Metabolic Diseases. Nutritional Deficiencies Endocrinopathies In-Born Errors of Metabolism

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1 Metabolic Diseases

2 Metabolic Diseases Nutritional Deficiencies Endocrinopathies In-Born Errors of Metabolism

3 Endrocrinopathies Anterior Pituitary Posterior Pituitary Thyroid Parathyroid Adrenal Pancreatic Islets

4 Anterior Pituitary Growth hormone Hyperpituitarism Acidophilic Adenoma Gigantism Acromegaly Deficiency of Growth Hormone Dwarfism, diminutive stature

5 Hypopituitarism: Posterior Pituitary Diabetes Insipidis Infiltration of posterior pituitary by pathologic processes Langerhans Cell Histiocytosis, chronic disseminated form Exophthalmos Diabetes insipidis Intraosseous lesions (including jaws) Loss of Anti-Diuretic Hormone secretion Polyuria, Nocturia

6 Langerhans Cell Histiocytosis Teeth floating in space

7 Hyperthyroidism

8 Hyperparathyroidism Primary form Parathyroid Adenoma or Hyperplasia Secondary forms) Renal osteodystrophy Hormone secreting small cell carcinomas (lung) Elevated levels of parathormone stimulate osteoclastic resorption Elevated serum calcium, Low serum phosphate, normal alkaline phosphatase Osteolytic lesions (brown tumors): Giant cell tumors in many sites including jaws Stones, Bones, Abdominal Moans Urolithiasis secondary to hypercalciuria Giant cell lesions of bone Painful abdomen due to stones

9 Hyperparathyroidism

10 Brown Tumor Hyperparathyroidism

11 Nutritional Diseases Protein Deficiency(Kwashiorkor) Caloric Deficiency(Marasmus) Fat Soluble Vitamins Vitamin A Vitamin D Vitamin E Vitamin K Water Soluble Vitamins Riboflavin Thiamine (Beri-Beri) Nicotinamide (Pellagra) Vitamin B12 Folic Acid

12 Ascorbic Acid Deficiency (Scurvy) Defective collagen synthesis Vessel wall fragility, petechiae Delayed wound healing Excessive cartilage with defective osteoid formation (scorbutic lattice rib cage) Periodontal Breakdown

13 Scurvy (avitaminosis C) Chondroid nodules at costochondral Junction > Scorbutic Lattice Accelerated Periodontitis

14 Pellagra (Nicotinamide) Dermatitis, Diarrhea, Dementia (3 Ds)

15 Pernicious Anemia(B12) bald tongue

16 In-Born Errors of Metabolism (enzyme deficiency diseases) Hematopoietic Thalassemia Sickle Cell Anemia Hemophilia (Pseudotumor) Lipid Reticuloendothelioses Vitamin D Refractory Rickets Hypophosphatasemia

17 Thalassemia Major

18 Sickle Cell Anemia Hair on end Sickled erythrocytes Hemoglobin electrophoresis HGB-S/A HGB-S/S

19 Hemophilia Two major forms and other rare forms hemophila A factor VIII (X linked hemophila B factor IX recessive) Due to mutated clotting factors, a hemorrhagic diathesis occurs Bleeding tendencies are evident in childhood Intraosseous hemorrhage leads to pseudotumors that may occur in the jaws.

20 Vitamin D Dependent Hereditary Rickets Inherited as an autosomal dominant trait with inability to form 1α,25 dihydroxy-vit D3 Type I mutation in 1α hydroxylase Type II mutation in vitamin D receptor complex Coronal fissures in which bacteria can gain access to the pulp in the absence of carious lesions Periapical radiolucencies representing spread of endodontic infection Generalized features in common with vitamin D deficient rickets (without response to vitamin therapy)

21 Coronal Fissures, Apical Infections Vitamin D Refractory Rickets

22 Hypophosphatasemia Defective ossification Genetic defect in alkaline phosphatase, an enzyme need for mineralization Dental manifestation: Cemental Agenesis Normal Cemental Agenesis

23 Osteopetrosis (Marble Bone Disease) Albers-Schonberg Disease A genotypically diverse group of diseases with similar phenotype Defective osteoclastic resorption resulting in dense, generalized ossification, autosomal dominant and autosomal recessive forms Muations in Genes for carbonic anhydrase II, vacuolar protein pump of the brush border, cathepsin K (pycnodysostosis) Teeth are obscured by bone density as seen radiographically, impaired eruption Malignant forms with pancytopenia (myelophthisic anemia)

24 Osteopetrosis (Marble Bone)