Periodic meningitis and familial Mediterranean fever.

A 33-year-old man had had, since he was 20 years old, recurrent attacks of fever, rash, and aseptic lymphocytic meningitis. A nephrotic syndrome developed that was found, on renal biopsy, to be due to amyloid deposit. After colchicine therapy, no further recurrence of fever and meningitis was observed. These findings suggest that aseptic periodic meningitis (Mollaret's syndrome) should be considered as an unusual manifestation of familial Mediterranean fever.

The evolution of alpha heavy chain disease.

Two patients with alpha heavy chain disease are described. In the first patient, treatment with cyclophosphamide, prednisone and doxycycline was associated with a 28 month-long remission and the disappearance of the paraprotein and lymphoplasmocytic infiltration of the intestine. Shortly afterwards, a retroperitoneal immunoblastic lymphoma was found associated with an immunoglobulin G-kappa-paraproteinemia, and gamma heavy and kappa-light chains in the urine; the intestinal biopsy specimen was normal. In the other patient, the alpha chain only appeared two years after the malabsorption syndrome. The fact that in the first, apparently cured patient, a tumor of different anatomic site and secretory capacity appeared, suggests the existence of a B-cell neoplasia of different clone from that which gave rise tothe original disease. In the second patient, it is probable that only the increase in the mass of neoplastic cells led to the detection of the protein abnormality, or alternatively the antigenic-oncogenic stimulus led to the abnormal secretion only after two years.

The effect of heparin on serum lipoprotein-X.

Serum lipoprotein-X was investigated in 12 patients with cholestasis in basal conditions and 20 minutes after intravenous heparin. In all cases, lipoprotein-X was positive in the first sample and became negative after heparin. A decrease in triglycerides was observed in all patients after heparin with an increase in free fatty acids and mobilization of prebetalipoproteins, and a transformation of lecithin into lysoleithin in 4 cases after heparin. These facts suggest that heparin plays an important role in the mobilization of serum lipoprotein-X.

The clearance of lipoprotein X in vitro, and effect of phospholipase A on serum lipoprotein X.

The in vitro effect of post-heparin and post-heparin plus post-protamine normal serum on serum lipoprotein X (LP-X) is described. Serum LP-X is cleared after incubation with post-heparin normal serum, and serum LP-X remain unmodified when it is incubated with post-heparin plus post-protamine normal serum. The action of phospholipase A from snake venom on serum LP-X is also studied. A very small quantity of phospholipase is necessary to degrade LP-X and to transform lecithin into lysolecithin. It is concluded that phospholipase seems to be the enzyme that most likely induces the LP-X changes after heparin administration.

Hepatitis in exanthematous mediterranean fever.

In 38 patients suffering from rickettsiosis caused by Rickettsia conorii (Mediterranean Exanthematous Fever), hepatic involvement was studied via laboratory tests and in 26 cases by means of liver biopsy. SGOT, SGPT and alkaline phosphatase were found to be elevated in more than half of the patients (SGOT 74.4 +/- 93 U.K., SGPT 82.2 +/- 93 U.K., a.p. 58 +/- 21 mU/ml). In 14 patients, liver biopsy showed the existence of inclusion corpuscles in Kupffer's cells. Electron microscopic study demonstrated the existence of phagosomes inside the epithelioid cells, which, however, were difficult to categorize. The frequent existence of granulomatous hepatitis in this rickettsiosis was confirmed, while the presence of the infecting agent in the liver could not be established.

Diagnostic usefulness of anti-HBc-IgM and anti-HD antibodies in acute viral hepatitis.

To evaluate diagnostic improvement achieved with the determination of antibody to hepatitis core antigen of IgM type (anti-HBc-IgM) and antibody to delta antigen (anti-HD) in acute type B hepatitis, 155 patients (of whom 100 were drug addicts) were studied, using these markers in association with the classical markers of viral hepatitis (anti-HBc, anti-HBs, anti-HAV-IgM and anti-cytomegalovirus antibodies). With classical diagnostic criteria, 121 patients would have been diagnosed as having B hepatitis (HBs Ag and/or anti-HBc antibody positive without anti-HBs antibody), 33 non A non-B hepatitis (all markers negative or anti-HBc and anti-HBs positive) and 3 A hepatitis (anti-HAV-IgM antibody positive). With the current markers, 89 were B hepatitis (anti-HBc-IgM antibody positive), 18 were simultaneous B and D infections (anti-HBc-IgM and anti-HD antibodies positive), 6 were D hepatitis (anti-HBc-IgM antibody negative and anti-HD antibody positive), 39 non-A non-B hepatitis (anti-HBc-IgM, anti-HD, HAV-IgM and anti-cytomegalovirus antibodies negative) and 3 hepatitis A. Of the 121 cases initially diagnosed as B hepatitis, only 79 were actually B hepatitis, 16 were simultaneous B and D infections, 6 D hepatitis, 18 non-A non-B hepatitis and 2 A hepatitis. Of the 33 cases considered to be non A non-B hepatitis, only 21 were really non-A non-B hepatitis; 10 were B hepatitis and 2 B and D hepatitis. When anti-HBc-IgM and anti-HD antibodies were determined, the initial diagnosis was modified in 54 of 155 cases (35 p. 100).(ABSTRACT TRUNCATED AT 250 WORDS)

[Treatment of acute brucellosis with cotrimoxazole, doxicyclin and streptomycin. A comparative study (author's transl)]. / Tratamiento de la brucelosis agunda con cotrimoxazol, doxiciclina y estreptomicina. Estudio comparativo.

Human brucellosis can be managed with different therapeutic measures. The present study compares the therapeutic efficacy of the classical treatment with streptomycin, doxicyclin and cotrimoxazole, every one of the treatment regimes being applied to 19 patients with acute brucellosis. The results do not demonstrate an advantage of the new association over the classical treatment. Relapses, time elapsed until the patient was afebrile and drug tolerance were similar for both treatments. The classical treatment has a lower cost but the new association implies more patient comfort because the number of pills to be ingested daily is reduced to less than half, a fact that could make it the treatment of choice.

[Diagnosis of viral hepatitis type A by radioimmunoassay (author's transl)]. / Diagnóstico de la hepatitis A por radioinmunoanálisis.

In the present work radioimmunoassay technique detecting antibodies have been used to diagnose viral hepatitis type A. Twenty-nine patients with sporadic acute hepatitis were studied. All of the cases were negative to surface antigen of viral hepatitis type B. Blood samples were obtained at the onset of clinical picture and on subsequent weeks. Antibody levels (anti-hepatitis virus A) showed a significant increase between the second and the sixth week in 21 cases (72.4 percent). An increased antibody titer in a single determination, however, is not sufficient for the diagnosis of hepatitis virus A, since antibodies against such virus are present in a large percentage of the normal population. At least two augmented titers in consecutive samples are necessary to diagnose viral hepatitis type A.

[Histologic evolution of acute alcoholic hepatitis]. / Evolución histológica de la hepatitis alcohólica aguda.

Although acute alcoholic hepatitis is a frequent disease with distinct histologic diagnosis, the prognostic factors of its evolution are largely unknown. The present report analyzes the data of 18 patients with acute alcoholic liver disease submitted to two liver biopsies with a mean interval of 35 months. The results demonstrate a favorable histologic evolution in six of seven patients that abandoned alcohol ingestion, and an evolution to chronic liver disease in the remaining case. Out of six patients with continuous alcohol ingestion the evolution was unfavorable in five, while one case evolved to healing. Centrilobular fibrosis disappeared in the six cases that discontinued alcohol ingestion and who had favorable evolution, and it persisted in four of the six patients that continued drinking. The results suggest that centrilobular fibrosis is not always an unfavorable prognostic marker, and that the evolution of acute alcoholic hepatitis is not exclusively dependent on the continuation of alcohol abuse.

[Primary liver carcinoma and markers of virus of type B hepatitis (author's transl)]. / Carcinoma primitivo de hígado y marcadores del virus de la hepatitis B.

Association of primary liver carcinoma with virus of type B hepatitis has been demonstrated around the world, especially in African and Asian countries where high titles for surface antigen of B hepatitis (HBsAg) have been found. Presence of viral markers of B hepatitis has been studied in a group of 34 patients with primary liver carcinoma, in 139 cirrhotic patients, and in 100 normal individuals. Positive titles of HBsAg or of antibodies against "core" antigen (anti-HBc) without evidence of antibodies against HBsAg (anti-HBs) were considered as positive markers for virus B infection. Percentages of positive markers in the series studied were as follows: 52% in the cases of primary liver carcinoma, 38% in the cirrhotic cases, and 5% in the control subjects. There is a significant difference in relation to the incidence of viral markers between patients with liver carcinoma and normal controls. A possible role of the virus of B hepatitis in the pathogenesis of primary liver carcinoma could be suggested on the basis of these results.

[Granulomatous hepatitis. Etiologic study of 107 cases (author's transl)]. / Hepatitis granulomatosa, estudio etiológico de 107 casos.

An etiologic study was made of 107 cases of granulomatous hepatitis which were observed in a Department of Internal Medicine between January, 1971 and December, 1977 (excluding the hepatobiliary diseases). The most common etiology was tuberculosis (30 cases, 28 percent) followed by sarcoidosis (19 cases, 17.7 percent), Mediterranean exanthematous fever (13 cases, 12.1 percent), brucellosis (8 cases, 7.4 percent) typhoid fever (7 cases, 6.5 percent) and the idiopathic forms (8 cases, 7.4 percent). A lower rate of incidence was among Hodgkin's disease, toxoplasmosis, adenocarcinomas, leprosy, and those of unknown etiology, classified in this way because the study and follow-up of the patients could not be completed. There were, moreover, individual cases caused by mononucleosis, BCG reaction, hypogammaglobulinemia, celiac disease, and temporal arteritis. From a clinical point of view 50 percent of the patients had hepatomegaly and moderate disturbance of the liver enzymes. The most important enzymatic increases were detected in the cases caused by brucellosis; in the cases which were secondary to sarcoidosis the liver enzymes were normal. A comparison is established between the etiologic incidence of the present series and of others published in the literature. The causes and diagnostic problems of this type of lesion are discussed.

[Clinical course characteristics of polymyalgia arteritica. Study of 16 cases]. / Características clínico-evolutivas de la polimialgia arterítica. Estudio de 16 casos.

Clinical and biological characteristics are studied in 16 patients with polymyalgia arteritica. 12 of them were diagnosed by biopsy of the temporal artery and the other 4 because they presented clinical, biological data and a high response to corticosteroids. There were no differences according to sex. Most patients (75%) had symptoms since 1-6 months, headache being the most common (75%). Fever (56%), polymyalgia (50%), weight loss (37%), intermittent claudication, loss of vision and arthritis (12%) were the symptoms seen in these patients. ERS was high in all cases, hemoglobin was less than 8 g/100 ml in 8 cases and an increase of alfa-2-globuline was found in 8 patients. Temporal artery palpation was abnormal in 11 patients. Two of 5 patients who presented a normal arterial palpation had a positive biopsy. All patients received 6-metil-prednisolone. 3 are well after 3, 4 and 6 months therapy. 8 are also well but receiving small doses of steroids as treatment.