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  • 1
    Electronic Resource
    Electronic Resource
    Haemolytic uraemic syndrome and pulmonary hypertension in a patient with methionine synthase deficiency (1999)
    Springer
    European journal of pediatrics 158 (1999), S. 734-739 
    Details
    ISSN: 1432-1076
    Keywords: Key words Haemolytic uraemic syndrome ; Pulmonary hypertension ; Cobalamin ; Methylcobalamin ; Methionine synthase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An 18-month-old girl presented with macrocytic megaloblastic anaemia followed by haemolytic uraemic syndrome. Metabolic investigations led to the identification of an inborn error of cobalamin metabolism consisting of defective methylcobalamin biosynthesis, probably cobalamin G, since methionine synthase activity was decreased under standard reducing conditions. Despite treatment, pulmonary hypertension progressively developed and responded to oxygen therapy. Renal involvement evolved to terminal failure and haemodialysis, while pulmonary hypertension was controlled by oxygen therapy. Such clinical manifestations have never been reported in association with a defect of methylcobalamin and thus of methionine biosynthesis. Conclusion A congenital abnormality of cobalamin metabolism was suspected then confirmed in the presence of typical haematological features associated with unusual clinical manifestations such as progressive renal failure and pulmonary hypertension.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310051190
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  • 2
    Electronic Resource
    Electronic Resource
    Renal Granulomatous sarcoidosis in childhood: a report of 11 cases and a review of the literature (1999)
    Springer
    European journal of pediatrics 158 (1999), S. 154-159 
    Details
    ISSN: 1432-1076
    Keywords: Key words Sarcoidosis ; Child ; Granulomatous interstitial nephropathy ; Renal failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We analysed retrospectively 11 children with renal granulomatous sarcoidosis confirmed by renal histology in order to describe the course and prognosis of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10.1 years. Nine children had renal involvement at the time of diagnosis. In the course of the disease, nine patients developed renal failure and mild proteinuria, seven had transient sterile leucocyturia, four showed microscopic haematuria, seven had a urinary concentrating defect, and enlarged kidneys were seen in three patients. One child had hypercalcaemia and hypercalciuria, none had hypertension. Light microscopy of the kidney showed interstitial infiltration by mononuclear cells in all children, interstitial fibrosis in nine patients, epithelioid granulomas in seven, tubular involvement in eight, and mild glomerular involvement in seven patients. Renal immunofluorescence was negative. Ten children received prednisone for 1–11 years. After a mean follow up of 5.5 years, three patients had entered end-stage renal failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. Conclusion Renal failure, proteinuria, leucocyturia, haematuria, and concentration defect are the prominent features of renal granulomatous sarcoidosis in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310051038
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  • 3
    Electronic Resource
    Electronic Resource
    Peanut Agglutinin (1979)
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 9 (1979), S. 0 
    Details
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Peanut agglutinin (PNA) has previously been shown to be a ‘marker’ for early T subpopulations in mice. We have investigaied whether it could also be used in the study of human mononuclear cells. 50–60% of human thymocytes have binding sites for PNA. When separated on a discontinuous Ficoll gradient, the PNA-positive thymocytes are found preferentially in the layers corresponding to the immunoincompetent cells. In the peripheral blood only 5% of the mononuclear cells are PNA-positive and we have shown that these cells are monocytes. In tonsils 13% of PNA+ cells are found and they are mostly lymphocytes. Thus, PNA is a marker for some T cell subsets present in the thymus and tonsils.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-3083.1979.tb03067.x
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  • 4
    Electronic Resource
    Electronic Resource
    Activation of Human T Lymphocytes by Soluble Protein A (1985)
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 22 (1985), S. 0 
    Details
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The proliferative response of highly purified human peripheral blood or tonsil lymphocytes in the presence of soluble protein A (SpA) was investigated. SpA was shown to be a potent mitogen for T lymphocytes from peripheral blood or tonsils. Conversely, the non-T lymphocyte population from peripheral blood responded poorly to SpA, and SpA did not stimulate non-T lymphocytes from tonsils. The addition of mitomycin-treated T lymphocytes from peripheral blood enhanced the response of blood non-T lymphocytes to protein A. This effect was not found when non-T lymphocytes isolated from tonsils were cultured with mitomycin-treated T lymphocytes. The proliferate response of unseparated cells or purified lymphocyte populations was enhanced when adherent cells were added to the cultures. It is concluded that the soluble form of protein A activates mainly T lymphocytes and does not induce B lymphocyte proliferation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-3083.1985.tb01922.x
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  • 5
    Electronic Resource
    Electronic Resource
    Tumor cell line characterization of a malignant histiocytosis transplanted into nude mice (1982)
    Springer
    Virchows Archiv 397 (1982), S. 171-181 
    Details
    ISSN: 1432-2307
    Keywords: Malignant histiocytosis ; Nude mouse ; Histochemistry ; Membrane receptors ; Monoclonal antibodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The successful transplantation of a human malignant histiocytosis into nude mice allowed the examination of its atypical histiocytic cell proliferation. Histiocytic type cells were identified by positive reactions with acid phosphatase and non-specific esterase and with anti human DR or OKI1 antisera. Presence of OKT9 antigen and negative results obtained with most OKT antisera, rosettes, erythrophagocytosis and lysozyme corroborate the histiocytic immature state of the cells and preclude another type of tumor. All positive tests to prove a mature mononuclear phagocytic origin were attributable to the murine host cell reaction.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442387
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  • 6
    Electronic Resource
    Electronic Resource
    Immunological factors in organ transplantation (1989)
    Springer
    Intensive care medicine 15 (1989), S. S61 
    Details
    ISSN: 1432-1238
    Keywords: Organ transplantation ; Cyclosporin ; Monoclonal antibodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Several immunological factors affect the outcome of human kidney transplants. HLA-A,-B and-DR matching improves kidney graft survival rate, especially matching for HLA-DR antigens. The beneficial effect of pretransplant blood transfusion has been confirmed although the mechanisms of the beneficial effect are not clear. Donor specific transfusion prior to living related donor kidney transplantation improve graft survival but some 30% of potential recipients become sensitized to the donor during the transfusion process. Major improvements in the results of organ transplantation have been achieved during the past few years with the use of new immunosuppressive agents, namely cyclosporin and monoclonal antibodies reacting with T lymphocytes. Both agents act selectively on T lymphocytes. However, nephrotoxicity of cyclosporin may limit its use.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00260890
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  • 7
    Electronic Resource
    Electronic Resource
    Treatment of lupus nephritis in children (2000)
    Springer
    Pediatric nephrology 14 (2000), S. 158-166 
    Details
    ISSN: 1432-198X
    Keywords: Key words Lupus nephritis ; Pulse methylprednisolone ; Oral prednisone ; Cyclophosphamide ; Azathioprine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  In children, systemic lupus erythematosus (SLE) is often more severe than in adults. Renal disease is very common in SLE, with clinical symptoms of renal involvement occurring in 30%–70% of patients. In the absence of appropriate treatment the child may die from the disease or progress rapidly to renal failure. However, aggressive treatment regimens, in particular corticosteroids, carry the risk of growth retardation, accelerated atherosclerosis, and severe infectious complications. Lupus nephritis is classified into six groups depending on the severity of the histological lesions. The most-appropriate treatment for optimal efficacy with minimal side-effects depends on the disease severity. Mild lesions (class I or II) require only careful follow-up to identify any disease progression. Patients with class III nephropathy (focal and segmental glomerulonephritis) may have mild clinical symptoms, in which case no specific therapy is indicated, or more-severe symptoms of the nephrotic syndrome, hypertension, and sometimes moderate renal insufficiency. These patients require the same aggressive therapy as those with class IV disease (diffuse proliferative glomerulonephritis). Our current protocol starts with three methylprednisolone pulses followed by 1.5 mg/kg per day oral prednisone and six monthly pulses of cyclophosphamide. After a second renal biopsy the patient may be maintained on azathioprine while the prednisone dosage is slowly tapered. In children with milder disease we use lower doses of oral prednisone (1–1.5 mg/kg per day). Patients with membranous glomerulonephritis (class V) require no specific therapy if they have pure membranous nephropathy, but require aggressive therapy if they have the nephrotic syndrome. In those patients who progress to end-stage renal disease, clinical and serological remission is common and renal transplantation can be performed, as recurrence in the transplant is very rare.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050034
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  • 8
    Electronic Resource
    Electronic Resource
    Pretransplant blood transfusions with cyclosporine in pediatric renal transplantation (2000)
    Springer
    Pediatric nephrology 14 (2000), S. 451-456 
    Details
    ISSN: 1432-198X
    Keywords: Key words Blood transfusion ; Cyclosporine ; Renal transplantation ; Cytotoxic antibodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Pretransplant transfusions were repeatedly shown to be associated with improved graft survival in the ”pre-cyclosporine era,” and have recently been shown to be beneficial in patients on modern immunosuppressive regimes. In an attempt to improve this transfusion effect and minimize the potential development of cytotoxic antibodies, we have given these transfusions, with concomitant cyclosporine cover, prior to transplantation. Ninety-two renal transplantations were performed in 91 children in the study group (group 1) and all received pretransplant transfusions with cyclosporine cover. Results were compared with a preceding group of 102 children (104 transplantations) who had received pretransplant transfusions without cyclosporine cover (group 2). There were 70 cadaver and 22 living-related donor (LRD) transplants in group 1, and 88 cadaver and 16 LRD transplants in group 2. Graft survival rates (1- and 5-year) for cadaver transplantation were 96% and 90% in group 1 compared with 78% and 64% in group 2 (P=0.001). For LRD transplantation, these figures were 95% and 87% in group 1 and 81% and 69% in group 2. There was no difference between the two groups in terms of age at transplantation, sex, donor age, HLA-A, -B, -DR mismatches, or cold and warm ischemia times. All cadaver graft recipients received quadruple, sequential immunosuppression post transplant. However, 9 patients in group 1 were changed to tacrolimus for recurrent rejection episodes. No patient developed persistent lymphocytotoxic antibodies post transfusion or side effects of cyclosporine. Cyclosporine can be safely given with whole blood prior to transplantation with no adverse effect and no sensitization. Graft survival was significantly improved in this group of patients and graft loss due to rejection was exceptional. This effect should be further evaluated in prospective studies.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050791
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  • 9
    Electronic Resource
    Electronic Resource
    Haemolytic uraemic syndrome: prognostic factors in children over 3 years of age (1995)
    Springer
    Pediatric nephrology 9 (1995), S. 24-29 
    Details
    ISSN: 1432-198X
    Keywords: Haemolytic uraemic syndrome ; Prognosis ; Atypical form
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Previous studies have shown that age at onset of primary haemolytic uraemic syndrome (HUS) is a feature of prognostic significance, the disease being of much better outcome in paediatric patients younger than 3 years than in older children. In an attempt to find an explanation for such a difference, we analysed the clinical and pathological features of 42 children over 3 years of age who presented with HUS between 1955 and 1990 in our department. On the basis of the presence of a prodromal diarrhoea, we divided our patients into two groups: 21 children presented with the diarrhoea-associated (typical or D+) form of HUS, whereas 21 had the non-diarrhoea-associated (atypical or D-) form. Of the 42 children, 20 (47.5%) progressed to end-stage renal failure. However, our study shows that age at onset of HUS is not a prognostic feature per se. The difference in outcome between children and infants is most likely related to the high incidence of the atypical subset of HUS in children over 3 years, a subset that is very uncommon in infants. The ominous features which characterise this form of the disease are: (1) the absence of a diarrhoeal prodrome, (2) normal urine output, (3) marked proteinuria, (4) hypertension, (5) the occurrence of relapses or recurrences and (6) the presence of widespread and severe arteriolar changes on renal biopsy. The poor prognosis of the atypical form of HUS warrants the use of fresh-frozen plasma infusions and/or plasma exchange as early as possible in the course of the disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00858960
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  • 10
    Electronic Resource
    Electronic Resource
    Clinicopathological quiz (1987)
    Springer
    Pediatric nephrology 1 (1987), S. 245-247 
    Details
    ISSN: 1432-198X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00849299
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