Diarrhea / ORT DEFINITION | SIGNS/SYMPTOMS | Most commonly due to acute infectious diarrhea (gastroenteritis) from a variety of causative organismsAntibiotics are a common cause of acute diarrhea! | Acute inflammation of the lining of the stomach and intestines caused by viruses, bacteria or their toxins or parasitesPresents commonly with diarrhea, abdominal cramps, and vomiting.CommunicabilityOften fecal-oral route (especially viruses)Food poisoning (especially bacteria)Day care centers, crowded living conditions, poor sanitation and cleanliness | DIAGNOSTIC TESTS | MAIN TREATMENT | Stool gram stain and culture (if bloody stools)Stool for Ova & Parasites (if hx suggestive)CBC – assess for anemia/infectionUrinalysis and urine culture (r/o UTI)Electrolytes | Oral rehydration therapy (ORT) is one of the major worldwide health advances of the last decade:Safer, less painful, and less costly than IV rehydrationOral rehydration solution enhances and promotes reabsorption of H2O and NaReduces vomiting, diarrhea, and duration of illnessORT GuidelinesDiarrhea w/o dehydrationMild dehydrationMod dehydrationSevere dehydrationReplacing ongoing losses | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | Teach parents at well childcare visits in first yearKeep 24 hour supply of ORT in homeBegin with first sign of diarrheaReplace with ½ cup ORT for each diarrheal stoolSeek medical attention prn signs or dehydrationAfter re-hydration resume breast/formula feeding or normal diet in the older childAdvise them never to try to formulate an OR solution themselves.Teach personal hygiene/ hand washingProper preparation and storage of breast milk or formulaCareful food preparationImmunization for RotavirusAvoid unnecessary antibiotic usage | Acute diarrhea is leading cause of illness in children <5 years (10’s of millions cases in US)65% of hospitalizations and 85% of deaths are in infants < 12 months |

GERD DEFINITION | SIGNS/SYMPTOMS | GER:Reflux of gastric contents into esophagus.GERD:Becomes a disease when complications such as growth failure, bleeding, dysphagia, or esophagitis (“heartburn”) develop.GERD is associated with respiratory symptoms, including apnea, bronchospasm, laryngospasm, and pneumonia. | Infants:Frequent regurgitation and/or vomitingWeight loss, FTTCoughing, gagging, choking, wheezing with feedingsExcessive irritabilityHemataemesisApnea or “ALTE”Children:HeartburnAbdominal painNoncardiac chest painChronic coughDysphagiaNocturnal asthmaRecurrent pneumonia | DIAGNOSTIC TESTS | MAIN TREATMENT | Upper GI series24 hour esophageal pH monitoring studyScintigraphy (detects radioactive substances in the esophagus after a feeding of the compound and assesses gastric emptying)Endoscopy with biopsy | Infants with mild disease:Avoid large feedingsThicken formula with rice cerealHold infant upright for 20-30min after mealsDecrease stimulation after mealsChildren / Teens:Weight loss if indicatedSmaller more frequent mealsAvoid exacerbating foods: (caffeine, citrus, tomatoes, alcohol, peppermint, spicy or fried foods)Pharmacologic: (H2-receptor antagonists: cimetidine [Tagamet], ranitidine [Zantac], famotidine [Pepcid]; PPI’s: esomeprazole [Nexium], lansoprazole [Prevacid], omeprazole [Prilosec], pantoprazole [Protonix], rabeprazole [Acephex])Surgical | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | Educating patents regarding home care, including feeding, positioning, and medications | Identifying children with symptomsProviding care for the child undergoing surgical repair |

INTUSSUSCEPTION DEFINITION | SIGNS/SYMPTOMS | Invagination of a portion of small or large bowelLeads to acute obstructionMost common site is at the ileocecal valveMost common cause of acute intestinal obstruction in children younger than 5 years, Most common in males 3-9 mosUsually idiopathic | Intermittent Severe abdominal painSudden onset, screaming/legs drawn upAppears normal in between pain episodesSausage shaped mass in RUQCurrant jelly bloody stoolsTender distended abdomenVomitingEventual fever, prostration and other signs or peritonitis | DIAGNOSTIC TESTS | MAIN TREATMENT | Diagnosis based on symptoms w/ US to confirm10% spontaneously reduceReturn of normal BM indicates resolutionNotify MD as treatment regimen will changeNon-surgical treatmentStabilize with IV, NG, start antibioticsReduce the defect with air or saline administered rectally with Ultrasound guidanceSurgical treatmentLaparotomyStandard pre/post op care | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | Prepare parents for the immediate need for hospitalization, the nonsurgical technique of hydrostatic reduction, and the possibility of surgery. | Passage of a normal brown stool usually indicates that the intussusception has reduced itself. This is immediately reported to the practitioner, who may choose to alter the diagnostic and therapeutic care plan. |

AOM DEFINITION | SIGNS/SYMPTOMS | An inflammation of the middle ear space with a rapid onset of the signs and symptoms of acute infection (i.e., fever and otalgia [ear pain]) | Follows an upper respiratory infectionOtalgia (earache)FeverPurulent discharge (otorrhea) may or may not be presentInfant or Very Young Child:CryingFussy, restless, irrtibleTendency to rub, hold, or pull affected earRolls head from side to sideDifficulty comforting childLoss of appetiteOlder Child:Crying or verbalizing feeling of discomfortIrritabilityLethargyLoss of appetite | DIAGNOSTIC TESTS | MAIN TREATMENT | Assessment of tympanic membrane mobilityDiagnosis is made if purulent discolored effusion and a bulging or full, opacified, or reddened immobile membrane | Antiobiotics: oral amoxicillin for initial episode, second-line antiobiotics include amoxicillin-clavulante, azithromycin, and cephalosporins such as defdinir, defuroxime, and cefpodoxime | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | Review preventive measures with parentsBreastfeeding is protective;Feed in upright position,ImmunizationsAvoidance of 2nd hand smokeNo pacifierAdvise parents to seek treatment at first signsComplete entire course of antibioticsNotify MD if symptoms increase or do not resolve with treatment | Administer analgesics and antipyreticsTylenol, Motrin, CodeineHeat or ice application if helpfulSoft diet, plenty of fluidsLie on effected side for comfort and if drainageRegular hearing and speech screening (q 6mos) if recurrent AOM or OME |

MYRINGOTOMY DEFINITION | NURSING CONSIDERATIONS | A surgical incision of the eardrum, may be necessary to alleviate the severe pain of AOM. A myringotomy is also performed to provide drainage of infected middle ear fluid in the presence of complications (mastoiditis, labrinthitis, or facial paralysis) or to allow purulent middle ear fluid to drain into the ear canal for culture. A minimally invasive laser-assisted myringotomy procedure may be performed in outpatient settings. | Outpatient procedurePost-operative pain uncommonAntibiotic eardrops usually prescribedLimit activities for few daysNo blowing of nose for 7-10 daysAvoid getting water in ears;Tubes typically fall out in 6-18 mosNotify provider, but usually not replaced |

VESICO-URETER REFLUX (VUR) DEFINITION | SIGNS/SYMPTOMS | Vesicoureteral reflux (VUR) is the backward flow of urine from the bladder into the kidneys. | Causes frequent UTIsUsually diagnosed in infancy/early childhoodSevere cases can cause kidney damage | DIAGNOSTIC TESTS | MAIN TREATMENT | A urine culture, to check for a UTIUltrasound of the kidneys. This test uses sound waves to find out the size and shape of the kidneys. It can't detect reflux.Cystourethrogram (cystogram) after the UTI has been treated. This test can detect VUR and help find out if it's mild or severe. The voiding cystourethrogram, for example, uses an X-ray to take pictures of the urinary tract. The bladder is filled with dye, and pictures are taken of the bladder as it fills and empties. | Mild to moderate cases are treated with low dose continuous antibioticsMinor to major surgical procedures for more severe cases |

STRABISMUS DEFINITION | SIGNS/SYMPTOMS | “Squint” or cross-eye – Malalignment of eyesEstropia – Inward deviation of eyeExotropia – Outward deviation of eye | Infant: Lack of tracking; lack of eye contactToddler/Preschooler: squinting, tilting head, closing one eye, falling, difficulty with self feeding, inaccurate judgement in picking up objects, unable to see print or moving objects clearly | DIAGNOSTIC TESTS | MAIN TREATMENT | Physical Exam TechniquesRed light reflexCorneal reflexCover testVision ScreeningVisual trackingLea symbolsTumbling E | Depends on cause of strabismusMay involve occlusion therapy (patching stronger eye) or surgery to increase visual stimulation to weaker eyeEarly diagnosis essential to prevent vision loss |

PRECOCIOUS PUBERTY DEFINITION | SIGNS/SYMPTOMS | Manifestations of sexual development before age 9 years in boys or age 8 years in girls. | Early maturation and development of the gonads with secretion of sex hormones, development of secondary sex characteristics, and sometime production of mature sperm and ova.Premature thelarche, premature pubarche, and premature menarche. | DIAGNOSTIC TESTS | MAIN TREATMENT | | Is directed toward the specific cause when known.Precocious puberty of central origin is managed with monthly injections of a synthetic analog of luteinizing hormone-releasing hormone, which regulates pituitary secretions.Treatment is discontinued at a chronologically appropriate time. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | Both parents and the affected child should be taught the injection procedure. | Psychologic management of the patient and family is an important aspect of care. |

SCOLLOSIS DEFINITION | SIGNS/SYMPTOMS | Scoliosis is a complex spinal deformity in three planes, usually involving lateral curvature, spinal rotation causing rib asymmetry, and thoracic hypokyphosis. | It is most noticeable during the preadolescent growth spurts. Parents frequently bring a child for foll up on an abnormal school scoliosis screening or because of ill-filling clothes, such as poorly fitting jeans. | DIAGNOSTIC TESTS | MAIN TREATMENT | ObservationAdams test (bending forward with arms hanging down)ScoliometerRadiographs, Cobb technique (standard measurement of angle curvature), Risser scale, Tanner maturity rating | ObservationBracing and ExerciseSurgical Management |

BACTERIAL MENIGITIS DEFINITION | SIGNS/SYMPTOMS | Infectious, high morbidity/mortalityLarge decline in incidence since vaccines begunRisk FactorsFollowing bacterial URICollege dorm outbreaksImmunosuppressionPenetrating head wound | 2 mos-2yearsFever – cold hands & feetRefusing food or vomitingFretful, dislike of being handledPale blotchy skinBlank, staringDrowsyStiff neckHigh pitched2yrs-adolescenseFever/chillsExcruciating headache; photophobiaIrritability and restlessness, eventually change in LOCNausea/vomitingNuchal Rigidity/opisthotonosSeizure may be first signPositive Kernig’s and Brudzinski’s signs | DIAGNOSTIC TESTS | MAIN TREATMENT | Lumbar PunctureBlood CulturesTo identify appropriate antibioticsCT/MRIr/o abscess, identify increased ICP | Initiate isolation in ICUMaintain NPOInitiate/Monitor IV therapyAdminister Medications as prescribedIV antibioticsDecadronAnti-convulsants such as DilantinKeep family informed | NURSING DIAGNOSES | NURSING CONSIDERATIONS | | Frequent MonitoringV.S., Pain, Neuro status/ICP, fluid status, Urine outputSeizure precautionsDarkened, quiet environmentPadded side rails upProvide for comfortAdminister analgesicsKeep room temp cool,position on side or on back with no pillow and HOB up slightly |

CONSTIPATION DEFINITION | SIGNS/SYMPTOMS | An alteration in the frequency, consistency, or ease of passing stool. | Affected children are prone to chronic fecal retention and overflow incontinence. | DIAGNOSTIC TESTS | MAIN TREATMENT | History of bowel habits, diet, drugs.Digital examination. | NewbornDigital evacuation but may require irrigation with a hypertonic solution or contrast medium.InfancyIncrease the amount of cereal, vegetables and fruit in the infant’s diet.ChildhoodChange diet to provide more fiber and fluids. Establish a bowel routine. Stool softners. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | Parents benefit from guidance in selecting foods that facilitate bowel movements. | |

APPENDICITIS DEFINITION | SIGNS/SYMPTOMS | Most common pediatric surgical emergency70,000 cases per yearAge Differential1-2/ 10,000 Birth to age 425/10,000 10-18yrsVery rare in neonateMortalityVaries from .1 to 1%Usually related to peritonitis>>Sepsis>>Shock | Fever- usually low gradeAbdominal pain with guardingGradual onset of pain followed by vomitingStarts periumbilical then localizes to RLQEspecially tender at Mc Burney’s PointRebound tenderness not necessary to elicitVomiting, decreased bowel soundsDifficulty walking, R hip pain, stoopingAnorexia, irritabilitySigns of ruptureSudden relief of painFollowed by increase of diffuse pain with rigid guarding of abdomenDiffusely Tender abdomenHigher feverMore toxic appearance with tachycardia and tachypnea | DIAGNOSTIC TESTS | MAIN TREATMENT | Diagnostic conundrum for pediatriciansDiagnoses based on clinical symptoms supported by imagingMany differentials to include; especially in the adolescent femaleAt least 15 differentials must be considered including:Diverticulitis, inflammatory bowel disease, IntussusceptionMesenteric lymphadenopathy,Ectopic pregnancy, ruptured ovarian cystLaboratoryCBC, urinalysis and culture, bhcg in adolescent femalesIncreased WBC with L shiftCRP takes 12 hours to riseImagingAbdominal U/S is preferred imaging in childrenCT however is considered more definitive | |

ADHD DEFINITION | SIGNS/SYMPTOMS | Characterized by:inattention, impulsiveness and hyperactivitySymptoms must be present in 2 settings andTo have begun by age 7Other causes have been ruled outLasts longer than 6 monthsRegularly disrupts school or other activitiesInterferes with relationships both peer and adult | Symptoms of inattention include:Fails to pay attention to details, makes careless mistakesHas trouble sustaining attention during tasks or playSeems not to listen even when spoken to directlyOften fails to finish schoolwork, chores or other tasksAvoids or dislikes tasks that require sustained mental effort, such as schoolwork or homeworkFrequently loses needed items, such as books, pencilsSigns and symptoms of hyperactive and impulsive behavior may include:Fidgets or squirms frequentlyOften leaves his or her seat in the classroom ( anywhere!)Often runs or climbs excessively when it's not appropriateFrequently has difficulty playing quietlyTalks excessivelyFrequently has difficulty waiting for his or her turn | DIAGNOSTIC TESTS | MAIN TREATMENT | | MedicationsStimulants (Ritalin, Intuive, Concentra)Non-stimulates (Strattara)Antidepressants (tricyclic or Wellbutrin)Advise parents to give medication in AM, and to report insomnia, worsening hyperactivity, anorexia with weight loss.Q 6mos visits for growth and development assessmentsBehavior management and/or behavioral therapyDecrease external stimuli (e.g. turn TV off)Consistent routines; reduce alternativesConsistent and effective disciplineIncrease physical activityDiet: does food additives cause ADHD? | NURSING DIAGNOSES | NURSING CONSIDERATIONS | | Altered brain function and anatomyHeredityExposure to maternal drugs, toxinsPrematurityExposure to environmental toxins (lead)ADHD frequently occurs with other conditionshyperthyroidism, anxiety disorder, learning disability or gifted, Tourette syndrome |

SPEECH/SENSORY IMPAIRMENT DEFINITION | SIGNS/SYMPTOMS | The most formidable task in the education of a child who is profoundly hearing impaired is learning to speak. Speech is learned through a multisensory approach, using visual, tactile, kinesthetic, and auditory stimulation. Parents are encouraged to participate fully in the learning process | |

IRON DEFICIENCY ANEMIA DEFINITION | SIGNS/SYMPTOMS | Anemia caused by an inadequate supply of dietary iron is the most prevalent nutritional disorder in the United States and the most common mineral disturbance. | May be underweight or overweight (milk babies) | DIAGNOSTIC TESTS | MAIN TREATMENT | Blood test. | Iron-fortified formulaIron rich foodsOral iron supplementsIV or IM iron administration | MAJOR TEACHING POINTS | | Instruction parents in the administration of iron.Family education about diet. | |

HYPERCHOLESTEROLEMIA DEFINITION | SIGNS/SYMPTOMS | Refers to excessive cholesterol in the blood. | | DIAGNOSTIC TESTS | MAIN TREATMENT | Blood draw, full lipid profile, 12-hour fast. | Lifestyle modification.Heart-healthy diet.Drug therapy (statins). | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | Dietary changes.Medication education. | Keep child fasting for at least 12-hours before screening.Take family history. |

STREP THROAT DEFINITION | SIGNS/SYMPTOMS | Generally a relatively brief illness that varies in severity from subclinical (no symptoms) to severe toxicity. | Pharyngitis, headache, fever, and abdominal pain.Tonsils and pharynx may be inflamed and covered with exudates. | DIAGNOSTIC TESTS | MAIN TREATMENT | Throat culture. | Oral penicillin or oral erythromycin if allergic to penicillin. | MAJOR TEACHING POINTS | | Replace toothbrush after on antibiotics for 24 hrs.Should not return to school or daycare until after on antibiotics for 24 hrs.Avoid close contact with sick child and avoid sharing drinks or food. | |

RETINOBLASTOMA DEFINITION | SIGNS/SYMPTOMS | Arises from the retina, is the most common congenital malignant intraocular tumor of childhood. | White eye reflex (most common sign)Strabismus (second most common sign)Red, painful eye, often with glaucomaBlindness (late sign) | DIAGNOSTIC TESTS | MAIN TREATMENT | Ophthalmoscopic examinationUltrasonography & CT of the orbit | Depends on stage of tumorPlaque brachytherapy (surgical radioactive implant on the sclera until maximum dose has been delivered to the tumor)Laser photocoagulation (laser beam to coagulate blood supply to the tumor)Cryotherapy (freezing the tumor by destroying the microcirculation to the tumor thorough microcrystal formation)Thermotherapy (using microwaves or infrared radiation to deliver heat to the tumor) | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | Care of socketCleaning and inserting prosthesisUse of antibiotic ointment | Prepare parents for the child’s facial appearance after surgery (eye removed) |

DUCHENE MUSCULAR DYSTROPHY DEFINITION | SIGNS/SYMPTOMS | Most severe and the most common MD of childhood.It is inherited as an X-linked recessive trait. | Mild subtle delays. Muscle weakness appears during 3rd to 7th year. Difficulties in running, riding a bike, climbing stairs. Abnormal gait. Lordosis, frequent falls, Gower sign (child turning onto side or abdomen, flexing knees to assume a kneeling position, then with knees extended gradually pushing torso to an upright position by “walking” the hands up the legs), enlarged muscles (especially thighs and upper arms), Later profound muscular atrophy, cognitive impairment, contracture deformities of hips, knees and ankles, and obesity. | DIAGNOSTIC TESTS | MAIN TREATMENT | Blood polymerase chain reaction (PCR) for the dystrophin gene mutation.Prenatal diagnosis possible at 12 weeks.Serum enzyme measurement, muscle biopsy, and EMG. Serum creatine kinase levels are extremely high in the first 2 years of life. | No effective treatment exists.Corticosteriods prednisone and deflazacort possible treatment.Maintaining optimum function in all muscles for as long as possible. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | Inform parents about respite care and make referrals to to other services needed. | Help the child and family cope with the chronic, progressive, incapaciting disease.Design a program that will afford maximal independence. |

TYPE 1 SPINAL MUSCULAR ATROPHY DEFINITION | SIGNS/SYMPTOMS | AKA Werdnig-Hoffmann Disease.Progressive weakness and wasting of skeletal muscles caused by degeneration of anterior horn cells. It is inherited as an autosomal recessive trait and is the most common and most severe paralytic form of the floppy infant syndrome (congenital hypotonia). | Scoliosis. Hip subluxation and dislocation may also occur. Muscle and joint contractures. | DIAGNOSTIC TESTS | MAIN TREATMENT | Diagnosis is based on the molecular genetic marker for the SMN (survival motor neuron) gene, which is located on chromosome 5q13. Prenatal diagnosis may be made by genetic analysis. Diagnostic studies include muscle electromyography (EMG), however genetic analysis is the gold standard. | There is no cure, treatment is symptomatic and preventive, primarily preventing joint contractures and treating orthopedic problems (scoliosis). | NURSING DIAGNOSES | NURSING CONSIDERATIONS | | Main goal is to assist the child and family in dealing with the illness while progressing toward a life of normalization within the child’s capabilities.Prevention of muscle and joint contractures.Promoting independence. |

IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP) DEFINITION | SIGNS/SYMPTOMS | An acquired hemorrhagic disorder characterized by (1) thrombocytopenia, excessive destriction of platelets; (2) purpura, a discoloration caused by petechiae beneath the skin; and (3) normal bone marrow eith normal or increased number of immature platelets (megakaryocytes) and eosinophils. Cause is unknown, but believed to be an autoimmune response to disease-related antigens. | Acute form: seen after upper respiratory tract infections, after measles, rubella, and chickenpox, or after infection with parvovirus B19.Easy bruising (petechiae, ecchymoses, most often over bony prominences), Bleeding from mucous membranes (epistaxis, bleeding gums, internal hemorrhage evidenced by hematuria, hematemesis, melena, hemarthrosis, or menorrhagia), hematomas over lower extremities. | DIAGNOSTIC TESTS | MAIN TREATMENT | Suspected on the basis of clinical manifestations.Reduced ITP platelet count. | Is primarily supportive.Acute: prednisone, IV immune globulin (IVIG), and anti-D antibody.Splenectomy if have ITP for 1 year or longer. | MAJOR TEACHING POINTS | | Possible side effects of therapy and limitation in activities.No contact sports, bike riding, skateboarding, in-line skating, gymnastics, climbing, or running.No aspirin or NSAIDs. | |

APLASTIC ANEMIA DEFINITION | SIGNS/SYMPTOMS | Refers to a bone marrow failure condition in which the formed elements of the blood are simultaneously depressed. The peripheral blood smear demonstrates pancytopenia or the triad of profound anemia, leucopenia, and thrombocytopenia (Fanconi syndrome).It can be primary (congenital) or secondary (acquired). | Anemia, leucopenia, and decreased platelet count. | DIAGNOSTIC TESTS | MAIN TREATMENT | Bone marrow aspiration. | Immunosuppressive therapy to remove the presumed immunologic functions that prolong aplasia.Replacement of the bone marrow through transplantation.Antilymphocyte globulin (ALG) or antihymocyte globulin (ATG) is the principal drug treatment. | MAJOR TEACHING POINTS S | NURSING CONSIDERATIONS | Prepare child and patients for all procedures. | Prevent complications from severe pancytopenia.Emotionally support child and family of a potentially fatal outcome. |

DYSRHYTHMIAS DEFINITION | SIGNS/SYMPTOMS | Abnormal heart rhythms. | Bradydysrhythmias – abnormally slow rate.Tachydysrhythmias – abnormally rapid rate.Conduction disturbances – irregular heart rate. | DIAGNOSTIC TESTS | MAIN TREATMENT | Cardiac monitor (12 lead ECG).Compare apical and radial pulses. | Pacemaker for bradydysrhythmias.Vagal maneuvers and radiofrequency ablation for Tachydysrhythmias. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | Education of the family regarding symptoms and treatment. Show how to take radial pulse for a full minute. Instructions for medication. | |

HEMOPHILIA DEFINITION | SIGNS/SYMPTOMS | A group of bleeding disorders in which there is a deficiency of one of the factors necessary for the coagulation of blood. | Prolonged bleeding, hemorrhage from any trauma, excessive bruising, hematomas, spontaneous hematuria | DIAGNOSTIC TESTS | MAIN TREATMENT | Hx of bleeding episodes, partial thromboplastin time, DNA (carrier testing) | Factor VIII, DDVAP (synthetic vasopressin), NSAIDS, corticosteroids. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | Refer to parent-group discussions with childs comprehensive team.Discuss mothers feelings of responsibility. | Avoid IM injections. Apply firm direct bleeding for 5 minutes when bleeding occurs. Suction gently, teach the patient and family the early and late clinical manifestations of bleeding. Use a soft bristled toothbrush. |

SICKLE CELL DEFINITION | SIGNS/SYMPTOMS | Sickle cell anemia is one of a group of diseases collectively termed hemoglobinopathies, in which normal adult Hgb is partly or completely replaced by abnormal sickle Hgb.Sickle cell disease includes all the hereditary disorders with clinical, hematologic, and pathologic features that are related to the presence of HbS. | Episodic crisis: vasoocclusive (distal ischemia & pain), acute splenic sequestration (pooling of blood in the liver & spleen w/decreased blood volume & shock), aplastic (diminished RBC production), hyperhemolytic (Accelerated rate of RBC destruction), cerebrovascular accident, chest syndrome (similar to pneumonia), and infection. | DIAGNOSTIC TESTS | MAIN TREATMENT | Newborn screening.Blood test. | Prevent the sickling phenomena, to treat the medical emergencies of sickle cell crisis.Rest to minimize energy expenditure & oxygen use, hydration through oral and IV therapy, electrolyte replacement because hypoxia results in metabolic acidosis, which alo promotes sickling, analgesics for severe pain from vasoocclusion, blood replacement to treat anemia and reduce the viscosity of the sickled blood, antibiotics to treat any existing infection. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | Education of disease and its consequences.Importance of adequate hydration to prevent sickling. | |

ULCERATIVE COLITIS DEFINITION | SIGNS/SYMPTOMS | Inflammation of colon characterized by eroded areas of mucus membranes and tissue beneath it. | Diarrhea, blood, pus mucus in stool, fecal incontinence, dehydration, weakness, and metabolic acidosis. | DIAGNOSTIC TESTS | MAIN TREATMENT | Barium enema, colonoscopy, and stool analysis. | Corticosteroids (to reduce inflammation), analgesics, antichloinergics, Gantrisin, anti- diarrheals, and antipersitlatics. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Patient should increase protein/calorie intake and decrease fat/fiber. They may need TPN. It’s also important to maintain fluid and electrolyte balance and or monitor electrolytes. |

CROHN’S DISEASE DEFINITION | SIGNS/SYMPTOMS | Inflammatory condition of any area of the small or large intestines. | Severe pain in LRQ, chronic diarrhea, mucus/pus/fatty stools, increase temp. and a decrease in weight. | DIAGNOSTIC TESTS | MAIN TREATMENT | Upper GI series, barium enema, and colonoscopy/ endoscopy. | Sulfasazine, Olsalazine, balslazide, and Mesalamine medications like Asacol, Lialda, and Pentasa. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Regular exercise is important, as well as increasing dietary protein and calorie intake and decreasing fiber and fat. Patients may need TPN. Maintain fluid and electrolyte balance and monitor electrolytes closely. |

GLOMERULONEPHRITIS DEFINITION | SIGNS/SYMPTOMS | Most cases are post-infections and have being associated with pneumococcal, streptococcal and viral infections. | Oliguria, edema, HTN, circulatory congestion, hematuria and proteinuria. | DIAGNOSTIC TESTS | MAIN TREATMENT | Onset of nephritis appears after an average latency period of about 10 days post streptococcal infection. Urinalysis: hematuria and proteinuria, azotemia reflected in increased BUN and creatinine. ASO titer. | Supportive measures and early recognition and treatment of complications. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Daily weights, the child with edema, htn and gross hematuria may be subject to complications and anticipatory preparations such as sz precautions and IV equipment are included, promote rest. |

NEPHROTIC SYNDROME DEFINITION | SIGNS/SYMPTOMS | Disorder characterized by increased glomerular permeability to plasma protein, which results in massive urinary protein loss. | Massive proteinuria, hypoalbuminemia, hyperlipidemia and edema. | DIAGNOSTIC TESTS | MAIN TREATMENT | Suspected based on clinical manifestations and weight gain in a previously well child that increases slowly over days or weeks, proteinuria, hypoalbuminemia, hyperlipidemia. | Diuretic therapy to provide temporary relief from edema, acute infections are treated with antibiotics, corticosteroids are the first line therapy, Prednisone 2mg/kg/day in one or more divided doses. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Reduce excretion of urinary protein, reduce fluid retention in tissues, prevent infection, minimize complications related to therapies, dietary restrictions low salt. |

DIABETES DEFINITION | SIGNS/SYMPTOMS | A chronic disorder of metabolism characterized by a partial or complete deficiency of the hormone insulin. | Polyphagia, Polyuria, Polydipsia, irritability, blurred vision, poor wound healing, headache, hyperglycemia, DKA. | DIAGNOSTIC TESTS | MAIN TREATMENT | 8hr fasting 126 or more, random blood glucose 200 or more with classic signs of diabetes or an oral glucose tolerance test 200 or more in 2 hour sample test. | Insulin therapy goal to maintain near-normal glucose levels of less than 126 and HA1c of 7% or less, use of oral hypoglycemic such as metformin, Glucophage, etc. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | A multi-disciplinary approach. Educate child and family about the sings and symptoms of hyper/hypoglycemia. Teach about importance to adherence to regular blood glucose testing and medication regimen. Teach about the long-term complications related to diabetes such as neuropathy, CV disease, kidney disease, retinopathy, etc. |

CF DEFINITION | SIGNS/SYMPTOMS | A genetic dysfunction of exocrine glands causing production of abnormally thick mucous secretions. | Steatorrhea (fatty, foul smelling stools), failure to grow, thin extremities, sallow skin, wheezing respirations, dry nonproductive cough, and the skin tastes salty. | DIAGNOSTIC TESTS | MAIN TREATMENT | A sweat chloride test, pancreatic enzymes, and chest radiography. | Albuterol, pancreas enzyme (pancrelipase), vitamins, mucolytic agent, and antibiotics. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Goals are to prevent or minimize pulmonary complications, ensure adequate nutrition for growth, encourage appropriate physical activity, promote reasonable quality of life for child/family, and increase protein/calories in the diet. |

CEREBRAL PALSY DEFINITION | SIGNS/SYMPTOMS | A neuromuscular disability in which voluntary muscles are poorly controlled because of brain damage. | Hypotonia, asymmetric posture/strength, abnormal coordination, increase reflexes, and abnormal gait, plasticity of muscles is the dominant characteristic. | DIAGNOSTIC TESTS | MAIN TREATMENT | There is no test to rule out CP. Performing a cranial ultrasound, computed tomography scan (CT Scan), and magnetic resonance imaging scans (MRIs) are all done to rule out other conditions. | Botox, baclofen, dantrolene (muscle relaxers), diazepam, anti cholinergic, elodea (dopamine), keppra, depakote (anticonvulsants), and tizanidine. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | The goal of treatment is to preserve the physical/mental capacity of child. Assist with early dx. Assist with physical/occupational therapy. Provide emotional support. Encourage measure to allow normal growth and Provide a high calorie diet. |

CLASSIFICATION OF CONGENITAL HEART DEFECTS Congestive Heart Failure | Defects with Increased Pulmonary Blood | the inability of the heart to pump a sufficient amount of oxygen to meet the metabolic needs of the body - In infants and children, inadequate cardiac output most commonly caused by congenital heart defect that produce an excessive volume or pressure load on the myocardium. - In infants and children, a combination of left-sided and right-sided heart failure is usually present. -The goal of treatment is to improve cardiac function, remove accumulated fluid and sodium, decrease cardiac demand, and decrease oxygen consumption. | Intracardiac communication along the septum or an abnormal connection between the great arteries allows blood to flow from the high-pressure left side of the heart to the low-pressure right side of the heart. The infant usually shows signs and symptoms of CHF - Atrial Septal Defect (ASD) - Atrioventricular canal defect - Patent dctus arteriosus - Ventricular septal defect (VSD) | Obstructive Defects | Defects with decreased pulmonary blood flow | Blood exiting a portion of the heart meets an area of anatomical narrowing (stenosis), causing obstruction of blood flow. The location of the narrowing is usually near the valve of the obstructive defect. Infant and children exhibits signs of CHF. Children with mild obstruction may be asymptomatic. - Aortic stenosis - Coarctation of the aorta - Pulmonary stenosis | Obstructed pulmonary blood flow and an anatomical defect (ASD or VSD) between the right and left side of the heart are present. Pressure on the right side of the heart increases, exceeding pressure of the left side of the heart, which allows desaturated blood to shunt right to left, causing desaturation in the left side of the heart and in the systemic circulation. Typically hypoxemia and cyanosis appear - Tetralogy of Fallot - Tricuspid artresia | Mixed Defects | | Fully saturated blood flow mixes with the desaturated blood flow, causing a desaturation of the systemic blood flow. Pulmonary congestion occurs and cardiac output decreases. Signs of CHF are present; symptoms vary with the degree of desaturation - Hypoplastic left heart syndrome - Transposition of the great arteries or transposition of the great vessels - Total anomalous pulmonary venous connection - Truncus arteriosus | |

COARCTATION OF THE AORTA DEFINITION | SIGNS/SYMPTOMS | Localized narrowing near the insertion of the ductus arteriosus. | Blood pressure is higher in the upper extremities than the lower extremities; bounding pulses in the arms, weak of absent femoral pulses, and cool lower extremities may be present. Signs of CHF may occur in infants. Signs and symptoms of decreased cardiac output may be present. Children may experience headaches, dizziness, fainting, and epistaxis resulting from hypertension. | DIAGNOSTIC TESTS | MAIN TREATMENT | Echocardiogram, CT, MRI | Mechanical ventilation and medications to improve cardiac output are often necessary before surgery. Resection of the coarcted portion with end-to-end anastomosis of the aorta or enlargement of the constricted section using a graft may be required. Because the defect is outside the heart, cardiopulmonary bypass is not required, and a thoracotomy incision is used. Management of the defect may be done via balloon angioplasty in children; restenosis can occur. A continuous intravenous medication, prostaglandin (PGE-1), is used to open the ductus arteriosus (and maintain it in an open state) allowing blood flow to areas beyond the coarctation. Antihypertensives may also be prescribed to control high blood pressure. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Monitor vital signs closely. Monitor respiratory status for the presence of nasal flaring, use of accessory muscles, and other signs of impending respiratory distress, and notify physician if any changes occur. Auscultate breath sounds for crackles, rhonchi, or wheezes. If respiratory effort is increased, place child in Trendelenberg position, elevating the head and upper body, to decrease the work of breathing. Administer humidified oxygen as prescribed. Provide endotracheal tube and ventilator care as prescribed. Monitor for hypercyanotic spells and intervene immediately of they occur. Assess for signs of CHF such as periorbital edema or dependent edema in the hands and feet. Assess peripheral pulses. Maintain fluid restriction as prescribed. Monitor intake and output, and notify physician if a decrease in urine output occurs. Obtain daily weight. Provide adequate nutrition (high calorie requirements) as prescribed. Administer medications as prescribed. Plan intervention to allow maximal rest for the child; keep the child as stress-free as possible. Prepare the child for cardiac catheterization, if appropriate. |

PATENT DUCTUS ARTERIOSUS DEFINITION | SIGNS/SYMPTOMS | Failure of ductus arteriosus (artery connecting the aorta and the pulmonary artery) to close within the first week of life. | Machinery-like murmur is present. An infant may be asymptomatic or may show signs of CHF. A widened pulse pressure and bounding pulses are present. Signs and symptoms of decreased cardiac output may be present. | DIAGNOSTIC TESTS | MAIN TREATMENT | X-ray and Echocardiogram | Indomethacin (Indocin), a prostaglandin inhibitor, may be administered to close a patent ductus arteriosus in premature infants and some newborns. Ibuprofen may also be given. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | |

WILM’S TUMOR DEFINITION | SIGNS/SYMPTOMS | Wilm’s Tumor is also known as nephroblastoma, the most common malignant renal and intra-abdominal tumor of childhood. It is a childhood form of kidney cancer with a peak age of occurrence at 3 years of age. It is sometimes associated with abnormalities of the urinary tracts or other birth defects. | Abdominal swelling or mass that is firm, non-tender, confined to one side. Hematuria, fatigue, malaise, hypertension, weight loss, fever. If metastasis to the lungs occurs - dyspnea, cough, SOB, chest pain. | DIAGNOSTIC TESTS | MAIN TREATMENT | History and physical exam. Radiographic studies such as abdominal ultrasound and chest-computed tomography scan, hematologic studies, biochemical studies and urinalysis. If large tumor is present, venacavagram is used to demonstrate possible tumor involvement adjacent to the vena cava. Bone marrow aspiration may be performed to rule out metastasis. | Chemotherapy with or without radiation. Surgery is required with confirmation of a renal mass, usually within 24-48 hours of admission. Never palpate unless needed to avoid rupture of the mass. If both kidneys are affected, child may be treated with radiotherapy or chemotherapy before surgery to decrease size of tumor. The most effective agents for treating Wilm’s Tumor are Actinomycin D, vincristine, and adriamycin, with addition of cyclophosphamide duration is 6-15 months. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Never palpate unless needed. Avoid contact sports. |

TURNER SYNDROME DEFINITION | SIGNS/SYMPTOMS | Is caused by absence of one of the X chromosomes. Most girls who have this disorder have one X chromosome missing from all cells. Often recognized at birth if the newborn has a webbed neck, low posterior hairline, widely spaced nipples, and edema of the hands and feet. It can also be diagnosed at puberty because of 3 features: short stature, sexual infantilism, and amenorrhea. Girls with Turner Syndrome are usually infertile. | Short stature, webbed neck, low posterior hairline, shield-shaped chest with widely spaced nipples, sterile, no development of secondary sex characteristics. | DIAGNOSTIC TESTS | MAIN TREATMENT | Diagnosis is confirmed on the basis of a negative sex chromatin test. | Hormone treatment and psychological counseling for both child and parents. Growth hormones, such as somatropin, (Humatrope, Genotropin, Saizen) given in weekly treatments. They may also be placed on estrogen and related hormone therapy in order to begin puberty and achieve adult sexual development. Estrogen therapy continues until menopause. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Linear growth can be increased by the administration of growth hormone if therapy has begun early. Estrogen therapy is initiated during the usual time for puberty to promote development of secondary sex characteristics |

HIRSCHPRUNG’S DISEASE DEFINITION | SIGNS/SYMPTOMS | (congenital aganglionic megacolon) is a mechanical obstruction caused by inadequate motility of part of the intestines. Occurs more in males than females. Characterized by the absence of ganglion cells. | Constipation, ribbon like foul smelling stools, abdominal distention, visible peristalsis, easily palpable fecal mass, and undernourished, anemic appearance. | DIAGNOSTIC TESTS | MAIN TREATMENT | Radiographs, an un-prepped barium enema, and anorectal manometric examinations assist in the differential diagnosis, which is confirmed by full thickness rectal biopsy, demonstrating the absence of ganglion cells in the myentetic and submucosal plexuses. | Surgery to remove the aganglionic portion of the bowel to relieve obstruction and restore normal bowel motility and function of the internal anal sphincter. First a temporary ostomy is created proximal to the aganglionic segment to relieve obstruction and allow the normally enervated and dilated bowel to return to its normal size. Drug treatment is not a component of the standard of care for this disease itself; however, some medications may be used to treat complications of Hirschsprung disease. Medications may include antibiotics for the treatment of enterocolitis or the use of botulinum toxin injection at the anal sphincter for the treatment of recurrent enterocolitis due to anal hypertonicity. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Enema or colonic irrigation with physiologic saline solution. Older child whose symptoms are chronic but not severe may be treated with isotonic enemas, stool softeners, and a low residue diet. |

DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) DEFINITION | SIGNS/SYMPTOMS | Abnormal formation of the hip joint that allows the femoral head slip in and out of the socket. | Shortened appearance of the affected leg, external rotation of the affected leg, uneven gluteal folds, restricted abduction of the affected hip, widened space between thighs. | DIAGNOSTIC TESTS | MAIN TREATMENT | Positive Ortolani test, positive Barlow test, radiographic tests such as x-ray, ultrasound, CT scan and MRI after 4 months of age. | Positioning device such as a Pavlik harness, hip Spica cast, closed/open reduction. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Educate parents to apply and maintain reduction device, assess device for need of adjustment, skin care (place clothing and diaper between the skin and the devise, check for redness and gently massage healthy skin). |

ASTHMA DEFINITION | SIGNS/SYMPTOMS | Narrowing of the airway caused by inflammation. | Cough, wheezing, shortness of breath, chest tightness, retraction of the ribs, increased pulse, increased blood pressure, barrel chest, tripod position. | DIAGNOSTIC TESTS | MAIN TREATMENT | Clinical manifestations, history, physical examination, arterial blood gas, chest x-ray, peak expiratory flow test, pulmonary function test. | Avoid triggers, quick-relief medications, long-term control medications and quick-relief medications: short-acting beta II agonists, anticholinergics and systemic corticosteroids; long-term control medications: inhaled corticosteroids, cromolyn sodium, nedocromil, long-acting beta II agonists, methylxanthines and leukotriene modifiers (see pages 1337-1340 of Maternal Child Nursing Care for complete explanation of meds). | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Physical and historical assessment, promote normal activity, education patient and parents how to: avoid allergens, recognize signs and symptoms, when to seek emergency services, properly use medications and delivery devices. |

PYLORIC STENOSIS DEFINITION | SIGNS/SYMPTOMS | Narrowing of the pylorus that prevents stomach contents from emptying into the duodenum between 1 week and 5 months of age. | Nonbilious projectile vomiting, olive shaped mass palpable in the upper middle abdomen or right upper quadrant of the abdomen, abdominal pain, constant hunger, dehydration, failure to gain weight or weight loss, wake like motion from right to left in the upper abdomen, shortly after feeding. | DIAGNOSTIC TESTS | MAIN TREATMENT | Physical examination, abdominal ultrasound, barium x-ray, chemistry panel. | Surgical treatment such as: pyloromyotomy or pyloroplasty. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Physical assessment focused on hydration status, IV fluid and electrolyte therapy, intake and output, observe character of emesis and stools, monitor vital signs and lab values, maintain and use of NG tube, hygienic care of the mouth, mucus membranes and skin. |

OSTEOGENISIS IMPERFECTA DEFINITION | SIGNS/SYMPTOMS | Osteoporosis syndrome characterized by excessive fractures and bone deformity. | Bone fragility, bone deformity, fracture, blue sclera, hearing loss, discolored teeth. There are 6 classifications of OI. | DIAGNOSTIC TESTS | MAIN TREATMENT | Differential diagnosis. | Treatment is supportive. Preventing contractures and deformities, muscle weakness and osteoporosis, and mal-alignment of lower extremity joints. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Careful handling, must be supported even when changing. |

DOWN’S DEFINITION | SIGNS/SYMPTOMS | A chromosomal abnormality that occurs when a child is born with an extra chromosome 21. | Separated sagittal suture, oblique palpebral fissures, small nose, depressed nasal bridge, high arched narrow palate, protruding tongue, hyper-flexibility, and muscle weakness. | DIAGNOSTIC TESTS | MAIN TREATMENT | Usually diagnosed by clinical manifestations or a chromosome analysis to confirm genetic abnormality. | No cure exists. Surgery to correct serious congenital anomalies (heart defects), treatment is focused on preventing complications related to the diagnosis, such as PT/OT/ST therapy, infection prevention, and assisting child to reach highest developmental level of independent functioning. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Support family, assist in preventing physical problems. Swaddle or wrap infant tightly. Clear nose often, and provide adequate fluid intake. |

BRONCHIOLITIS DEFINITION | SIGNS/SYMPTOMS | Acute viral infection of lower airways with maximum effect at the bronchiolar level. | Labored respirations, poor feedings, cough, tachypnea, retractions and flaring nares, emphysema, increased nasal mucus, wheezing. | DIAGNOSTIC TESTS | MAIN TREATMENT | Nasal secretions for RSV antigen detection using ELISA and DFA techniques. | Cool humidified oxygen, adequate fluid intake, palivizumab to high-risk infants. | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Airway maintenance, contact and standard precautions, monitor O2 and pulse-oximetry. |

PHYSICAL ASSESSMENT DEFINITION | SIGNS/SYMPTOMS | The detailed examination of the body from head to toe using the techniques of observation/inspection, palpation, percussion, and auscultation. Use paper doll technique for teaching about body part being examined. | | DIAGNOSTIC TESTS | MAIN TREATMENT | Growth charts, scales, thermometer | | MAJOR TEACHING POINTS | NURSING CONSIDERATIONS | | Use a head to toe fashion; examination should be as pleasant as possible, use different approaches for different developmental groups. |