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Biomedical subjects

J Ziegan

Publications and source records attributed to J Ziegan.

At least 19 recordsLinked to original sources
PubMed1994

Enzyme activity patterns of myosin ATPase, alpha-glycerophosphate dehydrogenase and succinate dehydrogenase within different muscle fibre types.

Muscle fibre compositions of five different rabbit muscles were determined by combining two enzyme-histochemical reactions (NADH tetracolium oxidoreductase and myosin ATPase after alkaline preincubation). The differentiation into the fibre types, fast twitch glycolytic (FTG), fast twitch oxidative (FTO), and slow twitch oxidative (STO) was possible by a reliable staining classification. Aim of the study was the estimation of enzyme activity patterns within the three different fibre types. For this purpose, four serial cross-sections with several enzyme histochemical reactions were performed: alkaline combination method for fibre type determination, the reactions of myosin ATPase, alpha-glycerophosphate dehydrogenase (GPDH), and succinate dehydrogenase (SDH). The measurement procedure for the estimation of enzyme activities was based on the proportionality between the intensity of the enzyme histochemical staining reaction and the degree of enzyme activity. The activities of GPDH (indicator for glycolytic metabolism) and SDH (oxidative metabolism) were inverse. The calcium-activated myosin ATPase showed only little activity in slow twitch fibres after alkaline preincubation. In contrast to slow twitch fibres, ATPase activity in fast twitch fibres was relatively high. The results showed that the classification of muscle fibre types due to their myosin ATPase activities and their main metabolisms (oxidative and glycolytic respectively) is justified.

Animals↗
PubMed1989

[Nosologic evaluation of oculopharyngeal myopathic syndromes].

Three patients with oculopharyngeal involvement of neuromuscular origin are presented: one patient suffering from an oculopharyngeal neuromuscular disease (developing an oculopharyngeal muscular dystrophy?) and two patients representing a neuromuscular mitochondriopathy. There is no evidence that the oculopharyngeal myopathy and neuromuscular disease, respectively, are the same, despite the simularity of the syndrome. The different clinico-pathological types of oculopharyngeal syndromes are discussed. Following-up the development of further manifestations of the oculopharyngeal syndrome can helf classifying them.

Adolescent↗
PubMed1989

[Facioscapulohumeral neuromuscular syndromes--problems of differential and early diagnosis and genetic counseling].

Among 20 kinships affected by facioscapulohumeral and scapulohumeroperoneal neuromuscular syndromes 6 probands with autosomal dominant transmission and 5 obviously sporadic cases revealed myopathic changes on biopsy and were classified to have facioscapulohumer (operone)al muscle dystrophies. 5 autosomal dominant cases and one sporadic case with neurogenic muscle changes and 3 other non-dystrophic phenocopies emphasize the necessity of muscle biopsy for nosological assignment. Definite correlations between mode of inheritance, clinical and morphological findings could not be found. Pathological ultrasound and EMG findings in parents and children of seemingly sporadic cases suggest to assume autosomal dominant inheritance with incomplete penterance in such cases.

Adult↗
PubMed1989

[Secondary muscular carnitine deficiency following immunosuppressive treatment].

Carnitine deficiency syndromes can be classified into two groups: primary carnitine deficiency and secondary carnitine deficiency syndromes. A lipid storage myopathy with carnitine deficiency following an immunosuppressive therapy is described in a young man suffering from a possible polymyositis. After treatment with L-carnitine both biochemical and morphological features recovered. A secondary carnitine deficiency syndrome due to an immunosuppressive therapy is supposed.

Adult↗
PubMed1986

Myopathy with mitochondrial abnormalities and rimmed vacuoles.

A man of 44 years suffering from an exercise-induced neuromuscular disease with mitochondrial abnormalities and rimmed vacuoles is reported. The mitochondrial abnormalities and rimmed vacuoles (autophagic vacuoles) are interpreted as sequential changes of the same pathogenetic process depending on the degree of energy deficiency.

Extremities↗
PubMed1984

[Metabolic triglyceride storage disorders. A report of 2 cases of systemic carnitine deficiency].

Two cases of triglyceride storage in liver, kidney, heart, and skeletal muscle are described in infants who died at the age of 1 1/2 years and 4 d, respectively. In the first patient, a previously normal girl, the clinical symptoms began two months before death with encephalopathy (vomiting, unconsciousness), liver enlargement, hypoglycemia, increase in serum transaminases. These signs disappeared within the following days. Some weeks later she died during the second attack. The 4-d-old boy, the second child of healthy consanguineous parents, showed at the third day of life an impaired sucking, muscular hypotonia, respiratory arrest and bradycardia. An intensive therapy was inefficient. At autopsy gross examination showed only a moderately enlarged yellow liver and an edematous brain in the first case and pale organs in the second one but no cause of death. The microscopial examination of all tissues of both cases showed fat storage within the four organs mentioned above. The common histochemical methods for neutral lipids were positive, the Schultz-reaction for cholesterol and cholesterol esters was negative. The lipid loaden cells did not show birefringence in polarized light. A predominance and strong fat storage of the type I fibres was found in the skeletal muscle. The storage of triglyceride could be confirmed by histochromatography, a thin-layer chromatography of tissue sections. The triglyceride accumulation in liver, heart, kidney, and skeletal muscle is a characteristic feature of systemic carnitine deficiency. The clinical symptoms of the first patient are in agreement with reports of this disease also. A carnitine deficiency in a newborn was not yet described. Family studies revealed a low carnitine concentration in the mother's serum in both cases, while the serum of father and brother resp. sister showed normal carnitine levels.

Carnitine↗
PubMed1983

[Sporadic occurrence of benign myopathy with early contractures (Emery, Dreifuss and Rotthauwe)].

A myopathy which sporadically appeared in a young male corresponds clinically, electromyographically, histologically and cardiologically to the rare picture of the X-chromosomally recessive benign myopathy with early contractures. The coordination to the clinical picture mentioned and the genetic advice, taking into consideration the X-chromosomally recessive heredity, are discussed and proved.

Adult↗
PubMed1979

[Contribution to the formal origin of multiple branched ossifications in the lung].

The observation of multiple ossifications in the lungs as secondary findings of the post-mortem examination of a 62-year-old male with chronic cardiac stasis and emphysema of the lung is reported. Apart from bone nodules larger branched mature bone clasps with marrow caves as well as a in most cases fibromatosis with a small focus which represents the matrix of ossification is represented. Apart from this histologically a hyperaemia with an oedema rich in protein, focal precipitation of protein with formation of a granulation tissue and later fibrosation are to be proved as presteps of nodular fibromatosis which according to the kind of the desmal ossification changes into bones. The chronic haemostasis in the pulmonary circulation is thus apparently of importance in our observation. The case is compared with literature. Up to now about 65 of such observations are reported which nearly exclusively concern old men. The etiology remains unclear.

Emphysema↗
PubMed1979

[Combinations of enzyme-histochemical methods for differentiating of fibers types and evaluating the skeletal musculature (author's transl)].

This paper reports 2 combinations of enzyme-histochemical reactions (NADH tetrazolium oxidoreductase and myosin ATPase after alkaline preincubation, menadione-dependent glycerol-3-phosphate oxidoreductase and myosin ATPase after acid preincubation). One type of skeletal muscle fiber is stained golden-brown and the other blue. The differentiation of types of fibers is greatly improved by reliable classification. In addition, the same section can be used to determine the oxidative and glycolytic metabolic capacity, respectively. Finer differences in the structure of fibers may be recognized more easily and allowed of arrangement in a larger number of classes without dispensing with the old-established method of differentiating. It is possible for myopathological alterations to be made clearly visible. New and other combinations also offer promise of an advantageous extension of the method to other applications.

Adenosine Triphosphatases↗