Pediatri Flashcards
Just before delivery, strong uterine contractions leads to reduced placenta function.
- Every normal vaginal delivery leads to hypoxemia.
- The fetus has during the delivery SaO2 of 30-40%
(down to 20 %).
That´s why the heart rate is high: 110-160 bpm
Umbilical cord pH ~7.25 (7.15-7.35) is normal.
- Mild acidosis is well tolerated
- Pathological acidosis: pH < 7.00 (0,3-0,4 % of all newborn)
A newborn infant is always CYANOTIC right after birth!
May persist for 5-10 min in healthy infants.
Asphyxia (greek); without pulse
Def. Asphyxia: Impaired gas exchange in a fetus/newborn infant leading to:
- Hypoxia
- Hypercapnia
- Metabolic acidosis (lactacidosis)
- Glycogen use
• The fetus has ”buffer capacity” and is able to compensate for acidosis around 15 min, thereafter
”big trouble”.
Perinatal asphyxia - causes:
Prenatal
1. Sub-chronic
– Placental insufficiency leading to growth restriction
– Redistribution of blood to brain and heart
2. Sub-acute
– Preeclampsia
3. Acute
– Placental abruption, shoulder dystocia, compressed cord etc.
Postnatal
- Prenatal asphyxia continues (80 % !)
- Congenital infection?
- Maternal drugs?
- Congenital malformation?
How do we perceive the asphyxia?
Before/during birth:
• Less fetal activity
• Meconium-stained amniotic fluid
• Fetal tachycardia (even worse bradycardia….)
• Uniform late decelerations and/or “Silent pattern” (CTG)
After birth: • Apgar-score 0-6 at 1 or 5 minutes • No BREATHING effort within 30 sec • No CRYING within 60 sec • Initial bradycardia and floppy • ”No signs of life”
Normal CTG
Baseline of fetal heart rate:
• 110 – 150 beats/min
Variability/accelerations:
• 5 – 25 beats/min
• ≥ 2 accelerations/60 min
Variability/accelerations:
• Uniform, early decelerations
• Variable, uncomplicated decelerations
(amplitude < 60 beats)
Preterminal CTG: (Red flag!!!)
- No variability (< 2 beats/min) without accelerations regardless of decelerations / heart beat
APGAR-score (0-10, der 10 er best, ønsket)
- Appearance
- Pulse
- Grimace
- Activity
- Respiration
Observe: 1. Respiration – Breathing /crying? – Gasping/apnea? 2. Heart rate – Evaluation only if child is not breathing adequately – Listen to the heart, palpate the cord. 3. Is the muscle tone good?
If these parameters are fine: Relax!
However, if the child is not breathing/crying or if the child is very floppy it might need: A. Airway (and initial measures) B. Breathing = ventilation C. Circulation = chest compressions D. Drugs
- 6-10 % of all newborn infants need some stimulation/ ”assistance” to initiate breathing after birth.
- < 1 % need resuscitation.
Of 100 000 newborn in Sweden:
– 0,2 % (> 32 weeks) required unexpected resuscitation and 90% of these responded to mask ventilation alone.
CONCLUSION: Mask ventilation is essential
Skin color/complexion:
- 0: blue or pale
- 1: blue extremities, pink body
- 2: no cyanosis :-)
Pulse rate:
- 0: absent
- 1: <100
- 2: >100 :-)
Reflex irritability:
- 0: no response to stimulation
- 1: weak cry/grimace
- 2: cry or pull away :-)
Muscle tone:
- 0: none
- 1: some flexion
- 2: flexed arms and legs that resist extension :-)
Breathing:
- 0: absent
- 1: weak, irregular, gasping
- 2: strong, lusty cry :-)
Initial measures
A) Stimulation
• Dry the baby warm with towels.
But don’t waist time with this if the baby is very sick!
• If the baby has established good spontaneous respiration and heart rate > 100 the situation is good :-)
B) Ventilation: mask cover mouth and nose.
Indicated if:
– Heart rate < 100/min in spite of stimulation or always when the baby has a very poor respiratory effort / is gasping.
– Always if very bradycardic and pale, start immediately.
• Suction?
– Not routinely. Too vigorous suctioning can cause bradycardia and delay the onset of respiration!
• Open airways, avoid/minimize leak around face mask
• Self expanding bags or Neopuff®
• Ventilation rate 30-60/min.
• Initiate ventilation with room air (21% O2) in neonatal resuscitation (term).
Open airway
- Head in neutral position
The primary measure of adequate initial ventilation is prompt improvement in heart rate.
C. Circulatory support = Chest compression
Heart rate < 60/min in spite of adequate ventilation for at least 60 s.
Ventilation most important and must be optimized before you start chest compressions
- Two thumb technique (+)
- Two finger technique
- Combined ventilation and chest compression
Chest compression 90/min - Ventilation 30/min
Ratio 3:1 (120 events/min)
D. Drugs
• Rarely need for drugs, if needed give drugs via umbilical vein
– Adrenaline (repeat every 3-5 min):
• IV: 20 (10-30) μg/kg = 0.2 ml K-adrenaline/kg
(Term: 0,5 ml)
– Volume (suspect hypovolemia or haemorrhage):
• NaCl 10-20 ml/kg over 10 min
• Blood transfusion (same volume)
– Buffer: rarely indicated
– Naloxone: Not routinely
Summary
• Healthy newborn baby – Cries – Skin colour pink after 5-10 min – Heart rate > 100/min – Measures: Give to mom!!
• Moderate asphyxia
– Poor respiratory effort
– Heart rate > 60/min
– Measures: Stimulation, maybe ventilation.
• Severe asphyxia – Pale, no breathing – Heart rate < 60/min (down to zero) (but detected within 15-20 min before delivery) – Measures: ”Full” resuscitation!
.
Normal values Hgb [g/dl]:
Newborn: 17
3 months: 11
Over 12 y ♀: 14
Over 12 y ♂: 15
Anemia if toddlers have hgb < 10
Lav MCV, small erytrocytes,
think Iron Anemia, meassure iron and ferritin.
β-thalassemia: compensatory elevation of HbF Ineffective production of erythrocytes – Target cells • Blood transfusion
Composition of the hemoglobin chains
• Fetal: HbF Alfa-2-Gamma-2
• Adult:
- HbA: Alfa-2-Beta-2
- HbA2: Alfa-2-δ-2 (2-3%)
High leukocytes in newborns: (10-25 × 10^9/L)
• Platelets as in adults
TEC (transient erythroblastopenia of childhood) = Transient erytroblastopenia
- Sudden anemia in otherwise healthy young children (usually 6 months to 3 years).
- Autoimmune response to earlier (often viral) infection?
- Anemia-development prolonged => adaptation, mild symptoms.
- Transient stop in erythropoiesis.
- Bone marrow: Missing red precursors.
- Reticulocytes 0, Hg often 5-6.
- Normal iron, ferritin, trc, leucocytes.
- Spontaneous remission (after 1-2 months).
Hereditary spherocytosis
• Frequent hemolytic anemia in Northern Europe,
1: 5000,
- 75% autosomal dominant
- Defective cell wall => spherocytes => degradation in the spleen.
- Clinic picture variable, ranging from asymptomatic to significant anemia and jaundice.
- Hemolysis, increased reticulocyte count.
- Can get gallstones secondary to hyperbilirubinemia.
- Risk of aplastic crisis by infection with parvovirus B19.
- Treatment: Await Transfusion? Splenectomy?
Trombocytopenia in children
• Petecchiae/nose bleeding: When trc < 10
- ITP
- Leucemia
- Aplastic anemia
- Treatment with cytostatic drugs
- Sepsis / DIC
- HUS
Idiopathic thrombocytopenia (ITP)
• Bleeding disease in childhood - Trc-antibodies following infection (1-3 weeks in advance) - Debut symptoms: Petechiae, low trc - Treatment: Only if mucosal bleeding Gamma globuline, prednisolone - 20% last more than 6 months
von Willebrands disease
• Autosomal dominant, variable penetrance.
- Different types: Reduced production and / or abnormal function of vWF.
- Symptoms: Nosebleeding, gum-bleeding, menorrhagia, postoperative bleeding, …
- Mild cases: Normal INR, APTT, trc Measure vWF factor level and -activity.
Treatment:
- secure hemostasis.
- Cyklokapron® (fibrinolysis inhibitor)
- Octostim® (Releases endogenous factor VIII)
- Plasma-derived factor VIII contains also vWF
- Oral contraceptive pill
Leucemia – debut symptoms • Pallor / anemia - Fever / frequent infections (skin-) bleedings - Enlarged lymphglands - Liver / spleen enlargement - Bone / joint pain - Reduced general condition
ALL - ca. 35 children annually in Norway - age: most often 2-5 years Treatment • Chemotherapy NOPHO - Total 2.5 years of treatment - Normally no bone marrow transplantation (Only in case of high risk and lack of treatment response or at relapse).
Mononukleosis / EBV
• Age-dependent symptoms:
- Post infectious fatigue
- generalized lymphadenopathy
- hepatosplenomegaly
- Activated lymphocytes in PB
Diagnostic tools:
- Mononucleosis Rapid Test
- EBV serology
Acute tonsillopharyngitis / acute lymphadenitis
Throat inflammation in children: 70% viral
Bacterial tonsillopharyngitis:
- Gr. A streptococcus
- Some GAS strains form pyrogenic toxins -> scarlet fever
Possible, but rare complications:
- Peritonsillar / retropharyngeal abscess
- Post-streptococcal diseases
Symptoms:
- Throat pain, fever, enlarged, injected and coated tonsils and regional tender nodal sites. Rapid growth, high CRP.
Diagnostics:
- Throat bact.swab and / or streptococcal antigen test
- Evtl: Hb, leuc and CRP
- Treatment: Penicillin
Acute lymphadenitis:
- Bilateral cervical: Most often Gr. A streptococcus
- Unilateral cervical: Usually Staph. aureus
Atypical mycobacteria / chronic lymphadenitis
- Other mycobacteria than M. tuberculosis and M. leprae
- Inoculation (eg. Sandboxes or swimming pool)
In healthy children (1-5 years): Isolated lymphadenitis (± abscess) - Cool, without inflammatory signs - Discolouration - Slow development
Diagnostics: -> Clinical! Mantouxtest / QuantiFeron
Histology + direct microscopy Cultivation (4-6 weeks !!!)
Surgical excision of the bump (evtl. + AB treatment)
Atopic eczema gives recurrent infections
–> Lymphadenopathy
Several axillary and inguinally palpable lymph glands (1 x 1 cm).
Causes of lymphadenopathy in children:
- Bacterial infections: Streptococcus, staphylococcus, atypical mycobacteria, tbc,
- Viral infections: EBV, CMV
- Autoimmune diseases: SLE
- Kawasaki
- Malignancies: Leukemia, Lymphoma
- Reactive hyperplasia
Pediatrics
Defined by age: Prematurely born infants (down to 23 weeks gastation up to 18 years)
Neonate < 4 weeks
Infant < 1 year
Normal respiratory rate, decreases with age:
Newborn: 40
1 year: 30
5 years: 20
Adults: 12
Heart rate:
<1 år: 110-160 1-2 år: 100-150 2-5 år: 95-140 5-12 år: 80-120 >12 år: 60-100
Blood pressure, increases with age
Systolisk BT: <1 år: 70-90 1-2 år: 80-95 2-5 år: 80-100 5-12 år: 90-110 >12 år: 100-120
Acronym HELP
To help the doctor and the patient ensuring high quality of health care!!
- History
- Examination
- Logical deduction
- Plan for management
Examination - 1 Look (observation) first! • Severity of illness • Breathing • Growth, nutrition • Behaviour • Hygiene, care
• Do NOT ask the child for permission, instead..
– Say friendly that
“I am now going to examine you”
Examination - 2
• The order of the exam can be individualized.
• Start by observation, introduce instruments and let the child check them out, keep invasive or painful parts for the end.
• Explain everything you will be doing
– Use age-appropriate, non-threatening terms
– Give feedback
• The child has to be undressed for the examination, but this can be done gradually and in stages.
Examination - 3
• General appearance!
– Observe
– Listen to history
• “The sick looking child”?
• Kids are impatient, so a systematic full examination may be difficult. Examine the most pertinent area first.
• Best examination method by age:
– Neonates, very young infants: on examination table.
– Up through preschool: lying/sitting on mother’s/father’s lap.
– Adolescent: sometimes without family present (ask what they want).
• Warm hands and warm the stethoscope!
Examination - 4
• Record respiratory rate FIRST, before crying starts.
– In child, breathing sounds are easier to hear, but hard to localize and hard to interpret.
• Auscultation of the heart early!
• Circulation
– Skin, capillary refill time!
• ENT (Ear, Nose & Throat) exam likely to induce a cry; goes last! Often most distressing.
• Opportunism (“use the chance you get”)
– If child dozes/sleeps – auscultate the heart.
– If child kicks examiner - observe hip range of motion.
– If cries - sometimes possible to inspect the throat.
Throat
Don’t use a spatula if you don’t need it!
Examination of the throat is best left as the last item of any examination as it is seldom comfortable.
It is much easier if the child willingly opens his mouth,
but if not, be firm, gentle, quick and careful.
Severity of illness ”60 second assessment”
• Consciousness • Airway – Effort, stridor, wheeze • Breathing – Repiratory rate, cyanosis • Circulation – HR, pulse, peripheral temp, capillary refill time
Respiration - 1 Important to assess: • Respiratory rate! • Chest wall recessions • Inspiratory stridor? • Expiratory grunting or wheezing? • Ausculation – Limitations in children!
Tachypnoea: Neonate > 60 Infant > 50 Young children > 40 Older children > 30
Respiratory rate: Count before you use your stethoscope!!
Abdomen
- Inspection first
- Palpation then
- Flat hand
- Hips flexed!
• Genital examination often routine in infants
– E.g. are testicles normally descended?
– Later only if relevant
• Rectal examination
– NOT part of routine
– Only if relevant
- ‘Milk’ the testicle downwards towards the scrotum.
- Do not feel from “the bottom” to see if the testicle
is present, this will induce the cremasteric reflex and make it difficult for you to find the testicle. - Warm surroundings/bath tub.
Brief neurological screen
• Parents account of developmental milestones.
• Adapt the neurological examination to the child age.
– Use common sense to avoid unnecessary examination
– Play, write
– Walk, run and jump!
– Language, vision, hearing (ask parents)
– Social interactions
• Usually not necessary with a “formal” neurological examination incl. reflexes, only in cases where you suspect neurological disease.
Motor development - Milestones
Newborn: limbs flexed, symmetrical postures
6-8 weeks: Raises head to 45 degrees.
3 months: elbow support when laying on stomach
6-8 months: sits without support. (At 6 months with round back, at 8 months with straight back).
8-9 months: crawling
10 months: walk around furniture
12 months: walking unsteadily, broad gait, hands apart
15 months: walks alone steadily
DEVELOPMENTAL EVALUATION IN CHILDREN
- GROWTH
- Weight, length, head circumference, puberty - MOTOR
- Pattern of movements, tonus, reflexes - SENSES
- Vision, hearing - MENTAL
- Social abilities / communication and language - NATURAL FUNCTIONS
- Bladder and bowel control, menstruation
Anthropometry • Weight • Length • Head circumference • BMI
Weight Average birthweight ~ 3500 (2500-4500) g – Physiological weight loss < 10 % – Normal weight gain pr week: • 3-4 weeks: 250 g / week • 6 months: BW x 2 • 12 months: BW x 3
Length • Average length at birth ~ 50 cm • 0-1 year: 25 cm / year • 1-2 year: 12,5 cm / year • 2 years: 1⁄2 adult height
Head circumference
• Average at birth 35 cm
• 1 year 46 cm
• 16 years 56 cm
Growth charts
• Based on cross- sectional studies of healthy children
• 50 centile = median value (0 SD) = Normal for halvparten (median)!
• 2.5 perc (- 2 SD) Liten
• 97.5 perc (+ 2SD) Stor
Concern when:
• Crossing of two centile lines
• Measurements above/below 97,5p/2.5p
Ex: the first steps
25 % by 11 months
50 % by 12 months (median)
75 % by 13 months
90 % by 15 months
97.5 % by 18 months (2 SD)
24 months (”limit age”)
Goal: early detection of delayed development
DANGER SIGNS: • Primitive reflexes remains • Asymmetry • Absence of reflexes • Exaggerated reflexes • (OBS: Spasticity!!!)
Primitive reflexes
Assymetry or longer persistence suggest neurological deficit.
• Sucking reflex/ rooting reflex
– From birth
– Disappearance 2-3 months
• Palmar grasp
– From birth
– Disappearance 3-4 months
• Plantar grasp
– From birth
– Disapperance 8-12 months
MORO reflex
– From birth
– Disappearance 3-4 months
• Asymmetric tonic neck reflex:
– From 1 month,
– disappearance 6-7 months.
• Stepping reflex:
– From birth
– disappearance 1-2 months
• Babinsky reflex:
– Initially up (hard to elicit)
– down from around 1 year
• Postural reflexes
– Parachute: From 9-11 months, Persists !!
– Landau reflex: From 3-6 months, disappears around 1 year
– Lateral support reflex: From 7 months, persists
Fine motor development
Newborn: Follows face and light to the midline
6 weeks: Smiles. Follows face and object to the midline
4 months: reaches out for toys
7 months: transfer toys from one hand to the other
10 months: mature pincer grip !
16-18 months: makes marks with a crayon 2 years: make a line 3 years: make a circle 4 years: make a cross 4,5 years: make a square < 5 years: make a triangle
3-4 months: Laughs !
6 months: Follow moving objects, 180 degrees
7 months: turn to soft sounds out of sight
7-8 months: BOEL-test (Eye Orients After Sound)
Social skills:
6 weeks: smiles responsively
6-8 months: puts food in mouth
10-12 months: waves bye-bye, plays peek-a-boo
2 years: dry by day & pulls of clothing when peeing !!
Bedwetting?
• Coordination and bladder control generally occurs by
4 years of age.
• Nighttime between 5-7 years old.
• Still ~10% of 7 year old children have some difficulty staying dry.
Bowel control
• Expected bowel control by the age of 4.
• 1-2 % of school children has passage of stools in inappropriate places.
– The vast majority due to functional constipation.
When to worry • At any age: – Parental concern – Hypotonia – Regression in previously acquired skills – Persistent primitive reflexes.
When to worry • 6-8 weeks: – Not smiling – No eye-contact • 6 months. – Little interest in people, toys, noises • 10 months – No sitting – No pincer grasp – Hand preference development • 18-24 months – Not walking independently – No speech • 3 years – No sentences (2-3 words) • 4 years – No intelligible speech
Causes of developmental delay
• Motor
– Normal variant (e.g. bottom shufflers)
– Neurological disorder (e.g. CP)
– Neuromuscular disorder (e.g. Duchennes)
– Any cause of global developmental delay
• Communication (speech, language and nonverbal)
– Hearing disorder
– Visual disorder
– Lack of stimulation
– Articulation defect (e.g. physical abnormality)
– General developmental delay
– Autism
• Global
– Genetic low intelligence
– Lack of stimulation
– Chronic illness
– Genetic disorder or syndrome (Down, metabolic disorder)
– Antenatal disorder (e.g. congenital infection)
– Birth asphyxia
– Prematurity
– Hypothyroidism
– Neurological insult (e.g. trauma, meningitis)
Further investigations – Blood • thyroid function tests • chromosome analysis • metabolic screen • other – Imaging • Cranial ultrasound • CT /MRI – other • EEG • Nerve conduction studies • Muscle or nerve biopsy
-.
Global causes of death in children < 5 years of age
- 5.9 million in 2015
- ~2/3 infectious causes
Maternally IgG is tranferred from placenta
What is fever?
Rectal temperature > 38
• High fever (>40-40.5 oC) in children < 3-6 months is associated with a substantial risk of severe infection.
• Low threshold for admission!
BUT
• Children <3-6 months may be afebrile and still have a severe infection.
• Always consider the general condition as well !!
Investigations • Complete blood count (CBC) – Often high or very low WBC in severe bacterial infections • CRP , procalcitonin • Blood culture • Urinary specimen – Dip stick and culture • Lumbar puncture • Chest X-ray
Capillary refill time, press thumb on chest, should refill within 2-3 sec.
Failure to pass meconium
- No bowl movements the first 48 hours since birth.
- It is normal to pass bowel movements the first 24 hours.
Diseases • Imperforate anus - no hole. may be stool in fistula. - VACTERL syndrome V = vertebrae anomalies A = anus (imperforated) C = cardiac problems T = tracheal esophagal fistula E = esophageal atresia R = renal L = limb - Ultrasound, x-ray, ecco-cor, catheter into stomach, voiding cyster urethra, x-ray of wrist. Prepare for intubation/tracheotomy.
• Meconium ileus
- Pathology: Cystic Fibrosis, often discovered on prenatal screening; chlorine sweat urine. Give baby Vit ADEK and pancreatic enzymes, cause exocrine ones are disfunctional.
- Diagnosing: x-ray, transition, gas filled plug.
- Treatment: water enema may dissolve meconium plug.
.Other
• Hirschsprungs, constipation year 2, absent plexus.
- Failure of migration during embryogenesis.
- Diagnosis: palpable colon, explosive diarrhea.
Good colon is dilated, bad colon looks normal.
Sepsis
< 3 måneder (neonatal)
- Group B strep
- E.coli og andre gram neg.
- Listeria
- Staph. aureus
> 3 måneder
- Strep. pneumonia
- Group A strep
- Neisseria
- Haemofilus influenza B
- Staph. aureus
- Salmonella
Immunsupprimert
- Gram neg.
- Fungi
- Opportunister
Meningitis after the neonatal period Bacterial agents • Meningococci and pneumococci – H. influenzae B ”eradicated” through vaccination • Similar scenario with pneumococci (?) – Other agents (streptococci etc.) • “Rule of thumb” Meningitis UTEN petecchia → Pneumococci Meningitis MED petecchia → Meningococci
Meningitis Management • Seldom septic shock • Lumbar puncture – if not contraindicated • Antibiotics iv – 3rd generation Cephalosporine • Symptomatic (as sepsis) – Careful with fluids
Meningococcal sepsis • Rash – Initially pale – Later classical skin bleedings • Poor general appearance! • Irritability, reduced level of consciousness, influenza like body pain
Management, Prehospital • Follow respiratory state and administer oxygen • Fluid resuscitation (IV/IO) if poor circulation – if shock: 20 ml/kg over 10 min • Give antibiotics (IV/IM/IO) – Penicillin or Cefotaxime/Ceftriaxone • “Shout for help” – AMK/Emergency service
Symptoms
• Small children (< 18 months)
– High fever, rash, vomiting, fatigue/
irritability, seizures, BULGING fontanel
– Often no obvious neck stiffness
• Older children (> 18 months)
– More ”classical” symptoms with high fever, neck stiffness, headache, photophobia, irritability, reduced level of consciousness
Respiratory tract infections (RTI)
~ 2/3 of all infections in children
• Upper RTI
– Common cold, otitis media, pharyngitis, tonsillitis, laryngitis
• Lower RTI
– Bronchitis, bronchiolitis,
pneumonia
• “United airways disease”
RTI - etiology Children
• Virus!!!
• Bacteria (less frequent):
Suspect when fever continues > 72 h!
– Pneumococci (otitis, pneumonia) and Group A streptococci (tonsillitis)
• Both almost uniformly susceptible to Penicillin in Norway.
– Bordetella pertussi
• Whooping cough; not completely protected by vaccination.
– Mycoplasma, chlamydophila
• Often, but not always, more severe clinical picture in children > 5 years.
• May be self limiting or require treatment, MACROLIDE
SpO2 < 92 % without oxygen is a serious condition
Otitis media
• 80 % recover WITHOUT antibiotics within 2-3 days
– Nose drops and Paracetamol
• “Wait and see” prescription
– Penicillin V
• Antibiotics if:
– < 1 year of age
– Persistent high fever
– Recurrent otitis
Throat infections
Aetiology
- Virus 90%
- Streptococci (GAS) 10%
Diagnostics
- Strep A test
- CRP, blood count?
Treatment
- Penicillin if streptococci
Infectious mononucleosis
Incubation time: 30–50 d.
EBV infects epithelia and B-lymphocytes in lymph nodes and spleen.
Host response; CD8+ T-lymphocytes (activated lymphocytes, blood slide).
Mainly symptomatic in adolescents.
Often asymptomatic in small children.
Possible complications
- Airway obstruction
- Spleen rupture (very rare)
- Feeding problems
- CNS (incl. encephalitis)
- Hematology (ITP)
Acute laryngitis
Spasmoid/viral croup (”falsk krupp”)
Inspiratory stridor
Barking cough
- Diagnosed per phone..
Treatment:
- Elevated head position, cold air
- Nebulized adrenaline
- P.o. Steroids
DD: Epiglottitis (uncommon today)
Acute bronchiolitis
- Affecting the small bronchioles
• RS virus (RSV) and/or many other respiratory viruses (hMPV, adeno, influenza, rhino etc.)
• “Winter” disease
– Infants 1-12 months.
• Children < 6 months and those with chronic underlying conditions are most severely affected.
• Only 1 out of 100 infants with RSV infection are admitted to hospital.
Symptoms • Nasal congestion!! • Cough and fever +/- • Apnea (in the youngest) • Breathing difficulties!!
Management • Oxygen via nasal prongs – Low flow or high flow with heated humidified gas • Give nasal spray/droplets! • Nebulized physiol. SALINE • Adrenaline (Norway) • CPAP • IV or enteral fluid
Pneumonia
Symptoms and findings • Cough and fever (> 38,5 o C) • Respiratory distress – Grunting, rapid respiratory rate, nasal flaring, but not always possible to diagnose on auscultation. • Consolidation on chest X-ray.
• Bacteria and/or virus!
For Infants: RSV, Adenovirus, Influenzavirus, Parainfluenza, strep. pneumonia, Staph. aureus, Haemophilus influenza, c. trachomatis.
Antibiotic treatment for pneumonia and other RTI
• If antibiotics are prescribed, judged by clinical picture and after careful diagnostics;
Please choose Penicillin V first!
– Narrow spectrum, but covers both pneumococci and streptococci (Gram positive)!!
• Second choice; a macrolide
– Associated with more resistance
– GIT disturbance
– Cover mycoplasma and chlamydophila
Osteomyelitis and septic arthritis
Infectious agent in the joint or bone.
Early recognition important.
Prompt antibiotic treatment.
Bacteria enter the bone/joint, usually through the blood stream (hematogenous).
Different blood supply in young children.
Osteomyelitis may spread through non-ossified growth plate into the joint (arthritis).
Symptoms – Painful, immobile joint – Fever May also observe • Swelling • Tenderness • Red and warm
Most common infectious agents in childhood osteoarticular infections
0 - 1 month: Strep B, Staph aur, Gram negative bacilli
1 month - 4 years: Staph aur, Kingella kingae, Strep A
> 4 years: Staph aur, Strep A
Anatomical location Osteomyelitis • Neonate – Often FEMUR and humerus • Child – Metaphysis of long bones (distal femur, proximal tibia etc) – Pelvic and vertebral infections
Septic arthritis
• Knee, hip, ankle, elbow
• In 25 % a concomitant osteomyelitis
Diagnosis and treatment of Osteomyelitis
• X-ray
– Not sensitive early!!
• Radionuclide bone scan
• MRI !!
– BEST method
• Blood tests (CRP, ERS) • Blood cultures • Aspiration from bone? • Antibiotics (long) – Initially IV, then PO – Staphylococcal coverage
Septic arthritis
• Hematogenous spread of bacteria to the joint
• Often hip
• DD reactive arthritis
• Antibiotics
– Like for osteomyelitis
Leg held: flexed, abducted, externally rotated.
No spontaneous movements (pseudoparalysis).
Vaccination
Immunologic memory to a pathogen.
The most effective means of controlling infectious diseases.
Herd immunity.
How are vaccines made?
• Weakening the pathogen (live):
Cell culture adaptation.
– Examples: measles, mumps, rubella, chickenpox, BCG
• Inactivating the pathogen:
The virus is killed; cannot replicate.
– Examples: polio (IPV)
• Use part of the pathogen:
A piece of the microbe is used to make the vaccine using recombinant techniques.
– Examples: HPV and hepatitis B
• Toxoid vaccines:
Inactivated toxins are called toxoids.
– Examples: diphtheria, tetanus and pertussis (DTP)
• Polysaccharide vaccines:
– Not good inducing immunological memory in children, and less effective < 2 years.
– Used in adults (pneumococcal vaccine etc.)
• Conjugated vaccines:
polysaccharides are attached (conjugated) to a helper protein to make the vaccine.
– Hib, pneumococcal and meningococcal vaccines.
– Induce immunological memory in children < 2 years.
Immunisation programme in Norway:
6 weeks: Rotavirus, given orally
3 months: Rotavirus, diptheria, tetanus, whooping cough, poliomyelitis, Hib infection and hep B, pneumococcal
5 months:
12 months:
15 months: MMR (measles, mumps, rubella)
2nd grade, approx. 7 years:
6th grade, approx. 11 years: MMR
7th grade, approx 12 years: HPV, 2 doses
10th grade, approx 15 years: Diptheria, tetanus, whooping cough and poliomyelitis (DTP-IPV)
Tuberculosis (BCG)
Aim (WHO 1988): Eradicate polio!
– A reduction from an estimated 350,000 cases in 1988 to about 1,000 cases per year today.
– America and Europe are declared “polio-free”.
Haemophilus influenzae B (HiB) vaccination introduced in Norway in 1992
– ”Eliminated” HiB meningitis and epiglotitis
MMR • Measles – a major killer in developing countries – May cause severe encephalitis. – Severely sick patients – High fever and rash "Prior to vaccine “measles was as inevitable as taxes and death”.
Facts about TB (WHO)
• In 2016
– ~ 1/3 of the world’s population infected.
– ~ 10.4 million developed TB disease.
• ~ 0.5 million develop MDR-TB.
• ~ 1 million children infected.
– Annually ~ 250 000 children die of TB.
– Preventive treatment: ~ 13% of eligible children.
• Norway around 1900
– 6-7000 people died of TB each year.
Tuberculosis
Exposition
- Infected (~10-30 %)
- Latent TB (~90%, asymptomatic)
TB disease
~ 10% (clinical symptoms)
No treatment ~ 50% die within 2 years
Treatment –> Cured
TB symptoms in children
• Small children: Fever, chronic cough, failure to thrive.
• Older children: Chronic and Productive cough.
What is different with child TB?
• Immature immune system.
– Development from latent to active TB.
– Short incubation time: 4-8 weeks..
• Extra-pulmonal TB more often (25-35%).
– Lymphglands, pleura, meninges, joints..
• 60% among children < 5 years.
• Early diagnostics and treatment of latent TB is very effective.
Pulmonary TB
• Segmental lesions (< 10 years) – Obstructive emphysema – Atelectasis (collapse-consolidation) – Bronchopneumonia – (Hilar adenopathy)
• Chronic cavernous disease
– > 8-10 years of age
– HIV+
Ekstrapulmonary TB • Meningitis and miliary TB – Mainly < 2 years of age • Lymph nodes – Most common extrapulmonary TB • Bone/spine • CNS
Diagnosis
- Clinical history
- Symptoms/clinical signs
- X-ray; thorax
• Mantoux: Positive 4-6 weeks after infection !
Negative: 0–5 mm
Positive: ≥ 6 mm
BCG reaction: 5–10 mm
Quite sensitive, but not specific.
Also positive after BCG-vaccination and after exposure to atypic mycobacteria.
May be false negative in HIV+, malnutrition, small and very sick children.
• InterferonGammaReleaseAssay = IGRA
– More specific (98-99%); for M. tubeculosis
• No reaction to BCG !
• No reaction to atypic mycobacteria
– More sensitive in immunosuppression
• Does NOT distinguish between latent and active TB
• Bacteriology- SMEAR is the «GOLD standard»
– Microskopi positiv
– PCR (m. tuberculosis complex)
TB treatment
Standard: 6 months antibiotic therapy
Rifampicin (RIF), Isoniazid (INH), Pyrazinamide (PZA), Ethambutol (EMB) (Husk RIP-E)
Daily for 2 months
RIF + INH
Daily or 3 times a week for the next 4 months
Latent TB - Asymptomatic • Pos. Mantoux and IGRA-test • Chest X-ray: Normal • Clinically healthy !! • If untreated the TB can reactivate later in life and turn into TB disease
TB prophylaxis - Latent TB (LTB)
• Indication (FHI in Norway)
– If a child/adolescent is < 18 years of age and originally from a high prevalence region, then
they should receive TB prophylaxis.
– TB prophylaxis = INH + RIF for 3 months
• RIF 10 mg/kg/d (maks 600 mg)
• INH 10 mg/kg/d (maks 300 mg)
BCG
• Recommendation today (Norway)
– Vaccinate newborn infants of parents from high prevalence regions.
– Vaccinate any not infected child where there is TB in the local environment.
– Since 2009 the BCG vaccine is NO longer part of universal immunisation programme (10. class).
• Infants are vaccinated in the delivery ward or at the public health service.
.
Hepatitis A • Fæco-oral transmission • Icteric • Usually benign course • Vaccination
Hepatitis C
• Transmission: blood
• HCV-PCR positive are contagious
• Vertical transmission most important among children
– 79-80% develop chronic HCV infection –>
fibrose –> Cirrhose –> cancer.
• HCV-PCR positiv mother: 4-7% risk of infecting the child
• HCV risk women
– test for HCV antibodies during pregnancy.
Treatment
• HCV-PCR pos children should considered for treatment.
• Antiviral treatment.
• Goal treatment: lower viral replication to stop liver damage development.
Hepatitis B
• Acute infection.
– Small children - few symptoms.
– Older children - flu-like symptoms, fatigue, abdominal pain and joint pain.
• Leading cause of chronic hepatitis, liver cirrhosis, liver failure and hepatocellular carcinoma in the western world.
Vertical transmission (perinatal)
– Cause ~ 50% of chronic hepatitis worldwide.
– 90% with hepatitis positive mothers are infected.
– Cecarian section does NOT protect.
– Breastfeeding no risk of infection
Horizontal tranmission
– Blood
– Vaginal secret and other body fluids
– Injection drug users
• HBsAg (surface antigen);
Virus are present, infectious
• Anti-HBs (surface antibody);
Vaccination response
• HBeAg (antigen);
Active infection and replicating virus
• AntiHBc (core antibody);
Increases after/during infection, NOT vaccination
• HBV-DNA;
Conc of virus in blood, 1 week after infection
• Sero-conversion;
Clearance of HBeAg and production of Anti-HBe
Surface AG and core = Infection
Anti-HBs = Vaccinated
Prevention of mother-child transmission
• No prevention: 90% of children are infected
• Prevention:
– Immunoglobulins and Hep-B vaccine within 24 hours after birth!
– Hep-B vaccine at 1 month
– Vaccine at 3, 5, 12 months
– AntiHBs and HbsAg 1-2 months after the last vaccine
.
When to expect a primary immunodeficieny condition?
- Often congenital.
- Resulting from genetic defects in some components of the immune system.
• Frequent serious infections.
• Infections with unusual pathogens.
• Abscess.
• Often failure-to- thrive.
- 120 genetical defects associated
- SCID = Severe combined immunodeficiency
- IgA deficiency
- IgG deficiency
Secondary = acquired
Patient grops with increased risk of infections
- Premature: Immature immune system.
- Cytotoxic drugs: decreased number gr.c, bacteria, fungi.
- Prednisolon: Reduced inflammatory response.
- Transplanted: Immunosuppressive treatment.
- Anti-TNF: inhibits important parts of the immune response.
- HIV: The virus destroys Th cells first.
- Spleen-ectomized: pneumococcal infections.
- Bone marrow failure: reduced number gr.c and lymphocytes.
- Neutropenia.
- Primary immune deficinency.
IgA deficiency
– Most common humoral antibody deficiency !
– 50-80% asymptomatic.
– Recurrent sinopulmonary infections most frequent manifestation.
– May have severe malabsorption (chronic diarrhea).
– Isolated low IgA level.
– Increased risk of autoimmune disorders.
X-linked agammaglobulinemia (Bruton)
- Asymptomatic to 6 months.
- As long acting maternal IgG (from placenta).
- Repeated lung infections.
- Lacking BKT gene (Bruton tyrosine kinase) that creates a signaling molecule req. for early B cell ext.
- Lacking B lymphocytes, and also Ig.
- Reduced lymphoid tissue.
- Beh: IgG sc.
Neutropenia
Severe: granulocytes < 0.5 !
Very serious: Granulocytes < 0.1
Consequences: Reduced infection defense, especially against bacteria and fungi.
Reduced inflammatory reaction -> little CLINIC.
Causes:
- Acute leukemia (bone marrow infiltration)
- Cytostatics
Fever and neutropenia = MEDICAL EMERGENCY
> 38.5 C, gr.c < 0.5
Often small clinical symptoms.
CRP good parameter (the day after).
High mortality in sepsis, if not early iv treatment !
Clinical presentation:
- Most commonly, bacteremia without focal findings
- Lung (pneumonia)
- Soft tissues (cellulitis, perianal abcess, CVC)
- sepsis
- UTI
Antibiotic treatment
- Ampicillin and Gentamycin
- Alternatively: cefotaksim, ceftazidim
If no effect after 3 days, consider:
- Vancomycin (against KNS - Koagulase-negative stafylokokker)
- Metronidazol against anaerobics, esp with gut symptoms - Fluconazole/ambisome against fungi
Microbiology
Bacteria that need to be covered:
Gram positive:
- Staf. aureus
- Staf. epidermidis streptococcus
Gram negative:
- E. coli
- Klebsiella
- Pseudomonas
- Candida albicans
Varicella • Can be disseminated in immunodef pat • In seronegative: • Acyclovir po day 7-14 after exposure • Treatment (not in at children’s dep) • Acyclovir iv
..
Club foot – talipes equinovarus • Talipes --> talus + pes Talus – ankle Pes – foot • Equinovarus --> Equino + varus Equino – heel elevated varus
Treatment over 4-6 weeks. Ponseti - bandage, then percutaneous achilles-tenotomy.
Summary • Equinus, varus, cavus, adductus • 1/1000 • Boys > girls • 50% bilateral • Ponseti • BRACING !!!!
Developmental dysplasia of the hip - DDH
3 main classes
- Dysplasia 25-50/1000
- Subluxation 10/1000
- Luxation 1-5/1000
Diagnosis
- Leg length and skin folds
- Ortolani and Barlow
Treatment
- Frejka pillow orthosis
- Abduction
Why traction?
• Contract iliopsoas
• Constricted capsule
Summary • Dysplasia --> sublux --> lux • 25-50/1000 • Girls > boys • Clinical examination until walking! • X-ray or ultrasound if suspicion • Pillow/orthosis • Traction/casting • Surgery/casting
Calvé – Legg – Perthes disease
Summary: • Boys > girls • 25 / 100 000 • Self limiting, 2-4 years • Hip/knee pain • Containment! • Avoid running/jumping • Physical therapy • Surgery if loss of containment
Slipped Capital Femoral Epiphysis –> Pin fixation
Summary: • Boys > girls • BMI>25 • Hip or knee pain • Acute • Chronic • Acute on chronic • Pin fixation • Pin removal at age 16-18
Coxitis simplex – transient synovitis
- Acute inflammation
- Limping
- Unloading
- Upper airway infection
- –> X-ray: exclude other
- –> Ultrasound
- Fever? CRP?
- –> septic arthritis
Kochers kriteria:
- Fever (>38,5)
- inability to weight bear
- SR > 40
- WBC > 12
- CRP > 1mg/dL
• Effusion + 2 or more kriteria: –> joint aspiration
Hips and age
Age (years) and Diagnosis
0-2 DDH 2-5 Transient synovitis 3-10 Calvé-Legg-Perthe 10-15 Slipped capital femoral epiphysis 15-50 Secondary coxarthrosis >50 Primary coxarthrosis
- Flat feet
- Leg-length discrepancy = anisomieli
- Scoliosis
Calcaneo-valgus = ankel innover --> plattfot Calcaneo-varus = ankel vender utover
(Rum opens you legs xD)
Leg length discrepancy
- Normal variation 0,5 – 1,5 cm
- Previous injury
- Disease
Scoliosis: Adam´s forward bend test
• Idiopathic
- Infantil (<3 years)
- Juvenile (3-10 years)
- Adolescent (> 10 years)
• Neuromuscular scoliosis
- CP, Duschenne
• Syndrome associated
- Achondroplasia, marfan, downs, neurofibromatosis, osteogenesis imperfecta
• Leg length discrepancy –> functional scoliosis
BREAST MILK
0 – 3 months: 150 ml/kg bodyweight and day
4 – 6 months: 130 ml/kg bodyweight and day
Self regulation
THE INFANT DIET FROM
0 - 6 MONTHS OF AGE
Exclusive breast feeding is highly recommended
ESPGHAN:
No complementary feeding is advised before 17 weeks of age (~ 4 months) and should not b postponed to later than 26 weeks of age (~ 6 months).
The human milk substitute formulas are the only approved nutritional substitution for human breast milk when breastfeeding is not possible.
VITAMIN D
Vitamin D supports the absorption of calcium.
Vitamin D supplementation is highly recommended
to all infants from 4 weeks of age.
Supplements: 5 ml cod liver oil (tran) or 5 vitamin D- drops
gives 10 μg of vitamin D.
THE INFANT DIET FROM 6-12 MONTHS OF AGE
Gradual introduction of complementary foods.
Small portions.
Porridge and soft foods are necessary before introducing bread and more lumpy foods.
PORRIDGE FOR INFANTS
It is important to choose an IRON enriched porridge.
Non enriched cereals should, if used, mainly be used as taste variation, due to the lower iron content.
HOME MADE BABY PORRIDGE?
It is important to know what you are advising!
«Experts» advise parents to use dried apricotes to enrich the porridge with iron.
One desiliter iron enriched Nestlé powder (porridge from 8 months) gives 3,0 mg jern.
From 6 months of age breast milk / formula should not be the only source of energy.
Iron rich foods and enriched porridge is important to prevent iron deficiency.
FOODS THAT SHOULD - NOT - BE INTRODUCED DURING THE FIRST YEAR:
- Honey (infant botulism).
- Spinach, beetroot, leaf celery, nettle and fennel (nitrate / affect oxygen transportation).
- Smoked and salted fish and meat (salt).
- Fish liver and Svolvær- and Lofot pate (dioxines).
- Be aware of soil residues on herbs, cabbage and similar (bacterias).
- Salt should not be added to the infants diet before 12 months of age due to the limited ability to excrete salt.
- Artificial sweeteners and products containing these are not recommended for children before 3 years of age.
RICE MILK AND RICE COOKIES
- Not suitable for children under the age of 6 due to the high levels of inorganic ARSENIC.
DRINKS FROM 6 - 12 MONTHS
- Breast milk and / or formula together with meals.
- If the infant is thirsty between meals some water can be offered from 6 months of age.
- Fruit juices and saft should not be given in bottle (dental health).
BREAST MILK VERSUS COW’S MILK PER 100 ML
Breast milk vs Cows milk Kcal 70 vs 63 Protein 1,3 vs 3,3 Carbohydrates (lactose) 7,2 vs 4,6 Fat 4,1 vs 3,5
Breast milk = more energy, carbohydrates and fat,
LESS protein.
COW’S MILK
- From 1 year of age it is recommended that children drink cow’s milk.
- Fat reduced dairy products.
- Three glasses per day.
Cow’s milk and other dairy products are the only sufficient calcium source in the diet.
Portion sizes:
- 250 ml of milk = 290 mg Ca
- 2 slices of cheese (30 gram) = 215 mg Ca
- 125 ml of yoghurt (1 cup) = 90 mg Ca
- 100 grams of boiled broccoli = 46 mg Ca
”CALORIES” = KILOCALORIES = KCAL
- Gives the energy that the little body need for maintenance, growth and activity
- 1 gram of carbohydrate = 4 Kcal
- 1 gram of protein = 4 Kcal
- 1 gram of fat = 9 Kcal
Neophobia:
A “picky eater”
- Afraid of unfamiliar tastes
- Bitter versus sweet
- A normal phase of development
- Peaks around 1,5 years of age
- A “picky eater” may still be able to get enough variation and have sufficient growth
- 10-15 taste repetitions
- Regulation may be week-to-week rather than day-to-day
- This is normal!
- Focus on growth, not portion sizes
Toddler = 12-36 months.
CHECK QUESTIONS:
When should parents start introducing solid foods?
- When the baby seems interested, earliest by 4 months, latest by 6 months
What texture is recommended to start with?
- Soft, mixed
What kind of supplements will the infant need?
- Vitamin D (10 μg)
Why choose an iron enriched porridge above home made?
- It’s easier to cover the needs
Why should not a 9 month old infant drink cow’s milk?
- It contains too much protein compared to needs and it does not contain iron
Should a toddler drink full fat milk?
- No, the recommendations says lean milk with maximum 0,7 % of fat
How much milk products will a toddler need to get enough calcium from the diet?
- 500 - 600 ml/day including milk and yoghurt
How can we get a 1,5 year old to like vegetables?
- Awareness of the neophobic phase (normal development)
- Keep on trying without pressure
Should we act or await if we see abnormal weight gain in a 3 year old?
- It’s recommended to act early. The intervention should though be “invisible” in front of the child.
What do the Norwegian recommendations say about duration of breastfeeding?
- Exclusive breastfeeding is highly recommended and should be encouraged for the first 6 months, continued in addition to solid foods between 6 - 12 months and thereafter as long as the mother and baby feels happy with it.
Will the breast milk fully cover all needs during the first 6 months of life?
- Yes, except for vitamin D
Henoch Schönlein purpura (HSP)
- the MOST COMMOM systemic VASCULITIS in childhood.
- IgA immune complex deposites in small vessels of the skin, kidney joints and gut.
- Epidemiology: 10-30/ 100 000 children < 17 yrs
Primarily a clinical diagnosis of a child presenting with purpura, + 1 of the following 4:
- arthralgia/ arthritis
- abdominal pain
- kidney: proteinuria and/or hematuria
- skin biopsy: leucocytoclastic vasculitis with IgA deposition
Other symptoms: - headache, CNS manifestations - skin ulcerations - hemoptysis (lung vasculitis) - HSP may also be found in adults diff diagnosis - meningococcal disease - thrombocytopenia
Treatment
- supportive: paracetamol/NSAID if needed for pain
- severe abdominal pain: prednisolon 1 g/kg/d
- glomerulonephritis (GN): immunosuppressive Tx
Follow-up and prognosis
- usually benign course and complete recovery if no kidney disease.
- recurrent attacks of abdominal pain, bloody diarrea.
Urine stix for should be performed
- in the acute phase
- every 2 weeks untill no more clinical signs of purpura or abdominal pain attacks
- outpatient follow-up after 3 months
- individually if hematuria or proteinuria, consider kidney biopsy
Complications HSP Gut: - recurrent attacks of abdominal pain, bloody diarrea - intussusception (3-4%?) Kidney: - 50% mild hematuria - <10% severe nephritis - 2% chronic nephritis - <0.5-1% end-stage kidney failure
If hematuria, follow weekly
If proteinuria, check creatinine, blood pressure
Kidney biopsy if
- severe nephritic syndrome
- nephrotic syndrome > 10 days
- severe proteinuria
Kawasaki disease KD
- febrile immunemediated multisystem VASCULITIS.
- vasculitis in small and medium size arteries, the coronary arteries are especially vulnerable
(coronary artery aneurysms = CAA in 20% if untreated KD, 5% transient and 1% permanent if treated)) - mucocutaneous lymph node syndrome
Epidemiology
- Japan 239 / 100 000 in children <5 years
- Denmark 4-5 / 100 000 in children <5 years
Diagnostic criteria KD:
High fever of sudden onset
1. Bilateral non-purulent conjunctivitis.
2. Polymorph exanthema without vesicula eller crusts
3. Mucosal changes in lips and mouth:
Red cracked lips, «strawberry- tongue», rubor in mouth- and pharyngeal mucosa.
4. Rubor and oedema in palms of hands and feet, followed by skin desquamation in fingers and toes the following weeks.
5. Cervical lymphadenopathy (at least one gland > 1,5 cm).
Complete KD
- high fever >5 days
- 4 of 5 criteria
Incomplete KD
- high fever in young children with mucocutaneous findings, not fulfilling the diagnostic criteria.
NB Kawasaki may also be found in adults.
Mnemonic 1-2-3-4-5 KD
1 for one ‘mouth’ (strawberry tongue and fissured lips).
2 for two ‘eyes’ (bilateral conjunctivitis).
3 for three fingers palpation of neck lymph nodes (cervical lymphadenopathy).
4 for limb changes (swelling of hands and feet).
5 for multiple skin rash throughout the body.
Treatment KD
Immunoglobuline i.v. (IVIG)
- one dose 2 g/kg over 8–12 hours preferably < 10 days after symptom onset.
Aspirin orally:
- given in anti-inflammatory dose (80–100 mg/kg/d in 4 daily doses) until 2 days without fever, then antithrombotic dose (3–5 mg/kg/d) until inflammatory response (ESR, CRP, thrombocytes is normalized, minimum 6–8 weeks.
If no effect 36 hours after IVIG or relapse within 2 weeks, a new dose of IVIG 2 g/kg is given, then consider infliximab 5 mg/kg iv highdose-steroids (methylprednisolone) 30 mg/kg iv over 2 hours in 1–3 days may help prevent coronary artery aneurism formation !
Follow-up and prognosis
- often god response and complete recovery if no coronary heart disease.
Markers of high risk for coronary disease:
- elevated Pro-BNP
- Hyponatremia
- High neutrophile vs lymphocyte counts
EKKO cardiography should be performed
- in the acute phase.
- after 2 and 6 weeks (stop low- dose aspirin if normal), then after 1 year.
- individually if coronary artery aneurisms develop.
Periodic fever syndrome / autoinflamatory diseases
CRP > 100
PFAPA
- Periodic
- Fever
- Aphtous stomatitis
- Pharyngitis
- Adenitis
Several other
- FMF
- Mb Blau
- Hyper IgD syndrome (=MKD)
- PAPA, DIRA, TRAPS, etc
ARTRHITIS
- Hydrops = fluid in the joint or - Limitation of movement AND > one of the following: - Pain on movement - Tenderness over the joint
Differential diagnostics
- Septic arthritis
- Transient coxitis, CLP (Perthe), Epiphysiolysis
- Juvenile idiopathic arthritis
- Reactiv arthritis (streptococci, gut bacteria, chlamydia m.fl.)
- Chronic pain conditions.
- CANCER (Leucemia, sarcoma).
- Chronic inflammatory connective tissue diseases.
Rheumatic diseases in chldren
- Juvenile idiopathic arthritis.
- Systemic connective tissue diseases.
- Juvenile systemic lupus erythematosus (JSLE).
- Juvenile dermatomyositis (JDM).
- Mixed connective tissue disease (MCTD).
- Systemic sclerosis
- Vasculitis
- Henoch Schonlein purpura
- Kawasaki
- Takayasu
- Granolumatous polyangitis etc
JIA = Juvenile idiopatic arthritis
• unknown etiology
• duration > 6 weeks
• onset < 16 years of age
JRA = Juvenile rheumatoid arthritis
JCA = Juvenile chronic arthritis
How common is JIA?
- Insidence: 15 - 22 / 100 000
- Prevalence: 65 - 140 / 100 000 (~1-2 pr 1000)
- Ca. 150 new cases / year in Norway
- Girls : Boys = 2 : 1
Etiology - unknown
- autoimmune mechanisms
- genetic disposition
- triggers; antibiotics? infections? trauma?
Main features JIA
- stiffness and limited range of movement more prominent than pain.
NB: growth disturbancies, contractures, eyes.
Systemic type (stills disease) - 2-7 % - high spiking fever - exanthema - hepatosplenomegalia - serositis - joint symptoms/arthritis + / - - girls = boys - all ages Diff diagn: septicemia, leucemia
Prognosis JIA
- moderate to severe ongoing disease in 40 – 60 - 80% ?
- a minority in long term remission «..burns out»
- oligo persistent: best prognosis
Oligo articular JIA
- 50 - 60 %
- ≤ 4 joints (first 6 months)
- large joints, asymmetrical
- general condition OK
- 2/3 persistent oligo
- 1/3 extend to 5 or more joints
Polyarticular JIA - 25 - 40 % - ≥ 5 joints (first 6 months) - +/- general symptoms - Subset: teenage girls anti-CCP/ RF-positive arthritis
Entesitis-related arthritis
- boys > girls
- age 10 - 16 years
- HLA B 27 positive
- Axial joints: hip, knees,
- sacro-iliacal, vertebral
ENTESITIS = inflamed entheses and tendon insertions
Juvenile psoriatic arthritis
- symmetric arthritis in small joints
- rash may start before or after the arthritis
- dactylitis
Uveitt
Chronic uveitis:
- asymptomatic!
- 15-20 % in all JIA
- risk factors: early onset, girls, ANA IF+
- ophtalmologic screening is essential
Acute uveitis:
- painful eye
- typically entesitis-related arthritis
(juvenile Mb Bekterew)
Eye screening
slit lamp examination x1-4 /year
complications:
- synechiae, cataract, glaucoma, corneal precipitations
- visus ↓
Treatment uveitis:
- local steroid eye drops
- systemic medication
Limping or restricted movement in joints:
always pathology!
Aims
Functional ability: quality of life Avoid sequela: - contractures - growth disturbancies - reduced vision
Systemic treatment
NSAID: Ibuprofen, naproxen, etc
methotrexate (folic acid antagonist)
biologic treatment incl anti-TNF-: Etanercept, infliximab, adalimumab, abatacept, tocilizumab etc
Corticosteroids: prednisolone
Others: hydroxychlorokin (antimalarial), sulfasalazin, etc.
Thumb rule: vaccinate!
- first dose live vaccines: avoid or be careful!
- booster MMR and VZV OK on individual consideration, should be given if methotrexate>15mg/m2, anti-TNF- or prednisolone >20 mg/d or >2mg/kg/d
Pediatric hematology and oncology
Diagnostic tools • Full blood count • MCV: - Microcytic: Iron-deficiency / Thalassemi - Normocytic: Mål bilirubin og haptoglobin for hemolyse. - Macrocytic: Folate- or B12 deficiency • MCH (Mean corpuscular hemoglobin): - Hypochromic - Normochromic - Hyperchromic • MCHC
• Iron metabolism:
- Iron: Reduced level doesn’t prove deficiency!
- Ferritin: Iron storage, acute phase protein!
- Transferrin: Iron transport
- Transferrin saturation
- TIBC: Total iron-binding capacity
- Hb-electrophoresis
Parameters of hemolysis • Bilirubin • Reticulocytes – BM-production • Haptoglobin – Hb-transport • LDH • Coombs-test: • Erythrocyte-antibodies? • Blood-/bonemarrow-smear
Iron deficiency anemia • Inadequate intake • Low birth weight • Malabsorption • Blood loss
Therapy:
• Treatment of underlying condition
• Oral substitution + dietary advice
In chronic inflammation/disease:
• Iron stored as ferritin can’t be mobilized -> not iron deficiency, but inaccessibility
Aplasia is a birth defect,
where an organ, or a tissue, is absent, or defective.
Aplastic anemia is the failure of the body to produce blood cells.
Anemia due to aplasia
- Low/absent reticulocytes!
Hereditary conditions – often part of syndromes:
• Isolated anemia: Diamond-Blackfan-anemia
• Pancytopenia: Fanconi’s anemia
Acquired conditions
• Transient erythroblastopenia of childhood (TEC)
- Autoimmun reaction to prior infection (often viral)?
- Spontaneous remission
• In children with chronic hematologic disease:
- Aplastic crises triggered by Parvovirus B 19-infektion
• Aplastic anemia due to infection, drugs – other cell lines involved
Hemolytic anemias
Cell membrane disorders
Hereditary spherocytosis • Mutation in gene for cytoskeletal protein - Uneven surface -> loss of membrane in capillaries - Spherocytes • Destruction in spleen • Hemolysis -> Bilirubin ↑ - Jaundice -> Gallstones - Splenomegaly • Varying anemia -> aplast. crisis - Transfusion? - Splenectomy?
Enzyme disorders
Glucose-6-P dehydrogen. Deficiency
• Susceptible to oxidants (lack of glutathione)
• Symptoms depend on level of enzyme activity
• Some drugs, chemicals and food worsen the deficiency
- Hemolysis within 24h
- Avoidance of the drugs
Hemolytic anemias
Hemoglobinopathies
Thalassemias
• Mutation of globin genes
- Number of affected chains determines severity (major[3α; 2β ]/minor[2α; 1β])
- Microcytic, target cells
- Hb-electrophoresis
- Severe forms: Regular transfusions -> hemosiderosis
- If untreated: Extramedullary hemopoiesis
Sickle cell disease • Mutation β-globulin gene - HbS-tetramer with α-chains - Deoxygenation -> Sickle shape • Destruction in spleen - Hemolytic anemia • Thrombosis -> Ischemia - Vaso-occlusive crisis (pain!) - Splenic sequestration crisis - Acute sickle chest syndrome - Analgesia, hydration
Bleeding disorders
Abnormal bleeding
Into soft tissues (muscles, joints): • Hemophilia A - Factor VIII deficiency • Hemophilia B - Faktor IX deficiency
• Mucocutaneusly, bruises, following surgery:
- von Willebrand’s disease
• Vitamin K deficiency:
- Preventive treatment of all newborns
Cancer in children
• Most common cause of death beyond 1 year of age, nevertheless uncommon condition (circa 140 new cases/year in Norway).
• > 85 % have typical symptoms
• More immature types of cancer than in adults.
- «blastoma» = cancer originating from embryonal tissue.
• Chemotherapy is crucial (and more effective than in adults).
• Often disseminated disease at diagnosis -> still curative approach.
• Not related to lifestyle factors.
Late effects:
• Encephalopathy due to irradiation.
• Cardio-, oto-, nephrotoxic side effects of chemotherapy.
• Infertility.
• Endocrine disorders (hypopituarism, hypothyreoidism, precocious/delayed puberty, impaired growth).
Secondary malignancies
Cancer in children - epidemiology
- ALL (28 %)
- Brain tumours (20%)
- Neuroblastoma (9 %)
Nephroblastoma (7 %) NHL (7 %) Hodgkin’s (7 %) AML (5 %) Rhabdomyosarcoma (5 %) Osteogenic sarcoma (3%) Ewing’s sarcoma (3 %) Germ cell tumours (3 %) Carcinomas (3 %) Hepatoblastoma (1 %) Other (4%)
Warning signs for pediatric cancer
- Lasting reduced general condition
- Enlarged lymph nodes
- Tumors
- Anemia, bleeding, infections
- Hepato-/splenomegaly
- Bone or joint pain
- Precocious puberty
- Exophtalmus
- Headache and nausea
Generelt ALL:
Generelt: sykdomsfølelse, anoreksi
Beinmargs-infiltrasjon:
- anemi –> blekhet, slapphet
- nøytropeni –> infeksjon
- trombocytopeni –> blåmerker, petekkier, neseblod
- beinsmerter
Retikulo-endotelial-funksjon:
- Hepatosplenomegali
- Lymfadenopati
Andre organ-infiltrasjoner
- CNS –> hodepine, oppkast, nerveparese
- Testikler –> forstørret testikler.
Approx. 35 children/year i Norway - most commonly 2-5 years of age.
Total length of treatment: 2,5 years
- Induction
- Consolidation I
- Delayed intensification I
- Consolidation II
- Maintenance I
- Maintenance II
Acute complications of leukemia
- Tumor lysis syndrome
- Hyperleukocytosis / leukostasis
Hjernesvulster
40% Astrocytoma !
20% Medulloblastoma
Neuroblastoma
• Derived from neural crest tissue
• Adrenal medulla and sympathetic nervous system
- Abdominal mass
- Spinal cord compression
- Typically bone and BM-metast.
• Spektrum from benign to highly malignant -> molecular biology!
• Young children
- If < 1 year often spont. regression and maturation.
• Tumour marker: VMA, HVA (vanillylmandelic acid (VMA) and homovanillic acid)
• Multimodal, intensive treatment for high-risk patients.
Lymphoma
• Hodgkin’s vs Non Hodgkin’s lymphoma
Most common types of NHL in children: • Mature B-cell NHL. • (i.e. Burkitt’s + diffuse large B-cell lymphoma). • Lymphoblastic lymphoma. • Anaplastic large cell lymphoma.
Molecular differences between adult and pediatric cancer
- Pediatric cancers have fewer mutations than adults cancer types.
- Thus fewer targets for targeted therapy available.
- 7–8% of the children with cancer carry an predisposing germline variant.
Acute diarrhea:
Causes: • infections • AB/drug associated surgical conditions: - appendicitis, intussusception, Mb. Hirschprung • symptomatic - extra-intestinal infections / fever conditions • allergic reactions and intolerance • vasculitis; Henoch Schønleins purpura
Infectious gastro-enteritis
• Very common condition!
- 1-3 episodes/ y first year in kindergarden
• Most frequently a benign self-limiting condition.
• Seldom in need of healthcare or hospitalization, but potentially life- threatening. 1-2 mill childhood-deaths /y
Viral GE
- Highly infectious
- Smaller or greater brake-outs
- Fecal-oral transmission
- Most common during first 2-4 years
- Recovery during few days
- Severity related to degree dehydration
- Rota virus
- Adeno virus
- Noro-virus
- GE
- Anemia
- Thrombocytopenia
- Elevated creatinine
Bacterial GE
• Food-poisoning. Toxin producing bacteria. Contaminated food/water.
• Staf. aureus, Clostridium perfringens
• Campylobacter, Salmonella, Shigella, Yersinia, Clostridium, E.Coli
• How to differentiate from viral GE?
- bloody stools (“dysenteria”)
- travel in high-endemic countries
- high and more long-lasting fever
- abdominal pain
- extra intestinal manifestations
- info about local outbreak
Treatment:
• Symptomatic
• Fluids!
Replace:
• Deficit (correction 4-8 h isotonic dehydration)
- mild-moderate 30-80 ml/kg; start 10-15 ml/kg/h
- severe >90 ml/kg; start 15-20 ml/kg rapid infusion(< 30 min), then 20 ml/kg/h
• consecutive losses (watery stool: 10 ml/kg, vomit 3-4 ml/kg)
• normal fluid intake (basal needs)
- 10 kg: 100 ml/kg
- +10-20 kg: 50 ml/kg
- +> 20 kg : 20 ml/kg
Rehydration fluids • OR : - Frequent small amounts - Non hypertonic fluids(ORS) Important with sufficient information and guidance of parents: - How to manage rehydration - Critical /warning signs/symptoms - How to evaluate response
• IV:
- Moderate-severe and severe dehydration; hospitalization, acute life-threatening condition.
- Slow rehydration to children with both hypo-and hypertonic dehydration (s-Na < 120 / > 150 mmol/l), correction max 10 mmol/ day; risk of life-threatening complications.
• NaCl 0,9 % (bare Na og Cl, mer hyperton, surere) Na+ 154 mmol Cl- 154 mmol. Osmolaritet: Ca. 290 mosmol/kg vann. pH ca. 5.
• Ringer-Acetat Na+ 130 mmol K+ 4 mmol Ca2+ 2 mmol Mg2+ 1 mmol Cl- 110 mmol acetat 30 mmol Osmolaritet: Ca. 277 mosmol/liter. pH 5-6.
Functional diarrhea is defined as the painless
passage of 3 or more large, unformed stools for 4
or more weeks, with onset in infancy or the preschool years (begins within 6 and 36 months of age),
and without failure to thrive or a specific definable cause.
Chronic diarrhea Mechanisms:
• Secretorical-:
- Secretion > absorption
- High amounts water and electrolytes
- Watery and high volume feces
- Classical toxin induced (Cholera/ entero-toxigenic E.Coli)
- No improvement when fasting
• Osmotic-:
- Malabsorption, osmotic gradient over the luminal wall, low pH and high osmolarity, improvement when no po
- carbohydrate malabsorption
- Fructose
- Sorbitol
- Lactulose
- Dysmotility
- Combinations; most frequent!
- Celiac disease
- Pancreas insufficiencies
- Bile-acid: Malabsorption / colestasis
- Inflammation/infectious GE/immune deficiency
- Drugs (axatives, AB, antacid)
- Chronic non-specific diarrhea/Toddler’s diarrhea/IBS
Allergy/intolerance?
• History!
Infants:
- Food-protein induced enteropathy/enterocolitis and/or proctocolitis
- GI symptoms +/- other allergy symptoms
• Specific IgE and skin prick-test; little importance
• Non-IgE mechanisms most common
• Elimination diet; followed by oral challenge-test; simultaneously record symptoms
Constipation
• prevalence: 3-5% (preschool age)
• 10 % of all referred to a specialist clinic (25% to a GE clinic)
”Vicious circle”
- Paradoxal contraction of anal sphincter
- Pseudodiarrhea, Encopresis
- Hard painful stool
- Painful defecation, Analfissur
90-95 % functional!
Must include one month of at least 2 of the following in infants up to 4 years of age
- 2 or fewer defecations a week
- At least one episode of fecal incontinence per week.
- History of painful or hard bowel movements.
- Presence of a large fecal mass in the rectum
- History of large diameter stools which may obstruct the toilet.
History: • Symptom start(meconium) • Symptoms/signs of organic disease or malformations • Growth • Diet
Clinical examination
• Somatic, including neurological status.
Perianal inspection and rectal exploration
Treatment • Counseling/education/reassurance! • Disimpaction; enemas • Maintenance therapy; Laxatives (e.g. lactulose, macrogol) - “High dose” - 3-6 months (non-painful stool: out of the “viscous circle”) • Diet interventions; - Sufficient fiber intake - 10% improve on milk-free diet • Surgery (ARM / Hirscprung)
