Current status of intestinal transplantation in East Asia.

PURPOSE OF REVIEW: Intestinal transplantation (ITx) activity remains low in East Asia. We conducted a multinational, retrospective study on patients who underwent ITx in Korea, Taiwan, and Japan, to provide an overview and to foresee future developments and collaborations in this region. RECENT FINDINGS: Total 71 ITx were performed in 67 patients. Living donor ITx was most commonly conducted in Japan (n = 13). Despite the low caseload, all three countries demonstrated acceptable patient survival rates of approximately 70% at 5 years. Over 70% of recipients with a functioning graft were free from total parenteral nutrition. SUMMARY: There is an urgent need to establish a nationwide and multinational registry of ITx recipients and patients with intestinal failure in East Asia. An efficient referral system to specialized intestinal rehabilitation and ITx centers and a multidisciplinary team approach is also warranted to provide state-of-the-art treatment for patients desperately waiting for a chance to survive.

Long-Term Outcomes of Renal Function After Adult Intestinal Transplantation at a Single Small-Volume Center: 2 Cases of Renal Transplantation.

PURPOSE: Renal dysfunction more frequently occurs after intestinal transplantation (ITx) than after heart, lung, or liver transplantation. We provide a clinical analysis of renal function after adult ITx. METHODS: We retrospectively analyzed 8 adult ITx patients who survived for at least 6 months between 2004 and 2018. Glomerular filtration rate (GFR) measurements were performed at baseline, at 3 and 6 months post-transplantation, and yearly. The median follow-up duration was 53.5 months. RESULTS: All cases were isolated ITx; 3 received living-donor ITx, and 5 received deceased-donor ITx. The mean baseline GFR was 97 mL/min/1.73 m2. The GFR had decreased by more than 50% of baseline at 1 year post-transplant. Renal dysfunction was observed in 4 patients. Two patients developed acute kidney injury due to acute rejection and sepsis. One of these patients fully recovered renal function, but the second patient died. Another 2 patients developed chronic kidney disease and required hemodialysis (HD) within 6 and 3 years, respectively. The first living-donor ITx patient lost renal function progressively over 6 years after ITx. She received a renal graft from the same living donor as for the ITx after 3 years of HD. The other patient (deceased-donor ITx) received a kidney from his daughter at 5 months after HD. CONCLUSIONS: To obtain an accurate assessment of renal function, frequent direct measurements of GFR should be performed to facilitate early diagnosis of renal impairment and to determine subsequent strategies to improve renal function after ITx.

A missense mutation in EBF2 was segregated with imperforate anus in a family across three generations.

The etiology of imperforate anus, a major phenotype of anorectal malformation (ARM), is still unknown and not a single gene has been reported to be associated with it. We studied a Korean family with six affected members with imperforate anus across three generations by whole exome sequencing and identified a missense mutation in the EBF2 gene (c.215C > T; p.Ala72Val). This mutation is completely segregated with the disease phenotype in the family and is evolutionarily highly conserved among diverse vertebrates. Also, this mutation was predicted to be functionally damaging. These results support that missense mutation in the EBF2 c.215C > T (p.Ala72Val) is very likely to contribute to the pathogenesis of ARM in this family.

Cushing syndrome secondary to CRH-producing Wilms tumor in a 6 year old.

Abstract Cushing syndrome is caused by prolonged exposure to elevated serum cortisol. It is uncommon in children, and etiology includes pituitary adenoma, adrenal tumor, and exogenous glucocorticoid administration. Rarely, it is paraneoplastic in origin. We present a case of paraneoplastic Cushing syndrome due to Wilms tumor that secreted corticotropin-releasing hormone (CRH). A 6-year-old male presented with polyphagia and weight gain. He showed Cushingoid appearance, hypertension, and palpable left flank mass. Serum cortisol and adrenocorticotropic hormone (ACTH) levels were elevated. Computed tomography showed a neoplasm originating from the left kidney. Pathologic diagnosis of Wilms tumor was made upon nephroureterectomy. Immunohistochemical staining was positive for CRH and negative for ACTH. All features of Cushing syndrome disappeared after surgery. This represents a rare case of Cushing syndrome secondary to Wilms tumor in which CRH production has been demonstrated.

[Efficacy of feeding pump for patients on enteral tube feeding: a systematic literature review and analysis].

BACKGROUND/AIMS: This study was conducted to establish a guideline on the utilizing of feeding pump in patients requiring enteral tube feeding. METHODS: As a first step, textbooks on nutrition and guidelines from regional clinical nutrition societies were analyzed. Afterwards, data on the efficacy, safety, and practicality of feeding pump application were collected and evaluated by systematically reviewing the related literature. As data sources, 8 domestic databases including KoreaMed and global databases such as Ovid-MEDLINE, Ovid-EMBASE, and Cochrane Library were utilized. A total of 2,016 related articles was selected by applying the keyword "(enteral feeding.mp AND pump.mp)". RESULTS: Textbooks and guidelines were not able to draw conclusions on the effects of the feeding pump because the injection speed, tube size, and etcetera were different for enteral feeding. Feeding pump assisted enteral tube feeding was an efficient, safe, and practical procedure for reducing maladjustment-related complications of enteral tube feeding, which are obvious obstacles for maintaining nutritional balances in patients requiring tube feeding. CONCLUSIONS: Feeding pump application can be considered an efficient and safe measure that is acceptable in patients on small intestinal tube feeding, critically-ill patients on gastro-intestinal tube feeding, premature babies, and critically-ill or severely malnourished children (recommendation grade D).

Effects of branched-chain amino acid infusions on liver regeneration and plasma amino acid patterns in partially hepatectomized rats.

BACKGROUND/AIMS: Even though solutions of high branched-chain amino acids (BCAAs) are known to be beneficial to patients with hepatic encephalopathy, data on the effects of BCAA on liver regeneration are limited. METHODOLOGY: Two groups of young adult rats were utilized in this study: standard total parenteral nutrition solutions were administered to group SS (22.59% of BCAAs in total amino acids) and solutions high in BCAAs were given to group HS (34.44% of BCAAs). After 2 days of infusion, 70% partial hepatectomy was performed and the TPN infusions were continued for another 3 days. Liver regeneration was estimated and changes in serum amino acid patterns were determined 3 days after hepatectomy. RESULTS: Thymidine incorporation into liver DNA, in group HS was significantly higher than that in group SS (378±56 vs. 324±38, p=0.032). In group SS, the total concentration of BCAAs decreased (165.11±39.90mg/dL and 145.56±29.53mg/dL, p=0.014) over the experimental period, whereas that of aromatic amino acids (AAAs) increased (226.56±36.5mg/dL and 247.00±48.36mg/dL, p=0.104). However, group HS showed no changes in BCAA or AAA sums over the experimental period. CONCLUSIONS: After major hepatectomy, supplementation with high BCAAs helps not only to maintain a stable plasma BCAA/AAA ratio, but also promotes liver regeneration.

Clinical analysis of living donor liver transplantation in patients with portal vein thrombosis.

The aim of this study was to improve outcomes in living donor liver transplantation (LDLT) patients with portal vein thrombosis (PVT). Of 246 adult patients who underwent LDLT with a right lobe graft between January 2000 and May 2007, PVT was diagnosed in 50 patients (20.3%), who were further subdivided into partial (n = 39, 78%) and complete (n = 11, 22%) types. Patients with PVT, especially complete PVT, showed high incidences of variceal bleeding (p = 0.021), operative RBC transfusion (p < 0.046) and a post-transplantation complications related to bleeding (p = 0.058). We also classified PVT according to its location and the presence of collaterals: type I (n = 41, 82%): PVT localized above the confluence of the splenic and superior mesenteric veins (SMV); type II (n = 7, 14%): PVT extending below the confluence with a patent distal SMV; type III (n = 2, 4%): complete portal vein and SMV thrombosis except for a coronary vein. LDLT could be safely undertaken in patients with PVT without increased mortality. In our type II and III PVT, when thrombectomy fails, jump grafting using a cryopreserved vessel may serve as a reliable alternative method to restore portal flow.

Liver transplantation versus liver resection for the treatment of hepatocellular carcinoma.

PURPOSE: Liver resection (LR) and liver transplantation (LT) are considered the only two potentially curative treatments for hepatocellular carcinoma (HCC). Recently, there has been an intense debate as to whether LR or LT is the optimal initial treatment for patients with Child A or B cirrhosis. The aim of this study was to compare the results of LR and LT in patients with HCC and with Child A or B cirrhosis in a single center over a 10-year period. METHOD: Seventy-eight patients were treated with LT and 130 were treated with LR. We evaluated patient characteristics, short-term results such as hospital stay, postoperative complication, mortality, and long-term results such as overall and recurrence-free survival and recurrence. RESULTS: The hospital stay of the LT group was significantly longer than that of the LR group (P < 0.001). The postoperative complication rate and the early operative mortality rate were similar between the two groups. The overall survival rate was higher after LT than it was after LR, but not to a statistically significant degree (P = 0.267). The recurrence-free survival rate was significantly higher after LT than it was after LR (P = 0.002). Within and beyond the Milan criteria, the overall survival rate was higher after LT than it was after LR, but not to a statistically significant degree. The recurrence-free survival rate was significantly higher after LT than it was after LR in the patients within Milan criteria (P < 0.001). HCC recurred more frequently after resection (51.5%) than it did after transplantation (29.5%) (P < 0.001), and HCC recurrence developed in the liver more frequently after LR than it did after LT (P = 0.002). However, after recurrence, LR had better survival than LT did, but not to a statistically significant degree (P = 0.177). CONCLUSION: LT should be considered as the primary treatment in patients with HCC within the Milan criteria. LR is recommended for patients with HCC beyond the Milan criteria. The LT group showed a significantly lower recurrence rate than the LR group. However, in the case of recurrence, the LT group showed a poorer long-term outcome than the LR group.

Epidemiology and risk factors for bacteremia in 144 consecutive living-donor liver transplant recipients.

PURPOSE: Bacteremia is a major infectious complication associated with mortality in liver transplant recipients. The causative organisms and clinical courses differ between medical centers due to variations in regional bacterial epidemiology and posttransplant care. Further, living donors in Korea contribute to 83% of liver transplants, and individualized data are required to improve survival rates. PATIENTS AND METHODS: We retrospectively analyzed 104 subjects who had undergone living-donor liver transplant from 2005 to 2007. RESULTS: Among the 144 consecutive living-donor liver transplant recipients, 24% (34/144) developed bacteremia, 32% (46/144) developed non-bacteremic infections, and 44% (64/144) did not develop any infectious complications. Forty episodes of bacteremia occurred in 34 recipients. The major sources of bacteremia were intravascular catheter (30%; 12/40), biliary tract (30%; 12/40), and abdomen (22.5%; 9/40). Gram-positive cocci were more common (57.5%; 23/40) than Gram-negative rods (32.5 %; 13/40) and fungi (10%; 4/40). The data revealed that the following factors were significantly different between the bacteremia, non-bacteremic infection, and no infection groups: age (p = 0.024), posttransplant hemodialysis (p = 0.002), ICU stay (p = 0.012), posttransplant hospitalization (p < 0.0001), and duration of catheterization (p < 0.0001). The risk factors for bacteremia were older than 55 years (odds ratio, 6.1; p = 0.003), catheterization for more than 22 days (odds ratio, 4.0; p = 0.009), UNOS class IIA (odds ratio, 6.6; p = 0.039), and posttransplant hemodialysis (odds ratio, 23.1; p = 0.001). One-year survival rates in the bacteremic, non-bacteremic infection, and no infection groups were 73.2%, 91.3%, and 93.5%, respectively. CONCLUSION: Early catheter removal and preservation of renal function should focus for improving survival after transplant.

Isolated small bowel transplantation from a living-related donor at the Catholic University of Korea--a case report of rejection -free course-.

The bowel transplantation team at the Catholic Medical Center, Korea, on April 9 2004, accomplished a case of isolated small bowel transplantation (SBT) in a 57 year-old female with short bowel syndrome. The primary surgery was a jejunocolostomy due to mesenteric vein thrombosis, while maintaining 30 cm of the jejunum and colon distal to the splenic flexure. Her renal function was partially unbalanced. During more than 2 years of home TPN, the superior vena cava (VC) and subclavian veins had become occluded, but the inferior VC line remained. SBT was planned due to the repeated life-threatening infections of the last central line. One hundred and fifty centimeter of the distal ileum of the 27 year-old living-related donor, the patient's daughter, was harvested. The graft mesenteric artery and vein were anastomosed to the recipient's inferior mesenteric vessels. A proximal end-to-end jejuno-ileostomy and a distal end-to-side ileo-colostomy of the graft were made, creating a Bishop-Koop enterostomy for graft surveillance. A tube jejunostomy, via a gastrostomy, was established for early feeding and simultaneous gastric drainage. Induction with Daclizumab and immunosuppression consisted of tacrolimus and methylprednisolone, given intravenously, and then mycophenolate mofetil (MMF), enterally from day 3. The patient was discharged on day 42. A CMV infection on day 83 was successfully treated with 3 weeks of gancyclovir therapy. She has been nutritionally independent, with complete oral feeding, and free of rejection until day 170 after the transplantation.

Stability of total nutrient admixtures in reference to ambient temperatures.

OBJECTIVE: To evaluate the stability of emulsions under different temperatures simulating clinical conditions of storage and exposure during infusion, five total nutrient admixture formulas in this institution were analyzed: adult, patients with hepatic failure, infants, stressed patients, and patients with renal failure. METHODS: Each mixture was allocated in a sterile 100-mL glass bottle, which was prefilled and refilled with nitrogen gas. Bottles were stored at 4 degrees C for 0 d, 3 d, and 7 d and then exposed to three different temperatures: usual room temperature (18 degrees C to 25 degrees C), high (>28 degrees C) in a water bath, or storage (4 degrees C) for 24 and 48 h. The gross inspection of the emulsions and parallel measurements of pH, particle sizes, divalent ions, peroxide levels, and microbial cultures were performed. RESULTS: Every lot was stable near 18 degrees C, but 8 of 10 lots stored for 7 d (25 degrees C and >28 degrees C) and 15 of 20 lots stored for 3 and 7 d (25 degrees C and >28 degrees C) showed coalescence. The overall coalescence incidences by storage, exposure, and heat were statistically significant (P < 0.005). CONCLUSION: For the safety of total nutrient admixtures, special attention is required to keep the ambient temperature below 28 degrees C and completely exclude air from the container.

[A clinical analysis on liver transplantation for hepatocellular carcinomas].

BACKGROUND/AIMS: Currently, the role of liver transplantation in the treatment of hepatocellular carcinoma (HCC) with cirrhosis is controversial. What remain to be determined are the best treatment protocol and who are likely to have a good outcome after liver transplantation. METHODS: Twenty-five patients with hepatocellular carcinoma underwent liver transplantation between 1993 and 2001 at the Department of Surgery, Catholic University of Korea. The follow-up period was from one month to 51 months. The pathologic findings, the recurrence, and survival of the 25 cases were analyzed. RESULTS: Two patients had a tumor larger than 5 cm in diameter and three patient had above 3 nodules in number. Five patients had bilobar tumors. Vascular invasion was present in 11 patients (45.8%). Among the 25 patients, postsurgical TNM staging was stage III in 3 patients, stage IVA in 5 patients, and stage IVB in 1 patient. Number of high risk patients were 16 (64%). During follow-up, 23 of the 25 patients (92%) were alive and the number of disease-free survivals was 21 among the 23 patients (91.3%). CONCLUSIONS: According to our small experience, HCC can be a good indication of liver transplantation, especially in low risk patients and even in the recurrent cases. A long-term survival can be achieved by aggressive treatment. However, the best protocol remains to be determined, especially for the case with large tumors.

Glutamine on the luminal microbial environment after massive small bowel resection.

To evaluate the oral glutamine (GLN) on the luminal microbes and bacterial translocation (BT) in short bowel, 45 Wistar rats were utilized in three groups; A (control), and B and C (short bowel, 85% of small bowel resected). The group A was fed with elemental diet (EmD), B with EmD+2% glycine, and C with EmD+2% GLN. The groups B and C were isocaloric and isonitrogenous. Wet weight, DNA, protein, and histomorphometry of the mucosa and parallel microbial culture from cecal contents, caval blood, and tissue blocks of the liver, spleen, and mesenteric lymph nodes were performed on the 5th, 10th, and 15th day. Mucosal growth was higher in group C than B. Colony forming units (CFU) from cecal contents increased more in group B than in C. BTs in A, B, and C were 7/15, 8/15, and 2/15, respectively. Total CFUs in blood and tissues were 5.8 X 10(4)/g, 5.5 X 10(6)/g, and 1.8 X 10(4)/g, respectively. As for BT, the most frequent organism was Klebsiella in A (79.3%), but E. coli in B and C (94.2% and 55.6%). GLN seems to suppress luminal microbes, and reduces BT in short bowel due to enforced barrier function and proliferation of the mucosa.