Malformación linfática quística gigante del hígado: dos casos pediátricos con diferente abordaje quirúrgico / Giant cystic lymphatic malformation of the liver: two pediatric cases with different surgical approach

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.

Cistoadenoma mucinoso biliar: Un reto diagnóstico / Mucinous biliary cystadenoma: A diagnostic challenge

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

Combined liver-kidney transplant in polycystic diseases: a case report

ABSTRACT Polycystic liver disease, a hereditary pathology, usually manifests as autosomal dominant polycystic kidney disease. The many cysts in the liver cause massive hepatomegaly, majorly affecting the patient's quality of life. In cases of refractory symptoms, liver transplantation is the only treatment choice. A 43-year-old woman was followed up as a hepatology outpatient in August 2020, with a progressive increase in abdominal volume, lower limb edema, and cachexia. The patient was diagnosed with polycystic renal and liver disease with massive hepatomegaly in March 2021, a combined kidney-liver transplant. Liver size represented 13% of the patient's corporal composition, weighing 8.6kg. The patient was discharged on the 7th postoperative day with no complications. Only 10-20% of patients with polycystic liver disease have clinical manifestations, most of which result from hepatomegaly. An increase in liver volume deteriorates liver function until the condition becomes end-stage liver disease, as kidney function is already compromised; liver-kidney transplantation remains the only treatment choice. The case described drew significant attention to the massive hepatomegaly presented in the patient, with the liver representing over 10% of the patient's body weight, approximately five to six times larger than a normal-sized liver.

Schisandrae Fructus oil-induced elevation in serum triglyceride and lipoprotein concentrations associated with physiologic hepatomegaly in mice

Objective: To investigate hypertriglyceridemia and hepatomegaly caused by Schisandrae Sphenantherae Fructus (FSS) and Schisandra chinensis Fructus (FSC) oils in mice. Methods: Mice were orally administered a single dose of Schisandrae Fructus oils. Serum and hepatic triglyceride (TG), triglyceride transfer protein (TTP), apolipoprotein B48 (Apo B48), very-low-density lipoprotein (VLDL), hepatocyte growth factor (HGF), alanine aminotransfease (ALT) and liver index were measured at 6-120 h post-dosing. Results: FSS and FSC oil caused time and dose-dependent increases in serum and hepatic TG levels, with maximum increases in the liver (by 297% and 340%) at 12 h post-dosing and serum (244% and 439%) at 24-h post-dosing, respectively. Schisandrae Fructus oil treatments also elevated the levels of serum TTP by 51% and 63%, Apo B48 by 152% and 425%, and VLDL by 67% and 38% in mice, respectively. FSS and FSC oil treatments also increased liver mass by 53% and 55% and HGF by 106% and 174%, but lowered serum ALT activity by 38% and 22%, respectively. Fenofibrate pre/ co-treatment attenuated the FSS and FSC oil-induced elevation in serum TG levels by 41% and 49% at 48 h post-dosing, respectively, but increased hepatic TG contents (by 38% and 33%, respectively) at 12 h post-dosing. Conclusions: Our findings provide evidence to support the establishment of a novel mouse model of hypertriglyceridemia by oral administration of FSS oil (mainly increasing endogenous TG) and FSC oil (mainly elevating exogenous TG).

Angiossarcoma hepático primário: uma trágica evolução ­ relato de caso e revisão de literatura / Primary hepatic angiosarcoma: a tragic evolution ­ case report and literature review / Angiosarcoma hepático primario: una evolución trágica ­ reporte de caso y revisión de la literatura

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.

Enfermedad de Caroli: revisión sistemática de la literatura / Characterization of patients with Caroli's disease: Systematic review

Resumen Introducción: La enfermedad de Caroli es un trastorno congénito poco usual, el cual cursa con dilatación segmentaria multifocal de los conductos biliares intrahepáticos. Fue descrita por Jacques Caroli en 1958 como una dilatación sacular, segmentada o fusiforme de los ductos biliares intrahepáticos. Objetivo: Caracterizar a la población que ha padecido la enfermedad de Caroli. Metodología: Revisión sistemática de la literatura. Resultados: Se analizaron 66 artículos, el grupo de edad más afectado es los menores de 10 años, en el que se evidenció una mayor prevalencia en varones, y se encontró asociado con otras comorbilidades como el riñón poliquístico (20 %). La manifestación más frecuente fue la hepatomegalia (44,7 %), seguido de la fiebre (42,4 %) y el dolor abdominal en el hipocondrio derecho (41,2 %). Como método diagnóstico más utilizado se encontraba la resonancia magnética (73,8 %); dentro de los hallazgos predominó la dilatación intrahepática (76,5 %) y el manejo más empleado fue la antibioticoterapia para tratar las recurrencias por colangitis. Conclusión: La enfermedad de Caroli tiene una baja prevalencia, se presenta con mayor frecuencia en el continente americano, afecta principalmente a la primera década de la vida, tiene predilección por el sexo masculino y se caracteriza por una dilatación de los conductos intrahepáticos que pueden afectar a otros órganos como los riñones, lo que produce quistes renales.

Abstract Caroli's disease (CD) is a rare congenital disease, which presents with multifocal segmental intrahepatic bile duct dilatation. It was first described by Jacques Caroli in 1958 as a saccular or fusiform dilatation of the intrahepatic bile ducts. Objective: To characterize the population that has been diagnosed with Caroli's disease. Materials and methods: Systematic review. Results: 66 articles were analyzed. The age group most affected was children under 10 years old, where a higher prevalence was evidenced in males. This condition was associated with other comorbidities such as polycystic kidney in 20%. The most frequent manifestation was hepatomegaly (44.7%), followed by fever (42.4%), and abdominal pain in the right upper quadrant (41.2%). The most used diagnostic method was magnetic resonance imaging in 73.8% of the sample. The findings showed predominance of intra-hepatic dilatation in 76.5%. The most widely used treatment was antibiotic therapy to treat recurrences due to cholangitis. Conclusion: Caroli's disease has an extremely low incidence and occurs more frequently in the American continent, affecting mainly patients in the first decade of life, with a predilection for the male sex. It is characterized by a dilatation of the intrahepatic ducts that can affect other organs such as the kidneys, causing renal cysts.

Rare and unknown canine systemic mastocytosis: clinical and laboratory features of an aberrant c-Kit mutation neoplasia / A rara e desconhecida mastocitose sistêmica canina: características clínicas e laboratoriais de uma neoplasia aberrante na mutação c-Kit

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)

Linfoma hepatoesplénico de células T en un paciente adolescente / Hepatosplenic T-cell lymphoma in an adolescent patient

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.

Hepatoesplenomegalia relacionada a linfoma de Burkitt en paciente pediátrico / Hepatoesplenomegalia related to Burkitt´s lymphoma in a pediatric patient

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).

Características clínicas, epidemiológicas, diagnóstico y tratamiento de los pacientes pediátricos con histoplasmosis en el Hospital Materno infantil José Domingo de Obaldía, desde enero 2008 a diciembre 2018 / Clinical, epidemiological characteristics, diagnosis and treatment of pediatric patients with histoplasmosis at the Hospital Materno Infantil José Domingo de Obaldia, since January 2008 to December 2018

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.

Peliosis Hepatis and Iron Deficiency – An Interesting Case Report

Peliosis hepatis (PH) is a rare condition showing presence of multiple blood-filled cystic cavities in the liver. It does not have any gender predilection, and is suspected to be idiopathic. However, in patients with predisposing diseases, its prevalence can range from 0.2 to 22%. The association between PH and anemia has not been completely established. PH has been reported in a patients with hematologic disease, and also in patient with spherocytic haemolytic anemia. It is also suggested that acute sequestration of blood can happen in these sinusoidal cavities, which can lead to development of anemia and thrombocytopenia. We present a case of 40-year female who presented with abdominal pain and recurrent iron deficiency anemia. On examination, she was severely pale, and had moderate hepatosplenomegaly. Ultrasonography showed hepatomegaly and splenomegaly, and her blood investigations revealed severe iron deficiency with bone marrow showing hypercellular marrow with depleted iron stores. Upper gastrointestinal endoscopy was normal. Liver biopsy showed changes suggestive of peliosis hepatis. She was treated with iron andmultiple blood transfusions and is in good health 6 months post presentation. Also, the association between anemia and PH has not been established

Clinical and laboratory profile of dengue fever in children in a tertiary care hospital of Navi Mumbai, India

Background: Dengue fever is a mosquito born arboviral illness endemic in tropical countries and causes significant mortality and morbidity due to lack of definitive treatment. The objective of this study was to assess the clinical and laboratory profile of confirmed cases of dengue in children up to 12 years of age and also to charactrise the risk factors for severe dengue.Methods: This is a descriptive, observational, retrospective study done in the Department of Pediatrics, Terna Medical College, Nerul, Navi Mumbai, India.   Medical records of all children up to 12 years of age diagnosed to have dengue   were reviewed.  Their clinical and laboratory profile were recorded in a pro forma and analyzed. All cases were classified as per WHO guidelines into non severe and severe dengue cases.Results: Among the 117 confirmed dengue cases, (84.6%) had non severe dengue and (15.4%) had severe dengue. The most common age group affected was 9-12 years (45.3%) with a male to female ratio of 2.4:1. Fever was the most common clinical feature seen in all cases followed by headache (83%), myalgia (81%), ascites (24.7%), vomiting (17.9%). Clinical signs of ascitis, hepatomegally, gastrointestinal bleeding, pleural effusion and shock were predominantly associated with severe dengue cases. Laboratory parameters showed leukopenia in 58.1% and thrombocytopenia (platelet counts <20,000/cumm3) in16.2% cases. Elevated liver enzymes, raised hematocrit (36.3%) and coagulation abnormalities were seen in over 50% dengue cases and were significantly associated with severe dengue dengue.Conclusions: Knowledge of clinical   and laboratory profile of dengue cases of a particular area will help in early prediction of risk factors for severe dengue resulting in favourable outcome of such cases.

Analysis of predictors in outcome of fever with thrombocytopenia of pediatric age upto 12 years

Background: It is well recognized that many fetomaternal and neonatal conditions are associated with thrombocytopenia. Study aimed to establish the possible etiology of children presenting with thrombocytopenia.Methods: The study was carried out in 100 children 1 month-12 years, admitted at department of paediatrics, Narayana Medical College and Hospital, Nellore, Andhra Pradesh, India with fever and thrombocytopenia. A thorough history was obtained and a general and systemic examination done. Routine investigations were done in all cases and specific investigations as and when required.Results: Total 100 cases were studied. Among them, 65% developed only fever, 10% developed fever with bleeding and 25% developed fever with shock. In total admissions 55% of children admitted with warning symptoms including abdominal pain, vomiting, reduced urine output and black colored stools. There is a significant influence of warning symptoms in predicting the outcome of fever with thrombocytopenia. 30% of children admitted with respiratory distress. 30% of children admitted with abdominal distension. 60% children admitted with hepatomegaly. In these children, 20% of them developed fever with shock and 13% of them developed fever with bleeding, 52% children admitted with platelet count less than 1 lakh. In these patients, 19% of them developed fever with shock and 9% of them developed fever with bleeding. 60% had dengue serology positive, 12% had positive Widal test, 8% children had positive urine culture and sensitivity. There was a significant influence of etiology in predicting the morbidity as 15% of children having positive serology for dengue developed fever with shock. 10% of children having positive serology for dengue developed fever with bleeding. Bleeding manifestations were seen most commonly in children with a platelet count less than 50000/?l.Conclusions: The commonest cause of febrile thrombocytopenia in this study was dengue fever in Children. Platelet count was the predictive of bleeding manifestations.

A study of various hepatic manifestations in dengue fever and their correlation with severity of dengue fever

Background: Hepatic involvement in Dengue is known with protean of manifestations ranging from hepatomegaly, elevated liver enzymes to fulminant hepatic failure. Aim of the study was to study the hepatic manifestations in children with dengue illness.Methods: This is a prospective Study with 60 Patients hospitalized with Dengue infection (Seropositive for Dengue). Dengue Seropositive patients are selected and examined for Hepatomegaly and Jaundice and subjected to complete blood count and Liver function tests were analysed.Results: Of 60 serologically confirmed cases hospitalized with dengue, were classified into (i)(DF), (ii) DHF I (iii) DHF II (iv) DHF III and (v) DHF IV.' In our study, upon 60 seropositive cases were reported at our hospital during the study period of which 18 were DF, 12 were DHF I, 15 were DHFII, 8 were DHF III and 7 were DHF IV respectively. The Hematocrit levels were raised 20% from the baseline in four classes of Dengue and not raised in DF. Most commonly occurred in age group of 5-7 years. Hepatomegaly was the commonest clinical sign seen. Thrombocytopenia was seen in 88% of all cases. Serum total bilirubin was raised in 10% of subjects with severe dengue infection in DHF III and DHF IV. Serum SGOT and SGPT was raised in 63.3% and 56.7% of patients with dengue of all classes including DF respectively. Thrombocytopenia occurred in 75% of patients with dengue fever, 98% with warning signs and 100% in severe dengue.Conclusions: In developing country like India, incidence of dengue outbreaks is increasing. Hepatic involvement of varying degrees have been reported. As hepatic dysfunction in dengue is transient and reversible, early identification of the same would help to reduce life threatening complications. The role of hepato protective drugs in reducing morbidity and mortality should be analysed by further studies.

Mioma intraligamentario de gran tamaño en una adolescente / Intra-ligamentary leiomyoma of giant elements in adolescent patient

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.

A study on clinical profile of typhoid fever at Government General Hospital, Nizamabad, Telangana, India

Background: The disease is most common in India. Children are most commonly affected. Typhoid fever, also known simply as typhoid, is a bacterial infection due to specific type of Salmonella that causes symptoms.Methods: Prospective observational study, 100 children with fever more than 7 days attending pediatric departed at GGH, Nizamabad were included in this study.Results: Total 100 children included in this study, male were 62, female were 38. More cases were (43) in 5-10 years age group, 53 children, belongs to middle class, 30 children belongs to lower class and 17 children belongs to upper class. Most common physical finding was toxic look (52), fallowed by coated tongue (41), hepatomegaly (32), pallor (12), spleenomegaly (11), hepatospleenomegaly (8).Conclusions: Safe drinking water, hand washing, proper sanitation, health education to children and food handlers in schools and hostels will decrease the incidence of typhoid fever in children.

Hepatomegalia como forma de presentación en pericarditis constrictiva: Caso clínico pediátrico / Hepatomegaly as a form of presentation in constrictive pericarditis: A pediatric clinical case

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.