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1.
Allergol. immunopatol ; 46(1): 3-8, ene.-feb. 2018. tab
Artigo em Inglês | IBECS | ID: ibc-170781

RESUMO

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 (AU)


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Assuntos
Humanos , Lactente , Proctocolite/etiologia , Hemorragia Gastrointestinal/etiologia , Hipersensibilidade Alimentar/complicações , Proteínas Alimentares/efeitos adversos , Colonoscopia , Eosinofilia/imunologia , Hipersensibilidade Imediata/imunologia , Testes Cutâneos/estatística & dados numéricos
2.
Allergol Immunopathol (Madr) ; 46(1): 3-8, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28739021

RESUMO

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.


Assuntos
Eosinófilos/imunologia , Hipersensibilidade Alimentar/epidemiologia , Proctocolite/epidemiologia , Alérgenos/imunologia , Colonoscopia , Proteínas do Ovo/imunologia , Feminino , Hipersensibilidade Alimentar/diagnóstico , Humanos , Imunoglobulina E/sangue , Lactente , Masculino , Proteínas do Leite/imunologia , Proctocolite/diagnóstico , Estudos Retrospectivos , Testes Cutâneos , Turquia/epidemiologia
3.
Transplant Proc ; 43(2): 595-7, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-21440772

RESUMO

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.


Assuntos
Ductos Biliares/cirurgia , Procedimentos Cirúrgicos do Sistema Biliar/métodos , Transplante de Fígado/métodos , Doadores Vivos , Adolescente , Anastomose em-Y de Roux/métodos , Anastomose Cirúrgica/métodos , Criança , Pré-Escolar , Feminino , Seguimentos , Sobrevivência de Enxerto , Humanos , Masculino , Complicações Pós-Operatórias , Resultado do Tratamento
4.
Transplant Proc ; 43(2): 601-4, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-21440774

RESUMO

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.


Assuntos
Constrição Patológica/patologia , Transplante de Fígado/métodos , Veia Porta/cirurgia , Criança , Pré-Escolar , Feminino , Trato Gastrointestinal/patologia , Rejeição de Enxerto , Hemorragia/etiologia , Humanos , Falência Hepática/complicações , Falência Hepática/terapia , Doadores Vivos , Masculino , Pediatria/métodos , Complicações Pós-Operatórias , Estudos Retrospectivos
5.
Transplant Proc ; 43(2): 605-8, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-21440775

RESUMO

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.


Assuntos
Artéria Hepática/patologia , Transplante de Fígado/métodos , Fígado/irrigação sanguínea , Doadores Vivos , Trombose/terapia , Adolescente , Criança , Pré-Escolar , Feminino , Rejeição de Enxerto , Humanos , Fígado/patologia , Masculino , Pediatria/métodos , Terapia Trombolítica , Ultrassonografia Doppler/métodos
6.
JIMD Rep ; 1: 17-21, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-23430822

RESUMO

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.

7.
Acta Paediatr ; 99(5): 786-788, 2010 May.
Artigo em Inglês | MEDLINE | ID: mdl-20064138

RESUMO

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.


Assuntos
Doença Celíaca/complicações , Deficiência de Glucosefosfato Desidrogenase/complicações , Glucosefosfato Desidrogenase/sangue , Doença Celíaca/diagnóstico , Doença Celíaca/dietoterapia , Contraindicações , Dieta Livre de Glúten , Endoscopia Gastrointestinal , Deficiência de Glucosefosfato Desidrogenase/diagnóstico , Hemólise , Humanos , Lactente , Mucosa Intestinal/patologia , Masculino , Vitamina K , Deficiência de Vitamina K/tratamento farmacológico , Deficiência de Vitamina K/etiologia
8.
Clin Cardiol ; 33(1): E26-30, 2010 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-19816973

RESUMO

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.


Assuntos
Valva Aórtica/patologia , Doença de Gaucher/complicações , Doenças das Valvas Cardíacas/patologia , Hidrocefalia/complicações , Valva Mitral/patologia , Adolescente , Valva Aórtica/cirurgia , Calcinose/patologia , Calcinose/cirurgia , Evolução Fatal , Feminino , Doença de Gaucher/diagnóstico , Doença de Gaucher/cirurgia , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/cirurgia , Humanos , Valva Mitral/cirurgia
9.
Transplant Proc ; 41(7): 2875-7, 2009 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-19765461

RESUMO

Four children underwent living related liver transplantation because of Crigler-Najjar syndrome type 1. Three were infants aged 2, 8(1/2), and 15 months, and weighed 5, 8, and 10 kg, respectively. Pretransplantation unconjugated bilirubin concentration was 22 to 30 mg/dL despite 12 to 14 hours of phototherapy daily. Patient 1, the 2-month-old infant, with unconjugated bilirubin concentration of 30 mg/dL, had a high-pitched cry, suggestive of bilirubin encephalopathy; results of neurologic examination were normal. Plasmapheresis and urgent liver transplantation were performed. Patient 4, a 13-year-old girl, had learning difficulties at school and attended a special class. Three patients received left lateral liver segments, and 1 patient received a left lobe. Biliary reconstruction was completed with duct-to-duct anastomosis. Bile leakage developed at the anastomosis in 2 patients, which was treated successfully with cholangioplasty. In all patients, the unconjugated bilirubin concentration normalized by day 1 posttransplantation, and no phototherapy was necessary. After transplantation, the 2-month-old infant with suspected encephalopathy exhibited hypotonia, spasticity of the lower extremities, and lack of head control. He died after vomitus aspiration during sleep at 10 months posttransplantation. The other 3 patients are alive with normal neurodevelopmental milestones. Irreversible brain damage may occur early in the course of Crigler-Najjar syndrome type 1. Urgent treatment including plasmapheresis, exchange transfusion, phototherapy, and liver transplantation may not reverse brain damage. Young infants must be evaluated carefully for subtle signs and symptoms of bilirubin encephalopathy. Liver transplantation is curative if performed before development of neurologic dysfunction.


Assuntos
Síndrome de Crigler-Najjar/cirurgia , Transplante de Fígado/estatística & dados numéricos , Doadores Vivos , Adolescente , Bile/metabolismo , Família , Feminino , Humanos , Lactente , Transplante de Fígado/efeitos adversos , Masculino , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/terapia , Fatores de Risco
10.
Transplant Proc ; 41(7): 2878-80, 2009 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-19765462

RESUMO

INTRODUCTION: Epstein-Barr virus (EBV) infection occurring in the postoperative period represents a significant risk for pediatric transplant recipients. It presents in various manners, including a mononucleosis-like syndrome, hepatitis, encephalopathy, or posttransplant lymphoproliferative disease (PTLD). Valacyclovir has in vitro activity against EBV. We sought to review our experience with valacyclovir on peripheral blood EBV viral loads among a group of EBV-infected patients after liver transplantation (OLT). PATIENTS AND METHODS: Twelve children of ages 6-36 months (median, 12 months), underwent OLT. Eight (66%) were EBV immunoglobulin (Ig)G seronegative at the time of the operation. Eight patients developed primary infection and 4 patients developed reactivation of a post primary infection. Valacyclovir was prescribed immediately to 3 patients when we detected an acute-primary EBV infection. Valacyclovir was prescribed for 2 patients who had primary EBV infections followed by PTLD. Three patients who had primary EBV infection were administered valacyclovir after they became chronically EBV PCR positive for more than 1 year. Four out of 12 cases (33%) were EBV seropositive at the time of OLT, and underwent postprimary EBV reactivation displaying chronic EBV carrier state for 8-10 months before valacyclovir treatment. Peripheral blood EBV viral loads were tested every 2 months. The primary outcome was the proportion of subjects with EBV viremia who had a >or=2 log 10 decrease in EBV copies/mL after valacyclovir treatment. The duration of valacyclovir treatment was a median of 10 months (range, 8-11 months). At the beginning of the treatment period the median level of EBV viral load was 1.1 x 10(4) (range, 1 x 10(4) to 1 x 10(7)). EBV virus was cleared in only 1 patient with primary acute EBV infection. EBV viral loads did not change in 7 of 12 patients and decreased only 1 log 10 (n = 2) or 2 log 10 (n = 2). CONCLUSION: In this small, non-placebo-controlled study, valacyclovir treatment was not effective to decrease peripheral blood EBV viral loads.


Assuntos
Aciclovir/análogos & derivados , Antivirais/uso terapêutico , Infecções por Vírus Epstein-Barr/tratamento farmacológico , Transplante de Fígado/fisiologia , Valina/análogos & derivados , Doença Aguda , Aciclovir/uso terapêutico , Peso Corporal , Pré-Escolar , Doença Crônica , Humanos , Lactente , Testes de Função Hepática , Complicações Pós-Operatórias/epidemiologia , Recidiva , Estudos Retrospectivos , Valaciclovir , Valina/uso terapêutico , Carga Viral/efeitos dos fármacos , Ativação Viral
11.
Transplant Proc ; 41(7): 2881-3, 2009 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-19765463

RESUMO

Posttransplant lymphoproliferative disease was first reported in 1968. Posttransplant lymphoproliferative disease encompasses a range of abnormalities from benign infectious mononucleosis-like illnesses to non-Hodgkin's lymphomas with nodal and extranodal site involvement. We evaluated five children who had posttransplant lymphoproliferative disease after liver transplantation. Since 2001, we have performed 118 liver transplantations in 115 children. Five children (4.6%), including three girls and two boys of overall mean age, 3.9 year, developed posttransplant lymphoproliferative diseases. The indications for liver transplant were hepatoblastoma in one recipient and cholestatic liver disease in the remaining four subjects. Posttransplant lymphoproliferative disease was diagnosed at 6, 11, 17, 22, and 27 months after the liver transplantation. Imaging modalities identified generalized lymphadenopathy in one, multiple liver masses in one, a large portal mass in one, multiple stomach ulcers in one, and a large mediastinal mass in one recipient. At diagnosis, the recipient with the large mediastinal mass displayed cough; the remaining four recipients were asymptomatic. Histological findings showed B-cell lymphomas in three recipients and T-cell lymphomas in two. The results of in situ hybridization for Epstein-Barr virus were negative in one recipient and positive in four. Four recipients were treated with chemotherapy; the remaining recipient was treated with anti-CD20 monoclonal antibodies. The one recipient who had a large mediastinal mass died at 2 months after receiving the diagnosis of chemotherapy-related sepsis; the remaining four children are alive at 9, 11, 18, and 34 months after treatment. Our rate of posttransplant lymphoproliferative disease was similar to that in the literature. From a few months to several years after liver transplantation, radiologists must be alert to the possibility of posttransplant lymphoproliferative disease. Thorough imaging is required to detect the wide variety of potential presentations.


Assuntos
Transplante de Fígado/efeitos adversos , Linfoma de Células B/epidemiologia , Transtornos Linfoproliferativos/epidemiologia , Adolescente , Adulto , Criança , Feminino , Seguimentos , Humanos , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/epidemiologia , Transplante de Fígado/mortalidade , Linfoma de Células B/diagnóstico por imagem , Linfoma de Células B/patologia , Masculino , Complicações Pós-Operatórias/epidemiologia , Radiografia , Taxa de Sobrevida , Sobreviventes , Fatores de Tempo , Adulto Jovem
12.
Cephalalgia ; 28(9): 945-9, 2008 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-18624809

RESUMO

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.


Assuntos
Autoanticorpos/imunologia , Autoantígenos/imunologia , Doença Celíaca/epidemiologia , Proteínas de Ligação ao GTP/imunologia , Imunoglobulina A/imunologia , Transtornos de Enxaqueca/epidemiologia , Transglutaminases/imunologia , Adolescente , Autoanticorpos/sangue , Biópsia , Doença Celíaca/sangue , Doença Celíaca/diagnóstico , Doença Celíaca/imunologia , Doença Celíaca/patologia , Criança , Comorbidade , Suscetibilidade a Doenças , Duodeno/patologia , Feminino , Humanos , Imunoglobulina A/sangue , Masculino , Transtornos de Enxaqueca/sangue , Transtornos de Enxaqueca/imunologia , Prevalência , Proteína 2 Glutamina gama-Glutamiltransferase , Risco
13.
Transplant Proc ; 40(1): 213-8, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18261590

RESUMO

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.


Assuntos
Carcinoma Hepatocelular/cirurgia , Neoplasias Hepáticas/cirurgia , Transplante de Fígado , Adolescente , Adulto , Carcinoma Hepatocelular/patologia , Criança , Pré-Escolar , Feminino , Sobrevivência de Enxerto , Humanos , Lactente , Neoplasias Hepáticas/patologia , Transplante de Fígado/mortalidade , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica , Estadiamento de Neoplasias , Estudos Retrospectivos , Análise de Sobrevida
14.
Transplant Proc ; 40(1): 228-30, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18261593

RESUMO

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.


Assuntos
Degeneração Hepatolenticular/cirurgia , Transplante de Fígado/fisiologia , Adolescente , Adulto , Cadáver , Causas de Morte , Ceruloplasmina/análise , Criança , Pré-Escolar , Família , Feminino , Seguimentos , Humanos , Transplante de Fígado/mortalidade , Doadores Vivos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Análise de Sobrevida , Doadores de Tecidos
15.
Transplant Proc ; 40(1): 231-3, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18261594

RESUMO

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.


Assuntos
Atresia Biliar/cirurgia , Transplante de Fígado/fisiologia , Perda Sanguínea Cirúrgica , Peso Corporal , Criança , Pré-Escolar , Feminino , Seguimentos , Sobrevivência de Enxerto , Humanos , Lactente , Transplante de Fígado/mortalidade , Masculino , Complicações Pós-Operatórias/classificação , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Análise de Sobrevida
16.
Pediatr Transplant ; 11(4): 402-7, 2007 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-17493220

RESUMO

An increased frequency of infections has been reported in patients with chronic liver disease. The tendency of patients in this population to acquire UTI is not completely understood. We aimed at investigating the incidence of UTI in children with cirrhosis, before liver transplantation. Twenty-six children (9 girls, 17 boys; mean age, 7.66 +/- 5.73 yr) with chronic liver disease who had undergone liver transplantation between 2002 and 2004 were included. On admission for liver transplantation, patients were examined for presence of UTI. Serum biochemistry, complete blood cell count, urinalysis and culture, glomerular filtration rate, and abdominal ultrasonography were performed prior to liver transplantation. Ten of 26 patients (38.5%) were found to have symptomatic UTI. Urine cultures revealed E. coli in five (50%), Klebsiella pneumoniae in three (30%), Enterococcus faecalis in one (10%), and Enterobacter aeruginosa in one (10%) patient(s), respectively, as etiologic factors. The etiologies of chronic liver disease in our patients with UTI were BA in five, PFIC in three, Wilson's disease in one, and alpha-1 antitrypsin deficiency in one patient. We found a significantly greater number of UTIs in patients with biliary atresia than in those without biliary atresia (p < 0.05). The mean age of the patients with UTI was 2.75 +/- 3.49 yr, which was significantly lower than in those without UTI (9.75 +/- 4.86 yr, p < 0.05). Levels for white blood cells, thrombocytes, ALT, and alkaline phosphatase were significantly higher in patients with UTI than in those without UTI. There were no significant differences between the groups with regard to serum albumin, bilirubin, AST, GGT, BUN, or creatinine levels, glomerular filtration rate, duration of disease, and PELD scores. In patients with bacteriuria, renal USG revealed normal findings in all, but except one patient who had pelvicalyceal dilatation. Scintigraphic findings demonstrated acute pyelonephritis in six (60%) patients with UTI. VCUG demonstrated vesicoureteral reflux in two patients. In conclusion, symptomatic UTI is common in children with cirrhosis. It occurs more frequently in patients with biliary atresia than it does in patients with other types of chronic liver disease. In febrile children with chronic liver disease, UTI should be considered in the differential diagnosis.


Assuntos
Hepatopatias/complicações , Transplante de Fígado , Infecções Urinárias/epidemiologia , Criança , Pré-Escolar , Doença Crônica , Enterobacter aerogenes/isolamento & purificação , Infecções por Enterobacteriaceae/complicações , Infecções por Enterobacteriaceae/epidemiologia , Infecções por Enterobacteriaceae/microbiologia , Escherichia coli/isolamento & purificação , Infecções por Escherichia coli/complicações , Infecções por Escherichia coli/epidemiologia , Infecções por Escherichia coli/microbiologia , Feminino , Humanos , Incidência , Lactente , Infecções por Klebsiella/complicações , Infecções por Klebsiella/epidemiologia , Infecções por Klebsiella/microbiologia , Klebsiella pneumoniae/isolamento & purificação , Hepatopatias/cirurgia , Masculino , Estudos Retrospectivos , Turquia/epidemiologia , Infecções Urinárias/complicações , Infecções Urinárias/microbiologia
17.
Transplant Proc ; 39(4): 1153-6, 2007 May.
Artigo em Inglês | MEDLINE | ID: mdl-17524918

RESUMO

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.


Assuntos
Transplante de Fígado/fisiologia , Anastomose Cirúrgica , Ductos Biliares/cirurgia , Peso Corporal , Pré-Escolar , Feminino , Sobrevivência de Enxerto , Hepatectomia/métodos , Humanos , Lactente , Fígado/anatomia & histologia , Hepatopatias/classificação , Hepatopatias/cirurgia , Transplante de Fígado/mortalidade , Doadores Vivos , Masculino , Tamanho do Órgão , Estudos Retrospectivos , Análise de Sobrevida , Coleta de Tecidos e Órgãos/métodos , Resultado do Tratamento
18.
Transplant Proc ; 39(4): 1157-60, 2007 May.
Artigo em Inglês | MEDLINE | ID: mdl-17524919

RESUMO

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).


Assuntos
Falência Hepática Aguda/cirurgia , Transplante de Fígado/fisiologia , Adolescente , Amônia/sangue , Cadáver , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Falência Hepática Aguda/etiologia , Transplante de Fígado/mortalidade , Doadores Vivos , Masculino , Tempo de Protrombina , Estudos Retrospectivos , Análise de Sobrevida , Doadores de Tecidos , Resultado do Tratamento
19.
Transplant Proc ; 39(4): 1171-4, 2007 May.
Artigo em Inglês | MEDLINE | ID: mdl-17524923

RESUMO

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.


Assuntos
Carcinoma Hepatocelular/cirurgia , Neoplasias Hepáticas/cirurgia , Transplante de Fígado , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Transplante de Fígado/mortalidade , Masculino , Pessoa de Meia-Idade , Seleção de Pacientes , Estudos Retrospectivos , Análise de Sobrevida , Taxa de Sobrevida
20.
Transplant Proc ; 38(10): 3576-8, 2006 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-17175335

RESUMO

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.


Assuntos
Hepatectomia/métodos , Transplante de Fígado , Fígado/anatomia & histologia , Doadores Vivos , Coleta de Tecidos e Órgãos/métodos , Tomografia Computadorizada por Raios X/normas , Algoritmos , Família , Feminino , Humanos , Fígado/diagnóstico por imagem , Masculino , Tamanho do Órgão , Estudos Retrospectivos , Segurança , Irmãos , Cônjuges
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