Congenital toxoplasmosis is a preventable and treatable disease with predominant neurological and ophthalmologic manifestations. These manifestations and approaches to diagnosis, treatment, and prevention of this infection are considered. Outcomes with and without treatment are emphasized. The following also are discussed: recent advances in understanding the organism; its life cycle, epidemiology, pathogenesis, and pathology; and areas in which improvements in diagnosis, treatment, and prevention are needed.
OBJECTIVE To report the clinical experience of three patients with fever of unknown origin ultimately diagnosed as having cat-scratch granulomatous hepatitis in the absence of peripheral adenopathy. DESIGN Case-control study. SETTING Referral center at university-based referral practice. PATIENTS Three children with fever of unknown origin. Follow-up following presentation was 6 months for each patient. MEASUREMENT AND RESULTS All three patients with fever of unknown origin were diagnosed radiographically to have multiple hepatic defects. The defects were shown histologically to be granulomatous. Two of the three patients had Warthin-Starry staining bacilli in the granulomas consistent with a diagnosis of Afipia felis. All three had positive cat-scratch skin test results. CONCLUSIONS Cat-scratch disease in the absence of peripheral adenopathy is a heretofore unrecognized cause of fever of undetermined origin and may account for a small, but significant, percentage of children presenting with it.
OBJECTIVE To evaluate the incidence and clinical features of abnormal cardiac hypertrophy during treatment of infantile spasms with corticotropin. DESIGN A prospective, echocardiographic study. PARTICIPANTS Eighteen children treated for infantile spasms with high-dose corticotropin at a large tertiary children's hospital. INTERVENTIONS None. MEASUREMENTS AND RESULTS Abnormal cardiac hypertrophy was seen in 13 (72%) of 18 patients. Five of 18 patients (group 1) developed hypertrophic cardiomyopathy with asymmetric septal hypertrophy; none developed significant left ventricular outflow obstruction. Concentric left ventricular hypertrophy was seen in eight patients (group 2), whereas no changes were observed in five (group 3). CONCLUSION Abnormal ventricular hypertrophy occurs in the majority of patients treated with corticotropin for infantile spasms. In our study, many of these patients developed hypertrophic cardiomyopathy with dramatic asymmetric septal hypertrophy; however, none developed significant obstruction to left ventricular outflow. All changes were reversible, and did not require premature discontinuation of therapy.
OBJECTIVE To present the clinical and roentgenographic features of caudal duplication syndrome. DESIGN Retrospective review of the medical records and all available imaging studies. SETTING Two university-affiliated teaching hospitals. PARTICIPANTS Six children with multiple anomalies and duplications of distal organs derived from the hindgut, neural tube, and adjacent mesoderm. INTERVENTIONS None. RESULTS Spinal anomalies (myelomeningocele in two patients, sacral duplication in three, diplomyelia in two, and hemivertebrae in one) were present in all our patients. Duplications or anomalies of the external genitalia and/or the lower urinary and reproductive structures were also seen in all our patients. Ventral herniation (in one patient), intestinal obstructions (in one patient), and bowel duplications (in two patients) were the most common gastrointestinal abnormalities. CONCLUSIONS We believe that the above constellation of abnormalities resulted from an insult to the caudal cell mass and hindgut at approximately the 23rd through the 25th day of gestation. We propose the term caudal duplication syndrome to describe the association between gastrointestinal, genitourinary, and distal neural tube malformations.
To compare the safety and immunogenicity of Lederle Laboratories' (Pearl River, NY) diphtheria and tetanus toxoids and acellular pertussis (DTaP) vaccine with diphtheria and tetanus toxoids and whole-cell pertussis (DTwP) vaccine when administered simultaneously with measles-mumps-rubella (MMR) vaccine and trivalent oral poliovirus (OPV) vaccine at 15 to 16 months of age.Randomized and double-blind.Two general pediatric practices.Ninety-seven infants, aged 15 to 16 months, who had received three previous DTwP immunizations. SELECTION PROCEDURES AND INTERVENTIONS: Healthy children received the DTaP or DTwP vaccine. Infants received the MMR vaccine at a separate site and the OPV vaccine concurrently. Blood was obtained on day 0 and at 6 weeks. Adverse events were recorded by parents at specified times after immunization.Within 3 days of immunization, DTaP vaccine recipients had less fever, drowsiness, and irritability (P = .01, .04, .01, respectively). They also experienced less tenderness, erythema, and induration (.001, .001, and .002, respectively). There was no difference in the frequency of adverse reactions 6 to 14 days after immunization. Enzyme-linked immunosorbent assays were used to determine all antibody values. Antibody responses to filamentous hemagglutinin and pertussis toxoid were significantly greater in the DTaP group (P = .0001 and .02, respectively). Immune responses to the other measured antigens were similar.Simultaneous administration of the Lederle DTaP with MMR and OPV vaccines did not interfere with antibody response to pertussis antigens measured or measles, mumps, or rubella viruses and was associated with fewer local and systemic adverse events during the first 3 days following immunization when compared with the simultaneous administration of the DTwP, OPV, and MMR vaccines. We conclude that the DTaP vaccine can be administered at 15 months of age concurrently with the MMR and OPV vaccines.
OBJECTIVE:To examine whether a protein-sparing modified fast diet and a hypocaloric balanced diet are safe and effective for children in an outpatient weight reduction program.DESIGN:Randomization of two groups to either diet, with follow-up at 14.5 months.SETTING:Physician or parent referral to outpatient program at Children's Hospital of New Orleans, La.PARTICIPANTS:Nineteen children, ranging in age from 7.5 to 16.9 years, weighing 45% to 131% more than the mean weight for age, sex, and height.INTERVENTION:During the first 10 weeks, 10 children were placed on a protein-sparing modified fast diet (2520 to 3360 J), and nine children and adolescents were placed on a hypocaloric balanced diet (3360 to 4200 J). Subsequently, all participants were placed on a hypocaloric diet; calories were increased from 4200 to 5040 J in a 3-month period and maintained for 1 year.SELECTION PROCEDURES:Children were assigned to one of two diets for the first 10 weeks according to their time of enrollment.MEASUREMENTS/MAIN RESULTS:Both diets produced significant weight loss during the first 6 months. However, the protein-sparing modified fast diet produced significantly greater changes in the percentage of overweight at 10 weeks (-30% vs -14%) and at 6 months (-32% vs -18%). At 10 weeks, a significant loss of adipose tissue with preservation of lean body mass occurred in the protein-sparing modified fast group. A transient slowing of growth velocity was noted at 6 months in both dietary groups compared with values at 14.5 months. Growth velocity approached normal levels at 14.5 months compared with standards for North American children. When dietary groups were combined, the initial mean blood pressure decreased significantly at all points in the study. The initial mean serum cholesterol value also decreased significantly at 10 weeks. No biochemical or clinical complications were observed.CONCLUSIONS:These hypocaloric diets appear to be safe and effective in the short-term management of pediatric obesity. However, these diets should not be used without close medical supervision.
X-linked lymphoproliferative disease (XLP) is an inherited immune defect caused by germline mutations in SH2D1A (SH2domain containing gene 1A; also known as DSHF SAP), a gene that encodes a small adaptor protein expressed in T, NK, and some B cells. The clinical features of UP are variable and include fulminant infectious mononucleosis, lymphoma, and hypogammaglobulinemia. Studies of the SH2D1A protein revealed important pathways regulating antiviral immunity. By interacting with specific cell-surface receptors, such as 2B4 (CD244), SLAM (CD150), and NTB-A, SH2D1A might regulate important aspects of immune cell function, including NK cell cytotoxicity, T-cell proliferation, and T(H)1 cytokine secretion. Abnormalities in SH2D1A amino acid composition and protein expression levels seem to cause aberrant lymphocyte activation, contributing to the dysregulated immune response that characterizes XLP.
Sir. —Discussion of the magnitude and public health importance of low exposure to lead among American children continues to focus on lead-based paint as the major source of potential ingestion. 1,2 Other sources, often related to behavioral rather than environmental factors, have been presumed unusual. Included are childhood oral exploration involving lead-containing objects, such as bullets and fishing sinkers, or parental use of lead-bearing unglazed pottery, cosmetics, or Mexican folk medicines. A concern among public health practitioners and pediatricians has been the lack of differentiation in policy recommendations to allow for geographic and demographic (cultural) variation in magnitude and source of lead poisoning. 3 Alternative Lead Exposures. —One recent study tested more than 3000 children living in low-socioeconomic areas in a large jurisdiction in southern California and demonstrated that the majority of individuals with high levels of blood lead were causally related to parental use of lead-bearing unglazed pottery and
Pediatricians are concerned about appropriate monitoring of vital functions in infants both in the hospital and at home. Besides respiratory frequency and apnea duration, most electronic monitors evaluate instantaneous heart rate and activate an auditory and visual alarm at a preset value. The choice of the low heart-rate threshold might be based on either statistical probability or on clinical significance. In this issue ofAJDC, Hodgman et al1report observations of heart rates in 14 full-term and 19 preterm infants at several chronological ages. Having selected an operational definition oftransient episode of bradycardia, they analyzed tracings collected over one night in the laboratory. The definition included episodes of fall in heart rate to an instantaneous value equal to or less than 90 beats per minute, or a fall of 30 beats per minute when associated with apnea or a decrease in transcutaneous Po2of 15 mm Hg
Sir.—We would like to report an additional case of benign neonatal sleep myoclonus1recently seen when the patient was 5 days of age. On day 3 the child developed the typical features of jerking when asleep; this lasted a few seconds with no associated features. Results of examination that included electroencephalogram when the patient was awake were normal, and no abnormality appeared on biochemical, hematologic, or infection screens. Of interest was the presence of pronounced stimulus-induced myoclonus when the patient was asleep. He was being nursed in a clear cot; when the cot was tapped, several myoclonic jerks were evident in the arms. These did not disappear with holding of the limbs. Recent pediatric neurology textbooks2,3have referred to this condition as benign neonatal myoclonus, but the paucity of information in these sources demonstrates the difficulty of keeping up with the broad expanse of knowledge in our
ObJective.-To assess the height outcome of newborns born small for gestational age.Design.-A historical prospective study.Setting.-A cohort of 1758 newborns born at a single university hospital maternity ward and subsequently examined at the military draft medical board at age 17 years.Participants.-Newborns whose weight at birth was below the third percentile were defined as small for gestational age. Their body measurements at age 17 years were compared with those of their peers who were appropriate for gestational age.Measurements/Main Results.-The adjusted mean+/-SEM height for boys born small for gestational age vs peers born appropriate for gestational age was 169.9+/-1.5 vs 175.4+/-0.8 cm (P<.0001); and for girls, 159.4+/-1.3 vs 163.1+/-0.8 cm (P<.0005). In addition, the risk for height attainment below the 10th percentile was significantly increased for newborns born small for gestational age. The adjusted odds ratio was 4.13 for boys (95% confidence interval, 1.66 to 10.25; P<.0006) and 3.32 for girls (95% confidence interval, 1.38 to 8.05; P<.0005).Conclusion.-Infants born small for gestational age may be at increased risk for short stature in late adolescence.
Sir.—Chronic sinusitis associated with nasal polyposis is extremely common in patients with cystic fibrosis (CF), often necessitating frequent sinonasal surgery. The incidence of nasal polyposis in patients with CF is reported to range from 6% to 48%.1,2The incidence of pansinusitis in children with CF has been reported to approach 100%.1,3,4Although CF is usually diagnosed within the first 2 years of life, the diagnosis may be delayed until later childhood and adolescence or early adulthood in rare cases. Cystic fibrosis usually presents as recurrent bronchopulmonary infections and gastrointestinal malabsorption. However, sinonasal disease may be the initial presenting symptom. The prevalence of patients with CF presenting with sinonasal disease has not been reported. We describe three children with CF who presented with sinonasal symptoms. The diagnosis and management of sinus disease in patients with CF will be discussed, as well as the role of sweat testing in
Sir.—The improved survival rate of very-low-birth-weight infants (<1500 g) in the United States during the past two decades has been associated with a concomitant rise in the prevalence of retinopathy of prematurity (ROP).1The findings of the Multicenter Trial of Cryotherapy for Retinopathy of Prematurity (CRYO-ROP)2confirm that cryotherapy reduces ocular morbidity in infants with ROP. With the possible emergence of retinal cryotherapy and/or laser treatment3,4as standards of care for this disease, the role of the pediatric ophthalmic consultation assumes increasing importance. The ROP examination form presented herein provides ophthalmologists, neonatologists, and nursery staff with an efficient and graphically expressive method for recording the ROP diagnosis and monitoring the disease progression. Retinopathy of prematurity is a disorder affecting the incompletely vascularized retina of premature infants. Retinal vasculogenesis begins at the optic disc at 16 weeks' gestation when blood vessels grow from the posterior pole out
• Objective. —To provide an approach toward the diagnosis of children with human immunodeficiency virus (HIV) infection in the acute care setting. Design. —Patient reports and review of recent literature. Setting. —Emergency departments or acute care clinics. Selection Procedures.—Analysis of important recent clinical publications. Interventions. —None. Results and Conclusions. —The clinical and historical findings associated with pediatric HIV infection are occasionally subtle and differ from those findings in adults. In this article, we have divided the first presentation of HIV in the acute care setting into five categories: recurrent bacterial infections, failure to thrive, unexplained organomegaly, adolescents with HIV, and late presentation of perinatally acquired HIV. Each category is illustrated by a patient treated in the acute care setting. Recognition of these categories of presentation will hopefully assist acute care physicians in the early diagnosis of HIV infection. (AJDC. 1993;147:1104-1108)
Sir .—Typically, the infant with meconium aspiration syndrome (MAS) is a term or postterm neonate with a birth weight exceeding 2500 g. In a series of 88 meconium-stained infants, Gregory et al 1 found a mean birth weight of 2911 g and a mean gestation of 290 days or 10 days after the expected delivery date. 1 After reviewing 125 cases of meconium-stained infants, Ting and Brady 2 reported similar results. In utero meconium passage occurs in only 3% of infants of gestational age younger than 36 weeks. 3 In the past, meconium aspiration has been regarded entirely as a postnatal event. We report an unusual case of intrauterine meconium aspiration in a premature infant. Patient Report .—A 610-g white male neonate was born to a 24-year-old gravida 2, para 0010 mother at 27 weeks' gestation by dates and examination. The pregnancy was complicated by maternal diethylstilbestrol exposure, hyperemesis, and
