Exam 4 Flashcards
Etiology and Pathophysiology of UTI
Mostly caused by E. coli
Urinary stasis can contribute to development
Stasis may be caused by abnormal anatomical structures or abnormal function (e.g. neurogenic bladder)
Clinical Manifestations of UTI
Many are asymptomatic and discovered accidentally on routine examination
Bladder Exstrophy
Rare congenital defect in which the posterior bladder wall extrudes through the lower abdominal wall
Corrected with surgical reconstruction
Obstructive Uropathy
Structural or functional abnormalities of the urinary system that interfere with urine flow and result in urine backflow into the kidneys
Often causes hydronephrosis
Early diagnosis and treatment prevents kidney damage and deterioration of kidney function
Vesicoureteral Reflux
Retrograde flow of urine from the bladder into the ureters
Creates a reservoir for bacterial growth
Recurrent infection within 1-2 months
Culture and sensitivity every 2-3 months
Prune Belly Syndrome
Congenital defect characterized by failure of the abdominal musculature to develop
Primary Enuresis
Child has never had a dry night; attributed to maturational delay and small functional bladder
Not associated with stress or psychiatric cause
Secondary Enuresis
Child who has been reliably dry for at least 6 months begins bed-wetting
Associated with stress, infections, and sleep disorders
Milestones in the Development of Bladder Control
1.5 years: child passes urine at regular intervals
2 years: child announces when voiding is taking place
2.5 years: child makes known the need to void; can hold urine
3 years: child goes to the bathroom alone; holds urge if preoccupied with play
2.5-3.5 years: child achieves nighttime control
4 years: child shows great interest in going to bathrooms when away from home
5 years: child voids approximately 5-6 times per day; prefers privacy; is able to initiate emptying of bladder at any degree of fullness
Nephrotic Syndrome
Alteration in kidney function secondary to increased glomerular basement membrane permeability to plasma protein
Characterized by edema, massive proteinuria, hypoalbuminemia, hypoproteinemia, hyperlipidemia, and altered immunity
Acute Postinfectious Glomerulonephritis
Most common inflammation of the glomeruli of the kidneys
Many children are asymptomatic
Onset usually abrupt with flank or midabdominal pain, irritability, malaise, and fever
Treatment focuses on relief of symptoms and supportive therapy
Acute Renal Failure
Sudden loss of adequate renal function in which the kidneys are unable to clear metabolic wastes and to regulate extracellular fluid volume, sodium balance, and acid-base homeostasis
Causes of Acute Renal Failure
Hemolytic-uremic syndrome, acute glomerulonephritis, sepsis, poisoning, nephrotoxic medications, hypovolemia, obstructive uropathy, and complication of cardiac surgery
Clinical Manifestations and Treatment of Acute Renal Failure
Dark urine or gross hematuria, headache, edema, fatigue, crackles, gallop heart rhythm, hypertension, hematuria, lethargy, nausea and vomiting, oliguria
Treatment depends on the underlying cause of the renal failure
Chronic Renal Failure
Progressive, irreversible reduction in kidney function
Usually results from developmental abnormalities of the kidney or urinary tract, obstructed urine flow and reflux, hereditary diseases such as polycystic kidney disease, infections such as hemolytic-uremic syndrome, and glomerulonephritis
Clinical Manifestations and Treatment of Chronic Renal Failure
Fatigue, malaise, poor appetite, nausea and vomiting, failure to thrive, short stature, oliguria/polyuria, headache, decreased mental alertness/ability to concentrate, chronic anemia, hypertension, edema, fractures with minimal trauma, rickets, valgus deformity
Treatment is dialysis and eventually a kidney replacement
Hemolytic-Uremic Syndrome
Most common cause of acute renal failure in young children
Triad of signs includes hemolytic anemia, thrombocytopenia, and acute renal failure
Treatment focuses on the complications of ARF and includes fluid restrictions and a high-calorie, high-carbohydrate diet that is low in protein, sodium, potassium, and phosphorus
Polycystic Kidney Disease
Genetic disorder that has autosomal recessive and dominant forms
Liver abnormalities are associated with both forms of the disease
Low-set ears, small jaw, and flattened nose
Treatment is supportive
Cryptorchidism
Undescended testes
Congenital defect of the gonads
Surgery may be necessary for correction
Pelvic Inflammatory Disease
Serious infection of the upper genital tract caused by the ascending spread of organisms in the cervix and vagina
Clinical Manifestations and Treatment of Pelvic Inflammatory Disease
Fever, mild/dull bilateral lower abdominal pain, dysmenorrhea, dysuria, vaginal discharge, prolonged or increased menstrual bleeding
Antibiotics used for treatment
Normal BUN
5-18
Normal CRE
- 2-0.4 in infants
0. 3-0.7 in children
Confusion
Disorientation to time, place, or person; loss of clear thinking. Answers to simple questions may be correct, but responses to complex ones may be inaccurate
Delirium
State characterized by disorientation, fear, irritability or agitation, and mental or motor excitement
Lethargy
Profound slumber in which speech and movement are limited. The child is aroused with moderate stimulation, but falls asleep easily once stimulation is removed
Stupor
Deep sleep or unresponsiveness; the child is aroused only with repeated vigorous stimulation, but returns to the unresponsive state when the stimulus is removed
Coma
Unconsciousness; cannot be aroused even by painful stimuli
Caused by pathologic processes (meningitis, trauma, hypoxia, poisoning, seizures, alcohol/substance abuse, endocrine/metabolic disorders, etc.) that cause increased intracranial pressure
Early Signs of Increased Intracranial Pressure
Headache, visual disturbances, diplopia, nausea/vomiting, dizziness/vertigo, slight change in vital signs, pupils not as equal or reactive, sunsetting eyes, slight change in level of consciousness
Late Signs of Increased Intracranial Pressure
Significant decrease in level of consciousness, seizures, fixed and dilated pupils, papilledema
Cushing Triad (increased systolic blood pressure and widened pulse pressure, bradycardia, irregular respirations)
Focal/Partial Seizures
Caused by abnormal electrical activity in one brain hemisphere or a specific area of the cerebral cortex
Generalized Seizures
Result of diffuse electrical activity that begins in both brain hemispheres simultaneously
Febrile Seizures
Occur in susceptible infants and children in connection with a rise in temperature to 102.2
Bacterial Meningitis
Secondary to other infections such as otitis media, sinusitis, pharyngitis, cellulitis, pneumonia, or septic arthritis; brain trauma; or a neurosurgical procedure
Brain becomes inflamed and edematous, leading to cerebral edema
Clinical Manifestations of Bacterial Meningitis
Febrile, altered consciousness, vomiting, complaints of muscle/joint pain, headache, photophobia, esotropia, nuchal rigidity
Viral Meningitis
Inflammatory response of the meninges characterized by an increased number of blood cells and protein in the CSF
Clinical Manifestations of Viral Meningitis
Abrupt onset of fever, headache, photophobia, stiff neck, back pain, myalgia, irritability, lethargy
Encephalitis
Acute inflammation of the brain often caused by an arbovirus that is transmitted by a mosquito
Clinical Manifestations of Encephalitis
Fever, irritability, severe headache, bulging fontanelle, altered mental status
Flaccid or spastic paralysis
At risk for seizures, respiratory failure, and increased ICP and receives supportive treatment in the ICU
Reye Syndrome
Acute encephalopathy caused by drug toxic to the liver or other toxin, metal, or metabolite and results in a poorly functioning organ
Guillain-Barre Syndrome
Acute inflammatory peripheral neuropathy with an acute onset of rapidly developing symmetric motor weakness that progresses in an ascending pattern
Postinfectious disorder that affects motor, sensory, and autonomic neurons
Clinical Manifestations of Reye Syndrome
Cerebral edema, hypoglycemia, and an enlarged liver
Clinical Manifestations of Guillain-Barre Syndrome
Hypotonia, respiratory distress, irritability, feeding difficulties in infants, pain, numbness, paresthesia
Migraine Headaches
May be triggered by stress; foods containing nitrates, glutamate, caffeine, tyramine, and salt; menses; fatigue; and hunger
Tension Headaches
Associated with stress related to school, anxiety, demanding schedules, fasting, and inadequate sleep
Medication Overuse/Rebound Headaches
Associated with the frequent use of medications for headaches
Microcephaly
Small brain with a head circumference below the third percentile on growth curves
May be caused by genetic disorder or destructive insult during infancy such as an infection, metabolic disorder, or hypoxia-ischemia
Intellectual disability is common
Hydrocephalus
Body’s response to an imbalance between the volume of CSF produced and absorbed
May be congenital or acquired as a result of intraventricular hemorrhage, meningitis, traumatic brain injury, or brain tumor
Clinical Manifestations of Hydrocephalus
Increased head circumference, irritability, vomiting, poor appetite, disordered sleep, fever
Anencephaly
No development of the brain above the brainstem, which is ultimately fatal
Encephalocoele
Protrusion of meningeal tissue or meninges-covered brain through a defect in the skull
Spina Bifida Occulta
A vertebral defect in which the posterior vertebral arches fail to fuse, but the spinal cord and meninges are contained in the vertebral canal
Spina Bifida Cystica
A posterior vertebral arch defect with protrusion of meninges through the bony spine
Meningocoele
Protrusion of a meningeal sac filled with CSF through a vertebral defect, associated with no abnormalities of the spinal cord
Myelodysplasia
Protrusion of a meningeal sac that contains CSF, a portion of the spinal cord, and nerves through a vertebral defect
Etiology of Myelodysplasia/Spina Bifida
Chemicals, medications, genetic factors, and maternal health conditions
Clinical Manifestations of Myelodysplasia
Thoracic Level: paralysis of the legs, weakness and sensory loss in the trunk and lower body region
Lumbar 1-2 Level: some hip flexion and adduction, cannot extend knees
Lumbar 3 Level: can flex hips and extend the knees; paralyzed ankles and toes
Lumbar 4-5 Level: can flex hips and extend the knees; weak or absent ankle extension, toe flexion, and hip extension
Sacral Level: mild weakness in the ankles and toes
Bowel and bladder incontinence occurs with all except sacral
Mobility problems, intellectual disability, and visual impairment
Craniosynostosis
Premature closure of cranial sutures in utero or during the first 18 months of life
Positional Plagiocephaly
An asymmetric flattening of the skull, associated with sleep position to prevent SIDS
Neonatal Abstinence Syndrome
Caused by opioids, CNS stimulants, CNS depressants, antidepressants, and benzodiazepines
Initial signs include irritability, jitteriness, tremors, excessive crying
Other signs include yawning, diarrhea, vomiting, seizures, inconsolable high-pitched crying, difficulty sleeping, hypertonia, and ANS signs
Neurofibromatosis
Autosomal dominant genetic disorder in which tumors grow along nerves
Characterized by six or more cafe au lait spots
Pain, vision deficits, precocious puberty, delayed puberty, thinning/bowing of the tibia, fractures that do not heal properly, and scoliosis are all possible signs
Cerebral Palsy
Common syndrome of movement and posture development disorders caused by a nonprogressive lesion abnormality in the fetal or infant brain that results in activity limitations
Associated hearing, vision, communication, perceptual, cognitive, and behavioral problems
Etiology of Cerebral Palsy
Risk factors include low birth weight, placental abnormalities, birth defects, meconium aspiration, birth asphyxia, neonatal seizures, respiratory distress syndrome, hypoglycemia and neonatal infections
Clinical Manifestations of Cerebral Palsy
Diplegia: both legs are affected
Hemiplegia: only one side of the body is involved, the arm is usually more severely affected than the leg
Quadriplegia: all four extremities are affected
Motor impairment, spasticity, delayed developmental milestones
Metatarsus Adductus
Most common congenital foot deformity characterized by an inward turning of the forefoot at the tarsometatarsal joints
Treatment depends on the degree of foot flexibility
Braces, casting, orthopedic shoes
Clubfoot
Congenital abnormality in which the foot is twisted out of its normal position
Exact cause is unknown, however some believe neuromuscular or vascular problems
Midfoot is directed downward (equinus), the hindfoot turns inward (varus) and the forefoot curls toward the heel (adduction)
Foot is small with a shortened Achilles tendon; muscles in the lower leg are atrophied, but leg lengths are generally normal
Early treatment is essential
Genu Varum
Bowlegs; deformity in which the knees are widely separated while the ankles are close together and the lower legs are turned inward (varus)
Braces, osteotomies, casting
Developmental Dysplasia of the Hip
Refers to a variety of conditions in which the femoral head and the acetabulum are improperly aligned
Conditions include dislocation, hip instability, subluxation, and acetabular dysplasia
Genetic factors appear to play a role
Common signs and symptoms include limited abduction of the affected hip, asymmetry of the gluteal and thigh skinfolds, and telescoping or pistoning of the thigh
60-80% of hip abnormalities resolve by 2 months of age
Pavlik harness, casting
Legg-Calve-Perthes Disease
Self-limiting condition in which there is avascular necrosis of the femoral head
Results from an interruption of the blood supply to the femoral epiphysis
Early symptoms include a mild pain in the hip or anterior thigh and a limp
Later symptoms include limited range of motion and weakness and muscle wasting
Surgery, braces
Slipped Capital Femoral Epiphysis
Occurs when the femoral head is displaced from the femoral neck
Seen often in adolescents following a growth spurt
Predisposing factors include obesity, a recent growth spurt, and endocrine disorders
Symptoms include a limp knee; thigh, hip, or groin pain; and loss of hip motion
Scoliosis
Lateral S- or C-shaped curvature of the spine that is often associated with a rotational deformity of the spine and ribs
May be congenital, idiopathic, or acquired
Classic signs include truncal asymmetry, uneven shoulder and hip height, a one-sided rib hump, and a prominent scapula
Does not usually cause pain or discomfort
Torticollis
Tilt of the head caused by rotation of the cervical spine
Generally an injury sustained to the sternocleidomastoid muscle at the time of birth or to a cervical spine abnormality
Stretching exercises or surgical lengthening of the sternocleidomastoid are usual treatments
Osteoporosis
A condition in which there is decreased density and mass of bone, promotes the risk of fractures and is commonly associated with aging
Preceded by osteopenia or low bone mass, which is between 1.0 and 2.5 standard deviations below the norm
Osteomyelitis
Infection of the bone, most often of the long bones of the lower extremity
May be acute or chronic and may spread into surrounding tissues
Caused by a microorganism, often from URIs, trauma to bone, and surgery
Symptoms include pain and tenderness with swelling, decreased mobility of the affected joint, and fever
Skeletal Tuberculosis
A rare microbacterial infection that can be very destructive; spine is the most frequent site of infection
Clinical Manifestations: depending on the site, pain, limp, severe muscle spasms, kyphosis, muscle atrophy, “doughy” swelling of the joints, decreased joint motion, changes in reflexes, low-grade fever
Diagnostic Tests: TB skin test, CBC, synovial fluid analysis, radiographs
Clinical Therapy: antibiotic therapy
Septic Arthritis
A joint infection of the synovial space; most common site is the knee, followed by the hip, ankle, and elbow
Clinical Manifestations: fever, pain, local inflammation, joint tenderness, swelling, loss of spontaneous movement
Diagnostic Tests: CBC with differential, ESR, blood cultures
Clinical Therapy: joint aspiration, open drainage and irrigation, followed by antibiotic therapy
Achondroplasia
Dwarfism is a genetic condition usually resulting in an adult height of 58 inches or less
Marfan Syndrome
Most common problems are mitral valve prolapse, aortic regurgitation, abnormal aortic root dimensions, pectus excavatum, long arms and digits, scoliosis, elongated head, high arched palate, lens subluxation, and pneumothorax
No treatment for this syndrome
Osteogenesis Imperfecta
Brittle bone disease; connective tissue disorder that primarily affects the bones
Biochemical defect in the production of collagen
Clinical manifestations include multiple and frequent fractures, blue sclerae, thin soft skin, altered joint flexibility, short stature, enlargement of the anterior fontanelle, weak muscles, and brittle bones
Pseudohypertrophy
Enlargement of the muscles as a result of their infiltration with fatty tissue
Duchenne Muscular Dystrophy
X-linked recessive disorder seen in boys
Clinical Manifestations: delayed walking, frequent falls, easily tired when moving, toe walking, hypertrophied calves, waddling gait, lordosis, positive Gowers maneuver, intellectual disability frequently seen
Clinical Therapy: supportive care (physical therapy and braces)
Becker Muscular Dystrophy
X-linked recessive disorder
Clinical Manifestations: similar to Duchenne but more milder and delayed
Supportive care
Facioscapulohumeral Muscular Dystrophy
Autosomal dominant disorder
Clinical Manifestations: face, shoulder girdle, lower limbs affected, unable to raise arms over head, lordosis, cannot close eyes or whistle or smile, facial weakness
Physical therapy
Emery-Dreifuss Muscular Dystrophy
X-linked recessive disorder
Clinical Manifestations: early onset of contractures followed by weakness; achilles tendon elbow and spine affected, cardiac conduction defect may occur
Physical therapy, surgery, pacemaker insertion
Congenital Muscular Dystrophies
Autosomal recessive group of disorders
Clinical Manifestations: muscle weaknesses present at birth; motor development delay; contractures and joint deformities; hypotonia
Correction of skeletal deformity (orthosis or surgery), usually nonprogressive
Fractures
A break in the bone that occurs when more stress is placed on a bone than a bone can withstand
Reduction and immobilization indicated
Strain
Stretching or tearing of either a muscle or a tendon, usually from overuse
Sprain
Stretching or tearing of a ligament, usually caused by falls, sports injuries, or motor vehicle crashes
Skin Traction
Pull is applied to the skin surface, which puts traction directly on the bones and muscles
Dunlop Traction (Skeletal or Skin)
Used for fracture of the humerus
Arm is suspended horizontally with straps placed on both the upper and lower portions for pull from both sides
Bryant Traction
Used specifically for the child under 3 years of age who has developmental dysplasia of the hip or a fractured femur
Buck Traction
Used for knee immobilization, to correct contractures or deformities, or for short-term immobilization of a fracture
90-90 Traction
Used for fractures of the femur or tibia
Skeletal Traction
Pull is applied directly to the bone by pins, wires, tongs, or other apparatus that have been placed surgically
Skeletal Cervical Traction
Used for cervical spine injuries to reduce fractures and dislocations
Halo Traction
Used to immobilize the head and neck after cervical injury or dislocation
Russell Traction
Used for fractures of the femur and lower leg
External Fixators
Attached to the extremity by percutaneous transfixing of pins or wires to the bone
Spontaneous Reflexes
Pupil dilation, swallowing, gag reflexes, withdrawing from pain, tonic neck reflex, stepping reflex, sucking reflex
Elicited Reflexes
DTRs (want them to be 2+), Babinski, rooting reflex, Moro reflex
Decorticate Posturing
Flexion
Decerebrate Posturing
Extended and outward
Indicative of worsening neurological status
Eye Assessment
Fixed and dilated pupils are emergent
Early Signs of Increased Intracranial Pressure
Challenging to catch early due to vague symptoms
Irritability, increased head circumference in infants, not feeling normal, decreased appetite, fatigue, mild confusion, nausea
Middle Signs of Increased Intracranial Pressure
Slower to arouse, changing pupil size/reaction time, nausea and vomiting, generalized weakness, seizures, more severe headache, tense or bulging fontanelles, suture separation
Later Signs of Increased Intracranial Pressure
Decreasing LOC, decreased motor response on command, decreased sensory response due to pain, pupil size and reactivity worsens, posturing, Cheyne-Stokes respirations, setting-sun eyes, bulging fontanelles
Full LOC
Responsive, alert and oriented x3, demonstrating perfectly developmentally appropriate behavior
Confusion LOC
Impaired decision-making
Disorientation LOC
Disorientation to time/place
Lethargy LOC
Drowsy, limited spontaneous movement
Obtunded LOC
Aroused only with stimulation
Stupor LOC
Responds only to vigorous and repeated stimulation
Coma
Unable to arouse
Persistent Vegetative State
No brain activity, only reflexive movement
Glasgow Coma Scale
15 points total 13-14 is mild impairment 9-12 is moderate impairment Less than or equal to 8 is comatose Less than 3 is persistent vegetative state
Lab Tests for Neurological Injury
Glucose, CBC, electrolytes, blood culture, evaluate for toxic substances, liver function tests
Imaging for Neurological Injury
CT, MRI, PET scan, echoencephalography, ultrasound, nuclear brain scans
Lumbar Puncture
Used when meningitis/encephalitis is suspected
EEG
Used to diagnose seizure activity, LOC
ABCs for Brain Injury
Need to guard against cerebral hypoxia (permanent damage after 4 minutes)
Risk of aspiration
Minimize invasiveness, maximize oxygenation
Safety Considerations with Brain Injury
Positioning: avoid venous neck compression, keep head supported, turn from side to side
Avoid activities that increase intracranial pressure (Valsalva, crying/tantrums, stress)
Pain Considerations with Brain Injury
Look for behavior changes
Analgesics, opioids, anti-seizure medications, sedatives
Rest is necessary for the brain to heal
Concussion
Transient and reversible by definitiion
Instantaneous loss of awareness, often amnesia or confusion
May or may not lose consciousness
Standard is asymptomatic for 24 hours
Post-Concussion Syndrome
Dementia, seizure disorders, chronic headaches, behavior changes
Pathophysiology of Spina Bifida
Vertebral defect that allows the spinal cord to protrude
Can occur anywhere along the spinal cord–vast majority are lumbar or sacral
Risk Factors for Spina Bifida
Chemical exposures in utero, medications (anti-epileptic), diet (lack of folic acid), genetics, gestational diabetes, maternal obesity
Clinical Presentation of Spina Bifida
Paralysis, weakness, sensory loss, sac-like protrusion, bowel and bladder incontinence, neurogenic bladder, hydrocephaly if above the sacral region, intellectual deficit, visual impairment, spinal curvature, musculoskeletal/joint pain, skin sores, pressure ulcers, precocious puberty, sexual dysfunction
Diagnosis of Spina Bifida
Early fetal ultrasound
Maternal alpha-fetoprotein
CT, MRI, X-ray
Medical Management of Spina Bifida
Fetal corrective surgery if identified before 26 weeks gestation
Permanent catheter required with bladder incontinence
Laxatives used with chronic constipation
Braces and assistive devices to match degree of function patient needs
Diet includes lots of calcium and vitamin D, weight-bearing exercise is necessary
Spina Bifida Initial Nursing Interventions
Cover the protrusion with moist sterile dressing
Prone position with legs flexed and abducted
Assess for other congenital deformities
Prevent sac from leaking
Give small bottle feedings in the prone position
Surgical preparation
Support parent-child bonding
Spina Bifida Post-Operative Nursing Interventions
Prone or side-lying position for healing
Assess site routinely for signs/symptoms of infection and/or CSF leakage
Pain management
Intake and output
Daily head circumference
Bowel/bladder assessment and motor deficit assessment
Gentle ROM
Parental support
Hemiplegia
Arm and leg on one side
Arm bent; hand spastic or floppy, often of little use
Walks on tiptoe or outside
of foot on affected side
Unaffected side is completely or almost normal
Paraplegia
Both legs only
Upper body usually normal or with very minor signs
Child may develop contractures of the ankles and feet
Quadriplegia
Both arms and both legs
When walking, head arms, and mouth may twist strangely
Often have severe brain damage and never are able to walk
Knees pressed together
Legs and feet are turned inwards
Pathophysiology of Cerebral Palsy
Underlying cause is a nonprogressive brain lesion
Clinical Presentation of Cerebral Palsy
Activity limitations, abnormal muscle tone and lack of coordination, hemiplegia, diplegia, quadriplegia, some degree of motor impairment and muscle spasticity, muscle weakness, delayed development, delayed speech and language, seizures, feeding difficulties related to decrease in oral muscle tone
Diagnosis of Cerebral Palsy
Assessment and symptoms
CT, MRI
Genetic/metabolic testing
Functional Mobility Scale in newborns and infants
Medical Management of Cerebral Palsy
Aimed at developing maximal independence and ability to perform ADLs
Braces, splints, serial casting, positional devices, therapeutic medications
Special Education services
Orthopedic surgery–tendon lengthening, muscle release
Cerebral Palsy Mobility Nursing Implications
Anything to help maintain proper body alignment
Support their head, especially when younger
Range of motion to prevent contractures
Massage, therapeutic touch, riding therapy
Adaptive device referrals, parent teaching
Cerebral Palsy Nutrition Nursing Implications
Supplement for a higher calorie diet
Avoid aspiration
Adaptive utensils
G-tube
Bowel program may be indicated
Congenital Muscular Dystrophy
Present at birth, usually nonprogressive, nonterminal, more rare
Duchenne Muscular Dystrophy
Onset is around 3-4 years of age, life expectancy is somewhere around adolescence, most common
Wheelchair users by 12 years of age
Cardiovascular/respiratory failure is typically cause of death
Pseudohypertrophic
Responsible gene is absent or deleted
Becker Muscular Dystrophy
Appears after age 5, life span is around 30-40 years of age
Pathophysiology of Muscular Dystrophy
Inherited disorder
Muscle fiber degeneration and muscle wasting
Onset can be early or later in life
Clinical Manifestations of Muscular Dystrophy
Generalized muscle weakness
Gower’s Sign: using hands to push on legs to stand
Diagnosis of Muscular Dystrophy
Confirmed by muscle biopsy
Initially by clinical signs and pattern of muscle
Medical Management of Muscular Dystrophy
No effective treatment
Steroids can preserve muscle treatment and walking for a longer amount of time
Rehabilitation/physical therapy
Mental/emotional support
Dietary enhancement
Massage
Aggressive treatment of respiratory infections
Growth Hormone Deficiency
Inhibits somatic growth throughout the body
Proportional decrease in growth, no dwarfism
Growth hormone injections given until growth plates close
Primary Pituitary Hyperfunction
Increased production of growth hormones before the growth plates close
Delayed closure of fontanelles, increasing head circumference are early indicators
Vertical growth followed by rapid increase in muscle development
Weight proportional to height
Acromegaly
Occurs after growth plate closure
No increase in vertical growth; increased size of head, nose, tongue, jaw, lips, malocclusion of teeth, thick creased skin
Treatment of Acromegaly
Hormone replacement therapy
Tumor removal/radiation
Precocious Puberty
Sexual development before the age of 9 in boys and before the age of 7 in Caucasian girls and 6 in African American girls
Treated by Leuprolide until normal onset of puberty
Diabetes Insipidus
Undersecretion of ADH leads to uncontrolled diuresis, polyuria, polydipsia
Biggest risk is dehydration
Treatment is Vasopressin
SIADH
Hypersecretion of ADH leads to inability to void or lacking the urge to void
Concern is fluid overload
Fluid retention, hypotonicity of muscles, anorexia, nausea, vomiting, irritability, behavioral changes
ADH antagonists used to treat
Hyperparathyroidism
Hypercalcemia occurs
Rare in children
Cushing’s Syndrome
Excessive circulating free cortisol
Side effect of prolonged steroid treatment
Moon face, red cheeks, weight gain, pendulous abdomen, red striae, ecchymosis, hyperglycemia
Diagnostics for Type I Diabetes
Fasting blood glucose under 126 is normal
HbA1C
Urine ketone testing
REEDAR
Redness Edema Ecchymosis Drainage Approximation Raised
Acne
Multifactorial (hygiene, genetics)
Use a mild moisturizing soap, products with salicylic acid or benzoil peroxide
Penicillin Allergy
Extremely urticaric
Appears on day 3-5 of treatment
Stop the medication
Eczema
Atopic dermatitis
Inflamed, irritated, overly dry skin
Treatment is hydration and moisturize with lipid-soluble creams and lotions
Lichenification common
Candida Albicans
Yeast infection
Centralized area of erythema surrounded by satellite lesions
Antifungal medications required (nystatin, diflucan)
Fifths Disease
“Slapped cheek” appearance
Caused by human parvovirus B19
Facial erythema with maculopapular rash that moves proximodistal
Contagious–emergent in kids with sickle cell disease
Symptomatic support
Hand Foot and Mouth Disease
Coxsackie virus
Presents as 5-10 mucocutaneous lesions that are very painful and hundreds of cutaneous lesions that are painless
Warts
Human papillomavirus is responsible
Treat with chemicals, laser treatments, cryotherapy, surgery
Prevention using HPV vaccine
Moloscum Contagiosum
Viral infection
Flesh-colored papules with an umbilicated center
Passed skin-to-skin
Self-resolving
Contact Dermatitis
Not an allergic reaction, just an inflammatory process
Need to wash poison off as soon as possible
Only be concerned with there is a secondary complication such as periorbital edema
Scarlet Fever Rash
Strep rash
Will resolve as strep throat resolves
Tinea
Fungal infection that is contagious
Treatment is over the counter azole medications
Need to treat several times a day for at least 2-3 weeks
Herpes Simplex Virus
Vesicles that form on the mucocutaneous borders
Tingling on the skin before lesion appears
Contagious when there are active lesions
