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Non-Hodgkin lymphoma in childhood and adolescence: frequency and distribution of immunomorphological types from a tertiary care center in South India
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Marie Therese Manipadam, Sheila Nair, Auro Viswabandya, Leni Mathew, Alok Srivastava, Mammen Chandy |
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Author Affiliations: Department of Pathology, Christian Medical College, Vellore, India (Manipadam MT, Nair S); Department of Hematology, Christian Medical College, Vellore, India (Viswabandya A, Srivastava A, Chandy M); Department of Child Health, Christian Medical College, Vellore, India (Mathew L)
Corresponding Author: Marie Therese Manipadam, Associate Professor, Department of Pathology, Christian Medical College, Vellore- 632004, Tamil Nadu, India (Tel: 91-416-2282005; Fax: 91-416-2232035; Email: mtm2005@cmcvellore.ac.in)
doi: 10.1007/s12519-011-0303-7
Background: There is a dearth of published literature on the frequency and distribution of pediatric and adolescent non-Hodgkin lymphoma (NHL) in India according to the 2001 WHO classification. The aim of this study was to record the distribution of the different subtypes, analyze the major subtypes, and compare it with the published data from other countries. A review of pediatric NHL statistics from population-based cancer registries was included in this study.
Methods: The study was conducted using information retrieved from the files of our institution. A total of 467 patients with lymphoma (excluding mycosis fungoides) were recorded in the under 20 years group over a period of 6 years, of which 252 patients suffered from NHL. The demographic characteristics, frequency and distribution of different subtypes were noted and compared with published reports from other parts of the world.
Results: T-lymphoblastic lymphoma/leukemia constituted the majority (32.1%) of all NHLs in children and adolescents in our study. The other major subgroups were Burkitt's lymphoma, anaplastic large cell lymphoma, and diffuse large B cell lymphoma. Burkitt's lymphoma in this study had clinical presentations similar to those seen in western countries.
Conclusions: The distribution of different subtypes of lymphoma in pediatric and adolescent NHL in India differs considerably from that in western countries and other eastern countries.
Key words: adolescent; anaplastic large cell lymphoma; Burkitt's lymphoma; children; non-Hodgkin lymphoma
World J Pediatr 2011;7(4):318-325 |
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[Abstract] [Full Text] [PDF]
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Glucosidase acid beta gene mutations in Egyptian children with Gaucher disease and relation to disease phenotypes
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Zakarya El-Morsy, Mohamed T Khashaba, Othman El-Sayed Soliman, Sohier Yahia, |
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Mansoura, Egypt
Author Affiliations: Department of Pediatrics, Faculty of Medicine, Mansoura University, Egypt (El-Morsy Z, Khashaba MT, Soliman OE, Yahia S, Abd El-Hady D)
Corresponding Author: Othman EL-Sayed Soliman, Mansoura University Children's Hospital El-Gomhoria St. Postal Code: 35516, Mansoura, Egypt (Tel: +20115691116; Fax: 002 050 2234092; Email: oesoliman65@yahoo.com)
Introduction: More than 200 mutations have been found in patients with Gaucher disease (GD) and some mutations usually have a high frequency in certain populations. Genotype/phenotype correlation in patients with GD has not been established. This study was designed to determine underlying mutations in Egyptian children with GD and to assess their relation to disease phenotypes.
Methods: This study comprised 17 patients with GD and 10 healthy controls. Thirteen patients were type 1 GD, 2 type 2, and 2 type 3. DNA was extracted from peripheral blood leukocytes. Exons 9 and 10 were amplified by polymerase chain reaction, and deoxyribonucleic acid sequencing was done with an ABI 310 genetic analyzer.
Results: Wild type allele was detected in 95% (19/20) and a normal variant in 5% (1/20) of controls. L444P allele was encountered in 50% (13/26) of the alleles in type 1 patients, H451P in 7.7% (2/26) and recombinant alleles (RecNcil, RecNcil + M450L, RecFs, RecFs + M450L) in 34.6% (9/26). L444P and Rec alleles each occurred in 50% (2/4) of type 2 and 3 patients. A new mutation was seen in this study {g.7336A>C, (M450L)} and 2 mutant alleles were not determined. Type 1 GD patients had L444P/L444P genotype (23.1%) and Rec alleles/L444P (53.8%), while type 2 and 3 GD patients had Rec alleles/L444P genotypes (100%) with a poor phenotype/genotype correlation.
Conclusions: L444P and Rec alleles are common in the studied patients. Novel mutations are continuously detected, adding to the expanding panel of GD mutations. No significant genotype-phenotype association was observed.
Key words: children; Gaucher disease; glucosidase acid beta mutation; phenotype
World J Pediatr 2011;7(4):326-330 |
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[Abstract] [Full Text] [PDF]
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Twelve-year prevalence of common neonatal congenital malformations in Zhejiang Province, China
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Ge Sun, Zhe-Ming Xu, Jian-Feng Liang, Lin Li, Da-Xing Tang |
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Author Affiliations: The Neonatal Intensive Care Unit, Women's Hospital, Zhejiang University School of Medicine, Hangzhou, China (Sun G); Department of Urology, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China (Xu ZM, Tang DX); Department of Information, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China (Liang JF); Department of Women's Health Care, Women's Hospital, Zhejiang University School of Medicine, Hangzhou, China (Li L)
Corresponding Author: Ge Sun, The Neonatal Intensive Care Unit, Women's Hospital, Zhejiang University School of Medicine, Hangzhou 310006, China (Tel: +86 571 87061501 ext 2033; Fax: +86 571 87061878; Email: sunge051266@sina.com)
doi: 10.1007/s12519-011-0328-y
Background: One of the challenges that pediatricians face when examining birth defects is to understand the trends in its occurrence and provide clues to etiology. This study was undertaken to retrospectively assess the prevalence of 10 common neonatal congenital malformations by reviewing a database of all deliveries from 28 weeks up until 7 days of birth from January 1998 to December 2009 in Zhejiang Province, China.
Methods: Ten common neonatal congenital malformations were selected for analysis. The incidence and the Cochran-Armitage Trend were assessed via SAS9.2. A P value ¡Ü0.05 was considered statistically significant.
Results: Of 83 888 perinatals, 374 (4.46¡ë) suffered from congenital heart diseases (CHD), 77 (0.92¡ë) from congenital hydrocephalus, 32 (0.38¡ë) from intestinal atresia/stenosis, 36 (0.43¡ë) from anorectal malformations, 149 (1.78¡ë) from kidney malformations, 139 (3.31¡ë) from hypospadias (male), 178 (2.12¡ë) from orofacial clefts (OFC), 188 (2.24¡ë) from polydactyly, 62 (0.74¡ë) from syndactyly, and 269 (3.21¡ë) from accessory auricle anomaly. Their trend of prevalence varied as follows: CHD, P=0.0026; hydrocephalus, P=0.0042; intestinal atresia/stenosis, P=0.0103; anorectal malformations, P=0.4332; kidney malformations, P<0.0001; hypospadias, P=0.0021; OFC, P=0.005; polydactyly, P=0.0867; syndactyly, P=0.1941; and accessory auricle anomaly, P=0.0011. The upward trend was as follows: CHD (P=0.0026), intestinal atresia/stenosis (P=0.0103), kidney malformations (P<0.0001), hypospadias (P=0.0021), OFC (P=0.005), and accessory auricle anomaly (P=0.0011). The incidence of hydrocephalus (P=0.0042) showed a downward trend. No statistical significant trend was found in anorectal malformations (P=0.4332), polydactyly (P=0.0867) and syndactyly (P=0.1941).
Conclusions: The incidences of CHD, intestinal atresia/stenosis, kidney malformations, hypospadias, OFC, and accessory auricle anomaly have increased in the last 12 years, but the incidences of anorectal malformations, polydactyly and syndactyly remain stable. The incidence of hydrocephalus shows a downward trend.
Key words: congenital malformation; incidence; neonate
World J Pediatr 2011;7(4):331-336 |
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[Abstract] [Full Text] [PDF]
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Swallowing dysfunction in very low birth weight infants with oral feeding desaturation
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Jang Hoon Lee, Yun Sil Chang, Hye Soo Yoo, So Yoon Ahn, Hyun Joo Seo, Seo Hui Choi,Ga Won Jeon, Soo Hyun Koo, Jong Hee Hwang, Won Soon Park |
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Author Affiliations: Department of Pediatrics, Ajou University School of Medicine, Suwon, Korea (Lee JH); Department of Pediatrics, College of Medicine, Inje University, Busan Paik Hospital, Busan, Korea (Jeon GW); Department of Pediatrics, College of Medicine, Inje University, Haeundae Paik Hospital, Busan, Korea (Koo SH); Department of Pediatrics, Ilsan Paik Hospital, College of Medicine, Inje University, Ilsan, Korea (Hwang JH); Department of Pediatrics, Sungkyunkwan University School of Medicine, Samsung Medical Center, Seoul, Korea (Chang YS, Yoo HS, Ahn SY, Seo HJ, Choi SH, Park WS)
Corresponding Author: Won Soon Park, MD, Department of Pediatrics, Samsung Medical Center, 50 Irwon-dong, Kangnam-gu, Seoul 135-710, Korea (Tel: 82-2-3410-3523; Fax: 82-2-3410-0043; Email: wonspark@skku.edu)
doi: 10.1007/s12519-011-0281-9
Background: We detected swallowing dysfunction by the modified barium swallow (MBS) test and determined risk factors for swallowing dysfunction in very low birth weight (VLBW) infants with oral feeding desaturation near discharge.
Methods: We retrospectively reviewed 41 VLBW infants referred for MBS test because of significant oral feeding desaturation at ¡Ý35 weeks of postmenstrual age. Infants who showed impaired airway protection, including inadequate epiglottic closure, laryngeal penetration and/or tracheal aspiration by MBS test, were compared to those without impaired airway protection.
Results: Eleven infants (26.8%) showed impaired airway protection by MBS test. They had a significantly lower gestational age at birth but a similar postmenstrual age compared to those without impaired airway protection. All infants with impaired airway protection were born at ¡Ü28 weeks of gestation.
Conclusions: Swallowing dysfunction resulting in aspiration should be considered as a cause of significant oral feeding desaturation in infants born at ¡Ü28 weeks of gestation regardless of postmenstrual age.
Key words: aspiration; modified barium swallow; penetration; prematurity
World J Pediatr 2011;7(4):337-343 |
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[Abstract] [Full Text] [PDF]
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Association between asthma and dental caries in the primary dentition of Mexican children
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Eliza M V¨¢zquez, Francisco V¨¢zquez, Mar¨ªa C Barrientos, Jos¨¦ A C¨®rdova, Dolores Lin, Francisco J Beltr¨¢n, Carlos F V¨¢zquez |
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Author Affiliations: Autonomous University of Tamaulipas (UAT), University Center Tampico-Madero, Tamaulipas, M¨¦xico (V¨¢zquez EM, Barrientos MC, C¨®rdova JA, Lin D, Beltr¨¢n FJ); Mexican Institute of Social Security (IMSS), Madero city, Tamaulipas, M¨¦xico (V¨¢zquez F); Public Health Institute, Veracruzana University, M¨¦xico (V¨¢zquez CF)
Corresponding Author: Eliza M. V¨¢zquez, MD, Faculty of Medicine of Tampico. Autonomous University of Tamaulipas (UAT), 89339 University Center Tampico-Madero, Tamaulipas, Mexico (Tel: (52) (833) 216-5247; Email: dra.eliza.vazquez@gmail.com)
doi: 10.1007/s12519-011-0300-x
Background: Asthma has been associated with a great number of negative health outcomes. This study was undertaken to detect the association between asthma and dental caries in the primary dentition.
Methods: Data were obtained from a cohort of 1160 Mexican children aged 4-5 years. We used the questionnaire of the International Study of Asthma and Allergies Diseases in Childhood. Asthma was classified according to parents' reports. Caries indexes were measured as the number of decayed (d), missing (m), and filled (f), teeth (t) (dmft) or surfaces (dmfs). Decayed teeth included initial caries in this study. Adjusted odds ratios (adjusted ORs) were determined for asthma using logistic regression model. Gender, sugary products consumption, and oral hygiene habits were utilized as covariates.
Results: The prevalence of dental caries was 17.9% in the 1160 children. Approximately 226 (19.5%) children were identified with asthma. Among them, 166 (73.5%) presented with symptoms during the day and 60 (26.5%) during the day and night. The prevalence of caries in children with asthma was 19.9%; it was higher in children with nocturnal asthma symptoms than in those with asthma symptoms only during the day. Logistic regression model showed that asthma (adjusted OR=1.24; 95% confidence interval [95% CI]=0.84¨C1.81) was not associated with caries. A significant association was found between nocturnal asthma symptoms (adjusted OR=1.85; 95% CI=1.00¨C3.44) and dental caries.
Conclusions: Asthma is not associated with dental caries. Nocturnal asthma symptoms appear to be associated with dental caries in the primary dentition.
Key words: asthma; dental caries; tooth deciduous
World J Pediatr 2011;7(4):344-349 |
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[Abstract] [Full Text] [PDF]
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Diagnosis and treatment of subclinical hypothyroidism detected by neonatal screening
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Xiao-Xiao Chen, Yu-Feng Qin, Xue-Lian Zhou, Ru-Lai Yang, Yu-Hua Shi, Hua-Qing Mao, Yi-Ping Qu, Xu Wang, Zheng-Yan Zhao |
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Author Affiliations: Department of Genetics and Metabolism (Chen XX, Zhou XL, Yang RL, Shi YH, Mao HQ, Qu YP, Wang X); Department of Preventive Medicine and Child Health Care, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China (Qin YF, Zhao ZY)
Corresponding Author: Zheng-Yan Zhao, Department of Preventive Medicine and Child Health Care, Children's Hospital, Zhejiang University School of Medicine, 57 Zhu Gan Xiang, Hangzhou, 310003, China (Tel: +86-571-87061007-2435; Fax: +86-571-87033296; Email: zhaozy@zju.edu.cn)
doi: 10.1007/s12519-011-0314-4
Background: This study was undertaken to explore the clinical outcome and prognosis of subclinical hypo-thyroidism detected by newborn screening.
Methods: Newborn screening was conducted at 1156 health care institutions in Zhejiang Province from October 1999 to September 2006. Included were (1) infants who had thyroid-stimulating hormone (TSH) ¡Ý20 mU/L, and normal or lower normal levels of triiodothyronine (T3) and thyroxine (T4) and (2) infants with TSH between 5.6 mU/L and 20 mU/L at a confirmatory examination and follow-up showing TSH levels ¡Ý20 mU/L or delayed reduction in T4 levels. These infants were considered as having subclinical hypothyroidism and levothyroxine (L-T4) at an initial dose of 3-5 ¦Ìg/kg per day was administered. The levels of TSH and T4, developmental quotient (DQ), and index of growth were evaluated.
Results: A total of 204 infants met our criteria for subclinical hypothyroidism, with an incidence of 1/8809. After 2-4 weeks of standard therapy, serum TSH level dropped to normal and T4 reached a higher normal level in all the 204 infants. Evaluations of 60 patients after 2 years of therapy showed that their average DQ was 101¡À14.61, and body weight and height were within the normal ranges. Bone age test for 54 patients revealed normal development in 44, slightly retarded development in 7, and advanced development in 3.
Conclusions: Newborns with high TSH levels should be given particular attention to ensure early diagnosis. A L-T4 dose of 3-5 ¦Ìg/kg per day was effective in the initial treatment of subclinical hypothyroidism.
Key words: congenital hypothyroidism; diagnosis; L-thyroxine; newborn screening
World J Pediatr 2011;7(4):350-354 |
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[Abstract] [Full Text] [PDF]
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