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Biomedical subjects

N Belmatoug

Publications and source records attributed to N Belmatoug.

At least 19 recordsLinked to original sources
PubMed2006

Schnitzler's syndrome: 3-year radiological follow-up.

Schnitzler's syndrome comprises urticaria, monoclonal gammapathy, inflammatory signs (fever, enlarged lymph nodes, hyperleukocytosis), and bone lesions. We report the imaging findings and follow-up of a new case with extensive osteosclerosis of the iliac bone, associated with inflammatory signal changes on MRI and foci of increased uptake on bone scintigraphy. When a diagnosis of Schnitzler's syndrome is established, treatment should be towards symptoms only; a long follow-up is warranted, as a delayed evolution towards a lymphoproliferative syndrome is possible.

Diagnosis, Differential↗
PubMed2004

Langerhans' cell histiocytosis of the labyrinth in adults.

OBJECTIVE: To describe and analyze three unusual cases of Langerhans' cell histiocytosis of the temporal bone in adults. STUDY DESIGN: Retrospective case review. SETTING: A tertiary referral center. PATIENTS: Three adult patients with progressive sensorineural or mixed hearing loss, vertigo, and tinnitus as presenting symptoms of Langerhans' cell histiocytosis of the temporal bone. INTERVENTION: Patients were evaluated by means of computed tomography and magnetic resonance imaging. All patients underwent complete surgical excision of the lesion via a transmastoid approach, extended to a translabyrinthine approach in one case. One patient with a multifocal disease underwent excision of a mandibular lesion 1 year later, followed-up by chemotherapy. RESULTS: The Langerhans' cell histiocytosis was located adjacent to or within the area of the endolymphatic sac region in all cases. The middle ear was spared. Hearing function was preserved in the two patients who had serviceable hearing preoperatively. No complication occurred. CONCLUSION: Langerhans' cell histiocytosis of the temporal bone is centered on or adjacent to the endolymphatic sac. The fact that the endolymphatic sac plays an immunologic role in the inner ear suggests that the infiltration of the Langerhans' cell may derive from it.

Adult↗
PubMed2003

Two cases of cerebral aspergillosis successfully treated with voriconazole.

Described here are two cases of cerebral aspergillosis successfully treated with voriconazole after the failure of first-line treatment with amphotericin B deoxycholate. In both cases, clinical and radiological cure was achieved within 6 weeks. Voriconazole should be considered as first-line therapy for cerebral aspergillosis.

Administration, Oral↗
PubMed2003

[Gaucher's disease in children: first clinical signs, natural course and benefits of enzyme replacement therapy].

OBJECTIVES: Gaucher's disease is a rare genetic disease. Paediatric patients present with highly severe disease and, until now, literature does not provide many descriptions in children only. A specific enzyme replacement therapy has been available for the last decade. Quality of life has been improved by the treatment. POPULATION AND METHODS: We studied 17 Gaucher patients (14 type 1 and 3 type 3) diagnosed before the age of 15 and treated afterwards. The first clinical signs and the course of the disease are described before and during treatment. To appreciate the impact of the treatment on quality of life, we established a clinical score with the functional symptoms of the patients. RESULTS: The mean age was 6.4 years at diagnosis and 18.3 years at the beginning of the treatment. With specific treatment, all clinical and biological symptoms dramatically improved but some specific organic damages were irreversible. CONCLUSION: The comparison of our patients with other reported paediatric cases confirm that Gaucher's disease in children is severe and has to be precociously diagnosed and treated. Clinical scores appeared to be helpful to choose the moment for treating and to follow-up the patients under treatment.

Adolescent↗
PubMed2003

The role of the iminosugar N-butyldeoxynojirimycin (miglustat) in the management of type I (non-neuronopathic) Gaucher disease: a position statement.

N-Butyldeoxynojirimycin (NB-DNJ, miglustat 'Zavesca') is an orally active iminosugar which inhibits the biosynthesis of macromolecular substrates that accumulate pathologically in glycosphingolipidoses. Clinical trials of NB-DNJ in patients with Gaucher's disease demonstrate the therapeutic potential of such substrate inhibitors in the glycolipid storage disorders. However, macrophage-targetted enzyme replacement using intravenous mannose-terminated human glucocerebrosidase (imiglucerase, Cerezyme) is highly effective in ameliorating many of the manifestations of Gaucher's disease and is a treatment in widespread use. Given that imiglucerase and miglustat are now both licensed for the treatment of Gaucher's disease, there is a need to review their therapeutic status. Here the treatment of type 1 (non-neuronopathic) Gaucher disease is evaluated with particular reference to the emerging role of oral N-butyldeoxynojirimycin (miglustat) as a substrate-reducing agent. This position statement represents the consensus viewpoint of an independent international advisory council to the European Working Group on Gaucher Disease.

Clinical Trials as Topic↗
PubMed2001

Somatostatin receptor scintigraphy and gallium scintigraphy in patients with sarcoidosis.

UNLABELLED: Somatostatin receptor scintigraphy (SRS) has been shown to reveal sarcoidosis sites. The aim of this study was to prospectively compare SRS and gallium scintigraphy in the evaluation of pulmonary and extrapulmonary involvement in patients with proven sarcoidosis. METHODS: Eighteen patients with biopsy-proven sarcoidosis were included. Nine were or recently had been receiving steroid therapy at the time of the examination. Planar gallium scintigraphy (head, chest, abdomen, and pelvis) and thoracic SPECT were performed at 48-72 h after injection of a mean dose of 138 +/- 21 MBq 67Ga. Planar SRS and thoracic SPECT were performed at 4 and 24 h after injection of a mean dose of 148 +/- 17 MBq 111n-pentetreotide. RESULTS: Gallium scintigraphy found abnormalities in 16 of 18 patients (89%) and detected 64 of 99 clinically involved sites (65%). SRS found abnormalities in 18 of 18 patients and detected 82 of 99 clinically involved sites (83%). Of the 9 treated patients, gallium scintigraphy found abnormalities in 7 (78%), detecting 23 of 39 clinically involved sites (59%), whereas SRS found abnormalities in 9, detecting 32 of 39 clinically involved sites (82%). CONCLUSION: This study suggests that, compared with gallium scintigraphy, SRS appears to be accurate and contributes to a better evaluation of organ involvement in sarcoidosis patients, especially those treated with corticosteroids.

Adrenal Cortex Hormones↗
PubMed2001

Deflazacort versus prednisone in patients with giant cell arteritis: effects on bone mass loss.

OBJECTIVE: To compare bone mass loss due to deflazacort versus prednisone in longterm treatment of patients with giant cell arteritis (GCA) in a randomized double blind comparative trial. METHODS: Seventy-four patients were included in a prospective multicenter study. Half received deflazacort (DFZ) and the other half prednisone (PR) for a minimum of 12 months. Calcium and vitamin D supplements were also provided to all subjects. Our intent was (1) to evaluate bone mineral density, using dual energy x-ray absorptiometry, at baseline and comparatively at 3, 6, and 12 mo; vertebral fractures by Meunier score and size variations after 12 mo treatments were also analyzed; (2) to assess calcium/phosphate metabolism modifications in both groups at baseline and after 12 mo. RESULTS: No significant difference was observed between the 2 groups in terms of treatment efficacy. Patients taking PR were slightly older on average versus the DFZ group (74 vs 70 yrs). Bone mass loss between entry and month 12 was not statistically different in the PR group (-0.026 +/- 0.007 g/cm2) compared to the DFZ group (-0.03 +/- 0.005 g/cm2). No significant difference was found in Meunier score variations (0.77 and 1.18 in the PR and DFZ groups, respectively; p = 0.3), nor in vertebral size variations (-0.4 and -0.2 in the PR and DFZ groups, respectively; p = 0.4). There was no difference in calcium/phosphate metabolism evaluations at month 12. CONCLUSION: In older patients taking longterm glucocorticoids who are at risk of osteoporosis, deflazacort did not result in less bone loss than prednisone.

Absorptiometry, Photon↗
PubMed2001

[Adult Gaucher disease].

PURPOSE: This review presents the clinical, biological and therapeutic aspects of adult Gaucher disease. CURRENT KNOWLEDGE AND KEY POINTS: Gaucher disease is an uncommon inborn recessive autosomal disease, due to a deficient activity of the lysosomal enzyme beta-glucocerebrosidase. The enzymatic defect leads to the accumulation of the lipid glucocerebroside in liver, spleen and bone marrow. Patients with Gaucher disease have been classified into three types. Type 1 is the most frequent and non-neuronopathic, with enlarged liver and spleen and bone damage. Biological abnormalities are found: visceral infiltration (pancytopenia, cholestasis), macrophage damage (angiotensin-converting enzyme increase, ferritin increase, immunoglobulin increase, hemostasis abnormalities) and lysosomal damage (acid phosphatase tartrate-resistant increase, chitotriosidase increase). Enzyme replacement therapy has become available, has proven effectiveness in many patients and has successfully reversed many of the manifestations of the disorder. FUTURE PROSPECTS AND PROJECTS: The new biological abnormalities must help in treatment management. Gene transfer and oral treatment must be taken into account and validated in large clinical studies.

Adult↗
PubMed2000

[Outcome of pregnancies in lupus: experience at one center].

We determined the outcome of all pregnancies in SLE patients in our lupus cohort between 1991 and 1997. The women were advised that pregnancy was acceptable if the disease had been inactive for 6 months (SLEDAI < or = (4 at 2 serial examinations) and daily prednisone dose was below 10 mg. Patients were advised against pregnancy in case of active nephritis or neurolupus. In case of antiphospholipid antibodies, patients were treated with aspirin or heparin if previous fetal losses were documented. In case of anti-SSA ab, patients were monitored with ultrasound and given dexamethasone in case of atrioventricular block. Fifty-nine pregnancies were registered among 31 women: mean age at diagnosis of SLE was 25.3 +/- 3.7 years (range: 17-31); mean disease duration before pregnancy 4.4 +/- 3 years (0-14); mean ACR score 5.4 +/- 1.5 (4-9). Seven patients had ACL ab, 8 had anti-SSA ab. Pregnancies ended in: 13 early spontaneous abortions (9 not related to disease flare up, 4 related to SAPL); 7 elective abortions (patient decision in 5 cases, severe lupus flare up in 2); one in utero death; 19 full term births (> 38 weeks); and 19 preterm births. Cesarean section was performed in 11 cases (6 for fetal distress, dystocia and previous ceasarian; 5 for active lupus). Severe sepsis occurred in one premature infant who died at the age of 1 week. Intrauterine growth retardation was observed in 11 cases, mean APGAR score was 8.9 +/- 1.43. Child development was normal in all cases except one child with mild mental retardation. Severe lupus flare ups occurred in 6 cases, of which 4 were pregnancies in unadvised situations. Six mild flare ups were documented in the post partum. One fatal case of neonatal lupus with AVB was observed. In conclusion, in our experience, the live birth rate is similar to the general population and the risk of lupus flare up is low when the above mentioned criteria are applied. Systematic increase of steroid dose at pregnancy onset does not seem to be necessary. The high rate of prematurity remains a problem to be solved.

Adult↗
PubMed1997

[Inflammatory amyloidosis].

Inflammatory amyloidosis consists of AA protein. In developed countries, its predominant causes are chronic inflammatory rheumatism (rheumatoid arthritis, chronic juvenile arthritis, spondylarthropathy, etc.). More rare are other chronic inflammatory disorders (Crohn's disease, ulcerative colitis, Whipple's disease, etc.), hemopathies and neoplasia. In developing countries, infectious causes are the most common (tuberculosis, leprosy, chronic bacterial suppuration). Other infectious diseases complicated with amyloidosis are less common (bronchial dilatation, mucoyiscidosis, heroin injection-related skin suppuration). In chronic infection, amyloidosis is observed a mean of 10 years after the beginning of the triggering disorder and is mainly localised in the kidney, the liver and the gastrointestinal tract. The course is most often slow and prolonged survival has been reported.

Amyloidosis↗
PubMed1997

Pacemaker endocarditis due to Candida albicans: case report and review.

We describe a case of pacemaker endocarditis due to Candida albicans in a patient who responded favorably to combined surgical and antifungal therapy. Only five cases of candidal pacemaker endocarditis have been reported previously. We review these five cases and discuss the clinical presentation and therapy for this disease in comparison with candidal prosthetic valve endocarditis.

Candida albicans↗
PubMed1997

[Manifestations and diagnosis of extrapulmonary tuberculosis].

Extra pulmonary tuberculosis currently represents 25 per cent of notified tuberculosis in France. All organs can be involved but the most frequent localisation are the lymph nodes. Immunodepressed patients notably those infected with HIV are particularly prone to a polyvisceral tuberculous infection. The diagnosis is often difficult on account of the polymorphism of the disease and the weak specificity of the clinical manifestation. A firm diagnosis always rests on histological or microbiological evidence. Certain recent developments should enable a more rapid microbiological diagnosis notably as a result of development in molecular biology and rapid culture techniques. The mortality and morbidity remain elevated in developing counties as in industrialised counties and clinical situations in which extra pulmonary tuberculosis occur often worsen the prognosis.

Bacteriological Techniques↗