Case summary
A 17-year-old Black male presented to the emergency department (ED) with 2 days of diffuse abdominal pain accompanied by non-bilious, non-bloody emesis, and diarrhea. He had no significant past medical or social history; his family history was significant for a maternal aunt with lupus. On examination, he was afebrile, and his blood pressure was within normal limits. He improved with supportive care and symptomatic management, so he was discharged from the ED with a diagnosis of acute gastroenteritis. Ten days later, he presented to the ED again with persistent abdominal pain and occasional bloody stools. His vital signs were as follows: temperature 98.1 F, blood pressure 119/72 mmHg, pulse rate 56/min, respiratory rate 16/min, and pulse oxygen saturation of 99% on room air. On examination, he appeared uncomfortable and had exquisite abdominal tenderness in the umbilical and left lower quadrant regions. A gastrointestinal pathogen PCR panel was ordered and was negative for all bacterial and viral organisms tested. SARS-CoV-2 PCR was negative. His laboratory findings are summarized in Table 1.
Table 1.
Basic laboratory findings upon initial presentation
| Patient’s value | Reference range | |
|---|---|---|
| WBC | 23.7 k/μL | 3.7–11.7 k/μL |
| Hemoglobin | 15.7 gm/dL | 11.3–15.1 gm/dL |
| Hematocrit | 44.6 % | 35–46% |
| Platelet count | 319 k/μL | 135–450 k/μL |
| Peripheral blood smear | ||
| Absolute basophils | 0.23 k/μL | 0.0–0.0 k/μL |
| Absolute eosinophils | 0.23 k/μL | 0.0–0.3 k/μL |
| Absolute lymphocytes | 1.16 k/μL | 1.2–5.2 k/μL |
| Absolute monocytes | 2.33 k/μL | 0.2–0.6 k/μL |
| Absolute neutrophils | 19.31 k/μL | 1.8–8.0 k/μL |
| Schistocytes, ovalocytes | Occasional | --- |
| Platelet estimate | Normal | --- |
| Sodium | 128 mmol/L | 134–144 mmol/L |
| Potassium | 3.9 mmol/L | 3.4–5.5 mmol/L |
| Chloride | 89 mmol/L | 98–107 mmol/L |
| CO2 | 28 mmol/L | 20–31 mmol/L |
| Blood urea nitrogen | 16 mg/dL | 7–21 mg/dL |
| Creatinine | 0.9 mg/dL | 0.2–1.4 mg/dL |
| Glucose | 98 mg/dL | 65–110 mg/dL |
| Protein | 6.4 g/dL | 6.5–9.0 g/dL |
| Albumin | 2.9 g/dL | 3.0–4.8 g/dL |
| Total Bilirubin | 0.5 mg/dL | 0.3–1.3 mg/dL |
| AST | 20 U/L | 8–53 U/L |
| ALT | 38 U/L | 7–56 U/L |
| Alkaline phosphatase | 126 U/L | 39–253 U/L |
| Lipase | 24 U/L | 5–62 U/L |
| ESR | 9 mm/hr | 0–20 mm/hr |
| CRP | 14.6 mg/dL | > 0.4–1.0 mg/dL |
| Urinalysis | ||
| Ur appearance | Clear | --- |
| Bacteria | None seen | None seen |
| Ur bilirubin | Negative | Negative |
| Ur blood | 3+ | Negative |
| Ur color | Yellow | Colorless, straw, yellow |
| Epithelial cells | 2–5/HPF | 0–5/HPF |
| Ur glucose | Negative | Negative |
| Granular casts | 0–2/LPF | Negative |
| Hyaline casts | 0–2/LPF | 0–2/LPF |
| Ur ketones | Negative | Negative |
| Leukocyte esterase | Negative | Negative |
| Ur nitrate | Negative | Negative |
| pH, UA | 6.0 | 5.0–8.5 |
| Ur protein | 2+ | Negative |
| RBC, UA | 5 – 10 /HPF | 0–2/HPF |
| Specific gravity | 1.020 | 1.003–1.035 |
| Urobilinogen | 0.2 EU/dL | 0.2–1.0 EU/dL |
| WBC, UA | 2–5 | 0–5/HPF |
| Fecal calprotectin | 1140 μg/g | < = 49 μg/g |
| Fecal occult blood | Positive | Negative |
Abdominal x-ray was unremarkable. Abdominal ultrasound revealed a small amount of free fluid in the right lower quadrant and thickened bowel in the left lower quadrant. He was evaluated by the pediatric surgery team. Due to concern for peritonitis, he was taken emergently to the operating room for a diagnostic laparoscopy. This was converted to an exploratory laparotomy when an area of bowel appeared abnormal. Operative findings included thickened ileum and edematous mesentery with prominently enlarged lymph nodes near the border of the bowel. The possibility of a reduced intussusception was considered as the cause of his abdominal pain. There were no fat creeping or masses concerning for intraluminal pathology. Peritoneal fluid cultures were obtained, and he was started on empiric intravenous (IV) ceftriaxone and IV metronidazole for possible intraabdominal infection.
Post-operatively, intermittent, severe abdominal pain requiring narcotic analgesia persisted. In addition, he developed a localized, painless, non-pruritic, palpable purpuric rash on his right hand that extended to his elbow and included some of the palm. Labs were notable for hypoalbuminemia with a serum albumin of 1.7 gm/dL and an increase in serum creatinine from 0.9 to 1.4 mg/dL on hospital day (HD) #3. Peritoneal fluid cultures remained negative at 96 h, so his antibiotics were discontinued. Blood pressure readings and urine output remained appropriate. Nephrology was consulted due to acute kidney injury; additional labs were obtained and are summarized in Table 2. Ultimately, a kidney biopsy was obtained on HD #4 and revealed the kidney-specific diagnosis.
Table 2.
Additional laboratory findings obtained at the time of nephrology consult
| Patient’s value | Reference range | |
|---|---|---|
| Creatinine | 1.4 mg/dL | 0.2–1.4 mg/dL |
| Albumin | 1.7 g/dL | 3.0–4.8 g/dL |
| Urine protein/creatinine ratio | 1,207 mg/g | --- |
| Antinuclear Ab (ANA) | Negative | Negative |
| C3 | 100 mg/dL | 35–181 mg/dL |
| C4 | 20 mg/dL | 6–49 mg/dL |
| Antineutrophil cytoplasmic Ab (ANCA), IgG | < 1:20 | < 1:20 |
| Anti-double-stranded DNA Ab | Negative | Negative |
| Glomerular basement membrane IgG | Negative | Negative |
| Rheumatoid factor | Negative | Negative |
| Streptolysin O Ab | < 55 IU/mL | 0–330 IU/mL |
Questions
What findings do you expect the kidney biopsy to reveal?
Which serological markers might typically be active in patients with this diagnosis?
What is the treatment and prognosis?
Declarations
Conflict of interest
The authors declare no competing interests.
Footnotes
The answers to these questions can be found at 10.1007/s00467-021-05037-4.
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