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1.
Rev Port Cir Cardiotorac Vasc ; 27(3): 217-219, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33068513

RESUMO

A 19 years-old woman, on her 17th week of pregnancy presented to the emergency department with thoracic pain and vomiting. An empyema was diagnosed and she was transferred to a tertiary hospital for treatment. After drainage of the empyema a mediastinal mass was detected and a thoracic MRI revealed a multicystic lesion of the anterior mediastinum, causing cardiac and left lung compression, suggestive of a complicated teratoma. After a multidisciplinary discussion involving pulmonology, radiology, obstetrics and thoracic surgery, she was operated successfully by clamshell incision. A mature complicated teratoma was resected and a left pleurectomy/decortication performed. She was discharged on day 17 with no obstetrical or respiratory symptoms..


Assuntos
Neoplasias do Mediastino , Complicações Neoplásicas na Gravidez , Teratoma , Cirurgia Torácica , Dor no Peito , Feminino , Humanos , Neoplasias do Mediastino/complicações , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/cirurgia , Gravidez , Radiografia , Teratoma/complicações , Teratoma/diagnóstico por imagem , Teratoma/cirurgia , Adulto Jovem
2.
Arch. argent. pediatr ; 115(6): 454-457, dic. 2017.
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-887413

RESUMO

El incremento del amonio en sangre, hiperamoniemia, es pasible de provocar compromiso neurológico al atravesar la barrera hematoencefálica. La causa más frecuente y conocida de hiperamoniemia es la alteración en la función hepática. Sin embargo, se deben considerar otras patologías, de menor frecuencia y poco conocidas. La infección del tracto urinario por gérmenes productores de ureasa debe ser contemplada a pesar de ser infrecuente en pediatría. Se reporta el caso de un niño con encefalopatía aguda grave, con niveles elevados de amonio en sangre, en quien, luego de descartar otros diagnósticos diferenciales, se asumió el cuadro como hiperamoniemia secundaria a infección del tracto urinario por Corynebacterium riegelii, un germen productor de ureasa. Se implementaron medidas generales de tratamiento para la encefalopatía hiperamoniémica y tratamiento antibiótico específico, con buena evolución el paciente.


Elevated level of ammonia in the blood, defined as hyperammonemia, is feasible to cause neurological symptoms when crossing the blood-brain barrier. The most frequent and studied cause of hyperammonemia is liver failure. Nevertheless, other less frequent and known etiologies must be considered. Urinary tract infection caused by urea-splitting bacteria, despite being unusual in pediatric patients, must be taken into account. We report a pediatric patient with severe acute encephalopathy and high levels of ammonia in blood. After ruling out other causes of hyperammonemia, it was assumed secondary to urinary tract infection by Corynebacterium riegelii, a ureasplitting bacteria. General treatment for hyperammonemic encephalopathy was established, as well as specific treatment with antibiotics. The patient evolved favorably.


Assuntos
Humanos , Masculino , Pré-Escolar , Ureia/metabolismo , Infecções Urinárias/complicações , Encefalopatias Metabólicas/etiologia , Infecções por Corynebacterium/complicações , Hiperamonemia/etiologia , Infecções Urinárias/microbiologia , Infecções por Corynebacterium/metabolismo
3.
Arch Argent Pediatr ; 115(6): e454-e457, 2017 Dec 01.
Artigo em Espanhol | MEDLINE | ID: mdl-29087134

RESUMO

Elevated level of ammonia in the blood, defined as hyperammonemia, is feasible to cause neurological symptoms when crossing the blood-brain barrier. The most frequent and studied cause of hyperammonemia is liver failure. Nevertheless, other less frequent and known etiologies must be considered. Urinary tract infection caused by urea-splitting bacteria, despite being unusual in pediatric patients, must be taken into account. We report a pediatric patient with severe acute encephalopathy and high levels of ammonia in blood. After ruling out other causes of hyperammonemia, it was assumed secondary to urinary tract infection by Corynebacterium riegelii, a ureasplitting bacteria. General treatment for hyperammonemic encephalopathy was established, as well as specific treatment with antibiotics. The patient evolved favorably.


El incremento del amonio en sangre, hiperamoniemia, es pasible de provocar compromiso neurológico al atravesar la barrera hematoencefálica. La causa más frecuente y conocida de hiperamoniemia es la alteración en la función hepática. Sin embargo, se deben considerar otras patologías, de menor frecuencia y poco conocidas. La infección del tracto urinario por gérmenes productores de ureasa debe ser contemplada a pesar de ser infrecuente en pediatría. Se reporta el caso de un niño con encefalopatía aguda grave, con niveles elevados de amonio en sangre, en quien, luego de descartar otros diagnósticos diferenciales, se asumió el cuadro como hiperamoniemia secundaria a infección del tracto urinario por Corynebacterium riegelii, un germen productor de ureasa. Se implementaron medidas generales de tratamiento para la encefalopatía hiperamoniémica y tratamiento antibiótico específico, con buena evolución el paciente.


Assuntos
Encefalopatias Metabólicas/etiologia , Infecções por Corynebacterium/complicações , Hiperamonemia/etiologia , Ureia/metabolismo , Infecções Urinárias/complicações , Pré-Escolar , Infecções por Corynebacterium/metabolismo , Humanos , Masculino , Infecções Urinárias/microbiologia
7.
Arch Argent Pediatr ; 111(1): e31-4, 2013.
Artigo em Espanhol | MEDLINE | ID: mdl-23381715

RESUMO

Continuous infusion of vancomycin may be a strategy for critically ill patients who do not achieve adequate plasma levels. There is few literature on this dosage regimen. We present six children (2 months to 7 years, 4 male and 2 female), admitted to the Intensive Care Unit of the "Prof. Dr. Garrahan Children Hospital", with methicillin-resistant Staphylococcus aureus sepsis, treated with vancomycin 40 and 60 mg/kg/day every 8-6 hrs. Continuous infusion at 50 mg/kg/day was implemented due to poor outcome, persistent fever, positive cultures and inadequate vancomycin plasma levels. All patients achieved levels between 10 and 25 ug/ml, their outcome was favorable and cultures became negative, with no signs of nephrotoxicity. Treatment duration of the continuous infusion was 9 to 18 days. Continuous infusion of vancomycin was effective in these patients without evidence of associated nephrotoxicity.


Assuntos
Antibacterianos/administração & dosagem , Vancomicina/administração & dosagem , Antibacterianos/sangue , Criança , Cuidados Críticos , Feminino , Humanos , Lactente , Infusões Intravenosas , Masculino , Vancomicina/sangue
8.
Arch. argent. pediatr ; 111(1): e31-e34, Feb. 2013. graf
Artigo em Espanhol | LILACS | ID: lil-663657

RESUMO

La administración de vancomicina en infusión continua es una estrategia de tratamiento posible en pacientes críticos que no alcancen niveles plasmáticos adecuados. Existe escasa bibliografía acerca de este tipo de administración. Se presentan 6 niños (2 meses a 7 años; 4 varones y 2 mujeres) que ingresaron en la unidad de cuidados intensivos del Hospital de Pediatría Garrahan con un cuadro clínico de sepsis por Staphylococcus aureus resistente a la meticilina, tratados con vancomicina, en dosis de entre 40 y 60 mg/kg/día cada 8-6 horas. Debido a la evolución clínica no favorable, la persistencia de la fiebre, los cultivos positivos y los niveles plasmáticos del antibiótico insuficientes, se implementó la infusión continua a 50 mg/kg/día. Todos los pacientes alcanzaron niveles entre 10 y 25 µg/ml, evolucionaron favorablemente y negativizaron los cultivos, sin signos de nefrotoxicidad. El tiempo de tratamiento en infusión continua fue entre 9 y 18 días. La infusión continua de vancomicina fue eficaz en estos pacientes, sin evidencias de nefrotoxicidad asociada.


Continuous infusion of vancomycin may be a strategy for critically ill patients who do not achieve adequate plasma levels. There is few literature on this dosage regimen. We present six children (2 months to 7 years, 4 male and 2 female), admitted to the Intensive Care Unit of the "Prof. Dr. Garrahan Children Hospital", with methicillin-resistant Staphylococcus aureus sepsis, treated with vancomycin 40 and 60 mg/kg/day every 8-6 hrs. Continuous infusion at 50 mg/kg/day was implemented due to poor outcome, persistent fever, positive cultures and inadequate vancomycin plasma levels. All patients achieved levels between 10 and 25 ug/ml, their outcome was favorable and cultures became negative, with no signs of nephrotoxicity. Treatment duration of the continuous infusion was 9 to 18 days. Continuous infusion of vancomycin was effective in these patients without evidence of associated nephrotoxicity.


Assuntos
Criança , Feminino , Humanos , Lactente , Masculino , Antibacterianos/administração & dosagem , Vancomicina/administração & dosagem , Antibacterianos/sangue , Cuidados Críticos , Infusões Intravenosas , Vancomicina/sangue
9.
Arch. argent. pediatr ; 111(1): e31-e34, feb. 2013. graf
Artigo em Espanhol | BINACIS | ID: bin-131162

RESUMO

La administración de vancomicina en infusión continua es una estrategia de tratamiento posible en pacientes críticos que no alcancen niveles plasmáticos adecuados. Existe escasa bibliografía acerca de este tipo de administración. Se presentan 6 niños (2 meses a 7 años; 4 varones y 2 mujeres) que ingresaron en la unidad de cuidados intensivos del Hospital de Pediatría Garrahan con un cuadro clínico de sepsis por Staphylococcus aureus resistente a la meticilina, tratados con vancomicina, en dosis de entre 40 y 60 mg/kg/día cada 8-6 horas. Debido a la evolución clínica no favorable, la persistencia de la fiebre, los cultivos positivos y los niveles plasmáticos del antibiótico insuficientes, se implementó la infusión continua a 50 mg/kg/día. Todos los pacientes alcanzaron niveles entre 10 y 25 Ag/ml, evolucionaron favorablemente y negativizaron los cultivos, sin signos de nefrotoxicidad. El tiempo de tratamiento en infusión continua fue entre 9 y 18 días. La infusión continua de vancomicina fue eficaz en estos pacientes, sin evidencias de nefrotoxicidad asociada.(AU)


Continuous infusion of vancomycin may be a strategy for critically ill patients who do not achieve adequate plasma levels. There is few literature on this dosage regimen. We present six children (2 months to 7 years, 4 male and 2 female), admitted to the Intensive Care Unit of the "Prof. Dr. Garrahan Children Hospital", with methicillin-resistant Staphylococcus aureus sepsis, treated with vancomycin 40 and 60 mg/kg/day every 8-6 hrs. Continuous infusion at 50 mg/kg/day was implemented due to poor outcome, persistent fever, positive cultures and inadequate vancomycin plasma levels. All patients achieved levels between 10 and 25 ug/ml, their outcome was favorable and cultures became negative, with no signs of nephrotoxicity. Treatment duration of the continuous infusion was 9 to 18 days. Continuous infusion of vancomycin was effective in these patients without evidence of associated nephrotoxicity.(AU)


Assuntos
Criança , Feminino , Humanos , Lactente , Masculino , Antibacterianos/administração & dosagem , Vancomicina/administração & dosagem , Antibacterianos/sangue , Cuidados Críticos , Infusões Intravenosas , Vancomicina/sangue
10.
Arch Argent Pediatr ; 111(1): e31-4, 2013 Jan-Feb.
Artigo em Espanhol | BINACIS | ID: bin-133189

RESUMO

Continuous infusion of vancomycin may be a strategy for critically ill patients who do not achieve adequate plasma levels. There is few literature on this dosage regimen. We present six children (2 months to 7 years, 4 male and 2 female), admitted to the Intensive Care Unit of the "Prof. Dr. Garrahan Children Hospital", with methicillin-resistant Staphylococcus aureus sepsis, treated with vancomycin 40 and 60 mg/kg/day every 8-6 hrs. Continuous infusion at 50 mg/kg/day was implemented due to poor outcome, persistent fever, positive cultures and inadequate vancomycin plasma levels. All patients achieved levels between 10 and 25 ug/ml, their outcome was favorable and cultures became negative, with no signs of nephrotoxicity. Treatment duration of the continuous infusion was 9 to 18 days. Continuous infusion of vancomycin was effective in these patients without evidence of associated nephrotoxicity.


Assuntos
Antibacterianos/administração & dosagem , Vancomicina/administração & dosagem , Antibacterianos/sangue , Criança , Cuidados Críticos , Feminino , Humanos , Lactente , Infusões Intravenosas , Masculino , Vancomicina/sangue
11.
Arch Argent Pediatr ; 110(6): e114-7, 2012 Dec.
Artigo em Espanhol | MEDLINE | ID: mdl-23224314

RESUMO

Postpneumonectomy syndrome is a rare entity in children. We report a case secondary to pneumonectomy performed due to an intrathoracic tumor. A 4-year-old boy with a history of right pneumonectomy and progressive respiratory distress, with extrinsic airway obstruction due to displaced mediastinal structures. There were failure to intubate the airway with endoluminal devices and impossibility of withdrawing from mechanical ventilation. As mediastinal repositioning was not possible, an aortic bypass with a prosthetic tube graft was performed, with favorable outcome and hospital discharge.


Assuntos
Hérnia/etiologia , Pneumopatias/etiologia , Doenças do Mediastino/etiologia , Pneumonectomia/efeitos adversos , Pré-Escolar , Humanos , Masculino , Síndrome
12.
Arch. argent. pediatr ; 110(6): e114-e117, dic. 2012. ilus
Artigo em Espanhol | LILACS | ID: lil-662134

RESUMO

El síndrome postneumonectomía es una entidad poco frecuente en pediatría. Comunicamos un caso secundario a una neumonectomía realizada por un tumor intratorácico. Niño de 4 años con antecedente de exéresis pulmonar derecha y dificultad respiratoria progresiva, con obstrucción extrínseca de la vía aérea por estructuras mediastínicas desplazadas. Hubo intentos frustros de tutorización con dispositivos endoluminales de la vía aérea, e imposibilidad de retiro de la asistencia ventilatoria mecánica. Ante la imposibilidad de reposicionamiento mediastinal, se realiza puente (bypass) aórtico con tubo protésico, con evolución favorable y egreso hospitalario.


Postpneumonectomy syndrome is a rare entity in children. We report a case secondary to pneumonectomy performed due to an intrathoracic tumor. A 4-year-old boy with a history of right pneumonectomy and progressive respiratory distress, with extrinsic airway obstruction due to displaced mediastinal structures. There were failure to intubate the airway with endoluminal devices and impossibility of withdrawing from mechanical ventilation. As mediastinal repositioning was not possible, an aortic bypass with a prosthetic tube graft was performed, with favorable outcome and hospital discharge.


Assuntos
Pré-Escolar , Humanos , Masculino , Hérnia/etiologia , Pneumopatias/etiologia , Doenças do Mediastino/etiologia , Pneumonectomia/efeitos adversos , Síndrome
13.
Arch. argent. pediatr ; 110(6): e114-e117, dic. 2012. ilus
Artigo em Espanhol | BINACIS | ID: bin-129069

RESUMO

El síndrome postneumonectomía es una entidad poco frecuente en pediatría. Comunicamos un caso secundario a una neumonectomía realizada por un tumor intratorácico. Niño de 4 años con antecedente de exéresis pulmonar derecha y dificultad respiratoria progresiva, con obstrucción extrínseca de la vía aérea por estructuras mediastínicas desplazadas. Hubo intentos frustros de tutorización con dispositivos endoluminales de la vía aérea, e imposibilidad de retiro de la asistencia ventilatoria mecánica. Ante la imposibilidad de reposicionamiento mediastinal, se realiza puente (bypass) aórtico con tubo protésico, con evolución favorable y egreso hospitalario.(AU)


Postpneumonectomy syndrome is a rare entity in children. We report a case secondary to pneumonectomy performed due to an intrathoracic tumor. A 4-year-old boy with a history of right pneumonectomy and progressive respiratory distress, with extrinsic airway obstruction due to displaced mediastinal structures. There were failure to intubate the airway with endoluminal devices and impossibility of withdrawing from mechanical ventilation. As mediastinal repositioning was not possible, an aortic bypass with a prosthetic tube graft was performed, with favorable outcome and hospital discharge.(AU)


Assuntos
Pré-Escolar , Humanos , Masculino , Hérnia/etiologia , Pneumopatias/etiologia , Doenças do Mediastino/etiologia , Pneumonectomia/efeitos adversos , Síndrome
14.
Acta Med Port ; 25(6): 448-53, 2012.
Artigo em Português | MEDLINE | ID: mdl-23534598

RESUMO

Systemic lupus erythematosus is a chronic inflammatory disease, resulting from an auto-immune dysfunction. The etiology of this disease is unknown. It frequently occurs in women of childbearing age. Pregnancy in patients with systemic lupus erythematosus may be associated with several complications (maternal, obstetrical and fetal). The prognosis for both mother and child is better when systemic lupus erythematosus has been quiescent for at least six months before pregnancy. Thus, preconceptional assessment and management is crucial for helping women to achieve a period of disease remission before pregnancy as well as for allowing an adjustment of therapy. Maternal health and fetal development should be closely monitored during pregnancy. These patients should be surveilled by a multidisciplinary team (obstetrician, rheumatologist or internist, nephrologist if necessary and a pediatrician), in a tertiary care hospital. Antiphospholipid syndrome, positivity for anti-SSA/Ro or anti-SSB/LA antibodies, hypertension or renal involvement are associated with an increase of adverse pregnancy outcomes. In this article the authors review the main aspects of Systemic lupus erythematosus (SLE) and pregnancy.


Assuntos
Lúpus Eritematoso Sistêmico , Complicações na Gravidez , Feminino , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/terapia , Gravidez , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/terapia
15.
Pediatr Crit Care Med ; 12(3): e136-40, 2011 May.
Artigo em Inglês | MEDLINE | ID: mdl-20431501

RESUMO

OBJECTIVES: To describe the clinical features of patients admitted to the pediatric intensive care unit (PICU) with acute lower respiratory tract infection (LRTI) attributable to influenza A pH1N1 virus and compare them with those admitted with LRTI due to other viral pathogens. DESIGN: Retrospective, observational, comparative study. SETTING: PICU in a university-affiliated, tertiary-care, pediatric hospital. PATIENTS: Patients aged >1 month with acute viral LRTI admitted to the PICU who met the following criteria: 1) influenza A pH1N1 virus infection detected between June 1, 2009 and July 16, 2009; and 2) patients with LRTI due to other viral pathogens infection detected in the same period of the year 2008 plus patients with 2009 influenza A non-pH1N1 infection. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We evaluated age, weight, gender, length of hospital stay before PICU admission, length of PICU stay, underlying disease, clinical diagnosis, severity, complications, treatment, risk of death, mortality, and cause of death. We identified 30 patients with acute LRTI due to influenza A pH1N1 virus (group 1) and 62 patients with LRTI due to other viral pathogens (group 2). Relevant characteristics in group 1: 59% of patients were aged >2 yrs. Median length of PICU stay was 15 days. An underlying condition was present in 83% of the patients. None of them had bronchiolitis on PICU admission; 12 (40%) had bilateral pneumonia; 15 (50%) had acute respiratory distress syndrome; and nine (30%) had shock. Twenty-nine patients required mechanical ventilation (96.6%); only one required extracorporeal membrane oxygenation; and 19 (63%) had respiratory complications. Six (20%) children died. When both groups were compared, patients with influenza A pH1N1 infection were older; pneumonia, acute respiratory distress syndrome, and shock were more frequently seen at the time of PICU admission, although length of stay and mortality were similar. CONCLUSIONS: Patients with influenza A pH1N1 respiratory virus infection presented with more severe illness. Outcomes were similar in both groups.


Assuntos
Hospitalização , Vírus da Influenza A Subtipo H1N1 , Influenza Humana/fisiopatologia , Unidades de Terapia Intensiva Pediátrica , Sistema Respiratório/virologia , Adolescente , Criança , Pré-Escolar , Feminino , Hospitais Pediátricos , Humanos , Lactente , Recém-Nascido , Influenza Humana/virologia , Masculino , Estudos Retrospectivos
16.
Gynecol Obstet Invest ; 68(4): 272-5, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19797903

RESUMO

AIM: To evaluate the success rate and the safety profile of labor induction with a new misoprostol formulation - vaginal capsules of 25 microg of misoprostol. METHODS: Labor induction was performed in 250 singleton term pregnancies; 149 (59.6%) were nulliparous. Vaginal capsules of 25 microg of misoprostol were placed in the posterior vaginal fornix every 6 h. Success rate, contractility and fetal heart rate abnormalities and fetal outcomes were evaluated. RESULTS: The success rate of labor induction was 97.6%. The average number of vaginal administrations was 1.5. The mean interval between induction and active labor was 10 h and 20 min and the average length of labor was 15 h and 35 min. The cesarean section rate was 18.8%. There were 15 cases of tachysystole, 3 cases of hypertonus and 1 case of hyperstimulation syndrome. There were no adverse neonatal outcomes. CONCLUSIONS: This study allowed to conclude that labor induction with vaginal capsules of 25 microg of misoprostol is associated with an excellent success rate and safety profile.


Assuntos
Trabalho de Parto Induzido/métodos , Misoprostol/administração & dosagem , Administração Intravaginal , Adulto , Índice de Apgar , Peso ao Nascer , Cesárea , Parto Obstétrico/métodos , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Misoprostol/efeitos adversos , Gravidez , Fatores de Tempo , Resultado do Tratamento
17.
J Perinat Med ; 35(1): 62-6, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-17313312

RESUMO

OBJECTIVE: The aim of our study is to evaluate the clinical importance of near term weight discordance in twin pregnancies with both appropriate or with one small for gestational age newborn (AGA and SGA, respectively). METHODS: We retrospectively studied 230 twin pregnancies that ended at >or=34 weeks' gestation. Discordance was defined as an intertwin birth weight difference>or=20% calculated from the heavier newborn. The following data were compared between discordant and concordant pairs: maternal age, parity, mode of conception, placentation, hypertensive disorders of pregnancy, gestational age at birth, route of delivery, reason for termination of pregnancy, Apgar scores, birth weights, admission to neonatal intensive care unit, significant morbidity, malformations found at birth, and perinatal mortality. The discordant pairs were subdivided into two groups: (1) Both twins were AGA; (2) One of the twins was SGA. The two groups were compared to each other, and to the control group of concordant pairs. RESULTS: One hundred and seventy-six twin pairs were concordant (control group) and 54 were discordant. The comparison between the concordant and the discordant groups did not show statistically significant differences in any of the examined parameters. However, the discordant group had a greater incidence of neonatal morbidity. When the discordant subgroups (AGA, n=24 vs. SGA n=30) were compared to the concordant group, these differences persist, particularly in the SGA group. CONCLUSION: In near term twin pregnancies, birth weight discordance does not seem to predict adverse perinatal outcome except when one of the newborns is SGA.


Assuntos
Peso ao Nascer/fisiologia , Recém-Nascido/fisiologia , Recém-Nascido Pequeno para a Idade Gestacional/fisiologia , Gêmeos/fisiologia , Adulto , Feminino , Humanos , Gravidez , Resultado da Gravidez , Estudos Retrospectivos
19.
Pediatr Crit Care Med ; 3(3): 227-233, 2002 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-12780961

RESUMO

OBJECTIVES: To determine risk factors of mortality in the preoperative, perioperative, and immediate postoperative period of a pediatric population that has undergone orthotopic liver transplantation for fulminant hepatic failure in a pediatric intensive care unit. DESIGN: Retrospective review of medical records. SETTING: A pediatric intensive care unit in a children's hospital. PATIENTS: Sixty patients with fulminant hepatic failure who fulfilled King's College criteria for liver transplantation. INTERVENTION: Orthotopic liver transplantation was performed according to standard techniques. Before transplantation, patients were admitted to a pediatric intensive care unit when intensive care was required, and patients were always admitted to a pediatric intensive care unit after the operation. Measurements: A total of 20 variables were studied via univariate and multivariate analysis; statistical significance was accepted when p

20.
Acta gastroenterol. latinoam ; 30(4): 245-7, 2000. ilus, tab
Artigo em Espanhol | LILACS | ID: lil-272968

RESUMO

There are few cases reported of autoinmune hepatitis (AIH) tipe 2 presenting as fulminant hepatic failure (FHF) in children. The purpose of this study was to report three girls with AIH type 2 that presented as FHF. METHODS: Over a period of 12 years, 123 patients with AIH diagnosed based on international criteria, 9 (7 per cent were type 2.3 of them presented as FHF. Other etiologies (viral, metabolic and toxic) were ruled out. The treatment was started with prednisone (2 mg-kg-day) and azathioprine (2 mg-kg-day). EVOLUTION: Patients 1 and 3 died while waiting for liver transplant (LT) at 72 and 48 hours after initiating medical treatment. Patient 2 underwent LT3 days after starting treatment, with excellent evolution at 3 years and 7 months of follow up. CONCLUSIONS: 1--AIH type 2 was very infrequent in our group. 2--33 per cent of cases had initial presentation as FHF. 3--The course of the disease was aggressive, not responding to immunosupreessive therapy. The evolution was unfavorable in all patients. 4--LT is an alternative treatment for this severe disease.


Assuntos
Humanos , Feminino , Adolescente , Pré-Escolar , Criança , Hepatite Autoimune/etiologia , Falência Hepática/complicações , Evolução Fatal , Seguimentos , Hepatite Autoimune/diagnóstico , Cirrose Hepática/complicações , Cirrose Hepática/patologia , Transplante de Fígado , Fatores Sexuais
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