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  • Phenylketonuria  (3)
  • Developing countries  (2)
  • 1
    Electronic Resource
    Electronic Resource
    Investigation of the nutritional state of children in a Congolese village (1988)
    Springer
    European journal of pediatrics 148 (1988), S. 159-163 
    Details
    ISSN: 1432-1076
    Keywords: Essential fatty acids ; Plasma ; Developing countries ; Africa ; Child nutrition
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The fatty acid status of an unselected group of 84 Congolese children was determined by estimating the fatty acid composition of the plasma phospholipid (PL) and cholesterolester (CE) fractions using capillary gas chromatography. In comparison with North American and European children a wide range of values and low mean percentages (related to the fatty acids of a chain length of 14–24 carbon atoms) were found for the following fatty acids (mean±SD): linoleic acid (LA) (PL: 17.19±3.85; CE: 40.13±7.72); arachidonic acid (AA) (PL: 6.60±2.28; CE 4.32±1.71); dihomo-gammalinolenic acid (DHLA) (PL: 1.80±0.68; CE 0.41±0.22). On average, ω3 fatty acids were higher than in reference groups, while similar values were found for the monoenoic and ω9 fatty acids. No sample contained eicosatrienoic acid, 20:3ω9 (ETA) which, dependent on analytical methods, may be difficult to separate from behenic acid (22:0). Changes in the relation between the two lipid fractions occurred when the LA content in CE was less than 35%. Also the correlation between LA and AA in CE, which was significant below 35% LA (r=0.84), changed with higher values for LA (r=0.01). These findings may indicate that alterations in fatty acid metabolism occur when LA values in CE — which are supposed to be directly related to the dietary intake of polyunsaturated fatty acids — fall below 35% of all fatty acids in this lipid fraction.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00445928
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  • 2
    Electronic Resource
    Electronic Resource
    Investigation of the nutritional state of children in a Congolese village (1988)
    Springer
    European journal of pediatrics 148 (1988), S. 155-158 
    Details
    ISSN: 1432-1076
    Keywords: Plasma proteins ; Immunoglobulins ; Circulating immune complexes ; Anthropometry ; Developing countries
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The nutritional status of an unselected group of 111 children from the village of Bouansa, People's Republic of the Congo, was studied. Comprehensive clinical examinations, anthropometrical measurements and analysis of albumin, prealbumin, ferritin, C-reactive protein (CRP), IgA, IgG, IgM, IgE, IgG- and IgM-circulating immune complexes (CIC) were carried out. The results show, by anthropometrical classification, a high prevalence of moderate malnutrition. Low levels of plasma proteins and high levels of immunoglobulins and CIC were found. No correlation between anthropometrical classification and plasma proteins was established. Children with increased levels of CRP showed low prealbumin values and increased levels of ferritin. Patterns of immunoglobulins and CIC were close to those found in other studies in tropical countries. To evaluate the anthropometrical and biochemical findings it is necessary to take into consideration the apparently healthy appearance of the children, which shows the degree of adaptation to the limited availability of food and the high rate of acute and chronic infections.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00445927
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Neurological outcome in adult patients with early-treated phenylketonuria (1998)
    Springer
    European journal of pediatrics 157 (1998), S. 824-830 
    Details
    ISSN: 1432-1076
    Keywords: Key words Intelligence ; Neurology ; Neuropsychology ; Phenylketonuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Due to the observation of severe neurological symptoms in single patients as well as brain imaging, neuropsychological and neurophysiological abnormalities, the long-term prognosis of treated phenylketonuria is still under discussion. We investigated the neurological outcome of 57 (24 male, 33 female) patients with phenylketonuria (diet onset 〈3 months) at a mean age of 23.6 (17–33) years in comparison to control subjects. Methods used were a clinical-neurological examination, tests for fine motor abilities, IQ test (WAIS-R), a neuropsychological attention task and MRI (30 patients only). Tremor was increased in the patients (28%) compared to controls (15%). Fine motor abilities were significantly reduced in three areas: hand-wrist steadiness, finger-hand dexterity and hand-wrist speed. Tremor as well as reduced fine motor skills were not associated with treatment-related variables, e.g. diet onset, strictness of biochemical control or amount of MRI white matter change. IQ was lower in patients (mean 97.6) compared to matched control subjects (mean 105.5). IQ at 12 years was correlated with biochemical control from birth up to the age of 12 and remained stable up to adult age, independent of biochemical control after 12 years of age. In contrast to the other outcome parameters, the performance in a neuropsychological attention task was influenced by the concurrent plasma phenylalanine concentration. Specific late-onset neurological impairment was not identified in this sample of early-treated adults with phenylketonuria. Conclusion Careful neurological investigation revealed subtle symptoms of brain damage even after early-initiated treatment in adult patients with phenylketonuria. At present it cannot be excluded that further neurological deterioration could emerge later in life. Thus, patients with phenylketonuria – either on or off diet – should be monitored throughout life.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050945
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  • 4
    Electronic Resource
    Electronic Resource
    Selenium intake of infants and young children, healthy children and dietetically treated patients with phenylketonuria (1984)
    Springer
    European journal of pediatrics 143 (1984), S. 99-102 
    Details
    ISSN: 1432-1076
    Keywords: Selenium ; Intake ; Children ; Phenylketonuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In 20 healthy infants and children, 5–20 months old, the Se intake was estimated by analysing food samples by instrumental neutron activation analysis. The intake was calculated by weighing the portions offered and actually consumed. The median Se content of the food amounted to 27 ng/g wet weight (gww) and median daily Se intake to 33.5 μg. The Se intake was not equally distributed over the day. About 50% of the daily Se intake was derived from the supper. The main Se sources (41%) for young children were cereal paps. Commercially, available meals (30 ng/g) contained less Se than home-made ones (50 ng/g). In nine dietetically treated patients with phenylketonuria the median Se intake amounted only to 6.9 μg/day corresponding to a mean Se content of the diet of 7.9 ng/g. The main Se source in the diet was vegetables (36.3%) and 20% derived from their protein supplements. The Se intake of young children, healthy or dietetically treated, cannot be calculated accurately from tables but must be estimated by measuring the Se content of the local food because cereals and vegetables-the main Se sources-exhibit great regional variations.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00445794
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    Comparison of the protein quality of dietetically treated phenylketonuria patients with the recommendations of the WHO Expert Consultation (1996)
    Springer
    European journal of pediatrics 155 (1996), S. S153 
    Details
    ISSN: 1432-1076
    Keywords: Key words Protein quality ; Dietetic treatment ; Phenylketonuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The protein quality of the diets of phenylketonuria (PKU) children of different ages (3 months, 10 months, 3 years, 8 years, 12 years, 16 years) with low or high phenylalanine (Phe) tolerance was assessed according to the recommendations of the FAO/WHO consultation group [13]. The amount of each essential amino acid (AA) per gram dietary protein was calculated and compared to the reference. The resultant amino acid score (AAS) indicated a limited to inadequate biological protein quality of the diets in 3-month-old infants (2.2 g protein/kg body weight/day) and 10-month-old infants (2.0 g protein/kg body weight/day) with a “high” Phe tolerance. In all other age groups the AAS was 〉 100%. However remarkable imbalances in the AA pattern were apparent. Beginning with the age of 3 years (1.7 g protein/kg body weight/day) the intake of the AA lysine and isoleucine was three or two times higher than recommended. At the age of 8 years (1.4 g protein/kg body weight/day) the intake of three AA (valine, isoleucine, lysine) was – related to the WHO recommendations – 217%, 229% and 291%. Similar results could be found in the age groups of 12 years (1.1 g protein/kg body weight/day) and 16 years (0.9 g protein/kg body weight/day), respectively. These calculations might help to reconsider the composition of the AA mixtures used in the dietetic treatment of PKU patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014235
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