Hemifacial microsomia is a feature of:

 # Hemifacial microsomia is a feature of:

A. Goldenhar syndrome

B. Crouzon syndrome

C. Treacher Collins syndrome

D. Mandibulofacial dysostosis

The correct answer is A. Goldenhar syndrome.

Hemifacial microsomia is a feature of Goldenhar syndrome. Synonyms for hemifacial microsomia include hemifacial hypoplasia, craniofacial microsomia, lateral facial dysplasia, Goldenhar syndrome, and oculoauriculovertebral dysplasia (OAV) spectrum. 

B is incorrect; Hemifacial microsomia is not a feature of Crouzon syndrome. Patients with Crouzon syndrome characteristically have brachycephaly (short skull front to back), hypertelorism (increased distance between eyes), and orbital proptosis (protruding eyes). 

C is incorrect; Hemifacial microsomia is not a feature of Treacher Collins syndrome. The most common clinical findings in Treacher Collins syndrome are relative underdevelopment or absence of the zygomatic bones, resulting in a small narrow face; a downward inclination of the palpebral fissures; underdevelopment of the mandible, resulting in a down-turned, wide mouth; malformation of the external ears; absence of the external auditory canal; and occasional facial clefts. 

D is incorrect, Mandibulofacial dysostosis is a synonym of Treacher Collins syndrome.

The gene aberrant in dentinogenesis imperfecta is:

 # The gene aberrant in dentinogenesis imperfecta is:

A. Chromosome 4

B. Chromosome 13

C. Chromosome 21

D. Chromosome 24

The correct answer is A. Chromosome 4.

Aberration in chromosome 4 (4q21.3) is associated with Dentinogenesis imperfecta. It encodes dentin specific sialophosphoprotein (DSPP) gene. Deficiency of sialoprotein is also suggested as a causative factor for DI. They are generally tulip shaped. 

Chromosome number              Disease 

1                                               Vanderwoude syndrome

4                                               Dentinogenesis Imperfecta 

4                                               Cherubism

15                                             Marfan's syndrome

19                                             Osteogenesis imperfecta

Deficiency of which vitamin causes glossitis, dementia, rough keratotic areas on skin and gastrointestinal symptoms?

 # Deficiency of which vitamin causes glossitis, dementia, rough keratotic areas on skin and gastrointestinal symptoms?

A. Riboflavin

B. Pyridoxine

C. Niacin

D. Pantothenic acid

The correct answer is C. Niacin.

Pellagra is a disease caused by a lack of the vitamin niacin (vitamin B3). Symptoms include inflamed skin, diarrhea, dementia, and sores in the mouth. Areas of the skin exposed to either sunlight or friction are typically affected first. Over time affected skin may become darker, stiffen, peel, or bleed.

There are two main types of pellagra, primary and secondary. Primary pellagra is due to a diet that does not contain enough niacin and tryptophan. Secondary pellagra is due to a poor ability to use the niacin within the diet. This can occur as a result of alcoholism, long-term diarrhea, carcinoid syndrome, Hartnup disease, and a number of medications such as isoniazid. Diagnosis is typically based on symptoms and may be assisted by urine testing. 

FIGLU excretion test is used to estimate deficiency of:

 # FIGLU excretion test is used to estimate deficiency of:

A. Vitamin K

B. Vitamin B12

C. Vitamin Folic acid

D. Niacin

The correct answer is C. Vitamin Folic acid.

Folic acid is associated with the metabolism of histidine. Formiminoglutamate (FIGLU) formed in histidine metabolism accumulates and is excreted in urine. Histidine load test utilizing the excretion of FIGLU in urine is used to assess folic acid deficiency. 

Which of the following vitamins is destroyed by heat?

 # Which of the following vitamins is destroyed by heat?

A. Vitamin A

B. Vitamin D

C. Biotin

D. Thiamin

The correct answer is C. Biotin.

Biotin is present in many foods, including liver, grains, and eggs. It is also synthesized by intestinal bacteria. The conversion to a coenzyme simply requires that it be covalently linked to the appropriate enzymes. Biotin acts as a carrier of "activated carboxyl" groups for three key enzymes that catalyze carboxylation reactions. The enzymes and the pathways they participate in are: Pyruvate carboxylase (gluconeogenesis), acetyl-CoA carboxylase (fatty acid synthesis), and propionyl-CoA carboxylase (branched chain amino acid catabolism). Biotin deficiency is rare. Excessive consumption of raw egg impairs biotin absorption due to the presence of a biotin-binding protein, avidin, in egg whites. Antibiotics that alter the intestinal flora can also lead to biotin deficiency. Symptoms of deficiency include alopecia, skin and bowel inflammation, and muscle pain. 

AIIMS MAY 2011 MDS ENTRANCE EXAM PAST QUESTIONS

# All of the cranial nerve have parasympathetic outflow EXCEPT:
A. 5th Nerve
B. 7th Nerve
C. 3rd Nerve
D. 9th Nerve

# The future TMJ shows development at:
A. 12 week
B. 10 week
C. 18 week
D. 16 week

# Area that lies immediately lateral to the anterior perforating substance is:
A. Orbital gyrus
B. Uncus
C. Optic chiasma
D. Limen insulae

 

# Which of the following organ is located posterior to pancreas?
A. Kidney
B. Stomach
C. Colon
D. Duodenum

# Intrinsic factor of Castle is secreted by which of the following cells in Gastric glands?
A. Chief cells
B. Parietal cells
C. Mucus cells
D. B cells

# A patient was diagnosed with isolated increase in LDL. His father and brother had the same disease with increased cholesterol. The likely diagnosis is:
A. LDL receptor mutation
B. Familial lipoprotein lipase deficiency type I
C. Familial type 3 hyperlipoproteinemia
D. Genetic defect in LDL receptors

During a Le fort I maxillary osteotomy, the tooth most likely to be injured by a low osteotomy line is the:

 # During a Le fort I maxillary osteotomy, the tooth most likely to be injured by a low osteotomy line is the:

A. Central incisor

B. Cuspid

C. First bicuspid

D. Lateral incisor

The correct answer is B. Cuspid.

The cuspids, or canine teeth, have the longest roots in both the maxilla and mandible. The average length of a cuspid tooth from the tip of the root to the tip of the crown is 27 mm. It is vital to know the length and position of the dental roots to avoid injuring the apices of the cuspid teeth during Le Fort I osteotomy and placement of internal fixation (interosseous miniplates or microplates and screws). The dentition may also be injured during stabilization of maxillary or mandibular fractures. The bicuspid, central and lateral incisor, and molar teeth all have roots that are shorter than the roots of the cuspid teeth; the average length of a molar tooth from the tip of the root to the tip of the crown, for example, is 24 mm. Consequently, these teeth are much less likely to be injured during LeFort I osteotomy.