El hierro (Fe) es un metal indispensable en el funcionamiento de diferentes sistemas enzimaticos ... more El hierro (Fe) es un metal indispensable en el funcionamiento de diferentes sistemas enzimaticos del organismo. En su mayoria se encuentra formando parte de la hemoglobina, en donde participa en el intercambio gaseoso en tejidos y pulmon; en otros sistemas enzimaticos celulares participa en diferentes mecanismos de oxido-reduccion. En condiciones normales se obtiene a partir de la dieta de donde se absorben aproximadamente 2 mg/dia, y la misma cantidad se pierde por la descamacion de epitelios, guardando un estrecho equilibrio. Esta cantidad es insuficiente para mantener la eritropoyesis, para la cual se utiliza el hierro proveniente de la hemolisis fisiologica, que aporta alrededor de 18 mg de Fe al dia, el que por otra parte no tiene otra forma de eliminarse del organismo. En los pacientes que requieren transfusion de concentrado eritrocitario el aporte de Fe se incrementa 3.46 mg/g de hemoglobina contenido en la transfusion o 250 mg/ unidad, de manera tal que al cumplirse la vida...
IntroductionDown syndrome is associated with various congenital anomalies and metabolic alteratio... more IntroductionDown syndrome is associated with various congenital anomalies and metabolic alterations such as hematological alterations. Values for the major hematological indicators vary with age and sex, but these values have not been described for Mexican children with Down syndrome.Objective. To describe the complete blood count (CBC) values of pediatric patients with Down syndrome and report the most common non-malignant hematological alterations. Materials and Methods.The analysis includes data from 450 patients with Down syndrome, 55.5% ware males, aged 0-18 years who were patients at the Mexican National Institute of Pediatrics and whose clinical charts included CBC panel results for the period January 2008 through March 2018. Results.A total of 3438 CBC panels were analyzed with descriptive statistics to find the values and statistical dispersion of the major indicators, with percentiles, and reported separately by sex and age group. The most common non-malignant hematologica...
Purpose: This is the first report to our knowledge of ischemic retinopathy in a pediatric patient... more Purpose: This is the first report to our knowledge of ischemic retinopathy in a pediatric patient with Upshaw-Schulman syndrome (USS). Methods: A 6-year-old girl previously diagnosed with USS was referred to our clinic with exodeviation of the left eye and a 2-month-long decrease in vision of both eyes. A dilated fundus examination showed a total vitreous hemorrhage in both eyes. The first course of action was conservative treatment, with the patient experiencing visual-acuity improvement in her right eye. Results: An ischemic retina and optic nerve atrophy was found once the left eye was cleared of the hemorrhage. Conclusions: We present a case of a vitreous hemorrhage, possibly secondary to an episode of severe thrombocytopenia. Following USS diagnosis, providers should perform dilated ophthalmologic examinations as part of initial and follow-up general evaluations. This case exemplifies that, in understudied and underdescribed pediatric retinal diseases, extreme therapeutic decis...
In Mexico, due to the high rates of diabetes, overweight, and obesity, there has also been noted ... more In Mexico, due to the high rates of diabetes, overweight, and obesity, there has also been noted an increased newborn weight, which may be contributing to the elevated incidence rate of childhood acute leukemia (AL). We conducted a case-control study in public hospitals of Mexico City aimed to know whether a greater weight at birth is associated with a higher risk of developing leukemia. We included incident cases with acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML) diagnosed between 2010 and 2015. Controls were frequency-matched to the cases by age, sex, and health institution. Logistic regression analysis was performed adjusting risks by child's sex, overcrowding index, birth order, and mother's age at the time of pregnancy. Adjusted odds ratios (aORs) and 95% confidence intervals were calculated. A total of 1455 cases and 1455 controls were included. An evident association between ALL and child's birthweight ≥2500 g was found (aOR 2.06; 95% CI: 1.5...
Occupational exposure of parents to carcinogens is of great interest in the etiology of leukemias... more Occupational exposure of parents to carcinogens is of great interest in the etiology of leukemias. Evidence of the impact of such exposure on infants or small children is scarce. Here we estimated whether occupational exposure of parents to carcinogens could be a risk factor for leukemias in their children. Cases of acute leukemia (AL) in infants ≤24 months old diagnosed in Mexico City (1998-2013) were included in a population-based, case-control study. Each of the 195 cases was matched with at least one healthy child (n = 369). For each of four exposure windows studied, the degree of exposure to carcinogens was determined for both parents by using a validated occupational exposure index. An unconditional logistic regression was carried out. Odds ratios (OR) and the 95% confidence intervals (CI) of the overall occupational exposure for parents during the four exposure windows indicated no association with risk of AL in their children. Pre-conception, the OR by the father 0.77 (0.49-...
Chronic granulocytic leukemia (CGL) is a rare hematologic disease in pediatric patients. It usual... more Chronic granulocytic leukemia (CGL) is a rare hematologic disease in pediatric patients. It usually presents with insidious symptoms. However, some cases may have an atypical presentation. We report herein the case of a 13-year-old female admitted to the emergency department with acute abdomen. She had hyperleukocytosis of 500.0 × 1000 cells/mm suggestive of CGL. A paracentesis was performed due to abdominal compartment syndrome that demonstrated hemoperitoneum. At laparotomy, a ruptured ovarian mass was found with multiple tumor implants in the serosal surface. Pathology revealed a CGL-infiltrated ovary. The patient is currently stable, has finished adjuvant chemotherapy, and is at 24 months of follow-up. To our knowledge, this is the first report of such a case.
Novel biomarkers for risk refinement and stratification in childhood acute lymphoblastic leukemia... more Novel biomarkers for risk refinement and stratification in childhood acute lymphoblastic leukemia (ALL) are needed to optimize treatment results. We studied the expression of CASP8AP2 and H2AFZ associated with relapse and survival in bone marrow samples from newly diagnosed children with ALL. We found: (a) an increased risk for early relapse in those patients with low expression of CASP8AP2 (odds ratio [OR] 3.93, 95% confidence interval [CI] 1.40-11.02, p < 0.05) confirming its usefulness as a predictive risk marker, although H2AFZ did not present the same effect; (b) patients with low expressions of CASP8AP2 and H2AFZ had inferior survival rates (p < 0.001); (c) the predictive values regarding low expressions of H2AFZ and CASP8AP2 and high white blood cell count suggest that these features could help to identify more accurately patients at greater risk of relapse.
El hierro (Fe) es un metal indispensable en el funcionamiento de diferentes sistemas enzimaticos ... more El hierro (Fe) es un metal indispensable en el funcionamiento de diferentes sistemas enzimaticos del organismo. En su mayoria se encuentra formando parte de la hemoglobina, en donde participa en el intercambio gaseoso en tejidos y pulmon; en otros sistemas enzimaticos celulares participa en diferentes mecanismos de oxido-reduccion. En condiciones normales se obtiene a partir de la dieta de donde se absorben aproximadamente 2 mg/dia, y la misma cantidad se pierde por la descamacion de epitelios, guardando un estrecho equilibrio. Esta cantidad es insuficiente para mantener la eritropoyesis, para la cual se utiliza el hierro proveniente de la hemolisis fisiologica, que aporta alrededor de 18 mg de Fe al dia, el que por otra parte no tiene otra forma de eliminarse del organismo. En los pacientes que requieren transfusion de concentrado eritrocitario el aporte de Fe se incrementa 3.46 mg/g de hemoglobina contenido en la transfusion o 250 mg/ unidad, de manera tal que al cumplirse la vida...
IntroductionDown syndrome is associated with various congenital anomalies and metabolic alteratio... more IntroductionDown syndrome is associated with various congenital anomalies and metabolic alterations such as hematological alterations. Values for the major hematological indicators vary with age and sex, but these values have not been described for Mexican children with Down syndrome.Objective. To describe the complete blood count (CBC) values of pediatric patients with Down syndrome and report the most common non-malignant hematological alterations. Materials and Methods.The analysis includes data from 450 patients with Down syndrome, 55.5% ware males, aged 0-18 years who were patients at the Mexican National Institute of Pediatrics and whose clinical charts included CBC panel results for the period January 2008 through March 2018. Results.A total of 3438 CBC panels were analyzed with descriptive statistics to find the values and statistical dispersion of the major indicators, with percentiles, and reported separately by sex and age group. The most common non-malignant hematologica...
Purpose: This is the first report to our knowledge of ischemic retinopathy in a pediatric patient... more Purpose: This is the first report to our knowledge of ischemic retinopathy in a pediatric patient with Upshaw-Schulman syndrome (USS). Methods: A 6-year-old girl previously diagnosed with USS was referred to our clinic with exodeviation of the left eye and a 2-month-long decrease in vision of both eyes. A dilated fundus examination showed a total vitreous hemorrhage in both eyes. The first course of action was conservative treatment, with the patient experiencing visual-acuity improvement in her right eye. Results: An ischemic retina and optic nerve atrophy was found once the left eye was cleared of the hemorrhage. Conclusions: We present a case of a vitreous hemorrhage, possibly secondary to an episode of severe thrombocytopenia. Following USS diagnosis, providers should perform dilated ophthalmologic examinations as part of initial and follow-up general evaluations. This case exemplifies that, in understudied and underdescribed pediatric retinal diseases, extreme therapeutic decis...
In Mexico, due to the high rates of diabetes, overweight, and obesity, there has also been noted ... more In Mexico, due to the high rates of diabetes, overweight, and obesity, there has also been noted an increased newborn weight, which may be contributing to the elevated incidence rate of childhood acute leukemia (AL). We conducted a case-control study in public hospitals of Mexico City aimed to know whether a greater weight at birth is associated with a higher risk of developing leukemia. We included incident cases with acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML) diagnosed between 2010 and 2015. Controls were frequency-matched to the cases by age, sex, and health institution. Logistic regression analysis was performed adjusting risks by child's sex, overcrowding index, birth order, and mother's age at the time of pregnancy. Adjusted odds ratios (aORs) and 95% confidence intervals were calculated. A total of 1455 cases and 1455 controls were included. An evident association between ALL and child's birthweight ≥2500 g was found (aOR 2.06; 95% CI: 1.5...
Occupational exposure of parents to carcinogens is of great interest in the etiology of leukemias... more Occupational exposure of parents to carcinogens is of great interest in the etiology of leukemias. Evidence of the impact of such exposure on infants or small children is scarce. Here we estimated whether occupational exposure of parents to carcinogens could be a risk factor for leukemias in their children. Cases of acute leukemia (AL) in infants ≤24 months old diagnosed in Mexico City (1998-2013) were included in a population-based, case-control study. Each of the 195 cases was matched with at least one healthy child (n = 369). For each of four exposure windows studied, the degree of exposure to carcinogens was determined for both parents by using a validated occupational exposure index. An unconditional logistic regression was carried out. Odds ratios (OR) and the 95% confidence intervals (CI) of the overall occupational exposure for parents during the four exposure windows indicated no association with risk of AL in their children. Pre-conception, the OR by the father 0.77 (0.49-...
Chronic granulocytic leukemia (CGL) is a rare hematologic disease in pediatric patients. It usual... more Chronic granulocytic leukemia (CGL) is a rare hematologic disease in pediatric patients. It usually presents with insidious symptoms. However, some cases may have an atypical presentation. We report herein the case of a 13-year-old female admitted to the emergency department with acute abdomen. She had hyperleukocytosis of 500.0 × 1000 cells/mm suggestive of CGL. A paracentesis was performed due to abdominal compartment syndrome that demonstrated hemoperitoneum. At laparotomy, a ruptured ovarian mass was found with multiple tumor implants in the serosal surface. Pathology revealed a CGL-infiltrated ovary. The patient is currently stable, has finished adjuvant chemotherapy, and is at 24 months of follow-up. To our knowledge, this is the first report of such a case.
Novel biomarkers for risk refinement and stratification in childhood acute lymphoblastic leukemia... more Novel biomarkers for risk refinement and stratification in childhood acute lymphoblastic leukemia (ALL) are needed to optimize treatment results. We studied the expression of CASP8AP2 and H2AFZ associated with relapse and survival in bone marrow samples from newly diagnosed children with ALL. We found: (a) an increased risk for early relapse in those patients with low expression of CASP8AP2 (odds ratio [OR] 3.93, 95% confidence interval [CI] 1.40-11.02, p < 0.05) confirming its usefulness as a predictive risk marker, although H2AFZ did not present the same effect; (b) patients with low expressions of CASP8AP2 and H2AFZ had inferior survival rates (p < 0.001); (c) the predictive values regarding low expressions of H2AFZ and CASP8AP2 and high white blood cell count suggest that these features could help to identify more accurately patients at greater risk of relapse.
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Papers by Norma López Santiago