RESUMEN
BACKGROUND: The appropriate surgical procedure for patients with upper third early gastric cancer is controversial. We compared total gastrectomy (TG) with proximal gastrectomy (PG) in this patient population. METHODS: A multicenter, non-randomized trial was conducted, with patients treated with PG or TG. We compared short- and long-term outcomes between these procedures. RESULTS: Between 2009 and 2014, we enrolled 254 patients from 22 institutions; data from 252 were included in the analysis. These 252 patients were assigned to either the PG (n = 159) or TG (n = 93) group. Percentage of body weight loss (%BWL) at 1 year after surgery, i.e., the primary endpoint, in the PG group was significantly less than that of the TG group (- 12.8% versus - 16.9%; p = 0.0001). For short-term outcomes, operation time was significantly shorter for PG than TG (252 min versus 303 min; p < 0.0001), but there were no group-dependent differences in blood loss and postoperative complications. For long-term outcomes, incidence of reflux esophagitis in the PG group was significantly higher than that of the TG group (14.5% versus 5.4%; p = 0.02), while there were no differences in the incidence of anastomotic stenosis between the two (5.7% versus 5.4%; p = 0.92). Overall patient survival rates were similar between the two groups (3-year survival rates: 96% versus 92% in the PG and TG groups, respectively; p = 0.49). CONCLUSIONS: Patients who underwent PG were better able to control weight loss without worsening the prognosis, relative to those in the TG group. Optimization of a reconstruction method to reduce reflux in PG patients will be important.
Asunto(s)
Gastrectomía/métodos , Neoplasias Gástricas/cirugía , Estómago/cirugía , Adulto , Anciano , Anciano de 80 o más Años , Anastomosis Quirúrgica , Femenino , Gastrectomía/mortalidad , Humanos , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Tempo Operativo , Pronóstico , Estudios Prospectivos , Estómago/patología , Neoplasias Gástricas/mortalidad , Neoplasias Gástricas/patología , Tasa de Supervivencia , Resultado del Tratamiento , Pérdida de PesoRESUMEN
Microbeam radiotherapy (MRT) is the delivery of spatially fractionated beams that have the potential to offer significant improvements in the therapeutic ratio due to the delivery of micron-sized high dose and dose rate beams. They build on longstanding clinical experience of GRID radiotherapy and more recently lattice-based approaches. Here we briefly overview the preclinical evidence for MRT efficacy and highlight the challenges for bringing this to clinical utility. The biological mechanisms underpinning MRT efficacy are still unclear, but involve vascular, bystander, stem cell and potentially immune responses. There is probably significant overlap in the mechanisms underpinning MRT responses and FLASH radiotherapy that needs to be further defined.
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Oncología por Radiación , Radiobiología , Humanos , RadioterapiaRESUMEN
Dystrophin is associated with several novel sarcolemmal proteins, including a laminin-binding extracellular glycoprotein of 156 kD (alpha-dystroglycan) and a transmembrane glycoprotein of 50 kD (adhalin). Deficiency of adhalin characterizes a severe autosomal recessive muscular dystrophy prevalent in Arabs. Here we report for the first time two mongoloid (Japanese) patients with autosomal recessive muscular dystrophy deficient in adhalin. Interestingly, adhalin was not completely absent and was faintly detectable in a patchy distribution along the sarcolemma in our patients. Although the M and B2 subunits of laminin were preserved, the B1 subunit was greatly reduced in the basal lamina surrounding muscle fibers. Our results raise a possibility that the deficiency of adhalin may be associated with the disturbance of sarcolemma-extracellular matrix interaction leading to sarcolemmal instability.
Asunto(s)
Proteínas del Citoesqueleto/deficiencia , Matriz Extracelular/fisiología , Laminina/análisis , Glicoproteínas de Membrana/deficiencia , Distrofias Musculares/metabolismo , Sarcolema/fisiología , Adulto , Distrofina/análisis , Humanos , Inmunohistoquímica , Laminina/química , Masculino , Distrofias Musculares/genética , SarcoglicanosRESUMEN
Textured ferromagnetic Fe nanowire arrays were electrodeposited using a rectangular-pulsed potential deposition technique into anodized aluminum oxide nanochannels. During the electrodeposition of Fe nanowire arrays at a cathodic potential of - 1.2 V, the growth rate of the nanowires was ca. 200 nm s-1. The aspect ratio of Fe nanowires with a diameter of 30 ± 5 nm reached ca. 2000. The long axis of Fe nanowires corresponded with the <200> direction when a large overpotential during the on-time pulse was applied, whereas it orientated to the <110> direction under the potentiostatic condition with a small overpotential. By shifting the on-time cathode potential up to - 1.8 V, the texture coefficient for the (200) plane, TC200, reached up to 1.94. Perpendicular magnetization performance was observed in Fe nanowire arrays. With increasing TC200, the squareness of Fe nanowire arrays increased up to 0.95 with the coercivity maintained at 1.4 kOe at room temperature. This research result has opened a novel possibility of Fe nanowire arrays that can be applied for a new permanent magnetic material without rare-earth metals.
RESUMEN
We recently reported the selective reduction of the B1 subunit of laminin in two Japanese patients with adhalin deficiency. We here investigated immunohistochemically the expression of other components of the extracellular matrix (ECM), including collagen type IV, heparan sulfate proteoglycan can (HSPG), chondroitin-4-sulfate proteoglycan, decorin, and fibronectin in adhalin deficiency, compared with other types of muscular dystrophy. We found a reduction of HSPG on the basal lamina surrounding each muscle fiber in adhalin deficiency compared with HSPG in other diseases. This finding may be characteristic evidence of the disturbance of the sarcolemma-ECM interaction and the sarcolemmal instability in adhalin deficiency. Recently, a direct role of HSPG in fibroblast growth factor (FGF) signal transduction was demonstrated. Further investigation is required to determine if the dysfunction of FGF is relevant to the pathogenesis of adhalin deficiency.
Asunto(s)
Proteínas del Citoesqueleto/deficiencia , Heparitina Sulfato/biosíntesis , Glicoproteínas de Membrana/deficiencia , Fibras Musculares Esqueléticas/metabolismo , Adolescente , Adulto , Niño , Preescolar , Matriz Extracelular/metabolismo , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Proteínas Musculares/biosíntesis , Distrofias Musculares/metabolismo , Sarcoglicanos , Sarcolema/metabolismoRESUMEN
The effect of hydrogen bonding at hetero atoms of oxidized flavin on its electron acceptability was studied by the ab initio molecular orbital method. The calculations were carried out for all possible lumiflavin-H2O complexes and for some lumiflavin-formamide complexes. Calculated data showed that the magnitudes of hydrogen bonding energy at the hetero atoms are in the order of N(3)H greater than N(5) greater than O(12) greater than N(1) greater than O(14). It was found that the atomic orbital coefficient of the lowest unoccupied molecular orbital is the largest at N(5) and that hydrogen bonding at N(1), N(5), O(12), and O(14) increases the electron acceptability of the oxidized flavin at N(5), while hydrogen bonding at N(3)H decreases it.
Asunto(s)
Flavinas , Fenómenos Químicos , Química Física , Electrones , Enlace de Hidrógeno , Modelos Químicos , Oxidación-Reducción , Teoría CuánticaRESUMEN
A case of nemaline myopathy with ophthalmoplegia is reported. The patient was a 35-year-old man born of consanguineous parents. He had a myopathic face, high-arched palate, nasal voice, scoliosis, very thin trunk and marked muscle weakness involving face, neck, limbs and trunk. He also had ptotis of the left eyelid and mild bilateral ophthalmoplegia, also detected by electrooculogram. Biopsy of gastrocnemius muscle revealed nemaline rods. At the ultrastructural level, the rods appeared to have axial and cross striations, and in cross-sections at high magnification they seemed to have a crystal lattice structure. Intranuclear rods were also observed. In addition to the rods, abnormal mitochondria including a number of paracrystalline inclusions were seen.
Asunto(s)
Mitocondrias Musculares/ultraestructura , Enfermedades Musculares/congénito , Oftalmoplejía/congénito , Adulto , Biopsia , Blefaroptosis/congénito , Núcleo Celular/ultraestructura , Electrooculografía , Humanos , Cuerpos de Inclusión/ultraestructura , Masculino , Músculos/patología , Enfermedades Musculares/patología , NAD/metabolismoRESUMEN
We describe here a 10-year-old patient with high levels of serum IgE and inflammatory myopathy whose muscle fibers exhibit excessive autophagy. Previous studies have demonstrated surface expression of class I MHC antigens on muscle fibers from patients with inflammatory myopathy. The muscle fibers of this patients showed marked expression of both class I and class II MHC antigens. The reaction products were demonstrated not only on sarcolemma but also in and around some vacuoles. Both CD4-positive and CD8-positive T-lymphocytes were noted in inflammatory exudates surrounding these fibers but B-lymphocytes were rare. We hypothesize that myocyte expression of both class I and class II antigens may play a role in the pathogenesis of this new type of inflammatory myopathy.
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Enfermedades Autoinmunes/inmunología , Antígenos HLA/análisis , Antígenos HLA-DR/análisis , Músculos/inmunología , Miositis/inmunología , Enfermedades Autoinmunes/tratamiento farmacológico , Enfermedades Autoinmunes/patología , Niño , Humanos , Inmunoglobulina E/sangre , Cuerpos de Inclusión Viral , Masculino , Músculos/patología , Miositis/tratamiento farmacológico , Miositis/patología , Fagocitosis , Prednisolona/uso terapéutico , VacuolasRESUMEN
We have reported the reduction of the B1 subunit of laminin and that of heparan sulfate proteoglycan (HSPG) in two Japanese patients with adhalin deficiency. We here investigated immunohistochemically the expression of cell adhesion molecules, including intercellular adhesion molecule-1 (ICAM-1), neural cell adhesion molecule (NCAM), and CD44 (HCAM), in four Japanese patients with adhalin deficiency, compared to other types of muscular dystrophy. We found that NCAM was upregulated in a fair number of muscle fibers, regardless of the type of muscular dystrophy. ICAM-1 was detected on the rare muscle cell membrane in all patients. CD44 was barely detected on the muscle cell membrane in adhalin deficiency, in contrast to the strong expression of CD44 which was observed in other types of muscular dystrophy. These findings suggest that a different degenerative or regenerative process is involved in adhalin deficiency compared to other types of muscular dystrophy.
Asunto(s)
Moléculas de Adhesión Celular Neuronal/biosíntesis , Proteínas del Citoesqueleto/deficiencia , Molécula 1 de Adhesión Intercelular/biosíntesis , Glicoproteínas de Membrana/deficiencia , Adulto , Biopsia , Moléculas de Adhesión Celular Neuronal/análisis , Proteínas de la Matriz Extracelular/análisis , Proteínas de la Matriz Extracelular/biosíntesis , Femenino , Humanos , Receptores de Hialuranos/análisis , Receptores de Hialuranos/biosíntesis , Inmunohistoquímica , Molécula 1 de Adhesión Intercelular/análisis , Masculino , Persona de Mediana Edad , Fibras Musculares Esqueléticas/química , Fibras Musculares Esqueléticas/metabolismo , Fibras Musculares Esqueléticas/patología , Músculo Esquelético/química , Músculo Esquelético/metabolismo , Músculo Esquelético/patología , Distrofias Musculares/metabolismo , Distrofias Musculares/patología , SarcoglicanosRESUMEN
A new homozygous alpha-sarcoglycan (adhalin) gene mutation was found in a Japanese patient with severe childhood autosomal recessive muscular dystrophy (SCARMD). Muscle biopsy specimens from the patient showed marked reduction but not complete deficiency of alpha-sarcoglycan. The sequence of part of exon 3 of the alpha-sarcoglycan gene exhibited a cytosine to thymidine substitution at nucleotide position 220. Since the same mutation was not found in 100 normal control samples, this new alpha-sarcoglycan gene mutation is not a polymorphism but is presumed to be responsible for the marked reduction of alpha-sarcoglycan in skeletal muscle. Most patients with homozygous alpha-sarcoglycan gene mutation were reported to show complete alpha-sarcoglycan deficiency. Present case showed the homozygous missense mutation of alpha-sarcoglycan and associated with incomplete alpha-sarcoglycan deficiency and severe clinical phenotype.
Asunto(s)
Proteínas del Citoesqueleto/deficiencia , Proteínas del Citoesqueleto/genética , Glicoproteínas de Membrana/deficiencia , Glicoproteínas de Membrana/genética , Distrofias Musculares/genética , Mutación/fisiología , Potenciales de Acción/efectos de los fármacos , Potenciales de Acción/fisiología , Adulto , Aberraciones Cromosómicas , Trastornos de los Cromosomas , Electromiografía , Genes Recesivos/genética , Humanos , Inmunohistoquímica , Masculino , Músculo Esquelético/metabolismo , Músculo Esquelético/patología , Distrofias Musculares/metabolismo , Distrofias Musculares/patología , Conducción Nerviosa/fisiología , Reacción en Cadena de la Polimerasa , SarcoglicanosRESUMEN
Thalamic- and cerebellar-projecting interpolaris neuron responses to afferent inputs from the temporomandibular joint (TMJ) and/or the masseter muscle (Mm) were examined in rats. Of 230 neurons tested, 24 could be antidromically stimulated from the contralateral ventral posteromedial thalamic nucleus (VPM), and 27 of 91 neurons tested were stimulated from the ipsilateral posteromedial part of crus II of the cerebellar cortex. None had dual projections. The thalamic-projecting neurons were recorded in the dorsomedial region of the interpolaris; most cerebellar-projecting neurons were at the medial border of the interpolaris. Ten of 24 thalamic- and 17 of 27 cerebellar-projecting neurons received nociceptive information. Afferent inputs from the TMJ and the Mm converged on 6 of 24 thalamic-projecting neurons and on 16 of 27 cerebellar-projecting neurons. In both the thalamic- and cerebellar-projecting neurons, there was no difference between the non-nociceptive and nociceptive neurons in mean antidromic latency. The results suggest that the interpolaris integrates and relays afferent inputs from deep oral structures.
Asunto(s)
Vías Aferentes/fisiología , Cerebelo/fisiología , Músculo Masetero/inervación , Neuronas/fisiología , Articulación Temporomandibular/inervación , Tálamo/fisiología , Vías Aferentes/anatomía & histología , Vías Aferentes/citología , Animales , Corteza Cerebelosa/anatomía & histología , Corteza Cerebelosa/citología , Corteza Cerebelosa/fisiología , Cerebelo/anatomía & histología , Cerebelo/citología , Estimulación Eléctrica , Potenciales Evocados , Femenino , Neuronas/citología , Ratas , Ratas Wistar , Piel/inervación , Tálamo/anatomía & histología , Tálamo/citología , Tráquea/inervaciónRESUMEN
Retro-nociceptin methylester (retro-Noc-ME), which has an oppositely directed structure to that of nociceptin, showed weak affinity for nociceptin receptor and antagonized nociceptin-induced inhibition of contraction in a guinea pig ileum (GPI) assay. The peptide induced analgesia after intracerebroventricular (i.c.v.) administration at a dose of 100 nmol per mouse. Analgesia was not blocked by the opioid antagonist naloxone, which suggests that the analgesia is not mediated by opioid receptor. Furthermore, analgesia caused by retro-Noc-ME was not attenuated after repeated administration, that is, there was an absence of tolerance. The peptide improved learning ability after i.c.v. administration in a step-through experiment in mice.
Asunto(s)
Analgésicos/farmacología , Memoria/efectos de los fármacos , Péptidos/farmacología , Secuencia de Aminoácidos , Analgésicos/administración & dosificación , Animales , Cobayas , Inyecciones Intraventriculares , Aprendizaje/efectos de los fármacos , Ratones , Datos de Secuencia Molecular , Péptidos/administración & dosificación , Péptidos/química , Receptores Opioides/efectos de los fármacos , Receptor de NociceptinaRESUMEN
We report a patient with X-linked muscular dystrophy who had rapidly progressive muscle weakness and became wheelchair-bound at age 10 years. Clinically, he was diagnosed as having Duchenne muscular dystrophy; however, he was diagnosed as having Becker muscular dystrophy by dystrophin tests using a C-terminal monoclonal antibody. No immunolabelling was observed with a monoclonal antibody against the N-terminal domain. Multiplex polymerase chain reaction analysis revealed the deletion of exons 3-19. The data suggest that the deletion of the N-terminal domain of dystrophin can cause a severe phenotype even when the C-terminus of the protein is well preserved.
Asunto(s)
Deleción Cromosómica , Distrofina/genética , Ligamiento Genético/genética , Distrofias Musculares/genética , Fenotipo , Aberraciones Cromosómicas Sexuales/genética , Regiones Terminadoras Genéticas/genética , Cromosoma X , Adolescente , Anticuerpos Monoclonales , Biopsia , Exones/genética , Humanos , Immunoblotting , Masculino , Músculos/patología , Distrofias Musculares/patología , Reacción en Cadena de la Polimerasa , Aberraciones Cromosómicas Sexuales/patologíaRESUMEN
This study investigated the role of [18F]fluorodeoxyglucose (FDG) dual-head gamma camera coincidence imaging (GCI) in the pretreatment evaluation of patients with oesophageal cancer. Twenty-two patients (20 men; mean age, 64 years) with untreated, biopsy proven squamous cell carcinoma of the oesophagus underwent positron emission tomography (PET) and GCI 1 and 3 h after a single injection of FDG, respectively. Computed tomography (CT) was performed within 2 weeks of the FDG imaging. The sensitivity of lesion detection was compared between GCI and PET. Regional (N) and distant (M) metastases detected by GCI were evaluated with reference to PET and CT. The staging obtained by each modality was also compared with pathological staging in nine patients who underwent surgery. FDG PET detected 22 primary tumours, 34 metastatic lymph nodes and four organ metastases. Of them, GCI detected all primary tumours, 24 (71%) metastatic lymph nodes, and none of the organ metastases. Lymph nodes missed by GCI were smaller in size and the majority of them were located in the thoracic region. GCI provided N and M staging identical to CT and PET in eight patients and improved staging over CT in four patients. On the other hand, GCI missed metastases detected by both PET and CT in five patients. The addition of GCI to CT could improve detection of patients with metastasis to 82% (18/22) compared with 64% (14/22) detected by CT alone. In patients with pathological staging (n = 9), GCI could influence management changes in two patients (22%). In conclusion, FDG GCI has a role that is complementary to CT in the initial staging of patients with oesophageal cancer, and due to the additional detection of nodal metastasis, GCI can provide staging information, which may influence changes in management.
Asunto(s)
Neoplasias Esofágicas/diagnóstico por imagen , Fluorodesoxiglucosa F18 , Neoplasias de Células Escamosas/diagnóstico por imagen , Neoplasias de Células Escamosas/secundario , Cintigrafía/métodos , Anciano , Anciano de 80 o más Años , Neoplasias Esofágicas/diagnóstico , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neoplasias de Células Escamosas/diagnóstico , Radiografía , Radiofármacos , Reproducibilidad de los Resultados , Sensibilidad y Especificidad , Tomografía Computarizada de Emisión/métodosRESUMEN
OBJECTIVE: The objective of this study was to examine the attractive force of cast Fe-Pt alloys of varying compositions to dental iron-neodymium-boron magnets. METHODS: Ingots of Fe-40 at%Pt, Fe-38 at%Pt, Fe-37 at%Pt and Fe-36 at%Pt alloys were cast in pattern molds for dental magnetic attachment keepers. The attractive forces of the cast Fe-Pt alloy keepers and magnetic stainless steel keepers to dental Fe(14)Nd(2)B magnets (MAGFIT and HICOREX) were measured and statistically evaluated. The saturation magnetization of each Fe-Pt alloy was determined by recording the hysteresis loop using a vibrating sample magnetometer under a magnetic field of 1.6 MA/m. RESULTS: Decreasing the Pt percentage increased the saturation magnetization value and resulted in an increase of the attractive force to each magnet. There was no statistical difference (p>0.05) in attractive force between the Fe-36 at%Pt alloy specimens and the stainless steel keepers for both magnets. A definite correlation between Pt percentage and the value of saturation magnetization was also found (r(2)=-1.000). SIGNIFICANCE: The Fe-Pt alloys with less than Fe-39.5 at%Pt produced high saturation magnetization values and great attractive force to the magnet, and thus, they have the potential to serve as magnetic attachment keepers. Of the Fe-Pt alloys tested, Fe-36 at%Pt seemed to be the best composition for making magnetic attachment keepers.
Asunto(s)
Aleaciones Dentales/química , Hierro/química , Magnetismo/instrumentación , Platino (Metal)/química , Análisis de Varianza , Boro/química , Revestimiento para Colado Dental/química , Humanos , Ensayo de Materiales , Metalurgia , Neodimio/química , Acero Inoxidable/química , Estadística como Asunto , Propiedades de Superficie , VibraciónRESUMEN
Using parvalbumin immunohistochemistry to determine the distribution of muscle fiber types in the feline thyroarytenoid muscle (TA), we clearly distinguished the vocalis (with predominance of "slow" type 1 fibers) from the external TA (in which "fast" type 2 fibers predominated, especially in its rostral part). Reconstruction of serial frontal sections of the TA allowed the stereoscopic study of each division. The existence of a rudimentary laryngeal ventricle separating the true and false vocal folds in cats was demonstrated anatomically and histologically, and its relationships to each division of the TA were established. Our results suggest that the vocalis, fitted for enduring activities, is suited for voice control. The fast, rostral part of the external TA seems suited to laryngeal sphincteric demands, while its caudal counterpart may act in both functions. The anatomic individualization of the divisions of the TA may suggest that they play distinct physiological roles and may imply that they should not be considered a single functional unit.
Asunto(s)
Músculos Laríngeos/fisiología , Músculos Laríngeos/ultraestructura , Fibras Musculares de Contracción Rápida/clasificación , Fibras Musculares de Contracción Rápida/fisiología , Fibras Musculares de Contracción Lenta/fisiología , Animales , Gatos , Técnicas Histológicas , Inmunohistoquímica/métodos , Fibras Musculares de Contracción Lenta/clasificación , ParvalbúminasRESUMEN
Recent epidemiologic studies have shown that human T-lymphotropic virus type I (HTLV-I) is associated with polymyositis. To clarify the histological features of HTLV-I positive polymyositis, we studied muscle tissue from two groups of patients with polymyositis, those seropositive for HTLV-I (5 patients) and those seronegative (5 patients). We examined the lymphocyte subsets in muscle biopsies using monoclonal antibodies. In the endomysium, a variable number of T-lymphocytes and macrophages and a smaller proportion of B-lymphocytes were found in both groups. In both HTLV-I positive and negative patients a variable number of T-lymphocytes and macrophages, with a smaller number of B-lymphocytes, were found in the perimysium. As a whole, the T-lymphocytes were predominantly of the helper/inducer phenotype in both groups. We failed to find any specific phenotype distribution among T-cells infiltrating the muscle of HTLV-I carriers with polymyositis.
Asunto(s)
Antígenos Virales/análisis , Virus Linfotrópico T Tipo 1 Humano/inmunología , Subgrupos Linfocitarios/inmunología , Músculos/inmunología , Polimiositis/microbiología , Polimiositis/patología , Adulto , Anciano , Antígenos CD/análisis , Femenino , Humanos , Inmunidad Celular , Inmunofenotipificación , Macrófagos , Masculino , Persona de Mediana Edad , Polimiositis/inmunologíaRESUMEN
A benign Becker muscular dystrophy (BMD) patient with a marked decrease in dystrophin exhibited remarkable expression of dystrophin-related protein (DRP) on most of the muscle cell membrane. A phenotypic Duchenne muscular dystrophy patient with a truncated form of dystrophin exhibited no DRP expression on the muscle cell membrane except for the neuromuscular junction. Increased DRP expression might compensate for a lack of dystrophin in some BMD patients.
Asunto(s)
Proteínas del Citoesqueleto/análisis , Proteínas de la Membrana , Fibras Musculares Esqueléticas/química , Distrofias Musculares/metabolismo , Adolescente , Adulto , Anticuerpos Monoclonales/inmunología , Membrana Celular/química , Cromosomas Humanos Par 6/genética , Proteínas del Citoesqueleto/genética , Proteínas del Citoesqueleto/inmunología , Distrofina/análisis , Exones/genética , Humanos , Inmunohistoquímica , Masculino , Fibras Musculares Esqueléticas/patología , Distrofias Musculares/genética , Distrofias Musculares/patología , Fenotipo , Reacción en Cadena de la Polimerasa , Regulación hacia Arriba , UtrofinaRESUMEN
OBJECT: The aim of this study was to reveal variations in the patterns of expression of the cell surface proteins in regenerating fibers and those in the number of satellite cells to gain an understanding of the pathological processes involved in sarcoglycanopathy. METHODS: We have reported that there is a reduction of the beta-1 subunit of laminin, heparan sulfate proteoglycan (HSPG), and HCAM (CD44) in Japanese patients with sarcoglycanopathy. Here, we investigated immunohistochemically the expression of the neural cell adhesion molecule (NCAM), which is a marker for human regenerating muscle and satellite cell, and CD24, which appears to be expressed in the early stages of the regeneration process. PATIENTS: We investigated six Japanese patients with sarcoglycanopathy, and compared to age-matched Becker muscular dystrophy. RESULTS: We found that the incidences of muscle fibers with increased NCAM were not statistically different between the two groups. However, the incidences of muscle fibers with increased CD24 and those of NCAM positive satellite cells were very low in sarcoglycanopathy and were statistically different between sarcoglycanopathy and age-matched Becker muscular dystrophies. CONCLUSION: The poor expression of CD24 and the fewer satellite cells in sarcoglycanopathy without significant difference in the number of total regenerating fibers suggest that a different regeneration process is involved in sarcoglycanopathy compared to that in other types of muscular dystrophy.
Asunto(s)
Antígenos CD/metabolismo , Proteínas del Citoesqueleto/deficiencia , Glicoproteínas de Membrana/deficiencia , Fibras Musculares Esqueléticas/metabolismo , Músculo Liso/metabolismo , Distrofias Musculares/metabolismo , Sarcolema/metabolismo , Adolescente , Adulto , Edad de Inicio , Antígeno CD24 , Femenino , Humanos , Técnicas para Inmunoenzimas , Masculino , Persona de Mediana Edad , Fibras Musculares Esqueléticas/patología , Distrofias Musculares/patología , Moléculas de Adhesión de Célula Nerviosa/metabolismo , Sarcoglicanos , Sarcolema/patologíaRESUMEN
Ascorbic acid, reported in 1988 to be effective for idiopathic thrombocytopenic purpura (ITP), is an attractive drug because of its lack of toxicity. Further studies are necessary in order to improve its effectiveness without increasing secondary effects. We present a chronic ITP patient treated with a combination of ascorbic acid and methylprednisolone pulse (MP) therapy who was previously treated with MP therapy alone. The effect of this combination therapy seems to be better than MP therapy alone. This therapy is worth further examination as another therapeutic choice due to its fewer secondary effects than the usual regimen of corticosteroids, splenectomy, and other immunosuppressive drugs.