The early diagnosis and differential diagnosis of IgG4-related diseases / 解放军医学杂志

[Abstract] The immunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic autoimmune disease characterized by diffuse inflammatory cell infiltration and/or organ enlargement in one or more organ foci and elevated serum IgG4 levels. Typical histopathological changes include lymphocytic cell infiltration, schlieren interstitial fibrosis, and/or occlusive phlebitis. The incidence of IgG4-RD is low, very heterogeneous, and similar to many other diseases in clinical, pathological and laboratory examination, so delayed diagnosis and misdiagnosis are very common. The early diagnosis and differential diagnosis of IgG4-RD have been reviewed in present paper, especially IgG4-related hepatobiliary diseases, IgG4-related skin lesions, Mikulic disease and autoimmune pancreatitis, in order to reduce misdiagnosis.

Research progress on biomarkers of Fabry's disease / 医学研究生学报

Fabry disease (FD) is a rare X⁃linked lysosomal storage disorder. It is characterized by deficient activity of α⁃galactosidase A, which causes the storage of globotriaosylceramide in tissues and organs, leading to fatal complications and poor prognosis. Therefore, it is essential to use the possibilities of specific biomarkers for early diagnosis, identification of organ involvement and therapy monitoring. Lyso⁃Gb3 is a valuable biomarker to establish the diagnosis and also important for evaluating the pathogenic mutations. Proteinuria and creatinine are the most valuable biomarkers to detect renal damage. Troponin I and high⁃sensitivity assays for cardiac troponin T can identify the patients with myocardial injury. This article mainly reviews the markers of FD⁃related target organs such as kidney, heart and nervous system damage and its guiding value for disease progression, curative effect and prognosis evaluation.

The progression of intervention cell death in kidney diseases / 医学研究生学报

Precise regulation of cell death and survival is essential for proper maintenance of organismal homeostasis, development, and immune system. Kidney diseases, especially acute histology changings linked ischemia-reperfusion injury, are among illnesses that are profoundly affected by improper regulation or execution of cell death pathways. Given the truth that improper regulation of cell death participates in a common pathway of various pathologies, specific therapeutic strategies that aim at the regulation are promising. In current review, we reviewed the most common pathway on cell death in renal injury, also described several potential therapy strategies that may influence the prognosis of kidney diseases，such as targeting receptor interacting protein 1 kinase.

Heterogeneity of the clinical manifestations and pathology features in c3 glomerulopathy / 解放军医学杂志

C3 glomerulopathy is a kind of glomerular diseases mediated by abnormal activation of alternative complement pathway. As diversity and multiplicity of pathogenic mechanism, heterogeneity exists in the clinical manifestation and pathological features of C3 glomerulopathy. The clinical manifestation of the disease may be shown as abnormality in urine, hypertension, hematuria, nephrotic syndrome, nephritic syndrome, renal insufficiency, etc. Membranoproliferative glomerulonephritis, mesangial proliferation, crescent formation, focal segmental necrosis, diffuse hyperplasia and exudative lesions, etc may be found in renal biopsies. Also, the prognosis of C3 glomerulopathy is not uniform. The clinical manifestations and pathological features of C3 glomerulopathy were reviewed in the present paper.

The pathogenic mechanism of C3 glomerulopathy and its countermeasures / 解放军医学杂志

C3 glomerulopathy is a series of diseases of glomeruli mediated by abnormal activation of alternative complement pathway. A series of researches have revealed in recent years that there are diversity and multiplicity of pathogenic mechanism in the pathogenesis of C3 glomerulopathy. The pathogenic mechanism of C3 glomerulopathy may be different in different individuals and types of disease. Congenital genetic defects and/or acquired autoantibodies may be found in the same individual. Individualized therapy should be given to individual patient in order to target different pathogenic mechanisms. Chinese herbal medicine, Tripterygium wilfordii, shows promise as a potential therapeutic agent for C3 glomerulopathy. The pathogenic mechanism and countermeasures for C3 glomerulopathy have been reviewed in present paper.

Pseudoangiosarcomatous squamous cell carcinoma of the penis: a case report with clinicopathological and human papilloma virus analyses / 中华男科学杂志

<p><b>OBJECTIVE</b>To further understand the clinicopathological, ultrastructural and molecular features of penile pseudoangiosarcomatous squamous cell carcinoma (PASCC), and improve its diagnosis and treatment.</p><p><b>METHODS</b>A 47-year-old male patient with penile PASCC was reported and the relevant literature reviewed. The main clinical manifestations of the patient were a typical surface ulceration with hemorrhage and purulent secretion with a foul smell, a papillary mass about 5.0 cm x 5.0 cm x 4.0 cm for 1 year on the foreskin of the penis, and 3 enlarged bilateral inguinal lymph nodes. CT scanning showed no enlarged lymph nodes in the abdomen and pelvis, and X-ray examination revealed no abnormality in the chest.</p><p><b>RESULTS</b>The diagnosis was established by biopsy. Partial penectomy and bilateral inguinal lymphadenectomy (T2N2M0) were performed, followed by adjuvant pelvic radiotherapy. Two months later, total penectomy was necessitated by penile flap necrosis and local recurrence. Eleven months after the first surgery, the patient died of extensive metastasis to the pelvis and lungs. Under the light microsope, the tumor was mainly composed of vessel-like lacunar reticularis spindle cells and a few local squamous cancer cells. Careful examination revealed some focal areas with evident transition from squamous nests to the more acantholytic areas extending towards the pseudoangiosarcomatous spaces. Pathogenetically, it appeared to be the variant of acantholytic squamous cell carcinoma. Immunohistochemically, most tumor cells were strongly positive for keratin (AE1/AE3) and focally positive for EMA, with the typical squamous cells focally positive for 34betaE12 and vimentin. The vessels that proliferated in the tumor were decorated by CD31, CD34 and factor VIII-related antigens, but the tumor cells were negative for HMB45, SMA, Desmin and CEA. HPV DNA (HPVpan, HPV6B/11, HPV16/18, HPV31/33) was not detected by in situ hybridization in the primary and metastatic tumors.</p><p><b>CONCLUSION</b>PASCC is a specific and extremely rare subtype of penile SCC with dramatic similarity to angiosarcoma under the microscope, with poor prognosis. Its diagnosis depends on histopathological, immunohistochemical and ultrastructural studies. Such a presentation underscores the importance of timely consultation, early diagnosis and prompt treatment.</p>

Juxtaglomerular cell tumor of the kidney: a clinicopathologic analysis of five cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the morphologic characteristics and immunophenotype of juxtaglomerular cell tumor of the kidney (JGCT), with discussion on its diagnostic clues and possible histogenesis.</p><p><b>METHODS</b>The clinical, pathologic and immunohistochemical features of 5 cases of JGCT were evaluated. In addition, 5 cases of hemangiopericytoma and 5 cases of cutaneous glomus tumor were selected for comparative immunohistochemical analysis.</p><p><b>RESULTS</b>The JGCT cases came from 4 females and 1 male (mean age at diagnosis = 32 years). All of them manifested symptoms of systemic hypertension. Four of the patients received partial nephrectomy and the remaining patient was treated by radial nephrectomy. All of them were followed up for a period of 4 to 66 months (average = 27 months). There was no evidence of local recurrence or distant metastases. On gross examination, these JGCTs were well-circumscribed and situated in the renal cortex and measured 4.4 cm in greatest dimension on average. Histologically, the tumor was characterized by the following three features: (1) solid sheets of relatively uniform polygonal to round cells with lightly eosinophilic cytoplasm, sometimes containing PAS-positive intracytoplasmic granules; (2) absence of or very scanty mitotic figures; (3) interstitium rich in thin-walled capillaries, associated with focal hyaline change and hemangiopericytoma-like architectural pattern. Under electron microscopy, characteristic rhomboid-shaped renin granules were found in the cytoplasm. All JGCTs were immunoreactive for renin, CD34, actin, and calponin. In contrast, all glomus tumors were negative for renin and all hemangiopericytomas were negative for actin.</p><p><b>CONCLUSIONS</b>JGCT is a rare benign renal neoplasm typically found in young adults and manifests as systemic hypertension. The tumor cells may be originated from modified vascular smooth muscle cells. The identification of renin granules by electron microscopy and demonstration of the characteristic immunophenotype is the key to correct pathologic diagnosis.</p>

Clinical application of propofol in critical patients with mechanicalventilation after major operation / 中国临床药理学与治疗学

Aim To evaluate the clinical application of propofol in critical patients with mechanical ventilation.Methods 22 patients with mechanical ventilation after major operation were randomly divided into control group and propofol group patient in the propofol group were continually transfused with propofol by micro pump.Clinical manifestations, blood pressure, heart rate and the concentration of epinephrine (E) and norepinephrine (NE) in patients of both groups were observed.Results The patients in propofol group were all in sleeping state, while those in the control group had evidently higher blood pressure, heart rate and the concentration of epinephrine and norepinephrine after wearing of ventilator.There was a significant difference between the two groups (P