Role of contrast-enhanced ultrasound in the evaluation of post-liver transplant vasculature.

Liver transplantation is a frequently used treatment for patients with end-stage liver disease and ultrasound is often the first-line imaging technique for detection of vascular complications after liver transplant. Although colour Doppler ultrasound is a good screening method for evaluation of post-liver transplant vasculature, it has limitations in evaluating small-calibre vessels and vessels in close proximity. Contrast-enhanced ultrasound (CEUS) has been proposed to overcome these limitations by improving visualisation of post-liver transplant vasculature and reducing the number of false-positive cases, which necessitate unnecessary additional investigations such as computed tomography or angiography. Liver transplant anatomy and the wide array of post-transplant imaging findings on colour Doppler have already been well described but literature on the use of CEUS and its image interpretation remain scarce. This review aims to discuss the indications for CEUS after liver transplant, to demonstrate CEUS technique and familiarise readers with the imaging appearances of post-transplant vascular complications on CEUS.

Mild to Moderate Intrapulmonary Shunting in Pediatric Liver Transplantation: Is Screening Necessary?

BACKGROUND: Despite reported associations between intrapulmonary vascular shunting (IPVS) and morbidity and mortality in pediatric liver transplantation (LT), there are no guidelines for screening. OBJECTIVE: To investigate IPVS before and after pediatric LT. METHODS: Retrospective records review of all pediatric LT (n = 370) from 2005 to 2015 at a single institute in Japan. All children with cirrhosis and clinical suspicion of IPVS without cardiac or pulmonary conditions were included. 99mTechnetium labelled macroaggregated albumin (99mTcMAA) scans were performed before and after LT. The severity of IPVS was graded using shunt ratios. RESULTS: Twenty-four children fulfilled inclusion criteria and underwent Tc99MAA scans. All revealed mild (<20%) to moderate (20%-40%) grades of IPVS. Following LT, the mean shunt ratio regressed from 20.69 ± 6.26% to 15.1 ± 3.4% (P = .06). The median (range) follow-up was 17 (4-85) months. Mortality was zero. The incidence of portal vein thrombosis (4.2%) biliary strictures (12.5%) and graft loss (4.1%) in the study group was not statistically significant compared to the remainder of the 370 transplants (3.2%, 9.4% and 3%, respectively). Sub-group analysis revealed hepatopulmonary syndrome (HPS) in 2 out of 24 children. The mean shunt ratios before and after LT were 39.2 ± 0.77% and 16.2 ± 8.5%, respectively (P = .08). There was 1 complication (intra-abdominal abscess). CONCLUSIONS: HPS is less likely in mild to moderate IPVS. LT may achieve comparable results when performed in the presence of mild to moderate IPVS.

Ileal Cystic Lymphangioma presenting with Acute Appendicitis.

Mesenteric lymphatic malformations are rare benign tumors that are most commonly found in children. The presentation of these tumors can be variable. It has been suggested that mesenteric lymphatic malformations are congenital; however, there is evidence that their size may be increased by infection. A 3-year 10-month-old boy presented with rhinorrhea and cough followed by acute abdominal pain. Ultrasonography revealed a lobulated mass in the lower abdomen. Computerized tomography scan of the abdomen diagnosed an inflamed appendix with perforation. Laparotomy revealed a multicystic mass within the mesentery closely adherent to the ileum; with omentum wrapped around. The appendix was dilated and firm. An ileal resection and an appendicectomy were performed. Histology confirmed acute transmural appendicitis with concurrent cystic lymphangioma of the ileal mesentery. Although rare, cystic lymphangioma must be considered in the differential of pediatric acute abdomen. Surgeons must be aware of this association and be reminded to inspect the rest of small bowel during appendicectomy especially if the preoperative imaging is suspicious.

Total internal biliary diversion during liver transplantation for type 1 progressive familial intrahepatic cholestasis: a novel approach.

LT for PFIC type 1 is often complicated by postoperative diarrhea and recurrent graft steatosis. A 26-month-old female child with cholestatic jaundice, pruritus, diarrhea, and growth retardation revealed total bilirubin 9.1 mg/dL, gamma-glutamyl transpeptidase 64 IU/L, and TBA 295.8 µmol/L. Genetic analysis confirmed ATP8B1 defects. A LT (segment 2, 3 graft) from the heterozygous father was performed. Biliary diversion was performed by a 35-cm jejunum conduit between the graft hepatic duct and the mid-transverse colon. Stools became pigmented immediately. Follow-up at 138 days revealed resolution of jaundice and pruritus and soft-to-hard stools (6-8 daily). Radioisotope hepato-biliary scintigraphy (days 26, 68, and 139) confirmed unobstructed bile drainage into the colon (t1/2 34, 27, and 19 minutes, respectively). Contrast meal follow-through at day 62 confirmed the absence of any colo-jejuno-hepatic reflux. At 140 days, contrast follow-through via the biliary stent revealed patent jejuno-colonic anastomosis and satisfactory transit. Graft biopsy at LT, 138 days, and 9 months follow-up revealed comparable grades of macrovesicular steatosis (<20%). TIBD during LT may be a clinically effective stoma-free biliary diversion and may prevent recurrent graft steatosis following LT for PFIC type 1.

Regulation and therapeutic strategies of 4-hydroxy-2-nonenal metabolism in heart disease.

4-Hydroxy-2-nonenal (4-HNE), a reactive aldehyde, is generated from polyunsaturated fatty acids (PUFAs) in biological membranes. Reactive oxygen species (ROS) generated during oxidative stress react with PUFAs to form aldehydes like 4-HNE, which inactivates proteins and DNA by forming hybrid covalent chemical addition compounds called adducts. The ensuing chain reaction results in cellular dysfunction and tissue damage. It includes a wide spectrum of events ranging from electron transport chain dysfunction to apoptosis. In addition, 4-HNE directly depresses contractile function, enhances ROS formation, modulates cell signaling pathways, and can contribute to many cardiovascular diseases, including atherosclerosis, myocardial ischemia-reperfusion injury, heart failure, and cardiomyopathy. Therefore, targeting 4-HNE could help reverse these pathologies. This review will focus on 4-HNE generation, the role of 4-HNE in cardiovascular diseases, cellular targets (especially mitochondria), processes and mechanisms for 4-HNE-induced toxicity, regulation of 4-HNE metabolism, and finally strategies for developing potential therapies for cardiovascular disease by attenuating 4-HNEinduced toxicity.

Structure and microhardness of cu-ta joints produced by explosive welding.

The structure and microhardness of Cu-Ta joints produced by explosive welding were studied. It was found that, during explosive welding, an intermediate layer 20⋯40 µ m thick with a finely dispersed heterophase structure, formed between the welded copper and tantalum plates. The structure of the layer was studied by scanning and transmission electron microscopy. Microvolumes with tantalum particles distributed in a copper matrix and microvolumes of copper particles in a tantalum matrix were detected. The tantalum particles in copper have a size of 5⋯500 nm, with a predominance of 5⋯50 nm particles. A mechanism for the formation of the finely dispersed heterophase structure in explosive welding is proposed. The microhardness of interlayers with the heterophase structure reaches 280 HV, which far exceeds the microhardness of copper (~130 HV) and tantalum (~160 HV). Many twins of deformation origin were found in the structure of the copper plate. The effect of heating temperature in the range from 100 to 900°C on the microhardness of copper, tantalum, and the Cu-Ta welded joint was studied. Upon heating to 900°C, the microhardness of the intermediate layer decreases from 280 to 150 HV. The reduction in the strength properties of the weld material is mainly due to structural transformations in copper.

Vascular complications in pediatric liver transplantation; single-center experience from Singapore.

AIM: Vascular complications (VC) are a major cause of significant morbidity and mortality in pediatric liver transplantation (LT). We reviewed our series to study the evolution of vascular reconstructions and its effect on the incidence of VC after LT, particularly with regard to the portal vein (PV). METHODS: The medical records of 81 pediatric LT performed in 76 children (38 boys) from 1991 to 2010 in the National University Hospital, Singapore, were reviewed to identify VC pertaining to PV, hepatic artery (HA), and hepatic veins (HV) and to analyse the data for the entire series and in 2 consecutive cohorts: initial 40 LT (group 1) and subsequent 41 LT (group 2). Specific interventions in group 2 were characterized by surgical innovations for reconstruction of the difficult PV and routine use of Doppler ultrasound intraoperatively and postoperatively. RESULTS: The overall incidence of VC was 19.7% (n = 16) and individually HA thrombosis 4.9% (n = 4), HA stenosis 1.2% (n = 1), PV thrombosis 12.3% (n = 9), PV stenosis 1.2% (n = 1), and HV thrombosis 1.2% (n = 1). The overall 1- and 5-year survival rates in our series were 89% and 85%, respectively. The 1- and 5-year survival rates in patients with and without VC were 81.25% and 68.75% and 90.8% and 89.2%, respectively. The incidence of VC decreased from 27.5% in group 1 to 12.1% in group 2 (p = .08). The major contribution to this appears to be a decrease in PV complications from 17.5% in group 1 to 7.3% in group 2 (P = .1). The incidence of HA (3 vs 2) and HV (1 vs 0) complications was similar between the 2 groups. CONCLUSIONS: Vascular reconstructions in small recipients are technically challenging and associated with a learning curve. Application of meticulous techniques in general, surgical innovations to the difficult PV in particular and attention to postoperative monitoring contribute toward a major reduction in VC.

Unusual shunt for symptomatic portal vein thrombosis after liver transplantation - Clatworthy revisited.

PV thrombosis is not an uncommon occurrence following pediatric LT. Symptomatic PHT following PV thrombosis is treated medically, surgical portosystemic shunting (mesorex, splenorenal, and mesocaval) being reserved for refractory cases. A 10-yr-old boy suffered recurrent malena and hemorrhagic shock because of chronic PV thrombosis following LT nine yr ago (1999). Extensive work-up failed to localize the bleeding source. The liver function remained normal. Initial attempts at surgical shunts failed owing to thrombosis (mesocaval 2001, splenorenal, inferior mesenteric-left renal vein, splenic-left external iliac vein 2008). In this situation, we performed a Clatworthy shunt by anastomosing the divided lower end of the LCIV to the side of SMV. There was a single, large caliber anastomosis. Post-operatively, the malena stopped completely, and clinically, there was no lower limb edema or encephalopathy. Doppler USG revealed persistence of hepatopetal flow within the portal collaterals. Follow-up at two yr reveals stable hepatic function with a patent shunt. To the best of our knowledge, we are not aware of a Clatworthy shunt being performed in a transplant setting. We reviewed the literature pertaining to this shunt in non-transplant patients with PHT.

Inflammatory tumour of the retroperitoneum--a case report.

INTRODUCTION: Neoplastic growths of myofibroblasts occurring on a background of plasma cell and lymphocytic proliferation have been designated as inflammatory myofibroblastic tumours (IMTs). These unusual tumours were first described in pulmonary location in adults. Though extrapulmonary masses have been reported in children; retroperitoneal growths are exceedingly rare. We report a case of retroperitoneal IMT that presented with constitutional symptoms without any palpable abdominal mass. CLINICAL PICTURE: A previously well 12-year-old boy presented with fever, right-sided flank pain and weight loss of 1-month duration. There were no foci of infection. The erythrocyte sedimentation rate (ESR) was raised; the white cell count was normal. An abdominal computed tomography (CT) scan revealed a right suprarenal tumour measuring 3.5 cm without any calcification. The urinary catecholamines and vanilmandelic acid were normal. TREATMENT: A laparotomy with complete excision of the tumour was performed. Final histology revealed an inflammatory myofibroblastic tumour without any correlates of aggressive behaviour. OUTCOME: Postoperatively, the constitutional symptoms of fever, weight loss and raised ESR normalised. Follow-up CT was normal and further treatment was not necessary. CONCLUSION: Although rare, IMTs should be considered in any abdominal solid tumour with associated constitutional and laboratory features of an inflammatory response. Complete surgical excision is effective treatment for biologically benign tumours.

Blunt renal trauma in occult congenital hydronephrosis.

Since the routine use of antenatal ultrasonography, congenital pelviureteric junction (PUJ) obstruction rarely presents as an incidental diagnosis following renal trauma. Gross haematuria following seemingly trivial trauma should arouse the suspicion of a pre-existing abnormality. We report a 14-year-old girl with previously asymptomatic and undetected PUJ obstruction who first presented with gross haematuria following a trivial fall. The diagnosis was established by an abdominal computed tomography that showed left chronic hydronephrosis, and was subsequently confirmed on a renal isotope scan. Since the pre-existing hydronephrosis has little impact on the recovery from the injury itself, the initial management was non-operative with an aim to conserve the kidney. As standard surgery has a good outcome, once the renal scan confirmed good renal function, a delayed Anderson-Hynes' pyeloplasty was performed following healing of the kidney laceration as seen on ultrasonography.

Chemistry and biology of diazonamide A: second total synthesis and biological investigations.

As an especially unique target for chemical synthesis, diazonamide A has the potential to be constructed through a plethora of synthetic routes, each attended by different challenges and opportunities for discovery. In this article, we detail our second total synthesis of diazonamide A through a sequence entirely distinct from that employed in our first campaign, one whose success required the development of several special strategies and tactics. We also disclose our complete studies regarding the chemical biology of diazonamide A and its structural congeners, and more fully delineate the scope of our protocol for Robinson-Gabriel cyclodehydration using pyridine-buffered POCl(3).

Management of ventral hernia after giant exomphalos with external pressure compression using helmet device.

PURPOSE: The aim of this study was to evaluate an alternative technique of reducing a ventral hernia that follows the primary conservative treatment of a giant omphalocoele. METHODS: The patient is a full-term male neonate with a giant exomphalos. Initially triple dye was applied as an eschar-inducing agent. This resulted in a ventral hernia after 1 month. It was decided to achieve expansion of the abdominal cavity based on the principle of external pressure compression using a sphygmomanometer cuff over the hernia. The cuff was worn continuously, and manual pressure was applied daily. Care was taken to avoid intraabdominal hypertension using the reading of the manometer that was attached. The external pressure was corroborated with observations of respiration and circulation. RESULTS: The child did not show any ill effects of raised intraabdominal pressure. Throughout the treatment, the child was on full oral feedings and did not require any ventilator support. Reduction of the ventral hernia was achieved in 9 months. Surgical repair of the residual hernia defect was carried out by double breasting of the fascia. CONCLUSIONS: The application of controlled external pressure using a specially constructed device is a safe, noninvasive, and effective method of achieving reduction of a ventral hernia after primary conservative treatment of a giant omphalocoele.

Studies toward diazonamide A: development of a hetero-pinacol macrocyclization cascade for the construction of the bis-macrocyclic framework of the originally proposed structure.

In this article, we describe further studies toward the originally proposed structure of diazonamide A (1). After confronting a number of failures in synthesizing the heterocyclic core of that structure, success was finally realized through the development of a novel hetero-pinacol-based macrocyclization cascade sequence. Subsequent elaboration led to an advanced compound bearing both of the 12-membered rings of the target molecule. In addition, preliminary biological studies with intermediates and simplified analogues obtained via the developed sequences are also described.

Anorectal malformation with congenital absence of vagina: a case report and review of the literature.

Congenital anorectal malformations are relatively common, and associated genitourinary malformations have been reported in up to 40% of patients. Uterovaginal malformations are also not rare in children. They are known to occur with increased frequency in children with anorectal malformations, but the diagnosis may still be difficult. We report herein one such case of anorectal malformation associated with congenital total absence of the vagina in which the uterovaginal malformation was not diagnosed until the operative repair of the anorectal malformation. The operative procedure was thereafter directed, along with the anorectoplasty, towards restoring a functional uterovaginal tract. Review of the literature revealed that such a diagnosis is unsuspected or delayed in more than half of affected patients. Furthermore, these patients present with many diagnostic and therapeutic problems. Our report highlights the need to be aware of this condition to allow for an earlier diagnosis and appropriate operative treatment.

Meckel's diverticulum: an alternative conduit for the Mitrofanoff procedure.

The Mitrofanoff procedure is a versatile technique that is successfully used in achieving continent urinary diversion for a wide variety of urological conditions. Appendix and usable segment of ureter are commonly employed for this purpose and provide desirable results. This communication describes a teenage girl with lumbosacral agenesis and neurogenic bladder in whom Meckel's diverticulum was successfully used for the Mitrofanoff procedure.

An Immunity (QL3) Breaking Strain of Sorghum Mosaic Potyvirus in India.

Sorghum (Sorghum bicolor) line Tx2786 is immune to strains or isolates of sorghum mosaic potyvirus (SrMV strains SCH and SCH) but susceptible to strains of maize dwarf mosaic (MDMV) and sugarcane mosaic (SCMV) potyviruses (1,2). When grown in proximity to sugarcane, Tx2786 was infected with a virus that caused mosaic and necrotic symptoms during the postrainy season of 1994 (14 to 30°C) at Parbhani (Maharashtra, India). Leaf-dip electron microscopy performed on virus-infected sorghum and sugarcane tissues revealed particles, 720 × 12 nm, typical of a potyvirus. A potyvirus isolate (designated IBS) was readily transmitted mechanically from field-infected sorghum (Tx2786) and sugarcane (Saccharum officinarum cv. Co.740) to 18 glasshouse-grown sorghum inbred lines. All sorghum lines (QL-3 from India and Tx2786), including those resistant to SrMV, SCMV, MDMV, and johnsongrass mosaic potyvirus (JGMV), were infected. IBS caused necrosis in Cargill-40, Hegari, PI-35038, RTx09, Tx2786, QL-3 from India, and Redlan lines and mosaic symptoms in RTx430, SA-394, Atlas, Martin, BTx3048, Caprock, NM-31, QL-11, SCO-175-14E, Rio, and BTx623 lines. Sorghum reactions to sorghum and sugarcane virus isolates were similar. IBS also infected maize (Zea mays cv. African Tall), johnsongrass (S. halepense cv. 81, symptomless infection), and sorghum (S. bicolor cv. CS-3541) but not prosomillet (Panicum miliaceum), fingermillet (Eleusine coracana cv. IE-2540), pearl millet (Pennisetum typhoides cv. NHB-3), or oat (Avena sativa cv. Kent) when sap transmitted. IBS also was transmitted by the corn leaf aphid (Rhopalosiphum maidis) in a nonpersistent manner from sorghum and sugarcane to all 18 sorghum lines in a glasshouse. Based on direct antigen-coating enzyme-linked immunosorbent assay (DAC-ELISA), serologically specific electron microscopy, and Western blot tests, IBS was serologically related to SrMV strains SCH and SCI (2-4) but not to MDMV strains A, D, and E; SCMV strains A, B, D, SC, and MDB; or JGMV type strain from Australia and strain O from Texas (2). IBS induced formation of cytoplasmic cylindrical inclusions consisting of pinwheels associated with long, straight, laminated aggregates (subdivision type II) in sorghum and sugarcane host cells. Purified IBS contained one major polypeptide of 40 kDa and a ribonucleic acid of 3.0 × 106 Da. Polyclonal antiserum to IBS was produced in rabbits and used in DAC-ELISA to confirm the identity of IBS in sugarcane, sorghum lines, and other test plant species. On the basis of biological and serological properties, IBS isolated from field-infected sorghum grain Tx2786 and sugarcane cv. Co.740 is identified as an immunity (QL3) breaking strain of SrMV (SrMV-IBS). References: (1) L. M. Giorda et al. Plant Dis. 70:624, 1986. (2) D. D. Shukla et al. Phytopathology 79: 223, 1989. (3) D. D. Shukla et al. 1998. AAB Description of Plant Viruses No. 359. AAB, Kew, Surrey, England. (4) Z. N. Yang and T. E. Mirkov. Phytopathology 87:932, 1997.