = 0.027), meningitis (OR:1.59; 95%CI:1.02-2.50; p = 0.047), postoperative diabetes insipidus (DI) (OR:1.22; 95%CI:1.04-1.43; p = 0.018), desmopressin use (OR:1.29; 95%CI:1.07-1.56; p = 0.009), hypopituitarism (OR:1.06; 95%CI:1.01-1.33; p = 0.048), and steroid use (OR:1.19; 95%CI:1.01-1.40; p = 0.035). Sensitivity analysis demonstrated consistent results across the different PSM models. CONCLUSIONS: Among patients undergoing ETS for PA, preoperative smoking status was associated with higher risks of postoperative CSF leakage, meningitis, DI, desmopressin use, hypopituitarism, and steroid use.