Secondary Nephrogenic Diabetes Insipidus Research Articles

A Novel Renal Collecting Duct Model to Study Secondary Nephrogenic Diabetes Insipidus Associated with Cystinosis

A Novel Renal Collecting Duct Model to Study Secondary Nephrogenic Diabetes Insipidus Associated with Cystinosis

Gradient washout and secondary nephrogenic diabetes insipidus after brain injury in an infant: a case report

BackgroundDisorders of water and sodium balance can occur after brain injury. Prolonged polyuria resulting from central diabetes insipidus and cerebral salt wasting complicated by gradient washout and a type of secondary nephrogenic diabetes insipidus, however, has not been described previously, to the best of our knowledge. We report an unusual case of an infant with glioblastoma who, after tumor resection, was treated for concurrent central diabetes insipidus and cerebral salt wasting complicated by secondary nephrogenic diabetes insipidus.Case presentationA 5-month-old Hispanic girl was found to have a large, hemorrhagic, suprasellar glioblastoma causing obstructive hydrocephalus. Prior to mass resection, she developed central diabetes insipidus. Postoperatively, she continued to have central diabetes insipidus and concurrent cerebral salt wasting soon after. She was managed with a vasopressin infusion, sodium supplementation, fludrocortisone, and urine output replacements. Despite resolution of her other major medical issues, she remained in the pediatric intensive care unit for continual and aggressive management of water and sodium derangements. Starting on postoperative day 18, her polyuria began increasing dramatically and did not abate with increasing vasopressin. Nephrology was consulted. Her blood urea nitrogen was undetectable during this time, and it was thought that she may have developed a depletion of inner medullary urea and osmotic gradient: a “gradient washout.” Supplemental dietary protein was added to her enteral nutrition, and her fluid intake was decreased. Within 4 days, her blood urea nitrogen increased, and her vasopressin and fluid replacement requirements significantly decreased. She was transitioned soon thereafter to subcutaneous desmopressin and transferred out of the pediatric intensive care unit.ConclusionsGradient washout has not been widely reported in humans, although it has been observed in the mammalian kidneys after prolonged polyuria. Although not a problem with aquaporin protein expression or production, gradient washout causes a different type of secondary nephrogenic diabetes insipidus because the absence of a medullary gradient impairs water reabsorption. We report a case of an infant who developed complex water and sodium imbalances after brain injury. Prolonged polyuria resulting from both water and solute diuresis with low enteral protein intake was thought to cause a urea gradient washout and secondary nephrogenic diabetes insipidus. The restriction of fluid replacements and supplementation of enteral protein appeared adequate to restore the renal osmotic gradient and efficacy of vasopressin.

Polyuria: A Pathophysiologic Approach

Polyuria: A Pathophysiologic Approach

Inherited secondary nephrogenic diabetes insipidus: concentrating on humans

The study of human physiology is paramount to understanding disease and developing rational and targeted treatments. Conversely, the study of human disease can teach us a lot about physiology. Investigations into primary inherited nephrogenic diabetes insipidus (NDI) have contributed enormously to our understanding of the mechanisms of urinary concentration and identified the vasopressin receptor AVPR2, as well as the water channel aquaporin-2 (AQP2), as key players in water reabsorption in the collecting duct. Yet, there are also secondary forms of NDI, for instance as a complication of lithium treatment. The focus of this review is secondary NDI associated with inherited human diseases, such as Bartter syndrome or apparent mineralocorticoid excess. Currently, the underlying pathophysiology of this inherited secondary NDI is unclear, but there appears to be true AQP2 deficiency. To better understand the underlying mechanism(s), collaboration between clinical and experimental physiologists is essential to further investigate these observations in appropriate experimental models.

A case of Bartter syndrome type I with atypical presentations

Bartter syndrome (BS) is an autosomal recessively inherited rare renal tubular disorder characterized by hypokalemic metabolic alkalosis and hyperreninemic hyperaldosteronism with normal to low blood pressure due to a renal loss of sodium. Genetically, BS is classified into 5 subtypes according to the underlying genetic defects, and BS is clinically categorized into antenatal BS and classical BS according to onset age. BS type I is caused by loss-of-function mutations in the SLC12A1 gene and usually manifests as antenatal BS. This report concerns a male patient with compound heterozygous missense mutations on SLC12A1 (p.C436Y and p.L560P) and atypical clinical and laboratory features. The patient had low urinary sodium and chloride levels without definite metabolic alkalosis until the age of 32 months, which led to confusion between BS and nephrogenic diabetes insipidus (NDI). In addition, the clinical onset of the patient was far beyond the neonatal period. Genetic study eventually led to the diagnosis of BS type I. The low urinary sodium and chloride concentrations may be caused by secondary NDI, and the later onset may suggest the existence of a genotype-phenotype correlation.In summary, BS type I may have phenotype variability including low urine sodium and chloride levels and later onset. A definitive diagnosis can be confirmed by genetic testing.

A Long Way to Become ‘Dry’: A Case of an Eleven-Year-Old Boy Born with an Obstructive Ureterocele, Reflux and Secondary Nephrogenic Diabetes Insipidus

PURPOSE To give an overview of the multidisciplinary approach in the treatment, the definite solution and the specific problems which developed in this specific case. MATERIAL AND METHODS Retrospective analysis of the medical, nursing, physiotherapheutic and psychological history of this specific case as an example of case management by a pediatric urology nurse practitioner within a multidisciplinary team. RESULTS All different aspects of care problems of an eleven-year-old boy born with an obstructive ureterocele, reflux and secondary nephrogenic diabetes insipidus are reviewed and presented. In his first year he underwent right nephrectomy of an afunctional kidney due to reflux- nephropathy. Because of the obstructive ureterocèle he received a temporary ureterocutaneostomy on the left side. He has polyuria and mild renal insufficiency. At age 5 treatment for his incontinence was started. Many different treatment options with different success and outcome were applied. The specific problems and the definite solution (Bricker urostomy) will be presented and discussed. Also, a special focus will be given to the multidisciplinary approach, discussions with the child and parents, and different aspect from the patients point of view. CONCLUSIONS A multidisciplinary approach with extensive discussions and explanation of expected outcome as well as additional psychological, nursing and physiotherapeutic support for children and parents can be necessary in difficult cases, so assure that chosen solutions are accepted by the child and parents as their best option.

Water Transport in the Kidney and Nephrogenic Diabetes Insipidus

Nephrogenic diabetes insipidus is caused by an inability of the kidney to concentrate urine despite adequate concentration of vasopressin in blood and is characterized by polyuria, polydipsia, and hyposthenuria in the presence of plasma hyperosmolality. Nephrogenic diabetes insipidus is the result of defects in water homeostasis in the kidney. Nephrogenic diabetes insipidus occurs when the kidneys cannot or do not respond to vasopressin. There are 2 categories of nephrogenic diabetes insipidus. Congenital nephrogenic diabetes insipidus is a rare, inherited, irreversible cause of polyuria and polydipsia in humans that is even rarer in animals. Acquired nephrogenic diabetes insipidus is more common and is often secondary to illness or medication that interferes with the action of vasopressin in the renal tubules. Unlike congenital nephrogenic diabetes insipidus, acquired or secondary nephrogenic diabetes insipidus is often reversible with correction of the associated or causative problem.

Water transport in the kidney and nephrogenic diabetes insipidus.

Nephrogenic diabetes insipidus is caused by an inability of the kidney to concentrate urine despite adequate concentration of vasopressin in blood and is characterized by polyuria, polydipsia, and hyposthenuria in the presence of plasma hyperosmolality. Nephrogenic diabetes insipidus is the result of defects in water homeostasis in the kidney. Nephrogenic diabetes insipidus occurs when the kidneys cannot or do not respond to vasopressin. There are 2 categories of nephrogenic diabetes insipidus. Congenital nephrogenic diabetes insipidus is a rare, inherited, irreversible cause of polyuria and polydipsia in humans that is even rarer in animals. Acquired nephrogenic diabetes insipidus is more common and is often secondary to illness or medication that interferes with the action of vasopressin in the renal tubules. Unlike congenital nephrogenic diabetes insipidus, acquired or secondary nephrogenic diabetes insipidus is often reversible with correction of the associated or causative problem.

Traitement du diabète insipide en 1999

Most cases of acquired central diabetes insipidus are caused by destruction of the neurohypophysis by: 1) anatomic lesions that destroy the vasopressin neurons by pressure or infiltration, 2) damage to the vasopressin neurons by surgery or head trauma, and 3) autoimmune destruction of the vasopressin neurons. Because the vasopressin neurons are located in the hypothalamus, lesions confined to the sella turcica generally do not cause diabetes insipidus because the posterior pituitary is simply the site of the axon terminals that secrete vasopressin into the bloodstream. In addition, the capacity of the neurohypophysis to synthesize vasopressin is greatly in excess of the body's needs, and destruction of 80–90% of the hypothalamic vasopressin neurons is required to produce diabetes insipidus. As a result, even large lesions in the sellar and suprasellar area generally are not associated with impaired water homeostasis until they are surgically resected. Regardless of the etiology of central diabetes insipidus, deficient or absent vasopressin secretion causes impaired urine concentration with resultant polyuria. In most cases, secondary polydipsia is able to maintain water homeostasis at the expense of frequent thirst and drinking. However, destruction of the osmoreceptors in the anterior hypothalamus that regulate vasopressin neuronal activity causes a loss of thirst as well as vasopressin section, leading to severe chronic dehydration and hyperosmolality. Vasopressin deficiency also leads to down-regulation of the synthesis of aquaporin-2 water channels in the kidney collecting duct principal cells, causing a secondary nephrogenic diabetes insipidus. As a result, several days of vasopressin administration are required to achieve maximal urine concentration in patients with CDI. Consequently, the presentation of patients with central diabetes insipidus can vary greatly, depending on the size and location of the lesion, the magnitude of trauma to the neurohypophysis, the degree of destruction of the vasopressin neurons, and the presence of other hormonal deficits from damage to the anterior pituitary.

Urinary arginine vasopressin (AVP) measurement in children: Water deprivation test incorporating urinary AVP

The value of a water deprivation test incorporating urinary arginine vasopressin (AVP) measurement was investigated in 13 patients with polydipsia and/or polyuria (complete central diabetes insipidus [CCDI] in four; incomplete central diabetes insipidus [ICDI] in five; secondary nephrogenic diabetes insipidus [NDI] in three; compulsive water drinking [CWD] in one) and a group of 25 control subjects (C). Urine samples were collected after water deprivation during sleep and the urinary osmolalities and AVP concentrations were measured. Analysis of the results of 104 urine samples from the 25 control subjects demonstrated a close correlation between urinary osmolality and AVP (r = 0.89, P < 0.001). After water deprivation during sleep, the respective mean maximal urinary osmolalities and AVP concentrations were: 127.4 +/- 34.4 mOsm/kg and 1.1 +/- 0.5 pg/mL in the patients with CCDI (14 samples, four children); 410.3 +/- 101.8 and 6.1 +/- 3.5 in those with ICDI (16 samples, five children); 348.7 +/- 71.2 and 100 +/- 45.1 in those with NDI (nine samples, three children); 541.5 +/- 143.5 and 43.6 +/- 33.2 in the patient with CWD (two samples, one child) and 898.8 +/- 186.3 and 97.4 +/- 50.4 in group C (54 samples, 18 children). Furthermore, the urinary AVP level relative to the osmolality in each patient varied depending on the AVP secretion status and renal concentrating ability. Each patient, except the one with CWD, could be discriminated from the normal subjects using this test. It seems that this test is easy to perform and useful for diagnosis and follow-up of patients with partial/complete posterior pituitary function defects and those with renal concentration impairment.