Jamaican vomiting sickness

BOX 24-A MAPLE SYRUP URINE DISEASE AND JAMAICAN VOMITING SICKNESS... 

Jamaican vomiting sickness is caused by a toxin (hypoglycin) from the unripe fruit of the akee tree. This toxin inhibits an acyl CoA dehydrogenase of P-oxidation consequently, more glucose must be oxidized to compensate for the decreased ability to use fatty adds as a fuel, and severe hypoglycemia can occur. co-Oxidation of fatty acids is increased, and dicarboxylic acids are excreted in the urine. Unwary children are usually the victims of this frequently fatal disease. 

EMA aciduria maybe associated with several other inherited and acquired conditions, including (1) glutaric acidemia type II (some cases are actually labeled to have ethylmalonic adipic aciduria),(2) disorders of the intramitochon-drial flavin adenine dinucleotide pathway, (3) mitochondrial respiratory chain disorders, and (4) ethylmalonic encephalopathy. Jamaican vomiting sickness (due to ingestion of unripe ackee fruit containing the poison hypoglycin A) and ifosfamide treatment represent two additional causes of ethylmalonic aciduria. 

Tanaka K, Ikeda Y. Hypoglycin and Jamaican vomiting sickness. Prog Clin Biol Res 1990 321 167-84. 

The unripe fruit of the akee tree produces a toxin, hypoglycin, which causes a condition known as Jamaican vomiting sickness. The victims of the toxin are usually unwary children who eat this unripe fruit and develop a severe hypoglycemia, which is often fatal. 

Chalmers, R.A., Lawson, A.M., Whitelaw, A. and Purkiss, P. (1980a), Twin siblings with a Reye s-like syndrome associated with an abnormal organic aciduria, hypoglycaemia, diarrhoea, and vomiting with close similarities to Jamaican Vomiting Sickness. Pediatr. Res., 14,1097. 

Jamaican vomiting sickness is characterized biochemically by profound hypoglycaemia, acidosis without ketosis, and post-mortem by extensive diffuse fatty degeneration of liver and kidneys with small droplet infiltration closely similar to that observed in patients with Reye s syndrome (Section 14.4.2). 

Fig. 14.7 Chromatogram of organic acids extracted using ethyl acetate and diethyl ether from the urine of a child with Jamaican vomiting sickness, separated as their methyl derivatives (diazomethane) on 5 per cent OV-1 using temperature programming from 70°C to 300°C at 4°C min with a 4 min initial isothermal delay. Peak identifications are 1, 3-hydroxybutyrate plus 3-hydroxyisovalerate (approx. 4 6) 2, ethylmalonate 3, methylsuccinate 4, octanoate 5, glutarate 6, adipate 7, isovalerylglycine 8, 4-octenedioate plus furandicarboxylate 9, suberate (octanedioate) 10, /i-hexanoyl-glycine 11, decenedioate 12, sebacate (decanedioate) 13, hippurate 14, n-pentadecanoate (internal standard) 15, palmitate 16, stearate. (Redrawn with modifications from Tanaka et al, 1976)...

Table 14.5 Urinary organic acids in siblings with Jamaican vomiting sickness (after Tanaka et al, 1976a).

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