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R Ploier

Publications and source records attributed to R Ploier.

34 records · Page 2Linked to original sources

[Endocrinological aspects of ketotic hypoglycemia and adrenal calcification (author's transl)].

Case report on a 2 6/12 years old girl with bilateral adrenal calcifications and ketotic hypoglycemia. Adrenal function tests showed a normal response of the adrenal cortex but signs of adrenal medullary insufficiency. Urinary adrenaline in samples before and after insulin did not increase significantly and plasma adrenaline was undetectable during hypoglycemia. We suggest, that the absence of adrenaline, caused by perinatal adrenal hemorrhagia, is one of the possible pathogenetic keys of ketotic hypoglycemia and examinations on this disease should always include the search for adrenal calcifications.

Acidosis↗

[The value of the blood xylose test in children with the malabsorption syndrome (author's transl)].

Blood xylose levels were studied 30, 60 and 90 minutes after oral administration of D-xylose in 121 patients aged 2 months to 12 years with symptoms of malabsorption. The results show that the 60-minute test aline is sufficiently accurate and the dose of 15g xylose/m2 surface area seems perferable to a uniform dosage of 5g xylose. Reference values of blood xylose were determined in children with normal intestinal mucosa and prove a significant difference between babies and elder children. Comparison between the results of the xylose test in normal children and in different conditions of intestinal mucosa showed that the one-hour blood xylose test is of little value in the selection of cases requiring primary diagnostic intestinal biopsy in children with malabsorption. This test, however, might be valuable in the control of patients with diagnosed coeliac disease and as a screening test for other members of those families.

Age Factors↗

[Pathogenetic investigations on a case of mauriac syndrome (author's transl)].

The results of clinical and biochemical investigations on a girl with all obligatory signs of Mauriac syndrome already in infancy were compared with the different hypotheses suggested in order to explain the pathogenesis of this disease. One possible explanation for the origin of MS might be a decreased sensitivity of adenylate-cyclase to glucagon or adrenalin. Hypersensitivity to insulin, resulting in a decreased production of cyclic AMP and activation of glycogen synthetase could be excluded by measuring the urine excretion of cAMP with and without insulin. Furthermore no signs of dyspituarism were detectable on our case and the hypothesis of MS being a combination of primary glycogenosis and diabetes mellitus could also be refuted. Liver enzyme activities were normal.

Cyclic AMP↗

[A kin with a "silent" pseudocholinesterase gene (author's transl)].

After injection of short acting muscle relaxant suxamethonium on a 5-year-old boy during bronchography he was suffering from prolonged apnoe. Although using acetylthiocholin, butyrylthiocholin and benzoylcholin for tests we were not able to detect any activity of pseudocholinesterase in the patient's serum, Since there was no evidence of hepatic disease or hypoproteinemia, we supposed a genetically caused deficiency of serumcholinesterase. Examinations done on 18 members of this kin showed a complete absence of serumcholinesterase on 3 children (homozoygotes for "silent gene") and a significant decrease of pseudocholinesterase on 6 persons. It was not possible to detect the "silent gene" by counter immunelectrophoresis. The half value time after injection of purified human serumcholinesterase was between 8 to 9 days. Genetic aspects and clinical problems of the serumcholinesterase deficiency are discussed.

Adolescent↗

[Immunological aspects of a child with idiopathic pulmonary hemosiderosis and celiac disease].

A case report of a girl aged 3.5 years affected by the extremely rare combination of idiopathic pulmonary haemosiderosis (IPH) and coeliac disease (CD) is presented. It is the 13th such case that has been published over the last 25 years and only the 7th to be reported in a child. We believe that the concurrence of these two diseases is not coincidental, because a gluten-free diet had beneficial effects on the pulmonary symptoms not only in our case but also in other such patients. However, the pathogenetic relation between IPH and CD remains unclear. Although circulating immune complexes were detected in our patient's serum, there was no evidence of their putative damaging effect on the basement membrane of the alveolar capillaries. Furthermore, no IgA deposits could be demonstrated in alveolar basement membranes. Therefore the hypothesis that there is a reaction between IgA reticulin or endomysial antibodies and an alveolar basement membrane antigen with consecutive structural damage is unlikely.

Antigen-Antibody Complex↗

[Predictive value of latex agglutination on Helicobacter pylori in children with recurrent abdominal pain].

In a prospective investigation, a rapid latex test for Helicobacter pylori in the serum (Pyloriset) was carried out in 39 patients with recurrent abdominal pain aged from six to 15 years. The test was positive in 19 patients. All of these children were subjected to gastroduodenoscopy. Seven cases showed a Helicobacter-associated chronic active antrum gastritis, whereas in the remaining 12 children gastritis not induced by Helicobacter or normal mucosa was found. Of the 20 Pyloriset-negative patients, only five could be biopsied. One of these showed a Helicobacter pylori-induced antrum gastritis. The latex test investigated had a positive predictive value which was too low (37%) to make it helpful in deciding for or against gastroduodenoscopy and the general anesthesia mostly associated with this.

Abdominal Pain↗