Quinacrine added to ongoing therapeutic regimens attenuates anticardiolipin antibody production in SLE

The benefit of combining quinacrine (Qn) with hydroxychloroquine (HCQ) in the treatment of systemic lupus erythematosus (SLE) was previously re-evaluated by us. In our current study we observed that, in 11 active SLE patients (SLEDAI score 5-12), the addition of Qn (100 mg/day) to their existing ongoing therapeutic regimens resulted in a significant attenuation of their previously persistent anticardiolipin antibody (aCL) response. This was in comparison with a matched non-Qn treated control group composed of 14 randomly chosen aCL-positive SLE patients with a similar SLEDAI score 6-10. Prior to Qn treatment the therapeutic regimens of 12 months' duration, included in all cases HCQ (400 mg/day), in many cases prednisone (P, 10-20 mg/day) and in some additional cases immunosuppressive drugs. SLEDAI scores and aCL levels were monitored during the entire follow-up period which totaled 24 months in the study group and 15-18 months in the controls. Along with the beneficial effect of the added Qn on SLEDAI scores, aCL disappearance was documented in eight of 11 patients and remained negative during 8-12 months of follow-up (P = 0.004), compared with such a change in only three of 14 non-Qn treated aCL-positive patients (P = 0.18). We conclude that the added Qn treatment to former established therapeutic protocols may eliminate aCL response in SLE patients. Whether this agent's effect is permanent needs further elucidation.     

Huge left atrial thrombus and valve degeneration in a patient with a bioprosthetic, porcine, mitral valve

A 43 year old man with a Hancock porcine bioprosthetic valve in the mitral position developed a huge thrombus filling the entire left atrium and chronic degeneration of the bioprosthetic mitral valve. The effective valve orifice was less than 2 mm. These severe findings leading to the patient's death remained undetected while he was alive. This case illustrates the great need for a serial phonoechocardiographic studies in all patients with prosthetic valves.     

Microscopic polyangiitis presenting as idiopathic pulmonary fibrosis: is anti-neutrophilic cytoplasmic antibody testing indicated?

We report a 55-year old woman with microscopic polyangiitis who presented with idiopathic pulmonary fibrosis and 1 year later developed hematuria and proteinuria. She had a high serum level of perinuclear anti-neutrophilic cytoplasmic antibodies. Renal angiogram was normal. The diagnosis of microscopic polyangiitis was confirmed by renal biopsy, which showed pauci-immune crescentic glomerulonephritis. The patient received immunosuppressive therapy and improved markedly. Consideration of small vessel vasculitis is important in the differential diagnosis of idiopathic pulmonary fibrosis.     

Spontaneous regression of congenital leukaemia with an 8;16 translocation

Congenital leukaemia (CL) is a rare disorder that presents with extramedullary infiltrates and a myeloid phenotype. CL can progress rapidly without adequate treatment, but, paradoxically, may also remit spontaneously. Because of the significant toxicity involved in delivering chemotherapy to newborns, it is important to identify those newborns who may not require treatment. We describe an infant who presented at 1 week of age with congenital myeloid leukaemia. Cytogenetic analysis revealed a t(8;16)(q11;p13) translocation. The infant's leukaemia underwent a spontaneous regression. This case further confirms the possibility of spontaneous remission in congenital leukaemia. Moreover, it suggests that the presence of a clonal cytogenetic aberration does not preclude the possibility of a spontaneous regression in CL.     

Arthropathy,hypouricemia and normal serum iron studies in hereditary hemochromatosis

A patient manifesting the arthropathy of hemochromatosis without abnormal serum iron studies is described. Hemochromatosis was confirmed by liver biopsy. This case serves to emphasize the diagnostic value of the characteristic arthropathy of hemochromatosis. Our observations in this patient support the hypothesis that the pathogenesis of hereditary hemochromatosis differs from that of acquired iron overload states. The concurrent presence of hypouricemia is explored in this patient and in 18 other patients with hereditary hemochromatosis. Men with hereditary hemochromatosis were found to have lower serum uric acid levels than expected. In our patient, a renal defect in tubular reabsorption of uric acid appears responsible for hypouricemia.The apparent association of hemochromatosis and hypouricemia deserves further investigation.     

Subclinical atherosclerosis in psoriatic arthritis: a case-control study

OBJECTIVE: To investigate the prevalence of subclinical atherosclerosis among patients with psoriatic arthritis (PsA). METHODS: Forty patients with PsA were enrolled. Controls were matched by age, sex, and atherosclerotic risk factors. All patients and controls underwent duplex scan of the carotid arteries. Carotid intima-media thickness (IMT) was evaluated and the presence of atherosclerotic plaques was recorded. The plaques were graded and carotid plaque index was calculated. RESULTS: Patients with PsA had a higher IMT (mean +/- standard deviation, 1.04 +/- 0.35 mm vs 0.88 +/- 0.29 mm in controls; p = 0.03), and had a higher carotid plaque index than did matched controls (2.3 +/- 2.6, compared to 1.12 +/- 2.09; p = 0.03). Multivariate analysis demonstrated that PsA status as well as age and triglyceride levels were associated with the presence of carotid plaque. Other traditional risk factors were more prevalent among patients with PsA; however, they were not statistically significant. CONCLUSION: Our study demonstrates that patients with PsA may have an increased prevalence of subclinical atherosclerosis. These findings may not be solely attributable to traditional risk factors alone. Special attention and strict control of atherosclerotic risk factors in patients with PsA is warranted.     

Association between fruit and vegetable intake and change in body mass index among a large sample of children and adolescents in the United States

OBJECTIVE: To assess whether intake of fruits and vegetables was associated with change in body mass index (BMI) among a large sample of children and adolescents in the United States. DESIGN: Prospective cohort study of children and adolescent who were 9-14 y of age in 1996, when the study began. SUBJECTS: The subjects included 8203 girls and 6715 boys in an ongoing cohort study who completed at least two questionnaires between 1996 and 1999. MEASUREMENTS: Fruit and vegetable intake was assessed in 1996-1998 with a validated food frequency questionnaire designed specifically for children and adolescents. The outcome measure was change in age- and gender-specific z-score of BMI (kg/m(2)). Self-reported weight and height, which were used to calculate BMI, were collected annually from 1996 to 1999. RESULTS: During 3 years of follow-up, annual changes in BMI were slightly greater among the boys than the girls. After controlling for Tanner stage of development, age, height change, activity and inactivity, which are known or suspected predictors of change in BMI, among the girls there was no relation between intake of fruits, fruit juice, or vegetables (alone or combined) and subsequent changes in BMI z-score. Among the boys, intake of fruit and fruit juice was not predictive of changes in BMI, however, vegetables intake was inversely related to changes in BMI z-score (beta per serving=-0.003). However, after adjusting for caloric intake, the magnitude of the effect was diminished and no longer significant. CONCLUSION: Our results suggest that the recommendation for consumption of fruits and vegetables may be well founded, but should not be based on a beneficial effect on weight regulation.     

Get secure soon: attachment in abused adolescents and young adults before and after trauma-focused cognitive processing therapy

Severe posttraumatic stress symptoms (PTSS) are connected to a variety of health-related and interpersonal problems, among them are the insecure attachment orientations. However, psychotherapy seems to improve not only PTSS but also attachment insecurities. In a large multicenter, randomized clinical trial, the attachment characteristics and PTSS of 85 adolescents and young adults (aged 14–21 years) with clinically relevant abuse-related PTSS were assessed at study entry, at the end of treatment, and 3 months after the end of treatment. Participants were randomized either to a developmentally adapted cognitive processing therapy (D-CPT) or to a wait-list with treatment advice (WL/TA). The purpose of the study was to analyze the association between PTSS and attachment at study entry as well as changes in attachment during the trial. We found that attachment-related avoidance (AR avoidance) was positively associated with PTSS from both self-reports and clinician ratings, whereas attachment-related anxiety (AR anxiety) was only related to self-reported PTSS (Pearson correlation coefficients between 0.37 and 0.46). Changes in AR anxiety occurred in both conditions at some point during the study (baseline to 3-month follow-up effect size was d = 0.60 for D-CPT and d = 0.44 for WL/TA) whereas for AR avoidance, only participants in D-CPT improved significantly (baseline to 3-month follow-up effect size was d = 0.75). The results indicate that PTSS and attachment are connected. Positive changes in attachment insecurities brought about by trauma-focused psychotherapy seem possible.

Trial registration: German Clinical Trials Register (DRKS); Germanctr.de; identifier: DRKS00004787; date of registration: 18 March 2013.