Non-Hodgkin's lymphoma with protein S deficiency]

Venous thrombosis is rare in children. It can be either acquired or of constitutional origin. Thrombosis during non-Hodgkin lymphoma remains exceptional and is usually locally associated to the tumoral process, raising the issue of its tumoral or cruoric nature. The treatment is based on anticoagulation concomitantly to chemotherapy. We report on a 4-year-old boy admitted for mediastinal non-Hodgkin lymphoma, who developed a thrombosis of the superior vena cava associated to protein S-deficiency. The mechanism of thrombosis may have been multifactorial: associated protein S-deficiency, vascular compression, tumoral process and chemotherapy.     

Recombinant activated factor VII for intractable bleeding post splenectomy in a patient with myeloproliferative disorder.

Recombinant activated factor VII has been Food and Drug Administration approved to treat hemorrhages in hemophiliac patients with inhibitors and in acquired hemophilia patients. Recombinant activated factor VII use has also been considered for the management of uncontrolled bleeding in a number of congenital and acquired hemostatic abnormalities. The myeloproliferative disorders are a group of clonal hematologic diseases where, frequently, abnormal platelet function is considered a hallmark. This is the first case report addressing the clinical benefit of off-label use of recombinant activated factor VII in an attempt to control intractable bleeding in a patient with a myeloproliferative disorder after splenectomy.     

L'atteinte du pied au cours du rhumatisme psoriasique. À propos de 26 cas

Objective. – To describe the clinical and radiological features of foot involvement in patients with psoriatic arthritis.Methods. – We retrospectively reviewed the medical records of patients admitted between 1972 and 1999 for psoriatic arthritis with involvement of the foot. We included all patients who had peripheral and/or axial, asymmetric, chronic inflammatory joint disease meeting or not Avila's radiological criteria for psoriatic arthritis, with or without other imaging findings suggestive of psoriatic arthritis and with or without psoriasis.Results. – Twenty-six patients were included. Inflammatory heel pain was reported by 14 patients, whereas forefoot involvement was found in only seven patients. Sausage toe was present in two patients. None of the patients had Bauer's toe (combining arthritis and psoriatic skin and/or nail changes) or psoriatic onychopachydermoperiostitis of the great toe. Radiological abnormalities were found in 20 patients. Half the patients had calcaneal changes. Osteoperiostitis of the great toe was noted in two patients and mushrooming in five.Discussion. – The features of psoriatic arthritis in Morocco seem similar to those in other countries. Hindfoot involvement was present in 53% of patients. Involvement of the forefoot was rarely recorded in the charts, suggesting missed cases because of insufficient attention to the forefoot during the physical examination and availability of anteroposterior radiographs only. Oblique views (Hirtz and Chaumet) should be obtained because they give a clearer image of the distal part of the toes, which is often difficult to analyze on anteroposterior films.     

Antimutagenic properties of bell and black peppers.

The wing Somatic Mutation And Recombination Test (SMART) in Drosophila melanogaster was used to study the modulating action of bell pepper (Capsicum annuum) and black pepper (Piper nigrum) in combination with the alkylating agent methyl methanesulfonate (MMS) and the promutagen agent ethyl carbamate (EC). Larvae trans-heterozygous for the third chromosome recessive markers multiple wing hairs (mwh) and flare-3 [flr(3)] were fed genotoxins alone or in combination with each of the two spices. Genetic changes induced in somatic cells of the wing's imaginal discs lead to the formation of mutant clones on the wing blade. Our results showed that bell pepper was effective in reducing the mutational events induced by EC and MMS and black pepper was only effective against EC. Pretreatment of 2-day-old larvae with the spices for 24 h followed by a treatment with EC and MMS was only effective in reducing mutations induced by EC. Suppression of metabolic activation or interaction with the active groups of mutagens could be mechanisms by which the spices exert their antimutagenic action.     

A lethal skeletal dysplasia with features of chondrodysplasia punctata and osteogenesis imperfecta: an example of Astley-Kendall dysplasia. Further delineation of a rare genetic disorder.

An unusual osteochondrodysplasia presenting with lethal neonatal short limbed dwarfism, defective ossification, and punctate calcification within cartilage is presented. The features resemble four cases previously described with Astley-Kendall dysplasia.     

Sevelamer hydrochloride with or without alphacalcidol or higher dialysate calcium vs calcium carbonate in dialysis patients: an open-label, randomized study.

BACKGROUND: Sevelamer hydrochloride was recently proposed as a phosphate binder to prevent hypercalcaemia in place of calcium alkaline salts in dialysis patients. So far, it has been evaluated only in patients receiving calcitriol, without comparison with CaCO(3) alone, although the latter was found to be as effective as the combination of calcitriol and Al(OH)(3) in suppressing parathyroid hormone (PTH) without inducing hypercalcaemia and to have a better lowering effect on serum phosphate. Moreover, this bile salt binder may decrease serum 25-OH vitamin D. Therefore, we compared for 5 months two strategies for controlling moderate hyperparathyroidism: CaCO(3) alone vs sevelamer in conjunction with measures to increase calcium balance. METHODS: Forty-two patients were randomized: 21 continued their treatment with 4.8 g/day CaCO(3) and 21 were switched to sevelamer (initial dose: 2.4 g/day, increased to 4.4 g/day). Each month, when serum-corrected calcium decreased below 2.30 mmol/l, dialysate calcium was increased or alphacalcidol was given at each dialysis session, according to serum PO(4) levels. The following parameters were monitored: serum Ca, PO(4), bicarbonate and protein, weekly; and serum PTH, 25-OH vitamin D and total, LDL and HDL cholesterol monthly. RESULTS: Except for higher serum phosphate at month 1, lower serum bicarbonate at month 2 and lower LDL cholesterol at month 5 in the sevelamer group, no difference was found between the two groups. Compared with baseline levels, PTH increased and 25-OH vitamin D decreased significantly in both groups, these two parameters being inversely correlated. CONCLUSIONS: Given comparable control of plasma calcium, phosphate and 25-OH vitamin D, PTH control is comparable in both strategies. Sevelamer does not induce greater vitamin D depletion than CaCO(3). The transient decrease of serum bicarbonate after discontinuation of CaCO(3) in the sevelamer group suggests a less optimal prevention of acidosis. The sevelamer-induced decrease in LDL cholesterol gives this drug a potential advantage in cardiovascular prevention.     

A rare case of nasopharyngeal angiofibroma in an elderly female

Nasopharyngeal angiofibromas occur predominantly in males in their puberty and adolescence; the incidence in other age groups and in women is exceptional. This report describes a case of a 57-year-old woman with nasopharyngeal angiofibroma presenting typical radiological findings in computed tomography, MR imaging and angiography. The tumour was successfully removed and histopathological examination confirmed the diagnosis. In 6 years follow-up the patient is free of symptoms.     

Development of an enzyme‐linked immunosorbent assay (ELISA) for the detection of porcine pepsin as a milk clotting enzyme

An enzyme‐linked immunosorbent assay (ELISA) for the detection of porcine pepsin in milk‐clotting enzyme preparations has been developed. The assay is capable of detecting porcine pepsin in the range 1 μgto 1 mg ml^‐1 without enhancement or modification. The specificity of the technique was studied by inhibition assay. Slight cross‐reactions with bovine rennet and Mucor miehei rennet occurred at high concentrations (1.0 mg ml^‐1). The ELISA used in this investigation appears to provide a quick, sensitive and specific method for the detection of porcine pepsin and has potential applications in the dairy industry.