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Biomedical subjects

Merja Möttönen

Publications and source records attributed to Merja Möttönen.

6 recordsLinked to original sources
PubMed2005

Treatment-related death in childhood acute lymphoblastic leukaemia in the Nordic countries: 1992-2001.

Despite continuously more successful treatment of childhood acute lymphoblastic leukaemia (ALL), 2-5% of children still die of other causes than relapse. The Nordic Society of Paediatric Haematology and Oncology-ALL92 protocol included 1652 patients < or =15 years of age with precursor B- and T-cell ALL diagnosed between 1992 and 2001. Induction deaths and deaths in first complete remission (CR1) were included in the study. A total of 56 deaths (3%) were identified: 19 died during induction (1%) and 37 in CR1 (2%). Infection was the major cause of death in 38 cases. Five patients died of early death before initiation of cytotoxic therapy. Five patients died because of toxicity of inner organs and one of accidental procedure failures. Seven patients died of complications following allogenic haematopoietic stem cell transplantation (HSCT) in CR1. Girls were at higher risk of treatment-related death (TRD) [relative risk (RR) = 2.2; 95% confidence interval (CI(95%)): 1.2-4.0, P < 0.01], mostly because of infections. Risk of TRD was also higher in children with Down syndrome (RR = 4.5; CI(95%): 2.0-10.2, P < 0.00). In conclusion, 3% of children with ALL died of TRD, with bacterial infections as the most common cause of death. Girls and Down syndrome patients had a higher risk of TRD. Infections still remain a major challenge in childhood ALL.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2005

[Gaucher disease].

Explore the source record for details and available documents.

Child Development↗
PubMed2004

Favorable outcome in 20-year follow-up of children with very-low-risk ALL and minimal standard therapy, with special reference to TEL-AML1 fusion.

BACKGROUND: As the treatment results of childhood ALL have improved, avoidance of late effects has become increasingly important. Identification of favorable prognostic factors helps to achieve this goal. PROCEDURE: We studied the prognostic value of TEL-AML1 fusion and a risk factor composed of age, white blood cell count (WBC), lymphomatous features, and hemoglobin level (Hb). We also compared outcome between two cohorts; cohort 1 (n=100) diagnosed 1975-1981, and cohort 2 (n=102) 1989-1991. Both cohorts were retrospectively divided in two groups: very-low-risk (WBC <10 x 10(9)/L, age 2 to <10 years, no lymphomatous features, Hb <90 g/L), and non-low-risk (=the remainder). We performed fluorescent in situ hybridization (FISH) of TEL-AML1 fusion of the marrow samples obtained at diagnosis. RESULTS: The median follow-up is 20 years in cohort 1, and 8 years in cohort 2. In both cohorts, the very-low-risk category comprised one-fourth of the children. TEL-AML1 fusion was more frequent in the very-low-risk (35%) than in the non-low-risk group (17%) (P=0.03). The 8-year event-free survival (EFS) of children with the fusion was better than of those without, 74 vs. 54% (P=0.040). The 8-year EFS in the very-low-risk group was 76% in cohort 1, and 79% in cohort 2 (n.s.). In the non-low-risk groups, EFS was 39 vs. 64% (P=0.02), respectively. CONCLUSIONS: Our data support the reported association of TEL-AML1 fusion with a favorable outcome although the risk group had a greater impact. These very-long-term follow-up data also indicate that children with very-low-risk ALL (slow disease) had a favorable outcome already in the late 1970s, and may be over treated with the contemporary ALL protocols.

Adolescent↗
PubMedDate not supplied

Ovarian granulosa cell tumors in childhood.

Granulosa cell tumors (GCT) of the ovary are prepubertal in 5% of the patients. In girls less than 20 years old, 80% of GCTs differ from those among adults. These juvenile granulosa cell tumors (JGCTs) are usually benign. GCTs belong to ovarian sex cord-stromal tumors, the more common ovarian tumors being epidermal and germinal. The etiology of GCT remains unknown. Most young children with GCT present with precorious pseudopuberty. Among adolescents GCT often causes menstrual irregularities, virilization, abdominal swelling, and pain. When JGCT is limited to the ovaries the outcome is excellent with only salpingo-oophorectomy. However, more widely spread tumors are difficult to treat and cause mortality. Cisplatin-containing chemotherapy can induce remissions in adult GCTs. Estrogens and peptide hormones, i.e., inhibin, are useful in the follow-up of the patients. The authors describe 3 children with GCT and review current data on this rare tumor from molecular biology to clinical aspects.

Child↗
PubMedDate not supplied

Mucosal pathology of the upper gastrointestinal tract associated with intensive chemotherapy in children: vitamin A supplements do not prevent lesions.

Intensive chemotherapy (ICT) for a malignant disease in children may be associated with clinically significant mucosal lesions of the upper gastrointestinal tract. This prospective and randomized study was conducted to evaluate more thoroughly the mucosal damage and to find out whether vitamin A therapy might prevent the development of these lesions. Gastroduodenoscopy and non-invasive methods were used to examine a consecutive series of 20 patients (10 females, 10 males, aged 1-15 years) 4 weeks after initiating the therapy regimen. Half of the patients were randomized to take vitamin A supplements for 6 weeks. During a follow-up of 6 weeks, 13 (65%) reported some symptoms of the gastrointestinal (GI) tract, diarrhea and mouth pain being most prominent. Endoscopic abnormalities were found in 13 (65 %) subjects: esophagitis in 10, erosive duodenitis in 8, and gastritis in 7. Histologically, 11 subjects had duodenitis, 5 had gastritis, 3 had eosinophilic esophagitis, and 2 had lymphocytic esophagitis. Both endoscopic and histological abnormalities of the duodenum showed a close relationship with long-term (more than 2 weeks) granulocytopenia. The 4 patients with the most extensive endoscopic abnormalities were treated with HCl inhibitors, and re-endoscopy performed 4-8 weeks later showed complete recovery. The sugar permeability values, measured as the lactulose/mannitol ratio were comparable to the values obtained in the controls, and lactose intolerance was found in only 3 (20%) of the 15 children able to perform the breath test. Both the incidence of the reported GI symptoms and the endoscopically or histologically observed GI lesions were similar in the subjects randomized to take vitamin A supplements as in the controls. In this study, two-thirds of children with ICT showed erosive mucosal lesions of the upper gastrointestinal tract, and vitamin A therapy failed to prevent them. Endoscopic examination is recommended if a patient has severe symptoms indicative of mucosal pathology.

Adolescent↗