Inducible co-stimulatory molecules participate in mesenteric vascular endothelial-mesenchymal transition and sclerosis of mesenteric vessels in spontaneously hypertensive rats / 南方医科大学学报

OBJECTIVE@#To investigate the correlation of inducible co-stimulatory molecules (ICOS) with mesenteric vascular endothelial- mesenchymal transition (EndMT) and sclerosis in spontaneously hypertensive rats (SHR).@*METHODS@#Twenty 4-week-old WKY rats and 20 SHRs of the same strain were both randomly divided into 4 groups for observation at 4, 6, 10 and 30 weeks of age. ICOS expression frequency in rat spleen CD4+T cells was analyzed using flow cytometry, and the expressions of ICOS, VE-cad, α-SMA and Col3 mRNA in rat mesentery were detected by RT-PCR. The distributions of ICOS, IL-17A and TGF-β in rat mesentery were detected by immunohistochemistry. The levels of IL-17A and TGF-β in rat plasma were measured using ELISA. The morphological changes of rat mesenteric vessels were observed with Masson staining. Spearman or Pearson correlation analyses were used to evaluate the correlation between ICOS expression and the expressions of the markers of vascular EndMT and sclerosis.@*RESULTS@#Compared with the control WKY rats, the SHRs began to show significantly increased systolic blood pressure and ICOS expression frequency on CD4+T cells at 6 weeks of age (P < 0.05). In the SHRs, the mRNA and protein expressions of ICOS, α-SMA, Col3, IL-17A and TGF-β in the mesentery were significantly higher than those in control group (P < 0.05), while the mRNA and protein expressions of VE-cad started to reduce significantly at 10 weeks of age (P < 0.05). The plasma levels of IL-17A and TGF-β were significantly increased in SHRs since 6 weeks of age (P < 0.05) with progressive worsening of mesenteric vascular sclerosis (P < 0.05). ICOS mRNA and protein expression levels in the mesenteric tissues of SHRs began to show positive correlations with α-SMA and Col3 expression levels and the severity of vascular sclerosis at 6 weeks of age (P < 0.05) and a negative correlation with VE-cad expression level at 10 weeks (P < 0.05).@*CONCLUSION@#ICOS play an important pathogenic role in EndMT and sclerosis of mesenteric vessels in essential hypertension by mediating related immune responses.

A case of encapsulated peritoneal sclerosis after peritoneal dialysis-related peritonitis / 中南大学学报(医学版)

Encapsulating peritoneal sclerosis (EPS) is a rare but severe complication of peritoneal dialysis. A total of 50% of the patients died within 12 months after being diagnosed. There are no obvious clinical symptoms in the early stage of EPS, which is easy to be missed. And there are few case reports of EPS in early stage. On December 22, 2018, a 70-year-old male patient undergoing peritoneal dialysis for 17 months, who was diagnosed as EPS, was admitted to the Department of Nephrology, the Third Xiangya Hospital, Central South University. The patient's peritoneal dialysis catheter was obstructed after peritonitis. The peritoneal dialysis fluid couldn't be drain in and out of the abdominal cavity. Therefore, the laparoscopy was performed to repair the catheter. The operation in progress showed that the peritoneum was slightly thickened and the ileocecal intestinal tube was closely adhered to the parietal peritoneum where the catheter was wrapped, indicating the early stage of EPS. Peritoneal relaxation was performed. The patient's catheter was normal after adhesiolysis. He underwent hemodialysis, nutritional supporting as well as peritoneal dialysis transition, etc. The peritonitis was controlled after 10 days and the peritoneal dialysis was resumed. After discharge from hospital, the patient took moxifloxacin for 2 more weeks. We followed up the patient for 6 months. The automated peritoneal dialysis is maintained, and everything remains normal. Clinicians need to improve understanding of EPS. Early diagnosis and laparoscopic adhesiolysis is helpful to continue peritoneal dialysis treatment.

Intravascular and intraparenchymatous hepatic segmentary sclerosis

Abstract Purpose: To evaluate the morphological effects of injected sclerosing agents into the liver. Methods: This study was performed on twenty dogs, distributed into five groups: Group 1 (n = 5) - control, Group 2 (n = 5) - injection of 50% glucose solution inside hepatic parenchyma and animals followed during seven days, Group 3 (n = 10) - injection of ethanol inside hepatic parenchyma and animals distribution into two subgroups Subgroup 3A (n = 5) - followed during 24 hours and subgroup 3B (n = 5) - followed during seven days (group 3B), Group 4 (n = 5) - ethanol injection inside left portal vein branch and followed during 24 hours. Livers were macroscopically evaluated, submitted to hepatic arteriography and portography, then histology. Results: All animals in Group 4 died within 23 hours due to diffuse hepatic necrosis. The animals of groups 2 and 3 had a satisfactory evolution. Fibrosis formed in the segment reached by the sclerosant solution and interruption of the contrast flow injected into the portal system. Conclusion: Intrahepatic parenchymal ethanol injection is well tolerated and causes sclerosis restricted to a specific segment; however, intraportal ethanol injection causes massive hepatic necrosis and can lead to death.

Usefulness of Diffusion Tensor Tractography in Pediatric Epilepsy Surgery

PURPOSE: This study was conducted to assess the clinical relevance of diffusion tensor tractography (DTT) in pre- and post-operative evaluations of childhood epilepsy surgery. MATERIALS AND METHODS: Seventy-two patients who received epilepsy surgery between March 2004 and July 2008 were retrospectively analyzed (M : F=40 : 32, ages of 3 months to 24 years, mean age=8.9 years). DTT was performed using a 3.0 T scanner and single-shot spin-echo echo-planar imaging with 32-different diffusion gradient directions. We reviewed the data focusing on the type of surgery, final pathological diagnosis, and how the DTT data were clinically used. RESULTS: The most common form of childhood epilepsy surgery was complete resection of an epileptogenic lesion (n=52, 72.2%). The reported etiologies included cortical dysplasia (n=32, 44.4%), hippocampal sclerosis (n=9, 12.5%), brain tumors (n=7, 9.7%), and non-pathologic lesions (n=4, 5.6%) in the final diagnoses. Twenty-one dysplastic cortexes and four brain tumors involved an approximal relationship with the corticospinal tract (n=18), optic radiation (n=2), and arcuate fasciculus (n=5). Additionally, although DTT demonstrated white matter tracts clearly, DTT in the hippocampal sclerosis did not provide any additional information. In cases of callosotomy (n=18, 25%), post-operative DTT was utilized for the evaluation of complete resection in all patients. DTT information was not used in functional hemispherectomy (n=2, 2.8%). CONCLUSION: Preoperatively, DTT was a useful technique in cases of cortical dysplasia and brain tumors, and in cases with callosotomy, postoperatively. DTT should be included among the routine procedures performed in management of epilepsy.

Abdominal cocoon: Case report of a rare cause of intestinal obstruction.

Abdominal cocoon or sclerosing encapsulated peritonitis is a rare cause of intestinal obstruction often seen in adolescent girls. We present a case of an abdominal cocoon in a 14-year-old female presenting as a surgical emergency. The patient underwent excision of the involved segment of small bowel along with a part of ascending colon. Gross morphology revealed intestinal coils wrapped within a thick fibrous membrane. Diagnosis of this condition is seldom possible on radiological imaging. Characteristic gross appearance is showcased in our case, to create more awareness of this finding.

Hereditary Sclerosing Poikiloderma

Hereditary sclerosing poikiloderma (HSP) is a very rare disease. The clinical features are principally widespread poikiloderma and linear hyperkeratotic and sclerotic bands. We report an 18-yr-old male who presented reticular hyperpigmented lesions on the trunk and extremities since 2-yr-old. Also, linear sclerosing bands appeared on both antecubital and popliteal fossae after yr. Histopathologic finding showed dense sclerotic collagen fibers with telangiectasia in the upper dermis and fragmentations of damaged elastic fibers in the elastic stain, consistent with HSP. We report the first Korean case of HSP.

Características clínicas e laboratoriais associadas à nefroangioesclerose hipertensiva confi rmada por biópsia renal / Clinical and laboratorial characteristics associated with biopsy proved hypertensive nephroangiosclerosis

INTRODUÇÃO: A nefroangioesclerose hipertensiva é importante causa de doença renal crônica com necessidade de diálise. As características que distinguem um portador de hipertensão arterial que evolui com nefroangioesclerose de outro que mantém função renal estável não são bem estabelecidas, devido à dificuldade em assegurar que os portadores daquela doença não sejam, na verdade, portadores de glomerulopatias ou outras doenças renais confundíveis. Dessa maneira, o objetivo deste trabalho foi identificar características clínicas ou laboratoriais que distingam os pacientes que desenvolveram doença renal crônica a partir da hipertensão, confirmada por biópsia renal, daqueles que, mesmo apresentando hipertensão arterial, não desenvolveram nefroangioesclerose. MÉTODOS: Realizou-se comparação retrospectiva de dados clínicos e laboratoriais de 15 portadores de nefroangioesclerose hipertensiva confirmada por biópsia renal e 15 hipertensos oriundos do ambulatório do Centro de Hipertensão Arterial, cuja ausência de nefroangioesclerose foi definida pela ausência de proteinúria. Os grupos foram pareados quanto à idade e gênero. RESULTADOS: Dentre as variáveis avaliadas, tempo de hipertensão arterial, pressão de pulso, glicemia, ácido úrico, creatinina e frequência de uso de diuréticos e simpatolíticos diferiram estatisticamente entre os dois grupos. Todas essas variáveis apresentaram valores maiores no grupo com nefroangioesclerose hipertensiva. CONCLUSÃO: O presente estudo associa a nefroangioesclerose hipertensiva, confirmada por biópsia, com alterações metabólicas, duração e intensidade da hipertensão e corrobora a ideia de que a prevenção primária da hipertensão arterial, postergando o seu início, o controle pressórico mais estrito, quando a hipertensão já está estabelecida, bem como o controle metabólico têm a potencialidade de prevenir o desenvolvimento de nefroangioesclerose hipertensiva.

INTRODUCTION: Hypertensive nephroangiosclerosis is a major cause of chronic kidney disease requiring dialysis. Clinical characteristics that distinguish a patient with hypertension that evolves to nephroangiosclerosis from another that keeps stable renal function are not well established because of the difficulty in ensuring that the carriers of that disease are not actually suffering from glomerulonephritis or other kidney diseases. Thus, our objective was to identify clinical or laboratory features that distinguish the patients who developed chronic renal failure from hypertension, confirmed by renal biopsy, of those who, even with arterial hypertension, did not develop nephroangiosclerosis. METHODS: We conducted a retrospective comparison of clinical and laboratory data of 15 patients with hypertensive nephroangiosclerosis confirmed by renal biopsy and 15 hypertensive patients from the outpatient clinic of the Hypertension Center, whose lack of nephroangiosclerosis was defined as absence of proteinuria. The groups were matched for age and gender. RESULTS: Among the evaluated variables, duration of hypertension, pulse pressure, blood glucose, uric acid, creatinine and frequency of use of diuretics and sympatholytic differed statistically between the two groups. All these variables were higher in nephroangiosclerosis patients. CONCLUSION: This study links biopsy proven hypertensive nephroangiosclerosis with metabolic features, hypertension intensity and duration, corroborating the idea that primary prevention of hypertension, postponing its initiation, a more intensive hemodynamic control (when hypertension is well established) and metabolic control of these patients have the potential to prevent hypertensive nephroangiosclerosis.

Hipertensión portal en niños: historia natural, evolución, tratamiento y pronóstico / Portal hypertension in children: natural history, evolution, therapy and prognosis

La Hipertensión Portal (HTP) en niños es de difícil manejo debido a múltiples etiologías y al empleo de tratamientos no estandarizados. Reportar la historia natural de la HPT en niños atendidos en la Unidad, evaluar la evolución y el impacto del tratamiento en el pronóstico. 27 pacientes evaluados desde 1999 hasta 2009. Registro de: etiología, clínica, laboratorio, Eco- Doppler clasificación endoscópica de las várices esofágicas, gástricas y fúndicas, Child-Pugh y terapéutica endoscópica. 18/27(66,66%) Cavernomatosis de la Porta, 3/27(11,11%) Cirrosis Hepática Secundaria a Hepatitis Auto-inmune, 3/27(11,11%) Fibrosis Hepática Congénita, 2/27(7,40%) Colestasis Intrahepática Familiar y 1/27(3,70%) Síndrome de Budd Chiari; promedio de edad 4,6 años +/- 4,47. Debutaron con hemorragia digestiva superior 17/27(62,96%). Varices Esofágicas grado III 13/27(48,14%) y grado IV 4/27(14,81%); Gastropatía hipertensiva severa 5/27(18,51%), Várices gastroesofágica tipo II 9/27(33,33%). Profilaxis Primaria con propanolol 9/27(33,33%) y 1/27(3,70%) ligadura endoscópica más propanolol. Profilaxis secundaria: 17/27(62,96%), con ligadura endoscópica 47,05%, Escleroterapia 35,29% y ligadura más esclerosis 17,64%. Uso de somatostatina 2/17(11,76%) y falla en el control de hemorragia post-tratamiento 1/17(5,88%). Child Pugh B 4/27(14,81%) y Child Pugh C 4/27(14,81%). Posterior al Tratamiento: erradicación de várices esofágicas con ligadura endoscópica en 50% contra 35,71% con la escleroterapia (p< 0.05), un promedio de 2 +/- 1,41 sesiones vs 3,4 +/- 1,78 respectivamente. Resangrado en 2/17(11,76%). En niños con cirrosis con Child Pugh B y C con terapéutica endoscópica compensaron la afectación hepática. Un seguimiento 5,3 años: mortalidad de 1/27(3,70%), un trasplante hepático, 2 derivación porto-cava y 3 en espera. La profilaxis primaria o secundaria indicada disminuyo el riesgo de hemorragia variceal con pocas complicaciones, y mejoró el pronóstico de los pacientes...

Portal Hypertension (PHT) in children is hard to handle with as a result of multiple etiologies and because of using non standardized treatments. Reporting the PHT’s natural history in children assisted in the Unit, assessing the evolution and impact of the treatment in the prognosis. 27 patients assessed between 1999 and 2009. The following was recorded: etiology, clinic, laboratory, Eco-Doppler, endoscopic classification of esophageal, gastric and fundic varices, Child-Pugh, and endoscopic therapy. 18/27 (66.66%), Portal Cavernomatosis; 3/27 (11.11%), Secondary Liver Cirrhosis to Autoimmune Hepatitis; 3/27 (11.11%), Congenital Hepatic Fibrosis; 2/27 (7.40%), Familial Intrahepatic Cholestasis; and 1/27 (3.70%), Budd-Chiari Syndrome; average age: 4.6 ± 4.47. First time with upper digestive hemorrhage: 17/27 (62.96%). Grade III Esophageal Varices, 13/27 (48.14%); and Grade IV: 4/27 (14.81%); Severe Hypertensive Gastropathy: 5/27 (18.51%); Type-II Gastro-esophageal Varices: 9/27 (33.33%). Primary prophylaxis by Propanolol: 9/27 (33.33%); and endoscopic ligature plus Propanolol: 1/27 (3.70%). Secondary prophylaxis: 17/27 (62.96%); with endoscopic ligature: 47.05%; Sclerotherapy: 35.29%; and ligature plus sclerosis: 17.64%. Usage of somatostatin: 2/17 (11.76%); and failure in controlling post-treatment hemorrhage: 1/17 (5.88%). Child-Pugh B: 4/27 (14.81%); and Child-Pugh C: 4/27 (14.81%). Post-Treatment: eradication of esophageal varices by endoscopic ligatures in 50% vs. 35.71% with sclerotherapy (p< 0.05), a 2 ± 1.41 average of sessions vs. 3.4 ± 1.78, respectively. Rebleeding in 2/17 (11.76%). Children with cirrhosis, with Child-Pugh B and C, compensated the hepatic disturbance with endoscopic therapy. A 5.3-year follow-up: 1/27 (3.70%) morbidity; one liver transplantation; 2 portal-cava derivation, and 3 in the wait list. The prescribed primary or secondary prophylaxis diminished the risk of varicose hemorrhage with few complications...

Usefulness of ISN/RPS Classification of Lupus Nephritis

About 50-80% of patients with lupus suffer from lupus nephritis which is one of major causes of morbidity and mortality. Renal pathologists and nephrologists should evaluate the degree of histological damages to establish therapeutic plans for lupus nephritis. In order to standardize definitions, to emphasize clinically relevant lesions, and to improve interobserver reproducibility, the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification was proposed. Recently, several retrospective validation studies concerning the utility of the ISN/RPS classification, especially among class IV, were performed. In these reports, reproducibility is improved by the definition of diagnostic term, but the outcome related with classification, especially in class IV, is controversial. We performed retrospective analysis of 99 biopsy- proven subjects with lupus nephritis in our facility using the ISN/RPS classification. The class IV-G group tended to exhibit a worse renal outcome, but the difference compared with IV-S was not significant. In a Cox proportional hazards models, Independent histological predictors of poor renal outcome were extracapillary proliferation, glomerular sclerosis and fibrous crescents, while hyaline thrombi and fibrous adhesions were of favorable renal outcome. Both were similarly observed in IV-G and IV-S. The more qualitative categorization by the response to standard treatment may be needed to emphasize clinically relevant lesion related to renal outcome.

A Case of Idiopathic Mesenteric Phlebosclerosis / 대한소화기학회지

Idiopathic mesenteric phlebosclerosis, rare disease entity causing chronic mesenteric ischemia is a member of non-thrombotic, non-inflammatory stenosis or occlusion of the mesenteric veins. The histologic hallmark is marked fibrous mural thickening and sclerosis of the vessel wall. It is frequently accompanied by calcification in the vessel wall. We report the case of a 61-year-old woman with idiopathic mesenteric phlebosclerosis. To our knowledge, this is the first case reported in Korea.

A Case of Idiopathic Mesenteric Phlebosclerosis / 대한소화기학회지

Idiopathic mesenteric phlebosclerosis, rare disease entity causing chronic mesenteric ischemia is a member of non-thrombotic, non-inflammatory stenosis or occlusion of the mesenteric veins. The histologic hallmark is marked fibrous mural thickening and sclerosis of the vessel wall. It is frequently accompanied by calcification in the vessel wall. We report the case of a 61-year-old woman with idiopathic mesenteric phlebosclerosis. To our knowledge, this is the first case reported in Korea.

Hippocampal sclerosis and status epilepticus: cause or consequence? A MRI study

BACKGROUND: Transient imaging abnormalities, including changes on diffusion-weighted imaging (DWI), may be seen in status epilepticus. These abnormalities can be followed by hippocampal sclerosis. CASE REPORT: We report a 15-year-old lady with focal non convulsive status epilepticus (NCSE) and focal slowing on EEG. DWI exhibited abnormal hyperintense signals in bilateral temporal and insular cortices. After 3 weeks, MRI performed a localizated hippocampal atrophy. CONCLUSION: The MRI findings indicated vasogenic and cytotoxic edema during seizure activity and subsequent loss of brain parenchyma.

INTRODUÇÃO: Anormalidades transitórias de imagem, incluindo imagens de ressonância magnética por difusão (DWI), podem ser vistas no status epilepticus. Essas anormalidades podem ser seguidas de esclerose hipocampal. RELATO DE CASO: Nós relatamos uma jovem de 15 anos com status focal não convulsivo e lentificação focal no EEG. DWI mostrava sinal hiperintenso em regiões temporais bilaterais e córtex insular. Após 3 semanas, RM de encéfalo mostrava atrofia localizada do hipocampo. CONCLUSÃO: Os achados de RM indicam edema vasogênico e citotóxico durante as crises epilépticas com subseqüente atrofia de parênquima cerebral.

Recent progress in epilepsy in neuropathology / 法医学杂志

Epilepsy is a common cerebral disease, and may cause sudden death. Although electric activity study of epileptic brain had been emphasized in the past, the neuropathological study of epilepsy has become a main focus in clinical and forensic medicine recently. This article reviews the recent progress in neuropathology of epilepsy including developmental disorder, abnormal tumoral proliferation, hippocampal sclerosis, dual pathological alteration, and mossy fiber sprouting. Its significance in forensic medicine, particularly for the diagnosis of sudden unexpected death in epilepsy, is discussed.

Sclerosing Encapsulating Peritonitis (Abdominal Cocoon) after Abdominal Hysterectomy

Sclerosing encapsulating peritonitis (SEP) is a poorly understood and rarely documented cause of small bowel obstruction. Although recurrent peritonitis has been reported as the main contributory factor leading to secondary SEP, the pathogenesis of primary (idiopathic) SEP is still uncertain. A 40-year-old woman with a history of total abdominal hysterectomy due to gestational trophoblastic disease presented with progressive lower abdominal pain and abdominal distension. Ultrasonography and contrast-enhanced abdomen-pelvis computed tomography of the abdomen revealed encapsulation of the entire small bowel with a sclerotic capsule. At laparotomy, a fibrous thick capsule encasing small bowel loops was revealed. Extensive adhesiolysis and removal of the capsule from the bowel loops were performed. The patient recovered uneventfully; she was discharged without complications. SEP is a rare cause of small bowel obstruction. We treated a case of abdominal cocoon with intestinal partial obstruction in a woman with a history of abdominal hysterectomy due to gestational trophoblastic disease. Surgical treatment was effective and the patient recovered without complication.

Características morfológicas do lobo da ínsula em pacientes portadores de epilepsia do lobo temporal medial / Morphological characteristics from the insula's lobe in patients with medial temporal lobe epilepsy / Morphological characteristics from the insula's lobe in patients with medial temporal lobe epilepsy

A esclerose medial temporal (EMT) é caracterizada pela esclerose hipocampal e diferentes graus de acometimento das estruturas vizinhas como amígdala, giro parahipocampal e córtex entorrinal. O estudo avaliou 40 indivíduos com EMT e 40 do grupo controle. Os casos foram avaliados por um método para as medidas da ínsula (E-Film) e outro método para o cálculo do volume (Neuroline). Não houve diferença estatística de alteração de volume e das medidas do lobo da ínsula nos pacientes portadores de EMT. O estudo não demonstrou alteração morfológica da ínsula quando comparado os dois grupos.

The temporal medial sclerosis (TMS) is characterized by hippocampal sclerosis in temporal and by distinghished grades of injury near to other neurological structures such as: amygdaloid nucleus, parahippocampal girus and entorhinal region. The study analyzed 40 patients with TMS and 40 people from the control cluster. All the cases were appreciated by one method for measurement of insula's cortex (E-Film) and another method to calculate the insula's volume (Neuroline). There is no variation statistical between the insula's volume and insula's measurement for the two clusters. This paper didn't show the insula's morphological variation when these two groups were compared.

Esclerosis sistémica / Systemic sclerosis

La esclerosis sistémica (ES) es una enfermedad del tejido conectivo de etiología desconocida que se caracteriza por cambios fibróticos de la piel, tejido subcutáneo y visceral, anormalidades de la microvasculatura y desórdenes inmunes.

Disseminated spina ventosa.

Spina Ventosa is a rare condition. A rare case of disseminated tuberculosis of bones and skin without primary foci is presented. Gross sclerosis of the short bones of hand and leg were noted.

Uso da sequência FLAIR-EPI na análise da esclerose mesial temporal / EPI-FLAIR sequence in the evaluation of mesial temporal sclerosis

O objetivo deste estudo é analisar as alteraçöes morfológicas e de intensidade de sinal das regiöes hipocampais em pacientes, com epilepsia temporal fármaco-resistente. Para tal, estudamos 8 pacientes com esclerose mesial temporal, utilizando aparelhagem de RM de 1,5T, com sequências Spin Eco - SE, Fast Spin Eco - FSE, Fluid Atenuation Inversion Recovery, com Eco Planar Imaging - FLAIR-EPI. Observamos a superioridade da sequência FLAIR na detecçäo do aumento da intensidade de sinal da regiäo hipocampal, particularmente com cortes coronais, em relaçäo às sequências SE e FSE, com a vantagem de ser uma técnica de rápida execuçäo. A sequência STIR evidenciou adelgaçamento da cortical do hipocampo, na metade dos casos que apresentavam alteraçäo de sinal