Bronchiectasis, lymphadenopathies related to BAFF overexpression and lymphoplasmacytic cystitis as adverse events associated with prolonged use of rituximab in systemic autoimmune rheumatic diseases.

BACKGROUND: The long-term use of rituximab (RTX) has been gaining ground in the treatment of systemic autoimmune diseases. The adverse events (AEs) associated with its use different to infections are being reported. METHODS: A cohort of patients with SAIDs treated at a high-complexity center in Cali (southwestern Colombia) with follow-up from January 2008 to December 2022 were examined to search for potential AEs associated with prolonged use of RTX. RESULTS: From 178 patients with long-term use of RTX 3 (1.68%) had lymphadenopathies with lymphoid follicular hyperplasia related to BAFF overexpression, 4 (2.24%) with bronchiectasis, and 4 (2.24%) with lymphoplasmacytic cystitis. CONCLUSION: Bronchiectasis, lymphoid follicular hyperplasia related to BAFF overexpression, and lymphoplasmacytic cystitis may be life-threatening long-term AEs in patients with prolonged use of RTX.

[Professional and psychological impact of second victims among hospital healthcare professionals]. / Impacto laboral y psicológico de las segundas víctimas entre los profesionales sanitarios hospitalarios.

OBJECTIVE: To analyze the occupational and psychological consequences suffered by healthcare workers who are considered second victims (SV). MATERIAL AND METHODS: Observational, descriptive and cross-sectional study among the healthcare workers of a university hospital. The answers collected in a specifically designed questionnaire about psychological consequences at work and the result of a post-traumatic stress scale, "Impact of Event Scale-Revised (IES-R, spanish version)" were evaluated. The variables between the groups were compared using the Chi square test (or Fisher's exact test) when both were qualitative and with the Student's T (or the Mann-Whitney U test for independent data), when one of them was quantitative. The level of statistical significance was P<.05. RESULTS: 75.5% (148/207) of the participants in the study suffered some adverse event (AE) and, of these, 88.5% (131/148) were considered SV. Physicians had a 2.2 times higher risk of feeling SV than nurses (95% CI: 1.88-2.52). The impact on the patient related to the AE explained why the professionals involved in it felt SV (P=.037). 80.6% (N=104) of the SVs presented post-traumatic stress. Women were 2.4 times more likely to suffer from it (OR: 2.4; 95% CI: 1.5-4.0). Intrusive thoughts in the SV were almost three times more frequent when the damage suffered by the patient was permanent or death (OR: 2.5; 95% CI: 0.2-3.6). CONCLUSIONS: Many healthcare workers, especially physicians, considered themselves to be SV, and many of them suffered from post-traumatic stress. The impact on the patient related to the AE was a risk factor for being SV and for suffering psychological consequences.

Body Mass Index impact on Extended Total Extraperitoneal Ventral Hernia Repair: a comparative study.

PURPOSE: Obesity is a risk factor for developing abdominal wall hernias and is associated with major postoperative complications, such as surgical site infection, delayed wound healing and recurrent hernia. Therefore, treating incisional hernia in this patient subgroup is a challenge. METHODS: We conducted a comparative, prospective study on patients who underwent primary ventral hernia surgery or incisional hernia surgery through the extended totally extraperitoneal pathway, with body mass indices (BMIs) ≤ 30 (no obesity) and BMI > 30 (with obesity). We collected demographic data, preoperative and intraoperative variables, complication and recurrence rate, hospital stay and follow-up as postoperative data. RESULTS: From May 2018 to December 2020, 74 patients underwent this surgery, 38 patients without obesity and 36 with obesity. The median area of the hernia defect measured by CT was 57 cm2 and 93 cm2 in patients without and with obesity, respectively (p = 0.012). The median follow-up was 16 months. One patient without obesity experienced some postoperative complication compared with four patients with obesity (p > 0.05). No patient without obesity had recurrent hernia compared with two patients with obesity (p > 0.05). CONCLUSIONS: There were statistically significant differences between patients with and without obesity in the size of the hernia defect. However, there were no significant differences in terms of complications, hospital stay, postoperative pain or relapses. Therefore, the minimally invasive completely extraperitoneal approach for patients with obesity appears to be a safe procedure despite our study limitations. Studies with longer follow-ups and a greater number of patients are needed.

Mixed vascular naevus syndrome: report of three children with somatic GNA11 mutation and new systemic associations.

Mixed vascular naevus (MVN) is characterized by the co-occurrence of telangiectatic capillary malformation and naevus anaemicus, which can appear as a pure cutaneous phenotype or be combined with systemic manifestations such as brain malformations, neurological abnormalities and musculoskeletal disorders. Recently, GNA11 and GNAQ somatic mutations have been reported in some patients with isolated and syndromic MVN. We report three children with MVN syndrome with generalized cutaneous manifestations and a number of systemic associations not reported to date, including ophthalmological anomalies, musculoskeletal abnormalities such as Sprengel deformity and posterior vertebral fusion anomalies, and septal heart defects. We also confirm a somatic mutation of GNA11 in both telangiectatic naevus and naevus anaemicus in two of our patients and discuss a possible common pathogenic mechanism underlying the different manifestations of the syndrome. Currently, there are no guidelines for the evaluation of patients with MVN syndrome, but according to the different known aspects of the disease, a complete clinical examination should be made, and complementary laboratory and imaging tests should be considered.

Múltiples úlceras perianales en relación con el uso de una pomada antihemorroidal con acetónido de triamcinolona, lidocaína y pentosano polisulfato sódico como principios activos: una serie de 11 pacientes españoles / Multiple Perianal Ulcers Related to Use of a Hemorrhoidal Ointment With the Active Ingredients Triamcinolone Acetonide, Lidocaine, and Pentosan Polysulfate Sodium: A Series of 11 Spanish Patients

La aparición de úlceras perianales en relación con una pomada antihemorroidal es una condición nunca antes reportada en la literatura. Presentamos una serie de 11 casos de 10 hospitales españoles con diagnóstico de úlceras perianales tras la aplicación de una misma pomada antihemorroidal con acetónido de triamcinolona, lidocaína y pentosano polisulfato sódico como principios activos. No se ha podido identificar ninguna condición previa o enfermedad concomitante que pudiera justificar un diagnóstico etiológico alternativo y tras retirar la pomada antihemorroidal se ha evidenciado una resolución completa de las úlceras en un periodo medio de 8 semanas. Esta serie de casos evidencia el potencial efecto dañino de un producto farmacéutico no sujeto a prescripción ni seguimiento médico y la necesidad de interrogar por el uso de agentes tópicos ante la aparición de úlceras perianales (AU)

The development of perianal ulcers related to the use of a hemorrhoidal ointment has not been reported in the literature. We describe a series of 11 patients who were treated for perianal ulcers in 10 Spanish hospitals after they used the same ointment containing the active ingredients triamcinolone acetonide, lidocaine, and pentosan polysulfate sodium. No prior or concomitant conditions suggesting an alternative cause for the condition could be identified, and after the patients stopped using the ointment, their ulcers cleared completely in 8 weeks on average. This case series shows the damage that can be caused by an over-the-counter pharmaceutical product used without medical follow-up. It also illustrates the need to ask patients with perianal ulcers about any topical agents used before the lesions appeared (AU)

Multiple Perianal Ulcers Related to Use of a Hemorrhoidal Ointment With the Active Ingredients Triamcinolone Acetonide, Lidocaine, and Pentosan Polysulfate Sodium: A Series of 11 Spanish Patients. / Múltiples úlceras perianales en relación con el uso de una pomada antihemorroidal con acetónido de triamcinolona, lidocaína y pentosano polisulfato sódico como principios activos: una serie de 11 pacientes españoles.

The development of perianal ulcers related to the use of a hemorrhoidal ointment has not been reported in the literature. We describe a series of 11 patients who were treated for perianal ulcers in 10 Spanish hospitals after they used the same ointment containing the active ingredients triamcinolone acetonide, lidocaine, and pentosan polysulfate sodium. No prior or concomitant conditions suggesting an alternative cause for the condition could be identified, and after the patients stopped using the ointment, their ulcers cleared completely in 8 weeks on average. This case series shows the damage that can be caused by an over-the-counter pharmaceutical product used without medical follow-up. It also illustrates the need to ask patients with perianal ulcers about any topical agents used before the lesions appeared.

Anatomical connectivity and efficacy of electro-therapy for seizure control: A SANTE's single-center regression analyses.

OBJECTIVE: To assess based on a single-center data from a multicenter trial (Stimulation of the Anterior Nucleus for the Thalamus for Epilepsy (SANTE)), the role of anatomical connectivity and other factors (e.g., stimulating electrode placement) on efficacy of electro-therapy of the anterior thalamic nuclei (ATN), a node in Papez network, on pharmaco-resistant seizures. DATA SOURCE: Adults with at least 6 seizures /month were enrolled in this trial. Percent seizure reduction was compared between subjects with seizures emerging inside Papez's network (IPN) to those with seizures outside it (OPN). Statistical analyses were performed on the first year of the trial. RESULTS: Data from 11 subjects were analyzed. At Year 1, median seizure reduction was 80.5% (-100% to -40.3%) in 8/11 subjects with seizures IPN, vs. 52.8% (-61.4% to -23.7%) for 3/11 subjects with seizures OPN (2-sided Wilcoxon p = 0.08). At year 7, 3/11 subjects with seizures IPN had been seizure free for several years vs. 0/11 subjects with seizures OPN. Addition of 4 subjects from a pilot trial with nearly identical protocol to SANTE's, increased to 12/15 the number of subjects with seizures IPN. A 2-sided Fisher's exact test applied to seizure frequency reduction in the 12/15 cohort compared to the 3/15 with seizures OPN, showed significant (p = 0.04) differences in efficacy at the 70% seizure reduction rate. Median quality of life (QOL) scores for subjects with seizures IPN improved by 81% vs. 53% for subjects with seizures OPN. No other factors (e.g., current intensity) had a statistically significant effect on efficacy. CONCLUSIONS: Degree of anatomical connectivity between stimulation targets and epileptogenic networks (ENs) plays an important role in therapeutic efficacy. This may be explained by the minimization of signal attenuation inherent in impulse transmission in nervous tissue partly as a function of fiber tract length, tissue anisotropy, and number of synaptic relays between stimulation target and epileptogenic networks.

Hiperpigmentación de las falanges distales del lactante / Hyperpigmentation of an Infant's Fingers and Toes

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Treatment with intravenous immunoglobulins in systemic lupus erythematosus: a single-center experience with 63 patients.

BACKGROUND: Intravenous immunoglobulin (IVIG) is prepared using purified human plasma. IVIG therapy has immunomodulatory effects on autoimmune diseases, including severe systemic lupus erythematosus (SLE). However, reports of its effects on large cohorts are scarce. METHODS: This single-center retrospective study included SLE patients treated with at least one IVIG cycle for SLE complications. Demographic data, indications, cycle numbers, and clinical improvement with IVIG were evaluated. SLE Disease Activity Index 2000 (SLEDAI-2K) scores were calculated at admission and after IVIG treatment in order to measure clinical improvement. RESULTS: Sixty-three SLE patients treated with IVIG (median age: 29 years; interquartile range 21-36 years; 84.13% female) were included, who received 2 g/kg IVIG for two to five days. Main indications were immune thrombocytopenia, hypogammaglobulinemia, infection during a SLE flare, bicytopenia, and immune hemolytic anemia. Seven patients received more than one IVIG cycle without severe adverse effects. Significant differences were found in SLEDAI-2K scores when the indications were immune thrombocytopenia and hypogammaglobulinemia, with a trend for hemolytic anemia. Patients with concomitant infection, myopathy, and gastrointestinal involvement showed a considerable reduction in their last SLEDAI-2K scores. Fourteen patients died during hospitalization, mainly due to septic shock and active SLE. CONCLUSIONS: IVIG showed adequate tolerance and effectiveness in selected severe SLE manifestations, mainly hematological involvement. It was useful for concomitant infection.

Effectiveness and safety of daclatasvir/sofosbuvir with or without ribavirin in genotype 3 hepatitis C virus infected patients. Results in real clinical practice.

OBJECTIVE: Direct-acting antivirals have shown high efficacy in all hepatitis C virus (HCV) genotypes, but genotype 3 (G3) treatments continue to be a challenge, mainly in cirrhotic patients. The aim of this study is to analyse effectiveness and safety of daclatasvir associated with sofosbuvir with or without ribavirin in G3-HCV infected patients in real clinical practice. METHODS: An observational, prospective, cohort study over 2.5 years, in G3-HCV infected adult patients, in all fibrosis stages including patients with decompensated cirrhosis. Treatment was a combination of sofosbuvir 400 mg/day + daclatasvir 60 mg/day, with or without a weight-adjusted dosing of ribavirin for 12 or 24 weeks. The primary efficacy endpoint was sustained virologic response rates 12 weeks after therapy (SVR12). The primary safety endpoint was treatment withdrawal rates secondary to severe adverse events. RESULTS: A total of 111 patients were enrolled, 32.4% cirrhotics and 29.9% treatment-experienced. The global SVR12 rate was 94.6%, while the SVR12 rate in F3-4 fibrosis stage patients was 90.8% versus 100% in patients with F0-2 fibrosis (p=0.03). In cirrhotic patients, SVR12 was 100% versus 40% depending on whether ribavirin was added or not to daclatasvir/sofosbuvir (p=0.001). No other patient or treatment basal variables influenced the treatment effectiveness. No patient treatment withdrawal secondary to severe adverse events was observed. CONCLUSIONS: Daclatasvir/sofosbuvir ± ribavirin is highly effective in G3-HCV infected patients. Advanced degrees of fibrosis significantly decrease the effectiveness of this treatment, which motivates the need for the addition of ribavirin in cirrhotic patients. The regimen was safe and well tolerated.