Paediatric hypertension and nephrology Flashcards
Phaeochromocytoma
A rare catecholamine secreting tumour. 10% familial (MEN type II, neurofibromatosis or von Hippel-lindau), 10% bilateral, 10% malignant, 10% extra-adrenal. Presents with hypertension, headaches and anxiety. diagnose by 24hr urinary collection of metanephrines and treat with surgery after stabilisation with an alpha-blocker then a Beta-blocker
Causes of Hypertension in children
Renal parenchymal disease (80%) Renal Vascular disease Aortic Coarctation Phaeochromocytoma congential adrenal hyperplasia Essential hypertension (more common as children get older)
Minimal Change disease
75% of Nephrotic syndrome in children and 25% in adults. Drug induced (NSAIDs, rifampicin) in 10-20% of cases. 80% respond to steroids (if not then cyclophosphamide). T cell and cytokine mediated leading to selective proteinuria without hypertension. 1/3 will have 1 relapse, 1/3 will have frequent relapses.
Hypertension with Hypokalaemia
Cushing’s syndrome
Conn’s sydnrome (primary hyperaldosteronism)
Liddle’s Syndrome
11-beta hydroxylase deficiency
Hypokalaemia without hypertension
Diuretic use GI K+ loss (diarrhoea or vomiting) Bartter's syndrome Gitelman syndrome Renal Tubular acidosis
Nephrotic syndromes in Children
Triad of oedema, hypoalbuminaemia (<25g/L) and proteinuria. peak incidence 2-5yrs due to minimal change GN. Can also lead to hyperlipidaemia, hypercoagulable state and increased risk of infection.
Nephritic syndromes in Children
Protein and blood in the urine. Can present with oedema and hypertension, plus poor renal function. Most commonly post-infection with strep in children
Post-infectious glomerulonephropathies
Usually after strep throat in the children (6-10yrs). (1-2wks after, or 3-4wks if skin infection). Causes nephritic syndrome with oligouria, haematuria, oedema and possibly joint pain/stiffness. Usually self resolves and treatment is supportive and symptomatic (steroids or anti-inflammatories) are generally ineffective.
SIADH
A condition of excessive ADH secretion leading to hypervolaemic hyponatreamia with high urinary Na. Can be due to pituitary dysfunction (infection or trauma) or ectopic production (small cell lung cancer). Treat with water restriction and Demeclocycline. Hypertonic saline with furosemide may be needed.
Congential Adrenal hyperplasia
A group of autosomal recessive disorders of cortisol biosynthesis (95% 21-hydroxylase deficiency). Can be with or without aldosterone deficiency and androgen excess. In girls this leads to virilisation and ambiguous genitalia (severe form) or hyperandogenism in early adulthood. Boys can present with hyponatraemia, hyperkalaemia and shock.
Stress/exercise induced Haematuria
Painless, isolated haematuria occuring after vigorous exercise. If long term without a history of exercise it may be benign familial haematuria.
IgA nephropathy
AKA Berger’s Disease
Young adults with haematuria following an URTI
Diffuse proliferative glomerulonephritis
Classically post-strep infection in children presenting with nephritic syndrome. Also the most common form of kidney injury in SLE
UTIs in children
More common in boys up to 3months. In infant under 3months should be immediately referred to a paediatrician, if older then admit or oral antibiotics for 7-10days (if upper) or 3 days if lower. consider prophylaxis if recurrent UTIs
Vesicoureteric reflux (VUR)
abnormal backflow of urine because of change in the angle the ureters enter the bladder. 30% of children with a UTI and 35% of children suffer with scaring so should be investigated with a micturating cystourethrogram and DMSA if concerned about renal scarring
