The Importance of First Aid to Burned Patients: 30 Years of Experience at the Burns Centre in Pisa.
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ABSTRACT:
SUBMITTER: Gianfaldoni R
PROVIDER: S-EPMC5535647 | biostudies-other | 2017 Jul
REPOSITORIES: biostudies-other
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Project description:BackgroundGuidance for the management of thermal injuries has evolved with improved understanding of burn pathophysiology. Guidance for the management of cold burn injuries is not widely available. The management of these burns differs from the standard management of thermal injuries. This study aimed to review the etiology and management of all cold burns presenting to a large regional burn centre in the UK and to provide a simplified management pathway for cold burns.MethodsAn 11-year retrospective analysis (1 January 2003-31 December 2014) of all cold injuries presenting to a regional burns centre in the UK was conducted. Patient case notes were reviewed for injury mechanism, first aid administered, treatment outcomes and time to healing. An anonymized nationwide survey on aspects of management of cold burns was disseminated between 13 July 2015-5 October 2015 to British Association of Plastic Reconstructive and Aesthetic Surgeons (BAPRAS) and Plastic Surgery Trainees Association (PLASTA) members in the UK. Electronic searches of MEDLINE, EMBASE and the Cochrane Library were performed to identify relevant literature to provide evidence for a management pathway for cold burn injuries.ResultsTwenty-three patients were identified. Age range was 8 months-69 years. Total body surface area (TBSA) burn ranged from 0.25 to 5 %. Twenty cases involved peripheral limbs. Seventeen (73.9 %)cases were accidental, with the remaining six (26.1 %) cases being deliberate self-inflicted injuries. Only eight patients received first aid. All except one patient were managed conservatively. One case required skin graft application due to delayed healing. We received 52 responses from a total of 200 questionaires. Ninety percent of responders think clearer guidelines should exist. We present a simplified management pathway based on evidence identified in our literature search.ConclusionsCold burns are uncommon in comparison to other types of burn injuries. In the UK, a disproportionate number of cold burn injuries are deliberately self-inflicted, especially in the younger patient population. Our findings reflect a gap in clinical knowledge and experience. We proposed a simplified management pathway for managing cold burn injuries, consisting of adequate first aid using warm water, oral prostaglandin inhibitors, deroofing of blisters and topical antithromboxane therapy.
| S-EPMC5105282 | biostudies-literature
Project description:BackgroundIn the event of a sudden illness or injury, elderly individuals are often dependent on self-help and mutual assistance from partners. With poor access to medical services during natural and other disasters, the importance of first aid knowledge of elderly individuals increases even more. We assessed the opinions of different generations of Slovenian population regarding the importance of knowing the basic first aid measures. In addition, we aimed to examine the knowledge of first aid in the most common emergencies that threaten elderly people's health and lives, focusing on the knowledge of elderly.MethodsA structured questionnaire was conducted with a representative Slovenian adult population (n = 1079). Statistically significant differences in average ratings of the importance of first aid knowledge were compared among different age groups with one-way ANOVA followed by a post hoc test. Significant differences in percentages of correct answers in particular cases of health conditions between different age groups were determined using the χ 2 test followed by post hoc tests.ResultsSlovenes are well aware of the importance of first aid knowledge and feel personally responsible for acquiring this knowledge. The general opinion is that older retirees need less first aid knowledge than individuals in younger populations. We found a high level of knowledge about symptoms and first aid measures for some of the most common health conditions that occur in old age. The level of knowledge in the group of the oldest respondents was comparable with that of younger age groups. However, their recognition of health conditions was also somewhat worse, especially when recognising the symptoms and signs of hypoglycaemia and heart attack. Most of the tested knowledge did not depend on a person's age but on the time since that person was last educated in first aid.ConclusionsThe knowledge of people older than 80 years is somewhat poorer than that in the younger population, mainly because too much time has passed since they were last educated in first aid. Public awareness of first aid needs to be increased and appropriate guidelines should be given with a focus on the elderly population.
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Project description:Background Percutaneous mitral commissurotomy ( PMC ) was the first available transcatheter technique for treatment of mitral valve diseases. Experience has led to extending the indications to patients with less favorable characteristics. We aimed to analyze (1) the temporal trends in characteristic and outcomes of patients undergoing PMC in a single center over 30 years and (2) the predictive factors of poor immediate results of PMC . Methods and Results From 1987 to 2016, 1 full year for each decade was analyzed: 1987, 1996, 2006, and 2016. Poor immediate results of PMC were defined as a mitral valve area <1.5 cm2 or MR (mitral regurgitation) grade >2. Mitral anatomy was assessed using the Cormier classification and the fluoroscopic extent of calcification. Six hundred three patients were included: 111, 202, 205, and 85, respectively. Mean age increased >10 years over time ( P<0.0001). Mitral anatomy was less favorable over the years: the presence of calcification increased from 25% of patients at the beginning of PMC to >40% during the past decade ( P<0.0001) with a 3-fold increase in severe mitral calcification. Consistently, the proportion of good immediate results decreased over time ( P<0.05) but remained at 76% in 2016. Multivariate analysis showed 3 predictive factors of poor immediate results: smaller baseline mitral valve area ( P<0.0001), pre- PMC MR grade 2 ( P<0.01), and the presence or amount of calcification ( P<0.001). Conclusions This clinic's patients became significantly older with more frequent and severe calcification in the past decade. Predictive factors of poor immediate results were related to valve anatomy, including calcification. Despite challenges raised by severe calcification, PMC was still successful in >3 out of 4 patients in recent years.
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Project description:BackgroundEmergency medical services regularly encounter severe burns. As standards of care are relatively well-established regarding their hospital management, prehospital care is comparatively poorly defined. The aim of this study was to describe burned patients taken care of by our physician-staffed emergency medical service (PEMS).MethodsAll patients directly transported by our PEMS to our burn centre between January 2008 and December 2017 were retrospectively enrolled. We specifically addressed three "burn-related" variables: prehospital and hospital burn size estimations, type and volume of infusion and pain assessment and management. We divided patients into two groups for comparison: TBSA < 20% and ≥ 20%. We a priori defined clinically acceptable limits of agreement in the small and large burn group to be ±5% and ± 10%, respectively.ResultsWe included 86 patients whose median age was 26 years (IQR 12-51). The median prehospital TBSA was 10% (IQR 6-25). The difference between the prehospital and hospital TBSA estimations was outside the limits of agreement at 6.2%. The limits of agreement found in the small and large burn groups were - 5.3, 4.4 and - 10.1, 11, respectively. Crystalloid infusion was reported at a median volume of 0.8 ml/kg/TBSA (IQR 0.3-1.4) during the prehospital phase, which extrapolated over the first 8 h would equal to a median volume of 10.5 ml/kg/TBSA. The median verbal numeric rating scale on scene was 6 (IQR 3-8) and 3 (IQR 2-5) at the hospital (p < 0.001). Systemic analgesia was provided to 61 (71%) patients, predominantly with fentanyl (n = 59; 69%), followed by ketamine (n = 7; 8.1%). The median doses of fentanyl and ketamine were 1.7 mcg/kg (IQR 1-2.6) and 2.1 mg/kg (IQR 0.3-3.2), respectively.ConclusionsWe found good agreement in burn size estimations. The quantity of crystalloid infused was higher than the recommended amount, suggesting a potential risk for fluid overload. Most patients benefited from a correct systemic analgesia. These results emphasized the need for dedicated guidelines and decision support aids for the prehospital management of burned patients.
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Project description:Phelan-McDermid syndrome (PMS) was initially called the 22q13 deletion syndrome based on its etiology as a deletion of the distal long arm of chromosome 22. These included terminal and interstitial deletions, as well as other structural rearrangements. Later, pathogenetic variants and deletions of the SHANK3 gene were found to result in a phenotype consistent with PMS. The association between SHANK3 and PMS led investigators to consider disruption/deletion of SHANK3 to be a prerequisite for diagnosing PMS. This narrow definition of PMS based on the involvement of SHANK3 has the adverse effect of causing patients with interstitial deletions of chromosome 22 to "lose" their diagnosis. It also results in underreporting of individuals with interstitial deletions of 22q13 that preserve SHANK3. To reduce the confusion for families, clinicians, researchers, and pharma, a simple classification for PMS has been devised. PMS and will be further classified as PMS-SHANK3 related or PMS-SHANK3 unrelated. PMS can still be used as a general term, but this classification system is inclusive. It allows researchers, regulatory agencies, and other stakeholders to define SHANK3 alterations or interstitial deletions not affecting the SHANK3 coding region.
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Project description:The lysosomal storage disorder Fabry disease is caused by deficient or absent activity of the GLA gene enzyme α-galactosidase A. In the present study we present the molecular and biochemical data of the Danish Fabry cohort and report 20 years' (2001-2020) experience in cascade genetic screening at the Danish National Fabry Disease Center. The Danish Fabry cohort consisted of 26 families, 18 index patients (9 males and 9 females, no available data for 8 index-patients) and 97 family members with a pathogenic GLA variant identified by cascade genetic testing (30 males and 67 females). Fourteen patients (5 males and 9 females; mean age of death 47.0 and 64.8 years respectively) died during follow-up. The completeness of the Fabry patient identification in the country has resulted in a cohort of balanced genotypes according to gender (twice number of females compared to males), indicating that the cohort was not biased by referral, and further resulted in earlier diagnosis of the disease by a lower age at diagnosis in family members compared to index-patients (mean age at diagnosis: index-patients 42.2 vs. family members 26.0 years). Six previously unreported disease-causing variants in the GLA gene were discovered. The nationwide screening and registration of Fabry disease families provide a unique possibility to establish a complete cohort of Fabry patients and to advance current knowledge of this inherited rare lysosomal storage disorder.
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Project description:Specialized adult care of phenylketonuria (PKU) patients is of increasing importance. Adult outpatient clinics for inherited errors of metabolism can help to achieve this task, but experience is limited. Ten years after establishment of a coordinated transition process and specialised adult care for inherited metabolic diseases, adult PKU care was evaluated with respect to metabolic control, therapy satisfaction, life satisfaction, sociodemographic data, economical welfare as well as pregnancy outcome.All PKU patients transferred from paediatric to adult care between 2005 and 2015 were identified. A retrospective data analysis and a cross-sectional survey in a sub-cohort of 30 patients including a questionnaire for assessing quality of life (FLZm) were performed as a single-centre investigation at the metabolic department of the University Hospital Leipzig, Germany. For statistical analysis, Mann-Whitney-U-test, t-test for independent samples, ANOVA and chi square test were used as appropriate.96 PKU patients (56 females/40 males; median age 32 years, range 18-62) were included. In the last 3-year period, 81% of the transferred patients still kept contact to the adult care centre. Metabolic control was stable over the evaluation period and dried blood phenylalanine concentrations mostly remained within the therapeutic range (median 673.0 ?mol/l, range 213.0-1381.1). Sociodemographic data, economical welfare and life satisfaction data were comparable to data from the general population. However, differences could be revealed when splitting the cohort according to time of diagnosis and to management during childhood. 83% of the PKU adults were satisfied with the transition process and current adult care. 25 completed pregnancies were supervised; three newborns, born after unplanned pregnancy, showed characteristic symptoms of maternal PKU syndrome.Continuous care for adult PKU patients in a specialized outpatient clinic is successful, leading to good to satisfactory metabolic control and social outcomes. Uninterrupted good metabolic treatment throughout childhood and adolescence positively influences educational, professional and economic success in later life. Further effort in specialized paediatric and adult metabolic care is needed to prevent loss of follow-up and to support the recommended life-long treatment and/or care.
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Project description:Classic infantile Pompe disease (IPD) is a rare lysosomal storage disorder characterized by severe hypertrophic cardiomyopathy and profound muscle weakness. Without treatment, death occurs within the first 2 years of life. Although enzyme replacement therapy (ERT) with alglucosidase alfa has improved survival, treatment outcome is not good in many cases and is largely dependent on age at initiation. The objective of the study was (a) to analyse the different stages in the diagnosis and specific treatment initiation procedure in IPD patients, and (b) to compare clinical and biochemical outcomes depending on age at ERT initiation (<1 month of age vs. <3 months of age). Here, we show satisfactory clinical and biochemical outcomes in two IPD patients after early treatment initiation before 3 months of life with immunomodulatory therapy in the ERT-naïve setting, with a high ERT dose from the beginning. Despite the overall good evolution, the patient who initiated treatment <1 month of life presented even better outcomes than the patient who started treatment <3 months of life, with an earlier normalization of hypertrophic cardiomyopathy, along with CK normalization, highlighting the importance of early treatment initiation in this progressive disease before irreversible muscle damage has occurred.
| S-EPMC8620435 | biostudies-literature
Project description:Background: The technique of urogenital plastic surgery continues to pose difficulties for surgeons due to physical features of the anatomical region, which complicates the correction of various severe male urogenital pathologies. The goal of the surgeon is to create a neophallus that allows for urination and intercourse with minimal damage to the donor site. This special topic provides a historical overview and principles for optimal phalloplasty. To improve results in latissimus dorsi free-flap phalloplasty, we share our approach, its benefits, and the lessons we have learned. Methods: We performed a retrospective review of patients who underwent shaft-only, two-stage or one-stage latissimus dorsi flap phalloplasty with or without reinnervation at a single institution from 1991 through 2020. Patients with a minimum of 1 year of follow-up were included. Data on the patient’s demographics, the procedure, and the results of the operation were maintained. Results: In total, 592 latissimus dorsi flap phalloplasties were performed during the entire study period. Of the phalloplasties, 494 (83.5%) were performed for gender-affirming surgery, of which 470 were performed for transgender patients and 24 for intersex patients. Twenty-five patients (4.2%) had congenital malformations, 17 (2.9%) had oncologic resections, and 56 had posttraumatic loss (9.5%). Conclusion: Latissimus dorsi flap total phalloplasty with reinnervation is currently one of the few methods that can solve not only an aesthetic problem, but also a functional one without the use of an endoprosthesis if the level of reinnervation and muscle contraction is sufficient.
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Project description:BackgroundInborn errors of metabolism (IEM) are a diverse group of genetic disorders that can result in significant morbidity and sometimes death. Metabolic management can be challenging and burdensome for families. Liver transplantation (LT) is increasingly being considered a treatment option for some IEMs. IEMs are now considered the second most common reason for pediatric LT.AimTo review the data of all children with an IEM who had LT at The Children's Hospital at Westmead (CHW), NSW, Australia between January 1986 and January 2019.MethodsRetrospective data collected from the medical records and genetic files included patient demographics, family history, parental consanguinity, method of diagnosis of IEM, hospital and intensive care unit admissions, age at LT, graft type, clinical outcomes and metabolic management pre and post-LT.ResultsTwenty-four LT were performed for 21 patients. IEM diagnoses were MSUD (n = 4), UCD (n = 8), OA (n = 6), TYR type I (n = 2) and GSD Ia (n = 1). Three patients had repeat transplants due to complications. Median age at transplant was 6.21 years (MSUD), 0.87 years (UCD), 1.64 years (OA) and 2.2 years (TYR I). Two patients died peri-operatively early in the series, one died 3 months after successful LT due to septicemia. Eighteen LTs have been performed since 2008 in comparison to six LT prior to 2008. Dietary management was liberalized post LT for all patients.ConclusionsReferral for LT for IEMs has increased over the last 33 years, with the most referrals in the last 10 years. Early LT has resulted in improved clinical outcomes and patient survival.
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