Prognostic Factors in Acetabular Development Following Reduction of Developmental Dislocation of the Hip in Patients under the Age of 24 Months / 대한정형외과학회잡지

PURPOSE: The aims of this study are to evaluate the outcome of treatment for developmental dislocation of the hip (DDH) in children under the age of 24 months who underwent open reduction (OR) or closed reduction (CR) and to determine radiologic prognostic factor. MATERIALS AND METHODS: A total of 90 hips of 88 children under the age of 24 months treated for DDH were included. The treatments for these children were CR in 29 hips and OR in 61 hips. All patients were followed up for more than 5 years. Radiographic evaluations, including acetabular index (AI), Yamamuro's distance a and b, center-edge angle (CEA), sourcil shape, and teardrop shape have been proposed to indicate the degree of DDH. Hips were reclassified according to the Severin criteria (classes I and II, satisfactory; classes III and IV, unsatisfactory). RESULTS: Among the 90 hips, 67 hips (74.4%) were included in the ‘satisfactory group’, while 23 hips (25.6%) were included in the ‘unsatisfactory group’. In the CR group, 23 hips (79.3%) were included in the ‘satisfactory group’, while 6 hips (20.7%) were included in the ‘unsatisfactory group’. In the OR group, 44 hips (72.1%) were included in the ‘satisfactory group’, while 17 hips (27.9%) were included in the ‘unsatisfactory group’. There was no significant difference between the reduction methods. At 1 year follow-up after reduction, the AI improvement in the ‘satisfactory group’ (8.1° [23.4%]) was significantly higher than that in the ‘unsatisfactory group’ (6.7° [18.5%]) (p=0.012). A significant difference of the mean CEA values was observed between the ‘satisfactory group’ and the ‘unsatisfactory group’ 3 years after the treatment (p=0.001). Five years after reduction, the V shape of teardrop and the upward shape of acetabular sourcil were observed in 2 hips (3.0%) and 4 hips (6.0%) of the ‘satisfactory group’, respectively, whereas the corresponding findings were observed in 3 hips (13.0%) and 5 hips (21.7%) of the ‘unsatisfactory group’, respectively (p=0.023, 0.005). CONCLUSION: The improvement of AI at 1-year and CEA at 3-year follow-ups, as well as teardrop shape and sourcil shape at 5-year followup, were reliable radiographic prognostic factor of DDH.

Survival according to Treatment Modalities in 137 Patients with Aplastic Anemia / 대한혈액학회지

BACKGROUND: We performed this study to compare response rate and survival according to treatment modalities, such as allogeneic bone marrow transplantation (BMT), immunosuppressive therapy (IS) and androgen therapy in patients with aplastic anemia (AA). METHODS: Medical records of one hundred and thirty-seven patients who diagnosed with aplastic anemia at the Asan Medical Center from September 1989 to December 2000 were retrospectively analyzed. RESULTS: Forty-one patients received supportive care only. Ninety-five out of 96 treated patients were evaluable for response. In severe AA group (N=79), the response rate according to treatment modalities was 25.0% in the androgen group (N=4), 30.3% in the ALG or ATG group (N=33), 25.0% in the cyclosporine (CSA) group (N=8), 44.4% in the ATG plus CSA group (N=9), and 73.8% in the BMT group (N=23) (P<0.001). Five- and 10-year survival of overall patients was 67.5% and 50.9%, respectively. Ten-year survival according to disease severity was 42.3% in severe AA group and 75.8% in non-severe AA group (P=0.0043). Five- year survival according to treatment modalities in patients with severe AA was 75.0% in the androgen group, 49.0% in the ALG or ATG group, 75.0% in the CSA group, and 88.9% in the ATG plus CSA group. Five-year survival was 82.6% in the BMT group and 57.7% in the IS group (P=0.0259). CONCLUSION: This study showes that BMT resulted in significantly better response rate and overall survival compared to IS in patients with severe AA.

A Case of Pulmonary Hypertension Associated with Portal Hypertension / 결핵

Since its first description in 1951 by Mantz and Craig, pulmonary hypertension associated with portal hypertension has been observed more frequently. In a recent prospective study Hadengue et al. reported 2% incidence of pulmonary hypertension in patients with portal hypertension. Thus this simultaneous occurrence can no longer be considered to be coincidental. The etiology remains unclear. It is most likely that vasoactive substances (,)normally metabolized by the liver(,) may gain have gained access to the pulmonary circulation through portosystemic collaterals in portal hypertension. In genetically susceptible individuals, these substances could lead to pulmonary hypertension by inducing vasoconstriction or direct toxic damage to the wall of the small pulmonary arteries. A recent case of pulmonary hypertension in a 49-year-old woman with portal hypertension due to liver cirrhosis is reported as well as a review of the literature.

Nuclear Hyperploidy of Megakaryocytes and Platelet Aggregation Test in Essential Thrombocythemia / 대한혈액학회지

BACKGROUND: Essential thrombocythemia (ET) is a rare chronic myeloproliferative disorder characterized by an extremely high platelet count in the circulating blood and abnormal proliferation of the megakaryocytes in bone marrow, resulting in splenomegaly, thromboembolic or hemorrhagic complications. We studied the presence of nuclear hyperploidy of the megakaryocytes in bone marrow, the presence of abnormal response to the individual reagent on platelet aggregation test, and its clinical implication. METHODS: We analyzed the 43 cases of ET at the Asan Medical Center between January, 1989 and March, 1999. The Polycythemia Vera Study Group criteria were used to diagnose ET. RESULTS: Nuclear hyperploidy was observed at 43 cases (100%). Platelet aggregation test was done at 32 (74.4%) cases, of which 27 (84.4%) cases showed abnormal response to more than one reagent, 16 (50%) cases to more than two reagents. Abnormal response to epinephrine and collagen was most common, but 5 cases showed normal response. By individual reagent, 1 (3%) cases to adenosine diphosphate, 1 (3%) case to ristocetin, 22 (69%) cases to epinephrine, 19 (59%) cases to collagen showed abnormal response. CONCLUSION: We observe that nuclear hyperploidy of the megakaryocyts and abnormal response on platelet aggregation test are frequent in ET in this study.

Breast Disease with Skin Thickening: Differential Diagnosis with Mammography & Ultrasonography

Diffuse skin thickening of the breast is produced by lymphedema usually secondary to obstruction of theaxillary lymphatics. On physical examination, the affected breast is, due to increased fluid content, larger,heavier, and of higher overall density. Mammography reveals an increased coarse reticular pattern. Thickening ofthe skin can have many causes. It may be a result of tumor invasion or a tumor in the dermal lymphatics; orbecause of lymphatic congestion through obstruction of lymphatic drainage within the breast, in the axilla, orcentrally in the mediastinum. Further causes may be congestive heart failure, benign inflammation, primary skinprocesses such as psoriasis, or systemic diseases which involve the skin. Mammographic appearance is known to benonspecific. Ultrasound can demonstrate skin thickening directly, but despite some reports suggesting that thecause of skin thickening can be inferred from the results of ultrasound, this is not usually of practicalimportance. The purpose of this study is to review the causes of skin thickening of the breast and to usemammography and US to differentiate the causes.