The term Munchausen syndrome by proxy (MSP) was
introduced by Meadow in 19771 to describe a unique form of
child abuse that was often easily overlooked. In this condition
children are the victims of illness, ingenuously fabricated or actively
induced by parents, usually the mother.2,3 In MSP the
director of the fictitious play is the socially deprived or
psychologically disturbed mother and the actor is the child who may be
a passive or an active participant in this folly.
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CASE HISTORY |
A.A. first presented to accident and emergency
department at the age of 3.5 years with a 1-week history of fever and
abdominal pain. Within few minutes of arrival he became very lethargic
and profusely sweaty. Blood glucose was 1.1 mmol/L. He was managed with
intravenous glucose infusion. Clinical examination revealed a left
gluteal abscess, soft hepatomegaly of 3 cm.The gluteal abscess was
drained and he was treated with antibiotics. He improved within a few
days and was discharged with advice to undergo further investigations
for hypoglycemia at a later date. He was lost to follow-up. He
presented again after a long gap of 2 years with 3 episodes of
hypoglycemic convulsions in a period of 3 months. Despite rather
extensive investigations, the diagnosis remained unclear and he was
referred to the endocrine unit for further evaluation.
Review of his previous hypoglycemic episodes showed that each of these
episodes occurred in the middle of the night after a good dinner
without any history of preceding febrile illness. On each occasion,
there was severe hypoglycemia, and a minimum of 36 hours of high
concentration glucose infusion was required before an euglycemic state
was reached. The results of laboratory findings at the time of previous
hypoglycemic episodes were as follows: serum electrolytes, venous blood
gas analysis, liver function tests, blood lactate, and blood
acylcarnitine were normal. Urine was consistently negative for ketones
and organic acids. Serum cortisol was appropriately elevated and serum
C-peptide was similarly low for the blood glucose level. He was
subjected to 20-hour of controlled fasting study as a part of protocol
for the investigation of hypoglycemia. At the end of fasting the lowest blood glucose concentration recorded was 4.5 mmol. The fasting urine
sample showed traces of ketones and was negative for organic acids.
He was later readmitted twice within a short interval of few days with
hypoglycemia. The results of both serum C-peptide and insulin levels
estimated at the time of above hypoglycemic attacks were as follows:
serum insulin was 70 mU/L and 473 mU/L and C-peptide was <214 pg/L on
both occasions. Urinalysis did not show the presence of any oral
hypoglycemic agent.
Family and Social History
A.A., the youngest in a sibling ship of 3 lived with his mother,
(henceforth called R.). His parents are divorced. The family lived with
his grandmother, who is a diabetic on insulin. R. is 1 among 9 siblings. She lost her father at an early age and discontinued her
studies after primary school. From her early teens she has a history of
hysterical attacks and depressive episodes. She has been treated by
faith healers and traditional remedies for what have been described as
attacks by evil spirits.
In her early teens she was married to a distant relative many years her
senior. This marriage ended in a divorce within few months. Within a
year of her divorce, R. married again. Her second husband was her first
cousin, a man much older than herself. He had 2 living wives and
several children. This marriage also ended in a divorce. However, R. remarried the same man shortly afterward. The reconciliation was
short-lived, and they separated again after A.A. was born. R. and her
children have been living with her mother in an extended kinship that
includes her second husband, his other wives, and their children. She
is unemployed.
After her mother was diagnosed to have diabetes, R. has learned the
technique of giving insulin injection and helps her mother in the day
to day care of diabetes. A.A. appears to be a very hyperactive child.
He has a very close relationship with his mother and will not interact
with the medical staff except in the presence of his mother.
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DISCUSSION |
In a tradition-bound society, it seems almost heretical to doubt
the axiom that the mother is a child's best historian and that she
would do nothing to harm her child. In the case of A.A., all evidence
seemed to point to the contrary.
The course of each episodes of hypoglycemia in the hospital was
stereotyped. All the documented hypoglycemic episodes occurred in
middle of the night. The duration of each hypoglycemic episode was
prolonged, lasting >24 and 30 hours, despite continuous intravenous infusion of 10% dextrose, intermittent boluses of 25%, 10% dextrose, and the unusual quantity of food and drinks he was taking. Episodes of
hypoglycemia were not related to stress or inter-current illness. All
the hypoglycemic spells occurred after the dinner. Hypoglycemia was not
associated with metabolic acidosis or lactic acidemia and urine was
consistently negative for ketones, despite severe and prolonged
hypoglycemia. In between these spells of hypoglycemia he was in
perfectly good health.
The history, clinical picture, results of the basic investigation, and
the course in hospital did not correspond with the disorders of
glycogenolysis, Neoglucogenesis, fatty acid oxidation, endogenous
hyperinsulinemia, pituitary, and adrenal insufficiency. Exogenous
insulin administration or poisoning with hypoglycemic drugs were the
only explanations for the clinical picture of intractable hypoglycemic
episode refractory to treatment. The source of insulin was unlikely to
be endogenous, because of episodic nature, it did not bear any temporal
relationship to fasting, additionally, the hypoglycemia always occurred
in the middle of the night shortly after a good dinner. The endogenous
hyperinsulinism was excluded by appropriately low C-peptide
concentration during hypoglycemia.
Insulin abuse has been widely reported among adults4 and
in children.5,6 The triad of hyperinsulinism,
low C-peptide, and hypoglycemia is diagnostic of exogenous
insulin.7
The mother's passive and unconcerned behavior to the medical
management, easy access to insulin at home, and her good knowledge about insulin and insulin injection clearly suggested that the mother
was the perpetrator. However, what was unusual was that the mother has
only secondary school education; her knowledge of medical matters is
limited to what she has been taught about the management of her
mother's medical condition.
In the tradition-bound Arab society, women are always kept under the
protection of the extended family. Their access to external influences,
outside their kinship, is limited. In modern, progressive societies,
MSP has been attributed to the stresses and strains of single
parenthood, with little support from the partner.3 Our
patient demonstrates that MSP can occur even in a large extended family
that follows traditional tribal rules.
When the mother was confronted with evidence of factitious nature of
A.A.'s illness, she denied giving the injection to her son. Although
family members were aware of her abnormal personality, appropriate
medical help was not sought. Strangely, male members of the family
refused to participate in counseling. It was only after much persuasion
that the child was moved to a paternal uncle's house. There is as yet
no system of community health follow-up and statutory laws for child
protection in the country.
In a child with unexplained and intractable hypoglycemia administration
of parenteral insulin as a form of child abuse should always be
considered even when it is least likely and simultaneous estimation of
C-peptide and insulin is the only way to establish the diagnosis
of fictitious hyperinsulinemia.
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Abstract
Introduction
1331, Seeb-111, Muscat, Sultanate of Oman.
E-mail: bappal{at}omantel.net.om
