Treatment of a Severe Pediatric Lyell Syndrome with Amniotic Membrane: Case Report and Histological Findings

Background: Lyell Syndrome (TEN, Toxic epidermal necrolysis) represents a medical emergency particularly in pediatric patients in whom the massive skin damage can quickly lead to multi-organ dysfunction and death. Prompt restoration of the physiologic mucosal/cutaneous barrier is mandatory. The use of amniotic membranes has been described in the treatment of ophthalmic Lyell Syndrome, but its use has not yet been adopted for the management of larger cutaneous wounds. Study Hypothesis: Here we report the use of amniotic membranes in a pediatric case of severe Lyell Syndrome with complete skin surface, ocular and mucosal involvement with life threating presentation. Methods: A 7-year old female was admitted to our Burn Centre for severe cutaneous/mucosal exfoliation (100% Total body surface area, TBSA) as a result of an adverse reaction to ibuprofen administration. Supportive fluid administration, cardiac-pulmonary assistance and pain management were complemented by serial grafting of amniotic membranes on all affected areas to provide coverage of the exfoliated skin/mucosa. Biopsies were obtained to monitor histological skin changes. Results: The patient showed an excellent response to amniotic membrane treatment, with rapid restoration of mucosal and cutaneous layers in the grafted areas. This resulted in a decreased need for dressing changes, avoidance of additional surgeries and a reduced dependence on supportive therapy. Lower pain levels than usually expected led to a reduced need for narcotic pain medications and allowed for early physical rehabilitation and a short hospital stay. Histology confirmed evidence of topical immune-modulation in treated areas (reduction of inflammatory infiltrate). Conclusion: As we tested in numerously TEN and burn pediatric injuries Amniotic membranes with their regenerative and immune-modulatory effects may represent an life saving treatment even in the worst cases of pediatric Lyell syndrome

Bone health and body composition in transgender adults before gender-affirming hormonal therapy: data from the COMET study

Purpose: Preliminary data suggested that bone mineral density (BMD) in transgender adults before initiating gender-affirming hormone therapy (GAHT) is lower when compared to cisgender controls. In this study, we analyzed bone metabolism in a sample of transgender adults before GAHT, and its possible correlation with biochemical profile, body composition and lifestyle habits (i.e., tobacco smoke and physical activity). Methods: Medical data, smoking habits, phospho-calcic and hormonal blood tests and densitometric parameters were collected in a sample of 125 transgender adults, 78 Assigned Females At Birth (AFAB) and 47 Assigned Males At Birth (AMAB) before GAHT initiation and 146 cisgender controls (57 females and 89 males) matched by sex assigned at birth and age. 55 transgender and 46 cisgender controls also underwent a complete body composition evaluation and assessment of physical activity using the International Physical Activity Questionnaire (IPAQ). Results: 14.3% of transgender and 6.2% of cisgender sample, respectively, had z-score values < -2 (p = 0.04). We observed only lower vitamin D values in transgender sample regarding biochemical/hormonal profile. AFAB transgender people had more total fat mass, while AMAB transgender individuals had reduced total lean mass as compared to cisgender people (53.94 ± 7.74 vs 58.38 ± 6.91, p < 0.05). AFAB transgender adults were more likely to be active smokers and tend to spend more time indoor. Fat Mass Index (FMI) was correlated with lumbar and femur BMD both in transgender individuals, while no correlations were found between lean mass parameters and BMD in AMAB transgender people. Conclusions: Body composition and lifestyle factors could contribute to low BMD in transgender adults before GAHT

Reduced auditory steady state responses in autism spectrum disorder

Background Auditory steady state responses (ASSRs) are elicited by clicktrains or amplitude-modulated tones, which entrain auditory cortex at their specific modulation rate. Previous research has reported reductions in ASSRs at 40 Hz for autism spectrum disorder (ASD) participants and first-degree relatives of people diagnosed with ASD (Mol Autism. 2011;2:11, Biol Psychiatry. 2007;62:192–197). Methods Using a 1.5 s-long auditory clicktrain stimulus, designed to elicit an ASSR at 40 Hz, this study attempted to replicate and extend these findings. Magnetencephalography (MEG) data were collected from 18 adolescent ASD participants and 18 typically developing controls. Results The ASSR localised to bilateral primary auditory regions. Regions of interest were thus defined in left and right primary auditory cortex (A1). While the transient gamma-band response (tGBR) from 0-0.1 s following presentation of the clicktrain stimulus was not different between groups, for either left or right A1, the ASD group had reduced oscillatory power at 40 Hz from 0.5 to 1.5 s post-stimulus onset, for both left and right A1. Additionally, the ASD group had reduced inter-trial coherence (phase consistency over trials) at 40 Hz from 0.64-0.82 s for right A1 and 1.04-1.22 s for left A1. Limitations In this study, we did not conduct a clinical autism assessment (e.g. the ADOS), and therefore, it remains unclear whether ASSR power and/or ITC are associated with the clinical symptoms of ASD. Conclusion Overall, our results support a specific reduction in ASSR oscillatory power and inter-trial coherence in ASD, rather than a generalised deficit in gamma-band responses. We argue that this could reflect a developmentally relevant reduction in non-linear neural processing

Fascia lata allografts as biological mesh in abdominal wall repair: preliminary outcomes from a retrospective case series.

BACKGROUND:The use of biological meshes in management of infected abdominal hernias or in abdominal fields at high risk of infection (potentially contaminated or with relevant comorbidities) is well established. Available products include xenogenic patches or decellularized dermal allografts. Despite their biomechanical features, banked fascial allografts have not been investigated yet in this setting. The authors evaluated the safety and effectiveness of banked fascia lata allografts as biological meshes in abdominal wall repair. METHODS: A consecutive series of patients affected by abdominal wall defects and who were candidates for repair by means of a biological mesh and treated in the authors' institution with banked fascia lata allografts were reviewed retrospectively. Data from clinical and instrumental follow-up evaluations up to 48 months (average, 23 months) were analyzed. RESULTS: Twenty-one patients (aged 1 to 86 years) with abdominal wall defects resulting from traumatic (n = 1), neoplastic (n = 6), or multiple previous laparotomies (n = 14) were treated from January of 2008 to October of 2012. Operations had no relevant postoperative complications. At clinical/instrumental follow-up examinations, no major signs of recurrence, laxity, infection of grafts, or other related pathologic symptoms were recorded. Three patients suffered from temporary minor complications (e.g., wound seroma, partial cutaneous dehiscence). At instrumental (computed tomographic scan or magnetic resonance imaging) evaluations, the neofascial tissue appeared stable until medium-term follow-up (3 to 6 months), later being gradually degraded and apparently replaced by host tissue. CONCLUSION: According to limited preliminary outcomes, banked fascia lata allografts seem to provide a biocompatible, safe, and effective alternative to other biological meshes. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV

Current Surgical Therapy of Locally Advanced cSCC: From Patient Selection to Microsurgical Tissue Transplant. Review

Among the non-melanoma skin cancers (NMSC) the squamous cell carcinoma (SCC) is one of the most challenging for the surgeon. Local aggressiveness and a tendency to metastasize to regional lymph nodes characterize the biologic behavior. The variants locally advanced and metastatic require wide excision and node dissection. Such procedures can be extremely detrimental for patients. The limit of the surgery can be safely pushed forward with a multidisciplinary approach. The concept of skin oncoplastic surgery, the ablative procedures and the reconstructive options (skin graft, pedicled flap, microsurgical free flap) are discussed together with a literature review

BH3-Promoted Stereoselective b-Lithiation of N-Alkyl-2-phenylaziridines

BH3 complexes of N-alkyl-2-phenylaziridines have been synthesized and their structure and stereochemistry proved with DFT calculations and NMR experiments. It has been demonstrated that the Lewis acid complexation is able to promote a regioselective β-lithiation in 2-phenylaziridino-borane complexes. The lithiated intermediates were configurationally stable, allowing an enantioselective preparation of cis- 2,3-disubstituted aziridines

Multidisciplinary care in severe pediatric electrical oral burn.

Oral burns in pediatric patient are commonly due to electrical injuries, representing an important reconstructive issue even for functional than esthetic reason. Different classification, surgical management and even oral device were described to allow the best long-term result. In most case a multidisciplinary approach is necessary to achieve a satisfactory outcome. A severe case of pediatric oral burn with germinative teeth damage is presented, describing a multispecialist team approach that guarantee a satisfactory outcome by reconstructive surgery, careful progressive evaluation of dental and soft tissue healing and speech recovery. The use of acellular dermal substitute template within traditional reconstructive surgery had provided a good functional and esthetic result joint to valid preservation of germinative dental element as shown at long-term X-ray evaluation. Intensive rehabilitation speech program has also avoided phonetic impairment in an important speech develop period. It was so evident that the necessity of a multispecialist care in such difficult injury to achieve the best long-term result