Paediatrics NOTES 2 Flashcards
What causes chickenpox?
Primary infection with the varicella zoster virus
What is shingles?
The reactivation of the dormant virus in the dorsal root ganglion
How is chickenpox spread?
Via respiratory route, can be caught from someone with shingles
When is chickenpox infectious?
4 days before the rash, until 5 days after it appears
What is the incubation period of chickenpox?
10-21 days
What is the disease course for chickenpox?
Prodrome fever. Then itchy rash, starting on head/trunk then spreading. Initially macular then papular then vesicular.
When is chickenpox no longer infectious?
When the lesions are dry and have crusted over
Who should receive varicella zoster immunoglobulin?
Immunocompromised patients and newborns with peripartum exposure
What lotion should be given to help calm chicken pox scratching?
Calamine
When should IV aciclovir be considered in chickenpox?
immunocompromised patients and newborns with peripartum exposure who have recieved varicella zoster immunoglobulin (VZIG). If chickenpox develops then IV aciclovir should be considered
What is a common complication of chickenpox?
Secondary bacterial infection of the lesions
What might increase the risk of secondary bacterial infection of chickenpox lesions?
NSAIDs
If there is secondary bacterial infection of chickenpox, what can then happen to the infection?
Commonly it is just a small area of cellulitis, but in a small number of patients, invasive group A streptococcal soft tissue infections may occur resulting in necrotizing fasciitis
What are the possible complications of chickenpox?
Pneumonia, encephalitis, arthritis, nephritis, pancreatitis
When does chicken pox infection typically occur?
ages 1 to 6 years, maximal transmission during winter and spring
What are the features of fetal varicella syndrome (if baby encounters chickenpox whilst in utero)?
Skin scarring, eye defects, limb hypoplasia, microcephaly, learning disabilities
Who should recieve vaccination against primary varicella infection?
- healthcare workers who are not already immune to VZV
- contacts of immunocompromised patients
What dermatomes are most commonly affected by shingles?
T1-L2 (trunk)
What are the prodromal period for shingles?
Burning pain over the affected dermatome for 2-3 days, pain may be severe and interfere with sleep
What is the characteristic rash of shingles?
Initially erythematous, macular rash over the affected dermatome. Becomes vesicular Well demarcated by the dermatome
When are patients with shingles infectious?
Until the vesicles have crusted over
What causes diphtheria?
Gram positive corynebacterium diphtheriae
What is the diphtheric membrane seen in diphtheria infection?
Membrane on tonsils caused by necrotic mucosal cells. Grey, pseudomembrane on the posterior pharyngeal wall
What is the presentation of diphtheria?
Sore throat with diphtheric membrane, bulky cervical lymphadenopathy, high temperature, sore throat, difficulty breathing and swallowing
How is diphtheria diagnosed?
Culture of throat swab; uses tellurite agar or Loeffler’s media
What is the pathophysiology of diphtheria?
Exotoxin is released, which leads to necrosis of tissue
What is the management of diphtheria?
Antitoxin (must be given quickly, won’t work once the exotoxin is bound with tissues), and antibiotics
What antibiotics are given in diphtheria?
Erythromycin or IM penicillin
What is the cause of tuberculosis?
Mycobacterium tuberculosis
What is the pathophysiology of tuberculosis?
Primary infection of the lungs occurs. A small lung lesion (Ghon focus) develops. This is composed of tubercle-laden macrophages. Leads to granuloma formation, with necrosis in the centre. This either heals by fibrosis via a type 4 hypersensitivity reaction, though in immunocompromised disseminated disease may occur
What is the gold standard diagnosis method of TB?
Sputum culture
How is latent tuberculosis diagnosed?
Mantoux test (inject tuberculin in the intradermal space in forearm). If >5mm= positive result
What is a ghon focus in tuberculosis?
pulmonary lesion (disease laden macrophages) with ipsilateral hilar lymph node involvement
What drugs are given in TB, and what are the side effects?
Rifampicin, isoniazid, pyrazinamide, ethambutol
What are side effects of TB drugs?
R: orange/red urine
I: burning sensation in feet
P: joint pains
E: visual impairment
4 for 2 months, 2 for 4 months
What is the presentation of TB in children?
chronic cough, haemoptysis, lethargy, fever or night sweats, weight loss, lymphadenopathy, erythema nodosum (tender, red nodules on the shins cause by inflammation of subcutaneous fat), spinal pain
What is erythema nodosum?
Tender, red nodules on the shins caused by inflammation of subcutaneous fat
What does acid fast mean in the context of TB?
M.tuberculosis has a waxy coating, makes gram staining ineffective. Resistant to acids used to stain; making them acid fast
What stain is used to diagnose TB?
Zeihl-Neelsen stain
What is seen on a chest xray in a patient with disseminated miliary tuberculosis?
Millet seeds (small nodules throughout the lungs)
What is usually prescribed alongside Isoniazid and why?
Pyridoxine (vitamin B6): reduces peripheral neuropathy
What are the most common causes of bacterial meningitis in children and adults?
Neisseria meningitidis (meningococcus) and streptococcus pneumoniae (pneumococcus)
What is the commonest cause of bacterial meningitis in neonates?
Group B strep
What is meningococcal septicaemia referring to?
A meningococcus bacterial infection in the bloodstream
What causes the classic non-blanching rash in meningococcal septicaemia?
Infection has caused DIC and subcutaenous haemorrhages
Do all forms of meningococcal septicaemia present with a non-blanching rash?
No, classically only meningococcal septicaemia
What is the classic presentation of meningitis?
Fever, neck stiffness, vomiting, headache, photophobia, altered consciousness, seizures
What are signs of bacterial meningitis that might present in neonates/babies?
Generally very non specific. Hypotonia, poor feeding, lethargy, hypothermia, bulging fontanelle
When do NICE recommend lumbar puncture as part of investigations?
All children:
- under 1 month presenting with fever
- 1 to 3 months with fever and unwell
- under 1 year with unexplained fever and other signs of illness
What are the special tests that can look for meningeal irritation?
Kernig’s test, Brudzinski’s test
What is kernig’s test?
Lie the patient on their back, flex one hip and knee to 90 degrees, then slowly straighten the knee whilst keeping the hip flexed at 90 degrees. Creates slight stretch in the meninges; if meningitis, will produce spinal pain
What is Brudzinski’s test?
Lie patient flat on back, gently lift head and neck off bed and flex chin to their chest. Will cause patient to involuntarily flex their hips and knees
How should suspected meningococcal meningitis be treated in the community?
Stat IM BenPen
How should meningococcal meningitis be dx?
meningococcal PCR (tests directly for meningococcal DNA)
When is vancomycin added to meningitis treatment regime?
Risk of penicillin resistant pneumococcal infection, for example recent foreign travel or prolonged antibiotic exposure
Why are steroids used in meningitis treatment? What is the regime, and for what age?
Reduce frequency and severity of hearing loss and neurological damage. Dexamethasone given 4 times daily for 4 days to children over 3 months
What is the treatment for meningitis (under 3 months vs over 3 months)?
- under 3 months: cefotaxime plus amoxicillin
- over 3 months: ceftriaxone
What is the prophylaxis treatment for meningitis?
Single dose of ciprofloxacin
Is viral or bacterial meningitis worse?
Viral tends to be milder and require only supportive treatment
What are the most common causes of viral meningitis?
Herpes simplex, enterovirus, varicella zoster virus
What are potential complications of meningitis?
- sensorineural hearing loss
- seizures and epilepsy
- memory loss
- cerebral palsy with focal neurological deficits
What is found in a CSF sample with bacterial infection present?
Cloudy, high protein, low glucose, high neutrophils
What is found in a CSF sample with viral infection present?
Clear, normal protein and glucose, high lymphocytes
Why is amoxicillin given to neonates with meningitis?
To cover listeria infection
What is Waterhouse-Friderichsen syndrome, and how does it link to meningococcal meningitis?
Patients with meningococcal meningitis are at risk of Waterhouse-Friderichsen syndrome (adrenal insufficiency secondary to adrenal haemorrhage).
What are contraindications to performing a lumbar punctures?
- focal neurological signs
- papilloedema
- significant bulging of the fontanelle
- DIC
- signs of cerebral herniation
What is the treatment of meningitis that may be viral, before cultures return?
If suspected viral, still start on broad spectrum IV abx until results back
What is encephalitis?
Inflammation of the brain
What is the most common cause of encephalitis?
Infection with a virus; most commonly herpes simplex virus. Also varicella zoster virus.
Which type of herpes simplex virus most commonly causes encephalitis (children vs neonates)
Children: HSV-1 from cold sores
Neonates: HSV-2 from genital herpes
What is the presentation of encephalitis?
Altered consciousness, altered cognition, unusual behaviour, acute onset focal neurological symptoms, acute onset focal seizures, fever
What is the diagnosis of encephalitis?
- LP: CSF for viral PCR
- CT scan (if LP C/I)
- MRO scan
- EEG
- HIV testing
What is the management of encephalitis?
Antiviral medications: aciclovir (used empirically)
What investigation is done before stopping antiviral medication?
Repeat lumbar puncture to ensure successful treatment
What parts of the brain are typically affected in encephalitis?
Temporal and inferior frontal lobes
What is impetigo?
Superficial bacterial skin infection
What typically causes impetigo?
S.aureus (though rarely also S.pyogenes)
What is the characteristic presentation of a staphylococcus skin infection?
A golden crust
What is the difference between bullous and non bullous impetigo? Which is more common?
Non-bullous; blisters are not present. Most common
Bullous: fluid filled blisters present.
Why does impetigo occur?
When bacteria enters via a break in the skin. Can occur also related to eczema or dermatitis
Where does non-bullous impetigo typically occur?
Around the mouth or nose
What is the treatment for localised non-bullous impetigo?
Hydrogen peroxide 1% cream is first line. Then topical fusidic acid
What is the treatment for wide spread or severe impetigo?
Oral flucloxacillin
What causes the vesicles in bullous impetigo?
Due to s.aureus bacteria, which produce epidermolytic toxins that break down the proteins holding skin cells together. Causes 1-2cm fluid filled vesicles.
Is there systemic symptoms in impetigo?
Non-bullous: no
Bullous: yes (feverish and generally unwell)
What is severe infections with widespread bullous impetigo lesions called?
Staphylococcus scalded skin syndrome
How is bullous impetigo diagnosed?
Swabs of the vesicles
What is the treatment of bullous impetigo?
Oral or IV flucloxacillin
What are possible complications of impetigo?
Cellulitis, sepsis, scarring, Staphylococcus scalded skin syndrome
How is impetigo spread?
Spread is by direct contact with discharges from the scabs of an infected person. The bacteria invade the skin through minor abrasions and then spread to other sites by scratching. Infection is spread mainly by the hands, but indirect spread via toys, clothing, equipment and the environment may occur.
Whats the incubation period for impetigo?
Between 4 and 10 days
What is toxic shock syndrome?
Acute disease that is characterised by fever, diarrhoea, nausea, diffuse erythema, shock that is associated with the presence of a bacterium (S. aureus)
What causes toxic shock syndrome?
Staphylococal exotoxins, the TSST-1 superantigen toxin
What are the symptoms of toxic shock syndrome?
Systemic illness with high fever, GI (vomiting, watery diarrhoea), shock and hypotension, severe myalgia, rash
What rash is seen in toxic shock syndrome?
Red mucous membranes and diffuse macular rash; 10 days after infection desquamation of the palms, soles, fingers and toes
What is the diagnostic criteria for staphylococcal TSS?
Temperature over 39 degrees celsius, low systolic blood pressure, rash, involvement of 3 or more organ systems
What are the causative organisms of toxic shock syndrome?
S.aureus or group A streptococcus
What is the treatment for toxic shock syndrome?
IV fluids and resuscitation, abx against staphylococci and streptococci, IV immunoglobulin
What is staphyloccal scalded skin syndrome?
Skin breaks down due to staphyloccus aureus bacteria producing epidermolytic toxins that break down the proteins that hold the skin together
What is the difference in presentation between toxic shock syndrome and scalded skin syndrome?
The latter has less specific systemic manifestations: though still fever, irritability, lethargy. SSS has no involvement from other organ systems
What is nikolsky sign?
Where gentle rubbing of the skin causes it to peel away. No scarring
When is nikolsky’s sign present?
Stevens-Johnson syndrome and scalded skin syndrome
How does scalded syndrome present?
Generalised patches of erythema, skin looks thin and wrinkled. Followed by formation of bullae, which burst and leave sore erythematous skin below
What is the treatment of scalded skin syndrome?
IV flucloxacillin
What bacteria usually keeps candida albicans under control?
Lactobacillus
What are the predisposing factor to thrush?
Moist body folds, treatment with broad spectrum abx, immunosuppression, diabetes
What is the treatment of thrush?
Fluclonazole
What are possible causses of nappy rash?
Irritant dermatitis, candida dermatitis, seborrhoeic dermatitis, psoriasis, atopic eczema
What is the most common cause of nappy rash? Why does it occur?
Irritant dermatitis due to the irritant effect of urinary ammonia and faeces. Creases characteristically spared
What is signs that would indicate candidal infection rather than just simple nappy rash?
Rash extending into skin folds, larger red macules, well demarcated scaly border, satelline lesions (small patches near main leison), circular pattern to the rash spreading outwards
Where does eczema typically present?
Flexor surfaces, with dry, red, itchy sore patchs of skin
What type of virus is HIV?
RNA retrovirus (mostly HIV-1)
What is the basic pathophysiology of HIV?
The virus enters and destroys CD4 T helper cells. Initial seroconversion flu like illness occurs within a few weeks of infection. Then asymptomatic until it progreses and the patient becomes immunocompromised
How is HIV transmitted?
Sexual activity, mother to child (pregnancy, birth, breastfeeding –> vertical transmission), mucous membrane/blood/open wound exposure
How should HIV transmission during birth be prevented?
Via determining viral loads.
<50 copies/ml (normal vaginal delivery), C-section if >50 copies and in all >400 copies/ml. IV zidovudine with c-section if unknown viral load or >10,0000 copies/ml
What prophylaxis treatment can be given to prevent transmission of HIV during birth?
- low risk: zidovudine for 4 weeks
- high risk: zidovudine, lamivudine, nevirapine for 4 weeks
Is breastfeeding ever recommended for mothers with HIV?
No, as it can also be transmitted, even if the viral load is undetectable
When might a child get a false positive result for HIV?
Positive results may be due to maternal antibodies in children aged under 18 months. This does not necessarily mean they are HIV positive
What are the two options for testing HIV?
- HIV antibody screen (can take 3 months to become positive)
- HIV viral load
When are babies to HIV positive parents tested for HIV?
- HIV viral load test at 3 months (if negative, means they have not contracted HIV during birth)
- HIV antibody test at 24 months
What are the key principles of HIV treatment?
- antiretroviral therapy to suppress HIV infection
- normal childhood vaccines
- prophylactic co-trimoxazole for children with low CD4 to protect against pneumocystis jirovecii pneumonia (PCP)
- treatment of opportunistic
What are later features of paediatric HIV infection?
GI: chronic diarrhoea, failure to thrive, CNS: delayed development and cerebral palsy, recurrent bacterial and viral infection, opportunistic infections, respiratory distress: cough, hypoxaemia, bilateral nodule infiltrates on CXR
What opportunistic infections may occur in HIV?
pneumocystis carinii, Candida, herpes virus, varicella, and atypical mycobacteria
What are AIDS defining infections in a HIV- positive child
Lymphocytic interstitial pneumonitis, Pneumocystis carinii (PCP) infection, and Candida oesophagitis
When is vaginal delivery recommended in HIV positive women?
Vaginal delivery is recommended if viral load is less than 50 copies/ml at 36 weeks, otherwise caesarian section is recommended
What is a possible long term complication of polio?
Irreversible paralysis
What are the two broad causes of immunodeficiency?
Primary and secondary
What is the secondary causes of immunosuppresion in children?
Cancer, immunosuppressive agents, HIV infection, splenectomy, nephrotic syndrome
What is SCID?
Severe combined immunodeficiency: presents in first few months, failure. to thrive, persistent infection to viruses and fungi
What is infectious mononucleosis?
Condition due to infection with the estein barr virus
How is infectious mononucleosis spread?
Via kissing, sharing cups, toothbrushes, and other equipment that transmits saliva
When is infectious mononucleosis infectious?
Secreted in the salvia of infected individuals and can be infectious several weeks before illness begins and intermittently for the rest of the patient’s life
What is a classical response of mono to abx?
Mononucleosis causes an intensely itchy maculopapular rash in response to amoxicillin or cefalosporins
What are the features of mono?
Fever, sore throat, fatigue, lymphadenopathy, tonsillar enlargement, splenomegaly
What antibodies are looked for to diagnose mono?
Heterophile antibodies (can take 6 weeks. tobe produced)
How are heterophile antibodies diagnosed?
Monospot test and Paul-Bunnell test
What is the monospot test?
This introduces the patient’s blood to red blood cells from horses. Heterophile antibodies (if present) will react to the horse red blood cells and give a positive result. (for EBV)
How long does EBV usually last?
2-3 weeks, but patients can be fatigued for months
Why should patients who had EBV avoid alcohol and contact sports?
Patients are advised to avoid alcohol, as EBV impacts the ability of the liver to process the alcohol. Patients are advised to avoid contact sports due to the risk of splenic rupture.
What is the classic triad of EBV?
Sore throad, pyrexia and lymphadenopathy
What is palatal petechiae and when is it seen?
Bruising on roof of mouth, can be seen following mono
What is the treatment for moni?
Supportive
What are possible complications of EBV?
Splenic rupture, glomerulonephritis, haemolytic anaemia, chronic fatigue
What is the time limit for neonatal sepsis?
First 28 days of life
What divides neonatal sepsis into early onset and late onset?
Early: within 72 hours, Late: between 7-28 days
What is an independent risk factor for Group B strep sepsis?
Black race
What are the most common causes of neonatal sepsis?
Group B strep, and E.coli
What is the commonest cause of early onset sepsis?
GBS infection
What are the risk factors for neonatal sepsis?
Mother who has a previous baby with GBS infection, current GBS colonisation from prenatal screening, current bacteruria, intrapartum temperature more than 38 degrees celsius, membrane rupture more than 18 hours, current infection in pregnancy, PREMATURITY, LOW BIRTH WEIGHT
What is the most common presentation of neonatal sepsis?
Respiratory distress and grunting. Can also be a vague presentation such as lethargy, temperature, poor feeding
What is the first line treatment for suspected or confirmed neonatal sepsis?
IV BenPen with gentamycin
How is neonatal sepsis diagnosed?
Blood culture (x2 in most cases)
What is the basic pathophysiology in sepsis?
- causative pathogens recognised by macrophages, lymphocytes, mast cells
- cytokines released: activates immune system and release of chemicals such as nitrous oxide that causes vasodilation. Inflammation throughout body
- cytokines lead to endothelial lining of blood vessels to become permeable; fluid leaks to extracellular space, leads to oedema, and reduction in intravascular volume
- oedema around blood vessels creates a space between blood and tissues; less oxygen reaches tissues
- hypoperfusion; anaerobic respiration; rise in blood lactate
- activation of coagulation system: DIC
What is septic shock?
Sepsis has lead to cardiovascular dysfunction; arterial blood pressure falls, organ hypo-perfusion. Lactate rises; anaerobic respiration.
How should septic shock be treated?
Aggressively with IV fluids to improve blood pressure and tissue perfusion. Also inotropes if no improvement; stimulate cardiovascular system, improve blood pressure
What is a sign for all infants under 3 months should be treated for sepsis?
Temperature of 38 degrees celsius or above
What are causes of red urine in children (not haematuria)
Foods (eg, beetroot), drugs (eg, ridampicin), urate crystals, external source (eg, menstrual blood losses)
What are the possible causes of haematuria in children?
UTI, glomerular (eg, post infectious glomerulonephritis, henoch-schonlein purpura nephropathy, SLE), urinary tract stones, trauma, renal tract pathology (renal tract tumour, polycystic kidney disease), vascular, vigorous exercise, sexual intercourse
What are the possible causes of polyuria?
Renal disorders (CKD, post obstructive uropathy, nephrogenic diabetes insipidus), diabetes mellitus, cranial diabetes insipidus, hypoadrenalism
What is acute pyelonephritis?
When a UTI affects the tissue of kidney: can cause scarring and a reduction in kidney function
What are the symptoms of UTI in younger children?
Fever (may be the only symptom, especially in young children). Non specific symptoms: lethargy, irritability, vomiting, poor feeding, urinary frequency
What are the signs of UTI in older children?
Fever, abdominal pain (suprapubic), vomiting, dysuria, urinary frequency, incontinence
When is acute pyelonephritis diagnosed instead of UTI?
When there is a temperature greater than 38 degrees celsius, loin pain or tenderness
How are UTIs diagnosed?
clean catch urine collection
What are the NICE guidelines for checking a urine sample in a child?
Signs or symptoms suggestive of UTI, unexplained fever of 38 degrees or higher, site of infection appearing unclear
What is the management of a UTI in a child younger than 3 months?
Referral to a paediatrician immediately
What is the management of a UTI in children aged more than 3 months old?
Consider admission to hospital. If no admission, oral abx such as cephalosporin or co-amoxiclav
What is the management of lower UTI in children more than 3 months?
3 days oral abx, usually trimethoprim, nitrofurantoin, cephalosporin or amoxicillin
What are the common causative organisms of UTIs in children?
E.coli (80%), proteus, pseudomonas
What are predisposing factors for UTIs in children?
Incomplete bladder emptying (infrequent voiding, hurried micturition, obstruction by full rectum due to constipation, neuropathic bladder), vesicoureteric reflux, poor hygiene
What is likely food in a urine dipstick for a diagnosis of a UTI?
Nitrites (nitrates broken down by gram negative bacteria), leukocytes
Should a child be treated for a UTI if only leukocytes are found in urine?
If only leukocytes are present the patient should not be treated as a UTI unless there is clinical evidence they have one.
When should a child have a ultrasound following a UTI?
All children under 6 months within 6 weeks, or during illness if recurrent UTIs or atypical bacteria. All children with recurrent UTIs should have US within 6 weeks, and children with atypical UTI should have US during illness
What is DMSA and when should it be done?
Scan involves injecting radioactive material and using a gamma camera to see how well the material is taken up by the kdineys. Used around 6 months after recurrent or atypical UTI to check for scarring
How is vesico-ureteric reflux diagnosed?
Micturating cystourethrogram (MCUG)
What is vesico-ureteric reflux, and what does it predispose to?
Vesico-ureteric reflux (VUR) is where urine has a tendency to flow from the bladder back into the ureters. This predisposes patients to developing upper urinary tract infections and subsequent renal scarring.
What is the management of vesico-ureteric reflux?
Avoid constipation, avoid excessively full bladder, prophylactic antibiotics, surgical input
What additional investigation to US can be done for children under 6 months old for recurrent or atypical UTI?
MCUG; can help diagnose vesico-ureteric reflux. Involves catheterising the child, injecting contract to the bladder, and using xray to determine whether contrast is refluxing into ureters
When is MCUG indicated?
Children under 6 months with atypical or recurrent UTIs, FHx of vesico-ureteric reflux, dilatation of the ureter on ultrasound, or poor urinary flow
What is the difference in upper or lower UTI symptoms?
Upper: fever, vomiting, loin pain
Lower: dysuria, frequency, mild abdominal pain, enuresis
What is UTI prophylaxis?
Trimethoprim or nitrofurantoin
When do the majority of children achieve day and night time continence
3 or 4 years old
What is the management of children under 5 years old who have nocturnal eneruesis?
Management, advice ,reassurance
What is the difference between primary and secondary nocturnal enuresis?
Primary: child has never achieved continence
Secondary: child has been dry for at least 6 months
What is the first line treatment of nocturnal enuresis?
Enuresis alarm: sensor pads that sense wetness
What is the second line method for nocturnal anuresis, that can also be used for short term control?
Desmopressin
Which sort of enuresis is more likely to have an organic cause?
Secondary (renal tract, neurological, endocrine, behavioural issues, abuse). Primary u
What can cause primary enuresis?
Majority of cases have no underlying organic cause and is thought to be due to delayed maturation of bladder control mechanisms
At what age is desmopressin typically considered for treatment of enuresis?
> 7 years
What are key pathologies to consider in secondary enuresis?
Urinary outflow obstruction in boys, chronic constipation, neurodevelopmental problems, psychological problems
What complication does vesicoureteric reflux predispose to?
UTI. MAny of these children then go on to experience renal scarring
What is the pathophysiology of vesico-ureteric reflux?
The ureters are displaced laterally, entering the bladder in a more perpendicular fashion. Shortened intramural course, and the vesicoureteric junction therefore can’t function adequately
What are the possible presentations of vesico-ureteric reflux?
Hydronephrosis on ultrasound, recurrent childhood UTIs, reflux nephropathy (chronic pyelonephritis secondary to VUR)
What is reflux nephropathy?
Term used to describe chronic pyelonephritis secondary to vesico-ureteric reflux. Commonest cause of chronic pyelonephritis
How can vesico-ureteric reflux lead to hypertension?
Vesico-ureteric reflux is the commonest cause of chronic pyelonephritis: reflux nephropathy. The renal scar that is formed may produce increased quantities of renin causing hypertension
How is VUR diagnosed?
Micturating cystourethrogram. DMSA scan can diagnose secondary renal scarring
What is AKI?
Reduction in renal function following an insult to the kidneys
What is the divisions of causes of AKI?
Pre-renal, intrinsic, extra-renal
What are pre renal causes of AKI?
Hypovolaemia (DKA, nephrotic syndrome, gastroenteritis, haemorrhage), peripheral vasodilation (eg, sepsis), impaired cardiac output, drugs
What are examples of causes of renal AKI?
Glomurolonephritis, acute tubular necrosis, acute interstitial nephritis, rhabdomyolysis, tumour lysis syndrome
What are examples of post-renal AKI causes?
Obstruction, post-urethral valves, neurogenic bladder, tumours
What is the definition of hyperkalaemia?
> 6.5 mmol/L
Why might a history of sore throat/rash precede AKI?
Can lead to streptococcal glomerulonephritis
When should CKD be suspected in children?
Failure to thrive, polyuria and polydipsia, lethargy, poor school concentration, other abnormalities such as rickets
What are potential causes of paediatric CKD?
Congenital abnormalities, hereditary (PKD), glomerulopathies, multisystem disorders (SLE, HSP), Wilms tumour, renal vascular disease
What is the triad of nephrotic syndrome in children?
Proteinuria, hypoalbuminaemia, oedema
What is the peak incidence of nephrotic syndrome in children?
Between 2 and 5 years of age
What is the most common cause of nephrotic syndrome in children?
Minimal change glomerulonephritis
How is minimal change glomerulonephritis treated? Is there a good prognosis?
High dose oral steroids, carries very good prognosis
Other than the classical triad, what re other symptoms of nephrotic syndrome in children?
Hyperlipidaemia, hypercoaguable state, predisposition to infection
What is nephrotic syndrome?
Occurs when the basement membrane in the glomerulus becomes highly permeable to protein, allowing proteins to leak from the blood into the urine
What is minimal change disease?
Nephrotic syndrome that occurs in isolation, without any clear underlying condition or pathology.
Other than minimal change disease, what can cause nephrotic syndrome?
Intrinsic kidney disease, secondary to underlying systemic illness (HSP, diabetes, infection)
What causes minimal change disease?
Generally unclear
What is the treatment of minimal change disease?
Corticosteroids (eg, prednisolone).
What is seen on renal biopsy of minimal change disease?
Normal glomeruli
What type of proteinuria occurs in minimal change disease?
Highly selective proteinuria; only intermediate sized proteins such as albumin and transferrin leak through the glomerulus
What is seen in urinalysis in minimal change disease?
Small molecular weight proteins and hyaline casts
What is the treatment of nephrotic syndrome in children?
High dose steroids, low salt diet, diuretics if oedema, albumin infusions
What is the treatment of steroid resistant nephrotic syndrome?
ACE inhibitors and immunosuppressants such as cyclosporine
What is the pathophysiology of minimal change disease syndrome?
T-cell and cytokine-mediated damage to the glomerular basement membrane
Why might thrombosis occur after nephrotic syndrome?
Thrombosis can occur because proteins that normally prevent blood clotting are lost in the kidneys, and because the liver responds to the low albumin by producing pro-thrombotic proteins
What might infection occur in kidneys after nephrotic syndrome?
Infection occurs as the kidneys leak immunoglobulins, weakening the capacity of the immune system to respond. This is exacerbated by treatment with medications that suppress the immune system, such as steroids
What is the triad of haemolytic uraemic syndrome?
Normocytic anaemia, thrombocytopenia, AKI
What is the most common cause of haemolytic uraemic syndrome?
Shiga-toxin producing E.coli (90% cases)
What causes haemolytic uraemic syndrome?
Complement dysregulation. Thrombosis in small blood vessels throughout the body, typically after a episode of gastroenteritis
What medications increase the risk of haemolytic uraemic syndrome, if used in an episode of gastroenteritis?
Antibiotics and anti-motility medication (such as loperamide) if E.coli caused
What causes the classical triad of symptoms in haemolytic uraemic syndrome?
Thrombocytopenia if formation of blood clots has consumed platelets. AKI due to the blood flow through the kidney being affected by thrombi and damaged RBC. Microangiopathic haemolytic anaemia involves the destruction of RBCs due to pathology in the small vessels; the thrombi partially obstruct the vessels and cause passing RBCs to rupture
What is the presentation of haemolytic uraemic syndrome?
Diarrhoea is the first symptom, which then will turn bloody in 3 days. A week after: fever, abdominal pain, lethargy, pallor, oliguria, haematuria, HTN, bruising, jaundice (Due to haemolysis), confusion
What is the dx of haemolytic uraemic syndrome?
Stool culture; PCR. forshiga toxin
What is seen on a full blood count in haemolytic uraemic syndrome?
- anaemia: microangiopathic anaemic with low haemoglobin
- htrombocytopenia
- fragmented blood film; helmet cells
What is a classic finding on blood film in disseminated intravascular coagulation?
Helmet cells and shistocytes
What is the treatment for haemolytic uraemic syndrome?
- supportive
- no role for abx
- blood transfusion
- IV fluids for hypovolaemia
- treat possible HTN
What is the commonest cause of AKI in children?
Haemolytic uraemic syndrome
Where is swelling often seen in nephrotic syndrome in children?
Facial/periorbital
What cases the majority of cases of congenital nephrotic syndrome?
Genetic defects in the components of the glomerular filtration barrier
What is nephritis?
Inflammation within the nephrons of the kidneys. This causes reduction in kidney function, haematuria, and proteinuria (but less than in nephrotic syndrome)
What are the two most common causes of nephritis in children?
Post streptococcal glomerulonephritis and IgA nephropathy (Berger’s disease)
What precedes post-streptococcal glomerulonephritis?
Beta-haemolytic streptococcus infection, such as tonsillitis caused by streptococcus pyogenes
What is the pathophysiology of post-streptococcal glomerulonephritis?
Immune complexes of streptococcal antigens, antibodies, complement proteins get stuck in the glomeruli of the kidney and cause inflammation. Reduces kidney function and leads to AKI
When should a dx of post-streptococcal glomerulonephritis be considered?
Evidence of recent tonsillitis caused by streptococcus: positive throat swab and anti-streptolysin antibody titres on a blood test
What is the treatment of post streptococcal glomerulonephritis?
Supportive; if progressive worsening of renal function, may need tx with antihypertensive medications and diuretics
How long after streptococcal infection does post streptococcal glomerulonephritis occur?
1-2 weeks
How long after a URTI does IgA nephropathy develop?
1-2 days
What are the general presentation of post-streptococcal glomerulonephritis?
Visible haematuria, proteinuria/oedema, hypertension, oliguria
How is a recent streptococcal infection confirmed in post-streptococcal glomerulonephritis?
Raised anti-streptolysin O titre
What is IgA nephropathy?
Immune complex deposition in the glomerulus. Leads to inflammation. Considerable overlap with HSP
What is the presentation of IgA nephropathy?
Macroscopic haematuria in young people following URTI
What is the presentation of IgA nephropathy?
Young male, recurrent episodes of haematuria, recent URTI.
What additional features are seen in HSP that won’t be present in IgA nephropathy?
Palpable purpuric rash over buttocks and extensor surfaces of arms and legs, abdominal pain, polyarthritis, renal involvement
What is the pathophysiology of Henoch Schonlein Purpura?
IgA mediated small vessel vasculitis. Overlap with IgA nephropathy
What should be monitored in children with HSP?
Blood pressure and urinalysis to monitor any progressive renal involvement
What is the typical age of a patient with HSP? How does this differ in IgA nephropathy?
Under 10. In IgA nephropathy the patient is older
Why does purpura occur in henoch shconlein purpura?
Due to inflammation and leaking blood from small blood vessels under the skin
How often is there a rash in HSP?
100% of the time
Where does arthralgia typically occur in HSP?
Knees and ankles
What is the difference in presentation between HSP and ITP?
Both: purpuric rash. HSP: abdominal and joint pain. ITP: otherwise well
How can haemolytic uraemic syndrome and HSP be differentiated?
HUS: more likely to have bloody diarrhoea
What happens if HSP affects the kidneys?
Leads to IgA nephritis. Can lead to microscopic or macroscopic haematuria and proteinuria
What are the differentials for a non blanching purpuric rash?
HSP, ITP, HUS, leukaemia, meningococcal septicaemia
What are the investigations for HSP?
FBC, renal function, dipstick, urinalysis, and full renal investigation with biopsy if evidence of renal involvement (crescentic IgA glomerulonephritis)
What is the treatment of HSP?
Largely supportive. Debatable use of steroids: considered if severe GI pain or renal involvement
What is Alport’s syndrome?
Genetic condition that leads to abnormal glomerular basement membrane (due to type IV collagen miscoding)
Why might a renal transplant fail in a patient with Alport’s syndrome?
Due to anti glomerular basement membrane antibodies; leads to Goodpasture syndrome picture
Why does do proteins get leaked in nephrotic syndrome?
Nephrotic syndrome occurs when the basement membrane in the glomerulus becomes highly permeable, resulting in significant proteinuria
What are the typical features of Alport’s syndrome?
Renal failure, bilateral sensorineural deafness, ocular abnormalities, nephritis leading to haematuria
What is the diagnosis for Alport’s disease?
Genetic testing, renal biopsy (basket weave appearance)
What is lupus?
Inflammatory autoimmune connective tissue disorder
What is the typical disease course in SLE?
Relapsing remitting
What is the pathophysiology of SLE?
Autoantibodies produced (anti-nuclear antibodies), which are against proteins in the cell nucleus. Generate a chronic inflammatory response. Complexes may be formed
What populations is SLE more severe in?
Afro-caribbean, hispanic, far eastern women
What is the presentation of SLE?
Fatigue, weight loss, arthralgia, non-erosive arthritis, fever, photosensitive malar rash, lymphadenopathy, splenomegaly, shortness of breath, pleuritic chest pain, mouth ulcers, hair loss, raynaud’s phenomenon, oedema (due to nephritis)
What inflammatory marker is best for monitoring lupus flares?
ESR: CRP can be normal in active disease. Raised CRP more likely indicates underlying infection
What type of hypersensitivity reaction is lupus?
Type 3
How does lupus lead to renal disease? How should this be monitored?
Lupus nephritis is a severe manifestation of systemic lupus erythematosus (SLE) that can result in end-stage renal disease. SLE patients should be monitored by performing urinalysis at regular check-up appointments to rule out proteinuria.
What is typically seen on renal biopsy in a patient with renal SLE?
Diffuse proliferative glomerulonephritis: wire loop appearance of glomeruli
Are complement levels high or low in active disease in SLE?
Low: formation of complexes leads to consumption of complement
What antibody is nearly always positive in lupus?
Antinuclear antibody (low specificity: raised in other diseases)
What is a highly specific test for SLE?
anti double stranded DNA. Also anti-Smith (but less sensitive)
Why might SLE patients have na increased risk of venous thromboembolism?
Antiphospholipid antibodies and antiphospholipid syndrome can occur secondary to SLE in up to 40% of patients.
What is the treatment in SLE?
NSAIDs, sun cream, hydroxychloroquine, also biological therapies +DMARDs in more severe disease
What are the general presentation of SLE?
Fatigue, fever, mouth ulcers, lymphadenopathy
What is hypospadias?
Condition affecting males where the urethral meatus is displaced to the ventral side of the penis towards the scrotum
What is epispadias?
Where the meatus is displaced to the dorsal side of the penis
What conditions may be associated with chordee? What is chordee?
Chordee: head of the penis bends downwards. Can be associated with hypospadias and epispadias
What conditions may be associated with hypospadias?
Cryptochidism (when the testes fail to descend), chordee, inguinal hernia
When is hypospadias surgery typically performed?
Around 12 months
Why should circumcision not occur before hypospadias surgery?
The foreskin may be used in corrective procedure
What is posterior urethral valve and what can it cause?
A posterior urethral valve is where there is tissue at the proximal end of the urethra (closest to the bladder) that causes obstruction of urine output. It occurs in newborn boys. The obstruction to the outflow of urine creates a back pressure into the bladder, ureters and up to the kidneys, causing hydronephrosis. Stops bladder from fully emptying, which can increase risk of UTIs
How does posterior urethral valve present?
Difficulty urinating, weak stream, chronic urinary retention, palpable bladder, reucrrent UTIs, impaired kidney function
What is hydronephrosis?
When there is a build up of fluid inside the kidneys, and over time they become stretched
How can a posterior urethral valve lead to underdeveloped fetal lungs?
Urine outflow is obstructed in the developing fetus, which results in bilateral hydronephrosis and olgiohydramnios (low amniotic fluid). Oligohydramnios leads to underdeveloped fetal lungs (pulmonary hypoplasia)
How can olgiohydramnios lead to lung hypoplasia in fetuses?
During lung development, the main physical force experienced by the lungs is stretching induced by breathing movements and the lung fluid in airspaces. Oligohydramnios reduces the intrathoracic cavity space, thus disrupting fetal lung growth and leading to pulmonary hypoplasia
How can posterior urethral valve be diagnosed?
May be identified on antenatal scans by olgiohydramnios and hydronephrosis. Or, can present after birth with young boys presenting with UTIs; MCUG, abdominal ultrasound, cytoscopy to view extra tissue
How is posterior urethral valve managed?
Definitive management is ablation of removal of extra tissue, usually during cystoscopy
What can cause renal cysts?
Multicystic dysplastic kidneys, polycystic kidneys
What can cause unilateral hydronephrosis?
Vesicoureteric junction obstruction, vesicoureteric reflux
What can cause bilateral hydronephrosis?
Bladder outlet obstruction
What is a hydrocele?
Collection of fluid in the tunica vaginalis that surrounds the testes
What is a communicating hydrocele?
Where the tunica vaginalis around the testicle is connected within the peritoneal cavity via the processus vaginalis; allows movement of fluid, which leads to fluctuation of size
What is seen on examination of a hydrocele?
Soft, smooth, non tender around the testes. Swelling is in front of and below the testicle. Will transilluminate with light
What are key differentials for scrotal and inguinal swelling in a neonate?
Hydrocele, partially descended testes, inguinal hernia, testicular torsion, haematoma
How are hydroceles treated?
Simple: usually resolve after around 2 years, can reassure parents. Communicating: will require surgical operation to remove connection (processus vaginalis)
What are potential complications of undescended testis?
Infertility, torsion, testicular cancer, psychological
What is the management of unilateral undescended testis?
Referral after 3 months if it has not resolved, which baby ideally seeing urological surgeon before 6 months of age
What is potter syndrome?
Lack of amniotic fluid, which leads to dysmorphic features such as underdeveloped ear cartilage, low set ears, flat nasal bridge, abnormalities of the skeleton.
What causes olgiohydramnios?
Reduced urine production by the fetus
What is the underlying pathology of polycystic kidney disease?
Cystic enlargement of the renal collecting ducts, olgiohydramnios, pulmonary hypoplasia and potter syndrome, congenital liver fibrosis
