-
Question 1
Incorrect
-
A 7-year-old girl is brought to the pediatrician by her father. She has been experiencing coryza and a fever of 38C for the past 3 days. This morning her father noticed a red rash on both cheeks and pallor surrounding her mouth. What is the most probable organism responsible for these symptoms?
Your Answer: coxsackievirus A16
Correct Answer: Parvovirus B19
Explanation:The cause of the boy’s symptoms, which include a red rash following coryza and fever, is erythema infectiosum, also known as slapped-cheek syndrome. This infection is caused by parvovirus b19, a common organism responsible for childhood infections.
The table provides information on various childhood infections including chickenpox, measles, mumps, rubella, erythema infectiosum, scarlet fever, and hand, foot and mouth disease. Each infection has its own unique features such as fever, rash, and systemic upset. Chickenpox starts with fever and an itchy rash that spreads from the head and trunk. Measles has a prodrome of irritability and conjunctivitis, followed by a rash that starts behind the ears and spreads to the whole body. Mumps causes fever, malaise, and parotitis. Rubella has a pink maculopapular rash that starts on the face and spreads to the whole body, along with suboccipital and postauricular lymphadenopathy. Erythema infectiosum, also known as fifth disease, causes lethargy, fever, and a slapped-cheek rash. Scarlet fever is a reaction to erythrogenic toxins produced by Group A haemolytic streptococci and causes fever, malaise, tonsillitis, and a rash with fine punctate erythema. Hand, foot and mouth disease causes mild systemic upset, sore throat, and vesicles in the mouth and on the palms and soles of the feet.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 2
Correct
-
Which statement regarding ventricular septal defect (VSD) is correct?
Your Answer: There may be a diastolic murmur at the apex
Explanation:Ventricular Septal Defects
Ventricular septal defects (VSDs) are a type of congenital heart defect that can cause a diastolic murmur. This murmur can occur due to aortic incompetence or increased flow across the mitral valve, which can lead to relative mitral stenosis. In some cases, right to left shunting can occur, which can cause cerebral abscesses.
While large VSDs may be associated with soft murmurs, pulmonary hypertension can occur in association with increased flow across the shunt. However, it may also indicate decreased flow across the shunt and increased pulmonary vascular resistance, which can result in a softer murmur.
It’s important to note that the risk of bacterial endocarditis is high in individuals with VSDs, even those with haemodynamically trivial lesions. Therefore, it’s crucial to monitor and manage this condition carefully.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 3
Incorrect
-
A 27-year-old man presents with a persistent cough for the past 20 days which initially started with a few days of cold symptoms. He describes it as ‘the worst cough I've ever had’. He has bouts of coughing followed by an inspiratory gasp. This is usually worse at night and can be so severe that he sometimes vomits. He is otherwise fit and well and confirms he completed all his childhood immunisations. Examination of his chest is unremarkable.
What is the most suitable initial management for this likely diagnosis?Your Answer: Start a course of oral prednisolone
Correct Answer: Start a course of oral clarithromycin
Explanation:If the onset of cough is within the previous 21 days, the recommended first-line treatment for whooping cough is a course of oral clarithromycin or azithromycin. In this case, the correct answer is to start a course of oral clarithromycin as the patient’s history is consistent with whooping cough. Starting treatment within 21 days of onset of the cough can help to reduce the risk of spread. It is incorrect to not start any treatment as the patient has presented within the appropriate timeframe. Offering an immediate booster vaccination is also not indicated in the initial management of the index case, and starting a course of oral doxycycline is not the first-line treatment for whooping cough.
Whooping Cough: Causes, Symptoms, Diagnosis, and Management
Whooping cough, also known as pertussis, is a contagious disease caused by the bacterium Bordetella pertussis. It is commonly found in children, with around 1,000 cases reported annually in the UK. The disease is characterized by a persistent cough that can last up to 100 days, hence the name cough of 100 days.
Infants are particularly vulnerable to whooping cough, which is why routine immunization is recommended at 2, 3, 4 months, and 3-5 years. However, neither infection nor immunization provides lifelong protection, and adolescents and adults may still develop the disease.
Whooping cough has three phases: the catarrhal phase, the paroxysmal phase, and the convalescent phase. The catarrhal phase lasts around 1-2 weeks and presents symptoms similar to a viral upper respiratory tract infection. The paroxysmal phase is characterized by a severe cough that worsens at night and after feeding, and may be accompanied by vomiting and central cyanosis. The convalescent phase is when the cough subsides over weeks to months.
To diagnose whooping cough, a person must have an acute cough that has lasted for 14 days or more without another apparent cause, and have one or more of the following features: paroxysmal cough, inspiratory whoop, post-tussive vomiting, or undiagnosed apnoeic attacks in young infants. A nasal swab culture for Bordetella pertussis is used to confirm the diagnosis, although PCR and serology are increasingly used.
Infants under 6 months with suspected pertussis should be admitted, and in the UK, pertussis is a notifiable disease. An oral macrolide, such as clarithromycin, azithromycin, or erythromycin, is indicated if the onset of the cough is within the previous 21 days to eradicate the organism and reduce the spread. Household contacts should be offered antibiotic prophylaxis, although antibiotic therapy has not been shown to alter the course of the illness. School exclusion is recommended for 48 hours after commencing antibiotics or 21 days from onset of symptoms if no antibiotics are given.
Complications of whooping cough include subconjunctival haemorrhage, pneumonia, bronchiectasis, and
-
This question is part of the following fields:
- Paediatrics
-
-
Question 4
Incorrect
-
What are the possible causes of cyanosis in a newborn?
Your Answer: VSD
Correct Answer: Transposition of the great arteries
Explanation:Common Heart Conditions and Their Characteristics
Ventricular septal defect (VSD) is a heart condition where there is a hole in the wall that separates the two lower chambers of the heart. This results in a left to right shunt, which means that oxygen-rich blood from the left side of the heart flows into the right side of the heart and mixes with oxygen-poor blood. This can lead to symptoms such as shortness of breath, fatigue, and poor growth in infants.
Coarctation is another heart condition where there is a narrowing of the aortic arch, which is the main blood vessel that carries blood from the heart to the rest of the body. This narrowing can cause high blood pressure in the arms and head, while the lower body receives less blood flow. Symptoms may include headaches, dizziness, and leg cramps.
Hyperbilirubinaemia, on the other hand, is not associated with cyanosis, which is a bluish discoloration of the skin and mucous membranes due to low oxygen levels in the blood. Hyperbilirubinaemia is a condition where there is an excess of bilirubin in the blood, which can cause yellowing of the skin and eyes.
Lastly, Eisenmenger syndrome is a rare but serious complication that can develop much later in life following a left to right shunt, such as in VSD. This occurs when the shunt reverses and becomes a right to left shunt, leading to low oxygen levels in the blood and cyanosis. Symptoms may include shortness of breath, fatigue, and heart palpitations.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 5
Correct
-
As a doctor, you have been requested by a midwife to conduct a newborn examination on a 24-hour old infant. During the examination, the parents express concern about their baby's penis appearing abnormal and request your assistance. Upon inspection, you observe that the urethral meatus is situated on the ventral aspect of the glans and the prepuce is hooded. However, the baby has already passed urine with a strong stream, as noted by the midwife earlier in the day. What should be the doctor's next course of action?
Your Answer: Refer to a specialist for possible surgery around 12 months of life
Explanation:It is recommended to refer a child with hypospadias to a specialist for possible surgery around the age of 12 months. This timing is considered optimal, taking into account various factors such as developmental milestones, tolerance of surgery and anesthesia, and the size of the penis. It is important to refer the child to a specialist at the time of diagnosis. Referring a child for surgery within the first month of life or within four hours is not necessary, as this is not an urgent or life-threatening issue. Hypospadias always requires a specialist referral, even if it is mild, and it is the specialist’s responsibility, along with the parents, to decide whether surgery is necessary. It is crucial to advise parents not to circumcise their child with hypospadias, as the prepuce may be used during corrective surgery.
Understanding Hypospadias: A Congenital Abnormality of the Penis
Hypospadias is a condition that affects approximately 3 out of 1,000 male infants. It is a congenital abnormality of the penis that is usually identified during the newborn baby check. However, if missed, parents may notice an abnormal urine stream. This condition is characterized by a ventral urethral meatus, a hooded prepuce, and chordee in more severe forms. The urethral meatus may open more proximally in the more severe variants, but 75% of the openings are distally located. There appears to be a significant genetic element, with further male children having a risk of around 5-15%.
Hypospadias most commonly occurs as an isolated disorder, but it can also be associated with other conditions such as cryptorchidism (present in 10%) and inguinal hernia. Once hypospadias has been identified, infants should be referred to specialist services. Corrective surgery is typically performed when the child is around 12 months of age. It is essential that the child is not circumcised prior to the surgery as the foreskin may be used in the corrective procedure. In boys with very distal disease, no treatment may be needed. Understanding hypospadias is important for parents and healthcare providers to ensure proper management and treatment of this condition.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 6
Correct
-
A 6-month-old boy is brought to the paediatrics emergency department by his parents. His parents report that he has had a fever for 2 days with reduced activity and feeding. On examination, he is not smiling, his respiratory rate is 50 breaths/minute (normal range: 30-60 breaths/minute) and his heart rate is 155 beats/minute (100-160 beats/minute). He also has reduced skin turgor and looks pale. A nurse looks up the NICE traffic light system to identify his risk of serious illness.
What is the most concerning symptom in this patient?Your Answer: Reduced skin turgor
Explanation:According to the NICE traffic light system, reduced skin turgor is a red flag symptom in children. This system is used to identify the risk of serious illness in children. Decreased activity is considered an amber symptom, while age <6 months is not a red flag symptom. Age 3-6 months with a temperature of >=39ºC is an amber flag symptom, and age <3 months with a temperature of >=38°C is a red flag symptom. A respiratory rate of >60 breaths/minute is also a red flag symptom. Finally, a heart rate of >160 beats/minute in children under 12 months is an amber flag symptom.
The NICE Feverish illness in children guidelines were introduced in 2007 and updated in 2013. These guidelines use a ‘traffic light’ system to assess the risk of children under 5 years old presenting with a fever. It is important to note that these guidelines only apply until a clinical diagnosis of the underlying condition has been made. When assessing a febrile child, their temperature, heart rate, respiratory rate, and capillary refill time should be recorded. Signs of dehydration should also be looked for. Measuring temperature should be done with an electronic thermometer in the axilla if the child is under 4 weeks old or with an electronic/chemical dot thermometer in the axilla or an infrared tympanic thermometer.
The risk stratification table includes green for low risk, amber for intermediate risk, and red for high risk. The table includes categories such as color, activity, respiratory, circulation and hydration, and other symptoms. If a child is categorized as green, they can be managed at home with appropriate care advice. If they are categorized as amber, parents should be provided with a safety net or referred to a pediatric specialist for further assessment. If a child is categorized as red, they should be urgently referred to a pediatric specialist. It is important to note that oral antibiotics should not be prescribed to children with fever without an apparent source, and a chest x-ray does not need to be routinely performed if a pneumonia is suspected but the child is not going to be referred to the hospital.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 7
Correct
-
A 5-year-old boy with an otherwise unremarkable medical history develops an ulcer in his ileum. What is the most likely congenital birth defect that caused his condition?
Your Answer: Meckel’s diverticulum
Explanation:Common Congenital Abnormalities of the Digestive System
The digestive system can be affected by various congenital abnormalities that can cause significant health problems. Here are some of the most common congenital abnormalities of the digestive system:
Meckel’s Diverticulum: This condition is caused by the persistence of the vitelline duct and is found in the small intestine. It can contain ectopic gastric mucosa and can cause painless rectal bleeding, signs of obstruction, or acute appendicitis-like symptoms. Treatment involves excision of the diverticulum and its adjacent ileal segment.
Pyloric Stenosis: This congenital condition is associated with hypertrophy of the pyloric muscle and presents with projectile, non-bilious vomiting at around 4-8 weeks of age.
Tracheo-Oesophageal Fistula: This condition is associated with a communication between the oesophagus and the trachea and is often associated with oesophageal atresia. Infants affected struggle to feed and may develop respiratory distress due to aspiration of feed into the lungs.
Gastroschisis: This is a ventral abdominal wall defect where part of the bowel, and sometimes the stomach and liver, herniate through the defect outside the body. It is corrected surgically by returning the herniating organs to the abdominal cavity and correcting the defect.
Omphalocele: This is an abdominal wall defect in the midline where the gut fails to return through the umbilicus to the abdominal cavity during embryonic development. The protruded organs are covered by a membrane, and correction is surgical by returning the herniating organs into the abdominal cavity and correcting the umbilical defect.
In conclusion, these congenital abnormalities of the digestive system require prompt diagnosis and treatment to prevent complications and improve outcomes.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 8
Incorrect
-
A 7-year-old girl arrives at the emergency department with severe wheezing and shortness of breath. She is struggling to speak in full sentences and her peak expiratory flow rate is 320 l/min (45% of normal). Her oxygen saturation levels are at 92%. Her pCO2 is 4.8 kPa.
What is the most concerning finding from the above information?Your Answer: Cannot complete sentences
Correct Answer: pCO2 (kPa)
Explanation:Assessing Acute Asthma Attacks in Children
When assessing the severity of asthma attacks in children, the 2016 BTS/SIGN guidelines recommend using specific criteria. These criteria can help determine whether the attack is severe or life-threatening. For a severe attack, the child may have a SpO2 level below 92%, a PEF level between 33-50% of their best or predicted, and may be too breathless to talk or feed. Additionally, their heart rate may be over 125 (for children over 5 years old) or over 140 (for children between 1-5 years old), and their respiratory rate may be over 30 breaths per minute (for children over 5 years old) or over 40 (for children between 1-5 years old). They may also be using accessory neck muscles to breathe.
For a life-threatening attack, the child may have a SpO2 level below 92%, a PEF level below 33% of their best or predicted, and may have a silent chest, poor respiratory effort, agitation, altered consciousness, or cyanosis. It is important for healthcare professionals to be aware of these criteria and to take appropriate action to manage the child’s asthma attack. By following these guidelines, healthcare professionals can help ensure that children with asthma receive the appropriate care and treatment they need during an acute attack.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 9
Correct
-
Which one of the following statements regarding cow's milk protein intolerance/allergy in toddlers is true?
Your Answer: The majority of cases resolve before the age of 5 years
Explanation:Understanding Cow’s Milk Protein Intolerance/Allergy
Cow’s milk protein intolerance/allergy (CMPI/CMPA) is a condition that affects around 3-6% of children, typically presenting in the first 3 months of life in formula-fed infants. Both immediate and delayed reactions can occur, with CMPA used for immediate reactions and CMPI for mild-moderate delayed reactions. Symptoms include regurgitation, vomiting, diarrhea, urticaria, atopic eczema, colic symptoms, wheeze, chronic cough, and rarely, angioedema and anaphylaxis. Diagnosis is often clinical, with investigations including skin prick/patch testing and total IgE and specific IgE (RAST) for cow’s milk protein.
Management for formula-fed infants includes using extensive hydrolyzed formula (eHF) milk as the first-line replacement formula for mild-moderate symptoms and amino acid-based formula (AAF) for severe CMPA or if no response to eHF. Around 10% of infants are also intolerant to soya milk. For breastfed infants, mothers should eliminate cow’s milk protein from their diet and consider prescribing calcium supplements to prevent deficiency. eHF milk can be used when breastfeeding stops until at least 6 months and up to 12 months of age.
The prognosis for CMPI is usually good, with most children becoming milk tolerant by the age of 3-5 years. However, a challenge is often performed in a hospital setting as anaphylaxis can occur. It is important to refer infants with severe symptoms to a pediatrician for management. Understanding CMPI/CMPA and its management can help parents and healthcare providers provide appropriate care for affected children.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 10
Incorrect
-
A 4-year-old girl is brought to the emergency department by her father after noticing bruising on her arms. The bruising started 2 days ago and has gotten worse. The girl has no significant medical history, but had a mild cold 4 weeks ago. Upon examination, the only notable finding is extensive bruising on her upper limbs and neck. Routine blood tests were conducted and the results are as follows (with age-appropriate reference ranges): Hb 85 g/L (110-140), Platelets 68 * 109/L (150-450), WBC 19.0 * 109/L (5.0-17.0), Neutrophils 0.72 * 109/L (1.5-8.0).
What is the most probable underlying diagnosis?Your Answer: Immune thrombocytopenic purpura
Correct Answer: Acute lymphoblastic leukaemia
Explanation:The most probable diagnosis for a 6-year-old boy presenting with bruising, anaemia, and neutropenia is acute lymphoblastic leukaemia, which is the most common form of childhood leukaemia. Other forms of leukaemia, such as acute myeloid leukaemia, chronic lymphocytic leukaemia, and chronic myeloid leukaemia, are less likely to be found in children and therefore not the best answer. It is important to note that CML is associated with the Philadelphia chromosome and often presents with fatigue and mild anaemia symptoms.
Acute lymphoblastic leukaemia (ALL) is a type of cancer that commonly affects children and accounts for 80% of childhood leukaemias. It is most prevalent in children between the ages of 2-5 years, with boys being slightly more affected than girls. Symptoms of ALL can be divided into those caused by bone marrow failure, such as anaemia, neutropaenia, and thrombocytopenia, and other features like bone pain, splenomegaly, and hepatomegaly. Fever is also present in up to 50% of new cases, which may indicate an infection or a constitutional symptom. Testicular swelling may also occur.
There are three types of ALL: common ALL, T-cell ALL, and B-cell ALL. Common ALL is the most common type, accounting for 75% of cases, and is characterized by the presence of CD10 and a pre-B phenotype. Poor prognostic factors for ALL include age less than 2 years or greater than 10 years, a white blood cell count greater than 20 * 109/l at diagnosis, T or B cell surface markers, non-Caucasian ethnicity, and male sex.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 11
Correct
-
Sophie is a 15-year-old who has visited her GP seeking advice on contraception. She discloses that she has been sexually active with a 15-year-old male partner for the past 3 months. Sophie has done her research on various contraceptive methods and expresses her interest in trying the combined oral contraceptive pill (COCP). She has a history of depression due to childhood abuse and is currently receiving treatment from the Child and Adolescent Mental Health Services. However, she reports feeling much better since starting her relationship with her partner. Sophie's parents are unaware of the situation, and she is unwilling to inform them. What is the appropriate course of action?
Your Answer: Prescribe the COCP, providing there are no contraindications
Explanation:The Fraser guidelines state that if a child meets the criteria, they can be prescribed the combined oral contraceptive pill (COCP) without parental knowledge or consent. It is important to maintain confidentiality and not breach it by discussing with the child’s parents. The child’s age or lack of consent for sexual intercourse should not affect their entitlement to contraception. While a full STI screen and pregnancy test are important aspects of holistic care, they are not necessary to prescribe the COCP. The GMC’s guidance on contraception, abortion, and STIs for those aged 0-18 also allows for providing such advice and treatment to young people under 16 without parental knowledge or consent if certain criteria are met.
Guidelines for Obtaining Consent in Children
The General Medical Council has provided guidelines for obtaining consent in children. According to these guidelines, young people who are 16 years or older can be treated as adults and are presumed to have the capacity to make decisions. However, for children under the age of 16, their ability to understand what is involved determines whether they have the capacity to decide. If a competent child refuses treatment, a person with parental responsibility or the court may authorize investigation or treatment that is in the child’s best interests.
When it comes to providing contraceptives to patients under 16 years of age, the Fraser Guidelines must be followed. These guidelines state that the young person must understand the professional’s advice, cannot be persuaded to inform their parents, is likely to begin or continue having sexual intercourse with or without contraceptive treatment, and will suffer physical or mental health consequences without contraceptive treatment. Additionally, the young person’s best interests require them to receive contraceptive advice or treatment with or without parental consent.
Some doctors use the term Fraser competency when referring to contraception and Gillick competency when referring to general issues of consent in children. However, rumors that Victoria Gillick removed her permission to use her name or applied copyright have been debunked. It is important to note that in Scotland, those with parental responsibility cannot authorize procedures that a competent child has refused.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 12
Correct
-
A premature neonate is delivered via category II emergency caesarean section at 34+3 weeks for fetal distress. The obstetrician notes the baby is delivered with the cord wrapped around its neck and appears floppy and 'blue'. The neonatal team immediately begins resuscitation as the initial APGAR score is 3. At 10 minutes, the score is 5 due to cyanosis, heart rate of 120 bpm, minimal response to stimulation, poor tone, slow and irregular respiratory effort with a respiratory rate of 12/min. What is the most appropriate management of this premature neonate to reduce hypoxic ischemic encephalopathy (HIE)?
Your Answer: Therapeutic cooling
Explanation:Therapeutic Cooling for Neonates with Hypoxic Brain Injury
Therapeutic cooling, also known as therapeutic hypothermia, is a medical procedure that involves deliberately lowering a patient’s body temperature to cool the brain and prevent brain damage. This procedure has been proposed for several therapeutic uses, including neuroprotection during open-heart and neurosurgical procedures, in patients following a return of spontaneous circulation post-ventricular fibrillation arrest, in patients with trauma head injuries, in patients who have suffered acute ischemic stroke, and in neonates with moderate to severe hypoxic ischemic encephalopathy (HIE). However, the only use whose efficacy has been consistently proven in existing literature is the use of therapeutic cooling in neonates.
The use of therapeutic cooling in carefully selected term neonates with moderate to severe HIE has been recommended as standard care by the National Institute for Health and Care Excellence (NICE). It has been shown in studies to decrease mortality and improve the neurological and neurodevelopmental outcomes of treated neonates. Hypoxic perinatal brain injury is caused by a decrease in the amount of oxygen supplied to an infant’s brain just prior to, or during the process of, labor. Neonates who survive a hypoxic brain injury can develop HIE, which occurs in an estimated 2.5/1000 term births in developed countries and 26/1000 term births in the developing world.
Therapeutic cooling remains the only intervention shown to reduce neuronal damage caused by perinatal hypoxia. The procedure is thought to influence the extent of secondary neuronal death in a multifactorial manner, although the exact way in which it achieves this is not fully understood. The procedure involves placing the infant on a cooling blanket or mattress circulated with coolant fluid or circulating cold water in a cap fitted around the head. Temperature is continuously monitored throughout the treatment using either a rectal or nasopharyngeal thermometer. Close surveillance of infants during the cooling process is required given the risk for complications of both HIE and the process of cooling itself.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 13
Incorrect
-
A 6-week old infant is seen by the health visitor. She was born via breech caesarean section at 36+2 weeks gestation due to suspected chorioamnionitis and received antibiotics post-partum. Her hospital newborn physical examination (NIPE) was normal. She is currently thriving and following the 60th centile. What further assessments should the health visitor arrange for this infant based on her medical history?
Your Answer: Once weekly review for progression of weight and length in the community
Correct Answer: Ultrasounds of pelvis in 2 weeks
Explanation:An ultrasound of the pelvis in 2 weeks is the correct answer. This is because infants born in a breech position have an increased risk of DDH and require screening at 6 weeks to ensure there is no hip laxity. Vaginal swabs for group B streptococcus are not necessary if the mother is asymptomatic. Reviewing the infant’s progress along the centiles once or twice weekly is too frequent, as infants are usually weighed no more than once a month up to 6 months of age unless there are concerns about development. A full blood count is unnecessary for a well-looking infant without signs of anaemia or infection.
Developmental dysplasia of the hip (DDH) is a condition that affects 1-3% of newborns and is more common in females, firstborn children, and those with a positive family history or breech presentation. It used to be called congenital dislocation of the hip (CDH). DDH is more often found in the left hip and can be bilateral in 20% of cases. Screening for DDH is recommended for infants with certain risk factors, and all infants are screened using the Barlow and Ortolani tests at the newborn and six-week baby check. Clinical examination includes testing for leg length symmetry, restricted hip abduction, and knee level when hips and knees are flexed. Ultrasound is used to confirm the diagnosis if clinically suspected, but x-ray is the first line investigation for infants over 4.5 months. Management includes the use of a Pavlik harness for children under 4-5 months and surgery for older children with unstable hips.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 14
Incorrect
-
A 10-year-old boy is seen in the paediatric clinic for a follow-up appointment. His standing height is below the 0.4th centile, while his sitting height is on the 9th centile. These measurements have been consistent with their respective centiles for the past three years. What genetic diagnosis is most probable for this boy?
Your Answer: Fragile X syndrome
Correct Answer: Achondroplasia
Explanation:Achondroplasia and Other Causes of Short Stature
Achondroplasia is a genetic condition that affects bone growth, resulting in disproportionately short limbs and a greater sitting height compared to standing height. This is because the condition impairs the growth of cartilaginous bone, leading to much shorter arms and legs than the spine. Measuring sitting height can help estimate axial skeleton growth compared to standing height, which includes the limbs.
Other causes of short stature include Down’s syndrome and Noonan’s syndrome, which result in proportionate short stature and a similar standing and sitting centile. However, Fragile X syndrome and Klinefelter’s syndrome typically do not cause short stature. It is important to understand the underlying causes of short stature in order to provide appropriate medical care and support for individuals affected by these conditions.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 15
Correct
-
A 4-year-old child has been referred by their GP due to chronic constipation that is not responding to treatment. What specific details in the child's medical history could suggest a possible diagnosis of Hirschsprung's disease?
Your Answer: Passage of meconium at day 3
Explanation:When a baby has difficulty passing stool, it may be a sign of Hirschsprung’s disease, a condition where nerve cells in the colon are missing. This disease is more common in males and can be diagnosed through a biopsy. It is important to note that not all babies with delayed passage have this disease. Hirschsprung’s disease can also present in later childhood, so it is important to ask about the timing of symptoms in children with chronic constipation or obstruction. This disease is associated with MEN 2A/B, not MEN1, and meconium ileus is a common differential. Pyloric stenosis is associated with non-bilious vomiting, while a temperature is not a factor in suggesting Hirschsprung’s disease.
Paediatric Gastrointestinal Disorders
Pyloric stenosis is more common in males and has a 5-10% chance of being inherited from parents. Symptoms include projectile vomiting at 4-6 weeks of life, and diagnosis is made through a test feed or ultrasound. Treatment involves a Ramstedt pyloromyotomy, either open or laparoscopic.
Acute appendicitis is uncommon in children under 3 years old, but when it does occur, it may present atypically. Mesenteric adenitis causes central abdominal pain and URTI, and is treated conservatively.
Intussusception occurs in infants aged 6-9 months and causes colicky pain, diarrhea, vomiting, a sausage-shaped mass, and red jelly stool. Treatment involves reduction with air insufflation.
Intestinal malrotation is characterized by a high caecum at the midline and may be complicated by the development of volvulus. Diagnosis is made through an upper GI contrast study and ultrasound, and treatment involves laparotomy or a Ladd’s procedure.
Hirschsprung’s disease occurs in 1/5000 births and is characterized by delayed passage of meconium and abdominal distension. Treatment involves rectal washouts and an anorectal pull through procedure.
Oesophageal atresia is associated with tracheo-oesophageal fistula and polyhydramnios, and may present with choking and cyanotic spells following aspiration. Meconium ileus is usually associated with cystic fibrosis and requires surgery to remove plugs. Biliary atresia causes jaundice and increased conjugated bilirubin, and requires an urgent Kasai procedure. Necrotising enterocolitis is more common in premature infants and is treated with total gut rest and TPN, with laparotomy required for perforations.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 16
Incorrect
-
A 29-year-old primiparous woman who is 20 weeks pregnant comes in for a consultation after her foetal anomaly scan revealed polyhydramnios and a midline sac containing bowel. She has no significant medical history and is not taking any regular medications. She had planned for a home birth and is now concerned about how this condition will affect her delivery.
What is the best course of action for managing this condition?Your Answer: Plan for delivery on the ward
Correct Answer: Schedule an elective caesarean section
Explanation:If a foetus is diagnosed with exomphalos before birth, it is recommended to schedule and perform a caesarean section at term to reduce the risk of sac rupture, infection, and atresia. While a ward delivery provides access to theatres, specific surgical planning is necessary to minimize complications. Instrumental delivery in theatre does not decrease the risk of sac rupture. It is important to explain to the mother why hospital delivery is necessary and the risks associated with a home birth. Induction of labour at 37 weeks is not advisable as it increases the risk of complications during vaginal delivery.
Gastroschisis and exomphalos are both types of congenital visceral malformations. Gastroschisis refers to a defect in the anterior abdominal wall located just beside the umbilical cord. In contrast, exomphalos, also known as omphalocoele, involves the protrusion of abdominal contents through the anterior abdominal wall, which are covered by an amniotic sac formed by amniotic membrane and peritoneum.
When it comes to managing gastroschisis, vaginal delivery may be attempted, but newborns should be taken to the operating theatre as soon as possible after delivery, ideally within four hours. As for exomphalos, a caesarean section is recommended to reduce the risk of sac rupture. In cases where primary closure is difficult due to lack of space or high intra-abdominal pressure, a staged repair may be undertaken. This involves allowing the sac to granulate and epithelialise over several weeks or months, forming a shell. As the infant grows, the sac contents will eventually fit within the abdominal cavity, at which point the shell can be removed and the abdomen closed.
Overall, both gastroschisis and exomphalos require careful management to ensure the best possible outcome for the newborn. By understanding the differences between these two conditions and the appropriate steps to take, healthcare professionals can provide effective care and support to both the infant and their family.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 17
Incorrect
-
During your ward round on the paediatric unit you review a 7-year-old African American male who has been admitted for chemotherapy as he has recently been diagnosed with acute lymphoblastic leukaemia (ALL). His father anxiously asks you what his chance of survival is and how you determine this.
Upon reviewing the patient's medical records, you notice that he is on the 10th percentile for weight and the 25th percentile for height. His white cell count at diagnosis was 15 * 10^9/l and there were no noted T or B cell markers on his blood film.
What is the poor prognostic factor in this case?Your Answer: White cell count over 11 * 10^9/l at diagnosis
Correct Answer: Male sex
Explanation:Male gender is identified as a negative prognostic factor, while being Caucasian does not have a significant impact on prognosis. Other factors that may indicate a poor prognosis include presenting with the disease either less than two years or more than ten years after onset, having B or T cell surface markers, and having a white blood cell count greater than 20 billion per liter at the time of diagnosis.
Acute lymphoblastic leukaemia (ALL) is a type of cancer that commonly affects children and accounts for 80% of childhood leukaemias. It is most prevalent in children between the ages of 2-5 years, with boys being slightly more affected than girls. Symptoms of ALL can be divided into those caused by bone marrow failure, such as anaemia, neutropaenia, and thrombocytopenia, and other features like bone pain, splenomegaly, and hepatomegaly. Fever is also present in up to 50% of new cases, which may indicate an infection or a constitutional symptom. Testicular swelling may also occur.
There are three types of ALL: common ALL, T-cell ALL, and B-cell ALL. Common ALL is the most common type, accounting for 75% of cases, and is characterized by the presence of CD10 and a pre-B phenotype. Poor prognostic factors for ALL include age less than 2 years or greater than 10 years, a white blood cell count greater than 20 * 109/l at diagnosis, T or B cell surface markers, non-Caucasian ethnicity, and male sex.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 18
Correct
-
A 4-year-old girl presents with a 5-day history of fever, increasing irritability, and a rash. Her mother is concerned as she has been giving her paracetamol and ibuprofen but there has been no improvement. During examination, the child's temperature is 39.1°C, respiratory rate is 32 breaths/min, and heart rate is 140 beats/min. Further examination reveals bilateral conjunctivitis without exudate, cervical lymphadenopathy, erythema of the oral mucosa, and a non-vesicular rash that is spreading from her hands and feet. What is the immediate treatment that should be administered?
Your Answer: High dose aspirin and a single dose of intravenous immunoglobulin
Explanation:The appropriate treatment for the child with Kawasaki disease, who meets at least five of the six diagnostic criteria, is a high dose of aspirin and a single dose of intravenous immunoglobulin. The initial dose of aspirin should be 7.5-12.5 mg/kg, given four times a day for two weeks or until the child is afebrile. After that, the dose should be reduced to 2-5 mg/kg once daily for 6-8 weeks. Intravenous immunoglobulin should be administered at a dose of 2 g/kg daily for one dose, and it should be given within 10 days of the onset of symptoms. These recommendations are based on the BNF for Children.
Understanding Kawasaki Disease
Kawasaki disease is a rare type of vasculitis that primarily affects children. It is important to identify this disease early on as it can lead to serious complications, such as coronary artery aneurysms. The disease is characterized by a high-grade fever that lasts for more than five days and is resistant to antipyretics. Other symptoms include conjunctival injection, bright red, cracked lips, strawberry tongue, cervical lymphadenopathy, and red palms and soles that later peel.
Diagnosis of Kawasaki disease is based on clinical presentation as there is no specific diagnostic test available. Management of the disease involves high-dose aspirin, which is one of the few indications for aspirin use in children. Intravenous immunoglobulin is also used as a treatment option. Echocardiogram is the initial screening test for coronary artery aneurysms, rather than angiography.
Complications of Kawasaki disease can be serious, with coronary artery aneurysm being the most common. It is important to recognize the symptoms of Kawasaki disease early on and seek medical attention promptly to prevent potential complications.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 19
Incorrect
-
A 2-year-old child is brought to the emergency department by his parents. The parents have noticed that he is clutching his stomach. He has not eaten or drank any fluids for the entire day and he has vomited twice. His mother states the vomit was green in colour.
The doctor suspects a diagnosis of intestinal malrotation due to the child's symptoms and orders an abdominal ultrasound. The ultrasound shows a whirlpool sign, confirming the diagnosis. On examination, the child appears distressed and has a distended abdomen with absent bowel sounds. He looks unwell.
What is the appropriate management for this patient?Your Answer: Kasai procedure
Correct Answer: Ladd's procedure
Explanation:The appropriate treatment for a pediatric patient with intestinal malrotation and volvulus is Ladd’s procedure, which involves the division of Ladd bands and widening of the base of the mesentery. Malrotation occurs when the midgut does not complete its rotation during development, resulting in the cecum being fixed in the right upper quadrant by peritoneal bands known as Ladd bands. This can lead to the formation of a volvulus, which requires untwisting and removal of the Ladd bands, as well as resection of any necrotic bowel and removal of the appendix. The Kasai procedure is used for biliary atresia, Ramstedt pyloromyotomy for pyloric stenosis, and rectal washouts for Hirschsprung’s disease.
Paediatric Gastrointestinal Disorders
Pyloric stenosis is more common in males and has a 5-10% chance of being inherited from parents. Symptoms include projectile vomiting at 4-6 weeks of life, and diagnosis is made through a test feed or ultrasound. Treatment involves a Ramstedt pyloromyotomy, either open or laparoscopic.
Acute appendicitis is uncommon in children under 3 years old, but when it does occur, it may present atypically. Mesenteric adenitis causes central abdominal pain and URTI, and is treated conservatively.
Intussusception occurs in infants aged 6-9 months and causes colicky pain, diarrhea, vomiting, a sausage-shaped mass, and red jelly stool. Treatment involves reduction with air insufflation.
Intestinal malrotation is characterized by a high caecum at the midline and may be complicated by the development of volvulus. Diagnosis is made through an upper GI contrast study and ultrasound, and treatment involves laparotomy or a Ladd’s procedure.
Hirschsprung’s disease occurs in 1/5000 births and is characterized by delayed passage of meconium and abdominal distension. Treatment involves rectal washouts and an anorectal pull through procedure.
Oesophageal atresia is associated with tracheo-oesophageal fistula and polyhydramnios, and may present with choking and cyanotic spells following aspiration. Meconium ileus is usually associated with cystic fibrosis and requires surgery to remove plugs. Biliary atresia causes jaundice and increased conjugated bilirubin, and requires an urgent Kasai procedure. Necrotising enterocolitis is more common in premature infants and is treated with total gut rest and TPN, with laparotomy required for perforations.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 20
Incorrect
-
A 3-year-old child is admitted with poor appetite. Over the last 24 hours, they have eaten only 25% of their usual amount and have had significantly fewer wet diapers than usual. The child has been particularly irritable over this time and is not settling. There is no past medical history, no known allergies, and there has been no travel outside of the UK.
On examination, the child appears unsettled and grouchy. There are no rashes on exposure and the fontanelles appear normal. The physical observations reveal tachycardia and a fever of 39ºC. When you attempt to manually flex the child's neck you note that they also flex the hips and knees.
What is the most appropriate empirical intravenous treatment for this child?Your Answer: Cefotaxime + amoxicillin
Correct Answer: Ceftriaxone
Explanation:Investigation and Management of Meningitis in Children
Meningitis is a serious condition that can affect children. When investigating meningitis, it is important to note any contraindications to lumbar puncture, such as signs of raised intracranial pressure, focal neurological signs, papilloedema, significant bulging of the fontanelle, disseminated intravascular coagulation, or signs of cerebral herniation. For patients with meningococcal septicaemia, a lumbar puncture is contraindicated, and blood cultures and PCR for meningococcal should be obtained instead.
The management of meningitis in children involves administering antibiotics, such as IV amoxicillin (or ampicillin) and IV cefotaxime for children under three months, and IV cefotaxime (or ceftriaxone) for children over three months. Steroids should be considered if the lumbar puncture reveals certain findings, such as purulent cerebrospinal fluid, a high white blood cell count, or bacteria on Gram stain. Fluids should be administered to treat shock, and cerebral monitoring should be conducted, including mechanical ventilation if necessary.
It is also important to notify public health authorities and administer antibiotic prophylaxis to contacts. Ciprofloxacin is now preferred over rifampicin for this purpose. Overall, prompt and appropriate management of meningitis in children is crucial for ensuring the best possible outcomes.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 21
Incorrect
-
A female infant is presenting with dyspnea and cyanosis. The mother attempted to feed her but noticed milk coming out of her nose and difficulty breathing during feeding. Upon examination, the infant was found to be tachypneic and tachycardic with intercostal recession. A bulge was observed on the praecordium and an early systolic murmur was heard along the left sternal edge. The chest x-ray revealed cardiomegaly, a loss of the normal thymus shadow, and a right aortic notch. Blood tests were normal except for low corrected serum calcium. What is the most likely diagnosis?
Your Answer: Turner's Syndrome
Correct Answer: DiGeorge syndrome
Explanation:DiGeorge Syndrome
DiGeorge syndrome is a genetic disorder caused by a deletion of chromosomal region 22q11.2. It affects around 1 in 3000 live births and is characterized by a spectrum of disorders. The poor migration of neural crest cells to the third and fourth pharyngeal pouches is thought to be the cause of the midline abnormalities found in DiGeorge syndrome. These abnormalities include cardiac defects, abnormal facies, thymic hypoplasia, and hypocalcemia.
Characteristic facies develop as the child grows and include high broad noses, low set ears, small teeth, and narrow eyes. Other systems may also be affected, and cognitive and psychiatric problems are common but variable. Around 80% of patients have an associated cardiac defect, often of a conotruncal variety. Tetralogy of Fallot is also found, as are other defects such as ventricular septal defects and atrial septal defects.
The diagnosis of DiGeorge syndrome can be difficult, but clinical features consistent with the diagnosis include abnormalities of heart sounds and features of cardiac failure or cyanosis. The chest x-ray helps with the diagnosis, and an echocardiogram and possibly high resolution contrast CT imaging would be helpful in these cases. The above example has a persistent truncus arteriosus, which is a failure to separate the aorta and the main pulmonary artery. This can lead to dyspnea, cyanosis, and cardiac failure.
In summary, DiGeorge syndrome is a complex disorder that affects multiple systems in the body. Early diagnosis and management are crucial to prevent complications and improve outcomes for affected individuals.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 22
Correct
-
Which of the following does not result in feeding challenges during the neonatal phase?
Your Answer: Physiological jaundice
Explanation:Feeding Difficulty and Physiological Jaundice
Feeding difficulty is a common problem among infants, but it is not associated with physiological jaundice. Physiological jaundice is a benign condition that is short-lived and does not generally cause any symptoms. This means that it is not related to feeding difficulties that infants may experience.
It is important for parents to be aware of the signs of feeding difficulty in their infants, such as difficulty latching, poor weight gain, and excessive crying during feeding. These symptoms may indicate an underlying medical condition that requires prompt attention. On the other hand, physiological jaundice is a normal occurrence in many newborns and typically resolves on its own without any treatment.
In summary, while feeding difficulty is a common problem among infants, it is not associated with physiological jaundice. Parents should be aware of the signs of feeding difficulty and seek medical attention if necessary, but they can rest assured that physiological jaundice is a benign condition that does not generally cause any symptoms.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 23
Incorrect
-
A 14-month-old boy is brought to the children's emergency department by his parents who report loss of consciousness and seizure activity. Paramedics state that he was not seizing when they arrived. He has a temperature of 38.5ºC and has been unwell recently. His other observations are normal. He has no known past medical history.
After investigations, the child is diagnosed with a febrile convulsion. What advice should you give his parents regarding this new diagnosis?Your Answer: Febrile convulsions are not a risk factor for epilepsy
Correct Answer: Call an ambulance only when a febrile convulsion lasts longer than 5 minutes
Explanation:Febrile convulsions are a common occurrence in young children, with up to 5% of children experiencing them. However, only a small percentage of these children will develop epilepsy. Risk factors for febrile convulsions include a family history of the condition and a background of neurodevelopmental disorder. The use of regular antipyretics has not been proven to decrease the likelihood of febrile convulsions.
Febrile convulsions are seizures caused by fever in children aged 6 months to 5 years. They typically last less than 5 minutes and are most commonly tonic-clonic. There are three types: simple, complex, and febrile status epilepticus. Children who have had a first seizure or any features of a complex seizure should be admitted to pediatrics. Regular antipyretics do not reduce the chance of a febrile seizure occurring. The overall risk of further febrile convulsion is 1 in 3, with risk factors including age of onset, fever duration, family history, and link to epilepsy. Children without risk factors have a 2.5% risk of developing epilepsy, while those with all three features have a much higher risk.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 24
Incorrect
-
A 4-year-old patient is brought to the GP by their mother due to a high fever and sore throat. Upon examination, the child appears comfortable but feverish, with a rash on both arms that is more pronounced in the cubital fossas. The lesions are rough and erythematosus in texture, and the throat and tongue are both red in color. The child has no prior medical history. What is the appropriate course of action for this case?
Your Answer: Prescribe oral azithromycin for 5 days
Correct Answer: Prescribe oral penicillin V for 10 days
Explanation:The recommended treatment for scarlet fever in patients who do not require hospitalization and have no penicillin allergy is a 10-day course of oral penicillin V. This condition is characterized by symptoms such as fever, sore throat, strawberry tongue, and a rash that is more prominent in the cubital fossas. Scarlet fever is caused by erythrogenic toxins produced by Group A haemolytic streptococci, and if left untreated, it can lead to complications such as otitis media and rheumatic fever. Administering varicella-zoster immunoglobulin is not appropriate for this condition. Prescribing analgesia and asking the patient to return in 5 days for review is also not recommended, as antibiotics should be given as soon as possible to prevent complications. Oral azithromycin for 5 days is not the first-line treatment for scarlet fever, and co-amoxiclav is not indicated for this condition.
Scarlet fever is a condition caused by erythrogenic toxins produced by Group A haemolytic streptococci, usually Streptococcus pyogenes. It is more prevalent in children aged 2-6 years, with the highest incidence at 4 years. The disease spreads through respiratory droplets or direct contact with nose and throat discharges, especially during sneezing and coughing. The incubation period is 2-4 days, and symptoms include fever, malaise, headache, nausea/vomiting, sore throat, ‘strawberry’ tongue, and a rash that appears first on the torso and spares the palms and soles. The rash has a rough ‘sandpaper’ texture and desquamation occurs later in the course of the illness, particularly around the fingers and toes.
To diagnose scarlet fever, a throat swab is usually taken, but antibiotic treatment should be initiated immediately, rather than waiting for the results. Management involves administering oral penicillin V for ten days, while patients with a penicillin allergy should be given azithromycin. Children can return to school 24 hours after commencing antibiotics, and scarlet fever is a notifiable disease. Although usually a mild illness, scarlet fever may be complicated by otitis media, rheumatic fever, acute glomerulonephritis, or rare invasive complications such as bacteraemia, meningitis, or necrotizing fasciitis, which may present acutely with life-threatening illness.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 25
Correct
-
A 4-year-old child was admitted with a high fever, cervical lymph node enlargement, conjunctival congestion, redness of lips and palms, and desquamation of fingertips. Upon examination, erythema of the oral cavity was observed. Blood tests showed a hemoglobin level of 110 g/l and a platelet count of 450,000. The symptoms resolved after two weeks, but during the third week, the child unexpectedly passed away. An autopsy revealed vasculitis of the coronary arteries and aneurysm formation. What is the most likely diagnosis?
Your Answer: Kawasaki’s disease
Explanation:Understanding Kawasaki’s Disease and Differential Diagnosis
Kawasaki disease, also known as mucocutaneous lymph node syndrome, is a multisystem disease that primarily affects children under the age of 5. It is characterized by fever, cervical adenitis, and changes in the skin and mucous membranes. While generally benign and self-limited, it can lead to coronary artery aneurysms in 25% of cases and has a case-fatality rate of 0.5-2.8%. Treatment with high-dose intravenous globulin and aspirin has been shown to be effective in reducing the prevalence of coronary artery abnormalities.
When considering a differential diagnosis, it is important to distinguish Kawasaki disease from other conditions with similar symptoms. Scarlet fever, rheumatic fever, diphtheria, and Marfan syndrome can all present with fever and cardiovascular involvement, but each has distinct clinical features and underlying pathophysiologic mechanisms. Careful evaluation and diagnosis are essential for appropriate treatment and management.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 26
Correct
-
A 4-year-old boy, with a history of acute lymphoblastic leukaemia (ALL), is admitted to the Paediatric Ward due to a fever of 38.9 °C at home. He received his last treatment 1 week ago. His parents report that he has been feeling well, without cough, cold, rash, or vomiting.
Upon examination, his vital signs are stable except for a temperature of 38.4 °C. Physical examination is unremarkable. Blood tests and blood culture are taken from the portacath, revealing a haemoglobin (Hb) level of 11.5, a white cell count (WCC) of 1.1, neutrophils of 0.2, and a C-reactive protein (CRP) level of 85.
What is the most crucial next step in managing this patient?Your Answer: Start intravenous (iv) Tazocin®
Explanation:Management of Febrile Neutropenia in a Child with ALL Receiving Chemotherapy
Febrile neutropenia is a serious complication in cancer patients, particularly those receiving chemotherapy. It is defined as neutropenia with fever or symptoms of significant sepsis. In such cases, immediate initiation of broad-spectrum intravenous antibiotics is crucial to prevent mortality.
In this scenario, a child with acute lymphoblastic leukemia (ALL) presents with febrile neutropenia. The first step is to start intravenous Tazocin® monotherapy, pending blood cultures and a thorough physical examination. A urine sample should also be obtained, particularly for children under the age of 5. Chest radiography is only necessary for symptomatic children.
After 48 hours, if the child shows improvement and cultures are negative, empirical antibiotic treatment can be discontinued or switched to oral antibiotics such as Augmentin®. Intravenous fluids may be necessary if the child deteriorates or becomes haemodynamically unstable.
It is also important to send urine and stool samples for microbial culture and sensitivity (MC&S) to investigate sepsis. However, initiating antibiotics should be the first priority.
In summary, febrile neutropenia in cancer patients is a medical emergency that requires prompt initiation of broad-spectrum antibiotics. Close monitoring and appropriate investigations are necessary to ensure timely and effective management.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 27
Correct
-
A toddler is brought to the emergency department after a near-drowning incident in a swimming pool. The child is unresponsive and requires intubation for airway management. Upon examination, the child is found to have global hypotonia and abnormal reflexes, indicating possible hypoxic brain injury. What is the most crucial intervention for this child?
Your Answer: Therapeutic cooling
Explanation:The most important intervention for reducing the likelihood of significant hypoxic-ischaemic brain injury in neonates with poor neurological status following a traumatic delivery and acidosis is therapeutic cooling at 33-35 degrees. This approach attempts to prevent severe brain damage. The use of sodium bicarbonate correction, blood transfusion, skin to skin contact with mum, and vitamin K are not essential or effective in reducing brain damage from hypoxic injury.
Therapeutic Cooling for Neonates with Hypoxic Brain Injury
Therapeutic cooling, also known as therapeutic hypothermia, is a medical procedure that involves deliberately lowering a patient’s body temperature to cool the brain and prevent brain damage. This procedure has been proposed for several therapeutic uses, including neuroprotection during open-heart and neurosurgical procedures, in patients following a return of spontaneous circulation post-ventricular fibrillation arrest, in patients with trauma head injuries, in patients who have suffered acute ischemic stroke, and in neonates with moderate to severe hypoxic ischemic encephalopathy (HIE). However, the only use whose efficacy has been consistently proven in existing literature is the use of therapeutic cooling in neonates.
The use of therapeutic cooling in carefully selected term neonates with moderate to severe HIE has been recommended as standard care by the National Institute for Health and Care Excellence (NICE). It has been shown in studies to decrease mortality and improve the neurological and neurodevelopmental outcomes of treated neonates. Hypoxic perinatal brain injury is caused by a decrease in the amount of oxygen supplied to an infant’s brain just prior to, or during the process of, labor. Neonates who survive a hypoxic brain injury can develop HIE, which occurs in an estimated 2.5/1000 term births in developed countries and 26/1000 term births in the developing world.
Therapeutic cooling remains the only intervention shown to reduce neuronal damage caused by perinatal hypoxia. The procedure is thought to influence the extent of secondary neuronal death in a multifactorial manner, although the exact way in which it achieves this is not fully understood. The procedure involves placing the infant on a cooling blanket or mattress circulated with coolant fluid or circulating cold water in a cap fitted around the head. Temperature is continuously monitored throughout the treatment using either a rectal or nasopharyngeal thermometer. Close surveillance of infants during the cooling process is required given the risk for complications of both HIE and the process of cooling itself.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 28
Correct
-
Which of the following statements is true regarding X-linked recessive inheritance?
Your Answer: A female child of a heterozygous female carrier has a 50% chance of being a carrier
Explanation:Understanding X-Linked Recessive Inheritance
X-linked recessive inheritance is a genetic pattern where only males are affected, except in rare cases such as Turner’s syndrome. This type of inheritance is transmitted by heterozygote females, who are carriers of the gene mutation. Male-to-male transmission is not observed in X-linked recessive disorders. Affected males can only have unaffected sons and carrier daughters.
If a female carrier has children, each male child has a 50% chance of being affected, while each female child has a 50% chance of being a carrier. It is important to note that the possibility of an affected father having children with a heterozygous female carrier is generally rare. However, in some Afro-Caribbean communities, G6PD deficiency is relatively common, and homozygous females with clinical manifestations of the enzyme defect are observed.
In summary, X-linked recessive inheritance is a genetic pattern that affects only males and is transmitted by female carriers. Understanding this pattern is crucial in predicting the likelihood of passing on genetic disorders to future generations.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 29
Correct
-
A worried 28-year-old mother expresses concern about a lump that has emerged on her infant's head, stating with certainty that it was not present at birth and was only noticed half an hour ago. The baby was delivered vaginally with forceps 10 hours ago at 39 weeks gestation.
Upon examination, there is a clearly defined, soft swelling that does not cross suture lines and is located on the parietal bone. The baby appears to be in good health otherwise, with normal skin color and tone, as well as regular vital signs.
What is the most probable diagnosis?Your Answer: Cephalhaematoma
Explanation:The most likely diagnosis for the newborn baby’s well-demarcated, fluctuant swelling that appeared two days after birth is cephalohaematoma. This is a haemorrhage between the skull and periosteum that occurs when blood vessels crossing the periosteum rupture. The swelling is limited by the individual bones and does not cross suture lines. It may take months to resolve as the blood clot is reabsorbed. Cephalohaematoma is more common in prolonged and/or instrumental deliveries and may lead to jaundice, anaemia or hypotension if severe. It may also indicate a linear skull fracture or risk of infection. Caput succedaneum, chignon, normal skull shape, and subgaleal haemorrhage are less likely diagnoses.
A cephalohaematoma is a swelling that appears on a newborn’s head, usually a few hours after delivery. It is caused by bleeding between the skull and periosteum, with the parietal region being the most commonly affected site. This condition may lead to jaundice as a complication and can take up to three months to resolve.
In comparison to caput succedaneum, which is another type of swelling that can occur on a newborn’s head, cephalohaematoma is more localized and does not cross suture lines. Caput succedaneum, on the other hand, is a diffuse swelling that can cross suture lines and is caused by fluid accumulation in the scalp tissue. Both conditions are usually harmless and resolve on their own, but medical attention may be necessary in severe cases.
-
This question is part of the following fields:
- Paediatrics
-
-
Question 30
Correct
-
A 5-year-old patient is referred to the Paediatric Unit after having presented to her General Practitioner (GP) twice over the course of the week with fever and a red tongue and throat. A course of penicillin V has so far been ineffective. She is fully immunised and has not been in contact with any other children with notifiable infectious diseases. Her parents report that she has not been well for around 7 days with a high fever, which they have been unable to bring down with paracetamol and ibuprofen. She has had a cough with coryzal symptoms and has now developed conjunctivitis.
On examination, she looks unwell. Temperature is 38 °C, heart rate 124 bpm and respiratory rate 28. Capillary refill time is 2 s centrally. She has bilateral conjunctivitis. She has a red oropharynx and a red tongue. There is cervical lymphadenopathy and a widespread maculopapular rash. Her hands and feet are red and there is some peeling of the skin around the toes.
Which of the following diagnoses should be made?Your Answer: Kawasaki disease
Explanation:The child in question is suffering from Kawasaki disease, a febrile vasculitis that affects small to medium-sized arteries and primarily affects children under the age of 5, with males being more commonly affected. Symptoms include sudden-onset fever lasting at least 5 days, nonexudative conjunctivitis, polymorphous rash, lymphadenopathy, mucositis, and cardiovascular manifestations such as coronary artery aneurysms. Diagnosis is based on the presence of fever lasting for >5 days and at least four or five of the following: bilateral conjunctivitis, changes in the lips and oral mucosal cavities, lymphadenopathy, polymorphous rash, and changes in the extremities. Treatment involves inpatient care, intravenous immunoglobulins (IVIG), aspirin, and monitoring of cardiovascular function. If left untreated, Kawasaki disease can lead to arterial aneurysms and congestive heart disease. Other potential causes of the child’s symptoms, such as Coxsackie infection, measles, viral upper respiratory tract infection with exanthema, and scarlet fever, have been ruled out based on the child’s symptoms and medical history.
-
This question is part of the following fields:
- Paediatrics
-
00
Correct
00
Incorrect
00
:
00
:
0
00
Session Time
00
:
00
Average Question Time (
Secs)