Objective:To investigate the clinicopathological features and the prognosis of IgA nephropathy (IgAN) in children with massive proteinuria.Methods:It was a retrospective cohort study. Clinical data of IgAN children with massive proteinuria admitted to the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2008 to December 2021 were retrospectively analyzed. Patients were divided into effective group and ineffective group according to whether urine protein turned negative after 6 months of initial treatment. The follow-up endpoint event was defined as a reduction in proteinuria of less than 50% or end-stage renal disease (ESRD) achievement. MedCalc software was used to perform Kaplan-Meier survival analysis, and Log-rank test was used to compare the difference of renal survival between the two groups.Results:A total of 127 patients were diagnosed as primary IgAN by renal biopsy, of whom 57 patients with IgAN showed massive proteinuria. These 57 IgAN patients with macroproteinuria accounted for 44.9% of the total IgAN patients and were enrolled in the study. Among the 57 cases, 33 cases (57.9%) were Lee's grade Ⅲ, 11 cases (19.3%) were below Lee's grade Ⅲ, and 13 cases (22.8%) were above Lee's grade Ⅲ. The follow-up time was 4.0 (3.0,5.8) years. In the initial treatment, among 57 patients, 46 (80.7%) were effective (effective group) and 11 (19.3%) were ineffective (ineffective group). Compared with the effective group, the ineffective group had a higher proportion of concurrent AKI at the onset of disease and longer recovery time of renal function, with significant difference (7/11 vs. 13/46, χ2=4.878, P=0.027). Compared with the effective group, the proportion of Lee grade Ⅲ or above was higher in the ineffective group, and the difference was statistically significant (5/11 vs. 8/46, χ2=3.971, P=0.046). There were significant differences in endocapillary hypercellularity (E1), segmental glomerulosclerosis or adhesion (S1) and cellular/fibrocellular crescents (C2) of Oxford classification between IgAN children with Lee grade Ⅲ or below and those over Lee grade Ⅲ (11/13 vs. 20/44, χ2=6.204, P=0.013; 12/13 vs. 17/44, χ2=11.566, P=0.001; 9/13 vs. 7/44, χ2=14.131, P=0.001). Among 57 patients, endpoint events occurred in 2 patients who both were urinary protein unmitigated, and none of the children progressed to ESRD. There was no significant difference in cumulative renal survival between the two groups by Kaplan-Meier survival analysis and Log-rank test ( χ2=0.537, P=0.460) after addition of calcineurin inhibitors (CNIs) to the initial treatment ineffective group. Conclusions:Macroproteinuria is the prominent manifestation of IgAN in children. The pathological type is mainly Lee grade Ⅲ. Children with macroproteinuria have a good prognosis in the short and medium term after active treatment. For IgAN with macroproteinuria that does not respond well to initial treatment, AKI is more common at onset, and renal function recovery time is longer. The application of CNIs may have a certain effect on improving the renal outcome of IgAN with massive proteinuria.

This article reported the diagnosis and treatment of a boy with Dent disease presenting with massive proteinuria.He was 3 years old and found to have massive proteinuria during routine physical examination without hypoalbuminemia, urine protein electrophoresis indicated mainly low molecular weight proteins, with hypercalciuria, and metabolic acidosis, no diabetes, no amino acid urine, and renal ultrasound showed no renal calcium deposition, He had no mental and physical developmental delay and no abnormal family history. Gene detection revealed one missense mutation in exon 15 of the OCRL1 gene, c.1477C > T (p.Arg493Trp). After the diagnosis was confirmed, restrictions in dietary intake of calcium, sodium, and oxalate was restricted and oral potassium citrate and hydrochlorothiazide was prescribed. During two months of follow-up, we observed a decrease in urinary calcium levels and normal renal function. This article aims to improve the understanding of this disease among physicians and provide reference for the diagnosis and treatment of this disease through typical case report and review of previous literatures.

The treatment of head and neck cancer is dominated by surgery and radiochemotherapy.For elderly patients,however,the rationality of invasive treatment is questioned in clinical practice.Although the proportion of geriatric patients with head and neck canc-er is increasing,there are few consistent standards in the literature and guidelines.From the perspective of evidence-based medicine,this paper presents current evidence for treatment so as to provide specific recommendations for treating head and neck cancer in the elderly.Furthermore,based on a systematic literature review,a clinical algorithm is proposed to evaluate the treatment risk of elderly patients with head and neck cancer.

A patient who underwent posterior lumbar interbody fusion was treated with imrecoxib,the patient's liver biochemical examination was abnormal.Follow taking polyene phosphatidylcholine injection combined with glutathione for liver protection,alanine aminotransferase(ALT),aspartate aminotransferase(AST)and alkaline phosphatase(AKP)still increased progressively,with peaks of 404.7,122.8 and 255.1 U·L-1,respectively.With stopping the use of imrecoxib,and adjusting the liver protection treatment of polyene phosphatidylcholine injection combined with compound glycyrrhizin injection,ALT,AST and AKP decreased gradually before discharge and were 54.6,21.2,137.2 U·L-1,respectively.Associations were evaluated by RUCAM rating scale,with an imrecoxib association score of 8,and it may be causing liver damage of this patient.The hepatotoxicity should be vigilant and the liver function should be monitored in application of imrecoxib to ensure the safety of drug in patients.

Objective:To explore the application value of singular value decomposition(SVD)in optimizing the image quality of contrast-enhanced ultrasound(CEUS)and perioperative CEUS assessment of microwave ablation(MWA)for thyroid nodule.Methods:The data sets of two-dimensional ultrasound and CEUS images of two patients with thyroid nodules,who underwent MWA in China Resources&Wisco General Hospital affiliated to Wuhan University of Science and Technology from April 2023,were collected.The contrast images based on CEUS images after SVD filtering were obtained according to the normalized cross-correlation algorithm,and the differences of signal-to-noise ratio(SNR)and micro-vessel density(MVD)between the contrast images after SVD filtering and original CEUS images were compared.Results:Compared with the original CEUS images of thyroid nodules before and after MWA,the contrast images that were processed by SVD filtering can more clearly show the vascular structure with thyroid nodules,and can detect more signals of blood flow.The results showed that the average SNR of the original CEUS images after operation was(1.07±0.66)dB,and the average SNR of the contrast images by SVD filtering after operation was(6.21±1.01)dB(t=-6.015,P=0.038).The average MVD of the original CEUS images after operation was(0.60±0.33)%,and the average MVD of the contrast images of SVD filtering after operation was(5.42±0.67)%(t=-9.076,P=0.029).It can show that the SNR and MVD of the contrast images of SVD-filtering after operation were significantly higher than these of the original CEUS images after operation,and the results of these studies were significant.Conclusion:SVD filtering can effectively suppress noise and enhance the signals of micro-blood flow.The improved quality of contrast images can be used in the accurate diagnosis of perioperative MWA of thyroid nodules,it can sensitively detect the residually incomplete areas of ablation after operation,so as to guide clinicians to carry out more thorough ablation treatment and provide decision support for the assessment of curative effect of accurate ablation.

Objective To study the clinical characteristics and related factors affecting the severity of acute tinnitus in patients.Methods A retrospective analysis was conducted on the data of 319 patients with acute tinni-tus.All patients had detailed case history,including basic patient information,tinnitus location,course of disease,acoustic characteristics of tinnitus,hearing loss,combined headache,dizziness,aural fallness,earache and other symptoms,as well as accompanying clinical diseases.All patients completed the tinnitus handicap inventory(THI),generalized anxiety disorder(GAD-7),patient health questionnaire(PHQ-9),pittsburgh sleep quality index(PSQI),hyperacusis questionnaire(HQ),type D personality scale-14(DS-14),etc.The mild tinnitus group was determined based on a THI score of ≤36.The moderate tinnitus group was determined based on a THI score of 38～56.The severe to extremely severe tinnitus group was determinded based on THI 56～100.THI grouping was used as the dependent variable and the above factors as independent variables.The correlation between each factor and the severity of acute tinnitus was analyzed using ordered multinomial logistic regression.Results Among 319 patients with acute tinnitus,158(49.5％)were in the mild tinnitus group,and 72(22.6％)were in the moderate tinnitus group,and 89(27.9％)were in severe to extremely severe tinnitus group.Ordered multinomial logistic re-gression analysis found that hyperacusis(OR=3.921),anxiety(OR=2.495),depression(OR=2.921),and D-type personality(OR=0.349)were associated with the severity of acute tinnitus(P＜0.05),with more serere tin-nitus in these patients.Conclusion Hyperacusis,anxiety,depression,and D-type personality may be factors that affect the severity of acute tinnitus,so high attention should be paid when treating patients with acute tinnitus.

Objective:To summarize and analyze the clinical phenotype and genotype characteristics of atypical hemolytic uremic syndrome (aHUS) in Chinese children.Methods:It was a retrospective study. The clinical data and genetic results of 6 children with aHUS admitted to Children's Hospital Affiliated to Capital Institute of Pediatrics from May 2016 to October 2022 were analyzed, and literature on Chinese aHUS children with genetic screening data by searching databases such as Wanfang, CNKI, and PubMed were reviewed and summarized. Through literature search, the children with aHUS were divided into genetic variation group and non-genetic variation group according to the results of genetic testing, and the differences of clinical phenotype, laboratory examination, follow-up and outcomes were compared between the two groups. Logistic regression method was used to analyze the risk difference of disease recurrence, end-stage kidney disease and death between genetic variation group and non-genetic variation group.Results:Among the 6 aHUS children in this center, there were 1 male and 5 females, with onset age of 7 months to 10 years old. Four patients had gene variations, including 1 patient of complement factor H ( CFH) gene variation, 1 patient of C3 gene variation, and 2 patients of CFHR1 combined with CFHR3 gene variation. Six children had gross hematuria, proteinuria, hypertension and decreased complement C3. The mean values of serum creatinine in 4 genetic variation and 2 non-genetic variation children were 153.9 μmol/L and 214.3 μmol/L, respectively; the mean values of estimated glomerular filtration rate were 26.4 ml·min -1·(1.73 m 2) -1 and 28.9 ml·min -1·(1.73 m 2) -1, respectively; the mean values of hemoglobin were 81 g/L and 57 g/L; the mean values of platelet were 46×10 9/L and 71×10 9/L; the mean values of lactic dehydrogenase were 2 408 U/L and 2 106 U/L, respectively; there were 1 and 2 cases of positive CFH antibody, and 1 and 1 case of nervous system complication, respectively. Ninety-seven aHUS children were retrieved including the reported 6 cases in this center, with 60 males and 37 females, and median onset age of 5 years old. The positive detection rate of genetic variation was 58.8% (57/97). The main type of genetic variation was CFHR gene variation (43.9%, 25/57), followed by CFH gene variation (33.3%, 19/57).There was no significant difference in onset age, sex distribution, proportions of gross hematuria, massive proteinuria, hypertension, complement C3 decline, positive CFH antibody and treatment method, platelet, and lactic dehydrogenase between genetic variation group and non-genetic variation group (all P>0.05). Compared with the genetic variation group, non-genetic variation group had higher serum creatinine ( Z=2.311, P=0.021) and lower hemoglobin ( Z=-2.636, P=0.008). The median follow-up time in genetic variation group and non-genetic variation group was 1 year and 2 years, respectively. The proportions of non-remission and recurrence in the genetic variation group were significantly higher than those in non-genetic variation group ( χ2=12.016, P=0.002; χ2=4.689, P=0.030). Logistic regression analysis showed that the recurrence risk of aHUS in children with genetic mutations was higher than that in children with non-genetic mutations ( OR=2.807, 95% CI 1.014-7.772). Conclusions:The main type of aHUS gene variation in Chinese children is CFHR gene variation, and the children with gene variation have poor prognosis and a higher risk of recurrence.

It was a retrospective study. The case data of thirteen patients diagnosed with methylmalonic acidemia (MMA) combined with hemolytic uremic syndrome (HUS) hospitalized at the Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2010 to December 2022 were analyzed, to explore clinical and genetic characteristics of MMA combined with HUS (MMA-HUS). The onset age of MMA was 18 days to 5 years old, with 11 patients of early onset and 2 patients of late onset, 4 patients of simple MMA and 9 patients of combined MMA, and 7 patients of secondary hypertension and 3 patients of secondary pulmonary arterial hypertension. Among 13 patients, 7 patients underwent genetic testing, with 1 patient of MMUT gene mutation and 6 patients of MMACHC gene mutation. The mutation sites were all from their parents and all were known pathogenic variants. Among them, 5 patients had MMACHC gene c.80A>G heterozygous variation, accompanied by cardiovascular involvement. After etiological and symptomatic treatment, 6 patients improved, 7 patients died of multiple organ failure, and all the deaths were early-onset MMA. This study shows that MMA-HUS is more common in early-onset MMA, with a severe condition and a high mortality rate. Its prognosis is related to multiple factors such as genotype, diagnosis and treatment timing. c.80A>G variation of MMACHC gene may be related to HUS and cardiovascular involvement.

Objective:To summarize and analyze the clinical features and risk factors of acute focal bacterial nephritis (AFBN) in children.Methods:It was a retrospective cohort study. The clinical data of patients diagnosed with upper urinary tract infection in Children's Hospital Affiliated to Capital Institute of Pediatrics from July 1, 2016 to July 31, 2021 were collected, and the patients all received abdominal enhanced CT examination. According to the imaging examination results, the patients were divided into AFBN group and acute pyelonephritis (APN) group, and the clinical manifestations, laboratory and imaging examination between the two groups were compared. Logistic regression model and receiver operating characteristic curve were used to analyze the risk factors of AFBN.Results:A total of 135 patients with upper urinary tract infection were enrolled in this study, with age of 2.5 (0.5, 3.7) years old, and 68 males (50.4%). There were 67 patients (49.6%) in AFBN group and 68 patients (50.4%) in APN group. There were statistically significant differences in the highest fever temperature, duration of fever after treatment, proportion of lower urinary tract irritation symptoms, proportion of urinary tract malformation or abnormality, white blood cell count, neutrophil count, procalcitonin, C-reactive protein, proportion of pyuria, urinary β2 microglobulin and proportion of using carbapenem antibiotics between the two groups (all P<0.05). Multivariate logistic regression analysis result showed that urinary tract malformation/abnormality ( OR=3.34, 95% CI 1.23-9.10) and leukocytosis ( OR=1.25, 95% CI 1.03-1.51) were the independent risk factors of AFBN. Conclusions:The children with urinary tract infection who have high peak fever, long duration, obvious increase of inflammatory indexes and urinary β2 microglobulin may suggest AFBN. Urinary tract malformation/abnormality and high white blood cells are risk factors of AFBN.

Objective:To compare the clinical value of visual analogue scale （VAS）, Lebel scale and total nasal symptom scores （TNSS） in evaluating nasal allergen provocation test （NAPT）. Methods:A total of 151 patients suspected of allergic rhinitis admitted to the Department of Otolaryngology-Head and Neck Surgery of our hospital from April 2020 to September 2020 were included, of which 76 were positive for house dust mites and 75 were negative for allergens. Nasal airway resistance（NAR） was measured by active anterior nasal manometry. Nasal symptoms were evaluated by VAS, Lebel and TNSS. House dust mite allergen was used for NAPT by spray method. An increase≥40% in NAR was used as the gold standard for objective evaluation of NAPT. ROC curves of VAS, Lebel and TNSS were drawn to compare the evaluation effectiveness of different subjective evaluation methods, and the optimal critical point of each ROC curve was obtained. Results:With NAR increased by ≥40% as the gold standard, the area under ROC curve of VAS was 0.884, and the sensitivity and specificity were 97.75% and 80.65%, respectively. The area under ROC curve of Lebel was 0.773, and the sensitivity and specificity were 68.54% and 75.81%, respectively. The area under ROC curve of TNSS was 0.792, and the sensitivity and specificity were 68.54% and 79.03%, respectively. There was no significant difference between Lebel and TNSS（P>0.05）. The VAS differed significantly from Lebel and TNSS（P<0.05）. The Kappa values of VAS, Lebel, TNSS and NAR were 0.803, 0.432 and 0.459, respectively. Conclusion:The VAS, Lebel, TNSS subjective scale and NAR are consistent in evaluating the efficacy of NAPT, with the VAS assessment showing highest consistency with NAR. As objective assessment instruments are not widely used in China, subjective assessment method could be adopted to evaluate the efficacy of NAPT in clinical practice, and VAS scale is recommended as a priority.
Animals ; Humans ; Allergens ; Nasal Provocation Tests/methods* ; Rhinitis, Allergic/diagnosis* ; Nose ; Pyroglyphidae