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1.
Dermatol Surg ; 49(1): 25-28, 2023 01 01.
Article in English | MEDLINE | ID: mdl-36533791

ABSTRACT

BACKGROUND: The h-index is a measure of research achievement using not only the number of publications of an individual, but also the impact of the publications. OBJECTIVE: The objective of this study is to evaluate the h-indices of Mohs surgeons within a variety of practice settings. MATERIALS AND METHODS: A list of all American College of Mohs Society (ACMS) members with corresponding fellowships years were collected using the ACMS membership directory. Publicly available demographic information was obtained including fellowship year, practice setting, PhD status, practice location (region), total number of publications, and h-index. Descriptive statistics were calculated to compare h-indices among the demographic data. RESULTS: A total of 1150 ACMS members were included. The Practice setting distribution was as follows: 10.6% academic, 85.7% private practice, and 3.7% combined. H-index differed significantly based on practice setting (p < .001), with higher h-indices in academic and combined settings compared with the private practice setting. Subanalysis among academic Mohs surgeons revealed higher mean h-indices among professors (23.9) > associate professors (10.6) > assistant professors (8.6) > clinical instructors (5) (p < .001). CONCLUSION: H-indices were highest among Mohs surgeons in the academic setting with increasing values correlating with higher academic rank and time since fellowship completion.


Subject(s)
Academic Success , Surgeons , Humans , United States , Fellowships and Scholarships , Efficiency
2.
Dermatol Online J ; 28(4)2022 Aug 15.
Article in English | MEDLINE | ID: mdl-36259856

ABSTRACT

Mucous membrane pemphigoid, formerly known as cicatricial pemphigoid, is a rare and difficult-to-treat bullous disorder that occurs most commonly in older adults. We describe a 32-year-old woman who was diagnosed with anti-laminin 332 mucous membrane pemphigoid through indirect immunofluorescence for laminin 332 following nonspecific histologic and direct immunofluorescence findings. At 16 weeks following completion of her first cycle of with rituximab 375mg/m2 weekly for four weeks, her mucosal erosions had resolved. Although not widely available, this case highlights the utility of anti-laminin 332 immunofluorescence for diagnostic confirmation of this entity and the efficacy of rituximab in obtaining disease control.


Subject(s)
Pemphigoid, Benign Mucous Membrane , Pemphigoid, Bullous , Humans , Female , Aged , Adult , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Benign Mucous Membrane/drug therapy , Rituximab/therapeutic use , Pemphigoid, Bullous/pathology , Autoantibodies , Mucous Membrane/pathology
3.
Clin Dermatol ; 40(6): 782-787, 2022.
Article in English | MEDLINE | ID: mdl-35948236

ABSTRACT

Lack of standardized dermatoscopy training limits confidence and accuracy. We assessed the effect of a dermatoscopy lecture series on the diagnostic accuracy of dermatology residents' biopsies. Additionally, we evaluated resident comfort with and knowledge of dermatoscopy before and after the curriculum. Twelve dermatology residents were enrolled in a 5-month dedicated dermatoscopy curriculum. To assess knowledge of and comfort with dermatoscopy, residents were given a 50-question assessment and 21-question survey before and after the curriculum. Change in diagnostic accuracy was assessed by comparing the suspected clinical diagnosis to the final histopathologic diagnosis of lesions biopsied by residents before and after the course. Upon completion of the curriculum, residents felt significantly more comfortable performing dermatoscopy (P = .002) and using dermatoscopy to identify melanocytic nevi (P = .037) and melanomas (invasive and in situ) (P = .012). Postgraduate year 2 residents also showed significantly improved diagnostic accuracy after the training course (odds ratio, 1.33; 95% confidence interval, 1.06-1.67; P = .013). Our study was limited by a small sample size of 12 residents from a single academic institution. A formal dermatoscopy course can effectively improve dermatology residents' knowledge, confidence, and diagnostic accuracy when using dermatoscopy.


Subject(s)
Dermatology , Internship and Residency , Melanoma , Nevus, Pigmented , Skin Neoplasms , Humans , Nevus, Pigmented/diagnostic imaging , Nevus, Pigmented/pathology , Melanoma/diagnostic imaging , Melanoma/pathology , Curriculum , Clinical Competence , Skin Neoplasms/diagnostic imaging , Skin Neoplasms/pathology , Case-Control Studies
4.
Plant Physiol ; 185(2): 295-317, 2021 03 15.
Article in English | MEDLINE | ID: mdl-33721892

ABSTRACT

Sugar supply is a key component of hypoxia tolerance and acclimation in plants. However, a striking gap remains in our understanding of mechanisms governing sugar impacts on low-oxygen responses. Here, we used a maize (Zea mays) root-tip system for precise control of sugar and oxygen levels. We compared responses to oxygen (21 and 0.2%) in the presence of abundant versus limited glucose supplies (2.0 and 0.2%). Low-oxygen reconfigured the transcriptome with glucose deprivation enhancing the speed and magnitude of gene induction for core anaerobic proteins (ANPs). Sugar supply also altered profiles of hypoxia-responsive genes carrying G4 motifs (sources of regulatory quadruplex structures), revealing a fast, sugar-independent class followed more slowly by feast-or-famine-regulated G4 genes. Metabolite analysis showed that endogenous sugar levels were maintained by exogenous glucose under aerobic conditions and demonstrated a prominent capacity for sucrose re-synthesis that was undetectable under hypoxia. Glucose abundance had distinctive impacts on co-expression networks associated with ANPs, altering network partners and aiding persistence of interacting networks under prolonged hypoxia. Among the ANP networks, two highly interconnected clusters of genes formed around Pyruvate decarboxylase 3 and Glyceraldehyde-3-phosphate dehydrogenase 4. Genes in these clusters shared a small set of cis-regulatory elements, two of which typified glucose induction. Collective results demonstrate specific, previously unrecognized roles of sugars in low-oxygen responses, extending from accelerated onset of initial adaptive phases by starvation stress to maintenance and modulation of co-expression relationships by carbohydrate availability.


Subject(s)
Oxygen/metabolism , Plant Proteins/genetics , Sugars/metabolism , Transcriptome , Zea mays/metabolism , Anaerobiosis , Glucose/metabolism , Glyceraldehyde-3-Phosphate Dehydrogenases/genetics , Plant Roots/genetics , Plant Roots/metabolism , Pyruvate Decarboxylase/genetics , Stress, Physiological , Zea mays/genetics
5.
J Pediatr Gastroenterol Nutr ; 72(5): 674-676, 2021 05 01.
Article in English | MEDLINE | ID: mdl-33661244

ABSTRACT

ABSTRACT: A case of low-γ-glutamyltranspetidase cholestasis associated with ubiquitin-specific peptidase 53 (USP53) gene mutation in a Brazilian child is described. Transient jaundice and hypocholia started at the age of 10 days. Liver enzymes, total bilirubin, and total bile acids were elevated at presentation. During follow-up, he developed cholelithiasis treated with cholecystectomy, and an intracranial hemorrhage resolved with full recovery. At last, evaluation at the age of 18 months, he was not jaundiced and had normal liver tests, but experienced from moderate pruritus despite treatment with rifampicin and ursodeoxycholic acid. A genetic study revealed novel homozygous mutations c.1687_1688delinsC p.Ser563Profs∗25 in the USP53 gene. His parents carried the same heterozygous mutation in the USP53 gene.


Subject(s)
Cholestasis, Intrahepatic , Cholestasis , Brazil , Child , Cholestasis/genetics , Humans , Infant , Male , Mutation , Ubiquitin-Specific Proteases/genetics
7.
Liver Transpl ; 26(12): 1644-1651, 2020 12.
Article in English | MEDLINE | ID: mdl-32852894

ABSTRACT

Pediatric living donor liver transplantation (PLDLT) is a successful therapeutic option for children with chronic and acute liver disease. After early transplant results, many technical advancements were introduced in the field to reduce the rate of complications and improve survival. The aim of this study is to present the outcomes of 975 primary PLDLTs in 3 periods: initial practice (period 1, 29 patients, January 1995 to December 1999), second period (period 2, 331 patients, January 2000 to December 2009), and third period (period 3 [P3], 615 patients, January 2010 to September 2019). Among the technical refinements introduced in P3 are the use of hyperreduced left lateral segment grafts, abdominal wall prosthetic mesh closure, double hepatic artery anastomosis, and increased use of vascular grafts for portal vein reconstruction. The outcomes included significant reductions of hepatic artery thrombosis (HAT), early portal vein thrombosis (EPVT), and retransplantation, with better patient and graft survival in P3. Additional analyses showed that the factors independently associated with worse 90-day patient survival were HAT, EPVT, and increasing Pediatric End-Stage Liver Disease score. In conclusion, the introduction of technical refinements in P3, in addition to improvements in patient care, determined a reduction in EPVT, HAT, and retransplantation. Consequently, patient and graft survival rates increased in all time points studied.


Subject(s)
End Stage Liver Disease , Liver Transplantation , Child , End Stage Liver Disease/surgery , Graft Survival , Hepatic Artery/surgery , Humans , Liver Transplantation/adverse effects , Living Donors , Retrospective Studies , Severity of Illness Index
8.
J. pediatr. (Rio J.) ; 95(4): 419-427, July-Aug. 2019. tab, graf
Article in English | LILACS | ID: biblio-1040348

ABSTRACT

Abstract Objective: This large study with a long-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis. Methods: The medical records of 828 children and adolescents with autoimmune hepatitis were reviewed. A questionnaire was used to collect anonymous data on clinical presentation, biochemical and histological findings, and treatments. Results: Of all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmune hepatitis-2. The female sex was predominant in both groups. The median age at symptom onset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis 1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and 58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher for autoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-fold higher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were significantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels were lower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochemical remission was achieved in 76.2%. The actuarial survival rate was 93.0%. A total of 4.6% underwent liver transplantation, and 6.9% died (autoimmune hepatitis-1: 7.5%; autoimmune hepatitis-2: 2.4%). Conclusions: In this large clinical series of Brazilian children and adolescents, autoimmune hepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higher disease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.


Resumo Objetivo: Este estudo com acompanhamento de longo prazo visou a avaliar o quadro clínico, os achados laboratoriais, o perfil histológico, os tratamentos e os resultados de crianças e adolescentes com hepatite autoimune. Métodos: Foram analisados os prontuários médicos de 828 crianças e adolescentes com HAI. Foi usado um questionário para coletar os dados anônimos sobre o quadro clínico, os achados bioquímicos e histológicos e os tratamentos. Resultados: De todos os pacientes, 89,6% tinham hepatite autoimune-1 e 10,4% hepatite autoimune-2. O sexo feminino foi predominante nos dois grupos. A idade média no início dos sintomas foi 111,5 (6; 210) e 53,5 (8; 165) meses nos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente. Foi observado início clínico agudo em 56,1% e 58,8% e sintomas insidiosos em 43,9% e 41,2% dos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente. A probabilidade de insuficiência hepática foi 1,6 vezes maior para hepatite autoimune-2; 3,6% e 10,6% dos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente, apresentaram insuficiência hepática fulminante; o risco foi 3,1 vezes maior para hepatite autoimune-2. Os níveis de gamaglobulina e imunoglobulina G foram significativamente maiores nos pacientes com hepatite autoimune-1, ao passo que os níveis de imunoglobulina A e C3 foram menores em pacientes com hepatite autoimune-2; 22,4% dos pacientes apresentaram cirrose e a remissão bioquímica foi atingida em 76,2%. A taxa de sobrevida atuarial foi de 93,0%. Um total de 4,6% pacientes foram submetidos a transplante de fígado e 6,9% morreram (hepatite autoimune-1: 7,5%; hepatite autoimune-2: 2,4%). Conclusões: Nesta grande série clínica de crianças e adolescentes brasileiros, a hepatite autoimune-1 foi mais frequente e os pacientes com hepatite autoimune-2 mostraram maiores taxas de remissão da doença com respostas mais rápidas aos tratamentos. Os pacientes com hepatite autoimune-1 apresentaram maior risco de óbito.


Subject(s)
Humans , Male , Female , Child , Adolescent , Azathioprine/therapeutic use , Prednisone/therapeutic use , Hepatitis, Autoimmune/pathology , Immunosuppressive Agents/therapeutic use , Autoantibodies/analysis , Biopsy, Needle , Brazil , Immunoglobulins/analysis , Magnetic Resonance Imaging , Survival Analysis , Antibodies, Antinuclear/blood , Retrospective Studies , Immunosuppression Therapy , Treatment Outcome , Hepatitis, Autoimmune/immunology , Hepatitis, Autoimmune/drug therapy , Liver/pathology
9.
J Pediatr (Rio J) ; 95(4): 419-427, 2019.
Article in English | MEDLINE | ID: mdl-29856944

ABSTRACT

OBJECTIVE: This large study with a long-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis. METHODS: The medical records of 828 children and adolescents with autoimmune hepatitis were reviewed. A questionnaire was used to collect anonymous data on clinical presentation, biochemical and histological findings, and treatments. RESULTS: Of all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmune hepatitis-2. The female sex was predominant in both groups. The median age at symptom onset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis 1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and 58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher for autoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-fold higher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were significantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels were lower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochemical remission was achieved in 76.2%. The actuarial survival rate was 93.0%. A total of 4.6% underwent liver transplantation, and 6.9% died (autoimmune hepatitis-1: 7.5%; autoimmune hepatitis-2: 2.4%). CONCLUSIONS: In this large clinical series of Brazilian children and adolescents, autoimmune hepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higher disease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.


Subject(s)
Azathioprine/therapeutic use , Hepatitis, Autoimmune/pathology , Immunosuppressive Agents/therapeutic use , Prednisone/therapeutic use , Adolescent , Antibodies, Antinuclear/blood , Autoantibodies/analysis , Biopsy, Needle , Brazil , Child , Female , Hepatitis, Autoimmune/drug therapy , Hepatitis, Autoimmune/immunology , Humans , Immunoglobulins/analysis , Immunosuppression Therapy , Liver/pathology , Magnetic Resonance Imaging , Male , Retrospective Studies , Survival Analysis , Treatment Outcome
10.
Pediatr Transplant ; 22(1)2018 02.
Article in English | MEDLINE | ID: mdl-29131462

ABSTRACT

LT exerts considerable stress on the heart perioperatively. Limited data exist on impact of cardiovascular diseases on LT children. This study evaluated the outcomes of children with CVD who underwent LT and compared with pretransplant findings. From 518 LT recipients, 82 (15.8%) had CVD. Sixty patients were classified as low-risk adjustment for congenital heart surgery 1 (RACHS 1 and 2). Five patients were classified as RACHS ≥3. The most common echocardiographic finding in the CVD patients (25/82) was ASD. CVD patients had more abnormal EKG (32.4% vs 14.5%, P < .001), abnormal chest X-ray (11.8% vs 1.4%, P < .001), and altered echocardiography (89.7% vs 15.4%, P < .001) findings compared with the No-CVD group pretransplant. Post-transplant, significant differences between groups were observed related to abnormal EKG (14.7% vs 7.0%, P = .03) and echocardiography (48.5% vs 3.2%, P < .01) findings. Pretransplant ASD spontaneously closed in 22 patients. At 1 and 5 years post-transplant, there was no difference in the survival rate between groups (P = .96). The prevalence of CVD in recipients of LT was high, and its presence was associated with significantly higher cardiac decompensation before and after LT. Minor and moderate cardiovascular disease did not impact the long-term survival.


Subject(s)
Cardiovascular Diseases/complications , End Stage Liver Disease/surgery , Liver Transplantation , Adolescent , Cardiovascular Diseases/diagnostic imaging , Cardiovascular Diseases/mortality , Child , Child, Preschool , Echocardiography , End Stage Liver Disease/complications , End Stage Liver Disease/mortality , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Kaplan-Meier Estimate , Liver Transplantation/mortality , Male , Retrospective Studies , Survival Rate , Treatment Outcome
11.
J Clin Microbiol ; 52(12): 4425-7, 2014 Dec.
Article in English | MEDLINE | ID: mdl-25275004

ABSTRACT

We describe a case of chronic hepatitis E virus (HEV) infection in a 13-year-old female liver transplant recipient with recurrent increased aminotransferase levels and acute cellular rejection. This finding demonstrates that chronic HEV infection can occur and should be further investigated in immunocompromised patients in Latin America.


Subject(s)
Hepatitis E virus/isolation & purification , Hepatitis, Chronic/diagnosis , Liver Transplantation , Transplant Recipients , Child, Preschool , Female , Graft Rejection , Hepatitis, Chronic/virology , Humans , Immunocompromised Host , Latin America , Molecular Sequence Data , RNA, Viral/genetics , Sequence Analysis, DNA , Transaminases/blood
12.
Liver Transpl ; 20(10): 1157-67, 2014 Oct.
Article in English | MEDLINE | ID: mdl-24954288

ABSTRACT

The technique of vascular reconstruction plays a major role in the outcome of living donor liver transplantation (LDLT). An increased use of vascular grafts (VGs) as replacements for sclerotic portal veins has become a standard technique for our group. The aim of this study was to analyze the factors associated with portal vein thrombosis (PVT) in pediatric LDLT. We performed a retrospective analysis of 486 primary pediatric LDLT procedures performed between October 1995 and May 2013. VGs used for portal reconstruction included living donor inferior mesenteric veins, living donor ovarian veins, recipient internal jugular veins, deceased donor iliac arteries, and deceased donor iliac veins. Thirty-four patients (7.0%) developed PVT. The incidence of PVT dropped from 10.1% to 2%; the overall utilization of VGs increased from 3.5% to 37.1%. In a multivariate analysis, only the use of VGs remained an independent risk factor for the occurrence of PVT (hazard ratio = 7.2, 95% confidence interval = 2.8-18.7, P < 0.001). There was no difference in survival rates between patients with PVT and patients without PVT. No patient with PVT underwent retransplantation. In conclusion, the use of VGs was independently associated with the development of PVT. Over time, there was a reduction in the incidence of early PVT in this cohort, and there was a trend toward a reduction in total PVT. The occurrence of isolated PVT in this study was not associated with decreased patient or graft survival.


Subject(s)
Liver Transplantation/adverse effects , Living Donors , Portal Vein , Transplant Recipients , Venous Thrombosis/etiology , Brazil/epidemiology , Child, Preschool , Female , Follow-Up Studies , Humans , Incidence , Infant , Liver Transplantation/methods , Male , Postoperative Complications , Prognosis , Retrospective Studies , Risk Factors , Survival Rate/trends , Venous Thrombosis/epidemiology
13.
Pediatr Blood Cancer ; 61(9): 1584-9, 2014 Sep.
Article in English | MEDLINE | ID: mdl-24852359

ABSTRACT

BACKGROUND: Untreated tyrosinemia type 1 (HT1) is manifested by liver failure associated with renal tubular dysfunction, growth failure, and rickets. The indication for liver transplantation (LT) is restricted to non-responders to 2-(2-nitro-4-trifluoromethylbenzoyl)-1, 3-cyclohexanedione (NTBC) treatment, patients not treated with NTBC or for patients with HCC. The aim of this study is to report on a series of NTBC naive HT1 patients submitted to LT along with the prevalence of HCC in their liver explants. PROCEDURE: This is a retrospective study of 16 children with HT1 who underwent liver transplantation between January 1993 and December 2012. CLINICAL FEATURES: liver failure in 12 (75%), growth failure in 4 (25%), rickets in 5 (31.2%), hypertrophic cardiomyopathy in three (18.7%), and renal tubulopathy in seven patients (43.7%). Median AFP level was 64,335 ng/ml. Abdominal CT scans showed multiple nodules in most patients. Histopathology of the explants showed cirrhosis in all patients and HCC in 12 (75%), 3 with microvascular invasion. The majority of the tumors were well differentiated. Patient survival rate was 86% at a median follow-up of 6.6 years. All survivors were tumor-free with no adjuvant chemotherapy. CONCLUSION: In countries where neonatal screening programs are not effective and NTBC treatment is not widely available, LT still plays an important role in the treatment of children with HT1. An early indication in patients who present with multinodular livers can also serve to treat an otherwise underdiagnosed HCC condition.


Subject(s)
Carcinoma, Hepatocellular/pathology , Drug Resistance , Liver Neoplasms/pathology , Liver Transplantation , Liver/pathology , Tyrosinemias/pathology , Carcinoma, Hepatocellular/epidemiology , Carcinoma, Hepatocellular/therapy , Child , Child, Preschool , Cyclohexanones/pharmacology , Enzyme Inhibitors/pharmacology , Female , Follow-Up Studies , Humans , Infant , Liver Neoplasms/epidemiology , Liver Neoplasms/therapy , Male , Nitrobenzoates/pharmacology , Prognosis , Retrospective Studies , Tomography, X-Ray Computed , Tyrosinemias/therapy
14.
Liver Transpl ; 20(8): 882-92, 2014 Aug.
Article in English | MEDLINE | ID: mdl-24760734

ABSTRACT

The incidence of biliary complications (BCs) after living donor liver transplantation (LDLT) can reach 40%. Published data on the pediatric population are limited, and treatment protocols vary. Our aim was to describe the clinical scenario for BCs and treatment approaches after LDLT. Between October 1995 and December 2012, 489 pediatric LDLT procedures were performed. BCs developed in 71 patients (14.5%). Biliary strictures (BSs) developed in 45 (9.2%) patients, and bile leaks (BLs) developed in 33 patients (6.7%). The BL diagnosis was clinical in all cases, and 69.7% of the patients underwent surgery. Nearly half of the BS cases had clinical features or suggestive ultrasound findings. Liver biopsy findings suggested BSs in 51.7%. Percutaneous transhepatic cholangiography was performed in 95.6% of the BS patients. The success rate was 77% [mean number of percutaneous biliary interventions (PBIs) = 3.9 ± 1.98, median drainage time = 8 months]. In conclusion, BL patients can be managed with conservative therapy, even though most of these patients will ultimately be treated with surgery. Diagnosing a BS requires a high degree of clinical suspicion because the available resources for its identification can fail in up to 50% of cases. A higher number of PBIs and the use of a drainage catheter for a longer time may be required to achieve better results with this technique.


Subject(s)
Constriction, Pathologic/diagnosis , Liver Transplantation , Liver/surgery , Adolescent , Alagille Syndrome/therapy , Biliary Atresia/therapy , Biliary Tract Surgical Procedures/adverse effects , Child , Child, Preschool , Cholangiography , Constriction, Pathologic/etiology , Drainage/methods , End Stage Liver Disease/mortality , End Stage Liver Disease/surgery , Fibrosis/therapy , Hepatitis, Autoimmune/therapy , Humans , Infant , Kaplan-Meier Estimate , Liver Failure, Acute/therapy , Living Donors , Male , Postoperative Complications/etiology , Retrospective Studies , Transplant Recipients , Tyrosinemias/therapy
15.
Hepatology ; 59(5): 1964-71, 2014 May.
Article in English | MEDLINE | ID: mdl-24122953

ABSTRACT

UNLABELLED: Ascites is the most common complication of cirrhosis and in adults it is associated with 50% mortality at 5 years if patients do not receive a liver transplant. The occurrence of hyponatremia in these patients has been associated with increased mortality on the waiting list. The importance of serum sodium levels and the presence of ascites in the pediatric setting remain to be clarified. A retrospective analysis of pediatric patients with cirrhosis on the transplant list was carried out between October 2000 and February 2012. The primary objective of this study was to evaluate the association of pretransplant variables with mortality within 90 days following the inclusion of patients on the waiting list. In all, 522 patients were included in the study; 345 (66%) patients were under 1 year of age; 208 (40%) of the children presented ascites. A multivariate Cox proportional hazards analysis was conducted and total bilirubin (P < 0.001, hazard ratio [HR] = 2.09, 95% confidence interval [CI] = 1.35-3.21), international normalized ratio (INR) (P < 0.001, HR = 9.83, 95% CI = 4.51-21.45), serum sodium levels (P = 0.03, HR = 0.96, 95% CI = 0.92-0.99), ascites (P = 0.001, HR = 2.59, 95% CI = 1.44-4.64), and categorized age (0-1 versus ≥ 1 year old) (P = 0.025, HR = 2.33, 95% CI = 1.11-4.86) were independently associated with risk of death in 90 days. Malnutrition (Z score height/age, weight/age) and serum albumin (pediatric endstage liver disease [PELD] formula) were not included in the final model. CONCLUSION: The presence of ascites and serum sodium levels are important variables associated with decreased patient survival while candidates wait for a liver graft. Multicenter studies are necessary to validate these findings in order to improve current allocation policies based on the PELD score.


Subject(s)
Ascites/mortality , End Stage Liver Disease/mortality , Liver Transplantation , Sodium/blood , Waiting Lists , Adolescent , Ascites/etiology , Biomarkers/blood , Child , Child, Preschool , End Stage Liver Disease/complications , End Stage Liver Disease/surgery , Female , Humans , Infant , Living Donors , Male , Proportional Hazards Models , Retrospective Studies
16.
Liver Transpl ; 19(4): 431-6, 2013 Apr.
Article in English | MEDLINE | ID: mdl-23447137

ABSTRACT

The vascular anomalies encountered in patients with biliary atresia associated with polysplenia syndrome and situs inversus (SI) demand technical refinements when liver transplantation is being performed. The available data describing the technique used in living donor liver transplantation (LDLT) in this population are limited; the short vascular stumps of the donor's liver can impart additional technical difficulties during vascular reconstruction. Here we describe our experience with 9 children with biliary atresia and SI who underwent LDLT. In our series, the retrohepatic vena cava was absent for 7 patients, 7 had a preduodenal portal vein (PV), and 4 had a variant arterial anatomy. The donor's left hepatic vein was anastomosed to the confluence of the recipient's 3 hepatic veins in 7 patients. Vascular grafts were used for PV reconstruction in 3 cases. A left lateral segment graft was used in all but 1 patient who needed a graft reduction. All grafts were placed in the upper left abdomen. There were no vascular complications after transplantation. All patients were alive and well at a median follow-up of 55 months. In conclusion, LDLT can be successfully performed in pediatric patients with SI. Complex vascular anomalies associated with the use of partial liver grafts obtained from living donors are not associated with an increased occurrence of vascular complications.


Subject(s)
Biliary Atresia/surgery , Liver Transplantation/methods , Living Donors , Situs Inversus/surgery , Vascular Grafting , Vascular Malformations/surgery , Age Factors , Biliary Atresia/complications , Female , Hepatic Artery/abnormalities , Hepatic Artery/surgery , Humans , Infant , Liver Transplantation/adverse effects , Male , Portal Vein/abnormalities , Portal Vein/surgery , Retrospective Studies , Situs Inversus/complications , Treatment Outcome , Vascular Grafting/adverse effects , Vascular Malformations/complications , Vena Cava, Inferior/abnormalities , Vena Cava, Inferior/surgery
17.
Liver Transpl ; 19(3): 322-7, 2013 Mar.
Article in English | MEDLINE | ID: mdl-23239564

ABSTRACT

The association of biliary atresia (BA) with congenital heart diseases has been extensively described, and there are a number of reports on the outcomes of patients in this group who undergo liver transplantation (LT). The intraoperative management and the timing of LT for patients with end-stage liver disease are matters of debate, especially when complex heart diseases are involved. This report describes the outcome after LT for a pediatric recipient with BA and hypoplastic left heart syndrome. The patient underwent Norwood-Sano and Glenn procedures for heart palliation before LT. He was cyanotic, was severely malnourished, and had complications secondary to chronic liver failure. At the time of transplantation, the child was 16 months old and weighed 5175 g. Despite the critical clinical scenario and the long hospitalization period, there were no cardiac, vascular, or biliary complications after LT. At the age of 48 months, the patient was awaiting the final cardiac repair. In conclusion, the presence of complex cardiac malformations may not be a contraindication to LT. An experienced surgical team and a multidisciplinary approach are key to a successful outcome.


Subject(s)
Biliary Atresia/surgery , End Stage Liver Disease/surgery , Fontan Procedure , Hypoplastic Left Heart Syndrome/surgery , Liver Transplantation , Biliary Atresia/complications , Cyanosis/etiology , End Stage Liver Disease/etiology , Humans , Hypoplastic Left Heart Syndrome/complications , Infant , Infant Nutrition Disorders/etiology , Male , Palliative Care , Time Factors , Treatment Outcome
18.
Liver Transpl ; 18(5): 577-84, 2012 May.
Article in English | MEDLINE | ID: mdl-22271646

ABSTRACT

The availability of living donors allows transplant teams to indicate living donor liver transplantation (LDLT) early in the course of liver disease before the occurrence of life-threatening complications. Late referral to transplant centers is still a problem and can compromise the success of the procedure. The aim of this study was to examine the perioperative factors associated with patient and graft survival for 430 consecutive pediatric LDLT procedures at Sirio-Libanes Hospital/A. C. Camargo Hospital (São Paulo, Brazil) between October 1995 and April 2011. The studied pretransplant variables included the following: recipient age and body weight, Pediatric End-Stage Liver Disease score, z score for height/age, bilirubin, albumin, international normalized ratio, hemoglobin, sodium, presence of ascites, and previous surgery. The analyzed technical aspects included the graft-to-recipient weight ratio and the use of vascular grafts for portal vein reconstruction. In addition, the occurrence of hepatic artery thrombosis (HAT), portal vein thrombosis (PVT), and biliary complications was also analyzed. The liver grafts included 348 left lateral segments, 5 monosegments, 51 left lobes, and 9 right lobes. In a univariate analysis, an age < 12 months, a low body weight (≤10 kg), malnutrition, hyperbilirubinemia, and HAT were associated with decreased patient and graft survival after LDLT. In a multivariate analysis, a body weight ≤ 10 kg and HAT were significantly associated with decreased patient and graft survival. The use of vascular grafts significantly increased the occurrence of PVT. In conclusion, a low body weight (≤10 kg) and the occurrence of HAT independently determined worse patient and graft survival in this large cohort of pediatric LDLT patients.


Subject(s)
Graft Survival , Liver Transplantation/mortality , Living Donors , Adolescent , Adult , Body Weight , Female , Hepatic Artery , Humans , Liver Transplantation/adverse effects , Male , Middle Aged , Multivariate Analysis , Portal Vein , Thrombosis/mortality , Venous Thrombosis/mortality
19.
Liver Transpl ; 17(11): 1299-303, 2011 Nov.
Article in English | MEDLINE | ID: mdl-21504049

ABSTRACT

The increasing number of transplants performed each year has led to the identification of unusual diseases in liver grafts from asymptomatic donors that were unrecognized before liver transplantation. Here we report our experience with patients who received liver grafts infected with schistosomiasis. From September 1991 to August 2010, 482 pediatric liver transplants were performed at A. C. Camargo Hospital/Sírio-Libanês Hospital (São Paulo, Brazil). For the identification of Schistosoma mansoni infections, pathology slides for the recipients were reviewed; these included postreperfusion and follow-up liver biopsy samples. We were able to identify 6 cases of schistosomiasis transmitted through infected grafts (5 of these grafts were from living donors). All living donors were confirmed to have normal liver chemistries, negative fecal tests for parasitic diseases, and normal abdominal ultrasound findings. Liver biopsy was not performed before transplantation. In all cases, features of schistosomiasis were absent in the liver explants. The living donors were treated with praziquantel and were taught to avoid risk factors for reinfection. No specific treatment for schistosomiasis was given to the recipients. There were no perioperative deaths, but 2 recipients died after living donor liver transplantation (LDLT) because of Kaposi's sarcoma and non-Hodgkin's lymphoma. In conclusion, using liver grafts infected with S. mansoni eggs did not compromise the results of LDLT in this pediatric cohort. Because of the parasite's life cycle and the therapeutic target of praziquantel, only donors should be treated for the infection. Three years of follow-up showed an uneventful recovery for the living donors.


Subject(s)
Liver Failure/parasitology , Liver Failure/surgery , Liver Transplantation , Schistosomiasis mansoni/surgery , Biopsy , Brazil , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Liver/parasitology , Liver/pathology , Liver/surgery , Liver Failure/pathology , Male , Retrospective Studies , Schistosomiasis mansoni/pathology , Tissue Donors , Treatment Outcome
20.
Liver Transpl ; 16(4): 426-30, 2010 Apr.
Article in English | MEDLINE | ID: mdl-20213836

ABSTRACT

The Pediatric End-Stage Liver Disease (PELD) scoring system is a formula developed to provide a continuous numerical assessment of the risk of death in order to allocate livers to children in need of transplantation. The PELD scoring system was introduced in Brazil in July 2006. An important change was made in the system: the final number for listing patients less than 12 years old for transplantation was the calculated PELD score multiplied by 3. The consequences of this allocation policy were analyzed in 2 ways in this research: nationally and in the state of São Paulo (SP State). In the analysis of the national data, a comparison of the pre-PELD era (July 2003 to July 2006) and the post-PELD era (August 2006 to April 2009) showed that the total number of pediatric transplants for children under 12 years of age decreased 7%. Regionally, in SP State, there was a 62% increase in the number of deceased donor liver transplantation procedures for the pediatric population after the introduction of the modified PELD system. There was also a 6.1-fold increase in split liver transplantation as well as a statistically significant decrease in the time on the waiting list (P < 0.001). In conclusion, changing the allocation policy in Brazil in order to benefit pediatric patients on the waiting list had different results according to analyses of national and regional data. A significant increase in deceased donor liver transplantation/split liver transplantation and a shorter time on the waiting list were observed in SP State. The modified PELD scoring system is simple and optimizes the utilization of deceased donor liver grafts in centers performing pediatric transplants.


Subject(s)
Liver Diseases/diagnosis , Liver Diseases/therapy , Liver Transplantation/methods , Adolescent , Adult , Brazil , Child , Geography , Humans , Pediatrics/methods , Severity of Illness Index , Time Factors , Transplantation, Homologous/methods
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