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1.3 CLINICAL DIAGNOSIS OF CARDIAC BERIBERI. - Clinical diagnosis Powered By Docstoc
					                                                                                                  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
                                                                                  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
                                                                               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
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