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Making a Transcutaneus Icterometer in Neonates  [PDF]
Hamid Amoozgar,Maziar Rastegar
Iranian Journal of Pediatrics , 2007,
Abstract: Objective: Approximately 50% of newborn develop jaundice, however only 6% of them have significant hyperbilirubinemia that need more evaluation. Actually, most of sampling (50-90%) for detection of hyperbilirubinemia is not necessary and cause some problems such as infection, tissue injury, pain, discomfort, wasting time and money. So non-invasive methods is desirable to decrease unnecessary sampling.Material & Methods: This study performed on neonates who referred to Shiraz University of Medical Science affiliated Nemazee hospital neonatal emergency room due to jaundice. Initially 92 neonates were selected. After taking digital images and calculating mean and 95 percentile yellow wave's lengths of forehead skin and detection of total serum bilirubin, the correlation between wave's lengths and serum total bilirubin were determined. 95 percentile of skin yellow wave lengths that determined bilirubin level of 5, 10, 15 and 20 mg/dl were used for making 4 yellow strips of the icterometer. This icterometer was used for screening of 209 neonates who referred for evaluation of jaundice.Findings: Using icterometer in evaluation of 209 neonates with jaundice demonstrated sensitivity 94.7%, specificity 55.2%, positive predictive value of 85%, negative predictive value of 95% and accuracy 87%. Conclusion: This Icterometer can be applied as a screening tool in neonatal hyperbilirubinemia to detect less significant degree of hyperbilirubinemia that doesn't need sampling in Shiraz and even other regions of Iran with the same skin color.
A prospective study of the effect of delivery type on neonatal weight gain pattern in exclusively breastfed neonates born in Shiraz, Iran
Azadeh Saki, Mohammad R Eshraghian, Kazem Mohammad, Abbas Foroushani, Mohammad R Bordbar
International Breastfeeding Journal , 2010, DOI: 10.1186/1746-4358-5-1
Abstract: A cohort of 92 neonates born in Shiraz, from July 10 to August 10, 2007 was followed longitudinally. The data were collected during the first month postpartum at three occasions: 3 to 7 days postpartum, 10-21 days postpartum and 24-31 days postpartum.Among 92 mothers in this study, 35 (38%) were delivered by C-section. Generalized estimating equation (GEE) showed that delivery type (p < 0.01), receipt of advice about breastfeeding (p = 0.03) and neonate's age (p < 0.01) significantly affected weight gain. GEE estimated the values of the parameters under study and the testing contribution of each factor to weight gain, leading to the conclusion that gender, parities and maternal education did not contribute to weight gain. The neonate's weight gain pattern for C-section deliveries lies below that of normal vaginal deliveries until 25 days postpartum, when weight gain for C-section deliveries became higher than that for normal vaginal deliveries.Type of delivery contributes strongly to the weight gain pattern in the first month of infancy. In spite of greater weight loss among C-section birth neonates in the first days of life, at the end of the first month neonates showed a similar weight gain. Consequently, mothers with C-section delivery can successfully exclusively breastfeed.It is normal for newborns to lose weight during the first days of life. Although much of this weight loss is thought to be due to changes in the volume and distribution of water in the body, some studies show that early skin-to-skin contact, initiating breastfeeding as soon as possible, and feeding practices also influence the degree of weight loss [1-5]. Dehydration and/or failure to thrive during the first days postpartum may occur as a result of lactation failure and lack of awareness about feeding problems. Recent reports recommend monitoring infants' weight through the neonatal period [6-8].Extensive research on the biology of human milk and health outcomes associated with normal methods
Incidence of thrombocytopenia in hyperbilirubinemic neonates during phototherapy
Pishva N,Pishva H
Acta Medica Iranica , 2000,
Abstract: Thrombocytopenia has been reported as a complication of phototherapy. In this study the effect of conventional phototherapy on platelet count was studied in 101 newborns with indirect hyperbilirubinemia, out of whom 50 patients (49.5%) had decreased levels of platelets; 20 (19.8%) of the latter had a platelet count of below 100000/mm3. Decreased platelet count was maximum during the first 24 hours of phototherapy. Ultrviolet light may increase plateled turnover and injury during phototherapy by and unkown mechanism.
GENE THERAPY IN THALASSEMIA AND HEMOGLOBINOPATHIES  [cached]
Laura Breda,Roberto Gambari,Stefano Rivella
Mediterranean Journal of Hematology and Infectious Diseases , 2009, DOI: 10.4084/mjhid.2011.
Abstract: Sickle cell disease (SCD) and -thalassemia represent the most common hemoglobinopathies caused, respectively, by the alteration of structural features or deficient production of the -chain of the Hb molecule. Other hemoglobinopathies are characterized by different mutations in the α- or -globin genes and are associated with anemia and might require periodic or chronic blood transfusions. Therefore, -thalassemia, SCD and other hemoglobinopathies are excellent candidates for genetic approaches since they are monogenic disorders and, potentially, could be cured by introducing or correcting a single gene into the hematopoietic compartment or a single stem cell. Initial attempts at gene transfer of these hemoglobinopathies have proved unsuccessful due to limitations of available gene transfer vectors. With the advent of lentiviral vectors many of the initial limitations have been overcame. New approaches have also focused on targeting the specific mutation in the -globin genes, correcting the DNA sequence or manipulating the fate of RNA translation and splicing to restore -globin chain synthesis. These techniques have the potential to correct the defect into hematopoietic stem cells or be utilized to modify stem cells generated from patients affected by these disorders. This review discusses gene therapy strategies for the hemoglobinopathies, including the use of lentiviral vectors, generation of induced pluripotent stem cells (iPS) cells, gene targeting, splice-switching and stop codon readthrough.
Therapy of hemoglobinopathies: update
P. Cianciulli
Drugs and Cell Therapies in Hematology , 2012, DOI: 10.4081/dcth.2012.1s.32
Abstract: Every year, about 300,000 children are born affected by hemoglobinopathies including 30% with Thalassemia syndromes and 70% with sickle cell disease. 50,000 to 100,000 children die annually for Thalassemia. These numbers represent 0.5-0.9% of all deaths in low-income countries and constitute 3.4% of all deaths occurring under the age of 5 years. Thalassemia gene is more frequent than HbS gene and because HbS gene is particularly frequent in certain regions this means that the number of people born with SCD is higher of newborns with Thalassemia...
INFECTIONS IN THALASSEMIA AND HEMOGLOBINOPATHIES  [cached]
Bianca Maria Ricerca,Arturo Di Girolamo,Deborah Rund
Mediterranean Journal of Hematology and Infectious Diseases , 2009, DOI: 10.4084/mjhid.2011.
Abstract: The clinical approach to thalassemia and hemoglobinopathies, specifically Sickle Cell Disease (SCD), based on transfusions, iron chelation and bone marrow transplantation has ameliorated their prognosis. Nevertheless, infections still may cause serious complications in these patients. The susceptibility to infections in thalassemia and SCD arises both from a large spectrum of immunological abnormalities and from exposure to specific infectious agents. Four fundamental issues will be focused upon as central causes of immune dysfunction: the diseases themselves; iron overload, transfusion therapy and the role of the spleen. Thalassemia and SCD differ in their pathogenesis and clinical course. It will be outlined how these differences affect immune dysfunction, the risk of infections and the types of most frequent infections in each disease. Moreover, since transfusions are a fundamental tool for treating these patients, their safety is paramount in reducing the risks of infections. In recent years, careful surveillance worldwide and improvements in laboratory tests reduced greatly transfusion transmitted infections, but the problem is not completely resolved. Finally, selected topics will be discussed regarding Parvovirus B19 and transfusion transmitted infections as well as the prevention of infectious risk postsplenectomy or in presence of functional asplenia.
Incidence of hemoglobinopathies in Northwest Paraná, Brazil
Seixas, Flavio A. V.;Silva, Cíntia D.;Tominaga, Jane;Ferro, Octávia C.;Nilson, Luana G.;
Revista Brasileira de Hematologia e Hemoterapia , 2008, DOI: 10.1590/S1516-84842008000400011
Abstract: immigrants from many parts of the world settled in paraná state in brazil, contributing to the diversified genetic patrimony of its population. this characteristic led us to investigate, for the first time, the incidence of hemoglobinopathies in the population of one city in paraná. a total of 585 blood samples were collected from individuals living in umuarama. hemoglobinopathy tests were carried out using the classical methodology. the results show that 93.17% have the normal electrophoretic pattern (aa); 2.73% have the beta-thalassemia trait; 2.05% have the sickle cell trait (as); 1.37% are heterozygous for alpha-thalassemia; 0.34% heterozygous for hemoglobin c (ac); 0.17% have both alpha-thalassemia and sickle cell traits and 0.17% are heterozygous for alpha and beta-thalassemia. a comparison of these results with other works suggests that the frequency of hemoglobinopathies can significantly vary between cities within the same state. this fact may be attributed to the miscegenation of the population or even to the diverse prevalence of hemoglobinopathies in distinct populations.
Inhibitory Effects of Palm Tocotrienol-Rich Fraction Supplementation on Bilirubin-Metabolizing Enzymes in Hyperbilirubinemic Adult Rats  [PDF]
Yusof Kamisah, Jing Jye Lim, Chew-Lian Lim, Ahmad Y. Asmadi
PLOS ONE , 2014, DOI: 10.1371/journal.pone.0089248
Abstract: Background Phenylhydrazine, a hemolytic agent, is widely used as a model of experimental hyperbilirubinemia. Palm tocotrienol-rich fraction (TRF) was shown to exert beneficial effects in hyperbilirubinemic rat neonates. Aim To investigate the effects of palm TRF supplementation on hepatic bilirubin-metabolizing enzymes and ocidative stress status in rats administered phenylhydrazine. Methods Twenty-four male Wistar rats were divided into two groups; one group was intraperitoneally injected with palm TRF at the dose of 30 mg/kg/day, while another group was only given vehicle (control) (vitamin E-free palm oil) for 14 days. Twenty-four hours after the last dose, each group was further subdivided into another two groups. One group was administered phenylhydrazine (100 mg/kg, intraperitoneally) and another group was administered normal saline. Twenty-four hours later, blood and liver were collected for biochemical parameter measurements. Results Phenylhydrazine increased plasma total bilirubin level and oxidative stress in the erythrocytes as well as in the liver, which were reduced by the pretreatment of palm TRF. Palm TRF also prevented the increases in hepatic heme oxygenase, biliverdin reductase and UDP-glucuronyltransferase activities induced by phenylhydrazine. Conclusion Palm tocotrienol-rich fraction was able to afford protection against phenylhydrazine-induced hyperbilirubinemia, possibly by reducing oxidative stress and inhibiting bilirubin-metabolizing enzymes in the liver.
Assesment of Etiology of Hyperbilirubinemic Newborn Had Exchange Transfusion  [PDF]
Selahattin Katar,Celal Devecio?lu,A. Kadir ?zel,?clal Sucakl?
Dicle Medical Journal , 2006,
Abstract: Neonatal hyperbilirubinemia is a common newborn problem and may cause important mortality and morbidity when early recognition and appropriate management was not made. In this study we aimed to asses the etiology of hyperbilirubinemia in 56 neonates who had exchange transfusion. Of patients 64% were male and 36% were female. Mean age of admission was 6.4 2.5 days and mean total bilirubin level was 38 11,9 mg/dl. The cause of hyperbilirubinemia was ABO incompatibility in 34%, Rh incompatibility in 21.4%, glucose-6-phospatase deficiency in 9%, intrauterine growth restriction in 5%, sepsis in 4%, hypothyroidism in 3.6%, minor blood group incompatibility in 1.8%, cephal hematoma in 1.8%, diabetic mother’s child in 1.8% of patients. Exchange transfusion was made once in 86% and twice in 14% of patients.
Evaluation of Cytomegalovirus Infection after Six Months of Liver Transplantation in Children in Shiraz, Southern Iran
N. Honar,M. H. Imanieh,S. M. Dehghani,M. Haghighat
International Journal of Organ Transplantation Medicine , 2011,
Abstract: Background: Liver transplantation (LT) is a life-saving treatment for end-stage liver diseases (ESLD). Cytomegalovirus(CMV) infection is one of the important causes of morbidity after LT.Objective: To evaluate the incidence of late-onset (after 6 months of LT) CMV infection in pediatric recipients.Methods: A retrospective analysis was conducted to evaluate 50 pediatric patients who underwent LT for8 years at the LT Unit of Nemazee Hospital affiliated to Shiraz University of Medical Sciences, Shiraz, Iran.We retrospectively investigated episodes of CMV infection after 6 months of LT proven by CMV antigenemiatest.Results: Three recipients (6%) developed late-onset CMV infection. These patients finally responded toganciclovir.Conclusion: CMV infection is one of the most common post-LT viral infections that usually occurs in the firstsix months of LT. Our study shows that the incidence of late-onset CMV infection is relatively low, but itstill remains a significant problem. Therefore, monitoring and management is crucial for improving thesurvival of children.
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