BACKGROUND: Three types of progressive familial intrahepatic cholestasis (PFIC) have been identified, but their etiologies include unknown mechanisms.
DATA SOURCES: A PubMed search on "progressive familial intrahepatic cholestasis" and "PFIC" was performed on the topic, and the relevant articles were reviewed.
RESULTS: The etiologies of the three PFIC types still include unknown mechanisms. Especially in PFIC type 1, enterohepatic circulation of bile acid should be considered. Ursodeoxycholic acid, partial external biliary diversion and liver transplantation have been used for the treatment of PFIC patients according to disease course.
CONCLUSIONS: Since the etiologies and disease mechanisms of PFIC are still unclear, detailed studies are urgently required. Strategies for more advanced therapies are also needed. These developments in the future are indispensable, especially for PFIC type 1 patients.

BACKGROUND: Pancreatic adenocarcinoma remains the fourth leading cause of cancer-related death and is one of the most aggressive human tumors. At present, surgical resection is the only potentially curative treatment. Early neck division is inadequate when invasion of the superior mesenteric artery (SMA) is suspected or in cases of replaced or accessory right hepatic artery. Malignant periampullary tumors often invade retroperitoneal peripancreatic tissues and a positive resection margin is associated with a poor long-term survival.
DATA SOURCES: English-language medical databases, PubMed, ELSEVIER and SPRINGERLINK, were searched for articles on "posterior approach pancreaticoduodenectomy", "superior mesenteric artery first approach", "retroperitoneal tissue", "hanging maneuver", and related topics.
RESULTS: The modification allowed the surgeon to early identify the nonresectability of a replaced right hepatic artery if present, enabling complete dissection of the right side of the SMA and portal vein as well as complete excision of the retroportal pancreatic lamina.
CONCLUSION: Pancreaticoduodenectomy with early retro-pancreatic dissection is a useful and safe technical variant, which is indicated for the improvement of the safety and curative effect of the procedure.

BACKGROUND: Hepatitis B virus (HBV) is a hepatotropic, noncytopathic, DNA virus which can cause acute and chronic infection. Viral persistence is associated with a weak or absent specific immune responses to HBV, particularly the cellular immune response. Dendritic cells (DCs) are professional antigen-presenting cells with a unique T cell stimulatory aptitude that play a crucial role in the instruction of adaptive immune responses upon infection. An impaired function of DCs was suggested by recent studies to account for the T and B cell hyporesponsiveness in chronic HBV infection. This review summarizes recent insights into the recognition of HBV antigens by DCs.
DATA SOURCES: Studies were identified by searching MEDLINE and/or PubMed for articles using the key words "hepatitis B virus (HBV)", "dendritic cells", "C-type lectins", "mannose receptor", "toll-like receptor", and "dendritic cell-specific intercellular-adhesion-molecule-3 grabbing nonintegrin (DC-SIGN)" up to December 2009. Additional papers were identified by a manual search of the references from the key articles.
RESULTS: DCs play an important role in the progress of hepatitis B, especially in the recognition of HBV. There are three main ways of recognition of HBV antigens by DCs. First, HBV DNA can be recognized by DCs through toll-like receptor 9 (TLR9) which activates the NF-κB signal pathway and p38 MAPK to up-regulate the expression of interferon (IFN) regulatory factor 7 (IRF-7) in a manner independent of type I IFN signaling, resulting in secretion of type I IFN and inflammatory cytokines, and induction of DC maturation and the adaptive immune response. Second, HBc/HBeAg cannot be recognized by DCs, but DNA or ssRNA encapsulated within HBcAg can be internalized by DCs through TLRs. Third, HBsAg can be internalized by DCs through the mannose receptor, which lacks the ability to induce DC maturation without the assistance of DC-SIGN. Meanwhile, there is some cross-talk among the three mechanisms, which induces an effective anti-viral response or HBV persistence.
CONCLUSIONS: On the basis of these recognition processes, methods have been used to enhance the efficacy of DC-based vaccine against HBV and have been useful in the clinical application of HBV vaccine therapy. But the interactions between HBV antigens/HBV DNA and DCs are not clear, and cross-talk between TLRs and various ligands makes HBV antigen recognition by DCs more complicated. More efforts should be made to define the mechanisms and develop effective vaccines and therapies.

BACKGROUND: The ischemic-type biliary lesion (ITBL) is one of the most serious biliary complications of liver transplantation. This study aimed to investigate the effects of autologous bone marrow mononuclear cell (BM-MNC) implantation on neovascularization and the prevention of intrahepatic ITBL in a rabbit model.
METHODS: The rabbits were divided into control, experimental model, and cell implantation groups, with 10 in each group. The model of intrahepatic ITBL was established by clamping the hepatic artery and common bile duct. Autologous BM-MNCs were isolated from the tibial plateau by density gradient centrifugation and were implanted through the common hepatic artery. Changes in such biochemical markers as aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, gamma-glutamyltranspeptidase, total bilirubin and direct bilirubin were measured. Four weeks after operation, cholangiography, histopathological manifestations, differenti-ation of BM-MNCs, microvessel density and the expression of vascular endothelial growth factor were assessed.
RESULTS: Compared with the experimental model group, the BM-MNC implantation group showed superiority in the time to recover normal biochemistry. The microvessel density and vascular endothelial growth factor expression of the implantation group were significantly higher than those of the control and experimental model groups. The ITBL in the experimental model group was more severe than that in the implantation group and fewer new capillary blood vessels occurred around it.
CONCLUSIONS: Implanted autologous BM-MNCs can differentiate into vascular endothelial cells, promote neovascularization and improve the blood supply to the ischemic bile duct, and this provides a new way to diminish or prevent intrahepatic ITBL after liver transplantation.

BACKGROUND: The use of staged liver resections for colorectal metastases has been increasing in recent times. The aim of this study was to determine the practices and outcomes of those surgeons attending the Australia and New Zealand Hepatic, Pancreatic and Biliary Association (ANZHPBA) meeting in 2008 who perform staged resections.
METHODS: A questionnaire was sent to all members of the ANZHPBA and the international faculty who were invited to attend the annual meeting held in Coolum, Queensland, Australia in October 2008.
RESULTS: There were 30 responses from 7 centres across the UK, Germany and Australia. Twenty-eight patients completed treatment. The study population was predominantly male (n=20, 67%), with an average age of 59.4 years. All patients had bilobar disease. A right-sided first resection was planned in 39% of cases. Seventeen percent of patients underwent portal vein embolization prior to first resection. A second operation was performed at an average of 2.8 months from the first resection. Overall, 50% (n=14) of patients eventually achieved a complete (R0) staged procedure. Twelve complications after the first resection were seen in 32% patients (n=9). Twenty-three patients underwent a second liver resection. Twenty-five complications after the second resection were present in 57% (n=13).
CONCLUSIONS: Two-stage liver resections are beneficial if both stages are completed and an R0 resection is achieved. While there is increased morbidity and mortality, we believe that staged liver resection for colorectal metastases is a valuable strategy in selected cases.

BACKGROUND: Contrast-enhanced ultrasonography (CEUS) is an important technique for depiction and assessment of tumor vascularity. This study aimed to explore the relationship between the morphological characteristics of tumor microvessels and enhancement patterns on CEUS in hepatocellular carcinoma (HCC).
METHODS: Eighty patients with HCC underwent CEUS using SonoVue before hepatectomy. Contrast-enhanced ultrasonographic enhancement patterns and quantitative parameters were recorded. The tumor tissue sections were immunostained with human CD34 monoclonal antibody. The patients were classified into a point-line type group (n=36) and a loop-strip type group (n=44) according to microvessel morphology. The microvascular density (MVD) in the different types of microvessels was calculated. The relationship between enhancement patterns of HCC lesions and morphological characteristics of tumor microvessels was analyzed.
RESULTS: The mean MVD in HCC was 22.4±3.5 per 0.2 mm2 in the point-line group, and 19.6±6.7 per 0.2 mm2 in the loop-strip group, and there was no significant difference between them (t=0.948, P=0.354). In the portal vein phase, hypoenhancement was significantly more frequent in HCC (χ2=4.789, P=0.029) in the loop-strip group (40/44, 90.9%) than in the point-line group (26/36, 72.2%). The time to hypo-enhancement in the loop-strip group (mean 64.84±26.16 seconds) was shorter than that in the point-line group (mean 78.39±28.72 seconds) (t=2.247, P=0.022). The time to hypo-enhancement was correlated with MVD in the loop-strip group (r=-0.648, P=0.001).
CONCLUSIONS: The enhancement patterns on CEUS are related to tumor microvascular morphology, and the type of microvascular morphology influences CEUS characterization. CEUS, an important noninvasive imaging technique, is used to evaluate microvascular morphology and angiogenesis, providing valuable information for antiangiogenic therapy in HCC.

BACKGROUND: The bioartificial liver is anticipated to be a promising alternative choice for patients with liver failure. Toxic substances which accumulate in the patients) plasma exert deleterious effects on hepatocytes in the bioreactor, and potentially reduce the efficacy of bioartificial liver devices. This study was designed to investigate the effects of plasma from patients with acute on chronic liver failure (AoCLF) on immortalized human hepatocytes in terms of cytochrome P450 gene expression, drug metabolism activity and detoxification capability.
METHODS: Immortalized human hepatocytes (HepLi-2 cells) were cultured in medium containing fetal calf serum or human plasma from three patients with AoCLF. The cytochrome P450 (CYP3A5, CYP2E1, CYP3A4) expression, drug metabolism activity and detoxification capability of HepLi-2 cells were assessed by RT-PCR, lidocaine clearance and ammonia elimination assay.
RESULTS: After incubation in medium containing AoCLF plasma for 24 hours, the cytochrome P450 mRNA expression of HepLi-2 cells was not significantly decreased compared with control culture. Ammonia elimination and lidocaine clearance assay showed that the ability of ammonia removal and drug metabolism remained stable.
CONCLUSIONS: Immortalized human hepatocytes can be exposed to AoCLF plasma for at least 24 hours with no significant reduction in the function of cytochrome P450. HepLi-2 cells appear to be effective in metabolism and detoxification and can be potentially used in the development of bioartificial liver.

BACKGROUND: Melanoma differentiation-associated gene-7 (MDA-7)/interleukin-24 (IL-24) is a novel tumor suppressor gene, which has suppressor activity in a broad spectrum of human cancer cells. We investigated the effect of the replication-competent oncolytic adenovirus SG600-IL24 and replication-incompetent adenovirus Ad.IL-24, both expressing human MDA-7/IL-24 on the hepatocellular carcinoma cell lines HepG2, Hep3B, SMMC-7721, HCCLM3, and the normal liver cell line L02.
METHODS: Hepatocellular carcinoma cell lines and the normal liver cell line were infected with SG600-IL24 and Ad.IL-24. The mRNA and protein expression of MDA-7/IL-24 in infected cells was confirmed by RT-PCR, ELISA, and Western blotting. MTT assay was used to investigate the proliferation effect. Hoechst staining and Annexin-V and PI staining were performed to study the MDA-7/IL-24 gene expressed in HCC cell lines and the normal liver cell line. Flow cytometry was used to analyse the cell cycle.
RESULTS: RT-PCR, ELISA and Western blotting confirmed that the exogenous MDA-7/IL-24 gene was highly expressed in cells infected with SG600-IL24. MTT and apoptosis detection indicated that SG600-IL24 induced growth suppression, promoted apoptosis, and blocked cancer cell lines in the G2/M phase in hepatocellular carcinoma cell lines but not in the normal liver cell line.
CONCLUSIONS: SG600-IL24 selectively induces growth suppression and apoptosis in hepatocellular carcinoma cell lines in vitro but not in the normal liver cell line L02. Compared with Ad.IL-24, SG600-IL24 dramatically enhances antitumor activity in hepatocellular carcinoma cell lines.

BACKGROUND: Primary biliary cirrhosis (PBC) is an uncommon autoimmune cholestatic disease that predominantly affects women. Certain human leukocyte antigens (HLAs) have been reported to be associated with susceptibility for PBC. We describe the profiles of PBC in Brunei Darussalam.
METHODS: All patients with PBC (n=10) were identified from our prospective databases. The HLA profiles (n=9, PBC) were compared to controls (n=65) and patients with autoimmune hepatitis (n=13, AIH).
RESULTS: All patients were women with a median age of 51 years (27-83) at diagnosis. The prevalence rate of the disease was 25.6/million-population and the estimated incidence rate varied from 0 to 10.3/million-population per year. Chinese (41.15/million) and the indigenous (42.74/million) groups had higher prevalence rates compared to Malays (22.62/million). The prevalence among female population was 54.6/million-population. All patients were referred for abnormal liver profiles. Five patients had symptoms at presentations: jaundice (20%), fatigue (20%), arthralgia (30%) and pruritus (20%). Serum anti-mitochondrial antibody was positive in 80% of the patients. Overlap with AIH was seen in 30%. Liver biopsies (n=8) showed stage I (n=2), II (n=4) and III (n=2) fibrosis. There were no significant differences in the HLA profiles between PBC and AIH. Compared to the controls, PBC patients had significantly more HLA class I alleles specifically B7 (P=0.003), Cw7 (P=0.002) and Cw12 (P=0.007) but not the class II alleles. At a median follow-up of 23.5 months (2 to 108), all patients were alive without evidence of disease progression.
CONCLUSIONS: PBC is also a predominant female disorder in our local setting and most had mild disease. The HLA profiles of our patients were different to what have been reported.

BACKGROUND: Transumbilical single-incision laparoscopic cholecystectomy (SILC) is a new procedure. It has been described by some authors as scarless surgery. To our knowledge, however, there has been no study on outpatient SILC. The present study was designed to determine the safety, feasibility and benefits of transumbilical outpatient SILC.
METHODS: Twenty-two patients underwent transumbilical outpatient SILC at our department from December 2008 to October 2009. In all patients, the preoperative work-up and operation were completed in the outpatient clinic. To perform the operation, a 2- to 2.5-cm semi-circular incision was made around the umbilicus and three 5-mm trocars were inserted separately by direct puncture. A 5-mm flexible laparoscope, an UltraCision harmonic scalpel and curved instruments were used to perform the laparoscopic cholecystectomy (LC) procedure.
RESULTS: All patients except one were operated on successfully. The conversion rate to standard LC was 5%. In the 21 successfully completed patients, the median duration of operation was 56.5 minutes and estimated operative blood loss was 16.2 ml. The time to resume liquid food was 10.8 hours and semi-liquid food was 16.2 hours after the operation. Nine patients went home on the same day, and 12 on the second day after the operation. The mean postoperative hospital observation time was 18.5 hours. Urinary retention was observed in 1 patient. The follow-up was conducted for all patients at 2 weeks after surgery. All patients were satisfied with the good cosmetic effect of the surgery. The total satisfaction rate was 95%.
CONCLUSIONS: Outpatient SILC is a safe and feasible technique for operating with fewer scars and reducing perioperative discomfort at the same time. A direct puncture method to insert trocars is technically feasible. Using a flexible laparoscope and curved instruments make the procedure easier and more time-saving.

BACKGROUND: Pancreatitis is associated with arterial complications in 4%-10% of patients, with untreated mortality approaching 90%. Timely intervention at a specialist center can reduce the mortality to 15%. We present a single institution experience of selective embolization as first line management of bleeding pseudoaneurysms in pancreatitis.
METHODS: Sixteen patients with pancreatitis and visceral artery pseudoaneurysms were identified from searches of the records of interventional angiography from January 2000 to June 2007. True visceral artery aneurysms and pseudoaneurysms arising as a result of post-operative pancreatic or biliary leak were excluded from the study.
RESULTS: In 50% of the patients, bleeding complicated the initial presentation of pancreatitis. Alcohol was the offending agent in 10 patients, gallstones in 3, trauma, drug-induced and idiopathic pancreatitis in one each. All 16 patients had a contrast CT scan and 15 underwent coeliac axis angiography. The pseudoaneurysms ranging from 0.9 to 9.0 cm affected the splenic artery in 7 patients: hepatic in 3, gastroduodenal and right gastric in 2 each, and left gastric and pancreatico-duodenal in 1 each. One patient developed spontaneous thrombosis of the pseudoaneurysm. Fourteen patients had effective coil embolization of the pseudoaneurysm. One patient needed surgical exclusion of the pseudoaneurysm following difficulty in accessing the coeliac axis radiologically. There were no episodes of re-bleeding and no in-hospital mortality.
CONCLUSIONS: Pseudoaneurysms are unrelated to the severity of pancreatitis and major hemorrhage can occur irrespective of their size. Co-existent portal hypertension and sepsis increase the risk of surgery. Angiography and selective coil embolization is a safe and effective way to arrest the hemorrhage.

BACKGROUND: Recent studies have confirmed that the expression of Ezrin, hepatocyte growth factor (HGF) and its receptor (C-met) is related to the genesis, progress, invasion and metastasis of some malignant tumors. Researches have also found that the biological function of Ezrin is closely related to HGF/C-met in malignant tumors. However, there is no report on the expression levels of Ezrin, HGF and C-met in rat pancreatic cancer induced by dimethylbenzanthracene (DMBA). This study aimed to detect the expression of Ezrin, HGF and C-met in rat pancreatic cancer and non-cancerous pancreatic tissues, and assess its effect in cancer induction by DMBA.
METHODS: Ninety Sprague-Dawley rats were divided into 3 groups randomly: 40 in a pancreatic cancer model group (group A), 40 in a trichostatin A (TSA) intervention group (group B), and 10 in a control group (group C). DMBA was directly implanted into the parenchyma of rat pancreas in group A+group B. The rats of group B were treated with 1 ml of TSA saline solution (1 µg/ml) via intraperitoneal injection weekly. The carcinogenesis of rats executed within 3-5 months in groups A and B was observed by macrograph and microscopy. Meanwhile, the rats in group C were executed within 5 months. The EnVisionTM immunohistochemistry for detecting the expression levels of Ezrin, HGF and C-met was used in paraffin-embedded sections of the pancreatic specimens.
RESULTS: The incidence of pancreatic cancer in group A was 48.6% and in group B 33.3%. The maximal diameter of tumor mass was significantly larger in group A than that in group B (P<0.05). No pathological changes were observed in the pancreas of group C and other main organs of groups A and B. The positive rates of Ezrin, HGF and C-met were significantly higher in ductal adenocarcinoma than in non-cancerous pancreatic tissues of groups A and B (P<0.01). The positive rates of Ezrin, HGF and C-met were significantly higher in ductal adenocarcinoma of group A than those in non-cancerous pancreatic tissues of group A (P<0.05), but there was no significant difference in group B (P>0.05). The positive rates of Ezrin, HGF and C-met in non-cancerous pancreatic tissues proved mild to severe atypical hyperplasia of the ductal epithelia. The pancreas of group C and 2 cases of fibrosarcoma showed the negative expression of Ezrin, HGF and C-met. There was a trend of consistency in the expression of Ezrin, HGF and C-met in ductal adenocarcinoma (P<0.05 or P<0.01).
CONCLUSIONS: DMBA directly implanted into the parenchyma of the pancreas can produce a model of pancreatic cancer with a high incidence in a short time. TSA might inhibit the carcinogenesis and growth of pancreatic cancer, and its effects may be related to the inhibition of the expression of Ezrin, HGF and C-met during the process. Ezrin, HGF and C-met may have positive effects on the carcinogenesis of rat pancreas.

BACKGROUND: Toll-like receptor 4 (TLR4) plays an important role in the occurrence and development of acute pancreatitis (AP). Apoptosis of pancreatic cells is closely related to the severity of AP. TLR4 is known to induce apoptosis in some cell types and therefore it is of importance to investigate potential associations between TLR4 activity and apoptosis in the setting of AP.
METHODS: A total of 50 wild-type (C57BL/10J) and TLR4-deficient (C57BL/10ScNJ) mice were divided into three groups: 2-hour, 4-hour, and control groups. Each group was divided into two equal subgroups: TLR4-wild-type mice and TLR4-deficient mice. AP was experimentally induced by 7 intraperitoneal injections of 50 µg/kg cerulein at hourly intervals. Control mice received 7 injections of equal volumes of saline. The severity of pancreatic injury during AP was assessed by serum amylase concentration and histopathology. The level of apoptosis of pancreatic cells in response to AP was evaluated by calculating the apoptotic index (AI) and comparing the expression levels of cytochrome C and Fas-associated protein with death domain (FADD) between TLR4-wild-type and TLR4-deficient mice at 2 time points.
RESULTS: The AI was found to be significantly lower in the pancreas of TLR4-deficient mice with AP compared to TLR4-wild-type mice at two hours after the last treatment injection. Enzyme-linked immunosorbent assay and real-time reverse transcription-polymerase chain reaction also revealed significantly lower expression of cytochrome C and FADD in the pancreas of TLR4-deficient mice than in TLR4-wild-type animals at the same time point. Serum amylase concentration and morphological severity of AP in pancreatic tissue were found to be similar in the two strains of mice at both time points.
CONCLUSION: We postulate that TLR4 can mediate apoptosis of pancreatic cells during the early stages of AP, via the activation of both intrinsic and extrinsic apoptotic signaling pathways.

BACKGROUND: Primary myelofibrosis (PMF) is a myeloproliferative disorder characterized by bone marrow fibrosis. Extra-medullary hematopoiesis sometimes occurs even in the peritoneal cavity, apart from organs such as the liver, spleen, and lymph nodes. This may sometimes be complicated by spontaneous infection and complications. We report a rather unusual case of PMF, who presented as an emergency with spontaneous peritonitis to general surgery department and had a fulminant clinical course.
METHOD: A clinical case note review was done and a literature search was undertaken.
RESULTS: A rather unusual case of PMF, who presented as an emergency with spontaneous peritonitis to general surgery department. The patient underwent a laparotomy and had a fulminant clinical course.
CONCLUSIONS: Peritonitis in myelofibrosis may have a number of causes. Clinicians need to be aware of them and provide conservative management prior to surgical treatment.

BACKGROUND: Obstructive jaundice is a common condition in advanced digestive cancer. Palliative procedures can improve quality of life and allow patients to attempt a systemic treatment. Bilioenteric anastomosis is still the procedure of choice for patients in many centers. When a surgical bypass is not possible, biliary drainage can be done by placing endoscopic or transparietal stents, which are less durable methods even when an expandable stent is employed.
METHODS: A 47-year-old male with an excellent clinical status and a previous cholecystectomy and an exploratory laparotomy for advanced gastric cancer was referred with obstructive jaundice. A preoperative CT scan showed a dilated bile duct and a small mass at the distal hepatic hilum. No other signs of metastasis were found. A surgical bilioenteric anastomosis was indicated. At surgery, a distal choledochal obstruction and a mesenteric retraction by a lymph node mass prevented the jejunum to ascend for a bilioenteric anastomosis. Surgically, an alternative bilioenteric bypass was performed by means of an ileal loop interposition between the bile duct and the jejunum.
RESULT: The recovery of the patient was uneventful and his bilirubin levels normalized after one week. The patient was then referred for systemic chemotherapy.
CONCLUSIONS: This alternative biliary bypass can be safely and easily performed, and may be a good alternative for patients already referred for surgery because of a better life expectancy and when the jejunum is not an alternative.

BACKGROUND: The 2009 H1N1 influenza A virus was first identified in April 2009 and rapidly evolved into a pandemic. Recipients of solid-organ transplants have a higher risk for severe infection because of immunosuppression. There are limited reports of 2009 H1N1 influenza in liver transplant recipients, especially in China.
METHODS: We present a case of a 48-year-old male liver transplant recipient with 2009 H1N1 influenza A virus. He received therapy for acute rejection after transplantation and was confirmed with H1N1 virus infection.
RESULTS: The patient was started on oseltamivir (75 mg, orally twice daily) and had a benign hospital course, with defervescence and resolution of symptoms within 72 hours. The follow-up chest radiograph after discharge was normal.
CONCLUSIONS: The 2009 H1N1 influenza in this hospitalized transplant recipient was relatively mild, and prolonged viral shedding was not noted. Oseltamivir can be a valid measure in immunocompromised individuals.

To the Editor:
Although diabetic ketoacidosis (DKA) occurs in totally pancreatectomized patients,[1] it has never been reported after partial pancreatectomy. We describe the development of DKA in a patient who underwent distal pancreatectomy.
A 60-year-old man with gastric cancer was treated with D2 gastrectomy along with splenectomy and distal pancreatectomy followed by Roux-en-Y reconstruction in July, 2009. He had not been previously diagnosed as having diabetes. He received chemotherapy with TS-1 (100 mg/d) from October, 2009 to February, 2010. At the beginning of February, 2010, he complained of appetite loss and general fatigue. Gastro-endoscopy showed no abnormality, and contrast-enhanced computed tomography of the abdomen also did not show any abnormality other than the absence of stomach, spleen, and pancreatic body and tail (Fig.). However, he still could not eat food except for candy and a nutritional supplementary high-calorie drink. He was admitted to the emergency department in a coma with Kussmaul breathing in the middle of February, 2010. Blood gas analysis revealed severe metabolic acidosis (pH, 7.13; pCO2, 15.6 mmHg; HCO3-, 5.2 mEq/L; anion gap, 37.7). Plasma glucose (1179 mg/dl) and hemoglobin A1c levels (13.2%; normal range, 4.3%-5.8%) were significantly elevated; however, the serum insulin level was very low (0.84 µIU/ml; normal range, 1.7-10.4 µIU/ml). Serum levels of total ketones (14 063 µmol/L; normal range, <130 µmol/L), acetoacetate (2981 µmol/L; normal range, <55 µmol/L) and beta-hydroxybutyrate (11082 µmol/L; normal range, <85 µmol/L) were greatly increased. These data suggested DKA. Hydration and continuous insulin infusion promptly ameliorated his consciousness and blood glucose levels. Intensive insulin therapy following insulin infusion further reduced the glucose toxicity, and his insulin secretion recovered (C-peptide level, 30.8 µg/d) at 5 days after admission. Finally, his blood glucose level before each meal became <150 mg/dl with metformin (750 mg/d), mitiglinide (30 mg/d) and insulin glargine (3 units/d) treatment, and he left hospital.
This is the first report of the development of DKA in a patient who underwent distal pancreatectomy. We usually pay attention to the development of diabetes in totally pancreatectomized patients; however, we are not likely to be attentive to glucose metabolism in patients who undergo distal pancreatectomy. Although D2 gastrectomy, splenectomy, and chemotherapy with TS-1 have not been reported to be associated with the development of DKA, total gastrectomy has been reported to be involved in the development of diabetes.[2] In this case, over-intake of simple sugar, total gastrectomy, and distal pancreatectomy may lead to the development of DKA. We should presume that distal pancreatectomy is a risk for the development of DKA.