Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035078 (renal failure)
 31,970 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifty-nine patients who had undergone augmentation cystoplasty were studied over a period of 18 years. The indications for the operation were a tuberculous contracted bladder in 51, interstitial cystitis in 7 and carcinoma in 1. The ileum was used in one patient, the colon in 16 and the caecum in 42. The results did not seem to be influenced by the segment of bowel and the long-term results of using the colon as bladder substitute were similar to those achieved by using the caecum. An excellent result has been achieved in 78% of the 49 surviving patients. Operative mortality was 5.1% (3 patients). Contraindications include progressive severe renal failure, enuresis and a history of previous psychiatric disturbance.
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PMID:Bladder augmentation--a long-term review. 46 1

We describe a brother and sister with amelogenesis imperfecta, nephrocalcinosis and impaired renal concentrating ability. This is the second sibship reported, further substantiating autosomal recessive inheritance of this condition. There is lack of enamel, lifelong nocturnal enuresis, progressive punctate nephrocalcinosis, and decreased calcium and phosphate excretion over 24 hours and after an acute load. Increased serum osteocalcin and decreased urine delta-carboxyglutamic acid suggest involvement of vitamin K-dependent calcium binding proteins, although this may represent a secondary finding. No other evidence of abnormal calcium metabolism was found. Renal function is stable in the early teens, but the previously reported patients went on to renal failure.
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PMID:Syndrome of amelogenesis imperfecta, nephrocalcinosis, impaired renal concentration, and possible abnormality of calcium metabolism. 387 71

The management of 108 patients with posterior urethral valves from 7 pediatric surgical centers has been reviewed. All the patients have been treated in the 10 year period since 1970. 53 were under 3 mo of age at presentation (Group I), 8 between 3 mo and 1 yr (Group II), 22 between 1 and 5 yr (Group III), and 25 over 5 yr of age (Group IV). Under 5 yr of age urinary infection (37) and renal failure (22) were the commonest methods of presentation. Over 5 yr of age diurnal and nocturnal enuresis were the commonest symptoms. Endoscopic fulguration of the valves with or without preliminary catheter drainage has been the treatment of choice. Repeated fulguration of residual valve cusps was often required. The incidence of urethral stricture (2 patients) was highest after the use of the resectoscope. Refashioning and reimplantation of the ureters is associated with a high complication rate and is not recommended. Secondary operations on the bladder neck have been avoided in this series due to the risk of incontinence. 8 patients died (7.4%) and 7 of these deaths occurred in patients presenting under 3 mo of age. One patient died from renal failure 7 yr after the diagnosis of posterior urethral valves at 2 yr of age. It is suggested that the high mortality from renal failure in the group presenting early is related to renal dysplasia.
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PMID:Posterior urethral valves in the British Isles: a multicenter B.A.P.S. review. 683 30

Superficially, the causes of voiding difficulties in children may seem quite simple, but a thorough understanding of the anatomy physiology and neurology of normal and abnormal lower urinary tract function is necessary in uncovering the etiology of often seemingly siple complaints. An ectopic ureter, neurogenic bladder, urinary tract obstruction, or simple urinary tract immaturity, for example, can all cause a child to present with the complaint of "wetting." A familiarity with all the possible causes of voiding dysfunction is required to distinguish significant from insignificant symptoms. While a "wait and see" attitude often results in the spontaneous resolution of a problem such as simple nocturnal enuresis, the misdiagnosis of a severe disorder, such as urinary outflow obstruction, may permit the insidious development of irreversible renal failure. Our purpose in writing this monograph was not to provide a comprehensive guide to the diagnosis of voiding dysfunction in children, but rather to enhance the clinician's appreciation of the complexity of these problems. Our hope is that the perspective that we have provided will obviate the diagnostic and therapeutic exaggeration of insignificant voiding complaints, while assuring that the presence of significant lesions is not overlooked.
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PMID:Voiding dysfunction in children. 741 33

A 10-year-old boy with a chief complaint of nocturnal enuresis was identified as having three cysts in each kidney on ultrasonographic examination. These findings together with a positive family history for renal failure prompted us to examine his father ultrasonographically on the same day, resulting in the identification of multiple large cysts in both kidneys. These observations supported a diagnosis of autosomal dominant polycystic kidney disease. Enhanced X-ray CT demonstrated more sensitively the presence of renal cysts in the boy. He had a normal urinary concentrating ability and no other abnormalities except for mild hypertension, which was successfully treated only by salt restriction. Ultrasonography thus appears to be a valuable tool for diagnosing polycystic kidney diseases.
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PMID:Autosomal dominant polycystic kidney disease in a child diagnosed by ultrasonography. 834 Oct 17

Micturating dysfunctions with urinary retention and with diurnal and nocturnal enuresis in children sometimes have a psychogenic genesis. They can appear during the period of development of complete control of micturition. A late recognition of this condition makes the prognosis worse, since high pressure and infections in the urinary tract can cause end-stage renal failure. Here we describe a dramatic case of a 3 year-old boy affected by a psychogenic urine and faecal retention with recurrent pyelonephritis, that was favourably treated for five years by an integrated approach involving clinicians, psychologist, educational and social operators and adoptive parents.
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PMID:[Psychogenic urinary retention in childhood. A severe case treated by an integrated global approach]. 876 78

Two sisters, eight and six years old, respectively, were admitted to Mackay Memorial Hospital in 1993 with the chief complaints of growth retardation, polyuria and nocturnal enuresis. Poor urinary concentration, sodium wasting, anemia and renal insufficiency were noted during hospitalization. Ultrasonography revealed increased renal echogenicity, loss of corticomedullary differentiation and multiple tiny corticomedullary cysts in both kidneys. Renal histopathology showed mild increase in glomerular mesangial cellularity and matrix, mild focal tubular atrophy with thickening of the tubular basement membrane. Other family members were screened by ultrasonography and found another six patients in two generations of the paternal side. Renal cysts were found in five cases. Three of them had progressively deteriorating renal failure. Five had stable renal function after three years of supportive treatment. Thus, it was concluded that the age of onset does not differentiate medullary cystic disease (MCD) from juvenile nephonophthisis (JN), and that JN and MCD could be considered a clinical complex. The absence of corticomedullary cysts on ultrasonography does not preclude the diagnosis. It is also suggested that any children with clinical symptoms of polyuria, polydipsia, anemia and growth retardation from their early years should be carefully examined, and the family history should be investigated to permit early detection of the disease.
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PMID:Juvenile nephronophthisis-medullary cystic disease complex: a family study. 915 64

Nocturia is a common and troublesome symptom in otherwise healthy elderly men and women. Nocturnal polyuria (an excessive nighttime urine output) has been documented to be a common finding in healthy men with lower urinary tract symptoms. It is also a presenting feature of various medical conditions, such as renal failure, hypercalcemia and diabetes. Fluid balance therapy is an option in those whose nocturia is secondary to nocturnal polyuria. If a reduction in fluid intake fails to reduce nocturnal frequency a variety of drug treatments may be beneficial. Several studies have confirmed the efficacy of intranasal DDAVP, a synthetic analog of antidiuretic hormone, in both healthy patients and those with neuropathic bladders, although fluid overload and hyponatremia are potential side effects. Other drug treatments include early evening diuretics, such as frusemide or bumetanide. More recently imipramine has shown therapeutic benefit in young adults with enuresis, and might prove to be useful in the elderly with nocturnal polyuria.
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PMID:Fluid balance therapy of nocturia in women. 1020 67

This retrospective study discusses the incidence of symptomatic voiding dysfunction, the etiological factors and the changes with age and with increasing duration of follow up in 67 boys treated for posterior urethral valves and followed up for a period ranging from 1-21 years. Twenty three of the 67 patients (34.3%) had symptomatic voiding dysfunction; the commonest symptom being nocturnal enuresis with diurnal urgency and frequency. Data in this study suggests a definite decrease in the incidence of urinary incontinence as the patient grows older or as the duration of follow up after valve fulguration increases. A Tc-99m DRCG based uroflowmetry was available in 31 of the 67 patients at the time of this review and evidence of detrusor dysfunction was identified in 9 of the 31 patients on the basis of uroflowmetry. Of significance was the detection of detrusor dysfunction on uroflowmetry in 3 patients with a history of normal urinary control and urinary stream with persistent post treatment hydroureteronephrosis eventually culminating into renal failure. Imipramine therapy was given to 10 of the 17 patients with symptoms of urinary frequency, urgency and nocturnal enuresis with a significantly symptomatic improvement in 9 of these patients. Urinary incontinence, detrusor dysfunction and its effect on the upper tracts and renal function is a significant determinant of the long term outcome of the boys treated for posterior urethral valves and this paper emphasises on the need to carefully evaluate and treat this aspect of all valve patients.
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PMID:Urinary continence following posterior urethral valves treatment. 1079 36

Single-system ureteral ectopia (UE) encompasses a spectrum of malformations involving the bladder trigone, ureter, and kidney. The clinical presentation is variable, and both diagnostic and therapeutic problems are common. Reduced renal function in these patients may result from primary dysplasia, obstruction, vesicoureteral reflux, or recurrent infection. Based on our experience of seven patients, suggestions for diagnostic procedures and criteria for renal saving versus nephrectomy are offered. The relationship between ostium localization, renal function, and long-term results was investigated. From 1972 to 1990, five female and two male patients were studied. During the same period, 31 patients with UE and duplex kidneys were seen. Ages ranged from 1 day to 7 years. A ureteric opening into the bladder neck was associated with dilatation of the ureter and renal pelvis. Two patients had vaginal ectopia and severe renal dysplasia. In one, a cyst of the vaginal wall (Gardner's cyst) was detected at birth. A male newborn had multicystic renal dysplasia on the left and ureteric ectopia to the ductus deferens on the right side. To our knowledge, he is the first patient reported with renal function totally dependent on a kidney with severe UE. Follow-up ranged from 4 to 9 years. One patient died in the postoperative period because of renal failure and sepsis. All the others are well and have normal creatinine values. Improvement of renal function was noted after ureteral reimplantation (URI) in patients with bladder-neck ectopia. The numbers of infections were also drastically reduced. Our observations suggest that the combination of ultrasound, cyst urethrography, and cystoscopy will be diagnostic in most patients. A suspicion of UE should be raised in symptomatic patients with apparently solitary kidneys, enuresis ureterica, or atypical obstructive uropathy. Reduced renal function in some patients with ectopia to the bladder neck will improve after URI. This may be of importance in patients with bilateral anomalies and marginal renal function.
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PMID:Long-term results of treatment of single-system ectopic ureters. 1105 50


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