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Deprecated: Implicit conversion from float 217.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Ther+Clin+Risk+Manag 2017 ; 13 (ä): 629-34 Nephropedia Template TP
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Pulmonary hypertension and vasculopathy in incontinentia pigmenti: a case report #MMPMID28533687
Alshenqiti A; Nashabat M; AlGhoraibi H; Tamimi O; Alfadhel M
Ther Clin Risk Manag 2017[]; 13 (ä): 629-34 PMID28533687show ga
Incontinentia pigmenti (IP; Bloch?Sulzberger syndrome) is a rare, genetic syndrome inherited as an X-linked dominant trait. It primarily affects female infants and is lethal in the majority of males during fetal life. The clinical findings include skin lesions, developmental defects, and defects of the eyes, teeth, skeletal system, and central nervous system. Cardiovascular complications of this disease in general, and pulmonary hypertension in particular, are extremely rare. This report describes the case of a 3-year-old girl with IP complicated by pulmonary arterial hypertension. Extensive cardiology workup done to the patient indicates underlying vasculopathy. This report sheds light on the relationship between IP and pulmonary hypertension, reviews the previously reported cases, and compares them with the reported case.