Acute Shoshin beriberi syndrome immediately post–kidney transplant with rapid recovery after thiamine administration

Abstract

Pediatric kidney transplant surgery is usually well tolerated, despite suboptimal physical conditioning that may result from uremia and nutritional deficiencies that accompany end‐stage kidney failure. Nutritional supplementation is used to overcome such deficiencies, especially for children needing dialysis. Thiamine, a water‐soluble vitamin also known as vitamin B1, is a critical cofactor in energy metabolism and may be competitively inhibited by the antimetabolite oxythiamine, a uremic toxin that accumulates in kidney failure. We report a case of a thiamine deficiency syndrome leading to overwhelming cardiac dysfunction, metabolic instability, and hemodynamic compromise, after otherwise uneventful kidney transplant surgery. Prior to transplant, this 14‐year‐old boy was treated with peritoneal dialysis and received thiamine supplementation. Post‐transplant, the patient first developed hyperglycemia, then lactic acidosis, and subsequently hemodynamic instability despite escalating treatment with volume resuscitation and inotropic medication. He made a rapid and complete recovery after administration of IV thiamine. This is the first reported case of Shoshin beriberi syndrome in a pediatric kidney transplant recipient. Inadequate dialysis may have been a key factor, with toxin accumulation and thiamine transporter downregulation contributing to his status. Functional thiamine deficiency should be considered as a potential treatable cause of early post‐transplant hemodynamic instability.

Pediatric thiamine deficiency disorders in high‐income countries between 2000 and 2020: a clinical reappraisal

“…The outcome can be exacerbated by loop diuretics without prompt thiamine administration. 66,113,114,125 One of the major findings was the occurrence of acute respiratory distress in tachycardic infants, expressed as a pulmonary arterial hypertension with hyperlactatemia, which is remarkably reversed by thiamine treatment, as noted earlier. 72,74,111,125,127,131 The popularization of pediatric bedside cardiac Doppler ultrasound in infants and the readily available assay of lactate point-of-care testing have allowed such a refined diagnostic and therapeutic achievement, which is becoming routine practice in HICs.…”

“…Diverse TD expressions are described, with clinical, biological (associated hyperlactatemia), and radiologically specific or atypical signs of TD. The cases cover a broad panel of clinical manifestations: shock, pulmonary arterial hypertension, Shoshin beriberi, type B lactic acidosis (reversible by thiamine administration), altered mental status that can progress to coma, limb weakness, ocular abnormalities, and signs of truncated or the entire triad of WE 23,108–127 C10 : PTD related to a few documented cases of autistic spectrum disorders associated with TD and a case of botulism associated with thiaminase production (2%) 128–132 …”

“…This can be caused by the coexistence of underlying malabsorption or excessive digestive or renal loss of thiamine, and/or insufficient removal of oxythiamine (a uremic toxin inhibiting transketolase), as in end‐stage renal disease 205–207 . As such, an unusual cluster of TD cases has been reported to be mostly tied to advanced techniques in dialysis or hemofiltration 114,121,126 . Such a subcategory of PTD risk linked with end‐stage renal diseases has not been fully delineated by Lallas and Desai, 173 while there has been significant and incremental advances in renal replacement‐dialysis modalities (peritoneal dialysis, intermittent hemodialysis, and continuous and hybrid therapies) 208 …”

“…[205][206][207] As such, an unusual cluster of TD cases has been reported to be mostly tied to advanced techniques in dialysis or hemofiltration. 114,121,126 Such a subcategory of PTD risk linked with end-stage renal diseases has not been fully delineated by Lallas and Desai, 173 while there has been significant and incremental advances in renal replacementdialysis modalities (peritoneal dialysis, intermittent hemodialysis, and continuous and hybrid therapies). 208 C4 is represented by a set of reports of neonates and infants/children on TPN or EN with poor thiamine intake or increased intestinal losses in which TD appears after insufficient thiamine supply in therapeutic feeding.…”

Shoshin beriberi in a patient with oral and cutaneous graft-versus-host disease

“…5 Comorbid renal dysfunction decreases expression of vitamin transporters in the intestine, liver, and heart and also leads to accumulation of oxythiamine, a competitive inhibitor of thiamine. 5,8 Diuretics in the setting of renal dysfunction exacerbate thiamine deficiency by increasing urinary excretion of thiamine. 4 Even before the actual hematopoietic cell transplant, cytotoxic conditioning regimens can precipitate thiamine deficiency from prolonged mucositis, nausea, vomiting, and diarrhea.…”