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HOSA - Pathophysiology > Other? > Flashcards

Other? Flashcards

1
Q

define ROBINOW SYNDROME

A

condition w/several notable characterisitcs

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2
Q

s/s: robinow syndrome

A

dwarfism, short lower arms, small hands w/clinodactyly, small feet, vertebral segmentation defects, single butterfly vertebra/multiple vertebral segmentation defects, hemivertebrae/vertebral fusions/narrow interpediculate distance, flat face & larger head, bulging forehead w/widely spaced eyes, wide triangular mouth, crowded/misaligned teeth, underdeveloped penis, undescended testicles, clitoris & labia minora underdeveloped

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3
Q

how to diagnosis robinow syndrome

A

characterisitc symptoms & genetic study

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4
Q

tx: robhinow syndrome

A

none

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5
Q

prognosis of robinow syndrome

A

normal life span

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6
Q

define CRI-DU-CHAT SYNDROME

A

hereditary condition with deletion of genetic material from chromosome 5

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7
Q

aka: cri-du-chat syndrome

A

cat’s cry syndrome

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8
Q

s/s: cri-du-chat syndrome

A

microcephaly, deficient cerebral brain tissue, intellectual development disorder, weak/mewing/cat like cry, orbits spaced far apart, slow growth, poor muscle tone, slow development

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9
Q

prognosis of cri-du-chat syndrome

A

fatal w/no cure. results in stillborn/death shortly after birth

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10
Q

define DOWN SYNDROME

A

condition where people have extra 21st chromosome causing them to have 47 chromosomes

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11
Q

s/s: down syndrome

A

small head w/flat back skull, slant to eyes, flat nasal bridge, small low-set ears, small mouth w/protruding tongue, small weak muscles, short hands w/stubby fingers, deep horizontal crease across palm, intellectual development disorder, wide gap between big and little toes

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12
Q

aka: down syndrome

A

trisomy 21

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13
Q

what is the former name of down syndrome

A

mongolism

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14
Q

how to diagnose down syndrome

A

ultrasonography, blood test, amniocentesis, eye exam, karyotype

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15
Q

how often does down syndrome occur

A

1 in 700 live births

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16
Q

tx: down syndrome

A
  • multidimensional approach to maximize development of motor & mental skills
  • surgical correction & antibiotic therapy
  • home/facility care
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17
Q

define PHENYLKETONURIA

A

lack enzyme to metabolize amino acid (phenylalanine) causing build up

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18
Q

sx: phenylketonuria

A
  • musty odor to sweat and urine
  • rashes
  • irritability
  • hyperactivity
  • personality disorders
  • arrested brain development
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19
Q

how is phenylketonuria diagnosed

A

mandatory newborn screening

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20
Q

tx: phenylketonuria

A

phenylalanine free diet

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21
Q

aka: chicken pox

A

varicella zoster

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22
Q

sx: chicken pox

A
  • red macules that turn into papules
  • papules become vesicles w/crusts
  • lesions all over body
  • rash
  • fever
  • malaise
  • anorexia
  • intense itching
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23
Q

list complications of chicken pox

A
  • secondary bacterial infection
  • viral pneumonia
  • conjunctival ulcers
  • Reye syndrome
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24
Q

cause: chicken pox

A

direct/indirect contact w/respiratory droplets or fluid from cutaneous lesions hosting varicella-zoster virus

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25
Q

aka: varicella zoster virus

A

human herpes virus 3

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26
Q

tx: chicken pox

A
  • cool bicarbonate of soda baths
  • cornstarch dusting/calamine lotion
  • acetaminophen
  • antiviral durgs
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27
Q

t/f: aspirin can be given to kids w/chicken pox

A

false bc risk of reye syndrome

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28
Q

when is the chicken pox vaccine commonly administered

A
  • 1st dose @ 12-18 months
  • 2nd dose @ after 24 months
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29
Q

cause of tetanus

A

bacteria Clostridium tetani enter skin

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30
Q

aka: tetanus

A

lockjaw

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31
Q

incubation period for tetanus?

A

3-21 days

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32
Q

tx: tetanus

A

sedatives, muscle relaxants, quiet and dark environment, maintain respiratory integrity, human tetanus immune globulin

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33
Q

what is the mortality rate of tetanus

A

35%

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34
Q

how can tetanus be prevented

A

clean wounds w/hydrogen peroxide

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35
Q

cause: lead poisoning

A

eat lead paint, drink water from lead pipes, ingest lead salts

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36
Q

how is lead poisoning diagnosed

A

blood test, lead excretion through urine, changes on ends of bone in xrays

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37
Q

tx: lead poisoning

A

chelating agents, antiemetics, penicillamine

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38
Q

define HYPERACUTE REACTIONS

A

transplant rejection during operation

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39
Q

define ACUTE REJECTION

A

transplant rejection occurring weeks after surgery or when antirejection meds become ineffective

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40
Q

define CHRONIC REJECTION

A

transplant rejection over months/years bc of vascular injury and inflammation

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41
Q

define ACQUIRED IMMUNODEFICIENCY SYNDROME

A

progressive impairement of immune system from HIV

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42
Q

cause: AIDS

A

HIV

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43
Q

list common malignancies and opportunistic infections/conditions in AIDS pts

A

kaposi sarcoma, lymphomas, pneumocystis carinii pneumonia, TB, herpes simplex, herpes zoster, candida albicans, toxoplasmosis, neurologic complications, diarrhea, hairy leukoplakia

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44
Q

where is HIV2 mainly found

A

west africa

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45
Q

what is the top killer of men and worldwide threat to human kind

A

AIDS (and subsequently HIV)

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46
Q

about how many people are affected with AIDS

A

35 million

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47
Q

what causes AIDS

A

HIV type 1 or 2

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48
Q

how is HIV transmitted

A

direct contact with infected blood, semen, breast milk or in utero

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49
Q

how is AIDS diagnosed

A

ELISA test, Western blot test, positive p24 antigen test, positive PCR assay

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50
Q

there is no cure for AIDS, but this treatment prolongs life and maintains quality of life

A

HAART

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51
Q

describe HAART

A

three drug combo treatment for AIDS (nucleoside reverse transcriptase inhibitors, protease inhibitior, non-nucleoside reverse transcriptase inhibitor)

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52
Q

define COMMON VARIABLE IMMUNODEFICIENCY

A

acquired B cell deficiency causing decreased antibody production/function

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53
Q

aka: common variable immunodeficiency

A

acquired hypogammaglobulinemia

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54
Q

cause of common variable immunodeficiency

A

unknown, suspected to be genetics

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55
Q

how is common variable immunodeficiency diagnosed

A

history of repeated and chronic infections, antibody response to vaccines poor, hypogammaglobulinemia and IgA/IgM deficiency

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56
Q

tx: common variable immunodeficiency

A

regular immunoglobulin replacement and vaccination

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57
Q

cause of selective immunoglobulin A deficiency

A

B cells fail to produce enough IgA

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58
Q

how is selective immunoglobulin A deficiency diagnosed

A

CBC, complement assay, vaccine response testing, monitor for 4 years

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59
Q

tx: selective immunoglobulin A deficiency

A

none; prophylactic antibiotics, IV immunoglobulin with low concentration of IgA

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60
Q

aka: x-linked agammaglobulinemia

A

bruton agammaglobulinema

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61
Q

what is the cause of x-linked agammaglobulinemia

A

congenital defect in Burton tyrosine kinase gene

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62
Q

how is x-linked agammaglobulinemia diagnosed

A

clinical findings, pt history, decreased serum levels of IgM/IgA/IgG, BTK gene mutation

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63
Q

tx: x-linked agammaglobulinemia

A

IV/subQ infusion of immune globulin, antibiotics, killed vaccines

64
Q

describe SEVERE COMBINED IMMUNODEFICIENCY

A

group of disorders with absence of T cell immunity and B cell immunity

65
Q

aka: severe combined immunodeficiency

A

bubble boy disease

66
Q

cause of severe combined immunodeficiency

A

genetic (x-linked and autosomal)

67
Q

how is severe combined immunodeficiency diagnosed

A

routine newborn screen, measure immunoglobulin levels, antibody titers, number of T and B cells, lymphopenia, absence of thymic shadow on chest xray

68
Q

tx: severe combined immunodeficiency

A

bone marrow transplant, gene therapy, supportive therapy, sterile environments

69
Q

children with SCID generally live until

A

1 year old

70
Q

aka: DiGeorge Anomaly

A

Thymic Hypoplasia; aplasia; DiGeorge Syndrome

71
Q

define DIGEORGE ANOMALY

A

congenital condition from defective development of pharyngeal pouch system

72
Q

cause of DiGeorge Anomaly

A

abnormal development of third and fourth pharyngeal pouches

73
Q

how is DiGeorge anomaly diagnosed

A

reduced number of T cells and 2 of three conditions (cardiovascular defects, hypocalcemia greater than 3 weeks, microdeletion involving chromosome 22); absence/hypoplasia/abnormal placement of thymus

74
Q

tx: DiGeorge Anomaly

A

Vitamin D and parathyroid hormone replacement therapy, thymic transplantation, Bone marrow transplant

75
Q

define CHRONIC MUCOCUTANEOUS CANDIDIASIS

A

group of disorders with persistent and recurrent candidal infections of skin, nails, and mucous membranes

76
Q

how is chronic mucocutaneous candidiasis diagnosed

A

history of recurrent fungal infections, scrapings from lesions show yeast pseudohyphae (string of atypical fungal cells), normal T cell circulation and antibody response to all organisms except Candida

77
Q

tx: chronic mucocutaneous candidiasis

A

systemic therapy with antifungal agents, transfer factor, topical treatment

78
Q

define WISKOTT-ALDIRCH SYNDROME

A

congenital disorder with inadequate B and T cell function, thrombocytopenia, and eczema

79
Q

cause of Wiskott-Aldrich Syndrome

A

x-linked trait, mutation of Wiskott-Aldrich protein

80
Q

how is Wiskott-Aldrich Syndrome diagnosed

A

boy, thrombocytopenia, small platelet size, frequent infections, eczema, low number of T and B cells

81
Q

tx: Wiskott-Aldrich Syndrome

A

bone marrow therapy

82
Q

define AUTOIMMUNE HEMOLYTIC ANEMIA

A

autoimmune condition where RBCs are destroyed by antibodies

83
Q

cause of autoimmune hemolytic anemia

A

B cell antibodies don’t recognize RBCs causing agglutination, drugs, autoimmune diseases, malignancies, fixation of complement proteins on IgM

84
Q

how is autioimmune hemolytic anemia diagnosed

A

direct Coombs test showing antibody coated RBCs, spherical RBCs, elevated serum bilirubin, RBC count/platelet count/Hgb concentration/hematocrit decreased

85
Q

tx: autoimmune hemolytic anemia

A

corticosteroids, cytotoxic drugs, splenectomy, IVIG administration, RBC transfusions, dress warmly (cold)

86
Q

describe PERNICIOUS ANEMIA

A

anemia from chronic atrophic gastritis causing shortage of intrinsic factor and decreased HCI production

87
Q

how is PERNICIOUS ANEMIA diagnosed

A

schilling test, blood tests (decreased Hgb and RBC and increased increased MCV), bone marrow studies, reduced/absent gastric acid, hypersegmented neuropathy

88
Q

tx: pernicious anemia

A

IM injections of vitamin B12, blood replacement, rest, gentle nonirritating mouth care, well-balanced diet high in vitamin B12, tranquilizers

89
Q

tx: pernicious anemia

A

none

90
Q

describe IDIOPATHIC THROMBOCYTOPENIC PURPURA

A

autoantibodies against platelets from isolated deficiency of platelets

91
Q

cause of idiopathic thrombocytopenic purpura

A

idiopathic

92
Q

how is idiopathic thrombocytopenic purpura diagnosed

A

CBC, peripheral blood smear, clinical symptoms, prolonged bleeding time, reduced platelets, reduced circulating platelet time

93
Q

tx: idiopathic thrombocytopenic purpura

A

corticosteroid administration, IVIG, blood transfusion, vitamin K, therapeutic plasma exchange, splenectomy

94
Q

kids with idiopathic thrombocytopenic purpura may experience

A

spontaneous remission

95
Q

define IMMUNE NEUTROPENIA

A

decrease in number of circulating neutrophils from production of antineutrophil antibodies

96
Q

cause of immune neutropenia

A

infection, drug exposure, ITP, autoimmune hemolytic anemia, connective tissue disease

97
Q

how is immune neutropenia diagnosed

A

significantly reduced number of neutrophils in WBC count, immunofluorescence, agglutination test, antiglobulin analysis

98
Q

tx: immune neutropenia

A

corticosteroids, immune globulin, granulocyte-colony-stimulating factor, transfusions of WBC concentrates

99
Q

aka: goodpasture syndrome

A

anti-glomerular basement membrane antibody disease

100
Q

define GOODPASTURE SYNDROME

A

autoimmune kidney disease from antibodies directed against antigen in GBM

101
Q

cause of goodpasture syndrome

A

antibodies cause damage in glomerular and alveolar basement membranes causing glomerulonephritis and pulmonary hemorrhage

102
Q

how is goodpasture syndrome diagnosed

A

detection of anti-GBM antibodies in serum or kidney: ELISA, immunofluorescence, renal biopsy, protein and blood in urine

103
Q

tx: goodpasture syndrome

A

plasmapheresis, immunosuppressive agents, hemodialysis, kidney transplant

104
Q

connective tissue diseases destroy what

A

collagen

105
Q

aka: systemic lupus erythematosus

A

lupus

106
Q

cause of lupus is unknown, but what are some precipitate factors

A

stress, immunization reactions, pregnancy, overexposure to UV light

107
Q

how is lupus diagnosed

A

at least 4 sx or positive lab tests (butterfly rash on face, discoid skin lesion, photosensitivity, nasopharyngeal ulceration, polyarthritis w/o deformity seizures/psychosis, chronic pleuritis or pericarditis, false positive serologic test for syphilis/DNA/SM antibodies, cellular casts in urine, thrombocytopenia, and more)

108
Q

tx: lupus

A

antiinflammatory drugs (aspirin/NSAIDS/prednisone), antimalarial agents, corticosteroids, immunosuppressive meds

109
Q

aka: scleroderma

A

systemic sclerosis

110
Q

define SCLERODERMA

A

chronic progressive disease of sclerosis of skin and scarring of internal organs

111
Q

demographic of scleroderma

A
  • four times more often in women
  • gen 30-50 yrs
112
Q

how is scleroderma diagnosed

A

complete pt history, physical exam, lab testing (Scl-70 and centromere antibodies), skin biopsy, blood tests, urinalysis

113
Q

tx: scleroderma

A

palliative care and physical therapy

114
Q

cause of scleroderm

A

unknown

115
Q

define SJOGREN SYNDROME

A

inflammation of moisture secreting glands

116
Q

cause of sjogren syndrome

A

genetics

117
Q

most people with sjogren syndrome are

A

female

118
Q

how is sjogren syndrome diagnosed

A

dry eyes during schirmer test, anemia, decreased WBC count, elevated ESR, autoantibodies, high rheumatoid factor titers

119
Q

tx: sjorgen syndrome

A

increase fluids, chew sugarless gum, oral sprays, artificial tears, sunglasses, blockage of tear ducts, prednisone, antimalarial meds

120
Q

demographics of rheumatoid arthritis

A
  • affects women three times more than men
  • gen ppl in 30-40 yrs
121
Q

how many americans does rheumatoid arthritis affect

A

1.5 million

122
Q

cause of rheumatoid arthritis is unknown, but what are some potential causes

A

hereditary and viral infection

123
Q

how is rheumatoid arthritis diagnosed

A

sx, physical exam, pt and family history, xrays, elevated rheumatoid factor

124
Q

tx: rheumatoid arthritis

A

antiinflammatory meds, corticosteroids, DMARDS, immunosuppressive agents, special splints, surgery, artificial joints

125
Q

aka: juvenile idiopathic arthritis

A

juvenile rheumatoid arthritis

126
Q

what is the age for juvenile idiopathic arthritis, and what age range is most common

A

< 16 yrs; 2-5 yrs

127
Q

how is juvenile rheumatoid arthritis diagnosed

A

elevated rheumatoid factor

128
Q

tx: juvenile rheumatoid arthritis

A

diet, physical therapy, exercise antiinflammatory meds, splints, DMARDS, immunosupressive agents

129
Q

ankylosing spondylitis occurs more commonly in _____ and ____ generally have a milder form

A

young men; women

130
Q

cause of ankylosing spondylitis

A

unknown; genetics

131
Q

how is ankylosing spondylitis diagnosed

A

xray of symptom areas, bilateral sacroiliitis, squaring of vertebrae, fusion of joints, generalized osteoporosis, bamboo spine

132
Q

tx: ankylosing spondylitis

A

none; antiinflammatory meds, physical therapy, analgesics, moderate exercise

133
Q

define POLYMYOSITIS

A

inflammation of muscle fibers

134
Q

how is polymyositis diagnosed

A

pt history, physical exam, electromyography, muscle biopsy, elevated creatinine phosphokinase/aldolase/serum glutamate oxaloacetate transaminase/serum glutamate pyruvate transaminase/lactate dehydrogenase

135
Q

tx: polymyositis

A

steroids, immunosuppressive agents, exercise

136
Q

define MULTIPLE SCLEROSIS

A

inflammation and demyelination of CNS causing scarring

137
Q

cause of MS is unknown, but what is thought to be a trigger

A

unknown virus

138
Q

list the types of MS

A
  • relapsing-remitting
  • primary-progressive
  • secondary-progressive
  • progressive-relapsin
139
Q

list risk factors fo rMS

A

being white and living in north

140
Q

MS more commonly affects

A
  • women
  • people 20-40 yrs
141
Q

how is MS diagnosed

A

at least 2 episodes of CNS dysfunction, cerebral/spinal plaque, elevated immunoglobulin levels, oligoclonal bands on MS panel

142
Q

tx: MS

A

corticosteroids, biologic medications, immunosuppressive agents, improved meds (interferon beta-1a, interferon-beta-1b, glatiramer, mitoxantrone, siponimod, cladribine), muscle relaxants, vitamin supplements, physical therapy, diet, rest, mobility devices

143
Q

what group of MS pts have best prognosis

A
  • young females
  • relapsing
  • impairment of sensory pathways as initial symptoms
144
Q

how is myasthenia gravis diagnosed

A

tensilon test and EMG

145
Q

myasthenia gravis demographics

A
  • women 20-40 yrs
  • men >60 yrs
146
Q

cause of myasthenia gravis

A

antibodies against acetylcholine receptors preventing nerve impulses and/or thymus hyperplasia or thymoma

147
Q

tx: myasthenia gravis

A

restricted activity, bed rest, soft/liquid diet, anticholinesterase drugs (pyridostigmine bromide), thymectomy, corticosteroids, immunosuppression

148
Q

describe VASCULITIS

A

inflammation of walls of blood vessels causing it to be necrotic and infarct adjascent tissue

149
Q

what are the two types of vasculitis

A

small vessel vasculitis and systemic necrotizing vasculitis

150
Q

how is small vessel vasculitis diagnosed

A

histology, immunofluorescence studies, immunoglobulins and complement in blood vessel, pattern of vascular involvement, present cell types

151
Q

tx: small vessel vasculitis

A

treat underlying cause

152
Q

cause of small vessel vasculitis

A

unknown; immune disorders, chemicals, foreign proteins, medications, foods, infections

153
Q

cause of systemic necrotizing vasculitis

A

autoimmune response, illicit drug use, HBV and HCV

154
Q

how is systemic necrotizing vasculitis diagnosed

A

elevated WBC and platelet count, pulmonary infiltrates on chest xray, angiography, biopsy

155
Q

tx: small necrotizing vasculitis

A

corticosteroids, analgesics, immunosuppressive agents

156
Q
A

HOSA - Pathophysiology (5 decks)

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