We describe a novel, and likely the first, nonpharmacological therapeutic tool that might be able to counteract the muscle atrophy and the declining strength that usually occur in IBM.

The pathogenesis of congenital heart block (CHB) remains unclear. The occurrence rate of neonatal CHB is low, even in murine models of lupus erythematosus. The assessment of heart block in murine maternal lupus models by measuring atrioventricular conduction in neonatal offspring is potentially confounded by fetal wastage. We therefore sought to develop a murine CHB model with a superior immune response and to use embryonic Doppler echocardiography to observe conduction system damage in the fetus. Mature 8-wk-old female C3H/HeJ mice (n ϭ 43) were immunized with 60 kD Ro, 48 kD La, or recombinant calreticulin autoantigens. ELISA confirmed that significant serum autoantibodies developed in all three immunized groups when compared with controls. Starting at 13 d of gestation, a significantly lower fetal heart rate (HR) and a higher percentage of fetal bradycardia/atrioventricular block (AVB, nonadvanced second degree) were observed in all immunized groups, compared with controls. There was 9 -18% nonadvanced second-degree AVB in immunized groups and 0% in controls at Ͻ18 d of gestation. Neonatal electrocardiograms demonstrated only 1°AVB in immunized groups. Maternal immunization with 60 kD Ro, 48 kD La, or recombinant calreticulin autoantigens resulted in AVB in a significant percentage of fetuses, however, lesser degrees of AVB were seen at birth. Significant fetal bradycardia and AVB may be missed by assessment only at birth in murine models of CHB due to fetal wastage. (Pediatr Res 57: 557-562, 2005) Abbreviations AVB, atrioventricular block CHB, congenital heart block HR, heart rate A century has passed since the first description of CHB in the medical literature (1). The disease is defined as the demonstration of the establishment of heart block "in a young patient by graphic records" and "there must be evidence of the existence of the slow pulse at an early age and absence of a history of any infection which might cause the condition after birth" (2). The ECG criteria specify that atrial and ventricular activity are completely dissociated, the ventricular rate is slower, and no captured beats are present. This definition remains as the standard for the diagnosis of CHB.The pathogenesis of CHB remains unclear. The association of the disease with maternal autoantibodies has been recognized since 1977 (3,4). Immune-mediated damage to the fetal conduction system by maternal autoantibodies crossing the placenta in early to mid-gestation has been proposed (5,6) but not confirmed as the mechanism of injury in CHB. Serological studies have demonstrated an association with anti-Ro and anti-La antibodies that is sensitive but not specific for the development of CHB (5,7-10). It has also been suggested that there is an association between anti-calreticulin antibodies and CHB (11).Research into the pathogenesis of CHB has been hindered by its low incidence in infants (1 in 14,000 births) (12) and by the absence of a naturally occurring animal model. Therefore, experimental models have been developed, including neonatal ...

-Context -Gastroparesis is defined by delayed gastric emptying without mechanical obstruction of the gastroduodenal junction, which has been increasingly investigated. Nevertheless, knowledge on the relationships between etiology, symptoms and degree of delayed gastric emptying is limited. Objectives -The demographic, clinical and etiological features of Brazilian patients with gastroparesis were studied and the relationships between these findings and the severity of gastric emptying were determined.Method -This is a retrospective study of medical records of 41 patients with symptoms suggestive of gastroparesis admitted between 1998 and 2011, who had evidence of abnormally delayed gastric emptying on abdominal scintigraphy. Cases with idiopathic gastroparesis were compared with those of patients with neurologic disorders or diabetes mellitus, in whom autonomic neuropathy is likely to occur. Results -The majority of the patients were women (75.6%) with a median age of 41 years and a long-term condition (median: 15 years). Twelve patients (29.3%) had a body mass index of less than 20 kg/m 2 . The most common presenting symptoms were dyspepsia (53.6%), nausea and vomiting (46.3%), weight loss (41.4%) and abdominal pain (24.3%). Regarding etiology, 16 patients had digestive disorders including idiopathic gastroparesis (n = 12), 12 had postoperative conditions, 11 had diseases of the nervous system, five had diabetes mellitus and in three cases gastroparesis was associated to a variety of conditions. In the majority of patients (65.8%) gastric emptying was severely delayed. There was no association between etiology of gastroparesis, type of presenting symptoms and the degree of delay in gastric emptying. Gastroparesis patients with proven (neurological conditions) or presumed (diabetes) nervous system involvements were significantly younger (P = 0.001), had more recent symptom onset (P = 0:03) and a trend towards more severe gastric empty (P = 0:06). There were no significant differences between this subgroup of patients and that comprising cases of idiopathic gastroparesis regarding any of the variables studied. Conclusions -The demographic, clinical and etiological characteristics of Brazilian patients with gastroparesis are quite varied, but there is a predominance of women with long-standing symptoms and marked delay in gastric emptying. The type of presenting symptoms and the degree of delay in gastric emptying do not predict the etiology of gastroparesis. However, severely delayed gastric emptying in younger patients with recent symptom onset should raise the suspicion of impaired neural control of gastro-duodenal motility. HEADINGS -Gastroparesis. Digestive signs and symptoms. Neuropathies. Diabetes mellitus.

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