[Safety of modified T-piece resuscitator versus nasal cannula oxygen in electronic bronchoscopy for infants: a prospective randomized controlled study]. / 改良Tç»„åˆå¤è‹å™¨ä¸Žé¼»å¯¼ç®¡å¸æ°§ç”¨äºŽå©´å„¿ç”µå­æ”¯æ°”ç®¡é•œæ£€æŸ¥çš„å®‰å ¨æ€§æ¯”è¾ƒï¼šä¸€é¡¹å‰çž»æ€§éšæœºå¯¹ç §ç ”ç©¶.

OBJECTIVES: To optimize the oxygen therapy regimens for infants with pulmonary diseases during bronchoscopy. METHODS: A prospective randomized, controlled, and single-center clinical trial was conducted on 42 infants who underwent electronic bronchoscopy from July 2019 to July 2021. These infants were divided into a nasal cannula (NC) group and a modified T-piece resuscitator (TPR) group using a random number table. The lowest intraoperative blood oxygen saturation was recorded as the primary outcome, and intraoperative heart rate and respiratory results were recorded as the secondary outcomes. RESULTS: Compared with the NC group, the modified TPR group had a significantly higher level of minimum oxygen saturation during surgery and a significantly lower incidence rate of hypoxemia (P<0.05). In the modified TPR group, there were 6 infants with mild hypoxemia, 2 with moderate hypoxemia, and 1 with severe hypoxemia, while in the NC group, there were 3 infants with mild hypoxemia, 5 with moderate hypoxemia, and 9 with severe hypoxemia (P<0.05). The modified TPR group had a significantly lower incidence rate of intraoperative respiratory rhythm abnormalities than the NC group (P<0.05), but there was no significant difference in the incidence rate of arrhythmias between the two groups (P>0.05). CONCLUSIONS: Modified TPR can significantly reduce the risk of hypoxemia in infants with pulmonary diseases during electronic bronchoscopy, and TPR significantly decreases the severity of hypoxemia and the incidence of respiratory rhythm abnormalities compared with traditional NC.

MicroRNA profile of circulating CD4+ T cells in aged patients with atherosclerosis obliterans.

BACKGROUND: To evaluate the specificity of the expression patterns of microRNAs (miRNAs) in circulating CD4+ T cells in aged patients with atherosclerosis obliterans (ASO). METHODS: A comprehensive miRNA expression study was conducted using a miRNA microarray of CD4+ T cells isolated from peripheral blood mononuclear cells (PBMCs) of 33 patients with ASO and 24 healthy donors. A t test was used for statistical analysis, and the average linkage method was used for hierarchical clustering. The results were validated by qRT-PCR. Putative targeted pathways associated with validated miRNAs were predicted with the online software DIANA miRPath. RESULTS: We identified 44 miRNAs based on a cutoff value of a 1.3-fold change in expression between the two groups, with 18 miRNAs showing a false discovery rate (FDR) p value < 0.05. The qRT-PCR analysis validated differences in 12 miRNAs, and 6 miRNAs were proven to be differentially expressed among three age groups (age: 35-55 years; 56-75 years; 76-95 years): the miRNAs miR-21 (p: 0.0008; 0.0009; 0.0022), miR-29b (p: 0.453; < 0.0001; < 0.0001), and miR-374b (p: < 0.0001; < 0.0001; 0.2493) showed upregulated expression in patients with ASO, while miR-142-3p (p: < 0.0001; < 0.0001; < 0.0001), miR-142-5p (p: < 0.0001; < 0.0001; < 0.0001), and miR-150 (p: < 0.0001; < 0.0001; 0.0001) showed downregulated expression in patients with ASO. The validated miRNAs participated in CD4+ T cell activation, proliferation, and migration pathways. CONCLUSIONS: Circulating CD4+ T cells in aged patients with ASO may show a distinct molecular signature. This is the first time that a distinctive, validated miRNA profile from circulating CD4+ T cells in atherosclerosis has been presented. This miRNA signature may be used to help elucidate the underlying mechanism of atherosclerosis. Further clinical studies and in-depth reports will contribute to identifying predictive and therapeutic targets in these patients with atherosclerosis.

Outcomes of Popliteal Stent-Graft Placement at the Artery Hinge Point for Popliteal Artery Aneurysm.

BACKGROUND: To assess whether stent-grafts crossing the hinge point (HP) in the popliteal artery are associated with increased complications and decreased patency rates, after endovascular treatment of the popliteal artery aneurysm. METHODS: This was a single-center, case-control study. Patients were allocated to either the HP group (subjects with stent-grafts crossing the HP) or the control group (subjects with stent-grafts above and/or below the HP) based on stent-graft location in the femoropopliteal artery. HP was defined as the main curve in the popliteal artery in the most acute angle toward the femur that appeared during knee flexion, which was identified by reviewing postoperative angiograms. Independent, blinded reviews were performed for all imaging data. Graft evaluation by CTA or duplex ultrasound was performed at 1, 3, 6, and 12 months and annually thereafter. Outcomes measured included: stent-graft patency, stent-graft fracture, other stent-related complications, and major adverse events, including reintervention, death, amputation, stroke, and myocardial infarction. RESULTS: A total of 44 limbs treated with placement of heparin-bonded Viabahn endoprostheses were included in this study. Twenty and twenty-four patients were allocated to the HP group and the control group, respectively. Primary patency rates of the HP group at 1, 2, 3, and 5 years were 84.1 ± 8.4%, 84.1 ± 8.4%, 84.1 ± 8.4%, and 72.1 ± 13.3%, respectively. The primary patency rates of the control group were 87.0 ± 7.0%, 82.4 ± 8.0%, 82.4 ± 8.0%, and 82.4 ± 8.0%, respectively. There was no significant difference between the 2 groups (P = 0.81). No reintervention was performed in the control group. In the HP group, 5 limbs (25.0%) developed endoleak, 3 (15.0%) developed thrombosis, and 1 (5.0%) developed a stent fracture followed by thrombosis. Thrombosis occurred in 2 limbs (8.3%) of the control group, and stent-graft migration was observed in another 2 cases (8.3%). Neither group demonstrated stent-graft infection or acute popliteal artery embolism. Overall, incidence of stent-related complications were significantly higher in the HP group (P= 0.04). Event-free survival rates of the HP group at 1, 2, 3, and 5 years were 75.0 ± 9.7%, 69.6 ± 10.4%, 61.9 ± 11.8%, and 29.0 ± 12.8%, respectively. Corresponding rates in the control group were 79.2 ± 8.3%, 79.2 ± 8.3%, 79.2 ± 8.3%, and 79.2 ± 8.3%, respectively. The difference was not statistically significant between the 2 groups (P = 0.20) CONCLUSIONS: crossing the HP with femoropopliteal artery stent-grafts increased the risk of stent-related complications and reinterventions but did not decrease stent patency or event-free survival.

Comparison of the Bifurcated Graft Reconstruction and Aortic Stump Closure in Open Surgical Conversion After Endovascular Aneurysm Repair.

BACKGROUND: The optimal management of the aortic stump in open surgical conversion (OSC) after Abdominal aortic aneurysm (AAA) endovascular aneurysm repair (EVAR) is debated. Therefore, we aimed to compare the efficacies and safety between the bifurcated prosthetic vascular graft in situ stump reconstruction (p-graft ISSR) and aortic stump closure (ASC) in OSC. METHODS: We analyzed 973 elective AAA patients admitted from January 01, 2001 to December 31, 2020, at the First Affiliated Hospital of Sun Yat-sen University. We conducted a statistical analysis of the clinical characteristics, procedural data, as well as outcomes and technique considerations of aortic stump management in OSC patients. RESULTS: A total of 24 male patients had OSC after EVAR. The rate of stent graft infection was 54.17% before OSC. Eleven patients underwent ASC, and 13 patients were treated with p-graft ISSR. The major complication after OSC was aortic stump bleeding (total incidence was 37.50%) (1 patient with a periaortic hematoma and 8 patients with a stump blowout). The total incidences of stump blowout between the patients with ASC and those with p-graft ISSR were significantly different (45.45% vs. 23.08%, P < 0.05). The total perioperative mortality was 25.00% (6 patients with stump blowouts). The perioperative survival rates between these 2 aortic stump management approaches were 72.72% and 76.92% (ASC vs. p-graft ISSR, P < 0.05). In total, 18 patients were followed up (3-180 months). There were 3 aorta-related deaths during the late follow-up period (including both of the 2 stump-blowout-related deaths just treated with ASC). CONCLUSIONS: If the condition of the aorta and peri-aortic tissue are suitable for a prosthetic graft bypass, the p-graft ISSR is highly recommended for OSC patients after EVAR.

[Analysis of CASR gene variant in a child with idiopathic epilepsy and autism].

OBJECTIVE: To explore the genetic basis for a child featuring idiopathic epilepsy and autism. METHODS: Peripheral blood samples of the child and his parents were collected with informed consent for the extraction of genome DNA. Whole exome sequencing was carried out for the family trio. Candidate variants were verified by Sanger sequencing and bioinformatic analysis. RESULTS: The proband was found to harbor a heterozygous nonsense c.3025C>T (p.Arg1009Ter) variant in exon 7 of the CASR gene exon 7, which may produce a truncated protein. Based on the guidelines of the American College of Medical Genetics and Genomics, the variant was predicted to be deleterious and classified as possibly pathogenic (PVS1+PM2). CONCLUSION: The c.3025C>T (p.Arg1009Ter) variant of the CASR gene probably underlay the disease in this child.

Parallel Covered Stents Technique in the Treatment of Abdominal Aortic Diseases.

PURPOSE: To investigate the safety and efficacy of the parallel covered stents technique in the treatment of anatomically challenging aortic aneurysms, pseudoaneurysms, and dissections. MATERIALS AND METHODS: Data were retrospectively collected from 16 patients with abdominal aortic diseases who were treated with parallel covered stents (Gore Excluder, n = 14; Medtronic Endurant, n = 2) between January 2016 and July 2018. Patients were treated with this technique if they were unsuitable for either open repair or standard endovascular aortic repair with bifurcated stents. Such unfavorable anatomy included narrow aortic necks (≤18 mm), small vascular access (occluded or ≤6.0 mm), or compressed aortic lumens (≤18 mm). All patients were male, with a mean age of 64.7 ± 13.3 years. For true aneurysms (n = 4) and pseudoaneurysms (n = 4), the mean diameter and length of the proximal necks were 17.5 ± 2.6 mm (range, 14-21 mm) and 51.0 ± 12.5 mm (range, 39-75 mm), respectively. The minimal diameter of true lumen in cases with aortic dissection and penetrating ulcers (n = 8) was 14.8 ± 3.1 mm. Small or occluded femoral access was found in 3 patients. RESULTS: Technical success was 100%. Minor type I endoleaks, which were seen on completion angiography in 5 patients, had all resolved within 3 months. There were no perioperative deaths. Postoperative complications included supraventricular tachycardia in 1 patient and pneumonia combined with heart failure in 1 patient. Patency of all stents was observed at a mean follow-up of 21.8 ± 10.1 months. CONCLUSIONS: The parallel covered stents technique appears to offer a feasible solution for abdominal aortic diseases with unfavorable anatomy. Long-term follow-up is needed to further evaluate the safety and efficacy of this technique.

Failures and Lessons in the Endovascular Treatment of Symptomatic Isolated Dissection of the Superior Mesenteric Artery.

BACKGROUND: To discuss failures and lessons in the endovascular treatment of symptomatic isolated dissection of the superior mesenteric artery (SIDSMA). METHODS: Data from 33 patients with SIDSMA treated between July 2007 and September 2013 were retrospectively collected. The technical failures in and lessons from endovascular management were analyzed in terms of causes and prophylaxis. RESULTS: Eighteen patients were successfully treated medically, 13 underwent stent placement, 1 underwent a hybrid procedure, and 1 had open fenestration. Full follow-ups (37 ± 21 months) were achieved in 28 patients. Failures to cannulate the true lumen occurred in 8 patients. Among these patients, femoral and brachial approaches were taken in 6 and 2 patients, respectively, and a 2-sided Fisher's exact test revealed no significant differences (P = 0.204). Among the 6 femoral failures, the true lumina were cannulated after conversion to the brachial approach in 3 cases. The perfusion of the distal SMA was not improved until the second stent was distally placed to cover the entire expanded false lumen in 1 case. Numerous branches originating from the false lumen were overlooked in 1 patient and were apparently compromised after stenting. Consequently, the patient died of intestinal necrosis. In a patient with a huge dissecting aneurysm, a stent was misplaced across the false lumen. Fortunately, a remarkable aneurysmal thrombosis formed at 3 months. In a patient who received a hybrid procedure, the stent was occluded at 2 weeks, most likely because the thrombus protruded into the stent. CONCLUSIONS: Difficulty in cannulating the true lumen is not uncommon in the endovascular treatment of SIDSMA, and the selection of the arterial approach would benefit from being based on the morphology of the SMA arch. The lengths and branches of the involvement of the false lumen should be evaluated beforehand. Covered stents would be a reasonable option for lumina that contain thrombi.

[Endovascular repair for isolated iliac artery aneurysm].

OBJECTIVE: To evaluate the short and middle-term efficacies of endovascular repair for isolated iliac artery aneurysms (IIAAs). METHODS: Retrospective analyses were performed for the clinical and follow-up data of 27 patients undergoing endovascular repair at Department of Vascular Surgery, Affiliated Zhongshan Hospital, Fudan University from January 2008 to December 2012. For asymptomatic aneurysms, repair criterion was a diameter over 3 cm while under 3 cm for symptomatic aneurysms. RESULTS: There were 19 asymptomatic and 8 symptomatic cases. And 25 selective and 2 emergent operations were performed. The interventions included endovascular stent grafts (n = 17), coil embolization (n = 1), both stent grafts and coils (n = 7) and hybrid operations (n = 2). Technical success was achieved in all cases. No delayed healing or infection of inguinal access sites occurred. In-stent thrombosis could be observed in 2 cases. Buttock claudication was observed in one case. Sexual dysfunction occurred in three cases. Endoleaks were confirmed by intraoperative digital subtraction angiography in three cases. There was no occurrence of colorectal ischemia or postoperative aneurysmal rupture. Patients were followed up for a mean period of 33 ± 18 months. One patient died of recurrent renal cell carcinoma. CONCLUSION: Endovascular repair of isolated iliac artery aneurysms provides good short and middle-term patency. It may become a first-choice for treating isolated iliac artery aneurysms in the future.

A rare case of fetal retroperitoneal solid mature teratoma.

article's main point: This article retrospectively analyzes clinical data from a rare case of fetal retroperitoneal solid, mature teratoma, aiming to provide insights into diagnosing and treating fetal teratomas. This case of fetal retroperitoneal teratoma provides the following insights into diagnosis and treatment: 1) Due to the special nature of the retroperitoneal space, retroperitoneal tumours grow hidden, especially in fetal retroperitoneal tumours that are even more difficult to detect. Prenatal ultrasound screening is of great value for the diagnosis of this disease. 2) Although ultrasound can determine the location and blood flow of the tumour and monitor changes in its size and composition, there is a certain degree of misdiagnosis due to fetal position, clinical experience, and imaging resolution. When necessary, fetal MRI can provide further evidence for prenatal diagnosis. 3) Although fetal retroperitoneal teratoma is rare, a few tumours grow rapidly and have the potential for malignant transformation. When a solid cystic mass lesion in the retroperitoneal space is found during the fetal period, this disease should be considered as one of the differential diagnoses and distinguished from fetal renal tumours, adrenal tumours, pancreatic cysts, meconium peritonitis, parasitic fetus, and lymphangioma, among others. 4) Based on the situation of the pregnant woman, fetus, and tumour, the time and method of termination of pregnancy should be decided. After birth, the timing and manner of surgery and postoperative follow-up should be determined by neonatology and pediatric surgery.

A rare case of aplasia cutis congenita.

In this study, we retrospectively analyzed the clinical data of a newborn with aplasia cutis congenita (ACC) to provide insights for diagnosing and treating the disease. It is believed that ACC with an intact skull and a skin defect diameter of less than 2 cm can be treated conservatively. The main strategies include local disinfection and regular dressing changes to promote epithelial regeneration. The lesion can heal over weeks or months through epithelization adjacent to the defect tissue, resulting in a healed contracture scar with a smooth, hairless surface that can be surgically removed later. For children with large scalp defects or skull defects, skin transplantation, free flap, and cranioplasty can be performed to repair the wound and restore the tissue structure. It is worth mentioning that although this child had a scalp defect larger than 2 cm, conservative treatment still had a significant effect. This suggests that conservative treatment can be considered as the first choice for ACC neonates without skull defects, and surgical treatment can be considered when necessary.

The role of necroptosis in common respiratory diseases in children.

Studies have shown that necroptosis (NEC) relies on a unique gene-regulated molecular pathway to cause cell death. With the development of knockout mouse models and specific molecular inhibitors of necrotic proteins, this cell death pathway has been considered one of the important causes of the pathogenesis of human diseases. In this review, we explored the possible roles and mechanisms of NEC in common respiratory diseases in children, such as acute lung injury, acute respiratory distress syndrome, pulmonary infection, childhood asthma, pulmonary hypertension, etc., in order to provide new ideas for the prevention and treatment of such diseases.

Clinical and genetic analysis of nonketotic hyperglycinemia: A case report.

BACKGROUND: Nonketotic hyperglycinemia (NKH) is a rare autosomal recessive genetic disorder of abnormal glycine metabolism caused by insufficient activity of the glycine cleavage enzyme system. Glycine is believed to function mainly as an inhibitory neurotransmitter, but it can also act as a co-agonist of the N-methyl-D-aspartate (NMDA) receptor. The accumulation of a large amount of glycine in the brain leads to neuronal and axonal injury via overactivation of NMDA receptors located in the hippocampus, cerebral cortex, olfactory bulb, and cerebellum and to stimulation of the inhibitory function of glycine receptors located in the spinal cord and brain stem, resulting in central apnea, hiccups, and hypotonia in the early stage of the disease. CASE SUMMARY: The child described in this report had typical clinical manifestations of NKH, such as hiccups, disturbance of consciousness, hypotonia, and convulsions, within the first week after birth. Whole-exome genetic testing revealed that the child had a compound heterozygous mutation, namely, c.395C>A (p.S132X) and c.2182G>A (p.G728R), in the GLDC gene, and he was diagnosed with NKH. For treatment, we administered an oral levetiracetam solution and added topiramate and prednisone for epilepsy control, but the epilepsy remained uncontrollable. Ketogenic diet therapy was started at 6 mo of age, his seizures were significantly reduced, and there were no obvious adverse reactions during ketogenic treatment. Furthermore, we found that with the development of the disease, high levels of serum glycine decreased or even disappeared without intervention, and as the disease progressed, the corpus callosum became dysplastic. CONCLUSION: This case shows that plasma glycine levels cannot be used to evaluate the prognosis of NKH, that the development of the corpus callosum can be affected by NKH, and that a ketogenic diet may be effective for seizure control in NKH patients.

Deficiency of endothelial FGFR1 alleviates hyperoxia-induced bronchopulmonary dysplasia in neonatal mice.

Disrupted neonatal lung angiogenesis and alveologenesis often give rise to bronchopulmonary dysplasia (BPD), the most common chronic lung disease in children. Hyperoxia-induced pulmonary vascular and alveolar damage in premature infants is one of the most common and frequent factors contributing to BPD. The purpose of the present study was to explore the key molecules and the underlying mechanisms in hyperoxia-induced lung injury in neonatal mice and to provide a new strategy for the treatment of BPD. In this work, we reported that hyperoxia decreased the proportion of endothelial cells (ECs) in the lungs of neonatal mice. In hyperoxic lung ECs of neonatal mice, we detected upregulated fibroblast growth factor receptor 1 (FGFR1) expression, accompanied by upregulation of the classic downstream signaling pathway of activated FGFR1, including the ERK/MAPK signaling pathway and PI3K-Akt signaling pathway. Specific deletion of Fgfr1 in the ECs of neonatal mice protected the lungs from hyperoxia-induced lung injury, with improved angiogenesis, alveologenesis and respiratory metrics. Intriguingly, the increased Fgfr1 expression was mainly attributed to aerosol capillary endothelial (aCap) cells rather than general capillary endothelial (gCap) cells. Deletion of endothelial Fgfr1 increased the expression of gCap cell markers but decreased the expression of aCap cell markers. Additionally, inhibition of FGFR1 by an FGFR1 inhibitor improved alveologenesis and respiratory metrics. In summary, this study suggests that in neonatal mice, hyperoxia increases the expression of endothelial FGFR1 in lung ECs and that deficiency of endothelial Fgfr1 can ameliorate hyperoxia-induced BPD. These data suggest that FGFR1 may be a potential therapeutic target for BPD, which will provide a new strategy for the prevention and treatment of BPD.

E-selectin inhibitor is superior to low-molecular-weight heparin for the treatment of experimental venous thrombosis.

BACKGROUND: This study evaluated E-selectin inhibition with GMI-1271 (Uproleselan [GMI]) alone and in combination with the standard of care low-molecular-weight heparin (LMWH) to improve vein recanalization, decrease vein wall inflammation and protect against adverse bleeding in a primate model. We sought to examine this novel treatment of venous thrombosis. METHODS: Using a well-documented primate animal model, iliac vein thrombosis was induced by balloon occlusion of the iliac vein for 6 hours. Starting on day 2 after thrombosis, animals began treatment in two phases. In phase one, nontreated controls received no treatment (n = 5) vs animals treated with the E-selectin inhibitor GMI, 25 mg/kg, subcutaneous (SC), once daily (n = 4) for 21 days (previously published data). In phase two, animals were treated with GMI plus a combination of LMWH 1.5 mg/kg or 40 mg (GMI + LMWHc) SC once daily (n = 8) for 19 days; and animals treated with LMWH 1.5 mg/kg or 40 mg (LMWHc) SC once daily (n = 6) for 19 days. Animals were evaluated by magnetic resonance venography for vein recanalization and inflammation by gadolinium extravasation, duplex ultrasound, coagulation tests (thromboelastography, bleeding time, prothrombin time, activated partial thromboplastin time, fibrinogen) and complete blood count at baseline, days 2, 7, 14, and 21 at euthanasia. Statistical analysis included using unpaired t test with Welch's correction for direct comparisons and one-way analysis of variance for comparison between the groups. RESULTS: Percent vein recanalization by magnetic resonance venography was highest in the GMI alone group followed by GMI + LMWHc, both significantly different from control. On ultrasound examination, animals treated with GMI alone had no decrease in open vein lumen by day 21, whereas decreases were observed in groups GMI + LMWHc (-26%), LMWHc (-27%), and controls (-80%). Vein wall inflammation decreased significantly in all treated groups. Intimal fibrosis and intimal thickness was best preserved in the GMI alone group. An analysis of total vein wall collagen revealed a trend in all treatment groups of decreasing vein wall collagen. No clinically significant bleeding events were noted in any group. The LMWH groups trended to have prolonged coagulation test values, whereas E-selectin inhibition with GMI did not cause clinically significant changes in coagulation measures. CONCLUSIONS: Treatment with E-selectin inhibition results in improved vein recanalization, a decrease in vein wall inflammation and vein wall intimal thickness and fibrosis, with no changes in markers of coagulation. E-selectin inhibition with GMI alone is superior to E-selectin inhibition combined with LMWH, LMWH alone, and no treatment in this deep vein thrombosis model of iliac vein thrombosis.

Disseminated Fusarium bloodstream infection in a child with acute myeloid leukemia: A case report.

BACKGROUND: Disseminated Fusarium is rare in healthy children. Children with hematological tumors may have secondary fungal infections, including Fusarium infections, which are due to tumor bone marrow infiltration or prolonged bone marrow suppression after chemotherapy. Because of the lack of typical clinical manifestations and effective antifungal drugs, early diagnosis and treatment of the disease are difficult, and the prognosis is poor. CASE SUMMARY: The patient in this case was a 13-year-old female child with rash and fever as the first symptoms. She had the characteristics of the four stages of skin that are typical of Fusarium infection. She was diagnosed with disseminated Fusarium infection through skin biopsy and blood culture and diagnosed with Fusarium solani infection based on the morphological characteristics of the blood culture. After treatment with liposome amphotericin B combined with voriconazole, the child recovered. CONCLUSION: This case highlights that for children with secondary agranulocytosis after receiving chemotherapy for hematological malignancies, once typical abnormal skin damage is found, the possibility of Fusarium infection should be considered, and voriconazole alone or in combination with polyenes may be the most effective anti-Fusarium drugs. Amphotericin B, the traditional drug of disseminated Fusarium disease, has a high mortality rate, and it is not recommended to use it alone. Adequate neutrophil counts are essential for the treatment of disseminated Fusarium bloodstream infection.