Clinical manifestations of valproic acid (VPA) toxicity can range from just mild confusion and drowsiness to serious encephalopathy, leading to depressed sensorium and even coma and death. The exact cause(s) of how VPA influences the integrity of brain function remains unknown. Nevertheless, several mechanisms have been postulated including a surge in the blood ammonia concentration. Valproic acid–induced hyperammonemic encephalopathy is a rare yet serious sequalae and that can lead to grave outcomes. We report a case of hyperammonemic encephalopathy with preserved liver function following a moderate VPA intoxication in a toddler, who was successfully managed conservatively. Additionally, we briefly discuss mechanistic basis of VPA toxicity and highlight some of the available potential therapies.

Sickle cell Anemia (SCA) is a common inherited haemoglobinopathy resulting from a single-point mutation on the β-globin subunit of hemoglobin. It is a chronic condition with multi-system involvement. Growth delay, osteopenia and hypogonadism are common endocrine dysfunctions with a lower frequency of impaired glucose tolerance. However, there is an association between SCA and diabetes mellitus (DM), though it is very rare. Certainly, there are only a few published reports worldwide outlined this uncommon combination. In this report, we will present the first Libyan case study of co-existence of the two diseases in a-16-month-old male toddler recording the youngest patient diagnosed with such a rare combination. The child, who was diagnosed earlier with SCA, brought with concerns of frequent changing nappies (polyuria) and excessive thirst (polydipsia) for 2 weeks that worsened recently. On admission, the toddler was distressed, lethargic and his lab parameters showed hyperglycemia, ketonuria, glycosuria and acidosis, a diagnosis of diabetic ketoacidosis (DKA) on the background of SCA therefore was made. Further observations are warranted to properly guide about the diagnosis and management of such rare cases.