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Priapism as a complication of high dose testosterone therapy: A medication error [Case report and a review of literature]

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Priapism is defined as a prolonged, sustained penile erection without sexual stimulus. It rarely occurs in children, but may result in erectile dysfunction and sexual aversion behaviors as one gets older. Testosterone therapy is safely used in children with hypogonadism, delayed puberty, and micropenis. We report a rare case of testosterone-induced priapism in an 8-year-old boy with a micropenis, after receiving an incorrect dose of 500 mg of testosterone injection; his mother (a paramedic) administered it. The patient was managed with observation and cold compresses; without any surgical intervention. This case suggests that hormonal injections be administered by either registered nurses or physicians, "but not by relatives."
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International Journal of Medical and Health Research
17
International Journal of Medical and Health Research
ISSN: 2454-9142
Impact Factor: RJIF 5.54
www.medicalsciencejournal.com
Volume 4; Issue 2; February 2018; Page No. 17-21
Priapism as a complication of high dose testosterone therapy: A medication error
[Case report and a review of literature]
1 Elaf Alzarnougi, 2 Abdulmoein Eid Al-Agha, 3 Yara Kano
1 King Abdulaziz University Hospital, Jeddah, Saudi Arabia
2 Professor of Pediatric Endocrinology, King Abdulaziz University Hospital, P.O.BOX 80215, Jeddah, Saudi Arabia
3 King Abdulaziz University Hospital, Jeddah, Saudi Arabia
Abstract
Priapism is defined as a prolonged, sustained penile erection without sexual stimulus. It rarely occurs in children, but may result in
erectile dysfunction and sexual aversion behaviors as one gets older. Testosterone therapy is safely used in children with
hypogonadism, delayed puberty, and micropenis. We report a rare case of testosterone-induced priapism in an 8-year-old boy with
a micropenis, after receiving an incorrect dose of 500 mg of testosterone injection; his mother (a paramedic) administered it. The
patient was managed with observation and cold compresses; without any surgical intervention. This case suggests that hormonal
injections be administered by either registered nurses or physicians, "but not by relatives."
Keywords: priapism, testosterone, micropenis, medication error
Introduction
The United State Institute of Medicine reports that To Err is
Human’ in November 1999, which focused on preventable
medical errors in order to build a safer health system and
making patient safety the preeminent issue in health care.
Over time, the concept of patient safety increased, and it
became clear that the prescribing, dispensing, and
administration of medications is the key of preventable
medical errors [1]. The National Patient Safety Agency
revealed the fact of those medication errors could occur at
each stage of the medication treatment process. Children were
more prone to these errors than adults. Medication errors in
pediatrics have been estimated to be 3-37% during
prescribing, 5-58% during dispensing, 72-75% during
administration, and 17-21% during documentation [2].
Testosterone is a hormone used in children with micropenis,
hypogonadism, constitutional delay of growth and puberty,
hypospadias, and disorders of sexual differentiation. It is
considered safe in both adults and children, with proper doses
maintained, so that monitoring is recommended [3].
Kaplowitz studied the value of testosterone therapy in males
with delayed puberty. He noted increased frequency of
erections, but it was not excessive in any patient. This appears
to be the case for the majority of patients, but it is important to
be aware that priapism could occur during testosterone
therapy [4-6].
Priapism is a prolonged complete or partial penile erection
lasting more than four hours, but unrelated to sexual stimulus.
There are three categories of priapism: ischemic priapism,
which is considered the most common type in children,
stuttering priapism (intermittent), and nonischemic priapism
[7].
Priapism after administration a high dose of testosterone has
been rarely reported in literature. There were 21-reported
cases of priapism associated with testosterone therapy in
English literature. Two cases were due to administering a high
dose, whereas the rest were therapeutic doses [3, 5, 8-18]. We
reported a case of an 8-year-old boy with micropenis and
priapism, secondary to a high dose of testosterone in a
medication administration error.
Case Report
An 8-year-old Yemeni boy was born prematurely after a 28-
week gestation to no consanguineous parents. He is one twin
in a pair of dizygotic twins, born with a birth weight of 1200 g
and good Apgar scores. Presented to the Pediatric Endocrine
Clinic with a history of micropenis (stretched penile length
was equal to 3.7 cm, which is below the 10th percentile for
ages plotted on the penile length chart). Testicular volume was
2 ml on both sides. No family history of micropenis or
hypogonadism existed. His height was 110 cm (-3.71 standard
deviation below the normal) and his weight was 16.5 kg (-3.02
standard deviation below the normal). He was otherwise
healthy with no dysmorphic features and unremarkable
systemic examinations. His initial work-up revealed a serum
testosterone level of 0.087 nmol (normal 8.4-28.7 nmol/L),
while follicle-stimulating hormone (FSH) and luteinizing
hormone (LH) were 0.2 (normally less than 2). Bone age
according to Greulich and Pyle was that of a 7-year-old
(delayed for chronological age). He had no risk factors for
priapism: no history of trauma, sickle cell disease, or
malignancies.
The patient was prescribed intramuscular testosterone
injections of 50 mg, but received an accidently high dose of
500 mg by his mother who, as mentioned, is a paramedic
personnel. Two hours after the injection, he started to have
International Journal of Medical and Health Research
18
painless prolonged erections for six hours in duration, but
which resolved spontaneously and recurred again within
hours; he continued to experience multiple erections, with six
of them being more than 4 hours long (ranging from 6 to 12
hours), and accompanied by nocturnal erections. Both testicles
became engorged, with a feeling of heaviness, discomfort, and
darkness of scrotum color. His genital examination revealed a
stretched penile length of 6 cm [Figure 1], with each testicular
volume increased to 3 ml on both sides, along with pubic hair
starting to appear at stage 2.
Fig 1: Erected patient's penis with penile length of 6 cm.
Our investigations were done to monitor precocious puberty
(post high dose of testosterone) revealed that his serum
testosterone was 23.54 ng/dL, LH 0.29 IU/L and FSH 0.11
IU/L. Testicular ultrasound showed prepubertal testicles size
[Figure 2]. Complete blood count (CBC) and coagulation
profile were otherwise normal with no evidence of anemia,
polycythemia, thrombocytopenia, or thrombocytosis. Sickling
test was negative.
Fig 2: Ultrasound of testicles showed right testicles measures 1 x 0.8
x 1 cm and left testicle measures 1.4 x 0.8 x 0.8 cm.
There is no antidote for testosterone, so he was managed with
cold penile compresses to decrease blood flow, daily
monitoring of frequency and duration of erections, as a
surgical shunt was planned if erections became worse; his
erections were less frequent, however, along with shorter
duration over time.
Discussion
This case suggests a strong association between a high dose of
testosterone administration and development of priapism. This
patient did not have a previous history or risk factors for it.
Causes of priapism can involve sickle cell anemia, leukemia,
malignancies, and trauma. Drugs have also been considered,
but rarely has this been the situation. The World Health
Organization (WHO) Uppsala Monitoring Centre received
122 reports of priapism from 1968 to 1987, containing 138
suspected drugs, although testosterone was not one of them [8].
Testosterone hormone is important for penile erections.
Androgen receptors exist in large quantities in the immature
phallus, including the erectile tissue, but decrease with age
and present in low quantities in an adult phallus [19].
Androgens aid erection initiation by increasing nitric oxide
synthase expression and activity. Increased nitric oxide causes
cavernosal artery and sinusoidal smooth muscle relaxation, yet
it also enhances phosphodiesterase type-5 that works on
restoring the resting smooth muscle contractility [3]. The exact
mechanism by which testosterone administration causes
priapism is not clear yet.
In this reported case, priapism was obvious due to medication
administration error. The patient was given a high dose of
testosterone (500 mg) instead of the correct 50 mg by his
mother (a paramedic) who erroneously read the written
prescription. This is consistent with two other reported cases
who developed priapism after a high dose of testosterone due
to medication administration error. The first case was reported
by Zargooshi, in a patient with hypogonadotropic
hypogonadism, who had an increased dose from 250 to 500
mg. His conclusion was that testosterone-induced priapism is
dose-dependent [13]. Ichoka reported a second case of
testosterone-induced priapism in a patient with Klinefelter
syndrome. The patient was given the third injection by
mistake 12 days after the second injection, but the interval
between the two injections was too short. Accumulation of
testosterone enanthate, in addition to the supraphysiologic
local testosterone level, could be the cause of priapism. Ichoka
suspected that the sudden exposure to testosterone after a long
period of deprivation could have caused priapism, via an
increase in local sensitivity to testosterone [15]. This hypothesis
was supported by other cases in the literature as well [Table
1]. Shergill reported a case of priapism after testosterone
injection in a patient with Kallmann’s syndrome [10], while
Whalen reported two cases of priapism in patients receiving
gonadotropin-releasing hormone (GnRH) for
hypogonadotropic hypogonadism [9]. Zelissen reported a case
of testosterone-induced priapism in a patient with idiopathic
hypogonadotropic hypogonadism [8].
From previous reported cases, priapism can happen as a side
effect of testosterone therapy itself, especially in patients
deprived of testosterone, or as a complication of high doses.
The 21 cases found in a literature review [Table 1] found that
most of them were in adolescence between 12 and 23 years.
Four of them were above thirty, but no one was in the
International Journal of Medical and Health Research
19
childhood range, as was this indexed case. From these 21
patients, only one of them was documented to have
micropenis, although no one was taking testosterone for
isolated micropenis, as in this case. The onset of priapism
after testosterone administration ranged from 2 to 10 days. In
this patient, it started 2 hours after injection and continued for
6 hours, and resolved spontaneously; after that, he continued
to have night and day erections with multiple episodes of
priapism for one week until he sought medical advice.
To date, there is no antidote for testosterone overdose.
Regarding priapism management and medical evaluation, it is
recommended for any erection lasting more than 4 hours.
Management and prognosis varies among cases. Initial
management aims to determine the type of priapism and
achieve detumescence. With the current treatments available,
antiandrogens are seen as the optimal management of
testosterone-induced priapism. However, they are relatively
contraindicated in males who have not reached sexual or
skeletal maturation. Opiate analgesia is required in ischemic
priapism. Cold packs are also considered analgesic, and
causing vasoconstriction and decreasing penile blood flow as
that was effective in this indexed case. Aspiration of blood
from the corpus cavernosum, along with irrigation, is the
initial treatment in ischemic priapism. If not effective, an
intracorporal injection of phenylephrine in a small dose should
be performed. Last choice if the previous measures are not
effective is surgery, with distal or proximal shunts being
performed [6].
Follow-up of patients was not documented in most reports.
Key reported a case of priapism after a therapeutic dose of
testosterone for delayed puberty, who developed impotence
one year after puberty [5].
Conclusion
Although a relative of the patient may work in the medical
field, it is not advisable for that person to give the injection, as
frequent errors can happen as a function of an emotional
connection. For children, parents must be counseled before
testosterone administration about its side effects - e.g., pubic
hair appearance, frequent erections, and priapism, especially if
given in too high a dose. Monitoring is needed for early
puberty induction in these cases by regular examination of
testicular size and Tanner’s pubertal staging, in addition to
biochemical monitoring of pubertal hormones and ultrasound
monitoring of testicular size; this would naturally include
serum testosterone levels returning to normal for his age.
Table 1: Reported cases of testosterone induced priapism
Year
Authors
Agent used
Disease
Age of the
patient
Risk factors
Onset from
injections
Outcome
1940
Finkler20
Testosterone
propionate
Eunuchoid
12 year old
-
Not
mentioned
Resolved spontaneously
1970
Lundh12
Testosterone,
enanthate and
cypionate.
Sickle cell
Disease
3 patients:
20, 20 and
31 year old
Previous Priapism
before starting the
therapy.
Not
mentioned
All these cases needed
surgical intervention
1971
Shaldon18
Testosterone
as a mixture of
its ester
Renal Failure
2 patients,
age not
mentioned
Dialysis
Not
mentioned
Not mentioned
1977
Hartitzsch21
Testosterone
as a mixture of
its ester
Renal Failure
Not
mentioned
Dialysis
Not
mentioned
Not mentioned
1983
Landefeld22
Clomiphene
Sickle cell
Disease
19 year old
-
Not
mentioned
Resolved after drug
discontinuation
1987
Endres11
Testosterone
undecanoate
Fabry's disease
17 year old
Accumulation of
globotriaosytceramide
in autonomic ganglia.
At the 17th
day of
treatment
Cavernosoglandular
shunt had to be
performed.
1988
Zelissen8
Testosterone
enanthate
Idiopathic hypo-
gonadotropic
hypogonadism
20 year old
-
Three days
after third
injection
Cavernosum-spongiosum
shunt. Recurrent one day
later and a second shunt
operation was necessary.
1989
Ruch14
Testosterone
as a mixture of
its ester
Delayed Puberty
15 year old
-
7 Days after
first injection
Shunt operation was
planned. At induction of
anesthesia, penis
detumescence occurred.
1989
Key LL5
Testosterone
enanthate
Delayed Puberty
14 year old
Manipulation of the
erected penis
5 days after
first injection
Responded to irrigation
But one year later, after
puberty, he developed
impotence.
14 year old
& 13 year
old
-
Following the
initial dose
Responded to amyl
nitrite therapy.
International Journal of Medical and Health Research
20
Table 2: Reported cases of testosterone induced priapism
Year
Agent used
Disease
Age of the
patient
Risk factors
Onset from
injections
Outcome
1991
Gonadotropin
Releasing
Hormone
Hypogonadism
32 year old
-
-
Responded to injection of
1:100,000 epinephrine
solution followed by
aspiration
38 year old
-
-
Shunt procedure was
performed
1995
Testosterone
enanthate
Sickle cell
Disease
14 year old
-
7 Days after
first
injection
Respond to terbutaline +
topical nitropaste
13 year old
-
8 Days after
first
injection
Treated with aspiration
2000
Testosterone
enanthate
Hypogonadism
23 year old
The patient
increased the dose
Following
the fourth
injection
Corporeal glandular shunt
was performed
2003
Testosterone
ester
Kallmann’s
Syndrome
18 year old
-
10 Days
after first
injection
Treated with aspiration but
recurred within 24 hours. So,
shunt was performed
2005
Testosterone
Constitutional
delay of growth
and puberty
14 years old
-
2 Days after
second
injection
Treated by repeated aspiration
and irrigation with
phenylephrine
2005
Testosterone
enanthate
Klinefelter
syndrome
41 year old
The interval
between 2nd & 3rd
injection was short
8 Days after
third
injection
Treated by aspiration and
injection of adrenaline
2012
Testosterone
ester
Constitutional
delay of growth
and puberty
15 year old
-
7 Days after
first
injection
treated with insertion of
bilateral T-shunts. After 24
hour Bilateral mid corporal
corporotomies were
performed.
Sickle cell
Disease
14 year old
-
3 Days after
first
injection
Treated with hyper hydration
and analgesia.
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... Androgen replacement therapy has been occasionally reported to trigger priapism in the literature. The presumed mechanism is via testosterone-induced upregulation of nitric oxide (NO) synthase, which increases cavernosal artery and smooth muscle relaxation [44][45][46][47]. ...
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Priapism describes a persistent erection arising from dysfunction of mechanisms regulating penile tumescence, rigidity, and flaccidity. A correct diagnosis of priapism is a matter of urgency requiring identification of underlying hemodynamics. To define the types of priapism, address its pathogenesis and epidemiology, and develop an evidence-based guideline for effective management. Six experts from four countries developed a consensus document on priapism; this document was presented for peer review and debate in a public forum and revisions were made based on recommendations of chairpersons to the International Consultation on Sexual Medicine. This report focuses on guidelines written over the past decade and reviews the priapism literature from 2003 to 2009. Although the literature is predominantly case series, recent reports have more detailed methodology including duration of priapism, etiology of priapism, and erectile function outcomes. Consensus recommendations were based on evidence-based literature, best medical practices, and bench research. Basic science supporting current concepts in the pathophysiology of priapism, and clinical research supporting the most effective treatment strategies are summarized in this review. Prompt diagnosis and appropriate management of priapism are necessary to spare patients ineffective interventions and maximize erectile function outcomes. Future research is needed to understand corporal smooth muscle pathology associated with genetic and acquired conditions resulting in ischemic priapism. Better understanding of molecular mechanisms involved in the pathogenesis of stuttering ischemic priapism will offer new avenues for medical intervention. Documenting erectile function outcomes based on duration of ischemic priapism, time to interventions, and types of interventions is needed to establish evidence-based guidance. In contrast, pathogenesis of nonischemic priapism is understood, and largely attributable to trauma. Better documentation of onset of high-flow priapism in relation to time of injury, and response to conservative management vs. angiogroaphic or surgical interventions is needed to establish evidence-based guidance.
Anaemia is a common complication of chronic kidney disease. Of the various contributory factors, the most important is an inappropriately low circulating concentration of erythropoietin, a hormone largely produced by the peritubular cells of the kidney. Chronic anaemia causes debilitating symptoms, particularly if severe, including tiredness and lethargy, muscle fatigue, reduced exercise capacity and breathlessness on exertion. There are adverse consequences for a number of physiological systems, particularly those of the heart and brain. The anaemia of kidney disease tends to develop once the glomerular filtration rate falls below 60 ml/minute, and anaemic patients with a lesser degree of renal impairment should be screened carefully for another cause of anaemia. Synthetic erythropoietin therapy has transformed the management of renal anaemia; it induces an increase in haemoglobin concentration and prevents the need for repeated blood transfusions, particularly in dialysis patients. Many patients receiving erythropoietin therapy will require supplemental iron, and this is often given intravenously. Several new strategies for stimulating erythropoiesis are currently in development, including prolyl hydroxylase inhibition (HIF stabilizers), erythropoietin gene therapy, and modulation of hepcidin activity.
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• A brief course of testosterone injections is known to be an effective treatment for boys with constitutional delayed puberty. In this study, data from seven boys at least 14 years old who received testosterone enanthate (100 mg intramuscularly monthly for four months) were analyzed to see if linear and testicular growth responses could be useful diagnostically in excluding growth hormone deficiency (GHD) and isolated gonadotropin deficiency, two conditions that are often difficult to distinguish from constitutional delayed puberty. During four months of testosterone therapy, growth rate increased from 4.0±1.0 cm/y to 10.7±2.3 cm/y, and was greater than 8 cm/y in all patients. Since testosterone-induced stimulation of linear growth is largely GH-mediated, the large increase in growth rate in all boys is considered indicative of GH sufficiency. Testis length, which did not increase during testosterone therapy, increased by 0.6 to 0.8 cm in every patient (from 2.7 ± 0.3 cm to 3.4 ± 0.4 cm) over the following four months, indicating normal gonadotropin secretion and normal pubertal progression; in contrast, the increase in serum testosterone concentrations after discontinuation of testosterone treatment was more variable. It is concluded that the growth response to a four-month course of testosterone is helpful in excluding GHD in boys with delayed puberty, and an additional four months of follow-up is sufficient to document the onset of puberty, thereby eliminating the possibility of isolated gonadotropin deficiency. (AJDC 1989;143:116-120)