Shoshin beriberi mimicking central line sepsis in a child with short bowel syndrome

Greenspon, José; Perrone, Erin E.; Alaish, Samuel M.

doi:10.1007/s12519-010-0022-5

Cited by 17 publications

(17 citation statements)

References 16 publications

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“…10 We know from mouse models of kidney disease that thiamine transporters THTR-1 and THTR-2 are downregulated in the intestines. 8 Both radiation and ESRD can also contribute to malabsorption, and case reports have linked short gut syndrome with beriberi, 35 while abdominal radiation can lead to chronic radiation enteritis. 36 Monitoring of trace metals and other nutrients minimizes the possible contribution of malabsorption in this case.…”

Section: Picu Admitmentioning

confidence: 99%

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

Elias

Sinclair

Blydt‐Hansen

2019

Pediatric Transplantation

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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.

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Section: Picu Admitmentioning

confidence: 99%

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

Elias

Sinclair

Blydt‐Hansen

2019

Pediatric Transplantation

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“…Increased lactic acidosis from TD may be misdiagnosed as characteristics of sepsis or inadequate perfusion 55 . For example, Shoshin beriberi mimicking central line sepsis has been reported regarding a child with short‐bowel syndrome receiving PN 125 . This patient was treated unsuccessfully with antibiotics and supportive measures but was treated successfully with administration of thiamin and folate.…”

Section: Patients On Nutrition Supportmentioning

confidence: 99%

Thiamin in Clinical Practice

Frank

2015

J Parenter Enteral Nutr

132

150

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Thiamin is a water-soluble vitamin also known as vitamin B1. Its biologically active form, thiamin pyrophosphate (TPP), is a cofactor in macronutrient metabolism. In addition to its coenzyme roles, TPP plays a role in nerve structure and function as well as brain metabolism. Signs and symptoms of thiamin deficiency (TD) include lactic acidosis, peripheral neuropathy, ataxia, and ocular changes (eg, nystagmus). More advanced symptoms include confabulation and memory loss and/or psychosis, resulting in Wernicke's encephalopathy and/or Wernicke's Korsakoff syndrome, respectively. The nutrition support clinician should be aware of patients who may be at risk for TD. Risk factors include those patients with malnutrition due to 1 or more nutrition-related etiologies: decreased nutrient intake, increased nutrient losses, or impaired nutrient absorption. Clinical scenarios such as unexplained heart failure or lactic acidosis, renal failure with dialysis, alcoholism, starvation, hyperemesis gravidarum, or bariatric surgery may increase the risk for TD. Patients who are critically ill and require nutrition support may also be at risk for TD, especially those who are given intravenous dextrose void of thiamin repletion. Furthermore, understanding thiamin's role as a potential therapeutic agent for diabetes, some inborn errors of metabolism, and neurodegenerative diseases warrants further research. This tutorial describes the absorption, digestion, and metabolism of thiamin. Issues pertaining to thiamin in clinical practice will be described, and evidence-based practice suggestions for the prevention and treatment of TD will be discussed.

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“…Thiamine deficiency can result from various mechanisms that are not mutually exclusive. The most frequent mechanisms are insufficiency of dietary thiamine (enteral–parenteral supply) and poor intestinal absorptive capacity during malnutrition, tropical enteropathy, or secondary to surgical resection of large portions of the gastrointestinal tract ( 11 , 69 – 71 ). A relative inadequacy of thiamine content to caloric ratio (high carbohydrate diets) is also common.…”

Section: Conditions and Risk Factors Leading To Td In Tropical Pediatmentioning

confidence: 99%

“…Eventually edema, cyanosis, and congestive heart failure may appear. Shoshin beriberi, a fulminant form of congestive heart failure (cyanosis without edema) with type B lactic acidosis, has also been documented in infants ( 6 , 54 – 56 , 67 , 71 , 86 ). The administration of loop diuretics in such cases may unexpectedly worsen TD-related congestive cardiac failure due to increased renal thiamine clearance ( 87 , 88 ), and TD should be suspected in any cardiogenic shock not responding to appropriate therapy ( 6 ).…”

Section: Syndromes Of Td In Childrenmentioning

confidence: 99%

Thiamine Deficiency in Tropical Pediatrics: New Insights into a Neglected but Vital Metabolic Challenge

Hiffler¹,

Rakotoambinina

Lafferty

et al. 2016

Front. Nutr.

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In humans, thiamine is a micronutrient prone to depletion that may result in severe clinical abnormalities. This narrative review summarizes current knowledge on thiamine deficiency (TD) and bridges the gap between pathophysiology and clinical presentation by integrating thiamine metabolism at subcellular level with its function to vital organs. The broad clinical spectrum of TD is outlined, with emphasis on conditions encountered in tropical pediatric practice. In particular, TD is associated with type B lactic acidosis and classic forms of beriberi in children, but it is often unrecognized. Other severe acute conditions are associated with hypermetabolism, inducing a functional TD. The crucial role of thiamine in infant cognitive development is also highlighted in this review, along with analysis of the potential impact of TD in refeeding syndrome during severe acute malnutrition (SAM). This review aims to increase clinical awareness of TD in tropical settings where access to diagnostic tests is poor, and advocates for an early therapeutic thiamine challenge in resource-limited settings. Moreover, it provides evidence for thiamine as treatment in critical conditions requiring metabolic resuscitation, and gives rationale to the consideration of increased thiamine supplementation in therapeutic foods for malnourished children.

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Shoshin beriberi mimicking central line sepsis in a child with short bowel syndrome

Cited by 17 publications

References 16 publications

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

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

Thiamin in Clinical Practice

Thiamine Deficiency in Tropical Pediatrics: New Insights into a Neglected but Vital Metabolic Challenge

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