The usefulness of speckle tracking echocardiography for the prediction of cardiac involvement in patients with biopsy-proven sarcoidosis.

INTRODUCTION: Cardiac sarcoidosis (CS) is commonly diagnosed based on clinical criteria and abnormalities in noninvasive imaging reported in patients with biopsy-proven extracardiac sarcoidosis. Electrocardiogram and two-dimensional echocardiography have a low sensitivity for CS detection. Cardiovascular magnetic resonance imaging (CMR) and positron emission tomography (PET) have limitations in terms of cost and availability. OBJECTIVES: This study aimed to assess the usefulness of left ventricular longitudinal strain, measured using two-dimensional speckle tracking echocardiography (STE), for the prediction of late gadolinium enhancement (LGE) presence in CMR in patients with biopsy-proven sarcoidosis. PATIENTS AND METHODS: A total of 119 patients with biopsy-proven extracardiac sarcoidosis were divided, according to the clinical criteria proposed by the 2014 Heart Rhythm Society expert consensus statement (HRS 2014), into two groups: 43 individuals with "probable cardiac sarcoidosis", CS(+) and 76 individuals without cardiac sarcoidosis, CS (-). Data from echocardiography, CMR, 12-lead ECG and 24 h Holter monitoring were analyzed. RESULTS: Left ventricular global longitudinal strain (LV-GLS) was slightly reduced in the entire sarcoidosis group (-18.61± 2.96), no difference between the CS (+) and CS (-) subgroups was found (-18.0% ± 3.2% and -18.9% ± 2.8%, respectively; p = .223). No cut-off value for LV-GLS was identified that could predict the presence of LGE. Segmental longitudinal strain impairment partially correlated with the presence of LGE on CMR. CONCLUSIONS: In our cohort of sarcoidosis patients, segmental longitudinal strain proved more helpful in the diagnostic process than LV-GLS. The ultimate role of STE in the diagnosis of CS remains to be established.

Left Ventricular Function and Iron Loading Status in a Tertiary Center Hemochromatosis Cohort-A Cardiac Magnetic Resonance Study.

Background: Haemochromatosis (HCH), a common genetic disorder with variable penetrance, results in progressive but understudied iron overload. We prospectively evaluated organ iron loading and cardiac function in a tertiary center HCH cohort. Methods: 42 HCH patients (47 ± 14 years) and 36 controls underwent laboratory workup and cardiac magnetic resonance (CMR), including T1 and T2* mapping. Results: Myocardial T2* (myoT2*), myocardial T1 (myoT1) and liver T2* (livT2*) were lower in patients compared to controls (33 ± 4 ms vs. 36 ± 3 ms [p = 0.004], 964 ± 33 ms vs. 979 ± 25 ms [p = 0.028] and 21 ± 10 ms vs. 30 ± 5 ms [p < 0.001], respectively). MyoT2* did not reach the threshold of clinically significant iron overload (<20 ms), in any of the patients. In 22 (52.4%) patients, at least one of the tissue parameters was reduced. Reduced myocardial T2* and/or T1 were found in 10 (23.8%) patients, including 4 pts with normal livT2*. LivT2* was reduced in 18 (42.9%) patients. MyoT1 and livT2* inversely correlated with ferritin (rs = −0.351 [p = 0.028] and rs = −0.602 [p < 0.001], respectively). LivT2* by a dedicated sequence and livT2* by cardiac T2* mapping showed good agreement (ICC = 0.876 p < 0.001). Conclusions: In contemporary hemochromatosis, significant myocardial iron overload is rare. Low myocardial T2* and/or T1 values may warrant closer follow-up for accelerated myocardial iron overload even in patients without overt liver overload. Cardiac T2* mapping sequence allows for liver screening at the time of CMR.

Feasibility of Intravoxel Incoherent Motion (IVIM) and Dynamic Contrast-Enhanced Magnetic Resonance Imaging (DCE-MRI) in Differentiation of Benign Parotid Gland Tumors.

Aim: The aim of this prospective study is to identify quantitative intravoxel incoherent motion and dynamic contrast-enhanced magnetic resonance imaging parameters of the most frequent benign parotid tumors, compare their utility and diagnostic accuracy. Methods: The study group consisted of 52 patients with 64 histopathologically confirmed parotid focal lesions. Parametric maps representing apparent diffusion coefficient (ADC), pure diffusion coefficient (D), pseudo-diffusion coefficient (D*), perfusion fraction (FP) and transfer constant (Ktrans), reflux constant (Kep), extra-vascular extra-cellular volume fraction (Ve), and initial area under curve in 60 s (iAUC) have been obtained from multiparametric MRI. Results: Statistically significant (p < 0.001) inter-group differences were found between pleomorphic adenomas (PA) and Warthin tumors (WT) in all tested parameters but iAUC. Receiver operating characteristic curves were constructed to determine the optimal cut-off levels of the most significant parameters allowing differentiation between WT and PA. The Area Under the Curve (AUC) values and thresholds were for ADC: 0.931 and 1.05, D: 0.896 and 0.9, Kep: 0.964 and 1.1 and Ve: 0.939 and 0.299, respectively. Lesions presenting with a combination of ADC, D, and Ve values superior to the cut-off and Kep values inferior to the cut-off are classified as pleomorphic adenomas. Lesions presenting with combination of ADC, D, and Ve values inferior to the cut-off and Kep values superior to the cut-off are classified as Warthin tumors. Conclusions: DWI, IVIM and quantitative analysis of DCE-MRI derived parameters demonstrated distinctive features of PAs and WT and as such they seem feasible in differentiation of benign parotid gland tumors.

The Duration of the correction loss after removing cheneau brace in patients with adolescent idiopathic scoliosis.

OBJECTIVE: The aim of the study was to evaluate the loss of truncal rotation over 54 hours after removing Chêneau brace. METHODS: The studied groups consisted of 39 girls aged 10-18 years old, diagnosed with adolescent idiopathic scoliosis (AIS) and treated with Chêneau brace (CAST) and 20 AIS girls aged 10-18 years old, not treated with bracing. Posterior-anterior radiographs were obtained from the clinical assessment of all subjects and were subsequently used to determine Cobb angles. The measurements of the angle of trunk rotation (ATR) were taken with the Scoliometer® and back-contour device during Adams forward bending test by the two evaluators. The changes in ATRs during 54 hours of observation were performed after the brace had been taken off (0, 2, 24, 30, 48 and 54 hours after debracing). This was described using VATR variable, defined as the change in the absolute Scoliometer® readings in the time intervals against the time interval Δt between the measurements. During back-contour assessment the differential factor (kra) has been used for the digital analysis. The changes in kra over 54 hours of observation were expressed as Vkra factor, defined as the difference in the absolute value of the amplitude differential factor (kra) in the time intervals against the time interval Δt between the measurements. RESULTS: The highest changes were observed in the thoracic as well as in lumbar spine in patients with Cobb angle ≥30°, axial rotation of the apical vertebrae within 5-15°, Risser sign 0-2. The biggest change in the trunk rotation after Chêneau brace had been taken off was noted within the first two hours of observation. CONCLUSION: The patients should be advised to take the brace off for a minimum of two hours before the scheduled x-ray, to allow full relaxation of the trunk in order to obtain reliable radiological images of the deformation. LEVEL OF EVIDENCE: Level III Therapeutic study.

Arthrogryposis multiplex congenital - multidisciplinary care - including own experience.

Arthrogryposis multiplex congenital (AMC) is a heterogeneous disorder, characterized by nonprogressive multiple intra-articular contractures, which can be recognized at birth. The prevalence in Europe is estimated at about 1 per 12,000. Etiopathogenesis of arthrogryposis is multifactorial. Symptoms of some forms of arthrogryposis can be found in the clinical presentation of selected genetic disorders, e.g. Pena Shokeir syndrome. Arthrogryposis can also result from environmental factors such as medication, trauma or chronic illness during pregnancy, as well as from oligohydramnios or abnormal structure of the uterus. In this particular disorder prenatal diagnosis is crucial, because it determines the level of hospital reference during the delivery of the affected child. The highest reference degree hospital is preferential, with staff prepared for the multidisciplinary approach to the treatment of the newborn. The key to success is rehabilitation treatment and it should be initiated as soon as possible. In a substantial number of cases, physical therapy can replace invasive corrective surgery, but even when orthopedic treatment is required, it should always be preceded and followed by rehabilitation.

Fibromyalgia Syndrome - a multidisciplinary approach.

According to American College of Rheumatology fibromyalgia syndrome (FMS) is a common health problem characterized by widespread pain and tenderness. The pain and tenderness, although chronic, present a tendency to fluctuate both in intensity and location around the body. Patients with FMS experience fatigue and often have sleep disorders. It is estimated that FMS affects two to four percent of the general population. It is most common in women, though it can also occur in men. FMS most often first occur in the middle adulthood, but it can start as early as in the teen years or in the old age. The causes of FMS are unclear. Various infectious agents have recently been linked with the development of FMS. Some genes are potentially linked with an increased risk of developing FMS and some other health problems, which are common comorbidities to FMS. It is the genes that determine individual sensitivity and reaction to pain, quality of the antinociceptive system and complex biochemistry of pain sensation. Diagnosis and therapy may be complex and require cooperation of many specialists. Rheumatologists often make the diagnosis and differentiate FMS with other disorders from the rheumatoid group. FMS patients may also require help from the Psychiatric Clinic (Out-Patients Clinic) due to accompanying mental problems. As the pharmacological treatment options are limited and only complex therapy gives relatively good results, the treatment plan should include elements of physical therapy.

Arthrogryposis in infancy, multidisciplinary approach: case report.

BACKGROUND: Arthrogryposis multiplex congenita is an etiopathogenetically heterogeneous disorder characterised by non-progressive multiple intra-articular contractures, which can be recognised at birth. The frequency is estimated at 1 in 3,000 newborns. Etiopathogenesis of arthrogryposis is multifactorial. CASE PRESENTATION: We report first 26 weeks of life of a boy with severe arthrogryposis. Owing to the integrated rehabilitation approach and orthopaedic treatment a visible improvement in the range of motion as well as the functionality of the child was achieved. This article proposes a cooperation of various specialists: paediatrician, orthopaedist, specialist of medical rehabilitation and physiotherapist. CONCLUSIONS: Rehabilitation of a child with arthrogryposis should be early, comprehensive and multidisciplinary. Corrective treatment of knee and hip joints in infants with arthrogryposis should be preceded by the ultrasound control. There are no reports in the literature on the ultrasound imaging techniques which can be used prior to the planned orthopaedic and rehabilitative treatment in infants with arthrogryposis. The experience of our team indicates that such an approach allows to minimise the diagnostic errors and to maintain an effective treatment without the risk of joint destabilisation.