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Acta Dermato-venereologica Jun 2024The MDHHgermany registry was initiated to characterize the "real-life" situation of affected individuals with Darier's disease (DD; Morbus Darier, MD) and Hailey-Hailey...
The MDHHgermany registry was initiated to characterize the "real-life" situation of affected individuals with Darier's disease (DD; Morbus Darier, MD) and Hailey-Hailey disease (HH), including their treatment and healthcare. To gain deeper insights into medical care of patients with DD, various aspects such as demographics, subjective symptoms, patient satisfaction with medical care, past and current therapies were explored. Patients with diagnosed DD were included. Subjective symptoms such as itch, pain and burning sensation were assessed. Individual therapy goals were recorded and patients assessed previous/current therapies along with satisfaction of medical care and treatment. A total of 55 patients were recruited; 47 patients were eligible for the analysis. Pruritus was rated the most bothersome symptom. Some 42.6% had not received systemic treatment so far or systemic therapies were rated ineffective (32.6%). Most commonly oral retinoids were prescribed, followed by corticosteroids. Patient satisfaction with medical care and treatment proved to be mediocre. This "real-life" data show an alarming unmet need regarding patients' satisfaction with medical care and treatment, evidenced by the reported lack of disease control. Further studies and interventions are needed to improve the spectrum of available therapies. MDHHgermany provides a foundational platform for future clinical trials, epidemiological studies, and pathophysiological analyses.
Topics: Humans; Registries; Darier Disease; Male; Female; Germany; Patient Satisfaction; Middle Aged; Aged; Adult; Treatment Outcome; Health Services Needs and Demand; Pemphigus, Benign Familial; Pruritus; Needs Assessment; Adrenal Cortex Hormones; Retinoids
PubMed: 38860622
DOI: 10.2340/actadv.v104.19663 -
International Journal of Dermatology Jun 2024
PubMed: 38858836
DOI: 10.1111/ijd.17310 -
International Journal of Dermatology Jun 2024
PubMed: 38856051
DOI: 10.1111/ijd.17318 -
International Journal of Dermatology Jun 2024The current incidence of chronic kidney disease-associated pruritus (CKD-aP) in patients with end-stage renal disease (ESRD) is approximately 70%, especially in those... (Review)
Review
The current incidence of chronic kidney disease-associated pruritus (CKD-aP) in patients with end-stage renal disease (ESRD) is approximately 70%, especially in those receiving dialysis, which negatively affects their work and private lives. The CKD-aP pathogenesis remains unclear, but uremic toxin accumulation, histamine release, and opioid imbalance have been suggested to lead to CKD-aP. Current therapeutic approaches, such as opioid receptor modulators, antihistamines, and ultraviolet B irradiation, are associated with some limitations and adverse effects. The skin barrier is the first defense in preventing external injury to the body. Patients with chronic kidney disease often experience itch due to the damaged skin barrier and reduced secretion of sweat and secretion from sebaceous glands. Surprisingly, skin barrier-repairing agents repair the skin barrier and inhibit the release of inflammatory cytokines, maintain skin immunity, and ameliorate the micro-inflammatory status of afferent nerve fibers. Here, we summarize the epidemiology, pathogenesis, and treatment status of CKD-aP and explore the possibility of skin barrier repair in CKD-aP treatment.
PubMed: 38855995
DOI: 10.1111/ijd.17254 -
Experimental Dermatology Jun 2024Itchy skin or pruritus is a common cutaneous symptom that causes an urge to scratch, and the role of interleukins (IL) in itchy skin has been widely studied. IL-4 and...
Itchy skin or pruritus is a common cutaneous symptom that causes an urge to scratch, and the role of interleukins (IL) in itchy skin has been widely studied. IL-4 and IL-13 are known to induce chronic itch. Similarly, the direct role of IL-31 in inducing itch has been demonstrated in clinical situations such as atopic dermatitis and prurigo nodularis. Moreover, IL-4 receptor α antibodies (dupilumab) and IL-31 receptor A antibodies (nemolizumab) inhibit pruritus. However, the interplay between these ILs in pruritus remains unclear. Therefore, we investigated the reciprocal effects of these cytokines on pruritus in mice. The intradermal administration of IL-31 induced itch-associated scratching behaviour in a dose-dependent manner. Interestingly, the amount of IL-31 and IL-4/IL-13, co-administration or 30 min pre-administration of IL-4/IL-13 and intradermal or intravenous pre-administration of IL-4 did not affect IL-31-induced itch-associated scratching behaviour when it was observed for 30 min, 2 h, 24 h or 48 h. Pre-administration of neutralising antibodies against IL-4 and IL-13 also did not affect IL-31-induced itch-associated scratching behaviour. These results suggest that IL-31 can induce itching independently of IL-4 and IL-13 in vivo.
Topics: Animals; Pruritus; Interleukin-13; Interleukin-4; Mice; Interleukins; Behavior, Animal; Male; Antibodies, Monoclonal, Humanized
PubMed: 38855893
DOI: 10.1111/exd.15115 -
Journal of Clinical Anesthesia Jun 2024The mid point-transverse process to pleura block (MTPB) is a new variant of thoracic paravertebral block (TPVB). This study aimed to compare TPVB and MTPB with respect...
Ultrasound-guided mid point-transverse process to pleura block versus thoracic paravertebral block in pediatric open-heart surgery: A randomized controlled non-inferiority study.
STUDY OBJECTIVE
The mid point-transverse process to pleura block (MTPB) is a new variant of thoracic paravertebral block (TPVB). This study aimed to compare TPVB and MTPB with respect to intraoperative attenuation of the hemodynamic stress response to surgery and postoperative analgesia in pediatric open heart surgery with midline sternotomy.
DESIGN
A single-center, randomized, controlled, double-blind, non-inferiority study.
SETTING
Tertiary care children's university hospital.
PATIENTS
We recruited 83 children aged 2-12 years of both sexes with American Society of Anesthesiologists (ASA) physical status class II who were scheduled for elective open cardiac surgeries with midline sternotomy for the repair of simple noncyanotic congenital heart defects.
INTERVENTIONS
Eligible participants were randomized into either the TPVB or MTPB groups at a ratio of 1:1. In the TPVB group, patients were bilaterally injected with 0.4 ml/kg of 0.25% bupivacaine in the paravertebral space at T4 and T5. In the MTPB group, patients were bilaterally injected with 0.4 ml/kg of 0.25% bupivacaine mid-transverse process and pleura just posterior to superior costotransverse ligament at the level of T4 and T5.
MEASUREMENTS
The primary outcome was the hemodynamic responses to sternotomy incision, including heart rate (HR) and invasive mean arterial pressure (MAP), recorded before and after the induction of anesthesia, after skin incision, after sternotomy, 15 min after cardiopulmonary bypass (CPB), and after the closure of the sternum. The secondary outcomes were time needed to perform the bilateral block, intraoperative fentanyl consumption, postoperative fentanyl consumption, modified objective pain score (MOPS) measured at 1, 2, 6, 12, 18, and 24 h after extubation, extubation time, intensive care unit (ICU) discharge time, and the incidence of non-surgical complications (postoperative pruritus, postoperative vomiting, pneumothorax, hematoma or local anesthetic toxicity).
MAIN RESULTS
There were no significant differences in HR and MAP in the TPVB group compared with the MTPB group at the following time points: baseline, after induction, after skin incision, after sternotomy, 15 min after CPB, and after sternal closure. Intergroup comparisons of HR and MAP did not reveal significant differences between the groups. The median (IQR) time needed to perform bilateral MTPB (7[6-8] min) was significantly (p < 0.001) shorter than that of TPVB (12[10-13] min). Intraoperative fentanyl consumption and fentanyl consumption in the first postoperative 24 h after extubation were similar in the TPVB and MTPB groups (4[2-4] vs 4[2-4] and 4.66 ± 0.649 vs 4.88 ± 1.082 μg/kg), respectively. Extubation time and ICU discharge time were comparable in the TPVB and MTPB groups (2[1-3] vs 2[1-3] h and 21.2 ± 2.5 vs 20.8 ± 2.6 h), respectively. Measurements of MOPS pain scores at 1, 2, 6, 12, 18, and 24 h after extubation were similar in both groups. The incidence of nonsurgical complications was similar in both groups.
CONCLUSIONS
MTPB is non-inferior to TPVB in attenuating the intraoperative hemodynamic stress response to noxious surgical stimuli and in reducing perioperative opioid consumption, extubation time, and ICU discharge time. Moreover, MTPB is technically easier than TPVB and requires less time to perform. Clinical trial registration number The clinical trial registration was prospectively performed at the Pan African Clinical Trials Registry (PACTR202204901612169, approval date 01/04/2022, URL https://pactr.samrc.ac.za/TrialDisplay.aspx?TrialID=22602).
PubMed: 38852396
DOI: 10.1016/j.jclinane.2024.111507 -
The Veterinary Clinics of North... Jun 2024Allergy to insects is the most common skin allergy in horses. Pruritus in affected patients can be extreme. Face, ears, mane, and tail area are commonly affected areas.... (Review)
Review
Allergy to insects is the most common skin allergy in horses. Pruritus in affected patients can be extreme. Face, ears, mane, and tail area are commonly affected areas. Diagnosis of insect bite hypersensitivity (IBH) is clinical and is based on history, clinical signs, and response to repellents. Allergy tests are not to be used for diagnostic purposes. Currently, there is no specific treatment for IBH other than insect avoidance, treatment of secondary infections, and symptomatic relief of pruritus. Many allergic horses become also sensitized to pollens. For these patients, allergen specific immunotherapy is beneficial.
PubMed: 38852012
DOI: 10.1016/j.cveq.2024.05.002 -
Journal of Hepatology May 2024Genetic cholestatic liver diseases are caused by (often rare) mutations in a multitude of different genes. While these diseases differ in pathobiology, clinical...
Genetic cholestatic liver diseases are caused by (often rare) mutations in a multitude of different genes. While these diseases differ in pathobiology, clinical presentation and prognosis, they do have several commonalities due to their cholestatic nature. These Clinical Practice Guidelines (CPGs) offer a general approach to genetic testing and management of cholestatic pruritus, while exploring diagnostic and treatment approaches for a subset of genetic cholestatic liver diseases in depth. An expert panel appointed by the European Association for the Study of the Liver has created recommendations regarding diagnosis and treatment, based on the best evidence currently available in the fields of paediatric and adult hepatology, as well as genetics. The management of these diseases generally takes place in a tertiary referral centre, in order to provide up-to-date approaches and expertise. These CPGs are intended to support hepatologists (for paediatric and adult patients), residents and other healthcare professionals involved in the management of these patients with concrete recommendations based on currently available evidence or, if not available, on expert opinion.
PubMed: 38851996
DOI: 10.1016/j.jhep.2024.04.006 -
Farmacia Hospitalaria : Organo Oficial... Jun 2024Topical rapamycin is the pharmacological treatment of choice for facial angiofibromas in rare tuberous sclerosis disease. A new, more advanced, and complex formula was...
AIM
Topical rapamycin is the pharmacological treatment of choice for facial angiofibromas in rare tuberous sclerosis disease. A new, more advanced, and complex formula was developed in our pharmacy service: rapamycin 0.4% liposomal formulation, with better organoleptic characteristics and a more favorable release profile of the active ingredient. The purpose of this study is to evaluate the effectiveness and safety of liposomal topical rapamycin for the treatment of facial injuries in this rare disease.
METHOD
This was an observational, prospective, and multicenter study. Effectiveness was evaluated mainly through facial angiofibromas severity index (FASI), investigator's global assessment (IGA) scores, and dermatology life quality index (DLQI) questionnaire. To assess the safety profile of rapamycin, adverse reactions were reported, and blood tests and blood rapamycin levels were performed during treatment.
RESULTS
Eleven patients were included, of which 8/11 (73%) patients obtained successful treatment according to FASI and IGA scores after 24 weeks of treatment. Statistical analysis demonstrated a significant improvement (p<.05) in FASI and IGA scores, erythema, and FA size after treatment with rapamycin liposomal formulation (FASI before treatment, median (interquartile range): 6.0 (2.0), FASI after treatment: 3.5 (2.0), p=.0063). Five patients also improved their quality of life after treatment. Regarding safety profile of rapamycin, the most common adverse reaction was mild pruritus and 2 patients reported erythema, who discontinued treatment prematurely. All hematological tests were normal, and blood rapamycin levels were undetectable.
CONCLUSIONS
After galenic improvements and clinical evaluations, the rapamycin liposomal formulation proved to be effective and safe for this therapeutic indication. This new formulation was included as a magistral formula in our hospital pharmacy service, now accessible for prescribing by dermatologists. Drug development in hospital pharmacy is often the only pharmacological alternative available to treat the symptoms of rare diseases, when treatment options are limited or inadequate.
PubMed: 38851908
DOI: 10.1016/j.farma.2024.04.023