Peds Exam 2

Cystic Fibrosis

exocrine gland dysfunction that involves multiple systems; mucous produced by the exocrine glands that is abnormally thick; this causes obstructions of the small passageways

Clinical Manifestations of CF

Meconium Ileus; abd. distention with vomiting and inability to pass stool, growth failure and malabsorption; hyperglycemia and polyuria

Pulmonary Pseudomonas Colonization

caused by mucous plugging, decreases QOL and increases r/o hospitalization, characterized by resistance to antibiotics, prev

Gastrointestinal issues with CF

Meconium becomes thickened in the ileum of neonate, cannot pass and is earliest postnatal manifestation of CF; Distal Intestinal Obstruction Syndrome(DIOS), distal cecum is constipated and can lead to obstruction

Pancreatic Insufficiency of CF

pancreatic duct filled with mucous and malabsorption of fat-soluble vitamins which can lead to weight loss

CF related diabetes

insulin insufficiency and resistance; not the same a type 1 or 2 diabetes; factors can include increased energy expenditure, chronic infection and liver and glucagon deficiency

Reproductive factors of CF

puberty will be delayed, most men become sterile d/t a block of the vas deferens; women are fertile but can be inhibited by highly viscous cervical mucous

Diagnosis of CF

newborn screening with IRT analysis and DNA analysis of Delta F 508; Sweat test is gold standard*, checks the amount of sodium and chloride in sweat, levels greater than 60 in child older than 6 months = CF; can also use stool fat and pulm. function test

Pulm issues and management of CF

monitor for pseudomonas; encourage forced expiration and use flutter mucous clearance device

Medications for CF

Bronchodilators for acute and Mucolytics for mucous removal via nebulizer

Nutrition for CF

replace pancreatic enzymes through meals and snacks, high protein high calorie diet

Bilateral Lung transplant for CF

for pts. with end stage CF; Cadaveric is more successful

Iron Deficiency Anemia

inadequate supply of loss of iron; caused by gi issues which lead to malabsorption, decreased supply, increased needs or cows milk ingestion

Nursing Implications for Iron deficiency anemia

enforce iron supplements for breast fed baby, vitamin C increases absorption

Care management for Iron Deficiency

taken supplement with straw or dropper to avoid tooth discoloration and brush teeth; do not give with milk products

Sickle Cell Anemia

abnormal sickle shaped cells accompanied by inflammation causing vasooclusion; can cause local hypoxia and cellular death

Diagnosis of Sickle Cell

newborn screening with Sickle Dex and Hgb electrophoresis

Clinical Manifestations

vitreous hemorrhage, retinal detachment, *Vaso-occlusive crisis: chest pain, fever, cough, CVA, cerebral infarction, limb pain; cardiomegaly and murmurs, and dilute urine

Sickle Cell Crisis

avoid anything that increases need for oxygen including trauma, fever, infection, stress and dehydration

Management of Sickle Cell

pain management is key, keep hydrated, blood transfusion, and replacing electrolytes; HYDRATION IS IMPORTANT*

Sickle Cell Meds

folic acid, prophylactic penicillin starting a 2 months- 5 y/o, Hydroxyurea to increase HbF

Hemophilia

A group of hereditary bleeding disorders that are X-linked (more in males)

Types of Hemophilia

A- Factor VIII deficiency accounts for 80% of cases

B- Christmas disease deficiency of IX factor

Von Willebrand- deficiency or absence of vWF and factor VIII

Diagnosis of Hemophilia

lab will show low levels of factor VIII or IX and prolonged PTT, other labs are normal (platelets, PT and fibrinogen)

Clinical Manifestations of Hemophilia

excessive bleeding and bruising, hemarrosis, neck swelling and airway obstruction, pain

Bleeding Prophylaxis for Hemophilia

factor replacement therapy, joint bleed will get replacement 3x/week after bleed stops

Nursing Management of Hemophilia

prevent bleeding episodes, *RICE=rest ice compress and elevate

Dehydration

can develop quicckly in children and infants; vomiting or diarrhea main cause

Signs and Symptoms of Dehydration

Tachycardia*, no tears, weight loss, cyanosis, decreased voiding, dry mucous membranes, pale, high BUN

Management of Dehydration

oral rehydration: Mild- 50ml/kg; Moderate- 100ml/kg; Diarrhea- 10ml/kg/stool; Vomiting- 2-5 ml syringe every 2-3 min

Acute Diarrhea (Gastroenteritis)

rota virus major cause; c/o lack of clean water, crowding, poor hygiene, poor sanitation, nutritional deficiency

Management of Gastroenteritis

rehydration therapy, NO BRAT DIET, no soda or juice and mx fluid and electrolytes

Hirschsprung Disease

mechanical obstruction of intestine d/t absence of ganglion cells in colon; inability to relax internal sphincter and decreased peristalsis

Clinical Signs of Hirschsprung Disease

no stool in 24-48 hours, growth failure, refusal to eat, ribbon like stools or diarrhea d/t encopresis

Diagnosis of Hirschsprung Disease

Rectal biopsy to confirm; missing recto sphincter nerve, monitor K+ and replace as needed, surgery to remove a ganglionic portion of bowel with temp ostomy

Nursing Management of Hirschsprung Disease

preop- bowel evacuation, abd. measurements

postop- NPO, NGT, enterostomal therapy

Appendicitis

inflammation of vermiform appendix, obstruction of lumen of appendix, swollen lymphoid tissues

Diagnosis of Appendicitis

McBurney point pain, RLQ pain, vomiting, fever, pain, elevated WBC, CT scan

Management of Appendicitis

watch for fever, sudden pain relief, abd. distention, tachy, pallor chills or irritability; pre and post op give IVF and NG to suction

Hypertrophic Pyloric Stenosis

constriction of the pyloric sphincter with obstruction of gastric outlet; vomiting 30-60 minutes after eating, develops in first 2-5 weeks of life

Hypert. Pyloric Stenosis Signs and Symptoms

Projectile, non bilious vomiting, olive-like mass with vomiting, dehydration, metabolic alkalosis and growth failure

Management of Hypertrophic pyloric stenosis

pyloromyotomy; make NPO and correct met. alkalosis, NGT, post op- mx I/O, vitals, and infusions, some vomiting is common

Cleft Lip surgery

repaired between 2-3 months; rule of 10’s- 10 weeks old and 10-12 lbs

Cleft palate surgery

repaired between 6-12 months, take advantage of palatal changes with growth

Nursing management of cleft lip and palate

soft nipple for feedings, burp, special bottles, cheek support

post op- sit pt. uprights with elbow restraints, soft foods only; no brushing for 1-2 weeks, palate- no food in mouth for 7-10 days

Esophageal Atresia and Tracheoesophageal fistula

congenital issue, esophagus ends before stomach or a fistula forms in trachea, blind pouch is formed (atresia)

Diagnosis of EA and TEF

maternal history of polyhydramnios*(high amniotic fluid), abd distention

The 3 C’s in EA and TEF

coughing, choking, and cyanosis; failure to pass NGT and suction, apnea, abd distention and airless scaphoid abdomen

Management of EA and TEF

prevent aspiration, NPO, IVF, 02 if needed, no pacifier

Leukemia

broad group of malignant diseases of the bone marrow and lymphatic system; no tumor but there is an abnormal white count, liver and spleen are mostly affected

Patho of Leukemia

leukocyte count is low, unrestricted proliferation of immature WBC’s in the blood forming tissues of the body

Signs and Symptoms of Leukemia

pale skin, irritability, fever, fatigue, bleeding and bruising, leg pain and weight loss

Diagnosis of Leukemia

Bone marrow aspiration or biopsy shows infiltration of blast cells; peripheral blood smear, and LP to evaluate CNS involvement

Therapeutic Management of Leukemia

induction therapy for 4-6 weeks; intrathecal chemo for CNS prophylaxis; consolidation to prevent resistant leukemia clones; maintenance therapy

Neuroblastoma

develop in the adrenal gland or retroperitoneal sympathetic chain in abdomen, metastasis may have already occurred before diagnosis

Diagnosis of Neuroblastoma

locate primary site, CT scan, MIBG scan and bone marrow evaluation, urinary catecholamines

Clinical Manifestations of Neuroblastoma

most common s/s is firm, non tender irregular mass in the abdomen that crosses the midline

Prognosis of Neuroblastoma

silent tumor, diagnosis made after metastasis, the younger the age the better prognosis, may have spontaneous regression

Wilms Tumor/Nephroblastoma

painless swelling or mass in the abdomen, does not cross the midline, causes fatigue, hematuria, high bp and weight loss

Nephrotic Syndrome

glomerular membrane becomes permeable to proteins and albumin; results in hyper albuminuria and hypo albunemia

Clinical Manifestations of nephrotic syndrome

massive protein uria >2+ on test, weight gain, edema, ascites

Management of Nephrotic Syndrome

reduction of excretion of protein, reduce fluid retention, prevent infection; limiting sodium, prednisone, immunosuppressants and diuretics

Acute Glomerulonephritis

caused after strep throat, decrease in plasma filtration allowing for excessive accumulation of water and salt

Diagnosis for AGN

previous strep infection; not positive for strep but Titer positive

Clinical Manifestations of AGN

generalized edema, anorexia, pallor, irritability, lethargy, oliguria, hematuria, proteinuria

Management of AGN

normal bp and urine output, daily weights, dietary restrictions

Type 1 diabetes

autoimmune disease, destruction of beta cells leading to insulin deficiency

Hyper/Hypoglycemia manifestations

increased thirst, frequent urination, fatigue, blurred vision, increased hunger

shaking, sweating, palpitations, extreme hunger

Diabetic Ketoacidosis

metabolic consequence of insulin defi. ketones become released; s/s- hyperglycemia, abd pain, chest pain, kussmaul resp. n/v, dehydration, LOC and Coma

DKA and K+

must have K+ return to cells, body is dangerously low in K+

Treatment of DKA

adequate insulin to reduce the elevated blood glucose level, fluids→ NS at first, electrolyte replacement

Type 2 Diabetes

usually arises due to insulin resistance in the body

Type 2 Diabtese Managment

BGM monitoring, nutrition, exercise, test urine for ketones, mx for DKA

Precocious Puberty

sexual development before 9 in boys and 7 in girls, caused by congenital abnormalities, meningitis, trauma, neoplasms, radiotherapy

Management of Precocious Puberty

normally resolves on own, provide psychological care for child and family, anticipatory guidance, dress age appropriate

Congenital Hypothyroidism

early detection and prompt treatment essential, neonatal screening is mandatory

Clinical s/s of hypothyroidism

poor feeding, lethargy, prolonged jaundice, resp. difficulties, short forehead. puffy eyelids and thick mottled skin

Older kid hypothyroidism s/s

short stature, obesity, intellectual difficulties, abnormal tendons, slow movements

Management of Hypothyroidism

Synthroid, hormone replacement