Pediatric hematopoietic stem cell transplantation in Taiwan.
Explore the source record for details and available documents.
Adolescent↗
Biomedical subjects
K H Lin
Publications and source records attributed to K H Lin.
Longitudinal observation and outcome of nonfamilial childhood haemophagocytic syndrome receiving etoposide-containing regimens.
The long-term outcome of 22 children treated with etoposide-containing regimens for haemophagocytic syndrome (HS) were longitudinally studied; none of them had a family history of the disease. All patients received etoposide-containing (150 mg/m2/d) regimens, combined, in 16 cases, with intravenous immunoglobulin (IVIG) and prednisolone. Complete remission (CR) was achieved in 12 patients, partial remission in seven, and early mortality occurred in three. Of the 12 CR patients, only four remain alive and disease-free, with a median follow-up of 47.4 months; one CR patient died due to infection and the remaining seven had relapsed diseases. Three patients with a partial response or with relapsed disease progressed to T-cell lymphoma, characterized, in the two cases tested, by clonal chromosomal abnormalities. Epstein-Barr virus (EBV) infection was implicated in disease pathogenesis in 15/22 patients. The overall survival was 45.5%, 40.9% and 40.9% at 1, 3 and 5 years, respectively, and disease-free survival for CR patients at these same times was 45.5%, 36.4% and 36.4%. The etoposide-containing regimen would appear to be an effective initial therapeutic option for childhood HS. However, in view of the frequency of partial remissions and relapsed disease, a more intensive chemotherapy or bone marrow transplantation should be applied. The progression to EBV-containing T-cell lymphoma in three patients is consistent with the previous observation that EBV-associated HS is a potentially malignant disease.
Adolescent↗
Inhibition of mitochondrial gene expression by antisense RNA of mitochondrial transcription factor A (mtTFA).
Mitochondrial transcription factor A (mtTFA) plays an important role in regulating the expression of mitochondrial genes. To gain a better understanding of the relationships between mitochondrial gene expression and mtTFA, the mtTFA gene was inserted into a mammalian expression vector, both in the sense orientation and in the antisense orientation. After construction, these plasmids were transfected into COS-7 cells. In antisense-transformed cells, the expression of the cytochrome c oxidase subunit I and subunit III genes encoded by mitochondrial DNA was inhibited and there was an accompanying reduction of the level of mtTFA protein. These results provide direct evidence that the expression of mitochondrial genes is under the control of mtTFA.
Animals↗
Simultaneous multiple viral infections in childhood acute lower respiratory tract infections in southern Taiwan.
Thirty paediatric patients with acute lower respiratory tract infections (ALRI) caused by simultaneous multiple viral infections (SMV) in a 3-year interval were reviewed. Twenty patients were infected with two viruses simultaneously; nine patients with three viruses; and one patient with four viruses. The frequency of individual viruses were: adenovirus, 18 (60 per cent); respiratory syncytial virus, 7 (23 per cent); influenza virus type A, 6 (20 per cent); influenza virus type B, 15 (50 per cent); parainfluenza virus type 1, 11 (37 per cent); parainfluenza virus type 3, 13 (43 per cent). There was no difference between the clinical presentations of ALRI with SMV and those of ALRI with a single virus. In conclusion, SMV was not uncommon in children with ALRI; the clinical presentations of multiple viral infection were similar to those of single viral infection.
Acute Disease↗
Mononucleosis syndrome and coincidental human herpesvirus-7 and Epstein-Barr virus infection.
Two girls (a 5 year old and a 21 month old) experiencing mononucleosis syndrome with coincidental human herpesvirus (HHV)-7 and Epstein-Barr virus (EBV) infections are described. One patient had primary HHV-7 infection and reactivated EBV infection. The other had primary HHV-7 and EBV infections. These cases indicated that HHV-7 is capable of inducing infectious mononucleosis-like illness. Multiple herpesvirus infection in one of the patients also suggests that interaction among herpesviruses can occur in vivo. The consequence of this interaction may have clinical implications.
Child, Preschool↗
Relation between histological intensity of transforming growth factor-beta isoforms in human osteosarcoma and the rate of lung metastasis.
Even though adjuvant chemotherapy has improved the 5-year survival rate of osteosarcoma patients, a significant percentage of patients eventually die from lung metastasis. Since transforming growth faCtor-beta (TGF-beta) has been demonstrated to be related to the tumor progression, we investigated the clinical implications of the presence of TGF-beta isoforms in 16 human osteosarcoma tissue. There were 10 males and 6 females with a mean age of 20.8 years of age (range, 8 to 57 years). Biopsied specimen before chemotherapy was fixed in 10% formalin, demineralized and followed by paraffin embedding. The locations of tumor included femur (10), tibia (3), humerus (1), fibula (1), and ilium (1). Histologic subtypes included osteoblastic (11), chondroblastic (2), and fibroblastic (3). All patients were followed for a minimum of 1 year (range 12 to 44 months) or to the development of lung metastasis. Five patients (31.3%) developed subsequent lung metastasis during the follow up. We used immunohistochemistry technique to investigate the presence of the TGF-beta isoforms in osteosarcoma tissue and its relationship to the subsequent pulmonary metastasis. The results showed the presence of one or more TGF-beta isoforms in tumor cells in osteosarcoma tissues (13 of 16, 81.3%) in all of the subtypes. However, minimal presence of TGF-beta isoforms was shown in the tumor bone matrix. The expression of TGF-beta1 or TGF-beta3 isoforms was associated with a higher rate of subsequent lung metastasis (p < 0.05, chi-square test). Further research is warranted to determine the utility of routine TGF-beta analysis in the clinical practice.
Adolescent↗
Ki-1 lymphoma in two children.
Childhood Ki-1 lymphoma is rarely reported in Taiwan. We present two 8-year-old girls with Ki-1 lymphoma diagnosed and treated at the National Taiwan University Hospital. The first patient presented with a neck mass with hepatosplenomegaly. The second patient initially presented with joint pain and pathologic fracture; she was later found to have bone marrow involvement. Histologic studies of both tumors revealed bizarre large cells with pleomorphic nuclei and prominent nucleoli. All these cells were immunolabeled with monoclonal antibody Ki-1. The two patients received different polyregime chemotherapy protocols followed by bone marrow transplantation (BMT); patient 1 had autologous BMT and patient 2 had allogeneic BMT. In addition, local radiotherapy and retinoic acid were tried for patient 1 but she responded poorly and died 2 years after presentation. In patient 2, although bone marrow was initially involved, allogeneic BMT was performed smoothly after tumor loading was reduced and the patient was in remission at last follow-up, 26 months after presentation. Ki-1 lymphoma in children is characterized by clinical presentations, and histologic and immunologic findings. Aggressive polyregime chemotherapy and bone marrow transplantation are treatments for this disease.
Bone Marrow Transplantation↗
Effects of an abdominal binder and electrical stimulation on cough in patients with spinal cord injury.
We explored the effect of an abdominal binder, with or without electrical stimulation, on peak expiratory flow rate (PEFR) in 12 paraplegics with complete thoracic cord (T2-T12) injury (mean age 36.0 +/- 1.5 yr) and 12 quadriplegics with complete cervical cord (C4-C8) injury (mean age 36.2 +/- 1.9 yr). The cough was assessed by measuring the PEFR during forceful expiration in a sitting position. The subjects underwent the following experimental maneuvers in a random order with a 10-minute interval between any two maneuvers: 1) voluntary coughing, 2) voluntary coughing with an abdominal binder, and 3) voluntary coughing with an abdominal binder and electrical stimulation. The electrical stimulator (50 Hz with 300 microseconds pulse width) was applied to the abdominal wall. Data were analyzed using multivariate analysis of variance for repeated measures. The abdominal binder did not significantly increase PEFR in either paraplegics or quadriplegics; the abdominal binder combined with electrical stimulation significantly increased PEFR by 15% in the paraplegics and 18% in the quadriplegics. These results indicate that electrical stimulation combined with an abdominal binder improves the cough ability in patients with cervical or thoracic spinal cord injury.
Abdominal Muscles↗
Transcatheter closure of atrial septal defect guided by on-line transesophageal echocardiography.
Transcatheter occlusion of secundum atrial septal defect has been tried since 1976. Some investigators have incorporated on-line transesophageal echocardiography so as to better monitor the procedure. Most, however, have used endotracheal intubation and general anesthesia. The aim of this study was to evaluate the feasibility of adjunct guidance using on-line transesophageal echocardiography without intubation and general anesthesia in adolescent or adult patients undergoing transcatheter occlusion of secundum atrial septal defects. Ten consecutive cases (age 15-68 years) of secundum atrial septal defects with a pulmonary to systemic flow ratio of > 1.5 and a balloon-stretched diameter of < or = 25 mm were enrolled in the study. The Sideris' buttoned devices were used. The procedure was guided by on-line transesophageal echocardiography and fluoroscopy. Endotracheal intubation and general anesthesia were not employed. The diameters of the atrial septal defects ranged from 6 to 19 mm as determined by transesophageal echocardiography, from 7 to 20 mm as determined by atrial angiography and from 11 to 25 mm as determined by balloon sizing. The Sideris' buttoned devices were successfully deployed in all the patients. On-line transesophageal echocardiography greatly facilitated balloon sizing, device development and immediate assessment. One device unbuttoned 24 hours after the procedure and was retrieved smoothly. The remaining 9 patients were followed-up for 12 months. Two patients had no shunt, 3 had a trivial (average diameter by transesophageal echocardiography = 1-3 mm) and 4 had a small (average diameter = 4-6 mm) residual shunt at the latest follow-up. The cardiothoracic ratios decreased from 0.52 +/- 0.06 to 0.48 +/- 0.06 (p = 0.0131). There was no mortality, stroke or device fracture during the follow-up period. Thus, transcatheter occlusion of secundum atrial septal defect under adjunct guidance using on-line transesophageal echocardiography without endotracheal intubation and general anesthesia is promising for selected patients.
Adolescent↗
The differential hormone-dependent transcriptional activation of thyroid hormone receptor isoforms is mediated by interplay of their domains.
Human thyroid hormone nuclear receptor isoforms (TRalpha1 and TRbeta1) express differentially in a tissue-specific and development-dependent manner. It is unclear whether these two isoforms have differential functions. We analyzed their interaction with a thyroid hormone response element with half-site binding motifs arranged in an everted repeat separated by six nucleotides (F2). Despite extensive sequence homologies, the two isoforms bound to F2 with different affinities and ratios of homodimer/monomer. Using F2-containing reporter gene, we found that the transcriptional activity of TRbeta1 was approximately 6-fold higher than that of TRalpha1. The lower activity of TRalpha1 was not due to differences in expression of the two isoforms because similar nuclear localization patterns were observed. To understand the structural determinants responsible for these differences, we constructed chimeric receptors in which hinge regions (domain D), hormone binding domains (domain E), and domains (D + E) were sequentially interchanged and their activities were compared. Chimeric TRs containing the domains D, E or (D + E) of TRbeta1 showed increased propensities to form homodimers and mediated higher transactivation activities than TRalpha1. Thus, differential transactivation activities of TR isoforms are mediated by interplay of their domains and could serve as an important regulatory mechanism to achieve diversity and specificity of pleiotropic T3 effect.
Binding Sites↗
Malaria infection after allogeneic bone marrow transplantation in a child with thalassemia.
A 12-year-old girl with beta-thalassemia hemoglobin E disease received a marrow transplant from her HLA-identical elder brother in July 1995. She had previously been treated by repeated blood transfusions. Conditioning included busulfan 16 mg/kg for 2 days and cyclophosphamide 120 mg/kg for 2 days. Cyclosporine was used for graft-versus-host disease prophylaxis. Spiking fevers occurred on days 6 and 11. Plasmodium falciparum parasites, both trophozoites and gametocytes, were found on the peripheral blood smear. Quinine 30 mg/kg three times a day for 7 days followed by a single dose of mefloquine 25 mg/kg was given. The fever subsided within 2 days and parasitemia cleared in 4 days. After transplant, the girl autologously reconstituted and was followed-up over 15 months.
Bone Marrow Transplantation↗
Quality of life of spinal cord injured patients in Taiwan: a subgroup study.
The major purposes of this study were to assess the quality of life (QOL) of spinal cord injured patients, and to assess the possible factors affecting the QOL. The survey was conducted from 1992-1993 by mailed questionnaires to members of Spinal Cord Injury Association of the Republic of China. There were 347 quality responses with the mean age of 37.5 +/- 10.2 years old and the mean duration of illness of 7.8 +/- 6.8 years. The questionnaire included five domains, physical mobility, environment-transportation, psychosocial adjustment, education and economics for a total of 39 items. Each item contained a rating of 'importance' and 'satisfaction'. The quality of life index (QLI) was calculated by multiplying the satisfaction score with the importance score, then dividing by the possible highest score. The major results included: (1) the subjects had mild to moderate dissatisfaction with most items in five domains except psychosocial adjustment: (2) quality of life in those with complete tetraplegia (QLI = -0.41) and incomplete tetraplegia (QLI = -0.31) was significantly lower than that of those with complete paraplegia (QLI = -0.13) and incomplete paraplegia (QLI = -0.04); (3) both the severity of injury and the post-injury working status were the major factors affecting the life quality of spinal cord injured patients in Taiwan.
Adult↗
Dominant negative activity of mutant thyroid hormone alpha1 receptors from patients with hepatocellular carcinoma.
Complementary DNAs for two mutant thyroid hormone alpha1 receptors (TR alpha1) were isolated from hepatocellular carcinomas of two patients. Sequence analyses of the complementary DNAs showed a single Val390Ala and double Pro398Ser/Glu350Lys mutations in mutants H and L, respectively. We characterized their hormone-binding, DNA-binding, and dominant negative activities. Mutants H and L did not bind the hormone T3. Their DNA-binding activities were analyzed using three types of thyroid hormone response elements (TREs) in which the half-site binding motifs are arranged in an everted repeat (Lys), an inverted repeat (Pal), or a direct repeat separated by four nucleotides (DR4). Compared with wild-type TR alpha1 (w-TR alpha1), which bound these TREs with different homodimer/monomer ratios, binding of mutant L to the three TREs as homodimers was reduced by approximately 90%. However, binding of mutant H to these TREs was more complex. Although it bound normally to DR4 as homodimers, its binding to Lys as homodimers was reduced by approximately 80%. Surprisingly, its binding to Pal was markedly enhanced compared with w-TR alpha1. The binding of these two mutants to the three TREs as heterodimers with retinoid X receptors (RXR alpha and -beta) was not significantly affected. Consistent with the lack of T3-binding activity, both mutants had lost their trans-activation capacity. Mutants H and L exhibited dominant negative activity, but differed in their TRE dependency. The dominant negative potency of mutant H was in the rank order of Pal > DR4 > Lys, whereas no TRE dependency was observed for mutant L. The present study indicates that mutations of the TR alpha gene do occur in patients and that these novel TR alpha1 mutants provide a valuable tool to further understand the molecular basis of the dominant negative action of mutant TRs.
Base Sequence↗
Long-term culture of primary rat hepatocytes on heparin- or lambda carrageenan-containing collagen gels.
The interactions of glycosaminoglycans (GAGs) with collagen are thought to be important in cell adhesion and cell differentiation. To investigate whether the interactions of GAG or sulfated polysaccharide with collagen can maintain the functions of cultured primary rat hepatocytes, GAG- or sulfated polysaccharide-containing collagen gels were reconstituted in vitro and used for culture of hepatocytes. Among the GAGs and sulfated polysaccharides examined, heparin- and lambda carrageenan-containing collagen gels were found to be able to stimulate and sustain albumin synthesis, while the other GAG- or sulfated polysaccharide-containing collagen gels had almost no effect on maintenance of albumin synthesis. In the cultures using collagen gels that contained 400 micrograms/ml heparin or 100 micrograms/ml lambda carrageenan, albumin synthesis by rat hepatocytes was prolonged to about 4 and 5 weeks, respectively, but albumin synthesis was kept up for only one week in the cultures using conventional collagen gels. These results suggest that the interactions of heparin or lambda carrageenan might be of importance for long-term maintenance of hepatocyte functions.
Albumins↗
Cord blood transplantation for acute lymphoblastic leukemia in a pediatric patient.
The prognosis of Philadelphia chromosome positive (Ph+) acute lymphoblastic leukemia (ALL) is poor. While umbilical cord blood transplantation has been used successfully for hematopoietic reconstitution, patients' size may be a limiting factor. We report an 11-year-old, 55-kg patient with Ph+ ALL, who received human leukocyte antigen-identical sibling donor cord blood transplantation (5.94 x 10(6) CD34+ cells) during the second ALL relapse. On days 25, 41, 75 and 103, successful engraftment was confirmed by cytogenetic studies. However, the leukemia relapsed on day 117 and the patient died on day 146 due to refractory ALL. In conclusion, based on the documented engraftment in our patient, we believe cord blood transplantation may be successfully employed in adolescent or possibly even adult patients.
Blood Transfusion↗
Allogeneic bone marrow transplantation for Philadelphia chromosome-positive chronic myelogenous leukemia in childhood.
Allogeneic bone marrow transplantation (BMT) offers the only potential for long-term control of chronic myelogenous leukemia. From November 1992 to August 1994, we prospectively studied five pediatric patients with Philadelphia chromosome-positive chronic myelogenous leukemia, a unique finding in Taiwan, who were treated with allogeneic BMT at different stages of the disease. Their ages at diagnosis ranged from 2 to 10 years. Four donors were HLA-matched siblings and the other was an HLA-matched unrelated donor. All patients received busulfan (4 mg/kg/day for 4 days) followed by cyclophosphamide (60 mg/kg/day for 2 successive days) as the conditioning regimen. Engraftment was documented within 22 days after transplantation in all five patients. Two out of the four patients in the sibling donor group, both of whom had BMT in the first chronic phase, achieved event-free survival after follow-up for 41 months and 17 months. The other two patients, who had BMT in the second lymphoblastic crisis and the second chronic phase, died within 6 months after transplantation due to lymphoid blastic crisis and complication of cytomegaloviral pneumonitis, respectively. The patient who received marrow from the unrelated donor underwent BMT in the accelerated phase and died within 6 months after transplantation due to myeloid blastic crisis. In conclusion, allogeneic BMT performed in the first chronic phase of childhood Philadelphia chromosome-positive chronic myelogenous leukemia seems to have better results than BMT after the first chronic phase.
Bone Marrow Transplantation↗
Human T-lymphotropic virus type II infection in Vietnamese thalassemic patients.
Anti-human T-lymphotropic virus type I/II (HTLV-I/II) antibodies were screened by particle agglutination test in a total of 66 patients with thalassemia major who received multiple transfusion from paid donors at the Blood Transfusion Hematology Center of Ho Chi Minh City in South Vietnam. HTLV-II infection was confirmed in 6 patients (9.1%) by Western blot analysis and/or polymerase chain reaction. Phylogenetic analysis revealed that long terminal repeat sequences of HTLV-II proviruses from 5 thalassemic patients in Vietnam belonged to the same phylogenetic subgroup of HTLV-IIb as those from intravenous drug abusers in North America and Europe. These data shed light on the route of introducing HTLV-II into Vietnam.
Adolescent↗
Relapse of acute leukemia in childhood presenting as proptosis due to an orbital mass.
Extramedullary relapse of acute leukemia in sites outside the central nervous system or testes is rare. We report two children who developed proptosis due to an orbital mass as the initial presentation of leukemic relapse. The first patient was an 11-year-old girl with acute myelogenous leukemia who had undergone bone marrow transplantation 4 years previously. She had been in remission until 2 months prior to the present admission, when she developed isolated relapse of disease in the left orbit. The second patient, an 11-year-old girl with acute lymphoblastic leukemia, had been off chemotherapy for 2 years and in remission for 5 years. She suffered from concurrent orbital and central nervous system relapse. Both patients were treated with systemic chemotherapy and orbital radiotherapy. More than 1 year after the relapse, the proptosis was completely cured in both patients.
Child↗