Final Exam!!! 210 PDF

HIRSCHSPRUNG DISEASE - Also called congenital aganglionic megacolon - Mechanical obstruction from inadequate motility of intestine - Incidence: 1 in 5,000 live births; more common in males and in down syndrome - Birth defect; nerve cells missing in intestines, lacking gangl...

HIRSCHSPRUNG DISEASE - Also called congenital aganglionic megacolon - Mechanical obstruction from inadequate motility of intestine - Incidence: 1 in 5,000 live births; more common in males and in down syndrome - Birth defect; nerve cells missing in intestines, lacking ganglion cells and ganglion nerve tissue - Typically in rectum, not allowing rectum to relax and not allowing child to defecate - GOLD STANDARD: Rectal biopsy shows absence of ganglion cells in colon - Because of the inability to pass the stool, they don't have that nerve tissue, so the sphincter does not relax which means that stool is backing up. This is when you will result in a Megacolon. Backs up and you end up with distended sigmoid colon PATHOPHYSIOLOGY - Aganglionic segment usually includes rectum and proximal colon - Accumulation of stool with distention - Failure of internal anal sphincter to relax - Enterocolitis may occur - Ribbon-like stools common A CHILD WITH A COLOSTOMY - When lacking ganglion cells there is nothing we can do but remove these portions - Could be temporary or permanent - If temporary we left bowel rest for 6 months - Usually if they have to remove more than 1 foot it becomes a permanent colostomy - Megacolon is treated with a resection/colostomy - Again, can be temporary or permanent depending on how much was removed INTUSSUSCEPTION - Telescoping or invagination of one portion of intestine into another - Usually happens with ileum - Cutting off blood flow at the bottom is a big issue - Occasionally due to intestinal lesions - Often cause is unknown - Abdominal pain, abdominal mass and blood stools are classic triad - Ileum comes up and telescopes in itself MANIFESTATIONS - Acute abdominal pain alternating with intervals of no pain - Goes back in and out causing the intervals - Empty RLQ (Dance sign) - b/c it is all in the RUQ - Passage of red currant jelly stools - Sausage shaped mass in RUQ  - b/c intestine of lower quadrant have been pushed up TREATMENT - Therapeutic management: - Radiologist guided pneumo or air enema (80% successful) - Infant placed in prone position so radiologist can squeeze the buttocks closed, and rapidly insert air into the colon - Inserting the air causing the intestine to move out and correct itself - Ultrasound guided saline enema - How we know if treatment is successful: - Passage of normal brown stool means that it has reduced itself – notify physician immediately!! INCIDENCE OF SYMPTOMS - Colic like pain 86% - Vomiting 64% - Lethargy 19% CYSTIC FIBROSIS (CF) - Exocrine gland dysfunction that produces multisystem involvement - One dysfunction which causes total mayhem in the body - Most common lethal genetic illness among white children - Approximately 1,000 new diagnoses per year in the US PATHOPHYSIOLOGY: - In normal exocrine cells, a functional chloride channel (Cystic Fibrosis Transmembrane Conductance Regulator or CFTR) actively transports chloride from inside the cell to the cell surface. Water follows passively. This permits hydration of the substance that the cell secretes (such as mucus in the lungs). - In CF, however, the chloride channel is abnormal in ALL exocrine glands. The water does not follow so the exocrine secretion is thick & viscous. Increased Viscosity of Mucous Gland Secretion: - Result in mechanical obstructions - THICK inspissated mucoprotein accumulates, dilates, precipitates, coagulates to form concretions in glands and ducts - Respiratory tract and pancreas are predominantly affected RESPIRATORY MANIFESTATIONS: - LUNGS: - Mucous does not carry inhaled materials out of the lungs  - Cilia cannot beat - The mucus is stuck blocking the cilia from protecting and capturing all that bacteria - mucociliary clearance does not occur due to inflammation - Mucous still captures bacteria but it is kept in the lungs & leads to pulmonary infections which result in destruction of airway cartilage and progressive lung damage - Respiratory Progression: - Gradual progression follows chronic infection - Bronchial epithelium is destroyed - Infection spreads to peribronchial tissues, weakening bronchial walls - Peribronchial fibrosis - Decreased O2/CO2 exchange - Presentation: - Wheezing respirations, dry nonproductive cough - Generalized obstructive emphysema - Patchy atelectasis - Cyanosis - Clubbing of fingers and toes - Repeated bronchitis and pneumonia - GI Tract: - THICK secretions block ducts – cystic dilation – degeneration – diffuse fibrosis - Prevents pancreatic enzymes from reaching duodenum - Impaired digestion/absorption of fat - Steatorrhea: fatty stools, oily stools - Impaired digestion/absorption of protein - Azotorrhea: excessive discharge of greasy oily look - Clinical manifestations of GI Tract: - Pancreatic enzyme deficiency - Sweat gland dysfunction - Failure to thrive - Increase weight loss despite increased appetite - Gradual gastrointestinal deterioration GI TRACT: - Same mechanism in liver, pancreas & intestines causes thick secretions which alter ability of exocrine secretions to be delivered, so food is not digested or absorbed - Affects 85% of those with CF - Fats are the most poorly digested which leads to bloating, abdominal pain & gas - Supplementation at the beginning of meals - Like pancreatic enzymes: with the first bite of food!! PRESENTATION: - Meconium ileus - Distal intestinal obstruction syndrome - Excretion of undigested food in stool – increased bulk, frothy, and foul - Wasting of tissues - Prolapse of the rectum - Lacking hydration- constipation - could result in this PANCREATIC and BILE DUCT presentation: - Type 1 diabetes mellitus develops because retained digestive enzymes actually “digest”/destroy pancreas over time - Liver cirrhosis develops in about 87% of people with CF - CF patients lose salt through their sweat glands and are at risk for dehydration during hot weather & exercise; they often prefer salty foods ADDITIONAL PRESENTATIONS: - Delayed puberty in females - Sterility in males - hyponatremic/hypochloremic alkalosis - Metabolic disorder when body loses too much water and fluoride and bicarb levels increase causing alkalosis and compensatory mechanisms - Hypoalbuminemia DIAGNOSTIC EVALUATION: - Sweat Chloride Test: (most helpful diagnostic test) - Accurate results dependant on stimulation a maximal sweat rate - Must be 1 month old for this test - Pilocarpine is driven into sweat glands by low voltage electrical current to stimulate sweat production - Normal value 60mM/L - Other Testing: - Chest X Ray - Pulmonary Function Test (PFT) - Stool fat and/or enzyme analysis - Barium enema GOALS: - Prevent/minimize pulmonary complications - Adequate nutrition for growth - Assist in adapting to chronic illness ASSOCIATED TREATMENTS: - RESPIRATORY: - Respiratory Hygiene: - Average patient spends approx. 2 ½ hours per day on respiratory treatments - Antibiotics - Often in higher doses - Aerosol mucolytics - Bronchodilators - Percussion & postural drainage (CPT) - Exercise & physical fitness - Anti-inflammatory agents - Lung Transplant: - survival is now 50-60% @ 5 years; due to more aggressive treatment - transplants currently less common in pediatrics - GASTROINTESTINAL: - Vitamins (A, D, E & K in water soluble form) - Salt supplementation - Pancreatic enzymes whenever food is ingested - This should be sprinkled on food with first bite! - High calorie & protein diet with moderate fat as much as 150% RDA - Management of GERD - Enemas or isotonic fluid lavage of intestines - Iron, if deficient - Other medications to decrease or slow hepatic lesions - PANCREAS: - Insulin - VAS DEFERENS (absent): - Adoption - Sperm Aspiration - Artificial insemination with donor sperm - FEMALE INFERTILITY: - Affects 20-35% - NASAL & SINUS CAVITIES: - Symptomatic - Anti-inflammatory, mucolytic, etc. agents INNOVATIVE PHARMACOTHERAPY:  - The usage of CFTR modulators (first approved in 2012), which are medications that boost function of the CFTR protein that is altered in CF - Examples include: - Kalydeco (ivacaftor) - Orkambi (lumacaftor/ivacaftor) - Symdeko (tezacaftor/ivacaftor) - Trikafta (elexacaftor/tezacaftor/ivacaftor) GENETICS: - Current consensus recommendations for CF carrier testing (first in 1997): - Couples seeking prenatal care or planning a pregnancy, especially if one partner caucasian - Adults with family history of CF - Partners of persons with CF - Newborn screening for CF is now mandated in all 50 states & Washington, D.C. - Positive screens referred to CF Center to do sweat chloride determination after 1 month of age PROGNOSIS OF CF: - Estimated life expectancy for child born with CF in 2003 is 40 - 50 years - Death in CF is usually due to progressive lung damaged to the point that respiratory failure stresses the heart, causing right heart failure & death - Maximum health potential - Nutrition - Prevention/early aggressive treatment of infection - Pulmonary hygiene - New research – hope for the future - Gene therapy - Bilateral lung transplants - Improved pharmacologic agents FAMILY SUPPORT: - Coping with emotional needs of child and family - Child required treatments multiple times a day - Frequent hospitalization - Implications of genetic transmission of disease KAWASAKI DISEASE: - Acute systemic vasculitis of unknown cause - Inflammation in the walls of the medium to small size blood vessels - More common in boys - 80% of cases in kids < 5 yrs old - Incidence peaks during toddlerhood - Seen most frequently late winter, early spring - 5 of the 6 following must be present for positive diagnosis:  - Fever > 5 days - Bilateral conjunctival infection without exudate (not gunky but inflammation b/c of inflammation of those vessels) - Changes in oral mucous membranes: Strawberry tongue is classic - Tongue is very vascular causing strawberry tongue - Rash - Cervical lymphadenopathy - Lymph in neck, everything inflamed blocking of your lymphatic system - Changes in the extremities: during acute phase edema and erythema, then peeling - Changes in extremities like edema, then peeling of hands and feet - Other S&S include: - Between 10-14 days, danger of aneurysm forming - b/c inflammation in vasculature has gone down = vessels are now weakened - Child is typically very irritable ! - Vessels in gut are upset because of vasculature inflammation - Increased WBC with left side shift, increased C reactive protein (inflammation), increased platelets and decreased Hgb - Without treatment 20% of these kids develop sequelae - Death, although rare, can result from coronary thrombosis/aneurysm rupture - (thrombosis b/c increased platelets) - TREATMENT includes: - Antiplatelet: - ASA 80-100 mg/kg/day for fever, then 3-5 mg/kg/day for antiplatelet - Anti-inflammatory: - IVIG for anti-inflammatory purpose EPIGLOTTITIS - Swelling of tissues above the vocal cords - Rapid and progressive - LIFE THREATENING emergency - Usual cause: Haemophilus Influenza Type B (HIB) - HIB is a bacteria not a virus  - b/c of HIB vaccine this has decreased epiglottitis incidences - Given at 2 months and 12-15 months SYMPTOMS: - Sudden sore throat - Tripod position: due to respiratory distress - Drooling: b/c they cant swallow due to inflammation of upper airway and narrowing - Fever - Muffled voice - Dyspnea - Stridor - Absence of spontaneous crying TREATMENT: - Artificial airway - Generally placed on a vent - If suspecting this, it is important NOT to examine the throat. Do not open and look into the airway unless prepared to intubate the child. Any tissue trauma can cause more swelling so less air getting in - Try to provide support and education to parents: this is scary and stressful - Oxygen - Corticosteroids: decrease swelling - Fluids/IV - Antibiotics - Prevention: H. Influenzae B vaccine ANTIDOTE - POISONING!!! Substance Antidote Acetaminophen N-Acetylcysteine Mucomyst Opioid Naloxone Narcan Benzodiazepine Flumazenil Romazicon Digoxin Digoxin Immune Fab Digibind Carbon monoxide Oxygen CONJUNCTIVITIS - Inflammation of the conjunctiva - Pink eye is a bacterial infection - This can be viral as well  - Risks: - Under 2 weeks of age - Recent upper respiratory infection - Recent ear infection SYMPTOMS - Tearing - Pain - Redness - Crusting TREATMENT - Antibiotics - Surgery Sickle Cell Anemia - A group of genetic disorders which have an overabundance of abnormal hemoglobin - Includes sickle cell anemia, sick beta thalassemia syndrome, and other hemoglobinopathies such as Hgb SS, SC, SD, and SO Hemoglobin Electrophoresis: - Blood test to determine the different types of hemoglobin that an individual has - Sickle turbidity quick screen test - Done on newborn screening - Abnormal hemoglobin includes: C, S, D, H, A2, E, Barts, O-Arab, etc. - Person with most common sickle cell anemia has SS hemoglobin with >40% Hbg S (usually > 90%) Sickle Cell Trait: - Carries one gene for Hgb S but rarely display any symptoms of the disease - Hgb S per hemoglobin electrophoresis is 12 years – 70-150 mg/dL (at bedtime 90-150) Remember that blood glucose levels are a snapshot of current glucose & does not tell you if patient is trending up or down. A1C looks at glucose level over time. The American Diabetes Association recommends that all adolescents use the intensive therapy regimen (tight control) and encourages the use of an insulin pump (continuous subcutaneous infusion of insulin) Insulin pumps are being used now on some babies Pumps take motivation & person who is good problem solver Can eat anything as long as they can count carbohydrates, but there are differences between high & low glycemic foods Intensive therapy involves: ○ Monitoring blood glucose four times a day & once a week at 3 AM ○ Monitoring dietary intake ○ Varying the insulin dose to fit carbohydrates eaten at each meal or snack ○ Anticipating exercise in the routine The biggest drawback of intensive therapy is hypoglycemia INSULIN PUMP: Delivers basal rate continuously that may vary throughout day; for example: ○ 7-11 AM @ 0.25 units per kg ○ 11 AM-6 PM @ 0.20 units per kg ○ 6-8 PM @ 0.25 units per kg ○ 8 PM-7AM @ 0.20 units per kg Individual delivers bolus based on CHO intake; for example: ○ Infant may need 1 unit for every 45 grams of CHO DIABETIC KETOACIDOSIS: Develops over a period of several hours or days Complications include: electrolyte imbalances, thrombosis, pancreatitis, arrhythmia, intestinal necrosis Cerebral edema is a life-threatening complication of DKA ○ 1 in 100 cases of DKA ○ Mortality as high as 90% ○ Early intervention important S&S OF CEREBRAL EDEMA: Headache Uncharacteristic behaviors Decline in LOC Agitation Change in pupil size Seizure Recurrence of vomiting Bradycardia (drop in heart rate > 20) Hypertension (diastolic > 90) TREATMENT OF CEREBRAL EDEMA: Hyperventilation ○ -Blow off extra CO2, by intubating and ventilating to control RR so hyperventilation Intubation & ventilation support Mannitol (cerebral diuretic) ○ concerned about rebound, slow and steady wins the race Decrease fluid therapy Elevate head of bed COMPLICATIONS OF DIABETES: Every 24 hours in the U.S. the following occurs as a consequence of diabetes: Death = 810 Amputation = 230 Kidney failure = 120 Blindness = 55 TIDBITS: Physical activity increases insulin sensitivity; Regular exercise & fitness improve metabolic control with a lower insulin dose Absorption rate of insulin varies by site ○ Fastest absorption is from the abdomen  Check the blood glucose level of a toddler who is extremely sleepy or irritable, as these can be sign of either hypoglycemia or hyperglycemia Puberty may be delayed if diabetic control is inadequate Medical alert identification is needed for all diabetics The school health plan needs to be readily available with information & supplies to manage hypoglycemia & hyperglycemia EDUCATION IS ONGOING: Children are usually able to perform some of the tasks associated with insulin administration by 6-8 years of age, with supervision Adolescents are cognitively able to manage self-care ○ desire to be like peers often interferes with compliance Find teaching moments & teach in small increments The fewer changes the family/child has to make as a result of the diabetes, the greater the chance of compliance Child & family need to know how to manage an emergency & have a “sick day” plan PHYSICAL ASSESSMENT FINDINGS!!!!: Acanthosis nigricans: Thick, hyperpigmented areas of skin, usually in folds; a common sign of insulin resistance long before diabetes type 2 develops. Watch for this when doing a physical assessment on pediatric patients. Ramchandani, Neesha. Type 2 diabetes in children. AJN 2004;104 (3): 65-68. GROWTH HORMONE DEFICIENCY: Decreased levels of growth hormone r/t decreased activity of the pituitary gland Decreased stature Radiographic imaging of the wrist to evaluate ossification DX: Pituitary function testing confirms diagnosis Treatment: ○ biosynthetic growth hormone, subq daily injection, while they're growing (full height is achieved) PRECOCIOUS PUBERTY: Secondary Sex characters before 8 years in girls and 9 years in boys Why problematic? ○ Growth plates could close early, social implications, self esteem issues Treatment: Gonadotropin releasing hormone analog, discontinued at age of puberty Children at risk of? ○ Self esteem issues Medication is $$  Immunizations and Communicable Diseases Special Vulnerability of Infants and Children: Infants and young children are susceptible hosts because their immune system is not yet fully developed. Newborns are particularly susceptible because they are unvaccinated and have very immature immune systems Herd immunity: the resistance to the spread of a contagious disease within a population that results if a sufficiently high proportion of individuals are immune to the disease TYPES OF VACCINES:!! Inactivated (Killed): The microorganism is killed but will still produce an immune response ○ Example: poliovirus (IPV) Live (Attenuated): The microorganism is still alive but weakened ○ Example: Measles, influenza Toxoid: A toxin has been weakened but still has an effect antigen ○ Examples: Tetanus, Diphtheria Recombinant: Genetically altered organism that is used in a vaccine and will stimulate active immunity ○ Examples: Pertussis, HPV, Hepatitis B VACCINE REACTIONS: Common reactions: Local reactions: Erythema, pain, and induration at the injection site Systemic reactions: fever, fussiness, irritability, general malaise, anorexia Allergic reaction: ○ Mild Typically minutes to hours after injection Symptoms: Weals, rash, urticaria (rashed rash) Treatment: observation, Benadryl for comfort ○ Severe ○ Typically occurs 2-8 hours after vaccination ○ Symptoms: warmth, erythema, edema, especially near or at airway, petechiae, possible ulceration around the site of injection VACCINE CONTRAINDICATIONS AND BARRIERS: Contraindications to vaccine administration ○ History of anaphylactic reaction to the vaccine or one of its components ○ Moderate to severe acute illness ○ For specific vaccines, pregnancy or allergy to some components Barriers ○ Limited access to health care & Misconceptions are biggest 2 barriers in the US. ○ Lack of convenient primary care and hours possible for working parents  ○ Parental knowledge deficit ○ Religious/cultural prohibitions ○ Misconceptions NURSING CONSIDERATIONS: Document and educate!!! ○ Provide parents with information ○ Informed consent must be obtained before immunization administration ○ Required documentation: Date of administration, manufacturer, lot number, expiration date, site and route, name and title of who gave it Ensure proper age-appropriate administration techniques and holds Ensure proper storage of the vaccines ○ Check for damaged or expired vaccines ○ Store vaccines according to manufacturer guidelines ERYTHEMA INFECTIOSUM (FIFTHS DISEASE): - A common and highly contagious childhood ailment causing a distinctive face rash. - The rash appears after several days and may spread. - The facial rash looks as if the cheeks were slapped. -Sore throat, slight fever, upset stomach, headache, fatigue, and itching are among other symptoms. - The virus usually clears up on its own, but pain relievers may help with symptoms. MEASLES: The measles prodrome starts 3-4 days before the rash. The prodrome is characterized by a fever and by the “3 C’s”: cough, coryza, and conjunctivitis. Towards the end of the prodrome, tiny white (Koplik) spots may appear inside the cheeks. Photophobia is common. Following the prodrome, a maculopapular rash typically begins on the forehead or hairline and spreads downward to the neck, trunk, and upper and lower extremities (palms and soles rarely involved). The rash usually appears about 14 days after a person is exposed. Please note that immunocompromised patients may not develop a rash and those with a previous history of measles infection or vaccination may have a modified disease presentation characterized by milder symptoms MENINGOCOCCUS: - Meningococcal disease is a rare, serious illness caused by a bacteria (Neisseria meningitidis). It can cause meningitis, which is an infection of the brain and spinal cord, and it can also cause blood infections. Up to one out of seven (10-14 percent) of those who get the disease die. MONONUCLEOSIS: MOLLUSCUM CONTAGIOSUM: A viral skin infection that results in round, firm, painless bumps. The condition spreads through contact with an infected person or a contaminated object. Though painless, the small bumps might itch. Scratched bumps can spread infection to surrounding skin. The bumps usually disappear on their own. In rare cases, the bumps can be removed using medications or other procedures. PERTUSSIS/WHOOPING COUGH: Highly contagious respiratory infection caused by the bacterium Bordetella pertussis. - Main symptom: hacking cough, whooping - Tx: azithromycin, fluids(iv or oral) small frequent meals to prevent vomiting, apply O2 as needed ADD/ADHD: CNS processing disorder Some kids with ADD have a deficit in dopamine and norepinephrine (catecholamines), which lowers their ability to tolerate stimulation (they become easily distracted) Delay in brain maturation as it relates to self regulation Developmentally inappropriate behaviors involving attention Sleep disturbances are common May have difficulty forming social relationships Anxiety common  Hallmark symptoms: ○ Decreased attention span ○ Impulsiveness ○ Increased motor activity FAILURE TO THRIVE: Below 5th percentile in growth ○ Inadequate intake ○ Inadequate absorption ○ Increased metabolism ○ Defective utilization Diagnostics: ○ Ht/weight - diet intake - parent-child interaction ○ Organic problems – laboratory values Growth failure Developmental delay Undernutrition Apathy Withdrawn Feeding disorder No stranger fear Avoid eye contact Wide eye gaze Stiff Unresponsive Minimal smiling ANOREXIA NERVOSA: Eating disorder: refusal to maintain normal body weight Restrictive Eating and extreme exercise/laxatives/diuretics Primarily in adolescent and young adult females Average age of onset: 19 years Multifactorial cause Life threatening S&S: Severe weight loss Altered metabolic activity ○ Amenorrhea ○ Bradycardia, decreased blood pressure ○ Hypothermia, cold intolerance ○ Dry skin, brittle hair and nails ○ Appearance of lanugo  DEPRESSION: Likely that genetics, environment and culture interact to influence the differences in prevalence that we see among various cultures In children whose parents also suffer from depression/anxiety, researchers are unclear if the behavior in children is learned or biological. It’s likely a combination of both S&S: Poor school performance Not being with friends and not doing activities Sad and hopeless emotions and attitudes No energy or enthusiasm Anger towards others and people Taking criticism badly Thinking your unable to finish ideas to the best quality Guilt Forgetfulness Always being tired Changes in your eating and sleeping patterns Substance abuse Having problems with authority Suicidal thoughts or actions Treatment: Cognitive Behavior Therapy Medications Psychotherapy SUICIDE: 15% of youth consider suicide as an option each year 86% of suicide deaths are adolescent males Guns, suffocation are poisoning are most common means of suicide Under reported: labeled “accidents”