• Corpus ID: 23758595

Physiology in Medicine: a Series of Articles Linking Medicine with Science Physiology in Medicine: Nephrogenic Diabetes Insipidus Web-only Cme Quiz Conversion of Figures into Slides

@inproceedings{AusielloPhysiologyIM,  title={Physiology in Medicine: a Series of Articles Linking Medicine with Science Physiology in Medicine: Nephrogenic Diabetes Insipidus Web-only Cme Quiz Conversion of Figures into Slides},  author={Dennis A. Ausiello and Dale J. Benos and Francois Abboud and William J Koopman and Paul Epstein and Jeff M Sands and Daniel G Bichet},  url={https://api.semanticscholar.org/CorpusID:23758595}}
Patients with congenital nephrogenic diabetes insipidus now live well into adulthood and need to be followed by internists to avoid bladder dysfunction, renal dysfunction, and complications related to inaccessibility to water.
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GENETICS IN ENDOCRINOLOGY Pathophysiology, diagnosis and treatment of familial nephrogenic diabetes insipidus.

The purpose of this review is to describe classical phenotype findings that will help physicians to identify early, before dehydration episodes with hypernatremia, patients with familial NDI.

Central and nephrogenic diabetes insipidus: updates on diagnosis and management

A stepwise approach to recognition, diagnosis, and treatment of diabetes insipidus is described, which may simplify and improve the accuracy in diagnosing DI in the future.

Review Article Diabetes insipidus: the basic and clinical review

The best management for primary polydipsia is fluid restriction while fluid intake is used for adipsic diabetes insipidus, and ADH replacement therapy is widely used to treat DI.

Nephrogenic diabetes insipidus: a comprehensive overview

The treatment of NDI consists in decreasing the urine output, while allowing appropriate fluid balance, normonatremia, and ensuring an acceptable quality of life.

Nephrogenic Diabetes Insipidus is a Rare Complication of Chronic Kidney Disease - A Case Report

A 4-year old boy was admitted in the Department of Paediatric Nephrology, Bangabandhu Sheikh Mujib Medical University with the complaints of failure to thrive, refusal to feed, nausea and vomiting and for the last six months, he had also complaints of polyuria and polydipsia.

Hereditary Nephrogenic Diabetes Insipidus: Molecular Basis of the Defect and Potential Novel Strategies for Treatment

An overview on the most recent strategies under investigation for rescuing the mutated gene products activity or for bypassing defective vasopressin receptor signaling is provided.

Diabetes Insipidus: Pathogenesis, Diagnosis, and Clinical Management

Treatment of DI is dependent on the disease classification, but severe complications may arise if not tended to appropriately, and central and nephrogenic subtypes of DI share a paradoxical treatment in thiazide diuretics.
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32 References

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The hypokalemic salt-losing tubulopathy with chronic renal failure and sensorineural deafness represents not only genetically but also clinically a disease entity distinct from hyperprostaglandin E syndrome/antenatal Bartter syndrome.

A comparison of plasma vasopressin measurements with a standard indirect test in the differential diagnosis of polyuria.

It is concluded that the incorporation of a vasopressin assay improves accuracy in the differential diagnosis of polyuria and may be explained by increased sensitivity to low concentrations of vasoppressin in the former disorder and a reduction of maximal concentrating ability in both.

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In patients with HPS/aBS, excessive PGE2 synthesis and hyperreninemia is dependent on COX-2 activity, and the stimulatory role of COx-2 on renin-secretion in salt-depletion in humans is proved.

Cognitive and psychosocial functioning of patients with congenital nephrogenic diabetes insipidus.

The current prevalence of MR among patients with this disease is considerably lower than suggested in literature, with the exception of a negative correlation between age at start of therapy and verbal IQ in one age group.

Decreased aquaporin-2 expression and apical plasma membrane delivery in kidney collecting ducts of polyuric hypercalcemic rats.

The results strongly suggest that AQP2 downregulation and reduced apical plasma membrane delivery of AQP1 play important roles in the development of polyuria in association with hypercalcemia.

Urinary concentrating ability in patients with Jk(a-b-) blood type who lack carrier-mediated urea transport.

The studies raise the possibility that the erythrocyte urea transporter and the kidney Urea transporter are encoded by a single gene and that a lack of facilitated urea transport impairs urea recycling in the kidney and, hence, maximal urinary concentrating ability.

Three families with autosomal dominant nephrogenic diabetes insipidus caused by aquaporin-2 mutations in the C-terminus.

The results suggest that the trafficking of mutant AQP2 is impaired because of elongation of the C-terminal tail, and the dominant-negative effect is attributed to oligomerization of the wild-type and mutant AQp2s.

Water channels encoded by mutant aquaporin-2 genes in nephrogenic diabetes insipidus are impaired in their cellular routing.

In Xenopus oocytes the inability of Gly64-Arg, Arg187Cys or Ser216Pro substituted AQP-2 proteins to facilitate water transport is caused by an impaired routing to the plasma membrane.

Early polyuria and urinary concentrating defect in potassium deprivation.

The studies suggest that the very early urinary concentrate defect in KD results primarily from the suppression of cortical AQP2, whereas the later onset of a urinary concentrating defect (after 24 h) also involves a downregulation of medullary AQP 2.

Altered expression of urea transporters in response to ureteral obstruction.

Reduced expression of UT-A1,UT-A3, and UT-B levels in both BUO and UUO rats suggests that urea transporters play important roles in the impaired urinary concentrating capacity in response to urinary tract obstruction.

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