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Test 3: Chapters 21, 22, 27, 28, & 29

by: Trisha Riley

Test 3: Chapters 21, 22, 27, 28, & 29 NBSN3010C

Trisha Riley
GPA 3.6
Continuum of Care for Children and Families

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About this Document

Based off the chapters in Child Health Nursing textbook by Ball, Binder and Cowen, 3rd edition.
Continuum of Care for Children and Families
Study Guide
pediatrics, peds, pain, hematologic function, immune health, immune system, child with cancer, pediatric cancer, pediatric immunizations, communicable diseases
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This 18 page Study Guide was uploaded by Trisha Riley on Sunday October 4, 2015. The Study Guide belongs to NBSN3010C at University of Cincinnati taught by Butts in Fall 2015. Since its upload, it has received 22 views. For similar materials see Continuum of Care for Children and Families in Nursing and Health Sciences at University of Cincinnati.


Reviews for Test 3: Chapters 21, 22, 27, 28, & 29


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Date Created: 10/04/15
Chapter 21 Pain Assessment and Management Friday September 11 2 15 1 2 7 AM Neonatal Infant Pain Scale NIPS 0 Facial Expression 0 0 relaxed muscles 0 1 grimace O Scoring criteria I Restful face with neutral expression I Tight facial muscles furrowed brow chin and jaw I Note at low gestational ages infants may have no facial expression 0 Cry 0 0 No cry 0 1 Whimper o 2 Vigorous cry 0 Scoring criteria I Quite not crying I Mild moaning intermittent cry I Loud screaming rising shrill and continuous I Note silent cry may be scored if infant is intubated as indicated by obvious facial movements Breathing Patterns 0 0 Relaxed o 1 Changing in breathing O Scoring criteria I Relaxed usual breathing pattern maintained I Change in breathing irregular faster than usual gagging or holding breath 0 Arm Movements O 0 Relaxedrestrained with soft restraints o 1 Flexedextended O Scoring criteria I Relaxed no muscle rigidity occasional random movements of arms I Tense straight arms rigid or rapid extension and flexion Leg Movements O 0 Relaxedrestrained with soft restraints o 1 Flexedextended O Scoring criteria I Relaxed no muscle rigidity occasional random movements of legs I Tense straight arms rigid or rapid extension and flexion State of Arousal O 0 Sleepingawake o 1 Fussy O Scoring criteria I Quiet peaceful sleeping or alert and settled I Alert and restless or thrashing fussy FLACC Observational Pain Assessment Tool 2 months 7 years 0 Face 0 0 no particular expression or smile o 1 occasional grimace or frown withdrawn disinterested Pediatrics Exam 3 Page 1 o 2 frequent to constant frown clenched jaw quivering chin Legs O 0 normal position or relaxed o 1 uneasy restless tense o 2 kicking or legs drawn up Activity 0 0 lying quietly normal position moves easily 0 1 squirming shifting back and forth tense o 2 arched rigid orjerking O 0 no cry awake or asleep 0 1 moans or whimpers occasional complaint 0 2 crying steadily screams or sobs frequent complaints Consolability O 0 content relaxed o 1 reassured by occasional touching hugging or being talked to distractible o 2 difficult to console or comfort WongBaker FACES Pain Rating Scale Early as 3 years 2 1 E MU ll39LlFlT HURTS HLlFiTS HURTS HURTS HLlFiTS lLllT39Il39LE BllT LITI39ILE MHE EliIE Hi MFIE WHEI39LE ILDT WEREquot PCA Patient Controlled Analgesia 0 Schoolage 7 and older 0 Discontinued once child can take oral analgesics 0 Lockout Other Pain Management 0 Distraction holding touching position change pacifier in infants 0 Visualization 0 Heat or cold applications Pediatrics Exam 3 Page 2 Chapter 28 Alterations in Hematologic Function Wednesday September 16 2 15 838 AM Types of blood cells 0 Made in the bone marrow 0 RBC erythrocytes O Formed through the process of erythropoiesis O Polycythemia above average increase in the number of red cells in the blood 0 Erythropoietin hormone produced by the kidney that stimulates RBC production 0 WBC leukocytes fight infection 0 Platelets thrombocytes form hemostatic plugs to stop bleeding synthesized components of red bone marrow and stored in the spleen O Thrombocytopenia bleeding disorder caused by deficiency of platelets Leukopenia decrease in the number of white blood cells Neutropenia neutrophils of WBC are specifically decreased Anemia 0 Reduction in the number of RBC39s quantity of Hgb and volume of packed red cells to below normal levels 0 Iron Deficiency Anemia 0 Most common type of anemia 0 Most common nutritional deficiency in children 0 Occurs in children ages 6 months to 3 years and in adolescence 0 Children I Tachycardia I Pale 0 Sickle Cell Disease 0 Hereditary hemoglobinopathy partial or complete replacement of normal Hgb with abnormal Hb S hemoglobin S in RBC39s Autosomal recessive 25 chance if both parents have gene with each pregnancy Causes occlusion of small blood vessels ischemia and damage to organs Fever needs to be controlled hydrated pneumonia is common altitude ANY condition that increases the body39s need for oxygen or alters the transport of oxygen may result in sickle cell crisis 0 Types of SCD I Sickle Cell Anemia Hb SS III Most common 65 of cases III RBC39s are crescent shaped III Homozygous condition child has 2 sickle Hgb genes III Child subject to sickle cell crisis III Average life span of 45 years I Sickle C Disease Hb SC III Child inherits 1 HbS gene and one HbC gene 25 of cases 11 RBC39s are C shaped III Anemia is generally milder than is Hb SS disease III Painful crises occur about 50 as often as in Hb SS III Average life span of 65 years 0 Factors that contribute to sickling I Dehydration OOOO Pediatrics Exam 3 Page 3 I Infection I Altitude I Extremes in temperature I Vomiting I Emotional Distress I Fatigue I Alcohol consumption I Pregnancy I Elevated Hgb levels I Elevated reticulocyte counts I Excessive exercise or physical activity I Acidosis 0 Types of Sickle Cell Crises I VasoOcclusive Crisis Pain Crisis III Most common may last for days to weeks III Precipitated by dehydration exposure to cold acidosis or localized hypoxia III Caused by stasis of blood with clumping of cells in the microcirculation ischemia and infarction Thrombosis and infarction of local tissue may occur if the crisis is not reversed Cerebral occlusion can result in stroke Extremely painful Symptoms fever tissue engorgement painful swelling ofjoints in hands and feet priapism severe abdominal pain I Splenic Sequestration III Lifethreatening death can occur within hours III Caused by pooling of blood in the spleen since the spleen can hold much of the body39s blood supply cardiovascular collapse can result III Symptoms profound anemia hypovolemia shock I Aplastic Crisis III Diminished erythropoiesis and increased destruction of RBC39s III Triggered by viral infection or depletion of folic acid III Symptoms profound anemia pallor fatigue 0 Children with sickle cell disease are living longer because advancements in transfusions and infection prevention DUDE Clotting Disorders 0 Hereditary O Hemophilia I Xlinked disease males III Hemophilia A classic hemophilia is caused by deficiency of clotting factor VIII account for 85 III Hemophilia B Christmas disease is caused by deficiency of clotting factor IX accounts for 1015 I Hemophilia C is caused by deficiency of clotting factor X autosomal recessive disease occurs equally in males and females I Pathophysiology III Degree of bleeding is related to the amount of clotting factor dependent upon the phase of coagulation affected and the severity of injury III Potential complications internal hemorrhaging transfusion reactions hypovolemic shock and death I Clinical Manifestations III Do not demonstrate symptoms until after 6 months of age as they become Pediatrics Exam 3 Page 4 more mobile and incur injuries and bleeding from falls or from tooth eruption III Hemarthrosis spontaneous bleeding into a joint space ie knees ankles elbows RICE III Male children can have bleeding after circumcision III Other 5amp5 ecchymosis easy bruising nosebleeds hematuria spontaneous bleeding bleeding after tooth extraction minor trauma or minor surgical procedure III Subcutaneous intramuscular hemorrhages and GI bleeding can occur III Intracranial bleeding can occur 1830 mortality rate III Females who carry the trait do not usually manifest symptoms but can have prolonged bleeding during dental work surgery or trauma Diagnostic Tests III Can be done before birth through chrionic villus sampling or amniocentesis III Genetic testing can identify carriers in the family Clinical Therapy III Goal control bleeding by replacing the missing clotting factor III DDAVP desmopressin stimulates the release of factor VIII stored in the blood vessels therefore increasing the of available factor by about 3 times effective in some patients with mild to moderate hemophilia A III Children with severe hemophilia may be on a prophylactic regimen of factor replacement therapy mild to moderate may only receive episodic therapy III Prognosis has greatly improved due the availability of transfusion therapy mild to moderate hemophilia can lead normal lives today 0 Von Willebrand Disease 0 Acquired Most common hereditary bleeding disorder Variety of subtypes vWF von Willebrand factor a plasma protein and the carrier for clotting factor VIII Most common form is an autosomal dominant trait can occur in both genders gene located on chromosome 12 vWF is not sufficient in quantity or is dysfunctional leading to impaired clot forming and bleeding control Symptoms prolonged and excessive mucoustaneous bleeding In children gingival bleeding epistaxis bruising minor wounds or lacerations Increased bleeding can occur during surgery and dental extractions Affected teenage girls have menorrhea increased menstrual bleeding GI bleeding can also occur D EDD o Disseminated Intravascular Coagulation DIC Lifethreatening disease which occurs as a complication of other serious illness Clotting system is abnormally activated leading to widespread clot formation in small vessels throughout the body Most common cause is sepsis Caused by increased protease activity caused by unregulated release of thrombin increased thrombin Manifestations range from minor oozing to hemorrhage o Immune Thrombocytopenic Purpura ITP Increased destruction of platelets in the spleen platelet production in bone marrow is normal Platelets destroyed as a result of binding of autoantibodies to platelet antigens Once the rate of destruction exceeds the rate of production blood clotting slows Occurs annually in 4 to 5 per 100000 Cause is unknown usually follows an infectious illness Pediatrics Exam 3 Page 5 I Uncommon complication in 1 in 25000 after MMR I Symptoms multiple ecchymosis and petechiae mucosal bleeding 0 HenochSchonlein Purpura HSP I Vasculitis inflammation of the blood vessels I Most common in children affecting 14 per 100000 each year I Peak incidence of 5 to 6 years of age most affected are under 10 I Twice as common in males as females I Cause is unknown IgA is known to play a role in the development and generally follows an upper respiratory infection I Symptoms slightly raised purpuric lesions arthralgias colicky abdominal pain I Leads to endstage renal disease in 15 of children affected Types of Blood amp Products for Administration to Children with Hematologic Disorders Whole Blood To replace blood volume Generally given in hypovolemic shock Packed RBC39s Increased oxygencarrying capacity in anemia and some leukemia39s also given in case of hypovolemic shock Fresh Frozen To expand blood volume Plasma Albumin To expand blood volume in shock and trauma Factor VIII To treat factor VIII deficiency hemophilia A and von Willebrand Disease Concentrate Factor X To treat IXdeficiency hemophilia B Concentrate Pediatrics Exam 3 Page 6 Chapter 29 The Child with Cancer Wednesday September 16 2 15 924 AM Protocol plan of action for chemotherapy that is based on the results of staging type of cancer stage and location and particular cell type Clinical Manifestations Pain may result from neoplasm either directly or indirectly affecting nerve receptors through obstruction inflammation tissue damage stretching of visceral tissue or invasion of susceptible tissue can be located in any body part Cachexia syndrome characterized by anorexia weight loss anemia asthenia weakness and early satiety feeling of being full Anemia experienced during times of chronic blood loss or iron deficiency in chronic illness the body uses iron poorly also present in cancers of the bone marrow when the of RBC39s is reduced in part because of large numbers of other bone marrow products treatment of cancer often promotes further anemia due to bone marrow suppression Infection usually a result of an altered or immature immune system occurs when bone marrow cancers inhibit maturation of normal immune system cells can also occur in children who are treated with corticosteroids since the immune response is altered normal signs of infection may notappear Bruising ecchymosis can occur if the bone marrow cannot produce enough platelets petechiae may be present prolonged bleeding can occur after minor trauma Neurologic symptoms may result from impingement on the brain or nervous system signs of ICP decreased or altered consciousness eye abnormalities or other neurologic or behavior changes may be evident Palpable Mass may be present for certain cancers most commonly abdominal by may be the neck along lymph nodes or in other sites Tumor Lysis Syndrome Etiology Breakdown of Metabolic emergency resulting from the lysis dissolving or decomposing of tumor cells Releases high levels of uric acid potassium calcium and phosphates in the blood leading to hyperuricemia hyperphosphatemia hypocalcemia and hyperkalemia Low levels of sodium potentially leading to metabolic acidosis cardiac arrhythmias and renal failure Seen most commonly in children with nonHodgkin lymphoma especially Burkitt lymphoma Clinical Manifestations Clinical Therapy Nursing Management Hyperuricemia 0 Vigorous hydration with 24 0 Administer fluids malignant Hyperkalemia times maintenance fluid beginning before cells releases Hyperphosphatemia 0 Correction of electrolyte therapy intracellular Hypocalcemia imbalances Carefully measure I amp 0 components into the blood 0 Urine specific gravity should remain less than 1010 0 Monitor for desired and side effects of drug therapy 0 Administration of allopurinol or urate oxidase rasburicase to reduce conversion of metabolic by products to uric acid 0 Administration of calcium to treat hypocalcemia and resultant tetany Pediatrics Exam 3 Page 7 Electrolyte Cardiac arrhythmias 0 ECG monitoring 0 Administer electrolytes imbalance causes Impaired renal 0 Medications such as and medications metabolic acidosis function furosemide to facilitate 0 Urine pH should remain and serious Tetany neurologic potassium excretion 7075 abnormalities and mental status 0 Dialysis may be needed 0 Perform Trousseau and changes Chvostek signs for tetany monitoring and assess neurologic function 0 Perform mental status examination 0 Obtain laboratory specimens as needed Oncologic Emergencies 0 Result from cancer itself or as a side effect of treatment 0 Metabolic emergencies 0 Tumor lysis syndrome 0 Septic shock I During immune suppression vulnerable to overwhelming infection resulting in circulatory failure hypothermia or hyperthermia tachypnea mental changes inadequate tissue perfusion and hypotension I Can be fatal early and aggressive treatment needed I Factors inadequate neutrophil production abnormal granulocytes not able to be actively phagocytic erosions through normal barriers such as blood vessels and mucous membranes and altered bone marrow production caused by chemotherapy and some forms of radiation I Vigorously treated with antimicrobial therapy and hydration management 0 Large amounts of bone are destroyed by treatment leading to hypercalcemia I Most common in older children with ALL I Treatment hydration and adequate intake of phosphate by oral supplement I Some children develop SIADH and have excessive release of ADH resulting decreased urinary output leads to water intoxication 0 Hematologic emergencies 0 Bone marrow suppression or infiltration of brain and respiratory tissue with high numbers of leukemic blast cells hyperleukocytosis 0 Bone marrow suppression results in anemia and thrombocytopeniadecreased platelets with resultant coagulation disturbance and hemorrhage DICdisseminated intravascular coagulation lifethreatening GI and central nervous system bleeding stokes can occur Hyperviscosity of the blood can result from increased blood cellular components Treatment I Infusion of packed RBC39s for anemia and platelet transfusion vitamin K and fresh frozen plasma for thrombocytopenia and hemorrhage I Hyperviscosity treated by plasmapheresis hydroxyurea and close management of chemotherapy I Respiratory and other vital support is needed 0 Spaceoccupyinglesions 0 Extensive tumor growth can result in spinal cord compression ICP brain herniation seizures massive hepatomegaly GI obstruction cardiac and respiratory complications superior vena cava syndrome obstruction of SVC by tumor 0 Often caused by neuroblastoma medulloblastoma astrocytoma Hodgkin disease or 0000 Pediatrics Exam 3 Page 8 lymphoma 0 After biopsy treatment involves radiation chemotherapy and corticosteroids Side Effects of Chemotherapy 0 Bone Marrow Suppression 0 Nausea and Vomiting 0 Anorexia and Weight Loss 0 Mucositis 0 Constipation 0 Pain Common Cancers in Pediatrics Neuroblastoma 0 Solid tumor most commonly occurring outside the cranium of children 0 Responsible for 8 10 of childhood cancers and 15 of cancer deaths in children 0 Average age at diagnosis is 1722 months 0 Most common tumor in infants during the first year of life 0 Commonly smooth hard nontender mass that can occur anywhere along the sympathetic nervous system chain the abdomen is a frequent location other sites are adrenal thoracic and cervical areas 0 Etiology and Pathophysiology O Originates in primitive neurocrest cells of the autonomic nervous system that form the adrenal medulla paraganglia and sympathetic nervous system of the cervical sympathetic chain and the thoracic chain 0 About 50 develop in adrenal medulla 30 in the cervical thoracic or pelvic ganglia remaining are elsewhere along sympathetic chain 0 Lymph nose metastasis is common 0 Cause is unknown genetic defect found in many cases deletion of the short arm of chromosome 1 0 Clinical Manifestations 0 Location of the mass determines the symptoms 0 Retroperitoneal mass I Altered bowel and bladder function I Weight loss abdominal distention enlarged liver irritability fatigue fever 0 Mediastinal mass I Dyspnea or infection may occur I Neck and facial edema may result from superior vena cava syndrome when large 0 Metastasis to the bone I Malaise fever and a limp can occur I Bone marrow disease can manifest pancytopenia abnormal depression of all cellular blood components with neutropenia causing infections and anemia causing fatigue 0 Metastatic spread can result in an array of symptoms affecting multiple organs 0 Diagnostic Tests 0 Begins with careful HampP exam to identify changes in behavior 0 Routine blood cell counts are needed ie CBC with differential 0 Test may reveal anemia and thrombocytopenia O No classic WBC response thrombocytopenia may occur in association with disseminated intravascular coagulation o Leukocytosis increased leukocyte count and leukopenia decreased leukocyte count have Pediatrics Exam 3 Page 9 O 0 been observed with bone marrow involvement Serum electrolytes liver function studies LDH coagulation studies and urinalysis are performed Baseline cardiac function if doxorubicin will be used in treatment 0 Clinical Therapy O O O 0 Stage of tumor determines treatment protocol Lowrisk stages surgical excision of the mass Higher risk stages surgery followed by chemotherapy with a combination of drugs When the mass is large or wrapped around major blood vessels chemotherapy may be needed before surgery Chemotherapy may include I Cyclophosphamide I fosfamide I Doxorubicin I Cisplatin I Carboplatin I Teniposide I Etoposide Radiation is often used especially in disseminated disease or when tumors are unreceptive to chemotherapy Autologous stem cell transplantation may be performed for advanced disease sometimes followed by the biological modifier cisretinoic acid and fenretinide to promote apoptosis Protocols are determined by age of child tumor stage and molecular characteristics of the tumor Wilms Tumor Nephroblastoma 0 An intrarenal tumor common abdominal tumor of childhood and accounts for 6 of all childhood tumors 0 Incidence is approx 76 cases per 1 million children annually 0 Most currently seen in children between 2 and 3 years young ages more commonly seen in bilateral disease 0 Etiology and Pathophysiology O 0 Associated with several congenital anomalies I Aniridia absence of the iris I Hemihypertrophy abnormal growth of half of the body or a body structure I Genitourinary anomalies I Nevi I Hamartomas benign noduelike growths Genetic links chromosome deletions at 11p13 and 11p15 have been associated TP53 tumor suppressor gene and CTNBl an oncogenes have been related High incidence in BeckwithWiedeman syndrome characterized by macroglossia hemihypertorphy visceromegaly and hypoglycemia Most children with Wilms tumor have no other abnormalities Grows very quickly doubling its size in 11 to 13 days generally contributes to a large tumor by the time of diagnosis Chemotherapy drugs have significantly increased survival rates 90 survival rates 0 Clinical Manifestations 0 Usually asymptomatic firm lobulated mass located to one side of the midline in the abdomen Mass often discovered when parent is bathing or handling child or clothing is tight around the abdomen HTN caused by increased renin activity related to renal damage is reported in 25 of cases Hematuria or abdominal pain is sometimes present Pediatrics Exam 3 Page 10 Diagnostic Tests O 0 000 0 Ultrasound study of the abdomen and an intravenous pyelogram CT scanning or MRI of the lungs liver spleen and brain may be performed to identify any metastases Information from these is used to stage the tumor Complete blood count is obtained with BUN and Crt levels Liver function test Histologic exam for tissue typing once the tumor is removed Clinical Therapy O O O Multifaceted and increasing successful 90 of early stages and 70 of metastatic cases have longterm survival Surgery is performed to remove the affected kidney to examine the opposite kidney to remove lymph nodes for examination and to look for other sites of metastasis Total tumor is removed taking care not to rupture the tumor capsule Generally the entire kidney is removed when bilateral a kidneysparing procedure is needed Chemotherapy andor radiation is sometimes used before surgery to reduce the size of the tumor Radiation may follow surgery especially with disseminated disease Longterm complications liver damage portal HTN mild cirrhosis Acute Lymphoblastic Leukemia WBC39s are very high but are immature and nonfunctional no room for RBC39s or platelets Bruising and pale Accounts for 25 of all childhood cancer and 78 of leukemia in children Peak age at onset is 2 to 3 years More common in Whites and Boys Subtypes are based on the FrenchAmericanBritish system of classification FAB L1 L2 L3 Etiology and Pathophysiology 0 Increased risk in children with chromosomal defects Down Syndrome neurofibromatosis type 1 Bloom syndrome Shwachman syndrome Chromosomal abnormalities hyperploidy hypodiploidy translocations and deletions are present in most children Children with ataxiatelangiectasia congenital hypogammagloulinemia and WiskottAldrich syndrome are at increased risk Stem cells in the bone marrow produce immature WBCs that cannot function normally These proliferate rapidly by cloning instead of normal mitosis causing the bone marrow to fil with abnormal WBCs The abnormal WBCs spill into the circulatory system where they steadily replace the normally functioning WBCs the protective lymphocytic functions ie cellular and humeral immunity are reduced leaving the body vulnerable to infections Malignant WBCs rapidly fill the bone marrow replacing stem cells that produce erythrocytes RBCs and other products like platelets decreasing the amount of these products circulating Stem cells are replaced with leukemia commonly experience abnormal bleeding ecchymosis or petechiae because of reduced platelet amounts Clinical Manifestations 0 000000 Fever Pallor Ecchymosis Petechiae Bleeding Lethargy Malaise Pediatrics Exam 3 Page 11 OOOO Anorexia Large joint and bone pain Enlargement of the liver and spleen and changes in the lymph nodes lymphadenopathy If it infiltrates the CNS child may exhibit I Headache I Vomiting I Papilledema I Sixth cranial nerve palsy inability to move eye laterally These findings are caused by the leukemic cells massing and putting pressure on the nerves Testicles spinal cord and bone marrow are common sites of infiltration testicle has painless enlarging mass 0 Diagnostic Tests O O 0 Initially diagnosed by blood counts and bone marrow aspiration Blood counts reveal anemia thrombocytopenia and neutropenia Bone marrow aspiration is definitive reveals immature and abnormal lymphoblasts and hypercellular marrow of blast cells in marrow measured Neutropenia thrombocytopenia and anemia are commonly noted Abnormal lab findings elevated serum uric acid elevated calcium potassium and phosphorus levels 0 Clinical Therapy 0 O O 0000 Approached with analysis of molecular targeting for the origin and type of disease identified Radiation and chemotherapy combinations using information from these targeted molecular approaches Radiation used for CNS disease in Tcell leukemia and for testicular involvement Chemotherapy is organized into 4 phases I Induction I Consolidation I Delayed intensification I Maintenance of remission Prognosis is much improved with current therapy Important factor is the initial leukocyte count over 50000mm3 at diagnosis the worse the prognosis Low risk group survival is high at 90 Higher risk group survival rate is 7580 Approx 1520 of children have relapse within a year after completing treatment Children who relapse and achieve a second remission may have HSCT for treatment during remnssnon Familial Cancers Type of Cancer Retinoblastoma Wilms Tumor BeckwithWiedeman Syndrome Multiple endocrine neoplasia type 2 Familial Genetic Location Description Chromosome 13q14 RBl gene Tumor of the eye retina Chromosome 11p13 WT1 gene Tumor of the kidney Multiple abnormalities including tumors of kidney liver and adrenals omphalocele macroglossia Chromosome 11p15 genes CDKNlC KCNQlOTl LIT1 H19 IGF2 Chromosome 11q13 proto Medullary thyroid cancer oncogene RET mutation APC tumor suppression gene Invasive widespread colon polyps Pediatrics Exam 3 Page 12 adenomatous mutation on chromosome 5q21 polyposis LiFraumeni Chromosome 17p131 22q121 Earlyonset breast and other soft tissue cancers syndrome gene TP53 and CHEKZ Von HippelLindau VHL gene on chromosome Hemangioblastoma of retina cerebellum or disease 3p26p35 spinal cord renal cell carcinoma pheochromocytoma How to Compute an ANC 0 Blood component ratio of segmental neutrophils of bands immature neutrophil x WBC count 0 Should be greater than 1000 0 ANC less than 500 indicates risk for infection Pediatrics Exam 3 Page 13 Chapter 27 Alterations in Immune Function Friday September 18 2015 1010 AM De ne Antibodies proteins that works against antigens Antigens foreign substances that trigger and immune response Natural Immunity composed of the defenses present at birth ie intact skin body pH natural antibodies from mother inflammatory and phagocytic properties Acguired immunity consists of humoral antibodymediated and cellmediated immunity and is Passive immunity immunity passed from mother to the fetus Review Tables 271 272 and 273 AampP pediatric differences Classes of lmmunoglobulins lmmunoglobulin Location Action IgM Present in intravascular spaces Mediates cytotoxic response and activates blood and lymph complement First antibody produced with primary immune response IgG Present in all body fluids Active against bacteria bacterial toxins and viruses Activates complement Only immunoglobulin to cross the placenta IgA Present in secretions of GI Prevents binding of viruses to cells of the respiratory and GU tracts respiratory and GI tracts IgD Present in blood lymph and Function not fully understood surfaces of B cells IgE Present in internal and external Releases chemical mediators responsible for body fluids immediate hypersensitivity response DiGeorge Syndrome 0 Tcell disorder caused by chromosome deletion at 22q112 usually diagnosed soon after birth 0 Estimated incidence of 1 per 3000 to 6000 births 0 Characterized by absence or hypoplasia of parathyroid or thymus glands hypocalcemia with tetany with 24 to 48 hours after birth cardiac defects lowset ears hyperteloris widely set eyes viral and bacterial infections in the neonatal period 0 Generally mild to moderate decrease in Tcell counts 0 Prophylactic antibiotics are used to prevent bacterial infections 0 Children with partial DiGeorge treated with calcium and vitamin D supplements 0 Children with complete DiGeorge need thymus transplantation to survive Severe Combined Immunodeficiency Disease SCID 0 Most severe form of primary immune deficiency is a congenital condition characterized by absence of both humoral and cellular immunity that is manifested by lack of appropriately Pediatrics Exam 3 Page 14 functioning T cells and B cells Occurs in Xlinked recessive and autosomal recessive forms May result in chromosomal abnormalities More common in males than females estimated to occur in 1 per 50000 live births Without treatment children usually do not survive more than 1 year Etiology and Pathophysiology O Caused by genetic mutations that lead to impaired lymphoid development in children with low T and NK cells 0 B lymphocytes present may appear normal in number function is compromised due to severe Tcell deficiency Clinical Manifestations 0 Symptoms develop early in life 0 Infant demonstrates susceptibility to infection presents during first few months of life with persistent respiratory infection and diarrhea O Recurrent oral candidiasis failure to thrive skin infections also occur frequently 0 Failure to completely recover from infection frequent reinfection infection with viruses ie cytomegalovirus amp bacterium Pneumocystis O Susceptible to serious infections ie meningitis skin or organ infection osteomyelitis or sepsis Diagnostic Tests 0 Marked reduction in lymphocyte counts is indicative 0 Generally have very few T cells and NK cells 0 Blymphocyte may be decreased elevated or normal although they do not function normally 0 Immunoglobulin levels are significantly reduced 0 Diagnosis made after extensive lab testing 0 Chest radiograph is conducted to assess thymus size Clinical Therapy 0 Standard therapy administration of IV immune globulin to provide protection until humoral immunity is established 0 Hematopoietic stem cell transplantation offers the best hope 0 Transplantation restores Tcell function and new cells appear in 3 to 4 months after infusion of the donor stem cells 0 Prognosis is poor without aggressive therapy and transplant 0 Prevention and prompt treatment of infection is essential antibiotic therapy is targeted are infectious agents WiskottAldrich Syndrome WAS Combined immunodeficiency syndrome Xlinked recessive disorder that occurs in males and causes mutation in the WAS gene and changes in the WAS protein Gene resides on Xp11221123 Incidence is 4 in 1 million live male births IgM levels are low IgG levels are normal or slightly low IgA and IgE levels are elevated Diagnosis is made in early neonatal period on the basis of thrombocytopenia which leads to bleeding petechiae hematuria bloody diarrhea hematemesis Thrombocytopenia and related symptoms Eczema and recurrent infections in infancy and childhood ie otitis media bacterial pneumonia skin infections Treatment antibiotic prophylaxis platelet transfusions IVIG O Splenectomy may reduce the risk of bleeding done sparingly due to risk for infection 0 Treatment of choice and only cure hematopoietic stem cell transplantation HSCT Pediatrics Exam 3 Page 15 I Child is at risk for rejection and graftversushost disease Human Immunodeficiency Virus HIV pg 872 Acquired Immunodeficiency Syndrome AIDS pg 872 Autoimmune Disorders Immune system damaged by pathologic changes immune response may occur to some of the body39s own proteins resulting in production of autoantibodies Conditions in which the body directs the immune response against itself identifying quotselfquot as quotnonselfquot Primary feature tissue injury caused by a probable immunologic reaction of the host with its own tissues Structural or functional changes occur as immune cells attack other cells in the body Autoimmune disorders are grouped into systemic and organspecific disease Systemic diseases generally involve more than one organ 0 Systemic lupus erythematosus SLE 0 Juvenile arthritis Organspecific disease primarily affect a single organ 0 Type 1 diabetes 0 Thyroiditis Immune thrombocytic purpura immune disease affecting blood platelets and clotting Psoriasis Tcell mediated autoimmune disease of the skin Systemic Lupus Erythematosus SLE Chronic inflammatory autoimmune disease of unknown origin that involve many organ systems Primarily diagnosed in adulthood approx 20 of cases are diagnosed prior to age 16 Effects 15 million people in the US More common among Blacks Native Americans Hispanics and Asians than Whites 7 times more common in females than males Etiology and Pathophysiology o Etiology is unknown 0 Genetic component is suspected and initiated by outside environmental agent 0 Body produces autoantibodies and combines with antigens to form immune complexes 0 These antigenantibody complexes are deposited in the connective tissue triggering an inflammatory response Chronic inflammation destroys connective tissue 0 Tissue damage varies according to the organ involvement tissues most likely affected are the small blood vessels glomeruli joints spleen and heart valves 0 Many systems can be affected organ damage with Clinical Manifestations 0 May be acute with onset of nephritis arthritis or vasculitis may be noted as a gradual onset with nonspecific symptoms 0 Symptoms depend on the organ involved and amount of tissue damage I Fever I Fatigue I Malaise I Weight loss I Rash I Arthritis I Nephritis 0 Pediatrics Exam 3 Page 16 Butterfly rash on the face with pinkred rash over the bridge of the nose extending to the cheeks May have anemia leukopenia and thrombocytopenia Renal disease leading cause of morbidity and mortality in these children CNS disorders may occur I Headaches I Mood disorders I Seizure disorders I Cerebrovascular disease Characterized by periods of remission and exacerbations flares triggered by a variety of causes I Sun exposure I Upper respiratory infections I Stress I Particular events activities or situations may also be identified as triggers 0 Diagnostic Tests O 0000 Blood tests reveal anemia elevated BUN abnormal plasma proteins abnormal ESR presence of antinuclear antibodies positive LE cell reaction indicates nonspecific inflammation Coombs test is positive Radiologic exams CT and MRI of affected joints Urinalysis may reveal proteinuria Diagnosis determined based on the presence of at least 4 of the following I Butterfly malar rash I Red raised patches discoid rash I Photosensitivity I Mouth or nasal ulcers I Arthritis I Serositis I Renal disorders I Neurologic disorders I Blood disorders I Immunologic disorders I Positive antinuclear antibody 0 Clinical Therapy 0 Diet may be restricted if child has excessive weight gain or fluid retention from steroids and renal damage Prognosis depends on the severity of the internal organ involvement Was once considered fatal now 5 year survival rates are as high as 100 10 year 85 15 years 80 Kidney failure is managed by hemodialysis or peritoneal dialysis Renal transplantation has been very successful for treatment of renal failure secondary to lupusnephn s Pediatrics Exam 3 Page 17 Chapter 22 Immunizations and Communicable Diseases Friday September 18 2 15 225 AM 0 5ths disease 0 Chicken pox 0 Meningococcemia can die within 46 hours purpura spreads quickly 0 Rotavirus bright green stool 0 Roseola pinkred rash on trunk 810 mo Old sudden high fever acts normal after fever subsides maculopapular rash appears 0 Rubella German Measles pregnant women STAY AWAY 0 Rabies 0 West Nile Virus Pediatrics Exam 3 Page 18


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