Psoriatic arthritis in British and Italian patients: a comparative clinical, radiologic, and scintigraphic study.

OBJECTIVE: To investigate the similarities and differences in pattern and severity of clinical expression of psoriatic arthritis (PsA) in British and Italian patients. METHODS: Detailed clinical data were obtained on 48 British patients with PsA and compared with the data of 48 Italian patients. A systematic evaluation of joints, spine, and skin was performed. Forty-one British and 42 Italian patients also underwent a whole body bone scintigram. Radiographic details of hands and wrists were also taken. RESULTS: The Italian patients did not differ from their British counterparts with respect to duration of arthritis, sex, clinical subgroups, axial involvement, number of tender and swollen joints, or radiographic score. However, significant differences were found with respect to age, duration of psoriasis, number of joints with deformity and/or loss of function, number of affected joints, and number of "active" joints on scintigraphy, which were all higher in the British group. After correction for age, duration of arthritis, and duration of psoriasis the only significant difference was the number of affected joints. CONCLUSION: Our findings suggest that British patients with PsA are more severely affected than a matched cohort of Italian patients.

[Internal medicine evaluation of the surgical risk in cirrhosis patients]. / Valutazione internistica del rischio chirurgico nel cirrotico.

A personal case series triggers an examination of surgical risk in patients with uncomplicated cirrhosis of the liver. After a general introduction the conditions that increase surgical risk in cirrhotic patients are analysed. These include generally poor resistance, altered haemostasis, a tendency towards cholestasis, water retention and hepatic encephalopathy. The conditions most often requiring surgical treatment are then considered. They include associated pathologies (cholelithiasis, hernias, tooth extractions, bleeding haemorrhoids etc) and complications of cirrhosis variceal bleeding, intractable ascites, splenomegaly, hepatocytoma).

Hepatitis B virus infection in porphyria cutanea tarda.

Serum markers of hepatitis B virus (HBV) infection were determined in 82 patients with porphyria cutanea tarda (PCT). Pathogenetic factors (alcohol, thalassemia minor, drugs) and clinical and histologic findings of PCT were taken into account. The prevalence of HBV infection was very high (70.7%). Hepatitis B surface antigen (HBsAg) was positive in 14 patients (17%). Eight patients had HBV infection as the only documented acquired factor. The clinical picture and histologic findings were aggravated by HBV infection; primary hepatic carcinoma occurred in four patients with HBV infection. Liver siderosis was histologically documented in 82.6% of cases, serum ferritin was pathologically increased in 91%, confirming the role of iron overload in PCT. A correlation (p less than 0.02; chi-squared method) was found between increased serum ferritin levels and HBV infection, suggesting a possible relationship between liver siderosis and HBV clearance. HBV infection appears to be a relevant additional factor in the pathogenesis of PCT liver disease.

Iron removal therapy in porphyria cutanea tarda: phlebotomy versus slow subcutaneous desferrioxamine infusion.

Twenty-five patients with overt clinical and biochemical findings of porphyria cutanea tarda took part in a study comparing intensive phlebotomy with slow subcutaneous desferrioxamine treatment. Fifteen male patients (Group A) had intensive venesection therapy. Ten patients (Group B) with associated diseases (minor thalassemia, cardiovascular impairment, pulmonary tuberculosis or severe liver cirrhosis) received 1.5 g of desferrioxamine by slow subcutaneous infusion using an automatic syringe pump 5 days a week. No patient complained of appreciable side effects. Serum iron, ferritin and uroporphyrins were normalized in all subjects by the end of treatment. The mean time necessary for complete recovery was 13.8 months (range 9-19) and 11.2 months (range 6-14) in Groups A and B, respectively. Liver function significantly improved during and after the treatments in both groups. We conclude that recovery from porphyria cutanea tarda can be achieved equally well using phlebotomy or desferrioxamine subcutaneous infusion. Phlebotomy is easily performed and remains the treatment of choice; slow subcutaneous desferrioxamine treatment, although expensive, is recommended when severe associated diseases contra-indicate venesection.

Serum ferritin in the assessment of liver iron overload and iron removal therapy in porphyria cutanea tarda.

Serum ferritin, an index of iron stores, was studied in 60 patients with porphyria cutanea tarda (PCT), in 21 patients who had other liver diseases without siderosis (cirrhosis [LC] and chronic active hepatitis [CAH]), and in 32 patients with associated liver siderosis (alcoholic LC, LC and CAH in minor thalassemia). Ferritin levels were higher in patients with porphyria than in healthy controls and patients without liver siderosis (P less than 0.001), whereas no statistical difference was observed between patients with porphyria and those with liver siderosis. Because iron removal is considered the treatment of choice for PCT, some patients with PCT underwent phlebotomy and others received chelating therapy with subcutaneous infusion of deferoxamine. Follow-up of the patients showed a correlation between serum ferritin level and urinary porphyrin excretion; when the clinical and biochemical syndrome became normal, serum iron and ferritin had fallen to normal values (t test pair data analysis before and after: P less than 0.001 in each group). No appreciable difference was found between controls and patients with PCT whose conditions had been normalized, irrespective of the chronic liver damage always present in PCT. Our results suggest that serum ferritin increase in PCT is related more to liver iron overload than to liver damage, and ferritin follow-up is recommended to indicate the exhaustion of hepatic iron stores during iron depletion therapy, as well as to detect an early replenishment after remission.

Standard or branched-chain amino acid infusions as short-term nutritional support in liver cirrhosis?

The metabolic effects of selected and branched-chain amino acid (BCAA)-enriched parenteral solutions were studied in liver cirrhosis. After 3 days of an oral protein-free diet with balanced amino acid (AA) infusion, 36 cirrhotic patients without encephalopathy were randomly divided into four groups. Groups A and B were infused for 5 days with BCAA (valine, leucine, isoleucine) at doses of 0.5 and 1.0 g/kg/day, respectively, as the only nitrogen source. Group C received 0.8 g/kg of essential and nonessential AA solution with a prevalence of BCAA; the last group (D) continued the basic standard diet, as control. Routine chemistry, urinary nitrogen losses, nitrogen balance, and the whole plasma AA pattern were detected before and after the treatment period. BCAA alone led to an impressive and significant improvement in the basic AA pattern in both the A and B groups. The same results were obtained in group C for plasma AA. In particular, the ratio of BCAA to aromatic amino acids in groups A, B, and C was significantly increased (p less than 0.01, less than 0.02, less than 0.02, respectively). In group D the AA pattern and the BCAA/aromatic amino acid ratio remained unchanged. The negative nitrogen balance of the base state remained unchanged after 0.5 g of BCAA (A); it improved significantly and became positive during and after the infusions of a double dose of BCAA (B), as it did in the case of selective solutions (C), although to a lesser extent; the negative nitrogen balance of the control group showed only a slight improvement.(ABSTRACT TRUNCATED AT 250 WORDS)

Advances in the treatment of porphyria cutanea tarda. Effectiveness of slow subcutaneous desferrioxamine infusion.

The authors present the results of long-term subcutaneous desferrioxamine (DFX) infusion in 16 porphyria cutanea tarda (PCT) patients who cannot undergo repeated phlebotomies because of severe liver damage, haemolytic anemia, cardiovascular impairment or pulmonary and bone tuberculosis. They employed an automatic, portable syringe pump for subcutaneous infusion (8-10 h) to overcome the short half-life of the drug. Photodynamic cutaneous lesions and hyperpigmentation quickly disappeared (2-3 months). Uroporphyrin excretion sharply decreased and normalized within 3-12 months. Also, serum iron and ferritin, as well as liver function, showed a significant improvement. The authors therefore propose subcutaneous DFX therapy in PCT treatment when phlebotomy is contraindicated.

Coproporphyrin excretion in healthy newborn babies.

The urinary and fecal coproporphyrins (CP) undergo significant changes in cholestatic diseases of both adults and infants and their determination may provide a diagnostic tool. Little is known about CP excretion in the first days of life. The authors have studied the daily urinary and fecal excretion of CP as well as the I and III isomer distribution in 10 healthy newborn babies from 1 to 10 days old. CP were determined by the solvent partition method and the isomer distribution by thin-layer chromatographic technique. Preliminary studies on urinary porphyrin pattern were performed using a personal high-performance liquid chromatographic method. CP excretion was almost 10 times higher on the 1st day than on the 10th, when expressed by adult standards. The isomer I accounted for almost 80% of the total amount on the first days, whereas at the end of the study, both the CP total amount and isomer distribution overlapped the infant and adult pattern. The authors propose a personal interpretation based on a possible transient enzymatic defect in the metabolic chain of heme synthesis.

[Determination of serum ferritin in porphyria cutanea tarda. A reliable sign of hepatic siderosis]. / Il dosaggio della ferritinemia nella porfiria cutanea tarda. Un attendibile indice di siderosi epatica.

Some parameters of iron metabolism in 26 patients with porphyria cutanea tarda (PCT) which is often associated with mild iron overload and hepatic siderosis, are studied. Serum iron, percent transferrin saturation and ferritin were pathologically increased. Statistical comparisons were performed between PCT patients and healthy controls, liver disease patients (cirrhosis, chronic active hepatitis) and patients with associated liver siderosis (alcoholic cirrhosis, cirrhosis and chronic active hepatitis in thalassemia). Ferritin levels are higher in patients with porphyria than in healthy controls (p less than 0,001) and in patients without liver siderosis (p less than 0,001). No statistical difference is observed between patients with porphyria and patients with siderosis. A significant decrease in ferritin levels is registered after venesection therapy. The conclusion is drawn that serum ferritin increase in PCT is related to hepatic iron store amounts rather than hepatic necrosis. It is assumed that ferritin follow-up during phlebotomy therapy and also during remission is useful to indicate the exhaustion or an early replenishment of hepatic iron stores.

Porphyria cutanea tarda in beta-thalassemia trait carriers.

The authors report 5 cases of porphyria cutanea tarda (PCT) occurring in carriers of the beta-thalassemia trait. This hematologic condition may be responsible for the development of liver damage resulting in siderosis, higher susceptibility to hepatitis B virus infection, and the earlier appearance of clinical features of PCT. Consequently, the authors propose chelation therapy with long-term subcutaneous desferioxamine infusions.

[Diagnostic-prognostic significance of long-term (5-11 years) variations in serum GOT-GPT levels in the blood]. / Sul significato diagnostico-prognostico delle variazioni a lungo termine (5-11 anni) dei valori sierici di AST e ALT.

A group of 48 patients (42 suffering from hepato-biliary diseases and 6 without hepatic diseases) was followed by the authors for a period lasting from 5 to 8 years, 13 out of them for longer. The hepatic disease was assessed on the basis of physical examination, current liver chemistry and proper and specific instrumental procedures. Initial and final diagnosis and the aminotransferases (AST, ALT) trend in years were carefully considered. First of all it was concluded that no advantage is obtained in monitoring the two aminotransferases instead of one alone. Moreover it is stressed the opportunity of referring aminotransferases activities in a simple way such as per cent of variation as referred to considered upper normal value differing from one to the other laboratory. The aminotransferase increase maintains an important and diagnostic significance in acute liver damage such as in acute hepatitis. An inappreciable prognostic value may be drawn from the follow up of these enzymatic parameters: for example the development of posthepatic fibrosis, or cirrhosis or hepatoma cannot be foreseen on the basis of the aminotransferases trend. A greater variability and sharp increases in AST-ALT values are recorded in patients with biliary gallstones.

Faecal and urinary coproporphyrin isomers in biliary atresia and neonatal hepatitis.

Based on the assumption that faecal and urinary coproporphyrin excretion is closely dependent on biliary function, coproporphyrin excretion was investigated in severe cholestatic diseases in infants and adults. The following subjects were investigated: biliary atresia (5), neonatal hepatitis (3), normal infants (11), adults with biliary obstruction (5) and normal adults (18). Urinary and faecal coproporphyrin concentrations were determined by solvent partition methods, and the isomers (I and III) were separated by thin-layer chromatography with direct spectrofluorometric scanning. The results showed a significant increase in urinary coproporphyrin in biliary atresia and neonatal hepatitis and in adults with biliary obstruction. All the cholestatic diseases showed the same marked increase in urinary isomer I. In biliary atresia and neonatal hepatitis there was a significant decrease in faecal coproporphyrin and a concomitant increase in isomer III (of bacterial origin) which was related to the extent of the biliary defect. Determination of urinary and faecal coproporphyrin, and particularly of the isomer distribution, may be a sensitive tool for diagnosis.

Selective amono acid solutions in hepatic encephalopathy treatment (a preliminary report).

Hepatic encephalopathy may complicate chronic liver diseases and portosystemic shunting. Recently it has been suggested that a change in the blood amino acid pattern (with elevated phenylalanine, tyrosine, methionine and depressed branched chain amino acids) and increased levels of octopamine and phenylethanolamine (a false neurotransmitter) are related causally to neurological symptoms. Previous works have suggested that an amino acid mixture of special formulation (enriched in branched chain amino acids and poor in aromatic amino acids) can normalize the plasma amino acid levels and consequently improve the neurological state. In order to test the actual therapeutic effect of these specially formulated solutions, the following therapeutic protocols were administered to 3 groups of cirrhotic patients: group 1 (15 cases): lactulose; group 2 (8 cases): special amino acid solutions; group 3 (8 cases): special amino acid solutions and lactulose. Clinical, laboratory and EEG examinations in a preliminary experiment, before and after treatment, showed that the best effect was obtained by the combined treatment with amino acid solutions and lactulose.