Objective To assess the value of functional magnetic resonance urography (fMRU)for the unilateral renal function in children with hydronephrosis.Methods Fourteen children with congenital hydronephrosis (unilateral hydronephrosis in 1 2 cases,bilateral hydronephrosis in 2 cases)examined by fMRU in Beijing Children′s Hospital,Capital Medical University,were enrolled.In 7 patients of them,diuretic renal scintigraphy (DRS)was per-formed within 1 0 days before fMRU examination.The following parameters in fMRU,as renal parenchymal volume,volu-metric differential renal function (vDRF),Patlak,Patlak differential renal function (pDRF),index of glomerular filtra-tion rate (GFR)and differential renal function based on index of GFR (gDRF),were calculated and analyzed.Statisti-cal analysis was performed by using SPSS 1 3.0.Results In 7 cases whose fMRU and DRS were examined,the indexes of GFR obtained from fMRU and GFR from DRS were well correlated (r =0.892,P <0.001 )in 1 4 kidneys.The gDRF determined by 2 methods on the left kidneys[the average was(46.80 ±1 9.20)% and(45.1 8 ±20.29)%,respective-ly]had no significant difference (t =0.051 6,P =0.624),which was also highly correlated (r =0.91 2,P =0.004). In 1 2 cases with unilateral hydronephrosis,vDRF,pDRF,index of GFR and gDRF in hydronephrotic side[(43.54 ± 9.61 )%,(42.80 ±1 0.83)%,(38.56 ±29.23)mL/min,(38.37 ±1 3.61 )%]were all less than those in the con-tralateral side[(56.46 ±9.61 )%,(57.1 9 ±1 0.83)%,(57.02 ±26.22)mL/min,(61 .63 ±1 3.61 )%](t =2.326, 2.300,2.422,2.960;P =0.040,0.042,0.034,0.01 3).However,there was no statistical difference in both renal pa-renchymal volume and Patlak between the hydronephrotic and the contralateral side kidneys(t =1 .765,1 .450;P =0.1 05,0.1 75).Conclusions fMRU is a very valuable examination method in evaluating single kidney function in children with congenital hydronephrosis,and able to demonstrate that gDRF,indexes of GFR,vDRF and pDRF decrease in the hydronephrotic kidney.

Objective:To investigate the impact of different type of dyslipidemia on clinical and pathological characteristics in children with IgA nephropathy (IgAN).Methods:A retrospective study was performed at the Children Kidney Disease Center, the First Affiliated Hospital of Sun Yat-sen University between January 2006 to September 2019. Children diagnosed with primary IgAN was divided into dyslipidemia group and normal blood lipid group according to whether the blood lipid is normal, and was divided into the following four groups: hypercholesterolemia group, hypertriglyceridemia group, mixed hyperlipidemia group and low high-density lipoprotein cholesterol (HDL-C) group according to clinical classification. The clinical and pathological features in different groups were analyzed, and the risk factors of dyslipidemia were analyzed by using multivariate logistic regression analysis.Results:A total of 252 children with IgAN were enrolled in this study, including 169 males and 83 females, with a male/female ratio of 2.04∶1 and an age of (9.3±3.1) years. Among them, 34.5% IgAN children were complicated with hypertension, and 170 cases (67.5%) were in dyslipidemia group, 82 cases (32.5%) in normal blood lipid group. According to clinical classification, the children in dyslipidemia group were divided into hypercholesterolemia group (58 cases, 23.0%), hypertriglyceridemia group (16 cases, 6.3%), mixed hyperlipidemia group (77 cases, 30.6%) and low HDL-C group (19 cases, 7.5%). The systolic blood pressure, diastolic blood pressure, proportion of hypertension, blood urea nitrogen, uric acid and urinary protein in dyslipidemia group were higher than those in normal blood lipid group (all P<0.05), and the levels of serum albumin, blood IgA and estimated glomerular filtration rate (eGFR) were less (all P<0.05). The proportion of IgAN children in chronic kidney disease (CKD) stage 1 and CKD stage 2-5 with dyslipidemia was 65.0% and 84.4% respectively, and the proportion of IgAN children with CKD stage 2-5 in dyslipidemia group was higher than that in normal group ( P<0.05). The dyslipidemia group had a higher proportion of Lee Ⅲ-V grade than normal blood lipid group ( P<0.01). The results of Oxford pathological classification showed that the proportions of M1 and E1 in dyslipidemia group were higher than those in normal lipid group (all P<0.05), and there was no significant difference in segmental glomerulosclerosis, tubular atrophy or interstitial fibrosis and crescent between the two groups (all P>0.05). The comparison results between groups with different types of dyslipidemia showed that systolic blood pressure, diastolic blood pressure, serum uric acid and urinary protein in the mixed hyperlipidemia group were higher than those in other groups (all P<0.05), and the serum albumin level was less ( P<0.01). The results of Oxford pathological classification showed that the proportion of E1 in hypercholesterolemia group and mixed hyperlipidemia group was higher ( P<0.05). Multivariate logistic regression analysis showed that hypertension ( OR=2.734, 95% CI 1.327-5.632, P=0.006) and low serum albumin ( OR=0.838, 95% CI 0.791-0.889, P<0.001) were the risk factors of dyslipidemia in children with IgAN. Conclusions:In our center, 67.5% IgAN children are accompanied by dyslipidemia. The clinical manifestations and pathological changes of these dyslipidemia children are more severe than those with normal blood lipid, and the IgAN children with mixed hyperlipidemia are more notable. Hypertension and low serum albumin are the risk factors of dyslipidemia in children with IgAN.

Objective:To explore clinicopathological features and prognosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in children induced by antithyroid drugs.Methods:The clinicopathological features, treatment and prognosis of 3 children with AAV induced by antithyroid drugs in the Department of Pediatric Nephrology and Rheumatology of the First Affiliated Hospital of Sun Yat-sen University were analyzed retrospectively, and the literatures were reviewed.Results:(1) Among the 3 cases, there were 2 females and 1 male, whose ages were 12.6, 13.9 and 13.1 years old, respectively. All patients had medication history of propylthiouracil (PTU) and/or methimazole (MMI) before onset. Initial manifestation was pallor and renal involvements with nephrotic proteinuria, hematuria and renal function abnormality, while 2 of them had hypertension. Extrarenal manifestations were also presented: case 1 presented with rash, arthralgia and cardiac insufficiency; case 2 had brain involvement with repeated convulsions; case 3 presented with arthralgia and lung involvement. They were all tested positive for p-ANCA and MPO-ANCA. Initial renal histopathology of the 3 cases were consistent with ANCA-associated glomerulonephritis, which were classified into sclerosis, crescentic and mixed class respectively. After 8 months of treatments, repeated renal biopsy of case 3 had demonstrated progression to sclerosis class. Antithyroid drugs (PTU or MMI) were discontinued in 3 cases, and the children were all treated with corticosteroid combined with intravenous pulse cyclophosphamide therapy. Plasma exchange was performed in case 2 and case 3 due to rapidly progressive glomerulonephritis and disease recurrence (suspected pulmonary hemorrhage), respectively. Case 3 was treated with rituximab combined with mycophenolate mofetil after recurrence. The extrarenal symptoms relieved quickly after treatments in all cases. P-ANCA and MPO-ANCA became negative in case 1 and case 2 after 6 months of treatments but they were persistently positive in case 3. Three cases were followed up for 24 months, 10 months and 12 months, respectively: case 1 develop chronic kidney disease (CKD) stage 2 with normal urinalysis; case 2 develop CKD stage 5 and had sudden death at home at 10-month follow-up; case 3 develop CKD stage 4 with nephrotic proteinuria and microscopic hematuria. (2) There were totally 30 pediatric cases with AAV induced by PTU and MMI, including 27 reported cases in the literature and 3 cases in this study. Symptoms of AAV appeared in children after an average administration of (37.5±4.0) months of PTU (range from one month to 96 months and 8 months of MMI alone). Kidney (28 cases, 93.3%) and lung (12 cases, 40.0%) were commonly involved, while brain (2 cases, 6.7%) was rarely involved. The pathological changes of kidney were crescent nephritis (5/23) and necrotizing pauci-immune complex nephritis (11/23). The total remission rate was 93.3% (28/30) after antithyroid drugs withdrawal and treatment with corticosteroids and immunosuppressive therapy, however, there were still severe cases with progression to CKD stage 5, and death. (3) Thirty cases were divided into complete response group ( n=19) and incomplete response group ( n=11) according to the treatment response. Compared with complete response group, the proportions of massive proteinuria (8/11 vs 5/19), fibrinoid necrosis (7/9 vs 4/14), deposition of immune complex in renal tissues (6/9 vs 2/14) and administration of immunosuppressants (10/11 vs 5/19), and degree of tubular atrophy (0/1/2/3 grade, 2/4/2/1 vs 9/5/0/0) in incomplete response group were higher (all P<0.05). Conclusions:PTU and MMI can both induce AAV in children, and AAV may occur after short-term course of administration. Kidney and lung are commonly involved while brain involvement is rarely seen. Timely withdrawal of antithyroid drugs and proper treatments with corticosteroids and immunosuppressants can result in high remission rate, though there are still some severe cases. Nephrotic-range proteinuria, renal fibrinoid necrosis, immune-complex deposition and tubular atrophy may be the risk factors of AAV for poor prognosis.

The paper reported a patient under maintained hemodialysis for 11 years, with a large mass appeared in the right thigh after local injury. The mass was clinically considered as tumoral calcinosis combined with clinical, imaging and pathological findings. Several treatments such as enhancing dialysis adequacy, low calcium dialysate, calcimimetic agent, non-calcium- phosphorus binding agents, parathyroidectomy and intravenous infusion of sodium thiosulfate could not vanish the mass. Finally, the lump was surgically removed. The treatment of tumoral calcinosis in the hemodialysis patient can provide a instruction for similar situations in clinical practice.

Objective:To study the variation in activity in patient′s body with differentiated thyroid cancer (DTC) treated with 131I and external dose level, analyze the relationship between the both, and estimate the correction factor for the dose equivalent rate for the patients with residual activity of 400 MBq. Methods:A total of 43 DTC patients who received 131I therapy for the first time after total thyroidectomy were studied. The dose was 1 850-3 700 MBq and average dose was (2 405±777) MBq. The measurements of residual activity in patient′s body and of dose equivalent rate at 0.3, 1 and 3 m in front of the patients were performed at 2, 6, 20, 22, 24, 27, 30, 44, 46, 48, 54, 68 and 72 h after administration of 131I. Results:The residual activity in patient′s body after 131I therapy varied with time as a function of A= A0 (1.033 16e -0.062 4t+ 0.017 17). It can be estimated that the effective half-life of DTC patients treated with thyroid remnant 131I ablation therapy is 12.19 h. It needs only 26.4-38.9 h to reduce the internal activity to the 400 MBq. The functions of variation with time of normalized dose equivalent rate at 0.3, 1, and 3 m away from patients were: H· 0.3=127.220 7e -0.054 8t+ 3.765 71; H· 1=30.225 8e -0.064 4t+ 0.824 67; and H· 3=4.161 9e -0.061 5t+ 0.167 97, respectively. There was a positive correlation between residual activity and dose equivalent rate at 1 m ( r=0.982, P<0.05), and the function is H· 1=0.025 A+ 1.245. When residual activities in DTC patient′s body were 1 000, 700 and 400 MBq, the corresponding dose equivalent rates at 1 m from patients were 26.2, 18.7 and 11.2 μSv/h, respectively. The correction factors for dose equivalent rate at 0.3, 1 and 3 m from patients with 400 MBq were 0.25, 0.49 and 0.70, respectively. Conclusions:DTC patients with administration of 131I activity below 3 700 MBq need only to be hospitalized for two days to reach the discharge standards. When the residual activity in DTC patient′s body drops to 400 MBq, the dose equivalent rate at 1 m is far less than 25 μSv/h. Simply using the point source formula to estimate the dose equivalent rate around the patient will result in overestimation. Therefore, the correction factor used in the estimation of radiation doses to patients by using the formula needs to be further studied so as to make the model-based estimated result more consistent with the actual situation.

AIM: To evaluate the efficacy of phacoemulsification with intraocular lens implantation(PEI)combined with goniosynechialysis(GSL)and goniotomy(GT)under direct vision with gonioscope in the treatment of advanced primary angle-closure glaucoma(PACG)combined with cataract.METHODS: Retrospective case series study. A total of 62 patients(65 eyes)with advanced PACG combined with cataract who were treated in the Second Hospital of Anhui Medical University from December 1, 2021 to March 31, 2023 were enrolled, and they were divided into two groups according to different surgical methods. The control group(32 cases, 33 eyes)received PEI+GSL, whereas the observation group(30 cases, 32 eyes)received PEI+GSL+GT. The intraocular pressure(IOP), best corrected visual acuity(BCVA)and the number of anti-glaucoma medications of the two groups before surgery and at 1 d, 1 wk, 1, 3, and 6 mo after surgery were evaluated. In addition, the visual field, cup-to-disc ratio(C/D), angle open range, anterior chamber depth, and average thickness of retinal nerve fiber layer(RNFL)were evaluated before and 6 mo after surgery.RESULTS: There were significant differences in IOP and lowering range of average IOP at 6 mo between the PEI+GSL+GT group(16.68±2.65, 11.12±8.53 mmHg)and the PEI+GSL group(18.71±2.51, 8.32±4.17 mmHg; P<0.05), and there was no difference in the rate of IOP reduction(44.57%±21.79% and 35.20%±17.94%, P>0.05). The number of anti-glaucoma medications, BCVA, anterior chamber depth, and angle closure range were improved in the two groups at 6 mo after operation(all P<0.01). The number of medication reductions and the range of angle opening at 6 mo after surgery in the PEI+GSL+GT group were significantly higher than those in the PEI+GSL group(P<0.05), and there was no difference in the other indicators between the two groups(all P>0.05). There was no difference in the mean deviation of visual field, C/D and average thickness of RNFL between the two groups at 6 mo after operation compared with those before operation(all P>0.05). The complete surgery success rate of the PEI+GSL+GT group was 81%(26/32), and the conditional success rate was 94%(30/32); while those rates of the PEI+GSL group were 58%(19/33)and 76%(25/33), respectively. There were statistical significance in the success rate of surgery between the two groups(complete success rate χ2=4.275, P=0.039; conditional success rate χ2=4.040, P=0.044). No vision-threatening complications and another surgery occurred in either group.CONCLUSION: The study showed that for patients with advanced PACG with cataract, PEI+GSL+GT is more effective than PEI+GSL.