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  • 1
    ISSN: 1432-1440
    Keywords: Diabetes mellitus (type I) ; Retinopathy ; Erythrocyte aggregation ; Deformability of erythrocytes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In vitro measurements were carried out to study the aggregation of erythrocytes in 33 children and young adolescents, three older adolescents, and 38 adults with type I diabetes. The aggregate formation of erythrocytes in stasis was increased in adult patients with both “good” and “poor” metabolic control when compared to control values. The aggregation of red cells in all children and the younger adolescents, both those under good and poor metabolic control, did not statistically differ from those of controls. Each of the three older adolescents showed an increased aggregate formation of their erythrocytes compared to controls. All adult patients under poor metabolic control exhibited various stages of diabetic retinopathy as shown by fluorescence angiography. Only one child under poor metabolic control showed an early stage of retinopathy (stage I according to Malone). The three older adolescents showed an increased aggregate formation of their erythrocytes. These patients exhibited also stage I of retinopathy. We suggest that the increased aggregation of erythrocytes of both adult and older adolescent patients may be one of the reasons for the development of diabetic retinopathy. Moreover, the normal erythrocyte aggregation of the diabetic children and younger adolescents could help to explain the rare occurrence of microangiopathies in childhood diabetes.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Acute lymphoblastic leukemia ; Hypothyroidism ; L-asparaginase ; Thyroxine binding globulin ; Thyroid hormones
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Recently it has been observed that L-asparaginase causes transient thyroxine binding globulin (TBG) deficiency in adults. In the present study we investigated the influence of L-asparaginase on the pituitary-thyroid axis and on the synthesis of TBG. 14 children with acute lymphoblastic leukemia were treated with a combination of L-asparaginase, vincristine, prednisone and daunomycin for remission induction. Thyroid function was monitored by measuring total T4, free T4, total T3, TSH and TBG with specific radioimmunoassays before, during and after treatment. Within 3 weeks of L-asparaginase therapy total T4 fell significantly from 10.7±1.6 to 2.9±1.8 μg/100 ml, free T4 from 1.77±0.4 to 0.94±0.35 ng/100 ml, total T3 from 0.99±0.23 to 0.35±0.2 ng/ml and TBG from 29.4±3.6 to 8.0±3.8 μg/ml. Basal TSH values tinuation of L-asparaginase, but following further treatment with other antileukemic agents, all values became normal within 2–4 weeks. In 6 patients with hypothyroid free T4 values TRH induced TSH release was totally blocked during L-asparaginase therapy. Our data clearly demonstrated that L-asparaginase caused a transient TBG deficiency. Total T4 and T3 were in the hypothyroid range because of low TBG concentrations. In addition to TBG deficiency transient, secondary hypothyroidism occurred in approximately 40–50% of all patients treated with L-asparaginase. These alterations were most likely caused by drug induced inhibition of protein synthesis. Under certain circumstances thyroid hormone replacement might be life-saving in severely ill patients suffering from transient, drug induced hypothyroidism.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Acute lymphoblastic leukemia ; Hypergonadotropic hypogonadism ; SHBG-deficiency ; Hyperprolatinaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Five pubertal boys (puberty stage Iv–V) and four prepubertal girls with acute lymphoblastic leukemia were treated with a combination of prednisone, vincristine, daunorubicin and l-asparaginase for remission induction. The hypothalamopituitary-gonadal axis was investigated by measuring basal plasma levels of LH, FSH, and PRL in both groups as well as the response to LHRH/TRH stimulation in pubertal boys before, on day 10, 21, and 28 during induction therapy and 23 days after the induction phase (day 51). Furthermore, the binding capacity of sex-hormone-binding globulin (SHBG), plasma levels of testosterone (T) and estradiol (E2) were monitored as well as the testicular volumes of the boys. Within three weeks of induction chemotherapy, plasma T, E2 and the binding capacity of SHBG decreased in both groups, together with a reduction of testicular volumes in the boys. Concommitantly, basal LH, FSH, and PRL values doubled with a normal gonadotrophin response to LHRH. The PRL response to TRH increased at the end of the induction phase, when chemotherapy with vincristine, daunorubicin and l-asparaginase was terminated, but prednisone treatment was continued for 7 another days. During the subsequent prophylactic irradiation of the central nervous system combined with other antileukemic drugs, all hormonal values including testicular volumes in pubertal boys became normal within a period of 3 weeks. Our data clearly demonstrate that an induction chemotherapy regimen such as that employed by the Berlin protocol leads to a transient castrating effect at the gonadal level and to a transient failure of synthesis of SHBG at the hepatic level. Increased prolactin values as well as an increased response to TRH may be related to a decrease in T indicating the existence of a negative feed-back-loop mechanism between T and PRL.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 148 (1988), S. 220-227 
    ISSN: 1432-1076
    Keywords: Noonan syndrome ; Growth ; Ullrich-Turner syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have analysed growth and the major clinical manifestations of 144 patients (89 males, 55 females) with Noonan syndrome from two West German centres. Size at birth was normal in both sexes. In both males and females, the mean height followed along the 3rd per centile until puberty, but decreased transiently due to an approximately 2 year delay in onset of puberty. Final height approaches the lower limits of normal at the end of the 2nd decade of life. The mean adult height was found to be (n=20) 162.5 cm in males and (n=13) 152.7 cm in females, respectively. Smoothed means and standard deviations for height were derived. These data may be used for the statistical evaluation of height of Noonan syndrome patients. Except for mental retardation and microcephaly, which are more frequent in males, the relative frequencies of minor anomalies and malformations were found to be similar in both sexes. The characteristic non-cyanotic heart defects in the Noonan Syndrome do not appear to have a major influence on growth. The auxological data were compared with those in the Ullrich-Turner syndrome.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 135 (1980), S. 97-99 
    ISSN: 1432-1076
    Keywords: Congenital goiter ; Congenital hypothyroidism ; Amniofetography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A newborn infant who presented with goitrous congenital hypothyroidism is described. Thyroid dysfunction was due to amniofetography performed 4 days before delivery, during which a total of 5.22 g of iodine as water- and lipid-soluble contrast medium was injected. After oral l-thyroxine treatment hypothyroidism disappeared rapidly. Thyroid function remained normal when treatment was withdrawn after 28 days, underlining the transient character of hypothyroidism.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 138 (1982), S. 82-83 
    ISSN: 1432-1076
    Keywords: Goiter ; Hypothyroidism ; Iodine ; Lipiodol ; Lymphography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Hypothyroid goiter is a rare but well recognized complication following long term administration of iodine containing expectorants and disinfectants in children. Only few reports exist on iodine-induced hypothyroidism after a single injection of the iodized radiopaque dye Lipiodol. A 15-year-old boy with previously normal thyroid function is described who developed hypothyroid goiter within six weeks following bipedal lymphography. Urinary iodine excretion was extremely elevated up to 18 mg/day while serum concentrations of total thyroxine were below the euthyroid range and thyrotropin levels were elevated. After oral L-thyroxine treatment the goiter disappeared. Thyroid function remained normal when treatment was discontinued after five months although iodine excretion was still 50 times higher (2.5 mg/day) than in normal age matched children. The observed alterations of the thyroid gland were caused by a long lasting Wolff-Chaikoff effect with a delayed adaptation to high iodide concentrations.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1076
    Keywords: Neurometabolic disease ; Organic aciduria ; 2-oxoglutarate dehydrogenase deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A boy and a girl born to a consanguineous Tunisian couple are suffering from a slowly progressive nervous disorder. Initially they both had normal psychomotor development with acquisition of gait and speech. First symptoms in the boy were athetoid movements during the second year of life. He later lost all motor and language skills and developed muscular rigidity and intention tremor. At the age of five years, he was completely bedridden while he appeared mentally much less affected. His younger sister followed a similar course. The major specific abnormality detected was a strikingly elevated excretion of 2-oxoglutaric acid, which was identified by gas liquid chromatography, mass spectrometry, and enzymatic analysis. 2-oxoglutarate dehydrogenase activity in homogenates of cultured skin fibroblasts was reduced to about 25% of control values in both children. Although the pathogenetic mechanisms leading to brain damage remain obscure, the finding strongly suggest an autosomal recessive neurometabolic disease with predominant involvement of the extrapyramidal system.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 140 (1983), S. 299-300 
    ISSN: 1432-1076
    Keywords: Hypothyroidism ; Iodine ; Neonatal screening
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Transient hyperthyrotropinemia and/or hypothyroidism have been found in many newborn infants during thyroid screening programs. In Europe the most likely causes are iodine deficiency and iodine overload. Because of the high incidence of transient hyperthyrotropinemia in Berlin we measured iodine concentrations in casual urine samples of newborns with TSH elevations. Urine and blood samples were collected on the 5th day of life. In the prospective study 99 out of 9320 newborns (1.06%) displayed TSH concentrations ranging from 20 to 152 μU/ml. All infants had normal TSH levels during a control examination. The urinary iodine concentrations were significantly elevated in 76 out of the 99 newborns. Most of the patients were born in obstetric departments where iodine-containing antiseptic agents were routinely used for disinfection during labor. The use of iodine-containing antiseptic agents not only results in unnecessary control determinations for the thyroid screening program but also causes an undesirable metabolic situtation that may be a potential hazard for the development of the central nervous system.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1076
    Keywords: Tall stature ; Oestrogen treatment ; Dose dependency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The effects of different doses of oestrogens in constitutionally tall girls were evaluated in two centres for paediatric endocrinology. In one centre, 38 girls were treated with a high oestrogen dose of 0.3 to 0.5 mg ethinyloestradiol (EE) daily. In the other, 44 girls received a comparably low dose of 0.1 mg EE per day. Height prediction (HP), chronological age (CA), and height at the onset of treatment were comparable in both groups. Although the duration of treatment was significantly longer in those receiving the low dose, the cumulative oestrogen dose was still significantly lower. The dose of EE had no effect on final height reduction (high dose group: 4.9±2.6 cm, low dose group: 5.1±2.4 cm). Final height was more reduced in both groups when treatment was started at an early bone age (BA) (≤13 years). No serious side effects were observed in either group, however weight gain was more pronounced in girls receiving the higher dose. We conclude that treatment of constitutionally tall girls with low doses of oestrogens is equally effective in reducing the final height as the usually administered high doses. The lowest effective dose has to be determined in a randomized, prospective clinical trial.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 135 (1996), S. 0 
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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