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Objective: To investigate the etiology, complications, and prognostic factors of stage 5 chronic kidney disease (CKD5) in children. Methods: A case series study was conducted to retrospectively analyze the general situation, clinical manifestations, laboratory tests, genetic testing, and follow-up data (until October 2022) of 174 children with CKD5 who were diagnosed and hospitalized at the Children's Hospital of Chongqing Medical University from April 2012 to April 2021. The characteristics of complications in the children were compared based on age, gender, and etiology. Based on the presence or absence of left ventricular hypertrophy (LVH), patients were divided into LVH group and non LVH group for analyzing the influencing factors of cardiovascular disease. Patients were also divided into death group and survival group, peritoneal dialysis group and hemodialysis group based on the follow-up data for analyzing the prognostic factors. The chi-square test, independent sample t -test, Fisher exact probability test, Mann-Whitney U test and Kruskal Wallis test were used to analyze data among different groups. Multivariate Logistic regression analysis was used to identify the prognostic factors. Results: A total of 174 children with CKD5 were enrolled in the study (96 boys and 78 girls), aged 11.2 (8.2, 13.0) years. Congenital kidney and urinary tract malformations (CAKUT) were the most common causes of the CKD5 (84 cases, 48.3%), followed by glomerular diseases (83 cases, 47.7%), and among which 28 cases (16.1%) were hereditary glomerular diseases. The common complications of CKD5 included anemia (98.2%, 165/168), mineral and bone disorder in chronic kidney disease (CKD-MBD) (97.7%, 170/174), lipid metabolism disorders (87.5%, 63/72), hypertension (81.4%, 127/156) and LVH (57.6%,57/99). The incidences of hypertension in primary glomerular disease were higher than that in CAKUT(93.8%(30/32) vs. 73.7%(56/76), χ 2 =5.59, P <0.05). The incidences of hypertension in secondary glomerular disease were higher than that in CAKUT and that in hereditary kidney disease (100.0%(20/20) vs. 73.7%(56/76), 68.2%(15/22), both P <0.05). The incidence of hypocalcemia in CAKUT, primary glomerular disease, and hereditary kidney disease was higher than that in secondary glomerular disease (82.1%(69/84), 88.2%(30/34), 89.3%(25/28) vs. 47.6%(10/21), χ 2 =10.21, 10.75, 10.80, all P =0.001); the incidence of secondary hyperparathyroidism in women was higher than that in men (80.0%(64/80) vs. 95.0%(57/60), χ 2 =6.58, P =0.010). The incidence of LVH in children aged 6-<12 was higher than that in children aged 12-18 (73.5%(25/34) vs. 43.1%(22/51), χ 2 =7.62, P =0.006). Among 113 follow-up children, the mortality rate was 39.8% (45/113). Compared to the survival group, the children in the death group had lower hemoglobin, higher blood pressure, lower albumin, lower alkaline phosphatase and higher left ventricular mass index ((67±19) vs . (75±20) g/L, 142 (126, 154) vs . 128(113, 145) mmHg(1 mmHg=0.133 kPa), (91±21) vs . (82±22) mmHg, 32 (26, 41) vs . 40 (31, 43) g/L, 151 (82, 214) vs . 215 (129, 37) U/L, 48 (38, 66) vs . 38(32, 50) g/m 2.7 , t =2.03, Z =2.89, t =2.70, Z =2.49, 2.79, 2.29,all P <0.05), but no independent risk factors were identified (all P >0.05). The peritoneal dialysis group had better alleviation for anemia, low calcium, and high phosphorus than the hemodialysis group ((87±22) vs. (72±16) g/L, (1.9±0.5) vs. (1.7±0.4) mmol/L, (2.2±0.7) vs. (2.8±0.9) mmol/L, t =2.92, 2.29, 2.82, all P <0.05), and the survival rate of the peritoneal dialysis group was significantly higher than that of the hemodialysis group (77.8% (28/36) vs . 48.4% (30/62), χ 2 =8.14, P =0.004). Conclusions: CAKUT is the most common etiology in children with CKD 5, and anemia is the most common complication. The incidence of complications in children with CKD 5 varies with age, gender and etiology. Anemia, hypertension, hypoalbuminemia, reduced alkaline phosphatase and elevated LVMI may be the prognostic factors in children with CKD5. Peritoneal dialysis may be more beneficial for improving the long-term survival rate.

Objective: To retrospectively analyze the clinical features and survival analysis of primary intestinal extranodal NK/T-cell lymphoma nasal type(ENKTL). Methods: Patients with intestinal lymphoma at the First Affiliated Hospital of Air Force Military Medical University were collected from January 2009 to December 2019, and those with primary intestinal ENKTL screened. The general situation, main symptoms, ECOG scale, lactic dehydrogenase(LDH), and β(2)-microglobulin(β(2)-MG)in the serum, lesion site and form, numbers of extranodal invasion, Lugano stage, pathological features, and lifetime and survival outcomes were evaluated. Results: In total, 34 patients with confirmed diagnosis of primary intestinal ENKTL were identified. The incidence rate of primary intestinal ENKTL is 16.2% (34/210). A total of 26 patients were men and 8 were women with the median age at diagnosis of 45 (range, 20-69)years and patients younger than 60 years account for 85.3%. It included abdominal pain (76.5%), diarrhea(20.6%), hematochezia(29.4%), and abdominal mass(2.9%), 85.3% with B-symptoms, and abnormally elevated LDH in the serum(73.5%). The lesion sites included large intestine(52.9%), small intestine(29.4%), and both of them(17.7%). The lesion forms were featured by mass(8.8%), ulcer(32.4%), diffuse infiltration(2.9%), and hybrid(55.9%). Lugano stage included stage Ⅰ/Ⅱ (70.6%)24 patients and stage Ⅳ(29.4%)10 patients. About 94.1% patients were EBER positive. The median survival time was 60 days. The overall survival rate had significant differences on ECOG scale, LDH in the serum, IPI score and complications( P =0.037, 0.009, 0.002, and 0.000, respectively). Conclusion: Primary intestinal ENKTL was commonly observed in men at young or middle age, and the most common symptom was abdominal pain, often with B-symptoms, abnormally elevated LDH in the serum. The most common site was the large intestine. The lesion forms were mainly featured by the hybrid and ulcer. It seemed that patients with IPI low-risk group and without any complication would have longer survival time.

Objective: To summarize the clinical and genetic characteristics of children with mitochondrial epilepsy. Methods: Clinical data of 62 children who were clinically and genetically diagnosed with mitochondrial epilepsy by the Department of Neurology, Beijing Children's Hospital from October 2011 to December 2018 were analyzed retrospectively, and the control of epilepsy was followed up. T test or χ(2) test were used to analyze the related factors affecting the prognosis of epilepsy between the effective group and the ineffective group. Results: Of the 62 patients, 33 were male and 29 were female. The age of onset was 3.38 (0-12.00) years; for the type of seizures, 68% (42/62) of the patients had focal seizures, generalized or secondary generalized tonic-clonic seizures were seen in 32% (20/62), myoclonic seizures in 23% (14/62), spastic seizures in 7 cases, tonic seizures in 4 cases, absence seizure, atonic seizure and clonic seizure in 1 case each; 16 cases (26%) had status epilepticus, of whom 6 cases had epilepsia partialis continua; 52% (32/62) had 2 or more types of seizures. The clinical phenotypes were mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) in 29 cases, Leigh syndrome (LS) in 11 cases, combined oxidative phosphorylation deficiency in 6 cases, myoclonus epilepsy with ragged-red fibers in 5 cases, Alpers syndrome in 4 cases, pontocerebellar hypoplasia type 6 and mitochondrial DNA depletion syndrome 9 in 2 cases each, mitochondrial complex Ⅰ deficiency nuclear type 20, progressive cavitating leukoencephalopathy, and biotinidase deficiency in 1 case each. Of the 62 cases, 40 cases (65%) had mitochondrial DNA (mtDNA) variations, of which 26 cases had m.3243A>G variants, 6 cases had m.8344A>G variants, and 3 cases had m.8993T>G/C variants, m.3271T>C, m.3481G>A, m.3946G>A, m.13094T>C, m.14487T>C variant was in 1 case each; nuclear DNA (nDNA) variations were identified in 22 cases (35%), of which 7 cases carrying variations in mitochondrial ammonia acyl tRNA synthetase coding gene, mutations in POLG and the gene encoding complex Ⅰ were in 4 cases each, variations in SUCLG1 and SDHA genes were in 2 cases each, and variations in PDHA1, BTD and TRIT1 genes were in 1 case each. Forty-three patients were followed up, and the follow-up time was 20 (3-84) months. According to the follow-up results, the anti-epilepsy treatment was effective in 19 cases (44%) and ineffective in other 24 cases (56%). The onset age of the effective group was 3.42 (0-11.50) years and that of the ineffective group was 0.92 (0-9.50) years. The onset duration of the effective group was 0 (0-7.00) years and that of the ineffective group was 0 (0-4.83) years. There was no significant difference between the effective group and the ineffective group ( t= 1.662, 0.860; P= 0.104, 0.395). In the effective group and the ineffective group, 12 cases and 9 cases used less than 2 kinds of antiepileptic drugs, 7 cases and 15 cases used more than or equal to 2 kinds of antiepileptic drugs, 13 and 15 cases had first epilepsy, 6 and 9 cases had non-first epilepsy, 14 and 11 cases had mtDNA variation, 5 and 13 cases had nDNA variation, respectively. There was no significant difference between the two groups (χ(2)=2.794, 0.164, 3.380; P= 0.095, 0.686, 0.066). Conclusions: The types of seizures with mitochondrial epilepsy in children varied, with focal motor seizures being the most common, followed by generalized or secondary generalized tonic-clonic seizures. Most children have more than two types of seizures. MELAS is the most common clinical phenotype, followed by LS; mtDNA variation is the dominant gene variation, of which m.3243A>G variation is the most common hotspot variation, followed by gene variation encoding mitochondrial aminoacyl tRNA synthase.

Objective: To investigate the prognostic values of HIF-1α, APE1, VEGF, and COX-2 protein expressions and their predictive value of tumor necrosis rate and prognosis in osteosarcoma, as well as their interrelationships., Methods: Formalin-fixed paraffin-embedded tissue samples were obtained from patients with osteosarcoma. Immunohistochemical assay was performed in pre-chemotherapy samples to determine the HIF-1α, VEGF, APE1, and COX-2 protein expression levels. Hematoxylin-eosin staining was used in post-operative samples to determine the tumor necrosis rate. Univariate and multivariate analyses were used to assess the impact of protein expression on prognosis., Results: Tumor tissues were obtained from 49 patients. Their median follow up was 29 months. HIF-1α was significantly correlated to every protein we tested: VEGF (P = 0.032), APE1 (P < 0.001), and COX-2 (P < 0.001). HIF-1α protein expression had a significant impact on disease free survival (P = 0.006). Expression of HIF-1α had a sensitivity of 64.7% and a specificity of 71.9% for poor pathological response (< 90% of tumor necrosis) versus good pathological response to chemotherapy (≥ 90% necrosis)., Conclusion: Expression of HIF-1α is a predictor of tumor response to neoadjuvant chemotherapy and outcome in osteosarcoma and is correlated with VEGF, APE1, and COX-2 expression.

Objective: To analyze the impact of radical surgery with different extent of lymph nodes dissection on the prognosis of colorectal carcinoma patients and to see if lymph nodes adjacent to mesenteric artery root should be excised., Methods: Data of 1409 cases with colorectal carcinoma treated in Shanghai Cancer Hospital during 1985-2000 were collected. These patients had primary colorectal carcinoma treated by radical surgery with different extent of lymph nodes excision. They were divided into two groups: in group D3 the lymph nodes adjacent to mesenteric artery root were excised (n = 857), while in group D2 (n = 552) were not. The time of follow-up was 6 approximately 289 months (median: 48 months)., Results: The 1-, 3-, 5-, 10-year total survival rates (TS) in group D3 patients were 90.3%, 81.4%, 77.0% and 73.0%, respectively. The 1-, 3-, 5-, 10-year tumor-free survival rates (TFS) in group D3 patients were 89.9%, 79.4%, 74.5% and 70.3%, respectively. In group D2 patients, the 1-, 3-, 5-, 10-year TS were 91.04%, 84.12%, 79.33% and 76.17%, and those of TFS were 90.0%, 82.7%, 76.0% and 71.8%, respectively. The differences in TS and TFS in the two groups of patients were not significant according to Kaplan-Meier analysis (P > 0.05). During the follow-up period, 42 patients in group D3 had local recurrence (4.9%), while in group D2 the rate of local recurrence was 5.4% (P > 0.05). Metastases developed in 79 cases in group D3 and in 60 cases in group D2 (P > 0.05). Multivariate analysis revealed that the excision of lymph nodes adjacent to mesenteric artery pedicle did not statistically correlate with recurrence, metastasis and survival time after radical operation of colorectal cancer., Conclusion: Excision of lymph nodes adjacent to the mesenteric artery root has no significant impact on prognosis and is unnecessary in the radical surgery for colorectal carcinoma.

Objective: To investigate the effect of gene Af116609 on gastric cancer multi-drug resistance (MDR) by introducing it into gastric cancer multi-drug resistant (MDR) cell line SGC7901/VCR., Methods: Gene Af116609 was cloned from SGC7901/VCR by RT-PCR and its differential expression between gastric cancer MDR cells and its parental cells was displayed by Northern blot. The gene was introduced to gastric cancer cells by transfection of recombinant eukaryotic expression vector by electroporation. MTT assay in vitro was applied to investigate its effect on multi-drug resistance phenotype of gastric cancer cells., Results: The full length CDS of gene Af116609, as long as 327 bp, was cloned from gastric cancer MDR cell line SGC7901/VCR and its sequence was coincident with the hypothetical gene Af116609 in GenBank. It was overexpressed in MDR cells than its parental cells at mRNA level. In the MTT assay in vitro, the drug sensitive cells transfected with sense eukaryotic expression vector showed upregulated targeted gene, with increased resistance to vincristine, 5-fliorouracil and arabinoside, and decreased resistance to adriamycin, but no influence on resistance to methotrexate. However, the drug resistant cells transfected with anti-sense eukaryotic expression vector, showed down regulated targeted gene, with less resistance to all the five anticancer drugs to different degrees., Conclusion: Gene Af116609 is related to MDR phenotype of gastric cancer cells and may become a candidate molecular target to reverse the MDR of gastric cancer.

Objective: To study the expression and possible function of RhoA in human gastric cancer cell lines., Methods: The expression of RhoA in human gastrointestinal cancer cell lines was detected by Western blot. Antisense plasmid of RhoA was constructed by pGEFL and transferred into gastric cancer cell line AGS by lipofectamine. Cell survival was examined by MTT assays, and cell cycle was detected by flow cytometry., Results: The expression of RhoA protein in 10 different kinds of human cancer cell lines was much higher than that in immortalized human intestinal epithelial cell line. After being transfected with antisense RhoA, with the decrease in RhoA protein expression, the growth rate of AGS was inhibited, and the number of cells in S phase was increased by 14%., Conclusion: RhoA is overexpressed in many human cancer cell lines. Some of the malignant characteristics of a gastric cancer cell line can be partially reversed by inhibiting RhoA expression.

Objective: To investigate the differential expression of RPL6/Taxreb107 between drug-resistant gastric cancer cell line SGC7901/ADR and gastric cancer cell line SGC7901 as well as its correlation with multiple-drug resistance (MDR) in gastric cancer cells., Methods: Total RNA was extracted from SGC7901 and SGC77901/ADR, with internal control RT-PCR, Northern blot, gene cloning and expression, construction of eukaryotic expression vector, gene transfection by electroporation. The accumulation and retention of ADR in transiently transfected cell was detected by flow cytometry., Results: The internal control RT-PCR and Northern blot showed high RPL6/Taxreb107 expression in SGC7901/ADR cell line. Sense and antisense eukaryonic expression vectors demonstrated by double enzyme digestion were successfully transfected into gastric cancer cell line SGC7901 and SGC7901/ADR respectively by electroporation. The accumulation and retention of ADR detected 48 hours after transfection showed that RPL6 gene had shown effect on drug resistance in gastric cancer cell., Conclusion: The high expression of RPL6/Taxreb107 in drug resistant gastric cancer cell shows its correlation with multiple-drug resistance in gastric cancer.

Seven cases of primary parovarian borderline tumors are presented, Comprising 1.6% of 451 cases of parovarian cysts treated. The age range of the 7 cases was from 16 to 60, with a mean value of 37 years. Abdominal mass, lower abdominal pain or discomfort were chief clinical complaints. In one case, because of amenorrhea of 2 months' duration, the preoperative diagnosis was mistaken to be extra-uterine pregnancy. Surgical examination revealed normal appearance of ovaries and fallopian tubes. Smooth-surfaced cystic masses arising from the broad ligaments were found in all of the 7 cases, their size ranged from 3cm x 2cm x 1.5cm to 8cm x 8cm x 7cm. Internal papillary projections single or multiple, and clear serous fluid were seen in all of the 7 cystic specimens. Microscopically the cyst wall and papillary projections were lined with stratified cuboid or columnae epithelium. Abundant and complicated ramifications of the projections were seen. The nuclei showed slight to moderate metaplasia; mitoses were rare. 6 of the cases originated from paramesonephros and 1 ease was of mesonephric origin. The seven patients were living and well during a postoperative follow-up period of 12 months to 11 years. Literature review on primary parovarian borderline tumors, their clinical presentation, pathological characteristics, therapy and prognosis are presented and discussed.

Flow cytometry and image analysis technique were used to quantitate the nuclei of various soft tissue tumors. A single representing section from soft tissue sarcoma was used for histologic grading. Histologic and cytometric comparative analyses showed that all 21 benign tumors were diploid. Among 62 cases of soft tissue sarcoma, 45 (73%) were aneuploid. There was a significant difference in the nuclear area between benign and malignant tumors (P less than 0.01), diploid and aneuploid tumors (P less than 0.05). The two new techniques are valuable in cellular quantitative measurement for soft tissue tumors.

Ultrastructures of 11 cases of neuroblastoma and ganglioneuroblastoma were observed. Ultrastructurally, the neuroblastoma could be classified into four types: immature, poorly differentiated, differentiated and ganglioneural cell. Neurosecretory granules (NSG) of round or oval shapes with various diameters in the tumor cells and neural processes were observed. The presence of NSG in the tumor tissue is one of the pathognomic features for neuroblastoma. Comparing and study of the relation between the ultrastructural findings and the excretory patterns of the urinary catecholamine as well as the prognosis, it was shown that the tumor of the differentiated type and the presence of abundant NSG lead to a good prognosis. Some slight differences in ultrastructure were found in neuroblastoma specimens from children of various ages. Infants under 18 months had a longer survival time, which is related to the differentiation and other features of the tumor cells.