paeds wk 2

Question 1

A 45 year old mother missed most of her antenatal scans and check ups. She gave birth to her son yesterday in an uncomplicated delivery. However, during feeding she has noted that her son often tires easily and becomes disinterested in feeding any longer. Then this morning she became very worried as he appeared to turn very blue especially in the face.
Given the probable cause of these symptoms what would be the best investigation to confirm the diagnosis?

Answer 1

Echocardiogram
Correct. ECHO should be ordered in any newborn with a suspected diagnosis of congenital heart disease. Echocardiography is the definitive investigation for diagnosis of TOF

Question 2

A 7-year-old girl is brought into the GP by her mother, as she is concerned about her daughters growth. She has noticed that she is noticeably shorter than her other classmates.
On clinical examination, her height is measured to be in the 0.4th centile, and weight in the 50th centile. Widely spaced nipples and a low hairline are noted.
Which of the following treatments may commenced?

Answer 2

Somatotropin, or recombinant human growth hormone, is used to treat children with growth hormone deficiency or certain genetic conditions that affect growth, such as Turner syndrome. Given the child’s significantly low height percentile and clinical features suggestive of Turner syndrome, growth hormone therapy is an appropriate treatment to help increase growth rate and improve final adult height.

Question 3

A 2 month old baby boy is seen by the GP because of difficulty breastfeeding and poor weight gain.
The mother reports that although the baby has a good latch and a strong suck, he gets really tired and out of breath after a few minutes of feeding. This has been getting worse since he was about a week old. He was born by spontaneous vaginal delivery at 39+6 weeks after a normal pregnancy and antenatal scans.
On inspection, he is active, alert and smiley. There is a pan-systolic murmur loudest at the lower left sternal edge. The lung fields are clear to auscultation. His abdomen is soft with no organomegaly. His temperature is 36.9 degrees. Plotting his weight on his growth chart shows he has dropped two centiles between 6 and 8 weeks of life.
Which of the following is the likely management for the underlying condition?

Answer 3

The description suggests a small ventricular septal defect (VSD), which is the most common congenital heart defect. The pan-systolic murmur at the left sternal edge without significant symptoms such as respiratory distress or organomegaly often indicates a small VSD. Many small VSDs close spontaneously as the child grows, and in such cases, ongoing monitoring and reassurance are appropriate. The fact that the baby is alert, smiley, and not in respiratory distress supports this conservative approach.

Question 4

An 8-year-old boy is brought to the GP by his mother due to a 2 day history of a runny nose, sore throat and cough. He feels okay otherwise and is normally well. He has a past medical history of Down’s syndrome.
The boy has a respiratory rate of 20 and oxygen saturations of 100%. His throat is pink with no swellings or exudate and his tympanic membranes are visible with no bulging or effusion. A soft systolic murmur is heard at the left sternal edge which does not radiate and there is no thrill on palpation. He has never been noted to have a murmur before.
What is the most appropriate management?

Answer 4

Review again in 2 weeks
This patient is presenting to the GP with a common cold and a soft systolic murmur at the left sternal edge. This is most likely to be an innocent murmur. Innocent murmurs are very common in children (up to 30% of children at some point), and can be precipitated by febrile illness. As this child currently has a mild viral infection, the best option is to review the murmur in a few weeks once the infection has resolved, to see if the murmur has disappeared with the infection. The infection is likely to be a mild viral upper respiratory tract infection, as evidenced by a lack of tonsillar exudate, angry red throat and no sign of ear infection (bulging tympanic membrane with effusion)

Question 5

when Abx in tonsillitis

Answer 5

centor 3+

Question 6

what to use instead of phenoxymethylpenicillin in tonsillitis

Answer 6

macrolide - azithromycin

Question 7

A 2-year-old child presents with poor feeding, poor weight gain, and cold extremities. On examination, you notice a weak or absent femoral pulse and a difference in blood pressure between the upper and lower limbs.
What is the most likely diagnosis?

Answer 7

aortic coarctation

Question 8

A 1 year old girl is brought in to A&E by her parents as they are concerned about her breathing. She has been feeling unwell with the flu-like symptoms over the last few days. The parents describe a barking cough. They think she has had all her immunisations. She has a high grade fever. A constant high-pitched sound on inspiration can be heard and she has a hoarse voice.
Humidified oxygen, dexamethasone and nebulised adrenaline is given. The symptoms do not improve.
What is the most likely diagnosis?

Answer 8

Always consider bacterial tracheitis in a barking cough with continuous stridor that does not resolve

Question 9

A 4 year old boy is brought by his parents to the GP practice as they are concerned that there is discharge from his right ear. For the last two days, he has been irritable and tugging on the outside of his ear. He has just recovered from a recent upper respiratory tract infection (URTI). He is afebrile and is alert and active.
On otoscopy, there is a perforated tympanic membrane on the right.
What is the most appropriate management?

Answer 9

amoxicillin. no ENT referral needed unless recurrent

Question 10

most common cardiac defect

Answer 10

The most common congenital cardiac defect is a ventricular septal defect, accounting for about 30-60% of all congenital heart defects

VSD = pan systolic (ASD would be ej sys with fixed split second heart sound)

Question 11

You are an FY2 in Paediatrics working on the neonatal unit. A 3 day old premature baby boy, born at 34 weeks’ gestationn by emergency C-section, has had several episodes where he has stopped breathing.
The baby has started breathing again spontaneously after about 10-15 seconds. These episodes have been accompanied by bradycardia. He has been on a ventilator since delivery, and attempts to wean his ventilation have not been successful. On examination, the pulse is bounding and there is a continuous machine-like murmur heard loudest at the upper left sternal edge.
Which of the following medications would help with this presentation?

Answer 11

This baby with a collapsing pulse, continuous machine-like murmur and apnoeic episodes with bradycardia most likely has a persistent ductus arteriosus. If symptomatic, this can be managed medically with a prostaglandin synthesis inhibitor (NSAID) like indomethacin, causing vasoconstriction to close the duct

Prostaglandin would help to keep the ductus arteriosus open, which may be necessary in ductus-dependent congenital cardiac disease

Question 12

A 3-week-old girl presents to A&E with episodes of grunting. Her parents report three episodes of grunting and irritability. She was born prematurely at 30 weeks and has spent 2 weeks in the Neonatal Intensive Care Unit at birth. There is no significant family history.
On examination, she has signs of increased work of breathing. There is nasal flaring and intercostal recession; there is no cyanosis. On auscultation of her chest, a continuous murmur is heard that is loudest in the second intercostal space on the left side. Her observations are all in normal range.
What is the most appropriate management?

Answer 12

The patient has presented with a patent ductus arteriosus (PDA). She has non-specific symptoms of poor respiratory status. Infants can also present with difficulty feeding and irritability. She also has strong risk factors including prematurity and female sex. Other risk factors include neonatal respiratory distress syndrome, family history and maternal rubella. The murmur is the strongest element of the history that suggests PDA. PDA is a congenital heart defect that results in an abnormal connection between the pulmonary trunk and the descending aorta. The initial management in premature infants is oral indomethacin or ibuprofen.

Question 13

turners heart issues

Answer 13

aortic coarctation and bicuspid aortic heart valve

Question 14

diabetic mother most common heart problem in baby

Answer 14

The most common form of congenital heart defect in infants of diabetic mothers is transposition of the great vessels

Question 15

most common heart block in paeds

Answer 15

complete (third degree)

Question 16

emergency fluid bolus in children

Answer 16

10ml per kilo stat over 10-15 mins

Question 17

meningococcal sepsis mx

Answer 17

ceftriaxone 100ml per kilo

Question 18

A 6-month-old female infant is found to have a heart murmur at a routine 6-month baby check. She has previously been healthy, apart from infantile colic. Her weight is on the 25th percentile and height is on the 10th percentile.
She is not cyanosed, has no chest deformity, and her peripheral pulses are normal. There is a palpable thrill at the left sternal edge, and the apex beat is palpable in the fifth intercostal space on the midclavicular line. A grade 4/6 systolic murmur is audible at the left sternal edge, which is radiating to the left axilla and to the back. There is no variation with respiration or posture.
What is the most likely diagnosis of her presentation?

Answer 18

The presence of a thrill with a murmur always indicates a pathological lesion and in combination with the nature, quality and radiation of the murmur as described negates a benign flow murmur as the cause. A thrill is usually not felt with lesions less than grade 4 out of 6 intensity. A ventricular septal defect is a congenital hole in the septum between the two ventricles. It is commonly associated with an underlying genetic condition, Down syndrome or Turner syndrome. VSDs are initially symptomless, and patients can present as late as adulthood. Initially, the shunt is from the left to the right ventricle due to high pressure on the left side. Over a while, after the onset of pulmonary hypertension, increased right-sided pressure causes a right-to-left shunt, known as Eisenmenger syndrome. Large VSDs require surgical correction with transvenous catheter closure via the femoral vein or open-heart surgery.

The murmur of atrial septal defect is a soft mid-systolic murmur at the left upper sternal edge due to the increased flow of blood over the normal pulmonary valve due to the shunt at the atrial level.

Question 19

degree of cyanosis in ToF is determined by

Answer 19

Of the four features, it is the degree to which the RV outflow tract is obstructed that is the greatest determinant of the magnitude of the shunt, and accordingly, the degree of cyanosis

pulmonary stenosis

Question 20

A 3 month old boy, corrected for gestational age, born in May, with trisomy 21 has a ventricular septal defect. He is currently on a high-calorie nastrogastric feed and furosemide.
What prophylactic therapy is indicated for this patient?

Answer 20

This is a premature boy under the age of six months (corrected) approaching bronchiolitis season. He has significant acyanotic heart disease, requiring furosemide to prevent from him being symptomatic. He meets the indications for RSV prophylaxis. Palivizumab is a monoclonal antibody that minimises the risk of infection by RSV. It is given subcutaneously once a month during bronchiolitis season

Question 21

Kawasaki fever

Answer 21

5 days

Question 22

which drug is not good in long QT syndrome

Answer 22

Clarithromycin
Macrolides are a drug class associated with prolonging the QT interval.

Question 23

Turners hormonal levels

Answer 23

Females diagnosed with Turner syndrome will present with primary amenorrhoea and underdeveloped ovaries (streak ovaries). FSH levels are elevated due to the lack of negative feedback from the decreased oestrogen production from the ovaries.

Question 24

abnormality in PDA

Answer 24

A collapsing pulse arises from the increased pressure gradients and a continuous murmur from turbulent flow across the ductus arteriosus.

Question 25

A junior doctor is asked to review a neonate on the postnatal ward. He was born 10 hours ago and has suddenly turned blue and developed an increased work of breathing. Upon examination, he has central cyanosis, he is not tachypnoeic, there are no additional breath sounds, and on auscultation of the heart there are no murmurs. A chest x-ray shows cardiomegaly. He has been given Oxygen by the nurses but this has not improved the cyanosis.
Which of the following is the next best step in the management of this patient?

Answer 25

Alprostadil

This is a case of duct dependent congenital heart disease, which is managed with prostaglandins. This is to promote patency of the ductus arteriosus, which begins to close shortly after birth and is the cause of cyanosis in this situation. This will allow the infant to survive long enough to allow investigations to confirm the diagnosis, which is most likely transposition of the great arteries, and thus surgical correction

Question 26

A mother brings in her neonate to the GP for her 6-week check. The mother has a past medical history of bipolar disorder, and gave birth via spontaneous vaginal delivery at 38 weeks with no complications. The GP hears a pan-systolic murmur on auscultation and so is referred for an echocardiogram. Cardiac echocardiogram shows an enlarged right atrium, a small right ventricle and tricuspid incompetence.

Answer 26

Ebstein’s anomaly
This is correct. The mother suffers from bipolar disorder and so is most likely taking Lithium monotherapy which, in pregnancy, can cause a congenital heart defect known as Ebstein’s anomaly. This is characterised by a large right atrium and small right ventricle, usually due to low insertion of the tricuspid valve, which also causes tricuspid incompetence

Question 27

An 8-year-old boy is brought to the Emergency with facial redness and swelling. He is not complaining of any other symptoms and feels generally well.
On examination, his physical observations are normal. You note redness and swelling around the right eye, which is hot to touch. The eye movements are normal with no pain and no decrease in visual acuity. Chest and abdominal examinations are normal.
What is the most appropriate management for the likely diagnosis?

Answer 27

Oral antibiotics and follow up
The likely diagnosis is peri-orbital cellulitis (also known as pre-septal cellulitis). In a generally well child, with no suspicion of orbital cellulitis, it would be appropriate to start the patient on oral antibiotics, provided regular close follow up can be arranged and followed.

Question 28

A 23 month old boy is brought to the general practice by his mother with a one-day history of fever. His mother reports noticing him rubbing his ears over the past two days. He has no past medical history except for a recent viral upper respiratory tract infection. His temperature is measured at 37.3 degrees Celsius. On examination, he looks clinically well and stable. Otorrhoea is noted bilaterally and there is no evidence of rash, and both Kernig’s and Brudzinski’s signs are negative.
Based on the most likely diagnosis, what is the best management plan for this patient?

Answer 28

Offer amoxicillin
The statement described above is most suggestive of a diagnosis of bilateral otitis media. The patient is younger than 2 years old, hence a 5-7 day course of amoxicillin should be offered to the patient.