Isolation and sequence analysis of the complete VP2 gene of canine parvovirus from Chinese domestic pets and determination of the pathogenesis of these circulating strains in beagles.

Canine parvovirus (CPV) causes acute gastroenteritis in domestic dogs, cats, and several wild carnivore species. In this study, the full-length VP2 gene of 36 CPV isolates from dogs and cats infected between 2016 and 2017 in Beijing was sequenced and analyzed. The results showed that, in dogs, the new CPV-2a strain was the predominant variant (n = 18; 50%), followed by the new CPV-2b (n = 6; 16.7%) and CPV-2c (n = 3; 8.3%) strains, whereas, among cats, the predominant strain was still CPV-2 (n = 9; 25%). One new CPV-2a strain, 20170320-BJ-11, and two CPV-2c strains, 20160810-BJ-81 and 20170322-BJ-26, were isolated and used to perform experimental infections. Multiple organs of beagles that died tested PCR positive for CPV, and characteristic histopathological lesions were observed in organs, including the liver, spleen, lungs, kidneys, small intestines, and lymph nodes. Experimental infections showed that the isolates from the epidemic caused high morbidity in beagles, indicating their virulence in animals and suggesting the need to further monitor evolution of CPV in China.

Partial monosomy 3p (3p26.2 --> pter) and partial trisomy 5q (5q34 --> qter) in a girl with coarctation of the aorta, congenital heart defects, short stature, microcephaly and developmental delay.

A 1-year-and-3-month-old girl presented with psychomotor retardation, developmental delay, clinodactyly of the thumb, coarctation of the aorta, patent ductus arteriosus, peripheral pulmonary stenosis, atrial septal defect, microcephaly, brachycephaly, a small oval face, almond-shaped eyes, a down-turned mouth, a widened nasal bridge, hypertelorism, epicanthic folds, long philtrum, low-set large ears and but no craniosynostosis. Oligonucleotide-based array comparative genomic hybridization revealed a -4.79-Mb deletion of 3p26.2 --> pter encompassing CHL1 and CNTN4, and a -19.56-Mb duplication of 5q34 --> qter encompassing MSX2, NKX2-5 and NSD1. The karyotype of the girl was 46,XX,der(3)t(3;5)(p26.2;q34) pat. The present case adds distal 5q duplication to the list of chromosome aberrations associated with coarctation of the aorta.

Phenotypic features of pure 9p deletion in a male infant include cryptorchidism, congenital heart defects and postaxial polydactyly.

We report a 2 1/2-year-old male infant with a karyotype of 46,XY,del(9)(p22) and the phenotypic features of craniofacial dysmorphisms, hypotonia, psychomotor developmental delay, mental retardation, ventricular septal defect, atrial septal defect, cryptorchidism and postaxial polydactyly of the fingers. A rudimentary poorly developed extra digit in the ulnar side of the fifth finger was observed in each hand. The present case adds to the literature of postaxial hexadactyly of the fingers in chromosome 9p deletion syndrome. We suggest that 9pter-p22 may contain genetic loci associated with human postaxial polydactyly.

Fracture of the clavicle does not affect arthritis of the ipsilateral acromioclavicular joint compared with the contralateral side: An osteological study.

In an osteological collection of 3100 specimens, 70 were found with unilateral clavicular fractures which were matched with 70 randomly selected normal specimens. This formed the basis of a study of the incidence of arthritis of the acromioclavicular joint and the effect of clavicular fracture on the development of arthritis in the ipsilateral acromioclavicular joint. This was graded visually on a severity scale of 0 to 3. The incidence of moderate to severe arthritis of the acromioclavicular joint in normal specimens was 77% (100 specimens). In those with a clavicular fracture, 66 of 70 (94%) had arthritis of the acromioclavicular joint, compared to 63 of 70 (90%) on the non-injured contralateral side (p = 0.35). Clavicles with shortening of 15 mm or less had no difference in the incidence of arthritis compared to those with shortening greater than 15 mm (p = 0.25). The location of the fracture had no effect on the development of arthritis.

Regulation of protein expression and function of octn2 in forskolin-induced syncytialization in BeWo Cells.

Placental OCTN2 is a high-affinity carnitine transporter that can interact with a number of therapeutic agents. The process of syncytialization is associated with the expression of a variety of genes. However, the association between syncytialization and OCTN2 expression is not yet clear. Given that forskolin induces BeWo cells to undergo biochemical and morphological differentiation, the purpose of the present study was to investigate whether the function and expression of OCTN2 are influenced by forskolin treatment during syncytialization. The forskolin-induced differentiation of BeWo cells was validated by secretion of beta-human chorionic gonadotropin (beta-hCG) and syncytin expression. Cellular localization of OCTN2 was analyzed by confocal microscopy. Expression of OCTN2 and the modular proteins PDZK1, PDZK2, NHERF1 and NHERF2 was analyzed by Western blotting and carnitine uptake by BeWo cells was estimated and the kinetic properties of uptake measured. The results showed that forskolin treatment increased beta-hCG secretion and syncytin expression, suggesting induction of syncytialization. Confocal images of BeWo cells showed the localization of OCTN2 in the brush-border membrane. OCTN2 protein expression was upregulated in isolated brush-border membranes by long-term forskolin treatment, but the V(m) for carnitine uptake was unchanged, although the K(m) increased. PDZK1, NHERF1 and NHERF2 protein expression in the brush-border membrane was downregulated by forskolin treatment, whereas PDZK2 levels remained unchanged. In conclusion, protein expression and function of OCTN2 in BeWo cells can be regulated by forskolin treatment. While the presence of forskolin results in an increase in OCTN2 protein expression, the increase in uptake capacity may be compensated by the decreased expression of PDZK1, NHERF1 or NHERF2.

24 Mb deletion of 6q22.1-->q23.2 in an infant with pulmonary atresia, ventricular septal defect, microcephaly, developmental delay and facial dysmorphism.

24 Mb deletion of 6q22.1-->q23.2 in an infant with pulmonary atresia, ventricular septal defect, microcephaly, developmental delay and facial dysmorphism.

Mucopolysaccharidosis I under enzyme replacement therapy with laronidase--a mortality case with autopsy report.

There is little information about MPS I-related complications during laronidase therapy. We describe the first autopsy report of a young male MPS I patient who died of infection-induced cardiopulmonary failure following 2 years of weekly treatment with laronidase.

Modified arterial switch operation by sharing the common wall between the great arteries.

BACKGROUND: Suprapulmonary stenosis and coronary arterial obstruction still remain as problems after an arterial switch operation (ASO). We used a modified ASO applying the common wall and in situ transfer techniques to improve the current procedure. METHODS: From October 1996 to December 1997, 11 babies aged 6 days to 3 months with transposition of the great arteries underwent a modified ASO which included sharing the common wall between the great arteries until above the anterior neoaortic suture-line for coronary and pulmonary artery reconstruction. Coronary arteries were of usual type in three cases, juxtacommissural origin in five, and a high takeoff in one; all were redirected almost in situ. RESULTS: There was no early death (< 30 d), coronary or bleeding problems. One late death occurred after a repeat surgery for suprapulmonary stenosis. This was caused by upward stretching of the left pulmonary artery, which was placed above the high left-sided neoaortic anastomosis for in situ transfer of the high takeoff coronary arteries. Intraluminal growth of the adventitia also contributed to suprapulmonary stenosis, which decreased significantly when the common wall adventitia was cleaned in the last two cases we operated on. Ten patients were doing well at follow-up (30.9 +/- 5.2 mo). CONCLUSIONS: This modified ASO by common wall and in situ transfer might avoid coronary kinking and lessen the chance of postoperative bleeding. To avoid suprapulmonary stenosis, common wall adventitia inside the pulmonary pathway should be cleaned, and the left and right pulmonary arteries should also be kept in situ as possible as in coronary redirection.

Valveless outflow reconstruction using autologous tissue as a posterior wall for pulmonary atresia with ventricular septal defect.

BACKGROUND AND PURPOSE: Reoperation is inevitable for some patients with pulmonary atresia who receive a heterograft or homograft in a primary Rastelli operation. Nonetheless, the need for reoperation in patients with classic Fallot's tetralogy who have undergone total correction with a transannular patch is unusual. We sought to change pulmonary atresia into Fallot's tetralogy and used a transannular patch instead of the conventional Rastelli operation. PATIENTS AND METHODS: Valveless outflow direct reconstruction was performed on 10 consecutive patients with pulmonary atresia and ventricular septal defect between August 1997 and 1999. Patient ages ranged from 1.3 to 11.5 years. A Blalock-Taussig shunt was previously constructed in four of these patients and a central shunt was constructed in five. The major aortopulmonary collateral arteries were occluded in one patient by repeated coil embolization after the central shunt. The strategy was to connect the right ventriculotomy with the pulmonary arteries directly, even if there was a gap with a long atretic cord. In patients with a previous central shunt covered with a Gore-Tex membrane, the reactive visceral pericardium over the in situ tissue (the left atrium, right ventricle, or aorta) was used as the autologous posterior wall. Thus, only autologous, fresh pericardium without a valve was used to cover the anterior part of the right ventricular outflow tract, as in the repair of classic Fallot's tetralogy with a transannular patch. RESULTS: There was no mortality, and the postoperative central venous pressure was low in all patients. No gradient was noted across the right ventricular outflow tract. Follow-up echocardiography revealed a competent tricuspid valve with mild pulmonary regurgitation in all patients. CONCLUSIONS: The results of this study suggest that valveless outflow direct reconstruction provides adequate pulmonary circulation without hypertension in pulmonary atresia patients with a ventricular septal defect if the tricuspid valve is competent.

Intestinal pseudo-obstruction followed by major clinical features of Kawasaki disease: report of one case.

Kawasaki Disease (KD) or atypical KD (AKD) rarely presents with intestinal obstruction or pseudo-obstruction. Others have reported gastrointestinal symptoms appearing with and up to 4 weeks after the occurrence of major clinical symptoms of KD. However, we presented a 1-year-old boy with prolonged fever who was found to have pyuria and liver dysfunction on the fourth day of fever. He developed a picture of intestinal pseudo-obstruction including bilious vomiting and abdominal distention on his fifth day of fever, four days before he developed the fissured lips. Because of the emergence of four major clinical criteria of KD, coronary artery dilatation, and aseptic meningitis, AKD was initially diagnosed and prompted the use of intravenous immunoglobulin on the tenth day of fever. Hydrops of the gallbladder, leukocytosis, increased ESR, and thrombocytosis were noted during hospitalization. These atypical features rarely develop all together in one patient with KD or AKD. Nevertheless, our patient had early intestinal pseudo-obstruction with almost all of the above unusual systemic manifestations and the late occurrence of clinical features of KD. We demonstrate that intestinal pseudo-obstruction in KD may develop earlier than other major clinical features and may improve under the conservative treatment. Surgery should be reserved for those who have complete intestinal obstruction presenting with significant peritoneal signs.

Metabolic disturbance in obese children: glucose, insulin levels and lipid profile.

Obesity is a common nutritional disorder among children and adolescents. The purpose of this study was to investigate the biochemical changes in obese children in Taiwan, in comparison with those observed by other authors. Children with weights above 120 percent of ideal body weight were considered obese. Serum glucose, insulin, uric acid, and creatinine levels and lipid profiles of 298 obese children (mean age 11.3 +/- 2.4 years, 158 boys) and 60 controls (mean age 11.6 +/- 2.2 years, 38 boys) were determined using accepted procedures. Demographic and serum biochemical characteristics were compared between obese and control groups overall and by gender. Serum glucose, insulin, cholesterol, and triglyceride levels, known risk factors for coronary heart disease, were higher in the obese patients than in the control group. Early treatment and prevention of childhood obesity may keep the metabolic disturbance from deteriorating and becoming risk factors for coronary heart disease.

Floating the raft hypothesis: the roles of lipid rafts in B cell antigen receptor function.

The initiation of antibody responses to foreign antigens requires that B cells receive and integrate a variety of signals through an array of cell surface receptors including the B cell antigen receptor (BCR) as well as a number of essential coreceptors. Recent evidence indicates that cholesterol-rich plasma membrane microdomains, referred to here as lipid rafts, serve as platforms for BCR signaling and trafficking in B cells. The existence of rafts suggests a previously unappreciated level of organization at the B cell surface that may explain, at least in part, how BCR signaling is coordinated. Here the current evidence that lipid rafts play a key role in B cell responses is reviewed.

Use of bacterially expressed EBNA-1 protein cloned from a nasopharyngeal carcinoma (NPC) biopsy as a screening test for NPC patients.

EBV serological tests have been used for many years as accessory diagnostic predictors of nasopharyngeal carcinoma (NPC). To increase the sensitivity and specificity of the NPC detection rate, a novel enzyme-linked immunosorbent assay (ELISA) was established using a bacterially-expressed GST-EBNA-1 protein, containing the EBNA-1 sequence cloned from an NPC patient. Serum samples were collected from age- and gender-matched patients with NPC, community control subjects and hospital control patients and tested using this ELISA. The positivity rates were 78.7% (247/314) in NPC, 11.5% (28/244) in hospital controls and 3.8% (10/263) in the community control group. These serum samples were also tested for IgA anti-VCA antibodies and their ability to neutralize EBV DNase and the sensitivities of the anti-VCA antibody and DNase-neutralization tests also were analyzed. The optimum combination is VCA plus EBNA-1, which can identify 92.5% (287/310) of NPC patients, and shows a specificity of 92.7% (242/261) for normal individuals.

Combined atrial and arterial switch operations for congenitally corrected transposition.

Conventional repair of congenitally corrected transposition of the great arteries (CCTGA) is directed at eliminating the associated defects and leaves the right ventricle in a systemic position. The long-term outcome of this procedure may involve deterioration of right ventricular function with tricuspid regurgitation and failure of the conduction system. We describe two consecutive patients with CCTGA, one of whom had apicocaval juxtaposition. The patients were aged 19 and 16 months, respectively, and both underwent a combination of atrial and arterial switch. These are the first two reported cases of successful completion of this type of operation in Taiwan. Our review of previously reported cases suggested that no significant difference exists in the outcome of patients with this condition who undergo either arterial switch or Rastelli-type repair plus atrial redirection. However, reported patients who underwent anatomic repair had lower early mortality, late mortality, and incidence of complete heart block than those who underwent conventional repair. The present two cases and our review of the literature suggest that, among patients with apicocaval juxtaposition, 1) Mustard operation is optimal for patients with small atrial volume; 2) one-and-one-half ventricular repair may be helpful to the outcome, especially when treatment is combined with Rastelli-type repair; and 3) excellent access to the ventricular septal defect through the tricuspid valve is afforded via a left atriotomy. From the present two cases and our review of the literature, we conclude that anatomic repair is superior to conventional repair of CCTGA in terms of protection against dysfunction and failure of the anatomic right ventricle, tricuspid valve, and conduction system. Long-term follow-up is mandatory.

Comparative study of timolol gel versus timolol solution for patients with glaucoma.

BACKGROUND: The timolol gel was developed for the simplification of multi-dose medications for glaucoma patients. This gel can be used once per day instead of twice per day of the aqueous form. It is necessary to understand the clinical effects of intraocular pressure maintained in glaucoma by there two forms of timolol. METHODS: Fifty-two eligible patients were included and completed the 12-week study. In this randomized, open-label, two-period crossover study design, patients received either the gel form of 0.5% timolol once daily or the aqueous form of 0.5% timolol twice daily for the first 6 weeks, then crossed over to the other treatment form for the remaining 6 weeks. Intraocular pressure was measured at baseline, week 6 and week 12 at the end of the treatment period. The incidences of all the adverse events were recorded and summarized in each follow-up visit. RESULTS: No statistically significant difference in intraocular pressure was observed between the two different control treatment groups (p>0.05). However, stickiness and transient blurred vision was reported more often by patients using the gel form than the aqueous form of timolol. CONCLUSIONS: The gel form of 0.5% timolol used once daily offers a new alternative that is probably more convenient than the aqueous form of 0.5% timolol given twice daily in maintaining the intraocular pressure of patients with glaucoma.

Complete myocardial revascularization using only pedicled arterial conduits in Kawasaki disease.

A 16-year-old boy with Kawasaki disease suffered from progressive angina and exercise intolerance for 1 year. Coronary angiography showed 60% stenosis of the left main coronary artery, a calcified aneurysm with total occlusion at the proximal left anterior descending artery (LAD), and another aneurysm with total occlusion at the middle portion of the right coronary artery. Aortocoronary bypass was done with the left internal mammary artery (IMA) anastomosed to the first obtuse marginal branch, the right IMA to the distal LAD, and the right gastroepiploic artery to the posterior descending artery. Graft patency was documented by follow-up coronary angiography 1 month after surgery. During follow-up, his rapid improvement led to an upgrade from New York Heart Association functional class III to class I. The results of the present case suggest that complete myocardial revascularization using arterial conduits in patients with Kawasaki disease with coronary artery occlusion is safe and effective.

Midterm results of aortic valve reconstruction for congenital aortic stenosis with or without regurgitation.

Aortic valve is often replaced if valvular stenosis fails to be balloon dilated. Aortic valve reconstruction was performed on 4 patients from August 1993 to 1999. Their ages ranged from 1 month to 15 years (mean 8.3 years). Unicuspid aortic valve was present in three of them and bicuspid in the other one. Two patients were associated with a patent arterial duct, one aortic regurgitation, and one pulmonary stenosis. Commissurotomy was done in three of them to transform the aortic valve into tricuspid except one, in whom bicuspid valve was preserved. In one case with unicuspid aortic valve, a piece of tanned autologous pericardium was used to augment one myxomatous and retracted leaflet. The sinus of Valsalva was molded together with a bulging shape of its aortic leaflet. All four were weaned from cardiopulmonary bypass smoothly. Transesophageal echocardiography in one case prompted rebypass to decrease the degree of regurgitation from moderate to mild by further shaping of the leaflet and sinus of Valsalva. In one patient chylopericardium was complicated and subsided in 5 days after conservative treatment. All patients were doing well on follow up at 56.8 +/- 34.4 months after surgery, with trivial to mild systolic pressure gradient (20 +/- 26 mmHg; preoperatively: 88 +/- 36 mmHg) and mild regurgitation. Aortic valve reconstruction is feasible in the setting of congenital aortic stenosis in our limited experience; repair instead of replacement is recommended even when regurgitation is present.

Polymorphism of transmembrane region of MICA gene and Kawasaki disease.

Kawasaki disease is a febrile disease of children complicated with vasculitis of the coronary arteries and potential aneurysm formation. It has been recognized worldwide and appears to be increasing in frequency. Studies have found that Kawasaki disease is associated with major histocompatibility complex (MHC) class I B antigens. The MHC-class-I-chain-related gene A (MICA) is located near HLA-B. It has a triplet repeat microsatellite polymorphism in the transmembrane region. We investigated the microsatellite polymorphism in children with Kawasaki disease and controls. Seventy children (46 boys), age at diagnosis 1.68 +/- 1.69 years, with Kawasaki who were treated with aspirin as well as intravenous gamma-globulin were enrolled. Control subjects consisted of 154 children (87 boys), age 2.81 +/- 2.12 years. Phenotype frequency of allele A4 in patients with aneurysm formation was significantly lower than in patients without aneurysms [relative risk (RR) = 0.06, 95% confidence interval (CI) = 0.01-0.48, p = 0.00469, pc = 0.0232] and showed a similar tendency when compared with controls. Gene frequency of allele A4 was also significantly lower in patients who developed aneurysms than in patients who did not (RR = 0.07, 95% CI = 0.01-0.57, p = 0.0057, pc = 0.0282). Gene frequency of allele A5 showed a tendency to be higher in patients who developed aneurysms than in controls (RR = 2.35, 95% CI = 0.98-5.63, p = 0.0486, pc = 0. 220). Allele A5.1 tended to be negatively associated with Kawasaki disease (RR = 0.57, 95% CI = 0.35-0.93, p = 0.022, pc = 0.105). Our study showed that allele A4 was negatively associated with coronary aneurysm formation in Kawasaki disease. This suggests that allele A4 protects the children with Kawasaki disease from developing coronary aneurysms after aspirin and gamma globulin therapy.

Modified arterial switch operation by spiral reconstruction of the great arteries in transposition.

BACKGROUND: Spiral relationship of the normally related great arteries (SRGA) has never been reconstructed in an arterial switch operation. METHODS: From March 1998 to April 1999, 9 consecutive cases of transposition of the great arteries (TGA) family (from 2 days to 1.6 years old) underwent arterial switch operations with SRGA at our hospital. Two had a congenitally corrected TGA (plus atrial redirection). Lecompte maneuver was not used in all. The posterior wall of pulmonary trunk was not divided but three were reattached, two of whom had had previous pulmonary trunk banding. Thus the wall was shared between the great arteries facing each other. RESULTS: All survived the operation. Supraaortic stenosis was balloon-dilated in 2 cases of early series, but technical modifications later were able to avoid it. Angiogram showed smooth flow into SRGA without upward and anterior tilting of the pulmonary bifurcation. All great and coronary arteries were patent. All were doing well on follow-up (16.5 +/- 4.2 months). CONCLUSIONS: We concluded that the techniques to relocate the coronary arteries using common wall and in situ switch could also be applied to pulmonary arterial reconstruction, so that SRGA can be resumed in TGA.

Occlusion of azygos vein via direct percutaneous puncture of innominate vein following cavopulmonary anastomosis.

A 2-year-10-month-old boy was diagnosed with a complex congenital heart disease: right atrial isomerism, left superior vena cava (LSVC), complete atrioventricular septal defect, secundum type atrial septal defect, transposition of the great arteries with pulmonary atresia, patent ductus arteriosus, absence of a right superior vena cava (RSVC), and dextrocardia. He had received a left Blalock-Taussig (BT) shunt at the age of 3 months and a left bidirectional Glenn shunt one year after BT shunt. Progressive cyanosis was noted after the second operation and cardiac catheterization showed a functional Glenn shunt with an engorged azygos vein, which was inadvertently skipped for ligation. Because of the absence of RSVC, transcatheter occlusion of the azygos vein was performed successfully via direct puncture of the innominate vein.