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Case Report

Indian Pediatr 2012;49: 481-482

Septic Shock Due to Tuberculosis in Down Syndrome


Baljeet Maini, Vipul K Gupta and Sunny Narang

From the Department of Pediatrics, MMIMSR, Mullana, Ambala, Haryana, India.

Correspondence to: Dr Baljeet Maini, House no 465-466, Housing Board Colony, Sector 4,
Karnal, Haryana 132 001, India.
Email: [email protected]

Received: June 16, 2011;
Initial review: June 30, 2011;
Accepted: September 5, 2011.



Both Immune dysfunction and deficiency, are known in Down syndrome. Tuberculosis commonly presents as insidious illness and septicemic shock is its rare presentation, mostly in immunocompromized patients. We report a 16 year old boy with Down syndrome presenting with septicemic shock due to tuberculosis.

Key words: Immune deficiency, Trisomy 21, Tuberculosis.


Tuberculosis commonly presents as insidious illness and septicemic shock is its rare presentation, mostly reported in HIV patients[1-3]. We report here a case of septicemic shock due to tuberculosis in a child with Down syndrome.

Case report

A 16 year old boy, known case of Down syndrome, was brought with history of fever and cough for 2 weeks, and cold extremities and breathing difficulty for last 12 hours. There was no history of diarrhea, vomiting or blood loss. Child was fully vaccinated for age and had a BCG scar. Capillary refill time was prolonged, heart rate was 160/min and blood pressure was 80/60 mm Hg (below 5th centile). Except for bilateral crepitations, systemic examination was normal. Child was managed on lines of septicemic shock. Normal saline bolus, antibiotics, vasopressor support, oxygen and packed red cell transfusion were given.

Hemogram revealed hemoglobin 8 g/dl, total leucocytes count (TLC) 8600 cells/cubic mm, platelet count-2,10,000/cubic mm. X-ray chest suggested patchy opacities with multiple cavitatory lesions in both lungs. Sonography of abdomen was normal. Echocardiogram revealed normal structural and functional cardiac condition. Blood culture was sterile. Widal test was negative. Sputum microscopy examination revealed acid fast bacilli (AFB) (Mycobacterium tuberculosis). Child was started on 4 drug antitubercular treatment (ATT). Hemodynamic improvement started on day 3 and vasopressor treatment was gradually withdrawn by day 6 of admission. Oxygen support was gradually withdrawn and weaned off on day 7 of admission. Category I Direct Observed Treatment Short course (DOTS) [4] was started and patient was discharged after 10 days of admission. Patient is on regular follow up and has clinically improved considerably. Follow up chest X-ray after 5 months revealed fibrocystic changes in the lungs suggestive of healing tuberculosis. Repeat sputum examinations (as per DOTS protocol) were negative for AFB.

Discussion

There is an increased incidence of respiratory infections in children with Down syndrome. Although every arm of immune system shows evidence of dysfunction in these patients, particularly T cells (CD4+ and suppressor T cells) and NK cells show marked derangement of number and activity [5-9]. No data has so far shown difference in incidence of tuberculosis in this syndrome and general population [10].

Septicemic shock is commonly caused by pyogenic organisms. It is reported to be caused by tuberculosis in immunocompromized patients only [1-3]. We thus suspected some immunodeficiency or dysfunction in our case. This case was HIV negative and had normal total leukocyte count.

In the absence of adequate facilities, we were not able to exactly point out the immune defect in our patient. The possibility of immune dysfunction in Down’s syndrome as a cause of shock in mycobacterial infection can be explored further, in view of similar presentation seen in cases of acquired immunodeficiency [2,3].

Contributors: BM, SN and VKG were involved in clinical management of the case, literature search and writing the paper. All authors approved the final manuscript.

Funding: None;

Competing interests: None stated.

References

1. Gachot B, Wolff M, Clair B, Régnier B. Severe tuberculosis in patients with human immunodeficiency virus infection.Intensive Care Med.1990;16:491-3.

2. Vadillo M, Corbella X, Carratala J. AIDS presenting as septic shock caused by Mycobacterium tuberculosis. Scand J Infect Dis.1994;26:105-6.

3. Lim KH, Chong KL. Multiple organ failure and septic shock in disseminated tuberculosis. Singapore Med J. 1999;40:176-8.

4. Revised National Tuberculosis Control Program. DOTS plus guidelines 2010. Available from URL: http://www.tbcindia.org/pdfs/DOTS_Plus_ Guidelines_Jan2010.pdf. Accessed on June 15, 2011.

5. Kusters MAA, Verstegen RHJ, Gemen EFA, De Vries E. Intrinsic defect of the immune system in children with Down syndrome: A review. Clin Exp Immunol. 2009;156:189-193.

6. Ugazio AG, Maccario R, Notarangelo LD, Burgio GR. Immunology of Down syndrome: A review. Am J Med Genet (Suppl.). 1990;7:204-12.

7. Cuadrado E, Barrena MJ. Immune dysfunction in Down’s syndrome: primary immune deficiency or early senescence of the immune system? Clin Immunol Immunopathol. 1996;78:209-14.

8. Nespoli L, Burgio GR, Ugazio AG, Maccario R. Immunological features of Down’s syndrome: A review. J Intellect Disab Res.1993;37(Pt 6):543-51.

9. Meguid NA, Basheer HH, Ismail EA, Dardir AA, Sharaf TM. Immune dysfunction in Egyptian children with Down syndrome. The Egyptian Journal of Medical Human Genetics.2004;5:43-54.

10. Verma SK, Sodhi R. Down’s syndrome and cardiac tamponade with pulmonary tuberculosis in adults. Indian J Hum Genet. 2009;15:72-4.
 

 

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