GI #11 Flashcards
What bacteria causes Whipple’s Disease?
-Tropheryma Whipplei: gram positive non acid-fast positive rod
Transmission of Whipple’s Disease
-Contaminated soil (most commonly seen in farmers)
Symptoms of Whipple’s Disease
- Malabsorption: chronic diarrhea, weight loss, steatorrhea, fever, LAD
- Nondeforming arthritis: (doesn’t change shape of bones)
- Neurologic symptoms: memory impairment, confusion, rhythmic motion of eye while chewing
How do you diagnose Whipple’s Disease?
- Duodenal biopsy: periodic acid-Schiff (PAS)-positive macrophages
- -Dilation of lacteals in small intestine
- -Non-acid-fast bacilli
Treatment for Whipple’s Disease
- ABX therapy is prolonged (1-2 years): Penicillin or Ceftriaxone followed by maintenance therapy of Bactrim orally
- Alternative: Doxy + Hydroxychloroquine
What is G6PD deficiency?
An X-linked recessive enzymatic disorder of RBC’s that may cause episodic hemolytic anemia
What are some risk factors for G6PD deficiency?
- Primarily males (X-linked recessive)
- 10-15% of African American males
- Mediterranean, African, Middle Eastern, SE Asian
What is the pathophysiology of G6PD deficiency?
- G6PD normally catalyzes NADP to NADPH which protects the RBCs from oxidative injury
- In this condition, decreased G6PD activity results in oxidative form of Hb (methemoglobin) which predicates as Heinz bodies
- RBC membrane damage and fragility causes hemolysis by macrophages
What are some exacerbating factors for G6PD deficiency
- Infection (MCC)
- Fava beans
- Medications: Dapsone, Primaquine, Methylene blue, Nitrofurantoin
- Sulfa drugs at high doses
True or False: Most patients with G6PD deficiency are asymptomatic until oxidative stress?
True
What are some symptoms of oxidative stress in G6PD deficiency?
- Symptoms begin 2-4 days after exposure
- Back or abdominal pain
- Anemia, jaundice, dark urine
- Neonatal Jaundice
What is seen on a peripheral smear for G6PD deficiency?
Normocytic hemolytic anemia only during crises
- Schistocytes (bite or fragmented cells)
- Heinz bodies (HALLMARK)
- -Smear is usually normal when not in acute stage
What is the management for G6PD deficiency attack?
Usually self-limited, avoid offending foods and drugs
-If severe anemia, iron and folic acid supplementation
Treamtent for neonatal jaundice
Phototherapy is first line
-Exchange transfusion if refractory
What is Paget Disease of the Bone (Osteitis Deformans)
Abnormal bone remodeling (increased osteoclastic resorption and increased osteoblastic bone formation). This leads to larger, weaker bones
Who is at risk for Paget Disease?
- Western European descent
- > 40 years old
Symptoms of Paget Disease of the bone
- Most asymptomatic (found incidentally due to high alkaline phosphatase on labs)
- Bone pain (MC)
- Skull enlargement: deafness due to compression of CNVIII
- Headache
What is the hallmark lab finding for Paget Disease of the bone?
-Isolated markedly increased alkaline phosphatase
What do skull radiographs show in Paget Disease of the bone?
-Cotton wool appearance: sclerotic patches that are poorly defined and fluffy
How do you treat Paget Disease of the bone?
- If asymptomatic, no treatment is needed.
- Bisphosphonates are first line management.
- NSAIDs for pain
- Calcitonin
What is given during bisphosphonate treatment to prevent hypocalcemia?
-Vitamin D and Calcium supplementation
What is the mechanism of action of Bisphosphonates?
-Inhibits osteoclast activity (decrease bone resorption and turnover)
What is special about Aledronate and Risedronate?
They inhibit both osteoclast and osteoblast activity
Osteoblasts:
Osteoclasts:
Blasts build
Clasts break down
