Diffuse mesangial sclerosis: association with unreported congenital anomalies and placental enlargement

A case of diffuse mesangial sclerosis (DMS) associated with a number of undescribed congenital anomalies is reported. The occurrence of additional anomalies, especially ocular anomalies, is a common finding in DMS. However, neither megalocornea, Dandy‐Walker malformation, postaxial hexadactyly, rock...

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Bibliographic Details
Published in:Acta Paediatrica 1998-12, Vol.87 (12), p.1301-1303
Main Authors: Mildenberger, E, Lennert, T, Kunze, J, Jandeck, C, Waldherr, R, Versmold, H
Format: Article
Language:English
Subjects:
Abnormalities, Multiple
Biological and medical sciences
Complex syndromes
Congenital nephrotic syndrome
diffuse mesangial sclerosis
Fatal Outcome
Female
Galloway-Mowat syndrome
Humans
Infant, Newborn
Medical genetics
Medical sciences
Nephrotic Syndrome - complications
Nephrotic Syndrome - congenital
Nephrotic Syndrome - diagnosis
Nephrotic Syndrome - pathology
Placenta - pathology
placental enlargement
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Summary:A case of diffuse mesangial sclerosis (DMS) associated with a number of undescribed congenital anomalies is reported. The occurrence of additional anomalies, especially ocular anomalies, is a common finding in DMS. However, neither megalocornea, Dandy‐Walker malformation, postaxial hexadactyly, rocker‐bottom feet, nor atrial septal defect, as observed in our patient, has been reported previously in association with DMS. This case might be considered an atypical manifestation of the Galloway‐Mowat syndrome. In contrast to most cases of DMS, the patient revealed intrauterine proteinuria as the placenta was enlarged to 31% of birth weight. This case demonstrates that the large placenta, > 25% of birth weight, is not only pathognomonic of the congenital nephrotic syndrome of the Finnish type but can also occur in DMS.
ISSN:0803-5253
1651-2227
DOI:10.1111/j.1651-2227.1998.tb00956.x