Hemophagocytic lymphohistiocytosis (HLH)- Aggressive proliferation of activated macrophages and histiocytes, which
phagocytose other cells, namely RBCs, WBCs, and platelets
- Commonly appears in infancy, although
it has been seen in all age groups
- Features:
- Fever, hepatosplenomegaly,
pancytopenia, lymphadenopathy, rash
- Cutaneous involvement occurs in as many as 65% of patients
- Subtypes:
- Primary hemophagocytic
lymphohistiocytosis (ie, familial erythrophagocytic lymphohistiocytosis
[FEL])
- Heterogeneous autosomal recessive disorder found to be
more prevalent with parental consanguinity
- Secondary hemophagocytic
lymphohistiocytosis (ie, acquired hemophagocytic lymphohistiocytosis)
- Occurs after strong immunologic activation, such as that which can occur
with systemic infection, immunodeficiency, or underlying malignancy
- Both forms are characterized by the overwhelming activation of normal T
lymphocytes and macrophages
- Invariably leads to clinical and
haematologic alterations and death in the absence of treatment
Macrophage-activation syndrome (MAS)
- Similar + closely related to HLH
- Severe, potentially
life-threatening, complication of several chronic rheumatic diseases of
childhood
- Associations:
- Systemic-onset juvenile idiopathic arthritis (SoJIA)
- SLE
- Kawasaki disease
- Adult-onset Still's disease
Epstein–Barr virus (EBV)- AKA human herpesvirus 4
- Half of all five-year-old children and 90 to 95 percent of adults have evidence of previous infection
- Causes few or no symptoms in children
- Causes infectious mononucleosis 35 to 50 percent of the time if infected for the first time during adolescence
- Infects B cells + epithelial cells
Duplex kidney
- Duplicated kidney
- May have its own ureter
- Often associated with vesicoureteric reflux
- => Risk of pyelonephritis
Special feeds
- Peptisorb
- Nutritionally complete semi elemental tube feed
- For patients with impaired gastrointestinal function
- Neocate
- Nutritionally complete amino-acid based powdered infant formula, with
long chain polyunsaturated fatty acids (LCP), DHA and ARA
- For the
dietary management of infants with cow milk allergy
Drugs and chemicals- VMA
- Vanillylmandelic acid
- End-stage metabolite of adrenaline + noradrenaline
- Check in pheochromocytoma, neuroblastoma etc
- Fluconazole
- Triazole antifungal drug used in the treatment and prevention of superficial and systemic fungal infections
- Amikacin
- Aminoglycoside antibiotic
- Octreotide
- Somatostatin analogue
- Treatment of severe, refractory diarrhea (off-label)
- Flixotide = Fluticasone inhaler
- Montelukast
- Leukotriene receptor antagonist (LTRA)
- Atrovent: Ipratropium bromide
- Klean-Prep
- Macrogol
- AKA polyethylene glycol
- => Osmotic diarrhoea
- Also sodium sulphate, sodium bicarbonate, sodium chloride and potassium
chloride to replace those lost
- Used to empty bowel
Reticulocyte production index - Start with the % of reticulocytes
- Correct for:
- Haematocrit (as in anaemia the proportion should be higher)
- Longer life span of immature reticulocytes
- Reticulocyte index (RI) should be between 1.0% and 2.0% for a healthy individual.
- RI < 1% with anemia indicates decreased production of reticulocytes and therefore red blood cells
- RI > 2% with anemia indicates loss of red blood cells
(destruction, bleeding, etc.) leading to increased compensatory
production of reticulocytes to replace the lost red blood cells
Water deprivation test
- Used to distinguish cause of polyuria / polydipsia
- Deprive of water:
- Primary polydipsia => Urine concentrated (fixed the problem)
- Diabetes insipidus => Urine still dilute
- Then can continue test, but add exogenous desmopressin (ADH):
- Central DI => Urine concentrated (fixed the problem)
- Nephrogenic DI => Urine still dilute (can't respond to ADH)
Notes- POSCU
- Paediatric Oncology Shared Care Unit
- APML
- Acute promyelocytic leukaemia
- AML subtype
- Histiocyte
- Part of the mononuclear phagocyte system
(also known as the reticuloendothelial system or lymphoreticular
system)
- Histiocytes are:
- Tissue macrophages
- Dendritic cells
- Langerhans cells
- NCEPOD
- National Confidential Enquiry into Patient Outcome and Death
- Sarcomas arises from mesenchymal tissue
- i.e. Bone, cartilage, fat, muscle, vascular, hematopoietic
- Mesothelium
- Protective lining that covers many of the internal organs of the body
- Kidney scans:
- DMSA (Static)
- Most reliable test for the diagnosis of acute pyelonephritis
- Other indications:
- Renal scar, small or absent kidney, occult duplex system, renal masses, systemic hypertension or suspected vasculitis
- MAG3 (Dynamic)
- VIW: Viral induced wheeze
- MCUG test
- Micturating cystourethrogram
- To check for vesicoureteric reflux
- DIB: Difficulty in breathing
- Mastoiditis
- Inflammation of mastoid process
- Part of the temporal bone behind the ear
- Contains open air-containing spaces
- Can be caused by untreated otitis media
- Pinnaplasty
- Correction of prominent ears
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