Ring formation by Vibrio fusion protein composed of FliF and FliG, MS-ring and C-ring component of bacterial flagellar motor in membrane.

The marine bacterium Vibrio alginolyticus has a single flagellum as a locomotory organ at the cell pole, which is rotated by the Na+-motive force to swim in a liquid. The base of the flagella has a motor composed of a stator and rotor, which serves as a power engine to generate torque through the rotor-stator interaction coupled to Na+ influx through the stator channel. The MS-ring, which is embedded in the membrane at the base of the flagella as part of the rotor, is the initial structure required for flagellum assembly. It comprises 34 molecules of the two-transmembrane protein FliF. FliG, FliM, and FliN form a C-ring just below the MS-ring. FliG is an important rotor protein that interacts with the stator PomA and directly contributes to force generation. We previously found that FliG promotes MS-ring formation in E. coli. In the present study, we constructed a fliF-fliG fusion gene, which encodes an approximately 100 kDa protein, and the successful production of this protein effectively formed the MS-ring in E. coli cells. We observed fuzzy structures around the ring using either electron microscopy or high-speed atomic force microscopy (HS-AFM), suggesting that FliM and FliN are necessary for the formation of a stable ring structure. The HS-AFM movies revealed flexible movements at the FliG region.

Possible Paternal Hepatitis B Virus Infection with Different Clinical Courses between Siblings: A Report of Two Cases.

The incidence of hepatitis B virus (HBV) infection is expected to decrease in the future owing to the preventive measures adopted against mother-to-child transmission of HBV and implementation of universal HBV vaccination for children. However, no countermeasure has been established against horizontal infection in nonvaccinated children. We report the case of two siblings who had different clinical courses of possible paternal HBV infection. The younger sibling developed acute hepatitis, whereas the older sibling was an asymptomatic HBV carrier. To eradicate HBV, HBV vaccination of all children and HBV infection screening of fathers and other family members should be encouraged.

Granulomonocytapheresis Using the Single-Needle Method for a Girl with Ulcerative Colitis.

Granulomonocytapheresis (GMA) is an effective treatment for inducing remission in patients with refractory ulcerative colitis (UC). Furthermore, GMA has very few side effects and can be performed without using drugs except anticoagulants. However, GMA is sometimes challenging to perform, especially in children, as it usually requires securing two blood vessels. We attempted GMA by the single-needle method in a girl with UC, which is performed by securing only one blood vessel. In the present case, GMA could be performed 10 times without any side effects. Our case shows that GMA with the single-needle method was feasible in children with UC.

Role of the N- and C-terminal regions of FliF, the MS ring component in Vibrio flagellar basal body.

The MS ring is a part of the flagellar basal body and formed by 34 subunits of FliF, which consists of a large periplasmic region and two transmembrane segments connected to the N- and C-terminal regions facing the cytoplasm. A cytoplasmic protein, FlhF, which determines the position and number of the basal body, supports MS ring formation in the membrane in Vibrio species. In this study, we constructed FliF deletion mutants that lack 30 or 50 residues from the N-terminus (ΔN30 and ΔN50), and 83 (ΔC83) or 110 residues (ΔC110) at the C-terminus. The N-terminal deletions were functional and conferred motility of Vibrio cells, whereas the C-terminal deletions were nonfunctional. The mutants were expressed in Escherichia coli to determine whether an MS ring could still be assembled. When co-expressing ΔN30FliF or ΔN50FliF with FlhF, fewer MS rings were observed than with the expression of wild-type FliF, in the MS ring fraction, suggesting that the N-terminus interacts with FlhF. MS ring formation is probably inefficient without FlhF. The deletion of the C-terminal cytoplasmic region did not affect the ability of FliF to form an MS ring because a similar number of MS rings were observed for ΔC83FliF as with wild-type FliF, although further deletion of the second transmembrane segment (ΔC110FliF) abolished it. These results suggest that the terminal regions of FliF have distinct roles; the N-terminal region for efficient MS ring formation and the C-terminal region for MS ring function. The second transmembrane segment is indispensable for MS ring assembly.ImportanceThe bacterial flagellum is a supramolecular architecture involved in cell motility. At the base of the flagella, a rotary motor that begins to construct an MS ring in the cytoplasmic membrane comprises 34 transmembrane proteins (FliF). Here, we investigated the roles of the N and C terminal regions of FliF, which are MS rings. Unexpectedly, the cytoplasmic regions of FliF are not indispensable for the formation of the MS ring, but the N-terminus appears to assist in ring formation through recruitment of FlhF, which is essential for flagellar formation. The C-terminus is essential for motor formation or function.

Refeeding syndrome in a 12-year-old girl with distal renal tubular acidosis.

Distal renal tubular acidosis is a risk factor for refeeding syndrome. Frequent measurement of serum phosphorus levels at the initiation of nutrition and rapid administration of phosphate preparations are required to prevent organ failure.

Eosinophilic gastroenteritis with persistent abdominal pain: a case report.

Two months ago, a 9-year-old boy experienced intermittent abdominal pain regardless of food intake, without diarrhea, bloody stool, or nausea. Blood test results revealed a peripheral blood eosinophil count of 660 cells /µL, which was marginally elevated, without inflammatory reaction, occult blood, or enteric pathogens. The intestinal mucosa from the terminal ileum to the rectum was endoscopically normal, but mucosal biopsy revealed eosinophilic infiltration of the terminal ileum and whole colon with ≥20 eosinophils/high power field (HPF). Subsequent upper gastrointestinal endoscopy revealed normal endoscopic mucosa from the esophagus to the second part of the duodenum, but mucosal biopsy showed an eosinophil infiltration of ≥20 eosinophils/HPF. Based on the above findings, he was diagnosed with eosinophilic gastroenteritis (EGE). The cause of EGE and mechanisms of eosinophil infiltration have yet to be fully elucidated. For these nonspecific abdominal symptoms, evidence of eosinophilic infiltration of the gastrointestinal mucosa, specifically 20 eosinophils/HPF in each intestinal mucosa, is required for the definitive diagnosis of EGE. Even if only persistent abdominal pain develops, EGE diagnosis should be confirmed with the analysis of mucosal biopsy in addition to the review of allergic disease history and peripheral blood eosinophil counts.

A Case of Nonalcoholic Steatohepatitis in Central Precocious Puberty Aggravated by Gonadotropin-releasing Hormone Analog.

We report the case of a girl with central precocious puberty (CPP) and nonalcoholic steatohepatitis (NASH) aggravated by gonadotropin-releasing hormone analog (GnRHa). She was diagnosed with CCP and began treatment with GnRHa at the age of 8 years and 9 months. She already had mild liver dysfunction and was obese at that time; however, liver dysfunction was aggravated during GnRHa initiation. Her liver dysfunction improved after the discontinuation of GnRHa. Liver biopsy was performed twice during GnRHa initiation and findings suggested NASH. In this case, NASH may have been aggravated by the mechanism of estrogen suppression by GnRHa besides obesity. In conclusion, NASH should be ruled out in obese CPP patients with abnormal liver function before starting GnRHa therapy. CPP patients treated with GnRHa require close examination for the early diagnosis of NASH or its progression.

Rotavirus Vaccine Effectiveness against the Risk of Hospitalization and the Impact of Using Public Funds for the Vaccine on a Regional Rotavirus Gastroenteritis Epidemic in Japan.

An epidemic of rotavirus (RV) gastroenteritis occurred from April to July 2015 across a wide area of Hokkaido, surrounding the Abashiri-Kosei General Hospital. The RV vaccine for children in Shari and Koshimizu was provided at public funds by their local governments, while children in Abashiri were charged for the vaccine. This study examined the effectiveness of the RV vaccine against the risk of hospitalization based on a retrospective cohort study and the impact of using public funds for RV vaccination on a regional RV gastroenteritis epidemic. The vaccination coverage was significantly higher in children in Shari and Koshimizu than in Abashiri (87.8% vs. 42.7%, respectively, p < 0.001). The RV gastroenteritis-related risk of hospitalization was slightly lower in children from Shari and Koshimizu than in those from Abashiri (1.6% vs. 3.2%, respectively, p = 0.07). In addition, the risk of hospitalization in the vaccinated children was significantly lower than that in the unvaccinated children (0.7% vs. 4.8%, respectively, p < 0.001); indicating that the RV vaccine effectiveness against the risk of hospitalization was 96.5% (95% confidence interval 45.7%-99.8%). In conclusion, the use of public funds for the provision of RV vaccine increased the vaccination coverage, which, in combination with high vaccine effectiveness, led to a decrease in the number of hospitalizations in children during a regional RV gastroenteritis epidemic.

Successful treatment of normokalemic periodic paralysis with hydrochlorothiazide.

BACKGROUND: Periodic paralysis (PP) is an autosomal dominant muscle disorder characterized by periodic muscle weakness attacks associated with serum potassium level variations. It is classified into hypokalemic (hypoKPP), hyperkalemic (hyperKPP), and normokalemic (normoKPP) forms based on the ictal serum potassium level. HyperKPP and normoKPP are caused by mutations of the same gene SCN4A, the gene encoding the skeletal muscle voltage-gated sodium channel. Prophylactic treatment with thiazide diuretics is highly effective in preventing attacks in hyperKPP. However, the efficacy and safety of such diuretics in normoKPP remain unclear. CASE: We describe a familial case of normoKPP wherein the affected individuals showed periodic muscle weakness attacks, with an early childhood onset, and a lack of serum potassium level variation during the paralytic attacks. Sequencing analysis of SCN4A gene revealed a heterozygous missense mutation (c. 2111C > T, p. Thr704Met) in all symptomatic family members. Oral administration of hydrochlorothiazide, a thiazide diuretic, markedly improved the paralytic attack frequency and duration in the affected individuals without adverse effects. CONCLUSION: Our case demonstrates the efficacy of hydrochlorothiazide in the prophylactic treatment of normoKPP caused by the SCN4A mutation of p.Thr704Met, the most frequent mutation of hyperKPP.

Pharmacokinetics of drugs for pediatric pulmonary hypertension.

BACKGROUND: Over the past few years, several drugs, each with a different mechanism, have been developed for the treatment of pulmonary hypertension (PH) and are now prescribed in the clinical setting. While the optimal doses of these drugs in adults have been determined, the optimal dose in children, however, is unclear. The aim of this study was therefore, to measure blood drug levels and analyze the pharmacokinetics of two such drugs in children. METHODS: From April 2010 to May 2015, we prospectively enrolled 23 children with PH for treatment with bosentan and/or tadalafil. Twenty children were treated with bosentan and 19 received tadalafil. Sixteen children were given both drugs. Blood samples were collected after 2 weeks of treatment, and blood drug levels measured using high-performance liquid chromatography. RESULTS: For both drugs, the peak plasma concentration was lower and the half-life was shorter than the known values in adults. The blood trough level of bosentan significantly correlated with its dose, but no such correlation was seen for tadalafil. For both drugs, no correlation was observed between age and blood drug levels. CONCLUSIONS: Oral dosing with bosentan and tadalafil in children may not achieve therapeutic blood concentration. Thus, the optimal dosing must be established individually while monitoring blood drug level.

Construction of a scoring system for predicting the risk of severe gastrointestinal involvement in Henoch-Schönlein Purpura.

OBJECTIVE: To evaluate the parameters associated with significant gastrointestinal (GI) involvement in Henoch-Schönlein Purpura (HSP), and construct a scoring system for the identification of patients at high risk of gross blood in stools. STUDY DESIGN: Data for HSP patients hospitalized at each of seven institutes were retrospectively analyzed. Patients were divided into four groups according to the consequent severity of GI involvement. Identification of laboratory parameters at the time of admission were then used to differentiate the groups, and a scoring system to predict gross intestinal bleeding was constructed. Prognostic efficiency, correlation with the subsequent duration of abdominal pain, and association with manifestations excluding abdominal pain were also analyzed. RESULTS: An analysis of variance (ANOVA) test showed significant intergroup differences in white blood cell (WBC) count, neutrophil count, serum albumin, potassium, plasma D-dimer and coagulation factor XIII activity. A scoring system consisting of these parameters showed a good prognostic value for gross intestinal bleeding in a receiver operating characteristic (ROC) analysis, and a cut-off value of 4 points showed a sensitivity of 90.0% and specificity of 80.6%. The score was also correlated with the duration of abdominal pain after admission. A significantly higher score (s) was observed in patients presenting with nephritis, although the predictive value was poor. CONCLUSION: A scoring system consisting of generally available parameters was of use in predicting severe GI involvement in HSP patients. Although further study is needed, initial therapy in accordance with disease activity may be taken into consideration using this scoring system.

A case of pancreatic agenesis and congenital heart defects with a novel GATA6 nonsense mutation: evidence of haploinsufficiency due to nonsense-mediated mRNA decay.

Recently, GATA6 heterozygous loss-of-function mutations were reported to cause pancreatic agenesis and congenital heart defects (PACHD [OMIM:600001]). However, the molecular mechanisms resulting from premature termination codons have not been examined in this disorder. The objective of this study was to perform a genetic analysis of a patient with PACHD. A female patient presented with ventricular septal defect, patent ductus arteriosus, and congenital diaphragmatic hernia at birth. Permanent neonatal diabetes mellitus and pancreatic exocrine deficiency due to pancreatic agenesis was diagnosed at 1 month of age. PCR-direct sequencing of GATA6 revealed that the patient is heterozygous for a novel de novo nonsense mutation of c.1477C>T, p. Arg493X in exon 5. RT-PCR direct sequencing of the RT-PCR products of total RNA from peripheral blood of the patient for the region encompassing exons 4-6 revealed only the wild-type allele. This finding provides the evidence for the occurrence of nonsense-mediated mRNA decay (NMD) in the p.Arg493X mutation. Quantitative RT-PCR analysis revealed that the expression of GATA6 transcript in the patient was less than half compared with normal control samples. This is the first evidence that GATA6 haploinsufficiency is caused by NMD in vivo, and we conclude that GATA6 haploinsufficiency causes not only PACHD but may affect other organs derived from the endoderm. Further screenings of GATA6 mutations in patients with various forms of diabetes and/or congenital heart disease with other visceral malformation may reveal the impact of GATA6 mutations on diabetes and congenital malformation.

Assessment of pulmonary arterial pressure by velocity-encoded cine magnetic resonance imaging in children with congenital heart disease.

BACKGROUND: Velocity-encoded cine magnetic resonance imaging (VEC-MRI) has recently been reported as effective for assessing not only pulmonary blood flow (Qp) but also pulmonary arterial pressure (PAP) in adults. However, there have been few reports on the usefulness of VEC-MRI for assessing PAP in children with congenital heart disease (CHD). METHODS AND RESULTS: We evaluated 34 children with CHD. Qp and systemic blood flows (Qs) were determined by cardiac catheterization and VEC-MRI. The right-to-left Qp ratio (R/L) was measured by pulmonary perfusion scintigraphy and VEC-MRI. The pulmonary-to-systemic blood pressure ratio (Pp/Ps) was determined by cardiac catheterization. The acceleration time (AcT), ejection time (ET), peak velocity (PV), acceleration volume (AcV), and maximal change in flow rate during ejection (MCFR) in the pulmonary arteries, which were standardized by body surface area, were determined by VEC-MRI. The children were divided into 2 groups according to Pp/Ps. The Qs, R/L ratio and Qp/Qs obtained by VEC-MRI strongly correlated with those obtained by catheterization and scintigraphy. No significant differences in AcT, ET, AcT/ET, PV, or AcV were observed between the 2 groups. However, a significant difference was observed in MCFR. Furthermore, a significant correlation was observed between the MCFR and Pp/Ps. CONCLUSIONS: This study clearly demonstrated that VEC-MRI is useful for assessing not only blood flow, but also PAP, by referring to MCFR in children.

The impact of cardiac surgery in patients with trisomy 18 and trisomy 13 in Japan.

Congenital heart defects (CHD) are very common in patients with trisomy 18 (T18) and trisomy 13 (T13). The surgical indication of CHD remains controversial since the natural history of these trisomies is documented to be poor. To investigate the outcome of CHD in patients with T18 and T13, we collected and evaluated clinical data from 134 patients with T18 and 27 patients with T13 through nationwide network of Japanese Society of Pediatric Cardiology and Cardiac Surgery. In patients with T18, 23 (17%) of 134 were alive at this survey. One hundred twenty-six (94%) of 134 patients had CHDs. The most common CHD was ventricular septal defect (VSD, 59%). Sixty-five (52%) of 126 patients with CHD developed pulmonary hypertension (PH). Thirty-two (25%) of 126 patients with CHD underwent cardiac surgery and 18 patients (56%) have survived beyond postoperative period. While palliative surgery was performed in most patients, six cases (19%) underwent intracardiac repair for VSD. Operated patients survived longer than those who did not have surgery (P < 0.01). In patients with T13, 5 (19%) of 27 patients were alive during study period. Twenty-three (85%) of 27 patients had CHD and 13 (57%) of 27 patients had PH. Atrial septal defect was the most common form of CHD (22%). Cardiac surgery was done in 6 (26%) of 23 patients. In this study, approximately a quarter of patients underwent surgery for CHD in both trisomies. Cardiac surgery may improve survival in selected patients with T18.

Volume overload and pressure overload due to left-to-right shunt-induced myocardial injury. - Evaluation using a highly sensitive cardiac Troponin-I assay in children with congenital heart disease-.

BACKGROUND: Cardiac troponin I (cTnI) is currently considered to be the most sensitive and specific biochemical marker of acute coronary syndrome and acute myocardial infarction. However, few reports have described the use of cTnI assays for evaluating abnormal hemodynamic load in children with congenital heart disease (CHD). It was hypothesized that significant hemodynamic overload due to a left-to-right shunt induces myocardial injury. METHODS AND RESULTS: A highly sensitive cTnI assay was used to measure the serum cTnI levels in 30 children with atrial septal defect (ASD), 32 children with ventricular septal defect (VSD), and 350 healthy children. Cardiac catheterization was performed in the children with ASD and VSD to determine the ratio of pulmonary to systemic blood flow, the ratio of pulmonary to systemic arterial pressure (Pp/Ps), the pulmonary vascular resistance index, and the right and left ventricular end-diastolic volume. Serum cTnI levels in both the ASD and VSD children were significantly higher than those in healthy children (P<0.05 and P<0.01, respectively). Furthermore, serum cTnI levels significantly correlated with Pp/Ps (r=0.745, P<0.001) in VSD children. CONCLUSIONS: Significant volume and pressure overload due to a left-to-right shunt induce myocardial injury and might eventually cause irreversible myocardial remodeling in children with CHD. The serum cTnI level is a useful biomarker for evaluating myocardial damage associated with pulmonary hypertension in VSD children.

5.78 Mb terminal deletion of chromosome 15q in a girl, evaluation of NR2F2 as candidate gene for congenital heart defects.

All patients with terminal deletion of chromosome 15q have been reported to show intrauterine growth retardation, postnatal growth retardation, abnormal facial appearance and developmental delay. Haploinsufficiency of IGF1R was considered to be responsible for these symptoms. However, it is difficult to explain other symptoms seen in some of the patients, such as congenital heart defects by the absence of IGF1R alone. Here, we reported a patient with congenital heart defects and a 5.78 Mb terminal deletion of chromosome 15q detected by array-CGH. Among the patients reported to share congenital heart defects and terminal deletion of chromosome 15q, our patient had the smallest deletion. Evaluating the deletion map, NR2F2 was considered a candidate gene contributing to congenital heart defects in patients with terminal deletion of chromosome 15q.

The role of N-terminal pro-B-type natriuretic peptide in the diagnosis of congestive heart failure in children. - Correlation with the heart failure score and comparison with B-type natriuretic peptide -.

BACKGROUND: Both B-type natriuretic peptide (BNP) and N-terminal pro-BNP (NT-proBNP) are useful biomarkers for the assessment of congestive heart failure (CHF) in adults. The purpose of this study was to determine whether BNP and NT-proBNP levels could be used to stratify the severity of CHF in children. METHODS AND RESULTS: The study comprised 181 children with CHF and 232 healthy children aged from 4 months to 14 years who were categorized into CHF grades I, II, III and IV according to the modified Ross scoring system. The plasma BNP and serum NT-proBNP levels were significantly correlated with increasing CHF grades. The NT-proBNP levels were significantly different among the 4 CHF grades. However, only 2 significant differences were observed in the BNP levels between each CHF grade. NT-proBNP testing with cut-off points of >438 pg/ml (> or =grade II), >1,678 pg/ml (> or =grade III) and >7,734 pg/ml (grade IV) in the patients below 3 years of age, and >295 pg/ml (> or =grade II), >1,545 pg/ml (> or =grade III) and >3,617 pg/ml (grade IV) in those above 3 years of age was determined to be highly sensitive and specific by receiver operating characteristic analysis. CONCLUSIONS: The blood levels of BNP and NT-proBNP therefore reflect the severity of CHF in children. In particular, NT-proBNP is a useful biomarker for evaluating CHF in children.

Oxygen-induced tension in the sheep ductus arteriosus: effects of gestation on potassium and calcium channel regulation.

Compared with the full-term ductus arteriosus, the premature ductus is less likely to constrict when exposed to postnatal oxygen concentrations. We used isolated fetal sheep ductus arteriosus (pretreated with inhibitors of prostaglandin and nitric oxide production) to determine whether changes in K+ - and CaL-channel activity could account for the developmental differences in oxygen-induced tension. In the mature ductus, KV-channels appear to be the only K+-channels that oppose ductus tension. Oxygen concentrations between (2% and 15%) inhibit KV-channel activity, which increases the CaL-channel-mediated increase in tension. Low oxygen concentrations have a direct inhibitory effect on CaL-channel activity in the immature ductus; this is not the case in the mature ductus. In the immature ductus, three different K+-channel activities (KV, KCa, and KATP) oppose ductus tension and contribute to its decreased tone. Oxygen inhibits the activities of all three K+ -channels. The inhibitory effects of the three K+-channel activities decline with advancing gestation. The decline in K+ -channel activity is not due to decreased K+ -channel expression. Super-physiologic oxygen concentrations (>or=30% O2) constrict the ductus by using calcium-dependent pathways that are independent of K+- and CaL-channel activities. Super-physiologic oxygen concentrations eliminate the difference in tensions between the two age groups.

Calcium-dependent and calcium-sensitizing pathways in the mature and immature ductus arteriosus.

Studies performed in sheep and baboons have shown that after birth, the normoxic muscle media of ductus arteriosus (DA) becomes profoundly hypoxic as it constricts and undergoes anatomic remodeling. We used isolated fetal lamb DA (pretreated with inhibitors of prostaglandin and nitric oxide production) to determine why the immature DA fails to remain tightly constricted during the hypoxic phase of remodeling. Under normoxic conditions, mature DA constricts to 70% of its maximal active tension (MAT). Half of its normoxic tension is due to Ca(2+) entry through calcium L-channels and store-operated calcium (SOC) channels. The other half is independent of extracellular Ca(2+) and is unaffected by inhibitors of sarcoplasmic reticulum (SR) Ca(2+) release (ryanodine) or reuptake [cyclopiazonic acid (CPA)]. The mature DA relaxes slightly during hypoxia (to 60% MAT) due to decreases in calcium L-channel-mediated Ca(2+) entry. Inhibitors of Rho kinase and tyrosine kinase inhibit both Ca(2+)-dependent and Ca(2+)-independent DA tension. Although Rho kinase activity may increase during gestation, immature DA develop lower tensions than mature DA, primarily because of differences in the way they process Ca(2+). Calcium L-channel expression increases with advancing gestation. Under normoxic conditions, differences in calcium L-channel-mediated Ca(2+) entry account for differences in tension between immature (60% MAT) and mature (70% MAT) DA. Under hypoxic conditions, differences in both calcium L-channel-dependent and calcium L-channel-independent Ca(2+) entry, account for differences in tension between immature (33% MAT) and mature (60% MAT) DA. Stimulation of Ca(2+) entry through reverse-mode Na(+)/Ca(2+) exchange or CPA-induced SOC channel activity constrict the DA and eliminate differences between immature and mature DA during both hypoxia and normoxia.

Impaired cardiac sympathetic nerve function in patients with Kawasaki disease: comparison with myocardial perfusion.

Kawasaki disease (KD) is a leading cause of CAD in children. The impairment of cardiac sympathetic nerve function (CSNF) in the adult patients with coronary artery disease (CAD) could often be seen. However, little is known concerning the impairment of CSNF in KD patients. We investigated CSNF and its relationship with myocardial perfusion in KD patients. Eleven children with KD and 4 controls were studied with 123I-metaiodobenzylguanidine (MIBG) and stressed 201Tl single photon emission computed tomography. By the findings on coronary artery angiography (CAG), the patients were divided into 2 groups: A, without stenosis; B, with significant stenosis and/or old myocardial infarction. CSNF was evaluated from the uptake of 123I-MIBG. While myocardial perfusion was evaluated from 201Tl uptake. The numbers of patients in the groups A and B were 7 and 4. Perfusion defect was found in 0, and 2 patients in group A (0%), and B (50%). 123I-MIBG defects were found in 1 and 4 patients in the group A (14%) and B (100%). There were excellent concordances between the finding of 201Tl and 123I-MIBG in group A. While in group B, the coronary territories with 123I-MIBG defects were significantly more than those with perfusion defects (p < 0.05). In KD patients, the impairment of CSNF might be subsequent to coronary artery stenosis and was more severe than the injury of myocardial perfusion.