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Severe congenital cyclic neutropenia: A case report
Vidyavathi H Patil1, Shivayogi M Hugar1, Girish Balikai2
1Department of Paedodontics and Preventive Dentistry, KLE University, KLE VK Institute of Dental Sciences, Belagavi, Karnataka, India.
International Journal of Applied & Basic Medical Research
|November 19, 2016
Summary
Congenital cyclic neutropenia is a rare genetic disorder causing low neutrophil counts. Early diagnosis in children with recurrent infections and mouth ulcers is crucial for preventing severe complications.
Area of Science:
- Genetics
- Immunology
- Pediatrics
Background:
- Congenital cyclic neutropenia syndrome (CCN) is a rare constitutional genetic disorder.
- It is characterized by recurrent, severe neutropenia (abnormally low neutrophil count).
- Neutrophils are critical white blood cells for fighting infection.
Observation:
- This case report details a child presenting with recurrent oral ulcers and fever.
- The child was later diagnosed with severe congenital cyclic neutropenia.
- Recurrent oral ulcers and poor dental hygiene can be indicators of underlying immunosuppressive conditions.
Findings:
- The study highlights a case of severe congenital neutropenia in a child.
- Diagnosis was confirmed through clinical presentation and likely genetic analysis (implied).
- The condition leads to recurrent infections and significant oral health issues.
Implications:
- Early identification of rare immunosuppressive disorders like CCN in children is vital.
- Prompt diagnosis can prevent severe oral cavity complications.
- Timely intervention reduces the risk of life-threatening neutropenic sepsis and mortality.
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