Glomerular parietal epithelial expression of CD44 in minimal change nephrotic syndrome and primary focal segmental glomerulosclerosis: A clinico-pathological study

Introduction: Minimal change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS) are the two common causes of nephrotic syndrome (NS) in both children and adults with overlapping clinical features, but with distinct prognostic and therapeutic implications. The distinction between these relies entirely on histopathology, which can sometimes be difficult. CD44 is expressed by activated parietal epithelial cells, plays a role in matrix deposition and thus in the pathogenesis of FSGS. Aims: To assess the expression of CD44 in MCNS and FSGS and to evaluate its association with the known clinical and histopathological prognostic factors. Materials and Methods: Thirty cases each of MCNS and FSGS were studied. The clinical, laboratory, histopathological, and CD 44 immunohistochemical data were recorded. The findings were analyzed and correlated. A P value of < 0.05 was considered statistically significant. Results: Statistical association was noted between CD44 positivity and serum creatinine (p = 0.031), estimated glomerular filtration rate (p = 0.040), segmental sclerosis (p < 0.001), tubular atrophy (p = 0.027), interstitial fibrosis (p = 0.027), and histological diagnosis (p < 0.001). The sensitivity, specificity, positive predictive, and negative predictive values were 90%, 76.67%, 79.41% and 88.46%, respectively. Conclusions: CD44 immunostain can effectively distinguish MCNS from FSGS. The congruent results of CD44 positivity with known prognostic factors support the possibility of using the CD44 marker as a predictive tool in selecting high-risk patients and offering appropriate therapeutic measures.

Hemangioma segmentario de la barba / Segmental Beard Hemangioma

Los hemangiomas infantiles son el resultado de la proliferación de células del endotelio vascular y representan los tumores benignos más frecuentes en la infancia, con una incidencia estimada del 4-10% en bebés caucásicos. Se clasifican según el número, la profundidad y la distribución. Dentro de esta última clasificación se encuentran aquellos denominados segmentarios, que se caracterizan por su distribución extensa en áreas de prolongaciones mesodérmicas embrionarias. Se comunica el caso de una paciente evaluada al mes y medio de vida, con un hemangioma extenso del área mandibular y cuello anterior (hemangioma segmentario de la barba). Se describe la importancia de los estudios complementarios para evaluar el compromiso de órganos subyacentes, para detectar síndromes asociados y definir el tratamiento sobre la base de estos resultados. (AU)

Infantile hemangiomas arise from the proliferation of vascular endothelial cells and represent the most common benign tumors in infancy, with an estimated incidence of 4-10% in Caucasian infants. They vary according to their number, depth, and distribution. Within the latter classification are the so-called segmental ones, which feature an extensive distribution in areas of embryonic mesodermal extensions. We report the case of a patient evaluated at one and a half months of life with an extensive hemangioma of the mandibular area and anterior neck (segmental hemangioma of the beard). We describe the importance of complementary studies for evaluating the involvement of underlying organs, detecting associated syndromes, and defining the treatment based on these findings. (AU)

Reconstruction Plate Fracture: Mechanism and Recent Advances

Reconstruction plates with or without bone grafts are used to restore mandibular continuity, form and function following segmental resection of mandible. Fracture of reconstruction plate is observed in 2.9 % to 10% of cases reported in the literature excluding other complications. In this case, we report the fracture of stainless steel reconstruction plate used without bone graft and its management using locking reconstruction plate with non vascularised iliac crest graft following removal of the fractured plate. Review of literature describing incidence, pattern and causes of reconstruction plate fracture and its management is discussed.

Glomerulopatía colapsante en un paciente con Covid-19. Reporte de caso / Collapsing glomerulonephritis in a COVID-19 patient. Case report

La injuria renal aguda por glomerulopatía colapsante, presenta alta morbimortalidad, incluso con requerimiento de diálisis crónica; la Covid-19 es una de sus causas. Se presenta el caso de un paciente con Covid-19 y glomerulopatía colapsante. Varón de 17 años, sin antecedentes patológicos; con historia de cuatro meses de edema, orina espumosa y disminución del flujo urinario. Al examen: anasarca. Exámenes: creatinina 4,2 mg/dl, albumina 1,9 gr/dl, colesterol y triglicéridos aumentados; orina: proteinuria 6,7 gr/24h, leucocituria y hematuria con urocultivo negativo. Serología para VIH, sífilis y hepatitis negativos. Inmunología para lupus negativa, prueba rápida para la Covid-19 IgG (+). La biopsia renal mostró Glomeruloesclerosis Focal y Segmentaria, variante Colapsante. Recibió corticoides y ciclosporina. La creatinina mejoró, la proteinuria se mantiene >3 gr/24horas.

SUMMARY Acute renal injury due to collapsing glomerulonephritis is associated with high morbidity and mortality, requiring chronic dialysis, COVID-19 is one of its causes. A 17-year-old male patient presented with a four-month history of edema, foamy urine and reduction in the urine flow; anasarca was observed at physical examination. Laboratory values showed creatinine 4,2 mg/dl; albumin 1,9 gr/dl; cholesterol and triglycerides were high; proteinuria 6,7 gr/24h: leucocyturia and hematuria with negative urine culture. Serologies for HIV, syphilis and hepatitis were negative. Studies for systemic lupus were negative. An antigenic test for SARS-CoV-2 was positive as well as an IgG. Renal Biopsy showed Focal and Segmental Glomerulosclerosis, Collapsing variant. He received corticosteroids and cyclosporine. Creatinine improved; proteinuria remained >3 gr/24 hours.

De Novo Focal Segmental Glomerulosclerosis (FSGS) Post Covid 19 Vaccination: Case Series in A Single Centre in Malaysia

@#Introduction: Mass COVID-19 vaccination has been pivotal in the fight against this pandemic. The occurrence of glomerular disease following COVID-19 vaccinations particularly mRNA vaccine has been reported. The reported cases in the region are limited and number of cases reported are low in contrast to the total number of vaccine doses given worldwide, the healthcare providers should be alerted about such issues to provide swift and proper management. Case Series: Here, we report 3 cases of Focal segmental glomerulosclerosis (FSGS) following COVID-19 vaccination and their outcomes. Two of the patients received BNT162b2 vaccination and one received CoronaVac vaccination. The mean age of the patients was 33+/-7 years old. The mean duration onset of FSGS was 23+/-19 days post vaccinations. Two of the patients (BNT162b2 vaccination and CoronaVac vaccination) achieved complete remission after corticosteroid therapy. This is the first reported case of De Novo FSGS following CoronaVac vaccination in the literature. The third patient, who received BNT162b2 vaccination and presented late (42 days post vaccination) was not in remission despite three months of immunosuppressive treatment. Conclusion: The treating physician needs to be aware of the possibility of the development of FSGS associated with COVID-19 vaccination and how to proceed with vaccination schedule in these populations. Overall, the advantage of COVID-19 vaccination far outweighs the possibility of COVID-19 vaccine-associated glomerular disease.

Significance of target volume delineation of tumor bed according to metal clips or seroma in determing tumor bed position during radiotherapy after breast-conserving surgery for breast cancer / 肿瘤研究与临床

Objective:To investigate the differences between the mental clips placed intraoperatively and the tumor bed's target volume delineation of seroma based on CT scanning during radiotherapy for breast cancer patients who received breast-conserving surgery in the persuit of a better solution to determine the tumor bed position.Methods:The clinical data of 13 patients with early breast cancer who received postoperative radiotherapy after breast-conserving surgery at Beijing Shijingshan Hospital and Beijing Shijitan Hospital of Capital Medical University from December 2020 to January 2022 were retrospectively analyzed. They all had surgical clips implanted during the surgery. The following methods were used to delineate the target volume of tumor bed, including gross target volume delineation of tumor bed based on the mental clips (GTVtb-Clip), the tumor bed's gross target volume delineation of seroma based on CT scanning (GTVtb-Seroma), and the combination of both (GTVtb-C+S). The volume, diameter on three coordinate axis, neutral point displacement and conformability of these delineation methods were compared.Results:The volume of GTVtb-Clip, GTVtb-Seroma and GTVtb-C+S was (25±10) cm 3, (38±17) cm 3, (49±20) cm 3, and the differences were statistically significant (all P<0.05). The diameter on X axis was (4.7±1.2) cm, (5.3±1.4) cm, (5.7±1.6) cm, respectively in GTVtb-Clip, GTVtb-Seroma and GTVtb-C+S; the diameter on Y axis was (4.6±1.7) cm, (5.0±1.6) cm, (5.7±1.7) cm, respectively in GTVtb-Clip, GTVtb-Seroma and GTVtb-C+S; the diameter on Z axis was (4.4±1.5) cm, (5.2±1.4) cm, (5.6±1.4) cm in GTVtb-Clip, GTVtb-Seroma and GTVtb-C+S. The differences in the diameter of GTVtb-Clip and GTVtb-C+S on X,Y, Z axis were statistically significant (all P<0.05); the differences in the diameter of GTVtb-Seroma and GTVtb-C+S on X, Z axis were statistically significant (all P<0.05); the difference in the diameter of GTVtb-Clip and GTVtb-Seroma on X axis was statistically significant ( P<0.05) .Neutral point displacement was (5.8±1.6) cm, (5.5±1.9) cm, (6.0±1.7) cm, respectively of GTVtb-Clip, GTVtb-Seroma, GTVtb-C+S, and the difference was not statistically significant ( P>0.05). Conformability of GTVtb-Clip and GTVtb-Seroma, GTVtb-Clip and GTVtb-C+S, GTVtb-Seroma and GTVtb-C+S was 0.412±0.112, 0.525±0.095, 0.774±0.112,respectively, and the differences were statistically significant (all P<0.05). Conclusions:During radiotherapy after breast-conserving surgery for breast cancer, compared with the single method, the combination of GTVtb-Clip and GTVtb-Seroma can better cover the real tumor bed, thus reducing the omission of tumor bed and recurrence rate. CT position should better take place at 4 to 8 weeks for patients receiving radiotherapy after breast-conserving surgery, and target volume of tumor bed will be delineated based on the postoperative changes of both mental clips and seroma.

Long-term prognostic analysis of IgA nephropathy with focal segmental glomerulosclerosis in children / 中华肾脏病杂志

Objective:To analyze the long-term prognosis of IgA nephropathy (IgAN) with focal segmental glomerulosclerosis (FSGS) and the risk factors related to renal prognosis in children with IgAN-FSGS.Methods:A retrospective study was concluded in IgAN-FSGS children who were followed up for more than 5 years and diagnosed by renal biopsy for the first time in the Eastern Theater General Hospital from January, 2004 to December, 2018. The end-point events of the study were entering end-stage kidney disease (ESKD) or estimated glomerular filtration rate (eGFR) decreased by ≥50% from baseline, which were defined as poor renal prognosis. Baseline clinicopathologic data of IgAN-FSGS children were compared between the end-point event group and the non-end-point event group. The cumulative renal survival rate of IgAN-FSGS children was calculated by Kaplan-Meier survival analysis. The influencing factors of poor renal prognosis in IgAN-FSGS children were analyzed by Cox proportional hazards model, and the diagnostic value was evaluated by the receiver operating characteristic curve (ROC curve) and area under the curve (AUC). The diagnostic value was verified by time dependent-ROC and time dependent-AUC.Results:A total of 204 IgAN-FSGS children were enrolled in this study, of whom 132 cases were males (64.7%). The median age of renal biopsy was 16 (14, 17) years old. During a median follow-up time of 90.7 (71.7, 114.8) months, 57 cases (27.9%) reached the end-point events. Compared with the non-end-point event group ( n=147), the end-point event group ( n=57) had higher proportions of males and hypertension, higher levels of 24-hour urinary protein, serum creatinine, serum uric acid, urinary N-acetyl-β- D-glucosaminidase, urinary retinol binding protein, higher proportions of glomerular segmental sclerosis (S1) ≥25% and tubular atrophy/interstitial fibrosis (T1/T2), and lower levels of serum albumin, serum IgA, and serum IgG (all P<0.05). There was no statistical difference between the two groups in treatment (all P>0.05). Kaplan-Meier survival analysis showed that with entry of ESKD or eGFR decreased by ≥50% from baseline as the end-point events, the 5-year, 10-year, and 15-year cumulative renal survival rates in IgAN-FSGS children were 88.7%, 67.6%, and 50.7%, respectively. Multivariate Cox regression analysis showed that proteinuria >1 g/24 h ( HR=3.702, 95% CI 1.657-8.272, P=0.001), hyperuricemia ( HR=3.066, 95% CI 1.793-5.245, P<0.001), S1≥25% ( HR=2.017, 95% CI 1.050-3.874, P=0.035), T1/T2 ( HR=1.863, 95% CI 1.021-3.158, P=0.016) were the independent related factors for poor renal prognosis. ROC curve analysis showed that S1≥25% ( AUC=0.605, P=0.021, sensitivity 26.3%, specificity 94.6%), T1/T2 ( AUC=0.624, P=0.006, sensitivity 43.9%, specificity 81.0%), hyperuricemia ( AUC=0.658, P<0.001, sensitivity 52.6%, specificity 78.9%), proteinuria>1 g/24 h ( AUC=0.670, P<0.001, sensitivity 87.7%, specificity 46.3%) could accurately predict the renal outcome of IgAN-FSGS. Time dependent-ROC curve validation showed that the combined diagnosis of S1≥25%, T1/T2, hyperuricemia and proteinuria>1 g/24 h had a good predictive value for renal prognosis (3-year AUC=0.846 and 5-year AUC=0.777, respectively). Conclusions:During a median follow-up of 90.7 months, 27.9% of IgAN-FSGS children have poor renal prognosis, and the 5-year, 10-year, and 15-year cumulative renal survival rates are 88.7%, 67.6%, and 50.7%, respectively. Urinary protein >1 g/24 h, hyperuricemia, T1/T2, and S1 ≥25% are the risk factors for renal prognosis in IgAN-FSGS children.

Application of preoperative localization coupled with CT three-dimensional reconstruction in pulmonary nodule-centered uniportal thoracoscopic combined subsegmental/segmental resection / 中国胸心血管外科临床杂志

@#Objective     To assess the clinical value of preoperative localization coupled with computed tomography (CT) three-dimensional reconstruction in pulmonary nodule-centered uniportal thoracoscopic combined subsegmental/segmental resection. Methods     The clinical data of 30 patients of combined subsegmental/segmental resection in our hospital from December 2019 to October 2021 were retrospectively collected. There were 19 males and 11 females with the mean age of 56.4 (32.0-71.0) years. The pulmonary nodules were located by CT-guided injection of glue before operation. The three-dimensional reconstruction image and operation planning were carried out by Mimics 21.0 software. Results    The operations were all successfully performed, and there was no conversion to open thoracotomy or lobectomy. The mean tumor diameter was 11.6±3.5 mm, the mean distance between the nodule and the visceral pleura was 13.6±5.6 mm, the mean width of the actual cutting edge was 25.0±6.5 mm, the mean operation time was 110.2±23.8 min, the mean number of lymph node dissection stations was 6.5±2.4, the mean amount of intraoperative bleeding was 50.8±20.3 mL, the mean retention time of thoracic catheter was 3.2±1.1 d, and the mean postoperative hospital stay was 4.5± 1.7 d. There was 1 patient of subcutaneous emphysema, 1 patient of atrial fibrillation and 1 patient of blood in sputum. Conclusion     Preoperative CT-guided injection of medical glue combined with CT three-dimensional reconstruction of pulmonary bronchus and blood vessels is safe and feasible in pulmonary nodule-centered uniportal thoracoscopic  combined subsegmental/segmental resection, which ensures the surgical margin and reserves lung tissues.

Clinicopathological features analysis of focal segmental glomerulosclerosis after kidney transplantation / 器官移植

Objective To investigate the clinicopathological features of recurrent and de novo focal segmental glomerulosclerosis (FSGS) after kidney transplantation. Methods Thirty-four recipients pathologically diagnosed with FSGS by renal allograft biopsy were enrolled in this clinical trial. According to the detection of primary diseases of renal allografts and circulating permeability factors, 34 recipients were divided into the recurrent FSGS group (n=12) and de novo FSGS group (n=22). The differences of clinical indexes and the degree of pathological injury of renal allografts were compared between two groups. Results There was no significant difference in the mesangial hyperplasia score, glomerulosclerosis rate, renal tubular atrophy score, interstitial fibrosis score and podocyte proliferation rate between two groups (all P > 0.05). In the recurrent FSGS group, segmental glomerulosclerosis rate of the recipients was 0.10 (0.08, 0.27), lower than 0.19 (0.13, 0.33) in the de novo FSGS group (P < 0.05). No significant difference was found in the incidence of antibody-mediated rejection, drug-induced renal tubular injury and BK virus infection between two groups (all P > 0.05). The incidence of T cell-mediated rejection in the recurrent FSGS group was 17%, lower than 55% in the de novo FSGS group (P < 0.05). Immunohistochemical staining showed that the infiltrating inflammatory cells in the renal allografts were mainly T lymphocytes. The positive rates of C4d deposition in peripheral capillaries between the recurrent and de novo FSGS groups were 33% (4/12) and 32% (7/22), with no significant difference (P > 0.05). Immunofluorescence results revealed IgM deposition in the segmental glomerulosclerosis area of renal allografts in most cases. Electron microscopy showed extensive fusion or segmental distribution of podocytes in the glomerulus of renal allografts. Conclusions The degree of renal functional injury and the incidence of T cell-mediated rejection in the recurrent FSGS group are lower than those in the de novo FSGS group. Comprehensive analysis of preoperative and postoperative clinical manifestations, laboratory testing and pathological examination of kidney transplant recipients contribute to early diagnosis and treatment of recurrent and de novo FSGS.

Role of suPAR in the Pathogenesis of Podocyte Injury of Primary Focal Segmental Glomerulosclerosis / 中山大学学报(医学科学版)

ObjectiveThis study aims to investigate the role of suPAR in the pathogenesis of podocyte injury in FSGS. Methods① The sera of primary FSGS patients （17 cases） were collected. Healthy volunteers （10 cases） and patients with other types of primary nephrotic syndrome （10 cases） were set as normal and disease controls. SuPAR levels were detected by ELISA； ② Podocytes were stimulated by suPAR in vitro， and cells were collected to analyze apoptosis by flow cytometry and for RNAseq analysis； ③ Differentially expressed genes （DEGs） were screened from RNAseq data. Both up-regulated and down-regulated genes were analyzed by KEGG and GO enrichment analysis. Heat map was used to show expression of genes related to podocyte focal adhesion， slit diaphragm and actin dynamics and endocytosis. Differentially expressed genes were verified by qPCR. Results① The level of suPAR in FSGS patients was significantly increased， and that in other nephrotic syndrome（NS） patients was also significantly increased； ② suPAR stimulation significantly altered the transcriptome pattern of human podocytes. A total of 272 up-regulated genes and 288 down-regulated genes were screened； ③ KEGG and GO enrichment analysis of up-regulated and down-regulated genes showed that Focal adhesion and DNA replication and DNA repair related pathways were significantly down-regulated； ④ suPAR did not increase podocyte apoptosis. ConclusionThe level of suPAR is significantly increased in patients with primary FSGS. SuPAR may promote podocyte injury by interfering with genomic homeostasis and disrupting focal adhesion， slit diaphragm， actin dynamics and endocytosis-related functional molecules of podocytes.

Observation of early clinical efficacy of unilateral biportal endoscopy technique in the treatment of lumbar postoperative adjacent segmental diseases / 中国骨伤

OBJECTIVE@#To investigate early clinical efficacy of unilateral biportal endoscopy technique for the treatment of lumbar postoperative adjacent segmental diseases.@*METHODS@#Fourteen patients with lumbar postoperative adjacent segmental diseases were treated with unilateral biportal endoscopy technique from June 2019 to June 2020. Among them, there were 9 males and 5 females, aged from 52 to 73 years old, and the interval between primary and revision operations ranged from 19 to 64 months. Adjacent segmental degeneration occurred after lumbar fusion in 10 patients and after lumbar nonfusion fixation in 4 patients. All the patients received unilateral biportal endoscopy assisted posterior unilateral lamina decompression or unilateral approach to the contralateral decompression. The operation time, postoperative hospital stay and complications were observed. The visual analogue scale (VAS) of low back pain and leg pain, Oswestry Disability Index (ODI), modified Japanese Orthopaedic Association (mJOA) score were recorded before operation and at 3 days, 3 months, and 6 months after operation.@*RESULTS@#All procedures were successfully completed. Surgical duration ranged from 32 to 151 min. Postoperative CT showed adequate decompression and preservation of most joints. Out of bed walking 1 to 3 days after surgery, postoperative hospital stay was 1 to 8 days, and postoperative follow-up was 6 to 11 months. All 14 patients returned to normal life within 3 weeks after surgery, and VAS, ODI, and mJOA scores improved significantly at 3 days and 3, 6 months after surgery. One patient occurred cerebrospinal fluid leak after operation, received local compression suture, and the wound healed after conservative treatment. One patient occurred postoperative cauda equina neurologic deficit, which was gradually recovered about 1 month after rehabilitation therapy. One patients advented transient pain of lower limbs after surgery, and the symptoms were relieved after 7 days of treatment with hormones, dehydration drugs and symptomatic management.@*CONCLUSION@#Unilateral biportal endoscopy technique has a good early clinical efficacy in the treatment of lumbar postoperative adjacent segmental diseases, which may provide a new minimally invasive, non-fixation option for the treatment of adjacent segment disease.

Peritumoral infiltration of local anesthetic before surgery in early breast cancer: a comment

This is a comment on a study recently published about peritumoral infiltration of local anesthetic before surgery in early breast cancer. Previously, animal models and a randomized study for stage IV breast cancer patients inferred that the removal of the primary tumor resulted in increased growth factors and worse distant disease control. Therefore, breast cancer surgery might not be a strictly local intervention. In this new randomized study, the intervention was a peritumoral infiltration of local anesthetic ­ lidocaine 0.5% in the six tumor margins, as an attempt to limit the systemic repercussions of surgery. Although the adjuvant treatment available for the study seems outdated, leading us to question the external validation, limited resources may have increased the power of surgery. Unknown mechanisms during surgery can change the patient's journey, and it is our duty to look at surgical studies with due seriousness

Focal segmental glomerulosclerosis in a patient with congenital lecithin-cholesterol acyltransferase deficiency

Lecithin-cholesterol acyltransferase deficiency is a rare genetic disease caused by a mutation of the gene coding for the lecithin-cholesterol acyltransferase protein, and mainly affects low density lipoprotein metabolism. It typically manifests with diffuse corneal opacities, normocytic anemia and kidney disease. We present the case of a 30-year-old man with chronic kidney disease and nephrotic syndrome. His initial kidney biopsy showed focal segmental glomerulosclerosis, thought to be primary, a disease which was refractory to multiple immunosuppressive schemes. Manifestations such as anemia, splenomegaly, corneal opacities and an association with low high-density lipoproteins alerted to the possibility of glomerular damage secondary to lecithin-cholesterol acyltransferase enzyme deficiency, which was confirmed through genetic sequenc ing. Due to the low incidence of the disease, diagnosis is a clinical challenge. The signs and symptoms tend to be interpreted as isolated events, which significantly delays its confirmation. Understanding this entity and the clinical exercise needed to arrive at its diagnosis will serve as a reference, resulting in the suspicion and reporting of cases in the future. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2558).

La deficiencia de lecitin-colesterol aciltransferasa es una enfermedad genética rara, causada por una mutación en el gen que codifica la proteína lecitin-colesterol aciltransferasa y afecta principalmente el metabolismo de las lipoproteínas de baja densidad. Se manifiesta típicamente con opacidades corneales difusas, anemia normocítica y enfermedad renal. Se presenta el caso de un hombre de 30 años con enfermedad renal crónica y síndrome nefrótico, con biopsia renal inicial que demostró un patrón de glomeruloesclerosis focal y segmentaria, interpretada como primaria, enfermedad que fue refractaria a múltiples esquemas de inmunosupresión. Las manifestaciones como anemia, esplenomegalia, opacidades corneales y la asociación con lipoproteínas de alta densidad bajas, alertaron sobre la posibilidad de compromiso glomerular secundario a un déficit de la enzima lecitin-colesterol aciltransferasa, confirmado mediante estudio de secuenciación genética. Dada la baja incidencia de la enfermedad, el diagnóstico representa un desafío clínico. Las manifestaciones suelen interpretarse como eventos aislados, lo que lleva a retraso significativo en su confirmación. El conocimiento de esta entidad y el ejercicio clínico necesario para llegar al diagnóstico, servirán como referencia que derive en la sospecha y reporte de futuros casos. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2558).

Takayasu arteritis associated with focal and segmentary glomerulosclerosis

Takayasu arteritis (TA) is a large vessel vasculitis that affects young people, related to cardiovascular outcomes and chronic kidney disease. We present the case of a 20-year-old male with a diagnosis of TA, who developed chronic kidney disease, impaired renal blood flow was ruled out, renal biopsy was compatible with focal and segmental glomerulosclerosis of a collapsing variety, other possible aetiologies were excluded. The mechanisms that mediate this association have not been determined, immune-mediated mechanisms are proposed. According to our review, this is the second reported case of this association and the first with a collapsing variety.

La arteritis de Takayasu es una vasculitis de grandes vasos que afecta a personas jóvenes y se relaciona con desenlaces cardiovasculares y enfermedad renal crónica. Se presenta el caso de un paciente masculino de 20 arios, con diagnóstico de arteritis de Takayasu, que desarrolla enfermedad renal crónica. Se descartan alteraciones en el flujo sanguíneo renal, en tanto que la biopsia renal resulta compatible con glomeruloesclerosis focal y segmentaria de variedad colapsante. Se excluyeron otras posibles etiologías. No se han determinado los mecanismos que median en esta asociación; se proponen mecanismos inmunomediados. Según nuestra revisión, se trata del segundo caso reportado de esta asociación y el primero con variedad colapsante.

Oncological Outcomes of Nipple-Sparing Mastectomy in an Unselected Population Evaluated in a Single Center / Desfechos oncológicos da mastectomia poupadora do complexo areolo-mamilar em uma população não selecionada avaliada em um único centro

Abstract Objective Nipple-sparing mastectomy (NSM) has been traditionally used in selected cases with tumor-to-nipple distance > 2 cm and negative frozen section of the base of the nipple. Recommending NSM in unselected populations remains controversial. The present study evaluated the oncological outcomes of patients submitted to NSM in an unselected population seen at a single center. Methods This retrospective cohort study included unselected patients with invasive carcinoma or ductal carcinoma in situ (DCIS) who underwent NSM in 2010 to 2020. The endpoints were locoregional recurrence, disease-free survival (DFS), and overall survival (OS), irrespective of tumor size or tumor-to-nipple distance. Results Seventy-six patients (mean age 46.1 years) (58 invasive carcinomas/18 DCIS) were included. The most invasive carcinomas were hormone-positive (60%) (HER2 overexpression: 24%; triple-negative: 16%), while 39% of DCIS were high-grade. Invasive carcinomas were T2 in 66% of cases, with axillary metastases in 38%. Surgical margins were all negative. All patients with invasive carcinoma received systemic treatment and 38% underwent radiotherapy. After a mean of 34.8 months, 3 patients with invasive carcinoma (5.1%) and 1 with DCIS (5.5%) had local recurrence. Two patients had distant metastasis and died during follow-up. The 5-year OS and DFS rates for invasive carcinoma were 98% and 83%, respectively. Conclusion In unselected cases, the 5-year oncological outcomes following NSM were found to be acceptable and comparable to previous reports. Further studies are required.

Resumo Objetivo A mastectomia poupadora do complexo areolo-mamilar (MPM) tem sido tradicionalmente utilizada em casos selecionados com distância tumor-mamilo > 2 cm e biópsia de congelação da base do mamilo negativa. Recomendar MPM em populações não selecionadas continua controverso. Este estudo avaliou os resultados oncológicos de pacientes submetidas à MPM em uma população não selecionada atendida em um único centro. Métodos Coorte retrospectivo incluindo pacientes não selecionadas com carcinoma invasivo ou carcinoma ductal in situ (CDIS) submetidas à MPM entre 2010 e 2020. Os desfechos incluíram: recorrência locorregional, sobrevida livre de doença (SLD) e sobrevida global (SG), independentemente do tamanho do tumor ou da distância tumor-mamilo. Resultados Setenta e seis pacientes (média: 46,1 anos de idade) (58 carcinomas invasivos/18 CDIS) foram incluídas. A maioria dos carcinomas invasivos era hormônio-positivo (60%) (superexpressão de HER2: 24%; triplo-negativo: 16%), enquanto 39% dos CDIS eram de alto grau histológico. Os carcinomas invasivos foram T2 em 66% dos casos, com metástases axilares em 38%. As margens cirúrgicas foram todas negativas. Todas as pacientes com carcinoma invasivo receberam tratamento sistêmico e 38% receberam radioterapia. Após um período médio de 34,8 meses, 3 pacientes com carcinoma invasivo (5,1%) e 1 com CDIS (5,5%) apresentaram recidiva local. Durante o acompanhamento, duas pacientes tiveram metástase à distância e vieram a óbito. As taxas de SG e SLD aos 5 anos para carcinoma invasivo foram de 98% e 83%, respectivamente. Conclusão Em casos não selecionados, os resultados oncológicos de 5 anos após MPM foram considerados aceitáveis e comparáveis a resultados anteriores. Estudos adicionais são necessários.

Evaluation of Ocular Findings in Vitiligo

Vitiligo is a pigmentary disorder of the skin and mucous membranes with worldwide incidence of 0.5-2%. The aim of this cross sectional study was to assess the ocular involvement in vitiligo patients. Two hundred patients clinically diagnosed vitiligo attending vitiligo clinic, dermatology department, Jomhorya hospital, Benghazi-Libya over a period of 6 months enrolled in this cross-sectional study. All patients were exposed to detailed disease history and thorough dermatological examination. Out of the total cases, 100 patients had a standard Ophthalmologic examination in cooperation with an ophthalmologist in Benghazi eye hospital. Results of our study showed a slightly higher prevalence of vitiligo in females (male: female ratio 1:2.8). The mean age of onset for males was 22.4 years and for females 24.8 years and 58.5% of patients develop vitiligo below 20 years of age. Generalized vitiligo was the commonest clinical type of vitiligo (57.5%). The relationship between the duration of the disease and its type was statistically significant (P<0.05), there was also a significant relationship between the type of vitiligo and its severity (P< 0.01). Sixteen patients (8%) had segmental vitiligo and the face was the common site involved. The lower limbs were the most commonly affected sites of the body (57%). Ocular changes were seen in 40% of patients and specific ocular abnormalities like uveitis, iritis, and iris and retinal pigmentary abnormalities are present in 15% of patients, nonspecific ocular abnormalities were present in 33% of patients. There was no statistically significant correlation between specific ocular abnormalities, severity and duration of vitiligo.

Neurofibromatosis segmentaria, a propósito de un caso / Segmental neurofibromatosis, case report

Resumen Introducción: la neurofibromatosis es un desorden genético, que afecta el crecimiento de tejidos neurales, con una incidencia de 1 en 4 000 personas, con impacto en la esperanza de vida por su asociación con neoplasias y enfermedad vascular. La neurofibromatosis segmentaria es una variante de la neurofibromatosis tipo 1, con una incidencia aproximada de 1 en 20 000 a 25,000 personas, se caracteriza por lesiones cutáneas que afectan un segmento corporal sin cruzar la línea media. Generalmente no tienen historia familiar ni compromiso sistémico. Caso clínico: paciente de sexo femenino de 63 años con dermatosis que afecta el tronco posterior de manera unilateral a nivel de los dermatomas T10-T11, caracterizada por múltiples neoformaciones exofíticas milimétricas en forma de domo, de consistencia blanda y depresibles a la palpación. El estudio histopatológico de una de ellas confirmó el diagnóstico de neurofibroma. La paciente no presentaba afectación neurológica ni ocular, además, sin afección en familiares, por lo que se establece diagnóstico de neurofibromatosis segmentaria. Conclusiones: la neurofibromatosis segmentaria es una patología poco frecuente, Aunque posiblemente sea subdiagnosticada por su carácter asintomático, lo que ocasiona una aparente baja incidencia. Los pacientes que la padecen pueden presentar penetrancia sistémica variable y un riesgo similar de neoplasias al descrito en pacientes con neurofibromatosis tipo 1. Pese al carácter benigno reportado en la literatura sugerimos un abordaje multidisciplinario de los pacientes.

Abstract Introduction: neurofibromatosis is a genetic disorder that affects the growth of neural tissues, with an incidence of 1 in 4 000, with impact on life expectancy due its association with neoplasms and vascular disease. segmental neurofibromatosis is a subtype of neurofibromatosis type 1, with an approximate incidence of 1 in 20 000 to 25 000 people, it is characterized by skin lesions that affects a body segment without crossing midline, they generally have no family history or systemic involvement. Clinical case: a 63-year-old female patient with dermatosis affecting the posterior trunk unilaterally at the level of dermatomes t10-t11, characterized by multiple exophytic, dome-shaped, millimeter sized neoformations, soft in consistency and depressible on palpation. the histopatologic study of one of them confirmed the diagnosis of neurofibroma. the patient did not present neurological or ocular involvement, without affection in relatives. diagnosis of segmental neurofibromatosis was made. Conclusions: segmental neurofibromatosis is a rare pathology, although it is possibly underdiagnosed due its asymptomatic nature, which causes an apparent low incidence. patients may present variable systemic penetrance and a similar risk of neoplasm compared to patients with neurofibromatosis type 1. despite the benign nature reported in the literature, we suggest a multidisciplinary approach to patients.

Salvage Nipple-sparing Mastectomy for Patients with Breast Cancer Recurrence: A Case Series of Brazilian Patients / Mastectomia preservadora de mamilo para pacientes com recidiva de câncer de mama: Uma série de casos de pacientes brasileiras

Abstract Objective Few studies analyzed the safety of salvage nipple-sparing mastectomy (NSM) for local relapse treatment. We evaluated the outcomes of patients with indications for mastectomy who chose to undergo NSM for ipsilateral breast tumor recurrence (IBTR). Methods Between January 2001 and December 2018, we evaluated 24 women who underwent NSM for local relapse after conservative surgery. Results Thepatientswere followedupfor amean time of132months since thefirst surgery. After the NSM, 5 (20.8%) patients were diagnosed with local recurrence and only 1 (4.2%) patient died. The patients presented 4.8% (2) of partial and 2.4% (1) of total nipple necrosis. Conclusion In this long-term follow-up since the first surgery, we observed low rates of complication and good survival, although associated with high local recurrence in patients diagnosed with IBTR undergoing NSM as salvage surgery.We demonstrated that NSMmay be considered after IBTR for patients who did not want to undergo total mastectomy.

Resumo Objetivo Há poucos estudos sobre a segurança de se realizar adenomastectomia (nipple-sparing mastectomy, NSM, em inglês) para tratamento de recidiva local. O objetivo deste estudo foi avaliar os resultados de pacientes com indicação para mastectomia que optaram por se submeter a NSM para o tratamento de recorrência local. Métodos Foram analisadas 24 pacientes submetidas a NSM para tratamento de recidiva local após tratamento conservador entre janeiro de 2001 e dezembro de 2018. Resultados As pacientes foramacompanhadas por um períodomédio de 132meses a partir da primeira cirurgia. Após a NSM, 5 (20,8%) pacientes foram diagnosticadas com recorrência local, e apenas 1 paciente foi a óbito. As pacientes apresentaram 4,8% de necrose parcial e 2,4% de necrose total do mamilo. Conclusão Em um longo período de acompanhamento desde a primeira cirurgia, foram observadas baixas taxas de complicação pós-operatória e boa sobrevida, porém, associadas comuma alta taxa de recorrência local em pacientes submetidas a NSM para tratamento de recidiva local após cirurgia conservadora. Neste estudo, demonstrou-se que a NSM pode ser considerada uma opção cirúrgica para pacientes que não querem se submeter a mastectomia total.

An alternative method for reconstructing anterior segmental mandibulectomy defects with a plate and pectoral major muscle flap

Background: Reconstruction of anterior segmental mandibulectomy still remains a challenge. Osteocutaneous free flap remains the ideal choice of reconstruction because it restores cosmesis and function. The use of other locoregional flaps compromises cosmesis and function. Here, we have introduced a unique technique of reconstruction with lingual cortex mandibular plate as an alternate choice for free flap. Method: Six patients aged 12–62 years underwent oncological resection for oral cancer which involved the anterior segment of the mandible. Following resection, they underwent lingual cortex mandibular plating with pectoralis major myocutaneous flap reconstruction. Adjuvant radiotherapy was delivered to all patients. Results: The mean bony defect was 9.2 cm. There were no significant perioperative events related to the surgery. None required tracheostomy and all were safely extubated with no post-surgical complications. The cosmetic and functional outcomes were acceptable. Following the completion of radiotherapy with a median follow-up of 11 months, Plate exposure occurred in one patient. Conclusion: The technique is cheap, quick and simple, and can be effectively applied in resource-constrained and demanding situations. It is possible to consider this as an alternative treatment strategy for osteocutaneous free flap in anterior segmental defects

A comparison between different management surgical approaches in the treatment of splenic flexure colon cancer

Background: There are many surgical approaches which described extent of resection of the colon for adequate surgicalmanagement of splenic flexure cancer, but up till now there is no established surgical procedure, this is because the presence of double lymphatic drainage of themesenteric vessels. Segmental resection of the colon for the management of splenic flexure cancer was a recently accepted surgical procedure. Objective: In the present study, we aimed to compare three surgical management techniques to clarify the best management approach of Egyptian patients with splenic flexure cancer regarding operative, clinical, and oncological outcomes: segmental resection, and extended left or right hemicolectomy,. Materials and Methods In the present study, we included 90 patients with splenic flexure cancer. Cases were divided into 3 groups. Each group included 30 patients in order to compare three surgical techniques: segmental resection, extended left hemicolectomy, and extended right hemicolectomy. Results: We have found no statistically significant differences between the three included groups regarding operative findings, postoperative complications, local recurrence, distant recurrence, disease progression, recurrence-free survival rate, progression-free survival rate, and overall survival rate. The operative time was longer, and the number of lymph nodes was higher in the extended right hemicolectomy group (p<0.001). Conclusion: We have shown that segmental resection of the splenic flexure is surgically and clinically suitable for the adequate management of operable cases of carcinoma of the splenic flexure. (AU)