Deep learning to assess right ventricular ejection fraction from two-dimensional echocardiograms in precapillary pulmonary hypertension.

BACKGROUND: Precapillary pulmonary hypertension (PH) is characterized by a sustained increase in right ventricular (RV) afterload, impairing systolic function. Two-dimensional (2D) echocardiography is the most performed cardiac imaging tool to assess RV systolic function; however, an accurate evaluation requires expertise. We aimed to develop a fully automated deep learning (DL)-based tool to estimate the RV ejection fraction (RVEF) from 2D echocardiographic videos of apical four-chamber views in patients with precapillary PH. METHODS: We identified 85 patients with suspected precapillary PH who underwent cardiac magnetic resonance imaging (MRI) and echocardiography. The data was divided into training (80%) and testing (20%) datasets, and a regression model was constructed using 3D-ResNet50. Accuracy was assessed using five-fold cross validation. RESULTS: The DL model predicted the cardiac MRI-derived RVEF with a mean absolute error of 7.67%. The DL model identified severe RV systolic dysfunction (defined as cardiac MRI-derived RVEF < 37%) with an area under the curve (AUC) of .84, which was comparable to the AUC of RV fractional area change (FAC) and tricuspid annular plane systolic excursion (TAPSE) measured by experienced sonographers (.87 and .72, respectively). To detect mild RV systolic dysfunction (defined as RVEF ≤ 45%), the AUC from the DL-predicted RVEF also demonstrated a high discriminatory power of .87, comparable to that of FAC (.90), and significantly higher than that of TAPSE (.67). CONCLUSION: The fully automated DL-based tool using 2D echocardiography could accurately estimate RVEF and exhibited a diagnostic performance for RV systolic dysfunction comparable to that of human readers.

Echocardiographic estimation of right ventricular diastolic stiffness based on pulmonary regurgitant velocity waveform analysis in precapillary pulmonary hypertension.

Right ventricular (RV) diastolic stiffness is an independent predictor of survival and is strongly associated with disease severity in patients with precapillary pulmonary hypertension (PH). Therefore, a fully validated echocardiographic method for assessing RV diastolic stiffness needs to be established. This study aimed to compare echocardiography-derived RV diastolic stiffness and invasively measured pressure-volume loop-derived RV diastolic stiffness in patients with precapillary PH. We studied 50 consecutive patients with suspected or confirmed precapillary PH who underwent cardiac catheterization, magnetic resonance imaging, and echocardiography within a 1-week interval. Single-beat RV pressure-volume analysis was performed to determine the gold standard for RV diastolic stiffness. Elevated RV end-diastolic pressure (RVEDP) was defined as RVEDP ≥ 8 mmHg. Using continuous-wave Doppler and M-mode echocardiography, an echocardiographic index of RV diastolic stiffness was calculated as the ratio of the atrial-systolic descent of the pulmonary artery-RV pressure gradient derived from pulmonary regurgitant velocity (PRPGDAC) to the tricuspid annular plane movement during atrial contraction (TAPMAC). PRPGDAC/TAPMAC showed significant correlation with ß (r = 0.54, p < 0.001) and RVEDP (r = 0.61, p < 0.001). A cut-off value of 0.74 mmHg/mm for PRPGDAC/TAPMAC showed 83% sensitivity and 93% specificity for identifying elevated RVEDP. Multivariate analyses indicated that PRPGDAC/TAPMAC was independently associated with disease severity in patients with precapillary PH, including substantial PH symptoms, stroke volume index, right atrial size, and pressure. PRPGDAC/TAPMAC, based on pulmonary regurgitation velocity waveform analysis, is useful for the noninvasive assessment of RV diastolic stiffness and is associated with prognostic risk factors in precapillary PH.

Impact of cancer on the prevalence, management, and outcome of patients with chronic thromboembolic pulmonary hypertension.

INTRODUCTION: Chronic thromboembolic pulmonary hypertension (CTEPH) results from unresolved thrombotic obstruction of the pulmonary vasculature. Cancer is a known risk factor for CTEPH. This study aimed to determine the impact of cancer on the prevalence, management, and outcomes of patients with CTEPH. MATERIALS AND METHODS: In this retrospective study involving 99 patients sequentially diagnosed with CTEPH in our hospital, the prevalence of 10 comorbid conditions including a past history of cancer at the time of CTEPH diagnosis were calculated. RESULTS: Among the 99 patients, 17 (17%) had a history of cancer. Breast cancer (n = 6) was the most common cancer type, followed by gastrointestinal cancer (n = 3), uterine cancer (n = 2), and malignant lymphoma (n = 2). Between patients with and without cancer, there were no differences in the demographics, severity of CTEPH, and management; however, the 5-year survival rate was lower for patients with cancer (65%) than for those without (89%). In addition, patients with cancer had significantly worse survival than those without (p = 0.03 by log-rank test). During follow-up, nine patients developed cancer after the diagnosis of CTEPH. Among the 99 patients, 13 died during follow-up, 6 (46%) of whom died of cancer. CONCLUSIONS: 17% of our patients with CETPH were diagnosed with cancer, with breast and gastrointestinal tract cancers being the most common. Cancer comorbidity was associated with a poor prognosis and contributed to death in 46% of deceased patients. The impact of cancer on CTEPH should be further evaluated in the future.

Pulmonary arterial hypertension in an 80-year-old man with long-term use of cyclophosphamide.

An 80-year-old man diagnosed with primary macroglobulinemia 7 years earlier had been treated with cyclophosphamide, following which he developed dyspnea on exertion. Cyclophosphamide was discontinued. The patient's dyspnea, however, failed to improve. Right heart catheterization (RHC) revealed precapillary pulmonary hypertension (PH). He was transferred to our institution for further examination. Prior use of cyclophosphamide was the patient's only risk factor for PH, and cyclophosphamide use was considered as a possible cause of PH in this case. He was treated with tadalafil and dyspnea gradually improved. A follow-up RHC exhibited improvement in mean pulmonary arterial pressure and pulmonary vascular resistance.

Clinical and Hemodynamic Responses to Imatinib in Pulmonary Veno-Occlusive Disease/Pulmonary Capillary Hemangiomatosis: A Retrospective Pilot Study of Five Cases and Review of the Literature.

BACKGROUND: Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare types of pulmonary arterial hypertension with dismal prognoses; there is no established medical treatment for these conditions. Possible efficacy of imatinib against these conditions has been reported in 15 cases; however, how and in whom imatinib is effective remain unknown. METHODS: We retrospectively evaluated clinical data from consecutive patients with PVOD/PCH treated with imatinib at our institution. The diagnosis of PVOD/PCH was established using the following criteria: pre-capillary pulmonary hypertension; diffusion capacity of the lung for carbon monoxide < 60%; and two or more high-resolution computed tomography findings of interlobular septal thickening, centrilobular opacities, and mediastinal lymphadenopathy. The dose of pulmonary vasodilators remained unchanged during the assessment of imatinib. RESULTS: The medical records of five patients with PVOD/PCH were reviewed. The patients were aged 67 ± 13 years, their diffusion capacity of the lung for carbon monoxide was 29 ± 8%, and their mean pulmonary artery pressure was 40 ± 7 mmHg. Imatinib was administered at 50-100 mg/day; consequently, the World Health Organization functional class improved in one patient. In addition, imatinib improved the arterial oxygen partial pressure in this and another patient (these two also experienced a decreased mean pulmonary artery pressure and pulmonary vascular resistance after imatinib usage). CONCLUSIONS: This study indicated that imatinib improves the clinical condition, including pulmonary hemodynamics, of some patients with PVOD/PCH. In addition, patients with a certain high-resolution computed tomography pattern or PCH-dominant vasculopathy may respond favorably to imatinib.

Efficacy and safety of oral pulmonary vasodilators in pulmonary veno-occlusive disease.

Pulmonary veno-occlusive disease (PVOD) or pulmonary capillary hemangiomatosis (PCH) is a rare subtype of pulmonary hypertension with dismal prognosis. Limited data are available on the efficacy and safety of orally administered pulmonary vasodilators for PVOD/PCH. Whether and how systemic sclerosis (SSc) affects the clinical outcomes of PVOD/PCH is also unknown. This study aimed to determine the clinical and hemodynamic efficacy and safety of oral pulmonary vasodilators for PVOD/PCH and clarify the possible effects of SSc on the clinical presentation of PVOD/PCH. We retrospectively analyzed the clinical data of 15 patients with PVOD/PCH treated with oral pulmonary vasodilators in our department since 2001. Six of them had SSc. Oral pulmonary vasodilators were administered either as single agents (n = 10) or in combination (n = 5). Treatment improved the functional class of five patients, and pulmonary arterial pressure and pulmonary vascular resistance decreased by 10 ± 12 mmHg and 36 ± 19%, respectively (p < 0.05 for both, n = 13), whereas pulmonary edema developed in three patients. The mean survival was 3.9 years, and the 1- and 3-year survival rates were 93% and 65%, respectively. The clinical presentation, including survival, was similar between patients with and without SSc. In our PVOD/PCH cohort, oral pulmonary vasodilators caused pulmonary edema in 20% of patients, but more than 80% of patients experienced significant pulmonary vasodilatory effects, and the overall prognosis was better than that previously reported. SSc does not adversely affect the clinical outcomes of PVOD/PCH.

Combination therapy with remdesivir and immunomodulators improves respiratory status in COVID-19: A retrospective study.

Immunomodulators (tocilizumab/baricitinib) improve outcomes of coronavirus disease 2019 (COVID-19) patients, but the synergistic effect of remdesivir is unknown. The effect of combination therapy with remdesivir, immunomodulators, and standard treatment in COVID-19 patients was investigated. This retrospective, single-center study included COVID-19 patients who were treated with tocilizumab or baricitinib. The severity of respiratory status in the two groups on Days 14 and 28 and the duration to respiratory recovery in both groups were compared, and the effect of remdesivir use on respiratory status was examined in a multivariate analysis. Ninety-eight patients received tocilizumab or baricitinib; among them, 72 used remdesivir (remdesivir group) and 26 did not (control group). The remdesivir group achieved faster respiratory recovery than the control group (median 11 vs. 21 days, p = 0.033), faster weaning from supplemental oxygen (hazard ratio [HR]: 2.54, 95% confidence interval [CI]: 1.14-5.66, p = 0.021). Age, body mass index, diabetes mellitus, and time from onset to oxygen administration were independent prognostic factors. The remdesivir group achieved better severity level at Days 14 and 28 (p = 0.033 and 0.003, respectively) and greater improvement from baseline severity (p = 0.047 and 0.018, respectively). Remdesivir combination therapy did not prolong survival (HR: 0.31, 95% CI: 0.04-2.16, p = 0.23). Among severely ill COVID-19 patients who received immunomodulator, remdesivir contributed to a shorter respiratory recovery time and better respiratory status at Days 14 and 28. Concomitant remdesivir with immunomodulators and standard treatment may provide additional benefit in improving respiratory status of COVID-19 patients.

Accuracy of Swan‒Ganz catheterization-based assessment of right ventricular function: Validation study using high-fidelity micromanometry-derived values as reference.

Right ventricular (RV) function critically affects the outcomes of patients with pulmonary hypertension (PH). Pressure wave analysis using Swan‒Ganz catheterization (SG-cath) allows for the calculation of indices of RV function. However, the accuracy of these indices has not been validated. In the present study, we calculated indices of systolic and diastolic RV functions using SG-cath-derived pressure recordings in patients with suspected or confirmed PH. We analyzed and validated the accuracies of three RV indices having proven prognostic values, that is, end-systolic elastance (Ees)/arterial elastance (Ea), ß (stiffness constant), and end-diastolic elastance (Eed), using high-fidelity micromanometry-derived data as reference. We analyzed 73 participants who underwent SG-cath for the diagnosis or evaluation of PH. In this study, Ees/Ea was calculated via the single-beat pressure method using [1.65 × (mean pulmonary arterial pressure) - 7.79] as end-systolic pressure. SG-cath-derived Ees/Ea, ß, and Eed were 0.89 ± 0.69 (mean ± standard deviation), 0.027 ± 0.002, and 0.16 ± 0.02 mmHg/ml, respectively. The mean differences (limits of agreement) between SG-cath and micromanometry-derived data were 0.13 (0.99, -0.72), 0.002 (0.020, -0.013), and 0.04 (0.20, -0.12) for Ees/Ea, ß, and Eed, respectively. The intraclass correlation coefficients of the indices derived from the two catheterizations were 0.76, 0.71, and 0.57 for Ees/Ea, ß, and Eed, respectively. In patients with confirmed or suspected PH, SG-cath-derived RV indices, especially Ees/Ea and ß, exhibited a good correlation with micromanometry-derived reference values.

Comparative Efficacy of Tocilizumab and Baricitinib Administration in COVID-19 Treatment: A Retrospective Cohort Study.

Background and Objectives: Tocilizumab and baricitinib have been observed to improve the outcomes of patients with coronavirus disease 2019 (COVID-19). However, a comparative evaluation of these drugs has not been performed. Materials and Methods: A retrospective, single-center study was conducted using the data of COVID-19 patients admitted to Hokkaido University hospital between April 2020 and September 2021, who were treated with tocilizumab or baricitinib. The clinical characteristics of the patients who received tocilizumab were compared to those of patients who received baricitinib. Univariate and multivariate logistic regression analyses of the outcomes of all-cause mortality and improvement in respiratory status were performed. The development of secondary infection events was analyzed using the Kaplan-Meier method and the log-rank test. Results: Of the 459 patients hospitalized with COVID-19 during the study, 64 received tocilizumab treatment and 34 baricitinib treatment, and those 98 patients were included in the study. Most patients were treated with concomitant steroids and exhibited the same severity level at the initiation of drug treatment. When compared to each other, neither tocilizumab nor baricitinib use were associated with all-cause mortality or improvement in respiratory status within 28 days from drug administration. Conclusions: Age, chronic renal disease and early administration of TCZ or BRT from the onset of COVID-19 were independent prognostic factors for all-cause mortality, whereas anti-viral drug use and the severity of COVID-19 at baseline were associated with an improvement in respiratory status. Secondary infection-free survival rates of patients treated with tocilizumab and those treated with baricitinib did not significantly differ. The results suggest that both tocilizumab and baricitinib could be clinically equivalent agents of choice in treatment of COVID-19.

Differences in post-operative complications after reconstruction for congenital biliary dilatation in a single institution-Roux-en-Y hepaticojejunostomy versus hepaticoduodenostomy.

BACKGROUND: The standard surgical procedure for congenital biliary dilatation (CBD) consists of excision of extrahepatic bile duct and Roux-en-Y hepaticojejunostomy (HJ). However, alternative reconstructive operations for CBD includes hepaticoduodenostomy (HD). We compared postoperative complications and therapeutic outcomes of these two operations at our institution. METHODS: From 1981 to 2009, there were 23 traceable patients who underwent operation for CBD, They were divided into an HJ Group (n = 15) and an HD Group (n = 8). Demographic and outcome data were compared. RESULTS: There were no significant differences in postoperative complications (cholangitis, pancreatitis, and anastomotic stenosis) and current blood test data (AST, ALT, Total Bilirubin, Direct Bilirubin, Amylase) between the two groups. Current abdominal pain and carcinogenesis were not observed in either group. Intrahepatic stones occurred in one patient in each group, both > 25 years post-operation. CONCLUSION: HD is considered to be an acceptable reconstruction method compared to HJ, based on our results. There has been a suggestion that inadequate diversion in HD might increase the risk of cholangiocarcinoma. To date, in this series, that has not happened.

Basal cell adenoma of the lung identified with obstructive pneumonia.

The seromucinous glands of the bronchi can give rise to tumors resembling those of the salivary glands. Basal cell adenoma (BCA) has not been reported in salivary gland-type tumors of the lung. Here, we report a case of an 86-year-old Asian man who suffered from pneumonia in the left upper lung field that may be associated with an endobronchial tumor occluding B1 + 2a + b. The bronchoscopic biopsy of the tumor revealed that the basaloid cells, which constituted a major component of the tumor and were positive for p40/p63 immunohistochemistry, exhibited a cord-like structure. The Ki-67 (MKI67) staining was less than 1% positive. These findings suggest that the endobronchial tumor was BCA of the lung, which caused obstructive pneumonia in the patient.

Relation between amount of bile ducts in portal canal and outcomes in biliary atresia.

BACKGROUND: There is no good prognostic indicator for biliary atresia (BA). We reviewed liver biopsies taken during the initial procedure to find a prognostic marker. METHODS: Thirty-two BA cases underwent Kasai operation from 1976 to 2009. We compared two groups at 1, 3, and 9 years. Group A required liver transplantation or died. Group B survived with their native liver. Biopsies were analyzed for liver fibrosis, portal-central vein bridging (P-C bridging), ductal plate malformation (DPM) and the number of the bile ducts in portal canal/measured surface area of the portal canal (BDP ratio). Statistical comparisons of the multiple data were evaluated by Mann-Whitney U test, Student's t test and Pearson's Chi-square test. Regression analysis with P < 0.05 was considered significant. RESULTS: BDP ratios (/mm(2)) were 2.4 ± 1.5 in Group A1 (n = 9) vs 4.6 ± 2.4 in Group B1 (n = 23) (P = 0.01); 2.6 ± 1.4 in Group A3 (n = 14) vs 5.1 ± 2.5 in Group B3 (n = 18) (P < 0.01), 3.0 ± 2.2 in Group A9 (n = 15) vs 4.9 ± 2.5 in Group B9 (n = 15) (P < 0.05). There was no significant difference in any other finding. CONCLUSION: The BDP ratio is a sound prognostic indicator in BA.

Internal Hernias in the Era of Multidetector CT: Correlation of Imaging and Surgical Findings.

Clinical diagnosis of internal hernias is challenging because of their nonspecific signs and symptoms. Many types of internal hernias have been defined: paraduodenal, small bowel mesentery-related, greater omentum-related, lesser sac, transverse mesocolon-related, pericecal, sigmoid mesocolon-related, falciform ligament, pelvic internal, and Roux-en-Y anastomosis-related. An internal hernia is a surgical emergency that can develop into intestinal strangulation and ischemia. Accurate preoperative diagnosis is crucial for appropriate management. Multidetector computed tomography (CT), with its thin-section axial images, high-quality multiplanar reformations, and three-dimensional images, currently plays an essential role in preoperative diagnosis of internal hernias. The diagnostic approach to internal hernias at multidetector CT includes detecting an intestinal closed loop, identifying the hernia orifice, and analyzing abnormal displacement of surrounding structures and key vessels around the hernia orifice and hernia sac. At each step, multidetector CT can depict pathognomonic findings. A saclike appearance suggests an intestinal closed loop in several types of internal hernias. Convergence, engorgement, and twisting of mesenteric vessels in the hernia orifice can be seen clearly at multidetector CT, especially with use of multiplanar reformations. For definitive diagnosis of an internal hernia, analysis of displacement of anatomic landmarks around the hernia orifice is particularly important, and thin-section images provide the required information. Detailed knowledge of the anatomy, etiology, and imaging landmarks of the various hernia types is also necessary. Familiarity with the appearances of internal hernias at multidetector CT allows accurate and specific preoperative diagnosis. (©)RSNA, 2015.

Are there reliable indicators predicting post-operative complications in acute appendicitis?

PURPOSE: To clarify the predictors of post-operative complications of pediatric acute appendicitis. METHODS: The medical records of 485 patients with acute appendicitis operated on between January 2006 and November 2014 were retrospectively reviewed. Age, sex, preoperative WBC, CRP, and appendix maximum short diameter on diagnostic imaging (AMSD) were compared retrospectively with the complications group (Group C) vs the non-complication group (Group NC) by Student's T test, Fisher exact test and Multivariate analysis. Regression analysis with p less than 0.01 was considered significant. We analyzed the most recent 314 laparoscopic appendectomy patients similarly. RESULTS: Complications were found in 29 of the 485 appendectomies (6.0%). Comparing Group C to Group NC, preoperative WBC (×10(3)/µl) 16.4 ± 5.6 vs 14.1 ± 4.1 (p < 0.01), CRP (mg/dl) 8.3 ± 7.1 vs 3.3 ± 4.6 (p < 0.01), AMSD (mm) was 12.1 ± 3.7 vs 9.9 ± 2.8 (p < 0.01). The CRP was significantly different by Multivariate analysis, but the WBC and AMSD wasn't. The results following laparoscopic appendicectomy data were identical. CONCLUSION: Preoperative WBC, CPR and AMSD all indicated an increased risk of complications. If WBC (/µl) >16,500, CRP >3.1 mg/dl and AMSD >11.4 mm, complications increased sixfold.

Noninvasive acoustic radiation force impulse (ARFI) elastography for assessing the severity of fibrosis in the post-operative patients with biliary atresia.

PURPOSE: Liver biopsy (LB) is still considered the "gold standard" for hepatological evaluation, but recently noninvasive methods have attempted to replace this invasive procedure. Recently, acoustic radiation force impulse (ARFI) imaging has been developed as a noninvasive modality to evaluate the stiffness of tissues. ARFI imaging theoretically measures liver stiffness of all the segments independently. The aim of this study was to determine whether ARFI elastography is a reliable method for predicting the severity of fibrosis in the post-operative patients with biliary atresia. METHODS: ARFI elastography was performed 21 times in eight patients with biliary atresia over the last 2 years. At the same time, we measured serum hyaluronic acid (H value), which is one of the serum elastic makers, to compare ARFI versus values in these patients. We obtained ARFI versus values as the median of S2 to S8 by three consecutive measurements acquired with a Siemens Acuson S2000 (Siemens Medical Systems, Germany). RESULTS: Histological evaluation of fibrosis is graded from F0 (normal) to F4. The normal H value is under 50 mg/dl. One patient had F0 (H value 29.2 mg/dl), four had F1 (H value 11.5-18.1 mg/dl), one had F3 (H value 61.3 mg/dl), two had F4 (H value 29.2, 112 mg/dl). One patient with F4 whose ARFI versus value (3.56 m/s) was the highest, needed liver transplantation and her liver was cirrhotic. CONCLUSION: These findings suggest that ARFI measurement may be a reliable method for predicting the severity of fibrosis after a Kasai operation.

The long-term prognosis of two-flap anoplasty for mucosal prolapse following anorectoplasty for anal atresia.

AIM: Mucosal prolapse is a common complication following anorectoplasty for anorectal malformation. The symptoms such as soiling, staining, and pain significantly reduce the patients' quality of life. Millard et al. (Plast Reconst Surg 69(3):399-411, 1982) reported the two-flap anoplasty that creates an anal canal using two perineal pedicle skin flaps to form a "deep anus". We have used this procedure for mucosal prolapse since 1990. This study evaluated the long-term benefits of this method. METHODS: From 1990 to 2009, 18 patients suffering mucosal prolapse following anorectoplasty for high imperforate anus were treated using a two-flap anoplasty (TFARP) or just mucosal resection (MR). For each procedure, the long-term clinical follow-up (maximum of 20 years) was assessed by review of medical records against the frequency of recurrence, and the recurrence of preoperative symptoms postoperatively. RESULTS: Of the 18 patients, 8 presented with simple mucosal prolapse, 4 with bleeding, 3 with staining, 2 with incontinence, and 1 with pain. TFARP was performed for 14 patients and MR for 6 patients. In the MR group, during the maximum of 15 years follow-up, two patients (33 %) suffered a recurrence or failed to improve their symptoms such as bleeding and/or soiling. In the TFARP group, during the maximum of 20 years follow-up, there were no recurrences and their preoperative symptoms resolved completely. Furthermore, two patients in the TFARP group gained normal sensation prior to defecation. CONCLUSION: The advantages of this procedure were no recurrences and complete resolution of preoperative symptoms. Moreover, it provides the possibility of gaining sensate defecation, possibly because the skin flap around the anus might help develop sensation.

Tracheoinnominate artery fistula after laryngotracheal separation: prevention and management.

AIM: Tracheoinnominate artery fistula (TIF) is an often fatal complication of laryngotracheal separation (LTS) for which there has been no systematic therapeutic strategy for prevention or management of TIF. The aim of this study was to establish such a strategy based on our clinical experience. MATERIALS AND METHODS: From 2000 to 2010, 14 patients received LTS. We reviewed these patients to develop a therapeutic approach to prevent or manage TIF. RESULTS: Three patients had major bleeding, and another 3 received preventive treatment before major bleeding. In the major bleeding group, 1 patient died of choking from uncontrollable hemorrhage, but the others were rescued by brachiocephalic trunk separation and/or endovascular embolization. At operation, median sternotomy with its high risk of mediastinitis was avoided. In the preventive treatment group, prophylactic brachiocephalic trunk separation was performed for 2 patients because their severe scoliosis narrowed the mediastinum, compressing the innominate artery on computed tomography. Another avoided major bleeding by converting the tracheostomy tube to a length-adjustable type. CONCLUSION: Tracheoinnominate artery fistula is a dramatic, often lethal complication. The strategic approach should be designed to prevent it and includes evaluation of the spinal deformity on computed tomography, brachiocephalic trunk separation at the same time as LTS, and recognizing the importance of "herald" or warning minor bleeds.

The usefulness of laryngotracheal separation in the treatment of severe motor and intellectual disabilities.

Intractable aspiration is a life-threatening medical problem in patients with severe motor and intellectual disabilities (SMID). Laryngotracheal separation (LTS) is a surgical procedure for the treatment of intractable aspiration which separates the upper respiratory tract from the digestive tract. We performed LTS for 14 patients with SMID to prevent intractable aspiration, performing two types of operation. The standard diversion procedure connected the upper trachea to the esophagus. The modified diversion includes closure of the proximal trachea and a high tracheostomy, avoiding a tracheoesophageal anastomosis. LTS was performed on 14 patients. Operations performed before the LTS included tracheostomy in four patients, fundoplication in six and gastrostomy in two. A standard diversion was performed in 11 patients and a modified diversion in 3. There were no operative complications. Eleven patients were safely transferred to home-care after their LTS. Twelve patients are still alive and two died some months after operation. One patient died from their primary disease and the other died a tracheo-innominate artery fistula (TIAF). We recently experienced a patient who was at high risk of developing a TIAF. LTS is an effective operation, preventing intractable aspiration in patients with severe motor and intellectual disabilities. The results are similar for the standard or modified diversion procedure with the procedure chosen being related to the initial tracheostomy site. The most serious complication is a lethal TIAF.

2-(4-Hydroxy-phenyl-sulfon-yl)phenol.

The title compound, C(12)H(10)O(4)S, is a phenolic color developer used for leuco colorants. The two benzene rings with substituent hydr-oxy groups are nearly perpendicular to each other [dihedral angle = 91.5 (1)°]. There are inter-molecular O-H⋯O hydrogen bonds between an OH group of one mol-ecule and a sulfonyl O atom of a neighboring mol-ecule. One mol-ecule is hydrogen bonded to four symmetry-related mol-ecules, forming a two-dimensional hydrogen-bond network.

Altered renin-angiotensin system gene expression causes renal hypoplasia in the rats with nitrofen-induced diaphragmatic hernia.

The association between renal hypoplasia and pulmonary hypoplasia in congenital diaphragmatic hernia (CDH) has become recently appreciated. However, the underlying mechanisms responsible for this association are still unknown. Renin-angiotensin system (RAS) plays an important role in renal and somatic growth, angiogenesis and reproduction. We hypothesized that abnormal expression of RAS components may be responsible for renal hypoplasia in CDH. We therefore designed this study to examine the gene expression of main components of RAS in the kidney of nitrofen-induced CDH in the rat. Pregnant rats were exposed to either olive oil or 100 mg of nitrofen on day 9.5 of gestation. Foetuses were recovered at term and divided into three groups: control (n=8), nitrofen without CDH (n=8) and CDH (n=8). Reverse transcription polymerase chain reaction was performed to evaluate the relative amount of angiotensinogen (AGT), angiotensin II type 1 receptor with 1a and 1b subtypes (AT(1a)R and AT(1b)R), angiotensin II type 2 receptor (AT(2)R), angiotensin-converting enzyme (ACE) and renin expression in the kidney. AT(1a)R, AT(1b)R, AT(2)R, AGT and renin levels were significantly decreased in the kidney of CDH rats compared with controls. We did not find a significant difference in ACE between CDH animals and controls. Our data show that the downregulation of RAS may play an important role in the pathogenesis of renal hypoplasia in the nitrofen-induced CDH.