Case presentation
A 5-year-old child with CAH presented to the emergency department with fever and chills, abdominal pain, recurrent vomiting, cough and loss of appetite since 5 days ago. He was a term baby with a history of CAH (21- hydroxyprogesterone deficiency) from infancy and was operated for it and was on treatment with corticosteroid (Hydrocortisone 10mg ½ tablet t.i.d). He had two brothers with CAH who were about 21 years old and 23 years old with normal development. His parents reported symptoms of cough and a runny nose for a few days prior to this. At presentation, he had tenderness in right upper quadrant in abdominal examination. He had hypotension (BP: 75/pulse) and tachycardia (HR=130/min) and hypoxemia (85%) with tachypnea (RR=52/min). He had no symptoms of meningismus. He had no dermatological involvement. His eyes and throat were normal in exam. In right lung he had decrease in lung sound.
Pediatric endocrinology was consulted and according to his sepsis, stress dose of hydrocortisone (100 mg/m2) was started. Oxygen therapy was started and portable chest X-ray was done. It showed lower lobe pneumonia in right lung with little pleural effusion which was confirmed by chest sonography (consolidation was seen in lower and middle lobes of right lung) (Figure1). Antibiotic therapy for him was started by Ceftriaxone and Clindamycin. Vitamin C, famotidine and zinc gluconate was started. He had a rise in creatinine (1.5 mg/dL) in his laboratory exam with high CRP (66 mg/dL) with no leukocytosis or lymphopenia. His other exam was in normal range. He received a normal saline bolus and was then placed on maintenance IV fluids. His echocardiography showed no pericardial effusion or low ejection fraction. According to immunosuppressive status of patient (under treatment with long-term corticosteroid) and pandemic of COVID19, PCR test for detection of SARS-CoV-2 was sent. After 48 hours his CBC showed lymphopenia (ALC: 720), rise in CRP (97 mg/dL) and SARS-CoV-2 was detected on nasopharyngeal swab. Blood culture, urinalysis, and urine culture were negative. In his abdominal sonography which was done for his recurrent vomiting, it showed two small stone in right kidney (the largest was 3.5 mm in size) and several lymphadenitis (the largest lymph node was 5cm in size). After 24 hour of treatment, his hypoxemia was cured and he did not dependent on oxygen. His tachypnea and respiratory distress was resolved. He received acetaminophen and ondansetron for abdominal pain and vomiting. According to hypotension, inotrope (Dopamine 5μg/Kg/min) was started and patient was transferred to PICU for better monitoring and isolation. His antibiotic was changed to Meropenem and Vancomycin. After 72 hours of admission, his inotrope was discontinued and his fever and vomiting was stopped. Over the course of his admission, he remained hemodynamically stable. He fed appropriately and did not require respiratory support. After 7 days of admission, his vital sign was stable and he had no complaint of vomiting, cough or abdominal pain and his lymphopenia was recurred. He was discharged by advice in quarantine for 2 weeks, oral antibiotic therapy and stress hydrocortisone dose was weaned down to his home regimen. He was followed on the day 14 after admission in clinic and had no complaint and his CXR shows improvement of pneumonia without effusion.