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Biomedical subjects

R Feldmann

Publications and source records attributed to R Feldmann.

At least 19 recordsLinked to original sources
PubMed2007

[Leucocytoclastic eosinophilic vasculitis with Löffler syndrome].

Histologically, leukocytoclastic vasculitis (LV) presents with neutrophilic granulocytes with leukocytoclasia and erythrocyte extravasation, associated with variable counts of lymphocytes, plasma cells and eosinophilic granulocytes. The association of a LV with eosinophilic granulocytes and eosinophilic pneumonia was first described by Chan et al. in 1982. Our case represents the second report in the literature of this rare disease: a 85 year old patient with LV and numerous eosinophilic granulocytes in association with intermittent blood eosinophilia and Löffler syndrome (eosinophilic pulmonary infiltrates). The recurrent episodes were treated successfully with oral corticosteroids.

Aged, 80 and over↗
PubMed2005

[Intelligence, attention, and memory in patients with myasthenia gravis].

BACKGROUND: In patients with myasthenia gravis (MG), poor performance on cognitive tests has been found. Performance on memory tasks has been reported to correlate with disease activity, but memory in MG patients was also recently found to be unimpaired. PATIENTS AND METHODS: Cognitive functioning was examined in 23 patients with MG and 23 healthy controls. The patients were assessed for IQ, memory, attention, and motor performance. Immunoglobulin G antiacetylcholine receptor autoantibody titers were determined. Event-related potentials were generated for patients and controls. RESULTS: Mean IQs of patients were at average. Memory and attention were not more impaired in patients than controls. Event-related potentials were not delayed in either group. Patients with elevated autoantibody concentrations similarly showed unimpaired neurocognitive and motor functioning. CONCLUSION: In this study, no evidence of neuropsychological impairment was found in MG, arguing against the involvement of higher cortical functions. Sleep abnormalities rather than central mechanisms may explain the memory impairments reported by some studies.

Adolescent↗
PubMed2005

Frontal lobe-dependent functions in treated phenylketonuria: blood phenylalanine concentrations and long-term deficits in adolescents and young adults.

Even early-treated phenylketonurics may suffer from phenylalanine-related deficits. Elevated phenylalanine concentrations can interfere with the development and function of the CNS. Outcome beyond childhood has not been extensively investigated. This long-term study was performed to determine whether adolescents and young adults with PKU show frontal lobe-dependent deficits when compared to diabetic patients. The comparative study covered 35 PKU patients, 13-21 years of age (mean 17.8 years), and 35 diabetic patients matched for sex, age and socioeconomic status. Patients were assessed for IQ (Culture Fair Intelligence Test), information processing (Trail Making Test), and selective and sustained attention (Stroop Task, Test d-2). Assessments were repeated within a 3-year follow-up. PKU patients showed no increase in blood phenylalanine concentrations at follow-up. They had significantly poorer test results than the diabetic patients at both assessment times. Within the tests, however, this was due to reduced performance speed but not to deficits in specific frontal lobe-dependent functions. Elevated phenylalanine concentrations seem to exert a global effect slowing performance speed. This effect is enduring in adolescence and early adulthood.

Adolescent↗
PubMed2003

Neurofibromatosis type 1: motor and cognitive function and T2-weighted MRI hyperintensities.

BACKGROUND: Cognitive impairment and fine motor deficits are frequent manifestations in patients with neurofibromatosis type 1 (NF1). More than 50% of patients with NF1 show focal areas of high signal intensity (T2H) on T2-weighted MRI of the brain. It has been hypothesized that T2H may be associated with the cognitive and motor problems. METHODS: The authors investigated 100 patients with NF1 and 100 healthy control subjects matched for age, sex, and socioeconomic status for their IQ (Wechsler Intelligence Scale for Children-Revised [WISC-R] and Wechsler Adult Intelligence Scale-Revised [WAIS-R]), fine motor abilities (Motorische Leistungs-Serie [motor performance task]), and T2H (MRI). RESULTS: As a group, the 100 patients performed within normal limits of WISC-R and WAIS-R scores. However, the scores for the NF1 patients with normal MRI were at the mean, whereas those for the patients who had T2H (n = 58) were significantly depressed. On measures of fine motor skills, patients with T2H performed poorer than patients with normal MRI. CONCLUSION: Hyperintensities on T2-weighted MRI represent a biological marker for impaired cognitive and fine motor performance in patients with NF1.

Adolescent↗
PubMed2002

Individual blood-brain barrier phenylalanine transport in siblings with classical phenylketonuria.

Recent studies indicate that individual blood-brain transport characteristics of phenylalanine may lead to different clinical outcomes in phenylketonuria (PKU) patients in spite of comparable dietary control. To check these preliminary data, we investigated four pairs of siblings with classical PKU (and identical genotype) using in vivo nuclear magnetic resonance spectroscopy in the course of an oral phenylalanine load (100 mg/kg body weight). Patients' brain phenylalanine concentrations were different in spite of similar blood levels. Interindividual variations of the apparent transport Michaelis constant, Kt,app, ranged from 0.10 to 0.84 mmol/L. The ratio of the maximal transport velocity, Tmax, over the intracerebral consumption rate, Vmet, varied between 2.61 and 14.0. Siblings with lower values for Kt,app, higher values for Tmax/Vmet, and higher concurrent brain phenylalanine levels showed a lower IQ and a higher degree of cerebral white matter abnormalities. The results indicate that blood-brain barrier transport characteristics and the resultant brain phenylalanine levels are causative factors for the individual clinical outcome in PKU.

Adolescent↗
PubMed2001

Individual blood-brain barrier phenylalanine transport determines clinical outcome in phenylketonuria.

Different clinical outcomes in spite of comparable dietary controls are well known in patients with phenylketonuria. Currently, reasons for this phenomenon are unknown. Kinetic investigations in 15 patients with classic phenylketonuria were performed using in vivo nuclear magnetic resonance spectroscopy before and after an oral phenylalanine load (100 mg/kg body weight). Patients' brain phenylalanine concentrations were quite different in spite of similar blood phenylalanine levels. Interindividual variations of the apparent transport Michaelis constant, K(t,app), covered a range from 0.10 to 1.03 mmol/L. The ratio of the maximal transport velocity, Tmax, over the intracerebral consumption rate, Vmet, varied between 2.61 and 14.0. Both parameters as well as the preload brain phenylalanine levels correlated significantly with the degree of cerebral white matter abnormalities on magnetic resonance images. Correlations of K(t,app), Tmax/Vmet, and the preload brain phenylalanine levels with patients' intelligence scores approached significance. In conclusion, blood-brain barrier phenylalanine transport characteristics and the resultant brain phenylalanine levels seem to be causative factors for the individual clinical outcome in phenylketonuria. This observation may lead to individual dietary recommendations in the future.

Adolescent↗
PubMed2001

Normal clinical outcome in untreated subjects with mild hyperphenylalaninemia.

ABSTRACT There is international consensus that patients with phenylalanine (Phe) levels <360 microM on a free diet do not need Phe-lowering dietary treatment whereas patients with levels >600 microM do. Clinical outcome of patients showing Phe levels between 360 and 600 microM in serum on a free nutrition has so far only been assessed in a small number of cases. Therefore, different recommendations exist for patients with mild hyperphenylalaninemia. We investigated in a nationwide study 31 adolescent and adult patients who persistently displayed serum Phe levels between 360 and 600 microM on a normal nutrition with a corresponding genotype. Because of limited accuracy of measurements, Phe levels should be looked on as an approximation, but not as an absolute limit in every instance. In addition to serum Phe levels, the assessment program consisted of comprehensive psychological testing, magnetic resonance imaging of the head, (1)H magnetic resonance spectroscopy, and genotyping. We found a normal intellectual (intelligence quotient, 103 +/- 15; range, 79-138) and educational (school performance and job career) outcome in these subjects as compared with healthy control subjects (intelligence quotient, 104 +/- 11; range, 80-135). Magnetic resonance imaging revealed no changes of cerebral white matter in any patient, and (1)H magnetic resonance spectroscopy revealed brain Phe levels below the limit of detection (<200 microM). In the absence of any demonstrable effect, dietary treatment is unlikely to be of value in patients with mild hyperphenylalaninemia and serum Phe levels <600 microM on a free nutrition, and should no longer be recommended. Because of a possible late-onset phenylketonuria, Phe levels of untreated patients should be monitored carefully at least during the first year of life. Nevertheless, problems of maternal phenylketonuria should still be taken into account.

Adolescent↗
PubMed2000

[Lichen striatus or Blaschkitis in the adults. Variations of the same entity?].

A 64-year-old woman presented with extensive erythematous papulosquamous lesions along the lines of Blaschko on the right half of the trunk and the right leg. The clinical and histological features led to the diagnosis of lichen striatus, a dermatosis which usually appears during childhood. Treatment with acitretin led to marked improvement. Lichen striatus and its differential diagnoses--particularly "blaschkitis of adulthood" as first described in 1990--are discussed.

Biopsy↗
PubMed2000

Behavioural and emotional problems in early-treated adolescents with phenylketonuria in comparison with diabetic patients and healthy controls.

Even early-treated patients with phenylketonuria (PKU) have a higher risk of psychosocial maladjustment. This study was performed to determine whether emotional and behavioural problems are specific in phenylketonurics and whether they depend on the quality of biochemical control. This comparative study covered 42 PKU patients aged 10-18 years (mean 14.7 years) and 42 diabetic patients matched for sex, age and socioeconomic status. Patients' groups were compared with a control sample of healthy controls (n = 2900) from an epidemiological study. We used the Child Behavior Check List (CBCL) according to Achenbach, intelligence quotient (IQ) test according to Weiss, and monitoring of blood phenylalanine concentrations and HBA1 concentrations. Internalizing problems such as depressive mood, anxiety, physical complaints or social isolation were significantly elevated in both phenylketonuric and diabetic patients, whereas externalizing problems were not. The two patient groups did not differ significantly either in the degree or in the pattern of their psychological profile. In both groups no significant correlations could be computed between the psychological characteristics and the biochemical control, the IQ, and the socioeconomic status. No patient was undergoing psychiatric treatment or psychotherapy. Our results strongly support a psychological perspective for the development of behavioural and emotional problems in both phenylketonuric and diabetic patients. Thus, medical treatment should be accompanied by psychological support for the families.

Adolescent↗
PubMed2000

Cutaneous pseudolymphoma, lymphomatoid contact dermatitis type, as an unusual cause of symmetrical upper eyelid nodules.

We describe a 74-year-old woman who rapidly developed bilateral voluminous nodules on the upper eyelids, following 4 months' treatment for chalazion. Histological and immunohistochemical findings were suggestive of cutaneous pseudolymphoma (CPL), and extensive screening for malignant lymphoma (ML) remained negative. Cutaneous pseudolymphomas are inflammatory diseases that can simulate ML either clinically, histopathologically, or both. They are a rare cause of nodules of the upper eyelids, usually characterized by a benign evolution, that can be secondary to ultraviolet sensitivity, adverse reactions to systemic medications and contact sensitization, among others. In our case, epicutaneous patch tests were strongly positive for a series of allergens contained in the ophthalmological preparations used; therefore, they were suggestive that the patient had CPL, lymphomatoid contact dermatitis type. In addition, the patient had been treated previously with several drugs, known to cause CPL and immune dysregulation. Complete regression of the lesions required treatment with systemic steroids and chlorambucil. No relapse occurred within a 5-year follow-up period.

Aged↗
PubMed2000

[Prognostic and therapeutic relevance of cognitive characteristics for the long-term course of schizophrenic illness following psychoeducational psychotherapeutic treatment].

In a prospective, randomized clinical trial cognitive characteristics of schizophrenic patients were examined as predictors of the efficacy of a psychoeducational psychotherapeutic intervention. The aim of this study was to select adequate cognitive predictors. The reduction of the selected cognitive deficits by means of a psychoeducational psychotherapy was measured. Additionally, the prophylactic effects of the improvement of cognitive deficits were examined. Predictors of the course of illness were basic cognitive deficits and metacognitive constructs of 106 schizophrenic outpatients. Additionally, the modification of the cognitive skills of these patients was taken into account. Relevant factors of the course of illness representing the therapeutic effect of the intervention were investigated within a five-year follow-up. By means of logistic regression analyses thought disorders (AMDP system) and idiosyncratic and fatalistic assumptions (KK-scale) were obtained as appropriate cognitive predictors of the long-term course of illness. Thought disorders and attentional deficits could not be improved significantly. Though, there was a correlation between the therapeutic improvement of idiosyncratic and fatalistic assumptions and the rehospitalization rate within the follow-up.

Adult↗
PubMed2000

Primary mucinous carcinoma of the scalp.

Primary cutaneous mucinous carcinoma (MC) is a rare epithelial neoplasm derived from the sweat glands. Herein, we report a case of MC located on the head. A 66-year-old woman underwent excision of a nodular tumor with a reddish brown surface on the left parietal region. Histopathology revealed a neoplasm extending from the reticular dermis into the subcutaneous fat. The tumor cell aggregates showed cribriform and solid lobules and were embedded in lakes of mucin, separated by thin, fibrous septae. Focally single neoplastic cells were arranged in an Indian-file pattern. The tumor cells displayed an eosinophilic cytoplasm, large basophilic nuclei and some discrete nuclear atypia. Vascular spaces, filled by densely packed erythrocytes between the septae, were also observed. We compared the mucinous component with the tumor cell and the stromal component by light microscopy. Analyzing the tumor by an image analysis system in Alcian-blue-stained serial sections, we found the averaged total tumor area measuring 99.7 mm(2). The area of the mucinous component measured 92.4 mm(2), that of the tumor cells 3.7 mm(2) and that of the stromal component 3.6 mm(2). The extensive checkup of the patient disclosed no evidence for a further malignant neoplasm. After excision of the tumor an adjuvant radiotherapy was performed. The patient was free of recurrence and metastatic spread of the mucinous carcinoma during a 4-year follow-up.

Adenocarcinoma, Mucinous↗
PubMed1999

[Papular-purpuric gloves and socks syndrome].

In 1990, we first described the papular-purpuric "gloves and socks" syndrome (PPGSS). It is characterized by an itching erythema and edema of the hands and feet together with oral mucosal lesions and fever. The skin lesions subsequently become purpuric and resolve spontaneously after a few days. In more than half of the patients, an acute parvovirus B 19 infection has been proven. In a few cases of PPGSS, other viral infections have been found, for example hepatitis B and measles. We report on a patient with PPGSS and review the literature.

Acrodermatitis↗
PubMed1999

[Psychoeducational-psychotherapeutic treatment of schizophrenic patients and their caregivers. II. Supplementary findings at a 2-year follow-up].

In this study we investigated whether, in conjunction with neuroleptics, a psychoeducational and cognitively oriented treatment for schizophrenic outpatients and their key-persons can improve the course of schizophrenic illness within a 2-year follow-up. This prospective randomized study covered a total of 191 schizophrenic patients (according to DSM-III-R) and comprised a psychoeducational training and cognitive psychotherapy for patients and counseling for their key persons in various combinations. Patients were examined before, immediately after and 2 years after the end of the intervention. Patients in the treatment groups reduced their overall psychopathology and their attention deficit. For patients receiving all three treatment conditions, there was a relevant preventive effect with regard to the rehospitalization rate appearing during the second year of the follow-up. We conclude that in the mid-term, a combination of psychoeducational and cognitively oriented therapy for patients and their keypersons can improve the course of schizophrenic illness.

Adult↗
PubMed1999

Long-term effects of a psychoeducational psychotherapeutic intervention for schizophrenic outpatients and their key-persons--results of a five-year follow-up.

The study examines long-term effects on rehospitalization rates of a psychoeducationally and cognitive-behaviorally oriented intervention for schizophrenic outpatients and their key-persons. 191 patients and their key-persons were allocated by random into four different treatment groups and one control group. Five years after completion of treatment 126 patients were reexamined by interviews or case notes. The rate of patients experiencing psychiatric rehospitalization during the follow-up was assessed in each respective treatment group. Concerning rehospitalization rates there was no significant difference between controls (n = 35) and patients of the four treatment groups (n = 91). There were, however, fewer rehospitalized patients in the group with combined psychoeducational and cognitive treatment, including key-person counselling (42%), than in the control group (69%). Regarding the overall functioning, the patients in this treatment group did slightly better than those in the control group. These results are in accordance with the findings of comparable studies.

Ambulatory Care↗
PubMed1999

Regression of neuropsychological deficits in early-treated phenylketonurics during adolescence.

Even early-treated phenylketonuric patients suffer from phenylalanine-associated (mild) neuropsychological impairment. To date it is still unclear whether patients' deficits show a progression on ageing. This unsolved question seems to be an important aspect in the still ongoing debate about how long and how strictly the patients should be maintained on diet. Twenty early-treated (20 +/- 10, 9-30 days) adolescent phenylketonurics (10 boys, 10 girls) and 20 healthy controls, matched for age, sex and IQ, were investigated twice at a mean ages of 11 and 14 years for their IQ (Culture Fair Intelligence Test-Scale 2; CFT-20), fine motor abilities (Motor Performance Task), sustained (Test d2) and selective attention (Stroop-Task). At the first test, examinations revealed significant blood phenylalanine-correlated neuropsychological deficits in PKU patients. In spite of raised blood phenylalanine concentrations during the following 3 years and significantly elevated concurrent blood phenylalanine concentrations, the repeated measurements revealed a significant decrease of patients' deficits compared to controls. Clinical-neurological status of patients and controls was normal at both test times. The results indicate a decreased vulnerability of PKU-patients with respect to their neuropsychological functioning against elevated blood phenylalanine levels on ageing.

Adolescent↗