Displaying publications 81 - 100 of 3443 in total

Abstract:
Sort:
  1. Ainoon O, Yu YH, Amir Muhriz AL, Boo NY, Cheong SK, Hamidah NH
    Hum Mutat, 2003 Jan;21(1):101.
    PMID: 12497642 DOI: 10.1002/humu.9103
    We performed DNA analysis using cord blood samples on 86 male Malay neonates diagnosed as G6PD deficiency in the National University of Malaysia Hospital by a combination of rapid PCR-based techniques, single-stranded conformation polymorphism analysis (SSCP) and DNA sequencing. We found 37.2% were 871G>A (G6PD Viangchan), 26.7% were nt 563 C>T (G6PD Mediterranean) and 15.1% were 487G>A (G6PD Mahidol) followed by 4.7% 1376G>T (G6PD Canton), 3.5% 383T>C (G6PD Vanua Lava), 3.5% 592C>T (G6PD Coimbra), 2.3% 1388G>A (G6PD Kaiping), 2.3% 1360C>T (G6PD Union), 2.3% 1003G>A (G6PD Chatham), 1.2% 131C>G (G6PD Orissa) and 1.2% 1361G>A (G6PD Andalus). Seventy-one (82.6%) of the 86 G6PD-deficient neonates had neonatal jaundice. Fifty seven (80%) of the 71 neonates with jaundice required phototherapy with only one neonate progressing to severe hyperbilirubinemia (serum bilirubin >340 micromol/l) requiring exchange transfusion. There was no significant difference in the incidence of neonatal jaundice, mean serum bilirubin level, mean age for peak serum bilirubin, percentage of babies requiring phototherapy and mean number of days of phototherapy between the three common variants. In conclusion, the molecular defects of Malay G6PD deficiency is heterogeneous and G6PD Viangchan, Mahidol and Mediterranean account for at least 80% of the cases. Our findings support the observation that G6PD Viangchan and Mahidol are common Southeast Asian variants. Their presence in the Malays suggests a common ancestral origin with the Cambodians, Laotians and Thais. Our findings together with other preliminary data on the presence of the Mediterranean variant in this region provide evidence of strong Arab influence in the Malay Archipelago.
    Matched MeSH terms: Infant, Newborn
  2. Ainoon O, Alawiyah A, Yu YH, Cheong SK, Hamidah NH, Boo NY, et al.
    PMID: 12971572
    Neonatal screening for G6PD deficiency has long been established in many countries. The aim of the study was to determine whether the routine semiquantitative fluorescent spot test could detect all cases of G6PD deficiency, including those cases with partial deficiency (residual red cell G6PD activity between 20-60% of normal). We compared the results of G6PD screening by the semiquantitative fluorescent spot test and quantitative G6PD activity assay on a group of 976 neonates and 67 known female heterozygotes. The values for mean G6PD activity of G6PD-normal neonates and 293 healthy adult females were determined. There was no significant difference in the mean normal G6PD activity between the two racial groups in the neonates (669 Malays, 307 Chinese) and in the 293 healthy adult females (150 Malays, 143 Chinese) group. The values for the upper limits of total deficiency (20% of normal residual activity) for neonates and adult females were 2.92 U/gHb and 1.54 U/gHb, respectively. The upper limits of partial deficiency (60% of normal residual activity) were 8.7 U/gHb and 4.6 U/gHb respectively. The prevalence of G6PD deficiency among the male neonates was 5.1% (26) by both the fluorescent spot test and the enzyme assay method. The G6PD activity levels of all 26 cases of G6PD-deficient male neonates were < 20% normal (severe enzyme deficiency). In the female neonate group, the frequency of G6PD deficiency was 1.3% (6 of 472) by the fluorescent spot test and 9.35% (44 of 472) by enzyme assay. The 6 cases diagnosed as deficient by the fluorescent spot test showed severe enzyme deficiency (< 2.92 U/gHb). The remaining 38 female neonates had partial enzyme deficiency and all were misdiagnosed as normal by the fluorescent spot test. In the female heterozygote group, G6PD deficiency was diagnosed in 53% (35 of 67) by enzyme assay and in 7.5% (4 of 67) of cases by the fluorescent spot test. The 4 cases detected by fluorescent spot test had severe enzyme deficiency (<1.6 U/gHb). The remaining 31 (46.3%) cases, diagnosed as normal by fluorescent spot test, showed partial G6PD deficiency. In conclusion, we found that the semiquantitative fluorescent spot test could only diagnose cases of total G6PD deficiency and misclassified the partially-deficient cases as normal. In this study, the overall prevalence of G6PD deficiency was 3.28% by the semiquantitative fluorescent spot test and 7.17% by enzyme assay. This means that 3.9% of G6PD-deficient neonates were missed by the routine fluorescent spot test and they were found to be exclusively females. This study demonstrates a need to use a method that can correctly classify female heterozygotes with partial G6PD deficiency. The clinical implication is that these individuals may be at risk of the hemolytic complication of G6PD deficiency.
    Matched MeSH terms: Infant, Newborn
  3. Ainoon O, Joyce J, Boo NY, Cheong SK, Hamidah NH
    Malays J Pathol, 1995 Dec;17(2):61-5.
    PMID: 8935127
    G6PD deficiency is the most common human enzymopathy and affects 200 million people worldwide. To date more than 400 biochemical variants and at least 60 different point mutations in the G6PD locus have been discovered. In Malaysia the overall incidence of G6PD deficiency among males is 3.1%, being more prevalent among the Chinese and Malays and less common among the Indians. As part of our initial effort to characterise G6PD deficiency in the Malaysian population, we investigated 18 G6PD deficient Chinese male neonates for the G6PD mutation G-->T at nt 1376, a common mutation seen among the Chinese in Taiwan and mainland China. The mutation was detected by a PCR-based technique using primers that artificially create a site for restriction enzyme Xho I. We found 61% (11 out of 18) of the Chinese G6PD deficient male neonates positive for this mutation. Study of enzyme electrophoretic mobility in 7 of the cases positive for this mutation revealed three different patterns of mobility. 107% (5 out of 7), 103% (1 out of 7) and 100% (1 out of 7). This study shows that mutation G-->T at nt 1376 is a common allele causing G6PD deficiency in Malaysians of Chinese origin. The finding of different patterns of electrophoretic mobility among the 7 cases positive for 1376 G-->T mutation supports the notion that diverse biochemical variants may share the same mutation.
    Matched MeSH terms: Infant, Newborn
  4. Aisha Fadhilah Abang Abdullah, Kee, Sze Ying, Kamarul Azhar Mohd Razali, Jamal Mohamed, Thahira A., Zubaidah Abdul Wahab, Norlijah Othman
    MyJurnal
    Introduction and Objective: Pneumococcal disease is a leading cause of morbidity and mortality worldwide. There were limited publications on invasive pneumococcal infection (IPD) in Malaysia. The aim of this study is to describe restrospectively cases of IPD in hospitalised children of less than 12 years old and highlighting the unusual cases.

    Methodology: A retrospective review of children with IPD from March 2002 to November 2005 at a tertiary paediatric hospital. IPD cases were defined as isolates of Streptococcus pneumoniae from a normally sterile body fluid site.

    Results: Twenty-four patients were identified with a male preponderance. Two-thirds of patients were below 1-year-old; with three cases presenting in the premature newborn. Thirty-seven percent of cases had underlying conditions. Sepsis and pneumonia were the commonest manifestation, followed by meningitis. The unusual manifestations were in a form of postinfectious glomerulonephritis and overwhelming purpura fulminans. There were two mortalities; both infants had meningitis. Antibiotic susceptibility pattern showed that more than half of the isolates were sensitive towards penicillin and erythromycin. Penicillin resistance was found in 6 (25%) isolates. Conclusion: IPD results in significant morbidity and mortality, especially in young children below 2 years of age and justifies further evaluation of preventive strategies including the implementation of pneumococcal vaccine in the national immunisation programme.
    Matched MeSH terms: Infant; Infant, Newborn
  5. Aisha Fadhilah Abang Abdullah, Zurina Zainudin, Dg. Zuraini Sahadan
    MyJurnal
    Cytomegalovirus (CMV) is frequently isolated from neonates. Symptomatic infection is only apparent in 10% of affected babies with particular predilection for the reticuloendothelial and central nervous system. Isolated respiratory system involvement is rarely encountered. We report a case of a premature 32 weeks infant who required prolonged oxygen dependency and treated for bronchopulmonary dysplasia. The diagnosis of CMV pneumonitis was only discovered after detection of CMV DNA in the bronchoalveolar lavage. A high level of clinical awareness is crucial as a definite diagnosis and treatment will significantly alter the morbidity and the cost of therapy.
    Matched MeSH terms: Infant; Infant, Newborn; Infant, Premature
  6. Aishah Faiqah Mohd Yusof, Prabhakaran P, Nur Diyana Azli, Norrakiah Abdullah Sani, Wan Syaidatul Aqma
    Sains Malaysiana, 2017;46:903-908.
    Pacifier nipples are in permanent contact with saliva and with the oral microflora therefore, act as a favoured site for the growth of biofilms. This research was conducted to identify the bacterial biofilms that has been found on the pacifiers that collected from local child nursery and to analyse the formation of biofilms by Cronobacter sp. during growth in infant formula milk. Pacifiers collected were analysed to obtain colony forming unit (CFU) and isolated bacteria were identified using several biochemical tests according to Bergey's Manual. Biofilm assay of three Cronobacter sp. were conducted using 24 wells microtiter plate and stained with 1% of crystal violet solution at different time interval: 6, 12, 18 and 24 h. The hydrophobicity of the bacterial cell suspension was evaluated using bacterial adhesion to hydrocarbons (BATH) method. Extracellular polymeric substances (EPS) analysis was done to identify percentage of carbohydrate and protein content by using phenol sulphuric acid method and Bradford method, respectively. The results obtained showed that the normal microflora bacteria were the most abundant microorganisms that were found on the pacifier with the main genus isolated was Staphylococcus sp., Enterobacteriaceae sp. and Clostridium sp. Based on biofilm and EPS analysis, Cronobacter sakazakii formed a strong biofilms after 18 h, with carbohydrate was identified as main component of EPS.
    Matched MeSH terms: Infant; Infant Formula
  7. Ajura Abdul Jalil, Lukman Md Auzair, Hin, Lau Shin
    MyJurnal
    Congenital epulis is a fairly rare soft tissue tumour occurring exclusively on the alveolar ridge of newborns. The exact origin of congenital epulis is still debatable. The objective of the study is to determine the clinicopathological features and immunohistochemical findings of congenital epulis. A retrospective study was carried out to determine the clinicopathological features of congenital epulis, diagnosed histologically in the main oral histopathology laboratory in Malaysia from 1967 to 2014. Immunostaining using vimentin, muscle specific actin, smooth muscle antigen, desmin, S100, CD34, CD68 and CD1a was carried out. Twelve cases of congenital epulis were reviewed. All of the patients were females and the presentation age ranged from 2 to 90 days. The patients comprised of 6 Malays, 3 Chinese, 2 Indians and 1 Orang Asli. Most of the cases (n=7) involved the maxillary ridge and presented as pedunculated well-defined lumps (n=8). Excisional biopsy was performed in all cases. Via immunohistochemistry, vimentin expression was observed in all cases; but negative for CD34, muscle specific actin, smooth muscle antigen, and desmin. CD1a and S100 positivity was seen in five cases. The interstitial cells were highlighted by CD68. Although congenital epulis has been first described 130 years ago, the exact nature of its histogenesis remains a mystery.
    Matched MeSH terms: Infant, Newborn
  8. Akseer N, Lawn JE, Keenan W, Konstantopoulos A, Cooper P, Ismail Z, et al.
    Int J Gynaecol Obstet, 2015 Oct;131 Suppl 1:S43-8.
    PMID: 26433505 DOI: 10.1016/j.ijgo.2015.03.017
    The end of the Millennium Development Goal (MDG) era was marked in 2015, and while maternal and child mortality have been halved, MGD 4 and MDG 5 are off-track at the global level. Reductions in neonatal death rates (age <1 month) lag behind those for post-neonates (age 1-59 months), and stillbirth rates (omitted from the MDGs) have been virtually unchanged. Hence, almost half of under-five deaths are newborns, yet about 80% of these are preventable using cost-effective interventions. The Every Newborn Action Plan has been endorsed by the World Health Assembly and ratified by many stakeholders and donors to reduce neonatal deaths and stillbirths to 10 per 1000 births by 2035. The plan provides an evidence-based framework for scaling up of essential interventions across the continuum of care with the potential to prevent the deaths of approximately three million newborns, mothers, and stillbirths every year. Two million stillbirths and newborns could be saved by care at birth and care of small and sick newborns, giving a triple return on investment at this key time. Commitment, investment, and intentional leadership from global and national stakeholders, including all healthcare professionals, can make these ambitious goals attainable.
    Matched MeSH terms: Infant Mortality; Infant, Newborn
  9. Akter SF, Heller RD, Smith AJ, Milly AF
    J Infect Dev Ctries, 2009 Jul 01;3(6):447-51.
    PMID: 19762958
    BACKGROUND: Antimicrobials are often used inappropriately in paediatric wards of medical college hospitals in Bangladesh. Most of the antimicrobials are prescribed based on clinical grounds-signs and symptoms. This intervention study assessed the effectiveness of a training intervention on antimicrobials prescribing by physicians in paediatric wards of tertiary care level hospitals.

    METHODOLOGY: This study was conducted at medical college hospitals in Bangladesh during the period from 1998 through 2000. The pre-intervention survey of antimicrobial use was conducted during 1998 in five hospitals. The post-intervention survey was conducted after the interactive training during the succeeding year in three of the original five hospitals, of which one was the intervention hospital and two control hospitals. A total of 3,466 admitted paediatric patients' treatment charts (2,171 in the pre-intervention and 1,295 in the post-intervention surveys) were reviewed.

    RESULTS: The most commonly used antimicrobials were ampicillin, gentamicin, amoxicillin, cloxacillin and ceftriaxone. Appropriate antimicrobial therapy for the most common infectious diseases, pneumonia and diarrhoea, increased by 16.4% and 56.8% respectively in the intervention hospital compared with the two control hospitals and these improvements were significant (p = < 0.001 and p = 0.002, for pneumonia and diarrhoea respectively).

    CONCLUSIONS: An interactive, focussed educational intervention, targeted at physicians, appears to have been effective in improving appropriate antimicrobial prescribing for the most common paediatric infectious diseases in a medical college hospital in Bangladesh.

    Matched MeSH terms: Infant; Infant, Newborn
  10. Al Maghaireh DF, Abdullah KL, Chong MC, Chua YP, Al Kawafha MM
    J Pediatr Nurs, 2017 06 19;36:132-140.
    PMID: 28888494 DOI: 10.1016/j.pedn.2017.06.007
    PURPOSE: To investigate the stressors and stress levels among Jordanian parents of infants in the NICU and their relationship to three factors: anxiety, depression and sleep disturbance.

    DESIGN AND METHODS: A cross-sectional survey was conducted in two hospitals in Jordan among 310 parents of infants in the NICU by using PSS: NICU and PROMIS.

    RESULTS: Both parents experienced high levels of stress, anxiety, depression and sleep disturbance. There was a significant difference in stress level between mothers and fathers [t (308)=3.471, p=0.001], with the mothers experiencing higher stress than the fathers [mean: mothers=108.58; fathers=101.68]. The highest and lowest sources of stress were infant behavior and appearance (M=4.09) and sights and sounds in the NICU (M=3.54), respectively. The correlation between stress levels with anxiety (r=0.79) and depression (r=0.75) was strong and positive while sleep disturbance was significant and moderate (r=0.43).

    CONCLUSIONS: The mothers experienced higher levels of stress compared to fathers, with positive correlations between stress and anxiety, depression and sleep disturbance.

    PRACTICAL IMPLICATIONS: The findings of this study create nursing awareness of parent stress and its impact, which will help them to improve nursing care for parents.

    Matched MeSH terms: Infant; Infant, Newborn
  11. Al Qabbani A, Rani KGA, Syarif J, AlKawas S, Sheikh Abdul Hamid S, Samsudin AR, et al.
    PLoS One, 2023;18(4):e0283922.
    PMID: 37018321 DOI: 10.1371/journal.pone.0283922
    Current immunological issues in bone grafting regarding the transfer of xenogeneic donor bone cells into the recipient are challenging the industry to produce safer acellular natural matrices for bone regeneration. The aim of this study was to investigate the efficacy of a novel decellularization technique for producing bovine cancellous bone scaffold and compare its physicochemical, mechanical, and biological characteristics with demineralized cancellous bone scaffold in an in-vitro study. Cancellous bone blocks were harvested from a bovine femoral head (18-24 months old) subjected to physical cleansing and chemical defatting, and further processed in two ways. Group I was subjected to demineralization, while Group II underwent decellularization through physical, chemical, and enzymatic treatments. Both were then freeze-dried, and gamma radiated, finally producing a demineralized bovine cancellous bone (DMB) scaffold and decellularized bovine cancellous bone (DCC) scaffold. Both DMB and DCC scaffolds were subjected to histological evaluation, scanning electron microscopy/energy-dispersive X-ray spectroscopy (SEM/EDS), fourier-transform infrared spectroscopy (FTIR), quantification of lipid, collagen, and residual nucleic acid content, and mechanical testing. The osteogenic potential was investigated through the recellularization of scaffolds with human osteoblast cell seeding and examined for cell attachment, proliferation, and mineralization by Alizarin staining and gene expression. DCC produced a complete acellular extracellular matrix (ECM) with the absence of nucleic acid content, wider pores with extensive interconnectivity and partially retaining collagen fibrils. DCC demonstrated a higher cell proliferation rate, upregulation of osteogenic differentiation markers, and substantial mineralized nodules production. Our findings suggest that the decellularization technique produced an acellular DCC scaffold with minimal damage to ECM and possesses osteogenic potential through the mechanisms of osteoconduction, osteoinduction, and osteogenesis in-vitro.
    Matched MeSH terms: Infant
  12. Al-Areeqi MA, Sady H, Al-Mekhlafi HM, Anuar TS, Al-Adhroey AH, Atroosh WM, et al.
    Trop Med Int Health, 2017 04;22(4):493-504.
    PMID: 28151567 DOI: 10.1111/tmi.12848
    OBJECTIVES: To investigate the molecular epidemiology of Entamoeba histolytica, E. dispar and E. moshkovskii infections among rural communities in Yemen.

    METHODS: In a community-based study, faecal samples were collected from 605 participants and examined by wet mount, formalin-ether sedimentation, trichrome staining and nested multiplex PCR techniques. Demographic, socio-economic and environmental information was collected using a pre-tested questionnaire.

    RESULTS: Overall, 324 (53.6%) of the samples were positive for Entamoeba cysts and/or trophozoites by microscopic examination. Molecular analysis revealed that 20.2%, 15.7% and 18.2% of the samples were positive for E. histolytica, E. dispar and E. moshkovskii, respectively. Multivariate analysis showed different sets of species-specific risk factors among these communities. Educational level was identified as the significant risk factor for E. histolytica; age and gender were the significant risk factors for E. moshkovskii; and sources of drinking water and consumption of unwashed vegetables were the significant risk factors for E. dispar. Moreover, living in coastal/foothill areas and presence of other infected family members were risk factors for both E. histolytica and E. moshkovskii infections.

    CONCLUSION: The study reveals that Entamoeba spp. infection is highly prevalent among rural communities in Yemen, with E. histolytica, E. dispar and E. moshkovskii differentiated for the first time. Identifying and treating infected family members, providing health education pertinent to good personal and food hygiene practices and providing clean drinking water should be considered in developing a strategy to control intestinal parasitic infections in these communities, particularly in the coastal/foothill areas of the country.

    Matched MeSH terms: Infant
  13. Al-Herz W, Essa S
    Front Immunol, 2019;10:1231.
    PMID: 31191561 DOI: 10.3389/fimmu.2019.01231
    Objective: To present the frequency and spectrum of viral infections in primary immunodeficient children. Methods: The data was obtained from the Kuwait National Primary Immunodeficiency Disorders (PIDs) Registry during the period of 2004-2018. Results: A total of 274 PID children were registered in KNPIDR during the study period with predominance of immunodeficiencies affecting cellular and humoral immunity, followed by combined immunodeficiencies with associated syndromic features and diseases of immune dysregulation. Overall infectious complications affected 82.4% of the patients, and viral infections affected 31.7% of the registered patients. Forty-five patients (16.4%) developed viral infections caused by at least 2 organisms, among those 20 patients were affected by three or more viral infections. There was a statistically significant association between viral infections and PID category. However, there was no statistically significant association between viral infections and gender or the patients' onset age. There was a total of 170 viral infections during the study period and the causes of these infections were predominated by CMV (22.2%), adenovirus (11.7%), EBV (11.1%), and enteroviruses (7.4%). CMV and parainfluenza infections were more common in the group of immunodeficiencies affecting cellular and humoral immunity while EBV and human papilloma virus (HPV) were more common in the immune dysregulation group and combined immunodeficiencies with associated syndromic features, respectively. The most common presentation was viremia (28.8%) followed by pneumonia (28.2%) and skin infections (17.6%). The most common causes of viremia were CMV followed by adenovirus and EBV, while the most common organisms causing pneumonia were CMV followed by rhinovirus and parainfluenza. There were 80 deaths among the registered patients, 10% were caused by viral infections. Conclusions: Viral infections are common in PIDs and result into a wide-range of clinical manifestations causing significant morbidity and mortality.
    Matched MeSH terms: Infant
  14. Al-Herz W, Al-Ahmad M, Al-Khabaz A, Husain A, Sadek A, Othman Y
    Front Immunol, 2019;10:1754.
    PMID: 31396239 DOI: 10.3389/fimmu.2019.01754
    Objective: To present the report from the Kuwait National Primary Immunodeficiency Registry between 2004 and 2018. Methods: The patients were followed prospectively between January 2004 and December 2018 and their collected data included sociodemographic, diagnosis, clinical presentation, laboratory tests, and treatment. Results: A total of 314 PID patients (165 males and 149 females) were registered during the study period. Most of the patients (n = 287, 91.4%) were Kuwaiti nationals and the prevalence among Kuwaitis was 20.27/100,000 with a cumulative incidence of 24.96/100,000 Kuwaitis. The distribution of the patients according to PID categories was as follow: immunodeficiencies affecting cellular and humoral immunity, 100 patients (31.8%); combined immunodeficiencies with associated syndromic features, 68 patients (21.7%); predominantly antibody deficiencies, 56 patients (17.8%); diseases of immune dysregulation, 47 patients (15%); congenital defects of phagocyte number or function, 20 patients (6.4%); autoinflammatory disorders, 1 patient (0.3%); and complement deficiencies, 22 patients (7%). The mean age of the patients at onset of symptoms was 26 months while the mean age at diagnosis was 53 months and the mean delay in diagnosis was 27 months. Most of the patients (n = 272, 86%) had onset of symptoms before the age of 5 years. Parental consanguinity rate within the registered patients was 78% and a positive family history of PID was noticed in 50% of the patients. Genetic testing was performed in 69% of the patients with an overall diagnostic yield of 90%. Mutations were identified in 46 different genes and more than 90% of the reported genetic defects were transmitted by an autosomal recessive pattern. Intravenous immunoglobulins and stem cell transplantation were used in 58% and 25% of the patients, respectively. There were 81 deaths (26%) among the registered patients with a mean age of death of 25 months. Conclusions: PID is not infrequent in Kuwait and the reported prevalence is the highest in the literature with increased proportion of more severe forms. Collaborative efforts including introduction of newborn screening should be implemented to diagnose such cases earlier and improve the quality of life and prevent premature deaths.
    Matched MeSH terms: Infant, Newborn
  15. Al-Herz W
    J Clin Immunol, 2008 Mar;28(2):186-93.
    PMID: 18008151
    Primary immunodeficiency disorders are heterogeneous group of illnesses that predispose patients to serious complications. Registries for these disorders have provided important epidemiological data and shown both racial and geographical variations. The clinical features of 76 patients with primary immunodeficiency disorders registered in Kuwait National Primary Immunodeficiency Registry from 2004 to 2006 were recorded. Ninety-eight percent of the patients presented in childhood. The prevalence of these disorders in children was 11.98 in 100,000 children with an incidence of 10.06 in 100,000 children. The distribution of these patients according to each primary immunodeficiency category is: combined T and B cell immunodeficiencies (21%), predominantly antibody immunodeficiency (30%), other well defined immunodeficiencies (30%), diseases of immune dysregulation (7%), congenital defects of phagocyte number, function or both (8%), and complement deficiencies (4%). The consanguinity rate within the registered patients was 77%. The patients had a wide range of clinical features affecting different body systems. Primary immunodeficiency disorders are prevalent in Kuwait and have a significant impact into the health system.
    Matched MeSH terms: Infant; Infant, Newborn
  16. Al-Herz W, Husain EH, Adeli M, Al Farsi T, Al-Hammadi S, Al Kuwaiti AA, et al.
    Pediatr Infect Dis J, 2022 11 01;41(11):933-937.
    PMID: 36102730 DOI: 10.1097/INF.0000000000003678
    AIMS: To present the details of Bacillus Calmette-Guérin (BCG)-vaccine associated complications (VACs) in combined immunodeficiencies (CID) patients.

    METHODS: Five centers participated in this retrospective study and completed a data form, which included general patients' information, clinical and laboratory data.

    RESULTS: Among 236 CID patients, 127 were BCG vaccinated. 41.9% of patients with family history of CID and 17.1% who were diagnosed by screening were BCG vaccinated. Twenty-three patients (18.1%) developed BCG-VACs. The median age of VACs was 6 months and the median time from vaccination to complications was 6 months. The highest rate of BCG-VACs was recorded in patients receiving the Russian BCG strain compared to the Tokyo and Danish strains. Univariate analysis of T-lymphocyte subsets showed increased odds of BCG complications in patients with CD3+, CD4+, and CD8+ counts of ≤250 cells/µL. Only CD8 + count ≤250 cells/µL had increased such odds on multivariate analysis. VACs were disseminated in 13 and localized in 10 patients. Localized complication occurred earlier after vaccination (median: 4 months) compared with disseminated ones (median: 7 months). There were no significant associations between sex, administered vaccine strain, serum immunoglobulins levels, lymphocyte subsets counts, and the chance of having either localized or disseminated BCG-related complications.

    COCLUSIONS: Although contraindicated, many patients with CID continue to be vaccinated with BCG. Low CD8 + count is a risk factor for BCG-related complications and localized complications occurred earlier than disseminated ones. Considerations should be undertaken by health care authorities especially in countries with high incidence of CID to implement newborn screening, delay the time of BCG vaccine administration beyond 6 months of age and to use the relatively safer strains like the Danish and Tokyo ones.

    Matched MeSH terms: Infant; Infant, Newborn
  17. Al-Herz W, Zainal M, Nanda A
    Front Immunol, 2021;12:751469.
    PMID: 34659256 DOI: 10.3389/fimmu.2021.751469
    Background and Objectives: Reports on skin manifestations in inborn errors of immunity (IEI) are based on retrospective analysis, small series, or isolated case reports. The present prospective study aimed to determine the spectrum of skin manifestations in children with IEI and their relevance to specific molecular defects.

    Materials and Methods: The data were obtained from the Kuwait National Primary Immunodeficiency Disorders Registry during the period of 2004-2020.

    Results: A total of 313 pediatric cases of IEI, 71% diagnosed at molecular level, were registered with a cumulative follow-up period of 29,734 months. Skin manifestations were seen in 40.3% of the patients, and they were among the presenting manifestations in 33%. Patients with skin manifestations were older at both onset and diagnosis ages of IEI symptoms, but this was statistically significant for the latter only. The diagnosis delay was significantly longer in patients with skin manifestations. There was a statistically significant association between having skin manifestations and IEI category, being more common in patients with complement deficiencies, combined immunodeficiencies, and diseases of immune dysregulation. There was no statistically significant association between having skin manifestations and both gender and survival. Skin infections were the most frequent manifestations followed by eczema and autoimmune associations. Among IEI with more than 10 cases, skin lesions were a consistent finding in dedicator of cytokinesis 8 (DOCK8) deficiency, hyper IgE syndrome, ataxia-telangiectasia, and recombination activation gene (RAG)1 deficiency.

    Conclusions: Skin manifestations are common in IEI patients, and they had significant diagnosis delay and referral to specialists. Improvement of awareness about IEI is needed among pediatricians and dermatologists.

    Matched MeSH terms: Infant
  18. Al-Herz W, Ziyab AH, Adeli M, Al Farsi T, Al-Hammadi S, Al Kuwaiti AA, et al.
    Pediatr Allergy Immunol, 2022 Dec;33(12):e13901.
    PMID: 36564872 DOI: 10.1111/pai.13901
    BACKGROUND: There is an increased demand for hematopoietic stem cell transplant (HSCT) to treat various diseases including combined immunodeficiencies (CID), with limited worldwide availability. Variables affecting the decision regarding CID patients' prioritization for HSCT are not known. We aimed to determine general, clinical, and immunologic factors associated with the higher risk of early death (≤6 months after diagnosis) in untransplanted CID patients.

    METHODS: Data collection was done retrospectively from five centers and included general patients' information, and clinical and laboratory variables. Inclusion criteria were untransplanted patients who are either dead or alive with a follow-up period ≥6 months after diagnosis.

    RESULTS: Two hundred and thirty-six CID patients were reported by participating centers, of whom 111 were included in the study with a cumulative follow-up period of 278.6 years. Seventy-two patients died with the median age of death of 10.5 months. 35.1% of the patients succumbed within 6 months after the diagnosis. Having a history of Candida infections, sepsis or hepatomegaly was associated with an increased risk of early death. None of the other general or clinical variables was associated with such risk. Bivariate analysis of lymphocyte subsets showed that patients with the following counts: CD3+  

    Matched MeSH terms: Infant
  19. Al-Herz W, Ziyab AH, Adeli M, Al Farsi T, Al-Hammadi S, Al Kuwaiti AA, et al.
    Clin Immunol, 2023 Sep;254:109696.
    PMID: 37481010 DOI: 10.1016/j.clim.2023.109696
    AIMS: To understand the characteristics of combined immunodeficiency disorders that affect cellular and humoral immunity (CID) in the Arabian Peninsula.

    METHODS: Retrospective study of 236 patients with CID from the region were enrolled from 2004 to 2022.

    RESULTS: 236 patients were included with a majority being profound CID. Among patients with a family history of CID, the ages at onset and diagnosis, and the delay in diagnosis were lower compared to those with no family history of CID, but this did not affect time to transplant. HSCT was performed for 51.27% of the patients with median time from diagnosis to HSCT of 6.36 months. On multivariate analysis, patients who underwent early transplant had increased odds of having CD3 count ≤1000 cell/μl, diagnosed by screening or erythroderma.

    CONCLUSION: There is a delay in diagnosis and treatment of CID in our region. Establishing newborn screening programs and HSCT units in our region are the urgent need.

    Matched MeSH terms: Infant, Newborn
  20. Al-Mekhlafi AM, Mahdy MA, Al-Mekhlafi HM, Azazy AA, Fong MY
    Parasit Vectors, 2011;4:94.
    PMID: 21619624 DOI: 10.1186/1756-3305-4-94
    Malaria remains a significant health problem in Yemen with Plasmodium falciparum being the predominant species which is responsible for 90% of the malaria cases. Despite serious concerns regarding increasing drug resistance, chloroquine is still used for the prevention and treatment of malaria in Yemen. This study was carried out to determine the prevalence of choloroquine resistance (CQR) of P. falciparum isolated from Yemen based on the pfcrt T76 mutation.
    Matched MeSH terms: Infant; Infant, Newborn
Filters
Contact Us

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

External Links