Spina bi \201 da: p140 139 Head and neck words To outsiders, it seems as if pediatricians are obsessed with measuring head circumference and head shape, and translating the latter into Latin \200\224as if de \201 ng the outside could explain what what is going on inside. To insiders, though, \200 \207it is to diagnose and treat craniostenois that we we measure heads. So always know w here your patient is on his or her centile charts. Craniostenosis=craniosynostosis=prematur e closure of one or more of the skull \200\231s \201 brous sutures by ossi \201 cation. It aff ects 1 : 2000 of whom 2 \200\22311% have a family history. 15 \200\22340% have one of 180 recognized syndromes (with a fam- ily history it \200\231s 50%). Normal time for sut ures to close is 3 \200\2239 months for the metopic (frontal) suture, 29 \200\22339yrs for other sutures (saggital, coronal, lam- boid). The skull compensates for closure by growing in the direction parallel to the closed suture. If the compensatory growth allows insuffi c ient space for the growing brain there will be \200\200ICP visual loss, sleep impairmen t (ob- structive sleep apnoea), eating problems and \200\201 IQ. Babies with insuffi cient head growth (centile charts p222 \200\2234) or skull deformity need assessment by a cranio facial surgeon. 4 \200\22320% of children with single suture closure have raised intracrani al pressure, up to 60% if more than one suture involved. Look for papilloedema. Skull X-ray : single closed suture. CT: structural brain abnormalities and suture fusion and will diagn ose deformational plagio- cephaly (due to absent suture) and pansynostosis secondary to mic ephaly. Subarachnoid spaces are larger in microcephaly. Surgery at 6 \200\22312 months aims to normalize the cranial vault and to allow for brain growth. Cyclopia: A single eye in th e area normally occupied by the root of the nose, which is missing, or present in the form of a proboscis (a tubular append- age) located above the eye. In some, it can be viewed as an extreme form of hypotelorism. It may be part of trisomy 13 (Patau \200\231s syndrome). D olicephalic: The head is elongated, eg as in Marfan \200\231s, or El Greco portraits. Dysto pia canthorum: Intercanthal distance is increased, but not the inter- pupillary or (bony) i nterorbital distances. Holoprosencephaly: (a whole, ie single-sphered, brain) Hypotelorism with cleft palate premaxillary agenesis cyclopia cebocephaly (see above) \200\224 follows failure of the lateral ventricles to separate (defective cleavage of the prosenceph alon), eg with fusion of the basal ganglia. Lissencephaly: Smooth cortex with no convolutio ns (agyria). Metopic suture: This is the same as the frontal suture. Micrognathia: The mand ible is too small. Neurocranium: That part of the skull holding the brain. Oxycephalic (=tu rricephaly=acro-cephaly) The top of the head is pointed. Plagiocephaly: If fully expressed, synos- tosis aff ects coronal (rarely lambdoidal) sutures ( palpable bony ridge) with a \202 at forehead and elevation of the orbit on one side. Minor (unfused) plagiocephalic as ymmetry is common in infants sleeping on their backs, improves with time, and is of no sign i \201 cance. Associations: scoliosis and pelvic obliquity ( \201 g 1). Rachischisis: Spi nal column \201 ssure. Sinciput: Anterior, upper part of head. Viscerocranium: Facial ske leton. Wormian bones: Supernumerary bones in the sutures of the skull. s c i r t a i d e a P Fig 1. Plagiocephaly. Neural tube defects (NTDS) Neural tube defects result from failure of the neural tube to cl ose between the 3rd and 4th week of in utero development. Myelodysplasia: Any neuroectoderm defect, eg of the cord, either multiple anterior horns, several central canals, failure of cord fusion so that there is a \202 at neural plaque, not a fused tube (myelocele), or a double neural tube (diplomyelia), or herniation through a bony defect as a meningocele (co nt- ains dura & arachnoid) or meningomyelocele (the cord is involved too, \201 g 1). Spin a bi \201 da implies an incomplete vertebral arch (spina bi \201 da occulta if covered by skin). Anencephaly implies absent skull vault and cerebral cortex. Encephalocele means tha t part of the brain protrudes through the skull. Incidence in Europe 4000 preventable NTDs /yr. 311 Risk increases with young primips, lower social class, and homozygosity for a poin t mutation (C677 \200\203T; prevalence \211\21010%, interfering with folate metabolism). R ecurrence risk rises 10-fold if one pregnancy is aff ected, 20-fold if 2, 40-fold if 3 preg nancies af- fected; and 30-fold if a parent is aff ected. See below for risk reduction. Neu rological de \201 cit is variable, depending on level of the lesion and the degree to whic h the lower cord functions independently from the upper cord. The de- fect may progress aft er birth; hydrocephalus gradually worsens mental perfor- mance. A child who learns to walk during his 2nd year may subsequently outgrow his ability to support (weight increases as th e cube of surface area, power only as its square). Those with lumbosacral myelomeningoceles usually learn to walk with calipers by the age of 3, but \211 20% with higher lesions eve r walk. When there is paralysis below L3, as unopposed hip \202 exors and adductors are l ikely to dislocate the hips; only 5 \200\22313% retain their ability to walk. 312n=173 Post natal surgery Firm guidelines on whom to treat often prove simplistic in individual infants . The \201 nal outcome of early closure of the defect depends on the state of the kidneys after multiple UTIs, and the extent of delayed hy- drocephalus (requiring ventriculoperito neal CSF shunts). Early post-operative mortality may account for 25% of deaths. Many opera tions may be needed for spinal deformity (often severe and very hard to treat). Intrauterin e diagnosis A maternal serum \200 -fetoprotein >90U/mL at 18 weeks detects 80% of open sp ina bi \201 das and 90% of anencephalics, but also 3% of normal singleton fetuses, twins, and some with exomphalos, congenital ne- phrosis, urethral valves, Turner \200\231s syndrom e, trisomy 13, and oligohydramnios. Amniocentesis and skilled ultrasound \200\200pick-up r ates further. Intrauterine surgery (eg at 23 weeks \200\231 gestation) This is very controv ersial. Hurdles for the developing child \200 Urinary and fecal incontinence. Penile appl iances, urinary diversions, or in- termittent self-catheterization save laundry and bed sor es. Regular evalua- tion of bladder function is essential (surgery may or may not help).1 140 s c i r t a d e a P i The mother who \200\230does it all \200\231 can prevent maturity developing. \200 Immobi lity. Mobility allowances are small and of little help. \200 Social and sexual isolation, if a special school is needed. 313 Prevention In mothers who have already had an aff ected baby, there is good evidence that folic acid (eg 5mg/day \200\224or if diabetic, obese, on anti-epileptics, p2) given from before conception (as the neural tube is formed by 28 days , before pregnancy may even be recognized) reduces the risk of recurrence of neural tube de fects by 72%. If no previous neural tube defects, 0.4mg of folic acid is recommended in the months before conception and for 13 weeks after; see p2. 314 Many mothers don \200\231t ta ke folate pre-conception, hence the call for folate-forti \201 cation of bread etc. This i s adopted in the USA, but is not ideal, as presentation of low B12 states may be delayed (s ee BOX 1). 315 \200\223317 1 Untethering the spinal cord may be indicated: seek expert advi ce. See J Urol 2007 331.318 141 Intraspinous ligaments CSF in subarachnoid space Displaced spinal cord Dura mater with overlaying membranous sac Skin stops here Fig 1. Meningomyelocele. Should we fortify staple foods with folic acid? This is done in many places but not in Euro pe, from inertia as much as from regard to civil liberties/freedom of choice, but this iner tia is being overtak- en by research showing that the hoped-for decline in neural tube defe cts is not occurring \200\224as most people are ignorant of (or do not act on) advice to ta ke folic acid before conception. 319 Inertia is reasonable, perhaps, as some unforeseen har m may occur from forti \201 cation. If forti \201 cation is favored, forti \201 cation o f \202 our is one attractive option. 320 40 countries have tried this; success in prevent ing NTDs ranges from 19% to 78%. 321 So what harm could there be from folic acid? It can co rrect anemia due to a low B12 \200\224and hence might mask the underlying disease, and allo w development or progression of B12-related neuropathy and subacute com- bined degeneration of the spinal cord, if diagnosis depended on the pres- ence of anemic symptoms. This possi bility may be partly overcome by educating ourselves, so that we do not believe that macroc ytic anemia is a necessary sign of B12-related neurological disorders. 322 Another argument in favor of universal forti \201 cation is that signi \201 cant neuropsychiatric mor- bi dity is also preventable by folic acid supplementation. 323 the FACIT trial 2007 ? s t a o r h t s c i r t a i d e a P r u o n w o d s l a c i m e h c g n \201 f u t s e t a t s y n n a n a s t n a w o h W Involuntary euthanasia for some neonates with spina bi \201 da? Deliberate termination of life of newborns with meningomyelocele is prac- ticed openly only in the Netherlands. \200 \230Unbearable and hopeless suff ering \200\231 is the single most cited criterion for this termination, together with the statement that \200\230there are no other proper medical m eans to alleviate this suff ering \200\231. 324 Covert termination may or may not be more w idespread. There are many sides to this question and many assumptions behind the phrases in blue which need examining. How to decide? We cannot decide here: only bear in mind one que stion \200\234How can rational compassionate people reach diametrically opposed positions? \200\235 Part of the answer is to realize that we ourselves change over time and then to go on to as k how this change comes about. What changes? As a thought experiment, catalog what changes when you change your mind. The facts? Fashion? The context? Experience? Other people \200 \231s expectations of what your role is? A paralyzing ability to see both sides of every qu es- tion, like mirrors re \202 ecting each other ad in \201 nitum? Changing our mind is a process well worth studying. It is as if we are relieved when the seesaw swings from one s table-and-smug state of knowing-with-certainty to an- other. We hastily brush away the inel egant internal maneuverings which precede change. But hold on \200 it is these inelegant ma neuvers which vali- date us as human beings. So \200 let \200\231s try getting more comfort able with being uncomfortable. And perhaps this leads us to a yardstick to help answer the question: which position augments our humanity most? ? d e t a i v e l l a e b s y a w l a g n i r e f f u s t s u M ND=noti \201 able disease MeaslesND, rubellaND, mumps ND, and erythroviruses Measles (RNA paramyxovirus) Spread: Droplets. Incuba- tion: 7 \200\22312 days; infective from prodrome ( cough, conjuncti- vitis, T \200\200) until 5d after rash starts. Koplik spots ( \201 g 1 ) are pathognomonic. They are often fading as the rash appears (eg behind ears, on day 3 \200\2235, spreading down the body, be- coming con \202 uent). \201\231 : IgM & IgG +ve; 325 PCR for typing. Com- plications: More common if <5yrs or >20yrs. Otitis media is Courte sy of CDC. most common complication. Croup, tracheitis and bronchi- olitis occur in infants . Pneumonia is the most common cause of death in mea- sles. Older patients may develop ence phalitis \200\224of these 15% die; 25% develop \201 ts, deafness, or \200\201cognition. Subacute sclerosing parencephalitis is a chronic complication of measles which develops 7 \200\22313yrs after primary infection with pro- gressive changes in behavior, myoclonus, ch oreoatheotosis, dystonia, dementia, coma and death. \201\227 : Isolate \200\224in hospital , if the patient is ill or immunocompro- mised or malnourished, or has pneumonitis, CNS sig ns, or dehydration, then: \200 Ensure adequate nutrition (catabolism is very high). Conti nue breastfeeding, 142 Fig 1. Koplik spots. even during diarrhea. Pass a nasogastric feeding tube if intake is poor. In the developing world, need for vitamin A arises; consider 2 doses, + 1 more s c i r t a d e a P at 6 weeks later (p450). CI: pregnancy; known not to be de \201 cient. Treat secondary bacterial infection; antibiotics such as amoxicillin for otitis media and p neumonia. Prophylactic antibiotics have no clear role. 326 Immunization: p151. Only 80% eff ective. 327 Prognosis: Good in rich countries; in poor areas death rate is 10%. 328 (0.9 million/yr, mostly in Africa). 329 Rubella (RNA virus). Incubation: 2 \200\2233wks. Infecti vity: 5 days before to 5 days after start of rash. Signs: Macular rash; suboccipital lympha denopathy. Im- munization: Live virus, p151. 330 Complications: Small joint arthritis. Malf orma- tions in utero (p34). Infection during the 1st 4 weeks: eye anomaly (70%); wks 4 \200 \2238: cardiac abnormalities (40%); wks 8 \200\22312: deafness (30%). Mumps (RNA paramyxovi rus) Spread: Droplets/saliva. Incubation: 14 \200\22321d. Immunity: Lifelong, once infected . Infectivity: 7 days before and 9 days after parotid swelling starts. Signs: Prodromal mal aise; T \200\200; painful parotid swelling, becoming bilateral in 70% ( \200 \200 : Sj gren \200\231s; leukemia; dengue; herpes-virus; EBV; HIV; sarcoid; pneumococci; haemophilus ; staphs; anaphylaxis; blowing glass or trumpets; drugs; \201 g 2). Complications: Usuall y none; orchitis ( infertility), ar- thritis, meningitis, pancreatitis, myocarditis, deaf ness, myelitis. \201\227: Rest. Vac- cine: p151, for any non-immune adult or child (SE: ra re parotitis/pancreatitis). Erythrovirus ( \200\230 \201 fth disease \200\231, erythema in fectiosum; parvovirus B19; \201 g 3). Signs: Usually a mild, acute infection, with malar erythema ( \200\230slapped cheek \200\231) and a rash mainly on the limbs (gloves and socks syndrome, in adults). 331 By the time this appears, infectivity has waned. Constitutional upset is mild. Ar- thralgia is commoner in adults \200\224who may present as \200\230gland ular fever \200\231 (false +ve Paul \200\223Bunnell). 332 Spread by droplet 331 is rapid in closed communities. It can also cause the marrow to stop making RBCs (aplastic crisis) \200\224serious if RBC lifespan is already short (eg sickle-cell disease, thalassaemia, sph erocy- tosis, HIV). \200 : IgM (PCR if immunocompromized). \201\227: Transfusions and im mu- noglobulins are rarely needed. 331 Pregnancy: Risk of fetal death is 10% (esp. midtrim ester) 333 \200\224eg from hydrops fetalis \200\223 inhibition of multiplication and lysis of erythroid progenitor cells (monitor AFP for several weeks; if it rises abnormally, do u ltrasound). Fetal/neonatal problems: hydrops (in 3%; treat by intrauterine transfusion if severe), growth retardation, meconium peritoni- tis, myocarditis, glomerulonephritis, place ntomegaly, hepatomegaly, oedema, pancytopenia. Respiratory insuffi ciency/death is rare. 10 % of those aff ected before 20 weeks miscarry; in the rest, risk of congenital abnormality is 1%. 334 Hand, foot & mouth disease The child is mildly unwell; develops vesicles on i palms, soles, and mouth. They may cause discomfort until they heal, without crusting. Incub ation: 5 \200\2237 days. Treatment is symptomatic. Cause: Coxsackie- virus a16 or enterovir us 71 (suspect in outbreaks with herpangina, meningitis, \202 accid paralysis pulmonar y oedema). Herpangina entails fever + sore throat + vesicles or macerated ulcers (on palate or uvula, which heal over 2 days) abdominal pain and nausea. This has nothing to do wit h the bovine form. Herpes infections See OHCM p400 \200\2231. Varicella zoster/chickenpox: p144. Roseola infantum This is a common, mild, self-limiting illness in infants, caus- ing T \200\200, then a maculopapular rash on subsidence of fever at the end of the 4th febril e day. Uvulo-palatoglossal junctional ulcers may be a useful early sign. 335 Cause: Herpes virus 6 (HHV6; double-stranded DNA). It is related to other herpes viruses (HSV 1 & 2, vari cella zoster, EBV & CMV). Interaction between host im- munities and other viruses may lead to cellular immunode \201 ciency and fatal illness. Synonyms: exanthem subitum, fourth dis ease, 3-day fever. It is neuro- tropic (a rare cause of encephalitis/focal gliosis on MRI, maybe accounting for why the not uncommon roseola \200\230febrile \201 ts \200\231 tend to occur after the fever). 336 Other causes of rashes in children See also skin diseases se ction (p582). \200 A transient maculopapular rash is a feature of many trivial viral illn esses (but 143 s c i r t a i d e a P a few macules may be a sign of early meningococcaemia). Purpuric rashes: meningococcaemia (p202); Henoch \200\223Sch nlein purpura (p197); idiopathic thrombocytopenic purpura (check FBC and \201 lm). Drug rashes (maculopapular) from eg penicillins or phenytoin are common. \200 Scabies (p6 08); insect bites. \200 Eczema (p596); urticaria (p584); psoriasis \200\224guttate psoria sis may follow a respiratory tract infection in children (p594); pityriasis rosea (p602). \200 Still \200 \231s disease: transient maculopapular rash, fever, and polyarthritis. Fig 3. Slapped cheeks (parvovirus B19). Fig 2. Mumps: generalized salivary swelling. Both images courtesy of CDC. Managing distressing fever in viral illnesses \200\207Unwrap \200\207rehydrate \200\207a ntipyretics tepid sponge (a partly validated alter- native/additional method; 337 NB: co ld water may vasoconstrict). Antipyretics aren \200\231t always needed 338 ( \200\200mortal ity if used in severe sepsis, 339 and paraceta- mol is implicated in asthma/eczema). 340 n= 205,487 Keep records of quantity used. Ibuprofen at 10mg/kg/8h ( 100mg/8h if 1 \200\2234yrs old; twice this if 7 \200\22310yrs) is better than paracetamol at 15mg/kg/6h, so try it \201 rst. Giving both alternate- ly is a bit better than giving one alone, if sustained eff ects are needed. 341 Oral paracetamol doses 1 \200\2233 months old: 30 \200\22360mg/8h. Ch ildren over 3 months use maximum of 4 doses/24h then doses are: 3 \200\2236 months 60mg/4 \200\223 6h; 6 months \200\2232yrs 120mg/4 \200\2236h; 2 \200\2234 years 180mg/4 \200\2236h ; 4 \200\2236 years 240mg/4 \200\2236h; 6 \200\2238 years 240 \200\223250mg/4 \200\2236h; 8 \200\22310 years 360 \200\223375mg/4 \200\2236h; 10 \200\22312 years 480 \200\223500mg/4 \200\2236h; 12 \200\22316 years 480 \200\223750mg/4 \200\2236h; 16+ years 500mg \200\2231g/ 4 \200\2236h. Maximum of 4 doses in 24h. Paracetamol suppositories are available (60, 125, or 250mg). N=165 Varicella (herpes) zoster virus (VZV) Chickenpox is a primary infection with varicella-zost er virus. Shingles (OHCM p400) is a reactivation of dormant virus in posterior root ganglia . Chickenpox Signs: Crops of skin vesicles of diff erent ages, often starting on face, scal p or trunk. The rash is more concentrated on the torso than the ex- tremities. Incubation: 11 \200\22321 days. Infectivity: 4 days before the rash, until all lesions have scabbed ( 1 wk). Spread: Droplets. It can be caught from some- one with shingles. It is one of the most infectious diseases known. 95% of adults have been infected; immunity is lifelong. Tests: Fluorescent antibody tests and Tzanck smears are rarely needed. \201\231 \201\231: Hand, f oot, and mouth disease; insect bites; scabies; rickettsia. Course: T \200\200; rash start s 2 days later, often starting on the back: macule \200\203papule \200\203vesicle with a re d-surround \200\203ulcers (eg oral, vaginal) \200\203crusting. 2 \200\2234 crops of lesions occur during the illness. Lesions cluster round areas of pressure or hyperaemia. Complicat ions: If spots are blackish (purpura fulminans) or coalescing and bluish (necrotizing fasci itis), get urgent help on ITU; avoid ibuprofen. Be alert to pneumonia, meningitis, myelitis , CNS thrombi, DIC, LFT \200\200, Guillain \200\223Barr , Henoch \200\223Sch nlein, nephr itis, pancreatitis, myositis, myocarditis, orchitis, cerebellar ataxia. If susceptible, liv e attenu- ated vaccine pre-cytotoxics/steroids may be wise. Immunization: occurs in the US/ Japan/Australia and other countries, but is not routine in the UK. Rea- sons not to vaccina te include paradoxically increasing shingles/chickenpox in adults 342 and lack of cost-eff ectiveness. 343 Dangerous contexts: Immunosup- pression; cystic \201 brosis; severe eczem a; neonates. \201\227: Keeping cool may reduce lesion numbers. Calamine lotion soothes. Tr im nails to lessen damage from scratching. Consider daily antiseptic for spots (chlorhexidi ne). Flucloxacillin 125 \200\223250mg/6h PO if bacterial superinfection \200\224treat for s epticaemia if worsen- ing. Antivaricella-zoster immunoglobulin (if \211 10 days post-expos ure) + aci- clovir if immuno-suppressed or on steroids (it \200\231s licensed as a 7-day co urse in chickenpox); begin within 24h of the rash. In renal failure, \200\201 dose. There is no clear evidence on aciclovir \200\201complications if immunocompetent, but it may hel p severe symptoms, eg in adolescents, or 2nd or 3rd family contacts. If used, use at the 1s t sign of infection, or as a 7-day attenuating dose of 10mg/kg/6h starting 1wk post-exposur e. Famciclovir is less well-studied. 344 Shingles Treatment: Oral analgesia. Ophthalmic shi ngles: p420. Aciclovir may reduce progression of zoster in the immunocompromised (may be ra mpant, with pneumonitis, hepatitis, and meningoencephalitis). Aciclovir IVI dose: 10mg/kg/8 h (over 1h), with concentration <5mg/mL, over >1h. Varicella in pregnancy Pneumonitis and e ncephalitis are no commoner in pregnancy, despite pregnancy being an immunocompromised stat e (1 in 400 and 1 in 1000, respectively). Infection in the 1st 20 weeks (esp. 13 \200\22320 weeks) may cause varicella zoster virus (VZV) fetopathy in 2%. 345 Signs of vzv fetopathy are variable, eg cerebral cortical atrophy and cerebellar hypoplasia, manifest- ed by micro cephaly, convulsions and IQ \200\201; limb hypoplasia; rudimentary digits pigmented scar s. Maternal shingles is not a cause. If the mother is aff ected from 1 week before to 4 wee ks after birth, babies may suff er severe chicken- pox. Give the baby zoster immunglobulin 250mg IM at birth; if aff ected, isolate from other babies, and give aciclovir. 144 \200 s c i r t a d e a P i Infection is preventable by pre-pregnancy vaccination with live varicella vaccine, 346 but testing for antibodies pre-conceptually is expensive, and cost- eff ectiveness depends on l ocal rates of seronegativity. 80% of those who can- not recall any previous chickenpox are , in fact, immune. 347 Varicella zoster globulin prevents infection in 50% of susceptible contacts, eg 1000mg IM ( adults). Infection in pregnancy merits aciclovir (it \200\231s probably OK for the fetus). Chickenpox at birth is a problem. Barrier nursing mothers causes distress and is of unprove n value. Infant mortality: up to 20%. 348 145 Vertical HIV infection Prenatal/labor: p34/p 23, adult HIV: OHCM p408 In many sub-Saharan c ountries, 40% of all under-5 mortality is a result of AIDS. If an HIV +ve mother breastfee ds, this \200\200risk of vertical transmission by 50%. Moth- ers with HIV should bottle f eed in the UK, but in countries without reliable clean wa- ter, breastfeeding is less risky than bottle feeding. 349,350 Infection can occur from the 1st trimester; 50% of infection s occur at the time of delivery, and are more likely if mothers have symptomatic HIV infect ion or a high viral load1. Transmission rates with full intervention (ie antiretrovirals ar ound birth, cesarean section, no breastfeeding) are <5%. In a cohort of 330 HIV +ve mothers , those with the p24 an- tigen had a 3-fold risk of transmission compared with those who we re p24 \200\223ve. For cesarean section and zidovudine in preventing vertical transmission , see p34. PCP (below). CMV may be fatal in infants whose mother \200\231s HIV status wasn \200\231t recognized in pregnancy. 351 Diagnosing vertically acquired HIV-1 Don \200\231t u se standard tests (placentally transferred IgG lasts for \211 18 months). Virus culture an d PCR are reliable, and more sensitive than \201 nding p23 antigen in blood. HIV-speci \201 c IgA doesn \200\231t cross the placenta, but only occurs in 50% of infected infants < 6 months old. Discuss with lab. \200\207Aim to diagnose 95% of infected infants be- fore t he age of 1 month. Monitoring CD4 counts (OHCM p413) helps in staging HIV. The all clear ca n only be given if all tests are negative at 18 months. Consider HIV in children with: PUO; lymphadenopathy; hepatosplenomegaly; persistent diarrhea; parotid enlargement; shingles; e xtensive molluscum; platelets \200\201; recurrent slow-to-clear infections; failure to thri ve; clubbing, un- explained organ disease; 352 TB; pneumocystosis; toxoplasmosis; cryptococ co- sis; histoplasmosis; CMV; LIP (below). Suspect non-vertical HIV seroconver- sion illnes s if: T \200\200, fatigue, rash, pharyngitis, lymphadenopathy, oral ulcers, D&V, headache , myalgia, arthralgia, meningism, peripheral neuropathy, thrush, weight \200\201, night swe ats, genital ulcers, WCC \200\201; platelets \200\201; transaminases \200\200. 353 HIV & th e lung: TB; lymphocytic interstitial pneumonia (LIP), immune reconsti- tution in \202 amma tory syndrome, malignancy, bronchiectasis. 354 LIP: tachypnoea; hypoxia; clubbing; diff use reticulonodular in \201 ltrates on CXR; bilateral hilar lym- phadenopathy. It is not AIDS -de \201 ning. It is less serious than pneumocystosis. Prognosis By 3yrs old, up to half w ith early-onset opportunistic infection have died vs 3% of those with no such infection. Ch ildren with slow progression of HIV have persistent neutralizing antibodies. Transplacental passage of mater- nal neutralizing antibody may also have a role. Guidelines for preventin g pneumocystosis (PCP) with co-trimoxazole Age/HIV status 0 \200\2235wks, HIV exposed 5wks \200\2234 months, HIV exposed 4 \200\22312 months: \200\223HIV infected \200 s c i r t a i d e a P PCP prophylaxis No No Yes Yes No Yes, if CD4 <500/ L* Yes, if CD4 <200/ L* *or CD4 %age < 15% Do CD4 cell counts at: 1 month 3 months 6, 9, and 12 months 6, 9, and 12 months No monitori ng needed At least every 3 \200\2234 months At least every 3 \200\2234 months \200\223Status unknown \200\223No HIV infection 1 \200\2235yrs, HIV infected 6 \200\22312yrs, HIV infected Ensure full course of vaccines (+Pneumococcus; avoid live vaccines if very im- munocompromi sed, and BCG if symptomatic and TB prevalence is low). 355,356 Highly active antiretroviral therapyHAART Use PENTA regimen. Pediatric Eurpean Network for the Treatment of AIDS 357 (O HCM p413). Those with AIDS-de \201 ning conditions or CD4 <15% (esp. if falling) should st art HAART at once. If few symptoms and CD4 stable at >20% get advice. 358 Obstacles: Poor a dherence (unpleasant tasting pills); SE (lipids \200\200, glucose \200\200, bone metabolism \200\201); lack of family routines. 359 Diarrhea-related morbidity: 360 micronutrient (eg Zn) zinc supplementation helps. 361 \200\207Teach HIV+ve children about safe sex and other HIV issues before puberty. 1 NB: it is perfectly possible for a woman who has vertical HIV to produce HIV \200\223ve babies. Non-accidental injury (NAI) \200\207Involve social services today when issues of abuse ari se. Read what follows with local child protection and NICE guidelines, 362 and relevant leg islation in your country, eg in England, the Children \200\231s Act (states that the child \200\231s welfare is always paramount). Abuse may be physical, sexual, emotional, or by neg lect. In Munchausen \200\231s by proxy, a parent fabricates alarming symptoms in their chil d to gain attention via dangerous interventions. Risk factors: Birthweight <2500g; mother < 30yrs; unwanted pregnancy; stress; poverty. 363 Prevalence: 1% of A&E work. 364 Suspect abu se if: Disclosure by child, or: \200 Odd story, incongruent with injuries; odd mode of in jury; odd set of signs. \200 Late visit to an unknown doctor, or taken by someone who is not a parent. \200 History inconsistent with the child \200\231s development. Can the bab y really walk? \200 Eff orts to avoid full examination, eg after an immersion burn. \200 Psychological sequelae (stress; depression) from sexual or emotional abuse.1 \200 Unexp lained fractures, eg forearm or rib (esp. posterior, from squeezing). 365 \200 Buttock, p erineum or face injury; intracranial bleeds; 366 torn lingual frenulum; vitreous/retinal bl eeds, hyphaema (p452), lens dislocation, bulging fontanelle, head circumference \200\200 xanthochromia; \200\207if in doubt, do CT. Also: Cigarette burns; whip marks (outline of belt/buckle, or double electric \202 ex); bruised non- mobile baby; signs of suff ocation ; \201 ngermark bruising; perforated pharynx. 367 \200\207NB: site or type of fracture c an never be relied on to distinguish abuse from an accident (extraordinary things, even spi ral fractures, can happen in play). \201 \201 : Osteogenesis imperfecta; osteoporosis, eg from propionic acidaemia. 368,369 Dangerous questions Although non-medical, these need add ressing because we are not simply technicians following protocols, and they in \202 uence what we do, how we react to child abuse, and how society perceives our role. \200 Could p roving of abuse be more destructive than the abuse itself?1 Note that 146 \200 s c i r t a d e a P i even if the answer is yes, society places a duty on us to report abuse. Is it better for him to be loved and battered than neither? \200 Is help from the extende d family more desirable than the law? Is it possible that the parents can grow through cris is, as abuse is discussed and help given? Remember that the \201 rst aim is to prevent or gan damage, murder, and other signi \201 cant harm. If this is a real danger \200\207cont act the duty social worker to- day \200\224eg for an emergency protection order. Off er hel p to the parents. Learn to listen, leaving blame and punishment to judges. Find out about l ocal policies and referral routes. Remember that often our duty is not to diagnose child ab use, but to recognize possible abuse, and then to get help. Sexual abuse This may be preven ted by teaching about \200\230personal safety \200\231 and how to say \200\230No \200\231 . Know your local guidelines. Follow them. Inform Social Ser- vices. If you do not, ask you rself with whom you are colluding. Forensic speci- mens (eg pubic hair, vaginal swabs) are to be taken by an expert who knows how to be gentle, and to avoid a \200\230second rape \200\231. Prepubertal venereal disease means abuse until proven otherwise. Does abuse cause psychological harm? Yes, usually, but the position is complex. See p323. Repertoire of act ions in primary care \200\207After informing Social Services, li- aise with health visitor (may be a very helpful source of information) or NSPCC (National Society for the Preventio n of Cruelty to Children). \200 Admission to a place of safety (eg hospital or foster hom e). \200 Continuing support for parents and protection for siblings. \200 Prevention: e ncourage impulses to be shared, and not acted on. \200 Attend a case conference (social w orker, health visitor, pediatrician; police).2 1 K Hulme 1984 Bone People. This novel tests Samuel Johnson \200\231s aphorism that \200 \230it is better that a man should be abused than be forgotten \200\231. Read it before mak ing quick judgments about families. 147 A possible sequence of events might be: Unexplained signs (or disclosure, or allegations), eg odd bruising \200\201 \200\230Testi ng of professional hypotheses \200\231 \211\210 weighing it up in your own mind \200\201 \200\230Clari \201 cation by discussion with an experienced colleague \200\231 \211\210 tell your boss \200\201 \200\230Reach a critical threshold of professional concern \200 \231 \211\210 you \200\231re both worried \200\201 \200\230Weigh the pros and cons of br eaking con \201 dentiality \200\231 \211\210 you must breach con \201 den- tiality if do ing so prevents a serious crime against a child. \200\201 \200\230Sharing concerns with s tatutory agencies \200\231 \211\210 phone Social Services/police \200\201 \200\230Act wi thin a timeframe not detrimental to the child \200\231 \211\210 aim to do it now \200\201 \200\230Contemporaneous records detailing all your sources \200\231 \211\210 write it dow n now \200\201 \200\230Preliminary consultation with all concerned \200\231: don \200\231 t promise to keep secrets \200\201 \200\230Strategic multidisciplinary discussion \200 \231 \211\210 is an abuse investigation needed? \200\201 If so, \200\230Instigation of c hild abuse investigation \200\231 \211\210 plan case conference \200\201 \200\230Must pa rents/child be present? \200\231 \211\210 bend the ear of the conference chairman \200 \201 Tell parents & child (if appropriate) what your report to conference will be \200\201 Case conference timed to let doctors ful \201 l their major role \211\210 ?get a locum \200\201 Register your dissent (if any) to the conference conclusions in its minutes \200 \201 Child is placed on a Register indicating that questions of abuse are unresolved and th at the child remains at risk \200\201 \200\230Establish networks for information exchange , discussion & advice \200\231 \211\210 follow up by Social Services, or a national societ y protecting children from cruelty (NSPCC) \200\201 Second (review) conference to weigh ne w evidence \200\201 Death of a child \200\201 Agencies must issue reports to the Area Chi ld Protection CommitteeUK \200\201 Judge issues life sentence ( \200 breadwinner removed from family for 10yrs) \200\201 No \200 role model for siblings \211\210 perpetuation o f cycles of poverty and abuse \200\201 Unexplained bruising in a member of the next genera tion \200\201 Man hands on misery to man* s c i r t a i d e a P M an hands on misery to man. It deepens like a coastal shelf. Get out as early as you can, And don \200\231t have any ki ds yourself Philip Larkin, This Be The Verse Not all our eff orts to protect children end thus. Success es are frequent. And of course this sequence oversimpli \201 es \200 the sign \211\210 is not meant \202 ippantly: it is intended as shorthand, denoting the exercise of re \202 ection, good judgment, action, and the following of agreed procedures. 2 The UK Families at Risk Review (2008) speci \201 es joined-up agencies that \200\230ens ure \200\231 that there is \200\230no wrong door \200\231 that families knock on \200\224a ll doors lead to getting the correct help. Also: \200 Con \201 rm social services referr als in writing \200 Question other \200\231s opinions if you disagree. \200 Document al l phone-calls \200 Record dis- cussions if there is disagreement over risk of deliberate harm. Sudden unexplained infant death (SUID/SIDS) De \201 nition Sudden unexplained infant death re- fers to unexpected death <12 months where a cause is not immediately obvious prior to investigation. It includes deaths due to infection, metabolic errors, accidental suff ocati on and SIDS. Sudden infant death syndrome (SIDS) is \200\230Sudden death under 12 months o ld, which is unexpected after a thorough case investiga- tion, including a complete autopsy , examination of the death scene, and review of the clinical history. \200\231 \200\207SID S is the leading \200\230cause \200\231 of death in infants aged over 1 week old. Epidemio logy Peak incidence: 1 \200\2234 months; risk \200\200 if: poor, parents are smokers, baby is male or premature, winter, previous sibling aff ected by SIDS; co-existing minor upper r espiratory infection is common, co- sleeping. There are many causal theories (see MINIBOX). 148 \200 Putative causes: Obstructive apnoea: \200 Inhalation of milk \200 Airways oedema \200 Passive smoking Central apnoea: \200 Faulty CO2 drive \200 Prematurity \200 Brainste m gliosis Others: \200 Long Q \200\223T interval1 \200 Staph infection2 \200 Overheat ing \200 \200\200Vagal tone or Mg2+ \200\200 \200 Immature diaphragm \200 Genetic & viral causes Sleeping supine ( \200\230back to sleep \200\231, even for short naps), 370 preventing over heat- ing, and cigarette smoke exposure are the chief preventive interventions: risk from p assive smoking is dose-dependent, and often at least doubles risk. 371 s c i r t a d e a P The face is an important platform for heat loss \200\224and it is known that the incidence of SIDS is 5 \200\22310-fold higher among infants usually sleeping prone (17- fold higher if sleeping in a room separated from parents): \200\207so always recom- mend sleeping supi ne. Advise as follows: \200 Do not overheat the baby \200\231s bedroom. Aim for a tempera ture of 16 \200\22320 C. \200 Do not use too much bedding, and avoid duvets if less than 1 year of age. \200 If ill or feverish, consult a GP \200\224do not increase the amount of bedding. \200 Have feet come down to the cot \200\231s end to avoid under-blankets mig ration. \200 While sleeping, avoid heaters, hot water bottles, electric blankets, and hat s. Do wrap up for trips out in winter, but unwrap once indoors, even if this means disturbi ng the baby. Never tuck in blankets higher than the armpit. 372 \200 Babies >1 month do n ot need to be kept as warm as in hospital nurseries. \200 Avoid co-sleeping if possible a nd never, even if very tired (new parents!), if parents are deep sleepers, or if they have had any alcohol or drugs. 373,374 Autopsy is unrevealing; minor changes are common; petechi al hemorrhages over pleura, pericardium, or thymus, and vomit in the trachea may be agonal events. Causes to exclude: sepsis, metabolic defects (eg MCAD de \201 ciency; me- dium-cha in acyl-CoA dehydrogenase \200\201, p183); heart defects; and always bear in mind the possi bility of murder. Action after failed resuscitation in the Emergency Department \200 Docu ment all interventions, venepuncture sites, and any marks on the baby. You don \200\231t ha ve to keep all tubes in situ, but ensure that someone who did not intubate con \201 rms en dotracheal placement of the tube before extubation. i Take samples of blood for culture, CSF for culture/PCR, urine, and stool. \200 Keep all c lothing and the nappy. \200 Explain clearly to parents that despite your best eff orts, t he baby has died. \200 Unless the cause is obvious, be non-committal about cause of death . Explain the baby must have a post mortem (this is a coroner \200\231s case). Contact the consultant on call, the police, child protection team, and the coroner at once; also GP, health visitor, and any other involved professions. 1 Risk \200\200 if Q \200\223T corrected for rate (QTc) \200\206440msec. QTc=(Q \200\223T) /( \200 R \200\223R.375,376 LQTS genes are important.377 2 Staphs in mattress foam are impl icated ( \200\230 do not reuse); Jenkins 2005378,379