IP CME  - Program Guidelines

Editorials

IP 2000—The Globalization of Information for Pediatricians

Fima Lifshitz
Editor-in-Chief, International Pediatrics
Chief of Staff, Miami Children's Hospital, Miami, Florida USA

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Management of Otitis Media with Effusion in the Next Century

Otto Ramos
Division of Infectious Diseases, Miami Children's Hospital, Miami, Florida USA

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Urinary Tract Anomalies and Congenital Heart Disease

Richard M. Zakheim
Division of Cardiology, Miami Children's Hospital, Miami, Florida USA

In the paper entitled "Silent Anomalies of the Urinary Tract in Children With Congenital Heart Disease,"1 Drs. Cil and Bostan recommended routine cineurograms after cardiac catheterization and angiography in children. This actually was a common practice in the United States at one time; however, it was realized that many of these hastily acquired images were misleading and led to unnecessary studies.2 In the Baltimore-Washington infant study, a regional case study of 4390 live born infants, it was found that the incidence of urinary tract anomalies in nonsyndromic malformations of the heart was 1% excluding five cases of hypospadias which should be clinically obvious. Of the 13 remaining cases cited, 10 were hydronephrosis, 1 child had agenesis of the kidney, and 1 child had an ectopic kidney.3 There is, of course, a much higher incidence of urinary tract anomalies in children with chromosomal anomalies and syndromes, but these should be clinically obvious. Currently, in the United States, the majority of cardiac catheterizations are done for interventional rather than diagnostic reasons and the kidneys are rarely visualized; this rarely leads to significant clinical problems. If one suspects a renal anomaly, it can easily be ruled out without radiation by means of a renal ultrasound.

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Review Articles

Tilo Gerhardt

Abstract

In 1992, the American Academy of Pediatrics recommended to place infants to sleep in the supine position. From 1992 to 1996, the incidence of sleeping in the prone position decreased from 70% to 24%. This change in sleeping position was followed by a decrease in SIDS rate from approximately 1.4 per 1000 live births before the recommendation to 0.7 per 1000 live births in 1997. This dramatic decrease in SIDS rate achieved by a very simple intervention challenges the validity of the apnea hypothesis of SIDS. This hypothesis postulates abnormalities in cardiopulmonary control as a cause of SIDS. These abnormalities, however, should not disappear by changing an infant’s sleeping position. Also, the rationale for home monitoring of infants believed to be at risk for SIDS has now to be reexamined. Traditional risk factors for SIDS, such as race, socioeconomic status, season of the year, being a sibling of a SIDS infant, maternal drug abuse, and low birth weight are largely explained by an adverse postnatal environment and harmful child rearing practices rather than by abnormalities in cardiorespiratory control. Prevention efforts, therefore, should be aimed at educating mothers about the known risk factors of SIDS so they can avoid a harmful environment for their infants. These efforts need to be concentrated on the socioeconomically deprived who are least aware of these risks. Int Pediatr. 1999;14(4):199-203.

Key words: infant sleeping position, sudden infant death syndrome, SIDS, apnea hypothesis, risk factors

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Neonatal Seizures

Richard Koenigsberger

Abstract

The classication of neonatal seizures has been evolving since the 1960s when polygraphic monitoring of infants was introduced. During the 1970s and 1980s, the term "subtle seizure" was introduced to explain almost every sort of neonatal adventitious movement that was not clearly a focal or a multifocal clonic seizure. Video EEG, first used in the nursery in the late 1980s, revealed that most movements previously labeled subtle seizures usually did not have EEG correlation. Even with this evidence, the use of the term subtle seizure persists. This author prefers calling these phenomena simply abnormal movements until their exact genesis is known. These movements often presage a poor prognosis. Because of the uncertainties of what truly constitutes a true seizure, practitioners still rush to give immediate benefit-of-the-doubt therapy, even though there queries whether seizures themselves can cause brain injury in newborn subjects leading to subsequent neurological sequelae. The effectiveness of the two most common agents used for therapy, phenobarbital and phenytoin, is also being questioned. It is hoped that ongoing animal research and clinical trials will produce more effective agents for the pharmacological treatment of seizures. On thing seems clear: prognosis is related to the underlying cause of the seizures. Int Pediatr. 1999;14(4):204-207.

Key words: neonatal seizure, subtle seizure, abnormal movement, excitatory amino acid (EAA), phenobarbital

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Consequence and Treatment of Refractory Status Epilepticus

Gregory L. Holmes, James J. Viviello, Jr.

Abstract

Status epilepticus is a serious, life-threatening emergency that occurs commonly in pediatric patients. Agents such as diazepam, phenytoin, or phenobarbital are usually effective in terminating seizure activity. Refractory status epilepticus lasting longer than 30 minutes requires additional intervention. There is not yet a consensus as to how to treat refractory status epilepticus. Pentobarbital has been the most commonly prescribed agent for the management of refractory status epilepticus in children. Midazolam and propofol are increasingly being used. The prognosis of refractory status epilepticus is poor, but some children do surprisingly well. Int Pediatr. 1999;14(4):208-212.

Key words: status epilepticus, midazolam, pentobarbital, valproate

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Nutritional Care in Childhood Cholestasis

Maria Eugênia F. A. Motta, Ana Lúcia P. Starling, Francisco J. Penna

Abstract

This article reviews the causes of malnutrition and the effects of supplementation on the nutritional status of children with cholestasis. A comprehensive review of the literature of the last 10 years by the Medline system was made. Children with cholestasis develop variable degrees of malnutrition. Periodical anthropometric measurements and laboratory evaluation must be done to detect malnutrition in its earliest stages. Specific nutrient deficits, particularly those dependent on fat absorption, are usually present though deficits of other nutrients may also occur. Appropriate supplementation of nutrients must be prescribed early and continued throughout all stages of the illness. Int Pediatr. 1999;14(4):213-220.

Key words: cholestasis, malabsorption, malnutrition, nutritional status

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Articles

Management of Otitis Media with Effusion with Prednisone in Combination with Antibiotic

Stephen Berman, Karen Wu, Robert Roark

Abstract

This retrospective study assessed the clinical outcomes of children with middle ear effusions present for 6 weeks or longer that were treated with combination prednisone-antimicrobial therapy. Among the 149 children treated with combination therapy with a follow-up visit, 95 (63.8%) improved (87 had complete resolution, 8 had partial resolution) and 36.2% failed (41 had no resolution and 13 developed acute otitis media). The stepwise logistic regression analysis for improvement incorporated two factors into the model: insurance status (odds ratio 0.26 95%, CI 0.08-0.81) and child care (odds ratio 0.71 95%, CI 0.51-0.98). Int Pediatr. 1999;14(4):221-224.

Key words: otitis media with effusion, persistent middle ear effusion, corticosteroids, outcomes

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Silent Anomalies of the Urinary Tract in Children with Congenital Heart Disease

Ergün Cil, Özlem Bostan

Abstract

The purpose of this study was to determine the incidence of urinary tract anomalies (UTA) in children with congenital heart disease (CHD). We studied 295 patients undergoing angiocardiograms for diagnosis of CHD. A cineurogram was obtained in all patients in order to detect unsuspected UTA. We found UTA in 55 of 295 patients (18.6%). Pyelo-chaliceal dilation was found in 25.5%, malrotation in 20%, hydronephrosis in 14.5%, ectopic kidney in 14.5%, hydro-ureteronephrosis in 12.7%, double collecting system in 9.1%, and ectopic ureter in 3.6%. In our study, the highest incidence of UTA was found in CHDs with left-side stenosis like aortic stenosis or coarctation of aorta (30.6%) and the lowest incidence was in those with left-to-right shunt (13.6%). The postangiocardiographic cineurogram appears to be an inexpensive, easy, and useful method of screening for UTA in children with CHD. Int Pediatr. 1999;14(4):225-228

Key words: congenital heart disease, urinary tract anomaly, cineurography

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Clinical Articles

Nasal Septal Abscess: A Case Report

Rafael Santiago, Pedro Villalonga, Andrea Maggioni

Abstract

We report a case of nasal septal abscess (NSA) secondary to ethmoid sinusitis in an 11-year-old boy. The signs and symptoms, etiological agents, treatment and complications are discussed. We report this case to alert the pediatrician of this rare condition. Int Pediatr. 1999;14(4):229-231.

Key words: nasal septal hematoma, nasal septal abscess, sinusitis, nasal obstruction, saddle nose deformity

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Clinical Findings in Untreated Classic Phenylketonuria

Santiago Restrepo, Harry Aguero, Parul Jayakar, Alejandro Jose Luis, Israel Alfonso

Abstract

Phenylketonuria (PKU) is usually detected in the neonatal period with the use of Guthrie test. We present a 3-year-old girl whose diagnosis of PKU was established at 2½ years of age. She presented with severe learning disability, poor behavior control, and aphasia. A urine phenylpyruvic acid test using ferric chloride done in Paraguay at 5 days of age was normal. Physical examination was remarkable for a very light skin color and red-white hair. The patient’s mother has dark skin and black hair an the father has light skin and brown hair. Neurological examination revealed mental retardation and hyperactivity. A low phenylalanine diet was started and her behavior and vocabulary improved. This case report describes the clinical manifestations of untreated classic PKU and confirms that ferric chloride urine test has a low sensitivity and should not be used to exclude PKU. Int Pediatr. 1999;14(4):232-234.

Key words: phenylketonuria, ferric chloride test, hyperactivity

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The Child with Tip-Toe Gait

Michael Tidwell

Abstract

Idiopathic toe walking (ITW) is a common finding in otherwise normal children who walk on feet held in equinus position. Parents bring the child to medical attention some time after the age at which the child begins to walk. It is the role of the physician or surgeon to identify the child with ITW as compared to the child with other pathologic forms of toe walking. The child with ITW begins walking in equinus as his or her initial independent pattern of gait. Toe walking developing later usually has a pathologic etiology. Children with ITW may be able to rock back into a foot flat position when cued, but resume "toe walking" preferentially. Neurological examination is normal with no cutaneous signs of spinal dysrhaphism. Physical examination may reveal mild to moderate contracture of the Achilles tendon. Diagnosis of ITW is made by exclusion and is frequently determined on the basis of history and physical examination alone. When necessary, laboratory tests for inflammatory joint disease or muscular dystrophic etiologies are obtained. Radiographic, ultrasonic, and magnetic resonance imaging may be useful. Electrodiagnostic and computerized gait analysis are occasionally utilized. Treatment of ITW is usually indicated in the child who continues to toe walk after the age of 2 years and certainly if over the age of 3 years. Treatment varies across the country but usually includes some combination of exercises, casts, and braces. Surgery is rarely indicated. Spontaneous resolution does not always occur, but it is rare to see a child with ITW after school age. Int Pediatr. 1999;14(4):235-238.

Key words: ideopathic toe walking, tip-toe gait

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Book Review

Diseases of the Small Intestine in Childhood. Walker-Smith J, Murch S. Isis Medical Media Ltd., 1999, 412 pp. $175.00, ISBN: 1-901865-0307.

Small intestine diseases account for one of the major causes of morbidity and mortality in children worldwide. Conditions such as gastroenteritis are encountered daily in every pediatric practice. In the past 2 decades our understanding of gastrointestinal disorders has evolved enormously and gastroenterology as emerged as a solid specialty in pediatrics. The authors of this book have been major contributors to this field. With the fourth edition of this book, we appreciate the continuity of their efforts both as researchers and as pediatricians. Drs. Walker-Smith and Murch have been acknowledged for their vast knowledge of immunology principles applied to gastroenterology.

This book is didactically divided into two sections. The first section is composed of five chapters and includes a comprehensive review of the anatomy, histology, and physiology of the small intestine. In addition, this section has an excellent chapter about the mechanisms involved in malabsorption. The second section, consisting of twelve chapters, is a detailed description of common clinical problems. Each chapter begins with a brief and interesting chronological review of the disorder. The description of the physiopathology of each condition is very detailed. The explanation of the immunologic mechanisms involved in the production of the disease of the small intestine is outlined very clearly. The authors give the reader not just their personal experience in the approach, examination, and management of the patient but also the opinion of other authors around the world. The graphics and diagrams used in this book make it easy to understand the text and the mechanisms outlined. This book also provides extensive references including updated and historical bibliographies.

The number of books regarding the gastrointestinal tract in children are few. It is even more difficult to find literature in pediatric gastroenterology that applies so well to clinical practice. This book is definitely an important resource not just for pediatric gastroenterologists, but also for pediatricians, residents, and medical students.

Marco Danon, MD
Director, Medical Education
Miami Children’s Hospital, Miami, Florida USA

Eric Hernandez, MD
Pediatric Resident
Miami Children’s Hospital, Miami, Florida USA

 

Abstracts from the Literature

Immune thrombocytopenic purpura ITP. Imbach P, Kühne T. Vox Sang. 1998;74(Suppl 2):309-314. Immune thrombocytopenic purpura ITP is characterized by early platelet destruction due to an imbalanced immune response. In acute ITP, a transient increase of HLA-DR molecules has been detected while in individuals with chronic ITP, in addition, increased serum concentrations of IL-2 and other cytokines reflecting in vivo T-cell activation have been observed. Clinically, the hemorrhagic manifestation of ITP rather than the platelet count should define the indication for active intervention. In a staging system a patient with stage III has bleeding signs and platelet counts below 10 or 20 x 109/L and needs treatment, a patient with stage II should be treated on an individual level (prevention of bleeding) and a patient with stage I (no bleeding, platelet count above 50 x 109/L) should be observed only.

Comment: Idiopathic (immune) thrombocytopenic purpura (ITP) is an acquired autoimmune disease of both children and adults. The disease affects approximately 10 individuals per 100 000 annually in the United States, and about 50% of those affected are children. The disease is an acute, usually self-limiting disorder, with distinct clinical manifestations in children and adults. In childhood ITP, the peak age is 4 to 8 years, and in most children, the disease is self-limited with spontaneous recovery occurring in several weeks to several months. Girls and boys are equally affected.1,2

Treatment for acute ITP remains controversial, and its goal is to prevent life-threatening complications such as intracranial bleeding, and to maintain a platelet count over 20 to 30 x 109/L with a minimum of toxicity. The treatment may include single or combination therapy with corticosteroids, intravenous immunoglobulin (IVIG), anti-D, and splenectomy.1,2

The primary choice is often corticosteroids. Corticosteroids are relatively inexpensive, can be given orally, are not a blood product, and the toxicity is tolerable when the duration of treatment is limited. Steroid therapy leads to a more rapid rise in platelet count than no therapy. Steroids also effect a shortening of the bleeding time; however, corticosteroid therapy has had no effect on the duration of ITP, and other studies have also been unable to show a decrease of intracranial hemorrhage complication in the treated group.1,2

Infusion of high dose intravenous methylprednisolone has been reported to be an effective way of managing children with chronic ITP or when used for children as initial therapy in severe, life-threatening bleeding, alone or in combination with IVIG; the rate of response was equivalent to that of IVIG and is less expensive.1,2

Intravenous immunoglobulin G (IVIG). In 1981, P. Imbach and co-workers first reported the successful use of high dose intravenous pooled immunoglobulins for the treatment of acute ITP in a small heterogenous group of 13 children. Within a few years, IVIG was being used extensively in children and adults with acute or chronic ITP. IVIG usually leads to a rapid rise in platelet count after the initial course of therapy; however, it is very expensive and may have adverse side effects.1-4

Anaphylaxis may occur in patients who are totally deficient in IgA, in whom IgA-depleted preparations should be used. Viral inactivation steps in the purification process of IVIG have minimized the risks of infectious complications.1,2

Anti-D. In 1983, A. Salama and co-workers reported platelet responses in Rh(D)-positive ITP patients treated with anti-D. Anti-D is a plasma-derived immunoglobulin (IgG fraction) prepared from donors selected for a high titer of Rho(D) antibody. The role of anti-D in acute ITP is similar to IVIG in platelet response, and is less expensive. The main adverse reaction with anti-D, some degree of hemolysis, Coombs reaction, is inevitable due to the binding of anti-D antibody to Rh-positive erythrocytes, which most of the time, do not require medical intervention. Other adverse reactions to anti-D are rare and generally mild. Hypersensitivity reactions are rare. Anti-D content of IgA is low.1-4

Anti-D is less expensive than IVIG and is much easier to administer, requiring only a brief intravenous injection. Anti-D has an almost equivalent response rate to IVIG infusion. The indications for anti-D are the same as those for IVIG. The limitations for anti-D treatment include its inapplicability to Rh(D)-negative individuals, dose-limiting hemolytic anemia, slower onset of response, and lower increments in the platelet count. In splenectomized patients, anti-D efficacy may be also limited.1-4

In patients with ITP, the decision of whether to intervene with corticosteroids, IVIG, or anti-D treatment, remains controversial, especially when symptoms are mild or the platelet count is greater than 20 x 109/L.1-4

Platelet transfusions have little benefit in ITP. After a transfusion, there is usually no significant rise in platelet count, although exceptions occur. Splenectomy is reserved for refractory thrombocytopenia with life-threatening hemorrhage in acute ITP or after recurrent severe thrombocytopenia in chronic ITP.1,2

In 1992, "Guidelines for Management of Idiopathic Thrombocytopenic Purpura" were published by O.B. Eden and J.S. Lilleyman, on behalf of the British Paediatric Haematology Group. Different issues, questions, and recommendations were discussed, such as the need for bone marrow aspiration and other laboratory tests, the need for hospitalization of children, the approaches for treatment with steroids or intravenous IVIG, chronic ITP, and the risks of splenectomy, life-threatening hemorrhage, and late sequelae of ITP.5

In 1996, the American Society of Hematology (ASH) developed practice guidelines for the diagnosis and management of patients with ITP in both children and adults. These guidelines were developed from an extensive review of the literature and therapeutic strategies that were commonly employed prior to 1994. The panel did not fully take into account newer developing therapies such as anti-D.3,6

The panel recommended that children with platelet counts greater than 30 x 109/L should not be hospitalized and do not routinely require initial treatment if they are asymptomatic or have only minor purpura.6 Children with platelet counts less than 20 x 109/L and significant mucous membrane bleeding should be treated with specific regimens of IVIG or glucocorticoids.6 Children with severe, life-threatening bleeding should be hospitalized and receive conventional critical care measures, along with treatment for ITP, such as platelet transfusions, high-dose intravenous glucocorticoid therapy and IVIG, alone or in combination.6

The panel also concluded that glucocorticoids or IVIG are appropriate as initial therapy for children with platelet counts of less than 10 x 109/L and minor purpura, and such patients should be treated.6 This conclusion created strong controversy among American and British pediatric hematologists, most of whom do not routinely recommend specific initial treatment for children with severe thrombocytopenia and only minor purpura. Even among the ASH panel members, only 5 out of 6 members responded in favor of appropriate treatment for such patients.7,8

The debate was considered to have a constructive outcome, opening the opportunity to conduct further clinical trials that "will resolve and provide firm evidence on the most appropriate management for children with ITP."8

Julia M. Retureta-Soler, MD
Department of Pediatrics
Jackson Memorial Hospital, Miami, Florida USA

© 1999 by Miami Children’s Hospital.