Scimitar Syndrome

Rarer Components of a Rare Disease: Accessory Hemidiaphragm and Horseshoe Lung with Scimitar Syndrome: Two Case Reports

Introduction: Scimitar syndrome is a rare developmental anomaly with an incidence of 2/100.000 births. Major components of this disease are partial anomalous pulmonary venous drainage, pulmonary hypoplasia, systemic arterialization of the right basal lung, and dextroposition of the heart. Horseshoe lung and accessory hemidiaphragm are two rarer components of this disease.

Case Presentation: In this paper, horseshoe lung and accessory diaphragm associated with Scimitar syndrome have been reported in two cases.

Conclusion: In conclusion, being aware of rare manifestations of rare diseases is important to fully describe the pathologic spectrum of the disease. This will assist in better management and decision-making process.

Diagnostic Strategy for Suspected Unilateral Absence of the Pulmonary Artery

Background: Unilateral absence of the pulmonary artery (UAPA) is a very rare congenital anomaly.

Objective: To analyze the diagnostic strategy applied to seven patients with UAPA who were examined and subsequently treated at the National Lung Hospital, Hanoi, Vietnam.

Methods: All seven patients, including three pediatric cases (1, 2, and 14 years old) and four adult cases (21, 26, 44, and 53 years old), had a history of recurrent pneumonia, and the clinical symptoms on admission included cough, progressive dyspnea, chest pain, and fatigue. The patients were initially examined clinically, followed by hematological testing, blood biochemistry testing, and chest X-ray radiology. The results suggested UAPA, so echocardiography and contrast-enhanced chest computed tomography (CT) were performed as soon as practical.

Results: The echocardiographic and CT imaging findings confirmed the suspected diagnosis of UAPA in all seven patients, which was accompanied by congenital heart disease in three patients. Three of the seven patients had mild and medium pulmonary hypertension. All seven patients were treated with drugs, which led to improvement in symptoms.

Conclusion: Frontal chest X-ray provided the initial signs suggesting a diagnosis of UAPA. Subsequent echocardiography and contrast-enhanced chest CT were effective diagnostic tools for fast and accurate confirmation of UAPA.

Group 5 Pulmonary Hypertension: Multiple Systemic Diseases, Multiple Mechanisms of Pulmonary Hypertension, and Multiple Management Challenges

Group 5 pulmonary hypertension (PH) with unclear and/or multifactorial mechanisms includes a wide variety of conditions associated with PH, and the mechanisms by which PH develops vary dramatically depending on the underlying condition. Indeed, in many group 5 conditions, such as sarcoidosis, multiple distinct drivers of PH are present concurrently in a single patient, with the predominant factor depending on the predisposing disease phenotype. For this reason, thorough diagnostic evaluation to most accurately phenotype every patient with group 5 PH is essential. Treatment of these patients should begin by fully characterizing and optimizing the management of their underlying disease, often in conjunction with disease experts. Initial targets of PH treatment include identifying and correcting factors that worsen PH, such as volume overload and hypoxemia, as well as a complete PH evaluation, searching for other undiagnosed causes of PH (e.g., congenital heart disease or chronic thromboembolic disease). Data to guide treatment with therapies specific to pulmonary arterial hypertension (PAH) are inadequate for any specific recommendations, and adverse effects in group 5 patients are common. If these therapies are considered, evaluation by a multidisciplinary team that includes a PH specialist is recommended. Factors in the selection of PAH therapies should include consideration of the dominant physiologic features of the underlying disease, the severity of hemodynamic and right ventricular abnormalities, the risk of adverse drug effects, and any known contraindications to PAH-specific medications based on the underlying condition. Vigilant monitoring following initiation of PAH-specific therapy is critical, as the clinical effects are hard to predict, and untoward events, such as uncovering pulmonary veno-occlusive disease, may occur. Collaborative care by a multidisciplinary team of experts is key to the management of this challenging patient population.

Variations and Abnormalities of Major Thoracic Vascular Structures

Purpose: To determine the variations and abnormalities of major thoracic vascular structures in patients exposed to thoracic examination via computerized tomography with 64 detectors.

Materials and Methods: In this retrospective study, 2,479 patients who underwent thoracic computed tomography between May 2006 and October 2007 in the Radiation Department at Dicle University’s School of Medicine were examined. Of these patients, 1,389 were male and 1,090 were female. No variations or abnormalities were detected in 1,588 of the patients.

Results: In 1,588 out of 2,479 patients (64.1%), no abnormalities or variations were detected. The most frequently detected variations occurred in the brachiocephalic trunk and the sole root point of the carotid artery from the aorta; these variations were detected in 838 (33.8%) patients.

In 74 of the 2,479 patients (3.0%) examined, it was found that the left vertebral artery directly initiated from the arch of the aorta. In addition, in 33 patients, the right brachiocephalic trunk and left major carotid artery initiated from the aorta as a single root.

Conclusion: Via multi-slice CT, it is possible to detect variations and abnormalities of major thoracic vascular structures; therefore, there is no need for extra diagnostic invasive digital subtraction angiography.

Raman Spectroscopy and Imaging: Promising Optical Diagnostic Tools in Pediatrics

This review focuses on the use of Raman spectroscopy, an analytical technique based on the inelastic scattering of harmless laser light with biological tissues, as an innovative diagnostic tool in pediatrics. After a brief introduction to explain the fundamental concepts behind Raman spectroscopy and imaging, a short summary is given of the most important and common issues arising when handling spectral data with multivariate statistics. Then, the most relevant papers in which Raman spectroscopy or imaging has been applied with diagnostic purposes to pediatric patients are reviewed, and grouped according to the type of pathology: neoplastic, inflammatory, allergic, malformative as well as other kinds. Raman spectroscopy has been used both in vivo, mostly using optical fibers for tissue illumination, as well as on ex vivo tissue sections in a microscopic imaging approach defined as “spectral histopathology”. According to the results reported so far, this technique showed a huge potential for mini- or non-invasive real-time, bedside and intra-operatory diagnosis, as well as for an ex vivo imaging tool in support to pathologists. Despite many studies are limited by the small sample size, this technique is extremely promising in terms of sensitivity and specificity.

A Sensitive Voltammetric Sensor for Detecting Betamethasone in Biological Fluids

A rapid and sensitive voltammetric sensor based on reduction of betamethasone has been developed using single wall carbon nanotube modified edge plane pyrolytic graphite electrode (SWNT/EPPGE). The reduction of betamethasone occurred in a well-defined, pH dependent peak. Linear calibration curve was obtained in the range 1 to 25 nM in 1.0 M phosphate buffer solution (PBS) of pH 7.2 with the limit of detection (3/slope) as 0.50 nM. The analytical utility of the developed method has been demonstarted by sensing the drug in human body fluids and for the determination of betamethasone content in several commercially available pharmaceutical preparations. Interfering effect of some common metabolites including ascorbic acid, uric acid, albumin and hypoxanthine has also been evaluated. A comparison of the observed results of proposed method with HPLC clearly indicates that the results of both methods are essentially similar.