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Biomedical subjects

H Bickel

Publications and source records attributed to H Bickel.

At least 37 records · Page 2Linked to original sources
PubMed1994

Incidence and relative risk of dementia in an urban elderly population: findings of a prospective field study.

To ascertain the frequency and distribution of new cases of dementing illness in the elderly population of Mannheim (population 308,000), a survey sample of community residents of > 65 years of age was re-examined after an interval of 7-8 years, and a sample of elderly persons in long-stay care after 5-6 years. The estimated annual incidence rate for all forms of dementia, after correction for ageing of the samples, was 15.4 per 1000 persons aged > 65, made up of dementia of Alzheimer type (8.9 per 1000), vascular dementia (4.4 per 1000) and other forms (2.1 per 1000). Apart from the expected association with age > 65 years, the most powerful predictors of onset of a dementing illness were residence in long-stay care and the presence of minor cognitive deficits at the initial examination, each of which was associated with a highly significant increase in risk. In addition, a number of socio-demographic characteristics (marital status, occupational history and quality of present living accommodation) was found to be predictive, though analysis of larger samples or pooled research data would be necessary to establish their importance in this respect. The findings suggest that identification of mild cognitive impairment on screening could prove helpful in assessing risk for dementia from two to three years before the condition becomes clinically apparent.

Aged↗
PubMed1994

Growth and skeletal maturation in children with phenylketonuria.

Growth and skeletal maturation was evaluated in 82 children participating in the German Collaborative Study of Children Treated for Phenylketonuria (PKU). Height, weight, head circumference and bone age were recorded at regular intervals for the first 6 years of life. The mean SD score (SDS) for height was not significantly different from zero at study entry, but decreased mainly during the second year of life to a nadir of -0.78 in boys and -0.54 in girls at 2.5 years. During the subsequent years, a significant trend towards a regain of height SDS was noted in both sexes. Weight-for-height SDS was close to zero in both sexes, with a significant continuous increasing trend throughout the observation period. Head circumference SDS decreased in boys during the first year of life from -0.28 to -0.68, whereas girls showed only a minor change. During the further follow-up period, head circumference SDS remained at approximately -0.3 in boys and 0.0 in girls. While the mean verbal and performance IQ of the total study population at 5 and 6 years of age did not differ from a group of 212 healthy non-PKU children, patients with a head circumference SDS less than the population median at 2 years of age exhibited poorer cognitive abilities at school age than those patients with a relative head size greater than the population median. The children with a head circumference less than the median at 2 years had smaller head sizes already at birth; in addition, the change in relative head size during the first 2 years was correlated significantly with cognitive abilities at school age in boys. Mean bone age was identical to chronological age at each time point of observation. The rate of maturation was one year of bone age per year of chronological age. No correlation between phenylalanine intake or phenylalanine concentrations and the rates of body or head growth or skeletal maturation could be established. We conclude that despite adequate weight gain, moderate growth retardation occurred during the first 2 years of life in this group of children treated for PKU. Growth was more compromised in boys than in girls and tended to be compensated during later follow-up. Early infantile head circumference and growth appear to be predictors of cognitive development.

Age Determination by Skeleton↗
PubMed1994

[Utilization of inpatient care and management in aging].

The use of nursing homes and of homes for the aged during the remaining lifetime of elderly persons was investigated by means of a retrospective longitudinal study. The findings are based on a sample of persons 65 years of age or older who died during twelve months in the city of Mannheim. Information is given with regard to the probability of home admission, the duration of stay in the homes and the distribution of care provision according to demographic variables. The results point to a further increase in the need for long-term care due to the growing life expectancy.

Aged↗
PubMed1993

Genotype-phenotype correlations in phenylketonuria.

Genotyping of the phenylalanine hydroxylating system offers a new way of characterizing patients with phenylalanine hydroxylase (PAH) deficiency. This paper investigates the power of genotyping as a parameter for differential diagnosis and as a measure of the risk factor of brain damage in well-treated patients with phenylketonuria (PKU). Thirty-three PKU patients were followed up over 9 years and the quality of dietary treatment, plasma phenylalanine (phe) in the newborn period before treatment and intellectual outcome at the age of 9 years were measured and correlated with the predicted residual activity (PRA) of the phe hydroxylase system as estimated from mutation analysis of the PAH gene. Patients were grouped in group Ia (PRA = 0%), group Ib (PRA = 5-15%) and group II (PRA > or = 25% of the normal activity). Mean plasma phe levels in the newborn in group Ia were 37.9 +/- 6.5 (2296 +/- 394), in group Ib 40.8 +/- 15.9 (2472 +/- 963) and in group II 16.2 +/- 4.2 (981 +/- 254) mg/dl (mumol/l). Difference in mean plasma values of groups Ia and Ib on the one hand and group II on the other were highly significant (P < 0.0001). No difference could be seen between groups Ia and Ib. There was a higher mean IQ at the age of 9 years in group II (97.4 +/- 5.4) in comparison with groups Ia (92.7 +/- 12.8) and Ib (85.0 +/- 14.4). The difference between group Ib and group II was significant (P < 0.040).(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1993

[Psychiatric disorders in elderly general hospital patients: incidence and long-term prognosis].

As part of a survey conducted in six general-hospital departments of internal medicine, in the neighbouring cities of Mannheim and Ludwigshafen (total pop. 470,000), 626 patients in the age range 65 to 80 years, all admitted from private addresses, were screened by means of a standardized questionnaire (Cognitive and Affective Screening of the Elderly--'CASE'). All patients whose scores indicated possible mental abnormality, together with a proportion of those having normal scores, were then examined in greater detail, using the Clinical Psychiatric Interview. Following correction, the screening results indicated a frequency of 30.2% for clinically significant psychiatric disturbance, made up of 9.1% with organic mental disorders and 21.1% with functional mental illness only. These rates are considerably higher than could be expected on the basis of a field study of the background population. One year after hospital discharge, the numbers of deaths and of admissions to long-stay care were established for the whole sample, and in addition, individually matched sub-samples of 100 mentally ill and 100 mentally normal patients were reinvestigated. A second follow-up of the matched sub-samples was undertaken after a further interval of 5.6 years on average. The results of follow-up show that 75% of the identified cases ran a chronic or recurring course, while only a small proportion proved to be transient reactions to physical illness or hospital admission. In general, the psychiatrically ill patients had a relatively unfavourable outcome, even after the effects of age, physical disability and other relevant variables had been controlled for. When compared with the matched group of mentally normal patients, they manifested a 43% excess of mortality, and an increase of 157% in the risk for having to be admitted to long-stay care.

Activities of Daily Living↗
PubMed1992

[Dementia diseases and minor cognitive impairments in elderly patients in general practice. Results of a cross-sectional study].

General practitioners in 24 Mannheim practices kept a record of all over-65-year-old patients seen during four weeks (n = 3,737), and made ratings of their cognitive functioning with the help of simple guidelines. For a sub-sample of patients (n = 407), these ratings could be compared with assessments made by the research team, on the basis of a standardized interview and test procedure (Hierarchic Dementia Scale). The research data indicate that 8% of the patients manifested clinical dementia, a further 8% so-called 'mild dementia' and 17% milder, non-disabling degrees of cognitive impairment. The proportion of affected persons in each of the groups rises steeply with increasing age above 65 yr. The ability of the practitioners to detect dementia--including the milder degrees--among their elderly patients exceeded expectation (sensitivity 92%; specificity 76%). The test-score profiles of the patients, grouped according to their own doctors' ratings, conformed to clinical concepts of the progressive course ('staging') of dementing illness, while the degree of disability in everyday life, and dependency on others, also increased steeply across the groups. These findings emphasize the importance of general medical practice for the early detection of dementia in the elderly population, and potentially also for case management.

Activities of Daily Living↗
PubMed1991

Mental illness in a cross-national perspective. Results from a Brazilian and a German community survey among the elderly.

Findings for unselected samples for the elderly in two urban populations - one in Mannheim, Germany (n = 418) and the other in Sao Paulo, Brazil (n = 111) - are compared and contrasted. Each study was restricted to persons aged over 65 years living in private households, and each employed a single-stage method of psychiatric case-identification, based on the Clinical Interview Schedule (CIS). Apart from marked differences in educational standards and proportions living alone the two samples were broadly similar in their recorded socio-demographic characteristics. Comparison revealed no significant difference in total prevalence, though there was a trend towards a higher case-frequency in Sao Paulo (29.7%) than in Mannheim (23.3%). The clinical-item profiles for the two samples indicated a higher rate of symptom reporting in Sao Paulo, whereas the Mannheim sample had higher mean scores for a number of psychiatric abnormalities observed at interview. Separate cluster analyses carried out on the two data sets divided the samples into four pairs of sub-groups with similar clinical profiles, which were designated respectively as 'organic', 'depressive', 'neurotic' and 'normal'. While a more careful standardization of method would probably reduce the observed disparities between the samples, some of these are thought to be real and to relate to sociocultural differences, as well as to the greater stresses of daily life in Sao Paulo.

Aged↗
PubMed1990

Significance of the in vivo deuterated phenylalanine load for long-term phenylalanine tolerance and psycho-intellectual outcome in patients with PKU.

In 20 patients with PAH deficiency, in vivo RA was determined by an intravenous deuterated Phe load. Sixteen patients had RAs of less than 0.4% of normal, 3 a clearly detectable activity between 0.8 and 1.4% of normal. Long-term Phe tolerance as measured by the distribution of plasma Phe levels in categories (0-3.9, 4.0-9.9, 10-15.9 and over 16 mg/dl) was much improved in patients with RAs greater than 0.8%. There was a negative correlation between RA and number of plasma Phe levels greater than 16 mg/dl. Relationship between full scale IQ at the age of 9 years and dietary control showed a positive correlation between IQ and the number of Phe levels between 0-10 mg/dl (k = .50 p less than 0.05). Highest (negative) correlation (k = -0.67 p less than 0.007) was found between full scale IQ and the number of Phe values greater than 16 mg/dl as measured over 9 years. On the one hand detectable RA of PAH reduces the risk of high Phe levels and thus may also reduce the risk of brain damage in untreated or suboptimally treated patients with PAH. On the other hand enzyme measurement of PAH is no predictive parameter for Phe tolerance in an individual patient since RA may be very similar in phenylketonuric/hyperphenylalaninaemic patients. For practical purposes the oral protein loading test at the age of 6 months will give the most reliable results for differential diagnosis of PAH deficiency.

Aging↗
PubMed1989

[psychogeriatric disorders and mortality].

In two representative samples of persons aged over 65, living in private households (N = 343) and in nursing homes (N = 146), mortality was studied prospectively in relation to psychiatric status. After a cross-sectional baseline examination the two groups were followed up over eight and six years respectively. The study shows a high mortality risk among persons with dementing disorders and a short-term increase among persons with functional psychiatric disorders, even after controlling for the most significant confounding variables (age, sex, physical impairment) using the proportional hazards regression model developed by Cox.

Aged↗
PubMed1989

[Probability and duration of inpatient care in the aged].

The utilization of geriatric homes by the elderly population of Mannheim was investigated by means of a retrospective longitudinal study. The findings give information with respect to the cumulative probability of geriatric-home admission, the mean duration of stay in the homes, the proportion of persons in the elderly population resident in geriatric homes, and the distribution of home-care provision according to age, sex and marital status.

Aged↗
PubMed1988

Long-term development of intelligence (IQ) and EEG in 34 children with phenylketonuria treated early.

In 34 children with phenylketonuria (PKU) treated early the prognostic value of the age on institution of the diet (within the first 3 months of life) and of the quality of dietary treatment was determined in two different ways: 1) following intelligence closely (IQ) and (2) evaluating the EEG development up to their 12th (n = 34) and 15th (n = 18) years of life as appropriate. In general, IQ scores were found to be normal from the 4th-15th years of life. In our group of patients there was no effect on the IQ of the timing of diet onset. Children with "strict" dietary control showed a significantly higher IQ than those with "loose" control. One hundred and fifty-four EEGs (10/20 system, awake with eyes closed) were recorded at intervals of 2 years and conventionally evaluated. The development of alpha-activity was found to be normal. Beta-activity was enhanced. Abnormal EEG findings like general slowing and generalized paroxysmal activity (GPA) with or without spikes were more frequent in children with PKU than in controls, with the exception of focal abnormalities. EEG abnormalities increased with advancing age independently of IQ development and showed no relation to either the age at the onset nor the quality of dietary treatment.

Adolescent↗
PubMed1987

Fanconi-Bickel syndrome.

Clinical, biochemical, functional and morphological data are presented in nine infants, children and adults, with Fanconi-Bickel syndrome. Long-term follow-up studies show severe growth retardation, partly compensated for by late onset of puberty. Glomerular filtration rate is normal or slightly decreased. Renal tubular dysfunction is characterized by a specific pattern of impaired proximal tubular transport mechanisms, with marked impairment of glucose transport. The utilization of glucose and galactose is defective, whereas fructose metabolism seems to be normal. Glycogenosis of the liver may be an epiphenomenon. Glycogen accumulation in the kidney is limited to the proximal tubule, with maximal levels in the straight part. The Fanconi-Bickel syndrome is a defined clinical entity which is distinguished from other inherited metabolic diseases by complex defects of renal tubular transport and other forms of glycogenosis.

Adolescent↗
PubMed1987

[Possible interfering factors in the screening of newborn infants for inborn errors of metabolism and measures for their prevention].

From taking a sample to communicating the result, the success of neonatal screening is endangered by a series of pitfalls. Besides internal and external quality control and an adequate practical experience due to high sample throughput, it is important to know these possibilities of errors in order to be able to reduce them to a minimum in daily routine work.

False Positive Reactions↗
PubMed1986

Follow-up study of 16 years neonatal screening for inborn errors of metabolism in West Germany.

Capillary blood samples from almost one million neonates from Baden-Württemberg were investigated for inborn errors of metabolism between 1969 and 1984 in our screening centre. Besides 7 patients with maple syrup urine disease (MSUD), 3 with homocystinuria and 18 with galactosaemia, a follow-up of the positive screening results confirmed 94 patients with phenylketonuria (PKU) and 76 with non-PKU hyperphenylalaninaemia (non-PKU HPA). The incidence of PKU is 1:10000, and that of HPA in the wider sense (PKU and non-PKU HPA) as obtained by newborn screening before further classification at 6 months 1:5532. For West Germany as a whole, the number of newly discovered cases with persistent hyperphenylalaninaemia was 1480 in the same period. The subdivision into PKU and non-PKU HPA is not yet possible from this figure. It is strongly suggested that the abnormal results of newborn screening for phenylalanine be designated as hyperphenylalaninaemia (in the wider sense) and that the terms "PKU" or "non-PKU HPA" be used only after further differentiation as carried out by us at the age of 6 months.

Capillaries↗