Transient antidiuretic hormone insufficiency caused by severe hyperosmolar hyperglycemic syndrome based on nephrogenic diabetes insipidus

The antidiuretic activity in the plasma of four children with nephrogenic diabetes insipidus was measured by a rat assay technique. The evidence presented to indicate that this activity was due to antidiuretic hormone (ADH) was as follows: (a) the activity was higher in jugular vein plasma than in femoral or antecubital vein plasma, (b) it was high when the children were thirsted and decreased when they drank water, (c) it was destroyed when the plasma was incubated with thioglycollate, and (d) it was ultrafilterable, and vasopressin (Pitressin), when injected, was distributed as though it was ultrafilterable. When the children were given vasopressin, there was no change in urine flow or osmolality, but plasma antidiuretic activity was higher than it was when water deprivation led to a reduction in urine flow and an increase in urine osmolality. The inference of these findings is that ADH is secreted normally in children with nephrogenic diabetes insipidus, it is ultrafilterable, but it is not a factor in modifying urine flow in response to dehydration.

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NEPHROGENIC DIABETES INSIPIDUS

PEDIATRICS ◽

10.1542/peds.15.4.424 ◽

1955 ◽

Vol 15 (4) ◽

pp. 424-432

Author(s):

James R. West ◽

James G. Kramer

Keyword(s):

Weight Gain ◽

Diabetes Insipidus ◽

Antidiuretic Hormone ◽

Clinical Features ◽

Nephrogenic Diabetes Insipidus ◽

Posterior Pituitary ◽

Maternal Grandmother ◽

Genetic Transmission ◽

Basic Defect ◽

Poor Weight Gain

1. Two cases of congenital diabetes insipidus resistant to pitressin, or diabetes insipidus of the nephrogenic type, occurring in male cousins during infancy have been described in which the most striking manifestations were recurrent pyrexia, polyuria, polydipsia, poor weight gain and development and hyperelectrolytemia. 2. The basic defect in these patients appears to be renal; in the nature of an endorgan failure to respond to the antidiuretic hormone of the posterior pituitary body. 3. The demonstration of the trait in the mothers of the patients and probably an uncle and maternal grandmother suggests genetic transmission by means other than the previously postulated sex-linked recessive pattern. 4. The literature pertinent to this condition is reviewed and some of the clinical features and diagnostic problems are discussed.

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Urinary adenosine 3',5'-monophosphate (cAMP) response to antidiuretic hormone in diabetes insipidus (DI): comparison between congenital nephrogenic DI type 1 and 2, and vasopressin sensitive DI

Acta Endocrinologica ◽

10.1530/acta.0.1080485 ◽

1985 ◽

Vol 108 (4) ◽

pp. 485-490 ◽

Cited By ~ 8

Author(s):

Takehiko Ohzeki

Keyword(s):

Urinary Excretion ◽

Diabetes Insipidus ◽

Antidiuretic Hormone ◽

Arginine Vasopressin ◽

Nephrogenic Diabetes Insipidus ◽

The Other ◽

Familial Cases ◽

Before And After

Abstract. Urinary adenosine 3',5'-monophosphate (cAMP) excretion before and after administration of aqueous vasopressin (pitressin) and 1-deamino-8-d-arginine vasopressin (DDAVP) was measured in congenital nephrogenic and in vasopressin sensitive diabetes insipidus (VS-DI). Excretion of cAMP into the urine increased markedly in response to pitressin (676%) and to DDAVP (252%) in VS-DI. Nephrogenic diabetes insipidus (N-DI) could be divided into two categories (type 1 and type 2) in respect to urinary cAMP responsiveness. In type 1, cAMP excretion showed no definite change after stimulation with pitressin (102%) or DDAVP (127%). On the other hand, urinary excretion of cAMP was significantly elevated in response to DDAVP in familial cases of N-DI type 2 (1269%) without producing any concentrating effect on the urine. Two different defects are considered to be involved in the pathogenesis of N-DI.

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A novel mutation in AVPR2 causing congenital nephrogenic diabetes insipidus with complete resistance to antidiuretic hormone

Clinical Kidney Journal ◽

10.1093/ndtplus/sfn192 ◽

2008 ◽

Vol 2 (2) ◽

pp. 127-129

Author(s):

A. Staffler ◽

M. R. Benz ◽

L. T. Weber ◽

A. Holzinger

Keyword(s):

Diabetes Insipidus ◽

Antidiuretic Hormone ◽

Nephrogenic Diabetes Insipidus ◽

Novel Mutation ◽

Congenital Nephrogenic Diabetes Insipidus ◽

Complete Resistance

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The occurrence of nephrogenic diabetes insipidus in domestic fowl

AJP Renal Physiology ◽

10.1152/ajprenal.1989.256.4.f639 ◽

1989 ◽

Vol 256 (4) ◽

pp. F639-F645 ◽

Cited By ~ 2

Author(s):

E. J. Braun ◽

J. N. Stallone

Keyword(s):

Diabetes Insipidus ◽

Antidiuretic Hormone ◽

Domestic Fowl ◽

Nephrogenic Diabetes Insipidus ◽

Water Deprivation ◽

Plasma Osmolality ◽

Urine Osmolality ◽

Arginine Vasotocin ◽

Basal Plasma ◽

Response To Water

Nephrogenic diabetes insipidus (NDI) results from an inability of the kidney to concentrate the urine. The underlying cause of NDI is the failure of the collecting ducts to respond to antidiuretic hormone, however, the specific tubular defect is not well understood. In the present investigation an apparent case of NDI was studied in a strain of White Leghorn domestic fowl. In this strain, water intake of the males equaled 24.0% (controls 5.4%) of their body mass (BM) per day while that of the females equaled 51.4% (controls 11.7%) of their BM per day. Plasma osmolality (mosmol/kgH2O) of the NDI birds was significantly higher than that of controls (males 319 +/- 1.7 vs. 311 +/- 1.2; females 323 +/- 1.5 vs. 310 +/- 2.2). Urine osmolality of NDI birds was substantially lower than that of controls (males 90 +/- 6.2 vs. 524 +/- 4.0; females 70 +/- 4.7 vs. no value). In response to water deprivation, plasma osmolality of the NDI birds increased more markedly than that of the control animals (males 357 +/- 2.5 vs. 331 +/- 1.2; females 375 +/- 6.0 vs. 348 +/- 1.4 at 48 h of water deprivation). Basal plasma antidiuretic hormone (plasma arginine vasotocin, PAVT) levels in male NDI birds (9.9 +/- 0.7 microU/ml) and in female NDI birds (7.0 +/- 0.5 microU/ml) were nearly sixfold or nearly threefold higher, respectively, than in control birds. In response to water deprivation, PAVT of both NDI and control birds increased to similar levels, although the absolute increases in PAVT levels were substantially less in NDI birds.(ABSTRACT TRUNCATED AT 250 WORDS)

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Nephrogenic Diabetes Insipidus - A Rare Report of Two Affected Sibling

Bangladesh Journal of Child Health ◽

10.3329/bjch.v41i3.36956 ◽

2018 ◽

Vol 41 (3) ◽

pp. 193-195

Author(s):

Rumana Riaaz ◽

Mahbub Mutanabbi ◽

Kohinoor Jahan Shamaly ◽

CA Kawser

Keyword(s):

Child Health ◽

Diabetes Insipidus ◽

Antidiuretic Hormone ◽

Nephrogenic Diabetes Insipidus ◽

Water Deprivation ◽

Failure To Thrive ◽

Urine Osmolality ◽

Affected Sibling ◽

Before And After ◽

Rare Report

Nephrogenic Diabetes Insipidus (NDI) is a type of Diabetes Insipidus (DI) where distal nephrons are unresponsive to antidiuretic hormone resulting in polyuria and polydipsia. NDI can be congenital or acquired. There are very few cases of congenital NDI, more in sibs. Here we report two sibs affected with congenital NDI. Both of them presented with polyuria, polydipsia and failure to thrive since early infancy. In both cases, water deprivation tests and urine osmolality were done before and after DDAVP that suggested NDI and the acquired causes has been excluded. Both of them were treated with oral Hydrochlorothiazide and improved.Bangladesh J Child Health 2017; VOL 41 (3) :193-195

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