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Lawrence-Moon-biedl syndrome: Symptoms, Cause and Treatment


Biedl-Bardet Syndrome also called Lawrence-Moon-Biedl syndrome - a syndrome of obesity, pigmentation of the retina, and extra digits accompanied by intellectual disability.

Laurence-Moon-Biedl syndrome is an autosomal recessive condition characterised by:

  • mental retardation
  • polydactyly
  • cataract
  • squint
  • renal anomalies
  • hypogonadism
  • spastic paraplegia
  • obesity
  • retinitis pigmentosa

Glomerulonephropathy of Laurence-Moon-Biedl syndrome

A patient with Laurence-Moon-Biedl syndrome and nephrotic range proteinuria is presented. Radiological investigation of the urinary tract revealed clubbed calyces but no evidence of obstruction or vesicoureteric reflux. Renal biopsy revealed occasional sclerotic glomeruli, extensive foot-process fusion and segmental glomerular basement membrane abnormalities with negative immunofluorescence for immunoglobulins and complement. Nephrotic proteinuria responded to steroid therapy but mild proteinuria persisted. The findings were consistent with minimal change nephropathy superimposed on the glomerular lesions of Lawrence-Moon-Biedl syndrome.

 

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