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Question 1 An infant born at 34 week gestation has low Apgar score. The mother has PMH of hypertension and bipolar disease and did not stop any of her medications during pregnancy. Which of the following foetal complications is he at risk of? • Nasal hypoplasia • Ebstein Anomaly • Oligohydroamnios • Atrial septal defect • Poor teeth Question 1 An infant born at 35 week gestation has low Apgar score. The mother has PMH of hypertension and bipolar disease and did not stop any of her medications during pregnancy. Which of the foetal complication is he at risk of? • Nasal hypoplasia • Ebstein Anomaly • Oligohydroamnios • Atrial septal defect • Poor teeth Ebstein Anomaly • Rare congenital heart disorder occurring approximately 1 in every 200,000 live births and accounting for <1% of all cases of congenital heart disease. • It is malformation of the tricuspid valve and right ventricle causing a bigger right atrium and a smaller right ventricle. This leads to poor flow from the right atrium to right ventricle and therefore poor flow to pulmonary vessels. It is often associated with right to left shunt across the atria and atrial septal defect. • Presentation: evidence if heart failure, gallop rhythm, cyanosis, Sob • Diagnosis: Echo • Management: medical management includes treating arrhythmia and heart failure. Definitive management is surgery, Question 2 A 4 year old male presented to GP with fever and red tongue throat. A course of Pen V has so far been ineffective. She has had >7 days of high fever (unable to bring down the fever pharmacologically). She has had cough and coryza. On examination she has widespread maculopapular rash, red hands and feet and peeling pf the skin around the toes. • Coxsackie infection • Measles • Kawasaki disease • Scarlet fever • Viral URTI Question 2 A 4 year old male presented to GP with fever and red tongue throat. A course of Pen V has so far been ineffective. She has had 7 days of high fever (unable to bring down the fever pharmacologically). She has had cough and coryza. On examination she has widespread maculopapular rash, red hands and feet and peeling pf the skin around the toes. • Coxsackie infection • Measles • Kawasaki disease • Scarlet fever • Viral URTI Kawasaki Disease • Medium vessel vasculitis (common around age 2 and rare after the age of 5. • Signs and Symptoms: persistent fever >5 days, dry bilateral conjunctivitis, non-vesicular, desquamating rash which starts at the extremities (including palms and soles). • Strawberry tongue • Management: Aspirin and IV immunoglobulins in acute phase (Reye’s in kids) • Long term: lifelong aspirin as 25% develop coronary artery aneurysms and are at risk of early MI. Question 3 A 5 year old boy walks with a limp due to right hip pain which is relieved by rest and worsened on walking or standing. X-ray reveals a periarticular left hip swelling in soft tissue. A bone scan reveals reduced activity in the anterolateral left capital femoral epiphysis. • Slipped Capital Femoral Epiphysis • Perthes disease • Septic arthritis • Epiphyseal dysplasia • Synovitis Question 3 A 5 year old boy walks with a limp due to right hip pain which is relieved by rest and worsened on walking or standing. X-ray reveals a periarticular right hip swelling in soft tissue. A bone scan reveals reduced activity in the anterolateral right capital femoral epiphysis. • Synovitis • Perthes disease • Septic arthritis • Epiphyseal dysplasia • Slipped Capital Femoral Epiphysis Perthes Disease • years old necrosis of the femoral head, usually affects boys age 5-10 • buttocks. Worse with activities. May appear similar to transient synovitis initially but it usually persist for longer • sclerosis, fragmentation and eventually flattening of femoral head., • needed.ay be normal in early disease do MRI or bone scan maybe • Management: simple analgesic and rest (followed by early mobilization) acetabulum if severe or >/= 6 years old surgery to secure femoral head in Question 4 A 13 year old boy with haemophillia B has fallen whilst playing football. He has a swollen and bruised knee. Which of the following is the best treatment option in the acute phase? • Vitamin K • Recombinant factor VIII • Recombinant Factor XI • Cryoprecipitate • Recombinant factor IX Question 4 A 13 year old boy with haemophillia B has fallen whilst playing football. He has a swollen and bruised knee. Which of the following is the best treatment option in the acute phase? • Vitamin K • Recombinant factor VIII • Recombinant Factor XI • Cryoprecipitate • Recombinant factor IX Haemophilia B • Factor 9 deficiency due to X-linked mutation – exclusively affect males. • Haemoarthrosis, haematoma in muscles, intracranial haemorrhages. Patient can bleed exclusively in response to minor trauma and cause spontaneous haemorrhage without any trauma • Diagnosis is based on bleeding score, coag screen and genetic testing • Management: IV infusions of clotting factors. Acute episodes: IV infusions, Desmopressin, Tranexamic acid. Question 5 A 5 year old body is brought to his GP by a concerned mother. Patient otherwise fit and well but for the past 2 weeks he has wet his bed consistently every night. • Urodynamic study • Bladder USS • Trial of fluid restriction • Urodynamic testing • Urinalysis Question 5 A 5 year old body is brought to his GP by a concerned mother. Patient otherwise fit and well but for the past 2 weeks he has wet his bed consistently every night. • Urodynamic study • Bladder USS • Trial of fluid restriction • Urodynamic testing • Urinalysis Enuresis • This is a term that describes involuntary urination. Bedwetting is called nocturnal enuresis. During the day if the child is unable to control bladder it is called diurnal enuresis. • 2 type: Primary and secondary nocturnal enuresis • Primary nocturnal enuresis: • development, particular if the child is <5 years. Often patients will have a family history. Other causes: overactive bladder, fluid intake, failure to wake due to deep sleep, psychological distress, secondary causes include chronic constipation, UTI and learning disability • Management: Reassure parents, lifestyle changes, treat underlying cause, alarms, positive reinforcement and pharmacological treatment • Secondary Nocturnal Enuresis: • When the child starts wetting bed when they have been previously dry. This is more concerning because it points towards underlying illness. Common causes include: UTI, constipation, T1DM or Abuse • common/easily treatable cause is UTI and constipation. Other problems may require referral to secondary care for further management. Question 6 A 14 year old girl presents to her GP with primary amenorrhea and cyclical abdominal pain, fullness in the abdomen. On examination patient has normal secondary sexual development • Hyperprolactinemia • Congenital Deformity of Uterus • Hypothyroidism • Normal physiological delay • Imperforate hymen Question 6 A 14 year old girl presents to her GP with primary amenorrhoea and cyclical abdominal pain, fullness in the adbomen. On examination patient has normal secondary sexual development • Hyperprolactinemia • Congenital Deformity of Uterus • Hypothyroidism • Normal physiological delay • Imperforate hymen Imperforate Hymen • Rare condition when the hymen completely covers the opening to vagina. It is considered congenital anomaly. The hymen surrounds the opening of vaginal. Bloods becomes trapped inside the vaginal during menstruation because the hymen covers the exit to the uterus. • Symptoms: abdominal/pelvic pain, feeling of fullness in the abdomen. Lack of menstruation and painful urination • It can be detected in infants during a physical exam or in teenagers after puberty. • Management: Hymenectomy and dilator to prevent the hole from closing down. Question 7 An 8 year old girl is brought to her GP by her mother because she is shorter than most of her classmates. She does well academically. On examination she is short and has a webbed neck and widely spaced nipples. • 47 XXY • 45 XO • 47 XYY • Normal karyotype • 46 XY Question 7 An 8 year old girl is brought to her GP by her mother because she is shorter than most of her classmates. She does well academically. On examination she is short and has a webbed neck and widely spaced nipples. • 47 XXY • 45 XO • 47 XYY • Normal karyotype • 46 XY Turner’s syndrome • 1 in 3000 girls • Short stature, webbed neck, wide carrying angle, low set ears. • Other features: underdeveloped ovaries, aortic coractation/dissection and horseshoe kidney • Associated conditions: Recurrent otitis media, recurrent UTIs, obesity and Diabetes • Management: Growth hormone therapy, oestrogen and Progesterone replacement + fertility treatment Question 8 An 6 year old with colicky abdominal pain, nausea, vomiting and diarrhea over the past 3 days. There is also passage of blood in the diarrhea and mucus. Also, arthralgia of knees, elbows, ankles and wrists. On examination there is a palpable purpuric rash on his extremities. Urinalysis shows microscopic hematuria and proteinuria 1+ • HUS • ITP • DIC • HSP • Dysteney Question 8 An 6 year old with colicky abdominal pain, nausea, vomiting and diarrhea over the past 3 days. There is also passage of blood in the diarrhea and mucus. Also, arthralgia of knees, elbows, ankles and wrists. On examination there is a palpable purpuric rash on his extremities. Urinalysis shows microscopic hematuria and proteinuria 1+ • HUS • ITP • DIC • HSP • Dysteney ITP (Idiopathic Thrombocytopenic Purpura) • ITP is a condition that causes spontaneous, low platelet count causing a purpuric rash. It is a type 2 hypersensitivity reaction. It is caused by production antibodies that target and destroy platelets, This can happen spontaneously or it can be triggered by something like viral infection. • This condition presents with bleeding, bruising, petechial or pupuric rash • Management: urgent FBC for platelet count. Other causes should be excluded for example Heparin induced thrombocytopenia and leukemia • Treatment include: Prednisolone, IV immunoglobulins, Bloods transfusion and platelet transfusion • Avoid things like contact sport, IM injection and other procedure like LP. Question 9 A 2 year old boy is admitted with not being able to open bowels since birth. He has not passed meconium. His abdomen is distended and he has several episodes of bile stained vomit. Abdominal X-ray shows obstruction. Which of the following investigations will provide the definitive diagnosis • Abdominal USS • Erect CXR • Colonoscpy • Upper GI endoscopy • Rectal biopsy Question 9 A 2 year old boy is admitted with not being able to open bowels since birth. He has not passed meconium. His abdomen is distended and he has several episodes of bile stained vomit. Abdominal X-ray shows obstruction. Which of the following investigations will provide the definitive diagnosis • Abdominal USS • Erect CXR • Colonoscpy • Upper GI endoscopy • Rectal biopsy Hirschprung’s disease • This is a congenital condition where nerve cells of myenteric plexus are absent in distal bowel and rectum. The myenteric plexus, also known as Auerbach’s plexus forms the enteric nervous system. • The length of colon without innervation varies between patients. The aganglionic section of colon does not relax, causing it to become constricted and causing bowel obstruction • Presentation is usually: delay in passing meconium (>24 hours), chronic constipation since birth, abdominal pain and distention, vomiting and poor weight gain and failure to thrive • Management: Abdominal X-ray (can be helpful in diagnosing Rectal biopsy is used to confirm diagnosis. The bowel histology will demonstrate an absence of ganglionic cells. Unwell children will require IV fluids and IV abx. Definitive management is by surgical removal and aganglionic section of bowel Question 10 An infant born to a mother with poor nutritional status was born with midline lesion and cystic mass around spinal canal. Deficiency of which of the following could have caused this? • Vitamin B1 • Folic acid • Vitamin B3 • Vitamin B5 • Iron Question 10 An infant born to a mother with poor nutritional status was born with midline lesion and cystic mass around spinal canal. Deficiency of which of the following could have caused this? • Vitamin B1 • Folic acid • Vitamin B3 • Vitamin B5 • Iron Spina Bifida • Spina Bifida is a birth defect that affects the spine. Normally in first month of pregnancy a special set of cells form the neural tube. The top of the tube becomes the brain an remainder becomes the spinal cord and structures around it. In spina bfiida the tube does not close fully. Often abnormalities of the brain accompany the spine such as hydrocephalus. 4 different types of spina bifida. • Folic acid is an important vitamin for development of foetus. Food high in folic acid include dark green vegetables, egg yolks and fruits • Management: surgery Question 12 A 2 year old girl was running around the play room and bumped her head against a table, following which she gasped, became pale and lost consciousness, She had some limb jerking movements but regained consciousness quickly. Which of the following is the cause of this episode? • Meningitis • Epilepsy • Hypoglycemia • Reflex anoxic seizure • Febrile convulsion Question 12 A 2 year old girl was running around the play room and bumped her head against a table, following which she gasped, became pale and lost consciousness, She had some limb jerking movements but regained consciousness quickly. Which of the following is the cause of this episode? • Meningitis • Epilepsy • Hypoglycemia • Reflex anoxic seizure • Febrile convulsion Reflex Anoxic Seizure • This occurs when a child is startled. The vagus nerve sends strong signals to the heart that causes it to stop beating. The child will suddenly go pale, lose consciousness and may start to have some seizure-like muscle twitching. Within 30 seconds and the heart restarts. • Management: After excluding other pathology and making diagnosis, educating and reassuring parents about breath holding spells is the key to management. Breath holding spells have been liked to Iron deficiency anemia. • There are 2 types of breath holding spells – cyanotic breath holding spells and reflex anoxic seizure Question 13 A baby born at terms has no respiratory effort at 1 minute. The baby is tachycardia with looks blue. The baby is not making any movements and there is no femoral pulse palpable. What is the next most appropriate action to take? • Cardiac massage • Start CPR • Cardiac massage • Intubate and ventilate • 5 breaths via face mask Question 13 A baby born at terms has no respiratory effort at 1 minute. The baby is tachycardia with looks blue. The baby is not making any movements and there is no femoral pulse palpable. What is the next most appropriate action to take? • Cardiac massage • Start CPR • Cardiac massage • Intubate and ventilate • 5 breaths via face maskNeonatal Life support https://bnfc.nice.org.uk/medicines-guidance/life-support-algorithm-image/ Question 14 A 2 year old toddler is brought in by his mother to the ED with barking cough, fever, stridor and intercostal recession. Observations show tachycardia and raised respiratory count. He is diagnosed with croup. What is the most appropriate management? • No treatment required • Nebulised adrenaline and admit under paeds • Single dose of dexamethasone and discharge home with worsening advice • Single dose of dexamethasone and admit under paeds • Give high flow oxygen and nebulized salbutamol Question 14 A 2 year old toddler is brought in by his mother to the ED with barking cough, fever, stridor and intercostal recession. Observations show tachycardia and raised respiratory count. He is diagnosed with croup. What is the most appropriate management? • No treatment required • Nebulised adrenaline and admit under paeds • Single dose of dexamethasone and discharge home with worsening advice • Single dose of dexamethasone and admit under paeds • Give high flow oxygen and nebulized salbutamol Croup • Croup is an acute infective respiratory disease years. It is an upper respiratory tract infection causing oedema in larynx. The classic cause of croup is parainfluenza virus. It usually responds well to treatment particularly dexamethasone. • Causes: Parainfluenza, influenza, adenovirus, RSV • Presentation: barking cough, stridor, fever, hoarse voice • rest). During attacks it can help to sit the child up and comfort, Stat dose dexamethasone is very effective. If that fails  oral dex, oxygen, nebulized budesonide, nebulized adrenaline, intubation and ventilation Question 15 A 14 year old presents to her GP with 6 week history of knee pain. She does athletic out of school. On examination there is tenderness around the tibial tuberosities. Her ligament stress tests are all normal. What is the most appropriate management? • Routine X-ray • MRI • Referral to T&O • Analgesia, physiotherapy and reduction in physical excerise • Urgent MRI Question 15 A 14 year old presents to her GP with 6 week history of knee pain. She does athletic out of school. On examination there is tenderness around the tibial tuberosities. Her ligament stress tests are all normal. What is the most appropriate management? • Routine X-ray • MRI • Referral to T&O • Analgesia, physiotherapy and reduction in physical exercise • Urgent MRI Os-good Schattler’s syndrome • This is caused by inflammation at the tibial tuberosity where the patella ligament inserts. • Common cause of anterior knee pain in adolescents. • Presentation: visible or palpab e hard and tender lump at the tibial tuberosity. Pan in the anterior aspect of the knee • The pain is exacerbated by physical activity, kneeling and on extension of knee • Management: reduction in physical activity, NSAIDs, Ice Question 16 A baby born at terms starts non-billous vomiting at 2 weeks. There is a small olive mass in the epigastrium on examination What is the likely diagnosis • Duodenal atresia • Pancreatitis • Pyloric stenosis • Biliary stenosis • UGI obstruction Question 16 A baby born at terms starts non-billous vomiting at 2 weeks. There is a small olive mass in the epigastrium on examination What is the likely diagnosis • Duodenal atresia • Pancreatitis • Pyloric stenosis • Biliary stenosis • UGI obstruction Pyloric Stenosis • Pyloric stenosis presents in the first few weeks of life. The classic presentation is projectile vomiting. • If examined after feeding, often the peristalsis can be seen by observing the abdomen. IT feels like large olive. This is caused by hypertrophic muscle of the pylorus. • Management: Diagnosis is made using abdominal USS to visualize thickened pylorus. Definitive management involves laparoscopic pyloromyotomy. Conditions that we covered… • Ebstein Anomaly • Perthes Disease • Kawasaki Disease • Neonatal Life support • Pyloric Stenosis • Os-good schattler Disease • Croup • Spina Bifida • Haemophillia A and B • Turner’s syndrome • ITP • Enuresis • Reflex anoxic seizure