P year‎ > ‎Paediatrics‎ > ‎

12.09.25 Ward round + teaching notes

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
    • => Permanent latency


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
      • FAB subtype M3
  • 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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