PNP Management 3: Exam #3

-A condition that results from excessive excretion of protein in urine due to increased glomerular filtration permeability.
-It is massive proteinuria (3-4+) or protein-creatine ratio greater than 2-3:1.

The loss may be selective (albumin only) or nonselective (most serum proteins).

-Subsequent edema is a result of decreased plasma oncotic pressure. Because of protein loss, the liver increases protein synthesis, causing concurrent hyperlipidemia and lipiduria.
-Additionally, reduced intravascular volume stimulates antidiuretic hormone, resulting in further increased water reabsorption.

It is defined as the normalization of urinary protein excretion and serum albumin.

-Relapse is defined as the recurrence of proteinuria and hypoalbuminemia after complete remission.

-Genetic
-Immune
-Systemic
-Nephrotoxic
-Allergic
-Infectious malignant
-Vascular
-Idiopathic processes.
-The primary mechanism is thought to be immunologic.

-History of allergy
-Edema is cardinal feature, especially periorbital
-Low urine production
-GI symptoms including anorexia, paleness, listlessness, diarrhea, vomiting, and abdominal pain (RUQ)
-Respiratory difficulties (secondary to edema)

-Edema in tissues of low resistance and dependent areas, periorbital, scrotal, and labial
-Anorexia, irritability, and fatigue
-Muscle wasting, malnourishment, growth failure in prolonged disease
-Hypertension, however, may be normotensive in hypovolemic
-Chronically ill-appearing

-UA and microscopic (protein of 2+ or greater)
-Quantitative urine protein (24 hour urine collection)
-CBC, electrolyte, BUN, creatine, calcium, albumin, total protein, LFT, lipids, C3/C4, ANA

-Karotype if neonatal infant because of association with Dennys-Drash syndrome
-Possible referral for kidney biopsy.

-Primary goal is to control edema.
-Consultation with nephrologist
-Routine immunization during immunosuppression.
-Prednisone may be used to induce remission.
-Diuretics and albumin replacement may be used in acute phases of illness.
-Hospitalization for severe disease.

-Home BP monitoring may be recommended.
-Daily home proteinuria testing may be recommended to monitor the child and promptly identify exacerbations.

-Prednisone may be used to induce remission.
-Noncorticosteroid medication prescribed by nephrologist if steroid resistant/dependent.
-Diuretics and albumin replacement may be used in acute phases of illness.

-Persistent proteinuria greater than 2+ everyday for 3 days.

Prednisone (2mg/kg/day; maximum 60mg) to induce remission.

Remission can occur as early as 14 days as evidenced by diuresis.

Steroids can be continued for at least 4 to 6 weeks.

Relapses are treated with a short course of steroids.

The patient is weaned off steroids as soon as the proteinuria resolves.

Cyclophosphamide and cyclosporine.

-Steroid responsiveness
-Ninety-five percent of children are "steroid responders."
-The remaining 5% are steroid-resistant and demonstrate no response to steroid treatment
-There are still some children who are considered partial responders to steroid treatment, requiring high steroid doses with frequent relapses.

-Susceptibility to myoglobinuria
-Infections
-Hyper-or- Hypotension
-Thromboembolism

-No activity limitations
-Salt may be limited by the nephrologist during active disease, but otherwise an age-appropriate diet may be recommended.
-Important of regular care to monitor renal function and early treatment of the disease.

-Noninfectious, inflammatory response of the kidneys characterized by variable degrees of hypotension, edema, proteinuria, and hematuria that is either microscopic or macroscopic with dysmorphic RBCs and casts.
-Nephritis has an acute, intermittent, and chronic phase.

-Acute nephritis most commonly occurs as glomerulonephritis (GN), which is defined as inflammation in the glomeruli.
-Most commonly occurs as PSGN.

-Primary Glomerulonephritis
-Secondary Glomerulonephritis
-Poststreptococcal Glomerulonephritis (PSGN)

Refers to inflammation primarily in the glomeruli

Refers to inflammation in the interstitium primarily caused by drug reactions.

Occurs when there is glomerular impairment.

Occurs secondarily as a result of systemic disease.

-Is the MOST classic form.
-Characterized by history of streptococcal infection within the prior 2 weeks and an acute onset of edema, oliguria, hypertension, and gross hematuria.
-Usually does not present in children LESS than 3 years old.

Poststreptococcal Glomerulonephritis (PSGN)

Is inflammation of renal parenchyma.

-Intermittent gross hematuria/proteinuria syndromes include IgA nephropathy/Berger disease.
-Nephritis is also associated with Alport syndrome among other disorders.
-Severe forms of this type of nephritis can lead to X-linked disease often presenting as hypotension.

-More common in males than females
-Often precipitated by viral infections or strenuous exercise, and each episode lasts less than 72 hours.
-BP is normal, no edema is present and C3 is normal.

Vision and hearing screening are essential and PCPs should refer if abnormalities noted.

-Chronic nephritis is referred to as membranoproliferative GN.
-Chronic nephritis occurs after nephritis or in individuals with nonspecific complaints, such as anorexia, intermittent vomiting, and malaise.

-Diminished renal function that has detrimental effects on other organ systems.
-Types Ⅰ and Ⅱ may respond to steroids, but the overall prognosis is guarded.

-Can be caused by bacterial, fungal, viral, parasitic or rickettsial agents.

-Streptococcal skin (more likely) or pharyngeal infection within the past 2 to 3 weeks (PSGN) A latent period of 7 to 10 days elapses between infection and the onset of symptoms; if fever than 5 days or more than 14 days, consider other causes.
-Abrupt onset of gross hematuria

-Reduced urine output (with diuresis in 5 to days)
-Lethargy, anorexia, nausea, vomitting, abdominal pain.
-Chills, fever, backache (pyelonephritis)
-Medication taken in the past few weeks.

-Hypertension that is transient and resolves in 1 to 2 weeks.
-Edema, especially periorbital edema, or abrupt onset with weight gain.
-Circulatory congestion—dyspnea, cough, pallor, pulmonary edema if severe
-Ear malformations
-Flank or abdominal pain or a mass (in polycystic kidney or malignancy [e.g., Wilms tumor])

-Costovertebral angle tenderness (in pyelonephritis)
-Rashes or arthralgias (with SLE, HSP, or impetigo)
-Evidence of trauma or abuse

-UA with microscopic-urine may be tea colored
-Serum C3 or C4; total protein, albumin
-CBC, ESR, ASO, streptozyme test, DNA titer
-Electrolytes, BUN, creatinine, cholesterol.

-It is supportive because resolution occurs spontaneously.
-During the peak of oliguria and hypertension, in the first few days of illness, hospitalization may be required with fluid and sodium limitation and diuretic, antihypertensive, and antibiotic treatment if cultures are positive.
-Resolution occurs once diuresis begins.

-Gross hematuria persists for 1 to 2 weeks, urine can be abnormal for 6 to 12 weeks, and microscopic hematuria can persist for up to 2 years.
-Complement levels return to normal in 6 to 8 weeks.

-Consultation with nephrologist for all cases.
-Management of IgA nephropathy includes annual follow up of BP/UA.
-Management of acute nephritis may include hospitalization
-Management of benign familial or hereditary nephritis includes audiometry and review of family medical history.

-Management of benign recurrent nephritis should include close monitoring of the UA and renal function every 1-2 years.
-Chronic nephritis management utilizes a team approach.

-Prolonged oliguria and renal failure can occur if acute nephritis progresses.
-Hypertensive encephalopathy or congestive heart failure can occur secondary to PSGN.
-Irreversible parenchymal damage causes hypertension and renal insufficiency.

-PSGN they have macrohematuria or microhematuria for up to 6 to 12 months, but the long-range outcome is excellent.
-Thin basement membrane disease has a good outcome.
-IgA nephropathy with severe histologic findings has a poor outcome, especially if the child is African American.
-Patient education should stress the importance of continued, regular care to monitor renal function..

-Is a dysfunction of renal tubule transport capability, resulting in low serum carbon dioxide
-It is caused by a defect in the ability of the distal tubule to excrete hydrogen.
-Isolated, idiopathic, and primary problem.
-It is usually found in children evaluated for growth failure and when illness, dehydration, or starvation stresses a child. RTA
-More common in males than females.

-Also called classic or distal RTA (dRTA) is a result of distal tube defects.
-Type Ⅰ RTA causes hypokalemia, hyperchloremia, CO2 < 16, and urine pH > 5.5.

-Also called bicarbonate wasting RTA, resulting from defects in the proximal RTA (pRTA).
-It is the most common form of RTA. Type Ⅱ RTA causes hypokalemia, hyperchloremia, CO2 < 16, and urine pH < 5.5.

Type Ⅲ RTA is a subtype of type Ⅰ and is primarily found in premature infants.

Type Ⅳ RTA is also called hyperkalemic RTA and occurs when there are problems in the functioning of aldosterone found following the relief of obstructive uropathy.

Often found in the work-up for children with growth failure.

-Failure to gain weight (especially) and height-the most common symptoms
-Polyuria and polydipsia
-Muscle weakness (caused by hypokalemia)
-Irritability before eating, satiation after eating, vomiting, diarrhea, or constipation in dRTA
-Preference for liquids over solid foods, poor appetite, or anorexia, especially with type IV.

-Arrested growth curve toward the end of the first year with prior consistent growth
-Normal physical examination and development.

-Serum electrolytes, including carbon dioxide (CO2)
-Hypokalemia, hyperchloremic metabolic acidosis
-Renal function tests (BUN, creatine), calcium, phosphorus, alkaline phosphatase, and UA (first morning void) for glucose and pH.
-If initial lab work is abnormal, work up should:
also include a 24-hour
creatinine clearance
renal US

-Goals include:
correction of acidosis
maintenance of normal bicarbonate levels (greater than 20meq/L)
-Oral alkalizing medications to help achieve the goals of treatment.
-Maximizing caloric intake to promote growth.

-Common agents include Bicitra (sodium citrate and citric acid), Polycitra (sodium, potassium citrate, and citric acid), sodium bicarbonate, and baking soda.
-The dose must be titrated to the child's response as determined by weight and laboratory results (CO2 and electrolytes).
-Initiate medication at 3 mEq/kg/day, and check laboratory results in a few days. Titrate the dose until a serum bicarbonate level of 20 to 22 mEq/L is achieved
-Doses should be given frequently throughout the day (with meals) and as late as possible at night (at bedtime)..

Yes

-Frequent weight checks and lab monitoring biweekly to monthly until weight is stabilized and CO2 is stabilized.
-Weight should be measured on the same scale.
-Pseudoephedrine should be AVOIDED because it is minimally excreted in alkalinized urine and may cause toxicity.
-Referral to a pediatric nephrologist should be made if patient continues not growing well despite treatment

-Regular care to monitor renal function and growth
-Isolated pRTA responds quickly to treatment, with children "catching-up" growth and reaching normal maximum height.
-pRTA resolves spontaneously without symptom recurrence, often within 1 to 2 years, but may take up to a decade
-dRTA usually lasts a lifetime; type IV resolves with underlying problem correction.

-Take time to have a one-on-one private interview with the adolescent, even if the adolescent presents with the parent or partner
-Directly address confidentiality with the adolescent
-Know your state laws and policies as they relate to contraception
-Identify local resources for reproductive health care for adolescents

-Ensure education about LARC, including how and where to get it
-Address common misconceptions about contraception
-Ask about the opinions of friends and family
-Educate on noncontraceptive methods
-Discuss STI prevention, including using a contraceptive method along with a condom to prevent STI transmission
-Invest in the relationship with the adolescent through phone calls and follow-up visits

-Use quick-start contraceptive methods when possible
-Pelvic exams and testing are not required prior to starting contraception
-Multiple visits before and after starting contraception are not needed

-Estrogen:
suppresses FSH release
stabilizes the endometrium
enhances progestin effects
-Progestin:
suppresses LH surge
thickens cervical mucus
thins the endometrium

-Can be initiated when the provider is certain that the adolescent is not pregnant
-If the adolescent has engaged in unprotected sexual intercourse in the previous three weeks but has a negative pregnancy test currently, all methods of contraception except IUDs can be quick started

-Has never had sexual intercourse before
-Is currently using another effective method of contraception
-Has regular monthly periods and is less than 7 days from the start of a normal period
-Is less than 4 weeks postpartum or less than 7 days after spontaneous or induced abortion
-Has not had unprotected sexual intercourse in more than 3 weeks and has a negative pregnancy test today

-Implant
-Intrauterine Device (IUD)
-Depot Medroxyprogesterone Acetate (DMPA) injection
-Combined Hormonal Contraception (CHC) in the form of pills, patches, and rings

-Placed in the subdermal space of the upper inner nondominant arm
-Ethylene vinyl acetate copolymer (latex-free)
-Allows for controlled release of estrogen for 3 years
-Avoid sexual intercourse or use an alternative method for the first week

-Bleeding pattern changes in the first 3 months
-Reassurance from the PCP is crucial for irregular or prolonged bleeding
-NSAIDs for 5 to 7 days or combined an OCP or estrogen for 10 to 20 days for prolonged bleeding

-Copper IUD:
ParaGuard
-Hormone Levonorgestrel IUD:
Liletta
Mirena
Kyleena
Skyla

-Higher satisfaction with Levonorgestrel over Copper IUD
-Screening for chlamydia and gonorrhea annually for all sexually active teens (IUD can be placed still)
-Can be inserted at anytime during cycle

-Bleeding and cramping for the first days and weeks
-NSAIDs to decrease pain and bleeding
-Heavy bleeding, severe pain, nausea, vomiting or fevers should be evaluated
-Many women experience amenorrhea and it is safe

-No evidence-based recommendation to have patients check their string
-PCPs should be able to palpate and visualize IUD strings on pelvic exam
-If strings are not visible, a pregnancy test should be done followed by a pelvic ultrasound to confirm location
-Use other contraceptive methods until IUD is confirmed

-104-150 mg IM injection every 3 months
-DMPA or Depo-Provera
-Can be used as a quick start medication
-Safe in women with medical contraindications to estrogen

-Patients who are at risk for pregnancy due to recent unprotected sex should return 2-4 weeks later for a repeat pregnancy test
-Repeat injection should be given at 11-13 week intervals and can be given early if needed and up to 2 weeks late
-If a woman is more than 2 weeks late for injection

-Irregular menstrual bleeding in the first 6 months
-Begin 5-7 day treatment with NSAIDs or a combined hormonal contraceptive
-Weight gain (5lbs in the first year)
-Bone density loss

-Combination of both estrogen and progestin
-Includes: OCPs, contraceptive patches and rings

-Transdermal contraceptive patch
-Vaginal contraceptive ring

-Xulane
-Adhesive patch
-Contains progestin norelgestromin and ethyl estradiol
-Changes weekly

-NuvaRing
-Releases progestins etonogestrel and ethinyl estradiol
-Ring is inserted into the vagina and must be replaced monthly

-Take pill as soon as possible
-If single pill is missed but is taken within 24 hours of its usually scheduled time, contraceptive efficacy should not be affected
-If a woman misses more than 1 pill in any cycle she should use another form of contraception until they have ad at least 7 days of pills

-Common in the first 3 months
-Confirm patient is taking pills as directed
-Users of extended or continuous CHC should expect bleeding
-Users can either continue on the method, take a 5 day break to allow for a withdrawal bleed and enable to endometrium to stabilize or try a 5 day course of NSAIDs

-Some may experience amenorrhea during the hormone-free interval
-Reasonable to obtain a pregnancy test
-No treatment is needed if not pregnant
-Can change to another brand if user wants a monthly cycle

-Minimal side effects
-Breast pain and nausea in the first few weeks of use
-Can switch or try a lower dose if needed
-DVT are rare

-"Minipill" containing 0.35mg of norethindrone acetate
-Work by blocking sperm from entering the uterus by thickening the cervical mucus
-Patients should be educated to take them at the same time every day, and there should be no placebo breaks
-High failure rates when used alone

-Method of birth control that can be used after unprotected sex or when there are concerns that the contraceptive method failed
-The mechanism of action for emergency contraception is to delay or inhibit ovulation
-There are three emergency contraceptive pills available --Adolescents should be counseled that there is better efficacy the sooner it is used after intercourse
-They should be advised to take the medication right away; they do not need to wait until the morning after

-Progestin-only emergency contraceptive; levonorgestrel 1.5mg in one dose
-Ulipristal acetate 30mg tablet
-Yuzpe regimen

-1.5mg once
-Brand names: Plan B One-Step, Take Action, Next Choice One Dose, My Way
-OTC with unrestricted sale to gender or age

-30 mg
-Brand name: Ella
-Sold by prescription only regardless of age
-More effective than levonorgestrel ECP's
-Preferred option when possible

-Combined OCP's
-May cause nausea

-Both male and female, are the only method of contraception that provide protection against STIs
-Adolescents should be counseled on how to obtain condoms and apply them correctly

-Coitus interruptus, or withdrawal, is not an effective method of contraception and adolescents should be counseled on that
-Other methods that are less effective are spermicides, the sponge, cervical cap, and fertility awareness

-Not using contraception or from using a contraceptive method incorrectly or inconsistently
-Only a small percentage of teen pregnancies actually result from contraceptive failure

-Teen pregnancy and unintended pregnancy in women of all ages are more likely to occur in women who are less educated and have a lower income
-Teen pregnancy statistics in the United States reflect racial and ethnic disparities in income and education
-Additionally, barriers to health care, sex education, and contraception correlate with the pregnancy rates
-The role of the PNP creates a unique opportunity to impact teen pregnancy through policy and practices

-Pregnancy testing should occur on any teen who is menarchal and has missed one or more periods
-Urine hCG tests can detect pregnancy 14-21 days from sexual intercourse or at the time of a missed period
-If the adolescent reports a positive home pregnancy test, a follow-up second test should be performed in the office as home pregnancy tests can be misread
-After confirmation, an abdominal exam should be performed

Disclosure of positive pregnancy results should be done in private, as the results may be difficult for the teen, her partner, and her family
-Preconceptual counseling is recommended for reproductive age women to improve outcomes for women who are sexually active with no birth control methods

-Should be provided to the teen, which includes a discussion of choices that women can make after finding out they are pregnant:
continue the pregnancy and raise the child
continue the pregnancy and make an adoption or allow a kinship care plan
end the pregnancy
-The options counseling does not need to occur immediately after delivering the diagnosis, but the PCP should offer nonjudgmental, nondirective options

-Pregnancy that occurs when the fertilized egg implants outside of the uterine cavity, usually in the fallopian tube
-Adolescents who present with vaginal bleeding and abdominal pain and bleeding should be assessed for pregnancy because ectopic pregnancy rupture is associated with pregnancy-related mortality

-Bowleg (knees tilted away from midline)
-Normal until 3 years of age

-Blount disease
-Rickets
-Tumor
-Neurologic problems
-Infection

-Tibial-femoral angle greater than 15 degrees
-Associated internal tibial torsion
-Lower extremity length discrepancy
-Intercondylar distance with the ankles together-measurement of greater than 4-5 inches suggest the need for additional evaluation
-Joint laxity of lateral collateral ligaments in older children

Weight bearing AP of the lower extremities with the patellas facing forward and a lateral radiograph of the involved extremity

-No active treatment, spontaneous resolution is expected
-Corrective shoes and bracing are unnecessary
-Reassure parents
-Observe the patient over time (in 3 to 6 months) to be sure the problem is resolving, especially during the second year of life
-Serial photographs of the legs can be helpful

-Blount disease may be treated with bracing in children <3 years
->4 years, a proximal tibial valgus osteotomy and associated fibular diaphyseal osteotomy are the procedures of choice
-Monitor to be sure braces are used consistently with good fit
-Observe to be sure the problem is not worsening

-Family history of pathologic bowing
-Asymmetric deformity
-Short stature (<25th percentile)
-Late walking (>18 months)
-Blount varus angulation/increasing deformity
-Progressive varus deformity after 18 months of age
-Presentation at 24 months or later

-Knock knees (knees go inward)
-Physiologic improves after 4 to 6 years of age
-Pathologic causes:
rickets
rental osteodystrophy
skeletal dysplasia
posttraumatic physeal arrest
tumors
infections

-Progression of the deformity
-Joint pain or stiff gait
-Older child may report knee pain due to the stretching of the medial aspect of the knee

-Bilateral tibial-femoral angle <15 degrees of valgus in the child up to 6 years of age is considered normal
-A valgus of >15 degrees is outside the range of normal
-Unilateral deformity
-Awkwardness of gait
-Sublaxating patella
-Intermalleolar distance with knees together: measurement >4-5 inches suggests the need for additional evaluation
-Short stature

-No radiographic studies are needed unless a pathologic condition is suspected
-Long-length AP radiographs of the leg in a weight-bearing stance are used for preoperative planning

-Deformities >15 degrees and occuring after 6 years of age are unlikely to correct with growth and require surgical intervention
-Short stature with genu valgum should be referred
-In the skeletally immature: medial tibial epiphyseal hemiepiphysiodesis or stapling for surgery
-In the skeletally mature: osteotomy for surgery

-Flatfeet
-Resolves by 3 years of age
-Flatfeet in neonates and toddlers are associated with physiologic ligamentous laxity
-Rigid flatfoot is pathologic
-When it persists into adolescence, its associated with familial ligamentous laxity

-Flexible flatfoot
-Flexible flatfoot with a tendo-Achilles contracture
-Rigid flatfoot

-Noticed with weight bearing
-Painless and asymptomatic
-Examine shoes to see if there is abnormal wear on the inner side

-There is a normal longitudinal arch when examined in a non-weight-bearing position, but arch disappears on standing
-On standing, the hindfoot collapses into valgus and midfoot becomes evident
-Generalized ligamentous laxity is commonly observed
-Range of motion should be normal in flexible flatfoot

-Symptomatic feet and rigid flatfoot should be treated; refer to a podiatrist
-For painful, flexible flatfoot, a removable longitudinal arch support may be recommended by the podiatrist
-If the achilles tendon is tight, passive stretching may be helpful
-Routine radiographs are not indicated unless pathologic flatfoot is suspected

-Talipes equinovarus
-At birth, the foot cannot be manually corrected to a neutral position with the heel down

-Ankle is in equinus (the foot is in a pointed-toe position)
-The sole of the foot is inverted as a result of hindfoot varus or inversion deformity of the heel
-The forefoot has the convex shape of MA (forefoot adduction)

-Note the inflexibility of sole of the foot
-It is inverted as a result of hindfoot varus or inversion deformity of the heel, and the forefoot has a convex shape with forefoot adduction
-Can be bilateral

-AP and lateral radiographs are recommended, often with the foot held in a maximally corrected position
-Radiographic measurements can be made to describe misalignment between the tarsal bones
-A common radiographic finding is "parallelism" between lines drawn through the axis of the talus and calcaneus on the lateral radiograph, indicating hindfoot varus
-Radiographs are not required in an infant to diagnose the anomaly

-Refer to orthopedist, ideally shortly after the infant is born because the joints are the most flexible in the first hours to days of life
-Nonoperative treatment should be initiated ASAP
-Taping and strapping, manipulation and serial casting
-Ponseti method
-For older children with untreated clubfoot or for those who have residual deformity, osteotomies may be required in addition to the soft tissue surgery
-Many children will require a percutaneous tenotomy followed by long leg cast with full time bracing for 3 months and then nightly bracing for 3 to 5 years
-Stiffness remains a long term follow-up concern

-Serial casting
-Weekly casting changes
-5 to 10 casts are usually required

-Twisting of the long bone along its long axis
-Normal torsion at birth is 2.2 degrees with a normal adult torsion of 23 degrees
-Torsion is anything outside of the range of normal

-Internal tibial torsion
-Abnormal medial rotation or twisting
-Results in in-toeing of the feet

-Consists of abnormal lateral rotation
-Results in out-toeing

-With the child prone and the knees flexed 90 degrees, the foot and thigh are viewed from directly above
-The foot should be relaxed
-Normal range at 13 years of age is -5 to +30 degrees

-Observe child's gait
-Use the thigh-foot angle to assess for rotation
-MTT exists if the TFA is negative by more than 10-20 degrees
-TFA of >30 degrees indicates an abnormal LTT
-Abnormal lateral torsion is associated with forward pointing patellas and outward pointing feet

-For the normal variation, observation and monitoring
-LTT with TFA >+30 degrees should be referred to an orthopedist because it usually worsens with growth and does not correct spontaneously

-Should be referred to an orthopedist if the problem is significant (TFA >-20 by 3 years of age)
-Stretching exercises or external rotational splints may be recommended
-Surgical intervention may be needed for severe cases that persist into late childhood and cause significant functional problems
-Special shoes are ineffective
-Avoidance of certain postures are controversial (sleeping in the knee-chest position and sitting with feet under the buttocks)

-Equinovarus
-Plantar foot flexion, forefoot adduction and hindfoot varus

-Metatarsal adductus
-Curved foot; refer if not flexible

-Abducted great toe
-Searching toe: resolves spontaneously

-Medial tibial torsion
-Refer if thigh-foot angle more than -10 to -20 degrees

-Internal femoral torsion
-Refer if >70 degrees medial and <10 degrees lateral hip rotation

-Physiologic infantile out-toeing
-Feet may turn out when infant is positioned upright: resolves spontaneously

-Lateral tibial torsion
-Lateral femoral torsion
-Refer if TFA is >30 degrees
-Refer if >2 standard deviations of the mean

-Some young children initially stand on their toes until they establish the heel-toe pattern
-Usually within the first 6 months
-Generally resolves before 3 years of age
-Important to differentiate between idiopathic toe walking and the child who toe walks because of a neuromusculoskeletal condition associated with tight heel cords and contractures

-Consistent toe walking is associated with neurologic problems such as cerebral palsy
-Autistic children or those with early muscular dystrophy
-Tight heel cords
-Unilateral toe walking can be associated with a short leg (dislocated hip)
-Can be a habit (children who use jumpers or walkers)

-Looking at the shoe wear and heel wear to assess extent of toe walking
-Assessing for tight heel cords, the foot should be brought beyond a 90-degree angle
-Conducting a neurologic assessment
-Measuring leg lengths and examining hips

-Depends on etiology
-Orthopedic management is needed for tight heel cords, unequal leg lengths and hip problems

-Head and neck of the femur are rotated at an increased angle anteriorly in relation to the femoral shaft, resulting in an in-toeing gait
-In-toeing is normal until 3 to 6 years

-In-toeing gait, most noticeable with running
-Runs awkwardly; may actually trip or fall as a result of crossing the feet while walking or running
-Possible family history
-History of "W" sitting

-In-toeing gait with patellas medial
-Internal (medial) rotation:
normally <70 degrees
mild deformity, 70-80 degrees
moderate deformity, 80-90 degrees
severe >90 degrees
-External (lateral) rotation decreased (limited to 0 to 10 degrees)
-Knees medially rotated (kissing patella) when standing

Radiographs are not merited unless surgery is contemplated

-Inform family that the condition is not harmful and there are not any serious consequences
-Observation and referral to orthopedist if medial rotations are significant (no external rotation of the hip in extension) or the child or family has significant concerns
-Nonoperative management are ineffective
-Operative correction is successful but carries risks
-Osteotomy is rarely performed and is done only if the child the child is >8years with significant cosmetic and functional deformity

-Does not cause flatfoot, bunions, knee problems, back difficulties, difficulties in running, or degenerative arthritis of the hip in adults
-Primarily a cosmetic problem unless it is severe enough to interfere with activities
-Self-esteem problems

Cannot be prevented

-Also referred to as hyperlordosis, is an AP curve of the lumbar spine
-It is the least common spinal deformity, it is associated with scoliosis and kyphosis, and it can be secondary to hip problems
-Congenital lumbar lordosis is often progressive
-Lumbar lordosis secondary to hip flexion contractures is associated with cerebral palsy, spina bifida, and developmental dysplasia of the hip

-Forward bend test should be performed, assessing for the presence or absence of the deformity with the lumbar spine flattened
-If the lordosis is present in the forward bending position, this indicates a fixed structural deformity and requires evaluation by an orthopedic specialist

Described as a three-dimensional deformity seen as a lateral curvature on the spine in the frontal plane

Caused by a rotation of the spine

-Also called functional scoliosis, is a curvature without rotation of vertebrae
-It is reversible and caused by posture, pain, or leg length discrepancy

-Most common type
-It is multifactorial and the three sub-types of idiopathic scoliosis are age-dependent
-Infantile: 0 to 3 years old
-Juvenile: 3 to 10 years old (most common in girls)
-Adolescent: 11 years and older

-Structural anomaly present at birth
-It is associated with other congenital anomalies and rapidly worsens, especially during periods of rapid growth

-Most common type present in nonambulatory patients resulting from weakness, imbalance, and spasticity of the trunk muscles
-Caused by neuromuscular pathologies such as cerebral palsy or muscular dystrophy

-Based on a curvature of more than 10 degrees using the Cobb method
-The Cobb method measures the angle between the superior and inferior end vertebrae (tilted into the curve) on a spine x-ray

-Should be performed in a standing position and observations should occur from both the front and side to look for asymmetry
-Adams test
-Functional scoliosis disappears when placed in the forward bending position
-Structural scoliosis is enhanced in the forward bending position

-Unequal shoulder height
-Unequal scapular prominences/height
-Unequal waist angles
-Unequal rib prominences and chest asymmetry
-Asymmetry of the elbow-to-flank distance
-Unequal rib heights in a forward bending position

-Can be done with the infant lying prone
-Leg length evaluation for discrepancies
-Examination of the skin for hairy patches, nevi, café au lait, and dimples
-Assess for signs of neurologic pathologies, including any weakness or sensory disturbance
-Evaluate for cardiac etiologies such as Marfan syndrome

-Standing AP/lateral spine x-rays
-PA radiographs is when they use the Cobb method
-An MRI scan of the spine may be considered when there is a suspected etiology of scoliosis based on the patient's age (infantile vs juvinile)

-Stop the progression of the curvature and to improve pulmonary function
-Idiopathic scoliosis may resolve, remain static, or worsen
-Treatment is dependent on the natural history of each curvature
-The treatment goal is to delay spinal fusion to allow for the pulmonary system and thoracic system to reach maturity and for achievement of maximum trunk height
-Genetic testing can be used to personalize treatment for children with adolescent idiopathic scoliosis

-Patients should be referred to orthopedic surgery
-Management for idiopathic scoliosis includes referral to an orthopedist or a specialty scoliosis center

Curves <20 degrees

-Curves >30 degrees
-Skeletally immature patients with curves of 20 to 25 degrees
-Braces must be worn 23 hours per day
-Compliance is a problem

-Curves 45 to 50 degrees
-Those who did not respond to bracing
-Vertical expandable prosthetic titanium rib (VEPTR): surgery indicated for children with curvatures that restrict pulmonary function

-Refers to a group of hereditary disorders affecting the skeletal muscle
-Results in progressive degeneration of skeletal muscle, which leads to weakness
-It is autosomal dominant and X-linked
-X-linked dystrophies are the most common and Duchenne muscular dystrophy is the most common

-Symptoms usually become evident between 3 and 6 years of age
-There may be a family history of muscle disease
-Children with MD may have delayed motor milestones, especially independent ambulation
-Children may present as toe walkers
-There may be a regression in motor skills, such as the ability to stair climb with ease
-Children with MD will become fatigued easily with activity and see good and bad days relating to their physical activities but will generally experience gradual difficulty with motor activities over time

-They will likely have large, firm calf muscles that feel "doughy" (fibrotic) on palpation
-Their stance will be wide-based, and they may exhibit a waddling, Trendelenburg gait
-Their lower extremities will demonstrate early weakness in the gluteal muscles and they will have a positive Gower sign

-Obtained by asking the child to get up off the floor without help
-The sign is positive if the child uses their arms to push off of their legs in order to move into a standing position
-The child first gets into prone position, then forms a triangle, and finally climbs on their own thighs

-Should be referred to an interprofessional team that includes orthopedic, metabolic, and physical therapy, social service, and nursing care
-Genetic counseling may be needed for some forms of MD

-Regulates growth, puberty and reproduction, homeostasis, and energy.
-Hormones from secreting cells can have actions at distant sites, paracrine actions on adjacent cells, or autocrine actions on secreting cells and are often activated by feedback loops.

Our bodies is driven by enzyme activity and are complex biochemical processes that work to transform amino acids, carbohydrates, and lipids to energy for use at the cellular level

They are a deviation from the normal growth pattern.

-They may be the first sign of an endocrine disorder.
-This is one reason why plotting height, weight, and head circumference on a growth curve, and monitoring these at each well-child check, is so important.

The parents' heights

Abnormalities in growth patterns.

(Mother′sHeight+5inches)+Father′sHeight)÷2

(Father′sHeight−5inches)+Mother′sHeight)÷2

-Skeletal dysplasias
-Chromosomal abnormalities (Turner syndrome)
-Genetic short stature

-Undernutrition
-Chronic Disease
-Endocrine disorder
-Constitutional growth delay

It is released in response to sleep, exercise, and hypoglycemia in series of irregular pupulsatile bursts and most activity of this hormone occurs during sleep.

Can be congenital or acquired.

-Idiopathic (MOST COMMON)
-Pituitary or hypothalamic disease
-Trauma
-Minor organic hypothalamic lesion
-Infection
-Radiation

-Details of pregnancy, delivery, newborn period.
-Chronic illness,
-Hypothyroidism symptoms
-Parents/siblings height, weight, and growth
-Age when growth decelerates
-CNS trauma

-Dysmorphic features: will have a large, childlike face with a large, prominent forehead
-Midline defects
-Evaluation of the fundi for evidence of increased intracranial pressure
-Palpation of the thyroid gland to assess for goiter
-Tanner staging for puberty
-Measures of body proportions
-Signs of intracranial lesion.

-Slow growth rate with normal birth weight
-Signs and symptoms of increased intracranial pressure
-Microphallus
-Proportional short stature
-Delayed bone age

-Deficiency or impairment in the secretion of growth hormone-releasing hormone

-If growth velocity is subnormal (including when prior heights are not available), the initial evaluation should include:
serum glucose to assess for hypoglycemia
CBC and ESR
UA and chemistry panel
growth factors
thyroid function tests
bone age X-ray of left wrist and hand
karyotype to rule out Turner syndrome in girls

They should be referred to a pediatric endocrinologist.

GH dosing is based on a child's body weight, with doses ranging from 0.16 to 0.24 mg/kg/week, given at night SQ.

Growth velocity may exceed 4 times the normal rates.

Height velocity over the first four months of treatment.

Therapy is very expensive.

-Glucose intolerance
-Pseudotumor cerebri
-Edema
-Growth of nevi
-Slipped capital femoral epiphyses
-Scoliosis.

-Typically, children who have poor growth because of chronic illness are also underweight and they have weight loss before they have growth deceleration, which is not the case with GH deficiency.
-So, if you are seeing this pattern then it's not due to a growth hormone deficiency but rather chronic illness.

-It is NOT a disorder but rather a common variant, and its characterized by delayed bone age with normal growth for that bone age.
-Children with CGD typically reach normal adult height, although they may be shorter compared to other family members.

-Variation of normal growth
-NOT a disease

First years of life with impaired growth

-Normal length/weight at birth with a slowed linear growth between one to three years of age.
-The child's height is at or just below the third percentile, they have delayed puberty and there's a history of a similar pattern in the family.

-Delayed bone age with normal growth velocity for bone age.
-Normal neurological exam.

-Growth velocity is normal after three years of age
-Delayed puberty with a pubertal growth spurt
-Delayed bone age
-Positive family history

None-final height is appropriate for parent's heights.

-The same screening tests used to evaluate GHD are completed to rule out any pathological conditions.
-A bone age X-ray can be helpful in distinguishing between CGD and idiopathic short stature.
-As stated previously, the child with CGD will have a bone age consistent with his or her height, age, whereas the child with idiopathic short stature will have a bone age paralleling their chronically age.

-Management for CGD is reassurance and support.
-A pediatric endocrine referral may be necessary to differentiate CGD from GHD and for possible hormone replacement therapy.

-They are tall about compared to their other family members as young children and enter puberty early, yet they ultimately reach a height within the normal range for their family.
-Rarely will this necessitate a referral to pediatric endocrinology.

Marfan's syndrome, Klinefelter syndrome, and other overgrowth syndromes.

Overnutrition.

Weight gain.

The weight percentile is further above the growth curve than the height percentile.

Exposure to excess adrenal androgens or gonadal steroids.

Findings of early puberty.

The timing of menarche and reaching sexual maturity rating stage 5.

12 years of age.

14 years.

9 to 14 years.

If puberty begins early, is delayed, or occurs out of sequence.

-Premature Thelarche
-Premature Adrenarche
-Isolated Menarche
-True Precocious Puberty

Isolated breast development without other signs; usually resolves.

-Early-onset of pubic/axillary hair in boys or girls; not associated with other features; usually idiopathic; increased risk of polycystic ovarian syndrome.
-Either boys (prior to 10 years old) or girls (prior to eight years old

-Uncommon. One to two episodes of bleeding without breast development.
-Need to exclude sexual abuse, vaginal tumor, ovarian cyst, and hypothyroidism.

The onset of multiple features of puberty.

Thelarche or pubarche occurring before eight years of age in girls and before nine years of age in boys.

Before seven years of age.

Accelerated linear growth, breast development, penile enlargement, and pubic hair.

It is usually idiopathic.

Thirty percent.

-Age of onset
-Type, duration, and progression of symptoms
-Pattern of growth
-Evidence of CNS lesion
-Family pattern
-Exposure to topical estrogens/testosterone, oral estrogens, or hormone disruptors.

-Stature and growth velocity
-Sexual maturity rating

-No laboratory studies are necessary for the infant or toddler girl unless other pubertal features are present or there's a continued increase in breast size
-Serum 17-OHP to exclude congenital adrenal hyperplasia (CAH), plus a 24-hour urine collection for 17-ketosteroids or imaging of the adrenal glands to exclude an adrenal tumor

-Thyroid function and a pelvic ultrasound to rule out the presence of an ovarian cyst or pelvic tumor.

-If it's true precocious puberty then obtain a bone age, LH, FSH, and estradiol or testosterone.
*If LH and FSH are high, an MRI is necessary to exclude a CNS tumor.
*If LH and FSH are low, obtain a GnRH stimulation test to distinguish central from peripheral puberty.
-If it's peripheral puberty, then:
*Pelvic ultrasonography for girls
*Testicular ultrasonography for boys
*Serum 17-OHP to rule out a severe form of CAH

-Management should be done under the guidance of a pediatric endocrinologist and depends on the underlying disorder, age of the child, degree of advancement of the bone age, and the child and family's response to the diagnosis.
-Radiation, surgery, and chemotherapy can be utilized for CNS disorders. Treatment is necessary for precocious puberty to increase final adult height for the child.

14 years of age

13 years of age

Menarche

Within four and a half years

Constitutional growth delay

-History and physical exam should forces on clues for any chronic illness, hypothyroidism or evidence of CNS symptoms.
-You'll want to ask about a pattern of growth , especially growth velocity, sense of smell and galactorrhea, which can indicate pituitary tumor.
-Anosmia or absence of sense of smell, along with delayed puberty are signs of Kallman Syndrome

-Focused screening for acute or chronic illness (CBC, sedimentation rate, C-reactive protein, urinalysis, liver enzymes, electrolytes)
-Bone age X-ray
-Free T4 (Thyroxine) and TSH (thyroid-stimulating hormone)
-Growth factors to assess for growth hormone deficiency
-Serum prolactin
-Luteinizing hormone (LH) and follicle-stimulating hormone (FSH), if gonadal failure is present, LH and FSH are abnormally elevated

-Gastrointestinal with poor weight gain
-Chronic renal failure
-Anorexia nervosa or bulimia
-Chronic anemia
-Respiratory or cardiac disease
-Medication-induced poor weight gain

-Endocrine diseases associated with delayed bone age
-Hypothyroidism

-Failure of the hypothalamic-pituitary-gonadal axis

-Refer the patient to a pediatric endocrinologist to determine etiology and treatment.
-Hormonal replacement is the treatment of choice for children with hypogonadism, whereas those with CGD need only reassurance that puberty will occur spontaneously but mostly later compared to their peers.

They are controlled by the hypothalamic-pituitary axis.

Corticotropin-releasing hormone (CRH).

Adrenocorticotropic hormone (ACTH).

The production of glucocorticoids (cortisol) and androgens.

Cortisol is highest in the morning.

Hypoglycemia, hypotension, pain, or other stressors.

Mineralocorticoid hormones (aldosterone).

The renin-angiotensin system.

Problems with blood pressure, renal blood flow, electrolyte balance, and the body's response to stress.

-Is a deficiency of hormones produced by the adrenal cortex, with deficits of cortisol and aldosterone being most detrimental to the body's ability to function.
-There are two forms of adrenal insufficiency: primary and secondary.

-Deficiency of both mineralocorticoid (aldosterone) and glucocorticoid (cortisol)
-Congenital Adrenal Hyperplasia (CAH) or acquired defect
-At risk for salt-wasting crises
-Hypoglycemia/hypotension

-Deficiency only of glucocorticoid
-Syndrome associated with hypopituitarism
-Hypoglycemia/hypotension

CAH is a deficiency of enzymes in the cortisol pathway and causes an inability to produce cortisol.

Female infants born with CAH manifest with ambiguous genitalia from excessive androgen exposure while in utero.

Male infants have no clinical signs and symptoms at birth except for possibly subtle hyperpigmentation and mild penile enlargement.

Newborn screenings routinely test for CAH to avoid a potentially life-threatening salt-wasting crisis in affected infants.

-Cortisol deficiency: Poor appetite, FTT, weakness
-Aldosterone deficiency: Vomiting, poor feeding, lethargy, dehydration

-The physical exam will show dehydration and hypotension.
-If the etiology is primary adrenal insufficiency, the child will present with excessive pigmentation of the skin and mucous membranes.

-Serum glucose (hypoglycemia)
-Blood gases and bicarbonate (metabolic acidosis)
-Electrolytes (low sodium, elevated potassium with aldosterone deficiency)
-Serum ACTH (elevated in primary adrenal insufficiency)
-Serum cortisol
-Serum 17-OHP (diagnostic in children with suspected CAH)
-Serum renin level (elevated in aldosterone deficiency)
-Aldosterone level (low in aldosterone deficiency)

-Treatment includes hormone replacement therapy which is managed by a pediatric endocrinologist.
-An adrenal crisis is a medical emergency and requires IV dextrose, normal saline, and stress doses of IV hydrocortisone.
-Parents should be educated on the need for stress doses of hydrocortisone when their child is febrile, has surgery, or there's trauma; they should also be taught how to administer hydrocortisone via IM injection in case the child is vomiting or is unable to swallow or keep down oral medication.

-Treatment of CAH requires a fine balance in replacing steroids, without causing androgen overproduction and the complications which come with excess corticosteroid administration.
-Lifelong therapy of CAH includes replacement doses of oral hydrocortisone.

-Can be caused by the administration of exogenous glucocorticoids or an endogenous excess attributed to pituitary or adrenal tumor.
-Most often this is caused by administration of glucocorticoids while treating an illness such as asthma, prevention of transplant rejection, or as part of chemotherapy protocols.

-Weight gain and growth failure
-Osteopenia
-Hypertension
-Delayed puberty
-Plethora (Hypervolemia)
-Acne, purple striae, hirsutism
-Compulsive behavior

-24-hour urine for free cortisol or late evening serum/salivary cortisol

-Wean steroid doses if needed administration goes beyond 7 to 10 days.
-Even after the steroid has been safely discontinued, the child may not be able to respond adequately to severe stress for as long as 6 to 12 months.

Abnormalities in sexual differentiation.

These abnormalities are usually present in infants with ambiguous genitalia.

They occur when the XX fetus is exposed to excess androgen in utero or the XY fetus is unable to produce or respond to androgens.

They may not be identified until the time of an inguinal hernia repair when a testis is discovered or during the teen years when they fail to develop pubic hair or menstruate.

Karyotype for an infant with ambiguous genitalia

-Counseling
-Referral to specialists

When an infant is in utero.

Thyroid releasing hormone (TRH).

Thyroid-stimulating hormone (TSH).

T4.

T3.

It is important for growth and development, basal metabolic activity, oxygen consumption, brain development, and metabolism of lipids, carbohydrates, and proteins.

It is an abnormality in thyroid gland development or ability to make thyroid hormones.

It occurs in 1 in 3,000 to 4,000 infants and is more common in females than males.

Congenital hypothyroidism.

Irreversible brain damage, growth failure, deafness, and neurological abnormalities.

It is identified with newborn screening and diagnosis is usually made in infancy.

Hashimoto's thyroiditis

An autoimmune condition

Destruction of the thyroid gland

Pituitary disease or hypothalamus dysfunction

Children with Type 1 diabetes

-Growth failure
-Delayed puberty
-Weight gain, fatigue
-Dry skin
-A decline in school performance
-Menorrhagia
-Family history of thyroid disease or other autoimmune disorders is often present

-May appear normal
-Most common signs include prolonged jaundice, constipation, and umbilical hernia
-Large anterior and posterior fontanelles
-Macroglossia
-Decreased muscle tone
-Poor feeder
-Respiratory distress
-Poor peripheral circulation with cool, cyanotic skin in the extremities

-Delayed/subnormal growth velocity
-Goiter
-Weight gain
-Delayed deep tendon reflexes (DTRs)

-TSH elevated/free T4 normal or low in older children
-Free T4 low/normal TSH in central hypothyroidism
-Total T4 low/free T4 and TSH normal in thyroxine-binding globulin (TBG) deficiency
*TBG is a protein that specifically carries hormones

-For TBG deficiency there's no treatment required because it causes no health problems.
-Hypothyroidism is treated with replacement doses of levothyroxine sodium.

-10 to 15 mcg/kg/day of Levothyroxine Sodium (L-thyroxine) (once daily)
-Targeted serum T4 concentration (more than 10 mcg/dL); if risk for cardiac failure consult additional references

-8 to 10 mcg/kg/day of Levothyroxine Sodium (L-thyroxine) (once daily)