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

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

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Open Access
|May 2019

Figures & Tables

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.

Table 1

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
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.

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.

DOI: https://doi.org/10.2478/jccm-2019-0012 | Journal eISSN: 2393-1817 | Journal ISSN: 2393-1809
Language: English
Page range: 71 - 75
Submitted on: Mar 28, 2019
Accepted on: Apr 28, 2019
Published on: May 13, 2019
Published by: University of Medicine, Pharmacy, Science and Technology of Targu Mures
In partnership with: Paradigm Publishing Services
Publication frequency: 4 issues per year

© 2019 Neha Gupta, Chrystal Rutledge, published by University of Medicine, Pharmacy, Science and Technology of Targu Mures
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.