Biomedical subjects

L Y Shih

Publications and source records attributed to L Y Shih.

At least 55 records · Page 3Linked to original sources

PubMed1994

Extramedullary plasmacytoma of the head and neck.

Extramedullary plasmacytoma (EMP) is an uncommon malignant plasma cell neoplasm which usually presents as soft tissue mass in the head and neck. We retrospectively reviewed 7 cases with EMP of the head and neck between 1978 and 1992. All patients were male. Their ages ranged from 27 to 66 years. M-protein was identified in 5 patients (IgG-kappa in 3, IgG-lambda in 1 and Lambda light chain in 1). All but one were treated with local irradiation. Three patients received chemotherapy. All patients have been alive for 37+ to 116+ months since the diagnosis of EMP. One patient had a cervical node recurrence 9 years after local irradiation and the other one who refused local irradiation but received chemotherapy developed multiple myeloma 5 years later. Based on our experience and the review of the literature, it is recommended that irradiation to the primary sites and the involved cervical nodes is the treatment of choice. Additional chemotherapy may be considered in patients with disease progression, recurrence, or dissemination. The M-protein is a useful parameter to assess tumor control or disease progression.

Adult↗

PubMed1994

Solitary plasmacytoma of bone.

From 1978 to 1992, 19 patients were diagnosed with solitary plasmacytoma of bone (SPB) accounting for 5.5% of plasma cell neoplasms in Chang Gung Memorial Hospital. Fourteen were male and five were female, with ages ranging from 21 to 71 years (median, 53 years). M-protein was detected at diagnosis in five patients, and during follow-up in three. The most common sites of SPB were the vertebral bodies, with seven involving the thoracic, four involving the lumbar and two involving the cervical spine. The remaining sites included the skull in four, and femur and pelvis in one each. Fifteen of the patients received local radiotherapy, local control was achieved in all; nine patients were alive with no evidence of disease for seven to 77 months, two patients remained in apparent remission at 14 and 92 months, two patients progressed to multiple myeloma at 12 and 84 months, one patient developed extramedullary plasmacytoma at three months, and one patient died of intercurrent disease at four months. The four patients who were not treated with radiation therapy ultimately developed local recurrence or dissemination within four years. In our series, local recurrence or dissemination was always associated with the appearance of, or an increase in, the M-component; thus, measurement of M-protein is helpful in assessing tumor control or progression. Based on our experience and a review of the literature, it is recommended that irradiation of the primary sites is the treatment of choice. Chemotherapy is indicated in those patients with disease progression, recurrence, or dissemination.

Adult↗

PubMed1993

Familial Pallister-Hall syndrome: case report and hormonal evaluation.

Pallister-Hall syndrome is a usually lethal dysplasia/malformation syndrome characterized by hypothalamic hamartoblastoma, hypopituitarism, postaxial polydactyly, craniofacial malformations, imperforate anus, and other malformations. We report a familial case in a male infant and his female sib fetus, suggesting autosomal recessive inheritance, or germinal mosaicism for an autosomal dominant mutation, or a segregating submicroscopic chromosome abnormality. Detailed endocrine evaluation on the surviving infant revealed documented pituitary function, pituitary deficit, and hypothalamic deficiency. We suggest that hypothalamic dysfunction contributes to the hypopituitarism seen in Pallister-Hall syndrome.

Abnormalities, Multiple↗

PubMed1993

Limb salvage surgery for locally aggressive and malignant bone tumors.

Since 1983, 77 patients have had limb salvage surgery for locally aggressive or malignant bone tumor of the limbs or pelvis. Twenty-nine patients had locally aggressive lesions with giant cell tumors the most prevalent, and 48 patients had malignant lesions, of which osteosarcomas predominated. Resection with a wide margin could be achieved in most of the patients. A mobile joint reconstruction was performed in 57 patients and an arthrodesis in 14 patients. At follow-up, 14 patients have died of disease, 7 patients have survived with disease, and 56 patients (73%) have survived without disease. Local recurrences were encountered in 4 patients (5%). The overall functional results were classified as excellent in 11 patients, good in 45 patients, and fair in 9 patients. Twelve patients had poor results because of failure of the reconstruction or amputation due to complications. Twenty-seven complications were encountered, among which infection was the most serious. Careful selection of patients and consideration of procedure chosen to reconstruct the defect are important for a successful outcome. This decision is based on a number of factors related to the tumor and the patient. Presently, major neurovascular involvement, displaced pathological fracture, inappropriate biopsy incision, extreme youth of the patient, and infection were considered to be contraindications to resection in our institution. Although various procedures promise functional restoration, the reconstructive procedure should be individualized and designed to meet the needs of the patient.

Adult↗

PubMed1993

Uniparental disomy explains the occurrence of the Angelman or Prader-Willi syndrome in patients with an additional small inv dup(15) chromosome.

A patient with Angelman syndrome and a 46,XY/47,XY,+inv dup(15)(pter-->q11: q11-->pter) karyotype and a patient with Prader-Willi syndrome and a 46,XY/47,XY,+inv dup(15)(pter-->q12: q12-->pter) karyotype were investigated with molecular markers along chromosome 15. Paternal uniparental isodisomy was found for all informative markers in the first case which indicates that this, rather than the presence of the extra chromosome, is the cause of the Angelman syndrome phenotype. Similarly, the PWS patient showed maternal uniparental distomy with absence of PWS region material on the inv dup(15) chromosome. If (1) marker chromosomes are an occasional by product of 'rescuing' a trisomic fertilisation, or (2) if duplication of the normal homologue in a zygote which has inherited a marker in place of the normal corresponding chromosome 'rescues' an aneuploid fertilisation, or (3) if the presence or formation of a marker chromosome increases the probability of non-disjunction, then uniparental disomy might be found occasionally in other subjects with de novo marker chromosomes.

Angelman Syndrome↗

PubMed1993

Changes in gait and maximum ankle torque in patients with ankle arthritis.

Findings from quantitative gait analysis and maximum ankle torque were used to assess the walking pattern of patients with ankle arthritis and to correlate the changes of gait parameters and muscle strength with severity of arthritis. Gait analysis and the isokinetic maximum ankle torque test were performed in 20 patients with first to fourth degree traumatic ankle arthritis. Six patients without evidence of traumatic arthritis were used as controls. Isokinetic maximum ankle plantarflexion and dorsiflexion torques were determined with Cybex instrumentation. Force plate and foot switch data were gathered during level walking. Maximum ankle plantarflexion and dorsiflexion torques were diminished in the injured ankles. Velocity, stride length, and cadence were decreased in arthritic patients compared with controls. The arthritic limbs had shorter single limb stance and longer double stance during free and fast walking speeds compared with the controls' affected ankles. The patterns of ground reaction forces were similar in the injured and uninvolved limbs as well as the control subjects, except the magnitude of vertical forces during push-off were reduced in arthritic ankles. The gait parameters and muscle strength deteriorated as the arthritis became severe, but they showed significant changes only when the patients had third or fourth degree arthritis.

Adult↗

PubMed1993

Bone marrow necrosis in 38 adult cancer patients.

From 1977 to 1990, 40 cases of bone marrow necrosis (BMN) were diagnosed among 10,856 (0.37%) consecutive bone marrow aspirations performed alone or with biopsy. All but two patients had underlying malignancies. Leukemia, nasopharyngeal cancer and cancer of unknown origin were the most common underlying diseases. Severe bone pain and fever were the predominant presenting symptoms. Anemia, schistocytes and leukoerythroblastosis were noted in the peripheral blood smears in 97% (37/38), 72% (22/32), and 66% (23/35) of the patients with cancer and BMN. Malignant cells were found in bone marrow aspirates or biopsy specimens in all but one of the patients. Varied degrees of disseminated intravascular coagulation were demonstrated in all of the 10 patients examined who displayed coagulation status. Four of seven patients with leukemia achieved complete remission after chemotherapy, and the survival time for the complete responders ranged from 10 months to eight years. The outcome of cancer patients with BMN who did not respond to chemotherapy was poor, with a median survival time of six weeks.

Adolescent↗

PubMed1993

Multiple glomus tumors: a case report.

Multiple glomus tumor (MGT) is rare in incidence. A 37-year-old male was diagnosed to have localized MGT over his right leg. With the characteristics of unusual location (right lower leg), development in sequence, and distribution along the course of the lateral sural cutaneous nerve. Its pathological findings were also characteristic. When the overall presentations showed in cases with recurrent symptoms and signs of glomus tumor after surgical excision, recurrence and multiplicity should be considered.

Adult↗

PubMed1993

Erythrocytosis in patients with renal failure on hemodialysis: study of underlying mechanism by in vitro erythroid culture assay.

Five patients with erythrocytosis associated with renal failure on maintenance hemodialysis were investigated for in vitro erythroid progenitor growth and the effect of their uremic sera on normal erythropoiesis. The duration of hemodialysis prior to discovery of erythrocytosis ranged from 1 week to 96 months. None had acquired cystic disease and no other known cause of increased erythropoietin (Epo) production was identified. With the presence of Epo in cultures, all five patients grew erythroid colonies within normal or higher than normal ranges. Three patients formed spontaneous erythroid colonies in the absence of added Epo; all three fulfilled the clinical diagnosis of polycythemia vera (PV). The uremic sera from patients with PV lacked either a stimulating or an inhibiting effect on normal erythropoiesis. The association between renal failure and PV was coincidental. The other two patients without endogenous erythroid colony formation had enhanced erythropoietic activity in their sera, which increasingly stimulated the erythroid colony growth by normal bone marrow cells as the concentration of the uremic serum was increased. The etiology of increased Epo production in these 2 patients remained undefined during long-term follow-up. The present study on five uremic patients with polycythemia showed two different underlying mechanisms of erythrocytosis--characteristic autonomous erythroid proliferation for PV in three patients and inappropriate idiopathic Epo production in two patients.

Aged↗

PubMed1993

Segmental total knee arthroplasty after distal femoral resection for tumor.

Sixty-one patients treated with custom-made segmental total knee reconstruction after resection of a primary tumor of the distal femur were reviewed retrospectively. Two types of implants were used: the Walldius hinge prosthesis (Walldius Group, n = 45) and the Kinematic rotating hinge prosthesis (Kinematic Group, n = 16). The mean periods of follow-up observation were 6.4 years for the Walldius Group and 2.3 years for the Kinematic Group. The overall functional results in the Walldius Group were excellent in 7%, good in 26%, fair in 25%, and poor in 42% of the patients. Roentgenographic analyses showed that 50% of the patients had bony resorption, 33% had interface loosening, and 10% had prosthetic stems that were broken at the time of last follow-up evaluation. Forty-two complications occurred in 34 patients who needed additional surgical treatment. Nine of the prostheses in the Walldius Group failed (20%), and 27 of the complications (64%) associated with the use of the Walldius prosthesis occurred in the first three years after implantation. Nineteen Walldius prostheses (42%) were revised or removed for reasons other than local recurrence. The overall functional results of the Kinematic Group were as follows: excellent in 25%, good in 44%, fair in 25%, and poor in 6% of the patients. Roentgenographic analysis showed that there was no change in bone on 81% of the discharge roentgenograms, that there were no radiolucent lines in 87% of the prosthetic replacements, and that there were no radiolucent lines less than 2 mm thick on the roentgenograms. None of the Kinematic rotating hinge prostheses required revision, except one with a patellar button fracture, which was revised without compromising the functional results. Four complications occurred in three patients in the Kinematic Group, and two of them required surgical treatment. The patients with the Kinematic rotating hinge prosthesis had better early functional and roentgenographic results and fewer complications.

Adolescent↗

PubMed1992

HTLV-I-positive and HTLV-I-negative peripheral T-cell lymphomas in Taiwan Chinese.

The clinico-pathologic features of 107 adult Chinese patients with peripheral T-cell lymphoma (excluding primary cutaneous lymphoma) are described and a comparison between HTLVI+ and HTLV-I- patients is made. There were 27 HTLV-I+ and 80 HTLV-I- patients. The virus-positive and -negative groups both had a male predominance and an identical median age of 48. Most patients in both groups presented with stage-IV disease, B symptoms, lymphadenopathy and hepatosplenomegaly. The HTLV-I+ group had a significantly higher incidence of skin and pulmonary lesions, bone marrow and peripheral blood involvement, hypercalcemia, and elevated LDH level compared to the HTLV-I- group. Sinonasal lesions (10), mediastinal mass (5), and GI tract involvement (6) were only seen in the HTLV-I- group. Leukocytosis with the presence of circulating pleomorphic lymphoid cells was characteristic of HTLV-I+ cases, while cytopenia was more frequently present in HTLV-I- cases. All of the 24 HTLV-I+ patients tested were CD4+CD8-; of the 67 HTLV-I- patients tested, 46 were CD4+CD8-, 9 were CD4-CD8 , 5 were CD4-CD8- and 7 were CD4+CD8+. Phenotypic studies revealed significant differences in the expression of CD7 and CD25 between virus-positive and -negative groups. Both groups responded poorly to therapy. The median survival of HTLVI+ and HTLV-I- patients was 4 months and 13.5 months, respectively. Apart from the presence of more than 3 extranodal lesions, none of the other clinical features or histologic subtypes had prognostic significance in the entire group or either of the subgroups. This series of peripheral T-cell lymphomas in Taiwan indicate that HTLV-I+ and HTLV-I- patients had many features in common, but presented several distinct differences.

Adolescent↗

PubMed1992

Intrathoracic extramedullary hematopoietic tumor in hemoglobin H disease.

We report a Chinese patient with hemoglobin H (Hb H) disease who developed intrathoracic extramedullary hematopoiesis (EMH) 17 years following splenectomy for a blunt abdominal injury. The patient initially presented with extreme hyperbilirubinemia and multiple intrathoracic tumors. Hb H disease was diagnosed after investigation, and the marked jaundice, which declined gradually after supportive treatment, was attributed to his chronic hemolysis superimposed on an acute hepatitis C virus infection. A biopsy of the intrathoracic tumors revealed an EMH. Intrathoracic EMH, which is usually encountered in patients with beta-thalassemia and hereditary spherocytosis, has never been reported in Hb H disease. In areas where thalassemia is prevalent, EMH should be considered in the differential diagnosis of patients who have chronic anemia with asymptomatic intrathoracic tumor to avoid unnecessary surgical interventions.

Adult↗

PubMed1992

Skeletal metastasis from occult carcinoma.

We reviewed 177 patients with skeletal metastases, seen between 1984 and 1989, to define the characteristics of metastatic bone disease from an occult primary carcinoma. In 52 (30%) patients, the primary carcinomas could not be identified when the bone metastases were first diagnosed. This group was predominantly male, with intractable pain the most common symptom. The primary tumors were identified on antemortem evaluation in 28 (54%) patients after extensive examination. Among these, the primary tumor was in the lung in 9 patients, followed by liver (8), kidney (5), prostate (3), thyroid gland (2), and rectum (1). The identifiable occult malignancies possessed three common features: all were osteophilic tumors, all had a high incidence in the specific geographic area, and all were not amenable to early detection. The mean survival of these patients was 11 months. Current treatment modalities failed to affect the course of these patients, except for those with primary carcinomas of the kidney and prostate. This observation attests to our limitations in both the diagnosis and treatment of this problem. Efforts should be directed primarily toward excluding those common and/or treatable tumors only.

Bone Neoplasms↗

PubMed1992

Avascular necrosis of the femoral head--an unusual complication of an intertrochanteric fracture.

Avascular necrosis of the femoral head is an uncommon complication after intertrochanteric fracture. We report six patients with this unusual complication 1-5 years after intertrochanteric fracture. The possible etiologies of this complication include a more proximal intertrochanteric fracture, a high-energy injury with fracture displacement resulting in vascular damage, and adverse influences of the operative procedures on the vascularity of the femoral head.

Adult↗

PubMed1992

Mucolipidoses II and III variants with normal N-acetylglucosamine 1-phosphotransferase activity toward alpha-methylmannoside are due to nonallelic mutations.

Normal N-acetylglucosamine 1-phosphotransferase activity toward mono- and oligosaccharide acceptor substrates was detected in cultured skin fibroblasts from mucolipidoses II and III patients who were designated as variants (one of four mucolipidosis II and three out of six mucolipidosis III patients examined). The activity toward natural lysosomal protein acceptors was absent or deficient in cell preparations from all patients with classical as well as variant forms of mucolipidoses II and III. Complementation analysis, using fused and cocultivated mutant fibroblast combinations, revealed that, while cell lines with variant mucolipidosis III constituted a complementation group distinct from that of classical forms of mucolipidoses II and III, the variant mucolipidosis II cell line belonged to the same complementation group as did the classical forms. In contrast to the mutant enzyme from variant mucolipidosis III patients that failed to recognize lysosomal proteins as the specific acceptor substrates, the activity toward alpha-methylmannoside in the variant mucolipidosis II patient could be inhibited by exogenous lysosomal enzyme preparations (bovine beta-glucuronidase and human hexosaminidase A). These findings suggest that N-acetylglucosamine 1-phosphotransferase is composed of at least two distinct polypeptides: (1) a recognition subunit that is defective in the mucolipidosis III variants and (2) a catalytic subunit that is deficient or altered in the classical forms of mucolipidoses II and III as well as in the mucolipidosis II variant.

Alleles↗

PubMed1992

Primary myelodysplastic syndrome: an analysis of 56 patients.

A total of 56 patients were diagnosed as primary myelodysplastic syndrome (MDS) at Chang Gung Memorial Hospital, Kaohsiung from April 1986 to December 1991. The median age was 65 years with an equal sex ratio. All patients presented with anemia and 52% with pancytopenia. The overall median survival for the entire group was 7 months, in which the chronic myelomonocytic leukemia (CMMoL) was 7 months, and 4 months for each of the refractory anemia with excess of blasts (RAEB) or the refractory anemia with excess of blasts in transformation (RAEB-T), however, the median survival had not been reached at 27 months for refractory anemia (RA) and at 33 months for refractory anemia with ring sideroblasts (RARS). Low-does arabinosyl cytosine (Ara-C) was administered in 9 patients with RAEB and RAEB-T, but no survival benefit was noted. Infection, especially pneumonia, was the most common cause of death. In 61 febrile episodes with clinically suspected sepsis, 10 (17%) were documented to associate with bacteremia. Twelve patients (7 RAEB, 4 RAEB-T, and 1 CMMoL) evolved to acute myelogenous leukemia (AML), the median interval from diagnosis to evolution was 4.8 months. This series indicates that only two groups of FAB subtypes could be clearly separated in terms of morphological findings and clinical outcome; RA and RARS constitute a good prognostic group, whereas RAEB, CMMoL, and RAEB-T constitute a poor prognostic group.

Adolescent↗

PubMed1991

Human T-cell lymphotropic virus type I associated adult T-cell leukaemia/lymphoma in Taiwan Chinese.

Twenty-five Chinese patients with human T-cell lymphotropic virus type I (HTLV-I) associated adult T-cell leukaemia/lymphoma (ATLL) were identified in Taiwan. No patients had been outside Taiwan and none were descendants of Japanese heritage. Their ages ranged from 28 to 71 years. There were 17 men and eight women. Main clinical and laboratory features at presentation were lymphadenopathy (16), skin lesions (11), hepatosplenomegaly (11), pulmonary lesions (11), hypercalcaemia (10) and bone marrow infiltration (14). Peripheral blood was characterized by leucocytosis with presence of pleomorphic abnormal lymphocytes but rare anaemia or thrombocytopenia. The clinical subtypes were acute in 15, chronic in three, smouldering in one, and lymphoma type in six. The immunophenotypes of the ATLL cells were characterized by the expression of CD2+, CD4+, CD7-, CD8- and CD25+. The overall prognosis was poor with a median survival of 5 months. The acute form had a significantly shorter survival (2 months) than lymphoma type (13 months). Susceptibility to various infections was common. Pulmonary complications accounted for 73% of the causes of death. The clinicopathologic features of ATLL in Taiwan are indistinguishable from those in HTLV-I endemic areas. The present series adds to the knowledge of the worldwide pattern of the disease.

Adult↗

PubMed1991

Production of colony-stimulating activity by circulating leukocytes from patients with chronic myeloid leukemia at various phases of the disease.

The production of colony-stimulating activity (CSA) by peripheral blood leukocytes (PBL) from 107 patients with chronic myeloid leukemia (CML), 58 at diagnosis, 34 during hematological remission, 33 in relapse from hematological control, 39 in accelerated phase, and 28 in blast phase, was measured in the double-layer agar culture system using normal nonadherent bone marrow cells as the source of CFU-GM. The median CSA levels in various stages decreased to 28-60% of control, whereas in hematological remission, there was a normalization of CSA. Five fractionation experiments using monocytes as feeder layers did not show CSA production in untreated CML. The leukemic PBL of 6 patients in blast crisis had no apparently inhibitory effect on colony formation stimulated by normal PBL. There was no correlation between the CSA of PBL and the number of CFU-GM in their bone marrow or peripheral blood at different disease states. Likewise, we failed to find a relationship between the PBL CSA levels and the total WBC counts or the differential counts in the peripheral blood as well as in the feeder layers. Our study indicated that circulating leukocytes from patients with CML at various phases except in hematological remission produce less CSA, which is not attributed to a low number of monocytes or the inhibitory effects of leukemic cells.

Adult↗