Thromboembolic Pulmonary Hypertension Due to Disseminated Fibromuscular Dysplasia

We present two patients with thromboembolic pulmonary hypertension associated with unusual complications probably caused by disseminated fibromuscular dysplasia (FMD) or FMD-like vascular lesions. Intimal fibroplasia, which is typical of the vascular lesions associated with FMD, was observed in both patients. The presence of such intimal lesions suggests that there was a systemic factor that caused the formation of recurrent thrombi in the systemic vessels in these patients. These cases are the first ones reported in which an association between FMD and pulmonary hypertension has been observed. The pathogenesis of the thrombi in our patients was thought to be recurrent pulmonary thromboembolisms resulting from FMD.     

小儿先天性髋内翻Borden截骨术后12年以上随访

通过长期随访，评价Ｂｏｒｄｅｎ截骨术的应用价值。经Ｂｏｒｄｅｎ截骨术治疗１４岁以下１１例（１４髓）先天性髓内翻，并进行了１２年以上的随访。结果：功能和Ｘ线片皆正常５例（６髋）；短颈、大粗隆高位３例（３髋）；术后５年恢复正常，后因合并类风湿症服用肾上腺皮质激素致股骨头坏死后遗扁平髋１例（２髋）；术前即有股骨头坏死，后遗扁平髋１例（２髋）；复发１例（１髋）。正确应用Ｂｏｒｄｅｎ截骨术，可以使先天性髋内翻恢复接近正常的解剖形态和功能，但如果术中操作不当或术前合并有其他病变将影响疗效。     

A randomized clinical trial of beta carotene vs placebo for the treatment of cervical HPV infection

The objective of the study was to determine if oral beta carotene would improve abnormalities observed in Papanicolaou smears or reduce the amount of HPV DNA in genital samples. A randomized double blind placebo controlled trial was designed for 117 women with abnormal cervical morphology, not undergoing laser ablative therapy, at The Royal Women's Hospital, Victoria. Thirty milligrams of oral beta carotene were administered daily for 12 months. Post-intervention cervical cytology and the amount of HPV DNA present on tampon specimens as determined by polymerase chain reaction and Hybrid Capture were the main outcome measures. After 12 months therapy there was no difference between the beta carotene and placebo groups in Papanicolaou smear results (58% and 62% normal) and HPV positivity (42% and 46% positive) ( P > 0.86). Women taking beta carotene were not more likely to have improved cervical cytology or a decrease in the amount of HPV DNA ( P > 0.2). The median post-intervention beta carotene level was 0.63 µm ml⁻¹ (range 0.04–1.6) for the beta carotene group and 0.15 µm ml⁻¹ (range 0.02–1.51) for the lecithin group ( P < 0.0001). This clinical trial did not identify a beneficial effect of 30 mg of oral beta carotene on cervical cytology, or on the amount of HPV DNA present from tampon specimens.     

Ghosal haemato-diaphyseal dysplasia: A new disorder

We describe two siblings, products of a first cousin marriage, with diaphyseal dysplasia, severe anaemia, leukopenia, and thrombocytopenia. Radiologically, both had wide medullary cavities with discrete cortical hyperosthosis. Bone marrow was hypocellular. These, and six similar cases in the literature [6], suggest that they represent a form of diaphyseal dysplasia differing from Camurati-Engelmann disease by their radiological appearance, associated haematological abnormalities and autosomal recessive inheritance.     

Prediction of outcome of preterm infants with severe bronchopulmonary dysplasia

Abstract Forty-four preterm infants of less than 30 weeks gestation and birthweight £ 1250 g, with severe bronchopulmonary dysplasia requiring mechanical ventilation for at least 28 days, were reviewed. Twenty-seven infants (61%) survived; 17 died. There were no significant differences between survivors and non-survivors with respect to birthweight, gestational age, sex, Apgar score at 5 min or pulmonary diagnosis. Non-survivors displayed more severe changes on chest X-ray than the survivors. Peak inspiratory pressure (PIP), ventilator rate (VR), ventilator index and mean airways pressure were significantly higher in the non-surviving infants on days 2, 3, 4, 7, 14, 21 and 28, with non-survivors also having significantly higher alveolar-arterial oxygen gradients and lower arterial-alveolar oxygen ratios than the survivors. Discriminant analysis with cross-validation by pairing PIP and VR on day 28 produced a positive predictive value for non-survival of 88% and a negative predictive value of 89%. This result was better than was obtained for any other pair of ventilator parameter or oxygenation index. Discriminant analysis by combining X-ray appearances with ventilator settings did not improve the prediction. Having established a statistical model based on the PIP and VR of ventilator-dependent preterm infants on day 28, the outcome can be predicted with a high degree of confidence. This has the immediate potential application of indicating to staff in the neonatal unit a realistic approach to take when counselling parents of these infants.     

Hirschsprung's disease and intestinal neuronal dysplasia – a frequent association with implications for the postoperative course

Between 1991 and 1993, 106 newly diagnosed cases of Hirschsprung's disease (HD) were prospectively analyzed for the association of HD and intestinal neuronal dysplasia (IND) at ten pediatric surgical departments in central Europe. Hirschsprung-associated IND (HaIND) was found in 40% of cases. IND was disseminated in one-third and localized in two-thirds of the patients. Initial clinical symptoms were related to the length of the aganglionic segment, but not to the presence of HaIND. An enterostomy performed in 72 cases (67.9%) was located in a segment of pathologically innervated bowel in 50% of all cases, but in 72% of cases of HaIND. The proximal margin of the resected bowel showed pathological innervation in 44% of cases. Supplemental biopsies from the intestine (apart from diagnostic suction biopsies and biopsies at the enterostomy site) led to the first identification or definition of length of associated IND in 17.9% of cases. Postoperatively, the presence of long-segment aganglionosis or associated IND implied a delay in the restoration of normal defecation. Persistent constipation was found in 40% of patients with associated disseminated IND at follow-up at 6 months, compared to 20.6% in patients with isolated HD. These children needed secondary interventions more often than patients with associated localized IND or isolated HD. HaIND thus has clinical implications for the postoperative course if IND is disseminated. Accepted: 25 April 1999     

VERTICAL SUBLUXATION OF THE PATELLA: AN UNUSUAL FEATURE OF MULTIPLE EPIPHYSEAL DYSPLASIA

Book reviews in this article: Abnormally shaped, usually double-layered, patellae are recognized as occurring in multiple epiphyseal dysplasia. The case described is unusual in that an associated defect appeared in the femur and resulted in mechanical symptoms.     

全髋关节置换术治疗先天性髋关节脱位

目的 :探讨全髋关节置换术治疗成人先天性髋关节脱位伴骨性关节炎的经验。方法 :从 1996年 9月～2 0 0 3年 2月 ,对 14例 (2 0髋 )成人先天性髋关节脱位伴骨性关节炎患者 ,进行了全髋关节置换。其中双侧 10例 ,单侧 4例。病人全是女性 ,平均年龄 45岁 (3 5～ 67岁 )。双侧全髋置换 6例 ,单侧 8例。结果 :术后伤口均一期愈合 ,随访 6个月～ 6年 ,平均 4年 8个月 ;平均Harris评分由术前的 3 2 43分恢复到术后 1年的 90 15分。截止目前无 1例翻修。术后 6个月 ,患者均能下地行走 ,生活自理且恢复日常工作。结论 :全髋关节置换术是治疗成人先天性髋关节脱位伴骨性关节炎的有效方法 ,但手术难度较大。对于此类病人 ,充分理解全髋置换的复杂性和细致周密的治疗方案是成功的关键。     

High resolution MR imaging of the bilateral hips with dual phased-array coil

We applied dual-flex coils in a phased-array configuration to obtain high resolution MR images of the bilateral hips and compared the image quality with non-phased-array imaging. Multiple fast spin-echo images of both hips were obtained in a single acquisition. Two consecutive acquisitions with and without phased-array capability were performed in 22 hips and a fluid-filled phantom. The signal-to-noise ratio (S/N) enhancement values were calculated. Subjective image analysis was also obtained from three independent reviewers. High quality MR images were obtained using dual-array coils with a S/N enhancement value of 1.40 for a phantom and 1.83 to 2.22 for subject hips. On subjective analysis, the dual array enhanced delineation of the acetabular labrum, articular cartilage, and retained fluid. The dual array may be of diagnostic value with a higher S/N enhancement and subjective superiority of the images obtained.     

Magnetic resonance imaging of dialysis-related amyloidosis of the shoulder and hip

 Objective. The purpose of this study was to evaluate the usefulness of MRI in identifying abnormalities of the periarticular structures in patients with symptoms of dialysis-related amyloid arthropathy. Design and patients. MR images of shoulders and hips in 5 dialysis patients with symptoms of amyloid arthropathy were compared to images of shoulders and hips in 4 asymptomatic dialysis patients, shoulders in 9 nondialysis patients, and hips in 12 nondialysis patients. These were evaluated for the presence of focal periarticular osseous lesions, tendinous and capsular thickening, and periarticular fluid. Results. Increased thickness of the supraspinatus tendon was found in both symptomatic and asymptomatic patients. Capsular thickening at the hip was present in all symptomatic patients, and in 3 of 8 asymptomatic hips. Joint and bursal fluid was common in the symptomatic group, and was present in some asymptomatic individuals. Osseous lesions were detected in the absence of plain film findings, and demonstrated variable signal intensity. Conclusions. MRI is useful in detecting signs of dialysis-related amyloidosis. Findings of a milder degree in asymptomatic dialysis patients suggests that MRI may also be valuable in the early diagnosis of this syndrome.