Paediatrics Flashcards
Neonates.
<28 days. Preterm <37 weeks, post-term >42 weeks. Low BW <2.5kg. V low BW <1.5kg. Extremely low BW <1kg.
What factors increase the risk of newborn infection?
PROM >24 hour.
Chorioamnionitis.
Preterm labour.
What organisms are common in infection in infections of the newborn?
Group B strep.
Listeria.
Candida.
Neonatal sepsis.
Temp instability. Poor feeding. Abdo distention. Apnoea. Neutropaenia. Hypo/hyperglycaemia.
What is the management of neonatal sepsis?
Group B Strep - penicillin/amoxicillin
Ceph for gram -ve.
Neonatal rubella.
<8/40 - SNHL, congenital HD, cataracts, glaucoma.
13-16/40 - impaired hearing.
>18/40 - minimal risk.
Downs Syndrome.
Trisomy 21.
1/1000.
Symptoms:
Newborn - ++nuchal skin, hypotonia, sleepy.
Face: round face, epicanthic folds, protruding tongue, upslanting palpebral fissures, small low set ears.
Single palmar creases. Incurving 5th digit.
Complications of Downs Syndrome?
Learning difficulties. Congenital HD. Duodenal atresia. Resp infections. Visual/hearing impairment.
Patau Syndrome.
Trisomy 14. 1/14000. Symptoms: Small head and eyes. Absent corpus colloscum. Heart lesion. PCKD. Cleft lip/palate. Contractures on hand.
Edwards Syndrome.
Trisomy 18. Female>male. Rigid baby. Odd low set ears. Proptosis. Cleft lip/palate. Rocker bottom feet.
Turners Syndrome.
45X. Females only. Short stature, webbed neck. Wide carrying angle. Congenital HD. Ovarian dysenesis - infertility
Klinefelters Syndrome.
47XXY.
Males only.
Infertility, small testes and hypogonadism, tall stature.
Microdeletion syndromes.
Cri du chat - mewing cry, microencephaly, wide spaced eyes.
Di George - CATCH22 (congenital, abnormal face, thymic aplasia, cleft palate, hypoCa/PTH
RDS.
Due to surfactant deficiency.
Most babies <28 weeks affected.
Mx - Antenatal corticosteroids. Exogenous surfactant via ET tube. CPAP.
PTX in Neonates.
In RDS from overextended alveoli –> intersisitum.
20% of ventilated infants.
Sx - sudden increase in oxygen requirements.
Mx - chest drain.
Preventable by ventilating with low pressures.
Temp control.
Large SA: volume ratio.
Little subcut fat.
PDA.
May need inotropic support.
Fluid restriction and indomethacin (PG synthase)
Nutrition.
High nutritional requirement for rapid growth.
Suckling reflex develops at 34 weeks.
NG tube - preferred breast milk.
Consider parental nutrition.
Supplement with phosphate, Ca and Vit D for bone mineralisation.
Anaemia of preterms.
Iron is transferred in the last trimester.
Blood loss from blood samples and inadequate erythropoetin.
Neonatal infection.
Increased risk.
Especially with group B strep/coliforms.
Neonatal intracranial lesions.
Interventricular haemorrhage - high risk if asphyxia, RDS, PTX.
Post haemorrhage hydroencephalus - sutures seperate, bulging fontanelle.
Mx - LP/ventricular tap, shunt.
Periventricular leucomalacia - due to ischaemia.
Neonates renal.
Low renal flow in the fetus.
At 28 weeks - 35% of term. Doubles in 1st 2/52.
NEC.
Necrotising enterocolitis.
Bowel wall ischaemia.
No feeding. Milk aspiration. Bile stained vomit. Distended abdomen. Blood in stool.
Can cause shock if haemorrhagic colitis.
AXR: shows distended bowel + air in bowel wall/portal tract.
Mx -stop feeding, Abx, ventilate/circulatory support, surgery if perforated.
Complications - strictures, short bowel syndrome (malabsorption).
ROP.
Retinopathy of prematurity.
Common - 50%.
Can cause retinal detachment, fibrosis, blindness in 1%.
Pathogenesis - delayed retinal vascular growth. induced hypoxia releases factors to stimulate new growth via VEGF.
Mx - cryotherapy/laser.
Metabolic issues for neonates.
Hypoglycaemia - maintain BM >2.6 to prevent neurodamage.
Hypocalcaemia.
Electrolyte imbalance - poor renal function and poor resorption.
Osteopenia of prem.
Osteopenia of prematurity.
Ca, phosphate and vitamin D.
Bronchopulmonary dysplasia.
If an infant has prolonged o2 requirements - beyond 36/40.
Lung damage from - pressure/volume trauma, o2 toxicity, infection and lung secretions.
CXR - areas of opacification and cystic change.
Wean infants onto CPAP.
Consequences of traumatic delivery.
Injury occurs if baby is: Malpositioned. Too large for the pelvic outlet. Manual manoeuvres. Forceps. Ventouse.
Soft tissue injuries during delivery.
Caput succedaneum - oedema of scalp, immediate, resolves spontaneously.
Cephal haematoma - below periosteum within the skull sutures, can contribute to jaundice.
Chignon - caput from ventouse.
Bruising - prems bruise easily.
Abrasions - from scalp electrodes.
Delivery nerve palsies.
Erbs Palsy - upper nerve root injury of C5+6. Straight arm, limp pronated hand ‘waiters tip sign’.
Lower roots injury - less often. Weak wrist and intrinsic hand muscles.
When is classed as prolonged oxygen requirements?
> 36 weeks.
Delivery fractures.
Clavicles - from a shoulder dystocia, may hear a snap or see a lump/callus.
Humerus/femur - normally mid shift, occur in breech. Heal quickly if immobilised.
Neonatal jaundice.
Yellow pigmentation of skin/white of eyes.
High level of bilirubin in plasma.
Clinically jaundiced at 80-120.
Causes of neonatal jaundice <24 hours.
Rhesus heamolytic disease.
ABO incompatibilty.
G6PD deficiency.
Causes of neonatal jaundice 24 hours to 2 weeks.
Breast milk jaundice.
Infection.
Physiological.
ALL UNCONJUGATED.
Causes of neonatal jaundice over 2 week.
UNCONGUGATED -
Breast milk jaundice, most gone by 1 month.
Infection - UTI (haemolysis).
Congenital hypothyroidism.
CONJUGATED -
Dark urine, pale stools.
Biliary atresia.
Neonatal hepatitis.
Management of neonatal jaundice.
Hydration.
Phototherapy.
Exchange transfusion.
Respiratory distress.
Head bobbing. Subcostal recession. See-saw breathing. Tracheal tug. Expired grunting.
Management of neonatal respiratory distress?
Admit to SCBU.
Monitor.
Chest xray.
Causes of neonatal respiratory distress?
Transient tachypnoea of the newborn. Meconium aspiration. Pnuemonia. PTX. Milk aspiration. Diaphragmatic hernia.
Childhood asthma.
Reversible airway obstruction with wheeze, SOB and cough.
Risk factors for childhood asthma.
Male. FHx. Low birth weight. Bottle fed. Atopy. Past bronchopulmonary dysplasia.
Presentation of childhood asthma.
Recurrent wheeze.
Cough and SOB.
Noctural cough.
Diagnosis of childhood asthma.
Hyperinflation of chest.
Generalised expiratory wheeze with prolonged exp phase.
Signs of atopy.
Eosinophilia and raise IgE.
Investigations for childhood asthma.
Skin tests.
CXR - hyperinflation.
PEFR in over 5s.
Management of childhood asthma.
High dose inhaled . B2 bronchodilator. Neb ipatropium. O2 if low sats. IV aminophlline. IV hydrocortisone.
Cystic fibrosis.
AR.
CFTR gene mutation.
Pathogenesis of CF.
Mutation in CFTR gene.
Abnormal ion transport across the epithelial cells . of the exocrine glands of the respiratory system and pancrease.
Clinical features of CF.
Malabsorption and FTT from birth.
Persistant chest infections.
Meconium ileus - obstruction, vomiting, abdo distention.
Malabsortion and steatorrhoea.
Management of CF.
Genetic counselling. Abxs. Vaccines. Good nutrition. Physio. Creon. High calorie/protein diet. - Lung transplant.
Whooping cough.
Specific form of bronchiolitis.
Bouts of coughing and vomiting.
Worse at night and after feeding.
Whoop - inspiration against a closed glottis.
Peak age 3 years.
Mx - erythromycin. live vaccine at 2 months. admit if <6/12.
What causes whooping cough?
Bordatella pertussis.
What is the management of whooping cough?
Prolonged illness.
Hernias.
Bronchiectasis.
Microhaemorrhages.
Bronchiolitis.
Winter bouts - RSV.
Cold like symptoms. Sharp, dry cough. Low fever. Tachypnoea. High pitched wheeze.
Mx - admit if feeding difficulties/supportive care
Diagnosis of bronchiolitis.
Immunofluroesce of NP aspirates.
CXR - hyperinflation of lungs.
What prophylaxis can be given to babies at risk of RSV?
Monoclonal AB.
What is given to children with pneumonia?
Erythromycin or penicillin V.
Childhood TB.
Anorexia/FTT/prolonged low fever.
Dx - screen with Heaf, diagnose with Mantoux
Mx - RIP (RP 6/12, I 2/12)
BCG given to at risk at birth.
What will be seen on CXR in TB?
Consilidation, cavities, miliary spread.
Croup.
Laryngotracheobronchtis. >95% of laryngotracheal infections. Peak age 2 years. Symptoms start and worse at night. Barking cough, inspiratory stridor. Mx - o2, oral dex and nebulises steroids and adrenaline.
Cause of croup?
Parainfluenza, RSV
What is the management of croup?
Mild - cool mist inhalation, sleeping upright, dex.
Oral dexamethasone.
Inhaled steroids and adrenaline.
Humidified oxygen.
IV fluids.
Why would patients with croup need intubated?
Subglottic narrowing.
Acute epiglottitis.
Medical emergency.
Decreased incidence due to vaccine (HiB) now caused by streps (pyogenes, pnuemo).
Ix - DO NOT EXAMINE THROAT.
Cherry red epiglottis on pharyngoscopy.
Mx - secure airway in controlled environent, blood cultures, IVABx (ceph)
Presentation of acute epiglottitis.
Sudden very sore throat
Fever
Drooling
Muffled voice
Trouble swallowing
Blue skin coloring
Stridor.
Severe - Sit leaning forward
Keep his or her mouth open with the chin thrust forward
Look distressed or anxious
What is the PEP for epiglottitis?
Rifampicin.
Symptoms of the common cold?
Clear or mucopurlent nasal discharge
Nasal blockage
Common pathogens in the common cold.
Rhinovirus, coronavirus, RSV.
Pharyngiits.
Usually due to viruses (rhino, adeno)
Inflammed throat with local lymphadenopathy.
Tonsilitis.
Intense inflammation a nd purulent exudates.
Group A strep and EBV.
Marked constitutional disturbances - headache, abdo pain.
Management of tonsilitis.
Centor criteria. 3+ - Abx. Consider in under 14s (extra point). Pen V or erythromycin NOT amoxicillin - EBV MPRash.
Centor criteria.
Absence of cough.
Fever.
Purulent exudate.
Lymphadenopathy.
Otitis externa.
Inflammation of skin of the meatus.
Psuedomonas common.
Aural toilet is key.
Otitis media.
Inflammation of middle ear.
Paracetamol and ABx (amox)
Gastroenteritis.
Infective diarrhoea and vomiting.
Bacterial causes - blood in stool.
Ix - stool culture.
What is the common cause of gastroenteritis in children?
Rotavirus 60%
Management of paediatric gastroenteritis?
Fluid replacements.
Causes of secretory diarrhoea.
Bacteria . - campylobacter, staph, ecoli.
Giardiasis.
Rotavirus.
IBD.
Paediatric constipation.
Painful passage of hard, infrequent stools.
Causes:
INFANT - organic cause.
OLDER - functional.
Investigations for constipation.
Growth. General and anorectal. Faeces palpable per abdomen. Bloods - exclude systemic cause. AXR - faecal loading +/- megacolon.
Management of constipation.
Treat underlying cause.
Increase dietary fluids and fibre.
Softner - laculose, stimulant - senna.
Evacuate overloaded rectum - enema or manual evacuation.
Maintenance - stool should be kept soft for 3-6 months. encourage daily sits on toilet.
Hirschsprungs disease.
Absence of ganglionic cells in large bowel - leads to a narrow, contracted segment from rectum proximally.
Ends in normally innervated dilated colon.
How does Hirschsprungs disease present?
Neonate with intestinal obstruction (failure to pass mueconium in first 24 hours)
Abdomen distention and bile stained vomit.
Can present in the first week with Hirshsprungs enterocolitis - C.Diff.
Older child with soiling, constipation and abdo distention.
Investigations for Hirschrprungs disease.
DRE - Narrow segment. Withdrawing finger causes a gush of fluid and flatus.
Rectal biopsy is diagnostic - absence of ganglionic cells.
What is the management of Hirschsrpungs?
Colostomy then anastamosing normal bowel to anus.
Investigations in FTT?
Serial measurements of weight. Renal and CNS USS. Skeletal survery. ECG/Echo. Endomysial + antigliadin Abs (coeliac) Sweat test.
What are some causes of FTT?
Inadequate intake. Deprivation. Small for date. Familial short stature. Mental retardation. Steroids. Endocrine. Downs syndrome.
Faecal soiling.
With or without loaded rectum.
Faeces in rectum are always an abnormal finding.
Loaded rectum soiling.
Poor coordination with anal sphincter relaxation.
Following febrile illness.
Anxieties.
Mx - as for constipation, star charts, explain how retention = continence.
Empty rectum soiling
Urgency of defecation - cant hold it in.
Neuropathic bowel.
Learning disability.
Spite parents.
Reflux.
Gastro oesophageal reflux.
Lower oesophageal pH <4% for 24 hours is normal.
Increased - functional immaturity of LOS.
Common in under 1s, spont resolves.
Sliding hernia in some cases.
Ix - pH monitoring, barium swallow, endoscopy
What conditions are concerned with severe reflux?
Cerebral palsy
Bronchopulmonary dysplasia
Following surgery for atresia
Presentation of GOR.
Regurgitation.
Distress after feeds.
Apnoea.
FTT + aneamia.
Management of GOR.
Reassurance.
Lie on front.
Milk thickeners - Carobel.
Antacids - gaviscon.
Ranitidine (h2)
Fundoplificatoin
Crohns Disease.
Abdo pain, failure to grow and diarrhoea.
Barium and colonoscopy/
UC.
Rectal bleeding, diarrhoea, colicky pain and weight loss.
Coeliac disease.
Gliadin fraction - damaging immune response in small intestine.
Lymphoctic infiltrates and villous atrophy.
Presentation of coeliac disease?
In first 2 years of life Following introduction of cereals FTT General irritability Abnormal stools Abdominal distention Buttock wasting
Pyloric stenosis.
Hypertrophy of the pylorus causing gastric outflow obstruction
2-7 weeks of age
Boys
How does pyloric stenosis present?
Projectile vomiting after feeds
Constant hunger
Hyperchloraemic alkalosis
How is pyloric stenosis diagnosed?
Visible gastric peristalsis
Pyloric ‘olive’ palpable
What is intussusception?
Invagination of proximal bowel into distal.
Commonest cause of intestinal obstruction in infants.
Presentation of intussusception.
Acute, severe colicky pain Pallor Sausage shaped mass in abdomen Passage of red current jelly stools Abdo distention and shock
Kawasaki Disease.
Type of vasculitis in children. High grade, resistant fever. Conjunctival injection. Bright red, cracked lips. Strawberry tongue. Cx lymphadenopathy. Red, peeling palms.
Mx - high dose aspirin (even despite Reyes syndrome), IvIg, echo
Complications - coronary artery aneurysm
Management of Kawasaki disease.
High dose aspirin.
IvIg.
What causes TTN?
Resorption of fluid in the lungs.
Common in C-section - fluid not ‘pushed’ out of the lungs during birth
O2 may be required
Settles in 1-2 days
Omphalocele vs Gastroschisis.
Gastroschisis - associated with socioeconomic depravation (maternal age <20, maternal alcohol/tobacco use)
Gastroschisis refers to a defect lateral to the umbilicus
Omphalocele refers to a defect in the umbilicus itself.
Gastroschisis describes a congenital defect in the anterior abdominal wall just lateral to the umbilical cord
In exomphalos the abdominal contents protrude through the anterior abdominal wall but are covered in an amniotic sac formed by amniotic membrane and peritoneum
Cows milk protein intolerance.
regurgitation and vomiting diarrhoea urticaria, atopic eczema 'colic' symptoms: irritability, crying wheeze, chronic cough rarely angioedema and anaphylaxis may occur
immediate (IgE mediated) and delayed (non-IgE mediated)
3-6% of all children and typically presents in the first 3 months of life in formula fed infants, although rarely it is seen in exclusively breastfed infants
Klinefelters.
47, XXY
often taller than average lack of secondary sexual characteristics small, firm testes infertile gynaecomastia - increased incidence of breast cancer elevated gonadotrophin levels
Diagnosis is by chromosomal analysis
Androgen insensitivity.
X-linked recessive.
Genetically male children to have a female phenotype.
End organ resistance.
Primary amenorrhoea.
Undescended testes - groin swelling.
Breast development - result of testosterone to oestrdiol.
Dx - buccal smear.
Mx - couselling - raise as female. bilateral orchidectomy (ca risk). oestrogen therapy.
Eczema.
Causes:
Atopic (commonest)/allergic
Irritant
Venous stasis
Diagnosis of eczema.
Clinical features + high IgE + specific allergens identified on skin prick and RAST.
Management of eczema.
Avoid irritants. Emollients. Topical corticosteroids. Occlusive bandages. Abs and antivirals. Antihistamines. Dietary elimination.
Childhood psoriasis.
Rare before age 2.
Guttate type or pityruasis rosea.
Guttate type psoriasis.
Most common in children.
Follows strep/viral sore throat.
Small, raindrop shaped round oval scaly patches.
Resolves in 3-4/12.
Pityruasis rosea.
Acute, benign, self limiting of viral origin.
Single round scaly macule - number of small dull pink ones.
Spreads in posterior ribs - fir tree pattern.
Sometimes itchy.
Resolves in 4-6 weeks.
Nephrotic syndrome.
++ proteinuria
Triad: low albumin, proteinuria, oedema and hyperlipidaemia.
Symptoms - general (anorexia, GI disturbance, infection)
Oedema (orbital, genital), ascites, oliguria, breathlessness.
Investigations in nephrotic syndrome.
Urine - frothy, +++ albumin and casts, low Na
Bloods - hypoalbuminaemia, U&C nora
Renal biopsy in older . kids.
Most common cause of nephrotic syndrome in children?
Minimal change disease.
Management of nephrotic syndrome?
Low sodium diet limits the oedema.
Consider furosemide IV and spironolactone PO.
Prednisolone for 3-7/12.
What is the most common cause of nephritic syndrome in children?
Post streptococcal GN = nephritic syndrome
What is nephritic syndrome?
HTN, blood +++, protein +, low UO
What are some complications of nephrotic syndrome?
Hypovolaemia
Thrombosis
Infection
High cholesterol
Presentation of UTI in newborns?
Non specific
May develop rapidly into septicaemia
Presentation of UTI in toddlers?
May present as a gastroenteritis
FTT
Colic
Poor UO
Presentation of UTI in older children?
Dysuria without fever
Due to vulvitis in girls or balantitis in boys
How can you collect urine samples in children?
Clean catch in a sterile pot
Adhesive bag to perineum after careful washing
SPA in critically ill kid
How is a paediatric UTI diagnosed?
Culture
What organisms cause paeds UTI?
E.Coli, Proteus, Pseudomonas
Management of paeds UTI?
Oral or IV ABx.
Preventative - avoid constipation, increase oral fluids, encourage full voiding.
What is glomerulonephritis?
Haematuria + oliguria
+/- HT/uraemia
Produced by an immune mechanism in the kidney.
What is the most common cause of GN in children?
Post-strep - post sore throat/skin infection
What are some other causes of GN in children?
HSP
Toxins - heavy metals
Bergers disease
Malignancies
How does GN present in children?
Haematuria
Oliguria
HTN
Perioribital oedema
What is seen on investigation of GN in children?
Raised urea
Acidosis
Low C3 titire
How is GN managed?
Restrict protein until passing urine.
Pen V for strep.
Wilms Neuroblastoma.
Malignant tumour composed of embryonic nerve cells
Commonest intra-abdominal tumour in children.
Where does a Wilms tumour originate?
Renal embryonic tissue
What is the management of a Wilms tumour?
No biopsy.
Nephrectomy + 4 months of chemo.
+ radio.
What are the different types of enuresis?
Primary noctural - delay in sphincter competance.
Day time enuresis - lack of sensation, detrusor instability, bladder neck weakness
What is the management of noctural enuresis?
Behavioural
Enuresis alarm
Desmopressin
Management of genu varum/valgum?
Varum - common until 3 years
Valgus - common until 7
Usually resolve
Management of pes planus?
Toddlers - flat feet due to fat pad and flat medial long arch
Persistent - excercises, arch support, surgery.
Causes of intoeing?
Adduction of forefoot
Medial tibial torsion
Persisitent anteversion - femoral neck
Causes of toe walking?
CP
Tight AT
DMD
Risk factors for DDH.
Breech C-section Increased BW Older mother FHx
When should infants hips be examined?
1 day and 6 weeks
What is the ix for DDH?
USS
What is the management for DDH?
Pavlik harness
Serial casting
What are the 3 aspects of talipes?
Inversion
Adduction of forefoot
Plantar flexion
Management of talipes?
Foot manipulation, strapping and splinting
What is the definition of IJA?
Arthritis for > 6 weeks in kids under 16
Stills disease.
Systemic arthritis Affects young children Acute illness, malaise. High spiking fever. Salmon pink rash when fever is high. Low HB, high neutrophils, platelets and acute phase. Can develop polyarthritis.
Polyarticular arthritis.
Girls> boys.
Symmetrical.
Polyarticular disease + RF = juvenile rheumatoid arthritis.
Oligoarthritis.
Young children, usually knee.
Girls with ANA - high risk of eye disease.
Enthesis related arthritis.
Older boys.
Large joints and a sausage finger.
HLA-B27 types.
Acute iritis common.
Juvenile psoriatic arthritis.
Involves interpharyngeal joint.
Sausage fingers + skin lesions + nail pitting.
Transient synovitis.
Common. 2-12 Viral infection May be effusion on USS Bed rest and better in a few days
Perthes
Ischaemia of the femoral epiphysis -> AVN -> revascularisation and reossification
Male: female 5:1
Insidious onset, hip pain, limp
X-Ray - increased density in femoral heads
bed rest and traction, may need to be held in abduction with plaster
Age 5-10
SUFE.
Displacement of epiphysis of the femoral head postero inferiorly.
Bilateral in a 1/5th.
10-15 yo, in growing obese boys.
May be acute presentation after minor trauma.
Limp, hip pain, groin pain, limited movement.
Dx - xray.
Mx - surgical pinning
Osteomyelitis.
Infection in the metaphysis of the long bone.
Common sites - distal femur and prox tibia.
Ix - blood cultures, raised inflammatory markers, bone scan identifies the site of infection
What causes osteomyelitis in children?
Staph aureus, strep haem
What is the presentation of osteomyelitis in children?
Painful Immoblised limb Fever Swelling Tendereness
Management of osteomyelitis?
IVAbx for several weeks, then oral.
Surgical drainage if not responding to ABs.
Limb rest in splint then mobilised.
How is IDDM diagnosed in a child?
Symptomatic + BM 11.1 or fasting BM 7.8 + raised HBA1c
Congenital hypothyroidism.
Untreated can cause cretanism - learning difficulties.
Common and treatable.
What are the common causes of congenital hypothyroidism?
Maldescent of the thyroid and athyrosis.
Iodine deficiency.
Hypothyroidism due to TSH deficiency.
Dyshormongenesis.
What are the clinical features of congenital hypothyroidism?
FTT Constipation Cold, mottled skin Large tongue Hoarse cry Developmental delay
Management of congenital hypothyroidism?
Oral thyroxine.
Titrate dose to maintain normal growth, TSH and T4.
Juvenile hypothyroidism.
Usually AI
Girls
Delayed puberty, short . stature, obesity.
Mx - thyroxine
Congenital adrenal hyperplasia.
AR.
Consanginous marriages.
Abnormal adrenal steroid biosynthesis.
Cortisol deficiency = hyperplasia, increased ATCH and high androgens.
Clitoral hypertrophy, variable fusion or labia.
Enlarged penis.
What can CAH cause?
Salt-losing adrenal crisis - 1-3 weeks old, vomiting, weight loss, floppiness and circulatory collapse .
How is CAH diagnosed?
Increase in 17a OH-progesterone (from cholesterol)
Low Na, high K, metabolic acidosis, hypoglycaemia in crisis
Management of CAH?
Corrective surgery.
Hydrocortisone, saline + dextrose in crisis
What is epilepsy?
Tendancy to intermittent, abnormal electrical brain activity
What are the causes of epilepsy?
Idiopathic
Cerebral dysgenesis
Cerebral damage
Cerebral tumour
Generalised epilepsies.
Status - multiple seizures with no recovery of consciousness between.
Absence, tonic or tonic-clonic - may remit.
Atomic and myoclonic - may accompany cerebral dysgenesis and has a poor prognosis.
Localised seizures.
AKA focal or partial.
Temporal lobe epilepsy.
Most common.
Complex phenomena.
LOC, automatisms, deja vu, jaimas vu
Infantile spasms.
AKA West Syndrome.
Between 4-6 months.
Violent flexor spasms of head, trunk and limbs followed by extension of arms - salaam spasms.
Management of infantile spasms.
Vigabitran or corticosteroids.
What is the prognosis of West syndrome?
Later learning disability +/or epilepsy common.
Typical absence seizures.
AKA petit mal.
1-2% of childhood epilepsy. Induced by hyperventilation. Good prognosis- 95% remission in teens.
How is meningitis confirmed?
Inflammatory cells in the CSF.
What causes meningitis in children?
Viruses are most common - entero/arbo
Bacteria - dependent on age
Malignancy
Autoimmune diseases
Bacterial meningitis in children.
Usually follows bacteraemia.
Host response causes to most damage.
Pathogenesis of damage in meningitis.
Inflammatory mediators, leukocytes and endothelial damage –> cerebral oedema, raised ICP, low cerebral blood flow.
Which organisms cause bacterial meningitis in children?
<3 months - group B strep, e.coli
Others - N.Meningitidis, strep pneumoniae
Presentation of meningitis in children?
Meningeal - stiff neck, photophobia, spasms.
Raised ICP - irritable, high pitched cry, drowsy, vomiting, tense fontanelle.
Septic signs - fever, arthritis, purpuric rash (in meningococcus), cyanosis, DIC, raised WCC.
Shock - tachycardiac, hypotensive, poor CRT, oliguria.
Management of childhood meningitis?
Cefotaxime/cephtriaxpne
Ampicillin in <3 months
Dexamethosome reduces long term complications.
Complications of meningitis?
Hearing loss. DIC. Hydrocephalus. Ataxia. Epilepsy.
What is cerebral palsy?
A range of chronic disorders in posture and movement caused by non-progressive CNS lesions sustained before 2 years old.
Results in delayed motor development, evolving CNS signs +/- learning disability +/- epilepsy.
What are the causes of cerebral palsy?
Antenatal - cerebral dysgenesis, rubella, CMV.
Intra-partum - asphyxia/trauma
Post-partum - IVH/meningitis/trauma
Spastic cerebral palsy.
70%
Damage to UMN
Increased tone, brisk reflexes and down-going plantars
Hemiplegia, diplegia, quadriplegia
Dyskinetic cerebral palsy.
Leading to constant involuntary movements and poor postural control.
Intellect unimpaired.
Damage to basal ganglia and extra-pryamidal pathways.
Ataxic hypotonic cerebral palsy.
Signs are usually symmetrical.
Early hypotonia, poor balance and delayed motor development.
Later - incoordination and intention tremor - cerebellum dysfunction.
Intracranial neoplasms.
Present with signs of raised ICP +/- false localising signs. Headache. Vomiting. Papilloedema. Squint (6th nerve palsy) Nystagmus Behaviour change.
Duchennes Muscular Dystrophy.
X-linked recessive. Age at dx 5.5 Waddling gait Gowers sign Climb stairs 1 by 1 Pes Cavus
What is the prognosis of DMD?
Progressive, in wheelchair by 10-14.
Death in late teens.
Beckers Muscular Dystrophy.
Some function dystrophin maintained.
Slower progression.
Dx at 11, wheelchair in 20s, death in 40s.
Congenital muscular dystrophies.
Present with muscle weakness at birth/early infancy.
Benign acute myositis.
Follows URTI
Self-limiting course
Pain and weakness.
Dermatomyositis.
Gradual onset.
Ascending muscle weakness.
Heliotrope rash.
Post excercise pain.
Dystrophia myotonia.
Inherited.
Floppy baby.
Presents in school age with learning difficulties, expressionless face, distal wasting and myotonia.
Metabolic myopathies.
Floppy infant/older child with weakness + cramps on excercise.
Congenital myopathy.
Generalised hypotonia and muscle weakness.
Categorised according to muscle biopsy.
Management of paediatric IDA?
Dietary advice and oral Fe therapy.
Management of aplastic anaemia?
Stem cell or BM transplant.
Immuno-supressive therapy.
Antibodies.
What is sickle cell disease?
An inherited disorder of Hb structure or its production.
Sickle cell states.
HbS - homozygous state (SS)
Sickle cell trait (AS)
HbC - sickle cell disease
Sickle cell, homozygous state (SS).
A.A substitution on codon 6
Hb molecule becomes deformed in low o2, dehydration, cold –> thrombosis and ischaemia.
What is the management of sickle cell homozygous state?
Hydration Warmth Analgesia Blood transfusion Blood and BM transplant Penicillin Pneumococcal vaccine Folic acid
Sickle cell trait (AS).
Usually asymptomatic except under v.v.low o2 tensions.
Sickle cell, HbC (SC).
Nearly normal Hb levels, few painful crisis, but may develop proliferative retinopathy.
What is thalassaemia?
Syndromes are due to inherited defects of globin chain synthesis.
Low or absent chain - excess of the other type - precipitates in RBC membrane - cell death in BM and premature removal from the circulation by the spleen.
What are the 3 types of thalassaemia?
Major
Intermedia
Minor
B-Thalassaemia Major.
Severe anaemia and jaundice from 6/12
FTT
Hepatosplenomegaly
Extramedullary haemopoesis - BM expansion - classical faces (maxillary overgrowth and skull bossing).
Management of b-thalassaemia major.
Regular blood transfusions and chelating therapy.
BM transplant is curative.
Management of b-thalassaemia intermedia.
Clinical manifestations are more variable.
Anaemia is moderate.
Transfusion is sometimes required.
Management of b-thalassaemia minor.
Asymptomatic.
Confused with mild IDA and can leads to unneccesary Fe therapy.
Alpha-thalassaemia.
More common in Asians.
4 gene deletion - fetal hydrops.
3 gene deletion - moderate chronic haemolysis.
1-2 gene deletion - no clincial syndrome.
What is the presentation of leukaemia?
Malaise. Infection. Abnormal bruising. Herpatosplenomegaly. Lymphadenopathy. Bone pain.
What would investigations for leukaemia show?
Low hb.
Leukaemic blast cells on film.
What percentage of children have an innocent murmur?
30%
What are the 2 main types of innocent murmur in children?
Venous Hum:
In head and neck veins.
Disappears when lying flat.
Ejection Murmur: Turbulant blood flow. Soft blowing systolic murmur. Localised. No radiation.
VSD.
Parasternal heave Harsh, loud pansystolic murmur. Tachypnoea. Tachycardia. Enlarged liver.
Ix - ventricular hypertrophy.
What is the management of VSD?
20% close in 9/12.
Given diuretics and ACE-i.
ABx to prevent endocarditis.
PDA.
Common in preterms.
Left to right shunt (aorta - pulmonary artery)
What are the signs of a PDA?
FTT Pneumonia Heart failure Collapsing pulse Thrill Continuous murmur before L clavicle
What is the management of PDA?
Most close over time.
Give dex in preterm labour.
If symptomatic - fluid restriciton, diuretics, idomethacin (PG synthetase inhibitor).
Surgical ligation or occlusion.
ASD.
Seen in children with downs syndrome.
Widely split, midsystolic murmur, left sternal edge.
Coarctation of the Aorta.
Associated with other lesions
Duct dependant circulation
Circulatory collapse when the duct closes
Murmur between shoulder blades
Difference in femoral pulses, raised BP in arms vs elgs, no foot pulses.
What is the management of coarctation of the aorta?
Resection and end to end anastamosis
Balloon dilatation.
What are acyanotic heart lesions?
VSD
PDA
ASD
Coarctation
What are cyanotic heart lesions?
Tetralogy of Fallot
Transposition fo the Great Arteries
What is Tetralogy of Fallot?
4 features. Large outlet VSD. Overriding aorta. Pulmonary stenosis. RVH.
What are the signs of Tetralogy?
Cyanosis as the duct closes Hypercyanotic spells SOB Faints Squatting ay plau Lous ejection systolic murmur at left sternal base.
What is Transposition?
When there are 2 parallel circulation - no mixing is incompatible with life.
Signs usually present on day 1 or 2 of life with cyanosis.
Cyanosis, clubbing, CCF +/- systolic murmur.
What is the management of transposition?
PG infusion to keep DA open
Life saving balloon atrial septoplasty
