Analysis of 9 cases of drug induced hypersensitivity syndrome related hemophagocytic lymphohistiocytosis / 中华儿科杂志

Objective: To analyze the clinical features,treatment and prognosis of drug induced hypersensitivity syndrome related hemophagocytic lymphohistiocytosis (DIHS-HLH). Methods: This was a retrospective case study. Clinical characteristics, laboratory results, treatment and prognosis of 9 patients diagnosed with DIHS-HLH in Beijing Children's hospital between January 2020 and December 2022 were summarized. Kaplan-Meier survival analysis was used to calculate the overall survival rate. Results: Among all 9 cases, there were 6 males and 3 females, with the age ranged from 0.8 to 3.1 years. All patients had fever, rash, hepatomegaly and multiple lymph node enlargement. Other manifestations included splenomegaly (4 cases), pulmonary imaging abnormalities (6 cases), central nervous system symptoms (3 cases), and watery diarrhea (3 cases). Most patients showed high levels of soluble-CD25 (8 cases), hepatic dysfunction (7 cases) and hyperferritinemia (7 cases). Other laboratory abnormalities included hemophagocytosis in bone marrow (5 cases), hypofibrinogenemia (3 cases) and hypertriglyceridemia (2 cases). Ascending levels of interleukin (IL) 5, IL-8 and interferon-γ (IFN-γ) were detected in more than 6 patients. All patients received high dose intravenous immunoglobulin, corticosteroid and ruxolitinib, among which 4 patients were also treated with high dose methylprednisolone, 2 patients with etoposide and 2 patients with cyclosporin A. After following up for 0.2-38.6 months, 7 patients survived, and the 1-year overall survival rate was (78±14)%. Two patients who had no response to high dose immunoglobulin, methylprednisolone 2 mg/(kg·d) and ruxolitinib died. Watery diarrhea, increased levels of IL-5 and IL-8 and decreased IgM were more frequently in patients who did not survive. Conclusions: For children with fever, rash and a suspicious medication history, when complicated with hepatomegaly, impaired liver function and high levels of IL-5 and IL-8, DIHS-HLH should be considered. Once diagnosed with DIHS-HLH, suspicious drugs should be stopped immediately, and high dose intravenous immunoglobulin, corticosteroid and ruxolitinib could be used to control disease.

Manifestaciones cutáneas en pacientes pediátricos infectados por el coronavirus SARS-CoV-2 / Cutaneous manifestations in pediatric patients infected with SARS-CoV-2 coronavirus

Introducción: El nuevo coronavirus SARS-CoV-2 ha causado un brote grave de la enfermedad denominado COVID-19. El virus fue identificado por primera vez en Wuhan, China, en diciembre del 2019. Recientemente se describieron las manifestaciones cutáneas, con similitud clínica entre niños y adultos. Se identificaron cinco patrones cutáneos: erupción máculo-papular, urticaria, lesiones acrales tipo perniciosas, erupción vesicular y livedo reticularis. Objetivo: Caracterizar a pacientes pediátricos con manifestaciones cutáneas en el curso de la COVID-19. Presentacion de casos: Se estudiaron 10 pacientes pediátricos con COVID-19 ingresados en el Hospital Militar de Villa Clara Comandante Manuel Piti Fajardo. Del total de pacientes, 8 presentaron rash eritemato-papuloso y 2 rash urticariforme. Las lesiones urticariformes se presentaron solamente en las féminas y las eritemato-papulosas, de forma similar en ambos sexos con ligero predominio en las adolescentes pacientes femeninas. Ambas lesiones predominaron en el tronco y acompañadas de un prurito ligero o ausente. Solo requirieron tratamiento médico las lesiones urticariformes. Conclusiones: La principal expresión clínica en la población pediátrica atendida en Villa Clara son las manifestaciones cutáneas. Las lesiones eritemato-papulosas son las más frecuentes, tienen. aparición tardía y evolución favorable sin necesidad de tratamiento y sin acompañarse de otra sintomatología(AU)

Introduction: The new coronavirus called SARS-Cov-2 has caused a severe outbreak of the disease called COVID-19. This virus was identified for the first time in Wuhan, China on December,2019. There were recently described its cutaneous manifestations, with clinical similarities among children and adults. There were identified five cutaneous patterns: macular-papular rash, hives, acral lesions pernicious type, vesicular rash and livedo reticularis. Objective: To characterize pediatric patients with cutaneous manifestations during COVID-19 disease. Cases presentation: There were studied 10 pediatric patients with COVID-19 admitted in ´´Comandante Manuel Piti Fajardo´´ Military Hospital of Villa Clara province. From the total of patients, eight presented eritemato-papular rash and two had urticariform rash. Urticariform lesions were present just in the girls, the eritomato-papulosa lesions presented similarly in both sexes with slight predominance in female adolescents. Both types of lesions appeared with predominance in the torso and accompanied of slight or absent pruritus. Just the urticariform lesions required medical treatment. Conclusions: The main clinical presentations in the pediatric population attended in the Military Hospital of Villa Clara province are the cutaneous manifestations. Eritemato-papular lesions are the most frequent; they have late onset and favorable evolution with no need of treatment and with no other symptomatology(AU)

Síndrome de dress asociado a fenobarbital en paciente pediátrico. Reporte de caso / Dress Syndrome in a pediatric patient asocciated to Phenobarbital. Case Report

Introducción: El síndrome de DRESS por sus siglas en inglés (Drug Rash with Eosinophilia and Sistemic Symptoms), re-presenta una enfermedad grave (1) potencialmente letal que incluye reacción cutánea, iebre, anomalías hematológicas e involucro de diversos órganos. Caso Clínico: Masculino de 2 años con antecedente de uso de Fenobarbital, quien ingresó con historia de iebre, adenopatías y rash cutáneo generalizado. En los exámenes de laboratorio se evidencio leucopenia con eosinoilia con elevación de las enzimas hepáticas; se realizaron pruebas de imagen que reportaron hepatomegalia, y nefromegalia bilateral, mostrando evolución satisfactoria inmediata después del retiro de fenobarbital y una terapia esteroidea sistémica. Discusión: Al ser una reacción adversa de baja frecuencia que presenta una mortalidad de hasta un 30% se considera de importancia conocer su comportamiento clínico, así como el diagnóstico y manejo...(AU)

Reacción por drogas con eosinofilia y síntomas sistémicos (síndrome de DRESS): Estudio retrospectivo de nueve casos / Dress syndrome: Report of nine cases

Background: DRESS Syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) is an uncommon disease caused by drugs. It is characterized by a polymorphic disseminated eruption with fever and multiple organ dysfunction. Aim: To report the etiology, characteristics, treatment, prognosis, and follow up of patients with DRESS Syndrome admitted to a clinical hospital. Material and Methods: Review of medical records of patients admitted for drug reactions, selecting those patients complying with clinical criteria for DRESS Syndrome. Drugs used during three months prior to the onset of symptoms were evaluated as possible causes of the disease. Results: Nine patients aged 16 to 68 years (six males) complied with the clinical criteria for the disease. The causative medications were carbamazepine in three patients, phenytoin in three, antituberculous drugs in two and amoxicillin in one. All were treated with systemic steroids with a complete clinical resolution. Conclusions: DRESS syndrome is usually underdiagnosed and has a good response to systemic steroids.

Rash y anemia aplásica inducidos por fenitoína: caso clínico / Phenytoin-induced rash and aplastic anemia: case report

Rash is a common side effect associated with antiepileptic drugs. The rate of a phenytoin rash is 5.9 percent and increases to 25 percent in those with another antiepileptic drug rash. Aplastic anemia is an adquired hematopoietic stem-cell disorder characterized by pancytopenia of the peripheral blood and hypocellular bone marrow. The use of phenytoin is associated with a 3.5 fold increased risk of aplastic anemia. We report a case of a 70-year-old woman who developed two severe adverse reactions simultaneously with phenytoin: a maculopapular pruritic rash with involvement of mucous and an aplastic anemia. Both conditions normalized after phenytoin withdrawal.

El rash es un efecto secundario común asociado al uso de fármacos antiepilépticos. La frecuencia de rash con fenitoína se ha estimado en un 5,9 por ciento y asciende a un 25 por ciento en pacientes que han presentado rash con otro fármaco antiepiléptico. La anemia aplásica es una anomalía adquirida de las células madre hematopoyéticas caracterizada por pancitopenia de la sangre periférica y médula ósea hipocelular. Los pacientes tratados con fenitoína presentan un riesgo 3,5 veces mayor de desarrollar anemia aplásica. Presentamos el caso de una mujer de 70 años que desarrolló dos reacciones adversas severas y simultáneas a la fenitoína: un exantema maculopapular pruriginoso con compromiso de mucosas y una anemia aplásica. Ambas condiciones se resolvieron completamente con la suspensión del fármaco.

Two cases of toxic hepatitis caused by arrowroot juice / 대한간학회지

Herbal remedies and health foods are widely used, and their side effects have been reported. We describe two cases of symptomatic toxic hepatitis that developed in middle-aged women after ingesting arrowroot juice. The clinical manifestations were nausea, vomiting, and jaundice. The diagnosis of toxic hepatitis was made using the Roussel Uclaf Causality Assessment Method score on the basis of the patient's history and laboratory data. After supportive care, the patients showed rapid improvements of clinical symptoms, laboratory findings, and liver stiffness. Clinicians should be aware that the consumption of arrowroot juice can cause toxic hepatitis.

Síndrome de Ramsay Hunt: relato de caso / Ramsay Hunt Syndrome: case report

A Síndrome de Ramsay Hunt é definida por paralisia facial periférica acompanhada de um rash eritematosoe vesicular no pavilhão auricular ou na boca, causado por herpes zoster. Comparado com a paralisia de Bell,os pacientes com Síndrome de Ramsay Hunt geralmente apresentam um quadro de paralisia mais grave e dificilmentese recuperam totalmente. Alguns estudos sugerem que o tratamento com aciclovir e predinisona possa melhorar o prognóstico, entretanto estudos randomizados ainda são necessários. Os autores descrevem um caso desta síndrome acompanhado de ceratopatia de exposição.

Ramsay Hunt Syndrome consists in facial peripheric paralysis associated with auricular or mouth rash andvesicles, caused by herpes zoster. Compared to Bell’s paralysis, the patients with Ramsay Hunt Syndrome usually present worse paralisy and hardly have a total recovery. Some studies suggest that acyclovir and prednisone treatment may improve the prognosis, but random control studies are still necessary. The authors describeone case of this syndrome with exposure keratopathy.

Actualización de exantemas / Exanthem actualization

Se efectua una puesta al día de los exantemas clásicos, incluyendo un estudio sobre los agentes etiológicos de la 5ª y 6ª enfermedad y sus efectos indeseables en fetos, adultos y progenie eritrocítica; una revisión de la enfermedad de Kawasaki y de los virus asociados al díndrome de Gianotti-crosti y finalmente, la presentacioón de nuevos exantemas de etiología aún no confirmada,como el exantema simétrico periflexural y el síndrome pápulo purpurico de" Gloves and socks"