Shanghai Medical Center for Children, jointly set up by the municipal government and the World Health Foundation of America, possesses advanced intelligence hospital buildings and medical facilities. The authors gave a detailed account of the centers practice in implementing reform with modern ideas of hospital management. Specific ways include: ①improving the overall quality by training all workers and staff of the management contingent; ②strengthening medical quality control via orienting medical service towards the needs of patients; ③conducting widespread international exchanges and cooperation with specialty development serving as the link; ④enriching hospital culture by strengthening the building of a spiritual civilization with special characteristics; ⑤setting up the centers development department so as to raise social charity funds for the advancement of the hospital; ⑥outsourcing logistic service so as to improve operating efficiency.

ObjectiveTo compare the characteristics of urinary function in the patients with spinal cord injury or stroke. MethodsThe urinary diaries of 40 patients with spinal cord injury and 43 patients with stroke during a week were analyzed. ResultsIn the group of spinal cord injury, the average frequency of incontinence was 11 times a day, the average incontinence volume was (180.6±21.4) ml, and the average residual urine volume was (257±86.5) ml, while in the group of stroke, it was 16 times a day（P>0.05）, (298.8±34.6) ml （P<0.01）, and (28.5±18.5) ml （P<0.01）, respectively. Ultrasonic inspection discovered that 12% patients appeared ureterectasia and hydronephrosis in group of spinal cord injury, but none in group of stroke. ConclusionThere is significant difference of urinary dysfunction between patients with spinal cord injury and stroke, which need different management to protect their upper urinary tract.

Objective To investigate the diagnostic significance of synchronous observation on bone marrow biopsy,bone marrow smear and peripheral blood smear in stubborn hematonosis.Methods Thirty-seven patients with difficult and complicated hematologic diseases were detected by peripheral blood smear,bone marrow smear and bone marrow biopsy.Those slides and smears were observed.Results The 37 cases with blood disease was diagnosed of aplastic anemia in 3 cases,5 cases of myelodysplastic syndrome,6 cases of primary myelofibrosis,13 cases of secondary myelofibrosis,7 cases of lymphoma with bone marrow infiltration and 3 cases of metastatic carcinoma of bone marrow.In the diagnosis of aplastic anemia,myelodysplastic syndrome,primary and secondary myelofibrosis,lymphoma and metastatic carcinoma of bone marrow diagnosis,bone marrow biopsy is superior to smear.Conclusion Bone marrow biopsy,bone marrow smear and peripheral blood smear synchronous observation can effectively increase the diagnostic rate,decrease misdiagnosis rate,and correctly identify the stage of the disease.

Objective To assess the expression pattern of protease-activated receptor 2 (PAR2) in human keratinocytes and to characterize its biological functions in the regulation of skin barrier.Methods Primary human keratinocytes and human N/TERT keratinocytes were used as the subject of this study.The expression and distribution of PAR2 in the keratinocytes were analyzed by using immunoflorescence staining and Western blot.Two different PAR2 agonists,trypsin and a PAR2-activating peptide (AP),as well as a PAR2-antagonistic peptide (H2N-FSLLRY-COOH) and a control peptide were used to induce the activation of PAR2 in the keratinocytes.Then,a fluorescence-based calcium mobilization assay was performed to evaluate the biological function of PAR2.Data were statistically analyzed by one-factor analysis of variance.Results Under normal culture conditions,PAR2 was weakly expressed in keratinocytes,and the expression was unaffected by culture medium composition or culture duration.Calcium mobilization was induced by trypsin of 50-250 nmol/L and the PAR2-activating peptide in a dose-and time-dependent pattern.The maximal activation of PAR2 was observed in keratinocytes treated with the PAR2 agonist HAN-SLIGKV-COOH of 75-250 μmol/L.The PAR2-antagonistic peptide (H2N-FSLLRY-COOH) obviously suppressed the increase in calcium mobilization induced by trypsin,while the control peptide PAR-RAP showed no inductive effect on the PAR2 activation based on the absence of calcium mobilization.The substrate-induced calcium release was complete within 250 seconds,and peaked at 50 seconds after the initial trypsin or PAR-AP stimulation.Moreover,the activation of PAR2 was accompanied by an increase in ERK phosphorylation and elicitation of MAPK signaling pathway in keratinocytes.Conclusions Human keratinocytes positively express PAR2,which can be activated by trypsin and PAR2-activating peptides,and the activation of PAR2 may influence the physiological function of keratinocytes by inducing intracellular calcium release.

Objective To analyze the role of paroxysmal nocturnal hemoglobinuria (PNH) clones in children with acquired aplastic anemia (AA). Methods The relationship between the existence of PNH clones and clinical features in children with AA was retrospectively analyzed. The influence of PNH clones on the efficacy of combined immunosuppressive therapy (IST) of anti-thymocyte globuline (ATG) and cyclosporine A (CSA) was also observed. In addition, multiple factor analysis was used to analyze the main factors affecting the efficacy of AA. Results One hundred and forty-eight children with AA were enrolled, including 74 cases (50%) of granulocyte PNH clones positive, 68 cases (45.9%) of monocyte PNH clones positive, and 93 cases (62.8%) of total PNH clones (granulocytes and / or monocytes) positive. In 49 children having both granulocytes and monocytes PNH clones, the clone size of monocytes and granulocytes was 0.7% (0.4%-1.5%) and 0.2% (0.1%-0.7%), respectively, and the difference was significant (P<0.001) and there was a significantly positive correlation between them (r=0.65, P<0.001). According to the different PNH positive clones (monocytes, granulocytes, total), children were divide into three groups. And there were no differences in gender, age, concurrent infection, white blood cell count, hemoglobin concentration, platelet count, neutrophil absolute count, reticulocyte percentage in different PNH clones positive and negative groups (P>0.05). The group with monocytes PNH clones positive had a positive effect on the efficacy of IST (P=0.02). Multiple factor logistic regression analysis showed that the concentrations of hemoglobin and the positive PNH clones of monocytes were the main factors affecting the efficacy (P<0.05). Conclusions The high concentration of hemoglobin and the positive PNH clones of monocytes contribute the better effect of IST in children with AA.

Background: The clinical outcomes of delayed radioiodine remnant ablation (RRA) therapy in patients with low-risk papillary thyroid carcinoma (PTC) are unclear. We aimed to evaluate the clinical impact of the interval between total thyroidectomy (TT) and RRA therapy in patients with low-risk PTC. Methods: We included 526 patients who underwent TT and RRA for low-risk PTC with a primary tumor size of >1 cm between 2000 and 2012. Patients were divided into the early (<90 days) and the delayed (≥90 days) RRA groups based on the interval between TT and RRA. The results of diagnostic whole-body scan (DxWBS), ongoing risk stratification (ORS; response to therapy), and disease-free survival (DFS) were evaluated before and after propensity score matching (PSM). Results: Among the 526 patients, 75 (14.3%) patients underwent delayed RRA; they had more cervical lymph node metastasis and received a higher RRA dose than those who underwent early RRA. The median follow-up period was 9.1 years after initial therapy, and the structural recurrence rate was 1.9%. In DxWBS, 60 patients had focal iodine uptake limited in operative bed, with no significant difference between groups. According to ORS, 78%, 20%, 1%, and 1% patients were classified into excellent, indeterminate, biochemical incomplete, and structural incomplete response groups, respectively. There was no significant difference in ORS or DFS between groups before and after PSM. Conclusion The timing of the first RRA had no clinical impact in patients with low-risk PTC. Thus, the clinical decision for RRA can be determined >3 months after TT considering other prognostic factors.

Background: Immunoglobulin G4 (IgG4)-related disease is an entity that can involve the thyroid gland. The spectrum of IgG4-related thyroid disease (IgG4-RTD) includes Hashimoto thyroiditis (HT) and its fibrotic variant, Riedel thyroiditis, as well as Graves’ disease. The early diagnosis of IgG4-RTD is important because it is a medically treatable disease, and a delay in the diagnosis might result in unnecessary surgery. We present a case series of IgG4-RTD with a review of the literature. Methods: We retrospectively reviewed the clinical presentation and the radiological and pathological findings of patients diagnosed with IgG4-RTD between 2017 and 2021 at a tertiary medical center in Korea. We also conducted a literature review of IgG4-RTD. Results: Five patients were diagnosed with IgG4-RTD during the study period. The patients’ age ranged from 31 to 76 years, and three patients were men. Most patients visited the clinic for a neck mass, and hypoechogenic nodular lesions were observed on neck ultrasonography. Three patients had IgG4 HT, and two patients had IgG4 Riedel thyroiditis. All patients developed hypothyroidism that necessitated L-thyroxine replacement. The diagnosis of IgG4-RTD was confirmed after a pathological examination of the surgical specimen in the first two cases. However, the early diagnosis was possible after a core needle biopsy in three clinically suspected patients. Conclusion The diagnosis of IgG4-RTD requires clinical suspicion combined with serology and histological analyses using IgG4 immunostaining. The early diagnosis of IgG4-RTD is difficult; thus, biopsy with IgG4 immunostaining and serum IgG4 measurements will help diagnose patients suspected of having IgG4-RTD.

Background: The association between Graves’ disease (GD) and co-existing thyroid cancer is still controversial and most of the previously reported data have been based on surgically treated GD patients. This study investigated the clinicopathological findings and prognosis of concomitant thyroid cancer in GD patients in the era of widespread application of ultrasonography. Methods: Data of GD patients who underwent thyroidectomy for thyroid cancer between 2010 and 2019 in three tertiary hospitals in South Korea (Asan Medical Center, Chonnam National University Hwasun Hospital, and Pusan National University Hospital) were collected and analyzed retrospectively. In the subgroup analysis, aggressiveness and clinical outcomes of thyroid cancer were compared nodular GD and non-nodular GD groups according to the presence or absence of the thyroid nodules other than thyroid cancer (index nodules). Results: Of the 15,159 GD patients treated at the hospitals during the study period, 262 (1.7%) underwent thyroidectomy for coexisting thyroid cancer. Eleven patients (4.2%) were diagnosed with occult thyroid cancer and 182 patients (69.5%) had microcarcinomas. No differences in thyroid cancer aggressiveness, ultrasonographic findings, or prognosis were observed between the nodular GD and non-nodular GD groups except the cancer subtype. In the multivariate analysis, only lymph node (LN) metastasis was an independent prognostic factor for recurrent/persistent disease of thyroid cancer arising in GD (P=0.020). Conclusion The prevalence of concomitant thyroid cancer in GD patients was considerably lower than in previous reports. The clinical outcomes of thyroid cancer in GD patients were also excellent but, more cautious follow-up is necessary for patients with LN metastasis in the same way as for thyroid cancer in non-GD patients.

Background: The optimal dose of radioactive iodine (RAI) therapy for N1b papillary thyroid carcinoma (PTC) is controversial. We evaluated the clinical outcome of N1b PTC patients treated with either 100 or 150 mCi of RAI. Methods: We retrospectively analyzed N1b PTC patients who underwent total thyroidectomy and postoperative RAI therapy at a tertiary referral center between 2012 and 2017. As the baseline characteristics differed between treatment groups, we performed exact matching for various pathological factors according to RAI dose. We evaluated the response to therapy and recurrence-free survival (RFS) in the matched patients. Structural recurrent/persistent disease was defined as new structural disease detected after initial therapy, which was confirmed by cytology or pathology. Results: Of the total 436 patients, 37 (8.5%) received 100 mCi of RAI and 399 (91.5%) received 150 mCi of RAI. After an exact 1:3 matching, 34 patients in the 100 mCi group and 100 patients in the 150 mCi group remained. There was no significant difference in response to therapy between the groups in the matched population (P=0.63). An excellent response was achieved in 70.6% (n=24) of patients in the 100 mCi group and 76.0% (n=76) in the 150 mCi group. Two (5.9%) patients in the 100 mCi group and four (4.0%) in the 150 mCi group had recurrence and there was no significant difference in RFS between the groups in the matched population (P=0.351). Conclusion There were no differences in response to therapy and RFS in N1b PTC patients according to RAI dose.

Background: Hürthle cell carcinoma (HCC), a type of thyroid carcinoma, is rare in South Korea, and few studies have investigated its prognosis. Methods: This long-term multicenter retrospective cohort study evaluated the clinicopathological features and clinical outcomes in patients with HCC who underwent thyroid surgery between 1996 and 2009. Results: The mean age of the 97 patients included in the study was 50.3 years, and 26.8% were male. The mean size of the primary tumor was 3.2±1.8 cm, and three (3.1%) patients had distant metastasis at initial diagnosis. Ultrasonographic findings were available for 73 patients; the number of nodules with low-, intermediate-, and high suspicion was 28 (38.4%), 27 (37.0%), and 18 (24.7%), respectively, based on the Korean-Thyroid Imaging Reporting and Data System. Preoperatively, follicular neoplasm (FN) or suspicion for FN accounted for 65.2% of the cases according to the Bethesda category, and 13% had malignancy or suspicious for malignancy. During a median follow-up of 8.5 years, eight (8.2%) patients had persistent/recurrent disease, and none died of HCC. Older age, gross extrathyroidal extension (ETE), and widely invasive types of tumors were significantly associated with distant metastasis (all P<0.01). Gross ETE (hazard ratio [HR], 27.7; 95% confidence interval [CI], 2.2 to 346.4; P=0.01) and widely invasive classification (HR, 6.5; 95% CI, 1.1 to 39.4; P=0.04) were independent risk factors for poor disease-free survival (DFS). Conclusion The long-term prognosis of HCC is relatively favorable in South Korea from this study, although this is not a nation-wide data, and gross ETE and widely invasive cancer are significant prognostic factors for DFS. The diagnosis of HCC by ultrasonography and cytopathology remains challenging.