Multisystem Inflammatory Syndrome in a Child with Scrub Typhus and Macrophage Activation Syndrome.

LAY SUMMARY: Clinical and laboratory parameters of multisystem inflammatory syndrome in children (MIS-C) mimic Kawasaki disease (KD). KD has been described in association with dengue, scrub typhus and leptospirosis. However, MIS-C with concomitant infection has rarely been reported in literature. A 14-year-old-girl presented with fever and rash with history of redness of eyes, lips and tongue. Investigations showed anemia, lymphopenia, thrombocytosis with elevated erythrocyte sedimentation rate, C-reactive protein, pro-brain natriuretic peptide, Interleukin-6, ferritin and d-dimer. Scrub typhus immunoglobulin M was positive. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) immunoglobulin G (IgG) level was also elevated. A diagnosis of MIS-C with concomitant scrub typhus was proffered. Child received azithromycin, intravenous immunoglobulin and methylprednisolone. After an afebrile period of 2.5 days, child developed unremitting fever and rash. Repeat investigations showed anemia, worsening lymphopenia, thrombocytopenia, transaminitis, hypertriglyceridemia, hyperferritinemia and hypofibrinogenemia which were consistent with a diagnosis of macrophage activation syndrome (MAS). KD, MIS-C and MAS represent three distinct phenotypes of hyperinflammation seen in children during coronavirus disease pandemic. Several tropical infections may mimic or coexist with MIS-C which can be a diagnostic challenge for the treating physician. Identification of coexistence or differentiation between the two conditions is important in countries with high incidence of tropical infections to guide appropriate investigations and treatment.

Multi-System Inflammatory Syndrome in a Child Mimicking Kawasaki Disease.

There have been recent reports of children presenting with severe multi-system hyperinflammatory syndrome resembling Kawasaki disease (KD) during current COVID-19 pandemic. Exact pathophysiology is unknown, however, most of the children have multi-organ dysfunction and respiratory system involvement is less common compared to adults. These patients have certain characteristic laboratory parameters different from those seen in children with KD. However, only limited literature is available at present for identification and management of such patients. We report a young girl who presented with fever, rash and other manifestations mimicking classic KD and fulfilling the case definitions for pediatric multi-system inflammatory syndrome. She had lymphopenia, thrombocytopenia and hyponatremia in the absence of macrophage activation syndrome, similar to that seen in patients reported from UK and Italy. Clinical manifestations resolved and laboratory parameters improved with intravenous immunoglobulin and corticosteroids. Early recognition is important to administer immunomodulatory therapy which may be life saving for these patients.

Several cases of a severe multi-system inflammatory syndrome have been reported in children during the current COVID-19 pandemic. Clinical manifestations may resemble Kawasaki disease (KD) which is the most common childhood vasculitis. A 7-year-old-girl presented with fever, rash and abdominal pain. Examination showed maculopapular rash over lower limbs, back, right ear, trunk and abdomen; erythema and swelling over bilateral upper eyelids; conjunctival injection; reddened lips and erythema over palms and soles. She had lymphopenia, thrombocytopenia, with elevated erythrocyte sedimentation rate, C-reactive protein (CRP), pro-brain natriuretic peptide and interleukin-6 (IL-6). Real-time polymerase chain reaction for severe acute respiratory syndrome coronavirus 2 was negative. Serology, however, could not be performed due to unavailability. Macrophage activation syndrome was ruled out with normal ferritin and triglyceride with raised fibrinogen level. Echocardiography showed normal coronary diameters. Child received meropenem, intravenous immunoglobulin, aspirin and methylprednisolone. She improved with resolution of fever, decrease in CRP, increase in platelet and lymphocyte count. Index child had features similar to those reported from UK and Italy: features of KD, abdominal pain, lymphopenia, thrombocytopenia, elevated IL-6 and myocardial dysfunction with no significant respiratory involvement. Pediatricians should be aware of such uncommon presentation in children to initiate early treatment with immunomodulatory therapy.

Surgical management of gastroschisis in North Queensland from 1988 to 2007.

AIM: To review outcomes of gastroschises originating in North Queensland and repaired in the neonatal centre in Townsville from 1988-2007, and compare these outcomes with published data from other centres. METHODS: A retrospective chart review of outcomes after primary operative repair (POR) with wound closure in fascial layers in the theatre, primary non-operative repair (PNOR) with apposition of the umbilical cord and adhesive dressing in the ward, secondary repair (SR) after a silo, and complex repair (CR) of cases with obstruction, perforation or atresiae. Epidemiological data of babies originating in North Queensland but managed in Brisbane were secured from Mater Mothers' Hospital and the Royal Hospital for Children. RESULTS: Fifty cases were treated in Townsville: 16 by PNOR, 22 by POR, 6 by SR and 6 by CR. Outcomes of uncomplicated cases treated by PNOR, POR and SR were similar. Outcomes of complicated cases after CR were significantly worse. No significant differences were found between PNOR in Townsvillle and published outcomes after SR from four overseas units. No differences were detected in outcomes of inborn and outborn babies, those delivered by vaginal or caesarean birth or between indigenous and non-indigenous babies. Three (6%) died. Gastroschisis is increasing in North Queensland, particularly in younger mothers. The incidence is 2-3 times higher in indigenous mothers. CONCLUSION: No significant differences were found between PNOR and other techniques for uncomplicated cases. PNOR avoids the use of theatre and staff, reducing delay and cost, and the need to transport a sick baby.

Perinatal demography of gastroschisis in North Queensland.

AIM: To review the demography of gastroschisis in North Queensland. METHODS: A retrospective chart review of live born cases of gastroschisis originating in North Queensland from 1988 to 2007. RESULTS: Fifty-nine cases were identified, giving an overall rate of 3.2 per 10,000 live births. In mothers <20 years old compared with 20-24 and 25-29 it was 12.1, 6.3 and 1.7. Overall, the rate was higher in Indigenous mothers (6.6 vs. 2.6, OR 2.5; 95% CI 1.4-4.5, P= 0.0018). Overall, the rate increased significantly from 0.7 per 10,000 live births in the first 5 years of the study to 4.8 in the last (trend test P= 0.0015), but it increased particularly in mothers <20 years old, from 2.40 in the first 5 years to 19.3 in the last (trend test P= 0.0177). It also rose from 0 to 3.6 in those aged 25-29 (P= 0.0337) but remained stable in other age groups. There was no difference in the outcomes of babies born to Indigenous or non-Indigenous mothers, or in outcomes of babies delivered vaginally or by Caesarean section, or in outcomes of babies from any particular location in North Queensland. There was no significant difference in the average age of Indigenous and non-Indigenous mothers. CONCLUSION: Gastroschisis is increasing in North Queensland, especially in mothers <20. Overall, the rate in Indigenous mothers is two to three times higher.