Could thiazolidinediones increase the risk of heart failure in Friedreich's ataxia patients?

Clinical evidence and the recent decisions of the European Medicines Agency and the Food and Drug Administration challenge the safety of thiazolidinediones treatment. Recently, this treatment has been suggested for Friedreich's ataxia because thiazolidinediones improve neurological symptoms. Hypertrophic cardiomyopathy is the most prevalent cardiac feature and the cause of premature death in Friedreich's ataxia patients. We recommend that therapy with peroxisome proliferator‐activated receptor‐gamma agonists like thiazolidinediones be taken with caution, as they cause a decrease in the number of fast fibers and an increase in mitochondrial biogenesis in cardiac muscle because of the induction of peroxisome proliferator‐activated receptor‐gamma coactivator‐1α. Furthermore, the incidence of heart failure may increase when thiazolidinediones are combined with insulin, and moreover, they produce cyclooxygenase 2 inhibition, inducing a thrombotic response. Thus, patients are predisposed to adverse cardiovascular outcomes. In our opinion, the possible fatal consequences must be taken into account when peroxisome proliferator‐activated receptor‐gamma agonist drugs are considered as possible therapeutic agents for Friedreich's ataxia patients. © 2011 Movement Disorder Society     

Mandibular fractures in sports. Retrospective study of 48 cases

We present a retrospective study of 48 isolated mandibular fractures related to athletic activities. We studied patient age, sex, sport involved, mandibular location of the fracture and the therapeutic implication. The sex ratio was 4/1 and mean age 24 years. Rugby and cross-country biking were the more frequently involved sports (79%). We recall preventive measures. Miniplate osteosynthesis was used as often as possible in order to avoid intermaxillary fixation (IMF) (40%) or to limit the duration of IMF. This allowed early return to sport activities.     

Hypothermia-induced J waves

A 36 years old woman with acquired immunodeficiency syndrome was admitted to the hospital for pulmonary Mycobacterium Avis Complex infection. Seventy-two hours after the admission she became hypothermic and bradycardic. The ECG showed sinus bradycardia, J waves in leads II, III, aVF, aVR, aVL, V5 and V6 along with QT prolongation and T wave abnormalities. After rewarming the J waves and repolarization abnormalities disappeared. The proposed cellular basis of hypothermia-induced J waves is the accentuation of the spike-and-dome morphology of the action potential of M and epicardial cells.     

EBV-positive Hodgkin's lymphoma in children under 10 years

The number of EBV-positive child patients with HL varies in different countries and regions. In developing countries the number of child patients with this disease is higher than in the patient population of economically advanced countries. The authors examined 24 patients under 10 years with Hodgkin's lymphoma. Twenty patients were males, four females. In ten cases according to histological examination type II of HL was involved--nodular sclerosis (7 boys, 3 girls). In 14 instances the authors detected the mixed cellularity type (13 boys and 1 girl). EBV positivity was assessed immunohistologically by means of the antibody against LMP-1 and EBER-1 by the method of in situ hybridization. In 20 EBV positive HL 18 boys and 2 girls were affected. EBV positivity was proved in 83%. The marked intersexual difference in different EBV positive HL has not been explained unequivocally so far. The authors proved, that 14 EBV positive HL were type III--mixed cellularity. In 6 cases the type of nodular sclerosis was involved. The authors assume that marked positivity of child patients is associated with the initial massive EBV infection. EBV positive tumour cells are to a considerable extent removed by the immune apparatus and thus in the second and third decade EBV negative patients predominate.     

Transthyretin familial amyloidotic polyneuropathy: histopathological study of the explanted livers

FAP is an autosomal dominant inherited disease, characterized by systemic deposition of amyloid fibrils in various tissues. The purpose of this study is to describe the gross and microscopic findings of the explanted livers for FAP.10 patients were transplanted for FAP at our institution. Diagnosis was supported by positive familiar history, clinical data and detection of mutated TTR by electrospray ionization mass spectrometry with Val30Met mutation verified by PCR. All the explanted livers were photographed, fixed in formol and processed according to protocol. Later they were examined with HyE, reticulin, PAS diastasa, Masson trichromic, Congo red with polarised light and immunoreactivity against TTR. The gross aspect was normal. We obtained multiple samples representative of the organ and the hepatic hilium. All of the patients presented with deposits of amyloid substance in the lymph nodes and the nerves of the hepatic hilium These deposits were Congo red positive with a greenish birefringence to polarized light Deposits show immunoreactivity with antihuman TTR. Whereas liver transplantation restores hepatic function in patients with cirrhosis, liver transplantation cures the FAP patient of their genetic defect. Domino transplantation is a procedure in which the index patient receives an organ, while the explanted organ is reused for transplantation into another patient. In conclusion, exclusion of hepatic amyloid deposits which can cause functional alterations in the FAP liver is vital; and is important to study the explanted livers of patients with FAP to confirm the results of the scarce published series.     

Clinical trials in diastolic heart failure

Forty percent to 50% of patients with chronic heart failure have normal or relatively normal left ventricular ejections fractions. These are much more frequently women and tend to be older than those with reduced ejection fractions. In most, left ventricular diastolic dysfunction plays a major role in the genesis of heart failure. Until recently, these patients have been systematically excluded from heart failure trials, so that there are no proven treatments for this group. This article reviews the recent and ongoing trials in patients with diastolic heart failure, which hopefully will lead to improved treatment and outcomes.     

Tricuspid cleft or tetracuspid valve? Usefulness of three-dimensional echocardiogram in the assessment of isolated tricuspid regurgitation in pediatrics

Tricuspid regurgitation (TR) in children may be secondary to congenital anomalies of the tricuspid valve complex which is composed by annulus, leaflets, commissures, chordae tendineae, and papillary muscles. The most common congenital cause is Ebstein's anomaly; however, there are less frequent causes such as abnormal number of tricuspid leaflets, tricuspid cleft, leaflet prolapse, double orifice tricuspid valve, and congenital tricuspid valve dysplasia. Identifying the precise cause is important to plan an appropriate repair surgery. In this article, the case of a 4-year-old patient with a tetracuspid valve with significant tricuspid regurgitation is presented and the morphological analysis was made by two-dimensional (2D) and three-dimensional (3D) transthoracic echocardiography. The morphological differences between a tetracuspid valve and a cleft of the anterior leaflet tricuspid valve are exposed. 3D echocardiographic evaluation of the tricuspid valve allowed a better understanding of the tricuspid valve anatomy, which includes evaluation of the tricuspid annulus, leaflets, commissures, and subvalvular apparatus. Recognizing the accurate cause of isolated tricuspid regurgitation allows better planning of the surgical technique.     

Factors associated with clinically significant hypoglycemia in patients with type 1 diabetes using sensor-augmented pump therapy with predictive low-glucose management: A multicentric study on iberoamerica

Background and aimsDespite using sensor-augmented pump therapy (SAPT) with predictive low-glucose management (PLGM), hypoglycemia is still an issue in patients with type 1 Diabetes (T1D). Our aim was to determine factors associated with clinically significant hypoglycemia (<54 mg/dl) in persons with T1D treated with PLGM-SAPT.Methodology: This is a multicentric prospective real-life study performed in Colombia, Chile and Spain. Patients with T1D treated with PLGM-SAPT, using sensor ≥70% of time, were included. Data regarding pump and sensor use patterns and carbohydrate intake from 28 consecutive days were collected. A bivariate and multivariate Poisson regression analysis was carried out, to evaluate the association between the number of events of <54 mg/dl with the clinical variables and patterns of sensor and pump use.Results188 subjects were included (41 ± 13.8 years-old, 23 ± 12 years disease duration, A1c 7.2% ± 0.9). The median of events <54 mg/dl was four events/patient/month (IQR 1–10), 77% of these events occurred during day time. Multivariate analysis showed that the number of events of hypoglycemia were higher in patients with previous severe hypoglycemia (IRR1.38; 95% CI 1.19–1.61; p < 0.001), high glycemic variability defined as Coefficient of Variation (CV%) > 36% (IRR 2.09; 95%CI 1.79–2.45; p < 0.001) and hypoglycemia unawareness. A protector effect was identified for adequate sensor calibration (IRR 0.77; 95%CI 0.66–0.90; p:0.001), and the use of bolus wizard >60% (IRR 0.74; 95%CI 0.58–0.95; p:0.017).ConclusionIn spite of using advanced SAPT, clinically significant hypoglycemia is still a non-negligible risk. Only the identification and intervention of modifiable factors could help to prevent and reduce hypoglycemia in clinical practice.