Mesenchymal stromal cells (MSCs) have been proposed as a new therapeutic strategy to treat congenital and acquired respiratory system diseases. We describe a case report of an 18-month-old male patient with progressive chronic respiratory failure, associated with mutations of the surfactant protein C gene (SFTPC) due to c.289G > T variant p.Gly97Ser (rs927644577) and c.176A > G variant (p.His59Arg), submitted to repeated intravenous infusions of allogeneic bone marrow (BM) MSCs. The clinical condition of the patient was monitored. Immunologic studies before and during MSC treatment were performed. No adverse events related to the MSC infusions were recorded. Throughout the MSC treatment period, the patient showed a growth recovery. Starting the second infusion, the patient experienced an improvement in his respiratory condition, with progressive adaptation to mechanical ventilation. After the third infusion, five hours/die of spontaneous breathing was shown, and after infusion IV, spontaneous ventilation for 24/24 h was recorded. A gradual decrease of lymphocytes and cell subpopulations was observed. No variations in the in vitro T cell response to PHA were determined by MSC treatment as well as for the in vitro B cell response. A decrease in IFN-γ, TNF-α, and IL-10 levels was also detected. Even though we cannot exclude an improvement of pulmonary function due to the physiological maturation, the well-known action of MSCs in the repair of lung tissue, together with the sequence of events observed in our patient, may support the therapeutic role of MSCs in this clinical condition. However, further investigations are necessary to confirm the result and long-term follow-up will be mandatory to confirm the benefits on the pulmonary condition.
Pediatric burns represent a significant public health problem. We analyzed the characteristics of pediatric burns in a reference center, in order to identify better strategies for prevention and care. Burn patients admitted to the pediatric departments of our hospital from January 2020 to June 2022 were retrospectively evaluated. Age, gender, the etiology of injuries, the total burn surface area (TBSA), the degree of burns, the length of hospital stay (LOS), concomitant SARS-CoV-2 infection, and burn surface microbial colonization were analyzed. Forty-seven patients were included in the analysis (M:F = 1:0.67). Most of the cases involved patients between 0 and 4 years of age (83%). Hot liquid burns accounted for 79% of cases, flame burns for 9%, thermal burns for 6%, scald burns for 4% and chemical burns for 2%. Mean TBSA was 14 ± 11%. A second-degree lesion was detected in 79% of patients and third-degree in 21%. Mean LOS was 17 days. No additional infection risks or major sequelae were reported in patients with SARS-CoV-2 infection. Fifteen different species of bacteria plus C. parapsilosis were isolated, while no anaerobic microorganisms were detected. In the light of our experience, we recommend a carefully planned and proactive management strategy, always multidisciplinary, to ensure the best care for the burned child.
Thyroid function plays a crucial role in nervous system integrity and metabolic homeostasis. We evaluated the pattern of TSH, FT4 and FT3 release in children with neuromotor impairment (NI) in relationship with metabolic syndrome (MS). We enrolled 55 patients with NI and 30 controls. Clinical parameters, thyroid function and MS presence were recorded. Principal component analysis (PCA), cluster analysis, and logistic regression models were performed. MS was detected in 54.5% of patients. Four clusters were identified: the first one included only controls and, contrasting with cluster 4, was exclusively characterized by children with disability and MS. This latter showed increased FT4 and FT3 and decreased TSH levels. Cluster 2, characterized by disability without MS showed high FT4 and FT3, whereas cluster 3 with low FT4 and FT3 mainly included disability (90%) and showed prevalent MS (57%). The association between TSH and NI is represented by a U-shape structure. The TSH, FT3 and FT4 release patterns may reflect thyrotropic adaptation, allostatic response and compensatory mechanisms. These mechanisms, found in both MS and disability, show that the odds of having a condition of NI with or without MS increase as the TSH values deviate, in both directions, from a value of 2.5 mLU/mL.
Esophageal atresia (EA) is the most common congenital esophageal malformation. An improvement in survival led to a focus on functional outcomes and quality of life (QoL). We analyzed the long-term outcomes and QoL of patients submitted to surgery for EA. Perinatal characteristics, surgical procedures, gastrointestinal and respiratory current symptoms and QoL were investigated. Thirty-nine patients were included. Long Gap patients had a higher rate of prematurity and low birth weight. The prevalent surgical procedure was primary esophageal anastomosis, followed by gastric pull-up. Twenty-four patients had post-operative stenosis, while gastroesophageal reflux (GER) required fundoplication in eleven cases. Auxological parameters were lower in Long Gap patients. The lowest scores of QoL were in the Long Gap group, especially in younger patients, which was the group with the highest number of symptoms. In the long term, the QoL appeared to be more dependent on the type of esophageal atresia rather than on associated malformations. Surgical management of GER was indicated in all patients with Long Gap EA, supposedly due to the prevalence of gastric pull-up for this type of EA. The assessment of QoL is part of surgeon's management and needs to be performed in each phase of a child's development.
Video assisted thoracoscopic surgery (VATS) has been adopted in pediatric age for the treatment of congenital lung malformations (CLM). The success of VATS in pediatrics largely depends on the surgeon's skill ability to understand the airways, vascular system and lung parenchyma anatomy in CLM. In the last years, virtual reality (VR) and 3-dimensional (3D) printing of organ models and VR head mounted display (HMD) technologies have been introduced for completion of preoperative planning in adult patients. To date no reports about the use of VR HMD technologies in a pediatric setting are available. The aim of this report is to introduce a VR HMD model in VATS procedure to improve the quality of care in children with CLM. VR HMD set-up for planning thoracoscopic surgery was performed in a series of pediatric patients with diagnosis of CLM. The preoperative VR HMD evaluation allowed a navigation into the malformation with the aim to explore, interact, and make the surgeon more confident and skilled to answer to the traps. A development of surgical simulations models and teaching program dedicated to education and training in pediatric VATS is suitable among the pediatric surgery community. Further studies should demonstrate all the benefits of such technology in pediatric patients submitted to VATS procedure.
VATS (video assisted thoracoscopic surgery) is routinely and successfully performed in minor and major complex thoracic procedures. This technique has been recently introduced for the treatment of severe forms of idiopathic scoliosis (IS) with the aim to repair the deformity, reduce morbidity and to prevent its progression in patients with skeletal immaturity. This study aims to present VATS in anterior vertebral body tethering (AVBT) approach to support the pediatric orthopedic surgeons during vertebral body fixation. Surgical and anesthesiologic tips and tricks are reported to assure a safe procedure. The study includes preadolescents with IS and a grade of scoliosis >40° that had a high probability of deterioration due to remaining growth (December 2018 to April 2021). Skeletal immaturity of enrolled patients was assessed by Sanders classification and Risser sign. Patients had a Risser score between 0 and 1 and a Sanders score >2 and <5. AVBT technique using VATS was performed by a senior pediatric surgeon assisting the pediatric orthopedic surgeon. Twenty-three patients have been submitted to VATS AVBT in the period of study (age range 9-14 years). The patients had a classified deformity Lenke 1A or B convex right and all types of curves were treated. In all patients, the vertebrae submitted to tethering surgery ranged from D5 to D12; mean curve correction was 43%. Three postoperative complications occurred: one late postoperative bleeding requiring a chest tube positioning on 12th postoperative day; one screw dislodged and needed to be removed; one child showed worsening of the scoliosis and needed a posterior arthrodesis. Initial results of VATS AVBT in growing patients with spinal deformities are encouraging. An appropriate selection of patients and a pediatric dedicated multidisciplinary surgical approach decrease intraoperative complications, time of operation and postoperative sequelae and guarantee an optimal outcome.
