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Objective To analyze the Oxford classification (MESTC) and the International Study of Kidney Disease in Children (ISKDC) classification for evaluating the clinical manifestations,histological lesion and short-term prognosis of children with Henoch-Sch(o)nlein purpura nephritis (HSPN).Methods According to the Oxford classification and ISKDC classification,the histological lesions of children with HSPN diagnosed by renal biopsy from Beijing Children's Hospital affiliated to Capital Medical University from January 2018 to December 2018 were re-evaluated.The renal biopsy specimens of the selected subjects were scored according to the Oxford classification and the ISKDC classification.According to whether the first symptom was combined with renal performance,MESTC score and ISKDC classification,children were grouped.The differences in clinicopathological manifestations between the groups were compared.Correlation between MESTC and ISKDC grades was analyzed by nonparametric test rank correlation.Kaplan-Meier survival curve and Log-rank test were used to compare the difference of proteinuria remission rate between the two groups.Univariate and multivariate Cox regression equations were used to analyze the influencing factors of the proteinuria remission rate.Results A total of 78 children with HSPN were enrolled.There were 37 male patients (47.4％) with age of (10.4+2.9) years.When the patients were divided according to MESTC scores and ISKDC classification,the results showed that the proportion of children with nephrotic-range proteinuria in the group of endocapillary hypercellularity (E1,P=0.008),segmental glomerulosclerosis (S1,P=0.015) and ISKDC Ⅲ (P=0.041) was higher than that of E0,S0 and ISKDC Ⅱ groups.The proportion of children with E1 (P=0.015),crescents (C1&C2,P=0.025) or ISKDC Ⅲ (P=0.017) that had been treated with high-dose methylprednisolone was higher.The result of Kaplan-Meier survival curve showed more difficult for proteinuria remission in children with C2 are than C0&C1 group (P=0.026),while no difference were found when children were grouped by M,E,S,T and ISKDC.Multivariate Cox regression analysis showed that the C2 (HR=0.143,95％C1 0.020-1.046,P=0.055) might be a risk factor for proteinuria remission,while the P value was close to 0.05.Conclusions Children with HSPN scored as ISKDC Ⅲ,E1 and S1 are more likely to show nephrotic-range proteinuria.C2 may indicate that patients are more difficult to achieve proteinuria remission.
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Objective@#To analyze the Oxford classification (MESTC) and the International Study of Kidney Disease in Children (ISKDC) classification for evaluating the clinical manifestations, histological lesion and short-term prognosis of children with Henoch-Schönlein purpura nephritis (HSPN).@*Methods@#According to the Oxford classification and ISKDC classification, the histological lesions of children with HSPN diagnosed by renal biopsy from Beijing Children's Hospital affiliated to Capital Medical University from January 2018 to December 2018 were re-evaluated. The renal biopsy specimens of the selected subjects were scored according to the Oxford classification and the ISKDC classification. According to whether the first symptom was combined with renal performance, MESTC score and ISKDC classification, children were grouped. The differences in clinicopathological manifestations between the groups were compared. Correlation between MESTC and ISKDC grades was analyzed by nonparametric test rank correlation. Kaplan-Meier survival curve and Log-rank test were used to compare the difference of proteinuria remission rate between the two groups. Univariate and multivariate Cox regression equations were used to analyze the influencing factors of the proteinuria remission rate.@*Results@#A total of 78 children with HSPN were enrolled. There were 37 male patients (47.4%) with age of (10.4±2.9) years. When the patients were divided according to MESTC scores and ISKDC classification, the results showed that the proportion of children with nephrotic-range proteinuria in the group of endocapillary hypercellularity (E1, P=0.008), segmental glomerulosclerosis (S1, P=0.015) and ISKDCⅢ(P=0.041) was higher than that of E0, S0 and ISKDCⅡ groups. The proportion of children with E1 (P=0.015), crescents (C1&C2, P=0.025) or ISKDCⅢ(P=0.017) that had been treated with high-dose methylprednisolone was higher. The result of Kaplan-Meier survival curve showed more difficult for proteinuria remission in children with C2 are than C0&C1 group (P=0.026), while no difference were found when children were grouped by M, E, S, T and ISKDC. Multivariate Cox regression analysis showed that the C2 (HR=0.143, 95%CI 0.020-1.046, P=0.055) might be a risk factor for proteinuria remission, while the P value was close to 0.05.@*Conclusions@#Children with HSPN scored as ISKDCⅢ, E1 and S1 are more likely to show nephrotic-range proteinuria. C2 may indicate that patients are more difficult to achieve proteinuria remission.
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Objective@#To investigate the clinical, pathological features and differential diagnosis of testicular Leydig cell hyperplasia (LCH) .@*Methods@#Clinical data, histological features, immunohistochemical findings, ultrastructural characteristics and follow-up data were analyzed in three cases of LCH. The cases were collected from 2011 to 2014 at Beijing Children′s Hospital. A literature review was performed.@*Results@#Two males (1.8 years and 2.9 years of age) showed isosexual pseudoprecocity with elevated serum testosterone. Imaging study showed bilateral testicular enlargement with multiple small nodules in the parenchyma. Another 13 years-old patient showed male pseudohermaphroditism and cryptorchism. Gross examination showed the bilateral markedly enlarged testis without discrete lesion. Histologically, LCH was seen in both nodular and diffuse patterns without destruction of seminiferous tubules. Adjacent spermatogenesis was noted. Immunohistochemically, the Leydig cells were positive for inhibin, calretinin and Melan A and ultrastructural analysis showed enriched cytoplasmic endoplasmic reticulum. Two cases had followed up for 7 years. One patient was symptom-free and one was stable.@*Conclusion@#LCH is a rare benign condition, which is easily misinterpreted as testicular tumor or non-neoplastic diseases. Clinical presentation, imaging study and pathological evaluation are required for the diagnosis.
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Objective@#To investigate the clinicopathological and ultrastructural characteristics of Langerhans cell histiocytosis (LCH) in children.@*Methods@#A total of 345 cases of LCH from the Department of Pathology, Beijing Children Hospital from January 2012 to March 2016 were investigated by hematoxylin-eosin stain, EnVision immunohistochemistry and transmission electron microscopy.@*Results@#The rate of primary clinical diagnosis of LCH in children was 46.0%(210/457). Among 345 patients of LCH, 213 were male and 132 were female, the male to female ratio was 1.6∶1.0, and the median age was 21 months (range from 2 days after birth to 13.3 years). There were total 597 lesions, including bony lesions (258, 43.2%), skin lesions (206, 34.5%) , followed by lymph node (16, 2.7%), lung (28, 4.7%), liver (25, 4.2%) and head-neck (50, 8.4%). Single organ system LCH (SS-LCH) was seen in 295 cases (85.5%) and 50 cases (14.5%) presented with multiple organ system involvement LCH (MS-LCH). There was no significant difference in age and gender between SS-LCH and MS-LCH groups. Regarding sites, more lesions were seen in bone and skin in SS-LCH group, in contrast lymph node, lung, liver and head-neck involvements were often seen in MS-LCH group. Immunohistochemically, the expression of CD1a and Langerin was seen in 99.7% (341/342) and 98.8% (338/342) of the cases respectively. The diagnostic rates by light and transmission electron microscopy were 98.8% (341/345) and 97.4% (112/115) respectively (P>0.05).@*Conclusions@#LCH of children occurs predominantly in SS-LCH pattern, frequently involving bone, skin, lymph node, lung and liver and other sites with unique histopathological, immunophenotypical and ultrastructural features. Accurate diagnosis relies on the morphology, immunophenotype and ultrastructural features. Further refinement of specimen processing may improve the accuracy of pathological diagnosis.
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Objective To investigate the clinical features of pedistric Littre hernia.Methods Clinical data of 11 cases of Littre hermia admitted from January 2002 to December 2010 was studied retrospectively.Results The diagnosis of Littre hernia was all established by laparotomy.All of the 11 cases were boys,the median age was 1.2 years (22 days to 3 years and 7 months).The main clinical features were:painful,irregular and nonresetable mass in the groin area (11/11),vomiting in 6 cases (6/11 ),fever (＞38.5 ℃ ) in 4 cases (4/11 );X-ray showed intestinal obstruction in 9 cases (9/11 ),Ultrasound found mixed mass in the groin area in 11 cases ( 11/11 ),pouch-like structure were found in 6 cases (6/11).Eight cases (8/11) presented with elevated WBC ( ＞ 10000).Palpable lobulated structure were fell in 5 cases (5/11).All cases of Littre hernia were successfully operated on,Meckel diverticulum perforation was found in 2 cases ( 2/11 ),Meckel diverticulum adhered to the hernia sac in 8 eases (8/11).All patients were cured by surgery,and postoperative follow-up (10ms-8y) found no recurrence.Conclusions Pediatric Littre hernia is rare,preoperative diagnosis is difficult.Avoiding preoperative violent reduction and intraoperative injuring Meekel's diverticulum or the small bowel helps improve the cure rate of Littre hernia in children.