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Pediatrics Study Notes > Peds 2 > Flashcards

Peds 2 Flashcards

1
Q

Mutation of Bruton tyrosine kinase gene - Bruton agammaglobulinemia

A

Failure of pre-B cell expansion & maturation
Low or absent number of B-cells
Pan-hypogammaglobulinemia
Primarily seen in males
Develop infections after 6 mon.
Predisposed to recurrent infections of lungs, sinuses, & bones by encapsulated bacteria or enteroviral meningoencephalitis
Treat w. lifelong Ig-therapy & aggressive Abx for infections

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2
Q

Hyper IgM syndrome

A

High IgM levels
Low IgG, A, E levels
Occurs due to isotype class-switching
Susceptible to P. jiroveci infections, neutropenia, verrucous lesions, & autoimmune disease

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3
Q

Comparing common variable immunodeficiency & Bruton agammaglobulinemia

A

Later age of onset
Less severe infections
Normal B-cells
Failure to differentiate into immunoglobulin-producing cells

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4
Q

X-linked Lymphoproliferative disease

A

Inadequate response to EBV
Abnormal regulatory lymphocyte signaling molecule on T-cells & B-cells EBV infection occurs at first 5 yrs. of age
Can present as fulminant mononucleosis, acquired hypoglobulinemia or B-cell lymphomas
Death by 10 yrs. w/o intervention

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5
Q

Selective IgA deficiency

A

Failure of B-cell maturation into IgA-producing cells
Most likely AD
Increased risk of developing autoantibodies

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6
Q

Treatment of choice for strept throat

A

Oral penicillin V for 10 days or
IM benzathine penicillin G once or
Erythromycin, if allergic to penicillin

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7
Q

Repository form of penicillin that provides low concentrations of the drug over 12 hrs.

A

IM procaine penicillin

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8
Q

Indications of sepsis + meningitis

A 
Respiratory distress
Temp. instability 
Bradycardia 
Hypotonia
Poor feeding 
Lethargy/Irritability 
Vomiting/Diarrhea
Bacteria in CSF
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9
Q

AR disorder characterized by thrombocytopenia and large platelets that presents w. easy bruising, nosebleeds, mucosal bleeding, bleeding a/w surgical methods, menorrhagia, or GI bleeding.
Prolonged bleeding time
Do not aggregate in response to ristocetin
Not corrected by addition of normal plasma
Normal aggregation w. ADP, Epi, collagen

A

Bernard-Soulier syndrome

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10
Q

Deficient GpIIb/IIIa complex causing defective platelet aggregation
Decreased aggregation w. ADP, Epi, collagen
Normal ristocetin response

A

Glanzmann Thrombasthenia

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11
Q

Most common thrombocytopenic purpura in childhood
Present w. brushing and petechial rash (Immune mediated)
Commonly seen 1-4 weeks after a viral infection

A

Immune Thrombocytopenic Purpura

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12
Q 
Most common inherited bleeding tendency 
Presents w. epistaxis, bleeding from gums, menorrhagia, 
& prolonged bleeding from cuts 
Prolonged bleeding time
Absent response to ristocetin 
Corrected by addition of normal plasma
A

von Willebrand disease

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13
Q

Triad of recurrent infections, hemorrhage secondary to thrombocytopenia & eczema

A

Wiskott-Aldrich syndrome

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14
Q

Most common benign tumor of childhood

A

Infantile hemangiomas
Can cause HF & platelet sequestrations and destruction leading to coagulopathy
Involute after 1st year of life

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15
Q

Complication caused by large infantile hemangiomas fur to increased blood flow to the lesions

A

High-output HF

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16
Q

Wounds w. higher risk for tetanus

A 
Contaminated by feces or saliva 
Animal bites 
Puncture wounds 
Avulsions
Missile-related wounds 
Crush injuries 
Burns 
Frostbite
Wounds extended into muscles
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17
Q

TIG should be administered for tetanus-prone wounds in this group regardless of immunization history.

A

HIV-infected patients

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18
Q

X-linked disorder a/w defective generation of oxygen radicals

A

Chronic Granulomatous Disease
Neuts. unable to generate hydrogen peroxide
Susceptible to catalase-producing organisms like S. aureus, B. cepacia, Norcardia, Salmonella, Aspergillus, & Candida
Diagnosed w. flow cytometry using DHR-123 as it measures oxidant production via fluorescence
Only cure: Stem cell transplantation

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19
Q

Common infections seen w. complement-mediated immune deficiency

A

Neisseria

Pneumococcus

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20
Q

B-cell deficiency disorders increase the risk of acquiring infections from pyogenic and enteric organisms and have recurrent sinopulmonary infections.

A

Check quantitative Ig levels

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21
Q

Infantile spasm are commonly present in infants w. tuberous sclerosis and can present as flexion of body w. crying (often confused for colic pain or GERD)

A

Mainstay treatment: ACTH

As it decreases production of CRH, which can cause excess excitability seen in infantile spasms

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22
Q

Treatment of NEC

A

Surgical resection w. reanastomoses after 6 weeks

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23
Q

Suspect perforation when there is free air

A

On left lateral decubitus

Under the diaphragm

24
Q

Radiographic hallmark of NEC

A

Accumulation of gas in the submucosa of the bowel, AKA pneumatosis intestinalis
Progresses to total bowel necrosis, perforation, peritonitis, sepsis & death

25
Q

Type of nutrition administered after surgery in order to rest the bowel

A

Parenteral nutrition for 10-14 days

26
Q

Common presenation of NEC, a condition primarily seen in premature infants

A 
Feeding intolerance 
Emesis 
Abdominal distension 
Blood per rectum 
Temp. instability 
Lethargy 
Hypotonia
Hypoglycemia
Apnea
Bradycardia
27
Q

Relative indications for surgery w. NEC

A 
Single fixed loop of bowel 
Abdominal wall cellulitis
Bowel wall erythema 
Abdominal mass 
Failure of medical management
28
Q

Most common cause of FTT

A

Improper preparation of formula

29
Q

At 9 months of age, a child’s diet must consist of ____________ 4-6x a day

A

Chunky, lumpy & mashed foods
Finger foods
Start cup-drinking
Never allow > 4-6oz. of fruit juice

30
Q

Complications a/w cocaine use

A 
CVA due to HTN 
Rhabdomyolysis 
Pulmonary edema w. alveolar hemorrhage 
Pneumomediastinum 
Pneumothorax
31
Q

Nonketotic hyperglycemia and glycosuria are commonly seen in organophosphate poisoning.

A

Parathion

32
Q

Signs of theophylline intoxication

A 
N/V
Tachycardia
Arrhythmias 
Tremors 
Agitation 
Seizures
Hallucinations
Dysrhythmias 
Hypotension
33
Q

Most common cause of acute liver failure in US

A

Acetaminophen poisoning

34
Q

Process of acetaminophen poisoning

A

Increased production of NAPQI - Hepatocellular damage

Oversaturation of glutathione stores

35
Q

Acetaminophen: Toxic doses

A

<12yrs. — > 200 mg/kg
Adolescents/Adults — > 7.5-10g
(Repeat doses > 75 mg/kg/d on subsequent days

36
Q

Acetaminophen plasma levels should be measured > 4 hrs. post-ingestion.

A

Provide supportive care: ABCs
Trial of gastric decontamination w. activated charcoal
Recheck after 6-8 hrs. even if normal at 4 hrs.

37
Q

Graph used to plot the level of acetaminophen based on time since ingestion & plasma concentrations within 24 hrs. of single ingestion

A

Rumack-Matthew nomogram

38
Q

Presence of this factor indicated a favorable prognosis in patients w. juvenile rheumatoid arthritis

A

Anti-nuclear antibodies

39
Q

Juvenile rheumatoid arthritis presents w.

A

Morning stiffness
Joint pain
Lethargy
& Joint swelling + Decreased ROM on PE

40
Q

Causes of Trisomy 21

A

95% due to nondisjunction in maternal meiosis I
(Overall recurrence rate up to 37 yrs. maternal age: 1%)
1% are mosaics
4% due to Robertsonina translocations
(Overall recurrence rate: 5-7%)
(W. 21-21 translocation, recurrence rate: 100%)

41
Q

Monotherapy is the goal for epilepsy treatment due to:

A

Better compliance
Less adverse effects
Less potential for teratogenesis
Lower cost than polytherapy

42
Q

Treatment w. this drug have been a/w exacerbation of absence seizures

A

Vigabatrin

43
Q

Scarlet fever is caused by specific strains of Group A- beta hemolytic stretptococci that are infected by:

A

Bacteriophage
Via exotoxins A, B, & C
Are bacitracin sensitive

44
Q

One complication of amebiasis is formation of liver abscesses that can present w.

A

Fever
Incrased liver size w. tenderness to palpation
Elevated hemidiaphragm
Right lower lobe atelectasis or effusion
Increased alkaline phosphatase levels

45
Q

Complication fo amebiasis seen within 2 weeks of infection

A

Colitis w. gradual onset
Colicky pain w. frequent bowel movements & diffuse tenderness
Stool positive of occult blood

46
Q

Diagnosis of amebiasis can be confirmed w.

A

Presence of phagocytosed RBC
E. histolytica II stool antigen test
Indirect hemagglutination test

47
Q

Amebiasis is treated w.

A

Metronidazole or Tinidazole

+ Paromomycin

48
Q

Findings in PDA

A

Continuous murmur w. thrill minimizing heart sounds
Wide pulse pressure
Prominent apical impulse w. heave
Mid-diastolic, low-pitched murmur

49
Q

Indomethacin is given to premature infants within first 12 hrs. of life but is contraindicated in term or near term infants as it can cause

A

Constriction of pulmonary arteries leading to pulmonary HTN

50
Q

Course of action for term infants w. HF due to a persistent PDA

A

Prompt transcatheter or surgical closure

51
Q

Condition commonly see in preterm infants characterized by hemorrhage of choroid p;exus which can cause hydrocephalus (Grade III) and lead to liquefactive necrosis of periventricular white matter (AKA periventricular leukomalacia) and more severly, cerebral palsy (commonly spastic dipelgia or quadriplegia)

A

Intraventricular hemorrhage (IVH)

52
Q

Barium enema and anorectal mamometry screening with HD

A

Lack of anal sphincter relaxation w. rectal distention

Transition zone of caliber change

53
Q

HD is confirmed w.

A

Full thickness biopsy of rectal mucosa

54
Q

Development of hydrocephalus w. history of prematurity and seizures after birth

A

Germinal matrix hemorrhage (GMH)

- Highly vascular lining of ventricles that develops mostly b/w 22-30 wks. & vulnerable to hypoxic insults

55
Q

Three consequences of perinatal brain anoxia

A

GMH
Ulegyria
Periventricular leukomalacia

Pediatrics Study Notes (5 decks)

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