• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.16 no.2
• /
• pp.57-61
• /
• 2016

Isovaleric acidemia (IVA) is an autosomal recessively inherited organic acid disorder due to a defect of the enzyme isovaleryl-CoA dehydrogenase in the leucine metabolic pathway. Deficiency of this enzyme results in the accumulation of derivatives of isovaleryl-CoA. In acute illness in IVA, isovaleric acid and its derivatives accumulate and profound metabolic acidosis with ketosis, characteristic pungent body odor, hypoglycemia, and hyperammonemia can be developed. Additionally, recurrent vomiting, failure to thrive, developmental delay, epilepsy and mental retardation are chronic presenting symptoms and signs for IVA. On the result of newborn screening for inherited metabolic disorders, increased levels of isovalerylcarnitine (C5) are shown. However, C5 elevation can be accompanied with short/branched-chain acyl-CoA dehydrogenase (SBCAD) and therapy with certain antibiotics containing pivalic acid. Quantitative measurement of organic acids in urine and acylcarnitine profiles in plasma are necessary to differential diagnosis. Molecular genetic analysis of the IVD gene for IVA and ACADSB is also helpful to confirm IVA and SBCAD deficiency, respectively. Considering that IVA can be associated with significant morbidity and mortality at acute presentation of metabolic crisis, early diagnosis prior to the onset of symptoms by newborn screening enable to introduction of early treatment and prevention of acute and chronic complications.

• Clinical and Experimental Pediatrics
• /
• v.51 no.6
• /
• pp.655-659
• /
• 2008

Intestinal pseudo-obstruction (IPO) is a rare and poorly understood manifestation of systemic lupus erythematosus (SLE), especially in children. The characteristic clinical feature of IPO is obstruction without an identifiable obstructive lesion. The authors a 13-year-old girl whose first symptom of SLE was IPO. The patient presented with a 3-day history of nausea, bilious vomiting, abdominal distention, and no bowel movement. Simple abdominal radiographs revealed mild dilatation with partial air-fluid levels in the small intestine. Abdominal CT and methylcellulose small bowel studies showed massive ascites, engorgement of the small mesenteric vessels, pleural effusion, and diffuse bowel wall thickening of the gastric antrum, duodenum. and jejunum. The delayed passage of contrast for 15 days after the methylcellulose small bowel studies was suggestive of decreased bowel motility. Laboratory findings were positive for ANA, anti-double-stranded DNA, anti-Smith and lymphopenia. After 10-day treatment with high-dose corticosteroids, the symptoms improved. IPO associated with SLE should be considered in the differential diagnosis for patients presenting with symptoms of intestinal obstruction. Early recognition of IPO in SLE and appropriate therapy are important for prevention of complications and unnecessary surgery. This case raises awareness among pediatricians that although rare, IPO can be the presenting symptom of SLE in children.

• Radiation Oncology Journal
• /
• v.12 no.2
• /
• pp.191-199
• /
• 1994

Purpose : This study was undertaken to show the clinical results of combined radiotherapy and hyperthermia in primary hepatoma Materials and Methods : Between December 1989 and March 1993, 50 patients with hepatomas were treated by combined radiotherapy and hyperthermia. Among them, we analyzed retrospectively 33 patients who received the complete course of treatment. The ages of the patients ranged from 36 to 75(mean age: 55.5 years). Twenty-six patients ($78.8\%$) were men, and 7 ($21.2\%$ were women. According to Child's classification, nine patients ($27.3{\%}$) were A group, 9 ($27.3\%$) were B group, 15 ($45.4\%$) were C group. Radiation therapy was done by a 6 MV and 15 MV linear accelerator. Patients were treated with daily fractions of 150-180 cCy to doses of 2550 cGy -4950 cGy (median : 3000 cGy). Local hyperthermia was done by 8 MHZ RF capacitive heating device (Cancermia. Green Cross Co., Korea), 50-60 min/session, 1-2 sessions/wk, and 8.5 sessions (median number)/patient. We analyzed the prognostic factors including age, sex, tumor type, Child's classification, $\alpha$-fetoprotein, liver cirrhosis, ascites, portal vein invasion, esophageal varix, number of hyperthermia, chemotherapy, total bilirubin level, Karnofsky perfomance status. Results : The overall 1-year survival was $24.2\%$, with a mean survival of 10months. Of 33 patients, tumor regression (PR+MR) was seen in $30.4\%$, no response was seen in $52.2\%,\;17.4\%$ patient was progressed. In patients who had tumor regression, the overall 1-year survival was $42.1\%$ with a mean survival of 14 months. Factors influencing the survival were sex (p=0.05), tumor type (p=0.0248), Child's classification (p=0.0001), liver cirrhosis (p=0.0108), ascites (p=0.0009), and Karnofsky perfomance status (p=0.0028). Complications developed in 28 patients, including 18 hot pain,5 fat necrosis, 3 transient fever, 2 nausea and vomiting. Conclusion : In this study, the results suggests that combined radiotherauy and hyperthermia may improve the survival rate of hepatoma.

• Journal of Veterinary Clinics
• /
• v.25 no.4
• /
• pp.295-302
• /
• 2008

Two dogs were referred to Veterinary Medical Center, Chungbuk National University with the clinical signs of vomiting, diarrhea, anorexia, and jaundice. Both dogs were diagnosed as extrahepatic biliary tract obstruction(EHBO) with concurrent pancreatitis based on dilated common bile duct and hyperechoic or mixed-echoic pancreas in abdominal ultrasonographic examination and serum biochemical abnormalities, such as high serum bilirubin, cholesterol, and increased cholestatic enzyme activity. Percutaneous ultrasound-guided cholecytocentesis(PUCC) was performed to examine bile and decompress the gall bladder. After PUCC with medical therapy, both dogs were steadily improved clinical signs of jaundice and anorexia. Also, cholestatic enzyme activity and serum bilirubin concentration decreased. Any complications that have been described in previous studies, such as peritonitis resulting from bile leakage and hemorrhage, were not identified. It is assumed that percutaneous ultrasound guided cholecystocentesis may be an useful diagnostic and therapeutic tool in canine gallbladder disease and can be used easily and safely to gain bile for diagnosis of bacterial cholecytitis.

• Asian Pacific Journal of Cancer Prevention
• /
• v.16 no.11
• /
• pp.4791-4795
• /
• 2015

Purpose: This study was conducted to observe the efficacy and safety of pemetrexed based chemotherapy in treating patients with locally advanced or metastatic cancers as first-line, second-line or third-line therapy. Materials and Methods: From May 2011 to January 2015, we recruited 29 patients with advanced breast cancer, 19 patients with advanced ovary cancer, 17 patients with advanced esophageal cancer,5 patients with advanced gallbladder cancer,5 patients with advanced cervical cancer and 1 patient with advanced tongue cancer in Jiangsu Cancer Hospital and Research Institute.All of them were pathologically confirmed and treated with pemetrexed based chemotherapy. After two cycles of treatment,efficacy and safety can be evaluated. Results: For pemetrexed based regimens,including 76 patients with 6 kinds of advanced cancer were considered eligible for inclusion. Complete remission represents CR, partial remission represents PR, stable disease represents SD, progressive disease represents PD. Among 29 patients with advanced breast cancer, 4 patients chose pemetrexed based regimens as second-line treatment,1 of them was PR,the other 3 got SD. The last 25 patients made use of this chemotherapy as third-line treatment, except one patient could not be assessed, 2 of them got PR,6 of them got SD,the remaining 16 of them finally were PD.19 patients with advanced ovary cancer,5 patients used this regimens as second-line treatment, 3 of them got PD,the remaining patients got SD, respectively. The last 14 patients made use of pemetrexed based regimens as third-line treatment,. RR (CR+PR) was 28.5%. Among 17 patients with advanced esophageal cancer, 2 patients made use of pemetrexed based regimens as first-line treatment,both of them got PR.4 of them used this chemotherapy as second-line regimen, except 2 patients could not be assessed,the remaining 2 was PD at last. The last 11 patients was third-line users, RR (CR+PR) was 18.2%. Among 5 patients with advanced gallbladder cancer, pemetrexed based regimens was used in 1 patient as first-line treatment and 1 patient as second-line treatment. The curative effect was SD and PD, respectively. 3 patients accepted pemetrexed based regimens as third-line treatment, 2 of them got PD as results and another was SD. Among 5 patients with advanced cervical cancer, just 1 patient adopted pemetrexed based regimens as first-line treatment, whose curative effect was PR.2 patients chose this chemotherapy regimens as second-line treatment. Both of them got PD as their consequence. The last 2 patients made use of the regimens as third-line treatment, the effect of them was PD and SD, respectively. The one who with advanced tongue cancer, pemetrexed based regimens was used as second-line treatment, and the consequence was PD. About 71.1% patients experienced bone marrow suppression. Among them, 5 patients reached 4 grade. Other toxicity of pemetrexed were neurotoxicity, fatigue, diarrhea, dysphagia and vomiting. No treatment related death occurred with pemetrexed-based treatment. Conclusions: Pemetrexed based chemotherapy has considerable effect in patients with advanced cancers such as breast cancer,esophageal cancer and ovary cancer. More randomly clinical trials are needed to verify the results.

• Asian Pacific Journal of Cancer Prevention
• /
• v.16 no.9
• /
• pp.4037-4040
• /
• 2015

Objective: To explore the clinical efficacy and toxic and side effects of recombinant human endostatin (rhendostatin/endostar) combined with chemotherapy in the treatment of advanced gastric cancer. Materials and Methods: A total of 70 patients with advanced gastrointestinal adenocarcioma confirmed by histopathology and/or cytological examination were divided into group A (37 patients) and group B (33 patients). Patients in group A were given intravenous drip of 15 mg endostar added into 500 mL normal saline, once every other day until the cessation of chemotherapy or patients' maximal tolerance to chemotherapy. Patients in group B received chemotherapy alone. Two groups selected the same chemotherapy regimens. FOLFIRI scheme: 90-min intravenous drip of $180mg/m^2$ irinotecan, intravenous drip of $200mg/m^2$ calcium folinate (CF) and $400mg/m^2$ 5-fluorouracil (5-Fu) on d1, and continuous intravenous pumping of 2 $400mg/m^2$ 5-Fu for 46 h. FOLFOX4 scheme: intravenous injection of $85mg/m^2$ oxaliplatin (L-OHP), $200mg/m^2$ calcium folinate (CF) and $400mg/m^2$ 5-FU on d1 for 2 h, and then continuous intravenous pumping of 2 $400mg/m^2$ 5-Fu for 46 h. XELOX scheme: oral administration of 1 $500mg/m^2$ xeloda (or tegafur 50~60 mg) in twice during d1~14 and intravenous drip of $135mg/m^2$ L-OHP on d1 for 2 h. The modified FOLFOX scheme: intravenous injection of $135mg/m^2$ L-OHP on d1 for 2 h, $200mg/m^2$ CF and 1.0 g tegafur during d1~5. Whereas, control Group B received chemotherapy regimens which were same as Group A, but no addition of endostar. Before chemotherapy, patients were given intravenous injection of 8 mg ondansetron, intramuscular injection of 10 mg metoclopramide and 20 mg diphenhydramine for prevention of vomiting, protection of liver and stomach as well as symptomatic supportive treatment. One cycle was 21 d, 4~6 cycles in total. The efficacy was evaluated every 2 cycles. Results: 32 patients in Group A could be evaluated, and the response rate (RR) and disease control rate (DCR) were 59.38% and 78.13%, respectively. 31 patients in Groups could be evaluated, and the RR and DCR were 32.26% and 54.84%, respectively. The differences between 2 groups were significant. The toxic effects include myelosuppression, gastrointestinal reaction, fatigue, cardiotoxicity and peripheral neurotoxicity. Conclusions: Preliminary observations show that endostar (once every other day) combined with chemotherapy is effective in the treatment of advanced gastrointestinal cancer, with low toxic effects, good tolerance, deserving further study.

• Asian Pacific Journal of Cancer Prevention
• /
• v.13 no.8
• /
• pp.4187-4190
• /
• 2012

Aims and Background: To evaluate the efficacy of a combination of aprepitant and conventional antiemetic therapy in patients with advanced or recurrent lung cancer receiving moderately emetogenic chemotherapy (MEC). Methods: Patients with advanced or recurrent lung cancer who were treated with MEC regimens at the Department of Respiratory Medicine, Fukuoka University Hospital, were included and classified into the following groups: control group (treatment: 5-HT3 receptor antagonists + dexamethasone) and aprepitant group (treatment: 5-HT3 receptor antagonists + dexamethasone + aprepitant). The presence or absence of chemotherapy-induced nausea and vomiting (CINV) was evaluated according to the Common Terminology Criteria for Adverse Events (CTCAE) v4.0; patients with grade 1 or above were considered positive for CINV. Food intake per day, completion of planned chemotherapy, and progression-free survival (PFS) achieved by chemotherapy were investigated. Results: The complete suppression rate of nausea in the aprepitant group was significantly higher than that in the control group (p = 0.0043). Throughout the study, the food intake in the aprepitant group was greater than that in the control group, with the rate being significantly higher, in particular, on day 5 (p = 0.003). The completion rate of planned chemotherapy was also higher in the aprepitant group (p = 0.042). PFS did not differ significantly, but tended to be improved in the aprepitant group. Conclusions: The aprepitant group showed significantly higher complete suppression of nausea, food intake on day 5, and completion of planned chemotherapy than the control group.

• Asian Pacific Journal of Cancer Prevention
• /
• v.15 no.17
• /
• pp.7303-7307
• /
• 2014

Objective: To explore the clinical efficacy of gemcitabine concomitant with nedaplatin and drug resistance in the treatment of non-small cell lung cancer (NSCLC) and associated molecular predicators. Materials and Methods: A total of 68 patients diagnosed with NSCLC by histology served as the study objects and were randomly divided into an observation group treated with gemcitabine concomitant with nedaplatin and a control group with cisplatin concomitant with gemcitabine, 34 cases for each group. Short-term and long-term efficacies, adverse responses as well as the expression of nucleotide excision repair cross complementing 1 (ERCC1), ribonucleotide reductase subunit M1 (RRM1) and lung resistance-related protein (LRP) in NSCLC tissues in both groups were assessed. Results: The short-term objective response rate (ORR) and disease control rate (DCR) were 35.3% (12/34) and 76.5% (26/34) in the observation group and 38.2% (13/34) and 85.3% (29/34) in the control group, respectively, the differences not being statistically significant. The time to progression (TTP) in both groups were 1~12 months, while the median TTP was 135 d and 144 d, respectively. Though the survival was slightly higher in the control group, there were no significant differences in TTP and survival time. The rates of decreased hemoglobin, vomiting and nausea as well as renal toxicity were evidently lower in the observation group, while other adverse responses demonstrated no significant difference. The positive expression rates of ERCC1, RRM1 and LRP were 47.1% (16/34), 61.8% (21/34) and 64.7% (22/34) in the observation group, respectively. Compared with negative ERCC1 expression, ORR had decreasing trend and the overall survival time (OS) decreased significantly in patients with positive ERCC1 expression, which were markedly decreased by the positive expressions of RRM1 and LRP. Conclusions: Gemcitabine concomitant with nedaplatin has significant effects in the treatment of NSCLC, with an adverse response rate obviously lower than for cisplatin concomitant with gemcitabine, suggesting that wider use in the clinic is warranted. Additionally, the positive expressions of ERCC1, RRM1 and LRP may increase patient drug resistance, so they can be applied as the chemotherapeutic predicators to guide individualized therapy of NSCLC patients.

• Clinical and Experimental Pediatrics
• /
• v.50 no.3
• /
• pp.292-297
• /
• 2007

Purpose : Kawasaki disease (KD) rarely occurs in school-aged children. We clarified the characteristics of KD in this age group to provide tips for a high index of suspicion. Methods : Features of 38 patients with KD who were 7 years of age or older were retrospectively reviewed. Results : The incidence of the KD patients ${\geq}7years$ was 4.9 percent. The ratio of male to female was 2.5:1. Of the 38 patients, nine patients (24.0 percent) were diagnosed with typical KD and 29 patients (76.0 percent) with incomplete KD. In incomplete KD patients, cervical lymphadenopathy (69.0 percent) occurred most frequently, followed by conjunctival injection (62.0 percent) and polymorphous rash (45.0 percent). These patients occasionally presented with other additional symptoms including abdominal pain, headache, vomiting and arthralgia. Incomplete KD was initially diagnosed as cervical lymphadenitis (34.0 percent), viral infection (14.0 percent), scarlet fever (7.0 percent), meningitis (7.0 percent), and Kikuchi disease (7.0 percent). Coronary complications were noted in 15 patients (39.0 percent). Of the 37 patients treated with intravenous immunoglobulin, five (14.0 percent) were resistant to the therapy and all had coronary abnormalities. Conclusion : Most patients with KD ${\geq}7years$ of age have incomplete presentations. They tend to have a higher incidence of initial presentations of unilateral neck mass and coronary artery involvement. In school-aged children, fever and cervical lymphadenitis or suspected neck infection unresponsive to intravenous antibiotics should signal the possibility of KD. A high index of suspicion and prompt treatment is essential in this age group of patients.

• Journal of Veterinary Clinics
• /
• v.27 no.1
• /
• pp.79-82
• /
• 2010

A 1-year-old intact male Russian blue cat was admitted with a history of depression, partial anorexia, vomiting and dysuria. Through the blood analysis, ultrasonography and urinalysis, this cat was diagnosed as feline lower urinary tract disease with cystitis. Despite antibiotic therapy the cat showed stranguria and urinary catheterization were repeated several times. One week after, this patient appeared depression, hyperthermia and leukocytosis. Ultrasonography revealed a small amount of hypoechoic fluid around the right kidney and bacteria were found in the urine. The amount of the right subcapsular fluid was increased and bacteria were found in the fluid. On the excretory urography, leakage of contrast media were detected. The right kidney was surgically removed and suppurative fluid between the renal parenchyma and the thickened capsule was identified. After surgery, subcapsular abscess of the right kidney associated with pyelonephritis was confirmed histologically and this cat recovered without complications.