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Background: Fibroblast growth factor 23 (FGF-23) and α-Klotho protein appear to have an important role in the pathogenesis of CKD-mineral and bone disorders. The aim of this study was to investigate the association of FGF-23 and α-Klotho levels with adverse clinical outcomes in patients with non-dialysis CKD. Materials and Methods: We conducted a prospective cohort study, enrolling participants with non-dialysis CKD from a single center in Greece. At enrollment, glomerular filtration rate (GFR) was measured (mGFR) and plasma levels of carboxyl terminal FGF-23 (cFGF-23) and soluble α-Klotho (sKlotho) were determined by enzyme-linked immunoassay. Participants were followed for up to 5 years or until the occurrence of the primary endpoint of initiation of renal replacement therapy or death. Multivariate regression tree analysis was used to identify informative baseline parameters in order to categorize participants. Also, using median values of cFGF-23 and sKlotho, participants were categorized into 4 groups, in whom survival was compared using Kaplan-Meier and Cox regression analysis. Results: 128 participants were enrolled with a median mGFR of 41.5 mL/min/1.73 m 2 (IQR = 28.2). Baseline mGFR correlated with cFGF-23 and sKlotho (r = −0.54 and r = 0.49, respectively; p < 0.0001 for both). cFGF-23 and sKlotho levels correlated negatively (r = −0.24, p = 0.006). Multivariate regression tree analysis resulted in 3 groups defined by cutoff values of mGFR (60.9 mL/min/1.73 m 2 ) and phosphate (3.7 mg/dL). These groups correlated with CKD stage, cFGF-23, and sKlotho (p < 0.0001 for all). During a median follow-up of 36 months (IQR = 22), 40 (31.2%) participants reached the primary endpoint (31 initiated renal replacement therapy, 9 died). Survival to primary endpoint differed among the 4 groups formed using median values of both biomarkers, with the low FGF-23/high Klotho and high FGF-23/low Klotho having the longest and shortest survival, respectively. High FGF-23/low Klotho group, compared to the opposite one, had a significantly elevated risk of the primary outcome (HR, 6.8; 95% CI, 2.3–19.6; p = 0.0004). Conclusions: In patients with CKD stages 1–5, the combination of higher cFGF-23 and lower sKlotho levels along with mGFR and serum phosphate was associated with adverse clinical outcomes. The utility of combinations of traditional and novel biomarkers to predict outcomes warrants further study.

Background: Erdheim-Chester disease (ECD) is a rare hematopoietic neoplasm of histiocytic origin characterized by an insidious course. The coronavirus disease 2019 (COVID-19) pandemic has put an enormous strain on healthcare systems worldwide both directly and indirectly, resulting in the disruption of healthcare services to prevent, diagnose and manage non-COVID-19 disease. Case Presentation: We describe the case of a 58-year-old male patient with sporadic episodes of self-resolving mild fever and anemia of chronic disease with onset two years before the current presentation. Positron emission/computed tomography scan revealed the presence of moderately hypermetabolic perirenal tissue masses. In order to achieve diagnosis, repeated perirenal tissue biopsies were performed, and the diagnostic evaluation was complicated by the strain put on the healthcare system by the COVID-19 pandemic. The patient contracted SARS-CoV-2 and required hospitalization, but recovered fully. No further ECD target organ involvement was documented. Treatment options were presented, but the patient chose to defer treatment for ECD. Conclusion: A high index of suspicion and multidisciplinary team collaboration is paramount to achieve diagnosis in rare conditions such as ECD. Disruptions in healthcare services in the pandemic milieu may disproportionately affect people with rare diseases and further study and effort is required to better meet their needs in the pandemic setting.

Occlusive vasculopathy due to the development and accumulation of granulomas at the level of intima of large vessels, as well as mediastinal lymph nodes and fibrosing mediastinitis secondary to sarcoidosis, causing extrinsic compression of mediastinal vascular structure are uncommon mechanisms of sarcoidosis-associated pulmonary hypertension. We present a case of a 62-year-old woman with a rare manifestation of sarcoidosis, which was misclassified and treated as chronic thromboembolic pulmonary hypertension for a long period. Fluorine-18-fluorodeoxyglucose positron emission tomography played a major role in accessing final diagnosis. Mechanisms that lead to development of pulmonary hypertension, the contribution of novel imaging modalities, and treatment options are discussed.

This study aimed to determine the ultrasonographic prevalence of Baker’s cysts in knees with chronic osteoarthritic pain and investigate for cysts correlates and relationships with scintigraphically established synovitis. Consecutive patients with chronic osteoarthritic knee pain underwent clinical examination, X-rays, ultrasonography and early-phase bone scintigraphy. Eighty-nine Baker’s cysts were detected in 328 knees with chronic osteoarthritic pain (27%), whereas one cyst was identified among the 54 non-osteoarthritic knees (2%, P  < 0.001). Baker’s cysts were detected in 72/195 (37%) patients with knee osteoarthritis. Abnormal and intense tracer accumulation in early-phase bone scintigraphy were significantly more frequent in osteoarthritic knees with Baker’s cysts (97 and 56%, respectively), than in those without (89 and 40%, respectively, P  < 0.05 for both). Clinical and radiographic variables could not predict the presence of those cysts. Baker’s cysts are a common ultrasonographic finding in knees with chronic osteoarthritic pain and are associated with synovial inflammation and its grade.

Juvenile Paget's disease (JPD) is a rare, autosomal recessive osteopathy. Although it has phenotypic overlap with Paget's disease of bone (PDB), it is probably a distinct entity. Because of its rarity, optimal disease management has not yet been established by randomized controlled trials. However, clinical, biochemical, and radiographic improvement has been reported after treatment with antiresorptive agents, including calcitonin and bisphosphonates (BPs). Compared with other BPs, zoledronic acid (ZOL) has a higher affinity to bone mineral and is a stronger inhibitor of the enzyme farnesyl pyrophosphate synthase (the main target of nitrogen-containing BPs), properties that explain the prolonged effect of ZOL on bone turnover and render it a therapeutic option for JPD, similar to PDB. We describe hereby, for the first time in the literature, the case of a patient with JPD who developed severe hypocalcemia and secondary hyperparathyroidism following effective treatment with ZOL.

Antioxidant supplementation has been suggested to prevent exercise-induced muscle injury, but the findings are inconsistent. The objective of this study was to investigate the potential protective role of vitamin E treatment against eccentric exercise-induced muscle injury by examining morphological and functional alterations in rat soleus muscle after downhill running as well as muscle injury markers in the blood. Sixty adult male Wistar rats were randomly assigned to vitamin E-treated or placebo-treated groups studied at rest, immediately post-exercise or 48 h post-exercise ( n  = 10 per group). Vitamin E was administered by daily intraperitoneal injections of 100 mg/kg body mass of dl -α-tocopheryl acetate for five consecutive days prior to exercise, resulting in the doubling of its plasma concentration. Downhill running resulted in significant ( P  < 0.05) changes in all injury markers for the placebo-treated rats at 0 and 48 h post-exercise. However, significantly smaller soleus muscle single-twitch tension ( P t ) and unfused (40 Hz) tetanic force, and greater plasma creatine kinase (CK) and lactate dehydrogenase (LD) activities compared with the control were found only immediately post-exercise for the vitamin E-treated rats ( P  < 0.05). Maximal tetanic force ( P o ) did not decline significantly compared to sedentary controls at neither time points measured. The vitamin E-treated rats had significantly ( P  < 0.05) higher soleus muscle P t immediately post-exercise than the placebo-treated rats as well as lower plasma CK and LD activity 48 h post-exercise. However, there was no difference in P o decline between groups at either time points measured. Vitamin E-treated rats had less pronounced morphological alterations in muscle in the immediate and 48-h post-exercise period. In conclusion, the effect of short-term vitamin E supplementation against eccentric exercise-induced muscle injury did not appear to be physiologically significant, because vitamin E failed to prevent the decline in the functional measure of P o compared to the placebo conditions.

Bisphosphonates have long been used with success in the treatment of Paget’s disease of bone (PDB). The aim of this study was to evaluate the early (up to 3 months) and late (at 12 months) scintigraphic, biochemical, and clinical response to a single intravenous infusion of zoledronic acid (ZOL) in patients with PDB serially assessed for 1 year. Nine patients with 30 bone lesions caused by PDB were prospectively evaluated. Total serum alkaline phosphatase (SAP) was serially measured. Scintigraphy was performed before and at 3 and 12 months after ZOL administration, and bone lesions were assessed quantitatively. After treatment, pain was alleviated in five of six patients starting from the first month. At 3 months, a significant decrease of SAP levels compared to baseline values was found (322 ± 211 IU/l before vs. 101 ± 36 IU/l 3 months after; P < 0.05), with normal values attained in all except one patient. The scintigraphic index of involvement (SII), a marker for the perpatient activity of the disease, was reduced from 14.4 ± 7.6 to 7.2 ± 1.8 ( P = 0.01). The scintigraphic ratio (SR), a marker for the per-lesion activity of the disease, was reduced from 12.8 ± 7.7 to 7.0 ± 2.9 ( P < 0.001). The values of markers of disease activity remained unchanged up to 12 months. A single intravenous administration of ZOL leads to a favorable clinical, biochemical, and scintigraphic response in patients with PDB starting as early as 3 months after treatment and lasting no less than 12 months (i.e., considerably longer than the other existing therapies).

Microsurgical techniques are increasingly used for treating severe lymphoedema cases. The purpose of this study was to evaluate the effectiveness of free vascularized lymph node transfer (LNT) in stage II breast cancer-related lymphoedema patients in comparison with non-surgical management. During the last 3 years, 83 female patients were examined at our lymphoedema clinic. Finally, 36 cases were included in this study and randomly divided in two groups: group A patients ( n  = 18, mean age 47 years) underwent microsurgical LNT; followed by 6 months of physiotherapy and compression, while group B patients ( n  = 18, mean age 49 years) were managed by physiotherapy and compression alone for 6 months. Patients of both groups removed their elastic garments after 6 months and were re-examined 1 year later. All the 36 patients had detailed evaluation of the affected extremity including limb volume measurement, infection episodes and scale scoring of pain, feeling of heaviness and functional status both at baseline and 18 month. Limb volume reduction was observed in both groups; mean reduction was greater in group A (57 %) than in group B (18 %). Infection episodes in group A were significantly reduced compared to those in group B patients. All group A patients reported painless and feeling of heaviness-free extremities with overall functional improvement, while the corresponding changes in group B patients were no more than marginal. Moreover, the LNT procedure was estimated as cost effective compared to conservative treatment alone. LNT represents an effective therapeutic approach for stage II lymphoedema patients; it significantly reduces limb volume, decreases recurrent infections and improves the overall function.

Background: This retrospective study aimed to assess the impact of certain flap characteristics on long-term outcomes following microsurgical treatment in Breast Cancer-Related Lymphedema (BCRL) patients. Methods: Sixty-four out of 65 BCRL patients, guided by the “Selected Lymph Node” (“SeLyN”) technique, underwent Vascularized Lymph Node Transfer (VLNT) between 2012 and 2018. According to their surface size, flaps were divided into small (<25 cm2, n = 32) and large (>25 cm2, n = 32). Twelve large and six small flaps were combined with free abdominally based breast reconstruction procedures. Lymphedema stage, flap size, vascular pedicle and number of lymph nodes (LNs) were analyzed in correlation with long-term Volume Differential Reduction (VDR). Results: At 36-month follow-up, no major complication was recorded in 64 cases; one flap failure was excluded from the study. Mean flap size was 27.4 cm2, mean LNs/flap 3.3 and mean VDR 55.7%. Small and large flaps had 2.8 vs. 3.8 LNs/flap (p = 0.001), resulting in 49.6% vs. 61.8% VDR (p = 0.032), respectively. Lymphedema stage and vascular pedicle (SIEA or SCIA/SCIP) had no significant impact on VDR. Conclusion: In our series, larger flaps included a higher number of functional LNs, directly associated with better outcomes as quantified by improved VDR.