Pediatri Flashcards

Question

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.

Answer 1

.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.

Answer 2

``` 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

Answer 3

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

Answer 4

Throat infections Aetiology - Virus 90% - Streptococci (GAS) 10% Diagnostics - Strep A test - CRP, blood count? Treatment - Penicillin if streptococci

Answer 5

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)

Answer 6

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

Answer 7

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).

Answer 8

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.

Answer 9

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”. ```

Answer 10

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.

Answer 11

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)

Answer 12

``` 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)

Answer 13

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

Answer 14

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.

Answer 15

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.

Answer 16

Microbiology Bacteria that need to be covered: Gram positive: - Staf. aureus - Staf. epidermidis streptococcus Gram negative: - E. coli - Klebsiella - Pseudomonas - Candida albicans

Answer 17

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 ```

Answer 18

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 ```

Answer 19

Kochers kriteria: * Fever (>38,5) * inability to weight bear * SR > 40 * WBC > 12 * CRP > 1mg/dL • Effusion + 2 or more kriteria: --> joint aspiration

Answer 20

* Flat feet * Leg-length discrepancy = anisomieli * Scoliosis ``` Calcaneo-valgus = ankel innover --> plattfot Calcaneo-varus = ankel vender utover ``` (Rum opens you legs xD)

Answer 21

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

Answer 22

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.

Answer 23

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.

Answer 24

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.

Answer 25

”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

Answer 26

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

Answer 27

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.

Answer 28

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.

Answer 29

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 ```

Answer 30

Juvenile psoriatic arthritis - symmetric arthritis in small joints - rash may start before or after the arthritis - dactylitis

Answer 31

Eye screening slit lamp examination x1-4 /year complications: - synechiae, cataract, glaucoma, corneal precipitations - visus ↓ Treatment uveitis: - local steroid eye drops - systemic medication

Answer 32

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

Answer 33

``` 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

Answer 34

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

Answer 35

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

Answer 36

``` 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 ```

Answer 37

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

Answer 38

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

Answer 39

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.

Answer 40

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.

Answer 41

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

Answer 42

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

Answer 43

``` • 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. ```

Answer 44

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

Answer 45

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) ```

Answer 46

Visceral pain • Difficult to localize and describe. • Associated with higher levels of anxiety and vasovagal reactions compared to somatic pain.

Answer 47

Functional abdominal pain disorders: • Exclusion diagnoses • Peak; preschool/school start and teenagers • girls(5:3), 10-20% of all school aged children • Typically attacks with pain/recurrent pain, 50% < 1 hour, very rare > 3 hours • diffuse localization, but typically para-umbilical • No pain during night, but frequent before falling a sleep • Degree of pain; moderate-severe pain • Co-morbid symptoms frequent as headache, dizziness, nausea, fatigue and pallor

Answer 48

Probiotics? - IBS: significant less pain (frequency and degree pain) + less bowel symptoms - Other studies; somewhat less convicting results, but often more heterogeneous FAP subgroups Dietary fiber? - Possible IBS (bowel regulation)

Answer 49

”Failure to thrive (FTT)/ faltering weight”: • Poor physical growth (weight gain) in infants and young children - Wasting (weight loss) - Stunting (poor growth) • Repeated measurements: - Weight for age < 2 perc - Weight for height < 10 perc - Weight-chart crossing two percentiles - Combo poor weight gain/growth

Answer 50

Organic causes • Extra-intestinal diseases/disorders. Potentially; all chronic diseases that effect the energy balance. - Chronic infections - Immuno deficiencies - Cancer - Endocrinological diseases (hypotyreosis) - Cogenital heart failure - Renal failure - Lung diseases (severe asthma) - Systemic diseases (Cystic fibrosis, SLE, RA) - Neurological disabilities (CP) • Intestinal disorders - Celiac disease - Cystic fibrosis - IBD - Severe food allergy - Gastroesophagael relfux disease - Short gut snd

Answer 51

Celiac disease • Definition; permanent intolerance for prolamins/proteins that are found in cereal grains. • Wheat (gliadin), barley (hordein), rye (secalin). • Prevalence 3-4 /1000, more frequent in girls vs. boys. • More prevalent among children with other auto-immune diseases, siblings with CD, Down snd, Turner snd. ETIOLOGY: • Environmental factors (diet/infections) • Predisposing genetic factors: Tissue Ag HLA DQ2 or DQ8 pos-90%, NB! 20% healthy Norwegians are HLA DQ2 pos. Work-up 1. Clinical symptoms including treatment-response 2. Serologic (two methods, ex. TTG/Endomycium AB) Obs IgA deficiency 3. HLA DQ2/8 4. Diagnosis; combination clinical symptoms and biochemistry analyses incl. (serological and HLA), in addition to treatment response (ESPGHAN criteria) 5. Otherwise; Duodenal biopsy Follow-up • In doubt of diagnosis; challenge + new duodenal biopsy

Answer 52

Regurgitation (GER) • 4 months; 40 -70% • 12 months; 5 -10 % ``` GERD • < 1y: 1,5 - 5 % • < 5 y: < 1/1000- 8% • adolescents: 2-8 % • adults: 10-20 % ``` ``` ”Risk” child • CP (Cerebral Palsy/Parese); > 70% (makroscopic esophagitis) • Neurological disorders/disease • Down snd • CF/asthma ``` Regurgitation/vomiting • Acute and chronic • GER - “ happy spitters” • GER + reflux related symptoms = GERD

Answer 53

OBS: Secondary GERD: allergic disease to Eosinophilic Esophagitis Warning-signs and symptoms of Secondary GERD • Surgical conditions: Acute/subacute symptoms! - Vomiting/projectile - Hematemesis - Distended abdominal wall - Progressive and intermittent abdominal-pain symptoms - Fever • Allergic - Diarrhea/enterocolitis - Eczema - Family history - eosinophilic esophagitis • CNS - Macro/mikrocephaly - Hypotonia - abnormal development and neurological examination • Non-specific; illness, including infections - Nutritional and growth problems - Fever

Answer 54

Work-up; GERD (non acute) * Allergic disease (CMA/EE); * IGE/prick-test * Elimination diet; Ag provocation * Radiography (if signs of neurological disease/condition; MR/US) * pH monitoring (acid reflux) * EVD radiography (anatomical examination) * Endoscopy (esophagitis/EE/anatomical evaluation) * EGG/US (motility disorders) Treatment • Secondary GERD; treat underlying condition! • Infants with GER: Counseling - Reassurance - Diet (volume/consistence) - Obesity; weight loss • Acid related symptoms - antacida - PPI • Eosinophilic Esophagitis; PPI and diet intervention • Surgical (sever cases, risk-patients); fundoplication

Answer 55

Common weight gain: ``` 0-3 m 200 g /week 3-6 m 150 g / week 6-9 m 100 g / week 9-12 m 50-75 g / week 1-2 y 2-2.5 kg / y 2-5 y 2,0 kg / y ``` Recommendations routine measurements (Ministry of health) At birth: Weight, length, head circumference 2. - 4. days (hospital): Weight 7. -10. days: Weight, head circumference 6 weeks: 3 months: Weight, LENGTH, head circumference 5 months 6 months 10 months 12 months: Weight, length, head circumference 15-18 months: Weight, length 2 years 4 years

Answer 56

Kernicterus • UNconjugated bilirubin crosses the blood-/brain barriere • Neurotoxic – mainly in the basal ganglia Presentation • Jaundice within first day of life or rapidly increasing bilirubin levels: Suspect hemolysis due to immunization.

Answer 57

Rh-immunization Anti-D-prophylaxis • to all Rh NEGATIVE women who have given birth to a Rh POSITIVE child. • Substantial reduction in immunization. Suspect when: • Rh antibody titer >256 or significant increase in titer during the pregnancy (> 2x). ``` Umbilical cord sample from all Rh neg women: • Hb • Bilirubin • Direct Coombs (DAT) - Add anti-humane globulin ```

Answer 58

Physiologic jaundice ``` Bilirubin production ↑ • Breakdown of fetal erytrocytes - Shorter lifespan - High Hb at birth (14-24 g/dL) • Enterohepatic circulation ↑ - Low enteral intake first 2-3 days - Low amount of bacteria in the intestine ``` Conjugation in liver ↓ - Low activity of conjugating enzyme - 1% of adult activity at birth. Adult levels by 14 weeks - Low levels of bilirubin binding ligandin Physiologic jaundice - Normally a peak at 48-72 hours. - Later in preterm. - Plateau phase - Limited by uptake in liver and ligandi - Usually disappears in 4-7 days ``` Treatment • Oral hydration – breast milk • Phototherapy • Immunoglobuline • Exchange transfusion ```

Answer 59

Intravenous Immunoglubulin (IvIg) * Antibody mediated hemolysis * High dosis – 500mg/kg, eventual repeated after 12 h * Mechanism unknown * Reduced the number of exchange transusions substantially * Risk for late anemia (antibodie are not removed)

Answer 60

Perinatale infections * Transplacental * Ascending infection * Intrapartum * Postnatal

Answer 61

Congenital infections * Rubella * Cytomegalovirus * Toxoplasmosis * Parvovirus * Varicella * Syphilis * Herpes * Enterovirus * Hepatitis B/C

Answer 62

Cytomegalovirus • 70 % of Norwegian women IgG positive – 1-4% primary infected in pregnancy • 15% fetal death • Primary infection in pregnancy – 30-50% of the children infected Congenital (0,5-1,0 % of all newborn) - Asymptomatic (85-90%) .. but can develop deafness. - Symptomatic (10-15%): - FGR, jaundice, microcephali, intracerebral calcifications, retinitis, trombocytopenia, leukopenia, hepato-splenomegali - Mental retardation/ADHD? - Most important non-genetic cause of deafness Acquired - Most often asymptomatic at term. ``` Diagnosis of CMV • CMV in urine or saliva < 21 d = congenital infection - PCR • Blood samples - Quantitativ PCR: Best ! - Serology IgG and IgM mother (not baby) - Hematology/liver tests • Eye examination - Retinitis? • MRI/US - Early infections: Migration disturbances and malformations - Later infections: Affects white matter • Auditory test - Repeat!! • Lumbar punction (consider) ``` Treatment and follow up • Difficult, but symptomatic CMV: - Valganciklovir 6 months • 40-60% develop sequelae, most often hearing and cognitive deficits

Answer 63

Perinatal transmission • Risk of transmission If mother is HBsAg and HBeAg positive at least 70-90% of the children will be infected perinatally. NB: 90% of infected newborn babies will be chronic carriers.

Answer 64

2. Unknown HB-status (from risk regions...) * Serology from mother at admission. * Child HB vaccination within 12 hrs after birth and specific HBV-Immunoglobulin. • Later HB-vaccination - 4w - 3, 5 and 12 months • 70-95 % protection against HB

Answer 65

Toxoplasmosis • Toxoplasma gondii – protozoic parasite - Cat definitive host. • 11% of Norwegian women seropositive (1998). Higher in the south. • 1 : 1000–10.000 live born infected - 15-20 children in Norway, 2-4 sequela. • Most children asymptomatic – can develope chorioretinitis later. • Until 10% symptoms: - Hydrocephalus - Chorioretinitis - Intracerebral Calcifications ``` Other symptoms and clinical findings • Growth retardation • Microcephalia • Seizures • Feeding difficulties • Hypotermia ``` 50–90% with no symptoms

Answer 66

Group B streptococci (GBS) * In GI-tract/vagina in 15-40% pregnant women * 50 - 70% of the children colonized * 1-2% of the colonized infants develop infection/symptoms * 9 GBS serotypes (1a, III and V) * Early onset: Infected before or during birth - Early symptoms sepsis - pneumoni - Often dramatic • Late onset: Nosocomial, mother, breast milk - Less fulminant - Often meningitis • Possible to prevent early onset - Ampicillin/Penicillin before birth. GBS strategy in Norway • No consensus about screening. • Risk based strategy, but risk groups have been unclear/different defined. • Different views among professionals....

Answer 67

Transient tachypnoea ``` • RR > 60/min • General good condition • Unabsorbed lung fluid - Brief - hours - Declining - Normal chest x-ray - Exclude other causes ``` Wet (!) lung ``` • RR > 60/min respiratory problem - Retractions/grunting • Probably same conditition as TT • Chest X-ray shows INTERLOBAR FLUID ! • Not worse • Slowly improvement – hours-days • Exclude other causes ```

Answer 68

Lung hypoplasia * Primary * Reduced amniotic fluid - Kidney agenesi / dysplasi / Polycystic kidney disease - Prom • Space occupying process in chest - Diaphragmatic hernia - Other ``` Incidence • Varies between countries - 5 - 12% of all births. - Norway about 7% - Highest in USA ```

Answer 69

RDS ``` • Lack of surfactant • Mainly extremely premature (<28 w) • Increasing respiratory problems after birth • Worsening: - Increased RR - Retractions - Grunting - Cyanosis – need of oxygen - Chest x-ray: Diffusely reduced air, air bronchograms • Natural course - 3-5 days - Worsening - Culmination - Improvement ``` Treatment • Prophylaxis: Prenatal steroids • Treatment: - Surfactant - Gentle ventilation Ventilator/CPAP/High flow - Spontanous improvement • Milder course nowadays. Only a few dies in the acute phase.

Answer 70

Intraventicular hemorrhage * GERMINALE matrix * First 72 hours * Increasing risk in lower gestations IVH grade 1 - subependymal hemorrhage Prognosis • Grade 1 and 2 as other preterms with similar GA. • Grade 3 sequela in 50%. • Grad 4. Mortality 50-60%. 65-100% developes CP as spastic diplegia or quadriplegi. Periventriculær leukomalacia - Diffuse or focal ``` Causes • Unclear • Hypoxia - ischemia. Low BP – reduced systemic circulation. Hypocapnia • Pre-perinatal infection/inflammation ``` Prognosis • Cystic PVL strongly associated with later cerebral palsy! • Diffuse PVL associated with cognitive and behavioural problems?

Answer 71

• An epileptic seizure is caused by acute disturbances in the electric activity of the brain. • All people can experience an epileptic seizure! – Provoked by infections, head trauma, fever (child), drugs/alcohol, sleep deprivation etc. – ~ 10 % have one epileptic seizure attack during life. Highest prevalence in the first years of life – Cumulative risk of epilepsy first 20 years of life: 1% – Prevalence in childhood ~ 4-6/1000 • ↑ ↑ prevalence children with mental retardation and cerebral palsy. Pathophysiology: • Synchronized hyperactivation of brain cells. • Neuronal cells affected by neurotransmitters: – Excitatory synapses: Glutamate binds to NMDA- and AMPA-receptors. – Inhibitory synapses: GABA binds to GABA- receptors (α, β, γ subunits). Genetic epilepsies may be caused by mutations in genes that code for ion channels or their accessory subunits – Genetic panels (exome sequencing) Genetic epilepsies ≠ no obvious structural lesion/damage

Answer 72

Focal onset - Aware - Impaired awareness Generalized onset - Tonic-clonic Unknown onset Unclassified

Answer 73

Focal motor/autonomic seizures * Motor seizures, strange feelings/laughter/smile etc. * Duration often around 10-20 s. * Retained awareness/consciousness If altered awareness/consciousness • Often repeated movements/automatisms, longer duration

Answer 74

Neonatal seizures Incidence 2-9/1000 live born infants * Secondary to transient disturbances in oxygenation, cerebral blood flow, metabolism and/or infections. * Subclinical seizures are common; only possible to detect with continuous EEG/aEEG (amplitude integrated). * Associated with high mortality and morbidity! • Increased risk of neurological sequela and post-neonatal epilepsy

Answer 75

Infantile spasms Diagnostic work up and treatment • Typical EEG = Hypsarrhythmia – Chaotic, non-rhythmic, asynchronous, disorganized, high-voltage spike and slow-wave activity • MRI – Malformations/injuries? • Treatment – High-doses steroids and/or vigabatrin; side effects! – Other antiepileptic drugs or surgery Outcome • Only 5-10 % achieve normal or almost normal intelligence, the other become moderate to severe mentally retarded. • 60-90 % have persistent seizures and often develop other severe seizures types. • Associated with a favourable prognosis: 1. Normal development at onset 2. Unknown cause 3. Rapid response to treatment

Answer 76

Febrile seizures Can it be meningitis? Yes, but extremely rare (0-7 %) • Meningitis is very unlikely if no petechial, no neck stiffness and a simple seizure attack. • If < 12 months; often admitted, always consider LP. • Common (although often not necessary) to admit a child after first febrile seizure attack. Management • Antipyretics may improve the comfort of the child, but they will not prevent febrile seizures. • Always prescribe DIAZEPAM if needed later – Administer if seizure duration > 4-5 min • Information to the parents + empathy! – Very important – What to do during a seizure? – No deaths reported during/after a simple seizure

Answer 77

Absence epilepsy Childhood and juvenile type • Childhood type; typical onset 5-7 years – Very frequent, typical absence seizures lasting 2-20 sec – EEG: rhythmic 3 Hz generalised spike and wave complexes. – Childhood absences disappear before adulthood in up to 90% • Juvenile type; typical onset 10-12 years (girls > boys) – Frequent absences (daydreaming??) – May cluster upon awakening – Generalize tonic-clonic seizures may develop (++ by sleep deprivation) and long-term prognosis is unclear. – Associated with cognitive dysfunction and learning difficulties.

Answer 78

EEG * EEG, usually some weeks after the acute seizure (↑ prognostic value). * A routine interictal EEG has a sensitivity of 50-60 %. * Repeated EEG-examinations increase the sensitivity to 80-90 %. * Sleep deprived EEG. * Flicker stimulation. * 24 h EEG / videometry. Neuroimaging • MRI – Procedure of choice !! – Special epilepsy protocols • CT: Rarely used, not detailed enough for diagnosis. • Functional neuroimaging – In candidates for epilepsy surgery

Answer 79

Factors associated with a favourable prognosis ``` – Normal intelligence – No neurological abnormalities – Unknown cause (”idiopathic”) – Age at onset more than 2 years – Infrequent seizures – Limited numbers of “GTK” seizures – Only one seizure type – Absence of tonic-atonic seizures (drop-attacks) – Rapid response to anti-epileptic drugs (AED) ```

Answer 80

Breath-holding spells In ~ 4-5% of all children (F = M), most common between 6-18 months. • Cyanotic breath-holding spells – Usually precipitated by anger or frustration (or pain). – Child cries and has forced expiration sometimes leading to cyanosis, loss of muscle tone, and loss of consciousness. • Pallid breath-holding spells – Common stimulus a painful event. – Turns pale, loses consciousness with little if any crying. • Clinical diagnosis – Good history including the sequence of events, lack of incontinence and no post ictal phase. – Some have iron deficiency.

Answer 81

Prevention and treatment: 1. Avoid precipitating factors – Sleep deprivation, psychological stress etc. 2. Anti-epileptic-drugs (AED) – Choice depend on seizure type – Monitoring drug concentration – Slow release tablets; stable drug concentration 3. Vagus nerve stimulation (VNS) - Used as adjunctive treatment in pharmaco-resistant epilepsy. - Vagus nerve stimulation may reduce seizure frequency. - Mechanism of action not fully understood. 4. Surgical treatment 5. Ketogenic diet • Nutrition predominantly rich in fat and maintains ketosis in the long term. • High concentrations of ketone bodies have been correlated with better seizure control. – Mechanism of action not entirely known, but ketone bodies increase degradation of glutamate and increase the conversion of glutamine to inhibitory GABA. • 20–40%: > 90% reduction in seizure frequency. – Another 20–60%: > 50% reduction in seizure frequency.

Answer 82

AED in different seizure types • First choice Focal seizures: – Carbamazepine (Trimonil, Tegretol) – Oxcarbazepine (Trileptal) • First choice Generalised seizures: – Valproate (Orfiril) – Lamotrigine (Lamictal) • First choice SE – Benzodiazepines (Diazepam/Midazolam) – Phosphenytoin (Pro-Epanutin)

Answer 83

Epilepsy and school • Many children with epilepsy have normal IQ • May be problems with learning, cognition and memory – Subclinical seizures? – Side effects of drugs? – Absence from school? • The teacher needs to have knowledge about epilepsy – Refer to pedagogic centre if needed. – Know how to treat a seizure attack. Comorbidities • Learning problems/struggling at school – In up to 70% of children with epilepsy • ADHD • Behavioral problems • Psychiatric problems Of great importance to detect and ameliorate/ treat/help children with these problems.

Answer 84

Symptoms: • Headache usually pounding or throbbing, lasts between 1-2 hours, and may involve one or both sides of the head or the entire head. • Headache often accompanied by severe nausea and vomiting. • Sensitivity to light and noise; the child often wants to lay down in a dark, cool room. • Often pain relief after sleep. • If possible; avoid triggers – Stress, sleep deprivation, hunger etc. • Abortive treatment during migraine attack – Young children: Ibuprofen, paracetamol and in some cases anti-nausea medications. – Older children: same as above + sometimes a triptan given by nasal spray. • Preventive treatments: – Consider if > 3-4 attacks each month – Not used frequently

Answer 85

Orofacial cleft • Anatomy – Lack of tissue and disfigurement • Embryology – 5-7 w: Development of jaw and lip – 7-11w: Hard and soft Palate • Incidence – 2 : 1000 – Boys: Lip and Jaw – Girls: Palate, part of syndromes

Answer 86

Hypospadias * 1-3 : 1000 boys, 100/year in Norway * Ca. 80 – 90 % distal * Most may be operated in 6 – 18 months of age. * ”One stage” vs ”staged procedure” (200 – 300 methods) • 3 components in this congenital defect: – Meatus lies ventral/proximal – Ventral curvature – Lack of ventral foreskin, plenty dorsally • Correction of ventral curvature (chordee) – Degloving – straight penis in 80% – Release the urethral plate – another 15% • Reconstruct the Urine tube • Reconstruction of the ventral side of the penis: – Meatus, glans, spongiosum, skin, foreskin TIP – Tubularized Incised Plate (Snodgrass) • Create tube of urethral- plate • Dorsal incisjon i urethral-plate • May be combined with corr. of foreskin • May be used both on proximal and distal hypospadies ``` Aim • Urethra ending at the tip of glans penis • Normal urine flow • Straight penis (erection) and normal sensitivity • Main complications: – Poor cosmetics – Fistulas – Stricture in neo-urethra – Dilatation (cele) of neo-urethra ```

Answer 87

Prominent ears in 5 %

Answer 88

Treatment of vascular tumors • Nothing! (90 %) * Debride superficial necrosis or ulcerations * Corticosteroids (local/systemic) * Propranolol * Laser (only superficial) • Excision of maformations in danger zones – Periorbital/oral – Gastrointestinal – Social coping ``` Treatment of vascular malformations • Capillary MF – Pulse-dye laser – Surgical contour corrections • Lymphatic MF – Sclerotherapy – Surgery ``` ``` • Venous malfomations – Most common – Sclerotherapy – Surgical resection • AVM – embolization ```

Answer 89

Congenital abnormalities of the Breast • Poland syndrom – Absence of breast, nipple, pectoralis major – 1 : 7000–10 000 – 3 x male : female

Answer 90

Electrical injuries • Low voltage > 220 V • High voltage • More than meets the eye – subcutaneous damage Classification of burn injury by depth ``` Partial thickness burns • 1st degree burn - Damaged epidermis and edema • 2nd degree burn - Damaged epidermis and dermis ``` Full thickness burns - 3rd degree burn - Deep tissue damage

Answer 91

Meldeplikt Alle som arbeider i offentlige instanser og tjenester eller som utfører arbeid for det offentlige, har meldeplikt til barneverntjenesten. Meldeplikten vil si at du er pålagt å varsle barneverntjenesten dersom du blir kjent med forhold du tror vil kunne skade et barns/en ungdoms helse eller utvikling. Meldeplikten gjelder selv om du har taushetsplikt. En offentlig melder kan ikke levere anonym melding til barneverntjenesten. ``` Lover hvor meldeplikten er nevnt • Barnevernloven § 6-4 • Barnehageloven § 22 • Helsepersonelloven § 33 • Opplæringsloven § 15-3 • Sosialtjenesteloven § 8-8a ``` Til politiet: Opplysningsrett § 23 (helsepersonelloven) Opplysningsplikt § 31 (helsepersonelloven)

Answer 92

Straffeloven-Avvergeplikt • Straffelovens § 196 pålegger under trussel om fengselsstraff i inntil ett år en plikt til å søke avverget visse grovere forbrytelser ved anmeldelse til vedkommende myndighet. Eksempelvis gjelder det for terrorisme, drap, ran, familievold, seksuelle overgrep - særlig mot barn, kidnapping og grovere tilfeller av vold. Avvergingsplikten er overholdt ved at man inngir melding til rette myndighet - normalt politiet - og krever normalt ikke at noen selv fysisk griper inn med mindre dette kan skje på en enkel og ufarlig måte.

Answer 93

Hvordan? * Skjema på kommunene hjemmeside * Skal sendes i post, ikke email!

Answer 94

Gravid rusmisbruker? § 32.Opplysninger til den kommunale helse- og omsorgstjenesten (sosialtjenesten). Uten hinder av taushetsplikt etter § 21 skal helsepersonell av eget tiltak gi opplysninger til den kommunale helse- og omsorgstjenesten, når det er grunn til å tro at en gravid kvinne misbruker rusmidler på en slik måte at det er overveiende sannsynlig at barnet vil bli født med skade, jf. helse- og omsorgstjenesteloven § 10-3. Også etter pålegg fra de organer som er ansvarlige for gjennomføringen av den kommunale helse- og omsorgstjenesteloven, skal helsepersonell gi slike opplysninger.

Answer 95

Drøfte med barnevern eller politi * Hvis du er usikker på om din bekymringer er alvorlig nok, kan du drøfte saken anonymt med barneverntjenesten * En drøfting er ikke å anse som bekymringsmelding

Answer 96

Forbundet med straffeansvar med fengsel inntil 1 år å la være å melde. TAKE HOME MESSAGE • Skille mellom barnevernets og politiets jobb og din jobb. • Ikke vær redd for å melde • Vær redd for å overse et meldingsbehov • Vår plikt som leger er å melde fra om bekymring, ikke å gjøre selvstendige vurderinger om barnet får god nok omsorg/utsettes for vold.

Answer 97

* 5% of Norwegian children experience severe physical violence from their caretakers. * 20% of girls and 8% of boys are sexually abused. * 25% have care takers with substance abuse or psychiatric disease which is challenging in daily life. * Substance abuse (8%m, 3% f inc) * Depression (10% inc) * Suicide (11/100 000) * Heart disease (21% prev) * COPD (6% prev) * Cancer (1/3 prev) * Diabetes (5% prev) * IBD * Fibromyalgia * Reumathic disease (RA) * Obesity * STD 60% of people on disability benefits from the welfare system (uføre fra NAV) have diagnoses associated with • Musculo/sceletal system • Psyciatric disease.

Answer 98

Allostatic load = the long-term effect of the physiologic response to stress. – “Allostasis — the ability to achieve stability through change — is critical to survival. Through allostasis, the autonomic nervous system, the hypothalamic–pituitary–adrenal (HPA) axis, and the cardiovascular, metabolic, and immune systems protect the body by responding to internal and external stress. The price of this accommodation to stress can be allostatic load, which is the wear and tear that results from chronic overactivity or underactivity of allostatic systems.” Examples of allostatic load may be found in the: 1. primary mediators (hypercortisolemia, increased inflammatory cytokines), 2. secondary outcomes (elevated blood pressure, overweight, insulin resistance), or 3. tertiary outcomes (hypertension, diabetes, obesity, coronary heart disease, neurodegenerative disorders).

Answer 99

Implications for society * Our disposition for health and unhealth is not «fair». * Health is not just a product of how we ‘choose’ to live our lives. * Disease is not evenly distributed. • We could remedy this by giving most to those that need it most, but: – Medical and social resources are not evenly distributed. – The inverse care law: ”The availability of good medical care tends to vary inversely with the need for it in the population served. This inverse care law operates more completely where medical care is most exposed to market forces, and less so where such exposure is reduced.”

Answer 100

Take home message (for your life as a doctor) • ACEs are real, and will affect many of your patients. • Listen to your patient’s story – and be prepared to receive it when the patient is ready to tell you. • Don’t be afraid to ask. • Life stressors will affect people (and families) differently – never assume you know the baggage the person in front of you carries with them. • Train yourself in how to evaluate interaction between child and caretaker. • Contact child protective service when suspecting abuse or neglect. – If in doubt: – contact the child protective service and discuss the case anonymously. – Rather one time too many!

Answer 101

CP Aetiology – 1 Prematurity • Periventricular leukomalacia (PVL) • Intracranial haemorrhage (ICH), usually grade 3-4 – What caused the premature birth? • 5-10% of infants with BW < 1.5 kg develop CP – Higher risk if complications Severe ICH Grade 3 and 4 • Grade 3 – At least 10-15% develop severe problems – A higher number if persistent dilatation of the ventricles • Grade 4 – 50-60 % CP – Also cognitive problems

Answer 102

Aetiology – 4 Multiple perinatal risk factors? Exponential increase due to multiple factors? – Especially preterm infants – Term babies; only 1/3 have two or more factors Individual/genetic susceptibility? Unknown cause: 30-50% term babies with CP • Occult infection/inflammation? • Vanishing twin syndrome? ``` Aetiology – 5 Postnatal (< 2 years); 5% of all CP cases • Sequela after – Severe epilepsy – Meningitis/encephalitis – Injuries/drowning – Metabolic crisis ```

Answer 103

CP - early clinical signs ``` • Often detected during follow up of ”high risk babies” – Feeding difficulties – Poor or ”too good” head control – Abnormal pattern of movement – Fisting of one hand – Early favouring of one hand – “Limp” when starts to walk ``` ``` Change in muscle tone? • Scissoring of the legs after 2 months of age – Increased hip adduction • Extension of the legs • ”Good” head control in prone position – Extension of spine and neck ``` Difficult to diagnose < 6 months of age – Risk factors! Usually obvious by 12-18 months of age – Poor quality/delayed motor milestones – Asymmetrical pattern of movement – Muscular hypertonia (NB hypotonia early) – Persisting primitive reflexes and increased deep tendon reflexes

Answer 104

CP- classification 1. Motor type? – Spastic: 70-80% Most common! Muscular appear stiff and tight. Arises from motor cortex damage. – Dyskinetic (6%): Dystonic or Choreoathetotic Involunatay movements. Arises from basal ganglia damage. – Ataxic – Mixed 2. Parts of the body involved? – Unilateral or bilateral – How many limbs affected 3. Gross motor skills? – Difficult to classify < 2-3 years of age

Answer 105

Spasticity «Velocity dependent resistance to stretch” - Lack of inhibition results in excessive contraction of the muscles. - Occurs mainly in disorders of the CNS affecting the upper motor neuron.

Answer 106

Bilateral spastic CP (Spastic diplegia) • Spasticity both legs – Hypotonia 1st year – Upper extremities also involved, to a lesser degree • Often ex-premature babies! • Cognitive function often good • Perception, visual motor coordination often difficult • Severely handicapped children • Microcephaly, mental retardation, epilepsy, respiratory problems, feeding difficulties, visual problems • Causes – Factors early in pregnancy – Hypoxic ischaemic encephalopathy II-III

Answer 107

Complications – scoliosis – hip luxation Treatment • Permanent brain damage - no curative treatment • Goal: – Best possible cognitive/motor function – Improve level of functioning, daily living – Reduce spasticity Botox - Purified botulinum toxin protein 1. Injection directly in to the affected muscles. 2. Two-thirds of the patients receive doses in their legs 3. Inhibits the release of acetylcholine from nerve cells, blocking the signals that promote involuntary muscle contractions 4. Symptomatic treatment Intrathecal Baclofen • Indications: bilateral spastic, quadriplegia • Indications: severe spasticity, pain, difficult handling • NB complications! Orthopaedic surgery • Indications fixed contractions or joints in wrong position • Mainly older children • Elongation/transposition of muscle/tendon

Answer 108

Prematures and neonates: • More body water and less fat than older children • Liver and kidneys: Immature • Deviation from normal organ function – The younger and the more premature, the bigger deviation

Answer 109

Elimination • Renal function: Increases gradually the first 6 months • Liver function: Increases rapidly the first 4 weeks • Reduced elimination: → Increased half-life → Longer time until Steady State is obtained → Longer time until the drug is eliminated from the body Liver: • Enzymatic activity undeveloped at birth • Microsomal enzymes involved in oxidation and glucuronide conjugation may be missing or be present in very low amounts → Bilirubin and drugs like chlorpromazine are metabolized very slowly. • Rapid development towards adult levels (1-8 weeks after birth). Kidneys: Variation in clearance and maintenance dosage with age. Maintenance dose per kg is: • Higher for children • Lower for newborn and elderly in comparison to adults

Answer 110

• Paradoxical effect of certain CNS drugs Diazepam, phenobarbital - Insomnia, restlessness, irritability Increased effect: – Opioids can give respiratory depression even in moderate doses. In general, little is known about differences in pharmacodynamics between children and adults. ``` Children and drug posioning • Most common at the age 1-3 • Most serious poisonings: – Iron tablets – Salicylate – Sedatives ``` Commonly used medications for children • Penicillin and erythromycin – (Avoid tetracycline!) • Paracetamol / acetaminophen – (Caution with acetylsalicylate for children under the age of 12!) • Desmopressin (nasal spray for treatment of nocturnal enuresis).

Answer 111

Children do Not tolerate pain better than adults! Children’s tolerance to pain increase with age. Challenging to identify the presence and severity of pain in children. Pain during procedures and surgery, postoperative pain, and disease-related pain are inadequately controlled. ``` Physiological Indications of Acute Pain • Dilated pupils • Increased perspiration • Increased rate/ force of heart rate • Increased rate/depth of respirations • Increased blood pressure • Decreased urine output • Decreased peristalsis of GI tract ```

Answer 112

Procedural anxiety • Midazolam po, nasal or rectal (0,3-0,5 mg/kg, max 15 (-20 mg). – 10-30 min before procedure – Antidot: Flumazenile 3-5 microgr/kg iv • Dexmedetomidin (1-2 μg/kg nasal) • Nitrous oxide (“laughing or happy gas”) 50/50 N2O and O2

Answer 113

UTI – common in children • Cumulative incidence of UTI in children up to 16 years – Boys 3-4 % – Girls 11 % • Boys: – Often first UTI during first 6-12 months of life. Underlying abnormality more common than in girls. • Girls: – Stable incidence of UTI during the first 6 years of life, but gradually more non- febrile UTIs and less febrile UTIs. – Some older girls (> 2 years) have recurrent UTIs.

Answer 114

Urine collection bags (clean the skin first) – Reliable if negative! – Best: 2 samples before start of antibiotics – NB. At least 10 % of healthy children will have growth of bacteria in collection bags!!

Answer 115

Nitrite is normally not present in urine, but may be produced by Gram neg. bacteria – Nitrate → nitrite * Gram pos. bacteria do NOT produce nitrite * Nitrite-test: 40-50 % sensitivity for UTI in children less than 2 years of age. A combination of leukocyte esterase and nitrite test has a fairly good sensitivity and specificity for UTI • NB: – Isolated haematuria and/or proteinuria are not signs of UTI.

Answer 116

Urine culture • Urine should be sent cold to the laboratory or kept in the refrigerator before transport. – Culture within 4 hours of collection. • Growth of more than one bacterial species indicates contamination of the urine sample. • Bacteriology: – E. coli (80-90 %) – Non-E. coli bacteria: • S. saprophyticus • Proteus (UTI predisposes for nephrolithiasis) • Klebsiella (bladder dysfunction?) • Enterobacter • Enterococci • Pseudomonas (bladder dysfunction? malformation?)

Answer 117

Follow up? • All children < 3 years of age – Primarily ultrasound, timing based on presentation – Further follow up restricted to ”high risk group” • Children > 3 år with recurrent UTI’s – Voiding pattern? (history) – Urodynamic (flowmetry/bladder scanning)

Answer 118

Renal diseases Nephritic syndrome – Haematuria and proteinuria – Often hypertension – Reduced GFR (↑ creatinine) NephrOtic syndrome – Proteinuria – Oedema (often periorbital) – ↓ albumin (< 25 g/l) ``` Nephrotic proteinuria: Urine dipstick ≥3+ protein creatinine ratio > 300 mg/mmol Proteinuria > 3 g/d or protein ```

Answer 119

Nephrotic syndrome (NS) Child • 80 % Minimal change glomerulonephritis (MCGN) – Usually small boys (less than 8-10 years of age) – ”Pure” NS, often no haematuria and normal GFR. – Responds to steroid (90 %) – Often relapses, but ”everyone” eventually become healthy Electronemicroscopy – Fusion of foot processes – Often selecetive glomerular proteinuria • Low molecular weight, mainly albumin • 20 % Light microscopic changes (biopsy) – NS + often also nephritis-like picture – Often resistant to steroids – More prevalent in older girls

Answer 120

Isolated microscopic haematuria • May be a glomerular disease: – IgA nephritis, thin GBM nephropathy, sequela after a mild GN, Alport syndrome – Very rare urological disease • Specific diagnosis has no therapeutic consequence, thus renal biopsy not indicated. • Follow up: – Urine dipstick and BP annually. – Renal biopsy if concomitant/development of proteinuria

Answer 121

Congenital heart defects (CHD) * In ~ 1 % of all newborn infants; the most common type of major birth defect! * Most (~ 70 %) serious CHDs are detected within the first month of life. * A heart murmur, the hallmark of a CHD, is not always audible on the first 1-2 days of life, and may not be present at all ! * Most infants with CHD are asymptomatic at birth, but those who need treatment will develop symptoms.

Answer 122

Epidemiology and risk factors: ``` Chromosomal anomalies (5-20%) - Syndromes (Down, Di George, Noonan, Williams, etc.) ``` ”External” influence (2-4 %) - Virus, drugs, alcohol (FAS) No obvious cause (70-90%) - “Gene-environment interaction” - New genetic tools... Pathophysiology: • ↑ pulmonary blood flow – e.g VSD • ↓ pulmonary blood flow – e.g pulmonary stenosis, Fallot • ↓ systemic blood flow – e.g. critical aortic stenosis, HLHS • Parallel coupling of pulmonary and systemic circulation – Transposition of great arteries

Answer 123

CHD – ”Classification” • Mild, no symptoms – e.g. Small VSD • Surgical ”repair” – ”Functional” biventricular heart • Palliative surgery – Univentricular heart etc. • Lethal

Answer 124

Screening for CHD • Prenatal ultrasound: – Variable detection rate (30-70%) • Pulse oximetry screening: – ~10-20 % of all CHD are duct-dependent critical/life-threatening conditions – Most of these have low SpO2 – Introduced in Troms in 2009 • Newborn examination 2-3. day of life – Clinical examination – Murmur?

Answer 125

Cleft lip and palate ~ 1.5-2 per 1000 live born • 55-70% non-syndromic, the rest associated with anomalies/syndromes Multi-disciplinary management 1. Feeding problems! 2. Hearing and speech problems 3. Operation of the lip and the maxilla at 3 months of age 4. Reconstruction of the palate - usually at 1 year of age Special feeding bottle

Answer 126

What is normal in the newborn baby? Normal vomiting - Vomits amniotic fluid after delivery. - Vomits right after feeding (milk): * When burping * After large volume feeds (hungry!!) * Due to mild reflux (physiologic) Normal stool - Meconium: The earliest stool of an infant. Black, viscous/ sticky like tar, and has no odor (”barnebek”). - Later when baby gets mothers own milk the stool is soft, yellow, smells a bit like mustard (”morsmelk-bæsj”). Up to 10-14 days between each time they pass stool may be normal.

Answer 127

Intestinal obstruction • Atresia/stenosis – Oesophagus, duodenum, small bowel and rectum/anus. • Intestinal malrotation – incomplete duodenal obstruction (± volvulus) • Hirschsprung’s disease • Pyloric stenosis – Symptoms 2-8 weeks of age • Intraluminal obstruction – Meconium ileus (CF?)

Answer 128

Congenital Anomalies of the Kidney and the Urinary Tract (CAKUT) • HYDRONEPHROSIS; the most common congenital condition detected by prenatal ULTRASOUND. – Mild degrees; uncertain clinical importance. Transient dilatation of the upper urinary tract/renal pelvis is common in mid trimester. • Abnormalities more common in boys. Specific problems - Multicystic dysplastic kidney - Unilateral renal agenesis (1: 1000-1500) ``` Unspecific problems “Dilatation of renal pelvis”: - Normal/transient - Stenosis in pyeloureteric junction - Stenosis in vesicoureteric junction - Vesicoureteric reflux ``` Bilateral problems - Oligo-hydramnios? * 90% of amniotic fluid is fetal urine - Bilateral renal agenesis (Potter syndrome) - Posterior urethral valves (PUV) * May damage the kidneys

Answer 129

Hypospadia - Urethral meatus abnormally placed Epispadia Abnormal development of cloacal membrane and incomplete midline fusio.n (epispadia; a milder degree of bladder extrophy) Much more complicated than hypospadia! Bladder extrophy Bladder opens onto the abdominal wall, marked separation of pubic bones and epispadia of the genitalia

Answer 130

Disorders of sex development (DSD) • An extremely distressing situation for the parents when the sex of their child is not easily determined – A delicate task to meet the family correct • Gender assignment should be made after thorough investigation. Never guess in front of parents! • When talking to parents initially say ”your child”. – Never say ”he” or ”she” until gender assignment is clarified • Consider referral to specialized multidisciplinary team – Norway: Bergen or Oslo.

Answer 131

Myelomeningocele - MMC • Prenatal diagnosis. – Large defects are diagnosed prenatally. • Often hydrocephalus and a Arnold Chiari malformation (= downward displacement of the cerebellar tonsils through foramen magnum). • Variable sensory and motor impairment. • “Always” problems with bladder and bowel control – Neurogenic bladder • Orthopaedic problems (scoliosis, hip dysplasia)

Answer 132

Hydrocephalus Clinical presentation - Large head circumference (HC) > 97.5 p (> 2SD) - Anterior fontanel is large or bulging - Cranial sutures are widely separated - Initially vague clinical symptoms - HC grows fast - Intracranial pressure will gradually increase and cause failure to thrive and vomiting Microcephaly (HC < 2 SD) – Associated with poor neurodevelopmental outcome – Zika virus infection

Answer 133

Talipes (Pes equinovarus - club foot) • 1 out of 1000 newborn infants • Boys > girls (2 : 1) – Inversion at subtalar joint – Adduction at talonavicular joint – Equinus at ankle joint; plantar-flexion position, making the foot tend towards toe walking • Treatment should start relatively early – Stretching – Serial above-knee plaster casts changed on a weekly basis – Achilles tenotomy and other operations often necessary

Answer 134

Norway tops the european rates regarding inactivity levels. * 24 % of 16-24 year-olds have ”screentime” > 7h/day. * 50 % of all norwegian children walk or bike to school. * 6-10 % aged 7-10 has motorical problems. * 80 % of adolescents worldwide does not meet the guidelines of recommended PA. Age between 6-10 is the most vulnerable period for developing obesity due to increased fat storage. -> Tracking

Answer 135

Limit “Screen Time” • The Norwegian Directorate of Health recommends less than 2 hours of media time per day. - Television - Computer - Movies/DVDs - Video games • Turn commercial breaks into activity breaks • Turn off the television during mealtimes • Do not use screen time as a reward or punishment

Answer 136

Sindig-Larsen-Johansons syndrome * Similar pathogenesis as OSD * Caused by repetetive tractions from the patellar tendon to the apex patella. Symptoms and signs: • Pain in the patella • Pain when using active knee extensions (jump, running) Treatment: - Conservative; modify training. - Full recovery within 10-24 months.

Answer 137

Anklesprain • Ottawa Ankle Rules - Test to decide who needs X-ray after injury. • Sensitivity 96-99% Treatment: - RICE (rest, ice, compression, and elevation) - Taping - Nevromuscular training

Answer 138

Symptoms: - Lower back pain Diagnostics • One-legged hyperextension test • MRI Spondylolisthesis • Defined as forward translation of one vertebral segment over the one beneath it. • approximately 15 % of individuals with a pars interarticularis lesion have progression to spondylolisthesis. • most common at L5-S1 (90%) in pediatric population. • Grade I-IV.

Answer 139

Description of rash • Types of rash – Macula, papula, vesicula, pustula, cruste, – Petechiae/purpura * Size of primary elements * Color and color intensity * Confluent? Single elements? * Localization

Answer 140

Trombocytopenia in children Petechia / nose bleeding when trc < 10 Bleeding in the skin. Does not disappear on pressure Vasculitis caused by virus. Difficult differential diagnosis to meningococcal sepsis. Does not disappear on pressure Henoch-Schønleins purpura - ITP - Leucemia - Aplastic anaemia - Treatment with cytostatic drugs - Sepsis / DIC - HUS

Answer 141

Endocrine regulation of growth - Fetal growth: mainly nutrition and insulin. - Infancy growth: nutrition and thyroid hormone. - Childhood growth: growth hormone (GH) and thyroid hormone. - Puberty growth: testosterone or estrogen. Growth hormone - Predominant regulator of childhood growth. - GH secretion is pulsatile, mostly during night. - Stimulates IGF-1 production in liver, and binds to IGFBP3 + ALS (Acid labile subunit) in the circulation. - IGF-1 has the most effect on linear growth, although GH receptors in the epiphyses also has some effect on growth. - GH stimulates lipolysis /inhibits lipogenesis. - GH increases amino acid uptake in muscle.

Answer 142

Low in weight and height ”failure to thrive” Evaluate caloric intake Differential diagnoses - Undernutrition: Vit. D deficiency - rickets - Caloric losses: IBD, celiac disease, cystic fibrosis, diabetes etc. - Excess caloric needs: Cardiac disease, hyperthyroidism

Answer 143

Medical evaluation in primary care ``` Clinical exam incl pubertal stage Blood samples - Hb, SR, electrolytes, kreat - Tissue transglutaminase, folate, ferritin, Calcium, - ALP, vitamin D - FT4, TSH ``` In hospital - IGF-1, IGFBP3 - Chromosomes in girls - Bone age - Other genetic tests - Total body X-ray if bone dysplasia is suspected - GH secretion tests - Confirmation of chronic illness

Answer 144

Marfan syndrome - Autosomal dominant connective tissue disease - Tall, increased armspan, hyper extensible joints, dislocation of the lens, aortic root dilatation.

Answer 145

Endocrinology of puberty LH/FSH peak in pregnancy week 20-24. FSH/LH peak about 8-10 weeks after birth – ”mini puberty” Quiescence in childhood due to active suppression First sign is increased GnRH signalling and low but measurable levels of FSH/LH - Genetics - Androgens - Body mass/ leptin

Answer 146

Timing of puberty Girls usually 1-2 years ahead of the boys. First sign (90 % B2, 10 % P2) 9-13 år. Menarche 13.3 år. Average of 2 years from Tanner 2 to menarche Boys: Testis > 4 ml 11-15 år

Answer 147

Physical changes in boys Testicular enlargement, TV > 4 ml or long axis length > 2.5 cm Pubarche normally within next year. Penile/scrotal growth. Transition to adult voice around age 14. Facial hair around age 15.

Answer 148

Evaluation delayed puberty ``` History and clinical exam Bone age FSH/LH, prolaktin, FT4/TSH, hematology, SR, tissue transglutaminase, chromosome. Ultrasound in girls. LHRH test. MRI brain. ``` Treatment delayed puerty: Goal: Mimic sexhormone levels in normal puberty, mimic normal progression through puberty. Boys: increasing doses of testosterone. Girls: increasing doses of estrogen + gestagen after about 2 years for menarche.

Answer 149

AD – Location and Recovery Infants: Cheaks/face and most of body Children: Inside elbows, knees, wrists of hands and ankles, around ears and eyes. Spontanous regression by 2-3 years of age is common. 90% recovery by 20 years of age. ``` AD and Heritage 70 % have atopic disease in the family > 50% risk for AD if both parents have atopic disease Not related to a single gene. - Many different genes (and phenotypes). - Epigenetics seems to be important. ``` Parents search for allergens, however: Allergy is not the most common trigger for AD. 1/3 of 0-3 years of age exacerbate due to allergy. Food allergens (Egg, milk, wheat, soy). Airborne allergens (pollen, pets). Allergy is less important > 3 years of age. Reduced skin barrier due to AD, facilitates sensibilisation to allergens.

Answer 150

Dry skin - (Atopic) ``` Small sebaceous glands Small pores Little sebaceous matter Blood wessels tend to contract. Pale skin ```

Answer 151

SpO2 > 92 % = Serious acute asthma

Answer 152

Allergic Atopic Eczema - Atopic Eczema is most often not triggered by an allergen. - 30% of small children with exzema. - Food most common (egg, milk, soy, wheat, nuts, peanuts, fish). - May be triggered by airborne allergens (pollen, pets).

Pediatri Flashcards

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