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Background Commonly known as Batten disease, the neuronal ceroid lipofuscinoses (NCLs) are a genetically heterogeneous group of rare pediatric lysosomal storage disorders characterized by the intracellular accumulation of autofluorescent material (known as lipofuscin), progressive neurodegeneration, and neurological symptoms. In 2002, a disease-causing NCL mutation in the CLN6 gene was identified (c.214G > T) in the Costa Rican population, but the frequency of this mutation among local Batten disease patients remains incompletely characterized, as do clinical and demographic attributes for this rare patient population. Objective To describe the main sociodemographic and clinical characteristics of patients with a clinical diagnosis for Batten Disease treated at the National Children's Hospital in Costa Rica and to characterize via molecular testing their causative mutations. Methods DNA extracted from buccal swabs was used for CLN6 gene sequencing. Participants’ sociodemographic and clinical characteristics were also obtained from their medical records. Results Nine patients with a clinical diagnosis of Batten disease were identified. Genetic sequencing determined the presence of the previously described Costa Rican homozygous mutation in 8 of 9 cases. One patient did not have mutations in the CLN6 gene. In all cases where the Costa Rican CLN6 mutation was present, it was accompanied by a substitution in intron 2. Patients were born in 4 of the 7 Costa Rican provinces, with an average onset of symptoms close to 4 years of age. No parental consanguinity was present in pedigrees. Initial clinical manifestations varied between patients but generally included: gait disturbances, language problems, visual impairment, seizures and psychomotor regression. Cortical and cerebellar atrophy was a constant finding when neuroimaging was performed. Seizure medication was a common element of treatment regimens. Conclusions This investigation supports that the previously characterized c.214G > T mutation is the most common causative NCL mutation in the Costa Rican population. This mutation is geographically widespread among Costa Rican NCL patients and yields a clinical presentation similar to that observed for CLN6 NCL patients in other geographies.

Purpose The highest incidence of breast cancer is in senile patients. Treatment decisions are often based on their age and ECOG. The implementation of the G8 in the oncology consultation is useful for timely referral of vulnerable patients to specialized care, in order to generate an improvement on their treatment and prognosis.The aim of this work is to determine, using the G8, the proportion of patients aged >65 years with breast cancer, which requires a comprehensive geriatric assessment (CGA).Material and methodsThe G8 questionnaire was applied to patients >65 years with breast cancer. The baseline characteristics were prospectively collected. Descriptive statistics were used for the characteristics of the patients and the assessment tools. The difference between groups was assessed using Pearson's chi square with Yates correction. Additional scores were calculated using the Kaplan-Meier method and compared between groups using the log rank test. The hazard ratio (HR) with a 95% confidence interval (CI) was estimated using a Cox proportional hazard analysis, considering an abnormal G8 score as an exposure variable. A Spearman correlation was made between age and G8 score. SPSS v22 software (IBM, USA) was used for all analyzes.ResultsFrom December 2019 to May 2020, 357 patients were recruited. The mean age was 73.57 years. Seventy-four percent had an ECOG 1; 44.3% required a CGA (n = 158), but only 55.1% (n = 87) were performed. The G8 most affected domains were polypharmacy (51%) and ingestion (28.3%). We found significant association between the requirement for CGA and ECOG, p ConclusionThe G8 is a tool that allows us to discern which patients require a CGA in order to carry out interventions to improve cancer treatment, so it should be implemented routinely in the oncology consultation.*ECOG: Eastern Cooperative Oncology Group

BACKGROUND Limited data are available on childhood encephalitis in Latin America. Our study aimed to increase insight on clinical presentation, etiology and outcome of children with acute encephalitis in Costa Rica. METHODS We conducted a prospective, observational study during an 8-month period at the Hospital Nacional de Ninos "Dr. Carlos Saenz Herrera" in Costa Rica. Case definition was according to "International Encephalitis Consortium" in children

The aim was to describe clinical presentation, management, and outcomes of stroke in a tertiary emergency department (ED) of a developing country. Retrospective case series of patients aged 1 month to 18 years presenting to an ED with radiological confirmed acute stroke during a 7-year period were studied. Ninety-five patients were identified. Twenty-five patients were excluded because of incomplete records (8) or not presenting via ED (17). Thirty-four (48.5%) were diagnosed with hemorrhagic stroke (HS), 30 (42.8%) with arterial ischemic stroke (AIS), and 6 (8.5%) with sinus venous thrombosis (SVT). Mean age was 5.3 years, and 55.3% were male. The median time from onset of symptoms to ED presentation was 24 hours (mean, 55 hours; interquartile range [IQR], 14–72) for AIS, 24 hours (mean, 46.9 hours; IQR, 9–48) for HS, and 120 hours (mean,112 hours; IQR, 72–168) for SVT. Congenital cardiac disease was the most common risk factor (9%). For AIS, the most common symptoms were focal numbness 56.6% (95% confidence interval [CI], 37.8%–75.4%), focal weakness 56.6% (95% CI, 37.8%–75.4%), and seizures 50% (95% CI, 31%–68.8%). For HS, the most common symptoms were headache 64.7% (95% CI, 47.7%–81.6%), vomiting 79.4 (95% CI, 65–93.7), and altered mental status 64.7% (95% CI, 47.7–81.6). Computed tomography scan was done in 100% of the patients and magnetic resonance imaging in 54%. Twenty-five (36%) patients were admitted to intensive care unit and required intubation. Long-term deficit was identified in 24 (36%) patients based on medium-term follow-up. The spectrum of stroke in a developing country was similar to published series from developed countries in terms of final diagnosis, risk factors, and delay to ED presentation, neuroimaging, and long-term neurodeficits. No tropical diseases were identified as risk factors.

In 2014, 8.5% of people worldwide over the age of 18 had type 2 diabetes mellitus. It is estimated that, in recent years, the prevalence of this disease has increased mainly in low- and middle-income countries. Health behaviors are considered fundamental in the prevention, treatment, and control of this disease. The aim of this study was to determine if there is an association between health behaviors and satisfaction with family functioning in patients diagnosed with type 2 diabetes mellitus. This is an observational, transversal, analytical study. In the Family Medicine Unit No.33 in Chihuahua, Mexico, 297 patients with a diagnosis of type 2 diabetes mellitus were identified. Data was collected regarding sex, age and the Instrument for Measuring Lifestyles in Diabetics (IMEVID, for its acronym in Spanish) and the Family APGAR. We found that of the total number of patients, 91.6% presented some degree of unfavorable health behaviors, while 92.6% presented some type of family dysfunction. The categorical analysis positively correlated good family functionality with favorable health behaviors, Fisher's exact test p [Med-Science 2017; 6(4.000): 659-662]

e24010 Background: G8 is a geriatric screening tool designed to identify elderly cancer patients who benefit from a comprehensive geriatric assessment (CGA). Despite the increase in the incidence of cancer in the geriatric patients, there is absence of trials targeting this population. Decisions are often made based on their age and ECOG/Karnofsky scales, which does not reflect the actual role of an elderly patient. It is necessary to identify patients who require a CGA for an accurate evaluation and optimal oncological management. The routine implementation of the G8 geriatric screening tool in the oncology consultation consumes little time and is useful for timely referral of vulnerable patients to specialized care, in order to generate a positive impact on oncological therapy decision-making, improving their outcomes. Our main objective was to determine, using the geriatric screening tool G8, the percentage of patients aged >65 years with breast cancer, which require a CGA. Methods: The G8 questionnaire was applied to patients >65 yo with a diagnosis of breast cancer from December 2019 to May 2020. The baseline characteristics were prospectively collected and included clinical and demographic characteristics. The prognostic value of the functional status of the ECOG was evaluated and compared with that obtained from the G8. The difference between groups was assessed using Pearson's chi square with Yates correction. Additional scores were calculated using the Kaplan-Meier method and compared between groups using the log rank test. The hazard ratio with a 95% confidence interval was estimated using a Cox proportional hazards analysis, considering an abnormal G8 score as an exposure variable. Spearman correlation was made between age and the G8 score obtained. SPSS v22 software (IBM, USA) was used for all analyzes. Results: From December 2019 to May 2020, 357 patients were recruited. The mean age was 73.57 years (SD+7.02 y). The most frequent clinical stage was IIA (27.7%), followed by I (21.8%). 74.2% had an ECOG 1. Regarding treatment, 317 patients (88.8%) were on hormone therapy. The percentage of patients requiring a CGA was 44.3% (n = 158), of which only 55.1% (n = 87) were performed. We found no significant association between the requirement of CGA and clinical stage (p = 0.27) or treatment received (p = 0.345); but according to the ECOG, we obtained statistical significance p < 0.0001. Spearman correlation was performed between age and the G8 score, a correlation of 0.422 was obtained with p < 0.0001.The most affected domains of G8 were polypharmacy (51%) and ingestion (28.3%). Disease-free survival analysis was performed, without finding differences between vulnerable-frail and healthy patients. Conclusions: The G8 is a tool that allows to discern which patients require a CGA, in order to carry out interventions to improve comprehensive cancer treatment, so it should be implemented routinely in the oncology consultation.

This literature research, although not exhaustive, gives perspective to solar-driven photocatalysis, such as solar photo-Fenton and TiO2 solar photocatalysis, reported in the literature for the degradation of aqueous organic pollutants. Parameters that influence the degradation and mineralization of organics like catalyst preparation, type and load of catalyst, catalyst phase, pH, applied potential, and type of organic pollutant are addressed. Such parameters may also affect the photoactivity of the catalysts used in the studied solar processes. Solar irradiation is a renewable, abundant, and pollution-free energy source for low-cost commercial applications. Therefore, these solar processes represent an environmentally friendly alternative mainly because the use of electricity can be decreased/avoided.

Introduction: Clostridium botulinum is known to cause descending paralysis in infants throughout the world. Methodology: The subject of this study was a three-month-old Costa Rican boy who was hospitalized because of poor suction and feeding, hypotonia, and constipation. Clinical history and physical examination findings suggested infant botulism. Samples were sent to the Winnipeg Public Health Laboratory, where Clostridium botulinum toxin A was identified by PCR and culture from the stools, making this the first report of infant botulism in Central America. Conclusions: Although infant botulism is a known disease, the limitations in identifying it in Central America contributes to the misdiagnosis and under-reporting of this disease.