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).

Glucogenosis hepática: a propósito de un caso / Hepatic glycogenosis: a case report

RESUMEN Las glucogenosis abarcan un rango de enfermedades que se caracterizan por el almacenamiento o utilización anormal del glucógeno, siendo los órganos más afectados el músculo y/o el hígado. La hepatomegalia puede ser un signo clínico que guie al diagnóstico. Describimos a un paciente de 15 años de edad con hepatomegalia, hipertransaminasemia y retraso del crecimiento, a quien se le diagnosticó glucogenosis por biopsia hepática.

ABSTRACT The glycogen storage diseases contain a range of diseases that are characterized by the abnormal storage or utilization of glycogen, the organs most affected being muscle and / or liver. Hepatomegaly may be a clinical sign that could guide to the diagnosis. We describe a 15-year-old patient with hepatomegaly, hypertransaminasemia and growth retardation. He was diagnosed with a glycogen storage disease by liver biopsy.

Generalidades de un paciente afebril con hepatoesplenomegalia en pediatría / Overview of an afebrile patient with hepatosplenomegaly in pediatrics

Introducción: La hepatoesplenomegalia es el agrandamiento simultaneo del hígado y del bazo, aunque es frecuente en edad pediátrica su literatura se centra en causa infecciosa, siendo también importante otras causas sobre todo en el paciente afebril Método: revisión de literatura actual confrontando con artículos de revisiones de temas en búsqueda electrónica en bases de datos de RIMA, MEDLINE, PUB-MED, MEDSCAPE, de 1981 a 2018. Resultados: La hepatomegalia en paciente afebril se puede encontrar en afectación sistémica o enfermedades hereditarias, algunas prevalentes o extremadamente raras, por lo cual se desarrolló esta revisión para agrupar las causas de esta en un paciente pediátrico afebril.

Introduction: Hepatosplenomegaly is the simultaneous enlargement of the liver and spleen, although it is frequent in pediatric age, its literature focuses on an infectious cause, and other causes are important, especially in the afebrile patient. Method: review of current literature comparing articles from subject reviews. in electronic search in RIMA, MEDLINE, PUB-MED, MEDSCAPE databases, from 1981 to 2018. Results: Hepatomegaly in afebrile patient can be found in systemic involvement or hereditary diseases, some prevalent or extremely rare, for which reason developed this review to group the causes of this in an afebrile pediatric patient.

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.

Tumor maligno congénito. Síndrome de Pepper / Congenital malignant tumor: Pepper's Syndrome

Pepper's syndrome refers to a neuroblastoma originated in the adrenal glands that usually metastasizes to the liver with abdominal development and respiratory involvement because of thoracic compression. The metastasic tumors are usually infrequent with an unfavorable prognosis. The cases reported in the world literature are very few. The congenital form of neuroblastoma is uncommon. The aim of this report was to describe a typical clinical case of a new born who died because of a metastasic malignant tumor, comptible with a Pepper's syndrome

Síndrome de Mauriac en Argentina en el siglo XXI: serie de 5 casos / Mauriac syndrome in Argentina in the XXI century: series of 5 cases

El síndrome de Mauriac se describe en diabéticos tipo 1 con mal control metabòlico, caracterizado por hepatomegalia, elevación de las transaminasas, rasgos cushingoides y retraso puberal, que pueden ser reversibles con un buen control glucémico. Son escasas las referencias bibliográficas publicadas en los últimos años luego de la introducción del automonitoreo glucémico, de nuevas insulinas y del tratamiento insulínico intensificado. Describimos una serie de cinco pacientes evaluados en nuestro servicio con características de síndrome de Mauriac y realizamos una revisión de las publicaciones de casos en Argentina.

Mauriac syndrome is associated with poor metabolic control of type 1 diabetes mellitus, hepatomegaly and elevated transaminases characterized by growth failure and delayed puberty, which may be reversible with good glycemic control. There are few references published in recent years following the introduction of glycemic self-monitoring, new insulins and intensified treatment. We describe a series of five patients evaluated in our service with Mauriac syndrome characteristics, conducting a literature review of cases in Argentina.

Hepatic Sinusoidal Obstruction Syndrome Caused by Herbal Medicine: CT and MRI Features

OBJECTIVE: To describe the CT and MRI features of hepatic sinusoidal obstruction syndrome (HSOS) caused by herbal medicine Gynura segetum. MATERIALS AND METHODS: The CT and MRI features of 16 consecutive Gynura segetum induced HSOS cases (12 men, 4 women) were analyzed. Eight patients had CT; three patients had MRI, and the remaining five patients had both CT and MRI examinations. Based on their clinical presentations and outcomes, the patients were classified into three categories: mild, moderate, and severe. The severity of the disease was also evaluated radiologically based on the abnormal hepatic patchy enhancement in post-contrast CT or MRI images. RESULTS: Ascites, patchy liver enhancement, and main right hepatic vein narrowing or occlusion were present in all 16 cases. Hepatomegaly and gallbladder wall thickening were present in 14 cases (87.5%, 14/16). Periportal high intensity on T2-weighted images was present in 6 cases (75%, 6/8). Normal liver parenchymal enhancement surrounding the main hepatic vein forming a clover-like sign was observed in 4 cases (25%, 4/16). The extent of patchy liver enhancement was statistically associated with clinical severity classification (kappa = 0.565). CONCLUSION: Ascites, patchy liver enhancement, and the main hepatic veins narrowing were the most frequent signs of herbal medicine induced HSOS. The grade of abnormal patchy liver enhancement was associated with the clinical severity.

Langerhans Cell Sarcoma in Two Young Children: Imaging Findings on Initial Presentation and Recurrence

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells with malignant cytological features and multi-organ involvement that typically has a poor prognosis. We experienced 2 cases of LCS in children less than 2 years of age and report them based primarily on CT and MR findings. Both children had findings of hepatosplenomegaly with low-attenuation nodular lesions, had multiple lymphadenopathy, and had shown recurrent lesions invading the skull during follow-up after chemotherapy.

Semiología cardiovascular / Cardiovascular semiology

La semiología cardiovascular en pediatría debe integrar todos los sistemas, tienen gran importancia el enfoque familiar, la historia del período antenatal, perinatal y postnatal, las características y cronología de aparición de los síntomas, su repercusión general y por último, el examen físico con sus componentes semiológicos que incluyan la inspección, la palpación, la percusión y la ascultación.

Semiology in pediatric cardiovascular must integrate all systems are of great importance the family approach, the history of the period antenatal, perinatal and postnatal characteristics and timing of onset of symptoms, their overall impact and finally, physical examination with semiological components that include inspection, palpation, percussion and auscultation.

Four patients with hepatitis A presenting with fulminant hepatitis and acute renal failure and who underwent liver transplantation / 대한간학회지

Hepatitis A is generally known as a mild, self-limiting disease of the liver, but in rare instances it can progress to fulminant hepatitis, which may require liver transplantation for recovery. Such cases are known to be related to old age and underlying liver disease. We report four cases of hepatitis A in which patients presented with fulminant hepatitis and acute renal failure and underwent liver transplantation. The following common features were observed in our cases: (1) occurrence in relatively old age (> or =39 years old), (2) association with acute renal failure, (3) presence of hepatomegaly, and (4) microscopic features of submassive hepatic necrosis.

Granulomatous, hepatolithiasis and hepatomegaly caused by Capillaria hepatica infection: first case report of Thailand.

This is the first case report in Thailand of a Capillaria hepatica infection causing a granulomatous hepatic lesion, bile duct dilatation, hepatolithiasis and hepatomegaly. The patient's chief complaint was abdominal pain with fever and chills. Imaging of the liver revealed a 3-cm mass in the postero-inferior sub-segment of the right lobe of the liver with bile duct dilatation. Right hepatectomy and cholecystectomy were performed. Gross pathology of the right hepatectomy revealed focal intrahepatic duct dilatation with prominent periductal fibrosis. The histopathological section revealed chronic inflammation and some granuloma formation surrounding the bile ducts, generalized portal infiltration, prominence of eosinophils and hepatolithiasis. Histopathotogical section revealed oblique sections of C. hepatica egg (size 35.4+/-6.38 microm in width) and brown amorphous pigment.

Hemangiomatosis múltiple con afectación visceral, a propósito de un caso / Multiple hemangiomatosis with visceral involvement, a case report

Ese relata el caso de un paciente de 3 meses de edad con hemangiomas cutáneos y viscerales, hepatomegalia, anemia y datos de insuficiencia cardiaca. Una vez realizados los exámenes complementarios se concluye con el diagnóstico de hemangiomatosis múltiple con afectación visceral. Se aprovecha el caso clínico para hacer una actualización del tema.

Evaluation of the role of ultrasonography versus immunologic tests in diagnosis of chronic hepatosplenic sechistosomiasis

This study aims to evaluate the sensitivity of different EL1SA techniques versus ultrasonography as a 'stablished tool in diagnosis of chronic hepatosplenic schisosomiasis and assessment of different degrees of morbidity. Based on clinical, sonographic md laboratory testing 100 patients enrolled in this study were devided into 4 main groups: Group I with simple intestinal schistosomiasis, group II with periportal fibrosis with or without organomegaly, group III with hepatosplenomegaly and portal hypertension and group IV with advanced morbidity and end stage liver disease. All patients were subjected to ultrasonographic evaluation and serological assessment using Two techniques of ELISA conventional versus Dot ELISA with two types of antigens crude adult worm and soluble egg antigens assessing both IgG and IgM antibodies. The positivity of different ELISA techniques decreases with the advance of morbidity being 95% in group [compared to 15% in group 4 for conventional ELISA and 75% in group I compared to 10% in group 4 for the Dot ELISA. Ultrasonographic evaluation proved to have the main role with the advance of the degree of morbidity and there is no relation between different stages of the disease and the degree of antibody positivity. Antibody burden was higher in the earlier stages of the disease compared to stages of advanced morbidity. IgM antibodies are more prevalent in the earlier stages of the disease while IgG antibodies are more consistent in different stages of the disease being also higher in the [earliest stages of the disease. Based on the conditions of antigen preparations used conventional ELISA was more Sensitive compared to dot ELISA [88% versus 80%] for the conventional using adult worm and soluble egg antigen in group 1 and [68% versus 60%] for the dot ELISA using the Be types of antigens. Conventional ELISA is more sensitive than Fdot ELISA with the adult worm antigen more sensitive than isoluble egg antigens. With advance of morbidity of the disease the sensitivity of both tests as well as the antibody burden decreases that may be attributed to decrease of the intensity of infection as well as low immune status of the individual and this spotlight on the role of ultrasonography with the advance of morbidity. Targeting treatment and control programmes for earlier stages of the disease is essential to reduce heavy antigenic burden that might be responsible for intense immune reaction and progression of the disease state to advanced irreversible morbidity.

Rosai-Dorfman syndrome.

Rosai-Dorfman syndrome is characterized by sinus histiocytosis with massive lymphadenopathy. A 25-year-old woman presented with multiple erythematous and yellowish papules on the forehead, cheeks, chin and thigh. She had massive generalized, firm, non-tender and non-matted lymphadenopathy and mild hepatomegaly. Her hemogram was normal. A skin biopsy showed collections of histiocytes with emperipolesis and plenty of plasma cells. A lymph node biopsy showed partial loss of architecture, dilated sinuses filled with histiocytes, which showed lymphocytophagocytosis, anisonucleosis and a clear cytoplasm. She was referred to an oncologist for further management.

Accuracy and reliability of palpation and percussion for detecting hepatomegaly: a rural hospital-based study.

BACKGROUND: Palpation and percussion are standard bedside techniques used to diagnose hepatomegaly. Ultrasonography is a noninvasive and accurate method for measurement of liver size, but many patients in developing countries have limited access to it. We compared the accuracy of palpation and percussion in a rural population in central India, using ultrasonography as a reference standard. METHODS: The study design was a blinded, cross-sectional analysis of a hospital-based case series. Three physicians, blind to clinical data and to each other's results, independently used palpation and percussion to detect hepatomegaly. Diagnostic accuracy was measured by computing sensitivity, specificity, and likelihood ratio values. Inter-physician agreement was assessed using the kappa statistic. RESULTS: Of the 180 study patients, 36 (20%) had enlarged liver on ultrasonography. The likelihood ratios for findings at both palpation (2.2, 3.0, and 2.5 for the three physicians, respectively) and percussion (1.1 for all three physicians) as predictors of true hepatomegaly were low. The kappa values for inter-observer agreement between three physicians for the presence of hepatomegaly at palpation (=0.44-0.53) and percussion (=0.17-0.33) were low, indicating poor reliability of these techniques. CONCLUSION: Clinical assessment of hepatomegaly by palpation and percussion lacks both accuracy and reliability.