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Biomedical subjects

N Böhm

Publications and source records attributed to N Böhm.

At least 91 records · Page 5Linked to original sources
PubMed1978

Excretion of 2-methyl-3-oxovaleric acid in propionic acidemia.

A new case of propionic acidemia is presented, paying special attention to the early symptoms of this disease, such as increased drowsiness, muscular hypotonia, poor feeding, hypothermia, metabolic acidosis, ketonuria and vomiting. Investigation by gas chromatography (GC) and gas chromatography-mass spectrometry (GC-MS) revealed the excretion of fairly high amounts of 2-methyl-3-oxovaleric acid, a condensation product of two molecules of propionyl-CoA, as well as the known pathological metabolites such as propionic, 3-hydroxypropionic and methylcitric acids. Among the post mortem findings the histological studies of the liver were the most remarkable.

Acidosis↗
PubMed1978

[Experimental investigations on arter injury due to bone cement (author's transl)].

The observation of eight patients with lesions of great arteries after total hip replacement led to experimental investigations to determine whether Palacos might cause such vessel lesions. Palacos was applied to the adventitia of the abdominal aorta and both iliac arteries in rabbits. Angiographic examinations 14 days after implantation indicated no lesion at the intima of aorta and iliac arteries in the regio of Palacos. Histological investigations showed only necrosis of fat tissue and a slight distention of the muscle fibers of the artery wall. Lesions of aorta or great arteries are not caused by implantation of Palacos.

Animals↗
PubMed1978

Changes in cell population kinetics during epidermal carcinogenesis.

The changes in cell population kinetics in response to a mechanical stimulation by wounding were studied in a hyperplastic epidermis, papilloma and a squamous cell carcinoma of the mouse dorsal skin induced by repeated treatments with 20-methylcholanthrene. The G0 cell population, which consists of about 66% of the basal cells in the dorsal epidermis of the normal mouse, disappeared in hyperplastic epidermis, papilloma and carcinoma. The zone of proliferating cells enlarged in the papilloma, including several supra-basal layers, in contrast to its strict localization in the basal layer in hyperplastic epidermis. Proliferating cells were distributed in almost all areas in the carcinoma without localization. The normal responses to wounding were preserved in the hyperplastic epidermis, except for the absence of G0 cells, while the papilloma and carcinoma showed no increase in proliferating cells or shortening of the cell cycle time. From the view-point of cell population kinetics, papilloma and carcinoma are regarded as the tissues in which the flexible control mechanisms of irreversible cell differentiation to mature cells and of the length of cell cycle time are lacking.

Animals↗
PubMed1978

Chondroectodermal dysplasia (Ellis--van Creveld syndrome) with dysplasia of renal medulla and bile ducts.

A case of a 28-month-old boy with chondroectodermal dysplasia (Ellis-van Creveld syndrome) is reported. Besides polydactyly, ectodermal dysplasia, acromelic dwarfism and congenital heart defect, which are characteristic morphologic features of the syndrome, additional dysplastic developmental defects were discovered in the kidneys, liver, and lungs. Detailed histopathologic studies of the growth plates of tibia, femur and ribs disclosed an irregular, partly hyperplastic, partly dystrophic appearance of the epiphyseal cartilage, which was not resorbed properly by the invading blood vessels. Focal areas of necrosis occurred and barriers of tongue-shaped cartilaginous peninsulas persisted. Regular enchondral ossification was hindered and compensatory membrane ossification was found in the fibrosing metaphyseal bone marrow adjacent to the cartilage. Dysplasia and fibrosis of the renal medulla plus interstitial fibrosing nephritis in the cortex resulted in kidney contraction and renal failure. Hepatomegaly, portal fibrosis and bile duct hyperplasia and dysplasia were detected at autopsy, but did not have any clinical significance. These findings once again emphasize that derivatives of all three germ layers are involved in the Ellis-van Creveld syndrome. The possibility that a single metabolic or structural abnormality of the mesenchymal tissues could be responsible for the various organ dysplasias is discussed.

Bile Ducts↗
PubMed1977

Cytofluorometric analysis of cell proliferation and differentiation of the human erythroblasts.

The method of cytofluorometric measurement of the contents of Hb and nuclear DNA on a single erythroid cell (Fukuda et al., l975; 1977a) was used for the quantitative analysis of the erythropoiesis in normal bone marrow. The intracellular Hb in an erythroid cell was converted to fluorescent porphyrin after removing the Giemsa staning by irradiation with violet light in the presence of SH-donor (mercaptoethylamine hydrochloride, MEA) and its nuclear DNA was subsequently stained with pararosaniline Feulgen staining. With the two quantitative parameters, Hb content and DNA amount, the erythroid cells in normal human bone marrow were classified into 6 classes of different maturation stages (EI-EIV). The morphological characteristics of the most primitive erythroblast (EI cells) were described. The "proerythroblasts" which were identified on the bases of morphological criteria had in general aneuploid amounts of nuclear DNA with disproportional contents of Hb, thereby indicating that they are rather aberrations from the normal steps of cell maturation. The DNA amounts of "orthochromatic erythroblasts" (EV cells) showed continuous decrease from diploid range to almost zero suggesting that the removal of nuclear DNA from the erythroblast is not exclusively due to mechanical explusion of a whole intact nucleus.

Adult↗
PubMed1977

[Early embryonal maldevelopment of the umbilical cord with defect of the abdominal wall and severe body malformations (dysplasia umbilico-fetalis) (author's transl)].

A case of dysplasia umbilico-fetalis is demonstrated. The malformation comprises a rare and strange reduction anomaly of the feto-umbilical unit, which is caused during early embryonic life (around the 7 mm stage, corresponding to the end of the third week of gestation). The cause of the damage is not known. The folding off of the embryo from the yolk sac and the development of the abdominal stalk are impaired. No abdominal wall is formed, and the umbilicus and umbilical cord are not developed. The abdominal organs are enclosed in a short amnion-mesoderm tube, which is bordered by the retroperitoneum at the fetal side and by the chorionic plate at the placenta side. The umbilical vessels are only a few centimeters long and traverse in the mesodermal layer of the amnion-mesoderm tube. The thin wall of the tube usually ruptures before birth, thus causing an abacterial fibrinoid peritonitis by the chemical irritation of the peritoneum through the constituents of the amniotic fluid. The lower extremities of the fetus reveal varying degrees of reduction deformities. One leg and large parts of the pelvis may be entirely missing, if the side of origin of the extremity is included in the amnion-mesoderm tube. Severe kyphoscoliosis is probably a secondary phenomenon. In addition, malformations of the inner organs occur such as caudal displacement and hernia of the diaphragma, hypoplasia of the lungs, dysplasia of the genito-urinary tract and non-rotation of the gut. The pathophysiology of the rare developmental defect and its secondary implications are discussed with special reference to related malformations such as omphalocele and infraumbilical defect of the abdominal wall.

Abdominal Muscles↗