Repeated Remissions of Cushing's Disease Due to Recurrent Infarctions of an ACTH-Producing Pituitary Macroadenoma

Infarction of prolactin-secreting or growth hormone-secreting pituitary adenomas is not unusual. However, Infarction of ACTH-secreting adenomas has rarely been reported. Cyclical course of Cushing's syndrome alternating with adrenal insufficiency due to recurrent infarction of an ACTH-secreting pituitary adenoma has not been reported. We report here a 20-year-old lady who presented with florid signs of Cushing's syndrome but was found to have adrenal insufficiency on biochemical evaluation. Magnetic resonance imaging (MRI) of the pituitary gland showed that she had infarction of an ACTH-secreting macroadenoma. Over the next 6 years, her disease ran a cyclical course characterized by periods of hypercortisolism alternating with adrenal insufficiency due to repeated episodes of infarctions of the ACTH-secreting pituitary macroadenoma with corresponding changes in the pituitary adenoma on serial MRIs. The case alerts clinicians to this possibility when a patient presents with clinical picture of Cushing's syndrome but has adrenal insufficiency on biochemical testing. It also suggests that silent or subclinical infarction of pituitary adenomas is not uncommon and is probably under diagnosed.     

Dose-dependent effects of astaxanthin on cortical spreading depression in chronically ethanol-treated adult rats

Background: The consumption of alcoholic drinks is a frequent drug‐abuse situation, which is associated to a wide variety of pathological disturbances affecting several organs, including the brain. We have previously shown in the developing rat brain that ethanol intake facilitates the propagation of cortical spreading depression (CSD), an excitability‐related neural phenomenon present in several animal species. This electrophysiological effect was attenuated by a shrimp (Litopenaeus vannamei) carotenoids extract. Here we investigated the effects of pure astaxanthin, the main carotenoid found in shrimp, on CSD. Methods: Adult Wistar rats were treated per gavage, during 18 days, with 2.5, 10 or 90 μg/kg/d astaxanthin dissolved in ethanol (3 g/kg) and CSD was recorded on the cortical surface 1 to 3 days thereafter. Four groups, treated respectively with ethanol, distilled water and soybean oil with‐ and without astaxanthin were also studied for comparison with the ethanol + astaxanthin groups. Results: Ethanol‐treated rats displayed higher CSD‐velocities (mean values, in mm/min, per hour of recording ranging from 4.08 ± 0.09 to 4.12 ± 0.16), compared to the distilled water‐group (from 3.19 ± 0.13 to 3.27 ± 0.06). Addition of astaxanthin to ethanol lead to lower CSD‐velocities in a dose‐dependent manner, ranging from 3.68 ± 0.09 to 3.97 ± 0.22 for the 2.5 μg/kg/d‐dose, from 3.29 ± 0.09 to 3.32 ± 0.07 for the 10 μg/kg/d‐dose, and from 2.89 ± 0.13 to 2.92 ± 0.11 for the 90 μg/kg/d‐dose. The velocities of the soybean oil groups (with and without astaxanthin) were not statistically different from the 10 μg/kg/d astaxanthin + ethanol and distilled water groups. Conclusion: The results demonstrate the antagonistic effect of astaxanthin against the ethanol‐induced facilitation of CSD propagation. Probably carotenoid antioxidant properties are involved in such effects.     

A multicenter retrospective study defining the clinical and hematological manifestations of brucellosis and pancytopenia in a large series: Hematological malignancies, the unusual cause of pancytopenia in patients with brucellosis

The aim of the study is to review the clinical manifestations and the hematological findings of brucellosis and pancytopenia, with or without hematological malignancies. The records of 202 patients with brucellosis were evaluated retrospectively. Among these cases of brucellosis seen in a 6 year period between April 1999 and June 2005, 30 patients with pancytopenia were identified. The most common manifestation was fever, followed by weight loss, anorexia, malaise, arthralgia, and hepatosplenomegaly. Bone marrow biopsies revealed hypercellularity or normocellularity. The most common findings in the bone marrow evaluation were histiocytic hemophagocytosis and granulomas. Among all cases, we diagnosed 5 hematological malignancies (1 acute myelogenous leukemia, 2 acute lymphoblastic leukemia, and 2 multiple myeloma) concurrently with brucellosis. The clinical symptoms and findings were similar in patients with and without malignancies. In cases with malignancies, the bone marrow biopsy revealed predominant primary disease involvement. Significant increases in ESR and CRP, severe anemia and thrombocytopenia were observed in patients with malignancies. Peripheral blood counts in patients without malignancies returned to normal after antibiotic treatment for brucellosis. However, pancytopenia in two patients with malignancies did not recover because of primary resistant disease. We conclude that while histiocytic hemophagocytosis may be considered as a major cause of pancytopenia, leukemic infiltration can also be an extreme and unusual cause of pancytopenia in patients in whom brucellosis was concurrently diagnosed with hematological malignancies.     

The effect of erythroferrone suppression by transfusion on the erythropoietin–erythroferrone–hepcidin axis in transfusion-dependent thalassaemia: A pre–post cohort study

To track post-transfusion changes on the erythropoietin (EPO)–erythroferrone (ERFE)–hepcidin axis, we collected blood samples from 82 regularly transfused patients with β-thalassaemia major (β-TM) immediately before and 4–6 days after transfusion. The post-transfusion haemoglobin, hepcidin, and ferritin levels were increased, while the EPO, ERFE, and soluble transferrin receptor were suppressed. In addition, hepcidin change was inversely associated with erythropoietic change, which was confirmed by an increase in the hepcidin-to-ERFE ratio after transfusion. Age was the main predictor of serum ERFE, followed by EPO, transfusion frequencies, and ferritin. We found ERFE to be a highly sensitive indicator of erythroid activity in β-TM and that the hepcidin-to-ERFE ratio after transfusion may be used as an appropriateness index of serum hepcidin regulation relative to the degree of erythropoiesis.     

Role of musculoskeletal ultrasonography in the detection of subclinical synovitis in oligo and polyarticular juvenile idiopathic arthritis children

Aim of the work

To study the ability of ultrasound to detect subclinical synovitis in children with oligoarticular and polyarticular juvenile idiopathic arthritis (JIA) and to assess the disease activity according to the clinical, laboratory, and musculoskeletal ultrasonographic (MSUS) evaluation.

Multidisciplinary Treatment of the Metabolic Syndrome Lowers Blood Pressure Variability Independent of Blood Pressure Control

Blood pressure (BP) variability (BPV) contributes to target organ damage independent of BP. The authors examined the effect of a 1‐year multidisciplinary intervention on BPV in patients with the metabolic syndrome (MetS) as defined by criteria from the Third Report of the Adult Treatment Panel. Forty‐four nondiabetic patients underwent clinical and biochemical profiling, 24‐hour ambulatory BP monitoring (ABPM), body composition, carotid intima‐media thickness, and carotid‐femoral pulse wave velocity (PWV). The intervention targeted all MetS components. BPV was assessed by the standard deviation of daytime systolic BP derived from ABPM. Patients with low and high BPV (lower or higher than the median daytime standard deviation of 11.6 mm Hg) did not differ in regards to systolic and diastolic BP, age, fasting glucose, glycated hemoglobin, and body mass index, but the high‐variability group had higher values of low‐density lipoprotein and leg fat. The 1‐year intervention resulted in weight reduction but not BP‐lowering. BPV declined in the high‐variability group in association with lowering of PWV, C‐reactive protein, glycated hemoglobin, alanine aminotransferase, asymmetric dimethylarginine, and increased high‐density lipoprotein cholesterol. A multidisciplinary intervention independent of BP‐lowering normalized BPV, lowered PWV, and enhanced metabolic control.     

Long-term complete haematological and molecular remission after allogeneic bone marrow transplantation in a patient with a stem cell myeloproliferative disorder associated with t(8;13)(p12;q12)

A rare atypical myeloproliferative disorder (aMPD) associated with chromosomal translocations involving the short arm of chromosome 8, region p11-p12 has been described. In most patients, the cytogenetic abnormality is a t(8;13)(p12;q12) that fuses fibroblast growth factor receptor 1, the 8p12 key gene, to FIM/ZNF198 gene. Prognosis is poor with frequent evolution to acute myeloid leukaemia within 1 year of diagnosis. We report a new patient with aMPD with a t(8;13) translocation. Complete haematological, cytogenetic and molecular remission was demonstrated 39 months after allogeneic bone marrow transplantation. This is the first report to demonstrate a molecular remission in this disorder.     

Comparison of outcomes of viscocanalostomy and phacoviscocanalostomy

BACKGROUND: Many adults who undergo glaucoma filtering surgery also need surgical treatment of coexisting cataract. Recent reports of the use of new alternative non-penetrating techniques in combination with phacoemulsification have given encouraging results. The purpose of this study was to compare the safety and efficacy of viscocanalostomy and phacoviscocanalostomy in the management of medically uncontrolled open-angle glaucoma. METHODS: Twenty patients who underwent phacoviscocanalostomy in one eye each were enrolled in the study. Twenty matching patients who underwent viscocanalostomy in one eye each at our institution were selected as the control group. Postoperative reductions in intraocular pressure (IOP) and number of antiglaucoma medications were compared between the groups. Visual acuity and complications were secondary outcomes. RESULTS: Significant reductions of IOP and of use of antiglaucoma medication occurred in both groups (p < 0.05); these results were comparable in the two groups (p > 0.05). The mean IOP reduction was 34% in the eyes that underwent viscocanalostomy alone and 38% in the eyes that underwent phacoviscocanalostomy. The success rates of the two procedures were also comparable (p > 0.05). In the combined-surgery group the best-corrected visual acuity improved by 2 or more lines in 18 eyes (90%). Intraoperative complications did not occur in either group. INTERPRETATION: Combining phacoemulsification with viscocanalostomy did not have a negative effect on the IOP control achieved by viscocanalostomy alone and did not increase the complication rate. Phacoviscocanalostomy was safe and effective in the surgical management of eyes with both glaucoma and cataract.