Pelagia Kriki

Combined pulmonary fibrosis and emphysema associated with microscopic polyangiitis

To the Editors: Microscopic polyangiitis (MPA) is a necrotising multiorgan vasculitis associated with a variety of circulating autoantibodies, such as anti-neutrophil cytoplasm antibodies (ANCAs) against myeloperoxidase (MPO) [1]. Typical and most common pulmonary involvement comprises of alveolar haemorrhage secondary to pulmonary capillaritis as well as interstitial lung fibrosis [2] and progressive obstructive lung disease [1, 3, 4]. The combination of pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, encompassing a distinct radiology, revealing both upper-lobe emphysema and lower-lobe fibrosis on high-resolution computed tomography (HRCT) of the chest, as well as lung function profile, with apparently preserved lung volumes contrasting with disproportionally impaired gas exchange, as assessed by reduced diffusing capacity of the lung for carbon monoxide [5, 6]. CPFE has been recently described in the context of connective tissue diseases [7]. Nevertheless, it is still debatable whether CPFE represents a distinct syndrome or it is just a phenotype of pulmonary fibrosis with coincidental emphysema. Here, we describe for the first time, in a male patient, a novel type of pulmonary manifestation of MPA, the combination of pulmonary fibrosis and emphysema. In 2008, an 80-yr-old Greek-Caucasian male, heavy ex-smoker (80 pack-yrs), ex-farmer and coal worker with a history of idiopathic pulmonary fibrosis, based …

Pivotal Role of Paricalcitol in the Treatment of Calcific Uremic Arteriolopathy in the Presence of a Parathyroid Adenoma

Calcific uremic arteriolopathy, or calciphylaxis, is a serious and life-threatening complication of end-stage renal disease. Its pathogenesis is not yet fully elucidated and treatment is controversial. In the presence of severe hyperparathyroidism, parathyroidectomy should be considered. We report a case of a woman on maintenance hemodialysis therapy with calciphylaxis and parathyroid adenoma who refused to undergo parathyroidectomy. She was treated successfully with a combination of noncalcium phosphate binders, cinacalcet, and paricalcitol. Subcutaneous plaques disappeared, and the necrotic lesion was healed. Discontinuation of paricalcitol led to an increase in serum parathyroid hormone levels and reappearance of the patients symptoms, whereas its reintroduction resulted in complete remission of the clinical picture. Paricalcitol, a less calcemic vitamin D analogue, is also a selective vitamin D receptor activator with a number of nonclassic actions (such as inhibition of inflammation and ossification-calcification) that could prove beneficial in cases of calciphylaxis.

Calcific Uremic Arteriolopathy Treated with Cinacalcet, Paricalcitol, and Autologous Growth Factors

Background: Calcific uremic arteriolopathy is an uncommon cutaneous ischemic necrotizing disease, most commonly associated with renal disease and hyperparathyroidism, bearing a high mortality rate. Objective and Method: A case of a 57-year-old female renal patient with hyperparathyroidism who was successfully treated with combined paricalcitol and cinacalcet systemically, while autologous growth factors were locally applied, is herein presented. Result and Conclusion: The combination of cinacalcet and paricalcitol is a reliable alternative to parathyroidectomy in patients with calcific uremic arteriolopathy and hyperparathyroidism. Meticulous débridement of necrotic tissues is essential and application of autologous growth factors promotes healing.

Upper limb vascular calcification score as a predictor of mortality in diabetic hemodialysis patients

OBJECTIVE This study evaluated the correlation between an upper limb vascular calcification (Vc) score (VcS) and late all-cause mortality in diabetic hemodialysis patients with distal upper limb arteries medial wall sclerosis (Mönckeberg disease). METHODS We retrospectively reviewed Vc in bilateral upper limb plain radiographs and in duplex ultrasound images performed before radial-cephalic fistula (RCF) creation in diabetic hemodialysis patients. Only medial linear calcifications outlining the vessel wall were considered positive on X-ray images, whereas for ultrasound reviews, only continuous highly echogenic plaques producing bright white echos with shadowing were considered to be medial calcification. A VcS was then applied in each patient. Every half of each of the three main arterial conduits (brachial, radial, and ulnar arteries) in each arm was counted as 1 if it contained ≥ 6 cm of linear calcification, whereas absence of calcification or minimum calcification (length <6 cm) was counted as 0. Long-term all-cause mortality was compared between patients with a low or moderate VcS <8 (group I), patients with a high VcS ≥ 8 (group II), and patients with VcS = 0 (control group). Kaplan-Meier statistics were used for comparisons among the groups. RESULTS Nineteen patients had a VcS <8, 21 had VcS ≥ 8, and 43 patients had VcS = 0. The study patients had a mean age of 68 ± 10 years (range, 42-83 years; P = .23). Before early conversion to a RCF, dialysis therapy in 59 (71.1%) had already been initiated through central venous catheters (CVCs). The mean follow-up for groups I, II, and controls was 41.4 ± 41.2 months (range, 4-144 months), 34.15 ± 31.3 months (range, 1-108 months), and 66.7 ± 32.5 months (range, 12-126 months), respectively (P = .0009). Forty-seven patients died during the follow-up period (12 in group II and 24 in the controls; P = .88). Survival rates at 12, 24, 36, and 48 months were 78.3%, 65.7%, 54.8%, and 48.1% for group I; 75.2%, 58.8%, 49.3%, and 42% for group II; and 97.7%, 93.1%, 76.8%, and 71.8% for the control group, respectively (P = .013 for all groups; P = .044 for group II vs controls). Patients with (subgroups) or without CVCs at baseline had similar late mortality rates. Patients with CVCs/Vc had lower survival rates than those with CVCs/no Vc at 1 year (73.3% vs 96.5%) and at 3 years (47.7% vs 75.8%; P = .038). CVCs were related to increased risk of death only in subgroup II patients compared with the subcontrol group patients (75.4% vs 37.9% at 5 years, respectively; P = .034). CONCLUSIONS Diabetic hemodialysis patients exposed to high levels of upper extremity arterial medial VcSs upon receiving RCFs have an increased long-term mortality risk compared with diabetic hemodialysis patients with no Vc and receiving the same access. Patients with CVCs/Vc had the lowest survival rates.

Comparative in vitro Study of Different Peritoneal Dialysis Solutions on Cytokine Production by Peripheral Blood Mononuclear Cells

Background/Aims: Peritoneal dialysis solutions (PDS), new and conventional, do not yet have a clinical biocompatibility profile. We aimed at a comparative laboratory profile based on the effect of PDS on peripheral blood mononuclear cell (PBMC) cytokine release. Method: PBMCs from 19 healthy volunteers were incubated at a concentration of 106/ml in fresh PDS and control medium (RPMI 1640), and stimulated or not with 10μg/ml Escherichia coli lipopolysaccharide. The tested PDS were glucose/lactate 1.5 and 4.25%, glucose/pyruvate 1.0 and 4.0%, icodextrin and amino acid solutions. The initial incubation in culture flasks for 15 min was followed by 1:1 dilution with RPMI and by additional incubation for 22 h as a ‘recovery period’. At the end, the supernatants were tested for cytokines IL-6 and TNFα by ELISA. Results: The hypertonic glucose solutions, irrespective of the buffer, had the most adverse effect on PBMC cytokine release. The icodextrin and amino acid solutions seemed close to the isotonic glucose PDS. The substitution of pyruvate for lactate buffer seemed to offer an advantage only for the hypertonic glucose-based solutions. Conclusions: Icodextrin and amino acid solutions seem to offer an advantage only compared to hypertonic glucose PDS.

Rigorous Vibrio vulnificus Soft Tissue Infection of the Lower Leg in a Renal Transplant Patient Managed by Vacuum Therapy and Autologous Growth Factors

Background: Vibrio vulnificus is a gram-negative marine bacterium that grows well in coastal waters. It is an opportunistic pathogen that can cause serious life-threatening infections in patients with certain health conditions. Vibrio-induced wound infections in immunosuppressed patients are difficult to treat because the healing process may be significantly delayed. Reconstructive surgery may not be successful in early treatment as skin grafts are likely to fail, and there may be increased morbidity of donor sites of grafts or flaps. Objective: Herein a case of septicemia and wound necrosis owing to V. vulnificus wound infection in a renal transplant patient is reported. Method: To conservatively yet adequately débride the wound bed, stimulate angiogenesis, and accelerate granulation, vacuum-assisted closure was employed. Granulation was further enhanced by autologous platelet concentrate spray, which has also been reported to increase the epithelialization rate. Result: Complete epithelialization of the wound was achieved 4 weeks after completion of treatment. Conclusion: Noninvasive advanced modalities may be employed to successfully treat infectious soft tissue deficits in immunocompromised patients.