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1.
Tech Coloproctol ; 21(11): 879-886, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-29134385

RESUMO

BACKGROUND: The safety and feasibility of robotic-assisted multivisceral resection for locally advanced rectal cancer remain unclear. The aim of this study was to assess the short-term outcomes of this procedure at our institution. METHODS: From December 2011 to December 2016, patients who underwent robotic-assisted multivisceral resection for rectal cancer were investigated. Patient demographics, treatment characteristics, perioperative outcomes, and pathological results were evaluated retrospectively. RESULTS: There were 31 patients; 17 men (54.8%) and 14 women (45.2%), with a median age of 65 years (range 40-82 years). Twenty-one patients (67.7%) had a cT4 tumor, 9 patients (29.0%) had a pT4b tumor, and all patients except one (96.8%) underwent complete resection of the primary tumor with negative resection margins. Eleven patients (35.5%) received neoadjuvant chemoradiation. The most commonly resected organ was the vaginal wall (n = 12, 38.7%), followed by the prostate (n = 10, 32.3%). Lateral lymph node dissection was performed in 20 patients (64.5%). The median operative time was 394 min (range 189-549 min), and the median blood loss was 41 mL (range 0-502 mL). None of the patients received intraoperative blood transfusions or required conversion to open. Overall, postoperative complications occurred in 11 patients (35.5%). The most frequent complication was urinary retention (n = 5, 16.1%), and none of the patients developed serious complications classified as Clavien-Dindo grades III-V. CONCLUSIONS: Robotic-assisted multivisceral resection for rectal cancer is safe and technically feasible.


Assuntos
Genitália Masculina/cirurgia , Neoplasias Retais/cirurgia , Procedimentos Cirúrgicos Robóticos/métodos , Vagina/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Perda Sanguínea Cirúrgica , Feminino , Genitália Masculina/patologia , Humanos , Excisão de Linfonodo , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica , Estadiamento de Neoplasias , Duração da Cirurgia , Complicações Pós-Operatórias/etiologia , Próstata/patologia , Próstata/cirurgia , Neoplasias Retais/patologia , Estudos Retrospectivos , Procedimentos Cirúrgicos Robóticos/efeitos adversos , Glândulas Seminais/patologia , Glândulas Seminais/cirurgia , Fatores de Tempo , Resultado do Tratamento , Bexiga Urinária/patologia , Bexiga Urinária/cirurgia , Vagina/patologia , Ducto Deferente/patologia , Ducto Deferente/cirurgia
2.
Eur Surg Res ; 37(6): 370-8, 2005.
Artigo em Inglês | MEDLINE | ID: mdl-16465063

RESUMO

Matrix metalloproteinases (MMPs) have been implicated in tissue degradation in varicose veins. The aim of this study was to investigate the effects of 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors (statins) on the activity of MMPs in varicose veins. MMP-9 was present at significantly higher levels in varicose veins than in controls and was localized mainly in smooth muscle cells at the tunica media, where marked degradation of the extracellular matrix was observed. Both simvastatin and pravastatin strikingly suppressed MMP-9 activity in ex vivo culture of varicose veins. Simvastatin suppressed MMP-9 at both the mRNA and protein levels as well as at the urokinase-type plasminogen activator protein level, resulting in the dramatic suppression of MMP-9 activity induced by tumor necrosis factor-alpha. Therefore, statins suppress MMP-9 activity by multiple mechanisms in varicose veins, suggesting they may have clinical potential for the treatment of this disease.


Assuntos
Inibidores de Hidroximetilglutaril-CoA Redutases/farmacologia , Inibidores de Metaloproteinases de Matriz , Inibidores de Proteases/farmacologia , Varizes/tratamento farmacológico , Varizes/enzimologia , Idoso , Sequência de Bases , Estudos de Casos e Controles , Feminino , Humanos , Técnicas In Vitro , Masculino , Metaloproteinase 2 da Matriz/metabolismo , Metaloproteinase 9 da Matriz/genética , Pessoa de Meia-Idade , Músculo Liso Vascular/efeitos dos fármacos , Músculo Liso Vascular/enzimologia , RNA Mensageiro/genética , RNA Mensageiro/metabolismo , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Fator de Necrose Tumoral alfa/farmacologia , Ativador de Plasminogênio Tipo Uroquinase/metabolismo
3.
Microbiol Immunol ; 45(7): 543-7, 2001.
Artigo em Inglês | MEDLINE | ID: mdl-11529561

RESUMO

We identified a glucose and galactose transporter gene from the plant-pathogenic bacterium Xanthomonas oryzae pv. oryzae. Sequence analysis indicated that the gene, named glt, encoded a polypeptide of 592 amino acid residues and the product was significantly homologous with members of the Na+/glucose cotransporter (SGLT) family from mammalian and bacterial origin, especially with vSGLT from Vibrio parahaemolyticus (50% identity). GLT functioned as a glucose and galactose transporter in an Escherichia coli mutant deficient in glucose and galactose transport activity. A protonophore inhibited the transport activity, suggesting that GLT is a H+-coupled glucose/galactose symporter.


Assuntos
Proteínas de Bactérias , Galactose/metabolismo , Glucose/metabolismo , Proteínas de Membrana/genética , Proteínas de Transporte de Monossacarídeos/genética , Oryza/microbiologia , Xanthomonas/genética , Sequência de Aminoácidos , Sequência de Bases , Proteínas de Membrana/química , Proteínas de Membrana/metabolismo , Dados de Sequência Molecular , Proteínas de Transporte de Monossacarídeos/química , Proteínas de Transporte de Monossacarídeos/metabolismo , Doenças das Plantas/microbiologia , Simportadores , Xanthomonas/metabolismo
4.
Nihon Hoigaku Zasshi ; 50(6): 412-5, 1996 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-8997086

RESUMO

The well-known Minamata disease was caused by mercury; the Itai-Itai disease by cadmium; and lead poisoning by gasoline additives. Following our previous investigation on heavy metal concentrations in the umbilical cords in 1980, total mercury, cadmium and lead concentrations in the umbilical cords (from 20 males and 20 females) have been measured in 1990 for comparison in the present study. The changes in metal concentrations in 1980 and 1990 were: from 0.007 +/- 0.005 microgram/g to 0.011 +/- 0.008 microgram/g for total mercury; from 0.019 +/- 0.016 microgram/g to 0.006 +/- 0.005 microgram/g, from 0.151 +/- 0.123 microgram/g to 0.046 +/- 0.038 microgram/g for lead. Total mercury accumulated in the umbilical cords increased to approximately 1.6-fold, while cadmium and lead decreased to approximately 1/3-fold during these 10 years.


Assuntos
Cádmio/análise , Chumbo/análise , Mercúrio/análise , Cordão Umbilical/química , Adulto , Feminino , Humanos , Masculino
5.
J Cardiovasc Pharmacol ; 27(6): 824-30, 1996 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-8761849

RESUMO

We examined the effect of an antiplatelet agent, E5510, which inhibits both platelet aggregation and release of platelet-derived growth factor (PDGF), on anastomotic intimal hyperplasia and platelet aggregation. Twenty Beagle dogs underwent infrarenal aortic reconstruction with an expanded polytetrafluoroethylene (ePTFE) graft 5 mm in diameter and 3 cm long. The dogs were divided into three groups: placebo (control group, 7 dogs), E5510 1 mg/day (1-mg group, 6 dogs), and E5510 4 mg/day (4-mg group, 7 dogs). E5510 was administered orally 2 h before operation and once daily for 3 months after operation. Grafts were harvested 3 months after operation. All 13 grafts in the treated groups remained patent without evidence of intimal hyperplasia, whereas only 4 of 7 grafts (57%) remained patent in the control group, including 1 graft with > 50% stenosis. Three occluded grafts showed severe intimal hyperplasia at the anastomoses. The platelet aggregation ratio (PAR) with collagen (100 micrograms/ml) before drug administration at 3 months in the 4-mg group was significantly lower than that in the control and 1-mg groups. PAR after drug administration at 3 months in the 1- and 4-mg groups was significantly lower than that in the control group. Intimal thickness at the distal anastomosis was 817 +/- 190 microns in the control group, 240 +/- 80 microns in the 1-mg group, and 197 +/- 28 microns in the 4-mg group. Intimal thickness in the control group was significantly greater than that in the 1- and 4-mg groups. Smooth muscle cell (SMC) values in the intima at the distal anastomosis were 65.6 +/- 4.4% extinction (%E) in the control group, 47.6 +/- 3.4%E in the 1-mg group, and 51.3 +/- 3.5%E in the 4-mg group. SMC value in the control group was significantly greater than that in the 1- and 4-mg groups. E5510 inhibited PAR and reduced the degree of anastomotic intimal hyperplasia.


Assuntos
Anastomose Cirúrgica/efeitos adversos , Aorta/patologia , Ácidos Graxos Monoinsaturados/farmacologia , Inibidores da Agregação Plaquetária/farmacologia , Túnica Íntima/patologia , Animais , Aorta/cirurgia , Plaquetas/efeitos dos fármacos , Cães , Feminino , Hiperplasia/etiologia , Hiperplasia/prevenção & controle , Túnica Íntima/efeitos dos fármacos
6.
Surg Today ; 26(9): 756-9, 1996.
Artigo em Inglês | MEDLINE | ID: mdl-8883257

RESUMO

A 47-year-old woman was admitted to our hospital with a giant spinal arteriovenous malformation (AVM) causing heart failure and thoracic myelopathy. Angiography revealed that the spinal AVM had multiple feeding vessels branching from the 5th through 12th intercostal arteries. The drainage vein flowed to the azygos vein and superior vena cava. The AVM destroyed the 7th thoracic vertebra. The cardiac output was 16.7l/min and the shunt ratio was 64% before treatment. Embolization with cyanoacrylate was performed because the operation was considered to be associated with a significant risk of paraplegia and organ ischemia. The cardiac output decreased to 11.6l/min and the shunt ratio was reduced to 32%. After embolization the patient demonstrated no symptoms of either heart failure or sensory deficits. During embolization, provocative tests using sodium amytal and lidocaine with magnetic stimulation were also performed. The above findings suggest that provocative tests and magnetic stimulation are useful to predict paraplegia, which could result from embolization while, in addition, embolization is considered to be a useful treatment for multiple shunt and nidus in this region.


Assuntos
Malformações Arteriovenosas/terapia , Embolização Terapêutica , Doenças da Coluna Vertebral/terapia , Angiografia , Malformações Arteriovenosas/complicações , Malformações Arteriovenosas/diagnóstico por imagem , Malformações Arteriovenosas/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Doenças da Coluna Vertebral/complicações , Doenças da Coluna Vertebral/diagnóstico por imagem , Doenças da Coluna Vertebral/patologia , Tomografia Computadorizada por Raios X
7.
Surg Today ; 26(2): 115-8, 1996.
Artigo em Inglês | MEDLINE | ID: mdl-8919281

RESUMO

Systolic ankle pressure was measured in limbs in the extended and 90 degrees-flexed positions in three groups of patients who had undergone below-knee femorodistal arterial bypass. Group 1 consisted of eight limbs in seven patients who had received an autologous saphenous vein (ASV) graft; group 2 consisted of seven limbs in seven patients who had received a ringed expanded polytetrafluorethylene (ePTFE) graft; and group 3 consisted of five limbs in five patients who had received a reinforced biosynthetic borine collagen vascular (Omniflow) graft. Intravenous digital subtraction angiography was performed within 3 months of implantation. The ankle/brachial index (ABI) was significantly lower when the knee was flexed than when it was extended in group 1 and 3, but not in group 2. The ABI flexion in group 1 was similar to that in group 2. Morphologic changes such as kinking were demonstrated angiographically in groups 1 and 3. Given that the ABIs in groups 1 and 2 were similar with the knee flexed, despite the morphologic changes in group 1, we concluded that saphenous vein and ringed ePTFE grafts are more durable when subjected to acute knee flexion than Omniflow grafts. Thus, as graft failure is unlikely to result from kinking as a result of knee flexion alone, further clinical studies are required to elucidate the precise causes of graft failure.


Assuntos
Tornozelo/irrigação sanguínea , Arteriopatias Oclusivas/cirurgia , Pressão Sanguínea/fisiologia , Isquemia/cirurgia , Contração Isométrica/fisiologia , Perna (Membro)/irrigação sanguínea , Complicações Pós-Operatórias/fisiopatologia , Idoso , Angiografia Digital , Arteriopatias Oclusivas/diagnóstico por imagem , Arteriopatias Oclusivas/fisiopatologia , Bioprótese , Prótese Vascular , Feminino , Seguimentos , Oclusão de Enxerto Vascular/diagnóstico por imagem , Oclusão de Enxerto Vascular/fisiopatologia , Humanos , Isquemia/diagnóstico por imagem , Isquemia/fisiopatologia , Masculino , Pessoa de Meia-Idade , Politetrafluoretileno , Complicações Pós-Operatórias/diagnóstico por imagem , Veia Safena/transplante , Sístole/fisiologia
8.
Nihon Hoigaku Zasshi ; 49(6): 466-71, 1995 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-8583690

RESUMO

The concentration and component types of PCB in adult human liver and fat tissue (64 samples) from forensic autopsy cases of Osaka Prefecture in 1989 were measured for comparison with the data for the same organ and tissue during the period from 1974 to 1984. The mean values of PCB levels in 1989 were 0.06 microgram/g (whole base) in the liver and 2.20 micrograms/g (fat base) in the fat tissue. These concentrations both were the same as those observed for the 15-year period. The percentages of the 5,6-chlorinated compound of PCB in the liver and fat tissue were lower but those of the 7-chlorinated compound were 15-20% higher.


Assuntos
Poluentes Ambientais/farmacocinética , Medicina Legal , Bifenilos Policlorados/farmacocinética , Tecido Adiposo/metabolismo , Adulto , Feminino , Humanos , Japão , Fígado/metabolismo , Masculino , Fatores de Tempo , Distribuição Tecidual
9.
Nihon Hoigaku Zasshi ; 49(5): 294-8, 1995 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-8551696

RESUMO

A method for estimating the age at death through the image processing of soft X-ray pictures of pubic symphysis is reported. The collected 533 materials were sliced at the thickness of 5 mm. Prior to the image processing, subjective assessment was made on the five indices of gross morphological findings. The pictures showed roughening and darkening tendencies with age and two indices for image processing were introduced. Applying the natural logarithm to data obtained by the image processing and making regression analysis. two kinds of age estimation equations were derived in the form of; Y = -39.91 lnX + 189.63 and the estimated age Y was proportional to natural logarithm of measured indices X. The age estimation equations showed accuracies of 70% or more for the age range of +/- 5 years and 100% for +/- 8 years. The accuracy was satisfactory for age estimation in the field of person appraisal in legal medicine.


Assuntos
Determinação da Idade pelo Esqueleto/métodos , Processamento de Imagem Assistida por Computador , Sínfise Pubiana/diagnóstico por imagem , Adulto , Idoso , Povo Asiático , Feminino , Humanos , Japão , Masculino , Pessoa de Meia-Idade , Análise de Regressão , Sensibilidade e Especificidade
10.
Nihon Hoigaku Zasshi ; 49(4): 231-5, 1995 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-7563941

RESUMO

A method was developed for estimating the age at death based on image processing of soft X-ray pictures of thyroid cartilage from Japanese males. Data for 501 individuals were used to derive equations for this determination. Prior to image processing, gross morphological findings were used for estimation. Soft X-ray pictures indicated ossification with age and one index for image processing was introduced. The exponential function was applied to age estimation.


Assuntos
Determinação da Idade pelo Esqueleto/métodos , Cartilagem Tireóidea/diagnóstico por imagem , Adulto , Idoso , Povo Asiático , Humanos , Processamento de Imagem Assistida por Computador , Japão , Masculino , Pessoa de Meia-Idade , Osteogênese , Intensificação de Imagem Radiográfica , Sensibilidade e Especificidade , Caracteres Sexuais , Cartilagem Tireóidea/fisiologia
11.
Nihon Hoigaku Zasshi ; 49(4): 236-41, 1995 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-7563942

RESUMO

A method for estimating the age at death using the image processing of soft X-ray pictures of the thyroid cartilages of Japanese females was examined. The soft X-ray pictures showed increasing ossification with age. One index for image processing was introduced. It is ossification rate (OSFR). Corresponding to this index, one age estimation equation was obtained. This age estimation equation showed accuracies of 60% or more for the age range of +/- 5 years and about 80% for the age range of +/- 8 years.


Assuntos
Determinação da Idade pelo Esqueleto/métodos , Cartilagem Tireóidea/diagnóstico por imagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Povo Asiático , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Japão , Pessoa de Meia-Idade , Osteogênese , Intensificação de Imagem Radiográfica , Caracteres Sexuais , Cartilagem Tireóidea/fisiologia
12.
Nihon Hoigaku Zasshi ; 49(4): 260-4, 1995 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-7563946

RESUMO

We collected data from 30 autopsied cases, in which the heart weight were 800 g or more (maximum 1,300 g), at the Osaka Medical Examiner's office, for past 10 years. Among these cases, the cause of death was cardiac failure in 17 cases (56.7%), followed by pulmonary diseases (3 cases, 10.0%), cerebral infarction (3 cases, 10.0%), and aortic rupture (2 cases, 6.6%). The mean weight of the giant hearts was 880 g (about 77% of the cases) and 4 cases (13%) had 1,000 g or more. The mean height of the giant heart victims was close to the standard level, and thus the mean level of the ratio of heart weight to height were about 2.5 times higher than the standard levels. The liver weights were also greater by 500-1,000 g than the standard levels.


Assuntos
Cardiomegalia/mortalidade , Adulto , Idoso , Estatura , Cardiomegalia/patologia , Causas de Morte , Feminino , Humanos , Japão , Fígado/patologia , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Tamanho do Órgão
13.
Surg Today ; 24(7): 627-30, 1994.
Artigo em Inglês | MEDLINE | ID: mdl-7949771

RESUMO

We report herein the case of a 92-year-old man with an abdominal aortic aneurysm who underwent successful surgical resection using a ringed graft. His postoperative course was uneventful, and he was discharged after 42 days, following which he attended the outpatient department twice a month for regular check-ups. He finally died of pneumonia which developed from an upper respiratory tract infection 6 months after his operation. Thus, surgical treatment should always be considered when an aneurysm is detected, even in very aged patients for whom the activities of daily living are possible.


Assuntos
Aneurisma da Aorta Abdominal/cirurgia , Prótese Vascular/métodos , Idoso , Idoso de 80 Anos ou mais , Humanos , Masculino
15.
Nihon Hoigaku Zasshi ; 47(2): 153-9, 1993 Apr.
Artigo em Japonês | MEDLINE | ID: mdl-8315870

RESUMO

With the development of the mining and manufacturing industries and the petrochemical industry, cadmium (Cd) has been used not only for metal products but also petroleum products. Recently, environmental pollution due to waste fluids and gases from factories using cadmium and also domestic waste materials has become a cause for concern. In this connection, studies on the accumulated levels of Cd in the human body, its age-distribution in the general population and its secular changes during the last decade would be useful for clarifying the degree of contamination in the human body and providing an index which could be used as a basis for preventive measures against environmental pollution. For this purpose, the levels of Cd accumulated in main organs of the human body were determined using forensic data 3 times at 5-year intervals starting in 1976. The age intervals starting in 1976. The age group having the highest Cd level was 50-59 years, old and the mean weights of Cd per wet weight of heart, liver and kidney were 0.421, 2,984 and 56.43 micrograms/g, respectively. Each of these organs in babies less than a year old exhibited lower Cd levels, but teenagers had levels close to those in adult. However, the level in each organ was lower in individuals over 60 years old. 4 distinct age-related increase in the level of Cd was found only in the kidney. The levels of accumulated Cd in the liver and kidney showed a rise during the last 10 years from 2.358 to 5.568 micrograms/g and from 43.95 to 73.47 micrograms/g, respectively, whereas the Cd level in the heart has remained about the same, i.e. from 0.207 to 0.181 micrograms/g.


Assuntos
Cádmio/farmacocinética , Vísceras/química , Adolescente , Adulto , Fatores Etários , Criança , Pré-Escolar , Poluição Ambiental/análise , Feminino , Humanos , Lactente , Recém-Nascido , Japão , Masculino , Pessoa de Meia-Idade
16.
Nihon Hoigaku Zasshi ; 47(2): 147-52, 1993 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-8315869

RESUMO

As environmental pollution due to waste fluids and gases from factories using lead (Pb), as well as domestic waste materials, has become a matter for public concern, studies on the accumulated levels of Pb in man have become necessary for defining the degree of contamination in the ordinary population. For this purpose, Pb levels in tissues of 76 forensic cadavers were determined in order to investigate the age distribution of accumulated Pb, and 120 samples were used to clarify secular changes in Pb accumulation during the last decade in the general population. The age group showing the highest Pb level was 50-59 years, with mean Pb contents per unit wet weight of 0.262, 0.520, 0.427 and 4.479 micrograms/g for heart, liver, kidney and rib, respectively. Each of these organs in all age groups exhibited almost the same Pb levels, but babies less than one year old had levels close to those in adults. However, ribs in babies exhibited much lower Pb levels. The levels in each organ were lower in individuals over 60 years old. A distinct age-related increase in Pb levels was found only in ribs from age 0 year to 50-59 years. The Pb levels in the heart, liver, kidney and rib showed a decrease during the last 10 years from 0.188 +/- 0.073 to 0.006 +/- 0.003 micrograms/g, from 0.307 +/- 0.169 to 0.076 +/- 0.057 micrograms/g, from 0.287 +/- 0.145 to 0.021 +/- 0.011 micrograms/g and from 3.203 +/- 1.477 to 2.366 +/- 1.469 micrograms/g, respectively.


Assuntos
Chumbo/farmacocinética , Costelas/química , Vísceras/química , Adolescente , Adulto , Fatores Etários , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Japão , Masculino , Pessoa de Meia-Idade
17.
Nihon Geka Gakkai Zasshi ; 93(9): 1043-5, 1992 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-1470113

RESUMO

The purpose of this study was to determine the operative indication for patients with intermittent claudication because of arteriosclerosis obliterans, and to compare the late result of the surgical treatment group (182 cases, 250 limbs) with that of the non-surgical treatment group (35 cases, 43 limbs). There were 3 operative deaths and 34 late deaths in the surgical group, and 14 late deaths in the non-surgical group. The number one cause of death was heart failure, including ischemic heart disease, in both groups. The 4-year cumulative patency rates were 91% in the aorto-iliac, 71% in the femoro-distal and 79% in the aorto-femoro-distal arterial reconstructions. Long term symptom free rates of the surgical group and the non-surgical group, except fatal case, were 83% and 44% in the aorto-iliac, 79% and 27% in the femoro-distal, 87% and 33% in the aorto-femoro-distal arterial regions, respectively. Late result of intermittent claudication in the surgical group was better than that in the non-surgical group. We concluded that intermittent claudication should be considered to be the indication for surgery, except for the cases with high risk diseases or malignant diseases.


Assuntos
Angioplastia , Claudicação Intermitente/terapia , Idoso , Arteriosclerose Obliterante/complicações , Arteriosclerose Obliterante/mortalidade , Arteriosclerose Obliterante/terapia , Feminino , Seguimentos , Humanos , Claudicação Intermitente/etiologia , Claudicação Intermitente/mortalidade , Masculino , Pessoa de Meia-Idade , Prognóstico , Taxa de Sobrevida , Grau de Desobstrução Vascular
18.
Nippon Ganka Gakkai Zasshi ; 95(8): 795-801, 1991 Aug.
Artigo em Japonês | MEDLINE | ID: mdl-1950838

RESUMO

A case of immotile cilia syndrome accompanied by retinitis pigmentosa is reported. This syndrome involves congenital ciliary ultrastructural abnormality. A 27-year-old male complained of repeated pneumonia, sinusitis, and middle otitis. In addition, he had sperm motor insufficiency and electron microscopic finding of cilia led to the diagnosis of the present syndrome. Both fundi presented remarkable degeneration of retinal pigment epithelium and choroid and marked arterial narrowing. Constriction of the visual field and extinguished ERG were also noted. Abnormality of cilia of the retinal pigment epithelium was suggested. It was proposed that retinitis pigmentosa may be caused by abnormal cilia of the retinal pigment epithelium.


Assuntos
Transtornos da Motilidade Ciliar/complicações , Retinose Pigmentar/complicações , Adulto , Humanos , Masculino
19.
J UOEH ; 12(4): 411-8, 1990 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-2287841

RESUMO

Glycogenosis Type III is characterized by a deficiency of debranching enzyme (amylo-1,6-glucosidase, E.C. 3. 2. 1. 33) in most tissues. Low activity of liberating glucose from limited dextrin in the biopsied muscle can be demonstrated in a patient with this disease. We cultured fibroblasts from a skin biopsy from a patient with debrancher deficiency and examined the metabolism of glycogen in these cultured fibroblasts. Debrancher activity in the post-mitochondrial supernatant obtained from these fibroblasts showed a good concentration dependent manner but had approximately half of that from normal human fibroblasts (YH-1). Although the enzymatic activity of debrancher in the cultured fibroblasts from the skin was reduced essentially to the same levels as observed in muscle biopsy, little glycogen granules were accumulated in the cytoplasm of these fibroblasts as revealed by either light- or electron-microscopic observation. The fibroblasts obtained in the present study may be useful for the analysis of molecular mechanism of the debrancher deficiency disease, glycogenosis Type III.


Assuntos
Doença de Depósito de Glicogênio Tipo III/metabolismo , Glicogênio/metabolismo , Pele/metabolismo , Adolescente , Células Cultivadas , Fibroblastos/metabolismo , Sistema da Enzima Desramificadora do Glicogênio/metabolismo , Doença de Depósito de Glicogênio Tipo III/enzimologia , Histocitoquímica , Humanos , Masculino , Mitocôndrias/metabolismo , Pele/citologia
20.
Exp Cell Res ; 189(2): 227-32, 1990 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-1695156

RESUMO

Rapidly growing cells usually have high levels of ribosome biogenesis. The sequential expression of protooncogenes during the transition of quiescent hepatocytes to the replicative stage was assumed to be followed by activation of cellular genes related to cell growth such as ribosome biosynthesis. First, the expression of major nucleolar protein (nucleolin or C23) and major heat-shock protein (hsp 70) genes was examined during rat liver regeneration. hsp 70 may function in cell growth and has a characteristic nucleolar location after heat shock. Both nucleolin and hsp 70 mRNA began to increase simultaneously after peaks of c-fos and c-myc, showed a peak 6 h after partial hepatectomy, and declined to the control levels around 20 h. That is, the peaks of nucleolin and hsp 70 mRNA precede the peak of ribosome formation (12-20 h) and DNA replication (24 h). Second, the behavior of nucleolin and hsp 70 mRNA was examined in primary cultured hepatocytes during their G0-G1 transition. Although the amounts of c-myc mRNA reached a plateau around 20 h after the initiation of culture and remained at these levels, DNA synthesis has never been found to start without the addition of EGF and insulin to this system. Both nucleolin and hsp 70 mRNA began to increase at around 20 h (prereplicative stage) and simultaneously decreased in inverse proportion to DNA synthesis induced by these growth factors. Thus, it is possible that the simultaneous enhancement of nucleolin and hsp 70 genes as described above is not merely coincidental, but is important biologically during the transition of quiescent hepatocytes to proliferative cells.


Assuntos
Regulação da Expressão Gênica , Proteínas de Choque Térmico/genética , Fígado/metabolismo , Proteínas Nucleares/genética , Fosfoproteínas/genética , Proto-Oncogenes , Proteínas de Ligação a RNA , Animais , Células Cultivadas , Cinética , Fígado/citologia , Regeneração Hepática , Masculino , Proteínas Tirosina Quinases/genética , Proteínas Proto-Oncogênicas/genética , Proteínas Proto-Oncogênicas c-fos , Proteínas Proto-Oncogênicas c-myc , RNA/genética , RNA/isolamento & purificação , RNA Mensageiro/genética , Ratos , Ratos Endogâmicos , Nucleolina
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