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Biomedical subjects

M Wyss

Publications and source records attributed to M Wyss.

At least 91 records · Page 5Linked to original sources

[Transplantation of allogeneic bone marrow treated in vitro with Campath-1 monoclonal antibody].

5 patients underwent bone marrow transplantation for severe aplastic anemia (2) and acute leukemia (ALL) in first remission (3). Graft versus host disease prophylaxis was performed by depleting T lymphocytes in the donor bone marrow with the rat monoclonal Campath-1 and autologous complement. In addition, patients received cyclosporin A. Engraftment occurred normally in all 5 patients but 1 patient (SAA) had a late graft failure. Two patients suffered mild degrees of GvHD. All patients are currently in complete remission, one having undergone a second transplantation.

Adult↗

Lymphadenopathic form of Kaposi's sarcoma (KS) in an African child. No evidence for acquired immunodeficiency syndrome (AIDS).

A nine-year-old boy from the Central African Republic presented with an enlarged cervical lymph node. Biopsy revealed Kaposi's sarcoma. Lymphocyte subsets and reactivity to all antigens tested were normal, and the patient's serum contained no antibodies against LAV or HTLV3, the viruses presumably responsible for AIDS. We conclude that in this case, the lymphadenopathic form of Kaposi's sarcoma was not related to AIDS.

Antibodies, Viral↗

Cytogenetic study in a mentally retarded child with Bloom syndrome and acute lymphoblastic leukemia.

Bloom syndrome (BS) was diagnosed in a 7-year-old boy during hospitalization for acute lymphoblastic leukemia (ALL). The patient had most of the signs of BS along with some atypical manifestations: absence of telangiectases, obesity, and moderate mental retardation. Results of the cytogenetic studies were fully consistent with the diagnosis of BS: the occurrence of quadriradial figures and a very high incidence of sister-chromatid exchanges (SCE). This child's ALL was of non-B, non-T type with the presence, at the time of diagnosis, of a marrow clone including two markers. A Yq - chromosome was detected in about 10% of PHA-stimulated lymphocytes but neither in bone marrow cells nor in skin fibroblasts. This case is the fifth instance of ALL out of 104 registered cases of BS.

Bloom Syndrome↗

[Genetic factors linked to the HLA system conferring increased susceptibility to acute lymphoblastic leukemia in children].

Inheritance of HLA antigens in 55 families of patients with ALL was analyzed. Significantly increased sharing of DR antigens was observed among the parents of the affected children (p = 0.003). A similar increase was noted in the sharing of HLA-B antigens (p = 0.02). The observed number of DR homozygotes among the patients was twice the expected value in families where the parents shared a B and a DR antigen. Segregation analysis of the shared antigens disclosed significant prevalence of heterozygotes among the healthy siblings, which suggested the occurrence of gametic selection in such families. This study indicates that mating of certain shared alleles of the HLA system (especially of the DR locus) is associated with a risk for the offspring to develop ALL in childhood. Restricted heterogeneity of the parental HLA gene pool favours the expression of linked recessive genes and, presumably, of those involved in susceptibility to ALL.

Child↗

Acute lymphoblastic leukemia in two children with a congenital chromosome anomaly: familial inv(11)(p15q13) in one and ring chromosome No. 21 in the other.

A congenital chromosome abnormality was found in two unrelated children with acute lymphoblastic leukemia (ALL). In the first case, a pericentric inversion of chromosome No. 11, inv(11)(p15q13), was observed and discovered to be familial, being present in five other members of the family over two generations. In the second case, the presence of a congenital ring chromosome No. 21, 46,XX,r(21), was considered to be the result of a de novo mutation. The possible relation between these congenital chromosome anomalies and a predisposition to neoplasia is discussed and could be explained by different mechanisms: (1) amplification of oncogenic determinants by gene duplication, and/or (2) alteration of the effects of wildtype alleles through deletion or changes in position.

Bone Marrow↗

Evidence for HLA-linked susceptibility factors in childhood leukemia.

To test the hypothesis that susceptibility to leukemia can be governed by (a) recessive gene(s) associated with the major histocompatibility complex (MHC) in man, we performed an analysis of the inheritance of HLA antigens in 55 families in which one of the children developed ALL. We found among the parents of affected children a highly significant increased compatibility at the DR locus (p = 0.003). A similar increase was observed in sharing HLA antigens of the B locus (p = 0.02). The observed number of homozygotes among the patients was twice the expected value in families where the parents shared a B and a DR antigen. In segregation analysis, heterozygotes for the shared parental HLA antigen were significantly more prevalent among the healthy siblings. Our genetical analysis indicates that mating of certain shared alleles of the HLA system (especially of the DR locus) is associated with the risk for the offspring to develop ALL in childhood. This situation favors the expression of recessive genes associated with the MHC, and presumably those involved in the susceptibility to acute leukemia. Because familial leukemia is a rare event, the susceptibility to childhood ALL must also implicate genes outside the MHC and important environmental factors.

Child↗

In vitro efficacy of several antibiotics against intracellular S. aureus in chronic granulomatous disease.

We report the case of a one-year-old boy with chronic granulomatous disease (CGD), characterized by a defect in the polymorphonuclear leukocytes (PMN) which ingest, but do not kill catalase-positive bacteria such as S. aureus. This well-known observation led us to study the ability of clindamycin, rifampin, co-trimoxazole (TMP-SMX), methicillin and gentamicin to kill intracellular S. aureus in CGD and in normal PMN. Clindamycin killed S. aureus more effectively when these bacteria were intracellular than in the absence of PMN; rifampin was equally active on the same microorganisms in presence and absence of PMN, whereas TMP-SMX, methicillin and gentamicin were less effective in killing intracellular S. aureus. Similar results were obtained in CGD and in normal PMN. These results suggest that clindamycin and rifampin would be a reasonable choice in the treatment of staphylococcal infections in patients with CGD.

Anti-Bacterial Agents↗

[Chance of a second remission in acute juvenile lymphoblastic leukemia with favorable prognosis].

24 children with low risk acute lymphoblastic leukemia (ALL) in first relapse were re-treated with an aggressive protocol. Therapy of the first episode had adopted two different but equivalent approaches (SAKK-ALL "low risk" 76 and CALGB protocol 7611). With one exception, relapse occurred in all children before discontinuation of therapy. complete remission was achieved in 20 of the 24 children (83%). Five of 11 children in whom the length of the second remission could be evaluated had, at the time of the cutoff, been in continuous remission for 18 to 40 months. The therapy displayed considerable toxicity. From this study it is concluded that remission is achieved in the majority of children with first relapse of ALL, but that the remission can be maintained beyond 18 months only in a few children.

Adolescent↗

Haemostatic changes during open heart surgery with extracorporeal circulation and deep hypothermia in children.

The haemostatic status of twenty children with cyanotic and acyanotic cardiopathies was studied before, during and after cardiopulmonary bypass (CPB) under deep hypothermia and haemodilution. Eleven patients had various haemostatic troubles before surgery. Haemodilution with a crystalloid solution to an haematocrit of 21,8 vol. +/- 1,3% resulted in a severe lowering of all coagulation factors. Forced diuresis after CPB induced partial normalization. The observed alterations included moderate thrombocytopenia, prolongation of the prothrombin time, transient decrease of factors V and plasminogen, elevation of fibrin degradation products (FDP), significant lowering of factor VII-X, marked elevation of factor VIII and mild increase of fibrinogen. No correlation was found between coagulation abnormalities and postoperative bleeding, duration of CPB or type of cardiopathies. It is concluded that CPB with haemodilution proves as safe as more conventional approaches in respect to coagulant activities.

Adolescent↗

Low- and high-risk non-T and non-B and T-cell acute lymphoid leukemia (ALL) in childhood: different duration of remission and survival.

In a prospective, nonrandomized trial clinical (initial WBC and chest film) and immunological (surface immunoglobulin and rosetting with pretreated sheep red blood cells) criteria were used to stratify 69 children with previously untreated acute lymphoid leukemia (ALL). Forty of 61 evaluable patients had low-risk ALL (initial WBC less than or equal to 20,000/mm3, no mediastinal mass) and were treated less intensively. Twenty-one of 61 patients had high-risk ALL (initial WBC greater than 20,000/mm3 and/or mediastinal mass) and were treated more intensively. Of the high-risk patients 15 had non-T non-B and 6 T ALL. Sixty of 61 patients went into complete remission. After a median observation period of 27 months, 32 of 40 low-risk, 7 of 14 high-risk non-T non-B, and none of 6 high-risk T ALL patients were in continuous first remission. Thirty-six of 40 low-risk, 9 of 15 high-risk non-T non-B, and none of 6 T ALL patients were alive. Despite more intensive treatment, the duration of remission and the survival were significantly shorter in the high-risk than in the low-risk patients. Among the high-risk ALL, non-T non-B ALL did better than T ALL.

Adolescent↗

[Nephroblastoma and Wilms' tumor in hemihypertrophy. Presentation and discussion of a case].

The authors describe the case of a young girl with hemihypertrophy who developed a Wilms tumour in a kidney affected by nephroblastomatosis. The history and clinical findings are presented together with the histological results. The relationship between the congenital abnormality (hemihypertrophy), the dysgenesis (nephroblastomatosis) and the tumour is discussed.

Abnormalities, Multiple↗

Adjuvant chemotherapy with procarbazine, vincristine and prednisone for medulloblastomas. A preliminary report.

The survival of 20 children with medulloblastoma who received adjuvant chemotherapy with procarbazine, vincristine and prednisone after resection and craniospinal irradiation is compared with the preliminary results of the Children's Cancer Study Group (CCSG) and the international Society of Pediatric Oncology (SIOP) medulloblastoma studies. Survival with the chemotherapy used in the SPOG study ws not superior to the survival of children who received craniospinal irradiation only.

Cerebellar Neoplasms↗

[Hemoglobin D Punjab. Apropos of 2 families].

Two families who are carriers of hemoglobin DPunjab in the heterozygous state are described. This is the first time hemoglobin DPunjab has been reported in Switzerland. The propositus of the first family had two periods of acute hemolysis, probably drug-induced, which were followed by reversible renal complications and which led to the discovery of the hemoglobin. Study of the second family was undertaken because of anemia in one of its members.

Aged↗

[Malignat non-Hodgkin Lymphoma in childhood. Therapeutic results in 47 patients].

A preliminary report is presented on survival in 16 children with non-Hodgkin's lymphoma treated since 1975, as compared with that in 31 similar children treated between 1962 and 1974. In the former group, 10 of 14 children (71%) survived one or more years and 5 of 9 children (56%) two or more years with no evidence of disease. In the latter group, the corresponding survival rates were 26% and 19% respectively. This improvement is due to the introduction of an aggressive multidrug chemotherapy combined with radiotherapy, similar to the LSA2-L2 protocol. Considerable toxicity was observed with the new treatment. 23 of the 46 patients with diffuse non-Hodgkin's lymphoma had a Burkitt-type tumor. Treatment failures occurred mainly in children with a Burkitt-type tumor with primary intraabdominal localization.

Adolescent↗