Research advances on signaling pathways affecting sweat gland development and their involvement in the reconstitution of sweat adenoid cells in vitro / 中华烧伤杂志

The damage of sweat glands in patients with extensive deep burns results in the loss of thermoregulation, which seriously affects the quality of life of patients. At present, there are many researches on the repair of sweat gland function, but the mechanism of human sweat gland development has not been fully clarified. More and more studies have shown that the cascaded pathways of Wnt/β-catenin, ecto- dysplasin A/ectodysplasin A receptor/nuclear factor-κB, sonic hedgehog, and forkhead box transcription factor jointly affect the development of sweat glands, and it has been reported that the cascaded signaling pathways can be used to achieve the reconstruction of sweat adenoid cells in vitro. This article reviews the signaling pathways that affect the development of sweat glands and their involvement in the reconstruction of sweat adenoid cells in vitro.

Dermatite néonatale: à propos de 116 cas recueillis dans un CHU d'Abidjan / Newborns dermatitis: about 116 cases collected in a university hospital in Abidjan

Objectifs : Déterminer le profil épidémiologique des dermatoses du nouveau-né et de décrire les différents aspects cliniques des dermatoses néonatales observées. Matériel et méthodes : L'étude s'est déroulée au Centre hospitalier universitaire de Cocody (Abidjan). L'étude était transversale, à visée descriptive et analytique, réalisée sur la base d'un recrutement prospectif. Ont été inclus, les nouveau-nés ayant été vus en consultation externe ou en hospitalisationdu 4 avril 2018 au 23 août 2018 chez qui le médecin pédiatre avait observé des lésions cutanées et/ou muqueuses.Ensuite,le diagnostic était posé par le dermatologue référant de l'étude. Résultats : Pendant la période d'étude, 116 nouveau-nés ont été recensés. La moyenne d'âge était 16,86 ± 8,4 jours avec un âge médian de 19 jours. Lesex ratio (H/F) était de 1. Dans plus de la moitié (53,5%) des cas, les lésions évoluaient depuis moins de 5 jours. Une dermatose transitoire était diagnostiquée dans plus de la moitié des cas (51,7%) et dans près du tiers des cas (32,6%) une dermatose infectieuse. Les dermatoses transitoires du nouveau-né étaient dominées par la miliaire sudorale (40%), l'érythème toxique (23%), la desquamation néonatale (10,7%) et l'hyperplasie néonatale (10,7%). Les taches mongoloïdes représentaient3,3% des cas. Les dermatoses infectieuses étaient essentiellement représentées par des infections mycosiques (68,4%) et bactériennes (31,6%). Les autres dermatoses néonatales observées étaient dominées par dermite du siège (64,3%) et les nævi congénitaux (21,5%). Plus de la moitié (57,1%) des cas d'érythème toxique néonatal survenaient entre le 6e et le 10e jour de vie. L'âge moyen des patients présentant une dermatose transitoire était de 14,31 jours contre 19,41 jours pour ceux présentant les autres dermatoses. La différence observée au niveau de l'âge était statistiquement significative (p < 0,05). Conclusion: Les dermatoses néonatales sont multiples et variées. Certaines sont transitoires, ne nécessitant pas toujours de prise en charge thérapeutique. Leur diagnostic n'est pas toujours évident pour le pédiatre d'où la nécessité d'une étroite collaboration entre pédiatres et dermatologues afin d'améliorer la démarche diagnostique et parfois thérapeutique

Aims: To determine the epidemiological profile of newborn dermatitis and to describe the different clinical aspects of the observed neonatal dermatitis. Procedure: The study took place at the University Hospital of Cocody (Abidjan). The study was cross-sectional, descriptive and analytical, carried out on the basis of prospective recruitment. The study included newborns who were seen in outpatient or inpatient settings by 4 april 2018 to 23 August 2018 and in whom the pediatrician had observed cutaneous and/or mucosal lesions. The diagnosis was made with the collaboration of a dermatologist. Results: During the study period, 116 newborns were identified. The age of the patients seen in pediatrics with dermatitis varied from 1 to 28 days, with a mean of 16.86 ± 8.4 days. The median age was 19 days. The most representative age range (32.8%) was 24-28 days. The sex ratio (M/F) was 1. In almost 2/3 of the cases, the children were born at term, 29.3% were premature and 5.2% were born after term. In almost 2/3 of the cases (63.8%), the newborns had a birth weight of more than 2500 g. Only 3.4% of newborns seen in pediatric consultations were referred for a dermatitis. The age of the lesions at the time of consultation varied from 1 to 26 days, with a mean of 06.19 days ± 5.13. In more than half (53.5%) of the cases, the lesions had evolved for less than 5 days. Transient dermatitis was more frequent (51.7%), followed by infectious dermatitis (32.8%). Transient dermatitis of the newborn was dominated by sweaty miliaria (40%). Infectious dermatitis were mainly represented by mycotic (68.4%) and bacterial (31.9%) infections. Bacterial dermatitis were composed of neonatal impetigo (83.3%) and folliculitis (16.7%). In almost half of the cases (46.1%) the mycotic dermatitis were represented by candidosis intertrigo and in 38.5% of the cases there was oral candidiasis. The other neonatal dermatitis observed were dominated by diaper rash (64.3%) (Photo 2) and congenital nevi (21.5%). More than half (57.1%) of the cases of toxic erythema neonatorum occurred between days 6 and 10 of life. Nearly half (41.6%) of the cases of sudoral miliaria occurred between birth and day 5 of life. More than half (57.1%) of the cases of sebaceous hyperplasia occurred before the 5th day of life. All cases of neonatal scaling and mongoloid spots were already present between birth and day 5 of life. The mean age of patients with transient dermatitis was 14.31 days compared with 19.41 days for those with the other dermatitis. The difference in age was statistically significant (p < 0.05). The transient dermatitis predominated in male neonates while the other dermatitis predominated in females, however the difference observed at the level of sex was not statistically significant (p > 0.05). Conclusion: The diagnosis of neonatal dermatitis is not always obvious, especially on black skin where few publications have been published

A influência da idade e do genótipo sobre os níveis de cloreto do suor em uma população pediátrica com fibrose cística no estado da Bahia / The influence of age and genotype on sweat chloride levels in a pediatric population with cystic fibrosis in the state of Bahia

Introdução: a fibrose cística é a doença autossômica recessiva mais comum em populações caucasianas e a sua etiologia está associada a variantes patogênicas no gene CFTR. O teste do suor é considerado o padrão ouro para o diagnóstico dessa enfermidade. Estudos apontam que o genótipo do CFTR e a idade dos indivíduos influenciam as concentrações de cloreto no suor. Objetivos: pesquisar a correlação entre os níveis de cloreto no teste do suor e a idade ao diagnóstico de indivíduos com fibrose cística e comparar as concentrações iônicas do cloreto entre os sexos, diferentes faixas etárias e três grupos diversos de genótipos do CFTR. Metodologia: realizou-se um estudo de corte transversal, incluindo sujeitos de 0 a 20 anos, com diagnóstico confirmado de fibrose cística. Os indivíduos selecionados foram agrupados de acordo com as variáveis analisadas. Calcularam-se os valores descritivos das concentrações de íons cloreto de cada grupo. Utilizou-se o teste de Spearman para a análise da correlação entre a idade ao diagnóstico e os níveis de cloreto no suor. Resultados: 64 indivíduos foram incluídos no estudo, sendo 51,56% do sexo masculino. A mediana (Min ­ Max) da idade ao diagnóstico foi de 7 meses (1-206). Não foi observa da correlação entre a idade dos indivíduos ao diagnóstico e os níveis de cloreto no suor. As concentrações medianas de cloreto foram maiores nos escolares (106 mEq/l), no sexo feminino (102 mEq/l) e nos heterozigotos F508del/Classe I a III (108 mEq/l); e menores nos adolescentes (100 mEq/l) e nos heterozigotos F508del/Classes IV a VI (77 mEq/l). Conclusão: os níveis de cloreto no suor não apresentaram correlação com a idade dos indivíduos ao diagnóstico. A variação considerável dos níveis iônicos entre os grupos de diferentes genótipos corrobora que o teste do suor é um bom preditor da avaliação funcional do canal CFTR.

Introduction: cystic fibrosis is the most common autosomal recessive disorder in Caucasian populations and its etiology is associated with pathogenic variants in the CFTR gene. The sweat test is considered the gold standard for the diagnosis of the disease. Some studies suggest that CFTR genotype and age affect sweat chloride concentrations. Objectives: to investigate the correlation between sweat chloride levels and age at diagnosis of individuals with cystic fibrosis and to compare ionic chloride concentrations among sexes, different age groups and three distinct groups of CFTR genotypes. Methodology: a cross-sectional study was conducted, which included CF subjects from 0 to 20 years of age. The selected individuals were clustered on the variables in analysis. The description values for chloride ion concentrations in each group were calculated. The Spearman's test was used to analyze the correlation between the age at diagnosis and sweat chloride levels. Results: 64 individuals were included, 51,56% male. The median (Min ­ Max) age at diagnosis was 7 months (1-206). There was no correlation between the age at diagnosis and sweat chloride levels. The median of the chloride concentrations were higher for schoolchildren (106 mEq/l), females (102 mEq/l) and heterozygous F508del/Classes I to III (108 mEq/l), and reached the lowest values for teenagers (100 mEq/l) and heterozygous F508del/Classes IV to VI (77 mEq/l). Conclusion: sweat chloride levels did not correlate with the age of individuals at diagnosis. The substantial variation of ionic levels among groups of distinct genotypes corroborates that the sweat test is a good predictor for functional assessment of the CFTR channel.

Zika virus RNA excretion in sweat with concomitant detection in other body fluid specimens

We evaluated sweat, blood and urine specimens obtained from an ongoing cohort study in Brazil. Samples were collected at pre-established intervals after the initial rash presentation and tested for Zika virus (ZIKV) RNA presence by real-time reverse transcriptase polymerase chain reaction (rRT-PCR). From 254 participants with confirmed infection, ZIKV RNA was detected in the sweat of 46 individuals (18.1%). Sweat presented a median cycle threshold (Ct) of 34.74 [interquartile range (IQR) 33.44-36.04], comparable to plasma (Ct 35.96 - IQR 33.29-36.69) and higher than urine (Ct 30.78 - IQR 28.72-33.22). Concomitant detection with other specimens was observed in 33 (72%) of 46 participants who had a positive result in sweat. These findings represent an unusual and not yet investigated virus shedding through eccrine glands.

Chloride and sodium ion concentrations in saliva and sweat as a method to diagnose cystic fibrosis / Dosagem da concentração dos íons cloreto e sódio na saliva e no suor para o reconhecimento de pacientes com fibrose cística

Abstract Objective: Cystic fibrosis diagnosis is dependent on the chloride ion concentration in the sweat test (≥ 60 mEq/mL - recognized as the gold standard indicator for cystic fibrosis diagnosis). Moreover, the salivary glands express the CFTR protein in the same manner as sweat glands. Given this context, the objective was to verify the correlation of saliva chloride concentration and sweat chloride concentration, and between saliva sodium concentration and sweat sodium concentration, in patients with cystic fibrosis and healthy control subjects, as a tool for cystic fibrosis diagnosis. Methods: There were 160 subjects enrolled: 57/160 (35.70%) patients with cystic fibrosis and two known CFTR mutations and 103/160 (64.40%) healthy controls subjects. Saliva ion concentration was analyzed by ABL 835 Radiometer® equipment and, sweat chloride concentration and sweat sodium concentration, respectively, by manual titration using the mercurimetric procedure of Schales & Schales and flame photometry. Statistical analysis was performed by the chi-squared test, the Mann -Whitney test, and Spearman's correlation. Alpha = 0.05. Results: Patients with cystic fibrosis showed higher values of sweat chloride concentration, sweat sodium concentration, saliva chloride concentration, and saliva sodium concentration than healthy controls subjects (p-value < 0.001). The correlation between saliva chloride concentration and sweat chloride concentration showed a positive Spearman's Rho (correlation coefficient) = 0.475 (95% CI = 0.346 to 0.587). Also, the correlation between saliva sodium concentration and sweat sodium concentration showed a positive Spearman's Rho = 0.306 (95% CI = 0.158 to 0.440). Conclusions: Saliva chloride concentration and saliva sodium concentration are candidates to be used in cystic fibrosis diagnosis, mainly in cases where it is difficult to achieve the correct sweat amount, and/or CFTR mutation screening is difficult, and/or reference methods for sweat test are unavailable to implement or are not easily accessible by the general population.

Resumo Objetivo: O diagnóstico da fibrose cística depende do valor da concentração de íons de cloreto no teste do suor (≥ 60 mEq/mL - reconhecido como o indicador-padrão para o diagnóstico da doença). Além disso, as glândulas salivares expressam a proteína RTFC igualmente às glândulas sudoríparas. Nesse contexto, nosso objetivo foi verificar a correlação da concentração de cloreto na saliva e a concentração de cloreto no suor e entre a concentração de sódio na saliva e a concentração de sódio no suor em pacientes com fibrose cística e indivíduos controles saudáveis, como uma ferramenta para diagnóstico de fibrose cística. Métodos: Contamos com a participação de 160 indivíduos [57/160 (35,70%) com fibrose cística e duas mutações no gene RTFC conhecidas e 103/160 (64,40%) indivíduos controles saudáveis]. A concentração de íons na saliva foi analisada pelo equipamento ABL 835 da Radiometer® e a concentração de cloreto no suor e sódio no suor, respectivamente, por titulação manual utilizando o método mercurimétrico de Schales & Schales e fotometria de chama. A análise estatística foi realizada pelo teste qui-quadrado, pelo teste de Mann-Whitney e pela correlação de Spearman. Alpha = 0,05. Resultados: Os pacientes com fibrose cística apresentaram maiores valores na concentração de cloreto no suor, concentração de sódio no suor, concentração de cloreto na saliva e concentração de sódio na saliva do que os indivíduos-controle saudáveis (valor de p < 0,001). A correlação entre as concentrações de cloreto na saliva e cloreto no suor mostrou Rho de Spearman (coeficiente de correlação) positivo = 0,475 (IC de 95% = 0,346 a 0,587). Além disso, a correlação entre concentração de sódio na saliva e concentração de sódio no suor mostrou Rho de Spearman positivo = 0,306 (IC de 95% = 0,158 a 0,440). Conclusões: A concentração de cloreto na saliva e a concentração de sódio na saliva são candidatas a ser usadas como diagnóstico de fibrose cística, principalmente em casos em que é difícil atingir a quantidade correta de suor, e/ou o exame da mutação RTFC é difícil e/ou o método de referência para o teste do suor não se encontra disponível ou não é de fácil acesso ao público em geral.

A propósito de un caso: la niña que suda sangre: un raro caso de hematohidrosis / About a case: The girl who sweats blood: A rare case of hematohidrosis

Introducción: la hematohidrosis es una rara condición clínica donde el paciente exuda sangre. Presentación de caso: se presenta el caso de una joven de 13 años, sin antecedentes mórbidos conocidos, que fue referida por Departamento de Hematología a Salud Mental por presentar cuadro de 7 meses de evolución, caracterizado por sangrado de oídos, ojos, boca, ombligo, cabeza, mamas y dedos, ocurriendo con más frecuencia cuando cursa con períodos menstruales. Se le realizaron analíticas y estudios especializados que incluían hemograma, glicemia, perfil renal, hepático, virales, electrolitos, perfil tiroideo, uroanálisis, Factor viii, Factor vii, Factor de Von Willebrand, TP, TTP, INR, fibrinógeno y extendido de sangre periférica. Además, se realizó TAC de cráneo y radiografía de senos paranasales. Todas las pruebas estaban dentro de los parámetros de normalidad. Se inicia tratamiento con Hidroxicina 25 mg c/24 horas y clonazepam 2mg c/24 horas. Con el tratamiento farmacológico cedió el sangrado de la paciente. Conclusión: el paciente fue diagnosticado con hematohidrosis, clínicamente, por exclusión. Actualmente no hay tratamiento disponible para esta condición. La etiología es desconocida hasta la fecha. El estrés se vincula como un factor precipitante

Introduction: Hematohidrosis is a rare clinical condition where the patient exudes blood. Presentation: We present the case of a 13-year-old girl, with no known morbid history, who was referred by department of hematology to mental health for presenting a 7-month evolution, characterized by bleeding of ears, eyes, mouth, navel, head, breasts and fingers occurring more often when going through menstrual periods. Analyzes and specialized studies were performed that included hemogram, glycemia, renal, hepatic, viral, electrolyte, thyroid profile, uroanalysis, Factor viii, Factor vii, Von Willebrand Factor, TP, TTP, INR, fibrinogen and peripheral blood smear. In addition, a CT scan of the skull and x-ray of the paranasal sinuses was performed. All the tests were within normal parameters. Treatment was started with Hydroxyzine 25 mg every 24 hours and clonazepam 2mg every 24 hours. With the pharmacological treatment, the bleeding of the patient subsided. Conclusion: The patient was diagnosed with hematohidrosis clinically by exclusion. Currently, there is no treatment available for this condition. The etiology is unknown to date. Stress is linked as a precipitating factor

A case of anhidrotic ectodermal dysplasia presenting with pyrexia, atopic eczema, and food allergy

Anhidrotic ectodermal dysplasia (AED) is a rare hereditary disorder with a triad of sparse hair, dental hypoplasia, and anhidrosis. Here we report a case of AED with food allergy and atopic eczema. The patient was a 11-month-old boy admitted to our hospital with pyrexia for 2 weeks. He presented with a history of dry skin, eczema, and food allergy to egg. On clinical examination, his body temperature was 38.8°C, with dry skin and eczema almost all over the body, sparse eyebrows, and scalp hair. Laboratory investigations and physical examination did not show any evidence of infection. Radioallergosorbent test was positive to egg yolk, egg white, ovomucoid, milk, house dust, and house dust mite. As the child did not sweat despite the high fever, we performed the sweat test which revealed a total lack of sweat glands. Genetic examination revealed a mutation of the EDA gene and he was diagnosed as AED. His pyrexia improved upon cooling with ice and fan. His mother had lost 8 teeth and her sweat test demonstrated low sweating, suggestive of her being a carrier of AED. Atopy and immune deficiencies have been shown to have a higher prevalence in patients with AED. Disruption of the skin barrier in patients with AED make them more prone to allergic diseases such as atopic eczema, bronchial asthma, allergic rhinitis and food allergy. Careful assessment of the familial history is essential to differentiate AED when examining patients with pyrexia of unknown origin and comorbid allergic diseases.

Severe Hyperhidrosis in Apert Syndrome: A Case Report / 대한피부과학회지

Apert syndrome is a rare genetic disorder characterized by malformations of the skull, face, hands, and feet. We report a case of severe hyperhidrosis in a 13-month-old female infant with Apert syndrome who was born with craniosynostosis, midface hypoplasia, and syndactyly of both hands. She had a history of excessive sweating since birth and this was confirmed using the iodine-starch test. Hyperhidrosis was first reported as a key cutaneous manifestation of Apert syndrome in 1993. However, the main focus in the field of dermatology is on antibiotic-refractory acne, which serves as another cutaneous hallmark of the disease. This is the first report in the Korean literature that describes hyperhidrosis in Apert syndrome. We highlight the presentation of hyperhidrosis as a key cutaneous manifestation in Apert syndrome.

A Case of Persistent Acantholytic Dermatosis Presenting as a Chronic Single Patch / 대한피부과학회지

Transient acantholytic dermatosis, or Grover's disease, usually appears as pruritic erythematous papules and vesicles on the trunk in middle-aged men. The pathogenesis remains unclear, though sun exposure, heat, and sweating may be aggravating factors. A 58-year-old male visited our clinic for evaluation of an asymptomatic erythematous patch on the left temple that developed 40 years ago. Here, we report the rare case of Grover's disease with atypical features presenting as one large patch on the face.

Impacts of Symptom Clusters, Performance and Emotional Status on the Quality of Life of Patients with Gynecologic Cancer

PURPOSE: To determine impacts of symptom clusters, performance and emotional status on the quality of life of gynecologic cancer patients. METHODS: Subjects completed questionnaires consisting of four measurements: symptom cluster, performance and emotional status, and the quality of life. A total of 104 completed data sets were analyzed by descriptive statistics, t-test, ANOVA, Pearson's correlation coefficient, and a multiple regression analysis using the SPSS 21.0 program. RESULTS: Fatigue was identified as the most prevalent symptom (77.9%) and sweating (2.08) as the most severe and uncomfortable symptom (1.80). Six symptom clusters, performance status, anxiety and depression were negatively correlated with quality of life. Four symptom clusters were positively correlated with performance status, and six symptom clusters were positively correlated with anxiety and depression. Factors affecting quality of life were abdominal discomfort cluster (β=−0.23, p=0.005), performance status (β=−0.20, p=0.020), and depression (β=−0.42, p < 0.001). The model was statistically significant explaining 42.5% of variance (F=26.369, p < 0.001). CONCLUSION: The findings supported that symptom clusters and depression negatively influence the quality of life and need to be addressed as we are caring for patients and promoting quality of life.