Some cases of renal malignancy associated with adult (autosomal dominant) polycystic kidney disease have been reported. Most of these malignancies were diagnosed as renal cell carcinoma. But the case of transitional cell carcinoma has not been reported. We report a case of renal pelvic and urethral transitional cell carcinoma associated with adult polycystic kidney.
Adult* ; Carcinoma, Renal Cell ; Carcinoma, Transitional Cell* ; Humans ; Kidney ; Polycystic Kidney Diseases ; Polycystic Kidney, Autosomal Dominant*

OBJECTIVE: The aim of the study was to evaluate the effectiveness of ultrasouond-guided salivary gland injection of botulinum toxin A (BTX-A) for posterior drooling. METHOD: 11 patients with brain lesion (9 cerebral palsy, 1 hypoxic ischemic encephalopathy and 1 mental retardation) with posterior drooling (an initial PDAS score greater than 2) and related pulmonary problems were recruited. Drooling severity was measured at baseline, 4 weeks, 3 months and 6 months after botulinum toxin A injection, by using Teacher Drooling Scale (TDS), Visual Analogue Scales (VAS), Drooling Score System (DSS)-severity, frequency and Posterior Drooling/Aspiration System (PDAS). RESULTS: The TDS, DSS-severity, DSS-frequency, VAS, PDAS were significantly reduced at 4 weeks and 3 months after BTX-A injection into salivary glands compared to pre-injection (p<0.05). However, there were no significant changes at 6 months compared to pre-injection level. CONCLUSION: BTX-A injection into salivary glands may improve anterior drooling in patients with brain lesions. Furthermore BTX-A injection into salivary glands may also decrease the posterior drooling which might related to respiratory symptoms in aspiration pneumonia.
Botulinum Toxins ; Botulinum Toxins, Type A ; Brain ; Cerebral Palsy ; Humans ; Hypoxia-Ischemia, Brain ; Pneumonia, Aspiration ; Salivary Glands ; Sialorrhea ; Sorbitol ; Stress, Psychological ; Tyramine ; Weights and Measures

Stevens-Johnson syndrome (SJS) is a rare disease in which extensive toxic epidermolysis occursafter medication. Skin and mucous membranes are involved in about 90% of SJS cases, and webbingof mouth corners (microstomia) may occur when they are affected. Few reports have been issuedon microstomia in SJS, and no consensus has been reached regarding treatment methods,timings, or results. We encountered a case of microstomia following SJS after ofloxacin medicationin a 22-year-old woman treated by commissuroplasty using a lozenge-shaped excision. Wepresent an appropriate correction method and surgical timing for microstomia following SJS.

Background: Microstomia is defined as a condition with a small sized-mouth that results in functional impairment such as difficulty with food intake, pronunciation, and poor oral hygiene and cosmetic problems. Several treatment methods for microstomia have been proposed. None of them are universally applicable. This study aims at analyzing the cases treated at our institution critically reviewing the pertinent literature. Methods: The medical records of all microstomia patients treated in our hospital from November 2015 to April 2018 were reviewed retrospectively. Of these, all patients who received surgical treatment for microstomia were included in the study and analyzed for etiology, chief complaint, surgical method, and outcomes. The functional outcomes of mouth opening and intercommissure distance before and after the surgery were evaluated. The cosmetic results were assessed according to the patients’ satisfaction. Results: Five patients with microstomia were corrected. Two cases were due to scar contracture after chemical burn, two cases derived from repeated excision of skin cancer, and one patient suffered sequela of Stevens-Johnson syndrome. The following surgical methods were applied: one full-thickness skin graft on the buccal mucosa, three buccal mucosal advancement flaps after triangular excision of the mouth corner, and one local buccal mucosal flap. Mouth opening was increased by 6.0 mm, and the intercommissure distance improved by 7.2 mm on average. Follow-up was 9.6 months (range, 5–14 months). Cosmetic assessment was as follows: two patients found the results excellent, three judged it as good. Conclusion Microstomia has several causes. In order to achieve optimal functional recovery and aesthetic improvement it is important to precisely evaluate the etiologic factors and the severity of the impairment and to carefully choose the appropriate surgical method.

OBJECTIVE: To investigate the effect of intravenous infusion of peripheral blood mononuclear cells (mPBMC) mobilized by granulocyte-colony stimulating factor (G-CSF) on upper extremity function in children with cerebral palsy (CP). METHODS: Fifty-seven children with CP were enrolled. Ten patients were excluded due to follow-up loss. In total, 47 patients (30 males and 17 females) were analyzed. All patients' parents provided signed consent before the start of the study. After administration of G-CSF for 5 days, mPBMC was collected and cryopreserved. Patients were randomized into two groups 1 month later. Twenty-two patients were administered mPBMC and 25 patients received normal saline as placebo. Six months later, the two groups were switched, and administered mPBMC and placebo, respectively. Quality of Upper Extremity Skills Test (QUEST) and the Manual Ability Classification System (MACS) were used to evaluate upper motor function. RESULTS: All subdomain and total scores of QUEST were significantly improved after mPBMC and placebo infusion, without significant differences between mPBMC and placebo groups. A month after G-CSF, all subdomain and total scores of QUEST were improved. The level of MACS remained unchanged in both mPBMC and placebo groups. CONCLUSION: In this study, intravenously infused mPBMC showed no significant effect on upper extremity function in children with CP, as compared to placebo. The effect of mPBMC was likely masked by the effect of G-CSF, which was used in both groups and/or G-CSF itself might have other neurotrophic potentials in children with CP.
Cerebral Palsy* ; Child* ; Classification ; Follow-Up Studies ; Granulocyte Colony-Stimulating Factor ; Humans ; Infusions, Intravenous* ; Male ; Masks ; Parents ; Peripheral Blood Stem Cell Transplantation ; Upper Extremity*

PURPOSE: This study aims to investigate the actual compliance with chemotherapy and analyze several factors affecting the compliance in patients with gastric cancer. METHODS: From February 2012 to December 2014, we collected data of patients with gastric cancer who received adjuvant chemotherapy (TS-1 monotherapy or XELOX: capecitabine/oxaliplatin) in Korea. RESULTS: We collected data of 1,089 patients from 31 institutions. The completion rate and dose reduction rate by age (≥60 years vs. <60 years) were 57.5% vs. 76.8% (P < 0.001) and 17.9% vs. 21.3% (P = 0.354); by body mass index (BMI) (≥23 kg/m2 vs. <23 kg/m2) were 70.2% vs. 63.2% (P = 0.019) and 19.2% vs. 19.9% (P = 0.987), respectively. The compliance by American Society of Anesthesiologists physical status (ASA PS) classification was as follows: completion rate was 74.4%, 62.8%, and 60% (P = 0.001) and the dose reduction rate was 18.4%, 20.7%, and 17.8% (P = 0.946) in ASA PS classification I, II, and III, respectively. The completion rate of TS-1 and XELOX was 65.9% vs. 70.3% (P = 0.206) and the dose reduction rate was 15.7% vs. 33.6% (P < 0.001). Furthermore, the completion rate of chemotherapy by surgical oncologists and medical oncologists was 69.5% vs. 63.2% (P = 0.028) and the dose reduction rate was 17.4% vs. 22.3% (P = 0.035), respectively. CONCLUSION: The compliance was lower in patients who were older than 60 years, had BMI <23 kg/m2, and had higher ASA PS classification. Furthermore, the patients showed higher compliance when they received chemotherapy from surgical oncologists rather than from medical oncologists.
Body Mass Index ; Chemotherapy, Adjuvant ; Classification ; Compliance ; Drug Therapy ; Humans ; Korea ; Stomach Neoplasms