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Biomedical subjects

F Schabel

Publications and source records attributed to F Schabel.

At least 19 recordsLinked to original sources
PubMed1988

[Experiences with the preventive use of aminophylline (Euphylline) in apnea and periodic respiration in infants].

Sleep apnea and periodic breathing in infants are the result of a mild hypoxia and they are the requirement for SIDS. As a results of the modern medicine each 4.-5. death is the cause of SIDS during the first year of life. The positive effect of theophylline on apnea of prematurity is known since 1973. We have given theophylline (Euphyllin) from 1979 till 1986 to 198 premature infants with a birth weight below 2000 g for apnea prophylaxis orally in a doses of 9 mg/kg body weight per day distributed in 3 to 4 doses for a period of 1 to 4 months. Since then primary apnea in premature infants haven't any importance. Beside the home-monitoring we give theophylline for SIDS. In 1986 and 1987 1041 healthy term newborn infants received theophylline over a period of 6-8 weeks and more then 300 newborns until 6 months and more. There were no deaths from these infants form the cause of SIDS. Since 1987 all newborn infants with a pathological hypoxia-test as a screening test for the risk of SIDS received Euphyllin until normalization. With this method we have reduced evidently the death rate of SIDS in our district.

Aminophylline↗
PubMed1987

[Apneas and periodic breathing in mature newborn infants].

50 healthy term newborn infants without a history of sudden infant death syndrome among their relationship were investigated between the second and fourth day of life using a modified test of Brady and McCann. In a thermoneutral environment pneumograms (respiration by thoracic impendance, heart rate, tcpO2) were obtained with an FiO2 of 21, 16, and 30% oxygen. In 12 of the 50 sleeping infants (24%) apnea periods or periodic breathing were observed with an FiO2 of 16%. This test will bring up abnormalities in the regulation of breathing. Similarities to the SIDS are discussed.

Blood Gas Monitoring, Transcutaneous↗
PubMed1985

[Infantile transitory distal renal tubular acidosis with bicarbonate loss].

Apart from the classic distal renal tubular acidosis (RTA), the proximal RTA, and a few cases of distal RTA and renal bicarbonate wasting we know only 2 cases of infantile transient distal RTA with bicarbonate wasting. A 3 month-old male patient is admitted because of deficient suction, vomiting and dehydration. Despite a strong metabolic acidosis (pH 7,09, bicarbonate 8,6 mMol/l, chloride 110 meq/l) the urine is constantly alkaline; clinically the disease manifests itself in the form of an alkali-resistant RTA. Accompanying troubles such as inner ear deafness, G6PDH deficiency, hyperparathyroidism and vitamin D intoxication are to be excluded. A bicarbonate study carried out with care so as to prevent extracellular fluid expansion reveals the lack of excretion of titratable acid (-2.4 to +4.7 mueq/min/1.73 m2), an reduced excretion of ammonium (5 to 24.8 mueq/min/1.73 m2) with regard to GFR (42.4 ml/min/1.73 m2), and a constant loss of bicarbonate (FE HCO3- about 10%) covering most of the bicarbonate plasma concentration, which results in a constantly negative net acid excretion. Even with alkalosis there is no urine minus blood pCO2 increase. The renal excretion of gamma GT is significantly reduced. On substitution with high quantities of bicarbonate (10 meq/kg BW/day) the defect heals up at the age of 13 months. The pathogenesis of this disease is not quite clear, but is similar to that of the Lightwood infantile RTA. The acidification defect may be explained by a deficient hydrogen ions--secretion in the distal tubule; as for kinetics, it is not in the proximal tubule that the bicarbonate wasting occurs but it may be due to increased sodium delivery to the distal nephron.

Acidosis, Renal Tubular↗
PubMed1985

[Recurrent hemolytic-uremic syndrome with positive immunofluorescence].

The hemolytic uremic syndrome is a disease of infancy, its major clinical manifestations include reversible thrombocytopenia, hemolytic anemia, and renal failure. Although a great number of patients with HUS have been published, relapses as well as positive immunofluorescence studies are rare findings. In our patient the disease began at age of 7 years and recovered completely. At 10 1/2 years a relapse occurred and despite therapy the patient died two months later. Renal biopsy showed severe arterial and glomerular changes with remarkable similarity to the histological findings in thrombotic thrombocytopenic purpura, which could be explained as secondary hypertensive damage, and dense granular deposition of fibrinogen, IgG, IgA, C3, and Clq along the capillary loops of the glomerulus and throughout the wall of the renal arteries. The clinical data, histological findings, and the particularities of our patient with this special course of HUS are discussed.

Child↗
PubMed1981

Characterization of the biochemical basis of a complete deficiency of the adenine phosphoribosyl transferase (APRT).

In order to study the biochemical basis of a complete deficiency of adenine phosphoribosyl transferase (APRT) the enzyme was purified to homogeneity, its properties were characterized, and antibodies raised. The enzyme is indirectly involved in adenine uptake. Apparently, by forming AMP the internal concentration of adenine is kept low allowing it diffusion. The same APRT is present in various tissues as was revealed by antibody inactivations employing anti-erythrocyte APRT as well as by direct enzyme assays in cells from the APRT deficient patient. In vitro cultured fibroblasts derived from this patient had less than 0.02% enzyme activity. No cross-reacting material was found in erythrocytes obtained from an APRT deficient child.

Adenine↗
PubMed1981

[Aarskog syndrome (author's transl)].

Report of radiologic and hormonal results of a patient with typical stigmata of the Aarskog-syndrome. X-Ray findings are not been found to give diagnostic clues, whereas the hormonal findings are considered specific-typical: FSH and LH levels prior to orchidopexy are in a range are as seen with hypergonadotropic hypogonadism. One year after the orchidopexy LH values were found to be normal, the FSH again showed increased titer. The growth hormones concentrations are normal but Somatomedin-activity is decreased.

Abnormalities, Multiple↗
PubMed1981

[Pharmacokinetic of antibiotics in patients with mucoviscidosis (author's transl)].

Inadequate therapeutic results in the treatment of bacterial infections in patients with Cystic Fibrosis prompted a reevaluation of pharmacokinetic parameters of orally and parenterally administered drugs in these patients. Gentamicin, Azlocillin and Ticarcillin are eliminated faster in patients with Cystic Fibrosis. Serum concentrations show a rapid decrease over 60 to 90 minutes and surpass MIC values of Pseudomonas isolates for a maximum of only 60 minutes. 70% to 90% of the administered amount of drug is eliminated within two hours in the urine. Concomitantly determined clearance rates for creatinine didn't show abnormalities, however they pointed towards an additional tubular secretion of Gentamicin which is not seen in healthy controls. Cephalexin, Epicillin and both components of Cotrimoxazol show a delay in oral absorption. The renal elimination of Cephalexin and Trimethoprim is unaltered, but the excretion of Epicillin and Sulfametrol is enhanced again. This is seen by a delay and decrease in the maximal serum concentration (Cmax), but increased urine recovery. Doubling of the dose of gentamicin administered as i. v. infusion over 45 to 60 minutes results in smooth serum curve, the MIC values of most encountered organisms are surpassed for 3 hours and more. The clinical applicability of this recommendation however awaits further investigations concerning efficacy and safety.

Adolescent↗
PubMed1980

[Glomerulonephritis following snake bite].

A snake bite may quite frequently lead to haemorrhagic symptoms ranging from hypofibrinogenaemia to disseminated intravascular coagulation. In rare cases acute renal insufficiency associated with tubular necrosis may occur due to a direct toxic lesion, shock symptoms or DIC. However, it is extremely rare that glomerulonephritis results from a snake bite. A thirteen-year-old girl develops gross haematuria, albuminuria and cylindruria without any shock symptoms two days after having been bitten by a European viper. Coagulation state and complement show slight changes, and the histological examination reveals proliferative nephritis with deposition of immune complexes. The clinical picture corresponds to recurrent haematuria; renal function is normal after follow-ups over a one-year-period.

Adolescent↗
PubMed1980

Hereditary deficiency of adenine phosphoribosyl transferase.

We describe a 12 year old patient who developed acute renal failure at the age of 18 months due to radiolucent "uric acid" stones. Measurement of APRT activity showed a complete dificiency in this patient (less than 0.02%). In the urine we found instead of uric acid 2.8-dihyroxyadenine as measured by high pressure liquid chromatography. The pedigree indicates an autosomal-recessive inheritance of this disorder.

Acute Kidney Injury↗
PubMed1979

[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↗
PubMed1979

[Polycystic disease of early infancy in two sisters (author's transl)].

Polycystic disease of early infancy is a heritable disorder diffusely involving both kidneys with no other evidence of renal parenchymal malformation. After discussing the typical histological data of two sisters with normal family history a short survey about classification and differential diagnosis of similar heritable renal cysts is given. With regard to the few other cases with familiar occurrence an autosomal recessive transmission is the most likely form of inheritance, delayed manifestation has not been observed until now.

Chromosome Aberrations↗
PubMed1979

[Hydrothorax during the neonatal period (author's transl)].

The congenital hydrothorax is a rare cause of the RDS in the newborn. Our observations on three patients with bilateral pleural effusions and 44 cases from the literature will be discussed; we emphasize the importance of this serious disease in the newborn period. The pathogenesis is largely unknown, however, its possible etiology like birth trauma or dysplasia of the lymphatic system are discussed. It should be pointed out that this condition can be rapidly recognized by radiographic examination and successfully treated. The reported survival rate is 29 out of 44 (66%).

Birth Injuries↗
PubMed1979

[Exaggerated somatomedin activity in the Beckwith-Wiedemann syndrome (author's transl)].

Beckwith and Wiedemann described the syndrome of exomphalos, macroglossia and gigantism with hypoglycemia and visceral organ hyperplasias. In some cases of severe hypoglycemias hyperplasia of beta cells of the pancreas was found. Hyperinsulinism, which has to date rarely been investigated, reacts strongly to beta cell stimulation and can hardly be suppressed. The cause of gigantism and organ hyperplasias is still unknown. After a short description of a case of hypoglycemias in the first two weeks of life a long-term profile of the endocrinologic abnormalities and carbohydrate metabolism is given. Growth hormone response to insulin is normal, tolbutamide is followed by severe hypoglycemias without an increase in the immunoreactive insulin levels; the activity of somatomedin is excessively increased. The high activity of somatomedin explains the high potency of growth in the different tissues and the hypoglycemic reactions and it seems reasonable to assume that somatomedin could create nesidioblastosis of the pancreas with hyperinsulinism and severe hypoglycemias. It is likely that the Beckwith-Wiedemann syndrome and the Laron type familial dwarfism with high plasma growth hormone, absent activity of somatomedin, and disorders in carbohydrate metabolism represent complementary diseases.

Abnormalities, Multiple↗
PubMed1979

[Tetracyclin intoxication versus idiopathic pancreatitis: report of a case with multiple organ involvement (author's transl)].

The tetracycline class of antibiotics is infrequently used in clinical pediatrics due to its side effects: they include anorexia, nausea, vomiting and diarrhea. Hypersensitivity, a photosensibility reaction and a brownish discoloration of teeth is less frequently, a pseudotumor cerebri is rarely seen. Once therapeutic plasma levels are exceeded however, either by overdosage or decreased renal or hepatic clearance of the drug, serious complications like a secondary Fanconi-Syndrom or a nephrogenic diabetes insipidus can occur. The increased toxicity of tetracyclines in pregnant women is well known. We would like to report a fatal case, where serious complications like a secondary Fanconi-Syndrom, toxic degeneration of the liver, a clinically undected pancreatitis and a protein loosing enteropathy are though to be either direct consequences of tetracycline overdosage or the indirect effect of a shocklike syndrom by means of a nonoliguric renal failure induced by tetracycline.

Acute Kidney Injury↗
PubMed1979

[Pneumoperitoneum in a newborn without intestinal perforation (author's transl)].

This is the report of the rare complication of an isolated pneumoperitoneum in a premature infant of 28 weeks gestation with artificial ventilation due to severe RDS. This rare occurrence in immature babies with artificial ventilation should be considered in the differential diagnosis of abdominal emergencies in this age group. Etiology as well as therapeutic consequences will be discussed.

Humans↗
PubMed1979

[Renal upper pole calyceal obstruction: clinical and radiological significance (author's transl)].

Obstruction of the superior renal calyces due to intrarenal vascular compressions is assumed on the basis of a characteristic sharply defined oblique filling defect with or without blunting of the fornices. Scintillation camera renography shows prolonged retention in the superior collecting system of the involved kidney. 11 out of 23 children had a symptomatology of recurrent hematuria, other origins of hematuria were excluded. 5 patients had urinary tract infection, the remaining 7 had normal urinary findings. Renal function was always normal. There is no correlation between severity of blunting and hematuria. In contrast to other studies which included only patients with blunting and ectasis even cases without blunting of the fornices have a clinical symptomatology. Deterioration of radiologic appearance and kidney function was not found. Any idiopathic hematuria should be investigated for calyceal obstruction. Uncomplicated cases require no therapy, long term follow-ups with regard to complications such as urinary tract infection and urolithiasis are indicated.

Adolescent↗
PubMed1978

[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↗