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Stereotactic neuroendoscopic management of hydrocephalus: a three-year follow-up and analysis of Malaysian children with aqueduct stenosis

Abdullah J, Ariff AR, Ghazaime G, Naing NN

Stereotact Funct Neurosurg, 2001;76(3-4):175-80.
PMID: 12378096

The beneficial effects of stereotactic third ventriculostomy versus ventriculoperitoneal shunt were evaluated in 62 paediatric patients and analysed in relation to age, sex, clinical history, presence of meningomyelocele, magnetic resonance imaging measurements of hydrocephalus and third ventricle floor size. The third ventriculostomy were done on 50 patients using the Richard-Wolf Caemaert Endoscope and the Leksell Stereotactic Frame Model G. These patients were operated using the 4-French Fogarty catheter to open the base of the third ventricle. During the same period of study 12 paediatric patients with aqueduct stenosis who were managed by ventriculoperitoneal shunt were included. Both surgical procedures were compared. Statistically univariate analysis revealed that those patient with an age group of more than six months undergoing ventriculostomy had good outcome. Multivariate analysis revealed that past history of haemorrhage and/or meningitis were predictors of poor outcome. Sex, size of lumbar meningocele at birth, abnormal ventricular anatomy or narrow third ventricular floor size were non predictors of bad outcome in these patients. There was no difference in outcome in both the shunt or ventriculostomy group.

Matched MeSH terms: Infant, Newborn
Developmental haemostasis for factor V and factor VIII levels in neonates: a case report of spontaneous cephalhaematoma

Abdullah WZ, Ismail R, Nasir A, Mohamad N, Hassan R

Fetal Pediatr Pathol, 2013 Apr;32(2):77-81.
PMID: 22536947 DOI: 10.3109/15513815.2012.671447

Combined factor V and VIII deficiency is a rare bleeding disorder. Diagnosis of congenital coagulation factor deficiency in a neonate is challenging due to "immaturity" of the hemostatic system. A 2-day-old baby girl presented with spontaneous cephalhematoma. She was found to have persistent abnormal coagulation tests and finally diagnosed as combined factor V and VIII deficiency. Interestingly, factor V and factor VIII in developmental hemostasis are quite similar with adult levels in newborn, and hence early diagnosis is possible. An investigation to detect underlying hemostatic defects is recommended in newborns with spontaneous cephalhematoma.

Matched MeSH terms: Infant, Newborn; Infant, Newborn, Diseases
Fulltext Clinical situation of endemic malaria in Yemen

Abdulsalam M Q AM, Mohammed A K M, Ahmed A A, Fong MY

Trop Biomed, 2010 Dec;27(3):551-8.
PMID: 21399597 MyJurnal

Malaria remains a major public health problem causing mortality and morbidity in tropical and subtropical countries. A cross-sectional study was carried out to determine malaria prevalence and its clinical pattern during malaria season in Yemen. Blood samples were collected from 511 patients with fever who voluntary participated in this study, of them 268 were males and 242 females. Malaria was screened using Giemsa-stained thick and thin blood films. Clinical profile was recorded through physical and laboratory examinations and biodata were collected by pre-tested standard questionnaire. The overall prevalence was 15.3%. Three malaria species (Plasmodium falciparum, Plasmodium vivax and Plasmodium malarae) were detected with the predominance of P. falciparum (83.33%). People living in the rural areas had higher infection rate compared to urban areas (p < 0.005). Children were at higher risk of developing severe malaria compared to adults (p < 0.05). Severe anaemia, respiratory distress, jaundice, convulsion and bleeding were more apparent among younger age groups of malaria cases compared to older children. The study indicates that malaria is still a public health problem with children being at high risk of developing severe malaria which may lead to death.

Matched MeSH terms: Infant, Newborn
Fulltext The role of intravenous nutrition in low birth-weight infants

Abidin Z, Kuhan N

Med J Malaysia, 1984 Sep;39(3):192-6.
PMID: 6443580

Seven infants with birth weights less than lS00g received total parenteral nutrition for seven to 16 days through the peripheral route, commencing within the first 24 hours of life. Excessive weight loss was prevented and the infants showed significant weight gain. The time required to regain the initial birth weight showed an improvement over that expected in conventionally managed infants. Metabolic and septic complications were minimal and easily manageable. The results demonstrate that the technique, when properly used, warrants consideration in feeding low birth weight infants incapable of tolerating enteral feeding.

Matched MeSH terms: Infant, Newborn
Global systematic review of primary immunodeficiency registries

Abolhassani H, Azizi G, Sharifi L, Yazdani R, Mohsenzadegan M, Delavari S, et al.

Expert Rev Clin Immunol, 2020 07;16(7):717-732.
PMID: 32720819 DOI: 10.1080/1744666X.2020.1801422

INTRODUCTION: During the last 4 decades, registration of patients with primary immunodeficiencies (PID) has played an essential role in different aspects of these diseases worldwide including epidemiological indexes, policymaking, quality controls of care/life, facilitation of genetic studies and clinical trials as well as improving our understanding about the natural history of the disease and the immune system function. However, due to the limitation of sustainable resources supporting these registries, inconsistency in diagnostic criteria and lack of molecular diagnosis as well as difficulties in the documentation and designing any universal platform, the global perspective of these diseases remains unclear.
AREAS COVERED: Published and unpublished studies from January 1981 to June 2020 were systematically reviewed on PubMed, Web of Science and Scopus. Additionally, the reference list of all studies was hand-searched for additional studies. This effort identified a total of 104614 registered patients and suggests identification of at least 10590 additional PID patients, mainly from countries located in Asia and Africa. Molecular defects in genes known to cause PID were identified and reported in 13852 (13.2% of all registered) patients.
EXPERT OPINION: Although these data suggest some progress in the identification and documentation of PID patients worldwide, achieving the basic requirement for the global PID burden estimation and registration of undiagnosed patients will require more reinforcement of the progress, involving both improved diagnostic facilities and neonatal screening.

Matched MeSH terms: Infant, Newborn
Towards a new role for the infant food industry

Abraham SCE

Family Practitioner, 1977;2:61-63.

Matched MeSH terms: Infant, Newborn
Fixed presurgical orthopaedics for bilateral cleft lip and palate

Abu-Rub N, Samsudin AR, Abdullah AB, Abdullah N

Aust Orthod J, 2005 May;21(1):39-43.
PMID: 16433080

Presurgical orthopaedics has been employed since the 1950s as an adjunctive neonatal therapy for the correction of cleft lip and palate. It is accepted that presurgical orthopaedic plates facilitate lip repair and balanced orofacial growth.

Matched MeSH terms: Infant, Newborn
Biotin-thiamine responsive basal ganglia disease: a retrospective review of the clinical, radiological and molecular findings of cases in Kuwait with novel variants

Aburezq M, Alahmad A, Alsafi R, Al-Tawari A, Ramadan D, Shafik M, et al.

Orphanet J Rare Dis, 2023 Sep 05;18(1):271.
PMID: 37670342 DOI: 10.1186/s13023-023-02888-y

BACKGROUND: Biotin-thiamine-responsive basal ganglia disease (BTBGD) is a rare autosomal recessive neurometabolic disorder that is caused by biallelic pathogenic SLC19A3 variants and is characterized by subacute encephalopathy associated with confusion, convulsions, dysphagia, dysarthria, or other neurological manifestations.
METHODS: A retrospective review of the data registry in Kuwait Medical Genetics Center for all cases diagnosed clinically and radiographically and confirmed genetically with BTBGD.
RESULTS: Twenty one cases from 13 different families were diagnosed with BTBGD in Kuwait. Most cases (86%) presented with confusion, dystonia, convulsions, or dysarthria, while three individuals were diagnosed pre-symptomatically during familial targeted genetic screening. Symptoms resolved completely within 2-week of treatment in two-thirds of the symptomatic cases but progressed in six of them to a variety of severe symptoms including severe cogwheel rigidity, dystonia and quadriparesis due to delayed presentation and management. Neuroradiological findings of the symptomatic cases revealed bilateral central changes in the basal ganglia. Two novel homozygous missense SLC19A3 variants were detected in a Kuwaiti and a Jordanian individuals, in addition to the previously reported Saudi founder homozygous variant, c.1264A > G; p.(Thr422Ala) in the remaining cases. Age of diagnosis ranged from newborn to 32 years, with a median age of 2-3 years. All cases are still alive receiving high doses of biotin and thiamine.
CONCLUSION: This is the first study reporting the phenotypic and genotypic spectrum of 21 individuals with BTBGD in Kuwait and describing two novel SLC19A3 variants. BTBGD is a treatable neurometabolic disease that requires early recognition and treatment initiation. This study highlights the importance of performing targeted molecular testing of the founder variant in patients presenting with acute encephalopathy in the region.

Matched MeSH terms: Infant, Newborn
Case report: torsion of a gravid horn of a didelphic uterus

Achanna S, Monga D, Hassan MS

J Obstet Gynaecol Res, 1996 Apr;22(2):107-9.
PMID: 8697337

Acute abdominal pain during pregnancy presents a dilemma as signs and symptoms are often modified. Abdominal massage by traditional birth attendants (TBAs') during early labour is a common practice in the rural population, as it is perceived to give a soothening effect to the labouring mother. Many instances of abruptio placentae were reported in the past by this procedure, and in this case, the clinical picture presented as an abruptio placenta. Malpresentation and failure to progress were the indications for caesarean section despite the fetal demise. Severe post partum haemorrhage and failure to contract despite massive oxytocics resulted in the hysterectomy of the gravid horn, leaving the other horn intact.

Matched MeSH terms: Infant, Newborn
Fulltext Maternal, neonatal, and child health in southeast Asia: towards greater regional collaboration

Acuin CS, Khor GL, Liabsuetrakul T, Achadi EL, Htay TT, Firestone R, et al.

Lancet, 2011 Feb 05;377(9764):516-25.
PMID: 21269675 DOI: 10.1016/S0140-6736(10)62049-1

Although maternal and child mortality are on the decline in southeast Asia, there are still major disparities, and greater equity is key to achieve the Millennium Development Goals. We used comparable cross-national data sources to document mortality trends from 1990 to 2008 and to assess major causes of maternal and child deaths. We present inequalities in intervention coverage by two common measures of wealth quintiles and rural or urban status. Case studies of reduction in mortality in Thailand and Indonesia indicate the varying extents of success and point to some factors that accelerate progress. We developed a Lives Saved Tool analysis for the region and for country subgroups to estimate deaths averted by cause and intervention. We identified three major patterns of maternal and child mortality reduction: early, rapid downward trends (Brunei, Singapore, Malaysia, and Thailand); initially high declines (sustained by Vietnam but faltering in the Philippines and Indonesia); and high initial rates with a downward trend (Laos, Cambodia, and Myanmar). Economic development seems to provide an important context that should be coupled with broader health-system interventions. Increasing coverage and consideration of the health-system context is needed, and regional support from the Association of Southeast Asian Nations can provide increased policy support to achieve maternal, neonatal, and child health goals.

Matched MeSH terms: Infant, Newborn
Fulltext Coarctation of aorta repair at the National Heart Institute (1983-1994)

Adeeb SMSJ, Leman H, Sallehuddin A, Yakub A, Awang Y, Alwi M

Med J Malaysia, 2004 Mar;59(1):11-4.
PMID: 15535329

This retrospective study illustrates our approach to this problem over the years, from performing subclavian flap aortoplasty initially to the more accepted procedure now, which is resection and end-to-end anastomosis. Coarctation of aorta in our population is seen in a varying age groups and are also associated with other cardiac anomalies including both acyanotic and cyanotic congenital cardiac defects. Therefore a wide variety of surgical procedures were performed including resection of the coarcted segment and end-to-end anastomosis, subclavian flap aortoplasty, patch aortoplasty and synthetic tube graft interposition. Subclavian flap aortoplasty is not widely practised anymore in favour of resection with end-to-end anastomosis. Fifty four point four percent of patients had isolated coarctation, 10.5% had associated valvular defects, 28.1% had other simple congenital defects and 7.0% had associated complex cyanotic congenital defects. Perioperative mortality was 5.26% and is correlated with the younger age of patients at time of surgery and severity of cardiac failure at time of presentation. We did not see any difference in mortality for patients with complex congenital disease or between the different surgical procedures. However, we did find that in the early period when resection with end-to-end anastomosis was performed, there was a significantly higher incidence of morbidities.

Matched MeSH terms: Infant, Newborn
Fulltext Diagnosis of thanatophoric dwarfism in utero

Afzal M, Singh J, Ong SK

Med J Malaysia, 1983 Mar;38(1):47-50.
PMID: 6633336

Thanatophoric dwarfism is a severe form of neonatal shortlimbed skeletal dysplasia. Most infants are stillborn or die soon after birth. This disorder has well defined radiological features which distinguish it from the other forms of neonatal dwarfism. We report two cases where short limbs were detected on sonography and a diagnosis was made on antenatal radiographs and fetography.

Matched MeSH terms: Infant, Newborn
Fulltext Lethal neonatal dwarfism: a case of achondrogenesis

Afzal MK, Choo KE

Med J Malaysia, 1980 Sep;35(1):64-7.
PMID: 7254002

Achondrogenesis is a lethal neonatal chondrodysplasia with extreme micromelia and marked discrepancy between the relatively large head and the decreased trunk length. The affected neonates are usually delivered prematurely, and are stillborn or die soon after birth. Polyhydramnios is frequently present. It is an inherited autosomal recessive disease. The radiographic features are diagnostic.

Matched MeSH terms: Infant, Newborn
Fulltext Outcome of a newborn hearing screening program in a tertiary hospital in Malaysia: the first five years

Ahmad A, Mohamad I, Mansor S, Daud MK, Sidek D

Ann Saudi Med, 2011 Jan-Feb;31(1):24-8.
PMID: 21245595 DOI: 10.4103/0256-4947.75774

Universal newborn hearing screening (UNHS) was started in the Hospital Universiti Sains Malaysia (HUSM) in January 2003. To comply with international standards, we determined the outcome of the newborn hearing screening program for the first 5 years of its implementation, from January 2003 to December 2007.

Matched MeSH terms: Infant, Newborn
Characteristics of community- and hospital-acquired meticillin-resistant Staphylococcus aureus strains carrying SCCmec type IV isolated in Malaysia

Ahmad N, Ruzan IN, Abd Ghani MK, Hussin A, Nawi S, Aziz MN, et al.

J Med Microbiol, 2009 Sep;58(Pt 9):1213-1218.
PMID: 19528158 DOI: 10.1099/jmm.0.011353-0

Community-acquired meticillin-resistant Staphylococcus aureus (CA-MRSA) occurring among hospital isolates in Malaysia has not been reported previously. As CA-MRSA reported worldwide has been shown to carry SCCmec types IV and V, the aim of this study was to determine the SCCmec types of MRSA strains collected in Malaysia from November 2006 to June 2008. From a total of 628 MRSA isolates, 20 were SCCmec type IV, whilst the rest were type III. Further characterization of SCCmec type IV strains revealed 11 sequence types (STs), including ST22, with the majority being ST30/Panton-Valentine leukocidin positive. Eight out of nine CA-MRSA were ST30, one was ST80, and all were sensitive to co-trimoxazole and gentamicin. Five new STs designated ST1284, ST1285, ST1286, ST1287 and ST1288 were discovered, suggesting the emergence of novel clones of MRSA circulating in Malaysian hospitals. The discovery of the ST22 strain is a cause for concern because of its ability to replace existing predominant clones in certain geographical regions.

Matched MeSH terms: Infant, Newborn
Fulltext Aural myiasis in a neonate in peninsular Malaysia

Ahmad NW, Ismail A, Jeffery J, Ibrahim S, Hadi AA, Ibrahim MN, et al.

Parasit Vectors, 2009;2(1):63.
PMID: 20003466 DOI: 10.1186/1756-3305-2-63

Myiasis is a pathological condition in humans and animals caused by various species of dipterous larvae. Myiasis which occurs in a newborn baby is referred as neonatal myiasis. It is a rare condition and there are only a few reports to date. A case of neonatal aural myiasis in a two day old infant is reported in this paper.

Matched MeSH terms: Infant, Newborn
Fulltext Prenatal ultrasound heating impacts on fluctuations in haematological analysis of Oryctolagus cuniculus

Ahmad Zaiki FW, Md Dom S, Abdul Razak HR, Hassan HF

Quant Imaging Med Surg, 2013 Oct;3(5):262-8.
PMID: 24273744 DOI: 10.3978/j.issn.2223-4292.2013.10.05

Prenatal Ultrasound (US) is commonly used as a routine procedure on pregnant women. It is generally perceived as a safe procedure due to the use of non-ionizing radiation. However, the neurotoxicity of diagnostic prenatal US was detected to have a correlation with high susceptibility to early developing fetus. This research involved in vivo experimental model by using 3(rd) trimester pregnant Oryctolagus cuniculus and exposing them to US exposures for 30, 60, and 90 minutes at their gestational day (GD) 28-29. The output power and intensities, spatial peak temporal average intensity (ISPTA) of US were varied from 0.4 to 0.7 W and 0.13 to 0.19 W/cm(2) respectively were tested initially in free-field, water. Haematological analysis was carried out to detect any changes in blood constituents. Statistically significant differences were detected in red blood cell (RBC) count (P<0.001), haemoglobin (Hb) concentration (P<0.001) and also platelet (PLT) count (P<0.001) in newborn of Oryctolagus cuniculus. These findings indicate the possibility of US heating in causing defects on studied animal.

Matched MeSH terms: Infant, Newborn
Fulltext A Prospective Cohort Study of Factors Associated with Empiric Antibiotic De-escalation in Neonates Suspected with Early Onset Sepsis (EOS)

Ain Ibrahim N, Makmor Bakry M, Mohd Tahir NA, Mohd Zaini NR, Mohamed Shah N

Paediatr Drugs, 2020 Jun;22(3):321-330.
PMID: 32185682 DOI: 10.1007/s40272-020-00388-1

BACKGROUND: Prolonged empiric antibiotic use, resulting from diagnostic uncertainties, in suspected early onset sepsis (EOS) cases constitutes a significant problem. Unnecessary antibiotic use increases the risk of antibiotic resistance. Furthermore, prolonged antibiotic use increases the risk of mortality and morbidity in neonates. Proactive measures including empiric antibiotic de-escalation are crucial to overcome these problems.
METHODS: This was a prospective cohort study conducted in the neonatal intensive care units of two public hospitals in Malaysia. Neonates with a gestational age greater than 34 weeks who were started on empiric antibiotics within 72 h of life were screened. The data were then stratified according to de-escalation and non-de-escalation practices, where de-escalation practice was defined as narrowing down or discontinuation of empiric antibiotic within 72 h of treatment.
RESULTS: A total of 1045 neonates were screened, and 429 were included. The neonates were then divided based on de-escalation (n = 207) and non-de-escalation (n = 222) practices. Neonates under non-de-escalation practices showed significantly longer durations of antibiotic use compared to those under de-escalation practices (p

Matched MeSH terms: Infant, Newborn
Fulltext Complete molecular characterisation of glucose-6-phosphate dehydrogenase (G6PD) deficiency in a group of Malaysian Chinese neonates

Ainoon O, Boo NY, Yu YH, Cheong SK, Hamidah HN, Lim JH

Malays J Pathol, 2004 Dec;26(2):89-98.
PMID: 16329560

We performed DNA analysis on cord blood samples of 128 Chinese male neonates diagnosed as G6PD deficiency in Hospital Universiti Kebangsaan Malaysia by a combination PCR-restriction enzyme digest technique, Single Stranded Conformation Polymorphism analysis and DNA sequencing. We found 10 different G6PD-deficient mutations exist. The two commonest alleles were G6PD Canton 1376 G>T (42.3%) and Kaiping 1388 G>A (39.4%) followed by G6PD Gaohe 592 G>A (7.0%), Chinese-5 1024 C>T, Nankang 517 T>C (1.5%), Mahidol 487 G>A (1.6%), Chatham 1003 G>T (0.8%), Union 1360 C>T (0.8%), Viangchan 871 G>A (0.8%) and Quing Yang 392 G>T (0.8%). Sixty eight percent (88/125) neonates in this study had neonatal jaundice and 29.7% developed hyperbilirubinemia >250 micromol/l. The incidence of hyperbilirubinemia >250 micromol/l was higher in G6PD Kaiping (43.8%) than G6PD Canton (22%) (p< 0.05). There was no significant difference in the incidence of neonatal jaundice, mean serum bilirubin, mean age for peak serum bilirubin, percentage of babies requiring phototherapy and mean duration of phototherapy between the two major variants. None of the 88 neonates required exchange transfusion. In conclusion we have completely characterized the molecular defects of a group of Chinese G6PD deficiency in Malaysia. The mutation distribution reflects the original genetic pool and limited ethnic admixture with indigenous Malays.

Matched MeSH terms: Infant, Newborn
Glucose-6-phosphate dehydrogenase (G6PD) variants in Malaysian Chinese

Ainoon O, Joyce J, Boo NY, Cheong SK, Zainal ZA, Hamidah NH

Hum Mutat, 1999 Oct;14(4):352.
PMID: 10502785 DOI: 10.1002/(SICI)1098-1004(199910)14:4<352::AID-HUMU1

We screened 38 G6PD-deficient male Chinese neonates for known G6PD mutations using established PCR-based techniques. We found 50.0% (19 of 38) were mutation 1376G>T, 34.2% (13 of 38) were mutation 1388G>A, 5.2% (2 of 38 ) were mutation 95A>G and 2.2% (1 of 38) was mutation 1024C>T. In 7% (3 of 38) of the cases the mutations remained uncharacterised. Sixty three percent (24 of 38) of the G6PD deficient neonates had neonatal jaundice with 28.9 % (11 of 38) developing moderate to severe hyperbilirubinemia. The group of neonates with 1388 mutation showed the highest incidence of moderate to severe hyperbilirubinemia requiring phototherapy and/or exchange transfusion respectively. Majority (70%) of the G6PD deficient neonates showed severe enzyme deficiency. However, there was no meaningful association between the level of enzyme activity and the severity of neonatal jaundice. In summary, four mutations account for more than 90% of the G6PD deficiency cases among the Chinese in Malaysia and the pattern of distribution of the molecular variants is similar to those found among the Chinese in Taiwan and southern mainland China. Our findings also suggest the possible association of nt 1388 mutation with severe neonatal jaundice.

Matched MeSH terms: Infant, Newborn

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