Beta-thalassemia is the most-common genetic disorder of hemoglobin synthesis in Malaysia, and about 4.5% of the population are heterozygous carriers of the disorder. Prenatal diagnosis was performed for 96 couples using the Amplification Refractory Mutation System and Gap-Polymerase Chain Reaction. We identified 17 beta-globin defects-initiation codon for translation (T-G), -29 (A-G), -28 (A-G), CAP +1 (A-C), CD 8/9 (+G), CD 15 (G-A), CD 17 (A-T), CD 19 (A-G), Hb E (G-A), IVS1-1 (G-T), IVS1-5 (G-C), CD 41/42 (-CTTT), CD 71-72 (+A), IVS2-654 (CT), poly A(A-G), 100-kb Ggamma(Agammadeltabeta) degrees and 45-kb Filipino deletions. The 192 beta-alleles studied comprised Chinese (151 patients), Malay (21), Orang Asli from East Malaysia (15), Filipino (1), Indian (1), Indonesian Chinese (2), and Thai (1). In the Chinese, 2 beta-globin defects at CD 41/42 and IVS2-654 were responsible for 74% of beta-thalassemia. beta-mutations at CD 19, IVS1-1 (G-T), IVS1-5, poly A, and hemoglobin E caused 76% of the hemoglobin disorders in the Malays. The Filipino 45-kb deletion caused 73.3% of bthalassemia in the Orang Asli. Using genomic sequencing, the rare Chinese beta-mutation at CD 43 (G-T) was confirmed in 2 Chinese, and the Mediterranean mutation IVS1-1 (G-A) was observed in a Malay beta-thalassemia carrier. The beta-globin mutations confirmed in this prenatal diagnosis study were heterogenous and 65 (68%) couples showed a different globin defect from each other. The use of specific molecular protocols has allowed rapid and successful prenatal diagnosis of beta-thalassemia in Malaysia.

He has an experience of 51 years in the field of genetics and added valuable contribution in the field of pediatric genetics and community health in India. He handled approximately 2000 cases with expertise in Fetal Medicine and Reproductive Genetics. Current areas of interest and/or active clinical practices: Management of fetal medicine and reproductive genetics; Dysmorphology; Genetic counseling and prenatal diagnosis using molecular, cytogenetic and biochemical techniques.

of a patient with diabetes. Each institution will have to decide how they want to use expensive resources, such as specialty services. The more complex the medical needs of the institution's patients, the more involvement of specialty services will be required.As Dr Mooney suggested, many things are required to provide quality pain management: Education needs to be provided at multiple levels for nursing, physician, and other healthcare providers; education has to be ongoing as house staff and other trainees come and go; standardized and evidence-based treatment regimes need to be developed; quality of care needs to be regularly assessed; protocols should be reviewed and adjusted to reflect new knowledge. In a busy institution, these tasks should not fall to a single individual. We advocate a team approach whereby systems are put in place that ensure adequate pain management for all children at all times. The idea of a single individual as an advocate for pain management is not a new one. It is at the origin of most pediatric Pain Services. Developing and maintaining high standards of care in pediatric pain management was just more than a single individual could achieve in the long run. Furthermore, changing the culture of an organization should not rely on one person to champion the cause.'Selective bronchial blockade with Fogarty catheter in a child with acquired bronchobiliary fistula' SIR-Bronchobiliary fistula is a rare abnormal communication between bronchial and biliary tract characterized by presence of bileptysis (1).This aberrant interconnection develops subsequent to liver disease but it may be present as a congenital malformation. Patients may present with nonspecific symptoms like recurrent chest infections, fever, and jaundice. Thoracotomy and removal of the unhealthy segments with repair of the diaphragmatic defect is the definitive treatment (2,3). We present the anesthetic management of a three-year female child with acquired bronchobiliary fistula scheduled for right thoracotomy.A 3-year female child (weight, 10 kg; height, 95 cm) was admitted with cough, dyspnea, fever, and bilious vomiting for 1 week. She had severe abdominal pain associated with fever, one year back, and was diagnosed to have liver abscess (5 9 5 9 4.5 cm). Surgical drainage of the liver abscess was performed immediately. Few months after the surgery, she developed multiple bouts of lower respiratory tract infections, which were treated with antibiotics. Breath sounds were decreased in right infrascapular region. Bronchial breath sounds and rales were found in right infrascapular area. Opacification of right lower lung fields was found in chest radiograph. Ultrasonography showed right-sided pleural effusion and hyperechoic regions in right lobe of the liver. Computerized tomography revealed consolidation in right lower lobe of the lung and a transdiaphragmatic tubular tract connecting right lower lobe of lung to left lobe of liver suggestive of bronchobiliary fistula. Extension of tracer into right bronchus with a patent bili...

Objective-To analyse the effect of a fetal surveillance unit, which undertakes a wide range of maternal and fetal tests on an outpatient or inpatient basis, on the number and length of antenatal hospital admissions.

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