Gallbladder agenesis with hepatic impairment: a case report.

BACKGROUND: Gallbladder agenesis is a rare congenital malformation. More than 50% of cases are isolated and asymptomatic. These asymptomatic patients are principally healthy and need no interventions. However, some patients develop symptoms, presenting with clinical signs and complaints similar to those of biliary tract disease. Symptoms commonly occur in the fourth or fifth decade of life of the patient. At the present time, gallbladder agenesis is diagnosed using a combination of imaging modalities, without surgical intervention, to avert serious complications following surgery. CASE PRESENTATION: We describe a 13-year-old Japanese girl with a history of recurrent hepatic impairment, which had not been thoroughly investigated. She was referred to our hospital following 2 days of fever, fatigue, and abnormal blood tests suggested impaired liver function. Data from chest X-ray findings combined with a positive loop-mediated isothermal amplification assay result indicated Mycoplasma pneumoniae pneumonia, which was treated with oral azithromycin. To investigate potential hepatic impairment, we performed several imaging studies, namely, abdominal ultrasonography, magnetic resonance cholangiopancreatography, and contrast enhanced computed tomography. These imaging studies revealed a normal liver; however, the gallbladder was not in the usual nor any aberrant position in imaging investigations of the patient. Based on these results, we diagnosed gallbladder agenesis; however, the etiology of her hepatic impairment has not been elucidated. CONCLUSION: We present a case of gallbladder agenesis with hepatic impairment, where the diagnosis was made without surgical intervention. Clinicians should perform a detailed investigation when they encounter repeated hepatic impairment.

Characterization of the Pathogenicity of Streptococcus intermedius TYG1620 Isolated from a Human Brain Abscess Based on the Complete Genome Sequence with Transcriptome Analysis and Transposon Mutagenesis in a Murine Subcutaneous Abscess Model.

Streptococcus intermedius is known to cause periodontitis and pyogenic infections in the brain and liver. Here we report the complete genome sequence of strain TYG1620 (genome size, 2,006,877 bp; GC content, 37.6%; 2,020 predicted open reading frames [ORFs]) isolated from a brain abscess in an infant. Comparative analysis of S. intermedius genome sequences suggested that TYG1620 carries a notable type VII secretion system (T7SS), two long repeat regions, and 19 ORFs for cell wall-anchored proteins (CWAPs). To elucidate the genes responsible for the pathogenicity of TYG1620, transcriptome analysis was performed in a murine subcutaneous abscess model. The results suggest that the levels of expression of small hypothetical proteins similar to phenol-soluble modulin ß1 (PSMß1), a staphylococcal virulence factor, significantly increased in the abscess model. In addition, an experiment in a murine subcutaneous abscess model with random transposon (Tn) mutant attenuation suggested that Tn mutants with mutations in 212 ORFs in the Tn mutant library were attenuated in the murine abscess model (629 ORFs were disrupted in total); the 212 ORFs are putatively essential for abscess formation. Transcriptome analysis identified 37 ORFs, including paralogs of the T7SS and a putative glucan-binding CWAP in long repeat regions, to be upregulated and attenuated in vivo This study provides a comprehensive characterization of S. intermedius pathogenicity based on the complete genome sequence and a murine subcutaneous abscess model with transcriptome and Tn mutagenesis, leading to the identification of pivotal targets for vaccines or antimicrobial agents for the control of S. intermedius infections.

Coronary diameter in normal infants, children and patients with Kawasaki disease.

BACKGROUND: The coronary assessments in Kawasaki disease are mainly based on the Japanese Ministry of Health and Welfare criteria, which is simply classified according to the patient's age, over 5 years and less than 4-years-old. METHODS: We obtained normal values of coronary diameters adjusted for the body surface area and for the coronary anatomical site from 71 healthy children using 2-D echocardiography. We also studied patients with Kawasaki disease at three stages from the onset of illness: (i) 43 patients at admission; (ii) the subsequent 2-3 weeks; and (iii) 62 children followed at a clinic, for a median 2.2 years after the onset. No patients showed any coronary abnormalities by several echographic exams. RESULTS: The coronary diameters were strongly correlated with the body surface area (r = 0.81 in left main, r = 0.89 in proximal right, r = 0.89 in left anterior descending artery). The coronary diameters in the patient groups at admission and at 2-3 weeks later were significantly larger than those in the normal group (P < 0.01). Although the coronary diameters in the follow-up group did not show a significant difference compared with those of normal, 19% retained their coronary diameters at greater than two standard deviations above the expected mean in at least one coronary artery. CONCLUSIONS: The more strictly defined criteria adjusted for body size and for the anatomical site should be used to detect the subtle changes and to prevent the misclassification of the coronary artery abnormalities in KD.

Impaired vascular endothelium-dependent relaxation in Henoch-Schönlein purpura.

Few reports have focused on vascular endothelial function in children with Henoch-Schönlein purpura (HSP). The purpose of the present study was to assess endothelial function and to follow serial changes from the acute to convalescent phases in children with HSP. Forearm flow-mediated vasodilation was evaluated in 21 patients with HSP, aged 4.0-10.3 years (median 6.2 years), and in 14 control subjects. Vascular dimension, mean velocity, and flow volume were measured by ultrasonography in brachial artery before and after hyperemia, and during incremental infusions of nitroglycerin (0.5, 1.0 microg/kg per min). In the controls, significant increases in dimension, mean velocity, and flow volume were observed in reactive hyperemia ( P<0.01). In contrast, patients in the acute phase of HSP showed a flow velocity profile indicating a highly resistant forearm circulation, and significantly attenuated responses after hyperemia ( P<0.01 vs. control), whereas the responses to nitroglycerin were well preserved. In addition, the impaired hyperemic responses recovered in the convalescent phase, with no significant differences compared with controls. These results clearly suggest that forearm vascular endothelium-dependent relaxation was attenuated in patients with acute HSP.

Serum KL-6 level in newborns with meconium aspiration syndrome.

BACKGROUND: It has been reported that serum KL-6 increases in babies with progressing chronic lung disease (CLD). However, there have been few reports assessing KL-6 in meconium aspiration syndrome (MAS). KL-6 was measured in neonates with respiratory diseases including MAS. METHODS: Thirty-eight neonates with respiratory disease were enrolled in the study. These patients were classified into three groups, 14 patients with respiratory distress syndrome (RDS), 14 with MAS, and 10 with transient tachypnea of the newborn (TTN). The control group consisted of 12 healthy neonates. KL-6 levels were measured 1 day (median) after the birth. In the RDS group, measurement was repeated just prior to 36 weeks' postmenstrual age. RESULTS: The levels of KL-6 were 116 +/- 40 U/mL in the RDS, 281 +/- 138 U/mL in the MAS, and 106 +/- 41 U/mL in the TTN groups. KL-6 levels were significantly higher in the MAS group than in the control group (134 +/- 71 U/mL; P < 0.01). In addition, the levels were significantly higher in those with severe MAS than those with mild MAS (P < 0.05). In patients with RDS, KL-6 increased in patients who developed CLD (P < 0.05), while KL-6 levels did not change in those who did not develop CLD. CONCLUSION: These data confirm the high level of KL-6 in CLD, and suggest that KL-6 is increased in MAS.