Magnetic resonance imaging and ultrasound evaluation of "healthy" joints in young subjects with severe haemophilia A.

Magnetic resonance imaging (MRI) and ultrasonography (US) are increasingly used in haemophilia A (HA) to detect early joint changes. A total of 40 clinically asymptomatic joints, never involved by bleeding events ["healthy joints" (HJ)], were evaluated by MRI and, in parallel, by US in 20 young subjects with severe HA (22.45 ± 2.72 years old; no history of arthritides, of viral infections or of inhibitors against factor VIII). The same joints were evaluated in 20 matched non-haemophilic (no-HA) subjects (mean age 23.90 ± 2.31 years, P = 0.078 vs. HA subjects). US images were obtained with specific probe positions according to validated procedures. A validated US score and progressive (P-MRI) and additive (A-MRI) MRI scores were employed for data collection and analysis. The US score was higher in HA than in no-HA subjects (3.40 ± 1.72 vs. 0.80 ± 1.10, P < 0.001). Taking into account only moderate/severe alterations, joint effusion was found in 55% of HA and in 5% of no-HA joints (P < 0.001); synovial hypertrophy was found in 20% of HA and in none of the no-HA joints; cartilage erosion was found in 30% of HA and in none of no-HA joints. MRI examinations confirmed these findings and the US score correlated with the A-MRI (r = 0.732, P < 0.001) and with the P-MRI (r = 0.598, P < 0.001) scores. MRI and US data significantly correlated as to effusion (r = 0.819, P = 0.002), synovial hypertrophy (r = 0.633, P = 0.036) and cartilage erosion (r = 0.734, P = 0.010). Despite inherent limitations, joint US examination identified subclinical abnormalities of HJ in young subjects with severe HA.

Long-term clinical results and MRI changes after tendon ball arthroplasty for advanced Kienbock's disease.

The purpose of this study was to assess the long-term clinical results and morphological changes after tendon ball arthroplasty for advanced Kienböck's disease. Twenty-six patients were reviewed, with a mean follow-up interval of 125 months (range 50-226). At follow-up, mean score on the Disabilities of the Arm, Shoulder, and Hand questionnaire was 7.7 and mean visual analogue scale score for pain was 1. Mean carpal height ratio was significantly reduced with respect to the pre-operative value. On magnetic resonance imaging scans, cartilage damage, synovitis, and erosive or oedematous changes in the bones were detected in most patients. Calcification in the defect filled by the tendon ball was seen in all patients. Narrowing of the radioscaphoid joint and the presence of intercarpal synovitis were negatively associated with clinical outcome. Tendon ball arthroplasty in advanced Kienböck's disease results in long-term satisfactory clinical outcomes, despite widespread changes in the bones and joints within the wrist.

Interleukin (IL)-6 and receptor activator of nuclear factor (NF)-kappaB ligand (RANKL) are increased in the serum of a patient with primary pachydermoperiostosis.

Primary pachydermoperiostosis (PDP) is a rare syndrome, characterized by digital clubbing, periostosis, and pachydermia. We have evaluated biochemical bone turnover markers, including components of interleukin-6 (IL-6) and osteoprotegerin/receptor activator of nuclear factor (NF)-kappaB ligand (OPG/RANKL) systems, in an 18-year-old man affected by primary PDP. The acute phase of the disease was characterized in our patient by high serum levels of IL-6 and RANKL. The observed high serum levels of these parameters are associated with increased values in markers of bone resorption (degradation products of C-terminal telopeptides of type-I collagen and urinary hydroxyproline/creatinine ratio) and reduced serum levels of bone alkaline phosphatase, a marker of bone formation. Serum levels of osteotrophic hormones were in the normal range. Our data suggest that, despite the radiographic findings, the acute phase of primary PDP is characterized by increased bone resorption, probably mediated by IL-6 and RANKL.

Triphasic helical CT in Budd-Chiari syndrome: patterns of enhancement in acute, subacute and chronic disease.

AIM: To retrospectively evaluate helical computed tomography (CT) findings in a series of consecutive patients with Budd-Chiari syndrome. METHODS: Patterns of enhancement observed at contrast-enhanced helical CT in 10 consecutive patients (six women, four men; aged 27-51 years) with either acute, subacute or chronic Budd-Chiari syndrome were retrospectively evaluated along with the status of the hepatic veins. All patients underwent triphasic helical CT (10 mm beam collimation, 7 mm rec. intervals, 120 kV, 200-250 mA, pitch = 1.0) performed at 20-25, 70-75 and 300 s after i.v. bolus (3 ml/s) injection of 150 ml iodinated non-ionic contrast media. RESULTS: Abnormal patterns of enhancement were identified in eight patients. In all patients with acute Budd-Chiari disease (3/3) abnormal arterial enhancement of the caudate lobe, the so-called "fan-shaped pattern" was observed, whereas visible venous thrombosis was only depicted in two. Conversely, a "patchy pattern" of enhancement was observed in five out of seven patients with either sub-acute (2) or chronic Budd-Chiari disease (5) along with a strip-like appearance or lack of visualization of hepatic veins. CONCLUSIONS: The "fan-shaped" pattern of enhancement represent a characteristic finding of acute Budd-Chiari disease, and it may help to suggest the correct diagnosis even in absence of visible venous thrombosis.

Splenic hypoperfusion as a sign of systemic amyloidosis.

BACKGROUND: The spleen is frequently involved in systemic amyloidosis; however, the computed tomographic (CT) or magnetic resonance (MR) pattern of splenic amyloidosis is not sufficiently described in the literature. This study evaluated the contrast-enhanced CT and MR findings of the spleen in patients with systemic amyloidosis. METHODS: Data were extracted by reviewing pathology and radiology department records of the teaching hospital of Naples over 10 years, from 1 January 1993 to 31 December 2002. Thirty-three patients with amyloidosis were identified, 10 of whom had a CT scan and two of whom had an MR study. The population-based study was composed of 12 patients with histologically proved amyloidosis who underwent contrast-enhanced CT or MR scan of the abdomen. The spleen and liver were evaluated for organ size and perfusion. RESULTS: The spleen was hypoperfused in nine of 12 patients. Mild splenomegaly was present in only one case. Hepatomegaly was associated with markedly acute left lobe margin in nine patients and with rounded anterior profile of segments 3 and 4 in four patients. Moreover, a large area of low attenuation with indefinite geographic margins involving the right hepatic lobe was observed in three patients. CONCLUSION: The finding of splenic hypoperfusion may be a marker of systemic amyloidosis, which represents a useful clue when clinical findings fail to suggest the proper diagnosis.

Hepatic and splenic amyloidosis: dual-phase spiral CT findings.

Although the liver and spleen are frequently involved in primary systemic amyloidosis, the clinical manifestations of hepatic and splenic involvement are usually mild and a dominant symptomatic hepatic presentation is uncommon. We report a case of a 51-year-old woman with giant hepatomegaly, hypertransaminasemia, increase in alkaline phosphatase, and ascites, in whom the findings of dual-phase spiral computed tomography suggested liver and splenic amyloidosis.

[Morphological imaging of gastroenteropancreatic neuroendocrine tumours]. / Imaging morfologico dei tumori neuroendocrini gastroenteropancreatici.

Biologically active neuroendocrine tumours produce early symptoms and are often difficult to diagnose owing to their small dimensions (<1 cm), whereas biologically inactive forms are often coarse and sometimes found by chance. As well as identifying the lesion, locoregional staging is also particularly important for therapeutic planning. Morphological imaging plays an important role in the identification of gastroenteropancreatic neuroendocrine tumours, providing an anatomic substrate for receptorial imaging which usually precede it in the diagnostic work-up, whereas it plays a primary role in the locoregional staging of these neoplasms for which surgery is the first and essential therapeutic approach. In the case of endocrine tumours of the pancreas alone, the most accurate method of diagnosis is currently echo-endoscopy using high-frequency probes. Two-phase spiral CT and dynamic MR have proved equally effective means of identifying endocrine tumours of the pancreas with slightly higher sensitivity for MR, both playing a role in the locoregional staging of biologically active and inactive tumours. Traditional radiology also plays a role in the identification of intestinal carcinoids.

[CT in findings intestinal carcinoids]. / Aspetti TC dei carcinoidi intestinali.

CT findings in intestinal carcinoids are various. While recognition of the primary tumour is a rare event, the contribution of CT in evaluating patients with suspected intestinal carcinoid is relevant, both for loco-regional staging and during long-term follow-up. Moreover, in the relatively frequent cases with an aspecific clinical onset, CT may be findings useful to direct diagnosis.

[Abdominal mass in childhood: characterization with fine needle percutaneous biopsy guided with computerized tomography]. / Masse addominali nell'età pediatrica: caratterizzazione mediante biopsia percutanea con ago sottile guidata con Tomografia Computerizzata.

INTRODUCTION: CT-guided fine needle aspiration biopsy (FNAB) is known to improve diagnosis of expansile abdominal lesions, especially relative to more invasive procedures like explorative laparotomy. FNAB is not commonly used in pediatric patients because of their poor collaboration and of associated risks. We investigated the feasibility of FNAB in the pediatric age. MATERIAL AND METHODS: Over a 2-year period, we performed CT-guided FNAB of 21 abdominal lesions in a series of pediatric patients ranging in age 10 days to 14 years. Thirteen lesions were in intraperitoneal and 8 in retroperitoneal sites. CT had been performed in all patients but had failed to make a diagnosis. Cytologic samples were obtained with 22-23 G needles; the cytologist was always present to ensure adequate sampling. Follow-up CT was performed to assess the possible onset of complications. RESULTS: First-pass diagnosis was made in 14 of 21 biopsies and second-pass diagnosis in 5; histology was needed in three cases. Cytologic findings were compared with postoperative histologic results in 13 cases; clinical follow-up and further instrumental studies confirmed the diagnosis in nonsurgical patients. CONCLUSIONS: CT-guided FNAB can be performed in pediatric patients with accuracy and confidence. These patients' age calls for great skills of the operator and possible contraindications must be accurately evaluated; complications must not be neglected. FNAB should be performed during CT examination because young patients often require anesthesia. The pathologist's presence during biopsy permits to repeat sampling, if necessary, without repeating the anesthesia. CT-guided FNAB is a valid alternative to explorative laparotomy in the workup of expansile abdominal masses also in pediatric patients.

Soluble interleukin-2 receptor and soluble CD8 molecules in cerebrospinal fluid and serum of patients with multiple sclerosis.

The purpose of this study was to analyse soluble interleukin-2 receptor (sIL-2R) and soluble CD8 (sCD8) molecules in the cerebrospinal fluid (CSF) and serum of 18 patients with definite multiple sclerosis (MS) and of 16 with noninflammatory neurologic diseases (NIND). All MS patients suffered from an exacerbation of the relapsing-remitting form of the disease within one month before examination. The mean serum levels of sIL-2R and sCD8 in the MS patients were not significantly different from those of NIND patients. Only one patient with MS had detectable sIL-2R in the CSF. CSF sCD8 was detectable in 10 of 18 MS patients and in 1 of 16 NIND patients. Our data indicate that the CSF and serum sIL-2R concentrations do not correlate with the disease activity. Conversely, increased levels of sCD8 only in the CSF of MS patients support the hypothesis of an intrathecal activation of CD8+ cells in MS. We think that CSF sCD8 can be a useful marker for the presence of activated T cells in the central nervous system.

Unilocular hydatid liver cysts: treatment with US-guided, double percutaneous aspiration and alcohol injection.

Sixteen hydatid liver cysts in 14 patients were treated with a percutaneous double puncture-aspiration-injection (D-PAI) technique with alcohol used as the scolecidal agent. With ultrasound guidance, fine-needle drainage of cysts was performed, and 95% sterile alcohol was injected and left in situ to partly refill the cystic cavities. The same procedure, without reaspiration of the injected alcohol, was performed 3 days later. Viability of scoleces was assessed at each aspiration. Benzoimidazolic drugs were administered 1 week before and 3 weeks after the procedure, to reduce the risk of seeding scoleces. Follow-up ranged from 8 to 28 months (mean, 14 months). Six cysts disappeared within 40-75 days of completion of D-PAI. In the other patients, smaller liquid areas or hypo- or hyperechoic solid masses were observed. Anaphylactoid reactions did not occur. In one patient, a biliary fistula developed after the first aspiration; the second ethanol injection was postponed until 6 months later but was effective. Viable scoleces were found at the second aspiration in only two patients whose hepatic liver cysts completely healed. Serologic titers substantially decreased in seven cases and became negative in two.

Cytokines in the pathogenesis of multiple sclerosis.

The authors evaluate the involvement of various cytokines (interleukin-1, interleukin-2, interleukin-4, interleukin-6, tumor necrosis factor alpha and gamma-interferon) in the pathogenesis of multiple sclerosis. The cytokines might participate in nervous tissue damage by promoting demyelination and oligodendrocyte injury or by enhancing local immune response. In addition, several authors reported increased levels of some cytokines in serum and cerebrospinal fluid of patients with multiple sclerosis. These findings suggest that cytokines can play a significant role in the immunopathogenesis of the disease.

[Levels of interleukin-4 (IL-4) in lymphocyte cultures of subjects with HIV-1 infection]. / Levels of interleukin-4 (IL-4) in lymphocyte cultures of subjects with HIV-1 infection.

The authors report IL-4 levels in peripheral blood mononuclear cells (PBMC), stimulated with MoAb anti-CD3, in subjects with HIV-1 infection, classified according to the criteria of Centers for Disease Control (CDC) in various stages. The results "in vitro" have indicate that PBMC IL-4 levels were in normal range also in advanced stages of retroviral infection, and were correlated to low levels of CD4+ lymphocytes and, sometimes, to high levels of IgE immunoglobulins. The normality of IL-4 levels in advanced stages or in presence of decreased CD4+ cells, suggests that the production of this cytokine is also present in other subsets of mononuclear cells not involved in this infection. Moreover the presence of significant correlation between high levels of Ig E and IL-4 suggests a possible pathogenetic role of IL-4 in the determination of isotypic switch of this immunoglobulin.