Simple in vitro migration assay for neural crest cells and the opposite effects of all‐trans‐retinoic acid on cephalic‐ and trunk‐derived cells

Here, we describe a simple in vitro neural crest cell (NCC) migration assay and the effects of all‐trans‐retinoic acid (RA) on NCCs. Neural tubes excised from the rhombencephalic or trunk region of day 10.5 rat embryos were cultured for 48 h to allow emigration and migration of NCCs. Migration of NCCs was measured as the change in the radius (radius ratio) calculated from the circular spread of NCCs between 24 and 48 h of culture. RA was added to the culture medium after 24 h at embryotoxic concentrations determined by rat whole embryo culture. RA (10 μM) reduced the migration of cephalic NCCs, whereas it enhanced the migration of trunk NCCs, indicating that RA has opposite effects on these two types of NCCs.     

Hepatic artery reconstruction preserving the pancreaticoduodenal arcade in pediatric liver transplantation with celiac axis compression syndrome: Report of a case

CACS is rare, although it has been reported to be a potential risk factor for hepatic artery thrombosis following LT. We herein present the case of a 14‐yr‐old male with stenosis of the origin of the celiac trunk. Preoperative CT and color ultrasonography showed narrowing of the proximal celiac artery. The patient underwent DDLT with standard arterial reconstruction without dividing the gastroduodenal artery. His postoperative course was uneventful, with an excellent hepatic artery flow on Doppler ultrasonography. Applying a meticulous preoperative evaluation and the appropriate surgical technique is crucial in patients with CACS.     

Accuracy of telediagnosis of fetal heart disease using ultrasound images transmitted via the Internet

We verified the feasibility of telediagnosis of fetal disease by (i) grading telediagnosis by a pediatric cardiologist into five confidence levels; and (ii) comparison of fetal telediagnosis with hands‐on fetal diagnosis or postnatal diagnosis. In 114 patients suspected of having heart disease (real time, n = 15; recorded image transmission, n = 99), 79 patients were in level 5 (excellent), 17 in level 4 (good), eight in level 3 (fair), 10 in level 2 (poor), and no patients in level 1 (bad). The average was 4.5, and in 96 patients (84% of all) telediagnosis was accurate (above 4), whereas in 18 patients it was inaccurate (level 2 or 3). In re‐examination of 25 patients, telediagnosis was confirmed in patients in level 4 and 5, whereas heart disease was missed in patients in levels 2 or 3. The correct diagnosis matched the high confidence level of a specialist based on recognizable transmitted images.     

Experience of decortication for restrictive hemodynamics in adults with congenital heart disease

We treated four postoperative adults with congenital heart disease with severe restrictive hemodynamics (RH), and performed decortication (DC) with the anticipation of some relief of the RH. The catheterizations before DC showed high central venous, and right and left ventricular end‐diastolic pressures with “dip‐and‐plateau” pressure waveforms in the right and left ventricles. Upon myocardial histopathologic examination, moderate myocardial fibrotic change was demonstrated in two of three cases. DC led to decrease in type B natriuretic peptide levels in all cases, resulting in a decline in the central venous, right and left ventricular end‐diastolic pressures in three cases. Successful DC‐related relief of RH, dilatation of the ventricles with decline in central and end‐diastolic pressures, was observed in only one case. Our limited DC‐related hemodynamic improvement indicates a complexity of the severe RH, which may represent a unique intractable heart failure pathophysiology in intractable postoperative adult congenital heart disease.     

Case of Desbuquois dysplasia type 1: Potentially lethal skeletal dysplasia

We report a boy with Desbuquois dysplasia type 1. He had the typical skeletal changes: a “Swedish key” appearance of the proximal femora; advanced carpal ossification and other distinctive features of the hand, including an extra‐ossification center at the base of the proximal phalanx of the index and middle fingers; dislocation of the metacarpophalangeal joint of the index finger; and bifid distal phalanx of the thumb. In addition, he presented with very severe prenatal growth failure, respiratory distress as a neonate, subsequent failure to thrive and susceptibility to airway infection, and sudden death in early childhood. Molecular analysis identified homozygous 1 bp deletion in the Calcium‐Activated Nucleotidase 1 gene (CANT1). To our knowledge, this is the first report of Desbuquois dysplasia type 1 in Japan. Our experience suggests potential lethality in the disorder.     

DNA-daunorubicin complexes specifically suppress in vitro spontaneous anti-DNA antibody production in lymphocytes of patients with systemic lupus erythematosus

Elevated production of anti-DNA antibody in patients with systemic lupus erythematosus (SLE) is a central problem in the pathogenesis of tissue injury. In the present study, we attempted to manipulate anti-DNA antibody production through the antigen-cytotoxic drug conjugates, DNA-daunorubicin complexes. The effect of DNA-daunorubicin complexes was determined by examining SLE lymphocytes for spontaneous in vitro production of anti-DNA antibody. These complexes, at 2 μg/ml, suppressed anti-DNA antibody production, but not total IgG production, which suggests that specific suppression of anti-DNA antibody production was achieved at this concentration. We believe that the DNA-daunorubicin complexes affected mainly B cells, since such suppression was obtained by treating B cells, as well as B plus T cells. Furthermore, the complexes had no effect on the proliferative responses of SLE T cells to DNA, phytohemagglutinin, or concanavalin A. These results indicate that DNA-daunorubicin complexes may have the potential for selectively suppressing anti-DNA antibody production in patients with SLE.