Tricuspid atresia with Christmas disease (hemophilia B)

Merry christmas for patients with hemophilia B.

Hemophilia B (also known as Christmas disease) is due to deficiency of coagulation factor IX (FIX). In this issue of the Journal, Nathwani et al. report the first unequivocal evidence of successful gene therapy for hemophilia B — a major advance in this field.1 This success for hemophilia may translate into gene therapy for other blood protein deficiencies. Hemophilia is due to deficiency in a ...

Combined Hemophilia and Christmas Disease.

Hemophilia, Christmas disease, and matters of terminology.

Tricuspid Atresia

Left ventricular dimensions and contractility were determined by echocardiography in 33 patients with tricuspid atresia in 1985 and again in 1988. Eight patients remained palliated throughout the 3-year period; neither the left ventricular end-diastolic diameter (153±15% of normal vs. 157+19%o, p=NS) nor a load-independent index of contractility (rate-corrected velocity of shortening [VCFc]/end...

Tricuspid atresia with I-transposition.

Sir, In their recent article (1987;57:543-7) on tricuspid atresia with transposition of the great arteries Warnes and Somerville emphasised the inportance of subaortic stenosis in these patients, especially those with coarctation. They suggested that the Fontan procedure was the procedure of choice in these patients but remarked that in patients with subaortic stenosis the prognosis after Fonta...