[ introduction of the first case of jarcho- levin syndrome from Iran]

Jarcho-Levin syndrome is a rare central skeletal system defect with the involvement of ribs and vertebrae and association of short trunk stature and some other organ anomalies. One of the common problems is different rate of respiratory abnormalities. Chest X-Ray is the primary base for the diagnosis, but spiral 3D CT scan give us more accurate evaluation of the ribs situation. There are also cardiac problems associated with this syndrome. In this case we present a 9- month- old infant who had Jarcho-Levin syndrome with right-sided heart failure

Hepatic doppler ultrasound in assessment of the severity of esophageal varices in cirrhotic patients

Endoscopy is the gold-standard technique for the assessment of acutely hemorrhagic esophageal varices in patients with hepatic cirrhosis. The objective of this study is to determine the value of different hepatic vasculature Doppler ultrasonography and their flow characteristics for non-invasive assessment of esophageal varices. Fifty-five [31 male, 24 female] consecutive patients with a mean +/- SD age of 55 +/- 16 [range: 20-88] years, with biopsy-proven hepatic cirrhosis were prospectively studied using Doppler ultrasonography. All of these patients were also examined endoscopically and by echocardiography. None of patients had clinical or echocardiographic signs of right heart failure, tricuspid valve regurgitation or previous history of therapeutic interventions on varices. An ordinal logistic regression [OLR] model was used for determining the adjusted associations between sizes of esophageal varices and hepatic hemodynamic determinants. There was a significant correlation between the size of esophageal varices and maximum portal vein velocity, which was lower in patients with varices [p= 0.04]. Other parameters though not statistically significant, were of clinical importance. Those included portal vein mean velocity [p = 0.08], hepatic artery volume flow [p = 0.06] and hepatic venous waveform pattern [p = 0.15]. OLR model did not show any significant adjusted associations between these parameters and the size of esophageal varices. The maximum portal vein velocity and to a lesser extent, hepatic artery volume flow were superior to Doppler ultrasonographic spectral waveform pattern of hepatic vein in differentiating patients with esophageal varices from those with no varices. None of hepatic vasculature Doppler measurements had a significant role in predicting the size of esophageal varices, nonetheless

Agreement determination between coronary calciumscoring and coronary stenosis significance on CT-angiography

The most important lesions in coronary artery disease [CAD] are coronary artery plaques, many of which are calcified. Multi-slice spiral CT [MSCT] scanners can concurrently perform coronary calcium scoring [Ca-Score] as a predictor of CAD and coronary CT-angiography [CCTA] as the determining factor in therapeutic decision-making. We aimed to determine the agreement of a Ca-Score more than 100 [based on Agatston technique] with coronary artery stenosis significance on CCTA. Using ECG-gated MSCT, 65 patients who were referred for CCTA were assessed both for their Ca-Score and a significant [>/= 50% diameter reduction] coronary stenosis, simultaneously. Their total Ca-Score were classified in three groups [a-O, b-less than 100, and c- >/= 100]. The severity of coronary stenosis was categorized to further three groups [1-lack of stenotic lesion, 2- presence of non-significant stenosis, and 3-presence of significant stenosis]. Of 65 patients referred for CCTA, 42 [64.61%] had no CAD, 8 [12.3%] had non-significant lesions' and 15 [23.09%] had significant stenoses. Forty-three [66.2%] out of 65 subjects had a zero. 14 [21.5%] had scores < 100, and 8 [12.3%] had >/= 100 Ca-Score. In the first group [Ca-score = 0], only one had significant stenosis; while 50% of the patients in the second group [Ca-score < 100] and 87.5% from the third group [Ca-score of >/= 100] had significant stenosis. Significant coronary stenosis has a moderate-to-good agreement with a Ca-Score of 100 or higher, compared to those with a Ca-Score of less than 100, and this was statistically significant [P < 0.0001]. In patients with a calcium score of 100 or more, performing CCTA may be advisable to assess the likelihood of significant CAD