Journal of Pediatric Biochemistry - Volume 2, issue 1

Authors: McClory, Susan | Yu, Jianhua

Article Type: Other

DOI: 10.3233/JPB-2011-0042

Citation: Journal of Pediatric Biochemistry, vol. 2, no. 1, pp. 1-2, 2012

Authors: Stafstrom, Carl E.

Article Type: Research Article

Abstract: Iron plays a critical role in brain development and physiology, cytoplasmic protein function, and mitochondrial reactions. Brain iron levels are regulated tightly, and pathologies can result from both iron deficiency (learning and memory deficits, neuronal and dendritic developmental alterations, impaired myelin function, and abnormal neurotransmitter regulation) and iron overload (free radical production and oxidative stress, as proposed for Parkinson and Alzheimer diseases as well as rare genetic disorders of iron accumulation). This …review briefly summarizes the roles of iron in normal and abnormal brain function, with emphasis on the developing brain, and describes some disorders deriving from deficient or excessive iron levels. Show more

Keywords: Iron, brain, iron deficiency, transferrin, ferritin, aceruloplasminemia

DOI: 10.3233/JPB-2011-0035

Citation: Journal of Pediatric Biochemistry, vol. 2, no. 1, pp. 3-14, 2012

Authors: Zand, Hamid

Article Type: Research Article

Abstract: Acute lymphoblastic leukemia (ALL) is a cancer of white blood cells. ALL occurs in people of all ages but most commonly in children. Improvement in the survival of children suffering from ALL over past 35 years is one of the great success stories of cancer treatment. However, increased survival of ALL patients stimulates the need for new therapeutic protocols in order to diminish the late side effects of ALL-therapy in children. Certain dietary compounds may increase …cancer cells' sensitivity to drugs and radiation, and decrease the adverse effects of therapeutic protocols. Flavonoids are polyphenolic compounds which are categorized according to their chemical structure as flavonols, flavones, flavanones, isoflavones, catechins, anthocyanidins and chalcones. Numerous polyphenolic compounds showed antiproliferative and cytotoxic effects and more specifically proapoptotic activities in several cancer cells lines and in animal tumor models. This review covers recent literature and summarizes the chemopreventive potential of flavonoids and molecular mechanisms of apoptosis induced by flavonoids in lymphoblastic leukemia. Several types of flavonoids, either by themselves or in combination with chemotherapeutic agents, may be effective in ALL prevention and treatment. Flavonoids recruit several proapoptotic signaling routes and inhibit antiapoptotic pathways in order to exert their chemopreventive properties. Show more

Keywords: Acute lymphoblastic leukemia, polyphenolic compounds, flavonoids, apoptosis

DOI: 10.3233/JPB-2011-0036

Citation: Journal of Pediatric Biochemistry, vol. 2, no. 1, pp. 15-21, 2012

Authors: Pater, Agnieszka | Nowacki, Wieslaw

Article Type: Research Article

Abstract: Bone is a dynamic tissue that consistently undergoes remodeling. During formation and resorption processes, bone turnover markers are released. They are specific bone-derived molecules that circulate in the blood or are present in the urine reflecting the bone metabolic activity (during childhood and adolescence: bone growth in length, modeling and remodeling). The use of biochemical bone turnover markers provides dynamic indices of bone turnover and complements the static measures of bone, such as measurement of bone …mineral density using DEXA or measurement of bone geometry, mass and density using quantitative computed tomography. The assay of bone markers may be repeated at much shorter intervals than remaining measurements so may help in detecting influence of disease or effects of therapy much earlier than changes in bone mass or progression in bone disease can be ascertained. The usefulness of determination of bone turnover markers in children and adolescents requires that the influence of age, sex and pubertal stage be taken into consideration to interpret the results correctly. Bone turnover markers are used in children and adolescents to assess bone metabolic disorders, monitoring disease progression and antiresorptive therapy. Show more

Keywords: Bone turnover markers, bone formation markers, bone resorption markers, osteoclastogenesis markers

DOI: 10.3233/JPB-2011-0038

Citation: Journal of Pediatric Biochemistry, vol. 2, no. 1, pp. 23-32, 2012

Authors: Lakshmi, Srinivasan | Ganesan, Nalini

Article Type: Research Article

Abstract: Childhood obesity is a major health epidemic caused by both genetic and environmental factors. Evidence suggests a strong role of genetic factors in the predisposition to obesity. Several monogenic forms of obesity have also been reported. Mutations in the genes involved in the leptin-melanocortin pathway have been found to cause these forms of obesity mainly by increasing the energy intake. Genes involved in the leptin-melanocortin pathway play a crucial role in human energy homeostasis. In this …review we discuss the role of the leptin-melanocortin pathway in the regulation of energy balance with special reference to the association between the genetic variants in the genes involved in the pathway and childhood obesity. Show more

Keywords: Leptin, melanocortin, childhood obesity, single nucleotide polymorphisms

DOI: 10.3233/JPB-2011-0039

Citation: Journal of Pediatric Biochemistry, vol. 2, no. 1, pp. 33-42, 2012

Authors: Almeida, Liziane C. | Costa-Carvalho, Beatriz T. | Viana, Patrícia O. | Salomao, Reinaldo | Granato, Celso | de Moraes-Pinto, Maria Isabel

Article Type: Research Article

Abstract: Diagnosis of primary immunodeficiency diseases (PID), based on laboratory tests and assessment of T lymphocyte function, is crucial in patients who present with lymphopenia. We evaluated T lymphocyte function in healthy children and adults and in patients and with PID using flow cytometry. Whole blood cultures were stimulated with phytohemagglutinin, purified protein derivate (PPD) and candidin, followed by detection of intracellular interferon-gamma (IFN - gamma) and CD25 membrane expression on CD3+ T …cells by flow cytometry. Flow cytometry results were compared with 3H-thymidine (3HTdR) lymproliferation after in vitro cell stimulation and with delayed type hypersensitivity reaction (DTH). Patients with PID had lower intracellular IFN - gamma production than healthy children and healthy adults after PHA stimulation for 18 h (p=0.024 and p<0.0001, respectively); CD25 expression was also lower in patients with PID than in healthy children and adults after candidin stimulation (p=0.048 and p< 0.0001, respectively). CD25 expression after PPD and candidin stimulation were also higher in healthy adults when compared with both patients and with healthy children (p< 0.0001 for all comparisons). Lymphoproliferation assay with 3HTdR after candidin stimulation did not show any significant difference between healthy children and patients with PID, but the response was higher in healthy adults (p=0.029). The DTH for PPD was not different between PID and healthy children (p=0.281). Intracellular IFN-gamma after PHA stimulation for 18 h and CD25 membrane expression after candidin stimulation for 72 h on CD3+ T cells were most reliable parameters that could discriminate PID patients from healthy children. Our results confirm the high variability in functional cell assays and reinforce the idea that age differences must be taken into consideration during assay evaluation. Show more

Keywords: Cellular immunity, primary immunodeficiency disease, flow cytometry, interferon-gamma, lymphoproliferation assay, delayed type hypersensitivity reaction, candidin, phytohemagglutinin

DOI: 10.3233/JPB-2011-0037

Citation: Journal of Pediatric Biochemistry, vol. 2, no. 1, pp. 43-52, 2012

Authors: Allegaert, Karel

Article Type: Research Article

Abstract: Retinopathy of prematurity (ROP) is a multifactorial disease with risk factors related to immaturity (gestational age, birth weight) and disease severity. We aimed to validate the use of peak creatinaemia as risk factor for prethreshold ROP in a recently treated cohort following its association in two historical cohort studies. Data on clinical characteristics and peak creatinine were collected in ELBW (extreme low birth weight, < 1000 g) infants admitted between 2007–2011. Data in cases who developed …prethreshold ROP were compared to controls (Mann Whitney U, Chi square) and significant risk factors were entered in a logistic regression model. Based on 123 patients of whom 24 developed threshold ROP, gestational age, birth weight and indicators of respiratory disease were significant risk factors, while peak creatinine was not longer a significant risk factor for prethreshold ROP. Immaturity became the dominant risk factor. Creatinine is no longer a biomarker to develop prethreshold ROP in our unit, likely due to the overall decreased incidence of ROP in our unit. This illustrates the need to validate biomarkers (e.g. creatinemia) in consecutive cohorts within the same unit and in other units before such biomarkers can be considered for secondary ROP prevention strategies. Show more

Keywords: Retinopathy of prematurity, creatinine, biomarker, risk factors, ELBW infants

DOI: 10.3233/JPB-2011-0040

Citation: Journal of Pediatric Biochemistry, vol. 2, no. 1, pp. 53-56, 2012

Authors: Youssef, Doaa M. | Noseer, Ateef I. | Abdallah, Ahmed M. | Aboelmagd, Yosry E.

Article Type: Research Article

Abstract: Trace elements may be modified by dialysis procedures. To prevent complications in chronic hemodialysis patients, it is important to understand and regulate the levels of trace elements. The aim of this work is to evaluate the serum zinc (Zn) and copper (Cu) levels in children with chronic kidney disease. This case control study has 3 groups: 20 children with chronic kidney disease (CKD) stage 5 on regular hemodialysis as group A, 20 children with CKD stage …3 and 4 on conservative treatment as group B children, and 10 healthy children as control group C. Measurements of serum copper and zinc were performed by atomic absorption spectrophotometry. We found that serum Zn was (74.4 ± 12.6 μg/dL) in group A, (76.7 ± 13.5 μg/dL) in group B with no significant difference between both groups (p> 0.05) but they are both significantly lower than group C in which serum Zn was (97.7 ± 14.3 μg/dL) (p< 0.05). As regard Cu level it was (89.3 ± 15.1 μg/dL) in group A, (95.6 ± 16.2 μg/dL) in group B, while it was (116.6 ± 13.6 μg/dL) in group C which is significantly higher than in children with CKD (p< 0.05). In studying the correlation between Zn, Cu with other parameters either (demographic or laboratory) in our patients we found a significant negative correlation with urea, creatinine and a positive correlation with serum iron level. Serum Zn and Cu levels are significantly decreased in patients with CKD when compared with healthy children. Hence we recommend supplementation of Zn and Cu for patients on regular hemodialysis specially those with iron deficiency. Show more

Keywords: Zinc, copper, chronic kidney disease, hemodialysis, children

DOI: 10.3233/JPB-2011-0041

Citation: Journal of Pediatric Biochemistry, vol. 2, no. 1, pp. 57-60, 2012