Osmosis cards Flashcards

Question

if left untreated what can a pasturella infection cause

Answer 1

osteomyelitis

Answer 2

autosomal dominant involving demyelination and damage of the peripheral nerves. usually results in diminished strength and proprioceptive/vibratory senses. it most commonly involves the peroneal nerves and may feature pes cavus

Answer 3

GI disturbance, QT, rash, prolongation, eosinophilia, rash

Answer 4

stridor and a barking cough. this is a pediatric infection caused by parainfluenza or influenza.

Answer 5

corticosteroids

Answer 6

``` the Westley croup severity score <3 mild croup 3-7 moderate 8-11 severe >11 impending respiratory failure ```

Answer 7

level of consciousness, cyanosis, stridor, air entry, and retractions

Answer 8

vertebral, anal atresia, cardiac deficits, tracheoesphageal atresia, renal and radial anomalies, limb defects.

Answer 9

this is narrowing of the trachea on frontal Xray indicative of croup and tracheal inflammation

Answer 10

oral amoxicillin.

Answer 11

thick and foul smelling bowel movements, hyperinflated lungs, predisposition to infections. decreased height and weight. defect in chloride channels.

Answer 12

infants are highly subject to vitamin K deficiency | as well as vitamin D

Answer 13

The one-minute APGAR score helps describe how the baby tolerated labor. The five-minute APGAR score helps describe how thef baby tolerates life outside the womb, stimulation, and resuscitation.

Answer 14

prophylaxis is erythromycin ointment and treatment is ceftrixone.

Answer 15

1 and 2 years.

Answer 16

9, 18, 30 months

Answer 17

18 and 24 months

Answer 18

maternal depression screening

Answer 19

100-120kcal/kg/day

Answer 20

115-130kcal/kg/day

Answer 21

150kcal/kg/day

Answer 22

high-pitched cry, jaundice, hypotonia, seizures, poor suck, lethargy.

Answer 23

arched back, extensor flexion,

Answer 24

pathological term used to describe staining of the basal ganglia and cranial nerve nuclei by bilirubin. "Kernicterus" also describes the chronic clinical condition that results from the toxic effects of high levels of unconjugated bilirubin. abnormalities in tone and reflexes, choreoathetosis, tremor, oculomotor paralysis, sensorineural hearing loss and cognitive impairment.

Answer 25

hemolysis from Rh incompatibility.

Answer 26

3-4 times a day

Answer 27

6-8 times a day

Answer 28

meconium should no longer appear by day 3. the stools will be 3-4 times and often occur with feedings

Answer 29

Pre-discharge total serum bilirubin (TSB) or transcutaneous bilirubin (TcB) level in the high-risk zone Jaundice observed in the first 24 hours of life Blood group incompatibility, with positive direct antiglobulin test Gestational age 35–36 weeks Previous sibling received phototherapy Cephalohematoma or significant bruising Exclusive breastfeeding, particularly if nursing is not going well and weight loss is excessive East Asian

Answer 30

``` Pre-discharge TSB or TcB level in the high intermediate-risk zone Gestational age 37–38 weeks Jaundice observed before discharge Previous sibling with jaundice Macrosomic infant of a diabetic mother Maternal age > 25 y Male gender ```

Answer 31

``` TSB or TcB level in the low-risk zone Gestational age 41 week Exclusive formula feeding Black Discharge from hospital after 72 hours ```

Answer 32

rooting, increased alertness, increased physical activity, mouthing

Answer 33

as a level of consciousness characterized by the failure of a child to recognize parents or to interact with persons or objects in the environment.

Answer 34

A listless child shows no interest in what is happening around herself.

Answer 35

A toxic condition is characterized by an appearance of pending physiologic collapse such as may be seen in sepsis, poisoning, acute metabolic crises, or shock. The child may be febrile, pale or cyanotic, with depressed mental awareness or extremely irritable and may demonstrate tachycardia, tachypnea and prolonged capillary refill.

Answer 36

A distressed child may have the appearance of working hard to maintain physiologic stability such as grunting, rapid breathing in order to maintain adequate oxygenation and ventilation.

Answer 37

``` Feeding problems Decreased activity Constipation Prolonged jaundice Skin mottling Umbilical hernia ```

Answer 38

Decreased feeding and activity are common in infants with CAH. Salt-losing CAH presents with lethargy, vomiting, and dehydration that can progress to shock.

Answer 39

skeletal disorders, chromosomal abnormalities such as Down syndrome, hypothyroidism, hydrocephalus, malnutrition.

Answer 40

microcephaly, craniosynostosis, hyperthyroidism, normal variant

Answer 41

dehydration

Answer 42

meningitis, hydrocephalus, subdural hematoma, lead poisoning

Answer 43

for inborn errors in metabolism

Answer 44

around the second week of life because the mothers thyroid hormone is somewhat protective

Answer 45

``` feeding problems Decreased activity Constipation Prolonged jaundice Skin mottling Umbilical hernia ``` myxedematous facies

Answer 46

one of the most common causes of intellectual disability

Answer 47

within 14 days

Answer 48

viral illness

Answer 49

``` Urinary tract infection (UTI) - most common Meningitis Sepsis/Bacteremia Pneumonia Bacterial gastroenteritis Osteomyelitis Septic arthritis ```

Answer 50

Kernig's sign is resistance to extension of the knee.

Answer 51

Brudzinski's sign is flexion of the hip and knee in response to flexion of the neck by the examiner.

Answer 52

ceftriaxone

Answer 53

Flat, circumscribed discoloration < 1 cm (> 1 cm is a patch)

Answer 54

Elevated, circumscribed solid lesion < 1 cm

Answer 55

May cause upper respiratory tract infection, pharyngitis, conjunctivitis, tonsillitis, or otitis media Potential for more severe infections in immunocompromised hosts

Answer 56

``` Fever for at least 5 days Cervical adenopathy Nonpurulent conjunctivitis Nonspecific ("polymorphic") rash Swelling and erythema of extremities Mucosal inflammation ```

Answer 57

After a prodrome of fever (over 38.3 C, or 101 F), cough, coryza, and conjunctivitis, this maculopapular rash starts on the neck, behind the ears, and along the hairline. The rash spreads downward, reaching the feet in two or three days. The initial rash appears on the buccal mucosa as red lesions with bluish white spots in the center (known as Koplik spots). These have frequently disappeared by the time the patient presents to medical attention. Immunization is very effective in preventing this infection.

Answer 58

group A strep Fever A diffuse, erythematous, finely papular rash (described as having a "sandpaper" texture) is pathognomonic Rash often begins at neck, axillae, and groin and then spreads over trunk and extremities, typically resolving within four or five days

Answer 59

Chicken pox:" rash starts on trunk and spreads to extremities and head Each lesion starts as an erythematous macule, then forms a papule followed by a vesicle before crusting over Lesions at various stages of development are seen in the same area of the body Immunization is effective in preventing this infection

Answer 60

infection with Parvo virus B19 Also called 5th disease Low grade fever followed by a rash, which starts as a facial erythema to the face ("slapped cheek" appearance), which can spread to the trunk and extremities and appears lacy Can lead to pain and swelling of the extremities, as well as development of transient pure red cell aplasia which can lead to severe anemia in patients with underlying hemolytic disease.

Answer 61

"Exanthem subitum." Also called sixth disease Erythematous macules start on trunk and spread to arms and neck (less commonly face and legs) Rash is typically preceded by 3 to 4 days of high fevers, which end as the rash appears Usually occurs in children under age 2 years human herpes VI

Answer 62

Streptococcal pharyngitis Kawasaki disease Toxic shock syndrome

Answer 63

panvasculitis of unknown etiology. autoimmune response to unknown trigger. seasonal patterns of outbreaks, more likely to occur in Asians.

Answer 64

Acute phase: onset through ~10 days. Fever and clinical findings are present, with serologic evidence of systemic inflammation (elevated acute phase reactants).

Answer 65

Subacute phase: 10 days through ~3 weeks. Fever resolves and clinical findings largely subside (often with peeling of hands and feet). Serologic evidence of inflammation continues.

Answer 66

Convalescent phase: 3 weeks through 6-8 weeks. All clinical findings have resolved. Continued serologic evidence of inflammation.

Answer 67

coronary artery aneurysms

Answer 68

high dose aspirin or IVIG

Answer 69

viral URI, asthma, foreign body, gerd

Answer 70

greater than 4 week s

Answer 71

strep pneumonae and haemophilus influenza | also morexella catarrhalis

Answer 72

influezna, RSV and rhinovirus

Answer 73

amoxicillin.

Answer 74

when it is concurrent or H flu is suspected.

Answer 75

headache, recurrent vomiting, inappropriate slowing of the heart rate (bradycardia), hypertension, irregular respirations, restlessness, increased drowsiness, abducens nerve palsy, abnormal pupillary responses, fixed dilated pupils.

Answer 76

cerebral edema. this is rare, but the morbidity and mortality is high

Answer 77

young age, high BUN at presentation, profound acidosis and with hypocapnia, administration of bicarbonate.

Answer 78

distinctive deep rapid breathing where the patient is trying to blow off excess CO2.

Answer 79

aggressive fluid resuscitation. this will dilute the glucose, but insulin is the only thing that will correct the metabolic acidosis by suppressing lipolysis and keto genesis. need baseline CBC to see if an infection is the inciting factor. serum osmolality and urinalysis are necessary.

Answer 80

usually hyponatremia. this is due to the osmotic effects of glucose causing water to come out of the cell.

Answer 81

Higher surface area:body mass ratio; hence, a greater relative area for evaporation to occur Higher basal metabolic rates than adults, which generates heat and expends water, and Higher percentage of body weight that is water (in infants, 70% of body weight is water; in children, 65%; and in adults, 60%).

Answer 82

staph aureus

Answer 83

group B strep

Answer 84

Group A and Streptococcus pneumoniae

Answer 85

Haemophilus influenzae type b

Answer 86

Neisseria gonorrhea

Answer 87

Kingella kingae

Answer 88

generalized tonic clonic

Answer 89

With this type, there are often motor signs in a single extremity or on one side of the body. However, focal onset seizure activity may spread to become generalized, making it difficult to distinguish from a generalized seizure.

Answer 90

This type of seizure can occur at any age. Altered level of consciousness is one of the hallmark features. Complex partial seizures may include blank stare, lip-smacking, drooling, gurgling, as well as nausea and vomiting. Automatisms are quasi-purposeful motor or verbal behaviors that are repeated inappropriately and commonly accompany complex partial seizures. Complex partial seizures often last 30 seconds to 2 minutes and are associated with a post-ictal phase of confusion, sleep, or headache. Secondary generalization can occur in up to one third of children, so it is important to question witnesses about initial features to help differentiate a complex partial seizure from a generalized seizure

Answer 91

because of the risk of brain herniation f

Answer 92

doxycycline

Answer 93

ceftriaxone

Answer 94

ceftriaxone one time dose and doxycycline for 14 days ceftriaxone and azithromycin ceftriaxone, doxycycline and metronidazole.

Answer 95

Pregnancy Previous noncompliance High fever Intractable vomiting Inability to exclude a surgical emergency Inadequate response on oral therapy within 72 hours Tubo-ovarian abscess

Answer 96

Miosis and blurred vision Increased gastric motility (nausea, vomiting, diarrhea) Excessive tearing, salivation, sweating and urination Bronchorrhea and bronchospasm Muscle twitching and weakness Bradycardia Seizures and coma Mnemonic: "SLUDGE" (salivation, lacrimation, urination, defecation, GI motility, emesis)

Answer 97

``` Mydriasis (dilated pupils) "blind as a bat" Dry skin "dry as a bone" Red skin (flushed) "red as a beet" Fever "hot as Hades" Delirium and seizures "mad as a hatter" Tachycardia Urinary retention Ileus ```

Answer 98

``` Blurred vision (miosis or mydriasis) Hypotension Apnea and bradycardia Hypothermia Sedation, confusion, delirium, coma ```

Answer 99

``` Miosis (constricted pupils) Respiratory depression Bradycardia and hypotension Hypothermia Depressed mental status (sedation, confusion, coma) ```

Answer 100

Mydriasis Fever and diaphoresis Tachycardia Agitation and seizures

Answer 101

hypoxia, hypoglycemia, shock

Answer 102

heart rate

Answer 103

a physiological state in which there is inadequate delivery of substrates and oxygen to meet the metabolic demands of the tissues.

Answer 104

elevated heart and respiratory rates, peripheral blood vessel constriction (causing cool, clammy extremities and delayed capillary refill time), and decreased peripheral pulses (due to vasoconstriction and decreased stroke volume).

Answer 105

ciprofloxacin | alternatives are rifampin, azithromycin and ceftriaxone

Answer 106

asthma, allergies, eczema.

Answer 107

staph or strep pyogenes.

Answer 108

topical antibiotics such as mupirocin. sometimes, due to the serious complications such as abscess formation, systemic antibiotics are required.

Answer 109

an infant is < 5th percentile for weight an infant is < 5th percentile in weight for length, or the rate of growth results in the infant crossing more than two major lines on the standard infant growth curve.

Answer 110

``` Congenital heart defects Cystic fibrosis Gastroesophageal reflux Neurologic disorders Metabolic disease Genetic abnormalities ```

Answer 111

most are psychosocial

Answer 112

the infant trying to feed more frequently and continually acting hungry.

Answer 113

1 cm below the costal margin

Answer 114

``` Congestive heart failure Hepatitis Congenital infections Inborn errors of metabolism Anemias Tumors (less commonly) ```

Answer 115

``` dyspnea with feedings diaphoresis poor growth an active precordium hepatomegaly ```

Answer 116

Atrial septal defect and bicuspid aortic valve

Answer 117

VSD Severe aortic stenosis Coarctation of the aorta Large patent ductus arteriosus

Answer 118

trouble feeding and respiratory symptoms

Answer 119

because they have increased pulmonary vascular resistance. this makes the systemic and pulmonary vascular resistances the same and there is a failure to shunt thus the murmur is null

Answer 120

furosemide, digoxin, enalapril

Answer 121

circulatory insufficiency or collapse.

Answer 122

Infection (e.g. Epstein-Barr virus, cytomegalovirus, bacterial sepsis, endocarditis) is the most common cause of splenomegaly in children. Other causes include hemolysis (sickle cell disease), malignancy (leukemia, lymphoma), storage diseases (e.g. Gaucher disease), systemic inflammatory diseases (e.g. systemic lupus erythematosus, juvenile idiopathic arthritis), and congestion (a complication of portal hypertension).

Answer 123

``` Trauma Septic arthritis Transient synovitis Reactive arthritis (e.g., post-streptococcal arthritis) Lyme disease Rheumatic fever Juvenile idiopathic arthritis Systemic lupus erythematosus Henoch-Schönlein Purpura ```

Answer 124

(also known as anaphylactoid purpura) is a self-limited, IgA-mediated, small vessel vasculitis that typically involves the skin, GI tract, joints, and kidneys. most commonly diagnosed form of vasculitis in children (about 50% of cases). Boys are more affected than girls.

Answer 125

hallmark of HSP is non-thrombocytopenic purpura

Answer 126

About 5% of children with HSP progress to chronic renal failure. Fewer than 1% will develop end-stage renal disease. 50% of the patients with HSP will also develop GI symptoms, such as GI bleeding 5-10% develop intussusception.

Answer 127

ITP has low platelet

Answer 128

goes away in a month without treatment

Answer 129

minimal change disease.

Answer 130

brief resolved unexplained event. characterized by cyanosis or pallor absent, decreased, or irregular breathing marked change in tone (hyper- or hypotonia) altered level of responsiveness.

Answer 131

SCD is a group of disorders characterized by substitution of valine for glutamic acid at the sixth amino acid position of the hemoglobin molecule. This mutation leads to the formation of polymers of hemoglobin when the hemoglobin becomes deoxygenated. These polymers lead to deformation of the red blood cell into the characteristic "sickle" cells. Sickle cells have increased adherence and block blood flow in the microvasculature, which leads to local tissue hypoxia, pain, and tissue damage.

Answer 132

tonsillectomy and cholecystectomy.

Answer 133

because of all the hemolysis which leads to accumulation of bilirubin gallstones

Answer 134

Lymphoidal-tissue hypertrophy involving Waldeyer's ring is common in children with sickle cell disease. Excessive snoring may be observed, as well as obstructive sleep apnea. Some physicians think tonsillar hypertrophy may relate to desaturation of hemoglobin and increase the risk of sickling. Tonsillectomy with adenoidectomy will improve the obstructive apnea for most patients.

Answer 135

to prevent infections that lead to sepsis. they have autosplenectomy and are at risk for encapsulated infections

Answer 136

hydroxyurea

Answer 137

jaundice, anemia, stroke. pneumonia and lung issues are also common.

Answer 138

haemophilus and pneumococcal

Answer 139

autosomal recessive

Answer 140

impairment in growth in sickle cell is common

Answer 141

``` Chronic anemia Poor nutrition Painful crises Endocrine dysfunction Poor pulmonary function ```

Answer 142

between 6 and 9

Answer 143

yes. fever is a medical emergency in a sickle cell patient

Answer 144

fever, splenic enlargement, slurred speech, chest pain, rapid breathing, increased pallor, jaundice, priapism

Answer 145

Upslanting palpebral fissures Small ears (usually less than 34 mm at maximum dimension in a term infant) Flattened midface Epicanthal folds Redundant skin on back of neck (nuchal skin) Hypotonia (most consistent finding in infants with Down syndrome) small brachycephalic head, Brushfield spots, small shaped mouth, single transverse palmar crease, short fifth finger with clinodactyly, and wide spacing and a deep plantar groove between the first and second toes. The degree of cognitive impairment is variable and may be mild (IQ of 50–70), moderate (IQ of 35–50), or occasionally severe (IQ of 20–35).

Answer 146

congenital hearing loss, congenital cataracts, heart disease, gastrointestinal atresia, hip displasia and hypothyroidism

Answer 147

Annual thyroid screening (due to an increased incidence of hypothyroidism, even if not present at birth) Vision screening (due to increased incidence of vision problems) Hearing screening (due to increased incidence of hearing problems) Complete blood count in first month to assess for leukemoid reactions, or transient myeloproliferative disorders (TMD) Referral to a pediatric cardiologist (due to the increased incidence [50%] of structural heart disease in patients with Down syndrome and the difficulty of auscultating some of these cardiac defects) Beginning at 1 year of age, and then annually, a hemoglobin and hematocrit should be obtained to screen for iron deficiency anemia (due to increased risk for iron deficiency due to lower dietary iron intake than their peers) Referral for evaluation by early intervention is key to a child with Down syndrome receiving any needed therapies as early as possible.

Answer 148

Webbed neck Low ear placement Edema of the hands and feet Hyperconvex nails, and "Shield" chest, with widely spaced nipples Coarctation of the aorta is found in about 20% of affected girls. Short stature is common, and some girls are not diagnosed until early adolescence when they present with short stature and delayed sexual maturation (due to gonadal dysgenesis). Most have a normal IQ.

Answer 149

Gross motor Fine motor Communication (sometimes broken down into receptive and expressive language) Personal-social Problem-solving (also called self-help, cognitive, or adaptive in some frameworks)

Answer 150

9, 18, 24, 30 months

Answer 151

spastic quadriplegia, spastic diplegia, dyskinetic cerebral palsy, hemiplegia, ataxic cerebral palsy

Answer 152

usually legs greater than arms, periventricular white matter abnormality, found in premature infants.

Answer 153

basal ganglia, cerebellum, thalamic abnormalities. perinatal ataxia, kernicterus

Answer 154

arm and leg on one side; unilateral UMN, stroke

Answer 155

affects the entire body, cerebellar abnormalities, cerebellar hypoplasia, pontocerebellar hypoplasia.

Answer 156

prematurity by far the most common. intrauterine growth retardation, chorioaminitis, perinatal ataxia

Answer 157

Weight falls below the 3rd percentile Weight for height/length falls below the 3rd percentile The rate of weight gain slows compared with previous growth, crossing two or more major percentiles on the growth chart in a downward direction.

Answer 158

insufficiency of caloric intake Insufficient intake Excessive losses Excessive caloric need.

Answer 159

functional abdominal pain.

Answer 160

chronic recurrent abdominal pain that is nonspecific and non-life threatening. considered a functional disconnect between the gasterenteric nervous system and the mental nervous system.

Answer 161

Involuntary weight loss Deceleration of linear growth Gastrointestinal blood loss Significant vomiting Chronic severe diarrhea Persistent right upper or right lower quadrant pain Unexplained fever Family history of inflammatory bowel disease Abnormal or unexplained physical findings

Answer 162

basal ganglia, cranial nerve nuclei, subthalamic nuclei.

Answer 163

hypotonia, somnolence, and loss of primitive reflexes.

Answer 164

from the breakdown of red blood cells/aging red blood cells

Answer 165

Minor sources come from ineffective erythropoiesis and breakdown of hemoproteins including muscle myoglobin and liver enzymes.

Answer 166

cough, pharyngitis and fever

Answer 167

Hutchinson teeth, sensorineural hearing loss, saddle nose, saber shins. this can result in hydros fetalis, hepatitis and blindness.

Answer 168

penicillin G

Answer 169

periventricular calcifications, petechial rash, hepatosplenomegaly, jaundice, microcephaly, retinitis

Answer 170

Appearance: 2 - Pink, 1 - Acrocyanosis (Blueish extremities), 0 - Totally Blue Pulse: 2 - >100/min, 1 - <100/min, 0 - None Grimace: 2 - Crying, 1 - Whimpering, 0 - Silent Activity: 2 - Active muscle tone and movement, 1 - Moderate, 0 - Limp Respiratory: 2 - Regular breathing, 1 - Irregular breathing, 0 - No breathing

Answer 171

Gon is purulent; chlamydial is watery non purulent. treatment for gon is erythromycin treatment for chlamydial is azithromycin to mother before birth or oral erythromycin to prevent pneumonia in baby

Answer 172

this is the second most common genetic trisomy. | prominent occiput, micrognathia, flexed fingers with overlap, congenital heart defects.

Answer 173

the triad of cleft lip or palate, mircoophthalmia, polydactyly

Answer 174

meconium ileus, pancreatic insufficiency, diarrhea and malabsorption, vitamin D deficiency and rickets

Answer 175

hyperpnea, tachycardia, chest wall retractions, expiratpory grunting, nasal flaring, and cyanosis

Answer 176

commonly caused by 21-hydroxylase deficiency. affected female infants have ambiguous genitalia (males do not), hyperkalemia, hyponatremia, hypoglycemia, and shock within days of birth. similar to Addisons

Answer 177

hypotonia, poor feeding, lethargy, and lactic acidosis or E1 deficiency. treatment is increasing the ketogenic nutrient intake. causes elevated alanine and lactic acidosis

Answer 178

leucine and lysine

Answer 179

patchy hair loss with short split ends.

Answer 180

mcirosporum canis

Answer 181

not until 9-12 months | because there is not sufficient nutrients to supplement

Answer 182

oppositional defiant disorder, tourettes, depression

Answer 183

common cause of stridor in children characterized by biphasic stridor without signs of infection.

Answer 184

4 types of monozygotic twin pregnancies (depending on when division happens): 1. Dichorionic, Diamniotic - 0-3 days after fertilization 2. Monochorionic, Diamniotic - 4-8 days after fertilization 3. Monochorionic, Monoamniotic - 9-12 days after fertilization 4. Conjoined twins - 13+ days after fertilization

Answer 185

penicillin

Answer 186

enterobius vermicularis

Answer 187

triad of patchy skin pigmentation, bone abnormalities and endocrine/hormonal abnormalities. Skin findings include large cafe-au-lait spots with characteristic jagged borders. spontaneous mutation in the GNAS1 gene

Answer 188

auses encephalitis characterized by symptoms, such as headaches, to more severe symptoms like seizures.

Answer 189

pasteurella multocida penicillin FYI this is not the same bacteria that causes cat-scratch fever

Answer 190

bartonella henselae

Answer 191

fifths disease/erythema infectiosum | parvovirus

Answer 192

check the serum glucose and thyroid panel as celiac is autoimmune in nature and could have other autoimmune diseases

Answer 193

beta blockers and methimazole

Answer 194

dubin-jonsons and rotor

Answer 195

the biliary system cannot visualized in Dubin-johnsons

Answer 196

Gilbert and Crigler-Najjar

Answer 197

ASDs, VSDs, teratology of fallot, aberrant great vessels.

Answer 198

later onset 1-6 weeks of life.

Answer 199

usually an indirect hyperbilirubinemia

Answer 200

morbilliform rash between 7-14 days after receiving the shot. This reaction is well-described, and is not painful, itchy, or contagious to others. The rash is not a contraindication to receiving future doses of the measles or other vaccines

Answer 201

3-4 day prodrome of fever, cough, coryza and conjunctivitis. This is followed by 1-2 day presentation of Koplik spots and a 6 day presentation of a red, maculopapular rash that spreads from the forehead→ face→ torso→extremities and disappears in the same order.

Answer 202

subacute sclerosing pan encephalitis

Answer 203

They are contraindicated in babies under the age of 6 months.

Answer 204

Wilms tumor, aniridia, genitourinary abnormalities (cryptorchidism/hypospadius), and retardation (cognitive impairment) need a renal ultrasound for Wilms tumor

Answer 205

characterized by macroglossia, gigantism, hyperinsulinemia and hypoglycemia. macroglossia, large visceral organs, and a history of omphalocele or other abdominal wall defects.

Answer 206

About 10% of patients develop tumors such as Wilms' tumor, rhabdomyosarcoma, or hepatoblastoma.

Answer 207

reassure the parents. they can monitor the child at home

Answer 208

inhibin a normal AFP low estradiol low hCG low

Answer 209

α-fetoprotein and estriol concentrations are low, while human chorionic gonadotropin and inhibin A concentrations are high.

Answer 210

not considered a reliable screening method for this condition.

Answer 211

prostaglandin E1 to maintain the ductus and balloon angioplasty to fix the coarctation

Answer 212

viral illness weeks before

Answer 213

autosomal recessive platelet dysfunction disorder, that causes a deficiency of glycoprotein Ib (GpIb). Patients with BSS often present with recurrent epistaxis, menorrhagia, easy bruising, purpura, and petechiae.

Answer 214

it is concentric and diastolic

Answer 215

autosomal dominant disease often leading to sudden cardiac death, especially in young athletes. The myocardium becomes concentrically hypertrophied with sarcomeres in parallel which causes a thickened and stiff ventricle. These ventricles can no longer fill properly, leading to diastolic heart failure. A lack of cardiac output results in syncopal episodes, and outflow obstruction also occurs as the myocardial septum thickens and blocks flow of blood through the mitral valve.

Answer 216

``` MUDPILES methanol uremia diarrhea polyethylene glycol isoniazid/iroin lactic acidosis ethanol sepsis and salicylates ```

Answer 217

hydrogen excretion in the breath after lactose administration, in the context of clinical symptoms. Bacteria typically break down the undigested lactose into carbon dioxide and hydrogen. A lactose breath hydrogen test is more specific than acidic stool pH.

Answer 218

the knees, elbows and the buttocks

Answer 219

skin biopsy

Answer 220

like flesh colored bumps that are itchy. pompholyx-like" refers to a vesicular pattern of eczema that appears on the hands or feet.

Answer 221

magnesium sulfate | can cause hypotonia in the baby. this is self-limited

Answer 222

causes hypertonia and irritability

Answer 223

autosomal recessive disorder of methionine metabolism that leads to the buildup of homocysteine and its metabolites in the blood and urine. Homocysteine is a key metabolite in trans-sulfuration that leads to the synthesis of proteins.

Answer 224

increased B6 in the diet

Answer 225

intellectual disability, psychiatric disorder, seizures.

Answer 226

paucity of interlobular bile ducts. This paucity leads to chronic cholestasis in affected children. Commonly seen clinical manifestations are posterior embryotoxon (ring-like opacity around the cornea), butterfly vertebrae and triangular faces as seen in the case above. Also observed are cardiac abnormalities (usually pulmonic stenosis), short stature, renal disease and developmental delay.

Answer 227

The treatment of choice for corneal abrasions in children is erythromycin ointment, and no pressure patch is needed.

Answer 228

pleurodesis | either mechanical or chemical

Answer 229

strep pyogenes infection.

Answer 230

long term penicillin treatment

Answer 231

decreased due to the lack of pulsatile GnRH

Answer 232

they become overstimulated

Answer 233

dysentery | caused by shigella dysenteriae

Answer 234

a rare, X-linked genetic postnatal disorder of grey matter and is characterized by early normal growth and development, followed by a loss of milestones. It affects girls exclusively because males die in utero. Clinical features include small hands and feet and a decrease rate of head growth.

Answer 235

the buttocks in a circular restricted pattern

Answer 236

refractory celiac disease have an elevated risk of developing small bowel T-cell lymphoma, which is a non-Hodgkin lymphoma that affects T cells.

Answer 237

thymic hypoplasia, recurrent infections, ataxia, hypocalcemia

Answer 238

autosomal recessive metabolic disorder caused by a deficiency in galactokinase, the first enzyme involved in galactose metabolism. Deficiencies often leads to the accumulation of galactose and galactitol, which typically presents as cataracts in early infancy due to the accumulation of galactitol in the lens.

Answer 239

yes. they are a common cause of meningitis. they do not cause muscle paralysis, however, and that is one way to distinguish them from poliovirus

Answer 240

presents with gastric symptoms of diarrhea, vomiting, and melena due to gastric ulceration. The metabolic acidosis that results several hours after ingestion causes tissue damage, particularly of the liver and brain.

Answer 241

is an autosomal-dominant condition caused by a mutation in a keratin gene characterized by malformed nails, plantar and palmar keratoderma, oral leukoplakia, and other skin problems due to hyperkeratosis.

Answer 242

tuberous sclerosis

Answer 243

Tuberous sclerosis is an autosomal dominant phakomatosis disorder characterized by varied clinical manifestations, such as seizures, mental retardation, skin lesions, and multiple hamartomas affecting multiple organ systems (i.e., heart, brain, and kidney)

Answer 244

at the age of 11-12

Answer 245

is the most common cause of prepubertal short stature and pubertal delay. The parents of these parents will often describe themselves as “late bloomers.” this causes late puberty asa well, or below average testicular enlargement by 13 years old.

Answer 246

hepatoblastoma

Answer 247

haracterized by palpable abdominal mass, fever, nausea, vomiting, hematuria, and hypertension. Histologic examination shows a tumor arising from the fetal kidney and is characterized by blastemal, stromal, and epithelial cells (triphasic tumor).

Answer 248

to assess kidney function and metabolic function with a BMP

Answer 249

C3 complement deficiency is characterized by recurrent purulent infections and increased risk of type III hypersensitivity reactions.

Answer 250

blanching maculopapular rash beginning on the wrists/ankles, later spreading to the extremities and the trunk. Involvement of palms and soles is pathognomonic. RMSF is treated with doxycycline within 4 days of disease onset.

Answer 251

tits, pits, mits, lips. | thelarche, pubarche, growth spurt, menarche

Answer 252

incomplete fusion of the hillock of his | this is of the first and second brachial arch

Answer 253

caused by chlamydia trachomatis infections and is the leading cause of preventable blindness. untreated or retreated infections lead to scarring of the eye lids and cause the eye lashes to turn inwards and development of the corneal ulcers

Answer 254

an autosomal recessive disorder due to a lack of galactose-1-phosphate uridyl transferase. Avoid breastfeeding in children with galactosemia because lactose in the milk contains galactose and glucose monosaccharides. avoid lactose and galactose

Answer 255

because lactose is molecule consisting of glucose and galactose

Answer 256

this is dermatitis, protuberant abdomen, edema because of a lack of albumin production

Answer 257

an acute focal infection caused by Staphylococcus aureus involving either the glands of Zeis (external hordeola or styes) or the meibomian glands (internal hordeola). Hordeola essentially represent focal abscesses with symptoms of acute inflammation, such as a painful and swollen red lump on the eyelid. The eyelid lump can sometimes cause a corneal astigmatism, which results in blurry vision Medical therapy for hordeola includes eyelid hygiene, warm compresses and massages of the lesions, and topical antibiotic ointment in the inferior fornix if the lesion is draining. Oral doxycycline may also be added if there is a history of multiple or recurrent lesions.

Answer 258

frequent laughter, hand flapping, seizures, ataxia, and developmental delay.

Answer 259

fever, weight loss, cervical lymphadenopathy, and lower respiratory tract symptoms.

Answer 260

The first step in differentiating this from other respiratory diseases is a tuberculin skin test.

Answer 261

lacy macular rash

Answer 262

fever, runny nose, diarrhea and then a rash several days later watch for the macular lacy rash. also cheeks are red! SLAPPED CHEEK

Answer 263

endemic to the Ohio, Missouri and Mississippi River valleys in the United States. Transmission occurs through contamination of soil with bat droppings, and infection has been asspciated with construction and renovation activities that disrupt the contaminated soil.

Answer 264

is a respiratory infection caused by Coccidioides, a soil fungus that is endemic to Southwestern United States. While infection is often asymptomatic, in some patients it can cause pneumonia with hemoptysis and cavitary lesions

Answer 265

include ketoconazole, fluconazole, and itraconazole.

Answer 266

a 3rd generation cephalosporin such as ceftriaxone.Empirical therapy of community-acquired suspected bacterial meningitis in children and adults should include a combination of dexamethasone, a third- or fourth-generation cephalosporin, and vancomycin.

Answer 267

an obstruction in the urinary tract of a newborn male, most likely due to defective development. It often presents with difficulty in urination, and urinary tract infections.

Answer 268

A voiding cystourethrogram is a highly specific imaging test conducted to diagnose PUV.

Answer 269

POTTER syndromePulmonary hypoplasia, oligohydramnios (trigger),twisted face, twisted skin, extremities defects, and renal failure (in utero).

Answer 270

cause pica (cravings for non-nutritious foods like ice, hair, and dirt) and trichotillomania.

Answer 271

common symptoms include blue sclera, dental imperfections, and hearing loss.

Answer 272

an inherited defect in type IV collagen, resulting in glomeruloneuphritis, end-stage kidney disease, and hearing loss. Blood in urine is a classic sign, and symptoms often appear in young adults, not newborns.

Answer 273

which is a benign skin lesion that grows rapidly in the first few months, and will likely involute before the child reaches nine years of age.

Answer 274

ulceration

Answer 275

The carditis often affects the mitral, aortic, and tricuspid valves (in order of most common to least common) - all of which are exposed to relatively high-pressures, and it can lead to stenosis or regurgitation. The underlying mechanism is an immune mediated type II hypersensitivity reaction, where molecular mimicry causes the immune cells to attack the heart valves.

Answer 276

the most common neonatal conjunctivitis and presents 5-14 days after birth. presents as eyelid edema, red and injected conjunctiva, watery or mucopurulent discharge. Can cause pneumonia in the new born.

Answer 277

less common cause of conjunctivitis and usually seen earlier within the first 5 days after birth with bilateral, profuse, purulent exudates and edematous eyelids. Gonococcal conjunctivitis has more potentially serious complications such as corneal ulceration, scarring and visual impairment and as such should be excluded.

Answer 278

diarrhea, bleeding, anemia, increased BUN and creatinine, proteinuria. it can cause acute renal failure, but not acute tubular necrosis.

Answer 279

sinusitis, bronchitis, otitis media, laryngitis.

Answer 280

aerobic, diplococcus, gram negative, oxidase positive, unable to metabolize maltose.

Answer 281

this is when the bladder is outside the abdominal wall, upon birth. immediately covering and irrigation with saline is treatment. surgical correction is the final. epispadius is associated.

Answer 282

most common cause of nephrotic syndrome in pediatric patients. It often presents after a viral illness and is treated with corticosteroids.

Answer 283

hypoglycemia, seiures, polycythemia, hypocalcemia, hypomagnesemia, cardiomegaly, renal vein thrombosis, prematurity, macrosomia, shoulder dystocia and intrauterine growth retardation.

Answer 284

Mothers are at risk for preeclampsia, gestational hypertension, polyhydramnios, and infection.

Answer 285

observance and supportive.

Answer 286

of a brownish-red papule at the site of inoculation after 7-12 days, followed by tender regional lymphadenopathy 1-4 weeks later. Immunocompetent patients may appear well otherwise or may have a persistent fever; other systemic signs such as malaise, sore throat and anorexia are less common. Painless conjunctivitis is the most common atypical presentation

Answer 287

bacillary angiomatosis. Immunocompromised patients may have much more extensive systemic disease including bacillary angiomatosis and peliosis.

Answer 288

amoxicillin

Answer 289

change from amoxicillin to amoxicillin/clavulanate

Answer 290

Huntington, fredrick ataxia, myotonic dystrophy, fragile X syndrome

Answer 291

most frequently presents in adolescence with scoliosis, neurological abnormalities, diabetes mellitus, and cardiomyopathy.

Answer 292

a potentially life-threatening airway condition characterized by respiratory distress, stridor, copious drooling, and pharyngitis that is now uncommon due to the high uptake of the Haemophilus influenza B vaccine. The priority of management is obtaining a secure airway.

Answer 293

cryptorchidism and inguinal hernias.

Answer 294

No. this is a contraindication

Answer 295

an autosomal recessive condition that typically results from a deficiency in phenylalanine hydroxylase, an enzyme responsible for converting the amino acid phenylalanine to tyrosine. Treatment often includes dietary restrictions such as the avoidance of aspartame and amino acid (protein) rich foods.

Answer 296

avoiding protein rich foods such as meats. because the patients are deficient in the enzyme that converts phenylalanine to tyrosine

Answer 297

oral or intramuscular dexamethasone

Answer 298

central apnea

Answer 299

most commonly caused by Streptococcus pneumoniae, Moraxella catarrhalis, or Haemophilus influenzae (non-typeable strain)

Answer 300

dactylitis | severe pain the hands and feet

Answer 301

clinical suspcion, passing a nasal catheter and CT scan.

Answer 302

Streptococcal pharyngitis is caused by group A Streptococcus, which is gram-positive, beta-hemolytic, and bacitracin-sensitive. It is characterized by an exudative pharyngitis.

Answer 303

this is due to inadequate intake. this causes the decreased motion of the bowel and increased reuptake of the bilirubin from the stool, thus there will be more bilirubin from the enterohepatic circulation.

Answer 304

of course, because it doesnt cover all the genotypes.

Answer 305

leukocoria is caused by congenital cataract and retinoblastoma

Answer 306

anosmia, hypogonadotrophic hypogonadism, failure to begin or complete puberty

Answer 307

this is caused by coxsackie virus A16. is a common, mild, and short-lasting viral infection most often affecting children less than 5 years old. It is characterized by blisters on the hands and feet and in the mouth. Many children present with loss of appetite because they have oral ulcers that are very painfu

Answer 308

CaRS | Coxsackie A, Rickettsia/Rocky Mountain Spotted Fever, and Secondary Syphilis.

Answer 309

Acne vulgaris is most commonly associated with the Cutibacterium acnes (formerly Propionibacterium acnes).

Answer 310

neonatal lupus. | maternal antibodies can cross the placenta and cause neonatal lupus. the baby can even present with a rash

Answer 311

it reduces it. makes it acidic

Answer 312

phenobarbital this induces CYP450

Answer 313

uncomplicated nosebleeds includes upright + forward-leaning posture, application of pressure to the anterior of the nose, and potentially gauze wetted with decongestant spray. In repetitive epistaxis, clotting disorders like Von Willebrand Disease should be considered.

Answer 314

correct fluid loss with IV fluids, hyperglycemia with insulin, electrolyte disturbances and acid-base balance and treatment of concurrent infection if present. Fluid resuscitation is always the first best step in management of DKA.

Answer 315

primary ovarian failure | especially in the setting of primary amenorrhea

Answer 316

a genetic test should be performed for turners, vanishing testes syndrome, or absent testis determining factor

Answer 317

Dysfunctional ENaC --a sodium channel in the collecting tubules characterized by hypertension, hypokalemia, metabolic alkalosis. there is low serum renin, aldosterone, and low aldosterone urinary secretion.

Answer 318

this is the causal organism for cutaneous larva migrans

Answer 319

wuchereria bancrofti

Answer 320

reassurance, as there is a lot of variability during that time period for menstrual development.

Answer 321

ampicillin and cefotaxime

Answer 322

In acute leukemias, blast forms predominate - accounting for at least 20% of the total cellularity of a bone marrow biopsy sample.

Answer 323

Mumps is caused by viruses of the genus Paramyxoviridae and is characterized by conjunctivitis, parotitis, orchitis, and pancreatitis.

Answer 324

it is caused by a spread of infection from the ethmoid sinuses or a facial infection It is often characterized by a recent sinusitis or upper respiratory infection, with fever, eye pain and decreased vision. Proptosis and ophthalmoplegia are key findings that separate this condition from pre-septal (periorbital) cellulitis

Answer 325

IV antibiotics

Answer 326

Gower's sign is the hallmark sign of duchenne muscular dystrophy - the child will 'walk' his hands up his legs to push himself to a standing position. A dystrophin gene mutation causes muscle cell instability and breakdown.

Answer 327

an oral anti-fungal agent. Griseofulvin is first-line therapy, although terbinafine is also becoming first-line treatment and requires a shorter treatment course. Fluconazole and Itraconazole are alternatives.

Answer 328

Refeeding syndrome is one cause of hypophosphatemia, along with alcoholism, vitamin D deficiency, respiratory alkalosis, and hereditary diseases. Hypophosphatemia can cause muscle weakness, altered mental status, rhabdomyolysis, immunodeficiency, and osteomalacia

Answer 329

no fungiform papillae on the tongue, decreased deep tendon reflexes, lack of an axon flare following intradermal histamine, and no overflow tears with emotional crying. is a rare autosomal-recessive genetic disorder involving the IKBKAP gene. It results in autonomic and sensory neuropathies and can lead to decreased pain and temperature sensation.

Answer 330

Tocolytics (e.g. beta-2 agonists, calcium channel blockers) relax the uterus and are used in the treatment of preterm labor.

Answer 331

This patient's presentation with multiple ecchymotic patches, swollen gums, and a petechial rash is suggestive of platelet dysfunction disorder. Glanzmann's thrombasthenia is a rare type of platelet dysfunction disorder in which the platelets contain defective or low levels of glycoprotein IIb/IIIa (GpIIb/IIIa), which is a receptor for fibrinogen. As a result, no fibrinogen bridging of platelets to other platelets can occur, and the bleeding time is outstandingly prolonged.

Answer 332

congenital hypothyroidism

Answer 333

buttocks, back, shoulders

Answer 334

usually disappears in 3-5 years

Answer 335

ethosuximide

Answer 336

to the side affected by conductive hearing loss

Answer 337

bone conduction will be greater than air conduction on the affected side.

Answer 338

this is delayed type IV. | T cell mediated

Answer 339

PPD, poison ivy, allograft reaction and graft vs host

Answer 340

supplemental oxygen, ampicillin, and gentamicin.

Answer 341

yes. if surgery is performed before the age of one in unilateral, the fertility rate is close to 90%

Answer 342

hypotonia, lethargy, macroglossia, large fontanelles, and dry skin. protuberant abdomen dry, brittle hair and low hairline

Answer 343

temporal lobe abscess until proven otherwise

Answer 344

erythema or edema of palms and soles followed by membranous desquamation; generalized polymorphic rash that may be limited to groin or lower extremity; oropharyngeal changes (“strawberry tongue” and fissuring of lips); bilateral, nonexudative, painless bulbar conjunctivitis; cervical lymphadenopathy; and fever lasting for more than five days.

Answer 345

propanolol

Answer 346

joint hyperflexibility, bruising/ecchymoses, cigarette paper skin this is caused by collagen defect

Answer 347

this is a temporary normocytic anemia with low to normal reticulocyte count that occurs after a URI

Answer 348

NF1, fanconi anemia, li-freumeni, Klinefelter and down syndriome

Answer 349

FAT RN | fever, anemia, thrombocytopenia, neurological (psychiatric as well), renal failure

Answer 350

2 days - 2 weeks of life. A newborn's immature liver often can't remove bilirubin quickly enough, thus, an excess of unconjugated bilirubin remains in the bloodstream.

Answer 351

topical retinoids, benzoylperoxide, and an antibiotic (typically macrolide)

Answer 352

diffuse erythematous rash that desquamates as the patient recovers

Answer 353

infection with Staphylococcus aureus or Streptococcus pyogenes. Both form toxins that produce abrupt onset of local or diffuse pain, fever, confusion, vomiting, signs of soft tissue infection, and sometimes a diffuse erythema that desquamates as the patient recovers.

Answer 354

inability to produce glucocorticoid and androgen hormones, and a resultant increase in mineralocorticoid production. Patients with XY chromosomes are born with a female external appearance (with ambiguous genitalia) and an absence of internal female reproductive organs. hypokalemia, hypertension testes in the inguinal canal, female ambiguous genitalia

Answer 355

classic triad of chorioretinitis, hydrocephalus, and intracranial calcifications. Other manifestations include intrauterine growth restriction, premature birth, jaundice, hepatosplenomegaly, rash, microcephaly, and seizures.

Answer 356

jaundice, hepatosplenomegaly, thrombocytopenia, microcephaly, chorioretinitis, and intracranial calcifications; however, the most common sequela is sensorineural deafness,

Answer 357

cutaneous scarring (depressed and pigmented lesions in a dermatomal distribution), ophthalmic manifestations (cataracts, chorioretinits, microphthalmos, nystagmus), and limb hypoplasia. Affected neonates may also have cortical hypoplasia and seizures.

Answer 358

usually asymptomatic at birth, developing symptoms later. HSV infection may present with localized disease involving the characteristic vesicles but may also cause disseminated disease, which can progress rapidly to hypotension and shock.

Answer 359

"blueberry muffin" rash, hepatosplenomegaly, and thrombocytopenia. Sensorineural deafness, cataracts, and cardiac malformations are also common.

Answer 360

Folate deficiency can be diagnosed by elevated serum homocysteine.

Answer 361

B12 deficiency manifests as elevated levels of both homocysteine and methylmalonic acid.

Answer 362

the entire household because its highly contagious

Answer 363

permethrin

Answer 364

ventricular septal defect, cleft palate, facial anomalies, and (most concerning) spina bifida. Its potential for teratogenicity is why valproate is contraindicated in pregnancy.

Answer 365

congenital hyperinsulinemia from developing in a glucose rash environment

Answer 366

given immunoglobulin. Neonatal varicella can occur in infants born to mothers exposed to varicella-zoster virus within 2 weeks of delivery or exposed within 10 days of birth. this requires prophylaxis because neonatal varicella is high mortality. 30%

Answer 367

acute otitis media

Answer 368

acute otitis media, pneumonia, febrile seizures,

Answer 369

of patent ductus arteriosus on echocardiography, cataracts, hearing loss, and microcephaly

Answer 370

widespread formation of fluid filled blisters that are thin walled and easily ruptured and the patient can be positive for Nikolsky sign, where slight rubbing of the skin results in exfoliation of the skin's outermost layer.

Answer 371

human immunoglobulin and antitoxin

Answer 372

the most common selective immunoglobulin deficiency. IgA is the main immunoglobulin isotype mediating mucosal immunity thus patients will commonly experience recurrent sinopulmonary and gastrointestinal infections.

Answer 373

transfusion and phototherapy

Answer 374

high pitched cry, lethargy, hypotonia

Answer 375

mono amniotic, monochorionic

Answer 376

mono amniotic and dichorionic because the chorion develops first.

Answer 377

monoamniotic and monochorionic, diamnionic and monochorionic, and diamniotic and dichorionic.

Answer 378

Hypoglycemia results and can cause tremors, seizures, and diaphoresis.

Answer 379

aldolase B deficiency

Answer 380

MMR, Varicella + Zoster, Sabin polio, Influenza, Rotavirus and Yellow Fever.

Answer 381

a condition characterized by hair which is easily removed from the scalp. These hair follicles will still be in anagen phase. Typically, this condition is self limiting and improves with age. No treatment is generally warranted at this time.

Answer 382

the 3rd and 12th weeks of pregnancy. possible causes include chromosomal errors, gene mutations, parental infections, prenatal toxic exposures, structural blockage and metabolic disorders.

Answer 383

a rare phagocyte dysfunction disease cause by a defect of NADPH oxidase, which can be diagnosed using the nitroblue tetrazolium dye reduction test, as well as the dihydrorhodamine test.

Answer 384

Racemic epinephrine. Nebulized epinephrine will quickly reduce symptom severity and should be followed up by the administration of glucocorticoids (usually dexamethosone), which will provide longer lasting relief.

Answer 385

ependymoma and meningioma

Answer 386

they can develop a hypersensitivity reaction and an intensely pruritic, morbillum rash on the extensor surfaces

Answer 387

thyroid dysgenesis

Answer 388

ectopic thyroid;

Answer 389

lingual thyroid.

Answer 390

subdural hematoma

Answer 391

agalactiae

Answer 392

the combination of multinucleated globoid cells, optic atrophy, blindness, and motor delay. This condition is a sphingolipidosis disease caused by a deficiency of galactocerebrosidase.

Answer 393

supportive therapy with fluid replacement

Answer 394

burkholderia cepacia

Answer 395

pyruvate kinase deficiency

Answer 396

The catarrhal phase is similar to the common cold and manifests as a simple upper respiratory tract infection. This is likely the "very mild cold" that this patient experienced 6 weeks ago. The paroxysmal phase is characterized by "paroxysms" (or bouts) of coughing and lasts for up to 6 weeks. The name "whooping cough" comes from the rapid gasp for air observed in patients after a string of coughs, though this gasp is not always seen.

Answer 397

a rare, congenital, non-familial disorder that affects blood vessels - primarily capillaries. The hallmark feature of Sturge-Weber syndrome is facial capillary malformation, referred to as a port wine stain, which is usually visible at birth.

Answer 398

strep A infection which is pyogenesd

Answer 399

post-strep glomerulonephritis

Answer 400

The onset of Becker muscular dystrophy is around 15 years of age. Muscle biopsy shows degeneration, hypertrophic fibers, and replacement of muscle by fat and connective tissue.

Answer 401

physical findings of tall stature, epicanthal folds, hypotonia, and clinodactyly. Triple X syndrome is also associated with conditions such as seizures, renal abnormalities, premature ovarian failure, and learning disabilities.

Answer 402

IUGR, poly or oligo, clenched fists, rocker bottom feet

Answer 403

a harsh systolic murmur. heard at the left sternal border which decreases in intensity when moving from an upright to a supine position

Answer 404

autosomal dominant

Answer 405

anywhere, even in the artery

Answer 406

lacy reticular rash, well-demarcated at the wrists and feet (papular-pruritic glove and socks syndrome), aplastic anemia.

Answer 407

viremic highly contagious parvovirus B19

Answer 408

parvovirus B19 and no, they are no longer effective

Answer 409

chromosome 15 DNA helicase mutation. rash in sun-exposed areas

Answer 410

jaundice which either during the first day of life, has total bilirubin >12, direct bilirubin >2, or shows rate or rise of >5/day.

Answer 411

a lack of vision changes and pain with eye movement.

Answer 412

oral antibiotics

Answer 413

an emergency intravenous antibiotics and/or surgery.

Answer 414

erythromycin is a macrolide used as back up

Answer 415

Von Willebrand disease (vWD) is the most common bleeding disorder, affecting 1 in 100 individuals. In girls with no past bleeding history, vWD is often diagnosed around menarche.

Answer 416

a disease affecting the small vessels of the kidneys caused by prior infection of specific strains of group A β-hemolytic streptococcus. As with other types of glomerulonephritis, it is characterized by hematuria, proteinuria, intrinsic acute kidney injury, and hypertension. d

Answer 417

Mullerian agenesis (XX) and androgen insensitivity syndrome (XY); these are distinguished with serum testosterone tests and/or a karyotype.

Answer 418

a peroxisome biogenesis disorder which leads to the build up of very long chain fatty acids to toxic levels affecting multiple organ systems. ZS is characterized by high forehead, malformed earlobes, and flat looking face. Additionally seizures, hepatomegaly +/- jaundice, hydronephrosis, and hypotonia are key features.

Answer 419

by using a donor oocyte

Answer 420

Suspect ovarian failure in girls who have no breast development by age 12 years or who have not started menses by age 14 years. Elevated levels of luteinizing hormone (LH) and follicle stimulating hormone (FSH) confirm ovarian failure.

Answer 421

hypoxia, trauma, infection

Answer 422

a progressive neurodegenerative disease due to a deficiency of the enzyme hexosaminidase A. Prior to one-year-old, it is characterized by loss of developmental milestones, seizures, hypotonia, hyporeflexia, and macrocephaly.

Answer 423

severe respiratory distress within the first 24 hours of life and is associated with lung hypoplasia

Answer 424

polycythemia

Answer 425

smaller and more frequent meals, thickening the formula with rice cereal, elevating the head to 20° to 30° after feeds.

Answer 426

Down syndrome can be screened for by low α-fetoprotein and estriol concentrations. Brushfield spots are characteristic dysmorphic features on the iris of patients with Down syndrome.

Answer 427

coarctation and bicuspid aortic valve

Answer 428

decreased mineralization around the epiphyses as well as bow-legs with prominent forehead and bumpy ribs are clear indications

Answer 429

immediate surgery and bilateral orchioplexy

Answer 430

mild shampoo and a topical antifungal | seborrheic dermatitis

Answer 431

macroglossia, abdominal wall defects, hypoglycemia due to hyperinsulinemia, films tumor

Answer 432

empiric treatment with doxycycline because it is rapidly fatal

Answer 433

this is severe unconjugated bilirubinemia caused be a genetic defect.. liver transplant is required for survival and phototherapy is used while waiting

Answer 434

Coxsackie A19 and Enterovirus 71, members of the Picornoviridae family.

Answer 435

begins on the face and spreads to the trunk

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