[Allergy emergencies, management and prevention].
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Biomedical subjects
Publications and source records attributed to S Guez.
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INTRODUCTION: Familial occurrence of progressive systemic sclerosis is unusual. The occurrence of conjugal scleroderma is exceptional. EXEGESIS: We report here a case of systemic sclerosis in a wife and husband who both developed the onset of illness within a 10-year period. Solvent exposure was noted. CONCLUSION: The etiology of systemic sclerosis remains unknown. Environmental factors may play role in its pathogenesis.
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Bone mass is primarily genetically determined, but exogenous factors also play a major role. The prevention of osteoporosis can start from childhood, and optimal achievement of peak bone mass during childhood and adolescence is important in order to minimise future fracture risks. Chronic inflammatory diseases can have a detrimental effect on bone mass, by means of several mechanisms. Different diagnostic methods for detection and monitoring of osteoporosis are in use or under investigation. The role of calcium and vitamin D supplementation for the prevention and treatment of osteoporosis associated with paediatric rheumatic diseases remains to be established. New treatments such as bisphosphonates and calcitonin are now available, although their use in the paediatric age has been limited.
INTRODUCTION: Primary Sjogren syndrome is considered as the most frequent connective tissue disease. Neurological complications may affect the peripheral nervous system and to lesser extent the central nervous system. Autonomic system nervous dysfunction and epilepsy have been rarely reported. EXEGESIS: We present on case of Sjogren's syndrome with epilepsy and autonomic nervous system dysfunction. The epilepsia respond to valproate. CONCLUSION: Autoimmune investigations for Sjogren's syndrome should be initiated in any patient presenting with unexplained neurologic manifestations.
INTRODUCTION: Hereditary hemochromatosis is inherited as an autosomal recessive trait. It is characterized by increased absorption of dietary iron. The association between pernicious anaemia and hereditary hemochromatosis has never been described. EXEGESIS: We report a case of paradoxical association of hereditary hemochromatosis and pernicious anaemia. CONCLUSION: It seems that pernicious anaemia may prevent manifestations of hemochromatosis. We suppose that this protective role is due to atrophic body gastritis with iron malabsorption.
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BACKGROUND: Mycobacterium marinum has been recognized for some forty years. It generally occurs after trauma in a patient who manipulates tropical fish living in an aquarium. CASE REPORT: We report the case of an exotic fish seller who developed M. marinum nodular lymphangitis after being bitten by one of the fish. DISCUSSION: Nodular lesions occurring after trauma in subjects living in France who are in contact with exotic fish suggest the diagnosis of M. marinum. Nodular lymphangitis is seen in about 50% of the cases. Prolonged antibiotic therapy is required. Several antibiotics are effective including minocycline used in our case for 2 months after complete healing of the wound. Surgery is exceptionally required.
BACKGROUND: The safety and efficacy of sublingual-swallow immunotherapy (SLIT) in rhinitis caused by house-dust mite were evaluated in a double-blind, placebo-controlled study including 75 patients for 24 months. METHODS: Patients received either placebo or SLIT with a standardized Dermatophagoides pteronyssinus (D.pt.) - D. farinae (D.f) 50/50 extract. The mean cumulative dose was 90,000 IR, equivalent to 2.2 mg of Der p 1 and 1.7 mg of Der f I. Symptom and medication scores were assessed throughout the study. Exposure to house-dust mite, skin sensitivity, and serum specific IgE and IgG4 were assessed before starting treatment and after 12 and 24 months. RESULTS: Seventy-two patients (36 active-36 placebo) were eligible for intent-to-treat analysis. Thirty-six patients dropped out of the study. The number of patients who dropped out due to lack of efficacy was eight out of 37 (21.6%) in the active treatment group compared to 15 out of 38 (39.5%) in the placebo group (chi-square=2.81, P=0.09). Total symptom and medication scores decreased significantly after 12 and 24 months (P<0.05) of treatment in both groups, but no significant difference was observed between the active and placebo groups. After 24 months, the number of patients with high levels of indoor allergenic load decreased significantly in both groups compared to baseline data (P=0.01). Specific IgE (D.pt. and D.f.) increased significantly in the active treatment group after 12 and 24 months, while no change was observed in the placebo group. Specific IgG4 levels were not significantly modified in either group. Two patients in each group reported mild adverse effects. No severe adverse effects were reported. CONCLUSIONS: We conclude that SLIT in rhinitis caused by house-dust mite was safe, but there was a lack of consistent clinical benefit compared to placebo, probably due to the impact of the allergen avoidance measures that lowered the allergen burden.
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A 30-year-old woman developed progressive left sided hemiparesis with intracranial hypertension signs. CT scan and MRI showed a large temporo parietal cystic mass with marked surrounding edema. Surgical excision was performed, and histological analysis revealed an inflammatory granuloma. No disease elsewhere was found and all classical causes of granulomas such as tuberculosis, toxoplasma, fungus infections, inflammatory diseases, lymphomas and cancers were excluded. No treatment was administered and she remained neurologically stable for two years. Afterwards, she developed chronic meningo-encephalitis, hypothalamic-pituitary dysfunction and hydrocephalus requiring decompression. Sarcoidosis was suspected, a steroid therapy was initiated, she gradually improved and a ventricular biopsy confirmed this diagnosis. Nervous system lesions complicate the course of sarcoidosis in 5 to 15% of patients and most commonly involve the cranial and peripheral nerves. CNS involvement is typically meningeal with a predilection for the hypothalamic region. Intracranial mass lesions are rare and their occurrence in the absence of disease elsewhere is still more unusual. Three presentations have been described: an isolated intra parenchymatous mass, multiples nodules, and subdural plaques, that can be mistaken for meningiomas, gliomas or metastases. When systemic manifestations of sarcoidosis are absent, the diagnosis is difficult, and Gd-enhanced MRI is now considered the diagnostic method of choice. However brain biopsy is sometimes necessary. Corticosteroids are the mainstay of therapy. Immunosuppressive agents are also used and brain irradiation has been tried in some refractory cases. Surgical approach may be indicated to establish tissue diagnosis, to perform decompression and to remove brain tumors.
The combination of captopril and indomethacin has been shown to control nephrotic proteinuria in an infant with congenital nephrotic syndrome of the Finnish type. We report the satisfactory control of congenital nephrotic syndrome by enalapril, maintaining normal serum albumin levels without albumin infusions. The haplotype data of our patient were consistent with the diagnosis of a Finnish-type nephrotic syndrome. After 21 months, during which daily infusions of albumin allowed partial control of the symptoms, captopril treatment was started. No adverse effects were noted. Serum creatinine levels remained normal. Within 8 weeks, albumin infusions were completely stopped. After 1 month the treatment was changed to a single dose of enalapril (0.8 mg/kg per day). During the next 15 months, the serum protein concentration was maintained around 6.5-7 g/dl, although proteinuria persisted (0.3-0.5 g/day). Weight and length gain are now satisfactory. We conclude that enalapril may be safely used in infants with severe forms of congenital nephrotic syndrome and might allow the avoidance of aggressive treatments for prolonged periods.
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INTRODUCTION: Cadmium is a carcinogenic substance and bronchial and prostatic cancer may be readily identified as professional diseases. It is also a nephrotoxic substance but renal carcinoma are rarely imputed to this kind of intoxication. CASE REPORT: We report the observation of a 59-year-old woman with a long unprotected professional exposure to cadmium. She developed an epidermoid carcinoma of the kidney and later of the lung. Levels of cadmium in blood samples were abnormally high, confirming the existence of a chronic impregnation. CONCLUSION: We have judged cadmium to be responsible for her renal disease: the fact of a long unprotected professional exposure, the scarcity of renal epidermoid carcinoma, the well established nephrotoxicity of this substance and the association with other cancers, appear to us as convincing evidence, and the kidney cancer of our patient has been considered a professional disease.
OBJECTIVE: To determine: (i) the proportion of vesicoureteric reflux (VUR) associated with congenital renal damage and whether it can be severe enough to cause renal impairment from birth: (ii) to evaluate the distribution of males and females affected; and (iii) to describe the course of congenital damage in the first years of life. PATIENTS AND METHODS: A total of 108 children (76 male and 32 female, M:F 2.3:1), whose VUR was diagnosed before any infection, were followed from birth for a mean (range) of 4.3 (1-10) years. Renal damage was defined by serum creatinine concentration, creatinine clearance and renal imaging (ultrasonography and renal scintigraphy) performed within the first month of life and periodically thereafter. RESULTS: Of the 108 children, 58 had bilateral and 50 unilateral reflux (total number of refluxing units, 166). High-grade VUR (grade > or = 4) was found in 96 (58%) refluxing renal units (RRUs). Males had a prevalence of bilateral severe (> or = grade 4) reflux (M:F 5.2:1), while in those wit unilateral VUR, the M:F ration was 1.5:1. At birth, mild to moderate damage was present in 56 (36%) RRUs and only associated with VUR of grade > or = 3. Bilateral reflux of grade > or = 4 was associated with congenital moderate/severe renal failure in nine neonates (seven males). In infants with grade > or = 4 VUR who underwent surgical correction, VUR resolved in 92% of cases. In infants with VUR of grade > or = 4 followed medically, the reflux spontaneously resolved in 42% and ameliorated in 16% after 18 months. Serial renal scans during the follow-up showed no progression of renal damage. CONCLUSIONS: VUR diagnosed at birth on prenatal ultrasonography is associated with congenital damage, with males affected more often than females. The damage involves both kidneys in a consistent proportion and is an important cause of chronic renal impairment from birth. It does not progress in the first years of life if infections are prevented. It is suggested that males with this condition may constitute a major group at risk of developing chronic renal failure in later life.