Progeria Part 1

Progeria (also known as “Hutchinson–Gilford Progeria Syndrome“, “Hutchinson–Gilford syndrome“, and “Progeria syndrome“) is an extremely rare genetic condition wherein symptoms resembling aspects of aging are manifested at an early age. The word progeria comes from the Greek words “pro” (πρό), meaning “before”, and “géras” (γῆρας), meaning “old age”. The disorder has very low incidences and occurs in an estimated 1 per 8 million live births. Those born with progeria typically live to their mid teens and early twenties. It is a genetic condition that occurs as a new mutation (de novo), and is rarely inherited. Although the term progeria applies strictly speaking to all diseases characterized by premature aging symptoms, and is often used as such, it is often applied specifically in reference to Hutchinson-Gilford Progeria Syndrome (HGPS). Continue reading →

Hurler’s Syndrome

Hurler syndrome, also known as mucopolysaccharidosis type I (MPS I), Hurler’s disease, also gargoylism, is a genetic disorder that results in the buildup of glycosaminoglycans (formerly known as mucopolysaccharides) due to a deficiency of alpha-L iduronidase, an enzyme responsible for the degradation of mucopolysaccharides in lysosomes. Without this enzyme, a buildup of heparan sulfate and dermatan sulfate occurs in the body. Symptoms appear during childhood and early death can occur due to organ damage. Continue reading →

Chediak-Higashi syndrome

Chédiak–Higashi syndrome is a rare autosomal recessive disorder that arises from a microtubule polymerization defect which leads to a decrease in phagocytosis. The decrease in phagocytosis results in recurrent pyogenic infections, partial albinism and peripheral neuropathy. It occurs in humans, cattle, white tigers, blue Persian cats, Australian blue rats, mice, mink, foxes, and the only known captive albino orca. Continue reading →

Apert syndrome Part 2

Causes

Acrocephalosyndactyly may be an autosomal dominant disorder. Males and females are affected equally; however research is yet to determine an exact cause. Nonetheless, almost all cases are sporadic, signifying fresh mutations or environmental insult to the genome. The offspring of a parent with Apert syndrome has a 50% chance of inheriting the condition. In 1995, A.O.M. Wilkie published a paper showing evidence that acrocephalosyndactyly is caused by a defect on the fibroblast growth factor receptor 2 gene, on chromosome 10. Continue reading →

Pain in the Face and Mouth Part 4

Continued from Part 3

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Chronic post-traumatic headache

Most persons who have had head injuries have local pain or tenderness at the site of impact for a few hours or even for a few days, after which many become symptom-free. However up to one half of all persons who injure their heads sufficiently to warrant hospitalization develop chronic post-traumatic headaches. Continue reading →

Apert syndrome Part 1

Apert syndrome is a form of acrocephalosyndactyly, a congenital disorder characterized by malformations of the skull, face, hands and feet. It is classified as a branchial arch syndrome, affecting the first branchial (or pharyngeal) arch, the precursor of the maxilla and mandible. Disturbances in the development of the branchial arches in fetal development create lasting and widespread effects. Continue reading →

Pain in the Face and Mouth Part 3

Continued from Part 2

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Temporomandibuar joint

Pain from the temporomandibular joint may result from dysfunction, trauma, acute or chronic inflammation, or primary or secondary cancerous tumors. Examination may reveal the masticatory (chewing) muscles tender to palpation or occasionally the joint swollen and warm to touch or tender to palpation via the external auditory meatus. Pain from the temporomandibular joint: Continue reading →

Dentinogenesis

Odontoblasts

Dentin-forming cells, odontoblasts, which originate from the ectomesenchyme, form a single layer of cells between the dentin and pulp. The cell body is located on the pulpal wall of dentin and the cellular process extends into the dentinal tubule within the mineralized dentin. The cell bodies are from 3 to 5 m wide and 20 to 40 m long depending on the age of the tooth. The odontoblastic process fills the lumen of the dentin tubule and it is composed of a main trunk, with a diameter of 0.5 to 1 m, and lateral branches. Contrary to the cell body, cell organelles (Golgi apparatus, rough endoblastic reticulum or mitochondria) usually do not appear in the odontoblastic process; however, microtubules, filaments and coated vesicles are present. Odontoblasts are connected to each other with interodontoblastic collagen, the so-called von Korff fibers. Frequent bundles of collagen fibrils enter the odontoblast layer from predentin and are present between odontoblast cell bodies. Ultimately they pass through the odontoblast layer into pulp. Histologically, secretory odontoblasts are columnar in shape. A large number of cytoplasmic organelles are identifiable in young odontoblasts, whereas, aged odontoblasts lose their columnar shape and contain a small number of Golgi apparatus and a small-sized rough endoblasmic reticulum. Continue reading →

Leukemia and oral health

What is leukemia?

Leukemia is a malignant cancer of the blood in which one’s bone marrow produces white blood cells in excess in association with gene mutations.

leukemia: white blood cells (dark purple) produced in excess

Causes of leukemia

Genetic predisposition (family history, people with Down syndrome have higher tendency to get leukemia)

Artificial ionizing radiation

Chemicals (eg benzene and other petrochemicals)

Viruses, e.g. HIV (Human Immunodeficiency virus  or HTLV-1 (human T-lymphotropic virus) Continue reading →

Pain in the Face and Mouth Part 2

Continued from Part 1

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Acute periapical periodontitis

Pain associated with acute perapical periodontitis:

  • Is spontaneous in onset

  • Is moderate to severe in intensity

  • Persists for long periods of time (hours)

  • Is worsen by biting on tooth and in more advanced cases, even by closing the mouth and bringing the affected tooth gently into contact with the opposing teeth. In these cases, the tooth feels ‘high’ (extruded) and is sensitive to touch Continue reading →