Congenital cutis laxa is an uncommon disorder of generalized elastolysis. The clinical picture is characterized by inelastic, loose, hanging skin that gives the appearance of premature aging. The disease is inherited most commonly in a severe autosomal recessive form, or as a relatively benign, autosomal dominant form. There is often systemic organ involvement in patients with the autosomal recessive form. Cardiopulmonary abnormalities are common and mainly determine the prognosis and life expectancy. Pulmonary emphysema, cor pulmonale, and right-sided heart failure generally caused by pulmonary disease are often seen in infancy. Various cardiovascular abnormalities including aortic aneurysm, pulmonary artery multiple branch stenosis have been reported in patients with this form of congenital cutis laxa. We report a 10-month-old boy with the autosomal recessive form of congenital cutis laxa who had pulmonary valve stenosis. To the best of our knowledge, this is the first case of this association to be reported in the English language literature. We also emphasize the systemic complications that may be associated with congenital cutis laxa.
1 In the 10-year period 1975-1984, 1188 children were admitted to the Children's Hospital of Hacettepe University in Ankara with a diagnosis of poisoning. 2 Retrospective analysis of their medical records showed that the incidence of poisoning with medicinal drugs was 64.0%, while pesticides accounted for 17.8% and plants for 6.7% of total cases. 3 The majority (69.9%) of cases were due to accidental poisoning, 70.6% of which occurred in children under 5 years of age; 15.1% of the poisonings were diagnosed as therapeutic mishaps of which 68.3% involved children under 5 years of age. 4 Analgesics (186 cases), barbiturates (176 cases) and tranquilizers (37 cases) were the most common drugs encountered, however, the two drugs most frequently overused were aspirin (146 cases) and Optalidon (175 cases). 5 Overall mortality was 4.9% (58 cases). Fifty per cent of fatalities were due to accidental poisoning while 41.4% (24 cases) were due to therapeutic mishaps. 6 This study is presented as a background to the need for the development of a Poison Information Service for Ankara.
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