Things I struggle with Part 2 Flashcards
what two anterior pituitary hormones are derivatives of POMC
ACTH and MSH
what are the anterior pit hormones with the same alpha subunit
TSH, LH, FSH, hCG
what does the beta subunit of a hormone determine
it determines the hormone specificity
what hormone is secreted from the intermediate lobe of the pit
MSH
what is the anterior pit derived from
rathkes pouch which is oral ectoderm
what is the posterior pit derived from
neuroectoderm
what are the alpha cells, beta cells, and delta cells of the pancreas and where are they located within the pancreatic buds
alpha- glucagon- peripheral
beta- insulin- central
somatostatin- interspersed
what are the effects of glucagon
it does glycogenolysis, and gluconeogenesis, and lipolysis and ketone production
what regulated glucagon secretion and what suppresses it
it is secreted in response to hypoglycemia, and it is inhibited by insulin, hyperglycemia, and somatistatin
what is the antagonist of prolactin
dopamine
tesamorelin- what does it do
it increases GH and is used for AIDs lipodystrophy
what does increased prolactin decrease
GNRH so FSH AND LH
what does tonic GNRH do
it surpasses the HPG axis
what does pulsatile GnRH do
it leads to puberty and fertility
what are somatostatin analogs used to treat
GI bleeding and acromegaly
what does TRH increase
prolactin and TSH
prolactin function
increases milk production and stops ovulation by decreasing the GnRH production
what can first or second degree hypothyroidism do to prolactin
it can increase prolactin because of the increase in TRH
what does bromocriptine do to prolactin
it decreases the production because it increases the DA
what does antipsychotics and OCP and Pregnancy do to prolactin
it increases the prolactin because of the decrease of dopamine
what is the function of GH
it stimulates linear and bulky growth through the stimulation of production of IGF-1 by the liver
what can excess IGF1 lead to
it can cause gigantism but it leads to insulin resistance
when is there increased secretion of GH
exercise, deep sleep, puberty, and hypoglycemia
when is there decreased secretion of GH
from glucose of r somatostatin
ghrelin function
stimulates hunger and GH and is released from the stomach
what increases ghrelin
Prader Willi or sleep deprivation
function of leptin
satiety hormone
what decreases leptin
decreased sleep or starvation
what does a mutation in the leptin gene do
congenital obesity
what does leptin do to the lateral thalamic nucelus
it inhibits it, which if there is a lesion then there is anorexia
what does leptin do to the ventromedial thalamic nucelus
it stimulates it so if there is a lesion then there is hyperphagia
endocannabanoid function
act at hypothalamus and nucleus accumbent to increase hunger
where is ADH from in the thalamus
supraoptic nuclues
what is the function of ADH
it regulates osmolaitity and bp. increases urine osm and decreases blood osm
which receptors are for serum osmolality
V2
which receptors are for BP regulation by ADH
V1
where does ADH act specifically in the kidney
CT it adds aquaporins to rescue water
what happens to the ADH level in central DI and nephrogenic DI
in central DI there is decreased ADH and in nephrogenic theres increased ADH
what can cause neprhogenic DI
Lithium blocking aquaporins or mutation in V2 receptor
desmopressin acetate- use
ADH analog for treatment of central DI and nocturnal enuresis
what regulates ADH
osmoreceptors and hypovolemia
cortisol functions
increase blood pressure, insulin resistance, gluconeogenesis, lipolysis, proteolysis, decreased fibroblast activity (striae), decreased inflammatory and immune responses- inhibit neutrophil adhersion, blocks histamine, reduces eosinophils, blocked IL2 production, inhibits leukotrienes and prostaglandins, decreased bone formation
how does cortisol increase BP
increased alpha 1 receipts on the arterioles which increases the sensitivity to NE and epi
at very high concentrations what other receptors can cortisol stimulate
aldosterone receptors
why can cortisol reactivate TB
it blocks IL2 which is keeping the TB and candida in check
what regulates cortisol production
CRH stimualtes ACTH release and cortisol production. chronic stress induces it
increase in Ph does what to Ca homeostasis
it increases the affinity for albumin which draws Ca out of the free pool causing hypocalcemia effects
what does active vitamin D do
increases absorption of Ca and Po4 and enhances bone mineralization
what regulates vitamin D
increased PTH, decreased Ca, decreased phosphate, increase the production of vitamin D.
Parathyroid hormone function
increased bone resorption of Ca nada phosphate, increased kidney resorption of Ca in DCT, decreased resorption of phos in PCT, increased activation of vitamin D in PCT
where does PTH increase Ca respiration in kidney
DCT
where does PTH decrease phosphate reabsortion in the kidney
PCT
where does PTH increase vitamin D production in the kidney
PCT
what does increased RANKL do
it is secreted by osteoblasts and osteocytes which then bind to receptor in the class which breakdown the bone
which cancers produce parathyroid like hormone
renal cell carcinoma and squamous cell cancer of the lung
what increases PTH secretion
decreases Ca, increased phosphate, decrease Mg.
what happens if there is severely low Mg
it decreases PTH
what eznyme activates vitamin D in the kidneys
1 alha hydroxylase
what causes decreased Mg
diarrhea, aminoglycosides, diuretics, alcohol abuse
what does calcitonin do
oppose PTH and is from the parafollicular cells so it decreases resorption of bone
what are the functions of T3
brain maturation, bone growth, beta adrenergic, basal metabolic rate
what does thyroid hormone do to the heart
it increased B1 stimulation so increased CO, SVR, HR, and contractility
what does thyroid horomone do the BMR
increased Na/K ATPase activity so increased O2 consumption and RR and body temperatuer
what metabolic functions does thyroid hormone increase
glycogenolysis,gluconeogenesis, lipolysis
what increases TBG
pregnancy, estrogen, OCP- increased total T4 so less active hromone
what decreases TBG
it is decreased hy hepatic failure, steroids
wat converts T4 to T3
5 deiodinase
what is the wolff chaikoff effect
it is excess iodine temporarily inhibits thyroid peroxidase and decreases iodine organification and decreases T3 and T4 overall
What does thyroid peroxidase do
it oxidizes and organifies iodide as well as ccoupling MIT and DIT to form T3 and T4.
what does propylthiouracil do
it inhibits thyroid peroxidase and 5 diodinase
what does methimazole do
inhibits thyorid peroxidase only
cAMP pathway is which hormones
FSH, LH, ACTH, TSH, hCG, ADH, MSH, PTH, calcitonin, GHRH, glucagon
cGMP pathway is which hormones
BNP, ANP, EDGF (NO)
IP3 pathway is which hormones
GnRH, oxytocin, ADH, TRH, histamine, angiotensin II, gastrin
intracellular receptor pathway is which hormones
progesterone, estrogen, testosterone, cortisol, aldosterone, t3,t4, vitamin D
tyrosine kinase pathway pathway is which hormones
insulin, IGF, FGF, PDGF, EGF
JAK-STAT pathway
prolactin, IL2, IL6, IFN, GH, G-CSF, eryhtropoetin, thrombopoetin
what happens with increased sex binding globulin
gynecomastia from decreased free testosterone
what happens with decreased sex binding globlin in females
it raises free T and leads to hirsuitism
what increases sex binding hormone in females
pregnancy and OCP
what are sings of adrenal insufficiency
fatigue, weakness, hypotension, muscle aches, weight loss, GI disturbance, sugar and salt craving
primary adrenal insufficiency- what are the signs
hypertension from hyponatremic volume contraction, metabolic acidosis, increased potassium, and mucosal hyper pigmentation from the excess ACTH creating increased MSH.
what is the cause of acute primary adrenal insufficiency
sudden onset due to massive hemorrhage. Leads to massive shock and adrenal crisis- can be from waterhouse freinderichson with shock and DIC
chronic primary adrenal insufficiency
Addison disease- adrenal atrophy or destruction by autoimmune disease- can be destroyed by TB
secondary adrenal insufficiency
seen with decreased ACTH production. NO hyperkalemia or mucosal pigmentation- still get hypotension.
tertiary adrenal insufficiency
seen with withdrawal of long term steroids
hyperaldosteronism
increased secretion of aldosterone from adrenal gland leading to HTN, decreased or normal K and metabolic alkalosis but not edema
primary hyperaldosteronism
adrenal adenoma or conn or can be hyperplasia- get increased aldosterone and low renin
secondary hyperaldosteronism
see in patients with renovascular hypertension or JG cell tumor with increased aldosterone and renin, so there is no shut off of renin with aldosterone
neuroblastoma
adrenal medulla tumor of a child under 4 from neural crest cells can be along the sympathetic chain. It is a firm irregular mass that can cross the midline- causes dancing eyes and dancing feet
what is the different presentation between neuroblastoma and Wilms tumor
Wilms tumor is smooth and unilateral and neuroblastoma is firm, irregular and can cross the midline
what do you seen on labs for neuroblastoma
increased HVA and VMA and Homer wright rosettes with NSE and bombazine positive
what is the oncogene mutation in neuroblastoma
n-myc
pheochromocytoma
tumor of adrenal medullary chromatin cells which are from the neural crest
what mutations are pheochromocytoma associated with
NF1, VHL, RET,
what is the rule of 10s
10% are : malignant, bilateral, extra-adrenal, calcify, kids
what are the symptoms of pheochromocytoma
secrete NE and pi which epidoside hypertension, increased BP, headache, perspiration, palpitation, pallor
treatment for pheochromocytoma
use irreversible alpha antagonists like phenoxybenzamine followed by beta blocker
what should you not give first to treat pheochromocytoma
do not give beta blocker first it can create hypertensive crisis
hypothyrodism vs hypertthyroidism myopatyh
increased CK in hypothyroidism
what causes a smooth goiter
graves, hashi, iodine def, TSH adenoma
what causes a nodular goiter
toxic multinodular goiter, thyroid adenoma, thyroid cancer, cyst
hypoparathyroidism cause
due to accidental excision of parathyroid glands, autoimmune destruction, or Digeorge, tetany, hypocalcemia, hyperphosphatemia
Pseudomhypoparathroidism type 1A
unresponsiveness of kidney to PTH leading to decreased Ca with high PTH. can also see short 4/5 metacarpals, short stature. autsomal dominant with defect in Gs alpha subunit causing end organ damage resistance to PTH due to maternal imprinting
pseudopseudohypoparathyroidism
albright osteodystrophy but without end organ PTH resistance. Just from paternal Gs defect in alpha subunit.
familial hypocalciuric hypercalcemia
defective Ca sensing receptor in the kidneys and parathrougids so really high Ca levels cause the decrease in PTH so see high PTH to normal PTH with Increased CA- excessive renal repute of Ca
primary hyperparathrouid
usually due to adenoma or hyperplasia- hypercalcemina and uric- decreased phosphate. increased PTH- weakness, constipation, kidney stones, pancreatitis, depression can get bone lesions and peptic ulcers
secondary hyperparathyroidism
decreased Ca absorption and or increased phosphate from CKD and this leads to hypocalcemia, hyperphosphatemia in chronic renal failure.
tertiary hyperparathyroidism
hyperparathyroidism from chronic renal disease s increased Ca and PTH
osteitis fibrosa cystica
cystic bone lesions from osteoclasts and hemosiderin. hits the cortical bone, subperiodsteal thinning, subperiosteal in the hands and salt and pepper head
pituitary adenoma most common and how to treat
prolactinoma from the lactotrophs- use bromocrpitine
mass effect of pituitary
bitemporal hemianopia, hypopituitarism, headache
Nelson syndrome
elargement of an ACTH adenoma after the removal of adrenals and it removes the feedback so it gets bugger- get hyperpigm, headache, and bitemporal heminanopia
acromegaly
excess GH in adults- glucose tolerance
what is at increased cancer risk in acromegaly
colon polyps and cancer
what is the cause of death in gigantism
heart failure
what do you see with acromegaly labs
increased IGF1, failure to suppress serum Gh following oral glucose test
Laron syndrome- dwarf
decreased response to growth hormone because f lack of receptors- see increased GH and decreased IGF-1
diabetes insipidis Central
pituitary tumor, autoimmune, trauma, surgery, ischemic encephalopathy, synthesized in paraventricular and supraoptic nuceli- decreased ADH< but does respond to desmopressin
diabetes insipidis nephrogenic
hereditary ADH receptor mutation, can be second degree to hypercalcemia, hypokalemia, lithium, demeclocycline. Normal or increased ADH. cannot change in osmolality with administration of desmopressin.
treatment of nephrogenic DI
HCTZ, indomethacin, amiloride, hydration, avoid the offending agent
where does the lithium effect the nephrogenic DI
it hits the CT and it is normally excreted at PCT
what happens if the defect is in the hypothalamus
it is then permanent
SIADH- what are the characteristics
excessive free water retention, euvolemic hyponatremia with continuous urinary NA excretion, urinary osmolality>serum osmolality
SIADH physiological effects
decreased aldosterone, and increased ANP and BNP. There is increased NA secretion, normalization of extracellular fluid volume. euvolemic hyponatremia. Very low serum Na. This leads to cerebral edema, seizures. Correct to prevent demyelination syndrome
what can happen if there is rapid correction of hyponatreima
central pontine myelinolysis
what are the causes of SIADH
ectopic ADH (small cell lung cancer), CNS disorder or head trauma, pulmonary disease, drugs like cyclophosphamide.
Sheehan syndrome
ischemic infarcto f the pituitary following postpartum bleeding. Pregnancy induces pituitary growth, which then increases sociability to hypo perfusion. Usually presents with failure to lactate, absent menstruation, and cold intolerance
empy sella syndrome
atrophy or compression of pituitary which lies in the sella truck often idiopathic common in obese women
pituitary apoplexy
sudden hemorrhage into the pituitary gland often in the presence of an existing pit adenoma, usually presents with sudden onset severe headache, visual impairment,bitemporal hemianopsia, diplopia due to CN III palsy, feature of hypo pit can get cardiac collapse due to ACTCH and adrenocortical insufficiency
diabetes mellitus symptoms
polydipsia, polyuria, polyphagia, weight loss.
chronic complications of Dm- non enzymatic glycosilation
- nonezymatic glycosilation- small vessel disease- retinopathy, hemorrhage exudates, micro aneurysms, vessel proliferation, glaucoma, neuropathy, nephropathy and Kimmelsteil wilson nodules. progressive proteinuria, arterioloscrlosis, and hypertension
- large vessel issues of DM_ atheroscelorisis, CAD, peripheral vascular disease, gangrene, limb loss, cerebrowvascualr disease- MI is most common cause of death
what can be used to protect the kidneys from DM after microalbuminemia is started
use the ACE inhibitors
what is the osmotic damage from DM
sorbitol accumulation in organs with aldolose reductase and decrease or absent sorbitol dehydrogenase. Neuropathy, stocking glove, and autonomic degeneration, cataracts
glucagonoma
tumor of pancreatic alpha cells- overproduction of glucagon. Presents with dermatitis (narcoleptic migratory erythema), diabetes with hyperglycemia, DVT, declining weight, depression
treatment for glucagonoma
octreotide, and surgery
insulinoma
tumor of pancreatic beta cells, overproduction of insulin- hypoglycemia- may see whipple triad- hypoglycemia, lethargy, syncope, diplopia, resolution after normalization of glucose- decreased blood glucose and increased C peptide. some are associated with MEN 1
somatistatinoma
tumor of pancreatic delta cells,overproduction of somatistatin, decrease secretion of secretin, cholecystokinin, glucagon, insulin, gastrin- may present with diabetes, glucose intolerance, steatorrhea, gallstones.
treatment of somatistatinoma
somatistain analogs- octreotide and surgery
carcinoid syndrome
neuroendocrine tumor of rosettes, metastatic small bowel timers which secrete high levels of serotonin. not seen if the tumor is in the GI tract only. See diarrhea, flushing, asthmatic wheezing, right sided valve disease, tricuspid regurgitation, pulmonic stenosis, increased 5HIAA in the urine. Niacin deficiency and pellagra
treatment for carcinoid syndrome
surgical resection, somatistatin analog
rule of 1/3 with carcinoid syndrome
1/3 met, 1/3 have second malignancy, 1/3 are multiple- most common malignancy of the small intestine
pancreatic endocrine tumor, pituitary tumors, parathyroid adenoma- condition and mutation
MEN 1- mutation in menin and tumor suppressor gene on chromosome 11
parathyroid hyerplasia, medullary thyroid carcinoma, pheochromocytoma- condition and mutation
MEN 2A- ret mutation codes for receptor tyrosine kinase in cells of neural crest origin
medullary thyroid cancer, pheochromocytoma, mucosal neuromas, marfanoid- condition and mutation
MEN 2B- ret mutation
what develops if the lateral folds fail to close
omphalocele or gastroschisis
what develops if the caudal folds fail to close
bladder extrophy
what week foes the gut herniate through the umbilical ring
6
what week does the gut return and what does it rotate aroudn
it rotates around the SMA and its 10 weeks
extrusion of abdominal contents through abdominal folds- uncovered bowel
gastroschisis
persistence of herniation of abdominal contents into umbilical cord sealed by peritoneum
omphalocele
what is the most common type of TE
esophageal atresia with distal fistula
what are the symptoms of TE
drool, choke, on vomit with first feeding, air in the stomach on the stomach CX and cyanosis from laryngospasm and failure of NG tube to hit stomach
duodenal atresia
gailure to recanalize and dilation of stomach and proximal duodenum- associated with downs
what do intestinal atresias present with
bilious vomiting, and abdominal distention within the first 1-2 days of life
jejunal and ileal atresia
disruption of the mesenteric vessels leading to ischemic necroses and segmental reposition with bowel disontinuity- apple peal
olive mass, projective vomiting- what is it, patients typical, and what does it lead to with electrolytes
hypertrophic pyloric stensosis- it is first born males at 2-6 weeks old. It can be from macrolide exposure. It is hypokalemic, hypochloremic metabolic alkalosis from the vomiting
what forms the ventral bud of the pancreas
uncinate and pancreatic duct
what forms the dorsal bud of the pancreas
it is the body, till, isthmus, and accessory pancreatic duct
what does the annular pancreas form from and what does it strangulate
it encircles the second part of the duodenum, and it causes non-bilious vomiting- ventral buds encricle
what is pancreas divisum
it is where the dorsal and ventral buds final to fuse and they are usually asymptomatic or might have chronic pancreatitis
what is the embryonic origin of the pancreas and spleen
pancreas- endoderm
spleen- mesoderm
what are the retroperitoneal structures
adrenals, IVC, arota, duodenum (2-4), pancreas, ureter, colon, kidneys, esophagus,rectum
what part of the pancreas, colon, and duodenum are in the retroperitonem
pancreas- all but tail
colon- ascending and descending
duodenum- 2-4
how can blood get in the retroperitoneum during a cardiac procedure
it can be from the common femoral vein
where is a filter placed to prevent DVT from hitting the lungs in people who can’t take anticoagulants
in the IVC
on a transesophageal echo what is on the anterior and was is posterio
anterior- left atrium
posterior- descending aorta
if blaming the hepatoduodenal ligament does not stop bleeding where is the issue
IVC or hepatic vein
what is in the portal triad and what ligament is it associated with
proper hepatic artery, portal vein, common bile duct
what position of the gut wall has the meissner plexus and what has the myenteric plexus
meissener plexus- submucosa
myenteric plexus- muscularis externa
what is the difference between ulcers and erosions
ulcers erode into the submucosa and muscle layers, and erosions are mucosa only
where are the brunner glands and what are their purpose
they secrete bicarb and they are in the duodenum
where are the peyers pathcs
ileum
where are the largest number of goblet cells in the small intestine
ileum
what branches are at T12, L1, L2, L3, L4, L5
t12- celiac
L1-L2- renal gonadal, and SMA
L3- IMA
L4 bifurcation of the aorta
intermittant intestinal obstruction with postprandial pain what is compressed and what is compressing it
SMA is compressing the 3rd segment of the duodenum- this is the transverse part of the duodenum
esophageal anastomosis- clinical signs and what is the shunt
esophageal varices- left gastric to the azygous vein
umbilicus- clinical signs and what is the shunt
caput medusa, and paraumbilical to small epigastric veins of the anterior abdominal wall
rectum- clinical signs and what is the shunt
anorectal varices- superior rectal to middle and inferior rectal
when are these varices usually seen
portal HTN
treatment for varices
transjugular intrahepatic portosystemic shut between the portal vein and hepatic vein relieves the portal HTN by shunting the blood to the systemic circulation, bypassing the liver
what supplies about the pectinate line
superior rectal artery from the IMA- it drains to the portal ciruclation
what are the symptoms of internal hemorids
bright red blood with no pain
what is the lymph drainage about the pectinate line
it is to the internal iliac lymph nodes
what is the arterial suppliy and venous drainage below the pectinate line
it is inferior rectal artery off the internal pudendal artery and the venous drainage is to the IVC
what does the pudendal nerve supplie
perinemum and external gentialia with touch, temperature and pain.
what are the symptoms of external hemorids
painful from pudendal nerve and can blled
what is the lymph drainage of below the pectinate line
superficial inguinal nerves
anal fissure
pectinate line below, pain while pooping, blood, posterior because of poor perfusion from low fiber and constipation
what are kipper cells
specialized macrophages of the liver that line the sinusids
what do hepatic stellate cells store
vitamin A and can produce extracellular matrix
zone 1 liver - where is it, what affects it
periportal so near blood supply and it is affected by viral hepatitis, and ingested toxins like coke
zone2 liver- - where is it, what affects it
intermediate zone is hit by yellow fever
zone 3 liver- - where is it, what affects it
it is the pericentral centrilobular zone near the drain point- it is affects by ischemia, it houses the cytochrome p450 system and it is sensitive to metabolic toxins and it is the site of alcoholic hepatitis
where is the P45 site
zone 3
what is the flow of bile and blood through the zones
blood- 1-3
bile- 3-1
where would be the gall stone if it is causing cholangitis and pancreatitis
it is in the ampulla of vater
what can tumors in the head of the pancreas obstruct
common bile duct leading to painless jaundice
from lateral to medial what is the organization of the vessels of the femoral region
nerve, artery, vein, lymphatics (NAVeL)
what is in the femoral triangle
femoral nerve, artery, vein
what is in the femoral sheath
femoral nerve and artery and canal with deep inguinal LB but NOT the nerve
what side is the congenital diaphragmatic hernia on and what is it from
congential defect of pleuoperitoneal membrane occurs on the left side due to relative protects of the right side by the liver-
what is a hiatal hernia
it is where the stomach herniates through the esophageal hiatus
sliding hiatal hernia
LES moves up and there is an hourglass stomach
what is a paraesophageal hernia
gastroesophageal junction is usually normal and the funds protrudes into the thorax
indirect inguinal hernia- typical patients, where it dos and what is the origin of it
failure of the processes vaginalis so it can have a hydrocele as well. It enters at the external inguinal ring and internal ring and it slides into the scrotum and it is lateral to the inferior epigastric vessels. It is covered in all three layers of spermatic fascia
direct inguinal hernia
protrudes through the inguinal triangle and bulges through the abdominal wall medial to the inferior velds. It goes through the superficial ring and it is covered by external spermatic fascia
femoral hernia
protrudes below the inguinal ligament through the femoral canal below and lateral to the pubic tubercle. It is medial to the femoral vein and lateral to pubic tubercle it is inferior to the inguinal ligament- it is more likely to present with incarceration or strangulation
what forms the Hesselbach triangle
medial is lateral rectus
top- inferior epigastric vessels
lateral- inguinal ligament
gastrin is from, action, regulation
G cels of the antrum of the stomach and duodenum, and it increased gastric H secretion and increases growth of the gastric mucosa, and increases the gastric motility, it is increased by stomach distention/alkalinization, amino acids, vagal stimulation and decreased by PH<1.5
what increases gastrin
chronic PPI, chronic atrophic gastritis, ZE syndrome
somatostatin is from, action, regulation
D cells in the pancreatic islets and GI mucosa- it decreases gastric acid and pepsinogen secretion, it decreases pancreas and small intestine fluid secretion, decreases gallbladder contraction, decreases insulin and glucagon release- it increases with increased acid and decreases by vagal stimualtion
what is octreotide used for and what does it mimic
octreotide is a somatistatin analog that is used to treat acromegaly, carcinoid syndrome, and vatical bleeding.
somatistatin overall does what
decreases secretion of lots of hormones
cholecystokinin is from, action, regulation
I cells in the duodenum and jejunum- it increases pancreatic secretion, increased gall bladder contraction, decreased gastric emptying, increased sphincter of ODdi relaxation- increased by fatty acid amino acids-
CCK does what to pancreatic secretions
it boosts them and acts on neural muscarinic pathways to cause this
secretin is from, action, regulation
it is from S cells. It is kicked off by increased H and bicarb from the pancreas. It increases the pancreatic secretion of bicarb and decreases the gastric acid secretion and increases the bile sectarian. it is regulated by increased acid and fatty acids in the duodenum
why do you want bicarb secreted from S cells of the duodenum in the face of acid
it is from neutralizing the acid to allow the pancreatic enzymes to function
glucose- dependent insulinotropic peptide is from, action, regulation
it is from the K cells and it decreases gastric H secretion and increases insulin release in response to fatty acids, glucose and amino acids in the lumen of the duodenum It is also known as GIP
what hormone causes increased insulin secretion orally but does not work if glucose is through an IV
GIP stimulates extra insulin secretion so if glucose is through the stomach rather than the IV
motilin is from, action, regulation
motilin is from the small intestine and produces migrating motor complexes it increases in fasting state.
what are motion receptor agonists used for and what is an example
erythromycin is a motilin antagonist which stimulates peristalsis
vasoactive intestinal peptide is from, action, regulation
it is from ons ganglia in the sphincters, gallbladder, and small intestine- increased intestinal water and electrolyte secretion and increased relazation of intestinal smooth muscle and sphincters- increased by distention and vagal simulation decreased by SNS
VIPoma
non alpha and non beta cell islet tumor of the pancreas that causes watery diarrhea, hypokalemia, and achlorhydria (decreased gastric acid)
Nitric oxide is from, action, regulation (GI)
increased smooth muscle relaxation and LES- loss of NO secretion is implicated in the increased tone in achalasia
what does NO relate to achalasia
it is decreased which means the esophagus cannot relax
ghrelin is from, action, regulation
it is from the stomach and it increases appetite and it is increased in the fasting state and decreased by food. Increased in Prader Willi and decreased after gastric bypass
intrinsic factor is from, action, regulation
it is from the partietal cells and it creates the binding iwith B12 and it makes it ready for uptake in there terminal lileum
gastric acid is from, action, regulation
parietal cells of the stomach it decreases the ph- it is increased by histamine, each, gastrin and decreased by somatostatin, GIP, prostaglandin, and secretin
what increased gastric acid
ACH, gastrin and histamine
pepsin is from, action, regulation
chief cells of the stomach, protein digestion increased by increased vagal stimulation and local acid
what is the inactive source of pepsin
it is pepsinogen until its in the presence of H
bicarbonate is from, action, regulation
from mucosalcells and brunner glands to neutralize acid and it increased through pancratic and biliary secretion with secretin
amylase- what does is digest and anything special
it digests starch and is secreted in an active form
lipases- what does is digest and anything special
fat difestion
proteases- what does is digest and anything special
protein difestin inclues trypsin, chymotrypsin, elastase, and carboxypeptidases
trypsinogen- what does is digest and anything special
it is converted to active enzyme trypsin to activation of other proenzymes and cleaving of additional trypsinogen molecules into active trypsin. Converted to trypsin by enterokinase/enteropeptidase at bruch order
what happens if there is a deficiency of enteropeptidase
no tyrpsin so protein and fat malaspption, failure to thrive and edema
carbohydrate absorption
only monosaccharides are absorbed by enterocytes. Glucose and galactose are through SGLT1.
what is fructose taken up by
GLUT2
what takes up glucose and galactose in the intestine
SGLT1
what is the rate limiting step of carbohydrote digestion at the brush border
oligosaccharidase
what is the DXylose test
distinguishes GI mucosal damage from malabsoprtion
what is D xylose
it is still active without pancreatic enzymes and can decrease overgrowth of bacteria
where is iron absorbed
in duodenum
where is folate absorbed
small bowel
where is B12 absorbed
terminal ileum along with bile salts and requires intrinsic factor
what are peers patches, where are they, what do they have
unencapsulated lymphoid tissue in the lamina propr. and submusoca of the ileum contain specialized M cells that sample and present immune cells. Be cell stimulated in germinal centers of the eye patches differentiate into IgA secreting plasma cells which ultimately reside in the lamina propr.. IgA receives protective secretory comporntt to travell across the gut
what is the rate limiting step of bile acid synthesis
it is cholesterol 7 alpha hydroxylate
what are the functions of bile
digestion absorption of lipids and gat soluble vitamins, cholesterol excretion, and antimicrobial via membrane disruption
what is bilirubin conjugate with to make it direct billirubin
glucoronate
which bilirubin is soluble in water
conjugated
pleomorphic adenoma
benign mixed tumor. It is the most common it is chonromyxoid storm and epithelium and recurs if not complete exccises
mucoepidermoid carcinoma
malignant salvart tumor with squamous and mucous components
warthin tumor
papillary cystademona lymphomatosum- benign cystic tumor with germinal centers
which salivary glands are more malignant
submandibular or sublingual
which nerve is usually involved in the salivary gland tumors
VII
which nerve is usually involved in the salivary gland tumors
VII
achalasia
failure of relaxation of LES due to loss of myenteric plexus. High LES resting pressure and uncoordinated peristalsis. Progressive dysphagia.
what can cause achalisia secondarily
Chugs disease or mass effect
boerhaave syndrome
transmural usually distal esophageal rupture with pneumomediatum- due violent retching surgical emergency
eosinophilic esophagitis
infiltration of eosinophils into the esophagus often in atopic patients. food allergens- dysphagia, food impugn. rings and linear furrows often seen on endoscopy
what causes strictures in the esophagus
associated with caustic and acid reflux
esophageal varices
dilated submucosal veins in the lower 1/3 if esophagi second degree to portal HTN.
esophagitis with linear ulcers
CMV
esophagitis with punched out lesions
HSV
GERD
heartburn, regurgitation, dysphagia. Chronic cough and hoaarsenss
Mallory Weiss syndrome
mucosal lacerations that are linear- gastropeal junction from sever committing with the hematemisis- due to abdominal pressure
Plummer Vinson syndrom
dysphagia, iron deficiency, anemia, esophageal webs- glossitis
scelerodermal esophageal dysmotility
esophageal smooth muscle atrophy because of decreased LES pressure and dysmotility and acid reflux and dysphagia, stricture, barrett esophagus, ad aspiration part of CREST
barrett esophagus
specialized intestinal metaplasia- replacement of nonkeratozined stratified sqamous epithelium with intestinal epithelium. with goblet cells and in distal esophagus. Due to chronic reflux esophagitis GERD. Associated with increased risk of esophageal adenocracinoma
squamous cell carincoma esophagus
upper 2/3 and alcohol, hot liquids, caustic strictures, smoking, achalasia- more worldwide
adenocarcinoma esophagus
lower 1/3 and chronic GERD, Barrett esophagus, obesity, smoking, achalasia- america
acute gastritis- what causes it
erosions in the mucosa from NSAIDs- PGE2 decrease, decreased gastric mucosa protection
burns-Curling ulcer- hypovolemia- mucosal ischemia
brian injury- increased vagal stimulation- ACH increase, increase H production
chronic gastritis- what causes it
mucosal inflammation often leading to atrophy hypochloridia, hypergatrinemia and intestinal metaplasia and increased risk of gastric cancer
h pylori
most common to increase risk of peptic ulcer disease, MALT lymphoma
autoimmune chronic gastritis
autoantibodies to parietal cells and intrinsic factor and increase risk of pernicious anemia- affects body/fundus of stomach
menetrier disease
gastric hyperplasia of mucosa- hypertrophied rug, excess mucus production with resultant protein loss and parietal cell atrophy with decreased acid production- precancerous
gastric cancer intestinal kind
associated with h pylori, dietary nitrosamines, tobacco, achlorhydria, chronic gastritis. commonly on the lesser curvature looks like ulcer with raised margins
gastric cancer diffuse kind
not associated with hpylori- signet ring cells- mucin filled cells and stomach wall grossly thickened and leathery stomach
virchow node
icolvement of the left supraclaviculaar node by mets from stomach
Krukenberg tumor
bilateral metases to ovaries. Abundant mucin-secreting, signet ring cells
sister mary joseph nodule
subcutaneous periumbilical metatsis
gastric ulcer
usually h pylori, decreased mucosal protection against gastric acid. NSAIDs can be increased risk carcinoma. greater pain with meals and weight loss
duodenal ulcer
decreases with meals and weight gain. mostly h pylori. Decreased mucosal protection or increased gastric acid secretion. ZE syndrome, generally benign and hypertrophy Brunner. posterior wall rupture leads to gastroduodenal artery rupture
what is the blood vessel that can be eroded with an duodenal ulcer
gastroduodenal artery
if there is an ulcer on the lesser curve
bleeding from left gastric artery
what referred pain with duodenal ulcer
duodenal perforation with air under the diaphragm
systemic mastocytosis
abnormal mast cell proliferation and increased histamine release. Hyper secretion of gastric acid by parietal cells in the stomach as well as hypotension, flushing, puritis
lactose intolerance
lactase deficiency- normal appearing villi, when second degree to injury at tips of villi, osmotic diarrhea with decreased stool pH. viral enteritis can cause it. Lactose hydrogen breath: positive for lactose malabsorption if post lactose breath hydrogen value rises>20ppm compared with baseline
pancreatic insufficiency
due to chronic pancreatitis, cystic fibrosis, obstructing cancer- causes malabsorption of fat and fat soluble vitamines- ADEK as well as B12- decreased duodenal pH and decal elastase
old man with weight loss, diarrhea, abdominal pain, and increased excretion of fat and muscle
pancreatic insufficiency
tropical sprue
celiac sprue, affects bowel but responds to antibiotics but seen in residents of or recent visitors to tropics- decreased mucosal absorption affecting duodenum and jejnum but can involve ileum with time. associated with megaloblastic anemia due to folate deficiency and B12 deficiency
whipple disease
infection with trophynema while +PAS, foamy macrophages, cardiac syndromes, arthralgia, neurologic symptoms.
middle aged men with long term malabsorption and cardiac, psych and arthritis
whipple disease
what causes appendicitis
due to obstruction by facecloth or lymphid hyperplasia in children,
why is the pain in the periumbilical region for appendicitis
it is from peritoneal inflammation
what colonizes an appedicial abscess
bacteriodes fragilis
appendectomy can denervate what nerve
iliohypogastric so decreased suprapubic sensation
what anatomical strcture do you use to find the appendix
tinae coli
diverticulum
blind pouch from the alimentary canal that communicates with the lumen of thegut
where is the most common site of diverticula
sigmoid colon
what is a true diverticulum
all three gut wall layers
what is a false diverticulum
only the mucosa and submucosa wusually is where the vasa recta perforate the muscular externa
diverticulosis
many false diverticular of the sigmoid colon from intrudes intraluminal pressure and focal weakenss also called pulsing diverticular- can have some painless bleeding or lead to diverticulitis
diverticulitis
diverticulosis with inflamed micro perforations classically seen with LLQ pain, fever, leukocytosis, and treat with antibiotics can have access, fiscal, and obstruction or perforation which leads to air under the diarpharm
zenker diverticulum
false fiverticulum from pulsion diverticula- esophageal dynmotility at the thyropharngeal and cricopharyngus in the inferior pharyngeal constrictor- elderly males
meckel diverticulum
true diverticulum– vitalize duct and can have ectopic acid secreretion from pancreatic tissue or gastric mucosa. can cause melon, RLQ, intussusception, collies, or obstruction at terminal ileum
how do you test for meckels
pertechnetate study for uptake but ectopic gastric mucosa
what is the rule of 2s
2 times as likely in males, 2 inches long, 2 ft from the ileocecal valve, 2% of the population, first 2 years of life and two types of epithelium possible
Hirschspring disease
congenital megacolon characterized by lack of ganglion cells in the enteric nervous plexus in distal segment of colon due to failure of neural cres migration rectum is a mutation of RET- bilious emesis, abdominal distention failure of mechanism
what is the diagnosis of hirshcrpung
rectal suction biopsy from the rectum tight affection portion
malrotation of gut
anomaly of midgut rotation during deal development with improper position of the bowel and formation of Ladd bands leasing to voluvus and duodenal obstruction with bilious vomiting
volvulus
twisting of portion of bowel around its mesentery leads to obstruction and infarction.
which volvulus is more common in children
midgut
which volvulus is more common in old people
sigmoid volvulus
intussecuption
telescoping of proximal bowel segment into distal segment at illeocecal junction. Can lead to intermittent abdominal pain with currant jelly stool
why does intusseception in children
it is from idiopathic with peer patch hypertrophy following adenovirus infection or meckel diverticulum
why does intusseception happen in adults
from tumor foci
what does intussusception look like on ultrasound
bulls-eye
acute mesenteric ischemia
critical blockage of intestinal blood flow often at SMA causing small bowel necrosis and abdomnial pain out of proportion with findings- currant jelly stool
chronic mesenteric ischemia
intetinal angina from atheroscelosis and intestinal hyperperfusion- food aversion and weight loss
colonic ischemia
reduction in internal blood flow cuases cramp abdominal pain and bleeding from splenic flexure and distal colon
angiodysplasia
torituous dilation nof vessels leading to bloody stool, ceum, terminal ileum, or ascending colon
adherion
fibrous band of scar tissue after surgery blocks the small down with well demarcated necortic areas
ileus
intestinal hypo mobility without obstruction- constipation decreased farts, typamic abdomen with decreasd bowel sounds, often from abdominal surgery, hypokalemia, oepiates, sepsis
meconium ileus
CF, meconium plu obstructs intestin
necrotizing enterocolitis
premature formula fed infants with intestine mucosa colon necroses with possible performation- ascetic necroses and curvilinear lucency free air in abdominal wall
hyperplastic polyps
generally small rectosigmoid polyps that are benign
hamartomatous polyps
non-neoplastic solitary lesions that do not have risk of malignant transformation. Peutz Jegheer and juvenile polyposis
adenomatous polyps
neoplastic via chromosomal instabiltiy pathway with mutations in APC and KRAD. tubular is less malignant than villous. usually asymptomatic but can have some bleeding associated
serrated polyps
premalignant via CPG hypermethylation have microsattelie instability and mutations in BRAF- saw tooth miprholy of the crypts
Familial adenomatous polyposis
AD mutation of APC tumor suppressor gene on 5q- pancolonic always has the rectum- need a colectomy because they all go to cancer
gardner syndrome
FAP+osseus and soft tissue tumors, congenital hypertrophy of retinal pigment epithelium, impacted teeth
Turcot syndrome
FAP plus a malignant CNS tumor- Turcot=turban
Peutz Jegher
AD featuring hamratomas also the GI with hyper pigmented mouth lips, genitals, and increased breast and GI cancer like colorectal, stomach, small bowel, pancreatic
Juvenile polyposis syndrome
AD syndrome in children over 5 years old with numbours polyp and has some increased cancer risk
Lynch
DNA misamtch repair issue with MSH2 and MLH1- AD mutation with micro satellite instability that goes to cancer. Proxmal colon is involed and endometrial ovarian and skin cancer
risk factors for colon cancer
polyps, familial cancer syndromes, IBD, tobacco, diet of processed meat with low fiber
where is the colon cancer if there is blood in the stool
right side- ascending colon- iron deficiency anemia
where is the colon cancer if there is obstruction
left side- descending side
what tumor marker is good for measuring the recurrence of colon cancer
CEA
what bacteremia can colon cancer present with
strep bovis
what is the progressive mutations that cause colon cancer
APC (increased prolif), KRAS (forms adenoma increased intracellular signaling), p53 or DCC (loss of tumor suppression gene) leads to carcinoma
cirrhosis
diffuse briding fibrosis via stellate cells and regenerative nodules disrupt normal architecture of the liver. increased risk of hepatocellular carcinoma- usually alcohol, autoimmune, viral, billiard sides, genetic or metabolic disease
portal hypertension-
increased pressure in the portal venous system, vascular obsctustion, schistosomias, cirrhosis
what are the characteristis of cirrhosis caused by postal HTN
increased adrenal production of adronsteindione causes increased estrogen, spider angioma, gynecomastia, lose sex hair, testicular atrophy, palmar eryhtema
what are other characteristics of cirrhosis
jauncdice, purpura, petichiae, splenomegaly, ascites, cardiomyopathy, edema, hyperbillirubin, hyponatremia, coagulation issues, anemia, thrombocyotpenia, nausa, committing, dull abdominal pain, anorexia, hepatic encephalopathy, asterixis
hepatic encephalopathy symptoms
sleep changes, altered mental state, ataxia, asterixis
hepatic encephalopathy what precipitates it
GI bleeding increased nitrogen, sedatives, narcotics, hyperemia, infection, portosystemic shunt
how to treat hepatic encephalopathy
- lactulose- increased ammonia trapping in stool fro acidification
- rifaxonim or neomycin decrease the amount of bacteria producing NH3
why does hepatic encephalopathy happen
astrocytes normally take up glutamate in the synapse to prevent excess neuronal excitation, glutamate is then condensed with ammonia to form glutamine which is the released by astrocytes and reconverted to glutamate by neurons for use. Excess ammonia cause increased glutamine production and this causes swollen astrocytes an they can’t release the glutamine so decreased excitable neurotransmitter
what tests mark alcoholic liver disease
AST>ALT
what does increased alk phos indicate
cholestasis, infiltrative disorders, bone dies like PAgets
GGT what dos it indicate
, liver and billiard disease especially alcohol use but not bone disease
what is increased and decreased in liver disease- albumin, bilirubin, prothrombin, platelets
in liver disease- albumin decreased, bilirubin increase, prothrombin increase, platelets decrease
Reye syndrome
fatal childhood hepatic encephalopathy with mitochondrial abnormalities, micro vesicular steatosis, hypoglycemia, vomiting, hepatomegaly, coma. usually VZV or the flu treated with ASA. aspirin metabolises decrease beta ocisdation by reversible inhibition of mitochondrial enzymes
what type of steatosis is seen in Reyes
microvesicular
what type of steatosis is seen in hepatic steatosis
macrovesicular fatty change that changes with alcohol cessation- from increased TAG and decreased fatty acid oxidation from excess NADH
what type of steatosis is seen in alcoholic hepatitis
long term consumption leading to swollen necrotic hepatocytes with neutrophilic infiltration and Mallory bodies which are intracytoplasmic eosinophilic inclusions of damage keratin filaments- AST>ALT
what type of steatosis is seen in alcoholic cirrhosis
final irreversible form with micro nodular irredulatly shanked liver with hobnail appearance with sclerosis around the central vein in zone 3
what type of steatosis is seen in NASH
metabolic syndrome with insulin resistance, obesity, fatty infiltration of hepatocytes and cellular ballooning and necroses leading to cirrhosis and HCC independent of alcohol use
hepatic encephalopathy
cirrhosis with protpsystemic shunts and decreased NH3 metabolism and neuropsychiatric dysfunction. can be triggered increased NH# production from increased dietary protein, GI been, constipation, infection. or decreased NH3 removal due to renal failure, diuretics, bypassed hepatic blood flow after TIPS
autoimmune hepatitis type 1 antibodies
ANA and anti smooth muscle antibodies
autouimmune hepatits type 2 antibodies
anti live kindey microsomal antibody
classic presentation of autoimmune hepatits
increased AST and ALT and female autoimmune profile
hepatocellular carcinoma
primary malignant tumor of the liver and associated with HBC< HCV< alcohol, NASH, autoimmune, hemoachromaotsis, alpha 1 atritypsin, and aflatoxin. Jaundice, hepatomegaly, ascites, polycythemia, anorexia spread hematogenously- increased AFP
HCC levels to moniter
AFP
cavernous hemangioma
benign liver tumor from 30-50 year olds. Cannot biopsy. Enlarges by ectasia, spongy look on histology, and mulberry from dilated blood vessels with thin adventitia lacking elastic fibrils and smooth muscle. In the brain these can lead to neurological deficients, seizures and epilepsy
heaptic adenoma
rare benign from OCP of rupture leads to adbfomina pain and shock
angiosarcoma
malignant blood vessel tumor of the liver with arsenic or vinyl chloride exposure
metastes to the liver
GI malignancies, breast, lung
Budd Chiarri Syndrome
thrombosis or compression of hepatic veins with centrolobular congestion and necrosis causing congestive liver disease like hepatomegaly, sites, varices, abdominal pain, no JVD, hpercoagulable , polycythemia and postpartum and HCC cause it liver can look nutmeg
hepatic abscess
fever chills RUQ- developing countries from Entamoeba and echinococcus. Developed counties it can be an infection. like s aureus, ecoli, kleb, enterococcus
alpha 1 antitypsin deficiency
misfolded gene product aggregates in the hepatocellular ER and cirrhosis with PAS + globules. In lungs there is decreased so there is uninhibited elastase i the alvelo and decreased elastic tissue and panacinar emphysema
jaundice
abnormal yellowing of the skin or sclera due to bilirubin deposition. Hyperbillirubinemia and seoncdary to increased production or decreased disposition (impaired hepatic uptake, conjugation, excretion-
unconjugated hyperbillirubinemia
hemolyic and physiologic in newborns. Criggler Najjar and Gilbert
conjugate hyperbillirubinemia
billary tract obstruction: gallstones, cholangiosacrinoma, pancreatic or liver cancer, liver fluke, primary scleorising choalngiits, PBC, dubin johnson, rotor
mixed hyperbilirubinemia
hepatitis and cirrhosis
physiologic neonatal jaundice
at birth immature UDp leads to increased unconjugated which can lead to bilirubin deposition in the basal ganglia use phottherapy
Gilbert syndrome
mildly decreased UDP and this means imparired bilirubin uptake and asymptomatic or mild jaundice without overt hemolysis bilirubin increases with stress and fasting
Criggler Najjar- inhertiance pattern, symptoms and types and treatmetn
AR, if its type 1 get plasmapheresis, and phototherapy because of high unconjugated billirun. type II has some residual enzyme function so can induce liver enzyme synthesis with phenobarbitol
Dubin Johnson
conjugated hyperbilirubinmeia due to defective liver secretion with black liver
Rotor syndrome
milder presentation of hyperbillirubinemia of conjugated bilirubin without black liver due to impaired hepatic uptake and excretion
Wilson disease
recessive mutations in hepatocyte copper transportng ATPas on chromosome 13- inadequate copper excretion into bile and blood so decreased ceruloplasmin and increased urine copper. Copper accumulatres in the liver, brain, cornea, kidney, joints- [resetnt before 40 with hepatitis, liver failure, cirrhosis, neurologic like dysarthria, dystonia, term, parkinson, psych issues, Kayse fleisher rings, hemolytic anemia ,renal disease PCT dysfunction
treatment for wilson disease
chelation with penicillamine or trientine or zinc
where does copper deposit in the brain
basal ganglia causing atrophy
what is the hepatic injury from in wilson disease
generation of ROD
where is copper normally removed from the body
in the bile
onion skinning bile duct, alternating beads on a string, middle aged man with UC- what does he have, what is the test, what are the increased incidence with this
PSC, p anca, increased IgM, and can lead to secondary billiard cirrhosis and cholangiocarcinoma
autoimmune reaction with lymphocytic infiltration granulomas and destruction of the interlobular bile ducts and middle aged woman-what does she have, what is the test, what are the increased incidence with this
PBC, antimitochondrial antibody increased IgM and autoimmune conditions
extrahepatic biliary obstruction and increased pressure in intrahepatic ducts and injuryy and fibrosis and bile stasis- what causes it
patients with known obstructions like gallstones, strictures,pancreatic cancer- ascending cholangitis can occur
what is the mutation for hemochromotosis
HFE gene on chormosome 6
what is the pathology of hemochromotosis
abnormal iron sensing and increased intestinal absorption so increased fourteen, ion and decreased TIBC with increased transferrin. iron overload can be from transfusion. especially sits n the skin, liver, pancreas, heart and pituitary and joints
what is the histologic features of hemochromotosis
Prussian blue stain
presentation of hemochromotosis
age of 40 with high iron body total and women are slower to present. have cirrhosis, diabetes, skin pigmentation, dilated cardiomyopathy, hypogonadism, arthropatyh with calcium pyrophosphate. HCC is common cause of death
cholesterol stones
opaque from crohn, obesity, advance aged, estrogen, rapid weight loss, native american
pigmented gallstones
secondary to bacteria or helminths that increased the infection of the billiard tract which release beta glucoronidase by injured cells and this increased the amount of unconjugated bilirubin, crohn, hemolysis, alcoholic cirrhoses, age, total parentteral nutrition
billiary colic
neurohormonal activation after fatty meal triggers contraction of gallbladder forcing a stone into the cystic duct. not painful in diabetes
risk factors for gallstones
female, fat, forty, fertile
air in biliary tree would indicate what
fitular with gallbladder and GI tract and passage of gallstone to intestinal tract acan obstruct the ileocebal valve or the bowel and the bowel would have high pitched sound and pressure necroses and erosion
why does TPN increase the risk of stones
decreaed CCK so more bile stasis and cholesterol stones
cholecystitis
chronic or acute inflammation of the gall blaster with wall thikcingn from the block of cystic duet. Can be calculus due to ischemia and stasis or CMV.
when are acalculous stones in the gall bladder seen
critically ill like sepsis, burns, trauma, immunosuppression decrease blood supply to the gallbladder
porcelain gallbladder
calcificed gladdbladder due to chronic cholecystitis found incidentally. need to remove because of high rate of gall bladder cancer
acute pancreatitis is from what
actiavtion of trypsin leading to activation of the hormone cascase
what ar the causes of acute pancreatitis,
gallstone, ethanol, trauma, scorpian, mumps, steroids, autoimmune, ERCP, high calcium, high TG, sulfa drugs, NRTIS, protease inhibitor
what are pancreatic pseudocysts lined by
lined by granulation tisse
what are the complications of acute pancreatitis
pseudocysts, hypocalcemia, shock, ARDS, renal fialure, necoris, hemorrhage, infection
hereditary pancreatitis
trypsinogen or SPINK1. The most common pmuation leads to abnormal typist production thats not susceptible to inactiviting cleave by trypsin. This leads to repeated pancreatitis because it escapers from inactivation of trypsin mechanism
chronic pancreatitis
chronic inflammation, atrophy, calcification of pancreas, major causes are alcohol abuse, and idiopathic mutations in CFTR genr cause chronic pancreatic insufficiency
what do you seen in pancreatic insufficiency
steatorrhea, fat soluble vitamin deficient,y DM< and amylase and lipase may or may not be elevated
pancreatic adenocarcincoma
very aggressive tumor arising from pancreatic ducts with disorganized glandular strucutre often metastatic
where is the tumor first present
pancreatic head leading to jaundice
what is the associated tumor marker
CA19-9
risk factors for pancreatic adenocarcinoma
tobacco use, chronic pancreatitis, diabetes, age over 50, jews and blacks.
what is the presentation of pancreatic adenocarcinoma
abdominal pain radiation the the back, weight loss, migratroy thrombophlebitis, redness and tender of palpation of extremities. obstructive jaundice with palpable non tender gallbladder
what can linear xanthomas be associated with
PBC
life span of a RBC and platelet
RBC-120 days
plateley 8-10
aniscocytosis
varying size RBC
poikilocytosis
varying shapes of RBC
reticulocytoe
immature RBC
why are reticulocytes blue on Giemsa stain
can be seen when there is increased production of RBC and it is from residual ribosomal RNA
platelet energy source
glucose only
where are extra platelets stored
spleen
vWF receptor is
GpIb
fibrinogen receptor is
GpIIb/IIIa
what are the dense granules of platelets
adp and ca
what are the alpha granules of platelets
vWF, fibrinogen, and fibronectin
what are the granulocytes
neutrophils, eosinophils, basophils
what are the mononuclear celles
monocytes and lymphocytes
what the more abundant to least WBC
neutro, lympho, mono, eosino, basophils
neutrophil grnules
azurophlic granules which contain proteinases, acid phosphatase, myeloperoidase and beta glucoronidase
what are hyperhsegmented neutropils a sign of
b12 or folate deficiency.
what is the cause of increased band cells
these are immature neutrophils so they ar from increased myeloid proliferation like infection or CML
what are the neutrophil chemotactic signals
C5a, IL8, LTB4, kalikrenin, platelet activating factor
what causes petichae
decreased platelet function or thrombocytopenia
kidney shaped nucelated cell
monocytes
what do monocytes do
differentiate into macrophage in the tissue
macrophage- function and what activates them
it phagocytes bacteria, cellular debris, and dead RBC, and it is derived from monocytes. activated by interferon gamma and acts as an APC to MHCII cells
what role do macrophages play in shock
LIpid A from LPS brings CD14 on the macrophages to start septic shock
eosinophil- function and what does it produce
defend against helminths and it is billobate with highly phagocytic activity for immune complexes. it produces histamines and MBP
what are the causes of eosinophilia
neoplasia, asthma, allergic processes, chronic adrenal insufficiency, parasites
basophil- function and granules
mediates allergic reaction. densley basophilic granules contain heparin and histamine. Leikotrienes can also be synthesized.
what are the causes of basophilia
rare but can be a sing of CML
mast cell- function/ process of its reaction
mediates allergic reaction. contain histamine granule.s Binds the Fc portion of IgE and it crosslinks it so causes degranulation releasing histamine, heparin, tiptoes, and eosinophil chemotactic factors.
what type of hypersensitivity reactions are mast cells associated with
type I hypersensitivty
what drug prevents mast cell degranulatin
cromolyn
dendritic cell function
highly phagocytic APC and it links the innate and adaptive immune response. it expresses MHC class II and Fc receptors on the surface.
what are dendritic cells of the skin called
langerhans cells- birbeck granules
lymphocytes- what cells and what are adaptive and which are innate. what does this look like
B and T cells are adaptive and NK is innate immune. giant nucleus and small amount of cytoplasm
B cells- where are they from, where do they go, what do they differentiate into
humoral immune response- from stem cells in the bone marrow and matures in marrow then migrates to peripheral lymphoid tissue like LN follicles, white pulp of the spleen, unencapsulated lymphoid tissue, and when antigen is encountered here B cells differentiate into plasma cells that produce antibodies and memory cells. Can function as an APC to MHCII
T cells- where are they from, where do they go, what do they differentiate iinto
mediate cellular immune respones. originate from stem cells in the bone marrow but matures in the thymus. T cells differentiate into cytotoxic T cells that express CD8, helper cells that press CD4 and regulatory.
what signal is necessary for T cell activation
CD28 and B7
plasma cells- what do they look like, where re they found, and what do they do
produce large amounts of antibodies for specific antigens and have a clock face nucleus and abundant RER and golgi apparatus. Found in the bone marrow and do not ciculate
what is a cancer of plasma cells
mutliple myeloma
what does erythropoesis from 3-8 weeks
yolk sac
what does erythropoesis from 6 weeks to birth
liver
what does erythropoesis from 10-28 weeks
spleen
what does erythropoesis from 18 weeks to adult
bone marrow
what is the embryogenic globuin
squiggle, e
what is the fetal hemoglobin
a2,g2
what is the adult hemoglobin
a2b2
what is the difference between adult and fetal hemoglobin
HbF has higher affinity for O2 due to less avid 23BPG binding and allowing HbF to extract O2 from maternal hemoglobin across the placenta
why do beta globin defects appear after a few weeks
it is from the production of fetal hemoglobin for the first few weeks of life
HbA2
a2,d2
HbA1c
a2,b2,glucose
HbF
a2g2
HbGower
squiggle2, e2
HbS
a2,Bs2
HbC
a2bc20 glutamine for lysince
HbBart
severe alpha thal g4
HbH
severe alpha tal B4
what is the situation for RH hemolytic disease of the newborn
it is from Rh- mom’s who have had a rh+ baby. The IgG against it can cross the placenta on the next Rh+ baby and cause hemolysis. It is from erythrocyte opsonization
ABO hemolytic disease of the newborn
it is from A type O mother with a AB, A, B baby that has anti=A or B against the baby and it does not worsen with future pregnancy they child gets mild jaundice at birth which is fixed by phototherapy
what is the order (what runs the furthest) on a gel electrophoreseis of hemoglobin
A, F, S,C
what regulated primary hemostasis
platelet plug is the major factor driving primary hemostasis
what regulates secondary hemostasis
it is the coagulation cascade which drives the cross linking of the clot
what is the first step of primary hemostasis
transient vasoconstriction leadsto decerase dbloo in the region. endothelial cells secrete endothelin
go through the primary hemostasis
transient vasoconstriction, VWF is released by the waybill pallade body in the endothelium and this adheres to the collagen in the damaged endothelium then binds to platelets by GpIb. This activates the platelets to degranulate and release TXA2 and ADP. The ADp then induces the platelet to express GPIIb/IIIa which binds to other platelets to aggregate. TXA2 draws more platelets to the ares for further aggregation. The fibrinogen is also produces
what drugs inhibit the ADP induced expression of gpIIb/IIIa
clopidogrel, prasugrel, and ticlopidine
what drugs block gpIIb/IIIa directly
abciximad, eptifabine, and tirofiban
what does risotcetin do
it activates vWF to bind GpIb and failure of aggregation with ristocetin occurs with vWF and Benard Soulier
mucosal and skin bleeding is a sign of what kind of disorder
disorder of platelets quantitiy or quality
ITP- pathogenesis
antibody to IgG agaisnt gpIIb/IIIa and it causes thrombocytopenia produced by plasma cells in the spleen then the spleen takes these out of commison
what causes acute ITP and treatment
children with a viral illness or vaccine- treated with steroids
what causes chronic ITP and treatemetn
women of childbearing age with lupus or primary. It can be anti platelet IGG can cross placenta ad cause it in the child. The treatment is steroids which wear off, IVIG which distracts the spleen or splenectomy which is curative
what are the labs for ITP
decreased platelets, normal PTT or PT and increased megakaryocytes
microangiopathic hemolytic anemia- what are the causes and path
it is from the formation of platelet micro thrombi in the blood vessels which causes hemolysis and schistocytes as the RBC pass over these clots. This consumes platelets so seen in TTP and HUS
TTP- pathogenesis and everything else
problem with micro thrombi from decreased ADAMSTS13 which cleaves the multimers to get rid of VWF so there is excess VWF factor which leads to extra platelet micro thrombi. Genetic defect or autoimmune process. tend to see CNS issues- treat with plasmapheresis and corticosteroids
HUS- pathogenesis and everything else
due to endothelial damage from drugs or illness. It is forms microthrmbi because verotoxin damages the endothelium of the kindest and brain leads to diarrhea because its from ecoli
Bernard Soulier- pathogenesis and everything else
genetic GpIb deficiency so platelet adhesion is impaired. Large platelets from over production and mild thrombocytopenia. Cannot bind VWF to platelets.
Glanzman thrombocytopenia- pathogenesis and everything else
GpIIb/IIIa deficiency so decreased platelet aggregation-
ASA thrombocytopenia
irreversibly blocks COX so decreased TXA2 so less platelets can come to aggregate
uremic thrombocytopenia
from decreased kidney function leading to increased nitrogen. This messes up the adhesion and aggregation
where do coagulation factors come from
the liver and so this can get disordered from liver issues
what are the clinical features of a secondary hemostasis disorder
deep joint and muscle bleeding and wisdom teeth prolonged bleeding- all have increased PT or PTT
what factors are part of the common, intrinsic, and extrinsic pathway
common- 10, 5,2,1
intrinsic: 12,11,9,8
extrinsic: 7
hemophilia A
factor 8 deficiency can be denote or arise from X linked recessive mutations. It is increased PTT because its from the intrinsic pathway- treat with recombinant factor 8 and desmopresssin which increases vWF
hemophilia B
FIX deficiency and levels are decreased in the blood
coagulation factor inhibitor how to differentiate from hemo A
anti FVIII which leads to VII function decrease. In a plasma mixing study, there will be no decrease in PTT if there is a factor inhibitor but if there is hemo than it will correct the PTT
vWF deficiency
AD so decreased VWF so decreased platelet adhesion leading to mild mucosal and skin bleeding with an increased PTT because of decreased stability of 8 and increased BT from platelet nonadherance. It also has abnormal ristocetin- use desmopressin to increase VWF
vitamin K deficiency
Vitamin k is needed for 2,9,7,10, C,S- and these are EPOX reductase to activate the K in the liver. deficiency is from liver failure or GI colonization or long term antibiotics or malabsotpion.
HIT
platelt destruction that arises from heparin fragments that have antibodies against them so then the platelets lead to thrombosis and warfarin caused skin necorsis
DIC
pathogenic activation of coagulation cascase with widespread micro thrombi and result in ischemia and infarction. Consume the plates and factors which leads to all sorts of bleeding.
DIC cuases
sepsis from increased IL1 and TNF alpha, obstetric complications because amniotic fluid has tissue thromboplastin, and adenocarcinoma from mucin, APML which has auer rods, rattlesnake
what are the labs for DIC
decrease plate, Increased PT and PTT, and decreased fibrinogen, and increased fibrin products like D dimer
what does plasminogen do to become plasmin and what does plasmin do
it cleaves fibrinogen, cleaves fibrin, stops coag factors, and blocks platelt aggregation it is from plasminogen to plasmin by tpa
radical prostatectomy can do what to blood
it can have release of urokinase which activates plasminogen which increases bleeding
cirrhosis can do what to blood
decreased alpha 2 antiplasmin and decreased coagulation factors overall
plasmin overactivity labs
looks like DIC but there is normal platelets and no D dimer even if fibrinogen products are elevated
risks of clot formation
disruption of flow, endothelial damage, hyper coagulable state
what are the causes of disruptive flow
stasis, turbulence, imobilization, cardiac wall defects, aneurysm
what does thrombomodulin do
it is like protein C and stops the amplification of 5 and 8
protein C or S deficiency
decreased negative feedback on cascase so cannot stop activation of 5 or 8. It has increased risk of warfarin induced necrosis
warfarin degrades which factors first
it degrades C and S first so increased risk of clots so do heparin bridge first that way there is not too much activation of the intrinsic pathway
factor V liedan
mutation of factor V so can’t be cleaved or degraded by C or S so excessive V activity leading to clots
prothrombin 2021A
increased prothrombin expression
ATIII deficiency
heparin like molecules usually bind ATII to decrease formation of thrombin so the heparin cannot stop the formation of thrombin
why do OCP cause clots
increased estrogen can increase coagulation factor production from the liver
acanthocytes
liver disease or abetalipoproteinemia- spur cell
basophilic stipling
lead poisoning, sideroblastic anemia, myelodysplastic syndromes
teardrop cells
bone marrow infiltration, myelofibrosis
bite cell
G6PD remove the heinz body in the spleen
burr cell
endd stage renal disease aka uremia, pyruvate kinase deficiency, or liver failure- smaller projections that acanthoycyte
elliptocyte
hereditary elliptocytosis muataion in spectrin
macroovalocyte
megalobalstic anemia usually also see hyperhsegmented PMNS, marrow failure
ringed sideroblast
sideroblastic anemia with excess iron in the mitochondria
schistocyte
DIC, TTP, HUS, HELLP, mechanical heart valve
sickle cell
sickle cell enemy the sickling occurs with dehydration and deoxygenation or high altitude
spherocyte
hereditary spherocytosis, drug induced infection with hemolytic anemia
target cell
HBC disease, asplenia, lier disease, thallasemia
Heinz bodiees
G6PD deficiency- phagocytic damage occurs to these RBC
Howell Jolly bodies
hyposplenia or asplenia- basophilic nucelar remnants in the RBS and they are normally removed by the spleen
what are the signs of anemia
weak, fatigue, dyspnea, pale conjunctiva, skin issues, angina if history of CAD, headache, lighthearded
how do you measure anemia
decreased RBC mass so decreased hb, hct, rbc and this coaunt is concentration dependent
why does microcytic anemia happen
the precursors divide extra because they want to keep the same amount of hb in the cells, so they want to keep them as red as they usually but with decreased hemoglobin production it leads to smaller cells to maintain this same concentration in the cells
where is iron usually absorbed and that regulates it from enterocyte to macrophage pathway
iron is consumed from meat to heme then it is absorbed n the duodenum, and the enterocyte takes it up and the feroportin decides to bring it in or not, It uses it to bring it to the blood where it is bound to transferrin and then it is stored with fourteen in the macrophage
what are the deficiencies from the in age groups: infant, child, adult, elderly, and the two other general causes
infant- breast milk
child- dietary lack
adult- peptic ulcer disease or periods
elderly- cancer or hook work which is nectar or aniscolustm
gastrectomy from decreased acid production meaning the iron is Fe3 not Fe2 so harder to bring into the enterocytes
celiac destruction of the duodenum
stages of iron def
use up stored so decreased fourteen so increased TIBC
serum iron is depleted so serum fe is decreased
normocytic anemia- with bone marrow making lessRBC
microcytic hypochromic anemia
what are some of the specific signs for iron deficiency anemia
spoon nails, anemia, and pica
what are the labs for iron def anemai
increased RDQW, decreased fourteen, increased TBIC, decreased serum iron, decreased percent saturation increase FEP
what is FEP
it is extra protoporphyrin in the RBC that is not bound because of the decreased heme
Plummer Vinson
esophageal webs, glossitis, iron deficiency, and dysphagia
pathogenesis of anemia of chronic disease
increased hepcidin sequesters iron so decreased transfer from macrophage to erythroid precursors. hepcidin also decreases EPI and iron is essential for bacteria so this is why it tries to hide the iron from chronic inflammatory state
what are the labs looking like for anemia of chronic disease
increased fourteen, decreased TBIC, decreased serum iron, decreased % saturation, increased FEP
what is the treatment for anemia of chronic disease
treat underlying cause of it and decreased hepcidin. use EPO if they have cancer
sideroblastic anemia- pathogenesis
defective protoporphyrin production so leads to microcytic anemia
what is the acquired cause of sideroblastic anemia
alcoholism, lead poisoning, B6 deficiency because ala synthase needs it as a cofactor. B6 deficiency is from isoniazid
what is the congenital cause of sideroblastic anemia
defect is ala synthase
rate limiting step of protoporphyrin
ala synthase and B6 is the cofactor
what takes ala to propphyobillinogin
it is ala dehydrogenase
what catalyzes the final step of conjugation of proto to iron
ferrochelatase
where does the ferrochelatase reaction take place
mitochondria and the fe waits on the photo there
what are the labs for it
increased fourteen, decreased TIBC, increased serum Fe, increased saturation
what is the stain for iron and where do you see histology for sideroblastic anemia
see rings of mitochondria around the nucleus of the cell and it stains for the excess iron that has accumulated because of the lack of protoporphyrin
alpha thalasemia- where is the gene and how many copes and what if its messed up what is the defect
it is deleted. There are 4 copies, and they are on chromosome 16
what is 1 gene defect in alpha thal
asymptomatic
what is 2 gene defect in alpha thal
mild anemia with increased RBC count
- trans is african and is milder
- cis is Asian and can lead to offspring with increased risk of spontaneous abortion
what is 3 gene defect in alpha thal
severe anemia not seen in feet. It is HbH and it s beta chains in tetramers and damage RBC
what is 4 gene defect in alpha thal
it is seen in the fetus from the tetramer of gamma and leads to hydrous fetalis
beta thalasemia- where is the gene and how many copes and what if its messed up what is the defect
gene mutaton so there can be variable production of the bet a chain. it is on gene 11
beta thal minor
some anemia and asymptomatic with target cells and loss of central pallor. isolated increase in HbA2 and HbF
beta thal major
B0B0- seen with major sever with both knocked out appears in first few moths of lives. form alpha tetramers and ineffective erythropoiesis because they don’t get out of bone marrow so see hyperplasia of marrow in face and crewcut skull on XR. Increased risk of parvo B19- get target cells and some nucelated cells
folate deficiency
absorbed in the jejunum. there can be from alcoholic, elderly, pregnant or hemolytic anemia, or methotrexate- normal methylmalonic acid but increased homocysteine
B12 converts methylamalonic acid to what
succinyl coA
B12 deficiecny- route of digestion
from mouth its bound to R binder until it gets to the small bowel and its cleaved and binds IF from parietal cells of the body of the stomach and it foes to the terminal ileum for absorption. there are increased hepatic stores.
what color are parietal cells on stain
pink
what other things besides pernicous anemia can cause B12 deficiecny
pancreatic insufficiency, so cannot cleave binder off of B12 damage to terminal ileum Crohns diphyllobathium latum vegans who eat no meat
labs for B12 deficiency
macrocytic anemai, glossitits, subacute degeneration of spinocerebellar tracts and this is from increased methamalonic acid and increased homocyteine
what is the formula for adjusted reticulocyte count
it is % of reticulocytes times hit/45
hereditary spherocytosis
biconcave disc with blabbing membrane fro black of nnkyrin and spectate. they get blebs lost in spleen so increased RDW and increased MCHC. increased risk of aplastic crisis, risk of gallstones from increased hemolysis, osmotic fragility
what cells do you see with spherocytosis
howell jolly bodies which are nucleotide RBC
sick cells polymerize in what cases
hypoxia, dehydration, acidosis
what can you use to treat sick cell
hydroxyurea to increase hbf
what is the most common cause of death in sickle cell kids and adults
kids- encapsulated bacteria
adults- acute chest syndrome- get vasoconstition after pneumonia and get pulmonary vasculature contraction.
what happens to the kidneys in sickle cell
renal papillary necrosis which is gross hematuria and proteinura
what are things with sickle cell trait
some normal beta and microinfart of kidneys leave them slightly unable to concentrate the urine
HbC
glutamic acid for lysine- leads to mild anemia
paraoxysmal nocutal hemogloburinria
DAF and MIRL prevent complement normally from attacking the TBC. These are anchored by GpI and it is deficiency in this so when they sleep the slight respiratory acidosis activates complement and causes hemolysis so hemoglobinuria in the mornings and can test for it with lack of CD55 on TBC
what do people with paraoxysmal nocutal hemogloburinria die of
platelet fragmentation and thombosis
what can it cause paraoxysmal nocutal hemogloburinria
iron deficiency anemia and increased risk of AML because its from abnormal myeloid precursors to begin with
G6Pd deficeincy inhertiance
X linked recessive
how is african variant different from meditarean in G6Pd
the med is more severe because there is less enzyme so earlier hemolysis and african the enzyme has a longer half life
what cells do you see and symptoms
hemoglobinuria nd henize bodies and bite cells
IgG hemoltic anmeia
warm agglutination, lose membrane to the spleen can be from SLE, CLL< drugs, and like PCN causes issues in the membrane or Mdopa.
IGm immune hemolytic anemia
binds in cold and form MAC and it is from mycoplasma or mono
direct coombs
confirms antibody coated RBC and add anti-IgG
indirect coombs
are their antibodies in the serum and add substrate for them to bind
malaria causes what in the cells
rupture from RBC to form lifecycle and get ever with rupture.
underproduction of RBC can be from
micro and macro anemia, renal failure and decreased EPO, and damage to basement membrane
lead poisoning causes microcytic anemia why
lead inhibits ferrochelatase and ala dehydratase and this leads to inhibited rRNA digression so basophilic stilling is from remount rRNA. Lead lines in gingiva, encephalopathy, abdoomnial pain, sideroplastic anemia, wrist and foot drip
what do you treat lead poisoning with
dimercaperol and EDTA
what is used for lead poising in kids
succimer
orotic aciduria
inability to convert orotic acid to UMP because of a defect in Ump synthase. AR presents as child with failure to thrive, developmental delay, and megaloblastic anemia refractory to folate and B12- increased orotic acid and no hyper ammonia.
diamond blackfan anemia
rapid onset of anemia in first year of life due to intrinsic defect in erythroid progentiaro cells. short stature with craniofacial abnormalities, triphalengeal thumgs
what is the value of haptoglobin in intravascular hemolysis
it is decreased
what drugs can cause aplastic anemai
ratiaton, benzene, chlorampehnicol, alienating agents, antimetabolites
viral causes of aplastic anemia
B19, EBV, HIV, hepatitis
wy does fanzine anemia cause it
DNA repair defect causing bone marrow failure, short stature, increased tumors, cafe au lair, thumb and radial defects
pyruvate kinase difciency
AR defect in pyruvate kinase, so decreased ATP so rigid RBC that cause extravascular hemolysis
what causes neutropenia
sepsis, infection, drugs like chemo, aplastic anemia, SLE, radation
lymphopenia causes
HIV, Digeroge, SCID< SLE, corticosteroids, radation, sepsis, postoperative
eosinopenia- causes
cushing, corticosteroids
left shift
increased neutrophil precursors like band cells and metamyelocytes in peripheral boo usually seen with neutrophilic in the acute response to infection or inflammation. called the leukoerythorbalasti reaction when left shift is seen with RBC occurs with severe anemia or marrow fibrosis or tumor taking up space in the marrow
lead poisoning takes out which enzymes, and caues accumulation of what, and presenting symptosm
ferrochelatase, ala dehydratase
accumualte- delta ala
microcytic anemia ith basophilic stippling and GI and kidney disease
-children- mental dterioration
-adults- environmental exopsure- headache, memory loss, and demylination
acute intermittent porphyria takes out which enzymes, and caues accumulation of what, and presenting symptosm
prophobilinogen deaminase
accumulate porphobilinogen and delta ala
symptoms- painful abdomen, port wine urine, polyneuropathy, psych disturbance, precipitated by drugs, alcohol, starvation
porphyria cutana tarda-takes out which enzymes, and caues accumulation of what, and presenting symptosm
uroporphyrinogen decarboxylase
-uroporphyin builds up leading to tea colored urine
blistering cutaneous photosensitivity
mercury poisoning
accumulates in the kidneyy and brain with the pealing of fingertips, abdominal pain from large fish and common in the fetus from maternal ingestion
what do you use packed RBC for
acte blood loss and severe anemai- increased Hb and O2 carrying capacity
what do you use platelets for
increased platelet count, stop significant bleeding from thrombocytopenia and qualitative platelet defects
what do you use FFP for
increased coagulation factor levels- DIC, cirrhosis, immediate warfarin reversela
what do you use cryopercipitate for for
cogatulation deficiency like fibrinogen and factor VIII
what can happen from blood transfusions (Aka the risks)
infection, transfusion reaction, secondary hemochromatosis, hypocalcemia (citrate is a calcium kelator), and hyperkalemia (RBC may lyse in old units)
what comes from the lymphoid cell line
B and T cells
what comes from myeloid cells
RBC, neutro, baso, eosino, mono, megakaryocytes
low number of bone marrow cells
penia
high number of bone marrow cells
cytosis
neutropepnia
drug toxicity, sever infection treat with stumualating factors
lymphopenia
immunodeficiency like Digeorge or SCID, increased cortisol which increases apoptosis of these cells, SLE, whole body radiation. Lymphocytes are most sensitive to radiation
neutrophilic lymphocytosis
high neutrophils and this is from bacterial infection, tissue necrosis, increased cortisol which prevents margination.
left shift
immature are pumped out and so there is no Fc portion receptor so cannot function as well CD16 positive
monocytes
chronic inflammation and malignancy
eosinophilia
allergic reactions, parasites, or Hodgekins lymphoma
why does eosinophilia happen in Hodgekins lymphoma
increased IL5 causes the chemotaxis of eosinophils
what is basophilia associated with
CML
lymphocytic leukocytosis
CD8 and T cells increased from viral infection or broadtail pertussis.
infectious mono
EBV creates a lymphocytic leukocytosis of CD8 and T cells.
what part of the LN swell and spleen during mono
CD8 response and Lad is in the paracortex, splenomegaly at the PALS, weird CD8 cells
monospot
IgM heterphile antibodies.
what percentage blasts does it have to be for it to be leukemia
over 20% blasts
what do leukemia cells look like
they are large WBC with no cytoplasm, and punched out nuclei.
what is the specific marker for AML and ALL
AML- myeloperoxidase
ALL- tdt
ALL
lymphoblastic with TdT cells- DNA polymerase in the nucleus- children with downs after 5
CD10, 19, 20- good response to chemo but must add to the testes and CSF because they seed these areas
B cell ALL
thymic mass in a teenagers
T cell ALL with CD2 and CD8
t 12;21
B cell ALL
MPO+, Auer Rods and 50-60 years old
AML
what is the common mutation for AML
it is 15;17- APML- disrupt retinoid acid so cells font mature leading to DIC
infiltration of the gums and large swollen gums- type of leukemia
acute monocytic leukemia
lack of MPO and see in Downs before 5
acute megaloblastic leukemia
what leukemia do you get from previous radiation
AML can occur with cytosine, hyper cellular BL, div\e from infection or bleeding
naive B cells- CD5 and CD20 positive- increased lymphocytes and smudge cells
CLL
where can CLL go
it go to the lymph nodes and it is small lymphoblastic leukemia
what are the complications of CLL
hypergammaglobulinemia (low global level so increased infections), autoimmune hemolytic anemia from antibodies against RBC, and large B cell lymphoma, and patient has enlarging LN or spleen
TRAP positive with large spiky blue cells
hairy cell leukemia
what are the clinical findings for hairy cell
splenomegaly of the red pulp, dry bone marrow tap, and lymphadenopathy
what do you treat it with
2-CDA adenosine deaminase inhibitor so increased adenosine to toxic levels in B cells
leukima and lymphoma in a patient from Japan or the Carribena with HTLV1- lytic bone lesions, hypercalciia and rash
ATLL
rash, plaques, nodules, paether microabscesses, T cell in the epidermis and blood cells with cerebra form nuceli
mycosis fungoides
what levels can be increased in blood with myeloprofilerative disorders
gout and hyperurecimia with myelofibrosis going to acute leukemia
basophil increased, 9;22 translocation with bcr abl increase tyrosine kinase, splenomegaly,
CML
what is the usefulness of LAP
LAP can determine if cells are for fighting an infection (if positive) or if negative indicates more like leukemia
RBC with JAK2 kinase mutation, blurry vision, headache, hyper viscous blood, increased thrombu and Budd Chiari, flushed face, itch after bathing from mast cell degranulation- can progress to CML and AML
polycythemia vera
how to treat polycythemia vera
phelbotmany, hydroxyurea
what can be reactive polycythemia
it can be from increased EPO from lung disease or tumor RCC produce increased EPO and decreased EPO in polycythemia vera
increases platelets- Jak2 kinase and ton of playlets on smear with increased bleeding and thrombosis, no increase in uric acid- no increase for CML and AML
essential thrombocytopenia
Jak2 kinase leads to increased megakaryocutes and myelofibrosis- fibrotic BM with spenomegaly from increased heamtopoessts, increased risk of infection, thrombosis, and bleeding- tear drop cells are present
myelofiboris
painful lymphadenopathy indicates what
draining infection
painless lymphadenopathy inducates what
chronic infection, carcinoma, lymphoma
what does follicular enlargement of LN represent
rheumatoid arthritis and early HIV
what does paracortex enlargement of LN represent
viral infection
what does sinus histiocytes enlargement of LN represent
LN draining tissue with cancer
small cell lymphoma types
follicular, mantle, marginal
neoplastic small b cells with follicle like nodes with 14:18 translocation.
follicular LN
treatment for follicular LN
symptomatic rituximab. Anti-CD20- so can do to diffuse large B cell lymphoma
neoplastic cells CD20 in adults, large LN, expand area next to follicle- 11;14 transaction of cyclin D. so stops G1 to S phase
mantle lymphoma
CD20- chronic inflammatory states like hashimotos, sjogrens, h pylori, MALToma,
marginal lymphoma
EBV related lymphoma with extra nodal jaw mass or abdomen with a comic 8;14 translocation
Burkitt lymphoma
agressive not well differentiated lymphoma
diffuse large B cell lymphoma
Cd15, CD30- with owls wye cells and fever, chill, night sweats- RS cells.
hodgekin lymphoma
cervical or mediastinal mass in young woman with RS cells
nodular scelorsis- pink bands of fibrosis in the LN
which is a better prognosis lymphocyte risk or poor
rich
increases eosinophils and IL5 with RS cells
mixed cellularity
what cytokine is increased with multiple myeloma
IL6
mutliple myeloma
clast activating factor with punched out lesions, increased fracture risk, and increased immunoglobulin with increased serum protein and M spike from monoclonal immunoglobulin. IgG or IgA is spike and all the same so increased infection rate. Rolex form from loss of charge. Get primary amyloid from too much light chain free deposits in tissue and this can lead to bench jones proteins in the urine and deposit in the tubules leading to myeloma kidney
MGUS
m spike but no secondary features but can move to multiple myeloma
walderstrom macroglobulinemia
monoclonal IgM generalized LAD and increased M spike from IgM. Retinal hemorrhage, stroke -hyperviscous blood and cant aggregate- use plasamphoresis
Lagerhands cell histiocytosis- what are the cells positive for
Cd1a and S100
letterer Sieve
malignant skin in under 2year old and skin rash, cystic skeletal defect and rapidly fatal
eosinophilic granulomatosis
pathologic fracture of adolescent with lots of eosinophils
Han Schueller Christian
lytic skull, diabetes insipidus, exopthamosis, malignancy of langerhans cells, skull rash in children over 3
Pseudo Pelget Huet anomaly
neutrophils with bilobed nuclei. seen after chemotherapy
what can be a complication of AML
DIC and fibrinogen
what does the neuroectorderm form in the CNS and PNS
CNS neurons, ependymal cells, oligodendroglia, astrocytes
what does the neural crest form in the CNS and PNS
PNS neurons and schwann cells
what does mesoderm form in the CNS and PNS
microglia like macrophages
when do the neuropores fuse
15-40 days so can be persistent for connection. low folic acid. see increased alpha fetoprotein in amniotic fluid in maternal serum.
what levels are increased with NT defects
this is with increased acetylcholinesterase and alpha FP
spina bifida occulta
failure of bony spinal canal to close but without herniation seen more in the lumbar plexus. associated with overlying tuft of hair, dimple, or body defect. no increase in levels
meningocele
meninges but no neural tissue herniate through bony defect
meningomyeloccele
meninges and neural tissue like the caudal equine herniate through the bony defect
anacephaly
malformation of the anterior neural tube- no forebrain open calverium. Increased AFP and polyhydramnionsno swallow center in the brain so initial deformation leads to further deformations
holoprosencephaly
fairelu of the left and right hemispheres to spirit usually occurs at 5-6 weeks and this is related to sonic hedgehog mutations. Moderate is cleft lip and palate. most severe from results in cyplpia seen in PAtau syndrome and FAS
Chiairi II malformation
herniation of low lying cerebellar vermis through foramen magnum with aqueduct stenosis increased hydrocephalus- associated with lumbosarcral myelomeningocele
Dandy walker phenomenon
agencies of the cerebellar vermis with cystic enlargement of the fourth ventricle. It is associated with noncommunicating hydrocephalus and spina bifida
Chiari I malormation
only the cerebellar tonsil herniate and it is associated with syringomyelia
syringomyelia
cystic cavity within the central canal of spinal cord. Fibers crossing in the anterior white commissure which are the spinothalamic and these are damaged first and this causes the cape like bilateral loss of pain and temperature sensation in the upper extremeties with the fine touch and sensation preserved.
where is the most common location of a syringomyelia
C8-T1
what is sensation of the anterior tongue and taste
V3 for sensation and taste VII
what is the sensation and taste on posterior tongue
sensation and taste on IX
why does spinal stenosis have pain that gets better on leaning forward
pain of claudication goes away if lean forward and ligmentum flavum is hypertrophied
what is nisll substnace
it is the increased rough ER that is in the cell bodies. The ret is not in the axons
wallerian degeneration
degeneration distal to injury and axonal retraction proximally; allows for potential regeneration in the PNS
astrocytes
pyhsical support, repair K metabolism, removal of excess neurotransmitters, competent BBB, glycogen fuel reserve, reactive gloss in response to injury. marker is GFAP
microglia
phagocytic scavenger cells of the CNS. It is mesodermal, mononuclear origin, activated in response to tissue damage-
what is the major brain changee seen in HIV
microglia fuse to form nodules
what are the microglia derived from
mesoderm
myelin
increases the conduction velocity of signals transmitted down the axon, saltatory conduction of action potential at nodes where there is increased Na channels.
what are the myelin in the PNS and CNS
-PNS- schwann cells
CNS- oligodendrocytes
schwann cells
only myeline one PNS axon. Promotes axonal regeneration. It is from neural crest.
what can the schwann cells be injured by
GBS
where is a vestibular schwanoma located and what is it associated with
MF2 and in the internal acoustic meatus
oligodendroglia
myelinated the icons of neuron in the CNS. Myeline many axons. Predominate glia of white matter
what do oligodendrocytes look like on histology
fried egg cells
what are some of the reasons why oligodendrocytes damages
MS, OML, leukodystrophy
free nerve endings are: sensory neuron fibers, location, senses
C is slow unmyelinated and ad are the fast myelinated
all skin, epidermis, viscera
pain and temperature
meissner corpuscles: sensory neuron fibers, location, senses
large myelinated fibers that adapt quickly. Glabrous hairless skin, dynamic fine light touch and position sense
pacinian corpuscles: sensory neuron fibers, location, senses
large myelinated fibers that adapt
deep skin layers, ligaments and joints
vibration and pressure
merkel discs: sensory neuron fibers, location, senses
large myelinated fibers that slowly adapt
fingertips and superficial skin
pressure, deep static touch, sharp edges, and position sense
ruffini corpuscles: sensory neuron fibers, location, senses
dendritic cell endings in the capsule
finger tips and joints
pressure, slippage of objects along surface of skin, joint angle change
endoneurium-
invests a single nerve fiber layer- inflammatory infiltrate in GBS
what layer of the nerve is inflated in GBS
it is the endoneural inflammation of the peripheral nerve
perineurm
sururondthe fascicle of the nerve fibers. must be rejoined in microsurgery for limb retacchement
epineurium
dense connective tissue that surrounds entire nerve
where is the locus cerileus located
it is located i n the floor of the fourth ventricle
what nucleolus secreted serotonin
raphe nucleus target of SSRI and SNRI
what is the nucleus secreting GABA
nucelus accumbens
what are the layers of the BBB
- tight junctions between nonfenestrated capillary endothelial cells
- basement membrnae
- astrocyte foot processes
where is the area postrema and what does it do
it is the area of the medulla not protected by BBB in the area of the fourth ventricle that is for vomiting control
what drugs decrease REM sleep
alcohol, benzos, and barbs
awake has what EEG waves
beta
awake eyes closed what EEG waves
alpha
N1 what EEG waves
theta
N2 what EEG waves
sleep spindles and K complexes- this is where the benzos and alcohol hits
N3 what EEG waves
sleep walking, night terrors, bedwetting- delta waves
REM
loss of motor tone, and increased brain use of O2 and increase in variable pulse and BP when dreaming, nightmares, penil/clit tumescence occur and may serve as memory processing fucntion
sleep hygeine
sleep schedule, no caffeine, no alcohol, no smoking, no large meals dark cool bedroom and exercise
stimulus control for sleep hygiene
use bed for sleep and sex only in bed when sleepy, leave bed if can’t sleep. fixed wakeup time
relaxation for sleep
progressive muscle relaation, relaxation response
sleep restriction
restrict sleep to time pateint is lesep. increase time in 30 minute intervals
