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  • 1
    Electronic Resource
    Electronic Resource
    Follow-up study of a nation-wide neonatal metabolic screening program in Japan (1984)
    Springer
    European journal of pediatrics 142 (1984), S. 204-207 
    Details
    ISSN: 1432-1076
    Keywords: Neonatal screening ; Phenylketonuria ; Maple syrup urine disease ; Homocystinuria ; Galactosemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A nationwide neonatal sreening program for phenylketonuria (PKU), maple syrup urine disease (MSUD), homocystinuria, histidinemia and galactosemia was started in Japan in 1977. The total number of infants screened had reached 6,311,754 by March, 1982. A follow-up study revealed the incidence of the disease in Japan: 1/108,823 for PKU; 1/450,840 for hyperphenylalaninemia (HPA); 1/1,577,939 for biopterin deficiency; 1/525,980 for MSUD; 1/1,051,959 for homocystinuria; 1/8,371 for histidinemia, and 1/788,969 for galactosemia type 1. The incidences of PKU, HPA, homocystinuria, and galactosemia (type 1) were found to be markedly low in Japan as compared with those in Caucasian countries. There was no great difference in the incidence of MSUD between both. On the other hand, the incidence of histidinemia was higher in Japan. It was found that most of the patients with PKU, HPA, MSUD, homocystinuria, or galactosemia are developing normally due to the early initiation of dietary treatment. These results clearly indicate that the neonatal mass screening program plays a great role in preventing the occurrence of handicapped children.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442450
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  • 2
    Electronic Resource
    Electronic Resource
    d-lactic acidosis in two patients with short bowel syndrome: Bacteriological analyses of the fecal flora (1982)
    Springer
    European journal of pediatrics 138 (1982), S. 324-326 
    Details
    ISSN: 1432-1076
    Keywords: d-lactic acidosis ; Short bowel syndrome ; Lactobacillus ; Kanamycin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two cases of d-lactic acidosis associated with short bowel syndrome are described. The administration of kanamycin to the patients showed a decrease in d-lactate in blood and urine in parallel with disappearance of metabolic acidosis. Bacteriological analyses of the fecal flora showed an increase in Lactobacillus buchneri in the first patient and Lactobacillus fermenti IVa in the second; both bacteria were sensitive to kanamycin. Quantification of in vitro production of d-lactate by each species of bacteria isolated from the feces revealed that Lactobacillus produced more d-lactate than other species of bacteria. These observations indicate that Lactobacillus may play an important role in the induction of d-lactic acidosis in patients with short bowel syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442509
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  • 3
    Electronic Resource
    Electronic Resource
    Ultrastructural study of the childhood mitochondrial myopathic syndrome associated with lactic acidosis (1982)
    Springer
    European journal of pediatrics 139 (1982), S. 25-30 
    Details
    ISSN: 1432-1076
    Keywords: Mitochondrial myopathic symdrome ; Lactic acidosis ; Endothelium ; Ultrastructural study
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The quadriceps femoris muscle and the muscularis mucosae of the rectum from two children with mitochondrial myopathic syndrome associated with lactic acidosis were studied by electron microscopy. Striking morphological abnormalities of mitochondria were noted not only in the skeletal but also in the smooth muscle cells. Endothelial cells of blood capillaries distributed in these affected muscles were so greatly swollen that the capillary lumen was almost occluded. In contrast, surface epithelial and glandular epithelial cells of the rectum contained normal mitochondria, and fenestrated capillaries in the propria mucosae remained intact. Long-term ischemia resulting from occlusive changes of the capillary wall may be responsible for the mitochondrial alterations of muscle cells.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442074
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  • 4
    Electronic Resource
    Electronic Resource
    Dietary therapy in two patients with vitamin B12-unresponsive methylmalonic acidemia (1981)
    Springer
    European journal of pediatrics 135 (1981), S. 305-312 
    Details
    ISSN: 1432-1076
    Keywords: Vitamin B12 unresponsive methylmalonic acidemia ; Protein intolerance ; Dietary therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The biochemical and therapeutic responses to dietary therapy were studied in a 25-month-old girl and a 1-month-old girl with methylmalonic acidemia (MMA-emia), which was unresponsive to vitamin B12. The minimum daily intake of protein which patients could tolerate and display a good development was between 1.0 and 1.2 g per kg body weight. Supplementation with amino acid mixture devoid of toxic amino acids was required to prevent protein malnutrition when daily protein intake was restricted to 0.6 g per kg body weight. Caloric intake should be sufficient, not only to promote growth but also to prevent a rise in MMA level, especially when a patient has ketoacidosis. It was found that MMA excretion per mg creatinine in random urine specimens correlated significantly with serum MMA and twenty four-hour output of MMA per kg body weight. Therefore measurement of MMA in a single urine specimen is useful for evaluating the in vivo accumulation of MMA.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442109
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  • 5
    Electronic Resource
    Electronic Resource
    Epstein-Barr Virus-induced Lymphoblastoid Cell Lines from Patients with Primary Immunodeficiency Diseases (1980)
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 11 (1980), S. 0 
    Details
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Peripheral lymphocytes from eight patients with congenital immunodeficiency diseases were infected with Epstein-Barr virus (EBV) in an attempt to establish B lymphoblastoid cell lines (LCL). These patients included three boys with congenital agammaglobulinaemia, two girls with hypogammaglobulinaemia, one boy with common variable immunodeficiency, one boy with severe combined immunodeficiency with adenosine deaminase deficiency, and one boy with DiGeorge syndrome. Five of the patients bore no surface immunoglobulins (slg) on their peripheral lymphocytes. LCL were established from seven of the eight patients. All the LCL established formed rosettes with EAC3 and had the ability to produce cytoplasmic immunoglobulins (clg) of various classes. Culture supernatants concentrated up to 100-fold developed precipitin bands by Ouchterlony's method with antisera to human Ig in all the established LCL. These results suggested that both slg-, clg- and C3+ cells and slg+, clg- and C3+ cells might be the target cells for EBV and that slg-, clg- and C3+ cells might be the precursor cells of B lymphocytes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-3083.1980.tb00221.x
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  • 6
    Electronic Resource
    Electronic Resource
    Mammary tumorigenic effect of a new nitrosourea, 1,3-dibutyl-l-nitrosourea (B-BNU), in female Donryu rats (1980)
    Springer
    Journal of cancer research and clinical oncology 96 (1980), S. 35-41 
    Details
    ISSN: 1432-1335
    Keywords: Mammary tumor ; Nitrosourea ; 1,3-Dibutyl-l-nitrosourea ; Donryu rat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Four groups (groups 1-4) of female Donryu rats were given continuously 400, 200, 100, or 0 ppm solution of 1,3-dibutyl-1-nitrosourea (B-BNU) as their drinking water, and were studied for the development of tumors. The incidence of mammary tumors was 15/19 (79%), 20/24 (83%), 21/26 (81%), and 8/25 (32%) in groups 1, 2, 3, and 4, respectively. In addition, hematopoietic neoplasms, uterine tumors, and vaginal tumors developed in 13, 11, and six rats, respectively in 69 treated rats. Other tumors were infrequent.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00412895
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  • 7
    Electronic Resource
    Electronic Resource
    Theory of electric field-induced optical second harmonic generation in semiconductors (1980)
    Springer
    Optical and quantum electronics 12 (1980), S. 199-205 
    Details
    ISSN: 1572-817X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Electrical Engineering, Measurement and Control Technology , Physics
    Notes: Abstract The nonlinear optical coefficientd ij(kl) (2ω, 0,ω, ω) for electric field-induced optical second harmonic generation in semiconductors is calculated by means of a perturbation treatment. The result is given directly as a Brillouin zone integral over a resonant energy denominator. A simplified energy band structure model is used to carry out the Brillouin zone integral. The analytic closed-form expression ford ij(kl) (2ω, 0,ω, ω) thus obtained permits the calculation of the absolute value of its spectrum from available energy band parameters. The dispersion ofd 11(11) (2ω, 0,ω, ω) of Ge is numerically calculated, second harmonic photon energies being close to theE 0 andE 1 gaps. The results show pronounced resonant behaviours.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00620037
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  • 8
    Electronic Resource
    Electronic Resource
    Temperature compensated coupled cavity diode lasers (1984)
    Springer
    Optical and quantum electronics 16 (1984), S. 463-469 
    Details
    ISSN: 1572-817X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Electrical Engineering, Measurement and Control Technology , Physics
    Notes: Abstract It has been shown theoretically that the temperature coefficient of the oscillation wavelength in semiconductor lasers can be reduced to zero by coupling an external cavity whose optical length decreases with temperature. Here are described two types of temperature compensated coupled cavity (TC3) diode lasers and their properties calculated in the steady state. In such devices an interference effect due to multi-cavities is expected to cause unstable mode hopping behaviour, but it was found to be settled by choosing an adequate external cavity length.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00619639
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  • 9
    Electronic Resource
    Electronic Resource
    Clinical and biochemical findings on a child with multiple biotin-responsive carboxylase deficiencies (1982)
    Springer
    Journal of inherited metabolic disease 5 (1982), S. 67-68 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01799990
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  • 10
    Electronic Resource
    Electronic Resource
    Glycogen storage disease type 1b due to a defect of glucose-6-phosphate translocase (1982)
    Springer
    Journal of inherited metabolic disease 5 (1982), S. 227-228 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstracts Patients with glycogen storage disease (GSD) type 1b have shown normal activity of glucose-6-phosphatase (EC 3.1.3.9) as assayed in frozen liver, though their clinical and biochemical findings were similar to those of patients with GSD 1a (McKusick 23220) (Senior and Loridan, 1968). In 1978, we suggested that a basic defect of GSD 1b exists in the glucose-6-phosphate (G6P) transport system (Narisawa et al., 1978; Igarashiet al., 1979). Since then, there have been reports confirming our observation (Beaudetet al., 1980; Langeet al., 1980; Corbeelet al., 1981; Schaubet al., 1981). Recently, it was postulated that the G6Pase system contains a phosphate translocase which mediates the efflux of phosphate, in addition to a G6P translocase and a non-specific phosphohydrolase (Arionet al., 1980). Therefore, it is possible that GSD 1b is caused by a defect of phosphate translocase. In this paper, the basic defect in GSD type 1b was investigated in two patients; one with severe, the other with mild, clinical symptoms.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02179148
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