Twenty-one-year follow-up of variable onset MELAS syndrome with heteroplasmic nt3243A>G mtDNA mutation: A case report

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a maternally inherited mitochondrial disorder of which m.3243A>G is the most commonly associated mutation, resulting in an inability to meet the energy requirements of various organs. MELAS poses a diagnostic challenge owing to its multiple organ involvement and great clinical variability due to its heteroplasmic nature. We report three cases from a family who were initially misdiagnosed with myasthenia gravis or undiagnosed. Although there is no optimal consensus treatment approach for patients with MELAS because of the disease's heterogeneity, our 21-year-long therapy regimen of l-arginine, l-carnitine, and coenzyme Q10 supplementation combined with dietary management appeared to provide noticeable protection from the symptoms and complications. Prompt early diagnosis is important, as optimal multidisciplinary management and early intervention may improve outcomes.

A Case of Liver Fibrosis with Splenomegaly after Oxaliplatin-Based Adjuvant Chemotherapy for Colon Cancer

Previous studies reported that oxaliplatin is associated with sinusoidal obstruction syndrome. However few reports on oxaliplatin induced liver fibrosis are found in the literature. Furthermore pathogenesis of liver fibrosis is not well known. We report a case of 45-yr-old Korean man in whom liver fibrosis with splenomegaly developed after 12 cycles of oxaliplatin based adjuvant chemotherapy for colon cancer (T4N2M0). Thorough history taking and serological examination revealed no evidence of chronic liver disease. Restaging CT scans demonstrated a good response to chemotherapy. Five month after chemotherapy, he underwent right hepatectomy due to isolated metastatic lesion. The liver parenchyma showed diffuse sinusoidal dilatation and centrilobular vein fibrosis with necrosis without steatosis. We could conclude that splenomegaly was due to perisinusoidal liver fibrosis and liver cell necrosis induced portal hypertension by oxaliplatin. In addition, to investigate the pathogenesis of liver fibrosis, immunohistochemical stains such as CD31 and alpha-smooth muscle actin (alpha-SMA) were conducted with control group. The immunohistochemical stains for CD31 and alpha-SMA were positive along the sinusoidal space in the patient, while negative in the control group. Chemotherapy with oxaliplatin induces liver fibrosis which should be kept in mind as a serious complication.

The effects of bee venom pretreatment of an acupoint on inflammation and hyperalgesia induced by peptidoglycan / 대한마취과학회지

BACKGROUND: Systemic injection of peptidoglycan (PGN) special polymers, which are the primary structural components of most bacterial cell walls, leads to acute inflammation and pain behavior. This study was conducted to confirm that an intraplantar injection of PGN evoked hindpaw inflammation and hyperalgesia, and to evaluate the effects of bee venom (BV) pretreatment of an acupoint on PGN induced inflammation and hyperalgesia. METHODS: Inflammation and hyperalgesia were induced by injecting PGN into the plantar surface of one hindpaw of the rats. Inflammation and hyperalgesia were then evaluated by measuring the thickness of the hindpaw using a caliper and the paw withdrawal time (PWT) in response to noxious thermal stimulus (48degrees C hot water). In addition, spinal cord c-fos expression was quantitatively analyzed. The BV pretreatment was injected at the acupoint located 5 mm lower and 5 mm lateral to the anterior tubercle of the tibia in the hind limb. RESULTS: The PGN groups showed increased in paw thickness and spinal c-fos expression two hours after PGN injection, as well as decreased PWT in response to noxious thermal stimulus for each tested time. BV pretreatment of the acupoint was found to inhibit hindpaw thickness and led to a significant increase in PWT, but did not significantly inhibit spinal cord c-fos expression induced by PGN injection. CONCLUSIONS: These results indicated that BV pretreatment has both an anti-inflammatory and antinociceptive effect in PGN induced inflammatory pain, which suggests that peptidoglycan may be useful as an inflammatory agent for inflammatory pain models.

Pathologic Comparative Studies on the Protective Effects by Panax Ginseng and Panax Quinquefolium for Treating 2,3,7,8-tetrachlorodibenzo-p-dioxin-induced Toxicity in Male Rats

BACKGROUND: Panax ginseng is known to decrease the 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD)-induced testicular toxicity. Thus, we aimed to reveal the differences between Panax ginseng and Panax quinquefolium extract for their effects on TCDD-induced toxicity. METHODS: Forty rats were divided into four groups; the control group, the TCDD only group, the TCDD plus Panax ginseng group, and the TCDD plus Panax quinquefolium-treated groups. Ginseng extract was given orally to rats from day one to twenty-one. TCDD was intraperitoneally administered to rats at a single dose of 50 microgram/kg on the seventh day. The pathologic changes were then examined. The changes of body weight, cholesterol and GOT in the serum were also examined. RESULTS: The TCDD toxicity was prominent in the thymus, liver and testis. The thymus showed atrophy and an inverse pattern of lymphocyte density in the cortex and medulla. The liver revealed central necrosis with fatty changes. On electron microscopy, the seminiferous tubules showed destruction of the spermatogonia, clear spaces or vacuolar changes and degeneration in the Sertoli cells or germ cells. The above mentioned TCDD-induced changes were reduced in the rats that were administered with Panax ginseng, whereas Panax quinquefolium did not reduce these changes. CONCLUSION: The protective effects of Panax ginseng on the TCDD-induced toxicity were more effective than those of Panax quinquefolium.