Respiratory muscle dysfunction in facioscapulohumeral muscular dystrophy

Sleep and Breathing -     

Angiopathy caused by vibration and CREST syndrome: beware of hidden pathologies!

The etiologic definition of an occupational disease should be achieved via an accurate differential diagnosis. A careful assessment of any clinical features that are atypical or unusual for the disease in question can sometimes lead to the identification of other morbid states that are either hidden or show few symptoms, and may or may not interfere with the occupational picture. The case described is of a man who, after working for 10 years with vibrating tools, developed a vibration disease with typical circulatory alterations in the hands and osteoarticular and neurological alterations. However, the appearance of a necrotic ischaemic lesion at the tip of the second finger of the right hand and signs of sclerodactylia in the second finger of both hands led us to suspect the presence of another concomitant disease. The finding of anticentromere antibodies suggested the presence of a CREST syndrome, which is a serologic variant of diffuse scleroderma. More in-depth investigations and examination of the state of other organs and apparatuses confirmed the diagnostic suspicion, with evidence of impairment also of the cardio-pulmonary system. The presence of the CREST syndrome was interpreted as a state of hypersusceptibility to the traumatic action of vibrating tools on the vascular system.     

Effects of androgen treatment in impotent men with normal and low levels of free testosterone

The relation between sexual function and serum free testosterone (fT) levels, which represent the active fraction of circulating testosterone, was evaluated. Two groups of impotent male subjects with mild hypogonadism were treated with oral testosterone undecanoate (TU); these men presented with tT/luteinizing hormone (LH) ratio and tT levels at the lower limits of normal. The first group had serum fT below 6.6 ng/ml, considered the lower normal value, according to our laboratory method, whereas the second group had normal fT limits. Administration of TU improved sexual function only in impotent men with low fT levels, but not in subjects with normal fT levels, even though the tT levels and the tT/LH ratio of the two groups were not significantly different. The results of our study suggest the presence of a minimun serum fT threshold, lying near the lower normal range, which determines the male sexual function. Moreover, serum fT levels were a more sensitive index than tT for identifying impotent men who can be successfully treated with androgens.     

RESPONSIVENESS AND SENSITIVITY TO CHOLINERGIC AGONISTS AND ANTAGONISTS IN BOVINE ISOLATED BRONCHIAL MUSCLE

1. Airways derived from different levels of the lung exhibit a difference in the reactivity and sensitivity to agonists. We have evaluated the effect of acetylcholine and cholinergic selective (pirenzepine, gallamine and 4-dipherylacetoxymethyl piperidine [4-DAMP]) and non-selective (atropine) antagonists on bovine proximal and distal smooth muscle preparations. 2. The distal preparations are more sensitive to acetylcholine than proximal bronchi. The relaxant effect of three selective antagonists on the distal and proximal tissues was the same when the results for each drug were compared. 3. Atropine and 4-DAMP were more potent than pirenzepine and gallamine in relaxing both proximal and distal bovine smooth muscle preparations. 4. These data suggest that the muscarinic sites on the smooth muscle of bovine airways are of the M3 subtype.     

Saliva substitute in xerostomic patients with primary Sj?gren's syndrome: a single-blind trial.

OBJECTIVE: To evaluate the efficacy of oral moisturizing gel (Oral Balance) in xerostomic patients with primary Sj?gren's syndrome (SS). METHOD AND MATERIALS: Twenty-one xerostomic patients with primary SS were subjected to a single-blind trial in which the efficacy of Oral Balance gel in reducing xerostomia and xerostomia-related oral symptoms was compared with that of a placebo. Both gels were packaged identically and were indiscernible in appearance and taste. Xerostomia was confirmed for all the patients through measurement of stimulated whole saliva. Patients began using the Oral Balance gel three times a day for 90 days, and were then switched to a gel placebo to be used in the same way for the same length of time. Clinical response was evaluated through the patients' subjective assessment (improved, worsened, or unaltered) of both gels. RESULTS: Neither the Oral Balance gel nor the gel placebo affected the salivary output of the patients. The Oral Balance gel presented a substantial statistically significant advantage in the control of burning mouth, mastication, and swallowing. No statistically significant relief of the isolated sensation of oral dryness was established. CONCLUSION: Oral Balance is a useful tool in the management of dryness-related oral symptoms in primary SS, but there is room for enhancing the overall properties of topical preparations designed to reduce oral complaints in xerostomic patients.     

Primary endodermal sinus tumor of the prostate: report of a case

A 29-year-old man was admitted to the Department of Urology of the University of Padova with a diagnosis of prostatic adenocarcinoma. Cystourethrography showed a prostatic urethral obstruction, and CT scan demonstrated a low pelvic solid mass involving the prostate and the seminal vescicle. A radical prostatectomy was performed, and the prostate was found to be widely infiltrated by a neoplasia formed by tubules, papillary structures, and solid cords of cuboidal or flat elements, each having indistinct borders and scant cytoplasm, in a myxoid or fibrous stroma. "Schiller-Duval-like," PAS-diastase-resistant and alfa-fetoprotein hyaline bodies were present. An endodermal sinus (yolk-sac) tumor was diagnosed. There was infiltration of the periurethral prostatic ducts, prostatic urethra, and seminal vesicles and single lymph node metastasis. Testicular echotomography was negative. In spite of aggressive surgery and chemotherapy, the patient died 10 months after diagnosis. This is the third case in the literature after those presented by Benson and Michel.     

Production of specific retinal S antigen antibodies in patients with retinal detachment

One hundred patients with retinal detachment (61 primary operations and 39 reoperations) were studied for titers of autoantibodies to human retinal S antigen using the enzyme-linked immunosorbent assay technique (ELISA). There was no statistically significant difference when comparing the group of patients with primary retinal detachment with the control group. However, a statistically significant increase in anti-S titers was recorded when a comparison was made between the group of reoperated patients and the control group (²,P < 0.001) and between the group of reoperated subjects and the group of patients operated on for the first time (²,P < 0.005).Presented at the XVth Meeting of the Club Jules Gonin, Copenhagen, 10–15 August 1986     

I Get by with a Little Help from my Family and Friends: Adolescents' Support for Diabetes Care

Evaluated and compared the support provided by family membersand friends for adolescents' diabetes care. Family and friendsupport also were examined in relation to other measures ofsocial support, to demographic variables (age, gender, durationof diabetes) and to adherence. Using a structured interview,74 adolescents with diabetes described the ways that familymembers and friends provided support for diabetes management(insulin shots, blood glucose monitoring, eating proper meals,exercise), and for helping them to "feel good about their diabetes."Families provided more support than friends for three managementtasks (insulin injections, blood glucose monitoring, meals);this support was largely instrumental. In contrast, friendsprovided more emotional support for diabetes than families.Greater family support was related to younger age, shorter diseaseduration, and better treatment adherence. Implications of thefindings include encouraging parents to remain involved in adolescents'treatment management, and involving peers as supportive companionsfor meals and exercise.     

Role of Periductal and Ductular Epithelial Cells of the Adult Rat Pancreas in Pancreatic Hepatocyte Lineage: A Change in the Differentiation Commitment

Development of pancreatic hepatocytes in adult rats maintained on copper deficient diet containing 0.6% trien (CuDT) has been reported recently. To elucidate the histogenesis of hepatocytes a sequential study was undertaken using morphologic, histochemical, immunochemical, in situ hybridization, and Northern blot analysis. Male F-344 rats weighing 80 to 90 g were fed CuDT for 8 weeks and returned to normal rat chow. Beginning from 4 weeks of copper depletion, there was a progressive loss of acinar cells and by 8 weeks more than 90% of the acinar tissue was lost. During this period, there was an increase in the number of adipocytes in the interstitium, and in the number of interstitial and ductular cells. Morphologic observations were confirmed by immunoblot and Northern blot analysis, in which the amount of pancreatic proteins and their mRNAs decreased between 5 and 8 weeks. During this period, a progressive increase in the level of albumin mRNA was observed. In situ hybridization, performed at 7 weeks of copper deficiency, showed localization of albumin mRNA over interstitial and ductular cells. Pancreatic hepatocytes were identified immediately after the rats were returned to a normal diet and gradually increased in number. The hepatocytes occupied almost 60% of the pancreatic volume by 8 weeks. During the early recovery phase, hepatocytes were identified in ductules as well as in the interstitium. Based on these studies, it is concluded that both the ductular cells and interstitial cells, which resemble oval cells of liver, are capable of transforming into pancreatic hepatocytes and these cells may be considered stem-cell equivalent.