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DATE COMPLETED__________
Endocrine Physiology
Case Study -- Bathsheba
Background
A few years ago when I was teaching endocrine physiology to biology majors here at
USD one of the students came up to me with her case as example of how the endocrine
system works. Subsequently she came and talked with me about her life and the
consequences of the situation that she developed as a young child.
Situation
At age 12, I appeared to be a normal healthy child. In May of 1997, at my yearly
physical, my family doctor noticed that the chart representing my growth was quite
unusual. In fact, it showed no growth, in regards to height and weight, in the last sixteen
months. The doctor also noted that unlike my classmates, I did not exhibit any signs of
pre-puberty or puberty. These symptoms cued him to perform several blood tests. An
IGF-1 (evaluates pituitary gland function), free T4 & TI3 (thyroid hormone levels), and a
blood estrogen level were all taken. The IGF-1 level came back as 0.21 m-IU/L (normal
range 0.5 – 5.0 m-IU/L). This dramatically low test result alone could indicate endocrine
trouble, but coupled with the free T4 level of 0.3 (normal range 0.8-1.9) is indicative of
major loss of pituitary function. Upon seeing these results, my doctor referred me to a
pediatric endocrinologist who ordered repeat series on all the blood tests and found
them to be even lower than previously recorded. This prompted him to order a cranial
MRI with contrast to look at the pituitary gland. In doing the scan, he hoped to rule out
several possibilities. At that point, the possibilities ranged from simple lack of endocrine
function not based on anatomy to pituitary deformity to a tumor, be it benign or
malignant. A stage four tumor essentially encapsulating the pituitary gland was found.
1. How could this condition affect growth and development in Bathsheba?
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The medical staff needed to determine whether the hypopituitaryism was malignant or
benign. The doctor entered the skull through a small hole in the right parietal lobe to
take a biopsy of the tumor through a two and a half inch incision. The frozen sections
were examined in pathology and the tumor was identified as CNS Germinoma (very
uncommon accounting for about 0.052% of cancers), and normally such tumors arise in
the gonads or ovaries. Germ cell tumors, by definition, arise while in-utero, so this tumor
in theory had had twelve years to grow. In surgery, it was discovered that the tumor
was inoperable, taking that treatment option away. Other therapies would have to be
used quickly so high dose chemotherapy was chosen. After six cycles of chemotherapy
over the five months, the chemotherapy drugs not only did a fantastic job at destroying
the cancer, but they were devastating to my immune system. I was given eighteen
blood transfusions throughout. While radiating the remainder of the tumor, they
“accidentally” radiated my pituitary and thyroid glands as well, turning them into (as the
doctor put it) raisins. However, all of the therapy resulted in complete remission of the
cancer.
2. What would Bathsheba’s hypothyroidism do to her? How could she be treated for
low thyroid hormone?
3. What would Bathsheba’s hypocortisolism do to her? Would it be dangerous for
Bathsheba to be stressed?
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4. Is it possible to help Bathsheba grow more normally and go into puberty? How
would one do that?
5. If Bathsheba were your patient, what would you advise her about getting pregnant
and nursing an infant?
6. If artificial hormones were not available to Bathsheba, what would be her levels of
hypothalamic hormones? If too many artificial hormones were given to Bathsheba,
what would be her levels of hypothalamic hormones?
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7. Which hormones would Bathsheba be able to stop taking as she matures?
Don’t forget to add the posterior pituitary hormones vasopressin and oxytocin.
Hypothalamic Pituitary Axis Image
A version may be found in Silverthorn, Figure 7.9 from Human Physiology: An
Integrated Approach, sixth edition, from Pearson, copyright 2013.
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Please submit answers only, typed on a new sheet.
Thank you!
Special thanks to Bathsheba for sharing her story with future physiology students.
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APPENDIX: MEDICAL BACKGROUND FOR BATHSHEBA
At age 12, I appeared to be a normal healthy child. In May of 1997, at my yearly physical, my
family doctor noticed that the chart representing my growth was quite unusual. In fact, it showed no
growth, in regards to height and weight, in the last sixteen months. The doctor was, of course, bothered by
this. He also noted that unlike my classmates, I did not exhibit any signs of pre-puberty nor puberty.
These symptoms cued him to perform several blood tests. An IGF-1 (evaluates pituitary gland function),
Free T4 & T3 (thyroid hormone levels), and a blood estrogen level were all taken. The IGF-1 level came
back as 0.21 m-IU/L (normal range 0.5 – 5.0 m-IU/L). This dramatically low test result alone
could indicate endocrine trouble, but coupled with the Free T4 level of 0.3 (normal range 0.81.9) is indicative of major loss of pituitary function. Upon seeing these results, the doctor
realized that he, as a general family physician, was way over his head. He referred me to a
Pediatric Endocrinologist who happened to be running a clinic in my hometown within the next
month. The endocrinologist was duly concerned with the blood tests. He ordered a repeat series
on all and found them to be even lower than previously recorded. This prompted him to order a
cranial MRI with contrast to get a look at the pituitary gland. In doing the scan, he hoped to rule
out several possibilities. At that point, the possibilities ranged from simple lack of endocrine
function not based on anatomy to pituitary deformity to a tumor, be it benign or malignant. The
scan was done immediately and revealed what we had feared. A stage four tumor extending from
the sella turcica to the third ventricle (which is bound by the thalamus, the hypothalamus, and the
lamina terminalis) was discovered. The tumor had essentially encapsulated my pituitary gland,
explaining the lack of growth.
Now that the hypopituitaryism was no longer idiopathic, we had to determine if the tumor
was malignant or benign. An acclaimed neurosurgeon was called immediately and flew to Sioux
Falls from Minneapolis as I was transported to the hospital in Sioux Falls. After being checked in
and completing a creatine clearance, I was sedated. For the next five hours, a steriotactic biopsy
was performed. The doctor entered the skull through the right parietal lobe, as it allowed the least
invasive sampling of the tumor, through a two and a half inch incision. Three frozen sections
from the biopsy were examined in pathology and the tumor was identified as CNS Germinoma.
Germinoma is a type of cancer that is uncommon unto itself (accounting for only about 0.052%
of cancers), but normally such tumors arise in the gonads or ovaries. Germ cell tumors, by
definition, arise while in-utero, so this tumor in theory had twelve years to grow.
In surgery, it was discovered that the tumor was inoperable, taking that treatment option
away. Other therapies would have to be used, and quickly. Luckily, a specialist on brain
germinomas who happened to be working at the Sioux Falls hospital was called in. After
discussing our options on how to quickly destroy the tumor, high dose chemotherapy was
decided upon.
I underwent six cycles of chemotherapy over the next five months. Cisplatinin and VP-16
were the chemo drugs administered intravenously in the first three chemotherapy sessions. While
doing a fantastic job at destroying the cancer, they were devastating to my immune system. I was
given eighteen blood transfusions throughout. The time pattern for therapy was: one week of
chemo, three weeks off, one week of chemo, three weeks off, etc. Because of how harsh the
therapies were and because we had already reduced the tumor to 30% of its original size by the
third round of chemo, Carboplatnin (an experimental drug at the time) replaced Cisplatinin in my
cocktail for the last three sessions. A month was taken off to allow my body to reboot and restore
blood counts. After this, thirty cycles of radiation therapy and brain boost radiation were
administered at the University of Minnesota Hospital in St. Paul. While radiating the remainder
of the tumor, they “accidentally” radiated my pituitary and thyroid glands as well, turning them
into (as the doctor put it) raisins. However, all of the therapy resulted in complete remission of
the cancer.
The aftermath of cancer and therapy is: hypopituitaryism, hypothyroidism, diabetes
insipidus, and a menopause like state of estrogen levels. Hormone replacement had to wait for
about two years because of the chance of recurrence. (It’s not a good idea to provide a tumor
with hormones.) When the time came to begin hormone replacement, I was a sixteen-year-old in
the body of a twelve-year-old. Anti-diuretic hormone replacement had begun just after finishing
radiation therapy, as it didn’t pose a tumor boosting danger. Thyroid hormone replacement and
growth hormone injections were next to come. Synthroid was the thyroid hormone replacement
and Nutropin AQ was the growth hormone of choice because it provided the highest amount of
raw growth hormone without added testosterones or estrogens, both of which would age my
growth plates minimizing the growth we could achieve. This bone aging is also the reason that
estrogen therapy wasn’t begun for another year following the HGH and thyroid replacement.
When the time came, we began with estrogens similar to what a woman would produce during
her non-menstruating weeks. These were given for fourteen months prior to putting me on
cycling hormones. The results of all this exciting therapy were: fourteen inches of height
increase, and puberty! Thank you artificial endocrine hormones!