A 13-year-old boy was diagnosed as having primary nephrogenic diabetes insipidus, and symptoms developed at 3 years of age. Subsequently he developed bilateral hydronephrosis and a neurogenic bladder. His pedigree could be explored back 5 generations and represented an inheritance as an X-linked recessive transmission factor. He was treated with indomethacin 2 mg/kg/day plus chlorothiazide 500 mg/day and this new treatment showed a markedly decreased urine output and increased urine osmolarity. (Nephrogenic diabetes insipidus, Hydronephrosis, Indomethacin)
Adolescent ; Bladder, Neurogenic/etiology ; Chlorothiazide/therapeutic use ; Diabetes Insipidus/complications ; Diabetes Insipidus/congenital* ; Diabetes Insipidus/drug therapy ; Diabetes Insipidus/genetics ; Drug Therapy, Combination ; Human ; Hydronephrosis/etiology* ; Indomethacin/therapeutic use* ; Male

OBJECTIVETo summarize our experience in the management of adipsic central diabetes insipidus(ADI) accompanied with intracranial calcification.

METHODThe clinical data of one ADI patient accompanied with intracranial calcification who was treated in our hospital since December 2011 were retrospectively summarized.

RESULTSThe 24-hour urine volume was 800 ml. She didn't feel thirsty even with increased plasma sodium concentration(153 mmol/L) and blood osmotic pressure(333 mmol/L) . Combined water deprivation and vasopressin test revealed the diagnosis of central diabetes insipidus. The high intensity signal(on T1-weighted magnetic resonance imaging) in the posterior lobe of pituitary gland was found. Computed tomography showed calcifications in the bilateral basal ganglia.Serum cytomegalovirus IgG was positive. She was treated with desmopressin and asked for regular water intake regardless of the adipsia. The plasma sodium concentration was still below 150 mmol/L during the 4-month follow-up.

CONCLUSIONSRoutine adipsia evaluation and combined water deprivation and vasopressin test are critical for the diagnosis and treatment of ADI. Past insidious intracranial cytomegalovirus infection may explain the cause of ADI and calcification.

Brain Diseases ; complications ; Calcinosis ; complications ; Child ; Cytomegalovirus ; Diabetes Insipidus, Neurogenic ; complications ; diagnosis ; etiology ; Drinking ; Female ; Humans ; Sodium ; blood

Central diabetes insipidus (DI), characterized by unexpected fatal hypernatremia, is a rare complication after successful cardiopulmonary resuscitation with therapeutic hypothermia, but may be potentially fatal if recognition is delayed. We describe here a patient who experienced cardiac arrest due to a pulmonary embolism, followed by successful resuscitation after induction of therapeutic hypothermia. The patient, however, suddenly developed unexpected hypernatremia with increased urine output and was diagnosed with central DI as a complication of cerebral edema, and eventually died. Our findings suggest that central DI should be considered as a possible complication following unexpected hypernatremia with increased urine output during therapeutic hypothermia and that desmopressin acetate should be used to treat central DI.
Adult ; Cardiopulmonary Resuscitation/*adverse effects ; Diabetes Insipidus, Neurogenic/diagnosis/etiology ; Fatal Outcome ; Female ; Heart Arrest/complications/therapy ; Humans ; Hypernatremia/*etiology ; Hypothermia, Induced/*adverse effects ; Pulmonary Embolism/complications

We report a rare case of diabetes insipidus following fire burn injury. Meticulous fluid balance and the use of carbamazepine resulted in her survival.
Burns/*complications ; Carbamazepine/therapeutic use ; Diabetes Insipidus, Neurogenic/drug therapy/*etiology ; Female ; Fires ; Fluid Therapy/methods ; Humans ; Self-Injurious Behavior ; Young Adult

BACKGROUND: 3q21q26 syndrome includes chromosomal abnormalities of inv(3)(q21q26), t(3;3) (q21;q26), and ins(3;3)(q26;q21q26). It causes hematological diseases by the leukemogenic mechanism that the enhancer of ribophorin I gene in 3q21 induces the transcription of ecotropic viral integration site-1 gene in 3q26. Recently, it has been proposed that the 3q21q26 syndrome may be preceded by diabetes insipidus (DI), particularly when combined with monosomy 7, and is a unique disease entity. METHODS: From May 2001 to June 2006, a total of 5 patients with hematologic malignancy were found to have 3q21q26 syndrome and monosomy 7. Laboratory findings, clinical data, and association with DI were investigated. RESULTS: The rearrangement type of 3q21q26 was inv(3)(q21q26) in four patients and t(3;3)(q21; q26) in one. These patients' French American British types were AML M1, M2, M4 and M7, showing evident dysmegakaryopoiesis. Aberrant antigenic expressions of CD7 and CD56 were observed. The platelet count was relatively high as AML. All the five patients were refractory or in early relapse. Patient 5 was diagnosed with AML M7 20 days after being diagnosed with DI. While DI was well controlled with oral desmopressin, leukemia was refractory to chemotherapy. CONCLUSIONS: This study supports the recent opinion that 3q21q26 syndrome with monosomy 7 combined with DI is a disease of unique characteristics. In the relation between DI and monosomy 7 or 3q21q26 syndrome, there has been no explanation about how acquired abnormality of hematopoietic cells affects production of DDAVP by neurohormonal cells in hypothalamus. The mechanism needs further study, and this research should contribute to the understanding of genetic roles in leukemia appearing in different forms.
Adult ; Chromosome Disorders/*complications/*diagnosis/genetics ; *Chromosomes, Human, Pair 3 ; Diabetes Insipidus, Neurogenic/*complications ; Female ; Hematologic Neoplasms/*complications ; Humans ; Inversion, Chromosome ; Karyotyping ; Male ; Middle Aged ; Monosomy ; Prognosis ; Syndrome ; Translocation, Genetic

Central diabetes insipidus (DI) can be the outcome of a number of diseases that affect the hypothalamic-neurohypophyseal axis. The causes of the condition can be classified as traumatic, inflammatory, or neoplastic. Traumatic causes include postoperative sella or transection of the pituitary stalk, while infectious or inflammatory causes include meningitis, lymphocytic hypophysitis, and granulomatous inflammations such as sarcoidosis and Wegener's granulomatosis. Various neoplastic conditions such as germinoma, Langerhans cell histiocytosis, metastasis, leukemic infiltration, lymphoma, teratoma, pituitary adenoma, craniopharyngioma, Rathke cleft cyst, hypothalamic glioma, and meningioma are also causes of central DI. In affected patients, careful analysis of these MR imaging features and correlation with the clinical manifestations can allow a more specific diagnosis, which is essential for treatment.
Adolescent ; Adult ; Aged ; Diabetes Insipidus, Neurogenic/diagnosis/etiology/*pathology ; Female ; Human ; Inflammation/complications ; *Magnetic Resonance Imaging ; Male ; Middle Age ; Neoplasms/complications ; Pituitary Gland, Posterior/injuries/pathology ; Sella Turcica/pathology/surgery

Diabetes insipidus (DI) is characterized by excessive urination and thirst. This disease results from inadequate output of antidiuretic hormone (ADH) from the pituitary gland or the absence of the normal response to ADH in the kidney. We present a case of transient central DI in a patient who underwent a cardiopulmonary bypass (CPB) for coronary artery bypass grafting (CABG). A 44-yr-old male underwent a CABG operation. An hour after the operation, the patient developed polyuria and was diagnosed with central DI. The patient responded to desmopressin and completely recovered five days after surgery. It is probable that transient cerebral ischemia resulted in the dysfunction of osmotic receptors in the hypothalamus or hypothalamus-pituitary axis during CPB. It is also possible that cardiac standstill altered the left atrial non-osmotic receptor function and suppressed ADH release. Therefore, we suggest that central DI is a possible cause of polyuria after CPB.
Adult ; Antidiuretic Agents/therapeutic use ; Coronary Artery Bypass/*adverse effects ; Coronary Vessels ; Deamino Arginine Vasopressin/therapeutic use ; Diabetes Insipidus, Neurogenic/*diagnosis/drug therapy/etiology ; Humans ; Hypothalamus/radionuclide imaging ; Magnetic Resonance Imaging ; Male ; Pituitary Gland/radionuclide imaging ; Polyuria/diagnosis/etiology ; Postoperative Complications/*diagnosis/drug therapy/etiology