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1.
Arch Argent Pediatr ; : e202310246, 2024 Apr 25.
Article in English, Spanish | MEDLINE | ID: mdl-38656883

ABSTRACT

Gallbladder agenesis is a rare condition in pediatrics that is usually asymptomatic and represents a diagnostic challenge for physicians seeing these cases for the first time. Some patients may, however, present with symptoms that mimic other diseases of the bile ducts, and many of them undergo surgery due to such suspicion. Still, a timely diagnosis of gallbladder agenesis allows for medical treatment that is often sufficient to resolve the patient's problem. Although it is a benign condition, patients often present with other associated, more serious malformations and should be actively studied for a timely referral to other specialists. Here we describe our experience with the diagnosis and treatment of these patients and a brief review of the bibliography. We hope it will be helpful for physicians facing similar cases.


La agenesia de la vesícula biliar es una entidad rara en pediatría con una evolución normalmente silente, y representa un desafío diagnóstico para el médico que enfrenta estos casos por primera vez. Algunos pacientes pueden, sin embargo, presentar síntomas que simulan otras patologías del árbol biliar, y muchos de ellos son operados ante esta sospecha. Sin embargo, el diagnóstico oportuno de esta entidad permite llevar a cabo un tratamiento médico que muchas veces es suficiente para resolver el problema del paciente. Si bien es una condición benigna, los pacientes suelen presentar otras malformaciones asociadas que son más graves en naturaleza y que deben investigarse activamente para poder derivarlos a los especialistas de manera oportuna. Presentamos nuestra experiencia en el diagnóstico y tratamiento de estos pacientes, así como una breve revisión de la literatura. Esperamos que sea de utilidad para el médico que encuentre un caso similar.

2.
Arch. argent. pediatr ; 121(4): e202202719, ago. 2023. ilus
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1442967

ABSTRACT

La sífilis congénita es causada por la infección del feto con Treponema pallidum durante el embarazo. Los síntomas son variables. Si bien es común el daño endotelial, no suele estar presente en los casos congénitos. Reportamos el caso de un lactante de 42 días de vida hospitalizado por masa abdominal. Las imágenes confirmaban la presencia de una lesión en el lóbulo hepático izquierdo sin efecto de masa. Las biopsias mostraron cambios compatibles con infarto y hepatitis neonatal. Las serologías del paciente y de su madre confirmaron el diagnóstico de sífilis congénita, y recibió tratamiento con penicilina intravenosa. El hígado se encuentra protegido de los daños isquémicos gracias a su doble irrigación, pero la acumulación de noxas puede haber provocado dicha presentación inusual. Tres meses más tarde, el paciente se encontraba libre de síntomas y la resonancia de control mostró atrofia del lóbulo izquierdo, mientras el resto del parénquima no presentaba alteraciones.


Congenital syphilis is caused by Treponema pallidum infection of the fetus during pregnancy. Symptoms are variable. While endothelial damage is common, it is not usually present in congenital cases. Here we report the case of a 42-day-old infant hospitalized due to an abdominal mass. Imaging studies confirmed the presence of an injury in the left lobe of the liver without mass effect. Biopsies showed changes compatible with infarction and neonatal hepatitis. The patient's and his mother's serologies confirmed the diagnosis of congenital syphilis, and he was treated with intravenous penicillin. The liver protected from ischemic injury by its double irrigation, but the accumulation of harmful agents may have caused this unusual presentation. Three months later, the patient was symptom-free, and the control MRI showed atrophy of the left lobe, while the rest of the parenchyma was unchanged.


Subject(s)
Humans , Infant , Syphilis, Congenital/complications , Syphilis, Congenital/diagnosis , Syphilis, Congenital/pathology , Penicillins , Treponema pallidum , Pregnancy , Hepatic Infarction
3.
Arch. argent. pediatr ; 121(3): e202202933, jun. 2023. tab
Article in Spanish | LILACS, BINACIS | ID: biblio-1437257

ABSTRACT

Los avances tecnológicos y del conocimiento hicieron que un mayor número de pacientes con enfermedad crónica gastrointestinal pasen de ser atendidos por el pediatra al control por los médicos de adultos durante una de las etapas más vulnerables de la vida: la adolescencia. El Grupo de Trabajo de Transición del Comité de Gastroenterología de la Sociedad Argentina de Pediatría realizó una búsqueda de literatura exhaustiva y convocó a especialistas referentes del país, con el objeto de unificar los criterios basados en la evidencia y la experiencia. De esta manera, se proponen una serie de recomendaciones para todo el equipo de salud (pediatra, gastroenterólogo infantil, nutricionista, gastroenterólogo de adultos, psicólogo, enfermería), incluso para pacientes y familias, que faciliten el proceso de transición y optimicen el seguimiento, el control, la prevención de complicaciones y la calidad de vida de los pacientes con enfermedades crónicas gastrointestinales


Technological advances and the globalization of knowledge have led to a considerable increase in the number of patients with chronic gastrointestinal disease who transition from pediatric to adult care during one of the most vulnerable life stages: adolescence. The Transition Working Group of the Gastroenterology Committee of the Sociedad Argentina de Pediatría conducted an exhaustive literature search and summoned leading specialists in the most frequent chronic pathologies from all over the country to unify criteria based on evidence and experience. As a result, a series of recommendations are proposed for the whole health team (pediatrician, pediatric gastroenterologist, nutritionist, adult gastroenterologist, psychologist, and nurse) including patients and families, to facilitate the transition process, optimize follow-up, prevent complications, and improve the quality of life of patients with chronic gastrointestinal diseases.


Subject(s)
Humans , Adolescent , Adult , Inflammatory Bowel Diseases , Transition to Adult Care , Gastroenterology , Gastrointestinal Diseases/therapy , Quality of Life , Chronic Disease
4.
Arch Argent Pediatr ; 121(3): e202202933, 2023 06 01.
Article in Spanish | MEDLINE | ID: mdl-37191634

ABSTRACT

Technological advances and the globalization of knowledge have led to a considerable increase in the number of patients with chronic gastrointestinal disease who transition from pediatric to adult care during one of the most vulnerable life stages: adolescence. The Transition Working Group of the Gastroenterology Committee of the Sociedad Argentina de Pediatría conducted an exhaustive literature search and summoned leading specialists in the most frequent chronic pathologies from all over the country to unify criteria based on evidence and experience. As a result, a series of recommendations are proposed for the whole health team (pediatrician, pediatric gastroenterologist, nutritionist, adult gastroenterologist, psychologist, and nurse) including patients and families, to facilitate the transition process, optimize follow-up, prevent complications, and improve the quality of life of patients with chronic gastrointestinal diseases.


Los avances tecnológicos y del conocimiento hicieron que un mayor número de pacientes con enfermedad crónica gastrointestinal pasen de ser atendidos por el pediatra al control por los médicos de adultos durante una de las etapas más vulnerables de la vida: la adolescencia. El Grupo de Trabajo de Transición del Comité de Gastroenterología de la Sociedad Argentina de Pediatría realizó una búsqueda de literatura exhaustiva y convocó a especialistas referentes del país, con el objeto de unificar los criterios basados en la evidencia y la experiencia. De esta manera, se proponen una serie de recomendaciones para todo el equipo de salud (pediatra, gastroenterólogo infantil, nutricionista, gastroenterólogo de adultos, psicólogo, enfermería), incluso para pacientes y familias, que faciliten el proceso de transición y optimicen el seguimiento, el control, la prevención de complicaciones y la calidad de vida de los pacientes con enfermedades crónicas gastrointestinales.


Subject(s)
Gastroenterology , Gastrointestinal Diseases , Inflammatory Bowel Diseases , Transition to Adult Care , Adolescent , Humans , Adult , Child , Quality of Life , Chronic Disease , Gastrointestinal Diseases/therapy
5.
Arch Argent Pediatr ; 121(4): e202202719, 2023 08 01.
Article in English, Spanish | MEDLINE | ID: mdl-36706010

ABSTRACT

Congenital syphilis is caused by Treponema pallidum infection of the fetus during pregnancy. Symptoms are variable. While endothelial damage is common, it is not usually present in congenital cases. Here we report the case of a 42-day-old infant hospitalized due to an abdominal mass. Imaging studies confirmed the presence of an injury in the left lobe of the liver without mass effect. Biopsies showed changes compatible with infarction and neonatal hepatitis. The patient's and his mother's serologies confirmed the diagnosis of congenital syphilis, and he was treated with intravenous penicillin. The liver is protected from ischemic injury by its double irrigation, but the accumulation of harmful agents may have caused this unusual presentation. Three months later, the patient was symptom-free, and the control MRI showed atrophy of the left lobe, while the rest of the parenchyma was unchanged.


La sífilis congénita es causada por la infección del feto con Treponema pallidum durante el embarazo. Los síntomas son variables. Si bien es común el daño endotelial, no suele estar presente en los casos congénitos. Reportamos el caso de un lactante de 42 días de vida hospitalizado por masa abdominal. Las imágenes confirmaban la presencia de una lesión en el lóbulo hepático izquierdo sin efecto de masa. Las biopsias mostraron cambios compatibles con infarto y hepatitis neonatal. Las serologías del paciente y de su madre confirmaron el diagnóstico de sífilis congénita, y recibió tratamiento con penicilina intravenosa. El hígado se encuentra protegido de los daños isquémicos gracias a su doble irrigación, pero la acumulación de noxas puede haber provocado dicha presentación inusual. Tres meses más tarde, el paciente se encontraba libre de síntomas y la resonancia de control mostró atrofia del lóbulo izquierdo, mientras el resto del parénquima no presentaba alteraciones.


Subject(s)
Hepatic Infarction , Syphilis, Congenital , Infant , Infant, Newborn , Male , Pregnancy , Female , Humans , Child , Syphilis, Congenital/complications , Syphilis, Congenital/diagnosis , Syphilis, Congenital/pathology , Treponema pallidum , Penicillins
6.
Arch. argent. pediatr ; 120(1): e17-e20, feb 2022. tab, ilus
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1353739

ABSTRACT

La hipertensión portopulmonar (HTPP) es una complicación infrecuente de la hipertensión portal, que sigue un curso progresivo con un pronóstico sombrío. Los reportes en pacientes pediátricos son escasos y con períodos de seguimiento cortos. Se describe una paciente con cirrosis descompensada que desarrolló HTPP resuelta mediante trasplante hepático, que permanece asintomática tras diez años de seguimiento.


Portopulmonary hypertension is an uncommon complication of portal hypertension, running a progressive course with a negative prognosis. Reports in pediatric patients are scarce with short follow up. We describe the case of decompensated cirrhosis who developed PoPH and resolved with liver transplantation, remaining asymptomatic after ten years of follow up.


Subject(s)
Humans , Female , Child , Liver Transplantation , Hepatitis, Autoimmune/complications , Hypertension, Portal/complications , Hypertension, Pulmonary/etiology , Liver Cirrhosis/complications
7.
Arch Argent Pediatr ; 120(1): e17-e20, 2022 02.
Article in English, Spanish | MEDLINE | ID: mdl-35068124

ABSTRACT

Portopulmonary hypertension is an uncommon complication of portal hypertension, running a progressive course with a negative prognosis. Reports in pediatric patients are scarce with short follow up. We describe the case of decompensated cirrhosis who developed PoPH and resolved with liver transplantation, remaining asymptomatic after ten years of follow up.


La hipertensión portopulmonar (HTPP) es una complicación infrecuente de la hipertensión portal, que sigue un curso progresivo con un pronóstico sombrío. Los reportes en pacientes pediátricos son escasos y con períodos de seguimiento cortos. Se describe una paciente con cirrosis descompensada que desarrolló HTPP resuelta mediante trasplante hepático, que permanece asintomática tras diez años de seguimiento.


Subject(s)
Hepatitis, Autoimmune , Hypertension, Portal , Hypertension, Pulmonary , Liver Transplantation , Child , Hepatitis, Autoimmune/complications , Humans , Hypertension, Portal/complications , Hypertension, Pulmonary/etiology , Liver Cirrhosis/complications
8.
Zoonoses Public Health ; 69(3): 235-241, 2022 05.
Article in English | MEDLINE | ID: mdl-34941019

ABSTRACT

Hepatitis E virus (HEV) is a public health concern due to its zoonotic transmission to human, being pigs a highly recognized reservoir. We previously demonstrated HEV genotype 3 infections in pig herds from the highest commercial active region from Argentina. Here, we present a case of acute symptomatic hepatitis E in an elderly man with occupational exposure to pigs who referred regular consumption of pork and sausages. HEV infection in this patient was demonstrated by serological methods, as well as by HEV RNA detection in serum and stool samples using the HEV/MS2 duplex RT-qPCR, formerly optimized in our laboratory. We further detected HEV RNA in pig faeces from the patient´s farm. To confirm the potential role of swine in the transmission, we performed a phylogenetic analysis of all HEV RNA derived from both, the patient and the pig samples. A 303 nt region within the HEV 5 'ORF2 was amplified by nested RT-PCR and subsequently sequenced. Phylogenetic analysis showed that the strains isolated from the farmer and from his pigs presented a nucleotide identity of 100%. These results support the zoonotic transmission of circulating HEV strains and confirm this epidemiological association for the first time in Argentina.


Subject(s)
Hepatitis E virus , Hepatitis E , Swine Diseases , Animals , Argentina/epidemiology , Farmers , Genotype , Hepatitis E/epidemiology , Hepatitis E/veterinary , Humans , Phylogeny , RNA , RNA, Viral/analysis , RNA, Viral/genetics , Real-Time Polymerase Chain Reaction/veterinary , Swine
9.
Arch. argent. pediatr ; 118(2): e191-e193, abr. 2020. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1100475

ABSTRACT

Yersinia enterocolitica es un bacilo Gram-negativo causante de infección intestinal en los humanos. Se presenta con diferentes cuadros clínicos que obligan a descartar una variedad de etiologías, lo cual, a veces, hace difícil alcanzar un diagnóstico correcto en forma oportuna.Se expone el caso de un varón adolescente con dolor en la fosa ilíaca derecha a partir de una ileítis terminal con hallazgos similares a la enfermedad de Crohn, que se diagnosticó, finalmente, como infección por Yersinia enterocolitica. Se destaca la utilidad de los diferentes métodos auxiliares empleados.


Yersinia enterocolitica is a gram-negative rod causing intestinal infection in humans. It shows different clinical pictures with many different etiologies to be ruled-out, which sometimes makes it difficult to reach a timely and correct diagnosis. We report the case of an adolescent boy presenting with right lower quadrant pain from terminal ileitis with endoscopic findings akin to Crohn ́s disease finally diagnosed as Yersinia enterocolitica, highlighting the usefulness of the different ancillary methods employed.


Subject(s)
Humans , Male , Child , Yersinia enterocolitica , Crohn Disease/diagnosis , Ileitis/complications , Microbiological Techniques , Molecular Diagnostic Techniques , Diagnosis, Differential , Feces/microbiology
10.
Arch Argent Pediatr ; 118(2): e191-e193, 2020 04.
Article in Spanish | MEDLINE | ID: mdl-32199063

ABSTRACT

Yersinia enterocolitica is a gram-negative rod causing intestinal infection in humans. It shows different clinical pictures with many different etiologies to be ruled-out, which sometimes makes it difficult to reach a timely and correct diagnosis. We report the case of an adolescent boy presenting with right lower quadrant pain from terminal ileitis with endoscopic findings akin to Crohn´s disease finally diagnosed as Yersinia enterocolitica, highlighting the usefulness of the different ancillary methods employed.


Yersinia enterocolitica es un bacilo Gram-negativo causante de infección intestinal en los humanos. Se presenta con diferentes cuadros clínicos que obligan a descartar una variedad de etiologías, lo cual, a veces, hace difícil alcanzar un diagnóstico correcto en forma oportuna. Se expone el caso de un varón adolescente con dolor en la fosa ilíaca derecha a partir de una ileítis terminal con hallazgos similares a la enfermedad de Crohn, que se diagnosticó, finalmente, como infección por Yersinia enterocolitica. Se destaca la utilidad de los diferentes métodos auxiliares empleados.


Subject(s)
Crohn Disease/diagnosis , Ileitis/diagnosis , Yersinia Infections/diagnosis , Yersinia enterocolitica/isolation & purification , Child , Diagnosis, Differential , Humans , Ileitis/microbiology , Male
11.
Liver Transpl ; 26(2): 268-275, 2020 02.
Article in English | MEDLINE | ID: mdl-31606931

ABSTRACT

After the implementation of universal hepatitis A virus vaccination in Argentina, the outcome of pediatric acute liver failure (PALF) remains unknown. We aimed to identify variables associated with the risk of liver transplantation (LT) or death and to determine the causes and short-term outcomes of PALF in Argentina. We retrospectively included 135 patients with PALF listed for LT between 2007 and 2016. Patients with autoimmune hepatitis (AIH), Wilson's disease (WD), or inborn errors of metabolism (IEM) were classified as PALF-chronic liver disease (CLD), and others were classified as "pure" PALF. A logistic regression model was developed to identify factors independently associated with death or need of LT and risk stratification. The most common etiologies were indeterminate (52%), AIH (23%), WD (6%), and IEM (6%). Overall, transplant-free survival was 35%, whereas 50% of the patients underwent LT and 15% died on the waiting list. The 3-month risk of LT or death was significantly higher among patients with pure PALF compared with PALF-CLD (76.5% versus 42.5%; relative risk, 1.8 [1.3-2.5]; P < 0.001), and 3 risk factors were independently associated with worse outcome: international normalized ratio (INR) ≥3.5 (odds ratio [OR], 3.1; 95% confidence interval [CI], 1.3-7.2]), bilirubin ≥17 mg/dL (OR, 4.4; 95% CI, 1.9-10.3]), and pure PALF (OR, 3.8; 95% CI, 1.6-8.9). Patients were identified by the number of risk factors: Patients with 0, 1, or ≥2 risk factors presented a 3-month risk of worse outcome of 17.6%, 36.6%, and 82%, respectively. In conclusion, although lacking external validation, this simple risk-staging model might help stratify patients with different transplant-free survival rates and may contribute to establishing the optimal timing for LT.


Subject(s)
Liver Failure, Acute , Liver Transplantation , Argentina , Child , Humans , Liver Failure, Acute/diagnosis , Liver Failure, Acute/epidemiology , Liver Failure, Acute/etiology , Liver Transplantation/adverse effects , Prognosis , Retrospective Studies
12.
Zoonoses Public Health ; 66(7): 729-738, 2019 11.
Article in English | MEDLINE | ID: mdl-31259469

ABSTRACT

Hepatitis E virus (HEV) is currently considered as a global health concern due to the recognition of its zoonotic transmission to humans, mainly from swine, and its association with the development of severe cases of hepatitis in human risk populations. The lack of updated data on HEV state of infection in swineherds of Argentina, and the necessity of robust technologies for its detection in complex biological samples, positions HEV as an emerging issue in public health. Here, we have optimized a RT-qPCR with internal control for a more precise and accurate HEV RNA detection in swine stool samples. We implemented this optimized molecular tool to analyse the current epidemiological scenario of HEV infection in swine from the core region of commercial activity of Argentina. A total of 135 stool samples were collected from 16 different farms and tested for HEV presence, resulting in 11 positive cases (8.1%). Phylogenetic analysis demonstrated that all of them correspond to HEV genotype 3 and that different subtypes circulate in the region. Moreover, two of the detected strains presented a high nucleotide similarity with a previously identified isolate from human sewage discharges, suggesting the zoonotic transmission of HEV to humans. Collectively, this work provides a better understanding of HEV epidemiology in Argentina while contributes to the improvement of HEV detection technologies.


Subject(s)
Hepatitis E virus/isolation & purification , Hepatitis E/veterinary , Reverse Transcriptase Polymerase Chain Reaction/veterinary , Swine Diseases/virology , Animals , Argentina/epidemiology , Genotype , Hepatitis E virus/genetics , Phylogeny , Reverse Transcriptase Polymerase Chain Reaction/methods , Swine , Swine Diseases/epidemiology , Zoonoses
13.
Pediatr Transplant ; 23(2): e13356, 2019 03.
Article in English | MEDLINE | ID: mdl-30676683

ABSTRACT

Portal vein malformations might occur during the embryonic period, as a consequence of abnormal remodeling of vitelline veins during embryonic life. Patients suffering from biliary atresia are particularly prone to have vascular malformations; although being the most frequent indication for liver transplantation in the pediatric age, portal vein duplication has not been so far associated with biliary atresia, and to the best of our knowledge, there is no-written evidence describing how to manage it when it is first diagnosed while performing a pediatric liver transplant. Therefore, we present a recent case from our group, describing the intraoperative diagnosis of a double portal system in a patient with biliary atresia and failed Kasai. We aim to describe its surgical management, understanding that it is a real challenge to find them unexpectedly during the surgical procedure in the setting of cirrhosis and portal hypertension, particularly in small patients; therefore, by reporting this case, we aim to make readers aware about the chance of finding it, and how to managed it, to include this approach as part of the surgical armamentarium.


Subject(s)
Abnormalities, Multiple/surgery , Biliary Atresia/surgery , Liver Transplantation/methods , Living Donors , Portal Vein/abnormalities , Vascular Malformations/surgery , Child, Preschool , Female , Humans
14.
J Pediatr Gastroenterol Nutr ; 67(6): e107-e110, 2018 12.
Article in English | MEDLINE | ID: mdl-30095578

ABSTRACT

OBJETIVES: The aim of the study is to investigate the incidence and clinical features of autoimmune hepatitis (AIH) in children from the province of Santa Fe, Argentina, for 10 years. METHODS: From the records of all of the pediatric hepatologists in the province of Santa Fe, Argentina, we reviewed the clinical charts of patients <18 years who were diagnosed with AIH (simplified score >6 points) and followed between January 2003 and December 2013. Population data were extracted from the 2010 national census. Values were expressed as percentages and median ± interquartile range. Mann-Whitney U test was used for comparison between the groups. RESULTS: Sixty-seven patients fulfilled inclusion criteria, from which 11 (16%) were later reclassified as having "autoimmune sclerosing cholangitis" according to biochemical, histological, and radiological findings. A final sample of 56 patients (39 F) with AIH was analyzed, giving an annual incidence of 0.56/100,000. Median age at presentation was 8 (5.7-11) years, and the median follow-up was 4 (2-7) years. Type 1 AIH was diagnosed in 89%. An acute presentation was observed in 53%, while 13 (23%) showed cirrhosis on initial biopsy. Prednisone (87%) and azathioprine (60%) were the most common drugs prescribed. At the end of follow-up, 53/56 (95%) were alive, including 4 patients (7%) who underwent liver transplantation. CONCLUSIONS: AIH has an estimated incidence of 0.56/100,000 per year in children from the province of Santa Fe (Argentina). Overall survival rate was 95%. A subgroup of patients diagnosed as AIH develops predominant biliary disease and should be better classified as autoimmune sclerosing cholangitis.


Subject(s)
Hepatitis, Autoimmune/epidemiology , Argentina/epidemiology , Biopsy , Child , Child, Preschool , Cholangitis, Sclerosing/epidemiology , Cholangitis, Sclerosing/pathology , Female , Hepatitis, Autoimmune/pathology , Humans , Incidence , Liver/pathology , Male , Retrospective Studies , Survival Rate
15.
Arch. argent. pediatr ; 114(3): e155-e158, jun. 2016. ilus, tab
Article in English, Spanish | LILACS, BINACIS | ID: biblio-838216

ABSTRACT

La superposición del lupus eritematoso sistémico y la hepatitis autoinmune se describe ocasionalmente. Aunque ambas enfermedades pueden compartir ciertos hallazgos, como poliartralgias, hipergammaglobulinemia y anticuerpo antinúcleo positivo, son consideradas dos diferentes. Se presenta a una paciente de 14 años con lupus eritematoso sistémico, que, luego de dos años, consultó por ictericia. Sin antecedentes de ingesta de drogas, alcohol o exposición a virus hepatotropos. Tenia un aumento de las enzimas hepáticas con anticuerpos antinúcleo, anti-ADN de doble cadena y LKM 1 positivos. La biopsia hepática mostró una hepatitis de interfase con infiltrado linfoplasmocitario. De esta manera, cumplia con los criterios diagnósticos tanto para lupus eritematoso sistémico como para hepatitis autoinmune. Tratada con corticoides y micofenolato mofetil, mejoró su clinica y laboratorio. Conclusión. La hepatitis autoinmune puede ocurrir en el curso del lupus eritematoso sistémico. Un diagnóstico temprano es importante para prevenir el avance de la enfermedad; es obligatoria la realización de la biopsia hepática.


Overlap of systemic lupus erythematosus and autoimmune hepatitis is a rare condition, occasionally described. Both autoimmune diseases can share common autoimmune features such as polyartralgia, hypergammaglobulinemia and positive antinuclear antibody, but they have been considered as two different entities. We report a 14 year old female patient with systemic lupus erythematosus who developed autoimmune hepatitis two years later. She had jaundice with no history of viral infections, drug intake, alcohol abuses or exposition to blood products. She was positive for antinuclear antibody, double stranded DNA antibody and LKM 1 antibody. Histological examination of the liver showed interface hepatitis with lymphoplasmacytic cell infiltration. The patient satisfied the international criteria for both systemic lupus erythematosus and Type 2 autoimmune hepatitis. Clinical symptoms and laboratory findings improved under treatment with steroids and mycophenolate mofetil. Conclusion. Autoimmune hepatitis may occur during the course of systemic lupus erythematosus and an early diagnosis is important for preventing advanced liver disease. Definitive diagnosis of autoimmune hepatitis requires confirmation by means of a liver biopsy.


Subject(s)
Humans , Female , Adolescent , Hepatitis, Autoimmune/diagnosis , Undifferentiated Connective Tissue Diseases/diagnosis , Lupus Erythematosus, Systemic/diagnosis , Hepatitis, Autoimmune/complications , Undifferentiated Connective Tissue Diseases/complications , Lupus Erythematosus, Systemic/complications
16.
Arch Argent Pediatr ; 114(3): e155-8, 2016 Jun 01.
Article in English, Spanish | MEDLINE | ID: mdl-27164347

ABSTRACT

UNLABELLED: Overlap of systemic lupus erythematosus and autoimmune hepatitis is a rare condition, occasionally described. Both autoimmune diseases can share common autoimmune features such as polyartralgia, hypergammaglobulinemia and positive antinuclear antibody, but they have been considered as two different entities. We report a 14 year old female patient with systemic lupus erythematosus who developed autoimmune hepatitis two years later. She had jaundice with no history of viral infections, drug intake, alcohol abuses or exposition to blood products. She was positive for antinuclear antibody, double stranded DNA antibody and LKM 1 antibody. Histological examination of the liver showed interface hepatitis with lymphoplasmacytic cell infiltration. The patient satisfied the international criteria for both systemic lupus erythematosus and Type 2 autoimmune hepatitis. Clinical symptoms and laboratory findings improved under treatment with steroids and mycophenolate mofetil. CONCLUSION: Autoimmune hepatitis may occur during the course of systemic lupus erythematosus and an early diagnosis is important for preventing advanced liver disease. Definitive diagnosis of autoimmune hepatitis requires confirmation by means of a liver biopsy.


La superposición del lupus eritematoso sistémico y la hepatitis autoinmune se describe ocasionalmente. Aunque ambas enfermedades pueden compartir ciertos hallazgos, como poliartralgias, hipergammaglobulinemia y anticuerpo antinúcleo positivo, son consideradas dos diferentes. Se presenta a una paciente de 14 años con lupus eritematoso sistémico, que, luego de dos años, consultó por ictericia. Sin antecedentes de ingesta de drogas, alcohol o exposición a virus hepatotropos. Tenia un aumento de las enzimas hepáticas con anticuerpos antinúcleo, anti-ADN de doble cadena y LKM 1 positivos. La biopsia hepática mostró una hepatitis de interfase con infiltrado linfoplasmocitario. De esta manera, cumplia con los criterios diagnósticos tanto para lupus eritematoso sistémico como para hepatitis autoinmune. Tratada con corticoides y micofenolato mofetil, mejoró su clinica y laboratorio. Conclusión. La hepatitis autoinmune puede ocurrir en el curso del lupus eritematoso sistémico. Un diagnóstico temprano es importante para prevenir el avance de la enfermedad; es obligatoria la realización de la biopsia hepática.


Subject(s)
Hepatitis, Autoimmune/diagnosis , Lupus Erythematosus, Systemic/diagnosis , Undifferentiated Connective Tissue Diseases/diagnosis , Adolescent , Female , Hepatitis, Autoimmune/complications , Humans , Lupus Erythematosus, Systemic/complications , Undifferentiated Connective Tissue Diseases/complications
17.
Arch. argent. pediatr ; 113(6): e333-e335, dic. 2015. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-838146

ABSTRACT

El adenocarcinoma colorrectal es excepcional en ninos, por lo que, generalmente, se lo diagnostica en estadios avanzados, con mal pronóstico. Presentamos el caso de una nina de 5 anos de edad con proctorragia recurrente de 2 meses de evolución, sin alteraciones en los análisis de laboratorio, en quien se confirmó la existencia de un adenocarcinoma colorrectal por medio de una colonoscopía. La estatificación demostró un compromiso local sin enfermedad o metástasis a distancia. La paciente se trató con resección laparoscópica y quimioterapia adyuvante, y permaneció libre de enfermedad a 21 meses de seguimiento.


Colorectal adenocarcinoma is exceptional in children, generally diagnosed in advanced stages, with worse prognosis. We report the case of a 5 year old girl with isolated hematochezia lasting two months without other findings, in whom a colorectal adenocarcinoma was confirmed by colonoscopy. Staging revealed localized disease, extending to perisigmoid fat and a few adjacent ganglia. She was treated by laparoscopic resection and adjuvant chemotherapy, being free of disease at 21 moths of follow-up.


Subject(s)
Humans , Female , Child, Preschool , Colorectal Neoplasms/diagnosis , Colorectal Neoplasms/drug therapy , Adenocarcinoma/diagnosis , Adenocarcinoma/drug therapy , Chemotherapy, Adjuvant
18.
Arch Argent Pediatr ; 113(6): e333-5, 2015 Dec 01.
Article in Spanish | MEDLINE | ID: mdl-26593811

ABSTRACT

Colorectal adenocarcinoma is exceptional in children, generally diagnosed in advanced stages, with worse prognosis. We report the case of a 5 year old girl with isolated hematochezia lasting two months without other findings, in whom a colorectal adenocarcinoma was confirmed by colonoscopy. Staging revealed localized disease, extending to perisigmoid fat and a few adjacent ganglia. She was treated by laparoscopic resection and adjuvant chemotherapy, being free of disease at 21 moths of follow-up.


Subject(s)
Adenocarcinoma/diagnosis , Colorectal Neoplasms/diagnosis , Adenocarcinoma/drug therapy , Chemotherapy, Adjuvant , Child, Preschool , Colorectal Neoplasms/drug therapy , Female , Humans
19.
Arch. argent. pediatr ; 112(4): 369-374, ago. 2014. tab
Article in Spanish | LILACS, BINACIS | ID: biblio-1159620

ABSTRACT

El aumento de las cifras de transaminasas séricas puede observarse en diferentes escenarios de la consulta médica cada vez con mayor frecuencia. Esto supone un desafío complejo para el pediatra dada la escasa información bibliográfica que existe sobre este tema. Desde el paciente completamente asintomático hasta el niño con signos de cronicidad, el espectro es muy variado y obliga a orientar rápida y eficientemente la investigación a fin de evitar costos y sufrimientos innecesarios para el paciente y su familia. El trabajo coordinado con el hepatólogo es recomendable en la mayoría de los casos.


The raising of serum transaminases is seen with increasing frequency in different scenarios from daily practice. This poses a complex challenge to the pediatrician owing to the scarcity of bibliographic information available on this topic. From the completely asymptomatic patient to the child with signs of chronic liver disease, the full spectrum is highly varied, compelling to guide the investigation efficiently, in order to avoid unnecessary costs and suffering to the patient and their family. Coordinated work with the hepatologist is advisable in most instances.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Transaminases/blood , Metabolic Diseases/diagnosis , Metabolic Diseases/enzymology , Metabolic Diseases/blood
20.
Arch Argent Pediatr ; 112(4): 369-74, 2014 Aug.
Article in Spanish | MEDLINE | ID: mdl-24955909

ABSTRACT

The raising of serum transaminases is seen with increasing frequency in different scenarios from daily practice. This poses a complex challenge to the pediatrician owing to the scarcity of bibliographic information available on this topic. From the completely asymptomatic patient to the child with signs of chronic liver disease, the full spectrum is highly varied, compelling to guide the investigation efficiently, in order to avoid unnecessary costs and suffering to the patient and their family. Coordinated work with the hepatologist is advisable in most instances.


Subject(s)
Metabolic Diseases/blood , Metabolic Diseases/enzymology , Transaminases/blood , Adolescent , Child , Child, Preschool , Female , Humans , Male , Metabolic Diseases/diagnosis
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