A variant of unknown significance in the GLA gene causing diagnostic uncertainty in a young female with isolated hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is genetically heterogeneous, and largely caused by mutations in genes encoding sarcomere proteins. However, GLA mutations causing Fabry disease, an X-linked lysosomal storage disorder, may also present with isolated HCM. As HCM genetic testing panels are increasingly being used clinically, variants of unknown significance (VUS) are encountered, leading to challenges in interpretation.

Benign cardiac tumours, malignant arrhythmias.

Four cases of pediatric cardiac tumours (PCTs) associated with ventricular arrhythmias are reported. Sudden cardiac death attributable to the tumour occurred in two children. A third child received an implantable cardioverter defibrillator and the fourth had persistent ventricular arrhythmia despite medical therapy.

Postpericardiotomy syndrome: no evidence for a viral etiology.

Background: Postpericardiotomy syndrome has been considered a disorder induced by viral infection. This conclusion is based on serologic criterions, but these may be unreliable following either cardiopulmonary bypass or transfusion therapy. Previous studies have not verified the proposed etiology either by isolation of viruses, or by detection of their genome.

Usefulness of the electrocardiogram in diagnosing mechanisms of tachycardia.

The electrocardiogram, despite its simplistic technological composition, remains a valuable tool in the diagnosis of pediatric arrythmias. In this article the characteristic features of different tachycardias are reviewed.

US policy for disease control among imported nonhuman primates.

In 1990, in response to the occurrence of Ebola virus (subsequently identified as subtype Reston) infection among cynomolgus monkeys imported from the Philippines, the United States implemented strict disease control measures for handling nonhuman primates during transit and quarantine and initiated importer facility compliance inspections. Disease control measures emphasized protection of workers from exposure, use of containment facilities and procedures, measures to prevent spread of infection among animals, and laboratory testing of animals that die or become ill during quarantine. From 1991-1995, no outbreaks of filovirus infection occurred, and only one other disease outbreak (caused by Mycobacterium species) was recognized.

Interpretation of electrocardiograms in infants and children.

Electrocardiography is a useful tool in the management of paediatric heart disease. Interpretation requires practice due to changing norms which are brought about by the physiological changes that occur in the circulatory system in this age group. This article outlines these normal changes.

Tuberculosis among foreign-born persons in Los Angeles County, 1992-1994.

Objectives: To describe the epidemiology of foreign-born tuberculosis (TB) cases in Los Angeles County and to evaluate current TB screening and follow-up of immigrants and refugees (I&R) to the USA.

Design: Retrospective analysis of the Los Angeles County TB registry between October 1992 and December 1994. We matched all cases who entered the USA during fiscal year 1993 (FY93) with a database from the tracking system of I&R with suspected TB.

Overseas screening for tuberculosis in immigrants and refugees to the United States: current status.

The number of reported cases of tuberculosis (TB) in foreign-born persons in the United States during 1995 was 8,042, 36% of the national total. The overseas screening of immigrants and refugee visa applicants, which relies on a chest radiograph and smear microscopy, is designed to identify future U.S.

Tuberculosis screening for immigrants and refugees. Diagnostic outcomes in the state of Hawaii.

The effectiveness of the required overseas tuberculosis (TB) screening for immigrants and refugees to the United States has not been evaluated since new guidelines were introduced in 1991. Using data from the Hawaii State TB register for 1992-1993, patient records, and data from the U.S.

Surveillance for pneumonic plague in the United States during an international emergency: a model for control of imported emerging diseases.

In September 1994, in response to a reported epidemic of plague in India, the Centers for Disease Control and Prevention (CDC) enhanced surveillance in the United States for imported pneumonic plague. Plague information materials were rapidly developed and distributed to U.S.

Malaria in east African refugees resettling to the United States: development of strategies to reduce the risk of imported malaria.

Resettlement to the United States of malaria-infected refugees can pose problems for both the refugees and their resettlement communities. To formulate malaria management strategies for East African refugees before resettlement to the United States, epidemiologic data were reviewed and malaria prevalence surveys were conducted among refugees awaiting resettlement in Mombasa, Kenya, and Khartoum, Sudan, in 1993. Overall, 279 Somali (Mombasa) and 127 Ethiopian (Khartoum) refugees were surveyed.

Double-blind placebo-controlled trial of corticosteroids in children with postpericardiotomy syndrome.

The objective of this study was to assess the efficacy of corticosteroids in hastening the recovery of children with postpericardiotomy syndrome, using a randomized double-blind placebo-controlled trial in a tertiary care referral center for pediatric cardiology and cardiac surgery. Twenty-one children, 6 months of age or older (mean age 3.9 years) with postpericardiotomy syndrome following open or closed heart surgery were administered either prednisone 2 mg/kg/day reducing to zero over 14 days (n = 12) or placebo (n = 9).

Influenza--United States, 1988-89.

Problem/condition: CDC monitors the emergence and spread of new influenza virus variants and the impact of influenza on morbidity and mortality annually from October through May.

Reporting Period Covered: This report covers United States influenza surveillance conducted from October 1988 through May 1989.

Description Of System: Weekly reports from the vital statistics offices of 121 cities provided an index of influenza's impact on mortality; 58 WHO collaborating laboratories reported weekly identification of influenza viruses; weekly morbidity reports were received both from the state and territorial epidemiologists and from 153 sentinel family practice physicians.

Influenza--United States, 1989-90 and 1990-91 seasons.

During the 1989-90 influenza season, 98% of all influenza viruses isolated in the United States and reported to CDC were influenza A. Almost all those that were antigenically characterized were similar to influenza A/Shanghai/11/87(H3N2), a component of the 1989-90 influenza vaccine. Regional and widespread influenza activity began to be reported in late December 1989, peaked in mid-January 1990, and declined rapidly through early April 1990.

Effectiveness of oral sotalol for treatment of pediatric arrhythmias.

Sotalol is a beta blocker with class III activity. Few investigators have reported its use in pediatric patients. From August 1985 to May 1990, 66 patients (mean age 8.

Efficacy and safety of oral sotalol in early infancy.

Sotalol, a nonselective beta blocking agent with additional Class III activity has been shown to be extremely effective in the treatment of supraventricular tachycardias in adults and children. Little information is available on its use in infants. From August, 1985 to April, 1990, 18 infants, 2 months of age or less, were treated with oral sotalol for supraventricular arrhythmias.

Illness in hemodialysis patients after exposure to chloramine contaminated dialysate.

In September 1987, patients at an outpatient dialysis center were exposed to chloramine contaminated dialysate when the carbon filter in a recently modified water treatment system failed. Forty-one patients required transfusion to treat the resultant hemolytic anemia. Epidemiologic investigation demonstrated that the mortality rate among dialysis center patients increased during the 5 months after chloramine exposure when compared with the 12 months before chloramine exposure, but no deaths could be attributed to the exposure.

Swine influenza virus infections. Transmission from ill pigs to humans at a Wisconsin agricultural fair and subsequent probable person-to-person transmission.

In September 1988, a previously healthy 32-year-old pregnant woman was hospitalized for pneumonia and died 8 days later. The only pathogen detected was an influenza virus antigenically related to the swine influenza virus (SIV). Four days before illness onset, the patient visited a county fair swine exhibition where there was widespread influenzalike illness among the swine.

Virus zoonoses and their potential for contamination of cell cultures.

Silent virus infections of laboratory animals present a human health hazard, from direct exposure and from contamination of biological products for human use. Here we report two recent examples. In 1989, an outbreak of lymphocytic choriomeningitis virus (LCMV) infections was recognized among workers at a cancer research center after an animal caretaker developed viral meningitis.

Sepsis associated with transfusion of red cells contaminated with Yersinia enterocolitica.

Between April 1987 and May 1989, the Centers for Disease Control investigated seven cases of transfusion-associated Yersinia enterocolitica sepsis; four were caused by organisms of serotype O:3, and one each was caused by organisms of serotype O:1,2,3; O:5,27; and O:20. All seven recipients developed septic shock after receiving units of red cells (RBCs) contaminated with Y. enterocolitica; five recipients died.

Growth and endotoxin production of Yersinia enterocolitica and Enterobacter agglomerans in packed erythrocytes.

Since 1987, the Centers for Disease Control investigated six cases of transfusion-associated sepsis. All six patients developed septic shock after receiving units of packed erythrocytes (PRBCs) contaminated with Yersinia enterocolitica (five patients) and Enterobacter agglomerans (one patient); three of the blood recipients died. We studied the growth and endotoxin production of Y.

Pyrogenic reactions associated with the reuse of disposable hollow-fiber hemodialyzers.

We investigated 18 pyrogenic reactions (PRs) that occurred between July 1 and 13, 1987, in 16 patients receiving long-term hemodialysis at one dialysis center in Illinois. We defined a case of PR as the onset of chills or fever (oral temperature, greater than or equal to 37.8 degrees C) in a patient who was afebrile and had no signs or symptoms of infection before a dialysis treatment.

Pulmonary artery banding: results and current indications in pediatric cardiac surgery.

The results of pulmonary artery banding in 144 patients seen from 1971 to 1984 were reviewed. Age ranged from 1 week to 4 years (median, 8 weeks) and weight, from 1.1 to 16 kg (median, 4 kg).