[Arthrogryposis multiplex congenita in nemaline myopathy].
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Biomedical subjects
Publications and source records attributed to Y Maeda.
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The direct antitumoral effect of interferon-alpha on human glioma was suggested in a study involving tissue culture, and scanning and transmission electron microscopy. Consequently, intratumoral local administration of interferon-alpha for four cases of human glioma was performed, and each survival period from the initiation of its application was seven, eight, three and two months. Slight direct antitumoral effect of interferon was observed in the findings of both CT scan and autopsy in our series, but the survival time was not as great as we had expected. One of the reasons for this was thought to be insufficient permeability of interferon-alpha into the residual infiltrating tumor tissue. Therefore, the use of systemic administration of interferon or another adjuvant therapy with local administration of interferon would be expected to produce better results.
The retrovirus-associated adult T-cell leukemia/lymphoma (ATL) has not been previously documented in Taiwan. Five cases identified recently by the authors are reported. Three of the patients were women, and their ages ranged from 36 to 60 years. The most important diagnostic clue was the observation of polylobated lymphoid cells in the peripheral blood. Other variably observed significant features included hypercalcemia, cutaneous eruptions, osteolytic bone lesion, hepatomegaly, and lymphadenopathy. Surface marker studies revealed that the leukemic or lymphoma cells were T-helper cells. Histopathologic examination revealed one case of pleomorphic type and three cases of medium-sized cell type. No tissue was available for study in one case. The diagnosis of ATL was confirmed by the indirect immunofluorescence test on MT-1 cell for antibodies to adult T-cell leukemia virus-associated antigen (ATLA). Three patients were dead within 6 months, and two patients had been in clinical remission for 7 and 10 months, respectively. These two latter cases were similar to the so-called smoldering type of ATL. Two descendents among nine relatives of the patients were also positive for anti-ATLA (22%). Two husbands were negative. Four of the five patients lived in the same county in northeastern coastal Taiwan, which suggested a possible clustering of ATL in that region.
A serological survey of antibodies to the adult T-cell leukemia virus-associated antigen (HTLV-A) conducted in Taiwan revealed II seropositive individuals among 2,565 serum donors tested, which included 1,187 hospital or free clinic patients, 1,065 blood donors and 313 aborigines. The overall prevalence rate was 0.43% and was 0.90% (9/995) for adults aged 40 years or over, which was similar to the rates observed in the non-endemic areas in Japan. No highly prevalent area was demonstrated. The II seropositive individuals identified were 8 hospital or clinic patients, 1 blood donor and 2 aborigines. Among 108 various leukemia/lymphoma patients also studied, 7 were positive. These 7 anti-HTLV-A-positive patients also had clinicopathologic features of adult T-cell leukemia/lymphoma (ATL). Among 21 relatives of 3 seropositive individuals in the epidemiologic study, 2 were positive; and among 25 relatives of 5 ATL patients, 6 were positive. Our data support the contention that horizontal familial transmission of human T-cell leukemia virus (HTLV-I) is an important route and HTLV-I infection can occur on a different genetic background. Furthermore, the antibody titer did not correlate with the manifestation or the state of the disease.
Incubation of dispersed cells derived from an estrogen-responsive mouse Leydig cell tumor with [3H]estradiol in the presence or absence of unlabeled estradiol showed the existence of two types of binding components with high (Kd approximately 10(-9) M) and low (Kd approximately 1.5 X 10(-8) M) affinity respectively. The use of unlabeled diethylstilbestrol as a competitor, however, abolished the low-affinity binder, resulting in the demonstration of the high binding site. This diethylstilbestrol-suppressible binding site was exclusively located in the nuclear fraction, even without hormonal stimuli. A long (5-hr) exposure of these cells to estrogen caused the decrease in the number of nuclear estrogen binding sites, which was similar to so-called 'processing' of putative nuclear estrogen receptor. The direct stimulative effect of estrogen on the proliferation of these cells was demonstrated by measuring cell numbers as well as thymidine incorporation into DNA in the cultured condition. These results would indicate that estrogen directly exerts its effects on this malignant Leydig cell through this unique nuclear binding site.
The clinical and pathologic features of cutaneous lesions observed in three adult T cell leukemia/lymphoma (ATL) patients identified in Taiwan are described. They represent one classical case of ATL and two "smoldering" variants. The classical ATL patient when first seen had numerous erythematous or purpuric papules, nodules, and plaques with or without ulceration. The two "smoldering" cases developed encrusted purpuric plaques with subcutaneous erythematous nodules in one patient and pompholyx-like vesicular eruptions with tumor masses in another patient. The pompholyx-like eruptions have not been described before. Pruritus was the major complaint in two patients. Histopathologic studies revealed pleomorphic infiltration in the classical case and monomorphic infiltration with medium-sized cells in the two "smoldering" variants. Therefore, the cutaneous lesions of ATL are diverse and not pathognomonic. The diagnosis requires a high index of suspicion, detection of circulating characteristic multilobated lymphoid cells with T helper/inducer cell marker, and demonstration of serum antibody against the adult T cell leukemia virus-associated antigen.
After N tau-methylhistidine (N tau-MH) distribution among the various organs or the tissues was determined in male broiler chickens of 15 d of age, the rates of degradation of myofibrillar proteins in male layer and broiler chickens at different stages of growth were determined by means of N tau-MH. About 75 and 8% of the total N tau-MH in the tissues occurred respectively in skeletal muscle and stomach, and most of the remainder in the intestine and the skin. The rates of degradation of myofibrillar proteins in the male layer and broiler chickens of 21, 42 and 63 d of age were calculated to be 6.1, 4.5 and 2.4%/d (layer) and 5.0, 2.8 and 0.9%/d (broiler) respectively. These calculations involve the assumption that 80% of the total excreted N tau-MH was derived from skeletal muscle. The results strongly indicate that the rapid growth of the broiler chicken is facilitated by the reduced rate of protein degradation.
1. To elucidate the causal relation between leucine and the lowering of hepatic NAD content of rats fed on a leucine-excessive diet (Yamada et al. 1979), the effect of leucine on intestinal absorption of tryptophan was investigated. 2. Co-administration of [3H]tryptophan and leucine, with leucine at ten times the level of tryptophan, delayed absorption of L-[side chain 2,3-3H]tryptophan from the digestive tract and incorporation of [3H]tryptophan into portal blood, the liver and a protein fraction of the liver. After 120 min, more than 95% of tryptophan was absorbed whether [3H]tryptophan was administered with or without leucine. 3. Co-administration of a mixture of ten essential amino acids, in proportions simulating casein, with [3H]tryptophan markedly delayed absorption of tryptophan from the digestive tract. The addition of supplementary leucine to the amino acid mixture, however, caused no further delay. 4. In rats prefed a leucine-excessive diet for 1 week [3H]tryptophan was absorbed at the same rate as in rats fed on a control diet. 5. The results indicate that competition between tryptophan and leucine for intestinal absorption did not cause lowering of hepatic NAD.
A method for purification of astroprotein (an astrocyte-specific cerebroprotein) with HPLC is described. A linear gradient from 30 to 70% acetonitrile in 0.1% trifluoroacetic acid (pH 2.2) was applied to the reversed-phase C-1 (particle size 10 micron) column. Cerebroproteins from the crude extract from human glioma were clearly separated by this procedure. Highly purified astroprotein was homogeneous by sodium dodecyl sulfate-polyacrylamide gel electrophoresis and has immunoreactivity to antiserum against astroprotein. Reversed-phase C-1 HPLC offers advantages over previously available preparative techniques in the higher purity and better separation time of the products.
The 47-year-old man reported here showed large encapsulated masses in the left cerebellopontine angle and 6 years later in the enlarged left jugular foramen. Histologically, the tumors demonstrated a large deposit of amyloid composed of immunoglobulin light chain-derived proteins (AL). There was no evidence of chronic inflammatory or infectious processes or immunoglobulin abnormalities.
The authors report the case of a 35-year-old acromegalic woman who developed amenorrhea and decreased left vision, and who was found to have suprasellar and pulmonary hemangiopericytomas. Total removal of the suprasellar hemangiopericytoma resulted in normalization of the plasma human growth hormone (GH) level and a marked decrease in size of the pulmonary hemangiopericytoma. Immunoperoxidase studies for GH and human hypothalamic growth hormone-releasing factor (GHRF) demonstrated immunoreactive intracellular GH only in the suprasellar hemangiopericytoma, with no immunoreactive intracellular GHRF evident in either the suprasellar or pulmonary hemangiopericytoma.
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