Day 9 Pediatric Gastroenterology

Patient 1: Crohn's disease 
SMA (SMA in full form Spinal Muscular Atrophy is a crohns disease with multiple disorders, all having in common a genetic cause and the manifestation of weakness due to loss of the motor meurons of the spinal cord and brainstem.)SMA in full form Spinal Muscular Atrophy is a crohns disease with multiple disorders, all having in common a genetic cause and the manifestation of weakness due to loss of the motor meurons of the spinal cord and brainstem.)

6 or more bloody stools plus one other symptom is UC
Type 2 diabetes

Treatment: 

Rafassal (Infliximab) was given 2-gram, symp worsened 
Predsisone for 
bloody diarrhea  fever vomit
IV cipro and flagyl improve 2 d then 
worse.
No uc no cmv
Iv1wk hosp hydrocortisone ab ampicillin 
gentamycin
Imuran after 1 wk after release 
Swich to azathioprine,porin 
idk
Uc 3yrs
Infliximab no resp steriod
Fever
Hosp. Ab 
hydrocort,azathiprin,
And pain 
CT
Antibiotic: vancomycin
Doxycyclin
Amoxicillin
Metronidazol

Sigmoidoscopy, protocolectomy
Cortisone try improve mortality 
Steroid resistant uc,,, salvage therapy
Fail for salvage therapy, then what?
If patient spends a longer time in the hospital before sugery, statistically, they will have more complication so we need to predict which patients will have the complications so we can give surgery at a sooner time

3rd day surgery responded better.
Thiopurine preventative in future colectomy
Tacrolimus SIM to cyclosprorin
---decreases recruitment t cells 
Infliximab vs cyclosporin
Equivelant, what to choose?
Advantages and disadvantages
Infliximab, comfortable dosing schedule, long term and safe
TLR new trmt?

Patient 2: Vitamin D deficiency in nonalcoholic liver disease

Rickets
Cod liver oil and sunlight prevent
30-60 ng/ml is minimum daily value

Vitamin D deficiency is associated with many diseases, not only rickets: 
UC 
Dvdr system
Vitamin D decreased many cancers, bacterial infections, RA,MS,cardiovascular disease, chronic kidney disease, muscle weakness, cognitive impairment, alzhemers, depression, diabetes, 
But not all vitamin D is alike...

Vitamin D responds to HCV treatment.

NAFLD
Regenerating nodules, Mallory bodies, 
Relationship of vitamin D deficiency has one or more prevalent disease....

fatty liver is first stage of hepatitis. 

Patient 3: 5 yr old boy swallowed a plastic cube, not seen on x-ray-- radioluminescent
size: 2x2cm cube
2.5 cm remove, unless esophagus wait 24 hrs, unless choking bf removal.child 2 yrs guidelines.

Patient 4: Pneumonic pulmonary restrictive disease
Manual breathing 
Infiltration complication restrictive
Muscles seizing up
Pulmonary massage, not chest compression.
Tracheal tube
Deep pumps, short just gets ride of dead space

Patient 5: Micrvillous inclusion disease
Enterocyte probably
Treatment: TPN for life, intestinal transplant.
Can cause liver damage

Patient 6: cholestasis- 16 yr old boy
infliximab
Lab normal 
On cypro flagyl
3rd US for abcess because draining was not successful
Ecoli positive
Stricture, need theater OR 
pain brought him in for 3rd US
Antibiotics trimocane? ciproflagyl
Remacade for treatment
Metronidizol used as a case study
No Sharp pain, no suggestion of blockage at stricture. 

Patient 7: Atresia cynical mengitis
IV trmt 
Fever week later
CRP, ESR raising
Liver biopsy and liver fluid.
X ray 
Start IV antibiotics
Bloods, hemoglobin dropping, infection makes drop, too quick for anemia of chronic disease,,,bone suppression, not clear what he has,,,
Picolyn antibiotics start today.
Biliary atresia, need transplant.
Bile leak caused by pressure possibly, recect section of partial serotoc netrotic liver.
Klebsiella
Could not find infection for 2 months
Surgery to drain biliary track to colon.
Had a cough, related? Nasogasteral to see if virus
Bilirunin climbing, other liver enzymes ok
Maybe diagnose with another bat of ascending cholingitis and treat it? Mom wants to wait cause she thinks its something else.
Changed from cypro to other and still anemia, so think its the infection causing it, not the meds. 

Reaction
Tube into bile for drainage, can try it if need be.

Patient 8: Intermittent colitis and hepatitis B overlap
Note high fructose corn syrup same as sucrose, basically don't eat too much fructose, impairs satiety causes over eating and diseases. Fruit fructose level safe

patient with Congenital Microvillus Atrophy

In congenital microvillus atrophy, diarrhea starts in the first few days of life and is immediately life threatening

TPN for life, K+ high so need to change amount in IV fluids.

Gave consent for sending bloodwork to Austria for genetic testing.

Many cousins died from severe diarrhea, so most likely CMA.

 

The hallmark of the disease is the electron microscopic finding of disrupted enterocytic microvilli (ie, digitations of the apical membrane of the intestinal epithelial cell protruding into the lumen) and the appearance of characteristic inclusion vacuoles, the inner surfaces of which are lined by typical microvilli. Both lesions are seen only on electronic microscopy. In a notable percentage of consanguineous families, more than one child is affected; therefore, the disease appears to be transmitted as an autosomal recessive trait.   http://emedicine.medscape.com/article/928100-overview

patient 9: Diarrhea and lung problem, think about joining them together? test for CF

Liver enzyme high.
Alt, GGT 300 norm 55....both high
LDH bit high
GGT specific for cholistasis, hepatitis,,,will also see bilirunine high
But bilirubine normal
Hemolysis in test tube...prob shook tube that's why LDH high.

Elevated alt phosphatase norm. Bone or liver?
Test for GGT if norm not from liver...
Could be from bone break down disease or Ricketts
Eulysis syndrome 

Patient 10: 2 month old cholestasis patient obstruction of bile.
Dizoxide interferes with insuline release ,,,.
Lab:bilirubin raised alkaline phos raised.
IVC clot on US
Biard Chiary syndrome
Clot above liver, portal htn?
Hyperinsulinism
Pale stools sign of atresia BC bilirubin green dark stools
Also has PDA due for operation
Congested liver hepatic vein obstruction narrow ivc
Ggt low think DD pfic cholestasis

After hours liver clinic ppl sent from other places like west bank a d palestine

Patient 11: 

Liver kidney transplant at 9yr old, now 24
Polycystic kidney, polynephritis
Cyclosporin
Prednisone-since trand
Cypriflaxin because of cholengitis and cholocystitis 
Folic acid
Vitamin D
Sodium bicarbonate
Iron
Calcium
Stones in bile duct, two US now says one stone. 
Arpkd disease
Esk disease
Hepatic encephalopothy
Liver Lobe from mom
Kidney from aunt
Ascending cholengitis
Ggt elevated 4-500
Continue w cyprofloxin to prevent cholingitis? Don't want to make bacteria resistant, so decide wither to stop or not....
PE spider nevi check, and lymph nodes check finger nails check for clubbing.
Ggt elevated means the stone is blocking, so runs risk of infection,,,should we improve flow w ersalate, our body naturally produces it. Flow better less likely inf. Few month if glow better then stop antibiotics.
Try to get off cypro and on ersalate.
Joint pain.
Ursyolic colic acid 500 mg 2times a day. 3 months, us and complete blood lab, if works than quit ciprofloxin.
Can't drink grapefruit BC cyclosporin delayed by grapefruit juice...will make blood levels jump up.
Christian Arabic fastest shrinking religuos group in world in Bethlehem

Patient 12: Biliary atresia? Has cholestasis, but things getting better on own, which is often seen, just has minor obstruction.
Baby girl
allegile syndrome Canadian discovered not long ago.
PE hands nails,
http://en.m.wikipedia.org/wiki/Alagille_syndrome
US and repeat bloods in 4 weeks.
Cholestatic patient baby newborn, did biopsy
Biliary atresia
Not enough bile ducts for bile to drain through 
Stitch intestine to liver and make anastomoses called katai procedure from Japanese guy.
Ersolate atolic acid meds
I gave PE...liver big and hard eyes yellow skin yellow umbilical hernia.
4months from started chronic diarrhea, microcitic anemia, celiac negative, but be wary of false negatives 97% true beg, others false negative. off and on chronic diahrrea. Ferritin low, 
Family history:
Mom has hypercoagulation
PE look for fat stools. 
Ciliac gluten absorption problem.
Gastroscopy needed

Malabsorption, maldigestion.
Fat globules-maldigestion
Fat cristals mal absorption