Severe cholestatic jaundice induced by Epstein-Barr virus infection in the elderly.

Infectious mononucleosis due to Epstein-Barr virus (EBV) is almost always a self-limited disease, most commonly seen in young adults. Hepatitis is a well-recognized complication of EBV infection that usually resolves spontaneously. Jaundice occasionally results from the unusual complication of autoimmune haemolytic anaemia rather than hepatitis. We report a 60-year-old man with severe cholestatic jaundice whose history, liver histology and laboratory findings suggested EBV infection. He also developed significant jaundice related to his hepatitis, but not to autoimmune haemolysis, a situation that led to diagnostic delay. Costly diagnostic laboratory tests and invasive procedures were performed to rule out a malignant extrahepatic biliary obstruction. Physicians need to be aware of this complication and EBV infection should be included in the differential diagnosis of cholestatic jaundice in the elderly.

Progressive familial intrahepatic cholestasis among the Arab population in Israel.

BACKGROUND: Progressive familial intrahepatic cholestasis, which constitutes a heterogeneous group of imperfectly delineated syndromes and appears to be inherited as an autosomal recessive condition, has not been hitherto reported from the Middle East, in spite of the high rate of consanguineous marriage in this region. METHODS: Sixteen affected children from six Israeli Arab families were evaluated over 30 years. All were born to consanguineous parents. RESULTS: Jaundice appeared during the first 3 weeks of life in 15 babies. When first referred, 10 had hepatomegaly and nine had splenomegaly. A progression toward cirrhosis was the rule. Serum levels of conjugated bilirubin, liver enzymes, and alkaline phosphatase were raised; gamma-glutamyl transpeptidase levels were normal in all three infants in whom it was examined, but elevated in two siblings of another family at ages 2 and 3 years. No abnormal bile acids were detected in the serum and urine of patients. Histologic examination of the liver showed giant-cell transformation, paucity of intrahepatic bile ducts, cholestasis, fibrosis, or cirrhosis. The pattern of liver pathology differed at times among affected members within the same family. Therapeutic trials with phenobarbital, cholestyramine, or ursodeoxycholic acid were ineffective. Survival of the patients was from 5 to 18 months in four families; in the other two families, three children received liver transplants, and one is awaiting liver transplantation. CONCLUSIONS: Progressive familial intrahepatic cholestasis should be included in the differential diagnosis of infants with cholestatic jaundice of unknown etiology, especially those born to consanguineous Arab parents.

Upright tilt test: correlation between results and patient clinical features.

The aim of our study was to analyze how the clinical history and the main clinical characteristics of patients suffering from loss of consciousness may influence the results of the upright tilt test. A series of 745 patients (333 males, 412 females; mean age 44 +/- 18 years) with recurrent episodes of syncope or presyncope underwent complete clinical and noninvasive laboratory examination, including vagal maneuvers and upright tilt test (60 degrees for 45 min). Cardiological and neurological findings were normal in every case. Upright tilt test was positive in 462 patients (62%). Patients with presyncope showed a lower positivity compared to patients with syncope (70.2% vs 42.9%, P < 0.001). Younger patients (< 25 years) displayed highest upright tilt test positivity (68.5%), while familial occurrence of syncope or presyncope, results of vagal maneuvers, and different gender did not correlate with the results of the test. The time interval between the last syncopal episode and the day of upright tilt test negatively influenced the proportion of positive tests (> 30 days = 45.1% vs < 30 days = 77.2%; P < 0.001). Patients with more than three syncopal episodes in the 2 months preceding the test showed a higher upright tilt test positivity (83.9% vs 64.5%, P < 0.001). In conclusion, upright tilt test seems to be more sensitive in young patients with syncopal episodes during symptomatic periods. These findings suggest both an individual and temporal variability in autonomic nervous system activity, the implication of which are relevant to the indications for testing as well as the analysis of results.

Mucinous syringometaplasia. An immunohistochemical and ultrastructural study of a case.

Mucinous syringometaplasia (MS) is an unusual skin lesion of unknown etiology, characterized histologically by epidermal invaginations lined by mucin-laden goblet-like cells and by nonkeratinized squamous cells. The present case study was performed to elucidate further the characteristics of this lesion using immunohistochemistry and electron microscopy. The mucin-laden cells in the MS lesion stained positively for carcinoembryonic antigen, epithelial membrane antigen, and low molecular weight keratins. The ultrastructural examination, which was performed on deparaffined sections, revealed two morphological variants of mucous granules. Electron-dense mucous granules predominated in the mucus-containing cells, which were situated among the keratinocytes adjacent to the epidermal invaginations, mostly in the lower parts of the epidermis. Larger, electron-lucent granules containing flocculent material were found more abundantly in the cells lining the epidermal invaginations. Also, some of the mucus-containing cells showed bundles of tonofilaments; structures that appeared to be isolated short, stubby microvilli; and attachments to adjacent mucus-containing cells and keratinocytes by desmosomes. The ultrastructural and immunohistochemical findings in our case suggest that the mucinous changes occurred as a metaplastic process in the resident epidermal cells and were accompanied by changes in cellular antigen expression resembling those of simple secretory epithelium.

[Ventricular tachycardia secondary to myocardial contusion in an athlete: a clinical case report]. / Tachicardia ventricolare secondaria a contusione miocardica in atleta: descrizione di un caso clinico.

A 22-year-old athlete complained of palpitations shortly after a chest trauma during a basket competition. His electrocardiogram showed a ventricular tachycardia of 250 beats per minute, reverted to sinus rhythm with a chest thump. Standard electrocardiogram was normal. The following days he had an enzymatic pattern consistent with acute myocardial infarction, and the echocardiogram evidentiated an apical hypokinesia with a mural thrombosis. The patient recovered completely; and 15 days after the acute event, a coronary arteriography and ventriculography did not revealed any abnormality.

[Transjugular liver biopsy in patients with bleeding tendency].

Transjugular liver biopsy (TJB) was successful in 14 of 23 patients with chronic liver disease and abnormal coagulation profiles. There were 7 men and 7 women between the ages of 20 and 65. No bleeding followed the procedure, nor were there any other significant complications. The size of the specimens and the number of portal spaces included were compared with those obtained by percutaneous liver biopsy from 12 patients with advanced liver cirrhosis. TJB samples were smaller than those obtained by percutaneous biopsy (0.56 +/- 1.6 cm vs 1.0 +/- 0.05) and contained fewer portal spaces (2.2 +/- 1.6 vs 3.4 +/- 2.3). Despite the smaller size, the contribution of TJB to diagnosis and prognosis was defined as good in 78% of the patients. We conclude that TJB is safe and is an important tool for liver tissue diagnosis in patients with bleeding tendency.

Massive subcapsular fibrosis of the liver: ultrasonic and computed tomographic characteristics.

Massive and diffuse subcapsular fibrosis of the liver was detected in two patients with chronic liver disease. The subcapsular layer consisted of inflamed fibrous tissue containing rare portal tracts and myriads of bile ducts, indicating loss of subcapsular hepatocytes and their replacement by connective tissue. In the early evolutive phase, massive and diffuse subcapsular fibrosis of the liver appears on computed tomography (CT) scan as a low-density band, which merges with the hepatic parenchyma and is enhanced by contrast on dynamic imaging; it is undetectable by ultrasonography. In the advanced phase, it is easily recognizable by ultrasonography as a perihepatic sonolucent band and by CT scan as a low-density band, which is well demarcated from the hepatic parenchyma and is not enhanced with the dynamic technique.

Hepatitis B virus infection in patients with idiopathic liver disease.

We studied 67 HBsAg-negative Israeli patients (36 negative for all HBV serological markers as group 1 and 31 positive for antibodies to HBs and HBc as group 2) with chronic liver disease and cirrhosis of unknown origin using a rapid, sensitive and specific assay for the detection of low levels of hepatitis B virus in serum. This technique uses a high-affinity monoclonal antibody to HBs against an a domain epitope of HBsAg to capture the virion, followed by hepatitis B virus DNA amplification with the polymerase chain reaction. In addition, 55 subjects without liver disease served as controls: Group 3 (n = 32) was negative for all hepatitis B virus markers; group 4 (n = 23) was positive for antibodies to HBs and HBc. We found 11 individuals in group 1 (31%) and 10 in group 2 (29%) harboring low levels of hepatitis B virus DNA in serum. In contrast, no one in group 3 or group 4 was positive by this technique (p less than 0.0001). Using polymerase chain reaction primers spanning other regions of the hepatitis B virus genome and a method of restriction-fragment analysis of polymerase chain reaction-amplified sequences, we detected significant DNA sequence heterogeneity, suggesting infection with distinct hepatitis B virus strains. DNA extracted from paraffin-embedded liver biopsy specimens of 42 patients from groups 1 and 2 was shown to contain hepatitis B virus DNA by polymerase chain reaction in 11 of 12 patients with circulating virion DNA. More important, 18 additional patients whose sera were negative by HBs-antibody capture/polymerase chain reaction amplification had hepatitis B virus DNA sequences in their livers.(ABSTRACT TRUNCATED AT 250 WORDS)

[Unusual manifestations of Ebstein's disease in a 53 year old adult. Ebstein's disease in an adult]. / Manifestazioni inusuali della malattia di Ebstein in un adulto di 53 anni. Malattia di Ebstein in un adulto.

B.P., asymptomatic until her fifties, was admitted to hospital because of precordial pain. Her ECG and chest X rays were normal. When 52 she was again admitted, because of congestive heart failure. ECG showed repolarization abnormalities and chest X rays showed marked cardiomegaly. 2d echocardiogram was consistent with right atrial dilatation, dislodged posterior leaflet of the tricuspid valve and marked tricuspid regurgitation; these features were consistent with Ebstein anomaly. The patient was successfully treated by digitalis and diuretics. This case shows that in very rare cases this congenital heart disease can produce its clinical manifestations only late in lifetime.

[Incidence of conduction disorders in patients who underwent surgery for hypertrophic obstructive cardiomyopathy]. / Incidenza di disturbi di conduzione in pazienti operati per miocardiopatia ipertrofico-ostruttiva.

Thirteen non-consecutive patients, aging 7 to 61 (average 27) years, underwent left ventricular myotomy-myectomy for a severely symptomatic idiopathic hypertrophic subaortic stenosis (IHSS). In all patients the resting ECG before surgery showed P-R less than 0.18 sec, QRS duration less than 0.11 sec, QRS axis ranging from +10 to +80 degrees. In the immediate post-surgical period 3 patients has complete heart block and 1 had 2nd degree type 2 atrio ventricular block. Lesion was infra-Hisian in 3 patients and intra-Hisian in 1 patient. In the remaining 9 patients an immediate post-surgical left bundle branch block appeared; in 3 out of these patients ECG and an electrophysiologic study documented severe infra-Hisian conduction impairments after an average period of 4 years from surgery. During follow-up 3 patients died suddenly.

Early and late determinants of survival after surgery of left ventricular aneurysm.

The fate of 103 patients consecutively operated upon for chronic left ventricular aneurysm between 1978 and 1986 was examined with a multivariate statistical approach to verify the operative indications and results. In the early risk phase, up to 39 days after operation, 15 patients (15%) died. Mortality was mostly due to a low output syndrome and was significantly related to older age and to functional (NYHA) and anginal (CCS) class. In the late risk phase, starting 1.9 years after surgery, 9 patients died (10%) and the significant risk factors were anterior aneurysm and older age at operation. Actuarial survival curves showed 82% survival at 5 years and 61% at 9.5 years. In 25 patients older than 50 years and with an anterior aneurysm, these rates were 51% and 34%, respectively. Improved functional class was observed in 87% of the patients interviewed, but 30% complained of angina or new infarctions. Survival free of ischemia was 64% at 5 years and 13% at 9.5 years. This development of ischemic recurrences was significantly related to older age and to incomplete revascularization despite multiple grafts. These results suggest modification of the grafting policy and of the techniques of repair in identified high-risk subsets.

Idiopathic calcinosis of scrotum.

A case of idiopathic calcinosis of the scrotum is presented. This term has been applied to an uncommon disorder characterized by solitary or multiple, firm painless papules or nodules of the scrotum that usually appear during childhood or early adult life. The lesions are skin colored to white-yellow, increase in size and number, may itch or drain a chalky white material. X-ray film reveals calcified shadows. Biopsy specimens of the lesions show masses of calcium deposits in the dermis without evidence of an epithelial lining.

Diclofenac-induced hepatotoxicity.

Two patients developed clinical, biochemical and histopathological signs of a liver hypersensitivity reaction following treatment with diclofenac. Hepatic side effects of this drug are very rare. The relevant literature is reviewed.

Night blindness and liver cirrhosis as late complications of jejunoileal bypass surgery for morbid obesity.

A patient underwent end-to-side jejunoileostomy for morbid obesity, and 3 years later an end-to-end jejunoileostomy with ileotransversostomy was performed. Nine years later she presented with night blindness, severe diarrhea and mild jaundice and was found to have malabsorption with vitamin A and K deficiencies as well as asymptomatic liver cirrhosis. Her shunt was removed, and a gastric partition was performed. The night blindness and abnormal prothrombin time were corrected by the administration of vitamins A and K. This case demonstrates that complications may appear many years after jejunoileal bypass surgery, and therefore, the patients should remain under strict medical supervision indefinitely.

Primary cutaneous extravertebral meningioma. Case report.

A case of cutaneous extravertebral meningioma is presented. It was diagnosed in infancy as a lumbar meningocele. Operation was initially refused but was subsequently demanded for cosmetic reasons. The findings were a very thick corrugated skin and a cutaneous meningioma connected by a fibrous tract to the dura mater. The presence of a fibrous stalk linking the tumor to the dura mater might have been the pathogenetic connection between the meningocele and cutaneous meningioma.

Computed tomography attenuation values in fatty liver.

The accuracy of CT diagnosis of increased fat deposition in the liver was assessed in 11 alcoholic patients with suspected fatty liver. Single-energy CT attenuation values were compared with histological assessment of fatty liver and chemical analysis of liver triglyceride concentrations, obtained from liver biopsy specimens. Measured attenuation values showed an inverse correlation with the degree of fatty change assessed histologically (r =--0.90; p Less Than 0.0001) and with that assessed chemically (r=--0.57; p Less Than 0.05). CT scanning may provide a useful non-invasive method for detecting and following patients with fatty liver, particularly when liver biopsy is contraindicated.