Vascular type of Ehlers-Danlos syndrome (EDS), also known as EDS type IV (NIM#130050) is a life-threatening autosomal dominant inherited disorder of connective tissue, caused by mutations of the COL3A1 gene. Vascular EDS causes severe fragility of connective tissues with arterial and intestinal ruptures and complications of surgical and radiological treatment, and is of particular importance to medical professionals of many specialties: surgeons, internists, radiologists, and obstetricians. An accurate diagnosis may help in the management of visceral complications. In addition, when a family is faced with new information concerning a positive genetic test for vascular EDS, it is crucial that follow-up care for the family include comprehensive genetic counseling. After the genetic diagnosis of a COL3A1 mutation, various medical specialists, including the clinical geneticists must cooperate to perform genetic counseling and to provide a system of long-term follow up for individuals with vascular EDS.

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Background: Pleural effusions develop for various reasons in patients admitted to intensive care units (ICUs). To understand why this occurs is important, yet cytokine levels in pleural effusions have rarely been measured from a cardiovascular viewpoint.
Objective: To understand the characteristics of pleural cytokines in patients admitted to the ICU.
Methods: The subjects were 43 patients with pleural effusion who were admitted to the ICU from June 2001 through March 2006. We divided the patients into transudate (n=23) and exudate (n=20) groups. We measured levels of interleukin (IL)-6, IL-10, and tumor necrosis factor (TNF)-α in pleural effusions and peripheral blood and evaluated their relationships with body temperature, C-reactive protein (CRP) level, and the peripheral white blood cell (WBC) count.
Results: Levels of pleural IL-6 were significantly higher and levels of TNF-α tended to be higher in pleural effusions from the exudate than in those from the transudate group (3,350 ± 3,627 vs. 1,677 ± 1,086 pg/m and 6.6 ± 3.4 vs. 4.8 ± 2.6 pg/mL, respectively). However, in both groups levels of IL-10 in pleural effusions were similar to those in serum and levels of IL-6 were significantly higher in pleural effusion than in serum. Serum IL-6 levels correlated with inflammatory markers (CRP and body temperature), whereas cytokines in pleural effusion did not correlate with any of these markers (body temperature, CRP, and WBC).
Conclusion: Pleural levels of IL-6 were significantly higher in the exudate group than in the transudate group but did not correlate with serum levels of IL-6 or with systemic inflammatory markers. These findings suggest that pleural IL-6 levels correlate with local lung or pleural inflammation in patients admitted to the ICU.

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In this case-control study, we examined infants delivered vaginally at 37 weeks or later to identify factors associated with transient tachypnea of the newborn (TTN). We reviewed the obstetric records of all vaginal deliveries at the Japanese Red Cross Katsushika Maternity Hospital from 2005 through 2007. Demographic information and the characteristics of labor were extracted from patient charts. Multivariate analysis identified that the incidence of TTN was significantly associated with nulliparity; a history of infertility therapy, such as in vitro fertilization; augmentation of labor; nonreassuring fetal status; vacuum/forceps delivery; and low Apgar score (<7) at 1 and 5 minutes. In addition, a low Apgar score at 1 minute was the factor most strongly associated with the incidence of TTN (adjusted odds ratio, 20; 95% confidence intervals, 12-34; p<0.001). The present results indicate that the improvement of obstetric surveillance to diminish the frequency of low Apgar scores is important for preventing TTN in infants delivered vaginally at 37 weeks or later.

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Objective: The aim of this study was to see if curved baselines of F-waves could be mathematically approximated with universal spreadsheet software.
Methods: The subjects were 3 healthy persons and 3 patients with cervical myelopathy. Supramaximal electrical stimuli were applied 200 times to the median nerve at the wrist. Compound muscle action potentials (CMAPs) of the abductor pollicis brevis were recorded. To make polynomial approximation equations that represent latter part of the M-waves, records without F-waves were analyzed.
Results: There were 193 CMAPs without F-waves out of all 1,200 records. Polynomial equations were made for each record. Determinant coefficients for all the approximation equations were greater than 0.998, and the overall standard deviation of the difference between original data and approximated value was 3.05 μV.
Conclusions: Curved baselines of F-waves were represented by approximation curves. Baselines of the F-waves could be approximated as flat lines by subtracting calculated values from the original data.
Significance: This method was useful for analyzing waveforms of F-waves.

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Patients who undergo definitive chemoradiotherapy (CRT) face a risk of residual resistant disease or disease recurrence at the primary site; therefore, salvage treatment may be required. An optimum strategy to minimize these risks clearly needs to be established. Argon plasma coagulation (APC) is a safe and convenient procedure now applied widely for therapeutic endoscopy. In this report we describe the successful use of APC over 6 years for the treatment of recurrent esophageal cancer after CRT. A 61-year-old Japanese man underwent CRT for a thoracic esophageal cancer. Pathological examination before CRT revealed a well-differentiated squamous cell carcinoma. Locoregional failure was observed 2 years after CRT, and an initial APC treatment was performed. The patient has now undergone APC ablation 7 times with no postoperative complications. No metastasis to lymph nodes or to other organs has been detected during the last 6 years. The usefulness of APC as a salvage treatment for locoregional failure after definitive CRT has not been established. In our experience, salvage APC is the best treatment option for some patients.

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Bile duct invasion is rare in patients with hepatocellular carcinoma (HCC). We show the usefulness of selective transcatheter hepatic arterial embolization (TAE) followed by microwave coagulation therapy (MCT) in a case of HCC with portal and biliary tumor thrombi that ruptured into the biliary system. A 70-year-old man with HCC was admitted because of melena and postprandial abdominal pain. Four years earlier, he had undergone posterior segmentectomy of the liver for HCC. Portal venous thrombus was detected on computed tomography (CT) 3 months earlier. On admission laboratory tests revealed the following values: serum alkaline phosphatase, 760 IU/L; total serum bilirubin, 11.9 mg/dL; direct bilirubin, 9.8 mg/dL; serum hemoglobin, 7.7 g/dL; alpha-fetoprotein 103.9 ng/mL; and PIVKA-2, 52,655 mAU/mL. Serum examinations were positive for anti-hepatitis C virus antibody but negative for hepatitis B surface antigens. Ultrasonography revealed a hypoechoic mass in the right branch of the bile duct at the hepatic hilum. Doppler ultrasonography showed blood flow in the mass. CT showed diffuse tumor involvement throughout the liver parenchyma and the presence of a high-density substance in the right intrahepatic bile duct. The diagnosis was hemobilia secondary to HCC in the right hepatic lobe. The symptoms recurred, and emergency TAE was performed 5 days after the onset of hemobilia. The symptoms subsided, and liver function improved. Endoscopic retrograde cholangiography revealed obstruction of the right intrahepatic bile duct. Surgery was performed 15 days after TAE, and MCT of the right hepatic hilum was performed. After MCT, CT revealed necrosis of the right hepatic hilum. Seven months after TAE, the patient died of liver failure with no recurrence of hemobilia.

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A 16-year-old adolescent boy was admitted to our hospital with severe lower abdominal pain and was found to have peritonitis, probably caused by acute appendicitis. At laparotomy, we found a paper-thin, dilated sigmoid colon; the seromuscular layer on the antimesenteric side was torn, and the untorn mucosa showed a pinpoint perforation. The seromuscular defect had spread circumferentially to involve the entire circumference of the colon wall. We performed sigmoidectomy, and the patient recovered uneventfully. This case showed many similarities, both in terms of the macroscopic and pathological findings, to seromuscular tear, an entity specifically associated with seatbelt use. This case is noteworthy because seromuscular tear-like lesions of the colon without a history of trauma has not previously been reported.

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Biliary cystadenocarcinomas are rare cystic tumors arising from the biliary epithelium. Symptoms vary and are sometimes absent. Most lesions are found incidentally during diagnostic radiologic procedures or surgical exploration for unrelated conditions. We describe an 80-year-old man with anemia due to intracystic bleeding. A computed tomographic scan showed a low-density hepatic lesion with thick, irregular walls and septa. Left lobectomy with cholecystectomy was performed. Histological examination revealed that the surgical specimen was a biliary cystadenocarcinoma. The concentrations of both carcinoembryonic antigen and CA19-9 were high in the cyst contents. The patient died of pneumonia 1 year 4 months after the operation, without the recurrence of cystadenocarcinoma.

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A 67-year-old woman was admitted to our hospital for surgical management of cancer of the ascending colon. On admission, she had cholangitis due to choledocholithiasis. Abdominal computed tomography, ultrasonography, and magnetic resonance showed cholelithiasis, choledocholithiasis, and multiple liver tumors. Colonoscope showed advanced cancer of the ascending colon. Because of acute obstructive suppurative cholangitis, endoscopic sphincterotomy was performed. During the procedure, periampullary retroperitoneal perforation was identified on radiologic examination. Because computed tomography had shown extravasation of contrast medium and widespread pneumoretroperitoneum, an emergency operation was performed 2 hours after perforation. After cholecystectomy and choledocholithotomy had been performed and all bile duct stones had been removed, periampullary perforation was readily identified close to the duodenal diverticula and easily repaired. The postoperative course was uneventful. This patient could resume oral feeding soon after the operation, and colonic surgery could be performed immediately thereafter. Therefore, early surgical management is a possible first choice of treatment in patients with remaining biliary disease after periampullary perforation.

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Spontaneous intracystic hemorrhage rarely occurs in nonparasitic hepatic cysts. We describe a patient with spontaneous intracystic hemorrhage of a large simple hepatic cyst that mimicked a malignancy. A 59-year-old man presented with right abdominal discomfort. The patient's medical history included a simple hepatic cyst that had been detected 7 years earlier but was left untreated. Three weeks before presentation, right upper abdominal pain occurred but resolved spontaneously. The serum CA19-9 concentration was 48.3 U/mL (normal<37 U/mL). Ultrasonography revealed a large cystic mass, containing many hyperechoic structures and occupying nearly the entire right hepatic lobe. Computed tomography demonstrated a homogenous low-density area, 20 cm in diameter, in the right hepatic lobe. Magnetic resonance imaging revealed a heterogeneous hypointense lesion measuring 20 cm in diameter. The lesions showed linear hyperintense areas on T1-weighted sequences and mosaic heterogeneous hyperintensity on T2-weighted sequences. We suspected a hemorrhagic simple hepatic cyst, hydatid cyst, or hemorrhagic cystadenocarcinoma. Right hepatectomy was performed, and the enlarged right lobe was removed. The mass was soft, sponge-like, and contained fluid, but was not elevated. Pathologic examination of the surgical specimen confirmed the presence of a hemorrhagic benign hepatic cyst.

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Here, we report on two patients with hemorrhagic shock due to hematemesis from a gastrointestinal stromal tumor (GIST) of the stomach. Patient 1 was a 64-year-old woman who was admitted to our hospital because of syncope due to hemorrhagic shock resulting from massive hematemesis. Emergent upper gastrointestinal (GI) endoscopy revealed a 5-cm-diameter submucosal tumor on the lesser curvature of the lower gastric body. In addition to the central ulceration of the tumor, a Dieulafoy-like lesion was present. Neither lesions showed active bleeding at the time of observation. Because the patient collapsed twice with fluminant hematemesis after admission, she underwent distal gastrectomy with Billroth-I reconstruction. Histological examination revealed a gastric GIST with no nodal metastasis and the mitotic count was less than 5 per 50 HPFs. Dilated vessels were prominent in the peritumoral submucosa, and a thrombus was seen in these vessels, which seemed to be a bleeding point. The patient had an uneventful postoperative course and has been alive without recurrence for 5 and a half years. Patient 2 was a 60-year-old man who presented with syncope due to hemorrhagic shock resulting from massive hematemesis. Because the source of the bleeding was not elucidated with an initial upper GI endoscopy, he was treated for a gastric ulcer. One week after admission, he suffered from hemorrhagic shock again, and a submucosal tumor 6 cm in size was revealed on the greater curvature of the upper stomach with upper GI endoscopy. The patient subsequently underwent wedge resection of the tumor. Histopathological findings were consistent with a GIST and the mitotic count was less than 5 per 50 high-power fields. The tumor showed no necrosis or intratumoral hemorrhage. A peritumoral submucosal artery, which was responsible for the massive hematemesis, was located at some distance away from the central ulceration. Postoperative recovery was without complications. After 4 years, the patient remains healthy and disease-free. Although hematemesis associated with gastric GIST has been said to originated from the central ulceration of the GIST, life-threatening, massive hematemesis is rare. The exact bleeding points of the gastric GISTs in these cases were submucosal vessels adjacent to the GIST, not the central ulceration. There have been no reports of peritumoral, submucosal vessels causing massive hematemesis from gastric GISTs. Because the origins and manner of bleeding varies in gastric GISTs, we must decide the methods of hemostasis immediately including the tumor excision.

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A 71-year-old woman visited our hospital for routine follow-up cystoscopy and urine cytologic examination. During the preceding 3 years she had undergone left nephroureterectomy for a tumor of the left renal pelvis and had undergone transurethral resection three times for tumors of the urinary bladder and urethra. A small flare region on the posterior wall of the bladder was found with regular cystoscopy, and urine cytologic examination was positive for malignant cells. Computed tomography showed no distant metastasis and no right upper urinary tract mass. Transurethral resection was performed for the small flare region in May 2006. A papillary tumor of the vaginal wall was found incidentally during transurethral resection, and, therefore, transvaginal resection of the tumor was performed at the same time. Histologic examination of the excised genital lesion showed a G3 pT2 urothelial carcinoma, and the patient has been under observation since completion of external radiotherapy for the vaginal metastatic nodule.

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