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Haematology Chapter 5: Paediatrics 137
https://t.me/med1917
Management: → avoid NSAIDs & IM injections
For all acute bleeds: elevation, compression & tranexamic acid
• Mild (10–50%): PO/IV tranexamic acid ± SC or nasal desmopressin (DDAVP)
• Moderate/severe: recombinant vWF
ACQUIRED BLEEDING DISORDERS
1. VITAMIN K DEFICIENCY = causes haemorrhagic disease of newborn
Causes: inadequate intake, malabsorption, warfarin
Prevention: recommended that all newborns have
vitamin K after birth
2. THROMBOCYTOPENIA = platelets <150 × 109/L
Clinical features
• Bruising, petechiae, purpura
• Mucosal bleeding – epistaxis & gums
• Less common: GI haemorrhage, haematuria, intracranial bleed
3. IMMUNE THROMBOCYTOPENIC PURPURA (ITP) = most common
thrombocytopenia in childhood (4 in 100,000 per year)
Cause: destruction of circulating platelets by IgG autoantibodies → following viral
infection
Investigations: diagnosis of exclusion (child is well)
• Careful Hx & examination
• FBC & blood film – platelets = only abnormality
• Bone marrow examination – to exclude
leukaemia/aplastic anaemia if ATYPICAL FEATURES
& fail to respond to treatment
Management
A) Benign, self-limiting (80%) → resolves in 6–8w
• If major bleed / persistent minor bleed: oral prednisolone or IV Ig
• If life-threatening haemorrhage: platelet transfusion
B) Chronic ITP (20%) → persistently low platelets >6m
• If major bleed / persistent minor bleed: oral prednisolone or IV Ig
• Persistent severe bleeding (rare): monoclonal antibodies, splenectomy
Signs: PT & & APTT
excess bleeding
neutropenia/anaemia
hepatosplenomegaly
lymphadenopathy
Vit K needed for factors II, VII, IX, X
Plts × 109/L Symptoms
Severe <20 Spontaneous bleeds
Mod 20–50 Excess bleeds in
operations/trauma
Mild 50–150 Low risk of bleeding
→ Peak age of ITP: 2–10y
→ Onset 1–2w post viral infection
General advice for ITP:
1. Avoid NSAIDs & IM injections
2. Avoid contact sports if platelet count is low
Thrombotic thrombocytopenic purpura (TTP)
= enzyme activity = clotting
= schistocytes on blood smear
4. DISSEMINATED INTRAVASCULAR COAGULATION
= coagulation pathway activation → diffuse fibrin deposition & consumption of
coagulation factors & platelets
Causes
• Severe sepsis (infection)
• Trauma/burns
• Malignancy (especially AML)
• Toxins
Symptoms
Bruising, purpura, haemorrhage (can be life-threatening)
Management
1. Treat underlying cause e.g. sepsis
2. Supportive: FFP, platelets, cryoprecipitate (fibrinogen, vWF, other coagulation
factors)
Diagnosis: clotting screen
→ platelets, fibrinogen, d-dimers
→ antithrombin, proteins C & S
→ APPT & PT
→ Blood film: fragments (schistocytes)
SPLENECTOMY
Pre-op: vaccinations (Hib, meningococcus,
pneumococcus)
Post-op: lifelong low dose ABX (+ rescue high
dose ABX on standby)
Complications:
Acute: bleeding, wound infection, VTE
Long term: infection from encapsulated
bacteria e.g. streptococcus, Hib, meningococcus
Indications:
• Hereditary spherocytosis
• SCA
• ITP
• Lymphoma
• Trauma (splenic rupture)
138 Chapter 5: Paediatrics
https://t.me/med1917
Paediatric oncology
↳ 1 in 500 children <15y develop cancer
Side-effects of chemotherapy
1. Bone marrow suppression
(infection, anaemia, bleeding/bruising)
2. GI disturbance
(N&V, anorexia, undernutrition, mucositis)
3. Other: cardiotoxicity, neurotoxicity,
nephrotoxicity, ototoxicity, hair loss
4. Long-term: fertility, late/early puberty
Red flags of lymphadenopathy:
• LNs >2cm in diameter
• No decrease in size after 4–6w
• Any supraclavicular node
• Abnormal CXR
• Systemic Sx (fever, weight loss, night sweats,
hepatosplenomegaly)
Prognosis: approx. 90% cure rate
↳ 82% 5-year survival rate (most ‘cured’ by then)
↳ 2nd biggest killer of children 1–14y (after infection)
↳ Leukaemia & brain tumours are most common
Leukaemia
→ cancer of white blood cells
ACUTE LYMPHOBLASTIC LEUKAEMIA ALL = 80% of childhood leukaemia
→ peak age 2–5y
↳ Rapidly proliferating lymphocytes resulting in accumulation of abnormal,
immature ‘blast’ cells (B or T lymphocytes)
Symptoms: onset over several weeks
• General: malaise, anorexia, lymphadenopathy, hepatosplenomegaly
• Anaemia: lethargy, pallor, SOB
• Neutropenia: infections → risk of neutropenic sepsis
• Thrombocytopenia: bruising, petechiae, nose bleeds
• Bone marrow infiltration: bone pain
• CNS infiltration: headaches, vomiting
Investigations
• FBC: Hb, plts, neutrophils, or WCC
• Peripheral blood film: blast cells
• Clotting screen: 10% have DIC
• Bone marrow biopsy → diagnostic & prognostic
• CXR: mediastinal mass (characteristic of T-cell disease)
RF = Down’s
Before starting Tx: correct
thrombocytopenia (+ severe anaemia),
treat infections, renal protection
(allopurinol)*
*prevents tumour lysis syndrome
Poor prognostic factors
Age <1y or >10y
WBC >50 × 109/L
High risk genetic features of tumour cells
CNS involvement
Inadequate response to induction chemo
Fig. 5.26 ‘Blast’ cells.
Cytotoxic drugs don’t penetrate CNS, so
Management
1. Chemotherapy: including steroids and intrathecal chemotherapy
→ 2y for girls, 3y for boys
→ High risk patients receive more intensive chemotherapy
2. Prophylactic co-trimoxazole: given throughout treatment to prevent
PCPinfection
3. Newer treatment options: CAR-T cell therapy & monoclonal antibodies
4. Relapse treatment: consider bone marrow transplant ± CAR-T cell therapy
26
Children’s Cancer and Leukaemia Group (CCLG) (2017) Acute lymphoblastic leukaemia
26
intrathecal chemo (methotrexate) also given
Paediatric oncology Chapter 5: Paediatrics 139
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Lymphoma
→ solid malignancies of immune system → lymphocyte proliferation in LNs, spleen,
thymus, bone marrow
Investigations & diagnosis
• FBC & blood film
• Lymph node biopsy – RS cells in HL
• Bone marrow biopsy – for NHL
• ESR – helps staging of HL
• Lumbar puncture – helps staging of NHL
• MRI/CT/PET-CT – assess nodal sites
• CXR – check for mediastinal mass
Management
• Chemotherapy (± radiotherapy
if HL)
• PET to monitor Tx response
HODGKIN LYMPHOMA 20% = Reed–Sternberg cells on LN biopsy → more
common in adolescents (rare <5y)
Symptoms
• Painless lymphadenopathy
▶ usually cervical
▶ may cause mass effects (SVC/bronchial obstruction)
• B symptoms in 40%
▶ fever, drenching night sweats, unexplained weightloss
Usually systemically well at presentation
(need irradiated blood products)
Hodgkin lymphoma (HL) = Reed–Sternberg
cells present
Non-Hodgkin lymphoma (NHL) = no Reed–
Sternberg cells
Prognosis: >80% cure rate for both:
Hodgkin (HL) slightly better than non-Hodgkin
(NHL)
NONHODGKIN LYMPHOMA 80% = no Reed–Sternberg cells → more
common in childhood & males
Symptoms
• Painless lymphadenopathy
▶ cervical, thoracic, or abdominal
▶ often cause mass effects – mediastinal mass (SVC/bronchial obstruction)
• Abdominal symptoms
▶ palpable abdominal mass
▶ pain from bowel obstruction / intussusception if ileum involved
• B symptoms in 20%
More likely to be
systemically unwell at
presentation
Fig. 5.27 Reed–Sternberg cells → binucleate
High grade lymphomas: divide rapidly
• Rapid onset lymphadenopathy
• Weight loss, fever, pressure effects
Low grade lymphomas: divide slowly
• More insidious onset of symptoms
Brain tumours
→ #1 cause of childhood cancer deaths in UK → almost always primary
Tumour Location Symptoms (depend on location & age)
Low grade astrocytoma (40%) benign Cerebral hemispheres Seizures, headaches, focal signs
Medulloblastoma (20%) malignant Posterior fossa (cerebellum) spread via CSF Ataxia, abnormal eye movements
Ependymoma (5–10%) malignant Posterior fossa (cerebellum) Ataxia, abnormal eye movements
Brainstem glioma (5–10%) malignant Cranial nerve & pyramidal tract Ataxia
Craniopharyngioma (4%) benign From remnant of Rathke’s pouch Bitemporal hemianopia, pituitary Sx
‘mirror-cells’.
MANAGEMENT
• Surgery + chemo ± radiotherapy
• Steroids (for pressure Sx)
• Anti-epileptics (for seizure Sx)
• Rehab
General Sx of raised intracranial
pressure / hydrocephalus:
• Headaches
• Morning vomiting
• Drowsy/irritable
• Seizures
• Vision disturbance
140 Chapter 5: Paediatrics Paediatric oncology
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Neuroblastoma
most common in children <5y
Investigations
→ FBC
→ Urine: catecholamines
→ USS, CT/MRI abdo
→ Biopsy: diagnostic
→ Bone marrow biopsy, MIBG scan: detect mets
→ tumour arising from neural crest tissue in adrenal gland or sympathetic nervous system
SYMPTOMS
• Abdominal mass: usually adrenal gland BUT could be anywhere along
sympathetic chain from neck → pelvis
• Metastatic Sx: pallor, weight loss, malaise, bone pain, cervical
lymphadenopathy, constipation
• Orbital disease: proptosis, ‘bruises’ under eyes, eye movement disorders
• Cutaneous: ‘blueberry muffin’ rash
COMPLICATIONS
• Spinal cord compression (if paravertebral): paraplegia, Horner’s
• Adrenal tumour: hypertension
MANAGEMENT
1) Low risk:
• Observation only (if subtype ‘Ms disease’ in children <18m, which can
spontaneously regress)
• Chemotherapy/surgery
2) High risk: poor prognosis (40–50% survival)
• Induction chemo → surgery → high dose chemotherapy + radiotherapy +
immunotherapy
27,28
usually <5y (rare >10y)
5% present BILATERALLY →
suspect underlying genetic syndrome
e.g. Beckwith–Wiedemann
20% already metastatic at presentation → lungs
Prognosis for Wilms tumour: approx. 85%
cure rate (60% if distal mets at presentation)
Prognosis for soft tissue sarcomas:
approx. 65% cure rate (worse if present with
distal mets)
Wilms tumour (nephroblastoma)
→ tumour of embryonal renal tissue
SYMPTOMS
• Large abdominal mass: often only symptom
± abdo pain, anorexia, anaemia, haematuria, HTN
INVESTIGATIONS
• USS – intrarenal mass
± CT/MRI – assess for mets (especially to lungs)
MANAGEMENT
Pre-op chemo → nephrectomy → post-op chemo ± radiotherapy
Soft tissue sarcomas
→ tumours of connective tissue (muscles, tendons, fat, nerves, etc.)
RHABDOMYOSARCOMA = most common → highly malignant cancer of
muscle & fibrous tissue
Symptoms: can develop anywhere in body – symptoms depend on location
(usually painless lump)
Most common locations in children:
• Head & neck (40%): proptosis, nasal obstruction / bloody discharge
• Genitourinary: dysuria, haematuria, urinary obstruction, scrotal/perineal
mass, bloody PV discharge
Investigations
→
Biopsy
→ MRI/CT/PET to assess metastases → lung, liver, bone marrow
→ Bone marrow biopsy
Management
Chemotherapy ± surgery ± radiotherapy
27
CCLG (2020) Low and intermediate risk neuroblastoma treatment guidelines
28
CCLG (2019) Statement from CCLG Neuroblastoma SIG: Treatment and management of patients with
high-risk neuroblastoma
Paediatric oncology Chapter 5: Paediatrics 141
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Malignant bone tumours
→ osteosarcoma & Ewing sarcoma
M>F
OSTEOSARCOMA = more common in older children
→ affects end of long bones (hip, shoulder, knee)
EWING SARCOMA = more common in younger children
→ usually affects legs, back, pelvis, ribs, skull
Symptoms
• Persistent, localised bone pain & limp
• Swelling & pathological fracture
• Mass on X-ray – often substantial soft tissue mass in Ewing’s
Investigations
• Bone X-ray – destruction + new bone formation
• MRI
• Chest CT – assess mets
• Biopsy of lesion
Management
• Chemotherapy then surgery*
• Radiotherapy for Ewing sarcoma
*limb-sparing surgery > amputation where possible
Retinoblastoma
→ malignant tumour of retinal cells
CAUSE: de novo or inherited (40%) mutation in chromosome 13 → all
bilateral & 20% unilateral cases are inherited
SYMPTOMS: ‘SILVER’
Strabismus
Iris discolouration
Leukocoria → white pupillary reflex (instead of red)
Vision changes
Enlarged pupil
Red/sore/swollen eye
+ systemic Sx if mets present
(autosomal dominant)
Ewing:
‘moth-eaten’ appearance on radiograph
Red flags for bone tumours
• Progressive pain
• Night pain / pain wakes child up
• Reluctant to weight bear / stopped walking
usually in children <3y
INVESTIGATIONS
• MRI & examination under anaesthetic
• Lumbar puncture and bone marrow biopsy (for CSF spread)
COMPLICATIONS
• Severe visual impairment
• Cosmetic deformity
• 2° malignancy in future (bone/soft tissue, melanoma, breast/bladder)
MANAGEMENT
Chemo followed by local Tx e.g. surgery/radiotherapy
Fig. 5.28 Leukocoria.
142 Chapter 5: Paediatrics
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Nephrology
Bilateral hydronephrosis / suspected
bladder obstruction in males needs urgent
investigation with MCUG to r/o posterior
urethral valves
Congenital abnormalities
1 in 300 births
→ most detected in utero
→ given prophylactic ABX at birth to prevent UTI
RENAL AGENESIS = absence of both kidneys
• Result: oligohydramnios (as no fetal urine)
• Complications: FATAL POTTER SYNDROME
MULTICYSTIC DYSPLASTIC KIDNEY (MCDK)
• Cause: failed union of ureteric bud & renal mesenchyme
• Result: non-functioning kidney with variably sized fluid-filled cysts
& narrow ureter
• Complications: Potter syndrome if bilateral
• Mx: most involute by 2y (no Tx needed) → nephrectomy if remain large or
HTN develops
POLYCYSTIC KIDNEY DISEASE PKD = always bilateral but some/normal
renal function retained
• Cause: autosomal dominant (1 in 1000) or autosomal recessive
• Result: enlarged kidneys with separate, discrete cysts
• Complications: HTN, haematuria, renal failure
*false +ves if within 3m of UTI
HORSESHOE/PELVIC KIDNEY = lower poles are fused in the midline
• Complications: risk infection or obstruction
DUPLEX KIDNEY = bifid renal pelvis or 2 complete ureters for 1 kidney
• Complications: reflux in lower ureter, ectopic drainage / prolapse of upper
ureter (e.g. into vagina)
POSTERIOR URETHRAL VALVES = most common cause of outlet
obstruction in male newborns
• Cause: abnormal flaps of tissue / membranes grow in urethras, obstructing
drainage
• Complications: hydronephrosis / small, dysplastic, non-functioning
kidney / amniotic fluid (Potter syndrome)
• Mx: cystoscopic ablation of valves
Assessing the urinary tract:
1. eGFR = low in newborns (rises rapidly in 1–2y)
2. Plasma Cr = s through childhood with height & muscle
3. USS = visualise abnormalities, calculi & deposits
4. DMSA scan = static scan of renal cortex → shows functional defects e.g. scars*
5. MCUG (micturating cystourethrogram) = fill bladder with contrast medium to visualise
anatomy & flow direction → shows vesicoureteric reflux & obstructions
6. MAG3 renogram = measure drainage of isotope-labelled MAG3 → shows vesicoureteric
reflux & obstruction
NB Plain AXR
not very useful to show stones/deposits
Nephrology Chapter 5: Paediatrics 143
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Urinary tract infections
CLINICAL PRESENTATION → varies with age (non-specific in infants)
Infant Child
• Fever
• Vomiting
• Lethargic/irritable
• Poor feeding / FTT
• Jaundice
• Septicaemia
• Offensive urine
• Fever
• Abdo/loin pain
• Frequency/accidents
• Dysuria/haematuria
• Lethargic
• Vomiting/anorexia
• Offensive urine
Pyelonephritis / upper UTI
1. fever >38°C + bacteriuria OR
2. fever <38°C + bacteriuria + loin pain
Cystitis / lower UTI
Bacteriuria without systemic Sx (fever)
Collecting urine samples
Infants in nappies
• ‘Clean-catch’ – into prepared pot as nappy is removed → recommended method
• Adhesive plastic bag – applied to perineum after washing → contamination likely
• Urethral catheter – especially if need sample urgently
• Suprapubic aspiration (SPA) – needle into bladder via US guidance → invasive ( use)
Older children
• MSU – careful cleaning & collection avoids contamination
INVESTIGATIONS
1. Urine dip – screening test
→ NITRITES = SPECIFIC
→ LEUKOCYTES = SENSITIVE
2. MCS – ALWAYS NEEDED unless BOTH leukocytes & nitrites –ve
→ To confirm diagnosis: single org. >105 per ml
→ To determine organism: guides Tx
Prevalence <6y:
Females: 3–7% Males: 1–2%
Causative organisms:
E. coli
Klebsiella
Pseudomonas
Strep. faecalis
Proteus
Proteus causes alkaline urine &
risk phosphate stones
Nitrites Leukocytes Interpretation
+ + Confirms UTI
+ – Start ABX & confirm
with MCS
– + Only start ABX if
clinical Sx of UTI
– – Repeat sample
SPECIFIC: +ve result = likely UTI, but –ve result
does not rule out UTI
SENSITIVE: –ve result = unlikely UTI, but +ve
result may be another cause (e.g. other febrile
illness, balanitis/vulvovaginitis)
3. Further investigations for cause – ONLY if recurrent / atypical UTIs
• USS: shows structural abnormalities, renal defects, obstruction
• DMSA ± MCUG or MAG3: for scarring, VUR or obstruction
MANAGEMENT
29
↴
NB
then no further Ix
<3m: URGENT HOSPITAL ADMISSION
• IV ABX
>3m with upper UTI / pyelonephritis:
• PO ABX for 7–10d
>3m with lower UTI / cystitis:
• PO ABX for 3d
PREVENTION
• Well hydrated so high urine output (1–1.5L/day)
• Regular voiding & complete emptying – encourage double voiding
• Prevent/treat constipation
• Perineal hygiene – wipe front to back / foreskin hygiene
• Probiotic – Lactobacillus acidophilus = pathogens in gut
→
Prophylactic ABX (trimethoprim/nitrofurantoin)
previous upper UTI or severe VUR
29
NICE (2007, updated 2018) Urinary tract infection in under 16s [CG54]
– if age <2y with congenital abnormality or
Atypical UTIs
• Severely ill or septicaemia
• Poor urine flow
• Abdo/bladder mass
• No Tx response in 48h
• creatinine
• Non-E. coli organism
• FHx urinary tract abnormality
IV antibiotics if:
→ Treatment-resistant pyelonephritis
(no response in 48h)
→ Urosepsis
144 Chapter 5: Paediatrics Nephrology
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Vesicoureteric reflux (VUR)
→ developmental anomaly of vesicoureteric junctions
CAUSE
• Familial = 30–50% risk if first-degree relative
• Bladder pathology – neuropathic bladder, urethral obstruction, post-UTI
TYPES
1. Mild – reflux into ureter only → usually not significant
2. Severe – reflux into kidney → grossly dilated ureter & renal scarring
INVESTIGATIONS
• MCUS: shows dilated ureters & direction of flow → diagnostic (shows grade
of reflux)
• MAG3: shows direction of flow
• USS: show abnormalities/obstruction
MANAGEMENT
Mild: usually resolves within first few years of life
Severe: prophylactic ABX, surgery
Fig. 5.29 Bilateral VUR showing dilated
ureters and dilated, clubbed calyces.
COMPLICATIONS: (how it presents)
• Incomplete bladder emptying = risk of UTI
• Intrarenal reflux = risk of pyelonephritis → infection causes renal scarring
• High voiding pressures = transmitted back to kidneys → damaged renal papillae
Haematuria
CAUSES
Glomerular: brown urine, deformed RBCs, + protein
• Acute/chronic glomerulonephritis (+ proteinuria)
• IgA nephropathy
• Familial nephritis (Alport syndrome)
• Goodpasture syndrome (anti-basement membrane)
• Idiopathic/familial idiopathic
Non-glomerular: red urine, no protein
• Infection
• Trauma (to genitals/urinary tract/kidneys)
• Stones
• Tumours
• Renal vein thrombosis
• Sickle cell disease
• Bleeding disorders
INVESTIGATIONS
All patients with haematuria If suspect glomerular haematuria
• Urine dip & MCS
• FBC, ESR, platelets, clotting screen
• Sickle cell screen
• U&Es, Ca, phosphate, albumin
• USS KUB
• Complement levels – often
• Anti-DNA antibodies (ANCA present in vasculitis)
• Throat swab + antistreptolysin O/anti-DNAse B
• Hep B/C screen
• Renal biopsy – if atypical features / severe Sx
Nephrology Chapter 5: Paediatrics 145
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Nephritic syndrome
→ inflammation causes glomerular cellularity → restricts blood flow = filtration
1. HAEMATURIA (>10 red cell casts)
2. OLIGURIA (<0.5–1ml/kg/h)
3. PROTEINURIA (>3.0g/d)
Clinical features: volume overload
• Oedema (especially periorbital)
• Hypertension → seizures
Causes
• Post-infection (streptococcal)
• Vasculitis (HSP, SLE, Wegener granulomatosis)
• IgA nephropathy
• Membranoproliferative glomerulonephritis
• Goodpasture syndrome (anti-basement membrane antibodies)
Management
• Diuretics – maintain fluid & electrolyte balance
• Treat infection if present – ABX (penicillin)
• If rapid renal function: = renal biopsy, immunosuppression, plasma
exchange
Streptococcal nephritis:
7–21d after sore throat or skin infection
→ Low complement
→ Raised antistreptolysin-O (ASO)/anti-DNAse B
HENOCHSCHÖNLEIN PURPURA HSP: circulating IgA & IgG form
complexes that deposit in organs (kidneys, skin, joints)
Clinical features
• Palpable purpuric rash – symmetrical over buttocks & extensor surfaces/
ankles
• Arthralgia & periarticular oedema (65%)
• Colicky abdominal pain (50%)
• Glomerulonephritis – micro-/macroscopic haematuria (80%)
ANCA antibodies
→ ± fever & malaise
Complications: rare
• GI petechiae → haematemesis/melaena
• Intussusception → redcurrant jelly stools
• Nephrotic syndrome – if severe proteinuria
Management: normally self-resolves in 6w
• Analgesia* – paracetamol
• Corticosteroids (immunosuppressants) – ONLY if severe gut/joint involvement
IGA NEPHROPATHY BERGER’S DISEASE: similar pathology to HSP
(IgA vasculitis) but only affects kidneys
→ RFs: URTI, male, Asian, FHx
→ Symptoms: macroscopic haematuria, proteinuria, peripheral oedema, HTN
→ Investigations: bloods, urinalysis, renal biopsy
→ Management: same as HSP (analgesia, corticosteroids ± diuretics)
SYSTEMIC LUPUS ERYTHEMATOSUS SLE: dsDNA autoantibodies
(+ others), complement
→ RFs: adolescent girls / young women, Asian/Afro-Caribbean
→ Symptoms: haematuria & proteinuria (+ malar rash, malaise, arthralgia)
*avoid NSAIDs!
Risk factors for HSP
• 3–10y
• M>F (2:1)
prevalence of HSP = 1 in 5000
HSP needs follow-up in 1y if renal
involvement to ensure no CKD!
Glomerulonephritis: group of diseases causing
immune-mediated inflammation of glomerulus
( permeability) → post-strep (HSP), other
infections (malaria/staph/salmonella/candida),
IgA nephropathy, SLE
Sx: nephrotic/nephritic syndrome
Tx: diuretics ± steroids ± ABX
• Winter
• Preceding URTI (strep)
Fig. 5.30
146 Chapter 5: Paediatrics Nephrology
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Nephrotic syndrome
→ inflamed basement membrane allows passage of proteins into nephron
1. Heavy proteinuria (>200mg/d) = frothy urine
2. Hypoalbuminaemia (<25g/L)
3. Oedema – periorbital, legs, scrotal/vaginal
Complications of nephrotic syndrome
• Hypovolaemia = abdo pain, faintness, low
+
urine Na
• Thrombosis – exacerbated by steroid therapy
• Infection – especially pneumococcus (Igs lost
in urine)
• Hypercholesterolaemia/hyperlipidaemia
Prognosis of nephrotic syndrome
/ resolve directly
/ have infrequent relapses
/ have frequent relapses
NB Relapses identified by parents doing urine dip
Clinical features
• Periorbital oedema (especially on
waking)
• Leg, ankle, scrotal/vulval oedema
• Ascites
• Breathless (due to pulm. effusions)
Cause: unknown
→ may be 2° to systemic disease
(HSP, SLE, infections, etc.)
Investigations
• Urine dip – protein ++
• FBC, ESR
• U&Es, albumin
• Urine MCS
• Complement levels
• Throat swab/anti-strep
• Hepatitis B & C screen
• Malaria screen
STEROIDSENSITIVE NEPHROTIC SYNDROME (85–90%)
Risk factors: male, Asian, 1–10y, PHx atopy, preceding respiratory infection
Features: minimal change disease
• No macroscopic haematuria
• Normal BP, complement levels & renal function
• ‘Minimal change’ on histology
Management
30
1. Corticosteroids (8w PO) – prednisolone
• High dose oral steroids (then taper dose)
• Tx-resistant cases need renal biopsy
• Frequent relapses need high maintenance dose steroids
2. Fluid balance
• Restrict intake ± diuretics
• IV fluids if hypovolaemic
Educate parents on how to do urine
infection (risk of taking steroids)
3. ABX prophylaxis (penicillin V)
• As loss of Igs leaves them
susceptible to infection
→ Refer to specialist if atypical
features
→
• <1y or >12y
• HTN or impaired renal function
• Gross haematuria
• Hep B/C +ve
• Tx-resistant
STEROIDRESISTANT NEPHROTIC SYNDROME (rare)
Causes
• Focal segment glomerulosclerosis – most common
• Membranoproliferative – haematuria & low complement
• Membranous nephropathy – associated with hepatitis B
Management: → early referral to paediatric nephrologist
1. Fluid balance
• Restrict intake of water and salt
• Diuretics, ACEis
2. Pharmacological therapies
• Pulsed methylprednisolone
• Other immunosuppressants (e.g. tacrolimus, cyclophosphamide)
30
NIDDK (2021) Nephrotic syndrome in children
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