path shelf - deck 2 Flashcards
Myxedema, weight gain, slowing mental activity, muscle weakness, larynx decreased voice, cold intolerance with decreased sweating, oligomenorrhea, hypercholesterolemia
•Labs: Antithyroglobulin
Hashimoto Thyroiditis (CD8/ADCC destruction of TSH R)
•HLA-DR5
•Initially hyperthyroid -> hypothyroid
•Histology: Chronic inflammation with germinal centers and hurthle cells.
•`Complications: Large B Cell Lymphoma
Perioral numbness/tingling, cataracts, mental status changes, tetany chovstek sign, trousseau sign, cardiovascular changes, calcified basal ganglia
•1’ – decreased PTH, decreased Ca2+, increased P
Hypoparathyroidism
• Problem in production with PTH
• Causes: surgery, autoimmune, DiGeorge Syndrome
Perioral numbness/tingling, cataracts, mental status changes, tetany chovstek sign, trousseau sign, cardiovascular changes, calcified basal ganglia
Increased PTH and hypocalcemia : AD disease with short 4th and 5th digit
• Pseudohypoparathyroidism
• Target for DM1
glutamic decarboxylase
• DKA: Diabetic ketoacidosis often with stress/infection
Epinephrine increased glucagon exacerbating lipolysis with gluconeogenesis and glyconeolysis. Increased FFA -> ketone bodies. Hyperglycemia, HIGH anion gap metabolic acidosis, HYPERKALEMIA, kussmal respiration, dehydration, nausea, vomiting, fruit smelling breath, mental status changes. Treatment: fluids
lateral projection of growth plate “mushroom shape” men>women
• As the growth plate expands – there is a lateral projection of cartilage. Surface of cartilage with bone inside.
Osteochrondroma
• Cartilage cap – most common benign bone tumor
• Overlying cartilage can transform chondrosarcoma
Dental abscess causing osteomyelitis?
Staph Aureaus ( most common)
Trauma, weakened immune system and osteomyelitis
Pseudomonas IVDA
osteomyelitis with Black colonies Hektoen Agar – H2S
Salmonella Sickle cell
osteomyelitis with With uvetitis
N. Gonorrhea Reiter Syndrome
osteomyelitis with Vertebral – Lumbar with pain, tenderness
TB Potts Disease
Infectious Arthritis
• Symptoms: fever, single joint involvement (usually knee) which is warm with limited ROM
Reiters syndrome: can’t see, can’t pee, cant climb a tree – urethritis, conjunctivitis, arthritis
S. Gonorrhea Most common
Infectious Arthritis
• Symptoms: fever, single joint involvement (usually knee) which is warm with limited ROM
Older adults
S. Aureus 2nd most common
Infectious Arthritis
• Symptoms: fever, single joint involvement (usually knee) which is warm with limited ROM
<2 years old
Hib
Fever, malaise weight loss, Symptomatic involvement of PIP and MCP which improves thoughout the day, deformity of the hand, joint space narrowing, loss of cartilage
Rheumatoid Arthritis
•Complications: Vasculitis, Bakers cyst (behind knee), lymphadenopathy, Anemia of chronic disease, 2nd amyloidosis SAA-AA
•Labs: IgM autoantibody against Fc portion of IgG, Neutrophils and high protein in fluid
AD – rare form of cancer develops in the retina in children which is often bilateral due to a congenital mutation in chromosome 13 – RB1.
Complications?
Retinoblastoma and complications is osteosarcoma ( due to Rb mutation ) in later teenage life.
Neural Crest Cells
Neural Crest Cells
- PNS neurons, Schwann Cells
Neural tube
Neural tube
CNS neurons, ependymal cells, oligodendrocytes, astrocytes
Mesoderm
Mesoderm
• Microglia
Meningitis
complications?
- Headache, neck stiffness, fever – complications often only occur in bacterial meningitis
- Complications: Hydrocephalus, hearing loss, seizures
bacterial meningitis ?
viral? fungal?
Bacterial: No with low glucose
• Viral: lymphocytes with normal CSF
• Fungal: Lymphocytes with low glucose
meningitis that often presents immediately after birth ( most common in neonates)
GBS
E.Coli
Listeria
Most common in neonates
menigitis - College dorms, military recruits
N. Meningitidis Children and teenagers
meningitis Unvaccinated infants, children
H. Influenza
meningitis Adults, elderly
S. Pneumoniae
early alzheimers is caused by ?
mutation in presinilin 1
sporadic alzheimers is caused by ?
Decreased risk with?
- Sporadic: Changes increase risk with age and APOE4 Allele
* Decreased risk with APOE2
alzheimers pathologic feaures?
Pathologic features: Diffuse cerebral atrophy of the brain – narrowing of gyri and widening of sulci
alzheimers histology?
Histology: AB amyloid deposit (amyloid is extracellular) derived from APP – cerebral angiopathy causes increased risk of hemorrhage into the brain, neurofibrillary tangles (flame shape tail) with Tau protein
Polycystic Kidney Disease (bilateral kidneys are affected)
AR vs AD?
• AR – “juvenile” Potters Sequence – oligoydramnios.
o Associated with hepatic cysts with congenital hepatic fibrosis. With signs and symptoms of portal HTN.
• AD – young adults mutation in APKD1/APKD2 due to polycystin abnormality
o HTN, hematuria, and worsening renal failure
o Complications include berry aneurysm, hepatic cysts, and mitral valve prolapse (mid systolic click).
WBC casts in urine
Symptoms: fever, flank pain
Pyelonephristis
Common in vesicureflux disease – chronic scarring and interstitial nephritic. Cortical scarring (upper and lower)
common UTI infection which does not have WBC casts in urine. – pelvic pain, dysuria, fever
Cysitis
o E.coli most common UTI infection
patient taking : Ps – Pain free (NSAIDs), Penicillin, Pee (Diuretic), riPampin Symptoms: Oligouria, fever, rash disease? labs? histology? complications?
Acute interstitial nephritis
• Drug induced hypersensitivity reaction –
• Labs: eosinophils in the urine
• Histology: interstitial infiltrates
• Complications: renal papillary necrosis – gross hematuria, flank pain
Presents with classic triad of
1- hematuria
2- palpable mass
3- flank pain
also : fever , weight loss or paraneoplastic syndrome ( EPO, renin, PTHrP or ACTH )
GROSS- Yellow mass
Metastatic renal cell carcinoma
- rarely presents with left side varicocele
involvement of left renal vein by carcinoma blocks drainage of the left spermatic vein leading to varicocele
renal cell carcinoma pathogenesis
and cells?
-mass yellow and clear cytoplasm ( clear cell type)
-involves loss of VHL (3p) tumor suppressor gene which leads to
1- Increased IGF-1 ( Promotes growth0
2- increased HIF transcription factor ( increases VEGF and PDGF)
younger patient with tumor in the kidneys
von- hippel lindau disease is an autosomal dominant disorder associated with inactivation of the VHL gene leading to increased risk for hemangioblastoma of the cerebellum and renal cell carcinoma.
: Smoker - Napthylamine, Azo dye, Long term cyclophosphamide and phenacetin use
o Histology:
Papillary: low grade -> high grade -> invade;
Flat: starts a high grade tumor with early p53 mutations
o Symptoms: Painless hematuria
transitional/ urothelial bladder carcinoma
RF: chronic cystitis, Schistosoma hematobium – middle east, long standing nephrolithiasis (kidney stones)
Squamous Cell Carcinoma
bladder adenocarcinoma
causes?
presents most likely on what part ?
Urachal remnant – duct that connects fetal bladder to umbilical cord. Remnant can become adenocarcinoma which is present at the dome of the bladder
Cystitis glandularis
Exstrophy – failure for bladder to migrate
Unilateral Hydronephrosis
•Most common causes?
Most common cause KIDNEY STONES
• Congenital pelvic ureteric junction stenosis
• Tumor infiltrating the ureter
• Ureterocele – sac like opening located in bladder (congenital)
• Schistosomiasis
• Bladder tumor
Thrombocytopenia
•Labs: Decreased platelets, normal PT/PTT,
increased megakaryocytes
No schistocytes!
ITP : Immune thrombocytic Purpura
•Autoimmune destruction of IgG against platelet antigens (GP2b3a). Ab produced by plasma cells in the spleen and consumed by splenic macrophages cause acute thrombocytopenia.
•Acute: children weeks after viral infection which will resolve within several weeks
•Chronic: women of child bearing age – often associated with SLE (lupus), often secondary cause of ITP. When they become pregnant, IgG can cross the placenta which can cause thrombocytopenia in the offspring.
•Most common cause of thrombocytopenia in adults and children
• Treatment: Corticosteroids – adults often relapse, IVIG causes splenic macrophages to eat these over the platelet bound Ab. Effect is short lived. Splenectomy: Eliminate source and site of destruction
Schistocytes: Microangiopathic hemolytic anemia
Pathology?
- formation of platelet microthrombbi in small vessels
- platelets are consumed in the formation of microthrombi, resulting in hemolytic anemia with schistocytes
seen in TTP and HUS
TTP :
TTP : Thrombotic Thrombocytopenia Purpura
• Blood vessel causing platelet microthrombi in small blood vessels due to decreased ADAMTS13 or VWF metalloprotease. Unable to degrade multimers which leads to abnormal platelet adhesion -> which leads to schistocytes. Cause: Antibody to ADAMTS13 – adult female.
Symptoms: skin and mucosal bleeding, fever, microangiopathic hemolytic anemia, fever, renal insufficiency, CNS abnormalities
HUS: Hemolytic Uremic Syndrome
Childhood with E.Coli O157H7 from uncooked beef or shiga like toxin. Results in a toxin which damages platelet microthrombi. With damaging to the endothelial cells which leads to decreased ADAMTS13 leading to very similar presentation to TTP. Causing shistocytes.
Labs: Increased PT/PTT, Decreased fibrinogen, platelet count,
DIC – Disseminated Intravascular Coagulation
=Elevated fibrin split products (D-Dimer)
pregnant woman or recently gave birth Symptoms: SOB, neurologic changes, DIC
Amniotic Embolism
• Amniotic fluid goes into mothers lungs during liver
• Histology: Amniotic fluid loaded with TISSUE THROMBOPLASTIN characterized by squamous cells and keratin debris from fetal skin in embolism.
CML
9;22
• Philadelphia Chromosome: BCR-ABL seen in CML and ALL
• CML: LAP negative, increased basophils
Reid Sternburg CD15/30+ with owl eyes
Hodgkin Lymphoma
• Reid Sternburg CD15/30+ with owl eyes – increased eosinophils (IL-5)
- Infection causes increased WBCs
- Increased LAP
- Normal basophils
Leukemoid Reaction (acute infection)
VS CML - 9;22
• Philadelphia Chromosome: BCR-ABL seen in CML and ALL
• CML: LAP negative, increased basophils
Normocytic Anemia (80-100 MCV)
RT: Larger than RBCs with slightly more blue cytoplasm due to presence of RNA in cytoplasm. In a normal patient RT 2%.
Normocytic Anemia (80-100 MCV) Decreased RT count – underproduction <3% correct by RT x HCT/45
o ParvoB19
o Aplastic Anemia
o Myelophthisic Process= Pathologic process that replaces bone marrow – usually cancer
Normocytic Anemia (80-100 MCV) Normal RT count – peripheral destruction >3% correct by RT x HCT/45 - EXTRAVASCULAR VS INTRAVASCULAR
o Extravascular hemolysis: Excess bilirubin (unconjugated) – jaundice, supersaturation of bilirubin in bile -> gallstones, marrow hyperplasia with corrected RT count 3%.
VS
o Intravascular hemolysis: Destruction of RBCs within vessels -> hemoglobinemia, hemoglobinuria, hemosiderinuria (hemoglobin taken up by renal tubule cells – several days later with high iron). Decreased haptoglobin.
Normocytic Anemia (80-100 MCV) Normal RT count – peripheral destruction >3% correct by RT x HCT/45 - EXTRAVASCULAR EXAMPLES
1. Hereditary Spherocytosis
2. Sickle Cell Disease
3. HbC (C = lyCEEEine)
Normocytic Anemia (80-100 MCV)
Increased RDW, MCHC
Osmotic fragility test – Increased fragility in hypotonic solution -> burst
Hereditary Spherocytosis
• AD: RBC cytoskeleton which involve spectrin, ankyrin, band 3.1. Blebs are removed by the spleen losing membrane which causes the cell to become round. Consumed by the spleen -> biggest problem. Treatment = splenectomy -> Howell Jolly Bodies emerge on RBCs
• Complication: Increased risk of aplastic anemia with B19 infection
Normocytic Anemia (80-100 MCV) SYMPTOMS: Jaundice, crewcut appearance – expansion of hematopoiesis, extramedullary hematopoiesis with hepatomegaly • Histology: Target cells, Howell Jolly bodies if autosplenectomy
Sickle Cell Disease
• AR mutation in B chain of Hb glutamic acid is replaced with valine. 1 abnormal gene = <50% HbS production leading to sickle cell trait which don’t usually sickle. Renal medulla – with drecreased ability to concentrate the urine.
• 2 abnormal genes produces >90% production of HbS. HbS polymerizes when it is deoxygenated which allows them to aggregate. This is reversible. Increased risk hypoxemia, acidosis, dehydration. HbF is protective doesn’t present until 6 months old. Treatment: Hydroxyurea increased HbF (we don’t know mechanism)
• Complication: Membrane damage to RBCs – extravascular hemolysis causing anemia, jaundice, unconjugated hyperbilirubinemia, decreased haptoglobin.
• Vasoocclusive infarction: Dactylitis – swollen hands and feet in infants (6 month old), autosplenectomy – most common cause of death encapsulated organisms, increased risk for salmonella. Howell jolly bodies on RBCs. Acute Chest syndrome: usually co-occurs with pneumonia causing vaso-occlusive in pulmonary microcirculation. Most common cause of death in adults.
Normocytic Anemia (80-100 MCV) EXTRAVASCULAR
HbC (C = lyCEEEine)
HbC Crystals on RBCs
Normocytic Anemia (80-100 MCV)
Decreased haptoglobin.
INTRAVASCULAR HEMOLYSIS
Labs: Hemoglobinemia, hemoglobinuria, hemosiderinuria
PNH
• Acquired defect in myeloid stem cells resulting in absent GPI protein protects against complement which renders cells susceptible to complement. Due to absence of DAF/MIRL CD55 on RBC. Mild respiratory acidosis at night during shallow breathing activates complement causing cells to lyse.
• Labs: Hemoglobinemia, hemoglobinuria, hemosiderinuria
• Test: sucrose test, CD55 (DAF) lack on RBCs
• Complement: Thrombosis in hepatic, portal, or cerebral veins – platelets lack GPI release cytoplasmic contents into circulation, iron deficiency anemia, AML (10%) – mutation in myeloid stem cell.
Normocytic Anemia (80-100 MCV)
Decreased haptoglobin.
INTRAVASCULAR HEMOLYSIS EXAMPLES
1- PNH
2- G6PD deficiency
3- immune hemolytic anemia
Normocytic Anemia (80-100 MCV)
Decreased haptoglobin.
INTRAVASCULAR HEMOLYSIS
symptoms: back pain, hemoglobinuria – hours after exposure
• Histology: Heinz bodies, bite cell
• Test: Heinz preparation – after resolution of episode.
G6PD deficiency
• RBCs live in environment of oxidative stress. They use glutathione which protects against oxidative stress. Reduced half life. African vs Mediterranian – Protective role against M. Falciparum.
• RF: Infections, drugs, fava beans, sulfa drugs
Normocytic Anemia (80-100 MCV)
Decreased haptoglobin.
Immune hemolytic anemia
Immune hemolytic anemia
• Warm - IgG: Extravascular Hemolysis
o RBCs bind in warm areas of the bodies causing spherocytosis.
o Associated: SLE, CLL, drugs
• Cold – IgM: Intravascular Hemolysis
o Binds RBCs fixes complement with cold temperature
o Association: Mycoplasma pneumoniae, infectious mononucleosis
BPH- it effects the ?
BPH- it effects the periurethral zone
post- digital rectal exam ID’s prostate cancer in a male, what morphology would classify a worse prognosis?
- disorganized growth = loss of polarity
- nuclear pleomorphism
- high nuclear to cytoplasmic ratio ( dark blue nuclei)
- high mitotic activity with atypical mitosis
- invasion through basement membrane
Gastric reflux-
causes lower esophageal epithelium to become abnormal squamous before it becomes metaplastic as seen in barretts esophagus
pregnant woman has polyhydraminos and the fetus has 47 XY (+21 chromosome)- the cause of the polyhydraminos?
the baby has duodenal atresia
mousy/musty” smell, intellectual disability, when testing the urine, it turned blueish green
male with one swollen scrotum and pain just above on the same side, what could be the problem
PKU-
man has unilateral hydronephrosis what is his problem?
mass at the vsicoureteral junction, has calculi in the urethra, bladder cancer, …
Adult PKD patient has what extra-renal manifestation
either (berry ) aneurism or hepatic cysts or mitral valve prolapse (mid systolic click
Picture of adult _____kidney and asks what the other kidney would look like?
Decreased in size
mother brought her 14 year old daughter to the doctor bc she has not menstruated yet; examination reveals breast, normal hair growth, a vagina that ends in a blind pouch, and no ovaries or uterus- options were: 45XO, 47XXY, 46XX, or 46XY
46XY (we believe its androgen insensitivity, which points to 46XY)
PCOS presents with what lab value?
PCOS presents with elevated LH
an alcoholic male with bright red blood in his stool has hemorrhoids; what is the lining of the vasculature that is leaking blood?
endothelial cells (but not sure thats right)
**patient comes in with…….and endoscopy revealed a thinning, (and i think it said soft) gastric wall/mucosa and her labs showed an elevated hematocrit, normal bone marrow cells and in normal proportions, they wanted to know what her problem was?
i said pernicious anemia bc it was leading towards a B12 deficiency…or is this the renal question…
picture of cells: one i think were smudge cells and asked what the patient had-
i said CML, but they also had CLL, AML, ALL, and all so just know their histology
exposed to hydrocarbons, benzene, and etc, what test will be abnormal with respect to the hydrocarbon exposure?
chest x-ray
in a person with Shock- what is reason for low blood pressure/vol?
increased space btw endothelial cells is reason for low blood pressure/vol
what are the nodules on the cirrhotic liver of a patient with chronic hepatitis C- (in Dr. cones liver review)-
regenerative regions
Normally molecule X binds damaged DNA and causes the cell to enter the G0 phase, an experiment is done and found that a mutant version of molecule X wont bind damaged DNA leading to uncontrolled cell growth. this molecule X is most similar to_____?
Tumor suppressor genes
Hashmotos hypothyroidism
Hurthle cells- CD8 destruction
DiGeorge Syndrome
hypoparathyroidism (kid had hypocalcemia)