Search PubMed⌕ Search

Biomedical subjects

A Taieb

Publications and source records attributed to A Taieb.

At least 37 records · Page 2Linked to original sources
PubMed1999

Usefulness of the European standard series for patch testing in children. A 3-year single-centre study of 337 patients.

Results of patch tests in 337 children aged 1 to 15 performed in our paediatric unit during the past 3 years have been analysed retrospectively in order to optimize the patch test series and to assess their relevance. This study represents the most important single-centre study reported so far over a short period. We found a positive patch test rate of 66%, with a peak incidence among children less than 3 years of age (88% versus 58.9%). The most common allergens were metals, especially nickel, fragrances and, less frequently, rubber chemicals. Concerning nickel, positive reactions rarely correlated with a relevant exposure and were difficult to interpret, especially in patients with atopic dermatitis, who are probably more likely to have irritant or false-positive reactions to metals. Based on the results and their relevance, we propose a shortened standard series of patch tests for paediatric patients.

Adolescent↗
PubMed1999

[Classification and management of mastocytosis in the child].

OBJECTIVE: Mastocytosis is a frequently observed condition in children. We analyzed the initial manifestations and clinical course. PATIENTS AND METHODS: We restrospectively studied 49 cases of mastocytosis in children (29 boys and 20 girls) managed in our unit between 1985 and 1995. All of the children had typical manifestations. Photographic documents were available in all cases. RESULTS: There was pigmentary urticaria in 32 cases and a mastocytoma in 17. Axanthelasmoid aspect and bullae were observed in some cases in both of these clinical forms. Complementary explorations demonstrated one case of duodenal mast cell infiltration. Excepting the case with skin and duodenal manifestation, all of our patients improved and clinical cure was obtained during growth. DISCUSSION: The association of dermal atopia and mastocytosis does not influence the clinical course of these two conditions. The development of bullae does not appear to be a factor of poor prognosis. The xanthelasmoid aspect of the lesions and the similar course in childhood mastocytosis and juvenile xanthogranulma would suggest that a common process with a histological spectrum including mastocytoma and xanthoma is involved. In our experience, counselling against the use of anti-cough medicines containing codeine is an essential part of management. Antihistamine agents may be prescribed for pruritus.

Biopsy↗
PubMed1998

A variable monilethrix phenotype associated with a novel mutation, Glu402Lys, in the helix termination motif of the type II hair keratin hHb1.

Monilethrix is a rare human hair disorder with autosomal dominant transmission that can be caused by mutations in hair keratins. Up until now, pathogenic mutations in the type II hair cortex keratins hHb6 and hHb1 were restricted to a highly conserved glutamic acid residue Glu413 (Glu117 of the 2B subdomains) in the EIATYRRLLEGEE helix termination motif of the two keratins. The critical glutamic acid residue was substituted either by a lysine or, less frequently, by an aspartic acid residue. Here we report a novel mutation in a French monilethrix family, which again consists of a lysine substitution of another highly conserved glutamic acid residue, Glu402 (Glu106 of the 2B subdomain), in the EIATYRRLLEGEE motif of hHb1. Family members bearing the hHb1 Glu402Lys mutation exhibit a particularly variable disease phenotype. The pedigree comprises two infant members, one with pronounced dystrophic alopecia, follicular keratosis, and clear-cut moniliform hair, and one with no hair loss at all and moniliform hair detectable only by electron microscopy, as well as an adult individual without any clinically or electron microscopically detectable symptoms, but with clear historical proof of the disease.

Adult↗
PubMed1998

[Infantile acne and isotretinoin].

UNLABELLED: We report a case of severe infantile cystic acne, which required a treatment with oral isotretinoin. CASE REPORT: The patient, a 22-month-old boy, presented cystic acne since 6 months of age. The child was otherwise healthy and endocrine evaluation was normal. Previous treatments had no effect, oral isotretinoine led to cure without secondary effects. COMMENTS: The etiology of infantile acne is not clearly defined, but may result from a persisting androgen-driven stimulation of sebaceous glands. Oral isotretinoin is safe and effective in case of recalcitrant infantile acne, but close monitoring is necessary because of the well-known side effects of oral retinoids.

Acne Vulgaris↗
PubMed1998

[Etiological work-up of complex voiding disorders (CVD) in boys; evaluation of the imaging strategy based on a retrospective study of 58 files].

The strategy of radiological investigations in males with severe voiding disorders has not been clearly established. To define the most effective strategy, a retrospective study of 58 files of boys investigated for severe voiding disorders (excluding neurogenic bladder) was performed. The following investigations were performed in this series: intravenous urography (IVU) completed by voiding cystourethrogram (VCUG) (41%), VCUG alone or associated with urinary ultrasound (30%), IVU alone (20.5%) ultrasound alone (1.5%) and ultrasound completed by VCUG or IVU (7%). When prescribed first, VCUG was always sufficient for accurate diagnosis; in contrast, a second investigation was usually necessary when IVU (66%) or ultrasound (80%) were performed first. The interpretability of the voiding urethrogram was also higher with VCUG (90%) than with IVU (66%); the sensitivity was 94% for VCUG and only 69% for IVU and 8% for ultrasound. This study confirms that VCUG combined with urinary ultrasound is the most reliable way to radiologically investigate male severe voiding disorders.

Adolescent↗
PubMed1997

Mutations in the hair cortex keratin hHb6 cause the inherited hair disease monilethrix.

Pathogenic mutations in a large number of human epithelial keratins have been well characterized. However, analogous mutations in the hard alpha-keratins of hair and nail have not yet been described. Monilethrix is a rare autosomal dominant hair defect with variable expression. Hairs from affected individuals show a beaded structure of alternating elliptical nodes and constrictions (internodes). These internodes exhibit a high prospensity to weathering and fracture. Strong evidence that trichocyte keratin defects might underlie this hair disorder was provided by genetic linkage analyses that mapped this disease to the type-II keratin gene cluster on 12q13. All affected individuals from a four-generation British family with monilethrix, previously linked to the type-II keratin gene cluster, as well as three unrelated single monilethrix patients, exhibited a heterozygous point mutation in the gene for type-II hair cortex keratin hHb6, leading to lysine substitution of a highly conserved glutamic acid residue in the helix termination motif (Glu 410 Lys). In a three-generation French family with monilethrix of a milder and variable phenotype, we detected another heterozygous point mutation in the same glutamic acid codon of hHb6, which resulted in a conservative aspartic acid substitution (Glu 410 Asp). These mutations provide the first direct evidence for involvement of hair keratins in hair disease.

Adolescent↗
PubMed1997

[Encephalocraniocutaneous lipomatosis].

BACKGROUND: Encephalocraniocutaneous lipomatosis (ECL) involving the scalp and cerebellum was observed without asymptomatic expression. CASE REPORT: A seven-month-old infant presented with two soft subcutaneous hairless tumors of the scalp without any associated clinical anomaly. Neuroradiology explorations (radiography, CT-scan and MRI) showed a lipoma in the cerebellum linked with a occipital cutaneous lipoma through a bone defect. At the age of 3 years, the child remains healthy. DISCUSSION: ECL is a rare neurocutaneous disorder that consists of skin lipomas associated with various cerebral anomalies. ECL may occur as a circumscribed form of the Proteus syndrome, since a few ECL patients have associated manifestations of proteus syndrome as well as localized hypertrophy. However, minor forms of ECL are possible and may be compatible with normal life.

Brain Diseases↗
PubMed1996

Contact allergy to aeroallergens in children with atopic dermatitis: comparison with allergic contact dermatitis.

Immediate and delayed cutaneous hypersensitivity are believed to be implicated in the physiopathology atopic dermatitis (AD). The purpose of this study was to evaluate Type I and Type IV allergy to aeroallergens in children with AD. 59 children (mean age 5.2 years), presenting with AD according to Hanifin and Rajka's criteria, were skin tested (patch and corresponding prick tests) with common environmental aeroallergens and a restricted panel of the European standard series over a 1-year period. History and clinical data were carefully recorded using a standardized evaluation sheet; total and specific IgE serum levels were evaluated. 17 of 59 patients (28.8%) had at least 1 positive patch test, 32 of 59 patients (54.2%) had at least 1 positive prick test. Corresponding patch and prick tests were observed in 8 out of 17 patients. 5 children with positive patch tests had negative prick tests. Irritant pustular reactions (2/59, i.e. 3%), "angry back" reactions (6/59, i.e. 10%) and doubtful reactions (3/59, i.e. 5%) were excluded from the positive group. Positive patch tests observed included, in decreasing order: D. pteronyssinus and D. farinae (26.8%), garden trees (12.2%), plantain (9.8%), timothy grass, mugwort and damp area trees (4.9% each), and orchard grass (2.44%). 6 children with positive aeroallergen patch tests and 11 children with negative aeroallergen patch tests had at least 1 positive patch test to standard allergens. All children with an irritant reaction to aeroallergens had no reaction to standard patch tests. The relevance of aeroallergens in upgrading the severity of AD lesions has still to be explored by challenge studies and by long-term follow-up.

Adolescent↗
PubMed1995

The gene for Bazex-Dupré-Christol syndrome maps to chromosome Xq.

Bazex-Dupré-Christol syndrome is an inherited condition with skin cancer predisposition characterized by follicular atrophoderma, hypotrichosis, and early onset of multiple basal cell carcinomas. Previous reports suggested an X-linked mode of inheritance. We therefore performed linkage analysis with microsatellite markers of the X chromosome in three families. We obtained evidence for X-linkage and regional assignment to Xq24-q27 of this syndrome (maximal lod score = 5.26 with a recombination fraction of 0% at the DXS1192 locus). This represents a first step towards the identification of a gene involved in hair follicle development and skin tumor formation.

Basal Cell Carcinoma↗
PubMed1995

[Juvenile xanthogranuloma: a congenital giant form].

INTRODUCTION: Juvenile giant xanthogranuloma (JGX) is usually a benign skin disease which regresses spontaneously in the new-born or infant. Characteristic firm yellow-orange papulonodules lead to diagnosis. The nodules vary in size from a few millimetres to 1 to 2 centimetres. We observed an exceptional case presenting as a congenital giant form. CASE REPORT: A new-born girl had a bright red lesion of the right inguinal region measuring 4 x 2.5 cm. Inguinal node enlargement was found homolaterally. Histological examination of a biopsy specimen gave the diagnosis of JGX. There was no extension. The lesion and node involvement regressed spontaneously leaving a xanthomization of the skin. DISCUSSION: Clinical forms of JGX are rarely described. The large size of these lesions does not apparently affect the clinical course nor associated visceral involvement which remains exceptional. This type of lesion increases in size more rapidly and raises the problem of differential diagnosis requiring biopsy to eliminate other neonatal tumours with a less favourable prognosis. Spontaneous regression of JGX is the rule allowing simple regular surveillance.

Female↗
PubMed1994

[Microsporum langeronii dermatophytosis in a newborn infant contaminated in France].

Dermatophytosis due to Microsporum langeronii was observed in a 21-day neonate born in Bordeaux, France to a mother of West African origin. A typical misleading manifestations were: non alopecic squamation of the scalp with seborrhoea associated with circineous vesiculo-squamous lesions of the forehead. The source of the contamination was undoubtedly the mother who presented squamation without alopecia of the scalp. M. langeronii were observed in the scales and the hair. Epidemiological search for tinea should be carried out in the family as well as in schools attended by the brothers and sisters. Familial contamination is more frequent.

Dermatomycoses↗