Systemic Pathology Flashcards
(156 cards)
1
Q
Pellagra
A
Caused by a deficiency of Niacin. The three “D”s of Pellagra are diarrhea, dementia, dermatitis (and sometimes death)
2
Q
50 % of cases of subacute (bacterial) endocarditis are caused by
A
streptococcus viridans
3
Q
Bacteria that is the most common cause of infectious endocarditis in IV drug users
A
staphylococcus aureus
4
Q
50 % of cases of acute endocarditis
A
staphylococcus aureus
5
Q
most commonly encountered neck space infection is
A
ludwig’s angina-usually extension of infection from mandibular molars and involves submandibular, submental and sublingual spaces
6
Q
Contain characteristic sulfur granules (colonies of infecting organisms)
A
actinomycotic lesions
7
Q
Chlamydial cervicitis, most common STD is caused by what bacteria?
A
C. trachomatis
8
Q
The characteristic lesion of secondary syphilis (caused by Treponema pallidum) is known as
A
condyloma lata: primary (chancre), secondary (maculopapular rash and condyloma lata), tertiary (gumma)-Penicillin G is drug of choice for all stages
9
Q
Encephalitis is most often caused by
A
Viral infection: including enteroviruses (coxsackievirus, poliovirus, and echovirus), Herpesviruses type 1 and 2, rabies, HIV, VZV and CMV
10
Q
What are the most common causes for meningitis in adults?
A
Neisseria meningitidis and Streptococcus pneummonia
11
Q
What is the most common cause for meningitis in children under the age of 2?
A
Hemophilus influenzae
12
Q
Waterhouse-Friederichsen syndrome
A
overwhelming, rapidly progressing infection caused by Neisseria meningitidis. It produces severe diarrhea, vomiting, seizures, internal bleeding (bilateral adrenal hemorrhage), low blood pressure, shock, and often death
13
Q
What is the MOST infectious known bloodbourne pathogen?
A
Hepatitis B Virus
14
Q
What disease is associated with pancarditis, aschoff bodies and sydenham chorea?
A
Rheumatic fever-additional findings include migratory polyarthritis of the large joints, subcutaneous nodules, erythema margination of the skin.
15
Q
Addisons disease characteristics
A
Primary adrenaocortical deficiency caused by partial or complete failure of adrenocortical function and characterized by onset of weakness, fatigue, depression, hypotension, skin bronzing, hypoglycemia and increased serum potassium (treated with cortisol-hydrocortisone)
16
Q
Waterhouse-Friederichsen syndrome
A
Catastrophic adrenal insufficiency and vascular collapse due to hemorrhagic necrosis of the adrenal cotex. Characteristically due to neingococcemia, most often in association with meningococcal meningitis.
17
Q
Eosinophilic granuloma
A
Form of Langerhans cell histiocytosis characterized by proliferations of Langerhans cells admixed with variable number of eosinophils, lymphocytes, plasma cells and neutrophils. This is the most common and benign form.
18
Q
Hand-Christian-Schuller Disease
A
Form of Langerhans cell histiocytosis usually involving children with the clasic triad of calvarial bone defects, diabetes insipidus, and exopthalmos caused by involvement of the orbit.
19
Q
Letterer-Siwe Disease
A
Form of Langerhans cell histiocytosis occurring most frequently before 2 years of age. A dominant clinical feature is the development of cutaneous lesions resembling a seborrheic eruption, which is caused by infiltrates of Langerhans cells over the front and back of the trunk and scalp.
20
Q
Pulmonary Langerhans cell histiocytosis
A
Form of Langerhans cell histiocytosis represenging a special category of disease, most often seen in adult smokers, which may regress spontaneously upon cessation of smoking
21
Q
Bruton’s Disease AKA X-linked infantile agammaglobulinemia
A
features a defective tyrosine kinase B cell gene which results in a lack of mature B cells. This decreases all 5 immunoglobulin isotypes and leaves the person extremely vulnerable to a wide array of infections
22
Q
Primary hyperparathyroidism is most often caused by
A
parathyroid adenoma: the constellation of symtpoms includes bone disease, nephrolithiasis, gastrointestinal distrubances, CNS alterations, neuromuscular abnormalities and loss of lamina dura around multiple teeth. Hypercalcemia, decreased serum phosphorus and increased serum PTH
23
Q
Collagen vascular disease associated with systemic vasculitis
A
polyarteritis nodosa
24
Q
Collagen vascular disease associated with skin rash
A
Dermatomyositis
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Collagen vascular disease associated with widespread connective tissue fibrosis
Scleroderma
26
Collagen vascular disease associated with butterfly rash over the cheeks and bridge of nose
Systemic lupus erythematosus
27
Collagen vascular disease associated with stiffness of joints
Rheumatoid arthritis
28
polyarteritis nodosa
a serious blood vessel disease of unknown cause characterized by necrotizing immune complex inflammation of small and medium sized arteries (there is an association with hepatitis B viral infection)
29
Dermatomyositis
muscle disease that causes inflammation and a skin rash. It is a type of inflammation myopathy. The cause is unknown
30
Scleroderma
A widespread connective tissue disease that involves changes in the skin, blood vessels, muscles, and internal organs. It is common in YW, involves widespread connective tissue fibrosis and causes tight and mask-like facial skin, raynaud's phenomenon, pain, stiffness, and swelling of fingers and joints (also incrased chance of Barrett's esophagus)
31
Systemic lupus erythematosus
prototype connective tissue disease (SLE is an autoimmunde disease). 80% of patients are women presenting with symptoms of fever, malaise, lymphadenopathy and weight loss. There is a characteristic butterfly rash and extensive immune complex-mediated inflammatory lesions (of greatest clinical importance in the kidney)
32
Rheumatoid arthritis
Chronic systemic inflammatory disorder that may affect many tissues or organs-skin, blood vessels, lungs, heart, and muscles--but principally attacks the joints.
33
Multiple sclerosis
Most common demyelinating disorder of the brain and spinal cord (CNS) caused by progressive damage to the outer covering of nerve cells (myelin). Common symptoms include visual and speech disturbances, paresthesias, depression, and mood swings.
34
Primary amyloidosis
Unknown cause-related to abnormal production of immunoglobulins by malignant plasma cells. It is usually systemic in distribution. Typical sites of amyloid buildup include the heart, lungs, skin, tongue, thyroid gland, intestines, liver, kidney, and blood vessels.
35
Seconary (reactive) systemic amyloidosis
the amyloidosis is a complication of another disease such as TB, rheumatoid arthritis, or Familial Mediterranean fever. Amyloid tends to build up in the spleen, liver, kidneys, adrenal glands, and lymph nodes. The heart is rarely involved.
36
Hereditary amyloidosis
mostly rare and limited to specific geographic areas, these amyloidoses are the result of genetic mutations. Examples include Familial Mediterranean fever, systemic senile amyloidosis and several types of familial amyloidotic neuropathies.
37
What is characterized by deposits of amyloid in islet cells?
Diabetes mellitus type 2
38
Autoimmune disease characterized by pancreatic ß-cell destruction and an absolute deficiency of insulin
Type 1 diabetes
39
Caused by a combination of peripheral resistance to insulin action and an inadequate secretory response by the pancreatic ß-cells ("relative insulin deficiency")
Type 2 diabetes
40
What are typical values for normal blood sugars mg/dl and Hb1AC % respectively?
<6%
41
What condition is associated with joint effusions, Still's disease and unknown cause?
Rheumatoid arthritis
42
Rheumatoid arthritis characteristics
chronic systemic inflammatory disorder htat may affect many tissues or organs-skin, blood vessels, heart, lungs, and muscles-but principally attacks the joints producing a nonsuppurative proliferation and inflammatory synovitis. Radiographic hallmarks: joint effusions, juxta-articular osteopenia with eroisions and narrowing of the joint space with loss of articular cartilage.
43
Still's disease
type of rheumatoid arthritis that occurs in young people
44
Autoimmune neuromuscular disorder caused by the production of antibodies against the acetylocholine receptor which results in the inhibition of neuromuscular transmission and eventual paralysis
myasthenia gravis
45
Syndrome similar to myasthenia gravis in that it is an autoimmune disease that causes weakness, but different in that it is caused by inadequate release of acetylcholine rather than by abnormal antibodies to acetylcholine receptors.
Eaton-Lambert syndrome
46
Characterized by the deposition of monosodium urate crystals in joints and other tissues as a result of hyperuricemia. The disorder occurs most frequently in the metatarsophalangeal joint of the big toe.
Gout
47
Lesch-Nyhan syndrome
hyperuricemia with severe neurologic manifestations
48
Pseudogout (chondrocalcinosis)
caused by calcium pyrophosphate dihydrate crystal deposition, which elicits an inflammatory reaction in cartilage. Pseudogout clinically resembles gout
49
Results from inadequate secretion of thyroid hormones during fetal life or early infancy
cretinism
50
Cretinism characteristics
Stunted body growth and impaired mental development. Symptoms appear during early infancy and include the development of coarse, dry skin, slightly swollen face and tonge and an open mouth that drools.
51
Myxedema characteristics
Hypothyroidism in adults. Considerably more common in women and may inclue puffiness of the face and eyelids and a swelling of the tongue and larynx. Skin becomes dry and rough and the har becomes sparse.
52
Blood vessel associated with epidural hematoma
Middle meningeal artery
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Blood vessel associated with subdural hematoma
Bridging veins
54
Blood vessel associated with subarachnoid hematoma
circle of Willis (particularly the rupture of a berry aneurysm)
55
Disorders associated with a deficiency of Vitamin B1 (thiamine)
wet beriberi, dry beriberi, Wernicke-Korsakoff syndrome
56
Disorders associated with a deficiency of Vitamin B2 (riboflavin)
Cheilosis, glossitis, dermatitis
57
Disorders associated with a deficiency of Vitamin B3 (niacin)
pellagra
58
Disorders associated with a deficiency of Vitamin B6 (pyridoxine)
cheilosis, glossitis, anemia
59
Disorders associated with a deficiency of Vitamin B12 (cobalamin)
megaloblastic anemia, neurologic dysfunction
60
Disorders associated with a deficiency of Folic acid
megaloblastic anemia, ***neurologic dysfunction is not a feature (as in Vitamin B12 deficiency)
61
Disorders associated with a deficiency of Vitamin C (ascorbic acid)
Scurvy, defective wound healing
62
Trachoma is an eye infection caused by what?
Chylamydia trachomatis causes trachoma, an eye infection. It is the most common cause of preventable blindness in underdeveloped areas of the world
63
Retinopathy of prematurity
(retrolental fibroplasia) is due to toxicity of therapeutic oxygen, most often administered because of neonatal respiratory distress syndrome (hyaline membrane disease). It leads to blindness.
64
Retinitis pigmentosa
Characterized by hereditary night blindness with progressive loss of central vision. It is caused by early loss of rods and later loss of cones.
65
Open-angle glaucoma
Most common form of glaucoma; characterized by gradually increasing ocular pressure, leading to visual impairment and, eventual blindness
66
Angle-closure glaucoma
caused by narrow anterior chamber angle; increase in intraocular pressure on dilation of pupil
67
Neurofibromatosis type I AKA von Recklinghausen's disease characteristics
autosomal dominant, neurofibromas, gliomas of the optic nerve, pigmented nodules of the iris (Lisch nodules), and cutaneuous hyperpigmented macules (café-au-lait spots)
68
Neurofibromatosis type II
autosomal dominant resulting in a range of tumors, most commonly bilateral eighth-nerve schwannomas and multiple meningiomas. Gliomas, typically ependymomas of the spinal cord also occur in these patients.
69
Familial hypercholesterolemia
autosomal dominant disorder characterized by anomalies of receptors for low-density lipoproteins
70
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)
autosomal dominant disorder characterized by local telangiectases of the skin and mucous membranes and by recurrent hemorrhage from these lesions. Common in Mormon families of Utah.
71
Marfan syndrome
autosomal dominant disorder characterized by defects in dkeletal, visual, and cardiovascular structures. Patients are tall and thin with abnormally long legs and arms, spider-like fingers and hyperextensible joints. Heart problems include: aneurysm of the proximal aorta, mitral valve prolapse and dissecting aneurysm of the aorta.
72
Adult polycystic kidney disease
Autosomal dominant disorder characterized by numerous bilateral cysts that replace and destroy the renal parenchyma
73
Most common cause of hypothyroidism
Hashimoto's thyroiditis (aka chronic thyroiditis or Hashimoto's disease)
74
Most common cause of endogenous hyperthyroidism
Graves disease
75
Somatotropic adenoma with hypersecretion of growth horone that develops before epiphysial closure results in
gigantism
76
Somatotropic adenoma with hypersecretion of growth hormon
Second most common pituitary tumor. Gigantism results if adenoma develops before epiphysial closure and acromegaly if after.
77
Cushing syndrome
Corticotropic adenoma with hypersecretion of ACTH. This disorder is caused by any condition that produces elevated glucocorticoid levels. The vast majority are the result of the administration of exogeneous glucocorticoids.
78
The pathogenic lesion of erythema multiforme is a
"target lesion" Erythema multiforme is an uncommon self-limited disorder that seems to be a hypersensitivity reaction to certain infections and drugs.
79
Stevens-Johnson syndrome
An extensive and symptomatic febrile form of erythema multiforme
80
Toxic epidermal necrolysis
A variant of erythema multiforme resulting in diffuse necrosis and slouhing of cutaneous and mucosal epithelial surfaces, producing a clinical situation analagous to an extensive burn when both infection and fluid loss are clinical concerns.
81
Most closely linked skin disorder linked with vesicles on mucosa
pemphigus-auto antibodies against intercellular junctions of keratinocytes
82
Most closely linked skin disorder linked with large, red nose
rosacea
83
Most closely linked skin disorder linked with honey colored crust, superficial skin infection
impetigo-Staphylococcus aureus or Group A beta-hemolytic Streptococci
84
Most closely linked skin disorder linked with Herald patch
pityriasis-may be of viral origin
85
Most closely linked skin disorder linked with irregular depigmentation
vitiligo
86
Xanthoma
Hyperlipidemia, foamy histiocytes
87
Capillary hemangioma
"Salmon patches" and stork bites--spontaneously regresses. "Strawberry hemangiomas"--initially grows and later regresses
88
Cavernous hemangioma
"Port-wine stain"--seen in Sturge-Weber syndrome--does not resovle spontaneously
89
Zollinger-ellison syndrome
rare disorder that causes tumors in the pancreas and duodenum and ulcers in the stomach and duodenum.
90
Hypothalamic "central" diabetes insipidus
results from a deficiency in secretion of antidiuretic hormone from the posterior pituitary. Causes of this disease include head trauma, an dinfections or tumors involving the hypothalamus
91
Nephrogenic diabetes insipidus
occurs when the kidney is unable to respond to antidiuretic hormone. Most commonly, this results from some type of renal disease, but mutations in the ADH receptor gene or in the gene encoding aquaporin-2 have also been demonstrated in affected humans
92
Infectious mononucleosis is a benign, self-limiting disorder caused by
Epstein-barr virus (EBV)
93
Conditions associated with epstein-barr virus (EBV)
Infectious mononucleosis, Burkitt's lymphoma, nasopharyngeal carcinoma and hairy leukoplakia
94
Characterized by a breakdown in self-tolerance to thyroid auto-antigens, most importantly the TSH receptor
Graves disease
95
One type of hyperthyroidism (Grave's Disease)
Most common cause of endogenous hyperthyroidism. Women affected 10X more than men. It is a thyroid-specific autoimmune disorder in which the body makes antibodies to the TSH receptor, leading to hyperthyroidism. Symptoms include anxiety and restlessness to insomnia and weight loss. Eyeballs may also begin to protrude (exophthalmos) causing irritation and tearing.
96
A second type of hyperthyroidism (Plummer's disease)
(Toxic nodular goiter) arises from a long-standing simple goiter and occurs most often in the elderly. Exophthalmos does NOT occur as in Graves disease. This disease is never seen in children.
97
Vitamin B12 malabsorption occurs in what syndromes due to the absence of intrinsic factor?
pernicious anemia and Crohn's disease
98
Which malabsorption syndrome Is caused by a sensitivity to gluten in cereal?
Celiac disease-the mucosal lining of the small intestine is damaged by ingestion of gluten. This can be fatal in adults due to the development of lymphoma in the intestine.
99
Whipple disease
systemic bacterial illness usually affecting middle age men and presents with diarrhea, anemia, arthritis, fever, weight loss, swollen lymph nodes and skin pigmentation.
100
Characterized by the presence of numerous polyps along with skin and bone tumors
Gardner's syndrome-Autosomal dominant. Supernumerary teeth are common and the risk of malignant transformation approaches 100%.
101
Polyposis syndrome affecting the GI tract that is autosomal dominant and caused by the absence of APC (a tumor suprressor gene)
Familial adenomatous polyposis
102
Polyposis syndrome affecting the GI tract that is autosomal recessive and characterized by adenomatous polysps along with CNS tumors
Turcot's syndrome
103
Polyposis syndrome affecting the GI tract that is autosomal dominant and characterized by hamartomatous polyps of the colon and small intestine, melanin pigmentation in the mouth and on the lips, hands, and genitalia. This syndrome is also associated with an increased risk for adenocarcinoma of the colon.
Peutz-Jeghers syndrome
104
Complications of Down syndrome
congenital heart disease, increased risk of lymphoblastic leukemia and increased susceptibility of infection
105
Cri du chat syndrome
Caused by deletion of the short arm of chromosome 5; characteristics are severe mental retardation, microcephaly, and an unusual catlike cry
106
DiGeorge Syndrome
Caused by deletion of a portion of chromosome 22. Characteristics include CATCH 22-Cardiac abnormalities, Abnormal facies, T cell dificient because of thymic hypoplasia, Cleft palate, Hypocalcemia and microdeletion of 22q11.
107
Edwards Syndrome (trisomy 18)
Characteristics include mental retardation, face has a pinched appearanace, micrognathia (small jaw), head is small with low-set ears and congenital heart disease
108
Patau syndrome (trisomy 13)
Characteristics include mental retardation, microcephaly, micropthalmia, cleft lip and palate, brain abnormalities, polydactyly, and congenital heart disease
109
Sjögren's syndrome triad of findings
Keratoconjunctivitis sicca, xerostomia, and associated connective tissue disease (most often rheumatoid arthritis)
110
Immunodeficiency disorder featuring incompetent or absent T cells and B cells
Severe combined immunodificiency disease (think bubble boy syndrome)
111
Wiskott-Aldrich syndrome
AKA immunodeficiency with eczema and thrombocytopenia-affects only boys and is characterized by defective B cell and T cell functions. Clinical features include thrombocytopenia with severe bleeding, eczema, recurrent infection, and increased risk of lymphoid cancers
112
Ataxia-telangiectasia
Inherited disorder affecting many tissues and systems in the body. Symptoms include telangiectasis (dilation of capillaries), ataxic (uncoordinated) gait, proneness to infection, defective humoral and cellular immunity and increased risk of malignancies.
113
Hyperimmunoglobulin E syndrome
AKA Job syndrome-an immunodeficiency disorder characterized by very high levels of IgE antibodies and repeated infections, mostly by Staph aureus.
114
Klinefelter syndrome
XXY, hypogonadism
115
Turner syndrome
XO, infertility, webbed neck, and other abnormalities
116
Coccidioidomycosis
Chronic, necrotizing infection resembling TB endemic to arid regions of SW US and Latin America. Causes "Valley Fever and endospores form caseating granulomas. (One of the most infectious agents known)
117
Histoplasmosis
Usually a self-limited mycosis byt can lead to systemic granulomatous fungal infection. Granulomas heal by calcification unless there is immunosuppression (Amphotericin B is used with progressive lung lesions and disseminated disease)
118
Blastomyces dermatitidis
Systemic fungal infection mainly involving the respiratory tract, causing granulomatous lesions in the lungs.
119
Paracoccidioides brasiliensis
Systemic fungal infection endemic to parts of Central and South America and elicits a mixed granulomatous and suppurative response. Appears as a "mariners wheel" microscopically.
120
What are 5 opportunistic fungal infections that occur almost exclusively in immunocompromised patients? (AIDS, leukemia, lymphoma, diabetics, cancer, etc)
Cryptococcus neoformans, Aspergillus fumigatus, Mucormycosis (Zygomycosis), Candida albicans, Pnemocystis jirveci (carnii)
121
What are the top three causes of bacterial meningitis in persons over two months of age?
Meisseria meningitidis, St. pneumonia, H. influenza
122
What is the third most common STD in the United States behind venereal warts and Chlamydia?
Gonococcus caused by Neisseria gonorrhoeae
123
What is the leading cause of meningitis in young children and an important cause of upper RTIs and sepsis in children?
Haemophillus influenza
124
Four diseases associated with this gram negative enteric are diarrhea, UTIs, Neonatal meningitis and Gram negative sepsis
E. coli
125
What gram negative enteric is responsible for causing Typhoid fever (and the accompanying "Rose spots", colored macules on the abdomen?
Salmonella typhi
126
What are four gram-negative enterics primarly found wihtin the enteric tract?
Shigella dysenteriae and sonei, Vibrio cholera, Helicobacter pylori and Campylobacter jejuni
127
What are four gram-negative bacteria found outside the enteric tract?
Klebsiella-Enterobacter-Serratia Group, Proteus-Providencia-Morganella Group, Pseudomonas and Bacteroides
128
What pathogen is responsible for pneumonia with a thick hemorrahgic "current jelly" sputum?
Klebsiella pneumoniae
129
Which Gram-positive cocci contains exfoliatin, a protein which causes "Scalded-skin syndrome" in young children?
Staphylococcus aureus (also contains enterotoxins, toxic shock syndrome toxin, and alpha toxin)
130
What are some inflammatory Staph. Aureus diseases?
impetigo, furuncles, carbuncles, cellulitis, septicemia, endocarditis on normal or prosthetic heart valves, osteomyelitis and arthritis as well as pneumonia (esp. following viral infections)
131
What is the most frequent organism isolated from infected prosthetic devices (joints, heart valves, dialysis catheters) due to the adherence of the polysaccharide capsule?
Staphylococcus epidermidis
132
What are the top two leading causes of UTIs in sexually active young women?
E. coli and Staphylococcus saprophyticus
133
What pathogen is responsible for causing "Strept throat", Scarlet fever, Toxic Shock Syndrome, Acute Rheumatic Fever and Acute Post-Streptococcal Glomerulonephritis
Strept. Pyogenes
134
What are six of the major virulence factors of Group A Beta-hemolytic Strept?
M protein, streptolysin O, erythrogenic toxin, streptokinase, hyaluronidase, TSST toxin
135
What disease and associated pathogen is resonsible for characteristic "strawberry tongue"
Scarlet fever: St. pyogenes
136
What are the three main types of infections associated with viridans group Streptococci?
Dental infections, Subacute bacterial endocarditis, and abscesses
137
What is the most common cause of Pneumonia in adults?
Streptococcus pneumoniae (diagnosed by the Quelling reaction)
138
What is the only bacterium with an antiphagocytic capsure composed of the protein Glutamic acid instead of polysaccharides?
Bacillus anthrasis (aerobic Gram-positive rod)
139
What is the only gram-positive to produce an Endotoxin (LPS)?
Listeria monocytogenes (a gram-positive rod)
140
Although rare today in the U.S. what pathogen has a most prominent sign of adherent gray pseudomembrane over the tonsils and pharynx?
Corynebacterium diphtheriae
141
What spore-forming anaerobic gram-positive rod is responsible for causing Tetanus (lockjaw)?
Clostridium tetani (anaerobic gram-positive rod)
142
Which neural mediator is blocked when Clostridium tetani toxin is carried intra-axonally (retrograde) to the CNS?
GABA-thus the patient presents with violent muscle spasms, Trismus and "Risus Sardonicus"
143
What major pathogen is the reason behind which infants should not be fed honey?
Clostridium botulinum (anaerobic gram-positive rod)
144
What pathogen is responsible for causing Gas gangrene (Myonecrosis)
Clostridium perfringens (anaerobic gram-positive rod)
145
Besides gas gangrene-what other disease is caused by Clostridium perfringens?
Clostridial food poinsoning (the spores are heat-resistant and can produce enterotoxin after germinating in the GI tract)
146
What pathogen (anaerobic gram-positive rod) is responsible for antibiotic-associated Pseudomembranous colitis which can follow the use of broad-spectrum antibiotics?
Clostridium dificile
147
Which branching filamentous organism is associated with IUD use?
Actinomyces israelii
148
According to the CDC, any HIV-infected person is considered to have AIDS when the CD4+ T-cell count drops below what threshold?
<200/mm^3
149
What is the presumptive or standard test for the presence of antibodies to HIV (the antigen)?
ELISA-Enzyme-Linked Immunosorbent Assay
150
What is the confirmatory test for the presence of antibodies to HIV?
Western blot test
151
Albright Syndrome
polyostotic fibrous dysplasia + endocrine abnormality
Precocious puberty
Café au lait spots
Short stature
152
Paget's Disease
Osteitis deformans, Metabolic bone disease
Viral infection-Possibly with mumps, measles, paramyxovirus
Intranuclear osteoclast inclusions
Cotton-wool opacity
New bone is structurally enlarged, but weakened, filled with new vessels
Anemia, ↑↑ alkaline phosphatase, ↑ urine hydroxyproline
Risks: ↑ osteosarcomas
153
Diabetes insipidus
Caused by hypo activity of the posterior pituitary gland-Characterized by large volume of dilute urine
Central or nephrogenic
Central DI: damage to hypothalamus; supraotic nuclei or pituitary (posterior)
Lack or ↓ ADH secretion
Surgery, infection, inflammation, tumor, head injury, rarely idiopathic or genetic
Body fluid tonicity remains close to normal as long as patient drinks enough water to make up for ↑ water clearance in the urine
154
Diabetes mellitus (type I)
Most common pancreatic endocrine disorder
Metabolic disease involving mostly carbohydrates (glucose) and lipids
Causes
Absolute deficiency of insulin (type 1) or
Resistance to insulin action (type 2)
Classic symptom triad
Polydypsia, Polyuria, Polyphagia
155
Bruton's Aggamaglobulinemia
All five immunoglobulins and circulating B-cells are absent or deficient but T-cells are intact. It affects males almost exclusively and causes severe, recurrent infections during infancy. Patients with this disorder are deficient in antibodies and susceptible to repeated infections (mostly bacterial and fungal). This results from the failure of B-cells (B-Iymphocytes) to mature and to differentiate into plasma cells which produce antibodies.
156
Diabetes mellitus (type II)
85%, increased insulin resistance (reduced sensitivity at target cells), strong genetic disposition, ketoacidosis rare, adulthood
