Quality of Life After Lower Leg Reconstruction With the Latissimus Dorsi Free Flap in Pediatric Patients.

BACKGROUND: The latissimus dorsi free flap is a widely used reconstructive technique for complex lower leg defects in the pediatric population due to its reliability and anatomical features. However, the impact of this technique on the postoperative quality of life in children and adolescents, who require appropriate lower extremity function during their developmental period, remains to be analyzed. METHODS: Patients who underwent microsurgical lower leg reconstruction using the latissimus dorsi flap were analyzed retrospectively. The quality of life of these patients was assessed prospectively using the Lower Extremity Functional Scale (LEFS) at a minimum of 18 months after surgical reconstruction. RESULTS: Sixteen pediatric patients who had severe lower extremity injuries and underwent latissimus dorsi free flap reconstruction met the inclusion criteria. The mean follow-up period was 33.9 months (22-64 months). Two patients experienced postoperative complications: one had partial flap necrosis and surgical site infection, while the other developed a surgical site infection. The LEFS scores ranged from 26 to 80, with a mean score of 64.6. Remarkably, 14 of 16 patients achieved LEFS scores consistent with at least the 10th percentile when compared with normative data. Patients with severe associated fractures presented with the lowest scores. CONCLUSIONS: Based on our findings, the latissimus dorsi flap is reaffirmed to be an excellent choice for lower leg reconstruction in the pediatric population. It effectively restores the quality of life in patients who have experienced moderate to severe lower extremity injuries.

Experiencia de uso de hierro intravenoso para el tratamiento de la anemia en niños y adolescentes. Respuesta terapéutica y evolución / Experience in using intravenous iron for the treatment of anemia in children and adolescents. Therapeutic response and evolution

Introducción: El hierro de administración intravenosa (iv) está indicado en los casos en que el tratamiento oral no es posible. El objetivo de este trabajo fue describir el perfil de uso, respuesta terapéutica y seguridad de la administración de hierro iv en el tratamiento de la anemia ferropénica en niños, niñas y adolescentes (NNA) asistidos en un centro de referencia de Uruguay entre 2018 y 2023. Método: Estudio retrospectivo mediante revisión de historias. Incluyó todos los NNA que recibieron hierro iv. Se registraron variables sociodemográficas, comorbilidades, clínica y severidad de la anemia. Se evaluó: motivos de la indicación y tipo de hierro iv, dosis, tiempo de infusión, respuesta terapéutica y efectos adversos. Resultados: Se incluyeron 35 pacientes, mediana de edad 4 años; 51,4% de sexo masculino, con comorbilidades 37,1%. Todos los menores de 3 años presentaban factores de riesgo para anemia ferropénica, la falta de adherencia al hierro oral se asoció con mayor severidad de la anemia (p<0,05). El motivo principal de indicación de hierro iv fue la severidad de la anemia e inadecuada respuesta al hierro oral concomitante en 37,1%. Todos recibieron hierro sacarato; mediana de dosis: 2 mg/kg y de tiempo de infusión: 1 hora. Se registró un caso de edema y exantema de cara vinculado a la rápida infusión. La evolución fue satisfactoria. Conclusiones: La administración de hierro iv fue segura. Es necesario establecer consensos respecto a la posología y monitorización. Se requieren nuevos estudios para continuar evaluando la eficacia y seguridad del hierro iv en sus diversas formulaciones.

Introduction: Intravenous (IV) iron administration it is indicated in cases where oral treatment is impossible. The objective of this work was to describe the profile of use, therapeutic response, and safety of the iron IV administration in treating anemia in children and adolescents (NNA) assisted in a reference center in Uruguay between 2018 and 2023. Method: Retrospective study through review of histories. It included all children and adolescents who received IV iron. Sociodemographic variables, comorbidities, clinical symptoms, and severity of anemia they were recorded. They were evaluated: reasons for the indication and type of IV iron, dose, infusion time, therapeutic response, and adverse effects. Results: we included 35 patients, with a median age of four years; 51.4% were male, and 37.1% had comorbidities. All children under three years of age had risk factors for iron deficiency anemia; greater severity of anemia was associated with lack of adherence to oral iron (p<0.05). The main reason for the indication of IV iron was the severity of anemia and inadequate response to concomitant oral iron at 37.1%. All received iron saccharate; median dose: 2 mg/kg and infusion time: 1 hour. A case of facial edema and rash linked to rapid infusion was recorded. The evolution was satisfactory. Conclusions: The administration of IV iron was safe. It is necessary to establish a consensus regarding dosage and monitoring. New studies are required to continue evaluating the efficacy and safety of IV iron in its various formulations.

Adipose derived stromal vascular fraction and fat graft for treating the hands of patients with systemic sclerosis. A randomized clinical trial.

BACKGROUND: Systemic Sclerosis in the hand is characteristically evidenced by Raynaud's phenomenon, fibrosis of the skin, tendons, ligaments, and joints as well as digital ulcers with prolonged healing. Current medical treatment does not always cure these complications. Local adipose-derived stromal vascular fraction administration into the hands has been proposed as an emerging treatment due to its regenerative properties. The objective of this randomized controlled clinical trial was to evaluate the safety and clinical effects of fat micrografts plus adipose derived-stromal vascular fraction administration into the hands of patients with systemic sclerosis. METHODS: This was an open-label, monocentric, randomized controlled study. Twenty patients diagnosed with systemic sclerosis were assigned to the experimental or control group. Fat micrografts plus the adipose derived-stromal vascular fraction were injected into the right hand of experimental group patients. The control group continued to receive only medical treatment. Demographic, serologic data and disease severity were recorded. Digital oximetry, pain, Raynaud phenomenon, digital ulcers number, mobility, thumb opposition, vascular density of the nail bed, skin affection of the hand, serologic antibodies, hand function, and quality of life scores were evaluated in both groups. RESULTS: The results of the intervention were analyzed with the Wilcoxon rank test, and the differences between the control and experimental groups at 0 days and 168 days were analyzed with the Mann-Whitney U test. Adverse events were not observed in both groups. At the end of the study, statistically significant improvements were observed in pain levels (p<0.05) and number of digital ulcers (p<0.01) in the experimental vs control group. CONCLUSION: The injection of adipose derived-stromal vascular fraction plus fat micrografts is a reproducible, and safe technique. Pain and digital ulcers in the hands of patients with systemic sclerosis can be treated with this technique plus conventional medical treatment.

Detection of NOTCH1 mutations in paraffin samples in patients with potentially malignant lesions.

Background: On certain occasions, oral cancer is preceded by potentially malignant lesions. The degree of dysplasia in Guinea pigs attempts to determine the risk of developing a malignant lesion. The search for genetic mutations, biomarkers, as a more truthful and reproducible diagnostic tool, tries to fill the gaps in the anatomopathological study. In this line, the present retrospective case-control study is based on the detection of known mutations of the NOTCH1 gene in biopsied samples of potentially malignant lesions from 22 patients who attend the Oral and Maxillofacial Surgery service of the Virgen del Rocío University Hospital. Material and Methods: DNA extraction after dewaxing of the samples using the Minikit QIAamp DNA FFPE tissue extraction kit with extraction kit (reference 56404) of QIAGEN. Subsequently, with the DNA obtained, 4 amplification reactions were carried out using enzyme polymerase. Before sequencing the samples, they were purified with the ExoSAP-IT for PCR product cleaning kit of the INVITROGEN brand. Finally, to detect somatic mutations in NOTCH1, TaqMan Mutation Detection Assays was used and for the analysis of mutations we worked with the Mutation Detector software. Results: The mutation for NOTCH1 is not detected, the studied sample does not present the mutation, or it is below the limits of detection of the software. Conclusions: In the clinical setting of the sample, the NOTCH1 mutation seems to be not very frequent, although NOTCH1 has been described as a gene related to oral cancer in other geographical settings. Key words:Oral cancer, NOTCH1, mutations.

Mutations in p53 Gene Exons in a Sample from the South of Spain in Oral Cancer.

BACKGROUND: Cancer is a genetic disease caused by mutations in DNA and epigenetic alterations that control gene expression. The majority of epidermoid carcinomas develop within the fields of epithelial genetic alterations. The mechanisms underlying tumorigenesis of epidermoid carcinoma are as yet unknown; therefore, precise identification of the risk factors is needed. Aim: The main aim of this study is to analyse and identify the emergence of the mutations described in the literature of the p53 gene with regard to the emergence of cancer in a sample of dysplastic and cancerous lesions in oral cavity mucosa in the population of the south of Spain, in order to determine the presence of said mutations and the percentage of them in our population. MATERIAL AND METHODS: A cross-sectional study was carried out, with a sample size of 22 patients with potentially malignant oral lesions ancillary to biopsy. All were patients, of both sexes, over 18 years of age from the Virgen del Rocío University Hospital with potentially malignant lesions in oral mucosa ancillary to biopsy (leukoplakias, erythroplasias or leukoerythopkias). An anatomopathological study was performed on all the samples and the lesions were divided into three types: low-grade dysplasia, high-grade dysplasia and squamous cell carcinoma. In respect of the genome study process, a complete search or scan for mutations in exons 5, 6, 8 and 9 of the p 53 gene was carried out, given that in the IARC database we observed that the 5 and 6 as well as the 8 and 9 exon sizes can be scanned completely in this way, since they have amplificon sizes of 476 and 445 base pairs respectively. RESULTS: In the scan for the complete exons 5, 6, 8 and 9 only a single result of interest was found to be described. In patient NBI 57 a change was observed in the TAT triplet by ATT of EXON 6, the change being of the T nucleotide by the A and in both directions both in Forward and Reverse. The exact location in the NCBI is GR Ch 37 p13 on chromosome 17, EXON 6 of the P53 gene and the change is in the C.613 T>A nucleotide; NM_000546. CONCLUSIONS: On reviewing this genetic variant in different scientific databases, such as ENSEMBL among others, in at least 6 different biocomputing tools it is described as a pathogen, therefore we can conclude that it is a pathogenic mutation for this case in particular. The rest of the mutations described in the literature on exons 5, 6, 8 and 9 of the p53 gene have not been found in our sample. Key words:Oral cancer, p53, Mutations, Exon.

Mesenchymal Stem Cells for the Compassionate Treatment of Severe Acute Respiratory Distress Syndrome Due to COVID 19.

Mesenchymal stem cells (MSC) have received particular attention due to their ability to inhibit inflammation caused by cytokine storm induced by COVID-19. In this way some patients have been treated successfully. The aim of this study was to evaluate the safety and describe the clinical changes after IV administration of allogeneic human umbilical cord MSC (ahUCMSC), in patients with bilateral pneumonia caused by COVID-19, complicated with severe ARDS, as compassionate treatment. This was a pilot, open-label, prospective, longitudinal study. Five patients that did not improve in their clinical conditions after 48 hours of receiving the standard medical management used by the Medical Center and with persistent PaO2/FiO2 less than 100 mmHg were enrolled. ahUCMSC were infused IV, at dose of 1x106 per Kg of body weight over 15 minutes. Patients were monitored after the infusion to detect adverse event. Pa02/FiO2, vital signs, D-dimer, C reactive protein and total lymphocytes were monitored for 21 days after the infusion or until the patient was discharged from the hospital. Descriptive statistics were used with means or medians and standard deviation or interquartile range according to the type of variable. The Wilcoxon's rank-sum was used for stationary samples. Adverse events occurred in three patients and were easily and quickly controlled. Immediately after the infusion of ahUCMSC, constant rise of PaO2/FiO2 was observed in all patients during the first 7 days, with statistical significance. Three patients survived and were extubated on the ninth day post-infusion. Two patients died at 13 and 15 days after infusion. The infusion of ahUCMSC in patients with severe ARDS caused by COVID-19, was safe, and demonstrated its anti-inflammatory capacity in the lungs, by improving the respiratory function expressed by PaO2 / FiO2, which allowed the survival of 3 patients, with extubation at 9 days.

Proteomic analysis and optimized production of Alkalihalobacillus patagoniensis PAT 05T extracellular proteases.

Extracellular proteolytic extracts from the haloalkalitolerant strain Alkalihalobacillus patagoniensis PAT 05T have proved highly efficient to reduce wool felting, as part of an ecofriendly treatment suitable for organic wool. In the present study, we identified the extracellular proteases produced by PAT 05T and we optimized its growth conditions for protease production through statistical methods. A total of 191 proteins were identified in PAT 05T culture supernatants through mass spectrometry analysis. Three of the 6 detected extracellular proteases belonged to the serine-endopeptidase family S8 (EC 3.4.21); two of them showed 86.3 and 67.9% identity with an alkaline protease from Bacillus alcalophilus and another one showed 50.4% identity with Bacillopeptidase F. The other 3 proteases exhibited 55.3, 49.4 and 61.1% identity with D-alanyl-D-alanine carboxypeptidase DacF, D-alanyl-D-alanine carboxypeptidase DacC and endopeptidase LytE, respectively. Using a Fractional Factorial Design followed by a Central Composite Design optimization, a twofold increase in protease production was reached. NaCl concentration was the most influential factor on protease production. The usefulness of PAT 05T extracellular proteolytic extracts to reduce wool felting was possible associated with the activity of the serine-endopeptidases closely related to highly alkaline keratinolytic proteases. The other identified proteases could cooperate, improving protein hydrolysis. This study provided valuable information for the exploitation of PAT 05T proteases which have potential for the valorization of organic wool as well as for other industrial applications.

Experiencia y manejo de dos pacientes pediátricos grandes quemados e infección por SARS-CoV-2 en una unidad de cuidados intensivos / Experience and management of two paediatric patients infected with SARS-CoV-2 with major burn injuries in an intensive care unit

RESUMEN La infección por SARS-CoV-2 se ha diseminado rápidamente, provocando una pandemia mundial en la cual nos vemos afectados. En el Instituto Nacional de Salud del Niño de San Borja, centro de referencia nacional de pacientes pediátricos quirúrgicos, desde el 14 de abril al 12 de agosto del 2020, se hospitalizaron 106 pacientes con infección por SARS-CoV-2, de los cuales 11 tuvieron diagnostico quemadura y dos fueron pacientes grandes quemados con diagnóstico COVID-19 por prueba molecular. Detallamos el caso de una paciente pediátrica, de siete años, gran quemada que fue atendida en unidad de cuidados intensivos, y con COVID-19 asintomática, presentó evolución y pronóstico favorable, con recuperación total de su lesión. Por otro lado, el COVID-19 se puede manifestar de forma severa produciendo un síndrome inflamatorio multisistémico que presenta manifestaciones clínicas poco comunes y que puede empeorar el pronóstico, como fue observado en nuestro otro paciente de siete meses que falleció pese a recibir manejo especializado y oportuno.

ABSTRACT SARS-CoV-2 infection is rapidly disseminating, leading to a world pandemics in which we are also affected. In San Borja Peruvian National Children Health Institute, which is the reference center for surgical pediatric patients, 106 patients infected with SARS-CoV-2 were hospitalized. Of them, 11 presented with burns and two had large burns. The viral infection was diagnosed using molecular testing. We present in detail the case of a pediatric seven-year old patient who had large burns and was taken care of in the intensive care unit. She had asymptomatic SARS-CoV-2 infection, and her outcome was good, with a good prognosis, and she recovered completely and uneventfully. On the other hand, COVID-19 may present as a severe disease leading to a multi- systemic inflammatory syndrome with some uncommon clinical manifestations which affect its prognosis , as it was the case with another seven-month old patient who passed away in spite of having received specialized and timely therapy.

Bilateral Proximal Forearm Transplantation: Case Report at 7 Years.

BACKGROUND: Although return of function has been reported in patients undergoing proximal forearm transplantations (PFTs), reports of long-term function are limited. In this study, we evaluated the clinical progress and function 7 years postoperatively in a patient who underwent bilateral PFT. CASE PRESENTATION: A 58-year-old man underwent bilateral PFT in May 2012. Transplantation involved all of the flexor and extensor muscles of the forearm. Neurorrhaphies of the median, ulnar, and radial nerves were epineural and 7 cm proximal to the elbow. Immunosuppressive maintenance medications during the first 3 years postoperatively were tacrolimus, mycophenolate, and steroids, and later, tacrolimus, sirolimus, and steroids. Forearm function was evaluated annually using the Disabilities of the Arm, Shoulder, and Hand; Carroll; Hand Transplantation Score System; Short Form-36; and Kapandji scales. We also evaluated his grip and pinch force. RESULTS: Postoperatively, the patient developed hypertriglyceridemia and systemic hypertension. He experienced 6 acute rejections, and none were resistant to steroids. Motor function findings in his right/left hand were: grip strength: 10/13 kg; key pinch: 3/3 kg; Kapandji score: 6/9 of 10; Carroll score: 66/80; Hand Transplantation Score System score: 90/94. His preoperative Disabilities of the Arm, Shoulder, and Hand score was 50 versus 18, postoperatively; his Short Form-36 score was 90. This function improved in relation with the function reported in the second year. CONCLUSIONS: Seven years following PFT, the patient gained limb strength with a functional elbow and wrist, although with diminished digital dexterity and sensation. Based on data presented by other programs and our own experience, PFT is indicated for select patients.

Blefaritis crónica grave. Evolución tórpida de varios años / Severe chronic blepharitis. Torpid evolution of several years

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Clinical Significance of Alloantibodies in Hand Transplantation: A Multicenter Study.

BACKGROUND: Donor-specific antibodies (DSAs) have a strong negative correlation with long-term survival in solid organ transplantation. Although the clinical significance of DSA and antibody-mediated rejection (AMR) in upper extremity transplantation (UET) remains to be established, a growing number of single-center reports indicate their presence and potential clinical impact. METHODS: We present a multicenter study assessing the occurrence and significance of alloantibodies in UET in reference to immunological parameters and functional outcome. RESULTS: Our study revealed a high prevalence and early development of de novo DSA and non-DSA (43%, the majority detected within the first 3 postoperative y). HLA class II mismatch correlated with antibody development, which in turn significantly correlated with the incidence of acute cellular rejection. Cellular rejections preceded antibody development in almost all cases. A strong correlation between DSA and graft survival or function cannot be statistically established at this early stage but a correlation with a lesser outcome seems to emerge. CONCLUSIONS: While the phenotype and true clinical effect of AMR remain to be better defined, the high prevalence of DSA and the correlation with acute rejection highlight the need for optimizing immunosuppression, close monitoring, and the relevance of an HLA class II match in UET recipients.

Advantages of surgical simulation in the surgical reconstruction of oncological patients

BACKGROUND: Stereolithography, which consists of computer-aided designed/computer-aided manufactured (CAD-CAM) and computer simulations, is a manufacturing technologies used for the production of definitive models and prototypes printed in three dimensions, and is widely used in Oral and Maxillofacial Surgery. Surgical procedures using models made by these technologies offer several advantages. MATERIALS AND METHODS: This article describes three clinical cases of our experiences with patients diagnosed with squamous cell carcinoma and mandibular osteosarcoma, who underwent surgical removal of the lesions and subsequent mandibular reconstruction with a free fibula graft using surgical guides. RESULTS: In all three clinical cases, surgical guides were used for the mandibular osteotomy, fibula osteotomy, and graft placement in the recipient area. DISCUSSION: Surgical guidelines are useful for improving the accuracy of surgical interventions and are appropriate for many types of resection and mandibular reconstruction

Advantages of surgical simulation in the surgical reconstruction of oncological patients.

BACKGROUND: Stereolithography, which consists of computer-aided designed/computer-aided manufactured (CAD-CAM) and computer simulations, is a manufacturing technologies used for the production of definitive models and prototypes printed in three dimensions, and is widely used in Oral and Maxillofacial Surgery. Surgical procedures using models made by these technologies offer several advantages. MATERIALS AND METHODS: This article describes three clinical cases of our experiences with patients diagnosed with squamous cell carcinoma and mandibular osteosarcoma, who underwent surgical removal of the lesions and subsequent mandibular reconstruction with a free fibula graft using surgical guides. RESULTS: In all three clinical cases, surgical guides were used for the mandibular osteotomy, fibula osteotomy, and graft placement in the recipient area. DISCUSSION: Surgical guidelines are useful for improving the accuracy of surgical interventions and are appropriate for many types of resection and mandibular reconstruction.

Decoupling between the hand territory and the default mode network after bilateral arm transplantation: four-year follow-up case study.

Several studies have suggested both a local and network reorganization of the sensorimotor system following amputation. Transplantation of a new limb results in a new shifting of cortical activity in the local territory of the transplanted limb. However, there is a lack of information about the reversibility of the abnormalities at the network level. The objective of this study was to characterize the functional connectivity changes between the cortical territory of the new hand and two intrinsic network of interest: the sensorimotor network (SMN) and the default mode network (DMN) of one patient whom received bilateral forearm transplants. Using resting-state fMRI these two networks were identified across four different time points, starting four months after the transplantation surgery and during three consecutive years while the patient underwent physical rehabilitation. The topology of the SMN was disrupted at the first acquisition and over the years returned to its canonical pattern. Analysis of the DMN showed the normal topology with no significant changes across acquisitions. Functional connectivity between the missing hand's cortical territory and the SMN increased over time. Accordingly, functional connectivity between the missing hand's cortical territory and the DMN became anticorrelated over time. Our results suggest that after transplantation a new reorganization occurs at the network level, supporting the idea that extreme behavioral changes can affect not only the local rewiring but also the intrinsic network organization in neurologically healthy subjects. Overall this study provides new insight on the complex dynamics of brain organization.

Queratitis por Nocardia farcinica en paciente inmunocompetente. Descripción del primer caso en España / Keratitis by Nocardia farcinica in immunocompetent patient. Description of the first case in Spain

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