Early childhood cirrhoses (ECC) in Germany between 1982 and 1994 with special consideration of copper etiology.

In a multicentric retrospective clinical study with 16 pediatric centres we identified 103 cases of histologically confirmed early childhood cirrhosis (ECC) in Germany for the years 1984-1994. The most prominent diagnoses were congenital bile duct anomalies (47.5%), inborn metabolic disorders (17.5%) and unclear etiologies (17.5%). Chronic and excessive intake of copper might be discussed as an etiological factor in 8 other cases. 5 of these were proven to have coincided with very high hepatic copper contents and copper plumbing/acid well water. Their connection with copper exposure must be considered as probable, whereas 3 others were only suspected copper cases, mainly due to reliable exclusion of other etiologies. High corrosivity (base capacity) values and copper levels in the water for infants formula of 9-26.4 mg/L were determined in those probable cases for which exposure conditions could be exactly reproduced. Additional reports on copper associated ECC, either Indian Childhood Cirrhosis (ICC) from outside India or so-called Idiopathic Copper Toxicosis ( ICT ), originate from Austria, Australia, Germany, Ireland, USA.

[Leopard spot chorioretinopathy. Initial manifestation of recurrent acute lymphocytic leukemia]. / Leopardenfleck-Chorioretinopathie. Erstes Anzeichen eines Rezidivs einer akuten lymphozytischen Leukämie.

An 11-year-old girl with a history of acute lymphocytic leukemia of the central nervous system had attained complete remission for almost 3 1/2 years after combination chemotherapy and radiotherapy when she developed iritis and chorioretinopathy of the right eye. Neither an anterior chamber tap nor a diagnostic vitrectomy revealed leukemic cells. Both nonspecific anti-inflammatory therapy and antiviral treatment were unsuccessful. Finally, lymphoblasts were detected in the cerebrospinal fluid and in the bone marrow after repeated lumbar puncture and bone marrow aspiration. Combination chemotherapy alone was resumed, resulting in the resolution of all acute ocular symptoms and bone marrow involvement. Only the leopard-spot-like pigmentary fundus changes persisted. The child has now remained in continuous complete remission for 1 1/2 years.

Bilateral exudative retinal detachment in pregnancy.

A case of bilateral exudative retinal detachment associated with otherwise normal pregnancy is presented. Similar cases, but associated with toxemia of pregnancy from the literature are discussed. As far as we know, this is first reported case where bilateral exudative retinal detachment presented in a normal pregnancy and the re-attachment started promptly after a normal, spontaneous delivery. The re-attachment was complete, and the visual acuity after one year was 20/20 o.u. This case supports earlier theories that hypertension is not the only cause of exudative retinal detachment during pregnancy.

[Studies of biotransformation and liver function from the occupational medicine viewpoint]. / Biotransformation und Leberfunktionsprüfungen aus arbeitsmedizinischer Sicht.

General principles of biotransformation are described. Using examples from literature interindividual differences in the biotransformation capacity (induction and disposition) are discussed, especially concerning the combined effect of several xenobiotics (i.e., chemical working materials, ethanol, and drugs). The indocyanine greene excretion rate, the galactose elimination capacity and the 14C-aminopyrine breath test are described in detail as quantitative liver function tests.

Mitochondrial enzymes in human serum: comparative determinations of glutamate dehydrogenase and mitochondrial aspartate aminotransferase in healthy persons and patients with chronic liver diseases.

We measured the activities of two mitochondrial enzymes, the mitochondrial form of aspartate aminotransferase (EC 2.6.1.1) and glutamate dehydrogenase (EC 1.4.1.2), in the serum of apparently healthy persons (n = 84) and patients suffering from chronic liver diseases (n = 43). The distribution of activities for glutamate dehydrogenase, but not mitochondrial aspartate aminotransferase, was sex-dependent. The upper limits of the reference intervals (99th percentile) at 37 degrees C were 3.2 U/L for mitochondrial aspartate aminotransferase, 6.4 U/L for glutamate dehydrogenase (women), and 11.0 U/L for glutamate dehydrogenase (men); there was a weak correlation between the activities of both mitochondrial enzymes (r = 0.439). In patients with chronic liver diseases we found a greater increase in the activity of glutamate dehydrogenase than of mitochondrial aspartate aminotransferase and the correlation between the two mitochondrial enzymes was stronger. The diagnostic sensitivity and specificity of either mitochondrial enzyme was less than that of total aspartate aminotransferase, alanine aminotransferase (EC 2.6.1.2), or gamma-glutamyltransferase (EC 2.3.2.2).

JPC-80, a new slow acting antirheumatoid drug.

JPC-80 has been administered to man for the first time. Twelve patients received doses up to 300 mg daily for up to 12 weeks. Three suffered significant, but not serious, side effects: 1 skin rash, 1 elevation of liver enzymes, 1 diarrhea. All signs or symptoms returned to normal on discontinuation of drug. Four of 8 patients receiving cumulative doses of over 20 g showed moderate or major improvement. In patients with clinical improvements the mean erythrocyte sedimentation rate fell from 51 to 21, and rheumatoid factor titers fell from 1444 to 647. This pilot study justifies additional controlled studies.

An unusual family with retinoblastoma.

Reported here is an unusual family with retinoblastoma. The father has spontaneously regressed, bilateral, multifocal retinoblastoma with complete preservation of vision. Three of his six children have had bilateral retinoblastoma requiring bilateral enucleation. At least two of these three children have an associated mental and physical retardation. Chromosome number 13 in this family was normal.

Influence of posture on hepatic blood flow.

Hepatic blood flow was measured in 28 patients in supine and prone positions using the 133Xe-inhalation washout method. Even though the reactions in individual patients were considerably different, a mean blood flow of 60.9 ml/100 g/min was unaltered in both positions. This constancy of hepatic blood flow values is valid for patients without liver disease, with chronic hepatitis, and with liver cirrhosis.

The apoenzyme of aspartate aminotransferase and alanine aminotransferase in the serum of healthy persons and patients suffering from liver diseases.

In activity determination with addition of pyridoxal 5'-phosphate (P-5-P), aspartate aminotransferase (AST) activity increases by 6.5 U/l and that of alanine aminotransferase (ALT) by 2.5 U/l in the serum of healthy persons. This corresponds to a relative stimulation of initial activity by 37% and 15.2%, respectively. ApoAST activity in patients with chronic liver diseases is not changed as compared with that of healthy persons, the relative stimulation rate, however, is significantly smaller. ApoALT activity and corresponding relative stimulation is significantly greater as compared with healthy persons. In the case of acute viral hepatitis, a decrease of AST and ALT activity is followed by a decrease of apoenzyme activity in the course of disease. Diagnostic evidence of determinations of aminotransferase activities could not be improved by addition of P-5-P.

Lecithin: cholesterol acyltransferase activity in patients with chronic liver diseases and positive LP-X tests.

Lecithin: cholesterol acyltransferase activity has been measured in serum from patients with various liver disorders and positive LP-X tests and from healthy subjects. Statistical analysis indicated that lecithin: cholesterol acyltransferase activity provided no help in the discrimination between the persons of both groups although the mean activities differed significantly.

[Expert testimony and rehabilitation in liver cirrhosis]. / Begutachtung und Rehabilitation bei Leberzirrhose

The principles of rehabilitation of patients with chronic liver disease are expert opinion, therapy and social care. The questions relevant for clinical practice concerning fitness for work, invalidity, disability certificate and nursing money are explained for patients with liver cirrhosis. In such cases a differentiated expert opinion is recommended taking into consideration activity and portal hypertension. The principles of therapy and social care are discussed. Finally with the help of an analysis of the number of patients in the dispensary of the II. medical clinic of the Charité is demonstrated that only about 50% of all cirrhotics are invalidity pensioners. Nearly 1/3 of all cirrhotics with porto-systemic anastomoses has become capable for work.