Variant anatomy of the hepatic artery in adult Kenyans

Knowledge of the variant vascular anatomy ofthe subhepatic region is valuable to hepatobiliarysurgeons to limit operative complications due to unexpected bleeding. One hundred and two subhepatic regions were studied by gross dissection for the pattern of arterial blood supply. Measurements were made for the distance of hepatic bifurcation from the liver. The common hepatic artery (CHA) originated from the celiac trunk in 95.1% and from the superior mesenteric artery (SMA) in 4.9% of cases. The mean distance of bifurcation from the liver was 2.6cm and the CHA gave rise to 2 hepatic branches in 93.1% of cases. The right hepatic artery (RHA) passed anterior to the CHD in 48%, posterior in 41.2% and in 7.8% was related to the common bile duct (CBD). Accessory RHA and left hepatic arteries(LHA) were observed in 10.8% and 9.8% of the cases. There is wide variation in the pattern ofarterial supply to the liver and biliary apparatusin adult Kenyans. A comprehensive understandingand application of this aspect of surgical anatomy is essential for good results to be maintained and even improved when carrying out surgical procedures in this area (AU)

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Cardiovascular malformations in Omani Arab children with Down's syndrome.

BACKGROUND AND AIM: Reports from several countries suggest that the prevalence and spectrum of the congenital cardiac malformations seen in the setting of Down's syndrome vary in different ethnic groups and countries, and at different periods in the same country. Data on Arab children are lacking. Our study aimed to fill that void by ascertaining the pattern in Omani Arabs. METHODS: Prospective clinical and echocardiographic evaluation of consecutively recruited Omani children with Down's syndrome. RESULTS: We studied 110 children, aged from one day to 11 years, with a median of 2 months, detecting 76 cardiovascular malformations in 63 (57%) of them. Atrioventricular septal defect, the most prevalent lesion, accounted for 24 (32%) of the 76 defects. Next were atrial septal defects within the oval fossa (29%), patency of the arterial duct (17%), and ventricular septal defect (14%). There were only two cases of Fallot's tetralogy, one each of coarctation of the aorta and right-sided heart, respectively; and none of discordant ventriculo-arterial connections. CONCLUSION: The prevalence of cardiovascular malformations is high, at 57%, in our studied population of Arab children. Overall, 92% of the malformations are defects which are characterized by a left-to-right shunt, and the potential for pulmonary hypertension. These findings underscore the need for early detection and prompt, appropriate care. To achieve this, if feasible, all babies born with Down's syndrome should have echocardiography in the first month of life.

Racial differences in infant mortality attributable to birth defects in the United States, 1989-2002.

BACKGROUND: The objective is to study racial differences in infant mortality attributable to birth defects (IMBD) in the United States. METHODS: We analyzed 1989-1991 and 1995-2002 linked birth/death files for trends and racial differences in IMBD by selected categories of birth defects for infants of non-Hispanic white, non-Hispanic black, and Hispanic mothers. RESULTS: In 1989-2002, the IMBD rates declined. However, the decline in postneonatal mortality attributable to birth defects (PMBD) rate was significantly slower than that of overall postneonatal mortality. The adjusted rate ratio for non-Hispanic black and Hispanic versus non-Hispanic white for neonatal mortality attributable to birth defects (NMBD) remained unchanged from 1989-1991 through 2000-2002. For PMBD, it increased from 0.97 (95% confidence interval [CI], 0.90-1.13) in 1989-1991 to 1.12 (95% CI, 1.04-1.21) in 2001-2002 and from 1.08 (95% CI, 1.00-1.16) to 1.18 (95% CI, 1.10-1.27) for non-Hispanic black and Hispanic, respectively. Infant mortality due to cardiovascular and central nervous system defects were the main contributors to the increased racial disparities in PMBD rates. CONCLUSIONS: The disparity in PMBD between infants of non-Hispanic black and Hispanic mothers and infants of non-Hispanic white mothers increased significantly from 1989-1991 to 2000-2002. Further studies are needed to assess the extent to which delays in care or lack of access to care for infants with birth defects might be contributing to the disparity in IMBD.

Homozygous HOXA1 mutations disrupt human brainstem, inner ear, cardiovascular and cognitive development.

We identified homozygous truncating mutations in HOXA1 in three genetically isolated human populations. The resulting phenotype includes horizontal gaze abnormalities, deafness, facial weakness, hypoventilation, vascular malformations of the internal carotid arteries and cardiac outflow tract, mental retardation and autism spectrum disorder. This is the first report to our knowledge of viable homozygous truncating mutations in any human HOX gene and of a mendelian disorder resulting from mutations in a human HOX gene critical for development of the central nervous system.