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Biomedical subjects

R Heipertz

Publications and source records attributed to R Heipertz.

At least 19 recordsLinked to original sources
PubMed1979

[Water intoxication and brain edema in psychogenic polydipsia (author's transl)].

A case of psychogenic polydipsia is presented that showed psychic decompensation and compulsive drinking under the acute stress of an imminent operation for ovarian cyst. Without any indication of an underlying organic disease process the patient developed acute water intoxication due to the uncontrolled intake of water from the tap, this caused hyponatremia, brain edema, coma and status epilepticus. The physiology of water intoxication is reviewed in relation to this case, which is also remarkable for the acute onset and the shortness of the polydipsic state.

Adult↗
PubMed1979

Basic findings and current developments in sphingolipidoses.

Sphingolipidoses are caused by recessively inherited deficiencies of lysosomal hydrolases. The clinical backgrounds of and current biochemical and genetic approaches to the different forms and variants of gangliosidoses, trihexosylceramidosis (Fabry's disease), galactosylceramidosis (Krabbe's disease), sulfatidoses (metachromatic leukodystrophies), glucosylceramidosis (Gaucher's disease), sphingomyelinoses (Niemann-Pick disease) and ceramidosis (Farber's disease) are presented.

Fabry Disease↗
PubMed1979

The activity of 2',3'-cyclic nucleotide 3'-phosphohydrolase in human cerebrospinal fluid.

A method for the determination of cyclic nucleotide phosphatase (CNP) activity in cerebrospinal fluid is presented. In normal CSF the activity of CNP is very low. Comparing CSF from patients with multiple sclerosis to control CSF no significant difference is found, although a small proportion of MS patients has an elevated has an elevated CNP activity in their CSF. It is postulated that similar to other substances the CNP activity in CSF probably does not derive from the central nervous system.

Central Nervous System↗
PubMed1979

Primidone metabolism in renal insufficiency and acute intoxication.

Primidone (PRIM) is metabolized into phenobarbital (PB) and phenylethylmalonamide (PEMA). During anticonvulsant therapy with PRIM under normal conditions PB represents by fat the largest portion of the total concentration of all three components (PRIM + PB + PEMA). In combined therapy with diphenylhydantoin (DPH), and during chronic PRIM overdosage, the relative concentration of PB is even higher. A case of renal insufficiency while on PRIM therapy and a case of acute PRIM intoxication are presented. In both cases PRIM and PEMA are elevated while PB is relatively low. The mechanisms involved in this phenomenon are discussed. Excluding young children with chronic PRIM overdosage, and the endogenous and exogenous intoxication described here, a relative PB concentration below 40% indicates a lack of patient compliance if a steady treatment schedule has been maintained for at least 3 weeks.

Acute Disease↗
PubMed1979

Magnesium and inorganic phosphate content in CSF related to blood-brain barrier function in neurological disease.

In normal controls and in a large number of neurological patients divided into certain disease groups both Mg and PO4 were determined in cerebrospinal fluid (CSF) and serum. For both Mg and PO4 there was a marked concentration gradient between CSF and serum in normals where Mg was higher and PO4 content lower in CSF. Comparison of CSF values with serum values of patients showed pathological changes only in CSF, serum values always being within the control range. A number of disease processes associated with a disturbance of blood-brain barrier (BBB) function such as inflammatory CNS disease or CNS tumors showed significant alterations of PO4 concentrations in CSF which are interpreted as an approximation of serum values. A similar decrease of Mg did not reach statistical significance. Both Mg and PO4 in CSF showed a correlation with CSF protein concentrations, but no relationship with cells in CSF. Patients with cerebrosvascular disease were not significantly different from controls as regards their Mg and PO4 in CSF, but a small subgroup consisting of patients with an intracranial hemorrhage showed elevation of both Mg and PO4 which could signify cell necrosis rather than BBB dysfunction. Patients with disc protrusion or peripheral neuropathy did not demonstrate any abnormality of CSF Mg and PO4. In the multiple sclerosis group individual patients had elevated CSF concentrations of PO4 but the group as a whole is not different from the controls.

Blood-Brain Barrier↗
PubMed1979

Determination of IgG subgroups in cerebrospinal fluid of multiple sclerosis patients and others.

IgG subgroups (IgG1, IgG2, IgG3, IgG4) were determined by radioimmunoassay (RIA) in cerebrospinal fluid (CSF) of controls, multiple sclerosis (MS), infectious diseases (ID) and other neurological diseases (OND). The proportion of IgG1 in the total IgG subgroup concentration was significantly higher in the MS group compared to the other groups while the IgG2 proportion was significantly lower; IgG3 and IgG4 did not show any consistent change. The inverse relationship between IgG1 and IgG2 was similar in all diagnostic groups: high concentration of IgG1 was associated with low concentrations of IgG2 and vice versa. Patients with a high relative concentration of IgG1 in their CSF have a seven to eight times higher statistical risk to be suffering from MS than ID or OND. In the MS group only the IgG1 concentration correlated with the total IgG concentration determined by radial immunodiffusion, while in controls, ID and OND each IgG subgroup correlated significantly with the IgG concentration. This demonstrates that in MS a selective increase of IgG1 subgroup is mainly responsible for the increase of total IgG, while all subgroups are involved in OND and ID showing an increased total IgG concentration.

Albumins↗
PubMed1979

Urinary metabolites of clomethiazole. Detection and structural analysis by gas chromatography-mass spectrometry.

As the result of a renewed extensive investigation of clomethiazole (Distraneurin) metabolism five previously unknown metabolites could be isolated from human urine. Their structures were elucidated by mass spectrometry. Whereas previous investigations on the metabolism of clomethiazole had demonstrated changes only of the ethyl group, we now found metabolites attached to the methyl group, too. The newly isolated compounds 5-(1-hydroxy-2-chloroethyl)-4-methylthiazole (9) and 5-(2-hydroxyethyl)-4-thiazole carboxylic acid lactone (5) were found to be more abundant in human urine than 4-methyl-5-thiazole acetic acid previously considered as the main metabolite.

Biotransformation↗
PubMed1978

Interaction of nitrofurantoin with diphenylhydantoin.

Left-sided motor seizures in a patient with an operated brain tumor were controlled with 300 mg/d DPH. The introduction of antimicrobial therapy with nitrofurantoin caused a fall of serum DPH levels and the recurrence of seizures, at the same time serum gammaGT values were increased. These changes were reversible after nitrofurantoin treatment was terminated. Both increased DPH metabolism or impaired absorption could be responsible for this effect. The concomittant increase of gammaGT could be interpreted as indirect evidence of hepatic enzyme induction with increased metabolism of DPH. It is important to note the possibility of such interaction so that better anticonvulsant control can be achieved.

Humans↗
PubMed1978

Determination of immunoglobulin content of CSF based on light chain characteristics.

The immunoglobulin light chain (types kappa and lambda) content of normal cerebrospinal fluid is similar to that of normal serum. In inflammatory diseases of the central nervous system a shift in the k/l ratio in comparison to serum values, usually a relative increase of Igk, can be observed. This increase of the k/l ratio, though not specific for any disease, is most commonly found in multiple sclerosis. There is no correlation between the IgG content and the k/l ratio. The methods described here measure bound and free light chains simultaneously. The calculations show that free light chains are present in both normal and inflammatory CSF and that they appear to be of polyclonal origin. The detection of light chain abnormalities in CSF can be taken as an indicator of endogenous immunoglobulin production in the CNS and is of significance for the diagnosis of inflammatory CNS processes, especially when other signs of endogenous immunoglobulin production are absent.

Central Nervous System Diseases↗
PubMed1978

Determination of k/l immunoglobulin light chain ratios in CSF from patients with multiple sclerosis and other neurological diseases.

Using antisera against Bence-Jones protein, the concentration of light chains type k and l can be determined in CSF. The calculation of the ratio of type k to type I light chains in CSF represents a sensitive measure for the evaluation of immunological processes involving the CNS. Our results demonstrate that an increase k/l ratio is encountered in 48% of CSF specimen from multiple sclerosis (MS) patients, but also in 50% from patients with other inflammatory diseases involving the CNS, in contrast to only 18% from other neurological diseases. In none of the MS or inflammatory cases is the altered k/l ratio the only indicator of a CNS inflammation, most commonly it is accompanied by an overproportional CSF-IgG elevation (increased QG ratio), an increased cell count or both. For these reasons determination of CSF k/l ratios is helpful in the differentiation of MS and other neurological diseases, but not for the differentiation of other inflammatory CNS diseases from MS.

Central Nervous System Diseases↗
PubMed1978

Juvenile Huntington chorea: clinical, ultrastructural, and biochemical studies.

A brain biopsy from a 20-year-old patient whose clinical course was marked by progressive dementia and chorea since age 10 years showed increased amounts of lipofuscin, abnormal mitochondria, and other organelles in cortical neurons, neurites, and astrocytes. Juvenile Huntington chorea was confirmed at autopsy. High levels of three histone-like proteins (molecular weight 10,000 to 16,000) in the microsomal fraction of purified neurons were found by SDS-polyacrylamide gel electrophoresis. Fatty acids were abnormal in white matter sphingomyelin. These ultrastructural and biochemical findings conformed to those established in adult Huntington chorea, thus strengthening the concept of a uniform pathologic process in adult and juvenile Huntington diseases in spite of some clinical and histologic differences.

Adolescent↗
PubMed1978

[Clinical, preclinical and prenatal diagnosis of congenital sphingolipidoses by determining lysosomal hydrolases (author's transl)].

Sphingolipidoses in infancy and adulthood and associated metabolic disturbances are caused by a recessively inherited, circumscribed lysosomal enzyme deficiency in the catabolism of various structural tissue substances. After presenting detailed methods for the quantitative assay of activities of lysosomal hydrolytic enzymes in leukocytes, serum , fibroblasts, urine and organ tissue with the aid of synthetic chromogenic and fluorescent substrates the signigicance of these methods for clinical diagnosis, for the detection of homozygote persons before developing clinical symptoms (preclinical diagnosis), for the preventive prenatal diagnosis and forthe detection of heterozygote carriers is described for the following diseases: Deficiency of hexosaminidase A and B, deficiency of beta-glucosidase, deficiency or arylsulfatase A, deficiency of alpha-galactosidase, deficiency of alpha-glucosidase.

Clinical Enzyme Tests↗
PubMed1977

The fatty acid composition of sphingomyelin from adult human cerebral white matter and changes in childhood, senium and unspecific brain damage.

A micromethod for the investigation of the fatty acid composition of sphingomyelin in presented. In the cerebral white matter of 17 normal adult brains, analyzed for reference, the predominant fatty acids are C 18:0 and C 24:1. Our results are in agreement with those of other authors. Short chained fatty acids are relatively increased in young children; this shift is typical of "immature" myelin. Similar changes are described here in old persons and cases of non-specific brain damage associated with demyelination (autolysis, chronic uremia, juvenile chorea). Sphingomyelin fatty acid composition can be considered a sensitive measure of both disturbed myelination and demyelination.

Adolescent↗
PubMed1977

Serum concentrations of clozapine determined by nitrogen selective gas chromatography.

A simple gas-liquid-chromatographic method employing a nitrogen selective detector for the quantitative determination of clozapine in serum is presented. The method involves after the addition of dibenzepine as internal standard the extraction into diethylether followed by analysis of the extract dissolved in methanol. Detector linearity was established over the range of 100-1000 ng/ml serum. Clozapine levels of 9 manic patients analysed by this method are presented and discussed. A linear relationship between daily intake (mg/kg body weight) and serum levels (ng/ml) was established.

Bipolar Disorder↗