Management of paediatric monomorphic post-transplant lymphoproliferative disorders with low-intensity treatment: A multicentre international experience.

BACKGROUND: Monomorphic post-transplant lymphoproliferative disorder (mPTLD) is a major cause of morbidity/mortality following solid organ transplant (SOT), with infection, mPTLD progression and organ rejection presenting equal risks. Balancing these risks is challenging, and the intensity of therapy required by individual patients is not defined. Although an increasing body of evidence supports the use of a stepwise escalation of therapy through reduction in immunosuppression (RIS) to rituximab monotherapy and low-dose chemo-immunotherapy, many centres still use B-cell non-Hodgkin lymphoma (B-NHL) protocols, especially when managing Burkitt/Burkitt-like (BL) PTLD. This study sought to define outcomes for children managed in the UK or Spanish centres using low-intensity first-line treatments. PROCEDURE: Retrospective data were anonymously collected on patients younger than 18 years of age, with post-SOT mPTLD diagnosed between 2000 and 2020. Only patients given low-intensity treatment at initial diagnosis were included. RESULTS: Fifty-six patients were identified. Age range was 0.9-18 years (median 10.7). Most (62.5%) had early-onset PTLD. Haematopathological analysis showed 75% were diffuse large B-cell like, 14.3% were BL and nine of 33 (27%) harboured a MYC-rearrangement. Stage III-IV disease was present in 78.6%. All but one had RIS, 26 received rituximab monotherapy and 24 low-dose chemo-immunotherapy, mostly R-COP. Intensified B-NHL chemotherapy was required in 10/56 (17.9%). There were a total of 13 deaths in this cohort, three related to PTLD progression. The 1-year overall survival (OS), event-free survival (EFS) and progression-free survival (PFS) were 92.8%, 78.6% and 80.2%, respectively. CONCLUSIONS: R-COP provides an effective low-dose chemotherapy option. Escalation to more intensive therapies in the minority of inadequately controlled patients is an effective strategy.

Drain use can be avoided in reverse shoulder arthroplasty.

BACKGROUND: Drains have demonstrated no clear benefits and some potentially harmful effects in hip and knee replacements. There is little evidence about the effects of its use in shoulder arthroplasty. We hypothesized that drain use would increase postoperative blood loss without reducing wound complications. METHODS: We included 103 reverse shoulder arthroplasties (RSA), 71 were operated for degenerative pathology, 32 due to a fracture. All complications were recorded. Hemoglobin (Hb) and hematocrit (Htc.) level were collected and compared to postoperative data. Length of hospitalization and volume output were also noted. RESULTS: 45 patients received a closed-suction drain. Patients with coagulopathy had significant higher bleeding and were excluded (p = 0.03). Patients operated for a fracture were older (80.1y.o vs 72.1 p < 0.01) and had higher blood drop (∆Hb p = 0.01; ∆Htc p = 0.03). There were neither differences between drain and control group in ∆Hb or ∆Htc in the degenerative RSA group (1.84+/-0.89 vs 1.68+/-0.84, p = 0.36; 5.78+/-2.89 vs 5.53+/-2.87 p = 0.50) nor in the fracture RSA group (2.65+/-0.94 vs 2.65+/-1.01, p = 0.90; 7.91+/-2.99 vs. 7.09+/-4.21, p = 0.56). There were neither differences in complications (degenerative p = 0.33; fracture p = 0.21). Drain use was related to a longer hospital stay in elective surgery (2.6 vs 1.8 days; p < 0.01). DISCUSSION: The rate of complication is similar between patients with and without drain use. Drain use after shoulder arthroplasty does not affect postoperative bleeding but increases the length of hospital stay. Drains seems to be an unnecessary intervention after RSA that may increase associated costs and can be safely abandoned. LEVEL OF EVIDENCE: Level III retrospective comparative study.

Papel de la ecocardiografía speckle-tracking (rastreo de marcas) y la resonancia magnética cardíaca-feature tracking en la circulación de Fontan. Diferencias entre los ventrículos derecho e izquierdo / Speckle-tracking echocardiography and MCR-feature tracking in Fontan circulation. Differences between right and left ventricles

Resumen Introducción: En los pacientes con DCPT, la disfunción ventricular es inevitable, y más temprana en VU derechos. La deformación miocárdica por STE y RMC-FT parece promisoria. Objetivo: Analizar la función ventricular mediante STE y RMC-FT en pacientes con DCPT, en comparación con RMC convencional según la morfología del VU y la posible implicación en su diagnóstico temprano. Método: Se recogieron medidas del strain longitudinal y circunferencial por STE y RMC-FT, volúmenes ventriculares y FE por RMC en 64 pacientes con DCPT. Resultados: La morfología ventricular no se relacionó con disfunción por RMC. Los VU derechos tuvieron valores por STE y RMC-FT disminuidos respecto de los VU izquierdos, con FE similares. Existe correlación entre STE y RMC-FT, no equivalentes, con buena factibilidad y reproducibilidad. Conclusiones: La RMC-FT y el STE son técnicas útiles en el diagnóstico temprano y la vigilancia de la función ventricular en VU derechos con FE preservada.

Abstract Introduction: In patients with TCPC, the development of ventricular dysfunction is inevitable and is more precocious in SRVs. Myocardial deformation by STE and CMR-FT is promising. Objective: To analize ventricular function in patients with TCPC using STE and CMR-FT compared with conventional cMRI, depending on SV morphology, to determine their role in early diagnosis of ventricular dysfunction. Method: Sixty-four patients with TCPC were included. Longitudinal and circumferential strain by STE and CMR-FT and ventricular volume and EF were obtained. Results: Dysfunction analyzed by cMRI showed no association with ventricular morphology. SRVs had lower values in STE and CMR-FT compared with SLVs, with similar EF. While not equivalent, correlation was observed between the STE and the CMR-FT values, demonstrating good feasibility and reproducibility. Conclusion: The strain data in CMR-FT and STE could be useful for diagnosis and monitoring of ventricular function and as markers of early SRV dysfunction with preserved EF.

Uso de pomada de tacrolimus al 0, 1 por ciento durante un año en niños con vitiligo / Treatment during a year with 0.1 por ciento tacrolimus ointment in young patients with vitiligo

Introducción: El vitiligo es una alteración cutánea con un carácter predominantemente autoinmunitario, caracterizada por despigmentación de la piel y del pelo. En los niños, además de ser un problema estético constituye un reto terapéutico, ya que los tratamientos estándar (fototerapia y corticoesteroides) producen frecuentes efectos adversos. Materiales y métodos: En un estudio retrospectivo se evaluó la respuesta del tacrolimus tópico al 0,1 % durante un año, en 22 niños con vitiligo, en seguimiento en la Unidad de Dermatología Pediátrica del Hospital La Paz, durante el periodo comprendido de 2002 a 2008. Resultados: Se observó algún grado de repigmentación en 81,8 % de los pacientes. La repigmentación fue significativa en cara, cuello y extremidades, a los 9 meses, y en el tronco, a los 12 meses. No se observaron efectos secundarios sistémicos durante el seguimiento. Conclusión: El tacrolimus tópico podría considerarse como una opción terapéutica, en la población pediátrica con vitiligo.