Francesco Vaccaro

Cardiopulmonary exercise testing (CPET) in pulmonary emphysema.

In patients affected by chronic obstructive pulmonary disease (COPD), cardiopulmonary response to exercise was never related to the severity of emphysema (E) measured by high resolution computed tomography (HRCT). Sixteen patients (age=65±8 yrs; FEV(1)=54±18%pred; RV=160±28%pred) with moderate to severe E (quantified by lung HRCT as % voxels <-910 HU) were exercised on a cycle-ergometer to exhaustion. Oxygen uptake (V˙(O2)), carbon dioxide output (V˙(CO2)), ventilation (V˙(E)), tidal volume (V(T)), and end-tidal P(CO2) (PET(CO2)) derived variables were measured breath-by-breath. The % of E correlated with: (1) the ratio V(Tpeak) (r=0.74; p=0.001); (2) the V˙(E)/V˙(CO2) slope (r=-0.77; p=0.0004); (3) PET(CO2) values at peak exercise (r=0.80; p=0.0001). Also, the %E was strongly predicted by the following exercise equation: %E(EST) = 58.1 + 11.9 × ΔV˙(E)/V˙(CO2) (r=0.94; p<0.0001). A V(Tpeak)/FEV1 ratio>1 is typically observed in severe E patients; furthermore, the V˙(E)/V˙(CO2) slope and the PET(CO2peak) values decrease and increase respectively as more as the emphysema is severe.

Development of factor VIII:C inhibitors following vaccination.

G.M. Ferri, MD, Department of’Medicina Clinica’, V.le dell’Università 37, I-00185 Rome (Italy) Acquired inhibitors against factor VIIL·C (FVIIL·C) in nonhemophilic patients are associated with various conditions, including autoimmune diseases (mainly rheumatoid arthritis and systemic lupus erythematosus), malignancies, the postpartum state, drug reactions, and dermatological disorders [1-4]. One case of circulating inhibitor associated with viral infection has also been described [5]. In this report, we describe the case of a 57-year-old male, diagnosed with lichenoid dermatosis, who developed severe hemorrhagic manifestations due to the appearance of inhibitors against FVIIL·C following vaccination with BCG and a pool of various strains of live attenuated corynebacteria. A 57-year-old male was admitted to our hospital in August 1989 with severe fatigue, bleeding from the gums, macroscopic hematuria, and spontaneous leg and forearm pain. He had no family or past history of a tendency to bleed. In September 1988, a lichenoid dermatosis of the scalp was diagnosed. Because of worsening of the skin lesions, vaccine therapy with BCG and a pool of various strains of live attenuated corynebacteria was advised, and was started in June 1989 in a private clinic, after the patient had given his informed consent. This therapeutic approach was adopted on the basis of preliminary unpublished results showing the efficacy of nonspecific immu-notherapy in lichenoid dermatosis. The vaccine was administered monthly by subcutaneous injection. Following the second booster, the hemorrhagic manifestations occurred. On examination, the patient appeared in poor general condition. Swelling of both calves and of the right forearm were present, due to the presence of hematomas. The liver and spleen were palpable 2 and 1 cm below the costal margin, respectively. Laboratory investigations revealed Hb 12.7 g/dl, WBC 5.2 × 109/1 (72% neutrophils, 26% lymphocytes, 1 % monocytes, and 1 % eosinophils), platelets 229 × 109/1 and ESR 80 mm in 1 h. Macroscopic hematuria was present. Liver enzymes, serum creatinine, and blood urea nitrogen were normal. Tests for VDRL, rheumatoid factors and antinuclear antibodies were negative. The prothrombin time (PT), the activated partial thromboplastin time (aPTT), and the quantification of ñbrinogen

Idiopathic AL amyloidosis and biclonal paraproteinemia: a case report and review of the literature.

A case of 79 year-old man slrffering from nephrotic syndrome, infiltrative cardiomyopathy and sensitive neuropathy of the lower limbs, associated with biclonal gammopathy (IgG k and IgA λ), is described. There was a history of non-insulin dependent diabetes mellitirs and of two lung nodules considered as benign lesions on the basis of cytologic, hematologic and instrumental examination. A rectal biopsy positive for amyloid deposition (Congo red histology and immunofluorescence study) led to the diagnosis of AL amyloidosis. Considering that the patient did not fulfill diagnostic criteria for lymphoproliferative diaseases (myeloma, lymphoma or Waldenström s niacroglubulinemia), nor for secondary malignant paraproteinemia, a diagnosis of idiopathic AL amyloidosis with biclonal gammopathy was made. Very few cases of idiopathic AL amyloidosis with double component are reported in the literature. Our review suggests that idiopathic AL amyloidosis with biclonal gammopathy is similar to idiopathic AL amyloidosis with monoclonal paraproteinemia in terms of clinical features, response to therapy and prognosis. Further studies, however, are necessary to clarify the true incidence and the clinical features of idopathic AL amyloidosis associated with biclonal gammopathy.

Idiopathic Mixed Cryoglobulinemia: A New Therapeutic Approach with Alpha Interferon

Idiopathic mixed cryoglobulinemia (I.M.C.) sustained by diffuse lympho-proliferation is a disorder of unknown cause characterized clinically by arthralgias, purpura, weakness, glomerulonephritis and generalized vasculitis (Meltzer et AL, 1966). The diagnosis is based on finding cryoprecipitable serum proteins generally consisting of policlonal IgG and either policlonal or monoclonal IgM with rheumatoid factor or antigammaglobulin activity. The treatment of cryoglobulinemia is directed toward minimizing the signs and symptoms due to the presence of cryoglobulins and consists of drug therapy, immunosuppressive agents and plasmapheresis. Neither plasmapheresis nor immunosuppresors achieve definite successful results in this disease. On the other hand the good results reported with IFN in lymphoproliferative diseases suggested us to try it in 2 patients affected with I.M.C.