Displaying publications 161 - 180 of 336 in total

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  1. Fulltext Pulmonary Vein Stenosis: A Rare Disease with a Global Reach
    Schramm J, Sivalingam S, Moreno GE, Thanh DQL, Gauvreau K, Doherty-Schmeck K, et al.
    Children (Basel), 2021 Mar 06;8(3).
    PMID: 33800765 DOI: 10.3390/children8030198
    Pulmonary vein stenosis (PVS) is a rare, but high mortality and resource intensive disease caused by mechanical obstruction or intraluminal myofibroproliferation, which can be post-surgical or idiopathic. There are increasing options for management including medications, cardiac catheterization procedures, and surgery. We queried the International Quality Improvement Collaborative for Congenital Heart Disease (IQIC) database for cases of PVS and described the cohort including additional congenital lesions and surgeries as well as infectious and mortality outcomes. IQIC is a quality improvement project in low-middle-income countries with the goal of reducing mortality after congenital heart surgery. Three cases were described in detail with relevant images. We identified 57 cases of PVS surgery, with similar mortality to higher income countries. PVS should be recognized as a global disease. More research and collaboration are needed to understand the disease, treatments, and outcomes, and to devise treatment approaches for low resource environments.
    Matched MeSH terms: Heart Defects, Congenital
  2. Fulltext Unresolved tachypnoea: a presentation of congenital cor-triatriatum
    Fahisham Taib, Nur Atiqah Abdul Rahman
    Malaysian Journal of Paediatrics and Child Health, 2019;25(2):23-27.
    MyJurnal
    Cor-triatriatum is a rare cardiac anomaly. In literature, majority case reports on the condition focused on its late presentation in adulthood. It can be easily corrected by surgical intervention to avoid pulmonary congestion and subsequent pulmonary hypertension. We report a rare case of cor-triatriatum with severe pulmonary hypertension in a 7-week-old baby who presented with persistent tachypnoea.
    Matched MeSH terms: Heart Defects, Congenital
  3. Fulltext Cavernous lymphangioma of the digits: a rare cause of macrodactyly
    Leong, J.F., Levin, K.B., Rajkumar V., Abdullah, S., Jamari, S.
    Medicine & Health, 2019;14(2):261-265.
    MyJurnal
    Cavernous lymphangioma is a congenital malformation of lymphatic system causing dilated lymphatic sinuses that involve the skin and subcutaneous tissues. This was an interesting case of dystrophic macrodactyly of the left ring and little finger in a 18-month-old girl who presented with swollen and sausage like fingers deformity which turned out to be an isolated cavernous lymphangioma. This tumor, although rare to occur in the extremeties, must be differentiated from other congenital vascular lesions of the hand that include arteriovenous malformations and hemangiomas. Diagnosis should be solely based on histopathological analysis of the excised tissue mass. Surgical excision is usually necessary for satisfactory functional and cosmetic outcome
    Matched MeSH terms: Limb Deformities, Congenital
  4. Quantitative and qualitative comparison of low- and high-cost 3D-printed heart models
    Lau I, Wong YH, Yeong CH, Abdul Aziz YF, Md Sari NA, Hashim SA, et al.
    Quant Imaging Med Surg, 2019 Jan;9(1):107-114.
    PMID: 30788252 DOI: 10.21037/qims.2019.01.02
    Current visualization techniques of complex congenital heart disease (CHD) are unable to provide comprehensive visualization of the anomalous cardiac anatomy as the medical datasets can essentially only be viewed from a flat, two-dimensional (2D) screen. Three-dimensional (3D) printing has therefore been used to replicate patient-specific hearts in 3D views based on medical imaging datasets. This technique has been shown to have a positive impact on the preoperative planning of corrective surgery, patient-doctor communication, and the learning experience of medical students. However, 3D printing is often costly, and this impedes the routine application of this technology in clinical practice. This technical note aims to investigate whether reducing 3D printing costs can have any impact on the clinical value of the 3D-printed heart models. Low-cost and a high-cost 3D-printed models based on a selected case of CHD were generated with materials of differing cost. Quantitative assessment of dimensional accuracy of the cardiac anatomy and pathology was compared between the 3D-printed models and the original cardiac computed tomography (CT) images with excellent correlation (r=0.99). Qualitative evaluation of model usefulness showed no difference between the two models in medical applications.
    Matched MeSH terms: Heart Defects, Congenital
  5. Impact of ductal stenting on pulmonary artery reconstruction in patients with duct-dependent congenital heart diseases-an institutional experience
    Haranal M, Mood MC, Leong MC, Febrianti Z, Abdul Latiff H, Samion H, et al.
    Interact Cardiovasc Thorac Surg, 2020 08 01;31(2):221-227.
    PMID: 32437520 DOI: 10.1093/icvts/ivaa069
    OBJECTIVES: This study aims to review our institutional experience of ductal stenting (DS) on the growth of pulmonary arteries (PAs) and surgical outcomes of PA reconstruction in this subset of patients.

    METHODS: This is a retrospective study done in neonates and infants up to 3 months of age with duct-dependent pulmonary circulation who underwent DS from January 2014 to December 2015. Post-stenting PA growth, surgical outcomes of PA reconstruction, post-surgical re-interventions, morbidity and mortality were analysed.

    RESULTS: During the study period, 46 patients underwent successful DS, of whom 38 underwent presurgery catheterization and definite surgery. There was significant growth of PAs in these patients. Biventricular repair was done in 31 patients while 7 had univentricular palliation. Left PA augmentation was required in 13 patients, and 10 required central PA augmentation during surgery. The mean follow-up period post-surgery was 4.5 ± 1.5 years. No significant postoperative complications were seen. No early or follow-up post-surgery mortality was seen. Four patients required re-interventions in the form of left PA stenting based on the echocardiography or computed tomography evidence of significant stenosis.

    CONCLUSIONS: DS provides good short-term palliation and the growth of PAs. However, a significant number of stented patients require reparative procedure on PAs at the time of surgical intervention. Acquired changes in the PAs following DS may be the reason for reintervention following PA reconstruction.

    Matched MeSH terms: Heart Defects, Congenital/mortality; Heart Defects, Congenital/physiopathology; Heart Defects, Congenital/surgery*
  6. An accessory mitral valve leaflet causing left ventricular outflow tract obstruction and associated with severe aortic incompetence
    Tamin SS, Dillon J, Aizan K, Kadiman S, Latiff HA
    Echocardiography, 2012 Feb;29(2):E34-8.
    PMID: 22044509 DOI: 10.1111/j.1540-8175.2011.01543.x
    This case report describes a 20-year-old woman with Turner's syndrome who presented with reduced effort tolerance limited by dyspnea. She had previously been on pediatric cardiology follow-up for congenital subvalvular aortic stenosis first diagnosed at age 7. Unfortunately she defaulted after two visits before any intervention could be done. Transthoracic echocardiography demonstrated severe aortic incompetence (AI) with a membrane-like structure in the left ventricular outflow tract (LVOT). The mean pressure gradient across the LVOT on continuous wave Doppler was 41 mmHg. The membranous interventricular septum appeared aneurysmal and it was observed that the "subaortic membrane" had a connection to the anterolateral papillary muscle via a strand of chordal tissue. Further images were captured using two-dimensional and three-dimensional transthoracic and transesophageal echocardiography (iE33, Philips Medical Systems, Andover, MA, USA). After a review of the literature it was concluded that this appeared to be an accessory mitral valve (AMV) leaflet causing LVOT obstruction associated with AI. AMV tissue is a rare congenital malformation causing LVOT obstruction. Because it is so unusual, it may not be immediately recognizable even in a high volume echocardiography laboratory. The clue which helped with the diagnosis was the strand of chordal tissue which connected the mass to the papillary muscle. This anomaly is often associated with LVOT obstruction.
    Matched MeSH terms: Heart Defects, Congenital/complications*; Heart Defects, Congenital/surgery; Heart Defects, Congenital/ultrasonography
  7. Fulltext Congenital insensitivity to pain with recurrent septic arthritis of the left knee
    Zamzuri Z, Goh KL, Aminuddin CA, Mohamed Azril MA, Shukrimi A
    International Medical Journal Malaysia, 2011;10(1):51-52.
    MyJurnal
    A 15-year–old Malay male with congenital insensitivity to pain presented with recurrent septic arthritis of the left knee complicated by osteomylitis. Repeated arthrotomy and wound debridement was done, however, the condition was not resolved. Amputation was suggested as the best method of treatment to eradicate the infection.
    Matched MeSH terms: Pain Insensitivity, Congenital
  8. Fulltext Physical and Clinical Evaluation of Hip Spica Cast applied with Three-slab Technique using Fibreglass Material
    Bitar KM, Ferdhany ME, Ashraf EI, Saw A
    Malays Orthop J, 2016 Nov;10(3):17-20.
    PMID: 28553442 MyJurnal DOI: 10.5704/MOJ.1611.008
    Introduction: Hip spica casting is an important component of treatment for developmental dysplasia of the hip (DDH) and popular treatment method for femur fractures in children. Breakage at the hip region is a relatively common problem of this cast. We have developed a three-slab technique of hip spica application using fibreglass as the cast material. The purpose of this review was to evaluate the physical durability of the spica cast and skin complications with its use. Methodology: A retrospective review of children with various conditions requiring hip spica immobilisation which was applied using our method. Study duration was from 1st of January 2014 until 31st December 2015. Our main outcomes were cast breakage and skin complications. For children with hip instability, the first cast would be changed after one month, and the second cast about two months later. Results: Twenty-one children were included, with an average age of 2.2 years. The most common indication for spica immobilisation was developmental dysplasia of the hip. One child had skin irritation after spica application. No spica breakage was noted. Conclusion: This study showed that the three-slab method of hip spica cast application using fibreglass material was durable and safe with low risk of skin complications.
    Matched MeSH terms: Hip Dislocation, Congenital
  9. Fulltext Treatment of Developmental Dysplasia of the Hip: Short and Mid-term Outcome
    Malays Orthop J, 2011;5(1):17-20.
    MyJurnal
    Objective: To review the presentation and outcome of developmental dysplasia of the hip (DDH) cases in the last 6 years. Methodology:We retrospectively reviewed the presentation as well as short and mid-term outcome of 17children (18 hips) with DDH seen and treated from 2004 to 2010. Treatment was either splinting with Pavlik harness,closed or open reduction depending on age at presentation and the individual needs of each patient. Results:Theaverage age at presentation was 31.1 months, and the male to female ratio was 1:4.6. Six patients (35%) presented early(less than 6 months old) while 7 patients (41%) presented between 6 -24 months. In these 2 groups splinting or closed reduction under anaesthesia was initially utilized for treatment depending on age, and open reduction was use dafter failure of closed reduction and for those presented late.Average age at the time of closed reduction was 11 months,and 6 years for those underwent open reduction. The average period of follow up for all patients was 29.4 months. The final outcome was successful in 12 hips (66%) in 11 patients.Ten of these presented at less than 2 years old, in the other 6 hips in which there was failure, 50% presented after 3 years of age. Conclusion:Late presentation of DDH is still common in Malaysia, which necessitates more complex management and more frequent occurrences of unfavourable results.
    Matched MeSH terms: Hip Dislocation, Congenital
  10. Fulltext Undiagnosed traumatic diaphragmatic hernia in two cats
    Lau , S.F.
    Jurnal Veterinar Malaysia, 2016;28(1):12-15.
    MyJurnal
    Two cases of diaphragmatic hernia in cats caused by road traffic accident were undiagnosed during the initial presentation. Both cats showed no evidence of respiratory distress and clinicians did not notice the diaphragmatic hernia on thoracic radiographs. Later on, both cats showed signs of dyspnea and diaphragmatic hernia was diagnosed with repeated thoracic radiograph and ultrasound. Diaphragmatic hernia is rather common in veterinary practice, however, its diagnosis can be clinically challenging. This case report highlights the importance to re-evaluate and monitor the patient closely after the traffic accident. Repeated radiograph, different diagnostic modalities and techniques such as ultrasound, computed tomography and positive contrast peritoneagraphy should be used in order to diagnose the diaphragmatic hernia conclusively.
    Matched MeSH terms: Hernias, Diaphragmatic, Congenital
  11. Fulltext A case report of Goltz syndrome: a newborn with digital anomalies and skin lesions
    Lee, Von Yen, Leow, Poy Lee
    Malaysian Journal of Paediatrics and Child Health, 2016;22(101):0-0.
    MyJurnal
    Goltz syndrome or Focal Dermal Hypoplasia (FDH) is an uncommon genetically inherited disorder characterized by distinctive skin abnormalities and a wide variety of multisystem defects which was first described by Goltz (an American dermatologist) in 1962. About 200- 300 cases have been reported worldwide. FDH can be inherited in an X-linked dominant manner with in-utero lethality in males. Majority of the cases are sporadic with new mutations arising in the embryo and not inherited from a parent. Approximately 10% of cases occur in males; postzygotic somatic mosaicism accounts for the findings in these affected males. FDH is caused by abnormalities or mutations at the PORCN gene in the X chromosome. We report a case of FDH with characteristic skin lesions as well as multiple digital anomalies - oligodactyly, syndactyly and ectrodactyly.(Copied from article)
    Matched MeSH terms: Limb Deformities, Congenital
  12. Anatomical variants of the superficial temporal artery in patients with microtia: a pilot descriptive study
    Imran FH, Yong CK, Das S, Huei YL
    Anat Cell Biol, 2016 Dec;49(4):273-280.
    PMID: 28127502 DOI: 10.5115/acb.2016.49.4.273
    Superficial temporal artery (STA) based pedicled fascial flap plays a pivotal role in ear reconstruction for microtia patients. There is paucity of literature on the anatomy of the STA in microtia patients. The present study aimed to describe any possible anatomical variations seen in the STA of patients afflicted with microtia. Pre-operative carotid computer tomographic angiography images of patients under the microtia database of Plastic and Reconstructive Surgery Unit at a tertiary medical centre were selected and 3-dimensionally reconstructed. Measurements were made on the 3D reconstructed computed tomographic angiography images of the STA on both the sides of the microtic ear and the non-microtic ear to assess its various anatomical parameters. We managed to obtain a total of 39 computed tomographic angiography images of STAs for analysis. There was a significant difference in the number of main branches of STA between the two groups (P=0.006). The proportion of ears with 2 main branches was higher in the non-microtia group (89.5%) compared to the microtia group (45.0%). A significant difference was found in the STA diameter between the two groups (P=0.012). The mean diameter of STA in the non-microtia group was larger by 0.4 mm. Furthermore, the median angle of STA was larger on the side of the non-microtic ears compared to that of microtic ears by 24.5°, with a P-value of 0.011. The results of the study may be of clinical importance while planning and performing ear reconstructive surgeries using STA based pedicled fascial flaps.
    Matched MeSH terms: Congenital Microtia
  13. Projection the long-term ungauged rainfall using integrated Statistical Downscaling Model and Geographic Information System (SDSM-GIS) model
    Tukimat NNA, Ahmad Syukri NA, Malek MA
    Heliyon, 2019 Sep;5(9):e02456.
    PMID: 31687558 DOI: 10.1016/j.heliyon.2019.e02456
    An accuracy in the hydrological modelling will be affected when having limited data sources especially at ungauged areas. Due to this matter, it will not receiving any significant attention especially on the potential hydrologic extremes. Thus, the objective was to analyse the accuracy of the long-term projected rainfall at ungauged rainfall station using integrated Statistical Downscaling Model and Geographic Information System (SDSM-GIS) model. The SDSM was used as a climate agent to predict the changes of the climate trend in Δ2030s by gauged and ungauged stations. There were five predictors set have been selected to form the local climate at the region which provided by NCEP (validated) and CanESM2-RCP4.5 (projected). According to the statistical analyses, the SDSM was controlled to produce reliable validated results with lesser %MAE (<23%) and higher R. The projected rainfall was suspected to decrease 14% in Δ2030s. All the RCPs agreed the long term rainfall pattern was consistent to the historical with lower annual rainfall intensity. The RCP8.5 shows the least rainfall changes. These findings then used to compare the accuracy of monthly rainfall at control station (Stn 2). The GIS-Kriging method being as an interpolation agent was successfully to produce similar rainfall trend with the control station. The accuracy was estimated to reach 84%. Comparing between ungauged and gauged stations, the small %MAE in the projected monthly results between gauged and ungauged stations as a proved the integrated SDSM-GIS model can producing a reliable long-term rainfall generation at ungauged station.
    Matched MeSH terms: Hand Deformities, Congenital
  14. Fulltext Reading Performance and Compensatory Head Posture in Infantile Nystagmus after Null Zone Training
    Mohamad Fadzil N, Mohammed Z, Mohamad Shahimin M, Saliman NH
    Int J Environ Res Public Health, 2019 11 27;16(23).
    PMID: 31783492 DOI: 10.3390/ijerph16234728
    This study aimed to assess the visual function, reading performance, and compensatory head posture (CHP) in schoolchildren with infantile nystagmus. A total of 18 participants aged between 13 to 18 years old were divided into spectacle (n = 9) and null zone group (n = 9) based on their visual acuity. Visual acuity (LogMAR), contrast sensitivity (Pelli-Robson), reading time and rate (Tobii TX300), and CHP were measured pre and post null zone reading training. Participants in the null zone group received 10 sessions of training (5 weeks). Visual acuity and contrast sensitivity of participants in the spectacle and null zone groups were not significantly different pre and post training. Reading performance, i.e., reading time (z = -1.36; p = 0.173) and reading rate (z = -0.06; p = 0.953), of participants in the spectacle group was not significantly different after 5 weeks. Reading time (z = -2.55; p = 0.011) and reading rate (z = -2.07; p = 0.038 of participants in the null zone group showed significant improvement post training. After 5 weeks, CHP improved in six out of the nine participants (66.7%) of the null zone group and was unchanged in all participants in the spectacle group. Null zone reading training could benefit children with infantile nystagmus in improving reading performance and compensatory head posture.
    Matched MeSH terms: Nystagmus, Congenital
  15. Optic disc swelling in acromicric and geleophysic dysplasia
    Moey LH, Flaherty M, Zankl A
    Am J Med Genet A, 2019 09;179(9):1898-1901.
    PMID: 31228225 DOI: 10.1002/ajmg.a.61268
    Matched MeSH terms: Limb Deformities, Congenital/drug therapy*; Limb Deformities, Congenital/genetics; Limb Deformities, Congenital/pathology
  16. Mitral valve repair for congenital mitral valve disease: impact of the use of a biodegradable annuloplasty ring
    Yakub MA, Sivalingam S, Dillon J, Matsuhama M, Latiff HA, Ramli MF
    Ann Thorac Surg, 2015 Mar;99(3):884-90; discussion 890.
    PMID: 25579160 DOI: 10.1016/j.athoracsur.2014.09.016
    This study compares the midterm results of mitral valve repair using the biodegradable ring versus repair with non-ring annuloplasty techniques for congenital mitral valve disease in young children where it was not possible to use standard commercial rings.
    Matched MeSH terms: Heart Valve Diseases/congenital*
  17. Fulltext Macrodactyly and poliosis in tuberous sclerosis complex
    Sasongko TH, Ismail NF, Nik Mohd Ariff NA, Zabidi-Hussin ZA
    Jpn J Clin Oncol, 2014 Nov;44(11):1130.
    PMID: 25320338 DOI: 10.1093/jjco/hyu157
    Matched MeSH terms: Limb Deformities, Congenital/complications*
  18. Tibia vara and slipped upper femoral epiphysis: is there an association?
    Jamil K, Abdul Rashid AH, Ibrahim S
    J Pediatr Orthop B, 2015 Jan;24(1):46-9.
    PMID: 25192368 DOI: 10.1097/BPB.0000000000000101
    Tibia vara and slipped upper femoral epiphysis (SUFE) share a common risk factor, but their relationship is unclear. In both conditions, the patients are usually obese. To the best of our knowledge, there have been only two previous reports in the literature that have described the occurrence of tibia vara and SUFE in three patients. We report a child who was treated for bilateral tibia vara at the age of 3 years and subsequently developed a SUFE at the age of 13 years.
    Matched MeSH terms: Osteochondrosis/congenital*
  19. Fulltext Midgut malrotation with congenital peritoneal band: a rare cause of small bowel obstruction in adulthood
    Low SF, Ngiu CS, Sridharan R, Lee YL
    BMJ Case Rep, 2014;2014.
    PMID: 24717859 DOI: 10.1136/bcr-2013-202690
    Midgut malrotation commonly presents in the neonatal period, and rarely manifests its symptoms in adulthood with an estimated incidence of 0.2-0.5%. Nevertheless, the symptoms are non-specific with no strong pointers towards the clinical diagnosis. Consequently, the diagnosis is usually disclosed with imaging or surgery. We report a case of small bowel obstruction secondary to a congenital peritoneal band with underlying midgut malrotation in a 48-year-old man.
    Matched MeSH terms: Intestinal Volvulus/congenital
  20. Treatment of congenital chylothorax with octreotide in a hydropic preterm infant
    Choo YM, Lim CT
    Paediatr Int Child Health, 2013 May;33(2):116-9.
    PMID: 23925288 DOI: 10.1179/2046905512Y.0000000030
    Congenital chylothorax is rare in preterm infants. While most cases respond to conservative treatment, a few require surgery. Treatment with intravenous octreotide has been reported to have varying success in preterm infants. A fetus was diagnosed with bilateral hydrothoraces at 29 weeks of gestation and repeated thoracocentesis was performed antenatally to allow growth of the lungs. She was delivered electively at 32 weeks by caesarean section. Hydrops fetalis was confirmed and chest tubes were inserted bilaterally soon after birth. Intravenous octreotide was commenced on day 4 of life, titrated to a maximum of 10 μg/kg/hr for a total of 28 days. Hydrothorax resolved at day 30 and total parenteral nutrition was given for a total of 37 days. She was successfully extubated on day 40 of life and discharged on day 80. On review at 6 months of age, she was thriving and developing normally.
    Matched MeSH terms: Chylothorax/congenital*
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