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Biomedical subjects

N Zucker

Publications and source records attributed to N Zucker.

14 recordsLinked to original sources
PubMed2004

Prenatal diagnosis of Shone's syndrome: parental counseling and clinical outcome.

OBJECTIVE: To describe a series of fetuses diagnosed as having Shone's syndrome, which includes four cardiac defects and for which there is a wide variety of clinical presentations, surgical treatments and outcomes, and to discuss the counseling strategy. METHODS: We reviewed retrospectively the records of four babies who were suspected prenatally to have Shone's syndrome. RESULTS: The mean age at diagnosis was 27.5 (range, 22-35) weeks. A small left ventricle, mitral and aortic annulus and ascending aorta were detected in three cases. In three fetuses there was an abnormal mitral valve apparatus and in two fetuses a sub-aortic membrane was detected. Coarctation of the aorta was an impending diagnosis in three babies. Following counseling, all parents decided to continue the pregnancy to term. Echocardiographic evaluation was performed soon after birth. The mean mitral valve annulus diameter was 8.2 (range, 7-10) mm, and that of the aortic valve annulus was 6 (range, 5-7) mm. The aortic valve was bicuspid in all babies with an additional sub-aortic membrane in two babies. Doppler ultrasound examination revealed high-velocity flow through the mitral valve in three babies; two of them had a parachute mitral valve. Coarctation repair was performed in two babies during the first week of life. One patient underwent mitral valvuloplasty followed by later mitral valve replacement. All patients were alive after a mean of 7.8 (range, 3.3-10.5) years' follow-up. CONCLUSION: When counseling families regarding fetal Shone's syndrome, it is important to emphasize the wide variety of clinical presentations and possible outcomes. Differentiation between hypoplastic left ventricle and Shone's complex is crucial and may influence the physician's attitude, the presentation of the case to the family and the family's final decision.

Counseling↗
PubMed2001

The role of tissue plasminogen activator in the successful treatment of infected cardiac thrombus in children.

Infected cardiac thrombus is rare in children, with antibiotic or antifungal agents used as the first line of treatment. Persistence is an indication for surgical intervention. We describe two children who were treated successfully with a combination of antibiotic and antithrombotic agents. Use of antithrombotic agents promotes degeneration of fibrin, thus reducing the mass and facilitating the diffusion of the antibiotic and/or antifungal agents.

Anti-Bacterial Agents↗
PubMed2000

Congenital dyserythropoietic anemia type I presenting as persistent pulmonary hypertension of the newborn.

Congenital dyserythropoietic anemia (CDA) is a rare group of inherited bone marrow disorders characterized by anemia with ineffective erythropoiesis. We report 3 siblings from a family known to have CDA type I who presented with persistent pulmonary hypertension of the newborn (PPHN). We suggest that the diagnosis of CDA type I should be considered in any neonate with PPHN and anemia.

Anemia, Dyserythropoietic, Congenital↗
PubMed2000

Successful non-surgical treatment of Candida tropicalis endocarditis with liposomal amphotericin-B (AmBisome).

Fungal endocarditis in children is most commonly a complication of palliative or curative surgery for congenital heart disease, rheumatic valvulitis and prolonged indwelling central venous and umbilical catheters. We describe here the case of a 3-y-old patient with chronic diarrhoea and prolonged total parenteral alimentation who developed severe C. tropicalis endocarditis and was treated successfully using a liposomal preparation of amphotericin-B (AmBisome) without surgical intervention.

Amphotericin B↗
PubMed2000

Maintenance of androdioecy in the freshwater shrimp, Eulimnadia texana: estimates of inbreeding depression in two populations.

Androdioecy is an uncommon form of reproduction in which males coexist with hermaphrodites. Androdioecy is thought to be difficult to evolve in species that regularly inbreed. The freshwater shrimp Eulimnadia texana has recently been described as both androdioecious and highly selfing and is thus anomalous. Inbreeding depression is one factor that may maintain males in these populations. Here we examine the extent of "late" inbreeding depression (after sexual maturity) in these clam shrimp using two tests: (1) comparing the fitness of shrimp varying in their levels of individual heterozygosity from two natural populations that differ in overall genetic diversity; and (2) specifically outcrossing and selfing shrimp from these same populations and comparing fitness of the resulting offspring. The effects of inbreeding differed within each population. In the more genetically diverse population, fecundity, size, and mortality were significantly reduced in inbred shrimp. In the less genetically diverse population, none of the fitness measures was significantly lowered in selfed shrimp. Combining estimates of early inbreeding depression from a previous study with current estimates of late inbreeding depression suggests that inbreeding depression is substantial (delta = 0.68) in the more diverse population and somewhat lower (delta = 0.50) in the less diverse population. However, given that males have higher mortality rates than hermaphrodites, neither estimate of inbreeding depression is large enough to account for the maintenance of males in either population by inbreeding depression alone. Thus, the stability of androdioecy in this system is likely only if hermaphrodites are unable to self-fertilize many of their own eggs when not mated to a male or if male mating success is generally high (or at least high when males are rare). Patterns of fitness responses in the two populations were consistent with the hypothesis that inbreeding depression is caused by partially recessive deleterious alleles, although a formal test of this hypothesis still needs to be conducted.

Animals↗
PubMed1999

Surgical management of tetralogy of Fallot in the first year of life.

BACKGROUND: The surgical approach to tetralogy of Fallot (TOF) continues to evolve and now many centers favor early repair for TOF. METHODS: Our experience includes 82 consecutive patients less than 1 year old with TOF (n = 74) and TOF with pulmonary atresia (n = 8) who were operated on between January 1992 and March 1998. Mean age at repair was 5.2 +/- 1.2 months and mean weight was 4.5 +/- 0.4 kg. Seven patients (anomalous left anterior descending artery [n = 1], pulmonary atresia with hypoplastic pulmonary arteries [n = 6]), underwent palliative procedures in the neonatal period followed by complete repair. Forty-nine patients (59%) were symptomatic (severe cyanosis or hypoxic spells), and 33 patients (41%) were asymptomatic. A combined transatrial-transpulmonary approach was employed in 28 patients (34%), and transannular patch or conduit for reconstruction of the right ventricular outflow tract (RVOT) was required in 54 patients (66%). The mean Nakata index was 160 +/- 25 mm2/m2. RESULTS: There were no hospital deaths. Mean post-repair peak right ventricular/systemic pressure ratio was 0.48 +/- 0.1. There were no late deaths or reoperations during a mean follow-up of 23 +/- 5 months. All patients are currently asymptomatic and in New York Heart Association class 1. Postoperative evaluation by two-dimensional and Doppler echocardiography or cardiac catheterization showed minimal pulmonary artery stenosis with a mean pressure gradient of 15 +/- 6 mm Hg across the RVOT. CONCLUSIONS: Our experience suggests that early repair of TOF can yield excellent results and initial palliation does not preclude early complete repair.

Blood Pressure↗
PubMed1999

Acute dilated cardiomyopathy and central nervous system toxicity following propranolol intoxication.

OBJECTIVE: We report a case of a 16-year-old boy who developed central nervous system (CNS) depression and acute dilated cardiomyopathy following ingestion of 3200 mg of propranolol in a suicide attempt. Early echocardiographic findings were the only sign of cardiac toxicity. DESIGN: A case report. SETTING: Pediatric intensive care unit of a teaching hospital. RESULTS: This child developed significant acute dilated cardiomyopathy and severe CNS depression 2 hours after ingesting 3200 mg of propranolol. The child was treated with gastric lavage, activated charcoal, and mechanical ventilation. Following the echocardiographic findings, treatment with isoprenaline hydrochloride and glucagon were given intravenously. Echocardiographic examination 12 hours following treatment showed normal left ventricular size and function. No change in pulse rate or blood pressure was reported on admission and during his hospitalization. DISCUSSION: In the early stages of propranolol and other lipophilic beta-blocker intoxication, severe CNS depression can develop in the absence of clinical signs of cardiac toxicity. Early echocardiographic evaluation is important and may prevent delay in diagnosis and treatment of cardiac toxicity.

Acute Disease↗
PubMed1995

Successful radiofrequency ablation in a 3-month-old baby with permanent junctional reciprocating tachycardia: a new era in the treatment of incessant life-threatening arrhythmias in infants.

We report our experience in treating a 3-month-old baby with accessory atrioventricular pathway by radiofrequency (RF) ablation. The baby was discovered to have incessant supraventricular tachycardia at 4 weeks of age. At that time, two-dimensional echocardiography showed dilated cardiomyopathy. Multiple direct current shocks and multipharmaceutical treatment failed to restore sinus rhythm. Electrophysiology study showed the presence of posteroseptal accessory pathway. Immediately after delivery of two RF pulses, successful ablation of the accessory pathway and complete atrioventricular (AV) block with good junctional escape rhythm were observed. Normal rhythm and AV conduction subsequently returned and all medical treatment was discontinued. To our best knowledge, this is the first case of successful treatment with RF ablation in early infancy.

Catheter Ablation↗
PubMed1994

Cardiac abnormalities in the Bardet-Biedl syndrome: echocardiographic studies of 22 patients.

The Bardet-Biedl syndrome is an autosomal recessive disorder of polydactyly, obesity, tapetoretinal degeneration, mental retardation, hypogenitalism, and renal involvement. A high incidence of congenital and acquired heart disease was reported in the former "Laurence-Moon-Biedl-Bardet" syndrome. However, since the establishment of the Bardet-Biedl syndrome as a separate clinical entity, cardiac involvement has not been evaluated in this disorder. We have performed echocardiographic studies on 22 patients with the Bardet-Biedl syndrome from three extended, highly inbred Bedouin families. In addition to previously reported congenital heart defects we have observed hypertrophy of the interventricular septum and dilated cardiomyopathy. Our findings of cardiac involvement in 50% of the cases suggest that echocardiographic examination should be included in the clinical evaluation and follow-up of patients with Bardet-Biedl syndrome.

Adolescent↗
PubMed1994

Abnormal coronary perfusion in experimental scorpion envenomation.

Perfusion defects and left ventricular dilation after experimental scorpion envenomation were evaluated in five dogs. Left Left ventricular dilation was observed in three dogs and right ventricular dilation in one other, in scans immediately after envenomation. Perfusion defects were inferred from scans in four dogs. The data are strongly suggestive of coronary hypoperfusion, and the mechanics of abnormal coronary flow after scorpion envenomation are discussed.

Animals↗
PubMed1992

Abdominal pain with free peritoneal fluid detected by ultrasonography as a presenting manifestation of acute rheumatic fever.

The clinical and laboratory data for an 8 year old girl with abdominal pain as a presenting manifestation of acute rheumatic fever are reported. An abdominal investigation with ultrasonography carried out at the same time showed free peritoneal fluid. These findings support the proposal that the abdominal pain classically described in acute rheumatic fever is one of the manifestations of the inflammatory process. It is suggested that in patients with abdominal pain, fever, and increased erythrocyte sedimentation rate in whom the diagnosis is not clear, an abdominal investigation with ultrasonography could be helpful in establishing the correct diagnosis.

Abdominal Pain↗
PubMed1992

Short- and long-term comparative study of anistreplase versus streptokinase in acute myocardial infarction.

Streptokinase is well established as an effective thrombolytic. Anistreplase, a new thrombolytic drug, is a complex of streptokinase and acylated human plasminogen that can be administered by intravenous bolus and activates plasminogen at the clot site. Although both streptokinase and anistreplase are effected in treating myocardial infarction (MI), they have different pharmacologic properties. This study was designed to identify short- and long-term differences in their clinical effectiveness, safety in use, and survival rates in patients with acute MI. One hundred ten successive patients under seventy years of age admitted within three hours after onset of sustained chest pain suggestive of acute MI were randomized to receive either 30 units of anistreplase intravenously over five minutes or intravenous injection of 750,000 units of streptokinase over thirty to sixty minutes. Reperfusion was achieved in 34 of the 52 (65%) patients treated with anistreplase and in 41 of the 58 (71%) patients treated with streptokinase (p = NS). The two drugs were equally effective in preserving left ventricular ejection fraction, which was found to be significantly better in patients with anterior wall MI who had achieved reperfusion than it was in those who did not (p less than 0.02). One-month, twelve-month, and thirty-six-month survival rates were high (96% to 88%) with no significant difference between the two treatment groups. The authors conclude that the two drugs are equally effective thrombolytic agents but that anistreplase has the advantage that it can be administered as a bolus injection.

Aged↗
PubMed1979

Interferon production: variation in yields from human cell lines.

A survey of human diploid, aneusomic, transformed and tumor fibroblast or fibroblastoid cell lines for their capacity to produce interferon after polyriboinosinic acid:polycytidylic acid induction showed considerable variation in responsiveness. There was no apparent correlation between karyotype or phenotype and interferon production. Pretreating or "priming" the cells with human interferon generally led to increased yields of interferon after polyriboinosinic acid:polycytidylic acid induction in all cell lines tested. All the cells also showed the "super-induction" phenomenon, although to varying degrees. The combination of priming and superinduction conditions led to the production of very high yields of interferon in some cell lines, but in other lines, yields less than either the primed or superinduced amounts were found. A more limited survey of human cell lines for their capacity to produce interferon after Newcastle disease virus induction also showed that yields varied from line to line. However, there was little evidence to suggest that ability to produce interferon after Newcastle disease virus induction correlated with that after polyriboinosinic acid:polycytidylic acid induction.

Cell Line↗