Analysis of treatment outcome for children with recurrent or metastatic hepatoblastoma

For better total survival rate of children with hepatoblastoma, the therapeutic strategy for recurrent or metastatic hepatoblastoma should be improved. From 1991 to 1999, 134 cases of hepatoblastoma were treated by surgery and combination chemotherapy of cisplatin (CDDP) and THP-Adriamycin (THP-ADR) based on the JPLT-1 protocol. In 114 non-metastatic cases, 90 primary liver tumors were resected completely by partial hepatectomy, but 12 recurrences were observed in the liver (4 cases) and the lungs (8 cases). Distant metastases on the diagnosis were observed in 20 cases. The treatment outcome of these 12 recurrent and 20 metastatic tumors was analyzed. In four recurrent liver tumors, surgical resection was performed in all four cases, and all the patients were alive and well. In eight recurrent lung tumors, surgical resection was performed completely in six cases with unilateral lung disease, and five of the six patients were alive and well. In stage IV tumors, the survival rate of the patients having primary tumors within two hepatic sections was significantly higher than that of the patients having primary tumors over three hepatic sections. Active surgical intervention to lung metastases and a more intensive chemotherapy to facilitate complete resection of primary hepatic tumor could improve survival rate of children with refractory hepatoblastoma.     

Treatment combined with bone marrow transplantation for advanced neuroblastoma: An analysis of patients who were pretreated intensively with the protocol of the study group of Japan

One hundred and ten patients with advanced neuroblastoma were treated with the protocol of the Study Group of Japan between January 1985 and March 1991. Patients received six cyclic courses of regimen A₁, consisting of cyclophosphamide (1,200 mg/m²), vincristine (1.5 mg/m²), tetrahydropyranyl adriamycin (40 mg/m²), and cisplatin (90 mg/m²). Primary tumors and regional lymph node metastases were removed some time during the first six cycles of regimen A₁. After six cycles of A₁, the patients were divided into three groups. Patients in group 1 received alternating treatment with regimen B (cyclophosphamide and ACNU) and intensified A₁, and those in group 2 were treated with alternating administration of regimen C (cyclophosphamide and DTIC) and intensified A₁. Patients in group 3 were treated with supralethal therapy and bone marrow transplantation (BMT). Event-free survival rates at five years were 38.8% in the chemotherapy group (groups 1 and 2) and 50.0% in the transplant group (group 3). Because of the study design that was not in truly randomized fashion and because of the small number of patients in each risk group, it is indicated, though not concluded, that the transplant group had a better prognosis than the chemotherapy group in the cases with stage III disease or with amplified N-myc oncogene, based on the statistical calculations. Differences in survival rates for patients who underwent BMT when complete remission (CR) was achieved and for those who achieved CR but who did not undergo marrow transplant were statistically insignificant. BMT-related death occurred in 3 of 31 cases (9.7%) undergoing marrow transplant, and the causes of the death included hemorrhagic pneumonia, myocardial disturbance and hemorrhagic uremia. © 1995 Wi1ey-Liss Inc.     

Comparison of Autologous and Allogeneic Bone Marrow Transplantation in Children with Acute Leukemia

Thirty-one patients with acute non-lymphocytic leukemia (18 patients) or with high-risk refractory acute lymphocytic leukemia (13 patients) underwent bone marrow transplantation between March 1980 and March 1990. The high-dose conditioning regimen employed included cyclophosphamide followed by fractionated total body irradiation (12 GY). Fourteen patients who had an HLA-identical sibling donor received allogeneic bone marrow transplantation (ailo-BMT); the other 17 patients received autologous bone marrow transplantation (auto-BMT) purged with 4-hydroperoxycyclophosphamide (4HC). Four of the 14 allo-graft recipients died of leukemic relapse and 2 others died of graft-versus-host disease. Three of the 17 auto-graft recipients died of relapse and 1 suffered relapse in the testes. The actuarial risk of relapse was 29% for the allo-BMT patients and 24% for the auto-BMT patients (P<0.05). The event-free survival rate at five years was 57% and 74% respectively (P<0.05). Although there was no difference between them, a trend toward a higher survival rate and a lower mortality and morbidity was observed in the auto-BMT group. These results suggest that autologous bone marrow transplantation purged with 4HC is an effective and useful treatment for children with acute non-lymphocytic and lymphocytic leukemia who have no HLA-identical donor.     

Osteochondroma after Total Body Irradiation in Bone Marrow Transplant Recipients: Report of Two Cases

We present two cases of osteochondroma after total body irradiationin bone marrow recipients, the first in a 6-year-old boy withjuvenile chronic myelogenous leukemia and the second in a 13-year-oldboy with acute myelogenous leukemia. The patients developedmultiple osteochondromas three years and seven years, respectively,after 12 Gy of total body irradiation. Neither had a familyhistory of hereditary multiple osteochondromatosis. A reviewof the English literature revealed only one report describingfive cases of osteochondroma after 12 Gy of total body irradiationin bone marrow transplant recipients. Osteochondroma shouldbe considered as an additional adverse effect of total bodyirradiation.     

Familial Aggregation of Cancer from Proband Cases with Childhood Adrenal Cortical Carcinoma1

Family pedigree of Li-Fraumeni syndrome was investigated from probands with childhood adrenocortical carcinoma in Japan. From 47 probands, 7 families had 3 or more cancer cases at ages less than 45 years within the first generation; one satisfied Li's original criteria, two were acceptable because of multiple primary cancer in the probands, and others showed an aggregation of cancers with onsets at early ages, though no sarcoma of mesenchymal origin was found. A significantly higher occurrence of cancer in the mothers of the probands, especially of the breast, was consistent with reports from the USA, and liver cancer, osteosarcoma and lung cancer among family members under the age of 45 also showed a higher frequency than in the general population. Similarities and differences between Japanese and Caucasian cases are discussed.     

Toxic polyneuropathy due to glue sniffing

Four cases of toxic polyneuropathy due to glue-sniffing were reported. Neurological examination revealed motor predominant mixed type polyneuropathy. The cause of polyneuropathies in these cases was considered to be due to the inhalation of the vaporized elements of the adhesive agent, which contain mainly n-hexane and toluene. N-hexane is considered to be chiefly responsible for the polyneuropathy, though toluene also could have some influence on the illness.     

Intravascular papillary endothelial hyperplasia in an aneurysm of the superficial temporal artery: Report of a case

We herein report a rare case of an intravascular papillary endothelial hyperplasia in an aneurysm of the superficial temporal artery. The patient was a 67-yearold Japanese woman. She noticed a throbbing swelling in her left forehead, which had gradually been increasing in size. She had no previous history of head trauma. Ultrasonography and three-dimensional computed tomographic angiography revealed an aneurysm with a mural thrombus measuring 10 mm in diameter fed by the frontal branch of the left superficial temporal artery. The aneurysm of the superficial temporal artery was dissected from the surrounding tissues, and was resected after ligation of feeding vessels. A microscopic examination revealed papillary endothelial hyperplasia in a true aneurysm. Nontraumatic aneurysms of the superficial temporal artery are rare. In the previous English literature, there have only been a few reports of papillary endothelial hyperplasia in an artery, and none in an aneurysm of the superficial temporal artery.     

Hematopoietic stem cell transplantation with conditioning regimens containing melphalan in pediatric patients with acute lymphoblastic leukemia

A multicenter comparative study was carried out to investigate the efficacy and safety of hematopoietic stem cell transplantation with conditioning regimens containing melphalan in pediatric patients with acute lymphoblastic leukemia. One hundred twenty three patients at a variety of remission stages were eligible for study participation. Eighty-nine were transplanted with allogeneic grafts and 34 patients with autologous grafts (23 cases with bone marrow and 11 cases with peripheral blood stem cells). Conditioning regimens used were as follows: (A) melphalan and busulfan for 40 patients, (B) melphalan, busulfan and TBI for 44 patients, (C) other regimens for 39 patients. To accelerate engraftment G-CSF (lenograstim) was administered as a 1-hour or 24-hour drip infusion daily at 5 micrograms/kg from day 5 until hematological recovery. The five year disease free survival (DFS) was 63% for 42 patients at CR 1, 41% for 41 patients at CR 2 and 33% for 40 patients at other stages. There was no significant difference in the DFS between allogeneic-transplantation and autologous-transplantation in all disease stages. In patients at remission stage for CR 1 and CR 2, the 5-year DFS by conditioning regimen was 63% for regimen (A), 54% for regimen (B) and 54% for regimens with melphalan and TBI. There was no significant difference in the DFS between the groups. Serious complications such as renal failure were observed in 11%, veno-occlusive disease in 9%, and interstitial pneumonia in 9%. The most dominating cause of death was relapse in the disease (48% of deaths) which was most commonly observed in autologous transplantation. Contrary to that, treatment related toxic death was the most frequent cause of deaths in allogeneic-transplantation.     

A Prototype manufacturing process for a health care IT appliance: a case study on an artery pulse monitoring system (APMOS)

This paper explores how a prototype of an IT appliance is manufactured through designing a housing of the appliance aesthetically, and designing the interface of its display. The aim of this study is to understand the role of prototype, and to clarify the problems and insufficient technology with which we should confront, and to investigate the efficient solutions to manufacture IT appliances well. The prototype manufacturing process presented here are to sketch the housings, to create clay models, to create 3D CAD models, to design interface of a display for an IT appliance, to manufacture functional mock-ups, and to examine the usability. An appliance to be developed is an artery pulse monitoring system named (APMOS). APMOS can measure blood pressure and the artery pulse, and let us know the condition for judging the possibility of arteriosclerosis. In this paper, we discuss in detail a manufacturing process from the concepts of the idea sketches to the usability test of the functional mock-up of APMOS.     

Efficiency of protective isolation in allogeneic stem cell transplantation in children

Forty children with hematological malignancies, who underwent allogeneic hematopoietic stem cell transplants between September 1994 and May 2001, were divided into 2 groups according to their infection control procedure; the standard protective isolation and the efficient protective isolation groups. Efficient protective isolation procedures included well-cooked foods and oral prophylaxis with new quinolones and antifungal drugs, while inhalation of antibiotics and antifungal drugs was suspended. We then compared the febrile index (= X/Y) [febrile period (X) of > 38.5 degrees C and the days (Y) with a post-transplant neutrophil count < 500/microliter] between the two groups. We discovered no significant difference between the febrile index of the two groups (0.25 vs. 0.38, p = 0.08), regardless of the type of transplantation (0.36 vs. 0.38, p = 0.14). The efficient protective isolation procedure was therefore feasible in this clinical setting.