Increased gastric juice leukotriene B4, C4 and E4 concentrations in children with Helicobacter pylori colonization.

During recent years, the role of inflammatory lipid mediators in the pathophysiology of Helicobacter pylori (H. pylori) infections has been investigated in several studies. The concentrations of leukotrienes (LTs) in gastric juice from H. pylori positive (n = 13) and negative (n = 18) children with recurrent abdominal pain were studies in order to determine whether these lipid inflammatory mediators are involved in local and systemic biological actions. Gastric juice samples and biopsy specimens of mucosa were obtained endoscopically from 31 patients with recurrent abdominal pain for assessment of LTs and histopathological examination. In this study, all children with recurrent abdominal pain were investigated by rapid urease test and histological assessment for H. pylori colonization. Leukotriene levels were measured by high performance liquid chromatography (HPLC) and radioimmunoassay (RIA) in gastric juice samples. Gastric juice LTB4, LTC4, and LT4 levels were significantly higher in patients with H. pylori colonization than in children without H. pylori colonization. These results indicate that increased gastric content of proinflammatory mediators (LTB4, LTC4, and LT4) may be related to the pathogenesis of H. pylori-associated gastritis.

Blood levels of leukotrienes (LTC4, D4, E4, B4) and synthesis of leukotriene B4 by peripheral leukocytes in children with acute A and B hepatitis.

Leukotrienes (LTs) are cell-membrane derived lipid inflammatory mediators, synthesized and eliminated by the liver. LTs have effects on liver cells in some pathological conditions. In this study, we measured plasma endogenous and liberated leukotriene (LT) concentration in peripheral blood leukocytes stimulated in vitro by the calcium ionophore (CaA23187) and platelet-activating factor (PAF). Production of LTs was measured in type A (n=37) and type B (n=10) acute hepatitis patients and control subjects (n=10). LTs levels were measured by high performance liquid chromatography (HPLC) and radioimmunoassay (RIA). The concentration of LTB4 measured in plasma and stimulated peripheral blood leukocyte supernatants of children with hepatitis A infection was found to be statistically elevated and in positive correlation with serum alanine aminotransferase (ALT) levels. In plasma samples of hepatitis B patients, LTC4 and LTE4 were measured in significantly elevated concentrations. These results suggest that LTB4 may be a critical mediator of hepatitis A virus-induced hepatocellular injury.

A case of pycnodysostosis with growth hormone deficiency.

Pycnodysostosis is a skeletal dysplasia characterized by short stature. Treatment of pycnodysostosis with growth hormone (GH) has not been reported so far. We describe a case of pycnodysostosis with growth hormone deficiency in addition to low mean insulin-like growth factor 1 (IGF-1) concentration. Complete GH deficiency was determined by two pharmacological provocative tests (insulin and L-dopa). A good height-velocity response was obtained after GH replacement treatment. Pycnodysostosis with GH deficiency and replacement therapy have not been reported previously, to the best of our knowledge.

Zinc tolerance test patterns in normal children and in moderate and severe zinc deficiency states.

Twenty-six normal, 38 moderately and 14 severely zinc-deficient children, aged 2-12 years, were examined by clinical and laboratory approaches. After fasting-blood sampling, 120 mg zinc sulphate (25 mg elemental zinc) were administered orally to each group of children, to obtain zinc tolerance curve patterns. Sampling proceeded to the 2nd and 4th hours of the loading-test period. Plasma zinc was assessed on an atomic absorption spectrophotometer. In normal children, at the 2nd hour of loading, a significant (p < .001) elevation (1.764 +/- 0.133 mg/l) in the mean (+/- SEM) plasma zinc level was noted; also at the 4th hour a significant (p < .001) decrease (1.506 +/- 0.123 mg/l) in the mean plasma zinc level was shown. The mean plasma zinc level at the 4th hour was found higher than the mean fasting plasma zinc level (1.054 +/- 0.061 mg/l), but lower than the mean level found at the 2nd hour. In moderately zinc-deficient children, the rise in the 2nd hour and the fall in the 4th hour in the plasma zinc level were highly significant (p < .001 and p < .001, respectively) in relation to fasting blood level. However, in severely zinc-deficient children, the intensity of the increase (0.746 +/- 0.147 mg/l) in plasma zinc level at the 2nd hour was of lesser significance (p < .006) and the fall (0.424 +/- 0.061) mg/l) at the 4th hour was not significant. Therefore, in children with normal plasma zinc levels, an increase of more than 0.50 mg/l was seen at the 2nd hour of loading. This rise was seen to persist at the 4th hour. However, in children with moderate zinc deficiency, although again an increase of 0.50 mg/l was seen at the 2nd hour this increase did not persist at the 4th hour; and the 4th-hour value showed a significant decrease in relation to the 2nd hour value. Whereas, in children with severe zinc deficiency the rise of plasma level at the 2nd hour was less than 0.5 mg/l and the fall at the 4th hour was to such a level which was not significant in relation to fasting zinc level. This could be due to enhanced uptake of zinc off the circulation by the depleted tissues in severe zinc deficiency.

Four siblings with achalasia, alacrimia and neurological abnormalities in a consanguineous family.

Four siblings with achalasia, alacrimia and other problems involving the autonomic nervous system involvements are reported. Achalasia and alacrimia were present in all of them. Their parents are first cousins and have four other healthy children. Electrophysiological tests showed that autonomic dysfunction has progressed with age. Blood cortisol levels were normal in all four affected children. Depending on those findings of our case and previous reports, we conclude that triple-A syndrome and achalasia, alacrimia with or without neurological abnormalities could be variable manifestations of the same autosomal recessive gene defect.

Pseudoachondroplasia with immune deficiency.

A 5-year-old boy was admitted to the hospital with failure to thrive since he was 2 years old, with weakness in his legs and a waddling gait. He has normal mental development. His parents are normal phenotypically and are unrelated. In analyzing his pedigree only a grandfather is described to have waddling gait. He has a normal craniofacial appearance but a disproportionate body with normal trunk and short extremities with height below the 3rd percentile. The diagnosis of pseudoachondroplasia was made on clinical, radiological and laboratory findings. He also had immune deficiency characterized by low T-lymphocyte populations and a low level of serum immunoglobulin A.