Complicaciones ORL severas por mononucleosis infecciosa en adolescentes sanos: 2 casos clínicos y revisión de la literatura / ENT severe complications of infectious mononucleosis in healthy adolescents: report of two cases and literature review

Resumen La mononucleosis infecciosa (MI) es un cuadro clínico generalmente benigno y autolimitado en la infancia y adolescencia debido a la primoinfección del virus de Epstein-Barr caracterizado por la triada de faringitis, fiebre y adenopatías. El riesgo de complicaciones aumenta con la edad y la inmunosupresión, siendo las complicaciones letales más frecuentes las asociadas a rotura esplénica, alteraciones neurológicas y obstrucción de la vía aérea por el aumento del tamaño amigdalar. Los abscesos cervicales asociados a MI son poco frecuentes, siendo mayoritariamente periamigdalinos e intraamigdalares. Presentamos dos casos quirúrgicos de abscesos cervicales profundos de gran tamaño con afectación retrofaríngea y parafaríngea en adolescentes sanos de corta edad (14 y 15 años), sin ningún tipo de inmunosupresión o factores de riesgo, uno de ellos asociado además, a una relevante hemorragia amigdalar espontanea, condición no descrita previamente en la literatura en relación a MI en un paciente tan joven.

Abstract Infectious mononucleosis (MI) is a generally benign and self-limited condition in childhood and adolescence due to the primary EBV infection characterized by the triad of pharyngitis, fever, and lymphadenopathies. The risk of complications increases with age and immunosuppression. The most frequent fatal complications are those associated with splenic rupture, neurological alterations, and airway obstruction due to increased tonsillar size. Cervical abscesses associated with MI are rare, being mostly peritonsillar and intra-tonsil. We present two surgical cases of big deep cervical abscesses with retropharyngeal and parapharyngeal involvement in healthy very young adolescents (14 and 15 years old), without any type of immunosuppression or risk factors, one of them associated with a clinically relevant spontaneous tonsillar bleeding, which had not been described in the literature associated with MI in such young patient.

Rotura esplénica espontánea secundaria a mononucleosis infecciosa / Spontaneous splenic rupture secondary to infectious mononucleosis

Resumen La rotura esplénica es una complicación rara pero potencialmente fatal de la mononucleosis infecciosa. Presentamos el caso de una mujer de 18 años que consultó por dolor abdominal de siete días de evolución, asociado a fiebre y pérdida de conciencia brusca y transitoria. En el hemograma presentaba una anemia y linfocitosis. Se realizó una tomografía computada de abdomen y pelvis que mostró un extenso hemoperitoneo, con el bazo rodeado por un hematoma, y numerosas adenopatías cervicales, mesentéricas e inguinales. Se efectuó una laparoscopía que demostró abundante hemoperitoneo con coágulos a lo largo de la gotera parietocólica izquierda. El bazo estaba completamente decapsulado y rodeado por una colección hemática con sangrado en napa. Se realizó una esplenectomía total sin complicaciones. El estudio histopatológico esplénico mostró una atenuación de la pulpa blanca y expansión de la pulpa roja con áreas de hemorragia y necrosis. La IgM anti-cápside para virus de Epstein Barr fue positiva. La paciente evolucionó de manera favorable.

Abstract Splenic rupture is a rare but potentially fatal complication of infectious mononucleosis. We report the case of an 18-year-old woman, who presented a 7-day history of abdominal pain, sudden temporary loss of consciousness and fever. Admission blood tests showed anemia, and lymphocytosis. Computed tomography of the abdomen and pelvis demonstrated extensive hemoperitoneum and numerous cervical, mesenteric and inguinal enlarged lymph nodes. Laparoscopy was performed and abundant hemoperitoneum with blood clots along the left parietocolic gutter were observed. The spleen was completely decapsulated and surrounded by a hematoma and the subcapsular tissue was bleeding. Total splenectomy was performed without complications. Splenic histology demonstrated white pulp attenuation and expansion of the red pulp with focal hemorrhage and necrosis. IgM anti-viral capsid antigen of Epstein Barr virus was positive. The patient had a satisfactory recovery.

Acute hepatitis due to infectious mononucleosis in a 21-year-old-man / Hepatitis aguda por mononucleosis infecciosa en un varón de 21 años

A 21-year-old mole was admitted because of fever, fatigue, headache, pharyngitis, abdominal pain, loss of appetite, vomiting and dark urine for three days. The patient denied recent use of medicines or any other drug. His physical examination disclosed jaundice, hepato-splenomegaly, whitish-yellow covered tonsils, bilateral anterior and posterior cervical lymph node enlargement associated with edema on the face and neck. Routine blood tests detected abnormalities in serum bilirubins and liver enzymes (total bilirubin: 14.5 mg/dl, direct-reacting bilirubin: 12.9 mg/dl, AST: 697 U/l, ALT: 619 U/l, alkaline phosphatases: 260 U/l, and GGT: 413 U/l). Serological tests showed negative results for viral hepatitis, cytomegalovirus, HIV-1 and HIV-2, and toxoplasmosis markers, while serology for recent infection by EBV was positive (IgM: 70 and 29 U/ml; EBV IgG: 25 and 156 U/ml). Although infrequently, EBV infection can cause acute hepatitis with accentuated cholestatic jaundice (5% of cases), which may constitute an additional diagnostic challenge for primary care physicians. The patient improved with supportive management and was discharged after 12 days. This case study might contribute to increase the suspicion index about acute hepatitis related to EBV.

Un paciente varón de 21 años, fue hospitalizado por fiebre, astenia, cefalea, faringitis, dolor abdominal, pérdida del apetito, vómitos y orina oscura desde tres días antes. El paciente negó uso reciente de medicamentos y cualquier otra droga. Su examen físico reveló ictericia, hepato-esplenomegalia, amigdalitis, adenopatías cervicales anteriores y posteriores, asociadas con edema facial y cervical. Los exámenes de laboratorio mostraron elevación sérica de bilirrubina y enzimas hepáticas (bilirrubina total: 14,5 mg/dl, bilirrubina directa: 12,9 mg/dl, AST: 697 U/l, ALT: 619 U/l, fosfatasas alcalinas: 260 U/l, y gama-GT: 413 U/l). Los tests serológicos resultaron negativos para hepatitis viral A, B o C, citomegalovirus, VIH-1 y VIH-2, y toxoplasmosis; pero la serología de infección reciente por VEB fue positiva (IgM: 70 y 29 U/ml; IgG: 25 y 156 U/ml). Aunque raramente, las infecciones por VEB pueden causar una hepatitis aguda con acentuada ictericia colestásica (5% de los casos), que suele constituir un desafío diagnóstico adicional para los médicos en atención primaria. El paciente mejoró durante un tratamiento de apoyo y recibió alta hospitalaria después de 12 días. Este caso estimula a aumentar el índice de sospecha de hepatitis aguda relacionada con VEB.

Falla hepática fulminante por virus Epstein Barr: rol de los corticoides: caso clínico / Epstein Barr virus and acute liver failure: role of steroids: clinical case

La falla hepática fulminante (FHF) debida a Virus Epstein Barr (VEB) es poco frecuente en inmunocompetentes. La utilidad de los esteroides en este cuadro no ha sido definida y permanece muy controversial. Objetivo: Reportar el caso de una paciente con mononucleosis infecciosa por VEB que presenta FHF y es tratada con corticoides. Caso clínico: Escolar con cuadro de 2 sem de síntomas respiratorios altos, fiebre, adenopatías, con ictericia y orina oscura. Bilirrubina total: 9; B. Directa: 6,3; Fosfatasas Alcalinas: 523; GOT: 7.527; GPT: 6.537; Protrombina (PT): 17 por ciento INR: 4,7; Amonio 510 y glicemia 33. Ecografía abdominal hígado normal y esplenomegalia. Monotest Positivo. Se transfirió a centro de trasplante hepático (TH). Laboratorio de ingreso PT 21 por ciento; bilirrubina en 9,8; GOT 2717; GPT 3716 y amonio 177. EEG con enlentecimiento difuso compatible con encefalopatía grado 1. IgM VEB positiva, descartándose otras etiologías. Se activó para TH por FHF y mientras se administró Metilprednisolona por 5 días. Evolucionó con normalización de las pruebas hepáticas y mejoría clínica. Conclusión: En este caso el uso de esteroides se asoció a una rápida y favorable respuesta tanto clínica como de laboratorio sin presentar efectos secundarios negativos. Al igual que en otras presentaciones de infección grave por VEB, debiera considerarse el uso de esteroides en FHF por VEB.

Acute liver failure (ALF) due to Epstein Barr Virus (EBV) is rare in immunocompetent patients. The role of steroids in this case is not well defined and remains controversial. Case report: 7 years old female presenting with unspecific respiratory symptoms for 2 weeks, fever, lymphadenopathy, jaundice and dark brown urine. Total bilirubin: 9 and direct: 6.3, alkaline phosphatases: 523; AST: 7.527, ALT: 6.537; Prothrombin (PT): 17 percent, INR: 4.7; ammonium 510 and glucose 33. Abdominal ultrasound: normal liver and splenomegaly. Monotest Positive. She was transferred to a liver transplant centre (LT). Lab results at admission: PT 21 percent, bilirubin 9.8, AST 2717, ALT 3.716 and ammonium 177. EEG with diffuse and slowing conductivity consistent with encephalopathy. Positive IgM EBV, other aetiologies were ruled out. She was activated for LT due to ALF and while in waiting list methylprednisolone was administered for 5 days. She evolved with normalization of liver tests and clinical improvement. Conclusion: In this case the use of steroids was associated with a rapid and favourable clinical and laboratory response without negative side effects. As in other presentations of serious infection by EBV, should consider the use of steroids in ALF due to EBV.

O vírus Epstein-Barr e a mononucleose infecciosa / Epstein-Barr virus and infectious mononucleosis

JUSTIFICATIVA E OBJETIVOS: A mononucleose infecciosa (MI) é uma doença viral, contagiosa, causada pelo vírus Epstein-Barr (EBV). Apresenta distribuição universal e pode ocorrer em qualquer faixa etária. O presente estudo teve como objetivo descrever a interação entre o EBV e o Homo sapiens sapiens, no que diz respeito especialmente à MI. Para isso, realizou busca no Pubmed e no Scielo, bem como em livros-textos. Os artigos selecionados foram lidos e as informações organizadas de forma a contemplar os aspectos patogênicos - enfatizando o papel do EBV na doença -, epidemiológicos e diagnósticos dessa entidade. CONTEÚDO: A infecção do epitélio da orofaringe permite a replicação do vírus, o qual passa a ser eliminado na saliva, o que explica a transmissão pelo contato com tal fluido biológico. O período de incubação é de quatro a seis semanas. Manifesta-se do ponto de vista clínico, usualmente, com febre, faringite e linfadenomegalia generalizada de caráter agudo ou subagudo. As alterações laboratoriais incluem leucocitose, reações leucemoides, trombocitopenia e anemia hemolítica autoimune, além de aminotransferases elevadas e bilirrubina aumentada, destacando-se que a linfocitose atípica é habitualmente observada. O diagnóstico diferencial da MI abrange as demais causas de síndrome de mononucleose. O tratamento é sintomático, não havendo fármaco antiviral específico. Analgésicos habituais podem ser usados para a terapêutica da dor. CONCLUSÃO: Díspares grupos têm trabalhando no desenvolvimento de vacinas para o EBV, porém, a possibilidade de tumorigênese pelo vírus tem trazido inúmeras dificuldades para a confecção de imunoprofilaxia efetiva.

BACKGROUND AND OBJECTIVES: Infectious mononucleosis (IM) is a widespread viral disease caused by Epstein-Barr virus (EBV). It occurs worldwide and can reach any age group. This article aims at describing the interaction between EBV and Homo sapiens sapiens, regarding especially IM. Thus, a research on PubMed and Scielo was performed, as well as on books. The selected articles were read and the information was organized inorder to contain the pathological - emphasizing the role of EBV on the disease -, epidemiological aspects and its diagnosis. CONTENTS: The infection of the epithelium of the oropharynx leads to the replication of the virus, which is eliminated via saliva, and accounts for the transmission through contact with this biological fluid. The incubation period lasts from four to six weeks. Clinically, the classic symptoms are usually fever, faryngitis and generalized lymphadenopathy, which can be acute or subacute. The laboratorial changes include leukocytosis, leukemoid reactions, thrombocytopenia and severe autoimmune hemolytic anemia, besides high levels of transaminases and bilirubin; atypical lymphocytosis is usually observed. The differential diagnosis of IM includes the other causes of mononucleosis syndrome. Treatment is symptomatic and there is not a specific antiviral medicine. Common analgesics can be used to treat pain. CONCLUSION: Diverse groups have worked on the development of a vaccine for EBV; however, the possibility of tumorigenesis by the virus has brought up several difficulties to the production of an effective immunoprophylaxis.

Dacrioadenite bilateral causada pelo vírus Epstein-Barr: relato de caso / Epstein-Barr virus bilateral dacryoadenitis: case report

Os autores apresentam um caso raro de dacrioadenite aguda bilateral associada à mononucleose. Paciente do sexo feminino, 27 anos, tinha aumento agudo da glândula lacrimal, bilateral, com sinais flogísticos. As alterações tomográficas e laboratoriais confirmaram o diagnóstico e o tratamento com esteróide sistêmico foi introduzido, com resolução completa dos sinais e sintomas. COMENTÁRIOS: A dacrioadenite é uma apresentação incomum da mononucleose infecciosa e pode minimizar outras manifestações desta doença. O diagnóstico de mononucleose deve ser considerado em casos de dacrioadenite aguda bilateral. Os esteróides sistêmicos contribuem para a resolução do quadro inflamatório.

The authors present a rare case of acute bilateral dacryoadenitis in association with infectious mononucleosis. A 27 year-old woman had acute bilateral lacrimal gland enlargement with inflammatory signs. The CT scan findings and laboratorial investigations confirmed the diagnosis of infectious mononucleosis. The systemic treatment with intravenous steroids was initiated leading to complete remission of the signs and symptoms. COMMENTS: Dacryoadenitis is an uncommon manifestation of infectious mononucleosis and may minimize other signs of the disease. Mononucleosis has to be considered in acute dacryoadenitis cases. Systemic steroids contribute to the fast regression of inflammatory condition.

Infectious Mononucleosis Hepatitis in Young Adults: Two Case Reports

Infectious mononucleosis due to Epstein-Barr virus (EBV) infection sometimes causes acute hepatitis, which is usually self-limiting with mildly elevated transaminases, but rarely with jaundice. Primary EBV infection in children is usually asymptomatic, but in a small number of healthy individuals, typically young adults, EBV infection results in a clinical syndrome of infectious mononucleosis with hepatitis, with typical symptoms of fever, pharyngitis, lymphadenopathy, and hepatosplenomegaly. EBV is rather uncommonly confirmed as an etiologic agent of acute hepatitis in adults. Here, we report two cases: the first case with acute hepatitis secondary to infectious mononucleosis and a second case, with acute hepatitis secondary to infectious mononucleosis concomitantly infected with hepatitis A. Both cases involved young adults presenting with fever, pharyngitis, lymphadenopathy, hepatosplenomegaly, and atypical lymphocytosis confirmed by serologic tests, liver biopsy and electron microscopic study.

Proteinuria in a Boy with Infectious Mononucleosis, C1q Nephropathy, and Dent's Disease

C1q nephropathy is a proliferative glomerulopathy with extensive mesangial deposition of C1q. A three-year old boy presented with a nephrotic-range proteinuria during an acute phase of Epstein-Barr virus (EBV) infection, and he had a family history of Dent's disease. The renal biopsy findings were compatible with C1q nephropathy. However, EBV in situ hybridization was negative. The CLCN5 gene analysis revealed an R637X hemizygous mutation, which was the same as that detected in his maternal cousin, the proband of the family. The causal relationship between EBV infection and C1q nephropathy remains to be determined. Moreover, the effects of underlying Dent's disease in the process of C1q nephropathy has to be considered.

Infectious mononucleosis presenting as upper airway obstruction.

Upper airway obstruction though a common complication of infectious mononucleosis is rarely considered in differential diagnosis of stridor. We report a three-year-old child who had upper airway obstruction due to infectious mononucleosis, managed conservatively with oxygen, intravenous fluids and steroids.

Infectious mononucleosis presenting as acute hepatitis, with marked leukocytosis and renal involvement.

We report a 45-year-old lady with infectious mononucleosis due to Epstein-Barr virus. The unusual features of this case included a negative heterophil antibody test, marked leukocytosis, renal involvement and jaundice.

Síndrome de mononucleosis: enfrentamiento pediátrico / Mononucleosis syndrome: pediatric facing

Al conocer las diferentes etiologías y manifestaciones clínicas del síndrome de monucleosis infecciosa se ha observado una mayor frecuencia en su diagnóstico. Es así que se debe tener presente en lactantes con síntomas de infección respiratoria alta prolongada, y en niños mayores con síndrome febril o amigdalitis acompañada de esplenomegalia. Actualmente es imprescindible la corroboración del diagnóstico con serología específica para el virus de Epstein Barr u otras etiologías

Infectious mononucleosis hepatitis: report of two patients.

Icteric hepatitis and fulminant hepatic failure (FHF) are rare in infectious mononucleosis (IM). We report two patients with icteric IM hepatitis; one died after developing FHF, the other recovered uneventfully. Epstein-Barr virus infection causing hepatitis and FHF should be suspected when tests for other hepatotrophic viral infections are negative.

Obstruçäo de vias aéreas superiores por mononucleose infecciosa em crianças / Airway obstruction in infectious mononucleosis in children

A Mononuclease Infecciosa (MI) é doença viral aguda causada pela infecçäo dos linfócitos B pelo Epstein-Barr vírus, que geralmente acomete crianças e adultos jovens e causa doença linfoproliferativa difusa e transitória. Apesar de conhecida por sua beniginidade, existem apresentaçöes atípicas, muitas vezes corresponendo as complicaçöes reveladoras da MI que podem ter implicaçöes diagnósticas decisivas, sendo a obstruçäo de vias aéreas superiores (VAS) a mais grave dentre elas. Os autores apresentam três casos de crianças com história de obstruçäo de VAS que tiveram evoluçöes diferentes, discutem os vários métodos propostos para o tratamento de tais complicaçöes e alertam para a inespecificdade dos exames laboratoriais na criança. Concluem alertando para a necessidade do reconhecimento dessas apresentaçöes atípicas da MI e da importância do acompanhamento otorrinolaringológico nessas situaçöes