Optical coherence tomography for the screening of anterior chamber inflammation in paediatric patients diagnosed with juvenile idiopathic arthritis.

OBJECTIVES: To evaluate the effectiveness of the anterior segment optical coherence tomography (AS-OCT) for the screening of anterior uveitis in children diagnosed with juvenile idiopathic arthritis (JIA). METHODS: A cross-sectional, observational, non-randomised study was conducted in JIA patients younger than 18 years. All patients underwent anterior segment (AS-OCT) and macular OCT. RESULTS: A total of 300 eyes of 150 patients diagnosed with JIA were included; 74% were females, and mean age was 11.12 ± 3.51 years old (range 4.13-18.60). In the slit-lamp examination, anterior uveitis was diagnosed in 16 eyes. In the AS-OCT, anterior uveitis was suspected in 27 eyes; cells were detected in 27 eyes and retrokeratic precipitates in 5 eyes. Sensitivity was 0.94 and specificity was 0.96, positive predictive value was 0.59 and negative predictive value was 0.99, and Kappa-Cohen index was 0.71. CONCLUSIONS: AS-OCT could be considered for the screening of anterior segment uveitis in children diagnosed with JIA.

Similarities and differences between systemic juvenile idiopathic arthritis and adult-onset Still's disease: a multicenter Spanish study.

To describe the characteristics of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), compare their presentation and evolution, and analyse possible complication predictors. Multicenter study. Data were retrieved from a hospital-based study of patients with a diagnosis or suspected diagnosis of sJIA or AOSD according to the responsible physician and followed-up for at least one year. Descriptive variables (classification criteria, clinical manifestations, complications, family, and personal history) were collected at disease onset and during follow-up. We present the clinical characteristics of 326 patients, 67% of whom had a diagnosis of sJIA and 33% of AOSD. Clinical manifestation frequencies were similar between the two groups, except for odynophagia, which was significantly more frequent in AOSD than in sJIA (78.4% vs. 25.5%; p < 0.0001). Among the complications, macrophage activation syndrome (MAS) was significantly more common in sJIA than in AOSD (24.4% vs. 9.5%; p = 0.002), to the extent that an sJIA diagnosis significantly increased the risk of MAS, together with serositis presence, and the need for biological therapy. Patients with sJIA and AOSD showed similar characteristics, supporting the idea that they are both part of Still's disease, but are expressed at different ages. Differences in manifestations and complications might be due to different management between diseases and immune response maturity.

Clinical characteristics and prognostic factor in juvenile dermatomyositis: data of the Spanish registry.

BACKGROUND: Juvenile Dermatomyositis (JDM) is the most common chronic idiopathic inflammatory myopathy in children. The diagnosis is clinical. Baseline laboratory and complementary studies trace the phenotype of these patients. The objective of this study was to describe epidemiological, clinical and laboratory characteristics at diagnosis of JDM patients included in the Spanish JDM registry, as well as to identify prognostic factors on these patients. METHODS: We retrospectively reviewed clinical features, laboratory tests, and complementary studies at diagnosis of JDM patients included on the Spanish JDM registry. These data were analyzed to assess whether there was a relationship with the development of complications and time to disease inactivity. RESULTS: One hundred and sixteen patients from 17 Spanish paediatric rheumatology centres were included, 76 girls (65%). Median age at diagnosis was 7.3 years (Interquartile range (IQR) 4.5-10.2). All patients had pathognomonic skin lesions at the beginning of the disease. Muscle weakness was present in 86.2%. Median Childhood Muscle Assessment Scale was 34 (IQR 22-47). Twelve patients (34%) had dysphagia and 3,5% dysphonia. Anti-p155 was the most frequently detected myositis specific antibody, followed by anti-MDA5. Twenty-nine patients developed calcinosis and 4 presented with macrophage activation syndrome. 70% reached inactivity in a median time of 8.9 months (IQR 4.5-34.8). 41% relapsed after a median time of 14.4 months (IQR 8.6-22.8) of inactivity. Shorter time to treatment was associated with better prognosis (Hazard ratio (HR) = 0.95 per month of evolution, p = 0.02). Heliotrope rash at diagnosis correlates with higher risk of development complications. CONCLUSIONS: We describe heliotrope rash as a risk factor for developing complications in our cohort of JDM patients, an easy-to-evaluate clinical sign that could help us to identify the group of patients we should monitor closely for this complication.

Similarities and differences between the immunopathogenesis of COVID-19-related pediatric multisystem inflammatory syndrome and Kawasaki disease.

Multisystem inflammatory syndrome associated with the SARS-CoV-2 pandemic has recently been described in children (MIS-C), partially overlapping with Kawasaki disease (KD). We hypothesized that (a) MIS-C and prepandemic KD cytokine profiles may be unique and justify the clinical differences observed, and (b) SARS-CoV-2-specific immune complexes (ICs) may explain the immunopathology of MIS-C. Seventy-four children were included: 14 with MIS-C, 9 patients positive for SARS-CoV-2 by PCR without MIS-C (COVID), 14 with prepandemic KD, and 37 healthy controls (HCs). Thirty-four circulating cytokines were quantified in pretreatment serum or plasma samples and the presence of circulating SARS-CoV-2 ICs was evaluated in MIS-C patients. Compared with HCs, the MIS-C and KD groups showed most cytokines to be significantly elevated, with IFN-γ-induced response markers (including IFN-γ, IL-18, and IP-10) and inflammatory monocyte activation markers (including MCP-1, IL-1α, and IL-1RA) being the main triggers of inflammation. In linear discriminant analysis, MIS-C and KD profiles overlapped; however, a subgroup of MIS-C patients (MIS-Cplus) differentiated from the remaining MIS-C patients in IFN-γ, IL-18, GM-CSF, RANTES, IP-10, IL-1α, and SDF-1 and incipient signs of macrophage activation syndrome. Circulating SARS-CoV-2 ICs were not detected in MIS-C patients. Our findings suggest a major role for IFN-γ in the pathogenesis of MIS-C, which may be relevant for therapeutic management.

Resultados de la reparación de Bankart/plicatura y remplissage en el tratamiento de la luxación anteroinferior recurrente del hombro. Correlación con la escala de ISIS / Bankart repair and remplissage in recurrent anteroinferior shoulder dislocation, Correlation with ISIS scale

Introducción: La luxación recurrente anteroinferior del hombro es consecuencia de una luxación aguda traumática que, en pacientes jóvenes, menores de veinte años, se ha estimado puede llegar al 97% de recurrencia, pero que disminuye a medida que aumenta la edad, excepto en pacientes mayores con rupturas asociadas del manguito rotador. Al ser una patología incapacitante que afecta la calidad de vida de las personas, hay una mayor tendencia a tratarla por métodos quirúrgicos que con rehabilitación. La lesión fundamental es el desprendimiento del labrum anterior de la glenoides conocida como la lesión de Bankart-Perthes, estructura que tiene limitaciones para cicatrizar de manera anatómica con tratamientos incruentos, perpetuando la inestabilidad. A medida que recurre la luxación se presentan lesiones óseas asociadas tanto en la glenoides denominada Bankart ósea, así como en la cabeza humeral en su región posterosuperior o lesión de Hill-Sachs, que aumentan la posibilidad de recurrencia. Se han descripto diferentes técnicas abiertas y artroscópicas para corregir la luxación, pero por la heterogeneidad de la lesión no hay un tratamiento para corregirla que sea patrón de oro. Nuestro objetivo es describir los resultados clínicos en pacientes con luxación recurrente anteroinferior de hombro que fueron sometidos a cirugía artroscópica de reparación de Bankart/plicatura y remplissage y correlacionar los resultados con la escala preoperatoria de ISIS. Materiales y métodos: se revisaron las historias clínicas de pacientes con diagnóstico de luxación de la articulación del hombro (S430) (M253), que fueron llevados a cirugía entre junio de 2015 y enero de 2019, analizando características de la(s) lesión(es), clasificación preoperatoria de acuerdo con la escala de ISIS, tipo de procedimiento(s) quirúrgico realizado y número de implantes. Resultados: de veinticinco pacientes operados por el autor senior (MMA) de luxación recurrente de hombro, veintitrés completaron el seguimiento con los procedimientos de reparación de Bankart/plicatura y la adición de un remplissage en casos de lesiones Hill-Sachs enganchantes. Todos tenían una escala de ISIS preoperatoria igual o inferior a 6. Al final del seguimiento ningún paciente reportó recurrencia de su luxación. Conclusión: en nuestra casuística, la reparación de Bankart/plicatura en pacientes con lesiones Hill-Sachs no enganchantes en asocio del remplissage produce excelentes resultados a corto y mediano plazo cuando la escala de ISIS es igual o inferior a 6. Nivel de evidencia: IV

Introduction: Recurrent anteroinferior shoulder dislocation is the consequence of an acute traumatic dislocation which, in young patients, under twenty years old, has been estimated to reach 97% of recurrence, but which decreases with increasing age, except in older patients with associated rotator cuff tears. Being a disabling pathology that affects people's quality of life, there is a greater tendency to treat it by surgical methods than with rehabilitation. The fundamental lesion is the detachment of the anterior labrum of the glenoid known as the Bankart-Perthes lesion, a structure that has limitations to heal anatomically with bloodless treatments, perpetuating instability. As the dislocation recurs, there are associated bone lesions both in the glenoid called Bony Bankart, as well as in the humeral head in its posterior superior region or Hill-Sachs lesion, which increase the possibility of recurrence. Different open and arthroscopic techniques have been described to correct dislocation, but due to the heterogeneity of the lesion, there is no gold standard treatment to correct it. Our objective is to describe the clinical results in patients with recurrent anteroinferior shoulder dislocation who underwent arthroscopic Bankart/plication repair and fill surgery and correlate the results with the preoperative ISIS scale. Materials and methods: the medical records of patients with a diagnosis of shoulder joint dislocation (S430) (M253), who were taken to surgery between June 2015 and January 2019, were reviewed, analyzing characteristics of the injury(s), classification preoperative according to the ISIS scale, type of surgical procedure(s) performed and number of implants. Results: of twenty-five patients operated by the senior author (MMA) for recurrent shoulder dislocation, twenty-three completed follow-up with Bankart/plication repair procedures and the addition of a fill in Hill-Sachs engaging lesions. All had a preoperative ISIS scale equal to or less than 6. At the end of follow-up, no patient reported recurrence of their dislocation.Conclusion: in our casuistry, Bankart/plication repair in patients with non-engaging Hill-Sachs lesions in association with filling produces excellent results in the short and medium term when the ISIS scale is equal to or less than 6. Level of Evidence: IV

Encefalitis de Bickerstaff, síndrome oespectro de Fisher Bickerstaff, reporte de dos casos / Bickerstaff encephalitis/Fisher Bickerstaff syndrome, report of two cases

La encefalitis de Bickerstaff es una entidad infrecuente de origen posinfeccioso, de presentación grave y con un curso clínico generalmente benigno, cuya diferenciacióncon el síndrome de Fisher aún no es clara y su asociación con el síndrome de Guillain - Barré está ya establecida. Presentamos dos casos atendidos en el Hospital Universitario San Ignacio...

Bickerstaff encephalitis is a rare post-infectious origin disease, with an acute severe presentation form, but generally with a benign course, its differentiation from Fisher syndrome is unclear, and its association with Guillain - Barre syndrome is alreadyestablished. We report two cases treated at the Hospital Universitario San Ignacio...

Incidencia del transtorno de estres postraumático en pacientes atendidos en el servicio de urgencias del Hospital Regional Simón Bolívar con herida toracoabdominal por arma de fuego y/o cortopunzante / Post traumatic stress disorder in firearm wound or steelarm wound in thorax and/or abdomen

El trastorno del estrés postraumático (TSPT) es una entidad nosológica clasificada desde 1980 por el DSM III, asociado a eventos traumáticos extraordinarios para el paciente y se presenta al cabo de mínimo 6 horas. El estudio abarca una muestra de 31 pacientes, seleccionados por haber sufrido herida por arma de fuego o cortopunzante en tórax y/o abdomen, de los cuales 16 cumplieron los criterios para el DSM III-R para el TSPT. Se encontró que el criterio que mas se cumplía en la población estudiada era el criterio referente a revivir el evento (26 pacientes) (criterio B). El item que más cumplieron los 31 pacientes fué el referido a alteración psicológica ante situaciones similares al evento (criterio B4) correspondiendo al 100 por ciento de población estudiada; de los pacientes que desarrollaron el TSPT todos presentaron hipervigilancia con una significancia estadística (p<0.0001). El estudio permite mostrar que el TSPT si se presenta en nuestra población además permite sugerir que los pacientes con las características establecidas para el presente estudio ameritan un control psicológico a mediano plazo como mínimo