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Biomedical subjects

S E Levin

Publications and source records attributed to S E Levin.

At least 19 recordsLinked to original sources
PubMed1998

A review of Takayasu's arteritis in children in Gauteng, South Africa.

We have reviewed 31 patients with Takayasu's arteritis followed at two pediatric nephrology units in Gauteng, South Africa over a 15-year period. There were 25 black patients, 4 white, and 2 of mixed race. The mean age at diagnosis was 8.42+/-3.59 (range 2.4-14.5, median 8) years. The most common presenting sign was hypertension, followed by cardiac failure, bruits, and absent pulses. The Mantoux test was strongly positive in 27 patients (90%, control population 5%). Markers of activity included a raised erythrocyte sedimentation rate (23 patients) or Gallium single photon emission tomography (positive in 12 of 16 patients). Angiography revealed type II (abdominal aorta) and III (arch plus abdominal aorta) lesions to be most common (11 in each group). All patients received antituberculous therapy and most low-dose aspirin for its antithromboxane effect. Corticosteroids and further immunosuppression were used to control disease activity. We added total lymphoid irradiation (TLI) or cyclophosphamide. Twenty-six patients in all received further immunosuppression, with 13 patients in each group. Results were similar in the two groups, with similar pre- and posttherapy systolic blood pressures and creatinine clearances. Two patients in each group relapsed, 3 died in the TLI group and 2 in the cyclophosphamide group. Surgical intervention, usually in the quiescent phase, consisted mainly of renal autotransplantation. Because of the problems with TLI and 2 patients with papillary carcinoma of the thyroid with long-term follow-up, we no longer use TLI. We have shown that with active medical and surgical intervention the aggressive course of this disease in children can be modified.

Adolescent↗
PubMed1998

Sterile vegetations in children with severe tetralogy of Fallot.

The finding of bland, sterile vegetations in children with severe tetralogy of Fallot is unexpected, and to our knowledge, has not been reported previously. Eight patients diagnosed with tetralogy between January 1993 and July 1997 had sterile vegetations proven by histological and microbiological evaluation, in their right ventricular outflow tracts. Four of these patients were experiencing severe hypercyanotic spells, and four had severely reduced effort tolerance at presentation. They all underwent cardiac catheterization and were submitted for surgical repair. At surgery, the vegetations were thought to be causing further narrowing of the already tight fibrotic infundibular stenosis. Two of these patients had evidence of damaged valves, without evidence of active endocarditis. Although initially sterile, these vegetations, may in some instances, become infected.

Adolescent↗
PubMed1997

Acquired aortic regurgitation following occlusion of the persistent arterial duct: an echocardiographic assessment.

OBJECTIVE: To document the development of aortic regurgitation following occlusion of a patent arterial duct. DESIGN: Case series involving nine children referred for surgical ligation of an isolated patent arterial duct. SETTING: Academic referral centre. METHODS: A preoperative transthoracic and transoesophageal echocardiogram was performed in theatre to look for aortic regurgitation. Thereafter, aortic flow was monitored throughout the operation by colour flow mapping with the transoesophageal probe in situ. Onset of aortic regurgitation was documented. An immediate postoperative transthoracic echocardiogram was performed on all patients and then daily until discharge on day 5. A follow up clinical and echocardiographic assessment was performed six weeks postoperatively. RESULTS: With ligation of the patient arterial duct, transoesophageal echocardiography showed immediate regurgitation in seven of the nine patients, seen as a small central jet on colour flow mapping. Six of the seven patients continued to have aortic regurgitation on transthoracic echocardiography before leaving theatre. In none was aortic regurgitation audible clinically. At discharge, five patients still had evidence of aortic regurgitation; of four seen at follow up six weeks later, only one had residual regurgitation. CONCLUSIONS: Ligation of the patient arterial duct results in the acute termination of the "run off" in a volume overloaded situation. This, together with a rise in the peripheral vascular resistance and the persistence of increased proximal vascular capacitance, is considered to be the underlying aetiology of the acquired aortic regurgitation.

Aortic Valve Insufficiency↗
PubMed1997

Junctional ectopic tachycardia in six paediatric patients.

The presenting features and treatment responses of six children with junctional ectopic tachycardia are evaluated. Two of the patients were siblings and both presented in early childhood with cardiopulmonary failure. The elder sibling died, the surviving sibling was controlled on a combination of amiodarone, digoxin, and sotalol. The remaining four patients presented in later childhood with tachycardia induced cardiomyopathy. Two of the patients were diagnosed incidentally and have normalised their myocardial function on sotalol therapy. The other two presented in congestive cardiac failure. Radiofrequency His bundle ablation and insertion of a permanent pacemaker to control the arrhythmia was undertaken in the elder of the two patients. The remaining patient has had marginal recovery of myocardial function on a combination of amiodarone and sotalol treatment. Improvement in myocardial function may take several months and is dependent on control of the tachycardia in some patients. Sotalol, when used as single or combination treatment, was partially successful in four cases in reducing heart rate. None of the patients reverted to sinus rhythm.

Amiodarone↗
PubMed1995

Genetic heterogeneity of heart-hand syndromes.

BACKGROUND: Heart-hand syndromes compose a class of combined congenital cardiac and limb deformities. The proto-typical heart-hand disorder is Holt-Oram syndrome, which is characterized by cardiac septation defects and radial ray limb deformity. We have recently mapped the Holt-Oram syndrome gene defect to the long arm of human chromosome 12 in two families. The role of this disease locus in the pathogenesis of related conditions such as heart-hand syndrome type III (cardiac conduction disease accompanied by skeletal malformations) or familial atrial septal defects is unknown. METHODS AND RESULTS: Clinical evaluations and genetic linkage analyses were performed in five additional kindreds with Holt-Oram syndrome and also in one kindred with heart-hand syndrome type III and one kindred with familial atrial septal defect and conduction disease. Holt-Oram syndrome in all five kindreds mapped to chromosome 12q2. These studies and previous data provide odds of greater than 10(25):1 that the Holt-Oram syndrome disease gene is at chromosome 12q2. In contrast, neither the phenotypically similar disorder heart-hand syndrome type III nor the locus responsible for a familial atrial septal defect with atrioventricular block maps to chromosome 12q2. CONCLUSIONS: We demonstrate that heart-hand syndromes are genetically heterogeneous. Conditions that clinically appear to be partial phenocopies of Holt-Oram syndrome arise from distinct disease genes.

Chromosomes, Human, Pair 12↗
PubMed1992

Practice parameters for the management of anal fissure. The Standards Task Force American Society of Colon and Rectal Surgeons.

It should be recognized that these guidelines should not be deemed inclusive of all proper methods of care or exclusive of methods of care reasonably directed to obtaining the same results. The ultimate judgment regarding the propriety of any specific procedure must be made by the physician in light of all of the circumstances presented by the individual patient.

Fissure in Ano↗
PubMed1991

Management of severe hypertension in childhood Takayasu's arteritis.

Six children presented with severe hypertension caused by Takayasu's arteritis (TA), of whom four had bilateral renal artery narrowing and two coarctation syndrome. Two presented with hypertensive encephalopathy and four with congestive cardiac failure. All had a strongly positive skin reactions to purified protein derivative of mycobacterium tuberculosis. Bilateral renal arterial bypass grafts performed in two children resulted in prolonged normalization of their blood pressures, but the grafts clotted 12-18 months later. Primary renal autotransplantation was unsuccessful in two children, one with bilateral renal arterial narrowing and iliac vessel involvement and one with a long coarctation. Secondary renal autotransplantation was successful in a third child with localized aortitis. A successful aortic patch graft was performed in one child with coarctation of the aorta. Angiotensin-converting-enzyme inhibitors should be used with caution in treating the hypertension caused by TA, since bilateral renal arterial narrowing is common and their administration may result in renal insufficiency. The long-term prognosis is guarded in severely hypertensive children with extensive vascular disease due to TA.

Adolescent↗
PubMed1991

Long-term follow-up of coarctation of the aorta repaired by patch angioplasty.

The long-term complications of patch plasty repair for coarctation of the aorta were assessed retrospectively in 119 patients who were operated upon from 4 days to 13 years of age. There were 7 late deaths and 17 patients were lost to follow-up. Thus 95 patients were followed up for a minimum period of 3 years (mean 6.3 years). In addition, graded exercise tests were performed on 15 patients and 11 normal controls, measuring systolic blood pressure response and arm-leg blood pressure gradients. In this series, 16 patients (17%) were hypertensive, while 25 (26%) had a resting systolic arm-leg gradient greater than 20 mm Hg. Re-coarctation occurred more frequently when surgery had been undertaken under 1 month of age. Review of chest X-rays revealed calcification in the patch in 4 patients, one of whom subsequently went on to aneurysm formation. In a second child aneurysm formation was detected at repeat operation. Exercise tests showed a significantly higher systolic arm pressure in patients (mean: 165.3 mm Hg) when compared to controls (mean: 139.2 mm Hg) (P = 0.017) and a significant increase in arm-leg systolic gradient viz. 36 and 5.9 mm Hg, respectively (P = 0.0016). A good correlation was found between the systolic arm pressure and the systolic arm-leg gradient after exercise (r = 0.822; P = 0.0001). We conclude that the most important long-term complication following an aortic patch plasty, is re-stenosis. The development of an aneurysm was observed only twice. We believe that this apparently rare occurrence, as assessed on chest radiographs; is related to the young age (53% under 1 year) at which the repairs were carried out. Another possible reason may be that the follow-up period is not yet long enough.

Aortic Coarctation↗
PubMed1991

Turner syndrome.

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Abnormalities, Multiple↗
PubMed1990

Surgical correction of ventricular septal defects in black and white children--an 11-year study.

The clinical and ECG features, anatomical subtypes and outcome in 309 children (169 black; 140 white; 58% female) who underwent surgical closure of ventricular septal defects (VSDs) are presented. Clinical presentation was more severe in the black children; with recurrent pulmonary infections in 65% blacks compared with 34% whites. Congestive cardiac failure was noted in 60% blacks and in 37% whites. At cardiac catheterisation a left to right shunt greater than 2.5/1 was found in 45.5% of the black and in 39.3% of the white children. Severe pulmonary hypertension (greater than 80% of systemic systolic pressure) was evenly distributed in both groups. Of the 140 white children, 74.3% underwent surgery under the age of 2 years compared with 68.6% of the 169 blacks. A perimembranous VSD was found in 65% of patients in each group. Infundibular (muscular outlet) defects were observed in 28.6% of white and 30.8% of black children. Left axis deviation (LAD) on ECG was found in 11.9% of white and 9.2% of black patients; and 93% of the total of 27 cases had a perimembranous defect. Early mortality was 3.6% in white and 7.1% among the black children. Of the 17 fatal cases in the total group, 16 had severe pulmonary hypertension. It is concluded that: (i) operative results compared favourably with those reported elsewhere; (ii) the anatomical subtypes occurred with equal frequency in both ethnic groups; and (iii) this was also the case for LAD on ECG, which was most commonly associated with a perimembranous VSD.

Child↗
PubMed1990

Posterior d-transposition of the great arteries with an unusual form of aortic obstruction.

An unusual case of posterior transposition of the great arteries, with previously undescribed aortic obstruction is described. The patient, who underwent an arterial switch operation, was found at surgery to have a mass of myxomatous tissue arising from the tricuspid valve. This finding was responsible for the hemodynamically significant aortic stenosis.

Aorta, Thoracic↗
PubMed1990

Origin of left coronary artery from right pulmonary artery co-existing with coarctation of the aorta.

We report 2 infants with the unusual combination of origin of the left coronary artery from the right pulmonary artery as well as coarctation of the aorta. In the first case congestive cardiac failure persisted despite satisfactory repair of the coarctation. Further investigation revealed the anomalous origin of the left coronary artery. Corrective surgery, 24 days later was unsuccessful. When an identical diagnosis was made in the second child, it was decided, at a single operation, to combine correction of the coarctation together with re-implantation of the anomalous left coronary artery into ascending aorta. This patient has survived, and to our knowledge is the first such case with this outcome. The haemodynamic reasons for this approach are discussed.

Aortic Coarctation↗
PubMed1989

Aortic arch interruption in the neonate, with emphasis on early diagnosis and management.

Interruption of the aortic arch (IAA) is a rare but lethal congenital cardiovascular anomaly. Over a 3-month period we encountered 5 neonates with IAA, all of whom presented in the 1st week of life with congestive cardiac failure and diminished or absent pulses. All 5 underwent surgery, and 3 died from causes unrelated to the surgical repair. The diagnosis of this condition is difficult to make, although the absence of pulses may provide an important clinical clue (particularly if the left brachial and femoral pulses are involved). Using prostaglandins to re-establish ductal patency may be life-saving in these patients, since this restores blood flow to the lower trunk and kidneys. Administration of oral prostaglandin E2 to infants in whom this anomaly is suspected before they are referred to a tertiary care centre is therefore vital.

Aorta, Thoracic↗
PubMed1988

Kawasaki disease.

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Child, Preschool↗
PubMed1988

A disproportionately high incidence of symptomatic coarctation of the aorta in white infants in the Transvaal.

A retrospective study of Johannesburg Hospital records revealed that during a 4-year period (1978-1981) 49 infants who had been born in the Transvaal had presented with symptomatic coarctation of the aorta in the first year of life. The total number of live births for this period was 92,697. This incidence of 0.529 new cases per 1,000 live births, or 1/1,892 births, is three times higher than that observed in a careful study in the USA. The age at presentation and sex ratio were similar to other reported series. There was no definite seasonal incidence. Two of the patients had siblings with coarctation of the aorta. The exact reasons for the unusually high number of cases which occurred in the years 1979 and 1981 (1/1,405 and 1/1,241 live births respectively) could not be determined. It is suggested that it is probably due to a combination of genetic predisposition and as yet unidentified environmental factors.

Aortic Coarctation↗
PubMed1987

Surgical correction for one pulmonary artery arising from ascending aorta--report of five cases.

Over a 10-year period we encountered 5 infants with a pulmonary artery branch arising from ascending aorta. Surgical re-implantation of this vessel was carried out at ages 2.5, 5, 8, 9, and 19.5 months. Pre-operative cardiac catheterization demonstrated severely raised pulmonary artery pressures in all, equal to systemic in 3, and suprasystemic in 2. Four patients had origin of the right pulmonary artery from ascending aorta with a left aortic arch, and the remaining patient had an anomalous left pulmonary artery associated with a right-sided aortic arch. All patients had substantial reduction in pulmonary artery pressures immediately following surgery. One patient died 18 days post-operatively from extensive lung disease. In all 4 of the survivors, post-operative cardiac catheterization (11 to 85 months after surgery) has shown a drop in pulmonary artery pressures. One patient has been left with mildly elevated systolic values but normal diastolic levels. In 2 of the children, mild stenosis has been found at the site of reimplantation of the pulmonary artery. This anomaly should always be considered as a cause in the setting of a large left to right shunt with tricuspid incompetence and severe right ventricular hypertrophy. Prompt surgical repair, after confirmation of the diagnosis, should prevent death from heart failure or the development of irreversible pulmonary vascular disease.

Aorta↗