Biomedical subjects

K H Lin

Publications and source records attributed to K H Lin.

223 records · Page 13Linked to original sources

PubMedDate not supplied

Bone marrow transplantation for childhood acute myelogenous leukemia.

Six consecutive patients with acute myelogenous leukemia (AML) underwent 7 allogeneic bone marrow transplants at National Taiwan University Hospital. Marrow ablation for 4 patients consisted of busulfan 16 mg/kg and cyclophosphamide 120 mg/kg (BUCY 2). Two patients had busulfan 16 mg/kg and cyclophosphamide 200 mg/kg (BUCY 4) as marrow ablation. One had a second transplant following cytosine arabinoside 3 gm/m2/dose x 10 doses plus total body irradiation 12 Gy. Graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporine and short course methotrexate. Four patients received marrow from their HLA compatible siblings and two from their HLA-haplotype-matched fathers. Four transplants were performed during first remission and the other three during subsequent remission or relapse. All patients except one engrafted and achieved a complete remission (CR). Three of 4 patients transplanted in first CR are alive for over 10, 20 and 59 months respectively after transplant. One of the two patients who each received marrow from their fathers during 2nd CR and relapse, developed relapse 5 months later and the other developed aplasia 3 months later. Acute GVHD occurred in two of six patients. Localized chronic GVHD occurred in one of these two patients. Toxicities of BUCY 2 were minimal except veno-occlusive disease. One patient who received BUCY 4 developed hemorrhagic cystitis. There were no treatment related deaths except one patient who received 2nd transplant. These results demonstrate that BUCY 2 should be considered as a preparative regimen for allogeneic bone marrow transplantation for patients with AML in first remission.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMedDate not supplied

Cytomegalovirus-induced persistent mononucleosis in an infant.

Chronic mononucleosis has recently gained much attention in the literature. Almost all cases of this new syndrome have been ascribed to persistent Epstein-Barr virus infection. However, a case presented with infantile chronic mononucleosis syndrome caused by persistent cytomegalovirus infection. Transient clonal chromosomal change was noted during follow-up.

Chronic Disease↗

PubMedDate not supplied

Oculomotor apraxia in a case of Gaucher's disease with homozygous T1448C mutation.

We report a 10-month-old male infant with Type 2 Gaucher's disease. In addition to gradual arrest of neurological development, laryngospasm, opisthotonus, and limb rigidity, he presented characteristic oculomotor apraxia. History taking revealed that he had had abnormal horizontal gaze and had to turn his head to follow an object instead of moving the eyes alone. His eyes were in a divergent position while he was in a deep coma; however, when consciousness improved, he could open eyes with neutral eye position. Due to the impairment of reflex saccades, the doll's eye phenomenon was not reliable in evaluating the brainstem function when he was in the comatose stage. His leukocyte glucocerebrosidase activity was very low, but the typical Gaucher cell was absent in the sample of bone marrow aspiration. Molecular analysis by amplification refractory mutation system (ARMS) screening proved that he was a homozygote for T1448C mutation. To our knowledge, the T1448C gene frequency of Chinese Gaucher's disease is high. Thus, the ARMS screening method is applicable for further genetic diagnosis of Chinese Gaucher patients. Finally, this successful genetic diagnosis makes it possible in the future to perform prenatal diagnosis.

Apraxias↗

PubMedDate not supplied

Can severe neonatal jaundice be prevented by neonatal screening for glucose-6-phosphate dehydrogenase deficiency?--a review of evidence.

An evidence-based approach is used to evaluate the neonatal screening program for glucose-6-phosphate dehydrogenase (G-6-PD) deficiency. The primary consideration to include G-6-PD deficiency (G-6-PDD) in neonatal screening program was the public health burden of G-6-PDD-associated neonatal jaundice (G-6-PDDANJ) in the target population. However, the prevalence of G-6-PDD per se cannot be the sole index of the public health burden of G-6-PDDANJ. In more developed areas, G-6-PDDANJ is no longer a major public health problem. Further, most cases with G-6-PDDANJ in more developed areas are not precipitated by any identifiable icterogenic agents, and therefore not preventable by avoidance education. In less developed areas, however, G-6-PDDANJ is still a big public health burden and requires intervention. In this study, the effectiveness of neonatal screening programs for G-6-PDD to prevent severe neonatal jaundice(NJ) has been shown based on historical comparison, but the results may be confounded by other temporal factors. G-6-PDDANJ usually occurs in the first week after birth. Prompt need for G-6-PD screening results precludes it from incorporation into other existent neonatal screening programs (i.e., for PKU), and from centralization of laboratory work. The efficacy, adverse effects and cost-effectiveness of this mass screening program need further study.

Cost-Benefit Analysis↗

PubMedDate not supplied

Burkitt's lymphoma with intussusception mimicking a colonic polyp.

A 2-year-7-month old boy, presented with painless bloody stool was initially found to have an ulcerative, wide-based polyp in the ascending colon near the ileocecal region by colonoscopy. He received operation due to subsequent intussusception. Pathologic examination revealed an unusual case of polypoid Burkitt's lymphoma. The patient lives well 20 months later after receiving a standard chemotherapy. This case reminds us that colonic polyp and intussusception which is caused by Burkitt's lymphoma should be included in the differential diagnosis for a "wide-based polypoid" mass in the ascending colon.

Burkitt Lymphoma↗

PubMedDate not supplied

Absolute ethanol embolotherapy for hemangioma with Kasabach-Merritt syndrome.

This report describes a female baby having a hemangioma over the right thigh that had appeared as an irregular bruise since two days old. Severe thrombocytopenia, consumptive coagulopathy, anemia, and heart failure developed at three months old. With a diagnosis of Kasabach-Merritt syndrome, systemic corticosteroid, vincristine, subcutaneous alpha-interferon, and massive plate transfusion were given. However, the platelet count remained low and the skin lesion enlarged gradually. Trans-feeding-arterial embolotherapy with a 5 ml pure ethanol (1 ml/kg) injection was performed at four months of age. Thirty days later, her platelet count recovered and the hemangioma shrunk in size. This case illustrates that absolute ethanol embolotherapy is an effective treatment for hemangioma with Kasabach-Merritt syndrome and may be life-saving for those with lesions refractory to medical therapy.

Disseminated Intravascular Coagulation↗

PubMedDate not supplied

Transplantation for adrenoleukodystrophy with HLA-A and B nonidentical paternal marrow: report of one case.

We report the result of allogeneic bone marrow transplantation (BMT) in a 14-year-old boy who was neurologically severely involved with the childhood form of adrenoleukodystrophy (ALD) and received marrow from his HLA-A and B nonidentical, MLC-nonreactive paternal donor without T-cell depletion processing. Bone marrow transplantation corrected the excess content of very long chain fatty acid in plasma but did not arrest the deterioration of the neurological status during 3.5-year post-transplant follow-up period. Since partially matched or unrelated donors have been applied to clinical BMT successfully with current new techniques, ALD patients will have a better prognosis when they are transplanted in status of mild and early involvement. Our first experience may be helpful in more trials of BMT for genetic leukodystrophy in Taiwan.

Adolescent↗