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.

Validation of the Spanish version of the Electronic Facial Palsy Assessment (eFACE).

PURPOSE: The clinician-graded electronic facial paralysis assessment (eFACE) is a relatively new digital tool for assessing facial palsy. The present study aimed to determine the validity and reliability of the Spanish version of the eFACE. METHODS: Forward-backward translation from the original English version was performed. Videos and photographs from 65 adult patients with unilateral facial paralysis (any severity, time course, and etiology) were evaluated twice by five otolaryngologists with varying levels of experience in facial palsy evaluation. Internal consistency was measured using Cronbach's α and the intra- and inter-rater reliability were measured using intraclass correlation coefficient. Concurrent validity was established by calculating Spearman's rho correlation (ρ) between the eFACE and the House-Brackmann scale (H-B) and Pearson's correlation (r) between the eFACE and the Sunnybrook Facial Grading System (SFGS). RESULTS: The Spanish version of the eFACE showed good internal consistency (Cronbach's α > 0.8). The intra-rater reliability was nearly perfect for the total score (intraclass correlation coefficient: 0.95-0.99), static score (0.92-0.96), and dynamic score (0.96-0.99) and important-to-excellent for synkinesis score (0.79-0.96). The inter-rater reliability was excellent for the total score (0.85-0.93), static score (0.80-0.90), and dynamic score (0.90-0.95) and moderate-to-important for the synkinesis score (0.55-0.78). The eFACE had a very strong correlation with the H-B (ρ = - 0.88 and - 0.85 for each evaluation, p < 0.001) and the SFGS (r = 0.92 and 0.91 each evaluation, p < 0.001). CONCLUSION: The Spanish version of the eFACE is a reliable and valid instrument for assessment of facial function in the diagnosis and treatment of patients with facial paralysis.

Single fetal demise in monochorionic twins: How to predict cerebral injury in the survivor co-twin?

INTRODUCTION: The aims of the study were to evaluate perinatal outcome in monochorionic (MC) twins complicated with single intrauterine fetal death, spontaneously vs after fetal therapy, and to assess antenatal events that increase the risk of cerebral injury. MATERIAL AND METHODS: Historical cohort study of MC pregnancies with single intrauterine fetal death diagnosed or referred to a tertiary referral hospital (2012-2020). Adverse perinatal outcome included termination of pregnancy, perinatal death, abnormal fetal or neonatal neuroimaging and abnormal neurological development. RESULTS: A total of 68 MC pregnancies with single intrauterine fetal death after 14 weeks of gestation were included. Sixty-five (95.6%) occurred in complicated MC pregnancies (twin to twin transfusion syndrome: 35/68 [51.5%]; discordant malformation: 13/68 [19.1%], selective intrauterine growth restriction: 10/68 [14.7%], twin reversed arterial perfusion sequence: 5/68 [7.3%] and cord entanglement in monoamniotic twins: 2/68 [2.94%]). In 52 cases (76.5%) single intrauterine fetal demise occurred after fetal therapy and in 16 (23.5%) occurred spontaneously. Cerebral damage included 14/68 cases (20.6%): 6/68 cases (8.82%) were prenatal lesions and 8/68 cases (11.8%) were postnatal. Risk of cerebral damage tended to be higher in the spontaneous death group (6/16, 37.5%) compared to the therapy-group (8/52, 15.38%) (p = 0.07). The risk increased with gestational age at intrauterine death (OR 1.21, 95% CI: 1.04-1.41, p = 0.014) and was higher in those surviving co-twins who developed anemia (OR 9.27, 95% CI: 1.50-57.12, p = 0.016). Pregnancies complicated with selective intrauterine growth restriction tended to be at higher risk for neurological damage (OR 2.85, 95% CI: 0.68-11.85, p = 0.15). Preterm birth rate (<37 weeks of pregnancy) was 61.7% (37/60). Seven of eight postnatal cerebral lesions (87.5%) were related to extreme prematurity. Overall perinatal survival rate was 88.3% (57/68) and 7% (4/57) of children had an abnormal neurological outcome. CONCLUSIONS: Risk of cerebral damage in single intrauterine fetal death is especially high when it occurs spontaneously. Gestational age at single intrauterine fetal death, selective intrauterine growth restriction and anemia of the surviving co-twin are the main predictors for prenatal lesions and might be useful in parent counseling. Abnormal postnatal neurological outcome is closely related to extreme prematurity.

Análisis de las diferencias de calibre entre las venas mamarias internas derecha e izquierda y su relación con múltiples factores en reconstrucción microquirúrgica de mama / Analysis of caliber differences between right and left internal mammary veins and their relationship to multiple factors in microsurgical breast reconstruction

Introducción y objetivo: La congestión venosa es la principal causa de complicación en la reconstrucción microquirúrgica de mama. Entre sus posibles motivos, son de especial interés las características de los vasos receptores. Las venas mamarias internas son la primera opción como venas receptoras. El objetivo del presente estudio es evaluar las posibles diferencias existentes entre las venas mamarias internas izquierda y derecha. Material y método: Estudio observacional en 31 casos de reconstrucción mamaria con colgajo DIEP (deep inferior epigastric perforator flap – colgajo de perforante epigástrica inferior) seleccionando los vasos mamarios internos como vasos receptores para comparar posibles diferencias entre el calibre de las venas mamarias internas derecha e izquierda. Resultados: Fueron estadísticamente signifucativos mostrando un calibre de la vena mamaria interna derecha mayor al de la izquierda. Conclusiones: Nuestro estudio ha encontrado diferencias estadísticamente significativas en el calibre de las venas mamarias internas, presentando mayor tamaño las derechas sin verse modificado este resultado por el empleo de radioterapia previa a la cirugía ni por condiciones propias de la paciente, como su índice de masa corporal o su edad. (AU)

Background and objective: Venous congestion is the main resource of complication in microsurgical breast reconstruction. Among the possible causes of this congestion, the characteristics of the recipient vessels are of special interest. Internal mammary veins are the first choice as recipient vessels. The aim of this study is to evaluate the possible differences between the left and right internal mammary veins. Methods: Observational research conducted on 31 cases of breast reconstruction with DIEP flap (deep inferior epigastric perforator flap), selecting the internal mammary vessels as recipient vessels in order to compare possible differences between the caliber of right and left internal mammary veins. Results: The result were statistically significant, showing a larger caliber of the right internal mammary vein than the left. Conclusions: Our study has found statistically significant differences in the caliber of the internal mammary veins, with the right ones being larger without these results being modified using radio-therapy prior to surgery or by the patients own conditions, such as her body mass index or age. (AU)

Validation of the Spanish version of the Sunnybrook facial grading system.

PURPOSE: The Sunnybrook facial grading system (SFGS) is one of the most widely employed tools to assess facial function. The present study aimed to determine the validity and reliability of the Spanish language version of the SFGS. METHODS: Forward-backward translation from the original English version was performed by fluent speakers of English and Spanish. Videos from 65 patients with facial paralysis (FP) were evaluated twice by five otolaryngologists with experience in FP evaluation. Internal consistency and intra- and inter-rater reliability were assessed. The House-Brackmann scale was used to display concurrent validity which was established by Spearman's rho correlation. RESULTS: The Cronbach's α score exceeded 0.70. The intra-rater intraclass correlation coefficient (ICC) was nearly perfect for the composite score (0.96-0.99), voluntary movements (0.97-0.99), and synkinesis (0.91-0.98), and important to almost perfect for symmetry at rest (0.79-0.97). In both evaluations, the inter-rater ICC was higher than 0.90 for the composite score (0.92-0.96) and voluntary movements (0.91-0.96) and slightly lower for symmetry at rest (0.66-0.85) and synkinesis (0.72-0.87). A strong negative correlation was found between the H-B scale and SFGS (Spearman's rho coefficient = - 0.92, p < 0.001) in both evaluations. CONCLUSION: The Spanish version of the SFGS is a reliable and valuable instrument for the assessment of facial function in the diagnosis and treatment of patients with FP.

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.

Influence of Full Oral Feeding Acquisition on Growth of Premature Infants.

Objective: The main objective was to describe the impact of full oral feeding achievement in very low birth weight infants on weight, length, and head circumference, measured as the change in z-score from 32 weeks to discharge, the time at which full oral feeding occurs. Methods: This was a longitudinal retrospective observational study on infants younger than 30 weeks of gestational age, admitted to the Neonatology Unit of La Paz University Hospital, Madrid (Spain), from January 1, 2019 to December 31, 2019. The infant's anthropometric characteristics (weight, height, and head circumference) were compared at birth, at 32, 34, and 36 weeks of gestational age, at the time of full oral feeding, and at discharge from the unit. Results: A total of 66 infants were included, gestational age at birth range from 24 to 30. Full oral intake occurred at 37.1 ± 2.1 weeks postmenstrual age (PMA). We found an inverse correlation between gestational age at birth and birth weight with PMA at which full oral feeding (FOF) is achieved. PMA at discharge was 38.6 ± 2.5 weeks. Age of full oral intake and discharge occurred later in infants who had patent ductus arteriosus, retinopathy of prematurity, and sepsis or received a blood transfusion. A positive correlation was found between days of oxygen and both parameters. However, we found no relationship between necrotizing enterocolitis or intraventricular hemorrhage with age at full oral feeding or age at discharge. Conclusions: The transition from gastric tube to oral intake did not affect growth. We found a close relationship between preterm infants birth, earlier younger than 30 weeks of gestational age, and low birth weight, with a delay in full oral feeding achievement that correlated with age at discharge.

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.

Profile and 3-month evolution of geriatric patients after a hip fracture followed-up at a Fracture Liaison Service (FLS).

INTRODUCTION: There are few studies on Fracture Liaison Service (FLS) that evaluate older patients after a hip fracture (HF) through comprehensive geriatric assessment. We aim to determine these patients' characteristics, outcomes, and prescribed treatments. METHODS: A retrospective observational study of a cohort of patients older than 65 years admitted with HFs to an orthogeriatric unit between February 25th (2013) and December 16th (2016). After hospitalization, those patients with a good baseline social, functional, and cognitive situation were referred to the FLS. A comprehensive geriatric assessment and treatment adjustment were conducted. A comparison between FLS patients and HF patients non-referred was made. RESULTS: From 1887 patients admitted to the orthogeriatric unit, 469 (23%) were referred to the FLS. Of those, 335 were women (77.2%) and 337 (77.6%) lived in the community. The FLS patients had a better functional status (97.1% of the patients with independent gait versus 79.2%) than non-FLS patients (p<0.001). After 3 months in the FLS, 356 (82%) patients had independent gait and had improved their analytical values. Antiosteoporotic treatment was prescribed to 322 patients (74%), vitamin D supplements to 397 (91.5%), calcium to 321 (74%), and physical exercise to 421 (97%). CONCLUSIONS: Patients referred to an FLS were younger, with a better functional and cognitive situation. At hospital discharge, they frequently presented gait impairment and laboratory abnormalities (anemia, hypoproteinemia, vitamin D deficiency) that presented good recovery due to the patient's previous baseline. These patients benefit from comprehensive treatment (pharmacological and non-pharmacological).

Profile and 3-month evolution of geriatric patients after a hip fracture followed-up at a Fracture Liaison Service (FLS) / Perfil y evolución a 3 meses de pacientes con fractura de cadera evaluados en una unidad de coordinación de fracturas (Fracture Liaison Service [FLS])

Introduction: There are few studies on Fracture Liaison Service (FLS) that evaluate older patients after a hip fracture (HF) through comprehensive geriatric assessment. We aim to determine these patients’ characteristics, outcomes, and prescribed treatments.Methods: A retrospective observational study of a cohort of patients older than 65 years admitted with HFs to an orthogeriatric unit between February 25th (2013) and December 16th (2016). After hospitalization, those patients with a good baseline social, functional, and cognitive situation were referred to the FLS. A comprehensive geriatric assessment and treatment adjustment were conducted. A comparison between FLS patients and HF patients non-referred was made.Results: From 1887 patients admitted to the orthogeriatric unit, 469 (23%) were referred to the FLS. Of those, 335 were women (77.2%) and 337 (77.6%) lived in the community. The FLS patients had a better functional status (97.1% of the patients with independent gait versus 79.2%) than non-FLS patients (p<0.001). After 3 months in the FLS, 356 (82%) patients had independent gait and had improved their analytical values. Antiosteoporotic treatment was prescribed to 322 patients (74%), vitamin D supplements to 397 (91.5%), calcium to 321 (74%), and physical exercise to 421 (97%).Conclusions: Patients referred to an FLS were younger, with a better functional and cognitive situation. At hospital discharge, they frequently presented gait impairment and laboratory abnormalities (anemia, hypoproteinemia, vitamin D deficiency) that presented good recovery due to the patient's previous baseline. These patients benefit from comprehensive treatment (pharmacological and non-pharmacological). (AU)

Introducción: Hay pocos estudios sobre las unidades de coordinación de fracturas (Fracture Liaison Services [FLS]) que evalúen a pacientes mayores tras una fractura de cadera (FC) a través de una valoración geriátrica integral. Nuestro objetivo es determinar las características de estos pacientes, los resultados y los tratamientos prescritos.Métodos: Estudio observacional retrospectivo de una cohorte de mayores de 65años ingresados tras fractura de cadera (FC) entre el 25 de febrero de 2013 y el 31 de diciembre de 2016 en una unidad de ortogeriatría. Tras el alta hospitalaria, los pacientes con buen soporte social y buena situación funcional y cognitiva fueron citados en la FLS. Se realizó una evaluación geriátrica integral y un ajuste del tratamiento. Dichos pacientes se compararon con pacientes con fractura de cadera no derivados a esta unidad.Resultados: Un total de 1.887 pacientes ingresaron en la unidad de ortogeriatría, y 469 (23%) fueron derivados a la FLS. De ellos, 335 fueron mujeres (77,2%) y 337 (77,6%) vivían en el domicilio. Los atendidos en la FLS tuvieron mejor funcionalidad (97,1% de pacientes con deambulación independiente versus 79,2%) que los no incluidos (p<0,001). A los 3meses en la FLS, 356 (82%) pacientes presentaban deambulación independiente y habían mejorado sus valores analíticos. Se prescribieron antiosteoporóticos a 332 pacientes (74%), suplementos de vitaminaD a 397 (91,5%), calcio a 321 (74%) y ejercicio físico a 421 (97%).Conclusiones: Los pacientes atendidos en una FLS fueron más jóvenes, con mejor situación funcional y cognitiva. Al alta hospitalaria, frecuentemente presentaron inestabilidad de la marcha y alteraciones analíticas (anemia, hipoproteinemia, déficit de vitamina D) que tuvieron buena evolución dado el estado previo del paciente. Estos pacientes se benefician de un tratamiento integral (farmacológico y no farmacológico). (AU)