Portal vein thrombosis causing neonatal cerebral infarction.

Neonatal cerebral infarction often occurs in the absence of known risk factors. Two such cases are described in which portal vein thrombosis was documented during two dimensional echocardiography. In both cases, infarcts were consistent with embolic events. A novel mechanism is proposed, which may explain some cases of "idiopathic" neonatal cerebral infarction.

Cardiomyopathy in congenital complete lipodystrophy.

Molecular genetic studies have pointed to a relationship between congenital lipodystrophy syndromes and some cardiac disorders. For instance, mutations in LMNA cause either lipodystrophy or cardiomyopathy, indicating that different mutations in the same gene can produce these clinical syndromes. The present authors describe a 10-year-old female with Berardinelli-Seip congenital complete lipodystrophy (MIM 606158) caused by homozygosity for a frameshift mutation in BSCL2. In addition to the typical attributes of complete lipodystrophy, this subject had hypertrophic cardiomyopathy diagnosed in the first year of her life; its progress has been followed with non-invasive imaging. The mechanism underlying the hypertrophic cardiomyopathy in complete lipodystrophy is unclear. It may result from a direct effect of the mutant gene or it might be secondary to the effects of hyperinsulinemia on cardiac development. The variability of the associated cardiomyopathy in patients with complete generalized lipodystrophy may be caused by differential effects of mutations in the same gene or of mutations in different genes which underlie the lipodystrophy phenotype.

Early neonatal presentations to the pediatric emergency department.

UNLABELLED: As a result of shorter postpartum hospital stays, many medical concerns, which traditionally have been managed in newborn nurseries, are presenting to the pediatric emergency department (PED). We undertook a study to determine the profile of early neonatal visits to the PED. In addition, we examined the influence of maternal factors and length of postpartum hospital stay on PED visits. METHODS: We conducted a retrospective chart review of all neonates < or = 8 days of age who presented to the PED between June 1990 and May 1995. Data gathered from neonatal and corresponding maternal charts included neonatal and maternal demographics, prenatal instruction, length of postpartum stay, source of referral, age at presentation, presenting complaint, diagnosis, disposition, and course in hospital, if admitted. RESULTS: A total of 559 neonatal visits were identified. Neonatal use of the PED increased 245% compared to an overall increase in PED use of 8.7% during the study period. Jaundice, difficulty breathing, feeding problems, and irritability were the most common presenting complaints while the most frequent diagnoses were normal physiology, jaundice, feeding problems, and query sepsis. Our overall admission rate was 33%. Self-referred patients were at a significantly lower risk of serious illness (16% admitted vs 49 % of consult patients). The self-referral rate increased with maternal age less than 21, single marital status, no prenatal classes, and primiparity. The length of postpartum hospital stay was identified in 389 neonates (55 early discharge (ED) and 334 non-early discharge (NED). The ED group had a significant increase in annual PED utilization compared to the NED group changing from 2% of the total population in study year 1 to over 31% in the final year. The ED and NED groups did not differ significantly with respect to the majority of chief complaints, frequency of PED diagnoses, admission rates, or maternal characteristics. CONCLUSIONS: Use of the PED by neonates < or = 8 days of age increased significantly over the study period. The majority of neonates presented by self-referral and were discharged with advice only. Mothers who were young, single, primiparous, or who had not attended prenatal classes presented with neonates who were less seriously ill. Although ED neonates represent an enlarging subset of PED visitors, they do not appear to differ significantly from NED neonates with respect to PED complaints, diagnoses, and disposition.

Compliance with prescription filling in the pediatric emergency department.

OBJECTIVES: To determine the rate of compliance with filling of prescriptions written in a pediatric emergency department and to examine the reasons for not filling the prescriptions. DESIGN: Compliance with filling prescriptions was determined using a follow-up standardized telephone questionnaire, designed so that it was not obvious that assessing prescription filling was the major reason for the study. Compliance herein was defined as having the prescription filled on the same or next day of the pediatric emergency department visit. SETTING: Pediatric emergency department of a tertiary care hospital. SUBJECTS: Pediatric patients discharged home with a drug prescription. MAIN OUTCOME MEASURE: The proportion of prescriptions written in the pediatric emergency department that were filled on either the same or next day as determined by telephone follow-up. This outcome is expressed as a proportion with 95% confidence interval. RESULTS: Follow-up was completed in 1014 (83%) of the 1222 children, aged 4.5 +/- 4.2 (mean +/- SD) years. Compliance with prescription filling was 92.7% (940/1014). Parental reasons for not filling the prescription included medication unnecessary (27%), financial (6.8%), and not enough time (6.8%). Dissatisfaction with the explanation of the medical problem, instructions for treatment, and instructions for follow-up treatment were significantly associated with noncompliance by univariable logistic regression (P<.05). CONCLUSION: The rate of prescription nonfilling in children seen in a pediatric emergency department is at least 7%, although lower than that in adults in a similar setting.

Selection of treatment of cefaclor-associated urticarial, serum sickness-like reactions and erythema multiforme by emergency pediatricians: lack of a uniform standard of care.

BACKGROUND: Serum sickness-like reactions (SSLR) and erythema multiforme are common adverse effects of cefaclor therapy and can be associated with significant morbidity. No standardized evidence-based protocol for the optimal treatment of drug-induced SSLR exists. OBJECTIVES: To define the standard of care used by physicians treating adverse reactions associated with cefaclor. METHODS: A retrospective review of the medical records of children discharged from a pediatric emergency room with a diagnosis of adverse events to cefaclor was conducted. Charts of patients were reviewed to determine which therapy was prescribed. RESULTS: During the study period, 74 cases of adverse events attributed to cefaclor presented to the emergency department. SSLR were the most common pattern of adverse events seen (31 cases, 42%), followed by urticarial reactions (26 cases, 35%) and erythema multiforme (17 cases, 23%). An antihistamine was the treatment most often prescribed (88%) for erythema multiforme. Significantly more children with SSLR than with erythema multiforme or urticaria were treated with prednisone, either alone or in combination (P<0.05). CONCLUSIONS: The treatment most often prescribed for serious cefaclor-associated erythema multiforme was an antihistamine. In the case of SSLR, an antihistamine and prednisone were most commonly used. Prospective randomized, controlled trials are needed to define the role of various therapeutic agents and to determine the optimal therapy for SSLR and other serious adverse drug reactions.

The electrocardiogram and the secundum atrial septal defect: a reexamination in the era of echocardiography.

BACKGROUND: Ostium secundum atrial septal defects (ASDs) often present subtly and may be a diagnostic challenge to the community physician. Characteristic abnormalities of the electrocardiogram (ECG) have been described in adults. OBJECTIVE: To determine whether ECG abnormalities are consistently present in children with a hemodynamically significant ASD, and their potential for differentiating this group from patients with innocent murmurs. DESIGN: Retrospective evaluation of clinical characteristics, echocardiographic data, and ECGs was undertaken in 67 consecutive children with an ASD (mean age 2.9 +/- 2.8 years, 63% female) and 77 patients with innocent murmur (mean age 3.2 +/- 2.6 years, 61% male). Predetermined ECG criteria were derived from adult studies (rsR'-V1 with evidence of right ventricular hypertrophy, isolated rsR'-V1, and unequivocal right ventricular hypertrophy without rsR'-V1). ECGs were interpreted blindly by two observers. RESULTS: In the ASD group 58 (87%) patients had an ECG that met predetermined criteria compared with three (3.9%) controls (P < 0.001). Completely normal ECGs were found in only four (6.0%) ASD patients compared with 66 (86%) controls (P < 0.001). The ECG criteria had a sensitivity of 86% (95% CI 0.784 to 0.947) and a specificity of 96% (95% CI 0.918 to 1.000). When any ECG abnormality was considered the sensitivity increased to 94% (95% CI 0.884 to 0.997) with a decline in specificity to 86% (95% CI 0.779 to 0.935). CONCLUSIONS: The ECG is potentially a valuable adjunct to the physical examination in differentiating children with an ASD from those with an innocent murmur in the primary care setting.

Arteritis and increased intracellular calcium as a possible mechanism for tacrolimus-related cardiac toxicity in a pediatric transplant recipient.

We recently reported partially to wholly reversible hypertrophic cardiomyopathy, including severe hypertrophic obstructive cardiomyopathy, as a side effect in pediatric transplant recipients receiving tacrolimus immunosuppression. This seemed to be dose related. We describe a pediatric patient receiving tacrolimus who died 3 weeks after liver/bowel transplantation. Postmortem findings revealed arteritis of cardiac arteries and extensive calcification of cardiac tissue suggesting a possible mechanism of tacrolimus cardiac toxicity. This is consistent with recent reports of tacrolimus increasing calcium release into the sarcoplasmic reticulum of cardiac and striated muscle.

Appropriateness of hospitalization in a Canadian pediatric hospital.

Allocation of limited resources in the Canadian health care system is hampered by a lack of studies addressing the appropriateness of the pediatric patient days in hospital. The authors retrospectively reviewed one hospital day per month in 1988, using a Pediatric Appropriateness Evaluation Protocol previously used in the United States. Of 878 inpatients, 852 charts were reviewed, and 26 charts were unavailable for study. The patients ranged in age from premature newborns to 20 years old. There were 475 medical days, 359 surgical days, and 18 patients to other services. Statistical significance was tested using the chi 2 test for contingency tables. Twenty-four percent of patient days were inappropriate. Younger children and shorter lengths of stay were more likely to result in appropriate hospital days. For infants younger than 60 days, 11% of days in hospital were inappropriate, 21% of days for infants between 2 months and 1 year of age, 25% for children between 1 and 5 years, and 36% for children older than 5 years of age. Children hospitalized 2 days or less had inappropriate hospital days accounting for 16% of the reviewed days. This increased to 33% for 3 to 14 days of hospitalization. Inappropriate hospital days did not vary significantly from month to month. Surgical patients had more appropriate hospital days than medical patients. Admission route (elective, emergency, or transferred from another hospital) did not affect the appropriateness of the subsequent day reviewed. It is concluded that inappropriate hospitalization in a Canadian pediatric hospital occurs only slightly more frequently than in an American pediatric hospital.(ABSTRACT TRUNCATED AT 250 WORDS)

Gene dosage effects in human diploid and tetraploid fibroblasts.

The effects of cell ploidy on the biochemical characteristics of cultured cells were compared using human diploid vs tetraploid fibroblasts isolated with a non-selective method. Their DNA replication was compared by thymidine incorporation, and DNA content by Feulgen staining and quantitative analysis. Their RNA and protein content, cell sizes and the specific activities of glucose-6-phosphate dehydrogenase (G-6-PD) and 6-phosphogluconate dehydrogenase (6-PGD) were assayed quantitatively. With the exception of RNA content, all other parameters demonstrated a 2-fold increase reflecting the increase in cell ploidy. These direct gene dosage effects on the genetic material and functional expression of the human genome were in contrast to previous observations in other species and validate the use of human intraspecific euploid hybrids for biochemical and genetic studies.