Diagnostic Challenges of Urinary Schistosomiasis Among Adolescent Immigrants in New York City: A Report of Three Cases.

Urinary schistosomiasis is endemic in the tropical world. It is uncommon in geographical areas with advanced public health resources. Modern immigration from endemic communities to the United States supports the need to improve our diagnostic awareness. We describe 3 Brooklyn adolescent immigrants from Africa with urinary schistosomiasis, all of whom had an initial misdiagnosis that led to delay in therapeutic intervention.

Structural Inequities and Barriers to Accessing Kidney Healthcare Services in the United States: A Focus on Uninsured and Undocumented Children and Young Adults.

The population of children living in poverty and lacking healthcare insurance has increased in the United States of America in the last decade. Several factors have been responsible for this trend including illegal immigration, socioeconomic deprivation, young age, racial segregation, environmental degradation, and discriminatory housing policies. These systemic barriers have contributed to the exclusion of families from essential healthcare services. They are also contributory to the development of chronic illnesses (such as dialysis-dependent kidney disease) that are debilitating and frequently require considerable therapeutic resources. This unfortunate scenario creates a never-ending vicious cycle of poverty and diseases in a segment of society. For pediatric nephrologists, the challenges of caring for uninsured children with chronic kidney disease are all too familiar. Federally funded healthcare programs do not cover this patient population, leaving them the option of seeking care in emergency healthcare settings. Presentation with a critical illness often necessitates urgent placement of vascular catheters and the choice of acute hemodialysis. Adverse social environment influences the need for protracted chronic hemodialysis and a delay in kidney transplantation. Consequently, there is greater comorbidity, recurrent hospitalization, and a higher mortality rate. New policies should address the deficit in health insurance coverage while promoting social programs that will remove structural barriers to health care resources for undocumented children and young adults.

Infective dermatitis associated with HTLV-1 infection in a girl from Trinidad: Case report and review of literature.

Infective dermatitis (ID) associated with Human T-cell leukemia virus type-1 (HTLV-1) is a rare form of severe superinfected eczema seen mostly in the Caribbean islands and Latin America. Although rapid response to antibiotic treatment is observed, patients should be monitored for development of complications associated with this retroviral infection, including T-cell leukemia/lymphoma (ATLL) and HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP). Infective dermatitis is rarely seen in the United States and therefore may be under-recognized by physicians unfamiliar with this condition. Herein, we present an additional case report of an ID associated with HTLV-1 in an 11-year-old girl from Trinidad.

Spectrum of anemia after kidney transplantation: pathophysiology and therapeutic implications.

The prevalence of anemia in the first month after transplant is 70%-80%. The rate declines to 30%-40% at 3 months and 20% by 12 months. Its occurrence is influenced by the quality of the transplanted organ, bone marrow regenerative capacity, amount of surgical blood loss, and increased iron recycling. There is also a blunted response by oxidative inflammation to the effectiveness of supranormal levels of erythropoietin (EPO) release during ischemic-reperfusion allograft injury. The prevalence rate of late-onset post-transplant anemia (PTA) is 30%-50%. This category of patients falls into two ill-defined groups: (i) those with impaired renal capacity for EPO synthesis and (ii) those with bone marrow resistance. Given the difference in pathophysiology, the current KDIGO guideline that adopts uniform therapeutic approach for the two groups may be inappropriate. Comorbidity due to iron deficiency is common. Anemia is predictive of cardiovascular morbidity and shorter graft survival. Perhaps due to concern for the safe use of EPO stimulating agent (ESA) to correct anemia, there is often inadequate treatment of late-onset PTA. However, universal application of ESA may be harmful. Therefore, clinical trials are needed to define parameters for selecting patients (e.g., EPO assay) that will benefit the most from therapy for anemia.

Urinary schistosomiasis.

Schistosomiasis is the second most common socio-economically devastating parasitic disease after malaria, affecting about 240 million residents of developing countries. In Africa, it predominantly manifests as urogenital disease, and the main infective agent is Schistosoma hematobium. Endemicity is propagated by poor socio-economic status and environmental degradation due to rapid urbanization. Recreational swimming is a potent medium for the spread of disease in children and adolescents. Most affected individuals are asymptomatic. The male and female worms are equipped with an extraordinary capacity for immune evasion and are able to co-habit for several decades within the pelvic venous plexus. Eggs deposited in the bladder wall resist elimination by type 1 T lymphocytes. Instead, they are sustained by pro-fibrogenic encapsulation (as modulated by type 2 helper cells). Progressive bladder disease results in obstructive uropathy and predisposes to (mostly) squamous cell carcinoma. Schistosomal glomerulopathy manifests as a clinical spectrum of asymptomatic proteinuria, nephrosis and/or nephritic syndrome. Findings on renal biopsy may be influenced by co-morbidity with Salmonella bacteria, amyloidosis and hepatitis C infection. Potentially fatal Katayama fever and spinal radiculopathy may ensue in tourists visiting an endemic zone. Early detection by urine microscopy is hampered by low urinary excretion rates of the parasite eggs. Although useful in travelers with newly acquired disease, the results of the serological antibody assay may be false positive in residents of an endemic zone. Cystoscopy, however, may be invaluable. Due to its safety, effectiveness and once-daily dosing, praziquantel is the drug of choice. An integrated approach that includes mass chemotherapy, environmental health programs and public health education is the most cost-effective preventive strategy.

Pilot validation of objective malnutrition-inflammation scores in pediatric and adolescent cohort on chronic maintenance dialysis.

BACKGROUND: In recognition of the challenges inherent with the use of single-item indices for the diagnosis of malnutrition-inflammation morbidity in pediatric dialysis patients, to enhance accuracy, we validated a composite scoring system in a pilot study. The objective malnutrition-inflammation score seeks to validate the use of a composite scoring system as a tool for assessing malnutrition-inflammation burden in a pediatric dialysis population. METHODS: We enrolled 20 patients on hemodialysis (n = 14) and peritoneal dialysis (n = 6) over a period of 12 months. We derived composite scores from selected indices of renal pathology, nutrition, dialysis adequacy, protein catabolism, and dialysis modality. We assessed reliability by a test-retest method and measured validity by defining the relationship of the indices with serum C-reactive protein in a multiple regression analysis. We calculated sensitivity, specificity, accuracy, and precision for the malnutrition-inflammation score. RESULTS: The mean age was 12.8 years (standard deviation = 6.1), and male-female ratio was 12:8. Patients (n = 8) with elevated serum C-reactive protein (>0.3 mg/dL) had higher composite score for malnutrition-inflammation morbidity. Similarly, the pediatric cohort on hemodialysis had higher score than those on peritoneal dialysis. Upon reliability testing, a low value of typical error (0.07) and high correlation coefficient (r = 0.95) supported validity of the instrument. Moreover, multiple regression analysis showed a strong predictive relationship (R(2) = 0.9, p = 0.03) between the indices and serum C-reactive protein. Sensitivity of malnutrition-inflammation score was 62.5%, specificity was 83%, accuracy was 75%, and precision was 71%. CONCLUSION: Using criterion-validation method, we established the potential use of multi-diagnostic approach to quantify malnutrition-inflammation morbidity in a pediatric dialysis cohort. Given the small sample size, large-scale population-specific studies are needed to ratify these findings and to demonstrate its clinical effectiveness.

Pattern of resistance to erythropoietin-stimulating agents in chronic kidney disease.

Routine administration of erythropoietin (EPO)-stimulating agents (ESAs) for the control of anemia has improved the quality of life of subjects with chronic kidney disease (CKD). However, a wide variation in individual response to ESA is often observed. The reasons for EPO resistance include demographic variables such as age and gender distribution, morbidity pattern, and modality of dialysis. Despite suggestions by observational data, there is no biological characteristic that puts children at a disadvantage for adequate response to ESA. On the contrary, children possess a superior capacity for red cell production, including extramedullary erythropoiesis. The reasons for larger requirement of ESA in children (than in adults) are greater inflammatory burden, disproportionate blood loss, and greater EPO dosing by pediatric physicians. To minimize the harmful (including fatal) consequences of EPO resistance, surveillance programs must replenish nutrient (for example, iron and folate) stores, minimize oxidative hemolysis, control hyperparathyroidism, avoid catheter infection, and optimize uremic clearance. This clinical approach is justified by the inadequacy of laboratory diagnosis of pertinent etiological factors. Indeed, the best proof for functional nutrient deficiency is often a therapeutic trial. Finally, there are upcoming therapeutic agents that exploit the capacity for an endogenous EPO synthesis in CKD subjects, and may therefore minimize the off-target effect of excess dosages.

Validation of a composite scoring scheme in the diagnosis of folate deficiency in a pediatric and adolescent dialysis cohort.

BACKGROUND: Laboratory indices are often poorly diagnostic of folate deficiency (FD). Compared with iron depletion in hemodialysis (HD) populations, the impact of FD is less appreciated. The composite scoring of hematologic indices of FD may facilitate a prompt and accurate diagnosis, and enhance operational research on folic acid therapy. OBJECTIVE: Our objectives were to (1) validate composite scores of folate diagnostic indices, and (2) determine the reliability index of the diagnostic tool. METHODS: A cohort of 30 subjects, with a mean age of 16 (SD +/- 3.2 years), on HD and erythropoietin (EPO) for a minimum of 3 months was studied. After a baseline hematologic assessment, routine folates were administered for 6 months. Composite FD scores (FDS) of baseline mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration (MCHC), random distribution width (RDW), and hemoglobin were determined. Validation and reliability statistics were then analyzed, using the quantitative change in red blood cell folate/plasma homocysteine, or EPO requirement after 6 months of folate use, as diagnostic criteria. RESULTS: The mean FDS for FD and non-FD subsets were 3.0 +/- 1.3 and 1.4 +/- 0.9, respectively (analysis of variance; P = .0001). The correlation coefficient, r(2), between FD total and FDS was 0.61 (P = .001), and the coefficient between 2 (weekly) values of RDW, MCV, MCH, and MCHC was >0.84 (P = .0001). Scoring tools derived from the first (P = .002) and second (P = .01) halves of the laboratory indices remained discriminatory for the FD and non-FD groups. Baseline serum folate is poorly specific for FD, whereas FD score >or=3 had sensitivity, specificity, and positive and negative predictive values close to 90%. CONCLUSIONS: Composite scoring of erythrocyte indices was predictive of the FD diagnosis, as defined by the quantitative response of red blood cell folate, homocysteine, and EPO dose to folate therapeutic intervention. The diagnostic items yielded a high reliability coefficient. The FDS scheme is a potential tool for the diagnosis and surveillance of FD, particularly in at-risk populations (e.g., dialysis subjects).

Endemic Esherichia coil O157:H7 infections and hemolytic-uremic syndrome in Oklahoma, 2002-2005.

Hemorrhagic enterocolitis caused by Escherichia coli O157:H7 and its complication of hemolytic-uremic syndrome (HUS) are well known from large outbreaks caused by contaminated meats and vegetables. However most cases may be endemic, not related to an outbreak. We identified cases of HUS in Oklahoma, 2002-2005, from the Inpatient Hospital Discharge Database of the Oklahoma State Department of Health (OSDH) and also the cases of HUS and E. coli O157:H7 reported to the OSDH. 110 cases of HUS were identified from the hospital discharge database; only 14 (12.7%) were reported to the OSDH; 122 cases of E. coli O157:H7 infections were reported to the OSDH. Of the 110 cases of HUS, only six (5.5%) patients in two separate clusters may have had a common source of infection. Although interpretation is limited by the few reports to OSDH, our data suggest that E. coli O157:H7 infections and HUS, presumably related to contaminated food, are endemic throughout Oklahoma.

Recombinant tissue plasminogen activator infusion for hemodialysis catheter clearance.

Hemodialysis (HD) catheter occlusion is a common cause of poor blood flow and inadequate dialysis. In order to address this problem in our pediatric dialysis unit, we elected to use short (2-h) infusions of low-dose recombinant tissue plasminogen activator (rtPA) for thrombolysis of occluded catheters. Catheters meeting diagnostic criteria for thrombosis were infused with 2.5 mg rtPA in 25 ml 0.9 normal saline over 2 h prior to dialysis. Retrospective data collection was carried out to assess the success of this procedure. Variables assessed included blood flow (Qb), transmembrane pressure (TMP) and venous pressure (VP) before and after rtPA infusion. Seven catheter thromboses in six patients were successfully treated with rtPA; there were significant improvements in Qb ( p <0.01), TMP ( p <0.01), and VP ( p <0.02). At 32 weeks after rtPA therapy, Kaplan-Meier survival analysis showed a 60% probability of primary catheter patency. At the end of the study, 85% of catheters had adequate function as defined by a Qb >200 ml/min. No adverse events were observed. Low-dose rtPA infusion is safe and effective for catheter thrombolysis in outpatient pediatric HD patients. It may serve as an alternative method of administration to local instillation and may be used to restore patency before resorting to surgical revisions.