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Biomedical subjects

H Bickel

Publications and source records attributed to H Bickel.

At least 19 recordsLinked to original sources

[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

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

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

[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

[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

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

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

[Sulfites in infusion solutions].

9 commercially available high-calorie carbohydrate solutions were investigated for their sulfite content. It became evident that 6 manufacturers of infusion solutions add varying amounts of sulfite to their solutions. We consider such an addition to be unnecessary and risky from the toxicological point of view.

Carbohydrates

Maple syrup urine disease: treatment of the acutely ill newborn.

Three patients with maple syrup urine disease were treated during the acute neonatal stage. Multiple exchange transfusions proved to be a satisfactory means of achieving rapid clinical and biochemical improvement during this phase. On the other hand, evidence is provided suggesting that in addition to exchange transfusions, a high calorie intake above 150 Cal/kg body weight/day is necessary to lower the plasma concentration of the branched chain amino acids to near-normal levels. As long as this calorie intake was not provided, further exchange transfusions failed to lower the plasma leucine concentration to below 17 mg/100 ml in one patient. It is assumed that this high calorie intake is necessary to prevent the breakdown of endogenous protein. Treatment of acute episodes in maple syrup urine disease should therefore not only eliminate the elevated alpha-keto acids and branched chain amino acids quickly (i.e. by multiple exchange transfusions or peritoneal dialysis), but in addition should provide a high calorie intake.

Energy Intake

Effect of stopping low-phenylalanine diet on intellectual progress of children with phenylketonuria.

Forty-seven patients at the Hospital for Sick Children, London, who had phenylketonuria and were on a low-phenylalanine diet (21 early-treated--that is, treatment started before the age of 4 months--and 26 late-treated) were placed on a normal diet between the ages of 5 and 15 years. They showed significant falls in mean IQ of about six points after the diet was withdrawn. Twenty-two similar patients (five early-treated and 17 late-treated) at the Universitäts-Kinderklinik, Heidelberg, who were placed on a relaxed low-phenylalanine rather than a normal diet, showed smaller and non-significant falls in mean IQ. During the period of strict diet none of the patients in London or Heidelberg showed any consistent falls in IQ. These results suggest that complete withdrawal of the low-phenylalanine diet during childhood leads to a fall in intellectual progress in many patients.

Adolescent