ANCA-Negative Wegener's Granulomatosis with Multiple Lower Cranial Nerve Palsies

Wegener''s granulomatosis (WG) is a systemic vasculitis affecting small and medium-sized vessels with granulomatous formation. Though it is known for respiratory tract and kidney involvement, neurologic manifestation has been also reported. Herein we report a patient who suffered pansinusitis with multiple lower cranial nerve palsies but reached remission by immunosuppressant after the diagnosis of WG. A 54-yr-old female visited with headache, hearing difficulty, and progressive bulbar symptoms. She experienced endoscopic sinus surgeries due to refractory sinusitis. Neurologic examination revealed multiple lower cranial nerve palsies. Vasculitic markers showed no abnormality. Nasal biopsy revealed granulomatous inflammation and vasculitis involving small vessels. Given cyclophosphamide and prednisolone, her symptoms were prominently improved. WG should be considered in the patient with multiple cranial nerve palsies, especially those with paranasal sinus disease. Because WG can be lethal if delayed in treatment, prompt immunosuppressant is warranted after the diagnostic tissue biopsy.     

Screening of Dihydropyrimidine Dehydrogenase Genetic Variants by Direct Sequencing in Different Ethnic Groups

Dihydropyrimidine dehydrogenase (DPYD) is an enzyme that regulates the rate-limiting step in pyrimidine metabolism, especially catabolism of fluorouracil, a chemotherapeutic agent for cancer. In order to determine the genetic distribution of DPYD, we directly sequenced 288 subjects from five ethnic groups (96 Koreans, 48 Japanese, 48 Han Chinese, 48 African Americans, and 48 European Americans). As a result, 56 polymorphisms were observed, including 6 core polymorphisms and 18 novel polymorphisms. Allele frequencies were nearly the same across the Asian populations, Korean, Han Chinese and Japanese, whereas several SNPs showed different genetic distributions between Asians and other ethnic populations (African American and European American). Additional in silico analysis was performed to predict the function of novel SNPs. One nonsynonymous SNP (+199381A > G, Asn151Asp) was predicted to change its polarity of amino acid (Asn, neutral to Asp, negative). These findings would be valuable for further research, including pharmacogenetic and drug responses studies.     

Long-term Outcomes of Endoscopic Variceal Ligation to Prevent Rebleeding in Children with Esophageal Varices

After an episode of acute bleeding from esophageal varices, patients are at a high risk for recurrent bleeding and death. However, there are few reports regarding the long-term results of secondary prophylaxis using endoscopic variceal ligation (EVL) against variceal rebleeding in pediatrics. Thirty-seven, who were followed for over 3 yr post-eradication, were included in the study. The mean duration of follow up after esophageal variceal eradication was 6.4±1.9 yr. The mean time required to achieve the eradication of varices was 3.25 months. The mean number of sessions and O-bands needed to eradicate varices was 1.9±1.2 and 3.8±1.5, respectively. During the period before the first EVL treatment, 145 episodes of bleedings developed in 37 children. Over the 3 yr of follow-up after variceal eradication, only 4 episodes of rebleeding developed in 4 of 37 patients. The four rebleeding episodes consisted of an esophageal variceal bleed, a gastric variceal bleed, a duodenal ulcer bleed, and a bleed caused by hemorrhagic gastritis. There was no mortality during long-term follow up after variceal eradication. During long-term follow up after esophageal variceal eradication using solely EVL in children with esophageal variceal bleeds, rebleeding episodes and recurrence of esophageal varices were rare. EVL is a safe and highly effective method for the long-term prophylaxis of variceal rebleeding in children with portal hypertension.     

Pulmonary Tuberculosis and Lung Cancer Risk in Current Smokers: The Seoul Male Cancer Cohort Study

Authors evaluated pulmonary tuberculosis (PTB) history as a risk factor for lung cancer in current male smokers in a prospective, population-based cohort study. The subjects were the 7,009 males among the participants in the Seoul Male Cancer Cohort Study for whom there was full information on PTB history and smoking habits. With a 16-yr follow-up, 93 cases of lung cancer occurred over the 99,965 person-years of the study. The estimated relative risk (RR) of PTB history of current smokers in lung cancer after adjusting for three confounders - intake of coffee and tomatoes, and age at entry - was 1.85 (95% CI: 1.08-3.19). The observed joint RRs and attributable risks (ARs) across strata of three confounders were greater than the expected, indicating a positive interaction. Thus a history of PTB in current smokers may be another risk factor for lung cancer. Based on a synergic interaction, a heavy male smoker with a PTB history would be expected to belong to the group at high risk of lung cancer.     

Comparison of Complications between Endoscopic and Percutaneous Replacement of Percutaneous Endoscopic Gastrostomy Tubes

When replacing percutaneous endoscopic gastrostomy (PEG) tubes, an internal bolster may be retrieved either percutaneously or endoscopically. The aim of this study was to compare the complications of percutaneous and endoscopic method during PEG tube replacement. The medical records of 330 patients who received PEG tube replacement were retrospectively analyzed. According to the removal method of internal bolster, we categorized as endoscopic group and percutaneous group. Demographic data, procedure-related complications and risk factors were investigated. There were 176 cases (53.3%) in endoscopic group and 154 cases (46.7%) in percutaneous group. The overall immediate complication rate during PEG tube replacement was 4.8%. Bleeding from the stoma (1.3%) occurred in percutaneous group, whereas esophageal mucosal laceration (7.4%) and microperforation (0.6%) occurred in endoscopic group. The immediate complication rate was significantly lower in the percutaneous method (OR, 6.57; 95% CI, 1.47-29.38, P=0.014). In multivariate analysis, old age was a significant risk factor of esophageal laceration and microperforation during PEG tube replacement (OR, 3.83; 95% CI, 1.04-14.07, P=0.043). The percutaneous method may be more safe and feasible for replacing PEG tubes than the endoscopic method in old patients.     

De Vega Annuloplasty for Functional Tricupsid Regurgitation: Concept of Tricuspid Valve Orifice Index to Optimize Tricuspid Valve Annular Reduction

We evaluated long-term results of De Vega annuloplasty measured by cylindrical sizers for functional tricuspid regurgitation (FTR) and analyzed the impact of measured annular size on the late recurrence of tricuspid valve regurgitation. Between 2001 and 2011, 177 patients (57.9±10.5 yr) underwent De Vega annuloplasty for FTR. Three cylindrical sizers (actual diameters of 29.5, 31.5, and 33.5 mm) were used to reproducibly reduce the tricuspid annulus. Long-term outcomes were evaluated and risk factor analyses for the recurrence of FTR ≥3+ were performed. Measured annular diameter indexed by patient''s body surface area was included in the analyses as a possible risk factor. Operative mortality occurred in 8 patients (4.5%). Ten-year overall and cardiac death-free survivals were 80.5% and 90.8%, respectively. Five and 10-yr freedom rates from recurrent FTR were 96.5% and 93.1%, respectively. Cox proportional hazard model revealed that higher indexed annular size was the only risk factor for the recurrence of FTR (P=0.006). A minimal P value approach demonstrated that indexed annular diameter of 22.5 mm/m² was a cut-off value predicting the recurrence of FTR. De Vega annuloplasty for FTR results in low rates of recurrent FTR in the long-term. Tricuspid annulus should be reduced appropriately considering patients'' body size to prevent recurrent FTR.     

Hematologic Recovery after Tandem High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Children with High-Risk Solid Tumors

Although the number of studies using tandem high-dose chemotherapy and autologous stem cell transplantation (HDCT/autoSCT) for the treatment of high-risk pediatric solid tumors has been increasing, documentation of hematologic recovery after tandem HDCT/autoSCT is very limited. For this reason, we retrospectively analyzed the hematologic recovery of 236 children with high-risk solid tumors who underwent tandem HDCT/autoSCT. The median numbers of CD34⁺ cells transplanted during the first and second HDCT/autoSCT were 4.3 × 10⁶/kg (range 0.6-220.2) and 4.1 × 10⁶/kg (range 0.9-157.6), respectively (P = 0.664). While there was no difference in neutrophil recovery between the first and second HDCT/autoSCT, platelet and RBC recoveries were significantly delayed in the second HDCT/autoSCT (P < 0.001 and P < 0.001, respectively). Delayed recovery in the second HDCT/autoSCT was more prominent when the number of transplanted CD34⁺ cells was lower, especially if it was < 2 × 10⁶/kg. A lower CD34⁺ cell count was also associated with increased RBC transfusion requirements and a higher serum ferritin level after tandem HDCT/autoSCT. More CD34⁺ cells need to be transplanted during the second HDCT/autoSCT in order to achieve the same hematologic recovery as the first HDCT/autoSCT.     

Influence of Body Mass Index,Smoking, and Blood Pressure on Survival of Patients with Surgically-Treated,Low Stage Renal Cell Carcinoma: A 14-Year Retrospective Cohort Study

The association of body mass index, smoking, and blood pressure, which are related to the three well-established risk factors of renal cell carcinoma, and survival in patients with renal cell carcinoma is not much studied. Our objective was to evaluate this association. A cohort of 1,036 patients with low stage (pT1 and pT2) renal cell carcinoma who underwent radical or partial nephrectomy were enrolled. We retrospectively reviewed medical records and collected survival data. The body mass index, smoking status, and blood pressure at the time of surgery were recorded. Patients were grouped according to their obesity grade, smoking status, and hypertension stage. Survival analysis showed a significant decrease in overall (P = 0.001) and cancer-specific survival (P < 0.001) with being underweight, with no differences of smoking status or perioperative blood pressure. On multivariate analysis, perioperative blood pressure ≥ 160/100 mmHg (HR, 2.642; 95% CI, 1.221-5.720) and being underweight (HR, 4.320; 95% CI, 1.557-11.984) were independent predictors of overall and cancer-specific mortality, respectively. Therefore, it is concluded that being underweight and perioperative blood pressure ≥ 160/100 mmHg negatively affect cancer-specific and overall survival, respectively, while smoking status does not influence survivals in patients with renal cell carcinoma.