Pattern of primary tumors and tumor-like lesions of bone in children: retrospective survey of biopsy results

Background: Although primary bone tumors are relatively uncommon, they constitute the most important tumors in patients less than 20 years. We aimed to determine the frequencies of primary bone tumors and tumor-like lesions of bone and the anatomical sites of their occurrence. Methods: A retrospective review of histopathology reports of all bone specimens received in a private pathology laboratory in Istanbul between 2009 and 2015. Results: A total of 57 patients (aged 5 to 18 years) with a mean of 13.12 years were studied. Thirty five patients (61.4%) were males and 22 (38.6%) were females. Fifty five (94.4%) of the tumors were benign. Osteochondroma was the commonest tumor accounting for 31 cases (54.3%) followed by osteoid osteoma, 9 cases (15.7%). Chondrosarcoma observed in two patients and Ewing sarcoma in one patient as malignant tumors. Of the 57 bone tumors 13 (22.8%) occurred in the upper extremities, while 44 (77.2%) were in the lower extremities. Proximal humerus was the most commonly involved site in upper extremity tumors, with osteochondromas representing the most frequent type of tumor (4 patients; 7%). In the lower extremities again osteochondromas were the most common type of tumor (8 cases, 14%), with the femur being the most common site of involvement (18 patients, 31.5%). Of the patients with tumor-like lesions; four patients had fibrous dysplasia, 4 patients had non-ossified fibromas, 4 patients had simple bone cysts and 3 had aneurismal bone cyst. Conclusion: This study showed that primary bone tumors were mainly benign, settled predominantly in the lower extremities mostly in the femur with a male preponderance. Osteochondroma was the most common benign bone tumor. We didn’t observed osteosarcoma, which is the most frequent malignant bone tumor.     

Clinical outcomes and mortality in elderly peritoneal dialysis patients

OBJECTIVES:

To evaluate the clinical outcomes and identify the predictors of mortality in elderly patients undergoing peritoneal dialysis.

METHODS:

We conducted a retrospective study including all incident peritoneal dialysis cases in patients ≥65 years of age treated from 2001 to 2014. Demographic and clinical data on the initiation of peritoneal dialysis and the clinical events during the study period were collected. Infectious complications were recorded. Overall and technique survival rates were analyzed.

RESULTS:

Fifty-eight patients who began peritoneal dialysis during the study period were considered for analysis, and 50 of these patients were included in the final analysis. Peritoneal dialysis exchanges were performed by another person for 65% of the patients, whereas 79.9% of patients preferred to perform the peritoneal dialysis themselves. Peritonitis and catheter exit site/tunnel infection incidences were 20.4±16.3 and 24.6±17.4 patient-months, respectively. During the follow-up period, 40 patients were withdrawn from peritoneal dialysis. Causes of death included peritonitis and/or sepsis (50%) and cardiovascular events (30%). The mean patient survival time was 38.9±4.3 months, and the survival rates were 78.8%, 66.8%, 50.9% and 19.5% at 1, 2, 3 and 4 years after peritoneal dialysis initiation, respectively. Advanced age, the presence of additional diseases, increased episodes of peritonitis, the use of continuous ambulatory peritoneal dialysis, and low albumin levels and daily urine volumes (<100 ml) at the initiation of peritoneal dialysis were predictors of mortality. The mean technique survival duration was 61.7±5.2 months. The technique survival rates were 97.9%, 90.6%, 81.5% and 71% at 1, 2, 3 and 4 years, respectively. None of the factors analyzed were predictors of technique survival.

CONCLUSIONS:

Mortality was higher in elderly patients. Factors affecting mortality in elderly patients included advanced age, the presence of comorbid diseases, increased episodes of peritonitis, use of continuous ambulatory peritoneal dialysis, and low albumin levels and daily urine volumes (<100 ml) at the initiation of peritoneal dialysis.     

Autoimmune Polyglandular Syndrome Type 3c with Ectodermal Dysplasia,Immune Deficiency and Hemolytic-Uremic Syndrome

Autoimmune polyglandular syndrome (APS) is a disorder which is associated with multiple endocrine gland insufficiency and also with non-endocrine manifestations. The pathophysiology of APS is poorly understood, but the hallmark evidence of APS is development of autoantibodies against multiple endocrine and non-endocrine organs. These autoantibodies are responsible for the dysfunction of the affected organs and sometimes may also cause non-endocrine organ dysfunction. The hemolytic-uremic syndrome (HUS) is a serious and life-threatening disease which develops due to many etiological factors including autoimmune disorders. Here, we present an unusual case of APS. Ectodermal dysplasia with immune deficiency and HUS occurred concomitantly in the same patient with APS type 3c. Once the autoantibody generation was initiated in the human body, development of multiple disorders due to organ dysfunction and also autoantibody-related diseases may have occurred.     

Prepubertal Unilateral Gynecomastia: Report of 2 Cases

Prepubertal unilateral gynecomastia is an extremely rare condition. At present, its etiology and management strategy are not well known. Two unrelated prepubertal boys of ages 8 and 9 who presented with complaints of unilateral enlargement of breast tissue are reported. Physical examination, biochemical, hormonal and oncologic work-up findings were normal. Both patients were treated with peripheral liposuction successfully. Histopathological and immunohistochemical examinations showed benign fibroglandular gynecomastia and intensive (3+) estrogen receptor expression in 100% of periductal epithelial cells. Although an extremely rare and generally benign condition, patients with prepubertal unilateral gynecomastia should have a full endocrine and oncologic work-up.     

Navigational area of the cranio-orbital foramen and its significance in orbital surgery

The cranio-orbital foramen (COF) is located on the lateral wall of the orbit. It is a potential source of hemorrhage during deep lateral orbital dissection, since it functions as an anastomosis between the lacrimal artery and the middle meningeal artery. The aim of this study was to guide and facilitate the surgical procedures in the orbit, so as to determine a navigational area and the precise location of the COF and to standardize certain anatomical marks. The navigational area of the COF and topographical features were studied in 75 craniums with presented COF. 33 bilateral main COFs, 41 (18 on the right, 23 on the left) unilateral main COFs at the main cranium and 19 accessory COFs were studied for their navigational features on the orbit. The distances between the COF and the fronto-zygomatic suture, supraorbital notch, lateral angle of the superior orbital fissure (SOF) and Whitnall’s tubercle were measured. The mean distance of the COF from the fronto-zygomatic suture, supraorbital notch, lateral angle of the SOF and Whitnall’s tubercle was 26.3, 37.3, 92 and 27.1 mm, respectively. For the navigational area signs of the COF, areas of the orbit that form the transversal and vertical lines are generated on the reference points. Whilst the upper outer area of the orbit contains a potential bleeding risk, the bottom section of the outer column is identified as safe for the surgical operations of the lateral orbital wall. The fronto-zygomatic suture and Whitnall’s tubercle are recommended as the most reliable navigational landmarks for identifying the COF. Hence, the transversal and vertical orientation of the COF should be mastered by the surgeons reconstructing the anterior base of the skull and the orbit.     

Association of Thrombin Generation Potential with Platelet PAR-1 Regulation and P-Selectin Expression in Patients on Dual Antiplatelet Therapy

We studied the association of thrombin generation potential with platelet protease activated receptor (PAR)-1 regulation and platelet activation in 52 stable coronary artery disease patients on continuous therapy with aspirin and clopidogrel (n?=?42) or prasugrel (n?=?10). Compared to controls, peak thrombin generation potential was elevated in only 11 patients (p?>?0.05), while F1.2 was elevated in 26 patients (p?<?0.0001). PAR-1 and thrombin inducible P-selectin expression were significantly elevated in patients compared to controls (p?<?0.001). There were no significant correlations between levels of thrombin generation potential or F1.2 and PAR-1 regulation. However, there was a significant inverse correlation between levels of peak thrombin generation potential and in vitro thrombin-inducible expression of P-selectin (p?=?0.002), suggesting in vivo depletion of platelet alpha granules due to ongoing platelet activation.     

Mean Platelet Volume in Patients With Dilated Cardiomyopathy: Does it have a Role in Left Ventricular Thrombus Formation?

We assessed the clinical echocardiograhic, hematological, and biochemical parameters in patients with dilated cardiomyopathy (DCMP) and control individuals mainly focusing on the mean platelet volume (MPV) in terms of DCMP and left ventricle (LV) thrombus formation. Consecutive patients (n = 251) with DCMP and 266 patients without DCMP were studied. Mean platelet volume was significantly greater in patients with DCMP than in control patients (P < .05 for all comparisons). Comparing DCMP patients with LV thrombus (19 patients, 8%) and without LV thrombus (232 patients, 92%) showed that the prevalence of smokers was significantly higher and ejection fraction was significantly lower in patients with LV thrombus. We have shown that patients with DCMP have significantly higher MPV suggesting more platelet activation and the MPV of patients with DCMP and LV thrombus is comparable to those of patients without LV thrombus.