Utility and safety of parotid gland biopsy in Sjögren's syndrome.

Parotid gland biopsy was performed in 32 female patients affected by suspected Sjögren's syndrome. In all cases histologic findings were in agreement with this diagnosis and in two subjects led us to detect the malignant transformation into lymphoma. None of the patients developed relevant complications. Our study indicates that parotid gland biopsy in Sjögren's syndrome is a safe procedure and may be useful in performing diagnosis.

[Current views on the pathogenesis and therapy of ankylosing spondylitis]. / Orientamenti attuali in tema di patogenesi e terapia della spondilite anchilosante.

Ankylosing spondylitis is a frequent and disabling inflammatory chronic disease, that affects mainly young men. In this paper the authors reviewed the pathogenetic mechanism and pharmacological and physical treatment. Nowadays the pathogenesis of ankylosing spondylitis is considered similar to that of reactive arthritis. The management of each patient should take into account the different aspects of the disease; therefore it should be individually designed.

[Symmetric multiple lipomatosis with Charcot's joint and neuropathic ulcer. Description of a clinical case]. / Lipomatosi multipla simmetrica con articolazione di Charcot e ulcera neuropatica. Descrizione di un caso clinico.

Multiple symmetric lipomatosis (MSL) (or Madelung's disease or Launois-Bensaude syndrome) is a rare inherited disease clinically characterized by a massive development of large symmetric unencapsulated lipomas on the subcutaneous tissue of face, neck, trunk and arms, resulting in a grotesque aspect of the patient. Less frequently the accumulation of excessive fatty tissue can spread deeply to the superficial fascia. Peripheral neuropathy, macrocytic anemia and chronic hepatopathy have been reported to cohesist. Macrocytic anaemia and chronic hepatopathy are probably secondary to high alcohol consumption, that is frequently associated. MSL, that was first described by Sir Benjamin Brodie in 1846, affects mainly the men (ratio man/woman 30:1), with an incidence in Mediterranean area of 1:25.000 men; the ages at onset range from 20 to 50 years. It is not known yet the pathway of inheritance and the molecular basis of the genetic defect responsible for the development of fat accumulation. It has been postulated a defective lipolytic response to catecholamines; this altered response could be due to an abnormal amount or a defective function of Gs-protein, the coupler between beta-adrenergic receptors on the surface of adipocytes and adenylate cyclase, or, alternatively, the defect could be in the catalytic unit of adenylate cyclase. The number and function of alpha- and beta-adrenergic receptors and the lipolytic response to cAMP (the second messenger) are normal. Recently it has been hypothesized that the defective lipolysis is due to a disorder in the mitochondria of brown fat, whose distribution is similar to the peculiar position of the lipomas in this pathology; the brown fat, unlike white adipose tissue, has abundant mitochondria. The alcohol abuse, frequently present in these subjects, might facilitate the clinical expression of the molecular defect. The therapy of lipomas is essentially surgical, but this approach is not easy, because the lipomas are not capsulated and extremely vascularized. Moreover the surgical excision is not always a successful treatment for the lipomas as they frequently recurrent after a short period from the exeresis. In this report we describe a 59-years old white man, alcohol abuser, with a typical clinical picture of MSL, developed when he was 37 years old. The patient presented multiple lipomas around the shoulders, face, neck and arms, that had been surgically excised eight times. Magnetic resonance imaging showed the presence of fat deposits also in the mediastinum, that caused a tracheal compression. Hepatic cirrhosis and serious side effects from peripheral neuropathy, represented by Charcot's joint and neuropathic ulcer on the sole foot were observed.(ABSTRACT TRUNCATED AT 400 WORDS)

Serum copper and ceruloplasmin in early and in advanced hepatocellular carcinoma: diagnostic and prognostic relevance.

To evaluate the prognostic value of serum copper (S-Cu) and ceruloplasmin and their pathophysiologic significance in human hepatocellular carcinoma (HCC), we studied 49 patients with HCC (20 of which were submitted to partial hepatectomy) compared with 110 patients with liver cirrhosis. In HCC both S-Cu and ceruloplasmin were higher than in cirrhosis; moreover, S-Cu was correlated with the extension of HCC, evaluated by instrumental data and by surgical inspection. In cirrhotic patients, mean S-Cu was 122.9 micrograms/dl (SD, 29.3), in early HCC, 153.0 micrograms/dl (SD, 34.5), and in advanced HCC, 193.1 micrograms/dl (SD, 37.7). Variance analysis gave F = 59.4. In HCC patients S-Cu was positively correlated with ceruloplasmin and with fibrinogen. Survival, evaluated by Mantel's test stratified for surgical therapy, was longer in patients with S-Cu levels lower than 175 micrograms/dl and in those at an earlier stage. We therefore conclude that S-Cu has a relevant diagnostic value in detecting HCC also in early stage and allows prognostic evaluation as regards survival.

Fibronectin is related to prealbumin in plasma of decompensated cirrhotics.

In a group of 23 patients with advanced liver cirrhosis we have found a statistically significant positive correlation (r = 0.746; p less than 0.0001) between fibronectin and prealbumin levels measured in plasma by immunonephelometric methods and found significantly lower than in healthy controls (p less than 0.001). On the contrary, no correlation of fibronectin neither to albumin nor to the presence of an enlarged spleen was observed. Since the sensitivity of prealbumin as an index of liver function is believed to be higher than that of albumin, our results support the view that the decreased fibronectin in advanced cirrhotics is mainly due to their liver failure, an enlarged spleen playing only a minor role.

Procollagen III peptide and fibronectin in alcohol-related chronic liver disease: correlations with morphological features and biochemical tests.

In order to clarify the significance of procollagen III peptide (PIIIP) and fibronectin (FN) blood concentration in alcohol related chronic liver disease (ALD), we have investigated their relationships with histological liver features and biochemical liver tests in 44 ALD patients. PIIIP was measured in serum by radioimmunoassay whereas FN was determined in plasma using an immunonephelometric method. In each liver biopsy, steatosis, portal infiltrate, lobular necro-inflammation, portal fibrosis and lobular fibrosis were semiquantitatively assessed by scoring from 0 to 3. A close correlation of PIIIP was found with morphological features of fibrosis (both of lobular and portal type), but not with necro-inflammation or steatosis. PIIIP was also positively correlated with ALP and GGT and exhibited a good diagnostic value in liver fibrosis. On the contrary, FN did not distinguish between normals and patients and was not correlated with any morphological liver feature or biochemical liver test. We also conclude that serum NP3P effectively reflects liver fibrosis, whereas plasma FN seems not related to any of the main histological aspects of liver damage in ALD.

Plasma fibronectin in liver cirrhosis and its diagnostic value.

Plasma fibronectin (FN) has been measured by immunonephelometric method in 100 cirrhotic patients and compared with that of 77 normal subjects and with that of 57 patients suffering from liver disorders different from cirrhosis. Both, compensated and decompensated cirrhotics had lower plasma FN than controls (31.14 +/- 11.42 and 20.88 +/- 10.43 respectively vs 40.13 +/- 8.58 mg/dl; rho less than 0.02 and rho less than 0.001). FN in ascitic patients was lower than in non-ascitic (rho less than 0.001). These differences were not due to different weight or age of patients. It appears, therefore, that FN parallels in cirrhosis the grade of liver function impairment. No significant difference has been noted between plasma FN of patients with liver diseases different from cirrhosis and control subjects. In cirrhosis, a positive relation has been observed among FN and other parameters of liver function such as serum albumin, cholinesterase activity, fibrinogen and prothrombin time. Plasma FN has a low sensitivity but a high specificity and a good positive predictive value in distinguishing normals and patients with liver disorders different from cirrhosis. This diagnostic value is similar to that of serum albumin.