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Paediatrics Case Presentation

Donal O' Malley & Hichael Rashid
by

Haikal Mansor

on 26 October 2013

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Transcript of Paediatrics Case Presentation

Case Presentation
Donal O Malley Hichael Rashid
Case History
JE, 8 years of boy of Nigerian origin presented with acute onset of pain in right lower limb
Presentation
Admitted on Friday, 4th October
sudden sharp onset of right knee pain
without history of trauma
no relief with paracetamol or Ibuprofen
Previously well, slight cough and runny nose without fever, chest pain or vomiting
2 days history of constipation
Any Differentials?
Differential Diagnosis
Juvenile Rheumatoid Arthritis
Acute Rheumatic Fever
Infectious Diseases
Rheumatic Diseases
Sickle cell disease
Joint pain
- Systemic lupus erythematous
- Dermatomyositis
- Scleroderma
- Kawasaki's disease
- Septio Arthritis
- Osteomyelitis
- Viral infections
Pain from acute crises
Birth History
Born in UCHG at 40+5 week
Normal vaginal delivery
Weight 3.6 kg
Up to date vaccinations

Past Medical History
Diagnosed with sickle cell anaemia at 9 month
Multiple admissions to hospital since with acute sickle cell crises
Last admission in January 2013 with severe lower back pain
History of Asthma
Viral infection in 2005
Medications
Hydroxyurea since 2010 - antinecoplastic agent
Ventolin - Salbutamol
Calvepen 5ml BD - Phenoxymethylpenicillin
Folic acid 50ml OD
No allergy
Systemic Review
Good diet
Excellent sleep
CNS:
no dizziness, headache
CVS:
no palpitation
Respiratory:
no chest pain, SOB
GI:
no bowel problem
MSK:
no rash, muscle weakeness
Family History
Lives with parents and a 12 years old brother
Family history +sickle cell trait on mother's side, unknown paternal family history
Brother normal
In Class 3
Attends Crumlin Hospital on regular basis
Examination
alert, smart, communicative & not in distress
Vitals
T 36.5
BP 119/61
RR 21
Pain (5th) 3/10
Oxygen saturation 98%
Weight 32kg
EENT -
clear
CVS -
S1+S2, no murmur
Respiratory -
normal
Abdomen-
no tenderness, organomegalies
MSK -
no knee effusion
Skin -
well hydrated, no rash
Investigation
FBC:
WCC
(5.6),
Hb
(10.4),
Platelets
(109)
CRP: 6.1
LFT:
Billirubin
(24),
ALP
(161),
ALT
(48),
GGT
(28)
U&E: Results unknown
Diagnosis
Acute Sickle Cell Crises
Pathophysiology
Possible predisposing factors
Drug therapy insufficient
- Infection
- Dehydration
- Hydroxyurea dose review
Immediate Management
Intravenous fluids
Encourage oral fluids
Analgesia
Repeat bloods
X-ray of right femur
Haemoglobinopathy
Most common genetic disorder in children in many European countries
- Point mutation in codon 6 of the B-globin
- UK: 1 in 2000 live births
Prevalence
Ethnicity: Black
- African or Caribean origin
- Also
* Middle East
* Low prevalence in the rest of the world, apart from Northern Europe
Forms of Sickle Cell disease
Sickle cell anaemia
Sickle Cell disease
Sickle B-thalasaemia
Homozygous for HbA
All HB is HbS, no normal HbA
Inherit HbS + HbC
Also have no normal HbA
Inherit HbS + B-thalasaemia trait
Also have no normal HbA
Why is Sickle Cell trait prevalent in certain areas?
It provides a survival advantage over normal Haemoglobin cell carriers in areas where malaria is endemic, esp. Plasmodium Falciparum
Precise mechanism unknown
- Possibly related to certain enzymes only produced by sickle type cells affecting parasite metabolism and growth.
Pathogenesis
HbS polymerizes within RBCs to form rigid tubular spiral bodies leading to a deformed cell shape
Cells become trapped in microcirculation leading to thrombosis and ishchaemia
Clinical Presentation
Anaemia
Jaundice
Infection Susceptibility
Vaso-occlusive Crises
Splenomegaly
- H. influenzae
- Salmonella
- Hand-foot syndrome: dactylitis
- Organ damage
- Stroke
- Avascular necrosis of femoral head

Long term Complications
Short stature & puberty delay
Cognitive problems
Adenotonsillar hypertrophy
Cardiac enlargement + Heart failure
Renal dysfunction
Pigment gallstones
Psychosocial problems
- Sleep apnea
- Subtle neurological damage
Prophylactic Management
Penicillin BD
Pneumococcal Immunization
Folic acid supplementation
Avoid
- Prevents pneumococcal infection
- Increased demand for folic acid by chronic haemolytic anaemia
- Cold
- Dehydration
- Excessive exercise
- Undue stress
- Hypoxia
Acute Crises
Analgesia
Hydration
Antibiotics if infection precipitated
O2
Exchange transfusion
- Oral or IV as required
- Oral or IV as required
- Low oxygen saturation
- Acute chest syndrome
- Priapism
- Stroke
Long term Treatment
Hydroxyurea
Blood transfusions
Bone marrow transplant
- Increases fetal haemoglobin
- Protect against further crises
- Carried out regularly
- Needs HLA-identical siblings donor
Prognosis
Severe disease:
50% of patients die from complications before the age of 40 years old
3% , Usually from bacterial infection
Childhood mortality:
Prenatal Diagnosis & Screening
Most countries with a high prevalence of haemoglobinopathies perform a neonatal screening
- Blood spot screening test in UK
- Not screened for in Ireland
References
1.Lissauer T, Clayden G. Illustrated Textbook of Paediatrics, 3rd ed. : Mosby; 2007.
2. NICE guidelines. http://www.nice.org.uk/nicemedia/live/12956/59839/59839.pdf
3. Medscape. http://emedicine.medscape.com/article/205926-overview
4. Gong L, Parikh S, Rosenthal P, Greenhouse B. Biochemical and immunological mechanisms by which sickle cell trait protects against malaria. Malaria Journal 2013; 12(317)
A Sickling Animation
A Sickling Animation
Full transcript