Safety and efficacy of percutaneous electrocoagulation haemostasis in the treatment of grade IV haemorrhagic cystitis after allogeneic haematopoietic stem cell transplantation in children: a retrospective analysis.

BACKGROUND: To investigate the efficacy and safety of endoscopic electrocoagulation haemostasis via a percutaneous transhepatic approach for the treatment of grade IV haemorrhagic cystitis (HC) after allogeneic haematopoietic stem cell transplantation (allo-HSCT) in children. METHODS: The clinical data of 14 children with severe HC, who were admitted to Hebei Yanda Hospital between July 2017 and January 2020, were analysed retrospectively. There were nine males and five females, with an average age of 8.6 years (range: 3 to 13 years). After an average of 39.6 (7 to 96) days of conservative treatment in the hospital's haematology department, the bladders of all patients were filled with blood clots. A small 2-cm incision was made in the suprapubic area to enter the bladder and quickly clear the blood clots, and a percutaneous transhepatic approach to electrocoagulation and haemostasis was performed. RESULTS: In the 14 children, a total of 16 operations were performed, with an average operation time of 97.1 (31 to 150) min, an average blood clot of 128.1 (80 to 460) mL and an average intraoperative blood loss of 31.9 (20 to 50) mL. There were three cases of postoperative bladder spasm remission after conservative treatment. During the follow-up period of 1 to 31 months, one patient improved after one operation, 11 patients were cured after one operation, and two patients were cured after recurrent haemostasis by secondary electrocoagulation, four of whom died of postoperative non-surgical blood-related diseases and severe lung infections. CONCLUSION: Percutaneous electrocoagulation haemostasis can quickly remove blood clots in the bladders of children after allo-HSCT with grade IV HC. It is a safe and effective minimally invasive treatment.

Fabrication and characterization of highly sensitive flexible strain sensor based on biodegradable gelatin nanocomposites and double strain layered structures with crack for gesture recognition.

Flexible sensors have attracted extensive attention in the field of human-computer interaction. However, it is still a challenging task to realize accuracy gesture recognition with flexible sensor, which requires sensor not only have high sensitivity, but also have appropriate strain detection range. Here, a high gauge factor flexible sensor (gauge factor âˆ¼ 1296 under 12-20 % strain) based on crack structure is reported. The sensor is made of a biodegradable and stretchable gelatin composite combined with fabric bases, with good repeatability (6000 cycles) and a fast response (60 ms). Because of the double-layer structure, it has a suitable detection range (20 % strain). The sensor is manufactured by a screen-printing process, and it has been used to make data gloves and has realized 9 gestures recognition with machine learning algorithm (99.6 % accuracy). In general, this study offers a wearable gestures recognition scheme through the proposed sensor.

Multi-angle excited MOI and image processing strategies specified for detection of orthogonal weld defects.

This paper develops an integrative scheme combining new image acquisition, filtering and enhancement methods specified for orthogonal weld defect detection based on magneto-optical imaging (MOI) technique. For image acquisition, a controllable magnetic system enabling rotation of magnetic angles is invented to accurately collect MO images. Multiple images are acquired, yet few are utilized for further processing in the conventional method based on human subjective preferences, bearing chances that images containing defects are discarded. Therefore, we turn to an automated-filtering system to scrutinize MO images and filter effective images through Bhattacharyya coefficient screening method. This not only elevates efficiency and objectivity but also eliminates missed inspection. For image enhancement, normalization method is used to balance the image intensity, followed by image fusion and edge extraction by a two-dimensional gradient method. Our pre- and post-processing approaches significantly improve accuracy in defect recognition and precision in MO images.

Clinical characteristics of pulmonary alveolar proteinosis. / 肺泡蛋白沉积症的临床特征.

OBJECTIVES: Pulmonary alveolar proteinosis (PAP) is a rare disease with non-specific and various clinical manifestations, often leading to misdiagnosis. This study aims to raise the awareness of this disease via summarizing the clinical characteristics, diagnosis, and therapy of PAP. METHODS: We retrospectively analyzed clinical data of 25 hospitalized cases of PAP during 2008 and 2019 in the Department of Respiratory and Critical Care Medicine of the Second Xiangya Hospital of Central South University. RESULTS: Cough with unkown reason and dyspnea were common clinical manifastations of PAP. Five patients had a history of occupational inhalational exposure. Sixteen patients had typical image features including ground-glass opacification of alveolar spaces and thickening of the interlobular and intralobular septa, in typical shapes called crazy-paving and geographic pattern. Fourteen patients underwent pulmonary function tests, revealing a reduction in the diffusing capacity for carbon monoxide. The positive rate of transbronchial biopsy was 95%. Five patients received the whole lung lavage and the symptoms and imaging fcauters significantly relieved after five-years follow-up. CONCLUSIONS: PAP is characterized by radiographic pattern and pathology. Transbronchial lung biopsy is effective to make diagnosis of PAP. The whole lung lavage remains a efficient therapy.

Aberrant Hippocampal Network Connectivity Is Associated With Neurocognitive Dysfunction in Patients With Moderate and Severe Obstructive Sleep Apnea.

Objectives: This work aims to explore the changes of functional connectivity (FC) within the hippocampus network in patients with moderate and severe obstructive sleep apnea (OSA) and its correlation with neurocognitive dysfunction to explore the potential neurophysiological mechanism. Methods: A total of 32 treatment-naïve patients with moderate or severe OSA and 26 healthy controls (HCs), matched in age, gender, and education, underwent the evaluations of Epworth Sleep Scale, neurocognitive function, full-night polysomnography, and resting-state functional magnetic resonance imaging. The FC map of the hippocampus to other brain areas was compared among 15 OSA patients and 15 HCs with little head motion. Finally, the correlation between hippocampus FC strength and respiratory sleep parameters and neurocognitive assessments was analyzed. Results: Compared with HCs, the right hippocampus showed a significantly decreased FC with the bilateral insular lobe, right thalamus, and right anterior cingulate gyrus (ACG) and an increased FC with the right superior and middle temporal gyrus, left posterior cingulate gyrus, and left angular gyrus in the patients with OSA. The left hippocampus presented a significantly decreased FC with the left anterior cerebellum in patients with OSA. In addition, the aberrant right hippocampal FC with the right ACG was significantly correlated with disease severity and disrupted sleep architecture in the OSA group. Furthermore, after adjusting the related confounding factors, the FC strength between the right hippocampus, right insular lobe, and right thalamus was positively associated with the scores of Stroop Color-Word Test (SCWT) or Hopkins Verbal Learning Test-Revised (HVLT-R), while the FC between the right hippocampus and the right middle temporal gyrus was negatively correlated with the scores of HVLT-R. The right hippocampus FC with right superior temporal gyrus, left angular gyrus, and ACG were all negatively related to the scores of the symbol coding test (r = -0.642, p = 0.045; r = -0.638, p = 0.047; r = -0.753, p = 0.012), respectively. The FC between the left hippocampal and the left anterior cerebellar lobe showed a positive relationship with the scores of HVLT-R (r = 0.757, p = 0.011) and CPT-3D (r = -0.801, p = 0.005). Conclusion: The hippocampus presented abnormal FC with the cerebral and cerebellar regions extensively in OSA, and the correlation between abnormal hippocampal network FC and neurocognitive dysfunction in OSA suggests a promising insight to explore the potential biomarker and pathophysiologic mechanism of neurocognitive dysfunction of OSA.

Reduced regional homogeneity and neurocognitive impairment in patients with moderate-to-severe obstructive sleep apnea.

BACKGROUND: Neurocognitive dysfunction and abnormal regional homogeneity (ReHo) have been reported in patients with obstructive sleep apnea (OSA). However, little is known about whether brain functional alteration could be used to differentiate from healthy controls (HCs) and its correlation with neurocognitive impairment. METHODS: Thirty-three treatment-naive patients with moderate-to-severe OSA and 22 HCs with matched age, sex and education underwent the evaluation of Epworth sleepiness scale, neurocognitive function, full night polysomnography and resting-state functional magnetic resonance imaging scan. ReHo, support vector machine, and correlation with neurocognitive function were administrated to analyze the data. RESULTS: Compared with HCs, patients with OSA showed decreased ReHo in the bilateral superior frontal gyrus (FG), bilateral superior medial prefrontal cortex (PFC)/right supplementary motor area (SMA), left middle FG, and right precentral/postcentral gyrus. Negative correlations were observed between the ReHo values in the left superior FG/middle FG and apnea hypopnea index, oxygen desaturation index in the OSA group. The scores of Stroop word test, Stroop color-word test, symbol coding test were all negatively correlated with the ReHo values in the right precentral gyrus/postcentral gyrus in patients. Scores of the animal naming fluency test were positively correlated with the ReHo values in the left superior FG/middle FG in patients. Moreover, support vector machine analysis showed the ReHo values in the left superior FG/middle FG or bilateral superior medial PFC/right SMA both could discriminate patients from HCs with good accuracies, sensitivities, and specificities (85.45%, 87.88%, 81.82% and 81.82%, 84.85%, 77.27%, respectively). CONCLUSION: Dysfunction in the frontal lobe is a potentially pivotal neuro-pathophysiological mechanism of neurocognitive impairment in patients with moderate-to-severe OSA. And significantly lower ReHo values in the left superior FG/middle FG and/or superior medial PFC/SMA are promising imaging biomarkers to discriminate moderate-to-severe patients with OSA from HCs.

Myeloid neoplasm with the abnormality of PDGFRB gene secondary interstitial pneumonia: A case report. / 伴PDGFRB重排髓系肿瘤继发性间质性肺炎1例.

We report a case of myeloid neoplasm with the abnormality of PDGFRB gene secondary interstitial pneumonia. A 66-year-old male complained dry cough and shortness of breath after regular activities. Two years ago, high-resolution computerized tomography (HRCT) showed interstitial lung diseases, and no clear etiology was found. At that time, the diagnosis was idiopathic pulmonary fibrosis. In the period of hospitalization, the interstitial lung disease was significantly advanced, and the peripheral blood eosinophil was significantly increased. ETV6-PDGFRB fusion genes was positive detected by FISH. Myeloid neoplasm with the abnormality of PDGFRB gene was diagnosed. The patient was treated with the tyrosine kinase inhibitor-imatinib mesylate which is the first choice for this disease currently.Myeloid neoplasm with the abnormality of PDGFRB gene is a rare hematologic tumor characterized by myeloid dysplasia, eosinophilia, and PDGFR gene rearrangement. Most of this tumor occurs in male, often with splenomegaly, and a few have damages in liver, skin, heart, and other organ tissues.

A case report. / æˆäººæœ—æ ¼æ±‰æ–¯ç»†èƒžç»„ç»‡ç»†èƒžå¢žç”Ÿç—‡ç´¯åŠè‚ºã€åž‚ä½“ã€ç”²çŠ¶è ºã€è‚ã€æ·‹å·´ç»“ã€çš®è‚¤1例.

Langerhans cell histiocytosis (LCH) is a rare and unexplained disease that can involve in any organ or system in the body and displays a variety of clinical manifestations. A 31-year-old man, who had a more than 10-year smoke history, initially presented dry cough, polydipsia and diuresis, with recurrent spontaneous pneumothorax. Pulmonary high-resolution computed tomography showed diffuse cystic and nodular lesions. Langerhans cell histiocytosis was confirmed by a transbronchial cryobiopsy. The disease is involved in the lung, pituitary, thyroid, liver, lymph node, and skin. Glucocorticoid or systemic chemotherapy is commonly used in the treatment for this disease. BRAF gene mutation inhibitor is a new direction for the treatment.

Clinical analysis for 12 patients with thoracic Castleman's disease. / 累及胸部的Castlemanç— 12例临床分析.

OBJECTIVES: To explore the clinical characteristics and differential diagnosis for patients with thoracic Castleman's disease, thus to improve doctor's cognition and therapeutic level for the disease. METHODS: The clinical data of 12 patients with thoracic Castleman's disease were analyzed retrospectively, including clinical features, laboratory examination, imaging finding, pathological examination, treatment outcome, and prognosis. RESULTS: All 12 patients were diagnosed by mass or lymph code biopsy. Seven patients with unicentric Castleman's disease were asymptomatic or oligosymptomatic. Of them, the lung imaging features were mostly solitary and well-defined pulmonary hilar soft tissue mass and infrequently invasive mass. Pathologically, 7 patients were all hyaline vascular type. Six patients underwent surgery of complete mass resection, and 1 received chemotherapy because the nidus could not be resected completely. The prognosis of 7 patients was good. The clinical manifestations of 5 patients with multicentric Castleman's disease were diverse with negative prognosis. Their radiological features mainly showed multiple mediastinal/hilum lymph node enlargement, multiple serous cavity effusion, and hepatosplenomegaly, along with multifocal lymphadenopathy. Pathological features of 3 patients' mass were hyaline vascular type, 1 was plasma cell type, and 1 was mixed type. Three patients received comprehensive treatment on the basis of chemotherapy and another 2 patients did not receive any therapy. CONCLUSIONS: Unicentric Castleman's disease involving the chest is mainly manifested as mediastinal or hilar soft tissue mass with single clinical manifestation, while multicentric Castleman's disease involving the chest is mainly manifested as diffuse mediastinal/hilar lymph node enlargement, multifocal lymphadenopathy and multiple clinical manifestation. For patients with mediastinal/hilum mass or with multifocal lymphadenopathy, Castleman's disease should be considered the differential diagnosis. And early mass or lymph code biopsy is of great significance for diagnosis and treatment of Castleman's disease.

A case of eosinophilic granulomatosis with polyangiitis complicated with a similar condition to IgG4 related lung disease.

BACKGROUND: Atypical manifestations, such as elevated serum immunoglobulin-G4 (IgG4) and extra-pulmonary IgG4 positive plasmacyte infiltration, have been described in patients with eosinophilic granulomatosis with polyangiitis (EGPA), such complicated situation might not be readily differentiated from IgG4-related disease. CASE PRESENTATION: Here, we report an interesting and rare case of EGPA in a 41 year-old male with negative anti-neutrophil cytoplasmic antibodies (ANCAs), which showed abundant pulmonary IgG4-positive plasma cells infiltration and markedly elevated serum IgG4 levels without extra-pulmonary lesions of IgG4-related disease. The clinical characteristics hesitated us whether the diagnosis as EGPA overlapping with IgG4-related lung disease should be concluded because of the absence of storiform fibrosis and obliterative phlebitis with lymphoplasmacytic infiltration. The patient's systemic symptoms, pulmonary lesions, blood eosinophils count / percentage, and serum IgG4 levels were significantly improved with immunosuppressive therapy. CONCLUSIONS: We consider that the overlapping pathogenesis exists in the disease course of EGPA and IgG4-related disease, which may lead to interaction during the course of the diseases. And the utilization of diagnostic criteria for IgG4-related lung disease with the careful differentiation is needed in such cases.

[Progress in the evaluation of cognitive function in with obstructive sleep apnea by resting functional magnetic resonance].

Obstructive sleep apnea is often accompanied by functional changes in attention, alertness, long-term vision and language memory, visual space/structural competence, and executive function due to long-term intermittent hypoxia, high blood carbonate, and sleep structure disorders. In recent years, some scholars have found that the changes of brain structure, metabolism and function revealed by static functional magnetic resonance imaging (MRI) are closely related to the changes of cognitive function reflected in the cognitive function evaluation scale. In recent years, static functional magnetic resonance, especially voxel-based morphometry, diffusion tensor imaging, magnetic resonance spectroscopy and functional magnetic resonance imaging have confirmed significant changes in brain structure, metabolism and function in OSA patients, and the changes are closely related to the changes of cognitive function reflected in the cognitive function evaluation scale. It is great significance to study the mechanism of cognitive function change in OSA patients, and helpful to the early diagnosis and treatment as well as the evaluation of clinical efficacy.

Efficacy of sirolimus for the prevention of recurrent pneumothorax in patients with lymphangioleiomyomatosis: a case series.

Pneumothorax is one of the most common symptoms in patients with lymphangioleiomyomatosis (LAM). However, current management strategies for patients with LAM who present with recurrent pneumothorax remain inadequate. Here, we describe the successful prevention of recurrent pneumothorax by sirolimus treatment in five women with LAM. Before sirolimus treatment, all patients had received supplemental oxygen support, repeated chest tube drainage, or surgeries for management of the recurrent pneumothorax. Sirolimus treatment was initiated when the pneumothorax was completely resolved, and no patient developed pneumothorax during treatment. Moreover, they exhibited a significantly improved subjective quality of life, increased exercise capacity, and mild adverse effects such as mucositis, irregular menstruation, and delayed wound healing. On discontinuation of sirolimus or in the event that the plasma sirolimus level was markedly low, pneumothorax tended to relapse. The findings from these cases provide valuable insights that will aid in the improvement of treatment strategies for patients with LAM and recurrent pneumothorax.

[Obstructive sleep apnea hypopnea syndrome and alveolar hypoventilation syndrome in motor neuron disease: A case report and literature review].

OBJECTIVE: To investigate the clinical characteristics of a patient with motor neuron disease, which caused sleep-disordered breathing (SDB) and alveolar hypoventilation syndrome, and to improve the diagnosis rate for this disease.
 Methods: Retrospectively analyze the diagnosis and treatment process for a 52 year-old male patient, who was accepted by the Second Xiangya Hospital, Central South University because of dyspnea, shortness of breath and malaise for 4 months, and eventually was diagnosed as motor neuron disease associated with obstructive sleep apnea hypopnea syndrome and alveolar hypoventilation syndrome. In addition, we searched CNKI, Wanfang and PubMed databases to review relevant literature with keywords (motor neuron disease or amyotrophic lateral sclerosis or progressive bulbar palsy or progressive muscular atrophy or primary lateral sclerosis) AND (sleep apnea or sleep disordered breathing) from January 1990 to May 2017.
 Results: The major clinical manifestation of motor neuron disease included impaired upper and lower motor neuron displayed with proximal muscle weakness, muscle tremor, amyotrophy, bulbar symptoms and pyramidal sign. It was a chronic, progressive disease with worse prognosis, low survival and difficult in diagnosis. Electroneuromyography was a vital way for diagnosis. Furthermore, sleep disordered breathing was common in patients with motor neuron disease, which was featured as decreased rapid eye movement sleep, increased awaking time, apnea and hypopnea. The main mechanism for sleep disordered breathing in motor neuron disease might be due to the disturbed central nervous system and paralysis of diaphragm and respiratory muscle. Moreover, the patient suffered from restrictive ventilatory dysfunction, alveolar hypoventilation and subsequent partial pressure of carbon dioxide and hypoxemia. Therefore, respiratory failure was the most frequent cause of death for patients with motor neuron disease. Non-invasive positive pressure ventilation was suggested to apply to such patients, whose forced vital capability was less than 75 percent of predicted value.
 Conclusion: Sleep disordered breathing is common in patients with motor neuron disease. Hence, polysomnography is suggested as a routine examination to confirm the potential complications and give timely therapy. Treatment with non-invasive positive pressure ventilation is important for patients to improve life quality, survival rate and prognosis.

Dysfunction of Nrf2-ARE Signaling Pathway: Potential Pathogenesis in the Development of Neurocognitive Impairment in Patients with Moderate to Severe Obstructive Sleep Apnea-Hypopnea Syndrome.

The present study investigated the nuclear factor erythroid 2-related factor 2- (Nrf2-) antioxidant response element (ARE) signaling pathway in patients with moderate to severe obstructive sleep apnea-hypopnea syndrome (OSAHS). Their correlation with neurocognitive impairment metrics was investigated to explore potential pathogenesis in OSAHS. Forty-eight patients with OSAHS and 28 controls underwent testing with the Epworth Sleep Scale (ESS), MATRICS Consensus Cognitive Battery (MCCB), Stroop Color and Word Test, polysomnography (PSG), and measurements of the concentration of plasma superoxide dismutase (SOD) and thioredoxin (Trx). Further, 20 pairs of matched patients with OSAHS and controls were selected for measurement of the expression (protein and mRNA) of Nrf2 and of its downstream antioxidase, heme oxygenase-1 (HO-1), in peripheral mononuclear cells (PBMCs). Finally, correlations between neurocognitive impairment and the above metrics were analyzed. Expression of Nrf2 and HO-1 mRNA and protein in the PBMCs, as well as plasma SOD and Trx levels, were significantly reduced in patients with OSAHS. After adjusting for education, sex, age, and smoking index, the expression of Nrf2-ARE signaling pathway proteins (or mRNA) was closely correlated with sleep respiratory parameters. An inverse relationship was demonstrated between the expression of nuclear Nrf2 in PBMCs, concentration of plasma SOD and Trx, and apnea-hypopnea index (AHI) in patients with OSAHS. Trx, nuclear Nrf2 protein, and HO-1 protein were also negatively correlated with the percent of time that SaO2 was less than 90% (TSat90). Total Nrf2 protein level was positively correlated with AHI and TSat90 and negatively correlated with minimum SaO2 (LSaO2), while nuclear Nrf2 protein and HO-1 protein were positively correlated with LSaO2. Moreover, significant positive correlations were found between maze scores and expression of nuclear Nrf2 protein, HO-1 protein, and SOD and Trx levels. Furthermore, inverse relationships between total Nrf2 protein in PBMCs and HVLT-R and maze scores were found. Multiple linear regression showed plasma Trx concentration as a potential predictor of maze and BVMT-R scores. In conclusion, the expression of Nrf2-ARE molecules and related antioxidases is significantly decreased in patients with OSAHS and is correlated with neurocognitive dysfunction. The Nrf2-ARE signaling pathway may play a crucial role in neurocognitive impairment in patients with moderate to severe OSAHS. Further studies are needed to explore the exact mechanisms and potential treatment interventions.

[A case of severe obstructive sleep apnea hypopnea syndrome with urinary and anal incontinence].

A case of a young male patient, who came to the Second Xiangya Hospital, Central South University because of snoring for 10 years and nocturnal gatism for half month, was analyzed retrospectively. He was diagnosed as obstructive sleep apnea hypopnea syndrome (OSAHS) finally. The patient had been diagnosed and treated as stroke in the local hospital, while urinary and anal incontinence were not relieved. It was a dilemma for him to be properly diagnosed and treated. Polysomnography in our hospital revealed apnea hypopnea index (AHI) at 44.7 events/h, oxygen desaturation index (ODI) at 70.8 events/h and the longest apnea time at 185 seconds while the lowest blood oxygen saturation reduced to 31%. In addition, 413 events of apnea accounted for 61.2% of the sleep time and the minimal heart rate was 23 times/min. The patient was diagnosed as severe OSAHS with hypoxia metabolic brain disease, moderate pulmonary arterial hypertension, secondary polycythemia and obesity hypoventilation syndrome finally. He received the treatment of positive airway pressure non-invasive ventilator with an average pressure at 11.7 cmH2O with reduced AHI and increased blood oxygen saturation. The urinary and anal incontinence disappeared during the first night of treatment and it has been totally resolved so far. We considered that gatism was secondary to OSAHS with severe hypoxia resulted from attenuated regulation of primary defecation in the night. Physicians should pay attention to OSAHS when accepting obese patients with nocturnal incontinence, obvious daytime sleepiness and night snoring. Urinary and anal incontinence could be completely disappeared under therapy of positive airway pressure.