A Better Understanding of Pediatric Autoimmune Pancreatitis: Over 13 Years of Experience.

OBJECTIVES: Pediatric autoimmune pancreatitis (P-AIP) is an uncommon disease whose diagnosis requires strong clinical suspicion. Late diagnosis increases morbidity. We aimed to compare the usefulness of the 2011 International Consensus Diagnostic Criteria (ICDC) for Autoimmune Pancreatitis with the 2018 INSPPIRE (INternational Study Group of Pediatric Pancreatitis: In search for a cuRE) criteria. METHODS: We retrospectively analyzed demographics and clinical, laboratory, radiological, and histological findings at diagnosis and during long-term follow-up in children diagnosed with AIP in 2 tertiary hospitals between 2008 and 2021. RESULTS: We included 11 patients [6 girls; median age at diagnosis, 12.5 (range 2.8-15.7) years]. The most common symptom was abdominal pain. Pancreatic enzymes were elevated in 10 patients, and serum immunoglobulin G4 was elevated in 1. Magnetic resonance imaging showed enlargement of the pancreatic head in 10 patients and general pancreatic enlargement in 1. Pancreatic and papilla tissue were obtained from 9 patients. All patients received corticosteroids (prednisolone), and 4 also received azathioprine. According to the ICDC, all patients were classified as probable or non-otherwise specified AIP. According to INSPPIRE criteria, all patients were classified as AIP. Using the INSPPIRE criteria would have avoided biopsies in 6 patients who responded well to corticosteroids. CONCLUSIONS: The INSPPIRE criteria are useful. Using the ICDC in pediatric patients can delay diagnosis and result in unnecessary invasive tests.

Infectious complications of non-surgical biliary tract manipulation in paediatric patients. Role of antibiotic prophylaxis.

BACKGROUND: Infections related to non-surgical manipulation of the biliary tract (NSMBT) are common events despite periprocedural antibiotic prophylaxis (PAP). Since June 2017, our local protocol has indicated a 24-h regimen of intravenous piperacillin-tazobactam for this purpose. OBJECTIVE: We aimed to describe the incidence and characteristics of NSMBT-related paediatric infections, define risk factors for their development, and analyse adherence to our PAP protocol. MATERIALS AND METHODS: Epidemiological, clinical, and microbiological data were collected in consecutive NSMBT procedures performed in paediatric patients (<18 years) in our centre (2010-2019). RESULTS: 113 procedures in 37 patients, median age 4 years (IQR 1-8), were included. Main underlying diseases were biliary atresia (32%) and cancer (14%). Sixty-eight percent had undergone liver transplant and 70% hepaticojejunostomy. In 44 procedures (39%), the intervention was performed during the course of infection and previously prescribed antibiotic treatment was maintained. In the other 69, PAP was specifically indicated for NSMBT; antibiotic adequacy increased from 35% to 100% after June 2017. In total, 32 NSMBT-related infections (28%) occurred, mainly in the first 24h post-procedure (72%); no deaths happened. Causative pathogens were Gram-negative rods (64%), Gram-positive cocci (28%), and Candida spp. (8%). Main related risk factors were hepaticojejunostomy, biliary obstruction, and liver transplant. CONCLUSIONS: NSMBT in children entails a significant infection risk, even under antibiotic prophylaxis, being hepaticojejunostomy the main risk factor. Infectious complications mainly occurred immediately after the procedure. After establishing a PAP protocol, 100% of interventions received appropriate prophylaxis, decreasing antibiotic exposure time and potentially, the length of hospital stay.

Infectious complications of non-surgical biliary tract manipulation in paediatric patients. Role of antibiotic prophylaxis / Complicaciones infecciosas de la manipulación no quirúrgica de las vías biliares en pacientes pediátricos. Función de la profilaxis antibiótica

Background: Infections related to non-surgical manipulation of the biliary tract (NSMBT) are common events despite periprocedural antibiotic prophylaxis (PAP). Since June 2017, our local protocol has indicated a 24-h regimen of intravenous piperacillin–tazobactam for this purpose. Objective: We aimed to describe the incidence and characteristics of NSMBT-related paediatric infections, define risk factors for their development, and analyse adherence to our PAP protocol. Materials and methods: Epidemiological, clinical, and microbiological data were collected in consecutive NSMBT procedures performed in paediatric patients (<18 years) in our centre (2010–2019). Results: 113 procedures in 37 patients, median age 4 years (IQR 1–8), were included. Main underlying diseases were biliary atresia (32%) and cancer (14%). Sixty-eight percent had undergone liver transplant and 70% hepaticojejunostomy. In 44 procedures (39%), the intervention was performed during the course of infection and previously prescribed antibiotic treatment was maintained. In the other 69, PAP was specifically indicated for NSMBT; antibiotic adequacy increased from 35% to 100% after June 2017. In total, 32 NSMBT-related infections (28%) occurred, mainly in the first 24h post-procedure (72%); no deaths happened. Causative pathogens were Gram-negative rods (64%), Gram-positive cocci (28%), and Candida spp. (8%). Main related risk factors were hepaticojejunostomy, biliary obstruction, and liver transplant. Conclusions: NSMBT in children entails a significant infection risk, even under antibiotic prophylaxis, being hepaticojejunostomy the main risk factor. Infectious complications mainly occurred immediately after the procedure. After establishing a PAP protocol, 100% of interventions received appropriate prophylaxis, decreasing antibiotic exposure time and potentially, the length of hospital stay.(AU)

Antecedentes: Las infecciones relacionadas con la manipulación no quirúrgica de las vías biliares (MNQVB) son acontecimientos frecuentes, a pesar de la profilaxis antibiótica periprocedimiento (PAP). Desde junio de 2017, nuestro protocolo local indica una pauta de 24 h de piperacilina/tazobactam por vía intravenosa para este fin. Objetivo: El objetivo era describir la incidencia y las características de las infecciones pediátricas relacionadas con la MNQVB, definir los factores de riesgo para su desarrollo y analizar el cumplimiento de nuestro protocolo de PAP. Materiales y métodos: Se recogieron datos epidemiológicos, clínicos y microbiológicos en procedimientos consecutivos de MNQVB realizados en pacientes pediátricos (< 18 años) en nuestro centro (2010-2019). Resultados: Se incluyeron 113 procedimientos en 37 pacientes, con una mediana de edad de 4 años (RIC 1-8). Las principales enfermedades subyacentes fueron atresia biliar (32%) y cáncer (14%). El 68% se había sometido a un trasplante de hígado y el 70% a una hepaticoyeyunostomía. En 44 procedimientos (39%), la intervención se realizó durante el transcurso de la infección y se mantuvo el tratamiento antibiótico recetado previamente. En los otros 69, la PAP estaba indicada específicamente para la MNQVB; la eficacia de los antibióticos aumentó del 35 al 100% después de junio de 2017. En total, se produjeron 32 infecciones relacionadas con la MNQVB (28%), principalmente en las primeras 24 h posteriores al procedimiento (72%); no se produjo ninguna muerte. Los patógenos causantes fueron bacilos gramnegativos (64%), cocos grampositivos (28%) y Candida spp. (8%). Los principales factores de riesgo relacionados fueron la hepaticoyeyunostomía, la obstrucción biliar y el trasplante de hígado.(AU)

Hepatitis aguda per virus de l’hepatitis E / Hepatitis aguda por virus de la hepatitis E / Acute hepatitis by Hepatitis E virus

Introducció. L’hepatitis aguda és un procés necroinflamatoridel fetge causat per una noxa puntual. Analíticament esmanifesta per un augment de les transaminases i la presentació clínica pot ser variable (de formes subclíniques a insuficiència hepàtica aguda). L’estudi d’hepatitis inclouproves per valorar el grau d’alteració fisiològica i per identificar-ne l’etiologia. La causa més freqüent són les infeccions víriques.Cas clínic. Pacient de 8 anys amb família originària delPakistan, d’on retorna poc abans d’iniciar la clínica. Presenta abdominàlgia, diarrea, febre, colúria i acòlia. En l’exploració física destaca icterícia mucocutània, esplenomegàlia i hepatomegàlia lleus. L’analítica presenta augmentde transaminases i bilirubina directa. S’ingressa per a estudi i tractament simptomàtic. En les serologies s’obté unresultat positiu a virus d’hepatitis E.Comentaris. El virus de l’hepatitis E (VHE) és un virus RNAque pertany a la família Herpesviridae. La caracteritzaciómolecular ha permès identificar-ne quatre genotips: HVE1i HVE2 infecten únicament humans i són predominants enpaïsos en vies de desenvolupament. HVE3 i HVE4 infectentambé altres mamífers. Són responsables dels casos esporàdics a escala mundial. La majoria d’infeccions sónasimptomàtiques en pacients immunocompetents, però demés risc en pacients immunodeprimits pel risc més alt de cronificació. (AU)

Introducción. La hepatitis aguda es un proceso necroinflamatoriodel hígado causado por una noxa puntual. Analíticamente se manifiesta por un aumento de las transaminasas y la presentaciónclínica puede ser variable (de formas subclínicas a insuficienciahepática aguda). El estudio de hepatitis incluye pruebas para valorar el grado de alteración fisiológica y para identificar su etiología. La causa más frecuente son las infecciones víricas.Caso clínico. Paciente de 8 años con familia originaria de Pakistán,de donde vuelve poco antes de iniciar la clínica. Presenta abdominalgia, diarrea, fiebre, coluria y acolia. En la exploración físicadestaca ictericia cutánea-mucosa, esplenomegalia y hepatomegalia leves. En la analítica presenta aumento de transaminasas y bilirrubina directa. Se ingresa para estudio y tratamiento sintomático. En las serologías se obtiene un resultado positivo a virus dela hepatitis E.Comentarios. El virus de la hepatitis E (VHE) es un virus RNA quepertenece a la familia Herpesviridae. La caracterización molecularha permitido identificar cuatro genotipos: HVE1 y HVE2 infectanúnicamente a humanos y son predominantes en países en vías dedesarrollo. HVE3 y HVE4 infectan también a otros mamíferos. Sonresponsables de los casos esporádicos a nivel mundial. La mayoríade infecciones son asintomáticas en pacientes inmunocompetentes, pero de mayor riesgo en pacientes inmunodeprimidos por elmayor riesgo de cronificación. (AU)

Introduction. Acute hepatitis is a necroinflammatory process of theliver caused by isolated noxa. Its laboratory findings are characterized by an increase in liver transaminases, and the clinical presentation may be variable (from subclinical forms to acute liverfailure). The study of hepatitis includes tests to assess the degreeof physiological alteration and to identify its etiology. The mostcommon cause is viral infections.Clinical case. An 8-year-old patient who recently returned from atrip to Pakistan presented with abdominal pain, diarrhea, fever,choluria and acholia. Physical examination revealed mild mucocutaneous jaundice, splenomegaly, and hepatomegaly. Laboratoryevaluation showed an increase in liver transaminases and directbilirubin. He was admitted for diagnostic evaluation and symptomatic treatment. Serology for hepatitis E virus resulted positive.Comments. Hepatitis E virus (HEV) is an RNA virus of the Herpesviridae family. Molecular characterization has identified four genotypes, HVE 1-4. HVE1 and HVE2 infect only humans and are predominant in developing countries, whereas HVE3 and HVE4 alsoinfect other mammals. HVE is responsible for sporadic casesworldwide. Most infections are asymptomatic in immunocompetent patients but risk is higher in immunocompromised patientsdue to increased risk of chronicity. (AU)

Diagnostic action against hypertransaminasemia in paediatrics: Consensus document of Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátrica (SEGHNP), Asociación Española de Pediatría de Atención Primaria (AEPap) and Sociedad Española de Pediatría de Atención Primaria (SEPEAP).

Hypertransaminasemia is a frequent finding in pediatrics, which could reflect potentially treatable serious disease. The aim of this document is to establish, by reviewing the available evidence, a consensus for an adequate management of hypertransaminasemia, from its detection until the study is complete. To this end, a working group was formed with the participation of members of the Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP), the Spanish Association of Primary Care Pediatrics (AEPap) and the Spanish Society of Primary Care Pediatrics (SEPEAP). Twenty-one recommendations are established with a marked practical component that will be useful in hospital clinical practice and primary care.

Actuación diagnóstica ante hipertransaminasemia en pediatría: documento de consenso de Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátrica (SEGHNP), Asociación Española de Pediatría de Atención Primaria (AEPap) y Sociedad Española de Pediatría de Atención Primaria (SEPEAP) / Diagnostic action against hypertransaminasemia in paediatrics: Consensus document of Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátrica (SEGHNP), Asociación Española de Pediatría de Atención Primaria (AEPap) and Sociedad Española de Pediatría de Atención Primaria (SEPEAP)

La hipertransaminasemia es un hallazgo frecuente en pediatría, puede ser banal o reflejar enfermedad grave potencialmente tratable. El objetivo de este documento es establecer, mediante la revisión de la evidencia disponible, un consenso para un adecuado enfoque práctico desde la detección de la hipertransaminasemia hasta completar su estudio en la edad pediátrica. Para ello, se constituyó un grupo de trabajo con participación de miembros de la Sociedad de Gastroenterología, Hepatología y Nutrición Pediátrica (SEGHNP), Asociación Española de Pediatría de Atención Primaria (AEPap) y Sociedad Española de Pediatría de Atención Primaria (SEPEAP). Se establecieron 21 recomendaciones con el objetivo de que sirvan de utilidad en la práctica clínica habitual tanto en atención primaria como hospitalaria. (AU)

Hypertransaminasemia is a frequent finding in pediatrics, which could reflect potentially treatable serious disease. The aim of this document is to establish, by reviewing the available evidence, a consensus for an adequate management of hypertransaminasemia, from its detection until the study is complete. To this end, a working group was formed with the participation of members of the Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP), the Spanish Association of Primary Care Pediatrics (AEPap) and the Spanish Society of Primary Care Pediatrics (SEPEAP). Twenty-one recommendations are established with a marked practical component that will be useful in hospital clinical practice and primary care. (AU)

Epstein-Barr virus-associated risk factors for post-transplant lymphoproliferative disease in pediatric liver transplant recipients.

BACKGROUND: Post-transplant lymphoproliferative disorder (PTLD) are the most common de novo malignancies after liver transplantation (LT) in children. The aim of our study was to assess the role of pre-LT EBV status and post-LT EBV viral load as risk factors for developing PTLD in a cohort of pediatric LT recipients. METHODS: Data of all children who underwent LT between January 2002 and December 2019 were collected. Two cohorts were built EBV pre-LT primary infected cohort and EBV post-LT primary infected cohort. Moreover, using the maximal EBV viral load, a ROC curve was constructed to find a cutoff point for the diagnosis of PTLD. RESULTS: Among the 251 patients included in the study, fifteen PTLD episodes in 14 LT recipients were detected (2 plasmacytic hyperplasia, 10 polymorphic PTLD, 2 monomorphic PTLD, and 1 Classical-Hodgkin's lymphoma). Patients of the EBV post-LT primary infected cohort were 17.1 times more likely to develop a PTLD than patients of the EBV pre-LT primary infected cohort (2.2-133.5). The EBV viral load value to predict PTLD was set at 211 000 UI/mL (93.3% sensitivity and 77.1% specificity; AUC 93.8%; IC 0.89-0.98). In EBV post-LT primary infected cohort, patients with a viral load above 211 000 were 30 times more likely to develop PTLD than patients with a viral load below this value (OR 29.8; 3.7-241.1; p < 0.001). CONCLUSIONS: The combination of pretransplant EBV serological status with EBV post-transplant viral load could be a powerful tool to stratify the risk of PTLD in pediatric LT patients.

Delayed sequential abdominal wall closure in pediatric liver transplantation to overcome "large for size" scenarios.

BACKGROUND: Primary abdominal wall closure after pediatric liver transplantation (PLT) is neither always possible nor advisable, given the graft-recipient size discrepancy and its potential large-for-size scenario. Our objective was to report the experience accumulated with delayed sequential closure (DSC) guided by Doppler ultrasound control. METHODS: Retrospective analysis of DSC performed from 2013 to March 2020. RESULTS: Twenty-seven DSC (26.5%) were identified out of 102 PLT. Transplant indications and type of grafts were similar among both groups. In patients with DSC, mean weight and GRWR were 9.4 ± 5.5 kg (3.1-26 kg) and 4.7 ± 2.4 (1.9-9.7), significantly lower and higher than the primary closure cohort, respectively. The median time to achieve definitive closure was 6 days (range 3-23 days), and the median number of procedures was 4 (range 2-9). Patients with DSC had longer overall PICU (22.5 ± 16.9 vs. 9.1 ± 9.7 days, p < .05) and hospital stay (33.4 ± 19.1 vs 23, 9 ± 19.8 days (p < .05). These differences are less remarkable if the analysis is performed in a subgroup of patients weighing less than 10 kg. Two patients presented vascular complications (7.4%) within DSC group. No differences were seen when comparing overall, 3-year graft and patient survival (96% and 96% in the DSC group). CONCLUSIONS: DSC is a simple and safe technique to ensure satisfactory clinical outcomes to overcome "large for size" scenarios in PLT. In addition, we were able to avoid using a permanent biological material for closing the abdomen.

Infectious complications of non-surgical biliary tract manipulation in paediatric patients. Role of antibiotic prophylaxis.

BACKGROUND: Infections related to non-surgical manipulation of the biliary tract (NSMBT) are common events despite periprocedural antibiotic prophylaxis (PAP). Since June 2017, our local protocol has indicated a 24-h regimen of intravenous piperacillin-tazobactam for this purpose. OBJECTIVE: We aimed to describe the incidence and characteristics of NSMBT-related paediatric infections, define risk factors for their development, and analyse adherence to our PAP protocol. MATERIALS AND METHODS: Epidemiological, clinical, and microbiological data were collected in consecutive NSMBT procedures performed in paediatric patients (<18 years) in our centre (2010-2019). RESULTS: 113 procedures in 37 patients, median age 4 years (IQR 1-8), were included. Main underlying diseases were biliary atresia (32%) and cancer (14%). Sixty-eight percent had undergone liver transplant and 70% hepaticojejunostomy. In 44 procedures (39%), the intervention was performed during the course of infection and previously prescribed antibiotic treatment was maintained. In the other 69, PAP was specifically indicated for NSMBT; antibiotic adequacy increased from 35% to 100% after June 2017. In total, 32 NSMBT-related infections (28%) occurred, mainly in the first 24h post-procedure (72%); no deaths happened. Causative pathogens were Gram-negative rods (64%), Gram-positive cocci (28%), and Candida spp. (8%). Main related risk factors were hepaticojejunostomy, biliary obstruction, and liver transplant. CONCLUSIONS: NSMBT in children entails a significant infection risk, even under antibiotic prophylaxis, being hepaticojejunostomy the main risk factor. Infectious complications mainly occurred immediately after the procedure. After establishing a PAP protocol, 100% of interventions received appropriate prophylaxis, decreasing antibiotic exposure time and potentially, the length of hospital stay.

Manifestaciones cutáneas en contexto del brote actual de enfermedad por coronavirus 2019 / Cutaneous manifestations in the current pandemic of coronavirus infection disease (COVID 2019)

No disponible

Transition to Adult Care for Pediatric Liver Transplant Recipients.

In recent years a growing number of pediatric liver transplant recipients are reaching adulthood and are transferred to an adult team. Because pediatric to adult transition has become a common event with many particularities, specific clinical protocols are needed to guide professionals in this process. Transition must be seen as a complex process of high vulnerability for the patient. The incorrect assumption that the transition process is only a bureaucratic transfer of information leads to inappropriate transition procedures that result in young patients not ready to move to adult units with guaranteed success. To ensure this success, a correct coordination and transmission of the information, accompaniment by the health professional during the whole process, and the empowerment of the patient are required. To have a successful transition, a person within the pediatric team must be in charge of the process (named worker).

Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient.

We present an 8 years old girl who was diagnosed at 6 months of age of Progressive Familial Intrahepatic Cholestasis type 2. Although liver transplantation (LT) was classically considered curative for these patients, cholestasis recurrence with normal gamma-glutamyl transpeptidase (GGT), mediated by anti-bile salt export pump (BSEP) antibodies after LT (auto-antibody Induced BSEP Deficiency, AIBD) has been recently reported. Our patient underwent LT at 14 months. During her evolution, patient presented three episodes of acute rejection. Seven years after the LT, the patient presented pruritus with cholestasis and elevation of liver enzymes with persistent normal GGT. Liver biopsy showed intrahepatic cholestasis and giant-cell transformation with very low BSEP activity. Auto-antibodies against BSEP were detected therefore an AIBD was diagnosed. She was treated with Rituximab and immunoadsorption with resolution of the AIBD. As a complication of the treatment she developed a pneumocystis infection successfully treated with corticoids, cotrimoxazol and anidulafungin.

Endoscopic variceal ligation as primary prophylaxis for upper GI bleeding in children.

BACKGROUND AND AIMS: Variceal hemorrhage can be a life-threatening adverse event of chronic liver disease. In contrast to the well-described guidelines for the management of portal hypertension (PH) in adults, there is limited evidence about the optimal prophylactic management of variceal bleeding in children. This study was carried out to assess the efficacy of endoscopic variceal ligation (EVL) as primary prophylaxis to prevent upper GI bleeding in children with PH. METHODS: From January 2014 to April 2018, all pediatric patients with PH disease and medium to large esophageal varices or reddish spots, regardless of the grade of the varix, were prospectively included in the protocol of primary prophylaxis with EVL. A second retrospective group of patients was made after reviewing medical records of 32 pediatric patients with PH that presented esophageal varices in the upper endoscopy and had received propranolol as primary prophylaxis. RESULTS: Twenty-four patients (75%) reached varices eradication in the EVL group, with a median of 2 procedures (range, 1-4) before eradication and a median time to eradication of 3.40 months (range, 1.10-13.33). No EVL-related adverse events were observed. Statistically significant differences were observed in the bleeding rate at 3 years between propranolol and EVL groups (6/32 [21.9%] vs 1/32 [3.2%], P < .02). The hazard ratio for bleeding for patients treated with propranolol compared with those treated with EVL was 2.6 (95% confidence interval, 1.53-3.67). CONCLUSIONS: EVL is a safe and effective treatment to prevent upper GI bleeding in pediatric patients with PH. (Clinical trial registration number: NCT03943784.).

Adolescent home subsaharià de 15 anys amb dolor a fossa lumbar de 24 hores d'evolució / No disponible

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Combinación de ledipasvir/sofosbuvir como tratamiento de la infección crónica por hepatitis C / Ledipasvir/sofosbuvir combination for chronic hepatitis C infection in children and adolescents

Introducción: El objetivo del estudio fue evaluar la seguridad y la eficacia de la combinación de ledipasvir/sofosbuvir en la infección crónica por el genotipo 1 y 4 del virus de la hepatitis C (VHC) en pacientes pediátricos. Métodos: Se incluyó a pacientes de entre 6 y 18 años. La duración y la dosis de los fármacos antivirales se administraron según la edad del paciente, el estadio de fibrosis y los tratamientos previos con interferón pegilado y ribavirina. La variable principal de eficacia fue el porcentaje de pacientes con una respuesta virológica sostenida 12 semanas (RVS12) después del tratamiento. Resultados: Nueve pacientes con una mediana de edad de 14,8 años (8,48-17,91) fueron tratados con combinación de ledipasvir/sofosbuvir. Cinco pacientes habían recibido previamente tratamiento con interferón pegilado + ribavirina. Ocho pacientes tenían algún grado de fibrosis. La mediana de la carga viral previa al tratamiento fue de 6,2 log (5,9-6,8) con negativización del ARN del VHC 6 semanas después de comenzar el tratamiento en el 100% de los pacientes. Todos los pacientes mantuvieron una respuesta viral sostenida a las 12 semanas. Tres pacientes (33,3%) tuvieron algún tipo de efecto adverso (2 dolores de cabeza y un afta oral). La mediana de seguimiento posterior al tratamiento fue de 24 semanas (12-104). Conclusiones: El tratamiento con ledipasvir/sofosbuvir en pacientes pediátricos con infección crónica por VHC de genotipo 1 y 4 es seguro y efectivo con RVS12, similar a lo reportado en adultos

Introduction: Hepatitis C virus infection is world health problem. The aim of this study was to assess the safety and efficacy of ledipasvir/sofosbuvir combination in chronic Hepatitis C Virus (HCV) genotype 1 and 4 infection in paediatric patients. Methods: Eligible patients to be treated with ledipasvir/sofosbuvir were patients from 6 to 18 years old with a chronic HCV genotype 1 or 4 infection. The duration and doses of antiviral drugs were changed depending on patient age, fibrosis stage, and PEGylated interferon+ribavirin experience status. The primary efficacy endpoint was the percentage of patients with a sustained virological response 12 weeks post-treatment. Results: A total of nine patients (7 males) with a median age of 14.8 years (8.48-17.91) were treated with ledipasvir/sofosbuvir combination. Five patients received previous treatment with PEGylated interferon + ribavirin during a median of 8.5 months (3-12 months). Eight patients had some degree of fibrosis (1 patient presented with F1, three patients F2, 2 patients F3, and 2 patients F4). The median pre-treatment viral load was 6.2 Log [5.9-6.8] with the HCV RNA becoming negative six weeks after starting the treatment in 100% of the patients. All patients maintained a sustained viral response at 12 weeks. Three patients (33.3%) had some type of adverse effect (2 headache and one oral thrush). The median post-treatment follow-up was 24 weeks (12-104). Conclusions: Treatment with ledipasvir/sofosbuvir in paediatric patients with chronic HCV infection genotype 1 and 4 is safe and effective with SVR12 and similar to those reported in adults

Síndrome retroviral aguda en un adolescent amb herpes genital / Síndrome retroviral agudo en un adolescente con herpes genital / Acute retroviral syndrome in an adolescent with genital herpes

Introducció: La infecció aguda pel virus de la immunodeficiència humana (VIH) cursa amb clínica inespecífica i transitòria tipus síndrome mononucleòsica. És poc freqüent trobar un cas de VIH en l'edat pediàtrica i, a més, l'adolescent poques vegades expressa de forma espontània una exposició de risc. Per això és important considerar la infecció per VIH, sobretot si es detecten altres infeccions de transmissió sexual. Cas clínic: Adolescent de 13 anys amb discapacitat intel·lectual lleu, que consulta per febre, odinofàgia, astènia, miàlgies, anorèxia, vòmits i diarrea de 5 dies d'evolució. Després d'insistir en l'anamnesi, explica que els darrers dos dies apareix exantema a tronc i úlceres al penis, i que va tenir una única relació homosexual no consentida fa dos mesos. En l'exploració destaca exantema maculoeritematós al tronc, hiperèmia faríngia, adenopaties generalitzades i úlceres doloroses al gland i al prepuci. La reacció en cadena de la polimerasa al frotis de les lesions genitals és positiva per a virus herpes simple 1. El test ràpid d'anticossos pel VIH resulta indeterminat i el test confirmatori per immunocromatografia és negatiu. Presenta càrrega viral del VIH de 1.681.383 còpies/mL, test d'immunoassaig de 4a generació (inclou detecció anticossos VIH i antigen p24) positiu i recompte de limfòcits CD4 de 327 cèl·lules/μL. Rep tractament amb aciclovir i teràpia antiretroviral amb tenofovir, emtricitabina i darunavir/cobicistat, que després de l'alta hospitalària s'administra de forma supervisada al domicili. Comentaris: El pediatre ha d'estar alerta per reconèixer la infecció aguda per VIH i altres malalties de transmissió sexual en l'adolescent. El diagnòstic de la infecció evita la transmissió als altres, i l'inici precoç de la teràpia antiretroviral millora el pronòstic de la malaltia

Introducción: La infección aguda por el virus de la inmunodeficiencia humana (VIH) cursa con clínica inespecífica y transitoria tipo síndrome mononucleósico. Es poco frecuente encontrarse con un caso de VIH en la edad pediátrica y, además, el adolescente pocas veces revela de forma espontánea una exposición de riesgo. Por ello es importante considerar la infección por VIH, sobre todo si se detectan otras infecciones de transmisión sexual. Caso clínico: Adolescente de 13 años con discapacidad intelectual leve, que consulta por fiebre, odinofagia, astenia, mialgias, anorexia, vómitos y diarrea de 5 días de evolución. Tras insistir en la anamnesis, explica que en los últimos dos días aparece exantema en tronco y úlceras en pene, y que tuvo una única relación homosexual no consentida hace dos meses. En la exploración destaca exantema maculoeritematoso en tronco, hiperemia faríngea, adenopatías generalizadas y úlceras dolorosas en glande y prepucio. La reacción en cadena de la polimerasa en frotis de las lesiones genitales es positiva para virus herpes simple 1. El test rápido de anticuerpos VIH resulta indeterminado y el test confirmatorio por inmunocromatografía es negativo. Presenta carga viral del VIH de 1.681.383 copias/ml, test de inmunoensayo de 4ª generación (incluye detección anticuerpos VIH y antígeno p24) positivo y recuento de linfocitos CD4 de 327 células/μl. Recibe tratamiento con aciclovir y terapia antirretroviral con tenofovir, emtricitabina y darunavir/cobicistat, que tras el alta hospitalaria se administra de forma supervisada en domicilio. Comentarios: El pediatra debe estar alerta para reconocer la infección aguda por VIH y otras enfermedades de transmisión sexual en el adolescente. El diagnóstico de la infección evita la transmisión a otros y el inicio precoz de la terapia antirretroviral mejora el pronóstico de la enfermedad

Introduction: Acute human immune deficiency virus (HIV) infection is typically described as a transient and non-specific mononucleosis like syndrome. This acute presentation is rare in pediatrics, and adolescents rarely report a risk exposure. Thus, it is important to consider HIV infection specially if other sexually transmitted diseases are diagnosed. Case report: A 13-year-old boy with mild intellectual disability, presented with a 5-day history of fever, sore throat, asthenia, myalgia, anorexia, vomiting and diarrhea. Upon questioning, the patient disclosed having a rash on the trunk and penis ulcers for the last two days, and that he had non-consensual sex with a man two months prior. Physical examination was notable for a macular rash on the trunk, pharyngitis, generalized lymphadenopathy and painful ulcers on the glans and prepuce. Herpes simplex type 1 was detected in genital lesions by Polymerase Chain Reaction. Rapid HIV test was indeterminate and confirmatory test by immunochromatography was negative. Plasma HIV viral load was 1.681.383 copies/ml, 4th generation immunoassay (including HIV antibodies and p24 antigen detection) was positive and CD4 lymphocyte count was 327 cells/μl. He was treated with acyclovir and started antiretroviral therapy regimen, consisting of tenofovir, emtricitabine and darunavir/cobicistat that was given under supervision at home after discharge. Comments: Pediatricians must be aware of the signs of acute HIV infection and other sexually transmitted diseases in adolescent patients. Prompt diagnosis helps to prevent further transmission and early antiretroviral therapy improves outcomes

[Ledipasvir/sofosbuvir combination for chronic hepatitis C infection in children and adolescents]. / Combinación de ledipasvir/sofosbuvir como tratamiento de la infección crónica por hepatitis C.

INTRODUCTION: Hepatitis C virus infection is world health problem. The aim of this study was to assess the safety and efficacy of ledipasvir/sofosbuvir combination in chronic Hepatitis C Virus (HCV) genotype 1 and 4 infection in paediatric patients. METHODS: Eligible patients to be treated with ledipasvir/sofosbuvir were patients from 6 to 18 years old with a chronic HCV genotype 1 or 4 infection. The duration and doses of antiviral drugs were changed depending on patient age, fibrosis stage, and PEGylated interferon+ribavirin experience status. The primary efficacy endpoint was the percentage of patients with a sustained virological response 12 weeks post-treatment. RESULTS: A total of nine patients (7 males) with a median age of 14.8 years (8.48-17.91) were treated with ledipasvir/sofosbuvir combination. Five patients received previous treatment with PEGylated interferon+ribavirin during a median of 8.5 months (3-12 months). Eight patients had some degree of fibrosis (1 patient presented with F1, three patients F2, 2 patients F3, and 2 patients F4). The median pre-treatment viral load was 6.2 Log [5.9-6.8] with the HCV RNA becoming negative six weeks after starting the treatment in 100% of the patients. All patients maintained a sustained viral response at 12 weeks. Three patients (33.3%) had some type of adverse effect (2 headache and one oral thrush). The median post-treatment follow-up was 24 weeks (12-104). CONCLUSIONS: Treatment with ledipasvir/sofosbuvir in paediatric patients with chronic HCV infection genotype 1 and 4 is safe and effective with SVR12 and similar to those reported in adults.