Peds 2 Flashcards
(55 cards)
Mutation of Bruton tyrosine kinase gene - Bruton agammaglobulinemia
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
Hyper IgM syndrome
High IgM levels
Low IgG, A, E levels
Occurs due to isotype class-switching
Susceptible to P. jiroveci infections, neutropenia, verrucous lesions, & autoimmune disease
Comparing common variable immunodeficiency & Bruton agammaglobulinemia
Later age of onset
Less severe infections
Normal B-cells
Failure to differentiate into immunoglobulin-producing cells
X-linked Lymphoproliferative disease
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
Selective IgA deficiency
Failure of B-cell maturation into IgA-producing cells
Most likely AD
Increased risk of developing autoantibodies
Treatment of choice for strept throat
Oral penicillin V for 10 days or
IM benzathine penicillin G once or
Erythromycin, if allergic to penicillin
Repository form of penicillin that provides low concentrations of the drug over 12 hrs.
IM procaine penicillin
Indications of sepsis + meningitis
Respiratory distress Temp. instability Bradycardia Hypotonia Poor feeding Lethargy/Irritability Vomiting/Diarrhea Bacteria in CSF
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
Bernard-Soulier syndrome
Deficient GpIIb/IIIa complex causing defective platelet aggregation
Decreased aggregation w. ADP, Epi, collagen
Normal ristocetin response
Glanzmann Thrombasthenia
Most common thrombocytopenic purpura in childhood
Present w. brushing and petechial rash (Immune mediated)
Commonly seen 1-4 weeks after a viral infection
Immune Thrombocytopenic Purpura
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
von Willebrand disease
Triad of recurrent infections, hemorrhage secondary to thrombocytopenia & eczema
Wiskott-Aldrich syndrome
Most common benign tumor of childhood
Infantile hemangiomas
Can cause HF & platelet sequestrations and destruction leading to coagulopathy
Involute after 1st year of life
Complication caused by large infantile hemangiomas fur to increased blood flow to the lesions
High-output HF
Wounds w. higher risk for tetanus
Contaminated by feces or saliva Animal bites Puncture wounds Avulsions Missile-related wounds Crush injuries Burns Frostbite Wounds extended into muscles
TIG should be administered for tetanus-prone wounds in this group regardless of immunization history.
HIV-infected patients
X-linked disorder a/w defective generation of oxygen radicals
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
Common infections seen w. complement-mediated immune deficiency
Neisseria
Pneumococcus
B-cell deficiency disorders increase the risk of acquiring infections from pyogenic and enteric organisms and have recurrent sinopulmonary infections.
Check quantitative Ig levels
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)
Mainstay treatment: ACTH
As it decreases production of CRH, which can cause excess excitability seen in infantile spasms
Treatment of NEC
Surgical resection w. reanastomoses after 6 weeks
Suspect perforation when there is free air
On left lateral decubitus
Under the diaphragm
Radiographic hallmark of NEC
Accumulation of gas in the submucosa of the bowel, AKA pneumatosis intestinalis
Progresses to total bowel necrosis, perforation, peritonitis, sepsis & death
