Displaying publications 841 - 860 of 2816 in total

Abstract:
Sort:
  1. Sreeramareddy CT, Sathyanarayana TN, Kumar HN
    PLoS One, 2012;7(12):e51904.
    PMID: 23284810 DOI: 10.1371/journal.pone.0051904
    BACKGROUND: Information about utilization of health services and associated factors are useful for improving service delivery to achieve universal health coverage.
    METHODS: Data on a sample of ever-married women from India Demographic and Health survey 2005-06 was used. Mothers of children aged 0-59 months were asked about child's illnesses and type of health facilities where treatment was given during 15 days prior to the survey date. Type of health facilities were grouped as informal provider, public provider and private provider. Factors associated with utilization of health services for diarrhea and fever/cough was assessed according to Andersen's health behavior model. Multinomial logistic regression analyses were done considering sampling weights for complex sampling design.
    RESULTS: A total of 48,679 of ever-married women reported that 9.1% 14.8% and 17.67% of their children had diarrhea, fever and cough respectively. Nearly one-third of the children with diarrhea and fever/cough did not receive any treatment. Two-thirds of children who received treatment were from private health care providers (HCPs). Among predisposing factors, children aged 1-2 years and those born at health facility (public/private) were more likely to be taken to any type of HCP during illness. Among enabling factors, as compared to poorer household, wealthier households were 2.5 times more likely to choose private HCPs for any illness. Children in rural areas were likely to be taken to any type of HCP for diarrhea but rural children were less likely to utilize private HCP for fever/cough. 'Need' factors i.e. children having severe symptoms were 2-3 times more likely to be taken to any type of HCP.
    CONCLUSION: Private HCPs were preferred for treatment of childhood illnesses. Involvement of private HCPs may be considered while planning child health programs. Health insurance scheme for childhood illnesses may to protect economically weaker sections from out-of-pocket health expenditure during child illness.
    Matched MeSH terms: Child, Preschool
  2. Batra YK, Ivanova M, Ali SS, Shamsah M, Al Qattan AR, Belani KG
    Paediatr Anaesth, 2005 Dec;15(12):1094-7.
    PMID: 16324030 DOI: 10.1111/j.1460-9592.2005.01633.x
    BACKGROUND: Laryngospasm is a well-known problem typically occurring immediately following tracheal extubation. Propofol is known to inhibit airway reflexes. In this study, we sought to assess whether the empiric use of a subhypnotic dose of propofol prior to emergence will decrease the occurrence of laryngospasm following extubation in children.
    METHODS: After approval from the Institutional Ethics Committee and informed parental consent, we enrolled 120 children ASA physical status I and II, aged 3-14 years who were scheduled to undergo elective tonsillectomy with or without adenoidectomy under standard general anesthesia. Before extubation, the patients were randomized and received in a blinded fashion either propofol 0.5 mg.kg(-1) or saline (control) intravenously. Tracheal extubation was performed 60 s after administration of study drug, when the child was breathing regularly and reacting to the tracheal tube.
    RESULTS: Laryngospasm was seen in 20% (n = 12) of the 60 children in the control group and in only 6.6% (n = 4) of 60 children in the propofol group (P < 0.05).
    CONCLUSIONS: During emergence from inhalational anesthesia, propofol in a subhypnotic dose (0.5 mg.kg(-1)) decreases the likelihood of laryngospasm upon tracheal extubation in children undergoing tonsillectomy with or without adenoidectomy.
    Matched MeSH terms: Child, Preschool
  3. Arshad AR
    Br J Plast Surg, 2005 Jun;58(4):481-6.
    PMID: 15897031
    Hypospadias is a congenital deformity characterised by an abnormally located urethral opening, that could occur anywhere proximal to its normal location on the ventral surface of glans penis to the perineum. Many operations had been described for the management of this deformity.
    Matched MeSH terms: Child, Preschool
  4. Alwi M, Kandavello G, Choo KK, Aziz BA, Samion H, Latiff HA
    Cardiol Young, 2005 Apr;15(2):141-7.
    PMID: 15845156
    Some patients with pulmonary atresia with an intact ventricular septum, mild to moderate right ventricular hypoplasia, and a patent infundibulum remain duct dependent on the flow of blood through the arterial duct despite adequate relief of the obstruction within the right ventricular outflow tract. The objective of our study was to review the risk factors for stenting of the patent arterial duct, or construction of a Blalock-Taussig shunt, in the patients with pulmonary atresia and an intact ventricular septum who remain duct-dependent following radiofrequency valvotomy and dilation of the imperforate pulmonary valve. We reviewed the data from 53 patients seen between November 1995 and December 2001. Of the 47 patients who survived, 6 required stenting of the patent arterial duct, while 4 needed construction of a modified Blalock-Taussig shunt to augment the flow of blood to the lungs at a mean of 7 plus or minus 5.7 days following the initial intervention. The remaining 37 patients required no additional procedures. We compared the findings in these two groups. The mean diameter of the tricuspid valve in the patients requiring early reintervention was 8.5 plus or minus 3.7 millimetres, giving a Z-score of -1.1 plus or minus 1.47, whilst those in the group without early reintervention had values of 10.7 plus or minus 2.2 millimetres, giving a Z-score of -0.58 plus or minus 1.18 (p equal to 0.003). No statistically significant differences were found in right ventricular morphology, McGoon ratio, or residual obstruction across the right ventricular outflow tract after decompression of the right ventricle. The diameter of the tricuspid valve, therefore, appears to be the only factor predicting the need for augmentation of flow of blood to the lungs. As just over one-fifth of our survivors required such augmentation, we hypothesize that stenting of the patent arterial duct may be performed as an integral part of primary transcatheter therapy in patients with pulmonary atresia and intact ventricular septum who have moderate right ventricular hypoplasia and a small tricuspid valve.
    Matched MeSH terms: Child, Preschool
  5. Ariffin H, Lum SH, Cheok SA, Shekhar K, Ariffin WA, Chan LL, et al.
    J Paediatr Child Health, 2005 Mar;41(3):136-9.
    PMID: 15790325
    To study the clinical presentation, therapy and outcome of children diagnosed with both primary and secondary haemophagocytic lymphohistiocytosis (HLH) at the University of Malaya Medical Centre.
    Matched MeSH terms: Child, Preschool
  6. Vijay K, Anindya C, Bhanu P, Mohan M, Rao PLNG
    Med J Malaysia, 2005 Mar;60(1):81-4.
    PMID: 16250285
    Adhesive small bowel obstruction (ASBO) is an annoying postoperative complication. Though the diagnosis can be made easily, the role of conservative management in children is controversial. Hence a study was conducted to determine the role of conservative management, and to identify the factors that can predict / influence the outcome of conservative treatment in children with ASBO. Children admitted with ASBO from 1980 to 2002 (22 year period) formed the material for this study. The data was analyzed with respect to the influence of age at the time of presentation, primary disease for which original laparotomy was done, time interval between the primary surgery and the development of ASBO and the number of laparotomies prior to the development of ASBO on the outcome of conservative management. There were 74 episodes of ASBO in 69 children (Five children had two episodes). Out of 74 episodes, 5 episodes (6.75%) needed immediate laparotomy for suspected gangrene. All others were managed conservatively. Of the 69 episodes managed conservatively, 36 responded to conservative treatment (2-5 days) while 33 required subsequent surgical intervention, with 11 of them requiring bowel resection (two for gangrene and 9 for bowel damage during adhesiolysis) and in the rest 22 cases adhesiolysis. A substantial number of children with ASBO respond well to conservative treatment. Majority of the children developed ASBO within three months after the primary laparotomy. Children below the age of one year (at the time of presentation with ASBO) responded poorly to the conservative management. Children who had primary surgery for Hirschsprung's disease and intussusception also appeared to have responded poorly to conservative management, but statistically not significant. Time interval between the primary surgery and the number of laparotomies before the child developed ASBO did not influence the outcome of conservative management.
    Matched MeSH terms: Child, Preschool
  7. Chua KB, Devi S, Hooi PS, Chong KH, Phua KL, Mak JW
    Malays J Pathol, 2003 Jun;25(1):49-56.
    PMID: 16196378
    An in-house prepared M. furfur antigen was used to carry out a seroprevalence study in an urban population in Malaysia by indirect immunofluorescence assay. Of the 800 serum samples from all ages screened, 738 samples were positive for M. furfur specific IgG, giving an overall seropositive rate of 92.3%. There was no significant difference in the seropositive rates among the different gender group and races. However, there was a statistical significant difference in the seropositive rate among different age groups with a lower rate (73%) for the age group 5 years old and below, which increased rapidly to 99% for the 16 to 20 years old age group but declined slightly for the oldest age group. The degree of seropositivity, which semi-quantitatively reflect the anti-M. furfur specific IgG titre, did not show any significant difference among the gender and racial groups. On the other hand, there was a significant difference in the degree of seropositivity among the various age groups, with the 16 to 20 years old age group having the highest antibody titre and the extreme of age groups having the lower antibody titre.
    Matched MeSH terms: Child, Preschool
  8. Weiss SL, Fitzgerald JC, Maffei FA, Kane JM, Rodriguez-Nunez A, Hsing DD, et al.
    Crit Care, 2015;19:325.
    PMID: 26373923 DOI: 10.1186/s13054-015-1055-x
    Consensus criteria for pediatric severe sepsis have standardized enrollment for research studies. However, the extent to which critically ill children identified by consensus criteria reflect physician diagnosis of severe sepsis, which underlies external validity for pediatric sepsis research, is not known. We sought to determine the agreement between physician diagnosis and consensus criteria to identify pediatric patients with severe sepsis across a network of international pediatric intensive care units (PICUs).
    Matched MeSH terms: Child, Preschool
  9. Salmasi S, Khan TM, Hong YH, Ming LC, Wong TW
    PLoS One, 2015;10(9):e0136545.
    PMID: 26340679 DOI: 10.1371/journal.pone.0136545
    BACKGROUND: Medication error (ME) is a worldwide issue, but most studies on ME have been undertaken in developed countries and very little is known about ME in Southeast Asian countries. This study aimed systematically to identify and review research done on ME in Southeast Asian countries in order to identify common types of ME and estimate its prevalence in this region.

    METHODS: The literature relating to MEs in Southeast Asian countries was systematically reviewed in December 2014 by using; Embase, Medline, Pubmed, ProQuest Central and the CINAHL. Inclusion criteria were studies (in any languages) that investigated the incidence and the contributing factors of ME in patients of all ages.

    RESULTS: The 17 included studies reported data from six of the eleven Southeast Asian countries: five studies in Singapore, four in Malaysia, three in Thailand, three in Vietnam, one in the Philippines and one in Indonesia. There was no data on MEs in Brunei, Laos, Cambodia, Myanmar and Timor. Of the seventeen included studies, eleven measured administration errors, four focused on prescribing errors, three were done on preparation errors, three on dispensing errors and two on transcribing errors. There was only one study of reconciliation error. Three studies were interventional.

    DISCUSSION: The most frequently reported types of administration error were incorrect time, omission error and incorrect dose. Staff shortages, and hence heavy workload for nurses, doctor/nurse distraction, and misinterpretation of the prescription/medication chart, were identified as contributing factors of ME. There is a serious lack of studies on this topic in this region which needs to be addressed if the issue of ME is to be fully understood and addressed.

    Matched MeSH terms: Child, Preschool
  10. Loh CK, Weis B, van Velthoven V, Reiff C, Rössler J
    J Neurol Sci, 2015 Nov 15;358(1-2):522-4.
    PMID: 26474792 DOI: 10.1016/j.jns.2015.09.375
    Optic glioma (OPG) accounts for 4-8% of all brain tumors in children. En-block removal of intraorbital tumor is recommended in cases with disfiguring exophthalmos and impaired vision. Surgical resection of intraorbital optic nerve (ON) poses the risks of permanent ptosis and globe atrophy. We present here the case of a 4-year-old boy with exophthalmos and near blindness due to an intraorbital OPG. Despite chemotherapy he showed progressive exophthalmos and vision loss. Bony orbital decompression with ON transection temporally reduced his exophthalmos. OPG resection was required later for recurrence of his exophthalmos secondary to tumor progression. Post operatively, he had preserved oculomotor nerve functions but developed globe ischemia. Unusually, his ischemic globe caused him to have pain and severe photophobia, which later lead to enucleation. Photophobia has been reported in blind patients. Animal models and MRI functional imaging showed activation of trigeminal pathway during photophobia in completely transected ON. However, the exact neuro-ophthalmology pathway requires further study.
    Matched MeSH terms: Child, Preschool
  11. Halim AS, Chai SC, Wan Ismail WF, Wan Azman WS, Mat Saad AZ, Wan Z
    J Plast Reconstr Aesthet Surg, 2015 Dec;68(12):1755-62.
    PMID: 26420474 DOI: 10.1016/j.bjps.2015.08.013
    Reconstruction of massive bone defects in bone tumors with allografts has been shown to have significant complications including infection, delayed or nonunion of allograft, and allograft fracture. Resection compounded with soft tissue defects requires skin coverage. A composite osteocutaneous free fibula offers an optimal solution where the allografts can be augmented mechanically and achieve biological incorporation. Following resection, the cutaneous component of the free osteocutaneous fibula flaps covers the massive soft tissue defect. In this retrospective study, the long-term outcome of 12 patients, who underwent single-stage limb reconstruction with massive allograft and free fibula osteocutaneous flaps instead of free fibula osteal flaps only, was evaluated. This study included 12 consecutive patients who had primary bone tumors and had follow-up for a minimum of 24 months. The mean age at the time of surgery was 19.8 years. A total of eight patients had primary malignant bone tumors (five osteosarcomas, two chondrosarcomas and one synovial sarcoma), and four patients had benign bone tumors (two giant-cell tumors, one aneurysmal bone cyst, and one neurofibromatosis). The mean follow-up for the 12 patients was 63 months (range 24-124 months). Out of the 10 patients, nine underwent lower-limb reconstruction and ambulated with partial weight bearing and full weight bearing at an average of 4.2 months and 8.2 months, respectively. In conclusion, augmentation of a massive allograft with free fibula osteocutaneous flap is an excellent alternative for reducing the long-term complication of massive allograft and concurrently addresses the soft tissue coverage.
    Matched MeSH terms: Child, Preschool
  12. NikNadia N, Sam IC, Rampal S, WanNorAmalina W, NurAtifah G, Verasahib K, et al.
    PLoS Negl Trop Dis, 2016 Mar;10(3):e0004562.
    PMID: 27010319 DOI: 10.1371/journal.pntd.0004562
    Enterovirus A71 (EV-A71) is an important emerging pathogen causing large epidemics of hand, foot and mouth disease (HFMD) in children. In Malaysia, since the first EV-A71 epidemic in 1997, recurrent cyclical epidemics have occurred every 2-3 years for reasons that remain unclear. We hypothesize that this cyclical pattern is due to changes in population immunity in children (measured as seroprevalence). Neutralizing antibody titers against EV-A71 were measured in 2,141 residual serum samples collected from children ≤12 years old between 1995 and 2012 to determine the seroprevalence of EV-A71. Reported national HFMD incidence was highest in children <2 years, and decreased with age; in support of this, EV-A71 seroprevalence was significantly associated with age, indicating greater susceptibility in younger children. EV-A71 epidemics are also characterized by peaks of increased genetic diversity, often with genotype changes. Cross-sectional time series analysis was used to model the association between EV-A71 epidemic periods and EV-A71 seroprevalence adjusting for age and climatic variables (temperature, rainfall, rain days and ultraviolet radiance). A 10% increase in absolute monthly EV-A71 seroprevalence was associated with a 45% higher odds of an epidemic (adjusted odds ratio, aOR1.45; 95% CI 1.24-1.69; P<0.001). Every 10% decrease in seroprevalence between preceding and current months was associated with a 16% higher odds of an epidemic (aOR = 1.16; CI 1.01-1.34 P<0.034). In summary, the 2-3 year cyclical pattern of EV-A71 epidemics in Malaysia is mainly due to the fall of population immunity accompanying the accumulation of susceptible children between epidemics. This study will impact the future planning, timing and target populations for vaccine programs.
    Matched MeSH terms: Child, Preschool
  13. Reed Z, Cardosa MJ
    Vaccine, 2016 06 03;34(26):2967-2970.
    PMID: 26973065 DOI: 10.1016/j.vaccine.2016.02.077
    Although outbreaks of Hand, Foot, and Mouth Disease (HFMD) in young children have long been recognized worldwide, the occurrence of rare and life-threatening neurological, respiratory, and cardiac complications has propelled this common condition into the spotlight as a major public health problem in the affected countries. Various enteroviruses cause HFMD, but the severe complications have been mostly associated with enterovirus 71 (EV71). Medical treatment is supportive and measures to interrupt transmission have been challenging to implement. Preventive vaccines could have an important clinical impact, especially among children younger than 3 years old who are most susceptible to the neurological complications. Several groups in the highly affected Asia-Pacific region are working towards vaccines against EV71 and some candidates have progressed to late-stage clinical trials with two vaccines recently reported to have been approved by the regulatory authorities in China. This report summarizes current issues and progress in the development of vaccines against EV71.
    Matched MeSH terms: Child, Preschool
  14. Bao Khanh le N, Burgers MR, Huu Chinh N, Tuoc BV, Dinh Dung N, Deurenberg P, et al.
    Food Nutr Bull, 2016 Mar;37(1):100-11.
    PMID: 27004970 DOI: 10.1177/0379572116631642
    The traditional Vietnamese diet carries the risk of micronutrient deficiencies, and a substantial part of children <11 years do not meet the Vietnamese recommended dietary allowance (RDA) for a range of nutrients. Dairy products are known for their high nutrient density and milk in particular for its provision of high-quality protein and relevant concentrations of calcium, magnesium, vitamin B2, vitamin B12, and pantothenic acid.
    Matched MeSH terms: Child, Preschool
  15. El Kishawi RR, Soo KL, Abed YA, Wan Muda WA
    PLoS One, 2016;11(3):e0151494.
    PMID: 26990551 DOI: 10.1371/journal.pone.0151494
    BACKGROUND: In many developing countries nutritional, and epidemiological transitions are emerging into continuing undernutrition and escalating overnutrition, giving rise to the double burden of the malnutrition phenomenon.

    OBJECTIVES: This study aims to determine the prevalence of the dual form of malnutrition (overweight mother/underweight child) in the same household and its associated factors in the Gaza Strip.

    METHODS: A total of 357 mother-child pairs from the same households were surveyed from three different geographical locations in the Gaza Strip, namely, El Remal urban area, Jabalia refugee camp, and Al Qarara rural area. The height and weight of mothers aged 18-50 years were measured, and their body mass index (BMI) was computed. The mothers were categorized according to the criterion of the World Health Organization (WHO) for BMI as overweight if they have a BMI ≥ 25 kg/m2. Anthropometric indices were measured for children aged two to five years to classify the underweight children Z-score child's birth order (ORadj, 1.50, 95% CL, 1.22, 1.82; p = <0.001), father's educational (low or medium) levels (ORadj, 3.19, 95% CL, 1.07, 9.5; p = 0. 036), or (ORadj, 3.4, 95% CL, 1.12, 10.37; p = 0. 031), high scores of mothers' nutrition knowledge (ORadj, 1.23, 95% CL, 1.01, 1.52; p = 0. 048), and low monthly income (ORadj, 0.28, 95% CL, 0.09, 0.88; p = 0. 030).

    CONCLUSIONS: The results from this study showed the dual form of malnutrition in the same household was prevalent in the Gaza Strip. This is a public health issue that must be understood and addressed and policy makers must implement an appropriate nutrition action plan to control dual form of malnutrition based on the underlying specific risk factors in the study population. In addition, interventions are needed to help individuals to translate their nutrition knowledge into healthy dietary behaviors.

    Matched MeSH terms: Child, Preschool
  16. Hossain M, Mani KK, Mohd Sidik S, Kadir Shahar H
    Acta Paediatr, 2016 Aug;105(8):e373-8.
    PMID: 27111395 DOI: 10.1111/apa.13447
    AIM: There are an estimated 372 000 worldwide deaths by drowning every year, and it has been described as a secret epidemic in Bangladesh. The aim of this study was to develop an intervention package to prevent children under the age of five from drowning in rural Bangladesh.

    METHODS: This was a qualitative study using focus group discussions in three villages in rural Bangladesh. The 45 participants were mothers and fathers with children under five, the parents of children who had drowned and community leaders.

    RESULTS: The majority of the participants (71%) were male. The focus groups revealed that most drowning's occurred between 11am and 2pm and that risk factors included the following: children not being able to swim, ditches that were not filled in, lack of medical facilities, parents who were not aware of childhood drowning and lack of information through the media about how to prevent of childhood drowning. Suggestions included using a mobile-based short messaging service or voice calls to parents, especially mothers, could increase awareness and reduce the risk of childhood drowning.

    CONCLUSION: A safety education programme could be effective in increasing knowledge and changing attitudes, which could prevent drowning among children in Bangladesh.

    Matched MeSH terms: Child, Preschool
  17. Kosalaraksa P, Boettiger DC, Bunupuradah T, Hansudewechakul R, Saramony S, Do VC, et al.
    J Pediatric Infect Dis Soc, 2017 Jun 01;6(2):173-177.
    PMID: 27295973 DOI: 10.1093/jpids/piw031
    Background.: Regular CD4 count testing is often used to monitor antiretroviral therapy efficacy. However, this practice may be redundant in children with a suppressed human immunodeficiency virus (HIV) viral load.

    Methods: Study end points were as follows: (1) a CD4 count <200 cells/mm3 followed by a CD4 count ≥200 cells/mm3 (transient CD4 <200); (2) CD4 count <200 cells/mm3 confirmed within 6 months (confirmed CD4 <200); and (3) a new or recurrent World Health Organization (WHO) stage 3 or 4 illness (clinical failure). Kaplan-Meier curves and Cox regression were used to evaluate rates and predictors of transient CD4 <200, confirmed CD4 <200, and clinical failure among virally suppressed children aged 5-15 years who were enrolled in the TREAT Asia Pediatric HIV Observational Database.

    Results: Data from 967 children were included in the analysis. At the time of confirmed viral suppression, median age was 10.2 years, 50.4% of children were female, and 95.4% were perinatally infected with HIV. Median CD4 cell count was 837 cells/mm3, and 54.8% of children were classified as having WHO stage 3 or 4 disease. In total, 18 transient CD4 <200 events, 2 confirmed CD4 <200 events, and10 clinical failures occurred at rates of 0.73 (95% confidence interval [95% CI], 0.46-1.16), 0.08 (95% CI, 0.02-0.32), and 0.40 (95% CI, 0.22-0.75) events per 100 patient-years, respectively. CD4 <500 cells/mm3 at the time of viral suppression confirmation was associated with higher rates of both CD4 outcomes.

    Conclusions: Regular CD4 testing may be unnecessary for virally suppressed children aged 5-15 years with CD4 ≥500 cells/mm3.

    Matched MeSH terms: Child, Preschool
  18. Shanmugam S, Nathan AM, Zaki R, Tan KE, Eg KP, Thavagnanam S, et al.
    BMC Pediatr, 2016 06 23;16:80.
    PMID: 27339265 DOI: 10.1186/s12887-016-0616-8
    BACKGROUND: Noisy breathing is a common presenting symptom in children. The purpose of this study is to (a) assess parental ability to label wheeze, (b) compare the ability of parents of children with and without asthma to label wheeze and (c) determine factors affecting parental ability to label wheeze correctly.

    METHODS: This cross-sectional study in a tertiary hospital in Kuala Lumpur, Malaysia involved parents of children with asthma. Parents of children without asthma were the control group. Eleven validated video clips showing wheeze, stridor, transmitted noises, snoring or normal breathing were shown to the parents. Parents were asked, in English or Malay, "What do you call the sound this child is making?" and "Where do you think the sound is coming from?"

    RESULTS: Two hundred parents participated in this study: 100 had children with asthma while 100 did not. Most (71.5 %) answered in Malay. Only 38.5 % of parents correctly labelled wheeze. Parents were significantly better at locating than labelling wheeze (OR 2.4, 95 % CI 1.64-3.73). Parents with asthmatic children were not better at labelling wheeze than those without asthma (OR1.04, 95 % CI 0.59-1.84). Answering in English (OR 3.4, 95 % CI 1.69-7.14) and having older children with asthma (OR 9.09, 95 % CI 3.13-26.32) were associated with correct labelling of wheeze. Other sounds were mislabelled as wheeze by 16.5 % of respondents.

    CONCLUSION: Parental labelling of wheeze was inaccurate especially in the Malay language. Parents were better at identifying the origin of wheeze rather than labelling it. Physicians should be wary about parental reporting of wheeze as it may be inaccurate.

    Matched MeSH terms: Child, Preschool
  19. Tan JA, Kho SL, Ngim CF, Chua KH, Goh AS, Yeoh SL, et al.
    Sci Rep, 2016 06 08;6:26994.
    PMID: 27271331 DOI: 10.1038/srep26994
    Haemoglobin (Hb) Adana (HBA2:c.179>A) interacts with deletional and nondeletional α-thalassaemia mutations to produce HbH disorders with varying clinical manifestations from asymptomatic to severe anaemia with significant hepatosplenomegaly. Hb Adana carriers are generally asymptomatic and haemoglobin subtyping is unable to detect this highly unstable α-haemoglobin variant. This study identified 13 patients with compound heterozygosity for Hb Adana with either the 3.7 kb gene deletion (-α(3.7)), Hb Constant Spring (HbCS) (HBA2:c.427T>C) or Hb Paksé (HBA2:429A>T). Multiplex Amplification Refractory Mutation System was used for the detection of five deletional and six nondeletional α-thalassaemia mutations. Duplex-PCR was used to confirm Hb Paksé and HbCS. Results showed 84.6% of the Hb Adana patients were Malays. Using DNA studies, compound heterozygosity for Hb Adana and HbCS (α(codon 59)α/α(CS)α) was confirmed in 11 patients. A novel point in this investigation was that DNA studies confirmed Hb Paksé for the first time in a Malaysian patient (α(codon 59)α/α(Paksé)α) after nine years of being misdiagnosis with Hb Adana and HbCS (α(codon 59)α/α(CS)α). Thus, the reliance on haematology studies and Hb subtyping to detect Hb variants is inadequate in countries where thalassaemia is prevalent and caused by a wide spectrum of mutations.
    Matched MeSH terms: Child, Preschool
  20. Shamsuddin AM, Chen YC, Wong AR, Le TP, Anderson RH, Corno AF
    Interact Cardiovasc Thorac Surg, 2016 Aug;23(2):231-4.
    PMID: 27170744 DOI: 10.1093/icvts/ivw129
    OBJECTIVES: Doubly committed ventricular septal defects (VSDs) account for up to almost one-third of isolated ventricular septal defects in Asian countries, compared with only 1/20th in western populations. In our surgical experience, this type of defect accounted for almost three-quarters of our practice. To date, patch closure has been considered the gold standard for surgical treatment of these lesions. Our objectives are to evaluate the indications and examine the outcomes of surgery for doubly committed VSDs.

    METHODS: Between October 2013, when our service of paediatric cardiac surgery was opened, and December 2014, 24 patients were referred for surgical closure of VSDs. Among them, 17 patients (71%), with the median age of 6 years, ranging from 2 to 9 years, and with a median body weight of 19 kg, ranging from 11 to 56 kg, underwent surgical repair for doubly committed defects. In terms of size, the defect was considered moderate in 4 and large in 13. Aortic valvular regurgitation (AoVR) was present in 11 patients (65%) preoperatively, with associated malformations found in 14 (82%), with 5 patients (29%) having two or more associated defects.

    RESULTS: After surgery, there was trivial residual shunting in 2 patients (12%). AoVR persisted in 6 (35%), reducing to trivial in 5 (29%) and mild in 1 (6%). Mean stays in the intensive care unit and hospital were 2.6 ± 1.2 days, ranging from 2 to 7 days, and 6.8 ± 0.8 days, ranging from 6 to 9 days, respectively. The mean follow-up was 14 ± 4 months, ranging from 6 to 20 months, with no early or late deaths and without clinical deterioration.

    CONCLUSIONS: The incidence of doubly committed lesions is high in our experience, frequently associated with AoVR and other associated malformation. Early detection is crucial to prevent further progression of the disease. Patch closure remains the gold standard in management, not least since it allows simultaneous repair of associated intracardiac defects.

    Matched MeSH terms: Child, Preschool
Filters
Contact Us

Please provide feedback to Administrator (afdal@afpm.org.my)

External Links