A. Dalgic

Clinical approach to graft hepatic artery thrombosis following living related liver transplantation

Abstract: Hepatic artery thrombosis (HAT) has an occurrence rate of 1.7–26% following living donor liver transplantation (LDLT) and is one of the most common reasons for graft loss and mortality in this population. There is a higher incidence of HAT in pediatric recipients. The aim of this case report is to discuss clinical approaches for the treatment of HAT occurring in the early post‐operative period after LDLT. An 11‐month‐old, 7.8‐kg female with cirrhosis secondary to biliary atresia underwent LDLT at Gazi University Hospital in Ankara. The graft was a left lateral segment from her father with a left hepatic artery (HA) of 2 mm diameter and a graft weight/recipient body weight ratio of 2.0%. After an uneventful early post‐operative period, HAT was diagnosed by Doppler ultrasonography (USG) on the fifth post‐operative day. Following angiographic evaluation, immediate exploration and reanastomosis was performed using an operation microscope. Post‐operatively, the HA was patented by Doppler USG and graft function returned to normal. Now, 42 months later, the patient continues to do well with normal graft function, using a regimen of tacrolimus monotherapy for immunosuppression. In countries which have very limited resources for urgent re‐transplantation, given their serious donor shortage, graft salvage may be the only option for patient survival when HAT occurs. In these circumstances, early diagnosis and immediate revascularization may be the only method for graft salvage. A daily routine of Doppler USG examination in the early post‐operative period may provide a method for the early diagnosis of HAT, before liver enzymes are elevated and hepatic necrosis has begun.

Severe peripheral pulmonary artery stenosis is not a contraindication to liver transplantation in Alagille syndrome.

Abstract:  We described a case of Alagille syndrome with severe peripheral pulmonary artery stenosis and very high right ventricular pressure that underwent successful living‐related liver transplantation without any peri‐operative and mid‐term postoperative complication because of this cardiac malformation. The aim of this report is to point out that the severe pulmonary artery stenosis may be a risk factor but not a contraindication to liver transplantation in patients with Alagille syndrome.

Successful multimodal treatment for aggressive metastatic and recurrent fibrolamellar hepatocellular carcinoma in a child.

Fibrolamellar variant of hepatocellular carcinoma (FLHCC) does not have a favorable prognosis than conventional HCC, and there is no difference regarding the response to chemotherapy and the degree of surgical resectability. FLHCC commonly recurs after complete surgical resection, and there is a high rate of lymph node metastases. Herein, we report a 12-year-old girl with metastatic FLHCC with multiple recurrences aggressively treated with surgery, chemotherapy, and antiangiogenic agents. She is in complete remission after 4 years and 2 months after the diagnosis of metastatic FLHCC. The standard treatment of FLHCC is excision of the primary tumor and its metastases. Chemotherapy for FLHCC is controversial, and it has been suggested that cytoreductive chemotherapy was ineffective and adjuvant chemotherapy did not improve survival. Our patient with multiple recurrences was successfully treated with surgery, first-line chemotherapy with cisplatin and doxorubicin, second-line chemotherapy with 5-fluorouracil/interferon-&agr; combination, and adjuvant antiangiogenic agents like cyclophosphamide and thalidomide. As FLHCC patients have no underlying liver disease, they can tolerate higher doses of chemotherapy compared with conventional HCC patients. We support the use of repeated aggressive surgery with adjuvant chemotherapy and antiangiogenic therapy, which provided complete remission in our patient with metastatic and recurrent FLHCC.

Micronuclei and other nuclear anomalies in buccal epithelial cells of children with chronic kidney disease

Abstract The objective of this study was to reveal the likely genomic instability in children with chronic kidney disease (CKD) using micronucleus (MN) assay on buccal epithelial cells (BEC). We investigated the frequencies of micronuclei and other nuclear anomalies, such as nuclear buds, binucleated cells, condensed chromatin, and karyorrhectic and pyknotic cells in BEC. Children with CKD were grouped as follows: children in the pre-dialysis (PreD) stage (N=17), children on regular haemodialysis (HD) (N=14), and children who have undergone transplantation (Tx) (N=17). As a control group, twenty age- and gender-matched healthy children were selected. The MN frequency in BEC of all groups of children with CKD was significantly elevated (5- to 7-fold) as compared to the control group (p<0.001). In contrast, the frequencies of nuclear buds were not significantly higher in the study groups compared to the control group. The frequencies of binucleated cells and condensed chromatin cells were significantly higher in all subgroups of children with CKD relative to the control group (p<0.001). Our results show that the BEC of pediatric PreD, HD, and Tx patients with CKD display increased cytogenetic, cytokinetic, and cytotoxic effects. They also point to the sensitivity and usefulness of the BEC MN assay in the assessment of genetic susceptibility of patients with CKD.

Spontan hepatosellüler karsinoma kanaması: Olgu sunumu

Bleeding from hepatocellular carcinoma is the third leading cause of mortality in hepatocellular carcinoma. Difficulties in diagnosis and treatment represent the greatest challenges. A 49-year-old man with cirrhosis due to hepatitis B virus had admitted to the hospital because of abdominal pain lasting for two days. The patient had tachycardia and hypotension, and abdominal distension. The levels of bilirubin and albumin were 8.2 mg/dl and 2 g/dl, respectively. The abdominal tomography revealed a 5x6 cm mass and hemorrhagic fluid. In the paracentesis, hemorrhagic fluid was aspirated. The patient underwent emergency surgery because of the hemorrhagic paracentesis fluid and existing shock. Bleeding from the mass in the 6th segment of the liver was controlled by suturing. The bleeding stopped, but the patient died on the 5th day due to liver insufficiency. Diagnostic difficulties, liver insufficiency, hemorrhagic shock, coagulation problems, and unknown masses are the risk factors for increasing mortality. Risk factors for bleedings should be evaluated during the follow-up of patients with cirrhosis.

OUTCOME OF RENAL TRANSPLANTATION IN CHILDREN WITH DIFFERENT ORIGINATED URINARY TRACT DYSFUNCTION: A SINGLE-CENTER EXPERIENCE: 657

Introduction: Renal transplantation in patients with urinary tract dysfunction (UTD) of various origins is a challenging issue in the field of pediatric transplantation. AIM: To evaluate patient and graft survivals as well as the risks of the surgery and immunosuppressive therapy pediatric patients of UTD at Gazi University, Transplantation Center. PATIENTS AND METHODS: Among 56 pediatric transplantation recipients since 1996, UTD was displayed in 7 (12,5%) pediatric recipients. Videourodynamic tests were performed to all patients preoperatively as well as postoperatively if required. The cause of urologic disorders were as follows; PUV (n=2), PUV+neurogenic bladder (n=2) meningiomyocele+neurogenic bladder (n=1), and right multicystic dysplastic kidney (MCDK)+PUV (n=2). Clean intermittent catheterization (CIC) was needed in three patients to empty the bladder. None of patients had pretransplantation augmentation. Only one patient whom has VUR+neurogenic bladder has augmentation operation during transplantation. Carrel Patch technique in cadaveric, standard technique in Living donors with continuous 6/0 7/0 Propylene suture (end to side RA/V to Ext IA/V, Abd aorta and IVC in small kids) were used for vascular anastomosis. Haberal’s corner saving suture technique were used with ureteral stenting for ureteroneocystostomy anastomosis. Three out of 7 patients received kidneys from cadaveric and 4 from living donors. All patients were received calcineurin-based triple immunosuppressive therapy. RESULTS: The mean age at transplantation was 10,7±3,8 years old ( 415 years old). The median follow-up after transplantation was 26 months (18 to 142 months). Three patients who have neurogenic urine bladder had recurrent symptomatic curable urinary tract infections. Three out of 7 recipients have BKVAN. One out of 3 grafts was lost due to BKAVA and other two have normal graft functions. None of the recipients had urine leak after transplantation. None of the grafts were lost due to UTD. Although, we lost one graft (nonUTD problem), remaining all 6 patients are doing well with mean creatinine 1,5 ±1,1 mg/dl (median 1,2 mg/dl). CONCLUSION: Renal transplantation is a safe and effective treatment option.. While surgery and follow-up is more complicated in such patients group, UTD and/or underlying urologic diseases should be followed up very closely than other transplantation patients.

EC-MPS MAY HAS LESS HEPATOTOXICITY AND GASTROINTESTINAL IN KIDNEY TRANSPLANT RECIPIENTS (SUMMARY OF THE SIDE EFFECTS AT POSTOPERATIVE FIRST 3 MONTHS); SINGLE CENTER EXPERIENCE: 652

Introduction: Mycophenolates (MPA) are the most frequently used immunosuppressive drugs in solid organ transplantation. Entericcoated mycophenolate sodium (EC-MPS) is a new formulation of mycophenolic acid that delivers the active moiety MPA, the same active moiety delivered by mycophenolate mofetil (MMF). Aim: Here we retrospectively analyzed data from patient charts at Gazi University Transplantation Center to assess and compare efficacy and adverse events of MMF and EC-MPS in first 3 months after transplantation. Material and Methods: Data collected from patients who received first kidney transplantation years between 2006-2009 in Gazi University Transplantation Center. Between these years, totally 83 kidney transplantations were performed. Twenty one out of 83 are pediatric, 62 out of 83 are adult recipient. Eighty out of 83 recipients enrolled for this study. There were 52 de novo MMF (group A), and 28 EC-MPS (group B) patients in each group. In group A, mean age was 32,4 ±14,6 years old. Twenty three of the recipients were female and 29 of were male. Transplantations were performed from deceased donor in 29 and from living donor in 23. In group B, mean age was 29,1 ±13,5 years old. Twelve of recipients were female and 16 were male. Transplantations were performed from deceased donor in 18 and from living donor in 10. Immunosuppressive protocol consists of calsineurin based triple regimen and Basiliximab induction (only for cadavers, second or more transplantation, high PRA). All recipients received preoperatively single dose of either MMF 1000 mg or ECMPS 720 mg in living transplantation. After transplantation, from DO, all patients received either MMF 1000 mg b.i.d. or EC-MPS 720 mg b.i.d. Results: In group A, we have not seen any side effect of MMF in 42% (n=22) recipients during postoperative first 3 months. Fifty six percent of recipients had following side effects; gastrointestinal 23%, bone marrow 23% and hepatotoxicity 11%. Majority of the GISE is diarrhea (n=10), and dyspepsia (n=2) follows. Majority of BMSE is leukopenia (n=9), and pansitopenia (n=2), anemia (n=1) follows. Side effects have ceased by stopping MMF in 11 patients, by reducing the drugs in 11 patients and by converting in 7 patients. In group B, we have not seen any side effect of EC-MPS in 50% (n=14) recipients postoperative first 3 months. Fifty percent of recipients had following side effects; bone marrow 25%, gastrointestinal 21,5% and hepatotoxicity 3,5%. Majority of the GISE is diarrhea (n=4) and dyspepsia (n=2), floating (n=1). Majority of BMSE is leukopenia (n=6), and pansitopenia (n=1) follows. Side effects have ceased by stopping EC-MPS in 4 patients, by reducing the dose in 7 patients and by converting in 5 patients. We have not seen any statistical differences between two groups concerning side effects (p> 0,5) within 3 months after transplantation. Conclusion: EC-MPS and MMF demonstrated therapeutic equivalence in de novo renal transplant patients. EC-MPS has significantly less hepatotoxicity, and better GI side effect.

Influence Of Hla Compatibility On Success With Living-related Pediatric Liver Transplantation

It is not clear how HLA compatibility influences acute rejection and postoperative complications in cadaveric liver transplantation. Even less is known about this factor in pediatric living-related liver transplantation (LRLT). This research assessed HLA compatibility relative to rejection rates and complications in pediatric LRLT. The study retrospectively investigated data from 14 pediatric LRLTs in which the donor and recipient HLA genotypes were determined preoperatively. Three recipients (21.4%) developed biliary complications (two biliary leakage, one bile duct stenosis). Three others (21.4%) developed vascular complications (two hepatic artery thrombosis, one hepatic artery stenosis). Eight recipients (57.1%) were diagnosed with acute rejection. The incidence of acute rejection was not correlated with the number of HLA mismatches (P > .05), or with the number of HLA class I mismatches (P > .05); however, it was negatively correlated with number of HLA class II mismatches (P = .02). Arterial and biliary complications were not correlated with any of these categories of HLA compatibility. In conclusion, the data from this small group of patients provided no evidence that closeness of donor-recipient HLA matching influences outcome in pediatric LRLT.