Chronic and discrete stress as predictors of children's adjustment.

Diabetes, as a chronic stressor, and negative life events (NLEs), as a discrete stressor, were related to children's behavioral adjustment, along with moderating effects of the family environment. Diabetes and NLEs predicted both higher internalizing (INT) and externalizing (EXT) behavior problems, suggestive of nonspecific distress. Higher family conflict and lower cohesion each predicted more behavior problems (INT-EXT). However, conflict was the sole moderator of the stressors. Higher family conflict and diabetes exacerbated children's EXT behavior problems, with clinically elevated scores. Higher family conflict and higher NLEs resulted in clinically elevated INT-EXT behaviors. Conversely, low family conflict protected children's behavioral functioning from the stressors. Family cohesion was the sole predictor of children's social competencies but did not moderate the stressors.

Adrenal insufficiency in Smith-Lemli-Opitz syndrome.

We describe three unrelated patients with adrenal insufficiency and RSH or Smith-Lemli-Opitz syndrome (SLOS), a disorder due to deficient synthesis of cholesterol. These patients presented with hyponatremia, hyperkalemia, and decreased aldosterone-to-renin ratio, which is a sensitive measure of the renin-aldosterone axis. All patients had profound serum total cholesterol deficiency (14-31 mg/dl) and marked elevation of 7-dehydrocholesterol (10-45 mg/ dl). Two patients were newborn infants with 46, XY karyotypes and complete failure to masculinize; one of these patients also had cortisol deficiency. Both patients died within 10 days of birth of cardiopulmonary complications while on adrenal replacement therapy. The third patient diagnosed with SLOS at birth presented at age 7months with fever and diarrhea and was noted to have profound hyponatremia. This patient is maintaining normal serum electrolytes on mineralocorticoid replacement. We conclude that adrenal insufficiency may be a previously undetected and treatable manifestation in SLOS. We hypothesize that deficiency of cholesterol, an adrenal hormone precursor, may lead to insufficient synthesis of adrenal steroid hormones.

Behavior problems in children with diabetes: disentangling possible scoring confounds on the Child Behavior Checklist.

OBJECTIVE: The Child Behavior Checklist (CBCL; T. M. Achenbach, 1991), when used to assess the behavior of children with diabetes, may contain confounds because some behavioral items can have a physiologic etiology, and may skew reports of behavioral disturbance. METHODS: Two techniques were used to disentangle possible scoring confounds in the behavioral ratings of children with and without diabetes: (1) the Somatic Complaints scale was deleted, or (2) Diabetes Items, identified a priori with 89% agreement by nine medical personnel, were deleted. RESULTS: As expected, with traditionally scored protocols, children with diabetes obtained higher Internalizing and Total Behavior Problem scores than controls. This group difference persisted whether the Somatic Complaints scale or the Diabetes Items were deleted. CONCLUSIONS: Compared to controls, children with diabetes obtained mildly elevated scores on six of the eight CBCL scales, regardless of scoring method, suggesting that their mildly elevated behavioral profile is not confounded by physiologic symptomatology.

Sociodemographic risk factors to disease control in children with diabetes.

This study examined the effects of sociodemographic variables such as ethnicity, socioeconomic status (SES), and family structure on disease control in 58 children with diabetes stratified by ethnicity and SES. Three dependent variables were chosen to evaluate the disease control of the study participants, including HbA1 values averaged over the year prior to study participation, number of hospitalizations, and number of hypoglycaemic blackouts. SES and family structure, but not ethnicity, were the primary risk factors to disease control. Children from low SES families were in poorer glycaemic control (mean HbA1 = 12.6%) and experienced more episodes of hypoglycaemia-related loss of consciousness (mean = 0.5 per patient) than did children from middle income families (mean HbA1 = 10.4%; mean blackouts = 0.1 per patient). In addition, children from middle-class, two-parent families were in better metabolic control than all other groups. These results indicate that it may not be ethnicity per se, but other factors that often covary with ethnic status, that may pose a risk to the disease status of children and adolescents with diabetes.

Family environment and the interrelation of family structure, child behavior, and metabolic control for children with diabetes.

Examined perceptions of the family environment in a cross-regional sample of 90 families who had children with diabetes and 89 controls. Families were classified as either traditional (intact) or nontraditional (single-parent or blended families). Parents of children with diabetes reported less family expressiveness, which was a predictor of clinically higher levels of child behavior problems than controls. Parents in nontraditional families reported lower levels of organization, less emphasis on active-recreational pursuits, and more child behavior problems than traditional families. An additive effect of diabetes and nontraditional family structure was found for children with diabetes from nontraditional families, who reported substantially less cohesion than all other groups. Nontraditional family structure was more disruptive for children with diabetes than for controls; it was the best predictor of behavior problems and was related to poorer metabolic control.

Postpubertal disease status in diabetes and factor structure anomaly on the WISC-R.

Diabetic children have been found to display an anomalous factor structure on the Wechsler Intelligence Scale for Children-Revised (WISC-R) (Holmes, Cornwell, Dunlap, Chen, & Lee, 1992). The present study sought to extend this finding with a larger cross-regional sample of children to determine which, if any, demographic or disease factor(s) might be related to the anomalous structure. Results revealed that groups of older (> = 12 years) children and those with late disease onset (> = 5 years) exhibited an anomalous four-factor structure in which the traditional Perceptual Organization factor (II) split into two factors: Picture Completion and Picture Arrangement formed a visual discrimination factor; and Block Design and Object Assembly created a spatial conceptual factor. It is postulated that diabetic performance on this visual discrimination factor may reflect mild visual neuropathies, often associated with adolescence and postpubertal disease status.

Effect of extracorporeal shock wave lithotripsy on renal function and body height in pediatric patients.

Although extracorporeal shock wave lithotripsy (ESWL) is the preferred modality for treatment of most renal and upper ureteral calculi in adults, little is known about its effect on the pediatric population. We carefully followed 12 children 2.2 to 15.3 years old (mean age 9.4) treated with the Dornier HM3 lithotriptor. Effective renal plasma flow was obtained by quantitative 131iodine hippurate scan immediately preceding ESWL and at followup (range 74 to 238 weeks, mean 149). The treated kidney received an average of 1,702 shocks (range 1,000 to 2,200). Mean effective renal plasma flow increased in the treated kidney from 185 cc per minute before ESWL to 217 at followup (p = 0.016) and in the untreated kidney from 191 to 224 (p = 0.0013). Total effective renal plasma flow increased from 376 cc per minute before ESWL to 440 at followup (p = 0.0019). In the treated kidney mean and total effective renal plasma flow increased by 31 (expected 32) and 64 (expected 68) cc per minute, respectively, while in the nontreated kidney mean effective renal plasma flow increased by 33 (expected 36) cc per minute. None of the observed changes in effective renal plasma flow was significantly different from the expected changes using the paired t test at the 95% level. In addition, change in body height was evaluated using standard deviation scores. Mean body height (standard deviation) before ESWL was -0.39 (range -3.2 to 2.0) and at last followup it was -0.26 (range -2.6 to 2.4), which is not statistically significant (p = 0.37). Although these patients continue to be followed and caution is advised, this long-term study indicates that ESWL within the range of shocks delivered to this cohort does not statistically affect linear growth (body height) or renal function in the pediatric population.

The growth hormone-releasing activity of a synthetic hexapeptide in normal men and short statured children after oral administration.

It has been shown that iv GH-releasing peptide (GHRP; His-DTrp-Ala-Trp-DPhe-Lys-NH2) specifically releases GH in man. Because of the clinical value of having an orally active peptide to release GH, five normal men were given GHRP orally at dosages of 100 and 300 micrograms/kg. At the 300 micrograms/kg dosage, the GH rise was detectable at 30 min, peaked between 60-75 min, and gradually declined to the baseline level between 150-180 min. Peak GH levels rose 63- and 202-fold above the baseline after administration of 100 and 300 micrograms/kg GHRP, respectively. Nine short stature children with varying degrees of GH deficiency were also included in this study. All children had short stature, slow growth, and delayed bone age and were being treated with biosynthetic human GH. The studies were performed after stopping GH treatment for 2-3 weeks. The oral GHRP dose administered to all of the children was 300 micrograms/kg, because this dosage was found to consistently increase GH levels in adults. The magnitude and pattern of the GH response to oral GHRP in four of the nine children were essentially the same as those in the five normal men. In the other five children, the GH responses were low but still measurable in three and undetectable in two of the children. Serum immunoreactive GHRP (irGHRP) levels were measured before and at 15- to 30-min intervals 3-5 h after oral as well as iv bolus GHRP administration. For comparison of serum irGHRP and GH levels, results were included after iv bolus administration of 0.1, 0.3, and 1.0 micrograms/kg GHRP to normal men. Since 300 micrograms/kg oral GHRP released about the same amount of GH as 1 microgram/kg, iv, in normal men, it was calculated that oral GHRP has about 0.3% the activity of iv GHRP. After iv GHRP, the peak serum irGHRP levels were immediate and proportional to the dosage, and the disappearance rate decreased exponentially. Between 100-240 min, the mean serum irGHRP level was essentially the same and remained slightly elevated. After administration of 300 micrograms/kg GHRP orally to normal men, serum irGHRP was measurable within 15 min, the peak serum irGHRP level coincided with the GH rise at 60 min, and serum irGHRP levels fell more slowly than serum GH levels. The serum half-life was 20 min, and the distribution volume was 2.5 L after both oral and iv administration.(ABSTRACT TRUNCATED AT 400 WORDS)

Total IgG and IgG subclass specific antibody responses to insulin in diabetic patients.

Abnormal antibody responses to insulin in diabetic patients have been associated with syndromes of insulin hypersensitivity and abnormal insulin pharmacokinetics. In this study, we evaluated total and IgG subclass antibody responses to insulin in 70 diabetic subjects on insulin distributed into five clinical groups, and in two control groups using ELISAs with CDC/WHO recommended monoclonal antibodies. As expected, levels of total IgG insulin antibody were greater in diabetic patients treated with insulin than in the control group of diabetic patients on oral agents or nondiabetic controls. Insulin antibody responses of the IgG2 subclass were negligible to absent in all groups. Adult diabetic patients on insulin without complications and those with insulin associated anaphylaxis had mean values of IgG1, IgG3, and IgG4 insulin antibodies no different from those of controls. Patients with local hypersensitivity had elevated IgG1 responses. Type I diabetic patients had elevated IgG3 responses. A group of Type II diabetic patients selected for high levels of total IgG insulin antibodies had elevated levels of IgG1, IgG3, and IgG4 antibody responses. Thus, the IgG subclass response to insulin primarily involves IgG subclasses 1, 3, and 4 and varies with the type of diabetes and complications of insulin therapy.

Evaluation of patients with local reactions to insulin with skin tests and in vitro techniques.

To better understand the part played by IgE and IgG antibody in the production of dermal reactions to insulin and the usefulness of skin tests in the evaluation of these reactions, we studied 21 diabetic patients referred for evaluation of large local insulin reactions, 46 diabetic patients without local insulin reactions, and 22 healthy nondiabetic controls. Study subjects were skin tested with 15 different insulins, and the results were evaluated over 48 h. All control subjects and 41 of 46 diabetic patients without local reactions were skin-test negative to insulin. The 11% of diabetic patients who reacted had positive wheal-and-flare reactions at 20 min to animal-species insulin but negative skin tests to human insulin. Study revealed two subgroups of patients with histories of local reactions. Ten (48%) of these patients had negative skin tests to insulin. Five of this subgroup remained skin-test negative to quantities of less than or equal to 8 U insulin/skin test. Eleven (52%) of the patients formed a subgroup with positive insulin skin tests; most of these patients were skin-test positive to human insulin and to beef, pork, or both insulins as well. Although the group mean insulin-specific IgE values of this latter subgroup were significantly higher than those of any other study group, overlap of these individual IgE values did not allow separation of specific individuals with positive skin tests from those of patients on insulin without dermal reactions.(ABSTRACT TRUNCATED AT 250 WORDS)

Recurrent nosocomial polymicrobial sepsis secondary to child abuse.

Munchausen syndrome by proxy is a rare form of child abuse that can cause great diagnostic difficulties. We treated an 8-year-old boy who had recurrent polymicrobial sepsis over 6 years. An extensive evaluation to determine the infectious focus and to exclude immunodeficiency was carried out before it was determined that his mother had repeatedly introduced material containing faecal or vaginal bacterial flora into his intravenous lines.