The patient was a 1-year-and-4-mo-old boy. He had drunk about 1 L of an isotonic drink for infants daily since about 10 mo after birth. He was examined by a local doctor due to anorexia and vomiting, found to have car...The patient was a 1-year-and-4-mo-old boy. He had drunk about 1 L of an isotonic drink for infants daily since about 10 mo after birth. He was examined by a local doctor due to anorexia and vomiting, found to have cardiomegaly, and transported to our hospital with suspected myocarditis. After admission, the patient showed polypnea, a decreased level of consciousness, and marked metabolic acidosis and lapsed into circulatory insufficiency, requiring catecholamine administration, endotracheal intubation, and extracorporeal membrane oxygenation. Initially, low-output heart failure due to acute myocarditis was suspected, but the central venous oxygen saturation was high, at 82%. Considering high-output heart failure to be more likely, we evaluated its cause and noted, by urinary organic acid analysis, increases in lactate, pyruvate, 3-OH-butyrate, acetoacetate, metabolic products of branched-chain amino acids, 2-ketoglutarate, 2-OHglutarate, 2-keto-adipate, and 2-OH-adipate. Since the vitamin B1 level was reduced to 12 ng/m L(normally 20-50 ng/m L), a diagnosis of cardiac beriberi due to vitamin B1 deficiency was made. When unexplained heart failure is observed in children, cardiac beriberi must be excluded as a differential diagnosis of myocarditis and cardiomyopathy. The measurement of the central venous oxygen saturation may be useful for the diagnosis.展开更多
Shoshin syndrome is a rare fulminating heart failure caused by thiamine deficiency. In Japanese 'sho' means acute damage and 'shin' means heart. It consists of a rare and hyperacute manifestation of be...Shoshin syndrome is a rare fulminating heart failure caused by thiamine deficiency. In Japanese 'sho' means acute damage and 'shin' means heart. It consists of a rare and hyperacute manifestation of beriberi that was first described in polished white rice-eating communities in various locations across Asia including China, Japan and Indonesia. In a totally different context, ‘Shoshin’ is a word from Zen Buddhism meaning 'beginner's mind', which is considered the necessary attitude to approach the Zen practice. This case report is a prime example of clinical manifestations of thiamine deficiency for the presence of both cardiological and neurological disorders, for hyperacute onset of cardiac failure (shoshin syndrome), for the simultaneous involvement of peripheral and central nervous system, and for the dramatic global recovery after thiamine replacement therapy. We therefore explain why the physician should adopt a beginner’s mind in clinical practice.展开更多
文摘The patient was a 1-year-and-4-mo-old boy. He had drunk about 1 L of an isotonic drink for infants daily since about 10 mo after birth. He was examined by a local doctor due to anorexia and vomiting, found to have cardiomegaly, and transported to our hospital with suspected myocarditis. After admission, the patient showed polypnea, a decreased level of consciousness, and marked metabolic acidosis and lapsed into circulatory insufficiency, requiring catecholamine administration, endotracheal intubation, and extracorporeal membrane oxygenation. Initially, low-output heart failure due to acute myocarditis was suspected, but the central venous oxygen saturation was high, at 82%. Considering high-output heart failure to be more likely, we evaluated its cause and noted, by urinary organic acid analysis, increases in lactate, pyruvate, 3-OH-butyrate, acetoacetate, metabolic products of branched-chain amino acids, 2-ketoglutarate, 2-OHglutarate, 2-keto-adipate, and 2-OH-adipate. Since the vitamin B1 level was reduced to 12 ng/m L(normally 20-50 ng/m L), a diagnosis of cardiac beriberi due to vitamin B1 deficiency was made. When unexplained heart failure is observed in children, cardiac beriberi must be excluded as a differential diagnosis of myocarditis and cardiomyopathy. The measurement of the central venous oxygen saturation may be useful for the diagnosis.
文摘Shoshin syndrome is a rare fulminating heart failure caused by thiamine deficiency. In Japanese 'sho' means acute damage and 'shin' means heart. It consists of a rare and hyperacute manifestation of beriberi that was first described in polished white rice-eating communities in various locations across Asia including China, Japan and Indonesia. In a totally different context, ‘Shoshin’ is a word from Zen Buddhism meaning 'beginner's mind', which is considered the necessary attitude to approach the Zen practice. This case report is a prime example of clinical manifestations of thiamine deficiency for the presence of both cardiological and neurological disorders, for hyperacute onset of cardiac failure (shoshin syndrome), for the simultaneous involvement of peripheral and central nervous system, and for the dramatic global recovery after thiamine replacement therapy. We therefore explain why the physician should adopt a beginner’s mind in clinical practice.
