[Traumatic bending of the long bones in children].
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Biomedical subjects
Publications and source records attributed to G Leone.
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A patient suffering from Gaucher's disease observed for the first time five years age while in pregnancy and observed again during a second pregnancy ended with the delivery of a malformed female infant is presented. Laboratory findings of this case and their correlation with the clinical manifestations of this disease are underlined. Occurrence of foetal malformations in this disease is also discussed.
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4 cases of extramedullary hematopoiesis (EH) in the posterior mediastinum in patients affected with serious chronic anemia are presented. The contribution of conventional radiology and computed tomography (CT) to the assessment of an exact diagnosis, is discussed. The CT images are above all characterized by the presence of typical bilateral, asymmetrical paravertebral hyperdense masses (about 45 HU). Besides, a striated aspect of the vertebral bodies is common. For a rather sure diagnosis of EH in patients with chronic anemia, a conventional chest X-ray and CT of the thorax is sufficient. Biopsy and thoracotomy should be avoided.
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It was the purpose of this study to examine the effect of 1,4-bis[3-(3,4,5-trimethoxybenzoyl-oxy)propyl]-perhydro-1,4-diazepine (dilazep) on platelet function in vivo, compared with that of dipyridamole. 15 patients were given oral doses of 300 mg dilazep daily, and another 15 patients received oral doses of 450 mg dipyridamole daily. Blood was withdrawn 2 and 4 weeks after the start of treatment, in each case 2 h after administration of the drug. The results were as follows: bleeding time was prolonged in both groups; there was a percentage reduction of circulating platelet aggregates in both groups, but this was statistically significant in the dilazep group only; platelet aggregation was decreased in both groups, several parameters (minimum dose required to induce aggregation, collagen lag phase) were statistically significantly improved in the dilazep group only; platelet malondialdehyde production was unchanged; no changes were demonstrated in platelet shape, fibrinogen concentration, partial thromboplastin time, or prothrombin activity.
Partial nodular transformation is a rare disorder of the liver, that is becoming a very interesting speculative problem, in relation to the nodular regenerative hyperplasia of the liver and cirrhosis. This disease is a cause of non cirrhotic protal hypertension. Clinical and pathological aspects are discussed a propos of a case.
Five patients affected by aseptic dural sinus thrombosis have been treated with a combination of heparin sodium and urokinase. In all of them, the therapy was followed by complete clinical recovery. Postoperative cerebral angiography showed patency of the involved sinuses in all.
Percentage of megathrombocytes, platelet regeneration time (PRT) and platelet-Associated IgG (Pl-A-IgG) were investigated in 12 patients with clinical features consistent with idiopathic thrombocytopenic purpura and in 11 patients with thrombocytopenia associated with chronic liver disease. Bone marrow smears were also examined and megakaryocytes classified into stages I-III according to the current principle. Of 12 patients with idiopathic thrombocytopenic purpura the percentage of megathrombocytes was increased in 9, PRT reduced in 10, and Pl-A-IgG increased in 8 patients. A statistically significant correlation was found between the percentage of megathrombocytes and the level of Pl-A-IgG. A slight correlation was also found between PRT and the percentage of megathrombocytes, while a significant correlation was found between megakaryocytes in stage I and the percentage of megathrombocytes, suggesting that growth of megakaryocytes probably contributes to platelet heterogeneity. In patients with thrombocytopenia and chronic liver disease, the percentage of megathrombocytes was in the normal range. A moderately increased level of Pl-A-IgG was found only in patients with active chronic hepatitis, PRT was reduced only in a few patients, while most of them also showed an increase of Pl-A-IgG.
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A case of asymmetrical femoral hypoplasia in an adult male is described; the malformation consists of femoro-tibial fusion in the right leg and femoral hypoplasia affecting the shaft and proximal end in the left; associated abnormalities are pelvis hypoplasia, bilateral fibular emimelia, absence of lateral two rays of feet, triceps muscle hypoplasia of right humerus, thin upper lip and broad nasal tip.
A sporadic case of Weismann-Netter and Stuhl syndrome with typical bilateral leg changes, marked kyphoscoliosis anc costal deformity, iliac wings very expanded and horizontal sacrum, dolichophalangy in the hands is described. To explain the pathogenesis of tibial and fibular changes, a coexistence of exaggerated developmental external rotation of distal end of the tibia with a mechanism of functional adaptation is supposed.
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In order to investigate the role of an immune mechanism in the pathogenesis of thrombocytopenia in hepatic patients, we measured platelet associated IgG (PAIgG) in 84 patients with various hepatic diseases. Increased PAIgG levels were found in 84% of patients with chronic active hepatitis (mean 21.32 +/- 8.45 fg/platelet) and in 75% of those with cirrhosis with hepatitis (mean 17.3 +/- 11.2 fg/platelet). In these patients there was no significant correlation between PAIgG and platelet count. Increased PAIgG amounts were also observed in some patients with inactive cirrhosis (18%). Normal PAIgG values were found in all patients with acute viral hepatitis and chronic persistent hepatitis. An immunologic mechanism may play a role in the pathogenesis of thrombocytopenia in hepatic patients. Furthermore, measurement of PAIgG may have a great usefulness in the differential diagnosis of chronic hepatic diseases.