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Biomedical subjects

F Hanefeld

Publications and source records attributed to F Hanefeld.

At least 163 records · Page 9Linked to original sources
PubMed1983

Succinic semialdehyde dehydrogenase deficiency: an inborn error of gamma-aminobutyric acid metabolism.

Gamma-hydroxybutyric aciduria is a disorder of gamma-aminobutyric acid metabolism in which a compound of known neuropharmacologic activity accumulates. We have studied two patients in whom high levels of gamma-hydroxybutyric acid were found in blood, urine and cerebrospinal fluid. A coupled assay has been developed which estimates succinic semialdehyde dehydrogenase activity in isolated human lymphocytes. The mean activity of succinic semialdehyde dehydrogenase in a control and the four parents and two healthy siblings of these patients was 8.8 +/- 1.9 pmol . min-1 . mg-1 protein. In the patients the activities were 0.8 and 1.1 pmol . min-1 . mg-1 protein, approximately 9-13% of control. In the presence of saturating amounts of NAD+, lymphocyte sonicates, derived from the patients accumulated a significant amount of 14C-succinic semialdehyde from 14C-gamma aminobutyric acid, whereas none could be detected in controls. The data suggest a deficiency of succinic semialdehyde dehydrogenase in these patients, the first documented defect of the metabolism of gamma-aminobutyric acid in man.

4-Aminobutyrate Transaminase↗
PubMed1983

Adducted thumb syndrome. Report of a new case and a diagnostic approach.

We report on a female newborn baby with a "myopathic" stiff face, open mouth, high-arched palate, microgenia, generalized muscular hypotonia, limited extension of elbows, wrists and knees, flexed adducted thumbs, velopharyngeal insufficiency, and hypertrichosis. Death occurred at 3 months due to respiratory insufficiency. Muscular biopsy revealed myopathic abnormalities.

Abnormalities, Multiple↗
PubMed1983

Cranial computed tomography of 64 children in continuous complete remission of leukemia I: relations to therapy in modalities.

64 children with acute lymphoblastic leukemia and non Hodgkin's lymphoma in continuous complete remission were studied by cranial computed tomography two months to 7 years after cessation of antileukemic treatment. 17 (27%) patients showed abnormal widening (grade 2) of external cerebrospinal fluid spaces, 20 (31%) minimal widening (grade 1). A significant correlation between grade 2 abnormalities and the duration of maintenance therapy and single cranial radiation dose was found, whereas the correlations with total radiation dose and with intensity of induction protocols were not significant. Widening of external cerebrospinal fluid spaces after cessation of therapy persisted.

Acute Disease↗
PubMed1983

Cranial computed tomography of 64 children in continuous complete remission of leukemia II: relations to patient data and neurological complications.

Abnormal widening of cortical sulci as seen in posttherapeutic cranial computed tomography (CCT) of 64 children in complete continuous remission (CCR) of acute lymphoblastic leucemia (ALL)/non Hodgkin's lymphoma (NHL) was related to patient data and neurological complications during the application of West Berlin treatment protocol. Age and neurological/neurodevelopmental findings of the patients at diagnosis of their disease positively and significantly correlated with CCT abnormalities. Abnormal pre- and intratherapeutic electroencephalographic (EEG) recordings, the development of a severe polyneuropathy syndrome and/or a considerable loss of weight during treatment were frequently associated with an abnormal widening of cortical sulci. Occurrence of transient early encephalopathy syndrome, development of radiation induced blood brain barrier disturbance and somnolence syndrome were not correlated with these CCT changes. Possible explanations of these abnormalities are discussed.

Acute Disease↗
PubMed1983

Electroencephalogram investigations of the disequilibrium syndrome during bicarbonate and acetate dialysis.

Continuous long-time electroencephalographic (EEG) monitoring was performed during acetate and bicarbonate dialysis in 20 patients. Persisting normal basic activity of the EEG without neurological symptoms was found only during the course of bicarbonate dialysis. However, in acetate dialysis, during the decrease of arterial CO2 tension (PaCO2), we registered EEG disturbances with moderate to severe slowing, dysrhythmic activity and high voltage discharges. The decrease in PaCO2 and the deterioration in EEG activity in the patients during acetate dialysis was concomitant with severe neurological alterations, e.g. the typical symptoms of so-called 'disequilibrium' causing a cessation of dialysis in three patients.

Acetates↗
PubMed1983

[Electro-encephalographic study of the disequilibrium syndrome during bicarbonate dialysis and acetate dialysis].

Continuous long-time electroencephalographic (EEG) monitoring was performed during AHD and BHD in 20 patients. Persisting normal basic activity of the EEG without neurological symptoms could be found only during the course of BHD. However, in AHD, during the decrease of arterial CO2 tension (PaCO2) we registered EEG disturbances with moderate to severe slowing down, dysrythmic activity and high voltage discharges. The decrease in PaCO2 and the deterioration in EEG activity in the patients during AHD was concomitant with severe neurological alterations, e.g. the typical symptoms of so-called "disequilibrium" causing a cessation of dialysis in 3 patients.

Acetates↗
PubMed1983

[Effect of hyperbilirubinaemia on cerebellar development in Gunn rats].

The influence of bilirubin on cerebellar growth was studied in homozygous Gunn rats by measuring weight, DNA concentration and histochemical enzyme reactions at different ages and under different conditions. High bilirubin levels cause persistent reduction in cerebellar size and loss of Purkinje and granular cells. Sulfonamide application results in an almost complete cessation of cerebellar growth. Phototherapy can prevent these neurotoxic effects of bilirubin. The vulnerability of the cerebellum is particularly high during the period of rapid growth.

Animals↗
PubMed1982

Advantages of bicarbonate hemodialysis.

Six patients with frequent episodes of symptomatic hypotension during acetate dialysis were treated with bicarbonate dialysis. In all patients blood pressure, heart rate, and arterial acid-base values were measured every 30 minutes during each of the five treatments with acetate dialysis and bicarbonate dialysis. Hemodynamic parameters were measured invasively in all patients during bicarbonate dialysis and in three of them also during acetate dialysis. Additionally, continuous long-time monitoring with electroencephalography was performed during acetate dialysis and bicarbonate dialysis. During acetate dialysis the patients showed a frequent onset of sudden hypotension and arrhythmia with concomitant symptoms of the so-called disequilibrium syndrome, whereas these symptoms were nonexistent in the same patients during bicarbonate dialysis.

Acetates↗
PubMed1982

[Long-term sequelae of neonatal group B streptococcal septicemia/meningitis (author's transl)].

In recent years group B streptococci accounted for nearly 40% of all cases of neonatal septicemia in our intensive-care-unit. Nineteen of 38 babies did not survive the acute illness (mortality 50%). Six of 18 surviving children showed abnormalities related to the septicemia/meningitis (morbidity 33%). The severity of chronic complications ranged from minor neurological problems to marked retardation, deafness, blindness, and epilepsy. In our series there seemed to be a relation between the severity of complications during the acute illness (meningitis, convulsions), and later neurological sequelae. Early detection and early treatment were found to be most important for the final outcome.

Adolescent↗
PubMed1981

Occurrence of oligoclonal gammaglobulin in the CSF of children with prolonged and chronic CNS-infections.

CSF-proteins of 1770 children and adolescents with different neurological diseases and of 75 controls were examined by zone electrophoresis in agarose gel electrophoresis and by immunofixation electrophoresis. The quantitative evaluation of the phoretograms by an analog computer revealed oligoclonal changes of the gamma-globulin profile in 53 patients with subacute or chronic CNS-infections and in 5 children with a medulloblastoma. Seventeen of 37 children with congenital infections had 1--5 oligoclonal gamma-fractions consisting of IgG. Five to seven oligoclonal IgG fractions were detected in each of 16 children with SSPE. All 6 adolescents with multiple sclerosis had 2--5 oligoclonal IgG fractions. One to five oligoclonal gamma-bands occurred transiently in the CSF of 4 children with prolonged meningoencephalitis caused by different viruses, in 3 children with a prolonged non-bacterial meningitis of probable viral origin, in 2 infants with prolonged bacterial meningitis after corticosteroid therapy, and in 1 child with prolonged bacterial meningitis during cytostatic therapy. Four to six oligoclonal gamma-subfractions were found at different times during progressive viral encephalitis that developed during maintenance therapy of acute lymphoblastic leukaemia, and in 2 patients with chronic meningoencephalitis of unknown origin. Since oligoclonal gamma-globulin was detected almost invariably in the CSF of patients with prolonged or chronic neurologic infections, this finding implies persistence of antigens in the CNS and pathologic invasion of lymphocytes with selective proliferation of antigen-stimulated clones.

Adolescent↗
PubMed1980

Changes of CSF-protein pattern in children with acute lymphoblastic leukemia during prophylactic CNS therapy (Berlin protocol).

The cerebral spinal fluid (CSF)-protein profiles of ten children with previously untreated acute lymphoblastic leukemia (ALL) were investigated by agarose gel electrophoresis. The profiles were determined at diagnosis and during the fifth to eighth week of treatment when preventive therapy for central nervous system (CNS) leukemia (skull irradiation, intrathecal methotrexate (ithMTX) was administered. The profiles were compared with those obtained from a control group of 67 children and those from 42 patients with acute aseptic meningitis. The data from the latter group demonstrated the CSF-protein pattern of partial blood-CSF barrier (B-CSF-B) breakdown. The children with ALL showed no or only minor signs of a B-CSF-B impairment at diagnosis and after four weeks of systemic treatment. However, CSF changes indicative of a lesion of the B-CSF-B increased in all children continuously during CNS prophylaxis. The protein profile at the end of combined chemotherapy and radiotherapy was very similar to that in patients with acute aseptic meningitis. These observations point to neurotoxic side effects on the CNS barrier system with the combination of cranial radiation and ithMTX. A striking finding was restricted heterogeneity of gamma-globulin, observed in the CSF of nine out of the ten children with ALL before or during treatment. The significance of this abnormality is unknown.

Antineoplastic Agents↗
PubMed1980

Therapy of acute lymphoblastic leukaemia in childhood: effects on the nervous system.

A number of neurological disturbances occur during the treatment of childhood malignancies with cytotoxic drugs like vincristine, methotrexate, cytosine-arabinoside, cyclophosphamide, asparaginase and others. Neurological complications range from peripheral neuropathy, myopathy, myelopathy to encephalopathy with methotrexate induced encephalopathy leading to permanent brain damage or death in most cases. Irradiation of the brain can produce transient or permanent brain damage by a direct effect on nervous tissue and by altering the blood brain barrier or the blood circulation. The many conflicting reports concerning the quality of life of long-term survivors of childhood leukaemia do not give sufficient data for final conclusions. The experiences we have encountered over the past ten years seem to confirm the optimistic echo from others. There is, however, an urgent need for a prospective study to answer the all important question: will a child suffering from leukemia emerge physically and mentally unharmed after long and aggressive treatment?

Asparaginase↗
PubMed1979

Effects of riboflavin on Gunn rats under phototherapy.

Blue and white phototherapy was given to infant (and weanling) homozygous Gunn rats treated with different doses of riboflavin-5-phosphate (ribofl.5'p.) During the first hours after a flavin-injection, the effect of phototherapy with both types of fluorescent lamps was enhanced. With equal radiant power applied, the steepness of serum bilirubin decline depended on the ribofl.5'p. dose injected. After oral or cutaneous application, no similar effect occurred. After a single flavin dose, the serum bilirubin decline lasted for at least 3 hr. Nevertheless, in long-term studies with repeated injections (100 mg/kg every 48 hr), no protective effect beyond that of phototherapy alone could be ascertained on the Purkinje cells in the cerebella of the rats. In the skin of the animals, fluorescence was macroscopically noticeable after ribofl.5'p. injections. When an effective irradiance of about 3.0 mW/cm2 and a high flavin dose (100-200 mg/kg) was administered, histologic investigations of the skin in the abdominal and back region revealed a reversible inflammatory reaction with edema and morphologic changes in the epidermal cells that culminated 12-24 hr after the injection. After a further increase of the effective irradiance, tremendous vesicles on paws, ears, and tails were observed in most of the animals 24-72 hr after the flavin injections. The content of the blisters was primarily serous, later on, often hemorrhagic. Finally, necrosis developed. Acute toxicity of ribofl.5'p. differed markedly when the infant rats (homozygous jaundiced as well as heterozygous nonjaundiced) were kept in the dark or under intense blue phototherapy. Much higher doses were tolerated in the dark. Moreover, it could be demonstrated that ribofl.5'p. does not influence serum bilirubin of jaundiced Gunn rats kept in complete darkness. That suggests that the drug itself does not compete for albumin binding sites. But certain riboflavin ampules (Beflavin) contain stabilizers that considerably displace bilirubin from albumin bonds. Riboflavin disturbs direct photometric bilirubin measurements, but not the diazo reaction. When bilirubin is to be measured in sera containing riboflavin, lights must be extremely dim. Photodegradation in vitro is highly accelerated by the sensitizer.

Animals↗