Maternal-Child Final

• Most common permanent physical disability of childhood
• Non-progressive disorder
• Characterized by:Abnormal muscle tone and coordination, and failure to reach motor milestones. May have perpetual problems, language deficits and intellectual involvemnet

• Spastic (most common)
• Dyskinetic/athetoid
• Ataxic
• Mixed

• Most common type
• Decreased tone early, increased tone later
• Delayed gross motor development
• May have normal language and personal-social development

• Reflex abnormalities: persistant primitive infantile reflexes
• Abnormal motor performances: poor suck/swallow, feeding problems, facial grimacing, early hand dominance, abnormal crawling, may stand or walk on toes, hips flexed when prone

Mobilizing devices, surgery, medication (relaxants, botox), technical aids, physical, speech and occupational therapy

• Most are X-linked inheritance
• Affects males, mothers carry it
• Pathophysiology: mutation of the gene that encodes dystrophin

• Normal development until onset at age 3-5. Then they may have motor delay, difficulty running...etc.
• Progressive muscle wasting and weakness
• Calf muscle hypertrophy from fatty infiltration
• Waddling gait and frequent falls
• Lordosis
• Wheelchair bound by 12

• Contractures
• Atrophy of disuse
• Infections
• Obesity
• Scoliosis
• Cardiac failure

• Gowers Sign
• Related to MD
• + Sign: rising using hands walking up thighs

• Improper alignment of femoral head and acetabulum
• Hip instability, dislocation, subluxation, dysplasia
• More common in first-born females

• Newborn has lax joints from maternal relaxin
• Acetabulum is not fully formed
• Pressure from femoral head stimulates bone growth

• Positive Ortolani's and Barlow's maneuver
• Limited abduction of hips
• Affected side leg appears shorter
• Asymmetry of gluteal folds
• Limp/telescoping

• Assess for skin condition
• Provide for safety

• Present at birth
• Unable to passively realign the foot with manipulation
• Involves bone and soft tissue

• Serial casting or surgery
• Cast changes every week or two

Clavicle is the most frequently broken bone in children younger than 10

Fractured bone protrudes through skin

Bone fragments have damaged other organs or tissues

Small fragments of bone are broken from the fractured shaft and lie in surrounding tissue

Compressed side of bone bends, but tension side of bone breaks, causing incomplete fracture

• I & II: Break is not through growth plate
• III & IV: through the growth plate, abnormal/no growth later
• V: Crushing injury and plate is crushed

• Traction
• Casting
• Surgery

• S shaped lateral curve of >10 degrees
• Bracing and Surgery
• No intervention until >20 degrees, then, braces are used

• Leave the bone marrow and then develop into RBC between 24 and 48 hours later
• Immature RBC's and make up 1% of RBC's circulating

• A decrease in either total number of circulating RBCs or a decrease in concentration of Hgb or both
• Usually caused from iron deficiency
• Management:
• Reverse the anemia by treating underlying cause
• Iron supplements
• Increase bioavaliability with Vitamin C

• Gradual, vague, nonspecific onset
• Fatigue, pallor, grayish mucous membranes, irritability, anorexia

• Don't take with milk or tea
• May cause diarrhea or constipation
• Prevent accidental overdose
• Reticulocyte count

• Irritability
• Loss of appetite
• Weight loss
• Sluggishness
• Abdominal pain
• N/V/C
• Pallor

• Affects brain development-irreversible damage possible
• Nervous system damage
• Renal impairment
• Anemia
• Poor muscle coordination
• Behavior problems
• Decreased muscle and bone growth
• Hearing impairment, blindness

• Sickle cell anemia
• Thalassemias

Defect within the cell that shortens the life span of the RBC

• Genetic, autosomal recessive
• Normal is Hgb AA
• Sickle cell trait is Hgb As
• Sickle cell anemia Hgb ss
• Both parents w/trait=?% with each pregnancy
• Appears to be protective against malaria

• Pallor, weakness, fatigue, jaundice
• Spleen: enlarged from congestion and engorgement->splenic infarction-.replaced by fibrous tissue->functional asplenia
• Liver: enlarged->eventually fails from impaired hepatic blood flow and capillary obstruction
• Kidney: capillaries get congested with sickle cells->tissue necrosis->scarring
• Bones: hyperplasia and congestion of bone marrow->osteoporosis, widening of medullary spaces, thinning of cortices

• Vaso-occlusive crisis-distal ischemia from vascular occlusion
• Severe bone pain r/t tissue hypoxia
• Systemic damage
• Fever

• Prevent sickling
• Minimize tissue deoxygenation
• Promote hydration
• Minimize crisis: prevent infection
• Supportive therapies during crisis
• Medication:
• Immunization
• Prophylactic antibiotics

• Bed rest to O2 demand
• Hydration
• Replace electrolytes
• Analgesics
• Antibiotics
• Blood replacements
• Apply heat

• Inherited blood disorder: Hgb is not formed correctly
• Classified according to Hgb chain affected
• alpha or beta
• Results from:
• Defective synthesis of Hgb
• Structurally impaired RBCs
• Shortened life of RBCs
• Chronic Hypoxia:
• HA, irritability, precordial and bone pain, exercise intolerance, anorexia, epistaxsis

• Blood transfusion every 3 weeks
• Complications of blood transfusions->hemosiderosis (iron overload)
• Need desferal-iron chelating agent: gets rid of extra iron in the blood
• Bone marrow transplantation

• Caused by:
• Viral infection
• Mono

• X-linked recessive
• Signs/Symptoms:
• prolonged bleeding, oozing of blood into soft tissue
• Management:
• Replacement of missing factors, transfusion of factor concentrate
• Preserve joints and manage pain
• Prevent bleeding and joint degeneration
• Safety issues: no rough play/contact sports
• Medications:
• Steroids
• Ibuprofen
• Self-infusion

• Infants that acquire disease are asymptomatic until 18-24mos.
• Signs/Symptoms:
• Weight loss, failure to thrive
• Chronic diarrhea
• Developmental delay, loss of milestones
• Repeated respiratory infections
• Fever, night sweats
• Weakness, fatigue, malaise, myalgia
• Opportunistic infections
• Hepatospleenomegaly

• Children's kidneys can't concentrate urine or deal with extra fluid
• Increased risk for injury r/t lack of back fat

Infection of bladder, urethra or kidney

• Vesicoureteral Reflux
• I&II: can outgrow
• III: may or may not outgrow
• IV&V: severe UTI probably need surgery

• Voiding Cystourethrogram
• How far up ureter does urine go
• Surgery may be needed to re-implant ureters higher

• Infant: Non-specific (irritable, fever)
• Child >2 years: fever, anorexia, vomiting, diarrhea, constipation, abd, flank or back pain, foul smelling urine, dysuria, dibbling, enuresis, hematuria

• Cystitis
• Frequency, dysuria, urgency, enuresis, strong-smelling urine, cloudy urine, hematuria, abd pain, fever

• 5-7 days of antibiotics
• Encourage oral fluids
• Analgesic

• Pyelonephritis (ureters and kidneys)
• High fever, chills, abd pain, flank pain, costovertebral angle tenderness, persistant vomiting, moderate to severe dehydration

Rehydration, antipyretics, IV antibiotics initially then orally for 7-10 days

Usually follows strep infection

• Acute inflammation lasts about 2 weeks then a slow return to normal function in
• weeks to a few months – sometimes there is chronic renal failure

• Good health prior to causative infection
• Puffy face w/periorbital edema in AM
• Anorexia
• Oliguria
• Cola cored urine
• Irritability, lethargy, generalized discomfort

• No specific treatment
• Antibiotics if infection is still present
• Bed rest during acute phase
• Complications:
• HTN
• Acute renal failure
• Hypertensive encephalopathy
• Seizures
• Infection/sepsis
• Not likely to reoccur

• Edema
• Massive proteinuria
• Hypoalbunemia
• Hypoproteinemia
• Hyperlipidemia
• Altered immunity