Clinical Features and Outcomes of Shoshin Beriberi.

Shoshin beriberi is a fulminant form of wet beriberi, but there are no large-scale studies detailing the clinical features of this disease. We investigated the clinical features and outcomes of Shoshin beriberi using data from a nationwide database in Japan.Using the Diagnosis Procedure Combination database, we identified patients with Shoshin beriberi between July 2010 and March 2021. We retrospectively investigated the characteristics, comorbidities, treatment, and in-hospital mortality of patients with Shoshin beriberi. The chi-square test or Fisher's exact test was used for categorical variables, and the Mann-Whitney U-test was used for continuous variables.We identified 62 patients with Shoshin beriberi. The median (interquartile range) age was 63 (48-69) years. Furthermore, 54 patients were male (87%). The most common comorbidity was alcohol-related disorder (34%). The median (interquartile range) length of hospital and intensive care unit stays were 17 (range, 10-35) and 5 (range, 1-9) days, respectively. The proportion of patients who received venoarterial extracorporeal membrane oxygenation, intra-aortic balloon pump, continuous renal replacement therapy, and mechanical ventilation was 11, 5, 29, and 63%, respectively. Among the patients with Shoshin beriberi, 53% received 2 or more catecholamines or inotropes. The in-hospital mortality was 23%. Impaired consciousness at admission was significantly related to in-hospital death (P < 0.001).The present study is the first and largest to describe the clinical features of patients with Shoshin beriberi using a nationwide database. Impaired consciousness at admission was significantly associated with in-hospital death.

Altered Mental Status and Cardiac Failure Due to Thiamine Deficiency in an Overweight Teen.

We describe an overweight but otherwise previously healthy 17-year-old female who presented with altered mental status and rapidly progressive weakness. She was ultimately diagnosed with Wernicke encephalopathy and wet beriberi resulting from severe thiamine deficiency. She required admission to the pediatric ICU because of hypoventilation with progressive weakness and worsening encephalopathy and was found to have impaired cardiac function as assessed by echocardiography. Her heart function and encephalopathy improved on initiation of thiamine repletion. She remained in inpatient rehabilitation for 10 months but still remained weak at discharge. Thiamine deficiency is not commonly considered in the United States as a diagnosis other than in patients with severe alcohol use disorder. However, thiamine may be depleted in as little as 2 weeks if nutrition is inadequate. In such a setting, thiamine deficiency is an important etiology to consider early in the pediatric patient with altered mentation especially because it can be readily and safely treated.

Infrequent but serious? Beriberi And Thiamine deficiency among adolescents and young adults after bariatric surgery.

BACKGROUND: Thiamine deficiency (TD) among adolescents following metabolic and bariatric surgery (MBS) has not been assessed. OBJECTIVE: We assessed TD among adolescents following MBS. SETTING: University Hospital. METHODS: A retrospective chart review was conducted for all adolescents and young adults (aged 10-25 years) who had MBS and subsequently presented with TD at our institution (n = 30). Diagnosis used clinical, laboratory, brain imaging, and neurophysiology criteria. Of 1575 patients, 7 subsequently had TD. Another 23 adolescents had MBS at private hospitals or overseas and presented at our institution with TD. RESULTS: Based on MBS undertaken at our institution, TD prevalence was .45 cases per 100 MBS. The mean age of patients was 19.5 ± 3.23 years, 53.3% were male, 96.7% had sleeve gastrectomy, and time from MBS to admission averaged 4.97 ± 11.94 months. Mean weight loss from surgery to admission was 33.68 ± 10.90 kg. Associated factors included poor oral intake (90%), nausea and vomiting (80%), and noncompliance with multivitamins (71%). Signs and symptoms included generalized weakness, nystagmus, numbness, and paraparesis (83.3%-80%). Seven patients had Wernicke encephalopathy full triad; 16 displayed a mixed picture of Wernicke encephalopathy and dry beriberi; and there were no cases of wet beriberi. Half the patients achieved complete resolution of symptoms, whereas 47% and 40% had residual weakness or persistent sensory symptoms, respectively. There was no mortality. Most common concurrent nutritional deficiencies were of vitamins K, D, and A. CONCLUSIONS: This is the first in-depth study of TD among adolescents after MBS. Although TD is uncommon among adolescents after MBS, it is serious, requiring diligent suspicion and prompt treatment. Bariatric teams should emphasize compliance with multivitamin regimens and follow it up.

Descriptive spectrum of thiamine deficiency in pregnancy: A potentially preventable condition.

OBJECTIVE: Pregnancy, a nutritionally demanding situation in terms of macro- and micronutrient supply owing to heightened maternal, placental, and fetal needs, significantly affects thiamine reserves. Thiamine deficiency during pregnancy and the postpartum period, presenting with varied manifestations and outcomes, is a relatively common condition in our population. The study aimed to understand the various manifestations and outcomes of acute thiamine deficiency in pregnant and postpartum women, emphasizing the significance of early recognition and thiamine therapy to prevent serious complications during pregnancy and after childbirth. METHODS: This prospective study conducted in a tertiary care center in North India enrolled consecutive pregnant and postpartum women presenting with clinical features consistent with thiamine deficiency disorders, such as thiamine deficiency-related neuropathy, high-output heart failure, heart failure with reduced ejection fraction, Wernicke's encephalopathy, gastric beriberi, and thiamine-responsive acute pulmonary hypertension. In addition to capturing medical history including drug intake, dietary consumption, and comorbidities, women underwent brief relevant clinical examinations and laboratory assessments, including whole-blood thiamine levels. Response to intravenous thiamine supplementation was also monitored. RESULTS: Data of 31 women (12 pregnant, 19 postpartum) with a diagnosis of acute thiamine deficiency and a mean age of 28.88 ± 2.69 years were analyzed. The mean thiamine level was 1.28 ± 0.44 µg/dL with mean blood lactate of 3.46 ± 3.33. The most common presentation was gastric beriberi (n = 10), followed by paraparesis (n = 6), high-output heart failure (n = 6), acute pulmonary hypertension, heart failure with reduced ejection fraction (n = 3 each), and an acute confusional state (n = 2). All patients responded to thiamine challenge. CONCLUSION: In the context of borderline thiamine status, particularly in our population with endemic thiamine deficiency and heightened demand for thiamine during pregnancy and the peripartum period, the deficiency can have varied and serious manifestations of dry and wet beriberi. Early recognition of the clinical features and thiamine therapy can be life-saving. There is a need for validated clinical criteria owing to the non-availability of thiamine testing in resource-limited settings.

Pulmonary artery catheter usage in diagnosis of Shoshin beriberi presented with unexplained lactic acidosis.

Wet beriberi is a rare but fatal disease in modern society. The nonspecific clinical manifestations, including symptoms of heart failure and recalcitrant lactic acidosis, can prevent timely diagnosis. The use of a pulmonary artery catheter can promptly confirm a high cardiac output state and plays a crucial role in rapidly deteriorating cases. Appropriate treatment with intravenous administration of thiamine leads to dramatic recovery within hours. We present two cases of Shoshin beriberi, a fulminant variant of wet beriberi, diagnosed in 2016 and 2022 at our institute. The patients experienced haemodynamic collapse and refractory lactic acidosis, which were successfully diagnosed with the use of a pulmonary artery catheter and reversed by thiamine supplementation. We also reviewed 19 cases of wet beriberi reported between 2010 and 2022.

Thiamine responsive high output heart failure of adults: an under-recognized entity.

Thiamine deficiency, commonly presenting as dry and wet beriberi, a lesser-known entity in the present era, is increasingly being reported from Kashmir, a north Indian state. The present study aims to present the clinical profile of patients presenting with high-output heart failure (HOHF). Subjects with a primary diagnosis of denovo heart failure and features suggestive of HOHF were recruited; those who responded to intravenous administration of thiamine alone (responders) were adults with no co-morbidities and those who required other medications particularly diuretics (non-responders) were elderly with co-morbidities and underlying heart disease. Responders showed considerably lower mean thiamine pyrophosphate (TPP) levels and higher mean lactate and venous oxygen saturation than non-responders. More importantly, the mean drop in lactate and SVO2 following thiamine therapy was more in responders. In a setting of high risk for thiamine deficiency, features suggestive of HOHF along with elevated lactate and higher venous oxygen saturation, a response to thiamine challenge may serve as surrogate marker of thiamine deficiency.

Wernicke encephalopathy and beriberi disease presenting as STEMI-equivalent.

Thiamine deficiency is commonly associated with malnutrition, alcoholism and bariatric surgery. Thiamine deficiency can manifest in different ways, especially in developing countries: as peripheric neuropathy, as Wernicke encephalopathy or as beriberi disease. The authors present the case of a 72-year-old male, with a hiatal hernia that led to thiamine deficiency due to malnutrition. The initial clinical manifestation was an ST-elevation myocardial infarct equivalent, an ECG with a shark-fin pattern that evolved to a Wellens type B pattern. The patient evolved with severe altered mental status. A Wernicke encephalopathy diagnosis was confirmed by MRI; the patient was medicated with high-dose thiamine, with quick recovery, both neurologic and cardiac. The clinical history and response to treatment confirm the diagnosis of Wernicke encephalopathy and beriberi disease.

Resurgence of Shoshin beriberi during the COVID-19 pandemic.

This report describes two patients who presented with severe type B lactic acidosis and shock, initially thought to be due to bowel ischaemia/myocardial infarction and pulmonary sepsis, respectively. This led to a delay in the diagnosis of thiamine deficiency. In both cases there was a dramatic response to intravenous thiamine, confirming the diagnosis of Shoshin beriberi. Both patients admitted to drinking home-brewed alcohol during the time of COVID-19 restrictions on alcohol consumption. These cases highlight the need for early diagnosis and immediate empirical treatment with intravenous thiamine in patients presenting with unexplained severe metabolic acidosis and circulatory shock.

Shoshin beriberi and thiamine-responsive right heart failure: A case report in Mayotte Recognition and management of infant Shoshin beriberi.

Infant Shoshin beriberi is an acute life-threatening condition for which the diagnosis is frequently delayed. Therefore, rapid recognition of right heart failure with lactic acidemia is a crucial element in the diagnosis and therapeutic management. We present the case of a 2-month-old girl with bronchiolitis, right heart failure, and lactic acidosis, who quickly and favorably responded to thiamine supplementation. Thiamine deficiency was established through laboratory tests. We present a brief review of the literature with the different thiamine dosages proposed in emergencies and provide an emergency protocol in cases of clinical suspicion, since thiamine supplementation could help to speed up recovery in infants with Shoshin beriberi.

[Thiamin: Simply a vitamin?] / Thiamine : une simple vitamine ?

Vitamin B1 also known as thiamin is an essential vitamin assuring body functioning and comes exclusively from food. Vitamin B1 deficiency is an under-diagnosed disease because it is less frequently suspected in high income countries. However, its risk factors, like alcohol and malnutrition, are common in the general population. Thiamin deficiency can lead to three clinical entities, Gayet-Wernicke encephalopathy, which can progress to Korsakoff encephalopathy, wet Beriberi and its dry form. These diseases are associated with high mortality and heavy long-term sequelae. Rapid diagnosis enables timely treatment.

La vitamine B1 ou thiamine est une vitamine essentielle au bon fonctionnement de l'organisme et provient exclusivement de l'alimentation. La carence en vitamine B1 est une maladie sous-diagnostiquée car sous-évoquée dans les pays à haut revenu. Pourtant, les facteurs de risque, tels que l'alcool et la malnutrition, sont répandus dans la population. Les conséquences d'une carence en thiamine se manifestent sous trois formes, l'encéphalopathie de Gayet-Wernicke, pouvant progresser en encéphalopathie de Korsakoff, le béribéri humide ou sa forme sèche. Ces maladies sont grevées d'une haute mortalité et peuvent entraîner à long terme de lourdes séquelles. Un diagnostic rapide permet d'instaurer un traitement substitutif simple et efficace.

Clinical profile of patients presenting with thiamine-responsive upper-gastrointestinal upset: A pointer toward gastric beriberi.

OBJECTIVES: Although beriberi is considered a forgotten disease in the West, Kashmir has a rice-eating population that has beriberi in endemic proportions. Patients with a thiamine deficiency (TD) occasionally present with gastrointestinal (GI) symptoms, including nausea, recurrent vomiting, loss of appetite, and abdominal discomfort. Together these often respond to thiamine, which points to gastric beriberi. METHODS: Patients with GI symptoms suggestive of TD were recruited from the Department of Medicine at the Government Medical College and its associated hospital, SMHS, in Srinagar, India. Patients were evaluated for serum thiamine levels, serum lactate, biochemical parameters, and transabdominal ultrasonography after ruling out the usual causes of acute abdominal pain and vomiting. RESULTS: A total of 27 patients were recruited with a mean age of 47.28 ± 20.84 y. The mean lactate of patients at the time of admission was 6.43 ± 5.22 mmol/L, and the mean lactate at the time of discharge was 1.23 ± 0.50 mmol/L. All patients had a history of consuming polished rice, washed two to three times before cooking, as the staple diet. The most common GI symptoms were recurrent vomiting, nausea, and loss of appetite. All of the patients responded to the thiamine treatment, and showed improvement in their GI symptoms and decreased serum lactate levels within 2 to 6 h of their hospital stay. CONCLUSIONS: Gastric beriberi is a rare presentation of TD that can lead to severe GI symptoms and lactic acidosis. Given the rapid response to thiamine, it is the standard-of-care treatment in such cases. Thus, clinicians should suspect TD when patients present with either mild or moderate-to-severe GI symptoms and raised blood lactate.

A case of dry beriberi from alcohol use disorder and disordered eating.

Background: Thiamin is an essential vitamin that is involved in every organ system in the body. Thiamin deficiency can present as beriberi or Wernicke's encephalopathy. We seek to educate practitioners in developed countries to include beriberi on the differential diagnosis when a patient with alcohol use disorder, poor diet and/or disordered eating presents with ascending paralysis without albuminocytologic dissociation. Case: In this case report, a 20-year-old female with no past medical history presented with three weeks of ascending paralysis. At presentation, she could not grasp objects, walk, or rise from a seated position. She reported consuming excessive alcohol and an otherwise limited diet due to picky eating. The patient was ultimately diagnosed with acute inflammatory demyelinating polyneuropathy secondary to dry beriberi from severe protein-calorie malnutrition and alcohol use disorder. She received an aggressive thiamin replacement regimen as well as physical and occupational therapy. She was discharged to home 24 days after her initial presentation. Discussion: This patient case offers a unique presentation of ascending paralysis without albuminocytologic dissociation due to severe dry beriberi from a diet of unenriched carbohydrates and excessive alcohol in an otherwise young, healthy adult in the United States. Our goal is that in reviewing the unusual details of this case, providers will be better equipped for the timely diagnosis and treatment of similar cases in the future.

Lessons of the month 1: Shoshin beriberi: A case report of fulminant cardiovascular collapse, intractable hyperlactatemia and deteriorating consciousness.

Shoshin beriberi is a fulminant variant of thiamine deficiency, often presenting with severe lactic acidosis and cardiogenic shock. Due to the sparsity of this condition, delays in diagnosis can lead to fatality. However, rapid reversal of symptoms can be easily achieved through intravenous thiamine replacement.In this case report, we discuss a 57-year-old woman, who was previously fit and well, who presented to the emergency department with a 3-day history of extreme malaise, breathlessness and abdominal pain, with marked hypotension and tachycardia requiring vasopressor support and a severe rising lactic acidosis. Upon further questioning, a history of alcohol excess was noted. Rapid reversal of the marked haemodynamic instability was achieved upon administration of intravenous thiamine and the patient was discharged within 48 hours.

A fresh look at thiamine deficiency-new analyses by the global thiamine alliance.

Severe thiamine (vitamin B1 ) deficiency is generally regarded as a problem affecting mostly infants in low-income communities of Southeast Asia and adult alcoholics regardless of their location. However, recent scholarship shows that the disorders associated with thiamine deficiency may also affect heretofore unsuspected populations, and that the scope of disorders, including some long-lasting neurocognitive consequences, is broader than previously thought.

Case Report: Fulminant Infantile Beriberi: A Report of Six Cases.

Thiamine deficiency disorders are an under-recognized public health problem in low- and middle-income countries. Infantile beriberi, the most important symptom for children, is suspected to significantly contribute to infant mortality and lifelong neurodevelopmental morbidity. Lack of awareness, varied clinical presentation, and lack of a readily available diagnostic marker lead to frequent misdiagnoses. We report six thriving infants who presented with an acute fulminant illness with varied clinical manifestations mimicking common childhood illnesses like pneumonia and sepsis. Four of them presented with the severe cardiovascular form, called Shoshin beriberi, and severe pulmonary arterial hypertension. Empirical intravenous thiamine administered to four of the six infants resulted in dramatic recovery. Awareness of the clinical definition of infantile beriberi and treatment with empirical thiamine can be lifesaving.

A BERIBERI UNHEALTHY LATTE: ENCEPHALOPATHY AND SHOCK FROM SEVERE NUTRITIONAL DEFICIENCY.

BACKGROUND: Thiamine deficiency is an uncommon cause of severe illness in the United States that can lead to significant morbidity because of high-output cardiac failure, peripheral neuropathy, and permanent neurologic impairment. We report the case of a middle-aged woman with extreme malnutrition caused by complications of Roux-en-Y gastric bypass (RYGB) surgery who presented with signs and symptoms of severe thiamine deficiency and septic shock. CASE REPORT: A 43-year-old woman who had undergone RYGB surgery and who had multiple complications presented to the emergency department with agitation, confusion, and lethargy. The physical examination revealed an obtunded woman appearing much older than her reported age with significant peripheral edema. She was hypoxemic, hypotensive, and febrile. The initial laboratory analysis revealed a serum lactate level above the measurable limit, a normal thyroid-stimulating hormone, and elevated levels of troponin and brain natriuretic peptide. A transthoracic echocardiogram showed high-output heart failure. The patient's family later revealed that for the past year her diet had consisted almost exclusively of frozen blended lattes. High doses of thiamine and folate were started. Her shock, hyperlactatemia, and respiratory failure resolved by hospital day 3 and her encephalopathy resolved soon thereafter. Why Should an Emergency Physician be Aware of This?: Thiamine deficiency is a rare but reversible cause of shock, heart failure, and encephalopathy. Identifying patients who are at risk for severe nutritional deficiencies may aid in more rapid treatment with relatively benign medications with little downside, in this case high-dose vitamin B1, and ultimately improve patient-oriented outcomes such as mortality, morbidity, and hospital length of stay.

The rediscovery of thiamine deficiency disorders at a secondary level mission hospital in Northeast India.

In this article, we report the different presentations of thiamine deficiency disorders seen at a remote rural mission hospital in Northeast India, including investigations, treatment, and recovery. Two case studies, one of an infant with cardiac beriberi and the other of a nonalcoholic adult presenting with peripheral neuropathy, cardiomyopathy, and metabolic acidosis and responding to thiamine supplementation, are described in detail. We share our experience with these clinical entities over the past two decades, including recent research and lessons learned, and suggest ways forward to identify at-risk populations in Northeast India, improve early diagnosis and treatment, and promote preventive public health strategies.

Infantile thiamine deficiency: Redefining the clinical patterns.

OBJECTIVES: Thiamine deficiency (TD) is frequently suspected and treated at our hospital. In our retrospective study, we aimed at finding the clinical and laboratory spectrum of infantile TD presenting to a single center over a period of time. METHODS: The diagnosis was made on criterion standard of response to thiamine challenge. RESULTS: TD was suspected in 189 infants at admission; 43 infants were diagnosed as having TD in three distinct forms and a fourth group with mixed presentation. The first group (n = 30), which was the youngest (mean age = 67 d), was always associated with lactic acidosis. They had history of reflux and suddenly became irritable and developed acidotic breathing. This further worsened into shock (46%) and acute respiratory failure (50%). The second group (n = 5) presented with pulmonary arterial hypertension. They had hoarseness of voice and irritability. Chest radiograph showed prominent pulmonary conus. Their clinical course was complicated by congestive heart failure in three. Echocardiographic response to thiamine was uniformly seen within 3 d in this group. The clinical presentation of infants with Wernicke's encephalopathy (n = 5) who were the oldest of all (mean age = 190 d) was constantly marked by presence of bilateral ptosis and encephalopathy preceded by occurrence of vomiting. Their head ultrasonography showed presence of hyperechoic basal ganglia. CONCLUSIONS: Three clinically distinct forms of TD were recognized. Lactic acidosis was a universal finding in acidotic form. Infants with pulmonary hypertension as primary presentation are typically associated with aphonia. Infants with Wernicke's encephalopathy can be clinically diagnosed by presence of encephalopathy and ophthalmic signs (ptosis).