Gastrointestinal findings in atopic children.
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Adolescent↗
Biomedical subjects
S Similä
Publications and source records attributed to S Similä.
Cow's milk intolerance with melena.
The clinical features, the results of gastric secretory function tests, and the duodenojejunal morphology of six infants (aged 0.42-1.23 years) with anemia and melena considered to be due to latent cow's milk intolerance (LCMI) were compared with the findings in nine infants (aged 0.19-0.87 years) with cow's milk-induced malabsorption (CMI). The infants with LCMI had a short period of breast feeding, normal weight gain without symptoms of malabsorption, and no atopic history. The maximal acid secretion was decreased (p < 0.01) and the concentration of fasting serum gastrin raised (p < 0.01) compared with the controls. Gastric biopsy revealed epithelial degeneration in three and erosion in one out of four samples. The duodenojejunal biopsy revealed slight changes in two samples, the others being normal. The number of eosinophils was increased in four out of six biopsies. Although the number of intraepithelial lymphocytes was increased in LCMI the rise was not as significant as in children with CMI (p < 0.05). We conclude from our results that LCMI seems to be a seperate clinical entity. The determination of fasting serum gastrin, maximal gastric acid secretion and intraepithelial lymphocytes on duodenojejunal biopsy appear to be helpful in making the diagnosis.
Animals↗
Dipropylacetate and aminoaciduria.
Dipropylacetate (DPA) is an anticonvulsant, which has been successfully used in the treatment of several types of epilepsy. The mode of action has not yet been definitely elucidated, although evidence of influence on gamma aminobutyric acid (GABA) turnover in brain has been presented. Several recent reports of the occurrence of hyperglycinemia in association with DPA-treatment indicate that this agent also influences other areas of amino acid metabolism. In the present study of 10 patients receiving DPA for epilepsy, high concentrations of glycine in plasma and CSF were observed, whereas the levels of all other amino acids remained virtually unchanged. The effect of DPA on urinary excretion of amino acids seems to be of considerable significance as marked elevation of urine concentrations of alanine, asparagine, cystine, glycine, histidine, isoleucine and leucine, phenylalanine and tyrosine were observed. This secondary hyperglycinemia could be due to suppression of glycine conjugation reactions, whereas DPA or its metabolites might interfere with tubular reabsorption of various amino acids, thereby causing hyperaminoaciduria.
Adolescent↗
A case of Mulibrey nanism with associated Wilms' tumor.
The occurrence of a nephroblastoma in a 2-year-old girl with Mulibrey nanism is reported. As this tumor has also been reported in some other "mesodermal dysgenesis" syndromes, it seems probable that patients with this form of congenital disease run an abnormally high risk of Wilms' tumor.
Child, Preschool↗
An arthropathic form of osteogenesis imperfecta.
A 14-year-old girl gradually developed severe osteoporosis and destructive generalized joint disease, resulting in joint stiffness and anchyloses. She also had moderate hydroxyprolinemia and hydroxyprolinuria. Rheumatoid arthritis was highly unlikely. Anamnestic data revealed two long bone fractures. Collagen biosynthesis was studied in fibroblasts cultured from the patient's skin. Chromatograms of 3H-labelled culture media proteins on ion exchange celluloses revealed an increased ratio of type III collagen to type I collagen when compared with the chromatograms of age-matched control fibroblasts. This finding is typical of certain cell strains in osteogenesis imperfecta. The patient might thus express a new variety of osteogenesis imperfecta with chronic arthropathy.
Adolescent↗
Cholic acid and chenodeoxycholic acid concentrations in serum during infancy and childhood.
Concentrations of two primary bile acids (cholic and chenodeoxycholic acids) were determined by radioimmunoassay in the serum of infants and children at ages ranging from 1 hour to 15 years. The same bile acids were also measured in umbilical cord serum. Concentrations of the primary bile acids were significantly higher in the serum of 1-hour old infants than those in the umbilical cord serum or the peripheral vein serum of adults. The levels of cholic and chenodeoxycholic acid remained high until the age of 6 months, being about 5-fold higher than those in the sera of adults. Primary bile acid concentrations reached the adult level by the age of 1-2 years. These results indicate that developmental changes occur in the metabolism and excretion of bile acids in man. The relatively high concentrations of the primary bile acids in serum during the first 6 months of life suggest that up to this age, the mature ability of the liver to excrete the bile salts into the bile and/or to clear them from the circulation has not yet been reached.
Adolescent↗
Late pulmonary sequelae after type 7 adenovirus pneumonia.
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Adenoviridae Infections↗
Oral antipyretic therapy evaluation of the propionic acid derivatives ibuprofen, ketoprofen, fenoprofen and naproxen.
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Administration, Oral↗
Serum bile acid concentrations in the course of benign recurrent intrahepatic cholestasis.
The patient reported here represents the classic features of the syndrome of benign recurrent intrahepatic cholestasis (BRIC). She had conjugated hyperbilirubinaemia during the neonatal period and early infancy. The concentrations of serum primary bile acids, cholic acid (CA) and chenodeoxycholic acid (CDCA) were determined by radioimmunoassay and were continuously extremely raised during the icteric, recovery, and also the anicteric phases of the disease. The ratio of CA to CDCA was always within the normal range. The secondary serum bile acid, deoxycholic acid (DCA), was only slightly raised. The oral cholate tolerance test was abnormal in the patient during the anicteric phase. These observations support the suggestion that a disturbance of the hepatocellular bile acid transport may be the primary defect in BRIC.
Bile Acids and Salts↗
Failure of strychnine treatment during the neonatal period in three Finnish children with nonketotic hyperglycinemia.
Three Finnish infants with a severe neonatal-onset-type of nonketotic hyperglycinemia were treated with strychnine nitrate in a daily dosage of 0.2 to 0.9 mg/kg, given orally in four doses. In order to lower the plasma and CSF-glycine concentrations concomitant exchange transfusions (200 to 300 ml/kg of heparinized blood) were carried out in two of these infants. Although the strychnine therapy was started at ages 15, 40, and 62 hours, the strychnine produced no clinical effect, and the exchange transfusion caused only a transient decrease in the plasma glycine level. Despite treatment, the clinical course was the same as in the majority of children with the severe form of the disease--all died within the first ten days of life. Impressive effects of strychnine treatment initiated in two infants at ages 5 and 6 1/2 months, and given in addition to sodium benzoate and anticonvulsants, have been reported. These cases, however, probably represent a less severe type of nonketotic hyperglycinemia. Nevertheless, the therapeutic failure in the present cases probably indicates that strychnine treatment does not solve the therapeutic problems of severe forms of NKH.
Amino Acid Metabolism, Inborn Errors↗
Impaired gastric function in children with cow's milk intolerance.
Eight infants with cow's milk intolerance (CMI) were studied for basal and maximal gastric acid secretion and the fasting serum gastrin level. All these patients had clinical malabsorption. Jejunal biopsies revealed subtotal villous atrophy in six children and slight changes in the remaining two. The mean maximal acid secretion in the infants with CMI was significantly decreased being 85 +/- 78 mumol/h/kg (mean +/- SD), as compared with a control group of the same age with a corresponding value of 233 +/- 66 mumol/h/kg. The fasting serum gastrin level was elevated, being 104 +/- 116 pmol/l in the study group and 37 +/- 10 in the controls. Three infants with CMI underwent gastric biopsy. Marked changes with epithelial degeneration and prominent cellularity in the lamina propria were seen in two patients. The injury was most severe in the antrum of the stomach. When these patients with CMI were treated with human or soy milk, the maximal acid secretion returned normal in six months.
Atrophy↗
Gastric function and absorption of vitamin B12 in children with celiac disease.
Gastric acid secretion in nineteen children with celiac disease remained almost unchanged and the level of fasting serum gastrin was comparable with that of a control group of the same age. The absorption of vitamin B12 was significantly decreased, most clearly in the infants with celiac disease as compared with their controls. The serum B12 level, however, was decreased only in the oldest children. The results suggest that the mucosal lesion in the small intestine is most extensive in the youngest children, but the absorption defect of vitamin B12 becomes clinically significant only after a long duration of the disease and not in childhood.
Adolescent↗
Dipropylacetate and hyperglycinemia.
Hyperglycinemia was induced by dipropylacetate (DPA) (dose 14-41 mg/kg per day) in 10 Finnish epileptic patients with neurologically disabling diseases of various kinds. When comparing the levels of glycine in plasma, cerebrospinal fluid and urine from the patients with 10 otherwise comparable patients, and normal values there was a fourfold excretion of glycine in urine. All the treated patients had elevated CSF (49 +/- 23 mumol/l) and plasma (475 +/- 76 mumol/l) glycine concentrations. The plasma/CSF glycine ratio was not influenced by the treatment. In comparison with corresponding values in nonketotic hyperglycinemia and ketotic hyperglycinemia, this condition probably arises in a manner similar to secondary hyperglycinemia in organic aciduria. DPA probably influences metabolism in a way similar to some metabolites of amino acids in organic acidurias.
Adolescent↗
Nonketotic hyperglycinemia. A genetic study of 13 Finnish families.
In Finland, 19 children, born 1964--1977, from 13 families, have been diagnosed as suffering from nonketotic hyperglycinemia (NKH). This gives an incidence for NKH in the Finnish population of 1:55,000 newborns. The majority of these children were born in the northern part of the country, where the incidence is 1:12,000. The geographical distribution of the birth-places of the grandparents also seems to point towards an enrichment of the gene in northern Finland. An autosomal recessive mode of inheritance for this disease seems probable, since the corrected proportion of affected siblings (Apert's a priori method) is 0.288. Abnormally high plasma glycine concentration and elevated glycine urinary excretion in the parents of the NKH-children suggest the existence of a minor metabolic defect in heterozygotes of this disease. Some of the healthy siblings of the NKH-patients also show similary elevated levels. However, a definite diagnosis of the NKH-heterozygote state cannot easily be made on the basis of these laboratory findings, as the levels in some individuals are very close to, or even overlap corresponding values in a normal material.
Adolescent↗
Antipyretic effect and plasma concentrations of rectal acetaminophen and diazepam in children.
Plasma levels of acetaminophen (paracetamol) and diazepam were measured in 9 children by gas chromatography after administering these drugs simultaneously in separate suppositories. The antipyretic effects of oral and rectal acetaminophen-diazepam combinations were also studied and compared with that of oral or rectal acetaminophen alone. Diazepam at a dose of 0.2 mg/kg did not increase the antipyretic action of acetaminophen. Acetaminophen and diazepam seemed to be well absorbed from the rectal suppositories, the maximal plasma concentration of diazepam after a rectal dose of 0.5 mg/kg just reaching the assumed anticonvulsant level in about 2 hr. In light of this study, an acetaminophen-diazepam combination in separate suppositories may be suitable for the prevention of recurrent febrile convulsions in susceptible children, but its practical value and efficacy require evaluation in clinical experiments.
Acetaminophen↗
Sodium valproate, pregnancy, and neonatal hyperglycinaemia.
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Adult↗
Hydroxyproline metabolism in type II hyperprolinaemia.
Hydroxyproline metabolism was studied in two patients with type II hyperprolinaemia (HP II) using oral loadings of hydroxyproline or hydroxyproline-ornithine. delta 1-pyrroline-3-hydroxy-5-carboxylic acid (3 OH-PC) and delta 1-pyrroline-5-carboxylic acid (PC) were identified in the urine. The urinary excretion of both 3-OH-PC and PC increased in HP II patients but not in healthy controls during oral loading of hydroxyproline and hydroxyproline-ornithine. The plasma level of proline in patients with HP II is very high but the hydroxyproline concentration is normal or only slightly increased. Therefore one can assume that hydroxyproline is converted to pyrrole-2-carboxylic acid, which is excreted in urine as a glycine conjugate. In this study it was demonstrated that the highly elevated plasma level of proline in one of the patients with HP II decreased greatly after hydroxyproline-ornithine load; this change was followed by a 40-fold rise in urinary excretion of proline.
Adolescent↗
Achalasia sicca--juvenile Sjögren's syndrome with achalasia and gastric hyposecretion.
Sjögren's syndrome (SS) in its classical form, which includes keratoconjunctivitis sicca, xerostomia and recurrent enlargement of the salivary glands, is associated with a connective tissue disease in at least half the patients. According to the present study of three patients with SS, achalasia and gastric hyposecretion seem to be either further manifestations of SS, or separate phenomena associated with SS. The gastric hyposecretion involves both the hydrochloric acid and the total volume of the secretion, but the gastric mucosa has a normal appearance on microscopy. Because of the simultaneous presence of achalasia, gastric hyposecretion and reduced salivation, we have called the combination "achalasia sicca". The reduction in the secretions of the upper gastrointestinal tract might have a pathogenic association with achalasia.
Adolescent↗