Abdos

Question 1

Abdo Pain NYD

Answer 1

Acute Intermittent Porphyria (AIP)
Etiology]
Autosomal dominant disorder with low penetrance.
A mutation in the PBGD (Porphobillinogen deaminase) gene
results in disturbed heme synthesis. PBGD is necessary but not
sufficient for the AIP. Heme synthesis begins in the mitochodria,
then enters the cytoplasm, and ends in the mitochondria. Porphobillinogen
deaminase functions in the production of heme in the cytoplasm. When
the enzyme is deficient, the metabolite porphobillinogen accumulates.

Heme is used in hemoglobin production and cytochrome P450 liver
enzymes. In AIP, PBGD always affects the hepatic housekeeping enzyme.
Central, peripheral, autonomic and enteric nervous system is affected when
metabolites build up.

HPI]
abdominal pain
vomiting
psychiatric symptoms
constipation
muscle weakness

O/E] TC
Abdos – diffuse abdominal pain

INVESTIGATIO]
L(O)/Haim]
Dark or reddish brown urine is often a precurser
to an attack. Porphyrin or phorphyrin precursers accumulate
in the urine and can be mistaken for blood.

Familial Mediterranean Fever (FMF)
Etiology]
Hereditary autosomal recessive autoinflammatory disorder.
Among Armenians the carrier rate is 1/500. Among the Jewish
population in Israel, the carrier rate varies 1/8 in those of
Ashkenazi origin, to 1/6 in those of North African origin,
to 1/4 in those of Iraqi origin.

The MEFV gene encodes pyrin, a 781 amino acid protein that is
expressed predominantly in the cytoplasm in cells of myeloid
lineage among circulating cells predominately neutrophils,
along with synovial fibroblasts and dendritic cells. The protein appears
to act as an intranuclear regulator of transcription of the peptides involved
in inflammation.

The exact mechanism triggering the acute attacks in familial
Mediterranean fever (FMF) is unclear, but several lines of evidence
point to the **neutrophil** as the effector of the **inflammatory response
at serosal surfaces.**

Colchicine, which is effective in preventing attacks in
FMF, is known to act by suppressing neutrophil phagocytosis and
chemotaxis in gout.

HPI]
Abdominal pain
joint pain
chest pain
fever

O/E] Fever
Dermatos – Erysipelas-like skin lesion

INVESTIGATIO]
L(H)/Infla] ESR, CRP, Fibrinogen (acute phase reactants)

Question 2

Abdo Pain

Answer 2

DDx
Haim]
Mesenteric ischemia – classic triad – sudden pain poorly localized and out of proportion to exam (visceral pain), gut emptying (V, D), cardiac disease – late peritoneal signs, subacute with gradual development and vague symptoms, SMA embolism (50%) dislodged from LA (A fib), LV, heart valves; non-occlusive ischemia (20-30%) low flow state from cardiac failure, cocaine, digoxin, dialysis; mesenteric venous thrombus (5%), hypercoaguable state

Org(Vasculo)]
AAA – usually asymptomatic until rupture, epigastric and back pain, can mimic renal colic and diverticulitis, wide pulsatile abdominal mass, risk factors are age, male, HTN, smoking, CAD/PVD, syncope and shock

Org(Intestino)]

• *Gastritis** – epigastric pain, N, V, anorexia
• *GERD** – cough, heartburn, atypical angina
• *PUD** – epigasric pain worsened by food (gastric, increased stomach acid), or relieved by food (duodenal, brunner glands release HCO3), with back pain
• *Bowel obstruction** – colicky abdominal pain, abdominal distention, vomiting (bilious, feculent), obstipation/constipation
• *Bowel perforation** – sudden onset, sharp and severe abdominal pain, peritoneal signs (rigidity, rebound, guarding), motionless on bed, in distress
• *Diverticulitis – hypogastric pain that migrates to LLQ (like appendicitis),** fever, WBC, D,
• *Appendicitis – periumbilical abdominal pain that migrates to the LRQ,** N, V, anorexia, localized peritoneal irritation (McBurneys point), Rosvings (LLQ palpation produces RLQ), Psoas (active flexion of hip, or passive extension reproduces pain – retrocecal appendix), Obturator (flexion and internal rotation of hip reproduces pain), fever, tachycardia, WBC, CRP, Appy triad – WBC, CRP (inflammatory markers), peritoneal irritation, migration of pain (epigastric to RLQ)

Org(cholecystos)]
Biliary colic – obstruction of cystic duct by gallstones, symptomatic cholelithiasis, episodic pain in RUQ lasting minutes to hours, precipitated by meals
Cholecystitis – obstruction of cystic duct by gallstones, symptomatic cholelithiasis with inflammation, pain >6 hours, N, V, murphys sign (localized peritonitis over gall bladder causing the arrest of inspiration on gall bladder palpation), 1/2 will improve spontaneously in 7-10 days, 1/3 will worsen to choledocholithiasis and cholangitis, gallbladder wall thickening >3mm, pericholecystic fluid
Choledocholithiasis – CBD obstruction, similar presentation to cholecystitis except elevated Total Billi, elevated Alk phos, jaundice
Ascending cholangitis – CBD obstruction (choledocholithiasis) causing infection proximal to obstruction, Charcot’s triad – RUQ pain, jaundice, fever – Reynolds pentad – charcots triad, HypoTN, ALOC
Gallstone pancreatitis – CBD obstruction at the pancrease causing a backup of pancreatic enzymes that irritate and inflame the pancreas, elevated lipase

Org(Pancreos)]
Acute pancreatitis – epigastric pain radiating to back, N, V, fever, jaundice, peritoneal signs, cullens sign (periumbilical eccymosis), gray turner sign (flank eccymosis), common etiology is gallstones and alcohol

Org(Uros)]

• *Uncomplicated UTI (Cystitis)** – young, healthy, non-pregnant women, with normal urinary urinary tract, dysuria, frequency, urgency, suprapubic and low back pain, new or increased incontinance in older patients with AMS/delirium
• *Complicated UTI (Cystitis)** – pregnancy, DM, male gender, immunosupression, fuctional GU abnormality (neurogenic bladder or catheter), structural GU abnormality (stones, fistula, PCKD, transplant)
• *Renal colic** – obstruction of urinary tract, increased pressure leading to relan capsular distention causing visceral pain, N, V, and peristalsis of ureter leading to colicy pain – coming in waves with patient writhing unable to sit still – with flank pain radiating to groin (back pain->flank pain->LQ pain->penile or labial or testicular pain), CVA tenderness

Org(Genito)]
Ruptured ectopic –

I]
Abdo US
AAA
AAA<5.5, no improved survival seen with repair
AAA>5, admit for surgical evaluation
Org(Cholecystos)
Cholelithiasis – gallstones
Cholecystitis – gallstones obstucting cystic duct, gallbladder wall thickening >3mm, pericholecystic fluid
Choledocholithiasis – dilated CBD >6mm
Ascending cholangitis – dilated CBD with charcots triad (and reynolds pentad)
Gallstone pancreatitis – gallstones obstructing pancreatic duct to the small intestine causing enzymes to back up into the pancreas
Org(Pancreos)
Acute pancreatitis – peripancreatic free fluid, diffusely enlarged hypoechoic gland
Org(Intestino)
Appendicitis – aperistaltic and non-compressivle structure >6mm
Diverticulitis – diverticula, abscess, hypoechoic around bowel wall