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Paeds Vivashaematology-oncology-and-transfusion

Paeds Vivas · haematology-oncology-and-transfusion

Sickle cell acute complications: Viva

Branching clinical structured oral on the acute complications of sickle cell disease in children, covering the recognition and management of the vaso-occlusive pain crisis, acute chest syndrome, acute splenic sequestration, acute stroke, and priapism, with the exchange-transfusion rule for stroke, the cautious-transfusion rule for sequestration, and the four-hour rule for priapism, appraising the NHLBI 2014 report, the ASH 2020 guidelines, the STOP trial, the Vichinsky study, and the Platt epidemiology.

branching clinical structured oral4 min readVerification in progress

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RACP DWERACP DCEMRCPCH Clinical
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RACP DWERACP DCEMRCPCH Clinical
Prompt
A four-year-old boy with HbSS sickle cell disease, admitted overnight with a back and limb pain crisis, develops a fever, breathlessness, and a cough. His oxygen saturation is 89 percent on room air, and a chest radiograph shows a new right lower lobe infiltrate. The examiner asks how you make the diagnosis, what you do immediately, when you transfuse and by which method, and how you prevent recurrence.

This is acute chest syndrome complicating a vaso-occlusive pain crisis in a child with sickle cell disease. The structured oral runs from the diagnosis through the immediate resuscitation, the transfusion strategy, the avoidance of harm, and the prevention, and it ends with the parallel emergencies of the stroke and the sequestration. [4]

Opening: make the diagnosis

Examiner. The boy was admitted for a pain crisis and now has a fever, a cough, and a saturation of 89 percent on room air. What is the diagnosis, and why did it appear now? [4]

Candidate. The diagnosis is acute chest syndrome, defined by the new pulmonary infiltrate on the chest radiograph together with the fever, the breathlessness, the cough, and the hypoxia, and the saturation of 89 percent marks a moderate-to-severe episode. It appeared after admission because the rib and chest pain of the crisis caused splinting and atelectasis, which seeded the lung, and this is the typical pattern in which the acute chest follows the pain crisis by a day or two. The acute chest syndrome is the leading cause of death in sickle cell disease, so I act now. [4]

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References4ShowHide
  1. [1]Yawn BP, Buchanan GR, Afenyi-Annan AN Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA, 2014.PMID 25203083
  2. [4]Vichinsky EP, Neumayr LD, Earles AN Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med, 2000.PMID 10861320
  3. [2]DeBaun MR, Jordan LC, King AA American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults. Blood Adv, 2020.PMID 32298430
  4. [7]Turner JM, Kaplan JB, Cohen HW, Billett HH Exchange versus simple transfusion for acute chest syndrome in sickle cell anemia adults. Transfusion, 2009.PMID 19309475
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