Carotid sheath lipoma: first case report in the English literature

Lipomas are the most commonly encountered benign mesenchymal tumour, arising in any location where fat is normally present. Lipomas in the head and neck are rare in all age groups. Cases of vascular sheath lipomas in the femoral region have only been reported in adults. In children, vascular sheath lipomas have not been described to date. We report the first case of a carotid sheath lipoma in a seven-year-old boy. He had a non-tender soft mass with ill defined borders occupying the left upper part of the neck. Magnetic resonance imaging showed a mass at the bifurcation of the left common carotid artery without involving the same. The mass was hyperintense on T1 and T2 weighted sequences, suggestive of a lipoma. The lipoma was enucleated after incising the carotid sheath, safeguarding its contents. Histopathology confirmed it as a lipoma.     

Sinonasal T Cell Lymphoma: A Case Report

Sinonasal lymphomas are aggressive locally destructive midfacial necrotizing lesions. Most of them initially diagnosed as lethal midline granulomas, a term which is slowly replaced by sinonasal lymphoma. Here is one such case report of sinonasal T cell lymphoma where there was a difficulty in diagnosis and required an incisional biopsy.     

Repeated infusions of identical doses of norepinephrine show potentiation of metabolic responses in human subjects

Thermogenic responses to similar doses of norepinephrine appear to be different when repeatedly administered. This hypothesis was tested by three consecutive 30-minute infusions of a fixed dose of norepinephrine separated by a rest period of one hour between infusions. There was a significant increase or potentiation of the metabolic response as measured by oxygen consumption to the third dose of norepinephrine, while the cardiovascular responses showed no change. It is therefore important to make allowances for this phenomenon when assessing intergroup differences in regulatory thermogenesis using dose-response protocols for norepinephrine administration.     

Chronic synovitis and HLA B27 in patients with severe haemophilia

Chronic synovitis affects about 10% of patients with severe haemophilia in India. This disease has some features in common with ankylosing spondylitis, which has been linked to HLA B27. We therefore aimed to test whether there is an association between HLA B27 and chronic synovitis. We studied 473 patients with severe haemophilia (33 of whom had chronic synovitis), and 1175 healthy controls using a standard serological technique and the reverse line strip assay. 64% (21 of 33) of patients with haemophilia and chronic synovitis were positive for HLA B27, compared with 5% (23 of 440) of those with severe haemophilia, but not chronic synovitis (odds ratio 31.6 [95% CI 9.28-39.38], p<0.0001), and 9% (100 of 1175) of healthy controls (18.81 [9.6-27.7], p<0.0001). We conclude that there is a strong association between HLA B27 and chronic synovitis in Indian patients with severe haemophilia and screening in this population could allow treatment and prevention of the complication.     

Decreased expression of phospholipase C-beta 2 isozyme in human platelets with impaired function

Platelets from a patient with a mild inherited bleeding disorder and abnormal platelet aggregation and secretion show reduced generation of inositol 1,4,5-trisphosphate, mobilization of intracellular Ca2+, and phosphorylation of pleckstrin in response to several G protein mediated agonists, suggesting a possible defect at the level of phospholipase C (PLC) activation (see accompanying report). A procedure was developed that allows quantitation of platelet PLC isozymes. After fractionation of platelet extracts by high-performance liquid chromatography, 7 out of 10 known PLC isoforms were detected by immunoblot analysis. The amount of these isoforms in normal platelets decreased in the order PLC- gamma 2 > PLC-beta 2 > PLC-beta 3 > PLC-beta 1 > PLC-gamma 1 > PLC- delta 1 > PLC-beta 4. Compared with normal platelets, platelets from the patient contained approximately one-third the amount of PLC-beta 2, whereas PLC-beta 4 was increased threefold. These results suggest that the impaired platelet function in the patient in response to multiple G protein mediated agonists is attributable to a deficiency of PLC-beta 2. They document for the first time a specific PLC isozyme deficiency in human platelets and provide an unique opportunity to understand the role of different PLC isozymes in normal platelet function.     

Human platelet signaling defect characterized by impaired production of inositol-1,4,5-triphosphate and phosphatidic acid and diminished Pleckstrin phosphorylation: evidence for defective phospholipase C activation

Signal transduction on platelet activation involves phosphoinositide- specific phospholipase C (PLC)-mediated hydrolysis of phosphatidylinositides and formation of inositol-1,4,5-triphosphate [I(1,4,5)P3], which mediates Ca2+ mobilization, and diacylglycerol (DG), which activates protein kinase C (PKC) to phosphorylate a 47-kD protein (Pleckstrin). We studied these events in two related patients previously reported (Blood 74:664, 1989) to have abnormal aggregation and 14C-serotonin secretion, and impaired intracellular Ca2+ mobilization in response to several agonists. Thrombin-induced I(1,4,5)P3 and phosphatidic acid formation were diminished. Pleckstrin phosphorylation was impaired on activation with thrombin, platelet- activating factor, and ionophore A23187, but was normal with PKC activator 1,2-dioctonyl-sn-glycerol (DiC8). Ca2+ mobilization induced by guanosine triphosphate (GTP) analog guanosine 5'-0-(3 thiotriphosphate) (GTP gamma S) was diminished. Pretreatment with either A23187 or DiC8 did not correct the impaired adenine diphosphate- induced secretion; however, upon stimulation with A23187 plus DiC8, pleckstrin phosphorylation and secretion were normal, indicating that both PKC activation and Ca2+ mobilization are essential for normal secretion. We conclude that these patients have a unique inherited platelet defect in formation of two key intracellular mediators [I(1,4,5)P3 and DG] and in the responses mediated by them due to a defect in postreceptor mechanisms of PLC activation.