Which part of the blood in the knee joint is responsible for its detrimental effects? (An experimental study on the knee joint in rabbits).

AIM: This study aims to determine the potential causative elements which are responsible for the cartilage damage in case of frequent intra-articular bleeding and to evaluate the effects of intra-articular free iron and chelation of iron in the knee joint. METHODS: Thirty-five New Zealand rabbits were randomly divided into five groups according to substances injected into their knee joints. Plasma (group I) and cellular components (group II) of the blood harvested from the rabbits, iron (ferric hydroxide sucrose) (group III), iron&chelator (group IV) and only chelator (deferoxamine mesylate) (group V) were injected into their right knees three times a week for 12 weeks. The joint surface was examined histologically according to the classification system modified from Colombo et al. The changes in the synovial tissue were evaluated according to the scoring system modified from Madhok et al. RESULTS: Cartilage and synovial abnormality scores were significantly higher in all study groups when compared to their own controls (p < 0.0001). Cartilage scores of groups I and V were significantly lower when compared to groups III and IV (p = 0.002 for group I and p = 0.003 for group V). Synovial abnormality score of group I was significantly lower than scores of groups III and IV (p = 0.001); and of group V lower than groups III and IV (p = 0.003 and p = 0.001, respectively). CONCLUSIONS: All substances tested in this study caused a certain amount of damage in the cartilage tissue and led to synovial abnormalities. Both iron and iron&chelator caused more damage in the cartilage and led to more advanced synovial changes when compared to the plasma component of blood and chelator itself. Influence of iron and iron&chelators were found to be similar showing that chelation was inadequate in antagonizing the detrimental effects of iron.

Pulmonary Metastasectomy of Sarcoma: Is the Ratio of Surgical Margin to Nodule Size a Prognostic Factor?

BACKGROUND: Main prognostic factors of improved survival after pulmonary metastasectomy (PM) for osteogenic and soft tissue sarcomas are suggested as histological type, number and size of pulmonary nodules, and disease-free interval (DFI). METHODS: Sixty-nine patients who underwent PM between January 1999 and December 2017 were evaluated retrospectively. Relations between parameters and prognostic risk factors for overall survival (OS) and disease-free survival (DFS) were evaluated. RESULTS: Osteosarcoma was the most common histologic type (36.2%) and 21 of 25 cases were seen under the age 20 years (p < 0.001). Comparison of patient groups including osteosarcoma and nonosteosarcoma patients showed significant difference according to age (p < 0.001), nodule size (p = 0.033), ratio of surgical margin to nodule size (p = 0.007), and DFI (p = 0.039). Univariate analysis showed that the number of nodules (p = 0.008), ratio of surgical margin to nodule size (p = 0.001), and localization of nodule (p = 0.039) were significant factors associated with DFS. Also, nodule size (p = 0.042), number of nodules (p = 0.003), ratio of surgical margin to nodule size (p < 0.001), and laterality (p = 0.027) were significant prognostic factors associated with OS. Cut-off values of ratio of surgical margin to nodule size for DFS and OS were calculated as 0.94. Logistic regression analysis determined the ratio of surgical margin to nodule size as the common significant risk factor for DFS and OS. CONCLUSIONS: Our study showed that the ratio of surgical margin to nodule size ≥ 1 should be taken as a common risk factor for DFS and OS. Therefore, resection of nodules with the possible widest surgical margin is an important point of PM.

Integration of radiology and clinical score in pediatric appendicitis.

BACKGROUND: The efficacy of ultrasonography (US) and abdominal X-ray in combination with Pediatric Appendicitis Score (PAS) is complicated in the diagnosis of acute appendicitis. Abdominal X-ray is as useful as US with clinical assessment when evaluated by experienced pediatric radiologist in acute appendicitis. The aim of this study was to determine the value of US and abdominal X-ray for appendicitis in children when combined with clinical assessment based on PAS, and to establish a practical pathway for acute appendicitis in childhood. METHODS: A prospective, observational cohort study was conducted at an urban, academic pediatric emergency department. Patients were classified at low (PAS 1-4), intermediate (PAS 5-7), or high (PAS 8-10) risk for appendicitis. Low-risk patients were discharged with telephone follow-up in ≤10 days; those at intermediate risk underwent X-ray and US. High-risk patients received immediate surgical consultation. Patients were grouped on histopathology as having either proven acute appendicitis or no appendicitis. RESULTS: A total of 288 children were analyzed. Surgery was performed in 134 patients (46.5%), and 128 (95.5%) had positive histopathology. Mean PAS in the patients with and without appendicitis was 7.09 ± 1.42 and 4.97 ± 2.29, respectively (P = 0.00). The rate of missed cases was 6/288 (2%), and the negative appendectomy rate was 6/134 (4.4%). When the score cut-off was set at 6, the sensitivity and specificity of PAS was 86.7% and 63.1%, respectively. The diagnostic performance of daytime US had a sensitivity of 91.1% and specificity of 71.1%. Also, positive US or PAS >6 or both, had sensitivity and specificity 96.7% and 59.9%, respectively. CONCLUSION: US or abdominal X-ray in children with possible appendicitis should be integrated with PAS to determine the next steps in management. In the case of discordance between the clinical findings and radiology, prolonged observation or further imaging are recommended.

The effects of chemoradiotherapy on recurrence and survival in locally advanced rectal cancers with curative total mesorectal excision: a prospective, nonrandomized study.

BACKGROUND: There are only two prospective, randomized studies comparing preoperative long-term chemoradiotherapy and postoperative chemoradiotherapy in locally advanced rectal cancer (LARC); however, conflicting results in terms of locoregional recurrence (LR) and survival rates have been reported. This prospective study aims to compare the effects of preoperative versus postoperative chemoradiotherapy on recurrence and survival rates in LARC patients. METHODS: From January 2003 to January 2016, a total of 336 eligible patients who were clinically diagnosed with LARC (T3-T4 tm or node-positive disease) were prospectively assigned into preoperative chemoradiotherapy (n = 177) and postoperative chemoradiotherapy (n = 159) groups. The preoperative treatment consisted of 50.4 Gy total dose of radiotherapy (delivered in fractions of 1.8 Gy) and concomitant two cycles chemotherapy of 5-fluorouracil and leucovorin. The patients in the preoperative group underwent curative total mesorectal excision (TME) following long-term chemoradiotherapy. Surgery was performed 8 (range 4-12) median weeks after the completion of the chemoradiotherapy. Similar protocol was administered to the postoperative group 4 weeks after the operation. Four cycles of adjuvant chemotherapy were added to the groups. The primary end points were locoregional recurrences and 5-year cancer-specific, overall, and disease-free survivals. RESULTS: The mean follow-up period was 60.4 (range 12 to 168) months. Five-year cumulative incidence of locoregional recurrence (LR) was 7.4% in the preoperative group and 13.4% in the postoperative group (p = 0.021). Five-year cancer-specific survival (CSS) was 87.5% in the preoperative group and 80% in the postoperative group (p = 0.022). Overall survival (OS) was 79.8 versus 74.7% (p = 0.064), disease-free survival (DFS) was 75.2 versus 64.8% (p = 0.062), and severe late toxicity was 7.4 versus 13.2% (p = 0.002), respectively. The rate of patient compliance was higher in the preoperative group (p < 0.001). CONCLUSIONS: Preoperative chemoradiotherapy, as compared with postoperative chemoradiotherapy, significantly improved local control, patient compliance, CSS, and late toxicity and suggested a trend toward improved overall and disease-free survival.

Clinical and morphological characteristics of osteoid osteoma and osteoblastoma: a retrospective single-center analysis of 204 patients.

Osteoid osteoma and osteoblastoma are histologically similar, benign bone-forming tumors. In this retrospective study, we aimed to evaluate the natural history; clinical, pathologic, and radiologic findings; and treatment results in 204 patients between 1959 and 2006 in a single institution. According to the World Health Organization's definition, tumors ≤1 cm in diameter were classified as osteoid osteoma, and those ≥2 cm, as osteoblastoma. For tumors between 1 cm and 2 cm, other criteria, such as the bone involved, the site, the presence of a nidus, and presence of peripheral sclerosis, were used for diagnosis. There were 131 patients with osteoid osteoma (93 male, 38 female) and 73 patients with osteoblastoma (40 male, 33 female). The mean age in the osteoid osteoma and osteoblastoma groups was 16.4 ± 7 and 19.6 ± 9.9 years, respectively. The osteoid osteoma cases were mostly localized in the extremities, whereas the osteoblastoma cases involved the vertebral column and sacrum. The nidus size varied between 0.2 and 1.5 cm in osteoid osteoma cases, and the tumor size range was 1.3-10 cm in the osteoblastoma cases. The pain was encountered in 89% of osteoid osteoma and 45% of osteoblastoma patients. Histopathology was similar in both cases. The treatment of choice was conservative surgery for both diagnoses. In conclusion, osteoblastoma is clinically and radiologically more aggressive than osteoid osteoma.

Effects of hyaluronic acid and chondroitin sulfate on acute radiation proctitis in rats.

BACKGROUND: This study was designed to investigate the prophylactic and therapeutic effects of hyaluronic acid and chondroitin sulfate combination (HA/CS) on a model of acute radiation proctitis. METHODS: Rats were divided into five groups: SHAM; irradiation (IR) + saline (1 mL for 5th and 10th day); IR + HA/CS (1 mL for 5th and 10th day). A single fraction of 17.5 Gy was delivered to each rat. HA/CS was administered rectally each day after irradiation. Each rat was observed daily for signs of proctitis. Irradiated rats were euthanized on days 5 and 10. The mucosal changes were evaluated macroscopically and pathologically. RESULTS: According to the clinical findings, five rats in the irradiation + saline group showed grade 3-4 symptoms on the 10th day. No significant difference in the macroscopic finding scores on the 5th day was observed between the irradiation + saline and irradiation + HA/CS groups. In the pathological examination, radiation-induced mucosal damage was the most prominent finding 10 days after irradiation in saline-treated rats. On the 10th day, the irradiation + HA/CS group showed mild inflammation and slight crypt changes, which corresponded to grade 1-2 pathological findings. CONCLUSION: We think that HA/CS used in radiation cystitis can be beneficial for radiation proctitis.

Alveolar distraction osteogenesis in the human mandible: a clinical and histomorphometric study.

PURPOSE: To identify the quality of newly formed bone in the distraction region and to determine the percentage of mineralized bone formed in the distraction area. METHODS: Ten patients with vertically deficient mandibular alveolar ridges were treated by means of distraction osteogenesis. Four months after consolidation of distracted segments, a total of 40 dental implants were inserted in native bone and distracted bone. Bone biopsies were taken at the implant sites with trephine burrs for histological and histometric analyses. Four months after implant placement, abutments were connected, and prosthetic loading of the implants was started. RESULTS: The mean bone gain at the end of distraction was 7.2 ± 0.8 mm. The cumulative success rate of implants 3 years after the onset of prosthetic loading was 100%. The newly formed bone consisted of woven bone reinforced by parallel-fibered bone with bone marrow spaces and the percentage of mineralized bone ranged from 50.56% to 76.88%. CONCLUSIONS: Alveolar distraction osteogenesis is able to produce adequate mature bone for the correction of mandibular bone deficits before dental implant insertion.

Does bile reflux reduce Helicobacter pylori gastritis?

BACKGROUND: Chronic abdominal pain is a frequent childhood complaint. This study aims to determine the relationship between bile reflux, which is increasing with the growth in packaged food consumption resulting from the changing food industry, and Helicobacter pylori gastritis. METHODS: In this retrospective study, 804 cases where there was an endoscopic examination for abdominal pain were included. We recorded the patients` age, sex, and macroscopic and microscopic endoscopic findings. Patients with chronic diseases were excluded. RESULTS: Our study included 804 cases. Of patients, 61.8% were female and 38.2% were male. The mean age was 11.56±4.14 years. The Helicobacter pylori gastritis rate was found to be 22.3% among all patients. Bile reflux was seen in 192 (23.9%) patients. Only 27 (14.1%) of the 192 patients had Helicobacter pylori positivity (p=0.002). CONCLUSIONS: Helicobacter pylori gastritis is less common among patients with bile reflux. In another study conducted in our outpatient clinic before the 2000s, the frequency of Helicobacter pylori gastritis was found to be 40%, but after 2000 this rate decreased to 22.3% due to bile reflux caused by the changing food industry. This result may be explained by the bactericidal effects of bile acids.

The effectiveness of cetuximab and panitumumab when combined with FOLFIRI in second-line treatment of KRAS wild type metastatic colorectal cancers. Single centre experience.

Panitumumab and cetuximab are monoclonal antibodies known to be effective in metastatic colorectal cancer (mCRC). Although the survival benefits when combined with chemotherapy have been determined, there are no studies comparing the two agents with chemotherapy in the second-line treatment. In this study, we aimed to compare the efficacy of cetuximab vs panitumumab in patients who previously received chemotherapy. Who progressed after first-line treatment for K-ras wild type mCRC were analyzed. The efficacy of cetuximab vs panitumumab on overall survival (OS) and progression-free survival (PFS) when combined with FOLFIRI regimen was compared retrospectively. Median PFS was 6.9 months in the cetuximab group and 4.7 months in the panitumumab group. Median OS cetuximab and panitumumab groups were 18.4 and 12.2 months, respectively. In the second-line treatment of K-ras wild type mCRC, both PFS and OS were found to be longer in patients receiving cetuximab than in patients receiving panitumumab, but no statistically significant difference was found.

A rare cause of refractory ascites in a child: familial Mediterranean fever.

Familial Mediterranean fever (FMF) is hereditary episodic febrile syndrome characterized by acute attacks of fever and serosal inflammation, generally lasting 1-3 days and resolves spontaneously. Apart from abdominal pain, patients may present with variety of abdominal manifestations such as acute peritonitis, mechanical intestinal obstruction, diarrhea, bowel infarction, amyloidosis and small amounts of peritoneal fluid during the acute attacks. A 6-year-old boy was admitted with massive ascites. After extensive laboratory investigations, no causative agent could be identified. On subsequent days, he developed fever and skin eruptions. Acute-phase reactants were increased. A second tomography revealed cystic fluid collection near the anterior side of spleen that invades the stomach. An exploratory was performed and histopathological examination of the all resected specimens revealed mix inflammatory cell infiltrate associated with severe myofibroblast proliferation suggesting chronic inflammatory process on the mesenteric region. A diagnosis of FMF was suspected based on the clinical, laboratory and histopathological findings, and a trial of colchicine therapy initiated. Ascites and other serosal inflammations improved within 1 week without any recurrence during the next 12-month period on colchicine treatment. Atypical presentations of FMF have been increasingly reported. Pediatricians should keep FMF in mind in the differential diagnosis of massive ascites especially in regions where hereditary inflammatory disease are common.

Anti-Inflammatory Effect of Crude Momordica charantia L. Extract on 2,4,6-Trinitrobenzene Sulfonic Acid-Induced Colitis Model in Rat and the Bioaccessibility of its Carotenoid Content.

Momordica charantia L., known as bitter melon (BM), is a plant that belongs to the family Cucurbitaceae. Aims of this study are to investigate the anti-inflammatory effect of crude BM extract on 2,4,6-trinitrobenzene sulfonic acid (TNBS)-induced experimental colitis model in rat. It was also aimed to determine the content and bioaccessibility of carotenoids of BM. BM was purchased from local markets in Izmir, Turkey. Fruits of BM were lyophilized, powdered, and used in the experiment. Carotenoids were determined by high-performance liquid chromatography. To determine the bioaccessibility of ß-carotene, in vitro digestion was performed. Wistar albino rats were divided into four groups: group A (BM+TNBS), group B (BM), group C (TNBS), and group D (control). BM solution was given 300 mg/(kg·day) for 6 weeks orally. Colitis was induced by 0.25 mL of a solution containing 100 mg/kg 5% (w/v) TNBS in 50% ethanol (w/v) intrarectally after 6 weeks. After sacrification, macroscopic and microscopic evaluations were performed. Myeloperoxidase, cytokines levels (interleukin-17 [IL-17], TNF-alpha, and interleukin-10 [IL-10]) were measured in serum and colonic samples by ELISA test. Institutional Animal Ethics Committee approval was obtained. Total carotenoid content of BM was determined 11.7 mg/g dry weight as ß-carotene equivalents. Bioaccessibility of total carotenoids was determined as 2.1% with in vitro digestion. Pretreatment with crude BM extract significantly reduced weight loss, macroscopic, and microscopic colitis damages in colonic samples (P = .000), (P = .015), and (P = .026), respectively. Serum anti-inflammatory cytokine IL-10 increased significantly in both treatment groups (P = .000). BM is a rich source of carotenoids, but the bioaccessibility of its carotenoids is low. This study displays that BM has protective anti-inflammatory effects on TNBS-induced colitis.

Can N-acetylcysteine preserve peritoneal function and morphology in encapsulating peritoneal sclerosis?

Long-term use of the peritoneum as a dialysis membrane results in progressive irreversible dysfunction, described as peritoneal fibrosis. Oxidative stress during peritoneal dialysis has been established in many studies. Generation of reactive oxygen species (ROS) by conventional peritoneal dialysis solutions, regardless of whether produced by high glucose, angiotensin II, or glucose degradation products may be responsible for progressive membrane dysfunction. The well-known antioxidant molecule N-acetylcysteine (NAC) is capable of direct scavenging of ROS. The aim of the present study was to investigate the effect of NAC therapy on both progression and regression of encapsulating peritoneal sclerosis (EPS). We divided 49 nonuremic Wistar albino rats into four groups: Control group-2 mL isotonic saline intraperitoneally (IP) daily for 3 weeks; CG group-2 mL/200 g 0.1% chlorhexidine gluconate (CG) and 15% ethanol dissolved in saline injected IP daily for a total of 3 weeks; Resting group-CG (weeks 1 - 3), plus peritoneal resting (weeks 4 - 6); NAC-R group-CG (weeks 1 - 3), plus 2 g/L NAC (weeks 4 - 6). At the end of the experiment, all rats underwent a 1-hour peritoneal equilibration test with 25 mL 3.86% PD solution. Dialysate-to-plasma ratio (D/P) urea, dialysate white blood cell count (per cubic milliliter), ultrafiltration (UF) volume, and morphology changes of parietal peritoneum were examined. The CG group progressed to encapsulating peritoneal sclerosis, characterized by loss of UF, increased peritoneal thickness, inflammation, and ultimately, development of fibrosis. Resting produced advantages only in dialysate cell count; with regard to vascularity and dialysate cell count, NAC was more effective than was peritoneal rest. Interestingly, we observed no beneficial effects of NAC on fibrosis. That finding may be a result of our experimental severe peritoneal injury model. However, decreased inflammation and vascularity with NAC therapy were promising results in regard to membrane protection.

Medulloblastoma: clinicopathologic evaluation of 42 pediatric cases.

PURPOSE: The aim of this study was to assess the prognostic value of MIB-1 and p53 in the pediatric medulloblastoma group. MATERIALS AND METHOD: Forty-two pediatric medulloblastoma cases diagnosed in a single institution during the past 10 years were evaluated. Follow-up data were available for 35 patients. RESULTS: The immunoreactivity of MIB-1 ranged from 10% to 95%; p53 immunoreactivity was found in five cases. Of the 35 patients with follow-up, 34 patients received a combination of chemotherapy and radiotherapy, while one received chemotherapy alone. The follow-up period ranged from 5 to 64 months. Of the 35 patients, 21 were alive without any evidence of recurrent disease, three were alive with evidence of recurrent disease and 11 died of disease during follow-up. The mean survival for these 11 patients was 21.9+/-10.4 months. Of the 35 cases, 16 had MIB-1 value of 25% or lower and 19 had a value of 26% or more. Of the 16 cases with low MIB-1 value, six died of disease; of the 19 cases with high MIB-1 value, five died of disease. The statistical difference between MIB-1 and prognosis was not significant. Three of the 35 (8.5%) cases were found to be positive with p53 protein; no correlation was observed between p53 immunoreactivity and prognosis. CONCLUSION: It appears that the MIB-1 value and p53 immunoreactivity have no relation with prognosis in pediatric medulloblastomas. However, it is convenient to support these findings with large series.

Atypical teratoid/rhabdoid tumor of the central nervous system: clinicopathologic and immunohistochemical features of four cases.

BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare aggressive infantile neoplasm of uncertain origin. This study was performed to assess the clinicopathologic and immunohistochemical features of four AT/RT cases. CASE REPORTS: Two cases were male and two were female, and their ages ranged from 8 to 103 months. Tumors were located in the cerebellum (two cases), frontoparietal lobe (one case), and third ventricle (one case). Histopathologically, the tumors were composed of rhabdoid cells and undifferentiated small cells mixed with epithelial or mesenchymal components. However, one of the tumors was composed predominantly of a mesenchymal component mimicking a sarcoma. Immunohistochemically, vimentin (4/4), epithelial membrane antigen (4/4), cytokeratin (3/4), smooth muscle actin (4/4), glial fibrillary acidic protein (4/4), S-100 (4/4), and synaptophysin (1/4) were positive in varying proportions, while desmin and INI-1 were negative in all the cases. All of the patients died within a mean of 14 months due to tumor progression despite the chemotherapy. Only one of our patients lived for 40 months after the diagnosis. In conclusion, AT/RTs are aggressive tumors. They can occur in a variety of locations, such as the third ventricle. Morphologically, a large spectrum can be seen, like predominantly sarcoma in appearance, but immunohistochemistry is helpful in the correct diagnosis.

The effects of early or late treatment of osteochondral defects on joint homoeostasis: an experimental study in rabbits.

A 3.5 x 4 mm tubular osteochondral defect was created on the right medial femoral condyles of 51 adult rabbits. In the control group (CG), defects were left untreated. In the early-(ETG) and late-(LTG) treatment groups, defects were treated by an osteoperiosteal graft 1 and 12 weeks, respectively, after the index procedure. Synovial fluid (SF) samples were collected regularly and proteoglycan fragments (PF), total collagen (TC) and collagenase (MMP-1) levels were measured. Rabbits were killed at 4 (early period), 12 (intermediate period), or 24 (late period) weeks postoperatively. Histological examination indicated a more successful healing in both grafting groups than in the CG, but without any difference at any time period between the grafting groups. In the CG, PF, and TC levels in SF increased continuously until the late period, indicating an ongoing degenerative activity in the joints. In contrast, SF marker levels in both grafting groups indicated that normalization in joint metabolism could be achieved-at least partially-after treatment. However, PF levels in the SF showed that the treatment of defects in earlier stages might result in better outcomes since the negative effects were more prominent in chronic stages, presumably due to the more prolonged period of disturbed homeostasis. Thus, histological values and SF marker levels indicated that treatment of osteochondral defects at any time of the disease had a positive effect on healing when compared to no treatment. Early treatment might better assist the recovery of joint homeostasis than late treatment.

Secondary osteosarcoma arising in fibrous dysplasia, case report.

Malignant transformation of fibrous dysplasia is very rare. The frequency is increased in polyostotic forms, in McCune-Albright and Mazabraud's syndromes and previously irradiated cases. Pain, which is rapidly becoming worse over a short period unrelated to trauma is the most alarming symptom. Early radiological features of sarcomatous transformation are moth-eaten or cystic areas of osteolysis, cortical destruction and gradual formation of a soft tissue mass. The prognosis is unfavorable as most of the cases are in an advanced stage in the time of diagnosis. We present an unusual case of unsuspected secondary osteosarcoma arising in a previously unirradiated, monostotic fibrous dysplasia. A 46-year-old woman was admitted with hip pain, which worsened after a minor trauma occurred 1 year ago. Plain graphies of left femur showed a well-delineated lesion with endosteal scalloping and areas having a ground-glass appearance. The MRI revealed minimal contrast enhancement but no heterogenous signal intensity, cortical destruction, periost reaction or accompanying soft tissue component was noted. The lesion was initially curetted. But being diagnosed as osteosarcoma histologically, classical osteosarcoma protocol pre and postoperative chemotherapy was applied. Resected femur showed areas of fibrous dysplasia admixed with osteosarcoma having fibroblastic, chondroblastic and osteoblastic areas that were focally invading the soft tissue. Tumor viability was estimated as 95%. The clinical course worsened rapidly after the operation. She did not respond to postoperative chemotherapy and lost with pulmonary metastases less than a years' time after the operation. The case is presented to increase awareness on the possibility of malignant transformation in an otherwise unsuspected fibrous dysplasia.

Dedifferentiated chondrosarcoma of the thumb: a case report.

Dedifferentiated chondrosarcoma (DDCS) comprises approximately 10% of all chondrosarcomas and has the worst outcome with 5-year survival of 10%. The preferred localizations are femur, humerus and pelvis. We report a case of DDCS that arose in proximal phalanx of left-hand thumb of a 66-year-old man. He was admitted with a 2-month history of pain and swelling in his thumb. He had experienced a fracture 15 years ago in the same localization with a history of suspected preexisting enchondroma. Plain radiographs showed an expansive osteolytic lesion with milimetric calcifications while magnetic resonance imaging revealed intraosseous focal globular hyperintense spots consistent with chondroid areas as well as a surrounding soft tissue mass with intermediate signal intensity. In biopsy specimen we observed a low-grade chondrosarcoma next to a spindle cell sarcoma with an abrupt transition. He was treated with amputation and died of the disease within 9 months following the surgery. The presence of dedifferented chondrosarcoma in bones of hands was not previously reported. The presented case was provided with the macroscopic and microscopic features observed in amputation specimen in comparison with radiological findings.

Periosteal osteoblastoma of the distal femur.

Periosteal osteoblastoma is a rare benign osteoblastic bone tumour located on the cortical bone. We report a case of periosteal osteoblastoma located at the distal femur in a 31-year-old man. Clinical, radiological and histopathologic findings are described and differential diagnosis is discussed. Despite its rarity, periosteal osteoblastoma should be considered as a possible diagnosis together with periostitis ossificans, periosteal chondroma, periosteal osteosarcoma and parosteal osteosarcoma when confronted with a superficial bone lesion.

Gastrointestinal findings in 26 adults with common variable immunodeficiency: The fickle nature of the disease manifests in gastrointestinal biopsies.

BACKGROUND/AIMS: The aim of the present study was to demonstrate the histopathological findings in gastrointestinal (GI) biopsies in adults with common variable immunodeficiency (CVID). MATERIALS AND METHODS: A total of 172 GI biopsies of 26 patients with CVID obtained over a 16-year period were reevaluated. Findings were analyzed using descriptive analyzes and χ2 test. RESULTS: Female-to-male ratio was 1.36. The median age at diagnosis was 36±13.94 (16-72) years. Chronic esophagitis was noted in 3 patients. The absence of plasma cells in the stomach, duodenum, and colon was observed in 16, 14, and 9 patients, respectively. Divergent results for the presence of plasma cells in concurrent stomach and duodenum samples were found in 11 (44%) patients. Nodular lymphoid hyperplasia (NLH) was notable in the duodenum (56%). The mean number of eosinophils in one high-power field was significantly higher in duodenal biopsies with NLH (27.21 vs. 14.37, p=0.002). Active inflammation was more prominent in the colon (91%) than in the stomach (65%) and duodenum (60%). Helicobacter pylori infection was found in 57.6%, including a case with persistent infection by the coccoid form. Celiac-like villous blunting and increased intraepithelial lymphocytes were seen in 40% and 24%, respectively. In addition, 23% had giardiasis associated with acute duodenitis and duodenal NLH (p<0.05). CONCLUSION: CVID gastroenteropathy is a challenging entity, and due to the heterogeneity in the presence and distribution of plasma cells throughout the GI tract and diverse disease course, multiple concurrent biopsies may be needed for tissue diagnosis. Duodenal CVID may present with villous alterations and giardiasis, and NLH appears to be an important clue in the duodenum. The association between duodenal NLH and eosinophil infiltration deserves further investigation.

Clinical experiences in Turkish paediatric patients with chronic recurrent multifocal osteomyelitis.

Sözeri B, Aktay Ayaz N, Yildiz Atikan B, Karadag SG, Çakan M, Argin M, Sezak M. Clinical experiences in Turkish paediatric patients with chronic recurrent multifocal osteomyelitis. Turk J Pediatr 2019; 61: 879-884. Chronic recurrent multifocal osteomyelitis (CRMO) is a clinical entity which occurs mainly in children and adolescents with recurrent episodes of pain occurring over several years. Cause and physiopathology of disease is still uncertain. We aim to assess clinical characteristics and treatment options, need and response to anti-inflammatory therapies in children diagnosed chronic recurrent multifocal osteomyelitis Demographic data and clinical features of seventeen children diagnosed with CRMO in 2 pediatric rheumatology centers in Turkey were reviewed retrospectively. The diagnosis was based on clinical findings, radiological images and histopathological and microbiological studies. A total of 17 patients were included in the study. The median age of diagnosis was 9.6±4.2 years. The mean follow-up time was 31.6 months (range 6-35 months). Most patients (n: 10) had a recurrent multifocal disease course ( > 6 months), 6 patients had a persistent course and a patient had only one episode of CRMO. MEFV gene mutations were detected in 4 patients whose clinical features reduced with colchicine therapy. All patients had received nonsteroidal anti-inflammatory drugs but only one had complete response. Thirteen children with NSAID failure subsequently received corticosteroids, sulfasalazine, methotrexate, Anti TNF α drugs, or a combination of these drugs. This study is the largest cohort of pediatric CRMO patients in our country. Clinical evolution and imaging investigations should be closely done to avoid delays in diagnosis. Ethnic differences create changes in the presentation of the disease and response to treatment.