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.

Addressing Health Inequities and Disparities in Children With Kidney Disease in Disadvantaged Areas: the Latin American and Caribbean Experience.

Purpose of Review: Compared with high-income countries, healthcare disparities and inequities are more evident in low, lower-middle, and upper-middle-income countries with poorer housing and nutrition conditions. At least 20% of Latin America and the Caribbean are low and lower-middle-income countries. Despite the majority of the other countries being upper-middle income, the United Nations Children's Fund had classified all the regions as "less developed," with limited access to health care for the most vulnerable, the children. Latin America and the Caribbean regions represent an extensive territory with communication limitations and an unstable socio-political and economic environment. After considering the vast population affected by poverty worldwide and the long-term impact of kidney disease starting in childhood, it is crucial to better understand and analyze the multifactorial limiting conditions in accessing specialized care such as pediatric nephrology in disadvantaged areas. Recent Findings: Constraints in accessing basic healthcare in rural areas make it impossible to receive specialized pediatric nephrology care including dialysis and transplantation. Disturbingly, incidence and prevalence figures of acute kidney injury, chronic and end-stage kidney disease in some Latin American and the Caribbean countries are unknown, and these conditions still represent a death sentence for underprivileged populations. However, the monumental efforts of the dedicated healthcare providers and stakeholders that pioneered the actions in the past 50 years have shown remarkable progress in developing pediatric nephology services across the continent. Summary: In this review, we compile some of the latest evidence about the care of children and adolescents with kidney conditions in Latin America and the Caribbean, along with the experiences from the field in the care of these patients facing adverse conditions. We also highlight recommendations to address inequities and disparities.

IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome.

Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. Approximately 85-90% of patients attain complete remission of proteinuria within 4-6 weeks of treatment with glucocorticoids, and therefore, have steroid-sensitive nephrotic syndrome (SSNS). Among those patients who are steroid sensitive, 70-80% will have at least one relapse during follow-up, and up to 50% of these patients will experience frequent relapses or become dependent on glucocorticoids to maintain remission. The dose and duration of steroid treatment to prolong time between relapses remains a subject of much debate, and patients continue to experience a high prevalence of steroid-related morbidity. Various steroid-sparing immunosuppressive drugs have been used in clinical practice; however, there is marked practice variation in the selection of these drugs and timing of their introduction during the course of the disease. Therefore, international evidence-based clinical practice recommendations (CPRs) are needed to guide clinical practice and reduce practice variation. The International Pediatric Nephrology Association (IPNA) convened a team of experts including pediatric nephrologists, an adult nephrologist, and a patient representative to develop comprehensive CPRs on the diagnosis and management of SSNS in children. After performing a systematic literature review on 12 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, recommendations were formulated and formally graded at several virtual consensus meetings. New definitions for treatment outcomes to help guide change of therapy and recommendations for important research questions are given.

Effects of Prematurity and Growth Restriction on Adult Blood Pressure and Kidney Volume.

Gaining insight into the complex cycle of renal programming and its early-life clinical associations is essential to understand the origins of kidney disease. Prematurity and intrauterine growth restriction are associated with low nephron endowment. This increases the risk of developing hypertension and chronic kidney disease later in life. There is appreciable evidence to support mechanistic links between low nephron endowment secondary to intrauterine events and kidney size, kidney function, and blood pressure in postnatal life. A clear understanding of the cycle of developmental programming and consequences of fetal insults on the kidney is critical. In addition, the impact of events in the early postnatal period (accelerated postnatal growth, development of obesity, exposure to nephrotoxins) on the cardiovascular system and blood pressure of individuals born prematurely or with low birth weight is discussed. In summary, this review draws attention to the concepts of renal programming and nephron endowment and underscores the associations between intrauterine growth restriction, prematurity, and its clinical consequences in adult life.

PCRRT-ICONIC critical care pediatric nephrology course: the global prevalence of COVID-19 and associated sequelae.

After nearly three years of the COVID-19 pandemic, research has affirmed that COVID-19 is more than just a respiratory virus. There have been significant breakthroughs made surrounding the development of acute kidney injury (AKI) and chronic kidney disease (CKD), in pediatric populations. Additionally, patient populations susceptible to renal complications consist of pediatric transplant recipients, multisystem inflammatory syndrome (MIS-C), and dialysis. Although research is gradually becoming more available surrounding this prevalent topic, knowledge is sparse on the deleterious effects of COVID-19 on pediatric patients with kidney disease and requires more in-depth analysis. The virtual international conference, Pediatric Critical Care Nephrology & Dialysis Course, on August 7th, 2021, reviewed the severe cases of COVID-19 in the global pediatric population. By integrating international perspectives, statistics, techniques, and treatments for managing renal complications, we further develop scientific understanding of the renal complications seen in children with COVID-19 globally.

Paediatric nephrology in under-resourced areas.

BACKGROUND: Nearly 50% of the world population and 60% of children aged 0 to 14 years live in low- or lower-middle-income countries. Paediatric nephrology (PN) in these countries is not a priority for allocation of limited health resources. This article explores advancements made and persisting limitations in providing optimal PN services to children in such under-resourced areas (URA). METHODS: Medline, PubMed and Google Scholar online databases were searched for articles pertaining to PN disease epidemiology, outcome, availability of services and infrastructure in URA. The ISN and IPNA offices were contacted for data, and two online questionnaire surveys of IPNA membership performed. Regional IPNA members were contacted for further detailed information. RESULTS: There is a scarcity of published data from URA; where available, prevalence of PN diseases, managements and outcomes are often reported to be different from high income regions. Deficiencies in human resources, fluoroscopy, nuclear imaging, immunofluorescence, electron microscopy and genetic studies were identified. Several drugs and maintenance kidney replacement therapy are inaccessible to the majority of patients. Despite these issues, regional efforts with support from international bodies have led to significant advances in PN services and infrastructure in many URA. CONCLUSIONS: Equitable distribution and affordability of PN services remain major challenges in URA. The drive towards acquisition of regional data, advocacy to local government and non-government agencies and partnership with international support bodies needs to be continued. The aim is to optimise and achieve global parity in PN training, investigations and treatments, initially focusing on preventable and reversible conditions.

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.

Importance of clinical practice guidelines to practicing pediatric nephrologists and IPNA survey.

Clinical practice guidelines (CPGs) are systematically developed statements backed by scientific evidence to assist practitioners in management in clinical practice. An international cross-sectional survey was conducted by the IPNA to examine the perceptions of pediatric nephrologists on guidelines and their usage and to identify important diseases for future clinical practice guidelines (CPGs). The survey found that the majority of pediatric nephrologists find CPGs useful in clinical practice and admitted to using them most of the time. Developing CPGs is challenging and there are standards available to develop trustworthy guidelines. While evidence-based global guidelines are ideal, pediatric nephrologists expressed the desire that they address regional differences. Most respondents (89.2%) to the survey agreed that adult guidelines did not cover the pediatric perspective adequately and 71.4% opined that consensus-based pediatric guidelines can be developed when evidence for the pediatric population is lacking. The development of high-quality practice guidelines requires substantial resources and may not be feasible in resource-poor countries. Adaptation of an existing guideline has been suggested as an alternative and the ADAPTE collaboration provides a systematic approach to adapting guidelines. Several diseases where pediatric guidelines are needed as a priority including IgA and C3 glomerulopathy were identified in the survey. Implementation of guideline-based care is challenging and the survey found that lack of availability of guidelines (43%) and resources (22.8%) are important reasons for poor implementation in lower-middle and low-income countries. Perceived complexity of guidelines, physician attitudes, and lack of training also contribute to non-adherence to guidelines.

Disaster Management in a Nephrology Service: Lessons Learned from Hurricane Maria.

BACKGROUND: Puerto Rico suffered a major humanitarian -crisis after Hurricane Maria. We describe our experience with patients with renal disease in an academic medical center. SUMMARY: A comprehensive emergency response plan should be developed, shared and discussed with the team and the patients prior to the hurricane. The needs of the staff should not be ignored to ensure their ability to participate as responders. Physical damage to facilities, lack of basic services, shortage of disposable products, and the inability to get to treatment centers are the most common threats. Preemptive dialysis can avoid serious complications. A contingency plan to move patients to another center should be prearranged in case the unit is rendered nonfunctional after the storm. Patients must receive preventive education about fluid and dietary restrictions and the possible use of potassium binding drugs if they cannot reach a dialysis unit. A list of alternative drugs that could be used if patients are not able to fill their medications is required. The Internet and social media proved to be an invaluable communication tool. A registry of patients with updated contact information, as well as contact information for relatives and a physical address where an emergency rescue team can be dispatched is essential. Water safety should be reinforced. Key Message: Our experience showed us that preparing for the worst is not enough. Advanced planning of a streamlined response is the best tactic to decrease harm.

Potassium regulation in the neonate.

Potassium, the major cation in intracelluar fluids, is essential for vital biological functions. Neonates maintain a net positive potassium balance, which is fundamental to ensure somatic growth but places these infants, especially those born prematurely, at risk for life-threatening disturbances in potassium concentration [K+] in the extracellular fluid compartment. Potassium conservation is achieved by maximizing gastrointestinal absorption and minimizing renal losses. A markedly low glomerular filtration rate, plus adaptations in tubular transport along the nephron, result in low potassium excretion in the urine of neonates. Careful evaluation of clinical data using reference values that are normal for the neonate's postmenstrual age is critical to avoid over-treating infants with laboratory results that represent physiologic values for their developmental stage. The treatment should be aimed at correcting the primary cause when possible. Alterations in the levels or sensitivity to aldosterone are common in neonates. In symptomatic patients, the disturbances in [K+] should be corrected promptly, with close electrocardiographic monitoring. Plasma [K+] should be monitored during the first 72 h of life in all premature infants born before 30 weeks of postmenstrual age as these infants are prone to develop non-oliguric hyperkalemia with potential serious complications.

Fluid and electrolyte disorders in the newborn: sodium and potassium.

Tubular development continues after birth in full and pre-term infants. As the survival of premature infants increases, serious imbalances in water and electrolytes in this group have become more prevalent. A diminished ability of the immature kidney to reabsorb water and respond to mineralocorticoids, a high excretion of filtered sodium, perinatal complications affecting tubular function, and the use of medications such as diuretics, indomethacin and amphotericin B, are common factors leading to sodium and potassium imbalances in this age group. Appropriate diagnosis and treatment should be guided by a careful assessment of volume status, urine electrolytes and osmolality.

Peritoneal dialysis in the pediatric intensive care unit setting: techniques, quantitations and outcomes.

Acute kidney injury (AKI) is a common complication in pediatric and neonatal intensive care units (ICU). Renal replacement therapy (RRT) is frequently needed in children in whom supportive therapy is not enough to satisfy their metabolic demands or to be able to provide adequate nutrition. The decision to begin dialysis should not be delayed since experience in infants shows that the shorter the time from the insult to the beginning of dialysis, the higher the survival rate. As the use of continuous RRT in pediatric patients in the ICU has almost tripled, the use of peritoneal dialysis (PD) and intermittent hemodialysis has markedly decreased. The patient's age seems to be the most important factor influencing the decision on the choice of dialysis modality. PD is still the most common modality used in patients younger than 6 years of age. The relatively low cost, technical simplicity, no need for anticoagulation or placement of central venous catheters, and excellent tolerance in hemodynamically unstable patients are among the most significant advantages of PD. Much controversy exists regarding the adequacy of PD in hypercatabolic patients in the ICU. Nonetheless, when Kt/V has been applied to acutely ill children, it has been shown that PD can provide adequate clearances for most infants. The outcomes of critically ill patients with AKI treated with PD are comparable to other dialysis modalities. Therefore, the decision about dialysis modality should be based on local expertise, resources available, and patient's clinical status.

Peritoneal dialysis in the pediatric intensive care unit setting.

Acute kidney injury (AKI) is a common complication in pediatric and neonatal intensive care units (ICUs). Renal replacement therapy (RRT) is frequently needed in children in whom supportive therapy is not enough to satisfy metabolic demands or to provide adequate nutrition in cases of oliguric kidney failure. The decision to begin dialysis should not be delayed, because experience in infants shows that the shorter the time from the ischemic insult to the beginning of dialysis, the higher the survival rate. The use of continuous RRT (CRRT) in pediatric patients in the ICU has almost tripled; at the same time, the use of peritoneal dialysis (PD) and intermittent hemodialysis has markedly declined. Patient age seems to be the most important factor influencing the decision on the choice of dialysis modality. Although CRRT is reported as the preferred dialysis modality for acutely ill children, PD is still the most common modality used in patients under 6 years of age. Among the several advantages that PD offers, relatively low cost is probably the most significant. Other advantages include technical simplicity, lack of a need for anticoagulation or placement of a central venous catheter, and excellent tolerance in hemodynamically unstable patients. Much controversy exists regarding the adequacy of PD in hypercatabolic patients in the ICU. Nonetheless, when Kt/V has been applied to acutely ill children, it has been shown that PD can provide adequate clearances for most infants. No prospective studies have evaluated the effect of dialysis modality on the outcomes of children with AKI in the ICU setting. The decision about dialysis modality should therefore be based on local expertise, resources available, and the patient's clinical status.

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).