Objective To investigate serum β2-MG, sCHE, and PSGL-1 expression in patients with esophageal cancer and their relationship to lung infection after mediastinoscopy. Methods A total of 118 patients with esophageal cancer were selected and divided into infected and uninfected groups according to whether they developed lung infection after surgery. An automatic microbiological identification system was used to detect the pathogenic bacteria of lung infection. ELISA was used to detect the levels of β2-MG, sCHE, and PSGL-1. Multivariate logistic regression was used to analyze the influencing factors of postoperative lung infection in patients with esophageal cancer. ROC curves were plotted to analyze the assessment value of serum β2-MG, sCHE, and PSGL-1 on postoperative lung infection. Results Fifty-two strains of bacteria were isolated from the sputum of 38 patients with postoperative lung infections, and these included 35 (67.31%) Gram-negative, 14 (26.92%) Gram-positive, and 3 (5.77%) fungal strains. The difference in long-term smoking history between the infected and uninfected groups was statistically significant (P<0.05). Serum β2-MG and PSGL-1 levels were significantly higher and sCHE levels were significantly lower in the infected group than in the uninfected group (P<0.05). Serum β2-MG and PSGL-1 levels were sequentially higher (P<0.05) and sCHE levels were sequentially lower (P<0.05) in the mild, moderate, and severe lung infection groups. Long-term smoking history, β2-MG, and PSGL-1 were risk factors affecting postoperative lung infection in patients with esophageal cancer (P<0.05), and sCHE was a protective factor (P<0.05). The AUCs of serum β2-MG, sCHE, and PSGL-1 for assessing postoperative lung infections were 0.807, 0.845, and 0.800, respectively, and the AUC of the three combined factors for assessing postoperative lung infections was 0.954, which was superior to that assessed individually (Z_{combination vs. β2-MG}=2.576, Z_{combination vs. sCHE}=2.623, Z_{combination vs. PSGL-1}=2.574, all P<0.05). Conclusion The serum levels of β2-MG and PSGL-1 increase and the sCHE level decreases in patients with esophageal cancer and postoperative pulmonary infection, which are also related with lung infection. Combined testing can improve the evaluation value of postoperative pulmonary infection in patients.

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.

Bartter syndrome (BS, OMIM #601678) is a rare inherited salt-losing tubulopathy characterized by hypokalemia metabolic alkalosis with secondary renin-angiotensin-aldosterone system activation. As reported, BS type 1 is generally presented prenatal and neonatal period, and symptoms usually appear before and after birth or in infancy, accompanied by severe salt loss, whilst kidney function remains mostly normal. In this study, we report a case of BS type 1 with childhood onset and proteinuria and renal impairment. The child was born preterm due to hyperamniotic fluid, but there were no apparent symptoms after birth until the age of 3 when the child began to present with polydipsia, polyuria and increased nocturnal uria. At the age of 5, she had elevated serum creatinine level and proteinuria. After admission, she was diagnosed with chronic tubulointerstitial disease and stage 2 chronic kidney disease(CKD). According to the chloride clearance test, the abnormal function of medullary thick ascending limb Henle′s loop, was confirmed and BS type 1 was diagnosed by gene sequencing. After active management of complications, kidney function of the child improved. In the long-term follow-up, the urinary protein amount of the child still increased, eGFR slowly decreased, and the child was currently in the CKD2 stage. Children with prenatal BS may not present typical clinical manifestations immediately after birth until the onset of relevant clinical symptoms in childhood. BS type 1 patients may have renal impairment, which needs to be identified in time. Clinical differentiation diagnosis between BS and Gitelman syndrome can be made by chloride clearance tests. Early diagnosis and treatment are critical to improve prognosis.

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 evaluate the diagnostic efficacy and practicality of the Jaundice color card (JCard) as a screening tool for neonatal jaundice.Methods:Following the standards for reporting of diagnostic accuracy studies (STARD) statement, a multicenter prospective study was conducted in 9 hospitals in China from October 2019 to September 2021. A total of 845 newborns who were admitted to the hospital or outpatient department for liver function testing due to their own diseases. The inclusion criteria were a gestational age of ≥35 weeks, a birth weight of ≥2 000 g, and an age of ≤28 days. The neonate′s parents used the JCard to measure jaundice at the neonate′s cheek. Within 2 hours of the JCard measurement, transcutaneous bilirubin (TcB) was measured with a JH20-1B device and total serum bilirubin (TSB) was detected. The Pearson′s correlation analysis, Bland-Altman plots and the receiver operating characteristic (ROC) curve were used for statistic analysis.Results:Out of the 854 newborns, 445 were male and 409 were female; 46 were born at 35-36 weeks of gestational age and 808 were born at ≥37 weeks of gestational age. Additionally, 432 cases were aged 0-3 days, 236 cases were aged 4-7 days, and 186 cases were aged 8-28 days. The TSB level was (227.4±89.6) μmol/L, with a range of 23.7-717.0 μmol/L. The JCard level was (221.4±77.0) μmol/L and the TcB level was (252.5±76.0) μmol/L. Both the JCard and TcB values showed good correlation ( r=0.77 and 0.80, respectively) and agreements (96.0% (820/854) and 95.2% (813/854) of samples fell within the 95% limits of agreement, respectively) with TSB. The JCard value of 12 had a sensitivity of 0.93 and specificity of 0.75 for identifying a TSB ≥205.2?μmol/L, and a sensitivity of 1.00 and specificity of 0.35 for identifying a TSB ≥342.0?μmol/L. The TcB value of 205.2?μmol/L had a sensitivity of 0.97 and specificity of 0.60 for identifying TSB levels of 205.2 μmol/L, and a sensitivity of 1.00 and specificity of 0.26 for identifying TSB levels of 342.0 μmol/L. The areas under the ROC curve (AUC) of JCard for identifying TSB levels of 153.9, 205.2, 256.5, and 342.0 μmol/L were 0.96, 0.92, 0.83, and 0.83, respectively. The AUC of TcB were 0.94, 0.91, 0.86, and 0.87, respectively. There were both no significant differences between the AUC of JCard and TcB in identifying TSB levels of 153.9 and 205.2 μmol/L (both P>0.05). However, the AUC of JCard were both lower than those of TcB in identifying TSB levels of 256.5 and 342.0 μmol/L (both P<0.05). Conclusions:JCard can be used to classify different levels of bilirubin, but its diagnostic efficacy decreases with increasing bilirubin levels. When TSB level are ≤205.2 μmol/L, its diagnostic efficacy is equivalent to that of the JH20-1B. To prevent the misdiagnosis of severe jaundice, it is recommended that parents use a low JCard score, such as 12, to identify severe hyperbilirubinemia (TSB ≥342.0 μmol/L).

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.

Humans ; China/epidemiology* ; SARS-CoV-2 ; COVID-19

Infection is the most common complication of nephrotic syndrome in children.Serious infection leads to poor prognosis, and always deteriorates rapidly, especially in the infection of pneumocystis jeroveci and varicella.For the long-term use of steroid and immunosuppressor, patients with infection always have atypical clinical symptoms and the correct diagnosis is liable to be delayed.Therefore, it′s important to be well aware of medical histories, physical signs and associated laboratory tests.Timely control of infection and protection of renal function are the main principles of treatment in the children with nephrotic syndrome and serious infection.Meanwhile, daily health management should be strengthened for the patients to prevent the occurrence of infection.