Unusual presentation of Kawasaki disease with bloody diarrhea and acute renal failure

This is a case of an unusual presentation of Kawasaki Disease presenting as bloody diarrhea and acute renal failure. These have been increasingly reported to jeopardize the timely diagnosis and proper treatment. As there is not yet available blood test to diagnose it, low threshold should be taken into account for considering KD, when the clinical criteria are not typical. KD with renal manifestations is infrequently described.

A two and half year old child, with no medical issues in the past was admitted in the hospital with three days history of fever and bloody diarrhea. There was no history of vomiting. On examination child was looking unwell but nontoxic with high fever. He was hyperventilating. His throat was red and congested, left tympanic membrane was red and left upper cervical lymph node was enlarged of 2×1.5 cm size, firm, tender on palpation. There were signs of dehydration, blood pressure was normal, he was tachycardic and peripheries were cold. There were no signs of meningeal irritation. There were no skin rashes or any changes in the oral mucosa; abdomen was soft with no organomegaly. His height and weight were on the 50th centile.

The initial investigations revealed hemoglobin of 12 g/dL, white cell count 11,000/mm³ (polymorphs 78%, lymphocyte 20%, and monocytes 2%) platelet 120,000/mm³, C-reactive protein 246 mg/L, blood gas analysis revealed metabolic acidosis with respiratory alkalosis and pH 7.2, bicarbonate 13 and PCo2 30. Renal biochemistry was altered with sodium 127 mmol/L, potassium 3 mmol/L, blood urea 13.8 mmol/L and creatinine 91 mmol/L. Blood salicylate level was normal and blood ammonia level was also normal. Ultrasound of the neck reported as inflammatory lymphadenopathy.

Initial management was with fluid replacement and empirical antibiotics. The following day after admission, child became oliguric and puffy, urine output decreased despite an adequate amount of fluid administration and the high fever continued. Blood pressure was 99/76 mmHg on the 99th centile and he gained 300 g more of weight.

Urine analysis showed 10-12 pus cells/HPF and few RBC and urine culture was sterile, Ultrasound scan of the abdomen revealed kidneys of increased echogenicity with no mesenteric lymphadenopathy.

Stool analysis and culture reported as normal. Blood culture showed no organism Mono spot test was negative, throat swab culture was negative and chest x-ray was normal. Lumbar puncture yielded clear fluid with no cells, protein 0.2 g, glucose 3.5 mmol/L.

A repeat biochemistry on third day revealed: Electrolytes sodium of 127 mmol/L, potassium 5 mmol/L, blood urea increased to 15 mmol/L (normal value 1.2-6.3 mmol/L) and creatinine increased to 117 umol/L (normal value 35-58 umol/L), urine sodium was less than 20 mmol/L and urine potassium 3.5 mmol/L, urine urea was 137 mmol/L, urine urea/blood urea 9. Urine osmolality was 347 mos/L, plasma osmolality was 276. Urine osmolality/plasma osmolality was 1.25. Urine specific gravity was 1.012.

On a repeat hemogram, hemoglobin dropped to 10 g/dL and platelet count to 100,000/mm³, peripheral blood smear was suggestive of hemolytic picture with fragmented RBC and relatively low platelets. Meanwhile his urine output decreased and he gained weight more of 800 g.Until this point the overall picture was suggestive of hemolytic uremic syndrome.

He was carefully managed with strict input-output chart, daily weight monitoring, and 4-6 hourly BP recording, electrolytes measurements, careful monitoring of fluid balance with fluid restriction to previous days urine output with added insensible fluid loss.

On the fourth day of admission child became very sick, he was looking miserable, conjunctivae became red, developed measles like rash all over the body, lips cracked, tongue and buccal mucosa became intensively red and there was subcutaneous edema over palms and sole.

Based on the foregoing clinical findings, he was diagnosed as Kawasaki disease; intravenous immunoglobulin was given along with oral aspirin- anti-inflammatory dose and supportive management maintaining the fluid and electrolyte balance. There was dramatic improvement clinically. Fever subsided within 24 hours of immunoglobulin infusion and urine output gradually improved and before discharge the renal parameters were returned to normal. He developed peeling of skin around the perianal area. An echocardiography done showed normal anatomy and function of the heart. Subsequently, he was discharged on antiplatelet dose of aspirin and advised follow up.

Echocardiography repeated two weeks after discharge showed right main coronary artery aneurysm of 5 mm in size. Child was continued on antiplatelet dose of aspirin and regular follow up in cardiology outpatient department and an echocardiography which was repeated after a year from the disease onset revealed a normal heart.

Kawasaki disease (KD) is an acute vasculitis disease causing inflammation of medium-sized arteries which predominantly affects children less than 5 years of age. . It is manifested by fever followed by vasodilation and mucosal inflammation. It affects children <5 years of age, even if cases over 5 years old have been also reported. It is the commonest cause of acquired heart diseases in children which may lead to serious morbidity and mortality.

Kawasaki disease affects multiple systems of the body, the etiology of which is unknown. Perhaps body's immune reaction to infection along with genetic susceptibility can be attributed to the etiology of KD, as in the case of atopic diseases.⁹ However studies are in the offing on the role of a variety of genes related to inflammation.

It may mimic other systemic disorders as there is multiorgan involvement. Many complications has been described with reference to KD¹ and the most important and life threatening among them is the development of coronary artery aneurysm and is the most common cause of acquired heart disease in children in the most developed countries like Japan and USA.

However many extra cardiac complications are being reported and renal disease is one among them. It was believed that renal system involvement in KD was uncommon, except findings like sterile pyuria and trace proteinuria.

Based on the available information and an exhaustive literature search revealed that the, complications like prerenal acute kidney injury (AKI), renal acute kidney injury caused by tubulointerstial nephritis, immune complex mediated nephropathy, acute nephritic syndrome, renal tubular abnormalities, renal abnormalities in imaging studies, and renal artery lesions are increasingly being recognized in KD

The complications and mortality increase when the diagnosis is delayed. One of the main reasons leading to delayed diagnosis and consequent delayed treatment is the unusual presentation of KD.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5494447/