Document Detail


Polycystic kidney disease in the first year of life.
MedLine Citation:
PMID:  3668738     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
To determine the frequency of autosomal recessive and autosomal dominant polycystic kidney disease (PKD) in infants and to compare the rate of progression of these conditions, we conducted a retrospective survey of 48 patients who were seen with PKD before 1 year of age and who survived the first month of age. Seventeen patients had recessive PKD; six had dominant PKD. Eighteen patients had insufficient data to categorize the type of PKD with certainty. Seven patients were classified as "other"; three had glomerulocystic disease and the remainder had multiple malformation syndromes or tuberous sclerosis. Renal ultrasonography and excretory urography accurately detected 15 of 17 patients with recessive PKD, but only one patient with dominant PKD was correctly diagnosed by excretory urography. The majority of patients in all groups required antihypertensive therapy. The 17 children with recessive PKD have been followed up for 6.1 +/- 4.3 (SD) years. Eight patients are doing well. Two patients have died; five others have required treatment for renal failure. Only one patient has an estimated glomerular filtration rate within the normal range after 6 years of age. Long-term evaluation of most of the patients with dominant PKD is not yet available; however, by age 42 months one patient has required dialysis. To provide optimum genetic counseling and accurate diagnosis for patients with PKD, a combination of careful family evaluation, radiography, and liver or kidney biopsy is required. The outcome of patients who survive the neonatal period appears not to be so grim as previously feared, underscoring the importance of aggressive supportive care and the need for physician and family education.
Authors:
B R Cole; S B Conley; F B Stapleton
Related Documents :
2316588 - Acute renal failure in hypertensive disorders of pregnancy. pregnancy outcome and remot...
21242708 - Clinical value of serum neopterin, tissue polypeptide-specific antigen and ca19-9 level...
9543598 - Contribution of tubular anion and cation secretion to residual renal function in chroni...
11597038 - Factors associated with acceptance of patients for chronic dialysis.
21342138 - Cartilage oligomeric matrix protein - inflammation biomarker in knee osteoarthritis.
17162488 - The problems in sexual functions of vitiligo and chronic urticaria patients.
Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Journal of pediatrics     Volume:  111     ISSN:  0022-3476     ISO Abbreviation:  J. Pediatr.     Publication Date:  1987 Nov 
Date Detail:
Created Date:  1987-12-14     Completed Date:  1987-12-14     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  693-9     Citation Subset:  AIM; IM    
Affiliation:
Southwest Pediatric Nephrology Study Group, Washington University School of Medicine, St. Louis.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Age Factors
Antihypertensive Agents / therapeutic use
Child, Preschool
Diagnosis, Differential
Genes, Dominant
Genes, Recessive
Humans
Hypertension, Renal / drug therapy,  etiology
Infant
Infant, Newborn
Polycystic Kidney Diseases / diagnosis,  epidemiology*,  genetics
Prognosis
Renal Dialysis
Retrospective Studies
Chemical
Reg. No./Substance:
0/Antihypertensive Agents

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Phagocytosis of necrotic muscle in muscle isografts is influenced by the strain, age, and sex of hos...
Next Document:  Long-term prognosis of giant coronary aneurysm in Kawasaki disease: an angiographic study.