Ventricular dysfunction clinical research in infants, children and adolescents

These two issues of Progress in Pediatric Cardiology comprehensively illustrate the wealth of currently available information on the pathophysiology of heart failure, age-related myocardial responsiveness, energy metabolism, cardiopulmonary interactions, the pressure-volume relationship, the systemic inflammatory response, the management of heart failure, pediatric pharmacology, the use of heart failure therapies including digoxin, ACE inhibitors, beta-adrenergic blockers, inotropic agents, diuretics, vasodilators, calcium sensitizers, angiotensin and aldosterone receptor blockers, growth hormone, and future gene therapy. The etiology and course of ventricular dysfunction in children is poorly characterized. Furthermore, many changing developmental properties of the pediatric myocardium and differences in the etiologies of ventricular dysfunction in children compared with adults are illustrated in these articles, invalidating the concept that children can safely be considered small adults for the purpose of understanding heart failure pathophysiology and treatment. However, these articles reveal that strikingly little research in children with ventricular dysfunction exists in terms of well-designed large-scale studies of the epidemiology or multicenter controlled clinical therapeutic trials. A future research agenda is proposed to improve understanding etiologies, course and treatment of ventricular dysfunction in children that is based on organized and funded cooperative groups since no one pediatric cardiac center treats enough children with a particular etiology of ventricular dysfunction. In conclusion, significant understanding of basic mechanisms of pediatric ventricular dysfunction and effective therapies for adults with ventricular dysfunction exist. A multicenter pediatric cardiac ventricular dysfunction network would allow improved understanding of diseases and treatments, and result in evidence-based medicine for pediatric patients with ventricular dysfunction.     

Angiotensin-converting enzyme inhibitor fetopathy: long-term outcome

Fetal exposure to angiotensin-converting enzyme inhibitors (ACEIs) is associated with increased neonatal morbidity and mortality. Long-term follow-up of three patients with fetal ACEI exposure revealed impaired renal function in two, severe hypertension and proteinuria in one and isolated polycythaemia in all three. Careful long-term follow-up of children with ACEI fetopathy is recommended.     

Cardiovascular drugs in children: Angiotensin-converting enzyme inhibitors

Summary Angiotensin-converting enzyme inhibitors are potent vasodilators acting by inhibition of production of the vasoconstrictor angiotensin II. In adults, they are used for treatment of systemic hypertension and congestive heart failure and investigated for treatment of primary pulmonary hypertension.In infants and children, saralasin and captopril were found to be useful in treatment of systemic arterial hypertension, especially when associated with high plasma renin activity. Captopril has failed in the treatment of congestive heart failure associated with complex congenital heart diseases and in most cases of primary pulmonary hypertension. It has a clear beneficial effect in coarctation of the aorta and may have such an effect in endomyocardial diseases and ventricular septal defect.In adults, serious side effects have limited the use of captopril. New converting enzyme inhibitors, devoid of a sulfhydryl group, are expected to have a better safety profile.     

A novel method for indexing echocardiographic left ventricular mass in infants, children and adolescents: Evaluation of obesity-induced left ventricular hypertrophy

BACKGROUND: Measurement of left ventricular mass (LVM) is important to the diagnosis of left ventricular hypertrophy in children with various cardiovascular diseases. The purpose of this study was to determine the most appropriate method for standardization of LVM and to evaluate obesity-induced left ventricular hypertrophy in children across the entire age range, from infancy through adolescence. METHODS: We studied 928 children and adolescents (527 males, 401 females), aged 0-17 years, who were classified into two groups by degree of obesity. Left ventricular mass was calculated by M-mode echocardiography using the formula of Devereux et al. and was indexed using body size (body length, bodyweight or body surface area) raised to a non-integer power using logarithmic transformation of measurements in children without obesity. RESULTS: The body length, bodyweight and body surface area exponents were 1.85, 0.88 and 1.15, respectively, in males, and 1.72, 0.82 and 1.08, respectively, in females. Whereas indexing of left ventricular mass by body length both in males and in females revealed significant differences between the two groups, indexing using bodyweight or body surface area exponents did not manifest left ventricular hypertrophy induced by obesity. CONCLUSION: It is suggested that applying body length exponents 1.85 in males and 1.72 in females is an appropriate method for indexation of LVM in children and adolescents. This method is particularly useful for the evaluation of left ventricular hypertrophy in children.     

Cholelithiasis in infants, children, and adolescents

During the past two decades, cholelithiasis has been recognized in increasing numbers of pediatric patients. This diagnosis should be considered in the event of upper abdominal complaints, particularly when one or more risk factors are evident. The etiology may be unknown or may be related to risk factors, including hemolytic conditions. In recent years, it has become evident that approximately 80% of gallstones in children are not due to hemolytic disease and that the remaining 20% are related to recurring hemolysis. The diagnosis of gallstones is best confirmed with ultrasonography. Routine ultrasonographic evaluation should be performed at intervals for all children who received TPA for more than 4 weeks, particularly those who have had ileal resection or have had chronic enteritis (Crohn disease). Cholecystectomy is the procedure of choice for symptomatic children with cholelithiasis, regardless of age. Cholecystectomy is recommended for the asymptomatic child younger than 3 years of age when echogenic shadows have been present for at least 12 months following resumption of oral feedings or when the gallstones are radiopaque. Also, cholecystectomy is advised for asymptomatic children who are older than 3 years of age if ultrasonographic studies confirm that echogenic foci with shadowing are true stones and not echogenic sludge. Complications of common bile duct obstruction, pancreatitis, perforation with bile peritonitis, and life-threatening sepsis may thus be prevented. Morbidity and mortality following cholecystectomy are expected to be relatively low in the pediatric age group.     

Suicidality in children and adolescents: a review

Suicide and suicide attempts by children and adolescents continue to be a major public health problem in the United States. Major risk factors include a previous suicide attempt, a mood disorder, a family history of suicide, a parental psychiatric disorder, and a history of sexual abuse. Genetic vulnerability appears to be a factor as well. Efforts to prevent subsequent attempts at suicide have not generally been successful, and many children and adolescents are not receiving treatment for the mood disorders and other psychiatric conditions that are risk factors. Evaluation of suicide risk should be carried out through multiple informants, not simply the adolescent or the parents, although a thorough clinical interview of the adolescent is essential.     

Octreotide therapy for chylothorax in infants and children: A brief review.

OBJECTIVES: We review physiology and pharmacology relating to the use of octreotide for chylothorax in infants and children. We review the published experience of octreotide dosing in this context. DATA SOURCE: Systematic review of the literature, including PubMed (English-only journals), citations from relevant articles, major textbooks, and personal files. CONCLUSIONS: Octreotide has been used as a successful therapeutic adjunct in a small number of neonatal cases and a larger number of pediatric cases. No consensus has been reached as to the optimal route of administration, dose, duration of therapy, or strategy for discontinuation of therapy. We suggest using higher doses (80-100 microg/kg/day) and initiating therapy early rather than using a low initial dose with upward titration. Duration of therapy required to elicit a significant response may vary between patients.     

Lactose intolerance in infants, children, and adolescents

The American Academy of Pediatrics Committee on Nutrition presents an updated review of lactose intolerance in infants, children, and adolescents. Differences between primary, secondary, congenital, and developmental lactase deficiency that may result in lactose intolerance are discussed. Children with suspected lactose intolerance can be assessed clinically by dietary lactose elimination or by tests including noninvasive hydrogen breath testing or invasive intestinal biopsy determination of lactase (and other disaccharidase) concentrations. Treatment consists of use of lactase-treated dairy products or oral lactase supplementation, limitation of lactose-containing foods, or dairy elimination. The American Academy of Pediatrics supports use of dairy foods as an important source of calcium for bone mineral health and of other nutrients that facilitate growth in children and adolescents. If dairy products are eliminated, other dietary sources of calcium or calcium supplements need to be provided.     

Practitioner review: The effectiveness of systemic family therapy for children and adolescents

BACKGROUND: Systemic family therapy has become a widely used intervention in child and adolescent mental health services over the last twenty years. METHODS: This paper reviews the development of systemic family therapy, briefly describes the theory and techniques associated with the most prominent contemporary strands of systemic practice, and examines the empirical justification for using systemic family therapies with children and adolescents. RESULTS: There is a paucity of well-designed randomised controlled trials of systemic therapies with children and adolescents and those trials that do exist evaluate older structural and strategic therapies. Methodological limitations of existing research include the use of unrepresentative participants, small sample sizes and wide age ranges. There is a lack of credible no-treatment or alternative treatment controls, tests of clinical as opposed to statistical significance, and conceptually relevant outcome measures that examine underlying interactional mechanisms. The term 'family therapy' encompasses a wide range of interventions and it is not always clear what treatment intervention has been delivered. Nevertheless, there is good evidence for the effectiveness of systemic family therapies in the treatment of conduct disorders, substance misuse and eating disorders, and some support for their use as second-line treatments in depression and chronic illness. CONCLUSIONS: Systemic family therapy is an effective intervention for children and adolescents but further well-designed outcome studies are needed using clearly specified, manualised forms of treatment and conceptually relevant outcome measures.     

Diabetic nephropathy in children and adolescents: a critical review with particular reference to angiotensin-converting enzyme inhibitors

Clinical diabetic nephropathy is a well-recognized cause of increased morbidity and mortality in patients with type 1 diabetes. The finding that microalbuminuria predicts progression to overt nephropathy has allowed early diagnosis and preventive interventions. Several studies have demonstrated that treatment with angiotensin-converting enzyme (ACE) inhibitors slows down the rate of decline of the glomerular filtration rate in type 1 diabetes patients with established proteinuria. The renoprotective properties of the ACE inhibitor captopril extend beyond its antihypertensive effects. ACE inhibitors represent the most appropriate class of antihypertensive drugs for treating type 1 diabetes patients because of their efficacy and safety. When microalbuminuria is detected and confirmed in a diabetic child or adolescent, and if it persists despite 6-12 months of improved metabolic control, treatment with ACE inhibitors should be started, even if the child is normotensive. Careful follow-up of renal function is essential.     

Prevention of drowning in infants,children, and adolescents

Drowning is a leading cause of injury-related death in children. In 2000, more than 1400 US children younger than 20 years drowned. Most (91%) of these deaths were unintentional and were not related to boating. For each drowning death, it is estimated that at least 1 to 4 children suffer a serious nonfatal submersion event, many of which leave children with permanent disabilities. Environmental strategies, such as installation of 4-sided fences around swimming pools, and behavioral strategies, such as increased supervision of children while around water, are needed to prevent these tragedies.     

Sudden cardiac death in infants, children, and adolescents

Although SCD is relatively uncommon, its psychosocial impact is devastating. This article has reviewed the potential causes of SCD in infants, children, and adolescents. Many patients who die from SCD have identifiable cardiac disease and are known to have been at risk; however, the existence of other cardiac abnormalities, such as hypertrophic cardiomyopathy or long QT syndrome, may not be known, and SCD may be the first symptom. The authors' contention is that many of the patients in this latter group (e.g., patients who have hypertrophic cardiomyopathy or LQLTS but who have no symptoms) can be screened with a careful, accurate, and detailed history, including family history and review of systems, and physical examination. Any patient with a positive family history, positive review of systems, or positive physical examination should receive further in-depth evaluation, such as an ECG and echocardiogram. These studies permit the detection of most, if not all, of the entities potentially associated with SCD in the pediatric population.