Sickle cell thalassemia in pregnancy: a case report.

A 31-year-old pregnant woman at 20 weeks' gestation was sent to our hospital for fever, anemia, and arthralgias. As she was known to be a double heterozygote for beta-thalassemia and hemoglobin S, a diagnosis of hemolytic anemia caused by sickled red cells vasocclusive crises was made. The patient was submitted to partial exchange transfusion (PET) with a complete recovery. A second PET was performed at 36 weeks' gestation, and elective cesarean section was performed at 37 weeks, with the birth of a normal female neonate.

Hepatitis C virus testing in primary biliary cirrhosis.

We retrospectively investigated anti-HCV prevalence in a series of 160 consecutive patients with primary biliary cirrhosis who presented between 1980 and 1989. Of these, 19 (12%) were positive for anti-HCV by C-100 ELISA. Serum IgG levels were significantly higher in anti-HCV-positive patients and correlated to optical density values. A serum sample was again collected from all the patients from the same series who were seen in 1990 for follow-up, after a median period of 32 months. Anti-HCV positivity was found to be substantially unchanged in this subgroup of patients when the freshly drawn blood samples were retested with C-100 ELISA, while it increased from 10% to 17% when second generation ELISA was used. Three of the C-100 ELISA positive samples were C-100 RIBA reactive, and six of the second generation ELISA positive samples were 4-RIBA reactive. The HCV genome was not detected in any of the seven anti-HCV C-100 ELISA and second generation ELISA positive sera which were studied by polymerase chain reaction, including four cases confirmed by 4-RIBA. Life expectancy, as determined by survival analysis, did not differ significantly between anti-HCV-positive and -negative patients. These findings suggest that anti-HCV positivity does not influence the clinical presentation and course of primary biliary cirrhosis.

Antibodies to hepatitis C virus in primary biliary cirrhosis.

We investigated the prevalence of anti-HCV in 160 consecutive patients with primary biliary cirrhosis. By ELISA, 19 (12%) were positive, as compared to a 68% prevalence in 135 patients with chronic non-A, non-B hepatitis. Serum IgG levels were significantly higher in the anti-HCV positive group. By RIBA, seropositivity was confirmed for 4 patients, whereas 7 were indeterminate. A slight, non-significant reduction of life expectancy was found in anti-HCV positive patients. Until reliable and independent confirmatory tests become available, definitive conclusions on the importance of anti-HCV positivity in primary biliary cirrhosis are improper.

Effect of UVB plus tar therapy on serum levels of interleukin-2 receptors in patients with psoriasis.

Baseline serum levels of interleukin-2 receptor (IL2R) were measured in 65 patients with active psoriasis. IL2R levels in psoriatic patients were significantly higher than in healthy controls (582.4 +/- 289.23 u/ml vs. 369.64 +/- 111.10 u/ml; P less than 0.05), but did not differ statistically from values found in an atopic dermatitis control group (619.88 +/- 254.27 u/ml). Sex, age and severity of the disease do not affect levels of IL2R. The same IL2R levels were measured in 26 psoriatic patients receiving UVB plus tar therapy. This therapy, continued until clinical remission, lowered IL2R levels to values comparable to controls. This decrease may be due to an immunosuppressive effect of therapy.

Soluble interleukin 2 receptor in lung cancer. An indirect marker of tumor activity?

Circulating levels of the soluble interleukin 2 receptor (sIL-2R) could provide an in vivo measure of the immunologic response to human tumors. We performed a total of 326 sIL-2R serum assays in 126 patients with lung cancer (67 at diagnosis, 59 during and after treatment), 112 patients with pulmonary benign diseases, and 63 voluntary healthy subjects. Patients with lung cancer had a median value of sIL-2R of 791 U/ml, which was superior to that of both controls (398 U/ml, p less than 0.001) and patients with noninflammatory benign diseases (583 U/ml, p less than 0.02). However, infectious pulmonary disorders, such as tuberculosis and pneumonia, were associated with the highest values of the substance (median, 1150 U/ml; p less than 0.001). At the diagnosis of lung cancer, sIL-2R correlated neither with the stage of disease nor with the cell type. On the contrary, posttreatment levels of the receptor were significantly related to disease status (RO = .41, p less than 0.002), particularly in the subgroup of nonsurgical patients (RO = .48, p less than 0.001). Patients with abnormal sIL-2R levels had a nearly significant reduction in survival as compared with patients with normal values (p less than 0.1). Measurements of sIL-2R could be useful in monitoring patients under treatment for bronchogenic carcinoma, as well as in prognostication. In this setting, sIL-2R might open a new class of biologic markers, providing information that is complementary to those of the more classic tumor-derived markers.

[Factor VII and cardiovascular risk in obese subjects]. / Fattore VII e rischio cardiovascolare negli obesi.

We investigated whether or not obesity is related to increased factor VII activity. We studied 70 obese subjects (aged 25 to 50 years, 25 males and 45 females, body mass index (BMI): mean +/- SD = 32.44 +/- 5.44) and 33 non-obese subjects (aged 25 to 50 years, 12 males and 21 females, BMI: mean +/- SD = 21.80 +/- 1.70). None of them were smokers or affected by hyperlipidemia, diabetes mellitus, impaired glucose tolerance or arterial hypertension. Factor VII activity was measured by the coagulometric method. We found higher factor VII activity in obese subjects (115.74 +/- 26.10%) than in healthy subjects (98.55 +/- 23.49%, p less than 0.005). Increased factor VII levels could determine a thrombophilic state involved in the genesis of cardiovascular accidents in obesity.

Hepatitis B vaccination of 113 hemophiliacs: lower antibody response in anti-LAV/HTLV-III-positive patients.

One-hundred thirteen adults and children with hemophilia or other congenital bleeding disorders were vaccinated against the hepatitis B virus. Each patient was given three subcutaneous injections of the vaccine at monthly intervals and then a fourth booster dose 14 months after the first. The vaccine was highly immunogenic, since 111 of 113 patients (98%) produced anti-HBs (10 mIU/ml or more). After the first three vaccine doses and after the booster dose, ten anti-LAV/HTLV-III-positive hemophiliacs produced anti-HBs but had a lower average titer than anti-LAV/HTLV-III-negative hemophiliacs. Of the 23 patients treated with concentrates in the 15 month postvaccination period only, none acquired HBV infection. Of the 50 patients treated with concentrates also in the 6 month prevaccination period, one developed hepatitis B. In summary, the vaccine was highly immunogenic in both children and adults with hemophilia; anti-LAV/HTLV-III-positive patients responded to the vaccine, but the average anti-HBs response was lower; no case of hepatitis B occurred in patients treated with concentrates only in the postvaccination period.

Hepatitis B virus infection in multitransfused haemophiliacs.

A longitudinal study of 44 haemophilic children, all in a treatment programme with factor concentrates, was undertaken to evaluate the occurrence, characteristics, and evolution of hepatitis B virus (HBV) infection. Twenty four children (55%) (group I) showed signs of HBV infection, while 20 (45%) (group II) did not. Age at onset of treatment, number of infusions, and total amount of concentrate received did not show significant differences between the two groups. In group I only four children (16%) had symptomatic acute hepatitis. Chronic liver disease was present in nine patients (38% of infected children). The early age of infection would seem to be an important factor for predicting chronic evolution. Evidence of delta infection in three children with severe liver disease seemed to confirm the high pathogenicity of this agent. Because of the risks associated with chronic HBV infection a careful follow up of patients positive for hepatitis B surface antigen is mandatory.

Hepatitis B or non-A, non-B virus infection in multitransfused thalassaemic patients.

We undertook a four year study of 128 thalassaemic patients who had undergone several transfusions, to determine the incidence of hepatitis B virus markers and the activities of transaminases in their sera each month. The results showed that the possibility of these patients contracting hepatitis B virus infection is still high, although on only one occasion was a transient antigenaemia found, indicating low viral replication. Furthermore, the probability of contact with hepatitis B virus increases with the number of transfusions and, therefore, with age. About 25% of these patients were positive for hepatitis B markers and 80% for other hepatitis markers including the case of cytomegalovirus hepatitis.

Hemolytic disease of the newborn due to rare alloantibodies.

In the period January 1975 to December 1981, serum samples from 9,525 pregnant women were examined for irregular red cell antibodies. Red cell antibodies were found in 719 cases (7.5%); anti-D antibodies were present in 532 cases (5.5% of the whole series), rare antibodies (other than anti-D) in 187 cases (2%). Seven pregnant women with rare red cell antibodies were followed at our Institute up to delivery. The clinical management, which was based on the diagnostic and therapeutic procedures of hemolytic disease of newborn due to anti-D antibodies, is reported in detail. The findings underline the importance of careful screening for irregular red cell antibodies in all pregnant women, both Rh-negative and Rh-positive, and confirm the validity of Rh clinical management also for moderate or severe immunizations due to rare red cell antibodies.

[The prevention of liver disease in the hemophilic child]. / La prevenzione dell'epatopatia nel bambino emofilico.

The modern replacement therapy of inherited bleeding disorders has proved to be a major advance in the management of haemophilic children. However, the haemophiliacs, early treated with commercial clotting factor concentrates obtained from large amounts of plasma, are exposed to blood borne viruses responsible for post-transfusion hepatitis (PTH) and for their possible harmful long-term sequelae. Infact high prevalence of infection with hepatitis B virus, non-A, non-B agents, delta agent has been documented among haemophilic children. In this study we analyze the measures of surveillance at present available in order to reduce the risk of PTH in young haemophilic patients. Among these measures of prevention we point out the magnitude of administrating hepatitis B vaccine to susceptible children and of using antihaemophilic factor heat-treated to reduce infectivity in those children who have never been treated and without signs of active viral infections.

Hepatitis B prevalence and immunoprophylaxis with hepatitis B immunoglobulins in patients, relatives, and staff of a paediatric haemodialysis unit.

Hepatitis B virus (HBV) markers have been studied for 22 months in 16 haemodialysed patients, 60 relatives and 17 staff members of a paediatric haemodialysis unit, and in 9 children with chronic renal failure on conservative therapy. None of the 8 children negative for HBV markers and treated with specific immunoglobulins (HBIG) developed signs of hepatitis B. No hepatitis B occurred in 4 staff members after accidental infected needle prick and immunoprophylaxis, although one of them developed passive-active immunity. The immunoprophylaxis effectively prevented diffusion of HBV also in the relatives. Furthermore, HBsAg and HBeAg were found in urine samples of haemodialysed children with residual diuresis.

Acute hepatitis B in infants born to HBsAG asymptomatic carrier mothers.

Within an eight-month period, five cases of acute hepatitis B were observed in children born to asymptomatic HBsAg carrier mothers. At delivery, four mothers were HBeAg-positive and one was anti-HBe-positive. At an average age of 17+/-3 weeks the infants showed high transaminases levels and appearance of markers of HBV infection. Three children became HBsAG chronic carriers, one had a brief HBsAg-antigenemia (1 week), while the last one showed clinical and biochemical signs of hepatitis B in absence of detectable HBsAg but with anti-HBc and anti-HBe (window phase) and later became anti-HBs-positive.

Haemolytic disease of the newborn due to anti-Fya (first case reported in Italy).

A new case of haemolytic disease of the newborn due to anti-Fya antibodies is described. The maternal alloimmunization was related to a previous blood transfusion. The patient, her previous child and the donor of the blood transfused were tested. Foetal condition was monitored by measuring bilirubin in amniotic fluid on three different occasions during pregnancy. The infant developed a mild haemolytic disease that required treatment only with phototherapy.