Multiple Giant Coronary Artery Aneurysms in a Pediatric Patient with Granulomatosis with Polyangiitis.

Granulomatosis with polyangiitis (GPA) is characterized by necrotizing vasculitis of small and medium sized vessels and is rarely present in the pediatric population. Cardiac manifestations in pediatric patients with GPA are extremely uncommon, with only two known reported cases associated with coronary artery aneurysms (Rehani and Nelson in Pediatrics 147:e20200932, 2021, https://doi.org/10.1542/peds.2020-0932 ;Aghaei Moghadam et al. in Case Rep Cardiol 2020:3417910, 2020, https://doi.org/10.1155/2020/3417910 ). We report a case of a 14-year-old male who presented with a 1 month history of fatigue and shortness of breath. He ultimately was found to have multiple giant coronary aneurysms in both the left and right coronaries including a giant aneurysm in the posterior descending; this has not been previously reported. The case highlights the need for complete multi-modality imaging of the coronary arteries in patients with GPA.

Third trimester predictors of interventional timing and accuracy of fetal anticipatory guidance in tetralogy of Fallot: A multi-center study.

OBJECTIVE: The objective was to evaluate and improve accuracy of anticipatory counseling regarding neonatal intervention for prenatally diagnosed tetralogy of Fallot (TOF) by assessing new and previously published predictors of neonatal intervention. METHODS: This is a multi-center, retrospective study from three centers of 112 fetal TOF patients undergoing third trimester fetal echocardiograms from 2004 to 2017. Additional cardiac defects requiring neonatal intervention were excluded. Fetal echocardiographic, clinical, and consultation data were compared between neonatal and late intervention. Optimal echocardiographic values were determined. RESULTS: Twenty-six infants (23%) required neonatal intervention. Those infants had significantly different pulmonary valve (PV) z-scores, PV:aortic valve (AoV) ratios, PV:AoV z-score differences (absolute difference between z-scores), and increased likelihood of abnormal ductal flow. Counseling during fetal echocardiogram regarding interventional timing was accurate for 50% needing neonatal intervention and 86% undergoing late intervention (P = .002). The best neonatal intervention predictors were PV:AoV ratio of <0.6 and counseling for neonatal intervention. PV:AoV z-score difference ≥5 provided 89% negative predictive value for excluding patients from neonatal repair. CONCLUSIONS: Third trimester fetal echocardiograms can predict interventional timing. The best predictors of neonatal intervention are PV:AoV ratio <0.6, PV:AoV z-score difference ≥5, and cardiologist counseling that neonatal intervention was likely.

Aerococcus urinae associated aortic and tricuspid valve infective endocarditis.

Aerococcus urinae is a rare bacteria usually associated with urinary tract infection. It is unusually associated with endocarditis. To date only 18 cases have been reported. Among these cases, the majority had aortic valve involvement. Three had mitral and aortic valve involvement, and two had mitral and tricuspid valve involvement. We present the first reported case of A. urinae associated aortic and tricuspid valve endocarditis. Timely recognition and appropriate treatment of this fatal infection is essential to decrease morbidity and mortality.

Behcet's disease in acquired immune deficiency syndrome.

HIV/AIDS patients often present with orogenital ulcers. In the immunocompromised patient diagnosis of these ulcers pose a challenge, as there is a myriad of etiologies. We present a case of an HIV/AIDS patient with recurrent orogenital aphthosis that was confirmed to have concomitant diagnosis of Behcet's disease. Proper awareness of the causes of these ulcers is essential for prompt and effective treatment. While rare causes may be at the bottom of a differential list in an immunocompetent host, when HIV/AIDS is involved these rare causes often percolate to the top.

The first documented case of hemorrhagic stroke caused by Group B streptococcal meningitis.

We report the case of a 47 year-old female with Streptococcus agalactiae (Group B beta-hemolytic streptococcus) meningitis complicated by hemorrhagic stroke. The patient presented to the emergency department with altered mental status, agitation, confusion, respiratory distress and fever of one-day duration. Labs showed left shift leukocytosis. CSF exhibited a high white blood cell count with a predominant population of polymononuclear cells, high glucose and protein concentration. CSF cultures grew S. agalactiae. Despite appropriate antimicrobial treatment, her mental status did not improve and head CT showed two hemorrhages, diffuse cerebral edema and a right to left midline shift. After completing the course of her therapy, her mental status improved and the patient was discharged.

Genetic dissection of the ity3 locus identifies a role for ncf2 co-expression modules and suggests selp as a candidate gene underlying the ity3.2 locus.

Typhoid fever and salmonellosis, which are caused by Salmonella typhi and typhimurium, respectively, are responsible for significant morbidity and mortality in both developed and developing countries. We model typhoid fever using mice infected with Salmonella typhimurium, which results in a systemic disease, whereby the outcome of infection is variable in different inbred strains of mice. This model recapitulates several clinical aspects of the human disease and allows the study of the host response to Salmonella typhimurium infection in vivo. Previous work in our laboratory has identified three loci (Ity, Ity2, and Ity3) in the wild-derived MOLF/Ei mice influencing survival after infection with Salmonella typhimurium. Fine mapping of the Ity3 locus indicated that two sub-loci contribute collectively to the susceptibility of B6.MOLF-Ity/Ity3 congenic mice to Salmonella infection. In the current paper, we provided further evidence supporting a role for Ncf2 (neutrophil cytosolic factor 2 a subunit of NADPH oxidase) as the gene underlying the Ity3.1 sub-locus. Gene expression profiling indicated that the Ity3.1 sub-locus defined a global gene expression signature with networks articulated around Ncf2. Furthermore, based on differential expression and complementation analysis using Selp (selectin-P) knock-out mice, Selp was identified as a strong candidate gene for the Ity3.2 sub-locus.

Functional validation of the genetic architecture of Salmonella Enteritidis persistence in 129S6 mice.

The Gram-negative bacteria, Salmonella, cause a broad spectrum of clinical diseases in humans, ranging from asymptomatic carriage to life-threatening sepsis. We have designed an experimental model to study the contribution of genetic factors to the persistence of Salmonella Enteritidis during the late phase of infection in 129S6/SvEvTac and C57BL/6J mice. C57BL/6J mice cleared the bacteria from their reticuloendothelial system within a period of 42 days, whereas the 129S6 mice still presented a high bacterial load. Using this model, we have identified ten Salmonella Enteritidis susceptibility loci (Ses1, Ses1.1, and Ses3-Ses10) associated with bacterial persistence in target organs of 129S6/SvEvTac mice using a two-locus epistasis QTL linkage mapping approach. Significant statistical interactions were detected between Ses1 on chromosome 1 and Ses5 on chromosome 7 and between Ses1 and Ses4 on chromosome X. In this study, we functionally validated the genetic architecture of Salmonella persistence in 129S6 mice using single- (129S6.B6-Ses1.2 that combines Ses1 and Ses1.1 loci, 129S6.B6-Ses4, and 129S6.B6-Ses5) and double-congenic mice (129S6.B6-Ses1.2/Ses4 and 129S6.B6-Ses1.2/Ses5). These experiments demonstrate functional interactions between Ses1.2 and Ses4 or Ses5 that improve Salmonella Enteritidis clearance, validating the critical role played by gene-gene interactions in the contribution to bacterial clearance heritability. Improved bacterial clearance in double-congenic mice could be explained by the impact of Ses4 and Ses5 in combination with Ses1.2 on TH polarization since a TH2 bias (decreased Ifng and increased Il4 mRNA levels and reduced IgG2a immunoglobulins in the serum) was observed in 129S6.B6-Ses1.2/Ses5 mice and a TH17 (high Il17 expression) bias in 129S6.B6-Ses1.2/Ses4.