Distinctive HLA-II association with primary biliary cholangitis on the Island of Sardinia.

Background: The *08 allele associated with primary biliary cholangitis (PBC) among Caucasians is of low frequency in the Sardinian population.

Objective: The aim of our study was to type a cohort of PBC patients from the island of Sardinia for HLA class II antigens.

Methods: Twenty Sardinian patients affected by PBC, 14 with autoimmune hepatitis (AIH) and 89 healthy controls (HCs) were typed for HLA class II alleles by dot-blot analysis.

Peginterferon alpha-2a and ribavirin versus peginterferon alpha-2a monotherapy in early virological responders and peginterferon alpha-2a and ribavirin versus peginterferon alpha-2a, ribavirin and amantadine triple therapy in early virological nonresponders: the SMIEC II trial in naïve patients with chronic hepatitis C.

Objective: The objective of this study was to compare the efficacy of anti-hepatitis C virus (anti-HCV) treatment schedules on the basis of an early virological response (EVR), defined as undetectable serum HCV-RNA (<50 IU/ml) after a 12-week induction course of peginterferon alpha-2a (PEG-IFN) 180 mcg/week.

Methods: A total of 210 interferon-naïve patients (69% male; median age, 42 years) with histologically proven chronic hepatitis C infection (genotype 1: 62%) received PEG-IFN 180 mcg/week for 12 weeks. Patients with EVR (58%) were randomized to continue PEG-IFN monotherapy (n=64) or to add ribavirin (RBV), 800 mg/day (n=57), for 36 additional weeks.

[Late onset Wilson's disease].

Background: Wilson's disease (W.D.) is a metabolic disorder that occurs predominantly in children, adolescents, young adults and, rarely, in patients over 35 years.

TT virus infection in Italy: prevalence and genotypes in healthy subjects, viral liver diseases and asymptomatic infections by parenterally transmitted viruses.

This study was aimed to evaluate TT virus prevalence in subjects with hepatitis B virus (HBV), hepatitis C virus (HCV) and human immunodeficiency virus (HIV) infections in patients affected by hepatitis of unknown origin (non-A-non-E hepatitis) and in healthy subjects who had not been exposed to HBV, HCV and HIV. A total of 317 subjects were tested; 40 were HBsAg asymptomatic carriers, 57 subjects were anti-HCV positive (45 without chronic hepatitis and 12 with HCV-related chronic hepatitis), and 27 had chronic non-A-non-E hepatitis. Fifty-seven subjects were intravenous drug users (IVDUs) (52 with HCV or/and HIV infections), seven patients underwent a liver transplant for fulminant hepatitis and 137 were healthy subjects from the general population.

Gross pathologic types of hepatocellular carcinoma in Italy.

The prevalence and independent predictors of the different macroscopic types of hepatocellular carcinoma (HCC) were assessed in 1,073 unselected patients of 14 hospitals in Italy from May 1996 to May 1997. Solitary HCC was the most common cancer type (44.6%), followed by multinodular (44.

Characteristics of hepatocellular carcinoma in Italy.

Background/aims: This study aimed to assess the main features of hepatocellular carcinoma at the time of diagnosis in Italy, particularly in relation to the presence or absence of underlying cirrhosis, hepatitis virus marker patterns, age of the subjects and alpha-foetoprotein values.

Methods: A total of 1148 patients with hepatocellular carcinoma seen at 14 Italian hospitals in the 1-year period from May 1996 to May 1997 were the subjects of this prevalence study. Both newly diagnosed cases (incident cases) and cases diagnosed before May 1996 but still attending the hospitals during the study period (prevalent cases) were included.

Value of histochemical stains for copper in the diagnosis of Wilson's disease.

Aims: The histochemical demonstration of hepatic copper is important in the diagnosis of Wilson's disease (WD). Conflicting results have been published with regard to the ability of different histochemical methods to demonstrate copper storage in the liver. Therefore, we evaluated the diagnostic value of three available histochemical methods in a large series of patients affected by WD.

Scanning electron microscopy of chronic hepatitis C. An OsO4 maceration study on human biopsies.

A study at the scanning electron microscope (SEM) on the liver changes in chronic hepatitis C was carried out in human needle biopsies from four patients. Intracellular structures were visualized by a novel modification of the OsO4 maceration method that allows to investigate human pathological specimens. At low magnification we observed both sinusoidal and hepatic cells alterations: sinusoids appeared occluded by lymphocytes, hypertrophic Kupffer cells, activated perisinusoidal cells, necrotic material and apoptotic bodies.

Mammillo-hypophyseal duplication (diplo-mammillo-hypophysis).

A first-time description is given of a unique combination of congenital deformities encountered in a female infant born in the 35th week of gestation and surviving for minutes only. The principal malformations comprised duplicity of the hypophysis cerebri and mammillary bodies, olfactory aplasia, agenesis of corpus callosum, Dandy-Walker syndrome, thoracolumbo-sacral rachischisis and hydromyelia, associated with palato-gnatho-cheilo-schisis, defects in the pericardium and cardiac interventricular septum and gross maldevelopment of the diaphragm. Of these cerebral anomalies, we have chosen to concentrate on the condition which we denominate "diplo-mammillo-hypophysis" (mammillo-hypophyseal duplication) as the object of this study.

Hepatic expression of hepatitis B and delta virus antigens in patients with acute and chronic hepatitis.

Delta antigen (delta-Ag), hepatitis B surface antigen (HBsAg) and hepatitis B core antigen (HBcAg) were examined by immunofluorescence and immunoperoxidase staining in 106 deparaffinized liver biopsy samples from HBsAg-positive patients with acute and chronic hepatitis. The delta-Ag was present in 15 cases (14%), with nuclear positivity varying greatly in intensity and prevalence. Patients with chronic hepatitis associated with delta infection had a histological picture characterized by foci of intralobular inflammation, many apoptotic bodies and shrunken hepatocytes with no satellite signs of inflammation.

Necrotizing emphysematous pyelonephritis: a case report.

Emphysematous pyelonephritis (EP) is comparatively rare in urological practice. From 1898 to the present time only 45 cases have been described. In this report we describe the case of a 43-year old diabetic man, with right EP without obstruction of the urinary tract and with a urinary infection of E.

Neuronal loss in the basal nucleus of Meynert in a patient with olivopontocerebellar atrophy.

A morphometric study of the basal nucleus of Meynert has been performed in a case of familial olivopontocerebellar atrophy with mental deterioration. The magnocellular population of the basal nucleus was found to be substantially reduced in number (over 60%) as compared with that of three age-matched controls. This finding has not been reported previously and might represent one of the anatomic substrates of some of the cognitive disturbances exhibited by a considerable number of patients with olivopontocerebellar atrophy (OPCA).

The basal nucleus of Meynert in idiopathic Parkinson's disease.

The basal nucleus of Meynert (bnM) was examined in 6 patients with idiopathic Parkinson's disease and in 5 age-matched controls. The histopathological study was followed by a quantitative analysis of the magnocellular population of the nucleus, with the determination of the number of neurons and their nucleolar volume. In Parkinson's disease, there was a neuronal loss ranging from 29.

The basal nucleus of Meynert in patients with progressive supranuclear palsy.

The neuronal number and nucleolar volume of the magnocellular population of the basal nucleus of Meynert in 6 patients with progressive supranuclear palsy (PSP) - one associated with Alzheimer's disease - were compared with those of 5 controls. In the 5 typical PSP cases, an age-dependent neuronal loss was demonstrated ranging from 12.6% to 54.

Basal nucleus of Meynert. A neuropathological study in Alzheimer's disease, simple senile dementia, Pick's disease and Huntington's chorea.

The basal nucleus of Meynert (bnM) was examined in 9 patients with Alzheimer's disease--4 presenile and 5 senile cases--in 3 patients with simple senile dementia, 5 with Pick's disease, 5 with Huntington's chorea and 5 non-demented controls. The histopathological study was followed by a quantitative analysis of the magnocellular population of neurons and by the determination of their nucleolar volume. In Alzheimer's disease there was a neuronal loss ranging from 44 to 76%, which was negatively correlated with both the age at onset and age at death of the patients.

Neuronal loss in the basal nucleus of Meynert in progressive supranuclear palsy.

A morphometric study of the basal nucleus of Meynert (bnM) has been performed in a 70-year-old man with a 4-year history of pathologically confirmed progressive supranuclear palsy (PSP). An important neuronal loss (52%) was demonstrated in the bnM. This finding has not been previously documented with morphometric methods in PSP, but the involvement of the bnM is well known in other related conditions, i.

"Thalamic" dementia in herpes encephalitis: clinico-pathological report.

Herpes zoster (HZ) primary affections of the CNS are rare and, in most of the reported patients, are representing variously extended forms of ascending myelitis. Our examination concerns a man who at the age of 37 developed apathy after a feverish episode with iridocyclitis. Six months later an ophthalmic HZ was diagnosed and thenceforth the patient showed a dementia with Korsakow's syndrome, apathy and a right hemipalsy, and diplopia appeared; the later symptoms remitted after steroid therapy.

Observations on Baiji (Lipotes vexillifer) and finless porpoise (Neophocaena asiaeorientalis) in the lower reaches of the Chang Jiang.

Lipotes usually occurs in pairs or in small loose groups. The calves are born in March-April. The Finless porpoises are usually seen in groups of five or six individuals.

Adult metachromatic leucodystrophy: clinicopathological report of two familial cases with slow course.

Two cases of adult metachromatic leucodystrophy in one family are reported. The main clinical features in both were predominantly psychiatric with alcoholism and an extremely long duration of the illness. Neuropathological examination revealed a similar distribution of the lesions in both, and scanty metachromatic accumulation in the CNS and not at all elsewhere.