OBJECTIVEProgressive muscular dystrophy (PMD) is characterized by muscle fiber necrosis, regeneration, and endomysial fibrosis. Although absence of dystrophin and subsarcolemmic protein has been known as the cause of muscle fiber degeneration, pathogenesis of interstitial fibrosis is still unknown. The aim of this study was to investigate the role of connective tissue growth factor (CTGF) in PMD and its relationship with muscular fibrosis.

METHODSImmunological localization of CTGF was examined in frozen muscle specimens obtained via biopsy from 8 patients with Duchenne muscular dystrophy (DMD), 2 patients with Becker muscular dystrophy (BMD), 6 patients with congenital muscular dystrophy (CMD) and 6 cases with normal muscle by immunohistochemistry, double immunofluorescence and Western blot analysis.

RESULTSThe results of immunohistochemistry and double immunofluorescence showed that CTGF was positive only in vessels of normal muscle. Both immunohistochemistry and Western blot analysis showed that CTGF expression was distinctly increased in dystrophy muscles of PMD than that in normal muscles. In dystrophy muscle, marked immunostaining of CTGF was not only observed in vascular walls, but also strongly expressed in the cytoplasm and nuclei of regenerating muscle fibers, and also immunolocalized in the muscle fiber sarcolemma of non-regenerating fibers. Double labeling with antibodies against CTGF and CD68 demonstrated that CTGF was expressed in some macrophages and some macrophage infiltrated necrotic fibers. CTGF was strongly expressed in endomysial and perimysial connective tissues of dystrophy muscles of patients with DMD, CMD and FCMD. Double immunolabeling revealed that most activated fibroblasts in perimysium and endomysium were positive for CTGF, but not all of connective tissues were co-localized with CTGF. Older cases with FCMD showed poor or no expression of CTGF in advanced fibrosis.

CONCLUSIONCTGF may play a role in the pathogenetic process of muscular dystrophy, and CTGF may be important for muscle repair and fibrosis.

Adolescent ; Case-Control Studies ; Child ; Child, Preschool ; Connective Tissue Growth Factor ; metabolism ; Female ; Fibrosis ; Fluorescent Antibody Technique ; Humans ; Immunohistochemistry ; Infant ; Male ; Muscles ; metabolism ; pathology ; Muscular Dystrophies ; metabolism

During the 18-month period from January 1, 1981 to June 30, 1082, we encountered a total of 11 cases of child peptic ulcer-three gastric ulcer and eight duodenal ulcer casen.
There is every indication that the incidence of child peptic ulcer will increase in a rural area like Yuri, which is situated in Japan's northeastern prefecture of Akita.
To be noted is the fact that 10 cases out of 11 of peptic ulcer were found in three-year lower secondary schoolchildren and the remaining one was found in a sixth grader of six-year elementary school. The incidence as well as the number of visits to our clinics went up as the age advances and reached a peak in third graders of lower secondary school.
The reason why third graders of lower secondary school were attacked most by peptic ulcer is probably that they, at puberty, are under psychic or psychogenic stress with anxiety over high school entrance examinations, mental strain from forced attendance at a cramming school, trouble in getting along with friends, and dissatisfaction with parents.
Therefore, child peptic ulcer should not be treated as a disease of the digestive organ alone but as a disorder in the autonomic nervous system with an aid of psychosomatic medicine and psychiatry. For the prevention of the disease, it would also be necessary to take account of socio-cultural factors.