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Polycystic disease and hepatic fibrosis in children. Renal function studies
Insights
Children with infantile polycystic kidney disease (IPCD) and congenital hepatic fibrosis (CHF) show impaired renal function, including reduced glomerular filtration and concentrating ability. Acid excretion is also compromised, suggesting tubular or medullary structural abnormalities.
Area of Science:
- Pediatric Nephrology
- Renal Physiology
- Genetic Liver Diseases
Background:
- Infantile polycystic kidney disease (IPCD) and congenital hepatic fibrosis (CHF) are serious pediatric conditions affecting the kidneys and liver.
- Understanding the renal functional deficits in these diseases is crucial for patient management.
Purpose of the Study:
- To investigate and characterize the renal functional abnormalities in children diagnosed with IPCD and CHF.
- To assess glomerular filtration rate, urinary concentrating ability, and acid excretion in these patient groups.
Main Methods:
- Renal function tests including glomerular filtration rate measurement.
- Water deprivation and vasopressin challenge tests to assess urinary concentrating ability.
- Metabolic acidosis assessment and net acid excretion (NAE) evaluation before and after ammonium chloride loading.
Main Results:
- All IPCD patients exhibited reduced glomerular filtration rate and impaired urinary concentrating ability.
- Two of four CHF patients showed reduced glomerular filtration rate, and three of four had impaired urinary concentrating ability.
- All patients presented with asymptomatic metabolic acidosis; NAE was reduced in most, with subnormal increments after acid loading in four patients.
Conclusions:
- Children with IPCD and CHF experience significant renal functional impairment.
- Subnormal ability to concentrate urine and excrete adequate net acid suggests tubular dysfunction or medullary architectural changes due to scarring.
Abstract:
Renal function studies were done in five children with infantile polycystic disease (IPCD)of kidneys and liver and in four with congenital hepatic fibrosis (CHF). Glomerular filtration rate was reduced in all IPCD patients and in two of four CHF patients. Urinary concentrating ability following water deprivation and vasopressin administration was impaired in all IPCD patients and in three of four CHF patients. During control period, all patients had asymptomatic metabolic acidosis with total carbon dioxide content less than or equal to 20.5 millimols/liter, and net acid excretion (NAE) was reduced in all but one. Ammonium chloride was administered to seven patients; NAE increased in all, but the increments were subnormal in four. The inability to excrete maximally concentrated urine and an adequate amount of net acid may best be explained by abnormal tubular structure or alterations in medullary architecture secondary to progressive scarring, or both.