Teruo Kitagawa

Analysis of Blood Spot 17α‐Hydroxyprogesterone Concentration in Premature Infants—Proposal for Cut‐Off Limits in Screening for Congenital Adrenal Hyperplasia—

Blood Spot 17α‐hydroxyprogesterone (17‐OHP) concentrations in neonates, especially in premature babies, were determined in relation to 1) the gestational age at birth, 2) the equivalent age of gestation at blood sampling and 3) the birth weight. The 17‐OHP concentrations were found to be higher with prematurity. Accordingly, the cut‐off limit in screening for congenital adrenal hyperplasia (CAH) in premature infants is proposed as 20 ng/ml. Ideal cut‐off limits were set by the equivalent age of gestation at blood sampling. Cut‐off limits on the basis of gestational age at birth and birth weight are also suggested, where the sampling age is not so advanced. The rate of false positivity in premature infants can be reduced by this method.

A Comparative Study on the Epidemiology of IDDM Between Japan, Norway, Israel and the United States

Japn, Norway, Israel and the United States have studied have studied the epidemiology of IDDM in children by a similar method of data collection. Afterwards, the data were compared. Available data demonstrated that children in Norway, the United States and Israel were almost 20,15 and 5 times more likely respectively to develop IDDM than children in Japan. Although there was a samll difference in sex ratio, incidence in the smaller age group, and incidence of slowly progressing type IDDM between Japan and the other three countries, the other descriptive epidemiology of IDDM was quite similar, despite the large difference in the incidence and prevalence of IDDM.

Modulation of Immunological Abnormalities of Growth Hormone-Deficient Children by Growth Hormone Treatment

We studied the immunomodulatory role of growth hormone (GH) treatment in GH‐deficient children. CH potentiated natural killer (NK)cell activity and the PHA and Con‐A lymphocytoproliferative response. Two‐color fluorescence using monoclonal antibodies (CLM, 2H4, CD8 and CD11) showed a moderate reduction of the helper T cells and NK cells, but no changes of suppressor T cells or cytotoxic T‐cells during GH therapy. Cancer and slow viral infection in GH‐deficient children have been watched for carefully before and after GH therapy.

Height at onset of insulin-dependent diabetes mellitus in high- and low-risk countries*

The relative height at the onset of insulin-dependent diabetes mellitus was recorded in 340 cases from Japan and 191 cases from Pittsburgh, U.S.A. In Pittsburgh, the children diagnosed after puberty were shorter than normal whereas those diagnosed prior to puberty were much taller than normal. However, in Japan, there was no association between age at onset and relative height; at every age the newly diabetic children had similar relative heights to the non-diabetic population. This discrepancy may be related to the 18-fold incidence difference between the two countries.

SCREENING SYSTEM: Screening for Asymptomatic Hematuria and Proteinuria in School Children - Relationship between Clinical Laboratory Findings and Glomerular Pathology or Prognosis -

Early morning urine is collected at home and urinalysis is performed at a clinical laboratory. Simple dipstick methods are used for the detection of proteinuria and hematuria. When the urine tests are positive, a second test is performed in the same manner. When the urine abnormalities are persistently encountered, urine sediments are checked under the microscope and diagnostic steps are taken to determine the cause of the urine abnormalities. A study of the diagnosis and prognosis of the asymptomatic proteinuria and hematuria detected by urine screening was performed with the collaboration of eight different hospitals. Correlations between renal pathology or prognosis and urinary findings or other laboratory data in 170 patients who had renal biospy were studied. Cases with asymptomatic hematuria without proteinuria tended to have a better prognosis, with the pathology of minor abnormality or focal segmental proliferative lesions. In cases with marked proteinuria and hematuria diffuse proliferative lesions including membranoproliferative glomerulonephritis (MPGN) were frequently observed. Renal pathology suggesting a poor prognosis was not uncommon in the cases with marked proteinuria and hematuria. The above observations were confirmed by a 5‐year follow‐up study in 141 cases.

Neonatal Mass-Screening for Inborn Errors of Metabolism in Japan

This report describes the results of neonatal mass screening in Japan for PKU, maple syrup urine disease, histidinemia, homocystinuria, galactosemia, inborn errors of urea cycle metabolism and congenital hypothyroidism. The incidence of PKU is low but the incidence of histidinemia is somewhat high compared to that in the United States. Some genetic variants of each of these inborn errors of metabolism such as malignant hyperphenylalaninemia or mild form of MSUD have been detected in the neonatal screening. Clinical values of the screening for each of these in born errors of metabolism are discussed.

Analysis of Urinary and Circulating EDP Subfragments and Subunits: Application of the Western‐Blot Technique

We applied the Westem‐blot technique for qualifying fibrin/fibrinogen degradation products (FDP) subfragments and subunits. With this technique we examined urine or serum samples from patients with glomerulonephritis and disseminated intravascular coagulation (DIC), in order to observe how primary or secondary fibrinolysis functioned under these pathological conditions. We also tested the antigenic recognition of several FDP antibodies with this technique. The results obtained were as follows: 1) FDP in serum samples from patients with DIC consisted of both fibrinogenolytic and fibrinolytic products; 2) Fibrinolysis was predominant in the serum from one patient with HenochSchonlein purpura nephritis, and its level increased after fibrinolytic therapy with urokinase; 3) Fibrinogen and fibrin polymers were always the major components of urinary FDP, although fibrinolytic products such as subfragment D‐D dimer were detected in patients with glomerular disease and increased in acute exacerbation; 4) Anti‐FDP D‐D dimer monoclonal antibody (DD 3B6), which is usually considered to react specifically with cross‐linked fibrin derivatives, also seemed to have cross‐reactivity with nonnosslinked fibrin derivatives, (Acta Paediater Jpn 1989; 31: 127–135)

High‐Dose Gammaglobulin Therapy in Six Children with Newly Diagnosed Insulin‐Dependent Diabetes Mel I it us

We treated six newly diagnosed children with insulin‐dependent diabetes mellitus (IDDM) with high‐dose gammaglobulin therapy in a clinical trial of immunomodulation. Approximately 400 mg/kg/day of polyethylene glycol‐treated gammaglobulin was given by intravenous injection for four to five days.

Urinary Mass Screening for Child Health in Japan: FOREWORD

A program of urine screening for asymptomatic hematuria and proteinuria in school children has been conducted in Japan since 1973 by the Ministry of Education, Science and Culture with great success in the early detection of renal diseases. In Japan, the idea of urine screening in pediatrics and child health has recently extended not only to the detection of asymptomatic diabetes mellitus in school children but also to the detection of neuroblastoma in infancy. Suitable urine screening methods are now available for more than five chronic diseases and many more will follow.

Dietary Treatment of PKU Using A Low‐Phenylalanine Peptide Milk

A method of preparation of low phenylalanine (Phe) peptide fomiula for phenyketonuria (PKU) and the long term therapeutic effects of the milk in 6 PKU infants and one adult female patient aged 24 who wanted to become pregnant, are described. Low phenylalanine peptides (LPP) were prepared from whey protein. After hydrolysis by pronase (actinase), the hydrdysate was passed through a charcoal column to remove aromatic amino acids, and the free amino acids and salts were removed by passing through RD membranes to obtain LPP. Together with vegetable oils, lactose, dextrin, vitamins and minerals, as well as a small amount of amino acid mixture, low phenylalanine milk was madexs by freeze‐drying UP. The product (LPP milk), which does not have the bad taste and odor peculiar to amino acids, was administered to seven patients with PKU for one to three years. There were no differences between the LPP formula and conventional formulae collsisting of amino acids formula (AA formula), in reducing serum Phe levels in PKU patients. There were no adverse effects in patients who received LPP formula. UP formula can be used not only as milk, but also as an ingredient to make more palatable foods of low Phe content.