OSTEOCHONDROMA OF THE PROXIMAL HUMERUS WITH FRICTIONAL BURSITIS AND SECONDARY SYNOVIAL OSTEOCHONDROMATOSIS.

We report a case of multiple hereditary exostosis in a 33-year old patient with clinical symptoms of pain and impression of a growing mass of the left shoulder alerting potential risk of malignant transformation of an osteochondroma. Imaging studies illustrated perilesional bursitis surrounding an osteochondroma of the proximal humerus. Malignant transformation was excluded with MRI. Fragments of the osteochondroma were dislocated in the inflammatory synovial bursa illustrating a case of secondary synovial osteochondromatosis.

Corneal astigmatism measuring module for slit lamps.

We have developed an automatic keratometer module for slit lamps that provides automatic measurements of the radii of the corneal curvature. The system projects 72 light spots displayed in a precise circle at the examined cornea. The displacement and deformation of the reflected image of these light spots are analysed providing the keratometry. Measurements in the range of 26.8-75 D can be obtained and a self-calibration system has been specially designed in order to keep the system calibrated. Infrared LEDs indicate automatically which eye is being examined. Volunteer patients (492) have been submitted to the system and the results show that our system has a high correlation factor with the commercially available manual keratometers and the keratometry measurements from a topographer. Our developed system is 95% in agreement with the corneal topographer (Humphrey--Atlas 995 CZM) and the manual keratometer (Topcon OM-4). The system's nominal precision is 0.05 mm for the radii of curvature and 1 degree for the associated axis.

Multipolar polarizabilities of the sodium atom by a variationally stable procedure.

We present a formalism that combines a semiempirical model potential with a second-order energy correction variationally stable method to evaluate the multipolar dynamic polarizabilities of the sodium atom. In this framework, the alpha(L)(omega) up to L=4 were calculated achieving good precision including the resonance domains of omega.

Electron affinity of the sodium atom within the coupled-channel hyperspherical approach.

We present a nonadiabatic calculation, within the hyperspherical adiabatic approach, for the ground state energy of the alkali-metal negative ions. An application to the sodium negative ion (Na-) is considered. This system is treated as a two-electron problem in which a model potential is used for the interaction between the Na+ core and the valence electrons. Potential curves and nonadiabatic couplings are obtained by a direct numerical calculation, as well as the channel functions. An analysis of convergence is made and comparisons of the electron affinity with results of prior work of other authors are given.

Bound states of the barium atom by the hyperspherical approach.

We present a nonadiabatic hyperspherical calculation of the highly excited and low lying doubly excited states of the barium atom using effective potentials for the two optically active electrons' interactions. Within the hyperspherical adiabatic approach the investigation of the spectra is performed with potential curves and nonadiabatic couplings of a unique radial variable, which allows clear identification of the states. The convergence of energy is obtained within well established bound limits, and the precision is comparable to accurate configuration interaction calculations. A very good agreement with experimental results is obtained with only few nonadiabatic couplings.

Paracetamol intoxication and N-acetyl-cysteine treatment.

N-acetylcysteine is a good and reasonable specific antidotum in case of paracetamol intoxication. It is very active when administered within eight hours after intoxication. It can be used as well as in intravenous as peroral administration. There are few side effects.

Iron removal from milk and other nutrient media with a chelating resin.

A water-insoluble iron(III)-chelating resin was used to study iron removal from milk and other nutrient media. Seventy to 85% of the iron could be removed from wine and beer with the resin, which was a crosslinked copolymer of 1-(beta-acrylamidoethyl)-3-hydroxy-2-methyl-4(1H)- pyridinone and N,N-dimethylacrylamide. Iron removal from milk was dependent on the pH of milk and on the concentration of soluble chelators added. Under the same conditions as used for the removal of iron from wine and beer, only 11 to 19% of the iron could be removed from milk. However, in combination with water-soluble chelators, the resin removed 60 to 75% of the iron from the milk. Preliminary results showed that the growth of spores of Clostridium tyrobutyricum in the treated milk was reduced. Moreover, addition of the resin and sodium bicarbonate to the milk completely inhibited the growth of the spores.

Clinical aspects of incomplete septal cirrhosis in comparison with macronodular cirrhosis.

BACKGROUND/AIMS: Incomplete septal cirrhosis (ISC) is a form of macronodular cirrhosis characterized by slender, incomplete septa that demarcate inconspicuous nodules. Its clinical features have not been investigated in a large series. The aims of this study were to review the clinical symptoms and evolution of ISC in 42 patients. METHODS: Forty-two patients with at least one liver biopsy strongly suggestive of ISC were selected for the study covering a period between 1968 and 1987. Data for these patients were compared with the evolution of 49 patients with classical macronodular cirrhosis after chronic active hepatitis type B or C. RESULTS: Possible etiological factors for ISC were alcohol abuse, arsenic treatment, and hepatitis B infection. In three cases, a genetic factor could not be excluded. Patients with ISC had significantly lower serum concentrations of transaminases and bilirubin at diagnosis. Compared with macronodular cirrhosis, bleeding varices were more frequent (57% vs. 22%) in ISC. Ten-year survivals in the ISC and the macronodular cirrhosis groups were 54% and 57%, respectively. CONCLUSIONS: ISC represents a relatively stable burnt-out form of macronodular cirrhosis with an unusually high incidence of variceal bleeding. This could be explained by a superimposed insufficiency of the portal vascular supply.

Glucagon enhances bile flow, bilirubin uridine diphosphate-glucuronyltransferase activity and biliary bilirubin monoconjugate excretion in the rat.

Intravenous infusion of glucagon (100 micrograms/hr/100 g body weight) in rats produces a 20 to 35% increase in bile flow and enhances the activity of hepatic bilirubin uridine diphosphate-glucuronyltransferase to 132% after a 90 min infusion. When a bilirubin load is given to produce a constant and apparently maximal biliary bilirubin excretion rate (or transport maximum) the administration of glucagon increased the bilirubin transport maximum. The excretion rate of bilirubin monoglucuronides was more enhanced than that of diglucuronide. The enhanced rate of glucuronidation, assayed in vitro, correlated with the augmented biliary output and inversely with the plasma unconjugated bilirubin levels. It is concluded that glucagon, at the dosage used, leads to a higher formation rate of bilirubin monoconjugates and that the choleresis, also induced by the hormone, enhances the biliary secretion of the monoconjugates formed. The enhanced conjugation results in a decreased plasma concentration of unconjugated bile pigment and the associated choleresis leads to a decreased di- to monoconjugate ratio, opposite to what has been observed during bilirubinostasis and cholestasis. The secretory efficacy, as assessed from the bile-to-plasma concentration ratio, is enhanced for all bilirubin pigments after glucagon administration.

Prevalence and significance of anti-HCV antibodies.

The actual ELISA test for anti-HCV defects an antibody to a non-structural part of the virus. It is present in 0.3 to 1.5% of the blood donors and in about 60% of patients with chronic non-A, non-B hepatitis. Seroconversion occurs 4 to 6 months after the beginning of the disease. The risk of a sporadic transmission is very low but unknown. An ALT elevation occurred only in 7% of the sexual partners of positive chronic liver patients but nobody seroconverted. Vertical, mother to infant, transmission is rare. In all risk groups for HCV infection such as drug abuse, multiple transfusion, haemodialysis, the presence of the antibody is increased between 15 to 80%. There is also evidence that HCV may be an oncogenic factor for hepatocellular carcinoma. It is present in 30 to 60% of the cases.

[Mitochondrial antigens and their antibodies]. / De mitochondriale antigenen en hun antilichamen.

In the mitochondria nine antigens and their corresponding antibodies are already known. They can be subdivided in three groups. The first one (M2-M4-M6-M8) appears during the evolution of primary biliary cirrhosis. The antibody against M2 is practically pathognomic for this disease. The M9-antibody is found in PBC with a slow and favourable evolution. The antibodies against M4-M8 are signs of a worse prognosis and a more rapid evolution into terminal cirrhosis. The second group is connected with infections and collagen diseases. The M1-antibody is directed against cardiolipin and diagnostic for syphilis. The M5-antibody appears in definite collagenoses. The M7-antibody is found in certain forms of acute and chronic cardiomyopathy. The third group of antibodies is induced by drugs: the anti-M3 by Venocuran containing a.o. phenopyrazone and the anti-M6 by Iproniazid. The role of the antigens and their antibodies concerning the aetiology and pathogenesis of the relevant diseases, especially primary biliary cirrhosis, is not known.

Light chain deposition disease of the liver associated with AL-type amyloidosis and severe cholestasis.

A 67-year-old man with a 3-month history of jaundice presented with hepatomegaly. Laboratory studies revealed abnormal liver tests with raised bilirubin. Renal function was normal. Endoscopic retrograde cholangiopancreatography revealed normal extrahepatic bile ducts. Liver biopsy showed severe bilirubinostasis and a typical bile infarct. Laminar and globular deposits of PAS-positive diastase-resistant non-congophilic material were observed in the sinusoidal walls. In addition, congophilic material was detected in the portal tracts. Immunohistochemistry revealed the presence of lambda-light chain deposits both in the sinusoids and in the portal tracts. Collagens type I and IV and fibronectin appeared markedly increased in the perisinusoidal space. On electron microscopy, the deposited material in the Disse spaces was mainly composed of fibrils indistinguishable from amyloid, admixed with small amounts of granular electron-dense material. The similarities of light chain deposition disease and AL amyloidosis are discussed.

Percutaneous transhepatic cholecystostomy for acute complicated cholecystitis in elderly patients.

We report our experience with percutaneous transhepatic cholecystostomy in 10 elderly patients with acute cholecystitis, complicated by empyema formation. Most of these patients has severe underlying disease, rendering them at high risk for surgical intervention. In all patients, the percutaneous procedure was followed by a rapid regression of clinical symptoms and of radiologic abnormalities. Six were considered inoperable. Three of these remain free of biliary symptoms, respectively 22, 10, and 7 months after percutaneous cholecystostomy. Three others died of nonbiliary disease 1-4 months after cholecystostomy. Three patients underwent successful elective cholecystostomy 1-5 wk after percutaneous cholecystostomy. In one patient, cholecystectomy had to be performed because of recurrence of hydrops, 1 wk after catheter removal. In our opinion, percutaneous transhepatic cholecystostomy is a safe and effective procedure in the treatment of elderly patients with acute complicated cholecystitis. It can be followed by elective cholecystectomy in good surgical candidates, or by an expectant conservative management in high surgical risk patients.

Reversal of hepatorenal syndrome in four patients by peroral misoprostol (prostaglandin E1 analogue) and albumin administration.

Four consecutive patients with alcoholic cirrhosis and hepatorenal syndrome were treated with misoprostol, a synthetic methylester prostaglandin E1 analogue at twice the dosage advocated for anti-ulcer therapy (i.e., 0.4 mg four times per day orally) and albumin infusions. The mean urinary output obtained over the 3 days preceding misoprostol administration was 250, 315, 550 and 195 ml per 24 h, respectively, in the four patients, despite adequate volume expansion by plasma albumin to reach normal or high central venous pressure. Diuresis increased to 1450, 2440, 925 and 1300 ml, respectively, on days 2-4 after onset of therapy. Serum creatinine levels were 71, 51, 33 and 35 mg/l before and dropped to 26, 21, 13 and 17 mg/l during treatment. All patients had hyponatraemia (117-128 mequiv/l) which normalized, although they were continued on a low sodium intake of less than 10 mequiv per 24 h. Urinary sodium excretion increased from 0.4-3 mmol per 24 h, to 15-40 in the first two cases and only slightly to 3-5 in the last two patients. Three patients died after 10, 30 and 40 days due to oesophageal bleeding, encephalopathy or pulmonary infection, whereas one patient underwent an orthotopic liver transplantation when her serum creatinine attained a level of 13 mg/l. In the first patient, hepatorenal syndrome recurred 10 days after stopping the misoprostol treatment. High doses of misoprostol in the presence of adequate volume expansion thus seem to produce marked diuresis and creatininuria as well as mild natriuresis.(ABSTRACT TRUNCATED AT 250 WORDS)

Arsenic and non-cirrhotic portal hypertension. A report of eight cases.

It was discovered that eight patients with complications of non-cirrhotic portal hypertension had received an arsenical preparation for psoriasis as Fowler's solution some years age. Seven of them were admitted for bleeding oesophageal varices. Upon admission, splenomegaly and hypersplenism were present. Liver tests were normal and palmar skin keratosis and melanosis were noted. Liver biopsy of six patients showed features of incomplete septal cirrhosis. Malignant skin lesions were present in half of the patients. One patient died from lung carcinoma and another from an ovarium neoplasm. Chronic arsenic intake should be actively looked for in all patients with psoriasis and non-cirrhotic portal hypertension. They should be followed up for many years for development of malignant lesions in skin, lung and liver. Liver abnormalities present in the biopsies are often minor and may escape detection.

An exclusion map of Marfan syndrome.

The combined genetic data between the Marfan syndrome and 75 informative loci on 18 autosomes were used to construct an exclusion map for this disorder. Data are also presented for a further two unmapped markers. The most likely location of the Marfan syndrome gene is highlighted and all the unexcluded areas of the genome are displayed in a graphical form. This exclusion map shows that almost 75% of the genome has been excluded as a likely location for the Marfan syndrome gene in the majority of the families studied. Apart from chromosomes 8, 13, 21, and 22, for which no data were available, other regions not excluded yet include 5p, 6p, 9p, 10p, 12p, 15, 17p, 18, and 20p. Future linkage analysis using markers located in the highlighted regions should facilitate the identification of the site of the Marfan syndrome gene.