Treatment outcomes and time to healing of medication-related osteonecrosis of the jaw based on image findings.

OBJECTIVES: This study aimed to evaluate the prognostic treatment outcome of non-operative management of medication-related osteonecrosis of the jaw (MRONJ), particularly regarding the relationship between image findings and treatment outcomes. METHODS: This single-center, retrospective observational study included patients with MRONJ who were conservatively treated between 2010 and 2020. All patients were evaluated in terms of MRONJ treatment outcomes, time to healing, and prognostic factors, including sex, age, underlying disease, antiresorptive drug type, discontinuation of antiresorptive treatment, chemotherapy, corticosteroid treatment, diabetes mellitus, location of MRONJ, clinical stage of MRONJ, and computed tomography image findings. RESULTS: The complete healing rate among the patients was 68.5%. Cox proportional hazards regression analysis revealed that "Sequestrum formation" on the internal texture (hazard ratio = 3.66; 95% confidence interval, 1.30-10.29; P =.014) and chemotherapy (hazard ratio = 0.41; 95% confidence interval, 0.18-0.95; P =.037) were significantly associated with treatment outcome. The median time to healing in patients with "Sequestrum formation" on the internal texture (4.4 months) was significantly shorter than the median time to healing in those marked with "Sclerosis" or "Normal" (35.5 months; P <.001) and "Lytic changes with sclerosis" (14.5 months; P =.015). CONCLUSIONS: The image findings on the internal texture of the lesions at the initial examination and chemotherapy were associated with the treatment outcomes of nonoperative management of MRONJ. The image findings of "Sequestrum formation" were associated with lesions taking a short time to heal and better outcomes, whereas "Sclerosis" and "Normal" were associated with lesions with longer healing times.

Initial national investigation of the prenatal diagnosis of congenital heart malformations in Japan-Regional Detection Rate and Emergency Transfer from 2013 to 2017.

BACKGROUND: Investigation into the detection rate (DR) of congenital heart diseases (CHDs) in fetuses is important for the assessment of fetal cardiac screening systems. OBJECTIVES: We highlight issues of fetal cardiac screening in Japan. METHODS: We performed an initial national survey of fetal diagnosis of CHDs from the data of the national registry for congenital heart surgery from 2013 to 2017. Subjects were neonates and infants with moderate or severe CHDs. We investigated DR in each prefecture in Japan and emergency transfer (ET) for neonates by analyzing distance and admission day of ET with or without fetal diagnoses. RESULTS: The overall average DR in Japan was 0.41 (0.02 increase every year). No regional significant relationship was found between DR and population in each prefecture. ET was performed in 12% of neonates with prenatal diagnosis and in 63% of neonates without resulting in significant risk for ET in fetuses without a fetal diagnosis [OR 13.3 (11.6-15.3), p<0.001]. The distance of ET was shorter and admission was earlier in the neonates with a prenatal diagnosis than in those without [median 6.6 km (IQR: 4.1-25.7) vs 17.0 km (IQR: 7.4-35.3), median 0.0 day (IQR: 0.0-0.0) vs 0.0 day (IQR: 0.0-1.0), p<0.001, p<0.001, respectively] CONCLUSIONS: Prenatal cardiac diagnosis reduces geographic and chronological risks of ET for moderate to severe CHDs. DR is still developing and periodic official surveillance is required for improving prenatal cardiac diagnosis in Japan.

Influence of prostate cancer status on the prevalence of medication-related osteonecrosis of the jaw.

OBJECTIVE: The aim of this study was to evaluate the risk of osteonecrosis of the jaw (ONJ) in patients with prostate cancer, particularly the relationship between prostate cancer progression and ONJ development. STUDY DESIGN: This single-center, retrospective, observational study included 113 patients who received zoledronic acid or denosumab for prostate cancer with bone metastasis between January 2012 and March 2020. The risk of ONJ was evaluated regarding age; antiresorptive drugs; duration of antiresorptive treatment; prostate cancer status, including castration-resistant prostate cancer (CRPC) and prostate-specific antigen level; chemotherapy; radium-223 treatment; corticosteroid treatment; diabetes mellitus; and dental extractions. RESULTS: Overall, 28 patients had ONJ; 10 patients received zoledronic acid and 18 patients received denosumab. Multiple logistic regression analysis demonstrated that CRPC (odds ratio = 6.01; 95% confidence interval, 1.76-20.05; P = .004) and dental extractions (odds ratio = 12.40; 95% confidence interval, 3.42-44.70; P < .001) were significantly associated with ONJ. In addition, antiresorptive treatment lasting more than 1 year partially mediated between CRPC and development of ONJ. CONCLUSION: CRPC and dental extraction are risk factors for developing ONJ, and antiresorptive treatment lasting more than 1 year is a partial mediator between CRPC and ONJ.

A burden of sarcomere gene variants in fetal-onset patients with left ventricular noncompaction.

BACKGROUND: Left ventricular noncompaction (LVNC) is a hereditary cardiomyopathy, associated with high morbidity and mortality, but the role of genetics in cases of fetal-onset has not been fully evaluated. The goal of this study was to identify the genetic background in LVNC fetal-onset patients using next-generation sequencing (NGS). METHODS: Thirty-three fetal-onset Japanese probands with LVNC (20 males and 13 females) were enrolled. In the enrolled patients, 81 genes associated with cardiomyopathy were screened using next-generation sequencing (NGS) retrospectively. RESULTS: Twenty-three patients had congestive heart failure (CHF), and six patients had arrhythmias. Prominent trabeculations were mostly observed in lateral LV, posterior LV, and apex of LV in patients with LVNC. Twelve died; three patients experienced intrauterine death or termination of pregnancy. Overall, 15 variants were found among eight genes in 16 patients. Seven variants were detected in MYH7 and two in TPM1. Sarcomere gene variants accounted for 75.0%. A multivariable proportional hazards model revealed that CHF at diagnosis and a higher ratio of the noncompacted layer/compacted layer in the LV posterior wall were independent risk factors for death in LVNC fetal-onset patients (odds ratio = 4.26 × 106 and 1.36 × 108, p = 0.0075 and 0.0005, respectively). CONCLUSIONS: The present study is the first report focusing on genetic background combined with clinical features in LVNC fetal-onset patients using NGS. Sarcomere variants were most commonly identified in fetal-onset patients, and greater attention should be paid to fetal-onset patients with LVNC having prominent trabeculations in the LV because they are more likely to develop CHF.

Cardiofaciocutaneous syndrome with KRAS gene mutation presenting as chylopericardium.

A 12-year-old female patient with cardiofaciocutaneous syndrome in the presence of a KRAS gene mutation had episodes of pericardial effusion on ultrasound, later confirmed to be chylopericardium, which resolved after a lymphangiography. We discussed herein the pathophysiological background of this rare case and the efficacy of lymphangiography in the treatment of chylopericardium.

Right-sided hepatic hernia of normal left lobe resembling cardiac tumor.

A 3-year-old boy was referred for cardiac tumor diagnosed on transthoracic echocardiography (TTE) at another hospital. The tumor appeared to be in the right atrium and obstructed inferior vena cava flow. TTE, enhanced computed tomography (CT) and angiography were done to confirm diagnosis. Subsequently, cardiac tumor was ruled out and he was diagnosed with a very rare condition of hepatic hernia containing a normal left lobe. When cardiac tumor is suspected in the right atrium on the basis of TTE, enhanced CT or magnetic resonance imaging should be done for definitive diagnosis.