A Remarkable Coexistence of Systemic Capillary LeakSyndrome and Diabetes in an 11-Year-Old Boy: A CaseReport and Review of the Literature

Systemic capillary leak syndrome (ISCLS) is a rare disease characterized by unexplained reversible capillary hyperpermeabilityfollowed by hypoperfusion, hemoconcentration, and either hypoalbuminemia or total hypoproteinemia. An 11-year-old boy wasadmitted with vomiting, generalized edema, and hyperglycemia, which was preceded by 5 days of coryzal symptoms, lethargy, andoral aft, without fever. On physical examination, he had tachycardia and hypotension, with severe generalized systemic nonitchyedema, and the laboratory tests supported the conclusion that he had severe hemoconcentration with hemoglobin: 184 g/L,hematocrit: 51.3 %, urea: 20 mmol/L, blood glucose: 11.1 mmol/L, and albumin: 19 gr/L, with normal urine analysis. On the fourthday, the patient was diagnosed with ISCLS, by ruling out other causes of shock and hypoalbuminemia. Intravenous immu-noglobulin (IVIG) treatment regimen was administered on two consecutive days (day five and day six). His edema decreased onthe fifth day, and the patient was deemed clinically well. $ere was no compartment syndrome, rhabdomyolysis, or pulmonaryedema in the recovery period. However, respiratory virus panel PCR was positive for respiratory syncytial virus (RSV) andenterovirus, which were thought to be the triggering cause of ISCLS. For the differential diagnosis of diabetes, his fasting serumglucose was 13.4 mmol/L, simultaneous C-peptide was 0.44 nmol/L, and HbA1c was 64 mmol/mol, and urine ketone was positive.However, antiglutamic acid decarboxylase, anti-insulin antibody, and islet cell antibody were negative. At the last outpatient visit,22 months after the diagnosis, his insulin dose was still 0.4 IU/kg/day and HbA1c was 40 mmol/mol, and without prophylaxis,there was no ISCLS attack.Conclusion. Early recognition of ISCLS is important for therapeutic awareness, since it is very rare inchildhood and occurs usually without any prior provoking factors in healthy children. With the increase in awareness of thedisease, knowledge and experiences about pediatric patients may also increase. We think that our case will contribute to theliterature since there have been no pediatric diabetic patients with ISCLS reported.