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M Kirchhof

Publications and source records attributed to M Kirchhof.

9 recordsLinked to original sources
PubMed2000

[Late diagnosis of Curschmann-Steinert myotonic dystrophy in a female patient with dilated cardiomyopathy and in her son].

A 41 year old woman presented with dyspnoea at rest and swollen legs in the emergency room of our centre. She reported a history of slowly progressing dyspnoea and oedema in the legs. Physical examination showed signs of biventricular congestive heart failure and dysmorphia of the face. Routine laboratory examination revealed elevated CK levels without significant elevations of the CK-MB isoform. ECG showed complete left bundle branch block and first degree atrioventricular block. Echocardiography and angiography showed markedly reduced left ventricular systolic function, the ejection fraction was 25%. Coronary angiography excluded CAD and there was no evidence for congenital or valvular heart disease. The patient also reported a history of a serious complication during emergency general anaesthesia and cataracts of both eyes. Because of the clinical and chemical findings, the history of cataracts and complications during general anaesthesia, a systemic congenital disease of the muscular tissue was suspected. Molecular studies revealed a trinucleotide amplification at the myotonic dystrophy locus 19q 13.3, so the diagnosis myotonic dystrophy Curschmann-Steinert was established. The sixteen year old son of the patient suffered from an at this time unknown disease with retardation, muscular weakness and myotonia of the face. The diagnosis myotonic dystrophy was evident because of the clinical signs and the family history.

Adolescent↗
PubMed1982

Natural course of primary biliary cirrhosis. I. A morphological, clinical and serological analysis of 103 cases.

Clinical, biochemical and serological data obtained in 103 patients with primary biliary cirrhosis (PBC) were analysed with respect to the four defined morphological stages. Evaluation of the initial biopsies (99 needle biopsies/4 wedge biopsies) revealed that most patients were in stage I (focal bile duct destruction). Unequivocal distinction between stages I and II was possible in most cases, while considerable overlapping of criteria was observed in stages II to IV. Morphological cholestasis, a characteristic sign of stage IV was already found in 7% of PBC I cases. Four out of 12 autopsy specimens showed micronodular biliary cirrhosis (Hanot's type) and eight specimens had a macronodular type of biliary cirrhosis. No predominant clinical symptoms were found in patients with PBC I or II, but pruritus was observed in about 30%. Increased serum alkaline phosphatase (AP) and IgM levels as well as a positive antimitochondrial antibody (AMA) test were typical features of all stages in up to 80-90%, but patients with normal AP or IgM or negative AMA have been observed, especially in stages I and II. Five of 57 patients at stage I had increased bilirubin levels and in three patients IgM and IgG were simultaneously elevated in stage I. The natural course of PBC, as it is reflected in histological staging, was studied in 30 patients in whom biopsies were regularly taken over a period of 2-18 years. About 80% of PBC I-cases lasted between 1 and 7 years before reaching stage II, while another 5-10 years were necessary for the development of stage III-IV. Thus it appears that in the vast majority of patients PBC lasts about 10-15 years and in some instances even more than 20 years. The finding in stage I and II of normal AP indicates a benign course, while morphological and biochemical cholestasis seems to be associated with a rather progressive course.

Adult↗
PubMed1977

[Explorative laparoscopy or laparotomy? (author's transl)].

Explorative laparoscopy is an endoscopic examination of the abdominal cavity which, with the modern endoscopy tables, permits a considerably improved survey than formerly. Within one year, 105 patients were examined and a suspected tumor was confirmed 20 times in 57 patients. Metastases were sought 23 times in patients known to have a primary tumor, and demonstrated 11 times. The differential diagnosis of cholestasis was made 18 times, 6 times a negative cholecystogram required further clarification. Two of these showed an inoperable, infiltrating, growing carcinoma of the gall bladder. For the purpose of classification of lymphogranulomata, laparoscopy shows the advantage over laparotomy of careful magnifying observation of the surface of the liver which can be repeated as often as desired.

Abdominal Neoplasms↗
PubMedDate not supplied

Characterization and clinical relevance of a new complement-fixing antibody--anti-M8--in patients with primary biliary cirrhosis.

A new complement-fixing antimitochondrial antibody--anti-M8--was detected in patients with primary biliary cirrhosis. Anti-M8 was only found in association with anti-M2, however, not all anti-M2 positive patients had anti-M8. Thus, among 66 anti-M2 positive patients, 29 were also positive for anti-M8, whereas sera from patients who had the complement-fixing anti-M2 and anti-M4 antibodies in parallel always strongly reacted with the M8 antigen. This group was previously described as mixed form. The M8 antigen was isolated either from human liver mitochondria or pig kidney microsomes and could be clearly distinguished from the M4 antigen. In contrast to M4, M8 was trypsin sensitive and banded at sucrose densities from 1.16 to 1.24, while M4 was found at densities from 1.08 to 1.14. Like M4, the M8 antigen also co-purified with outer mitochondrial membranes. Fifty-three patients with primary biliary cirrhosis have been followed over a period of up to 16 years and were classified according to their complement-fixing antimitochondrial antibody profile. At the time of the first diagnosis, 95% of 31 patients being anti-M2 positive, but anti-M8 negative (antimitochondrial antibody Profile I) were in Stage I or II. In contrast, only 61% of 13 patients being anti-M2 and anti-M8 positive (antimitochondrial antibody Profile II) and 44% of 9 patients with anti-M2, anti-M8 and anti-M4 in parallel (antimitochondrial antibody Profile III) belonged to Stage I or II.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies↗