Case 276: Pulmonary Veno-Occlusive Disease and Pulmonary Capillary Hemangiomatosis Disease.

HistoryA 34-year-old man presented to the emergency department of our hospital for progressive shortness of breath and worsening productive cough of 2 weeks duration. He reported a 10-kg weight loss over 4 months but denied experiencing fever, chills, night sweats, or gastrointestinal, musculoskeletal, or neurologic symptoms. His medical history was unremarkable. Although he was a native of Morocco, he had lived in Europe for many years and worked as a truck driver. The patient had a smoking history but had quit smoking 5 years prior to presentation. He denied alcohol abuse or recreational drug use. He did not have any allergies. Besides bilateral clubbing, the physical examination findings were normal. At the time of admission, he had an oxygen (O2) saturation of 87% at ambient air, which increased to 100% with 1 L of O2 administered via a nasal cannula. The blood sample revealed a slight increase in his hemoglobin concentration (18.7 g/dL; normal range, 13.6-17.2 g/dL) and hematocrit level (50.8%; normal range, 39%-49%). His inflammatory parameters were normal, as were his hepatic and renal function. The arterial blood gas test showed partially compensated pulmonary alkalosis (pH, 7.43; normal range, 7.35-7.42; PCO2, 26 mmHg; normal range, 38-42 mmHg; PO2, 89 mmHg; normal range, 75-100 mmHg; bicarbonate level, 17 mEq/L [17 mmol/L]; normal range 22-26 mEq/L [22-26 mmol/L]). The results of the pulmonary function tests were expressed as the percentage of predicted values and were 92% for forced vital capacity, 93% for forced expiratory volume in 1 second, 116% for total lung capacity, and 60% for diffusing capacity of carbon monoxide. Anteroposterior chest radiography and enhanced chest CT were also performed at admission.

Case 276.

HistoryA 34-year-old man presented to the emergency department of our hospital for progressive shortness of breath and worsening productive cough of 2 weeks duration. He reported a 10-kg weight loss over 4 months but denied experiencing fever, chills, night sweats, or gastrointestinal, musculoskeletal, or neurologic symptoms. His medical history was unremarkable. Although he was a native of Morocco, he had lived in Europe for many years and worked as a truck driver. The patient had a smoking history but had quit smoking 5 years prior to presentation. He denied alcohol abuse or recreational drug use. He did not have any allergies. Besides bilateral clubbing, the physical examination findings were normal. At the time of admission, he had an oxygen (O2) saturation of 87% at ambient air, which increased to 100% with 1 L of O2 administered via a nasal cannula. The blood sample revealed a slight increase in his hemoglobin concentration (18.7 g/dL; normal range, 13.6-17.2 g/dL) and hematocrit level (50.8%; normal range, 39%-49%). His inflammatory parameters were normal, as were his hepatic and renal function. The arterial blood gas test showed partially compensated pulmonary alkalosis (pH, 7.43; normal range, 7.35-7.42; PCO2, 26 mmHg; normal range, 38-42 mmHg; PO2, 89 mmHg; normal range, 75-100 mmHg; bicarbonate level, 17 mEq/L [17 mmol/L]; normal range 22-26 mEq/L [22-26 mmol/L]). The results of the pulmonary function tests were expressed as the percentage of predicted values and were 92% for forced vital capacity, 93% for forced expiratory volume in 1 second, 116% for total lung capacity, and 60% for diffusing capacity of carbon monoxide. Anteroposterior chest radiography and enhanced chest CT were also performed at admission (Figs 1-3).[Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text].

Chronic Obstructive Pulmonary Disease: CT Quantification of Airway Dimensions, Numbers of Airways to Measure, and Effect of Bronchodilation.

PURPOSE: To determine the effect of bronchodilation on airway indexes reflecting airway disease in patients with chronic obstructive pulmonary disease (COPD) and to determine the minimum number of segmental and subsegmental airways required. MATERIALS AND METHODS: This study was approved by the local ethical committee, and written informed consent was obtained from all subjects. Twenty patients with COPD who had undergone pre- and postbronchodilator pulmonary function tests and computed tomographic (CT) examinations were prospectively included. Eight healthy volunteers underwent two CT examinations. Luminal area and wall thickness (WT) of third- and fourth-generation airways were measured twice by three readers. The percentage of total airway area occupied by the wall and the square root of wall area at an internal perimeter of 10 mm (√WAPi10) were calculated. The effects of pathologic status, session, reader, bronchodilation, and CT examination were assessed by using mixed linear model analyses. The number of airways to measure for a definite percentage error of √WAPi10 was computed by using a bootstrap method. RESULTS: There were no significant session, reader, or bronchodilation effects on WT in third-generation airways and √WAPi10 in patients with COPD (P values ranging from .187 to >.999). WT in third-generation airways and √WAPi10 were significantly different in patients with COPD and control subjects (P = .018 and <.001, respectively). Measuring 12 third- or fourth-generation airways ensured a maximal 10% error of √WAPi10. CONCLUSION: WT in third-generation airways and √WAPi10 are not significantly different before and after bronchodilation and are different in patients with COPD and control subjects. Twelve is the minimum number of third- or fourth-generation airways required to ensure a maximal 10% error of √WAPi10. (©) RSNA, 2015 Clinical trial registration no. NCT01142531 Online supplemental material is available for this article.

Severe COPD exacerbation: CT features.

UNLABELLED: Abstract Objective: To describe CT features associated with severe exacerbations of Chronic Obstructive Pulmonary Disease (COPD). MATERIALS AND METHODS: In this prospective ethical-committee-approved study, 44 COPD patients (34 men, 10 women, age range 49-83 years) who provided written informed consent were included at the time of hospital admission for severe exacerbation. Pulmonary function tests (PFT) and chest CT scans were performed at admission and after resolution of the episode following a minimum of 4 weeks free of any acute symptom. For each CT scan, two radiologists independently scored 15 features in each lobe and side. CT features and PFT results were compared for exacerbation and control through Mac-Nemar tests and paired t-tests, respectively. RESULTS: Forced expiratory volume in 1 second and vital capacity improved significantly after exacerbation (p = 0.023 and 0.012, respectively). Bronchial wall thickening and lymphadenopathy were graded significantly higher at exacerbation than at control by both readers (p ranging from < 0.001 to 0.028). Other CT features were not observed during exacerbation, or were so only by one reader (p ranging from < 0.001 to 0.928). CONCLUSION: Only lymphadenopathy and bronchial wall thickening are CT features associated with severe COPD exacerbation, respectively in 25% and 50% of patients. Our findings do not advocate a role for CT in the routine work-up of patients with severe COPD exacerbation.

Chronic obstructive pulmonary disease: CT quantification of airways disease.

Chronic obstructive pulmonary disease (COPD) is an increasing cause of morbidity and mortality worldwide and results in substantial social and economic burdens. COPD is a heterogeneous disease with both extrapulmonary and pulmonary components. The pulmonary component is characterized by an airflow limitation that is not fully reversible. In the authors' opinion, none of the currently available classifications combining airflow limitation measurements with clinical parameters is sufficient to determine the prognosis and treatment of a particular patient with COPD. With regard to the causes of airflow limitation, CT can be used to quantify the two main contributions to COPD: emphysema, and small airways disease (a narrowing of the airways). CT quantification--with subsequent COPD phenotyping--can contribute to improved patient care, assessment of COPD progression, and identification of severe COPD with increasing risk of mortality. Small airways disease can be quantified through measurements reflecting morphology, quantification of obstruction, and changes in airways walls. This article details these three approaches and concludes with perspectives and directions for further research.

Update on congenital nephrotic syndromes and the contribution of US.

The clinical classification of nephrotic syndrome (NS) is based on age at presentation. However, this classification is arbitrary because the majority of early onset NS has a genetic origin and has a widespread age of onset (from fetal life to several years). The aims of this review are to illustrate the knowledge accumulated on congenital nephrotic syndrome (CNS) in terms of genetics, classification, findings at histology and US-based on a review of the literature.

Variability of CT Airways Measurements in COPD Patients Between Morning and Afternoon: Comparisons to Variability of Spirometric Measurements.

RATIONALE AND OBJECTIVES: Computed tomography (CT) airways measurements can be used as surrogates to spirometric measurements for assessing bronchodilation in a particular patient with chronic obstructive pulmonary disease. Although spirometric measurements show variations within the opening hours of a hospital department, we aimed to compare the variability of CT airways measurements between morning and afternoon in patients with chronic obstructive pulmonary disease to that of spirometric measurements. MATERIALS AND METHODS: Twenty patients had pulmonary function tests and CT around 8 am and 4 pm. Luminal area (LA) and wall thickness (WT) of third and fourth generation airways were measured twice by three readers. The percentage of airway area occupied by the wall (WA%) and the square root of wall area at an internal perimeter of 10 mm (√WAPi10) were calculated. The effects of examination time, reader, and measurement session on CT airways measurements were assessed, and the variability of these measurements was compared to that of spirometric measurements. RESULTS: Variability of LA3rd and LA4th was greater than that of spirometric measurements (P values ranging from <.001 to .033). There was no examination time effect on √WAPi10, WT3rd, LA4th, or WA%4th (P values ranging from .102 to .712). There was a reader effect on all CT airways measurements (P values ranging from <.001 to .028), except in WT3rd (P> .999). There was no effect of measurement session on any CT airway measurement (P values ranging from .535 to >.999). CONCLUSION: As the variability of LA3rd and LA4th is greater than that of spirometric measurements, clinical studies should include cohorts with larger numbers of patients when considering LA than when considering spirometric measurements as end points.

Effect of total lung capacity, gender and height on CT airway measurements.

OBJECTIVE: Testing the hypothesis that CT airway measurements could be influenced by total lung capacity (TLC), gender and height in normal individuals. METHODS: In this ethics committee-approved prospective trial, 87 healthy never-smoking volunteers who provided written informed consent were included. From a helical CT scan of the chest, the wall thickness (WT) and the lumen area were twice measured in the third- and fourth-generation airways by three readers using a dedicated software. In addition, the TLC was also measured using CT (TLCCT). The percentage of airway area occupied by the wall (WA%) and the square root of wall area at an internal perimeter of 10 mm (√WAPi10) were calculated. CT airway measurements were compared between genders by using a mixed linear model. The correlations between height, TLCCT and CT airway measurements were tested by using a Pearson correlation analysis. These correlations were arbitrarily considered as significant if there was a significant correlation observed for each of the six measurement sessions. RESULTS: √WAPi10, WT3rd and WT4th were significantly different between genders. For both genders, there was no significant correlation between any airway measurements and TLCCT. In females only, WT3rd and WT4th were significantly and negatively correlated with height. CONCLUSION: √WAPi10, WT3rd and WT4th are different in males and females. WT3rd and WT4th are correlated with height in females only but not with TLC or in males. Advances in knowledge: CT airway measurements are influenced by gender and by height in females only but not by TLC. They should be adjusted according to gender and height in females.

Chronic lymphocytic leukemia mimicking recurrent carcinoma of the breast: case report and review of the literature.

Secondary localization of chronic lymphocytic leukemia (CLL) in breast is rare, while concurrent invasive ductal carcinoma and CLL manifesting as a collision tumor in breast is extremely rare. The observation of a CLL infiltration closely associated with a distinct breast neoplasm with the absence of any other localization for the leukemia is an indisputable argument for a relationship between the two diseases. The presence of both tumors is not simply due to chance. This association (CLL and carcinoma) has also been described in other organs. Hereafter, we report a second case of an 80 year-old woman in whom a leukemic infiltrate was confined to the region immediately surrounding poorly differentiated primary breast carcinoma, and we will discuss the association between CLL and carcinoma.