Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients.

INTRODUCTION: PRP is a rare entity of unknown etiopathogenesis. Lack of clinical practice guidelines makes management challenging for clinicians. OBJECTIVE: To add our experience to the corpus evidence on PRP. METHODS: This was a retrospective, descriptive, and multicentric study of 65 patients with PRP, the largest European case series of patients with PRP ever reported. RESULTS: PRP was more prevalent in men with a mean age of 51 years, yet erythrodermic forms presented in older patients (mean age, 61 years).Six (75%) pediatric patients and 10 (60%) non-erythrodermic adults controlled their disease with topical corticosteroids. However, 26 (68%) erythrodermic patients required biologic therapy as the last and effective therapy for a mean 6.5 months to achieve complete response. CONCLUSION: Our study showed a statistical difference in terms of outcome and response to treatment between children, or patients with limited disease and patients who develop erythroderma.

Estimation of gestational age in neonates using clavicular-pubis length on routine chest-abdomen radiographs.

BACKGROUND: Neonatal diseases differ depending on gestational age and weight. In the setting of an emergency in the neonatal intensive care unit (NICU), relevant clinical information is often not available when the first neonatal radiograph is obtained. When reading an initial chest-abdomen radiograph, the paediatric radiologist needs gestational age data for best radiologic practice. A transverse diameter of the chest has been previously described to estimate gestational age (GA). OBJECTIVES: To determine the strength of the correlation between GA/weight and clavicular-pubis length (CPL) on admission radiographs; to obtain a quadratic formula based on the correlation of CPL with GA and to demonstrate if a more simplified formula used by our group works as efficiently as the formula provided by the regression analysis. MATERIALS AND METHODS: A retrospective study was approved by the institutional review board and informed consent was waived. The length from the medial aspect of the clavicle to the pubic bone was measured on the initial portable chest-abdomen radiographs of 260 patients admitted to the NICU in 2016. Regression analysis was performed to investigate the association between CPL and GA/birth weight. RESULTS: One hundred eleven females and 149 males with GA between 23 and 42 weeks were evaluated. CPL was statistically associated with both GA (P<0,01) and birth weight. The estimation can be expressed with an equation of the model: GA (weeks) = (CPL in cm - 1.98)/0.42. A simplified formula: GA (weeks) = (CPL in cm) ×2+2, strongly correlates with the equation model. CONCLUSION: In patients in whom it is not known, GA can be estimated by measuring the length between medial clavicle and symphysis pubis using the formulae we propose.

Ultra-processed food intake and all-cause mortality: DRECE cohort study.

OBJECTIVE: To determine the association between ultra-processed food (UPF) intake and all-cause mortality in a representative sample of Spanish population. DESIGN: Prospective cohort design in which follow-up lasted from baseline (1991) to mortality date or 31 December 2017, whichever was first. Dietary information was collected using a validated frequency questionnaire and categorised following the NOVA classification according to the extent of food processing. The association between consumption of UPF and mortality was analysed using Cox models. Isoenergetic substitution models were constructed to compare the health effects of the NOVA groups. SETTING: Cohort from the Diet and Risk of Cardiovascular Diseases (CVD) in Spain (DRECE) study, representative of the Spanish population. PARTICIPANTS: Totally, 4679 subjects between 5 and 59 years old. RESULTS: Average consumption of UPF was 370·8 g/d (24·4 % of energy intake). After a median follow-up of 27 years, 450 deaths occurred. Those who consumed the highest amount of UPF had higher risk of mortality. For every 10 % of the energy intake from UPF consumption, an increase of 15 % in the hazard of all-cause mortality was observed (HR 1·15; (95 % CI 1·03, 1·27); P-value = 0·012). Substitution of UPF with minimally processed foods was significantly associated with a decreased risk of mortality. CONCLUSIONS: An increase in UPF consumption was associated with higher risk of all-cause mortality in a representative sample of the Spanish population. Moreover, the theoretical substitution of UPF with unprocessed or minimally processed foods leads to a decrease in mortality. These results support the need to promote diets based on unprocessed or minimally processed foods.

Meta-analysis of perioperative immunotherapy in renal cell carcinoma: Available, but the jury is still out.

INTRODUCTION: While surgical management of renal cell carcinoma (RCC) is curative for many patients, others may relapse and could benefit from adjuvant treatments. Immune checkpoint inhibitors (ICI) have been proposed as a potential adjuvant therapy for improving survival in these patients, but the benefit/risk ratio of ICI in the perioperative setting remains unclear. METHODS: A systematic review and a meta-analysis of phase III trials of perioperative ICI (anti PD1/PD-L1 alone or in combination with anti-CTLA4 agents) in RCC was conducted. RESULTS: The analysis included results from 4 phase III trials, comprising 3,407 patients. ICI did not show a significant increase in disease-free survival (Hazard Ratio [HR] 0.85; 95% confidence interval [CI] 0.69-1.04; p: 0.11) or overall survival [OS] (HR 0.73; 95% CI 0.40-1.34; p: 0.31). High-grade adverse events were more frequent in the immunotherapy arm (OR 2.65; 95% CI 1.53-4.59; p: <0.001), and high-grade treatment-related adverse events were 8 times more frequent in the experimental arm (OR: 8.07; 95% CI: 3.14-20.75; p: <0.001). Subgroup analyses showed statistically significant differences favoring the experimental arm in females (HR: 0.71; 95 CI 0.55-0.92; p: 0.009), in sarcomatoid differentiation (HR: 0.60 95% CI 0.41-0.89; p: 0.01), and PD-L1 positive tumors (HR HR: 0.74; 95% CI 0.61-0.90; p: 0.003). No significant effect was found in patients according to age, type of nephrectomy (radical vs. partial), and stage (M1 without evidence of disease vs. M0 patients). CONCLUSION: Our comprehensive meta-analysis generally suggests that immunotherapy does not confer a survival advantage in the perioperative setting for RCC, with the exception of one positive study. While the overall results are not statistically significant, individual patient factors and other variables may play a role in determining who benefits from immunotherapy. Therefore, despite the mixed findings, immunotherapy may still be a viable treatment option for certain patients, and further studies are needed to determine which patient subgroups would be most likely to benefit.

Home calligraphic exercises as manual dexterity training in patients with Parkinson's disease: a pilot feasibility study.

PURPOSE: To assess the feasibility and effects on manual dexterity and the quality of life (QoL) of a 12-week home calligraphic training program in patients with Parkinson's disease (PD). METHODS: A pilot study with participants recruited from the Movement Disorders consultation at the Hospital 12 de Octubre (Madrid). The main outcome, manual dexterity, was assessed using the Purdue Pegboard Test (PPT). Secondary outcomes included clinical rating scales that contemplate aspects related to manual dexterity (DextQ-24, UPDRSII, UPDRSIII), and QoL (PDQ-39 and EuroQoL-5D). RESULTS: Thirty PD patients (57% males) with a mean age of 66.11 (9.76) years and 93% adherence rate. The PPT scores improved significantly (p < 0.0001) from T0 (start of the study) to T1 (after 24 weeks). No statistically significant change was found in DextQ-24, UPDRS-II and UPDRS-III, but a clear improvement was observed in the QoL measurement: EuroQoL-5D (p < 0.0001), PDQ-39 (p < 0.0001) and modified PDQ-39 (p = 0.022). CONCLUSIONS: This is the first study to demonstrate the feasibility and improvement in hand dexterity assessed by the PPT for patients diagnosed with PD after a 12-week home calligraphic training program. A significant improvement was noted in the QoL measurements, such as the PDQ-39, modified PDQ-39, and EuroQoL-5D.Implications for RehabilitationMost patients with Parkinson's disease suffer from impaired manual dexterity, making it difficult to perform activities of daily living such as eating, buttoning, or shaving.A 12-week home calligraphic training program could improve hand dexterity in these patients.The advantage of this home calligraphic trainingis is that it is an easy-to-perform, low-cost and no side effects.This training also improves their quality of life.

Human Milk Composition and Nutritional Status of Omnivore Human Milk Donors Compared with Vegetarian/Vegan Lactating Mothers.

Women of childbearing age in Western societies are increasingly adopting vegetarian diets. These women are sometimes rejected as milk donors, but little about the composition of their milk is known. The present study aimed to compare the intake, nutritional status, and nutritional composition of human milk from omnivore human milk donors (Donors) and vegetarian/vegan lactating mothers (Veg). Milk, blood, and urine samples from 92 Donors and 20 Veg were used to determine their fatty acid profiles, as well as vitamins and minerals. In a representative sample of both groups, we also determined the lipid class profile as a distribution of neutral and polar lipids, the molecular species of triacylglycerols, and the relative composition of phospholipids in their milk. A dietary assessment was conducted with a five-day dietary record (while considering the intake of supplements). We highlight the following results, expressed as the mean (SE), for the Veg vs. Donors: (1) Their docosahexaenoic acid (DHA) intake was 0.11 (0.03) vs. 0.38 (0.03) g/day; the plasma DHA was 0.37 (0.07) vs. 0.83 (0.06)%; and the milk DHA was 0.15 (0.04) vs. 0.33 (0.02)%. (2) Their milk B12 levels were 545.69 (20.49) vs. 482.89 (4.11) pM; 85% of the Veg reported taking B12 supplements (mean dose: 312.1 mcg/day); and the Veg group showed no differences with Donors in terms of total daily intake or plasma B12. (3) Their milk phosphatidylcholine levels were 26.88 (0.67) vs. 30.55 (1.10)%. (4) Their milk iodine levels were 126.42 (13.37) vs. 159.22 (5.13) mcg/L. In conclusion, the Vegs' milk was shown to be different from the Donors' milk, mainly due to its low DHA content, which is concerning. However, raising awareness and ensuring proper supplementation could bridge this gap, as has already been achieved for cobalamin.

Frontline immune checkpoint inhibitor-based combination therapy in metastatic renal cell carcinoma patients with poor performance status.

BACKGROUND: Immune checkpoint inhibitor-based combination therapy (ICI-based combination) is a new standard of care for metastatic clear cell renal cell carcinoma (mRCC) in the frontline setting. Patients with poor performance status (PS) (≥2) were excluded from pivotal trials. Hence, the activity and safety of ICI-based combination therapy in this group of patients is still unknown. METHODS: We performed a multicentre retrospective study of PS ≥2 mRCC patients who received frontline ICI-based combination, either nivolumab-ipilimumab (NI) or pembrolizumab-axitinib (AP). Patients' characteristics, clinical outcomes, and toxicity were collected. We analysed overall response rate (ORR), median progression-free survival (mPFS), median overall survival (mOS) and grade ≥3 adverse events (G ≥ 3AEs). The association between the predictive biomarker IPI (immune prognostic index) and ORR/PFS/OS was also evaluated. RESULTS: We identified 70 mRCC patients with PS ≥2 treated with ICI-based combination across 14 institutions between October 2017 and December 2021, including 45 and 25 patients were treated with NI and AP, respectively. Median age at diagnosis was 63 years, 51 (73%) were male, only 17 (24%) had prior nephrectomy, 50 (71%) had synchronous metastatic disease at diagnosis, and 16 (23%) had brain metastases. Sixty-one (87%) and 9 (13%) patients had ECOG (Eastern Cooperative Oncology Group) PS 2 and 3, respectively, and 25 (36%) and 45 (64%) patients were intermediate and poor International Metastatic RCC Database Consortium (IMDC) risk, respectively. Among all, 91% were clear cell RCC, 7 patients had sarcomatoid features. At the time of the analysis (median follow-up 11.1 months), 41% patients were dead. Median PFS and mOS in the entire cohort were 5.4 months and 16.0 months, respectively; ORR was 31%. No significant differences in ORR, PFS, OS, or G ≥3AEs were seen between NI and AP. The intermediate and poor IPI groups were significantly associated with reduced ORR and shorter PFS. CONCLUSION: We report the first cohort of PS ≥2 mRCC patients treated with frontline ICI-based combination therapy. The survival outcomes in our cohort were inferior to that reported in pivotal trials. No significant differences in ORR, PFS, OS or toxicity were seen between NI and AP. Prospective real-world studies are needed to confirm these results.

Geographical and Temporal Variability of Ultra-Processed Food Consumption in the Spanish Population: Findings from the DRECE Study.

The consumption of ultra-processed foods (UPFs) has increased in recent decades, worldwide. Evidence on the negative impacts of food processing on health outcomes has also been steadily increasing. The aim of this study is to describe changes in consumption patterns of ultra-processed foods in the Spanish population over time and their geographical variability. Data from four representative cohorts of the Spanish population were used (1991−1996−2004−2008). Dietary information was collected using a validated frequency questionnaire and categorized using the NOVA classification. A total increase of 10.8% in UPF consumption between 1991 and 2008 was found in Spain (p-value < 0.001). The products contributing most to UPF consumption were sugar-sweetened beverages, processed meats, dairy products, and sweets. Those who consumed more ultra-processed foods were younger (p-value < 0.001) and female (p-value = 0.01). Significant differences between the different geographical areas of Spain were found. The eastern part of Spain was the area with the lowest UPF consumption, whereas the north-western part was the area with the highest increase in UPF consumption. Given the negative effect that the consumption of ultra-processed foods has on health, it is necessary to implement public health policies to curb this increase in UPF consumption.

Two Dimensions of Nutritional Value: Nutri-Score and NOVA.

Front-of-pack labels can improve the ability of consumers to identify which foods are healthier, making them a useful public health tool. Nutri-Score is a front-of-pack labelling system adopted by several European countries. This system ranks foods according to their nutritional quality, but does not consider other dimensions such as the degree of food processing. The aim of this study is to compare the nutritional quality (as assessed by Nutri-Score) and the ultra-processing (as assessed by the NOVA classification) of foods in the Open Food Facts database. A simple correspondence analysis was carried out to study the relationship between the two systems. Ultra-processed foods (NOVA 4) were found in all Nutri-Score categories, ranging from 26.08% in nutritional category A, 51.48% in category B, 59.09% in category C, 67.39% in category D to up to 83.69% in nutritional category E. Given the negative effect that the consumption of ultra-processed foods has on different aspects of health, front-of-pack labelling with Nutri-Score should at least be accompanied by complementary labelling indicating the level of processing, such as the NOVA classification.

Physical activity and risk of Parkinson's disease and parkinsonism in a prospective population-based study (NEDICES).

To investigate whether physical activity (PA) is a protective factor for the incidence of Parkinson's disease (PD) and parkinsonism after three years of follow-up. All participants of this study were obtained from the Neurological Disorders in Central Spain (NEDICES), a prospective population-based cohort survey of older subjects (≥65 years) that comprised 5278 census-based participants at baseline (1994-1995). A modified version of Rosow-Breslau questionnaire was applied to categorize PA into active versus sedentary group. The final diagnosis of PD and parkinsonism was made by an expert neurologist. Cox regression models (CRM) adjusted for several covariates (sex, age, education, alcohol consumption, tobacco, stroke, hypertension and body mass index) were used to calculate the association between PA (active group vs. sedentary) and risk of PD and parkinsonism after three years. 22 incident PD and 25 incident parkinsonism cases were identified among 2943 participants with available PA information (57.1% female; mean age = 73.28 ± 6.24 years) after three years of follow-up. The CRM showed that the active group (vs. sedentary) showed a lower risk of parkinsonism (Hazard ratio (HR) = 0.18; 95% CI [0.07-0.51]; p = 0.0001). However, this effect was restricted to men (HR = 0.34; 95% CI [0.11-0.99], p < 0.05) for incident PD. PA may be a protective factor for incident parkinsonism, whereas this effect was only significant for men in the case of PD. The mechanisms implicated for brain maintenance in active individuals and the neurophysiological differences behind the role of sex on PD are discussed.

Response to systemic therapy in fumarate hydratase-deficient renal cell carcinoma.

PURPOSE: Fumarate hydratase-deficient (FHdef) renal cell carcinoma (RCC) is a rare entity associated with the hereditary leiomyomatosis and RCC syndrome with no standard therapy approved. The aim of this retrospective study was to evaluate the efficacy of different systemic treatments in this population. METHODS: We performed a multicentre retrospective analysis of Fhdef RCC patients to determine the response to systemic treatments. The endpoints were objective response rate (ORR), time-to-treatment failure (TTF), and overall survival (OS). The two latter were estimated using the Kaplan-Meier method. RESULTS: Twenty-four Fhdef RCC patients were identified, and 21 under systemic therapy were included in the analysis: ten received cabozantinib, 14 received sunitinib, nine received "other antiangiogenics" (sorafenib, pazopanib, and axitinib), three received erlotinib-bevacizumab (E-B), three received mTOR inhibitors, and 11 received immune checkpoint blockers (ICBs). ORR for treatments were 50% for cabozantinib, 43% for sunitinib, 63% for "other antiangiogenics," and 30% for E-B, whereas ORR was 0% for mTOR inhibitors and 18% for ICBs. The median TTF (mTTF) was significantly higher with antiangiogenics (11.6 months) than with mTOR inhibitors (4.4 months) or ICBs (2.7 months). In the first-line setting, antiangiogenics presented a higher ORR compared with nivolumab-ipilimumab (64% versus 25%) and a significantly superior mTTF (11.0 months vs 2.5 months; p = 0.0027). The median OS from the start of the first systemic treatment was 44.0 months (95% confidence interval: 13.0-95.0). CONCLUSIONS: We report the first European retrospective study of Fhdef RCC patients treated with systemic therapy with a remarkably long median OS of 44.0 months. Our results suggest that antiangiogenics may be superior to ICB/mTOR inhibitors in this population.