the same file

1-. A 5-month-old boy presents with florid red rash over his scalp, nappy area and trunk.

Despite this he seems well in himself and feeding well. He was born at term weighing 3.1 kg and there were no neonatal problems. He is fully immunised and there is no FH/SH of note.

On examination he is apyrexial and well. The rash is florid red and confluent over his nappy area. He has a crusty confluent covering over his scalp, extending onto his forehead. Smaller 0.5-1 cm greasy lesions are present over his trunk.

What is the most likely diagnosis?

(Please select 1 option)

Acrodermatitis enteropathica Ammoniacal dermatitis Histiocytosis Infantile eczema Seborrhoeic dermatitis Correct

The picture is of extensive greasy scaly rash especially over the head (cradle cap) and nappy area without systemic upset.

This is highly characteristic of seborrhoeic dermatitis.

Selenium shampoo and topical steroids usually result in rapid resolution.

2- A 3-year-old girl presents with daily high fever, rash and joint pain of six weeks duration.

The rash appears at the height of fever. The knees, ankles and wrists are affected.

She had a full term normal delivery with no neonatal problems. Her immunisations are up to date. There is no family or social history of note.

On examination the temperature is 38.3°C, and she has a florid erythematous rash over the trunk, face and limbs. There is generalised lymphadenopathy, with 3 cm liver and 3 cm spleen palpable. The affected joints are warm, swollen, tender and limited in movement.



Henoch-Schönlein purpura Kawasaki disease Rheumatic fever Septic arthritis Systemic juvenile arthritis Correct

The duration and degree of joint inflammation suggest a chronic arthritis.

The systemic symptoms of fever and rash make the most likely diagnosis systemic juvenile idiopathic arthritis (Still's disease).

3- An 18-year-old woman presents with red, tender lumps on her shins and arthralgia.

Chest x ray shows bilateral hilar lymphadenopathy and clear lung fields. A clinical diagnosis of sarcoidosis is made.

Which one of the following is the most appropriate management plan?


24 hour urinary calcium measurement Incorrect answer selected Follow up appointment with chest x ray in three months This is the correct answer Mediastinoscopy and lymph node biopsy Skin biopsy Thoracic CT scan

This is also known as Löfgren's syndrome (a benign form of sarcoidosis).

The presentation of erythema nodosum, arthropathy, bilateral hilar lymphadenopathy syndrome is so characteristic that histological diagnosis is not necessary.

The prognosis is excellent with less than 10% having persistent disease.

With less characteristic presentations, positive biopsies are needed.

Mediastinoscopy is the method of choice for anterior mediastinal nodes.

4-A 12-year-old boy presents with a florid rash on the hands and feet. He became ill three days before, when he developed painful mouth ulcers. Yesterday he developed a rash on the hands and feet. He was a full term normal delivery, and previously has been very healthy. He is on no medications, is fully immunised, and there is no family history of note.

On examination he has a temperature of 38.5°C, respiratory rate 15/min and heart rate is 85/min. He has profuse oral ulcers on his tongue and buccal mucous membranes. He has many circular raised pinky red lesions over the hands and feet with darker centres. He has tender cervical lymphadenopathy.



Erythema multiforme This is the correct answer Hand, foot and mouth disease Infectious mononucleosis Incorrect answer selected Kawasaki disease Staphylococcal scalded skin syndrome

The history suggests an attack of oral ulceration, followed by the development of target lesions over the extremities.

The likely diagnosis is primary herpes 1 infection, complicated by erythema multiforme.

Treatment is supportive, though acyclovir may be given to control the herpes.

5- A 10-month-old girl is referred with a florid skin rash.

Soon after birth she developed cradle cap and dry skin on the cheeks. This gradually improved, but over the past few months she has had dry red skin over the trunk, the elbows, wrists, knees, ankles and base of the ears. This is itchy and disturbs sleep.

She was born at 37+3/40 weighing 3.34 kg and there were no other neonatal problems. She has been fully immunised. Mother has hay fever and asthma.

On examination she is on the 10th centile for height, weight and OFC. Temperature is 36.4°C, RR 30/min and HR 100/min. She has a dry red scaly rash on patches of the trunk, elbows, wrists, ankles and knees. She has enlarged glands in the axillae and groins.



Atopic dermatitis Correct Contact dermatitis Erythema multiforme Impetigo Stevens-Johnson syndrome

The type, distribution and progress of the rash, in conjunction with the family history, suggest atopic eczema.

This is treated with moisturisation and topical anti-inflammatories (steroids), the strength of the latter being titrated against effect.

Irritants and desiccants should be avoided.

6-. A 4-year-old boy presents with a perioral rash. This has been present for many months, but is not resolving.

He was born at term and there were no neonatal problems. He has been well and thriving on no medications. He is fully immunised to date and there is no FH/SH of note.

On examination his temperature is 36.9°C, HR 95/min, RR 20/min. He has dry flaky skin around his mouth, which he licks frequently.



Contact dermatitis Correct Erythema multiforme Impetigo Stevens-Johnson syndrome Viral exanthems

The history of local dryness associated with licking is a form of contact dermatitis.

Recurrent contact with irritant, such as saliva, bubble bath, detergents, soaps and citrus juices inflame the skin. Sweat may result in a similar foot problem.

Treatment is with emollients and avoidance of the trigger.

7- A 7-month-old girl presents with fever and a rash.

She was completely well until five days ago, when she developed a slight cold. The next day she developed fever to 39.7°C, which has persisted despite antipyretics. Despite this she has remained relatively well and continues to drink, though her appetite is poor. Today she has developed a rash over the face and trunk.

She was born at term weighing 3.8 kg and there were no neonatal problems. She is fully immunised to date and there is no FH/SH of note.

On examination she has a temperature of 36.8°C, RR 25/min and HR 100/min. The rash is macular, profuse, pink and blanching. It is most prominent over the face and trunk. She has shotty cervical lymphadenopathy.



Contact dermatitis Erythema multiforme Impetigo Stevens-Johnson syndrome Viral exanthems Correct

The history of a well child with high fever for a few days followed by resolution of fever at around the time of appearance of a rose-coloured rash is characteristic of roseola infantum.

Since the introduction of measles, mumps, rubella (MMR), this is by far the commonest cause of a measles-like rash.

The peak incidence is six to eighteen months. 5% develop febrile seizures.

It is caused by human herpes virus 6 and 7.

8- A 2-week-old girl presents with a history of poor feeding, worse over the last six hours. 38+3/40, 2.95 kg, no problems at birth. First pregnancy of healthy Caucasian mother. No FH/SH of note. No drugs nor immunisations.

On examination temperature is 35.4°C (tympanic), RR 60/min, HR 160/min on monitor (all pulses impalpable). Cold mottled peripheries, capillary refill time six seconds. Oxygen saturations 45% in air. Gallop rhythm, 4 cm hepatomegaly, no spleen. No bruising or rash.



Congenital adrenal hyperplasia Escherichia coli septicaemia Galactosaemia Group B streptococcal infection Hypoplastic left heart syndrome Correct

The history is of shock, hepatomegaly and hypoxia in a 2-week-old child.

The most likely diagnosis is duct-dependent congenital heart disease. Left heart lesions causing this presentation include:

aortic coarctation/hypoplastic arch truncus arteriosus critical aortic stenosis hypoplastic left heart syndrome.

Right-sided lesions include:

transposition severe Fallot's pulmonary atresia +/- VSD.

After intubation and ventilation the key step is to open the duct using prostaglandin E2 infusion.

Acidosis may require separate correction.

Urgent transfer to a cardiac centre should then follow so a specific diagnosis can be made.

9-A young child is noted to have a platelet count of 50 x109/L (150-400 x109). Which one of the following condtions may explain the thrombocytopenia?


Disseminated intravascular coagulation Correct Henoch-Schonlein purpura Kawasaki disease Leukaemia in remission Vitamin K deficiency

The platelet count will be reduced in acute leukaemia due to bone marrow infiltration by blasts, but is normal in remission.In Kawasaki disease there will be thrombocytosis. Disseminated intravascular coagulation is characterised by low platelets and abnormal clotting profile. In Henoch-Schonlein purpura there will be normal clotting profile and platelet count. Vitamin K deficiency causes prolonged Prothrombin time and no thrombocytopenia.

10-A 7-year-old boy presents with swelling under his arm. He was well until 7day before, when he developed mild fever and malaise. Full term normal delivery, no neonatal problems. Immunisations up to date. No family history of note. The family have a cat.

On examination the temperature is 37.9C. He has a 3cm smooth enlargement of a node in the right axilla. This is slightly red but not fluctuant. Otherwise there are no abnormalities to find.



Cat scratch disease Correct Hodgkin lymphoma Infectious mononucleosis Kawasaki disease Mycobacterial adenitis

The history suggests subacute regional gland enlargement due to inflammation. The most likely diagnosis is cat-scratch disease, which is caused by Bartonella henselae. The incubation period is 3-30 days and often small erythematous lesions can be found along the scratch marks. Regional adenopathy develops after 1-4 weeks.

11-The following statements are true of pertussis:

True / False

It can occur in the first three months of life Correct Absence of a whoop rules out the diagnosis in a child with a cough Correct A lymphocytic leucocytosis should be demonstrated to confirm the diagnosis

Correct Bronchiolitis obliterans is a recognised later complication Incorrect answer selected Ampicillin is the drug of choice to eradicate the infection Incorrect answer selected

Pertussis can occur at any time in a child without immunity to the condition who is exposed. The whoop may not be seen and apnoeic episodes can occur as a feature. Although a lymphocytosis is observed the diagnosis is confirmed by culture of the organism in nasal secretions. There is no reported association between bronchiolitis obliterans. Erythromycin may assist in eliminating the organism from nasal secretions.

12-Meningitis in the newborn baby:

True / False

May present with apnoeic attacks Correct Is always associated with a bulging anterior fontanelle Incorrect answer selected Is most commonly caused by Haemophilus influenzae Correct Is a risk factor for later conductive deafness Correct Has an above average incidence in babies with a meningomyelocoele Correct

Apnoeic episodes are a frequent presentation of sepsis in the newborn. The fontanelle may be normal in the initial stages. The commonest aetiology in newborn babies is group B streptococci, which may be acquired during or after delivery. The mortality is 5-15% in infants and of those who survive a proportion may be left with mental retardation, speech problems, visual impairment and neural deafness rather than conductive deafness. Meningomyelocoele is a risk factor for the introduction of meningeal infection .

http://en.wikipedia.org/wiki/Whooping_cough

http://news.bbc.co.uk/1/hi/health/medical_notes/98848.stm

13-Idiopathic thrombocytopenia:

True / False

Is caused by hypoplasia of megakaryocytes in the bone marrow Correct Results in a prolonged prothrombin time Correct Is self-limiting over a 3-6 month period in the majority of affected children Correct Requires splenectomy in less than 20% of affected children Incorrect answer selected Is an indication for corticosteroid therapy whenever the platelet count falls below 50 x 109/l Correct

The cause of idiopathic thrombocytopenia is not known but cases commonly follow a viral infection. Prothrombin time is normal, but platelet function tests are abnormal. The majority of cases will spontaneously remit. A minority of children go on to require splenectomy. When required it is usually delayed till the child is older than 5 years to minimise childhood infections. The absolute platelet count is not a criteria for immune suppression, but if the child develops bleeding complications steroids are usually started.

14-Characteristics of thalassaemia major include:

True / False

Polycythaemia Correct Low levels of foetal haemoglobin Correct Splenomegaly Correct High serum iron level Correct “Hair on end” appearance on x-ray of the skull Correct

Anaemia would result rather than polycythaemia. HbF and HbA2 levels will be high. Hepatosplenomegaly occurs secondary to chronic haemolysis and iron overload (multiple transfusions) occur leading to the risk of cirrhosis. Ineffective erythropoesis leads to excessive abnormal bone growth with skull and maxillary deformities.

More detail on Thalassaemia...

http://en.wikipedia.org/wiki/Thalassemia

http://www.emedicine.com/emerg/topic282.htm

15-An 11-year-old girl is brought into the accident and emergency department by the paramedics, along with three similarly aged friends who made the call to the emergency services. The girl’s friends have advised the paramedics that the girl had disclosed to them that she had taken 24 paracetamol tablets the previous night and had coughed up blood while they were playing at a local recreation ground.

You are in attendance to assess the child, who refuses to speak with you. She appeared alert and talkative with her friends. She says that she does not wish for her parents to be called, it was all a bad joke and she does not want any medical treatment.

What is the most appropriate action in this case?


Accept that this was a bad joke and discharge the child. Agree with the child not to contact the child’s parents but call social service to report this case. Assess whether the child is Gillick competent and discharge her only if she is Gillick competent. Speak with the child to assess her capacity and to advise her of the serious nature of a paracetamol overdose and the witnessed blood, and the importance of contacting her parents to attend. Correct Try to establish a full history from the child, and perform necessary investigations and treatment without the knowledge of the child’s parents.

It would be unusual for an 11-year-old child to be considered to be Gillick competent in such an situation, but even if the child were to be considered to be competent, her refusal of treatment could be lawfully overridden by authority given by a person with parental responsibility (such as a parent) – case Re W (a minor) [1992] 4 All ER 627.

It is important that a practitioner act in the best interests of their patients at all times and provide enough information to the patient, explaining the importance contacting her parents.

In circumstances where the child was incompetent it would be necessary to provide treatment in her best interests under the common law doctrine of necessity (This is consistent with the common law principles of the doctrine of necessity set out in the case of Re F [1990] 2 AC 1 and is consistent with ethical guidance published by the GMC in its booklet Seeking patients’ consent: the ethical considerations (November 1998) at paragraph 18:

“In an emergency, where consent cannot be obtained, you may provide medical treatment to anyone who needs it, provided the treatment is limited to what is immediately necessary to save life or avoid significant deterioration in the patient’s health…”). Alternatively, were the child to be competent and refusing treatment which was vital to protect life or serious harm, then urgent efforts should be made to obtain authority for treatment from someone with parental responsibility or occasionally through the courts; legal advice will almost certainly be required.

It may be considered appropriate to contact social services should there be a concern

that the child may be at risk of serious harm or abuse. It is important to review local child protection guidance and be familiar with the DOH guidance– What to do if you are concerned a child is being abused (2003)

Question supplied by the Medical Defence Union.

16-Theme:Respiratory paediatricsA AsthmaB Chronic lung disease of prematurityC Congenital lobar emphysemaD Cystic FibrosisE Drug misuseF Gastro-oesophageal refluxG HypogammaglobulinaemiaH Kartageners syndromeI Primary ciliary dyskinesiaJ Recurrent aspiration

Select the most likely diagnosis from the above list for the following cases:

A 6-year-old girl presents with productive cough, and is on the 3rd centile for height and 0.4% for weight.

Correct

A 7-year-old boy has had a runny nose from birth. He presents to the ENT clinic with recurrent sinusitis and cough. Chest X-ray is normal.

Correct

A 14-year-old boy who is a high performance athlete is on the 50th centile for height and 9th centile for weight. He has a nocturnal cough and dyspnoea during training.

Correct

Cough and exercise induced symptoms are a common combination in many respiratory disorders .A productive cough, especially associated with poor growth is highly suspicious of Cystic Fibrosis. A runny nose from birth is unusual and indicative of ciliary dysfunction, as are chronic otitis media and sinusitis. When associated with dextraposition, this is known as Kartageners syndrome. Diagnosis is by examination of nasal cilia .Asthma affects 10-15% of the paediatric population and presentation can occur at any age. Dry cough, wheeze and obvious trigger factors along with a history of atopy point towards the diagnosis. Competitive athletes need to inform governing bodies of all treatments for asthma.

17-Theme:GCS in childrenA 3B 4C 5D 6E 7F 8G 9H 10I 11J 12

What is the Glasgow coma Scale score for each of the following:

An 18 month old baby was involved in a car accident. He opens his eyes spontaneously, is persistently irritable, and flexes to pain.

Incorrect - The correct answer is 10

Eyes 4, verbal 3, motor 3

A 12-year-old boy fell off a scrambler bike. He opens his eyes to pain, speaks incomprehensibly, and withdraws to pain.

Correct


A 7-year-old girl falls off a swing. She is opens her eyes to voice, is confused, and localises pain.

Incorrect - The correct answer is 12


Glasgow Coma Scale (4-15 years)

EYE OPENING -spontaneously 4To verbal stimuli 3To pain 2No response 1

BEST MOTOR RESPONSE –Obeys verbal response 6Localises to pain 5Withdraws from pain 4Abnormal flexion (decorticate) 3Abnormal extension (decerebrate) 2No response 1

BEST VERBAL RESPONSE –Orientated and converses 5Disorientated and converses 4Inappropriate words 3Incomprehensible sounds 2No response 1

Childs Glasgow Coma Scale (<4yrs)

EYE OPENING –Spontaneous 4To verbal stimuli 3To pain 2No response 1

BEST MOTOR RESPONSE –Spontaneous/obeys verbal commands 6

Localises to pain or withdraws from touch5Withdraws from pain 4Abnormal flexion to pain 3Abnormal extension to pain 2No response 1

BEST VERBAL RESPONSE –Alert, babbles, coos as usual 5Less than usual words, spontaneous irritable cry 4Cries only to pain 3Moans to pain 2No response 1

The GCS is always the best possible response. The younger children are difficult, and the modified child’s score is used.

18-Theme:Language disorders.A Acquired hearing defectB AutismC Asperger syndromeD Congenital deafnessE Expressive language disorderF Landau-Kleffner syndromeG Pervasive developmental disorderH Selective mutismI Specific articulation disorder

For each scenario choose the most likely diagnosis:

A 2-year-old boy is referred with poor communication. He avoids eye contact and plays obsessively with a small range of toys. He does not speak.

Correct

The 2-year-old boy has autism, with defective language, poor reciprocal communication and obsessional behaviour.

A 5-year-old girl is referred with loss of speech. She previously spoke sentences, but now has difficulty both understanding and speaking.

Incorrect - The correct answer is Landau-Kleffner syndrome

The 5-year-old girl has lost speech. This may be associated with fits, and is characteristic of the Landau-Kleffner syndrome.

A 6-year-old girl refuses to speak in class. She converses normally at home.

Correct

This 6-year-old girl has context-dependent speech problems, so-called which is an anxiety disorder.

19-Concerning leukotrienes, which of the following statements is/are true/false?

True / False

Act on intracellular receptors Correct Are derived from palmitic acid Incorrect answer selected Cause bronchodilation Correct Generally increase vascular permeability Correct Leukotriene C4 and leukotriene D4 are important components with slow-reacting substance A Incorrect answer selected

Leukotrienes are produced, like prostaglandins, from arachidonic acid but via the lipo-oxygenase pathway, while prostaglandins are produced via the cyclo-oxygenase pathway.

Levels are increased in acute respiratory distress syndrome (ARDS).

Leukotriene E4 causes capillary leak in mevalonic aciduria. Leukotriene B4 activates polymorphonuclear lymphocytes, and C4 is responsible for allergic sensitisation.

D4 inhibitors are useful in asthma. C4, D4 and E4 levels are increased in asthma causing bronchus constriction and oedema.

Slow-reacting substance of anaphylaxis (SRSA) consists of leukotrienes C4, D4 and E4.

Further reading:Slow-reacting substance of anaphylaxis Ann R Coll Surg Engl. 1978 May; 60(3): 201-204.

Copyright © 2002 Dr Colin Melville

20-Spleen enlargement is invariably seen in:

True / False

Acute myeloid leukaemia Incorrect answer selected Idiopathic thrombocytopaenic purpura Correct Myeloproliferative disorders Incorrect answer selected Polycythaemia rubra vera Correct Subacute bacterial endocarditis Incorrect answer selected

A soft thin spleen may be palpable in 10% of neonates, 10% of normal children, and 5% of adolescents. In most individuals, the spleen must be 2-3 times its normal size before it is palpable.

Common causes of splenomegaly include:

Infection: o Bacterial: typhoid, endocarditis, septicaemia, abscess o Viral: EBV, CMV and others o Protozoal: malaria, toxoplasmosis o Haematological: haemolytic anaemia (congenital or acquired),

extramedullary haematopoiesis, thalassaemia, osteopetrosis, myelofibrosis.

Oncological: o Malignant: leukaemia, lymphoma, metastatic disease o Benign: haemangioma, hamartoma.

Infiltration/storage: o Lipidoses: Niemann-Pick, Gaucher o Mucopolysaccharidoses o Infiltration: histiocytosis.

Congestion: o Cirrhosis or hepatic fibrosis

http://my.onexamination.com/goto.aspx?url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2492068/

o Hepatic, portal or splenic vein obstruction o Congestive heart failure.

Cysts: o Congenital (true cysts) o acquired (pseudocysts).

Other: SLE, sarcoid, rheumatoid arthritis.

Although splenic enlargement is seen in all these conditions, this is not invariable. However, in polycythaemia rubra vera, the diagnostic criteria are increased total red blood cell volume, an arterial oxygen saturation of greater than or equal to 92%, and splenomegaly. The disorder in this condition is that erythroid precursors do not require erythropoietin to stimulate growth.

© 2002 Dr Colin Melville

21-Theme:ApnoeaA Blood culturesB CSF cultureC CT scan headD Drug screen on motherE EEGF Nasopharyngeal aspirate for virusesG Nasopharyngeal aspirate for pertussisH pH studiesI Urine metabolic screenFor each scenario choose the most specific diagnostic investigation:

A 5 day old girl presents to casualty following a brief apnoeic episode. On examination she is apyrexial, sweating, has a high-pitched cry and is difficult to settle.

Incorrect - The correct answer is Drug screen on mother

The 5day old presents with apnoea in the absence of fever or structural problem. The other symptoms point towards drug withdrawal, so a drug screen will be diagnostic.

A 4 month old girl presents with a 12 hour history of poor feeding and fever. She is rushed to hospital after she stops breathing. On examination she has an Oxygen saturation of 94%, and responds to pain. Serum glucose is 3.4 mmol/l.

Incorrect - The correct answer is Blood cultures

The 4 month old girl presents with a brief history of fever followed by apnoea. This suggests an infective cause. The decreased level of consciousness mean that LP should not be done yet. Blood cultures are likely to be the most helpful test.

A 5 month old boy has a 2 day history of coryza and poor feeding. On the day of admission he has a brief period of apnoea and is rushed to hospital. On examination he has 2 cm hepatomegaly and a blood glucose of 0.8 mmol/l.

Incorrect - The correct answer is Urine metabolic screen

The 5 month old presents with apnoea associated with mild infection and hypoglycaemia. This points to a metabolic cause, and a metabolic screen should be most helpful.

Apnoea can be caused by 3 main groups of conditions: infections, obstructions, or toxins/drugs.

22-Theme:Ear pain in children.A Acquired cholesteatomaB Acute mastoiditisC Acute otitis mediaD Dental problemsE Foreign bodyF FuruncleG Herpes zosterH Temporo-mandibular joint diseaseI TonsillitisFor each scenario choose the most likely diagnosis:

A 3-year-old boy presents with fever and ear pain. He was treated last week with antibiotics for acute otitis media, and initially improved. On examination he has swelling and tenderness behind the ear.

Correct

The 3-year-old child had symptoms of acute otitis media, and now has mastoid inflammation, mastoiditis.

A 14-year-old presents with a history of chronic ear pain and discharge. On examination he has a right perforated eardrum with whitish protrusion through it.

Incorrect - The correct answer is Acquired cholesteatoma

In the 14-year-old, there are chronic middle ear problems and a visible cholesteatoma.

A 6-year-old girl presents with left ear pain. On examination there are vesicles in the left ear canal and left facial weakness.

Correct

In the 6-year-old there are vesicles in the ear canal and facial weakness. This is likely to be Herpes zoster (Ramsay-Hunt syndrome).

Ear pain can be associated with abnormal external ear findings, abnormal middle ear findings or neither.

23-Theme:Bleeding/bruising.A Bernard-Soulier syndromeB Child abuseC Chronic liver diseaseD Disseminated intravascular coagulationE Ehlers Danlos syndromeF Glanzmann thrombastheniaG UraemiaH Vitamin K deficiencyI Von Willebrand diseaseFor each scenario choose the most likely diagnosis:

A 3 month old breast fed infant presents with fits and bruising. Haemoglobin 10.4 g/dl, White cell count 7.6x109/l, Platelets 298x109/l. Blood film Normal. Prothrombin

time ratio 2.3. Factor VIII and IX activity normal. Factors II, V, VII and X activity reduced.

Incorrect - The correct answer is Vitamin K deficiency

In 2 month old infant the low levels of II,V, VII, IX suggest vitamin K deficiency, with late-onset haemorrhagic disease most likely.

A 2-year-old girl presents with 12 hour history of lethargy, fever and bruising. Haemoglobin 11.2 g/dl, White cell count 4.5x109/l, Platelets 43x109/l, prothrombin time ratio 2.2, Activated partial thromboplastin time ratio 1.9. Thrombin Time 15s. Fibrinogen 0.9 g/l

Correct

In the 2-year-old girl the generalised abnormalities suggest DIC in relation to severe infection (eg meningococcal disease).

A 3-year-old boy presents with easy bruising and tissue-paper scars. Haemoglobin 12.1 g/dl White cell count 8.3x109/l, Platelets 275 x 109/L, with normal blood film. Bleeding time is elevated with normal von Willibrand factor activity.

Incorrect - The correct answer is Ehlers Danlos syndrome

In 3-year-old boy, the clinical appearance plust elevation of bleeding time with normal platelets and film suggest Ehlers Danlos. Without the tissue-paper scars Glanzmann would have to be considered.

In the assessment of bleeding or bruising an FBC, blood film, and clotting screen will make the diagnosis in most cases. Low platelets (thrombocytopaenia) are usually due to infection, ITP or leukaemia. Prolonged bleeding time may be due to von Willibrand's, platelet or connective tissue disorders. Raised APPT suggest specific clotting disorder, raised PT liver or vitamin K problems, and raised TT hepatic or renal disease. Generalised abnormalities are found in DIC and liver disease. If all tests are normal then Child abuse or Ehlers Danlos should be considered.

24-Theme:Renal masses.A Perinephric abscessB Polycystic kidney diseaseC Posterior urethral valvesD Prune-belly syndromeE Renal cystic dysplasiaF Renal vein thrombosisG Simple renal cystH UreterocoeleI Wilms tumourFor each of the following case scenarios select the most likely diagnosis from the list above:

On first day check a day old boy is noted to have mass centrally below the umbilicus which is dull to percussion.

Correct

In the neonate, a palpable bladder should prompt an urgent scan to exclude posterior urethral valves.

A 9 month old girl presents with a high fever. A tender mass is palpable in the right flank. Urine dipstix is positive for blood and leukocytes.

Correct

In the 9 month old girl the symptoms and signs suggest an upper pole renal infection, with perinephric abscess being most likely.

A 3 month old boy presents with abdominal mass in the left flank. Urine dipstix is positive for blood.

Correct

In the 3 month old boy, haematuria and renal mass without fever suggest Wilms' tumour. This may be associated with aniridia and hemihypertrophy.

25-In a 12-year-old adolescent girl with acute diabetic ketoacidosis:

True / False

Boluses of sodium bicarbonate are frequently necessary to correct metabolic acidosis Correct Nasogastric tube insertion should be considered Incorrect answer selected A bolus of 4 units/kg of human actrapid should be given IV Correct The estimated fluid deficit should be replaced over 24 hours Correct Hypokalaemia frequently occurs in the acute phase Incorrect answer selected

The management priorities in diabetic ketoacidosis are fluids, insulin, potassium, acidosis, identification and treatment of any underlying cause.

Fluids: if there is shock, then boluses of 20 ml/kg of normal saline should be given, followed by correction of the deficit over 48 hours. Over rapid correction may result in cerebral oedema. Monitoring should involve Glasgow Coma Scale, fluid inputs and outputs, regular checks for electrolytes, creatinine acid base status, and CVP or urinary catheterisation if required. A nasogastric tube is passed if there is vomiting or depressed consciousness to reduce the gastric dilatation.

Insulin: no bolus is now normally given, and the insulin infusion is usually started at 0.05-0.1 unit/kg/hour. The blood glucose should be monitored regularly, with an aim to reduce the blood glucose by 2 mmol/hour. More rapid reduction risks cerebral oedema.

Potassium: the initial potassium is usually high, but potassium will drop following treatment with insulin, as potassium and glucose enter cells together. Potassium replacement is therefore instituted as soon as urine pH is passed.

Acidosis: a degree of acidosis is inevitable, but bicarbonate should be avoided unless the pH is less than 7 or is not improving. The acidosis will self-correct with fluid and insulin therapy.

Other: infection may need antibiotics.


26-Burkitt's lymphoma:

True / False

Always presents with a mass in the jaw Correct

Is highly responsive to chemotherapy Correct

Is one of the commonest solid tumours of childhood in equatorial

Africa Correct

Occurs in endemic and non-endemic forms Correct

Usually presents with stage III or IV disease Correct

The Epstein-Barr virus is implicated in

Burkitt's lymphoma

lymphoepithelioma

Hodgkin disease.

90-95% of Burkitt's lymphoma in Africa are EBV related, versus only 20-30% in the USA.

Chronic stimulation of B lymphocytes by EBV can promote chromosomal translocations that contribute to malignant transformation. Chronic malarial infection appears to increase the risk of Burkitt's lymphoma by decreasing immune surveillance of genetically altered cells.

Burkitt's lymphoma is a small, non-cleaved cell (SNCC) type of non-Hodgkin's lymphoma (NHL). These are B cell tumours that express cell surface immunoglobulin and contain one of three characteristic chromosomal translocations: t(8;14), t(2;8), or t(8;22). Each involves the c-Myc oncogene and an immunoglobulin gene.

The endemic (African) Burkitt's lymphoma is often found in the jaw, and is the most common childhood cancer in equatorial East Africa and New Guinea. The mean age of onset is five years.

Only 20% of non-endemic (sporadic) Burkitt's lymphoma cases contains EBV genomes. Prognosis is good, as for most forms of non-Hodgkin's lymphoma (NHL).


Tag Question

27-The following features are recognised in profound hypothermia:

True / False

Hypertension Correct

Haemoglobinuria Correct

Beneficial effect of Prednisolone Correct

J wave on ECG Correct

Ventricular arrhythmia Correct

Hypothermia occurs particularly in winter sports injury, equipment failure, or exhaustion decrease the degree of exercise. Immersion and wet wind chill rapidly produce hypothermia, defined as a rectal temperature less than 35°C, when the body can no longer sustain normal temperature through shivering. Clinical features include extreme lethargy, fatigue, incoordination, apathy, which are followed by mental confusion, clumsiness, irritability, hallucinations, and finally bradycardia. Ventricular arrhythmia can be a problem because of vibration during transfers. J waves may be seen on the ECG. Haemoglobinuria may occur secondary to D.I.C.

Warming plus monitoring of blood pressure, fluid pH and oxygen status are necessary.


28-Regarding congenital hypothyroidism:

True / False

It is much less common than phenylketonuria. Correct

It is due to dyshormogenesis in about 10% of cases. Incorrect answer

selected

Hypothyroidism due to TSH deficiency is very rare. Correct

Despite early treatment, a few patients have severe learning

difficulties. Correct

Neonatal screening detects >95% of cases. Correct

The neonatal screening programme for congenital hypothyroidism is a major recent triumph for Paediatrics, with very few cases missed. It is one of the most common of the metabolic defects, detection is virtually complete, and the long term outcome should be an IQ within the normal range. Causes of congenital hypothyroidism include:

Athyrosis.

Maldescent.

Dyshormonogensis (10%).

Iodine deficiency (commonest worldwide, but rare in the UK because of iodination of salt).

TSH deficiency (usually associated with panhypopituitarism, very rare).Copyright © 2002 Dr Colin Melville

29-The following are features of congenital adrenal hypoplasia:

True / False

Low renin levels Correct

Exaggerated diurnal cortisol responses Correct

Association with Duchenne Muscular Dystrophy Incorrect answer

selected

Association with vitiligo Incorrect answer selected

Presentation with diarrhoea, vomiting and dehydration Correct

Hypoadrenalism usually presents in the neonatal period with increasing pigmentation, salt loss and cryptorchidism in males. The disorder is due to a mutation in the DAX 1 gene, a new nuclear hormone receptor family, located on Xp21.3-p21.2. Hypogonadotrophic hypogonadism (HHG) is caused by the same gene mutation, and the cryptorchidism is explained by this. The disorder is located very close to the Duchenne muscular dystrophy gene and the glycerol kinase gene, so these are associations.


Tag Question

30-The following are true of angiotensin II:

True / FalseIt is 50 time more potent than angiotensin I as a renal arterial

vasodilator. Correct

It inhibits aldosterone secretion. Correct

It reduces afterload in cardiac failure. Correct

Angiotensin I is converted to angiotensin II in the kidney. Correct

Secretion is increased by hypokalaemia. Correct

Renin is produced by the juxtaglomerular apparatus of the kidney, and converts and alpha-2 globulin to angiotensin I. In the lung angiotensin I is activated to angiotensin II, which is 50 times more potent as a presser agent than noradrenaline. Angiotensin II acts directly on the adrenal cortex to stimulate secretion of aldosterone, a potent mineralocorticoid produced in the zona glomerulosa. Renin secretion is increased by sodium deprivation, and aldosterone acts at the distal tubule to control sodium reabsorption (and indirectly, water reabsorption).


31-A 7-year-old child presents with a four month history of daily headache. This is frontal and is worsened by coughing, sneezing and bending. He has a persistent purulent nasal discharge.

Full term normal delivery with no neonatal complications. Immunisations up to date. There is no FH/SH of note. On examination he has a temperature of 37.3°C, respiratory rate 15/min, pulse 90/min. Blood pressure is 80/60 mmHg.

He has a blocked nose with purulent discharge, and is tender over both maxillae. Ear and throat examination are normal.



Cerebral abscess

Chronic sinusitis Correct

Cluster headache

Migraine

Tension headache The history of persistent chronic headache with persistent nasal discharge and maxillary tenderness suggest sinusitis affecting frontal and maxillary sinuses. This can be confirmed by CT scan. It is usually treated by antibiotics and decongestants but surgical drainage may be required

32-The platelet count is typically reduced in:

True / False

Acute leukaemia Correct

Kawasaki disease Correct

Disseminated intravascular coagulation Correct

Henoch-Schönlein purpura Correct

Vitamin K deficiency Correct

The platelet count will be reduced in acute leukaemia due to bone marrow infiltration by blasts.

In Kawasaki disease there will be thrombocytosis.

Disseminated intravascular coagulation is characterised by low platelets and abnormal clotting profile.

In Henoch-Schönlein purpura there will be normal clotting profile and platelet count.

Vitamin K deficiency causes prolonged prothrombin time (PT) and activated partial thromboplastin time (APTT), but not thrombocytopaenia.

The Prothrombin time is a measure derived from the Prothrombin Ratio (PR) and the International Normalised Ratio (INR). This is used in checking if the dose of warfarin is correct. The prothrombin time measures the extrinsic pathway of coagulation; this includes measuring Clotting Factors 2, 5, 7, 10 and Fibrinogen. The normal value is 12-15 seconds (depending on your control).

The Activated Partial Thromboplastin time measures the intrinsic pathway of clotting. It measures the clotting factors - 8, 9, 11, 12, and is used in Von Willebrand's disease. It is used to check if the dose of heparin is correct. The normal value is 25-39 seconds (depending on your control).

33-A female infant is born at 28+4/40 gestation weighing 1.01kg. She requires 13day ventilation, 2day CPAP and then continued nasal prong Oxygen at 0.02 l/min. At 8 weeks of age she still has this Oxygen requirement. Capillary gases show pH 7.38, pCO2 9.5 kPa, base excess of +8 mmol/l. She is discharged home on Oxygen at 0.02 l/min.

4 weeks after discharge she presents to the Paediatric ward with respiratory distress. Her O2 saturation is 85% in nasal prong Oxygen of 0.02 l/min. She has respiratory rate of 60/min, marked recession and heart rate of 165/min. There is 2 cm hepatomegaly.



Bronchiolitis This is the correct answer

Bronchopulmonary dysplasia Incorrect answer selected

Cardiac failure

Pneumonia

Wilson-Mikitty syndrome She fulfils the definition of chronic lung disease, which is O2 dependence at 36/40 corrected age. This condition is caused by a combination of O2 therapy, barotrauma (from ventilation) and fluid overload. The result is ongoing lung scarring, decreased lung compliance and gas exchange. There is respiratory acidosis (CO2 retention) with compensatory metabolic alkalosis. These infants have diminished lung reserve and are very vulnerable to respiratory infections, with a greatly increased risk of PICU. Her second presentation is likely to represent an acute infection, most probably bronchiolitis.

34-A 4-year-old boy presents with dizziness and vomiting. He has been unwell over the past few days with fever, right ear pain and discharge. Full term normal delivery with no neonatal complications. Immunisations up to date. There is no FH/SH of note.

On examination he has a fever of 38.6°C, RR 20/min and HR 100/min. Chest is clear. He has a purulent discharge from his right ear. He has nystagmus, and walks with a broad-based gait.



Brain tumour

Infections Correct

Ingestions The history suggests an acute otitis media with developing ataxia and vomiting. This suggests extension of the infection into the vestibular apparatus. From there it can extend into the brain. IV antibiotics and surgical drainage may be required.

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35-1, 25, dihydroxycholecalciferol:

True / FalseCauses suppression of parathyroid hormone (PTH) by direct action on the parathyroid gland. Incorrect answer selected

Causes suppression of parathyroid hormone indirectly by increasing serum calcium. Correct

Acts on cell surface receptors. Correct

Is biologically more active than 25, dihydroxycholecalciferol. Correct

Leads to increased osteoblast activity. Incorrect answer selected 7, dihydrocholesterol is converted by sunlight into cholecalciferol (Vitamin D3). In the liver this is 25, hydroxylated, and in the kidney this is 1, 25 hydroxylated. Vitamin D is not abundant in natural foods except for fish liver oils, fatty fish and egg yolk. Ergocalciferol (Vitamin D2) is used to fortify margarine. 1, 25 dihydroxycholecalciferol is the most active form of the vitamin. It is produced following parathormone secretion in response to a low plasma calcium. The effect of 1, 25 (OH2D3) is to induce synthesis of a calcium binding protein (calbindin-D) in the intestinal mucosa with the resultant absorption of calcium. It also promotes bone dissolution and mineralisation. Thyroid hormones also mobilises calcium by directly enhancing bone resorption, an effect that requires 1, 25 dihydroxy Vitamin D. The effects of PTH on bone and kidney are mediated through binding to specific receptors on the membranes of target cells and through activation of the transduction pathway involving a G protein coupled with an adenylate cyclase system. The active form of vitamin D binds to intracellular receptors that then function as transcription factors to modulate gene expression.


36A mother is concerned because her one-year-old girl was in contact with measles 2 days previously. Which one of the following statements is correct?


Nothing can be done to affect the course of the disease.

Gamma globulin can be administered up to 14 days after exposure. Incorrect answer selected

If she were to become infected then complications would be extremely rare.

Immunisation with the live attenuated virus will give long immunity if given immediately This is the correct answer

She should anticipate the rash occurring in the next 5 days. If the person is within 72 hours of exposure measles vaccine is the treatment of choice as it will confer life long immunity. In persons in whom the vaccine is contraindicated immunoglobulin can be given. Immunoglobulin is effective within 6 days of exposure. One third of those afflicted with measles can expect a complication. These include pneumonia, otitis media, diarrhoea. A serious complication is the development of sub-acute sclerosing, pan-encephalitis. The initial symptoms of measles are coryzal symptoms. Several days after the appearance of coryzal symptoms a rash appears.

37-A 10-month-old Afro-Caribbean boy presents with painful swelling of the hands and feet. This has worsened over the past six hours, making him distressed and weepy. Full term normal delivery born in Africa, no neonatal problems, moved to the UK age 4 months. Immunisations up to date. Mother is known to have sickle trait.

On examination he is apyrexial, with respiratory rate of 30/min and pulse of 105/min. There is symmetrical swelling of the hands and feet, which are painful to touch. His spleen is 3 cm below the costal margin.



Haemophilia A

Kawasaki disease

Reactive arthritis

Septic arthritis

Sickle cell crisis Correct The history of symmetrical painful swelling of hands and feet in an Afro-Caribbean child suggests sickle cell dactylitis ('Hand and foot syndrome'), which is often the first manifestation of sickle cell disease. It is due to ischaemic necrosis of the long bones as the bone marrow expands.

Haemoglobin electrophoresis confirms the diagnosis. Oxygen and analgesia should be given.

38-Theme:Stridor

A Acute Laryngitis

B Angioneurotic Oedema

C Bacterial Tracheitis

D Croup

E Diptheria

F Epiglottitis

G Foreign Body Inhalation

HRetropharyngeal Abscess

I Smoke Inhalation

J Vocal Cord PalsyFor each patient described below choose the single most likely option from the list above.

A 9 month old boy is referred to ENT as he has been noted to have stridor. He sometimes chokes when drinking or feeding. His OFC has risen from the 50% at birth to the 98% and he has short limbs.

Correct

A 4-year-old girl attends a birthday party and develops a choking episode. She recovers, but over the next 2 days becomes more unwell with a fever and a stridor. She is found to have marked intercostal recession.

Correct

A 3-year-old boy is brought to casualty with a sudden onset of stridor. Over the past 3 days he has been unwell with coryzal symptoms and ear temperature of 38.2C.

Correct

The 9 month old child has achondroplasia, which is often associated with hydrocephalus (Arnold-Chiari malformation). This in turn can be associated with vocal cord palsy.

Croup may be caused by different viruses although most commonly as a result of infection with Parainfluenza. Typically it occurs in young children a few days after a coryzal illness with symptoms of a barking cough, a low grade fever and an inspiratory stridor. Symptoms are worse at night.

The 4-year-old girl developing fever and stridor following a birthday party is a classical history for inhalation of a foreign body e.g. a peanut. This results in deterioration over 24-36 hours and widespread respiratory signs.

39-Theme:Seizures

A Absence seizures.

B Atonic seizures.

C Benign partial seizures with centrotemporal spikes.

D Complex partial seizures.

E Febrile seizures.

F Generalised tonic clonic seizures.

G Infantile spasms.

H Myoclonic seizures.

I Simple partial seizures

J Status epilepticus.For each of the cases below choose the single most likely option from the above list.

A 13-year-old girl complains of feeling unwell. She describes a strange taste in her mouth and suddenly becomes totally unresponsive.

Correct

A 9-year-old girl is seen to develop twitching over the left side of her face and neck. This lasts for approximately a minute and throughout the episode is fully conscious.

Correct

A 9-year-old boy presents with episodes of facial twitching. His EEG shows a characteristic pattern in the temporal region.

Correct

The 9-year-old boy has benign partial epilepsy of childhood (BPEC). It is a common type of partial seizure with an excellent prognosis. Age of onset is at 9-10 years with remission by 15 years. Seizures are partial with facial twitching and drooling. EEG pattern is characteristic with centro-temporal spikes with normal background activity. The 9-year-old girl has simple partial seizures (SPS) as she remains conscious throught the episode, whilst the 14-year-old girl has complex partial seizures. In complex partial seizures (CPS) an aura is often experienced and accompanied by automatisms. They may evolve into generalized tonic clonic seizures. In CPS there is a history of impaired consciousness, unlike SPS where consciousness is maintained. In SPS asynchronous movements are seen in the face, neck and extremities. An aura may be present but automatisms do not tend to exist.

Tag QuestionRemove Tag

40-Intussusception in children:

True / False

Is more common between the age of 3 months to 3 years Correct

Bile stained vomiting is an early feature Correct

Bleeding per rectum is the most common presenting symptom Incorrect answer selected

Is usually caused by a polyp or pedunculated tumour Incorrect answer selected

Hydrostatic reduction plays an important role in the management Correct Intussusception is more common in the 3 month - 3 year age group. It occurs more commonly in males. Clinical features include sudden paroxysmal colicky abdominal pain and vomiting. The vomiting is not bile stained. Bleeding per rectum occasionally is seen but it is not common and is a late sign. Intussusception tends to occur in regions in which a peristaltic segment abuts on to an aperistaltic segment. These include enlarged Peyers patches, Meckels diverticula, tumours and haematomas complicating Henoch Schonlein purpura. In most cases the cause is not identifiable

41-The following diagnoses can be reliably made on antenatal ultrasound performed before 20 weeks:

True / False

Spina bifida occulta Incorrect answer selected

Gastroschisis Correct

Ventricular septal defect Incorrect answer selected

Gestational age Correct

Down syndrome Incorrect answer selected Gestational age can be reliably estimated if performed before 20 weeks, but after this the margin for error increases. Multiple pregnancies can be identified.

Up to 70% of major structural abnormalities can be identified, and more detailed scans and specialist centres arranged.

Fetal growth can now be reliable measured from serial abdominal circumference, bi-parietal diameter and femur length.

Oligohydraminos and polyhydraminos can also be diagnosed.

Although specialist centres can reliably diagnose major cardiac malformations, VSD can be very difficult to detect.

Nuchal fold thickness is being investigated as a possible means of making a diagnosis of Down syndrome, and results are promising. However, the results are not currently reliable enough for routine screening.


42-Which of the following is true regarding sensory neural hearing loss?

True / FalseThe incidence is increased in babies admitted to neonatal intensive care units compared with the normal population Correct

The risk is increased in children who have had congenital rubella Correct

Approximately 1 per 1000 children will have greater than 40 db hearing loss Correct

The risk is increased in Noonan's syndrome Incorrect answer selected

The risk is increased in Down syndrome Incorrect answer selected Sensory neural hearing loss is caused by lesions in the cochlea or the auditory nerve or central connections. It may be unilateral or bilateral. Language acquisition and secondary educational difficulties follow, with social isolation, and an increased risk of mental health problems. The approximate incidence is 1 per 1000 children. Risk factors include:

NICU admission: low birth weight, less than 32 weeks' gestation, prolonged ventilation,

prolonged jaundice, ototoxic drugs, hypoxic ischaemic encephalopathy, neonatal

meningitis.

Congenital infection (rubella, CMV).

Dysmorphic syndromes (affecting head and neck).

Family history of a close relative needing a hearing aid below the age of 5 years.

Infections: acute bacterial or TB meningitis, mumps (latter usually unilateral).

If all risk factors are considered, only around 50% of cases could be identified by testing between 5 and 10% of all babies.

Conductive hearing loss is related to middle ear pathology. This is commoner in Down syndrome, cleft palate, Turner's syndrome, and facial malformation syndromes. Down's children also have an increased risk of sensorineural hearing loss.

In Noonan's syndrome the incidence of progressive high-frequency sensorineural hearing loss may be as high as 50%.

© 2002 Dr Colin Melville

43-Causes of lung cysts on chest x ray include which of the following?

True / False

Aspergillus infection Correct

Congenital lobar emphysema Correct

McLeod's syndrome Correct

Staphylococcal pneumonia Correct

Tuberculosis Incorrect answer selected Causes of lung cysts can be divided into:

Congenital:

o lobar emphysema

o cystic adenomatoid malformation

o bronchogenic cyst

o sequestration (intralobar, extralobar).

Acquired:

o bronchiectasis

o associations with any long-standing infection such as cystic fibrosis, post-measles, post-

pertussis, TB.

Staphylococcal and Klebsiella pneumonia are characteristically associated with cavitation.

Aspergillus usually, is happy to colonise cavities that are already present. Invasive pulmonary aspergillosis may cause nodular infiltrates on chest x ray.


44-The following are characteristic presentations of cystic fibrosis:

True / FalseA 26 week gestation infant with x ray appearance of meconium ileus Correct

A 3-week-old term female with prolonged unconjugated jaundice Incorrect answer selected

An 8-month-old girl admitted the second time with lower respiratory tract changes on chest x ray Correct

A 9-month-old Indian girl with failure to thrive Correct

A 4-year-old boy with mouth breathing due to nasal polyps Correct Typical clinical features at presentation are GI or respiratory.

Respiratory

Recurrent chest infections

Bronchiectasis

Pneumothorax

Sinusitis or nasal polyps

Haemoptysis

Aspergillosis.

Gastrointestinal

Meconium ileus (neonate)

Steatorrhoea

Malabsorption

Failure to thrive or growth failure

Rectal prolapse

Prolonged conjugated jaundice.


45-A black West African girl aged 15 years is complaining of severe pain in both legs. The haemoglobin is 8 g/dl.

If which of the following is true, is sickle cell anaemia unlikely to be the cause of her symptoms?

True / False

A Menarche occured at 12 years Correct

B She has haematuria Correct

C She is jaundiced Correct

D The urinary osmolality is 800 mosm/kg (specific gravity approximately 1022) Correct

E x Ray of the spine shows osteoporosis Correct A Growth and development are usually impaired.

B and C These are expected in sickle cell anaemia.

D Almost all are unable to produce concentrated urine.

E x Ray usually shows fish mouth vertebrae (infarcts).

Tag QuestionRemove Tag

46-Do the following suggest pulmonary rather than aortic valve stenosis?

True / False

A deep S wave in V2 Correct

An ejection click best heard in the second and third left intercostal spaces Correct

An ejection murmur radiating to the neck Correct

An upright T wave in V1 Incorrect answer selected

Prominence in the left upper mediastinum on chest x ray Correct An ejection murmur radiating to the back (along the direction of the pulmonary arteries) is found in pulmonary stenosis. The ejection click is found at the apex in aortic stenosis.

A deep S wave in V2 and large R wave in V6 suggests left ventricular hypertrophy (LVH), while an upright T wave in V1 suggests right ventricular hypertrophy (RVH).

A prominent left upper mediastinum on chest x ray may be caused by post-stenotic aortic dilatation in aortic stenosis.

47-The following features suggest tetralogy of Fallot rather than transposition of the great arteries:

True / False

Absence of clinical cyanosis Correct

Absence of S wave in V1 Incorrect answer selected

Increased pulmonary vascular markings on chest x ray Correct

Presentation at 2 months of age with a murmur Correct

Single second heart sound Correct On chest x ray, a 'boot-shaped' heart with pulmonary artery bay and uptilted apex suggests Fallot's, while an 'egg on side' appearance and pulmonary plethora suggests TGA.

In Fallot's lung blood flow is decreased, and the absence of an S wave in V1 suggests right ventricular hypertrophy.

The second heart sound is single in both conditions.

Transposition usually presents in the first few days of life, and the patient is invariably clinically cyanosed.

Copyright 2002 Colin Melville

48-Which of the following are true regarding a diagnosis of intussusception?

True / False

An abdominal mass Incorrect answer selected

An empty rectum on examination Incorrect answer selected

Fever Correct

Fluid levels on an erect abdominal x ray Correct

Increased bowel sounds Correct

An infant presenting with sudden onset of colicky abdominal pain needs to be assessed for intussusception.

The baby may have a palpable abdominal lump in the right upper quadrant.

There is not usually a fever.

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Abdominal x ray may show signs of small bowel obstruction.

The diagnosis is confirmed by ultrasonography, or with a barium enema which may reveal the claw sign at the point of the bowel invagination.

Hydrostatic enema is the mainstay of non-operative treatment, whereby reduction can be achieved.

49-Which one of the following statements is true concerning airway obstruction in the newborn?


Epiglottis is a likely diagnosis

In Pierre Robin syndrome the airway can be improved by a nasopharyngeal tube Correct

Stridor is less severe in the supine position

The commonest cause is subglottic stenosis

Vascular ring can be diagnosed by lateral x ray of the neck The most common cause of neonatal stridor is laryngomalacia, characterised by low pitched inspiratory stridor. Other causes include

unilateral vocal cord paralysis

subglottic stenosis.

In Pierre Robin syndrome there is

micrognathia

cleft palate

glossoptosis, where the tongue falls backwards and downwards.

Glossoptosis can be managed with a nasopharyngeal airway.

Epiglottitis is extremely uncommon now following the introduction of Haemophilus influenzae vaccine.

Stridor is more severe in the supine position and after agitation/crying.

Vascular ring is not diagnosed by x ray, but MRI can

50-Pneumocystis pneumoniae

True / False

Is an obligate intracellular organism Incorrect answer selected

May be usefully diagnosed by serology Correct

Can cause disease outside the respiratory system Correct

Can have normal chest x ray with generalised wheeze Incorrect answer selected

Is adequately treated with erythromycin alone Correct Pneumocystis is an obligate extracellular parasite with attributes of both fungi and protozoa. Most humans are seropositive by 4 years of age, though the transmission mode is unknown.

Forty percent of children with HIV and 10% with leukaemia get Pneumocystis carinii pneumonia (PCP) if no prophylaxis is given.

Infection is almost always of the lungs, and rarely outside. The onset is subtle, at a peak incidence of 3-6 months of age in HIV infected individuals, with a raised respiratory rate but no fever. There is a gradual increase in respiratory distress and cyanosis with few clinical signs.

Chest x ray usually reveals marked changes of alveolitis, on some occasions looking like 'post-neonatal hyaline membrane disease'.

Normal chest x ray is reported in 0-39% of cases in adults.

Diagnosis is most effectively by bronchi-alveolar lavage or brushings are even better by lung biopsy.

Treatment is high dose septrin and high dose steroids.


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