Endocrinol Japon

1992, 39 (2), 165-167

Gradual Progress of ACTH Deficiency in a Child with Panhypopiturtansm Associated with Pituitary Stalk Transection YUKIHIROHASEGAWA, TOMONOBUHASEGAWA, TETSUOYOKOYAMA, SHINOBUKOTOH, AND YUTAKATSUCHIYA DivisionofPediatricEndocrinologyand Metabolism,Tokyo MetropolitanKiyoseChildren'sHospital,Tokyo204, Japan

Abstract.

We present

and gradual insufficiency

here a 13-year-old

male with hypopituitarism

progress of ACTH deficiency. ACTH at the age of 12 years and 7 months.

gradual progress has been proved by not 13 years. The cause of panhypopituitarism

or at the delivery crisis,

because

his laboratory

results

only the laboratory including ACTH

of the stalk transection suggested

that

which accompanied

results but also the deficiency is thought

seen on the magnetic

he had

an insidious

deficiency finally led to an overt crisis of adrenal This patient is unique because the insidious and

secondary

adrenal

clinical course for over to have existed before

resonance

image (MRI). At the

insufficiency,

whereas

he showed

normal adrenal function proved by the insulin tolerance test (ITT) at the age of 4 years. Abrupt crisis of secondary

adrenal

insufficiency

developed

at the

age of

12 years,

although

he had

been

well until

the

crisis.

Key words: Hypopituitarism,

Transection

of pituitary

stalk,

Gradual

progress

of ACTH

deficiency.

(Endocrinol Japon 39: 165-167,

GRADUAL

LOSS

of

GH

tected in GH stimulation The

negative

correlation

hormone

deficiency

of growth

hormone

[3] suggests with age 13-year-old

in

GH

secreting

ability,

in patients

(GHD) between releasing

secretion

with

and

gradual

growth

the GH peak

factor

test and

decreasing

patients with GHD. We male with hypopituitarism

was seen insidious deficiency.

de-

Case

tests, is well known [1, 2].

progress

age

gradually present a in whom of ACTH

1992)

Report

This male patient was the 2.9kg product of a 39-week uncomplicated gestation. He was born by breech delivery. Allegedly, he had asphyxia and needed he was

to be resuscitated. referred to our

stature

(85.5cm,

been

otherwise

M-4.5

a micropenis

typical

face

TSH

for severe

deficiency,

but had

examination

length,

growth

based

during

Physical

(penile

We diagnosed

(2.0ng/ml)

SD; 11.3kg),

healthy.

vealed (GHD).

At the age of 4 years, clinic because of short re-

1.5cm)

hormone

deficiency

him as having

GHD

upon

GH

ITT

and

low peak

(0.1U/kg

and levels

intravenous

injection) and a low basal TSH and thyroxine level Received:

November

1, 1991

Accepted: January

29, 1992

Correspondence

Pediatric Kiyose

to:

Endocrinology Children's

204, Japan.

Dr.

(2.1ƒÊU/ml,

ACTH

Yukihiro

HASEGAWA,

and Metabolism,

Hospital,

1-3-1

Tokyo

Umezono,

Division

Metropolitan Kiyose,

Tokyo

of

3.6ƒÊg/dl,

and

mal (Table after

cortisol

respectively).

levels

during

At

ITT

this

1). He showed signs of catch-up

treatment

with

ment (123.5 cm, M-2.7

GH

and

thyroxine

point,

were

nor-

growth supple-

SD at the age of 11 years).

66

HASEGAWA

Table

1.

Serial

tests

for

adrenal

function

ITT: insulin tolerance test (0.1U/kg the age of 4 years was measured Table were standarized to the and

IRMA

hospital

(IRMA

respectively): Cortisol peak

during

during ITT,

and

peak

during

ACTH

test,

ITT,

(n=10,

age of 11 years,

(MRI)

range;

revealed

magnetic

pituitary

resonance

with lobe. to our of a

stalk

range;

20.7-76.2ƒÊg/dl),

46-663ƒÊg/ml);

and

values

(peak

testosterone;

few

of

shot

absolutely

asymptomatic

some

minor

stress

virus-like syndromes. His life had been uneventful until

he

had

probable

adrenal

i.v. infusion

100mg

for

ƒÊg /ml

and

10h)

were

his He

results

before

the

with ACTH

deficiency.

(before

treatment)

the

glucocorti-

We cannot explain the absence of hypoglycemia, by which a crisis of adrenal insufficiency is typically

insufficiency and had

7 a

On the day of the could not take the

accompanied.

performed T, blood

Two

weeks

sampling

before

previously-prescribed glucocorticoid. On arrival at our hospital, he was conscious, but complaining of light-headedness. On physical examination, his

he started

ment

(hydrocortisone,

face

age

pale,

his

he recovered,

ACTH and

we

test (ACTH-

8 h after

40U

i.m.

injection of cortrosin-Z), and ITT that confirmed adrenal

were

after

an intramuscular

tests,

membranes

and

completely.

respectively)

compatible

laboratory

100

infusion

started,

almost

and

(hydrocortisone) for 1 day. and serum cortisol levels (13

these

mucous

(hydrocortisone, were

14.2 ƒÊg/dl,

treatment

saline

by continuous

recovered

secondary

and

we he

blood glucose, 99mg/dl. The levels of BUN and Cr suggest mild dehydration with hyponatremia.

of common cold at the age of 12 years months. The day before the crisis he

skin

by

were the following: total protein, 6.8mg/dl; blood urea nitrogen, 30mg/dl, serum creatinine, 1.1 mg /dl; serum Na, 130 mEq/l, serum K, 3.9 mEq/l;

until

convulsion) during an episode

headache and he was febrile. crisis, he began to vomit and

pressure

as febrile,

such

without

blood

physiological

i.v., followed

consciousness

0.3

this time. Urinary 17-OHCS levels were low (Table 1). We recommended him to take glucocorticoid

crisis (hypotension,

as

and

after

Other

coid

shown

was about 55mmHg. While the physical examination,

minutes

mg one

deficiency (Table 1). It was subclinical,

he had

17-OHCS, are

cold,

ACTH

when

ug/dl);

began to lose visual acuity and a generalized clonic seizure developed and continued for 20 seconds.

glucocorticoid

(LH peak;