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BIRTHMARKS: 1.

Mongolian spots - stale gray or bluish discoloration patches commonly seen across the sacrum or buttocks due to accumulation of melanocytes. Disappear by 1 yr old 2. MIlla – plugged or unopened sebaceous gland . white pin point patches on nose, chin or cheek. 3. Lanugo – fine, downy hair – common preterm 4. Desquamation – peeling of newborn, extreme dryness that begin sole and palm. 5. Stork bites (Talengeictasi nevi) – pink patches nape of neck hair will grow as child grows old 6. Erythema Toxicum – (flea bite rash) - 1st self limiting rash appear sporadically & unpredictably as to time & place. 7. Harlequin sign – dependent part is pink, independent part is blue (side lying – bottom part is dependent pink) 8. Cutis Marmorata – transitory mottling of neonates skin when exposed to cold. 9. Hemangiomas – vascular tumors of the skin 3 types Hemangiomas a.) Nevus Flammeus – port wine stain – macular purple or dark red lesions seen on face or thigh. NEVER disappear. Can be removed surgically b.) Strawberry hemangiomas – nevus vasculosus – dilated capillaries in the entire dermal or subdermal area. Enlarges, disappears at 10 yo. c.) Cavernous hemangiomas – communication network of venules in SQ tissue that never disappear with age. - MOST DANGERIOUS – intestinal hemorrhage. Mgt for burns 1.) 1st aid a.) put out flames by rolling child on blanket b.) immerse burned part on cold H2o c.) remove burned clothing of with sterile material d.) cover burn with sterile dressing 2.) Airway a.) suction PRN, o2 with increased humidity b.) endotracheal intubation c.) tracheostomy 3.) Preventiuon of shock & F&E imbalance a. colloids to expand bld volume b. isotonic saline to replace electrolytes

c. dextrose & H2o to provide calories 4.) Tetanus toxoid booster 5.) Relief of pain – IV analgesic MORPHINE SO4 – needed for 2nd degree – very painful 6.) 1st defense of body – intact skin - prevention of wound infection a) cleaning & debriding of wound b) open or close method of wound care c) whirlpool therapy – drum with solution 7.) skin grafting – 3rd degree – thigh or buttocks (autograft), pigs/ animals xenograft frozen cadaver – hallow graft 8,) diet – increase CHON, increase calories. ATOPIC DERMATITIS - infantile eczema (galis) Papulo vesicular erythematus lesions with weeping & crusting .Cause – food allergies: milk, citrus juice, eggs, tomatoes, wheat Sx: - extreme pruritus, linear excoriation, weeping crusting; scaly shiny and white –lechenification Goal of care: decrease pruritus – avoid food allergens Diet: Prosobi or Isomil Hydrate skin, borow solution 1% hydrocortisone cream Prevent infection – proper handwahsing, trim nails IMPETIGO - skin disease. Causative agent – grp A beta Hemolytic streptococcus – papulovesicular surrounded by localized erythema – becomes purulent , oozes a honey colored crust. Pediculosiscapitis –“KUTO” Mgt: proper hygiene – wash soap and H2o, oral penicillin – bactroban ointment Can lead to acute glomerulonephritis AGN ACNE - adolescent problem – self limiting infl dis – sebaceous gland comedones – sebum causing white heads – sebum- lipids causing acne bulgaris Mgt: - proper hygiene- mild soap or sulfur soapantibacterial retin A or tretinoin ANEMIA-pallor Causes: 1.)early cutting of cord – preterm – cut umb cord ASAP fullterm – cut umb cord when pulsation stops 2.) Bleeding disorders – blood dyscrasias HEMOPHILIA

– deficiency of clotting factor. X linked recessive – inherited If mom – carrier, son – affected If father carrier- transmitted to daughter Hemophilia A – deficiency of coagulation component factor 8 Hemophilia B –or christmas disease, deficiency of clotting factor 9 Hemophilia C – deficiency of clotting factor 11 Assessment: – umphalagia – earliest sign – newborn receive maternal clotting factor – newborn growing – sudden bruising on bump areamarks earliest sign – continuous bleeding – hematrosis – damage or bleeding synovial membrane Dx test : PTT. Partial thromboplastin time – reveals deficiency in clotting factor Long Term Goal- prevention of injury Nsg Dx- increase risk of injury HT: avoid contact sport, swimming only, don’t stop immunization – just change gauge of needle Falls – immobilized , elevate affected part, apply pressure-not more then 10 mincold compress -determine case before doing invasive procedure LEUKEMIA- grp of malignant disease – rapid proliferation of immature WBC – WBC – protection from infection, soldiers of body Classification : 1. Lympho – affects lymphatic system 2. Myelo – affects bone marrow 3. acute / blastic- affects immature cells 4. chronic/ cystic- affects mature cells MOST COMMON CANCER– (ALL) – Acute Lymphocytic Leukemia S&Sx: 1. from invasion of bone marrow signs of infection a) fever b) poor wound healing c) bone weakness & causes fracture signs of bleeding a) petecchiae-small, round, flat, dark red spot b) epistaxis c) blood in urine/ emesis signs of anemia a) pallor , body malaise , constipation from invasion of body organ- hepato spenomegaly – abd pain , CNS affectation, increase ICP Dx Tests: 1. PBS- peripheral blood smear immature RBC 2. CBC – determine anemia, thrombocytopenia neutropenia – determine

3. lumbar puncture (LP) – determine CNS involvement. Before LP, fetal pos.- avoid flexion of neck – will cause a/w obstruction.“C” position or shrimp position only. 4. bone marrow aspiration – determine blast cells, – common site- iliac crest – post BMA s/effect – bleeding – apply pressure. Put pt on affected side to prevent hemorrhage 5. Bone scan – determine bone involvement 6. CT scan – determine organ involvement Therapeutic Mgt: TRIAD: 1. surgery 2. irradiation 3. chemotheraphy Focus Nsg Care: prevent infection 4 LEVELS OF CHEMOTHERAPHY 1. induction – goal of tx; to achieve remission meds: IV vincristine L- agpariginase Oral predinisone 2. Sanctuary- treat leukemic cells that invaded testes & CNS give: methotrixate- adm intrathecally via CNS or spine cytocine, Arabinoside, steroids with irradiation 3. maintenance- to continue remission give: oral methotrisate – check WBC -adm of methotrisate – do weekly WBC check 4. Reinductin – treat leukemic cells after relapse occurs. Meds – same as induction - give antigout agents: allopurinol or Zyloprim- treat or prevent hyperurecemic nephropathy. Nsg mgt: Outstanding nsg dx: alteration in nutrition less body requirement. Based on Maslow’s heirarchy S/Effect of Chemotherapy 1. N/V – adm antiemetic drugs 30 mins before chemo until 1 day after chemo 2. Ulcerations / stomatitis / abscess of oral mucosa(alteration nutrition less body req) - oral care – alcohol free mouthwash , betadine mouthwash - don’t brush – use cotton pledgets - topical xylocaine before meals diet- soft, bland diet according to child’s preference Temporary S/E of chemo: Alopecia – altered body image Hirsutism – hair -give emotional support to parents

ABO incompatibility Most common incompatibility – ( mom) O – ( fetus) A Most severe incompatibility (Mom) O– (Fetus) B Can affect 1st pregnancy Hydrops (h20) Fetalis– edematous on lethal state with pathologic jaundice Within 24 h

leukocytosis,

Mgt: 1. initiate breastfeeding to get colostrum 2. Temp suspension of breastfeeding – content breast milk pregnanedioles – that delays action of glucoronil transferees liver enzymes converts in direct bilirubin to become direct bilirubin 3. Needs phototherapy 4. needs exchange therapy Hyperbilirubinemia - > 12 mg/dL of indirect bilirubin among full term Normal – 0-3 mg/dL – bilirubin encephalopathy – Kenicterus - > 20 mg/dL among full term & >12 mg /dl of indirect – preterm =can lead to cerebral palsyPhysiologic jaundice – jaundice within 48 -72 h (23 days) expose morning sunlight Pathologic Jaundice – within 24h. Jaundice during delivery. Breastfeeding jaundice – caused by pregnanediole Assessment of Jaudice : 1. Blanching neonates forehead, nose or sternum - yellow skin & sclera - color of stool – light stool - color of urine – dark urine Mgt: Phototheraphy – photo oxygenation Nsg Resp: 1. cover eyes – prevent retinal damage 2. cover genitals – prevent priapism – painful continuous erection 3. change position regularly – even exposed to light 4. increase fld intake – due prone to dehydration 5. monitor I&O – weigh baby 6. monitor V/S – avoid use of oil or lotion due- heat at phototherapy = bronze baby syndrome-transient phototherapy weigh diaper 1gm = 1cc Head – largest part of baby ¼ of its length Craniostenosis or craniosinustosis – premature closing of fontanel Hydrocephalus – ant fontanel open after 18 mos Microcephaly – small growing brain due- alcohol & HIV mom Anencepahly – absence of cerebral hemisphere Craniotabes – localized softening cranial bone. Common – 1st born child -due early lightening (2 weeks prior to EDD) Rickets of Vit B deficiency – soft cranial bone in older children Caput Succedaneum – edema of scalp due prolonged pressure at birth Char: 1. present at birth 2. crosses suture lines 3. disappear after 2-3 days Cephalhematoma S/E of

- collection of blood due to rapture of pericostal capillaries Char : 1. present after 24 h 2. never cross suture line 3. disappear after 4-6 weeks 4. monitor for developing jaundice Seborrheic Dermatitis – ‘craddle cap” Scaling, greasy appearing salmon colored patches – seen on scalp behind ears and umbilicus Cause: - improper hygiene Mgt: 1. proper hygiene 2. put oil night before shampoo – baby oil Hydrocephalus – excessive accumulation of CSF 1. communicating – extra ventricular hydrocephalus 2. non-communicating- intraventricular hydrocephalus or obstructive hydrocephalus due to tumor obstruction Sx – ICP – abnormally large head, bulging fontanel – cushings triad – high pitched cry older child – diplopia – eye deviation, projectile vomiting – fontanel bossing – prominent forehead – prominent skull vein – sunset eyes Mgt: position to lessen ICP – low semi-fowlers 30 degree angle Administer- osmotic diuretic Mannitol/ Osmitrol , Diamex- Azetam Decrease CSF production Shunting – AV shunt or Vp shunt (ventriculoperitoneal shunt) Shave hair – in OR – to prevent growth of micro org. Nsg Care: 1.) post VP shunt – side lying on non operated site to prevent increase ICP monitor for good drainage - sign – sunken fontanel bulging fontanel – blocked shuntchange fontanel as child is growing SENSES EYES: Assessment 1. check for symmetry 2. sclera – normal color – light blue then become dirty white pupil – round- adult size coloboma- part of iris is missing sign: key hole pupil whiteness & opacity of lens congenital cataract cornea – round & adult size large – congenital glaucoma

Test for blindness common tests 1. newborn – general appearance - can only see 10 – 12 “ - visual acuity 20 /200 to 20/ 800 Doll’s eyes test- test for blindness – done 10th day – pupil goes opposite to direction when head is moved Globellars test – test for blink reflex. Points near nose – baby should blink 2. Infant & children - appearance - ability to follow object past midline 3. 3 yrs – school age - general appearance Allen cards – test for visual acuity. Show picture 20 ft away Ishiharas plates – test for color blindness Prechool E chart - test for stereopsis of depth perception Cover testing test – cover 1 eye for 10 – 15 min. Then remove. Test for strabismus 4. School age – adult - general appearance - snellens test Retinobastoma – malignant tumor of retina Outstanding sign : oat’s eye reflex-whitish glow of pupil – red painful eye – blindness surgery – Enucliation – removal of eyeball put artificial aye NOSE: 1. flaring alenase – case of RDS 2. cyanosis at rest – choanal atresia - post nares obstructed with bone or membrane Sx: 1. resistance during catheter insertion 2. emer. Surgery within 24 h normal color nasal membrane – pinkish rhinitis – presence of creases & pale check sense of smell – blindfold – smell Hair in nose – cilia Adolescent no hair with ulceration of nasal mucosa suspect cocaine user Epistasis – nosebleed – sit upright, head slightly forward to facilitate drainage – cold compress , apply gentle pressure, epinephrine most developed sense of newborn – sense of touch 1stsense to develop & last to disappear – hearing EARS: Properly aligned with outer cantus of eyes low set ear – kidney malformation ex. Renal aginesis – absence of kidney sign in uterus : oligohydramnios sign in newborn: 2 vessel cord failure to void within 24 h Mgt: kidney transplant Chromosomal aberrations : -advance maternal age

1. non disjunction – uneven division Trisomy 21 - down syndrome - extra chromosome 47xx + 21 - related to advance paternal age Sx: Mongolian slant Broad flat nose Protruding neck Puppy’s neck Hypotonic – prone to resp problem Simean crease – single transverse line on palm. Trisomy 18 – “endvard syndrome” Trisomy 13- patau syndrome Turner – Monosomy of X synd. – 45x0 – affected girls – signs evident during puberty – has poorly developed 2dary sexual char. - Sterile Klinefelters Syndrome- has male genitalia - 47 XXY - poorly devt secpndary sexual characteristics - no deepening of voice -small testes, penis -sterile Klinefelter – Calvin Kline – male Turner – Tina Turner – female Otitis Media – inflammation of middle ear. Common children due to wider & shorter Eustachian tube Causes 1) bottle propping 2) Cleft lip/ cleft palate – Sx: Otitis 1. bulging tympanic membrane, color – pearly gray 2. absence light reflex 3. observe for passage of milky, purulent foul smelling odor discharge 4. observe for URTI Nsg Care: 1. position side lying on affected aside – to facilitate drainage 2. supportive care- bedrest, increase fld intake Med Mgt: 1. Massive dosage antibiotic Complication – bacterial meningitis 2. Apply ear ointment School age – up and down < 3 yo – down & back > 3 yo – up & back Small child – down & back ( no age) surgery (to prevent permanent hearing loss)– otitis media – myringotmy with tympanostomy tube post surgery – position affected side for drainage both – put ear plug if tympanous tube falls – healed na Bells Palsy - facial nerve #7 paralysis R/T forcep delivery Sx. 1. Continuous drooling saliva 2. inability to open , eye & close either eye

Mgt: Refer to PT TEF (Tracheoesophageal Fistula)-TEA- no connection bet esophagus and stomach Outstanding Sx – Coughing Choking Continuous drooling Cyanosis Mgt: Emergency surgery Epstein pearl – white glistering cyst at palate & gums related to hypercalcemia

3. prevent colic feed – upright seating or prone pos burp frequently 2x at middle and after feeding-lower to upper tap 4. orient parents to type of feeding rubber tipped syringe – cheiloplasty paper cup/ soup spoon/ plastic cup – urano plasty 5. apply restraints – elbow restraints so baby can adjust post op Condition that warrants suspension of operation – colds & pharyngitis = can lead to generalized infection – septicemia Post Op Nsg Care : 1. airway – positon post cheilopasty – side lying for drainage post uranoplasty (tonsillectomy)- prone 2. assess for RDS sx bleeding 3. assess for bleeding – freq swallowing. 6-7 days after surgery – bleeding 4. proper nutrition – clear liquids- ( gelatin except red or brown color due may mask bleeding) – ( popsicle- not ice cream) full liquid soft diet regular diet 5. Maintain integrity of suture line such as: Logan bar – wash ½ strength Hydrogen Peroxide & saline solution- Bubbling effect traps microorganism - prevent baby form crying for pain- analgesic NECK1) check symmetry Congenital torticolis- “ wryneck”-burn injury of sternocleidomsstoid muscle during delivery – due to excessive traction at cephalic delivery Mgt: passive stretching exercise , Surgery Complication – scoliosis THYROID gland – for basal metabolism Congenial cretinism – absence or non functioning thyroid glands Reasons for delaying dx: 1. Thyroid glands covered by sternocleidomastoid muscles in newborn 2. baby received maternal thyroxine 3. baby sleeps 16 – 20 h a day earliest sign: 1. change in crying 2. change in sucking 3. sleep excessively 4. constipation 5. edema – moon face late sign 1. mental retardation prognosis : mental retardation preventable when Dx is early Dx: 1. PPI-protein 2. radioimmunoassay test

Moniliasis – oral candidiasis – white cheese like, curd like patches that coats tongue – oral thrush – Nsg Care – don’t remove, wash with cold boiled H2o Meds – Nystatin / Mysnastatin – antifungal Kawasaki Dse --strawberry tongue - originated in Korea - Dr. Kawasaki discovered it - common in Japan - “mucocutaneous Lymphnode Syndrome” Sx: -persistent fever – 5 days -strawberry tongue , -desquamation of palm & sole - lymph adenopathy > 1.5 cm Drug: aspirin Can lead to MI LIPS- symmetrical Cleft lip– failure of median maxillary nasal process to fuse by 5-8 wks of pregnancy – common to boys – unilateral Cleft Palate- Failed palate to fuse by 9 of pregnancy – common to girls – unilateral or bilateral Sx: 1. evident at birth 2. milk escapes to nostril during feeding 3. frequent colic & otitis media or URTI Mgt: 1. Surgery cleft lip repair – Cheiloplasty

– 12 wks

=done

1-3

months
months )

to save sucking reflex (lost in 6

Cleft Palate- uranoplasty = done to save speech

4-6 months

Pre op care 1. emotional support especially to mom 2. proper nutrition

3. radioactive iodine uptake Mgt: synthroid – sodium Levothyrosine -synthetic thyroid given lifetime – check pulse rate before giving synthroid – tachycardia – Sx of hyperthyroidism CHEST 1. symmetry 2. breast - transparent fluid coming out from newborn related to hormonal changes3. chest has retroactive – RDS 4. sternum sunken – pectus excavation ABDOMEN (in order) 1. inspection I 2. Auscultation A 3. percussion P 4. Palpation P = Will change bowel sounds, so do last Normal contour of abd – slightly protruding Sunken abd- diaphramatic hernia – protrusion of stomach content through a defective diaphragm due to failure of puroperitoneal canal to close. Sx: 1. sunken abd 2. Sx of RDS 3. R to L shunting Mgt: Emergency surgery within 24h Omphalocele – protrusion of stomach contents in between junction of abd wall and umbilicus. Mgt- very small surgery If large – suspension surgery Nsg Mgt: protect sac- sterile wet dressing Gastrochisis – absence of abd wall Nsg Mgt: sterile wet dressing Fx of GIT 1. assists in maintaining F&E & acid base balance 2. Processes & absorbs nutrients to maintain metabolism & support G & D 3. excrete waste products from digestive process Recommended Daily Allowance Calories : 120 cal / Kbw/day (kilo body wt) 360 – 380 cal/ day CHON_ 2.2g /Kbw/day Principles in Supplementary Feeding Supplementary Feeding usually – 6 mos Supplementary feeding given – 4 mos. a.) solid food offered to ff sequence! 1. cereals – rich in iron 2. fruits 3. veg 4. meat b.) begin with small quantities c,) finger foods – offered 6 months d.) soft table food – “modified family menu” given 1 yr e.) dilute fruit juices – 6 mos f.) never give half cooked eggs – usually causes of salmoneliosis g.) don’t give honey – infant botulism

h.) offered new food one at a time – interval of 4 – 7days or 1 week – determines food allergens Total Body Fluids- comprises 65 - 85% of body wt of infants & children Where fluids are greater in infants Extracellular fld – prone to develop dehydration Acid Base Balance dependent on the ff: a. chemical buffers b. renal & resp system involvement c. dilution of strong acids and bases in bld Resp Acidosis – carbonic acid excess – hypoventilation – RDS – COPD – Laryngotracheobronchitis (LTB) Resp Alkalosis – carbonic acid deficit – hyperventilation – fever – encephalopathy Met. Acidosis – base HCO3 deficit – diarrhea – severe dehydration – malnutrition – ciliac crisis Met Alkalosis – base HCO3 excess – uncontrolled vomiting – NGT aspiration – Gastric lavage PROBLEMS LEADIING TO F&E IMBALANCE 1. vomiting – forceful expulsion of stomach content Sx: 1. nausea 2. dizziness 3. facial flushing 4. abd cramping assess: amt, freq, force projectile vomiting= increase ICP or pyloric stenosis Mgt: BRAT diet - banana, rice – cereal, apple sauce, toast 2. Diarrhea – exaggerated excretion of intestinal contents Types: Acute diarrhea – related to gastroenteritis, salmoneliosis – dietary indiscretions – antibiotic use Chronic non specific diarrhea Cause: 1. food intolerance 2. excessive fld intake 3. CHO, CHON malabsorption Assess: freq, consistency, appearance of given colored stool. Best criteria to determine diarrhea : consistency Complication = dehydration Mild dehydration 5% wt loss Moderate dehydration 10% wt loss Severe dehydration 15 % wt loss Earliest sx of dehydration

tachycardia increase temp weight loss tachypnea sunken fontanel & eyeballs scanty urine hypotension absence of tears Severe dehydration: Oliguria , Prolonged capillary refill time Mgt: Acute – NPO ( rest the bowel ) – with fluid replacement – IV – prone to Hypokalemia – give K chloride before adm of K chloride – check if baby can void, if cant void – hypokalemia Drug: Na HCO3 – adm slowly to prevent cardiac overload Gastric Motility Disorder HIRSCHPRUNGS DISEASE – congenital aganglionic megacolon Aganglionic – absence of ganglion cells needed for peristalis Earliest sign 1. failure to pass mecomium after 24h 2. abd distension 3. vomitus of fecal material early childhood – ribbon like stool foul smelling stool constipations diarrhea Dx: 1. Barium enema – reveals narrowed portion of bowel 2. Rectal Biopsy – reveals absence of ganglionic cells 3. abd x-ray – reveals dilated loops on intestine 4. rectal manometry – revels failure of intestine sphincter to relax Therapeutic Mgt/Nsg care 1. NGT feeding – measure tube fr nose to ear to midline of xyphoid & umbilicus 2. surgery a) temp colostomy b) anastomosis & pull through procedure Diet: Increase CHON, increase calories , decrease residue – pasta

Meds of GERD Anti-cholinergic a) Betanicol ( urecholine) – increase esophageal tone & peristaltic activity b) Metachloporomide (Reglam) – decrease esophageal pressure by relaxing pyloric & duodenal segments – increase peristalsis without stimulating secretions c) H2 Histamine Receptor Antagonist – decrease gastric acidity & pepsin secretion – Zimetidine, Ranitidine (Zantac) – take 30 min before meals d) antacid – neutralizes gastric acid between feedings - Maalox Surgery: Nissen funduplication : Chronic vomiting – – thickened feeding with baby cereals - effective if without vomiting – feed slowly, burp often every 1 ounce – positioning < 9 months – infant sit with infant supine > 9 months – prone with head of mattress slightly elevated 30 degree angle OBSTRUCTIVE DISORDERS A. PYLORIC STENOSIS – hypertrophy of muscles narrowing & obstruction.

of

pylorus

causing

GER- Gastroesophageal Reflux Chalasia – presence of stomach contents to esophagus Will lead to esophagitis complication – aspiration pneumonia Esophageal cancer Assessment : 1. chronic vomiting 2. faiture to thrive syndrome 3. organic – organ affected 4. melena or hematemesis – esophageal bleeding Dx procedure 1. barium esophogram – reveals reflux 2. esophageal manometry – reveals lower esophageal sphincter pressure 3. intra esophageal pH content – reveals pH of distal esophagus

1.) outstanding Sx- projectile vomiting - vomiting is an initial sx of upper GI obstruction - vomitus of upper GI can be blood tinged not bile streaked. (with blood) - vomitus of lower GI is bilous ( with pupu) - projectile vomiting – increase ICP or GI obstruction - abd distension – major sx of lower GIT obst 2.) met alk 3.) failure to gain wt 4.) olive shaped mass – on palpation 5.)serum electrolyte – increase Na & K, decrease chloride 6.) ultrasound 7.) x ray of upper abd with barium swallow reveal “string sign” Mgt: 1. Pyleromyotomy 2. Fredet Ramstedt procedure INSTUSSUSCEPTION - invagination or telescoping of position of bowel to another Common site – ilio-cecal junction Prone pt: person who eats fat Complication – peritonitis – emergency Sx: 1) persistent paroxysmal abd pain 2) vomiting 3) currant jelly stool- dye bleeding & inflammation – palpate sausage shaped mass Mgt:

1) Hydrostatic reduction with barium enema 2) Anastomosis & pull thru procedura Inborn Errors of Metabolism- deficient liver enzymes Gluten – glutamine ( normal absorption) Gliadin ( toxic to epithelial cells of villi of intestines, effects is malabsorption syndrome) PHENYLKETONURIA(PKU) – deficiency of liver enzymes (PHT) Phenylalaninehydroxylase Transferase – liver enzyme that converts CHON to amino acid 9 amino acids: valine isolensine tryptophase lysine phenylalanine Thyronine – decrease malanine production 1) fair complexion 2) blond hair 3) blue eyes Thyroxine – decrease basal metabolism - accumulation of Phenyl Pyruvic acid 4.) Atopic dermatitis 5.) musty / mousy odor urine 6.) seizure – mental retardation Test – GUTHRIE TEST – specimen – blood – preparation increase CHON intake – test if CHON will convert to amino acid specimen and urine mixed with pheric chloride, presence of green spots at diaper a sign of PKU DIET: Low phenylalanine diet- food contraindicated- meats, chicken, milk, legumes, cheese, peanuts Give Lofenalac- milk with synthetic protein

1. diarrhea – failure to gain wt ff diarrheal episodes 2. constipation 3. vomiting Late Sx: 1. abd pain – protruberant abd even if with muscle wasting 2. steatorrhea Celiac Crisisinflammation exaggerated vomiting with bowel

Dx: 1. lab studies – stool analysis 2. serum antiglyadin – confirmatory of disease gluten free diet – lifetime all BROW – not allowed ok – rice & corn Mgt: 1. vitamin supplements 2. mineral supplements 3. steroids POISONING - common in toddlers. (falls- common to infant) 1. determine substance taken, assess LOC 2. unless poison is corrosive, caustic (strong alkali such as lye) or a hydrocarbon, vomiting is the most effective way to remove poison. – Give syrup 1 pecac to induce vomiting 3. 1 pecac – oral emetic – 15 ml – adolescent, school age & pre school – 10 ml to infant 4. UNIVERSAL ANTIDOTE- charcoal, milk of magnesia & burned toast 5. Never adm charcoal before 1 pecac 6. antidote for acetaminophen poisoning – acetylsysterine ( mucomyst) 7. caustic poisoning ( muriatic acid ) neutralize acid by giving vinegar . Don’t vomit Prepare tracheostomy set 8. Gas- mineral oil will coat intestine Lead poisoning Lead = Destroy RBC functioning = Hypochornic Microcytic Anemia = Destroy kidney functioning Accumulation of anemia = Encepalopathy Sx: 1. beginning sx of lethargy 2. impulsiveness, learning difficulties 3. as lead increases, severe encepalopathy with seizure and permanent mental retardation Dx: 1. Blood smear 2. abd x ray 3. long bones Mgt: 1. remove child from source 2. if > 20 ug/dL – need chelation therapy = binds with led & excreted by kidney

Galactosemia – deficiency of liver enzyme – GUPT – Galactose Urovil Phosphatetranferase – Converts galactose to phosphate tranferace glucose Galactose – will destroy brain cells if untreated – death within 3 days Dx: Beutler test – get blood -done after 1st feeding presence of glucose in blood – sign of galactosemia galactose free diet lifetime neutramigen – milk formula CELIAC DISEASE – gluten enteropathy Common gluten food: Intolerance to food with brow B- barley R- rye O- oat W- wheat Early Sx:

=nephrotoxic Amogenital Female: Pseudomenstration slight bleeding on vagina related to hormonal changes Tearing of fourchette with blood – rape/ child abuse Rape- Report within 48 h Shape pubic hair in inverted triangle ( female) Male: Undescended testes – cyrptorchidism -common to preterm surgery – orchidopexy assess scrotum- warm room & hands baby – pee within 24 h -check for arch of urination Epispadias- urinary meatus located dorsal or above glans penis Hypospadias- urinary meauts loc ventral or below glans penis Hypospadias with chordee- fibrous band causing penis to curb downward Mgt: Surgery Phimosis- tight foreskin Balanitis-infection of glands penis – due smegma Mgt: Circusicion Hydroseal – fld filled scrotum Tst of Dx: Transillumination with use of flashlight - glowing sign Varicoseal – enlarged vein of epididimis ( girlsvulvular varicosities)

AGN, autoimmune, Grp A Autoimmune Grp A beta hemolytic streptococcus 1. (PPP) primary peripheral periobital edema 2. moderate protenuria 3. gross hematuria( smokey urine) 4. serum K increased 5. fatigue 6. increase 1. anti HPNdrug - hydralazine or apresoline 2. iron 1. weigh daily 2. monitor BP & neurologiuc status 3. Diet: decrease K, decrease Na BPComplication : 1. hypersensive encephalopathy 2. anemia BACK- check for flatness & symmetry

Open Neural Tube Defect - decreased Folic Acid intake SPINA BIFIDA OCCULTA- failure of post laminae of vertebrae to fuse Sx: dimpling of back , Abnormal tufts of hair SPINA BIFIDA CYSTICA- failure of post laminae of vertebrae to fuse with a sac Types: 1. Meningocele – protrusion of CSF & Meninges 2. Myelomeningocele – protrusion of CSF & Meninges & spinal cord ( most dangerous) 3. Encephalocele ( CNS complication – hydrocephalus) – cranial meningocele or myelomeningocele Most common problem – rupture of sac – prone pos – sterile wet dressing Most common complication - infection Myelomeningocele – genitourinary urinary & fecal incontinence Nsg care: always check diaper Orthopedic complication – extremities Surgery to prevent infection Post op – prone position complication-

Renal Disorder Cause Sx Tx NSG CARE NEPHROTIC SYNDROME infectious 1. Anasarca -gen edema 2. massive protenuria 3. microscopic or non hematuria 4. serum CHON PrednisoneDiuretic Focus of care: monitor edema – weigh daily Diet: Increase CHON, Increase K- OJ, beef broth, banana Decrease Na decreased 5. serum lipid increased 6. fatigue 7. normal or decreased BP AGN ( acute Glomerulo Nephritis) 3A’s;

paralysis

of

lower

SCOLIOSIS- lateral curvature of the spine 2 types: 1. structural – rye neck 2. postural – improper posture Dx: 1. uneven hemline

2. bend forward- 1 hip higher 1 shoulder blade more prominent Nsg care: 1. conservative – avoid obesity, exercise 2. preventive – Milwaukee brace - worn 23 h a day 3. corrective surgery – insert Harrington rod post op- how to move log rolling- move client as 1 unit EXTREMITIES: check # of digits = 20 1. syndactyly – webbing of digits 2. polydactyly – extra digits 3. olidactyly – lack of digits 4. Amelia – total absence of digits 5. pocoamelia- absence of distal part of extremities ErQ duchennes– paralysis- brachial plexus injury or brachial palsy – birth injury caused by lateral & excessive traction during a breech injury Sx: 1. unable to abduct arms from shoulders, rotate arm externally or supinate forearm 2. absence or asymetrical moro reflex Mgt: 1. abduct arm from shoulders with elbow flex. CONGENITAL HIP DISLOCATION – head of femur is outside acetabulum Types; 1. subluxated – most common type 2. dislocated Sx: 1. shortening of affected leg 2. asymmetrical gluteal fold 3. limited movement – earliest sx 4. (+) ortolanis sign – abnormal clicking sound 5. when able to walk – child limps – late sxtrendelenburg sign Goal of Mgt: Facilitate abduction Mgt. 1. triple diaper 2. carry baby astride 3. Frejka splint 4. Pavlik harness 5. Hip Spica Cast TALIPES – “clubfoot” a) Equinos – plantar flexion – horsefoot b) Calcaneous – dorsiflexion – heal lower that foot anterior posterior of foot flexed towards anterior leg c) Varus- foot turns in d) Valgus- foot turns out Equino varus- most common Assessment: 1. Straighten legs & flexing them at midline pos Mgt: 1. Corrective shoe- Dennis brown shoe, spica cast Fx: of cast –

- to immobilize – bone alignment – prevent muscle spasm lead pencil – mark area to be amputated cold H20 – hasten setting process hot H20- slow setting process After cast application – how to move pt: - use open palm not fingers- fingers will cause indention – dry cast – natural air not blower – priority check : neurovascular check C- circulation M- motion S- sensation Cast – with bleeding - mask with ball pen edge of blood to know if bleeding is on going sign cast is dry = resonant sound, cast cold to touch do petaline – making rough surface of cast smooth CRUTCHES Fx: To maintain balance – To support weakened leg Principles in crutches – wt of body on palm! – Brachial pulsing – if wt of body in axila – Do palm exercise- squeeze ball Different crutch Gaits: 1. Swing Through 2. Swing to – no weight bearing are allowed into lower ext 3. Three point Gait - wt bearing is allowed in 1 ext 4. Four point gait 5. Two point Gait - wt bearing allowed in 2 lower ext