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Congenital epidermolysis bullosa is a rare, genetic condition in which even slight stimulation can cause blistering of the skin or mucosa. While previous reports of treatments requiring general anesthesia in these patients were focused on anesthesia-related procedures, such as endotracheal intubation, no report has described specific management required for these patients during surgery, such as preparation of the surgical site, fixation of infusion lines and other tubes, and adjustment of the operation table. This is probably the first report to address these issues. This report presents a case of recessive dystrophic congenital epidermolysis bullosa in which open hepatectomy was safely performed.

Background Intracranial pseudoaneurysm formation due to a ruptured non-traumatic aneurysm is extremely rare. We describe the radiological findings and management of pseudoaneurysms due to ruptured cerebral aneurysms in our case series and previously reported cases. Patients and methods Four additional and 20 reported patients presenting with subarachnoid hemorrhage (SAH) are included. Radiological findings and clinical features of these patients were reviewed. Results In our series, three-dimensional computed tomographic angiography (3D-CTA) and/or angiography showed an irregular- or snowman-shaped cavity extending from the parent artery. The radiological examination additionally revealed delayed filling and retention of contrast medium. These findings were the same as previously reported cases. One patient underwent direct clipping of the true aneurysm. For the other three patients with aneurysms at the basilar and anterior communicating arteries, the true portion of the aneurysm was embolized with platinum coils. During the procedures, care was taken not to insert the coils into the distal pseudoaneurysm portion to prevent rupture. The review of 24 cases revealed that the location of the aneurysms was most frequent in the anterior communicating artery (41.7%), and 86.7% of patients were in a severe stage of SAH (>Grade 3 in WFNS or Hunt & Kosnik grading) implying abundant SAH. Conclusions Pseudoaneurysm formation in SAH after non-traumatic aneurysm rupture is rare. However, in cases with an irregular-shaped aneurysm cavity, pseudoaneurysm formation should be taken into consideration.

Portal vein thrombosis (PVT) is a rare but serious postoperative complication of pancreaticoduodenectomy (PD). We reported a case of late-onset postoperative PVT with hemorrhage from the common hepatic artery (CHA) in a 73-year-old man who underwent pylorus-preserving pancreaticoduodenectomy (PPPD) for duodenum papilla cancer, followed by reconstruction using the modified Child's technique. The pancreaticojejunostomy was achieved by end-to-side, 2-layer invagination anastomosis without pancreatic duct stenting. Drain removal and hospital discharge were scheduled on postoperative day (POD) 18, but blood-stained fluid in the drain and sudden hematemesis were noted. Emergency surgery was performed because PVT and imaging findings were suggestive of necrosis of the lifted jejunum. Although no jejunal necrosis was identified during surgery, bleeding from the side of the CHA was detected and the bleeding point was suture-closed to achieve hemostasis. We suspected late-onset postoperative arterial hemorrhage and subsequent hematoma formation, which caused portal vein compression and PVT formation. We chose a conservative treatment strategy for PVT, taking into account the operation time, intraoperative vital signs and blood flow in the portal vein. Despite the complicated postoperative course, he was discharged home in a fully ambulatory state on POD 167.

Primary small cell carcinoma of the breast is a rare breast cancer. We report two cases of this entity showing a non-mass-like pattern on multimodality images and histopathology. Both women presented with a breast mass, and one had axillary lymphadenopathy. Both cases revealed fine calcification on mammography (MMG) and an irregularly shaped, hypoechoic lesion on ultrasonography. Computed tomography (CT) and magnetic resonance imaging (MRI) showed non-mass-like enhancement in both cases. Dynamic MRI showed medium initial enhancement followed by persistent delayed enhancement in one patient, whereas rapid initial enhancement with plateau delayed enhancement was observed in the other. The breast lesions showed very high signal intensity on diffusion-weighted MRI. Positron emission tomography showed moderate accumulation of 2-fluoro-2-deoxyglucose in the breast tumor and lymph node metastasis. The non-mass-like enhancement on CT and MRI and the segmental fine calcification on MMG indicate the abundance of components of ductal carcinoma in situ and the breast origin of the small cell carcinoma.

Background/aims: Perioperative management of pancreaticoduodenectomy (PD) is a constant dilemma and challenging for gastrointestinal surgeons. Postoperative pancreatic fistula (POPF) and delayed gastric emptying (DGE) are of particular concern, and the onset of these conditions indicates a prolonged postoperative stay (POS). The procedure and postoperative outcomes of pancreaticojejunostomy performed at our hospital are reported., Methodology: POPF, DGE, and POS were investigated in 54 patients who had undergone PD at our hospital since June 2007. Pancreaticojejunal end-to-side anastomosis using the invagination method without a stenting tube and without duct-to-mucosa anastomosis was performed in all patients, regardless of pancreatic duct diameter., Results: There were 26 patients (48.2%) without POPF, 24 (44.4%) with grade A, 4 (7.4%) with grade B, and none with grade C. The mean POS was 28.3 days. DGE was observed in 4 patients (7.4%) who underwent pylorus-preserving PD (PpPD). There were 34 patients with a soft pancreas. None of the patients experienced intraperitoneal bleeding or abscess, and no surgery-related deaths occurred., Conclusions: The reconstructive pancreaticojejunostomy procedure performed at our hospital appears to be safe and convenient, and we plan to collect additional data, including assessments of the function of the remaining pancreas, in the future.

Duplication of the alimentary tract is a rare congenital malformation that occurs most often in the abdominal region, whereas esophageal duplication cyst develops typically in the thoracic region but occasionally in the neck and abdominal regions. Esophageal duplication cyst is usually diagnosed in early childhood because of symptoms related to bleeding, infection, and displacement of tissue surrounding the lesion. We recently encountered a rare adult case of esophageal duplication cyst in the abdominal esophagus. A 50-year-old man underwent gastroscopy, endoscopic ultrasonography, computed tomography, and magnetic resonance imaging to investigate epigastric pain and dysphagia that started 3 months earlier. Imaging findings suggested esophageal duplication cyst, and the patient underwent laparoscopic resection followed by intraoperative esophagoscopy to reconstruct the esophagus safely and effectively. Histopathological examination of the resected specimen revealed two layers of smooth muscle in the cystic wall, confirming the diagnosis of esophageal duplication cyst.

We experienced a very rare case of occult breast lobular carcinoma with numerous circulating tumor cells in peripheral blood. The diagnosis was very difficult because there were no symptoms of breast cancer and the preceding chief complaints such as general fatigue and weight loss or abnormality of peripheral blood findings were suggestive of a hematological disease. We could make a correct diagnosis of this case by checking the findings of complete blood count and bone marrow biopsy at the same time using immunohistochemistry.

An 85-year-old woman was admitted to our hospital for steroid therapy for relapsing nephrotic syndrome. During hospitalization, she complained of sudden epigastric pain at night. Although there were signs of peritoneal irritation, CT showed a large amount of ascitic fluid, but no free intraperitoneal gas. Gram staining of ascitic fluid obtained by abdominal paracentesis showed Gram-negative rods, which raised a strong suspicion of gastrointestinal perforation and peritonitis. Therefore, emergency surgery was performed. Exploration of the colon showed multiple sigmoid diverticula, one of which was perforated. The patient underwent an emergency Hartmann's procedure. Imaging studies failed to reveal any evidence of gastrointestinal perforation, presenting a diagnostic challenge. However, a physician performed rapid Gram staining of ascitic fluid at night when laboratory technicians were absent, had a strong suspicion of gastrointestinal perforation, and performed emergency surgery. Gram staining is superior in rapidity, and ascitic fluid Gram staining can aid in diagnosis, suggesting that it should be actively performed. We report this case, with a review of the literature on the significance of rapid diagnosis by Gram staining.

A monodisperse molecularly imprinted polymer (MIP) for curcumin was first prepared by precipitation polymerization using methacrylamide (MAM) and 4-vinylpyridine as functional co-monomers, divinylbenzene as a crosslinker, and a mixture of acetonitrile and toluene as a porogen. The use of MAM as the co-monomer resulted in the formation of a monodisperse MIP and non-imprinted polymer (NIP). MIP and NIP, respectively, were monodispersed with a narrow particle size distribution (3.3 ± 0.09 and 3.5 ± 0.10 μm). In addition to shape recognition, hydrophobic and hydrogen-bonding interactions affected the retention and molecular-recognition of curcumin on the MIP. The MIP for curcumin could extract curcuminoids (curcumin, demethoxycurcumin, and bisdemethoxycurcumin) in Curcuma longa L.

Since superior mesenteric vein thrombosis (SMVT) is a relatively rare disease and shows no specific symptom, its diagnosis tends to be delayed. In this report, we present a patient in whom acute appendicitis was complicated by SMVT and portal vein thrombosis (PVT). A definitive diagnosis could be made by abdominal contrast-enhanced CT, and acute appendicitis was surgically treated. Anticoagulant therapy was continued for about half a year after surgery. Abdominal contrast-enhanced CT after discharge showed no recurrence of SMVT or PVT. We consider that acute appendicitis induced SMVT or PVT caused by the effect of inflammation. There is the possibility that these conditions lead to intestinal congestion or necrosis and liver dysfunction; appropriate diagnosis and treatment are necessary.

Heat shock protein 90α (Hsp90α) immobilized on aminopropyl silica gels was prepared via the N- or C-terminal, which was termed Hsp90α-NT or Hsp90α-CT, respectively. Binding interactions of biscoclaurine alkaloids (cepharanthine (CEP), berbamine (BBM), isotetrandrine (ITD), and cycleanine (CCN)) with Hsp90α were examined using the Hsp90α-NT or -CT columns by frontal and zonal chromatography studies. The dissociation constants of CEP, BBM, ITD, and CCN to Hsp90α-NT were estimated to be 5.3, 18.6, 46.3, and 159 μM, respectively, by frontal chromatography techniques. Similar results were obtained with the Hsp90α-CT column. These data suggest that these biscoclaurine alkaloids interact with the middle domain of Hsp90α. This was confirmed by demonstrating that CEP competed with endothelial nitric oxide synthase at the middle domain of Hsp90α, where it was shown to have a dissociation constant of 15 nM. Furthermore, the Hsp90α-NT column was applied for preliminary screening of natural Hsp90α inhibitors by zonal chromatography studies., (Copyright © 2012 Elsevier Inc. All rights reserved.)

We investigated the effectiveness of peptide nucleic acid (PNA) clamp PCR for detecting KRAS mutations in peripheral blood samples of colorectal cancer (CRC) patients. We compared KRAS point mutations between tumour tissue and blood samples. Forty-two patients were included in this study. We observed KRAS mutations in formalin-fixed, paraffin-embedded tissues by PCR direct sequencing and in blood samples by PNA clamp PCR. KRAS point mutations were detected in primary tumour tissue samples of 13 patients (31.0%) and in peripheral blood samples of 10 patients (23.8%). KRAS point mutations were detected in both samples for 8 patients (19.0%). The sensitivity, specificity and accuracy for detecting KRAS mutations in peripheral blood and tumour tissue samples were 61.5, 93.1 and 83.3%, respectively. The positive and negative predictive values were 80.0 and 84.4%, respectively. Five patients with mutant KRAS in their plasma preoperatively, did not exhibit KRAS mutations postoperatively. Our method detected KRAS point mutations in peripheral blood samples of CRC patients, which contained extremely small amounts of mutant cells. This method is helpful for identifying metastatic CRC patients in whom metastases will respond to EGFR-targeted monoclonal antibody therapy.

Background/aims: Intraoperative ultrasonography (IOUS) has been used to delineate anatomy during laparoscopic cholecystectomy (LapC) and screen for common bile duct stones (CBDS), however no consensus about its efficacy has been reached in Japan. In this study, we compared and evaluated the performance of intraoperative cholangiography(IOC) and IOUS during LapC., Methodology: We evaluated 295 cases of cholecystolithiasis that underwent surgery from 2005- 2009 at our hospital. Frequency of CBDS and duration of surgery was examined in cases with IOC alone or IOC and IOUS in combination., Results: There were 5 cases (1.7%) in which IOUS or IOC detected CBDS that had not been detected by preoperative evaluation using abdominal CT, abdominal ultrasonography or MRCP. Out of 62 LapC cases involving both IOC and IOUS, 60 cases showed the same findings, i.e. no false-negative results, indicating that IOUS is a trustworthy examination method. The mean duration of surgery involving IOUS alone was 135 minutes while a longer mean time of 163 minutes was found for that involving IOC alone or IOC and IOUS in combination., Conclusions: This study shows that IOC can be considered useful as it did not produce any false-negative results in screening CBDS and it also shortened the operation time. We also found some problems associated with IOC, such as small calculi falling within the gallbladder during examination procedures and the irradiation of patients and staff.

Aims: Sometimes, mastitis needs to be differentiated from carcinoma because of its association with induration and with ultrasound findings (such as low-echo lesions) that resemble those in carcinoma. The aim was to define this type of mastitis and to examine 18 cases to clarify its clinicopathological features., Methods and Results: All cases were categorized into three types: non-specific mastitis with neutrophilic infiltration (n = 7); non-specific mastitis with lymphoplasmacytic infiltration (n = 9); and granulomatous lobular mastitis (n = 2). The three types of mastitis presented similar ultrasound findings and shared certain histological features including fibrosis and diffuse or lobulocentric inflammation. Granulomatous lobular mastitis showed specific clinicopathological features including lobulocentric inflammation with giant cells, diffuse IgG4+ plasma cells, and also a high level of serum IgG4., Conclusions: Granulomatous lobular mastitis could be categorized into IgG4-related and non-IgG4-related granulomatous lobular mastitis. IgG4 immunohistochemistry serum IgG4 might be useful for diagnosis of IgG4-related granulomatous lobular mastitis and could help to avoid overtreatment such as wide excision.

A 60-year-old woman with Stage II, ER-positive, PgR-positive, HER2 (2+) cancer in the right breast underwent right mastectomy with right axillary dissection after chemotherapy with EC followed by docetaxel (DOC) alone. Exemestane was used for postoperative adjuvant treatment. She underwent a right chest wall tumor resection for local recurrence. Hormone therapy was continued with toremifene in place of exemestane. In December 2007, two years after the second surgery, CEA was elevated and PET showed a local recurrence in the right chest wall and metastases to the right axillary nodes and liver. The tumor was ER-positive, PgR-negative and HER2 (3+) at recurrence, and vinorelbine/trastuzumab combination was initiated as first-line chemotherapy for the recurrent lesion and liver metastasis. All lesions in the right chest wall, right axillary nodes and liver disappeared from PET and CT images after five courses of the regimen, resulting in clinical CR. Vinorelbine combined with trastuzumab appears to be a useful therapy for HER2-positive recurrent breast cancer.

Cellular membranes obtained from the 1321N1 and A172 astrocytoma cell lines were immobilized on a chromatographic phase to create cellular membrane affinity chromatography (CMAC) columns, CMAC(1321N1) and CMAC(A172). The columns were characterized using frontal affinity chromatography with [(3)H]-epibatidine as the marker ligand and epibatidine, nicotine, and methyllycaconitine as the displacers. The results indicated that the columns contained homomeric alpha7 nicotinic acetylcholine receptors (alpha7 nAChR) and heteromeric nicotinic acetylcholine receptors (alpha(x)beta(y) nAChRs), which was confirmed by the addition of subtype-specific inhibitors, alpha-bungarotoxin (alpha7 nAChR) and kappa-bungarotoxin (alpha(x)beta(y) nAChR) to the mobile phase. The presence of two additional ligand-gated ion channels (LGICs), gamma-aminobutyric acid (GABA(A)) and N-methyl-D-aspartic acid (NMDA), was established using frontal affinity chromatography with flunitrazepam and diazepam (GABA(A) receptor) and MK-801 and NMDA (NMDA receptor). The presence of the four LGICs was confirmed using confocal microscopy and flow cytometry. The results indicate that the CMAC(1321N1) and CMAC(A172) columns contain four independently functioning LGICs, that the columns can be used to characterize binding affinities of small molecules to each of the receptors, and that the CMAC approach can be used to probe the expression of endogenous membrane receptors.

We performed FOLFOX 4 therapy in a patient with lung metastasis (a 62-year-old woman) after surgery for rectal cancer and observed both normalization of tumor markers and disappearance of the metastasis. Low anterior resection was performed for progressive rectal cancer, and treatment with UFT and folinate was started one month after surgery. However, tumor markers increased after 2 months of treatment and CT scans showed metastases to both lungs. FOLFOX 4 therapy was started, and tumor markers became normal after four courses, while the lung metastases disappeared after 10 courses. The dosage of FOLFOX 4 was reduced after three courses due to neutropenia and diarrhea. This treatment appeared to be effective for the inhibition of lung metastasis associated with colorectal cancer.

We report a case of massive melena caused by a carcinoid of the small intestine. A 28-year-old woman was admitted to our department after presenting with massive melena. The source of the bleeding could not be localized by upper or lower gastrointestinal endoscopy, computed tomography (CT), or labeled red blood cell scintigraphy. Enteroscopy allowed visualization only up to the jejunum, and we could not localize the lesion. The melena subsided with conservative treatment, but the patient was readmitted 4 months later when she suffered another episode of massive melena. A contrast CT scan performed immediately showed extravascular leakage and the retention of contrast medium in the ileum. Thus, she underwent an emergency surgery, during which endoscopy confirmed a small ileal tumor accompanied by pulsating bleeding from the exposed blood vessels at its center. The small intestine was partially resected, including the swollen lymph nodes, the size of small beans. Pathological examination confirmed a carcinoid tumor 1 cm in diameter, with an arterial rupture at its center and lymph node metastasis.

Purpose: Our aim was to investigate the thymidine phosphorylase (TdRPase) expression and Ki-67 labeling index (LI) of primary tumors of colorectal cancer and hepatic metastases immunochemically and to evaluate the relationship of these parameters to various clinicopathologic factors and hepatic metastasis., Methods: We performed immunochemical studies in 74 patients with colorectal cancer, using anti-TdRPase antibody and MIB-1 antibody., Results: TdRPase expression and a Ki-67 LI > or =30% in primary lesions were significantly more common in patients with lymphatic invasion (ly), venous invasion (v), lymph node involvement (n), and hepatic metastasis. A Ki-67 LI > or = 30% of the primary tumor was associated with a significantly higher frequency of metachronous liver metastasis. In the same patients, the mean Ki-67 LI was 24.3+/-17.9 for primary lesions and 5.0+/-4.2 for hepatic metastases, this difference being significant., Conclusions: These results indicate that TdRPase expression and the Ki-67 LI are related to various clinicopathologic factors, suggesting their usefulness as indices of tumor malignancy. We suggest that the Ki-67 LI of primary colorectal cancer could be an important predictor of the future development of metachronous liver metastasis.

In Japan after 1969, 11 cases of aplastic anemia following radiotherapy for malignant disease were detected. The population at risk in irradiated patients was estimated at 674, 664 man-years. The expected cases of aplastic anemia in this population were calculated as 10.1. There is no statistically significant difference between the expected and the observed values.

A model of tumor regression during the course of radiotherapy is presented, which considered the lethal effect of radiation, the elimination of dying cells and the reproduction of surviving cells. The model was applied to multiple lung metastases in a woman, which were treated with different doses per fraction. The regression of tumor volume was measured from chest radiographs which were taken once a week during the treatment. Radiosensitivity of tumor cells estimated by the model was of D0=150 R and n=5. The half life of dying cells in the tumor was 15 days.

Patients with pulmonary tuberculosis treated in 4 sanatoria in Niigata prefecture during the period from 1941 to 1961 were followed up. Of 2,756 patients, 1,193 responded and sent us effective information. However, 1,224 did not come into contact because of changed and unknown adress, 326 did not respond, and 13 were omitted because of incomplete answers. Of 1,193 effective responders, 568 had been treated by artificial pneumothorax (the pneumothorax group), and 552 had been treated by the other measures than pneumothorax (the control group). There were 65 deaths in the pneumothorax, group, and 40 deaths in the control group. No deaths from chest malignancies were reported in the pneumothorax group, while 4 chest malignancies in the control. In this survey, no evidence of increased frequency of chest malignancies including leukemia following multiple fluoroscopies during artificial pneumothorax was detected.

Among the Japanese radiological technicans, four deaths from aplastic anemia have been reported after 1930. On the other hand, during the period from 1930 to 1960, the population of radiological technicians is estimated to be 74,400 man-years, in which 0.5 aplastic anemias are expected. However, actually three died from aplastic anemia. This difference is statistically significant at the 1% level. While, in the period from 1961 to 1973, the observed value is 1 against 0.7 expected. It is concluded that aplastic anemia had been induced frequently among the Japanese radiological technicians in the era when there was much exposure to occupational radiation.