Aspirin-ticlopidin in Kasabach-Merritt syndrome.
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Biomedical subjects
Publications and source records attributed to A Taieb.
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Rhenwald and Green's technique is currently the standard method for growing stratifying epidermal cell cultures. The serum free system developed in Ham's laboratory (MCDB 153) was designed to grow keratinocyte monolayers in clonogenic conditions. Our aim was to optimize conditions in serum-free MCDB 153 for culturing epidermal sheets from adult normal skin, and to assess the effect of extracellular calcium and temperature on proliferation and differentiation of cultured keratinocytes. Sixteen strains derived from plastic surgery specimens (mean age of donors 37 years; range 5-89) were used. Primary cultures were seeded at an optimal density of 8 x 10(4) cells/cm2 in primary cultures and 10(4) cells/cm2 in secondary cultures in complete medium including EGF, insulin, hydrocortisone and bovine pituitary extract, supplemented with isoleucine, tyrosine, methionine, phenylalanine, tryptophane and histidine. Amino acid (AA) supplementation allows a 5.8-fold increase in cell counts at confluency and monolayers with densely packed cells are obtained. In AA supplemented cultures, confluency is obtained in 16 +/- 3 days in primary cultures and in 13 +/- 0.5 days at first passages. Switches to 1.1 mM calcium at first or second passages resulted in a significant increase in cell counts (P less than 0.001), when compared with AA supplemented low calcium cultures. Low temperature/low calcium cultures resulted in a 50% decrease in cell counts. Low temperature/high calcium cultures gave similar cell counts as the 37 degrees C controls. AA and calcium supplemented cultures were evaluated for differentiation markers: involucrin expression was increased, keratins 5, 6, 14, 17 were expressed, and the sheets were 6-10 layers thick by electron microscopy, with keratohyalin granules and cornified envelopes appearing at layers 3-6 (from basal layer). Dispase treatment allowed an easy detachment of these sheets. These results show that the culture medium MCDB 153 can be adapted without serum supplementation to batch culture of human adult keratinocytes to produce epidermal sheets suitable for grafting. They also indicate that extracellular calcium in physiological range of concentration is not a sufficient signal for growth arrest when other growth conditions are optimized.
Xeroderma pigmentosum (XP) and trichothiodystrophy (TTD) are two recessively transmitted human diseases characterized by DNA repair deficiency. While XP is associated with a very high incidence of cancer on skin exposed to sunlight, TTD is not a cancer-prone disease. Therefore, unrepaired UV-induced DNA lesions do not appear to be enough to give rise to tumors. In order to understand the differences between these two syndromes, we measured catalase activity in cellular extracts, UV irradiated or not, and quantified H2O2 production following in vitro UV irradiation. We confirmed on 21 different XP diploid fibroblast lines that catalase activity was decreased on average by a factor of five as compared to controls, while XP heterozygote lines exhibited intermediary responses. All seven TTD lines we tested were deficient in UV-induced lesion repair and exhibited a high level of catalase activity. However, molecular analysis of catalase transcription showed no difference between normal, XP and TTD cell lines. This was confirmed by Western blots where the amount of catalase subunits was identical in all cell lines studied. Finally, UV irradiation induces five and three times more H2O2 production in XP lines compared with TTD or controls respectively. These striking differences between TTD and XP indicate that UV light, directly or indirectly, together with defective oxidative metabolism may increase the initiation and/or the progression steps in the XP environment compared to TTD. This may partly explain the different tumoral phenotype observed between the two diseases.
16 children with atopic dermatitis and 4 nonatopics were skin tested with flavourings and perfumes. Immediate reactions to balsam of Peru and fragrance-mix were found in 9 atopics, and none among nonatopics. An irritant is more probable than an immunologic mechanism. Allergen solutions should probably be assayed at a lower concentration in atopic patients. This study points to a possible aggravating factor from perfumes and flavourings ingested, inhaled, or used as cosmetics.
The authors have followed during 10 years a girl with Bartter's syndrome who developed severe insulin resistance with acanthosis nigricans. In this rare association, hypokalemia and renal failure did not appear to be relevant factors triggering the onset of diabetes. The therapeutic difficulties in this case have still not been resolved.
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In children as well as in adults, capillaroscopy is an unsophisticated and non invasive technique which allows to investigate vascular acrosyndromes and systemic diseases. We have studied nailfold capillaroscopy patterns in 80 children without over vascular or systemic disease: pericapillary halos and haemorrhages increased with age and capillaries matured towards the typical hair pin structure seen in adults. The number of minor dystrophies increased with age and the venous subpapillary plexuses became less easily visible.
An illustrative case report and a series of 18 well-documented cases of lichen striatus are presented. The mean age at diagnosis was 3 years (6 months to 14 years; median 2 years). The lesions were predominantly distributed on the trunk in 33% of cases and on the limbs in the remaining two thirds (upper limb: 48%; lower limb: 19%). Pruritus was noted in only 1 of 18 cases. Six cases were associated with clinical features of atopy and/or minor signs of atopic dermatitis (e.g., pityriasis alba). Two cases were considered to be clinically associated with lesions consistent with psoriasis. The mean duration was 9.5 months (4 weeks to 3 years; median 6 months). In one patient, two relapses occurred in 4 years. Hypochromic sequelae were noted in 50% of cases. Lichen striatus is the most common acquired self-limited linear eruption in childhood that follows Blaschko's lines. A new acronym is proposed to emphasize the developmental background of the disease: BLAISE for Blaschko linear acquired inflammatory skin eruption.
A 7-year-old girl developed a cutaneous bullous eruption with genital and nasal mucous membrane involvement. Direct immunofluorescence revealed linear deposits of IgA and IgM at the basement membrane zone. No circulating antibasement membrane zone antibodies were detected. Small bowel biopsies showed a partial villous atrophy. The clinical, histologic, and immunopathologic findings were consistent with a diagnosis of linear IgA dermatosis of childhood. Immunoelectron microscopy revealed IgA deposits in the lamina lucida in association with hemidesmosomes, confirming results of two recent studies.
The Proteus syndrome is a congenital hamartomatous disorder delineated in 1983. Because of its polymorphic appearance, the syndrome was named after the greek god Proteus whose name means much less than the polymorphous much greater than. Major clinical findings include hemi hypertrophy, macrodactyly, exostoses, scoliosis, epidermal nevi, haemangiomas, deeply rugated soles of the feet and a variety of deep and subcutaneous masses. We report on 7 new cases of Proteus syndrome. All reported cases have been sporadic. Therefore this syndrome could be due to the action of a dominant lethal gene surviving by mosaicism.
The Noonan syndrome is a multiple congenital anomalies syndrome with variable expressivity and autosomal dominant inheritance. We report an observation of a newborn with Noonan syndrome and an unusual molluscoid cutaneous excess over the scalp that might represent a new skin manifestation in Noonan Syndrome rather than a consequence of lymphatic dysplasia.
Two monozygotic female twins with linear porokeratosis of Mibelli are described. One had only minimal lesions of the right elbow. The other had a linear lesion of the right arm following Blaschko's lines. Monozygotism was established using DNA fingerprinting. A single-gene defect of variable expressivity involving the clonal development of epidermal cutaneous cells according to the lines of cutaneous embryogenesis is suspected. Dithranol and carbon dioxide laser treatment are discussed.
Seventeen cases of pityriasis lichenoides diagnosed over a nine-year period in children under 15 years of age are reported. Patients with this benign disease develop papular skin lesions covered with thick, coherent scales which detach in a single piece (reminiscent of sealing wax). Pruritus is not marked. Lesions may be necrotic (Mucha Habermann's small pox-like form, n = 6) or mild (leukodermic form, n = 2). Half of the patients studied developed several episodes and total duration of the disease exceeded two years in one third of cases. Recovery occurred after one or two episodes in half the children. Scars developed in some patients with severely necrotic lesions. None of the patients developed lymphoma. All patients with lymphomatoid papulosis progressing to lymphoma reported in the literature were adults. Pathogenesis of pityriasis lichenoides remains unknown but may involve lymphocytic vasculitis. No truly effective therapy is available. However, oral macrolides can be used especially in patients with early manifestations suggesting an infectious disease. Emollients, heliotherapy and ultraviolet therapy may also be recommended.
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In an open study, 19 infants with a bipolar seborrhoeic rash were treated with ketoconazole 2% in cream once a day and evaluated over 10 days of treatment. At day 10, 78.9% of patients were almost cleared. Percutaneous absorption peaked 1-3 h after topical treatment, and was minimal. No plasma ketoconazole accumulation over the 10-day treatment was detected. Treatment failures corresponded to histologically psoriasiform eruptions and probable atopic dermatitis.
Oxatomide is an antiallergic molecule which acts mainly as a calcium blocker. This article is a review of oxatomide treatment in atopic dermatitis patients. First, an overview of oxatomide treatment hypersensitivity reactions in atopic dermatitis and of their supposed immunologic mechanisms. The total number of patients treated so far in published studies is too low to fully appreciate the therapeutic benefit of this molecule. However, encouraging results were reported in two controlled studies and 3 open studies originating from four different groups. Tolerance was excellent (of 200 patients evaluated in 5 studies, transient sleepiness was noted in 5, and weight gain was noted in 3 children).
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Unlike other major abdominal organs, infarctions affecting the liver is extremely rare. In humans, the rarity of hepatic infarction is explained commonly on the basis of the double blood supply via the hepatic artery and portal vein. The imaging studies and clinical records of seven patients with hepatic infarcts were reviewed. These infarctions were essentially observed during post-operative periods: after aortoceliac graft and after accidental ligation of the hepatic artery (4 cases). The other etiologies included: acute hypovolemic shock in patients with atherosclerosis (2 cases) and finally one case of acute thrombocytosis. Dynamic CT was performed initially in all the cases and during the evolution, demonstrating peripheral low attenuation areas without enhancement. Associated splenic infarcts were present in 4 patients and renal infarcts in two. While the clinical and laboratory findings in hepatic infarction can be completely mimicked by hepatic abscess, CT-guided aspirations were performed in 6 patients demonstrating in only one case the presence of infection.