Clinical effectiveness and safety of sirolimus in pediatric patients with complex vascular anomalies: necessitating personalized and comprehensive approaches

BackgroundManaging complex vascular anomalies in pediatric care requires comprehensive approaches. Sirolimus, an mTOR inhibitor with immunosuppressive and anti-angiogenic properties, offers promise. We evaluated sirolimus's effectiveness and safety in pediatric patients with complex vascular anomalies at a tertiary children's hospital.MethodsOur study included 20 patients, aged 1 month to 19 years, with diverse vascular anomalies resistant to conventional therapies or located in high-risk areas precluding surgery. The evaluation of response encompassed measuring the reduction in the size of the targeted vascular or lymphatic lesions as observed on radiologic imaging, along with considering improvements reported by the patients.ResultsPatients used sirolimus for a median of 2.1 years, ranging from 0.6–4.3 years. Results indicated that 60% of patients achieved complete or partial response (CR/PR), whereas 40% had stable disease (SD). Notably, no disease progression occurred. Lesion size assessment was complex, yet patients' self-reported improvements were considered. Three patients reinitiated sirolimus after discontinuation due to worsening lesions. Sirolimus treatment demonstrated good tolerability, with minor complications except for one case of Pneumocystis jiroveci pneumonia. Group comparisons based on response highlighted better outcomes in patients with vascular tumors (CR/PR group 58.0% vs. SD group 0.0%, P = 0.015) or localized measurable lesions (83.3% vs. 12.5%, P = 0.005).ConclusionOur study underscores sirolimus's potential for treating complex vascular anomalies in pediatric patients. Challenges associated with optimal treatment duration and concurrent interventions necessitate a comprehensive approach and genetic testing to optimize outcomes

Health behaviors, periodontal conditions, and periodontal pathogens in spontaneous preterm birth: A case-control study in Korea

Background: This study aims to determine whether periodontal conditions or dental health behaviors are risk factors for preterm birth (PTB), and whether periodontal pathogens are risk indicators for PTB among Korean mothers. Methods: This study was designed as a hospital-based case-control study. Examiner masking was ensured for the validity of the examinations. The mothers included those who gave birth between November 2007 and July 2009 at the obstetrics clinic of a general hospital in Seoul, Korea. Information on demographic and health conditions, periodontal conditions, and microbacterial data was collected. Results: A total of 172 women met the inclusion criteria, 59 mothers who delivered a preterm neonate were assigned to the case group while the other 113 were assigned to the control group. There were no significant differencesin demographic information, oral health conditions, and obstetric characteristics. Among health-related behaviors, only scaling within 12 months before pregnancy showed a significant difference (P = 0.031). Even in the adjusted logistic model, only the difference in the experience of scaling before pregnancy was significant between the PTB cases and the controls (P = 0.039). Periodontal disease did not exhibit a significant relationship with PTB even after adjustment for potential confounding factors. Among the microbacterial factors, only Porphyromonas gingivalis showed a slight difference (P = 0.060). Conclusion: There was a significant difference inscaling experience within 12 months before pregnancy and P. gingivalis showed a marginal difference between the PTB and the control groups but clinical periodontal conditions showed no association with PTB.This work was supported by the Korea Research Foundation Grant funded by the Korean Government (MOEHRD, Basic Research Promotion Fund, Seoul, Korea; E00230)

Elective Laparoscopic Repair after Colonoscopic Decompression for Incarcerated Morgagni Hernia

Plain radiographs of an 88-year-old woman who had experienced vomiting and abdominal distention for 3 days revealed a severely obstructed ileus, and abdominopelvic computed tomography revealed an incarcerated Morgagni hernia. The endoscope was passed through the constrictions from the diaphragmatic indentations and a thin catheter was placed for decompression. The obstructive ileus regressed markedly after the procedure; the patient underwent elective laparoscopic repair of the hernia 1 week later. This is believed to be the first case of endoscopic preoperative decompression for an incarcerated Morgagni hernia

Multiplex reverse transcription-PCR for rapid differential detection of porcine epidemic diarrhea virus, transmissible gastroenteritis virus, and porcine group A rotavirus

A novel multiplex reverse transcription polymerase chain reaction (multiplex RT-PCR) that can detect porcine epidemic diarrhea virus (PEDV), transmissible gastroenteritis virus (TGEV), and porcine group A rotavirus (GAR) was developed. The 3 viruses (PEDV, TGEV, and porcine GAR) are major agents in viral enteric diseases of piglets. As the clinical signs of these diseases are similar, including watery diarrhea, differential detection is required for etiologic diagnosis. A mixture of 3 pairs of published primers was used for amplification of viral nucleic acids, yielding 3 different amplicons with sizes of 859 bp, 651 bp, and 309 bp for TGEV, PEDV, and porcine GAR, respectively. A total of 157 specimens (78 fecal and 79 intestinal samples) from piglets with acute gastroenteritis were collected in Korea between January 2004 and May 2005. They were tested for the presence of 3 viruses by multiplex RT-PCR. Coinfections with PEDV and porcine GAR were identified in 16 farms (43.2%). PEDV, porcine GAR, and TGEV infection were 26.3%, 13.2%, and 2.7% respectively. The relative sensitivity and specificity of multiplex RT-PCR were evaluated, with results suggesting that this assay is equal in quality to conventional single-agent RT-PCR assays (sensitivity:100%, 92.9%, 100% for TGEV, PEDV, GARs; specificity: 100% for all 3 viruses). This multiplex RT-PCR is a simple assay and may be a potentially useful for rapid, sensitive, and cost-effective etiological diagnostic tool for acute viral gastroenteritis in piglets.This work was supported by Korea Research Foundation Grants (KRF-2002-070-C00069) and the Brain Korea 21 Project of the Ministry of Education & Human Resources Development, Republic of Korea

Delayed Primary Repair of Perforated Epiphrenic Diverticulum

A 68-yr-old man complaining of sudden, postprandial chest pain visited the emergency room. His symptom had been aggravated during the preceding two days. Upper gastrointestinal contrast study with gastrographin showed leakage of dye from the epiphrenic diverticulum in the lower third of the esophagus. The primary repair was urgently carried out. Upper gastrointestinal contrast study 14 days after operation revealed an esophageal leakage which was small and confined. The patient was managed with conservative treatments such as intravenous hyperali-mentation and broad-spectrum antibiotics. Forty-two days after the operation, a gastrographin swallow study showed the absence of leaks. This is the first report-ed case of a perforated epiphrenic esophageal diverticulum repaired by delayed primary repair in Korea

The Changing Landscape for Stroke\ua0Prevention in AF: Findings From the GLORIA-AF Registry Phase 2

Background GLORIA-AF (Global Registry on Long-Term Oral Antithrombotic Treatment in Patients with Atrial Fibrillation) is a prospective, global registry program describing antithrombotic treatment patterns in patients with newly diagnosed nonvalvular atrial fibrillation at risk of stroke. Phase 2 began when dabigatran, the first non\u2013vitamin K antagonist oral anticoagulant (NOAC), became available. Objectives This study sought to describe phase 2 baseline data and compare these with the pre-NOAC era collected during phase 1. Methods During phase 2, 15,641 consenting patients were enrolled (November 2011 to December 2014); 15,092 were eligible. This pre-specified cross-sectional analysis describes eligible patients\u2019 baseline characteristics. Atrial fibrillation disease characteristics, medical outcomes, and concomitant diseases and medications were collected. Data were analyzed using descriptive statistics. Results Of the total patients, 45.5% were female; median age was 71 (interquartile range: 64, 78) years. Patients were from Europe (47.1%), North America (22.5%), Asia (20.3%), Latin America (6.0%), and the Middle East/Africa (4.0%). Most had high stroke risk (CHA2DS2-VASc [Congestive heart failure, Hypertension, Age  6575 years, Diabetes mellitus, previous Stroke, Vascular disease, Age 65 to 74 years, Sex category] score  652; 86.1%); 13.9% had moderate risk (CHA2DS2-VASc = 1). Overall, 79.9% received oral anticoagulants, of whom 47.6% received NOAC and 32.3% vitamin K antagonists (VKA); 12.1% received antiplatelet agents; 7.8% received no antithrombotic treatment. For comparison, the proportion of phase 1 patients (of N = 1,063 all eligible) prescribed VKA was 32.8%, acetylsalicylic acid 41.7%, and no therapy 20.2%. In Europe in phase 2, treatment with NOAC was more common than VKA (52.3% and 37.8%, respectively); 6.0% of patients received antiplatelet treatment; and 3.8% received no antithrombotic treatment. In North America, 52.1%, 26.2%, and 14.0% of patients received NOAC, VKA, and antiplatelet drugs, respectively; 7.5% received no antithrombotic treatment. NOAC use was less common in Asia (27.7%), where 27.5% of patients received VKA, 25.0% antiplatelet drugs, and 19.8% no antithrombotic treatment. Conclusions The baseline data from GLORIA-AF phase 2 demonstrate that in newly diagnosed nonvalvular atrial fibrillation patients, NOAC have been highly adopted into practice, becoming more frequently prescribed than VKA in Europe and North America. Worldwide, however, a large proportion of patients remain undertreated, particularly in Asia and North America. (Global Registry on Long-Term Oral Antithrombotic Treatment in Patients With Atrial Fibrillation [GLORIA-AF]; NCT01468701