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  • 1
    ISSN: 1432-1076
    Keywords: Human CFU-E ; Human CFU-C ; Bone marrow culture ; Erythropoiesis ; Granulopoiesis ; Renal anaemia ; Uraemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Anaemia is a frequent symptom in children with chronic uraemia. There is only limited information about committed haemopoietic stem-cell function in renal insufficiency. The ability of bone-marrow cells to form erythropoietic and granulopoietic cell colonies in vitro was therefore tested in 13 children with chronic renal insufficiency. Following separation of mononuclear cells from bone-marrow aspirates by Ficoll-Isopaque gradient centrifugation, erythropoietic precursor cells were stimulated in plasma clots by erythropoietin. Granulopoietic precursor cells were stimulated in soft-agar gel using feeder layers of normal human leukocytes. The colonies were identified by staining and counted. The number and proliferative capacity of erythropoietic precursor cells (CFU-E) did not seem to be significantly suppressed. Under in vitro conditions, the responsiveness of CFU-E to erythropoietin seemed to be normal. Addition of autologous serum resulted in different degrees of inhibition of erythroid colony formation. The inhibitory effect of the sera was also obvious when uraemic sera were added to bone-marrow cells from subjects without renal disease. Preliminary data indicate that haemodialysis is effective in reducing this inhibitory activity which may be an important factor in the pathogenesis of anaemia in chronic renal insufficiency. The numbers and proliferative capacity of granulopoietic precursor cells (CFU-C) were normal in all the children tested.
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  • 2
    ISSN: 1432-1076
    Keywords: Trace elements ; Haemodialysis ; Renal transplantation ; Atomic absorption spectroscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Whole blood levels of Cd and Pb (μg/g Hb), serum concentrations of Sr and Zn and their urinary excretion were measured in healthy, renal transplant and dialyzed children by atomic absorption spectroscopy. Furthermore, before and after dialysis the concentrations of these elements were determined in blood/serum as well as in the dialysis fluid. The predialytic concentrations of Cd, Pb and Sr in blood/serum were significantly increased compared to controls. Haemodialysis had only minor effects on whole blood Cd and Pb levels but serum Sr and Zn concentrations increased as a result of high metal levels in dialysis fluid. Children after renal transplantation showed no alterations in Pb, Sr and Zn concentrations compared to healthy children but whole blood Cd was increased. According to the renal function the Pb, Cd and Zn urinary excretion decreased in the following order: healthy, renal transplant and dialysis children. Sr urinary excretion was increased in haemodialysis and renal transplant patients. In conclusion, we found no Zn deficiency. Elevated blood levels of Cd and Pb and elevated Sr serum concentrations in paediatric patients undergoing dialysis and increased Cd whole blood levels in renal transplant children could be of clinical interest.
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  • 3
    ISSN: 1432-1076
    Keywords: Key words Latex allergy ; Spina bifida ; Fruit allergy ; Atopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Type 1 allergy against natural rubber latex is an increasing problem in health care workers and children with spina bifida or urogenital malformations. The aim of our study was to evaluate the prevalence of latex IgE antibodies and cross-reacting fruit antibodies in patients with spina bifida compared with atopic and non-atopic controls. Risk factors for sensitization should be determined. Sera of 148 patients with spina bifida and 98 controls (44 with atopy) were screened for IgE antibodies against latex, banana and kiwi by fluorescence enzyme immunoassay (CAP system). Atopies, allergic symptoms after latex contacts and the number of operations were compiled by a questionnaire. Patients with spina bifida developed latex IgE antibodies (≥0.7 kU/l) more frequently (40.5%) than atopic children (11.4%) or healthy controls (1.9%). All 18 symptomatic patients belonged to the spina bifida group and had high values of latex antibodies. The risk for developing latex antibodies increases with the number of operations. There was no difference in the history of atopic diseases and in a screening test of IgE antibodies against inhalative allergens between latex sensitized and not sensitized children with spina bifida. Antibodies against banana were more frequent in the latex sensitized children with spina bifida. (18.3% vs 3.4%, P = 0.002). Conclusion The high prevalence of latex antibodies in children with spina bifida justifies a primary prophylaxis by avoiding latex contacts, especially during anaesthesia and surgery, a correlation between the number of operations and the development of latex antibodies exists.
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  • 4
    ISSN: 1432-1076
    Keywords: Ulcerative Colitis in Childhood ; Immunosuppressive Therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Seit 1966 behandeln wir 22 Patienten mit Colitis ulcerosa. Davon wurden 15 schwere Erkrankungsfälle nach genau definierten Kriterien für die immunosuppressive Therapie ausgewählt. Komplikationen durch diese Behandlung wurden in 2 Fällen beobachtet. In den übrigen 13 Fällen führte die immunosuppressive Behandlung prompt zu klinischer und rectoskopischer Remission. Die mittels Kontrasteinlauf gewonnenen Röntgenbefunde waren in 2 Fällen völlig normalisiert, in 3 weiteren Fällen erheblich gebessert und in den restlichen 10 Fällen noch nicht wieder kontrolliert. Aus Gründen der Strahlenersparnis erfolgt die Röntgenkontrolle grundsätzlich am Ende der immunosuppressiven Behandlung, also frühestens 2 Jahre nach Behandlungsbeginn. Die Nebenwirkungen bei den beiden erwähnten Fällen bestanden in cholestatischem Ikterus (Fall Nr. 7) und heftigem Erbrechen (Fall Nr. 2). Die sonst bei cytostatischer Behandlung festzustellende Wachstumshemmung und auch Alopecie haben wir selbst bei langzeitig durchgeführter Behandlung nicht gesehen. Unsere Erfahrungen mit der immunosuppressiven Therapie der Colitis ulcerosa beim Kinde zusammenfassend möchten wir als durchaus ermutigend betrachten. Ihre Durchführung gehört jedoch in die Hand eines auf diesem Gebiete Erfahrenen.
    Notes: Abstract Since 1966 we treated 22 patients with ulcerative colitis, 15 of which with severe diseases were selected for immunosuppressive therapy according to strictly defined criteria. Difficulties were observed in 2 cases. In 13 cases immunosuppressive therapy promptly resulted in clinical and rectoscopic remissions. Roentgenologic findings of colon were normalized in 2 cases, improved in 3 cases and not yet controlled in the other cases. Side effects were noted in 2 patients, they included cholestatic jaundice and vomiting. Alopecia and decreased linear growth were not observed. In summary our experience with the immunosuppressive therapy of childhood ulcerative colitis so far has been encouraging.
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  • 5
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
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  • 6
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Williams Beuren-Syndrom ; Nierenarterienstenose ; Renovaskuläre Hypertonie ; Key words Williams-Beuren-syndrom ; Renovascular hypertension ; Renal artery stenosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Patients with Williams-Beuren-Syndrome characterized by the combination of typical morphology, supravalvular aortic stenosis and mental retardation. The typical cardiac malformation, the supravalvular aortic stenosis may be absent. Progressive peripheral vascular lesions as renal artery stenosis, coronary vascular abnormalities and cerebrovascular ischemia were not commonly observed in the past. Case report: We present a 12 year old girl with Williams-Beuren-Syndrome without typical signs of cardiac involvement who developed renovascular hypertension after 9 years of follow up due to stenosis of a renal artery. It was successfully treated by aortorenal bypass. Discussion: In the absence of typical cardiac involvement Patients with Williams-Beuren Syndrome can develop progressive vascular lesions as renal artery stenosis, coronary artery stenosis or cerebro-vascular lesions. Therefore irrespective of cardiac involvement patients with Willliams-Beuren syndrome should be followed up with special regards to this complication.
    Notes: Zusammenfassung Patienten mit Williams-Beuren-Syndrom werden durch die klassischen Symptome der auffälligen Morphologie des Gesichts, der supravalvulären Aortenstenose und der mentalen Retardierung beschrieben. Die klassische kardiale Beteiligung in Form der supravalvulären Aortenstenose kann bei Patienten mit Williams-Beuren-Syndrom fehlen. Unabhängig davon können progressive periphere Gefäßveränderungen bestehen. Diese können z. B. als Nierenarterienstenose, Koronaranomalie oder zerebrovaskuläre Ischämie symptomatisch werden. Fallbericht: Wir berichten über 1 Patientin mit Williams-Beuren-Syndrom ohne die kardiale Veränderung der supravalvulären Aortenstenose, die im Alter von 10 Jahren eine schwere arterielle Hypertonie infolge einer einseitigen Nierenarterienstenose entwickelte. Die Anlage eines aortorenoarterialen Bypasses war eine effektive Behandlung. Diskussion: Auch beim Fehlen der klassischen Symptomen des Williams-Beuren-Syndroms müssen progrediente Gefäßveränderungen rechtzeitig erkannt und als bedeutsame Komplikationen des Krankheitsverlaufs bedacht werden.
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Monatsschrift Kinderheilkunde 147 (1999), S. 911-911 
    ISSN: 1433-0474
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
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  • 8
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Carbamazepin ; Hypersensitivitätssyndrom ; Fieber ; Exanthem ; Lymphadenopathie ; Key words Carbamazepine ; Hypersensitivity syndrome ; Fever ; Skin rash ; Lymphadenopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary 2 weeks after initiation of carbamazepine therapy a 3 year old boy presented with skin rash, fever, lymphadenopathy, hepatosplenomegaly and nephritis. After exclusion of infectious diseases our differential diagnosis focussed on carbamazepine induced hypersensitivity syndrome. We found a positive lymphocyte transformation assay and patch test to carbamazepine, indicating a specific response, but no anti-carbamazepine antibody, no circulating immune complexes and no decrease in complement components. The hypersensitivity syndrome refers to a severe hypersensitivity response due to a idiosyncratic reaction. The origin is unknown. The similarity to other multisystem disorders is discussed. Discussion: Not only aromatic antiepileptic agents (carbamazepine, phenobarbital, phenytoin) sulfonamides but as well allopurinol and gold salts are associated with the syndrome. Withdrawal of the drug and treatment with corticosteroids usually lead to complete recovery.
    Notes: Zusammenfassung Wir berichten über einen 3 jährigen Jungen, der 2 Wochen nach Beginn einer antiepileptischen Behandlung an einem Carbamazepin-induzierten Hypersensitivitätssyndrom, bestehend aus therapieresistentem Fieber, polymorphem Exanthem, oropharyngealen Läsionen, generalisierter Lymphadenopathie, Hepatosplenomegalie und Nephritis, erkrankte. Wir fanden positive Lymphozytenstimulations- und Epikutantests auf Carbamazepin als Ausdruck einer spezifischen Überempfindlichkeit. Spezifische Anti-Carbamazepin-Antikörper, zirkulierende Immunkomplexe oder eine Komplementerniedrigung waren nicht nachweisbar. Es handelt sich um eine Überempfindlichkeitsreaktion unbekannter Ätiologie im Sinn einer Idiosynkrasie. Nach Ausschluß infektiöser Ursachen wird die Differentialdiagnose zu anderen Systemerkrankungen erörtert. Diskussion: Neben aromatischen Antikonvulsiva (Carbamazepin, Phenobarbital, Phenytoin) sind Sulfonamide und Goldsalze mögliche Auslöser eines Hypersensitivitätssyndroms. Absetzen des Induktors und systemische Kortikosteroide führen in der Regel zur vollständigen Remission.
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  • 9
    ISSN: 1432-0584
    Keywords: Asialo-glycophorin ; Asialo-erythropoietin-activation in vivo
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In vitro active asialo-erythropoietin has no effect on hemesynthesis in vivo. Asialo-glycophorin induces a very low 59Fe-uptake rate in heme in exhypoxic polycythemic mice. The combination of asialo-erythropoietin and asialo-glycophorin or asialo-fetuin induced an activation comparable to the activation by native erythropoietin. Obviously the combination of asialo-erythropoietin and tested glycoproteins influence the activity of erythropoiesis-stimulating capacity of asialo-erythropoietin.
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  • 10
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Allergy 53 (1998), S. 0 
    ISSN: 1398-9995
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background The effect of latex prophylaxis has not been investigated in spina bifida children, a high-risk group for latex allergy. As repeated operations have been identified as a major cause of latex sensitization, we wanted to find out whether primary latex prophylaxis during surgery could prevent latex allergy in children with spina bifida. Methods In December 1995, we established latex-free surgery and anesthesia for all patients with spina bifida regardless of their sensitization to latex. Twelve children born after that date (mean age 1.2 years, mean ntmiber of operations 3.3, range 1 -7) were tested for specific IgE against latex until December 1997 (ImmunoCap, Pharmacia, Uppsala, Sweden) and compared with eight children born before December 1995 (mean age 1.3 years, mean number of operations 3.6, range 1–8), in whom a test for latex IgE had been done before the age of 2 years. Results Before we established primary prophylaxis, three of seven children with spina bifida (38%) were sensitized to latex until the age of 2 years. After the establishment of a latex-free operating theater for spina bifida patients, none of the 12 patients were sensitized to latex despite up to seven operations in each child. Conclusions Primary latex prophylaxis during surgery can prevent latex sensitization in young spina bifida patients.
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