Objective:To explore the clinical treatment of retinoblastoma (RB) after being treated with vitrectomy (PPV) due to misdiagnosis.Methods:A retrospective case study. From July 2015 to July 2018, 5 cases and 5 eyes of RB children diagnosed by pathological examination at the Eye Center of Beijing Tongren Hospital were included in the study. Among them, there were 3 males with 3 eyes and 2 females with 2 eyes; all of them had monocular disease. The average age was 4.8±1.7 years old. At the first visit, the diagnosis was endophthalmitis in 2 eyes (40%, 2/5); vitreous hemorrhage in 3 eyes (60%, 3/5). All were treated with PPV. All children underwent slit lamp microscopy, orbital MRI and CT, and eye color Doppler ultrasound blood flow imaging. If there was no clear extraocular spread, the eyeball removal combined with artificial orbital implantation was performed; if there was clear extraocular spread, the modified orbital content enucleation operation was performed with part of the eyelid preserved. The average follow-up time after surgery was 34.6±7.9 months.Results:Among the 5 eyes, 2 eyes (40%, 2/5) underwent eyeball enucleation combined with stage I artificial orbital implantation, and 3 eyes (60%, 3/5) with modified orbital content enucleation. There were 2 eyes of endogenous type (40%, 2/5), 1 eye of diffuse infiltration type (20%, 1/5), and 2 eyes of mixed type (40%, 2/5). The orbit spread in 3 eyes, the tumor invaded the optic nerve in 1 eye, and regional lymph node metastasis in 2 eyes. All children received systemic chemical therapy (chemotherapy). During the follow-up period, there were no new metastatic diseases and no deaths.Conclusions:After RB misdiagnosis and PPV, surgical treatment should be performed as soon as possible. If there is no clear extraocular spread, eyeball removal or combined stage I orbital implantation should be performed. If there is clear extraocular spread, the orbital contents should be enucleated; Chemotherapy should be combined after surgery.

BACKGROUND: Autologous blood transfusion device has been widely used in the clinic, reduces al ogeneic blood transfusion, and avoids the occurrence of blood transfusion complications, and effectively improves the patient’s blood safety, but the application of autologous blood transfusion after total hip arthroplasty has been seldom reported. OBJECTIVE: To discuss the safety and effectiveness of autologous blood transfusion after total hip arthroplasty. METHODS: 200 patients were treated by primary unilateral total hip arthroplasty from March 2013 to March 2015. They were randomly divided into two groups. 127 patients in the autologous blood transfusion group received autologous blood transfusion by a drainage tube. 73 patients in the negative pressure drainage bal group received a negative pressure drainage tube. The standard for al ogeneic blood transfusion after replacement was hemoglobin < 80 g/L. The changes in hemoglobin were compared before and 1 and 7 days after replacement between the two groups. Total drainage volume and al ogeneic blood transfusion were compared within 6 hours after replacement between the two groups. RESULTS AND CONCLUSION: There were no statistical differences in hemoglobin levels at 7 days before and after replacement, in drainage volume within 6 hours and the total drainage volume between the two groups (P >0.05). Hemoglobin levels were significantly higher in the autologous blood transfusion group than in the negative pressure drainage bal group at 1 day after replacement (P < 0.05). In the autologous blood transfusion group, autologous blood transfusion volume was averagely 324.2 mL. Al ogeneic blood transfusion volume was averagely 146.7 mL in 31 patients. No reaction was found after autologous blood transfusion. In the negative pressure drainage bal group, 49 patients received al ogeneic blood transfusion (averagely 261 mL). The volume and proportion of al ogeneic blood transfusion were significantly lower in the autologous blood transfusion group than in the negative pressure drainage bal group (P < 0.05). Among patients receiving al ogeneic blood transfusion, seven patients affected pyrogenetic reaction during blood transfusion. These findings suggested that autologous blood transfusion is simple and effective, can effectively reduce the volume and reaction of al ogeneic blood transfusion after total hip arthroplasty and avoid blood-borne diseases, with good prospects.

OBJECTIVETo observe the treatment and prognosis of optic nerve invasion in retinoblastoma (RB).

METHODThe children who had been diagnosed with unilateral RB and had received enucleation from January 2006 to December 2013 in our hospital were recruited. Tumor extension into the optic nerve were disclosed. Optic nerve involvement was classified into four grades according to the degree of invasion. Grade I is superficial invasion of the optic nerve head only, grade II is involvement up to and including the lamina cribrosa, grade III is involvement beyond the lamina cribrosa, and grade IV is involvement up to and including the surgical margin. Grade I and II are called invasion of the optic nerve before the sieve plate. Grade III and IV are called invasion of the optic nerve after the sieve plate. Other high-risk factors included extensive invasions of the choroid, sclera, anterior chamber, iris, and ciliary body. They were divided into two groups according to whether the merger of other high histopathologic risk factors. Treatment was delivered accordingly, and the prognosis of different degrees of optic nerve invasion was observed. The subjects were followed up for 6 months to 7 years (average: 43 months).

RESULTThere were 465 subjects in this study, including 279 boys and 186 girls. The right eye was affected in 260 patients and the left eye in 205 patients. The average time from onset of symptoms to visit was 2.7 months (range 1 day-24 months). Twenty-five patients died, resulting in an overall survival rate of 94.6%. The mortality rate of patients with optic nerve involvement with grade I was 0.4%, grade II was 1.0%, grade III was 8.7% was and grade IV was 60.9%. Of the 338 with invasion of the optic nerve before the sieve plate, two died of recurrence, with a survival rates of 99.4% (336/338). Of the 127 patients who had invasion of the optic nerve after the sieve plate, twenty-three died of recurrence, with a survival rate of 81.9% (104/127) , the difference was statistically significant (χ² = 52.299, P = 0.000). A total of 379 patients did not have any other merged pathology high-risk factors, 8 died, the mortality rate was 2.1%. Of the 86 patients who had complicated with other high-risk factors, 17 died, the mortality rate was 19.8%, the difference was statistically significant (χ² = 42.955, P = 0.000). Of the 338 patients, 304 had invasion of the optic nerve before the sieve plate had not merged other pathology high-risk factors, none died, of the 34 patients who had complicated with other pathology high-risk factors, 2 had died, the mortality rate was 5.9%, the difference was statistically significant (P = 0.010). Of the 127 patients with invasion of the optic nerve after the sieve plate, 76 had not complicated with other pathological high-risk factors, 9 of whom had died, the mortality rate was 11.8%, 51 had complicated with other pathological high-risk factors, 14 of whom had died, the mortality rate was 27.5%, outcomes did significantly differ between the two subgroups (χ² = 5.014, P = 0.025). Cox multivariate analysis showed that invasion of the retrolaminar optic nerve, surgical margin of the optic nerve and sclera were influential factors of colorectal cancer.

CONCLUSIONPatients with optic nerve invasion have an excellent outcome with current therapy. But for those whose resection margin was invaded, which has a high incidence of recurrence, chemotherapy is recommended for patients with postlaminar optic nerve involvement.

Child ; Female ; Humans ; Male ; Multivariate Analysis ; Neoplasm Invasiveness ; Neoplasm Recurrence, Local ; Optic Nerve Neoplasms ; pathology ; surgery ; Prognosis ; Retinoblastoma ; pathology ; surgery ; Retrospective Studies ; Survival Rate

Transient receptor potential cation channel 6（TRPC6）gene is mainly expressed in renal podiocytes.Its variation can lead to steroid-resistant nephrotic syndrome（SRNS），and the specific pathogenesis is not clear.These children have poor response to hormones and immunosuppressants，with lack of specific therapeutic drugs，and poor prognosis.In recent years，it has been found that some drugs can slow down disease progression by inhibiting the expression of TRPC6 gene or its downstream signaling pathway，and the discovery of TRPC6 protein-specific blockers may be the hope of treating such children.This review focuses on the pathogenesis of SRNS induced by TRPC6 gene variation in children and the research progress of drug therapy.

Objective:To summarize the clinical and genetic characteristics, treatment and prognosis of four children with Steroid-resistant nephrotic syndrome (SRNS) due to variants of TRPC6 gene. Methods:Clinical data of four children with SRNS admitted to Children′s Hospital Affiliated to Zhengzhou University between May 2020 and August 2022 were collected. Peripheral blood samples were collected from the children and their parents, and whole exome sequencing was carried out. Sanger sequencing was used to verify the pathogenicity of the candidate variants among the children and their parents.Results:All of the four children were found to harbor heterozygous variants of the TRPC6 gene, including c. 523C>T (p.R175W), c. 1327T>A (p.F443I), c. 430G>C (p.E144Q) (unreported previously), and c. 523C>T (p.R175W), which were all missense variants. Two of the children have shown a simple type, whilst two have shown a nephritis type, none had extrarenal phenotype. Comprehensive renal pathology of three children revealed focal segmental glomerulosclerosis (FSGS). Two children were treated with steroids combined with calcineurin inhibitors (CNIs), among whom one showed significant improvement in symptoms. Conclusion:Discoveries of the novel c. 430G>C variant and the new SRNS phenotype of the c. 1327T>A variant have expanded the mutational and phenotypic spectrum of the TRPC6 gene, which has provided a reference for clinical diagnosis and genetic counseling for the families.

Objective:To investigate the factors affecting the time taken for B cell reconstitution after rituximab (RTX) treatment in children with steroid-sensitive nephrotic syndrome.Methods:This was a retrospective cohort study. The clinical data of 42 children with SSNS who received treatment with RTX in Department of Nephrology, Rheumatology and Immunology, Children′s Hospital Affiliated to Zhengzhou University between December 2019 and May 2023 were analyzed retrospectively. The data of demographics, immunosuppressant treatment and laboratory tests such as CD19 +B cell count, urinary protein quantification were collected. The patients were divided into 2 groups, the early B cell reconstruction group and the late reconstruction group based on the average time of B cell reconstruction. A multivariate logistic regression model was used to analyze the factors impacting the timing of B cell reconstruction, and the predictive value of these factors was assessed by plotting the receiver operating characteristic (ROC) curve. Results:There were 42 children, with 35 males and 7 females. They were aged 3.5 (2.2, 5.9) years at the onset of PNS and (8.4±3.3) years at their first RTX treatment. The time for B cell reconstitution was (152±53) d. There were 20 children in the early reconstruction group and 22 children in the late reconstruction group. There were no statistically significant differences (all P>0.05) between the 2 groups in terms of the cumulative dose of steroids within 1 year before receiving RTX infusion (0.29 (0.16, 0.50) vs. 0.29 (0.19, 0.46) mg/(kg·d)), the percentage of children using tacrolimus before RTX (65%(13/20) vs. 45%(10/22)) and cumulative doses (0.04 (0.03, 0.05) vs. 0.03 (0.03, 0.06) mg/(kg·d)), the steroid doses at the time of RTX infusion (0.73 (0.49, 0.90) vs. 0.71 (0.58, 0.89) mg/(kg·d)), the percentage of children using tacrolimus at the initial RTX infusion (50% (10/20) vs. 41% (9/22)) and the doses (0.03 (0.02, 0.04) vs. 0.02 (0.01, 0.04) mg/(kg·d)), the discontinuation time of tacrolimus post-RTX infusion (71 (42, 91) vs. 64 (42, 91) d). A multivariate analysis revealed a correlation ( OR=0.26, 95% CI 0.10-0.68, P=0.006) between B cell count following the second RTX infusion and the time taken for B cell reconstruction. The area under the ROC curve for B cell count after the RTX infusion in predicting the time to B cell reconstruction was 0.89 (95% CI 0.78-0.99, P<0.001) and the cut-off value was 0.925×10 6/L. Conclusions:The time of B cell reconstruction is not influenced by the previous or concurrent use of tacrolimus, regardless of its duration and the dosage of steroid and tacrolimus prior to the RTX infusion. Insteadly, the peripheral blood B cell count (0.925×10 6/L) following the second RTX infusion for SSNS is identified as an independent predictor of reconstruction time, allowing for a more precise prediction and early intervention to maintain disease remission.

OBJECTIVES: Postoperative delirium (POD) and postoperative cognitive dysfunction (POCD) are common operative neurocognitive disorders, which places a heavy burden on patients, families and society. Therefore, it is very important to search for preventive drugs. Previous studies have demonstrated that perioperative use of dexmedetomidine resulted in a decrease the incidence of POD and POCD. But the specific effect of dexmedetomidine on elderly patients undergoing hepatic lobectomy and its potential mechanism are not clear. This study aims to evaluate the efficacy of intraoperative use of dexmedetomidine on preventing POD and POCD in elderly patients undergoing hepatic lobectomy and the influence on the balance between proinflammation and anti-inflammation. METHODS: This trial was designed as a single-center, prospective, randomized, controlled study. One hundred and twenty hospitalized patients from January 2019 to December 2020, aged 60-80 years old with American Society of Anesthesiologists (ASA) II-III and scheduled for hepatic lobectomy, were randomly allocated into 3 groups (n=40) using a random number table: A C group, a Dex1 group, and a Dex2 group. After anesthesia induction, saline in the C group, dexmedetomidine [0.3 μg/(kg·h)] in the Dex1 group, and dexmedetomidine [0.6 μg/(kg·h)] in the Dex2 group were infused until the end of operation. The incidences of hypotension and bradycardia were compared among the 3 groups. Confusion Assessment Method (CAM) for assessing POD and Mini Mental State Examination (MMSE) for evaluating POCD were recorded and venous blood samples were obtained for the determination of neuron specific enolase (NSE), TNF-α, IL-1β, and IL-10 at the different time below: the time before anesthesia (T0), and the first day (T1), the third day (T2), the fifth day (T3), and the seventh day (T4) after operation. RESULTS: Compared with the C group, the incidences of bradycardia in the Dex1 group or the Dex2 group increased (both P<0.05) and there was no difference in hypotension in the Dex1 group or the Dex2 group (both P>0.05). The incidences of POD in the C group, the Dex1 group, and the Dex2 group were 22.5%, 5.0%, and 7.5%, respectively. The incidences of POD in the Dex1 group or the Dex2 group declined significantly as compared to the C group (both P<0.05). However, there is no difference in the incidence of POD between the Dex1 group and the Dex2 group (P>0.05). The incidences of POCD in the C group, the Dex1 group, and the Dex2 group were 30.0%, 12.5%, and 10.0%, respectively. The incidences of POCD in the Dex1 group and the Dex2 group declined significantly as compared to the C group (both P<0.05). And no obvious difference was seen in the incidence of POCD in the Dex1 group and the Dex2 group (P>0.05). Compared with the C group, the level of TNF-α and IL-1β decreased and the level of IL-10 increased at each time points (from T1 to T4) in the Dex1 group and the Dex2 group (all P<0.05). Compared with the Dex1 group, the level of IL-1β at T2 and IL-10 from T1 to T3 elevated in the Dex2 group (all P<0.05). Compared with the T0, the concentrations of NSE in C group at each time points (from T1 to T4) and in the Dex1 group and the Dex2 group from T1 to T3 increased (all P<0.05). Compared with the C group, the level of NSE decreased from T1 to T4 in the Dex1 group and the Dex2 group (all P<0.05). CONCLUSIONS Intraoperative dexmedetomidine infusion can reduce the incidence of POCD and POD in elderly patients undergoing hepatic lobectomy, and the protective mechanism appears to involve the down-regulation of TNF-α and IL-1β and upregulation of IL-10 expression, which lead to rebalance between proinflammation and anti-inflammation.
Aged ; Aged, 80 and over ; Bradycardia ; Cognitive Dysfunction/prevention & control* ; Delirium/prevention & control* ; Dexmedetomidine/therapeutic use* ; Humans ; Hypotension/drug therapy* ; Interleukin-10 ; Middle Aged ; Postoperative Cognitive Complications/prevention & control* ; Postoperative Complications/epidemiology* ; Prospective Studies ; Tumor Necrosis Factor-alpha

BACKGROUNDThe use of post-enucleation adjuvant therapy to decrease the extraocular relapse rate is frequently considered, but there is much controversy about the indications for adjuvant therapy. The aim of this retrospective study was to observe the treatment and prognosis for different degrees of invasion of eye tissue in retinoblastoma (RB) and identify the indications for post-enucleation adjuvant therapy.

METHODSWe recruited 537 children who had been diagnosed with unilateral RB and had received enucleation from January 2006 to December 2012 in our hospital, and divided them into three groups according to their number of histopathologic risk factors: 0 factor, 1 factor, or ≥2 factors. Histopathologic high-risk factors included invasion of the optic nerve posterior to the ethmoid plate (including optic nerve stumps) and extensive invasions of the choroid, sclera, anterior chamber, iris, and ciliary body. Treatment was delivered accordingly, and the prognosis of different degrees of histopathologic invasion was observed. The subjects were followed up for 6 months to 7 years (average follow-up time: 35 months). Statistical analysis was analyzed using χ(2) test. P < 0.05 was considered significant.

RESULTSOf the 537 RB patients who received enucleation, 25 died (overall survival: 95.3%). Of the 369 (68.7%) with no histopathologic risk factors, 1 died of recurrence, with a mortality rate of 0.3%, whereas of the 168 (31.3%) with histopathologic risk factors, 26 had recurrences and 24 died (mortality rate: 14.3%; P = 0.000). Of the 93 patients (17.3%), each of whom had a single risk factor, nine had recurrences, 16 died (8.6%). Of the 75 patients (14%) with two or more high-risk factors, 16 died (21.3%). These differences were statistically significant between the three (P = 0.000).

CONCLUSIONChemotherapy is recommended for patients with histopathologic risk factors, especially those with two or more histopathologic risk factors.

Antineoplastic Agents ; therapeutic use ; Child ; Child, Preschool ; Humans ; Infant ; Infant, Newborn ; Prognosis ; Retinoblastoma ; drug therapy ; pathology ; Risk Factors