Acute respiratory distress syndrome in a child with Kawasaki disease.
Abstract: This report presents a case of classic Kawasaki disease with progression to acute respiratory distress syndrome acute respiratory distress syndrome
See adult respiratory distress syndrome. . The severity of the patient's pulmonary disease led clinicians to suspect toxic shock syndrome toxic shock syndrome (TSS). acute, sometimes fatal, disease characterized by high fever, nausea, diarrhea, lethargy, blotchy rash, and sudden drop in blood pressure. It is caused by Staphylococcus aureus, an exotoxin-producing bacteria (see toxin). . Clinicians need to be aware that pulmonary manifestations of Kawasaki disease can include acute respiratory distress syndrome.
Key Words: Kawasaki disease, acute respiratory distress syndrome, intravenous immunoglobulin, systemic inflammatory immune response
Kawasaki disease (KD) is a systemic vasculitis characterized by fever for 5 days or more, along with four of five associated symptoms (polymorphous skin rash, nonexudative conjunctivitis, mucosal erythema, cervical lymphadenopathy greater than 1.5 cm, and palmar/plantar edema and/or erythema). (1) Pulmonary complications with KD have been reported infrequently. Severe pulmonary disease has been reported in a patient with KD treated with intravenous immunoglobulin (IVIG IVIG Intravenous immunoglobulin, see there ) after 10 days of fever. (2) We present a case of severe pulmonary disease in a patient with KD progressing to acute respiratory distress syndrome (ARDS Ards
District (pop., 2001: 73,244), Northern Ireland. Formerly part of County Down, Ards was established as a district in 1973. Much of its land is devoted to crops and pasture. Newtownards, settled c. 1608 by Scots, is its administrative seat and manufacturing centre. ) after the patient was treated with IVIG within the first 10 days of fever.
A 5-year-old male was transferred to the University of Mississippi Medical Center University of Mississippi Medical Center (UMC) is the health sciences campus of the University of Mississippi (Ole Miss). Located in Jackson, Mississippi (USA), it houses the Schools of Medicine, Dentistry, Nursing, Health Related Professions, and Graduate Studies in the Health with prolonged fever, abdominal pain, and respiratory distress. On the fifth day of fever, he presented to his local hospital with nausea, vomiting, a polymorphous erythematous rash, nonexudative conjunctivitis, red lips, and a strawberry tongue. He was initially treated with intravenous fluids and doxycycline. The next day he developed erythema of his hands and feet and frequent liquid stools. A diagnosis of KD was made, and the patient was given IVIG and high-dose (80 mg/kg per day) aspirin. Before a full 2 g/kg dose of IVIG could be completed, generalized edema, abdominal distension, and respiratory distress developed. A chest roentgenogram roent·gen·o·gram
A photograph made with x-rays. Also called roentgenograph.
roentgenogram (rent´g (CXR) revealed bibasilar infiltrates.
The patient was transferred to the University of Mississippi Medical Center on the seventh day of fever. At admission, his temperature was 100.1[degrees]F, pulse rate was 131 beats/min, respiratory rate was 48 breaths/min, and blood pressure was 75/37 mm Hg. Confluent erythroderma over the entire body was noted. The abdomen was distended distended Medtalk Enlarged, bloated. Cf Nondistended. and very tender to palpation palpation /pal·pa·tion/ (pal-pa´shun) the act of feeling with the hand; the application of the fingers with light pressure to the surface of the body for the purpose of determining the condition of the parts beneath in physical diagnosis. . The patient was treated with intravenous fluids, a dobutamine infusion, clindamycin, doxycycline, and ceftriaxone. An echocardiogram ech·o·car·di·o·gram
A visual record produced by echocardiography.
A non-invasive ultrasound test that shows an image of the inside of the heart. showed a small pericardial effusion, normal ejection and shortening fractions (60% and 30%, respectively), and no coronary changes. Laboratory studies showed a normal white blood cell count white blood cell count,
n a diagnostic clinical laboratory test to determine the number and types of leukocytes present in a measured sample of blood. Overall the normal number of leukocytes ranges from 5000 to 10,000/mm3. (8,400/[mm.sup.3]) with 38% band forms, mild anemia (hemoglobin, 9.4 mg/dL), thrombocytopenia (93,000/[mm.sup.3]), mildly abnormal coagulation coagulation (kōăg'ylā`shən), the collecting into a mass of minute particles of a solid dispersed throughout a liquid (a sol), usually followed by the precipitation or studies [Prothrombin and activated partial thromboplastin times were 13.6 (normal range, 11 to 14 seconds) and 40.5 (normal range, 23 to 36 seconds) seconds, respectively]. Fibrin split products Fibrin Split Products Definition
Fibrin split products (FSP) are fragments of protein released from a dissolving clot. The fibrin split products test is one of several tests done to evaluate a person with blood clotting problems (coagulation), were greater than 10 but less than 40 mg/mL, hypoalbuminemia (1.7 g/dL) and a C-reactive protein of 16.1 mg/dL. An arterial blood gas arterial blood gas Critical care Analysis of arterial blood for O2, CO2, bicarbonate content, and pH, which reflects the functional effectiveness of lung function and to monitor respiratory therapy Ref range pO2 on 2 L/min of oxygen showed a pH of 7.40 with partial pressures of oxygen (Pa[O.sub.2]) and carbon dioxide of 66 and 37, respectively.
The patient was admitted to the pediatric intensive care unit. On the second day of hospitalization, his breathing became less labored. He remained febrile but was hemodynamically stable. He was given 2 g/kg of IVIG over a period of 12 hours. The next hospital day, his abdominal pain improved slightly, but his oxygen requirement continued to increase. Within 36 hours, he had respiratory failure requiring intubation intubation /in·tu·ba·tion/ (in?too-ba´shun) the insertion of a tube into a body canal or hollow organ, as into the trachea.
endotracheal intubation and mechanical ventilation. A CXR showed a normal-sized heart with bilateral alveolar filling (Figure). The patient required significant respiratory support to maintain adequate oxygenation with maximum ventilator settings consisting of a positive end-expiratory pressure positive end-expiratory pressure
n. Abbr. PEEP
A technique used in respiratory therapy in which pressure is maintained in the airway so that the lungs empty less completely in expiration. of 12 mm Hg and fraction of inspired oxygen (FI[O.sub.2]) of 60%. The ratio of Pa[O.sub.2] to FI[O.sub.2] remained approximately 100. Central venous pressure central venous pressure
Abbr. CVP The pressure of the blood within the superior and inferior vena cava, depressed in circulatory shock and deficiencies of circulating blood volume, and increased with cardiac failure and congestion of readings were between 7 and 12 mm Hg. Echocardiography Echocardiography Definition
Echocardiography is a diagnostic test that uses ultrasound waves to create an image of the heart muscle. Ultrasound waves that rebound or echo off the heart can show the size, shape, and movement of the heart's valves and continued to show normal left ventricular function. The patient remained febrile. No pathogens were isolated from blood, urine, or tracheal aspirate cultures.
On the ninth day of hospitalization, day 15 of fever, an echocardiogram revealed normal left ventricular function with a prominent and irregular left coronary artery. The child was given another 2 g/kg dose of IVIG over a period of 24 hours. A heparin infusion was begun. The patient became afebrile afebrile /afe·brile/ (a-feb´ril) without fever.
afebrile adjective Feverless within 2 days. He slowly recovered and was extubated on the 18th day of hospitalization. Supplemental oxygen was discontinued before hospital discharge.
Three weeks into the patient's illness, an echocardiogram showed several aneurysms in the right and left coronaries measuring 3.5 to 5 mm in size. During this time, desquamation desquamation /des·qua·ma·tion/ (des?kwah-ma´shun) the shedding of epithelial elements, chiefly of the skin, in scales or sheets.desquam´ative
1. of the patient's hands and feet occurred. The child was discharged one month after admission on oral warfarin and low-dose aspirin. Warfarin was discontinued 1 month after discharge, since no large (6 mm or greater) aneurysms were seen by echocardiography. One year after discharge, echocardiography showed marked left coronary enlargement with a distal 6 mm aneurysm. Cardiac catheterization was then performed, revealing a 4.5-mm aneurysm in the left anterior descending artery, a dilated proximal circumflex circumflex /cir·cum·flex/ (serk´um-fleks) curved like a bow.
1. Curving or bending around.
curved like a bow. artery, a normal right coronary artery, and no stenosis.
Clinical, pathologic, and radiographic evidence of pulmonary disease has been reported in KD cases. Umezawa et al (3) retrospectively found approximately 15% of KD cases to have abnormalities on CXRs such as presence of a reticulogranular pattern, peribronchial cuffing, atelectasis atelectasis
or lung collapse
Lack of expansion of pulmonary alveoli (see pulmonary alveolus). With a large-enough collapsed area, the victim stops breathing. , air trapping, and pleural effusions. In this series, abnormal CXRs were associated with longer duration of fever, higher C-reactive protein levels, and an increased incidence of coronary lesions, pericardial effusions, and paralytic ileus and nervous system abnormalities. Pneumonia has been the prominent symptom in both classic and atypical cases of KD leading to a delay in diagnosis. (2,4,5) Most of the reported pulmonary disease in KD has been mild. However, in the report by Voynow et al, (2) interstitial lung disease Interstitial lung disease
About 180 diseases fall into this category of breathing disorders. Injury or foreign substances in the lungs (such as asbestos fibers) as well as infections, cancers, or inherited disorders may cause the diseases. with pleural effusions were seen in a child with KD who became hypoxic. Unlike our case, none of the other reports of pulmonary disease in KD have clearly described progression to ARDS.
ARDS is strictly defined as a condition involving impaired oxygenation with the Pa[O.sub.2]/FI[O.sub.2] ratio of 200 or less; bilateral pulmonary infiltrates on CXR; and a pulmonary artery occlusion pressure of 18 mm Hg or less or no clinical evidence of elevated left atrial pressure. (6) Our patient did not have his pulmonary artery occlusion pressure directly monitored. However, no indirect evidence of elevated left atrial pressure was noted: his left ventricular ejection and shortening fractions remained normal as well as his central venous pressure readings. The cause of our patient's ARDS is unknown but probably resulted from a generalized capillary leak after a systemic inflammatory immune response.
Many studies have demonstrated that pulmonary findings in KD are due to an inflammatory process. Pulmonary arteritis arteritis
Inflammation of the arteries. It occurs in diseases including syphilis, tuberculosis, and lupus erythematosus. Varieties not closely associated with systemic disease or disease of an organ outside the cardiovascular system have been described as temporal arteritis, was noted in a series of autopsied KD cases. (7) In a study by Rowley et al, (8) high numbers of IgA plasma cells were found in pulmonary vessels, the trachea, and peribronchial tissue. In a later study by Freeman et al, (9) immunohistochemical staining of inflammatory pulmonary nodules from two patients with KD revealed common leukocyte antigen and IgA positivity. Factor VIII staining was also high, suggesting presence of angiogenesis. These investigators proposed that the pulmonary nodules resulted from an IgA oligoclonal response to a respiratory antigen. (9)
Our patient met criteria for classic KD with four of five associated symptoms of KD and fever for 5 days or more without other explanation. The severity of his symptoms, particularly development of ARDS and his poor response to the first two doses of IVIG, led his physicians to suspect toxic shock syndrome (TSS). Clinical symptoms of TSS and KD are very similar, although fever may be more prolonged in KD. ARDS can occur in TSS and usually occurs as other symptoms of TSS are resolving. (10) It is not surprising that other investigators have reported cases of patients concurrently diagnosed with TSS and KD. (11-14) Microvascular leakage is part of the pathologic process in KD, TSS, and ARDS. (10,15,16) This increase in microvascular permeability may be due to vascular endothelial growth factor Vascular endothelial growth factor (VEGF) is an important signaling protein involved in both vasculogenesis (the de novo formation of the embryonic circulatory system) and angiogenesis (the growth of blood vessels from pre-existing vasculature). (VEGF VEGF vascular endothelial growth factor. ). In a study by Terai This article is about the regions of India and Nepal. For specific Terai/Tarai region of Nepal, see Madhesh. For the former town in Ishikawa Prefecture, Japan, see Terai, Ishikawa. et al, (16) patients with KD with higher post-IVIG treatment VEGF levels were more likely to be resistant to IVIG. Patients who were IVIG resistant were more likely to have weight gain after IVIG and have development of coronary aneurysms. IVIG has rarely been associated with transfusion-related acute lung injury transfusion-related acute lung injury Transfusion medicine 1. A type of ARDS characterized by acute noncardiogenic pulmonary edema–pulmonary white-out–and hypoxia within 4 hrs of transfusing a blood product from a multiparous donor containing plasma . In the only published report of IVIG-associated transfusion-related acute lung injury, a patient had transient noncardiogenic pulmonary edema after receiving a large volume of IVIG over a period of 3 hours. The authors hypothesized that the rapid infusion of a large quantity of granulocyte granulocyte /gran·u·lo·cyte/ (gran´u-lo-sit?) granular leukocyte.granulocyt´ic
band-form granulocyte band cell.
n. antibody provided a stimulus for lung injury (17) It is recommended that the large volume of IVIG required for treatment of KD be given over 10 to 12 hours. (18) Our patient was given IVIG infusions over appropriate periods of time. Moreover, he showed signs of systemic vascular damage with the presence of ascites and peripheral edema, suggesting that his underlying vasculitis Vasculitis Definition
Vasculitis refers to a varied group of disorders which all share a common underlying problem of inflammation of a blood vessel or blood vessels. The inflammation may affect any size blood vessel, anywhere in the body. , not IVIG, was responsible for his acute lung injury. Although we cannot definitely rule out IVIG as a contributing factor to our patient's ARDS, his respiratory status improved simultaneously with resolution of his systemic signs of noncardiogenic edema and inflammation within days of receiving a third dose of IVIG. We postulate that in severe KD, significant vascular leakage occurs, placing the patient at risk for noncardiogenic edema, ARDS, and coronary disease.
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16. Terai M, Honda T, Yasukawa K, et al. Prognostic impact of vascular leakage in acute Kawasaki disease. Circulation 2003;108:325-330.
17. Rizk A, Gorson KC, Kenney L, Weinstein R. Transfusion-related acute lung injury after the infusion of IVIG. Transfusion 2001;41:264-268.
18. American Academy of Pediatrics The American Academy of Pediatrics ("AAP") is an organization of pediatricians, physicians trained to deal with the medical care of infants, children, and adolescents. Its motto is: "Dedicated to the Health of All Children. . 2003 Red Book, Report of the Committee on Infectious Diseases. 26th ed. Elk Grove Village, Illinois Elk Grove Village is a municipality located in northeastern Illinois adjacent to O'Hare International Airport and the City of Chicago. Elk Grove Village encompasses 10.9 square miles in land area with 10.5 square miles located in Cook County and 0. : American Academy of Pediatrics, 2003.
April Lynne Palmer, MD, Thomas Walker, MD, and J. Clinton Smith, MD
From the University of Mississippi Medical Center, Department of Pediatrics, Jackson, MS. Email: firstname.lastname@example.org
Reprint requests to April Lynne Palmer, MD, Department of Pediatrics, 2500 North State Street, University of Mississippi Medical Center, Jackson, MS 39216-4505.
Accepted April 20, 2005.
RELATED ARTICLE: Key Points
* Pulmonary disease may manifest in several different ways in Kawasaki disease (KD).
* Acute respiratory distress syndrome can occur in cases of severe KD.
* Patients with severe pulmonary findings in KD may be resistant to intravenous immunoglobulin.