"Shadow" cells in proliferating trichilemmal tumors.
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Biomedical subjects
Publications and source records attributed to G Noto.
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The distribution of free SH groups and SS covalent linkages in hypertrophic, atrophic, acantholytic, bowenoid, pigmented solar keratoses (SK) and squamous cell carcinoma was evaluated. The sulphydryl groups were present in cytoplasms with a granular pattern and nucleoli mainly in atrophic, hypertrophic and bowenoid SK; the distribution of SS linkages appeared as a brilliant ovoid fluorescence localized in living layers, due to individually keratinized cells in SK. Similar results were found in squamous cell carcinoma. Our results agree with the opinion which considers SK as in situ carcinomas.
A female case of angiokeratoma corporis diffusum without systemic involvement, with alpha-galactosidase A activity in the normal range, alpha-L-fucosidase in the lower levels of the normal range, and a few amount of urinary sialic acid is reported. Some problem about differential diagnosis with inherited disorders as Fabry's disease, fucosidosis, sialidosis is discussed. Although cases of angiokeratoma corporis diffusum without any underlying enzyme defect have been reported, we believe that angiokeratoma corporis diffusum is always related to known or unknown enzymatic defect, which activities could result in the normal range probably in relation to enzymatic polymorphism.
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Ascites can extend from the peritoneal cavity into the posterior mediastinum through the esophageal hiatus. This mediastinal fluid can simulate the appearance of a mediastinal tumor. Demonstration of hiatal hernia and continuity between the thoracic and abdominal fluid assist in establishing the correct diagnosis.
A 42-year-old woman with a proliferating tricholemmal tumour (PTT) with regional lymph node metastases is reported. Histochemical studies showed evidence of tricholemmal keratinization. There was recurrence following excision and subsequently the patient developed lymph node metastases.
The identification of an abnormal paraproteinaemia has been considered as main criterium for the diagnosis of scleromyxedema. We report a case of scleromyxedema in a 51-year old man, without any immunochemical evidence of paraprotein. Our case is a clinical support to the independence of this disease from gammopathy, as underlined by other experimental and clinical data. Thus demonstration of paraprotein should not be considered as a fundamental criterion for the diagnosis of scleromyxedema.
The authors have studied the androgenic patterns in 29 women with late-onset persistent acne vulgaris. Clinical evaluation of acne, menstrual history and serum determinations of SHBG, total-T, free-T, DHEAS, delta 4A have been carried out. A mild and heterogeneous hyperandrogenism was found in 70% of women, thus, a greater steroid bioavailability for peripheral conversion and/or a direct stimulation of the pilosebaceous unit can be postulated. Androgenic evaluation in women with late-onset or persistent acne vulgaris is useful, mainly for hormonal management.
An unusual localization of lichen amyloidosus in a patient with IgG k benign monoclonal gammopathy is reported. After topical treatment with dimethylsulfoxide the lesions improved, but histological examination still showed amyloid deposits.
Aim of this study was to verify the prevalence of cardiac arrhythmias in subjects with mitral valve prolapse (MVP) and redundant leaflets in comparison with subjects with MVP without leaflets redundance. So, 60 subjects (aged 13 to 39 years), were subdivided in 3 groups on the basis of mitral leaflets shape at 2D-echocardiography; a continuous ECG monitoring (24 hours) was also performed. Arrhythmias were more frequent and more severe in the Group III (subjects with MVP and redundant leaflets), in comparison with both Group II (subjects with MVP without leaflets redundance) and Group I (control subjects). In particular, analysing the mean values of the single arrhythmias in the 24 hours, ventricular ectopic beats (VEB), were more frequent in Group II (p less than 0.01) and Group III (p less than 0.05) in comparison with Group I; the couplets and the runs of ventricular tachycardia were more frequent in Group III than in the other groups (p less than 0.001). The number of the subjects with a Lown class greater than 3 was higher in Group III than in the other groups (p less than 0.01). In conclusion, this study confirms that MVP is a disease presenting a large variability arrhythmic risk, that seems to be real only for a subgroup of these subjects.
Nuclear morphometric parameters and polarity of basal keratinocytes have been evaluated by computerized image analysis in solar keratoses, comparing the results with normal sun-exposed skin and squamous cell carcinoma 1st Broders degree. The cellular index demonstrated higher levels in all keratoses. The morphometric nuclear parameters revealed major values in atrophic, bowenoid, acantholytic, hypertrophic and pigmented solar keratoses, respectively. The loss of polarity demonstrated higher levels in bowenoid, atrophic, hypertrophic, pigmented and acantholytic histotypes, respectively. Solar keratoses could be classified as in situ carcinomas rather than precancerous lesions.
Sex-hormone binding globulin (SHBG) and androgen serum levels have been evaluated in a homogeneous group of women with female pattern of androgenetic alopecia (AA), stage II, without any clinical or anamnestic evidence of acne, hirsutism, irregular menses. Results did not show any significant difference between patients and controls. Since SHBG levels are androgen-dependent, the discordant results of previous published series regarding women with AA could be related to the presence of a variable number of patients with clinical and/or anamnestic evidence of a "cryptic" hyperandrogenism in some series, and to their absence in others. Statistically significant low average SHBG values could have been sustained by a mild, heterogeneous and not significant androgen excess. In our opinion the genetically-determined response of the target organ to androgens seems to play the major pathogenetic role in AA, at least when the woman does not reveal any clinical or anamnestic evidence of cryptic or clear hyperandrogenism.
The AA. have found IgM in cord sera of 903 newborns. In 13 (1.44%) there was an increase of IgM and the control made on neonatal serum within the first 5 days from birth has shown a positive result only in 1 case (7.69% of positives). The AA. present, in the conclusions, some considerations that justify the results of their work.
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The ovulatory patterns in women with acne vulgaris were evaluated in order to understand their relationship with androgenic levels. Ovulation disturbances were found in 58.3% of patients with prevalence of anovulation in the juvenile acne and of luteal insufficiency in the late-onset/persistent acne. Significant negative correlation was found between T free and P in the late-onset/persistent group (r: -0.629; p = 0.016): This may be interpreted as a rather steady endocrine status in which the raised androgenic levels, probably due to peripheral conversion, are concomitant to absent or insufficient ovulations. In the younger patients both the androgen excess and the ovulation disturbances could be due to an abnormal or delayed maturation of the hypothalamus-pituitary ovarian axis. The evaluation of the ovarian function in women with acne vulgaris may be useful to detect ovulatory disturbances in view of a possible resolution of both the problems by specific endocrine management.
We report a rare heterozygous status for Fabry's gene with severe kidney involvement and normal alpha-galactosidase A activity, together with the intrafamilial variations in the clinical expression of the disease. The random X inactivation hypothesis seems to explain such a variable expression of the alpha-galactosidase gene in our cases.