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  • 1
    Electronic Resource
    Electronic Resource
    Rhodium carbene complexes. The structure of chlorobis(triphenylphosphine)tris(ethoxycarbonyl isothiocyanato)rhodium and the synthesis and proposed mechanism of formation of this and related complexes (1977)
    s.l. : American Chemical Society
    Journal of the American Chemical Society 99 (1977), S. 2118-2126 
    Details
    ISSN: 1520-5126
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1021/ja00449a017
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  • 2
    Electronic Resource
    Electronic Resource
    A fluorescent microscopy study of biopsied muscles from infantile neuromuscular disorders (1983)
    Springer
    Acta neuropathologica 59 (1983), S. 48-52 
    Details
    ISSN: 1432-0533
    Keywords: Acridine Orange ; RNA ; Regeneration ; Denervation ; Fluorescent microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The Acridine Orange (AO) stain for muscle biopsies is particularly useful to identify regenerating and ongoing hypertrophic muscle fibers under fluorescent microscopy. This method was applied to muscle biopsies from 65 patients who suffered from various childhood neuromuscular disorders. While normal fibers showed dull green cytoplasm with small green-yellow nuclei, striking fluorescent fibers were observed in eight cases of congenital muscular dystrophy (CMD) and 12 cases of Duchenne muscular dystrophy (DMD); these fibers were characterized as follows: (1) small fibers with big oval or spherical nucleic which fluoresced strongly with a bright orange color; (2) fibers of various sizes and different degrees of orange fluorescence; and (3) opaque fibers with bright yellow cytoplasm. The small diameter fibers in Werdnig-Hoffmann (WH) disease, nemaline myopathy, and congenital fiber type dysproportion failed to show apparent AO-RNA fluorescence. Although all the atrophic fibers in Kugelberg-Welander (KW) disease showed a vague orange fluorescent color, this was obviously different from that of regenerating fibers seen in CMD and DMD. In addition to these findings, the hypertrophic fibers in a case of unclassified myopathy also showed moderate organe fluorescence around the entire periphery of the cytoplasm.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00690316
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  • 3
    Electronic Resource
    Electronic Resource
    Pathology of skeletal muscle and intramuscular nerves in infantile neuroaxonal dystrophy (1986)
    Springer
    Acta neuropathologica 69 (1986), S. 117-123 
    Details
    ISSN: 1432-0533
    Keywords: Skeletal muscle ; Intramuscular nerves ; Infantile Neuroaxonal Dystrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Biopsies of the biceps muscle and sural nerve were taken from a girl aged 2 years with infantile neuroaxonal dystrophy (INAD). In addition to the typical axonal spheroid bodies in a number of the i. m. nerve fibers, the neuromuscular junctions (NMJs) and motor nerve endings also contained axonal swellings. The sural nerve, except for three dystrophic fibers, was almost completely normal. A teased nerve preparation showed four additional abnormal fibers with focal axonal enlargement similar to those in giant axonal neuropathy (GAN). These results suggest that a biceps muscle biopsy may be more useful than a sural nerve biopsy for the diagnosis of INAD, because the muscle contains abnormal peripheral nerves and NMJs in high frequency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687047
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  • 4
    Electronic Resource
    Electronic Resource
    Aarskog syndrome with isolated growth hormone deficiency (1981)
    Springer
    European journal of pediatrics 135 (1981), S. 273-276 
    Details
    ISSN: 1432-1076
    Keywords: Aarskog syndrome ; Isolated growth hormone deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 5-year-old male with the Aarskog syndrome is described. He had abnormal facies, short stature, short fingers with interdigital webbing, a saddle type scrotum and mild mental retardation. In addition, he had isolated growth hormone deficiency as evidence by the insulin, arginine, and propranolol-glucagon tests. An arginine test after short-term stimulation with estrogen further supported this diagnosis. His mother had minor abnormalities of the hands and feet, and slight mental retardation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442102
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  • 5
    Electronic Resource
    Electronic Resource
    Circulating immune complex in the mucocutaneous lymphnode syndrome (1983)
    Springer
    European journal of pediatrics 141 (1983), S. 50-51 
    Details
    ISSN: 1432-1076
    Keywords: Mucocutaneous lymphnode syndrome (MCLS) ; Kawasaki disease-Circulating immune complex (CIC)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In 16 patients with mucocutaneous lymphnode syndrome (MCLS) during the first 2 weeks after the onset (acute phase) and 1 month after the onset (remission phase), measurement of the circulating immune complex (CIC) was performed by a C1q-binding assay (C1q-B.A.) and/or a Protein-A precipitation test (Protein-A P.T.). Seven out of 12 samples and four out of nine samples were shown to have raised levels of CIC in the acute phase with the C1q-B.A. and Protein-A P.T. test. In the remission phase, on the other hand, positive results were found in one out of six samples with the C1q-B.A. test and in three out of nine samples with the Protein-A P.T. test. High levels of CIC and disease activity were parallel. Our finding of a higher incidence of positive CIC in MCLS supports the possibility of the immunopathological mechanism.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00445669
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  • 6
    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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  • 7
    Electronic Resource
    Electronic Resource
    Depressed natural killer cell activity due to decreased natural killer cell population in a vitamin E-deficient patient with Shwachman syndrome: reversible natural killer cell abnormality by α-tocopherol supplementation (1997)
    Springer
    European journal of pediatrics 156 (1997), S. 444-448 
    Details
    ISSN: 1432-1076
    Keywords: Key words Vitamin E deficiency  ;  Natural killer cell activity  ;  Shwachman syndrome α-Tocopherol supplementation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Natural Killer (NK) cell activity was examined in a 16-month-old Japanese boy with Shwachman syndrome associated with severe vitamin E deficiency. As evaluated by 51Cr-release assay from K562 cells, NK cell activity was constantly decreased. After 8 weeks of oral α-tocopherol (α-Toc) supplementation (100 mg/day), NK cell activity had normalised. When α-Toc supplementation was interrupted for 16 weeks, NK cell activity again decreased. Flow cytometry of peripheral lymphocytes revealed a lowered number of CD16+ CD 56− fraction, which has the most potent NK cell activity. Single cell-in-agarose assay, to investigate the binding and cytolytic activity of NK cell at the single cell level, revealed that the number of NK cells which bind to K562 cell was decreased, but that the cytolytic activity of the individual binding cell was relatively unaffected. A second supplementation of α-Toc for 8 weeks successfully restored NK cell activity, the number of cells expressing NK cell markers and the number of K562-binding cells as compared to the age-matched normal range. Conclusion These results indicate that severe vitamin E deficiency caused impaired NK cell activity due to a decrease in the number of CD16+ CD56− NK cells and that this abnormality is reversible with α-Toc supple‐mentation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050634
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  • 8
    Electronic Resource
    Electronic Resource
    Plasma concentrations of vitamin K1 and PIVKA-II in bottle-fed and breast-fed infants with and without vitamin K prophylaxis at birth (1988)
    Springer
    European journal of pediatrics 148 (1988), S. 139-142 
    Details
    ISSN: 1432-1076
    Keywords: Vitamin K deficiency ; Breast-fed infants ; Vitamin K prophylaxis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Plasma vitamin K1 and proteins induced by vitamin K absence (PIVKA) were assayed simultaneously 1–4 days and 29–35 days after delivery in three groups of infants: breast-fed not receiving vitamin K at birth (n=12), bottle-fed without vitamin K administration at birth (n=7) and breast-fed receiving 1 mg vitamin K1 administered by intramuscular injection at birth (n=13). The bottle-fed infants had a significantly higher vitamin K1 plasma level than breast-fed infants who did not receive vitamin K1 at birth. Extremely high levels of vitamin K were obtained 1–4 days after intramuscular administration. At the age of 1 month, breast-fed infants had the same plasma vitamin K1 concentration whether or not they had received vitamin K1 supplements. Decarboxy prothrombin (PIVKA-II) a reliable indicator of biochemical vitamin K deficiency, was found in 5 out of 12 breast-fed and in 2 out of 6 bottle-fed infants who had not received supplemental vitamin K1 after birth. In a separate study, we followed up to 90 days after birth a larger group if infants. PIVKA-II was found with significantly greater frequency in breast-fed infants receiving no vitamin K than in breast-fed infants receiving 1 mg vitamin K intramuscularly at birth, or in bottle-fed infants without extra vitamin K1. These data form a strong argument for routine vitamin K prophylaxis after birth for all breast-fed infants. The optimum dose and manner of administration require further study.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00445922
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  • 9
    Electronic Resource
    Electronic Resource
    Follow up study on children with dyslipidaemia detected by mass screening at 18 months of age: effect of 12 months dietary treatment (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 939-943 
    Details
    ISSN: 1432-1076
    Keywords: Screening ; Familial hypercholesterolaemia ; ApoB ; Diet therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The present study was done to evaluate the effect of short-term dietary therapy on 148 dyslipidaemic children (24 familial hypercholesterolaemia, 105 non-familial hypercholesterolaemia and 19 hypertriglyceridaemia), detected by mass screening in children at 18 months of age. In the model diet used for treatment, 15% of the total calories were obtained from protein, 27% from fat and 57% from carbohydrate. Cholesterol intake was set at 〈200 mg/day and the ratio of polyunsaturated to saturated fatty acid (P/S ratio) was 1.2. When compared to the composition of the diet ingested by the dyslipidaemic children, only the P/S ratio changed from 0.7 to 1.2. During 12 months treatment, levels of total cholesterol, low density lipoproteins cholesterol and apoB decreased by 10%–15% in children with familial and non-familial hypercholesterolaemia. There was no significant change in the levels of high density lipoproteins. In 19 children with hypertriglyceridaemia, the intake of carbohydrate was limited to 55% of the total calories consumed and after 12 months of treatment, triglyceride levels reverted to normal. Throughout the study period, apprimately 70% of the children on this dietary therapy were seen in our clinics every 3–6 months and physical development was within normal ranges. These results, taken together, indicate that dietary therapy can be effective for correcting dyslipidaemia, even in young children.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957537
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  • 10
    Electronic Resource
    Electronic Resource
    A family of selective immunodeficiency with normal immunoglobulins: possible autosomal dominant inheritance (1994)
    Springer
    European journal of pediatrics 153 (1994), S. 328-332 
    Details
    ISSN: 1432-1076
    Keywords: Specific antibody deficiency ; Autosomal dominant ; TSST-1 ; Toxic shock syndrome ; Staphylococcal infection
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report here our findings in two Japanese siblings who experienced recurrent bacterial and viral infections since early infancy. Recent symptoms included diarrhoea, conjunctivitis, rashes, headache, sore throat, joint pain, vomiting and vertigo, all similar to those seen in toxic shock syndrome, except for shock. These symptoms improved following gammaglobulin treatment.Staphylococcus aureus with coagulase type IV was continuously isolated from nasal smears producing toxic shock syndrome toxin-1 (TSST-1). Serum antibodies did not or only poorly responded to TSST-1, diphtheria toxoid, varicella virus and rubella virus, whereas total and subclass levels of serum immunoglobulin and in vitro DNA synthesis of lymphocytes stimulated by TSST-1,Staph. aureus, varicella vaccine and mitogens were normal. In the family, ten other members in three generations (five males: five females) including the mother had similar clinical symptoms. Thus, the disease may be inherited in an autosomal dominant fashion.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01956412
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