Biomaterial Database

High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood. 1999

T Srivastava, and S D Simon, and U S Alon

Section of Pediatric Nephrology, Children's Mercy Hospital, University of Missouri at Kansas City 64108, USA.

In recent adult literature, there have been reports of an increasing incidence of focal segmental glomerulosclerosis (FSGS) among patients with nephrotic syndrome. To examine whether this observation is also relevant to the pediatric population we utilized our hospital computerized database to analyze the data on children with primary nephrotic syndrome seen first between the years 1984 and 1995. A questionnaire was also sent to all metropolitan Kansas City pediatricians to identify possible patients outside the database. The inclusion criteria were clinical nephrotic syndrome or proteinuria with a kidney biopsy. A total of 148 patients (group A) were identified; 86 of them from metropolitan Kansas City (group B). In group A the incidence of minimal change disease (MCD) and FSGS was 52.7% [95% confidence interval (CI) 44%-60%] and 23.0% (95% CI 16-29%), respectively and in group B 54.7% (95% CI 44%-65%) and 24.5% (95% CI 15%-33%), respectively. Those numbers were significantly different from the International Study of Kidney Disease in Children (IS-KDC) reported incidence of 76.4% for MCD and 6.9% for FSGS. Similar to the ISKDC, in our population children over 6 years had a higher incidence of FSGS than younger children (32.8% vs. 16.7%, P = 0.028). The annual incidence rate for nephrotic syndrome in group B was 2.2 cases/10(5) children per year, of which MCD comprised 1.22 cases/10(5) children per year and FSGS 0.5 cases/10(5) children per year. The annual incidence rates of both primary nephrotic syndrome (3.6) and FSGS (1.6) were significantly higher in African-Americans, than Caucasians (1.8 and 0.3 cases/10(5) children per year, respectively). Our study indicates nearly no change in the annual incidence of pediatric primary nephrotic syndrome, but a higher incidence of FSGS with reciprocal decline in the incidence of MCD. The possibility of primary nephrotic syndrome being caused by a non-MCD entity is further raised among African-American and in children over 6 years. We conclude that our perception of primary nephrotic syndrome of childhood as a benign condition has to be carefully reexamined and a more-guarded prognostic approach adopted in our geographic area.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008297	Male		Males
D009404	Nephrotic Syndrome	A condition characterized by severe PROTEINURIA, greater than 3.5 g/day in an average adult. The substantial loss of protein in the urine results in complications such as HYPOPROTEINEMIA; generalized EDEMA; HYPERTENSION; and HYPERLIPIDEMIAS. Diseases associated with nephrotic syndrome generally cause chronic kidney dysfunction.	Childhood Idiopathic Nephrotic Syndrome,Frequently Relapsing Nephrotic Syndrome,Multi-Drug Resistant Nephrotic Syndrome,Pediatric Idiopathic Nephrotic Syndrome,Steroid-Dependent Nephrotic Syndrome,Steroid-Resistant Nephrotic Syndrome,Steroid-Sensitive Nephrotic Syndrome,Multi Drug Resistant Nephrotic Syndrome,Nephrotic Syndrome, Steroid-Dependent,Nephrotic Syndrome, Steroid-Resistant,Nephrotic Syndrome, Steroid-Sensitive,Nephrotic Syndromes,Steroid Dependent Nephrotic Syndrome,Steroid Resistant Nephrotic Syndrome,Steroid Sensitive Nephrotic Syndrome,Steroid-Dependent Nephrotic Syndromes,Steroid-Resistant Nephrotic Syndromes,Steroid-Sensitive Nephrotic Syndromes,Syndrome, Nephrotic,Syndrome, Steroid-Sensitive Nephrotic
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D005260	Female		Females
D005923	Glomerulosclerosis, Focal Segmental	A clinicopathological syndrome or diagnostic term for a type of glomerular injury that has multiple causes, primary or secondary. Clinical features include PROTEINURIA, reduced GLOMERULAR FILTRATION RATE, and EDEMA. Kidney biopsy initially indicates focal segmental glomerular consolidation (hyalinosis) or scarring which can progress to globally sclerotic glomeruli leading to eventual KIDNEY FAILURE.	Glomerulonephritis, Focal Sclerosing,Hyalinosis, Segmental Glomerular,Focal Segmental Glomerulosclerosis,Glomerulosclerosis, Focal,Hyalinosis, Segmental,Segmental Glomerular Hyalinosis,Focal Glomerulosclerosis,Focal Sclerosing Glomerulonephritides,Focal Sclerosing Glomerulonephritis,Glomerular Hyalinosis, Segmental,Glomerulonephritides, Focal Sclerosing,Sclerosing Glomerulonephritides, Focal,Sclerosing Glomerulonephritis, Focal,Segmental Glomerulosclerosis, Focal,Segmental Hyalinosis
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000367	Age Factors	Age as a constituent element or influence contributing to the production of a result. It may be applicable to the cause or the effect of a circumstance. It is used with human or animal concepts but should be differentiated from AGING, a physiological process, and TIME FACTORS which refers only to the passage of time.	Age Reporting,Age Factor,Factor, Age,Factors, Age