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Biomedical subjects

J Karani

Publications and source records attributed to J Karani.

At least 19 recordsLinked to original sources
PubMed2001

Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study.

To investigate whether sclerosing cholangitis with an autoimmune serology characteristic of autoimmune hepatitis (AIH) and AIH are distinct entities, we studied 55 consecutive children with clinical and/or biochemical evidence of liver disease and circulating antinuclear (ANA), anti-smooth muscle (SMA), and/or liver-kidney-microsomal type 1 (LKM1) autoantibodies. They underwent liver biopsy, direct cholangiography, sigmoidoscopy, and rectal biopsy at presentation. Twenty-eight were diagnosed as AIH in the absence and 27 autoimmune sclerosing cholangitis (ASC) in the presence of radiological features of cholangiopathy. Twenty-six ASC and 20 AIH had ANA and/or SMA; 1 ASC and 8 AIH LKM1 autoantibody. Similarities between the 2 conditions included most clinical and biochemical parameters and a lower frequency of HLA DR4. Inflammatory bowel disease and histological biliary changes were more common in ASC; coagulopathy, hypoalbuminemia, lymphocytic periportal hepatitis, and HLA DR3 were more common in AIH. Histological biliary changes were observed in 65% of ASC and 31% of AIH patients. Eighty-nine percent responded to immunosuppression. Follow-up liver biopsies from 17 ASC and 18 AIH patients had similarly reduced inflammatory activity and no progression to cirrhosis. Sixteen follow-up cholangiograms from AIH patients and 9 from ASC patients were unchanged, while 8 ASC patients showed a progressive cholangiopathy. One child with AIH and ulcerative colitis developed sclerosing cholangitis 8 years after presentation. At 2 to 16 years (median, 7 years) from presentation, all patients are alive, including 4 ASC patients who underwent liver transplantation. In conclusion, ASC and AIH are similarly prevalent in childhood; cholangiography is often needed to distinguish between these 2 entities, which are likely to lie within the same disease process.

Adolescent↗
PubMed2000

Size reduction of small bowels from adult cadaveric donors to alleviate the scarcity of pediatric size-matched organs: an anatomical and feasibility study.

BACKGROUND: Small bowel transplantation in children weighing less than 10 kg is hindered by the lack of size-matched donors. The ability to create reduced size small bowel grafts from adult cadaveric donors suitable for use in young children has been studied. METHODS: Volumetric assessment of computed tomography scans were used to evaluate abdominal cavity and small bowel volumes in children. Small bowels were retrieved from adult cadaveric donors and reduced in size. RESULTS: Computed tomography studies of the abdominal cavity showed that the mean volume available for a small bowel graft was 260 ml in children less than 5 kg (n = 5) and 460 ml in children weighing 5-10 kg (n = 5). Fifteen small bowels were successfully reduced to provide an ileal graft of one meter while keeping the whole length of the superior mesenteric artery and vein after their dissection in the proximal part of the mesentery. The mean volume of the grafts created was 270 ml in seven thin patients (body mass index [BMI] <25), 390 ml in five preobese patients (25< BMI<30), and 490 ml in three obese patients (BMI>30). Mesenteric transillumination in thin donors allowed safe dissection and complete hemostasis. No diameter reduction was required. Technical modifications permitted the creation of two grafts, one ileal and the other jejunal from a single donor. Volumetric and surgical data show that implantation of up to two meters of ileum from a thin adult weighing up to 80 kg is feasible in children weighing less than 10 kg. CONCLUSION: Size reduction of adult cadaveric small bowels can provide suitable grafts for children of less than 10 kg and could expand the potential pool of donors for these patients.

Adult↗
PubMed2000

Respiratory distress in a neonate with an enlarged thymus.

UNLABELLED: Thymic hyperplasia, although not a rare condition in infancy, is usually asymptomatic. We describe an infant presenting in the perinatal period with marked tachypnoea. An enlarged thymus, demonstrated on chest radiograph and CT, was associated with small-volume, non-compliant lungs. Other causes of pulmonary malfunction and maldevelopment were excluded. CONCLUSION: Thymic enlargement is unusually associated with neonatal respiratory distress but should be considered in the differential diagnosis.

Humans↗
PubMed2000

The chest radiograph appearances of very premature infants at 36 weeks post-conceptional age.

The chest radiograph of very premature infants at 36 weeks post-conceptional age (PCA) was evaluated with regard to the degree of hyperinflation and cardiomegaly, and the presence of fibrosis/interstitial shadowing, cystic elements, air bronchograms and opacification. The evolution of abnormalities was assessed by comparing the radiograph appearance at 36 weeks PCA with that at 28 days post-natal age (PNA). Three scoring systems were used to determine how any abnormalities present could be best quantified to reflect disease severity as determined by chronic dependency upon supplementary oxygen status. Chest radiographs at 36 weeks PCA from 60 infants (median gestational age 26 weeks (range 24-28)) were studied. 47 infants also had radiographs at 28 days PNA. Only three infants had no chest radiograph abnormalities at 36 weeks PCA, although 24 infants were not dependent upon supplementary oxygen. The most common abnormalities were interstitial shadowing and hyperinflation, while cystic elements and cardiomegaly were rare. The radiographic appearance had deteriorated from 28 days PNA to 36 weeks PCA (p < 0.05); more infants at 36 weeks PCA were hyperinflated (p < 0.01). The chest radiograph appearances of infants who were dependent upon supplementary oxygen scored higher than those who were not (p < 0.01) using all three scoring systems. The system that assessed only the presence of interstitial shadowing, cystic elements and hyperinflation had the highest specificity in identifying oxygen dependency beyond 36 weeks PCA and had the highest area under the respective receiver operator characteristic curve. In conclusion, the majority of very immature infants have an abnormal chest radiograph appearance at 36 weeks PCA. The appearance can, however, be meaningfully scored by evaluating only three abnormalities.

Female↗
PubMed1999

Splenic artery aneurysm and orthotopic liver transplantation.

Splenic artery aneurysms are a rare but potentially fatal complication after liver transplantation. We report three cases presenting in a 12-month period in adult patients who underwent transplantation for chronic liver disease. Doppler ultrasound of the splenic artery should be performed in all patients with cirrhosis and portal hypertension who are being assessed for liver transplantation. The aneurysm can be ligated at the time of transplantation.

Adult↗
PubMed1999

Arterioportal hypertension: a rare complication of partial hepatectomy.

An arterioportal fistula (APF) with arterialisation of the portal-venous system is a rare cause of portal hypertension (PH) in children. The condition may be a congenital isolated fistula or occur as part of a more generalised haemangiomatous malformation. We report a case of PH secondary to an APF, which presented with bleeding gastric varices 6 years after partial hepatectomy for hepatoblastoma. The diagnosis was established by angiography and the fistula occluded by embolisation.

Aneurysm, False↗
PubMed1999

A radiographic method for assessing lung area in neonates.

The aim of this study was to determine whether computer assisted analysis of lung area on the chest radiograph reliably predicted lung volume in neonates. Anteroposterior chest radiographs taken for clinical purposes were scanned and analysed using a Power Macintosh computer with a Wacom A5 Ultra Pad and NIH image software. The cardiac, mediastinal and thymic densities and areas of perihilar and lobar consolidation were subtracted from the thoracic area to give the lung area. This was compared with lung volume, assessed by measurement of functional residual capacity (FRC), within 1 h of the chest radiograph being performed. 50 infants, median gestational age 30 weeks (range 24-43) were studied. Their median lung area was 11.23 cm2 (range 0.82-28.53) and lung volume 28 ml (range 3-103). The intraobserver and interobserver coefficients of repeatability of lung area were 1.0 cm2 and 1.06 cm2, respectively. Lung area correlated significantly with FRC (r = 0.60, p < 0.0001). It is concluded that computer assisted analysis of the chest radiograph lung area is a reliable method of assessing lung volume in neonates.

Functional Residual Capacity↗
PubMed1999

A simple chest radiograph score to predict chronic lung disease in prematurely born infants.

A simple scoring system has been evaluated with regard to its ability to characterize the pre-term infant's chest radiograph appearance at 28 days and predict oxygen dependency beyond 36 weeks post-conceptional age (PCA). Chest radiographs taken at approximately 1 month of age in 42 infants (median gestational age 28 weeks) were assessed by the scoring system for the presence of fibrosis/interstitial shadows, cystic elements and degree of hyperinflation (maximum score 8). The system's results were then compared with those obtained using two previously published scoring systems. Using all three systems, there were significant differences in the scores of infants who were and were not oxygen dependent at 28 days (p < 0.001) and 36 weeks PCA (p < 0.001). For the three systems, the positive predictive values of a score of 3 or more to predict oxygen dependency at 36 weeks (PCA) were between 67% and 80% and similar receiver operating characteristic curves were obtained. We conclude that scoring only three abnormalities of the 28 day chest radiograph appearance of pre-term infants gives useful predictive information.

Chronic Disease↗
PubMed1998

Accessory right hepatic artery arising from the left: implications for split liver transplantation.

BACKGROUND: Variant arterial anatomy must be recognized and appropriately managed during split liver transplantation to ensure complete vascular supply to both grafts. We describe an accessory posterior right hepatic artery, arising from the left and passing behind the portal vein bifurcation. METHODS: Thirty-seven consecutive livers were examined during ex vivo liver-splitting procedures. An abnormal right accessory artery arising from the left hepatic artery was identified high in the porta hepatis. The anatomical variant is described and illustrated by methylene blue injection and arteriography. RESULTS: The anomaly was encountered in 2 of 37 split liver procedures. The two right lobes with the abnormal artery were discarded. CONCLUSION: Care should be taken during dissection behind the portal vein bifurcation to exclude an accessory segmental right hepatic artery. If present, the liver may not be suitable for splitting without compromising the right lobe, unless the left hepatic artery can be divided distal to the origin of the accessory vessel.

Hepatic Artery↗
PubMed1998

Laparoscopic cholecystectomy injury: an unusual indication for liver transplantation.

The introduction of laparoscopic cholecystectomy has been associated with a rise in the number of reported bile duct injuries (0.3%-0.8%). Significant vascular injuries are rare (0.16%), but may lead to life-threatening complications. We present a case report of a patient undergoing transplantation for a laparoscopic cholecystectomy injury.

Adult↗
PubMed1998

Chylothorax after repair of congenital diaphragmatic hernia--risk factors and morbidity.

BACKGROUND/PURPOSE: Chylothoraces have been reported rarely after congenital diaphragmatic hernia (CDH) repair, but that is contrary to the authors' experience. The aim of this study was to audit the outcome of antenatally diagnosed CDH cases to determine the incidence, possible risk factors, and morbidity associated with chylothorax in CDH patents. METHODS: Twenty-four of 35 consecutive infants with CDH (69%) underwent surgical repair and survived the immediate postoperative period. RESULTS: Effusions developed in all 22 for whom chest radiographs were available. Six of the eight infants whose effusions required drainage had a chylothorax. Those six infants required a mean of 8 days of thoracentesis, during which a mean total volume of 358 mL/kg of fluid was removed. The infants remained on a medium-chain triglyceride (MCT) formula for a mean of 81 days. The six infants with a chylothorax differed from the rest with respect to their duration of oxygen dependence (a median of 28.5 versus 15 days, P < .05) and hospital stay (a median of 5.5 versus 4 weeks, P < .05), but no significant risk factors for the development of a chylothorax were identified. CONCLUSION: Chylothorax is a relatively common cause of effusion after CDH repair and is associated with increased morbidity.

Chylothorax↗
PubMed1998

Tumour ablation and hepatic decompensation rates in multi-agent chemoembolization of hepatocellular carcinoma.

Thirty-seven cirrhotic patients with 62 hepatocellular carcinoma (HCC) foci--most Child-Pugh class B or C and/or with large, inoperable tumours--underwent 148 sessions of transcatheter arterial chemoembolization (TACE) using lipiodol, doxorubicin and cisplatin. Treatment efficacy was assessed by serial hepatic arteriography in 34/37 (91.9%) patients and abdominal CT scanning in 3/37 (8.1%) patients. Child-Pugh status was determined prior to each treatment session. Varying degrees of control of tumour neovascularity occurred for a median 390 days (range 90 to > 1680 days) in 33/34 (97.1%) patients in whom progress hepatic arteriography was performed. Ablation of tumour neovascularity occurred in 6/6 (100%), 4/12 (33.3%) and 6/16 (37.5%) patients with HCC diameters < 4 cm, 4-7 cm and > 8 cm, respectively (p < 0.02). Significantly more sessions were required for ablation of larger tumours (p < 0.05). Recurrent HCC was detected in 50% of patients after a median 240 days (range 60-1120 days). Deterioration in Child-Pugh status followed a session of TACE on 19/148 (12.8%) occasions but resulted in unscheduled hospitalization on only 4/148 (2.7%) occasions, the highest incidence (8.3%) in Child-Pugh C patients. Actuarial survival was 27/36 (75.0%) at 6 months, 17/34 (50.0%) at 12 months, 14/34 (41.2%) at 18 months, 9/31 (29.0%) at 24 months and 4/27 (14.8%) at 36 months. Multi-agent TACE with lipiodol, doxorubicin and cisplatin provides a useful anti-tumour effect, even in cirrhotic patients with large HCCs. The incidence of clinically significant deterioration in hepatic function due to ischaemia of non-tumorous liver is acceptably low, even in Child-Pugh C patients.

Aged↗
PubMed1998

Split liver transplantation: King's College Hospital experience.

BACKGROUND: The purpose of split liver transplantation is to increase the source of pediatric grafts without compromising the adult donor pool. Early results have been discouraging because of technical complications and selection of poor risk patients. METHODS: The results of a single center experience of 41 split liver transplantations were analyzed. Patient and graft survival and complications related to the technique were analyzed. RESULTS: Patient and graft survival for the whole group was 90% and 88% respectively at a median follow up of 12 months (range 6-70 months). Patient and graft survival for the right lobe graft was 95% and the left lateral segment 86% and 82% respectively. Four patients died, of which two of the patients were first two splits following technical complications. Two others died, one from cerebral lymphoma and the other of multiorgan failure secondary to sepsis. One patient has been retransplanted for chronic biliary sepsis. CONCLUSION: Split liver transplantation has now become an acceptable treatment option for both adult and pediatric recipients with end stage liver disease. Right lobe recipients are not disadvantaged by the procedure. Good results can be achieved with better patient selection and by the use of good quality organs.

Adolescent↗
PubMed1997

The management of high-grade hilar strictures by endoscopic insertion of self-expanding metal endoprostheses.

BACKGROUND AND STUDY AIMS: Treatment options for unresectable hepatic hilar strictures include percutaneous or endoscopic stent insertion, using either standard Teflon or self-expanding metal endoprostheses. The use of Teflon stents is complicated by stent migration and high occlusion rates. Published series of endoscopically placed metal stents have not concentrated on their use in the treatment of high-grade hilar strictures (Bismuth grades II and III). We therefore undertook a prospective and open pilot study to evaluate the efficacy of endoscopically placed metal endoprostheses in the palliation of jaundice due to high-grade hilar strictures. PATIENTS AND METHODS: Self-expanding metal stents were placed endoscopically in 17 patients (nine women, eight men; median age 64 years, range 33-77). Of the 17 malignancies 11 patients (65%) had a diagnosis of cholangiocarcinoma, and 12 (71%) had Bismuth grade III strictures. RESULTS: The stents were inserted successfully in all 1 patients. Adequate drainage, as demonstrated by a significant reduction in bilirubin, was achieved in 1: (88%); the two patients in whom drainage failed had extensive intrahepatic disease. Early complications (cholangitis) developed in one patients (6%), and there were seven late complications (41%) in five patients (four occluded stents and three cases of duodenal compression). The stent patency appeared to be prolonged (median stent patency 12 months, range 1.5-24 months, in patients surviving five months or more). The median survival was ten months (range 1-48 months) after stent insertion. All of the patients died during follow-up. CONCLUSIONS: These results suggest that endoscopically placed metal stents offer effective palliation for high grade hilar malignancies, although controlled trial comparing the use of metal and Teflon endoprostheses in this patient group are required.

Bile Duct Neoplasms↗
PubMed1997

Liver transplantation for extra hepatic biliary atresia.

Kasai portoenterostomy has transformed the prognosis for children with Extra Hepatic Biliary Atresia (EHBA). However, for children developing end stage liver disease following portoenterostomy, liver transplantation (OLT) is the treatment of choice. Between February 1989 and March 1996, 64 children with EHBA underwent 79 transplants (26 males, 38 females; median age 2.2 years, range 5 months-17 years; median weight 11.4 kg, range 5-65 kg). Of these, 58 (85%) had undergone previous portoenterostomy. Nineteen patients (30%) had gastrointestinal bleeding prior to OLT assessment. Mean serum bilirubin was 229 mumol/liter (range 11-801 muml/liter). Four children had associated polysplenia syndrome. Of the 79 transplants, 30 received whole and 41 reduced-size cadaveric grafts and 9 living related grafts. Eleven patients (17%) died, nine within one month of surgery. Thirteen patients were retransplanted once and one twice. There were 16 vascular complications (10 hepatic artery thrombosis, 3 portal vein thrombosis, 3 venous outflow obstruction) and 10 biliary complications (4 anastomotic leaks, 6 strictures). Ten patients (16%) had bowel perforation following the transplant. The 5 year actuarial patient and graft survival for this group is 84% and 69% respectively with normal physical and mental development in the majority. OLT provides satisfactory treatment for children with EHBA with end stage liver disease with long term survival in the majority.

Adolescent↗
PubMed1996

Hepatic artery thrombosis after liver transplantation in children under 5 years of age.

The incidence of hepatic artery thrombosis (HAT) following orthotopic liver transplantation in children varies from 4% to 26% and represents a significant cause of graft loss. The purpose of this study was to analyze the risk factors for HAT following liver transplantation in children less than 5 years old. Seventy-three transplants were performed in 62 children under 5 years of age, including 16 for acute hepatic failure, 46 for chronic liver disease, and 11 retransplants. Twenty-four whole liver grafts (WLG) and 49 reduced size grafts (3 right lobes, 16 left lobes, and 30 left lateral segments) were transplanted. The recipient common hepatic artery was used to provide arterial inflow in 22 transplants and an infrarenal iliac conduit in 51 transplants. The overall incidence of HAT was 8 out of 73 transplants (11%). The cold ischemia time (14.3 +/- 3.03 hr) in this group was significantly longer than the cold ischemia time for those without HAT (11.7 +/- 3.94 hr) (P = 0.049). The incidence of HAT for whole and reduced grafts was 25% (6/24) and 4% (2/49), respectively (P = 0.01). HAT occurred in 6 of 22 grafts (27.3%) revascularized from the recipient common hepatic artery, compared with 2 of 51 grafts (3.9%) using an infrarenal arterial conduit (P = 0.008). The combination of recipient hepatic arterial inflow to a WLG resulted in HAT in 50% (6/12), whereas there were no cases of HAT with an iliac conduit to a WLG (P = 0.01). Of the eight patients with HAT, five are alive (median follow-up, 20 months; range, 7-27 months). Five patients were retransplanted, three within the first 2 weeks and two at 4 and 5 months for abnormal liver function in association with clinical and histological features of chronic rejection. Prolonged cold ischemia time and use of a whole graft with recipient hepatic arterial inflow are risk factors for developing HAT. The use of reduced size grafts and infrarenal iliac arterial conduits are associated with a low incidence of HAT.

Child, Preschool↗