Unit 5
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| cleft pallete | in plate & lip both; comes from failure of maxillary & medial nasal processes to fuse; when they dont close together and lip close; can be bilateral or unilateral; kids tend to have dental anomalies - missing/deformed teeth on side of cleft
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| complete cleft | involves bone and soft tissue
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| partial cleft | bones arent fused but tissue is ok
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| reasons for cleft pallete | closure appears by wk 10 of gestation; genetic hx; linked with folic acid, environmental, maternal alcohol and smoking
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| cleft pallete s/sx | not be able to make seal around nipple or suction of milk - leaks - complete cleft risk for choking
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| cleft pallete tx | surgery - sometimes before leaving hospital - stages of surgery depending on degree - post op - goal is to prevent trauma, infection of suture line - no spoon or straws
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| esophageal atresia | absense of normal body opening or abnormal closure of a body passage - esophagus ends in blind pouch; maybe fistula connecting trachea and esophagus
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| esophageal atresia s/sx | copious amts of thin mucous right after mouth, cyanotic, respir distress, choking/gasping, if fistula - air goes into belly - distended, risk for aspiration/pneumonia, put ng tube and wont pass - NPO
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| esophageal atresia tx/ni | preventing respiratory complications, IV fluids, suctioning and NPO, surgery with in 1st few days, if pt not able to have surgery - gastrostomy, surgery done in stages - fistula as getting older esophageal dilation done, put in ICU
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| pytoric stenosis (narrowing) | obstruction of pyloric canal d/t thickening of pyloric & narrowing of passage between stomach and duodenum, pylorus also elongates, can cause total obstruction and dilation of stomach, wont present initially - over time develop sx
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| pytoric stenosis s/sx | seen between wk 2-4, start to vomit after every feed and progress to projectile vomiting, irritable, miserable, dehydration, palpate round mass RUQ, parastalic waves in belly
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| pytoric stenosis tx | surgery, pyloromyotomy - aka FREDET - RAMSTEDT procedure, circular muscle fibers of pylorous cut to widen canal, 4-6hrs of clear liquids,
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| Gastroesophageal reflux | relaxation of cardiac sphincter allows gastric contents into esophagus, more common in premies or child with neurological impairment but can affect full term as well
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| GERD s/sx | spitting up lg amts, NOT projectile - but pneumonia and aspiration possible, irritable, getting older - chest hurts, abdominal pain, if severe enough - FTT - not gaining wt/growing, most of the time resolves by 18months
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| GERD tx | change formulas (soy milks), thicken (add rice), positioning after feeding - keep upright, can put on belly, if sx severe enough - meds/surgery to create valve reflux, older - watch what you eat, no spicy foods and sit upright
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| hirschprung disease AKA? | also termed congenital aganglionic mega colon
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| hirschprung disease def | autonomic parasympathetic ganglia in muscular wall of intestine are absent; leads to persistent constipation/obstruction; usually shows early in life but may not appear until teens
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| hirschprung disease s/sx | newborn - failure to pass meconium, refusing to suck, abdominal distention, bile-stained of meconium emesis; older child - hx of abdominal distention , chronic constipation w/passage of ribbonlike, foul smelling stools
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| hirschprung disease dx/tx | barium studies, rectal biopsy which confirms absense of nerve cells; surgery to remove aganglionic portion of bowel to relieve obstruction maybe done in stages, TPN, enema
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| intussusception | proximal segment of bowel telescopes into more distal segment, most common site - ileocecal valve ileum slips into cecum pulling mesentary w/it; walls of intestine rub causing itis & obstruction leading to ischemia, necrosis, perforation & hemorrhage
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| intussusception s/sx/tx | sudden severe abdominal pain, vomiting, palpable mass in abdomen, currant jelly stools - blood and mucous - classic sx; can be life threatening; barium enema may reduce telescoping d/t pressure it puts on bowel; may require surgery to reduce telescoping
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| lead poisoning (plumbism) | toxic levels of lead in blood; lead-based primary source; airborne exposure problem; children absorb & retain more lead in proportion to body wt-
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| What does lead interfere with in the bodily system? | lead interferes w normal cell fx, particularly nervous system - impacts brain, affects blood cells, kidneys & vitamin D & calcium metabolism, once in body deposited in bones & teeth then slowly released by bones
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| lead poisoning s/sx | early - irritabily, hyperactivity, poor concentration, aggression, impulsiveness, may also develop learning disabilities, hearing impairment & growth delays; can lead to encephalopathy can occur or degenerative disease of brain
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| blood levels of lead | if >10mcg/dl - follow up; >44mcg/dl - trmt - chelation therapy and follow up
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| lead poisoning tx | chelation therapy - admin of chemical that binds to lead and increase rate of excretion , find source of lead, not instint fx, catch early as possible b/c brain damage
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| gastroenteritis def and tx | diarrhea and vomiting - viral or bacterial cause; can lead to dehydration; tx to stop diarrhea including antibiotics, if bacterial cause, if in health care facility - contact isolation
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| phenylketouria (PKU) | disorder affecting body's ability to use protein; child lacks live enzyme - phenylanine hydroxylase needed to convert phenylanine into tryosine; can lead to mental retardation; 48-72hrs of milkthen test b/c not present at birth
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| what diet does PKU require? | no meat, dairy, nuts, chicken, can have fruits and veggies, special formula
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| malabsorption syndrome | celiac disease; hild unable to digest gluten which is found in wheat, barley, rye and oats
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| celiac disease | 6-8m or 1yr +; doesnt appear until solid foods ingested; gluten free diet required; cant absorb nutrients - immune system attacks vili; abdominal distention, chronic diarrhea & FTT; life-long; puts at risk for growth retardation and GI cancers as adults
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| what are gluten restrictions? what can they have? | can have rice and corn; gluten included in mayo and canned soups
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| hypospadius | uretheral opening along ventral surface of penile shaft - commonly seen w/CHORDEE - ventral curature of penis - downward - thick bond that pulls downward - picked up at birth
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| hypospadius tx/ni | surgery; extend urethra to penis & make opening where it should be; done in stages; 16-18months; if parents wants circumcision, wait until surgery - place stint b/c inflammation
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| epispadias | urethra opens on dorsal or upper surface of penis - often seen w/exstrophy of bladder' tx - surgery
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| cryptorchiolism def and dx | undescended testicle(s), can result in sterility; present at birht - CT or US to confirm
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| cryptorchiolism tx/ni | hormone therapy - HCG may help descent - usually by age 1; if HCG doesnt work orchiopexy by 2 yrs of age - can increase risk of testicular cancer
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| Wilms tumor (nephroblastoma) | highly metastatic cancerous tumor in kidney tumor; dx - 2-5yrs; genetix link; tumor comes from embryonic tissue left from birth and begins to grow rapidly in area of kidney
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| Wilms tumor s/sx | externally none - go for checkup and find a mass on abdomine - may have hematuria, hypertension - MUST NOT BE PALPATED once mass discovered; u/s, ct, MRI, will eval for metastasis to liver, lungs, bones
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| Wilms tumor tx/ni | surgery followed by chem, removal of kidney & tissue, radiation if lg tumor or extensive metastasis
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| nephrotic syndrome | characterized by edema, proteinuria, hypoalbuminemia, hyperlipidemia, altered immunity
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| primary nephrotic syndrome | MCNS or minimal change nephrotic syndrome - only affects kidneys
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| secondary nephrotic syndrome | systemic - such as diabetes, sickle cells, etc
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| nephrotic syndrome s/sx, dx | edema, hypertension, irritability, possible respiratory distress, anorexia, increased susceptibility to infection; dx - y/a, renal biopsy
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| nephrotic syndrome tx | chronic disorder - remission that lasts 12-18 months; corticosteroids to reduce edema, oychophosamide, diuretics, urine testing, diet changes, good skin care
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| acute glomerulonephritis | inflammation of glomeruli and nephrons of kidney, follow group A beta strep - blocks glomeruli which allows RBC & protein to escape into urine
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| acute glomerulonephritis s/sx | tea-colored urine, oliguria, proteinuria, comes on 1-3 weeks after strep infection, periorbital edema, anorexia & malaise, headache, fever, irritability
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| acute glomerulonephritis dx | u/a, blood & protein test, hx of strep
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| acute glomerulonephritis tx | bedrest, antibiotics, diuretics, I&O, wts, urine testing, diet changes - low salt diet - resolves completely usually but can go on for more than a year and turn into chronic glomerulonephritis - unusual
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