Comparison of the effectiveness of three different skin substitutes for the treatment of pediatric burns.

Unlabelled: Early debridement of partial-thickness burns and coverage with skin substitutes is currently the standard of care in children, although there is currently no "gold standard" skin substitute. Our aim is to compare the effectiveness of three different skin substitutes, analyzing the medium- and long-term outcomes.

Methods: A retrospective study was conducted on burn patients under 18 years admitted to our Burn Unit between 2015 and 2021, who were divided into 3 groups according to the type of skin substitute used (EZ-derm®, Biobrane®, and Suprathel®).

Modulation of the K/BxN arthritis mouse model and the effector functions of human fibroblast-like synoviocytes by liver X receptors.

The role of liver X receptors (LXR) in rheumatoid arthritis (RA) remains controversial. We studied the effect of LXR agonists on fibroblast-like synoviocytes (FLS) from RA patients and the K/BxN arthritis model in LXRα and β double-deficient (Nr1h2/3) mice. Two synthetic LXR agonists, GW3965 and T0901317, were used to activate LXRs and investigate their effects on cell growth, proliferation and matrix metalloproteinases, and chemokine production in cultured FLS from RA patients.

Acellular flowable dermal matrix for ostomy reconstruction: a safe and effective minimally invasive technique.

Background: Acellular dermal matrices have long been used for complex abdominal wall closure and, more recently, for ostomy reconstruction.

Purpose: To describe ostomy reconstruction with acellular flowable dermal matrix (AFDM) in a pediatric patient with a complex abdominal wall defect.

Case Report: A 14-year-old female who was diagnosed with unspecific hemorrhagic colitis at age 6 months and who underwent several abdominal surgeries (including total colectomy and terminal ileostomy) reported frequent, severe periostomy skin ulcerations secondary to improper fitting and ostomy bag leakage due to multiple periostomy skin retraction as a result of multiple abdominal scars.

Sedoanalgesia in the Debridement of Pediatric Burns in the Emergency Department: Is It Effective and Safe?

Background: The routine use of sedoanalgesia has increased the number of potential minor surgical procedures that can be performed in the Emergency Department (ED) without requiring general anesthesia and, thus, hospital admission. Our aim is to analyze the effectiveness and safety of the use of sedoanalgesia in childhood burns treated in the ED.

Methods: A retrospective study was conducted in burned children in whom burn debridement was performed under sedoanalgesia in the ED between 2017 and 2021 in a tertiary referral center for burns.

Mortality predictive factors in congenital hepatic hemangioma: a case-control study.

Unlabelled: Congenital hepatic hemangiomas (CHHs) are benign vascular tumors whose clinical, histological, and genetic correlation has recently been described in patients with long-term survival, although no mortality risk factors have been identified to date. The aim of this study is to analyze predictors of mortality in patients with CHH. A retrospective single-center case-control study of consecutive CHH patients diagnosed in our institution between 1991 and 2021 was performed, who were classified into two groups according to their survival.

Pediatric Burns Debridement Under Sedoanalgesia During the COVID-19 Pandemic: A Cost-Effective Alternative.

Early debridement of childhood burns under sedoanalgesia in the emergency department (ED) may help to reduce the need of surgery and delay in treatment. We performed a retrospective study in burned children who underwent debridement in the ED under sedoanalgesia between March 2020 and December 2020 (COVID group), and were compared with the same months in 2019 (control group). Demographics, burns features, admission rate, and estimated costs associated were collected.

The Use of Acellular Dermal Matrix (Integra Single Layer) for the Correction of Malformative Chest Wall Deformities: First Case Series Reported.

 Autologous tissue transfers have been used in chest wall reconstruction for decades, with high morbidity. Recently, acellular dermal matrices (ADMs) have emerged as an alternative. The aim of this article is to report our initial experience in the reconstruction of malformative chest wall deformities with ADM.

Clinical Factors in Trunk Capillary Malformations in the Neonate: When to Suspect Other Associated Malformations? A Case-Control Study.

Introduction:  Capillary malformations (CMs) can be sporadic or syndromic, in association with other underlying venous malformation (VM) or lymphatic malformation (LM). The objective of this study is to describe the clinical patterns in the neonate that allow us to differentiate sporadic CMs from those associated with other vascular malformations.

Materials And Methods:  A case-control study was performed in neonates with CM located in the trunk, followed at our institution between 2008 and 2018.

Alpelisib Treatment for Genital Vascular Malformation in a Patient with Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, and Spinal/Skeletal Anomalies and/or Scoliosis (CLOVES) Syndrome.

Background: Most patients with phosphoinositide-3-kinase, catalytic, alpha polypeptide (PIK3CA)-related overgrowth spectrum become symptomatic early in life and need treatment before puberty. Recently, the specific inhibition of PIK3CA pathways has been proposed as a therapeutic option for these patients improving their surgical options and quality of life. Alpelisib, a specific alpha fraction inhibitor, has shown promising results.

Oral Sirolimus: An Option in the Management of Neonates with Life-Threatening Upper Airway Lymphatic Malformations.

Mechanistic target of rapamycin (mTOR) inhibitors are being used off-label showing promising results in patients with vascular anomalies. Children with lymphatic malformations (LMs) involving the airway benefit from sirolimus therapy soon after birth, reducing the need of tracheostomy. Available information about efficacy and side effects in neonates remains poor.

Liver X Receptor Nuclear Receptors Are Transcriptional Regulators of Dendritic Cell Chemotaxis.

The liver X receptors (LXRs) are ligand-activated nuclear receptors with established roles in the maintenance of lipid homeostasis in multiple tissues. LXRs exert additional biological functions as negative regulators of inflammation, particularly in macrophages. However, the transcriptional responses controlled by LXRs in other myeloid cells, such as dendritic cells (DCs), are still poorly understood.

Using Tissue Expanders as a Choice for the Treatment of Congenital Facial Nevus in the Young Child.

Objective:  One of the principal objectives in treatment of facial nevus is to minimize psychological damage and encourage the child's schooling by the best possible cosmetic result. There are several therapeutic techniques: grafts, flaps, dermal regenerator, and tissue expanders.

Materials And Methods:  We reviewed 10 patients with facial nevus higher than 10 cm treated in the past 8 years.

Pancreatic Kaposiform Hemangioendothelioma Not Responding to Sirolimus.

 Kaposiform hemangioendothelioma (KHE) is a vascular tumor frequently associated with Kasabach-Merritt phenomenon (KMP), characterized by severe thrombocytopenia and consumptive coagulopathy. Visceral involvement in KHE is rare. In our recent experience, sirolimus has shown to be an effective treatment in cutaneous KHE, becoming indeed the treatment of choice in KMP.

Cholesterol Accumulation in CD11c Immune Cells Is a Causal and Targetable Factor in Autoimmune Disease.

Liver X receptors (LXRs) are regulators of cholesterol metabolism that also modulate immune responses. Inactivation of LXR α and β in mice leads to autoimmunity; however, how the regulation of cholesterol metabolism contributes to autoimmunity is unclear. Here we found that cholesterol loading of CD11c cells triggered the development of autoimmunity, whereas preventing excess lipid accumulation by promoting cholesterol efflux was therapeutic.

The nuclear receptor LXRα controls the functional specialization of splenic macrophages.

Macrophages are professional phagocytic cells that orchestrate innate immune responses and have considerable phenotypic diversity at different anatomical locations. However, the mechanisms that control the heterogeneity of tissue macrophages are not well characterized. Here we found that the nuclear receptor LXRα was essential for the differentiation of macrophages in the marginal zone (MZ) of the spleen.

Osteolysis and lymphatic anomalies: a review of 54 consecutive cases.

Background: Progressive osteolysis caused by lympathic malformations is a rare condition that should be known by specialists involved in the study of lymphatic disorders because they are necessarily involved in the treatment. The purpose of the present study is to report on a large series of patients to illustrate the multiple clinical pictures and the wide range of therapeutic measures necessary for arresting bone destruction and lymphatic leak.

Methods And Results: Inclusion criteria were osteolysis associated with lymphatic malformation that required treatment.

Oxysterol-induced soluble endoglin release and its involvement in hypertension.

Background: Ischemia in the placenta is considered the base of the pathogenesis of preeclampsia, a pregnancy-specific syndrome in which soluble endoglin (sEng) is a prognostic marker and plays a pathogenic role. Here, we investigated the effects of hypoxia and the downstream pathways in the release of sEng.

Methods And Results: Under hypoxic conditions, the trophoblast-like cell line JAR showed an increase in sEng parallel to an elevated formation of reactive oxygen species.

[Indication for early excision of the giant congenital nevus].

Introduction: Most of specialists involved in giant congenital nevi (GCN) care, agree that the risk of melanoma is probably not significant enough to warrant the prophylactic removal of all those lesions. However, many patients benefit from very early surgical procedures.

Material And Methods: In the last 15 years 41 children undergwent surgical removal of their large or giant melanocityc lesions in the first year of life at our institution.

Apoptotic cells promote their own clearance and immune tolerance through activation of the nuclear receptor LXR.

Effective clearance of apoptotic cells by macrophages is essential for immune homeostasis. The transcriptional pathways that allow macrophages to sense and respond to apoptotic cells are poorly defined. We found that liver X receptor (LXR) signaling was important for both apoptotic cell clearance and the maintenance of immune tolerance.

[Sensitization to three species of mites in allergic patients from the coastal area of Havana city].

Background: Sensitization to three allergic mite species (Dermatophagoides pteronyssinus, Dermatophagoides siboney and Blomia tropicalis) from house dust in Cuba has been reported in different studies.

Objective: To assess allergic sensitization to these mites in a north coast area of Havana city.

Patients And Methods: A total of 210 allergic patients were selected, classified in two groups: from 1 to 14 years, and from 15 to 55 years.

Giant rapidly involuting congenital hemangioma of the face: 15-year follow-up.

Rapidly involuting congenital hemangiomas are rare variants of congenital hemangiomas. They are different from the more common infantile hemangiomas in that their presentation, clinical course, and histopathologic features are dissimilar. We report a case of a giant rapidly involuting congenital hemangioma of the face, with a 15-year follow-up and a review of this rare entity.