neurofibromatosis

Inherited disorder , autosomal dominant inheritance pattern .

Local manifestation: cafe-au-lait spots, pigmented nevi, axillary freckling. slow growing cutaneous and subcutaneous neurofibromas.

Management :
Symptomatic treatment of assessed manifestation ( seizures, skeleton defects ; scoliosis, kyposis) and surgical removal of troublesome tumors

Comments :
Refer to support groups, family needs to know about genetic implications

Reference :
Hockenberry, 2009, Wong’s essentials of pediatric nursing, 8th edition. Mosby.

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