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Biomedical subjects

J Lössner

Publications and source records attributed to J Lössner.

At least 55 records · Page 3Linked to original sources
PubMed1979

[Serious side effects with D-penicillamine therapy for Wilson's disease].

Immunocomplex nephritis as one of the serious side-effects is dealt with on the basis of 41 patients with Wilson's Disease who have been treated for many years and were stabilised on D-penicillinamine. In one quarter of the patients, proteinuria was found 1 to 5 years after the beginning of the therapy. Until now, an immunocomplex nephritis with diffuse granular, mainly epimembranous IgG and C3 deposits on the glomerular basement membran was found in four patients. No circulating antibodies against cell nuclei were found. The finding of immunocomplex nephritis calls for the discontinuation of the therapy for an at present unknown period of time.

Basement Membrane↗
PubMed1979

[Some problems of Wilson's disease].

The Leipzig Center for Wilson's Disease in the GDR is charged with the registration and diagnosis of all homozygous Wilson gene carriers, the clarification of all suspected cases, including the heterozygote test, and the co-ordination of long-term treatment. At present, there are 78 recorded Wilson gene carriers living. On the basis of our own comprehensive observations and investigations over prolonged periods of time, questions concerning pathogenesis, genetics, diagnosis and therapeutic measures, including their side-effects, are dealt with.

Adolescent↗
PubMed1979

[Diphenylhydantoin stimulation test in Wilson's disease].

Coeruloplasmin and diphenylhydantoin levels were studied both in patients who were stabilised for the first time on diphenylhyantoin (300 mg daily) up to reaching a stable serum concentration (N = 6) and during steady-state concentration (N = 25). No significant DPH-dependent influence on the coeruloplasmin level in the serum was found. In our opinion, DPH is no suitable for the stimulation of coeruloplasmin and thus for the diagnosis of Wilson's disease, particularly in the preclinical stage, because absence of coeruloplasmin increase in the serum being found also in healthy persons.

Adult↗
PubMed1979

[The epidemiology of Wilson's disease in the German Democratic Republic and current problems from the viewpoint of population genetics].

Between 1949 and Nov. 30, 1977 a total of 123 cases of Wilson's disease have been registered in the GDR. Using the available statistics the incidence rate of the disease is assumed to be about 2.9/100,000, the gene frequency approx. 0.53 per cent and the heterozygote incidence 1.05 per cent, supposing that the Hardy-Weinberg law applies to a large extent. Conclusions are drawn from the available material with a view to detection of the disease infuture and to genetic family counselling.

Genetics, Population↗
PubMed1977

[EEG studies of spinocerebellar ataxia].

Electroencephalographic examinations of eighteen patients with spinocerebellar ataxias and of thirteen normal sibs of four of the subjects are reported. The authors frequently observed low levels of potential and a tendency toward slackening in basic activity, which is in agreement with data presented in the literature; theta-delta inclusions in the temporal regions were found especially in cases of cerebellar ataxia. Examinations of the sib frequently showed low-potential curves. However, the use of electroencephalography for the purpose of identifying, in the case of hereditary ataxia, latently diseased sibs for heterozygous individuals does not seem possible.

Adolescent↗
PubMed1975

The kinetics of copper uptake by the liver in Wilson's disease studied by a whole-body counter and a double labelling technique.

The hepatic uptake of 64Cu in the body was studied by whole-body counting in normal subjects, homozygotes, and heterozygotes of Wilson's disease. Special attention was paid to the copper kinetics during the first hour after injection of 64Cu. Two different measuring techniques were used simultaneously: one collimated NaI(Tl) crystal was in a fixed position over the liver, the counts being recorded by a single channel analyser connected to a multi-channel analyser in a multi-channel scaling mode; and a second collimated crystal makes scanning movements along the body axis, the counts being recorded by a multi-channel analyser in a multi-spectrum scaling mode. With this procedure it is possible to use a double nuclide labelling technique by means of which both the 64Cu values for an organ can be corrected for the 64Cu blood content in the region of interest, and information can be obtained on the actual dynamic 64Cu movement in the body.

Adolescent↗
PubMed1975

[Pathological visual field and brain tumor].

The general relationships between an operatively or autoptically localized tumor and the visual field determined by means of ikineticer perimetry are investigated, the patient stock being a total of 1756 neurosurgical brain-tumor inpatients. Examination of the visual field was possible with four fifths of the brain-tumor patients. Hemianopic, quadrantanopsic, or scotomatous defects of a topically localizable nature could be found in almost one third of the total patient stock available for examination. The clinically particularly interesting criteria of localization of homonymous-hemianopsia defects such as macular sparing and incongruence/congruence of visual field changes should be of no importance to the localization within the visual pathway of brain-tumor patients.

Blindness↗
PubMed1975

[Changes of the visual field with cerebral tumors].

Neurosurgical material of 1,746 tumours is reviewed for the relations between operative and post mortem localization of cerebral tumours and pathological findings of isopteric perimetry; and the incidence of defects of the visual field with the different localizations of tumours is discussed. The aim was to localize the tumours as accurately as possible. At least in brain tumour patients the defect of the visual field does not allow conclusions on the level of lesions to the visual pathway. Only the superior quadranopsia is important for the temporal localization.

Blindness↗
PubMed1975

[Changes of visual fields in intracranial tumors outside the cerebral hemispheres].

Based on neurosurgical material of 1,746 brain tumours, the changes of the visual field determined by isopteric perimetry are discussed with their significe with regard to tumours of corpus callosum, lateral ventricles, brain stem ganglia, IIIrd ventricle, region of the sella and adjacent areas as well as posterior fossa. The proportion of unilateral blindness with primary opticus atrophy and of skotomas is strinkingly high, so that in cases of loss of vision, not readily explicable by ophthalmology, as well as in unilateral defects of the visual field, intracranial processes must be thought of.

Blindness↗
PubMed1975

[Results of electroencephalographic and familial studies in Wilson's disease].

The results of electroencephalographic and familial studies of 31 Wilson's disease patients coming from 26 kindreds are presented. Prior to treatment, 8 patients showed pathological conditions, while 9 and 11 patients showed borderline and normal conditions, respectively, it having not been possible to determine electroencephalographic conditions characteristic of Wilson's disease and find any relationships between the intensity of E.E.G. changes and the clinical picture and stage of development of the disease, respectively. Under penicillamine treatment, there was observed a tendency toward normalization of pathological conditions rather than deterioration of normal E.E.G. findings. So far as familial studies are concerned, the authors report the results obtained for 9 fathers, 19 mothers, and 24 siblings from a total of 21 kindreds; what is remarkable in this connection is the comparatively small percentage of normal E.E.G. findings for healthy siblings. It was only in one single kindred that it was possible to observe similarities between E.E.G. variations of individual family members.

Adolescent↗
PubMed1975

[Experiences with penicillamine in Wilson's disease].

Reports are being made on the principles of treatment of morbus Wilson with the help of seventeen patients and two persons in the pre-clinical stage (asymptomatic). Treatment should be begun as soon as possible in the asymptomatic stage. On the whole the pseudo-sclerosis symptoms react more favourably to treatment than many hypokinetic symptoms. A continuous course of treatment with penicillamine still effects a disappearance of the symptoms even after three to four years. Since evidence of a negative copper balance is widely used in determining the amount of the penicillamine dosage, the measurement of basal copper secretion and copper secretion with the use of penicillamine is discussed as a useful criterion.

Adolescent↗