Smooth muscle tumors of the stomach in childhood and adolescence

Thirty-four smooth muscle tumors of the stomach in childhood and adolescence are reviewed, including two cases reported here for the first time. These are rare tumors, seldom diagnosed correctly preoperatively, and often present with gastrointestinal bleeding and anemia. Histologic differentiation between benign and malignant tumor has often proved difficult. Prognosis is good with wide surgical excision.     

A subacute rabbit model for hepatobiliary dysfunction during total parenteral nutrition

Total parenteral nutrition (TPN) in children is associated with the complicating syndromes of cholestasis and cholelithiasis. The causes of these syndromes are not completely clear. Gastrointestinal hypomotility associated with enteral fasting may be involved in the pathogenesis of both syndromes. We compared weanling rabbits maintained solely on TPN with chow pair-fed and free-fed controls over a 10-day period. Gastrointestinal transit time, assessed with a solid marker technique, was significantly greater in the TPN-treated animals. No difference in intestinal or biliary bacterial flora was demonstrated by aerobic or anaerobic cultures. Gallbladder bile contained a higher percentage of lithocholic acid, unconjugated bilirubin, and total calcium in the TPN-treated animals. Markers of hepatic dysfunction were elevated in the serum of the TPN-treated animals. Mild steatosis and edema were the only histologic differences in the livers of the TPN-treated animals. We conclude that gastrointestinal hypomotility associated with enteral fasting plays a role in the pathophysiologic changes leading to TPN-associated hepatobiliary dysfunction. This dysfunction may be mediated by an increase in the absolute and relative concentrations of lithocholic acid in the bile of TPN-treated animals.     

Pulmonary lesions in childhood onset systemic lupus erythematosus: analysis of 26 cases, and summary of literature

The pathological findings in the lungs and related organs of 26 patients (21 female, 5 male) with systemic lupus erythematosus (SLE), with onset of disease before age 20 years, were reviewed. Several categories of lung lesions were found. Chronic interstitial pneumonitis was present in all 26 patients and was severe in 5. Acute pneumonia was present in 20, mild in 13, moderate in 2, and severe in 5. Alveolar hemorrhage, massive enough to cause death in 5, was seen in 18 patients, and pulmonary edema was found in 13. Fourteen patients had hyaline membranes indicative of acute alveolar damage (DAD), 12 had alveolitis obliterans, indicative of prior episodes of DAD, and 9 had bronchiolitis obliterans. Other parenchymal lesions were mild interstitial fibrosis in 12, alveolar hemosiderosis and alveolar overinflation in 10 each, and alveolar septal calcinosis with chronic renal insufficiency in 3. Pleural effusion, pleuritis, or pleural thickening were noted in 15 of 26, 6 of 23, and 7 of 23 evaluable patients, respectively. Vascular lesions were present in 16 as intimal thickening (9), thromboemboli (8), medial hypertrophy (6), calcinosis (3), and vasculitis (2). A previously unreported lesion was chronic (proliferative) peribronchitis, noted in 11 patients. Diaphragmatic lesions included mild variation in fiber size in 7, mild fibrosis in 2, and calcinosis in 1 of 13 evaluable patients. Correlation of the above lesions with previously described lung syndromes in SLE such as lupus pneumonitis, hemorrhagic lung disease, chronic interstitial fibrosis, lupus cor pulmonale, pleurisy, and "shrinking lung syndrome" are discussed.     

Preferentially Longer Survival of Males than of Females with Untreated Extrahepatic Biliary Atresia: Correlations with Time Course of Intrahepatic Lesions and Suggestion of Possible Ethnic Influences

Males with untreated or unsuccessfully treated extrahepatic biliary atresia show statistically significantly longer survival than females. Females show a greater degree of intrahepatic biliary ductular proliferation than males in the early phase of the process (to age -200 days) and greater duct regression and more rapid connective tissue proliferation than males from approximately 200 to 400 days. Females also show greater frequency of statistically significant hepatomegaly than males over the first year. The black and Hispanic patients in this series tended to show a less marked degree of bile ductule proliferation in the early phase of the process than other patients, but the numbers of such patients available for study were not enough for statistical significance. This point, and its possible clinical correlates, will require further analysis, as will possible biochemical explanations for the differences between males and females in the time course of the intrahepatic lesions of extrahepatic biliary atresia.     

Left Atrial Atheromatosis in Childhood Nephrotic Syndrome

Three male children who had onset, at approximately age 2 years, of nephrotic syndrome, which progressed to renal insufficiency had left atrial atheromatosis at autopsy disproportionate to the degree of aortic or vascular atheromatosis found. The atrial atheromatous process was distributed in elongated nodules, which had a ridged or corduroy-like appearance on gross examination. Two of the patients showed renal lesions of advanced focal glomerulosclerosis, but one had membranoproliferative glomenilopathy, suggesting that the “syndrome” of early onset nephrotic syndrome” progressing to renal insufficiency, hyperlipidemia, and exaggerated left atrial atheromatosis, of which association we have not found a specific report, is etiologically heterogeneous. The patients reported died in 1943, 1952, and 1963. Whether more recent methods of treatment of nephrotic syndrome, hyperlipidemia, or chronic renal insufficiency in children have altered the incidence of such disproportionate left atrial atheromatosis is not known.     

Myopathic skeletal muscle fiber abnormalities in cardiomyopathies of childhood

Skeletal muscle fibers isolated from 50 muscle specimens from 10 children with cardiomyopathy of unknown cause are compared to those from 18 specimens from 5 patients with skeletal muscle myopathies, 45 specimens from 18 patients with congenital heart disease, and 15 specimens from 7 patients with no genetic, chromosomal, or cardiac disease. Muscle fibers from the myopathy specimens show increased nuclei/mm of fiber and increased nuclei/mm/micron of diameter (R value), as well as reduced surface area and volume of cytoplasm per nucleus, compared to control values. The values for cardiomyopathy deviate from normal in the same way as, but to a lesser degree than, those for myopathy--namely, in this material, diseases with cardiomyopathy tend also to produce mild myopathy. Since cardiac and skeletal muscle pathologic findings have not been adequately studied for the majority of the approximately 50 genetic disorders causing cardiomyopathy or otherwise affecting cardiac function described to date, the data indicate primarily that skeletal muscle biopsy will undoubtedly be more useful in cardiomyopathic disorders when the appropriate correlative studies of cardiac and skeletal muscle in such diseases have been done. Because larger biopsy specimens can be obtained, skeletal muscle merits further exploitation in biochemical research on basic mechanisms of disorders causing cardiomyopathy.     

Approximate Doubling of Numbers of Neurons in Postnatal Human Cerebral Cortex and in 35 Specific Cytoarchitectural Areas from Birth to 72 Months

From 1939 to 1967, J.L. Conel quantitatively studied the microscopic features of the developing human cerebral cortex and published the findings in eight volumes. We have constructed a database using his neuroanatomical measurements (neuronal packing density, myelinated large fiber density, large proximal dendrite density, somal breadth and height, and total cortical and cortical layer thickness) at the eight age periods (0 [term birth], 1, 3, 6, 15, 24, 48, and 72 postnatal months) he studied. In this report, we examine changes in neuron numbers over the eight age-points for 35 von Economo areas for which Conel gave appropriate data. From birth to 3 months postnatal age, total cortical neuron number increases 23–30%, then falls to within 3.5% of the birth value at 24 months, supporting our previous work showing that the observed decrease in the number of neurons per column of cortex under a 1-mm² cortical surface from birth to 15 months is almost entirely due to cortical surface expansion. The present study also shows a 60–78% increase in total cortical neuron number above the birth value from postnatal ages 24 to 72 months. The generalization, to humans at least, of the finding of no postnatal neurogenesis in rhesus macaques, a species belonging to a superfamily that diverged from that of Homo sapiens more than 25 million years ago, is not warranted until explicitly proven for humans. The data of the present study support the existence of substantial postnatal neurogenesis in humans for the 35 cortical areas studied. Received November 28, 1997; accepted June 22, 1998.     

Dietary selenium intakes and plasma selenium concentrations of formula-fed and cow's milk-fed infants

The plasma selenium concentrations of 57 infants 8 to 12 months of age were assessed using flameless atomic absorption spectrophotometry. The infants ingested either cow's milk or whey-predominant milk-based infant formula as their primary beverage as part of a mixed diet for at least 3 months. The calculated mean +/- standard deviation (SD) daily dietary selenium intake of 26 infants fed cow's milk (34 +/- 13 micrograms), assessed by a 3-day diet record and/or a 24-hour diet recall, was significantly (p less than or equal to .001) greater than that of 31 formula-fed infants (22 +/- 11 micrograms). The mean +/- SD plasma selenium concentration of infants fed cow's milk (39 +/- 11 micrograms/L) was also significantly (p less than or equal to .05) greater than that of infants fed formula (31 +/- 12 micrograms/L). Both groups of infants ingested similar amounts of total energy; however, the infants fed cow's milk received more total protein and selenium and a greater percentage of protein and selenium from their primary beverage than did the infants receiving formula. Both groups of infants were consuming a mixed diet with similar sources of selenium. To examine the selenium status of infants as well as other individuals better, further analysis of foods is clearly needed to provide more information on dietary selenium sources. The influence of variables such as body size and ethnicity, intake, sources and forms of dietary protein, and dietary forms of selenium on plasma selenium concentrations must also be investigated.