00_McClure_Prelims.indd xxvi 11/14/2012 11:16:25 PM DETAILED CONTENTS xxvii Skin infection: viral and bacterial 826 Fungal skin infections 828 Parasitic skin infection s 829 Protozoal skin infections 829 Hair disorders 830 Nail disorders 832 Photosensitivity and light eruptions 834 Pigmentation disorders 836 Collagen and elastin disorders 838 Conne ctive tissue disorders 839 Miscellaneous skin conditions 840 23 Pediatric surgery 843 Symptoms and signs that should cause concern 844 Congenital abnormalities: upper airway 846 Congenital abnormalities: tracheo-oesophageal 848 Congenital abnormalities: esophagus 850 Congenital abnormalities: lung 851 Congenital abnormalities: chest 852 Idiopathic hypertrop hic pyloric stenosis 854 Ingested foreign bodies 856 Bezoars 857 Midgut malrotation and vol vulus 858 Intussusception 860 Duodenal atresia 862 Small bowel atresias 862 Meconium ileus 862 Acute appendicitis 864 Mesenteric adenitis 865 Meckel \200\231s diverticulum 865 Gastro schisis 866 Exomphalos (omphalocele) 866 Inguinal hernias 868 Hydroceles 868 Hirschsprung \200\231s disease 870 Rectal prolapse 871 Anorectal malformations 872 Umbilical anomalies 8 74 Testicular torsion 876 Orchitis and epididymitis 877 00_McClure_Prelims.indd xxvii 11/14/2012 11:16:25 PM xxviii DETAILED CONTENTS Testicular trauma 877 Undescended testes (cryptorchidism) 878 Retractile testes 879 Hypospa dias 879 Phimosis and paraphimosis 880 Balanitis/balanoposthitis 880 Priapism 880 Penile tr auma 881 Imperforate hymen 881 Labial adhesions in infants 881 Miscellaneous conditions 882 Perioperative care 884 Consent for surgery 886 Post-operative care: \202 uids 888 Post-o perative care: analgesia 890 Post-operative care: drains and wounds 891 24 Special senses 893 Common presentations 894 Hearing assessment 896 Childhood deafness 898 Disorders of the ear 900 Common disorders of the nose 902 Disorders of mouth and tongue 904 Visual development and examination 905 Vision assessment 906 Vision screening in the UK 908 Squints (strabismu s) 910 Ametropia (refractive disorders) 911 Amblyopia 911 Visual impairment 912 Nystagmus 9 13 Disorders of the eye: infection and in \202 ammatory 914 Eye foreign body 916 Cataract 916 Glaucoma 917 Orbit and eyelids 918 Back of the eye problems 920 Trauma 922 00_McClure_Prelims.indd xxviii 11/14/2012 11:16:25 PM DETAILED CONTENTS CONTENTS xxix 25 Genetics 925 Useful resources 926 Clinical genetics and genetic counseling 927 When to refer to clinical genetics 928 Taking a family history 930 Genetic testing 932 Chromosome tests 933 Molecula r genetic analysis 934 Practical issues relating to genetic testing 935 Down syndrome 936 C ommon chromosomal disorders 938 Genetic disorders with cardiac features 940 Genetic testing in cognitive impairment 942 Genetic disorders with neuromuscular features 944 Genetic diso rders with dermatological features 946 Genetic disorders of growth 948 Miscellaneous geneti c conditions 950 Miscellaneous congenital malformations 951 26 Inherited metabolic disease 953 General principles 954 Metabolic syndromes: neurological 956 Metabolic syndromes: metabolic acidosis 958 Metabolic syndromes: storage dysmorphism 959 Metabolic syndromes: hepatic syn dromes 960 Metabolic syndromes: cardiac syndromes 962 Urea cycle disorders 964 Disorders of amino acid metabolism 965 Disorders of organic acid metabolism 966 Disorders of carbohydra te metabolism 967 Disorders of lipoprotein metabolism 967 Lysosomal storage diseases 968 Di sorders of fatty acid oxidation 970 Mitochondrial disorders 971 Peroxisomal disorders 972 D isorders of nucleotide metabolism 973 Disorders of porphyrin metabolism 974 Disorders of me tal metabolism and transportation 976 00_McClure_Prelims.indd xxix 11/14/2012 11:16:25 PM xxx DETAILED CONTENTS CONTENTS 27 Community child health 979 Introduction 980 Voluntary and charitable organizations 980 Organizations and structures 98 1 Health surveillance and promotion 982 Special educational needs 986 Children with disabil ities 987 Speci \201 c learning dif \201 culties 988 Chronic fatigue syndrome 990 Absence from school 991 Constipation and soiling 992 Enuresis 994 28 Child protection 997 De \201 nitions 998 Illness fabricated or induced by carers 999 Physical abuse 1000 Sexual abuse 1002 Emotional abuse 1003 Medical involvement in child protection 1004 Referrals to other agencies 1005 Medical assessment 1006 Assessment by social care 1008 Prevention strat egies 1010 Further reading 1010 29 Pharmacology and therapeutics 1011 Prescribing for children 1012 Adverse drug reactions 1014 Pharmacokinetics 1016 Drug metabo lism 1017 Pain management 1018 Sedation 1019 Fever 1019 30 International health and travel 1021 Child survival: world health 1022 World health: childhood illness 1023 00_McClure_Prelims.indd xxx 11/14/2012 11:16:25 PM DETAILED CONTENTS xxxi Taking children on holiday 1024 Illness after international travel 1026 Further reading 102 8 31 Pediatrics, ethics, and the law 1029 Ethics 1030 Common law 1031 Recognition of ethical issues in everyday clinical practice 103 2 Clinical case study 1033 The doctor \200\223child relationship 1034 Parental responsibili ty 1035 The doctor \200\223parent relationship 1035 Assent and consent 1036 Con \201 denti ality and disclosure 1038 Withholding or withdrawing treatment in children 1040 Good ethica l and legal practice in suspected child abuse 1042 Serious case reviews 1043 Legal aspects of international adoption 1044 00_McClure_Prelims.indd xxxi 11/14/2012 11:16:25 PM xxxii Symbols and abbreviations X i d n l p s 4 5 +/ \200\223 +ve \200\223ve AABR ABC ABCD ABPM ACE ACh ACL ACTH AD ADEM A DH ADHD ADP ADPKD ADR A&E AFP AG AIDS AIS AKI ALA ALCL ALD controversial topic increased decreased normal leading to primary secondary male female wit h or without positive negative Automatic auditory brainstem response airway, breathing, cir culation airway, breathing, circulation, disability ambulatory blood pressure monitoring an giotensin converting enzyme acetylcholine anterior cruciate ligament adrenocorticotrophin a utosomal dominant acute disseminated encephalomyelitis antidiuretic hormone attention de \201 cit hyperactivity disorder adenosine 5-diphosphate Autosomal dominant polycystic kidne y disease adverse drug reaction accident and emergency (department) alpha-foetoprotein anio n gap acquired immune de \201 ciency syndrome androgen insensitivity syndrome acute kidney injury aminolevulinic acid anaplastic large cell lymphoma adrenoleucodystrophy 00_McClure_Prelims.indd xxxii 11/14/2012 11:16:25 PM SYMBOLS AND ABBREVIATIONS xxxiii ALL ALP ALS ALT AML AN ANA ANC ANCA ANLL AP APC APD APH APRT APTT AR ARA ARDS ARF ARM ARPKD ARR AS ASA ASCA ASD ASIS ASOT AST AT ATP AV AVP AVSD AXR AZA BA BAL acute lymphoblastic leukemia alkaline phosphatase advanced life support alanine transferase acute myeloid leukemia anorexia nervosa anti-nuclear antigen antenatal care anti-neutrophi l cytoplasmic antibodies acute non-lymphoblastic leukemia antero-posterior activated protei n C automated peritoneal dialysis antepartum hemorrhage adenine phosphoribosyltransferase a ctivated partial thromboplastin time autosomal recessive acute rheumatoid arthritis acute r espiratory distress syndrome acute renal failure arti \201 cial rupture of membranes autos omal recessive polycystic kidney disease absolute risk reduction Angelman syndrome 5-aminos alicylic acid anti-Saccharomyces cerevisiae antibodies atrial septal defect anterior superi or iliac spine anti-streptolysin O titre aspartate transaminase ataxia telangiectasia adeno sine triphosphate arteriovenous arginine vasopressin atrioventricular septal defect abdomin al X-ray azathioprine bone age British anti-Lewisite 00_McClure_Prelims.indd xxxiii 11/14/2012 11:16:25 PM SYMBOLS AND ABBREVIATIONS xxxiv BBS BCG bd BiPD BLCL BLS BM BMD BMI BMT BP BPD BSS BWS BXO CA CACT CAD CaSR CAH CAM CAMHS C BT CCNU CD CDD CDGP CDH CDS CeO CER CF CFAM CFRD CFS CFTR CGA CGM CGMS CH Bardet-Biedl syndrome Bacille Calmette \200\223Gu rin twice a day bipolar disorder diffuse large B cell basic life support bone marrow Becker muscular dystrophy body mass index bone marrow transplantation blood pressure bronchopulmonary dysplasia Bernard \200\223Soulier s yndrome Beckwith \200\223Wiedemann syndrome balanitis xerotica obliterans choanal atresia c arnitine \200\223acylcarnitine translocase coronary artery disease calcium-sensing receptor congenital adrenal hyperplasia cystic adenomatoid malformation child and adolescent mental health services cognitive behavior therapy Lomustine Crohn \200\231s disease conduct disor der constitutional delay in growth and puberty congenital diaphragmatic hernia congenital d yserthropoietic anemia cerebral oedema control event rate cystic \201 brosis cerebral fun ction analysis monitoring cystic \201 brosis related diabetes chronic fatigue syndrome cy stic \201 brosis transmembrane receptor corrected gestational age continuous glucose moni toring continuous glucose monitoring system cystic hygroma 00_McClure_Prelims.indd xxxiv 11/14/2012 11:16:25 PM SYMBOLS AND ABBREVIATIONS xxxv CHARGE coloboma, heart defects, choanal atresia, retarded growth, genital anomalies, ear abnormali ties choriocarcinoma congenital heart disease Children \200\231s Hospital of Eastern Ontari o Pain Scale carbohydrate con \201 dence interval congenital ichthyosiform erythroderma Cr eutzfeldt \200\223Jakob disease creatine kinase chronic lung disease congenital lobar emphy sema congenital Myasthenia gravis chronic myeloid leukemia cytomegalovirus central nervous system carbon monoxide coarctation of aorta coagulase-negative staphylococci corticotrophin -releasing factor cerebral palsy continuous positive airway pressure cardiopulmonary resusc itation carbamyl phosphate synthetase de \201 ciency chronic renal failure Crying, Require s oxygen for saturation >95%, Increased vital signs, Expression, Sleepless chronic recurren t multifocal osteomyelitis C-reactive protein capillary re \201 ll time Cesarean section c erebrospinal \202 uid continuous subcutaneous insulin infusion convulsive status epilepti cus computerized tomography cardiotocogram central venous pressure cardiovascular system ch est X-ray cytochrome P450 CHC CHD CHEOPS CHO CI CIE CJD CK CLD CLE CMG CML CMV CNS CO CoA CONS CoRF CP CPAP CPR CPS C RF CRIES CRMO CRP CRT CS CSF CSII CStE CT CTG CVP CVS CXR CYP 00_McClure_Prelims.indd xxxv 11/14/2012 11:16:26 PM SYMBOLS AND ABBREVIATIONS xxxvi DALY DCD DCT DDAVP DDH DEND DEXA DHAP DHEAS DI DIC DIP DJF DKA DMARD DMD DMSA DPG DPT DSM D TPA DVM DVT EAR EBM EBV EC ECG ECLS ECMO EDS EEG EER EHEC ELBW ELISA EM EMDR EMG EMU disability-adjusted life year developmental coordination disorder direct Coombs \200\231 te st deamino-8-d-arginine vasopressin (desmopressin) developmental dysplasia of the hip devel opmental delay, epilepsy, and neonatal diabetes dual-energy X-ray absorptiometry dihydroxya cetone phosphate dehydroepiandrosterone sulfate diabetes insipidus disseminated intravascul ar coagulation distal interphalangeal (joint) duodenojejunal \202 exure diabetic ketoacid osis disease-modifying antirheumatic drug Duchenne muscular dystrophy dimercaptosuccinic ac id diphosphoglycerate diphtheria, pertussis, tetanus Diagnostic and statistical manual diet hylenetriamine pentaacetic acid delayed visual maturation deep vein thrombosis estimated av erage (nutritional) requirement expressed breast milk Epstein \200\223Barr virus embryonal carcinoma electrocardiogram Extracorporeal life support extracorporeal membrane oxygenation Ehlers-Danlos syndrome electroencephalogram experimental event rate enterohaemorrhagic E. coli extremely low birth weight enzyme-linked immunosorbent assay electron microscopy eye m ovement desensitization and reprocessing electromyogram early morning urine 00_McClure_Prelims.indd xxxvi 11/14/2012 11:16:26 PM SYMBOLS AND ABBREVIATIONS xxxvii EMV ENT EPO ERCP ES ESR ESRF ET ETT FA FB FBC FDG FDP FEL FEV1 FFP FH FHL FiO2 FISH FIX FLA G FRAXA FRC FSGS FSH FTT FVC FVL GA GAD GBS G-BS GCS G-CSF GCT GDAP GFR GH electromagentic valve ear, nose and throat emergency protection order endoscopic retrograde cholangiopancreatography Ewing \200\231s sarcoma erythrocyte sedimentation rate end-stage renal failure essential thrombocytheaemia endotracheal tube Fanconi \200\231s anemia foreig n body full blood count 18F- \202 udeoxyglucose \201 brin/ \201 brinogen degradation pr oducts familial erythrophagocytic lymphohistiocytosis forced expiratory volume in 1 second fresh frozen plasma familial hypercholesterolaemia familial haemophagocytic lymphohistiocyt osis fractional inspired oxygen \202 uorescence in situ hybridization factor IX \202 ud arabine, ara-C, and G-CSF (regime) fragile X syndrome functional residual capacity focal se gmental glomerulosclerosis follicle-stimulating hormone failure to thrive forced vital capa city factor V Leiden general anesthetic generalized anxiety disorder group B streptococcus Guillain \200\223Barr syndrome Glasgow coma scale granulocyte colony-stimulating factor g erm cell tumor ganglioside-induced differentiation-associated protein glomerular \201 ltr ation rate growth hormone 00_McClure_Prelims.indd xxxvii 11/14/2012 11:16:26 PM SYMBOLS AND ABBREVIATIONS xxxviii GHIS GHRH GI GluA GluAD GN GnRH GOR GORD GP GPI G6PD GSD GU GVHD HAV Hb HBeAg HBL HBsAg HBV HCC hCG Hct HCV HD HE HELLP growth hormone insensitivity syndrome growth hormone-releasing hormone gastrointestinal glu taric aciduria glutamic acid decarboxylase glomerular nephritis gonadotrophin-releasing hor mone gastro-oesophageal re \202 ux gastro-oesophageal re \202 ux disease general practiti oner glycosylphosphatidylinositol glucose-6-phosphate dehydrogenase glycogen storage diseas e genitourinary graft versus host disease hepatitis A virus hemoglobin hepatitis B virus e antigen hepatoblastoma hepatitis B surface antigen hepatitis B virus hepatocellular carcino ma human chorionic gonadotrophin haematocrit hepatitis C virus haemodialysis or hereditary elliptocytosis haemolytic anemia \200\223elevated liver enzymes \200\223low platelet count high frequency oscillatory ventilation hypogonadotrophic hypogonadism human herpes virus 6 hepato-iminodiacetic acid hypoxic \200\223ischaemic encephalopathy hiatus hernia heparin-in duced thrombocytopenia human immunode \201 ciency virus Hodgkin \200\231s lymphoma human l eukocyte antigen haemophagocytic lymphohistiocytosis HFOV HH HHV6 HIDA HIE HIH HIT HIV HL HLA HLH 00_McClure_Prelims.indd xxxviii 11/14/2012 11:16:26 PM SYMBOLS AND ABBREVIATIONS xxxix HLHS HMG-CO HOCM HPA HPLC HPRT HR HRCT HS HSD HSP HSV HUS HVA IAA IAP IBD IBS ICA ICD ICP I DM IDDM I:E iem IEM IGF Igs IGT IHPS IIH ILAR IO IM IMD INR IPPV IRT IT ITP hypoplastic left heart syndrome 3-hydroxy-3-methyl-CoA hypertrophic obstructive cardiomopat hy human platelet antigen hemoglobin electrophoresis hypoxanthine \200\223guanine phosphori bosyltransferase heart rate high resolution computerized tomography hereditary spherocytosi s Hirschsprung \200\231s disease Henoch \200\223Sch nlein purpura herpes simplex virus hae molytic \200\223uraemic syndrome homovanillic acid insulin auto-antibody intrapartum antibi otic prophylaxis in \202 ammatory bowel disease irritable bowel syndrome islet cell antibo dy International Classi \201 cation of Diseases intracranial pressure infant of diabetic m other insulin-dependent diabetes mellitus ratio of inspiratory time to expiratory time inbo rn error of metabolism inborn errors of metabolism insulin-like growth factor immunoglobuli ns impaired glucose tolerance idiopathic hypertrophic pyloric stenosis idiopathic intracran ial hypertension International League of Associations for Rheumatology intraosseous intramu scular inherited metabolic disease international normalized ratio intermittent positive pre ssure ventilation immunoreactive trypsinogen intrathecal idiopathic thrombocytopenic purpur a 00_McClure_Prelims.indd xxxix 11/14/2012 11:16:26 PM SYMBOLS AND ABBREVIATIONS xl ITT ITU IU IUGR IUT IV IVC IVGT IVH IVI IVIG JCA JDM JIA JRA JVP KS LBW LCH LDH LDL LFT LGA LH LHRH LI LIP LKM LKS LMW LOC LOS LP LR LRD LRTI LS LSCS LSE M4Eo insulin tolerance test intensive therapy unit international units intrauterine growth restr iction intrauterine blood transfusion intravenous inferior vena cava intravenous glucose to lerance test intraventricular hemorrhage intravenous infusion intravenous immunoglobulin ju venile chronic arthritis juvenile dermatomyositis juvenile idiopathic arthritis juvenile rh eumatoid arthritis jugular venous pressure Kallmann syndrome low birth weight Langerhan \200\231s cell histiocytosis lactate dehydrogenase low density lipoprotein liver function t est large for gestational age luteinizing hormone luteinizing hormone-releasing hormone lam ellar ichthyosis lymphoid interstitial pneumonitis liver/kidney microsomal (antibodies) Lan dau-Kleffner syndrome X-linked lymphoproliferative level of consciousness lower oesophageal sphincter lumbar puncture likelihood ratio living related donor lower respiratory tract in fection linear scleroderma lower segment Cesarean section left sternal edge acute myelomono cytic leukemia with eosinophilia 00_McClure_Prelims.indd xl 11/14/2012 11:16:26 PM SYMBOLS AND ABBREVIATIONS xli MA MAG-3 MAHA MAOI MAP MAS MCAD McAS MCD MCDK MCH MCHC MCP M,C&S MCTD MCUG MCV MD MDI MDP M DS MeA MELAS microalbuminuria mercaptoacetyltriglycine microangiopathic haemolytic anemia monoamine oxid ase inhibitor mean airway pressure meconium aspiration syndrome medium chain acyl-CoA dehyd rogenase de \201 ciency McCune \200\223Albright syndrome minimal change disease multicysti c dysplastic kidneys mean cell hemoglobin mean corpuscular hemoglobin concentration metacar pal phalangeal (joint) microscopy, culture, and sensitivity mixed connective tissue disease micturating cystourethrography mean cell volume Meckel \200\231s diverticulum metered dose inhaler myeloproliferative disorder myelodysplastic syndrome mesenteric adentitis mitochon drial encephalopathy \200\223lactic acidosis and stroke- like episodes (syndrome) multiple endocrine neoplasia Marfan syndrome membranous glomerulonephritis meta-iodo-benzylguanidine metachromatic leucodystrophy methylmalonic acidaemia mycophenolate mofetil measles, mumps, rubella (vaccination) maturity onset diabetes of young membranoproliferative glomeruloneph ritis mid-parental height mucopolysaccharidosis minimal residual disease magnetic resonance imaging Munchausen syndrome by proxy melanocyte-stimulating hormone MEN MFS MGN MIBG MLD MMA MMF MMR MODY MPGN MPH MPS MRD MRI MSbP MSH 00_McClure_Prelims.indd xli 11/14/2012 11:16:26 PM SYMBOLS AND ABBREVIATIONS xlii MSU MTHFR MTP MTX NAGS NAHI NAI NAIT NC NCStE NEC NF NFCS NG NGT NHL NIMH-MTS National Inst itute of Mental Health-Multimodal Treatment midstream urine methyltetrahydrofolate reductase metatarsal phalangeal (joint) methotrexate N-acetylglutamate synthetase de \201 ciency non-accidental head injury non-accidental inj ury neonatal alloimmune thrombocytopenia nasal cannula convulsive status epilepticus necrot izing enterocolitis neuro \201 bromatosis (NF1, NF2) neonatal facial coding scale nasogast ric nasogastric tube non-Hodgkin \200\231s lymphoma Study Neonatal and Infant Pain Scale neuromuscular junction number (of patients) needed to treat neonatal unit Noonan syndrome non-steroidal anti-in \202 ammatory drug neuron-speci \201 c enolase new variant Creutzfeldt \200\223Jakob disease oesophageal atresia otoacoust ic emission obsessive \200\223compulsive disorder once daily observed difference opposition al de \201 ant disorder occipitofrontal circumference oral glucose tolerance test osteogen esis imperfecta Online Mendelian Inheritance in Man (database) osteosarcoma obstructive sle ep apnoea obstructive sleep apnoea syndrome ornithine transcarbamylase de \201 ciency NIPS NMJ NNT NNU NS NSAID NSE nvCJD OA OAE OCD od OD ODD OFC OGTT OI OMIN OS OSA OSAS OTC 00_McClure_Prelims.indd xlii 11/14/2012 11:16:26 PM SYMBOLS AND ABBREVIATIONS xliii PaCO2 p-ANCA PANDAS arterial carbon dioxide tension perinuclear antineutrophil cytoplasmic antibody pediatric a utoimmune neuropsychiatric disorder associated with Streptococcus arterial oxygen tension p eripheral blood stem cell transplants patient-controlled analgesia paroxysmal cold haemoglo binuria polycystic kidney disease Polycystic ovarian syndrome pneumocystis pneumonia polyme rase chain reaction packed cell volume peritoneal dialysis patent ductus arteriosus pervasi ve developmental disorder psychologically determined paroxysmal events pulmonary embolism p ositive end-expiratory pressure peak expiratory \202 ow rate polyethylene glycol positron emission tomography platelet function assay prostaglandin E1 pseudohypoparathyroidism post -haemorrhagic ventricular dilatation pediatric intensive care unit pulmonary interstitial e mphysema peak/positive/proximal inspiratory pressure Premature Infant Pain Pro \201 le pyr uvate kinase phenylketonuria propellant metered dose inhaler promyelocytic leukemia parente ral nutrition Permanent neonatal diabetes mellitus primitive neuroectodermal tumor paroxysm al nocturnal haemoglobinuria orally/by mouth precocious puberty PaO2 PBSCT PCA PCH PCKD PCOS PCP PCR PCV PD PDA PDD PDPE PE PEEP PEFR PEG PET PFA PGE1 PHP PHVD PICU PIE PIP PIPP PK PKU PMDI PML PN PNDM PNET PNH PO PP 00_McClure_Prelims.indd xliii 11/14/2012 11:16:26 PM SYMBOLS AND ABBREVIATIONS xliv PPHN PPI PPROM PR PResp PrIP PROM PSS PT PTH PTHrP PTSD PTT PTV PUJ PUV PV PVH PVL PWS qds RA RAS RAST RBC RCC RCM RCT RDS RF rhGH RIF RMS RNP ROM ROP RP RR RRR RSV persistent pulmonary hypertension of newborn proton pump inhibitor preterm prolonged ruptur e of membranes rectally, per rectum parental responsibility proximal interphalangeal (joint ) prolonged rupture of membranes progressive systemic sclerosis prothrombin time parathyroi d hormone PTH-related peptide post-traumatic stress disorder partial thromboplastin time pa tient-triggered ventilation pelviureteric junction posterior urethral valve vaginally, per vagina periventricular hemorrhage periventricular leucomalacia Prader-Willi syndrome four t imes a day rheumatoid arthritis re \202 ex anoxic seizures radioallergosorbent test red bl ood cell red cell count red blood cell mass randomized, controlled trial respiratory distre ss syndrome rheumatoid factor recombinant human growth hormone right iliac fossa rhabdomyos arcoma ribonucleoprotein range of movement retinopathy of prematurity retinitis pigmentosa respiration rate relative risk reduction respiratory syncytial virus 00_McClure_Prelims.indd xliv 11/14/2012 11:16:26 PM SYMBOLS AND ABBREVIATIONS xlv RTA RV SAA SAD SaO2 SBR SC SCD SCID SCII SCL SDH SE SENCO SGA SHBG SIADH SIDS SIMV SIPPV SL E SM SMA SMN SN SOB SOS SPA SpO2 renal tubular acidosis residual volume severe aplastic anemia separation anxiety disorder a rterial oxygen saturation serum bilirubin subcutaneous sickle cell disease severe combined immunode \201 ciency SC insulin infusion subcortical leucomalacia subdural hemorrhage stan dard error special educational needs co-ordinator small for gestational age sex hormone-bin ding globulin syndrome of inappropriate antidiuretic hormone sudden infant death syndrome s ynchronized intermittent mandatory ventilation synchronized intermittent positive pressure ventilation systemic lupus erythematosus sternomastoid muscle spinal muscular atrophy or su perior mesenteric artery survival motor neuron sensorineural shortness of breath self-refer ral of symptoms suprapubic aspiration pulse oximetry measurement of oxyhaemoglobin saturati on steroid-resistant steroid-sensitive systemic sclerosis subacute sclerosing panencephalit is selective serotonin reuptake inhibitors status epilepticus sexually transmitted infectio n subependymal sudden unexpected death in an infant slipped upper femoral epiphysis SR SS SSC SSPE SSRI StE STI subE SUDI SUFE 00_McClure_Prelims.indd xlv 11/14/2012 11:16:27 PM SYMBOLS AND ABBREVIATIONS xlvi SVC SVT T1DM T2DM T3 T4 TA TaGVHD TAPVD TAR TAT TB TBI TBM TcCO2 TcO2 TCPL TDC TDD tds TdT TE TEC TEG TEWL TFT TH Ti TIBC TLC TNDM TNF TOF TORCH superior vena cava supraventricular tachycardia type 1 diabetes mellitus type 2 diabetes me llitus triiodothyronine thyroxine tricuspid atresia transfusion-associated graft versus hos t disease total anomalous pulmonary venous drainage thrombocytopenia \200\223absent radius (syndrome) transanamastic tube tuberculosis total body irradiation tuberculous meningitis t ranscutaneous carbon dioxide pressure transcutaneous oxygen pressure time-cycled, pressure limited thyroglossal duct cysts total digitalizing dose (for digoxin) three times a day ter minal deoxynucleotidyl transferase expiratory time transient erythroblastopenia of childhoo d thromboelastogram transepidermal water loss chapter 6 thyroid function test therapeutic h ypothermia inspiratory time total iron binding capacity total lung capacity Transient neona tal diabetes mellitus tumor necrosis factor tracheo-oesophageal \201 stula toxoplasmosis, others, rubella, cytomegalovirus, herpes virus II tissue plasminogen activator total paren teral nutrition Toddler \200\224Preschooler Postoperative Pain Scale TSH receptor antibody transfusion-related acute lung injury TPA TPN TPPPS TRAB TRALI 00_McClure_Prelims.indd xlvi 11/14/2012 11:16:27 PM SYMBOLS AND ABBREVIATIONS xlvii TSC TSH TSS TT TTG TTN TTP UAC UC U&E UNC UNHS UP:UCr URTI US USS UTI UV UVC VACTERL tuberous sclerosis complex thyroid-stimulating hormone toxic shock syndrome thrombin time t issue transglutaminase IgA antibody transient tachypnoea of newborn thrombotic thrombocytop enic purpura umbilical arterial catheter ulcerative colitis urea and electrolytes urine net charge universal newborn hearing screening urinary protein to urinary creatinine (ratio) u pper respiratory tract infection ultrasound ultrasound scan urinary tract infection umbilic al vein umbilical venous catheter vertebral anomalies, anal atresia, cardiac malformations, tracheo-oesophageal \201 stula, renal and limb anomalies vascular endothelial growth fac tor vitamin D-dependent rickets Venereal Disease Research Laboratory (test) ventricular \201 brillation von Hippel \200\223Lindau (disease) vasoactive intestinal polypeptide very low birth weight very low density lipoprotein vanillylmandelic acid vaso-occlusive very sev ere aplastic anemia ventricular septal defect ventricular tachycardia ventilation \200\223p erfusion ratio vesicoureteric junction vesicoureteric re \202 ux von Willebrand \200\231s disease von Willebrand \200\231s factor varicella zoster virus VEGF VDDR VDRL VF VHL VIP VLBW VLDL VMA VOD VSAA VSD VT V/Q VUJ VUR vWD vWF VZV 00_McClure_Prelims.indd xlvii 11/14/2012 11:16:28 PM SYMBOLS AND ABBREVIATIONS xlviii WAGR WBC WCC WG WS XLP YST ZIG Wilms, aniridia, gonadal dysplasia, retardation white blood cell white cell count Wegener \200\231s granulomatosis Williams syndrome X-linked lymphoproliferative yolk sac tumor zost er immunoglobulin 00_McClure_Prelims.indd xlviii 11/14/2012 11:16:28 PM 1 Chapter 1 Practicing pediatrics Reading and learning pediatrics 2 Professional conduct and attitudes 3 Professional skills 4 Knowledge 6 01_McClure_01.indd 1 11/14/2012 7:04:21 PM CHAPTER 1 Practicing pediatrics 2 Reading and learning pediatrics Welcome to pediatrics and child health! You will \201 nd this area of medi- cine challenging, rewarding, and above all fun. We have written this han d- book to help you develop as a practitioner \200\224whether it \200\231s in the emergency department, inpatient wards, outpatient clinic, or family health surgery. Six basic goals in your learning If you are a novice in the \201 eld, you will \201 nd that every day requires new skills, and sometimes this can seem daunting. Take heart. We ho pe that this experience will provide an education in the aspects of general paediat- rics t hat are important for all medical practitioners. Your curriculum goals should be the follow ing. \200 basic knowledge of growth and development: learn about Acquire physical, physiological, and psychosocial change from birth through to adolescence and see how this applies to clinical practice. Develop adolescents, and their families. Become children, and adolescents. Learn treatment of common acute and chronic pediatric illnesses. Improve your clinical problem-s olving skills. Take and health of children in modern society, and in our different communit ies. communication skills: this will help you to speak to children, competent in physical examination of babies, infants, toddlers, enough core knowledge so that you can make a diagnosis and start \200 a broader perspective and understand more about the upbringing As you scan through the handbook you will see that all of the chapters cover some aspect of these points. We hope that you will take time to annotate particularly helpful guides and record what you have learned. Perhaps, with time, things will not appear quite so daunting. What next after this foundation? For those who are using this handbook to progress in their postgraduate level of learning we have been more prescriptive in the next sections. We hav e itemized certain objectives that are deemed essential for profes- sional conduct, attitud es, skills, and knowledge. Use these as a checklist and monitor your progress as you work t hrough the handbook. Again, most chapters cover aspects of the material that you will need. 01_McClure_01.indd 2 11/14/2012 7:04:21 PM PROFESSIONAL CONDUCT AND ATTITUDES 3 Professional conduct and attitudes \200 Have you learned to adapt your clinical approach to patients of all ages? Can you communica te with the child or adolescent and family in the clinic? What about dealing with con \201 dentiality and privacy? (see b p.1038). Can news to new parents, or to the newly-diagnosed adolescent with chronic illness or disability? (see b p.794). Do with courtesy and recogni ze the contributions of each? Are you aware of cultural, ethnic, and socio-economic factors in your practice? you have a foundation in basic ethical principles? Do you appreciate Do the ethical challenges speci \201 c to pediatrics and child health? (see b pp.1032, 1038). you communicate clearly and sensitively? How do you break bad you work well in a team? Do you treat each member of the team 01_McClure_01.indd 3 11/14/2012 7:04:21 PM CHAPTER 1 Practicing pediatrics 4 Professional skills Interviewing (b Chapter 3) \200 Can period, immunizations, development, diet, family and social history, and systems review is unique to pediatrics. Can you collect this information in a timely manner \200\22440min in a complex case history. You should also be able to modify the medical history depending on the age of the child, with particular attention to the following age groups \200\224neo nate; infant, toddler, school age, and adolescence. Can to know what are the important ques tions to ask \200\224what is going to help you now with your treatment. you obtain a complete medical history? The history of the perinatal you obtain a focused medical history? In an emergency you will need Physical examination (b Chapter 3) \200 Can you complete a full physical examination of an infant, child, and adolescent, including the observation and documentation of normal physical \201 ndings? You should be able to do this \200\230long case \200\231 examination in less than 10min. Can you carry out a pro blem-orientated examination? child with a limp: what are the important positive and negativ e clinical \201 ndings? Are you a good observer? Can you assess behavior, neurodevelopmen t, and pubertal staging? For example, in the \200 Do you take time to look \201 rst? Communication \200 Can to identify the main concerns of the patient and family? Can you communicate informatio n to both the patient and parent, making sure both understand the diagnosis and treatment p lan, and do you give them the opportunity to ask questions? Can you write a discharge lette r for the family doctor? Can you write a full medical summary for the medical notes? Can Ca n you communicate effectively with other health care workers, including nurses and social w orkers, and explain the thought process that led to your diagnosis and treatment? you establish rapport with the patient and family? Are you able \200 \200 you present to colleagues a well-organized summary of your patient? Clinical problem solving \200 Can common clinical presentations? Can you use your knowledge of key signs and symptoms, an d the frequency and prevalence of diseases at different ages to develop a likely differenti al diagnosis? Can the results of commonly ordered laboratory tests, such as the full blood count, urinalysis, and serum electrolytes, and recognize that the normal values of some tes ts may vary with the age of the patient? you compile a problem list and differential diagnosis for each of the you make a management plan of investigations? Can you interpret 01_McClure_01.indd 4 11/14/2012 7:04:21 PM PROFESSIONAL SKILLS 5 Can and management of clinical problems? Can you critically appraise a topic (i.e. patient, intervention, outcome \200\224see b Chapter 2, p.16) and decide on best evidence for treat ment? you use the medical pediatric literature to research the diagnosis Practical procedures (b Chapter 7) \200 Do intravenous (IV) line, nasogastric (NG) tube, etc.)? Can you explain these procedures to parents and children? Specialist trainees in pediatrics should be able to perform the proc edures in the Boxes 1.1 and 1.2. you know when certain procedures are needed (e.g. lumbar puncture, \200 Box 1.1 Diagnostic procedures \200 \200 b p.203) Venepuncture and venous cannulation for blood sampling ( Collection of blood from central l ines and umbilical arterial lines (b pp.204 \200\223209) Capillary blood sampling ( Electro cardiogram (ECG) b p.220) Lumbar puncture (LP, Suprapubic aspiration of urine ( Non-invasiv e blood pressure (BP) measurement Urethral catheterization ( Urine analysis using standard bedside tests Blood sugar measurement using standard point-of-care glucometers \200 \200 \200 \200 \200 \200 \200 b p.202) b p.219) b p.218) Diagnostic procedures with supervision \200 \200 b p.205) Peripheral arterial cannulation ( Needle thoracentesis of pleural effusion for microbiology and cytology Box 1.2 Therapeutic procedures \200 \200 \200 \200 \200 \200 \200 b p.211) Bag, valve, and mask ventilation ( Placement of an oral airway ( External chest compression Tracheal intubation of term newborn babies ( Removal and replacement of a blocked tracheot omy tube Percutaneous long-line insertion ( Placement of NG tube b p.210) b p.212) b p.209) Therapeutic procedures with supervision \200 \200 \200 \200 \200 Injections: Insertion of an intraosseous (IO) needle Administer surfactant Tracheal intubat ion of preterm and older child Chest drain insertion for pneumothorax intradermal, subcutaneous (SC), intramuscular (IM), and IV 01_McClure_01.indd 5 11/14/2012 7:04:21 PM CHAPTER 1 Practicing pediatrics 6 Knowledge During your reading you should consider these questions as a starting point to th e knowledge that would be expected of a junior pediatrician. Growth (b Chapter 13) \200 \200 What are the intrauterine factors that affect growth of the fetus? Can you explain how grow th charts are used in the longitudinal evaluation of height, weight, and head circumference ? In particular, consider the following. \200 Can you recognize abnormalities of growth that warrant further evaluation? What is the sign i \201 cance of crossing centiles on a growth chart? What is the signi \201 cance of disc repancies between height, weight, and head circumference? What are: short stature; constitu tional delay; failure to thrive; obesity; microcephaly; and macrocephaly? \200 Development (b Chapters 15, 24, and 27) Why is following development important in clinical pediatrics? \200 What are the normal changes in re \202 exes, tone, and posture in the infant? What is the normal progression in motor milestones in the \201 rst year? What are the signs of cerebr al palsy? \200 Behavior (b Chapters 15, 16, and 21) What are the typical presentations of common behaviora l problems at various developmental levels and ages? \200 \200 \200 What are temper tantrums? How may somatic complaints represent psychosocial problems? In wh at types of situations does pathology in the family contribute to childhood behavior proble ms? Nutrition (b Chapter 10) \200 What factors contribute to the development of failure to thrive in infancy? What factors co ntribute to the development of child obesity? What are the special dietary needs of childre n with chronic illness? What caloric intake is needed for normal growth in infants and smal l children? \200 \200 Also consider the following. \200 What are the major differences between human milk and commonly available formulas? What are the advantages of breastfeeding? 01_McClure_01.indd 6 11/14/2012 7:04:21 PM KNOWLEDGE 7 Newborns (b Chapter 6) \200 \200 What diseases are detected by neonatal blood screening? What important historical informati on, physical examination \201 ndings, and laboratory data are needed for the differential diagnosis of the following problems: \200 \200 \200 \200 \200 \200 \200 \200 \200 jitteriness or seizures; jaundice; lethargy or poor feeding; respiratory distress; cyanosis ; bilious vomiting; non-bilious vomiting; hypoglycaemia; sepsis? Genetics and congenital malformations (b Chapters 23 and 25) \200 \200 What are the effects of teratogenic agents such as alcohol and phenytoin? What are the \201 ndings and implications of the common chromosomal abnormalities: \200 trisomy 21; se x chromosome abnormalities (e.g. Turner \200\231s syndrome, fragile X \200 syndrome); oth er genetic disorders (cystic \201 brosis, sickle cell disease)? Common pediatric illnesses For each of the common \200\230presentations \200\231 and \200 \230conditions \200\231 in this handbook can you review: \200 \200 \200 \200 \200 \200 cause; pathophysiology; natural history; presenting signs and symptoms; initial laboratory test and/or imaging needed for diagnosis; plan for initial management? 01_McClure_01.indd 7 11/14/2012 7:04:22 PM This page intentionally left blank 9 Chapter 2 Epidemiology, evidence, and practice Introduction 10 Descriptions in populations 10 Summary of study designs 11 Levels of eviden ce 12 Basics of statistics 14 Training and special knowledge skills 16 Useful websites and resources 18 References 18 02_McClure_02.indd 9 11/14/2012 2:00:43 PM CHAPTER 2 Epidemiology, evidence, and practice 10 Introduction The aim of this chapter is to provide the epidemiological information that we \201 nd useful in supporting our every-day clinical practice. You may have read an articl e and need a quick reference. Alternatively, you may want to examine some data published in a report and apply it to your work. Asking questions is a skill that you, the clinician, s hould develop. It is important to ask questions that fall into two main categories: \200 Those that de \201 ne the burden of disease: when questions. Those that understand or sear ch for the cause of childhood disease : i.e. why, and how questions. i.e. what, who, where, and