Williams-Campbell syndrome is a rare congenital syndrome characterized by the absence of cartilage in subsegmental bronchi leading to formation of bronchiectasis distal to the affected bronchi. The differential diagnosis of bronchiectasis is broad and the rarity of the disease poses a diagnostic and management challenge for clinicians. This present review aims to help the understanding of the clinical manifestations, pathophysiological features, diagnostic modalities, management and differential diagnosis of Williams-Campbell syndrome. A MedLine/PubMed search was performed identifying all relevant articles. No restrictions were used for publication dates. The author used the keywords "Williams-Campbell syndrome," "non-cystic fibrosis bronchiectasis" and "congenital bronchiectasis" finding 503, 195 and 489 articles, respectively.

Background: Atherosclerotic coronary artery disease (CAD) has long been shown to involve chronic low-grade subclinical inflammation. However, whether there is association between hematological indices assessed by complete blood count (CBC) and coronary atherosclerotic burden has not been well studied. Materials and Methods: Consecutive 868 patients without known CAD who presented with acute chest pain to emergency department and underwent coronary artery calcium (CAC) scoring evaluation by multi-detector cardiac computed tomography were included in our study. Clinical characteristics and CBC indices were compared among different CAC groups. Results: The cohort comprised 60% male with a mean age of 61 (SD = 14) years. Median Framingham risk of CAD was 4% (range 1-16%). Median CAC score was 0 (IQR 0-43). Higher CAC groups had significantly higher Framingham risk of CAD than lower CAC groups (P < 0.001). Among different CAC categories, there was no statistically significant difference in hemoglobin level (p 0.45), mean corpuscular volume (p 0.43), mean corpuscular hemoglobin (p 0.28), mean corpuscular hemoglobin volume (p 0.36), red cell distribution width (0.42), total white blood cell counts (p 0.291), neutrophil counts (p 0.352), lymphocyte counts (p 0.92), neutrophil to lymphocyte ratio (p 0.68), monocyte count (p 0.48), and platelet counts (p 0.25). Conclusion: Our study did not detect significant association between hematological indices assessed with CBC and coronary calcification in symptomatic patients without known CAD.

Background: Autotelic personality (AP) is known to have a positive effect on the quality of life. We hypothesized that inferiority feelings may be less pronounced in individuals with AP than in those with an average (AV) or a nonautotelic personality (NAP). Aims: This study aimed to compare inferiority feelings among three personality groups: An AP group, an AV group, and an NAP group. Materials and Methods: This study was a cross-sectional survey among 148 undergraduate students aged 18-24 undertaken in Okayama, Japan. Participants completed the Flow Experience Checklist and Inferiority Feelings Scale. Results: With the number of flow activities, participants were classified into three groups: 3+ for AP (n = 28, 18.9%), 1-2 for AV (n = 72, 48.6%), and 0 for NAP (n = 48, 32.4%). One-way analysis of variance showed significant differences among the three groups with respect to the Inferiority Feelings Scale. Multiple comparison analysis using Tukey's test showed that inferiority feelings in AP were significantly less pronounced than in the NAP group. Conclusion: The results of this study indicated that AP was lesser than NAP in association with pronounced inferiority feelings.

Background: Anatomical variations of the recurrent laryngeal nerve (RLN) such as extralaryngeal terminal bifurcation is an important risk for its motor function. Aims: The objective is to study surgical anatomy of bilateral bifurcation of the RLNs in order to decrease risk of vocal cord palsy in patients with bifurcated nerves. Materials and Methods: Surgical anatomy including terminal bifurcation was established in 292 RLNs of 146 patients. We included patients with bilateral bifurcation of RLN in this study. Based on two anatomical landmarks (nerve-artery crossing and laryngeal entry), the cervical course of RLN was classified in four segments: Pre-arterial, arterial, post-arterial and pre-laryngeal. According to these segments, bifurcation point locations along the cervical course of RLNs were compared between both sides in bilateral cases. Results: RLNs were exposed throughout their entire courses. Seventy (48%) patients had bifurcated RLNs. We identified terminal bifurcation in 90 (31%) of 292 RLNs along the cervical course. Bilateral bifurcation was observed in 20 (28.6%) patients with bifurcated RLNs. Bifurcation points were located on arterial and post-arterial segments in 37.5% and 32.5% of cases, respectively. Pre-arterial and pre-laryngeal segments contained bifurcations in 15% of cases. Comparison of both sides indicated that bifurcation points were similar in 5 (25%) and different in 15 (75%) patients with bilateral bifurcation. Permanent nerve injury did not occur in this series. Conclusion: Bilateral bifurcation of both RLNs was observed in approximately 30% of patients with extralaryngeal bifurcation which is a common anatomical variation. Bifurcation occurred in different segments along cervical course of RLN. Bifurcation point locations differed between both sides in the majority of bilateral cases. Increasing surgeons' awareness of this variation may lead to safely exposing bifurcated nerves and prevent the injury to extralaryngeal terminal branches of RLN.

Background: Triage at emergency department is performed to identify those patients who are relatively more serious and require immediate attention and treatment. Despite current methods of triage, trauma continues to be a leading cause of morbidity and mortality. Aims: This study was to evaluate the predictive value of shock index (SI) and modified shock index (MSI) for hospital mortality among adult trauma patients. Materials and Methods: In this prospective longitudinal study, all adult patients who sustained trauma enrolled as per as inclusion/exclusion criteria. After the collection of data, SI and MSI were calculated accordingly. All parameters were again recorded hourly and calculations were done at six-hour intervals. Further, to achieve a value that can be analyzed, we determined threshold value for vital signs, which set the threshold values as heart rate at 120 beats per minute, systolic blood pressure at less than 90, and SI at cut-off 0.5-0.9 and MSI at less than 0.7 to more than 1.3. Results: We analyzed 9860 adult trauma patients. Multivariate regression analysis demonstrated that heart rate more than 120 beats per minute, systolic blood pressure less than 90 mmHg, and diastolic blood pressure (DBP) less than 60 mmHg correlate with hospital stay and mortality rate. MSI <0.7 and >1.3 had higher odds of mortality as compared to other predictors. Conclusions: MSI is an important marker for predicting the mortality rate and is significantly better than heart rate, systolic blood pressure, DBP and SI alone. Therefore, modified SI should be used in the triage of serious patients, including trauma patients in the emergency room.

Background: Long-term regular follow up of ART is an important component of HIV care. Patients who are lost to follow-up (LTFU) while on treatment compromise their own health and the long-term success of ART programs. Aim: This study was aimed at determining the incidence and risk factors for LTFU in HIV patients on ART at ART clinic of Mizan-Aman General Hospital, Ethiopia. Materials and Methods: A retrospective cohort study of 2133 people living with HIV/AIDS and attending an ART clinic between 2005 and 2013 was undertaken. LTFU was defined as not taking an ART refill for a period of 3 months or longer from the last attendance for refill and not yet classified as 'dead' or 'transferred-out'. The log-rank test was used to measure differences in time to LTFU between groups and Cox proportional hazards modeling was used to measure predictors of LTFU. Results: Of 2133 patients, 53.9% were female. The mean (SD) age of the cohort was 31.5 (8.0), 16 (2.2), and 3.8 (3.0) years for adults, adolescents, and children, respectively. Around 574 (26.7%) patients were defined as LTFU. The cumulative incidence of LTFU was 8.8 (95% CIs 8.1-9.6) per 1000 person months. Patients with regimen substitution (HR 5.2; 95% CIs 3.6-7.3), non-isoniazid (INH) prophylaxis (HR 3.7; 95% CIs 2.3-6.2), adolescent (HR 2.1; 95% CIs 1.3-3.4), and had a baseline CD ₄ count < 200 cells/mm ³ (HR 1.7, 95% CIs 1.3-2.2) were at higher risk of LTFU. WHO clinical stage III (HR 0.6; 95% CIs 0.4-0.9) and IV (HR 0.8; 95% CIs 0.6-1.0) patients at entry were less likely to be LTFU than clinical stage I patients. There was no significant difference in risk of LTFU in males and females. Conclusion: Overall, these data suggested that LTFU in this study was high. Patients phase of life, drug related factors, and clinical stages were associated with LTFU in this study. Effective control measures in the at-risk population need to be implemented to improve retention.

Background: Emergency cases become a widespread problem in prisons across Turkey. The opening of a new prison hospital in January 2012 within the catchment of Silivri Penitentiary Institution gave a unique opportunity to treat the inmates quickly. Aims: The study was to conduct an extensive review for documentation of prisoners' healthcare problems leading to emergency admission following the first year after the opening of Penitentiary Institution Hospital and point to decrease redundant hospital transfers of this individual cohort. Materials and Methods: A cross-sectional study was carried out where 12,325 visits to the Silivri Penitentiary Institution Hospital for emergency visits from the period of 1 ^st January 2012 to the 31 ^st December 2012 were identified from electronic medical records. After obtaining consent from the local IRB, data including details of the type, cause and nature of the complaints of the illnesses were processed. Results: In the 12-month period, there were 12,325 visits to the emergency department, of which 4328 for surgical conditions (35.1%), 2684 for medical disorders (21.8%), 1867 for sports injuries (15.2%), 1327 for Ear Nose Throat (ENT) problems (10.8%), 827 for psychiatric disorders (6.70%), 396 for violence injury (3.2%), 169 for self harm (1.4%), and 727 for miscellaneous (5.8%). The most common cause of emergency visits was sports injuries, followed by non-specific abdominal pain and ENT problems. Eighteen prisoners re-attended 243 times, ranging from 8 visits to a maximum of 56 visits. Conclusion: Inmates in prison have a wide range of complaints, and sometimes these complaints do not suggest an illness. Prison population exhibited substantially higher prevalence rates of diseases than the civilian population. We conclude that this new healthcare system in prisons will prevent redundant hospital transfers and guarantee detainees have access to the same health care that is offered to non-detained population.

Background: Knowledge and attitude are significant factors impinging on whether individuals seek healthcare service. This flows on to impact public health knowledge of prevalence of diseases, and in turn, the practice of preventive medicine. As part of the international research collaboration agenda for Prediabetes and Cardiovascular Complications Study, a preliminary survey of one of the Ndokwa communities of Nigeria has been carried out. Aim: This study was to understand the baseline knowledge, attitudes and practices of a rural community in regards to cardiovascular diseases, and behavior toward risk management. Materials and Methods: Seventy-four volunteer participants were recruited, after public lectures, through secondary school and churches in the community. The survey was done using questionnaire. The knowledge component comprised questions about educational and personal health opinion. The attitude and practice components comprised questions about exercises and visiting healthcare facilities. Occupational backgrounds were also asked. Results: It is observed that majority of the community dwellers have (1) completed at least secondary education, (2) never attended a health check-up; and (3) do not engage in physical activity in the context of exercise. Twenty of the participants indicated not being in good health, of which only 35% have attended medical check-up for their ailment. Many of those who are yet to seek healthcare service cite affordability as their reason. With specific regards to diabetes and cardiovascular risk, over 71% of the survey participants are yet to do any blood sugar and/or lipid profile tests. Conclusion: This preliminary survey indicates that although the majority of respondents have secondary education and therefore are relatively literate, there is a gap between their knowledge of ill-health versus attitude and practice toward prevention; especially cardiovascular and diabetes diseases.

Background: Normal Percept with abnormal meaning (Agnosias) has been described from nineteenth century onwards. Later literature became abundant with information on the spectrum of Prosopagnosias. However, selective difficulty in identifying reflected self images with relatively better cognitive functions leads to problems in differentiating it from non-organic psychosis. Aim: In the present study, we investigated patients with dementia who showed difficulty in identifying reflected self images while they were being tested for problems in gnosis with reference to identification of reflected objects, animals, relatives, and themselves and correlate with neuropsychological and radiological parameters. Patients and Methods: Five such patients were identified and tested with a 45 cm × 45 cm mirror kept at 30-cm distance straight ahead of them. Results: Mirror image agnosia is seen in patients with moderate stage posterior dementias who showed neuropsychological and radiological evidence of right parietal dysfunction. Conclusion: Interpretation of reflected self images perception in real time probably involves distinct data-linking circuits in the right parietal lobe, which may get disrupted early in the course of the disease.

Background: Currently, the etiology of drug-induced gingival overgrowth is not entirely understood but is clearly multifactorial. Phenytoin, one of the common drugs implicated in gingival enlargement, is metabolized mainly by cytochrome P450 (CYP)2C9 and partly by CYP2C19. The CYP2C9 and CYP2C19 genes are polymorphically expressed and most of the variants result in decreased metabolism of the respective substrates. Aims: The present study was undertaken to investigate the influence of the CYP2C9*2 and *3 variant genotypes on phenytoin hydroxylation in subjects diagnosed with epilepsy from South India, thus establishing the genetic polymorphisms leading to its defective hydroxylation process. Materials and Methods: Fifteen epileptic subjects, age 9 to 60 years were included in the study. Among the study subjects, 8 were males and 7 were females. Genomic DNA was extracted from patients' blood using Phenol-chloroform method and genotyping was done for CYP2C9 using customized TaqMan genotyping assays on a real time thermocycler, by allelic discrimination method. The genetic polymorphisms *1, *2 and *3 on CYP2C9 were selected based on their function and respective allele frequencies in Asian subcontinent among the Asian populations. Results: CYP2C9*1*2 and CYP2C9*3/*3 were identified with equal frequency in the study population. There were seven subjects with CYP2C9*1/*2 genotype (heterozygous mutant), one subject with CYP2C9*1/*1 (wild type) and seven study subjects with CYP2C9*3/*3 (homozygous mutant). Conclusion: The results obtained in the present study will be helpful in the medical prescription purposes of phenytoin, and a more personalized patient approach with its administration can be advocated.

Context: Regional pericarditis is elusive and difficult to diagnosis. Healthcare providers should be familiar with post-cardiac ablation complications as this procedure is now widespread and frequently performed. The management of regional pericarditis differs greatly from that of acute myocardial infarction. Case report: A 52 year-old male underwent atrial fibrillation ablation and developed severe mid-sternal chest pain the following day with electrocardiographic findings suggestive of acute myocardial infarction, and underwent coronary angiography, a left ventriculogram, and 2D transthoracic echocardiogram, all of which were unremarkable without evidence of obstructive coronary disease, wall motion abnormalities, or pericardial effusions. Ultimately, the patient was diagnosed with regional pericarditis. After diagnosis, the patient's presenting symptoms resolved with treatment including nonsteroidal anti-inflammatory agents and colchicine. Conclusion: This is the first reported case study of regional pericarditis status post cardiac ablation. Electrocardiographic findings were classic for an acute myocardial infarction; however, coronary angiography and left ventriculogram demonstrated no acute coronary occlusion or ventricular wall motion abnormalities. Healthcare professionals must remember that the electrocardiographic findings in pericarditis are not always classic and that pericarditis can occur status post cardiac ablation.

Context: Hemophagocytic lymphohistiocytosis (HLH), due to the excessive activity of histiocytes and lymphocytes, is a rare but aggressive disease that typically occurs in infancy but can be seen in all ages. If left untreated, patients with HLH may live for only a few months and die from multi-organ failure. Case Report: We present two cases of HLH diagnosis. Fever, spleen, and hepatic abnormalities were noted in both cases. Conclusion: Early diagnosis is the key and these two cases of similar etiology highlight how fever of unknown origin should force us to broaden our differential.

Context: There are no established guidelines for the proper treatment of patients with bronchopleural fistulas (BPFs). Apart from attempts to close the fistula, emphasis of treatment and management is placed on preventive measures, early administration of antibiotics, drainage of the empyema and aggressive nutritional and rehabilitative support. Case Report: A 53-year-old male presented with nausea, vomiting, and dry cough with eventual respiratory failure. He was found to have an empyema of the left hemithorax which was managed with thoracostomy drainage and antibiotics. However, he had persistent air leak through the chest tube due to a BPF. Bronchoscopy failed to localize the involved segment. Application of fibrin glue through the chest tube succeeded in completely sealing the leak. Conclusion: To our knowledge, this is the first case report in which fibrin glue was successfully used intrapleurally to close a BPF related to an empyema.