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Persistent Hyperinsulinaemic hypoglycaemia
in a six-week old Nigerian boy: Clinical presentation and
management challenges in a developing country
1Alphonsus
N. Onyiriuka
 ,
,
2Matthias M. Okposio and 3Louis C.
Onyiriuka
1Department
of child health, university of Benin teaching hospital, PMB
1111, Benin City, Nigeria.
2Department
of child health, university of Benin teaching hospital, PMB
1111, Benin City, Nigeria.
3School
of medicine, college of medical sciences, university of Benin,
Benin City, Nigeria.
Accepted 16th January, 2012 |
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This paper reported a case of a six-week old Nigeria boy with
persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) who
presented with typical clinical and laboratory features and in
whom some management challenges commonly seen in developing
countries were encountered. The clinical diagnostic features
included: at the time of hypoglycaemia (blood glucose level 1.6
mmol/L), serum insulin level of 6 mU/ml; absence of ketonuria
and acidosis; and elevated level of serum C-peptide and a high
glucose infusion rate (12 mg/kg/day) with persistence of the
hypoglycaemia. Management challenges encountered included non-availablity
of a long-acting somatostatin analog (octereotide) for
treatment, lack of investigative facility for distingiuishing
between diffuse beta-cell hyperplasia and focal beta-cell
microadenoma, paucity of surgical expertise for pancreatectomy
and lack of fund to pay for medical treatment abroad in the
event of referral.
Key words:
congenital hyperinsulinism, persistent hypoglycaemia,
hyperinsulinaemia, infant, Nigeria.
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