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Biomedical subjects

L Y Shih

Publications and source records attributed to L Y Shih.

At least 73 records · Page 4Linked to original sources

Paroxysmal nocturnal hemoglobinuria: analysis of 40 cases.

Forty cases of paroxysmal nocturnal hemoglobinuria (PNH) were studied from 1978 to 1988. Thirty patients were male and 10 were female. Their ages ranged from 15 to 58 years with a median of 32 years. Symptoms related to anemia (85%) and dark colored urine (45%) were the most frequent clinical manifestations. Seven patients (17.5%) had a previous history of aplastic anemia. The interval between the diagnosis of aplastic anemia and PNH ranged from 11 months to 26 years. All the patients had anemia with varying combinations of cytopenia. In 36 patients, bone marrow examinations were performed, and 32 were hypercellular, one normocellular and 3 hypocellular. Documented thrombosis was noted in 3 patients, involving the intra-abdominal, cerebral and renal veins, respectively. The patient with intra-abdominal venous thrombosis subsequently died of E. coli septicemia. The remaining two patients achieved complete recovery. All 40 patients were treated with corticosteroids and/or anabolic agents, 32 (80%) patients improved and 4 (10%) achieved normal hemoglobin levels. Our studies demonstrate that there is a male predominance in Chinese PNH patients and the incidence of thrombotic complications is much lower than that reported by Western countries.

Adolescent↗

Diagnostic and prognostic values of in vitro culture growth patterns of marrow granulocyte-macrophage progenitors in patients with myelodysplastic syndrome.

The in vitro culture growth of marrow granulocyte-macrophage progenitors (CFU-GM assay) was studied in 102 consecutive patients with newly diagnosed primary myelodysplastic syndrome (MDS) to determine its diagnostic utility and prognostic value. There were 18 patients with refractory anemia (RA), eight RA with ringed-sideroblast (RARS), 30 RA with excess of blasts (RAEB), 18 chronic myelomonocytic leukemia (CMML), and 28 RAEB in transformation (RAEB-T). Patients with MDS had a significantly lower number of GM colonies and a significantly higher cluster to colony ratio than those of normal controls and patients with cytopenias of other causes. Six in vitro growth patterns were observed; 85% of patients with MDS showed various abnormal growth patterns, and 42% of all MDS patients exhibited a leukemic growth pattern at diagnosis. None of the 40 patients with cytopenias of other causes had a leukemic type growth. A leukemic growth pattern was rarely observed in patients with RA and RARS (4%), but was common in other subgroups (57%). The distribution of various growth patterns was not statistically different among patients with RAEB, CMML, and RAEB-T. Thirty-six patients developed acute leukemia during the follow-up period. The MDS patients with leukemic type growth were at increased risk of rapid progression to acute leukemia, and they also had a shorter survival time than patients with a non-leukemic pattern. These results showed that simply scoring the number of CFU-GM is of limited value for the diagnosis and the prediction of prognosis of MDS, whereas the in vitro marrow culture growth pattern is of prognostic significance independently of the FAB classification. It is concluded that the in vitro growth pattern of marrow CFU-GM is helpful in diagnosing patients with MDS as well as in predicting their clinical outcome.

Adult↗

[Hematological abnormalities in acute viral hepatitis and acute hepatitis in HBsAg carrier].

Various kinds of hematological abnormalities have been known to occur in liver diseases. To understand the hematological changes in acute viral hepatitis, 324 adults with acute viral hepatitis were studied. Of them, 3 were acute hepatitis A, 91 acute hepatitis B, 99 acute non-A, non-B hepatitis (NANB) and 181 acute hepatitis on chronic hepatitis B (AH on CH-B). There were 233 males and 91 females; age ranged from 16 to 74 years (mean age 39 years.) The results showed the incidences of thrombocytopenia (platelet less than 120,000/cmm), anemia (Hb less than 12 g% in male and less than 10% in female patients), leukocytosis (WBC greater than 10,000/cmm) and leukopenia (WBC less than 4,000/cmm) were 19.3%, 12.6%, 10.8% and 7.4%, respectively. Patients with AH on CH-B had significantly higher incidence of anemia and thrombocytopenia than those with acute B hepatitis; other than this, there was no significant difference. Patients with anemia, thrombocytopenia or leukocytosis had significantly higher mean levels of serum bilirubin and higher proportions of prolonged prothrombin time, suggesting that these hematological abnormalities were closely related to the severity of hepatocellular damage. In addition, there were 3 cases (0.9%) complicated with aplastic anemia. Two were NANB hepatitis and the other was AH on CH-B which was seronegative for anti-delta, possibly suggesting NANB virus superinfection. Of these 3 cases, 2 died of complications related to aplastic anemia and 1 survived with normal hematological findings 148 days later.

Acute Disease↗

Non-Hodgkin's lymphomas in Asia.

The relative frequencies of the various histopathologic types of lymphomas are generally similar among Asian countries. Hodgkin's disease and follicular lymphomas are relatively rare in Asia. Among NHL, the Asians have a higher rate of aggressive NHL, as compared with the NCI data. Immunologic analysis revealed that PTCL is common in Asia. The relative frequency of PTCL is comparable among Chinese in Taiwan, the east coast of China, and Hong Kong, as well as in adult T-cell leukemia/lymphoma (ATLL) nonendemic areas in Japan. The increased rate of T-cell lymphomas in Asia is attributed to the low incidence of follicular lymphomas. The similar patterns of distribution in histopathologic and immunologic subtypes of NHL in Asia suggest that a common ethnic or geographic factor exists. To elucidate it, further detailed epidemiologic studies are needed. Primary extranodal NHL is slightly more prevalent in Asia than in the United States; the most frequent primary site is Waldeyer's ring in Japanese patients and the GI tract in Chinese patients. Primary small intestinal lymphoma in Asia showed the pattern of the Western type. Primary cutaneous lymphomas are rare in Asia. The clinical features of PTCL in Asia are comparable with those described in the United States, except for a predilection for the nasal/paranasal region. In Asia, outside Japan, ATLL has been reported only in Taiwan. The seroepidemiologic survey of carriers of ATLL showed the rate of seropositivity for HTLV-I in Taiwan was similar to that in nonendemic areas in Japan. The clinicopathologic features of ATLL in Taiwan and Japan are essentially identical. In children in Japan and Taiwan, Hodgkin's disease is much less frequent than in the West. However, the relative frequencies of the histopathologic and immunologic subtypes of childhood NHL in Japan and Taiwan do not differ significantly from those of the West. Although Burkitt's lymphoma in Japan and Taiwan is of nonendemic type, in India it may comprise both endemic and nonendemic types in almost equal number.

Adult↗

Characterization of two newly established EBV-containing lymphoblastoid cell lines from patients with myeloid leukemias.

Two spontaneous outgrowing Epstein-Barr virus (EBV)-carrying lymphoblastoid cell lines (LCLs), CG2 and CG3, have been established from bone marrow cells of myeloid leukemia patients. CG2 was derived from a patient with chronic myelomonocytic leukemia (CMMoL) and who has a 45 XO karyotype. CG3 was derived from a patient with juvenile chronic myeloid leukemia (CML) and who carries a hypotetraploid karyotype, 91XXYY. Both CG2 and CG3 cells carry the same type of translocation; t(1;19)(q23;p13). Both cell lines are of an early B cell lineage as shown by their reactivities with monoclonal antibodies OKIa, B1, B2 and B4. The combination of horizontal discontinuous agarose slab gel and Southern hybridization results show CG2 and CG3 cells are of monoclonal origin and harbor episomal EBV genomes. Approximately 50 EBV genome equivalents were contained in CG2 and CG3 cells. Immunofluorescence studies demonstrate the expression of EBV-encoded antigen (EBNA) in almost all cells of these two lines. The expression of EA and VCA is only observed in a small percentage of cells and cannot be induced by treatment with TPA and SB. Therefore, CG2 and CG3 cells are probably nonproducer cell lines for EBV. The serum samples from both patients have been shown to contain elevated IgG antibody titers to EBV antigens. Both cells are found to be nontumorigenic in nude mice. These cells may provide an important tool in analyzing molecular epidemiological aspects of EBV infections in diseases such as CMMoL and juvenile CML.

Animals↗

Non-Hodgkin's lymphoma of Waldeyer's ring: an appraisal of therapeutic effect.

From July 1978 to June 1987, non-Hodgkin's lymphoma was diagnosed in 667 patients at Chang Gung Memorial Hospital. Forty-eight (7%) patients presented primarily with involvement of Waldeyer's ring; their provisional clinical stage was I or II. The tonsils represented the most frequent site of involvement within Waldeyer's ring. A work-up showed 33.3% of patients with stage I, 50% with stage II, 8.3% with stage III and 8.3% with stage IV. All the patients had diffuse lymphomas. Diffuse large cell (46%) and immunoblastic lymphomas (23%) were the most common histologic subtypes. Eight patients received radiotherapy alone, 39 patients were treated with combination chemotherapy with or without local radiation and one patient was lost to follow-up. Eleven of 16 stage I (69%) and 18 of 23 stage II (78%) patients achieved a complete remission. The median survival for stage I patients was over 54 months and the median survival of stage II was 72 months. There was no significant difference between the stage I and II patients with lymphoma of Waldeyer's ring with respect to the complete remission rate and survival. Stage I patients had a relapse-free survival rate of 55% at 4 years, similar to that (49%) of stage II patients at 8 years. We recommend that chemotherapy be used as primary therapy for those patients with localized aggressive lymphoma of Waldeyer's ring.

Adolescent↗

Congenital poisoning by polychlorinated biphenyls and their contaminants in Taiwan.

In 1979, a mass poisoning occurred in Taiwan from cooking oil contaminated by thermally degraded polychlorinated biphenyls. Because these chemicals persist in human tissue, children born to female patients after the outbreak were exposed in utero. In 1985, 117 children born to affected women and 108 unexposed controls were examined and evaluated. The exposed children were shorter and lighter than controls; they had abnormalities of gingiva, skin, nails, teeth, and lungs more frequently than did controls. The exposed children showed delay of developmental milestones, deficits on formal developmental testing, and abnormalities on behavioral assessment. These findings are most consistent with a generalized disorder of ectodermal tissue. This syndrome is one of very few documented to result from transplacental exposure to pollutant chemicals.

Conjunctivitis↗

Strain difference in galactokinase level and susceptibility to the teratogenic effect of dietary galactose in mice: I. Teratogenic and embryopathic effect.

Congenital cataracts have been noted to occur in infants when either the mother or the mother and infant have reduced activity of the enzymes galactokinase (GK) or galactose-1-phosphate uridyl transferase (GALT). Studies were undertaken to elucidate the possible genetic and dietary fetomaternal interactions leading to the presumptive galactose teratogenesis noted in two inbred strains of mice differing in GK activity. Pregnant A/J (high erythrocyte GK activity 134.18 +/- 17.89 mU/gm Hb) and C57BL/6J (low erythrocyte GK activity 55.05 +/- 11.39 mU/gm Hb) mice were treated with a diet containing either 25% or 50% galactose throughout gestation. A significantly higher percentage of C57BL offspring (92.3%) were observed to have lens opacities when their mothers were fed a high-galactose diet, whereas no increase in lens pathology was observed in the offspring of similarly treated A/J mothers. Additionally, reciprocal matings were carried out so that all offspring were genetically equivalent in terms of GK activity. However, when the mother had low GK activity, a significant incidence of lens opacities was present in their offspring; this was not found when the mother had high GK activity. After weaning, no difference in the incidence of lens opacities was observed when the 25% galactose diet was introduced to the offspring of these reciprocal crosses, providing additional support for a maternal dietary influence on the development of lens opacities.

Animals↗

In vitro culture studies of blood and marrow cells in chronic myeloid leukemia at different phases of the disease.

The in vitro culture growth of peripheral blood (PB) and bone marrow (BM) cells were studied simultaneously from 100 adult patients with chronic myeloid leukemia at different phases. Sixty-five patients were investigated at initial diagnosis, 30 patients in control phase, and 41 patients in blast phase. In untreated chronic phase, the relative concentrations of granulocyte-macrophage progenitor cells (CFU-GM) in BM were not significantly different from those of normal controls, but there was generally a marked increase in circulating CFU-GM. The 6 Ph1-negative patients did not show different growth characteristics. We were unable to correlate the CFU-GM number to any of the hematologic parameters as well as to the response to busulfan therapy. Pretreated patients with excessive cluster formation did not necessarily indicate impending blast crisis. In hematologic remission, the numbers of CFU-GM in both BM and PB were well within the ranges of normal controls. Culture results in blast phase revealed a spectrum of abnormal growth. In myeloid crisis, 14/29 BM and 12/29 PB samples showed increased colony and cluster formations which were composed predominantly of immature cells with variable degeneration. Marrow cells in lymphoid crisis produced low numbers of both colonies and clusters in 5 out of 8 patients, while blood cells from 8 out of 10 patients formed large amount of colonies of normal morphology. This study indicates that the in vitro CFU-GM assay may have diagnostic utility in differentiating lymphoid crisis from myeloid crisis.

Adult↗

Cutaneous and pericardial extramedullary hematopoiesis with cardiac tamponade in chronic myeloid leukemia.

A 48-year-old woman with Philadelphia chromosome-positive chronic myeloid leukemia developed skin and pericardial extramedullary hematopoiesis. The echocardiogram revealed massive pericardial effusion with signs of tamponade. The cytocentrifuge preparation of pericardial fluid demonstrated all three hematopoietic components. Assays for the granulocyte-macrophage progenitor cells and erythroid progenitors on her pericardial fluid gave rise to colony numbers comparable to those of normal bone marrow cells. The patient was successfully treated with pericardiocentesis followed by short-term indwelling catheter drainage and administration of hydroxy-urea. There was no reaccumulation of fluid at ten months.

Cardiac Tamponade↗

Estimate of attenuation coefficient for ultrasonic tissue characterization using time-varying state-space model.

The images generated from ultrasound pulse-echo signals have long been used to aid clinical diagnosis. Recently, there has been a growing interest in quantitatively determining the acoustic parameters of the tissue as a means of classification and diagnosis. For example, the frequency-dependent attenuation is known to be correlated with different diseases in the liver. In this paper we introduce a new technique for estimating the attenuation coefficient. The effect of attenuation on an interrogating signal with a gaussian-shaped spectrum can be obtained by studying the Wigner distribution of reflected rf data based on a one-dimensional signal model. We show that under the condition that the attenuation varies linearly with frequency, the spectral mean of the reflected signal decreases linearly with time. The estimation algorithm models the pulse-echo signal as the output of a second-order time-varying state-space innovations model driven by white noise. The state coupling matrix A and the output coupling vector C vary with time in a known fashion; moreover, they are also functions of an unknown constant parameter theta. The attenuation coefficient, which is one of the elements of theta, can be estimated directly using a recursive system identification algorithm. The algorithm was verified using both computer-generated synthetic data and in-vivo liver data of known diagnosis. The results show correlation between the estimated parameter and the pathological state of the tissue.

Algorithms↗

Kimura's disease. Involvement of regional lymph nodes and distinction from angiolymphoid hyperplasia with eosinophilia.

The clinicopathologic features of nine patients with Kimura's disease and 15 patients with angiolymphoid hyperplasia with eosinophilia (ALHE) were studied and compared in order to clarify the confusion between these two entities. The common features shared by both conditions included male predominance, predilection for the head and neck regions, tendency to recur, and vascular nature of the lesion with lymphoid and eosinophilic infiltrates. However, Kimura's disease was usually seen in younger individuals for a longer duration and occurred as a deeply seated, large soft-tissue mass, without significant change of the overlying skin initially. In addition, it was often accompanied by peripheral blood eosinophilia and elevated serum IgE. In contrast, ALHE lesions were multiple small dermal papular or nodular eruptions observed in older patients and present for a shorter duration; they were less frequently accompanied by peripheral blood eosinophilia. The main histopathological difference was the presence of "histiocytoid" or "epithelioid" blood vessels in ALHE but not in Kimura's disease. Kimura's disease was further characterized by eosinophilic folliculolysis; IgE deposits in the germinal centers; and frequent involvement of regional lymph nodes, salivary glands, and skeletal muscles. The eosinophilic infiltration, especially the formation of eosinophilic microabscesses, along with increased number of small blood vessels, perinodal eosinophilic infiltration, and eosinophilic folliculolysis characterized the nodal involvement by Kimura's disease. Our study indicates that Kimura's disease and ALHE are two distinct clinicopathologic entities. We place particular emphasis on the involvement of regional lymph nodes in Kimura's disease. In addition, we observed Charcot-Leyden crystals and polykaryocytes in both conditions. One of the patients with Kimura's disease also had an associated nephrotic syndrome.

Adolescent↗