A 17-year-old girl presented with polyuria (7 L/day) and polydipsia for one year. Initial urine osmolality was 113mOsm/kg H₂O. Following 6 h of fluid restriction, serum plasma osmolality reached 300mOsm/kg H₂O, whereas urine osmolality was 108mOsm/kg H₂O. Urine osmolality was increased by 427% from 108 to 557mOsm/kg after vasopressin challenge. The patient was diagnosed with central diabetes insipidus, possibly derived from the atypical occupation of a Rathke's cleft cyst at the pituitary stalk following magnetic resonance imaging with enhancement. She was discharged with desmopressin nasal spray (10 µg); urine output was maintained at 2-3 L/day, and urine osmolality was >300 mOsm/kg. Additional pituitary image studies and evaluation of hypopituitarism should be included in the differential diagnosis of patients with central diabetes insipidus.
Adolescent* ; Deamino Arginine Vasopressin ; Diabetes Insipidus, Neurogenic* ; Diagnosis, Differential ; Female* ; Humans ; Hypopituitarism ; Magnetic Resonance Imaging ; Occupations ; Osmolar Concentration ; Pituitary Gland ; Plasma ; Polydipsia ; Polyuria* ; Vasopressins

BACKGROUND: Determining risk factors for diabetes insipidus (DI) after pituitary surgery is important in improving patient care. Our objective is to determine the factors associated with DI after pituitary surgery. METHODS: We reviewed records of patients who underwent pituitary surgery from 2011 to 2015 at Philippine General Hospital. Patients with preoperative DI were excluded. Multiple logistic regression analysis was performed and a predictive model was generated. The discrimination abilities of the predictive model and individual variables were assessed using the receiving operator characteristic curve. RESULTS: A total of 230 patients were included. The rate of postoperative DI was 27.8%. Percent change in serum Na (odds ratio [OR], 1.39; 95% confidence interval [CI], 1.15 to 1.69); preoperative serum Na (OR, 1.19; 95% CI, 1.02 to 1.40); and performance of craniotomy (OR, 5.48; 95% CI, 1.60 to 18.80) remained significantly associated with an increased incidence of postoperative DI, while percent change in urine specific gravity (USG) (OR, 0.53; 95% CI, 0.33 to 0.87) and meningioma on histopathology (OR, 0.05; 95% CI, 0.04 to 0.70) were significantly associated with a decreased incidence. The predictive model generated has good diagnostic accuracy in predicting postoperative DI with an area under curve of 0.83. CONCLUSION: Greater percent change in serum Na, preoperative serum Na, and performance of craniotomy significantly increased the likelihood of postoperative DI while percent change in USG and meningioma on histopathology were significantly associated with a decreased incidence. The predictive model can be used to generate a scoring system in estimating the risk of postoperative DI.
Area Under Curve ; Craniotomy ; Diabetes Insipidus* ; Discrimination (Psychology) ; Hospitals, General ; Humans ; Incidence ; Logistic Models ; Meningioma ; Neuroendocrinology ; Neurosurgery ; Patient Care ; Postoperative Complications ; Risk Factors ; Specific Gravity ; Vasopressins

Localized granulomatosis with polyangiitis (loc-GPA) is a milder disease state of GPA restricted to the respiratory tract. Transition from localized form to systemic/generalized disease is predicted to occur in approximately 10% of the patients. We report an unusual case of loc-GPA involving multiple cranial nerves, which in 3 years progressed into systemic disease involving pituitary gland. Initially antineutrophil cytoplasmic antibody (ANCA) was negative, but as symptoms of diabetes insipidus started, ANCA became positive. Clinical course of ANCA negative loc-GPA should be carefully monitored for development of systemic disease. ANCA may be a useful marker for detecting transition from localized to systemic disease.
Antibodies, Antineutrophil Cytoplasmic ; Cranial Nerves ; Diabetes Insipidus ; Granulomatosis with Polyangiitis* ; Humans ; Pituitary Gland ; Respiratory System

A 15-year-old boy, who was diagnosed with Alport syndrome and end-stage renal disease, received a renal transplant from a living-related donor. On postoperative day 1, his daily urine output was 10,000 mL despite normal graft function. His laboratory findings including urine, serum osmolality, and antidiuretic hormone levels showed signs similar to central diabetes insipidus, so he was administered desmopressin acetate nasal spray. After administering the desmopressin, urine specific gravity and osmolality increased abruptly, and daily urine output declined to the normal range. The desmopressin acetate was tapered gradually and discontinued 3 months later. Graft function was good, and urine output was maintained within the normal range without desmopressin 20 months after the transplantation. We present a case of a massive polyuria due to transient deficiency of antidiuretic hormone with the necessity of desmopressin therapy immediately after kidney transplantation in a pediatric patient.
Adolescent ; Deamino Arginine Vasopressin ; Diabetes Insipidus, Neurogenic ; Humans ; Kidney Failure, Chronic ; Kidney Transplantation* ; Kidney* ; Male ; Nephritis, Hereditary ; Osmolar Concentration ; Polyuria* ; Reference Values ; Specific Gravity ; Tissue Donors ; Transplants

Group B Streptococcus (GBS) commonly causes neonatal meningitis and sepsis. In infants with late-onset meningitis, fever, irritability or lethargy or both, poor feeding, and tachypnea are common initial signs. Major neurologic sequelae are observed in 29% of children, the most serious including global or profound mental retardation, spastic quadriplegia, cortical blindness, deafness, uncontrolled seizures, hydrocephalus, and hypothalamic dysfunction. We report a 14-day-old full-term female infant who presented with grunting and irritability to the emergency room and was diagnosed with GBS meningitis subsequently complicated with central diabetes insipidus and secondary hypopituitarism. Central diabetes insipidus should be ruled out in infants with complicated GBS meningitis.
Blindness, Cortical ; Child ; Deafness ; Diabetes Insipidus, Neurogenic ; Emergency Service, Hospital ; Female ; Fever ; Humans ; Hydrocephalus ; Hypopituitarism* ; Infant ; Infant, Newborn ; Intellectual Disability ; Lethargy ; Meningitis* ; Quadriplegia ; Seizures ; Sepsis ; Streptococcus ; Tachypnea

Nephrogenic diabetes insipidus (NDI) can cause nonobstructive hydronephrosis. Congenital NDI (CNDI) is caused by a genetic mutation. This case report presents a 12-year-old girl who was incidentally diagnosed with nonobstructive hydronephrosis due to NDI caused by AQP2 gene mutation after being evaluated for microscopic hematuria found on routine health examination at school. The patient's medical and family history was unremarkable, and she complained of nocturia only at the time of the clinic visit. Bilateral hydronephrosis on abdominal ultrasonography prompted a water deprivation test, leading to diagnosis of NDI. Genetic study confirmed p.Asn (AAC)123Ser (AGC) in exon 2 of the AQP2 gene. Polyuria and hydronephrosis improved following arginine-vasopressin therapy. CNDI responsive to treatment should be considered as a possible cause of nonobstructive hydroureter.
Ambulatory Care ; Child ; Diabetes Insipidus, Nephrogenic* ; Diagnosis ; Exons ; Female ; Hematuria ; Humans ; Hydronephrosis* ; Nocturia ; Polyuria ; Ultrasonography ; Water Deprivation

Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis.
Adult ; Amenorrhea ; Biopsy ; Central Nervous System Neoplasms ; Diabetes Insipidus ; Diabetes Insipidus, Neurogenic ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Female ; Follow-Up Studies ; Germinoma ; Histiocytosis, Langerhans-Cell* ; Humans ; Hypopituitarism ; Magnetic Resonance Imaging ; Neoplasms, Germ Cell and Embryonal ; Polydipsia ; Polyuria ; Recurrence ; Sella Turcica

Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis.
Adult ; Amenorrhea ; Biopsy ; Central Nervous System Neoplasms ; Diabetes Insipidus ; Diabetes Insipidus, Neurogenic ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Female ; Follow-Up Studies ; Germinoma ; Histiocytosis, Langerhans-Cell* ; Humans ; Hypopituitarism ; Magnetic Resonance Imaging ; Neoplasms, Germ Cell and Embryonal ; Polydipsia ; Polyuria ; Recurrence ; Sella Turcica

We describe here two Graves' patients who have presented with atypical symptoms. Interestingly, both of them had symptoms related to imbalance of body fluid homeostasis. Chief complaint of a 35-year-old woman (case 1) was the ipsilateral edema of upper body. A 33-year-old man (case 2) who had severe polyuria has referred from urology department with impression of diabetes insipidus. Both of them denied any typical symptoms of Graves' disease at presentation. These rare extrathyroid manifestations of Graves' disease were completely subsided after antithyroid drug treatment. We introduce their clinical courses and possible pathophysiological mechanisms of the atypical symptoms of Graves' disease.
Adult ; Body Fluids ; Diabetes Insipidus ; Edema ; Female ; Graves Disease* ; Homeostasis ; Humans ; Polyuria ; Urology

We describe here two Graves' patients who have presented with atypical symptoms. Interestingly, both of them had symptoms related to imbalance of body fluid homeostasis. Chief complaint of a 35-year-old woman (case 1) was the ipsilateral edema of upper body. A 33-year-old man (case 2) who had severe polyuria has referred from urology department with impression of diabetes insipidus. Both of them denied any typical symptoms of Graves' disease at presentation. These rare extrathyroid manifestations of Graves' disease were completely subsided after antithyroid drug treatment. We introduce their clinical courses and possible pathophysiological mechanisms of the atypical symptoms of Graves' disease.
Adult ; Body Fluids ; Diabetes Insipidus ; Edema ; Female ; Graves Disease* ; Homeostasis ; Humans ; Polyuria ; Urology