Extent of regional lymphadenectomy and number-based nodal classification for non-ampullary duodenal adenocarcinoma.

BACKGROUND: This study aimed to evaluate the adequate extent of regional lymphadenectomy according to tumor location and the impact of number-based nodal classification on survival in patients with non-ampullary duodenal adenocarcinoma (NADAC). METHODS: A total of 85 patients with NADAC who underwent surgery were enrolled. The frequency of metastasis was calculated for each node group in the respective tumor locations for 63 patients who underwent lymphadenectomy for pT2-pT4 tumor. RESULTS: The frequency of metastasis in the pancreaticoduodenal (nos. 13 and 17) and superior mesenteric artery (no. 14) nodes was high (16.7 %-52.3 %) regardless of tumor location. Metastasis in the perigastric (nos. 3 and 4d) and right celiac artery (no. 9) nodes was not uncommon (14.3 %-22.2 %) for tumors in the first portion. The frequency of metastasis in the pyloric (nos. 5 and 6) and the other peripancreaticoduodenal (nos. 8 and 12) nodes varied depending on tumor location but could not be ignored for staging. When these nodes were classified as regional nodes, the 5-year survival in patients with pN0, pN1 (1-2 positive nodes), and pN2 (≥3 positive nodes) were 82.9 %, 51.7 %, and 19.2 %, respectively (p < 0.001). pN classification independently predicted survival (pN1, p = 0.022; pN2, p < 0.001). CONCLUSIONS: Nos. 5, 6, 8, 12, 13, 14, and 17 nodes in all advanced NADAC and nos. 3, 4d, and 9 nodes in advanced NADAC in the first portion should be considered as regional nodes for accurate staging. The number-based nodal classification allows good patients' prognostic stratification.

Mirizzi Syndrome Type IV Successfully Treated with Peroral Single-operator Cholangioscopy-guided Electrohydraulic Lithotripsy: A Case Report with Literature Review.

A 76-year-old man presented with liver dysfunction and intrahepatic bile duct dilatation. Imaging studies showed two large stones that had become impacted in the common hepatic duct, which was fused with the gallbladder. The patient was diagnosed with Mirizzi syndrome type IV. Hepaticojejunostomy and stone removal failed due to dense gallbladder adhesions involving the right hepatic artery. The bile flow was temporarily restored; however, the patient experienced cholangitis 16 months later. The stones were extracted via peroral single-operator cholangioscopy (SOC)-guided electrohydraulic lithotripsy. This is the first case in which stones were completely removed by SOC-guided treatment in a patient with Mirizzi syndrome type IV.

Neuron-specific enolase level is a useful biomarker for distinguishing amyotrophic lateral sclerosis from cervical spondylotic myelopathy.

The current study aimed to evaluate whether cerebrospinal fluid (CSF) neuron-specific enolase (NSE) levels are elevated in amyotrophic lateral sclerosis (ALS) and are effective in distinguishing ALS from cervical spondylotic myelopathy (CSM). We retrospectively evaluated 45 patients with ALS, 23 with CSM, 28 controls, and 10 with Parkinson's disease (PD) who underwent analysis of CSF NSE levels. The control group comprised patients aged above 45 years who underwent lumbar puncture because of suspected neurological disorders that were ruled out after extensive investigations. CSF NSE levels were evaluated using the electro-chemiluminescent immunoassay. The ALS group had significantly higher CSF NSE levels than the CSM and control groups (P < 0.001 for both comparisons). The CSM, control, and PD groups did not significantly differ in terms of CSF NSE levels. A receiver-operating characteristic curve analysis was performed to assess the diagnostic value of CSF NSE levels in distinguishing ALS from CSM. The area under the curve for CSF NSE levels was 0.86. The optimal cutoff value was 17.7 ng/mL, with a specificity of 87% and a sensitivity of 80%. Hence, CSF NSE levels are elevated in ALS and are effective in distinguishing ALS from CSM.

Clinical features of Guillain-Barré syndrome patients with elevated serum creatine kinase levels.

BACKGROUND: It is not well defined whether Guillain-Barré syndrome (GBS) patients with elevated serum creatine kinase (CK) levels have characteristic clinical features and are related to the subgroups of GBS. METHODS: We retrospectively studied 51 consecutive patients with GBS, who visited our hospital, and compared clinical, laboratory and electrophysiological findings between patients with and without elevated CK levels. RESULTS: Of 51 patients, 14 patients (27%) showed an elevation of serum CK levels. When compared with patients with the normal CK levels, the ratios of male, antecedent infections, and anti-GM1 antibody positivity were significantly higher in patients with elevated CK levels. The ratios of hypoesthesia, cranial nerve involvement, and urinary retention were significantly less in patients with elevated CK levels. There were no significant differences in disability at peak between two groups. In the electrophysiological examination, sensory nerve abnormalities were not observed. Although some patients with elevated CK levels showed prolongation of distal motor latencies (DMLs) and increase of durations in the initial examination, development of the prolongation of DMLs and increase of durations was not observed in the follow-up examinations. The findings were consistent with acute motor axonal neuropathy (AMAN) with reversible conduction failure (RCF) but not acute inflammatory demyelinating polyneuropathy (AIDP). CONCLUSIONS: The results suggest that the GBS patients with elevated CK levels represent not a group of AIDP but a group of AMAN with axonal degeneration or RCF even though the initial electrophysiological examination shows AIDP pattern.

Subacute Combined Degeneration of the Spinal Cord Caused by Autoimmune Gastritis.

A 68-year-old woman presented with a 2-year history of worsening unsteady gait. Her neurological examination revealed peripheral neuropathy with lower limb sensory dominance. T2-weighted imaging revealed a disorder of the posterior cervical cord. Blood test findings revealed vitamin B12 deficiency, and gastroscopy revealed typical findings of autoimmune gastritis. She received vitamin B12 supplementation, but some peripheral neuropathy symptoms persisted due to longstanding vitamin B12 deficiency. Asymptomatic patients should undergo gastroscopy to detect autoimmune gastritis, as chronic vitamin B12 deficiency causes irreversible peripheral neuropathy.

Posterior reversible encephalopathy syndrome with extensive cytotoxic edema after blood transfusion: a case report and literature review.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is described as a clinical-radiological disease entity with good prognosis. In brain MRI, PRES generally presents with vasogenic edema. Although PRES is induced by various causes, a small number of PRES cases have occurred after red cell blood transfusion. It is unclear whether there are characteristic features in PRES after blood transfusion. CASE PRESENTATION: Here, we report a case of 75-year-old Japanese woman who had acute exacerbation of subacute anemia by bleeding from gastric ulcer. After receiving a red cell blood transfusion, she showed disturbance of consciousness with extensive cytotoxic and small vasogenic edema in the occipitoparietal area on brain MRI. She was diagnosed as PRES and suffered irreversible impairments of visual acuity and fields in both eyes. We summarized and discussed clinical features of cases with PRES after blood transfusion. CONCLUSIONS: A total of 21 cases including the present one have been reported as PRES after blood transfusion. Of the cases, 20 of 21 were female, and 15 of 17 developed PRES in the course of chronic anemia lasting over 1 month. Anemia was severe in 15 of 20 cases, with hemoglobin levels < 3.5 g/dl. In 14 of 17 cases, hemoglobin levels increased to 5 g/dl by red cell blood transfusion until the onset of PRES. On brain MRI, 2 of 21 cases showed cytotoxic edema and 3 of 21 cases showed irreversible neurological disturbance. In this patient, the occurrence of PRES in subacute anemia and the presence of extensive cytotoxic brain edema with irreversible neurological deficits were characteristic points. When treating severe anemia, even with a subacute progression, we should consider a possibility that PRES occurs after blood transfusion with extensive cytotoxic brain edema and irreversible neurological changes.

Clinical characteristics of long-term survival with noninvasive ventilation and factors affecting the transition to invasive ventilation in amyotrophic lateral sclerosis.

INTRODUCTION: We evaluated post-noninvasive ventilation survival and factors for the transition to tracheostomy in amyotrophic lateral sclerosis (ALS). METHODS: We analyzed 197 patients using a prospectively collected database with 114 patients since 2000. RESULTS: Among 114 patients, 59 patients underwent noninvasive ventilation (NIV), which prolonged the total median survival time to 43 months compared with 32 months without treatment. The best post-NIV survival was associated with a lack of bulbar symptoms, higher measured pulmonary function, and a slower rate of progression at diagnosis. The transition rate from NIV to tracheostomy gradually decreased over the years. Patients using NIV for more than 6 months were more likely to refuse tracheostomy and to be women. DISCUSSION: This study confirmed a positive survival effect with NIV, which was less effective in patients with bulbar dysfunction. Additional studies are required to determine the best timing for using NIV with ALS in patients with bulbar dysfunction. Muscle Nerve 58:770-776 2018.

Lower cranial polyneuropathy in zoster sine herpete presenting with pain in the ear and throat: a case report.

A 64-year-old woman developed acute paralysis of glossopharyngeal, vagus, accessory, and hypoglossal nerves on the left side after pain in the head and the left ear and throat. Cerebrospinal fluid examination revealed lymphocytic pleocytosis and elevated protein concentration. Varicella-zoster virus (VZV)-DNA was detected by PCR from cerebrospinal fluid. The diagnosis of lower cranial polyneuropathy due to VZV reactivation was made. After oral administration of an anti-viral agent and steroid, all symptoms and signs dramatically improved. Notably, there was no evidence of cutaneous or mucosal rash during the whole course of the disease. VZV reactivation should be included in the differential diagnosis of acute lower cranial polyneuropathy, especially with pain in the ear and throat, even without cutaneous or mucosal rash.

Laparoscopic right hemicolectomy for a patient with idiopathic retroperitoneal fibrosis: A case report.

A 62-year-old man with abdominal pain and lumbago was admitted to our hospital. Blood examination revealed renal insufficiency, and CT revealed retroperitoneal fibrosis causing bilateral hydrocele and ureteral compression. A colonoscopy was performed to rule out secondary retroperitoneal fibrosis due to malignancies, and this imaging revealed an ascending colon cancer. Laparoscopic right hemicolectomy with lymphadenectomy and retroperitoneal biopsy were performed. The retroperitoneum was filled with hard, white fibrous tissue, which made it difficult to mobilize the right mesocolon from the retroperitoneum. Devascularization performed before mobilization allowed for a safe and oncologically feasible procedure. Histologically, there were no malignant cells in the retroperitoneal tissue. The patient has been without colon cancer reoccurrence for 4 years. When the surgical challenges that distinguish these patients from ordinary cases are recognized preoperatively, laparoscopic colectomy may be a feasible option for patients with colorectal cancer with idiopathic retroperitoneal fibrosis.

Diagnosis of ischemic small bowel disease by measurement of serum intestinal fatty acid-binding protein in patients with acute abdomen: a multicenter, observer-blinded validation study.

BACKGROUND: Intestinal fatty acid-binding protein (I-FABP) is a low-molecular-mass (15 kDa) cytosolic protein found exclusively in the epithelial cells of the small bowel mucosa. We aimed to evaluate the clinical usefulness of serum I-FABP measurement for the diagnosis of ischemic small bowel disease. METHODS: Patients with a clinical diagnosis of acute abdomen were recruited for this multicenter trial at one university hospital and nine city hospitals over a 13-month period. Serum I-FABP levels were measured in 361 eligible patients by an enzyme-linked immunosorbent assay using a specific monoclonal antibody. RESULTS: Of the 361 patients, 242 underwent surgery, and small bowel ischemia was diagnosed in 52 patients. The mean serum I-FABP level in the patients with small bowel ischemia was 40.7 ± 117.9 ng/ml, which was significantly higher than that in patients with non-ischemic small bowel disease (5.8 ± 15.6 ng/ml) and those with non-small bowel disease (1.8 ± 1.7 ng/ml). The serum I-FABP cutoff level for the diagnosis of small bowel ischemia was 3.1 ng/ml. Serum I-FABP was more efficient than conventional biochemical markers, in terms of sensitivity and positive and negative predictive values, in the diagnosis of small bowel ischemia. However, its specificity was slightly lower than that of creatinine phosphokinase or lactate dehydrogenase. The positive and negative likelihood ratios of serum I-FABP were 3.01 and 0.29, respectively. CONCLUSION: Serum I-FABP measurement is a non-invasive method that is potentially useful for the efficient identification of patients with acute abdomen who are at risk of small bowel ischemia.

[A case of advanced gastric cancer with multiple liver metastases successfully treated with TS-1 and CDDP].

A Case of Advanced Gastric Cancer with Multiple Liver Metastases Successfully Treated with TS-1 and CDDP: Akihiro Tsukahara, Kazuhiro Kaneko and Syuji Tanaka (Dept. of Surgery, Niigata Prefectural Koide Hospital) Summary A 70-year-old advanced gastric cancer patient with liver and lymph node metastases was treated by chemotherapy with TS-1 and CDDP. One course consisted of administration of TS-1 100 mg/body for 21 days followed by 14 days rest and infusion of CDDP 80 mg/body on day 8. At the end of 2 courses, the primary tumor showed a hypertrophic wall, but a partial response of the liver metastases (reduction ratio was 78.3%) and a complete response of the LN metastasis were achieved. PR and CR were maintained after 4 courses. There were no remarkable side effects for 4 courses. This chemotherapy may have therapeutic efficacy in cases of advanced gastric cancer with liver and lymph node metastases.

Thrombopoietin levels and peripheral platelet counts following living related donor liver transplantation.

BACKGROUND/AIMS: Thrombopoietin is the primary hematopoietic growth factor. Thrombopoietin deficiency may cause thrombocytopenia in advanced liver disease. The aim of our study was to investigate the relevance of thrombopoietin levels to peripheral platelet counts in patients with liver disease who underwent LRDLT (living related donor liver transplantation). METHODOLOGY: We divided the six patients who underwent LRDLT into two groups. Group 1 had thrombocytopenia and group 2 had normal platelet counts. We measured serum thrombopoietin and peripheral platelet counts before and after LRDLT. RESULTS: Pre-LRDLT thrombopoietin and peripheral platelet counts were lower in group 1 than in group 2. Thrombopoietin in group 1 significantly increased on the first day after LRDLT and peripheral platelet counts in group 1 increased following the rise in thrombopoietin (p < 0.05). Moreover, a marked increase in thrombopoietin and peripheral platelet counts was found in splenectomized patients during LRDLT. CONCLUSIONS: These findings suggested inadequate thrombopoietin production in advanced stage liver disease which caused thrombocytopenia. Improvement of thrombopoietin production in graft liver function may contribute to increase of peripheral platelet counts.

[A case of advanced gastric cancer treated with paclitaxel and TS-1].

We treated a case of advanced gastric cancer with paclitaxel and TS-1. A 64-year-old man underwent total gastrectomy, splenectomy, and D2 + No. 16 a 2, b 1 lymph node (LN) dissection for gastric cancer. Computed tomography (CT) revealed metastases of supraclavicular and para-aortic LNs in the 4th postoperative month. Paclitaxel 90 mg was infused once a week, and TS-1 100 mg was administered daily. One course consisted of infusion of paclitaxel for 3 weeks followed by 2 weeks rest and administration of TS-1 for 4 weeks followed by 2 weeks rest. At the end of 4 courses of paclitaxel and 3 courses of TS-1, a partial response of the supraclavicular LN metastasis and a complete response of the para-aortic LN metastasis were achieved. There were no remarkable side effects for 2 years after the operation. This chemotherapy might be suitable to treat patients with LN metastases of advanced gastric cancer.

Splenectomy for reduction of excessive portal hypertension after adult living-related donor liver transplantation.

BACKGROUND/AIMS: We investigated the effects of splenectomy on the reduction of excessive portal hypertension immediately after adult living-related donor liver transplantation, paying particular attention to peritransplanted portal pressure in seven adult patients. METHODOLOGY: We studied the relationship between portal hypertension and hyperbilirubinemia in small-for-size graft liver transplantation. RESULTS: In the three cases, the portal pressures increased beyond 30 cmH2O after living-related donor liver transplantation, despite the right lobe graft, and these patients underwent splenectomy. After splenectomy, their portal pressures decreased below 25 cmH2O. The portal pressure underwent auxiliary orthotopic partial liver transplantation due to the hypercitrullinemia and did not change after surgery (9.5 to 11.5 cmH2O). Interestingly, the hyperbilirubinemia occurring after living-related donor liver transplantation were as the primary result of direct bilirubin except for the patient with citrullinemia. The posttransplanted portal pressures were controlled below 25 cmH2O in all patients, with their peak serum total bilirubin levels not exceeding 15 mg/dL, and the patients were discharged without major complications. Three patients underwent splenectomy, and did not suffer from serious infection. The reduction in excessive portal hypertension after living-related donor liver transplantation might prevent liver injury and post-transplant hyperbilirubinemia. CONCLUSIONS: However, splenectomy remains a life-threatening factor. Therefore, transplant surgeons encountering living-related donor liver transplantation must continue to seek out additional solutions to problems with excessive portal hypertension.

[Dormant chemotherapy by low-dose FP and low-dose UFT-E in recurrent gastric cancer with long-term survival--a case report].

We experienced a case of recurrent gastric cancer with a long-term survival. A 64-year-old man was admitted to the hospital for advanced gastric cancer in the upper stomach. Abdominal CT scan revealed para-aortic lymph nodal metastases. The patient underwent total gastrectomy, distal pancreatectomy, splenectomy, left adrenectomy, and left nephrectomy with D4 lymph node dissection, in what was a curability B resection. Conclusive findings were t2 (ss), n4, H0, P0, M0, and stage IVb. One year after the operation, para-aortic lymph node recurrence was evaluated. The patient was treated with low-dose cisplatin-5-FU therapy, and a partial response was observed and continued for over 2 years with an administration of UFT-E (300 mg/day). He died of repeated aggravation of para-aortic lymph node metastases 6 years and 2 months after the operation. We considered that the long-term survival of this patient was attributable to a 3-year tumor dormancy induced by low-dose cisplatin-5-FU therapy and administration of low-dose UFT.