Idiopathic pulmonary arterial hypertension in a young patient with the Cohen syndrome.

[cohen syndrome]

We admitted a 16 -year -old boy with the Cohen syndrome to our institution for increasing dyspnoea . Investigations revealed idiopathic pulmonary hypertension . He was commenced on bosentan and oral anticoagulation and was followed up for nearly 7 years , during which he was readmitted for dynamic measurements of pulmonary artery pressure . Despite initial improvement , the right heart pressures increased again and sildenafil was added . His final hospitalisation was due to increasing breathlessness and episodes of syncope . The addition of prostacyclin conferred no reduction in pulmonary artery pressure . The patient suffered a cardiac arrest and remained intubated for 2 weeks , during which Klebsiella pneumonia and superinfection with the H 1 N 1 swine flu virus occurred . The patient died due to multi-organ failure , nearly 7 years after his initial diagnosis . The Cohen syndrome , its phenotype and clinical findings , and the incidence and treatment of pulmonary hypertension are discussed .

Diseases
Validation

Diseases presenting "pneumonia" symptom

22q11.2 deletion syndrome

acute rheumatic fever

allergic bronchopulmonary aspergillosis

alpha-thalassemia

classical phenylketonuria

cohen syndrome

congenital diaphragmatic hernia

heparin-induced thrombocytopenia

hydrocephalus with stenosis of the aqueduct of sylvius

junctional epidermolysis bullosa

lamellar ichthyosis

legionellosis

liposarcoma

lymphangioleiomyomatosis

monosomy 21

oculocutaneous albinism

omenn syndrome

pleomorphic liposarcoma

primary effusion lymphoma

proteus syndrome

pyomyositis

scrub typhus

severe combined immunodeficiency

triple a syndrome

waldenström macroglobulinemia

wiskott-aldrich syndrome

x-linked adrenoleukodystrophy

zellweger syndrome

This symptom has already been validated