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This article examines and compares psychosocial characteristics across 2 samples of children with recurrent abdominal pain (RAP). A tertiary care sample (n = 39) and a community sample (n = 47) of children meeting established criteria for identifying RAP were compared. Multiple independent statistical comparisons suggested that the samples were similar across many dimensions, including parent- and child-rated child somatization, functional disability, and parent-rated child psychopathology. However, parents of children in the tertiary care sample rated their children as experiencing more severe pain currently as well as in the past 2 weeks. Furthermore, child-rated pain was significantly different across the samples, with children in the tertiary care sample reporting more frequent pain episodes, longer pain episodes, and more severe pain. Description of these sample characteristics helps to better understand important psychosocial similarities and differences between the two samples. The results are disc...

An Argentine male child died at 4.5 years of age of a lethal mitochondrial disease associated with a MELAS mutation and a Barth syndrome-like presentation. The child had severe failure to thrive from the early months and for approximately two years thereafter. In addition, the patient had severely delayed gross motor milestones, marked muscle weakness, and dilated cardiomyopathy that progressed to congestive heart failure. He also had persistently elevated urinary levels of 3-methylglutaconic and 2-ethylhydracrylic acids and low blood levels of cholesterol. Detailed histopathologic evaluation of the skeletal muscle biopsy showed high activity of succinate dehydrogenase, a generalized decrease of COX activity, and abundant ragged-red fibers. Electron microscopic studies revealed multiple mitochondrial abnormalities in lymphocytes and monocytes, in the striated muscle, and in the postmortem samples (muscle, heart, liver, and brain). Biochemical analysis showed a pronounced and constant lactic acidosis, and abnormal urinary organic acid excretion (unchanged in the fasting and postprandial states). In addition, in CSF there was a marked increase of lactate and beta-hydroxybutyrate (beta-HOB) and also a high systemic ratio beta-HOB/acetoacetate. Enzymatic assay of the respiratory chain in biopsied muscle showed 10% of complex I activity and 24% of complex IV activity compared with controls. Molecular studies of the mitochondrial genome revealed an A to G mutation at nucleotide pair 3243 in mitochondrial DNA, a well-known pathogenetic mutation (MELAS mutation) in all the patient's tissues and also in the blood specimens of the probands mother and sibs (4 of 5). The diagnosis of MELAS mutation was reinforced by the absence of an identifiable mutation in the X-linked G4.5 gene of the propositus. The present observation gives additional evidence of the variable clinical expression of mtDNA mutations in humans and demonstrates that all clinical variants deserve adequate investigation to establish a primary defect. It also suggests adding Barth-like syndrome to the list of phenotypes with the MELAS mutation.

We prospectively measured soluble interleukin-2 receptor levels in 56 premature infants with suspected sepsis and demonstrated significant differences between those with positive results on blood, urine, or cerebrospinal fluid cultures, and those with negative results. Soluble interleukin-2 receptor levels can be used to facilitate the diagnosis of sepsis in premature infants with negative blood culture results.

Soluble interleukin-2 receptors (sIL2R) in plasma have been identified as a marker of lymphocyte activation. Lymphocyte activation as a manifestation of inflammation may be important in the pathogenesis of bronchopulmonary dysplasia (BPD). To test the hypothesis that infants with BPD have higher sIL2R levels, 12 infants with or at risk of developing BPD (GA +/- SD, 27 +/- 5 weeks; BW +/- SD 1,053 +/- 733 g) had plasma sIL2R levels determined and were compared to 20 infants being ventilated for respiratory distress syndrome (RDS) (GA +/- SD, 28 +/- 3.5 weeks; BW +/- SD, 1,133 +/- 390 g: P = NS for both GA and BW, t test). Tracheal aspirates in both groups were also analyzed for sIL2R levels. To control for the effects of postnatal age (PNA) and study weight (SW) on the sIL2R levels, another group of 16 nonventilated babies (NVB) had plasma analyzed for sIL2R (PNA +/- SD: 39 +/- 40 days NVB vs. 48 +/- 36 days BPD; P = NS); (SW +/- SD: 1391 +/- 250 g NVB vs. 1212 +/- 700 g BPD; P = NS). The following data were obtained for the plasma sIL2R levels (mean +/- SEM U/mL): RDS controls, 1,231 +/- 80; BPD infants, 1,790 +/- 120; NVB controls, 1,319 +/- 76; P = 0.0005 RDS vs. BPD and P = 0.002 BPD vs. NVB. There was no significant difference in the sIL2R levels for the infants at risk of developing BPD vs. the infants with established BPD. Also, when analyzed separately, infants at risk of BPD and the infants with established BPD had higher sIL2R levels than the RDS and NVB controls. No differences were noted in the tracheal sIL2R levels in the BPD vs. RDS groups. These data indicate that infants with BPD had significantly higher sIL2R levels in plasma than either RDS or NVB controls. Therefore, lymphocyte activation may play a role in the pathogenesis of BPD.

Serum concentrations of the soluble form of the interleukin-2 receptor (sIL-2R) were determined by an enzyme-linked immunosorbent assay in a group of 39 pediatric and adolescent patients with Crohn's disease and in age-matched ulcerative colitis patients and controls. sIL-2R levels were found to be elevated in patients with Crohn's disease (p less than 0.001), and increased sIL-2R levels were detected in patients with clinically more severe disease. sIL-2R levels correlated more closely with other laboratory markers of disease activity than with a disease activity score. A progressive increase in sIL-2R levels was noted to correlate with endoscopic measurement of disease extent, while sIL-2R levels did not correlate with other markers of systemic lymphocyte activation, suggesting possible local mucosal production. Sequential determinations in individual patients revealed a good correlation between sIL-2R and clinical course. More important, elevated levels of sIL-2R preceded clinical relapse of asymptomatic patients. We conclude that sIL-2R measurement may be a useful adjunct to clinical assessment and routine laboratory testing in pediatric and adolescent patients with Crohn's disease and that serial levels may be predictive of clinical course and the response to therapy.

Objectives: To examine psychosocial differences in diagnostic subgroups of children with recurrent abdominal pain (RAP). Methods: Children meeting Apley's 1975 definition of RAP were divided according to physician ratings into three subgroups, based on the Rome II diagnostic criteria of functional gastrointestinal disorders: functional dyspepsia (n = 17), irritable bowel syndrome (n = 18), and functional abdominal pain (n = 27). Groups were compared using measures of (a) child psychopathology, (b) parent psychopathology, and (c) child pain, somatization, and functional disability. Results: Multivariate results from a discriminant function analysis demonstrated that children classified according to these criteria could not be differentiated with respect to parent reported child psychopathology or child pain, somatization, and functional disability. There were significant univariate differences, however, between groups on parental psychopathology (F = 4.39, P = 0.02); parents of children with functional dyspepsia reported greater parental psychopathology symptoms than the other two groups. Conclusions: This study provides a preliminary comparison of pain, somatization, functional impact, and psychopathology ratings in the Rome II diagnostic subclassifications of children with RAP. Further investigation utilizing larger sample sizes, pain measures specifying pain location, and parental modeling of somatic behavior is indicated to better understand potential similarities and differences between these subgroups.

The detection of anti-neutrophil cytoplasmic antibodies (ANCA), in a perinuclear fluorescence pattern, in the serum of adults with inflammatory bowel disease has recently been described to be sensitive and specific for a diagnosis of ulcerative colitis in comparison to Crohn's disease and other colitides. We have examined the sera of 41 children and adolescents with ulcerative colitis, 27 with Crohn's disease, and a control group for the presence of ANCA. Anti-neutrophil cytoplasmic antibodies were detected in the serum of 27 of 41 patients with ulcerative colitis (66%), five of 27 with Crohn's disease (19%), and in none of our control subjects or patients with functional abdominal pain. Overall, the presence of ANCA was 66% sensitive and 84% specific for a diagnosis of ulcerative colitis when compared to Crohn's disease. There was no relationship between a positive ANCA value and disease activity or other clinical indicators. We conclude that evaluation for the presence of ANCA may be a useful adjunct for the clinical assessment of patients with inflammatory bowel disease. The presence of ANCA in children and adolescents, however, will not definitively distinguish between patients with ulcerative colitis and Crohn's disease.

Blastocystis hominis, a protozoan whose pathogenicity has been questioned, is sometimes found in the human gastrointestinal tract. We sought to determine the prevalence of Blastocystis in stool and to characterize clinical features of infection with Blastocystis in children. Forty-six (3%) of 1,736 patients undergoing fecal microscopy at Children's Hospital of Pittsburgh between January 1, 1985, and December 31, 1988, harbored Blastocystis. Of these 46 children, 75% had exposure to well water or had been in developing countries. Thirty-nine of the 46 (85%) experienced gastrointestinal symptoms, such as abdominal pain, diarrhea, vomiting, and weight loss. Blastocystis was the only parasite found in 35 of those 39 symptomatic children. Symptoms resolved within one month in 90% of patients receiving antiparasitic pharmacotherapy, but in only 58% (P

Idiopathic hypereosinophilic syndrome (IHES) is a heterogeneous group of disorders characterized by multisystem dysfunction and persistent, extreme eosinophilia of unknown cause. We describe a 9-1/2-year-old boy whose course included several unusual clinical features and terminated 2 years after diagnosis in acute lymphoblastic leukemia (ALL). Serial studies suggest that leukemia was not present earlier in his course. We speculate that this child may have had an evolving lymphoproliferative syndrome with a terminal blast crisis to which the eosinophilia was a nonmalignant leukemoid reaction.

We have reviewed 53 cases of allergic disorders of the gastrointestinal tract in children, including 15 with principal effects in the rectum (allergic proctitis) and 38 with dominant involvement of the upper and mid portions of the gut (allergic gastroenteritis). Most cases of allergic proctitis had their onset at less than 6 months of age, and all were under 2 years old when they presented with rectal bleeding alone or in combination with diarrhea. Rectal mucosal biopsy revealed in most cases a diffuse increase of eosinophils in the lamina propria together with a focal infiltration of the epithelium by eosinophils. Cases of allergic gastroenteritis affected all age groups and had a lower frequency of overt rectal bleeding. More common were other symptoms (vomiting, pain, and weight loss), an allergic history, anemia, blood eosinophilia, and increased serum IgE. Mucosal biopsy abnormalities were present in the gastric antrum in all cases sampled, the small intestine in 79%, the esophagus in 60%, and the gastric corpus in 52%. The lesions were usually diffuse and marked in the antrum and esophagus; in contrast, they tended to be focal and mild in the small intestine and gastric corpus. All cases of proctitis responded to a dietary change by cessation of symptoms without recurrences, whereas most of those with gastroenteritis had multiple relapses and required corticosteroid therapy.

A puff of air administered to the face of 102 subjects (from 33 week's gestation on the first day of life to age 42 years) elicited a reflex swallow identical to a normal primary peristaltic sequence in 88. Subjects who responded were younger than 24 months of age or severely disabled neurologically; those who did not respond were older than 11 months and free of neurological abnormality. Response was variable between 11 and 24 months. This swallow response appears to be a clinically useful but previously unrecognised infant reflex.