SAS 4: ORAL & DENTAL CARE OF LOCAL & SYSTEMIC DX

Sickle Cell Disease(SCD)

• amino acid is effected: thymine = adenine / glutamic acid = valine

• Considered as the most common genetic disorder of the blood.

• It is caused by a variant of the B-globin gene called sickle hemoglobin

• Red Blood Cells

  • Normally, the flexible, round red blood cell move easily through blood vessels.

  • In sickle cell disease, the red blood cell are shaped like sickles or crescent moons.

  • These rigid, sticky cells can get stuck in small blood vessels.

  • This can slow or block blood flow and oxygen to parts of the body.

hallmark of Sickle Cell Disease(SCD)

Anemia and vasculopathy – hallmarks of SCD.

Mean life span of a sickled RBC is 12–17 days (normal: 120 days).

Hemoglobin levels: 6–9 g/dL (normal: 12–18 g/dL).

Splenic Sequestration Crisis

• Caused by a large number of RBCs becoming trapped in the spleen.

• Can induce:

  • Sudden and severe anemia

  • Thrombocytopenia

  • Reticulocytosis

Splenectomy

Recommended because in patients with SCD spleen shrivel up and stop functioning.

Post-Splenectomy Sepsis → Caused by Streptococcus pnemoniae.

• Leading cause of mortality among infants affected by SCD.

Intervention of Sickle Cell Disease(SCD)

Administration of Penicillin V potassium 125mg orally twice daily starting 2 months of age to prevent pneumococcal infection. Age 3- dosage is doubled and should be continued until age 5.

Oral and dental manifestations of Sickle Cell Disease(SCD)

Nonspecific to the disease

Jaundice and glossitis of mucous membranes

Delayed tooth eruption

Radiographic Findings of Sickle Cell Disease(SCD)

decreased radiodensity in the bones, coarse trabecular pattern, thin inferior border of the mandible, pronounced lamina dura, dentin hypomineralization, calcifications in the pulp chamber, hypercementosis.

Craniofacial abnormalities of SCA radiographically:

bimaxillary protrusion with flared incisors

Prominent parietal and zygomatic bones(“Tower skull”)

Thinning of the outer table of the calvarium (“Hair-on-end appearance”)

“doughnut lesions”

biphosphonate-related osteonecrosis of the jaws(BRONJ)

must be ruled out from a detailed medical history

Bleeding Disorders

hemophila

Von Willebrand Disease

hallmarks of hemophila

-Hemarthroses, muscle and joint hemorrhages -Easy bruising -prolonged hemorrhage after surgery

hemophila A & B -are the MOST common inherited coagulation disorders

hemophila A

deficiency of plasma factor VIII coagulant activity

hemophila B

deficiency of plasma factor IX coagulant activity

hemophila C

factor XI

Von Willebrand Disease

characterized by abnormal quantity or quality of von Willebrand factor(vWF) -pretreatment should be discussed with a hematologist

most common inherited bleeding disorder

Oral and Dental Treatment Concerns

• Review of medical records and consultation with the hematologist before invasive procedures to avoid bleeding complications.

• No pretreatment is required for supragingival scaling in mild and moderate cases of hemophilia.

• Block anesthesia and certain infiltration anesthesia in severe hemophiliacs should not be performed until hemostatic problem is corrected because they can cause deep tissue bleeding and potential airway obstruction.

Pediatric Osteoporosis

• Bone fractures are the main reason for hospitalization in children between 10 and 14 y.o.

• Characterized by:

  • Low bone mass

  • Microarchitectural deterioration of bone structure

  • Increased bone fragility

Pediatric Cancer

• 2nd most common cause of fatalities in children between 5 and 14 years of age in the US.

Acute Lymphoid Leukemia

• 80% of childhood leukemias.

• Peak incidence at 4 y.o.

• 90% overall cure rate.

• most common blood cancer

• greater than 40,000/mm³.

dental procedures can be done when platelet count is __

Neutrophils

• Body's first line of defense.

• Absolute Neutrophil Count (ANC) <1,000/mm³

  • Defer treatment because the risk for development of infections and bacteremia increases greatly.

Hematopoietic Stem Cell Transplantation (HSCT)

• Used to replace the marrow of patients with:

  • Hematologic disorders

  • Congenital immunodeficiencies

  • Lipidoses

  • Inborn errors of metabolism

Mucositis

• Most common cause of oral pain in cancer treatments.

• Most common complication in HSCT.

normal Blood component values

4,000-10,000 /mL

normal Platelet Count values

150,000- 450,000/mm3

Primary Herpetic Gingivostomatitis

• Caused by Herpes Simplex Virus type 1 (HSV-1).

• Acquired through direct skin contact or body fluids.

• Children may present with severe local pain.

  • Difficulties with fluid and food intake.

  • At risk for dehydration.

  • is contagious. HSV-1 can be trasmitted through saliva.Avoid sharing drinking glasses or eating utensils . Also avoid sharing toys with other children because most young children put toys in their mouth

Nonspecific symptoms of primary herpetic gingivostomatitis

• Cervical lymphadenopathy

• Malaise

• Irritability

• Upper respiratory tract infection

• Low-grade fever

Oral lesions of primary herpetic gingivostomatitis

• Start as vesicles on the tongue, buccal mucosa, and gingiva.

• Rapidly rupture to become ulcers.

• Infection lasts for 10–14 days.

Management of Primary Herpetic Gingivostomatitis

1. Promote lesion healing, palliation, adequate hydration, and nutrition.

2. Prevent further spread of infection.

3. Cold items such as ice cream, popsicles, and ice chips can soothe affected tissues and help with hydration.

4. Analgesics, topical anesthetics, and coating agents help relieve pain and facilitate food intake.

5. Acyclovir.

Candidiasis / Moniliasis / Thrush

• Fungal infection caused by Candida albicans.

• Occurs in infants 5–7 days after birth.

• Appears as multiple, small, white curd-like patch lesions.

• Lesions may be easily removed from the mucosa, exposing areas of erythema and ulceration, which readily bleed.

• usually considered not to be contagious, one instance where the fungus can be passed back and forth is between infant and mother. An infant may develop thrush and transfer it to the mother’s nipples, which become painful with reddish, cracking skin. Conversely, mothers develop a Candida albicans yeast infection on their nipples while taking antibiotics or steroids and trasnfer the infection to their babies while breastfeeding.

Opportunistic infection caused by overgrowth of Candida species due to:

• Antibiotic and/or corticosteroid use

• Xerostomia

• Diabetes mellitus

• Appliances covering the palate

• Smoking

• Immature or compromised immune system

2 most common forms of Candidiasis in children

• Pseudomembranous candidiasis

• Erythematous candidiasis

Treatment of Candidiasis

• Antifungal agents such as:

  • Nystatin

  • Clotrimazole

treatment of Angular Cheilitis

• Nystatin

• Triamcinolone acetonide cream (Mycolog-II)

  • Applied to the corners of the mouth 3–5 times a day for 5 days

• Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

• Most common genetic disease.

• causes:

  • Decreased chloride absorption

  • Increased sodium absorption

  • pseudomonas aeruginosa

• tx: tobramycin

these are inhaled to prevent infection for Cystic Fibrosis

• Tobramycin

• Colomycin

hallmark of Cystic Fibrosis

Salty skin

Pseudomonas aeruginosa

most common pathogen in Cystic Fibrosis

Inflammatory Bowel Disease (IBD)

• A disorder of intestinal inflammation caused by chronic conditions.

• affects the proximal part of intestine

• Encompasses:

  • Ulcerative colitis

  • Crohn disease

• Diarrhea and abdominal pain are common features of the disorder.

hallmark of Inflammatory Bowel Disease (IBD)

• cobble stoning

Ulcerative colitis

(inflammation of the colon and rectum)

Crohn disease

(inflammation of the large intestine, small intestine)

Oral Manifestations of Crohn Disease

• Aphthous ulcers

• Lip swelling

• Buccal mucosa swelling (cobblestoning)

• Mucogingivitis

• Deep linear ulceration

• Mucosal tags

• The oral manifestations of Crohn disease are usually self-resolving and do not require specific treatment.

• Patients whose oral cavity is symptomatic should use a cinnamon- and benzoate-free diet.

Celiac Disease

• Gluten-related disorder.

• Gluten – main protein in wheat

Oral Manifestations of Celiac Disease

• Low caries experience

• Low levels of cariogenic microflora

• High occurrence of recurrent aphthous stomatitis

• Reduced salivary flow

practice question in sas