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Biomedical subjects

W Tulzer

Publications and source records attributed to W Tulzer.

At least 37 records · Page 2Linked to original sources

[Clinical findings and diagnostic problems in a case of shunt nephritis. (author's transl)].

A report is presented on a nine year old girl in whom a Spitz-Holter valve had to be implanted after operation on a meningomyelocele while a neonate. Because of a macrohematuria and a hypochromic anemia, she was admitted as an inpatient. In addition, there was a pyuria with bilateral hydronephrosis. The clinical picture was initially misinterpreted as hemorrhagic cystitis with ascending pyelonephritis in neurogenic bladder. Only the reduction of serum complement suggested the presence of a shunt nephritis. The diagnosis was verified by kidney biopsy. After removal of the infected valve system, there was a prompt normalization of all laboratory parameters. Besides description of the case history, above all the diagnostic problems of this rare syndrome, which is nevertheless of practical importance, are dealth with.

Anemia, Hypochromic↗

[Therapy of Bartter syndrome with indomethacin].

In two children with typical clinical and laboratory findings of the B. S., the therapeutic effect of the prostaglandin synthetase inhibitor indomethacin could be unequivocally proved in balance studies performed under inpatient conditions. Under this medication, the serum potassium rose significantly and the potassium balance became positive. In parallel to this, the plasma renin (and in the case in which it could be regularly investigated also the plasma aldosterone) fell significantly. In one of the two patients, the hypertensin-test was performed before and under indomethacin treatment; the initial angiotensin resistance could be eliminated by Indocid. Both children have now already received Indocid for twenty-four and sixteen months. The preparation was adequately tolerated, and the clinical symptoms of B. S. have largely subsided. Noteworthy is a substantial catching up of growth in one of the two patients. Despite normal renin and aldosterone values, there was no complete normalization of the serum potassium, indicating that besides the elevation of certain renal prostaglandins in the pathogenesis of B. S. described by several authors, an additional (probably superordinate) mechanism is likely to play a role.

Adolescent↗

[Clinical findings, pathogenesis and treatment of Bartter's syndrome (author's transl)].

In three children Bartter's syndrome was diagnosed on the basis of the typical laboratory findings and the characteristic histological changes of the kidney. Apart from the description of three cases especially the latest pathogenic findings are represented because of their important therapeutic consequences. In one of the patients the therapeutic effect of the prostaglandin synthetase inhibitor Indomethazin was statistically proved in a balance study performed under inpatient conditions and so it was indirectly proved that the prostaglandines play an essential role in the pathogenesis of Bartter's syndrome. The patients have now received Indomethazin for a period of 11 months up to two and a half years with the result of an impressive improvement of the clinical symptoms and an unequivocal increase of the serum potassium. The fact that despite of normal renin and aldosterone levels there was no complete normalization of the serum potassium level indicates that in addition of prostaglandines probably a superior mechanism plays a part in the origin Bartter's syndrome.

Aldosterone↗

[Pathogenesis of ketotic hypoglycemia (author's transl)].

4 children with ketotic hypoglycemia (KH) showed during a fasting period over 24 hours significant higher decreases of serum alanine levels than normal controls. Insulin induced hypoglycemia was followed by only minimal increase of urine epinephrine secretion, while all controls showed more than 6 times higher increases. 2-desoxy-glucose-tests were pathological in all cases with KH. One can speculate, that there is a connection between the reduced availability of alanine and the adrenal medullary hyporesponsiveness. Epinephrine stimulates glycogenolysis in muscle cells. Lack of epinephrine reduces pyruvate production and subsequently alanine synthesis. Alanine however is essential for gluconeogenesis in liver cells especially during starvation. After some days administration of diazoxide the 2-desoxy-glucose-test was normalised in all patients. This observation could probably be of some interest in therapy of KH.

Acidosis↗

[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↗