Liver transplantation for caroli disease

Caroli disease is a rare congenital disorder characterized by multifocal, segmental dilatation of intrahepatic bile ducts. Patients with Caroli disease who have recurrent bouts of biliary infection, particularly those who also have complications related to portal hypertension may require liver transplantation. In liver transplant ward of Shiraz University of Medical Science we had 4 patients with Caroli disease who were transplanted. Herein, we describe the demographic characteristics and post-transplant course of the patients. These patients presented with liver failure, recurrent cholangitis and portal hypertension sequelae unresponsive to medical treatment. The mean age of patients was 24.5 [range: 18-36] years, the mean MELD score was 17.5 [range: 11-23], three patients were female; one was male. All of the patients had good post-transplantation course except for one patient who developed post-operative biliary stricture for whom biliary reconstruction was done

Comparative screening of chlamydia trachomatis infection in women population in Tehran, Iran

There are more than 30 different sexually transmissible agents while the most common one is Chlamydia trachomatis. In this prospective study, we decided to compare the prevalence of infection in symptomatic and asymptomatic females. Two hundred sixty urine samples of women in two groups [symptomatic and asymptomatic] were collected from patients attending Mehrad Hospital in Tehran, Iran and tested by polymerase chain reaction. Thirty nine women in both groups were infected [14.99%], while 27/130 subjects were in symptomatic group [20.76%], compared with 12/130 person in asymptomatic group [9.23%]. No statistically significant difference was found between two groups. Data analysis showed infection with C. trachomatis in symptomatic women to be significantly associated with history of sexually transmitted infections, white blood cells in urine and epithelial cells in urine. The present study recommends that targeted screening programs in high risk sexually active women [like as individuals who had a history of STIs] are needed as part of case-finding strategies and treatment

En-bloc transplantation: an eligible technique for unilateral dual kidney transplantation

Kidney transplantation is the best available treatment for patients with end-stage renal disease. To evaluate the en bloc anastomosis technique for unilateral dual kidney transplantation [DKT]. From May to October 2011, 5 patients [4 women and 1 man] with mean age of 31.8 years underwent unilateral DKT with this technique in which distal end of the aorta and proximal end of inferior vena cava [IVC] were closed with running sutures. Then, proximal end of the aorta and distal end of the IVC were anastomosed to internal [or external] iliac artery and external iliac vein, respectively. Post-operative course was uneventful. No vascular and urologic complications developed; all patient had acceptable serum creatinine at discharge time and up of 2-6 months of post-operation follow up. Unilateral DKT is a safe method for performing DKT. The proposed en bloc anastomosis can improve the outcome of the graft by reducing the cold ischemia and the operation time

Prevalence of chlamydia trachomatis and mycoplasma genitalium in pregnant women of Sabzevar- Iran

As prenatal screening for sexually transmitted infections and treatment of infected pregnant women is not routinely performed in Iran and prevalence of two sexually transmitted pathogens, Chlamydia trachomatis and Mycoplasma genitalium, in Sabzevar [east of Iran] is unknown, we decided to perform this prospective study. One hundred ninety-six urine specimens of pregnant women attending the specialized maternity hospital of the city were collected and tested by duplex PCR. A total of 31 specimens were positive [15.81%] [27 Chlamydia trachomatis isolates, 13.77%; and 2 Mycoplasma genitalium isolates, 1.02%]. Co-infection with both species was detected in 2 specimens [1.02%]. A significant correlation was found between preterm labor and infection [P-value < 0.05]. The present study shows high prevalence of Chlamydial infections in comparison with Mycoplasma genitalium in this region. Further studies with larger sample size and more focused on different groups at risk are needed for a movement towards prevention and control of sexually transmitted infections [STIs]

Endothelial nitric oxide synthase gene t-786c polymorphism in renal transplant recipients

Nitric oxide [NO] is a major mediator in vascular biology, regulating regional blood flow. NO and the enzymes required for its production contribute to ischemia-reperfusion injury. The T-786C functional polymorphism in the promoter region substantially reduces promoter activity of the endothelial nitric oxide synthase [eNOS] gene and compromises endothelial NO synthesis. To examine the association between T-786C [rs 2070744] single nucleotide polymorphism [SNP] in eNOS gene and the development of acute rejection in renal transplant patients. 60 renal transplant recipients [30 with episodes of acute rejection [ARs] and 30 without rejection [non-ARs]], between June 2008 and March 2010, were included in this study. The polymorphism was determined by PCR-restriction fragment-length polymorphism analysis. The distribution of the genotypes were TT/TC/CC 60%, 33.4%, 6.6%, and 43%, 46.7%, 13.3% in ARs and non-ARs, respectively [p=0.28]. The frequency of T-allele was 76.7% and 66.3%; and for C-allele was 66.6% and 33.3% in ARs and non-ARs, respectively [p=0.09]. There were no significant associations between these polymorphisms and acute and chronic kidney allograft rejection. We could not detect any significant association between polymorphism in T-786C of eNOS gene and the development of acute rejection

Evaluation of cytomegalovirus infection after six months of liver transplantation in children in Shiraz, Southern Iran

Liver transplantation [LT] is a life-saving treatment for end-stage liver diseases [ESLD]. Cytomegalovirus [CMV] infection is one of the important causes of morbidity after LT. To evaluate the incidence of late-onset [after 6 months of LT] CMV infection in pediatric recipients. A retrospective analysis was conducted to evaluate 50 pediatric patients who underwent LT for 8 years at the LT Unit of Nemazee Hospital affiliated to Shiraz University of Medical Sciences, Shiraz, Iran. We retrospectively investigated episodes of CMV infection after 6 months of LT proven by CMV antigenemia test. Three recipients [6%] developed late-onset CMV infection. These patients finally responded to ganciclovir. CMV infection is one of the most common post-LT viral infections that usually occurs in the first six months of LT. Our study shows that the incidence of late-onset CMV infection is relatively low, but it still remains a significant problem. Therefore, monitoring and management is crucial for improving the survival of children

Liver transplantation and aortic valve replacement

Surgical procedures involving heart and liver are rare and have been limited to either combined heart and liver transplantation or coronary artery bypass graft surgery [CABG] or aortic valve surgery and orthotopic liver transplantation [OLT]. Aortic valve replacement [AVR] and pulmonary valve vegetectomy for bacterial endocarditis after OLT have also been reported. There are only five cases with aortic stenosis and cirrhosis reported to have combined AVR and liver transplantation. In the presence of cirrhosis, AVR has a significant risk for mortality because of bleeding from coagulopathy, renal failure, infection, and poor post-operative wound healing. Herein, we report on a case and management analysis of combined sequential AVR, and OLT in a 40-year-old cirrhotic man with Child and MELD score of C and 29, respectively. Echocardiography detected severe aortic insufficiency [AI] with enlarged left ventricle. Due to severe AI, the cardiologist recommended AVR prior to transplantation. The patient underwent metallic AVR. 4 months later, he received OLT. Both operations were successful and uneventful. Prioritizing AVR before OLT was successful in this patient. However, each patient must be evaluated individually and multiple factors should be assessed in pre-operation evaluation

Evaluation of candida infection after six months of transplantation in pediatric liver recipients in Iran

Liver transplantation [LT] is the standard treatment of end-stage liver diseases [ESLD]. Invasive fungal infection is one of the important causes of morbidity and mortality after transplantation. To determine the incidence of late-onset [after 6 months of LT] Candida infection in recipients. A retrospective study was conducted to evaluate 50 pediatric patients after LT for 8 years at the LT Unit of Nemazee Hospital affiliated to Shiraz University of Medical Sciences, Shiraz, Iran. We followed the patients until 6 months post-LT for episodes of Candida infection proven by culture. One recipient [2%] developed late-onset esophageal candidiasis with improvement after intravenous amphotricin therapy but finally expired with a diagnosis of post-transplant lymphoproliferative disorder [PTLD]. The incidence of late-onset Candida infection is not significant in pediatric liver recipient, but it still remains a significant problem. Control of Candida colonization would reduce the risk of invasive fungal infections and possibly more fatal complications

molecular and antigenic tissue impact of viral infections on liver transplant patients with neonatal hepatitis

Pathogenesis of neonatal hepatitis relates to various underlying causes including viral infections. Both hepatotropic and non-hepatotropic viruses may induce liver failures in infants before birth, during delivery, or shortly after birth. The tissue impact of HCMV, HSV, HBV, HCV, and rotavirus and adenovirus infections was evaluated in studied infants with neonatal hepatitis. The history of viral infections was analyzed in paraffin-embedded biopsy and autopsy tissues of 22 infants with neonatal hepatitis between years 1996 and 2007, retrospectively. The tissue molecular presentation of HBV, HCV, HCMV, HSV, adenovirus, and rotavirus was evaluated by different qualitative simple and nested PCR and RT-PCR protocols. Immunohistochemistry [IHC] method was used for studying the antigenic prevalence of HSV-1, 2; HBV, HCMV and adenovirus infections. Also the laboratory liver indices of all patients with neonatal hepatitis were analyzed. The HBV and HSV genomes were detected in 3 [14%] of 22 infants. The rotavirus and HCV-RNA and also the HCMV-DNA were detected separately in 1 [4%] of 26 paraffin-embedded autopsy and biopsy tissues. The HBV and HSV-1 specific antigens were separately diagnosed in 1 [4%] of 26 neonatal samples by IHC protocols. Also the HSV-2 antigen was seen in 5 [23%] of 22 liver autopsy and biopsy specimens. Co-infections with HCMV, HSV, HBV, HCV, and rotavirus were detected in these infants with hepatitis. Diagnosis of single and mixed molecular and antigenic traces of HCMV, HSV, HBV, HCV and rotavirus underlines the etiologic role of these viruses in clinical pathogenesis of neonatal hepatitis

Post-reperfusion syndrome and outcome variables after orthotopic liver transplantation

Post-reperfusion syndrome [PRS] is an important during liver transplantation. We studied the occurrence and severity of PRS in patients who underwent orthotopic liver transplantation [OLT] to investigate how PRS was correlated to clinical variables and outcomes. We retrospectively recorded intra- and peri-operative data for 184 adult patients who received cadaveric OLT during a 3-year period from 2005 to 2008. Patients were divided into two groups according to the severity of PRS: Group 1 [mild or no PRS] comprised 152 patients; and group 2 [significant PRS] consisted of 32 patients. There were no significant differences in demographic and pre-operative data between groups. Group 2 had more total blood loss than group 1 [p=0.036], especially after reperfusion [p=0.023]. Group 2 required more packed red cell transfusions [p=0.005], more fresh frozen plasma [p=0.003] and more platelets [p=0.043] than group 1. Fibrinolysis was more frequent in group 2 [p=0.004]. hospital stay in group 2 was significantly longer than in group 1 [p=0.034], but the frequencies of other outcomes including infection, re-transplantation, dialysis, rejection and extended donor criteria did not differ significantly between groups. Bleeding, blood transfusion and fibrinolysis occurred more often in the group of severe PRS after reperfusion. Although postoperative complications like rejection, infection and the dialysis rate were not significantly different in the two groups, hospital stay was more prolonged in the group with severe PRS

effect of simvastatin on lowering panel reactive antibody titer in sensitized dialysis patients: a randomized placebo controlled clinical trial

Patients with panel reactive antibodies [PRA] have many difficulties to find a crossmatch-nega- tive kidney for transplantation and are at a higher risk of post-transplantation rejection. To evaluate the effect of simvastatin on PRA and post-transplant outcome of these sensitized pa- tients. 82 patients with end-stage renal disease [ESRD] with a PRA >/= 25% were evaluated. In a one-year follow-up, the patients were treated with simvastatin. These patients were compared with 82 matched con- trols receiving placebo tablets. At the end of the second and 12th month, PRA was rechecked in all patients. Those patients who underwent transplantation continued to take simvastatin six months after transplanta- tion. Serum creatinine levels were checked at monthly intervals post-operation. The mean +/- SD PRA level at the end of the second month was 36.63% +/- 31.14% and 45.34% +/- 24.36% in cases and controls, respectively [P=0.012]. Seven patients in the case group and 10 in the control group were lost to follow-up. The remaining patients continued to take simvastatin for 12 month. The mean +/- SD PRA level at the end of the 12[th] month was 24.02% +/- 31.04% in cases and 43.15% +/- 26.56% in controls [P=0.001]. 25 patients underwent renal transplantation and continued to receive simvastatin 6 months after transplantation. These patients were matched with 25 controls treating with placebo. The mean +/- SD creatinine level 6 months after kidney transplantation was 2.05 +/- 1.14 mg/dL and 3.15 +/- 1.09 mg/ dL in cases and controls consecutively [P=0.02]. Simvastatin can be safely used to lower PRA and improve post-transplantation outcomes

Congenital hepatic fibrosis and need for liver transplantation

Herein, we describe two patients who underwent liver transplantation with the clinical diagnosis of hepatic failure and cryptogenic cirrhosis; histopathology of the explanted hepatectomy specimen revealed congeni- tal hepatic fibrosis. To the best of our knowledge, coexistence of hepatic failure and cirrhosis in congenital hepatic fibrosis, have not yet been reported in the English literature

Liver transplantation in the presence of old portal vein thrombosis

Portal vein thrombosis [PVT] has been mentioned as a potential obstacle to liver transplantation [LTx]. To review the impact of PVT on orthotopic liver transplant [OLT] outcome. Between January 2006 and April 2009, 440 OLT were performed in Shiraz Transplant Unit of whom, 35 [7.9%] cases had old PVT with recanalization. Data were retrospectively collected regarding the demograph- ics, indication for OLT, Child-Turgot-Pugh classification, pre-transplant diagnosis of PVT, perioperative course and managements, relapse of PVT, early post-operative mortality and morbidity. All patients received liver from deceased donors, underwent thrombendvenectomy with end-to-end anastomosis without interposition graft and evaluated daily for 5 days and thereafter, biweekly by duplex sonography during the follow-up period for 2 months. They were treated by therapeutic doses of heparin followed by warfarin to maintain an INR of 2-2.5. The causes of end-stage liver disease were hepatitis B in 11, cryptogenic cirrhosis in 11, primary scle- rosing cholangitis in 5 and other causes in 8 recipients. Extension of thrombosis was through confluence of superior mesenteric and splenic vein in 32 and to superior mesenteric vein in 3 patients. The mean +/- SD op- eration time was 7.2 +/- 1.5 hrs. The mean +/- SD transfusion requirement was 5.4 +/- 2.8 units of packed cells. The mean +/- SD duration of hospital stay in these patients was 17.7 +/- 10.9 days. Eight patients died; 1 developed early in-hospital PVT, 1 had hepatic vein thrombosis, and 1 died of in-hospital ischemic cerebrovascular ac- cident, despite a full anticoagulant therapy. The mean +/- SD follow-up period for those 28 patients discharged from hospital was 16.6 +/- 7.9 months; none of them developed relapse of PVT. The overall mortality and mor- bidity was 28% and 32%, respectively. There was no relapse of PVT in the other patients. The presence of PVT at the time of OLT is not a contraindication for the operation but those with PVT have a more difficult surgery, develop more postoperative complications, and experience a higher in-hospital mortality

Outcome and characteristics of patients on the liver transplant waiting list: Shiraz experience

The only curative therapy for end-stage liver disease is transplantation but due to a shortage of available donor livers the waiting list mortality is high. This study aimed to evaluate the outcome and characteristics of patients on the waiting list for liver transplantation in Shiraz, southern Iran during the period from April 2004 to March 2007. Medical records of all chronic liver disease patients >/= 14 years that were on the waiting list for liver transplantation at the Nemazee Hospital Organ Transplant Center during April 2004 to March 2007 were reviewed. Hospital records were used to retrieve demographic, clinical and laboratory data. Records of the referring gastroenterologists provided information about the etiology and complications of liver disease. The patients were followed at the end of the study period by clinic visits or telephone contact. There were 646 patients on the waiting list for liver transplant during April 2004 to March 2007. Hepatitis B was the most common etiology of liver disease [31.2%]. Of those on the waiting list, 144 patients 22.3%] underwent liver transplant and 166 [25.7%] died while waiting for a transplant. The mean waiting period for transplant was 6.6 months. Receiving a transplant was correlated with the etiology of liver disease and Rh blood group [p<0.05] but had no significant association with gender or ABO blood type. Among non-transplanted patients, survival was lower in those who had a history of encephalopathy, SBP or uncontrolled ascites and in patients with a Child-Turcotte-Puph [CTP] class C and/or a Model of End-stage Liver Disease [MELD] score >/= 15. Hepatitis B virus is the most common cause of end-stage chronic liver disease amongst patients on the waiting list for liver transplant in Shiraz, southern Iran. Patients with a MELD score >/= 15 particularly those with a history of SBP, hepatic encephalopathy or uncontrolled ascites are recommended for waiting list enrollment

Peri-operative high-dose V post-operative low dose steroid therapy in the management of biliary atresia: a preliminary report

The use of high-dose steroid therapy peri portoenterostomy may have a positive impact on the frequency of cholangitis and survival rate. A prospective study was conducted on two groups of patients [less than three months of age] suffering from biliary atresia from 1999 to 2005. The patients in group I [G I] were managed peri-operatively by high-dose methylprednisolone while the other group [G II] received low dose methylprednisolone only post-operatively [2mg/k/day for 1 month]. Infants in GI [n = 30] received methylprednisolone for 3 successive days before operation [10-8-6mg/kg/day], and 10 mg/k at the day of operation respectively. Thereafter the dose was tapered in the next successive 6 days by 8, 6, 5, 4, 3, and 2 mg/kg/day and continued for one month. Seventy two infants with biliary atresia were operated [39 girls and 33 boys]. Twenty-six of the 30 patients [86%] in G I became jaundice-free within 90 days after portoenterostomy while only seven [15%] of the 42 patients in G II had normal bilirubin [P < 0.0001]. Episodes of postoperative cholangitis in G I were 20% [6 of 30], and 53% [24 of 42] in G II [P < 0.005]. The difference in 3-year survival rate between the two groups is also remarkable: Eighty seven percent [26 of 30] in GI versus 29% [13 of 45] in G II [P < 0.005]. Death related to biliary atresia occurred in 1 [3.3%] patient in GI compared with 12 [29%] patients in G II [p < 0.005]. These results provide strong evidence that peri-operative high dose steroid therapy is not only safe in this patients population, but because of its anti-inflammatory and cholerrhetic effects has a positive impact on preventing recurrent cholangitis, and ultimately survival

Living-related liver trasplantation in children: the Shiraz experience

The liver transplantation program was established at Shiraz Nemazee Hospital in 1993. Shortage of cadaver organ supply due to various social and legal issues urged us to develop a living-related liver transplantation [LRLT] program. So far 7 [6 males, 1 female] living-related liver transplantations have been performed at this center. The mean age of patients was 8.21 years [ +/- 4.16], with a range of 4.5 to 14 years. Live donors [3 mothers, 2 fathers, and 1 brother] with a mean age of 30.83 years [ +/- 4.11] underwent procurement of the left lateral segment without mortality or any serious morbidity. The native liver disease was idiopathic cirrhosis [1 case], biliary atresia [3 cases], Budd-Chiari syndrome [1 case], neonatal cirrhosis [1 case], and cryptogenic cirrhosis [1 case]. Allografts were implanted using piggy-back surgical technique. Baseline immunosuppression consisted of a triple drug regimen including cyclosporine, mycophenolate mofetil and steroids. Acute graft rejection was treated with intra-venous bolus of methyl-prednisolone. Early death occurred in 2 patients due to vascular thrombosis. Biliary complication was observed in 1 patient. Five patients are alive with a normal functioning liver. In conclusion, LRLT program is promising in Iran. It can help to overcome the shortage of organs and minimize the mortality of patients in the waiting list

Abdominal wall abscess caused by enterobius vermicularis

A seven-year-old boy presented with abdominal wall abscess due to Enterobius vermicularis. Gastro-intestinal tract and overlying skin were normal. This report is to our knowledge the first abdominal wall abscess due to oxyuriasis. Possible hematogenous mode of involvement of the liver and lung may further be supported by the present case

Modified duhamel operation using a newly designed crushing clamp

Using a newly-designed crushing clamp, modified Duhamel Operation [MDO] was performed in 15 children aged between 2 to 8 years with Hirschsprung's disease. Post-operative recovery was uneventful in all patients. No clamp-related complication was observed. Complete bed rest was not necessary for more than 3-4 days. The clamp was passed spontaneously 8-11 days after operation. Endoscopy 3-4 weeks later did not reveal any colo-rectal septum in the neo-rectum in any patient. Performing MDO using this instrument is convenient to the patient. More importantly, with equal efficacy, it is more cost-effective as compared to the currently used GIA stapler