Biomedical subjects
Publications and source records attributed to G Fabrizi.
Matting of the hair is a rare condition characterized by an irreversible tangling of scalp hair. This phenomenon has an uncertain etiopathogenesis, and it is assumed that physical, chemical and electrostatic factors may lead to its onset. In our case, vigorous brushing of the hair can be suggested as the etiological factor of matting.
We assessed 195 subjects with histories of adverse reactions to aminopenicillins, using 1) skin tests with penicilloyl polylysine (PPL), minor determinant mixture (MDM), benzylpenicillin (PG), amoxicillin, and ampicillin (read after 20 min and 48 h); 2) patch tests with PG, amoxicillin, and ampicillin; and 3) RAST for penicilloyls G and V. Oral challenges with ampicillin, amoxicillin, and penicillin V were administered to 34/60 patients reporting maculopapular reactions. Immediate hypersensitivity (IH), in most cases for both penicillin and aminopenicillins, was diagnosed (based on skin tests, RAST, or both) in 35 subjects who had suffered anaphylactic shock, or urticaria, angioedema, or both urticaria and angioedema. Thirty-three of the 60 subjects reporting maculopapular reactions presented delayed intradermal and patch-test positivity, indicating delayed hypersensitivity (DH), for ampicillin and amoxicillin, and three were also positive for PG. Diagnoses were confirmed with oral challenges in 18/33. The remaining 27/60 were negative in all allergologic tests, with oral-challenge confirmation in 16. Our findings highlight the importance of the amino group in DH to aminopenicillins. Moreover, the mean time interval between the last reaction and our tests was significantly (P < 0.01) longer in DH subjects (54.96 months) than in those with IH (18.62 months), suggesting that the time of testing is less important in cases of DH.
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We analyzed the success of suprapubic cystotomy in patients with severe hemorrhagic cystitis after bone marrow transplantation. Seventy-three out of 963 patients developed severe hemorrhagic cystitis which resulted in urinary tract obstruction after high-dose cytoreductive therapy. Eleven patients (15%) failed medical treatment and required emergency suprapubic cystotomy. Three of these patients died of other complications prior to resolution of HC. Of the remaining 8 patients who underwent surgery, 4 are alive. The mortality rate was significantly higher in patients who required surgery than in those who responded to medical therapy. Patients whose HC required surgery also had a greater transfusion requirement than those who responded to medical therapy. We conclude that surgical treatment of severe HC should be undertaken only after failure of medical therapy.
1H-NMR and molecular dynamics simulations in vacuo and in water of (1-->4)-alpha-D-galacturono-disaccharide were performed. The results of the molecular dynamics simulations showed that the molecule fluctuates between two conformations characterized by different values of torsion angles around the glycosidic linkage and two different intramolecular hydrogen bonds. When these conformations are extrapolated to a regular polymeric structure, they generate pectic acid compatible with a 2(1)- or a right-handed 3(1)-helix.
BACKGROUND: Palpebral syringomas have been reported to be more frequent in patients with Down's syndrome than in the normal population. OBJECTIVE: The aim of the present study was to evaluate, in a population of institutionalized patients with Down's syndrome, the prevalence of syringomas and their possible cytogenetic relationships. METHODS: Sixty-one institutionalized patients with Down's syndrome were examined in order to assess the presence of palpebral syringomas. Sixty mentally retarded non-Down's syndrome individuals were used to control group. RESULTS: Fourteen patients, 13 females and 1 male, were found to be affected. The prevalence of syringomas in both sexes was 23%; 42% of all females, and 55% when only adult females were considered, had syringomas. Thirteen of the 14 affected patients had a karyotype of Down's syndrome with free trisomy 21, 1 had a mosaicism 47,XX, +21/46,XX. Histologic examination confirmed the diagnosis in all 4 biopsied cases. A clear-cell pattern was observed only in 1 patient while, sporadically, few tubules showed a central syringial-type cuticula. CONCLUSION: The higher prevalence found in females, as compared to males, can be partially explained by their older age (mean 23.8 vs. 13.9 years). Palpebral syringomas are a common cutaneous pathology in adult females with Down's syndrome.
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C8H10N2O3, Mr = 182.18, monoclinic, P2(1), a = 6.6405 (7), b = 7.9493 (9), c = 8.3662 (9) A, beta = 103.07 (1) degrees, V = 430.18 (8) A3, Z = 2, Dx = 1.41 Mg m-3, lambda (Cu K alpha) = 1.54184 A, mu = 0.879 mm-1, F(000) = 192, T = 298 K, R = 0.037 for 1247 reflections with Fo greater than or equal to 4 sigma (Fo). The configuration at C7 is S. The pyrimidine-2,4-dione ring is nearly planar [r.m.s. deviation: 0.010 (8) A] and is antiperiplanar with respect to the epoxide ring. This arrangement is stabilized by intermolecular C-H ... O interactions.
Atopic Dermatitis (AD) and asthma are closely associated with respect to epidemiology, hereditary factors and occurrence in the same individuals. Bronchial Hyperresponsiveness (BH), the hallmark of asthma, can also be a physiopathological feature of AD, even in the absence of clinical asthma. We studied 78 subjects with AD. A follow-up study was performed in 27 of these. Data on respiratory and dermatologic symptoms were collected by means of a standardized questionnaire. Skin reactivity was evaluated by prick testing, and in 57 subjects BH was assessed with a methacholine test (Mch). Twenty-one subjects had asthma and 36 showed a positive skin reaction. A PC20 FEV1 was measurable in 38 subjects. Males were found more likely to be Mch responders than females (p < 0.05). Mch responders also showed an earlier age at onset of AD than nonresponders (2.1 yrs vs. 6.2, p = 0.03). Determinants of the degree of BH were evaluated by a stepwise multiple regression analysis, taking the log of the slope of the concentration response curve as dependent variable. In the final model we found that the degree of BH was directly related to wheezing (p = 0.0017) and coughing (p = 0.04) and inversely related to lung function (p = 0.0082) and age (p = 0.0008). Neither skin reactivity nor grading of AD were statistically significant. The longitudinal study demonstrated that the courses of AD and BH seem to run parallel only in skin-negative subjects, whereas an increase in BH was observed in skin-positive subjects.
The authors describe the clinical phenotypes of hexosaminidase deficiencies (GM2 gangliosidosis). The symptoms, differently combined, include cerebellar ataxia, motor neuron disease, dystonia, psychosis, neurovegetative troubles with different severity. Morphological changes are evident in rectal, muscle or nerve biopsies. Minor clinical changes are described in carriers from a family. A chronic GM2 gangliosidosis has to be suspected in any atypical case with the above-mentioned symptoms with autosomal-recessive inheritance.
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We investigated the prevalence of bronchial asthma, immediate skin reactivity and bronchial hyperresponsiveness in 40 patients with atopic dermatitis (AD). Eight patients reported to have asthma while 29 were found responders to methacholine test (PC20 Mch less than 64 mg/ml) and 21 were skin reactors. The prevalence of skin positive reactions did not differ in responder and non-responder (PC20 Mch greater than 64 mg/ml) groups. Males were more likely both to have bronchial asthma and be responders to methacholine test than females. Moreover, responders had an earlier age of onset of AD than the non-responders. We suggest that bronchial responsiveness should be evaluated in children with AD.
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