Alpha-1-antitrypsin deficiency (carrier) as possible risk factor for development of colonic diverticula. A multicentre prospective case-control study: the ALADDIN study.

AIM: Connective tissue changes due to ageing or diseases leading to changes in the colonic wall are one theory for the development of diverticula. Alpha-1-antitrypsin (A1AT), a protease inhibitor that protects connective tissue, possibly plays a role in the aetiology of diverticulosis. The aim of this study was to explore associations between the development of diverticula and A1AT deficiency. METHODS: This was a multicentre prospective case-control study. A total of 221 patients aged ≥ 60 years with acute abdominal pain undergoing abdominal CT were included and analysed. Patients with diverticula were defined as the research group, patients without diverticula as controls. Genotype analysis for A1AT deficiency was performed. RESULTS: Twenty-six of 221 (11.8%) patients were diagnosed with (being a carrier of) A1AT deficiency. A non-significant difference in prevalence between patients with and without diverticula was found, 20 (13.9%) of 144 vs 6 (7.8%) of 77, respectively, with a crude OR of 1.9 (95% CI 0.7-5.0; P = 0.186) and after adjustment for confounders an adjusted OR of 1.5 (95% CI 0.5-4.0; P = 0.466). A non-significant difference in 30-day mortality rate from acute diverticulitis between A1AT deficient patients (or carriers) and those without was observed: two (22.2%) of nine patients with A1AT deficiency vs 1 (1.8%) of 55 without. CONCLUSION: We found no convincing evidence that A1AT deficiency plays a role in the aetiology of diverticulitis, although deficient patients and carriers had a higher mortality when experiencing diverticulitis. Diverticulitis is a multifactorial disease and larger numbers may be needed to explore the role of A1AT deficiency among other contributing factors.

Prevalence of alpha-1-antitrypsin deficiency carriers in a population with and without colonic diverticula. A multicentre prospective case-control study: the ALADDIN study.

PURPOSE: The underling pathophysiological mechanisms that cause the formation of colonic diverticula (diverticulosis) remain unclear. Connective tissue changes due to ageing that cause changes in collagen structure of the colonic wall is one theory. Alpha-1-antitrypsin (A1AT) is a protease inhibitor known to protect connective tissue in other organs. Associations between (carriers of) A1AT deficiency and the development of colonic diverticula will be the main focus of this study. METHODS: A multicentre prospective case-controlled study. In total, 230 patients ≥ 60 years with acute abdominal pain undergoing an abdominal computed tomography (CT) will be included. The research group consists of patients with diverticulosis and/or diverticulitis; controls are patients without diverticula (0 to ≤ 5 diverticula). Genotype analysis for A1AT deficiency will be performed. RATIONALE: Hypothetically, connective tissue changes, in particular related to (carriers of) A1AT deficiency, can contribute to the development of diverticula and diverticulitis. We expect to find a higher prevalence of A1AT carriers in patients with diverticulosis compared to patients without diverticulosis. Having diverticulosis does not affect the general health of these individuals per se, when asymptomatic. Once an association is found, present findings can be the basis for a second study to assess the risk of developing acute diverticulitis and its disease course in carriers of A1AT deficiency. Because a large cohort is needed in the latter, we shall first perform a pilot study to investigate the likelihood of the primary hypothesis. TRIAL REGISTRATION: Netherlands Trial register, NTR6251, NL55016.094.15.

Legionella longbeachae; don't miss it!

We here report on two immunocompetent patients admitted to our hospital within 3 weeks' time, both suffering from pneumonia caused by Legionella longbeachae (L. longbeachae). The pathogen was identified in broncho-alveolar lavage (BAL) liquid by Polymerase Chain Reaction (PCR), whereas sputum cultures remained negative. This organism is worldwide still relatively unknown and consequently underdiagnosed. However, with an increasing number of confirmed infections in Europe and more specifically in the Netherlands, early awareness and diagnostic measurements are indicated. As routine laboratory techniques like the urine antigen test do not detect L. longbeachae, we advocate early use of specific tests for non-pneumophila Legionella species such as PCR. Furthermore, we advocate the start of empirical antibiotic therapy (i.e. ciprofloxacin) and continuation in suspected cases.

Criteria for trismus in head and neck oncology.

The aim of this study was to determine a functional cut-off point for trismus in head and neck oncology. In total, 89 patients (13 dentate, 30 partially dentate and 46 edentulous) treated for cancer of the oral cavity or oropharynx were asked whether they experienced a limited mouth opening. The mandibular function impairment questionnaire (MFIQ) was filled out and mouth opening was measured. The proportion correctly predicted (proportion true positives+proportion true negatives) was calculated. For cut-off points from 25 to 45 mm, differences in MFIQ scores of the restricted and non-restricted groups were analyzed. A receiver operating curve was constructed. The proportion correctly predicted was highest for cut-off point

Contrast venography, the gold standard for the diagnosis of deep-vein thrombosis: improvement in observer agreement.

To determine whether the Rabinov-Paulin or the long-leg venography technique should be preferred in the diagnostic management of patients with clinically suspected deep-vein thrombosis, two independent experienced radiologists blindly assessed two different series of venograms of consecutive outpatients with clinically suspected deep-vein thrombosis. Venograms were obtained from two outpatient clinics of primary referral centres. In one centre the venograms were performed according to the technique of Rabinov and Paulin with the use of 100 ml of radiographic material and spot films of the calf, popliteal and more proximal veins. In the other centre, long-leg films were obtained after the administration of 150 ml of contrast material. The percentage venograms adjudicated as inadequate by at least one radiologist and inter-observer disagreement for both series were used as the main study outcome measures. Prior to the study, both radiologists agreed on the standardized criteria for a normal, abnormal and inadequate test result using a separate set of films. An inadequacy rate of 20% was found for the Rabinov-Paulin venography series (n = 123), whereas only 2% of the 126 long-leg films were inadequate for interpretation (p less than 0.001). The inter-observer diagreement for inadequacy, presence or absence of deep-vein thrombosis was 21% for the Rabinov and Paulin venograms and 4% for the long-leg films (kappa, 0.65 and 0.92; 95% confidence intervals: 0.53 to 0.77 and 0.84 to 0.99, respectively; p less than 0.002).(ABSTRACT TRUNCATED AT 250 WORDS)

Ileal obstruction due to tumorous heterotopic gastric mucosa.

A patient is reported with intermittent ileal obstruction due to tumorous heterotopic gastric mucosa not associated with an anatomic anomaly.

Detection of deep-vein thrombosis by real-time B-mode ultrasonography.

In 220 consecutive outpatients with clinically suspected deep-vein thrombosis of the leg, we compared contrast venography with real-time B-mode ultrasonography, using the single criterion of vein compressibility with the ultrasound transducer probe. The common femoral and popliteal veins were evaluated for full compressibility (no thrombosis) and noncompressibility (thrombosis). Both veins were fully compressible in 142 of the 143 patients with normal venograms (specificity, 99 percent; 95 percent confidence interval, 97 to 100). All 66 patients with proximal-vein thrombosis had noncompressible femoral veins, popliteal veins, or both (sensitivity, 100 percent; 95 percent confidence interval, 95 to 100). For all patients (including 11 with calf-vein thrombi), sensitivity and specificity were 91 (95 percent confidence interval, 82 to 96) and 99 percent, respectively. The sensitivity for isolated calf-vein thrombosis was only 36 percent. The compression ultrasound test was repeated in a subset of 45 consecutive patients by a second examiner, unaware of the results of the first test, whose results agreed in all patients with those of the first examiner (kappa = 1). We conclude that ultrasonography with the single criterion of vein compressibility is a highly accurate, simple, objective, and reproducible noninvasive method for detecting proximal-vein thrombosis in outpatients with clinically suspected deep-venous thrombosis.

Skin uptake of Gallium 67 in cutaneous sarcoidosis.

A patient is presented with cutaneous sarcoidosis of the scalp. Ga-67 was intensively taken up by the skin lesions. Systemic involvement of parotid glands and mediastinum was also demonstrated by Ga-67 scintigraphy. Prednisolone therapy reversed promptly the pathologic Ga-67 uptake. Ga-67 scintigraphy should be performed in all patients suffering from cutaneous sarcoidosis as being the most sensitive method to demonstrate systemic involvement.

Pityriasis rosea (Gibert): abnormal distribution pattern of antigen presenting cells in situ.

Pityriasis rosea is a skin disease which is obscure in its etiology and pathogenesis. We studied its immunopathology by immunophenotyping the inflammatory cells in situ using monoclonal antibodies that define leukocyte subsets. Findings as to T-cells and their major subsets did not reveal disease-specific data. Monocytes stained only rarely. Neither natural killer cells, B-cells nor plasma cells were ever found. An unexpected finding was the presence within the infiltrates and rarely within the epidermis of cells having the immunophenotype of interdigitating cells (RFD1+). Intense and dendritic staining with anti-T6 and anti-HLA-DR indicated Langerhans cells to be present in the dermal infiltrates, in between these infiltrates in the papillary dermis, and focally within the parakeratotic horny layer. This Langerhans' cell pattern provides evidence for dermal Langerhans cell compartmentalization and transepidermal Langerhans' cell elimination. Such a distribution indicates a change in Langerhans' cell migration processes in pityriasis rosea pathogenesis.