Discrepancies between clinical and pathological findings seen at renal biopsy in rheumatological diseases.

OBJECTIVE: Renal biopsy contributes to the diagnosis, follow-up, and treatment of many rheumatic conditions. This study assessed the diagnostic role and safety of renal biopsies in a tertiary rheumatology clinic. METHODS: Renal biopsies performed between June 2020 and December 2022 were screened, and demographic, clinical, histopathological, and safety data were collected from patient records. RESULTS: In this study, 33 males and 38 females were included. Except for 1 patient who received acetylsalicylic acid, antiaggregant, and/or anticoagulant drugs were stopped before the biopsy. Complications included a decrease of hemoglobin in 8 patients (11.3%) and microscopic hematuria in 40 patients (56.3%). Control ultrasonography was performed in 16 patients (22.5%), and a self-limiting hematoma was found in 4 of them (5.6%) without additional complications. While less than 10 glomeruli were obtained in 9 patients (9.9%), diagnosis success was 94.4%. Histopathological data were consistent with one of the pre-biopsy diagnoses in 54 of 67 cases (80.6%) but showed discrepancies in 19.4% (n=13) of patients. A repeat biopsy was performed in 7 patients for re-staging or insufficient biopsy. CONCLUSIONS: Renal biopsy significantly contributes to rheumatology practice, especially in patients with complex clinical and laboratory findings or in whom different treatments can be given according to the presence, severity, and type of renal involvement. Although the possibility of obtaining insufficient tissue and the need for re-staging and repeat biopsy in the follow-up might be expected, complication risk does not seem to be a big concern. Renal biopsy often evidenced discrepancies between pre-biopsy diagnosis and histopathological findings.

Serum and urine TNF-like weak inducer of apoptosis, monocyte chemoattractant protein-1 and neutrophil gelatinase-associated lipocalin as biomarkers of disease activity in patients with systemic lupus erythematosus.

OBJECTIVES: TNF-like weak inducer of apoptosis (TWEAK), monocyte chemoattractant protein-1 (MCP-1) and neutrophil gelatinase-associated lipocalin (NGAL) are proinflammatory cytokines/chemokines that are considered as potential biomarkers reflecting disease activity in systemic lupus erythematosus (SLE). In this study, we aimed to investigate the association of serum (s) and urine (u) levels of TWEAK, MCP-1 and NGAL with disease activity in both renal and extra-renal SLE. METHODS: Thirty active patients with SLE (15 renal and 15 extra-renal) were recruited. Thirty-one inactive patients with SLE (16 renal and 15 extra-renal), 14 patients with ANCA-associated vasculitis (AAV) all of whom had active renal involvement and 20 healthy volunteers were selected as control groups. Serum and urine levels of TWEAK, MCP-1 and NGAL were tested using ELISA. RESULTS: Serum and urine levels of TWEAK and NGAL were significantly higher in the active SLE group compared to the inactive SLE group (sTWEAK p = 0.005; uTWEAK p = 0.026; sNGAL p < 0.001; uNGAL p = 0.002), whilst no significant differences regarding serum and urine MCP-1 levels were observed (p = 0.189 and p = 0.106, respectively). uTWEAK (p = 0.237), sMCP-1 (p = 0.141), uMCP-1 (p = 0.206), sNGAL (p = 0.419) and uNGAL (p = 0.443) levels did not differ between patients with active renal and extra-renal SLE. Serum TWEAK was higher in patients with active renal SLE (p = 0.006). There were no differences between active renal SLE and active renal AAV. Levels of all biomarkers were correlated with the SLE Disease Activity Index. CONCLUSION: sTWEAK, uTWEAK, sNGAL and uNGAL are biomarkers showing disease activity in SLE. However, our results implicate that these biomarkers may not be specific for SLE, and can be elevated in patients with active renal involvement of AAV.

Juvenile systemic lupus erythematosus in Turkey: demographic, clinical and laboratory features with disease activity and outcome.

Objectives This paper aims to assess in a retrospective fashion the clinical and laboratory features, severity and outcome of juvenile systemic lupus erythematosus (jSLE) from a referral center in Turkey. Methods We have included all jSLE patients ( n = 92) diagnosed according to the revised American College of Rheumatology 1997 criteria between January 2004 and January 2017. Results The most prevalent clinical feature in our cohort was mucocutaneous manifestations (97.8%), followed by constitutional (81.5%), hematological (59.8%) and musculoskeletal manifestations (56.5%). Renal involvement was observed in 38% ( n = 35) of the patients, whereas biopsy-proven lupus nephritis was detected in 29.3% ( n = 27) of the cohort. Neurologic involvement was seen in 15 (16.3%) individuals. Among the patients positive for anticardiolipin IgM and/or IgG ( n = 11, 12%), only three developed antiphospholipid antibody syndrome. The mean SLEDAI-2K scores at disease onset (10.5 ± 4.8) showed a substantial decrease at last visit (4.3 ± 4.6). One-quarter of the patients (26.1%, n = 24) had damage according to the PedSDI criteria with a mean score of 0.45 ± 1.0 (range 0-7). When the PedSDI damage items were evaluated individually, growth failure was the most frequent damage criterion ( n = 6), followed by seizure ( n = 5). Two patients died during the designated study period of end-stage renal disease. The five-year and 10-year survival rate of our cohort was 100% and 94.4%, respectively. Conclusions Given the lower frequency of nephritis and central nervous system disease and lower basal disease activity and damage scores, we could conclude that children with jSLE in Turkey have a more favorable course compared to Asian and African American children, as expected from Caucasian ethnicity.

Decorin as a new treatment alternative in Peyronie's disease: preliminary results in the rat model.

The purpose of this study is to investigate the effect of decorin, a naturally occurring proteoglycan with anti-transforming growth factor beta (TGF-ß) activity, on the rat model of Peyronie's disease (PD). Twenty-five adult male Sprague-Dawley rats were divided in three groups: I) TGF-ß (0.5 µg) injected (n: 8); II) TGF-ß injected and decorin treated (n: 8); and III) controls (n: 9). Decorin (0.5 µg per day) was given with intracavernous injection on the second, third, fourth and fifth day following TGF-ß injection. All rats underwent electrical stimulation of the cavernous nerve after 6 weeks. Intracavernosal and arterial blood pressures were measured during this procedure. Cross-sections of the rat penises were examined using Mason trichrome and H&E stains. Statistical analyses were carried out using one-way anova. Histopathological examinations confirmed the Peyronie's-like condition in TGF-ß-injected rats, which exhibited a thickening of the tunica albuginea (TA), when compared to controls. Disorganisation of collagen on the TA was also prominent in TGF-ß-injected rats, but not in decorin-treated and control rats. Decorin-treated rats showed significantly higher maximal intracavernosal pressure (MIP) responses to cavernous nerve stimulation, when compared to group 1 (P < 0.05). Our results indicate that decorin antagonises the effects of TGF-ß in the rat model of PD and prevents diminished erectile response to cavernous nerve stimulation.

Prognostic factors associated with the response to sunitinib in patients with metastatic renal cell carcinoma.

OBJECTIVE: We investigated the prognostic clinicopathologic factors associated with overall survival (os) and progression-free survival (pfs) in the once-daily continuous administration of first-line sunitinib in a consecutive cohort of Turkish patients with metastatic renal cell carcinoma (rcc). METHODS: The study enrolled 77 Turkish patients with metastatic rcc who received sunitinib in a continuous once-daily dosing regimen between April 2006 and April 2011. Univariate analyses were performed using the log-rank test. RESULTS: Median follow-up was 18.5 months. In univariate analyses, poor pfs and os were associated with 4 of the 5 factors in the Memorial Sloan-Kettering Cancer Center (mskcc) score: Eastern Cooperative Oncology Group performance status of 2 or higher, low hemoglobin, high corrected serum calcium, and high lactate dehydrogenase. In addition to those factors, hypoalbuminemia, more than 2 metastatic sites, liver metastasis, non-clear cell histology, and the presence of sarcomatoid features on pathology were also associated with poor pfs; and male sex, hypoalbuminemia, prior radiotherapy, more than 2 metastatic sites, lung metastasis, nuclear grade of 3 or 4 for the primary tumour, and the presence of sarcomatoid features were also associated with poorer os. The application of the mskcc model distinctly separated the pfs and os curves (p < 0.001). CONCLUSIONS: Our study identified prognostic factors for pfs and os with the use sunitinib as first-line metastatic rcc therapy and confirmed that the mskcc model still appears to be valid for predicting survival in metastatic rcc in the era of molecular targeted therapy.

Elderly trauma: the two years experience of a university-affiliated emergency department.

OBJECTIVE: The elderly population is steadily increasing in the modern world. The aging of the population has led to an increase in geriatric trauma. Elderly trauma patients present unique challenges and face more significant obstacles in recovery compared to younger patients. This study is designed to determine the epidemiologic data of trauma in elderly patients and to contribute to the national trauma database. MATERIALS AND METHODS: We prospectively collected the data of trauma patients, aged 65 and older, presenting to our Emergency Department. Patients' data, including demographic data, diagnosis, prognosis, trauma scores [Glasgow coma scale (GCS), injury severity score (ISS)], mortality, body regions of injury and outcomes were analyzed. RESULTS: During the study period, 407 patients, of whom 63.9% were males and 36.1% females, were admitted to our Emergency Department. The mean age of, the patients was 73.14 +/- 7.14 years. Falls (59%) accounted for the most common cause of injury. The mean GCS and mean ISS were 14.39 and 10.69, respectively. Trauma to extremities was the most frequent body region of injury (56.3%). A total of 149 patients were hospitalized. The mean hospital length of stay was 11.07 +/- 14 days. The mortality rate was found to be 6.4%. Head trauma was the most common cause in mortality. CONCLUSIONS: The risk of trauma in the elderly population is higher. As a result of the decrease in physiological reserve and lack of adaptation to trauma, elderly trauma patients require more aggressive management and a multidisciplinary approach. Further studies are needed for data of elderly trauma patients.

Gastric metastasis of malignant fibrous histiocytoma presenting as gastrointestinal bleeding.

Malignant fibrous histiocytoma (MFH) is a soft-tissue sarcoma originating from fibroblast cells, characterized by a high rate of metastasis or recurrence. With only 4 cases described in the available English literature up to now, gastric metastasis of MFH is extremely rare. Among them only one case has been reported to lead to gastrointestinal bleeding. We report the case of a 55-year-old woman who underwent total gastrectomy, 14 months after resection of an MFH from the right side of retroperitoneum. The neoplasm was detected at the time of diagnostic workup for upper gastrointestinal bleeding. The resected specimen contained multiple polypoid nodular lesions which were located in the greater curvature. The clinical and pathological characteristics of gastric metastasis of MFH are presented herein with review of literature.

Urinary CXCL9 and CXCL10 Levels and Acute Renal Graft Rejection.

BACKGROUND: Monitoring of chemokines, CXCL9 and CXCL10, in serum may present a non-invasive detection method for rejection. OBJECTIVE: To investigate the relationship between urinary levels of CXCL9 and CXCL10 and graft function following renal transplantation. METHODS: 75 living-related donor renal transplant recipients were studied. Urinary levels of chemokines were collected pre-operatively, on post-operative 1st day, 7th day, 1st month, 3rd month, and at the time of rejection. Chemokines levels were assayed using and enzyme-linked immunosorbent assay. RESULTS: Clinical variables were monitored. 10 (15%) patients had biopsy-proven rejection during the follow-up period. The urinary CXCL9 level in those with rejection was significantly higher than that in those with non-rejection group at the 1st day (p<0.001), 7th day (p<0.001), and at the time of rejection (p=0.002). The urinary CXCL10 level was also significantly higher in those with rejection compared with non-rejection group at 1st day (p<0.001), 7th day (p<0.001), and at the time of rejection (p=0.001). Serum creatinine level was strongly correlated with the urinary CXCL9 and CXCL10 levels at the time of rejection (r=0.615, p=0.002; and r=0.519, p=0.022, respectively). Among those with T cell-mediated rejections the mean urinary CXCL10 level increased to as high as 258.12 ng/mL. CONCLUSION: Urinary CXCL9 and CXCL10 levels might have a predictive value for T cell-mediated rejection in early post-transplantation period. Measurement of urinary CXCL9 and CXCL10 levels could provide an additional tool for the diagnosis of rejection.

Inflammatory pseudotumor following hematopoietic stem cell transplantation: a new case and review of the literature.

Inflammatory pseudotumor (IPT) is a rare tumor that occurs in various organs and tissues. The clinical picture varies from the more frequent benign lesions to the rare malignant tumors with distant metastases. IPT associated with hematopoietic stem cell transplantation (HSCT) is rarely reported. In this article, we review the reports of IPT after HSCT and describe the first case of bladder IPT. We also review the possible factors involved in the pathogenesis. IPT might be rare but it is a potentially serious complication of HSCT. It should be considered in patients with otherwise unexplained inflammatory symptoms or signs or with any mass lesion in the post-HSCT period. A knowledge of this entity and insistence on a definitive biopsy of mass lesions in the post-HSCT period can avoid unnecessary treatment such as radical surgery, chemotherapy or radiotherapy.

Intraglomerular microlesions in renal angiomyolipoma.

A unique case of bilateral multiple intrarenal angiomyolipomas in a 21-year-old woman with tuberous sclerosis is reported. The microscopic examination showed peculiar intraglomerular microlesions, epithelioid areas, microscopic foci of renal cell carcinoma and renal cysts, and the classical features of angiomyolipoma lesion. HMB-45 positivity was detected within some nodules and epithelioid areas. Intraglomerular microlesions were composed of adipose and smooth-muscle cells within the glomerular capillary tuft. These lesions, which were continuous with the capillary tuft, did not show any attachment to the Bowman's capsule. These findings suggest that these are not a consequence of an infiltration from the outside but were originated from inside the glomerulus. The simultaneous presence of multiple angiomyolipoma nodules either inside or outside the glomeruli, multifocality and bilaterality of these lesions, together with the HMB-45 positivity and the finding of scattered epithelioid areas supports the theory that there is a progenitor cell giving origin to all these lesions, the cell which has been named as perivascular epithelioid cell by most authors.

Cardiac and intestinal amyloidosis in a renal transplant recipient with familial Mediterranean fever.

In Turkey, familial Mediterranean fever (FMF) is an important cause of nephrotic syndrome and endstage renal disease due to renal deposition of AA type amyloid. We report a case of living-related donor renal transplant recipient with FMF and renal AA type amyloidosis, who died of progressive heart failure due to cardiac involvement. The patient also had intractable diarrhea caused by biopsy-proven intestinal amyloidosis. The patient was on 1 mg/day colchicine. Although he was attack-free throughout the post-transplant period, intestinal and clinically significant cardiac amyloidosis, which implied the presence of sustained inflammation and continuing amyloid deposition, appeared three years after renal transplantation. Cardiac deposition of AA amyloid may cause clinically significant heart disease, leading to cardiovascular mortality after renal transplantation for end-stage renal disease in FMF patients.

Clinical outcomes of renal cholesterol crystal embolization.

Cholesterol crystal embolization is an increasingly recognized disease, presenting with a wide clinical spectrum, usually occurring in elderly men who undergo an angiographic procedure or vascular surgery. We report three patients who developed systemic cholesterol embolic disease and varying degrees of renal failure after angiographic interventions of the coronaries.

An intrapericardial teratoma with endocrine function.

Intrapericardial teratoma was diagnosed in a nine-year-old male infant with a three-month history of labored breathing and cough. The tumor was completely resected and found to be a mature teratoma, containing pancreatic tissue and producing insulin. A few glucagon and somatostatin containing cells were also present in the periphery of the islets. Postoperative course was uneventful. This is to our knowledge, the first report of an intrapericardial teratoma with such endocrine activity.

Narrowing of airway caused by ingestion of methyl ethyl ketone peroxide.

Although ingestion of methyl ethyl ketone peroxide (MEKP) is rare, it carries a high risk of morbidity and mortality. This paper reports the first such case from Turkey in which a 70-year-old man unintentionally ingested MEKP in his kitchen. The patient was brought into the emergency department (ED) within 1 hour of ingestion, with the symptoms of sore throat, shortness of breath, nausea and vomiting. Visual examination of the oropharynx revealed minor burns and uvular edema. A laryngoscope examination performed in the ED showed superficial mucosal injury with edema of the oropharynx, uvula, posterior pharynx, epiglottis, arytenoids and vocal cords. Lateral cervical radiography revealed a narrowing of airway due to a thickened epiglottis. After the diagnostic evaluation was completed, the patient was admitted to the gastroenterology intensive care unit for monitoring of adverse reactions. During follow-up, the patient made an uneventful recovery. Ingestion of MEKP generally results from accidental ingestion from a container. Therefore, these containers should be kept in safe places. In addition, the priority following the ingestion of MEKP should be given to maintain open and adequate airway in the ED.

Lymph nodal involvement by renal angiomyolipoma.

Angiomyolipoma of the kidney is a clonal neoplasm, apparently part of a family of neoplasms derived from perivascular epithelial cells. A 40-year-old woman presented with right flank pain and an otherwise non-significant medical history. An abdominal computed tomography scan revealed an 18 cm solid mass in the mid-portion of the right kidney and multiple perihilar lymph nodes. Presumptive diagnosis was renal cell carcinoma. Right radical nephrectomy and a perihilar lymph node dissection was performed through a Chevron incision for the anticipated diagnosis of renal adenocarcinoma. The renal tumor was diagnosed as angiomyolipoma and a component was identified pathologically in a dissected lymph node. There was no evidence of tumor recurrence in the follow-up period of eight years. The consensus from other studies suggests that this phenomenon is a manifestation of the multicentric nature of angiomyolipoma, rather than due to metastasis. Genetic studies may resolve this question in the future.

Cryptorchidism: is orchidopexy always preventive treatment for infertility?

Cryptorchidism is one of the testicular factors recognized as preventable disease processes. Although the effect of bilateral cryptorchidism on fertility is well documented, more studies are required in order to determine the bilateral functional and histologic effects of unilateral cryptorchidism. This clinical retrospective study analyses the effects of cryptorchidism on fertility and histopathologic changes in dystopic and eutopic testes. Among 1400 patients consulted in our infertility clinic between the years of 1989 and 1993, 47 patients (3.3%) had cryptorchidism (29 unilateral/18 bilateral). Among these patients 26 had a history of orchidopexy (mean age at the time of operation: 13.11 +/- 5.24 years). Semen analyses (3x) were available in 43 and hormone profile in 27 out of 47. The incidence of abnormal semen parameters in unilateral and bilateral group was 89.6% and 100%, respectively. Testicular biopsies were obtained from 17 patients (10 with unilateral and 7 with bilateral disease). The biopsy findings in 75% of eutopic testes in unilateral group were similar to that of dystopic testis. The high incidence and significant histologic changes in eutopic testis supports the hypothesis that the disease might be bilateral in nature. The question whether the progression of the disease can be stopped by early orchidopexy still remains unanswered until long term follow-up studies are completed.