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Cyclic neutropenia with a novel gene mutation presenting with a necrotizing soft tissue infection and severe sepsis: case report.

Boo YJ, Nam MH, Lee EH, Lee KC - BMC Pediatr (2015)

Bottom Line: The infection progressed rapidly and resulted in septic shock.After applying of negative-pressure wound therapy, her clinical symptoms improved.Both adequate medical treatment and effective control of the source of infection are critically important to reduce morbidity in such complex cases of necrotizing fasciitis as appeared in an immunocompromised pediatric patient.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Surgery, Department of Surgery, Korea University College of Medicine, 73 Inchonro, Sungbuk-gu, Anam-dong, Seoul, 136-705, Korea. drboo@korea.ac.kr.

ABSTRACT

Background: Cyclic neutropenia is a rare disease. We report a 31-month-old girl with congenital cyclic neutropenia with a novel mutation in the ELANE gene who developed an acute necrotizing soft-tissue infection on her left axillary legion.

Case presentation: A 31-month-old girl was admitted to our pediatric emergency room because of a necrotizing soft tissue infection of the left axillary area. The infection progressed rapidly and resulted in septic shock. Despite a medical treatment and surgical debridement, the sepsis was not controlled, and severe inflammation developed. After applying of negative-pressure wound therapy, her clinical symptoms improved. Finally, she was diagnosed with cyclic neutropenia with a novel genetic mutation. One month after admission, she was discharged with a completely recovered wound and no need for skin grafting.

Conclusion: Both adequate medical treatment and effective control of the source of infection are critically important to reduce morbidity in such complex cases of necrotizing fasciitis as appeared in an immunocompromised pediatric patient.

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Related in: MedlinePlus

Computed tomography shows diffuse swelling of the lateral chest wall and axilla 4 days after the initial surgery.
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Fig2: Computed tomography shows diffuse swelling of the lateral chest wall and axilla 4 days after the initial surgery.

Mentions: Granulocyte-colony stimulating factor (G-CSF) was also administered because of the strong suspicion of underlying CN. The neutropenia normalized 2 days after the G-CSF treatment. She underwent daily surgical wound debridement and irrigation to control the source of infection. The sepsis was not controlled, moreover, the inflammation spread to her chest wall and neck area. Computed tomography 5 days after the initial surgery showed diffuse swelling of the left lateral chest wall and axilla, indicating further spread of the inflammation (Figure 2). Five days after the initial treatment, we applied negative-pressure wound therapy (V.A.C., KCI, San Antonio, TX, USA) after surgical debridement (Figure 1C). The negative pressure was gradually increased to 100 mmHg, and the wound dressing was changed every 3 days. Several days later, her clinical symptoms dramatically improved, and she was transferred to the general ward. Granulation tissue grew so rapidly that she did not require mesh grafting or skin grafting to repair the wound (Figure 1D). One month after admission, she was discharged with a completely recovered wound.Figure 2


Cyclic neutropenia with a novel gene mutation presenting with a necrotizing soft tissue infection and severe sepsis: case report.

Boo YJ, Nam MH, Lee EH, Lee KC - BMC Pediatr (2015)

Computed tomography shows diffuse swelling of the lateral chest wall and axilla 4 days after the initial surgery.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4386099&req=5

Fig2: Computed tomography shows diffuse swelling of the lateral chest wall and axilla 4 days after the initial surgery.
Mentions: Granulocyte-colony stimulating factor (G-CSF) was also administered because of the strong suspicion of underlying CN. The neutropenia normalized 2 days after the G-CSF treatment. She underwent daily surgical wound debridement and irrigation to control the source of infection. The sepsis was not controlled, moreover, the inflammation spread to her chest wall and neck area. Computed tomography 5 days after the initial surgery showed diffuse swelling of the left lateral chest wall and axilla, indicating further spread of the inflammation (Figure 2). Five days after the initial treatment, we applied negative-pressure wound therapy (V.A.C., KCI, San Antonio, TX, USA) after surgical debridement (Figure 1C). The negative pressure was gradually increased to 100 mmHg, and the wound dressing was changed every 3 days. Several days later, her clinical symptoms dramatically improved, and she was transferred to the general ward. Granulation tissue grew so rapidly that she did not require mesh grafting or skin grafting to repair the wound (Figure 1D). One month after admission, she was discharged with a completely recovered wound.Figure 2

Bottom Line: The infection progressed rapidly and resulted in septic shock.After applying of negative-pressure wound therapy, her clinical symptoms improved.Both adequate medical treatment and effective control of the source of infection are critically important to reduce morbidity in such complex cases of necrotizing fasciitis as appeared in an immunocompromised pediatric patient.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Surgery, Department of Surgery, Korea University College of Medicine, 73 Inchonro, Sungbuk-gu, Anam-dong, Seoul, 136-705, Korea. drboo@korea.ac.kr.

ABSTRACT

Background: Cyclic neutropenia is a rare disease. We report a 31-month-old girl with congenital cyclic neutropenia with a novel mutation in the ELANE gene who developed an acute necrotizing soft-tissue infection on her left axillary legion.

Case presentation: A 31-month-old girl was admitted to our pediatric emergency room because of a necrotizing soft tissue infection of the left axillary area. The infection progressed rapidly and resulted in septic shock. Despite a medical treatment and surgical debridement, the sepsis was not controlled, and severe inflammation developed. After applying of negative-pressure wound therapy, her clinical symptoms improved. Finally, she was diagnosed with cyclic neutropenia with a novel genetic mutation. One month after admission, she was discharged with a completely recovered wound and no need for skin grafting.

Conclusion: Both adequate medical treatment and effective control of the source of infection are critically important to reduce morbidity in such complex cases of necrotizing fasciitis as appeared in an immunocompromised pediatric patient.

Show MeSH
Related in: MedlinePlus