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Aetiology

Src: Porter(2004)Acquired non-T1DM in childhood... [free pdf]
Diabetes in childhood: T1:15143pts, T2:24, MODY:17, CF:8, Iatrogenic:6, Wolfram:5, Prader-Willi:4, Neonatal:3, Pancreatectomy:3, Down's:2, others:40;

Algorithm: Child with Diabetes (P-Glu>11.1mM/L random, >7mM/L fasted)

  •  Unwell a/o ketonuria
    • T1DM most likely: start insulin, Ab studies (GAD/IA-2)
    • Review diag at 3+ mth if Ab neg or Insulin dose<0.5u/kg
  • Well ±polyuria/polydipsia ±weight loss; no ketonuria; no systemic features
    • consider DD, if unsure treat with insulin
      • T1DM and latent autoimmune: Ab pos (GAD/IA-2), lean, development of ketonuria: trial of insulin
      • T2DM: overweight, FHx of T2DM, Ab neg, metabol syndr (lipid abnmlties a/o acanthosis nigricans a/o Htn): trial of metformin
      • MODY: usu white, FHx of DM, usu lean, Ab neg: trial of diet (GK) or low dose SU (HNF)
  • Diabetes and disorder
    • iatrogenic DM
      • post-BMT
      • post organ transplant: 43% DM/IGT at 3yrs post transplant: RF: TBI, overweight, insulin-resistant, hypogonadism, tacrolimus, pulsed corticosteroids
      • thalassaemia: insulin-resistance ±beta cell damage/exhaustion
      • steroids: 20% of high-dose steroid recipients
    • other secific disorders
      • CF: CFRD: commonest form of secondary diabetes, in up to 34% in children with CF, usu no ketosis; DM manifesting as deterioration in lung fx and wt loss
      • HNF-1β MODY
      • Wolfram (DIDMOAD): AR: DM (non-autoimmune, <10y), progressive optic atrophy<16y, senorineural deafness, DI, dilated renal tracts, truncal ataxia; median survival; 30y; 90% mutations in Wolfram gene WFS-1
      • Wolcott-Rallison
      • Alström
      • Roger's
      • Bardet-Biedl: retinal dystrophy, obesity, hand abnmlties, renal abnmlties, male hypogonadism, ±mental retardation; 30% DM (insulin-resistant)
      • Mitochondrial
      • Prader-Willi
      • Down's: autoimmune, similar to T1DM; also autoimmune thyr and coel dis
      • Turner's
      • Klinefelter's
    • Insulin resistance syndromes
      • PCOS: menstrual disturb, acne, hirsutism, icrd testosterone, icrd LH
      • Lipodystrophy
      • Type A syndr
      • Donohue syndr (Leprechaunism): severe IUGR, lipodystrophy, fasting hypoglycaemia with postprandial hyperglycaemia: most die <1yr
      • Rabson-Mendenhall
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