Pyrexia And Rash

Question 1

What is Henoch-Schnonlein purpurin (HSP)?

Answer 1

• Henoch-Schönlein purpura (HSP) is an IgA-mediated, autoimmune hypersensitivity vasculitis of childhood.
• The main clinical features are skin purpura, arthritis, abdominal pain, gastrointestinal bleeding, and nephritis. The aetiology remains unknown.

Question 2

What is the epidemiology of HSP?

Answer 2

• HSP is a rare condition but is the most common form of systemic vasculitis in children; 90% of cases occur in childhood under the age of 10 years. The peak prevalence is in children aged 4-6 years.
• It is rare in infants and young children. It occasionally occurs in adolescents and adults, and tends to be more severe and more likely to cause long-term renal problems when it occurs in adults.

Question 3

What is the aetiology of HSP?

Answer 3

• The condition tends to be seasonal and there is often a history of recent infection.

Infections preceding HSP include those involving group A streptococci, mycoplasma, Epstein-Barr virus, Coxsackievirus, hepatitis A and B, parvovirus B19, campylobacter, varicella and adenoviruses. Vaccination has also been described as a trigger.

• There is also an association with malignancy; usually solid tumours rather than haematological malignancies, and more common in adult males.
• IgA immune complexes are involved in the pathophysiology of HSP, depositing in the small blood vessels of the skin, joints, kidneys and gastrointestinal tract, causing an inflammatory reaction.

Question 4

What is the presentation of HSP?

Answer 4

• The disease occurs mostly in the autumn or winter months. There may be a history of a preceding upper respiratory tract infection (URTI) or less commonly a gastrointestinal infection.
• Generally, patients appear to be mildly ill, with low-grade fever.
• There is a symmetrical, erythematous macular rash, especially on the back of the legs, buttocks and ulnar side of the arms.
• Within 24 hours, the macules evolve into purpuric lesions, which may coalesce and resemble bruises.
• Typically the purpura are slightly raised and palpable.
• Abdominal pain and bloody diarrhoea may precede the typical purpuric rash. HSP may also cause nausea and vomiting.
• Renal involvement
• Scrotal involvement may mimic testicular torsion.
• Headaches may occur, and occasionally seizures and other nonspecific neurological symptoms.

Question 5

What is the renal involvement in HSP?

Answer 5

o Usually occurs within three months of disease onset.
o There is usually no relationship between the severity of nephritis and the extent of the other manifestations of HSP.
o Microscopic haematuria with mild-to-moderate proteinuria may occur.
o Nephrotic syndrome may also occur.
o Oliguria and hypertension are uncommon.

Question 6

Which signs and symptoms are required before the diagnosis of HSP?

Answer 6

For a diagnosis of HSP there must be palpable purpura, which is not thrombocytopenic/petechiae, and one or more of the following:
o Diffuse abdominal pain.
o Typical histopathology (leukocytoclastic vasculitis or proliferative glomerulonephritis with predominant IgA deposits).
o Arthritis or arthralgia.
o Renal involvement (demonstrated by quantified proteinuria or haematuria).

Question 7

What is the differential diagnosis of HSP?

Answer 7

• Intussusception
• SLE
• Thrombocytopenia
• Meningococcal meningitis
• Glomerulonephritis
• G.I symptoms such as IBD

Question 8

What are the investigations for HSP?

Answer 8

• Diagnosis of HSP is clinical and not based on laboratory investigations, and there is no definitive test. The following tests may be relevant:
• Urinalysis (should always be performed): haematuria and/or proteinuria are present in 20-40% of patients.
• FBC: there may be raised white cell count with eosinophilia; normal or increased platelets. Helps in excluding other diagnoses such as thrombocytopenia.
• Raised ESR.
• Serum creatinine may be elevated in renal involvement.
• Serum IgA levels are often increased. This is not diagnostic.
• Autoantibody screen: connective tissue diseases.
• Abdominal ultrasound: if there are gastrointestinal symptoms - for diagnosis of intestinal obstruction.
• Barium enema: may be used to confirm and treat intussusception.
• Testicular ultrasound: assessment of possible torsion.
• Renal biopsy: if there is persistent nephrotic syndrome.
• Screening for cancer should be considered for older adults who develop HSP with no preceding infection

Question 9

What is the management of HSP?

Answer 9

HSP is usually self-limiting and no form of therapy has been shown appreciably to shorten the duration of disease or prevent complications. Therefore, treatment for most patients remains primarily supportive and is entirely symptomatic unless there is renal involvement. This includes rehydration, pain relief, wound care for ulcerative lesions and treatment for intussusception where present.

Non-steroidal anti-inflammatory drugs (NSAIDs) may help joint pain but should be used with caution in patients with renal insufficiency or gastrointestinal symptoms.

Admission to hospital may be required in some cases for monitoring of abdominal and renal complications.

Corticosteroids can ameliorate associated arthralgia and the symptoms associated with gastrointestinal dysfunction, but it seems there is no place for routine use and no evidence of benefit of prednisone in preventing serious long-term kidney disease in HSP.

Plasma exchange is used in the management of some adults with vasculitis and idiopathic rapidly progressive nephritis but further trials are needed.

Question 10

When is monitoring required in HSP?

Answer 10

GPs are likely to be involved in monitoring for renal involvement, usually under written guidance from the secondary care specialist.

Typical monitoring advice would be:
o For those with no proteinuria, blood pressure checking and urinalysis at days 7 and 14 and at 1, 3, 6 and 12 months.
o For those with proteinuria, follow-up at days 7 and 14, monthly from 1-6 months and then at 12 months.

Question 11

What are the complications of HSP?

Answer 11

• Renal impairment
• MI
• Pulmonary haemorrhage
• GI bleeding
• Intussusception
• Bowel infarction
• Testicular torsion
• Seizures

Question 12

What is immune thrombocytopenic purpura?

Answer 12

Immune thrombocytopenia (ITP) describes an autoimmune disorder in which the number of circulating platelets is reduced. This is due to their increased destruction, and sometimes also due to reduced production.

Question 13

What is the aetiology of ITP?

Answer 13

In ITP, otherwise normal platelets are destroyed, most often in response to an unknown stimulus.

This may occur in isolation (primary ITP) or in association with other disorders (secondary).

Question 14

What are the causes of secondary ITP?

Answer 14

o Other autoimmune disorders (including antiphospholipid antibody syndrome and systemic lupus erythematosus).
o Viral infections (including cytomegalovirus, varicella-zoster, hepatitis C and HIV).
o Infection with Helicobacter pylori.
o Medication.
o Lymphoproliferative disorders.

Question 15

How does ITP present in children?

Answer 15

• ITP in children most commonly occurs following a viral infection, or occasionally following immunisation.
• It is usually a self-limiting disorder which follows a benign course and recovers spontaneously after 6-8 weeks.
• The clinical picture ranges from no symptoms whatsoever to life-threatening intracranial haemorrhage. Some children with ITP will have no symptoms at all.
• The most common presentation is petechiae or bruising. Up to a quarter present with nosebleeds. Haematuria and gastrointestinal bleeds are less common. Older girls may have menorrhagia. Intracranial bleeds occur very rarely.

Question 16

What are the differentials of ITP?

Answer 16

o Aplastic anaemia 
o Leukaemia 
o Fanconi’s anaemia 
o VWD
o NAI 
o TTP
o DIC

Question 17

What are the investigations for ITP in children?

Answer 17

o FBC
o Peripheral blood smear
o Bone marrow examination- only required if atypical features or diagnosis are in doubt

Question 18

What is the management of ITP in children?

Answer 18

o Treatment is considered on the basis of clinical symptoms and not on the basis of platelet count alone, as children with severe thrombocytopenia are often asymptomatic and do not have a serious risk of a bleed.

o The majority of children with ITP do not need therapy and have a spontaneous resolution of the disease

o Any of the following may be used in a specialist setting if it is thought that the clinical condition requires that the platelet count should be raised:
o First-line treatment
Prednisolone.
Intravenous immunoglobulin.
Intravenous anti-D immunoglobulin (in rhesus-positive children).
o Second-line treatment for those resistant to first-line treatment and with significant bleeding
Rituximab.
High-dose dexamethasone.
o Other treatments
Tranexamic acid is used for menorrhagia, but is contra-indicated if there is haematuria.
Emergency platelet transfusions are sometimes used.
o Surgical
Splenectomy is rarely indicated in childhood ITP and is only used in the event of life-threatening bleeding, or in children with severe, chronic, unremitting ITP present for 12-24 months with severe symptoms.

Question 19

What is the presentation of ITP in adults?

Answer 19

• Unlike ITP in children, adult ITP does not normally follow an infection and usually has an insidious onset. It is more likely to follow a chronic course in affected adults than in children.
• FBC and peripheral blood smear are the essential first-line tests. All those with ITP should be screened for HIV and hepatitis C, as treating the underlying cause may alter the disease course.

Question 20

What are the differentials for ITP in adults?

Answer 20

o	Aplastic anaemia 
o	Leukaemia 
o	Effects of drugs such as alcohol
o	VWD
o	SLE 
o	TTP
o	DIC

Question 21

What is the management of ITP in adults?

Answer 21

• Adults with ITP, like children, will only require active management if their symptoms are severe enough to warrant it. Treatment is initiated in a specialist setting
• First line treatments include observation or steroids. Intravenous immunoglobulin (IVIG) can be used with corticosteroids when a more rapid increase in platelet count is required. Second-line therapies include splenectomy, a thrombopoietin receptor agonist (eltrombopag or romiplostim), or rituximab.
• General measures include stopping medication which has an effect on platelet function such as NSAIDs, controlling blood pressure, inhibition of menses and avoidance of trauma.

Question 22

What is intussusception?

Answer 22

Intussusception is a term derived from the Latin intus (within) and suscipere (to receive). One segment of the bowel (intussusceptum) invaginates into another (intussuscipiens) just distal to it, leading to obstruction.

The bowel may simply ‘telescope’ on itself (non-pathological lead point - up to 75%), or some pathology may be the focus of the invagination (pathological lead point)

Question 23

What is the pathogenesis of intussusception?

Answer 23

• The mesentery of the intussuscepted bowel becomes compressed. The bowel wall distends and obstructs the lumen.
• Peristalsis is disrupted leading to colicky abdominal pain and vomiting. Lymphatic and venous obstruction occurs, causing ischaemia.
• In most children the intussusception is ileocaecal, although ileo-ileocolic and ileo-ileal or colocolic cases can occur.
• Intussusception is the most common cause of intestinal obstruction in patients aged 5 months to 3 years and accounts for up to 25% of abdominal emergencies in children up to age 5.

Question 24

What are the causes of intussusception?

Answer 24

• Non-pathological lead point (>90%):
o Viral 50%- rotavirus, adenovirus and HHV6
o Amoebomata, shigella, yersinia
o Peyer’s patch hypertrophy

• Pathological lead point (<10%): 
o	 Meckel's diverticulum (75%).
o	Polyps and Peutz-Jeghers syndrome (16%).
o	Henoch-Schönlein purpura (3%).
o	Lymphoma and other tumours (3%).
o	Reduplication - a process by which the bowel wall is duplicated (2%).
o	Cystic fibrosis.
o	An inflamed appendix.
o	Ascariasis.
o	Nephrotic syndrome.

Question 25

What is the presentation of intussusception?

Answer 25

• It is usually of sudden onset and may be more insidious in the older child.
• There are paroxysms (about every 10-20 minutes) of colicky abdominal pain (>80%) ± crying.
• The child may appear well between paroxysms initially.
• There is early vomiting - rapidly becoming bile-stained.
• Neurological symptoms such as lethargy, hypotonia or sudden alterations of consciousness can occur.
• There may be a palpable ‘sausage-shaped’ mass (often in the right upper quadrant).
• There may be absence of bowel in the right lower quadrant (Dance’s sign).
• Dehydration, pallor, shock.
• Irritability, sweating.
• Later, mucoid and bloody ‘redcurrant’ stools.
• Late pyrexia.

Question 26

What is the investigations of intussusception?

Answer 26

• FBC:
o May show neutrophilia
• U and Es may reflect dehydration
• AXR shows dilated gas-filled proximal bowel, paucity of gas distally, multiple fluid levels (but may be normal in the early stages.
• Ultrasound - may show doughnut or target sign, pseudokidney/sandwich appearance. It is a very effective modality and many consider it the investigation of choice.
• Bowel enema - barium has been gold standard (crescent sign, filling defect) but air and water-soluble double-contrast now available; each has pros and cons - the choice is left to the individual radiologist.
• CT/MRI scanning - more often used in adults than in children

Question 27

What is the management of intussusception?

Answer 27

• Any child with possible intussusception or other serious cause of abdominal pain should be referred urgently to hospital for further assessment.
• Early diagnosis reduces the need for open surgery.
• Resuscitation - ‘drip and suck’ - nasogastric tube and IV fluids.

•Radiological:
o Reduction (three tries for three minutes each) if there is no sign of peritonitis, perforation or shock.
o Air enema <120 mm Hg of pressure or barium enema.
o The choice of enema is usually left to the radiologist (many now favour air enema).

• Laparotomy (reduction/resection) - indications:
o Peritonitis.
o Perforation.
o Prolonged history (>24 hours).
o High likelihood of pathological lead point.
o Failed enema.

• Hospital admission is usually required but outpatient management may on occasions be an acceptable alternative.

Question 28

What are the complications of intussusception?

Answer 28

• Missed diagnosis.
• Ischaemia of the intussusceptum/intussuscipiens.
• Sepsis and septicaemia.
• Necrosis.
• Haemorrhage.
• Perforation.
• Peritonitis.
• Failure of enema reduction.
• Chronic intussusception

Question 29

What is testicular torsion?

Answer 29

• Torsion of the testis may more accurately be called torsion of the spermatic cord. It causes occlusion of testicular blood vessels and, unless prompt action is taken, rapidly leads to ischaemia, resulting in loss of the testis (germ cells are the most susceptible cell line to ischaemia).
• Testicular torsion is a common urological emergency among adolescent boys and young men.
• The condition typically occurs in neonates or post-pubertal boys but can occur in males of all ages.

Question 30

Which testis is commonly affected?

Answer 30

• The left side is more commonly affected than the right.

Question 31

What are the two types of testicular torsion?

Answer 31

• Two types of testicular torsion may occur: intravaginal and extravaginal.

Question 32

What is intravaginal torsion?

Answer 32

Intravaginal torsion is secondary to the lack of normal fixation of the posterior lateral aspect of the testes to the tunica vaginalis.

This results in the testis being free to swing and rotate within the tunica vaginalis of the scrotum. This defect is referred to as the ‘bell-clapper deformity’. This occurs in 12% of all males and is bilateral in around 40% of cases.

Question 33

What is extravaginal torsion?

Answer 33

Extravaginal torsion occurs more often in neonates and occurs in utero or around the time of birth before the testis is fixed in the scrotum by the gubernaculum.

Consequently, both the spermatic cord and the tunica vaginalis undergo torsion together, typically in or just below the inguinal canal.

Question 34

What are the symptoms of testicular torsion?

Answer 34

• Acute swelling of the scrotum in a boy indicates torsion of the testis until proven otherwise.
• There is typically sudden, severe pain in one testis.
• There may be lower abdominal pain and, in any boy presenting with abdominal pain, the testes should be checked.
• It often comes on during sport or physical activity.
• There is quite often a history of previous, brief episodes of similar pain. This is presumably torsion that corrected itself.
• Nausea and vomiting often occur.
• Occasionally, the symptoms are milder and less acute.
• Easing pain is not necessarily a good sign of spontaneous resolution. Pain also eases as necrosis sets in.
• A history of recurrent attacks of severe pain that resolved spontaneously might suggest intermittent testicular torsion and de-torsion.

Question 35

What are the signs of testicular torsion?

Answer 35

o There is usually reddening of the scrotal skin.
o There is a swollen, tender testis retracted upwards.
o Lifting the testis up over the symphysis increases pain, whereas in epididymitis this usually relieves pain.
o In the early stages, the epididymis may be felt in an abnormal anterior rather than typical posterior position but this depends upon the degree of torsion that may be from 180-720°. Later, gross swelling prevents this finding.
o The testes on both sides are characteristically in the ‘bell-clapper position’ with a horizontal long axis.
o Looking for absence of the cremasteric reflex is a simple method with 100% sensitivity and 66% specificity for testicular torsion.
o The cremasteric reflex (L1/L2 spinal nerves) is elicited by gentle pinching or stroking of the inner thigh while observing the scrotal contents.

If the torsion occurs prenatally, the baby is born with a firm, hard, non-transilluminable scrotal mass. There are no symptoms. The scrotal skin is usually fixed to the underlying necrotic test

Question 36

What are the differentials of testicular torsion?

Answer 36

•Testicular appendage torsion- blue dot sign 
• Epididymo-orchitis
• Hydrocele
• Incarcerated hernia 
• Testicular tumour:
o Scrotal enlargement occurs more slowly
o Rarely accompanied by pain
• Mumps:
o Swelling of the parotid glands 
o Mumps orchitis is rare before puberty

Question 37

What are the investigations for testicular torsion?

Answer 37

• The most important investigation is ultrasound integrated with colour Doppler.
• The use of Doppler ultrasound may reduce the number of patients with acute scrotum undergoing scrotal exploration but it is operator-dependent and can be difficult to perform in prepubertal patients.
• Doppler ultrasound may also show a misleading arterial flow in the early phases of torsion and in partial or intermittent torsion. In addition, persistent arterial flow does not exclude testicular torsion.
• Scintigraphy and dynamic contrast-enhanced subtraction magnetic resonance imaging (MRI) of the scrotum may be used when diagnosis cannot be excluded from history, physical examination and ultrasound.
• Urinalysis may be helpful in borderline cases, to exclude urine infection and epididymitis. However, an abnormal urinalysis does not exclude testicular torsion.
• However, if clinical suspicion is high, surgical intervention should not be delayed for the sake of further investigation

Question 38

What is the management of testicular torsion?

Answer 38

• All cases of acute testicular pain are due to torsion until proved otherwise. If torsion is suspected after a prompt clinical assessment, a scrotal exploration should be carried out without delay.
• An immediate referral must be made to the emergency urology or surgical team. Before further assessment, food should be withheld and patients provided with adequate analgesia.
• It may be possible to reduce the torsion manually. It should initially be done by outwards rotation of the testis unless the pain increases or if there is obvious resistance. Success is defined as the immediate relief of all symptoms and normal findings at physical examination.
• Bilateral orchiopexy is still required after successful detorsion. This should not be done as an elective procedure but rather immediately following detorsion.
• Testicular torsion is an urgent condition, which requires prompt surgical treatment. The two most important determinants of early salvage rate of the testis are the time between onset of symptoms and detorsion, and the degree of cord twisting.

Question 39

What are the complications of testicular torsion?

Answer 39

• Complications of an untreated or delayed torsion include infarction of the testicle with subsequent atrophy, infection and cosmetic deformity.
• Subfertility and infertility may occur. This is caused by the cut-off of blood supply and also by the post-ischaemia-reperfusion injury that is caused after the detorsion when oxygen-derived free radicals are rapidly circulated within the testicular parenchyma.
• However the absence of one testis usually has no significant effect on fertility, provided that the other testis functions normally.

Question 40

What is ALL?

Answer 40

• Acute lymphoblastic leukaemia (ALL) is a malignant transformation of a clone of cells from lymphoid progenitor cells.
• The majority of cases are of B-cell origin but it can also arise from T-cell precursors.
• Lymphoid precursors proliferate and replace the normal cells of the bone marrow and blasts spill into the peripheral circulation.
• Most common cancer in children
• Patients with acute leukaemia typically deteriorate rapidly. Initial presentation is usually generalised fatigue and malaise but usually quickly progresses to bone marrow failure
• Chemotherapy is the choice of treatment

Question 41

What is AML?

Answer 41

• Occurs when myeloid stem cells produce too many immature white blood cells called myeloblasts.

Question 42

What is Ewing’s sarcoma?

Answer 42

• Bone cancer
• Presents with bone pain, tender lump or swelling, pyrexia, lethargy and weight loss.
• Legs, pelvis, arms, ribs and spine are the main areas affected.
• Investigations include xray, blood test, CT and bone biopsy.
• Treatments include radiotherapy and chemotherapy.

Question 43

What is hepatoblastoma?

Answer 43

• Malignant tumour of the liver with an association with familial polyposis coli or Beckwith-Wiedemann syndrome.
• Presents with hepatomegaly, anorexia, weight loss, lethargy, fever and jaundice
• Occurs from immature liver precursor cells and are typically unifocal.
• AFP is a useful biomarker.
• Treatment includes surgery and chemotherapy.

Question 44

What is neuroblastoma?

Answer 44

Mostly affects children under 5 years. Most commonly occurs in the adrenal glands, or in the nerves running along side the spinal cord in the neck, chest, abdomen. Urine test is VMA picks up 90%.

Question 45

What is retinoblastoma?

Answer 45

Retinoblastoma is a tumour of the retina.

It shows up as a white reflex and most commonly affects children under the age of 5. Can be associated with the RB 1 gene.

Question 46

What is Wilms tumour?

Answer 46

Otherwise known as a nephroblastoma . Most common in the under 5.

Often associated with other syndromes eg Beckworth-wiedemann sydrome (larger than normal internal organs).

It presents with a lump in the abdomen, often seen as child jumping up nappy sizes, blood in the urine.

Question 47

What is rhabdomyosarcoma?

Answer 47

This can present with bulging of the eye, but is a soft tissue sarcoma so does not affect the retina. If in the bladder can also present with blood in the urine and obstruction.

Question 48

What is optic glioma?

Answer 48

This is otherwise known as a Juvenile pilocytic astrocytoma.

This occurs in the optic nerve or around the optic chiasm and is a brain tumour.

Its presentation can be visual loss, proptosis, headaches, nausea and vomiting, growth delay due to compression of the hypothalamous and pituiatary gland. It is associated with Neurofibromatosis type 1.

Question 49

What causes head tilt?

Answer 49

• Any condition which causes diplopia and visual loss can present with squints or head tilt as the child tries to compensate.
• This can either be due to localised disease as seen in retinoblastoma, rhabdosarcoma, optic glioma or due to increased intracranial pressure affecting the 6th nerve as in medulloblastoma and again optic glioma.
• ALL can present with facial nerve palsy and a FBC should be done in children presenting with a Bell’s palsy.
• Childhood cancer is relatively rare and frequently has a non-specific presentation. If you are concerned at all that a patient may have a malignancy, then contact the local paediatric unit.

Study picture in the document