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Biomedical subjects

R Korinthenberg

Publications and source records attributed to R Korinthenberg.

At least 73 records · Page 4Linked to original sources
PubMed1994

Renal tubular dysfunction following treatment with anti-epileptic drugs.

UNLABELLED: To evaluate renal side-effects of anti-epileptic medication in children, we performed a cross-sectional study of various aspects of renal function. We studied 59 patients from our outpatient clinic. They had been on anti-epileptic monotherapy for at least 3 months. None had a history of renal disease. Twenty-three healthy children of the same age group served as controls. After collecting 24-h urine samples, glomerular function was derived from creatinine clearance and from the excretion of albumin. Proximal tubular function was investigated by the urinary excretion of alpha 1-microglobulin and of the tubular enzymes N-acetyl-beta-D-glucosaminidase, alanine-amino-peptidase and fructose-1,6-di-phosphatase. Distal tubular function was examined by the 24-h excretion of Tamm-Horsfall protein. On treatment with carbamazepine (n = 27) and phenytoin (n = 8), the excretion of alpha 1-microglobulin was significantly increased, as compared with the healthy controls. On valproate (n = 20), ethosuximide (n = 9) and phenytoin (n = 8), therapies significantly increased excretion of N-acetyl-beta-D-glucosaminidase. This must be interpreted as an indication of a functional disturbance of the proximal tubulus. The other parameters, indicating function of the glomerulus, loop of Henle and distal tubules did not differ from normal. CONCLUSION: Patients on anti-epileptic treatment with therapeutic drug levels may demonstrate minor signs of tubular dysfunction. These are probably insignificant from a clinical standpoint, but they should be considered in drug overdose.

Adolescent↗
PubMed1994

The metabolization of carbamazepine to CBZ-10,11-epoxide in children from the newborn age to adolescence.

CBZ-10,11-epoxide is a major metabolite of CBZ. It has anticonvulsive properties and may be responsible for side-effects of CBZ treatment. Fifty-two children between the age of 2 weeks and 15 years were treated with CBZ (mean dosage 17 mg/kg body weight) either as mono- (n = 36) or in polytherapy (n = 16). The drug was delivered as an oral solution, as a nonretarded tablet or, most frequently, as a retarded tablet. The duration of treatment ranged from 1 to 94 months with 23 patients being on treatment for less than 3 months. Blood samples were taken with random timing after the last ingestion of the drug. The relative concentration of CBZ epoxide (expressed in % of CBZ) was higher in infants (median 48.9%) than in older children (median 14.9% in the 12-15-year-old group). A significant linear correlation with age was found (p < 0.001). In addition to young age, polytherapy (p < 0.01) and administration as a nonretarded formulation rather than as a retarded tablet (p < 0.05) induced a higher relative concentration of the epoxide. The relative concentration of the epoxide did not correlate with the serum CBZ concentration and the duration of treatment. Although in our study high epoxide levels were not related to clinical side effects, we recommend that in very young children polytherapy treatment with carbamazepine should be performed with caution and in difficult cases a determination of the epoxide level should be considered.

Adolescent↗
PubMed1993

Neurological findings in newborn infants after extracorporeal membrane oxygenation, with special reference to the EEG.

Seventeen newborn infants were treated with extracorporeal membrane oxygenation (ECMO). Two died shortly after the start of ECMO due to the underlying disease, two died later in the course due to a lack of recovery of lung function and two others died weeks after ECMO from renal damage and a cardiac defect. Of the survivors, nine are developing normally (aged between one and four years) and two are severely disabled because of infarctions of the left hemisphere, acquired before and after ECMO. Intermittent-discontinuous EEGs did not indicate a poor prognosis if normalization of the EEG occurred within seven days. However, infarcted areas on ultrasonography, persistent EEG changes or deteriorating findings indicated disability or early death.

Brain↗
PubMed1992

Simultaneous measurement, using flow cytometry, of radiosensitivity and defective mitogen response in ataxia telangiectasia and related syndromes.

In a retrospective study, peripheral blood mononuclear cells from 13 patients with known ataxia telangiectasia (AT) (Louis Bar syndrome, McKusick #20890) were irradiated with different doses of X-rays prior to stimulation with phytohaemagglutinin. Mitogen response and cell cycle progression were assessed by two-parameter 5-bromo-2'-deoxyuridine/Hoechst--ethidium bromide flow cytometry. Compared to age-matched controls, AT cells show a severely defective mitogen response in both unirradiated and irradiated cells. Following irradiation with 1.5 Gy, AT cells exhibit significantly greater accumulations of cells in the G2 phase of the first cell cycle than controls. The ratio between the number of cells accumulated in the first cycle G2 phase and the growth fraction provides a clear distinction between AT and control cultures. In addition, two patients with microcephaly, normal intelligence, immunodeficiency, chromosomal instability and risk for lymphoreticular malignancies (Seemanová syndrome) and two patients with the Nijmegen breakage syndrome (both syndromes are listed as McKusick #25126) also exhibit very poor mitogen response and moderately increased G2 phase accumulations after X-irradiation. The simultaneous assessment of radiosensitivity and mitogen response in a single cell kinetic assay provides a speedy and accurate classification of cells of AT and AT-related syndromes.

Adolescent↗
PubMed1992

[Guillain-Barré syndrome in childhood. Characteristic course and therapeutic possibilities].

The incidence of the GBS in childhood is significantly lower than in adults. The clinical course may be equally severe, 10-25% of the affected children requiring artificial ventilation. Besides respiratory insufficiency, cardial arrhythmias are a major threat to the patients' lives. However, eventually more than 90% of the children recover completely. Controlled studies on the efficacy of therapeutic measures have been performed only in adults. To children their results should be transferred with caution. Steroids are of no value in the acute disease. However, they should be tried when progression for more than 4 weeks suggests the diagnosis of CIDP. Plasmapheresis is beneficial in CIDP and severe GBS; in GBS the time for weaning from the respirator and for achieving independent ambulation is significantly reduced. Recent studies have demonstrated that intravenous immunoglobulins can be equally effective. However, the administration of immunoglobulins in less severe cases should at present be reserved to a controlled study.

Adolescent↗
PubMed1992

[Grand mal epilepsy in childhood].

Generalized tonic-clonic seizures are the most common type of convulsive disorders in children. They are always a sign of an epileptogenic cerebral dysfunction and present either acutely, predominantly as a single event with detectable origin, or chronically, recurring as an epileptic syndrome. In view of the etiology and classification of convulsions it is important to differentiate between primarily and secondarily generalized seizures. This distinction is first of all based on an exact description of the very beginning and of the course of the seizures, on the EEG findings and on any connection between the seizures and a particular time of day. Primarily generalized tonic-clonic seizures with and without associated petit mal seizures are manifestations of an idiopathic epilepsy and are most probably genetically determined, secondarily generalized seizures on the other hand are often signs of a central nervous lesion or of another symptomatic form of epilepsy. Benign idiopathic partial seizures, however, take the from of secondarily generalized convulsions during the morning sleep. Prolonged tonic-clonic seizures of any origin require vigorous treatment with anticonvulsants, if necessary in an intensive care unit. Recurrent seizures are treated with long-term anticonvulsant medication. The first-line treatment is valproic acid or phenobarbitone (or if necessary, a bromide) in primarily generalized seizures and carbamazepine or phenytoin in secondarily generalized convulsions. The recommended duration of this therapy and the risk of recurrence of seizures vary widely with the underlying etiology and the type of epilepsy.

Anticonvulsants↗
PubMed1991

[Risk of recurrent seizures after the first afebrile grand mal seizure in childhood].

A cohort of 74 children three months to 16 years-old who presented with a first unprovoked seizure were followed for five years to assess the risk of recurrence. Children with febrile convulsions, immediate posttraumatic seizures, meningitis and encephalitis were not included. The risk of recurrence was 68% for a second seizure. 47% of the patients developed an epilepsy. 85% of recurrences occurred within the first 6 months and 100% within 2 1/2 years. A history of epilepsy in a first degree relative, age at first seizure, duration of seizure, initial EEG or neurologic status were not associated with significantly higher risk of recurrence.

Adolescent↗
PubMed1990

Prospective neurophysiological study in children treated for acute lymphoblastic leukaemia: serial EEG during treatment and long-term follow up with evoked potentials.

In 79 children treated for acute lymphoblastic leukaemia according to protocol ALL-BFM 81, serial EEG examinations were performed before, during and after therapy. Diffuse changes of the background activity were observed in 64% of the children at the time of diagnosis. During induction and reinduction treatment with vincristine and L-asparaginase, and with some delay after CNS irradiation, a marked slowing developed in up to 65% of patients. Children who had not been irradiated showed transient disturbances during treatment with medium-dose-methotrexate. Reinduction induced more abnormal EEGs in the children who had been irradiated. At the end of maintenance therapy, only slight EEG changes were found. No differences between the irradiated and non-irradiated group were then seen. Children with CNS leukaemia or seizures differed from those with an uncomplicated treatment in that they more often showed focal and persistent disturbances. In 39 patients who stayed in first remission for at least 18 months after the termination of treatment, a follow up investigation was performed. From the EEG examination, including power spectral analysis, no differences were found between irradiated and non-irradiated patients. Slowing of the dominant frequency was seen in the patients with more severe leukaemia and in those whose EEG had been markedly abnormal at diagnosis. The visually evoked potentials were normal in all groups of patients. In the brainstem auditory evoked potential, a prolongation of the latency of wave I and a decrease of the I-V interval was found in irradiated patients. We conclude that the diffuse EEG changes frequently emerging during treatment are reversible. Persistent or lateralized changes can indicate a neurological complication.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1990

Electrolytes, amino acids and proteins in lumbar CSF during the treatment of acute leukemia in childhood.

We performed analyses of electrolytes, amino acids, albumin, alpha 2-macroglobulin, gamma-globulin and LDH in the lumbar cerebrospinal fluid of children undergoing treatment for acute lymphoblastic leukemia, non-Hodgkin-lymphoma or acute myeloid leukemia. At the time of diagnosis signs of a disturbance of the blood-brain barrier were found in some patients. During induction treatment with L-asparaginase a rise of glutamic acid and a decrease of glutamine occurred. This finding correlated with slowing of the EEG. Treatment with vincristine was associated with a slight drop of sodium and chloride concentration in serum, but not in the cerebrospinal fluid. Central nervous system prophylaxis with cranial irradiation, and to a lesser degree with intravenous medium-dose methotrexate, gave rise to a further deterioration of the blood-brain barrier function as indicated by an increase in albumin, alpha 2-macroglobulin and LDH levels. During radiotherapy the concentration of several amino acids rose, probably due to a disturbance of active carrier mechanisms. Patients with elevated albumin at the end of radiotherapy more often suffered an early leukemia relapse while still on treatment. No other clinical or electroencephalographic correlations of altered barrier function could be found.

Amino Acids↗
PubMed1990

[Pyridoxine-dependent seizures. Remarks on clinical variability and pathogenesis].

The clinical variability of pyridoxine dependent seizures is greater than mentioned in most textbooks. The seizures can start during the first days of life, or later during the first year. Frequently they are therapy-resistant, but seizure free periods of several weeks duration can be achieved with anticonvulsants in some patients. The response to the injection of pyridoxine can be delayed. A trial with daily oral administration of pyridoxine is often more suitable than a single injection.

Anticonvulsants↗
PubMed1989

[Critical intracranial pressure crises in drained hydrocephalus with minimally enlarged or normal ventricles].

We report on 10 patients between 3 and 18 years of age who had been treated in the neonatal and postneonatal period for hydrocephalus of various causes with a ventriculo-atrial or ventriculo-peritoneal shunt. Between the age of 2 and 17 years these children suffered from recurrent attacks of headache, vomiting bradycardia and lethargy suggesting shunt-obstruction. 8 children showed slowing of electrical activity in their EEG, 4 had papilledema on fundoscopy. Splayed sutures were found in one child by skull x-ray. In all the above cases the CT-headscans assessed by radiologists showed no evidence of shunt-obstruction. Only on direct comparison with previous CT-scans we were able to detect minimally dilated ventricles in 6 children. In all cases conservative treatment failed. Symptoms only ceased after surgical shunt-revision. In all children treated with shunts headache, vomiting and vegetative symptoms must be considered as signs of shunt-obstruction until proved otherwise. A negative CT-scan done while symptoms are present cannot rule out raised intracranial pressure. Only by direct comparison with previous CT-scans carried out during an asymptomatic period a minor dilatation of the ventricles can often be detected. This should be taken as a sign of shunt-obstruction.

Adolescent↗
PubMed1988

Basal ganglion calcification in hyperphenylalaninemia due to deficiency of dihydropteridine reductase.

The disease course and therapy of a nine-and-a-half-year-old boy with hyperphenylalaninemia due to a dihydropteridine reductase deficiency are reported. Clinically, there is a marked mental retardation and complex basal ganglion symptoms. The cranial computed tomographic investigation shows bilateral, symmetrical, comma-shaped calcifications in the globus pallidus and the putamen of the lentiform nucleus. The cause of these basal ganglion calcifications remains unclear. Lowering of serum and CSF folic acid levels could not be detected, in contrast to cases with the same enzyme defect described previously.

Basal Ganglia Diseases↗