Kibreet Notes in Pediatrics.pdf

AWASIR91 ….. 0 Table of contents: 1- Neonatology 2 2- Malnutrition 25 3- Neuropediatrics 37 4- Hematology 46 5- Rheumatology 53 6-...

AWASIR91 ….. 0 Table of contents: 1- Neonatology 2 2- Malnutrition 25 3- Neuropediatrics 37 4- Hematology 46 5- Rheumatology 53 6- Exanthemas 59 7- Immunization 66  Dr. Mousab Mohammed Ibrahim (Kibreet) (88)  Dr. Emeirii Hatim Mustafa (88)  Noora Abubaker Fadul (90)  Maha Hussain (90)  Asia Hassan (91)  Hadeel Mohammed (91)  Huda Faisal (91)  Tasneem Ghazali (91)  Ohayla Elfatih (91)  Waad Taj (91)  Waad Amir (91)  Hajer Ismat (91)  Dan Elrasheed (91) 1 PART 1: NEONATOLOGY 1. Important terms 2. Maternal diseases 3. Drugs 4. Congenital infections 5. Birth injuries 6. Neonatal care 7. New born resuscitation 8. Neonatal seizures 9. Respiratory distress in neonates 10. Other problems of prematurity 11. Neonatal infections 12. Neonatal jaundice 13. Primitive reflexes 1. Important terms: - Embryo: < 9 weeks - Foetus: 9 weeks - birth - Still birth: born ≥ 24 weeks with no signs of life - Prenatal mortality = Still birth + up to 1 week of delivery/ 1000 X live still births - Neonatal mortality = Death in first 4 weeks of delivery/ 1000 X live births o Pre-term: < 37 weeks o Term: 37 - 41 weeks o Post-term: > 42 weeks o Low birth weight: < 2500g o VLBW: < 1500g o Extremely LBW: < 1000g o Small for GA: BW < 10th centile o Large for GA: BW > 90th centile  Neonate: up to 28 days  Infant: 1 - 12 months  Toddler: 1 - 3 years  Pre School: 3 - 5 years  School: 5 - 12 years ، ‫إرا فعلت اليوم ها يتناسل غيرك عن فعله‬.‫فسوف تفعل غذاً ها يعجز غيرك عن القيام به‬ 2  2. Maternal diseases: - Most diseases affect foetus and neonate are: 1- DM. 2- Graves. 3- SLE. 4- AITP (auto immune thrombothytopenic purpura). 1) Problems related to diabetic mother: 1- Congenital malformations: - Most common: Neural tube defects and cardiac malformations. - Characteristics: a- Caudal regression syndrome "sacral agenesis" b- Hypo plastic left colon - Control of DM decrease risk N.B. Congenital malformation does not increase in Gestational DM 2- IUGR 3- Macrosomia: - Shoulder dystocia - Cephalo-pelvic disproportion - Birth injuries In neonate, increase insulin can cause: 1- Hypoglycaemia > (early feeding + close monitoring), Hypocalcaemia, Hypomagnesaemia, Hyperbilirubinemia. 2- RDS (Insulin Anti cortisol) 3- Polycythaemia (Neonatal jaundice) - 4- Transient HoCM (hypertrophic obstructive cardiomyopathy) (resolves in few weeks but may cause heart failure) 2) Graves: - Antibodies cross the placenta - Foetus> Tachycardia = CTG, goitre = US - Neonates > irritability, weight loss, and tachycardia = Heart failure.... Diarrhoea - Xerophthalmous - May require Antithyroid 3) SLE - Anti Ro/ Anti La - Neonatal lupus syndrome (Self limiting rash +/- heart block 3  4) AITP: - Low platelets = increase intracranial haemorrhage - If at delivery = petichea/severe Thrombocytopenia = I.V IGs - If active bleeding = platelets. 3. Drugs: Drug Effect 1- Warfarin 1. Cartilage (nasal Hypoplasia/Epiphyseal stippling) 2. Haemorrhage 2- Lithium Ebstein’s Anomaly 3- Phenytoin Hydantoid face (mid facial Hypoplasia) 4- Thalidomide Phocomelia (short limbs) 5- Tetracycline Enamel Hypoplasia 6- Diethylstilbestrol Vaginal/CX adenocarcinoma (DES) 7- Alcohol 1-fetal alcohol syndrome 2-cardiac abnormalities 8- IV drugs Withdrawal ( jitteriness, seizures, yawing) 9- Smoking stillbirth/miscarriage , LBW, IUGR Ebstein anomaly: Rare heart defect in which the tricuspid valve doesn’t work properly, as a result blood leaks back through the valve into the right atrium. 4  Foetal alcohol syndrome: 1- Characteristic face - Flat nasal bridge - Maxillary Hypoplasia - Absent philtrum - Short thin upper lip 2- Growth abnormalities 3- Developmental delay 4- Cardiac abnormalities Phocomelia 4. Congenital infections Infection c/f Dx Tx 1- CMV Most common urine PCR for Gancyclovir, 90% Asymptomatic virus/culture Sedofovir, Foscarnet 5% Early symptoms IgG antibodies Periventricular calcification. show previous - Others: Hepatosplenomegaly, infection. SN deafness, Microcephaly. Primary infection is the most serious 2- rubella - Earlier > Most dangerous IgM antibody - Triad of: detection 1- Cataract 2- SN deafness 5  3- CHD (e.g. PDA) - Others: Blueberry muffins spots, extra medullary haematopoiesis - After 4 months of gestation > No infection 3-Toxoplasma - Triad of: IgM or IgG (later) Pyramethamine + 1- Diffuse calcification Sulfadiazine for 1 year 2- Chorioretinitis 3- Hydrocephalus > Long term neurological disability 4-varicella >>>> (Palloid B h attacks) become pale (a systole) + LOC (exaggerated vagal cardiac reflex). 2-Breath_ holding attacks Infant or toddler + crying (temper) = blue i.e.; holds his breath. “cyanotic B.H.A” 3-Syncope. - 4-Benign paroxysmal Recurrent episodes of vertigo associated with nystagmus>>> viral vertigo. labrynthitis 5-Basilar migraine. - 6-Others, fabricated, - pseudo seizures, etc. Childhood Epilepsy Some epilepsy syndromes: WEST syndrome 4-6 months, infantile spasm on walking (salaam spasmflexion then extension.) EEG :hypsarrythmia“chaotic pattern” Rx: ACTH. If caused by T.sclerosis>>vigabatran(visual field defect) Complications: epilepsy , learning difficulty N.B Infantile spasm +hypo arrhythmia =WEST syndrome DDx : T.S Hypoglycaemia. LENNOX –GASTAUT Multiple seizures types “1-3 yrs” syndrome (Atypical absence, tonic, a static “drop” attacks). Neurodevelopment arrest or regression and behavioural disorders JUVENILE At adolescence “ when absence end “ MYOCLONIC Throwing foods in the morning. EPILEPSY Lifelong Tx, remission is unlikely. Learning is unimpaired Anti-epileptic drugs: First line: Sodium valporate, Carbamezapine. Second line: Lamotrigine, Topiramate. Cerebral palsy: Def.: Abnormal movement / posture caused by non progr essive damage to the growing infant or foetal brain (Damage before 2 yrs). - Damage is non- progressive. But C/O Progressive. Incidence 2/1000 live births 39  Causes: 1. Antenatal (80%) , congenital infections , c.malformation , vascular problem, etc. 2. Natal (10%), HIE, Trauma. 3. Postnatal (10%), meningitis, encephalitis, hypoglycaemia, cardiopulmonary arrest, haemorrhage, etc C/O “clues” 1. Abnormal posture (limbs /trunk). 4. Persistent primitive reflexes. 2. Delayed motor milestones. 5. Gait problems when walking 3. Asymmetric hand function (less 6. Feeding difficulties (gagging , than 12 months) vomiting , in coordination) Types: 1. Spastic 90%. 3. Ataxic 4%. 2. Dyskinetic 6%. 4. Mixed.(chorioathetoid) spastic Dyskienetic ataxic Damage :corticospinal pathways Old , kernicterus Genetic *Hemiplegic: arms more than legs, sparing the face. now, HIE Damage: Cause: neonatal stroke. Damage : basal cerebellum and Arms: fist, flexed and pronated. ganglia its connections Legs: tip toeing. *Quadriplegic : Cause: HIE Most severe form and the most associated with learning difficulties and epilepsy. *Diplegic: Cause: PVL (preterm) N.B; Spasticity: crossing, tip toe gait. *Problems of C.P or complications 1. Learning difficulties 5. Speech problems 2. Epilepsy 6. Feeding problem>> growth 3. Hearing loss failure. 4. Visual impairment , squint 7. Joint contractures. Rx is multidisciplinary -Physiotherapist, orthopaedic surgeon, speech therapist, adequate feeding, etc. CP 40  PERIPHERAL MOTOR DISORDERS: - Degeneration of motor neurons in the anterior horn. (Fasciculation weakness) **Spinal muscular atrophy SMA: -AR “survival motor neuron (SMN) gene Type 1 Type 2 Type 3 (Warding - Hoffman's ) (intermediate): (kugelberg-Welander): C/o: sit but never walk Later in life (can walk). -foetus with decreased movement independently -At birth :arthrogryposis (deformity + contracture of at least 2 joints) then LMN features “remember tongue fasciculation’s” -Never sit unaided , death by respiratory failure at 12 month “diaphragm paralysis” -Gold standard Dx is genetic study. **Polio: Asymmetrical flaccid paralysis Peripheral nerves (areflexia/ pescavus/ distal weakness):- Hereditary motor sensory neuropathy (HMSN): TYPE 1 (Charcot -Marie – Tooth disease) -Peripheral muscular atrophy N.B inverted wine bottle leg. -AD. 1st decade: distal weakness/ wasting and pescavus. DDx: F. Ataxia -Normal life span. Nerves: enlarged “remyelination attempts” Gold standard Dx: sural nerve biopsy. Biopsy: onion – skin. 41  Fasciculation Muscles: Muscular dystrophies:- Duchene Becker's X-linked recessive (dystrophin) Same but slower C/o proximal weakness >>>waddling gait, gower sign. Dx: 11years “Duchene 4 to 5 yrs” Age 4 - 5 years. Unambulant : 20s “Duchene 10 to 14 yrs” Increased CPK. Death : late 40s “ Duchene late 20s” At 10-14 yrs : not ambulant Late 20s :death “respiratory failure , cardiomyopathy DCM” N.B Scoliosis is common. Rx 1. Physiotherapy 2. Respiratory: NIV at night. **Myotonia:-Delayed relaxation after sustained contraction. 42 **MyotoniaDystrophica: - AD (trineucleotide repeat) -Association: frontal bladness, testicular atrophy, cataract, learning difficulties and DM. Death: cardiomyopathy. Neuromuscular disease: illustrated p.480 FLOPPY INFANT: Rag – doll, head lag, slip through fingers. Frog – like posture ( abduction of LLs) Causes: Central 1. Cortex: HIE, C.malformation. 2. Genetics: Down’s, prder-willi. 3. Metabolic: hypothyroidsm, hypocalcaemia. Peripheral 1. SMA 2. Myopathy 3. Myotonia 4. Congenital myasthenia 43  ATAXIAS: Both are AR 1. Fr.ataxia 2. Ataxia telangectasia Gene:frataxin DNA repair gene (ATM) *Degeneration : -Infancy: oculomotor dyspraxia. -Spinocerebellarataxia -4 yrs: telangectasia (eyes, shoulders, neck). Corticospinalweakness + up going plantar -School age: ataxia. reflex “sinopulmonary” -P.nervesareflexia (all reflexes) &wasting. -Associations >> IgA deficiency = infections. -DCTLoss of vibration &proprioception. -Increase risk of ALL -There is pescavus “high arch “so DDx is -Increase alpha fetoprotein HMSN. Sensitivity of WBCs to radiation “remember -Death 40 – 50 (HOCM, kyphosciliosis) Fanconi” Other causes of ataxia:  Infections: e.g. varicella  Drugs: e.g..phenytoin  Posterior fossa neoplasm e.g. medulloblastoma Telangectasia Stroke: Causes: 1. Cardiac: cyanotic CHD 2. Heamatologic:SCD,anti-thrombin def 3. Post infective: varicella 4. Inflammatory:SLE 5. Metabolic\genetic :homocystinuria 6. Vascular malformations: moya-moya disease(abnormal vasculature). Investigations: - MRI, MRA, carotid Doppler studies - Echo - Metabolic test. 44  Child may need rehabilitation Aspirin prophylaxis. Neural tube defects -Failure of neural plate fusion to form neural tube in the first 28 days of life. It include: Anencephaly Encephalocele Spina bifida Meningiocele Myelomeningiocele : occulta Rx TOP Neurosurgery , Failure of fusion of Neurosurgery C/o (termination but usually v.arch ASAP: Risk 1-LL paralysis (walking aid of there is Skin : tuft of hair , of meningitis. , physiotherapy) pregnancy). underlying birth mark , lipoma Good 2-Muscle imbalance : Most severe: brain , sinus prognosis. dislocation , dead “still malformation Next step >>> U/S talipes(physiotherapy) birth” or die or MRI 3-Loss of sensation (skin shortly after WHY? To exclude care) birth tethering of the 4-Neuropathic bladder cord (intermittent “diastomatomyelia” catheterization, oxybutinin be If +ve ware of UTIs , RF , >>>neurosurgery. hydronehrosis) 5-Neuropathic bowel ( regular toileting , laxatives) 6-Scoliosis (surgery) Hydrocephalus 80% due to Arnold Chiari malformation (shunt) Most severe disease : above L3 Hydrocephalus ‫الشيت‬ N.C syndromes illustrated page 489 ‫ ال تعيش حياتل وأنت ترسن الحذود‬... ‫حتى تنجح‬ ‫عش حياتل وأنت تتخطى مل الحذود‬ 45 PART 4: HEMATOLOGY 1. Anemias 2. Sickle cell disease 3. Thalassemia 4. Anemia in newborn 5. Coagulation problems Anemia: Main site of hemopoiesis in the fetus => liver. Hb F Hb A Hb A2 Fetus 100% - - At birth 75% 26% 1% After one year - 97% 2% *Differences b/w Hb F /Hb A: Hb F: higher affinity for O2.Dissociation curve: shifted to the left. At birth: o Hb is very high (14-21g), gradually decrease over two months (10) => lowest value. o Blood volume: term 80 ml /kg , preterm 100 mg/kg o WBCs = higher (10-25) o PLTs = as adult. o Iron, B12, folic acid => adequate stores in both term and preterm. But in preterm: more ↓↓ and its lower (iron, folic acid). *Diamond black fan anemia: -Family hx =20% , Sporadic= 80% -C/O: At birth (25%) , rest in 2-3 months. Maybe associated with short stature, abnormal thumb. -Gene RPS (ribosomal protein). -Tx: Steroids. If unresponsive, monthly RBCs transfusion, stem cell transplantation. *Transient erythroblastopenia:- No RBS gene - Resolves in few weeks. - No family hx, no RPS, congenital anomalies. 46  **Triggered by viral infection** Increased RBCs destruction (hemolytic anemia) :- ↑Ritcs, ↑Bilirubin, ↑urobilinogen Hepatosplenomegaly. Some points *G6PD deficiency = most common enzymepathy. = most common cause of severe neonatal jaundice requiring transfusion. - Hemolysis in G6PD deficiency => dark urine, fever. Sickle cell disease: Autosomal recessive inherited hemglobinopathy. 1- HbSS (SC anemia): no HbA 2- Hb SC: no HbA 3- Hb SB: Thalassemia no HbA 4- Hb SA (SC trait):40% of Hb is Hb S Sickle cell crisis Dx Mx complications 1- vaso-occlusive 1-full blood Prophylactic : 1-Growth failure (delayed crisis count 1- full immunization puberty). 2-hemolytic crisis 2-blood film 2-daily oral penicillin 2-HF --> anemia. 3-aplastic crisis 3-sickling 3-folic acid 3-Gall stones. 4- splenic solubility test 4-hydroxy urea 4-Renal impairment => sequestration crisis 4-Hb 5- Avoid precipitants (warm , exacerbate enuresis electrophoresis. adequate hydration before (inability to concentrate exercise urine). Mx of acute crisis: 5- Leg ulcers. (Uncommon 1- hydration in children). 2-analgesia 6- Psychological problems. 3-oxygen 7-stroke 4-Abx 5- blood transfusion if needed Indications of exchange transfusion: - Sequestration crisis - Acute chest syndrome - Stroke , priapism 47  # Thalassemia: Defects in production of the α or β chains of hemoglobin resulting imbalance in globin chains leads to ineffective erythropoiesis and Hemolysis in the spleen or BM Types of Thalassemia:  β.Thalassemia :- -β- Thalassemia major => no Hb A -β- Thalassemia trait (intermediate) => few Hb A , high Hb f. β- Thalassemia major: C\O:- severe anemia (Transfusion – dependent) If no transfusion => - Extramedullary hemopoiesis(frontal bossing, maxillary overgrowth ) - Growth failure. Mx:- Regular blood transfusion + iron chelation (oral \ SC desofrroxamine) **aim: Hb = 10 ** Complication of blood transfusion:- 1. Hemochromatosis (cardiomyopathy, 3. Infections (Hep B, C \ HIV \ growth failure …etc). malaria). 2. Abs formation (10%). 4. Venous access problems. ** Definitive => BM transplantation** β- Thalassemia trait: Sx: HbA= >90% DDx: IDA. (Check ferritin) N.B* Dis ↓ in MCV, MCH “very low”, RBCs may be even. -Most important in DxHb A2.  α.Thalassemia :-4 genes , All absentHb Part (hydropsfetalis) 3 absent Hb H (Moderate anemia) 2/1 absent α.Thalassemia trait (mild/no anemia) **NB; β chain X  after 6 months, while α chain X at birth ** Perinatal diagnosis  chorionic villous sampling. Thalassemic face 48  Anemia in Newborn:- Causes ↓ Production congenital B19 infection, Diamond black fan ↑Destruction Immune / non immune(G6PD def., HS, α.Thalassemia ) Blood loss To mother (fetomaternalhemo.) / to twin (TTTTS) / at delivery ** ↑ Ritcs, normal Bilirubin Anemia of prematurity ↓RBCs life span, inadequate erythropoiesis, ↓iron& folic acid, frequent blood sampling Bone marrow failure (A plastic anemia): **Pancytopenia - Inherited: Fanconi, shawrman’s diamond - Idiopathic - Acquired: Infections (hepatitis), Drugs (sulfa amide, chemotherapy), toxins (benzene) Fanconi anemia Shwachman Diamond Syndrome (SDS) AR (DNA repair gene) - AR. C/O: - Association  Exocrine  At birth: congenital anomalies (absent pancreatic abnormalities, thumb),macropthalmia, Kidneys (horse-shoe, skeletal abnormalities, also absent), skin (café aulait) increase risk of leukemia.  5-6 years: Pancytopenia Dx: Dx: When exposed to damaging agent  ↑ chromosomal -prenatal Dx. breakage of peripheral blood lymphocytes - Genetic study (SBDS). Prenatal Dx, family screening ** ↑ risk of acute leukemia, other malignancies (e.g. GI, gyne) Mx: B.M transplantation Coagulation problems: Inherited Acquired Hemophilia -V k deficiency -VWD -liver diseases -ITB -DIC PT = extrinsic pathway. APTT = intrinsic pathway. PT = not used now. (Platelet function analyzer ). RiCoF “Ristocitin cofactor” = Vwf activity.(Vit D) Ristocitin- induced platelet aggregation = abnormal in VWD. 49 Hemophilia VWD - APTT. -AD - X-linked / recessive. BT, APTT. - A= factor VIII deficiency. -VwF: platelet aggregation, factor VIII B= factor IX deficiency. carrying and protection. C/O: Neonate  I.C hemorrhage, post- -Many subtypes: circumcision/ venipuncture bleeding. # Subtype 1 = mild (commonest). Toddlers when start to walk / crawl. ~ C/O : *Severity: puberty/ adolescence: - < 1% severespontaneous bleeding Skin bruising. - 1-5% moderate bleed after Minor Mucosal bleeding (menorrhagia/ epistaxis). injury. **N.B; Spontaneous bleeding not common. - 5-40% mild after major injury. Tx : Mx: -DDAVP mild (subtype 1). Very mild bleeding = DDAVP (desmopressin: Risk: hyponatremia (=seizure) especially if < 1 synthetic ADH). yr. Others: factor VIII concentrate -F VIII concentrates (plasma – derived only). How much? Not recombinant i.e.; do not containVwf. If minor bleeding: till conc. reaches 30%. -replacement therapy. If life- threatening: till “ “ 100%. Then Also avoid: maintain 30-50% for 2 wks. -Aspirin Severe hemophilia = F VIII prophylaxis. – NSAIDS. (Risk of joint damage). -I.M injections. also= Hemophilia (including psychosociotherapy, physiotherapy). ** avoid: I.M injection / NSAIDs- aspirin Complications of Hemophilia: Joint bleeding – Knees>elbows >ankles >shoulders – Leads to chronic synovitis Muscle bleeds – Leads to fibrosis and atrophy – Can lead to compartment syndrome Pseudo tumors Subdural hemorrhage Inhibitors HIV/hepatitis C – 90% if Rx started before 1994 50  Thrombocytopenia: Causes: Destruction Immune: ITP , SLE Non-immune: HUS , TTP , DIC , hypersplenism ↓ production Congenital :wiskott Aldrich , Bernard Soulier Acquired: BM infiltration (leukemia, a plastic anemia). PLt: If 50 - 150.000 = mild (major surgery/trauma) < 50 – 20 = moderate (moderate “surgery). < 20 = severe (risk of spontaneous bleeding) Bleeding: bruising, petichea, mucosal, GI , intracranial (rare) *ITP (immune thrombocytopenic purpura): - Commonest cause of thrombocytopenia in children. - IgG Abs against platelets. # C\O:- - Previously health child - 1-10 years - Sudden onset of generalized purpura and petichea - Profound thrombocytopenia - Bleeding from mucus membranes and gums. #Dx: - by exclusion (ALL, SLE …etc) N.B: you should examine the bone marrow if you want to give steroids, it will mask ALL. #Mx:- Self limiting in 6-8 wks. No TX unless: 1. Severe bleeding (I.C, GI). 2. If minor but affecting life  Oral prednisolone or IVIG or IV anti D Avoid contact sport when platelets are low. **Chronic ITP = > 6 months Also no TX when: Same indications Rituximab (monoclonal Abs against B lymphocytes). 51 - Splenectomy (non-responsive pt) ITP DIC: Sepsis, shock , major trauma , burns Lab: PT, PTT. - ↓PLTs, ↓fibrinogen. (Also ↓ protein C/S, ATIII). - f. degradation products ,  D-Dimers Mx: - Ttt of underlying cause. - ATIII, protein C concentrates. (if - F.F.B or cryoprecipitate needed) - PLTs. (if needed) ‫كن راضيا وكأهك ثملك كل ش ئ‬ ‫فما كحبه هللا لك ألطف مما جشاء‬ 52  PART 5: RHEUMATOLOGY 1) Juvenile idiopathic arthritis 2) SLE 3) Juvenile dermatomyocitis ◾Juvenile idiopathic arthritis: The most common chronic rheumatological disease in children. - Genetic susceptibility. - Environmental triggers. ◽C/O: ◽Criteria: - Onset < 16 years. - Morning stiffness (better during the day). - Joint swelling ≥ 6 weeks. - Arthritis or 2 of (joint pain / tenderness, - No other causes. - ⬇ range of movement, worm). ◽Long term: Overgrowth: - Discrepancy in length, genu valgus - Digits different in length. - Wrist advance bone age ◾Types of JIA - Systemic arthritis - Polyarthritis - Pauciarthritis or oligoarthritis - Entheistis-related arthritis. - Psoriatic arthritis. Pauciarticular Polyarticular 50%, Commonest. ≥5 joints (symmetrical) M F>M If RF +ve “early onset RA” (F adolescent 10-14 ANA +ve in 20% ( ⬆ risk of uveitis) years) worse prognosis than RF - ve. Prognosis good. Cervical spine may be involved. *N. B. If evolve to involved ≥5 joints after 6 months = extended Pauciarticular type. 53  ◽Systemic onset JIV: Fever, Hepatosplenomegaly, lymphadenopathy, salmon rash. ⬆ WBCs, ESR/CRP, Platelets, Anemia. Worst type especially if Polyarticular. ▪ Other types: Enthestitis- related arthritis Psoriasis related arthritis Inflammation of tendon area e. g Achilles’ or Arthritis + psoriasis or arthritis + ≥2 of : Arthritis + family Hx of psoriasis + dactilitis, o 1. uveitis finger nail changes. o 2.spinal pain o 3.sacroilietis o 4. FamilyHx of HLA. B27 related diseases (IBD, uveitis, and spondyloarthritis). o 5. HLA. B27 M>F ◾Complications: - Chronic A. Uveitis. - Osteoporosis (nutrition, steroids, ⬇Wt bearing). - Joint contracture = needs joint replacement - Growth failure, delayed puberty. - Amyleidosis. ◾Tx: Multi disciplinary: ❇ Drugs: 1st choice: NSAIDs (not aspirin) Avoid steroids unless severe sys. Onset JIA or severe arthritis (unable to walk). 2 nd line: - Methotrexate (hepatotoxicity, B.M suppression) - Sulfasalozine, hydroxychloroquine...etc If failed = anti TNFα (inftiximab, etanarcept) 54 * N. B if you do arthrocentisis: WBCs 50,000 – 100,000, lymphocytes (no neutrophils)  non suppurative Remember: physiotherapy, ophthalmic follow u ◾SLE: Criteria : MD SOAP N HAIR M = Malar rash D = discoid rash ( scaling) S = serositis... O = oral ulcer A= +ve ANA P = photosensitivity N = neurological (seizure, psychosis) H = hematology (+ve comb Anemia, Thrombocytopenia, lymphopenia). A = arthritis≥2 peripheral joints. I = immunology (anti ds-DNA, anti Smith Abs, false +ve tests for syphilis, anti cardiolipin Abs= thrombaitis). R = renal ( proteinuria, cellular casts) Other manifestations: Reynaud’s Best for screening (highest sensitivity) = ANA Specificity: - Anti ds DNA. ⬆During active disease - Anti Smith Abs. Most specific How to follow: - Anti ds DNA. Best for follow up. - ESR = in RA, (CRP is normal). - Complements: it is low first. ◾Tx: 55  - Severe flares (nephritis, CNS, Pul.Hemorrhage. etc). I. V cyclophosphamide. - Maintenances + skin diseaseHydroxychloroquine (monitor eyes). - Steroids as start ◾Neonatal lupus: - Maternal Anti Ro. Anti LA, Abs. - Rash, hepatitis, hematological reversible. - Congenital Heart block Permanent. TX: - Corticosteroids - IV.IG - Cardiac pacing Severe cardiomyopathy>>cardiac transplantation. Neonatal lupus ◾Juvenile Dermatomyocitis: - Most common idiopathic inflammatory myopathy in children. - Auto immune capillary vasculopathy>> genetic susceptibility. - 4 _10 yrs. (F>M). C/O: - Symmetrical proximal weakness (Difficulty with stairs, getting up, +ve Gower's.. etc). - May affect pharyngeal muscles =dysphagia. - May affect Resp. Muscles. - Skin : 56   Rash. (Face)  Shawl’s sign.  Gottrons papules.  Heliotrope Rash.  Periungualr erythema. - Arthritis Dx: - No sys : inflammation ( just muscles), WBCs, ESR, CRP are normal - EMG( electromyography changes) : myositis - ⬆Muscle enzyme (AST, ALT, CPK, LDH, Aldolase). - Biopsy : confirmatory Mx: - Steroids - Skin: hydroxychloroquine , avoid sun(= SLE) - #1 complication Calcinosis Heliotrope rash Gottrons papules 57 58  ‫ال جسخسلم كلما جعثرت انهض وكلما اخطأت صحح وكلما فشلد حاول‬ ‫وكلما اصزت الايام ان ثجعلك عابسا ابخسم رغما عنها‬ PART 6: EXANTHEMAS A- Maculopapular rashes 6- Infectious mononucleosis 1- Measles B- Vesicular rash 2- Rubella 1- Varicella zoster 3- Fifth disease 2- Hand foot and mouth disease 4- Rorseola infantum 3- Kwasakis disease 5- Scarlet fever ______________________________________________________ Measles: Paramyxovirus * Infectivity: 4 days before- 4 days after the rash C/O: High grade fever+3Cs (coryza, conjunctivitis, cough) Koplik’s spot (oral mucosa) maculopapular rash (starts in the facebehind the earsbody) Season: late winter, spring Complications: 1- Respiratory: pneumonia, otitis media (most common), and tracheitis 2- CNS: A-encephalitis B-Sub acute sclerosing C-Febrile convulsions pan encephalitis=SSPE -7 days after starting illness -7 years after infection - -15%=mortality -Dementia death -40%of survivors=long term -very high measles Abs in sequel(deafness, convulsions,) the serum/CSF 3- Others: appendicitis, hepatitis, Long term: - Diarrheadehydration - PEM (protein energy malnutrition) - Corneal cloudication - Mouth ulcers 59 ***Measles in special conditions (low CMI): PEM: severe disease HIV: modified or absent +rash severe disease Mx: - Antipyretics - Eye care=tetracycline ointment - Mouth care: salted water - Nutrition + Vit. A - Ribavirin: immune compromised only Rubella (German measles) Mild disease Risk= Congenital rubella syn. C/O: prodrome: mild or no fever - Congenital rubella syndrome - Arthritis in female usually - Rash: maculopapular (face trunk) not Myocarditis itchy Prevention: MMR Disappears in 3-5 days (3 days measles) Exanthemforscheiner spot in soft palate Dx: serology Most characteristically= Sub occipital post auricular lymphadenopathy N.B: since fever is mild or absent the rash is usually the first sign Complications: Erythema.infectiosum (Fifth disease) - B19 disease As x Prodrome (wk.) slapped cheek appearance 60 lace reticular rash in the trunk+extrimities Recurrence can occur; proactive factors Heat (sun exposure, hot path, emotional upset Complications: - Arthritis (adults) - A plastic crises 1/chronic Hemolysis 2/immunodeficiency (can’t form Abs to clear infections) E.g. malignancy - Non immune hydropsfetalis (vertical transmission); If affects pregnant women in the first trimester N.B: B19 affects eryothoblastoid cells (precursors of RBCs) (Bone marrow) Roseola infantum (Exanthemsubitum) - HHV 6, HHV 7 - Infant, high fever (but he is relatively well - Fever disappears in 3or4 days - Then rash appears in hours (rarely 24 hrs.) - Starts from the trunkextremitiesneckface N.B: miss diagnosed as drug allergy Infant +fever=Abx, rash appears Scarlet fever 61 Only rash covered by bacteria Group A streptococcus C/O:Fever, exudative pharyngitis, tonsillitis - Abdominal pain, DDx for acute appendicitis - Maculopapular rash (goose like skin or sand paper) especially deep areas= antecubital fossa, axilla, groin), areas of hyper pigmentation that doesn’t disappear by pressure - White coated tongue then strawberry tongue - Desquamation in 6 days sunburn like skin (esp. in fingers/ toes) +circumoral pallor. TX: Penicillin Complications: 1. RF 3. AGN 2. Peritonsillar abscess (hot potato 4. Retropharyngeal abscess like voice) Infectious mononucleosis (glandular fever) -EBV Association with EBV (oncogenic) 1/Burkett’s lymphoma 2/Nasopharyngeal CA Transmission: oral secretions (kissing disease) C/O: fever, tonsillopharyngitis (may obstruct breathing) 62  Lymphadenopathy (prominent cx) large Splenomegaly, hepatomegaly, rash N.B: if treated with amoxicillinamoxicillin rash Dx: Atypical lymphocytes (peripheral blood) Heterophil Abs +ve (monospot test is +ve) Serology: high EBV IgM, IgG TX: supportive, avoid contact sporting (spleen rupture) Vesicular rash Varicella zoster (chicken pox) Infectious: 2 Days before the rashuntil crusting of all lesions (5-7 days) Rash: itchy, vesicular, starts in the trunk in different stages (macule, papule, pustule, vesicle, crust) Goes in crop Sparing palms and soles Complications: 1/2ry bacterial infection s.aurus, strep (most common complication) -Mild or sever as toxic shock syndrome, necrotizing fasciitis How to suspect? Recurrence of fever 2/CNS: encephalitis (better prognosis than HSV), cerebellitis 3/ in immune compromised: pneumonitis hemorrhagic vesicle, disseminated disease (may be DIC) 4/Purpurafulminans=thrombocytopenia TX: Supportive - Antiviral: IV acyclovir or oral Val acyclovirImmunocompromized Oral Val acyclovir=adults VZIG  Immune compromised in contact with diseased person Severe disease: adults+immunocompromised Hand foot and mouth disease: 63 - Vesicles in these areas +buttocks - Cause: coxackie virus, A16 Dorsum of the hands and feet, may affect palms and soles Kawasaki disease( mucocutaneus LN disease) - Vasculities - Target: 6months-4years (5 days + 4 of: 1/non purulent conj. Injection 2/mucus membranes (red, cracked lips, pharyngeal injection, strawberry tongue) 3/rash: polymorphous, no vesicles, no crust 4/cervical lymphadenopathy (1 L.N >1.5 cm) 5/extremities changes (palms soles edema, peeling (conuelsence), erythema Lab: high WBCS/ESR/CRP/platelets (2nd wk.) Complications: 1/ Coronary aneurysm (6wks) 2/ MI (thrombosis) 3/ Sudden death TX: -IVIG (within 10 days) +high dose of aspirin until fever, inflammatory markers are down Then change to low dose aspirin (prevent thrombosis) Echo: If +ve aneurysm= warfarin+ follow up N.B: Aspirin is given for 6 wks. First high dose=anti-inflammatory. Then low dose=ant platelet If poor response to aspirin; infliximab or steroids N.B: IVIG within 10 daysdecrease incidence of coronary aneurysm 64 ‫فصبرا في مجال العلم صبرا فما هيل املنى سهل املزاد‬ ‫‪65‬‬  PART 7: IMMUNIZATION Passive Active - Natural: from the mother - Natural: post infection - Acquired: Igs or antitoxins - Acquired:vaccination - May be life long as in MMR & chicken pox - EPI: Eradication of poliomyelitis, measles & Neonatal Tetanus N. B.: 10 childhood infections + T. Toxoid ◾ Types of vaccines: 1. Live attenuated vaccine : most potent (BCG, OPV) 2. Killed vaccine 3. Recombinant vaccine : Hep B 4. Capsular polysaccharides vaccine : pneumococus&Hib Contraindications to all vaccines: 1. Previous history of allergy (edema, breathing difficulties, shock) 2. Severe local reaction 3. Fever >40 c° or severe illness. 4. Within 6m of blood transfusion For all live attenuated: - Pregnancy - Immunocompromized with exception of MMR & varicella in child with HIV infection Schedule: Day 0 - BCG(0.05)ml ,left upper third of forearm, intradermal - OPV(zero dose (Sabin))2 drops oral 6 wks - OPV 1 - Pentavalent: 0.5 ml. IM. Left Anterolateral thigh - Rota: 1ml oral. Live attenuated - Pneumococcal vaccine: 0.5 ml IM/SC Right Anterolateral thigh. Capsular conjugated to protein “Diphtheria Toxoid” 10 wks - OPV 2 - Rota - Pentavalent - Pneumococcal 14 wks - 0PV 3 - Pentavalent - Pneumococcal 9 months - Measles : 0.5 ml. SC. Live attenuated 18 months - Measles 66 New: IPV “Salk” gives in 3 doses with Pentavalent N.B. Pneumococcal vaccine: - 13 valent “ immunization “ - 23 valent ( SCD, Splenectomy, Nephrotic syndrome, Immunocompromized, cochlear implant, chronic diseases) N.B - MMR = Autism , IBD - Rota = intesseception - Pertussis = Encephalopathy Measles = Thrombocytopenia Side effects: - All.: Mild local reaction ( redness, swelling), Mild symptoms (e.g. Fever) - BCG: lymphadenitis, skin ulcer, scar, local abscess. N. B. Mild disease, low grade fever, vomiting, mild diarrhea =not contraindications N. B. # Polysaccharides = B cells response > poor immunity less than 2 years, short term immunity, Absence of Booster response when exposure. VS # conjugated polysaccharide = T cell response >⬆⬆⬆ level of functional Abs, long term persistent IgG response, immunological memory. Special contraindications: - BCG : prematurity, skin infection - DPT( Pertussis) : Encephalopathy within 7 days, seizure within 72 hours - Measles: Thrombocytopenia purpura, neomycin allergy, Egg allergy (caution), active TB. Rota: gastroenteritis, hx of intusseption. 67 68