Graham Little-Piccardi-Lasseur syndrome following HBV vaccination.
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Biomedical subjects
Publications and source records attributed to I Neri.
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The objective of the present study is to compare the effectiveness of transdermal glyceryl-trinitrate versus oral nifedipine in lowering blood pressure in patients affected by pregnancy-induced hypertension (PIH). Thirty-six consecutive pregnant women have been evaluated at different gestational ages after the diagnosis of PIH or preeclampsia (PE). After a 24-h ambulatory blood pressure monitoring, patients were allocated to three groups: those receiving oral nifedipine and those receiving transdermal glyceryl-trinitrate in a continuous (24 h/day) or intermittent (16 h/day) administration. A second blood pressure monitoring was performed after 2 weeks of treatment. Systolic and diastolic blood pressure were compared by using the Cosinor method looking at mesor, amplitude, and acrophase. Baseline systolic and diastolic blood pressure was similar among the three groups. Neither the transdermal glyceryl-trinitrate administered for 24 or 16 h nor oral nifedipine affected systolic and diastolic blood pressure. Analysis of variance showed that the posttreatment values were similar among the groups. Further studies are needed to verify the possible use of transdermal glyceryl-trinitrate as an antihypertensive drug during pregnancy.
BACKGROUND: In the present study we hypothesized that a derangement of the L-arginine-nitric oxide system could be involved in the development of the hypercoagulative status found during preeclampsia. In order to verify such hypothesis we have compared the effects of nitric oxide substrate, L-arginine on platelet aggregation. Moreover, we have also measured the L-citrulline plasma levels as a stochiometric metabolite resulting from the conversion L-arginine to nitric oxide. METHODS: Nine preeclamptic women and 11 normotensive pregnant women were enrolled for the study. Subjects were infused with saline and with 30gr of L-arginine. Blood samples were drawn during the saline infusion (30 min), during L-arginine administration (30 min) and 30 min thereafter. ADP and collagen-induced platelet aggregation was studied as per Born with a dual-channel aggregometer (Chrono-Log, Mascia Brunelli, Italy) and L-citrulline was measured by HPLC. RESULTS: In normotensive women the infusion significantly decreased ADP and collagen-induced aggregation after 15 minutes of L-arginine load; whereas no effects were observed in preeclamptic women. Similarly in normotensive but not in preeclamptic women L-arginine load was able to increase L-citrulline plasma levels. CONCLUSIONS: In normotensive women the in vivo L-arginine administration decreases platelet aggregation with an increase of L-citrulline plasma levels. On the contrary, no effects were observed in preeclamptic women. These findings confirm that a hypercoagulative status characterizes preeclampsia and that such phenomenon could be explained by a derangement of the platelet L-arginine-nitric oxide pathway.
We present a girl with lax, redundant skin, ectropion, bulbous nose, macrostomia, and absence of mammary glands. To our knowledge, she represents the fourth described case of Barber-Say Syndrome (BSS). BSS and ablepharon macrostomia syndrome (AMS) share common and distinctive clinical manifestations that involve the same structure of the skin and adnexa. We hypothesize that they may derive from a defective regulation of the same gene.
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The purpose of our study was to identify the clinical characteristics, epidemiologic data and histologic features in 29 cases of syringoma with a duration of lesions prior to the observation between 1 and 25 years. Only one patient complained of moderate itching. In two cases the lesion was solitary, in another the papules formed a lichenified plaque. In six patients only the eyelids were involved and in two patients a symmetrical localization on the forearms was observed. The other 18 patients showed generalized syringoma, 16 with an eruptive onset, 6 of which were familial. One of our cases showed lesions mimicking urticaria pigmentosa and two patients were affected by Down's syndrome. In two cases, histopathology showed association between syringoma and a melanocytic naevus and in one patient with a solitary lesion a clear cell syringoma was observed.
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BACKGROUND: Steatocystoma multiplex (SM) and eruptive vellus hair cysts (EVHC) are conditions characterized by numerous cutaneous cysts with a clinical resemblance, such as age of onset, location, appearance of the lesions and mode of inheritance, but with distinctive histologic features. Recently, some authors have proposed that the two conditions are variants of one entity originating in the pilosebaceous duct, while others suggest that SM and EVHC are two distinct entities, on the basis of the different expression of keratins. Milia are small round cysts, commonly involving the face. In 1994, Menni and Piccinno reported the first association, in a family, between persistent infantile milia in a 9-month-old girl and SM in the father. The authors hypothesized a relationship between these two conditions. OBJECTIVE: We describe a family in which the mother showed cystic lesions and milia and her 4-year-old son and her 18-month-old daughter presented persistent infantile milia. METHODS: In addition to the clinical and genetic documentation, a biopsy was performed in the son and two biopsies in the mother. RESULTS: The histologic examination of one of the son's milium-like papules showed in the superficial dermis a small cyst typical of a milium. In serial sections it was possible to see a connection with a vellus hair follicle by an epithelial pedicle. The histologic examination of one of the mother's papules on the forehead showed a large round cystic structure with histologic features of EVHC. Close to the cyst wall, serial sections showed flattened sebaceous gland lobules. The histologic findings of the second biopsy taken from the chest showed the typical features of a steatocystoma. CONCLUSIONS: The relationship between milia, SM and EVHC is discussed; the three disease should be considered as subtypes of multiple pilosebaceous cysts that may all present overlapping histologic features. The different level of the pilosebaceous duct where the cyst originates should explain the different clinical manifestations.
OBJECTIVE: To test the hypothesis that labor onset could be the result of a reduced release of nitric oxide (NO). STUDY DESIGN: Out of 91 consecutive healthy nulliparous women at term serum citrulline (Cit) and arginine (Arg) levels were measured at least twice in 37 subjects, by the means of HPLC with fluorometric detection. Twenty cases underwent a spontaneous onset of labor (group A) while in 17 cases labor was induced (group B) because of pregnancy prolongation or amniotic fluid reduction. RESULTS: Cit and Arg levels were unaffected by the gestational age. In group A, Cit levels undergo a progressive decrease toward the day of labor (from 32.9+/-4.7 microM/L the day - 18,-5 to 27.7+/-7.8 the day -4,0; p=0.012) whilst they remained stable in group B (from 33.3+/-7.7 to 34.2+/-9.2). No significant changes were observed in Arg levels. Cit/Arg ratio remained stable in group A whereas it showed a trend to increase in group B. CONCLUSION: These data indirectly suggest a reduced release of NO toward term. This phenomenon could play a permissive role in the spontaneous onset of labor of healthy nulliparous women.
INTRODUCTION: Pachydermodactyly is a superficial benign digital fibromatosis usually involving the proximal portions of the fingers. It is clinically characterized by an asymptomatic, bulbous, soft-tissue swelling around proximal phalanges and interphalangeal joints. We report here seven new cases of pachydermodactyly. CASE REPORTS: Seven patients (4 F, 3 M) ranging in age from 14 to 63 years were studied. Two of them were affected by tuberous sclerosis; two other patients were sisters, one of whom was affected by the transgrediens form of pachydermodactyly. The personal history of two male patients revealed the compulsive habit of interlacing the fingers. Finally a 23-year-old patient was affected by the localized pachydermodactyly. In all the patients roentgenogram and echography of the affected fingers as well as histological and ultrastructural of a cutaneous biopsy examination were carried out. The results of the tests confirmed the diagnosis of pachydermodactyly. DISCUSSION: Our data suggest that pachydermodactyly is underestimated rather than rare and more frequent in females than in males as until now reported in the literature. We suggest classifying pachydermodactyly into five types: classic pachydermodactyly frequently associated with mechanical trauma, monopachydermodactyly or localized pachydermodactyly, transgrediens pachydermodactyly in which the cutaneous thickness extends to the metacarpophalangeal areas, familial pachydermodactyly which may by transgrediens and pachydermodactyly associated with tuberous sclerosis.
Nitric Oxide (NO) inhibits platelet aggregation via activation of an intraplatelet soluble guanylate cyclase which induces an increase in cyclic GMP (1). It has been also demonstrated that platelets contain a constitutive, calcium-dependent, NO synthase which is activated by collagen-induced platelet aggregation. This leads to a NO synthesis from L-Arginine (L-Arg), which in turn increases cyclic GMP and down-regulates platelet aggregation (2). In vitro administration of supraphysiological concentrations of L-Arg enhances platelet cyclic GMP levels by increasing NO production and reduces platelet aggregation. This effect is reversed by pre-incubation with NO-synthase inhibitors (3). These results indicate that the L-Arg: NO pathway plays an important role in the modulation of human platelet aggregation (4). In vivo L-Arg, when administered i.v., induces hypotension (5) and vasodilatation (6,7) in humans, and when orally supplemented reduces platelet aggregability both in hypercholesterolemic rabbits and healthy men (8,9).
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