Impact of different automated peritoneal dialysis modalities on the inflammatory profile of elderly patients with chronic kidney disease / Impacto das diferentes modalidades de diálise peritoneal automatizada sobre o perfil inflamatório de idosos portadores de doença renal crônica

Abstract Introduction: Chronic kidney disease, more prevalent in the elderly, is considered a public health issue worldwide. Objective: To evaluate the impact of automated, peritoneal dialysis modalities, intermittent and continuous, on the inflammatory profile of elderly people with chronic kidney disease. Methods: Prospective, cross-sectional and analytical study carried out in a dialysis clinic in Brasília - Brazil, with 74 elderly people aged 60 years or older. The patients underwent rapid Peritoneal Equilibration Test, clinical assessment, blood collection for biochemical and cytokine assessments, interleukin 6 and transforming growth factor beta 1, and answered a quality-of-life questionnaire (KDQOL-SF36). We used a 5% significance level for data analysis, associations and correlations. Results: Patients in the continuous modality had higher serum values of transforming growth factor beta 1 than those in the intermittent modality, which had higher peritoneal transforming growth factor beta 1, age and residual renal function than those in continuous mode. Interleukin 6 dosage in the peritoneum was associated with age, while serum IL-6 was associated with IL-6 in the peritoneum, time on dialysis and age. There was no association between the modality and the presence of diabetes, blood volume or nutritional status. Both modalities enable good adaptation to the dialysis treatment. Conclusion: Inflammation in automated peritoneal dialysis is mainly associated with low residual renal function, advanced age and longer time on therapy, and not to the type of dialysis performed.

Resumo Introdução: A doença renal crônica, mais prevalente em idosos, é considerada um problema de saúde pública em todo o mundo. Objetivo: Avaliar o impacto das modalidades de diálise peritoneal automatizada, intermitente e contínua, no perfil inflamatório de idosos renais crônicos. Métodos: Estudo prospectivo, transversal e analítico realizado em uma clínica de diálise em Brasília, com 74 idosos com idade igual ou maior que 60 anos. Os pacientes foram submetidos ao Teste de Equilíbrio Peritoneal rápido, avaliação clínica, coleta de sangue para avaliações bioquímicas e de citocinas, interleucina 6 e fator de crescimento transformador beta 1, e questionário de qualidade de vida (KDQOL-SF36). Foram utilizadas para análise dos dados, associações e correlações com nível de significância de 5%. Resultados: Pacientes na modalidade contínua apresentaram valores séricos do fator de crescimento transformador beta 1 maiores do que os em modalidade intermitente. Estes apresentaram fator de crescimento transformador beta 1 no peritônio, idade e função renal residual maiores do que os em modalidade contínua. A dosagem da interleucina 6 no peritônio foi associada à idade, enquanto a IL-6 sérica foi associada à IL-6 no peritônio, ao tempo em diálise e à idade. Não houve associação entre a modalidade e a presença de diabetes, volemia ou estado nutricional. Ambas as modalidades permitem boa adequação à terapia dialítica. Conclusão: A inflamação na diálise peritoneal automatizada está associada principalmente à baixa função renal residual, à idade avançada e ao maior tempo em terapia, e não à modalidade de diálise realizada.

Persistent Nocturnal Enuresis in Adulthood Effectively Treated Using Kampo Medicine : A Case Report / 日本東洋医学雑誌

Here, we report a case of nocturnal enuresis persisting into adulthood treated with Kampo medicine. A 21-year-old woman had a history of nocturnal enuresis every night during childhood and once every 2-3 nights during school age. Although the frequency of urinary incontinence decreased to 1-2 times/week at 19 years of age, nocturnal enuresis persisted into adulthood. She was diagnosed with mild strain of the rectus abdominis muscles and palpable abdominal aortic pulsation above the umbilicus, and keishikaryukotsuboreito (decoction) therapy was initiated. She did not experience urinary incontinence at night for 3 weeks, except for one episode immediately after treatment commencement. She tended to urinate only 4 times from the time she awoke until bedtime ; therefore, we added a small amount of goreisan to her treatment regimen to increase the urinary output and daytime frequency of urination. The frequency of urination increased from 4 to 7 times and stabilized. We discontinued goreisan administration 2 months later, and keishikaryukotsuboreito monotherapy was continued. We did not observe recurrent nocturnal enuresis and, therefore, switched the decoction to an extract. After the symptom stabilized, the dosage of keishikaryukotsuboreito was reduced by half. Nocturnal enuresis occurred only once when the patient missed a dose for 3 consecutive days but did not recur for over 4 months after strict adherence to medication. This case report highlights the role of Kampo therapy as an effective therapeutic option for nocturnal enuresis persisting into adulthood.

Urodynamic evaluation of functional urinary incontinence in children / 现代泌尿外科杂志

Pediatric urinary incontinence (PUI) is common in clinical practice and seriously affects the quality of life as well as physical and mental health of patients. PUI is a multi-factorial related abnormality, very complex in etiology and types. The occurrence of PUI is mostly associated with abnormal vesicourethral function. Urodynamic examination (UDS) is the golden standard to assess voiding function and diagnose the type of bladder and urethral function in children with PUI. UDS of PUI is of great clinical value in determining the cause, making treatment protocol as well as evaluating the therapeutic response. However, UDS in children has not been popularized in China, which seriously affects the diagnosis and treatment of PUI. This article reviews the research progress in the clinical application of UDS in the evaluation of PUI, in order to provide reference for improving the diagnosis and treatment of this disease.

Analysis of current situation and risk factors of primary nocturnal enuresis in adolescents / 现代泌尿外科杂志

【Objective】 To investigate the prevalence and risk factors of primary nocturnal enuresis (PNE) in adolescents, and to explore its psychological effects. 【Methods】 During Sep.2020 and Dec.2020, an epidemiological survey was conducted among 6 408 junior and senior high school students in a region of Henan Province by stratified and cluster random sampling. The survey included general information questionnaire, urinary frequency, urgency, incontinence, recurrent urinary tract infection (RUTI), Enuresis Questionnaire, Self-esteem Scale (SES) and the Pittsburgh Sleep Quality Index (PSQI). 【Results】 A total of 7, 000 questionnaires were distributed and 6 408 (91.54%) were valid. The survey showed that the total prevalence of PNE among adolescents was 2.98%. The prevalence was 4.67% in those aged 12 years and 1.37% in those aged 18 years. The results of Logistic regression analysis showed that male (OR=1.677, P<0.05), overweight (OR=1.842, P<0.05), urgency (OR=1.676, P<0.05), frequency (OR=1.919, P<0.05), incontinence (OR=3.493, P<0.001), RUTI (OR=2.535, P<0.001) and family history (OR=3.005, P<0.001) were related to the risk of PNE. The SES score of PNE patients was lower than that of non-PNE group (z=-3.097, P<0.05), and the PSQI was higher (z=-5.456, P<0.05). 【Conclusion】 The prevalence of PNE is high in adolescents and decreases gradually with age. Male, overweight, frequency, urgency, incontinence, RUTI and family history are risk factors. PNE has a negative impact on self-esteem and sleep quality in adolescents.

Prey of the Patagonian species Hilarempis sigillata Collin (Diptera, Empididae, Empidinae, Hilarini), and the first record of nocturnal activity in the tribe

ABSTRACT The Empididae fauna of Chile is basically known from a taxonomic perspective. Herein, we have added biological data based on a species of Hilarini, improving our knowledge regarding the southern temperate fauna of the family. We report for the first time the prey and nocturnal activities of the Patagonian empidid species Hilarempis sigillata Collin, 1933. Specimens were collected until two hours after sunset on a white light sheet at a river margin in the Chilean region of Los Lagos, close to the Puyehue National Park. Adults were found on the sheet with prey of the Chironomidae genera Cricotopus, Xestochironomus, Microtendipes, Podochlus, Heptagyia and Reissmesa (Diptera), as well as a species of the Coniopterygidae genus Semidalis (Neuroptera) and a species of the Limoniidae genus Erioptera (Diptera). The empidids held the prey close to their body using the mid tarsi, in most cases with the ventral side of the prey turned up or laterally.

Electrophysiological detection of visible wavelengths of artificial lights inducing take-off in adults of Rhodnius prolixus (Hemiptera: Triatominae)

ABSTRACT Rhodnius prolixus is the most important vector of Trypanosoma cruzi in the northern part of South America. The compound eyes in adults of R. prolixus are involved in the nocturnal flight dispersion from sylvatic environments into human dwellings. During this behavior, the artificial lights play an important role in attracting R. prolixus; however, it is still not clear whether the compound eyes of this species use different visible wavelengths as a cue during active dispersion. We applied electrophysiological (electroretinography or ERG) and behavioral (take-off) experiments in a controlled laboratory setting to determine the spectral sensitivity of the compound eyes and the attraction of R. prolixus adults to discrete visible wavelengths. For the ERG experiments, flashes of 300 ms at wavelengths ranging between 350 and 700 nm at a constant intensity of 3.4 µW/cm2 were tested after adaptation to darkness and to blue and yellow lights. For the behavioral experiments, the adults were exposed to nine visible wavelengths at three different intensities, and their direction of take-off in an experimental arena was established with circular statistics. The ERG results showed peaks of spectral sensitivity at 470-490 nm and 520-550 nm in adults, while behavioral experiments showed attractions to blue, green and red lights, depending on the intensity of the light stimuli. The electrophysiological and behavioral results confirm that R. prolixus adults can detect certain wavelengths in the visible spectrum of light and be attracted to them during take-off.

Enuresis and upper airway obstruction: BNP and ADH hormones behavior before and after airway surgery

ABSTRACT Introduction: Upper airway obstruction (UAO) is a common condition in all pediatric population, with a 27% prevalence. Primary monosymptomatic nocturnal enuresis (PMNE) is a condition related to UAO in 8% to 47% of these children. The specific pathophysiological mechanism of this bond is not well understood. Some authors suggest a connection between brain natrituretic peptide (BNP) and anti-diuretic hormone (ADH) during sleep. The aim of this study was to evaluate hormone profile (ADH and BNP) and improvement in dry nights in a sample of children before and after surgical treatment of the UAO. Methods: This is a longitudinal prospective interventionist study in children, 5 to 14 years of age, with UAO and PMNE recruited in a specialty outpatient clinic. Children presenting UAO and PMNE were evaluated with a 30-day dry night diary and blood samples were collected to evaluate ADH and BNP before and after upper airway surgery. Data were analyzed prior to surgery and 90-120 days after surgery. Results: Twenty-one children with a mean age of 9.7 years were included. Mean BNP before surgery was 116.5 ± 126.5 pg/mL and 156.2 ± 112.3 pg/mL after surgery (p<0.01). Mean ADH was 5.8 ± 3.2 pg/mL and 14.6 ± 35.4 before and after surgery, respectively (p=0.26). The percentage of dry nights went from 32.3 ± 24.7 before surgery to 75.4 ± 33.4 after surgery (p<0.01). Conclusion: Surgery for airway obstruction contributed to an increase in BNP without increasing ADH. A total of 85.8% of the children presented partial or complete improvement of their enuresis.

Validación de un algoritmo hipnoterapéutico para niños con enuresis nocturna monosintomática / Validation of a hypnotherapeutic algorithm for children with monosymptomatic nocturnal enuresis i: en

Introducción: La enuresis nocturna monosintomática es la micción involuntaria intermitente durante la noche. Esta se presenta en 15 % de los niños de 5 años, en 5 % de los de 10 años y 12 % de los adultos jóvenes sin defectos congénitos o adquiridos. Objetivo: Validar un algoritmo hipnoterapéutico diseñado para pacientes con enuresis nocturna. Métodos: Se realizó un estudio complejo en la Clínica de Hipnosis Terapéutica de la Universidad de Ciencias Médicas en Santiago de Cuba, de febrero del 2021 a julio del 2022, el cual estuvo estructurado en dos etapas metodológicas; en la primera se diseñó el algoritmo terapéutico y en la segunda se validó el contenido de este. A tal efecto, se aplicaron un cuestionario del tipo Likert, el índice de validez de contenido, el coeficiente de concordancia de Kendall, así como el coeficiente alfa de Cronbach y el análisis de conglomerados. Resultados: La validación del contenido realizada por los expertos mostró una excelente consistencia interna, con un coeficiente alfa de Cronbach de 0,9221, que se interpreta como una altísima fiabilidad del instrumento. Asimismo, el índice de validez del contenido de los ítems fue superior a 0,89, lo que también denotó una validez elevada. Conclusiones: La validación del algoritmo hipnoterapéutico diseñado y sometido al criterio de expertos demostró ser adecuado, con elevada coherencia y concordancia evaluativas.

Introduction: Monosymptomatic nocturnal enuresis is the involuntary urine loss during sleep which affects 15 % of 5-years-old children, 5 % of 10-years-olds and 12 % of young teens without congenital or acquired defects. Objective: To validate a hypnotherapeutic algorithm designed for patients with nocturnal enuresis. Methods: A complex study was carried out at the Therapeutic Hypnosis Clinic of the University of Medical Sciences in Santiago de Cuba, from February 2021 to July 2022. It was structured in two methodological stages; in the first one the therapeutic algorithm was designed and in the second one the content of this algorithm was validated. For this purpose, a Likert scale questions, the content validity index, Kendall's coefficient of concordance, as well as Cronbach's alpha and cluster analysis were applied. Results: The content validation executed by the experts showed excellent internal consistency, with a Cronbach's alpha of 0.9221, which meant a very high reliability of the instrument. Likewise, the content validity index of the items was higher than 0.89 that also indicated high validity. Conclusions: The validation of the hypnotherapeutic algorithm designed and subjected to experts' criteria proved to be adequate, with high evaluative coherence and concordance.

Central Retinal Vein Occlusion Secondary to Paroxysmal Nocturnal Hemoglobinuria

Aims: To describe a Central Retinal Vein Occlusion Secondary to Paroxysmal Nocturnal Hemoglobinuria.Presentation of Case: A 25 years old, male, student, in regular follow-up in the Hematology sector due to Paroxysmal Nocturnal Hemoglobinuria in regular use of Eculizumab. He reports a month ago that he suddenly noticed, upon waking up in the morning, blurring and decreased visual acuity in her left eye.Discussion: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder characterized by hemolysis, thrombosis, and bone marrow failure caused by defective expression of glycosylphosphatidylinositol-anchored (GPI-anchored) complement inhibitors. Most commonly, PNH is caused by the loss of PIGA function, which is necessary for GPI biosynthesis.Conclusions: Patients with hemolytic anemia, unexplained thrombosis, especially in uncommon sites, cytopenias and bone marrow failure syndromes, dysphagia, and unexplained abdominal pain should be screened for PNH. PNH patients can benefit greatly from the treatments available, with a reduction in the risk of serious sequelae and a considerable improvement in their quality of life. So that the patient continues to be followed up in the ophthalmology department, with decreased visual acuity ipsilateral to the lesion, with a relative afferent pupillary defect on that side.

Association between winter season and desmopressin treatment efficiency in children with monosymptomatic nocturnal enuresis: a pilot study

ABSTRACT Objectives: The purpose of our study was to assess the association between the winter season and desmopressin treatment failure in South Chinese children with monosymptomatic nocturnal enuresis (MNE). Materials and Methods: A retrospective study was conducted to analyze the clinical data of children with monosymptomatic nocturnal enuresis who have visited our urology clinic from January to December 2019. All patients received desmopressin treatment. Final treatment outcomes were categorized as successful (complete response) or failed (absent and partial response). The relationship between winter season and treatment response to desmopressin was evaluated. Additionally, associated risk factors were investigated with both univariate and multivariate regression analysis. Results: In total, 393 patients diagnosed with MNE were included in the present study. There were no statistically significant differences in pretreatment variables at first visit between patients who visited the clinic in winter and those who did so in other seasons. However, the treatment failure rate of MNE in the winter season was higher than that of other seasons (77.50% vs. 52.74%). Multivariate logistic regression analysis demonstrated that the severity of symptoms and an initial clinic visit in the winter season were significantly related to desmopressin treatment failure in MNE patients. Conclusion: Winter season and severity of symptoms are two risk factors associated with desmopressin treatment failure in MNE patients.

Detection of paroxysmal nocturnal haemoglobinuria clones in cases of deep vein thrombosis in a tertiary care centre, western Rajasthan

Background & objectives: Paroxysmal nocturnal haemoglobinuria is a rare acquired disease characterized by bone marrow failure, intravascular haemolysis and thrombophilia. Thrombosis is the deadliest complication of paroxysmal nocturnal haemoglobinuria (PNH). The present study was conducted to study the prevalence of PNH in cases of deep vein thrombosis (DVT) which was previously undocumented from western Rajasthan. Methods: In the present cross-sectional study, 61 adult patients with DVT were tested using flow cytometry to detect PNH clones. Blood samples were processed using fluorescein-labelled proaerolysin, CD14, CD24, CD33 and CD45 panels for granulocytes and monocytes and CD59 and CD235a panel for red blood cells. Results: Three cases (4.92%) having large clones on monocytes as well as granulocytes, which fulfilled the diagnostic criteria of PNH were detected. Further, three cases (4.92%) showed small clones on both granulocytes and monocytes. Nine (15%) cases showed small clones only on granulocytes, and 11 (18%) cases showed small clones only on monocytes. Interpretation & conclusions: The results of the present study suggest that a higher proportion of patients had PNH in western Rajasthan compared to previously reported studies from elsewhere. It is suggested that PNH testing should be added to the procoagulant work-up panel in institutions of this region where it is not routinely done. This provides an otherwise missed opportunity to diagnose this disorder. Eculizumab may be employed, which is effective in reducing thrombophilic events in cases of PNH

Childhood aplastic anaemia with paroxysmal nocturnal haemoglobinuria clones: A retrospective single-centre study in South Africa

Background: Paroxysmal nocturnal haemoglobinuria (PNH) clones in children are rare but commonly associated with aplastic anaemia (AA) and myelodysplasia.Objective: This study aimed to determine the prevalence of PNH clones in paediatric patients with idiopathic AA, identify differences in clinical and laboratory features and outcomes, and determine the impact of clone size on clinical presentation.Methods: Patients with confirmed idiopathic AA who were tested for PNH between September 2013 and January 2018 at the Inkosi Albert Luthuli Central Hospital, Durban, KwaZulu-Natal, South Africa, were included. PNH clones were detected in neutrophils and monocytes by flow cytometry using fluorescent aerolysin, CD24, CD66b and CD14. Results: Twenty-nine children with AA were identified and 11 were excluded. Ten patients (10/18, 55.6%) had PNH clones ranging from 0.11% to 24%. Compared to the PNH-negative group, these children were older (median: 10 years vs 4 years, p= 0.02) and had significantly lower total white cell counts (median 1.7 × 109/L vs 3.2 × 109/L; p= 0.04). There was no difference in median absolute neutrophil count or haemoglobin concentration. Four patients in each group received immunosuppressive therapy (IST). At six months, all four patients with PNH clones had responded, compared to one in the PNH-negative group. Conclusion: More than half of children with AA had a PNH clone. The size of the clone did not impact clinical severity; however, IST use may positively impact prognosis. We recommend early initiation of IST in patients with AA to avoid delays associated with human leukocyte antigen typing.

Diagnostic Value of Mean Nocturnal Baseline Impedance for Gastroesophageal Reflux Disease by 24 - Hour Multichannel Intraluminal Impedance-pH Monitoring / 胃肠病学

The value of 24 - hour multichannel intraluminal impedance - pH (24 h MII - pH) monitoring is still unknown in most patients with gastroesophageal reflux disease (GERD), and the value of mean nocturnal baseline impedance (MNBI) in the diagnosis of GERD remains controversial. Aims: To analyze the characteristics of esophageal MNBI in GERD patients, and to investigate the diagnostic value of MNBI for GERD. Methods: The clinical data of 111 patients suspected of GERD and monitored for 24 h MII-pH from May 2019 to December 2021 at the Second Affiliated Hospital of Baotou Medical College were retrospectively analyzed. According to DeMeester standard, patients were divided into non - GERD group and GERD group. Reflux parameters and MNBI of each channel between the two groups were compared. The correlation between distal and proximal esophageal MNBI and reflux parameters were analyzed. ROC curve was used to evaluate the sensitivity and specificity of MNBI for GERD. Results: Compared with non-GERD patients, the acid exposure time (AET), DeMeester score, total reflux times, acid reflux times and non-acid reflux times in GERD group were increased, the differences were statistically significant (P<0.01). MNBI at 3 cm, 5 cm and 7 cm above the dentate line and distal MNBI in the GERD group were decreased, the differences were statistically significant (P<0.01). Spearman correlation analysis showed that distal MNBI was negatively correlated with AET, DeMeester score, acid reflux times and weak acid reflux times (P<0.05). There were significant negative correlations between proximal MNBI and AET, DeMeester score and weak acid reflux times (P<0.05). ROC curve analysis showed that AUC of distal MNBI for the diagnosis of GERD was 0.72 (95% CI: 0.66-0.81, P<0.01), when the cut-off value was 1 191.42 Ω, the sensitivity and specificity of distal MNBI for diagnosis of GERD were 82.9% and 53.9%, respectively. Conclusions: Distal esophageal MNBI has good diagnostic significance for GERD patients, and can be used as a new impedance index for the auxiliary diagnosis of GERD.

Complement Inhibitors in Rare Diseases / 罕见病研究

The complement system is a self-protection mechanism of the human body. The abnormal activation of the complement system is involved in the occurrence and development of various diseases. The application of complement inhibitors in many rare diseases was a milestone in leading to the progress of such disease as paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), and others. Recently, the application of complement inhibitors has gradually expanded to other complement-related diseases. This review summarizes the literature on the current application of complement inhibitors in rare diseases and looks into the prospects of the application in the rare diseases.

Clinical characteristics of paroxysmal nocturnal hemoglobinuria (PNH) complicated with ischemic bowel disease / 中华内科杂志

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem cell disease. Gastrointestinal involvement is rarely seen in PNH. This study aims to analyze the clinical features in PNH patients complicated with ischemic bowel disease. Clinical date of 6 patients were collected at Peking Union Medical College Hospital from January 2010 to December 2020. The clinical manifestations, laboratory tests,imaging, endoscopic,and histopathological features and treatment were analyzed.Five in 6 patients were men, with a median age of 31 years old at onset. Most of disease course were recurrent episodes of chronic disease, with abdominal pain (5/6) and gastrointestinal bleeding (5/6). Laboratory examinations showed pancytopenia, reticulocytosis, elevated serum lactate dehydrogenase, high D-dimer and C-reactive protein levels in all patients. Multiple segments of small intestine were the most commonly involved and colon was also affected. Abdominal CT scan showed thickening and roughness or exudation of the intestinal wall (6/6), increased mesenteric density or “comb sign”(4/6), and cholestasis or gallbladder stones (5/6). Endoscopic manifestations included irregular shallow ulcers in the annular cavity (5/6), swelling mucosa with well-defined margins (6/6). Pathological biopsy revealed chronic inflammation of mucosa. The efficacy of steroids combined with anticoagulant therapy was better than that of steroids alone. Ischemic bowel disease in PNH patients is different from typical ischemic enteritis. Young patients, involvement of intestine with multiple segments are common characteristics. The anticoagulant is an essential agent for these patients.

Significance of paroxysmal nocturnal hemoglobinuria clone in immunosuppressive therapy for children with severe aplastic anemia / 中国当代儿科杂志

OBJECTIVES@#To study the association between paroxysmal nocturnal hemoglobinuria (PNH) clone and immunosuppressive therapy (IST) in children with severe aplastic anemia (SAA).@*METHODS@#A retrospective analysis was performed on the medical data of 151 children with SAA who were admitted and received IST from January 2012 to May 2020. According to the status of PNH clone, these children were divided into a negative PNH clone group (n=135) and a positive PNH clone group (n=16). Propensity score matching was used to balance the confounding factors, and the impact of PNH clone on the therapeutic effect of IST was analyzed.@*RESULTS@#The children with positive PNH clone accounted for 10.6% (16/151), and the median granulocyte clone size was 1.8%. The children with positive PNH clone had an older age and a higher reticulocyte count at diagnosis (P<0.05). After propensity score matching, there were no significant differences in baseline features between the negative PNH clone and positive PNH clone groups (P>0.05). The positive PNH clone group had a significantly lower overall response rate than the negative PNH clone group at 6, 12, and 24 months after IST (P<0.05). The evolution of PNH clone was heterogeneous after IST, and the children with PNH clone showed an increase in the 3-year cumulative incidence rate of aplastic anemia-PNH syndrome (P<0.05).@*CONCLUSIONS@#SAA children with positive PNH clone at diagnosis tend to have poor response to IST and are more likely to develop aplastic anemia-PNH syndrome.

"Catatrenia": un trastorno del sueño poco conocido: descripción de tres casos clínicos pediátricos / Catathrenia: unknown sleep disorder: report 3 cases in pediatric patients

Catatrenia (gemido nocturno) es una condición rara caracterizada por sonidos irregulares que ocurren durante el sueño. Los comportamientos ocurren intermitentemente durante cualquiera de las dos etapas de sueño, REM o NREM y se caracterizan por gemidos prolongados, a menudo muy fuertes, socialmente perturbadores, durante la expiración. Es poco conocido y espera más definición y estudios terapéuticos. Hay pocos reportes y en su mayoría de pacientes adultos. Se presentan 3 casos en pacientes pediátricos.

Catathrenia (nocturnal groaning) is a rare condition characterized by irregular sounds that occur during sleep. The behaviors occur intermittently during either REM or NREM sleep and are characterized by prolonged, often very loud, socially disruptive groaning sounds during expiration. It is poorly understood and awaits further definition and therapeutic studies. There are few reports mostly adult patients are presented below 3 cases in pediatric patients.

Quality of life in enuretic children

ABSTRACT Introduction: Nocturnal enuresis is a highly incident chronic disorder that generates countless problems to the child and their parents. Bed-wetting has significant negative impacts on self-esteem and the performance of children. The aim of the current study is to assess the quality of life of enuretic children, as well as its association to sex and age. Patients and Methods: Thirty-nine enuretic children (23 boys) and 49 healthy children (27 boys) without any history of previous treatment for enuresis or voiding dysfunction were included. Age ranged between 6 and 11 years old. The "AUQEI" questionnaire was applied in a private environment to all children by the same researcher (psychologist) to evaluate quality of life. Results: Enuretic children displayed loss in quality of life when compared to non-enuretic (35.9% of enuretic x 16.3% of non-enuretic, p=0.035). They were mostly affected in their daily activities (p=0.02). No significant differences were found in the association of sex and gender with quality of life. These results suggest that, children with nocturnal enuresis have 2.87 times more chances of having loss in quality of life compared to non-enuretic. Conclusions: Enuresis has a great impact in quality of life of children. This impact is not related to the age or sex of the child.

Variablilidad de la presión arterial, hipertensión arterial nocturna y su asociación con tabaquismo / Blood pressure variability, nocturnal hypertension and its association with smoking / Variabilidade da pressão arterial, hipertensão arterial noturna e sua associação com o tabagismo

Resumen: Introducción: La hipertensión arterial y el tabaquismo son factores de riesgo independientes para el desarrollo de eventos cardiovasculares. El consumo de tabaco determina una elevación aguda de la presión arterial por acción del sistema simpático. Sin embargo los efectos a largo plazo son contradictorios. El aumento de la variabilidad de la presión arterial y la presencia de hipertensión arterial nocturna se asocia con eventos cardiovasculares adversos independientemente de los niveles de presión arterial. Objetivos: Evaluar la asociación entre tabaquismo e hipertensión arterial diurna, nocturna y variabilidad. Material y Métodos: Estudio analítico, observacional, transversal, multicéntrico, que incluye pacientes hipertensos mayores de 18 años con monitoreo ambulatorio de la presión arterial (MAPA). Resultados: Se incluyeron 391 pacientes, siendo 14.6% fumadores. Se encontraron cifras de presión arterial diurna sistólica y diastólica más elevadas en pacientes tabaquistas (p=0.204, p=0.087, respectivamente). Se observó una asociación significativa entre la hipertensión arterial nocturna y la presencia de diabetes mellitus e índice de masa corporal aumentado. No se encontró asociación entre el consumo de tabaco y los distintos patrones de variabilidad, así como tampoco con la hipertensión arterial nocturna. Conclusiones: El grupo de pacientes fumadores presentó una tendencia a cifras de presión arterial media diurna sistólica y diastólica más elevadas que los no fumadores, lo que podría sugerir que el tabaquismo incide en el control de cifras de presión arterial.

Abstract: Introduction: Hypertension and smoking are independent risk factors for the development of cardiovascular events. Tobacco use causes an acute elevation of blood pressure due to the action of the sympathetic system. However, the long-term effects are contradictory. Increased variability in blood pressure and the presence of nocturnal arterial hypertension are associated with adverse cardiovascular events regardless of blood pressure levels. Objectives: To evaluate the association between smoking and daytime and nighttime arterial hypertension and variability. Material and Methods: Analytical, observational, cross-sectional, multicenter study, which includes hypertensive patients over 18 years of age with ambulatory blood pressure monitoring (ABPM). Results: 391 patients were included, being 14.6% smokers. Higher levels of systolic and diastolic daytime blood pressure were found in smoking patients (p = 0.204, p = 0.087, respectively). A significant association was observed between nocturnal arterial hypertension and the presence of diabetes mellitus and increased body mass index. No association was found between tobacco consumption and the different patterns of variability, as well as with nocturnal arterial hypertension. Conclusions: The group of smoking patients showed a trend towards higher mean daytime systolic and diastolic blood pressure figures than non-smokers, which could suggest that smoking affects the control of blood pressure numbers.

Resumo: Introdução: Hipertensão e tabagismo são fatores de risco independentes para o desenvolvimento de eventos cardiovasculares. O uso do tabaco provoca elevação aguda da pressão arterial devido à ação do sistema simpático. No entanto, os efeitos de longo prazo são contraditórios. O aumento da variabilidade da pressão arterial e a presença de hipertensão arterial noturna estão associados a eventos cardiovasculares adversos, independentemente dos níveis de pressão arterial. Objetivos: Avaliar a associação entre tabagismo e hipertensão arterial diurna e noturna e variabilidade. Materiai e Métodos: Estudo analítico, observacional, transversal, multicêntrico, que inclui hipertensos maiores de 18 anos com monitorização ambulatorial da pressão arterial (MAPA). Resultados: Foram incluídos 391 pacientes, sendo 14,6% tabagistas. Níveis mais elevados de pressão arterial diurna sistólica e diastólica foram encontrados em pacientes fumantes (p = 0,204, p = 0,087, respectivamente). Foi observada associação significativa entre hipertensão arterial noturna e presença de diabetes mellitus e aumento do índice de massa corporal. Não foi encontrada associação entre o consumo de tabaco e os diferentes padrões de variabilidade, bem como com a hipertensão arterial noturna. Conclusões: O grupo de pacientes fumantes apresentou tendência a valores médios de pressão arterial sistólica e diastólica mais elevados do que os não fumantes, o que pode sugerir que o tabagismo afeta o controle dos valores da pressão arterial.