Objective: To review the surgical outcomes of patients who underwent endoscopic endonasal transsphenoidal surgery (EETS) for pituitary adenomas in our institution and compare them with the results of previous studies Methods:Design: Retrospective Review of RecordsSetting: Tertiary National University HospitalParticipants: Records of 56 adult pituitary tumor patients who underwent EETS at the Philippine General Hospital in Manila, Philippines from January 2020 to December 2023 were retrospectively reviewed. Their age, sex, tumor size, presenting symptoms, resection rates, surgery duration and length of stay and complications were recorded. Multivariate linear regression analysis identified factors influencing rates of gross total resection (GTR), surgical duration and hospital stay. Results: GTR was achieved in 82% (n=46/56). The median surgery duration was 260 minutes (IQR 160-329 minutes), and the median hospital stay was 10 days (IQR 19.75-23.25 days). Complications arose in 35.7% (20/56), commonly diabetes insipidus (25%, 14/56) and meningitis (3.6%, 2/56)), but there were no mortalities (0%, 0/56 at 24 months follow-up). No factors were associated with GTR. Galactorrhea ((β = 1.769, 95% CI: 0.531-3.007, p=.010) and tumor size up to 6 cm (β = 0.572, 95% CI: 0.076-1.068, p=.032) were associated with more than 400 minutes operative time. Preoperative visual loss was associated with shorter admissions (-7.4 days, 95% CI: -11.994, -2.853, days, p=.004), while preoperative seizures (+19.4 days, , 95% CI: 6.447-32.327, p=.007), hypercortisolism (+10.9 days, 95% CI: 0.700-21.130, p=.040), and acromegaly (+7.1 days, 95% CI: 0.723-13.523, p=.037) were associated with longer stays. Each centimeter increase in tumor size predicted an increase in hospital stay by 1.1 days (95% CI: 0.071-2.169, p=.049). Conclusion: EETS for pituitary adenomas in the Philippines led to high rates of GTR and manageable complication rates compared to previous studies. However, longer operative times and hospital stays were observed, which may reflect local practices, logistics, and demographics. Improved surgical infrastructure and experience may further optimize outcomes.

GPCRs serve to coordinate renal collecting duct function, orchestrating fine-tuned regulation of water, electrolyte, and acid-base homeostasis. Classical GPCRs such as the vasopressin V2 receptor (V2R) have well-established roles: the V2R serves to regulate water reabsorption, and V2R dysregulation can underly diabetes insipidus. However, transcriptomic and phosphoproteomic advances have revealed a diverse repertoire of understudied GPCRs expressed in the collecting duct whose physiological roles remain largely undefined. Here, we review emerging insights into three such receptors-GPR39, ADGRF5, and ADGRG3-highlighting their distinct contributions to collecting duct function. We also discuss atypical GPCR-related signaling, including PKD1 and MAGED2, which expand the conceptual framework of GPCR biology in renal function. Collectively, these findings illustrate that GPCRs play diverse and underappreciated roles in collecting duct physiology and pathophysiology, and that continued mechanistic studies may uncover new therapeutic opportunities for kidney disease.

Case series summary A 2-year-old neutered male domestic shorthair cat presented with severe lethargy, hyporexia, dehydration, and poor body condition. The incidence of polyuria/polydipsia was unknown because of the presence of multiple other cats in the household. Laboratory findings revealed severe hypernatremia, isosthenuria, hemoconcentration, and moderate azotemia. MRI of the head demonstrated partial hypoplasia/aplasia of the hypothalamus and pituitary gland, confirming congenital central diabetes insipidus; additional endocrine testing excluded other causes. Initial fluid therapy improved azotemia and stabilized the hematocrit, but hypernatremia persisted until desmopressin therapy was initiated. The cat was successfully managed long-term with gradually titrated oral desmopressin, achieving normalization of electrolytes. Relevance and novel information This report details the first documented case of central diabetes insipidus in an adult cat, attributed to presumed partial aplasia of the hypothalamus and pituitary gland, with no other hormonal deficiencies.

Primary hypothalamic involvement in primary central nervous system lymphoma is rare and can present with nonspecific neurological and endocrine manifestations, resulting in significant diagnostic delays. We report the case of an immunocompetent 29-year-old female who presented with intermittent fever, seizures, progressive behavioral changes, central diabetes insipidus, and hypopituitarism. She was initially treated empirically for encephalitis with anti-epileptics and corticosteroids. Neuroimaging subsequently demonstrated a homogeneously enhancing lesion along the chiasmatic-hypothalamic axis. Initial biopsy was nondiagnostic due to corticosteroid pretreatment, necessitating repeat biopsy via craniotomy, which confirmed diffuse large B-cell lymphoma. This case illustrates the importance of recognizing endocrine red flags in hypothalamic lesions, emphasizes the negative impact of prebiopsy steroid exposure on diagnostic yield, obscuring histopathology even in the absence of radiological regression.

Transition from Microsurgical to Endoscopic Endonasal Resection of Pituitary Adenomas: Early Experience and Learning Curve from a Single-Center Cohort in Vietnam.

Trans-sellar encephalocele (TSE) is a rare congenital anomaly of the cranial base characterized by herniation of intracranial contents through the sellar floor. Although clinical presentations are diverse, the prevalence and characteristics of endocrine dysfunction due to hypothalamic-pituitary involvement are unclear. This study aimed to clarify endocrine function in patients with TSE and emphasized the importance of multidisciplinary management. Here, we present two adult cases of TSE with progressive visual impairment and evident pituitary hormone deficiencies, and conducted a comprehensive literature review to investigate endocrine function and its postoperative outcomes in patients with TSE. Both cases presented with growth hormone (GH) deficiency and diabetes insipidus and showed deterioration in GH secretion, which was postoperatively assessed using dynamic testing, despite minimal or no direct manipulation of the pituitary gland. In a literature review, 87 cases from 41 case reports and 3 review articles were identified. Among the 87 cases, pituitary dysfunction was identified in 41 (47.1%); however, detailed hormonal assessments were performed in only 25 (28.7%) of the cases. In 33 cases with documented preoperative and postoperative endocrine status, pituitary function worsened in 16 (48.5%), improved in 6 (18.2%), and remained unchanged in 11 (33.3%) postoperatively. Pituitary dysfunction occurred in nearly half of patients with TSE, but was frequently underdiagnosed due to inadequate endocrine assessment. Comprehensive endocrine evaluation, including dynamic GH testing, performed at diagnosis and perioperatively, may be essential in optimizing patient outcomes.

Three Years to Diagnosis: Idiopathic Central Diabetes Insipidus in a Healthcare Worker

WCN26-6496 A case of fluconazole-induced arginine vasopressin resistance

Transient Syndrome of Inappropriate Antidiuretic Hormone Secretion and Sustained Improvement in Central Diabetes Insipidus After Roux-en-Y Gastric Bypass

WCN26-4926 Tosufloxacin-induced Nephrogenic Diabetes Insipidus in an Infant: A Case Report

Endocrine Disruptions Leading to Hypernatremia and Fluid Imbalance in Neurosarcoidosis: The Challenge of Central Diabetes Insipidus and Adrenal Insufficiency

WCN26-7418 Eosinophilic Granulomatosis with Polyangiitis Following Central Diabetes Insipidus: A Rare Case Report and Literature Review

WCN26-5374 A RARE CASE OF NEPHROGENIC DIABETES INSIPIDUS IN AN ADULT MALE: A DIAGNOSTIC PROBLEM

Concurrent Central Diabetes Insipidus and Panhypopituitarism in Newly Diagnosed Acute Myeloid Leukemia

WCN26-5855 GENERATION OF AVPR2 MISSENSE MUTATION RAT MODELS FOR CONGENITAL NEPHROGENIC DIABETES INSIPIDUS

Diuretics are a cornerstone of supportive therapy in pediatric patients, widely used for the management of edema, fluid overload, hypertension, and various other kidney diseases. Their clinical utility, however, is strongly influenced by developmental differences in kidney physiology, age-related pharmacokinetics, underlying kidney disease, and the risk of adverse effects unique to children. This review presents an extensive, evidence-informed synthesis of diuretic therapy in pediatric kidney disorders, encompassing pharmacology, mechanisms of action, indications, drug interactions, and safety considerations. We review the role of diuretics across common conditions in pediatric nephrology, including nephrotic syndrome, chronic kidney disease, acute kidney injury, hypertension, hypercalcemia, hypercalciuria, and nephrogenic diabetes insipidus. Special populations, including neonates and adolescents, are discussed, highlighting developmental pharmacology, vulnerability to toxicity, and long-term growth and bone health concerns. The review also summarizes emerging data on newer diuretic therapies, including sodium-glucose cotransporter-2 inhibitors, vasopressin receptor antagonists, and non-steroidal mineralocorticoid receptor antagonists, while emphasizing the current limitations of pediatric evidence. Adverse effects, including electrolyte disturbances, nephrocalcinosis, ototoxicity, and metabolic complications, are systematically addressed with practical monitoring recommendations. Overall, this review underscores that diuretics in children should be used judiciously, individualized to age and kidney function, closely monitored for potential harm, and integrated with definitive disease-modifying therapies to optimize outcomes in pediatric kidney care.

Background: Paraneoplastic limbic encephalitis (PLE) with anti-Ma2 antibodies is a rare immune-mediated disorder associated with testicular cancer, particularly in young males. While neurological manifestations are well documented, hypothalamic-pituitary dysfunctions remain underreported. We present a case of anti-Ma2 PLE associated with testicular cancer together with a systematic review of PLE associated with testicular cancer, selectively restricted to anti-Ma2 positive cases and focusing on hypothalamic-endocrine involvement. Case presentation: We describe a 21-year-old male diagnosed with anti-Ma2 PLE and intratubular germ cell neoplasia of the right testis. He underwent orchifunicolectomy and immunosuppressive therapy with neurological improvement. Four years later, he developed new-onset temporal seizures, decreased libido, and a polyuria-polydipsia syndrome. Dynamic endocrine testing, including a water deprivation test and copeptin measurement, supported a diagnosis of partial central diabetes insipidus (CDI). Methods: A systematic literature review was performed in accordance with PRISMA guidelines. PubMed was searched using predefined keywords without time restriction. Studies reporting PLE associated with testicular tumors in humans with confirmed anti-Ma2 antibody positivity were included. Results: Eleven studies were included, reporting a total of 38 patients with anti-Ma2-associated PLE and testicular cancer. Hypothalamic or diencephalic involvement was described in 16 patients (42.0%), while endocrine manifestations were explicitly reported in four cases. Only two previous reports mentioned CDI, without detailed diagnostic evaluation. Conclusions: This study highlights the importance of recognizing hypothalamic-endocrine manifestations in PLE. In patients presenting with polydipsia and polyuria, CDI should be carefully differentiated from primary polydipsia using dynamic testing. Hypothalamic involvement may emerge years after tumor treatment, warranting long-term endocrine surveillance.

Objective: The aim of this study was to evaluate the early clinical and surgical outcomes during the transition from microscopic transsphenoidal surgery to the endoscopic endonasal transsphenoidal approach. Materials and methods: This retrospective study included the first 10 consecutive patients with pituitary tumors who were operated on using the endoscopic endonasal transsphenoidal approach by a surgical team experienced in microscopic transsphenoidal surgery. All procedures were performed by the same multidisciplinary team using a four-hand technique in collaboration with an otorhinolaryngologist. Patients were evaluated in terms of demographic characteristics, tumor type and size, cavernous sinus invasion, extent of resection, complications, and early clinical outcomes. Results: Seven patients were male, and three were female, with a mean age of 52.5±13.1 years. Histopathological examination revealed non-functioning pituitary neuroendocrine tumor (pitNET) in three patients, gonadotroph pitNET in four patients, corticotroph pitNET in two patients, and somatotroph pitNET in one patient. Cavernous sinus invasion was observed in four patients. Early postoperative imaging demonstrated residual tumor in two patients, whereas gross total resection was achieved in eight patients. Intraoperative cerebrospinal fluid leakage occurred in three patients; one patient required additional surgical intervention for a postoperative cerebrospinal fluid fistula. No major vascular complications were observed. Permanent diabetes insipidus developed in one patient. The mean operative time was 4.5±0.6 hours, and the mean intraoperative blood loss was 178±15.5 mL. Conclusion: During the early phase of the transition from microscopic to endoscopic endonasal transsphenoidal surgery, acceptable surgical outcomes can be achieved with appropriate patient selection and a multidisciplinary team approach. These findings suggest that the endoscopic technique can be safely implemented even in the early stages of the learning curve.

Wolfram syndrome is a rare genetic disorder characterized by antibody-negative early-onset atypical diabetes mellitus, optic nerve atrophy, sensorineural hearing loss, diabetes insipidus (arginine vasopressin deficiency), and progressive neurodegeneration, with significant variability in disease severity. We assessed the accuracy of a genotype-based severity scoring system to predict the onset of cardinal symptoms in Wolfram syndrome. This system is based on the type of WFS1 variants (in-frame or out-of-frame) and their location relative to transmembrane domains. Severity scores were assigned to 324 patients with documented onset ages for diabetes mellitus, optic atrophy, hearing loss, and diabetes insipidus. Our analysis revealed a clear correlation between severity scores and earlier onset of diabetes mellitus and optic atrophy. Patients with in-frame variants outside transmembrane domains exhibited milder symptoms, especially WFS1 c.1672C>T (p.Arg558Cys) variant, whereas those with out-of-frame variants showed the earliest onset. Severity scores 3 and 4 did not follow the expected progression, suggesting that transmembrane domain involvement in both alleles may result in greater severity. These findings suggest that this scoring system provides valuable insights into the progression of Wolfram syndrome and may guide clinical care. Further refinement may improve its utility for predicting the onset of non-diabetic symptoms.

Pituitary neuroendocrine tumors (PitNETs) are rare in children and young adolescents under the age of 19years (referred to as CYP). This study describes our experience with endoscopic transnasal management of these tumors at a tertiary neurosurgical center over a 15-year period. This was a retrospective cohort study of patients aged less than 19years of age, with PitNETs diagnosed at our center and managed with endoscopic transnasal transsphenoidal resection surgery between January 2010 and December 2024. Demographic and clinicopathological data, surgical details, adjuvant therapy, hormonal and visual evaluation, and follow-up details were reviewed. The SN-5 questionnaire was used to assess the sinonasal quality of life. A total of 19 patients were enrolled, with a mean age of 15.05years, and a male-to-female ratio of 1.4:1. The most common presenting symptoms were headache in 84% and progressive diminution of vision in 47% of patients. Seventeen patients had functioning adenomas, the most common being GH-secreting adenomas (8). The median volume of the lesions was 9.53cc, with 15 macroadenomas and four microadenomas, with gross total resection achieved in 47.4% and near total resection in 31.6%. Complications included diabetes insipidus (5), CSF rhinorrhea resolving with temporary CSF diversion (6), and pituitary insufficiency requiring long-term hormone supplementation (8). Over a median follow-up period of 24.68months (range 10.5-68.1months), visual acuity improved in 87.5% patients, while 70.6% of patients with functioning adenomas achieved endocrinological remission at the last follow-up. Our experience reinforces the utility of endoscopic transnasal procedures in the pediatric and young-adolescent age group, achieving satisfactory clinical outcomes regarding vision and endocrinological status, which is especially important due to the dominance of functioning adenomas in this age group.