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Biomedical subjects

S Menahem

Publications and source records attributed to S Menahem.

At least 19 recordsLinked to original sources
PubMed1997

Monozygotic twins discordant for congenital complete heart block.

OBJECTIVE: Isolated congenital complete heart block (CCHB) occurs in 1/20,000 live births. More than 85% of mothers giving birth to affected infants are anti-Ro antibody positive, but only approximately 1% of babies with anti-Ro-positive mothers develop CCHB. We studied 2 sets of monozygotic twins discordant for CCHB. METHODS: Monozygosity was determined using placental examination and DNA microsatellite analysis. HLA typing was performed. Autoantibody studies were performed using counterimmunoelectrophoresis, immunoblotting, Ro 52 and Ro 60 enzyme-linked immunosorbent assay (ELISA), and indirect immunofluorescence (IIF) on Ro 60- and Ro 52-transfected HEp-2 cells. RESULTS: Both sets of twins were monozygotic. They had similar birth weights. Twin 2 in the second set required a pacemaker at age 2 months. Both mothers were positive for anti-Ro 52 and anti-Ro 60 antibody, and neither had anti-La antibody on immunoblot. One set of twins was studied at birth. Similar titers of anti-Ro 52 and anti-Ro 60 antibody were found by IIF and ELISA. CONCLUSION: There are no previous well-documented reports of monozygotic twins discordant for CCHB. These cases demonstrate that there is still discordance in the development of CCHB despite identical genetics and environmental exposure to anti-Ro antibody.

Adult↗
PubMed1997

Psychiatric collaboration in a paediatric department.

OBJECTIVE: To review the impact of integrating child psychiatry services into a paediatric department. METHOD: Following the arrival of a new head of paediatrics, a mandatory multidisciplinary meeting was set up to review, diagnose and manage selected patients under the care of the paediatricians and/or a child psychiatrist. A prospective audit was undertaken of the patients discussed with a brief review of their outcome. The educational value of the meetings and their overall impact on the department was reviewed. RESULTS: Over an 8-month period 30 patients were discussed in some detail with diagnoses ranging from a psychiatric disorder to a chronic organic illness, each subjected to the deliberations of the multidisciplinary team of the attending paediatrician, resident staff, child psychiatrist/psychologists, nurses, social worker, etc. Recommendations included further assessment by the psychiatric team, or management by the paediatricians with or without guidance of the former, involvement of an outside agency, etc. Such interventions generally had positive outcomes: at times dramatic, although early discharge often resulted in limited goals. There was also an overall increased awareness within the department of the psychological needs of the child and family and the importance of serious physical illness; this awareness is essential in the training of paediatricians and psychiatric staff. CONCLUSION: Closer integration of the two disciplines led to benefits both to the patients and staff. The initiation of a mandatory multidisciplinary meeting facilitated the process, overseen by a psychologically-minded new head of paediatrics. His departure led to a loss of some goals achieved, raising the question as to how such collaboration may be self-generating and ongoing. In addition, the issue of how to deal with those patients with ongoing difficulties not addressed during their limited inpatient stay was unresolved.

Adolescent↗
PubMed1995

A low allergen diet is a significant intervention in infantile colic: results of a community-based study.

BACKGROUND: The effect of diet change in 38 bottle-fed and 77 breast-fed "colicky" infants, referred from community-based pediatric facilities was studied over a 1-week period in a double-blind (within each feeding mode), randomized, placebo-controlled trial. METHODS: Bottle-fed infants were assigned to either casein hydrolysate or cow's milk formula. All mothers of breast-fed infants were started on an artificial color-free, preservative-free, additive-free diet and also randomized to an active low allergen diet (milk-, egg-, wheat-, nut-free) or a control diet. RESULTS: The response to diet was assessed on day 1 and day 8 with the use of a previously validated infant distress chart on which parents recorded distress levels. If successful outcome was defined as a reduction in distress of 25% or more, after adjusting for age and feeding mode, infants on active diet had a significantly higher rate of improvement than those on the control diet (odds ratio, 2.32; 95% confidence interval, 1.07-5.0; p = 0.03). Analysis of the day 8 to day 1 distress ratio, again adjusted for age and feeding mode, showed that infants on the active diet had distress reduced by 39% (95% confidence interval, 26-50) compared with 16% (95% confidence interval, 0-30) for those on the control diet (p = 0.012). CONCLUSION: The results suggest a period of dietary modification with a low allergen diet and appropriate nutritional support should be considered in healthy infants with colic.

Age Factors↗
PubMed1995

Surgical intervention for infective endocarditis in infancy and childhood.

BACKGROUND: Infective endocarditis is an uncommon but serious disease in children. Optimal treatment strategy, especially surgical indications, continues to evolve. METHODS: Retrospective review of 98 patients treated for infective endocarditis during the past 13 years at the Royal Children's Hospital, including medically and surgically treated patients. RESULTS: Thirty of 98 patients had surgical intervention with 6.7% hospital mortality, and 76% survival probability at 45 months. The remaining patients were treated medically, with 10% hospital mortality and 52% 5-year survival probability. The incidence of structural heart disease, congestive heart failure, and spectrum of organisms was similar in the two groups. CONCLUSIONS: Despite advances in antibiotic therapy, early surgical intervention is required in a significant subset. Concurrent intracardiac repair may be appropriate.

Adolescent↗
PubMed1994

Normal early infant behaviour patterns.

As part of a wider study, infant behaviour was reviewed prospectively, using a 24 h diary completed by the mother. Infants' sleep, awake, fussing, crying and feeding behaviour were recorded at 1,2,4,6 and 8 weeks. Completed records were obtained from 21 infants. As expected most time was spent asleep, with a mean of 16.2 h at 1 week and 15.5 h at 8 weeks. Most sleeping occurred between midnight and 4 a.m. even by week 1 and there was a progressive fall in the average duration of feeding during that time interval over the 8 week period. Fussing and crying were mostly noted between 4 and 8 p.m. with an additional peak between 8 a.m. and noon, the latter disappearing by week 4. There was also a progressive drop in both the average feeding and fussing/crying times by week 8, reflecting increased settled and awake periods. It is against a background of normal early infant behaviour patterns that one can evaluate interventions aimed at altering such behaviour and dealing with the vexed problem of 'colic'.

Adult↗
PubMed1994

Recurrent dermatitis from jellyfish envenomation.

Jellyfish envenomation can cause an immediate local skin reaction, which is usually a painful linear vesiculourticarial eruption. Persistent, delayed, or recurrent dermatitis is less common. Because jellyfish sting reactions and their management are unfamiliar to family physicians, we describe a case of recurrent local dermatitis after jellyfish envenomation and suggest appropriate treatment.

Adult↗
PubMed1994

Giant ovarian cyst mimicking ascites.

A 60-year-old female patient presented to our clinic with complaints of abdominal distention. The rapid accumulation of fluid was originally thought to be ascites, based on ultrasonographic examination. The cause, however, was ultimately determined to be a borderline malignant giant ovarian cyst. Several processes can mimick ascites: bladder distention or diverticulum, hydronephrosis, pancreatic pseudocysts, and large uterine or ovarian tumors. For this reason, clinicians must consider processes other than ascites in the differential diagnosis of large abdominal fluid accumulation.

Ascites↗
PubMed1993

Cardiac arrhythmias as a manifestation of acquired heart disease in association with paediatric respiratory syncitial virus infection.

Respiratory syncitial virus (RSV) is a common cause of upper and lower respiratory tract infection in infants. An association between RSV bronchiolitis and cardiac arrhythmias has been described but is rare. A further case of an infant with supraventricular tachycardia in association with culture-positive respiratory syncitial virus bronchiolitis is reported. This case prompted a review of the two other cases of arrhythmia associated with RSV infection seen at this institution and a review of all other published cases.

Bronchiolitis, Viral↗
PubMed1993

Prevention of heart disease by subcutaneous desferrioxamine in patients with thalassaemia major.

BACKGROUND: Cardiac siderosis from transfused iron remains the major cause of death in thalassaemia major, despite iron chelation therapy with desferrioxamine. AIMS: Our aim was to determine the nature and extent of cardiac involvement and its relationship to desferrioxamine use in a group with thalassaemia major. METHODS: We reviewed 76 patients with thalassaemia major and performed multiple logistic regression to analyse factors affecting cardiac involvement. Factors studied included: patient sex, age, haemoglobin, serum ferritin, total transfusions, liver iron, duration of desferrioxamine use, electrocardiograms, echocardiograms and compliance to desferrioxamine treatment. RESULTS: Thirty-seven patients developed heart disease. They were older (p < 0.001), began desferrioxamine later (p < 0.001), had more liver iron (p = 0.014), higher serum ferritin levels (p = 0.023) and received more blood (p = 0.018). Compared to those with optimal compliance the odds of developing heart disease were increased 10.7 times in fair compliers (p < 0.001) and 5.1 times in poor compliers (p = 0.016). However, there was no significant difference between those with fair and poor compliance. After multivariate analysis only compliance (p = 0.02) and age at desferrioxamine onset (p = 0.004) remained significant. Compliance was inversely related to liver iron (p < 0.001), serum ferritin (p < 0.001) and age at desferrioxamine commencement (p < 0.001). CONCLUSIONS: We conclude that late commencement of desferrioxamine and noncompliance are associated with greater iron loading and an increased risk of heart disease.

Adolescent↗
PubMed1993

Long-term follow-up after two coronary repair of anomalous left coronary artery from the pulmonary artery.

A retrospective analysis of ten patients with anomalous left coronary artery arising from the pulmonary artery operated between 1979 and 1990 was undertaken. All presented with evidence of left ventricular dysfunction and "ischemic" mitral regurgitation. Surgical repair consisted of an aortopulmonary tunnel (Takeuchi) procedure in eight and direct left coronary artery reimplantation in two. Two patients required postoperative support with a left ventricular assist device. There were no operative or late deaths (CL 0% to 17%) for a follow-up of over 670 patient months. All patients are in New York Heart Association Class I or II, though two patients are still receiving anticongestive medications. One patient has required further surgery for pulmonary artery stenosis, and another has had a mitral valve replacement because of severe mitral regurgitation. One additional patient has moderate-to-severe residual mitral regurgitation and two have a trivial left coronary to main pulmonary artery fistula. All have a patent, nonstenotic left coronary artery and much improved left ventricular function and perfusion as assessed by echocardiography, thallium scan, gated blood pool scan, and angiography. There have been no documented arrhythmias, clinically or on Holter monitoring. The ECGs have shown resolution or improvement of the initial changes of ischemia/infarction in all patients. Chest X-rays have shown normalization of cardiothoracic ratio in eight of ten patients. Excellent early and late results can be achieved following timely surgical repair. Marked improvement in left ventricular function has been observed in patients with poor preoperative left ventricular function, even in the presence of extensive ischemia/infarction.

Child, Preschool↗
PubMed1992

Interrupted aortic arch in infancy: a 10-year experience.

Fifty infants with interrupted aortic arch (IAA), admitted between 1979 and 1988, were reviewed. They usually presented early in severe cardiac failure or shock. In the initial 5-year period, 17 of the 21 infants underwent diagnostic or confirmatory cardiac catheterization, in contrast with the latter 5 years when only eight of the subsequent 29 patients underwent catheterization. Since 1987, all patients underwent surgery after cross-sectional echocardiography. Fifteen infants had a type A IAA and 35 had type B. All had associated cardiac anomalies. Four infants were not operated on. In the initial 5-year period, of 17 infants who were surgically treated, four had a one-stage total repair, the remaining had a two-stage repair with initial reconstruction of the arch and pulmonary artery banding. There was an overall surgical mortality of 65%, reflecting the precarious state of many of these infants before surgery with a significant contribution from unrelieved subaortic stenosis. In the latter 5-year period, 29 underwent surgery, 22 had a one-stage total repair. There were three deaths, all in infants whose active treatment was withdrawn. The outcome of the survivors has generally been good, subsequent surgery being mainly related to the associated anomalies (e.g., recurrent subaortic stenosis, conduit replacement). Over this 10 year period the greater accuracy of noninvasive diagnoses, and perioperative intensive care, have led to an improvement in the preoperative state of these infants. Single-stage total repair is our procedure of choice.

Aorta, Thoracic↗
PubMed1992

Charting infant distress: an aid to defining colic.

The value of a 24-hour distress diary, previously validated against a voice-activated audiotape record, was investigated in 30 infants with colic and 30 control infants. The infants with colic had significantly more distress behavior (300.0 minutes vs 102.5 minutes; p < 0.001), although overlap of duration of distress was noted. On the basis of a clinical definition of colic--total distress lasting 180 minutes in a 24-hour period--the diary had a sensitivity of 77% and a specificity of 87%. The sensitivity of the chart was confirmed in a separate study of another 90 infants with colic.

Child Behavior↗
PubMed1992

Prevention of peripheral side-effects of transdermal hyoscine by adjunctive therapy with low dosage of pyridostigmine.

1. The value of low dosage of pyridostigmine (30 mg three times daily) in preventing peripheral anti-muscarinic side effects of a transdermal controlled-release formulation of hyoscine, was tested in a double-blind placebo-controlled study, involving 47 healthy subjects. 2. Salivary excretion was repeatedly measured during 48 h of combined therapy of two transdermal hyoscine patches with pyridostigmine and 14 h after its cessation. Blood acetylcholinesterase activity was also measured, serving as an index of pyridostigmine bioavailability. 3. The adjunctive therapy with pyridostigmine was highly effective in preventing the substantial impairment in salivary flow caused by the transdermal formulation. An associated 23% inhibition of blood acetylcholinesterase activity was observed. 4. Small doses of pyridostigmine may therefore have a role in increasing the tolerability of transdermal hyoscine therapy. In some patients this drug combination might also allow some increment of the hyoscine dose.

Acetylcholinesterase↗
PubMed1992

Case studies in adolescent deliberate self-harm.

Four adolescents admitted to a general paediatric ward at a teaching hospital with deliberate self-harm were reviewed. Their initial psychosocial assessment was noted together with their subsequent course 12 months later. Despite an extensive assessment by a multidisciplinary team during the initial admission, follow-up 12 months later revealed that each subject appeared to have ongoing problems. This outcome raised doubts as to the appropriateness and effectiveness of a prolonged hospital admission in their initial assessment. The difficulties encountered with their management are highlighted.

Adolescent↗