Single and multiple food allergies in infants with proctocolitis.

BACKGROUND: Food protein-induced allergic proctocolitis is a frequent cause of rectal bleeding in infants. Characteristics of infants with multiple food allergies have not been defined. OBJECTIVE: This study aimed to identify characteristics of infants with proctocolitis and compare infants with single and multiple food allergies. METHODS: A total of 132 infants with proctocolitis were evaluated retrospectively. All of the infants were diagnosed by a paediatric allergist and/or a paediatric gastroenterologist according to guidelines. Clinical features of the infants, as well as results of a complete blood count, skin prick test, specific immunoglobulin E, and stool examinations or colonoscopy were recorded. RESULTS: Cow's milk (97.7%) was the most common allergen, followed by egg (22%). Forty-five (34.1%) infants had allergies to more than one food. Infants with multiple food allergies had a higher eosinophil count (613±631.2 vs. 375±291.9) and a higher frequency of positive specific IgE and/or positive skin prick test results than that of patients with a single food allergy. Most of the patients whose symptoms persisted after two years of age had multiple food allergies. CONCLUSIONS: There is no difference in clinical presentations between infants with single and multiple food allergies. However, infants with multiple food allergies have a high blood total eosinophil count and are more likely to have a positive skin prick test and/or positive specific IgE results.

Awareness of glucose-6 phosphate-dehydrogenase deficiency in celiac disease.

UNLABELLED: Individuals with celiac disease (CD) are predisposed to a number of haematological abnormalities including anaemia secondary to malabsorption of iron, vitamin B12 or folate; anaemia of chronic disease and coagulopathy secondary to vitamin K deficiency. Correction of coagulopathy with vitamin K is necessary before endoscopic biopsy in patients with suspected CD. However, vitamin K causes haemolysis in glucose-6 phosphate-dehydrogenase deficiency. CONCLUSION: When vitamin K administration becomes necessary for correction of coagulopathy in patients with CD; glucose-6 phosphate-dehydrogenase deficiency should be considered.

Increased risk for coeliac disease in paediatric patients with migraine.

The aim was to determine the prevalence of coeliac disease (CD) in paediatric patients with migraine. Serum tissue transglutaminase IgA (tTGA) antibodies and IgA concentrations were measured in 73 patients with migraine (age range 6-17 years) and the control group (n = 147). Patients having positive tTGA antibodies underwent duodenal biopsy. Four patients (5.5%) from the study group and one (0.6%) from the control group had positive tTGA antibody titres (P < 0.05). Three patients with migraine had normal duodenal histology and were considered as potential CD. One patient from the study group and one from the control group declined to have biopsy. tTGA antibody is considered as a reliable indicator for the presence of CD. However, some patients with positive antibodies may have normal biopsy initially and are classified as having potential CD. Our finding of a higher prevalance of tTGA antibodies in paediatric migraine patients suggests that an association between migraine and CD might exist.

Liver transplantation in patients with hepatocellular carcinoma: one center's experience.

Hepatocellular carcinoma (HCC), which worldwide is the fifth most common malignancy in men and the ninth most common malignancy in women, accounts for 6% of all malignant lesions. We evaluated our results of liver transplantation for patients with HCC. Between January 2004 and April 2007, 31 patients (5 females, 26 males; age range, 1.1-65 years) with preoperatively or incidentally diagnosed HCC underwent orthotopic liver transplantation (OLT) at our center. Eleven grafts were from deceased donors, and 20 from living-related donors. Inclusion criteria were no invasion of a major vascular structure and no evidence of extrahepatic disease. In 17 patients, tumors exceeded the Milan criteria. According to the tumor-node-metastasis staging system, 6 patients had stage 1, 8 had stage II, 2 had stage III, and 15 had stage 4A carcinoma. Three complications occurred in 31 patients: hepatic arterial thrombosis in 1 patient and biliary leakage in 2. At a mean follow-up of 24.3 +/- 12.5 months, 29 patients are well with excellent graft function. Two patients died at 23 and 17 months after OLT respectively. The longest graft survival is 43 months. There have been 4 tumor recurrences, namely, at 4, 26, 24, and 29 months after OLT, respectively. Patient and disease-free survival rates are 93.5% and 90%, respectively. In conclusion, OLT provided long-term disease-free survival for patients with HCC, even those with locally advanced tumors who had no effective alternative treatment than transplantation.

Liver transplantation for Wilson's disease.

Wilson's disease is an inherited disorder of copper metabolism characterized by reduced biliary copper excretion, which results in copper accumulation in the tissues with liver injury and failure. Orthotopic liver transplantation (OLT) can be lifesaving for patients with Wilson's disease who present with fulminant liver failure and for patients' unresponsive to medical therapy. The aim of this study was to review our experience with OLT for patients with Wilson's disease. Between September 2001 and April 2007, 25 OLTs were performed in 24 patients (7 females and 17 males) with Wilson's disease of mean age 15.6 +/- 9.9 years (range, 5-51 years). Six patients underwent transplantation owing to coexistent fulminant hepatic failure and 18 with chronic advanced liver disease with (n = 8) or without (n = 10) associated neurologic manifestations. We performed 3 full-size, deceased-donor OLTs and 22 living-related donor OLTs. Eight patients had a family history of Wilson's disease. We detected a Kayser-Fleischer ring in 18 patients. All patients had a low serum ceruloplasmin level (mean, 27.8 mg/dL) and a high urinary copper excretion level (mean, 4119 mug/d) before OLT. Following successful OLT, there was a significant reduction in urinary copper excretion (median, 37.1 mug/d) in all patients. Mean follow-up was 21.7 +/- 19.8 months (range, 2-60 months). Retransplantation was required in 1 patient at 12 days after the first OLT owing to primary graft nonfunction. Five of the 24 patients died within 4 months of the surgery. The remaining 19 survivors (79%) have remained well, with normal liver function and no disease recurrence. In conclusion, OLT was a curative procedure for Wilson's disease among patients presenting with fulminant hepatic failure and others with end-stage hepatic insufficiency. After OLT, the serum ceruloplasmin level increased to the normal range, urinary copper excretion decreased, and neurologic manifestations improved.

Liver transplantation for biliary atresia.

Biliary atresia is the most common indication for liver transplantation (OLT) in children. We present our experience with OLT as a treatment for end-stage liver disease in children with biliary atresia. We performed a retrospective review of 20 biliary atresia patients (11 male, 9 female patients; mean age, 21.4 months; range, 6 to 84 months) who had undergone OLT. Mean preoperative weight and height were 10.1 +/- 5.8 kg and 72.5 cm, respectively. Thirteen recipients were younger than 1 year of age, and 15 weighed less than 10 kg at the time of OLT. Fourteen recipients had undergone a Kasai operation prior to the OLT. The mean serum total bilirubin level was 22.56 mg/dL before OLT. Eighteen left lateral segment grafts and two whole grafts were transplanted. The mean recipient operative time was 9.25 hours. The mean recipient intraoperative blood loss was 1.81 U. Two hepatic arterial thromboses and one biliary leak occurred soon after surgery. Portal vein stenoses developed in two recipients at 10 and 12 months after OLT; both were treated with balloon dilatation. Two biliary stenoses, which occurred at 10 months and 3.5 years after surgery, were treated with balloon dilatation. Two recipients died at 2 and 12 days after OLT because of respiratory distress syndrome and sepsis, respectively. The remaining 18 (90%) recipients are alive with good graft function. The overall rejection rate was 31.25%. OLT is an effective treatment for children with biliary atresia and a failed Kasai procedure. Living related liver grafts represented an excellent organ supply for these patients.

Liver transplantation for small babies.

Orthotopic liver transplantation remains a major medical and surgical challenge in small pediatric patients. From April 2003 to June 2006, 21 small babies (each of whom weighed less than 10 kg or was younger than 1 year of age) underwent orthotopic liver transplantation. Five were girls and 16 were boys with a mean age of 15.7 +/- 9.3 months (range, 2-24 months); their mean weight at the time of transplantation was 9.8 +/- 3.6 kg (range, 6-16 kg). All transplants were obtained from a living-related donor. Left lateral segment was used for all transplantations. The median graft-to-recipient weight ratio was 3.5% +/- 1.2% (range, 1.5%-6.1%). During the early postoperative period, hepatic arterial thrombosis was identified in 4 patients, and a biliary leak was detected in 2 patients. In 2 patients, portal vein stenosis was identified during the late postoperative period. At the time of this writing, the 17 alive patients (81%) exhibited good graft function at median follow-up of 14.8 +/- 10.9 months (range, 1-39 months). Four patients died during the follow-up. Histological examination revealed hepatocellular carcinoma in 2 patients, and Burkitt's lymphoma in 1 patient. In conclusion, our data confirmed that living-related donors, especially in this age group, provide a reliable source for the organ pool. Satisfactory results can be achieved despite the anatomic handicaps of this age group.

Pediatric liver transplantation for acute liver failure.

The only proven therapy for patients unlikely to recover from acute liver failure (ALF) is liver transplantation. Correct diagnosis of these individuals and rapid referral to a transplant center are crucial. We evaluated 12 pediatric patients with ALF who underwent liver transplantation (LT) at our institution during a 3-year period. The reasons for transplantation were hepatitis A (3 patients); non-A, non-E hepatitis (3); autoimmune hepatitis (1); fulminant Wilson's disease (3); Amanita phalloides (mushroom) poisoning (1); and hepatitis B and toxic hepatitis with leflunomide treatment (1). Seven of the participants were female and five were male (mean age, 9.1 +/- 4.2 years). Three received right liver-lobe grafts, one received a whole liver graft, and the remainder received left or left-lateral liver lobe grafts. All patients recovered from hepatic coma the second postoperative day. Two patients died at postoperative days 57 and 71 due to adult respiratory distress syndrome and sepsis with multiorgan failure, respectively. One patient required retransplantation because of chronic rejection 7 months after the initial transplantation. That patient died 10 days after retransplantation because of sepsis. Nine patients were healthy at follow-up (range, 2-46 months). LT is the only treatment option for ALF in patients in countries with low organ-donation rates. In this scenario, donor preparation in a limited time frame is difficult. We have been able to decrease the duration of donor preparation to approximately 4 hours (including biopsy of the donated liver tissue).

Expanded criteria for hepatocellular carcinoma and liver transplantation.

In this study, we evaluated our early results of liver transplantation for hepatocellular carcinoma (HCC). Between January 2004 and June 2006, 26 patients (4 females, 22 males; aged 1.1-65 years) with preoperatively diagnosed or incidental HCC underwent liver transplantation at our center. Inclusion criteria (independent of tumor size and number of tumor nodules) were no invasion of major vascular structures and no evidence of extrahepatic disease. In 13 of the patients, tumors were beyond the Milan criteria. At this writing, at a mean follow-up of 16.5 months (range, 1-31 months), all patients were doing well with excellent graft function. The longest survival is 2.5 years, and our patient survival rate is 100%. There has been only 1 tumor recurrence, which occurred 4 months after liver transplantation. In conclusion, liver transplantation provides long patient and disease-free survival, even in patients with HCC that exceeds the Milan criteria.

Safety of multidetector computed tomography in calculating liver volume for living-donor liver transplantation.

Living-related liver transplantation was developed to overcome the organ shortage for both children and adults with end-stage liver disease. Because impaired liver function after resection and transplantation is caused by insufficient liver volume, the reliable volumetric assessment of the hepatic segments of potential living donors is a critical element in preoperative evaluation. In this study, we compared the results of multidetector computed tomographic (CT) volumetry with the intraoperative findings from 80 liver transplantations performed at our center. Resection borders were determined preoperatively with the aid of CT by manual delineation in which the hepatic vessels were used as guides. Resected liver grafts were weighed intraoperatively, and the calculation of their volume was based on the specific weight of 1 g/mL. Statistical analyses were performed with Pearson's correlation test; P < .05 was considered significant. The study subjects consisted of 48 women and 32 men (mean age, 35.6 +/- 9.7 years; range, 23-56 years). Forty-one donors underwent right lobectomy, 22 underwent left lobectomy, and 17 underwent left lateral segmentectomy. Manual volumetric measurement was completed within 15 minutes. No significant differences were found between the results of preoperative volumetry and the intraoperative measurement. We therefore concluded that manual CT volumetric calculation is a reliable method of calculating liver volume for living-donor liver transplantation.

Liver transplantation in children weighing less than 10 kilograms.

Orthotopic liver transplantation (OLT) remains a major medical and surgical challenge in small pediatric patients. From April 2003 through October 2005, 17 infants (each of whom weighed less than 10 kg) underwent the procedure. Four were girls and 13 were boys (mean age, 15.7 +/- 9.3 months [range, 2-36 months]; mean weight at the time of transplantation, 7.4 +/- 2.6 kg [range, 6-10 kg]). All transplants were obtained from living-related donors. Sixteen left lateral segments and 1 left lobe were transplanted. The median graft-to-recipient weight ratio was 3.5% +/- 1.2% (range, 1.5%-6.1%). During the early postoperative period, hepatic arterial thrombosis was identified in 2 infants, and a biliary leak in 1. Hepatic arterial thrombosis was treated by reanastomosis with polytetrafluoroethylene grafting in the first patient and by surgical embolectomy in the second. The biliary leak was treated with percutaneous drainage. In 1 infant, portal vein stenosis, which was identified during the late postoperative period, was treated by percutaneous balloon dilatation. At this time, 14 (82.3%) infants were alive, exhibiting good graft function at a median follow-up of 11 months (range, 2-36 months). Three infants died: 1 on postoperative day 47 from adult respiratory distress syndrome, 1 on postoperative day 12 from sepsis, and 1 on postoperative day 65 from sepsis associated with EBV infection. Episodes of acute rejection, which occurred in 5 patients, were treated with pulse steroid therapy. On follow-up, histologic examination revealed hepatocellular carcinoma in 2 infants and Burkitt's lymphoma in 1 infant. Our data confirm that extensive use of living-related donors in liver transplantation can result in an excellent outcome for small pediatric patients.

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.

Living-related liver transplantation in pediatric patients.

INTRODUCTION: Many developments in surgical technique, immunosuppression, and patient selection criteria have led to improved long-term patient and graft survival in pediatric patients receiving liver transplants. In this study, we examined the early results of 26 pediatric recipients who underwent 26 liver transplantations between January 2003 and December 2004 at our institution. MATERIALS AND METHODS: The most common indications for liver transplantation were cholestasis in 10 patients (38.5%) and Wilson's disease in 8 (30.8%). Other indications were fulminant hepatic failure (4 patients, 15.4%), tyrosinemia (2 patients, 7.7%), Caroli disease (1 patient, 3.8%), and cryptogenic cirrhosis (1 patient, 3.8%). One recipient with Byler disease and two with tyrosinemia also had incidental hepatocellular carcinoma. RESULTS: Of 26 patients, 24 (92.3%) underwent living-related liver transplantation and 2 (7.7%) underwent cadaveric transplantation. The medical records of all patients were retrospectively reviewed. Twenty-two of 26 survived with excellent graft function, showing 91.2%, 86.4%, and 81.6% at 3, 12, and 24 months graft and patient survival rates, respectively. Sixteen patients (61.5%) developed various morbidities with biliary and vascular complications being the most common. Four patients (15.3%) developed bile leaks. Four patients (15.3%) developed hepatic artery thromboses. Five patients (19.2%) developed life-threatening infections. Four patients (15.4%) died during the study period, three owing to infectious complications. The other patient died due to acute respiratory distress syndrome. CONCLUSION: Despite technical difficulties and a donor organ shortage, the results of liver transplantation in pediatric patients with end-stage liver disease have demonstrated promising results at our institution.

Duct-to-duct biliary reconstruction without a stent in pediatric living-donor liver transplantation.

PURPOSE: In pediatric liver transplantation, Roux-en-Y hepaticojejunostomy is often preferred for biliary reconstruction, especially in living-donor liver transplantation (LDLT). Limited numbers of duct-to-duct biliary reconstructions have been presented in pediatric recipients. We retrospectively reviewed our experiences with duct-to-duct biliary reconstruction without a stent in pediatric LDLT recipients. MATERIALS AND METHODS: Since September 2006, 32 LDLTs were performed using a duct-to-duct biliary reconstruction without a stent in 31 children (16 boys and 15 girls; overall mean age, 8.3±5.1 years). We transplanted 19 left lobe grafts, 11 left lateral segments, 1 monosegment, and 1 reduced-size right lobe graft. Twenty-eight grafts had a single bile duct; the remaining 4, two bile ducts. We created a single orifice at the back table for the grafts that had 2 bile ducts. RESULTS: Two recipients developed bile leakage in the early postoperative period; 3 bile duct stenoses occurred in the late postoperative period. All biliary complications were successfully treated with interventional radiologic or endoscopic approaches. There was no morbidity and no graft loss owing to biliary complications. During a mean follow-up of 23.5±13.6 months (range, 4-44), 4 children died and the remaining 27 (88%) are doing well with satisfactory liver function. CONCLUSION: Our results showed that duct-to-duct biliary reconstruction without a stent was a safe technique for biliary reconstruction even among pediatric cases.

Diagnosis and treatment of late-onset portal vein stenosis after pediatric living-donor liver transplantation.

PURPOSE: Portal vein stenosis is a relatively rare complication after living-donor liver transplantation, which sometimes leads to a life-threatening event owing to gastrointestinal bleeding or graft failure. This study sought to evaluate the diagnoses and management of late-onset portal vein stenosis in pediatric living-donor liver transplants. MATERIALS AND METHODS: Since September 2001, we performed 123 living-donor liver transplant procedures in 120 children, among which 109 children with a functioning graft at 6 months after living-donor liver transplant are included in this analysis. Seven instances of portal vein stenosis were diagnosed and were analyzed retrospectively. RESULTS: The median age of the children was 5.3 years, and the median body weight was 19.2 kg. Portal vein stenosis was diagnosed at 11.2±3.1 months after living-donor liver transplantation. Whereas 3 children were asymptomatic, splenomegaly and/or massive ascites were observed in the remaining 4. Additionally, platelet counts were below the normal limit in 4 children. All children were treated with transhepatic balloon dilatation except 1. Intraluminal stent placement was needed in 1 child owing to resistance of balloon dilatation. The mean pressure gradient decreased from 12.4 to 3.2 mmHg after successful treatment. We did not observe any treatment-related complications. Portal venous patency was maintained in all children during posttreatment follow-up of 43.2±20.4 months. There were no recurrences of portal vein stenosis. One child died; the remaining 6 children are alive with good graft function at 49.8±23.9 months of follow-up. CONCLUSION: Although most portal vein stenosis is asymptomatic, splenomegaly and platelet counts are 2 important markers for portal vein stenosis. Early detection of portal vein stenosis with these 2 markers can lead to successful interventional percutaneous approaches and avoid graft loss.

Management of early hepatic arterial thrombosis after pediatric living-donor liver transplantation.

PURPOSE: Early hepatic arterial thrombosis after living-donor liver transplantation is a cause of graft loss and patient mortality. We analyzed early hepatic arterial thrombosis after pediatric living-donor liver transplantation. MATERIALS AND METHODS: Since September 2001, we performed 122 living-donor liver transplants on 119 children. Ten hepatic arterial thromboses developed in the early postoperative period. The 7 male and 4 female patients of overall mean age of 6.3±6.1 years underwent 5 left lateral segment, 3 right lobe, and 2 left lobe transplantations. RESULTS: Among 10 children with hepatic arterial thrombosis, 8 diagnoses were made before any elevation of liver function tests. One child displayed fever at the time of the hepatic arterial thrombosis. The median time for diagnosis was 5 days. Hepatic arterial thrombosis was treated with interventional radiologic techniques in 9 children, with 1 undergoing surgical exploration owing to failed radiologic approaches, and a reanastomosis using a polytetrafluoroethylene graft. Successful revascularization was achieved in all children, except 1. Four children died, the remaining 6 are alive with good graft function. During the mean follow-up of 52.7±18.8 months, multiple intrahepatic biliary stenoses were identified in 1 child. CONCLUSION: Routine Doppler ultrasonography is effective for the early diagnosis of hepatic arterial thrombosis. Interventional radiologic approaches such as arterial thrombolysis and intraluminal stent placement should be the first therapeutic choices for patients with early hepatic arterial thrombosis; if radiologic methods fail, one must consider surgical exploration or retransplantation.

Mutation spectrum of fumarylacetoacetase gene and clinical aspects of tyrosinemia type I disease.

Tyrosinemia type I (OMIM 276700) is a rare, autosomal recessive disorder caused by a deficiency in the fumarylacetoacetate hydrolase (FAH) enzyme. This study examined the spectrum of FAH gene mutation in 32 patients with tyrosinemia type I. In addition, clinical and biochemical findings were evaluated to establish a genotype-phenotype relationship in the patients. Mutation screening was performed using a 50K custom-designed resequencing microarray chip (TR_06_01r520489, Affymetrix) and sequencing analysis. Of the 12 different mutations found, 6 are categorized as novel. Three of the mutations-IVS6-1G>A, D233V, and IVS3-3C>G-are the most common in Turkish patients, comprising 25%, 17.1%, and 12.5% of mutant alleles, respectively.Clinical evaluations suggest that the spectrum of symptoms observed in the patients with very early and early disease were of the more nonspecific form, whereas the patients with late-presenting disease had more of the distinctive form over the course of the disease. This study adds support to the notion that the D233V mutation is specific to the Turkish population.

Gaucher disease with communicating hydrocephalus and cardiac involvement.

A 14-year-old female with Gaucher disease presented with hydrocephalus, corneal opacities, cirrhosis, and cardiac valvular involvement. A homozygous D409H mutation was identified. She underwent surgery for aortic and mitral valve replacement. Because of severe calcification of the aortic root, no successful valve replacement was performed. She died on the third day after the explorative cardiac surgery. Cardiac abnormalities represent a life-threatening presentation of the homozygous D409H mutation. Identification of this type is essential prior to initiating appropriate therapy with enzyme replacement and cardiac corrective surgery.