Vol 41 # 3 September 2009 - Kma.org.kw

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KUWAIT MEDICAL JOURNAL September 2009
Case Report
Urosepsis Simulating Congenital Adrenal
Hyperplasia in an Infant
Fahad Al-Mukhaizeem 1 , Abdullah Shamsah 2 , Homoud Taher 3
1
Department of Pediatric Emergency Medicine, Mubarak Al-Kabeer Hospital, Kuwait
2
Department of Pediatric Emergency Medicine, New Dar Al-Shifa Hospital, Kuwait
3
Department of Family Medicine, Mishrif Polyclinic, Kuwait
Kuwait Medical Journal 2009; 41 (3): 254-256
ABSTRACT
Urinary tract infection in infants simulating congenital
adrenal hyperplasia is rarely reported in literature.
We report a case, which was misdiagnosed as a case of
congenital adrenal hyperplasia.
A three-month old female infant was admitted to a local
hospital for persistent vomiting and severe dehydration. Her
biochemical profile revealed hyponatremia, hyperkalemia
and metabolic acidosis. She was initially misdiagnosed as
congenital adrenal hyperplasia (CAH). Her urine examination
revealed a urinary tract infection. Upon investigation there
was no evidence of an obstructive uropathy. A full septic
work up was performed and she was treated for a suspected
urosepsis. Her electrolytes imbalance improved to normal and
she had a complete recovery after antibiotic therapy. This case
demonstrates the importance of urine culture and ultrasound
examination in suspected case of pseudo-hypoaldosteronism
KEY WORDS: congenital adrenal hyperplasia , pseudo-hypoaldosteronism , transient pseudohypoaldesteronism , urosepsis
INTRODUCTION
Pseudo-hypoaldosteronism (PHA) was
described as a salt losing condition seen in infants
with vomiting, dehydration, hyponatremia,
hyperkalemia, metabolic acidosis, and increased
plasma aldosterone concentration with normal
adrenal function and normal renal anatomy [1] . In
addition to sporadic cases, familial, recessive and
dominant inheritance has been described [2] .
PHA is a rare salt wasting syndrome of infancy
and is postulated to be caused by renal tubular
insensitivity to mineralocorticoids. It may be
an isolated condition or may be associated
with renal disease [3] . A wide variety of urologic
lesions have been reported in association with
transient pseudo-hypoaldosteronism (TPHA),
these include obstructive and non-obstructive
lesions [4] .
The prompt diagnosis of the potentially fatal
congenital adrenal hyperplasia (CAH) in neonate
is critical. However, neonatal infection is actually
more common. Early onset neonatal infection
occurs within the first five days of life.
Infection is caused by organisms acquired
during intra-uterine or intra-partum stages. Late
onset neonatal infection occurs after seven days and
frequently results from colonization. Escherichia coli
is one of the most important pathogen of new born
and young infants causing sepsis, meningitis, and
urinary tract infection [5] .
We report an unusual case of neonatal urosepsis
secondary to E.coli, with clinical presentation
simulating CAH. We will review the literature with
emphasis on the differential diagnosis and treatment
of this condition.
The purpose of this communication is to report
the apparent association of urinary tract infection
in a female neonate who had multiple electrolytes
abnormalities and the adrenogenital syndrome.
CASE HISTORY
A three-month-old girl born at term by
spontaneous vaginal delivery was seen in a
community hospital with a one week history of
persistent vomiting, increased irritability and
decrease feeding. She had previously been fit and
well. On the day of admission the parents reported
that the baby was lethargic and difficult to arouse.
On assessment at the community hospital, the infant
was found to be severely dehydrated. Physical
examination revealed a heart rate of 190 beats/min,
a respiratory rate of 48 breaths/min, capillary refill
of five seconds, a blood pressure of 84/54 mmHg
and a temperature of 37.2 o C. Chest and abdominal
examinations were unremarkable, and the infant
had normal external genitalia with no clitromegaly
and no hyperpigmentation. She was initially treated
with a 20 ml/kg bolus of intravenous saline (0.9 %
Address correspondence to:
Dr. Fahad Al-Mukhaizeem, MD, FRCPC, FAAP, Department of Pediatric Emergency Medicine, Mubarak Al-Kabeer Hospital, Kuwait.