Pulmonary hypertension in sickle cell disease

BACKGROUND: Recurrent vaso-occlusive episodes lead to progressive end-organ damage in patients with sickle cell disease. We sought to determine the prevalence of pulmonary hypertension in adult patients with sickle cell disease and to identify factors associated with this life-threatening complication. METHODS: Sixty patients (> or =18 years of age; mean [+/- SD] age, 37 +/- 13 years) followed at a University Medical Center were evaluated. They were selected by a systematic sampling of patients presenting to the clinic for routine follow-up visits. All enrolled subjects underwent a clinical examination, Doppler echocardiography, pulmonary function tests, and hematologic tests during a single visit. Pulmonary hypertension was defined using an age- and body mass index-adjusted nomogram. RESULTS: The prevalence of pulmonary hypertension was 30% (18/60). Ten patients had mild pulmonary hypertension (up to 44 mm Hg), 5 had moderate pulmonary hypertension (45 to 74 mm Hg), and 2 had severe pulmonary hypertension (> or =75 mm Hg). In a logistic regression model, both lower fetal hemoglobin level and lower systolic blood pressure were associated with the presence of pulmonary hypertension. CONCLUSION: We found that the prevalence of pulmonary hypertension in adult patients with sickle cell disease was substantial, particularly in those with lower levels of fetal hemoglobin and lower systolic blood pressure.     

Rasmussen encephalitis associated with Parry-Romberg syndrome

Parry-Romberg syndrome is a rare disorder associated with unilateral facial atrophy involving skin, subcutaneous tissue, skeletal muscle, and bone. Occasionally, there is CNS involvement with epilepsy being the most common CNS manifestation. The authors report a child with Parry-Romberg syndrome with a course strongly suggestive of Rasmussen encephalitis. The boy underwent hemispherectomy, and pathology showed the typical findings of Rasmussen encephalitis, suggesting that these two conditions may share common etiologic factors.     

Amplatzer Device closure of Atrial Septal Defects and Patent Ductus Arteriosus: Initial Experience

Surgical closure of Atrial Septal Defects (ASD) and Patent Ductus Arteriosus (PDA) can be performed successfully with low mortality. However, the morbidity associated with general anaesthesia, thoracotomy, cardiopulmonary bypass, postoperative monitoring in the intensive care unit, several days of hospital stay and the requirement of blood products is considerable. The expense associated with this morbidity, operative scar and the psychologic trauma to the patient and parents are additional disadvantages of surgery. Hence, the closure of these defects by transcatheter methods with various devices has been evaluated worldwide. We report the initial experience at our centre with closure of secundum ASDs and large PDAs with the Amplatzer Septal Occluder and Amplatzer Duct Occluder.Key Words: Amplatzer device, Large Patent Ductus Arteriosus, Secundum Atrial Septal Defects     

Fertility Enhancing Hysteroscopic Surgery: Our Experience

690 hysteroscopies done over a period of four and a half years for infertility were evaluated. 85 therapeutic hysteroscopic procedures like hysterscopic metroplasty, lysis of intrauterine adhesions, electroresection of fibroids, removal of foreign bodies and cornual cannulation were carried out. These minimally invasive procedures were associated with satisfying results, least morbidity, shorter hospital stay and high patient satisfaction.Key Words: Cornual cannulation, Electroresection, Hysteroscopy, Intrauterine adhesions, Metroplasty, Submucus fibroids     

Comparison of abdominal aortic aneurysm diameter measurements obtained with ultrasound and computed tomography: Is there a difference?

OBJECTIVES: Accurate diameter measurements of abdominal aortic aneurysm (AAA) with both computed tomography (CT) and ultrasound (US) are essential for screening, planning surgical intervention, and follow-up after endovascular repair. Often there is a discrepancy between measurements obtained with CT and US, and neither limit of agreement (LOA) nor correlation between the two imaging methods has been clearly established. The purpose of this study was to assess the paired differences in AAA diameter measurements obtained with CT and US in a large national endograft trial. METHODS: CT and US measurements were obtained from an independent core laboratory established to assess imaging data in a national endograft trial (Ancure; Guidant, Menlo Park, Calif). The study included only baseline examinations in which both CT and US measurements were available. Axial CT images and transverse US images were assessed for maximal AAA diameter and recorded as CT(max) and US(max), respectively. Correlations and LOA were performed between all image diameters, and differences in their means were assessed with paired t test. RESULTS: A total of 334 concurrent measurements were available at baseline after endovascular repair. CT(max) was greater than US(max) in 95% (n = 312), and mean CT(max) (5.69 +/- 0.89 cm) was significantly larger (P <.001) than mean US(max) (4.74 +/- 0.91 cm). The correlation coefficient between CT(max) and US(max) was 0.705, but the difference between the two was less than 1.0 cm in only 51%. There was less discrepancy between CT(max) and US(max) for small AAA (0.7 cm, 15.3%) compared with medium (0.9 cm, 17.9%) and large (1.46 cm, 20.3%) AAA; however, the difference was not statistically significant. LOA between CT(max) and US(max) (-0.45-2.36 cm) exceeded the limits of clinical acceptability (-0.5-0.5 cm). Poor LOA was also found in each subgroup based on AAA size. CONCLUSIONS: Maximal AAA diameter measured with CT is significantly and consistently larger than maximal AAA diameter measured with US. The clinical significance of this difference and its cause remains a subject for further investigation.