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Incomplete Kawasaki Disease in an Infant with Cholangitis Post Kasai Surgery for Biliary Atresia
Naman S. Shetty
,
Corresponding author
Naman.shetty@hotmail.com

Corresponding author.
, Ira Shah**
* Seth G. S Medical College and K.E.M. Hospital, Mumbai, Maharashtra, India
** Pediatric Hepatology, Nanavati Hospital, Mumbai, Maharashtra, India
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="s0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="st0015">Introduction</span><p id="p0005" class="elsevierStylePara elsevierViewall">Kawasaki s disease &#40;KD&#41; is an acute febrile&#44; usually self-limiting illness of infancy and childhood which is associated with vasculitis affecting medium sized vessels&#46; It mainly affects children under the age of five years who comprise about 75&#37; of the cases&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a>&#44;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Biliary atresia &#40;BA&#41; is characterized by obliteration or discontinuity of the extrahepatic biliary system&#44; resulting in obstruction to bile flow&#46; BA is corrected by Kasai Portoenterostomy &#40;KP&#41; and liver transplantation is reserved only for failed cases of KP&#46; Early surgical correction with KP leads to best chance for long term survival of patients with their native liver&#46; Early postoperative complications include&#58; cholangitis&#44; bleeding&#44; leak from anastomosis&#44; prolonged ileus&#44; and intestinal obstruction&#46; Late complications include&#58; cholestasis&#44; recurrent cholangitis&#44; portal hypertension&#44; ascites&#44; hepato-pulmonary syndrome&#44; and formation of bile lakes in the liver&#46; In most cases of BA&#44; biliary cirrhosis invariably develops even in those who have a successful surgically corrected KP&#46; Though cholangitis is a common complication in patients with operated BA&#44; KD has never been reported in patients with BA&#46; We present a child with BA who had undergone KP and then developed cholangitis and subsequently developed KD&#46;</p></span><span id="s0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="st0020">Case Report</span><p id="p0010" class="elsevierStylePara elsevierViewall">A 1 year old girl&#44; a known case of BA&#44; who underwent a KP at 2 months of age in June 2011 presented with fever&#44; clay coloured stools and upper respiratory symptoms for 10 days in April 2012&#46; She had been treated for enterobacter septicemia and pneumonia in March 2012 with meropenem and amikacin for 14 days&#46; She was asymptomatic for 15 days in between when again she presented with present symptoms&#46; On examination&#44; her weight was 7 kg&#46; She had jaundice&#44; hepatosplenomegaly with dilated veins over abdomen and bilateral wheeze&#46; Investigations revealed hemoglobin of 11 gm&#37;&#44; white blood cell &#40;WBC&#41; count of 18&#44;500 cell&#47;cumm&#44; platelet count of 268&#44;000 cells&#47; cumm&#44; bilirubin of 4&#46;5 mg&#47;dL and SGOT 117 IU&#47;L&#44; SGPT 130 IU&#47;L&#46; Urine examination was normal&#46; Ultrasound of abdomen showed hepatosplenomegaly with portal collaterals suggestive of portal hypertension&#46; Her current blood culture did not grow any organism&#46; Respiratory panel for various micro-organisms could not be done due to non-availability and non-affordability&#46; She was treated with the same antibiotics as used previously in view of her previous enterobacter infection along with ursdeoxycholic acid and multivitamins for suspected cholangitis&#46; She responded to the same and became afebrile in next 5 days&#46; She remained afebrile for 5 days and again developed fever&#44; leukocytosis and strawberry tongue with enlarged cervical nodes&#46; Her blood culture was still sterile&#46; Urine examination showed 8-10 pus cells&#47;hpf&#46; Urine culture did not grow any organism suggestive of sterile pyuria&#46; Antibiotics were changed to ciprofloxacin and cotrimoxazole and fluconazole was also added&#44; however she had no response&#46; In view of increasing platelets &#40;505&#44;000 cells&#47;cumm&#41;&#44; high CRP &#40;232 mg&#47;dL&#41; and high ESR &#40;120 mm at end of 1 h&#41; along with clinical features&#44; she was suspected to have incomplete KD&#46; She had no conjunctivitis&#44; edema or rash&#46; Her WBC count was 23&#44;500 cells&#47;cumm&#46; An echocardiography on Day 5 of fever showed left coronary ectasia &#40;1&#46;4 cm&#41;&#46; She was subsequently treated with intravenous immunoglobulin and fever subsided within 24 h&#44; leucocyte count and platelets normalized&#44; CRP&#44; ESR started decreasing&#46; Aspirin was not started in view of portal hypertension and underlying liver disease&#46; She was continued on ciprofloxacin and cotrimoxazole for 6 weeks&#46; On follow-up&#44; echocardiography&#44; in June 2012&#44; there was persistence of the coronary artery ectasia &#40;1&#46;4 cm&#41; and intermittent fever&#46; She was subsequently treated with prednisolone &#40;1 mg&#47;kg&#47;day&#41; which was tapered in next 21 days&#46; Her echocardiography in July 2012 showed decrease in coronary dimensions &#40;1&#46;2 cm&#41; and her fever disappeared&#46;</p></span><span id="s0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="st0025">Discussion</span><p id="p0015" class="elsevierStylePara elsevierViewall">The specific cause of the KD is unknown though viruses&#44; bacteria and allergens have been implicated&#46; Endemic instances of the disease have been found to be associated with the HLA B51 serotype&#46; KD shows a male to female ratio of around 3&#58;2&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Children of Asian descent especially Japanese and Korean are more susceptible to it&#46; The diagnosis of KD is made when the patient is suffering from a high grade fever which does not respond well to antibiotics or antipyretics for more than five days&#44; rash on the trunk&#44; erythema of lips or oral cavity&#44; cracking of lips&#44; strawberry tongue&#44; conjunctival inflammation&#44; enlarged cervical lymph nodes and erythema of palm and soles&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a>&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Myocarditis&#44; diarrhea&#44; pericarditis&#44; arthritis&#44; valvulitis&#44; aseptic meningitis&#44; pneumonitis&#44; lymphadenitis&#44; and hepatitis may be present and are manifested by the presence of inflammatory cells in the affected tissues&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Untreated patients develop cardiac symptoms such as coronary artery aneurysm&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> While coronary artery aneurysm is a late complication of KD&#44; a study by Kato&#44; <span class="elsevierStyleItalic">et al&#46;</span> consisting of 598 patients of which 146 patients demonstrated coronary artery aneurysm using coronary arteriography in the acute phase of the disease&#46; The arteriograms were taken at 22 days to 3 months of onset with a mean of 34 days after onset&#46; Giant aneurysms&#40;&#62; 8 mm&#41; were seen in 4 patients&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> Hepatobiliary dysfunctions such as elevated liver enzymes&#44; cholangitis&#44; hypoalbuminemia&#44; hepatomegaly&#44; cholestasis&#44; jaundice and hydrops of the gall bladder are known complications of KD while reduction in portal vein flow in patient of KD post liver transplant has been reported&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> Acute hepatitis is known to rarely occur in the atypical form of KD in children though cases of classical KD with acute hepatitis have been described Grech V&#44; <span class="elsevierStyleItalic">et al&#46;</span>&#44; and Andrea Taddio&#44; <span class="elsevierStyleItalic">et al&#46;</span><a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">9</span></a><span class="elsevierStyleSup">&#8211;</span><a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a> Acute febrile hepatitis associated with elevation in ESR and CRP can prove useful tools in the early diagnosis of KD&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a>&#44;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> The exact pathogenesis of hepatic dysfunction in KD is still not known but it is attributed to a combination of vasculitis&#44; inflammation&#44; use of non-steroidal antiinflammatory antipyretics&#44; toxin mediated damage and congestive heart failure secondary to myocarditis&#46;<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">13</span></a><span class="elsevierStyleSup">&#8211;</span><a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a> Vasculitis was seen in 6 of 37 KD patients autopsied and is suspected to be the potential cause of liver abnormalities&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">16</span></a> Hydrops of the gall bladder was hypothesized to occur secondary to enlarged lymph nodes in the porta hepatis by some but this has not been observed during surgery&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">17</span></a> Liver biopsy of a KD patient with hepatomegaly but normal LFT&#44; described by Edwards&#44; <span class="elsevierStyleItalic">et al&#46;</span>&#44; showed selective destruction of the of the biliary ductular epithelial cells by polymorphonuclear leukocytes with sparing of the hepatocytes&#46; It was postulated that this selective destruction extended to the distal biliary system as hydrops of the gall bladder was seen&#46; However&#44; vasculitis was not demonstrated in the portal area&#46; Giant mitochondria were also seen on electron microscopy&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">18</span></a> As giant mitochondria probably occur as a result of oxidative damage&#44; the liver abnormalities could be associated with the oxidative stress in a KD patient&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">19</span></a></p><p id="p0020" class="elsevierStylePara elsevierViewall">KD associated with cholangitis have been published in two separate case reports&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">17</span></a>&#44;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">19</span></a> However&#44; KD in association with cholangitis in a biliary atresia patients post KP has not been reported&#46; Post-KP cholangitis is characterized by pyrexia&#44; abdominal pain with or without jaundice&#44; an increase in liver function tests and it may be associated with clay coloured stools and a positive blood culture&#46; Cholangitis is most common in the first year post KP with an overall incidence of 40&#8211;93&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">20</span></a> Our patient presented with fever&#44; jaundice&#44; clay stools&#44; hepatosplenomegaly and portal hypertension&#46; She was treated with antibiotics for cholangitis and became afebrile&#44; but subsequently developed clinical features of KD which responded to IVIG therapy&#46; While KD may have bacterial or viral triggers&#44; a specific etiological agent was not determined&#46; One must also consider the possibility of a hypersensitivity reaction secondary to the use of antibiotics to treat the suspected cholangitis&#46; While our patient did not fulfill criteria for classical Kawasaki disease she could be classified as having incomplete KD along with cholangitis&#46; Resolution of the fever following IVIG therapy and the development of coronary aneurysm further supports the diagnosis of KD&#46; We report this case for the rarity of association and also to highlight that fever in a patient with biliary atresia post KP may not be only cholangitis and especially in the case of persistent fever&#44; other infections and vasculitis should be kept in mind&#46;</p><p id="p0025" class="elsevierStylePara elsevierViewall">Limitation of the case report&#58; Our patient had an incomplete KD along with cholangitis&#46; We could not prove it to be classical Kawasaki disease&#46; However defervescence of fever with IVIG is suggestive of response to treatment KD &#62; We could not rule out other infections in the patient due to cost issues&#46;</p></span><span id="s0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="st0030">Declaration of Conflict of Interest</span><p id="p0030" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span><span id="s0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="st0035">Funding</span><p id="p0035" class="elsevierStylePara elsevierViewall">The author&#40;s&#41; received no financial support for the research&#44; authorship&#44; and&#47;or publication of this article&#46;</p></span></span>"
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        ]
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      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<span id="abs0010" class="elsevierStyleSection elsevierViewall"><p id="sp0005" class="elsevierStyleSimplePara elsevierViewall">Kawasaki&#8217;s disease &#40;KD&#41; is a systemic vasculitis often seen with viral and bacterial infections&#46; Cholangitis is a known complication in biliary atresia patients post Kasai Portoenterostomy &#40;KP&#41;&#46; However KD&#44; in a biliary atresia patient post KP has not been previously reported&#46; A 1 years old girl who had previously undergone a KP for BA&#44; presented with cholangitis which was presumed to be caused by a previous enterobacter infection that she had 2 months ago&#46; However&#44; on treating the cholangitis&#44; the patient developed fever again after ten days which persisted even after changing the antibiotics&#46; By this time she also displayed three of five characteristic features of KD in form of fever&#44; strawberry tongue and cervical adenopathy&#46; Investigations showed high ESR&#44; high CRP&#44; thrombocythemia and dilated coronary vessels on echocardiography&#46; Treatment with intravenous immunoglobulin &#40;IVIG&#41; and steroids caused the symptoms to subside&#46;</p></span>"
      ]
    ]
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ISSN: 16652681
Original language: English
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es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

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Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos