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Postural deformities in congenital nephrotic syndrome
Archives of Disease in Childhood
|December 1, 1981
Summary
Congenital nephrotic syndrome can cause postural deformities like joint contractures and skull abnormalities. These findings suggest a potential link between fetal positioning and placental size in affected infants.
Area of Science:
- Pediatric Nephrology
- Medical Genetics
- Prenatal Development
Background:
- Congenital nephrotic syndrome (CNS) is a rare inherited kidney disorder.
- Previous studies have focused on the renal pathology of CNS.
- Skeletal and cranial abnormalities in CNS are not well-characterized.
Observation:
- Six cases of CNS exhibited consistent physical abnormalities.
- These included flexion deformities of knees and hips, widely open fontanelles, and separated skull sutures.
Findings:
- The observed postural deformities were present across different histological types of CNS.
- This suggests a common etiology for these external anomalies in CNS.
Implications:
- The study proposes that these abnormalities may be postural, influenced by factors like a large placenta.
- Further research into the biomechanical and environmental factors in utero is warranted.