CASE REPORT |
https://doi.org/10.5005/jp-journals-10081-1392 |
A Case Report on Pneumococcal Sepsis with Incomplete Kawasaki Disease
1–4Department of Paediatrics, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
Corresponding Author: Padmasani V Ramanan, Department of Paediatrics, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India, Phone: +91 9445140200, e-mail: padmasani2001@yahoo.com
Received on: 03 May 2023; Accepted on: 27 May 2023; Published on: 29 September 2023
ABSTRACT
Background: Kawasaki disease (KD) is a multisystem vasculitis which occurs due to various infectious triggers causing an abnormal immunological response. There are very few case reports on KD with pneumococcal infection. We report a case of pneumococcal sepsis associated with KD.
Case description: A 11-month-old girl was referred to our hospital with a history of fever and cough for 10 days and one episode of seizure on day 1 of fever. She had not been immunized with the pneumococcal conjugate vaccine (PCV). On examination, she was irritable and tachypneic and had crepitations on the left infrascapular and infra-axillary regions on auscultation. A diagnosis of pneumonia was made. Chest X-ray revealed left lobar consolidation. Blood culture showed Streptococcus pneumoniae 19A growth. In view of the history of seizures and the child’s irritability, an magnetic resonance imaging (MRI) brain with contrast was done to rule out intracranial infection and was found to be normal. In view of persistent fever, no response to antibiotics, marked irritability, and investigations showing leukocytosis, anemia, thrombocytosis, elevated inflammatory markers, incomplete KD was suspected. Echocardiogram (ECHO) showed left main coronary artery dilatation.
She was treated with intravenous immunoglobulin (Ig) and aspirin. The fever resolved within 24 hours, and the child became playful. She was discharged on oral amoxicillin and aspirin.
Clinical significance: We emphasize the need for pneumococcal vaccination (PCV13) for all children to prevent infection and infection-triggered complications.
How to cite this article: Menon K, Narasimhan S, Natarajan S, et al. A Case Report on Pneumococcal Sepsis with Incomplete Kawasaki Disease. Pediatr Inf Dis 2023;5(3):93–99.
Source of support: Nil
Conflict of interest: None
Patient consent statement: The author(s) have obtained written informed consent from the patient’s parents/legal guardians for publication of the case report details and related images.
Keywords: Immunization, Incomplete Kawasaki disease, Pneumococcal infection
INTRODUCTION
Kawasaki disease (KD) is an acute multisystem vasculitis commonly affecting children below five years with a peak incidence in late infancy (9–11 months of age) with a male predominance (21.0/100,000 vs 15/100,000 in females). KD can cause complications like coronary artery aneurysms and thromboembolic occlusions; hence early diagnosis and management are essential.1,2 The risk of coronary artery aneurysms is present in about 25% of all cases presenting with KD.3
Diagnostic criteria include mucosal changes, nonexudative conjunctivitis, polymorphous rash, extremity changes and nonsuppurative cervical lymphadenopathy.2
Classic KD is diagnosed if the patient has ≥5 days of fever as well as more than equal to four of these five principal clinical features. Incomplete KD is the term used for patients with less than four positive symptoms along with fever and abnormal lab values, while atypical KD refers to patients with KD who present with unusual symptoms like renal impairment.1,2
Pneumococcal infections are also most prevalent in children below 5 years of age, with the spectrum of disease ranging from an asymptomatic nasopharyngeal carrier to invasive pneumococcal diseases like sepsis, bacteremia, pneumonia, and meningitis. KD is being increasingly reported to be triggered by infection, and the infections reported include Staphylococcus aureus, Streptococcus pyogenes, Mycoplasma pneumoniae, Cytomegalovirus, adenovirus, rhinovirus, enterovirus, and bocavirus.1
We hereby report a case with incomplete KD and pneumococcal infection.
CASE DESCRIPTION
An 11-month-old girl child, second born out of a nonconsanguineous marriage, from Dharmapuri, was referred to our hospital with a history of fever and cough for 10 days and one episode of seizure on day 1 of fever. She had not taken any PCV. She had been treated in another hospital with intravenous antibiotics (piperacillin/tazobactam and amikacin for 4 days), but the fever was persistent, and her total leucocyte counts (TLCs) and platelet counts were rising.
On admission, she was irritable, tachypneic, and had tachycardia and crepitations in the left infrascapular and infra-axillary regions on auscultation. A clinical diagnosis of pneumonia with sepsis was made. Chest X-ray revealed left lobar consolidation (Fig. 1). Blood and urine cultures were sent on the day of admission, and the child was started on intravenous antibiotics. Investigations revealed a TLC (42150 cells/cumm) with a differential count of neutrophils 28%, lymphocytes 69%, and monocytes 3%, with nil eosinophils and basophils, microcytic hypochromic anemia (hemoglobin 8.9 g/dL), and thrombocytosis (12.01 lakhs/cumm), erythrocyte sedimentation rate (ESR) of 93 mm/first hour), serum ferritin 230 ng/mL, C-reactive protein (CRP) of 0.88 mg/dL, and hypertriglyceridemia with a serum triglyceride of 434 mg/dL.
Fig. 1: Chest X-ray revealed left lobar consolidation
In view of the history of seizures and the child’s irritability, an MRI brain with contrast was done to rule out intracranial infection and was found to be normal. Parents refused consent for lumbar puncture.
Management and Outcome
Since the fever had not responded to antibiotics given in the referring hospital, complications like metastatic infections, lung abscesses, and empyema were first ruled out clinically and radiologically. The dose and duration of previous antibiotics received were rechecked and found to be appropriate. In view of the marked irritability and lab reports showing leukocytosis, anemia, thrombocytosis, and elevated ESR, incomplete KD (fever being the only criteria present) was suspected. Two-dimensional (2D) ECHO was done on the same day (day 10 of fever), which revealed left main coronary artery dilatation (>3 Z-score) (Fig. 2). Though this is not synonymous with KD in view of the overall clinical picture and lab markers, she was treated with intravenous Ig and aspirin. The fever resolved within 24 hours, and the child became playful. The serial lab parameters are described in Table 1. Blood cultures showed growth of Streptococcus pneumoniae susceptible to cephalosporins (Fig. 3). Serotyping revealed 19A pneumococcal serotype. She was discharged on oral amoxicillin and aspirin with advice to repeat ECHO after 2 weeks, follow-up with the cardiologist, and take pneumococcal vaccination and iron supplements. Repeat ECHO after 6 weeks showed resolution of the coronary dilatation that had been previously noted (Fig. 4).
| Biochemistry | |||||
|---|---|---|---|---|---|
| Test | 5.11.22 | 6.11.22 | 7.11.22 | 8.11.22 | Outside investigations |
| Triglyceride | 434 | ||||
| Ferritin | 230 | ||||
| Lactate dehydrogenase | 872 | ||||
| Potassium | 6.1 | 5.1 | 5.6 | 5.4 | |
| Blood urea nitrogen | 20 | 10 | 29 | ||
| Creatinine | 0.3 | 0.2 | 0.5 | ||
| Sodium | 133 | 131 | |||
| Chloride | 97 | 100 | |||
| Bicarbonate | 21 | 22 | |||
| Serum glutamic-oxaloacetic transaminase | 64 | 38 | |||
| Serum glutamic pyruvic transaminase | 17 | 13 | |||
| Alkaline phosphatase | 170 | 182 | |||
| Total protein | 6.5 | ||||
| Albumin | 3.5 | ||||
| Globulin | 3.0 | ||||
| Adenine:guanine ratio | 1:1 | ||||
| Total bilirubin | 0.24 | ||||
| Direct | 0.031 | ||||
| Indirect | 0.21 | ||||
| CRP | 16.9 | ||||
| Test | 5.11.22 | 6.11.22 | 8.11.22 | ||
| ESR 1 hour | 93 | ||||
| Urine routine | Normal | ||||
| Clinical pathology | |||||
| Test | 5.11.22 | 6.11.22 | 8.11.22 | Outside investigation | |
| TC | 42150 | 24750 | 19000 | 30700 | |
| DC | |||||
| Poly | Segments 27 | 52 | |||
| Ig: (promyelo, myelo, and metamyelo) | Metamyelocyte 01 | 0 | |||
| Lymph | 61 atypical 08 | 33 | |||
| Eos | 0.0 | 2 | |||
| Monocytes | 03 | 12 | |||
| Basophils | 0.0 | 0 | |||
| Packed cell volume | 30.1 | 23.1 | 20.86 | 22.6 | |
| Mean corpuscular volume | 56.8 | 55.8 | |||
| Mean corpuscular hemoglobin (MCH) | 16.8 | 16.9 | |||
| MCH concentration | 29.6 | 30.3 | |||
| Hb | 8.9 | 8.9 | 7.6 | 8.4 | |
| Red blood cell (RBC) count | 5.3 | 4.14 | |||
| Platelet count | 12.01 | 9.45 | |||
| Peripheral smear (8.11.22) | |||||
| RBC microcytic hypochromic, anisopoikilocytosis with lymphocytic leukocytosis and thrombocytosis | |||||
| Microbiology | 5.11.22 | ||||
| Blood culture | Streptococcus pneumoniae | ||||
| Urine culture | No growth | ||||
| Acid-fast bacteria | Negative | ||||
Fig. 2: Two-dimensional (2D) ECHO done on admission revealed left main coronary artery dilatation (>3 Z score)
Fig. 3: Blood culture showed Streptococcus pneumoniae growth
Fig. 4: Repeat ECHO after 6 weeks showed resolution of the coronary dilatation
DISCUSSION
The etiopathogenesis of KD is multifactorial, with an interplay of infectious triggers and genetic susceptibility causing an abnormal immunological response. There are various observations supporting the theory that infections trigger KD. These include the mono-modal age distribution with a peak incidence in late infancy (9–11 months of age), which is when the protection of passively transmitted placental antibodies wanes and the seasonal variations in KD with a higher incidence in winter similar to seasonal fluctuations seen in infectious agents such as viruses. There are also epidemics and clusters of cases. The superantigen theory concludes that the bacterial super antigens are related to the causation of KD. The toxins of staphylococcus and streptococcal pyogenes might act as super antigens. The superantigens attach to T-cell receptors and release immunological mediators. In the acute phase, there are cluster of differentiation 8 T-cells infiltration, IgA plasma cells and macrophages in the coronary arteries, similar to acute viral infections.1,2
There are only a few case reports with proven bacteremia and KD reported worldwide.3,4 The various pathogens reported are Klebsiella, Escherichia coli, Staphylococcus aureus, Streptococcus pyogenes, Streptococcus sanguis, Streptococcus pneumoniae, and Clostridium baratii.3 The presence of bacteremia may not affect the treatment response or coronary artery outcome if KD is appropriately treated and the KD course is similar to that of patients without infection.3,5
A few authors have reported KD associated with pneumococcal infection. These children had been immunized with pneumococcal vaccinations and were infected with nonvaccine serotypes, such as 12 F,3 24 B,4 and 15 C.6
The most common prevalent serotypes of pneumococcus reported in India are 1, 2, 3, 4, 5, 6A, 6B, 7F, 8, 9A, 9N, 9V, 10A, 12F, 14, 15B, 18C, 19A, 19F, and 23F.7. Serotype 19A is included in PCV 13 but not in PCV 10. PCV 10 covers serotypes 1, 5, 7F, 4, 6B, 9V, 14, 18C, 19F, and 23F, and the additional serotypes covered by PCV 13 are 3, 6A, and 19A. The newer vaccines currently undergoing trials are PCV 15 and 20 valent pneumococcal vaccine.
CONCLUSION
Pneumococcal sepsis is a vaccine-preventable disease, and our patient had not received the Pneumococcal vaccine and had developed pneumococcal sepsis with vaccine serotype (19A).
CLINICAL SIGNIFICANCE
Thus, pneumococcal vaccination prevents not only the invasive disease but also such infection-triggered complications. We emphasize the need for pneumococcal vaccination (PCV13) for all children to prevent infection and infection-triggered complications.
ORCID
Swati Narasimhan https://orcid.org/0000-0002-7720-0452
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