Antineutrophil cytoplasmic autoantibodies and anti-Saccharomyces cerevisiae antibodies in inflammatory bowel diseases.

BACKGROUND/AIMS: Perinuclear antineutrophil cytoplasmic autoantibody is a marker for ulcerative colitis, and anti-Saccharomyces cerevisiae antibody is known to be associated with Crohn's disease. The purpose of this study was to search the value of detecting perinuclear antineutrophil cytoplasmic autoantibody and anti-Saccharomyces cerevisiae antibody for the diagnosis of Turkish inflammatory bowel disease patients. METHODS: Serum samples were obtained from 80 patients with ulcerative colitis, 61 patients with Crohn's disease and 40 healthy controls. Determination of both anti-Saccharomyces cerevisiae antibody and antineutrophil cytoplasmic autoantibody was performed with the standardized enzyme-linked immunosorbent assay. RESULTS: In cases with ulcerative colitis, 65% tested seropositive for antineutrophil cytoplasmic autoantibody, whereas the controls showed 2.5% positivity. In cases with Crohn's disease, 63.9% tested seropositive for anti-Saccharomyces cerevisiae antibody, whereas the controls showed 2.5% seropositivity. The combination of a positive anti-Saccharomyces cerevisiae antibody test and a negative antineutrophil cytoplasmic autoantibody yielded a sensitivity and specificity of 32.0% and 97.5%, respectively. The combination of a positive perinuclear antineutrophil cytoplasmic autoantibody and a negative anti-Saccharomyces cerevisiae antibody test yielded a sensitivity and specificity of 44.2% and 97.5%, respectively. CONCLUSIONS: Both serologic tests may aid in the differential diagnosis of inflammatory bowel disease.     

Rituximab plus ASHAP for the treatment of patients with relapsed or refractory aggressive non-Hodgkin’s lymphoma: a single-centre study of 20 patients

The anti-CD20 antibody rituximab improves the results of first-line therapy in aggressive non-Hodgkin’s lymphoma (NHL) of B cell lineage. The purpose of this retrospective study was to evaluate its efficacy and toxicity in combination with the doxorubicine, methylprednisolone, high-dose cytarabine, cisplatin (ASHAP) protocol, an established treatment regimen for relapsed or refractory aggressive NHL. After a median of four cycles, 9 of 20 patients treated achieved a complete remission and 6 a partial remission, resulting in a total response rate of 75%. Remissions were not only seen in patients with relapsed lymphomas but also in patients with primary refractory or transformed indolent lymphomas. The outcome in cases with an international prognostic index score ≥2 was poor. Five of 15 responders received consolidating high-dose therapy with autologous stem cell transplantation. All of them are in ongoing remission. The main toxicity was myelosuppression, with neutropenias or thrombocytopenias of World Health Organization (WHO) grades III or IV developing in more than 90% of the cycles. There was one therapy-related death due to neutropenic sepsis. Non-hematologic toxicity was generally mild. At the time of analysis, six patients have died. After a median observation time of 17.5 months, the 2-year overall survival rate is 62%. ASHAP plus rituximab is an active and well-tolerated salvage protocol for patients with relapsed or refractory aggressive NHL, which compares favourably with other immuno-chemotherapy regimens, especially in patients with primary refractory or transformed indolent lymphomas.     

Effects of normal blood pressure, prehypertension and hypertension on left ventricular diastolic function and aortic elastic properties

Background. The Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure (BP) provides guideline for the new category of BP levels as normal, prehypertension (PHT), and hypertension. Although PHT is associated with a markedly increased risk of developing hypertension within 4 years, its prognostic significance and predisposition to target-organ damage is unknown. Accordingly, we evaluated the effects of normal BP, PHT and hypertension on left ventricular (LV) diastolic function and aortic elasticity, which are sensitive indicators of target-organ damage. Methods. We evaluated LV diastolic function and aortic elastic properties of 60 subjects with PHT, 70 patients with hypertension and 50 normotensive healthy volunteers using transthoracic echocardiography. None of the subjects had any systemic disease. Results. LV diastolic function was more significantly impaired in the hypertension group than in the PHT group compared with controls, but it was not strongly different between the PHT and control group. Aortic distensibility was significantly lower, and aortic stiffness index was significantly higher in both the hypertension and the PHT group than those in the control group. However, aortic elastic properties did not significantly differ between the PHT and hypertension groups. Furthermore, we found that the presence of the PHT was significant predictor of impaired aortic elasticity in a multivariable model that adjusted for other variables. Conclusions. Aortic elastic properties are significantly and LV diastolic function is slightly impaired in subjects with PHT, and impairment of aortic elasticity is as severe as that in hypertension.     

Myocardial tissue velocities in neonates

BACKGROUND: Tissue Doppler echocardiography (TDE) has been applied to the children age group; however, myocardial tissue velocities of neonates have been reported by few researchers. METHODS: Myocardial velocities along the longitudinal axis of the left ventricle were measured in two groups: 50 term neonates within 5 days after birth and 54 healthy children by TDE. Sample volumes were placed in mitral lateral annulus (MLA), mitral medial annulus (MMA), and in the basal and middle parts of the left ventricular lateral wall (LVLW) and interventricular septum (IVS). RESULTS: The highest myocardial velocities were obtained from MLA. In all segments, the mean systolic velocity (Sw) was found significantly lower than the mean early diastolic (Ew) and atrial systolic velocities (Aw) (P < 0.0001). The mean Aw was the dominant component in the MMA and were significantly higher than Ew (P < 0.001). The mean Ew/Aw in the MMA and MLA increased significantly while replacing the Doppler beam from base to apex. TDE indexes of neonates, except Aw obtained from MLA and MMA, were significantly lower than that of children group (P < 0.0001 and P < 0.0001, respectively). The mean E/Ew ratio of the left ventricle in neonates was found to be significantly higher than that found in children group (P < 0.0001). CONCLUSION: In neonates, the diastolic and systolic myocardial velocities of the left ventricle were significantly lower than those measured in the children group. Decreased myocardial tissue values of neonates might be reflecting the immaturity of neonatal myocardium.     

Acute Lymphoblastic Leukemia Occurring in a Case of Chronic Lymphocytic Leukemia

Chronic lymphocytic leukemia (CLL) is a disease characterized by variable clinical presentation and a prolonged course. However, a small proportion of cases may evolve to more aggressive forms. These include acute leukemic blastic crises of both myeloid and lymphoid types, prolymphocytoid transformation, lymphoma and multiple myeloma. We describe a case of B-CLL treated with chlorambucil, in whom a picture of acute lymphoblastic leukemia of pre-B type developed. The diagnosis of both forms of leukemia is well documented on the basis of morphological, cytochemical and immunological findings. Documentation and investigation of cases of ALL occurring in CLL is of importance to determine whether the transformation represents a clonal evolution of the original CLL or an independent second malignancy in an immunocomprimised patient.     

A Rare Combination: Congenital Adrenal Hyperplasia Due To 21 Hydroxylase Deficiency and Turner Syndrome

A combination of Turner syndrome (TS) and classical congenital adrenal hyperplasia (CAH) is rare. A one-day-old newborn was referred to our hospital with ambiguous genitalia. The parents were third-degree relatives. The infant’s weight was 3350g (50-75p), and the head circumference was 34.5cm (50p). The gonads were nonpalpable. Presence of a 3 cm phallus, one urogenital opening into the perineum, and incomplete labial fusion were identified. Laboratory tests revealed a classical type of CAH due to 21-hydroxylase deficiency. Karyotyping revealed a 45X0(35)/46XX(22) pattern with negative sex-determining region Y (SRY) on gene analysis. At the most recent follow-up visit, the patient appeared to be in good health - her height was 70.4 cm [-1.5 standard deviation (SD)] and her weight was 9.8 kg (0.3 SD). She was receiving hydrocortisone in a dose of 10 mg/m²/day, fludrocortisone acetate in a dose of 0.075 mg/day, and oral salt of 1 g/day. System examinations were normal. The patient’s electrolyte levels were found to be normal and she was in good metabolic control. The findings of this patient demonstrate that routine karyotyping during investigation of patients with sexual differentiation disorders can reveal TS. Additionally, signs of virilism should always be investigated at diagnosis or during physical examinations for follow-up of TS cases. [i][/i]SRY analysis should be performed primarily when signs of virilism are observed. CAH should also be considered in patients with negative [i]SRY[/i].Conflict of interest:None declared.