SAMJ VOL 77 3 FEB 1990 125 Clinical diagnosis of cardiac beriberi D. P. NAIDOO, V. GATHIRAM, A. SADHABIRISS, F. HASSEN Summary Patients and methods The clinical features of 28 patients with biochemically proven Patients presenting to King Edward VIII Hospital, Durban, cardiac beriberi were prospectively studied. Patients recruited with cardiac failure and signs of a hyperdynamic circulation or over a 2-year-period fell into two groups: (I) those with signs acidotic breathing with or without shock were studied over a of a hyperdynamic circulation; and (iI) those presenting with 2-year period. All patients were entered into the study on heart failure and circulatory shock with or without metabolic admission to hospital or within 24 hours. Each patient had a acidosis. All patients showed a dramatic response to intra- venous thiamine; in group 1 the hyperdynamic state resolved comprehensive physical examination, which included chest within hours and in group 2 there was an immediate rise in radiography and ECG. In cases where concomitant acidosis the blood pressure with gradual resolution of the acidosis in was clinically suspected, an arterial blood-gas analysis and 6 - 8 hours. Two patients in whom the diagnosis was missed serum lactate estimation were performed. In all patients 5 ml died soon after admission to hospital. A notable finding was of venous blood was drawn into an ethylenediamine-tetra- that even in the presence of peripheral circulatory shock, acetic acid tube for analysis of thiamine status. A full blood patients with beriberi had preservation of the femoral pulses count, serum urea and electrolyte measurements, and serum often with 'pistol shots' and femoral bruit; this finding has albumin and liver enzyme evaluations were performed on most clinical value in the diagnosis of acute cardiovascular beriberi patients as part of the routine work-up at the time of admission (shoshin beriberi). to hospital. S AIr Med J1990; 77: 125-127. The patient was then given lOO mg thiamine intravenously. Clinical signs of cardiac failure, and the pulse rate, blood pressure and urine output were monitored over the next 5 Beriberi is a reversible form of myocardial failure precipitated days. The ECG was repeated daily for 5 days in most patients. by a dietary deficiency of thiamine. In the Western world it Further treatment decisions were left entirely to the dis- has been described mainly in association with excessive alcohol cretion of the consulting physician and as a result of this 4 intake. l patients were also given diuretics. Earlier studies in South Africa showed that a thiamine Forty-eight control subjects were used to determine the deficiency state could be demonstrated in 25% of patients range of normality in respect of the assay used to determine presenting in cardiac failure in Cape Town. 2 Similarly, Seftel thiamine status. In all of these subjects informed consent was ec al. 3 described a spectrum of myocardial syndromes attri- obtained before blood was taken. There were 20 healthy butable to beriberi in hostel dwellers in Johannesburg. In 1973 athletes; 8 patients with acidosis caused by diabetic ketosis, Lamont4 described an acute' form of cardiac failure in black septicaemia or renal failure, and a further 20 patients with labourers on a Natal sugar estate. In all the above studies cardiac failure as a result of rheumatic valvular disease, hyper- excessive alcohol intake combined with a high-carbohydrate teIlsion or idiopathic cardiomyopathy. diet was a striking feature. The patients described by Lamont4 Red blood cells were separated in the blood samples by responded well to bed rest, hospital diet and small doses of centrifugation and stored at -20°C. Tests of thiamine status diuretics; T-wave inversion in the precordial chest leads on the were done blindly by one of the authors (A.S.) using the ECG developed during recovery. Lamont proposed that these Anthrone method. s Briefly, red blood cells were first haemo- cases represented an early form of cardiomyopathy; however, lysed; the haemolysate was divided into three parts; one the clinical course of the illness together with the ECG sample was incubated with ribose-5-phosphate; the second changes suggests that beriberi was the probable diagnosis. sample with thiamine pyrophosphate (TPP), while the third Although recent clinical reports 5, have highlighted the sample served as a control. The reaction was stopped after 1 occurrence of both the classic high-output and shoshin types hour with trichloro-acetate and the protein-free filtrates of beriberi in the Durban area, biochemical confirmation of examined for the amount of pentose that had disappeared; the thiamine deficiency in these cases was lacking. A more recent baseline transketolase activity (TKA) was derived from this report 7 of 4 cases of biochemically confirmed beriberi describes value. An increase in TKA following addition of TPP (TPP a spectrum of disease manifestations ranging from a hyper- effect) beyond 15% of the baseline value was considered dynamic state to circulatory shock. With such diverse clinical indicative of thiamine deficiency.s presentations an objective assay of thiamine status is necessary Results were analysed for statistical difference using to identify the disease pattern. Student's c-test. This preliminary study reports the clinical presentation of beriberi in patients in the Durban area with biochemically confirmed thiamine deficiency. Results Both classic beriberi (characterised by high-output cardiac failure) and the shoshin type (characterised by shock and acidosis) were seen with equal frequency (Table I). Fourteen Departments of Medicine and Chemical Pathology, p~tients with hi?h-output beriberi were identified by their University of Natal and King Edward VIll Hospital, Durban D. P. NAIDOO, M.B. CH.B., D.C.H. (S.A.), MR.C.P., F.C.P (SA) chmeal presentation; they had a mean TKA level of 74,3 units V. G ATHIRAM, M.B. CH.B., F.C.P. (S.A.) (TPP effect = 17,5%). There were 14 patients who were A. SADHABIRISS, DIP. CHEM. PATH., DIP. CLIN. PATH. admitted in shock or acidosis (mean TKA of 61,6 units and F. HASSEN, M.B. ci·I.B., F.C.P. (SA) TPP effect of 32,4%). Thiamine levels were significantly depressed (P < 0,0001) in both groups compared with controls Accepted 29 Aug 1989. (TKA = 125,2 units, TPP effect = 5,5%). The TKA and TPP 126 SAMT VOL 77 3 FEB 1990 TABLE I. THIAMINE STATUS OF PATIENTS WITH BERIBERI AND NORMAL CONTROLS No. of Mean transketolase Mean thiamine patients activity pyrophosphate Classic beriberi* 14 74,3 ± 16,9 17,5± 14,2 Shoshin beriberi* 14 61,6 ± 27,5 32,4 ± 21,7 Controls 48 125,2 ± 32,6 5,5 ± 5,1 * p= 0,000. levels were much lower in patients with shoshin beriberi arteries and a high pulse pressure, all indicative of a high- indicating a relationship between' degree of deficiency and output state, were consistent features in all patients with the severity of myocardial failure. classic type of beriberi (Table Ill). In addition, femoral bruits There was no significant anaemia in either of the groups were present in 2 patients with classic beriberi. Seven patients (Table 1I); however, the mean corpuscular volume (MCV) was with the shoshin type of beriberi presented with peripheral elevated in both - this was more evident in patients with cardiovascular collapse with hypotension, thready pulses and shoshin beriberi (Table 1I). Although the serum albumin peripheral cyanosis (Table Ill). A surprising finding was that levels in patients with beriberi were normal there was a 7 patients who had been labelled as shoshin - because they marked elevation in the )'-glutamyl transferase levels, here either had severe acidosis or collapsed peripheral circula- again the elevation being more prominent in patients with tion - were found to have bounding carotid and femoral shoshin beriberi (Table 1I). pulses indicative of a hyperdynamic central circulation; 'pistol shots' were audible over the femoral arteries in 4 of these patients. Peripheral neuropathy was diagnosed clinically in 14 patients TABLE 11. MEAN HAEMATOLOGICAL AND BIOCHEMICAL (Table Ill); the neuropathy was of a mixed sensory and motor FINDINGS IN PATIENTS WITH BERIBERI type characterised by a loss of sensation to light touch and pinprick and loss of the Achilles tendon reflex. Three patients Classic beriberi Shoshin beriberi (2 shoshin, 1 classic) had concomitant Wernicke's encepha- Hb (gll) 13,75 ± 0,97 13,95 ± 2,0 lopathy. MCV (f1) 101,1 ± 3,5 107,2 ± 5,1 Two patients presented to the outpatient department in GGT (UII) 94,5 ± 45,0 116,4 ± 82,7 shock; both of them had severe acidotic breathing with clear Albumin (gll) 38,7 ± 5,5 38,5 ± 5,6 lung fields on auscultation. These patients died shortly after Hb = haemoglobin; MCV = mean corpuscular volume; GGT = -y-glutamyl trans- arrival while still being assessed and before any treatment ferase. could be instituted. Assay of thiamine status was subsequently done on a sample of blood drawn previously for a full blood count; in both cases severe thiamine deficiency was present. Acidotic breathing was present in 9 patients with shoshin A history of excessive alcohol intake was obtained from all . beriberi (Table Ill) and this was confirmed by a high anion patients; the alcohol consumed was usually in the form of a gap, low bicarbonate and elevated lactate levels (Table IV). traditional Zulu beer brewed at home or available commer- Acid-base balance was usually normal in patients with classic cially. The majority of patients appeared to be well nourished beriberi (Table IV). Metabolic acidosis was a feature in 2 with preservation of the albumin levels (Table 1I); 2 patients patients with high-output cardiac failure; the lactate level was had concomitant pellagra. elevated in one, who had concomitant bacterial pneumonia, Chest radiography on admission showed mild cardiomegaly which probably contributed to the acidosis. (mean cardiothoracic ratio 56,1%). Upper lobe congestion was A hyperdynamic precordium with collapsing peripheral noted in 3 patients and a right pleural effusion was present in pulses, Corrigan's sign, audible 'pistol shots' over the femoral a further 3; pulmonary oedema was not a feature. The response to treatment with thiamine was dramatic (Table V). The hyperdynamic state subsided within minutes in patients with classic beriberi. In patients with shoshin TABLE Ill. CLINICAL FINDINGS IN BERIBERI beriberi there was an immediate increase in blood pressure Classic beriberi Shoshin beriberi with the hyperdynamic state becoming more prominent; this Hyperdynamic state 14 7 gradually subsided within the next few days. Acidosis gradually Circulatory shock o reversed in the following 6 - 8 hours in all patients treated -- 7 Acidotic breathing 2 9 with thiamine. Intravenous sodium bicarbonate given to 5 Peripheral neuritis 7 7 patients on admission failed to correct the acidosis until thia- mine was added to the treatment. Diuresis occurred imme- diately in patients with high-output failure but was delayed by up to 24 hours in 5 patients with shoshin beriberi; this may have been due to the development of acute tubular necrosis following prolonged hypotension. 9 TABLE IV. MEAN ACID-BASE STATUS IN BERIBERI There were 3 deaths after the initiation of therapy. Two Classic beriberi Shoshin beriberi were patients with shoshin beriberi; one patient had an inter- Anion gap (mmolll) 11,8 ± 3,4 21,6 ± 12,7 current pneumonia and the other's course was complicated by Lactate (mmolll) 4,25 ± 3,4 11,4 ± 7,1 acute renal failure. One patient with classic beriberi showed a Bicarbonate (mmolll) 22,2 ± 3,8 5,8 ± 4,0 dramatic initial response to thiamine; this patient died suddenly on the 5th day in hospital, presumably from a pulmonary embolus. An autopsy was not performed. SAMJ VOL 77 3 FEB 1990 127 TABLE V. OUTCOME OF THERAPY IN BERIBERI Classic beriberi Shoshin beriberi Hyperdynamic state Subsided immediately Became more prominent Blood pressure Rise (diastolic) Rise (systolic) Diuresis Immediate After 24 h Resolution acidosis Not applicable Within 24 h T-wave inversion 9 patients 9 patients Discussion tion of beer with thiamine may obviate this serious complication of an already rampant social disease (alcoholism).12 Future In agreement with previous reports,2,3 both ends of the spec- research should also be aimed at determining the presence of trum of clinical manifestations of cardiac beriberi were thiaminases in home-brewed beer. observed in this locality. Intermediate stages between these As reported in other studies 3,9,13 this condition carries a high two extremes, viz. hyperdynamic state with acidosis and sho- mortality rate if it goes unrecognised. Two patients in this shin beriberi with bounding central pulses, which have not series died because the diagnosis had been missed. A high been descrjbed previously, were also seen. index of suspicion is necessary: heart failure with severe In an environment where the prevalence of idiopathic tachypnoea and/or shock should alert the clinician to the cardiomyopathy and valvular heart disease is high, the diag- diagnosis of beriberi. 12 The association of peripheral circulatory nosis of beriberi heart disease may easily be missed. In the shutdown and bounding femoral pulses is an important pointer absence of a diastolic murmur the high-output failure simulates to the diagnosis. The response to thiamine is dramatic, with a acute aortic incompetence while the shoshin type may be rise in blood pressure and disappearance of the acidosis. mistaken for congestive cardiomyopathy. However, in beriberi the lung fields are relatively clear and chest radiography shows only mild-to-moderate cardiomegaly with a mild degree of REFERENCES upper lobe blood diversion;lo furthermore, arterial blood gas I. Blakenhorn MA. 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