Dysplasia epiphysealis hemimelica with involvement of the hip and spine in a young girl.

Dysplasia epiphysealis hemimelica of the left proximal femur was diagnosed in an eight-month-old girl. At the age of 18 months, radiographs of the hip and MRI showed overgrowth and loss of containment of the femoral head. She underwent resection of the superior portion of the head and neck of the femur at the age of 2.5 years. Six months later further radiographs and an MR scan show that the mass has increased in size and that hip containment has been lost. Further plain radiographs have shown that the left knee, ankle and spine were involved. To the authors' knowledge, this is the first report of dysplasia epiphysealis hemimelica involving both the lower limb and the spine. A review of the literature is presented.

[Role of embolization in renal angiomyolipomas]. / Rôle de l'embolisation dans les angiomyolipomes du rein.

OBJECTIVE: The purpose of this study is to present the role of embolization in the treatment of renal angiomyolipoma (AML) in cases of hemorrhage and to prevent bleeding. METHODS: Over a period of 10 years, 35 AMLs in 34 patients, recruited in two medical centers, were treated with embolization: 16/35 AML were treated urgently to stop bleeding, and 19/35 AML had preventive embolization. Six patients were completely asymptomatic and 13 had a history of previous hematoma or flank pain. Catheterization was highly selective in all cases (coaxial microcatheter in 19 cases), and for embolization we used nonresorbable microparticles, coils, and alcohol. RESULTS: When patients presented with acute bleeding, embolization was efficient in 80% of cases; another embolization was necessary in two cases, and surgery in two others. In six of these cases, surgery was planned and done at a later date. When treatment was preventive, one embolization was necessary in 17 cases, and two embolizations per case were necessary in the other two. Over a period of 18 months of follow-up, we observed a 28% decrease in tumor volume; four patients were treated by surgery at a later date. CONCLUSION: Embolization is the technique of choice to treat a bleeding AML urgently. When preventive treatment is considered, in symptomatic or asymptomatic AML, embolization can be an alternative for surgery, but more data is needed to specify its proper place in the management of these tumors.

Hypovitaminosis D in a sunny country: relation to lifestyle and bone markers.

Hypovitaminosis D is associated with poor dietary intake and inadequate sunshine exposure. It is common worldwide, particularly in European elderly people. Information about vitamin D status in young adult populations from the Middle East is scarce. Furthermore, the relationship between hypovitaminosis D and some lifestyle factors such as style of clothing and dwelling location is not well defined. We assessed vitamin D intake and measured serum calcium, phosphorus, albumin, alkaline phosphatase, 25-hydroxyvitamin D [25(OH)D], parathyroid hormone (PTH), osteocalcin, and urinary-free deoxypyridinoline (DPD) in 316 Lebanese volunteers (99 men and 217 women) aged 30-50 years; 156 were recruited from rural areas and 160 from urban areas. Fifty-one women from each area were veiled. The average daily vitamin D intake was 100.3 +/- 67.9 IU and was found to be higher in men compared with women, in urban subjects compared with rural ones and in nonveiled women compared with veiled ones. The mean level of 25(OH)D was 9.71 +/- 7.07 ng/ml. Hypovitaminosis D [25(OH)D < 12 ng/ml] affected 72.8% of our population. It was more common in women than in men (83.9% vs. 48.5%). Severe hypovitaminosis D [25(OH)D < 5 ng/ml] was observed in 30.7% of our subjects and was more prevalent in women (41.5%), particularly in the veiled ones (61.8%). 25(OH)D levels were the lowest in veiled women, and in women living in rural areas. Rural men had the highest 25(OH)D levels despite their very low vitamin D intake. In a multivariate model, inadequate vitamin D intake, urban dwelling, veil wearing, and high parity in women were independent predictors of hypovitaminosis D. 25(OH)D was related inversely to PTH and free DPD whereas osteocalcin achieved only a weak positive correlation with 25(OH)D. In the absence of information regarding time spent outdoors, our results show that hypovitaminosis D is common among young Lebanese people and is related mostly to low vitamin D intake. This should emphasize the need for more vitamin D in our population.

Midterm results of renal artery stenting.

Percutaneous transluminal angioplasty (PTA) has become the treatment of choice for major renal artery stenosis. Nonetheless, about 10% of renal artery stenoses cannot be properly dilated, and among the patients successfully dilated, 10%-15% had a recurrence. Renal artery stenting was used in 21 patients in cases of insufficient results after PTA: persisting significant stenosis after a primary or several PTAs (15 cases), recurrences (9 cases). Follow-up in all patients was from 12 months to 4 years. Implantation was performed without any problems but the low radioopacity of the stent makes placement difficult in obese patients, particularly for ostial lesions. There was no major complication except occlusion of a segmental branch of the renal artery in 1 case. Radiological controls have shown a preserved patency in all cases except 2, which present restenosis inside the stent by intimal hyperplasia. A significant clinical improvement was obtained in 90% of cases. These results suggest that the endovascular prosthesis represents an important adjunct to renal PTA.

[Different methods of percutaneous desobstruction of peripheral arteries]. / Les différentes méthodes de désobstruction des artères périphériques par voie percutanée.

Chronic arterial occlusion of the iliac and femoro-popliteal vessels has been considered, until recently, to be a contraindication to percutaneous transluminal angioplasty. However, recent advances in radiological and catheterisation equipment and the introduction of new methods of recanalisation (laser, mechanical devices) have increased the therapeutic possibilities. The immediate and long-term results of iliac recanalisation are usually excellent with conventional angioplasty methods. The success rate of recanalisation of the femoro-popliteal segments is about 80% irrespective of the method used, the failure rate increasing with the length of the occlusion. However, the long-term patency rate is poor, about 50%. These methods of percutaneous recanalisation are technically difficult and require special training and sophisticated equipment. The indications should be carefully evaluated in a pluridisciplinary manner taking into account the potential clinical benefits, the technical difficulties, the risks and other therapeutic possibilities.

[Self-expanding endoprostheses. Applications in iliac and femoro-popliteal arteries]. / Endoprothèses vasculaires auto-expansibles. Applications au niveau des artères iliaques et fémoro-poplitées.

Vascular endoprosthesis are aimed at optimizing the results of angioplasty. They are metallic cylinders, most often meshed, laid against the wall of the vessel and progressively incorporated into the intima by endothelial coverage. Experimental studies show that this incorporation occurs within a few weeks. The prosthesis are either self-expanding or mounted on a dilatation balloon. Their aim is to restore a good-quality caliber by smoothing the parietal irregularities that were frequently encountered after dilatation. They are a true parietal prop, inserted into the arterial lumen through a percutaneous approach. The middle-term results of iliac and femoropopliteal implants are very encouraging, and they suggest that this technique may be a very useful complement to transluminal angioplasty in cases of poor results or recurrence. The insertion of an endoprosthesis also allows to extend the indications of transluminal angioplasty to cases that had been regarded as hardly favorable so far: long, irregular stenosis, iliac obliteration, etc.

Substantial spontaneous long-term improvement in subacute sclerosing panencephalitis. Six cases from the Middle East and a review of the literature.

Of 118 cases of subacute sclerosing panencephalitis identified in an extensive follow-up study in the Middle East, six patients were found by personal interview to have experienced substantial spontaneous long-term improvement. To our knowledge, this rate of 5% for such improvement is presently the most accurate estimate available. Relative to the onset of clinical illness, the conditions of two patients are still improving four to five years later, two are stable four to six years later, one relapsed and died recently after eight years, and one is currently in relapse after 11 years. The condition of five patients had not progressed beyond stage 2 before improvement began. All patients had characteristic clinical and electroencephalographic features. Two patients had brain biopsy specimens showing panencephalitis without inclusions. Five patients had highly elevated levels of serum and CSF measles antibodies. In this article, the course of illness and extent of disability are described, published reports of improvement are reviewed, and the possibility of subclinical illness is discussed.