Pulmonary and sinus diseases in primary humoral immunodeficiencies with chronic productive cough.

AIMS: To prospectively evaluate sinopulmonary disease in 24 patients with primary humoral immunodeficiency (11 with agammaglobulinaemia, nine with isolated IgA deficiency, and two with common variable immunodeficiency) and chronic productive cough, ascertain the usefulness of chest high resolution computed tomography (HRCT) in evaluating the progression of lung disease, and test a therapeutic approach to chronic sinusitis. METHODS: Pulmonary abnormalities were evaluated using lung function tests and HRCT (Bhalla score); chronic sinusitis was diagnosed clinically and confirmed by flexible fibreoptic endoscopy. Sixteen patients entered the three year follow up. RESULTS: Lung function testing revealed an obstruction in four patients; chest HRCT was abnormal in 16. There was a linear relation between the Bhalla score > or =4 and the number of months with cough/year over the previous two years (clinical score), and between the difference in clinical scores during follow up and in the previous two years and the difference in Bhalla score. The pulmonary lesions did not significantly progress over a three year period. Thirteen patients had chronic sinusitis; 6/10 patients followed up were successfully treated with antibiotics plus topical therapy and two with nasal polypoid disease with endoscopic sinus surgery. CONCLUSIONS: In patients with primary humoral immunodeficiency and chronic productive cough, HRCT is very useful in delineating the extent of lung damage. The correlation between Bhalla score and clinical findings and the favourable outcome of the disease suggests that in most patients chest HRCT should not be repeated annually as previously suggested. Medical therapy seems to be effective in many cases of chronic sinusitis.

Contrast-enhanced Doppler ultrasonography in the diagnosis of hepatocellular carcinoma and premalignant lesions in patients with cirrhosis.

Hepatocellular carcinogenesis in cirrhosis is a multistage process that includes large regenerative nodules, dysplastic nodules, and hepatocarcinoma. The aim of this study was to establish whether contrast-enhanced Doppler ultrasonography (US) is able to distinguish between early hepatocellular carcinoma (HCC) and small nonmalignant nodules in cirrhosis. Between January 1998 and December 1999, 500 cirrhotic patients with no previous history of HCC or evidence of hepatic focal lesions were enrolled and prospectively followed-up with US every 6 months until December 2000. Sixty-one patients developed focal lesions, 12 multifocal, and 49 monofocal. Biopsy of focal lesions, contrast-enhanced Doppler US, and spiral computed tomography (CT) were performed in 41 consecutive patients with small (<3 cm) monofocal lesions. Twenty nodules were diagnosed as HCC and 21 as nonmalignant (14 large regenerative nodules, 3 low-grade, and 4 high-grade dysplastic nodules) by liver biopsy. Intratumoral arterial blood flow was detected in 19 of 20 (95%) HCC and 6 of 21 (28%) nonmalignant nodules by contrast-enhanced Doppler US (P<.0001). The mean peak resistance and pulsatility indices were 0.82 +/- 0.09 and 1.56 +/- 0.2 in HCC and 0.62 +/- 0.08 and 0.82 +/- 0.08 in dysplastic lesions (P =.002 and.0001), respectively. Spiral CT revealed arterial perfusion in 19 of 20 HCC and in 4 of 21 nonmalignant nodules (high-grade dysplastic nodules). Four of the apparently false-positive nodules at enhanced Doppler US were high-grade dysplastic nodules and 2 evolved to HCC during follow-up. In conclusion, contrast-enhanced Doppler US is a noninvasive, very sensitive technique in differentiating malignant and premalignant lesions from nonmalignant focal lesions in the liver.

Favourable outcome of a case of pulmonary Langerhans' cell histiocytosis.

Pulmonary Langerhans' cell histiocytosis and eosinophilic granuloma are the terms used to describe a Langerhans' cell granulomatous interstitial lung disease of unknown aetiology, occurring predominantly in smokers and involving primarily lungs, bones, skin and lymph nodes. In this report a patient with fever, fatigue, dyspnoea, nocturnal perspiration and thoracic pain is described. The high-resolution computed tomography of the chest and histological examination of lung biopsies suggested the diagnosis of pulmonary Langerhans' cell histiocytosis. The disease was limited to the lung, since further investigations did not show any other localization. The patient had a good clinical outcome with avoidance of smoking and steroid therapy. The computed tomography scan follow-up showed a partial resolution of pulmonary lesions.

[Thoracic localizations of extramedullary erythropoiesis]. / Localizzazioni toraciche dell'eritropoiesi extramidollare.

A series of 8 patients affected with hemolytic beta thalassemia with intrathoracic extramedullary hematopoiesis is reviewed. All patients were studied with conventional radiologic techniques and with CT and MR imaging. There was good correlation between conventional radiology findings and MR and CT results. CT and MR imaging were useful to evaluate not only the topographic localization of the masses of extramedullary hematopoiesis, but also their functional status. On CT, masses in the active phase exhibit high density and marked enhancement after i.v. administration of contrast medium; in the remission phase, the masses are hypodense, due to fat substitution, and unenhanced. On MR images, the active phase is characterized by a relatively low signal in both T1 and T2, while in the remission phase high signal is observed in both T1 and T2. A sign is seen on MR images which is considered as pathognomonic: a peripheral ring with high signal. The use of CT and MR imaging allows detailed information on the evolution of the hemolytic disease to be obtained. These pieces of information cannot be obtained with conventional radiology, which allows, at any rate, the correct diagnosis to be made.