PEDS EXAM 3 PERIOD

Legg-Calve-Perthes Disease

  • Definition: A condition involving limited blood flow to the femoral head leading to tissue death, primarily affecting males aged 4-8.

  • Symptoms:

    • Painless limp

    • Hip pain or stiffness

    • Decrease in hip range of motion (ROM)

    • Resulting in a shorter affected leg

  • Genetic Aspect: Not genetic; more common in males.

  • Diagnosis:

    • Via X-ray, CT scan, MRI

    • Ortolani test

  • Treatment Options:

    • Activity restriction

    • NSAIDs for pain relief

    • Physical therapy

    • Osteotomy

    • Limiting weight-bearing activities

    • Use of braces/casts

    • Box extension traction

    • Position adjustments to avoid pressure sores.

Clubfoot

  • Definition: A congenital deformity wherein the foot is turned inward.

  • Risk Factors:

    • Family history of clubfoot

    • Maternal health issues such as gestational diabetes.

  • Prevalence: More common in males.

  • Symptoms: Affected foot appears turned inward; potentially smaller calf muscle.

  • Diagnosis: Visual inspection and ultrasound during pregnancy.

  • Treatment: Serial casting followed by bracing.

  • Patient Education: Importance of compliance with bracing.

Osteogenesis Imperfecta

  • Definition: A genetic disorder affecting collagen synthesis, resulting in brittle bones and frequent fractures.

  • Symptoms:

    • Bone deformities

    • Blue sclera

    • Short stature

    • Hearing loss

  • Diagnosis:

    • X-ray

    • CT scan

    • MRI

    • Bone biopsy.

  • Treatment: Supportive care including bisphosphonates, bracing, splinting, surgery, physical/occupational therapy, and assistive devices.

  • Education for Caregivers: Handle affected children gently.

Rickets

  • Definition: A disorder characterized by bone loss due to vitamin D deficiency.

  • Causes:

    • Insufficient sunlight exposure

    • Poor dietary intake of vitamin D

    • Disorders affecting vitamin D absorption (e.g., celiac disease, cystic fibrosis).

  • Symptoms:

    • Bone deformities such as bowed legs and knocked knees

    • Increased alkaline phosphatase levels

    • Decreased vitamin D levels.

  • Diagnosis: Recognized primarily through clinical signs and symptoms.

  • Treatment: Supplementation of vitamin D, increased sunlight exposure, and dietary changes.

Down Syndrome

  • Definition: A chromosomal condition characterized by trisomy of chromosome 21, leading to physical and developmental delays.

  • Risk Factors:

    • Family history

    • Advanced maternal or paternal age.

  • Signs and Symptoms:

    • Physical features: broad flat nose, low-set ears, protruding tongue, single uninterrupted palmar crease.

    • Associated health conditions: GI disorders (e.g., GERD, celiac disease), constipation, seizures, hearing and vision issues, congenital heart defects, intellectual and developmental disabilities.

  • Diagnosis: Prenatal testing and screening methods.

  • Treatment: Symptomatic management through specialists including GI, cardiology, physical/occupational therapy, and speech therapy.

  • Nursing Care: Provide family support, education, and counseling options.

Fragile X Syndrome

  • Definition: An X-linked dominant genetic disorder caused by a mutation on the X chromosome.

  • Prevalence: Males exhibit more severe symptoms than females.

  • Physical Features: Long face, prominent forehead, protruding ears.

  • Symptoms: Cognitive impairments, speech delays, poor eye contact, hyperactivity.

  • Diagnosis: Prenatal screening and testing.

  • Treatment: Symptomatic management with medications including antidepressants, antipsychotics, and ADHD medications;

  • Nursing Role: Referrals to speech, behavioral, and occupational therapy.

Duchenne Muscular Dystrophy

  • Definition: A progressive neuromuscular disease with an X-linked recessive inheritance pattern.

  • Onset: Typically between ages 3-5 years.

  • Symptoms: Atrophy of muscular and cardiac tissues, weakness, delayed motor skills, cognitive impairment, waddling gait, speech difficulties.

  • Diagnosis: Genetic testing and elevated creatine kinase (CK) levels.

  • Treatment Options: Palliative care; most patients typically do not survive past their twenties due to complications such as respiratory muscle weakness.

  • General Implications: Muscular dystrophy results in muscle fat replacement, decreased life expectancy (15-30 years), obesity risk, scoliosis.

Muscular Dystrophies Classification

  • Fascia-humeral scapular dystrophy:

    • Autosomal dominant inheritance.

    • Early adolescence onset.

    • Normal life expectancy with slow disease progression.

  • Limb-girdle muscular dystrophy:

    • Affects ability to walk and hand positioning (upward).

    • Onset typically after age 12.

    • Mild cognitive delays.

Hip Dysplasia

  • Definition: A condition characterized by dislocation or subluxation of the hip joint.

  • Symptoms: One leg may appear longer than the other with noticeable dislocation.

  • Diagnosis: Utilization of Barlow’s test for evaluation.

  • Treatment: Often involves the use of a Pavlik harness to promote hip joint relocation by maintaining abduction.

Spina Bifida

  • Definition: A neural tube defect associated with genetic predisposition.

  • Risk Factors: Vitamins B12 and folic acid deficiency, malnourishment, or medications that inhibit folic acid absorption.

  • Types:

    • Occulta: Minor defect with minimal protrusion and symptoms (e.g., discoloration, texture changes on skin along spine).

    • Meningocele: Meningeal sac protrusion may lead to motor and sensory deficits.

    • Cystica: Severe form with visible spinal protrusion, bowel and bladder control loss, flaccid muscles, and absent reflexes.

  • Diagnosis: Screening during the second trimester and physical examination upon birth.

Cerebral Palsy

  • Definition: A condition characterized by paralysis, abnormal sensation, and involuntary movements.

  • Risk Factors: Maternal drug use, malnutrition, and genetic mutations, among others.

  • Symptoms: Generally low reflexes in newborns, potential cognitive impairments.

  • Varieties: Spastic (muscle spasms, difficulty in motor skills), dyskinetic (twisted movements), ataxic (lack of purposeful movements).

  • Nursing Care: Assistance with activities of daily living (ADLs) and passive range of motion (ROM) exercises.

Seizures

  • Definition: Seizure episodes can be triggered by low sodium levels, febrile conditions, or other health issues.

  • Status Epilepticus: Definition of seizures lasting longer than five minutes.

  • Management of Seizures:

    • Immediate treatment with medications such as valproic acid, Keppra, and Diazepam.

  • Febrile Seizures: Typically begin at 102°F, highest risk observed in children aged 6 months to 5 years.

Marfan's Syndrome

  • Definition: An inherited disorder predominantly affecting connective tissues.

  • Characteristics: Individuals may present as tall, with long limbs, heart murmurs, and potential scoliosis.

  • Aorta: May necessitate surgical intervention.

  • Diagnosis: Achieved through echocardiograms, CT scans, MRIs, and genetic testing.

  • Treatment Goals: Management includes medications to lower blood pressure, surgery for aortic repair, and regular spinal examinations.

Disorders Related to Dwarfism

  • Types:

    • Disproportionate dwarfism: Some body parts are smaller relative to others.

    • Proportionate dwarfism: All body parts are uniformly small.

  • Symptoms: Limited mobility, bowed legs, and a head disproportionately larger than the body.

  • Treatment: Growth hormone therapy is often recommended.

Fractures and Osteomyelitis

  • Fractures: Treatment may involve open reduction with internal fixation using pins, plates, screws, and rods. Monitoring of neurovascular status is vital, employing the 5 P’s (Pain, Pulse, Pallor, Paresthesia, Paralysis).

  • Osteomyelitis: Definition of a bone infection characterized by swelling, warmth, and bone pain.

    • Risks: Risk factors include deep wounds, weakened immune systems, and certain conditions.

    • Complications: Bone death, septic arthritis, and long-term osteomyelitis may arise.

    • Diagnosis: Notably through imaging techniques and biopsies.

    • Treatment: Surgery to remove infected areas and long-term antibiotic therapy is recommended.

Leukemia

  • Prevalence: The most common childhood cancer, constituting 30% of childhood cancers.

  • Mechanism: Characterized by the overproduction of dysfunctional white blood cells due to bone marrow issues.

  • Symptoms: Include pallor, fatigue, lymphadenopathy, bone pain, and abnormal bleeding.

  • Risks: Previous cancer therapies, genetic disorders, exposure to toxins.

  • Diagnosis: Primarily through physical exams and blood tests.

  • Treatment Options: Chemotherapy, targeted therapies, bone marrow transplants.

Lymphoma

  • Hodgkin’s Lymphoma: Characterized by Reed-Sternberg cells, typically starts in lymph nodes around the chest area.

    • Symptoms: Swollen lymph nodes, weight loss, night sweats.

    • Diagnosis: Blood tests, CT/PET scans, biopsies.

  • Non-Hodgkin's Lymphoma: Various body sites with symptoms similar to Hodgkin’s but often harder to detect.

    • Diagnosis and Treatment: Similar methods as Hodgkin’s, often involving a “watch and wait” approach.

Sickle Cell Disease

  • Definition: An inherited disorder causing red blood cells to take on a sickle shape, leading to potential blockage of blood flow.

  • Symptoms: Fatigue, pain episodes, increased infection risks, delayed growth.

  • Diagnosis: Via blood tests including newborn screenings and amniotic fluid analysis.

  • Management: Pain management and preventive measures to reduce complications.

Hemophilia

  • Definition: A genetic disorder characterized by deficiencies in clotting factors, leading to increased bleeding risks.

  • Types: Women typically carry the trait while men express symptoms.

Neutropenia

  • Definition: A condition associated with insufficient neutrophils, increasing infection risk.

  • Nursing Care: Care strategies include monitoring for fever and implementing infection control measures.

  • Treatment: Filgrastim is commonly administered to boost neutrophil production.

Types of Leukemia: AML and ALL

  • Acute Myeloid Leukemia (AML): More common in adults, generally fast-growing and associated with ages over 65.

  • Acute Lymphoblastic Leukemia (ALL): The most common form of childhood leukemia characterized by the overproduction of lymphoid cells.

Arthritis

  • General Definition: Refers to a variety of conditions resulting in joint inflammation.

  • Common Types:

    • Osteoarthritis: Characterized by cartilage deterioration.

    • Rheumatoid Arthritis: An autoimmune condition targeting joint linings.

    • Gout: Resulting from uric acid crystal accumulation.

  • Symptoms: Joint pain, stiffness, swelling, and decreased range of motion.

  • Risk Factors: Family history, age, previous injuries, obesity.

  • Diagnosis and Treatment: Involves laboratory tests, imaging, and treatment may involve NSAIDs, exercise, and patient education.

HIV Infection

  • Definition: Human Immunodeficiency Virus, which harms the immune system by destroying CD4 T cells.

  • Transition to AIDS: Occurs when CD4 count falls below 200.

  • Transmission: Primarily through bodily fluids.

  • Symptoms: Fever, sore throat, swollen lymph nodes, fatigue, muscle/joint pain.

  • Complications: Varied opportunistic infections and diseases.

  • Diagnosis: Blood tests assessing antibodies and viral load.

  • Management: Antiretroviral therapy (ART) and lifestyle adjustments.

Electrophoresis Tests

  • Application: Used to diagnose various conditions including blood disorders, cancers, immune disorders, and nutritional deficiencies.

Pneumonia Vaccine

  • Purpose: Covers various strains of bacterial pneumonia to prevent related illnesses such as ear and sinus infections.

Lupus

  • Definition: An autoimmune disorder where the immune system attacks body tissues.

  • Symptoms: Inflammation affecting joints, skin, and various organs.

  • Diagnosis: Challenging, often includes patient history and serological testing.

  • Treatment: Symptomatic management with NSAIDs, anticoagulants, and lifestyle advice to mitigate flares.

Traction Types

  • Skeletal Traction: Achieved through pins; may lead to infections and muscle wasting.

  • Skin Traction: Applied through weights and requires proper skin care to prevent irritation and complications.

  • Pavlik Harness: Specifically for infants with hip dysplasia; must be monitored for potential complications.

Cast Types and Care

  • Types:

    • Short arm/long arm cast: For arm fractures.

    • Short leg/long leg cast: For lower extremities.

    • Spica casts: For specific surgical following recovery and injury.

  • Care Guidelines: Maintaining integrity, cleanliness, monitoring for complications such as skin breakdown and appropriate immobilization adjustments.

  • Instructions for Caregivers and Patients: Regular monitoring of symptoms, dry maintenance, and precautions for skin integrity and circulation.

Final Reminders and Instructions

  • Consult healthcare providers for any acute concerns (e.g., increased pain, swelling, or lymph node changes) while undergoing treatment for various conditions.