Rare complication of inflammatory bowel disease-like colitis from glycogen storage disease type 1b and its surgical management: A case report

BACKGROUNDGlycogen storage disease (GSD) is an autosomal recessive inborn metabolic disorder. Patients with GSD are prone to hypoglycaemia, hyperlactacidemia and bleeding. GSD type 1b (GSD-1b) patients specifically can develop neutropenia, recurrent bacterial infection and inflammatory bowel disease (IBD). Documentation of the long-term outcomes of surgical management of GSD-1b has been scarce, especially for Asian patients. We herein describe a case of GSD-1b complicated by IBD-like colitis and coloduodenal fistula. The patient was managed successfully with surgical intervention.CASE SUMMARYA 20-year-old Chinese lady confirmed by genetic testing to have GSD-1b was initially managed with uncooked cornstarch and granulocyte-colony stimulating factor. With recurrent abdominal symptoms, her condition was treated as clinical “Crohn’s disease” with mesalazine, prednisolone and azathioprine conservatively. Colonoscopy showed a tight stricture at the hepatic flexure. Subsequent computerized tomographic colonography revealed a phlegmon at the ileocaecal region with a suspected coloduodenal fistula. Eventually an exploratory laparotomy was performed and severe colitis at the ascending colon with coloduodenal fistula was confirmed. Right hemicolectomy with primary anastomosis and repair of the duodenum were performed. Surgical management of complications from GSD-1b associated IBD-like colitis has rarely been described. First-line treatment would usually be conservative. Surgical intervention like hemicolectomy is mainly reserved for refractory cases.CONCLUSIONSurgical management of coloduodenal fistula in GSD-1b patients is a feasible and safe option when failed conservative management.     

Analysis of Altered Baseline Brain Activity in Drug-Naive Adult Patients with Social Anxiety Disorder Using Resting-State Functional MRI

Objective

We hypothesize that the amplitude of low-frequency fluctuations (ALFF) is involved in the altered regional baseline brain function in social anxiety disorder (SAD). The aim of the study was to analyze the altered baseline brain activity in drug-naive adult patients with SAD.

Methods

We investigated spontaneous and baseline brain activities by obtaining the resting-state functional magnetic resonance imaging data of 20 drug-naïve adult SAD patients and 19 healthy controls. Voxels were used to analyze the ALFF values using one- and two-sample t-tests. A post-hoc correlation of clinical symptoms was also performed.

Results

Our findings show decreased ALFF in the bilateral insula, left medial superior frontal gyrus, left precuneus, left middle temporal gyrus, right middle temporal pole, and left fusiform gyrus of the SAD group. The SAD patients exhibited significantly increased ALFF in the right inferior temporal gyrus, right middle temporal gyrus, bilateral middle occipital gyrus, orbital superior frontal gyrus, right fusiform gyrus, right medial superior frontal gyrus, and left parahippocampal gyrus. Moreover, the Liebowitz Social Anxiety Scale results for the SAD patients were positively correlated with the mean Z values of the right middle occipital and right inferior occipital but showed a negative correlation with the mean Z values of the right superior temporal gyrus and right medial superior frontal gyrus.

Conclusion

These results of the altered regional baseline brain function in SAD suggest that the regions with abnormal spontaneous activities are involved in the underlying pathophysiology of SAD patients.     

Tumor necrosis factor-alpha polymorphism, bone strength phenotypes, and the risk of fracture in older women

TNFalpha is a proinflammatory cytokine that promotes osteoclastic bone resorption. We evaluated the association between a G-308A polymorphism (rs1800629) at the TNFA locus and osteoporosis phenotypes in 4306 older women participating in the Study of Osteoporotic Fractures. Femoral neck bone mineral density (BMD) and structural geometry were measured using dual-energy x-ray absorptiometry and hip structural analysis. Incident fractures were confirmed by physician adjudication of radiology reports. Despite similar femoral neck BMD, women with the A/A genotype had greater subperiosteal width (P = 0.01) and endocortical diameter (P = 0.03) than those with the G/G genotype. The net result of these structural differences was that there was a greater distribution of bone mass away from the neutral axis of the femoral neck in women with the A/A genotype, resulting in greater indices of bone bending strength (cross-sectional moment of inertia: P = 0.004; section modulus: P = 0.003). Among 376 incident hip fractures during 12.1 yr of follow-up, a 22% decrease in the risk of hip fracture was seen per copy of the A allele (relative risk 0.78; 95% confidence interval 0.63, 0.96), which was not influenced by adjustments for potential confounding factors, BMD, or bone strength indices. The G-308A polymorphism was not associated with a reduced risk of other fractures. These results suggest a potential role of genetic variation in TNFalpha in the etiology of osteoporosis.     

Factors for prolonged length of stay after elective hepatectomy for hepatocellular carcinoma. The surgeon's role in the managed care era

BACKGROUND/AIMS: Length of stay is an important marker of medical resource consumption. In the modern managed care era physicians are driven to deliver the highest quality of care while using fewest resources. Hepatectomy represents a technically challenging and resource-intensive procedure, particularly in the setting of hepatic malignancy and liver cirrhosis. For improving quality of surgical management in such cases, we aimed to identify the factors affecting length of stay after hepatectomy for patients with hepatocellular carcinoma. METHODOLOGY: A total of 375 consecutive surgical hepatocellular carcinoma patients at a tertiary referral center during a 7-year period were reviewed. Length of stay after hepatectomy longer than 14 days was defined as prolonged length of stay. Patients were divided into two groups according to their length of stay. Patients with length of stay less than or equal to 14 days were in Group A and those with length of stay longer than 14 days were in Group B. Data for comparative analysis between both groups were categorized according to preoperative patients' clinical demographic factors, operation-related factors, and pathological factors. The significant univariate factors were used for subsequent multivariate analysis. RESULTS: The significant independent factors were patients' preoperative prothrombin activity, intraoperative blood transfusion, surgical complication, and the time to abdominal drain removal. Among these independent factors, surgical complication (p < 0.001, relative risk 7.01, and 95% confidence interval 3.46 to 14.18) was the most powerful factor for prolonged length of stay after elective hepatectomy for hepatocellular carcinoma. CONCLUSIONS: Most of the independent factors for prolonged length of stay after elective hepatectomy for hepatocellular carcinoma patients are operation-related and surgeon-dependent. The surgeon plays a key role in determining length of stay. By minimizing blood transfusion, surgical complication, and the time to abdominal drain removal, length of stay can be significantly decreased in hepatocellular carcinoma patients undergoing hepatectomy.     

Impact of a Low-Glucose Peritoneal Dialysis Regimen on Fibrosis and Inflammation Biomarkers

♦ Background: The impact of a low-glucose peritoneal dialysis (PD) regimen on biomarkers of peritoneal inflammation, fibrosis and membrane integrity remains to be investigated.♦ Methods: In a randomized, prospective study, 80 incident PD patients received either a low-glucose regimen comprising Physioneal (P), Extraneal (E) and Nutrineal (N) (Baxter Healthcare Corporation, Deerfield, IL, USA) (PEN group), or Dianeal (control group) for 12 months, after which both groups continued with Dianeal dialysis for 6 months. Serum and dialysate levels of vascular endothelial growth factor (VEGF), decorin, hepatocyte growth factor (HGF), interleukin-6 (IL-6), macrophage migration inhibitory factor (MIF), hyaluronan (HA), adiponectin, soluble-intracellular adhesion molecule (s-ICAM), vascular cell adhesion molecule-1 (VCAM-1) and P-selectin, and dialysate cancer antigen 125 (CA125), were measured after 12 and 18 months. This paper focuses on results after 12 months, when patients in the PEN group changed to glucose-based PD fluid (PDF).♦ Results: At the end of 12 months, effluent dialysate levels of CA125, decorin, HGF, IL-6, adiponectin and adhesion molecules were significantly higher in the PEN group compared to controls, but all decreased after patients switched to glucose-based PDF. Macrophage migration inhibitory factor level was lower in the PEN group but increased after changing to glucose-based PDF and was similar to controls at 18 months. Serum adiponectin level was higher in the PEN group at 12 months, but was similar in the 2 groups at 18 months. Body weight, residual renal function, ultrafiltration volume and total Kt/V did not differ between both groups. Dialysate-to-plasma creatinine ratio at 4 h was higher in the PEN group at 12 months and remained so after switching to glucose-based PDF.♦ Conclusion: Changes in the biomarkers suggest that the PEN PD regimen may be associated with better preservation of peritoneal membrane integrity and reduced systemic vascular endothelial injury.     

Performance of a prehospital trauma diversion system in Hong Kong, China

Purpose: To evaluate the performance of a prehospital trauma diversion system in Hong Kong, China. Methods: A retrospective analysis of prospectively collected data in the trauma registry of Queen Mary Hospital, Hong Kong from 1 January 2009 to 31 December 2013 was done. All adult patients aged 18 years or above, either primarily or secondarily diverted to Queen Mary Hospital according to the trauma patient diversion protocol, were recruited. Need for trauma center level of care was based on a consensus-based criterion standard published in 2014. Performance of the protocol in terms of overdiversion and under-diversion was determined. Results: A total of 209 patients were included for analysis. About 30% of the patients required trauma center level of care. The most common reason was the need for vascular, neurologic, abdominal, thoracic, pelvic, spine or limb-conserving surgery within 24 h of presentation. The over-diversion rate and underdiversion rate were 69.6% and 19.7% respectively. Conclusion: The trauma patient diversion protocol currently in use in Hong Kong is not accurate enough. Further revision and refinement is needed.