Cloud Computing Research Paper Example | Topics and Well Written Essays - 2500 words - 2

Cloud Computing - Research Paper Example For instance, information of an organization needs to be protected and is the ultimate responsibility of the board of director for which they are also liable. We have selected a small medium enterprise called as ‘Wizni Incorporated’ this is transformed from a traditional IT service to a cloud service provider. The Information technology architecture of the company incorporates three components i.e. business analytics expertise, along with the provision of a quality cloud hosting provider. Likewise, the core competency of Wizni Inc. is business analytics. Moreover, the company is delivering business analytics bundled with powerful systems enabling their clients to make efficient decision making. Wizni Incorporated provides state of the art analytics solutions for empowering organizations to utilize business intelligence required for making efficient decision making. The company provides Business intelligence deployments that facilitate ease of discovery, patterns hidden i n IT systems, applications and unstructured data. The business analytics products are DIFEEâ„ ¢ (data-inference and federation engine) and Score IT. DIFEE is used for virtualization and Score IT is used for data mining, model scoring and life cycle management. Furthermore, IBM Cognos Express is also a first integrated business intelligence tool that is precisely developed and acquired by the company to meet customer requirements. The tool is bundled with critical reporting, scorecard, analysis, budgeting, forecasting dashboard and customization features and capabilities for the small medium enterprises at an affordable cost without large associated investments. Moreover, the tool incorporates a pre-configured solution that is very easy for installation and use for the end users. The transition of this new powerful cloud solution is approved by the board on the basis of gaining competitive edge among small medium enterprises within the region. Previously, there was no growth in cl ientele and after implementing the cloud based solution, clientele sky rocketed and stakeholders were satisfied at the same time ready to invest in the technological solution. On the other hand, cloud computing also reflects significant risks, principal risks of cloud computing is associated with federal agencies and regulations. Any vulnerability found in the software, platform or infrastructure of the vendors will expose serious exposures, as information may be related to more than one organization. Moreover, employees working internally on the cloud computing premises of ‘Business Cloud’ may also expose a serious threat if no proper background employee checks were performed during recruitment procedures. Furthermore, if any governmental agencies for instance, military or other sensitive body is also using the same cloud from the same vendor is also most likely to be compromised, if any breach of any one of these fundamental concepts Confidentiality, Integrity and Ava ilability is successful. In addition, the incident response function may not be efficient and effective if any incident occurs and affect the customer or employee. For instance, if a security incident affects the customer, the incident response function of the organization will trigger in a timely manner to isolate and investigate the root cause via root cause analysis. In a cloud computing scenario, it may be a different case and result

Analysis of the Chinese films Essay Example | Topics and Well Written Essays - 1000 words

Analysis of the Chinese films - Essay Example As pointed out, the protagonist was forced to be the custodian of Kosaburo Hanaya and Dong Hanchen. Before this incident, Ma Dasan’s life was flowing slowly without much problem. But this incident forced Ma Dasan to be in a dilemmatic situation because the person who handed over the prisoners pointed out that he must look after the prisoners with due care. The following events include the futile effort undertaken by Ma Dasan to keep the prisoners safe, hiring of One Stroke Liu to execute the prisoners and failure to do so, Kosaburo Hanaya’s return to his homeland, and the fire in Ma Dasan’s village, Ma Dasan’s attempt to take revenge and his ultimate doom. The most shocking incident in the film is the large scale massacre at the feast. This incident ignited the feeling of revenge in Ma Dasan’s mind. Earlier, Ma Dasan’s role in the village (say, his community or society) was to obey the law and order without showing his hesitation. But this in cident transformed him from a passive individual to an active individual who is ready to give up his life for a noble purpose. A bird’s eye view of the film leads one to misinterpret the same as an anti-Japanese invasion genre film. But deeper understanding leads one to the realization that the director’s attempt is to mock the hypocrisy of the Chinese authorities and the mentality of the Chinese villagers towards the Japanese invaders. For instance, in the film, children are ready to share secrets with the invaders and the villagers feel satisfied by the supply of food grains. Besides, this unveils the passive attitude shown by the people towards the authorities. The fundamental problem of violence in the film faced by the ignorant villagers without a unified authority is two sided. For... The contemporary Chinese cinema is undergoing rapid but positive change. Nowadays, the Chinese film makers show keen interest to inculcate innovative ideas in their films. For instance, the film Devil at the Doorstep by the Chinese actor cum director Jiang Wen is one of the best examples which highlight the foretold change. Jiang Wen’s prior status as an actor did not hinder him from choosing the story line of the film Devil at the Doorstep, which carries violence and mental trauma faced by individuals in a closed society. The film was able to gain the jury prize at Cannes, but that did not help Jiang Wen as a direThe film Devil at the Doorstep by Jiang Wen was accepted by the international viewers but less accepted by the Chinese government. The main problem faced by Jiang Wen was the restriction upon individual freedom of a director with the international outlook. The main reason behind the less acceptance and ban on the film was the realistic and critical attitude of the di rector towards power politics, violence and its effect upon the individuals. The protagonist’s transformation from an inactive stage to activism and as the mouthpiece of the director to communicate with the viewers is the most important development in the film. The narrative feature of the film is conversational and less ambiguous because of the usage of the historical plot. The stylistic features of the film raise the same from its limitations to the international arena of cinematography.

Electronic financial transactions over the Internet Essay

Electronic financial transactions over the Internet - Essay Example Technological innovation in terms of developing security software aided this process and so did the process of globalization. As a culmination of these parallel but complementary processes, e-commerce in general and electronic financial transactions in particular has firmly taken root in mainstream global economy. While this kind of progress in such a short span of time is an impressive achievement, there is much work to be done in terms of creating robust security systems for the Internet. This essay will look into the various types of security threats facing electronic financial transactions, the potential damage they could inflict, the remedial measures to counter these threats and the effectiveness of such measures. The architecture of the Internet is such that it is inherently difficult to ensure security. Alongside this, the concept of user anonymity makes the Internet â€Å"an attractive medium for extortion and crimes involving theft of personal information for illicit financial gain† (Misra Scherer et al, 2004). As per a report released by IDG News Service, many organized crime groups avail of the services offered by hackers to implement complex and sophisticated financial schemes to swindle money from unsuspecting participants. According to the Internet Crime Complaint Center, as much as $200 million was lost in the year 2006 alone. And each year this sum seems to increase, since financial institutions like banks and insurance companies are lucrative targets for cybercriminals. A FBI report released at the beginning of the new millennia has confirmed the nexus between computer hackers and organized crime groups. Many of these organized crime groups are said to operate from Eastern European countries that were part of the erstwhile Soviet Union. In such cases, the hackers first crack the security systems in place and gain access to vital data. The organized

Why Should We Forgive Others Essay Example | Topics and Well Written Essays - 500 words

Why Should We Forgive Others - Essay Example In Christianity which is a western religion, teaching about forgiveness takes a very central role in the spiritual life of the Christian adherent. The teaching is that, if we expect God to forgive us, we must in turn forgive our fellow man when they wrong us. This is best expressed in what is known as the Lord’s Prayer in Mathew 6:9-13 where the Christian petitions God to forgive him just as they forgive others. This is as a precondition that if we expect God to forgive us, we have a part to play in forgiving others as it is narrated in Mathew 6:15. We also find the example of a Christian who practiced forgiveness during the stoning of Stephen in Acts 7:60 where Stephen prayed and said ‘lay not this sin to their charge’, words almost similar to the ones uttered by Jesus Christ at His crucifixion, ‘forgive them for they know not what they do’, speaking about His crucifiers, yet He had committed no sin, Luke 23:34. This shows that a Christian who forgiv es is following the example of the author of Christianity, Jesus Christ. The Bible also teaches about turning your other cheek and giving your coat as well to someone who takes your cloak as it continues to express the virtue of forgiveness, Luke 6: 27-31. The Bible also talks about God’s demonstration of love in that Jesus Christ died for the sins of the world while man was still a sinner (Bash, 2007).

Electronic financial transactions over the Internet Essay

Electronic financial transactions over the Internet - Essay Example Technological innovation in terms of developing security software aided this process and so did the process of globalization. As a culmination of these parallel but complementary processes, e-commerce in general and electronic financial transactions in particular has firmly taken root in mainstream global economy. While this kind of progress in such a short span of time is an impressive achievement, there is much work to be done in terms of creating robust security systems for the Internet. This essay will look into the various types of security threats facing electronic financial transactions, the potential damage they could inflict, the remedial measures to counter these threats and the effectiveness of such measures. The architecture of the Internet is such that it is inherently difficult to ensure security. Alongside this, the concept of user anonymity makes the Internet â€Å"an attractive medium for extortion and crimes involving theft of personal information for illicit financial gain† (Misra Scherer et al, 2004). As per a report released by IDG News Service, many organized crime groups avail of the services offered by hackers to implement complex and sophisticated financial schemes to swindle money from unsuspecting participants. According to the Internet Crime Complaint Center, as much as $200 million was lost in the year 2006 alone. And each year this sum seems to increase, since financial institutions like banks and insurance companies are lucrative targets for cybercriminals. A FBI report released at the beginning of the new millennia has confirmed the nexus between computer hackers and organized crime groups. Many of these organized crime groups are said to operate from Eastern European countries that were part of the erstwhile Soviet Union. In such cases, the hackers first crack the security systems in place and gain access to vital data. The organized

The Great War career of Field Marshal Douglas Haig Essay Example for Free

The Great War career of Field Marshal Douglas Haig Essay Considered assessment of the Great War career of Field Marshal Douglas Haig. Douglas Haig was appointed as commander of the BEFs 1st Army Corps at the outbreak of World War One in 1914 with Sir John French as Commander in Chief of the British Army. By the end of 1915 it became apparent that Sir John French was ill-suited to the role and Douglas Haig replaced him as Commander-in-Chief. Haig became one of the most controversial figures in military history with tag-lines such as the butcher of the Somme and an incompetent leader being the most associated with him. His tough and decisive leadership style with apparent little compassion to the huge amounts of British deaths during World War One made him one of the most debated person in history with varying views of his leadership style. It is widely believed that Haig was unwilling to accept new ideas but stick to his traditional, military experience with reluctance to hear new ideas and recommendations. Major Desmond Morton who served as one of Haigs adjutants said He (Haig) hated being told any new information, however irrefutable, which militated against his preconceived ideas or beliefs. This reliable source that gives an insight to the leadership of Haig goes on to say that John Charteris was being a sycophant to Haig and although he was anincredibly bad head of intelligence, Haig favoured him because he was conservative of the truth and always concealed bad news, or put it in an agreeable light. This is backed up by General Sir James Marshall-Cornwall who said that One of the faults of Haigs nature was that he trusted too completely some of his immediate subordinates. This is supported by the History Learning Site who says that Haig had little time for new military ideas and was steeped in the ways that he knew-conventional tactics. His history as a cavalry commander enforces this quote of Haig sticking to what he knew best and an inability to listen to new ideas or react to a changing situation-essential characteristics of a cavalry commander. Further criticism to Haigs inability to listen to new ideas is given by Liddell Hart when he states that Haig failed in his poor receptivity of ideas. However, some people hold the view that Haig and the other generals in The Great War were receptive to new ideas and did change tactics. The BBC History site says that it is not true, as some think, that British Generals and troops simply stared uncomprehendingly at the barbed wire and trenches, in reality, the Western Front was a hotbed of innovation as the British and their allies and enemies experimented with new approaches. Whilst not directly talking about Haig, this does imply that although Haig may not have been the most experimental leader, despite this view it did not impact on the experimenting of new ideas that took place within the Army. Mike Hone would agree with this evaluation of Haig when he wrote the fact is that British tactics developed considerably during the war. The disastrous first day of the Battle of the Somme resulted in huge amounts of analysis and blame put on the event with mixed interpretations. With 19,240 soldiers being killed on the first day alone it was one of the most costly battles in the history of warfare. The planning and conducting of the battle of Somme by Field Marshal Haig has also been subjected to criticism and evaluation. As Commander in Chief of the British Army, Haig is responsible for the welfare and safety of all British Soldiers and this has primarily led to the vast criticism of Haig regardless of Haigs direct actions. A Battle of the Somme timeline compiled by gommecourt.co.uk says that on the 23rd January 1916 whilst preparing for the preliminary attacks on a 20,000 yard front on the Somme to commence on 20th April, General Joseph Jaques Cesaire Joffre, Commander in Chief of the French Army suggested to Haig wearing down attacks prior to the main joint offensive starting on 20th April and the other in May. However, the source goes on to say that Haig rejects the plan. Whilst this may not be an extremely surprising quote it does present a worrying situation. Dennis Wheatley who served during the Great War wrote that He (Haig) had a rooted dislike of the French and was not even a second rate general. It presents an extremely worrying possibility that Haigs personal feelings and attitudes could have led to poor decisions and the loss of many innocent lives. The rejection of the French plan is also a further example of both his decisive leadership and his inability to listen to advice and recommendations. This also shows that Haigs planning of attacks are of an extremely dictatorial nature with a strong sense that his traditions of leadership should be withheld regardless of any interventions. Later on in this timeline, Haig replies to Joffre again on 10th April 1916 to again reject another of his ideas and on the same day, Haig received a revised plan from Rawlinson suggesting a long artillery perpetration rather than Haigs preferred hurricane bombardment. Communication during Haigs planning of the Battle of the Somme has also been under scrutiny after GHQ writes to Rawlinson that it was not clear whether his attack or that of the Second Army at Messines would start first . Both communications and Haigs decisions were disputed during the timeline of events, creating a picture of a dictatorial, private ruler who wanted to plan the British attacks by himself, using traditional methods and without any assistance, information or ideas. PW Turner and RH Haigh wrote that the planning of the Somme campaign was ham-fisted and clumsy. The fault for the failure of most of the strategic planning must fall on Haig. They hold the view that the failure in planning for the Somme was not due to communications or incorrect decisions but of national and personal pride and that Haig and his generals must have some spectacular victory to prove how right they were. The historians conclude that Haig promised victory and failed. This account holds the view that Haig was fulfilling his role of winning the war. He was a traditional leader in the sense that he was given an order (to win the war) and he was to complete that task at whatever cost. Martin Gilbert gave a somewhat more favourable view to the plan that Haig drew up. Gilbert believed that Haig made a logical plan to wear out the enemy and exhaust his reserves and then prepare for a decisive attack made with the objective of piercing the enemy lines. Gilbert the goes on to explain how Haig elaborated and made it extremely clear that it was to be a decisive account similar to his leadership. Haigs plan went on to describe that once the Germans had been worn down and used up their reserves-but not until then-a mass of troops would be thrown in at some points where the Enemy has show himself to be weak with the definite objective to break through and win victory . Gilberts view of Haigs planning is of an optimistic plan by Haig with clear and logical objectives. Norman Stone agrees with Gilbert that Haigs plan was logical but points out that Haigs information and intelligence from the Somme was flawed. Stone explains how Haig still imagined that the German line could be breached and cavalry could pour through the gap, but it could have been poured more effectively elsewhere. Stone simply explains that the solidification of the German line in 1914 along ridges allowed their guns a greater advantage and gave them the benefit of earth less likely to turn into mud. Stone concludes that the most Haig could do would be to take those ridges. Although the Brittish war industry was rapidly expanding to capabilities able to make thousands of guns and millions of shells able to launch a bombardment Haig did not trust his mens capacity, and Hereford relied on crushing bombardment. Stone points out that he believed this was probably the error in the planning of the Somme. After the catastrophic first day of the battle, questions were being asked about why to continue with the battle, why should Haig risk another 20,000 British lives? Martin Gilbert says that the Germans knew that the British would not give up. It was part of the British spirit and would not honour the 20,000 already killed to simply give up. It also didnt comply with Haigs determination to fulfil his task of winning the war. Questions regarding the planning of the battle also arose- why was the wire not cut? Why were the Germans still alive after such heavy bombardment? Was it an British failure of a German success and who should ultimately be blamed for the deaths of so many innocent soldiers? Some people like Desmond Morton believe that figures such as John Carteris who was head of intelligence was incredibly bad and sycophant nature of his relationship with Haig led to incorrect predictions that formed Haigs plans. The overestimated results of the British bombardment by British generals is extremely clear by Martin Gilberts description of what British soldiers had to carry and what they were expecting. They carried a rifle with fixed bayonet, between 170 and 220 rounds of small arms, two grenades, a waterproof cape(although it was a beautiful summers day), two sandbags, a steel helmet, two gas helmets, a pair of google against tear gas, a first aid field dressing and iodine, waterproof groundsheet, filled water bottle, haversack, mess tin, towel, shaving kit, extra socks, message book, uneaten rations, extra cheese, one preserved and one iron ration. In addition 40% would carry shovels and 10% would carry picks and one battalion was given a tin of grey paint each. This resulted in about sixty-six pounds of equipment. Historian General Edmonds wrote the weight of this equipment made it difficult t get out of a trench, impossible to move much quicker than a slow walk or to rise and lie down quickly Historian Peter Liddle agrees with this conclusion adding thousands of men offering so bulky and slow-moving a target would crumple to the ground quickly enough but would not rise at all, never mind quickly In addition to this, a planned stun tactic was used to explode mines in front of German trenches two minutes before the assault but this resulted in craters being formed allowing the Germans to occupy these craters, install machine guns and deliver devastating fire upon the British Army. The overestimation of the success of the bombardment by Haig resulted in the false expectations of British soldiers to be able to simply walk across no-mans land and create the beginning of the end of the Great War. This is evident in Martin Gilberts long list of issued equipment-40% of men carried a shovel obviously for digging trenches, 2 sandbags each to protect their trenches, rations and groundsheets to be able to stop overnight during their long advance. If the British generals had correctly estimated the effects if the bombardment, British soldiers would not have gone over the top and 20,000 lives could have been spared. Personally, I believe the initial failure of the battle of the Somme was down to the leadership and intelligence of the British generals. Soldiers trusted them for the correct information that would lead to the overall success of the battle-in reality false predictions led to the slaughter of thousands of innocent lives. Haigs continuation of the battle led to the monumental and historical introduction of the tank and the eventual victory over the Germans. The planning of the offence in Passchendaele was viewed by Stone to have made sense that Haig wanted to advance in Flanders. Stone explains that the German position was strong with height, the Messines ridge and could fire at Ypres from the side. It also allowed the British to deal with the submarine base at Zeebrugge. Stone believed that the British army was very strong with millions of shells and considerable experience with the kind of bombardment that might loosen the defence. The problem of the water table at Passchendaele resulted in near certain considerable amounts of mud whenever it rained or was churned up by shell. Although eventual success occurred following the explosion of the Messines ridge on 7th June the initial success lured the generals into disaster Disaster arose when Haig threw away the advantage. Stone says that there was an extraordinary interval before the next British attack.during which the German defences were strengthened and allowed the Germans to install pill boxes in which heavy machine-guns were placed. Therefore, it becomes clear that the initial planning of Passchendaele was extensive and proved a huge success but the resulting actions from Haig led to a catastrophic German rebound as a direct consequence of the leadership of Haig. The 21st March 1918 saw a large German bombardment starting at 4:40 am and lasted until 9:40 pm. It resulted in a million shells being fired and a British retreat over the old Somme battlefield to the French town of Amiens. Later in the year when German reserves were disrupted, Norman Stone describes how Rawlinson, Monash and Currie had to persuade Haig to not persist with the attack beyond a few days. J Rickard wrote that during the planning of the Battle of Amiens, Haig had directed General Rawlinson, to prepare for an attack on the salient. He goes on to explain that Rawlinson developed a plan fro a tank battle. Rawlinson had a multi-national army with American, Australian, Canadian and British divisions. Interestingly, Haig was also given control of the French First Army. However, Haig launched a second attack further nothing, using the Third Army. The purpose of this attack known as the battle of Bapaume, was to force the Germans back to the line of the somme. This attack began n 21 August.the British advance forced the Germans to retreat to the Somme. The battle of Amiens gives an example of times when Haigs leadership proved to be successful. Although Haig used the same methods of leadership as he did at the Somme and Passchendaele, the decisive, stubborn approach was needed here to drive the Germans back at a time when the German defence was at its weakest, the perfect cond itions for the leadership of Haig. The National Archives describe how the final German assault which started in the Spring of 1918 very nearly succeeded. The final German assault, which culminated in the Spring of 1918, very nearly succeeded. American forces were vital in holding the line but it was the British who took the lions share of territory and prisoners, no doubt in part thanks to Haigs still inspiring leadership. However, questions have now been asked as to whether Haig nearly settled for a compromise with the Germans. Nick Allen wrote for the Daily Telegraph thatHaig didnt realise how weak German forces were towards the end and wanted to settle for a compromise, according to Dr J P Harris, senior lecturer in War Studies at the Royal Military Academy Sandhurst. Dr Harris said: He wanted to offer the Germans very, very, easy ceasefire terms in late 1918.That could have left Germany with its armed forces, including its artillery, and its territorial gains in Eastern Europe intact. The end of World War One on 11th November 1818 was a result of many factors that all came together. The arrival of fresh troops from America in the summer of 1918 gave the allied forces a very large advantage. History on the Net explains how The German commander Erich Ludendorff (right) was a brilliant military commander and had won decisive victories over Russia in 1917 that led to the Russian withdrawal from the war.In 1918 he announced that if Germany was to win the war then the allies had to be defeated on the Western Front before the arrival of American troops. The British Naval Blockade led to food shortages in Germany and subsequent protests on the streets of Berlin. October 1918 saw the resignation of German commander Ludendorff and a naval mutiny. Kaiser Wilhelm II then abdicated on November 8th 1918 and an armistice was signed on November 11th 1918. The controversial leadership of Field Marshal Haig throughout the Great War is subject to so many different views and opinions by different historians, making an informed assessment on his leadership is extremely challenging. However, I do believe that the infamous catastrophic first day of the Somme was down to poor intelligence, predictions and overestimation. Men were sent to their deaths in appalling conditions whilst I do not think that Haig was solely to blame, I do believe that his distinct leadership style was not suited to the planning of the Somme and as commander in chief he does have overall responsibility for the safety and wellbeing of all men in the Army. However, his leadership style fitted the circumstances of the final year of the great war. 1918 saw circumstances requiring decisive, quick and dictatorial actions at a time when German defences were weak and rapid advancements needed to be made. Haig played an incredibly important role in the final year which ultimately led to allied victory along with the help of the fresh American soldiers. In Conclusion, I believe that Field Marshal Haig had an incredibly unique leadership style that only fitted the final phases of the war. The inability to listen to new ideas and dictatorial style during the Battles of Somme and Passchendaele I believe led to the deaths of tens of thousands of innocent soldiers. Poor intelligence also contributed to the immense disaster of the first day of the Somme. Whilst Haig has a duty to take responsibility forthe deaths of British soldiers I certainly believe that a number of factors contribute to both the successes and failures of the Great War and no single person or factor can take overall responsibility.

Case study of cyanotic congenital heart disease

Case study of cyanotic congenital heart disease Baby J, a 3-week-old infant, was admitted to Ward 5A since birth due to severe central cyanosis caused by several congenital heart problems. Soon after birth, he suffered from respiratory distress, where his initial SaO2 was only about 70%. He was resuscitated and given 5 nanograms/kg/min of Prostin (Prostaglandin E2). On appearance, he was dusky-looking and his peripheries were cold and cyanosed. He was started on biphasic continuous positive airway pressure (CPAP) via an apnoea mask and also given positive end-expiratory pressure (PEEP) as an adjunct. His CPAP was delivered using nasal cannula the following day after his SaO2 increased to 80% and he remained on CPAP for the first 5 days after birth, which subsequently was weaned off. Antenatal scans found pulmonary atresia, overriding aorta and ventricular septal defect (VSD). Postnatally, cardiac catheterisation confirmed the antenatal findings with extra major aortopulmonary collateral arteries (MAPCAs). He tolerated feeding via TPN and was given 6ml of EBM (expressed breast milk) at intervals of 2 hourly. Bottle feeding was attempted on 28/09/09 and he tolerated the feedings well. Recently, the feedings increased to 44ml 2 hourly. Baby J passed urine normally and his stools were of normal consistency. Past Medical and Surgical History Baby J underwent a cardiac surgery on 1/10/09 through a median sternotomy and a Melbourne shunt was inserted to create an aortopulmonary connection. He was also diagnosed with Alagilles syndrome (an autosomal dominant inherited disorder affecting the heart, liver, spine, kidney and central nervous system). Family History Baby Js father was diagnosed with Alagilles syndrome as a child. He has congenital spinal abnormalities, cardiac defects (atrial septal defect, ASD, pulmonary stenosis and right ventricular dysfunction). Baby Js mother suffered from depression for about 3 years now. She also has asthma and a high BMI of 40. During pregnancy, Baby Js mother was on anti-depressant (Fluoxetine 20 mg OD) and inhalers (Becctide and Ventolin). Drug History NKDA Medication Dosage Frequency Aspirin 15mg OD Frusemide 3mg BD Spironolactone 3mg BD Social History Baby J is the 3rd child in the family. He has one brother and 2 sisters. His brother was also diagnosed with Alagilles and suffers from cardiac anomaly. Baby Js mother is a non-smoker and she did not drink alcohol while conceiving him. Systemic Enquiry Neurological None to note. Cardiovascular See above. Respiratory Cyanotic. Gastrointestinal None to note. Opened bowel normally with normal stools. Genitourinary None to note. Passed urine normally. Haematological None to note. No fevers or rigors Musculoskeletal None to note. Endocrine None to note PHYSICAL EXAMINATION General Inspection Not distressed or in pain. Alert and non-lethargic. Apyrexial (Temperature 37.1 0C) Mild bluish discolouration of lips and tongue. SaO2 83% on room air. Heart rate: 156 bpm RR: 48/min Anterior fontanelle normal. Cardiovascular Examination Examination Findings BP Pulse rate 78/ 45 mmHg 156 bpm (regular, normal volume, character) No radial-radial delay or radio-femoral delay. Normal radial/ brachial/ femoral pulses. Normal carotid pulses. Inspection Hands and nails Face and tongue Precordium Ankle Non-cyanotic fingers. CRT No finger clubbing/ splinter haemorrhages. Non-pallor palmar creases. Deep-set eyes, prominent forehead (features of Alagilles syndrome) Non-pallor conjunctiva Mild central cyanosis (bluish tinge to tongue) Median stenotomy scar. No peripheral oedema (rarely seen in children) Palpation Central trachea. No thrills or left parasternal heave. Auscultation HS=I+Single II (muscular pulmonary atresia) + Ejection click (due to high flow across aortic valve) and continuous murmur. Respiratory Examination Examination Findings Inspection Hands Mouth Eyes Chest wall Not breathless or in distress. Breathing at ease. No peripheral cyanosis. No finger clubbing. Mild bluish tinge to tongue. No jaundice and non-pallor conjunctiva. Normal AP diameter. Symmetrical movement of chest wall with respiration No accessory muscles used in respiration. Trachea Central, no tracheal tug Percussion Generally, resonant to percussion. Auscultation Vesicular breath sounds. Normal air entry. No wheeze or added sounds. Summary of Problems Baby J suffered from severe cyanosis immediately post-delivery due to closure of ductus arteriosus. To maintain the patency of the duct, he was given prostaglandin E2. However, this was just a temporary measure to maintain a duct-dependent pulmonary circulation. A more definitive treatment for Baby J was to establish a direct connection between the aorta and the pulmonary artery by a shunt in order to promote growth of central pulmonary artery. Differential Diagnosis *Most likely differential for Baby J bolded. Differentials of cyanosis:- Primary pulmonary disease Cyanotic congenital heart disease Reduced or duct-dependent pulmonary circulation Tetralogy of Fallot Pulmonary atresia Tricuspid atresia Abnormal mixing Transposition of great arteries Total anomalous pulmonary venous drainage (all draining into right atrium) Single truncus arteriosus Persistent pulmonary hypertension due to persistent fetal circulation Anaemia Asphyxia Sepsis Metabolic disorder Methaemoglobinaemia due to haemolytic anaemia Management Plan Initial management:- Respiratory distress at birth Resuscitation, give CPAP and PEEP to maintain oxygenation to lungs, immediate Prostin (5ng/kg/min) Check breathing Maintain circulation IV fluids Routine bloods and ABG Continuous monitoring oxygen saturation and vitals Cardiology review- echo and CXR Echo findings consistent with Fallot tetralogy with MAPCAs CXR found cardiomegaly Feeding via TPN (6ml/kg/2 hourly) Further management:- Cardiac catherisation to assess for cardiac anomaly Cardiology experts advised surgery to establish connection between aorta and pulmonary artery to increase pulmonary blood flow. Melbourne shunt was inserted via median sternotomy on 1/10/09. Echo post-op showed good flow in small pulmonary arteries and patent central shunt. Continue monitoring oxygen saturation aim to keep above 75% Perform ECG Monitor temperature post-op If pyrexial, culture blood and give vancomycin and gentamicin. Start on aspirin, frusemide, spironolactone and paracetamol PRN. Increase feed to 150ml/kg/day via bottle Relevant Investigations and Results: Bloods results (2/10/09) after cardiac surgery FBC Hb Platelets WBC 15.2 230 10.5 UE Na+ K+ Cl Urea Creatinine 142 4.7 100 3.9 77 LFTs Alk P AST ALT Pro Alb 274 29 31 55 27 CRP 100 Echo Results on 18/09/09 Pulmonary atresia, MAPCAs, VSD, overriding aorta. Results on 2/10/09 Patent central shunt with good flow to small pulmonary arteries. MAPCAs flow demonstrated from joined aortopulmary branches. X-ray of whole spine Single AP view of thoracolumbar spine no abnormality found. Reflective Commentary: Tetralogy of Fallot Tetralogy of Fallot (TOF) is the commonest cause of cyanotic congenital heart disease. It has 4 cardinal anatomical anomalies:- [1] Large outlet VSD Overriding aorta with respect to ventricular septum Right ventricular outflow obstruction (infundibular and valvular pulmonary stenosis) Right ventricular hypertrophy Epidemiology TOF affects 3-6 infants in every 10, 000 births. [1] Aetiology [4] The aetiology is unknown, but evidence suggests a multifactorial cause. Antenatal risk factors are:- Maternal rubella (or other viral infections during pregnancy) Poor antenatal nutrition Maternal alcohol use Maternal age > 40 years Diabetes Children with Down syndrome have a higher risk of TOF. Presentation [4] Symptoms Very few infants present with severe cyanosis in the first few days of life with duct-dependent pulmonary circulation. Most infants are diagnosed by murmur at the age of 1-2 months. Feeding difficulty and failure to thrive. Tet spells episodes of bluish pale skin during crying or feeding. Squatting is classical of infants with TOF. Exertional dyspnoea usually worsens with age. Physical examination Smaller than expected for age. Peripheral cyanosis is normally found at birth, and after 3-6 months, finger clubbing may develop. Cardiac examination:- A thrill at left sternal border. Ejection systolic murmur heard over the pulmonic area and the left sternal border. In babies with aortopulmonary collaterals, continuous murmurs may be detected The S2  is usually single. Diagnosis Diagnosis is through history and clinical examination, supported by chest x-ray and ECG, and confirmed by echocardiography. Bloods Haemoglobin and haematocrit are usually increased in relation to the degree of cyanosis. The arterial oxygen saturation varies from 65-70%. ECG ECG shows right ventricular hypertrophy (+ right axis deviation) and may also show right atrial hypertrophy. Imaging Radiography Classical boot-shaped heart. Echocardiography Used to diagnose ductus arteriosus, VSD, or ASD with Doppler echocardiography. *Comparison of Baby Js presentation to the classical presentation Baby J had severe pulmonary atresia (muscular in origin) due to a severely malrotated infundibular septum. He suffered from life-threatening cyanosis at birth which had to be attended to promptly by maintaining the patency of ductus arteriosis using prostaglandin E2. As he was highly symptomatic, a palliative surgery to increase pulmonary blood flow had to be done where he underwent placement of Melbourne shunt connecting his aorta to MAPCA. This was done hoping to promote the development of main pulmonary artery. Baby J also had another problem which was the 50% possible chance of inheriting Alagilles syndrome (autosomal dominant) from his father. His LFTs were checked for any liver abnormality which is commonly implicated in this syndrome. He also had spine X-ray to exclude spinal deformities. He will be having ophthalmology review soon as well. GMC theme 2: Treatment Acute treatment [4] Neonates with severe cyanosis due to ductal constriction are given an infusion of prostaglandin E2(0.05 to 0.1  ÃƒÅ½Ã‚ ¼g/kg/min IV) to reopen the ductus arteriosus. Tet spells are usually self-limiting and followed by a period of sleep. If prolonged (> 15 mins), treatment consists of:- [4] placing infants in a knee-chest position sedation and pain relief morphine  IM. IV fluids are used for volume expansion. Propanolol  IV acts as peripheral vasoconstrictor. Bicarbonate to correct acidosis. Muscle paralysis and artificial ventilator to reduce oxygen demand. Symptomatic or palliative treatment in first few months Palliative surgery can be performed in patients who are not suitable for complete repair or patients with tet spells. One of the commonly used procedures is the Blalock-Taussig shunt where the subclavian artery is connected to the ipsilateral pulmonary artery with a prosthetic graft. Baby J had a relatively new shunt placement (first developed in Melbourne) which connects the major aortopulmonary collateral artery (MAPCA) to aorta. This has been shown to promote the growth of central pulmonary artery. [2] Melbourne shunt: illustrates the completed shunt with the pulmonary artery anastomosed to the posterior and left lateral aspect of the ascending aorta close to the sinotubular junction. Adapted from Ref [2] Corrective Surgery Since TOF is a progressive disorder, Baby J will require a more definitive corrective surgery. Nowadays, surgery is commonly performed electively at around 6 months of age (or before 1 year). The timing of complete surgical repair on Baby J will depend on numerous factors like further symptoms, severity of lesions (multiple VSDs, pulmonary atresia),etc. Complete repair is achieved by patch closure of VSD and widening of right ventricular outflow tract. Perioperative mortality rate is   Primary repair is beneficial in terms of preventing long-term right ventricular outflow obstruction and the consequential development of right ventricular hypertrophy, prolonged cyanosis, and postnatal angiogenesis. [1] Summary Survival in children with simple forms of TOF is promising and quality of life is good. Studies showed that survivors are in NYHA 1 class with minimal reduction in exercise capacity. However, Baby J has a rather severe form of TOF and it is difficult to predict his prognosis as for now. This will depend on his progress after corrective surgery repair done. He will need life-long cardiac review and this can be quite stressful for the child and the family as well. Baby J is fortunate to have good supportive parents who are both rather anxious about the childs condition during the interview.