PubMed Health⌕ Search

Biomedical subjects

H Perrimond

Publications and source records attributed to H Perrimond.

At least 37 records · Page 2Linked to original sources

[The value of nuclear magnetic resonance in the study of iron overload in thalassemia patients].

In patients with thalassemia treated by long-term chronic blood transfusions who survive beyond the first ten or twenty years of life but received no or inadequate chelating therapy during the first years, evaluation of iron overload and its consequences on tissues may prove an arduous task. MRI is a non-invasive means of measuring the amount of iron in the liver and the consequences of the iron overload on the heart and other tissues. For this purpose, MRI is more satisfactory than CT scan studies. In this investigation, 20 patients with thalassemia major underwent MRI. Multiple spin echos were used to allow determination of the transversal relaxation constant T2. This constant, expressed in ms, is related to the concentration of iron in the liver as in the following expression: (C) = 5 410/T2-110. MRI studies disclosed a discrepancy between the severity of hepatic hemosiderosis and development of decompensated iron overload cardiomyopathy. In a unique case, in which a heart transplant and two MRI studies were performed, the severe iron overload that failed to respond to several years of subcutaneous chelating therapy was more than halved by intensive intravenous chelation through a central catheter. MRI studies of the heart provide valuable morphologic and functional data. Although the amount of iron in the myocardium cannot as yet be quantified, modifications of the transversal relaxation time provide information on the severity of the overload and the presence of other myocardial alterations.

Adolescent↗

[Yersinia infection and hemoglobin disorder. Apropos of 4 cases].

The authors report 4 cases of Yersinia enterocolitica (Ye) infection in patients with hemoglobin disorders (3 beta thalassemia major, 1 sickle cell anemia). Patients with hemoglobin disorders are particularly vulnerable to Ye infection. Predisposing factors consist mainly of iron overload and deferoxamine therapy; superinfection with HIV may be an additional factor. The diagnosis is performed by identifying patients at risk and by bacteriologic tests (blood and stool cultures, antibody titers). Therapy includes discontinuation of deferoxamine and initiation of an adequate antibiotic therapy.

Anemia, Sickle Cell↗

[Acute lymphoblastic leukemia in children. Results of a protocol including bone marrow transplantation during the first remission in high-risk forms for relapse].

Ninety-two previously untreated children with ALL were admitted to the same institution between November 1984 and November 1988. According to early prognostic factors, patients were divided into 3 groups: group 1 at "low risk" for relapse (n = 18), group 2 at "intermediate risk" (n = 62) and group 3 at "high risk" (n = 12). Every patient received an 8 week-long induction chemotherapy; after CNS prophylaxis, groups 1 and 2 children received a consolidation chemotherapy and then a classical maintenance treatment. Group 3 patients were selected to receive a bone-marrow transplantation during their first remission because of the presence, at diagnosis, of at least one of the following criteria: hyperleukocytosis greater than 100,000 (7 cases), translocation t(1;19) and t(4;11) (2 cases), adolescents (2 cases), no remission at day 30 (2 cases). Ninety-one of 92 children achieved a complete remission and none died during induction therapy. Probability of leukemia-free survival at 4 years is 73 +/- 7% for the whole patient population and 95%, 71% and 60% for patients of groups 1, 2, and 3 respectively. Persistence or disappearance of leukaemic cells in bone marrow after the initial 15 days of chemotherapy appears to influence the probability of a leukemia-free survival.

Actuarial Analysis↗

Hb Bruxelles: alpha 2A beta (2)41 or 42(C7 or CD1)Phe deleted.

Hb Bruxelles is a new beta-globin variant producing severe congenital Heinz body anemia. It results from the deletion of one of the two adjacent phenylalanines, beta 41 or beta 42, presumably by frameshift mutagenesis. Its whole blood oxygen affinity is significantly lowered.

Amino Acid Sequence↗

Repeated courses of high dose melphalan and unpurged autologous bone marrow transplantation in children with acute non-lymphoblastic leukemia in first complete remission.

Eleven children between the ages of 1 and 16 years with acute non-lymphoblastic leukemia (ANLL) in first remission were included in a study of double unpurged autologous bone marrow transplantation (ABMT). Prior to each ABMT patients received massive chemotherapy with melphalan at a dosage of 140 mg/m2. The first ABMT was done within a median of 4 months after the achievement of complete remission. As soon as the children had adequate hematologic recovery, a second marrow collection was done, followed by a second course of melphalan and a second ABMT. The duration of aplasia was significantly longer after the second ABMT than after the first, but the non-hematologic toxicity was relatively mild in each case and no patient died from the procedure. Four patients relapsed and seven are alive in unmaintained complete remission with a median duration of leukemia-free survival of 29 months (range 15-56 months) after the first ABMT. These data demonstrate the feasibility of repeating ABMT after melphalan in children with ANLL. The eventual impact of such therapy needs to be demonstrated in prospective randomized studies.

Adolescent↗

Poor prognosis of acute lymphoblastic leukemia with translocation (1;19) in childhood: potential interest of allogeneic bone marrow transplantation.

We report a new case of childhood acute lymphoblastic leukemia with translocation (1;19). At the time of diagnosis, the only adverse prognosis factor was the existence of this translocation. Under conventional chemotherapy, the girl experienced early marrow relapse (duration of first remission was 2 months). She received allogeneic bone marrow transplantation during the second remission and is alive in continuous complete remission 20 months after transplant. Several earlier reports have suggested that children with the (1;19) have a poor prognosis; If this poor response to conventional therapy is confirmed, an allogeneic bone marrow transplantation should be considered during the first remission.

Bone Marrow Transplantation↗

[Syndrome of activation of the mononuclear phagocyte system. Initial manifestation of visceral leishmaniasis].

We report a case of mononuclear phagocyte system activation observed in a 12 month-old girl presenting with fever, hepatosplenomegaly, pancytopenia, histiocytic hyperplasia with hemophagocytosis in the bone marrow, high triglyceride and low fibrinogen blood levels. This syndrome was associated with visceral leishmaniasis. We conclude that systemic leishmaniasis is a curable cause of disorders characterized by an activation of the mononuclear phagocyte system.

Animals↗

Severe erosive lichen planus and polycythemia vera in an adolescent.

Erosive lichen planus developed in a 12-year-old adolescent also presenting polycythemia vera (Vaquez' disease). Though a casual association cannot be excluded, the rarity of the two diseases in children suggests a pathogenetic link, possibly involving an abnormal hematologic stem cell line.

Biopsy↗

[Allogeneic bone marrow transplantation in the treatment of acute leukemias of children. Study of 26 patients].

Twenty-six children with acute leukemia were treated with allogeneic marrow transplantation from HLA identical siblings after a conditioning regimen with Cyclophosphamide-total body irradiation (19 patients), Melphalan-total body irradiation (6 patients) or Busulfan-cyclophosphamide (1 patient). Eighteen were transplanted in complete remission (4 with acute non lymphoblastic leukemia in first remission, 14 with acute lymphoblastic leukemia: 6 in first, 6 in second and 2 in subsequent remission): 2 died of cytomegalovirus pneumoniae, 1 relapsed and 15 survive in continuous complete remission from 5 to 42 months after transplantation (median = 22 months). Eight were transplanted in relapse, 7 achieve complete remission, 5 of them relapsed, 1 died of G.V.H. and 1 survives in continuous complete remission 46 months after transplantation. Actuarial analysis shows a disease free survival rate at 3 years of 82% for patients transplanted in remission and 12% for patients transplanted in relapse (p less than 0.01).

Acute Disease↗

[Registry of cancers in children in the regions of Provence, the Alps, Côte d'Azur and Corsica. Setting up and evaluation of the year 1984].

Epidemiology is a promising clinical way for research on cancer. A population based pediatric cancer registry was opened since January 1984 in 2 regions of south-eastern France. Its purpose is to analyse childhood cancer incidence and possible variations. A case-control study on risk factors was set up at the same time. Registration procedures and 1984 data are reported. The observed incidence is 11.86 new cancer cases/10(5) children/year.

Adolescent↗