Medstudy 2

Where are calcifications in toxoplasmosis?

All throughout the brain parenchya, especially in the caudate nucleus and basal ganglion.

Polyostotic fibrous dysplasia seen in what?

McCune-Albright syndrome, which also consists of large café au lait spots and endocrine hyperfunction.

Absence of corpus callosum seen in what?

trisomy 8, trisomy 18, and Aicardi syndrome (X-linked dominant disorder characterized by agenesis of the corpus callosum, infantile spasms, and retinal abnormalities).

Roseola

• aka?

• aka roseola infantum, exanthema subitum

• HHV-6

• incidence peaks between 6–15m

• pp

  • High fever and irritability

  • Rash

    • Fist seen 12-24h after defervescence

    • Begins on trunk → face, neck, postauricular area, prox extremities.

    • Lesions p ersist 1-3d, and are sually eddish-pink, discrete, and slightly papular rathrthan vesicular or petechial.

  • postauricular and/or occipital lymphadenopathy

  • edematous eyelids associated with palpebral conjunctivitis (Berliner sign)

  • macules and/or ulcerations at the junction of the uvula and soft palate (Nagayama spots)

  • bulging fontanelle

  • Upper respiratory and gastrointestinal symptoms may also occur

  • May see febrile seizures

• Tx

  • If toxic appearing and bulging fontanelle → ust get LP to evaluate for meningitis.

Less common parvovirus B19 presentation:

papular purpuric gloves and socks syndrome: tender, pruritic papules, petechiae and purpura of the hands and feet associated with edema.

Treatment for chancroid

• It is haemophilus ducreyi

• single dose of 1 g of oral azithromycin OR

• single intramuscular dose of 250 mg of ceftriaxone

• less desirable:

  • over 18 years of age, ciprofloxacin 500 mg orally twice daily for 3 days is equally effective but less desirable because multiple doses are required.

  • erythromycin base 500 mg 3×/day for 7 days is also effective, but it is not well tolerated during pregnancy; it, too, is less desirable because multiple doses are required.

Guillan Barree

• CSF findings

• Symptoms

• CSF

  • Elevated protein

  • Normal glucose

  • Normal WBC count

• pp

  • Flacid paralysis

  • Areflexia

  • Symmetric sensory and/or motor symptoms, often in association with severe pain.

  • Typically begin in the lower extremities and may progress to involve the trunk, upper extremities, and in some cases, the cranial nerves

  • May see respiratory depression and failure

  • Bradycardia, dysrhythmias, and fluctuations in blood pressure due to autonomic dysfunction can occur

Place what in the ear to remove a live insect?

Lidocaine or Mineral Oil

Maternal meds assoc with gastroschisis?

Other maternal factors assoc with gastroschisis?

Gastroschisis is caused by?

Elevated levels of maternal what are common?

• Meds: Cocaine and other vasoactive substances (e.g., nicotine, pseudoephedrine)

• Other factors: teen pregnancy, maternal hot tub use, and in utero exposure to alcohol and smoking.

• Caused by a vascular accident involving the right umbilical vein or right omphalomesenteric artery that leads to ischemic necrosis of the abdominal wall allowing bowel to enter the amniotic cavity.

• Elevated leels of serum α-fetoprotein.

Isotretinoin can cause what in a pregnancy?

• Congenital defects inluding

  • craniofacial, cardiovascular, neurological, and thymic malformations.

  • 30-60% are shown to have neurocognitive impairment, even in absence of physical defects

What associations are there for carbamazepine and pregnancy?

• congenital malformations, including spina bifida, craniofacial defects, cardiovascular malformations, and hypospadias.

IVIG given. What about MMRV?

Diminished immunogenicity if it was given within 2w prior, or within 11m after. Hold for ≥11m after giving IVIG.

If at risk, give vac, then give again ≥11m.

Screenings for those with T1DM:

• Starts when?

• What includes?

• Starts 5y after dx of diabetes

• Nephropathy: annual spot urine albumin:cr ratio at puberty or age > 10y (whichever is earlier)

• Retinopathy: q2y, dilated comprehensive eye exam at puberty or age 11y (whichever is earlier)

• Neuropathy: Annual comprehensive foot exam at puberty or age 10y (whichever is earlier)

• Annual TSH

• Lipid testing: Start when glycemic control is achieved and age is 2y. If initial LDL = 100, subsequent testing done once at 9-11 and repeated q3y thereafter.

vWD

• plt function abnormality

• PT always normal; PTT usually normal; Platelet function analysis (PFA) prolonged (PFA preferred over bleeding time)

• Type 1

  • most common (90%)

  • autodom

  • decreased, but not absent vWF.

• Type 2

  • functional defects in one of several activities (plt binding, factor 8 binding, multimeric asembly)

  • has several subtypes\

    • subtype 2n is autorecess

• Type 3

  • Absence of von willebrand antigen.

  • most severe variant

  • autorecess

Risk factors for dental caries?

When to see the pediatric dentist?

When to start fluoride varnish?

• RFs

  • Children with special health care needs

  • Premature birth

  • Late-order offspring

  • Primary caregiver or siblings with caries

  • Children with existing caries, demineralization, and/or staining

  • Infants sleeping with a bottle

  • Frequent snacking/excessive use of sippy cups

  • Breastfeeding after 24 months of age

  • Bottle use after 12 months of age

  • Low socioeconomic status

  • Poor parental dental health

• See a dentist:

  • By 12m for normal risk

  • If risk factors, as early as 6 months of age and no later than 6 months after 1^st tooth eruption or 12 months of age, whichever comes first.

• Fluoride

  • Done q3-6m starting at tooth eruption

When to refer to speech therapy for stuttering

• Ss severe or last > 4-8w

• stuttering causes anxiety or avoidance

• Fam hx of the disorder

Can also do cognitive behavioral therapy

What causes omphalocele?

What causes gastroschisis?

• Omphalocele

  • Failure of central fusion at the umbilical ring due to defective mesodermal growth, followed by herniation of the midgut.

• Gastroschisis

  • LIkely the result of a vascular accident invlving the omphalomesenteric artery or defective mesenchymal development at the junction of the body stalk and abdominal wall, leading to disruption of the abdominal wall and evisceration.

Initial tx of choice for long QT syndrome

b-blocker

Lymphocytic interstitial pneumonitis

• What is it?

• What on x-ray?

• Most commonly seen in who?

• one of the rare interstitial lung diseases seen in children

• homogenous chronic ground glass opacities

• most commonly seen with HIV as well as scleroderma

  • LIP and HIV is a well-described association

When is measles contagious?

3-5d prior to rash through 4d after its appearance

Which live vaccine is contra in those who live with an immunocompromised person?

LAIV (live attenuated influenza vaccine)

Labs you can see with anabolic steroid use

• ↓ HDL

• ↑ LDL

• ↑ serum transaminases

Is cystic fibrosis a restrictive or obstructive pattern?

Obstructive

Enterovirus aseptic meningitis

• pp

• dx

• tx

• morbidities

• pp

  • ha, ↓ activity, malaise, ↑ temp, photophobia, nuchal rigidity. Many have a rash.

• Dx

  • CSF

    • ↑ WBC

    • Normal glucose

    • Slightly-to-moderately ↑ protein

    • Neutrophhils may predominate in the first day or two of illness but transition to a lymphocytic pleocytosis within several days.

  • Confirmation

    • Usualy with PCR of spinal fluid for enterovirus RNA.

• Tx

  • Symptomatic and supportive care

• Morbidities

  • Most common are ha and impaired attention which resolve over months.

lead interferes with what process?

• Heme biosynthesis

  • inhibits d-aminolevulinic acid dehydratase (ALAD) and ferrochelatase activity → ↑ free erythrocyte protoporphyrin (FEP) in the blood due to inability to complete the heme syntheis pathway.

• FEP elevated in lead and iron def, but is extremely high in lead.

What levels of lead require what levels of treatment?

• > 5 µg/dL: confirmation with a venipuncture. ensure adequate intake of iron and calcium, milestone assessment, anticipatory guidance about sources of lead exposure, and follow-up testing

• >10 µg/dL: environmental investigation/intervention to remove potential sources of lead is also performed.

• ≥ 45 µg/dL: chelation

Severe allergy to yeast is a contra for what vaccine?

HPV

7 + 4 rule for teeth

7m → first teeth.

11m (7+4) → 4 teeth

15m (11+4) → 8 teeth

19m (15+4) → 12 teeth

23m (19+4) → 16 teeth

27m (23+4) → 20 teeth

Properdin deficiency

is C5-C9 deficiency

Giving a meningococcal vaccine in someone less than 2 years

• Menveo (MenACWY-CRM)

  • The only one that can be used.

  • If 7-23m: 2-doses ≥3m apart and 2nd dose must be in 2nd year of life

  • If ≥2y: 2 doses ≥8w apart

Giving the Menactra vaccine

• Must be ≥24m to avoid interference with PCV13 and 15.

  • 2 doses ≥8w apart. 1st dose ≥4w after completion of all PCV-13 or 15 doses.

Giving MenACWY-TT (MenQuadfi)

• Must be ≥24m

• 2 doses ≥8w apart

Transverse myelitis

• What is it?

• Some common reasons to get it?

• pp?

• Dx

  • CSF?

  • MRI?

• Tx?

• It is segmental spinal cord inflamation.

• sometimes is preceded by an illness associated with nonspecific systemic symptoms

• Comon reasons are EBV, rubella, mumps, varicella, influenza, Mycoplasma pneumoniae, and Borrelia burgdorferi; an autoimmune etiology is thought to be likely in many other cases.

• pp

  • Sudden progressive weakness and multimodality sensory disturbances (i.e., pain, temperature, touch) in the lower extremities.

• Dx

  • CSF: Mild lymphocyte pleocytosis, normal or slightly elevated levels of protein

  • MRI: spinal cord swelling with hyperintensity on T2-weighted imaging

• Tx

  • Corticosteroids

What causes in utero meconium passage

usually results from fetal hypoxic stress

a preductal and postductal oxygen gradient can indicate what?

pulmonary hypertension

conjunctivitis vs uveitis

Uveitis is inflammation of the structures underneath the sclera which include choroid, ciliary body, and iris.

Conjunctivitis is inflammation of the sclera which is above the uvea.

Enthesitis-related arthritis criteria for dx

• Enthesitis and arthritis, or

• Either enthesitis or arthritis with ≥ 2 of the following:

  • History or presence of sacroiliac joint tenderness and/or inflammatory lumbosacral pain

  • Presence of HLA-B27 antigen

  • Onset of arthritis in a male > 6 years of age

  • Acute symptomatic uveitis

  • A 1^st degree relative with ankylosing spondylitis, ERA, sacroiliitis with inflammatory bowel disease, or reactive arthritis

Stickler Syndrome

• ~20% of Pierre robins have stickler syndrome

• Stickler syndrome is a group of several genetic disorders affecting connective tissue, specifically collagen.

• stickler also assoc with ophthalmologic (myopia, cataracts, vitreous anomaly, retinal detachment), auditory (sensorineural or conductive hearing loss), and articular (joint hypermobility, early onset osteoarthritis, spondyloepiphyseal dysplasia) abnormalities.

Incontinentia pigmenti

• Stages?

• Systemic findings?

• 1: crops of inflammatory vesicles and bullae over the trunk and extremities

  • Often w/ periph eosinophilia

• 2. Vesicles clear and are replaced by irregular linearly distributed verrucous (wartyish) lesions on the extremities, hands, and/or feet.

• 3. thin bands of “swirly” brown, bluish-gray pigmentation that often coalesce and darken further before slowly fading by adolescence

• 4. As they fade, you get atrophy and hypopigmentation.

• Systemics

  • Dental (delayed dentition, pegged or conical teeth, partial anodontia)

  • Alopecia

  • Nail changes

  • Varying degrees of mental disability and/or seizure dsorders.

  • Eye problems (strabismus, cataracts, retinal detachment)

What is the rash seen in Langerhans cell histiocytosis?

Erythema and greasy, yellow scales over the trunk and extremities

• What is it?

• More in males or females?

• Associateds?

• Is it malignant?

• Tx?

• Becker nevus

• M>F

• may be congenital, but more often appears in adolescence

• the presence of hypertrichosis is characteristic of a Becker nevus

  • Hypertrichosis usually develops 12–24 months after changes in pigmentation are first noted.

• usually located on the trunk (especially chest, shoulder, or upper back)

• Most patients have no additional findings, although some patients do have associated anomalies (the so-called Becker nevus syndrome), including ipsilateral hypoplasia of the chest, breast, and areola; limb shortening; pectus deformities; scoliosis; and spina bifida occulta.

• Malignant changes do not occur.

• Tx is cosmetic only.

Criteria for Tourettes

• At least 2 motor tics and at least 1 vocal (phonic) tic have been present, not necessarily at the same time.

• Tics may wax and wane in frequency but have occurred for more than 1 year.

• Tics started to appear before the age of 18.

• Tics are not caused by the use of a substance or other medical condition.

dermoid cysts vs epidermal cysts

• Dermoid cysts

  • second most common midline neck mass after thyroglossal duct cysts

  • result from entrapment of skin along lines of embryonic closure

  • differ from epidermoid cysts in that they contain skin appendages, such as sebaceous glands and hair follicles within the cyst wall.

• Epidermal cyst

  • may occur on any hair-bearing skin

• Both do not elevate with swallowing

Branchial cleft cyst

• congenital lesions that arise from remnants of a slight cleft or defect during gestation

• Located laterall

• May change in size and shape

• often noticed after an upper respiratory tract infection

• Branchial cleft cysts or sinuses may have external openings or pores from which a mucus-like material drains out

• Criteria for Lyme ppx?

• Meds for ppx?

• A single dose of doxycycline prophylaxis (4.4 mg/kg/dose or 200 mg max) to prevent Lyme disease can be offered to nonpregnant adults and children of any age when all the following circumstances are satisfied:

  1. Doxycycline is not contraindicated.

  2. The tick can be identified as an adult or nymphal Ixodes scapularis tick (diagnostic tick testing for expert identification is recommended in 2020 guidelines but not testing the tick for Borrelia).

  3. The time of attachment is ≥ 36 hours based on the degree of tick engorgement or time of exposure to the tick.

  4. Prophylaxis can be started within 72 hours of tick removal.

  5. Lyme disease is common in the location where the patient lives or has recently traveled (high endemicity is when > 20% of nymphal ticks and > 50% of adult ticks are infected).

• Ppx

  • A single dose of doxycycline would be the choice for prophylaxis

• Tx

  • Doxycycline preferred (10d for E migrans) in adults and kids, regardless of age, due to its activity aginst burgdorferi and other oganisms transmitted by ixodes tick like anaplasma or ehrlichhia.

  • Amoxicillin or cefuroxime for 14 days are alternative options for treatment of erythema migrans (single or multiple) at any age

    • amoxicillin is not indicated to treat neurologic manifestations of Lyme disease (e.g., facial nerve palsy, meningitis)

  • Amoxicillin is preferred to doxycycline in children < 8 years of age with Lyme arthritis due to the length of recommended treatment (28 days)

  • IV ceftriaxone is indicated for treatment of Lyme disease if the patient has severe/complicated neurologic symptoms, early in course of treating Lyme carditis, or for treatment of Lyme arthritis unresponsive to oral agents.

Fetal warfarin syndrome

• Happens if there is exposure during 6-9 weeks GA

• pp

  • nasal hypoplasia

  • stippled epiphyses

Bone findings in congenital syphilis

• Osteochondritis and Periostitis, usually involving the metaphysis and diaphysis of 1 or more long bones.

• Metaphyseal lesions appear as irregular bands of decreased mineralization or focal circumscribed areas of bony destruction; e.g., destruction of the medial aspect of the proximal tibial metaphysis (Wimberger sign).

infantile cortical hyperostosis

a.k.a. Caffey disease

Radiographic findings of infantile cortical hyperostosis (a.k.a. Caffey disease) include significant cortical thickening (most often of the mandible, clavicle, and ulna) and periosteal reaction in otherwise normally mineralized bone.

When is toilet training usually complete?

The average completion of toilet training is at 30 months of age, with roughly 75% of children attaining daytime continence by 36 months of age.

Tympanogram type A

• Normal middle ear function

• “Normal box values” →

  • -150 0 +50 daPa (x-axis)

  • Peak compliance: 0.2-1.8 cc (y-axis)

• Thre is absence of middle ear pathology

• TM is mobile and intact

• Eustachain tub function is normal

• If there is hearing loss, it is likely sensorineural.

Tympanogram tye A(s)

• Shallow; poorly compliant middle ear system.

• Think: TM scarring, TM sclerosis, otosclerosis, or ossicular fixation.

• Compliance curve is in the normal position (x-axis), but peak compliance is very low (< 0.2 cc)

• NOT due to middle ear effusion!

• Conductive hearing loss

Tympanometry type A(d)

• Highly compliant middle ear system.

• Think: Ossicular disarticulation or hypermobile TM

• Peak compliance occurs between -150-50 (ie normal)

• Peak compliance is very high

• Conductive hearing loss is possible.

Tympanometry type B

• Retracted, poorly mobile.

• Think: Middle ear effusion or perforated ear drum

• Compliance curve is nearly flat to completely flat and always below normal.

• Negative middle ear pressure (Peak compliance is shifted left)

• Conductive hearing loss

Tympanometry type C

• Normal compliance but negative pressure in the middle ear.

• Think: eustachain tube blockage

• Clear peak

• Peak compliance has normal amplitude

• Peak compliance is shifted to the left (negative middle ear pressure)

• Conductive hearing loss is likely.

Magnesium exposure for neonates

• Mag is given to mothers to treat pre-eclampsia or for tocolytics.

• Elevations in neonate serum mag conc (>2.3 meq/L)L

• Levels <5 usually don’t result in serous comps but may cause lethargy and difficulty with feeding.

• pp

  • resp depression, apnea, generalized hypotonia.

  • Additional comps can be GI hypomotility and meconium plug syndrome mimicking intestinal obstruction.

• Tx

  • If kidneys normal, most only need symptomatic care for several days with monitoring of fluid intake and resp status.

  • In severe cases, tx with IV calcium and diuretics may be needed.

Adrenoleukodystrophy

• What causes it?

• inheritance?

• pp age?

• pp?

• dx?

• tx?

• Disorder of peroxisomal degradation of fatty acids → accumulation of very long-chain faty acids in the central and peripheral nervous system and adrenal glands.

• X-lnked recessive

• Usually presents between 5-15y

• pp

  • Due to associated cortisol deficiency (up to 70% of cases) → ↑ ACTH precursor (proopiomelanocortin) → ↑ melanin synthesis → hyperpigmentation of knees, elbows, palmar creases, axillae, gingival borders.

  • Seizures

  • Impaired auditory discrimination

  • Spatial orientation disturbances

  • Visual disturbances

• Dx

  • Symmetric periventricular white matter lesions in the posterior parietal and occipital regions of the CNS on CT or MRI are commonly idenified.

• Tx

  • Treat the adrenal insufficiency.

  • Pts may benefit from a bone marrow transplant.

Tinea capitis

• Most often by Trichophyton tonsurans

• Is an endothrix infection, meaning the dermatophyte invades the hair shaft and internalizes into the hair cell.

  • Does not fluoresce with wood lamp

• pp

  • Can have kerion (boggy plaque, inflamed, can have alopecia, pustules, crusting, purulence)

  • patches of alopecia often asoc with black dots, which are the distal ends of hairs that have broken at the surface of the scalp.

• Tx

  • Oral antifungal (griseofulvin (drug of choice) or terbinafine)

  • griseofulvin is 6-8w. take with milk or fatty meal

  • Ketoconazole shampo added to prevent spread to other inds.

  • some recommend household members use antifungal shampoo to prevent reinfection of a patient.

  • systemic corticosteroids occasionally added for inflammation and to prevent permanent alopecia. Benefit is unclear.

Infantile glaucoma

• associated conditions can be galactosemia, trauma, intraocular hemorrhage, ocular inflamm disease, sturge-weber syndrome, marfan, neurofibromatosis, lowe syndrome congenital rubella, juvenile xanthogranuloma

• pp

  • excessive tearing

  • photophobia

  • blepharospasm

  • corneal edema (and/or cloudy)

  • corneal and ocular enlargement

  • conjunctival injection

  • visual impairment

• Tx

  • Surgery to alleviate intraocular pressure immediately indicated

Congenital varicella syndrome

Hypoplastic limbs, cutaneous scars, cataracts, chorioretinitis, microphthalmos, and cortical atrophy.

Some causes of rectal prolapse

• Functional constipation and prolonged straining during defecation = #1

• chronic diarrhea caused by enteric and parasitic infections such as Trichuris trichiura = #2

• Cystic fibrosis (CF), malnutrition, polyps, and conditions leading to pelvic floor weakness are other common etiologies.

Dx criteria for NF1

By definition, individuals with NF1 must have at least 2 of the following findings:

• ≥ 6 café au lait macules measuring > 5 mm in greatest diameter in prepubertal individuals and > 15 mm in greatest diameter in postpubertal individuals

• ≥ 2 neurofibromas of any type or ≥ 1 plexiform neurofibroma

• Axillary or inguinal freckling

• ≥ 2 iris hamartomas (Lisch nodules)

• Optic glioma

• A distinctive osseous lesion, such as sphenoid dysplasia or thinning (bowing) of the long bone cortex (with or without pseudoarthrosis)

• A 1^st degree relative with NF1

Charcot-Marie-Tooth (CMT) disease

• aka?

• aka hereditary motor sensory neuropathy

• present in the 1^st or 2^nd decade

• family history is common

• pp

  • progressive distal weakness

  • mild to moderate sensory loss

  • depressed or no tendon reflexes

  • high-arched feet (pes cavus)

  • Muscle atrophy develops and leads to “stork legs.

  • Upper extremitieseventually involved in later stages.

Mononeuritis multiplex

• An asymmetric pathology of the peripheral nerves, which is typically painful.

• Is often associated with other disease states, such as

  • diabetes mellitus, amyloidosis, rheumatoid arthritis, or systemic lupus erythematosus.

• Diagnosis requires involvement in 2 separate nerve areas.

• As the disease progresses, muscle weakness and atrophy may develop

What is the most common cause of spinal cord compression in pediatrics?

spinal cord tumors

Most will have some combination of back pain, weakness, gait disturbances, scoliosis, and/or bladder dysfunction

In younger children, motor regression can be a presenting symptom.

Caudal regression syndrome

• A group of congenital anomalies that may involve the lower vertebral bodies, sacrum, spinal cord, and lower extremities; or, presents as sirenomelia in extreme cases (sirenomelia = mermaid syndrome = fused legs)

• 25% of cases assoc with maternal T1DM.

  • also been linked to env factors like exposure to organic solvents during pregnancy and folate deficiency.

• pp

  • Motor and sensory impaired below the level of the affected vertebrae.

  • Many have lifelong bowel and/or bladder incontinence, and some lower extremity dysfunction

  • There are assoc deformities of the hips, legs, and/or feet, in addition to progressive scoliosis, which often complicate the clinical course.

Uncoontrolled hyperthyroidism in pregnancy - probs for baby

↑ risk of preterm and low birth weight. Neonates with high thyrotropin receptor antibody titers are at ↑ risk of developing hyperthyroidism a few days after birth.

Bath salts

• Sympthomimetic syndrome with stimulant and hallucinogenic effects

• pp

  • ↑ bp, ↑ hr, ↑ rr, ↑ temp, diaphoresis, mydriasis, anxiety, agitation, combativeness, paranoid delusions, and/or seizures

• Dx

  • Clinical

  • Synthetic cathinone comounds may not be detected on routine urine drug screening

• Tx

  • Psychomotor agitation best treated with IV diazepam or lorazepam.

  • Treat assoc ele levels and/or hypoglycemia (decreases risk of seizures)

• Comps

  • arrhythmias, extreme hyperthermia, intracerebral hemorrhage, rhabdomyolysis, acute tubular injury (a.k.a. acute tubular necrosis), and electrolyte abnormalities (e.g., hyponatremia, hypokalemia, hypermagnesemia).

Which antiseizure med is assoc with neural tube defects

Valproate

Up to 2% of fetuses exposed to valproate in utero develop neural tube defects (NTDs), a 4× increase when compared to babies born to mothers with epilepsy who used other antiseizure monotherapies

Fetal exposure to valproate may also have adverse effects on cognitive and neurologic function (e.g., lower IQ scores, difficulty with verbal reasoning skills, delay in motor development, increase in the prevalence of autistic spectrum disorders).

Fetal exposure to phenobarbital can cause what?

cardiac, orofacial, and genitourinary anomalies

Contras to oral rehydration can include:

hemodynamic shock, abdominal ileus, persistent emesis, intestinal intussusception, stool output in excess of 10 mL/kg body weight/hour, and monosaccharide intolerance.

25 kg child has a 5% fluid deficit, use holliday-segar to find the fluids for the next 24h:

• “100/50/20”

• First 10 = 1,000mL, next 10 = 500 mL, remaining 5 = 100 mL. Total 1,600

• Deficit in liters = 5% (0.05) * 25 kg = 1.25 L = 1250 mL

• Add together = 2850 mL needed for next 24h.

Kwashiorkor

• protein-energy malnutrition

• low protein caarbohydrate diets (rice, casava, yams)

• pp

  • edematous abdomen

  • pitting edema

  • hepatomegaly

  • brittle hair

  • reddish-brown scaly rash

  • pigmentary changes

  • T-cell function and cell-mediated immunity affected → ↑ risk of infection

Marasmus

• protein-enegy malnutrition without edema

• severe nutritional deficit in calories

• generalized loss of muscle

• little or no subcutaneous fat

• appear emaciated

• loose, wrinkled skin due to the loss of subcutaneous fat

  • have the appearance of a “wizened old man” due to their loss of temporal and buccal fat pads

• frequently hypothermic, bradycardic, and hypotensive due to their chronic energy deficit

What are white line of Frankel seen in?

Vitamin C deficiency (Scurvy)

Lupus anticoagulant

• What is it?

• What does it cause?

• What about a 1:1 plasma mixing?

• It is a prothrombotic, antiphospholipid antibody.

  • It causes nonspecific inhibition of various clotting factors which leads to prolonged PTT.

• Will not correct with mixing of normal plasma

  • This because the prolonged PTT is caused by an inhibitory factor and not by deficiency of a clotting factor.

• It can be the first sign of SLE

Factor V Leiden

• An inherited thrombophilia

• It is assoc with activated protein C resistance.

Treatment for late lyme disease arthritis

• Late lyme

  • 28 days of oral doxy for those ≥8y or

  • amoxicillin for those < 8y, but amoxicillinis not approved for any neurologic lyme disease.

  • parenteral ceftriaone is used for lyme arthritis that is unresponsive or worsening desite oral therapy

• Early disseminated lyme

  • oral doxy x14d.

• Neuro lyme

  • No amoxicillin

  • Use IV ceftriaxone

• Cardio lyme

  • Start with IV, then switch to PO to complete the course (14-21d)

• Erythema migrains (single or multiple)

  • All ages: Oral doxy x10d, or an oral b-lactam (amoxicillin or cefuoxime) x14d.

Burkitt lymphoma endemic form

• African

• jaw or facial bone tumor that spreads both locally (oral, nasal, and sinus cavities; often associated with inappropriate loosening of the teeth) and to distant sites, including the mesentery, ovary, testis, kidney, bone marrow, and meninges

• Linked to EBV

Burkitt lymphoma nonendemic

• sporadic type

• presents with abdominal distention and ascites due to extensive disease within the abdominal cavity and with distant spread to the bone marrow and/or central nervous system.

• originates from relatively mature B cells within Peyer patches and may lead to intussusception.

• Linked to EBV

Gianotti-Crosti syndrome

• aka papular acrodermatitis of childhood

• multiple, slghtly edematous and erythematous papules on the face, but, extensor surface of forearms and legs. spares trunk.

• papules (or, less commonly, papulovesicles) may persist for weeks to months and are typically nonumbilicated, monomorphous, and brownish-tan in color, ranging in size from 1 mm to 10 mm

• Koebner phenomenon

  • (lesions that appear or become more prominent at sites of trauma)

• Upper respiratory symptoms, fever, diarrhea, hepatosplenomegaly, and/or lymphadenopathy may be noted prior to the appearance of the rash, suggesting an underlying infectious trigger.

• EBV most common cause

  • RSV, hep b, group a strep, myco pneumo, enterviruses, parainfluenza, parvovirus, hhv6, rotavirus, adenovirus.

Urticaria pigmentosa

Urticaria pigmentosa is characterized by brown macules that urticate when stroked (Darier sign).

normal prepubertal estradiol levels are what?

< 10 pg/mL.

Transient neonatal pustular melanosis

• benign, self-limited, unknown origin, most common in Black neonates.

• present at birth

• pp

  • characterized by superficial 2–5-mm vesiculopustules, which spontaneously rupture within several days, leaving pigmented macules with a fine collarette of scale.

  • hyperpigmentation fades in 3 weeks to 3 months

  • Infants may have any combination of pustules, scale, and/or hyperpigmented macules

  • typical distribution is along the forehead, anterior neck, upper shoulders, lower back, and legs.

• Dx

  • Clinical

  • Although unnecessary, examination of the contents of the pustules will reveal multiple neutrophils.

• Tx

  • None

ACEIs and fetal health

• 1st tri

  • Congenital cardiovascular (e.g., septal defects, patent ductus arteriosus) and CNS (neural tube defects)

• 2nd & third tri

  • Renal anomalies including neonatal renal failure

  • Fetal growth restriction

  • Oligohydramnios

  • In utero fetal demise

  • Pulmonary hypoplasia

  • Limb anomalies

B-blockers and pregnancy

• Propranolol → ↓ growth in utero (may be confoundes)

• Propranolol → bradycardia and/or hypoglycemia. Short-lived. Respond to supportive care until drug is metabolized.

• Labetalol first line

Toilet training

• Introduce the topic at 12m and tell parents it shouldn’t begin until ≥18m and only if kid shows interest in doing so at that time.

• Girls go faster than boys

• Firstborns often are later

• Ma require up to 3m of taining and there are often setbacks.

• Readiness signs

  • sphincter control, regular patterns of elimination, ambulates well, follows 2-step commands, pulls pants up and down, dislikes being wet, imitates behaviors, expresses an interest in the potty, and displays emerging independence.

When is foreskin retractable?

by 3 years of age, over 90% of males have easily retractable foreskin.

Pediatric Type 2 DM management

• If not metabolically decompensated at presentation, management depends on HbA1c.

• HbA1c < 8.5%

  • Metformin and lifestyle (diet and exercise) modification.

• Hba1c gqu 8.5%

  • Basal insulin + metformin is considered

  • After establishing negative pancreatic autoantibodies, insulin should be titrated and liraglutide may be considered.

Salmonella diarrhea and treatment

• With uncomplicated Salmonella → no abx; treatment prolonges shedding and increases the risk of resistance.

• Exceptions are

  • older adults

  • very young (< 3 months old)

  • immunocompromised

  • These should be treated with antibiotics because the risk that Salmonella may disseminate or cause more extensive problems is greater than the risk of prolonged shedding.

  • If antibiotics are to be started, obtain a blood culture before initiating antibiotics.

Complex regional pain syndrome

• more common in high-achieving adolescents

• pp

  • Allodynia (pain from light touch)

  • hyperalgesia (↑ sensitivity to pain) of the region

  • localized autonomic dysfunction with edema, coolness or excess warmth, mottling, and/or sweatiness.

• Tx

  • Aggressive PT and OT

    • In many cases, physical therapy includes desensitization to textures, pressure, and temperatures (particularly in those with allodynia)

  • Mental health support

  • do not immobilize the area. Pain will diminish as extremity function improves with physical therapy and range-of-motion exercises.

• Comps

  • CRPS can result in long-term disability and atrophic changes leading to permanent damage in the involved extremity.

Rosacea treatment

• If a papulopustular component → topical metronidazole.

  • Alt: azelaic acid, sufacetamide/sulphur, benzoyl peroxide (with or without a topical antibiotic), and retinoids.

  • Oral tetracyclines are often beneficial in a more severe disease and may be combined with a topical agent.

    • Alternative oral agents: erythromycin, azithromycin, and metronidazole.

  • Oral retinoids: may lead to improvement in patients not responding to initial therapies.