Solomon E. Levin

Platelet, erythrocyte, and fibrinogen kinetics in the hemolytic-uremic syndrome of infancy.

Thrombocytopenia, anemia, and uremia compose the “triad” of the hemolytic-uremic syndrome of infancy. To elucidate further the mechanism of the hematologic findings, radiochromium-labeled compatible allogeneic platelets, erythrocytes, and 125I fibrinogen were infused into ten infants with this syndrome. In all four patients into whom labeled platelets were infused, platelet life-span was shortened. External counting and scintiscan failed to demonstrate accumulation of radioactivity in the kidneys, suggesting that platelet deposition in this organ may not account for the persistent thrombocytopenia. In the three patients in whom erythrocyte survival was studied, the life-span of transfused cells was shortened, and external counting and scintiscan revealed a pattern compatible with sequestration in the reticuloendothelial system. Evidence for associated intravascular hemolysis was demonstrated. The clearance from the plasma of 125I fibrinogen was not rapid, and no excessive accumulation of 125I fibrinogen could be demonstrated in the kidneys. These findings suggest that when the diagnostic triad of the hemolytic-uremic syndrome of infancy is seen, the renal lesion is already established and continuing intravascular coagulation is not a feature.

Hypertension as the major problem of idiopathic arterial calcification of infancy

We describe two infants with severe hypertension associated with idiopathic arterial calcification of infancy. In both children, blood pressure control was refractory to aggressive antihypertensive therapy. There was radiologic and laboratory evidence of renovascular disease requiring the use of specific renin antagonists and later nephrectomy, resulting in moderate improvement of hypertension. Although on occasion there is a familial incidence, in the vast majority of cases the diagnosis hinges on a high index of suspicion.

Management of severe hypertension in childhood Takayasu's arteritis

Six children presented with severe hypertension caused by Takayasus 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.

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.

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.

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.

LOW MOLECULAR WEIGHT HEPARIN IN THE SUCCESSFUL TREATMENT OF A SPONTANEOUS AORTIC THROMBOSIS IN A NEONATE

A successfully treated case of a spontaneous aortic thrombosis in a neonate is described as an illustration of the advantages of using low molecular weight heparin (LMWH) over unfractionated heparin (UFH) for anticoagulation therapy. A 5-day-old neonate presented with an abdominal aortic thrombosis detected by echocardiography. Intravenous UFH was commenced following thrombectomy. Poor venous access made monitoring the anticoagulation therapy problematic. Subcutaneous LMWH was substituted for UFH. It requires substantially less monitoring and no intravenous access, has fewer side effects, and allows for much earlier discharge from the hospital. Extensive investigation for a hypercoagulable state revealed no definite cause for the thrombus. The findings of homozygosity for a methylene tetrahydrofolate reductase (MTHFR) mutation and a mildly elevated homocysteine level are interesting but unlikely to account for the thrombotic event in this case. Anticoagulation with LMWH proved effective and more convenient than using UFH.

Right-Sided Endocarditis Due to Salmonella typhi

Abstract. A case of right-sided endocarditis due to Salmonella typhi is described involving a native tricuspid valve in a child who was human immunodeficiency virus negative with no evidence of intravenous drug addiction. The patient had classic features of typhoid and tricuspid regurgitation without clinical evidence of bacterial endocarditis. Transthoracic echocardiography confirmed the tricuspid regurgitation. However, transesophageal echocardiography was necessary to demonstrate the vegetations affecting the tricuspid valve leaflets that made possible the diagnosis of endocarditis. The infection was cured with intravenous ceftriaxone and oral amoxicillin.

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.

Atrioventricular septal defect and type A postaxial polydactyly without other major associated anomalies: a specific association.

Four children are described, (three black and one white, two boys and two girls) with type A postaxial polydactyly. All four of them, in addition, had either a partial or complete atrioventricular septal defect (AVSD). None of these children had associated major malformations. Minor anomalies were observed (e.g., two patients with hypersegmentation of the sternal segments, one patient with undescended testes, one patient with hypoplastic lumbar vertebra, and one patient with a degree of craniofacial abnormality). Chromosome analysis was carried out for three of the four patients, and was normal in all of them. It is suggested that there is a specific association between type A postaxial polydactyly and the AVSD found in each of these patients. This picture does not conform to, but bears some resemblance to, the Ellis-van Creveld syndrome.