case presentation we reported a 2-year-old boy with pph, who presented unusual and intractable seizure which suggested the diagnosis of md. we also reviewed a few previous reports about the association of pph with moyamoya disease. discussion moyamoya disease is a kind of cerebral vasculopathy and should be considered as a differential diagnosis for patients with unusual seizure attacks who are...

Experimental animal models suggest that uric acid might have a pathogenic role in the early development of primary hypertension. We hypothesized that serum uric acid is correlated with blood pressure in children with new-onset, untreated, primary hypertension. We evaluated 125 consecutive children referred to the Baylor Pediatric Renal Program for evaluation of hypertension. None of the subject...

Pulmonary hypertension is well described in association with portal hypertension of any cause including end stage primary biliary cirrhosis (PBC). The essential feature of this association is the presence of portosystemic shunting, including surgically created shunts. A patient with primary pulmonary hypertension and PBC without portal hypertension is reported. This suggests that primary pulmon...

OBJECTIVE To examine sporadic cases of primary pulmonary hypertension of coancestry. DESIGN An epidemiologic study of families of patients with primary pulmonary hypertension. SETTING A university-affiliated referral population. PARTICIPANTS Family members of 13 patients with primary pulmonary hypertension. MEASUREMENTS Family pedigrees involving grandparents, parents, siblings, and chi...