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Ashok, L.
- BIND Syndrome - a Life Threatening Sequel to Kernicterus
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Authors
Affiliations
1 Department of Oral Medicine and Radiology, Bapuji Dental College and Hospital, Davangere, Karnataka, IN
1 Department of Oral Medicine and Radiology, Bapuji Dental College and Hospital, Davangere, Karnataka, IN
Source
Indian Journal of Public Health Research & Development, Vol 3, No 3 (2012), Pagination: 154-156Abstract
Bilirubin Induced Neurological Dysfunction Syndrome (BIND) Syndrome is a condition caused by bilirubin toxicity to the basal ganglia, hippocampus and various brainstem nuclei. In the acute phase, severely jaundiced infants become lethargic, hypotonic and suck poorly. If the hyperbilirubinemia is not treated, the infant becomes hypertonic and may develop a fever and a high-pitched cry.[1] The hypertonia is manifested by backward arching of the neck (retrocollis) and trunk (opisthotonus). Surviving infants usually develop a severe form of choreoathetoid cerebral palsy, hearing loss, dental dysplasia, paralysis of upward gaze and, less often, intellectual and other handicaps.[2] Hereby presenting a rare case report of a 9 year old female patient having BIND Syndrome with typical dental manifestations.Keywords
BIND Syndrome, Kernicterus, Hyperbilirubinemia, Dental Dysplasia, Cerebral Palsy
References
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- Johnson L, Brown AK, Bhutani VK. BIND: A clinical score for bilirubin induced neurologic dysfunction in newborns. Pediatr Suppl 1999; 104: 746-749.
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- AAP Subcommittee on Neonatal Hyperbilirubinemia. Neonatal jaundice and kernicterus. Pediatrics 2001; 108: 763-765.
- Aarskog Syndrome - a Rare Case Report
Abstract Views :220 |
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Authors
Affiliations
1 Department of Oral Medicine and Diagnostic Radiology, Bapuji Dental College and Hospital, IN
2 Department of Pediatries, Chigateri General Hospital, Davangere, Karnataka, IN
1 Department of Oral Medicine and Diagnostic Radiology, Bapuji Dental College and Hospital, IN
2 Department of Pediatries, Chigateri General Hospital, Davangere, Karnataka, IN
Source
Indian Journal of Public Health Research & Development, Vol 3, No 3 (2012), Pagination: 225-229Abstract
Aarskog syndrome is a rare X-linked inherited disorder characterized by short stature, multiple facial, dental, digital and urogenital abnormalities. Here is one such male patient aged 20 years who was short statured and had dysmorphic facies with lack of eruption of multiple permanent teeth. He also had shawl scrotum with low IQ. The clinical appearance was coinciding with most of the features suggested in Aarskog syndrome.References
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- Jerome L, Gorski , Lourdes Estrada, Changzhi Hu, Zhou Liu. Skeletal-specific expression of Fgd1 during bone formation and skeletal defects in faciogenital dysplasia (FGDY; Aarskog syndrome). Developmental Dynamics 2000; 218(4): 573 - 586.
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- Agnar Halse, Kjell Bjorvatn, Dagfinn Aarskog. Dental findings in patients with aarskog syndrome. European Journal of Oral Sciences 2007; 87(4): 253-259.
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- Porteous MEM et al: The gene for Aarskog syndrome is located between DXS255 and DXS566 (Xp11.2-Xq13). Genomics 1992; 14: 298-301.
- Angelman Syndrome - a Rare Case Report
Abstract Views :227 |
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Authors
Affiliations
1 Department of Oral Medicine and Diagnostic Radiology, Bapuji Dental College and Hospital, Davangere-577004, Karnataka,, IN
2 Department of Oral Medicine and Diagnostic Radiology, Bapuji Dental College and Hospital, Davangere 577004, Karnataka, IN
3 Department of Oral Medicine and Diagnostic Radiology, Bapuji Dental College and Hospital, Davangere 577004, Karnataka, IN
4 Department of Oral Medicine and Diagnostic Radiology, Bapuji Dental College and Hospital, Davangere 577004
5 Department of Pediatrics JJM medical college and Hospital Davangere 577004 Karnataka, IN
1 Department of Oral Medicine and Diagnostic Radiology, Bapuji Dental College and Hospital, Davangere-577004, Karnataka,, IN
2 Department of Oral Medicine and Diagnostic Radiology, Bapuji Dental College and Hospital, Davangere 577004, Karnataka, IN
3 Department of Oral Medicine and Diagnostic Radiology, Bapuji Dental College and Hospital, Davangere 577004, Karnataka, IN
4 Department of Oral Medicine and Diagnostic Radiology, Bapuji Dental College and Hospital, Davangere 577004
5 Department of Pediatrics JJM medical college and Hospital Davangere 577004 Karnataka, IN
Source
Indian Journal of Public Health Research & Development, Vol 2, No 1 (2011), Pagination: 78-81Abstract
Angelman Syndrome (AS) was first described in 1965 as "puppet children "characterized by severe neurodevelopmental disability, inability to speak, abnormal motor movement, easily provoked laughter, and epilepsy. Particular mechanisms identified as leading to Angelman Syndrome include deletions of part of the maternally inherited copy of chromosome15.
Here with we report a case of 14 year old girl with Angelman syndrome who presented to the department with complain of painful teeth.
Keywords
Angelman Syndrome, Chromosome 15 Deletions, Mental Retardation, Behavioral AbnormalitiesReferences
- Buggenhout GV, Fryns JP. Angelman Syndrome (AS MIM 105830). European Journal of Human Genetics 2009; 1-7
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