The S-Factor, a New Measure of Disease Severity in Spinocerebellar Ataxia: Findings and Implications.

Spinocerebellar ataxias (SCAs) are progressive neurodegenerative disorders, but there is no metric that predicts disease severity over time. We hypothesized that by developing a new metric, the Severity Factor (S-Factor) using immutable disease parameters, it would be possible to capture disease severity independent of clinical rating scales. Extracting data from the CRC-SCA and READISCA natural history studies, we calculated the S-Factor for 438 participants with symptomatic SCA1, SCA2, SCA3, or SCA6, as follows: ((length of CAG repeat expansion - maximum normal repeat length) /maximum normal repeat length) × (current age - age at disease onset) × 10). Within each SCA type, the S-Factor at the first Scale for the Assessment and Rating of Ataxia (SARA) visit (baseline) was correlated against scores on SARA and other motor and cognitive assessments. In 281 participants with longitudinal data, the slope of the S-Factor over time was correlated against slopes of scores on SARA and other motor rating scales. At baseline, the S-Factor showed moderate-to-strong correlations with SARA and other motor rating scales at the group level, but not with cognitive performance. Longitudinally the S-Factor slope showed no consistent association with the slope of performance on motor scales. Approximately 30% of SARA slopes reflected a trend of non-progression in motor symptoms. The S-Factor is an observer-independent metric of disease burden in SCAs. It may be useful at the group level to compare cohorts at baseline in clinical studies. Derivation and examination of the S-factor highlighted challenges in the use of clinical rating scales in this population.

Comparison of T1Rho MRI, Glucose Metabolism, and Amyloid Burden Across the Cognitive Spectrum: A Pilot Study.

OBJECTIVE: The pathological cascades associated with the development of Alzheimer's disease (AD) have a common element: acidosis. T1rho MRI is a pH-sensitive measure, with higher values associated with greater neuropathological burden. The authors investigated the relationship between T1rho imaging and AD-associated pathologies as determined by available diagnostic imaging techniques. METHODS: Twenty-seven participants (men, N=13, women, N=14; ages 55-90) across the cognitive spectrum (healthy control subjects [HCs] with normal cognition, N=17; participants with mild cognitive impairment [MCI], N=7; participants with mild AD, N=3) underwent neuropsychological testing, MRI (T1-weighted and T1rho [spin-lattice relaxation time in the rotating frame]), and positron emission tomography imaging ([11C]Pittsburg compound B for amyloid burden [N=26] and [18F]fluorodeoxyglucose for cerebral glucose metabolism [N=12]). The relationships between global T1rho values and neuropsychological, demographic, and imaging measures were explored. RESULTS: Global mean and median T1rho were positively associated with age. After controlling for age, higher global T1rho was associated with poorer cognitive function, poorer memory function (immediate and delayed memory scores), higher amyloid burden, and more abnormal cerebral glucose metabolism. Regional T1rho values, when controlling for age, significantly differed between HCs and participants with MCI or AD in select frontal, cingulate, and parietal regions. CONCLUSIONS: Higher T1rho values were associated with greater cognitive impairment and pathological burden. T1rho, a biomarker that varies according to a feature common to each cascade rather than one that is unique to a particular pathology, has the potential to serve as a metric of neuropathology, theoretically providing a measure for assessing pathological status and for monitoring the neurodegeneration trajectory.

Comparison of glucose time in range and area under curve in range in relation to risk of diabetic retinopathy in type 2 diabetes patients.

AIMS/INTRODUCTION: We proposed a novel continuous glucose monitoring (CGM)-based metric, area under the curve in range (AucIR), for integrating both the amplitude and duration of dysglycemia, and further compared AucIR with the emerging key CGM-derived metric, time in range (TIR). MATERIALS AND METHODS: A total of 2,030 adult patients with type 2 diabetes were enrolled during May 2020 to October 2021. AucIR and TIR were measured with 7-day CGM data. Logistic regression analysis and the C-statistic was carried out to assess the association of AucIR and TIR with diabetic retinopathy (DR). RESULTS: Both AucIR (r = -0.89) and TIR (r = -0.95) were strongly correlated with mean glucose levels. Compared with TIR, AucIR showed a tighter relationship with parameters of glycemic variability, including the coefficient of variation (r = -0.56), standard deviation (r = -0.89) and mean amplitude of glycemic excursions (r = -0.70). For each absolute 10% decrease in AucIR, the risk of DR was increased by 7% (95% confidence interval 1.02-1.13) after adjustment for confounders. With respect to TIR, each absolute 10% decrease was associated with an 8% (95% confidence interval 1.03-1.14) increased risk of DR. The model discrimination for DR, as measured by C-statistic, did not differ significantly between the two metrics (P > 0.05). CONCLUSIONS: AucIR did not provide added benefit over TIR in the assessment of DR risk among patients with type 2 diabetes. The potential value of AucIR needs to be explored in future studies.

Características demográficas, clínicas, radiológicas y de función pulmonar en pacientes con bronquiectasias / Radiological and pulmonary function clinical demographic characteristics in patients with bronchiectasias

OBJETIVOS: determinar las principales características demográficas, clínicas, radiológicas y de función pulmonar de los pacientes con bronquiectasias en la Clínica del Pulmón. MATERIAL Y MÉTODOS: estudio observacional, retrospectivo. Se revisaron las historias clínicas de 23 pacientes con diagnóstico de dilataciones bronquiales. RESULTADOS: de los 23 pacientes, con una edad media de 49,4 ± 3,87 años, 13 corresponden al sexo femenino y 10 al sexo masculino, el tiempo de evolución de la enfermedad desde el diagnóstico de la patología inicial es de 17,3 ± 2,92 años. La tuberculosis es la etiología principal en 15 pacientes (65,2%). Las manifestaciones clínicas más frecuentes son la tos y expectoración mucopurulenta por varios años en la mayoría de los pacientes, al que añadimos la disnea y hemoptisis, la auscultación pulmonar revela la presencia de crépitos en 17 pacientes (73,9%). La Tomografía de Tórax de Alta Resolución distingue dos tipos de bronquiectasias: La sacular o quística y la cilíndrica, de localización unilobar, bilobar y multilobar (difuso). La Espirometría Forzada fue indicada en 10 pacientes (43,5%) 7 mujeres y 3 varones, el Síndrome Bronquial Obstructivo fue el hallazgo más frecuente. La asociación de Tetraciclina con Metronidazol indicado en 9 pacientes (39,1%) mejoró el cuadro clínico. La fisiopatología de esta entidad clínica está sujeta a una constante actualización. CONCLUSIONES: en pacientes tosedores crónicos, las bronquiectasias deben tener prioridad diagnóstica, se trata de una patología antigua, pero de actualidad permanente.

The purpose of this document is to determine the main epidemiological and clinical characteristics of patients with bronchiectasis at the Lung Clinic. METHOD: observational, retrospective study. The medical records of 23 patients diagnosed with bronchial dilation were reviewed. RESULTS: the results of the 23 patients studied, with a mean age of 49,4 ± 3,87 years, 13 correspond to the female sex and 10 to the male sex, indicate that the time of evolution of the disease from the diagnosis of the initial pathology is: 17,3 ± 2,92 years. Tuberculosis is the main etiology in 15 patients (65,2%). The most frequent clinical manifestations were cough and mucopurulent expectoration of several years in most of the patients, to which we must add dyspnea and hemoptysis, pulmonary auscultation reveals the presence of crepitus in 17 patients (73,9%). High Resolution Chest Tomography distinguishes two types of bronchiectasis: the saccular or cystic and the cylindrical, the localization is unilobar, bilobar and multilobar (diffuse). Forced spirometry was indicated in 10 patients (43,5%), 7 women and 3 men, Chronic Obstructive Pulmonary Disease is the main diagnosis. The association of Tetracycline with Metronidazole indicated in 9 patients (39,1%) had positive results. The pathophysiology of this clinical entity is subject to constant updating. CONCLUSIONS: in chronic coughing patients, bronchiectasis must have diagnostic priority, it is an old pathology, but it is permanently current

Calidad de la atención en el Servicio de Neonatología del Hospital General Docente "Dr. Juan Bruno Zayas Alfonso" / Quality of care in the Neonatology Service of "Dr. Juan Bruno Zayas Alfonso" Teaching General Hospital

Se realizó un estudio descriptivo y transversal con vistas a evaluar la calidad de la atención en el Servicio de Neonatología del Hospital General Docente "Dr. Juan Bruno Zayas Alfonso", de septiembre de 2013 a marzo de 2014, para lo cual fue dividida la investigación en 3 dimensiones: estructura, proceso y resultados; y se establecieron diferentes criterios, indicadores y estándares. De forma general, se identificaron deficiencias en la calidad de la atención respecto a la estructura del Servicio, en el que no se contaba con ningún pediatra y de los 12 neonatólogos que debían estar laborando, solo lo hacían 7. En cuanto al equipamiento, se detectó que de las 2 cunas térmicas existentes, una se encontraba en mal estado, para 50,0 %, de manera que este indicador resultó inadecuado; de los 5 perfusores disponibles, 1 de ellos estaba en mal estado y 4 aptos, para 80,0 %, lo cual se consideró como inadecuado. Por su parte, de las 3 pesas convencionales, 2 de ellas estaban en mal estado, para 67,0 % del estándar establecido, de modo que este indicador fue inadecuado. Por último, no se detectaron deficiencias ni en el proceso ni en los resultados, por lo que todo el personal fue evaluado como adecuado.

A descriptive and cross-sectional study was carried out with the aim of evaluating the quality of care in the Neonatology Service of "Dr. Juan Bruno Zayas Alfonso" Teaching General Hospital from September, 2013 to March, 2014, for which the investigation was divided in 3 dimensions: structure, process and results; equally, approaches, indicators and standards were established. Generally, deficiencies were identified in the quality of care related to the structure of the Service, in which there was no pediatrician and of the 12 neonatologists who should have been working, there were only 7. Regarding the equipments, it was detected that of the 2 existing thermal cradles, one was not functioning well, for 50.0%, so that this indicator was inadequate; of the 5 available infusion pumps, 1 of them was in bad conditions and 4 were functioning, for 80.0 %, which was inadequate. On the other hand, of the 3 conventional weights, 2 of them were not right, for 67.0% of the established standard, so the indicator was inadequate. Lastly, deficiencies were detected neither in the process nor in the results, so that the whole staff was evaluated as competen.

Zambia.

PIP: Attention in this discussion of Zambia is directed to the following: geography; the people; history; government; the economy; foreign relations; defense; and relations between Zambia and the US. In 1986, the population totaled 7 million with an annual growth rate of 3.7%. The infant mortality rate is 87/1000 with a life expectancy of 51 years. Zambia, located in south-central Africa, is bordered by Zaire, Tanzania, Malawi, Mozambique, Zimbabwe, Botswana, Angola, and Namibia. The population is made up of over 70 Bantu-speaking tribes. Expatriates, mostly British (15,000 in 1986) or South African, live primarily in Lusaka where they are employed in mines and related activities. Some ancestors of present-day Zambians most likely arrived about 2000 years ago and eventually displaced or absorbed indigenous stone age hunters and gatherers. The major waves of Bantu-speaking immigrants began in the 15th century; the greatest influx occurred in the late 17th to the early 19th centuries. After the mid-19th century, the area was penetrated by Western explorers. In 1888, Northern and Southern Rhodesia (now Zambia and Zimbabwe) were proclaimed a British sphere of influence. Southern Rhodesia was annexed formally and granted self-government in 1923. Independence was realized on October 24, 1964. Zambia was the 1st British territory to become a republic immediately upon realizing independence. The constitution promulgated on August 25, 1973, abrogated the original 1964 constitution, and this new constitution and the national elections that followed in December 1973 were the final steps in achieving what is termed a "1-party participatory democracy." President Kenneth Kaunda is the major figure in the country's politics. He has wide popular support and traditionally has bridged the rivalries among the country's various regions and ethnic groups. The economy of Zambia is based primarily on its majority state-owned copper industry, which is the only significant source of foreign exchange. Copper production has dropped to less than 500,000 metric tons/year from a high of 720,000 in 1976. Beginning in late 1982, Zambian government leaders took several important steps to deal with the country's economic plight, including restricting public spending, reducing government subsidies, raising farm producer incentives, and devaluing the currency. The US maintains a substantial foreign assistance program in Zambia.^ieng