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Fulltext Desmopressin acetate (DDAVP) for preventing and treating acute bleeds during pregnancy in women with congenital bleeding disorders

Karanth L, Barua A, Kanagasabai S, Nair NS

Cochrane Database Syst Rev, 2019 02 13;2:CD009824.
PMID: 30758840 DOI: 10.1002/14651858.CD009824.pub4

BACKGROUND: Congenital bleeding disorders can cause obstetric haemorrhage during pregnancy, labour and following delivery. Desmopressin acetate (DDAVP) is found to be an effective drug which can reduce the risk of haemorrhage and can also stop bleeding in certain congenital bleeding disorders. Its use in pregnancy has been controversial. Hence beneficial and adverse effects of DDAVP in these groups of pregnant women should be evaluated.This is an update of a Cochrane Review first published in 2013 and updated in 2015.
OBJECTIVES: To evaluate the efficacy and safety of DDAVP in preventing and treating acute bleeding in pregnant women with bleeding disorders.
SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coaguopathies Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant and abstract books of conferences proceedings. We also searched several clinical trial registries and grey literature (27 August 2017).Date of most recent search of the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coaguopathies Trials Register: 01 October 2018.
SELECTION CRITERIA: Randomised and quasi-randomised controlled trials investigating the efficacy of DDAVP versus tranexamic acid or factor VIII or rFactor VII or fresh frozen plasma in preventing and treating congenital bleeding disorders during pregnancy were eligible.
DATA COLLECTION AND ANALYSIS: No trials matching the selection criteria were eligible for inclusion.
MAIN RESULTS: No trials matching the selection criteria were eligible for inclusion.
AUTHORS' CONCLUSIONS: No randomised controlled trials were identified investigating the relative effectiveness of DDAVP for bleeding during pregnancy in women with congenital bleeding disorders. In the absence of high-quality evidence, clinicians need to use their clinical judgement and lower level evidence (e.g. from observational trials) to decide whether or not to treat women with congenital bleeding disorders with DDAVP.Given the ethical considerations, future randomised controlled trials are unlikely. However, other high-quality controlled studies (such as risk allocation designs, sequential design, parallel cohort design) to investigate the risks and benefits of using DDAVP in this population are needed.Given that there are unlikely to be any trials published in this area, this review will no longer be regularly updated.

Matched MeSH terms: Blood Coagulation Disorders/congenital
A review of aortic disease research in Malaysia

Tan GJS, Khoo PLZ, Sailesh MK, Chan KMJ

Med J Malaysia, 2019 02;74(1):67-78.
PMID: 30846666

INTRODUCTION: Aortic disease includes conditions such as chronic aortic aneurysms, acute aortic syndromes and congenital aortic abnormalities, amongst others. This paper reviews all research on aortic disease performed in Malaysia and published between 2000-2016.
METHODS: A literature search was conducted in PubMed, Scopus, MyJurnal and the UKM Journal Repository. The search process was based on a previously published methodology. The medical subject headings (MeSH) search terms used were "aortic", "aorta" and "Malaysia".
RESULTS: Two-hundred-thirteen papers were identified, of which 60 papers were selected and reviewed on the basis of their relevance. The epidemiology, pathophysiology, clinical presentations, case reports, investigations, treatment and outcomes of aortic disease in Malaysia were reviewed and summarised. The clinical relevance of the studies performed are discussed.
CONCLUSION: The review provided an insight into the pathophysiology, prevalence and epidemiology of aortic diseases in Malaysia, how the condition is managed, and the outcomes of treatment. Limitations of the research performed in Malaysia to date were identified and recommendations for further research and improvement in clinical practice were recommended.

Matched MeSH terms: Aortic Diseases/congenital
Fulltext Behavioural and emotional issues among primary school pupils with congenital colour vision deficiency in the Federal Territory of Kuala Lumpur, Malaysia: A case-control study

Thomas BAWM, Kaur S, Hairol MI, Ahmad M, Wee LH

F1000Res, 2018;7:1834.
PMID: 30815251 DOI: 10.12688/f1000research.17006.1

Background: Congenital colour vision deficiency (CCVD) is an untreatable disorder which has lifelong consequences. Increasing use of colours in schools has raised concern for pupils with CCVD. This case-control study was conducted to compare behavioural and emotional issues among age, gender and class-matched pupils with CCVD and normal colour vision (NCV). Methods: A total of 1732 pupils from 10 primary schools in the Federal Territory of Kuala Lumpur were screened, of which 46 pupils (45 males and 1 female) had CCVD. Mothers of male pupils with CCVD (n=44) and NCV (n=44) who gave consent were recruited to complete a self-administered parent report form, Child Behaviour Checklist for Ages 4-18 (CBCL/ 4-18) used to access behavioural and emotional problems. The CBCL/ 4-18 has three broad groupings: Internalising, Externalising and Total Behaviour Problems. Internalising Problems combines the Withdrawn, Somatic Complaints and Anxiety/ Depression sub constructs, while Externalising Problems combines the Delinquent and Aggressive Behaviour sub constructs. Results: Results from CBCL/ 4-18 showed that all pupils from both groups had scores within the normal range for all constructs. However, results from the statistical analysis for comparison, Mann-Whitney U test, showed that pupils with CCVD scored significantly higher for Externalising Problems (U=697.50, p=0.02) and Total Behaviour Problems (U=647.00, p= 0.01). Significantly higher scores were observed in Withdrawn (U=714.00, p=0.02), Thought Problems (U=438.50, p<0.001) and Aggressive Behaviour (U=738.00, p=0.04). Odds ratios, 95% CI, showed significant relative risk for high Total Behaviour Problem (OR:2.39 ,CI:1.0-5.7), Externalising Problems (OR:2.32, CI:1.0-5.5), Withdrawn (OR:2.67, CI:1.1-6.5), Thought Problems (OR:9.64, CI:3.6-26.1) and Aggressive Behaviour (OR:10.26, CI:3.4-31.0) scores among pupils with CCVD. Conclusion: Higher scores among CCVD pupils indicates that they present more behavioural and emotional problems compared to NCV pupils. Therefore, school vision screenings in Malaysia should also include colour vision to assist in the early clinical management of CCVD children.

Matched MeSH terms: Color Vision Defects/congenital*
Clinical and genetic analysis of long QT syndrome in two Malay children

Wong AR, Zilfalil BA, Bhuiyan ZA

Med J Malaysia, 2019 08;74(4):341-343.
PMID: 31424047

Long QT syndrome (LQTS) is predominantly a genetic cardiac arrhythmia disorder. We report here our study on long QT syndrome from two children from Kelantan, Malaysia. Clinical and genetic findings of these two unrelated Malay children with LQTS is discussed. We found a Long QT, type 1 causal mutation, p.Ile567Thr in the KCNQ1 gene in the first child. A pathogenic mutation could not be detected in the second child, explaining the heterogeneity of this disease.

Matched MeSH terms: Long QT Syndrome/congenital
Fulltext Health-related quality of life of female patients with congenital adrenal hyperplasia in Malaysia

Zainuddin AA, Grover SR, Abdul Ghani NA, Wu LL, Rasat R, Abdul Manaf MR, et al.

Health Qual Life Outcomes, 2020 Aug 01;18(1):258.
PMID: 32738912 DOI: 10.1186/s12955-020-01515-9

BACKGROUND: This study investigates the health-related quality of life (HRQOL) of female patients with congenital adrenal hyperplasia (CAH) in Malaysia. The objectives were to attain socio-demographic and medical data on these Malaysian females with CAH and establish their health-related quality of life (HRQOL) in comparison to age matched diabetic controls.
METHODS: A cross-sectional study was conducted over 6 months in the two main tertiary centres for CAH patients in Malaysia. Participants including 59 female-raised CAH patients (mean age ± SD = 16.3 ± 4.2 years, range 10-28 years) compared to 57 age-matched female diabetic patients (mean age ± SD = 16.5 ± 3.4 years, range 10-26 years). Socio-demographic and medical profiles was obtained through semi-structured interviews. HRQOL of participants were evaluated utilising validated, Malay translated questionnaires which were age appropriate: Pediatric Quality of Life Inventory (PedsQL v4.0) scales for Child (8-12) and Adolescent (13-18) and Medical Outcome Survey 36-item Short Form version. These were then compared to the diabetic controls.
RESULTS: The CAH participants consisted of children (ages 10-12 years, n = 12), adolescents (ages 13-17 years, n = 29) and adults (≥ 18 years, n = 18). The majority were Malays (64.4%) and had salt-wasting CAH (67.8%). There were no significant differences between the total mean score of the HRQOL of the combined children and adolescents CAH group (total mean score ± SD = 81.6 ± 17.9, 95% CI = 75.6-87.6) when compared to age-matched diabetic patients (total mean score ± SD = 80.8 ± 11.0, 95% CI = 77.0-84.5, P = 0.81, effect size = 0.05); no significant difference between the adult CAH and diabetic controls in the physical [median score (IQR) CAH vs diabetics; 49.3 (11.4) vs. 50.2 (6.1), P = 0.60, effect size = 0.09] and the mental composite scores [median score (IQR) CAH vs. diabetics; 47.8 (14.1) vs. 50.0 (10.8), P = 0.93, effect size = 0.01].
CONCLUSIONS: The HRQOL of the Malaysian CAH cohort were comparable to the diabetic controls.

Matched MeSH terms: Adrenal Hyperplasia, Congenital/psychology*
Of "Cotton Balls" and "Owl's eyes"

Misra S, Gupta A, Saran RK

Malays J Pathol, 2020 Dec;42(3):487-490.
PMID: 33361734

Report of a 3-month old girl child who died due to multi-systemic infection of cytomegalovirus (CMV) involving the lungs, liver and kidneys along with pneumocystis jiroveci pneumonia (PJP). The mother of the child tested positive for CMV IgG and HIV with a very low CD4 count (160/ μl). Co-infection of cytomegalovirus and pneumocystis jiroveci always occurs in the setting of immunocompromise. Congenital CMV infection is transmitted through the placenta, especially during the first trimester and causes severe multi-systemic disease whereas perinatal infection is acquired during childbirth/ breastfeeding where the babies have maternal protective antibodies leading to much milder or asymptomatic infection. PJP is more common in infancy and presents as hypoxic pneumonia. CMV causes cyto-nucleomegaly and classic "owl's eye" inclusions on histology while PJP presents with characteristic fluffy "cotton ball" alveolar exudates.

Matched MeSH terms: Cytomegalovirus Infections/congenital*
Neonatal chylous ascites--report of three cases and review of the literature

Chye JK, Lim CT, Van der Heuvel M

Pediatr Surg Int, 1997 Apr;12(4):296-8.
PMID: 9099650

Three cases of neonatal chylous ascites (CCA) were managed in the neonatal unit, University Hospital, Kuala Lumpur, over the past 9 years. Fetal ascites and polyhydramnios were the sole abnormalities detected in all three babies by antenatal ultrasonography. They were born at 36 weeks' gestation and their birth weights ranged from 3.0 kg to 3.8 kg. All three infants had abdominal distension at birth. Milky ascitic fluid was obtained after starting enteral feedings. Analysis of the ascitic fluid revealed a raised white blood cell count (predominantly lymphocytic) and triglycerides (1.4 - 3.8 mmol/l), cholesterol (1.6 - 2.8 mmol/l), and protein levels (25 - 41 g/l). Conservative management with skimmed milk and medium-chain triglycerides in one infant and Pregestimil in another was instituted. these two infants with CCA were clinically normal when reviewed at 19 months and 3.5 years of age. The third infant had a gut malrotation and associated pyloric septum; he died from complications of a laparotomy. The literature on this rare condition is reviewed.

Matched MeSH terms: Chylous Ascites/congenital*
Fulltext Ectrodactary, ectodermal dysplasia, and cleft lip-palate syndrome

Choong YY, Norazlina B

Med J Malaysia, 2001 Mar;56(1):88-91.
PMID: 11503303

The EEC syndrome (ectrodactyly, ectodermal dysplasia and cleft lip-palate) is a rare disorder inherited as an autosomal dominant trait or can occur sporadically. We describe a case of this syndrome with chronic ocular surface disorder secondary to abnormalities of ocular adnexia.

Matched MeSH terms: Limb Deformities, Congenital/complications*
Fulltext Retrospective review of serologic rubella activity in University Hospital Kuala Lumpur

Chua KB, Lam SK, Hooi PS, Chua BH, Lim CT

Med J Malaysia, 2000 Sep;55(3):299-303.
PMID: 11200707

A retrospective review of rubella serological results carried out in the Virus Diagnostic Unit, University Hospital Kuala Lumpur (UHKL) from January 1993 to September 1999 showed the presence of rubella infection annually which appeared to increase periodically every two to three years. There was no statistical significant difference in the rubella positive rate between male and female population aged 14 to 48 years. Congenital rubella infections (CRI) occurred in babies delivered in UHKL yearly from 1993 to 1998 with an average incidence rate of 48 per 100,000 deliveries. Peaks of rubella cases appeared to be followed by an increase in incidence of CRI cases 6 to 9 months later. The study showed that only 50.8% clinically diagnosed rubella was confirmed by laboratory finding. This study also showed an increase of rubella activity for the months of July, August and September 1999 and this may herald an increase of CRI cases in the coming millennium.

Matched MeSH terms: Rubella/congenital
Fulltext Parents' awareness and knowledge of the special needs of their hearing-impaired child

Mukari SZ, Vandort S, Ahmad K, Saim L, Mohamed AS

Med J Malaysia, 1999 Mar;54(1):87-95.
PMID: 10972010

We surveyed parents of school-aged hearing impaired children to investigate their awareness and knowledge of the special needs of their child, especially in the area of the usage of hearing aids and methods of communication. Questionnaires were distributed to parents of hearing impaired children at 13 special schools for the deaf in Malaysia. Out of 1,267 questionnaires given out, 787 (62.1%) were completed and returned. Results of the survey indicated the majority of parents (68.6%) suspected hearing loss late, that is after their child's first birthday, and there was a significant time lag before the suspicion was confirmed. Over 82.8% of the children were diagnosed only after 1 year of age, with 41.3% being diagnosed after 3 years of age. Hearing aids were fitted late (mean = 5.32 years; SD = 2.66). Hearing aid ownership was influenced by the factors of socio-economic level and ethnic group (p < 0.01) whereas knowledge of use and proper care of the aids was influenced by socio-economic level (p < 0.01). Communication methods were generally inappropriate with 41.3% of the mothers and 48.5% of the fathers reporting ignorance of Bahasa Malaysia Kod Tangan, the sign language that is commonly used by their children. The parents' choice of communication method was not significantly influenced by socio-economic level or ethnic group. The study revealed the present inadequate state of services available for the rehabilitation of children with congenital hearing impairment.

Matched MeSH terms: Hearing Disorders/congenital*
Fulltext The orthotic management of the congenitally short lower limb--a new appliance

Devnani AS

Singapore Med J, 2000 Nov;41(11):534-7.
PMID: 11284611

To describe an appliance used for equalisation of severe congenital lower limb length discrepancy for patients who refuse to undergo any operative correction but wish to walk and look better.

Matched MeSH terms: Leg Length Inequality/congenital*
Fulltext EEC syndrome with urogenital anomalies and compound naevus

Omar I, Jidon AJ

Med J Malaysia, 1993 Sep;48(3):364-8.
PMID: 8183155
Matched MeSH terms: Limb Deformities, Congenital*
Fulltext Case report--a neonate with nonimmune hydrops fetalis

Ram SP, Ariffin WA, Kassim Z

Singapore Med J, 1993 Oct;34(5):459-61.
PMID: 8153701

A post-dated intra-uterine growth retarded male Malay baby was born to a 30-year-old mother gravida II by Caesarean section. Her previous pregnancy ended in still-birth. The baby was severely asphyxiated at birth. He was intubated and immediately admitted to the neonatal intensive care unit. He had anasarca, anaemia, purpura and firm, massive hepatosplenomegaly. X-rays revealed ascites and bilateral metaphysiitis of the long bones. The haemoglobin level was 5.0 gm/dl and PCV 18.3%. Coombs' test was negative. Prothrombin time (PT) and partial thromboplastin time (PTT) were prolonged. The baby and mother were positive for Venereal Disease Research Laboratory (VDRL) and the treponema pallidum haemagglutination assay (TPHA) tests. The baby was actively resuscitated but expired at three and a half hours of life due to overwhelming sepsis associated with severe anaemia and disseminated intravascular coagulation.

Matched MeSH terms: Syphilis, Congenital/complications*
Birth defects in the offspring of non-Caucasian, non-Indigenous women in Western Australia

Kwon S, Bower C, English D

Birth Defects Res. Part A Clin. Mol. Teratol., 2003 Jul;67(7):515-21.
PMID: 14565623

Birth defects in infants born to non-Caucasian, non-Indigenous mothers in Australia have not been described in detail previously.

Matched MeSH terms: Congenital Abnormalities/epidemiology*
Fulltext Congenital atlanto-axial dislocation

Tan CT, Loh TG, Balasubramaniam P

Med J Malaysia, 1981 Dec;36(4):230-3.
PMID: 7334959
Matched MeSH terms: Dislocations/congenital*
Fulltext Torsade de pointes and syncopal attacks in a 26-year old woman with congenital complete heart block and prolonged QT interval

Quek DK, Ong SB

Singapore Med J, 1990 Apr;31(2):185-8.
PMID: 2371586

A 26-year old woman with congenital complete heart block and prolonged QT interval presented for the first time with syncopal attacks associated with torsade de pointes in adulthood. Cardioversion followed by overdrive pacing was needed to finally control the unstable rhythm. During episodes of non-capture, paraoxysms of torsade de pointes leading to ventricular flutter were recorded by a 24-hour ambulatory electrocardiographic monitoring. Beta-blockade and permanent ventricular pacing finally abolished both the syncopal attacks and the torsade phenomena. The prognosis of congenital complete heart block associated with QT prolongation resembles that of the Romano-Ward syndrome. Recognition of this variant would facilitate earlier treatment of this rare but potentially lethal disorder.

Matched MeSH terms: Heart Block/congenital*
Fulltext Pattern of heart disease and pregnancy seen in a Malaysian hospital

Ong HC, Puraviappan AP, Sinnathuray TA, Chong CH, Sen DK

Singapore Med J, 1978 Jun;19(2):93-7.
PMID: 751193

Pregnancy was associated with cardiac disease in 0.94% of pregnancies. 0.66% with rheumatic lesions and 0.25'\'0 with congenital lesions. The Malays had a higher incidence than the Chinese and Indians. Most of the cardiac pregnancies were in patients between 20-30 years of age and gravida 1 to 4. Nevertheless. still about 30% of cardiac pregnancies were in patients 30 years and over in age and 20% in patient gravida 5 and above. Rheumatic lesions predominated. involving mainly the mitral valve. of which the major lesion was mitral stenosis. PDA. ASD and VSD were the major congenital lesions. Major antepartum matemal complications included arrhythmias. heart failure, anemia and pre-eclampsia. Of the arrhythmias. atrial fibrillation and sinus tachycardia occurred most commonly. A high incidence of prophylactic forceps and vacuum deliveries
was evident. There was one matemal death. The majority of infants were bom alive and well.

Matched MeSH terms: Heart Defects, Congenital/epidemiology*
Dental caries in Malaysian children with heart defects

Johnson RO, Grieve AW

Med J Malaysia, 1978 Sep;33(1):44-6.
PMID: 750895
Matched MeSH terms: Heart Defects, Congenital/complications*
Fulltext Pulmonary valve echocardiogram in the evaluation of pulmonary arterial hypertension in the presence of intracardiac shunts

Fong CY, Singham KT

Med J Malaysia, 1986 Sep;41(3):198-204.
PMID: 3670135

The purpose of this study was to assess M-mode echocardiographic criteria for pulmonary artery hypertension in patients with intracardiac shunts, and to observe any signs not previously described.
Forty-seven consecutive cardiac cathetherised patients with routine M-mode echocardiogram done were reviewed. Twenty-six patients had atrial septal defect, nine had ventricular septal defect (VSD), four had patent ductus arteriosus (PDA), five had Eisenmenger syndrome and three had combined VSD and PDA.
Fifteen of 20 patients with pulmonary artery mean pressure (PAMP) of more than 50 mmHg demonstrated diminished a-dip (p < 0.001), reduced e-f slope (p < 0.01) and increased b-e slope to > 380 mm/sec (p < 0.01). The presence of systolic flutter or mid-systolic notch, or both was not observed in patients with normal PAMP, but was noted in all of the 20 patients with raised pressure(> 50 mmHg).
This study confirms the limitation of M-mode echocardiographic features in the evaluation of pulmonary artery hypertension in patients with intracardiac shunt, though clinically useful.

Matched MeSH terms: Heart Defects, Congenital/physiopathology*
Fulltext Emotional and behavioural problems among adolescent off-springs of mothers with depression

Wan Salwina, W.I., Nik Ruzyanei, N.K., Nurliza, A.M., Irma, A.M., Hafiz, B., Ayob, I., et al.

Malaysian Journal of Medicine and Health Sciences, 2013;9(2):35-43.
MyJurnal

Maternal depression has been linked to the development of adolescents’ emotional and behavioural problems. The main objective of this study was to determine the association between maternal depressive disorders and externalizing and internalizing problems among their adolescent children. This was a cross-sectional, comparison study of 35 mothers with depression and their adolescents, matched with 35 healthy mothers and their adolescents as controls. The mothers completed Quick Inventory Depressive Symptomatology (QIDS) for assessment of current depression. The emotional and behavioural problems in the adolescents were assessed independently by the mothers and their adolescents off-springs using Child Behavioral Checklist (CBCL) and Youth Self-Report (YSR), respectively. SPSS version 12.0 was used for statistical analysis. The findings showed that adolescents who have mothers with depressive disorders had significantly higher scores of externalizing (mean difference = 4.686 + 10.887, p = 0.016) and total emotional and behavioural problems (mean difference = 10.171 + 23.007, p = 0.013) than controls. The cases also scored higher than the controls in the following CBCL syndrome scales: aggressive behaviour (mean difference = 3.200 + 6.773, p = 0.008), social problem (mean difference = 1.286 + 2.865, p = 0.012), and attention problem (mean difference = 1.543 + 4.435, p = 0.047). Mothers with depressive disorders reported that their adolescents have greater emotional and behavioural problems than the controls. The findings suggested a need for preventive strategies to curb problematic behaviour focusing on this vulnerable group.

Matched MeSH terms: Ichthyosiform Erythroderma, Congenital; Limb Deformities, Congenital

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