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Biomedical subjects

S Menahem

Publications and source records attributed to S Menahem.

At least 55 records · Page 3Linked to original sources

Cardiac conduction abnormalities and rhythm changes after neonatal anatomical correction of transposition of the great arteries.

Seventy three infants who underwent neonatal anatomical correction for transposition of the great arteries with or without a ventricular septal defect were reviewed for evidence of conduction and rhythm abnormalities on preoperative and postoperative 12 lead electrocardiograms and during 24 hour Holter monitoring. There was a partial right bundle branch block pattern in 47% (29/62) of all patients and in 60% (24/40) of those with simple transposition. Complete right bundle branch block was noted in 21% including 5% with simple transposition. Holter monitoring showed sinus rhythm in all patients except three: one had episodes of supraventricular tachycardia, another an intermittent second degree heart block, and a third a complete heart block. Atrial extrasystoles were noted in 47% (29/62) of patients but were frequent in only three patients. Occasional unifocal ventricular extrasystoles were encountered in 37% (23/62) of patients and were frequent in a further 3% (2/62). Only one patient (2%) developed multifocal ventricular extrasystoles. The frequency of important cardiac arrhythmias after neonatal anatomical correction of transposition of the great arteries was 5%, significantly less than that reported after atrial inflow diversion for the same malformation.

Arrhythmias, Cardiac↗

Endomyocardial biopsy in infants and children with cardiomyopathy.

We reviewed our experience of endomyocardial biopsy performed on 21 symptomatic infants and children with cardiomyopathy. Clinical congestive cardiomyopathy was noted in 18 patients, 2 had hypertrophic cardiomyopathy, and 1 a restrictive cardiomyopathy. The biopsy findings led to a diagnosis of hemochromatosis in one patient, Adriamycin cardiomyopathy in another, and lymphocytic myocarditis in a third. Five patients had features of endocardial fibroelastosis, one endomyocardial fibrosis, and a further one, a mitochrondrial abnormality. In 11 patients normal or nonspecific features were seen. There were 2 myocardial perforations, both patients being successfully resuscitated. Endomyocardial biopsy, although occasionally hazardous, may sometimes provide valuable information which may lead to a diagnosis, facilitate treatment, and be of prognostic value. Despite the low positive yield, it may still be indicated in selected patients, in view of the seriousness and often poor prognosis of this disorder: 5 of our study group subsequently died.

Adolescent↗

Familial fixed subaortic stenosis.

In this report, we give details of two families in which fixed subaortic stenosis was found in more than one member. It is rare for this entity to show familial incidence.

Adolescent↗

Valvar vegetations in the neonate due to fetal endocarditis.

Mass lesions were observed on the tricuspid valve by cross-sectional echocardiography in 5 neonates, 4 of whom presented with fetal distress. Four went on to develop severe respiratory distress, 3 from meconium aspiration and 1 from hyaline membrane disease, with 2 deaths despite intensive treatment. Autopsy confirmed meconium aspiration pneumonia. Sterile, unorganised vegetations were seen on the tricuspid valve (and in 1 infant the mitral valve too). Perinatal factors such as hypoxia, haemodynamic and coagulation disturbances may have contributed to the formation of the vegetations and may reflect the severity and refractoriness of persistent pulmonary hypertension.

Endocarditis↗

Respiratory syncytial virus and supraventricular tachycardia in an infant.

A 7-week infant admitted with bronchiolitis proven to be caused by respiratory syncytial virus developed a supraventricular tachycardia which responded to digitalization. She has remained well and no longer requires medication 6 months later, her electrocardiogram now being normal. The course of the illness suggests an association between infection with respiratory syncytial virus and the development of the tachycardia.

Bronchiolitis↗

Early surgical closure of a large ventricular septal defect: influence on long-term growth.

The pre- and postoperative growth patterns of 52 otherwise normal infants undergoing primary surgical closure of a large ventricular septal defect before 7 months of age were reviewed. Serial measurements of weight, length and head circumference were compiled for all patients preoperatively and in 46 long-term survivors and were expressed as Z scores (in standard deviations from the mean for age and gender). By the time of surgery at a mean age of 0.33 year, the mean weight, length and head circumference Z scores of all 52 infants were -2.9, -0.9 and -0.6, respectively, and were all significantly below normal (p less than 0.001). At a mean age of 5.7 years, the mean weight, length and head circumference Z scores of 35 patients of normal birth weight were normal or varied only marginally from those of the reference population (-0.4, -0.1 and +0.5, respectively; p less than 0.02, p greater than 0.05 and p = 0.008, respectively) and did not differ significantly in any variable from those of 44 normal siblings. However, among 11 infants with a low birth weight, all three variables remained abnormal at long-term follow-up when compared with the reference population (-1.7, -1.7 and -0.9, respectively; p less than 0.001 for each) and 22 normal siblings (p less than or equal to 0.008).(ABSTRACT TRUNCATED AT 250 WORDS)

Birth Weight↗

Administration of prostaglandin inhibitors to the mother; the potential risk to the fetus and neonate with duct-dependent circulation.

Two infants were delivered by urgent Caesarean section at 34 weeks because of fetal distress. One rapidly developed severe cardiac failure and the other marked cyanosis. Their mothers had been prescribed indomethacin and mefenamic acid (Ponstan) to treat premature onset of labour and chronic polyhydramnios, respectively. Both infants had duct-dependent circulations, their cross-sectional echocardiography showing only a small (1-2 mm diameter) patent ductus arteriosus. The early onset and severity of their symptoms suggested that the maternal intake of prostaglandin inhibitors may have deleteriously led to early closure of their ducts, essential in duct-dependent circulations. Although such occurrences are rare, fetal cross-sectional echocardiography should be performed prior to administration of prostaglandin inhibitors during pregnancy.

Cesarean Section↗

Severe subaortic stenosis in interrupted aortic arch in infancy and childhood.

Thirteen out of a total 50 infants with interrupted aortic arch (IAA) seen between 1979-1988 had or developed severe subaortic stenosis (SAS). One had type A interruption and 12 type B. All had a large ventricular septal defect (VSD). The infundibular septum was displaced posteriorly in eight infants, severely narrowing the left ventricular outflow tract (LVOT). Three had fibromuscular narrowing of the LVOT, one each a subaortic muscle bar and membrane. The aortic root and subaortic area were small and measured between 3-8 mm. An anomalous right subclavian artery was noted in eight of the 12 type B IAA, the anomalous vessel arising from the descending aorta in seven. Thirteen infants with IAA and SAS were submitted to surgery, 12 having their subaortic area resected with three perioperative deaths early in the series and three late deaths where active treatment was ceased, including one infant with renal dysplasia who had had a successful establishment of arch continuity and pulmonary artery banding. Of the seven survivors, six have residual Doppler gradients of between 20-50 mmHg, two requiring a second resection and one a third resection. Alternate management programs are suggested based on anatomical evaluation and echocardiography. Any newborn presenting with IAA requires careful evaluation of the subaortic area, best seen on cross-sectional echocardiography. The improved surgical survival in infants following complete repair of IAA has led this important associated anomaly of subaortic stenosis to assume greater importance as a cause of mortality and long-term morbidity.

Anastomosis, Surgical↗

Using case methods effectively in clinical medicine.

This paper reviews the potential advantages of using case methods in clinical medicine, focusing on paediatric medicine as an example. Case methods describe possible approaches based on realistic case material which allow the teaching-learning process to proceed. Cases may be used to illustrate a disease or, alternatively, the care of the patient with the disease. It is possible to observe and demonstrate data collection, appropriate interviewing, examination and interpersonal skills. The clinician may take on a role model for the students to emulate, his pastoral role increasing as he gets to know his students better. It is essential that the clinician observe and verbalize the diagnostic and clinical decision-making processes of the students or himself. Case methods may not always provide complete data for each area under consideration, and on occasions the clinician may not be fully conversant with the content of the problem at hand. Students would need to learn how to function in such situations, being set specific learning tasks to acquire the necessary knowledge and skills. The case material thereby provides a focus to problem-based learning.

Clinical Medicine↗

A possible association between neonatal jaundice and long-term maternal lithium ingestion.

Two newborn infants, who developed moderate-to-severe jaundice, were born to a mother who had been taking lithium throughout both pregnancies. Both infants were treated with phototherapy while the second infant also required an exchange blood transfusion. No specific cause for the jaundice was found, raising the possibility that the maternal lithium intake may have contributed to the infants' hyperbilirubinaemia.

Adult↗

Characterization of human fructose-1,6-bisphosphatase in control and deficient tissues.

The regulatory properties of human liver and muscle fructose-1,6-bisphosphatases (FBPase) have been studied in control tissues obtained at autopsy and in tissues from a neonate with FBPase deficiency who died as a result of an overwhelming acidosis. Evidence is presented which suggests that the alkaline isoenzyme of FBPase, which is widely regarded as a laboratory artefact, may have an important role in vivo in the regulation and control of glycolysis and gluconeogenesis. FBPase exhibits the hysteretic and dissociative properties associated with regulatory enzymes, and many of the factors which effect FBPase have inverse effects on phosphofructokinase activity, thus providing an integrated regulatory cycle for the control of the direction and rate of flux through the glycolytic pathway.

Catalysis↗

Familial aggregation of defects of the left-sided structures of the heart.

A family is described where a mother and her three children had left heart defects. Three members of a second family were also noted to have such defects. This experience adds support to the hypothesis that in some families, such defects may have an incidence of recurrence higher than the 3% predicted by a multifactorial model.

Adult↗

Haemochromatosis presenting as severe cardiac failure in a young adolescent.

A 13-year-old girl, who presented almost moribund in severe heart failure, was shown to have haemochromatosis. Repeated venesection has led to return of normal cardiac function. This adolescent would appear to be the youngest patient described with haemochromatosis whose initial presentation was that of congestive cardiac failure.

Adolescent↗

Role play for the clinical tutor: towards problem-based learning.

A method of helping students learn paediatrics was developed utilizing role play as a stimulus to problem-based learning to arrive at a diagnosis. The tutor briefly stated a common paediatric problem and then took on the role of the patient and/or child, allowing himself to be interviewed by students working co-operatively in a group. This process was interrupted by the students to discuss their progress and to determine what further information was required. A suitable patient was available to be examined if requested by the students. Although there was an initial tendency on the part of students to regard the exercise as 'unreal', they delighted in refining their communication skills and trying out their skills in problem solving. The tutor had to adopt a non-traditional role, as he was not directing the students or interrupting, so as to 'teach' the students more efficient information-gathering techniques or to correct any error made. The students were able to define and complete set tasks which they derived out of the session. The introduction of this technique encourages independent learning and the acquisition of problem-solving skills, and the students enthusiastically accepted this novel way of learning. The ready availability and versatility of the tutor allowed him to be used as the principal resource, and did away with the need for (and the additional expense of) specially constructed problem-based learning units, often unavailable in most departments. The method is described in detail so that other clinical tutors may feel encouraged to adopt a similar strategy.

Australia↗

Severe acidosis in a neonate with pulmonary valve stenosis: a possible stress inducer of a fatal syndrome of fructose-1, 6-biphosphatase and aldolase deficiency.

A neonate is described whose clinical condition rapidly and irreversibly deteriorated on day two. He developed a profound acidosis, hypoglycaemia and a shock-like syndrome. The infant was centrally cyanosed and had a systolic murmur from a moderately severe pulmonary valve stenosis and a small atrial septal defect. The overwhelming acidosis was inconsistent with the severity of the congenital heart defects and as no infection was found a metabolic cause was sought. Liver tissue obtained at autopsy shortly after death on day four, showed deficiencies of fructose-1, 6-biphosphatase and aldolase.

Acidosis↗