Stage I palliation for hypoplastic left heart syndrome in low birth weight neonates: can we justify it?

OBJECTIVE: Although the outcome of cardiac surgery in neonates with low birth weight (LBW) has improved, LBW remains a risk factor for surgical palliation. Few surgical series of LBW patients include those with hypoplastic left heart syndrome (HLHS). To identify variables associated with poor outcome in this group, we reviewed our experience with patients with HLHS and LBW who underwent Stage I Norwood palliation. METHODS: Between January 1998 and December 2000, 20 consecutive LBW (<2500 g) neonates with HLHS (n=13) or HLHS variant (n=7) underwent surgical palliation. Retrospective review of all patient data and analysis to identify risk factors was performed. RESULTS: Mean age at surgery was 5.1+/-4.6 days (range 1-17), mean weight was 1.98+/-0.44 kg (range 1.1-2.5), including nine patients under 2 kg. Ten patients were born at <35 weeks gestation. Anatomic diagnosis included HLHS in 13 patients (10 with aortic atresia), unbalanced atrioventricular canal defect in two, double outlet right ventricle in two and other variants in three. Mean ascending aortic size was 4.0+/-1.8 mm (range 1.5-8). Associated cardiac defects were present in three patients, and a genetic syndrome and/or congenital anomaly was present in four of them. Mean circulatory arrest time was 60+/-10 min. Extracorporeal support was used perioperatively in 10 patients. Early mortality was 9/20 (45%). At a mean follow up at 22+/-10 months (range 8-38), six patients underwent stage II, and are awaiting stage III; four patients have completed their Fontan. Anatomic variant, ascending aortic size, prematurity, age at surgery, weight, duration of circulatory arrest, cardiopulmonary bypass time and associated non-cardiac anomalies were not risk factors for poor outcome whereas restrictive pulmonary venous drainage and coronary artery anomalies were associated with decreased survival. CONCLUSION: LBW newborns with HLHS and physiologic variants have an increased early surgical risk but have acceptable intermediate survival rates for subsequent palliation including Fontan. LBW and prematurity should not be contraindications to early surgical palliation.     

Intestinal amyloidosis in hamsters with visceral leishmaniasis.

Thirty hamsters about 10 weeks old were inoculated intraperitoneally with Leishmania donovani amastigotes and were serially killed after 15, 30, 45, 60, 75 and 85-90 days. Both the small and large intestines were examined grossly, and the histopathology was assessed by light and electron microscopy. The lamina propria and the submucosa of the whole length of the intestinal tract showed a progressive deposition of amyloid, selectively identified by optical and ultrastructural techniques. The presence of amyloid fibrils in the cytoplasm of plasma cells suggests that appearance of intestinal amyloidosis during visceral leishmaniasis may be the result of a pathological dysfunction of these cells. In addition to these deposits, the presence of inflammatory infiltrates containing lymphocytes, plasmocytes and macrophages confirmed the establishment of leishmaniasis. In the end-stages of the infection both vacuolar degeneration of the epithelial cells lining the lumen of the intestine and a moderate hyperplasia of lymphatic nodules was observed.     

Documento nacional de recomendaciones sobre el seguimiento del paciente con carcinoma de células renales

Objectives

This document was developed to establish directives for the follow-up of patients with renal cell carcinoma (RCC) based on the best available scientific evidence and on expert opinions, which can help urologists in the decision-making process and standardise the criteria at the national level.

Routine primary repair of tetralogy of Fallot in the neonate

Uncertainty surrounds both the timing and ideal form of early management of tetralogy of Fallot. Some centers perform early complete repair in all patients regardless of age, symptoms and morphology. Others recommend a two-stage approach involving initial palliation in symptomatic neonates and young infants and those with unfavorable anatomy (anomalous coronary anatomy or hypoplastic pulmonary arteries). Advantages of early anatomic correction include alleviation of cyanosis, normal growth and organ development, removal of stimulus for right ventricular hypertrophy and avoidance of risks associated with initial palliation. With recent advances in anesthetic, operative and postoperative management, routine primary repair of tetralogy of Fallot in the neonate and young infant can be accomplished with excellent early and mid-term results. However, long-term follow-up is necessary to assess the impact of early repair on late right ventricular function, arrhythmias and need for reintervention.