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Biomedical subjects

S Menahem

Publications and source records attributed to S Menahem.

At least 73 records · Page 4Linked to original sources

Transcultural understanding of a hereditary disorder. Mucopolysaccharidosis VI in a Vietnamese family.

A case study of a family referred for clarification of cultural issues illustrates how a transcultural psychiatric service developed in the pediatric hospital setting can be used to advantage. A Vietnamese family with an inherited disorder. Mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome, MPS VI), resisted genetic counseling and contraception. Three out of their six children were affected, one with a fatal outcome. The transcultural consultation offered an understanding of their behavior and facilitated subsequent management.

Attitude to Health↗

Trigger segments: towards improving listening skills?

Written 'trigger' segments depicting emotionally charged clinical situations arising from real patient encounters, and ending with a pertinent statement by the parent, were prepared and presented to senior medical students, postgraduate medical and allied professionals. They were asked to record their immediate reactions, how they would have responded and on reflection, how they ought to have responded. General discussion followed, highlighting various issues dealing with patient management. Trigger segments, a relatively simple teaching approach, allow students to experience difficult clinical situations and learn, in a relatively supportive environment of their peers, their own responses. Such triggers as part of a wider programme, may provide a further method to improve listening skills and increase personal understanding of the professional's own responses.

Attitude of Health Personnel↗

Endocardial fibroelastosis in mucopolysaccharidosis type VI.

This case report describes two siblings less than 1 year of age who presented severely ill with a dilated cardiomyopathy. Full blood examination in both cases revealed marked granularity of neutrophils suggestive of mucopolysaccharidosis type VI. There were no physical features of a mucopolysaccharidosis but biochemical evaluation confirmed mucopolysaccharidosis type VI in both children. Autopsy in one patient confirmed endocardial fibroelastosis and electron microscopy of fibroblasts in the myocardium showed distention with membrane-bound vacuoles, consistent with a mucopolysaccharidosis. These siblings developed endocardial fibroelastosis before other clinical manifestations of the mucopolysaccharidosis. Assessment for metabolic causes of a cardiomyopathy is important, as cardiac disease may be the initial manifestation of a metabolic disease.

Endocardial Fibroelastosis↗

Cerebellar infarction secondary to subclavian aortoplasty repair for coarctation of the aorta.

A five-year-old boy developed a left cerebellar infarction following repair of coarctation of the aorta by subclavian aortoplasty. At operation a large left vertebral artery had been ligated. If a large vertebral artery is encountered at repair of coarctation of the aorta then consideration should be given to a method of repair which does not sacrifice this vessel.

Aortic Coarctation↗

The contribution of the paediatrician and psychiatrist to the management of the child, adolescent and his family--a paediatrician's viewpoint.

An overview highlighting the important contribution of the paediatrician to the psychological care of his patients and their families is presented. He is expected to be competent in his role of diagnosing and managing the physical disorders of his patients; in addition, the complete paediatrician needs to acquire skills in assessing and treating primary developmental and behavioural disorders, and in preventing and managing secondary emotional difficulties which may arise because of minor or major physical illness. The paediatrician may work in collaboration with the psychiatrist in managing children with psychosomatic disorders, and refer directly to the psychiatrist those children with psychoses and conduct disorders. For the rest, especially those who present with somatic symptomatology without organic findings, the psychologically minded paediatrician needs to understand the symptom and the child within the context of his family. Such an understanding may enable the paediatrician to treat the child himself or alternatively prepare his patient for a psychiatric referral, if appropriate. Issues of training of the paediatrician in the acquisition of these listening skills are discussed, emphasizing the additional need for self-directed independent learning. The important preventive role of the paediatrician is emphasized.

Adolescent Psychiatry↗

Observations on diagnostic skills in paediatric medicine and their perceived learning.

An attempt was made to review the diagnostic or problem-solving skills of a group of four students as they interviewed children and their parents, who presented with common paediatric problems. The students were instructed to complete a pro forma stating the most likely diagnosis with their reasons; their answers to be given as the interview proceeded and after completion of the patient encounter. The students entertained up to six diagnoses and despite considering the correct diagnosis early in the interview, often arrived at an incorrect final diagnosis. Some improvement was noted in the students' performance when the exercise was repeated at the end of their 10 week paediatric term. The students tended to follow a disengaged enquiry, in part related to an inadequate knowledge base. By way of comparison, four consultants were put through the same exercise and arrived at the correct diagnosis after considering only one or two diagnoses. The students were also asked how they considered they had acquired those skills. As part of their answers, all included "formal learning". The majority also suggested the observation of a suitable clinical model. When the consultants were asked the same questions all included "independent learning". All subjects came from traditional medical schools. The relevance of these findings is discussed in terms of patient management and the teaching of problem-solving skills to undergraduate and postgraduate students.

Adult↗

Surgical excision of primary cardiac tumours in infancy.

Six cases of primary cardiac tumour have been operated upon in a 7 year period from 1 June 1979 until 1 June 1986. All patients were under 6 months of age at the time of operation and two of the patients were in their first week of life. The principal indication for surgery was obstruction mainly at the right or left ventricular outflow tract level. More recently echo evaluation alone has been adequate to define the problem prior to surgery. Surgical excision has been performed without mortality or significant complications. In most cases resection has been complete, although in one case residual tumour has been left because of attachment of the tumour to vital structures. Follow-up of this case has not resulted in further surgery being required because of regrowth of the tumour. In one case, with co-existent congenital heart disease, the tumour was brought to notice after palliative systemic to pulmonary artery shunt had been performed. From the cardiac view point gratifying results have been obtained both in the short and long term following surgical resection. However, for patients with rhabdomyoma, later development of symptomatic tuberosclerosis should be anticipated in 50% of cases.

Dysgerminoma↗

Anomalous left coronary artery from the pulmonary artery: a 15 year sample.

Eleven patients with anomalous left coronary artery arising from the pulmonary artery were identified from the beginning of 1970 to the end of 1985. The only male patient presented at the age of eight years for assessment of a murmur and was symptom free. The remainder presented in infancy with features of cardiac failure. The electrocardiogram was abnormal in all patients, the majority showing anterolateral ischaemia. Cross sectional echocardiography when available showed a dilated, poorly contracting left ventricle, and in two cases what appeared to be a "normal" origin of the left coronary artery. Aortography was performed in 10 patients and led to the correct diagnosis in eight. The diagnosis was made at necropsy in three infants. One patient has remained well without treatment. The anomalous left coronary artery was ligated in three of the earlier patients, including the asymptomatic boy. Two of these cases had concurrent saphenous vein graft to the anomalous left coronary artery. The four most recent cases were treated by successful direct reimplantation of the anomalous left coronary artery to the aorta when they first presented in infancy. They continue to improve symptomatically and show objective improvement of myocardial function. In this recent experience supports our policy of early surgical re-establishment of a two coronary system from the aorta.

Aortography↗

Infant colic, distress, and crying.

The literature regarding infant colic is critically reviewed. Although there have been a number of theories proposed as to etiology of colic, the literature is characterized by difficulties in definition, methodologic problems, and numerous claims as to both etiology and management that are anecdotal. Infant colic is best conceptualized as the end result of a complex transaction between the infant and his environment, with multiple factors responsible for the crying and distress of an infant. The most important factors in appropriate intervention are a physician's receptivity and sensitivity toward the stressed mother, together with an interested and practical approach to providing adequate support while delineating the individual stresses acting on both mother and baby. Future research is needed to delineate markers for those subgroups of infants who may present with crying as a manifestation of specific clinical situations.

Animals↗