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[An adolescent with paroxysmal nocturnal hemoglobinuria].

作者信息

Rodrigues Pereira R, Swank D J, Sizoo W

机构信息

St. Clara Ziekenhuis, Rotterdam.

出版信息

Tijdschr Kindergeneeskd. 1988 Aug;56(4):163-7.

PMID:3176015
Abstract

Prompted by the case history of a 17 year old girl with anaemia, mononucleosis infectiosa and abdominal pain, paroxysmal nocturnal haemoglobinuria (PNH) is described. After a mononucleosis infectiosa infection she developed many complications of which the most prominent were hemolysis and thrombosis. Severe abdominal pain and episodic bowel obstruction occurred as a result of micro-infarction of the mesentery; bone marrow aplasia and lysis of platelets resulted in progressive thrombopenia. Pathogenesis and therapeutical possibilities are discussed. Coexistence of a necrotising enterocolitis with rectovaginal fistula, a heart infarction and the striking weight loss and hyponatremia during exacerbations, as seen in our patient, have not previously been described in PNH.

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