Bone and joints 2

Question 1

what is marinas syndrome and what are the 3 key features ?

Answer 1

• Autosomal dominant genetic
• affects the connective tissues in the body.
• skeletal, cardiovascular, neuro-occular
• rare
• 1 in 10000
• difficult to diagnose
• loose joints

Question 2

what are the skeletal features?

Answer 2

tall and thin body, a characteristic Marfanoid habitus, with an elongated armspan and long fingers (arachnodactyly).
loose joints
sometimes TMJ dislocation and malocclusions
peptic escavatum

Question 3

what are the cardiovascular features ?

Answer 3

• 90% experience= mitral valve prolapse and aortic dissection (life threatening)
• dilatation of descending aorta
• aortic regurgitation

Question 4

what are the neuro- ocular features ?

Answer 4

• Lens dislocation can occur, leading to vision problems.
• Associated with a mutation in the fibrillin-1 gene.
• Aortic dissection, retinal detachment, scoliosis.
• Increased risk of aortic aneurysms.
• Near-sightedness (myopia) is common.

Question 5

what are the key features of Ehlers danlos syndrome?

Answer 5

Rare genetic disorders affecting collagen formation.
- Autosomal dominant.
Clinical Features:
- Hyperextensible Skin: Elastic and fragile skin.
- Easy Bruising: Due to skin fragility.
- Loose Joints: Joint hypermobility and recurrent spontaneous dislocations
-.Impaired wound healing.
+Various Subtypes: EDS has different subtypes
- Mitral Valve Prolapse: Cardiovascular involvement.

Question 6

what are the dental aspects if Ehlers danlos syndrome ?

Answer 6

• Haematological Issues: Increased bleeding tendency.
• TMJ (Temporomandibular Joint): Joint hypermobility affecting the TMJ.
• Dental Abnormalities: Short teeth, abnormal roots, pulp stones.
• Micrognathia: Abnormally small jaw.
• Anterior Open Bite (AOB): Malocclusion with a gap between upper and lower front teeth
  -Gingival Hyperplasia: Overgrowth of gum tissue.

Question 7

What characterizes Osteoarthritis (O.A.) ?

Answer 7

• Degeneration of articular cartilage with the proliferation of new bone, cartilage, and connective tissue.
• Remodeling at joint contour with pain, stiffness, and swelling.
• No systemic symptoms like Rheumatoid Arthritis (RA).
• More common in women; genetic predisposition. - usually if overweight or obese
• Can impact any joint, especially weight-bearing joints.
• Usually starts in one joint and may spread.
• Unlike RA, there is no obvious inflammation in the joints.
• painful
• over 45

Question 8

management of osteoarthritis

Answer 8

• Analgesia:
• In severe cases, joint replacement surgeries may be considered.

Question 9

What are the key characteristics of Rheumatoid Arthritis (RA)?

Answer 9

• Common chronic inflammatory joint disease affecting all ethnic groups, predominantly females.
• Symmetrical, destructive joint involvement.
• : Possibly related to T-lymphocyte activation; multi-system, immunologically mediated disease.

Question 10

clinical features of RA?

Answer 10

Morning stiffness - worse in morning , joint pain, swelling, ulnar deviation, and increasing disability; cervical-spine involvement.

Question 11

how do we diagnose RA?

Answer 11

Clinical: Pain, swelling, heat, stiffness in affected joints, nodules, and systemic signs.
- Lab Tests: Rheumatoid Factor (RF) present in 60–70%, inflammatory markers (ESR, CRP), and radiographic features.
- arthritis in more than 3 joints
- widening in radiograph but narrower later on
- erosion of joints

Question 12

how do we manage RA?

Answer 12

Supportive Measures: Including analgesics (NSAIDs, Paracetamol), and corticosteroids.
- Disease-Modifying Anti-Rheumatic Drugs (DMARDs) & Biologic Agents: (reduce pain and swelling) Methotrexate, sulfasalazine, hydroxychloroquine, leflunomide, azathioprine, Etanercept, Adalimumab, Infliximab. = biologics can cause toxic side effects

Question 13

what are the dental issues related to RA?

Answer 13

Often associated with Sjogren’s syndrome.
- Some changes on OPT within TMJ.
- Medication Risks: Care needed due to risks of bleeding, infection, and medication-related osteonecrosis of the jaw (MRONJ).
- biologics can affect healing if carrying out surgery

Question 14

what is felts syndrome?

Answer 14

Rare complication of RA with splenomegaly and neutropenia, more common in Caucasians.
- More in women than men

Question 15

What are the key features of Juvenile Arthritis?

Answer 15

Systemic, Polyarticular, Pauciarticular.

Question 16

prevalence of juvenile arthritis?

Answer 16

mainly affects girls in late childhood

Question 17

presentation of juvenile arthritis ?

Answer 17

Involves any joint with associated symptoms such as fever, nodules, anaemia, and malaise.

Question 18

how do we diagnose juvenile arthritis?

Answer 18

Rheumatoid Factor (RF) is positive in approximately 75% of patients, and the condition presents with issues similar to those seen in adults.

Question 19

What are the key features of Ankylosing Spondylitis, including its areas of involvement, consequences?

Answer 19

A form of chronic inflammatory arthritis affecting the sacroiliac and spinal joints.
Causes fusion of vertebrae over time.
- seen in young males
- family history
- limited chest expansion
- may get anaemia and ASR raise

Question 20

symptoms of ankylosis spondylitis ?

Answer 20

Mainly presents with back pain and stiffness.
- Additionally, patients may develop eye lesions (25%) and cardiac disease (10%).

Question 21

tx for ankylosis spondylitis?

Answer 21

physiotherapy, exercises, and the use of NSAIDs. Anti-TNF medications may be considered in some cases

Question 22

dental considerations for ankylosis?

Answer 22

may require special care during general anesthesia. esp when positioning

Question 23

What is Gout, and what are its causes?

Answer 23

• Crystals: It involves crystals of monosodium urate monohydrate derived from hyperuricemic body fluids.
  can cause chronic inflammatory arthritis, tenosynovitis, bursitis or cellulitis, tophaceous deposits, urolithiasis, and renal disease.
  High uric acid levels, often due to the breakdown of purines found in foods like red meat, organ meats, and certain seafood.

Question 24

what conditions are associated with gout?

Answer 24

high BP - hypertension
diabetes
obesity

Question 25

tx for gout?

Answer 25

NSAIDs are commonly used for pain and inflammation management.
- Allopurinol is a medication that helps lower uric acid levels and prevent gout attacks.