Ambras Syndrome: First Reported Case in Bangladesh and its Oral Rehabilitation.

People with rare hypertrichosis syndromes became crowd-drawing money-making phenomena in many 19th century sideshow acts. These individuals have been referred to as dog-men, hair-men, and werewolves. In 1993, Baumister et al. described congenital hypertrichosis lanuginose or Ambras syndrome: a distinct form of congenital hypertrichosis characterized by excessive hair growth over the body and face associated with facial and occasional dental anomalies. Much is not known about this syndrome since fewer than 50 cases have been documented worldwide. In this case report, a nine year old girl presented with excessive hair growth throughout her body that was denser along her midline. Furthermore, her face displayed the typical dysmorphic features characteristic of Ambras syndrome: a round tip nose, thickened nasal cartilage, antiverted nares, prominent philtrum with deep groove, and a trapezoid mouth. Oral examination revealed normal oral mucosa with completely missing and unerupted decidious and permanent teeth. Panoramic radiographs confirmed unerupted deciduous teeth. Previous case reports have mentioned the presence of occasional dental anomalies such as retarded first and second dentition and absence of some teeth. However, this is the first reported case of Ambras syndrome presenting with complete anodontia. Prior cytogenetic studies performed on persons with Ambras syndrome have implicated a balanced pericentric inversion of chromosome 8. However, it is likely that dental anomalies are likely a result of a different genetic rearrangement. Further studies are needed to explore the cause of this rare phenotype of Ambras syndrome with complete unerupted dentition.

Prevalence of diabetes among hypothyroid subjects.

Thyroid dysfunction affects a significant portion of the general population. Most studies have found a higher prevalence of hypothyroidism in women, increasing with age. Prevalence of hyperthyroidism has been found to range 0.5-3.0%. Type 2 diabetes is a major public health problem affecting approximately 4.8 percent of the Bangladeshi population. The study was designed to see the prevalence of type 2 diabetes in hypothyroid patients of any etiology. Four hundred forty two consecutive cases of diagnosed hypothyroidism were recruited in the study. All diagnosed hypothyroid subjects (sub-clinical or overt) reported in one year not known to be diabetic previously, underwent standard OGTT (FPG and 2-hour post 75 gram glucose) before initiation of thyroid hormone replacement. Newly detected diabetes among the subjects diagnosed as hypothyroidism is significantly higher 4.8 vs. 7.01% (p<0.01) and the prevalence of pre-diabetic state Impaired Glucose Tolerance (IGT) is also higher (11% vs. 12.6%) among hypothyroid subjects. Pure IFG (FPG ≥6.1 mmol/l but <7.0 mmol/l, and 2-PG <7.8 mmol/l) was found in 5.2% according to WHO criteria and the prevalence is higher (6.8%) with ADA criteria (considering normal FBG <5.6mmol/l). Diabetes was found in 7.01% among hypothyroid subjects which is higher than the existing known prevalence (4.8%). IGT was also found higher among hypothyroid subjects than population prevalence (8.6%). Further larger sample study is essential to establish the findings.