Incidence and Prevalence of 73 Different Genodermatoses: A Nationwide Study in Sweden.

This retrospective registry-based cohort study aimed to estimate the incidence and prevalence of genodermatoses in the Swedish population and to analyse associated healthcare usage. Patients diagnosed with genodermatoses were identified from the patient registry of Sahlgrenska University Hospital (Gothenburg, Sweden) between 2016 and 2020. Clinical data from medical records were used to verify diagnoses recorded in the National Patient Registry (NPR). The NPR was then searched for International Classification of Diseases, Tenth Revision (ICD-10) codes Q80-82 and Q84 from 2001 to 2020. The local cohort included 298 patients with 36 unique genodermatosis diagnoses. Verification of these diagnoses in the NPR showed positive predictive values of over 90%. The NPR search yielded 13,318 patients with 73 unique diagnoses, including ichthyoses (n = 3,341; 25%), porokeratosis (n = 2,277; 17%), palmoplantar keratodermas (n = 1,754; 13%), the epidermolysis bullosa group (n = 1011; 7%); Darier disease (n = 770; 6%), Hailey-Hailey disease (n = 477; 4%) and Gorlin syndrome (n = 402; 3%). The incidence and prevalence of each diagnosis were calculated based on the nationwide cohort and are reported. A total of 149,538 outpatient visits were registered, a mean of 4.6 visits per patient. This study provides a valuable resource for the epidemiology of genodermatoses by reporting on the incidence and prevalence of 73 different genodermatoses.

Porokeratosis is one of the most common genodermatoses and is associated with an increased risk of keratinocyte cancer and melanoma.

BACKGROUND: Porokeratosis is a clinically heterogeneous group of keratinization disorders with a genetic background mainly affecting the mevalonate pathway, which is involved in the synthesis of cholesterol, an essential component for the formation of the extracellular lipid lamellae in the stratum corneum. Porokeratosis is reportedly associated with an increased risk of keratinocyte cancer, but to date, no large epidemiological studies have been conducted to further address this association. OBJECTIVES: The first objective was to characterize a cohort of patients diagnosed with porokeratosis at the Department of Dermatology and Venereology, Sahlgrenska University Hospital (SU), Gothenburg, Sweden. The second objective was to conduct a nationwide registry-based cohort study to investigate the association, if any, between porokeratosis and the cutaneous malignancies squamous cell carcinoma (SCC), basal cell carcinoma (BCC) and melanoma. METHODS: For the SU cohort, the hospital registry was searched for patients with a diagnosis of porokeratosis recorded between 2016 and 2020. Clinical data were extracted from the records of the identified patients. For the nationwide cohort, national registries were searched to identify patients with a diagnosis of porokeratosis between 2001 and 2020. A tenfold control cohort was formed by Statistics Sweden. The data was cross-referenced with the Swedish Cancer Register to study the associations between porokeratosis and SCC, BCC and melanoma. RESULTS: Disseminated superficial actinic porokeratosis was the most common clinical type among the 108 patients in the SU cohort. In the nationwide search, 2277 patients with porokeratosis were identified (prevalence 1/4132). Porokeratosis was associated with an increased risk for SCC, BCC and melanoma with hazard ratios (95% CI) of 4.3 (3.4-5.4), 2.42 (1.97-2.98) and 1.83 (1.18-2.82), respectively, in the patient cohort, compared to the matched control group. CONCLUSION: Porokeratosis is a common genodermatosis, and it is associated with an enhanced risk of skin cancer.

Cyanide poisoning by fire smoke inhalation: a European expert consensus.

Smoke inhalation is a common cause of cyanide poisoning during fires, resulting in injury and even death. In many cases of smoke inhalation, cyanide has increasingly been recognized as a significant toxicant. The diagnosis of cyanide poisoning remains very difficult, and failure to recognize it may result in inadequate or inappropriate treatment. Findings suggesting cyanide toxicity include the following: (a) a history of enclosed-space fire; (b) any alteration in the level of consciousness; (c) any cardiovascular changes (particularly inexplicable hypotension); and (d) elevated plasma lactate. The feasibility and safety of empiric treatment with hydroxocobalamin for fire smoke victims have been reported in the literature. On the basis of a literature review and a panel discussion, a group of European experts has proposed emergency management protocols for cyanide toxicity in fire smoke victims.

A case of hypothermic cardiac arrest treated with an external chest compression device (LUCAS) during transport to re-warming.

The recommended treatment for severe hypothermia with circulatory collapse is re-warming using cardiopulmonary by-pass. This may require transporting a patient to hospital with on-going cardiopulmonary resuscitation (CPR). Manual CPR during patient transport may result in sub-optimal chest compressions and can be a hazard for the ambulance crew. We report a case of a patient with a core temperature of 22.2 degrees C and crew-witnessed cardiac arrest due to hypothermia. After unsuccessful initial resuscitation he was transported to hospital for re-warming with cardiopulmonary by-pass. CPR was continued during transport using a mechanical active compression-decompression device (the LUCAS-device). During cardiopulmonary by-pass ROSC was achieved after 90 min of cardiac arrest. The patient recovered with a cerebral performance category of 3. Using a mechanical device for chest compressions during transport of a hypothermic patient with on-going CPR is feasible, effective and safe.

Emergency call processing and survival from out-of-hospital ventricular fibrillation.

OBJECTIVES: Our aim was to report the effect of the emergency call processing in the dispatching centre on survival from out-of-hospital ventricular fibrillation (VF). METHODS: This retrospective cohort study was conducted in Helsinki Emergency Medical Services. All consecutive cases with out-of-hospital bystander witnessed VF of cardiac origin between 1 January 1997 and 31 December 2002 were included. Data were collected prospectively. Call processing times, call numbers per dispatcher and telephone guided cardiopulmonary resuscitation (CPR) were studied. Discharge alive from hospital was used as primary end point. RESULTS: The study population consisted of 373 cases. Cardiac arrest (CA) was recognised in 296 cases (79.4%) by the dispatcher. Survival to discharge was 37.2% (110/296) if CA was recognised and 28.6% (22/77) if it was not recognised (p=0.1550). When the dispatcher handled <4 VF calls during the study period survival to discharge was 22.1% (17/77) compared to 38.2% (50/131) and 39.4% (65/165) when the call volume was 4-9 or >9 (p=0.0227). The mean time to dispatch a first responding unit (FRU) was 77.1+/-44.3 s. Survival to discharge was 39.4% (65/165) when the FRU dispatching time was <60s and 32.2% (67/208) when dispatching took > or =60 s (p=0.1496). The mean time to CA recognition was 170.2+/-130.1 s. Spontaneous circulation was achieved more rapidly when the time was <150 s (p=0.0426), but there was no difference in survival to discharge. Telephone guided CPR instructions were given in 123 cases (35.5%). Survival to discharge was 43.1% (53/123) when CPR instructions were given and 31.7% (72/223) when they were not given (p=0.0453). CONCLUSIONS: We showed that low CA call numbers per dispatcher is associated with a decreased probability of survival. Giving telephone guided CPR instructions should be promoted as they influence the outcome. Further studies are needed to determine optimal call processing times.

Prehospital mortality in an EMS system using medical priority dispatching: a community based cohort study.

OBJECTIVES: This study was planned to record prehospital death rates in four medical priority categories (A, B, C and D) and to evaluate if deaths in lower urgency categories C and D (target response times 20 and 90 min) could have been avoided by a faster ambulance response. METHODS: The design was a community based cohort study including an expert panel evaluation of the deaths. The study was conducted in the Emergency Medical Services in Helsinki, Finland. All consecutive ambulance calls excluding interhospital patient transfers between 1 January 1999 and 31 December 2002 were included. Prehospital mortality and avoidability of prehospital deaths by a faster ambulance response (maximum 8 min) were used as main outcome measures. RESULTS: A total of 151928 calls were prioritized in the dispatching centre (category A 8677 calls, B 41005, C 71991 and D 30255). Prehospital death occurred 451 times in category A, 468 times in category B, 73 times in category C and 8 times in category D calls. Respectively, the prehospital death rates per 1000 calls were 52.0 (A), 11.4 (B), 1.0 (C) and 0.3 (D) (P < 0.0001). The expert panel judged that 1 (1.3%) of category C deaths would have been avoidable, 24 (32.9%) potentially avoidable and 48 (65.8%) not avoidable by a more rapid ambulance response. The corresponding figures for category D deaths were 0 (0%), 5 (62.5%) and 3 (37.5%), respectively. CONCLUSIONS: The use of medical priority dispatching was associated with very low prehospital mortality in lower urgency categories C and D. Approximately, one-third of those deaths could probably be prevented by a faster ambulance response but the price would be a three-fold increase in calls with blue lights and siren. Further studies are needed to find out if our results are applicable to other types of EMS systems.