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H Zieglauer

Publications and source records attributed to H Zieglauer.

12 recordsLinked to original sources

[Investigations on the pathogenesis of distal renal tubular acidosis (author's transl)].

In distal (type 1) RTA, renal acid excretion is impaired by the inability to establish adequate pH gradients between plasma and distal tubular fluid at any level of acidosis. Main clinical signs in infancy are anorexia, vomiting and failure to thrive. Despite low serum bicarbonate levels the renal threshold of bicarbonate is normal, while urinary pH levels are high even with values below the threshold. Under conditions of bicarbonate-induced systemic alkalosis urinary the pCO2 exceeds blood pCO2 in normal subjects. by contrast, the urinary pCO2 tension is not significantly greater in distal RTA, indicating a failure of the cells of the distal nephron to secrete hydrogen ions even without a gradient. Red cell carbonic anhydrase is within the normal range, whilst the inhibition of carbonic anhydrase activity has no effect on distal tubular function. Until now no histological or enzymatic defect could be detected to explain the ineffective acidification. Bicarbonate loading is followed by a lowering of calcium excretion to within the normal range and a decrease in the uncharacteristic renal hyperaminoaciduria.

Acidosis, Renal Tubular↗

Vitamin B-6 absorption in children with acute celiac disease and in control subjects.

The concentration of serum pyridoxal phosphate was determined before and 15, 30, 60, 90, and 120 minutes following an oral load test with 5 mg pyridoxine hydrochloride/kg body weight in 14 children with acute celiac disease and in 15 control subjects. Children with acute celiac disease suffer from a biochemical vitamin B-6 deficiency. The increase in pyridoxal phosphate of children with acute celiac disease after loading was significantly decreased when compared with that of control subjects. In children with celiac disease maximal concentration of serum pyridoxal phosphate appeared later (after 60 minutes) and was decreased in comparison to control subjects (after 30 minutes). A positive correlation existed between the net increase of pyridoxal phosphate 60 minutes following pyridoxine loading and the net increase of blood xylose 60 minutes after oral loading. The results are compatible both with a malabsorption of pyridoxine in childhood celiac disease and a shifting of the site of pyridoxine absorption from the upper part of jejunum into the more distal parts of intestine.

Acute Disease↗

[Artificial ventilation of preterm and term infants (author's transl)].

One of the first aims of the neonatal care unit of the Paediatric Department of the University of Innsbruck (Austria) consists in taking care of dangerously ill children of Tyrol including the neighbouring federal states and the alpine part of Italy (South Tyrol). From SUMMER 1974 to the end of October 1976 1342 preterm and term infants were treated. 142 of these children died (mortality rate 10.5%). In 94 patients, i. e. 6.9% mechanical ventilation was necessary. 39 of these children, i. e. 41.5% survived. Two indications of artifical ventilation were predominant: Hyaline membrane syndrome in 59 children (survival rate 49%) and apnoe in 22 children (survival rate 27%). Electroencephalography was performed in 39 children during the neonatal period. 34 of these children were severely ill (gestational age of 24 to 37 weeks), 5 children were born at term nevertheless moribund. 18 of the 23 dead children of this group showed severe alterations in electroencephalography. The cause of death of 10 of these children was cerebral bleeding as verified by autopsy. 8 of the 16 surviving children showed a regular psychomotoric development, 5 children of this group had a normal electroencephalogram.

Apnea↗

[The value of the "impression smear" in detecting giardia lamblia infection (author's transl)].

Giardia lamblia has a cosmopolitan distribution. The organism exists in two stages--the trophozoite and the cystic stage. Infected children may have acute or chronic diarrhea, crampy abdominal pain, anorexia, malasorption and poor weight gain and may be misdiagnosed as celiac disease. Infection may be selflimited or chronic even over years. Diagnosis is usually made by finding the characteristic cyst in stool specimens or by duodenal aspiration. Histological sections and impression smears (AMENT) of intestinal mucosa biopsies have been proved to be the most reliable method for detecting giardiasis. Evaluation of impression smears for parasites is easier and quicker than examining serial sections of biopsies. Out of 175 selected patients with intestinal complaints which were undergone small intestinal biopsy 11 were infected with giardia lamblia (6.2%). All infected children were symptomatic, malabsorption could be demonstrated in 5/8, lactase levels were reduced in most children. Examination of duodenal aspirates, stool specimens and histological sections (routine histology) alone would not have been diagnostic in every case. Evaluation of impression smears proved to be a reliable method in detecting giardia lamblia infection and is recommended whenever an intestinal biopsy is performed.

Adolescent↗

[Vitamin K deficiency bleeding as a leading symptom in celiac disease (author's transl)].

Haemorrhagic diathesis was a leading symptom in diagnosing celiac disease in 4 patients. In all 4 patients, a duodenal biopsy showed total villous atrophy. Although 3 of the children were typically dystrophic, the weight of the 4th child, an 8 month old boy, was within the normal range. In this patient, who suffered from neither diarrhea nor vomiting, heavy cutaneous and mucous membrane bleeding were the only symptoms of the disease. In all 4 cases the haemorrhagic diathesis could be explained by a low prothrombin complex, whereas the rest of the coagulation tests were normal. After the administration of Vitamin K1 there was an immediate rise in the prothrombin complex and bleeding was quickly stopped. Noteworthy is that due to infections, 3 of the 4 patients, received antibiotics just before the onset of the bleeding. In celiac disease, the conversion from a K-hypovitaminosis into a K-avitaminosis by the administration of antibiotics is discussed.

Anti-Bacterial Agents↗

[Tubular and intestinal phosphate transport in vitamin D-resistant (hypophosphatemic) rickets (author's transl)].

Renal clearance and intestinal absorption studies were performed to investigate the renal and intestinal handling of phosphate under various conditions in two female patients with VDRR. The two types of transepithelial phosphate transport in the renal proximal tubule and intestinal mucosa are discussed. The defective parathormone sensible transport mechanism is slightly depressed under the influence of parathormone and activated after treatment of vitamin D in one patient, in the other no further inhibition by PTH could be observed. Electrolyte excretion in urine remains stable during treatment with vitamin D 3. Therapy consists of administration of oral phosphate and of pharmacologic doses of vitamin D.

Child↗

[Acid-base balance during exchange transfusion (author's transl)].

During exchange transfusion with ACD blood severe metabolic acidosis may occur. ACD blood contains increasing amounts of acid which depends on the age of the blood. The mature newborn infant has different ways to compensate metabolic acidosis: the lungs, the immature kidneys and the conversion of citrate to bicarbonate. In preterm infants as well as in newborns with respiratory distress syndrome and mechanical ventilation the compensatory mechanisms may be reduced or even absent. Therefore ACD blood may lead to severe complications. To prevent acidosis adequate buffering with TRIS is recommended.

Acidosis, Respiratory↗

[Usefulness of the serum-xylose-test in comparison with small-intestine morphology in the malabsorption syndrome].

In 148 children, 4 months to 14 1/2 years old, 192 serum-xylose-tests (XT) and small bowel biopsies have been performed because of suspected intestinal malabsorption. The results of the XT and of the biopsies were compared to investigate the diagnostic value of the XT with respect to the morphology of the small bowel mucosa. Further we wanted to determine at which time after the oral intake of xylose the best correlation of XT and morphology can be expected. In children with normal mucosa the XT differed significantly from those with subtotal mucosal atrophy but not with partial villous atrophy. However, partial villous atrophy could be differentiated from subtotal villous atrophy with the same test. In children with subtotal villous atrophy a statistically significant age-dependency could be observed, comparing children being less or more than 2 years old and also a dependency from periods with gluten-free diet. Partial villous atrophy with or without coeliac disease cannot be differentiated by means of the XT. Best correlation of the XT and the morphology of the small bowel mucosa can be found 60 minutes after the oral xylose load; no additional information is obtained by more blood-xylose-determinations (e.g. 30, 90, 120, 180 minutes). Because of the rather high standard deviation of the individual blood xylose values no exact prediction can be made from the XT to the mucosal morphology. Therefore the XT is only of value for screening purposes and its results should not influence the indication for a small bowel biopsy, derived from clinical considerations.

Adolescent↗

Vitamin B6 nutriture of children with acute celiac disease, celiac disease in remission, and of children with normal duodenal mucosa.

Patients with adult celiac disease excrete abnormal amounts of tryptophan metabolites after loading with this amino acid, suggesting vitamin B6 deficienty in these patients, In fact, the excretion of tryptophan metabolites returns to normal after administration of vitamin B6. The vitamin B6 nutriture was measured by means of determination of pyridoxal phosphate and activity of pyridoxalkinase in serum and in duodenal mucosa of 14 children with acute celiac disease and of six children with celiac disease in clinical and biochemical remission. Ten children with normal duodenal mucosa were studied as controls. Children with celiac disease had significantly decreased pyridoxal phosphate in serum and in duodenal mucosa when compared both with children in remission and controls. Activity of pyridoxalkinase, however, was significantly increased in serum and in duodenal mucosa when compared with controls but not when compared with children in remission. These children had the same increase in pyridoxalkinase activity as children with acute celiac disease. These data provide a strong evidence for the occurrence of vitamin B6 deficienty in children with acute celiac disease. The children with celiac disease in remission still had an increased activity of pyridoxalkinase which seems to be a compensating mechanism in consequence of vitamin B6 deficiency prior to the gluten-free diet.

Celiac Disease↗