No abstract available.
Diabetes Insipidus, Nephrogenic*

No abstract available.
Diabetes Insipidus, Nephrogenic* ; Humans ; Siblings*

No abstract available.
Diabetes Insipidus, Nephrogenic* ; Urinary Bladder*

No abstract available.
Diabetes Insipidus, Nephrogenic* ; Urinary Tract Infections ; Vesico-Ureteral Reflux*

We describe a case of congenital nephrogenic diabetes insipidus with severe dilatation of bilateral urinary tracts without anatomical obstructions. Functional obstruction can be occurred when polyuria surpasses the transporting ability of urine in the urinary tract. The patient was admitted to our hospital due to decreased mentality developed after traffic accident. On radiologic study, bilateral hydronephrosis and hydroureter were noted. Because the patient excreted copious dilute urine, we performed water deprivation test and the result was consistent with nephrogenic diabetes insipidus. We are presenting this case in an attempt to describe strong association between congenital diabetes insipidus and nonobstructive hydronephrosis in which polyuria is responsible for the hydronephrosis.
Accidents, Traffic ; Diabetes Insipidus ; Diabetes Insipidus, Nephrogenic* ; Dilatation ; Humans ; Hydronephrosis* ; Polyuria ; Urinary Tract ; Water Deprivation

Nephrogenic diabetes insipidus(DI) is characterized by insensitivity of the distal nephron to vasopressin(ADH) and the inability to concentrate urine, which leads to excreting excessive quantities of urine. Upper tract dilation secondary to polyuria was previously shown to be associated with nephrogenic DI. We report here on 3 males with nephrogenic DI that caused voiding difficulty as well as massive nonobstructive dilation of the urinary tract.
Diabetes Insipidus ; Diabetes Insipidus, Nephrogenic ; Humans ; Male ; Nephrons ; Polyuria ; Urinary Tract

A 13 year old boy was diagnosed as primary nephrotic diabetesinsipidus, whose symptom has been devloped from 3 years of age, subsequetly he developed bilateral hydronephrosis and neurogenic bladder. His pedigree could be explored 5 generations and represented inheritace as X-linked recesslive transmission, He was treated with indomethacin 2mg/Kg/day plus chlorothiazid 500mg/dau and this new method shows markedly decreased urine outpur and increased urine osmolarity.
Adolescent ; Diabetes Insipidus, Nephrogenic* ; Family Characteristics ; Humans ; Hydronephrosis ; Indomethacin ; Male ; Osmolar Concentration ; Pedigree ; Urinary Bladder, Neurogenic

The most common form of genetic nephrogenic diabetes insipidus(NDI), a rare inherited disorder, is congenital and is transmitted in an X-linked recessive mode. It is refractory to the antidiuretic effect of normal to moderately increased levels of plasma arginine vasopressin(AVP) but, in some cases, may respond to high levels of the hormone or its analogue, deamino-D-arginine vasopressin(DDAVP). X-linked congenital NDI has now been linked to over 128 different mutations in diverse coding regions of the AVP receptor 2(AVPR2) gene. The functional effects of these mutations vary from complete loss of responsiveness to a simple shift to the right in the dose response curve. We report a case of congenital partial NDI, with transversion of A to G at codon 280 of the AVPR2 gene, resulting in a subsequent change of amino acid from tyrosine to cysteine, and that has been effective with hydrochlorothiazide and high dose of DDAVP.
Antidiuretic Agents ; Arginine ; Clinical Coding ; Codon ; Cysteine ; Deamino Arginine Vasopressin ; Diabetes Insipidus, Nephrogenic* ; Hydrochlorothiazide ; Plasma ; Tyrosine

Nephrogenic diabetes insipidus is a rare hereditary disorder characterized by insensitivity of the renal tubule to vasopressin. We report a case of nephrogenic diabetes insipidus associated with severe hydronephrosis in a 18 year old male, which was improved in urine volume, urine osmolarity and urine specific gravity with chlorothiazide therapy.
Adolescent ; Chlorothiazide ; Diabetes Insipidus, Nephrogenic* ; Humans ; Hydronephrosis* ; Male ; Osmolar Concentration ; Specific Gravity ; Vasopressins

Nephrogenic diabetes insipidus is a rare hereditary disorder characterized by insensitivity of the renal tubule to vasopressin. We report a case of nephrogenic diabetes insipidus associated with severe hydronephrosis in a 18 year old male, which was improved in urine volume, urine osmolarity and urine specific gravity with chlorothiazide therapy.
Adolescent ; Chlorothiazide ; Diabetes Insipidus, Nephrogenic* ; Humans ; Hydronephrosis* ; Male ; Osmolar Concentration ; Specific Gravity ; Vasopressins