A case report of pediatric calciphylaxis-a rare and potentially fatal under diagnosed condition
Magdalena Stârcea1, Cristina Gavrilovici, Andra Elsayed
1University of Medicine and Pharmacy Grigore T. Popa Iasi Sf Maria Emergency Hospital for Children Iasi, Department of Pediatric Nephrology, Romania.
Insights
Calcific uremic arteriolopathy (CUA) is a rare but serious condition in children with chronic kidney disease. Early detection through urinary pattern monitoring and growth failure assessment is crucial for timely intervention.
Area of Science:
- Pediatric Nephrology
- Vascular Calcification
- Ectopic Calcification
Background:
- Calcific uremic arteriolopathy (CUA) is a rare but severe complication in patients with chronic kidney disease (CKD).
- CUA leads to significant morbidity and mortality due to ectopic calcification.
- This case highlights the challenges in diagnosing and managing CUA in pediatric patients.
Observation:
- A four-year-old boy with stage 5 CKD presented with acute respiratory failure.
- Physical examination revealed irritability, purple subcutaneous nodules, and tachypnea.
- The patient had a history of posterior urethral valve and developed severe renal osteodystrophy.
Findings:
- Pathology confirmed diffuse calcifications in multiple organs, consistent with CUA.
- Despite intensive care including oxygen therapy and inotropic support, the patient experienced rapid deterioration.
- Outcomes included severe hypoxemia, seizures, and cardio-respiratory arrest.
Implications:
- Close monitoring of urinary patterns in children is essential for early CUA detection.
- Growth failure in children warrants prompt investigation for underlying conditions like CUA.
- Timely diagnosis and intervention are critical for improving outcomes in pediatric CUA.
Rationale:
Though to be rare, calcific uremic arteriolophathy (CUA) is an ectopic calcification entity causing pain and disabilities in patients with chronic renal insufficiency, thus increasing the morbidity and mortality.
Patient Concern:
We report a case of four years old boy admitted with acute respiratory failure. Physical examination revealed: irritability, purple subcutaneous hard nodules, tachypnea, dry spasmodic cough, respiratory rate 45/min, heart rate 110/min, blood pressure 100/60 mmHg, with normal heart sounds, no murmurs, hepatomegaly with hepato-jugular reflux. He was diagnosed at 2 years old with stage 5 chronic kidney disease due to untreated posterior urethral valve, and subsequently started peritoneal dialysis. He developed severe renal osteodystrophy, refractory to standard phosphate binders.
Diagnoses:
Pathology examination revealed the presence of diffuse calcifications involving the skin, brain, heart, lung, kidney, stomach and pancreas, consistent with the underlying diagnosis of CUA.
Intervention:
Apart from standard treatment for end stage renal disease and associated co-morbidities, intensive care procedures have been initiated: oxygen therapy, continuous positive airway pressure, inotropic medication (Dopamine, Dobutamine), anticonvulsants (Diazepam), and antiedematous therapy (Dexamethasone).
Outcome:
His pulmonary function rapidly deteriorated up to the severe hypoxemia, seizures and cardio-respiratory arrest, despite the initiation of intensive care measures.
Lessons:
A careful follow up of small children might detect in time an abnormal urinary pattern. The diagnosis of growth failure should also trigger urgent further investigation.
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