PURPOSE: Children with mitochondrial disease (MD) have clinical phenotypes that are more severe than those found in adults. In this study, we assessed cardiac function in children with MD using conventional and advanced echocardiographic measurements, explored any unique patterns present, and investigated the development of early cardiomyopathy (CMP). MATERIALS AND METHODS: We retrospectively reviewed the medical records of 33 children with MD. All patients underwent transthoracic echocardiography with conventional and advanced myocardial analysis. We compared all data between patients and an age-matched healthy control group. RESULTS: Conventional echocardiographic diastolic measurements of mitral E, E/A, and tissue Doppler E′ were significantly lower and E/E′ was significantly higher in children with MD, compared with the measurements from the control group. There was no significant difference in longitudinal and radial strain between the groups. Circumferential strain in the endocardium (p=0.161), middle myocardium (p=0.008), and epicardium (p=0.042) were lower in patients, compared to the values in controls. Circumferential strain was correlated with E′ (p < 0.01, r>0.60). CONCLUSION: In children with MD, myocardial circumferential strain may develop early in all three layers, even with normally preserved longitudinal and radial strain. This may be an early diagnostic indicator with which to predict CMP in this patient population.
Biomechanical Phenomena ; Child ; Echocardiography, Doppler ; Female ; Humans ; Male ; Mitochondrial Diseases/diagnostic imaging ; Mitochondrial Diseases/pathology ; Mitochondrial Diseases/physiopathology ; Myocardium/pathology

It is difficult to take intraoral radiographs in some patients who are intolerable to place the film in their mouth. For these patients, Newman and Friedman recommended a new technique of extraoral film placement. Here we report various cases that diagnostic imaging was performed in patients using the extraoral periapical technique. This technique was used to obtain the radiographs for the patients with severe gag reflex, pediatric dental patients, and patients with restricted mouth opening. This technique can be recommended as an alternative to conventional intraoral periapical technique in cases where intraoral film placement is difficult to achieve.
Diagnostic Imaging ; Humans ; Hypogonadism ; Mitochondrial Diseases ; Mouth ; Ophthalmoplegia ; Radiography, Dental ; Reflex ; Technology, Radiologic