Successful Noninvasive Respiratory Management of an Infant with Bilateral Choanal Atresia and a Supernumerary Nostril Located on the Columella by a Mouthpiece: A Case Report.

BACKGROUND Choanal atresia with a supernumerary nostril located on the columella is extremely rare. Infants are obligate nasal breathers because the oral airway is invariably blocked during calm respiration. Infants breathe through the mouth only during crying, and they only have nasal breathing until 5 months of life. Congenital nasal anomalies have been reported to be fatal from birth, requiring tracheal intubation or tracheostomy in the early postnatal period. In these cases, it is crucial to maintain an adequate airway. CASE REPORT A 2948-g female infant was born at 40 weeks by normal vaginal delivery. Her Apgar scores were 9 and 9 at 1 and 5 min, respectively. She had retractive breathing, cyanosis, and a supernumerary nostril at birth. She had no other anomalies. Computed tomography showed bilateral membranous choanal atresia. She needed nasal continuous positive pressure or a high-flow nasal canula for oxygen desaturation during crying, apnea, and dyspnea. However, her respiratory symptoms did not improve completely. On day 25 of life, she was given a mouthpiece to support mouth breathing. Her respiratory symptoms improved gradually, and she was discharged on day 73 of life with a mouthpiece. CONCLUSIONS A very rare case of choanal atresia with a supernumerary nostril located on the columella was described. A mouthpiece was effective for breathing, obviating the need for emergency surgical intervention in the early postnatal period. Emergency procedures were avoided, probably because this case involved incomplete bilateral membranous choanal atresia rather than complete bony atresia.

Comparison of CO-RADS Scores Based on Visual and Artificial Intelligence Assessments in a Non-Endemic Area.

In this study, we first developed an artificial intelligence (AI)-based algorithm for classifying chest computed tomography (CT) images using the coronavirus disease 2019 Reporting and Data System (CO-RADS). Subsequently, we evaluated its accuracy by comparing the calculated scores with those assigned by radiologists with varying levels of experience. This study included patients with suspected SARS-CoV-2 infection who underwent chest CT imaging between February and October 2020 in Japan, a non-endemic area. For each chest CT, the CO-RADS scores, determined by consensus among three experienced chest radiologists, were used as the gold standard. Images from 412 patients were used to train the model, whereas images from 83 patients were tested to obtain AI-based CO-RADS scores for each image. Six independent raters (one medical student, two residents, and three board-certified radiologists) evaluated the test images. Intraclass correlation coefficients (ICC) and weighted kappa values were calculated to determine the inter-rater agreement with the gold standard. The mean ICC and weighted kappa were 0.754 and 0.752 for the medical student and residents (taken together), 0.851 and 0.850 for the diagnostic radiologists, and 0.913 and 0.912 for AI, respectively. The CO-RADS scores calculated using our AI-based algorithm were comparable to those assigned by radiologists, indicating the accuracy and high reproducibility of our model. Our study findings would enable accurate reading, particularly in areas where radiologists are unavailable, and contribute to improvements in patient management and workflow.

Association of Matrix Metalloproteinase-2 mRNA Expression with Subtypes of Pediatric Cholesteatoma.

OBJECTIVE: Cholesteatoma is a clinically heterogeneous disease, with some patients showing spontaneous regression, while others experiencing an aggressive, lethal disease. Cholesteatoma in children can be divided into two types: congenital and acquired. Identifying good prognostic markers is needed to help select patients who will require immediate surgical intervention. Matrix metalloproteinase-2 (MMP2) was previously reported to play an important role in cholesteatoma progression, by promoting bone destruction and keratinocyte infiltration. Herein, we analyzed MMP2 mRNA expression level in cholesteatoma using RNA-in situ hybridization in formalin-fixed, paraffin-embedded (FFPE) tissue samples. METHODS: Sixty patients with cholesteatoma under 15 years old, who underwent their primary surgery at Aichi Medical University's Otolaryngology Department, were analyzed for MMP2 expression level, using RNA-in situ hybridization. RESULTS: There were no significant differences in MMP2 mRNA expression level between congenital cholesteatoma and acquired cholesteatomas. In congenital cholesteatoma, higher MMP2 signals were observed in the open type than in the closed type (p < 0.001). In acquired cholesteatoma, higher MMP2 signals were observed in the pars tensa than in the pars flaccida (p < 0.001). MMP2 mRNA expression level was almost exclusively found in the fibroblasts or in the inflammatory cells in the stroma, but not in the epithelium. CONCLUSION: Our study reveals that MMP2 mRNA expression level is strongly associated with the subtypes of cholesteatoma. The findings suggest that the level of expression of MMP2 mRNA may be related to the pathogenesis and aggressive features of cholesteatoma.

Distribution of descending necrotizing mediastinitis and efficacy of distribution-specific drainage.

BACKGROUND: Descending necrotizing mediastinitis (DNM) resulting from oropharyngeal and cervical abscess is a life-threatening condition. This study attempted to improve our recognition of the extension and distribution of the abscess for ideal thoracic drainage. METHODS: We performed a retrospective clinical analysis of seven patients who underwent thoracic drainage for DNM with available clinical data. For mapping and classification of the distribution of the abscess, computed tomography and intraoperative findings were utilized. RESULTS: To cure patients, cervical drainage and thoracic drainage were performed 14 and 11 times, respectively. The operation time for thoracic drainage and intraoperative blood loss were 141±77 min and 103±103 g, respectively. The mean hospital stay was 66±41 days. All patients are alive without recurrence. We divided the abscess distribution into nine categories including the anterior thoracic wall, according to the computed tomography and intraoperative findings. The rate of abscess descended gradually toward the lower mediastinum. Abscesses were not necessarily continuous, and skipped lesions were occasionally noted. CONCLUSIONS: We were able to cure all seven patients with DNM. It might be helpful to recognize the exact distribution of the abscess and distribution-specific drainage using a new map and classification of thoracic abscess.

Availability of audiological evaluation for the differential diagnosis of clinical otosclerosis.

OBJECTIVE: The availability of an audiological evaluation for the differential diagnosis of clinical otosclerosis (OS) was examined. METHODS: Included were 98 patients (107 ears) with OS and 19 patients (20 ears) with incudostapedial disconnection (ISD) diagnosed by surgery between 2009 and 2017 at Aichi University Hospital. Results of preoperative pure-tone audiometry and impedance audiometry were analyzed. The most reliable index for distinguishing the two diseases was evaluated, and the cut-off value, sensitivity, and specificity were calculated. RESULTS: In the univariate analysis, age, static compliance, reversed ipsilateral acoustic reflex (reversed IAR) at 0.5 kHz and 2 kHz, negative contralateral acoustic reflex (negative CAR), difference between the air-bone gap at 0.25 and 2 kHz (0.25-2 kHzABG) and that at 0.25 and 4 kHz (0.25-4 kHzABG) showed statistical significance between the two diseases, whereas the Carhart notch did not. Multivariate logistic regression analysis by the variable selection method showed that reversed IAR (0.5 kHz), negative CAR, and 0.25-4 kHzABG were statistically significantly better factors for the differential diagnosis of the two diseases. The receiver operating characteristic curve for diagnosing OS using the difference between air-bone gap at 0.25 kHz and 4 kHz showed moderate accuracy. If the cut-off value of 0.25-4 kHzABG was 10 dB, then the sensitivity and specificity were 81.1% and 55.0%, respectively. In other words, if the cut-off value of 0.25-4 kHzABG was less than 10 dB, then the possibility of diagnosing ISD becomes higher, and if greater than 10 dB, then the possibility of diagnosing OS becomes higher. CONCLUSION: The differences in the air-bone gap at low and high frequency in pure-tone audiometry, static compliance, and acoustic reflex in impedance audiometry are useful for distinguishing OS from ISD. However, combinations of several examinations including computed tomography imaging are needed for the differential diagnosis of other ear diseases and specific preoperative planning.

Successful Introduction of Peritoneal Dialysis in an End-stage Renal Failure Patient with Idiopathic Aplastic Anemia.

A 45-year-old man with idiopathic aplastic anemia required renal replacement therapy (RRT) due to end-stage renal disease (ESRD). We succeeded in inserting the peritoneal dialysis (PD) catheter under cover of frequent red blood cell and platelet infusions because of severe pancytopenia. During the one-year period after starting PD using an ultraviolet-ray sterilization device, he developed severe leukopenia but no PD-related peritonitis or exit site/tunnel infection until he died of pneumonia. This case suggests that PD might be a suitable choice as RRT in ESRD patients with aplastic anemia, even in those with severe pancytopenia.

Vwf K1362A resulted in failure of protein synthesis in mice.

Von Willebrand factor (VWF) is synthesized in megakaryocytes and endothelial cells (ECs) and has two main roles: to carry and protect coagulation factor VIII (FVIII) from degradation by forming VWF-FVIII complex; and to mediate platelet adhesion and aggregation at sites of vascular injury. Previous research using the HEK293 cell line revealed that the VWF K1362 mutation interacted directly with platelet glycoprotein Ib (GPIb). Vwf K1362A knock-in (KI) mice were therefore generated to verify the in vivo function of residue 1362 in binding to platelet GPIb. The Cre-loxP system was employed to introduce the Vwf K1362A mutation systemically in mice. In blood coagulation analysis, the VWF antigen (VWF:Ag) of Lys1362Ala KI homozygous (homo) mice was below the sensitivity of detection by enzyme-linked immunosorbent assay. FVIII activities (FVIII:C) were 47.9 ± 0.3 and 3.3 ± 0.3% (K1362A heterozygous (hetero) and K1362A KI homo mice, respectively) compared to wild-type mice. Immunohistochemical staining analysis revealed that VWF protein did not exist in ECs of K1362A KI homo mice. These results indicated that VWF protein synthesis of K1362A was impaired after transcription in mice. K1362 seems to represent a very important position not only for VWF function, but also for VWF synthesis in mice.

[Biomarkers of Leukemia ~Description of the Practical Use and Operation in the Clinical Laboratory~]-.

Genetic testing of hematological malignancy is-.indispensable to categorize and diagnose leukemia. The quantitation of fusion gene mRNA built up by chromosomal translocation including BCR-ABL1 (major, minor), RUNX1-RUNX1T1, and PML-RARA and detection of the JAK2 (V617F) mutant gene are performed by our- selves in our hospital. Efficient, practical use is necessary because the number of medical technologists is limited and numbers of tests are increasing annually. The detection of leukemic cells is important in hematological tests. In addition, experienced medical technologists can predict the existence of fusion mutant genes. In this report, we introduce our experience regarding the practical use and operation of biomarkers for leukemia. Medical technologists take advantage of peripheral blood tests for screening, such as the complete blood count (CBC), hemogram, fibrin and fibrinogen degradation products (FDP), and the quantitation of fusion gene mRNA, which offers a definitive diagnosis including BCR-ABL1, RUNX1-RUNX1T1, and PML-RARA, and genetic tests are performed efficiently. [Review].

Factors affecting postoperative outcome in otosclerosis patients: Predictive role of audiological and clinical features.

OBJECTIVE: Factors affecting postoperative hearing results of patients with otosclerosis were analyzed. METHODS: Included were 191 patients with otosclerosis in whom 234 primary stapes surgeries were performed from August 1991 to December 2011 by one surgeon in three tertiary hospitals. Evaluation of factors affecting postoperative hearing thresholds at individual frequencies were performed by logistic regression analysis. RESULTS: Closure of the air-bone gap (ABG) after surgery was good at 2kHz, but poor at 4kHz and frequencies under 1kHz. In addition, improvement at 8kHz was worse than that at any other frequency. Multivariate logistic regression analysis by the stepwise method showed that under the mean preoperative ABG (odds ratio [OR]=2.42), unilaterality (OR=2.53) and male sex (OR=2.65) were significantly better prognostic factors at 250Hz. At 500Hz, under mean preoperative ABG (OR=2.56) was the significantly better factor. No significant factors were found at 1kHz. Cochlear otosclerosis (OR=3.57) was a significantly worse prognostic factor at 2kHz. Under mean preoperative ABG (OR=2.82) and younger age (OR=1.03) were significantly better prognostic factors at 4kHz. At 8kHz, worse preoperative air conduction threshold (OR=1.96) was a significantly better prognostic factor. CONCLUSION: Preoperative ABG, preoperative air conduction threshold, cochlear otosclerosis, male sex, laterality and age were significant prognostic factors for postoperative hearing outcome in otosclerosis patients. Especially, preoperative ABG was a significant prognostic factor at multiple frequencies. We suppose that patients with larger preoperative ABG have some other otosclerotic lesions outside the oval window niche.

Rhof promotes murine marginal zone B cell development.

RhoF is a member of the Rho GTPase family that has been implicated in various cell functions including long filopodia formation, adhesion, and migration of cells. Although RhoF is expressed in lymphoid tissues, the roles of RhoF in B cell development remain largely unclear. On the other hand, other members of the Rho GTPase family, such as Cdc42, RhoA, and Rac, have been intensively studied and are known to be required for B cell development in the bone marrow and spleen. We hypothesized that RhoF is also involved in B cell development. To examine our hypothesis, we analyzed B cell development in RhoF knockout (KO) mice and found a significant reduction in marginal zone (MZ) B cells in the spleen, although T cell development in the thymus and spleen was not affected. Consistent with these results, the width of the MZ B cell region in the spleen was significantly reduced in the RhoF KO mice. However, the antigen-specific antibody titer of IgM and IgG3 after MZ B cell-specific antigen (T cell-independent antigen, type I) stimulation was not affected by RhoF deletion. Furthermore, we demonstrated that RhoF was dispensable for stromal cell-derived factor-1α- and B lymphocyte chemoattractant-induced B cell migration. These results suggest that RhoF promotes MZ B cell development in the spleen.

Factors affecting fenestration of the footplate in stapes surgery: effectiveness of Fisch's reversal steps stapedotomy.

OBJECTIVE: To analyze the factors affecting the fenestration of the footplate in stapes surgery and to evaluate the effectiveness of Fisch's reversal steps stapedotomy. STUDY DESIGN: Retrospective study. PATIENTS: A total of 191 patients with otosclerosis in whom 230 primary stapes surgeries were performed by 1 surgeon in 3 tertiary hospitals. MAIN OUTCOME MEASURE: Evaluation of factors affecting the fenestration of the footplate in stapes surgery by logistic regression analysis. RESULTS: Stapedotomy (small hole in stapes footplate) was performed in 148 ears (64.3%). Partial stapedectomy (half removal of stapes footplate) was performed in 65 ears (28.3%). Total stapedectomy (total removal of stapes footplate) was performed in 17 ears (7.4%). Stapedotomy could be performed in 72.1% of cases (75/104) in which the stapes suprastructure was removed after insertion of the prosthesis and in 57.9% of cases (73/126) in which the stapes suprastructure was removed before the insertion. Stapedotomy could be performed in 65.4% of cases (117/179) in which a 0.6-mm-thick Schuknecht-type Teflon wire piston was used and in 60.8% (31/51) in which a whole Teflon piston was used. Multivariate analysis of factors affect stapedotomy using logistic regression analysis showed that the surgical order of the removal of the stapes suprastructure and insertion of the prosthesis were the most important 2 factors. CONCLUSION: Fisch's reversal steps technique was useful in performing stapedotomy in all otosclerosis patients. Manipulation of the prosthesis when crimping it to the incus is also important. The prosthesis should be crimped onto the incus and inserted in the footplate opening gently and atraumatically.

Craniometaphyseal dysplasia unnoticed until 19 years of age: First diagnosed from facial nerve paralysis.

Craniometaphyseal dysplasia (CMD) is a rare congenital bone disorder with facial dysmorphism developing from early childhood. We describe an unusual case of CMD unnoticed until the patient was 19 years old. Her disorder was diagnosed for the first time from her facial nerve paralysis, and was treated with high-dose corticosteroids. This report indicates the need for extreme caution in dealing with facial nerve paralysis since early detection and accurate diagnosis is important in the treatment of bone diseases. High-dose corticosteroid could be effective in treating facial nerve paralysis, even when nerves have been directly constricted by a bony overgrowth.