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Management of severe atopic dermatitis.

The lack of knowledge concerning the pathophysiology of atopic dermatitis (AD) explains the absence of any specific treatment specially in severe atopic dermatitis. New treatments were recently suggested for the management of the disease. They all act on some component of the immune mechanisms which provoke the eczematous reactions. Among recent treatments proposed, I will discuss the use of cyclosporin A, puva therapy, thymopoietin and thymostimulin, antifungal therapy, alpha and gamma interferon, and treatment with interleukin 2.

Cyclosporine↗

[Bejel: an unusual cause of stomatitis in the child].

INTRODUCTION: Bejel (endemic syphilis) is usually encountered in children living in intertropical areas, although imported cases have been exceptionally reported in Europe. OBSERVATION: We report on a young girl aged 5 diagnosed in France, who had painless stomatitis and enlarged cervical nodes. Diagnosis of bejel was confirmed by serology, and spirochetes were shown inside mucous patches by biopsy specimen silver staining. Favorable clinical and serological outcome occurred following benzathin-penicillin therapy. Contamination is likely to have occurred in Mali where she had been living for several years, but secondary stage manifestations only appeared in France, where she was living for several months. DISCUSSION: Although rare in France, bejel should not be overlooked in children originating from countries where endemic syphilis has a high prevalence.

Biopsy↗

[Use of an ion exchanger as a rapid means of extraction, applied to the determination of urinary estrogens and pregnanediol in pregnancy, using colorimetry or gas chromatography].

The method described permits, after classical hydrolysis, extraction without a solvent of estrogens during pregnancy. It also permits estimation of pregnanediol which is eluted in a different fraction. Twenty estimations may be carried out in half a day on test samples of from 2 to 10 ml of urine. The estimation of total estrogen by colorimetry takes into consideration the efficiency of extraction by the use of a standard range treated under the same conditions. For gas chromatography, two internal standards are added to the urine after hydrolysis: these are 16,17-epiestriol and 5 beta pregnane-3 alpha, 17 alpha, 20 beta triol (PGTS) have respectively the same behaviour as estriol on the one hand and pregnanediol and pregnanetriol, on the other hand. They take into consideration resin extraction and chromatographic behaviour and may be used for quantitative estimation. The graphs of standardisation and recovery have been studied for the main estrogens together with pregnanediol. The various eluates were controlled by gas chromatography coupled to mass spectrometry.

Chromatography, Gas↗

Proton MR spectroscopy of the brain.

Over the past five decades, MR spectroscopy has evolved from an analytical chemistry tool to a noninvasive clinical examination on FDA approved equipment with an AMA billing code. While proton MR spectroscopy has dominated current clinical studies, interest in other nucleii has arisen, particularly P-31 for the evaluation of membrane lipids, and C-13 for the evaluation of glutamate neurotransmission and excitotoxicity. Currently, the most common neuroradiological diagnostic indication is the differentation of suspected cerebral neoplasms for post-treatment effects, abcesses, subacute infarcts, demyelinating disease, and other non-neoplastic processes. Recent recommendations for monitoring multiple sclerosis treatment suggest an increasing role for MR spectroscopy in the future.

Brain↗

Is it possible to predict the efficacy at discharge of inhospital rheumatology department management of disk-related sciatica? A study in 150 patients.

OBJECTIVES: To identify criteria for predicting the outcome at discharge in patients scheduled for inhospital treatment of disk-related sciatica. PATIENTS AND METHODS: We studied 150 rheumatology department patients admitted for disk-related sciatica with a mean duration of 88 +/- 127 days to determine the impact on treatment outcomes of 50 parameters. RESULTS: After complete bed and 2.2 +/- 1.1 epidural glucocorticoid injections during a mean hospital stay of 9.9 +/- 4.3 days, 80% of patients reported either complete or partial pain relief (19% and 61%, respectively). The remaining 20% reported little (9%) or no (11%) change. Surgery was performed in 13% of cases. A tighter straight-leg raising test angle was correlated with treatment failure (P = 0.01). Complete bed rest duration prior to admission was shorter in the 80% of responders (P = 0.036) than in the 20% of nonresponders; in the overall population, patients spent on average 64% +/- 33% of daytime hours in bed. Patient predictions about their own outcomes were unreliable (P = 0.926). Patients who believed strongly that sciatica requires surgical treatment were more likely to be nonresponders (P = 0.06), as were patients with a family history of surgically-treated sciatica (P = 0.055). Outcomes were not correlated with any of the other parameters studied, including sciatica duration (P = 0.13), bedrest duration prior to admission (P = 0.52; mean duration, 18 +/- 29 days), the specialty of the physicians seen, investigations done prior to admission, or a history of sciatica (noted in 65% of cases and treated surgically in 16%). CONCLUSIONS: Eighty percent of patients benefited from their hospital stay. Patient opinions on the merits of nonsurgical and surgical treatments in their own case were not correlated with their own outcomes. A tight straight leg-raising test angle was correlated with a poorer outcome. Neither sciatica duration nor rest duration prior to admission had an influence on outcome. Absence of complete bed rest prior to admission was correlated with a greater likelihood of a therapeutic benefit from the hospital stay.

Adolescent↗

Multiple rheumatoid bursitis with migrating chylous cysts. Report of a case in a European woman and review of the literature.

We report a case of recurrent multiple bursitis (19 episodes at nine sites) requiring seven surgical procedures in a European women with a 38-year history of severe, nodular, destructive seropositive rheumatoid arthritis unresponsive to second-line drugs. The episodes of bursitis were not correlated with activity of the joint disease. Some cysts migrated over a considerable distance. At least two cysts contained chylous fluid. The histologic study of one cyst demonstrated a cholesterol crystal granuloma. Potential relationships linking cholesterol crystals, chylous cysts, and migrating multiple bursitis are discussed. The relevant literature is reviewed.

Aged↗

Skin elastic fibers in Williams syndrome.

The elastin gene is consistently deleted in Williams syndrome and as this protein represents the major component of the elastic fibers of the dermis, we sought to investigate skin elastic fibers in Williams syndrome as a key to unraveling extracellular matrix disorganization in this condition. Both morphometric parameters analyzed by using automated image analysis and immunofluorescence labeling with monoclonal antibodies against elastin and fibrillin 1 showed a disorganized pre-elastic (oxytalan and elaunin) and mature elastic fibers in the dermis of 10 Williams syndrome patients compared with five healthy children and one patient with isolated supravalvular aortic stenosis. Skin biopsies in Williams syndrome patients provide a simple mean to elucidate extracellular matrix anomalies. Hopefully, this method could give clues to the understanding of the elastic network anomalies in this condition and even to the consequences of these latter on elasticity and resilience of other tissues such as the arterial tree.

Adolescent↗

[Scleroderma in children: a retrospective study of 70 cases].

BACKGROUND: Scleroderma is uncommon in childhood. The aim of our study was to analyze the frequency of different clinical forms, their prognostic significance, biological features, and co-morbidities and to assess the pertinence of therapeutic options. PATIENTS AND METHODS: The files of 70 children with primary scleroderma seen from 1980 to 1997 were retrospectively reviewed. RESULTS: Localized scleroderma was observed in 56 children and diffuse lesions in 14. Localized scleroderma (44 girls, 12 boys) began early at a mean age of 7 years 2 months. The lesions presented as isolated bands (39 p. 100), associated with morphea (36 p. 100), or multiple morphea (5 p. 100). Mean duration of the clinical course was longer in cases with more and deeper lesions. Eosinophilia was observed at onset in 38 p. 100 of the cases and antinuclear antibodies were found in 28 p. 100. Local corticosteroid therapy (level I or II) appeared to be useful in the superficial and active lesions (morphea) but did not halt progression to deep scleroderma. General corticosteroid therapy (1 mg/kg/24 h) did not prevent the development of sequelae in cases with bands (16/16). Diffuse scleroderma corresponded to systemic scleroderma (6 cases), dual morbidity (dermatomyositis, mixed connective tissue disease) (6 cases), or scleroderma after eosinophil fasciitis (2 cases). Age at onset was around 9 years with female predominance. A particular gloves and socks form was observed and cardiac involvement was common, but there was no case of renal involvement. The therapeutic problems were similar to those in adults. DISCUSSION: Our findings emphasize that scleroderma occurs readily in childhood, unlike what has been reported 10 years ago. Prognosis depends on functional impairment resulting from major sequelae particularly important in localized forms and the life-threatening situations occurring in systemic forms.

Adrenal Cortex Hormones↗

[Prognostic value of preoperative electromyography for outcome of lumbosacral radiculopathy of discal origin].

OBJECTIVES: To assess the prognostic value of preoperative electromyography (EMG) for long-term outcome in patients who undergo surgery for discal radiculalgia at the lumbar level. PATIENTS AND METHODS: Thirty-seven patients who underwent surgery less than 6 weeks after the EMG were selected among a pool of 384 patients suffering from discal radiculalgia at the lumbar level who had had an EMG at our hospital since 1980. Among these 37 patients, 30 were contacted by telephone for an interview on outcome after a mean 42.1 +/- 22 month follow-up. RESULTS: Five out of 30 patients reported limitation for walking, 8/30 motor weakness, 14/30 had sensorial deficits, 12/30 had frequent or incapacitating radiculalgia, and 16/30 reported paresthesia. Patients were more satisfied with pain outcome (good or very good results in 22/30 patients (73%)) than with functional outcome (good or very good results in 20/30 patients (66%)). Preoperative EMG abnormalities (observed in 22/30 cases) were not correlated with patient age or duration of sciatica. The patients whose preoperative EMG was abnormal did not later report more paresthesia (11/20 versus 5/10) or severe radiculalgia (7/20 versus 5/10) than the others. Conversely, they complained more frequently of motor weakness (7/20 versus 1/10), but their global opinion about long-term outcome of surgery was not worse good or very good results for 15/20 patients with abnormal EMG (75%) versus 7/10 (70%) of those whose preoperative EMG was normal. CONCLUSION: This study shows that a normal EMG does not preclude surgical cure of discal sciatica. The results of preoperative EMG do not predict the long-term outcome after surgery: indeed they are only correlated with motor weakness (7/20 versus 1/10), but not with residual pain and functional outcome.

Adult↗