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Biomedical subjects

F Manz

Publications and source records attributed to F Manz.

At least 127 records · Page 7Linked to original sources
PubMed1978

Renal magnesium wasting, incomplete tubular acidosis, hypercalciuria and nephrocalcinosis in siblings.

Polyuria, hyposthenuria, hypomagnesemia, hypercalciuria, advanced nephrocalcinosis, low citrate excretion and low glomerular filtration rates were observed in two female siblings who were followed over 10 years. Acid loading revealed incomplete distal tubular acidosis. Hypomagnesemia was due to renal magnesium wasting. It is suggested that the defect in tubular transport of magnesium is an important factor in the pathogenesis of this syndrome.

Acidosis, Renal Tubular↗
PubMed1978

Renal transport of amino acids in children with oculocerebrorenal syndrome.

Inulin and amino acid clearance studies were made in 6 boys with oculocerebrorenal syndrome (Lowe) aged 11 months to 5 years. Glomerular filtration rate ranged between 50 and 112 ml/min/1.73 m2. Clearance values of all amino acids were increased. Tubular reabsorption of cystine, ornithine, lysine and arginine was relatively more reduced than that of the other amino acids. In Lowe's syndrome the transport system of the basic amino acids and cystine appears to be more impaired than that of other amino acids.

Amino Acids↗
PubMed1977

[Severe factor XIII-deficiency. Studies on subunits and turnover of the fibrin stabilizing factor (author's transl)].

An infant with congenital homozygous factor XIII deficiency demonstrated a severe retroperitoneal and intracerebral bleeding with development of a posthemorrhagic hydrocephalus in the first months of life. Factor XIII activity was not measurable by means of enzymatic method and the antiserum inhibition test. Quantitative immunoelectrophoresis according to Laurell presented absence of the subunit A, whereas the concentration of subunit S was reduced to 47% the normal value. After replacement therapy factor XIII activity was estimated at 23% and corresponded to the concentration of the subunit A, concentration of subunit S increased by 20%. The turnover rate of fibrin stabilizing factor could be observed over a period of 39 days. The half life was estimated at 4,7 days. The child developed normally after continous substitution with 250 units of factor XIII concentrate every 6 weeks.

Cerebral Hemorrhage↗
PubMed1977

Determination of glomerular function in advanced renal failure.

In 15 children with advanced chronic renal failure, glomerular filtration rate was determined by different methods. Inulin clearance correlated well with the mean of creatinine and urea clearance, and also with 51-chromium edetic acid (EDTA) clearance measured over 24 hours. The absolute values of creatinine clearance and of 51Cr-EDTA clearance measured up to 8 hours were higher than inulin clearance. In advanced renal failure both the 51Cr-EDTA clearance measured over 24 hours, and the mean of creatinine and urea clearance, provide acceptable estimates of true glomerular filtration rate.

Adolescent↗
PubMed1977

[Congenital tracheal stenosis due to pulmonary artery sling (author's transl)].

Congenital stridor was observed in a newborn due to left pulmonary artery sling. Clinical signs and the course of the disease were determined by an extreme tracheal stenosis. In newborns and infants with apneic episodes, inspiratory and expiratory wheezing and cyanosis a pulmonary sling should be considered. Unilateral hyperexpansion of the lung and an anterior indentation of the esophagus are helpful diagnostic signs. Surgical treatment for pulmonary sling is possible but remains unsatisfactory. Patients with associated tracheobronchial stenosis show a high over-all mortality rate.

Autopsy↗
PubMed1977

Acid-base status in dietary treatment of phenylketonuria.

Blood acid-base status, serum electrolytes, and urine pH were examined in 64 infants and children with phenylketonuria (PKU) treated with three different low phenylalanine protein hydrolyzates (Aponti, Cymogran, AlbumaidXP) and two synthetic amino acid mixtures (Aminogran, PAM). The formulas caused significant differences in acid-base status, serum potassium, and chloride, and in urine pH. In acid-base balance studies in two patients with PKU, Aponti, PAM, and two modifications of PAM (P2 + P3) were given. We observed a change from mild alkalosis to increasing metabolic acidosis from Aponti (serum bicarbonate 25,8 mval/liter) to P3 (24,0Y, P2 (19, 3) and PAM (17,0). Urine pH decreased and renal net acid excretion increased. In the formulas PAM, P2 and P3 differences in renal net acid excretion correlated with differences in chloride and sulfur contents of the diets and of the urines. New modifications of AlbumaidXP and of PAM, prepared according to our recommendations, showed normal renal net acid excretion (1 mEq/kg/24 hr) in a balance study performed in one patient with PKU and normal acid base status in 20 further patients.

Acid-Base Equilibrium↗
PubMed1976

Renal substrate uptake, oxygen consumption and gluconeogenesis at low temperature.

One of the important needs in the surgery of renal transplantation is the long-term preservation of the removed kidney. Up to now, preservation time has been limited to about 72 hours. The viability of the stored organ decreases sharply with prolongation of the preservation over 24 hours. At present, two methods of preservation are widely used: the storage of the organ in a cold electrolyte solution, and the continuous, hypothermic perfusion with albumin or cryoprecipitated plasma [1]. Both methods require a low temperature to reduce the endogenous renal metabolism. Besides the quality of the removed kidney, the type of perfusate and the duration of perfusion, two other factors might influence the viability and later function of the transplanted kidney: the absolute temperature during preservation, and the supply of energy - yielding substrates to the isolated kidney [2,3]. Up to now, the influence of the latter factor on the metabolic performance of the kidney tissue has been studied only in a few investigations [4,5,6,7]. In the following study, we determined the uptake of various energy - yielding substrates, oxygen consumption and gluconeogenesis at various low temperatures in isolated rat kidney tubules.

Animals↗
PubMed1975

[Orbicularis oculi reflexes and evoked response of orbicularis oris muscle in Bell's palsy. A prognostic study (author's transl)].

In 84 patients with idiopathic, clinically complete Bell's palsy the electrically induced blink reflexes with their two components (OOR I and II) were electromyographically recorded on both sides using skin electrodes. In 67 of these patients the evoked responses of the orbicularis oris muscle were also studied. The latencies and amplitudes were measured and related to the clinical outcome of the facial paralysis. The patients were divided into two groups, one with good recovery of the palsy (46 patients), the other with significant residual paresis and/or strong associated movements of the facial musculature (38 patients). In the group with good recovery the following results were obtained: 1. the OOR I remained elicitable or reappeated during the first 12 days after the onset of palsy; 2. the OOR II began to rise during the first 10 days of palsy; 3. the amplitude of the orbicularis oris response did not decrease to below 10%. In the group with poor recovery: 1. both components of the OOR were absent or diminished to below 4% for more than 12 days after the onset of palsy; 2. the latency difference of the OOR I exceeded 8 msec; 3. the amplitude of the orbicularis oris responses decreased to below 10%. Using these criteria it appears to be possible in about 85% of patients to make a prognosis between the 3rd to 5th and the 10th to 12th day after the onset of Bell's palsy.

Adolescent↗