Progressive familial heart block. Part I. Extent of the disease.
نویسندگان
چکیده
Progressive familial heart block (type I) has been identified in the RSA. Since 1977 many families have been referred for pedigree tracing. The present probands of some 9 pedigrees are the descendants of specific children of an immigrant; other genetic diseases appear in these pedigrees. The necessity of identifying, diagnosing and possibly treating the descendants of carriers is emphasized.
منابع مشابه
Progressive familial heart block. Part II. Clinical and ECG confirmation of progression--report on 4 cases.
Two types of progressive familial heart block controlled by a single gene have been described; 4 cases show that type I is progressive and that the pathogenesis is still unknown.
متن کاملProgressive familial heart block--two types.
Two types of heart block which occur extensively in families in the Republic of South Africa are reported. A type I heart block tends to have the pattern of a right bundle-branch block and/or left anterior hemiblock occurring individually or together, and manifesting clinically when complete heart block supervenes, either with syncopal episodes, Stokes-Adams seizures or sudden death. The condit...
متن کاملProgressive familial heart block type II (PFHBII): a clinical profile from 1977 to 2003.
An evaluation of a 38-year-old Caucasian woman, who was referred to Tygerberg Hospital (Western Cape Province, RSA) with Wenckebach second-degree or possibly complete atrioventricular (AV) block that had progressed from first-degree AV block, identified a family history of the cardiac conduction system disorder progressive familial heart block type II (PFHBII). This prompted a retrospective cli...
متن کاملProgressive Familial Heart Block Type I in a Korean Patient
A 29-year-old man was referred to the emergency department with a complaint of abdominal pain and dizziness. He had experienced two previous syncopal episodes. His family history revealed that his mother and his two uncles had received permanent pacemaker implantation. His initial heart rate was 49 beats per minute. The electrocardiography (ECG) showed atrial flutter and right bundle branch blo...
متن کاملIn-silico Evaluation of Rare Codons and their Positions in the Structure of ATP8b1 Gene
Background: Progressive familial intrahepatic cholestases (PFIC) are a spectrum of autosomal progressive liver diseases developing to end-stage liver disease. ATP8B1 deficiency caused by mutations in ATP8B1 gene encoding a P-type ATPase leads to PFIC1. The gene for PFIC1 has been mapped on a 19-cM region of 18q21-q22, and a gene defect in ATP8B1 can cause deregulations in bile salt transporters...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde
دوره 70 6 شماره
صفحات -
تاریخ انتشار 1986