Home :: Hand and Foot Care :: Nail Disorders :: Nail Patella Syndrome - NPS Nail Patella Syndrome Cause, Treatment, Symptoms, Medication
Nail-patella syndrome is an autosomal dominant condition which presents with triangular rather than half-moon shaped lunulae, especially of the thumb and forefingers. The nail plates may be small or dystrophic. The patellae are hypoplastic or absent. Other skeletal anomalies may be present and renal impairment (glomerulonephritis) occurs in up to 30% of individuals. Nail Patella Syndrome (also called Fong's Disease, Hereditary Onycho-Osteodysplasia ['HOOD'] is characterized by several typical abnormalities of the arms and legs as well as kidney disease and glaucoma. The incidence of NPS is about 2-3/100.000 births and it haven't any ethnic prevalence. Inheritance occurs as an autossomal dominant trait linked to ABO blood group locus. The risk of transmitting the disorder from affected parent to offspring is 50%, regardless of the sex of the child, with females being affected 10% more often. Causes of Nail Patella Syndrome - NPSThe mechanisms underlying the different manifestations and severity of the symptoms in this disorder remain uncertain. NPS is known to be an autosomal dominant hereditary disease. A genetic abnormality is believed to lead to altered connective tissue metabolism with widespread structural defects in collagen. Abnormal collagen deposition in the glomeruli probably causes the nephropathy associated with NPS. Treatment of Nail Patella Syndrome - NPSThe treatment for nps and glaucoma depends upon the nature and severity of each case. As a rule, glaucoma cannot be cured, but it can be controlled. Eye drops, pills, laser procedures, and surgical operations are used to prevent or slow further damage from occurring. With any type of glaucoma, periodic examinations are very important to prevent vision loss. Because glaucoma can worsen without your being aware of it, your treatment may need to be changed over time. Nail Patella Syndrome PrognosisPrognosis is usually benign although 10% of all NPS develop end-stage renal failure and the introduction of dialysis and renal transplantation is necessary.
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