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Biomedical subjects

F Laghi

Publications and source records attributed to F Laghi.

29 records · Page 2Linked to original sources

Fechtner syndrome: report of a third family and literature review.

We observed macrothrombocytopenia with leucocyte inclusions in 10 out of 14 members of a four-generation family. Morphological features of leucocyte inclusions and the presence of Alport-related symptoms supported the diagnosis of Fechtner syndrome. Compared to the two previously reported Fechtner families, our kindred showed reduced expression of Alport manifestations. These, in members aged less than 50, were represented by clinically silent ocular abnormalities. Due to the frequent non-recognition of macrothrombocytopenia. Fechtner variants with low penetrance might be difficult to diagnose. In addition, Sebastian syndrome, recently distinguished from Fechtner disease in lacking Alport manifestations, might be one of these variants.

Adolescent↗

Common infections complicating lung transplantation.

Infections are the most common cause of early and late morbidity and mortality in lung transplant recipients. Viral infections, in particular, have been linked to acute rejection, chronic rejection, and development of lymphoproliferative diseases.

Diagnosis, Differential↗

Influence of serum proteins on fructosamine concentration in multiple myeloma.

Serum fructosamine levels in 36 subjects with various types of multiple myeloma and in 64 normal controls were evaluated by means of a Nitroblue tetrazolium colorimetric assay. Only the IgA myeloma group showed significantly raised serum fructosamine values (P less than 0.001). In the IgG myeloma group, which showed a higher mean serum protein concentration, serum fructosamine levels were not significantly different from controls. The study shows that elevated IgA levels do influence serum fructosamine and this effect should be taken into due consideration in order to avoid possible misinterpretations in evaluating this widely used index of glucose metabolism.

Adolescent↗

Neutrophil oxidative burst activation and the pattern of respiratory physiologic abnormalities in the fulminant post-traumatic adult respiratory distress syndrome.

The role of neutrophil oxidative burst activation (OBA) in the development of fulminant post-trauma adult respiratory distress syndrome (ARDS) was studied in 30 patients. Neutrophil (PMN) chemiluminescence (LE) was used as the index of OBA. Serially, for 8 days post-trauma, patient neutrophils (Pc) were studied in their own serum (Ps) normal serum (Ns), or Gey's solution (G). Ps was checked against normal neutrophils (Nc) for inhibition. LE was initiated by the addition of preopsonized zymosan to 1 x 10(6) PMN, the LE response monitored by luminometer, and the peak of the integral of LE recorded. Seven developed ARDS within the first 4 days; 12 patients developed sepsis (TS) but no ARDS, and 11 patients had uncomplicated trauma (TR). All ARDS showed increased LE (P less than 0.0001), at 48-96 hr. Patients without ARDS showed no significant increase in LE, although their mean injury severity (ISS) was the same. The ARDS LE response was mediated by activation of Pc [74%] with only a small but significant additional effect (6%) by ARDS serum (Ps): LE = 0.672 (Pc) + 0.24 [ARDS(Ps)] + 1343; N = 146, r2 0.733, P less than 0.0001. However, sera (Ps or Ns) was required, as incubation in G inhibited LE; [cells + s] greater than [cells + G], P less than 0.0001. LE is a biologic marker of ARDS, and the delay between injury and the LE indicated that initiation of ARDS may have therapeutic importance. Neutrophil activation in ARDS requires sera, but the ARDS effect appears mainly due to cells with only a small ARDS-specific serum-mediated role. The physiologic response to ARDS was evaluated by serial 8-hr studies of blood gases and pH; the respiratory index (RI) to pulmonary shunt (QS/QT) relationship, compliance (COMPL), and net fluid balance (DFLUID) PMN and platelet (PLAT) counts were also measured. Compared with TR and TS, the ARDS patients at 48-96 hr, showed increased RI, QS/QT, and DFluid requiring increased FiO2 and PEEP as COMPL and PLAT fell and LE rose. These changes were all simultaneously significant (P less than 0.05 to P less than 0.0001) by Bonferroni t-statistic applied to ANOVA. The clinical importance of these physiologic and biochemical responses was emphasized by the significantly (P less than 0.005) increased mortality in the ARDS patients. These data suggest that PMN LE and simple measures of respiratory function are early biologic markers of the development of fulminant post-traumatic ARDS and can be used to predict ARDS severity.

Adult↗

Prospective evaluation of combined high-frequency ventilation in post-traumatic patients with adult respiratory distress syndrome refractory to optimized conventional ventilatory management.

This study explores the value of combined high-frequency ventilation (CHFV) in a prospective clinical trial of 35 patients suffering from severe post-traumatic and/or septic adult respiratory distress syndrome (ARDS) who were refractory to conventional controlled mechanical ventilatory (CMV) support. The severity of ARDS was quantified by lung mechanics and gas exchange variables and the patients were classified on clinical grounds as well as on the basis of their respiratory index/pulmonary shunt relationship [RI/(Qsp/Qt)]. During the same time period as the CHFV study, data from these patients were compared to those from 88 ARDS patients who had quantitatively similar degrees of respiratory insufficiency, but who were treated only with controlled mechanical ventilation (CMV). The use of CHFV in the 35 CMV refractory patients resulted in an increase in expired tidal volume (VTE) by reducing the CMV inspired tidal volume (VTI) while increasing the volume component derived from high-frequency ventilation (HFV). This procedure appeared to reveal potentially salvageable ARDS patients who were refractory to CMV. In these patients, CHFV significantly reduced pulmonary mean airway pressure (Paw). The RI also decreased significantly and it was possible to reduce significantly the FIO2. In surviving ARDS patients treated with CHFV, an improvement in blood gases at reduced FIO2, without decreased cardiac output, was produced. The CHFV technique was used for less than or equal to 25 days and resulted in 23% survival of patients who were clinically and physiologically indistinguishable from the patients in the ARDS nonsurvivor group who were treated by CMV only. In surviving CHFV patients the decrease in Paw permitted a sustained, or increased, cardiac output with a rise in the oxygen delivery/oxygen consumption ratio, thus allowing for a higher PaO2 for any given level of pulmonary shunt.

Adolescent↗

Respiratory index/pulmonary shunt relationship: quantification of severity and prognosis in the post-traumatic adult respiratory distress syndrome.

The relationship between the respiratory index (RI = alveolar-arterial oxygen gradient [P(A-a)O2] normalized by PaO2) and the pulmonary shunt (Qsp/Qt) has been examined in 929 studies from 240 critically ill post-traumatic patients. Of these, 88 patients (443 studies) were individuals who developed post-traumatic adult respiratory distress syndrome (ARDS) and 152 were patients (486 studies) who did not develop ARDS. This study demonstrates that the RI to Qsp/Qt [RI/(Qsp/Qt)] relationship was significantly (p less than .0001) increased in patients who developed fatal ARDS compared with those who did not develop ARDS, or with those whose ARDS resolved. Because of the increased oxygen consumption (VO2) in ARDS patients in association with their severe limitations in gas exchange (RI) and increased Qsp/Qt, surviving ARDS patients had a significant increase in the cardiac index which resulted in a higher oxygen delivery to VO2 ratio. ARDS patients showed significant (p less than .0001) evidence of increased pulmonary vascular tone, correlated with the increase in the RI/(Qsp/Qt) relationship. In addition, those patients with high RI/(Qsp/Qt) also had increased right ventricular (RVSW) to left ventricular work (LVSW) ratios which were shown to be a direct function of the rise in RI. This increase in both RVSW/LVSW and RI/(Qsp/Qt) ratios was significantly (p less than .0001) correlated with an increased mortality. Thus, the RI/(Qsp/Qt) relationship, which can be obtained from arterial and mixed venous blood gases and saturations only, can be used to predict the severity of the ARDS process as well as important pulmonary vascular and right ventricular overload consequences.

Hemodynamics↗

Early hemostatic disorders in patients with gynecologic tumors.

One hundred and seventeen female subjects were studied: 23 patients with cervical carcinoma; 25 with endometrial carcinoma; 29 with benign uterine diseases; 14 with ovarian carcinoma; 26 patients with benign ovarian tumors. These patients, together with 25 healthy female control subjects, underwent several coagulation tests including Beta-Thromboglobulin (Beta-TG) and Platelet Factor 4 (PF4) plasma levels. Among all Beta-TG and PF4 exhibited the most interesting results. They were increased in four groups of patients: those with malignant 92.2% (13/14) and benign 50% (13/26) ovarian tumors and those with endometrial 64% (16/25) and cervical carcinoma 69.5% (16/23). Our study showed a high incidence of abnormalities of Beta-TG and PF4, early signs of hemostatic disorders, in gynecological malignancies especially in ovarian carcinoma. These data suggest a possible value of these tests as tumor-markers and in order to detect the patients who develop thrombo-embolic accidents.

Adult↗

Coagulation disorders in patients with tumors of the uterus.

Sixty-eight previously untreated female subjects were studied: 26 patients with cervical carcinoma, 22 with endometrial carcinoma, and 20 with benign uterine diseases. These patients, together with 25 healthy female control subjects, underwent several coagulation tests, including beta-thromboglobulin (beta-TG) and platelet factor 4 (PF4) plasma levels. Of all the parameters considered, the variations in beta-TG and PF4 were the most interesting. They were increased in patients with cervical and endometrial carcinoma. The sensitivity of the two tests reached 79% (15/19) for patients with invasive cervical carcinoma and 74% (16/22) for all patients with endometrial carcinoma. Our data demonstrate that among the investigated parameters beta-TG and PF4 are the earliest disorders of the hemostatic system and are more frequently increased in the gynecologic malignancies.

Adenocarcinoma↗

[Beta-thromboglobulin and platelet regeneration time in children with mitral valve prolapse].

We studied 12 children with echocardiographic evidence of mitral valve prolapse (MVP) in order to demonstrate platelet activation or consumption as risk for thromboembolism. Therefore, with a standard radioimmunoassay, we measured the plasma levels of Beta-thromboglobulin (BTG), a platelet-specific protein released during platelet release reaction. Platelet turn-over was evaluated by measuring the Platelet Regeneration Time (PRT) with a non-radioisotopic method. Blood samples for BTG assay were collected at rest and after exercise. BTG plasma levels obtained in children with MVP were significantly higher than in normal subjects (p less than 0.01), both at rest and after exercise. We found no difference in either group between BTG level at rest and after exercise. The TRP was within the normal range in all patients except 2, in whom TRP was slightly shorter. Increased BTG levels and normal TRP suggest that in our patients platelet activation, but not consumption, was increased. In children with MVP, periodic controls of platelet activation may be useful to detect an increased risk of thromboembolism.

Adolescent↗

Platelet-associated IgG in acute and chronic hepatic diseases.

In order to investigate the role of an immune mechanism in the pathogenesis of thrombocytopenia in hepatic patients, we measured platelet associated IgG (PAIgG) in 84 patients with various hepatic diseases. Increased PAIgG levels were found in 84% of patients with chronic active hepatitis (mean 21.32 +/- 8.45 fg/platelet) and in 75% of those with cirrhosis with hepatitis (mean 17.3 +/- 11.2 fg/platelet). In these patients there was no significant correlation between PAIgG and platelet count. Increased PAIgG amounts were also observed in some patients with inactive cirrhosis (18%). Normal PAIgG values were found in all patients with acute viral hepatitis and chronic persistent hepatitis. An immunologic mechanism may play a role in the pathogenesis of thrombocytopenia in hepatic patients. Furthermore, measurement of PAIgG may have a great usefulness in the differential diagnosis of chronic hepatic diseases.

Blood Platelets↗

Aspergillus and lung transplantation.

BACKGROUND: Aspergillus infection is a known complication of transplantation. METHODS: We describe our experience with 37 patients who received lung transplants over 2 years at Loyola University Medical Center. All patients who had evidence of aspergillus on culture of clinical specimens or had biopsies with hyphal forms consistent with aspergillus were categorized according to the clinical manifestations. Important risk factors were analyzed in comparison with other lung transplant recipients during the same period. RESULTS: The incidence of invasive aspergillosis was high (16%). No patient with disseminated disease survived. Locally invasive disease responded well to treatment with amphotericin B and itraconazole. CONCLUSIONS: Lung transplantation patients may have a higher incidence of aspergillosis as compared with other transplantation groups. Prophylactic measures need to be explored.

Adult↗