Proximal airway obstruction caused by adenoid cystic carcinoma in a pregnant woman: A case report.

Respiratory tumours are very rare during pregnancy. Here, we report a case of a primigravida woman diagnosed at 27 weeks' gestation with stridor and dyspnoea, and a primary tracheal tumour. To the best of our knowledge, this is a 4th case report of tracheal adenoid cystic carcinoma during pregnancy. Our staged management of airway obstruction during pregnancy and definite treatment are discussed.

Comparison of Child-Turcotte-Pugh and pediatric end-stage liver disease scoring systems to predict morbidity and mortality of children awaiting liver transplantation.

BACKGROUND: The pediatric end-stage liver disease (PELD) scoring system has been used widely for prioritizing children awaiting orthotopic liver transplantation (OLT). The aim of the present study was to compare the Child-Turcotte-Pugh scoring system with PELD to predict morbidity and mortality of children scheduled for OLT before the organ was available. MATERIALS AND METHODS: From 1999 to 2006, 83 infants and children were evaluated and scheduled for OLT. Child and PELD scores were determined according to the initial assessment at the time of listing. Outcome was examined using records and follow-up data. RESULTS: Among 83 patients, 12% were Child A; 53%, Child B; and 35%, Child C. The mean PELD score at listing was 19.8+/-12.8. Patients with Child scores A, B, and C displayed mean PELD scores of 7.1+/-4.9, 15.7+/-9.3, and 30.5+/-11.7, respectively. Child classification and scoring showed a positive correlation with the PELD score (Spearman's correlation coefficient: 0.666, P=.001). A higher PELD score was associated with greater morbidity and mortality. CONCLUSION: Child classification has several shortcomings; therefore, PELD scores appear to be the best metric to prioritize children listed for OLT.

Transplantation of a cadaveric liver allograft with right lobe cavernous hemangioma, without back-table resection: a case report.

UNLABELLED: The use of extended criteria liver donors has become a necessity in an era of organ scarcity for transplantation. We present here a case report of orthotopic liver transplantation using a liver with a giant right lobe hemangioma without backtable resection. CASE REPORT: There were no data regarding the liver mass before organ procurement. The donor liver function tests and electrolyte profile were normal. During donor exploration a hemangioma was identified in segments V-VI, occupying approximately 20% of the total liver volume. It was prepared for transplantation on a sterile backtable without performing backtable hemangioma resection. A standard orthotropic liver transplant procedure was performed uneventfully, without veno-veno bypass. There was no bleeding from the hemangioma. The ischemic time was 9 hours and 20 minutes. Postoperative course was uneventful and the patient was discharged at 19 days after the operation. The hemangiomas showed evolution with some decrease in size upon later follow-ups. No clinically important complication was observed. CONCLUSION: Our case and other previous reports show that even large hemangiomas should not be considered to be a contraindication to organ procurement. These benign lesions either could be left in situ and observed or resected.

Are large nonfunctional kidneys risk factors for posttransplantation urinary tract infection in patients with end-stage renal disease due to autosomal dominant polycystic kidney disease?

OBJECTIVES: The objective of this study was to evaluate the effect of bilateral nephrectomy on posttransplantation urinary tract infection (UTI) among patients with end-stage renal disease (ESRD) due to autosomal dominant polycystic kidney disease (ADPKD). METHODS: In a retrospective case-control design, 62 patients with ESRD with ADPKD were divided into 2 groups: (A) 24 patients who underwent bilateral nephrectomies, and (B) 38 patients in whom bilateral nephrectomies had not been done. Pretransplantation and posttransplantation urine cultures were evaluated for UTI. RESULTS: Sixty-two patients with ESRD with ADPKD were enrolled in this study. The average age was 42 years (range, 6-60 years). Forty patients (64.5%) were male and 22 (35.5%) were female. The mean duration of hemodialysis was 24 months (range, 2-120 months), which was the same for both groups. Bilateral nephrectomies were done for 24 participants (38.7%). There were 38 patients (61.3%) in group B who did not have the operation. UTI occurred in 23 patients (37.1%): 6 patients (25%) in group A and 17 patients (44.7%) in group B. The incidence of UTI was not statistically different between the 2 groups (P>.05). Furthermore, no relationship was found between age, gender, blood group, and UTI in patients with ADPKD (P>.05). CONCLUSION: According to our study, the presence of large nonfunctional kidneys is not a risk factor for posttransplantation UTI in patients with ADPKD and ESRD.

Early hepatic artery thrombosis after liver transplantation: diagnosis and treatment.

BACKGROUND: Hepatic artery thrombosis (HAT) occurs in 3% to 9% of all liver transplantations with acute graft failure as a possible sequel. METHODS: Eleven episodes of HAT were identified among 256 orthotropic liver transplantations (whole, LDCT, split) performed on 253 patients between April 1993 and July 2006. HAT was suspected clinically and confirmed by Doppler ultrasonography, magnetic resonance angiography, angiography, or reexploration. One patient was excluded due to poor follow-up. Treatment options included exploration with HA thrombectomy plus thrombolysis, retransplantation, or conservative treatment of hepatic and biliary complications. RESULTS: Among 11 patients of mean age 29.98 +/- 17.14 years (range, 10 months to 56 years). 2 had split right lobe liver transplantations and 9 received whole organs. None of LDLTs were identified to have HAT. The causes of liver cirrhosis among HAT patients were autoimmune hepatitis (n=3), cryptogenic (n=3), Wilson (n=1), PBC (n=1), biliary atresia (n=1), and HBs (n=1). HAT was diagnosed at 5.9 +/- 4.43 (range, 2 to 16) days after operation. Most patients developed right upper quadrant (RUQ) pain at presentation. Two patients developed acidosis, fever, or SIRS and underwent retransplantation. Four underwent exploration of HA and 1 was treated conservatively. Three cases expired due to HAT complications. CONCLUSION: We found RUQ pain to be the presenting sign of early HAT in majority of cases. RUQ pain has been reported to occur in late HAT. Whenever HAT is confirmed, liver transplanted patients should be revascularized or even retransplanted. Intra-arterial thrombolysis and thrombolytic therapy for HAT should be done cautiously due to the potential risk of hemorrhage.

The effect of clamping of inferior vena cava and portal vein on urine output during liver transplantation.

BACKGROUND: Intraoperative hypotension, massive transfusion, liver disease, coexistent renal dysfunction, and decreased glomerular filtration rate during the anhepatic phase are major hazards for kidney function. We undertook this study to determine the change in urine output during clamping. METHOD: Twenty-four patients without preexistent renal disease, who were undergoing liver transplantation using the piggyback method, were enrolled in this study. Patients with a serum creatinine level >1.2 mg/dL were excluded. Urine output was monitored over 30 minutes before inferior vena cava and portal vein clamping, during clamping, and for 30 minutes after declamping. None of the patients had a clamping time >70 minutes. Our goal was to maintain mean arterial blood pressure and heart rate just by fluid administration diuretics were avoided. RESULTS: Participants had a mean age of 39.12 +/- 13.52 years (range, 15-67 years) with a male to female ratio of 1:4. Urine output 30 minutes before clamping was 3.64 +/- 3.58 (range, 1.25-15.18) mL/kg/h, decreased to 1.28 +/- 2.58 (range, 0-11.39) mL/kg/h during clamping (P=.00), and increased to 3.56 +/- 3.64 (range, 0.51-15.18) mL/kg/h 30 minutes after declamping (P=.00). CONCLUSION: Urine output was significantly reduced in all patients after clamping of the IVC and portal veins. This observation may be explained by increased venous pressure leading to decreased renal perfusion pressure. It has been stated that one of the advantages of veno-veno bypass (VVB) is increased renal perfusion pressure. However, if the clamping time in the piggyback method is <70 minutes and patients have normal preoperative renal function, the decreased renal perfusion pressure will not cause postoperative kidney dysfunction.

Coexistence of aspergilloma and pulmonary hydatid cyst in an immunocompetent individual.

Echinococcosis is a zoonotic disease caused by Echinococcus granulosus sensu lato. The liver and lungs are the most commonly sites of infections, but involvements of other organs were also observed. Recently, the coinfection of pulmonary hydatid cyst with aspergilloma has been reported in the literature. Herein, we report a successful treatment of coinfection of cystic echinoccosis with aspergilloma due to Aspergillus flavus in a 34-year-old female. In vitro antifungal susceptibility tests revealed that the MIC values for antifungals employed in this case were posaconazole (0.031µg/ml), itraconazole (0.125µg/ml), voriconazole (0.25µg/ml), and amphotericin B (1µg/ml). The minimum effective concentration for caspofungin was 0.008µg/ml. This coexistence of active pulmonary echinococcosis and aspergillosis is being reported because of its rarity and clinical importance for its management.

Synthesis and biological evaluation of purine-containing butenolides.

6-Chloropurine derivatives of gamma-(Z)-ethylidene-2,3-dimethoxybutenolide 3a, gamma-(Z)-ethylidene-2-methoxy-3-(4-nitro)benzyloxybutenolide 3b, gamma-(Z)-ethylidene-2-(4-nitro)benzyloxy-3-methoxybutenolide 3c, gamma-(Z)-ethylidene-2,3-di(4-nitro)benzyloxybutenolide 3d, and dimethylphosphono-gamma-(Z)-ethylidene-2,3-dimethoxybutenolide 11 as well as the adenine derivative of gamma-(Z)-ethylidene-2,3-dimethoxybutenolide 6 were synthesized. The key steps in the high-yield synthesis of 6 involved hydration/dehydration of the C(4)=C(5) in the precursor 3a. In the presence of NH4OH at elevated temperature, 3a underwent a reverse Michael-type addition with water to produce hydrate 5. At 37 degrees C, 6 was also hydrated in the presence of S-adenosyl-L-homocysteine hydrolase to afford 5. Butenolide 6 exhibited an inhibitory property toward the enzyme. Such type II (enzyme-mediated addition of water across C(4)=C(5)) mechanism is the first example of "enzyme-substrate intermediate" inactivation of S-adenosyl-L-homocysteine hydrolase. In contrast with type I mechanism-based inactivation, reduction of enzyme-bound NADP(+) to NADPH was not observed. Upon treatment with HCl, stereoselective dehydration of 5 occurred to give the target molecule 6. At ambident temperature, 3a was hydrated in the presence of NH4OH or pig liver esterase to produce 6-chloropurine derivative 4. An unambiguous proof of the structures of 3-5 was obtained by X-ray crystallographic analysis. For the synthesis of phosphonate derivative 11, the key step involved chlorination of phosphonate 9 by use of CF3SO2Cl and 1,8-diazabicyclo[5.4.0]undec-7-ene in CH2Cl2. 6-Chloropurine-containing butenolide 3d, 6-chloropurine derivative of 4-hydroxybutenolide 4, and adenine-containing 4-hydroxybutenolide 5 did not show anticancer and antiviral activities. 6-Chloropurine-containing ethylidene-2,3-dialkoxybutenolides 3a-c and phosphonate 11, however, exhibited inhibitory activity against murine leukemias (L1210 and P388), breast carcinoma (MCF7), and human T-lymphoblasts (Molt4/C8 and CEM/0) cell lines. They were also notably active toward thymidine kinase-deficient varicella-zoster virus (TK(-)VZV). Adenine-containing ethylidene-2,3-dimethoxybutenolide 6 exhibited marked selectivity in cytostatic activity against the murine leukemia (P388) cell line.

Design, synthesis, and biological evaluation of novel nucleoside and nucleotide analogues as agents against DNA viruses and/or retroviruses.

A novel strategy was developed for the synthesis of N(7)-purine acyclic nucleosides 9 and 14. The key step involved the reaction between [2-(p-methoxyphenyloxy)ethoxy]methyl chloride and N(9)-tritylated nucleobases 6 or 11 followed by concomitant self-detritylation. N(7)-Guanine acyclic nucleoside 9 exhibited antiviral activity, but was phosphorylated by both HSV and Vero cell thymidine kinases. Thus, it showed more potent cellular toxicity than acyclovir (2). N(7)-Adenine acyclic nucleoside 14 was found to be an excellent antiviral agent as well as a good inhibitor of calf mucosal adenosine deaminase. This inhibitory property allows for a greater expression of antiviral activity of antiviral agents, such as N(9)-adenine acyclic nucleoside 1 and ara-A (3). Compound 14 was phosphorylated neither by herpes simplex virus (HSV) thymidine kinase nor by Vero cell thymidine kinase, yet it enhanced the rate constant for the monophosphorylation of acyclovir (2) by HSV thymidine kinase. Consequently, the combination of acyclovir (2) and 14 exhibited greater antiviral activity than acyclovir alone. 7-[2-(Phosphonomethoxy)ethyl]adenine (20) was also synthesized. The key step involved the reaction of 9-(2-cyanoethyl)adenine (15) with methyl iodoacetate in the presence of lithium 2,2,6,6-tetramethylpiperidine in THF. Unlike 9-[2-(phosphonomethoxy)ethyl]adenine (PMEA, 4), the N(7)-isomer 20 was not phosphorylated effectively by 5-phosphoribosyl 1-pyrophosphate synthetase (PRPP synthetase). Thus, it did not exhibit pronounced antiviral activity. Dinucleotide 5'-monophosphate 24 and its butenolide ester 25 were also synthesized. Compound 24 showed substrate activity toward PRPP synthetase and exhibited notable activity against DNA viruses. The antiviral activity of the ester derivative 25 was found to be higher than that of the parent molecule 24. Dinucleotide 5'-monophosphate 24 is susceptible to degradation by snake venom and spleen phosphodiesterases. However, its respective butenolide ester derivative 25 was completely resistant to snake venom and spleen enzymes. Butenolide ester derivatives 28 and 29 were also synthesized and exhibited notable anti-DNA virus and anti-retrovirus activity in vitro. Compounds 2, 4, 9, 14, 20, 24, 25, and 28 were also evaluated for their inhibitory effect on HSV-1-induced mortality in NMRI mice. N(7)-adenine acyclic nucleoside 14 [LD(50) (intraperitoneal, ip) 950 mg/kg], nucleotide-containing butenolide 25 [LD(50) (ip) 675 mg/kg], and butenolide 28 [LD(50) (ip) 710 mg/kg] were found to be potent anti-HSV-1 agents in vivo. In addition, butenolide 28 efficiently decreased tumor formation induced by Moloney murine sarcoma virus (MSV) in NMRI mice while significantly increasing the survival time of MSV-infected mice.

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.

Morbidity and mortality of children with chronic liver diseases who were listed for liver transplantation in Iran.

Liver transplantation is the treatment of choice for end-stage liver disease in children, but donor shortage is still a main problem in this age group. The aim of the present study is to evaluate the complications and mortality of liver disease in children waiting for transplantation. We analyzed medical records of 83 children aged <18 yr, who were listed for liver transplantation but the organ was not available for them between 1999 and 2006. The outcome was assessed from their records or follow-up data. Among the children (mean age, 8 +/- 5 yr; 50.5% boys) listed for liver transplantation, but the organ was not available for them, the common causes of cirrhosis were biliary atresia (27.7%) and cryptogenic (24.1%). The mean follow-up duration was 14 +/- 13.4 months (range 0.5-54 months). Sixty-seven (80.7%) patients developed one or more complications while awaiting transplantation. The most common complications were gastrointestinal bleeding (44.6%), spontaneous bacterial peritonitis (36.1%), infectious complications (28.9%), encephalopathy (24.1%), renal (18.1%), and pulmonary problems (10.8%). Fifty-one (61.4%) patients needed hospital admission because of complications and 26 (31.3%) patients died while awaiting transplantation. About two-thirds of children listed for liver transplantation needed hospital admission because of complications and one-third of them died without any liver transplantation. It seems that more split liver transplantation as well as the introduction of a live-related program in our center will provide many benefits to our children.