Proof of the one-hit mechanism of complement-induced lysis.
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Biomedical subjects
Publications and source records attributed to C K Li.
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The authors report a case of angiomatoid malignant fibrous histiocytoma (AMFH), affecting a 9-year-old girl, with a highly aggressive clinical course. The tumor, noticed by the patient as a painless nodule in the dorsum of her left foot for 12 months, recurred 8 months after initial excision, and despite wide local reexcision, metastasized 4 months later to the liver and lung, where it grew at an alarming rate, to the extent of occupying the entire left hemithorax in a period of 10 weeks and killed the patient 14 months after initial excision. Review of the literature showed that the culminated rates of recurrence, metastasis, and mortality for AMFH were 23.2%, 8.7%, and 4.3%, respectively, indicating that it is definitely a malignant neoplasm with a potentially fatal outcome.
Document examiners are frequently asked to determine whether or not a colour printout has originated from a particular inkjet printer. The printer can rarely be identified unless some unique defects or irregularities of the printer are present on the printout. However, it is possible to decipher the make and/or model of the printer by comparing the ink-profile of the questioned printout with that of a seized inkjet printer cartridge or from one in a database. This paper presents an overview of a systematic approach to characterising and discriminating the inks of different inkjet printer cartridges using thin layer chromatography (TLC) and high pressure liquid chromatography (HPLC) with multi-wavelength ultra-violet/visible (UV/Vis) detection. Ink samples from 23 different inkjet printer cartridges (including originals and substitutes) of different brands and colour printouts, printed by known printers were examined with newly developed chromatographic methods. Subsequently, a database of the ink-profiles was generated. The methods provide a useful tool for discriminating coloured inks in inkjet printer cartridges of different brands.
A 6-year-old boy with Hurler's syndrome presented with right heart failure and pulmonary hypertension secondary to severe obstructive sleep apnoea. Both his sleep apnoea and cor pulmonale were effectively controlled with continuous positive airway pressure therapy.
BACKGROUND: Various open and semi-closed methods are used for red cell (RBC) depletion and hematopoietic progenitor cell (HPC) enrichment of bone marrow (BM) in vitro, but with variable efficacy. A simple, efficient, and safe method using dextran 110k was developed. STUDY DESIGN AND METHODS: An equal volume of 4.5-percent dextran was applied to major ABO-incompatible BM in transfer bags and sedimentation was allowed for 30 minutes. RBCs, nucleated cells (NCs), and mononuclear cells (MNCs) from BM allografts before and after dextran sedimentation (DS) were counted. Flow cytometry, short-term cultures, and long-term cultures were performed to assay the respective recovery of CD34+ cells, colony-forming units (CFUs), and long-term culture-initiating cells (LTC-ICs). RESULTS: Sixteen BM collections were processed. The mean volume was 666 mL (range, 189-1355 mL). The mean +/-1 SD post-DS NC, MNC, CD34+ cell, and CFU counts per kg of the recipient's body weight were 4.11 +/-1.74 x 10(8), 8.98 +/- 3.68 x 10(7), 2.90 +/- 1.95 x 10(6), and 2.03 +/- 2.01 x 10(5), respectively, with the corresponding post-DS recovery being 90.6 percent, 90 percent, 92.4 percent, and 100.8 percent. The numbers of LTC-ICs in cultures (up to 12 weeks) of pre-DS and post-DS samples of five BM allografts were comparable (p = 0.91). Residual RBCs were 5.1 +/- 4.6 (0.1-14) mL with depletion of 96.5 +/- 3.2 percent. There was no significant difference in the mean absolute RBC count in post-DS BM allografts and in four ficoll-treated BM allografts (8.09 x 10(10) vs. 4.9 x 10(9); p = 0.206) and in eight major ABO-incompatible peripheral blood HPC collections (8.09 x 10(10) vs. 9.81 x 10(10); p = 0.87). No posttransplant hemolysis was encountered. Engraftment occurred at 22 +/- 7 days, which is similar to that of four transplants with ficoll-treated BM allografts (22 +/- 9; p = 0.611) and 54 unprocessed BM allografts (19 +/- 6; p = 0.129). CONCLUSION: DS is an efficient method of depleting RBCs in major ABO-incompatible BM allografts without significant loss of HPCs.
A previous study demonstrated impaired systolic function in 29% of patients treated with anthracycline as part of their therapy for malignant disease. A follow-up echocardiographic study was performed to determine whether there had been further deterioration of cardiac function. At least 40 months after the first study, those patients in whom abnormal systolic function had been detected and who had not received further anthracycline were studied by echocardiography using the same protocol as the initial study (group A). A second group of pediatric oncology patients who had not been given anthracycline but who had previously had cardiac assessment was selected as a control group (group N). The age and sex distributions of the two groups were comparable. Group A comprised 29 patients assessed on 2 occasions at mean times of 46 months and 89 months from the last dose of anthracycline. The mean dose of anthracycline received was 233 mg/m2 (range 20-400). Nine of 16 patients and 4 of 5 patients who had abnormal ejection fraction (EF) and fractional shortening (FS) at first assessment had normal EF and FS at the second assessment. There were no significant changes in EF, FS, and left ventricular wall stress (LVWS) between the two examinations. In group N, 20 patients were assessed after a mean interval of 43 months. There were no significant changes in EF, FS, or LVWS between the two examinations. At the first but not the second examination there were significant differences in the left ventricular internal diameters, EF, FS, and LVWS between group A and group N. Mildly abnormal cardiac indices detected in children after cessation of treatment with anthracycline did not deteriorate in 3 to 4 years follow-up. A longer cardiac follow-up study is indicated to assess the late outcome.
Two thalassemia major patients received regular blood transfusion and desferrioxamine chelation, and 1 patient had a splenectomy at 9 years of age. Both patients developed Klebsiella pneumoniae meningitis at age of 27 and 15 years. They died within a short time despite appropriate antibiotic treatment. Klebsiella meningitis may be more common in transfusion-dependent thalassemia patients.
An extra Y chromosome(s) is occasionally found in patients with various hematologic neoplasias; however, an association with hereditary blood diseases is unknown. In a child with beta-thalassemia who received a transplant with sex-mismatched umbilical cord blood and neonatal blood, fluorescent in situ hybridization (FISH) with probes to X and Y chromosomes revealed an extra Y signal in 19.3% of the hepatocytes and in 38.9% of the pretransplant peripheral blood cells, yielding YY/Y ratios of 0.24 and 0.64, respectively. In granulocyte colony-stimulating factor-mobilized autologous peripheral blood stem cells, the FISH ratio of interphase XYY cells to XY cells was 1.64, and there were 3.4 times more G-banded XYY metaphases than normal metaphases. Both FISH and polymerase chain reaction persistently demonstrated posttransplant mixed chimerism. There was a greater proportion of residual autologous XYY cells than XY cells with a mean posttransplant YY/Y ratio of 1.56 (n = 13) (1 SD = 0.33; range, 1.10 to 2.17). In vitro clonogenic assays yielded a normal number of colony-forming units but no growth in cultures without growth factor supplement. This study suggests that hematopoietic stem cells with an extra Y chromosome may upregulate cell growth in response to cytokine stimulation. A posttransplant preponderance of XYY cells might be attributable to an extra Y chromosome in hematopoietic stem cells withstanding the myeloablative conditioning regimen.
Accurate clinical diagnosis of severe acute respiratory syndrome (SARS) based on the current World Health Organization definition is difficult and at times impossible at the early stage of the disease. Both false positive and false negative cases are commonly encountered and this could have far-reaching detrimental effects on the patients, their family and the clinicians alike. Contact history is particularly important in diagnosing SARS in children as their presenting features are often non-specific. The difficulty in making a correct diagnosis is further compounded by the lack of a sensitive rapid diagnostic test. Serology is not particularly helpful in the initial triaging of patients as it takes at least 3 weeks to become positive. Co-infection and other treatable conditions should not be missed and conventional antibiotics should remain as part of the first-line treatment regimen. We report five cases to illustrate the difficulties and dilemmas faced by clinicians in diagnosing SARS in children.
A girl with chronic myeloid leukemia received bone marrow transplantation with total body irradiation as part of a conditioning regimen. She developed leukoencephalopathy about 50 days after transplant. There was permanent neurologic impairment afterward. She had not received any cranial irradiation or intrathecal chemotherapy before transplant. She had received 10 months of interferon treatment before transplant, which might have played a part in the development of leukoencephalopathy.
We describe two adolescent girls with alveolar rhabdomyosarcoma arising from extremities who developed bilateral breast metastasis in their clinical course. In both cases, there was widespread systemic disease at initial presentation. Although complete remission was achieved on the 25th week post-chemotherapy initiation, the first patient developed breast metastasis in addition to systemic recurrence on the 44th week and expired. For the second patient, breast metastasis was noted in addition to systemic disease at initial presentation. Aggressive chemotherapy with autologous transplant and radiotherapy were given in addition to bilateral subcutaneous total mastectomy. The patient remained in complete remission 3 months post-therapy. We postulate that adolescent females with alveolar rhabdomyosarcoma of the extremities have a high risk of developing breast metastasis in the pubertal period, and aggressive multidisciplinary treatment is indicated.
We present the magnetic resonance imaging (MRI), computed tomography (CT) and ultrasound appearances of three cases of pediatric head and neck rhabdomyosarcomas, involving the orbit, parameningeal region (nasopharynx and nasal sinus), and trapezius muscle anatomically. The histopathological subtypes included two embryonal and one alveolar. MRI is a superior imaging technique with its high contrast resolution of soft tissue tumors and multiplanar noninvasive capability. The literature on CT and MRI appearances of head and neck rhabdomyosarcomas is reviewed.