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Biomedical subjects

M Rister

Publications and source records attributed to M Rister.

At least 19 recordsLinked to original sources
PubMed1993

[Clostridium difficile in early childhood ulcerative pancolitis].

Ulcerative colitis is a rare disease in young infants. Less than one per cent of cases occur during the first two years of life. We describe a male child who developed frequent bloody diarrhea at the age of 20 months. More common causes like infections or gastrointestinal food allergy were excluded. Endoscopy and histopathological evaluations revealed ulcerative colitis of the entire colon. Treatment with sulfasalazine and prednisone resulted in a clinical remission after seven weeks. The follow-up of 15 months was complicated by Rotavirus infection. Two relapses were caused by Clostridium difficile infections. The latter were successfully treated with oral vancomycine, but in the last relapse an increased dosage of prednisone was required, too. In relapses of inflammatory bowel disease gastrointestinal infections, especially caused by Clostridium difficile should be considered and treated adequately.

Biopsy↗
PubMed1992

Treatment of chronic neutropenia of childhood responsive to cyclosporin A in vitro and in vivo.

The clinical course of a 17-year-old patient who suffered from chronic neutropenia without cyclic variation since the age of 2 is presented. The bone marrow showed absent granulopoiesis and yielded very few colony-forming units (CFU-GM) in vitro with maturation up to segmented neutrophils. Incubation with cyclosporin A (CyA) increased CFU-GM markedly. Such an increase was not found after incubation of normal bone marrow with CyA in vitro. The patient also responded to CyA in vivo and maintained adequate granulocyte counts for 7 months when she became neutropenic again. She subsequently responded to high doses of prednisolone. The clinical course and bone marrow studies suggest that the defective granulopoiesis is due to an immunologically mediated mechanism sensitive to CyA and prednisolone. Other findings in this patient, such as impaired natural killer (NK) cell activity and random and chemotactic leukocyte motility, could further point to an imbalance in the regulation of hematopoiesis. Neutropenia, NK cell defect and impaired chemotaxis may be pathogenetically connected.

Adolescent↗
PubMed1990

Increase of CALLA-positive stimulated lymphoid cells in transient erythroblastopenia of childhood.

In 1986 and 1987 11 children with TEC (transient erythroblastopenia of childhood) were referred to our hospital. Bone marrow aspirations were performed to exclude haematological malignancy. There was a marked reduction of erythropoiesis in 9 cases (1%-8%), two children had already recovered (33% and 44% erythropoiesis). Eight patients exhibited high percentages of stimulated lymphoid cells. The subsequent immunotyping revealed the expression of CALLA (common acute lymphoblastic leukaemia antigen) on these cells but there was no other sign for malignancy. The patients recovered without any specific treatment except transfusions of packed red cells. Eight patients were followed up 11-18 months after initial presentation and were all found to be in good health. A prominent increase of CALLA-positive stimulated lymphoid cells has also been found in other haematological diseases such as neutropenia and immune thrombocytopenia. The expression of CALLA in bone marrow lymphocytes is a general reactive change to various alterations.

Antigens, Differentiation↗
PubMed1989

Bone marrow transplantation in childhood leukaemia--experience and strategies in the Federal Republic of Germany.

BMT has gained its place in the treatment of childhood leukaemia. Nevertheless, there are still many questions open. In acute lymphoblastic leukaemia children should normally be grafted in 2nd remission (CR). Some high risk cases, however, should probably be grafted in 1st CR. It is not clear whether children with late relapses benefit more from BMT than from renewed chemotherapy. Children with a relapse during maintenance therapy, however, have a better survival rate with BMT. In acute nonlymphoblastic leukaemia certain high risk patients should be grafted in 1st CR but it has still to be shown that BMT is superior to chemotherapy in such cases. It is not clear whether children with a relapse following intensive chemotherapy (such as the BFM-protocols) will benefit from BMT at all. In chronic myelocytic leukaemia, BMT in chronic phase should be performed. Thus, for the first time cure has become possible for this disease. Waiting for acceleration or even the occurrence of a blast crisis decreases the chance of survival after BMT dramatically. Since complications of BMT such as graft-versus-host reaction or severe infections are less frequent in children, relapses remain the main problem after BMT in childhood leukaemia.

Bone Marrow Transplantation↗
PubMed1989

Bone marrow transplantation in children with neuroblastoma.

Neuroblastoma is the third most frequent malignant tumor in childhood. One-third of the patients over one year of age at the time of diagnosis suffer from the disseminated form (stage IV). Despite highly aggressive chemotherapy survival rates are poor. One hundred and eighty-seven patients with neuroblastoma stage IV have been treated according to the German protocol NB 85. The probability of disease free survival is only 15% after 70 months. Treatment strategy in our protocol includes autologous and allogeneic bone marrow transplantation (BMT) for patients with stage IV (and greater than 1 year old). Twenty-two patients were grafted (7 allogeneic and 15 autologous). The conditioning regimen consisted mainly of high-dose melphalan (180 mg/m2) and total body irradiation (TBI) (3.4 Gy). Survival rates are discussed in the context of the chemotherapy protocol. Our own experience with autologous BMT is poor, despite of different purging methods. For this reason we decided to focus on allogeneic BMT. We have grafted five patients within the last 3 years. Three of them are alive and well, on died from veno-occlusive disease 70 days after BMT, and the remaining patient, grafted from a syngeneic donor, died from relapsing tumor. The main problem in neuroblastoma stage IV is resistance to chemotherapy. Intensification of the conditioning regimen or double autografting leads to a rate of toxic deaths close to 20% (Zucker, EBMT 1987) which is not tolerable. New improvements in the conditioning regimen have to be found to increase the effect of BMT.

Bone Marrow Transplantation↗
PubMed1989

Fractionated total body irradiation plus high-dose VP-16 prior to allogeneic bone marrow transplantation in children with poor risk acute leukaemias.

Nineteen children (median age, 13 years; range 4 to 18 years) with acute lymphoblastic leukemia/lymphoma (ALL) (10 patients) or acute nonlymphoblastic leukemia (ANNL) (9 patients) received allogeneic bone marrow transplants (BMT). Marrow was taken from HLA-identical sibling donors (16 patients) (pts), HLA-identical unrelated donor (1 pt), or one-antigen-missmatched sibling donor (1 pt). Preparatory regimen consisted of fractionated total body irradiation and high-dose VP-16 (50-70 mg/kg body weight). At the time of BMT nine of the pts were not in complete remission (CR): seven pts were refractory to aggressive multiagent chemotherapy and two pts were in first relapse. Six pts were in second CR, one pt in third CR; three pts grafted in first CR carried additional risk factors; e.g. induction failure. Ten out of the nineteen pts are alive and free of disease between one and 53 months (median, 28 months) after BMT. The actuarial disease-free survival rate is 37% for pts with ANLL and 54% for pts with ALL. Six pts have died from BMT-related complications. Only three pts (1 pt with ALL, 2 pts with ANLL) have relapsed between day +106 and day +134 after BMT and subsequently died. The four-year actuarial relapse rates of 29% for ANLL and 14% for ALL, respectively, demonstrate that the combination of fractionated total body irradiation and high-dose VP-16 is an effective antileucemic regimen for children with advanced leukemias.

Adolescent↗
PubMed1988

Fractionated total body irradiation and high-dose VP 16-213 followed by allogeneic bone marrow transplantation in advanced leukemias.

Thirty-eight patients (median age, 21 years) with acute nonlymphoblastic leukemia (ANLL) (17 patients), acute lymphoblastic leukemia/lymphoma (ALL) (18 patients), chronic myelogenous leukemia (two patients), and refractory anemia received allogeneic bone marrow transplants from HLA-identical sibling donors or a one-antigen-mismatched brother (one patient) after a preparatory regimen consisting of fractionated total body irradiation and high-dose VP 16-213 (60 to 70 mg/kg body weight). Of the 33 patients with acute leukemia who received grafts from HLA-identical donors, three patients with ANLL received transplants in first remission and one patient with standard-risk ALL received a graft while in second remission. All other patients were in more advanced stages of their disease or exhibited other high-risk features. At the time of analysis, 20 of the 33 patients were alive, with 19 of them remaining in continued complete remission for 6 to 35 months (median, 18 months). The 3-year actuarial disease-free survival rate of 56.6% +/- 9.7% (SE) and the actuarial relapse rate of 11.9% +/- 6.8% (SE) demonstrate that the combination of fractionated total body irradiation and high-dose VP 16 is an effective mode of therapy in patients with advanced leukemias. Preliminary experience cautions against the use of VP 16 instead of cyclophosphamide in any clinical situation carrying an increased risk of graft rejection because the immunosuppressive potency of VP 16 is largely untested but may be inferior to that of cyclophosphamide.

Adolescent↗
PubMed1987

Myeloperoxidase of polymorphonuclear leukocytes in psoriasis vulgaris.

The specific activity of myeloperoxidase (MPO), an enzyme located in the primary granules of polymorphonuclear leukocytes, was measured in patients with psoriasis vulgaris and compared with that in patients with atopic dermatitis and in healthy subjects. MPO catalyzes the oxidation of guaiacol into tetraguaiacol in the presence of H2O2. The activity was determined by photometric measurement of tetraguaiacol. The specific MPO activity showed no statistically significant difference between the healthy subjects and the patients with atopic dermatitis. In comparison to these two groups the specific MPO activity of the psoriatic patients showed a slight reduction which was, however, not statistically significant.

Dermatitis, Atopic↗
PubMed1987

[Isolated trisomy 21 in blasts of an acute lymphoblastic leukemia].

A two years and four months old boy without evidence of Down's syndrome exhibited a trisomy 21 in his acute lymphoblastic leucemic cells. During remission this chromosomal change disappeared. The cytogenetic examination of malignant cells serves an important tool in diagnosis and follow-up of acute leucemia as well as bone marrow transplantation.

Bone Marrow↗
PubMed1986

[Rhinocerebral mucormycosis as a complication of cytostatic therapy].

A twelve year old boy with acute lymphoblastic leukemia was treated with combination chemotherapy. Two weeks later a cellulitis of the left upper and lower eyelid appeared, followed by a rapidly increasing, deep necrotic inflammation of the periorbital area leading to proptosis. Mucormycosis was identified by histology and microbiology. The spreading inflammatory process was arrested by amphotericin B in a total dose of 1.2 g combined with a drainage of the necrotic tissue of the paranasal sinus, left orbit and bifrontal cerebral abscess. The patient is still in complete continuous remission four years later but a large defect of the nasal, maxillary and orbital area remains. This case demonstrates that the usual fatal outcome of mucormycosis in leukemia is curable with amphotericin B and aggressive surgery.

Amphotericin B↗
PubMed1985

Beta-glucuronidase release from leukocytes in children.

The release of beta-glucuronidase from polymorphonuclear leukocytes (PMNs) is important in the killing of bacteria and in producing tissue damage in acute inflammation. To investigate the effects of various diseases or drugs on degranulation, we studied the kinetics of beta-glucuronidase release from PMNs exposed to opsonized zymosan. PMNs of children with bacterial infections demonstrated increased degranulation. Within 5, 15, and 30 min the PMNs released 19 +/- 3%, 23 +/- 3%, and 26 +/- 3% of total beta-glucuronidase compared to 12 +/- 2%, 15 +/- 2%, and 16 +/- 2% of total beta-glucuronidase of control PMNs. Viral infections induced a significant delay of beta-glucuronidase release from PMNs. Maintenance therapy of acute lymphoblastic leukemia with 6-mercaptopurine and methotrexate, as well as administration of vincristine, diminished the degranulation. After 5, 15, and 30 min the PMNs released 8 +/- 1%, 10 +/- 1%, and 11 +/- 1%, as well as 6 +/- 3%, 8 +/- 2%, and 9 +/- 2% of total beta-glucuronidase. This study demonstrated that bacterial infections stimulate beta-glucuronidase release by PMNs. In contrast, cytostatic drugs inhibit lysosomal enzyme release, increasing the susceptibility to bacterial infections. The total enzyme activities were unchanged.

Adolescent↗
PubMed1985

Effect of hyperoxia on reduced glutathione in alveolar macrophages and polymorphonuclear leukocytes.

Reduced glutathione (GSH) protects alveolar macrophages (AMs) and polymorphonuclear leukocytes (PMNs) against oxidative damage. To obtain further knowledge of the oxygen toxicity we determined GSH in AMs and PMNs of guinea pigs exposed to an oxygen concentration of 85% for up to 90 h. AMs and PMNs from control animals contained 17.93 and 11.67 nmol GSH/mg protein, respectively. During the exposure to a FIO2 of 85% we observed a significant continuous increase of GSH in AMs. By 90 h of oxygen exposure, AMs contained 51.22 nmol GSH/mg protein. In addition, the protein content of AMs decreased during hyperoxia. In contrast, no change of the GSH amount and protein content was detectable in PMNs. The increase of GSH in AMs could serve as an adaptation of the cells to hyperoxia. The lack of the GSH increase in PMNs could be due to the different oxygen concentrations between the lung and the peritoneal cavity. The greater GSH content in AMs may account for the difference between these cells in their susceptibility to oxidant injury.

Animals↗
PubMed1985

Aplastic anaemia and the hypocellular myelodysplastic syndrome: histomorphological, diagnostic, and prognostic features.

In a retrospective study of 111 patients with aplastic anaemia iliac crest biopsies were evaluated for the presence of morphological features statistically related to the evolution of the disease. Prognostic variables for a transition to acute non-lymphatic leukaemia were: cellular atypias of the three haemopoietic lineages, as observed in the myelodysplastic syndrome, and especially "micromegakaryocytes"; high numbers or irregular distribution of megakaryocytes, or both; and (slight) marrow fibrosis. Clinical variables did not influence these prognostic correlations. Prognosis in relation to death from bone marrow failure without leukaemia might well have been influenced by a strong plasma cell reaction, but this correlation was weakened by clinical factors. On the basis of this study aplastic anaemia can thus be subdivided morphologically into two disease entities--namely, hypocellular myelodysplastic syndrome with a 23-82% risk of acute non-lymphatic leukaemia developing within three years, depending on how many variables associated with acute non-lymphatic leukaemia are present, and non-dysplastic myelohypoplasia.

Adolescent↗
PubMed1985

[Chemotaxis defect and recurrent E. coli meningitis].

The increased susceptibility to bacterial infections due to a decreased chemotaxis of neutrophil granulocytes was investigated in a premature small for gestational age baby, who such suffered from recurrent E. coli-meningitis. Causes as anatomic lesions, brain abscess, septic granuloumatosus disease and the Chédiak-Higashi-anomaly were ruled out. At the age of 9 and 13 weeks chemotaxis function compared to age-matched and adult controls was diminished. Addition of serum from healthy adults increased directed migration without reaching normal levels. The study of the complement status revealed a diminution of CH50, the alternate pathway components and C6-C9. Thus, decreased chemotaxis is believed to originate from a combined intrinsic-extrinsic defect. No relapses of infection were observed during prophylactic treatment with ascorbate and cotrimoxazole. Intrinsic as well as extrinsic chemotaxis became normal at the age of 7 months. Simultaneously, complement status was found to approach normal levels. The relevance of haematologic-immunologic evaluation in cases of relapsing severe bacterial infections in preterm newborns is discussed.

Chemotaxis, Leukocyte↗