Cystic fibrosis (CF) is a hereditary syndrome composed of exocrine gland dysfunction involving multiple systems which if untreated may result in chronic respiratory infections, pancreatic enzyme deficiency and failure to thrive. The association between CF and other inherited diseases or congenital anomalies is rare. We describe a rare case of CF with concomitant congenital adrenal hyperplasia (CAH). 21- Hydroxylase deficiency accounts for most CAH cases. Varity in clinical phenotypes depends on the amount of enzymatic activity which in turn depends on different combination of gene mutations. The genes of CAH and CF are located in different locations. The chance of these diseases coexisting in our patient would be a rare combination. However, such a case will be more frequent in our population than others because of consanguineous marriage and common ancestors. There are diagnostic difficulties, similarities and contradictions between two diseases and they are pointed out.
Objectives: There are variations in therapeutic regimens of different liver diseases. The accurate diagnosis ensures prompt recovery from these diseases. The present study aimed to evaluate the underlying causes of unexplained signs and symptoms associated with liver diseases through biopsies.
Methods: A retrospective study was conducted in a public child care specialty of Lahore, Pakistan. The data was collected from medical records of the patients who were index hospitalized with unexplained clinical presentation of liver disease between 1st July, 2017 and 31st December, 2017. Data were analyzed by using Statistical Packages for Social Sciences (IBM SPSS Statistics for Windows, Version 21.0. Armonk, NY: IBM Corp.), and Microsoft Excel (MS Office 2010).
Results: Overall, the records of 53 patients were selected for the study. Most of them were 11 to 15 years of age. The patients were presented with unexplained hepatomegaly (60.4%) and jaundice (40.7%) during index hospitalization which made them eligible for liver biopsy (LB). The findings of LB revealed that the underlying causes of liver diseases in most of the cases were metabolic (33.9%) and inflammatory disorders (22.6%). Majority of the patients were ≤4 years of age, however cryptogenic cirrhosis (39.1%) was commonly found in >10 years of age. Although most of the patients were suffering from metabolic disorders (p-value=0.07) and liver cirrhosis (p-value=0.08) but these were not statistically significant.
Conclusions: LB was beneficial in evaluating the etiologies of unexplained signs and symptoms of liver diseases. It was found that glycogen storage diseases and liver cirrhosis were the most common etiologies of liver diseases among pediatric patients. But etiologies like metabolic and inflammatory diseases were insignificantly associated with gender.
Mark Taubert, Lorenz Weidhase, Sirak Petros and Henrik Rueffert*
Published on: 17th October, 2018
A 64-year-old woman was referred to our hospital due to progressive dypnoea for the past week, combined with fever and type 1 respiratory failure. White blood cell count and procalcitonin level were normal. The Chest X-ray showed bilateral disseminated pulmonary infiltrates. Within the next 24 hours the patient developed a severe ARDS. A first diagnostic work-up for typical and atypical pathogens as well as serological tests for CMV, RSV, HIV and HSV were negative. Analysis of a second bronchoalveolar lavage fluid revealed Pneumocystis jiroveci DNA. The patient was successfully treated with trimethoprim-sulfamethoxazole and off label use with caspofungin. The cause of the infection was a six week treatment with dexamethasone. The patient developed a toxic epidermal necrolysis during further course, but completely recovered.
Pneumonia with Pneumocystis jirovecii must also be taken into account in non-HIV patients, whenever there are any indications that cellular immunity may be depressed.
The Ontario government has been battling with the issue of extensive wait times in hospitals for several years. Although there are many complex reasons that stem off of the issues of wait times, such as available in patient beds and bed blockers (patients who stay in the hospitals for long periods of time after sustaining a massive injury), the primary cause is concentrated as a system wide problem in access to care [1]. Through numerous reforming and restructuring plans, the Ontario government devised a Wait Time Strategy plan to monitor, manage and improve access to health care services including surgeries and time spent in the ER. The strategy was also devised to enhance the efficiency and effectiveness of healthcare provision. The information derived from the results of the Ontario Wait Time Strategy (OWTS) was to be made public to citizens and providers to ensure that everyone is well aware of the results. Yet, it is quite difficult to implement such a strategy if the leadership challenges within the hospital are not addressed [2].
Introduction: Hypertensive crisis (HC) is recognized consequence of inadequate blood pressure (BP) control. A hypertensive crisis is further divided into hypertensive emergency (HT-E) and hypertensive urgency (HT-U).
Method: Using a cross-sectional hospital-based study design, patients who had been diagnosed as having HC between January and October 2017 were consecutively recruited in the study. The criteria proposed by the Seventh Joint National Committee were used for the definition of HC.
Result: A total of 81 (.81%) patients newly diagnosed as having HC were enrolled in the study. Of these patients, 50 (61.7 %) patients met criteria for HT-E, while 31 (38.3%) patients had HT-U. Renal impairment (16%), stroke (30.8%), acute coronary syndrome (13.6%) and heart failure (22.2%) were predominant complications associated with HT-E. Out of 81 study subjects, 13 (16%) patients died. Although there was no significant difference in residence, history of smoking, Diabetes mellitus and history of alcohol consumption between groups, old age (P=.o22), male gender(.046), history of hypertension(.007), history of non-governmental employee(.003), poor compliance (p=.002) and high case fatality rate (p=.041) were significantly associated with hypertensive emergency (HT-E).
Conclusion: This study showed that HT-E has high case fatality rate among patients admitted with hypertensive crisis at kassala teaching Hospital. Therefore early detection of hypertension and appropriate management are the main stay for reducing morbidity and mortality among patients with hypertensive crisis.
The objective of this study is to determine prevalence of alcohol-substance use among university students, and to investigate the correlation between the childhood abuse, suicide probability and anger expression styles in students who have drinking problems. A survey was carried out among randomly selected students from the Faculty of Education in Baskent University in Turkey. Study sample consists of 399 university students. Childhood Trauma Questionnaire (CTQ), Trait Anger and Anger Expressions Scales (T-Anger-Anger EX), and Suicide Probability Scale (SPS) were used. The CAGE questionnaire was applied to identify the problems of alcohol use. Probable presence of an alcohol use disorder is indicated by a score of 1+, whereas a score of 2+ was taken as the cut-off point for assessing presence of clinically significant alcohol use problems. Data were analyzed using with t test and multiple binary logistic regression. Of the whole sample 36.9% reported that they had ever tried drinking alcohol. The overall prevalence of alcohol use problems according to CAGE 1+ was 14.4% and CAGE 2+ was 7.3%. Sexual abuse and Anger-In were predictors of CAGE 1+, suicide probability was predictor of CAGE2+. Childhood trauma experiences especially, sexual abuse, suicide probability, trait anger, the anger expressed inside and outside were main factors to identify alcohol use problems. Professionals and parents must pay attention to childhood traumatic experiences, suicide and anger expression styles in youths with alcohol use problems.
Background: Skin diseases is a common worldwide problem. It affected every aspect of patients’ quality of life (QOL) mainly physically, socially and psychologically.
Objectives: to assess the impact of skin disorders on patients’ quality of life and to identify factors associated with it.
Methodology: This cross-sectional study was conducted in outpatient dermatology clinic of a tertiary hospital in Malaysia. A random sample of 145 patients with acne, psoriasis and atopic dermatitis (AD) were interviewed using DLQI questionnaire during their scheduled follow-up appointments at dermatology clinic.
Main outcome measure: Self-reported patients’ QOL due to their skin diseases.
Results and discussion: Out of three skin diseases psoriasis patients had the highest prevalence (39.3%) followed by AD (34.5%) and acne (26.2%). Patients’ QOL was highly influenced by their skin conditions especially on working/schooling domain. Furthermore, several factors were identified, namely age, working environment, concurrent skin diseases, usage of supplement for skin diseases and type of food as aggravating factors—that may influence patients’ QOL. QOL among females and younger adults was found to be more significantly influenced as compared to males and elderly. With respect to working environment, those who had both indoor and outdoor working environment showed the highest impact of their skin conditions on their QOL. Single patients were more influenced by their skin conditions when compared to those who are married, however it was not significant.
Conclusion: Our findings revealed skin disease had negatively impacted individual QOL with different level of aspects. Among the three diseases, AD patients had the worst impact on QOL. Significant predictors of QOL did not relate solely to skin diseases but also other factors such as type of food and working environment.
Introduction: Risk factors for systemic reactions (SRs) from hymenoptera venom (HV) allergy are well known in the adult population but they have been little studied in the pediatric one.
Method: The aim of our study was to identify risk factors for SRs in a population of children allergic to HV, comparing a series of clinical (age, gender, atopy, asthma) and laboratory (total IgE, tryptase, venom-specific IgE levels) variables between patients with at least two large local reactions (LLRs) and patients with SRs of different severity for the identified insect. We selected a population of HV allergic children aged < 15 years with LLRs or SRs stratified according to Mueller grades after stinging.
Results: The population included 80 children, 35 with at least 2 LLRs and 45 with SRs. The level of specific IgE for vespid (Polistes dominula, Vespula species) venoms was significantly higher (p = 0.0321) in children with SRs (Mueller grade II+III+IV) than in those with LLRs and the same significance was also found for specific IgE for Apis mellifera, considering SRs group (Mueller grade I+II+III+IV) in respect with LLRs group (p = 0.0001).
Conclusion: The main difference in our pediatric population was the highest level of specific IgE in children with a history of SRs compared to those with a history of LLRs for both vespids and honey bees. These results, once confirmed on a larger population, could suggest the opportunity to follow the behavior of venom specific IgE in children with LLRs to reveal a risk to develop future more serious reactions.
Modern AP concepts are focused exclusively on the infectious nature of the disease and the presence of certain pathogens. This belief determines the principles of treatment, the lack of effectiveness of which remains a concern of health professionals. The article presents a fragment of the study devoted to the etiology of АP. 994 children aged 4 months to 14 years with various forms of so-called community-acquired pneumonia were examined and treated. Bacteriological examination of the material from the inflammation zone was carried out in 542 patients. Experiments on modeling АP and its pleural complications were performed on 44 animals. The obtained results and critical analysis of the literature data and scientific facts allow us to consider bacteria only as one of the etiological elements of АP, which is not mandatory in all cases of the disease. Scientifically based revision of existing ideas about the causes and mechanisms of AP development leads to the need for a radical change in the principles of treatment and is a strategic direction in solving the problem.
Introduction: DSM-5 mentions autoerotic asphyxia in the paraphilic disorders section, as a specifier for the diagnostic of sexual masochism disorder. Strangulation activities have also been observed in the “choking game”. The term “strangulation activity” is considered as more appropriate than “choking game”. While sharing a same behavior, autoerotic asphyxia and non auto-erotic strangulation activities might represent a very distinct pattern of disorders. We describe here a case report of a 25-year old male internship student who has practiced manual self-strangulation up to 40 times a day since adolescence. In the examination of this case we identify individual clinical aspects of this case in a process-based holistic case conceptualization.
Case description: The patient is a 25 years old male with a post-graduate degree who presented with a recent history of poor work performance and work-related stress during an internship. He has a concomitant history of both ketamine and cannabis use disorders, and reports urges to self-strangulate, sometimes specifically avoiding contact with friends engage in this behavior and that he has never attempted to discontinue self-strangulation. Neuropsychological assessment found a cognitive functioning below that expected given his educational level. Our intervention consists of a 3-weeks cognitive and motivational therapy program in addiction unit with associated abstinence.
Conclusion: Autoerotic asphyxia is a behavior observed not only in the context of sexual masochism disorder, but also as a specific addictive behavior, in the absence of sexual arousal, possibly as a result of emotional dysregulation.
Nuclear Medicine is an integral part of modern healthcare. The use of radioactive nuclides tagged biomolecules, evaluating their distribution in human bodies by SPECT or PET systems, provides longitudinal sets of volumetric and quantitative images that can be used to diagnose a wide range of disease and/or assess response to disease specific treatments [1].
Objectives: As the cancer patients are at higher risk of premature deaths due to candidemia. So, the present study aims to evaluate the predictors of candidemia along with its outcomes among hospitalized adults and pediatric cancer patients.
Methods: A retrospective study was conducted at a tertiary care cancer hospital in Lahore, Pakistan. The data was collected from the medical records of all the patients who were found positive for Candida species between 1st January 2017 and 31st June 2017. Data were analyzed by using Statistical Packages for Social Sciences (IBM SPSS Statistics for Windows, Version 21.0. Armonk, NY: IBM Corp.) and Microsoft Excel (MS Office 2010).
Results: Overall, 135 patients were detected with candidemia. Based on blood culture test results, it was found that out of 100 cultures positive for any microorganism there were 2 cases of candidemia. Multivariate analysis revealed that hematological malignancies (AOR: 2.1), and shock (AOR: 9.1) were significantly associated with high risk of mortalities during the index hospitalization, while risk of mortality among cancer patients suffering from Candida albican infection (AOR: 0.47) and those who were administered with antifungal agent after sensitivity report of the fungal culture (AOR: 0.2) was significantly less. Also, there was no significant association of empiric therapy of antifungal agent with the risk of mortality before a positive culture found (p>0.05).
Conclusion: Although, no risk factor was found to be associated significantly with candidemia among cancer patients. But hematological malignancies, non-albican candidemia and shock were predictors of higher risk mortality during index hospitalization.
Alcohol abuse is a global health problem. Alcohol withdrawal syndrome (AWS) ranges from mild to severe symptoms that can lead to fatal delirium tremens requiring ICU admission and incurring high health care cost as high as $20,000 a month. The latest published reports suggest that phenobarbital is a promising therapeutic option for management of AWS as evidenced by less ICU admissions, length of stay in hospital, use of adjunctive agents, health care costs and attention from the nursing staff than that of patients treated with commonly used benzodiazepines such as lorazepam, diazepam, and chlordiazepoxide. Phenobarbital is beneficial for the treatment of AWS, both in the emergency and inpatient settings and both as monotherapy or in conjunction with benzodiazepines. It is safe for patients without severe hepatic impairment, has a better mechanism of action and longer half-life than benzodiazepines, and leads to less delirium and agitation. Powered randomized controlled trials with large populations are required, yet phenobarbital can be used to safely to treat AWS.
The problem of the alcohol misuse in Russia is well known; but there is a tendency to exaggerate it, which seems to be used to disguise shortages of the healthcare and public assistance. In this way, responsibility for the relatively low life expectancy is shifted onto the patients, who are supposed to drink excessively. In parallel, there is a tendency to exaggerate successes of the anti-alcohol campaign (1985-1989) and to trivialize the harm caused by the campaign e.g. mass consumption of surrogates [1]. The bias can be illustrated with the example of papers by Yuri Razvodovsky [2-5]. Yuri asked me per e-mail to write a “friendly” letter to the Editor referring to his article. An excerpt from the correspondence is presented here as documentary evidence (Figure 1). In reply, I cited the phrase: “The official statistical data do not support the claims that the… аnti-alcohol campaign contributed to the dramatic growth in fatal poisonings by non-beverage alcohol surrogates” [4] and commented that I witnessed mass poisonings with window cleaner in 1988. Considering the large scale of the window cleaner sales, it was knowingly tolerated by authorities. Poisonings with methanol and organochlorides (used e.g. in dry cleaning) were known to occur as well. Yuri replied that “there are two realities: one, which we both witnessed, and another-official statistics” (Figure 1). Obviously, Razvodovsky should have discussed in his articles the “realities we both witnessed”, i.e. common knowledge and observations, otherwise his papers are misleading.
Stroke following coronary interventions is a devastating and most dreaded complication with signiβicant morbidity and mortality. Various factors have been ascribed for this complication including the technical errors [1]. A small percentage of strokes are iatrogenic, including those associated with invasive cardiac procedures. According to the literature, it is a rare complication of left heart catheterization [2]. Percutaneous coronary intervention is increasingly used to treat patients with diffuse atherosclerosis, acute coronary syndromes and even high-risk patients such as low ejection fraction [1]. The authors describe a patient who underwent percutaneous coronary intervention in the context of inferior infarction, which was complicated by ischemic stroke during cardic catheterization.
Background: There is a huge gap in the U.S. health care system when it comes to the prevention of substance abuse and addiction, specifically involving adolescents. It has been determined through literature review that parents in Utah are not informed about/knowledgeable of/aware of the current usage of alcohol/drugs in the adolescent population in this area or the inherent risks involved with consumption/use/misuse/abuse/addiction.
Aim: The overarching aim of the doctoral project was to prevent Utah County middle school students from harmful alcohol and drug consumption by involving parents in prevention efforts. The prevention goals of this project included: increased parental knowledge of the brain disease of addiction, warning signs of adolescent drug use, what to do if their child is already using, and increased parental self-efficacy on communicating with teens on this subject.
Design: For parents to be productively involved, barriers were broken down through education. The DNP student completed a 15-minute presentation at five separate parent meetings that took place annually during middle school registration at Lehi Jr High.
Population: The education was for parents, foster parents, guardians, etc. of first-year middle school students only; an estimated 420 participants were in attendance.
Data collection and analysis: A pre-and post- combined self-efficacy and knowledge test was given to the attendees. Data was gathered from 117 appropriately completed tests and analyzed using a paired t-test. The results were studied to evaluate effectiveness of the program relating to parental self-efficacy and knowledge.
Findings: The presentation ultimately increased parental self-efficacy and knowledge (p-value <0.01) which in turn increases protective factors and decreases risk factors associated with adolescent substance abuse.
Conclusions: The DNP project was successful, and the results assisted in the refinement of the presentation for sharing on a mass level to all Jr. High schools in Utah County, Utah.
Many pathologic disease can be considered as related to an Endogenous toxicological moves and in time dependent way (kinetics and dynamic of the process). In this work starting from the analysis of relevant literature involved with different disease and related to the endogenous local micro- environment some global conclusion useful as new tools for innovative pharmacological strategies will be submitted to the researcher. Physiology, pathology concept linked to the endogenous toxicological local micro-environment status as new research instruments. The same carcinogenesis process can be related also to endogenous agents that may have a major contribution in spontaneously process. (Reactive oxygen species (ROS), which are involved in multiple cellular processes by physiologically transporting signal as a second messenger or pathologically oxidizing DNA, lipids, and proteins).
Brain stem death is when a person has permanently lost the potential for consciousness and the capacity to breathe. When this happens, a machine called a ventilator keeps oxygen circulating through the person’s bloodstream - a person confirmed as being dead when their brain stem function is permanently lost [1].
A literature search was conducted using PubMed and PsycINFO to locate cyberbullying research that was published during the last 4 years. In this narrative review, cyberbullying research is briefly summarized and critiqued. The review is focused on the varying definitions and characteristics of cyberbullies, victims and bystanders. Highly variable prevalence rates have been reported for cyberbullies, victims and bystanders as a function of age, gender, country, size of the social network and socioeconomic factors. In addition, the effects of cyberbullying are reviewed including the frequent suicide attempts along with risk factors/predictors of cyberbullying which include previous cyberbullying, excessive internet use and lack of empathy, anger, narcissism and authoritarian/permissive parenting. To reflect the recent literature, special attention is given to the studies on victims of bullying. Research on cyberbullies and on prevention/intervention programs for bullying is extremely limited despite the increasing prevalence of bullying and the rapidly accumulating literature. Methodological limitations include the primary focus on the prevalence of bullying and on the victims of bullying. Longitudinal, multivariate studies are needed to identify profiles on risk factors for bullying that can inform prevention programs.
The ovarian serous Cystadenocarcinoma shared large number of deaths in gynecologic carcinoma. It has various numbers of molecular events from initiation to progression and at advance stage, surgery is the end product of such molecular signaling. We assess in this study the whole mechanistic view of TNFSF10 network which has the ideal apoptotic causing identity. We used fresh insilico strategy to uncover the secrets and inter-links from its protein-protein interaction complex. We retrieved the TNFSF10 signaling network from STRING database (www.string-db.org). The network contains 25 nodes and 152 edges with clustering presentation. After retrieval, we performed gene enrichment and characterization analysis of network from WebGestalt toolkit (www.webgestalt.com). Finally, we examined the participation of whole network in ovarian cancer progression from cBioPortal, a cancer genomic data portal (www.cbioportal.org). Our results showed that majority of cases have loss of function of death receptors (DR4 and DR5) that are the main unit of initiation of apoptotic signaling. Most of downstream signaling members showed amplification that regulates cell proliferative pathways including NFkB pathway. TNFSF10 cluster has loss of function and in future it gain attention for further research studies to discover its interactome level view for valuable therapy. FAS cluster has large number of members and majority showed amplification rendering them as co-targets for combinational drug designing.
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