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Pyruvate Dehydrogenase Complex Deficiency: An Unusual Cause of Recurrent Lactic Acidosis in a Paediatric Critical Care Unit

Neha Gupta

Neha Gupta

Department of Pediatrics, Division of Pediatric Critical Care, University of Alabama at BirminghamBirmingham, USA
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Gupta, Neha
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Chrystal Rutledge

Chrystal Rutledge

Department of Pediatrics, Division of Pediatric Critical Care, University of Alabama at BirminghamBirmingham, USA
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Rutledge, Chrystal
  
13. Mai 2019
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Artikel-Kategorie: Case report
Online veröffentlicht: 13. Mai 2019
Seitenbereich: 71 - 75
Eingereicht: 28. März 2019
Akzeptiert: 28. Apr. 2019
DOI: https://doi.org/10.2478/jccm-2019-0012
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© 2019 Neha Gupta, Chrystal Rutledge, published by Sciendo
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Fig. 1

Chest radiograph on admission. The image shows pulmonary hyperexpansion with interstitial prominence and areas of patchy density which may be due to a viral airway disease with areas of atelectasis.
Chest radiograph on admission. The image shows pulmonary hyperexpansion with interstitial prominence and areas of patchy density which may be due to a viral airway disease with areas of atelectasis.

Fig. 2

Change in lactate associated with introduction of feeds. The figure describes an increase in lactate after each time the patient is started on PediaSure® feeds. The level decreased to normal after discontinuation of PediaSure®. The figure also describes no change in lactate level after starting the patient on KetoCal® feeds.
Change in lactate associated with introduction of feeds. The figure describes an increase in lactate after each time the patient is started on PediaSure® feeds. The level decreased to normal after discontinuation of PediaSure®. The figure also describes no change in lactate level after starting the patient on KetoCal® feeds.

Fig. 3

Chest radiograph on hospital day 13. The image shows generalized opacification of the left hemithorax with no mediastinal shift. This could represent a combination of pleural fluid and atelectasis. Persistent right lower lobe atelectasis is seen along with right pleural fluid.
Chest radiograph on hospital day 13. The image shows generalized opacification of the left hemithorax with no mediastinal shift. This could represent a combination of pleural fluid and atelectasis. Persistent right lower lobe atelectasis is seen along with right pleural fluid.

Pertinent laboratory results of the patient

LabsValues
Time of collectionpHpCO2 (mmHg)pO2 (mmHg)HCO3 (mmol/L)Serum lactate (mmol/L)
2 hours prior to respiratory arrest (Hospital Day 6) (Capillary gas)7.42228114.28.9
Blood1 hour after respiratory arrest (Hospital Day 6) (Capillary gas)7.1241.512513.58.1
gases36 hours after initiation of Pediasure® (Hospital Day 8) (Venous gas)7.3151.145.725.49.3
After development of ARDS (Hospital Day 13) (Arterial gas)7.42525533.51.3
Serum pyruvate level (mg/dL)2.42 (Reference range: 0.3-1.5)
Blood lactate to pyruvate ratio14.87
Activated PDC level (nmol/min/mg protein)0.35 (Reference range: 1.2-6.52)
Skeletal muscle biopsyPDC/E3 ratio0.4 (Reference range: 0.82-4.54)
Pyruvate dehydrogenase and mitochondrial complex panelPositive for pyruvate dehydrogenase alpha 1 (PDHA1) gene mutation
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Medizin, Klinische Medizin, Allgemeinmedizin, Innere Medizin, Innere Medizin, andere, Chirurgie, Chirurgie, andere, Anästhesiologie, Intensivmedizin und Notfallmedizin
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