Accesibilidad, disponibilidad y asequibilidad de medicamentos para Niños con Enfermedad Renal Crónica en Guatemala / Access, availability and affordability of medications for children with Advanced Chronic Kidney Disease in Guatemala

El artículo tiene como objetivo analizar la disponibilidad, acceso y asequibilidad de los medicamentos para niños con Enfermedad Renal Crónica (ERC) en tratamiento con hemodiálisis (HD) en un país de bajos a medianos ingresos. Se llevó a cabo un estudio transversal para determinar los medicamentos más utilizados en una unidad de hemodiálisis pediátrica, incluyendo el nombre del medicamento, dosis, frecuencia, forma farmacéutica y vía de administración. Dos farmacias dentro del perímetro del hospital, una pública y una privada, fueron consultadas para determinar el costoy disponibilidad de medicamentos genéricos y de marca. De un total de 30 pacientes de la unidad de hemodiálisis, 22 expedientes fueron revisados. En general 94% de marca se encontraban disponibles en las farmacias consultadas en comparación a un 52% de los medicamentos genéricos. En farmacias públicas, 41% de medicamentos de marca y 29% de medicamentos genéricos se encontraban disponibles. El costo promedio para un mes de tratamiento con medicamentos de marca adquiridos en una farmacia privada era de $495.00 vs $299.00 en una farmacia pública. Para medicamentos genéricos, el costo promedio correspondía a $414.00 y $239.00 en farmacias privadas y públicas respectivamente. En promedio, los medicamentos de marca adquiridos en una farmacia privada requieren 41 días de trabajo en un mes a comparación de 25 días si se adquieren en una farmacia pública. Los medicamentos genéricos adquiridos en farmacias privadas corresponden a 34 días de trabajo vs 20 días en farmacias públicas. En general existió un acceso limitado a medicamentos genéricos y los medicamentos poseen un costo general más elevado a comparación de otros países lo que implica un posible impacto en la adherencia terapéutica y los padecimientos secundarios de la ERC en los pacientes pediátricos en Guatemala. Esta realidad se puede aplicar a otros países de bajos a medianos ingresos.

This article aims to analyze the availability, access, and affordability of medications for children with advanced Chronic Kidney Disease (CKD) treated with hemodialysis (HD) in a low to middle income country (LMIC). A cross- sectional chart review was carried out to determine the most common medications used in an HD pediatric unit, including medication name, dose, frequency, dosage form, and route of administration. Two pharmacies within the hospital perimeter, one public and one private, were consulted to determine medication cost and availability for generic and brand-name equivalents. From 30 patients attending the HD unit, 22 records were reviewed. Overall, 94 % of brand name medications were available at pharmacies consulted, versus and 52% of generic medications. In public pharmacies, 41% of brand name, and 29% of generic medications were available. The average cost for a full month´s treatment for brand name drugs in the private pharmacy was 495.00 USD versus 299.00 USD in the public pharmacy. For generic drugs, the average cost was 414.00 USD, and 239.00 USD in private and public pharmacies respectively. On average, brand-name drugs in the private pharmacy cost 41 days' wages versus 25 in the public pharmacy. Generic drugs in the private pharmacy cost 34 days' wages versus 20 in the public pharmacy. Overall, there was limited access to generic medications, medications had an overall high cost compared to other countries both of which have the potential to impact treatment adherence and overall outcomes of CKD5 pediatric patients in Guatemala. This reality can be translated to other LMIC.

Mesoamerican Endemic Nephropathy (MeN): A Disease Reported in Adults That May Start Since Childhood?

Mesoamerican endemic nephropathy (MeN) is a type of chronic kidney disease (CKD) of uncertain etiology that occurs along the Pacific coast of the southern part of Mexico and Central America. During the past 20 years MeN has become a leading cause of death in the region, clamming close to 50,000 lives, with 40% of these deaths occurring in young people. The cause remains unknown, but most researchers believe in a multifactorial etiology that includes social determinants of poverty. Existing evidence suggests that subclinical kidney injury begins early in life and leads to a higher than expected prevalence of CKD among children in Central America. Access to health services in the region, specifically kidney replacement therapy, remains limited. We proposed a strategy to address the perceived needs and urge coordinated efforts of governments, academic organizations, and international bodies to develop a comprehensive plan of action to mitigate this situation among the vulnerable and economically disadvantaged population.

Mortalidad hospitalaria por disfunción ventricular derecha diagnosticada por ecocardiografía en pacientes con sepsis y shock séptico, atendidos de mayo a agosto de 2022, hospital militar central / Hospital mortality due to right ventricular dysfunction diagnosed by echocardiography in patients with sepsis and septic shock, seen from May to August 2022, central military hospital

Los pacientes con sepsis y shock séptico presentan una alta mortalidad, representan un importante problema de salud; en estos pacientes, la incidencia de depresión miocárdica es conocida y ha sido documentada. La presente investigación encuentra la mortalidad asociada a la disfunción ventricular derecha, diagnosticada por ecocardiografía transtorácica al pie de cama, que se presenta o desarrolla secundaria a sepsis y su relación con la mortalidad, destacando la utilidad diagnóstica de esta técnica de imagen a través de la evaluación de parámetros ecocardiográficos medíbles y de fácil para cuantificar al lado de la cama del paciente. Metodología: fue un estudio observacional prospectivo analítico de cohorte única, en el Hospital Militar Central, San Salvador, El Salvador, diseñado para incluir pacientes atendidos o ingresados al servicio de medicina interna y cuidados intensivos en un periodo de 4 meses durante el año 2022, que cumplan con los criterios de sepsis y shock séptico, según sepsis-3 utilizando el ecocardiograma transtorácico. Se realizo el ecocardiograma como técnica para evaluar la función del ventrículo derecho (VD) en dos momentos, en las primeras 48 horas y el día 4 desde el diagnóstico de sepsis o shock séptico, definimos disfunción ventricular derecha (DVD) según el tipo: disfunción sistólica VD: TAPSE <1,6 cm, disfunción diastólica VD: cociente E/A <0,8 >2, se cuantificó el balance hídrico acumulado hasta el día 4. Se dio seguimiento a cada paciente para registrar muerte o egreso hospitalario en condición de vivo, relacionando estos desenlaces con disfunción ventricular derecha y balance hídrico. Análisis y resultados: La mortalidad se expreso mediante la razón proporcional de mortalidad intrahospitalaria asociada a DVD, se registró en 46,15%, RR: 1,81, la mortalidad fue mayor en quienes presentaron disfunción diastólica del ventrículo derecho. Interpretación: La disfunción del ventrículo derecho de cualquier tipo o global y el balance de líquidos mayor a 1 litro, se asoció directamente con una mayor mortalidad intrahospitalaria.

Patients with sepsis and septic shock carry high mortality even today, which is why it represents an important health problem; In these patients, the incidence of myocardial depression is known and has been documented. Research: to know the associated mortality with right ventricular dysfunction, diagnosed by transthoracic echocardiography at the bedside, which presents or develops secondary to sepsis and its relationship with mortality, highlighting the diagnostic utility of this imaging technique through the evaluation of measurable echocardiographic parameters and easy to quantify at the patient's bedside. Study Design and Methods: The Study is Observational Prospective Analytical Single Cohort, Monocentric (Hospital Militar Central, San Salvador, El Salvador). designed to include a cohort treated or admitted to the internal medicine and intensive care service in a period of 4 months during the year 2022, who meet the criteria for sepsis and septic shock, according to sepsis-3, a transthoracic echocardiogram was used as a research technique for those who had their right ventricular(RV) function evaluated in two moments, the first 48 hours and on day 4 from the diagnosis of sepsis or septic shock, to To document the presence or absence of an exposure factor: Right Ventricular Dysfunction (DVD) of any type, we defined RV Systolic Dysfunction: TAPSE <1.6 cm, RV Diastolic Dysfunction: E/A ratio <0.8 >2, and the accumulated fluid balance was quantified up to the day 4. Consecutively, each patient was followed up to record in-hospital death or Discharge in Living Condition, relating these outcomes with Right ventricular dysfunction and fluid balance. Results: The proportional ratio of Intrahospital Mortality due to DVD was recorded at 46.15%, RR: 1.81, Mortality was higher in those who presented diastolic dysfunction of the Right Ventricle. Interpretation: Right ventricular dysfunction of any type or global together with fluid balance greater than 1 liter, was directly associated with increased in-hospital mortality.

Hemolytic uremic syndrome in children in Puerto Rico: a rare disease with atypical features.

Hemolytic Uremic Syndrome (HUS) consists ofa triad of acquired hemolytic anemia, thrombocytopenia, and renal failure that occurs acutely in otherwise healthy individuals. HUS may be divided into two broad categories, typical, preceded by a diarrheal prodrome, and atypical. The clinical symptoms of HUS as well as its course, prognosis, and response to treatment appear to be significantly influenced by a number of factors, including age at onset, type and severity of underlying infections, and/or systemic diseases. A retrospective case series review of five patients diagnosed with Hemolytic Uremic Syndrome at the Pediatric University Hospital in Puerto Rico between 1997-2007 was performed. The study showed that the incidence of HUS in children in Puerto Rico is lower than other countries. However, the majority of cases have an atypical presentation, which places our patients at higher risk for life-threatening complications.

Lupus nephritis in Puerto Rican children and adolescents.

BACKGROUND: Lupus nephritis is associated with an unfavorable outcome. Its incidence is higher in children. It has been the impression of pediatric nephrologists and rheumatologists in Puerto Rico that the clinical manifestations in Puerto Rican children differ from what has been described in other geographic regions. The potential deleterious effects of sun exposure could suggest that Puerto Rican children living in the Island are at high risk for severe nephritis. PURPOSE/METHOD: To describe the manifestations and identify predictors of patient and renal survival, we reviewed the medical charts of children diagnosed with lupus nephritis in Puerto Rico between 1987-2001. RESULTS: Mesangial glomerulonephritis (Type II) was the most common histopathological lesion observed. We observed a high incidence of nephritis among males, with a male: female ratio of 1:1.9. CONCLUSIONS: The presence of type IV nephritis and need for acute dialysis at presentation were identified as predictors of poor clinical outcome (development of end stage renal disease or death).

Neonatal presentation of Gordon syndrome.

Gordon syndrome, the association of hypertension with hyperkalemic acidosis, has been described in older children and adults. We report an affected family in which two of the members had exhibited the metabolic manifestations of the disease since infancy. Both patients responded well to thiazides. To our knowledge, these are the youngest patients with documented cases of Gordon syndrome.

Renal acidification in children with idiopathic hypercalciuria.

Distal renal tubular acidosis is frequently associated with hypercalciuria. To further investigate the cause-and-effect relationships between the two conditions, we examined 20 children (5 to 18 years of age) with idiopathic hypercalciuria for evidence of renal tubular acidosis. Serum electrolytes and urine citrate levels were normal in all subjects. After a single dose of furosemide, 1 of the 20 subjects did not show a decrease in urine pH < 5.5, which suggests an acidification defect in the cortical collecting duct. Three other patients failed to show an increase in urine-minus-blood partial pressure of carbon dioxide > 20 mmHg after urine alkalinization with orally administered acetazolamide, a finding compatible with a rate-dependent distal renal tubular acidosis. These four subjects underwent acute acid loading with arginine hydrochloride. In all four subjects urine pH decreased < 5.5 but urinary ammonium excretion failed to increase normally; this supports the diagnosis of a defect in distal acidification. Four of six patients with nephrolithiasis had evidence of distal renal tubular acidosis, in contrast to none of the 14 patients without stones (p = 0.003). We conclude that distal acidification abilities seem to be intact in children with hypercalciuria in the absence of nephrolithiasis. We speculate that calcium precipitation may lead to tubular damage, including distal renal tubular acidosis.

Vólvulo del sigmoide / Sigmoid volvulus

La incidencia del vólvulo del sigmoides ha aumentado definitivamente. Hay que sospechar esta entidad en pacientes ancianos con distensión abdominal y especialmente en aquellos con enfermedades psiquiátricas o trastornos neurológicos, que están crónicamente encamados. Se recomienda la técnica del uso de la rectosigmoidoscopía y el tubo rectal por cuarenta y ocho horas, concomitantes con hidratación parenteral y sonda nasogástrica. La reducción no operatoria libera la obstrucción colónica y previene la gangrena y debe ser seguida dos semanas después de resección electiva previa preparación mecánica y química. En presencia de gangrena se prefiere la resección primaria o técnica de Hartman. Al lograr una adecuada preparación del colon, un adecuado equilibrio hidroelectrolítico y condición del paciente en el pre y post operatorio, disminuye la mortalidad post operatoria y las complicaciones. Se presenta un caso de paciente masculino con vólvulo del sigmoides; se establecen criterios fisiopatológicos, diagnósticos y de tratamiento

Tumor carcinoide gástrico / Gastric carcinoid tumor

Se reporta un caso de Tumor Carcinoide Gástrico y se efectúa revisión de los aspectos histopatológicos, clínicos, diagnósticos y de tratamiento de esta entidad