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Background: Compared to the past, patients with sickle cell disease (SCD) currently live longer due to improvements in diagnosis and comprehensive care. Due to these advances, long-term chronic complications pose a greater challenge in the management of patients with SCD. In particular, sickle cell nephropathy (SCN) is associated with significant morbidity and mortality across all age groups. Furthermore, SCN is an understudied condition with relatively few symptoms and therefore requires close surveillance. In this review, we sought to explore the epidemiology, natural history, and treatment options for SCN with an emphasis on the pediatric population. Summary: SCN invariably begins in childhood with evidence of structural changes detected as early as infancy. These indolent changes can progress undetected to advanced chronic kidney disease by late adolescence or early adulthood. The risk factors for progression are not well defined, but significant albuminuria (which is also the most common presentation in childhood) is a key factor in progression. One of the main challenges in understanding SCN in children is the poor correlation between estimated and measured glomerular filtration rates. Another challenge is the lack of large-scale longitudinal studies that track the clinical outcomes of pediatric patients over time. Several studies aim to identify early biomarkers of SCN in children, as albuminuria presents only following significant chronic damage. The utility of angiotensin converting enzyme inhibitors and hydroxyurea in treating albuminuria is addressed here as well as novel treatments that may be of benefit. [ABSTRACT FROM AUTHOR]

INTRODUCTION For two decades, various countries have experienced an epidemic of chronic kidney disease unexplainable by traditional causes. Typically a chronic tubulointerstitial nephritis, it predominantly affects young male agricultural workers and has been reported in several countries in Central America, as well as in Sri Lanka, India and Egypt. Recent studies in El Salvador have also reported the disease in male nonagricultural workers and in women, both those working in agriculture and not. Epidemiological information on whether this condition affects pediatric populations is virtually nonexistent. Globally, estimates of chronic kidney disease prevalence in pediatric populations range from 21 to 108 per million population. OBJECTIVE Determine the prevalence of urinary markers of renal damage and of chronic kidney disease in persons aged <18 years in rural Salvadoran communities. METHODS Pediatric NefroSalva was a descriptive epidemiologic study in three agricultural regions with known high prevalence of chronic kidney disease of uncertain etiology: Bajo Lempa, Guayapa Abajo and Las Brisas. Demographic and health data were collected and physical measurements were taken for 2115 persons aged <18 years, 1058 boys and 1057 girls. Urine samples were tested for markers of renal damage and blood samples analyzed to measure creatinine for estimating glomerular filtration rate (Schwartz formula). Median glomerular filtration rate was compared with reference values for age groups 2–12 and 13–17 years; mean glomerular filtration rate trends were assessed for age groups 2–5, 6–12 and 13–17 years. Positive test results were confirmed after three months. RESULTS Prevalence of urinary markers of renal damage was 4%, 4.3% in girls and 3.8% in boys. Microalbuminura (albumin:creatinine ratio 30–300 mg/g) was detected in both sexes and all age groups in all three regions, with prevalences of 2.6%–3.8% in boys and 3.3%–3.8% in girls. Macroalbuminuria (albumin:creatinine ratio >300 mg/g) was detected only in girls in Las Brisas, 2.3%. Glomerular hyperfiltration (compared to international norms) was found in all age–sex groups in all three regions. Prevalence of chronic kidney disease was 3.9%—4.1% in girls and 3.6% in boys. The prevalence of chronic renal failure was 0.1%. CONCLUSIONS High prevalence of chronic kidney disease in children and adolescents calls attention to the need for primary prevention from very early ages. This finding in children in areas where chronic kidney disease of uncertain etiology is common in adults is consistent with a contribution of environmental toxins to the epidemic observed in these areas. [ABSTRACT FROM AUTHOR]

In El Salvador, a form of chronic kidney disease (CKD) of nontraditional causes (CKDnt) affecting farmers is being reported. Its behavior has been epidemic and is responsible for tens of thousands of deaths. This article summarizes the results obtained from a series of studies conducted to identify the epidemiology and clinical behavior of this disease, proposing a case definition and an etiopathogenic hypothesis. Methods included a survey of CKD in agricultural communities studying 2,388 people ≥ 18 years and 1,755 < 18, a descriptive clinical study followed by histopathological assessment conducted in 46 possible cases of CKDnt ≥ 18 years, and a national survey to study the prevalence of CKD and associated risk factors in 4,817 participants ≥ 20 years followed by a nested case-control study. In the agricultural communities, the prevalence of CKD in adults was 18% (men: 23.9%, women: 13.9%), 26.8% in agricultural workers (non-agricultural 13.8%), CKDnt accounted for 51.9% of cases. CKD in the population < 18 years was 3.9% (mean estimated glomerular filtration rate > 160 mL/1.73m 2 ). The national CKD prevalence was 12.6% (urban: 11.3%; rural: 14.4%; males: 17.8%, females 8.5%), and CKDnt was only 3.8%; with associations between CKD and exposure to agrochemicals. The clinical study revealed the presence of markers of kidney damage (A3 albuminuria: 80.4%; β2-microglobulin: 78.2%), urine electrolyte anomalies (100% hypermagnesuria, 45.7% hypernatriuria, 43.5% osmotic polyuria), abnormal osteotendinous reflexes (45.7%), sensorineural hearing loss (56.5%), and damage of the tibial arteries by Doppler imaging (66.7%). Biopsies revealed a chronic tubulointerstitial nephropathy. The etiopathogenesis of CKDnt is possibly multifactorial, including environmental contamination by agrochemicals, heat stress, and dehydration.

Introduction: A chronic kidney disease of non-traditional causes (CKDu) has emerged in Central America and elsewhere, predominantly affecting male farmworkers. In El Salvador (2009), it was the second cause of death in men > 18 years old. Causality has not been determined. Most available research focused on men and there is scarce data on women., Objectives: Describe the clinical and histopathologic characteristics of CKDu in women of agricultural communities in El Salvador., Methodology: A descriptive study was carried out in 10 women with CKDu stages 2, 3a, and 3b. Researchers studied demographics, clinical examination; hematological and biochemical analyses, urine sediment, renal injury markers, and assessed renal, cardiac, and peripheral arteries, liver, pancreas, and lung anatomy and functions. Kidney biopsy was performed in all. Data was collected on the Lime Survey platform and exported to SPSS 19.0., Results: Patient distribution by stages: 2 (70%), 3a (10%), 3b (20%). Occupation: agricultural 7; non-agricultural 3., Risk Factors: agrochemical exposure 100%; farmworkers 70%; incidental malaria 50%, NSAIDs use 40%; hypertension 40%., Symptoms: nocturia 50%; dysuria 50%; arthralgia 70%; asthenia 50%; cramps 30%, profuse sweating 20%. Renal markers: albumin creatinine ratio (ACR) > 300 mg/g 90%; β microglobulin and neutrophil gelatinase- associated lipocalin (NGAL) presence in 40%. Kidney function: hypermagnesuria 100%; hyperphosphaturia 50%, hypercalciuria 40%; hypernatriuria 30%; hyponatremia 60%, hypocalcemia 50%. Doppler: tibial artery damage 40%. Neurological: reflex abnormalities 30%; Babinski and myoclonus 20%. Neurosensorial hypoacusis 70%. Histopathology: damage restricted mostly to the tubulo-interstitium, urine was essentially bland., Conclusions: CKDu in women is a chronic tubulointerstitial nephropathy with varied extrarenal symptoms.

Introduction: In El Salvador end-stage renal disease (ESRD) was the first cause of hospital mortality overall, the first cause of hospital deaths in men, and the fifth cause of hospital mortality in women in 2013. In agricultural communities, chronic kidney disease (CKD) occurs predominantly in male agricultural workers, but it also affects women to a lesser degree, even those who are not involved in agricultural work. Internationally, most epidemiological CKD studies emphasize men and no epidemiological studies focused exclusively on women., Objective: To describe the epidemiological characteristics of CKD in females in agricultural communities of El Salvador., Methods: A cross-sectional epidemiological study was carried out in 2009 - 2011 based on active screening for CKD and risk factors in women aged ≥ 18 years in 3 disadvantaged populations of El Salvador: Bajo Lempa (Usulután Department), Guayapa Abajo (Ahuachapán Department), and Las Brisas (San Miguel Department). Epidemiological and clinical data were gathered through personal history, as well as urinalysis for renal damage markers, determinations of serum creatinine and glucose, and estimation of glomerular filtration rates. CKD cases were confirmed at 3 months., Results: Prevalence of CKD was 13.9% in 1,412 women from 1,306 families studied. Chronic kidney disease of nontraditional causes (CKDu), not attributed to diabetes mellitus, hypertension, or proteinuric primary glomerulopathy (proteinuria > 1 g/L) was 6.6%. Prevalence of chronic renal failure was 6.8%. Prevalence of renal damage markers was 9.8% (microalbuminuria (30 - 300 mg/L) 5.7%; macroalbuminuria (> 300 mg/L) 2%; and hematuria, 2.1%. Prevalence of chronic kidney disease risk factors was: diabetes mellitus, 9.3%; hypertension, 23%; family history of CKD, 16%; family history of diabetes mellitus (DM), 18.7%; family history of hypertension (HT), 31.9%; obesity, 21%; central obesity, 30.7%; NSAID use, 84.3%; agricultural occupation, 15.2%; and contact with agrochemicals, 33.1%., Conclusions: CKD in women of Salvadoran agricultural communities is associated with disadvantaged populations, traditional (DM, HT, obesity) and non-traditional causes (environmental and occupational exposure to toxic agents and inadequate working conditions). Our results reinforce the hypotheses emerging from other studies, suggesting a multifactorial etiopathology including environmental and occupational nephrotoxic exposure.