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Biomedical subjects

K Langes

Publications and source records attributed to K Langes.

26 records · Page 2Linked to original sources
PubMed1985

Cardiomyopathy associated with Leigh's disease.

Clinical and postmortem findings in a female infant, suffering from Leigh's disease and cardiomegaly are described. The cardiac enlargement was due to symmetrical thickening of both ventricular walls and the septum. On light microscopy a widespread fibre disarray with a slight predilection for the ventricular septum was observed. Ultrastructural changes included an extreme reduction in the number of myofibrils and an excess of mitochondria. Abnormalities of the mitochondrial structure with tubular and myelinic transformation of the cristae suggested that a mitochondriopathy is responsible for the cardiomegaly in Leigh's disease.

Autopsy↗
PubMed1985

[Acute course of Leigh syndrome with hypertrophic cardiomyopathy in a female infant].

A female infant already as a newborn progressively developed nutritional disturbances, metabolic acidosis, muscular hypotension and other neurologic changes, and on its 20th day of life suffered from an acute cardiac and respiratory insufficiency due to pericardial effusions causing cardiac tamponade. In spite of intensive care including mechanical ventilation the child died on the 58th day of life. Cardiac changes and cranial CT-scans are demonstrated. The characteristic symptomatology as well as blood chemistry led to the tentative diagnosis of Leigh's disease. Postmortem examination confirmed this diagnosis.

Acidosis↗
PubMed1984

Congenital Leigh's disease: panencephalomyelopathy and peripheral neuropathy.

A dystrophic newborn girl (38th week of gestation) presented as a floppy infant with relapsing episodes of lactic acidosis and progressive cerebral deterioration. She died after serious apnoea at the age of 8 weeks. Neuropathological examination demonstrated widespread changes of Leigh's subacute polioencephalomyelopathy affecting the cerebral cortex, basal ganglia, mesencephalon and spinal cord. In addition, there was severe leukoencephalopathy of the cerebral hemispheres with sudanophilic tissue degregation, advanced axonal loss and reactive astrocytic gliosis. There was marked demyelination of the sciatic nerves. Electron-microscopical examination of the hypertrophic heart revealed pathological mitochondria with tubular inclusions suggestive of primary mitochondrial cardiomyopathy. Similar mitochondrial changes were not detected in the CNS and PNS. The complex of neuropathological alterations in this case is nevertheless consistent with a congenital variant of Leigh's disease.

Brain↗
PubMed1984

Pressure-induced hypertrophy in hearts of dwarf pigs.

Myocardial DNA-synthesis has been investigated in five adult dwarf pigs by autoradiography, light and electron microscopy 5, 10, 20, 50 and 100 days after banding the ascending aorta. By the banding procedure the mean ventricular pressure in the left heart rose to 40%, reaching an average of 74% at the time of pulse-labelling with 3H-thymidine. Absolute mean heart weights of the hypertrophic hearts reached 165 +/- 25 g in contrast to ten normal hearts with an average of 69 +/- 8 g. The highest labelling indices were found 10 days after onset of pressure-load: 0.46% in the left ventricular wall, 0.35% in the septum and 0.55% in the right ventricular wall. After 20 to 100 days of aortic constriction the labelling indices ranged from zero to 0.25% indicating a continuous slight DNA-synthesis. Electron micrographs demonstrate activation of the rough endoplasmic reticulum and synthesis of myofilamental and myofibrillar structures, changes of the number and size of mitochondria, the nuclear membrane, as well as changes due to hypoxia and degeneration.

Animals↗
PubMed1983

Postnatal DNA-syntheses and mitoses in hearts of dwarf pigs.

Postnatal DNA synthesis and mitoses have been investigated in the heart muscle of 23 dwarf pigs by autoradiography and by light and electron microscopy. During the first weeks the average labelling indices of the myocardium decrease from 3.7 +/- 1.6% to 1.9 +/- 0.6%, while considerable individual variations are observed. The average mitotic indices decrease from 1.0 +/- 0.7% on the tenth to 0.2 +/- 0.1% on the 30th day. The number of two synchronous mitotic figures in the same cell increases from 7.5 to 32.5% in the first postnatal month and causes the mitotic development of nuclear rows. The period between 2 and 8 months after birth is characterized by low DNA synthesis at least in one of the investigated ventricular tissues.

Aging↗