Chronic high Epstein-Barr viral load state and risk for late-onset posttransplant lymphoproliferative disease/lymphoma in children.

Increased use of serial EBV-PCR monitoring after pediatric transplantation has led to the identification of asymptomatic patients who carry very high viral loads over prolonged periods. The significance of this high-load state is unknown. We speculated that this state may identify patients at high risk for development of late PTLD/lymphoma. We reviewed data on 71 pediatric heart recipients who had serial viral load monitoring since 1997. Chronic high-load state was defined as the presence of >16,000 genome copies/mL whole blood on > or =50% of samples over at least 6 months. Among 20 high-load carriers (eight following prior PTLD, seven with prior symptomatic EBV infection, five without previous EBV disease), 9 (45%) developed late-onset PTLD 2.5-8.4 years posttransplant (including with four Burkitt's lymphoma). Among 51 controls with low (n = 39) or absent (n = 12) loads, only 2 (4%; p < 0.001 absent/low vs. high load) developed late PTLD/lymphoma. By multivariable analysis, high-load carrier state (OR = 12.4, 95% CI 2.1-74.4) and prior history of PTLD (OR = 10.7, 95% CI 1.9-60.6) independently predicted late PTLD. A chronic high EBV-load state is not benign and is a predictor of de novo or recurrent PTLD.

Primary antiphospholipid antibody syndrome and cardiac involvement in a child.

We report a case of polyvalvar disease and recurrent thrombosis in a 2-year-old boy due to primary antiphospholipid antibody syndrome. His diagnosis was delayed, and he was treated for other diagnoses, including culture-negative endocarditis. Primary antiphospholipid antibody syndrome is rarely discussed in the pediatric cardiology literature, and this case highlights the need for early diagnosis to prevent recurrent valve dysfunction and thrombosis.

Asymptomatic giant right atrial aneurysm.

A 5-year-old asymptomatic boy was found incidentally, on a chest radiograph, to have gross cardiomegaly; further evaluation by echocardiography showed a giant right atrial aneurysm. The patient underwent successful surgical reduction of the right atrium, closure of an atrial septal defect, and tricuspid valve repair These measures were taken to prevent thrombus formation in the right atrium, prevent paradoxical embolism, and lower the risk of atrial arrhythmias. The morphologic features of the resected atrial tissue showed paper-thin wall with a central aneurysm and focal endocardial fibrosis consistent with a diagnosis of idiopathic dilatation of the right atrium.

The effect of gestational age, birth weight, and disease on troponin I and creatine kinase MB in the first year of life.

OBJECTIVES: To determine the effect of gestational age and birth weight (BW) on troponin I (TnI) and creatine kinase MB fraction (CKMB) levels during the first year of life. METHODS: Troponin I and CKMB levels were determined in infants less than 1 year of age using the Immuno I (Bayer Corp.). RESULTS: Troponin I fractions were greatest in the preterm infant; the levels decreased significantly with increasing gestational age and BW, (p = 0.008 and p = 0.005, respectively). The CKMB levels did not exhibit a significant difference between the preterm and term infant groups when assessed for the effects of gestational age or BW (p = 0.12 vs. p = 0.35). Neither TnI nor CKMB levels were significantly different between preterm survivors and nonsurvivors (p = 0.31; p = 0.34, respectively). TnI levels were elevated in critically ill patients without documented myocardial infarction, and without a comparable rise in CKMB. CONCLUSION: The higher TnI levels during the first 3 months of life may indicate programmed cell death, or apoptosis. This may be especially true in the preterm infant in which the greatest values were documented.

Echocardiographic evidence for a ductal tissue sling causing discrete coarctation of the aorta in the neonate: case report.

A neonate presented to our institution with the physical findings of coarctation of the aorta. After starting prostaglandin E1 the signs and symptoms resolved despite persistent closure of the ductus arteriosus. We present echocardiographic evidence to support the contention that a ductal tissue sling contributes to the formation of juxtaductal coarctation of the aorta.

Renal autotransplantation for renovascular hypertension caused by midaortic syndrome.

Midaortic syndrome (MAS) is a well-recognized but rare cause of renovascular hypertension (RVH). Several techniques have been described to treat RVH caused by MAS. The authors recently treated two children with MAS and RVH. In both patients the right kidney had two renal arteries. A 13-year-old boy presented with severe headaches, pain in his lower extremities with exertion, and marked hypertension (blood pressure, 170/110). An aortogram demonstrated 70% narrowing of his abdominal aorta from the suprarenal region to 5 cm above the iliac bifurcation. There was significant stenosis of the celiac axis, superior mesenteric artery, and left renal artery. The right kidney had two renal arteries, and the upper pole artery was stenotic at its origin. A 10-year-old girl, known to have hypertension for several years had an aortogram that demonstrated 70% narrowing of the abdominal aorta from the suprarenal region to 3 cm above the iliac bifurcation. There was involvement of the left renal artery at its orifice. She also had two renal arteries to the right kidney with the right upper pole artery being stenotic at its origin and in the mid-portion of the vessel. Aortic reconstruction was accomplished with a polytetrafluoroehtylene (PTFE) bypass graft in each case. The first case also involved patch angioplasty of the celiac axis. In both cases, the right kidney was autotransplanted. It was removed intraoperatively, cold perfused, and the two renal arteries reconstructed followed by transplantation to the right iliac vessels. In both cases the left renal artery was reimplanted into the PTFE graft. Both patients had uncomplicated postoperative courses. The 13-year-old boy had evidence of renal ischemia in a portion of the lower pole of the autotransplanted kidney by DTPA scan. He has mild hypertension controlled with antihypertensive medication. The 10-year-old girl has a normal DTPA scan and is normotensive. MAS is a rare and challenging congenital vascular anomaly that causes RVH. In the presence of double renal arteries the technique of autotransplantation with cold perfusion and "bench" vascular reconstruction reduces the warm ischemia time and should produce satisfactory results.

Pediatric preventive cardiology: healthy habits now, healthy hearts later.

In recent years there has been a strong emphasis from both medical and nonmedical communities to improve overall cardiovascular health. Continued attention has been focused on finding improved treatment regimens, and, encouragingly, increased attention is being directed toward prevention strategies for coronary heart disease. Emphasis on heart-healthy diets, fitness, and the now commonplace free blood pressure and cholesterol screenings have become part of our daily life. Multiple studies have demonstrated that atherosclerosis begins in childhood, and increasingly, the need for prevention to begin during childhood is being highlighted. Preventive cardiology in children, however, has long relied on adult data for definitions of risk factors, study protocols, and norms. Gradually, the pediatric data is becoming available. Elevated cholesterol levels, hypertension, obesity, and other risk factors defined in adults are all areas of intense interest in the pediatric population. This brief review of preventive cardiology in children provides a look at the risk factors for adult coronary heart disease as they relate to children, the current recommendations (and associated controversies), and a summary of some of the most recent clinical trials.

Variability in response to a low-fat, low-cholesterol diet in children with elevated low-density lipoprotein cholesterol levels.

The reduction of dietary cholesterol and fat lowers low-density lipoprotein cholesterol (LDL-C) and reduces risk of coronary heart disease in adults. The purpose of this study was to determine the individual variability of response of serum lipid and lipoprotein levels to a low-fat, low-cholesterol diet in children with elevated LDL-C levels. Thirty-two children (2 to 16 years of age) enrolled in a diet modification program, who had LDL-C levels of at least 110 mg/dL but normal triglyceride levels for their ages, were studied. Lipid levels and dietary nutrients were analyzed at the time of admission, and final assessments were made at least 3 months after entry. There was a significant correlation, for the group as a whole, between change in LDL-C concentration and change in grams of dietary saturated fat; however, there was marked individual variability in LDL-C response. There were no significant correlations between changes in LDL-C levels and changes in either total fat, polyunsaturated fat, or cholesterol intake. It is concluded that modest decreases in dietary saturated fat coincide with a lowering of LDL-C concentration, over a short term, in many children, but the degree of lowering varies considerably from one child to another. This variability is consistent with the concept that response of serum lipid levels to dietary changes is modified by genetic, metabolic, and other, as of yet, undefined variables.

Hepatic cyst associated with ventriculoperitoneal shunt in a child with brain tumor.

A 12-year-old boy with a thalamic grade IV astrocytoma and ventriculoperitoneal (VP) shunt developed epigastric pain and symptoms of increased intracranial pressure. The SGOT and alkaline phosphatase levels were markedly elevated and the radiological studies showed a cyst in the right lobe of the liver, extending to the porta hepatis. Simple repositioning of the shunt resulted in complete resolution of clinical findings and disappearance of the cyst. Although abdominal pseudocysts associated with VP shunts have been reported, this is the first report of a cyst involving liver and causing hepatic dysfunction.