A nationwide survey of common viral infections in childhood among patients with primary immunodeficiency diseases.

Objectives: Patients with primary immunodeficiency diseases (PID) are highly susceptible to various microorganisms. However, no population-based studies have been performed among common viral pathogens, such as respiratory syncytial virus (RSV), rotavirus (RV), varicella-zoster virus (VZV) and influenza virus (IV). The objective of this study was to reveal the clinical burden of these four infections among PID patients in Japan.

Quantitative assessment of PTPN11 or RAS mutations at the neonatal period and during the clinical course in patients with juvenile myelomonocytic leukaemia.

To evaluate minimal residual disease (MRD) after chemotherapy and haematopoietic stem cell transplantation in juvenile myelomonocytic leukaemia (JMML), a locked nucleic acid-allele specific quantitative polymerase chain reaction (LNA-AS-qPCR) was developed for 13 patients (four types of PTPN11 mutation and four types of RAS mutation). The post-transplant MRD detected by LNA-AS-qPCR analysis was well correlated with chimerism assessed by short tandem repeat PCR analysis. Non-intensive chemotherapy exerted no substantial reduction of the tumour burden in three patients.

Spontaneous improvement of hematologic abnormalities in patients having juvenile myelomonocytic leukemia with specific RAS mutations.

Of 11 children with juvenile myelomonocytic leukemia (JMML) carrying RAS mutations (8 with NRAS mutations, 3 with KRAS2 mutations), 5 had a profound elevation in either or both the white blood cells and spleen size at diagnosis. Three patients had no or modest hepatosplenomegaly and mild leukocytosis at presentation but subsequently showed a marked increase in spleen size with or without hematologic exacerbation, for which nonintensive chemotherapy was initiated. The other three patients with NRAS or KRAS2 glycine to serine substitution received no chemotherapy, but hematologic improvement has been observed during a 2- to 4-year follow up.

Aggressive hepatectomy for complete remission of metastatic germ cell tumor following chemotherapy: report of a case.

A 12-year-old girl presented with hemoperitoneum caused by disseminated liver tumors accompanying a retroperitoneal germ cell tumor and was rescued by transcatheter hepatic arterial embolization. Following systemic chemotherapy, the liver tumors decreased in size and number, although the retroperitoneal tumor was resistant to therapy. We simultaneously resected the retroperitoneal tumor, and the liver lesions by extended left lobectomy combined with resection of the three major (right, middle, and left) hepatic veins, while preserving the inferior right hepatic vein.

Sacrococcygeal chordoma in infancy showing an aggressive clinical course: an autopsy case report.

The autopsy case of a 3-year 6-month-old boy with chordoma arising in the sacrococcygeal region is presented. The primary lesion of the sacrococcygeal area was unresectable and lung metastasis was detected. He was treated with multi-agent systemic chemotherapy and radiation therapy, but the tumor was less responsive to these therapies.

Analysis of the circumstances at the end of life in children with cancer: symptoms, suffering and acceptance.

Background: In an effort to improve the quality of life of children with cancer, this study analyzes the signs and symptoms at the end of life in such children. It is hoped that these data will contribute to the development of appropriate programs to address the challenges faced by these children.

Procedure: Between 1994 and 2000, 28 children died after treatment for cancer at Hamamatsu University Hospital, Japan.

Analysis of the circumstances at the end of life in children with cancer: a single institution's experience in Japan.

Background: With the aim of improving the quality of life of children with cancer, this study presents an analysis of one hospital's experience with terminal care.

Methods: Between 1994 and 2000, 28 children died after treatment for cancer at Hamamatsu University Hospital. The circumstances of their deaths were analyzed through medical records and interviews with 8 sets of bereaved parents.

Two groups of Philadelphia chromosome-positive childhood acute lymphoblastic leukemia classified by pretreatment multidrug sensitivity or resistance in in vitro testing.

The development of effective chemotherapy is imperative for children with Philadelphia chromosome-positive (Ph) acute lymphoblastic leukemia (ALL) because of the poor prognosis of this condition. Initial cellular drug resistance is thought to be an important cause of induction failure and early relapse. We carried out in vitro tests using a methyl-thiazol-tetrazolium assay on bone marrow samples from 274 children with newly diagnosed ALL.