[Acute lymphoblastic leukemia after growth hormone substitution therapy].
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Adolescent↗
Biomedical subjects
A Otten
Publications and source records attributed to A Otten.
[How sensitive and specific is the determination of gliadin antibodies in the serum of patients with severe damage of small intestine mucosa?].
Serum antigliadin antibodies of the IgG- and IgA-class were investigated by an ELISA-test in healthy children, children suffering from coeliac disease and children with recurrent diarrheas. The sensitivity and specificity of positive antibody-titres for the diagnosis of coeliac disease were evaluated. The investigation of antigliadin antibodies proved to be a useful instrument for the diagnosis of acute coeliac disease as well as for the follow-up of such patients. The limitations of this method are pointed out.
Adolescent↗
Islet cell antibodies and viral infections.
The sera of 127 non-diabetic children after mumps-infection were investigated for the presence of islet cell antibodies and islet cell surface antibodies. The study also included 4 children who developed diabetes mellitus shortly after an active mumps vaccination. 21 of the non-diabetic children and four of the vaccinated children exhibited islet cell cytoplasmic antibodies. Islet cell surface antibodies were observed more frequently, namely in 43 out of 68 patients studied after mumps infection and in 32 out of 44 patients studied after different viral diseases. With one exception, none of the mumps-infected children and none of the other viral infected patients developed diabetes mellitus.
Antibodies, Viral↗
Islet cell antibodies, circulating immune complexes and antinuclear antibodies in diabetes mellitus.
Serum samples of patients suffering from diabetes mellitus were tested for complement-fixing and non complement-fixing islet cell antibodies, antinuclear antibodies and circulating immune complexes. There was no correlation between circulating immune complexes or antinuclear antibodies and secondary diabetic complications. A close relationship was found between the ICA titer and complement fixation of ICA. The incidence of ICA at the onset of the disease was higher in the patients under the age of 10 (85%) and decreased with increasing age up to 45% in patients with onset above age 20. In five patients being positive and four patients being negative for ICA at onset of disease, changes and fluctuations in antibody titers were observed over 38 months. Since manifestation of diabetes mellitus is believed to be an endpoint of a long lasting autoimmune process, our observations indicate that the autoimmune phenomena are merely indicators of ongoing autoimmune reactions not necessarily reflecting the state of autoaggression or islet cell destruction.
Adolescent↗
[Sonography of the thyroid gland in childhood].
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Adolescent↗
[Autoimmunity and viral infections in type-I diabetes mellitus].
Cytoplasmic islet-cell antibodies, insulin antibodies, islet-cell surface antibodies and islet-cell specific cytotoxicity were determined in serum of the following groups: 131 patients with type I diabetes, 19 with type II diabetes, 29 with mumps, 29 with enterovirus infections, 18 with measles and 28 healthy controls. Cytoplasmic islet-cell antibodies were found predominantly in type I diabetics. Islet-cell surface antibodies, on the other hand, were relatively frequently (60-80%) present in sera of both diabetics and patients with various virus infections. Islet-cell specific cytotoxicity in vitro was found not only in sera of diabetics, but also of patients with mumps or enterovirus infections. Sera of five patients with measles, however, had cytotoxic reactions comparable to those of the controls. These results suggest that cytotoxic antibody reactions against islet cells in vitro occur also in sera of non-diabetic patients. Under certain circumstances, infections which induce such immune reactions may be of significance in the pathogenesis of diabetes.
Adolescent↗
Islet cell antibodies and the development of diabetes mellitus in relation to mumps infection and mumps vaccination.
Islet cell antibodies were investigated in 127 non-diabetic children after mumps infection and in four out of seven children who developed diabetes mellitus shortly after active mumps vaccination. Twenty-one of the children who had mumps and all four vaccinated children who were tested had islet cell cytoplasmic antibodies. In contrast, islet cell surface antibodies were detected in 43 out of 68 patients with mumps infection and in 32 out of 44 patients with other viral diseases. All but one mumps-infected child and all the other viral infected patients investigated did not develop diabetes mellitus. The mumps-infected ICA positive children did not show those HLA-frequencies associated with Type 1 diabetes.
Antibodies↗
[Carnitine in the treatment of methylmalonic aciduria (MMA)].
Carnitine metabolism was studied and a therapeutic trial with L-carnitine was undertaken in 3 patients with methylmalonic aciduria. Prior to carnitine therapy, the concentration of free carnitine was diminished and the contribution of acylated carnitine to total carnitine was increased in both plasma and urine. During a metabolic crisis, in a patient the intravenous administration of L-carnitine greatly increased, the urinary excretion of acylcarnitine and the plasma concentration of methylmalonic acid fell. In all 3 patients, the chronic oral administration of L-carnitine resulted in the normalisation of the plasma free carnitine concentrations and an increased urinary excretion of carnitine esters. One patient clearly showed clinical improvement under carnitine therapy. The administration of L-carnitine to patients with methylmalonic aciduria results in an increased elimination of toxic propionyl groups and thus to a regeneration of intramitochondrial CoA. In conjunction with appropriate dietary measures, this may improve the metabolic situation of these patients.
Amino Acid Metabolism, Inborn Errors↗
[Diagnosis and therapy of candidiasis in the premature infant].
Extension of intensive care seems to have increased the risk of systemic candida infection. We report the incidence of systemic candida infection in 8 low-birth-weight infants (gestational age 27-32 weeks, birth weight 710-1,550 g). All infants required respiratory treatment. Various silastic catheters were inserted. Antibiotic therapy was started on the first day of life, usually a combination of ampicillin and gentamycin. Candida septicaemia was diagnosed at the age of 8-69 days of life based on blood and urine cultures, in two children at autopsy. There were no specific clinical symptoms in regard to candida infection. Sonographic technique revealed hydronephrosis in 3 infants due to candida mycelium. Antimycotic therapy included amphotericin B (dosage 0.44-1.0 mg/kg X day) and 5-fluorocytosine (80-100 mg/kg X day) as well as a monotherapy of 5-fluorocytosine (100-200 mg/kg X day). Four children were treated successfully. We like to advice a regular search for candida in urine, blood, tracheal secretion, stool and skin in low-birth-weight infants under intensive care conditions. If antimycotic therapy is started in time, therapy can be successful.
Anti-Bacterial Agents↗
Complications of mumps infection, islet-cell antibodies, and HLA.
To investigate whether the development of islet-cell antibodies (ICA) in the course of mumps infection is associated with a "diabetes-like" immunogenetic condition, 45 children with mumps complications as well as 56 children with insulin-dependent diabetes mellitus (IDDM) were typed for HLA ABC and DR antigens. ICA were detected in 14 out of 35 mumps patients. In the IDDM group, significant deviations from antigen frequencies of normal controls were observed for HLA Bw39, DR2, DR3, and DR4. In contrast, in ICA positive mumps patients, the frequency of these antigens was normal, but Aw24 was significantly increased. Thus, no immunogenetic similarities of both groups of patients could be detected.
Adolescent↗
[Hypophosphatemic rickets in premature infants weighing less than 1500 grams on oral and parenteral feeding].
The effect of mode of feeding on the development of rickets in very-low-birth-weight (VLBW) infants in spite of regular vitamin D supplementation was examined. Parenterally fed infants develop rickets more often than orally fed ones in spite of sufficient vitamin D supplementation. On total parenteral feeding hypophosphatemia was observed because phosphate supplementation was significantly lower than on oral feeding or in the intrauterine growing fetus of comparable gestational age. Adequate parenteral phosphate supplementation can prevent rickets.
Humans↗
Mumps, mumps vaccination, islet cell antibodies and the first manifestation of diabetes mellitus type I.
To connect mumps and diabetes mellitus in children is an old problem in medical literature. The typical occurrence of ICA at the onset of diabetes in children, as well as the incidence of ICA approximately 3 weeks after mumps infection support the hypothesis of a direct relationship between virus infection and diabetes. But the mumps infection alone is not the key factor. Mumps vaccination may not provide protection against diabetes mellitus, it may even provoke it. (Genetic determination, expressed by the HLA-phenotype in all the patients reported, does not allow a differentiation.)
Adolescent↗
[Calcium-phosphate metabolism in pre-term infants].
Physiological and clinical aspects are discussed in this review on calcium-phosphate metabolism in pre-term infants. Calcium accumulation in the bone mass of the foetus is related to the gestational age, and mainly occurs during the last weeks of gestation. Therefore, after birth, hypocalcemia is more frequent in pre-term than term infants. However, clinical symptoms of hypocalcemia, e.g. attacks of apnea, hyperexcitability and hypotonia, are rarely observed. Such symptoms depend upon the serum concentration of ionized calcium and this concentration is influenced by various metabolic factors. During the first two weeks of life phosphate is elevated in comparison to later periods. In spite of sufficient vitamin D supplementation low serum phosphate levels occur due to insufficient supply of phosphate. This correlates with evidence of rickets. An increased alkaline phosphatase activity can be considered an early and sensitive indicator. Pre-term infants develop rickets more frequently than term infants due to calcium-phosphate deficiency. Vitamin D supplementation alone is insufficient and should be combined with phosphate, as had been stated previously.
Alkaline Phosphatase↗
[Autoimmune phenomena in diabetes mellitus. On the pathogenetic and diagnostic significance].
The detection of islet cell antibodies lead to an increasing interest in autoimmune mechanisms in Typ I diabetes mellitus. Other phenomena such as insulitis in juvenile diabetics and in experimental animals, cellular immune reactions and concomitant antibodies against other endocrine organs, antinuclear antibodies and circulating immune complexes supported these suggestions. HLA-association and viral infections could be predisposing and inducing factors, although there are no clear correlations to any viral infection in a larger number of patients so far. For clinical and therapeutic purposes there are not enough sufficient criteria to demonstrate a pathologic autoimmune process in patients before developing diabetes. Up to now there is no realistic possibility and justification for starting an early immunosuppressive therapy.
Animals↗
Wolcott-Rallison syndrome: diabetes mellitus and spondyloepiphyseal dysplasia.
In 1972, Wolcott and Rallison described three siblings with a combination of infancy-onset diabetes mellitus and multiple epiphyseal dysplasia. We have observed a brother and sister with the same disorder. The chondro-osseous lesions are those of a spondylo-epiphyseal dysplasia. The diabetes mellitus is relatively mild. Histologic and electron microscopic studies of chondro-osseous tissue show findings similar to those in other epiphyseal and spondylo-epiphyseal dysplasias. In addition, however, atypical collagen-like fibres are found inside and outside chondrocytes. Collagen production seems to be normal in cultured fibroblasts. From the available data it appears that the association of characteristic chondro-osseous and endocrine abnormalities is non-random and that the lesions are independent manifestations of a pleiotropic gene. We propose to call this disorder the Wolcott-Rallison Syndrome.
Biopsy↗
A new high performance liquid chromatography (HPLC) method for the quantitation of strychnine in urine and tissue extracts.
A high performance liquid chromatography (HPLC) method was developed for the quantitation of strychnine in urine of children with nonketotic hyperglycinaemia and other developmental disorders treated with the alkaloid. Mobile and stationary phases were polar, i.e. methanol-water-330 g/kg ammonia (volumes, 85 ml + 14.2 ml + 0.8 ml) and LiChrosorb Si-60, 7 microns. Brucine was the internal standard. Extraction was performed by the Extrelut technique. At strychnine nitrate concentrations in urine of 21, 126, and 70 micrograms/l, recovery was 92.1 +/- 8.7, 98.1 +/- 2.7, and 102.5 +/- 2.7%. A child with nonketotic hyperglycinaemia under continued strychnine treatment excreted 1 to 13.6% of the daily dose unmetabolized in urine. The method was also suitable for the estimation of unreacted strychnine in tissue extracts. The fast disappearance in vitro of strychnine from a guinea pig liver preparation was confirmed.
Amino Acid Metabolism, Inborn Errors↗
Islet cell antibodies in children with mumps infection.
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Autoantibodies↗
Excretion of pterins in phenylketonuria and phenylketonuria variants.
Total urinary biopterin (B), neopterin (Ne) and monapterin (M) were measured in 25 healthy newborns, children and adults, in 49 patients with phenylketonuria (PKU) assumed to be deficient in phenylalanine-4-hydroxylase (PH), in 7 patients with dihydrobiopterin synthetase (DHBS) deficiency and in 4 patients with dihydropteridine reductase (DHPR) deficiency. Excretion of Ne based on creatinine (Ne/C) was 6.6 times higher in healthy newborns than in adults, suggesting a slow maturation of DHBS activity. Newborns excreted more Ne than B and adults more B than Ne (32 and 72% B of the sum of B + Ne, respectively). In all cases, excretion of M was 4-15% of that of Ne. PH deficient patients excreted more B and Ne than healthy controls and again, newborns more than older children. In individual patients, excretion of pterins correlated with phenylalanine (Phe) concentration in plasma; plasma Phe of different patients did not correlate well with excretion of pterins. In PKU variants with deficiency of tetrahydrobiopterin (BH4), extreme pterin patterns were observed: in DHBS- and DHPR-deficient patients, less than 3.5 and more than 81% B were found, respectively. All 30 samples from these patients investigated could be distinguished from those of PH-deficient patients and controls by a two-dimensional plot of % B versus B/C. Thus it seems likely that PKU variants due to BH4 deficiency could be detected early and differentiated by measurement of urinary B, Ne and C. This was exemplified already in one case. - In urine of patients with DHBS deficiency, high concentrations of 3'-hydroxysepiapterin were found in addition to Ne.
Adolescent↗