Traumatic Ventricular Septal Defect in a 1-Year-Old Boy After Accidental Chest Compression by an Adult.

We report a 1-year-old boy with a delayed diagnosis of traumatic ventricular septal defect (VSD) related to chest compression. His cardiac function was stable after adequate medical treatment. Spontaneous closure of traumatic VSD occurred to this patient at the age of 4 years. This is a rare case of traumatic VSD associated with accidental chest compression, which is similar to rupture of the ventricular septum after blunt chest trauma. It should be kept in mind that traumatic VSD and concomitant thoracic injuries can develop during chest compression. The clinician should pay attention to the potential risk of traumatic VSD in patients experiencing chest compression. Echocardiography is a convenient and effective tool for serial follow-up examination and avoiding the delayed diagnosis. Troponin I level can be a useful screening test. Conservative management of traumatic VSD with hemodynamic stability is recommended because of possible spontaneous closure.

Echocardiographic Diagnosis of Incidentally Found Left Coronary Artery to Pulmonary Artery Fistula in an 11-Year-Old Girl.

We report on a healthy 11-year-old girl who presented to our facility with sudden onset of fainting in a strenuous running course. Transthoracic echocardiography at short-axis view showed a diastolic flow into the main pulmonary artery (PA). The diagnosis of left anterior descending artery (LAD) to PA fistula was documented by cardiac computed tomography and catheterization. Interventional therapy of LAD to the main PA fistula was not performed because of no evidence of myocardial ischemia or significant hemodynamic change. Presently, the patient remains asymptomatic. Coronary fistula with an incidence of about 0.1-0.8% is very rare and may be undetected, particularly in pediatric patients without cardiac murmur. We herein describe the diagnostic approach and discuss the current treatment modalities.

Amsacrine suppresses matrix metalloproteinase-2 (MMP-2)/MMP-9 expression in human leukemia cells.

This study explores the suppression mechanism of amsacrine (4-(9-Acridinylamino)-N-(methanesulfonyl)-m-anisidine hydrochloride) on matrix metalloproteinase-2 (MMP-2) and MMP-9 expression in human leukemia cells. Amsacrine attenuated cell invasion with decreased MMP-2/MMP-9 protein expression and mRNA levels in U937, Jurkat, HL-60, K562, KU812, and MEG-01 cells. Moreover, amsacrine reduced both MMP-2/MMP-9 promoter luciferase activity and MMP-2/MMP-9 mRNA stability in leukemia cells. Studies on amsacrine-treated U937 cells revealed that amsacrine-elicited ROS generation induced JNK and p38 MAPK activation but reduced the phospho-ERK level. Amsacrine-induced ERK inactivation and p38 MAPK/JNK activation were demonstrated to suppress MMP-2/MMP-9 promoter luciferase activity and promote MMP-2/MMP-9 mRNA decay, respectively. p38 MAPK/JNK activation led to up-regulation of protein phosphatase 2A catalytic subunit α (PP2Acα) in amsacrine-treated U937 cells. Okadaic acid (PP2A inhibitor) treatment increased MMP-2/MMP-9 mRNA stability in amsacrine-treated cells, whereas PP2Acα over-expression increased MMP-2/MMP-9 mRNA decay. Amsacrine-induced MMP-2/MMP-9 down-regulation was also related to PP2Acα up-regulation on Jurkat, HL-60, K562, KU812, and MEG-01 cells. Collectively, our data indicate that amsacrine induces MMP-2/MMP-9 down-regulation via simultaneous suppression of genetic transcription and mRNA stability in human leukemia cells.

PRKAG3 polymorphisms associated with sporadic Wolff-Parkinson-White syndrome among a Taiwanese population.

BACKGROUND: The aim of this study was to investigate whether mutation in AMP-activated protein kinase (AMPK) subunit genes (PRKAG3-230) is associated with sporadic, isolated Wolff-Parkinson-White (WPW) syndrome. METHODS: This study consisted of 87 patients with symptomatic WPW syndrome and 93 healthy controls. PRKAG3-230 genotypes were determined using real-time polymerase chain reaction assay. Genotype and allele frequencies of PRKAG3-230 between patients with WPW syndrome and healthy controls were ascertained using chi-square test or Fisher exact test when appropriate. RESULTS: PRKAG3-230 were genotyped in 87 patients (53 men and 34 women; age=24.4±18.0 years) with WPW syndrome and 93 healthy controls (57 men and 36 women; age=16.8±4.2 years). There were no significant differences between the two groups in terms of age and sex. The patients with CG and CG+CC genotypes had a significantly increased risk of WPW syndrome compared with those with GG genotype [odds ratio (OR)=1.99, 95% confidence interval (CI)=1.01-3.89, p=0.045; OR=1.99, 95% CI=1.04-3.78, p=0.037, respectively]. The allelic types were not associated with the risk of WPW syndrome. The patients with manifest type with CG and CG+CC genotypes had a significantly increased risk of WPW syndrome compared with those with GG genotype (OR=2.86, 95% CI=1.16-7.05, p=0.022; OR=2.84, 95% CI=1.19-6.80, p=0.019, respectively). The patients with right-side accessory pathways with CG and CG+CC genotypes had a significantly increased risk of WPW syndrome compared with those with GG genotype (OR=3.07, 95% CI=1.25-7.51, p=0.014; OR=2.84, 95% CI=1.19-6.80, p=0.019, respectively). The allelic types were not associated with the risk of WPW types and locations. CONCLUSION: This study shows that PRKAG3-230 may be associated with sporadic WPW syndrome among a Taiwanese population. Further studies are warranted to elucidate the role of mutations in AMPK subunit genes other than PRKAG3-230 in sporadic WPW syndrome.

Myeloperoxidase genetic polymorphisms and susceptibility to Kawasaki disease in Taiwanese children.

BACKGROUND/PURPOSE: The aim of this study was to investigate the myeloperoxidase (MPO) -463G>A polymorphism in Kawasaki disease (KD) patients, and the relationship between gene polymorphism and MPO levels. METHODS: A total of 334 KD children and 492 sex-matched controls were assayed for polymorphism analysis. TaqMan assays were used for genotyping. MPO was measured in 37 KD patients and 42 febrile controls. RESULTS: A significant linear trend of KD risk was found to be related to the G/G genotype (plinear trend = 0.032). The combined genotypes (G/A and A/A) of MPO -463G>A were associated with a significantly decreased KD risk compared to the G/G genotype [adjusted odds ratios (AOR) = 0.71, 95% confidence interval (CI): 0.52-0.99, p = 0.040]. In addition, KD patients with A allele were associated with a significantly decreased KD risk as compared to those with G allele (AOR = 0.73, 95% CI: 0.54-0.98, p = 0.033). MPO levels were significantly elevated in KD patients in preintravenous immunoglobulin (pre-IVIG) stage compared to febrile controls (p = 0.002). KD patients in pre-IVIG stage had significantly higher MPO levels than febrile controls in terms of G/G genotype (p = 0.003) and G allele (p < 0.001). KD patients with A allele had significantly lower MPO levels than those with G allele in post-IVIG acute stage (p = 0.042). However, there was no significant difference of individual MPO change for KD patients from pre- to post-IVIG stage in terms of genotypes (p = 0.837) or alleles (p = 0.631). CONCLUSION: Our results suggest that G allele of MPO -463G>A polymorphism is a potential genetic marker for KD risk in Taiwanese children.

Comparison of acute lobar nephronia and acute pyelonephritis in children: a single-center clinical analysis in southern taiwan.

BACKGROUND: Patients with acute lobar nephronia (ALN) require a longer duration of antimicrobial treatment than those with acute pyelonephritis (APN), and ALN is associated with renal scarring. The aim of this study was to provide an understanding of ALN by comparing the clinical features of pediatric patients with ALN and APN. METHODS: We enrolled all of the patients with ALN (confirmed by computed tomography) admitted to our hospital from 1999 to 2012 in the ALN group. In addition, each patient diagnosed with APN who was matched for sex, age, and admission date to each ALN patient was enrolled in the APN group. The medical charts of patients in these two groups were retrospectively reviewed and analyzed for comparison. RESULTS: The fever duration after hospitalization in the ALN group and the APN group were 4.85 ± 2.33 days and 2.30 ± 1.47 days respectively. The microbiological distributions and the majority of susceptibilities were similar in the ALN and APN groups. The majority of clinical manifestations are nonspecific and unreliable for the differentiation of ALN and APN. The patients with ALN were febrile for longer after antimicrobial treatment, had more nausea/vomiting symptoms, higher neutrophil count, bandemia, and C-reactive protein (CRP) levels, and lower platelet count (all p < 0.05). In multivariate analysis, initial CRP levels, nausea/vomiting symptoms, and fever duration after admission were independent variables with statistical significance to predict ALN. Severe nephromegaly occurred significantly more in the ALN group than in the APN group (p = 0.022). CONCLUSION: The majority of clinical manifestations, laboratory findings, and microbiological features are similar between patients with ALN and APN. Clinicians should keep a high index of suspicion regarding ALN, particularly for those with ultrasonographic nephromegaly, initial higher CRP, nausea/vomiting, and fever for > 5 days after antimicrobial treatment.

Mastoiditis diagnosed by clinical symptoms and imaging studies in children: disease spectrum and evolving diagnostic challenges.

BACKGROUND/PURPOSE: Acute mastoiditis has been increasingly reported. We reviewed our experience of mastoiditis in children in the era of expanding application of imaging tools and endless emerging antimicrobial resistance. METHODS: We reviewed all medical records of children (< 18 years of age) hospitalized with mastoiditis between January 2001and December 2010. Diagnosis of mastoiditis was based on clinical features and confirmed by imaging studies. Patients were classified as having acute or nonacute mastoiditis according to the duration of the disease. Acute mastoiditis was defined as illness of less than 3 weeks prior to hospitalization. Cases of longer than 3 weeks' duration were defined as nonacute mastoiditis. We compared the clinical, laboratory and microbiological features of acute and nonacute mastoiditis. RESULTS: A total of 104 children were enrolled in this study, comprising 56 acute cases and 48 nonacute cases. Fever and coryza were significantly more common in acute cases. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were both initially higher in acute mastoiditis. CRP, rather than ESR, declined faster in acute than in nonacute mastoiditis. Computerized tomography (CT) scans, but not plain films, were highly sensitive. Streptococcus pneumoniae and Haemophilus influenzae accounted for 52% of all isolates. Staphylococci, Pseudomonas spp. and polymicrobials were predominantly seen in non-acute mastoiditis. CONCLUSION: With the application of imaging studies, many cases of mastoiditis were identified. The classical postauricular signs were present in only 10% of patients. The presenting symptoms, inflammatory markers, pathogens, management and outcome were greatly influenced by the duration of the illness prior to admission.

Acquired duodenal obstruction in children.

Traumatic intramural hematoma of the duodenum is a rare cause of acquired duodenal obstruction in children, and a high degree of suspicion is therefore required to make an early and accurate diagnosis. We report a 6-year-old boy whose epigastrium was impacted by the handlebar of his bicycle during a traffic accident. The boy then experienced epigastralgia. Six days later, progressive bilious vomiting suggestive of gastrointestinal obstruction was noted. Imaging studies revealed a large hematoma extending from the fourth portion of the duodenum to the jejunum. Conservative methods of treatment failed to manage his condition. He underwent laparoscopic surgery to evacuate the hematoma. We also report a case of duodenal obstruction in a previously healthy 2-year-old girl who presented for the first time with acute symptoms of proximal intestinal obstruction. Contrast examinations showed apparent barium retention over the stomach and proximal duodenum. She underwent surgery due to persistent obstruction, and a mushroom-like foreign body was detected embedded in the orifice of the windsock duodenal web. After duodenoduodenostomy and removal of the bezoar, she had a smooth recovery and tolerated feeding well. We conclude that blunt abdominal trauma and incomplete duodenal obstruction, such as that caused by duodenal web, should be considered as possible causes of acquired proximal gastrointestinal obstruction in previously healthy children, despite their rarity.