Clinical and neurophysiological description of patients with POEMS syndrome.

INTRODUCTION: POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a monoclonal gammopathy with polyneuropathy as a mandatory criterion. OBJECTIVE: To describe potential associations between clinical expression and electrodiagnostic patterns in POEMS syndrome. METHOD: Observational, retrospective, cross-sectional study of cases cared for in a referral center, diagnosed with POEMS syndrome from 2009 to 2019. RESULTS: Eleven patients (8 men) were analyzed. Median age at diagnosis was 40 years (range: 31-51; mean: 37.19 ± 15.67 years). Mean latency at diagnosis was 9.7 ± 8.37 months. In all subjects, initial clinical manifestation was polyneuropathy. Most patients had an axonal pattern (n = 5), followed by demyelinating (n = 4) and mixed patterns (n = 2). Monoclonal gammopathy was observed in all (6 l and 5 k cases; immunoglobulin [Ig] G: 72 %; IgA: 18 %; IgM: 9 %). Medical Research Council sum score was lower in the axonal pattern (median: 37.00 vs. 45.5; p = 0.024). There were no differences in systemic involvement between electrophysiological patterns. CONCLUSION: Electrophysiological patterns are unlikely to have a clear extra-neurological clinical correspondence; however, this will have to be definitively proven with a larger sample size.

INTRODUCCIÓN: El síndrome POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) es una gammapatía monoclonal con polineuropatía como criterio obligatorio. OBJETIVO: Describir las potenciales asociaciones entre la expresión clínica y los patrones de electrodiagnóstico en el síndrome POEMS. MÉTODO: Estudio observacional, retrospectivo y transversal de los casos atendidos en un centro de referencia con diagnóstico de síndrome de POEMS de 2009 a 2019. RESULTADOS: Se analizó a 11 pacientes (8 hombres). La mediana de la edad al diagnóstico fue de 40 años (rango: 31-51, media: 37.19 ± 15.67 años). La latencia media al diagnóstico fue de 9.7 ± 8.37 meses. En todos la manifestación clínica inicial fue la polineuropatía. La mayoría de los pacientes presentaron un patrón axonal (n = 5), seguido del desmielinizante (n = 4) y mixto (n = 2). La gammapatía monoclonal se observó en todos (6 casos l y 5 k, inmunoglobulina [Ig] G: 72%, IgA: 18%, IgM: 9%). El Medical Research Council sum score fue menor en el patrón axonal (medianas: 37.00 vs. 45.5, p = 0.024). No se presentaron diferencias en la afección sistémica entre patrones electrofisiológicos. CONCLUSIÓN: Es poco probable que los patrones electrofisiológicos tengan una correspondencia clínica extraneurológica clara, sin embargo, esto deberá probarse de forma definitiva con un mayor tamaño de muestra.

Satisfação do enfermeiro no serviço de atendimento móvel de urgência aéreo / Satisfaction of nurses in service mobile service emergency air

Objetivo: descrever a satisfação do enfermeiro no Serviço de Atendimento Móvel de Urgência Aéreo e identificar os problemas que dificultam a assistência de enfermagem no SAMU Aéreo. Metodologia: trata-se de uma pesquisa descritiva de abordagem qualitativa, realizada por meio de entrevistas, com perguntas abertas no SAMU Aéreo estadual de Teresina. A pesquisa foi realizada no período 09 de setembro de 2014 a 29 de outubro de 2014. Participaram do estudo sete enfermeiros. Os relatos foram transcritos na íntegra e a análise foi de conteúdo. O estudo obedeceu aos princípios éticos e legais que rege a Resolução 466/12 do CNS e foi aprovado pelo Comitê de Ética com o CAAE n. 32385414.0.0000.5210. Resultados: Os resultados apontaram duas categorias: a satisfação dos enfermeiros sobre o seu trabalho no SAMU Aéreo, e a as dificuldades encontradas na assistência de enfermagem no SAMU Aéreo. Conclusão: conclui-se que o SAMU Aéreo, realiza um serviço de qualidade apesar das dificuldades. O enfermeiro tem papel importante, pois são atendimentos complexos e requerem habilidade, agilidade, capacitação e muito amor pelo trabalho.

Objective: to show the satisfaction of nurses on mobile air emergency care service and identify problems that difficult nursing assistance in air SAMU. Methodology: treatment is a descriptive research with qualitative approach, performed by interviews with open-ended questions at the SAMU. The research was carried out between September 9, 2014 to October 29, 2014. The study included seven nurses. The reports were transcribed and the analysis was content. The study followed the ethical and legal principles governing the CNS Resolution 466/12 and was approved by the Ethics Committee with the CAAE n. 32385414.0.0000.5210. Results: The results showed two categories: the satisfaction of nurses about their work in the air SAMU, and the difficulties encountered in nursing care in the Air SAMU. Conclusion: we conclude that air SAMU, performs a quality service despite the difficulties. The nurse has an important role because they are complex and require care ability, agility, training and lots of love for the work.

Evaluación multidimensional de los servicios de salud para adolescentes en centros de Atención Primaria en una comuna de Santiago / Multidimensional assessment of public health care services for adolescents in Chile

Background: Adolescents are an especially vulnerable age group in terms of behavioral issues and require skilled teams in health centers. Aim: To assess the quality of health services provided to teenage users in Primary Health Care. Material and Methods: A study of multiple cases was carried out in two family health centers in Puente Alto, Chile. Health services delivered to adolescents were evaluated from the provider's perspective, through qualitative design of focus groups and interviews to the care teams at each centre. For technical quality, comparing electronic records of two tracer conditions (prenatal care and depression) with technical standards established by Delphi methodology and from teenage users perspective, through a survey of service satisfaction. Results: In both centers, providers perceived a lack of training in adolescent care, a deficient preventive approach and a limited access to care. The technical evaluation showed an inappropriate recording of both tracer conditions. The instrument used to assess user satisfaction survey was reliable and showed that the best perceived issue was medical care and treatment, and the least perceived, was the access to the services. Conclusions: Professionals working in these health care facilities, feel unprepared to provide comprehensive approach to adolescents. The surveyed teenagers complained of limited access to care. Therefore this age group continues to be as a non-priority group for health care.

Clinical and laboratory features of youth onset Type 2 diabetes in Jamaica / Características clínicas y de laboratorios en la aparición de la diabetes de Tipo 2 en los jóvenes de Jamaica

OBJECTIVES: To assess the frequency of youth onset Type 2 diabetes mellitus (T2D) in Jamaica and the characteristics of youth with this form of diabetes. METHODS: Patients from two major referral hospitals, diagnosed with diabetes before age 25 years and < 6 years prior to the study, were evaluated. Classification was based on the presence of GAD-65 and IA-2 diabetes autoantibodies (AB), fasting (FCP) and stimulated C-peptide (SCP) measurements, serum leptin and clinical phenotype as follows: (i) Type 1A diabetes - AB+, (ii) Type 1B diabetes - AB- and FCP < 230 pmol/l and/or SCP < 660pmol/l, (iii) Type 2 diabetes - AB- and FCP > 500pmol/L and or SCP > 1160 pmol/l (iv) Untypeable diabetes - AB- and FCP 230-500 pmol/l and or SCP 660-1160 pmol/l and (v) Lipoatrophic diabetes - clinical phenotype and serum leptin. RESULTS: Fifty-eight participants (21M, 37F, age 20 ± 8 years, duration of diabetes 2.6 ± 2 years) were enrolled in the study. Using the classification criteria, Type 1 diabetes was the most common form of diabetes: 18(31%) Type 1A, 18(31%) Type 1B. Overall 22% (13 patients) had T2D. Patients with T2D were more likely to be female, older at diagnosis, obese and have a higher blood pressure when compared to those with Type 1 diabetes. In logistic regression analysis, age of diabetes onset, gender, BMI, systolic and diastolic blood pressure were significantly associated with T2D. Obesity measured by BMI was the strongest predictor of T2D. CONCLUSIONS: While Type 1 diabetes was the predominant form of diabetes in this study, a significant proportion of Jamaicans with youth onset diabetes may have T2D. Obesity is the strongest clinical predictor of Type 2 diabetes in the young diabetic patient.

OBJETIVOS: Evaluar la frecuencia de la aparición de la diabetes mellitus tipo 2 (DT2) en los jóvenes de (T2D) en Jamaica y las características de los jóvenes con esta forma de diabetes. MÉTODOS: Pacientes de dos importantes hospitales de remisión, fueron evaluados y diagnosticados con diabetes antes de los 25 años de edad y < 6 años antes del estudio. La clasificación se basó en la presencia de auto-anticuerpos (AC) GAD-65 e IA-2 de la diabetes, mediciones de péptido C en ayunas (PCA) y péptido C estimulado (PCE), leptina sérica y fenotipo clínico como sigue: (i) diabetes tipo A1 - AB+; diabetes tipo B1 - AB- y PCA < 230 pmol/l y/o PCE < 660 pmol/l; (iii) diabetes tipo 2 - AB - y PCA> 500pmol/L y/o PCE > 1160pmol/l; (iv) diabetes no tipificable - AB - y PCA 230-500 pmol/l y/o PCE 660-1160pmol/l; y (v) diabetes lipoatrófica - fenotipo clínico y leptina sérica. RESULTADOS: Cincuenta y ocho participantes (21 V, 37 H, 20 ± 8 años de edad, duración de la diabetes 2.6 ± 2 años) fueron enrolados en el estudio. Usando los criterios criterio de clasificación, la diabetes de tipo 1 fue la forma más común de diabetes: 18 (31%) tipo A1; 18 (31%) Tipo B1. En conjunto 22% (13 pacientes) tenían DT2. Los pacientes con DT2 presentaban una mayor probabilidad de ser mujeres, tener más edad a la hora del diagnóstico, ser obesos y tener una tensión arterial más alta en comparación con los que presentaban diabetes tipo 1. En el análisis de regresión logística, la edad de la aparición de la diabetes, el género, el IMC, la tensión arterial diastólica y sistólica estaban significativamente asociados con la DT2. La obesidad medida por el IMC fue el predictor más fuerte de la DT2. CONCLUSIONES: Aunque la diabetes tipo 1 fue la forma predominante de diabetes en este estudio, un número significativo de jamaicanos en los que la diabetes aparece en edad juvenil, pueden tener DT2. La obesidad es el predictor clínico más fuerte de la diabetes tipo 2 en el paciente diabético joven.

Cardiovascular risk profile in Caribbean youth with diabetes mellitus / Perfil de riesgo cardiovascular entre la yuventud caribeña con diabetes mellitus

OBJECTIVE: To assess the effect of diabetes mellitus type on conventional and novel cardiovascular risk factors in patients, diagnosed with diabetes from two major referral hospitals in Jamaica, before age 25 years and with diabetes duration < 6 years. METHODS: Participants were classified based on the presence of GAD-65 and IA-2 autoantibodies, C-peptide, leptin and clinical phenotype. Trained observers obtained anthropometric measurements and sitting blood pressure. Fasting blood was taken for glucose, A1c, lipids, high sensitivity C-reactive protein and lipoprotein profile. RESULTS: Fifty-eight participants (21M; 37F, age 20 ± 8 [Mean ± SD] years, diabetes duration 2.6 ± 2 years) were enrolled. Thirty-six had Type 1 diabetes (T1D), thirteen Type 2 diabetes (T2D), six were not typed and three had lipoatrophic diabetes. Patients with Type 2 diabetes (T2D) were more obese with a higher systolic blood pressure but a lower A1c than those with Type 1 diabetes (T1D). Total cholesterol, LDL-cholesterol, triglycerides, VLDL, LDL and HDL particle numbers were similar in patients with T1D and T2D. HDL-cholesterol and LDL and HDL particle sizes were lower in patients with T2D but differences were no longer significant after adjusting for BMI. CONCLUSIONS: Risk factors for cardiovascular disease are common in patients with all forms of youth onset diabetes. Clinicians should therefore investigate these risk factors in their patients regardless of diabetes type.

OBJETIVO: Evaluar el efecto del tipo de diabetes (DM) sobre los factores de riesgo cardiovasculares en pacientes diagnosticados con diabetes antes de los 25 años de edad, con una duración de la DM < 6 años, y remitidos de dos hospitales principales de Jamaica. MÉTODOS: Los participantes fueron clasificados a partir de la presencia de anticuerpos GAD-65 y IA-2, péptido C, leptina y fenotipo clínico. Observadores entrenados obtuvieron mediciones antropométricas y datos sobre la presión sanguínea en posición sentada. Se utilizó sangre en ayunas para los perfiles de glucosa, A1c, lípidos, proteína C de alta sensibilidad, y lipoproteína. RESULTADOS: Cincuenta y ocho participantes (21M; 37F, edad 20 ± 8 [Media ± SD] años, duración de la diabetes 2.6 ± 2 años) fueron enrolados. Treinta y seis tenían diabetes tipo 1 (DT1), 13 de ellos tenían diabetes tipo 2 (DT2), 6 no estaban clasificados, y 3 padecían de diabetes lipoatrópica. Los pacientes con diabetes de tipo 2 eran más obesos y su presión arterial sistólica era más alta, pero presentaban un perfil A1c más bajo que los de la diabetes tipo 1. El colesterol total, el colesterol LDL, los triglicéridos, y el número de partículas VLDL, LDL, y HDL fueron similares en los pacientes con DT1 y DT2. El colesterol HDL y los tamaños de las partículas LDL y HDL fueron más bajos en los pacientes con DT2, pero las diferencias dejaron de ser significativas luego de ser ajustadas para IBM. CONCLUSIONES: Los factores de riesgo para la enfermedad cardiovascular son comunes en pacientes con todas las formas de diabetes aparecidas entre los jóvenes. Por tanto, los clínicos deben investigar estos factores de riesgo en sus pacientes, independientemente del tipo de diabetes.

A new acyclic diketotriterpenoid isolated from the indonesian marine sponge hyrtios erectus

The structure of diketotriterpenoid 1, isolated from the marine sponge Hyrtios erectus collected in Indonesia, has been elucidated by spectroscopic analysis.

Molecular packing groups and ab initio crystal-structure prediction.

Ab initio crystal structure prediction can proceed by minimization of the packing energy of Z independent molecules per cell, or alternatively by energy minimization taking one molecule as independent and Z - 1 molecules as related by assumed space-group symmetry. In the former method, a large number of positional variables must be considered. In the latter method, a large number of space groups must be considered. An alternative, more efficient, procedure is proposed, where it is recognized that values of Z and the number of molecules in the asymmetric unit, Z', impose restrictions on possible space groups. Examples of application of this method to crystal structure prediction are given.