PEDS Module 4: Altered Immune System

At what age is hemoglobin screening typically performed?

At 12 months of age

What are common behavioral signs of immunological disorders?

Irritability and anorexia

What cardiovascular signs may appear with immunological disorders?

Tachycardia and systolic murmur

What nail changes are associated with immunological or nutritional deficiencies?

Brittle and concave nails

What are common muscular findings in children with immune disorders?

Poor muscle tone

Why are children with immunological disorders infection-prone?

Due to decreased immune function or insufficient immune components

What skin appearance is associated with immune disorders?

Porcelain-like skin

What are two physical signs related to fluid or nutrition issues in immunological disorders?

Edema and growth restrictions

Which serum levels are commonly decreased in immunological disorders?

Protein, albumin, gamma globulin, and transferrin

What developmental impact may be observed in children with immune disorders?

Delayed learning

What is a common finding in a CBC with RBCs for individuals with iron deficiency?

RBCs may be borderline or reduced.

How are RBCs typically characterized in cases of iron deficiency?

BCs are small in size.

What is the normal range for serum iron in newborns?

100–250 mcg/dL

What is the normal serum iron range for infants?

20–105 mcg/dL

What is the normal serum iron range for individuals aged 10–14?

20–145 mcg/dL

What is the normal range for serum ferritin in individuals aged 6 months to 15 years?

7–140 ng/mL

What does a total iron-binding capacity test measure?

It measures the ability of the blood to bind iron.

What are erythropoietin levels used to assess?

They are used to evaluate red blood cell production.

What does reticulocyte hemoglobin content indicate?

It indicates the amount of hemoglobin in immature red blood cells, often used to assess iron deficiency.

What do infants' immune systems consist mostly of?

Maternal antibodies.

How is IgG transferred to infants?

Through the maternal placenta and breast milk.

How is an infant's immune system shaped?

It is shaped by pathogen exposure and illness.

Why are adults less frequently sick compared to infants?

Due to retained memory immunit

How do vaccines help children?

Vaccines help develop immunity against common childhood illnesses.

What foods should be increased in consumption for iron and folate intake?

Foods rich in iron and folate.

What are some iron-rich foods?

Organ meats, shellfish, poultry, legumes, molasses, and fortified cereal.

What are some folate (folic acid)-rich foods?

Legumes, liver, dark green/leafy vegetables, lean beef, and potatoes.

When are Red Blood Cells (RBCs) indicated for transfusion?

For hemoglobin <8 g/dL, hypovolemia due to acute blood loss, chronic anemia, or postoperative anemia.

What is an autologous blood transfusion?

A transfusion of self-donated blood, typically used for general scheduled surgeries where blood transfusion might be necessary.

When is Whole or Packed Red Blood Cells (PRBC) with Fresh Frozen Plasma (FFP) indicated?

For hypovolemia due to blood loss, hematocrit <33%, or intraoperative hemoglobin <10 g/dL.

When are Platelets indicated for transfusion?

For a platelet count <20,000, active bleeding with disseminated intravascular coagulation (DIC), or prevention of bleeding due to thrombocytopenia.

: What does Fresh Frozen Plasma (FFP) replace?

It replaces deficiencies in Factor II, V, VII, IX, X, XII, Protein C, Protein S, and corrects prolonged PT or PTT.

When is Fresh Frozen Plasma (FFP) indicated for use?

In the replacement of clotting factor deficiencies, prolonged PT or PTT, or warfarin overdose correction.

When is Cryoprecipitate (CRYO) indicated for use?

For fibrinogen levels <150 mg/dL with active bleeding or as prophylaxis for Hemophilia A (Factor VIII deficiency).

When are Granulocytes (White Blood Cells) indicated for transfusion?

For bacterial or fungal sepsis unresponsive to medication or severe infection unresponsive to therapy.

What is Factor VII used for?

To treat Factor VII deficiency or Factor VII or IX inhibitors.

When is Factor IX Concentrate (Prothrombin Complex) used?

For the treatment of Hemophilia B or Hemophilia A with Factor VII inhibitors.

What is the purpose of Intravenous Immunoglobulin (IVIG)?

To treat congenital or acquired antibody deficiency, idiopathic thrombocytopenia (ITP), and severe bacterial infections in newborns.

What are some hematologic disorders?

Iron Deficiency Anemia, Acquired Thrombocytopenia, Sickle Cell Disease, Aplastic Anemia, Hemophilia, and Lead Poisoning.

What is the most common type of anemia?

Iron Deficiency Anemia.

What is the primary cause of Iron Deficiency Anemia in infants?

It is more common in infants who consume cow’s milk before 1 year of age.

What are common signs and symptoms of Iron Deficiency Anemia?

• Decreased RBC count

• Low serum iron

• Fatigue

• Weakness

• Shortness of breath

What are some interventions for managing Iron Deficiency Anemia?

• Iron supplementation through dietary changes

• Oral iron supplementation

• Intramuscular (IM) iron supplementation

• Iron-rich formula or iron supplementation for breastfed infants

• Educate parents on incorporating iron-rich foods into their child’s diet

What causes Acquired Thrombocytopenia?

Decrease in platelet count due to infections (e.g., Rocky Mountain spotted fever, Colorado tick fever, malaria) or exposure to certain drugs or chemicals.

How can Acquired Thrombocytopenia be managed?

It is often reversible once the causative agent is eliminated.

What are common signs and symptoms of Acquired Thrombocytopenia?

• Spontaneous bleeding (e.g., nosebleeds/epistaxis)

• Blood in feces

• Bleeding from mucous membranes

• Bleeding gums

• Bleeding from scabbed areas

• Hematemesis (vomiting blood)

• Petechiae and purpura (notable on face, thorax, extremities)

What are interventions for Acquired Thrombocytopenia?

• Gentle mouth care using a soft toothette

• Educate parents to avoid:

  • Intramuscular injections

  • Aspirin

  • Steroids

• Support positive body image and encourage safe, modified activities.

What is Sickle Cell Disease?

It is the most common genetic condition, caused by the partial or complete replacement of normal hemoglobin A with abnormal hemoglobin S, leading to red blood cells deforming from round to sickle (crescent) shape and causing tissue damage throughout the body.

What are some complications of Sickle Cell Disease?

• Delayed growth and sexual maturation

• Acute and chronic pulmonary dysfunction

• Stroke

• Aseptic necrosis (hip and shoulder)

• Retinopathy

• Dermal ulcers

• Chronic pain

What are common signs and symptoms of Sickle Cell Disease?

• Obstructed circulation with hypoxia and necrosis

• Lack of oxygen

• Acute chest syndrome

• Cerebrovascular accident (CVA)

• Altered neurological function such as:

  • Headache

  • Weakness in extremities

  • Seizures

  • Aphasia

  • Abnormal behavior

What are some interventions for managing Sickle Cell Disease?

• Analgesics for pain management

• IV hydration and oral fluids

• Blood transfusions for acute anemia

• Respiratory assessment and supplemental oxygen as needed

• Educate families on:

  • Genetic counseling

  • Lifelong nature of the disease

  • Signs and symptoms of infection

  • When to seek immediate care

What is Aplastic Anemia?

Aplastic anemia is a deficiency in the formation of blood elements, involving pancytopenia (decreased leukocytes, platelets, and erythrocytes), resulting in physiological anemia.

What is pancytopenia in Aplastic Anemia?

Pancytopenia refers to the decrease in all three blood cell types: leukocytes (white blood cells), platelets, and erythrocytes (red blood cells).

What are common signs and symptoms of Aplastic Anemia?

• Fatigue

• History of injuries that do not heal

What are key interventions for managing Aplastic Anemia?

• Monitor response to immunosuppressive therapy

• Monitor blood transfusions and IV access for infection prevention

• Emphasize hand hygiene to reduce infection risk

• Educate parents about:

  • Possibility of bone marrow transplant

  • Importance of participation in daily care

  • Maintaining a well-balanced diet

What causes Hemophilia?

Hemophilia is caused by Factor VIII deficiency (Hemophilia A), resulting in clotting factors that are present but function poorly, leading to uncontrolled bleeding.

How are Hemophilia A and B inherited?

Hemophilia A and B are X-linked disorders, meaning:

• Males are affected

• Females are carriers

What are common signs and symptoms of Hemophilia?

• Frequent nosebleeds

• Easy bleeding and bruising

• Hemarthrosis (bleeding into joints—knee, ankle, elbow)

• Hematomas with spontaneous pain, swelling, and limited motion

• Spontaneous hematuria (blood in urine)

What are key interventions for managing Hemophilia?

• Administer Factor VIII concentrate

• Administer corticosteroids for hematuria and hemarthrosis

• Avoid medications like codeine and aspirin due to interference with platelets

• Administer DDAVP (Desmopressin) to increase plasma Factor VIII in mild cases

What causes Lead Poisoning?

Lead poisoning is caused by chronic ingestion or inhalation of materials containing lead, resulting in both physical and mental dysfunction.

Why are children with iron deficiency at a higher risk of lead poisoning?

Children with iron deficiency absorb lead more readily, increasing the risk of poisoning.

When is routine screening for lead poisoning recommended?

Routine screening is recommended at 12 months of age

What are some common signs and symptoms of Lead Poisoning?

• Abdominal pain

• Vomiting

• Constipation

• Anorexia

• Headache

• Fever

• Lethargy

• Hyperactivity

• Impulsiveness

• Irritability

• Loss of developmental progress

• Hearing impairment

• Learning difficulties

What are the key interventions for treating Lead Poisoning?

• Prevent further exposure to lead

• Chelation therapy with:

  • Calcium disodium edetate

  • Succimer (injected into large muscle mass)

What should parents be educated about in relation to Lead Poisoning?

• Removal of lead hazards (e.g., paint chips)

• Relocation of the child to prevent further exposure

What causes Acquired Immunodeficiency Syndrome (AIDS)?

AIDS develops from HIV infection, often transmitted from mother to infant in utero. HIV affects T-lymphocytes.

What bodily fluids can HIV be found in?

HIV can be found in blood, semen, vaginal secretions, and breast milk.

How does acute HIV infection affect the body?

Acute infection increases viral load, and the virus incubates over months to years. It can also cause neutropenia.

What are common signs and symptoms of AIDS in children?

• Lymphadenopathy

• Mucocutaneous eruptions

• Failure to thrive

• Delayed development

• Hepatosplenomegaly

• Oral candidiasis

• Parotitis

• Chronic or recurrent diarrhea

What are key interventions for managing AIDS in children?

• Administer combination antiretroviral therapy

• Continue childhood immunization schedule

• Promote high-calorie, nutrient-dense foods

What should parents be educated about regarding AIDS?

• Preventing opportunistic infections

• Managing severe immune suppression

What is a neoplasm?

A neoplasm is abnormal tissue growth, which can be benign or malignant (cancerous).

What is Leukemia?

Leukemia is a type of cancer that affects the blood and bone marrow, leading to the production of abnormal white blood cells.

What is Acute Lymphoblastic Leukemia (ALL)?

ALL is a type of leukemia that involves an overproduction of immature lymphocytes, most commonly affecting children.

What is Acute Myeloid Leukemia (AML)?

AML is a fast-growing cancer of the blood and bone marrow that affects myeloid cells, leading to the production of abnormal white blood cells.

What is Lymphoma?

Lymphoma is cancer that begins in the lymphatic system, affecting lymph nodes and lymph tissues.

What is Hodgkin’s Lymphoma?

Hodgkin's lymphoma is a cancer of the lymphatic system characterized by the presence of Reed-Sternberg cells.

What is Non-Hodgkin’s Lymphoma?

Non-Hodgkin's lymphoma refers to a diverse group of blood cancers that do not involve Reed-Sternberg cells.

What is a Brain Tumor?

A brain tumor is an abnormal growth of cells in the brain that can be benign or malignant, affecting brain function.

What is Wilm’s Tumor?

Wilm's tumor is a rare kidney cancer that primarily affects children, typically between the ages of 3 and 4.

What is a neoplasm?

A neoplasm is uncontrolled, abnormal cell growth that forms a tumor, which can be benign or malignant (cancerous).

What is metastasis in relation to cancer?

Metastasis refers to the spread of cancer cells from the original tumor to other areas of the body, invading surrounding tissues.

What are some common causes of neoplasms?

Neoplasms are often linked to genetic or hereditary conditions, although the exact causes of most cancers remain unknown.

What are some common signs and symptoms of a neoplasm?

Pain, cachexia with anorexia, weight loss, weakness, anemia, bruising, and hematuria.

What are some interventions for managing neoplasms?

Administer chemotherapy, IV fluids, hydration, antiemetics, and promote nutritional intake with increased calorie foods.

What are important educational points for parents of children with neoplasms?

Educate on central line infection prevention and provide developmentally appropriate education to the child.

What is leukemia?

Leukemia is a group of malignant diseases affecting the bone marrow and lymphatic system, characterized by the overproduction of white blood cells (WBCs), though the actual WBC count may be low.

What percentage of childhood cancers is leukemia responsible for?

Leukemia represents 29% of all childhood cancers.

What are the common signs and symptoms of leukemia?

Anemia, fatigue, pallor, dyspnea, cardiac dilation, anorexia, respiratory distress, neutropenia with infections, thrombocytopenia, hematuria, hepatosplenomegaly, enlarged lymph nodes, nausea, vomiting, diarrhea, headache, and irritability.

What are some interventions for leukemia?

Administer chemotherapy, blood transfusions, and educate parents on infection prevention and chemotherapy side effects.

What should parents be taught regarding leukemia treatment?

Parents should be taught to recognize signs of oncological emergencies and to understand chemotherapy side effects.

What is Acute Lymphocytic Leukemia (ALL)?

ALL affects the bone marrow and B or T cells and is the most common type of leukemia in children.

What are the interventions for Acute Lymphocytic Leukemia (ALL)?

Administer chemotherapy, prevent mouth bleeding, and monitor for infections.

What is Acute Myeloblastic Leukemia (AML)?

AML involves immature myeloid cells, including RBCs, WBCs, and platelets.

What are the interventions for Acute Myeloblastic Leukemia (AML)?

Administer combination chemotherapy, use of PPE to prevent infection, and support nutrition and healthy elimination patterns.

What is Lymphoma?

Lymphoma is cancer of the immune system, where malignancy results from failed apoptosis and uncontrolled growth.

What are the two main types of Lymphoma?

The two main types are Hodgkin's Lymphoma and Non-Hodgkin's Lymphoma.

What are the common signs and symptoms of Lymphoma?

Enlarged cervical or supraclavicular lymph nodes, airway obstruction, cranial nerve palsies, fever, weight loss, night sweats, fatigue, abdominal discomfort, anorexia, nausea, and pruritus.

What are the interventions for Lymphoma?

Administer chemotherapy, give pneumococcal and meningococcal immunizations, use standard precautions to prevent infection, and educate parents on radiation therapy effects and the child's decreased activity tolerance.

What is Non-Hodgkin’s Lymphoma?

Non-Hodgkin’s lymphoma arises in peripheral lymph nodes, spreads early and widely, and can involve the chest/mediastinum. Staging is based on the Revised European-American Lymphoma system.