Background
Demographic, clinical, and morphological characteristics of cutaneous melanoma are unknown for the Colombian population. We aim to provide these characteristics as well as population‐based ...incidence and survival data.
Methods
All patients with an invasive cutaneous melanoma diagnosed in the period 2000–2009 registered in the population‐based cancer registry of the metropolitan area of Bucaramanga were included for analysis (n = 169). Age‐standardized incidence rates were calculated and melanoma‐specific and overall survival estimated with follow‐up until June 9, 2016, using Kaplan–Meier methodology, stratifying for gender, anatomical localization, and type of affiliation to the social security system.
Results
The age‐standardized melanoma incidence rate was 1.7 per 100,000, with lower limbs being the most affected body sites (42.6% of all melanomas). A high proportion of melanomas presented on the plants or palms (16%) and under the nails (7.1%); at least 24.3% of melanomas were ulcerated, and 21.1% had a Breslow thickness more than 2 mm. Melanoma‐specific 5‐year survival was 79.3%, with worst survival for melanomas localized on the plants (64.6%) and subungual areas (55.6%). Affiliation to the subsidized type of affiliation to the social security system was statistically significantly (P = 0.003) associated with poorer survival (68.8%) compared to the special regimes (95.8%).
Conclusions
Melanoma is a relatively rare cancer in Colombia with mainly the acral sites, high proportion of thick and ulcerated melanomas, and relatively poor survival being distinct features, indicating the need for tailor‐made primary and secondary prevention strategies. Better training of pathologists in the difficult field of melanoma would improve precision of available data.
Educational paper de Vries, Esther; Driessen, Gertjan
European journal of pediatrics,
02/2011, Letnik:
170, Številka:
2
Journal Article
Recenzirano
Odprti dostop
Primary immunodeficiencies (PIDs) are characterized by an increased susceptibility to infections due to defects in one ore more components of the immune system
.
Although most PIDs are relatively ...rare, they are more frequent than generally acknowledged. Early diagnosis and treatment of PIDs save lives, prevent morbidity, and improve quality of life. This early diagnosis is the task of the pediatrician who encounters the child for the first time: he/she should suspect potential PID in time and perform the appropriate diagnostic tests. In this educational paper, the first in a series of five, we will describe the most common clinical presentations of PIDs and offer guidelines for the diagnostic process, as well as a brief overview of therapeutic possibilities and prognosis.
Common variable immunodeficiency (CVID), characterized by recurrent infections, is the most prevalent symptomatic antibody deficiency. In ∼90% of CVID-affected individuals, no genetic cause of the ...disease has been identified. In a Dutch-Australian CVID-affected family, we identified a NFKB1 heterozygous splice-donor-site mutation (c.730+4A>G), causing in-frame skipping of exon 8. NFKB1 encodes the transcription-factor precursor p105, which is processed to p50 (canonical NF-κB pathway). The altered protein bearing an internal deletion (p.Asp191_Lys244delinsGlu; p105ΔEx8) is degraded, but is not processed to p50ΔEx8. Altered NF-κB1 proteins were also undetectable in a German CVID-affected family with a heterozygous in-frame exon 9 skipping mutation (c.835+2T>G) and in a CVID-affected family from New Zealand with a heterozygous frameshift mutation (c.465dupA) in exon 7. Given that residual p105 and p50—translated from the non-mutated alleles—were normal, and altered p50 proteins were absent, we conclude that the CVID phenotype in these families is caused by NF-κB1 p50 haploinsufficiency.
Epidemiology of rare cutaneous malignancies in the general population is poorly documented. This descriptive study aimed to estimate the incidence and trends of all skin malignancies between 1989 and ...2005. Data on skin tumors were extracted from the Netherlands Cancer registry (except for basal cell carcinoma (BCC) data—only available from Comprehensive Cancer Centre South) and categorized according to the International Classification of Diseases for Oncology, third edition, codes. Age-standardized incidence rates (European standardized population rate, ESR) per 100,000 person-years were calculated per year and for the period between 2001 and 2005. Estimated annual percentage changes (EAPCs) were estimated by Poisson regression models. A total of 356,620 skin tumors were diagnosed between 1989 and 2005. Excluding BCC, squamous cell carcinoma (SCC), and melanoma, the remaining skin tumors constituted about 2% of all skin malignancies. The incidence of melanoma showed the steepest increase (EAPC, 4.0%), and ESR was close to that observed for SCC (EAPC, 2.3%) between 2001 and 2005 (17.1 versus 19.6). Hematolymphoid tumors (ESR=0.74) were mainly cutaneous T-cell lymphomas (60.8%). No significant increases in incidence were observed for lymphomas, and appendageal, fibromatous, and myomatous carcinomas during 1989–2005. In addition to keratinocytic cancers and melanoma, there is a wide variety of skin tumors that constitute <2% of all skin malignancies. The incidence of UV-related skin tumors increased significantly and more steeply than did those of other skin malignancies.
Population‐based cancer registries (PBCRs) are the only means to provide reliable incidence and survival data as a basis for policy‐making and resource allocations within cancer care. Yet, less than ...3% and 10% of the respective populations of Central America and South America are covered by high‐quality cancer registries. The Global Initiative for Cancer Registry Development provides support to improve this situation via the International Agency for Research on Cancer Regional Hub for Latin America. In this paper, we summarize activities (advocacy, technical assistance, training and research) over the last 5 years, their impact and current challenges, including the implementation of new PBCR in four countries in the region. Despite the favorable political support to cancer registration in many countries, the sustainability of cancer registration remains vulnerable. Renewed efforts are needed to improve data quality in Latin America while ensuring maximum visibility of the data collected by disseminating and promoting their use in cancer control.
Background
Extensively hydrolyzed formulas are recommended for the dietary management of infants with cow's milk allergy (CMA).
Objectives
Hypoallergenicity, growth, and gastrointestinal (GI) ...tolerability of a new extensively hydrolyzed whey‐protein formula (eHWF) in CMA children were assessed.
Methods
In this prospective, randomized, international, multi‐center study (Trial NL3889), 34 children with confirmed CMA (74% IgE‐mediated) underwent a double‐blind, placebo‐controlled food challenge (DBPCFC) with an eHWF developed with non‐porcine enzymes, supplemented with prebiotic short‐chain galacto‐ and long‐chain fructo‐oligosaccharides (0.8 g/L, ratio 9:1), arachidonic acid (0.35/100 g), and docosahexaenoic acid (0.35/100 g). If tolerant to the eHWF, children participated in a 7‐day open food challenge with this eHWF. Anthropometrics and GI tolerability were assessed in an optional 16‐weeks follow‐up.
Results
Of the 34 children who started the DBPCFC with the eHWF, 25 subjects (19 boys, mean age: 61 weeks, 18 with IgE‐mediated CMA) completed the DBPCFC and 7‐day open challenge without major protocol deviations and tested negative at both challenges. One child experienced a late moderate eczematous allergic reaction in the optional follow‐up period, indicating the need for close monitoring of subjects starting new formula. Weight and length gain followed the World Health Organization growth curves. Changes in frequency and consistency of stools upon test formula intake were transient.
Conclusions
The newly developed eHWF is a suitable option in CMA treatment as all subjects tolerated the product. This result is in line with the international criteria for hypoallergenicity (American Academy of Pediatrics) that state that more than 90% of CMA children must tolerate the formula. Use of the formula is also associated with normal growth curves and GI tolerability.
Trial registration
Trial NL3889, https://www.trialregister.nl/trial/3889.
Background
Emergency department (ED) visits show a high volatility over time. Therefore, EDs are likely to be crowded at peak-volume moments. ED crowding is a widely reported problem with negative ...consequences for patients as well as staff. Previous studies on the predictive value of weather variables on ED visits show conflicting results. Also, no such studies were performed in the Netherlands. Therefore, we evaluated prediction models for the number of ED visits in our large the Netherlands teaching hospital based on calendar and weather variables as potential predictors.
Methods
Data on all ED visits from June 2016 until December 31, 2019, were extracted. The 2016–2018 data were used as training set, the 2019 data as test set. Weather data were extracted from three publicly available datasets from the Royal Netherlands Meteorological Institute. Weather observations in proximity of the hospital were used to predict the weather in the hospital’s catchment area by applying the inverse distance weighting interpolation method. The predictability of daily ED visits was examined by creating linear prediction models using stepwise selection; the mean absolute percentage error (MAPE) was used as measurement of fit.
Results
The number of daily ED visits shows a positive time trend and a large impact of calendar events (higher on Mondays and Fridays, lower on Saturdays and Sundays, higher at special times such as carnival, lower in holidays falling on Monday through Saturday, and summer vacation). The weather itself was a better predictor than weather volatility, but only showed a small effect; the calendar-only prediction model had very similar coefficients to the calendar+weather model for the days of the week, time trend, and special time periods (both MAPE’s were 8.7%).
Conclusions
Because of this similar performance, and the inaccuracy caused by weather forecasts, we decided the calendar-only model would be most useful in our hospital; it can probably be transferred for use in EDs of the same size and in a similar region. However, the variability in ED visits is considerable. Therefore, one should always anticipate potential unforeseen spikes and dips in ED visits that are not shown by the model.
Aim
To provide a comprehensive overview of breast cancer in Colombia.
Methods
Data on breast cancer in Colombia are scarce. We present incidence data from population-based cancer registries that ...represent 4 distinct regions of the country. Other data originate from non-governmental institutions and healthcare providers within Colombia, official sources, expert opinion, Colombian legislation, and the Cancer Mortality Atlas publishes by Colombian National Cancer Institute.
Results
In Colombia, the age-standardized incidence rate remained relatively stable between 2012 and 2020 (43.1 to 47.8 cases per 100,000 women-years); Additionally, survival since 1995 has presented a substantial improvement from 65.7 to 72.1.
In 33% of cases, the diagnosis of breast cancer was made in advanced stages, stage III or higher. The health demography survey conducted in 2015 showed that the participation in mammography screening in women aged 40 to 69 remains low 48.1%. Some limitations regarding access to early detection and diagnosis include economic strata, health insurance coverage, origin, and accessibility. On average, a 90-day period was reported from onset of symptoms to diagnosis of breast cancer.
Conclusion
The first action towards improving outcomes in breast cancer should be to improve stage at diagnosis and timely access to care.
Background Immunologic alterations caused by psoriasis and/or its therapies may affect the risk of serious infections. Objective For patients with psoriasis, we explored the overall and ...therapy-related risk of contracting an infectious disease (ID) requiring hospitalization in a large population-based cohort. Methods The incidence of ID was compared between patients with psoriasis and a randomly selected cohort (ratio 1:5) using hospital and pharmacy databases covering 2.5 million Dutch residents between 1997 and 2008. First and multiple IDs were defined and categorized into 20 groups based on primary International Classification of Diseases, Ninth Revision, Clinical Modification discharge diagnoses. Multivariate Cox regression and Poisson event-count models were used to test the risk difference of IDs between patients with psoriasis and reference cohort. Results A total of 25,742 patients with psoriasis and 128,710 reference subjects were followed up for approximately 6 years. The likelihood of IDs in patients with psoriasis was twice as high as the reference population (908 vs 438 events/100,000 person-years, crude hazard ratio 2.08, 95% confidence interval 1.96-2.22). In a multivariate model the hazard ratio decreased to 1.54 (95% confidence interval 1.44-1.65). This risk was highest for patients with more severe psoriasis (adjusted hazard ratio 1.81, 95% confidence interval 1.57-2.08), but was not associated with recent systemic antipsoriatic drug dispensing. Respiratory tract, abdominal, and skin infections occurred most frequently in patients with psoriasis. Multiple event analysis that counted the total number of infectious discharge diagnoses gave similar results. Limitations No data were available on lifestyle factors. Conclusion The risk of severe infections was significantly higher for patients with psoriasis compared with control subjects and could not be explained by exposure to systemic antipsoriatic drugs.
Common variable immunodeficiency disorders (CVID), the most common form of primary antibody deficiency, are rare conditions associated with considerable morbidity and mortality. The clinical benefit ...of immunoglobulin replacement therapy (IgGRT) is substantial: timely treatment with appropriate doses significantly reduces mortality and the incidence of CVID-complications such as major infections and bronchiectasis. Unfortunately, CVID-patients still face a median diagnostic delay of 4 years. Their disease burden, expressed in annual loss of disability-adjusted life years, is 3-fold higher than in the general population. Hurdles to treatment access and reimbursement by healthcare payers may exist because the value of IgGRT is poorly documented. This paper aims to demonstrate cost-effectiveness and cost-utility (on life expectancy and quality) of IgGRT in CVID.
With input from a literature search, we built a health-economic model for cost-effectiveness and cost-utility assessment of IgGRT in CVID. We compared a mean literature-based dose (≥450mg/kg/4wks) to a zero-or-low dose (0 to ≤100 mg/kg/4wks) in a simulated cohort of adult patients from time of diagnosis until death; we also estimated the economic impact of diagnostic delay in this simulated cohort. Compared to no or minimal treatment, IgGRT showed an incremental benefit of 17 life-years (LYs) and 11 quality-adjusted life-years (QALYs), resulting in an incremental cost-effectiveness ratio (ICER) of €29,296/LY and €46,717/QALY. These results were robust in a sensitivity analysis. Reducing diagnostic delay by 4 years provided an incremental benefit of six LYs and four QALYs compared to simulated patients with delayed IgGRT initiation, resulting in an ICER of €30,374/LY and €47,495/QALY.
The health-economic model suggests that early initiation of IgGRT compared to no or delayed IgGRT is highly cost-effective. CVID-patients' access to IgGRT should be facilitated, not only because of proven clinical efficacy, but also due to the now demonstrated cost-effectiveness.