deamino-arginine-vasopressin and Hydronephrosis

Topics: Antidiuretic Agents; Child; Chromosomes, Human, Pair 4; Deamino Arginine Vasopressin; Diabetes Insipidus; Family Health; Female; Hearing Loss, Sensorineural; Homozygote; Humans; Hydronephrosis; Membrane Proteins; Mothers; Mutation; Treatment Outcome; Uniparental Disomy; Urinary Bladder, Neurogenic; Wolfram Syndrome

Pyelectasis can be defined as mild to moderate dilatation of the urinary tract and is diagnosed by means of an ultrasound scan (0.5-2cm transverse diameter in the initial ultrasound performed after birth). There is some disagreement about whether cystography should be indicated as standard practice. The aim of this study was to establish if renal function tests are useful in determining which cases of mild to moderate dilatation of the urinary tract do not require an initial cystography.. The study was conducted on 79 infants (57 males, 22 females) with pyelectasis. Seventy-three were diagnosed in utero and 6 after birth. All infants underwent at least one cystography and one desmopressin urine concentration test before one year of age.. Compared to infants without vesicoureteral reflux (VUR) (n=68), infants with VUR (n=11; two with Grade I, three with Grade II, five with Grade III, two with Grade IV) showed a significantly lower (P=.006) maximum urine osmolality and a significantly higher microalbumin/creatinine ratio (P<.001) and NAG/creatinine ratio (P=.003). The negative predictive value of the first two tests was 93%. Sensitivity of the maximum urine osmolality to detect VUR was 72.7% (specificity 63.2%). Sensitivity of the microalbumin/creatinine ratio to detect VUR was 62.5% (specificity 75%). The positive probability ratio (PR) was 1.29 for the NAG/creatinine ratio, 2.03 for the maximum urine osmolality and 2.5 for the microalbumin/creatinine ratio. The negative PR was 0.95 for the NAG/creatinine ratio, 0.43 for the maximum urine osmolality and 0.5 for the microalbumin/creatinine ratio.. Pyelectasis is a benign condition. Only 2 patients required pharmacological intervention (prophylactic treatment for VUR Grade IV patients). Initially at least, cystography should not be indicated in cases of microalbuminuria and/or normal urine concentrations.

Topics: Acetylglucosaminidase; Albuminuria; Breast Feeding; Creatinine; Cross-Sectional Studies; Deamino Arginine Vasopressin; Early Diagnosis; Female; Humans; Hydronephrosis; Infant; Infant, Newborn; Kidney Function Tests; Male; Osmolar Concentration; Predictive Value of Tests; Prospective Studies; Pyelectasis; Radiography; Sensitivity and Specificity; Severity of Illness Index; Ultrasonography, Prenatal; Urinalysis; Urinary Bladder; Urinary Tract; Vesico-Ureteral Reflux

We report a case of pituitary dwarfism and diabetes insipidus due to pituitary stalk transection in a pregnant Japanese woman, 138 cm in height, born by breech delivery with no evidence of ante- or intrapartum asphyxia. The patient had no central nervous disturbance, was diagnosed with pituitary dwarfism during childhood and was treated at another hospital with growth hormone supplement from 5 to 14 years of age. This patient was referred to our department at 17 weeks' gestation due to a change of residence. At 30 weeks' gestation, she was hospitalized for assessment of hydronephrosis and polyuria (15-20 L/day). Analysis of a 24-h urine sample showed creatinine clearance of 157 mL/min and urine osmolality of 38 mOsm/L. The patient's urine output decreased after receiving a test dose of 0.75 g of 1-desamino-8-D-arginine vasopressin (DDAVP). Cranial magnetic resonance imaging showed transection of the pituitary stalk. Subsequently, the patient's urine output was well controlled by a maintenance dose of 0.275 mL/day intranasal DDAVP. A cesarean section was performed at 37 weeks, as the patient height was 138 cm, and a pelvic X-ray showed cephalopelvic disproportion. She delivered a female baby weighing 2302 g, and both 1- and 5-min Apgar scores were 9. The patient was followed up after 4 months and showed no visual deterioration or polyuria while on DDAVP therapy, while the neonate grew favorably.

Topics: Adult; Breech Presentation; Deamino Arginine Vasopressin; Diabetes Insipidus; Dwarfism, Pituitary; Female; Human Growth Hormone; Humans; Hydronephrosis; Magnetic Resonance Imaging; Pituitary Gland; Pituitary Hormones; Polyuria; Pregnancy; Pregnancy Complications; Pregnancy Outcome

Topics: Adult; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Hydronephrosis; Male; Polyuria; Renal Agents; Treatment Outcome

We describe a case of a novel mutant vasopressin 2 receptor (V2R)-dependent nephrogenic diabetes insipidus (NDI) with bilateral non-obstructive hydronephrosis in a middle aged man. This could be distinguished from aquaporin 2 (AQP2)-dependent NDI by the response of factor VIII and von Willebrand factor (vWF) to 1-deamino-8-D-arginine vasopressin (DDAVP) administration. A 47-year-old man was admitted to hospital because of polyuria, which had been present from infancy and was suspected of causing non-obstructive hydronephrosis. His mother's father, the older brother of his mother and his second daughter also all had polyuria. Sodium concentration, osmolality and vasopressin in blood were high, while sodium concentration and osmolality in urine were low. There were no changes in urine osmolality, factor VIII and vWF in response to DDAVP infusion. Neither was heart rate, diastolic blood pressure nor facial flushing affected. These findings suggested this case was V2R-dependent NDI rather than AQP2-dependent NDI. Molecular genetic analysis demonstrated that the patient had a V2R missense mutation involving a substitution of cysteine for arginine at position 104 (R104C) located in the first extracellular loop of the V2R. It was also found that the patient's mother and his second daughter were heterozygous for this R104C mutation.

Topics: Aquaporin 2; Aquaporins; Arginine; Base Sequence; Cysteine; Deamino Arginine Vasopressin; Diabetes Insipidus, Nephrogenic; Diagnosis, Differential; Factor VIII; Humans; Hydronephrosis; Male; Middle Aged; Mutation, Missense; Pedigree; Protein Isoforms; Receptors, Vasopressin; Renal Agents; von Willebrand Factor

Facilitated urea transport has been demonstrated in several mammalian tissues, including those of the collecting ducts and red blood cells. Two urea transporters have been recently cloned: UT2, expressed in rabbit inner medullary collecting ducts, and HUT11, expressed in human erythrocytes. Because of significant identity (63%) between these two transporters, and because HUT11 is also expressed in the human kidney, they could represent the same transporter with species-related differences in their-sequences. In the study presented here, two different cDNA fragments, corresponding to the rat equivalents (rUT2 and rUT11) of the two previously cloned urea transporters, were isolated by reverse transcription-polymerase chain reaction. These rat probes were used for Northern analysis of RNA extracted from rat tissues. From the following findings, the results show that rUT2 and rUT11 are two distinct urea transporters: (1) The two cDNA fragments isolated in the rat exhibit different sequences; (2) The mRNA for rUT2 is found exclusively in the kidney, with two transcripts (3.2- and 4.4-kilobase (kb)), whereas rUT11 (only one transcript, 4.2 kb) is present in the brain, spleen, kidney, and testis; (3) in the kidney, the inner stripe of the outer medulla expresses rUT11 mRNA and the short transcript of rUT2, whereas the inner medulla expresses rUT11 and the two rUT2 transcripts; (4) In hydronephrotic kidneys that have completely lost their tubular epithelium but have intact vasculature, rUT2 transcripts are no longer expressed, whereas expression of rUT11 is intensified; (5) Experimental chronic alterations in urine concentrating activity induced different changes in the expression of rUT2 and rUT11.

Topics: Amino Acid Sequence; Animals; Blood Vessels; Carrier Proteins; Deamino Arginine Vasopressin; Drinking; Humans; Hydronephrosis; Kidney; Kidney Concentrating Ability; Kidney Tubules; Male; Membrane Glycoproteins; Membrane Transport Proteins; Molecular Sequence Data; Rabbits; Rats; Rats, Sprague-Dawley; Tissue Distribution; Urea Transporters

A 46-year-old man who had a history of hypogonadism, bilateral hydronephrosis and huge residual urine volume during the past ten years was admitted complaining of fever and flank pain. Polyuria which was more than 4 liters per day and inability of urine concentration suggested diabetes insipidus. Magnetic resonance imaging (MRI) demonstrated a tumor which was compatible with craniopharyngioma. Tumor resection and administration of desmopressin improved polyuria and urinary tract dilatation with marked reduction of residual urine volume from 400 ml to 20 ml.

Topics: Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Dilatation, Pathologic; Humans; Hydronephrosis; Male; Middle Aged; Pituitary Neoplasms; Renal Agents; Urinary Tract

A case of familial central diabetes insipidus and dilatation of the urinary tract is reported. Administration of desmopressin for 1 year improved urinary tract dilatation with a concomitant reduction in urine volume. Urinary cyclic adenosine monophosphate and prostaglandin E2 excretion increased after treatment.

Topics: 6-Ketoprostaglandin F1 alpha; Adult; Cyclic AMP; Deamino Arginine Vasopressin; Diabetes Insipidus; Dinoprost; Dinoprostone; Humans; Hydronephrosis; Male; Thromboxane B2; Urine

In order to determine the renal concentration capacity in neonatal hydronephrosis, 10 micrograms of DDAVP were administered intranasally to 18 infants with hydronephrosis. Fluid intake was restricted to 50% of normal for 3 hours before and 6 hours after the administration of DDAVP. Maximal urine osmolality (mean +/- SD) was 348 +/- 180 mOsm/kg in 7 newborns younger than 21 days and 420 +/- mOsm/kg in 11 neonates between 22-50 days of age. Both osmolarities were inferior to the standard response to DDAVP reported in normal neonates. After 24 hours of clinical observation, we did not notice any secondary effects caused by this test.

Topics: Deamino Arginine Vasopressin; Female; Humans; Hydronephrosis; Infant, Newborn; Kidney Concentrating Ability; Male; Osmolar Concentration; Urine

Male Brattleboro rats with hereditary diabetes insipidus (BDI) were lifetime-treated with the vasopressin V2 receptor agonist desamino-8D-arginine vasopressin (DDAVP), given daily in the drinking fluid. The DDAVP-treated adult male BDI rats drank 34 +/- 6 ml/24 h (mean +/- s.e.m.) and excreted urine volumes of 22 +/- 5 ml/24 h compared with their age-matched untreated controls of 142 +/- 12 and 115 +/- 7 ml/24 h respectively. There was no significant difference between the mean body weights of chronically DDAVP-treated BDI rats (198 +/- 9 g) and untreated animals (207 +/- 9 g). Morphometry of sections of kidney confirmed extensive hydronephrosis in the right kidneys of the control untreated Brattleboro rats only. This was quantified as the area of pelvis expressed as a percentage of total cross-sectional area of kidney (17 +/- 3 compared with 5 +/- 1% in the chronically DDAVP-treated rats; P less than 0.002). Medium-term treatment of adult BDI rats with DDAVP reduced daily fluid output towards normal rat values but hydronephrosis was still present. These observations indicate that the restoration of fluid balance in adult BDI rats by treatment from conception with DDAVP may be an important factor in preventing the development of hydronephrosis in these animals.

Topics: Animals; Biometry; Deamino Arginine Vasopressin; Female; Hydronephrosis; Kidney; Kidney Concentrating Ability; Kidney Pelvis; Male; Rats; Rats, Brattleboro

Nitrofen and ethylenethiourea (ETU), agents known to prenatally induce hydronephrosis in rats, were assessed for their effects on postnatal renal functional maturation. Both were given by gavage to pregnant Sprague-Dawley rats on Gestation Day 11. Nitrofen was given at concentrations of 50 or 100 mg/kg, and ETU at 20, 40, or 60 mg/kg. Renal function was examined in the offspring from birth until after weaning, the period of renal functional maturation in the rat. Maximal urine concentrating ability was measured after DDAVP (desmopressin acetate, a vasopressin analog) challenge or water deprivation. Proximal tubule transport was measured in renal cortical slices. Various urinary parameters were measured. Both prenatal nitrofen and ETU exposure caused a large number of neonatal deaths at the high dose, and hydronephrosis was observed. The severity of the lesion increased with age. Hydronephrotic animals were deficient in urine concentrating ability, which became more pronounced after weaning. A few other urinary parameters were altered, but cortical function appeared to be unaffected. Rats prenatally exposed to nitrofen, but with apparently normal kidneys, were significantly compromised in their ability to produce a concentrated urine in response to DDAVP challenge, on Postnatal Days (PDs) 6 and 14. By PD 30, they were not different from controls in urine concentrating response. Rats prenatally exposed to the higher doses of ETU, but with grossly normal kidneys, had significantly decreased plasma clearances of certain electrolytes early in life, but by PD 27, they were not different from controls. Proximal tubule transport of PAH was increased on PD 7 in ETU-exposed pups, but this effect did not persist.

Topics: Animals; Animals, Newborn; Deamino Arginine Vasopressin; Ethylenethiourea; Female; Gestational Age; Herbicides; Hydronephrosis; Imidazoles; Kidney Concentrating Ability; Kidney Diseases; Kidney Function Tests; Kidney Tubules, Proximal; Phenyl Ethers; Pregnancy; Rats; Rats, Inbred Strains; Sodium; Teratogens

Renal function of 18 infants who had undergone surgery in the neonatal period because of severe congenital hydronephrosis was followed up for 5 to 36 months (mean +/- SD 21 +/- 10 months). In all cases the diagnosis was made prenatally by sonography and confirmed at birth by intravenous urography. Creatinine clearance developed normally in all the children. Eight had a reduction in maximal urinary concentrating ability after intranasal DDAVP; this defect was transient and resolved after 4 to 5 months in all but one child, in whom it persisted. However, other tubular abnormalities were present. Throughout the observation period, patient serum potassium concentrations were significantly higher than normal, paralleled by a significant increase in plasma aldosterone concentration but with normal excretion fraction of sodium and potassium. There were no disturbances of acid-base balance. These findings may be accounted for by a persistent partial reduced sensitivity of the distal tubule to the action of aldosterone despite normal renal function. This alteration is usually mild, but may constitute a persistent metabolic risk despite successful surgical intervention.

Topics: Aldosterone; Child, Preschool; Deamino Arginine Vasopressin; Drug Resistance; Follow-Up Studies; Humans; Hydronephrosis; Infant; Infant, Newborn; Kidney Concentrating Ability; Kidney Tubules; Male; Potassium; Renin

The systemic and renal effects of high partial ureteral obstruction were investigated in a new model of experimental hydronephrosis. The test group comprised 12 contralaterally nephrectomized growing male New Zealand rabbits. As compared to the pyelographic findings in 6 unilaterally nephrectomized control animals, the test group could be divided into partially obstructed but non-hydronephrotic and obstructed-hydronephrotic subgroups. Animals of all 3 groups were capable of increasing their weight during the first 2 postoperative months. The mean plasma creatinine concentration remained normal in the obstructed group and even hydronephrosis was compatible with a normal serum creatinine level. As studied during forced hypotonic expansion, the renal response to a vasopressin analogue was significantly different in all 3 animal groups. Reciprocal but less marked differences were noted in the animals' ability to retain water during this test. We conclude that in this experimental model the magnitude of the antidiuretic response is inversely related to the radiologically defined degree of obstruction.

Topics: Animals; Deamino Arginine Vasopressin; Disease Models, Animal; Diuresis; Hydronephrosis; Kidney; Kidney Concentrating Ability; Male; Nephrectomy; Osmolar Concentration; Rabbits; Radiography; Ureteral Obstruction

A ten year old boy with hereditary pituitary diabetes insipidus presented with massive bilateral hydronephrosis, hydroureters and an extremely large bladder. Radiological investigations excluded a mechanical obstruction or vesicoureteral reflux. Treatment with the adiuretin analog DDAVP resulted in regression of the urinary tract changes after 5 months and an almost complete disappearance after 3 1/2 years. The urinary tract dilatation probably results from the large urine flows which exceed the capacity of the urinary tract causing a functional obstruction and residual urine.

Topics: Arginine Vasopressin; Child; Deamino Arginine Vasopressin; Diabetes Insipidus; Humans; Hydronephrosis; Male

Three chemicals, known either to alter renal development when administered during fetal development or to affect renal function when administered to adult rats, were administered to Sprague-Dawley rats at critical periods of renal development. Chlorambucil (CHL) was administered ip on d 11 of gestation at doses of 0, 3, and 6 mg/kg; nitrofen (2,4-dichlorophenyl p-nitrophenyl ether) (NIT) was given po on d 8-16 of gestation at 0, 4.17, 12.5, and 25 mg/kg . d; and mercuric chloride (MER) was given sc on postnatal d 1 at 0, 14, and 28 micrograms/pup. To assess the effects of these toxicants on the functional development of the kidneys, a diuresis test with and without antidiuretic hormone was applied on postnatal d 3 (PD 3); a hydropenia test on PD 6; and kidney weights, glomerular counts in midhilar cross sections, and the specific activity of renal alkaline phosphatase were determined on PD 3 and 6. Data from pups with obvious malformations of the kidneys was eliminated from the statistical analyses of the data so that emphasis could be placed on alterations of functional development in individuals with apparently morphologically normal kidneys. CHL retarded the growth and biochemical differentiation of the kidney at 6 mg/kg. Pups from this treatment groups showed an attenuated response to exogenously administered antidiuretic hormone. NIT impaired growth and altered renal morphology at a dose of 12.5 mg/kg . d and altered physiological responses in the absence of anatomical changes at a dose of 4.17 mg/kg . d. MER, at doses near the maximum tolerated, failed to alter any parameter, indicating that the very young animal differs markedly from the adult in response to that compound. The data indicate that relatively simple tests of renal function are useful in the detection of perinatally induced nephrotoxicity.

Topics: Age Factors; Animals; Animals, Newborn; Chlorambucil; Deamino Arginine Vasopressin; Diuresis; Dogs; Dose-Response Relationship, Drug; Female; Hydronephrosis; Kidney; Kidney Diseases; Male; Mercuric Chloride; Mercury; Phenyl Ethers; Pregnancy; Rabbits; Rats; Rats, Inbred Strains

Topics: Animals; Animals, Newborn; Deamino Arginine Vasopressin; Diuresis; Female; Gestational Age; Herbicides; Hydronephrosis; Kidney Concentrating Ability; Kidney Diseases; Kidney Function Tests; Male; Nitrogen; Potassium; Pregnancy; Prenatal Exposure Delayed Effects; Rats; Rats, Inbred Strains; Water Deprivation

Two cases of diabetes insipidus (hypothalamic and nephrogenic) with massive nonobstructive trabeculation and dilation of the bladder and hydroureteronephrosis are reported. The cases are evaluated thoroughly--radiologically and urodynamically. Treatment options are discussed, including the use of an important new drug, dDAVP. The general subject of diabetes insipidus and its urologic implications is reviewed.

Topics: Adult; Child; Deamino Arginine Vasopressin; Diabetes Insipidus; Dilatation, Pathologic; Humans; Hydronephrosis; Male; Ureteral Diseases; Urinary Bladder Diseases; Urologic Diseases