Self-reported clinical features and treatment effectiveness of Papillon-Lefèvre syndrome patients from five Latin American countries: A cross-sectional online survey study.

BACKGROUND/OBJECTIVES: Most studies about Papillon-Lefèvre syndrome (PLS) are limited to case reports and patients of the same nationality. This study aimed to determine the self-reported prevalence of signs, symptoms and treatment effectiveness in PLS patients from five Latin American countries. METHODS: An online survey was conducted among adult and paediatric patients from Mexico, Argentina, Colombia and Brazil. Data were collected using multiple-choice, open-ended and image-chooser questions on demographics, signs and symptoms, perceived treatment effectiveness and quality of life. RESULTS: Seventeen patients (10 males and 7 females) aged 4-47 years were surveyed. All had palmoplantar hyperkeratosis. Other affected sites were the feet and hand dorsum (82.35%), Achilles tendon (88.24%), forearms (58.82%), legs (29.41%) and glutes (23.53%). They frequently presented hyperhidrosis and nail pitting. Four had a history of delayed umbilical cord separation. All used topical treatments, with moderate effectiveness; half used oral retinoids, perceived as highly effective. Most reported decreased quality of life and walking difficulties. CONCLUSIONS: The study's results align with prior research on PLS, but reveal new insights, including the impact on patients' quality of life and a history of delayed umbilical cord separation. These findings warrant consideration in future research and patient care.

Evaluation of quality of life (QOL) of young patients with primary hyperhidrosis (PH) before and after endoscopic thoracic sympathectomy (ETS).

BACKGROUND: Primary hyperhidrosis (PH) affects young patients and may cause emotional distress and a negative quality of life (QOL). OBJECTIVE: We sought to evaluate the QOL of children and adolescents with PH treated by endoscopic thoracic sympathectomy. METHODS: A study of 220 patients was performed, based on submitted QOL questionnaires from their first consultation. Patients were evaluated within 1 week and 24 months after surgery. RESULTS: Before endoscopic thoracic sympathectomy, the QOL in relation to PH was declared very poor by 141 patients, and poor by the remaining 79 (P = .552). Postoperative cure was reported in 100% of palmar and axillary PH cases, and in 91.7% of facial PH. After 24 months, the QOL was described as much better by 212 patients, a little better by 6 patients, and 2 patients reported no change. LIMITATIONS: Convenience sampling was used and patients were taken from private practice only, raising the possibility of bias in gathering the data. CONCLUSION: Onset of PH symptoms was mainly before the age of 10 years and substantially affected daily activities. Endoscopic thoracic sympathectomy cured PH and promoted significant improvement in the QOL of these young patients.

Parental report of dental pain and discomfort in preschool children is associated with sleep disorders: a cross-sectional study in Brazilian families.

PURPOSE: To evaluate the prevalence of sleep disorders in Brazilian preschool children and its associations with parental report of dental pain and discomfort. METHODS: This cross-sectional study involved 604 Brazilian preschoolers (4-5 years old). Sleep disorders (SD) and the parental report of dental pain and discomfort (DPD) were evaluated using the Brazilian versions of the Sleep Disturbance Scale for Children (SDSC) and the Dental Discomfort Questionnaire (DDQ-B), respectively. Bivariate and multivariate Poisson regression analyses with robust variance were performed to analyze the association between SDSC and DP. RESULTS: Prevalence of SD ranged from 7 to 21%. 7.9% of the children had DPD indicating the need for more invasive dental procedures (DDQ-B ≥ 5). Significant associations were found between DPD and the following SDSC domains: sleep hyperhidrosis (p = 0.024; PRa = 1.38; 95% CI: 1.04-1.83), disorders of initiating and maintaining sleep (p < 0.001; PRa = 1.41; 95% CI: 1.15-1.73), parasomnias (p < 0.001; PRa = 1.82; 95% CI: 1.39-2.37), and sleep-wake transition disorders (p = 0.018; PRa = 1.28; 95% CI: 1.04-1.58). Children with higher prevalence of DPD presented 20% higher prevalence of SD than children lower prevalence of DPD (p = 0.039; PRa = 1.20; 95% CI: 1.01-1.44). CONCLUSION: Preschool children with higher prevalence of DPD are more likely to have SD, such as hyperhidrosis, disorders of initiating and maintaining sleep, parasomnias, and sleep-wake transition.

Hyperhidrosis in sleep disorders - A narrative review of mechanisms and clinical significance.

Hyperhidrosis is characterized by excessive sweating beyond thermoregulatory needs that affects patients' quality of life. It results from an excessive stimulation of eccrine sweat glands in the skin by the sympathetic nervous system. Hyperhidrosis may be primary or secondary to an underlying cause. Nocturnal hyperhidrosis is associated with different sleep disorders, such as obstructive sleep apnea, insomnia, restless legs syndrome/periodic limb movement during sleep and narcolepsy. The major cause of the hyperhidrosis is sympathetic overactivity and, in the case of narcolepsy type 1, orexin deficiency may also contribute. In this narrative review, we will provide an outline of the possible mechanisms underlying sudomotor dysfunction and the resulting nocturnal hyperhidrosis in these different sleep disorders and explore its clinical relevance.

Nationwide Cross-sectional Analysis of Endoscopic Thoracic Sympathectomy to Treat Hyperhidrosis Over 12 years in Brazil: Epidemiology, Costs, and Mortality.

OBJECTIVE: To analyze the number of endoscopic thoracic sympathectomies performed to treat hyperhidrosis in the Universal Public Health System of Brazil, the government reimbursements, and the in-hospital mortality rates. BACKGROUND: Even though endoscopic thoracic sympathectomy has been widely performed for the definitive treatment of hyperhidrosis, no series reported mortality and there are no population-based studies evaluating its costs or its mortality rate. METHODS: Data referring to endoscopic thoracic sympathectomy to treat hyperhidrosis between 2008 and 2019 were extracted from the database of the Brazilian Public Health System, which insures more than 160 million inhabitants. RESULTS: Thirteen thousand two hundred one endoscopic thoracic sympathectomies to treat hyperhidrosis were performed from 2008 to 2019, with a rate of 68.44 procedures per 10 million inhabitants per year. There were 6 in-hospital deaths during the whole period, representing a mortality rate of 0.045%. The total expended throughout the years was U$ 6,767,825.14, with an average of U$ 512.68 per patient. CONCLUSIONS: We observed a rate of 68.44 thoracoscopic sympathectomies for hyperhidrosis' treatment per 10 million inhabitants per year. The inhospital mortality rate was very low, 0.045%, though not nil. To our knowledge, no published series is larger than ours and we are the first authors to formally report deaths after endoscopic thoracic sympathectomies to treat hyperhidrosis. Moreover, there is no other population-based study addressing costs and mortality rates of every endoscopic thoracic sympathectomy for the treatment of any site of hyperhidrosis in a given period.

Population-based analysis of the epidemiology of the surgical correction of hyperhidrosis in 1,216 patients over 11 years: a cross-sectional study.

BACKGROUND: Endoscopic thoracic sympathectomy is the definitive surgical treatment for hyperhidrosis and a nationwide study has suggested that cultural and socioeconomic factors play a role in the numbers of operations performed. Thus, there is a need to evaluate local data in order to understand the local epidemiology and trends in hyperhidrosis treatment. OBJECTIVE: To study the epidemiology of sympathectomy for treating hyperhidrosis in São Paulo, the largest city in Brazil. DESIGN AND SETTING: Population-based retrospective cross-sectional study. METHODS: Data on sympathectomies for treating hyperhidrosis between 2008 and 2018 were assessed from the database of the Municipal Health Department of São Paulo, Brazil. RESULTS: 65.29% of the patients were female, 66.2% were aged between 20 and 39 years and 37.59% had registered with addresses outside São Paulo. 1,216 procedures were performed in the city of São Paulo from 2008 to 2018, and 78.45% of them were in only two public hospitals. The number of procedures significantly declined over the years (P = 0.001). 71.63% of the procedures were associated with 2-3 days of hospital stay, only 78 intensive care unit days were billed and we did not observe any intra-hospital death. CONCLUSION: The profile of patients operated on in São Paulo (young women) is similar to that described in other populations. Sympathectomy is a very safe procedure, with no mortality in our series. There was a decreasing trend in the number of surgeries over the years.

La sudoración compensatoria como efecto secundario en la cirugía de la hiperhidrosis palmar primaria / Compensatory Sweating as a Side Effect of Surgical Treatment of Primary Palmar Hyperhidrosis

Introducción: La sudoración compensatoria es un efecto secundario de la simpaticotomía videotoracoscópica, que tiene una alta incidencia y puede provocar insatisfacción en los pacientes operados. Objetivo: Determinar el comportamiento de la sudoración compensatoria en los pacientes en que se les realizó una simpaticotomía videotoracoscópica por hiperhidrosis palmar. Métodos: Se realizó un estudio retrospectivo, transversal y descriptivo de 42 pacientes que tras la cirugía por hiperhidrosis palmar presentaron sudor compensatorio. Se analizó la incidencia, localización, severidad, afectación de la calidad de vida y nivel de satisfacción. Resultados: Predominó la sudoración compensatoria ligera, la localización en la espalda y el abdomen. Hubo un alto nivel de tolerancia, con solo un paciente insatisfecho y un 100 por ciento de mejoría de la calidad de vida. Conclusiones: La sudoración compensatoria no influyó negativamente en la calidad de vida de los pacientes(AU)

Introduction: Compensatory sweating is a side effect of videothoracoscopic sympathectomy. It presents high incidence and may cause dissatisfaction to operated patients. Objective: To determine the characteristics of compensatory sweating in patients who underwent videothoracoscopic sympathectomy for palmar hyperhidrosis. Methods: A retrospective, cross-sectional and descriptive study was carried out with 42 patients who presented compensatory sweating after surgery for palmar hyperhidrosis. Incidence, location, severity, change in quality of life and level of satisfaction were analyzed. Results: Light compensatory sweating predominated, with location on back and abdomen. There was a high level of tolerance, with only one dissatisfied patient and 100 percent of improvement in quality of life. Conclusions: Compensatory sweating did not influence negatively the patients' quality of life(AU)

Cohort study on 20 years' experience of bilateral video-assisted thoracic sympathectomy (VATS) for treatment of hyperhidrosis in 2431 patients.

BACKGROUND: Primary hyperhidrosis is a condition characterized by excessive sweating, inconsistent with the needs for thermoregulation. OBJECTIVE: To assess the effectiveness and the change in the quality of life of patients undergoing bilateral VATS (video-assisted thoracoscopic sympathectomy) for treatment of hyperhidrosis, in a large case series. DESIGN AND SETTING: Cohort study conducted in a tertiary hospital specializing in hyperhidrosis located in São Paulo, Brazil. METHODS: A total of 2,431 patients who underwent surgery consisting of bilateral video-assisted thoracoscopic sympathectomy between January 2000 and February 2017 were retrospectively assessed in an outpatient clinic specializing in hyperhidrosis. The patients underwent clinical and quality of life assessments on two occasions: firstly, prior to surgery, and subsequently, one month after the operation. The presence or absence of compensatory hyperhidrosis (CH) and general satisfaction after the first postoperative month were also evaluated. RESULTS: All the patients operated had poor or very poor quality of life before surgery. In the postoperative period, an improvement in the quality of life was observed in more than 90% of the patients. Only 10.7% of the patients did not present CH, and severe CH occurred in 22.1% of the patients in this sample. CONCLUSION: Bilateral VATS is a therapeutic method that decreases the degree of sweating more than 90% of patients with palmar and axillary hyperhidrosis. It improves the quality of life for more than 90% of the patients, at the expense of development of CH in approximately 90% of the patients, but not intensely.

Unexpected improvement of hyperhidrosis with cannabidiol.

Hyperhidrosis is characterized by excessive sweating and it affects almost 5% of the population. The affected age group is wide, and it can affect from children to elderlies. There are two types of hyperhidrosis: generalized and focal. Treatment depends on the symptoms presented. In more severe cases, radiofrequency sympatholysis and bilateral thoracic sympathectomy are the options. However, recurrence is possible or the postoperative appearance of conditions called compensatory hyperhidrosis or reflex hyperhidrosis. We describe two cases of patients treated with Cannabidiol who had significant and unexpected improvement of hyperhidrosis. The first patient received Cannabidiol specific for public presentations at work, and the second patient had a diagnosis of autism spectrum disorder. The hyperhidrosis improved in both patients immediately after using Cannabidiol.

Cohort study on 20 years' experience of bilateral video-assisted thoracic sympathectomy (VATS) for treatment of hyperhidrosis in 2431 patients

Abstract BACKGROUND: Primary hyperhidrosis is a condition characterized by excessive sweating, inconsistent with the needs for thermoregulation. OBJECTIVE: To assess the effectiveness and the change in the quality of life of patients undergoing bilateral VATS (video-assisted thoracoscopic sympathectomy) for treatment of hyperhidrosis, in a large case series. DESIGN AND SETTING: Cohort study conducted in a tertiary hospital specializing in hyperhidrosis located in São Paulo, Brazil. METHODS: A total of 2,431 patients who underwent surgery consisting of bilateral video-assisted thoracoscopic sympathectomy between January 2000 and February 2017 were retrospectively assessed in an outpatient clinic specializing in hyperhidrosis. The patients underwent clinical and quality of life assessments on two occasions: firstly, prior to surgery, and subsequently, one month after the operation. The presence or absence of compensatory hyperhidrosis (CH) and general satisfaction after the first postoperative month were also evaluated. RESULTS: All the patients operated had poor or very poor quality of life before surgery. In the postoperative period, an improvement in the quality of life was observed in more than 90% of the patients. Only 10.7% of the patients did not present CH, and severe CH occurred in 22.1% of the patients in this sample. CONCLUSION: Bilateral VATS is a therapeutic method that decreases the degree of sweating more than 90% of patients with palmar and axillary hyperhidrosis. It improves the quality of life for more than 90% of the patients, at the expense of development of CH in approximately 90% of the patients, but not intensely.

Unexpected improvement of hyperhidrosis with cannabidiol

ABSTRACT Hyperhidrosis is characterized by excessive sweating and it affects almost 5% of the population. The affected age group is wide, and it can affect from children to elderlies. There are two types of hyperhidrosis: generalized and focal. Treatment depends on the symptoms presented. In more severe cases, radiofrequency sympatholysis and bilateral thoracic sympathectomy are the options. However, recurrence is possible or the postoperative appearance of conditions called compensatory hyperhidrosis or reflex hyperhidrosis. We describe two cases of patients treated with Cannabidiol who had significant and unexpected improvement of hyperhidrosis. The first patient received Cannabidiol specific for public presentations at work, and the second patient had a diagnosis of autism spectrum disorder. The hyperhidrosis improved in both patients immediately after using Cannabidiol.

Calidad de vida luego de simpaticotomía toracoscópica por hiperhidrosis focal primaria / Quality of life after thoracoscopic sympathectomy for primary focal hyperhidrosis

RESUMEN Antecedentes: la simpaticotomía toracoscópica demostró ser una cirugía segura para el tratamiento de la hiperhidrosis focal primaria (HFP); sin embargo, la calidad de vida no es totalmente satisfactoria en algunos pacientes, teniendo en cuenta la sudoración compensatoria como principal efecto adverso. Objetivo: evaluar la calidad de vida de los pacientes operados por HFP mediante simpaticotomía toracoscópica utilizando una encuesta anónima posoperatoria. Material y métodos: se incluyó una serie consecutiva de pacientes operados de simpaticotomía toracoscópica entre agosto de 2016 y agosto de 2019. Se excluyeron pacientes que no respondieron a la encuesta de calidad de vida telefónica o cuyo tiempo de seguimiento fue menor de 6 meses. Se evaluaron variables clínicas, quirúrgicas y posoperatorias. Resultados: durante este período se operaron 61 pacientes; del total de la muestra se excluyeron 12 pacientes. El promedio de edad fue 28,9 años. Todos tenían hiperhidrosis primaria palmar y/o axilar moderada o grave, asociadas o no a rubor facial o hiperhidrosis plantar. La encuesta fue respondida por 49 pacientes; de esta observamos una resolución total de la HFP en 32 pacientes (65,3%) y parcial en 17 pacientes (34,6%), con un porcentaje de sudoración compensatoria del 61% (30 pacientes), de los cuales a 4 (8%) la sudoración compensatoria les alteró su calidad de vida, provocando malestar e insatisfacción, la mayoría transitorios. Con un tiempo promedio de 36 meses (6-72), el 97,9% (48 pacientes) sí recomendarían realizar el procedimiento. Conclusión: la simpaticotomía toracoscópica bilateral continúa siendo el tratamiento más eficaz para HFP. El nivel de satisfacción de los pacientes operados fue elevado. Si bien la sudoración compensatoria fue el efecto adverso más frecuente, generalmente se presentó de manera leve y transitoria.

ABSTRACT Background: Thoracoscopic sympathectomy demonstrated to be a safe surgical procedure for the management of primary focal hyperhidrosis (PFH); however, some patients are not completely satisfied with their quality of life as compensatory sweating is the main adverse event. Objective: The aim of this study was to evaluate the quality of life of patients with PFH undergoing thoracoscopic sympathectomy using an anonymous postoperative survey. Material and methods: Consecutive patients undergoing thoracoscopic sympathectomy between August 2016 and August 2019 were included. Patients who did not respond the telephone survey about their quality of life or who had been followed up for < 6 months. Clinical, intraoperative and postoperative variables were analyzed. Results: During this period 61 patients were operated on and 12 of them were excluded. Mean age was 28.9 years. All the patients presented moderate or severe primary palmar and/or axillary hyperhidrosis with or without facial flushing or plantar hyperhidrosis. Forty-nine patients responded the survey. Surgery completely solved PFH in 32 patients (65.3%) while 17 patients (34.6%) achieved a partial relief; 61% (30 patients) developed compensatory sweating affecting the quality of life in 4 (8%) causing transient discomfort and dissatisfaction in most cases. After a mean follow-up of 36 months (6-72), 97.9% (48 patients) recommended the procedure. Conclusion: Bilateral thoracoscopic sympathectomy is still the most efficient treatment for PFH with high level of satisfaction among the patients operated on. Compensatory sweating was the most common adverse effect and is mild and transient in most cases.

IS THERE NEURAL AND FUNCTIONAL RECOVERY AFTER CLIP REMOVAL IN CERVICAL EXPERIMENTAL SYMPATHECTOMY?

BACKGROUND: The surgical treatment of hyperhidrosis by thoracic sympathectomy has brought, in addition to symptomatic relief for many, its main adverse effect: compensatory or reflex sweating. The clipping technique in place of the sympathetic nerve section gave rise to the hope of reversibility, but the positive results showed to be quite divergent, evidencing the academic deficiency regarding the study of this phenomenon. AIM: To observe micro and macroscopic damage caused by the polymer clip on sympathetic nerve of rabbits seven days after their clipping and the findings after three weeks of clip removal. METHOD: In this experimental study, 20 rabbits were divided into two groups of 10, group 1 (clipping) and group 2 (de-clipping). The right cervical sympathetic nerve of all animals was clamped with polymeric clip, and in group 2 the nerve was unclipped seven days later. Group 1 rabbits were induced to death on the 7th postoperative day, and group 2 on the 21st after removal of the polymer clip. Macroscopic variables were: clip appearance, presence of discontinuity lesion, infection and adhesions around the nerve. H&E was used in the evaluation of the phases and degree of the inflammatory process and presence of necrosis, and picrosirius red F3BA for quantification of collagen. RESULTS: The cervical sympathetic nerve was intact, without necrosis or infection in all animals of the experiment; there were adhesions in both groups, being minimal in eight animals of each group and moderate or intense in two; the clip was completely closed in all animals at the 7th postoperative day; the inflammatory process shown was chronic, with monomorphonuclear predominance. There was no significant difference between groups regarding the intensity the inflammatory process, but the amount of collagen type I and type III was significantly higher in group 2. CONCLUSIONS: The injury caused by the polymer clip on the sympathetic nerve may be reversible, allowing functional return in the areas involved in the simulated cervical sympathectomy. Clipping of the cervical sympathetic nerve using a polymer clip does not cause discontinuity injury.

Perspectives of bilateral thoracic sympathectomy for treatment of heart failure.

Surgical neuromodulation therapies are still considered a last resort when standard therapies have failed for patients with progressive heart failure (HF). Although a number of experimental studies have provided robust evidence of its effectiveness, the lack of strong clinical evidence discourages practitioners. Thoracic unilateral sympathectomy has been extensively studied and has failed to show significant clinical improvement in HF patients. Most recently, bilateral sympathectomy effect was associated with a high degree of success in HF models, opening the perspective to be investigated in randomized controlled clinical trials. In addition, a series of clinical trials showed that bilateral sympathectomy was associated with a decreased risk of sudden death, which is an important outcome in patients with HF. These aspects indicates that bilateral sympathectomy could be an important alternative in the treatment of HF wherein pharmacological treatment barely reaches the target dose.

Thoracic sympathectomy for the treatment of primary axillary hyperhidrosis: systematic review and proportional meta-analysis.

INTRODUCTION: Primary hyperhidrosis is a disorder that involves excessive sweat production, which has a negative impact on the quality of life. OBJECTIVE: To evaluate the effectiveness and safety of video-assisted thoracoscopic sympathectomy (VATS) for treating primary axillary hyperhidrosis (PAH) and determine which level of ganglion resection offers the best outcome. METHOD: This was a systematic review and proportional meta-analysis of observational studies. The result was evaluated for satisfaction, control of symptoms, compensatory sweating and complications. A subgroup analysis was performed to compare the sympathetic trunk resection at high and low levels. RESULTS: Thirteen studies were selected with a total of 1463 patients. The satisfaction rate was 92% (95% CI = 88-95%, I2=47.5%), the symptom control rate was 96% (95% CI = 93-99%, I2=48.2%), and the presence of compensatory sweating could not be assessed because of high heterogeneity among studies. The complications were rare. CONCLUSION: This review demonstrated that thoracic sympathectomy by VATS is a viable and safe option for the treatment of PAH. There was no difference between high and lower levels of resection. However, the estimation of the effect is quite uncertain because the quality of evidence was extremely low.Key messagePure axillary hyperhidrosis has great potential to compromise quality of life.Surgery should be indicated only when clinical treatment fails.Thoracic sympathectomy by video-assisted thoracoscopy is a viable and safe option for the treatment of primary axillary hyperhidrosis.

Symptoms of anxiety and depression in patients with primary hyperhidrosis and its association with the result of clinical treatment with oxybutynin.

OBJECTIVES: Studies have identified correlations between the psychological characteristics of individuals with primary hyperhidrosis (HH), the degree of sweating, and the quality of life (QoL). This study aimed to evaluate the prevalence of anxiety and depression symptoms in patients with HH before and after oxybutynin treatment. METHODS: Data were collected from 81 patients. Palmar or axillary HH was the most frequent complaint (84.0%). All patients were evaluated before the medication was prescribed and after five weeks of treatment. The Beck Depression Inventory and Beck Anxiety Inventory were used to evaluate depression and anxiety. RESULTS: Improvement in HH occurred in 58 patients (71.6%), but there was no improvement in 23 patients (28.4%). The QoL before treatment in all patients was either "poor" or "very poor." Patients who experienced improvement in sweating rates also experienced a greater improvement in QoL than patients who did not experience improvement in sweating at the main site (87.9% vs. 34.7%) (p<0.001). A total of 19.7% of patients showed an improvement in their level of depression, and a total of 46.9% of patients exhibited improvements in their level of anxiety. A significant correlation was observed between sweating and anxiety (p=0.015). CONCLUSION: Patients with HH who experienced improvements in sweating immediately after treatment with oxybutynin exhibited small improvements in their levels of depression and significant improvements in their levels of anxiety and QoL.