Left Ventricular Noncompaction

According to the World Health Organization classification of cardiomyopathies, left ventricular noncompaction is still an unclassified cardiomyopathy. In 2006, the American Heart Association classified this entity as a primary cardiomyopathy of genetic origin. In 2008, the European Society of Cardiology updated the classification scheme similar to the World Health Organization classification. At present, there is no consensus on the diagnostic criteria, and diagnosis is based on the morphologic features identified by cardiac imaging studies or at autopsy. Due to lack of standardization of the diagnostic criteria and little awareness of this condition among clinicians, the true prevalence of this disease is not clear. There is no specific therapy for this condition. However, it seems prognosis is much better than initially reported. The current status of diagnosis, prognosis, and management of isolated noncompaction in adults is discussed in this review.     

The patellar thinning osteotomy in patellofemoral arthritis: four to 18 years' follow-up

Isolated patellofemoral osteoarthritis can be a disabling disease. When conservative treatment fails, surgical options can be unpredictable and may be considered too aggressive for middle-aged and active people. We analysed the clinical and radiological results of a new coronal osteotomy involving thinning of the patella in a selected group of patients with isolated patellofemoral osteoarthritis. Since 1991, 31 patients (35 knees) have been treated, of whom 34 were available for follow-up at a mean of 9.1 years. The Knee Society Score, the Patellar score and the Short-form-36 questionnaire were used for clinical evaluation. We also examined the radiological features to confirm bone consolidation and assess the progression of osteoarthritis. A significant improvement in the functional scores and radiological parameters was noted. All patients except one were satisfied with the operation. Radiological progression of the patellofemoral osteoarthritis was slowed but radiological femorotibial osteoarthritis progressed in 23 (65%) cases, with a total knee replacement becoming necessary in four cases without technical problems in resurfacing the patella. We compared the results with other forms of surgical treatment reported in the literature. This treatment offers good clinical and radiological results, presenting an alternative method of managing patellofemoral osteoarthritis.     

Prognostic markers in heart failure--congestion, neurohormones, and the cardiorenal syndrome

There are several markers of poor prognosis in heart failure (HF). The most established markers of poor prognosis in HF include neurohormonal (NH) imbalance, low ejection fraction (EF), ventricular arrhythmias, intraventricular conduction delays, low functional capacity, low SBP, and renal failure. The relative importance of these factors is unknown, as they have never been studied together. We present a 74-year-old female with nonischemic cardiomyopathy and an EF<20% who over 24 years since diagnosis, never developed clinical or hemodynamic congestion, was never hospitalized for HF, and never required a loop diuretic. She had all of the clinical indicators of poor prognosis in HF except for severe NH imbalance and renal failure, illustrating their importance in HF prognosis. While NH activation in HF is initially an adaptive mechanism, an imbalance of NH effectors causes congestion leading to a vicious cycle of congestion, renal dysfunction, and worsening of HF. The combination of NH activation and renal failure in HF is a vasomotor nephropathy known as the cardiorenal syndrome (CRS) and portends a poor prognosis. Pharmacological disruption of NH pathways early in HF may prevent CRS and, therefore, improve outcomes.     

Increased frequency of α-synuclein in the substantia nigra in human immunodeficiency virus infection

The frequency of neurodegenerative markers among long surviving human immunodeficiency virus (HIV)-infected individuals is unknown, therefore, the present study investigated the frequency of α-synuclein, β-amyloid, and HIV-associated brain pathology in the brains of older HIV-infected individuals. We examined the substantia nigra of 73 clinically well-characterized HIV-infected individuals aged 50 to 76 years from the National NeuroAIDS Tissue Consortium. We also examined the frontal and temporal cortical regions of a subset of 36 individuals. Neuritic α-synuclein expression was found in 16% (12/73) of the substantia nigra of the HIV+cases and none of the older control cases (0/18). β-Amyloid deposits were prevalent and found in nearly all of the HIV+ cases (35/36). Despite these increases of degenerative pathology, HIV-associated brain pathology was present in only 10% of cases. Among older HIV+adults, HIV-associated brain pathology does not appear elevated; however, the frequency of both α-synuclein and β-amyloid is higher than that found in older healthy persons. The increased prevalence of α-synuclein and β-amyloid in the brains of older HIV-infected individuals may predict an increased risk of developing neurodegenerative disease.     

Camel's milk and gastric emptying

BACKGROUND & AIM: Gastric emptying is determined by food consistency, pH, osmolality, lipid and calorie content as well as the presence of different nutrients in the duodenal lumen. Control of gastric emptying is essential for ensuring optimal digestion. The present study tested the hypothesis that due to its different precipitation properties, gastric emptying of camel's milk may be quicker than that of bovine's milk with the same caloric and fat content. METHODS: Gastric emptying was studied by a scintigraphic technique in a randomized, double blind fashion in 8 volunteers after drinking 450 mL of either cow's milk or camel's milk. RESULTS: No differences in gastric emptying rates were found between the two types of milk. The percentage of retention of the meal after 60 min was 74.6+/-13.2% for cow's milk and 79.8+/-10.8% for camel's milk. The 50% emptying time (T(1/2)) was 131.8+/-37.4 min for cow's milk and 136.8+/-55.8 min for camel's milk. CONCLUSIONS: Camel's milk is most probably not a useful substitute for other types of milk to shorten gastric emptying.     

The relation between c-myc expression and interferon sensitivity in uveal melanoma

BACKGROUND/AIM: Interferons (IFN) are currently being used to treat melanoma, including some patients with uveal melanoma. IFN is thought to inhibit tumour growth through downregulation of the c-myc oncogene; the overexpression of which has been shown to be associated with resistance in cell lines. The aim of this study was to investigate the relation between c-myc gene expression and IFN sensitivity in a series of uveal melanomas in a short term chemosensitivity assay. METHODS: Tumours from 45 patients with uveal melanoma who had undergone enucleation were studied. The ATP chemosensitivity assay was used to study sensitivity to IFN-alpha-2b in freshly isolated cells from each tumour. Flow cytometry was used to assess c-myc expression in formalin fixed material from the primary specimens. RESULTS: There was a wide range of IFN sensitivity between the specimens whereas c-myc expression was universal and present in 80% of the tumour cells in 80% of the specimens. Higher c-myc expression was associated with IFN-alpha resistance as measured by the maximum percentage of inhibition (p = 0.05) and there was a trend with the IFN sensitivity index (p = 0.07). CONCLUSIONS: These results demonstrate that tumours with high c-myc expression are also associated with IFN resistance. Future research is required to explore the potential of c-myc gene manipulation combined with IFN therapy.     

Evidence for altered neuronal organisation within the planum temporale in major psychiatric disorders

Reductions in neuronal size and glial cell density have been described in the frontal cortex in major psychiatric disorders. In this investigation, we performed a cytoarchitectural assessment within the planum temporale (PT), an auditory association region located within the superior temporal gyrus, using two-dimensional (2D) measures of cell size and density and spatial point pattern analysis. In sections of the PT from subjects with schizophrenia, bipolar disorder, major depressive disorder and controls (15 subjects per group), the laminar distribution and size of all neurons and glial cell nuclei was recorded. Spatial point pattern investigation demonstrated reduced neuronal clustering in bipolar disorder (p=0.033) and schizophrenia (p=0.027) compared with controls. Statistical analyses comparing each of the patient groups with the control group failed to identify differences in neuronal density between groups. Neuronal size was reduced in cortical layer 3 (p=0.02) and glial cell density reduced in cortical layer 6 (p=0.05) in bipolar disorder relative to controls but these findings did not remain significant after adjusting for six layer-wise comparisons. We propose that alterations in cortical cytoarchitecture within this region are subtle and involve reduced clustering of neurons, which may be due to altered neuronal organisation within cortical mini-columns or within cortical layers.