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Introducción. Las malformaciones linfáticas quísticas, también llamadas linfangiomas quísticos, aparecen muy raramente de forma aislada en el hígado. Casos clínicos. Se presentan dos pacientes femeninas de edad preescolar con marcada hepatomegalia, dependiente de lesiones quísticas multitabicadas, secundarias a malformación linfática quística gigante del hígado, que fueron tratadas en el Hospital Pediátrico Universitario William Soler, La Habana, Cuba. Resultados. En ambos casos el diagnóstico se apoyó en los estudios de imágenes, la laparoscopia y el análisis histopatológico. En un caso el tratamiento fue la hepatectomía derecha, mientras que en el otro se empleó la escleroterapia, ambas con evolución favorable. Conclusión. A pesar de su rareza, este diagnóstico no debe obviarse ante un paciente pediátrico con lesiones hepáticas quísticas. El tratamiento de elección es la resección quirúrgica, pero su indicación y envergadura debe valorarse de forma individualizada

Introduction. Cystic lymphatic malformations, also called cystic lymphangiomas, are very rarely found in the liver. Clinical cases. Two pediatric female preschool-age patients. presented with hepatomegaly due to multi-septated cystic lesions of the liver, who received treatment at Hospital Pediátrico Universitario William Soler, La Habana, Cuba. Results. We report two pediatric cases with giant cystic lymphatic malformation of the liver. In both cases, the diagnosis were based on imaging, laparoscopy and pathology. In one case the treatment was right hepatectomy, whereas in the other, sclerotherapy was performed, both with a favorable outcome. Conclusion. Despite its rarity, this diagnosis should be considered in pediatric patients with hepatic cystic lesions. The recommended treatment is surgical resection, but its indication and extent should be assessed individually for each patient.

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Introducción. El cistoadenoma mucinoso biliar es una neoplasia rara con alta probabilidad de malignidad. Su diagnóstico es un reto ya que se asemeja a otras masas benignas que pueden encontrarse en el hígado. Caso clínico. Mujer de 21 años con sensación de masa en hipocondrio derecho, a quien se le realizan marcadores tumorales y estudios de imágenes concluyendo que se trataba de un cistadenoma mucinoso biliar. Resultado. Se presenta el caso de una paciente con cistoadenoma mucinoso biliar, diagnosticada y tratada exitosamente con cirugía. Conclusión. El diagnóstico de cistoadenoma mucinoso biliar se confirma mediante marcadores tumorales y estudios radiológicos, y su tratamiento es quirúrgico debido al riesgo de malignidad

Introduction. Biliary mucinous cystadenoma is a rare neoplasm with a high probability of malignancy. Its diagnosis is a challenge since it resembles other benign masses that can be found in the liver. Clinical case. A 21-year-old woman with a sensation of a mass in the right hypochondrium, who underwent tumor markers and imaging studies, concluding with a diagnosis of biliary mucinous cystadenoma. Result. A case of a patient with biliary mucinous cystadenoma diagnosed and successfully treated by surgery is presented. Conclusion. The diagnosis of biliary mucinous cystadenoma is confirmed by tumor markers and radiological studies, and its treatment is surgical due to the risk of malignancy

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Resumen: la leucemia congénita neonatal se presenta en los primeros 30 días de vida y es una enfermedad muy poco frecuente en este grupo etario, pero sí de mayor presencia entre los pacientes con síndrome de Down. Su etiología es principalmente genética, pero también se asocia con el consumo de alcohol, la marihuana y el tabaquismo. Se debe hacer diagnóstico diferencial con el desorden mieloproliferativo transitorio, que es una entidad benigna y se resuelve de forma espontánea. Se presenta un caso clínico de un paciente recién nacido a término, de 16 días, que inicialmente presenta sintomatología sugestiva de enterocolitis necrotizante. Sin embargo, al ingresar a unidad neonatal, se evidencia abdomen muy distendido con hepatoesplenomegalia severa con hemograma con hiperleucocitosis severa, anemia y trombocitopenia asociado a blastos del 100 % y paraclínicos de extensión, sugestivo de síndrome de lisis tumoral, por lo que se inicia manejo con hiperhidratación, alopurinol y rasburicasa, sin mejoría. Por esta razón se adiciona citarabina. La citometría de flujo reporta patrón megaloblástico, es decir, un patrón de leucemia mieloide. Requiere hospitalización por mes y medio; luego de este tiempo pueden presentarse complicaciones esperadas. No obstante, se consideró que el recién nacido cursó con trastorno mieloide transitorio, en razón a evolución clínica satisfactoria, con disminución de sintomatología y de leucocitosis severa.

Abstract: Neonatal congenital leukemia manifests within the first 30 days of life and is exceedingly rare in this age group, but more prevalent among patients with Down syndrome. Its etiology is primarily genetic but is also linked to alcohol consumption, marijuana, and smoking. Differential diagnosis should be made with transient myeloproliferative disorder, a benign condition that resolves spontaneously. We present a clinical case of a full-term newborn, 16 days old, initially displaying symptoms suggestive of necrotizing enterocolitis. However, upon admission to the neonatal unit, the patient exhibited a greatly distended abdomen with severe hepatosplenomegaly, severe leukocytosis, anemia, and thrombocytopenia, accompanied by 100% blasts and laboratory findings consistent with tumor lysis syndrome. Management was initiated with hyperhydration, allopurinol, and rasbu-ricase, but there was no improvement. Therefore, cytarabine was added. Flow cytometry indicated a megaloblastic pattern, indicative of myeloid leukemia. The patient required hospitalization for a month and a half, during which expected complications might arise. However, it was considered that the newborn experienced transient myeloid disorder, given the satisfactory clinical evolution, with reduced symptoms and severe leukocytosis.

Resumo: a leucemia congênita neonatal ocorre nos primeiros 30 dias de vida e é uma doença pouco frequente nesse grupo etário, no entanto, ela é mais comum entre pacientes com síndrome de Down. Sua etiologia é principalmente genética, mas também está associada ao consumo de álcool, maconha e tabagismo. Deve-se fazer um diagnóstico diferencial com o distúrbio mieloproliferativo transitório, que é uma condição benigna e se resolve espontaneamente. Apresentamos um caso clínico de um recém-nascido, com 16 dias de idade, que inicialmente apresentou sintomas sugestivos de enterocolite necrosante. No entanto, ao ser admitido na unidade neonatal, foi evidenciado um abdômen muito distendido com hepatosplenomegalia grave, com hemograma mostrando hiperleucocitose grave, anemia e trombocitopenia, associado a 100% de blastos e resultados de extensão sugestivos de síndrome de lise tumoral. Portanto, foi iniciado tratamento com hiperidratação, alopurinol e rasburicasa, sem melhora. Por esse motivo, a citarabina foi adicionada. A citometria de fluxo relatou um padrão megaloblástico, ou seja, um padrão de leucemia mieloide. O paciente necessita, então, de hospitalização por um mês e meio; após esse tempo, complicações esperadas podem ocorrer. No entanto, considerou-se que o recém-nascido teve um distúrbio mieloide transitório, devido à evolução clínica satisfatória, com diminuição dos sintomas e da hiperleucocitose.

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Hyperadrenocorticism is a relatively common endocrinopathy in middle-aged and older dogs, which has become increasingly frequent in the clinical routine. The diagnosis is made by information obtained by history, physical exam findings and results of screening and specific endocrine tests. In addition to laboratory tests, imaging diagnosis, such as ultrasound, can aid on evaluation of possible changes in these cases; also, they may reveal the possible involvement of other organs and systems. The aim of this study was to present the main ultrasonographic changes observed in 18 dogs with hyperadrenocorticism, diagnosed from 2013 to 2020 by the low-dose dexamethasone suppression test, among which hepatomegaly, splenomegaly, gallbladder sludge, renal changes, cystitis and changes in adrenal size, such as bilateral and/or unilateral adrenomegaly, can be cited. Ultrasound examination, therefore, is an examination that can help the clinician in confirming the diagnosis of hyperadrenocorticism, in addition to allowing differentiation between pituitary-dependent hyperadrenocorticism (PDH) and adrenal tumors, and the evaluation of possible secondary changes to the disease.(AU)

iperadrenocorticismo é uma endocrinopatia relativamente comum em cães de meia idade e idosos, que tem se tornado cada vez mais frequente na rotina clínica O diagnóstico é confirmado por informações obtidas pelo histórico, achados de exame físico e resultados de exames de triagem e endócrinos específicos. Além dos exames laboratoriais, os exames de ima-gem, como a ultrassonografia, podem auxiliar na avaliação de possíveis alterações nesses casos; ademais, eles também podem revelar o possível envolvimento de outros órgãos e sistemas. O objetivo deste estudo foi apresentar as principais alterações ultrassonográficas observadas em 18 cães com hiperadrenocorticismo, diagnosticados de 2013 a 2020 pelo teste de supressão por dexametasona em baixa dose, dentre as quais podem ser citadas hepatomegalia, esplenomegalia, lama biliar, alterações renais, cistite e alterações no tamanho e formato das adrenais, como adrenomegalia bilateral e/u unilateral. A avaliação ultras-sonográfica, portanto, é um exame que pode ajudar o clínico a confirmar o diagnóstico de hiperadrenocorticismo, além de ajudar a diferenciar entre hiperadrenocorticismo hipófise-dependente (HHD) e tumores adrenais, e na avaliação de possíveis alterações secundárias à doença.(AU)

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O angiossarcoma primário hepático é o tumor mesenquimal mais comum do fígado, representando cerca de 2% das neoplasias malignas primárias do órgão. Esse raro tumor tem sintomas inespecíficos, evolução agressiva e diagnóstico usualmente tardio, com prognóstico reservado mesmo quando tratado. Este trabalho consiste em um relato de caso de um paciente do sexo masculino, de 44 anos, que foi encaminhado à emergência do Hospital Geral Roberto Santos para investigação de quadro de anemia grave sintomática, síndrome consumptiva e hepatoesplenomegalia. Durante investigação laboratorial, evidenciou-se anemia com provável componente microangiopático associado à anemia da doença crônica. As sorologias para doenças virais e baciloscopia do escarro foram negativas. Foram detectados em exames de imagem dois nódulos hepáticos de grandes dimensões, adenomegalias retroperitonais, esplenomegalia de grande monta, volumoso derrame pleural à direita, além de alterações do esqueleto axial e apendicular. Evoluiu com síndrome da lise tumoral após tratamento clínico com corticoterapia por suspeita de linfoma, com óbito. A biópsia guiada por uma tomografia realizada previamente teve como conclusão perfil imuno-histoquímico compatível com angiossarcoma hepático. O angiossarcoma é um raro tumor, de difícil diagnóstico e tratamento, com evolução agressiva e achados clínico-laboratoriais pouco elucidativos, devendo a hipótese desta doença ser considerada no diagnóstico diferencial das neoplasias hepáticas. As opções terapêuticas são limitadas. Relatos de casos como este são de suma importância para o aumento do grau de suspeição clínica e um diagnóstico mais precoce dessa entidade de costumeira evolução catastrófica.

Primary hepatic angiosarcoma is the most common mesenchymal tumor of the liver, representing about 2% of primary hepatic malignancies. This rare tumor has nonspecific symptoms, delayed diagnosis, and aggressive evolution, with a poor prognosis even when treated. This study reports the case of a 44-year-old male patient referred to the emergency department of the Hospital Geral Roberto Santos with symptomatic severe anemia, consumptive syndrome, and hepatosplenomegaly. Laboratory investigation indicated anemia with a probable microangiopathic component and chronic disease anemia. Serology tests for viral diseases returned negative results, as well as sputum smear microscopy for tuberculosis. Imaging exams revealed two large hepatic nodules, retroperitoneal adenomegaly, large splenomegaly, large pleural effusion in the right lung, and bone involvement. After clinical treatment with corticosteroids for suspected lymphoma, the patient evolved with tumor lysis syndrome and died. Tomography-guided liver biopsy was previously performed, indicating an immunohistochemical profile compatible with hepatic angiosarcoma ­ a rare tumor of difficult diagnosis and treatments due to its aggressive evolution and poor clinical and laboratory findings. Considering the nonspecificity of imaging exams, this disease should be considered in the differential diagnosis of liver neoplasms investigation. Case reports such as the one described in this study are important for increasing the degree of clinical suspicion and earlier diagnosis of this malignancy.

El angiosarcoma hepático primario es el tumor mesenquimatoso del hígado más común y representa el 2% de las neoplasias malignas primarias del hígado. Este raro tumor presenta una sintomatología inespecífica, diagnóstico tardío y evolución agresiva, con mal pronóstico incluso en tratamiento. Este es un reporte de caso de un hombre de 44 años de edad, que fue remitido al servicio de urgencias del Hospital Geral Roberto Santos para investigar anemia severa sintomática, síndrome de consunción y hepatoesplenomegalia. Durante la investigación de laboratorio, se evidenció anemia con un probable componente microangiopático asociado a anemia por enfermedad crónica. La serología para enfermedades virales resultó negativa, así como la microscopía de frotis de esputo para tuberculosis. Las imágenes revelaron dos grandes nódulos hepáticos, adenomegalia retroperitoneal, gran esplenomegalia, gran derrame pleural en el pulmón derecho, así como afectación del esqueleto axial y apendicular. El paciente evolucionó con síndrome de lisis tumoral tras el tratamiento clínico con corticoides por sospecha de linfoma, y no se resistió. Previamente se realizó biopsia hepática guiada por tomografía con perfil inmunohistoquímico compatible con angiosarcoma hepático. El angiosarcoma es un tumor raro, de difícil diagnóstico y tratamiento por su evolución agresiva y deficientes hallazgos clínicos y de laboratorio. Los exámenes por imágenes son inespecíficos y la posibilidad de esta enfermedad debe considerarse en el diagnóstico diferencial de la investigación de neoplasias hepáticas. Las opciones terapéuticas son limitadas. Reportes de casos como este son importantes para incrementar el grado de sospecha clínica y el diagnóstico precoz de este tipo de evolución catastrófica habitual.

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Systemic mastocytosis (SM) pathology is extremely rare in canine practice, with insufficient reported data. The knowledge of the clinical behavior of this pathology is scarce. In human medicine, SM has been widely investigated, being defined as a rare hematopoietic disorder by the World Health Organization (2016), within the type of myeloproliferative neoplasms. Herein, we describe a systemic mastocytosis case in a Portuguese Serra-da-Estrela dog, where a cutaneous grade III/high-grade MCT was also diagnosed. The clinical decline of the animal and owner's insistence throughout anamnesis that the dog was markedly different after the cytologic exam performed in another clinic, along with both severe eosinophilia and hepatomegaly, led to the clinical suspicion of SM. The animal passed away 7 days later. Post-morteminvestigation confirmed SM pathology, and a deletion of 15 base pairs change on c-Kit gene exon 11 was identified. Contemplating the low number of cases described in the literature, this publication aims to disclose clinical and laboratory features of rare and poorly described canine SM, taking into consideration human outcomes described in the literature.(AU)

A patologia da mastocitose sistêmica (SM) é extremamente rara na prática clínica canina, com escassos casos descritos na literatura científica. O conhecimento do comportamento clínico desta patologia é mínimo. Na medicina humana, a SM tem sido amplamente investigada, sendo definida como uma doença hematopoiética rara pela Organização Mundial da Saúde (2016), dentro do tipo de neoplasias mieloproliferativas. Descrevemos aqui um caso de mastocitose sistêmica num cão Serra-da-Estrela português, diagnosticado também com um mastocitoma cutâneo grau III / alto grau. O declínio clínico do animal e a insistência do proprietário durante a anamnese de que o cão estava marcadamente diferente após o exame citológico realizado em outra clínica, juntamente com eosinofilia e hepatomegalia graves, levantaram a suspeita clínica de SM. O animal faleceu 7 dias depois. A investigação post-mortem confirmou a patologia SM, e o estudo molecular revelou uma deleção de 15 pares de bases no exon 11 do gene c-Kit. Contemplando o baixo número de casos descritos na literatura, o objetivo desta publicação é divulgar características clínicas e laboratoriais de SM canina, levando em consideração informações clínicas descritas em humanos.(AU)

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Systemic mastocytosis (SM) pathology is extremely rare in canine practice, with insufficient reported data. The knowledge of the clinical behavior of this pathology is scarce. In human medicine, SM has been widely investigated, being defined as a rare hematopoietic disorder by the World Health Organization (2016), within the type of myeloproliferative neoplasms. Herein, we describe a systemic mastocytosis case in a Portuguese Serra-da-Estrela dog, where a cutaneous grade III/high-grade MCT was also diagnosed. The clinical decline of the animal and owner's insistence throughout anamnesis that the dog was markedly different after the cytologic exam performed in another clinic, along with both severe eosinophilia and hepatomegaly, led to the clinical suspicion of SM. The animal passed away 7 days later. Post-morteminvestigation confirmed SM pathology, and a deletion of 15 base pairs change on c-Kit gene exon 11 was identified. Contemplating the low number of cases described in the literature, this publication aims to disclose clinical and laboratory features of rare and poorly described canine SM, taking into consideration human outcomes described in the literature.(AU)

A patologia da mastocitose sistêmica (SM) é extremamente rara na prática clínica canina, com escassos casos descritos na literatura científica. O conhecimento do comportamento clínico desta patologia é mínimo. Na medicina humana, a SM tem sido amplamente investigada, sendo definida como uma doença hematopoiética rara pela Organização Mundial da Saúde (2016), dentro do tipo de neoplasias mieloproliferativas. Descrevemos aqui um caso de mastocitose sistêmica num cão Serra-da-Estrela português, diagnosticado também com um mastocitoma cutâneo grau III / alto grau. O declínio clínico do animal e a insistência do proprietário durante a anamnese de que o cão estava marcadamente diferente após o exame citológico realizado em outra clínica, juntamente com eosinofilia e hepatomegalia graves, levantaram a suspeita clínica de SM. O animal faleceu 7 dias depois. A investigação post-mortem confirmou a patologia SM, e o estudo molecular revelou uma deleção de 15 pares de bases no exon 11 do gene c-Kit. Contemplando o baixo número de casos descritos na literatura, o objetivo desta publicação é divulgar características clínicas e laboratoriais de SM canina, levando em consideração informações clínicas descritas em humanos.(AU)

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Resumen Introducción: Los linfomas de células T son infrecuentes y se caracterizan por presentarse en la población de los adultos jóvenes. Además, suele acompañarse de patologías como la anemia moderada, hepatoesplenomegalia y trombocitopenia e infiltración sinusoidal por linfocitos T en células de médula ósea, bazo e hígado. Caso clínico: Se presenta un caso clínico de un adolescente que tiene los síntomas característicos de esta patología, con sospecha clínica y diagnóstico paraclínico confirmado con histoquímica de médula ósea. Conclusión: Es una entidad infrecuente de pronóstico desfavorable, hasta el momento el paciente está estable recibiendo tratamiento. Para utilizar el enfoque adecuado en el diagnóstico y brindar tratamiento, es necesario considerar todos los hallazgos clínicos.

Abstract Introduction: T-cell lymphomas are uncommon; these tend to be present in young adult patients. Additionally, this condition is characterized by the existence of pathologies like moderate anemia, hepatosplenomegaly disorder, thrombocytopenia and sinusoidal infiltration by T-Lymphocytes in bone marrow cells, spleen and liver. In this study a case of this rare lymphoma is going to be presented. Case report: A clinical case of an adolescent who presents the characteristic symptoms of this pathology is exposed. This clinical suspicion held a paraclinical diagnosis that was confirmed by histochemistry of bone marrow tests. Conclusion: It is an infrequent condition with an unfavorable prognosis. Until now the patient remains stable and is receiving treatment, the clinical findings of the disease raise awareness about the importance of carrying out the appropriate diagnosis procedures and providing treatment.

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RESUMEN El linfoma de Burkitt, se trata de un subtipo poco frecuente del linfoma no Hodgkin, con elevada frecuencia en aquellos pacientes con sida. La hepatoesplenomegalia es un signo clínico de gran importancia para el diagnóstico oportuno de algunas patologías; entre los mecanismos de formación de la hepatoesplenomegalia se encuentra la infiltración celular, ocasionada por la migración de células tumorales. Se presenta por inflamaciones debido a la presencia de infecciones por virus o bacterias las cuales son muy comunes en pacientes con sida. Se presentó un caso de un paciente masculino de 4 años, diagnosticado con VIH positivo, con la configuración correspondiente de criterios clínicos en clasificación C para sida. El cual desarrolló a nivel de cavidad oral un Burkitt primario, que se acompañó de hepatoesplenomegalia. Se pretendió describir la relación y el comportamiento de este tipo de linfoma con la hepatoesplenomegalia, así como la repercusión a nivel del sistema estomatognático, a nivel sistémico y el plan de tratamiento. Por el cuadro clínico e inmunológico del paciente estudiado, se planteó un pronóstico reservado por presentar un cuadro clínico infrecuente, en el que se observó Burkitt; tanto a nivel del sistema estomatognático como a nivel abdominal. Se hizo necesario realizar un diagnóstico oportuno y certero para iniciar el tratamiento a tiempo, se comenzó inmediatamente con tratamiento (AU).

ABSTRACT Burkitt lymphoma (BL) is a rare subtype of non-Hodgkin lymphoma, with high frequency in those patients with AIDS. Hepatosplenomegaly is a clinical sign of great importance for the timely diagnosis of some pathologies; cellular infiltration is found among the mechanisms of hepatosplenomegaly formation; it is caused by the migration of tumor cells. It emerges by inflammations due to the presence of infections by virus or bacteria which are very common in patients with AIDS. The authors present the case of a male patient, aged 4 years, with a positive HIV diagnosis, and the correspondent configuration of clinical criteria in C classification for AIDS, who developed a primary Burkitt lymphoma at the level of oral cavity We present the case of a 4-year-old male patient diagnosed with HIV positive, with the corresponding configuration of clinical criteria in classification C for AIDS; who developed a primary LB at the oral cavity level that was accompanied by hepatosplenomegaly. The authors pretended to describe the relation and behavior of this kind of lymphoma with hepatosplenomegaly, and also the repercussion at the stomatognathic level, at the systemic level and the treatment plan. Due to the clinical and immunological characteristics of the studied patient a reserved prognosis was given because of presenting infrequent clinical characteristics in which a Burkitt was observed both, at the stomatognathic and at the abdominal level. It was necessary to make an opportune and accurate diagnosis to begin the treatment on time (AU).

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Introducción: La histoplasmosis es una enfermedad causada por el hongo Histoplasma capsulatum descrita por primera vez por Samuel Taylor Darling en un trabajador de la zona del canal de Panamá. La exposición al hongo es frecuente en áreas tropicales y subtropicales, la mayoría son infecciones asintomáticas o ligeramente sintomáticas Los pacientes pediátricos son más vulnerables a padecer formas graves de histoplasmosis, especialmente menores de dos años. Materiales y métodos: Se realizó un estudio transversal retrospectivo, en pacientes diagnosticados con histoplasmosis en el Hospital Materno Infantil José Domingo de Obaldía de enero de 2008 a diciembre 2018. Resultados:Se analizaron 8 casos en 11 años (2008-2018), El principal grupo afectado fueron los menores de 2 años (n=7). Las características clínicas encontradas fueron: hepatomegalia y esplenomegalia en todos los pacientes, además, fiebre, adenopatías, anorexia, y astenia Se encontró trombocitopenia en todos los casos, seguido de anemia y leucopenia; e infiltrado alveolo intersticial en la radiografía de tórax de 6 pacientes. No se encontraron pacientes con infección por VIH. El método diagnóstico fue a través de estudios histopatológicos en el aspirado de médula ósea. El tratamiento utilizado fue anfotericina B, seguido de Itraconazol. De los 8 pacientes, 6 fallecieron con una letalidad del 75%. Conclusiones: Es una entidad poco frecuente en nuestra institución. Tiene una alta letalidad, siendo los más vulnerables los menores de 2 años; representando el 89% de las defunciones. Los pacientes presentaron datos de infección diseminada. Se debe sospechar histoplasmosis en pacientes con hepatomegalia, esplenomegalia, trombocitopenia, anemia y/o leucopenia.

Introduction: Histoplasmosis is a disease caused by the Histoplasma capsulatum fungus, first described by Samuel Taylor Darling in a worker in the Panama Canal area. Exposure to the fungus is common in tropical and subtropical areas, most are asymptomatic or slightly symptomatic (self-limited) infections. Pediatric patients are more vulnerable to severe forms of histoplasmosis, especially under two years. Materials and methods: A retrospective cross-sectional study was carried out in patients diagnosed with histoplasmosis at Hospital Materno Infantil José Domingo de Obaldía from January 2008 to December 2018. Results: 8 cases were analyzed in 11 years (2008-2018), mostly of the male sex (n = 5). The main age group affected were those under 2 years of age (n = 7). The clinical features found were hepatomegaly and splenomegaly in all patients, in addition, fever, lymphadenopathy, anorexia, and asthenia. In the complementary studies thrombocytopenia was found in all cases, followed by anemia and leukopenia, in addition to alveolo-interstitial infiltrate on the chest radiograph of 6 patients. The diagnostic method was histopathological studies in bone marrow aspirate. The treatment used was amphotericin B, followed by Itraconazole. Of the 8 patients, 6 died with a lethality of 75%. Conclusions: It is a rare entity in our institution. It has a high lethality, the most vulnerable being those under 2 years. Patients presented disseminated infection data. Histoplasmosis should be suspected in patients with hepatomegaly, splenomegaly, thrombocytopenia, anemia and (or ) leukopenia.

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Resumen ANTECEDENTES: Los leiomiomas uterinos son los tumores benignos ginecológicos más frecuentes en las mujeres en edad reproductiva; por tanto, son extremadamente raros en las adolescentes (menos de 1%) y solo se encuentran reportes de caso en la bibliografía. CASO CLÍNICO: Paciente de 16 años, con tumor suprapúbico de rápido crecimiento, acompañado de dolor abdominal generalizado, enviada a la unidad médica con diagnóstico de miomatosis uterina para descartar su malignidad. El ultrasonido pélvico reportó múltiples lesiones hipoecoicas diseminadas en el miometrio, similares a metástasis. La tomografía abdomino-pélvica informó hepatomegalia, a expensas del lóbulo izquierdo y leiomiomas intramurales de medianos y grandes elementos; los marcadores tumorales se encontraron en límites normales. En la laparotomía se encontró una tumoración interligamentaria izquierda de 25 cm; el estudio histopatológico reportó: leiomioma uterino de patrón histológico convencional. CONCLUSIONES: El cuadro clínico de los leiomiomas en adolescentes representa un reto clínico por la edad, signos, síntomas y necesidad de preservación de la función reproductiva, aspectos decisivos a la hora de decidir el tratamiento.

Abstract BACKGROUND: The uterine leiomyomas are the most common gynecological benign tumors in fertile females; however, they are very rare in adolescents (less than 1%) and there are only a few reports in the literature. CLINICAL CASE: A 16-year-old patient with a fast-growing suprapubic tumor, accompanied by generalized abdominal pain, sent to the hospital with diagnosis of pelvic tumor probable uterine miomatosis, to rule out malignant tumor. Pelvic ultrasound was performed with a report of multiple disseminated hypoechoic lesions in myometrium giving appearance of metastasis, without being the characteristic images of myomas, pelvic abdominal tomography reported hepatomegaly of the left lobe, intramural leiomyomas of medium and large elements, tumor markers within normal limits. The laparotomy was performed, left intraligamentary myoma of giant elements was located; and the histopathological study reported uterine leiomyoma of conventional histological pattern. CONCLUSIONS: The clinical of leiomyomas in adolescent patients represents a challenge for the gynecologist. The age, symptomatology, and preservation of reproductive function are important aspects to decide the therapeutic regimen.

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La enfermedad de Gaucher es una patología de depósito lisosomal, autosómica recesiva, con mutación en el gen GBA, que afecta principalmente al hígado, bazo, huesos y a la médula ósea. Es una enfermedad rara con baja incidencia mundial. Existen 3 tipos; el tipo I es el más frecuente en la población pediátrica, y el tipo III es el de peor pronóstico y presenta manifestaciones neurológicas. El diagnóstico se realiza con el tamizaje prenatal de enfermedades de depósito, más específicamente de mutaciones GBA. El diagnóstico definitivo se realiza al detectar ß-glucocerebrosidasa, y los estudios genéticos para la tipificación del gen afectado: en el tipo 1 mutación N370S y mutaciones L444P o D409H en tipo 2 y 3. El tratamiento se realiza con terapia de reemplazo enzimático o con la terapia de reducción de sustrato. Describimos en este artículo a un paciente preescolar del Hospital Enrique Garcés, con cuadro respiratorio, hepato-esplenomegalia y afectación de las tres líneas celulares sanguíneas.

Gaucher's disease is an autosomal recessive lysosomal storage pathology, with mutation in the GBA gene, which mostly affects the liver, spleen, bone and bone marrow. It is a rare disease with low global incidence. There are 3 types: type I is the most frequent in the pediatric population, and type III has the worst prognosis and presents neurological manifestations. Diagnosis is made with prenatal screening of storage diseases, and more specifically GBA1 mutations. The definitive diagnosis is made by detecting ß-glucocerebrosidase, and genetic studies for the detarmination of the affected gene, in type 1 mutation N370S and L444P mutations or D409H in type 2 and 3. The treatment is enzyme replacement therapy or substrate reduction therapy. This article describes a preschool patient of Enrique Garcés Hospital, with respiratory symptoms, hepato-splenomegaly and involvement of the three blood cell lines.

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La pericarditis constrictiva es una entidad poco frecuente en pediatría, en la cual existe una limitación para la diástole cardíaca por fibrosis del pericardio. El origen etiopatogénico de esta patología es múltiple, encontrándose en primer lugar, la pericarditis constrictiva idiopática y, en segundo, la infección por Mycobacterium tuberculosis. El diagnóstico constituye un desafío clínico, ya que requiere de un alto grado de sospecha. Suele presentarse de forma oligosintomática. La presencia de edema, ascitis y alteración de la función hepática suele orientar el estudio hacia una enfermedad hepática primaria. Una cuidadosa historia clínica y examen físico, junto con estudios por imágenes adecuados, constituyen las piedras angulares del diagnóstico. El tratamiento quirúrgico realizado de forma oportuna resulta curativo en la gran mayoría de los pacientes. Se presenta el caso de un paciente de 16 años que inicia estudios por hallazgo de hepatomegalia asociada a disnea grado 1-2 en un control de salud habitual.

Constrictive pericarditis is a rare entity in pediatrics in which there is a limitation for cardiac diastole due to fibrosis of the pericardium. The etiopathogenic origin of this pathology is multiple, finding idiopathic constrictive pericarditis firstly and Mycobacterium tuberculosis infection secondly. Diagnosis is a clinical challenge since it requires a high degree of suspicion. It usually presents as oligosymptomatic or with signs and symptoms of low cardiac output. The presence of edema, ascites and impaired liver function usually guides the study towards primary liver disease. A careful clinical history and physical examination together with adequate imaging studies are the cornerstones of the diagnosis. Surgical treatment is curative in the vast majority of patients. We present the case of a 16-year-old patient with hepatomegaly and dyspnea grade 1-2 found in a routine health check-up.

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INTRODUCCIÓN: El hemangioendotelioma hepático (HEH) es un tumor benigno, raro en niños, que se presenta frecuentemente en el primer año de vida. La presentación clínica es variable y su diagnóstico se realiza en base a la sospecha clínica, estudios de laboratorio y de imagen. El objetivo fue describir un caso de hemangioendotelioma hepático multifocal. CASO CLÍNICO: Niña de 3 meses que presentó hepatomegalia sin elementos de falla hepática ni cardiaca. Se realizó ecografía y tomografía de ab domen que orientaron al diagnóstico de HEH que se confirmó con la angioresonancia de abdomen. Recibió glucocorticoides a altas dosis en forma prolongada. Al año y medio de iniciado el tratamiento se evidenció remisión del tumor. Presentó efectos secundarios por el tratamiento instaurado. CONCLUSIONES: La presencia de una hepatomegalia aislada en un lactante asintomático debe hacer pensar en una probable patología tumoral, orientándonos por la clínica e imagenología al diagnóstico nosológico de la misma. Siempre se debe sospesar las posibles complicaciones con los riesgos de los tratamientos a realizar. En este caso la extensión del tumor y sus probables complicaciones justificó el uso de corticoterapia prologada a altas dosis a pesar de sus efectos adversos.

INTRODUCTION: Hepatic hemangioendothelioma is a rare benign tumor in children, which frequently occurs in the first year of life. The clinical presentation is variable and the diagnosis is based on clinical suspicion, and laboratory and imaging studies. The objective was to describe a case of multifocal hepa tic hemangioendothelioma. CLINICAL REPORT: 3-month-old girl who presented hepatomegaly without elements of hepatic or heart failure. Abdominal ultrasound and CT scan were used to diagnose hepatic hemangioendothelioma, which was confirmed by CT abdominal angiography. The patient received glucocorticoid treatment at high doses for a prolonged period. A year and a half after treatment, there was evidence of tumor remission. She had side effects from the established treatment. CONCLUSIONS: In asymptomatic patients with isolated hepatomegaly, it should be considered a probable tumor patho logy, considering the clinic and imaging studies. Possible complications and treatments risks must always be assessed. In this case, the tumor extension and its probable complications justified the use of prolonged corticosteroid therapy at high doses despite its adverse effects.

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INTRODUCCIÓN. Los abscesos hepáticos piógenos en neonatos son una entidad rara con menos de cien casos reportados en la literatura mundial. El diagnóstico en esta edad es difícil porque tanto signos y síntomas son inespecíficos y se relacionan con sepsis. OBJETIVOS. Identificar la clínica y características patológicas del absceso hepático en neonatos y correlacionar al posicionamiento de catéter umbilical en hígado como factor de riesgo asociado para desarrollar absceso hepático en neonatos. MATERIALES Y MÉTODOS. Estudio retrospectivo, descriptivo, informes de casos, con una muestra y población conocida, de 5 pacientes recién nacidos. Criterios de Inclusión: neonatos de ambos sexos con edad gestacional mayor a las 34 semanas de gestación, con antecedentes de mal posicionamiento de catéteres umbilicales documentado mediante un examen de imagen "radiografía". Criterios de Exclusión: pacientes sin el antecedente de cateterización umbilical y/o que no se haya documentado mediante estudio de imagen con la evidencia del catéter colocado de manera errónea en tejido hepático. Se utilizó la base de datos registrada en los archivos del Servicio de Gastroenterología Pediátrica de las interconsultas solicitadas del Servicio de Neonatología y Unidad de Cuidados Intensivos Neonatales del, Hospital Civil de Guadalajara "Dr. Juan I. Menchaca". Jalisco- México, en un tiempo de 5 años, desde Enero 2013 a 2018. RESULTADOS. Fueron cinco pre términos (n=5) entre 34 y 36 semanas, con edad gestacional media de 35 semanas y 2039 gr de peso al nacer. El promedio de días para el diagnóstico fue de 7,8. Cuatro pacientes tuvieron hepatomegalia 80,0% (4;5), el volumen de los abscesos fueron entre 2 y 12mm en lóbulo hepático derecho. CONCLUSIÓN. El absceso hepático es poco común pero su clínica debe sospecharse en un recién nacido con sepsis y antecedente de cateterismo umbilical. El manejo y evolución clínico favorable en el 100,0 % (5;5), de los neonatos y no se requirió resolución quirúrgica.

INTRODUCTION. Pyogenic liver abscesses in neonates are a rare entity with less than one hundred cases reported in the world literature. Diagnosis at this age is difficult because both signs and symptoms are nonspecific and are related to sepsis. OBJECTIVES. Identify the clinical and pathological characteristics of liver abscess in neonates and correlate to the positioning of umbilical catheters in the liver as an associated risk factor for developing liver abscess in neonates. MATERIALS AND METHODS. Retrospective, descriptive study, case reports, with a sample and known population, of 5 newborn patients. Inclusion Criteria: neonates of both sexes with gestational age over 34 weeks of gestation, with a history of poor positioning of umbilical catheters documented by an "x-ray" imaging exam. Exclusion Criteria: patients without a history of umbilical catheterization and / or who have not been documented by imaging study with evidence of the catheter incorrectly placed in liver tissue. The database registered in the archives of the Pediatric Gastroenterology Service of the requested interconsultations of the Neonatal and Neonatal Intensive Care Unit of the Civil Hospital of Guadalajara "Dr. Juan I. Menchaca ". Jalisco- Mexico, in a time of 5 years, from January 2013 to 2018. RESULTS. There were five pre terms (n = 5) between 34 and 36 weeks, with an average gestational age of 35 weeks and 2039 grams of birth weight. The average number of days for diagnosis was 7,8. Four patients had 80,0% hepatomegaly (4; 5), the volume of abscesses was between 2 and 12mm in the right hepatic lobe. CONCLUSION. Hepatic abscess is uncommon but its clinic should be suspected in a newborn with sepsis and a history of umbilical catheterization. Management and favorable clinical evolution in 100,0% (5; 5) of infants and no surgical resolution was required.

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RESUMEN Fundamento: aunque la enfermedad hepática crónica como complicación de la fibrosis quística ha sido eclipsada por otros signos y síntomas más obvios a nivel respiratorio y pancreático, constituye la segunda causa de muerte en los pacientes con fibrosis quística. Objetivo: presentar el caso de una transicional de tres años con hepatomegalia y pruebas analíticas hepáticas alteradas como manifestaciones iniciales de la fibrosis quística. Caso clínico: paciente de tres años, femenina, con historia de distensión abdominal, hepatomegalia y desnutrición. Por esta sintomatología es remitida a la consulta de Gastroenterología. Conclusiones: se debe sospechar la fibrosis quística en pacientes pediátricos con hepatopatía crónica. Se observó mejoría clínica de las manifestaciones respiratorias y del estado nutricional, sin embargo, la hepatopatía evolucionó a la cirrosis hepática establecida.

ABSTRACT Background: although the chronic hepatic illness as complication of the cystic fibrosis has been eclipsed by other signs and more obvious symptoms at breathing and pancreatic level, is the second cause of death in the patients with cystic fibrosis. Objective: to present the case of a three year-old transitional one with hepatomegaly and hepatic analytic tests altered as initial manifestations of the cystic fibrosis. Clinical case: a three-year old, female patient with history of abdominal distension, hepatomegaly, and malnutrition. For this symptoms, she is remitted to the Gastroenterology consultation. Conclusions: the cystic fibrosis should be suspected in pediatric patient with chronic hepatic diseases. Clinical improvement of the breathing manifestations was observed and of the nutritional state, however the chronic hepatic disease evolved to the established hepatic cirrhosis.

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As intoxicações em animais de produção causadas por plantas são bastante relatadas, resultando em perdas econômicas diretas causadas pela morte dos animais, diminuição dos índices produtivos e reprodutivos, além de aumentar a ocorrência e susceptibilidade a outras doenças. Já as perdas indiretas, incluem os custos para o controle das plantas tóxicas nas pastagens, além das medidas de manejo como a utilização de cercas e o pastoreio alternativo para evitar as intoxicações, assim como também os gastos associados ao diagnóstico e tratamento. No presente trabalho relatou-se a intoxicação de um bovino de raça Girolando de 11 anos pelo consumo da planta conhecida como bordão-de-velho (Samanea tubulosa). As alterações pós-mortem macro e microscópicas demonstraram que a causa provável de morte foi a síndrome de disfunção de múltiplos órgãos. Estudos in vivo são necessários para conhecer mais a fundo o potencial tóxico desta planta.

Intoxications in production animals caused by plants are well reported, resulting direct losses caused by the death of animals, by the reduction of productive and reproductive indexes, as well as increase in the frequence and susceptibility to other diseases. Indirect losses can be attributed to control plants in pastures, in addition to manipulation measures, such as use of fences and alternative grazing to avoid intoxication. The present work reported the intoxication of an 11-year-old female breed of cattle by the consumption of the plant known as bordão-develho (Samanea tubulosa). Macro and microscopic finds demonstrate that the probable cause is multiple organ dysfunction syndrome. In vivo studies are needed to know more about the toxic potential of this plant.

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As intoxicações em animais de produção causadas por plantas são bastante relatadas, resultando em perdas econômicas diretas causadas pela morte dos animais, diminuição dos índices produtivos e reprodutivos, além de aumentar a ocorrência e susceptibilidade a outras doenças. Já as perdas indiretas, incluem os custos para o controle das plantas tóxicas nas pastagens, além das medidas de manejo como a utilização de cercas e o pastoreio alternativo para evitar as intoxicações, assim como também os gastos associados ao diagnóstico e tratamento. No presente trabalho relatou-se a intoxicação de um bovino de raça Girolando de 11 anos pelo consumo da planta conhecida como bordão-de-velho (Samanea tubulosa). As alterações pós-mortem macro e microscópicas demonstraram que a causa provável de morte foi a síndrome de disfunção de múltiplos órgãos. Estudos in vivo são necessários para conhecer mais a fundo o potencial tóxico desta planta.(AU)

Intoxications in production animals caused by plants are well reported, resulting direct losses caused by the death of animals, by the reduction of productive and reproductive indexes, as well as increase in the frequence and susceptibility to other diseases. Indirect losses can be attributed to control plants in pastures, in addition to manipulation measures, such as use of fences and alternative grazing to avoid intoxication. The present work reported the intoxication of an 11-year-old female breed of cattle by the consumption of the plant known as bordão-develho (Samanea tubulosa). Macro and microscopic finds demonstrate that the probable cause is multiple organ dysfunction syndrome. In vivo studies are needed to know more about the toxic potential of this plant.(AU)

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