IDIOPATHIC HYPOPARATHYROIDISM WITH BONY DEMINERALIZATION AND CARDIAC DECOMPENSATION Jerome L.

Schulman and Harold Ratner Pediatrics 1955;16;848-856

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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1948. PEDIATRICS is owned, published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright 1955 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.

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IDIOPATHIC HYPOPARATHYROIDISM DEMINERALIZATION AND DECOM PENSATION

By Jerome
L. Schulman, M.D.,* and
ing about

WITH CARDIAC
Ratner, minutes. episodes mumps the was ill with M.D. There

BONY

Harold 15

I
This

HYPOPARATHYIIOIDLSM

the normal so constant

bones
examination.

char-

were of diplopia

at

this and

time 1 of a

acteristicahly
density on

exhibit
roentgenographic

or increased that many

also

2 or 3 brief from

finding

has

been

aphasia. Recovery

was

followed

after

authors for the had the

regard it as an essential criterion diagnosis of the disease. We have opportunity to observe a patient in diagnosis seemed except of the entire of idiopathic hypoto be substantiated that a marked skeleton the was deminpresunusual without

few weeks by Nothing further came diarrhea day the and this pearance morning brought There
testinal

development noted until nausea, to was

of alopecia. the patient bevomiting, and The next to 38.3#{176}C. by the On apthe

acutely

whom the parathyroidism on all grounds eralization

5 days prior temperature

admission. elevated 24 hours respirations. in of diet

was followed after of rapid, labored of admission to the hospital was

disorder

she

ent. The patient feature of cardiac

also presented decompensation

collapsed and was a shock-like state. chronic was gastroinreferable

no tract. The 600 No

history
nor of

evidence of organic heart disease. It is behieved that the cardiac decompensation was related to the hypocalcemia. Evidence in support of this contention will be presented.

disturbance

to the all
minimum

urinary
of

The patient ml. of facts

adequate consumed fortified elicited illness. with which

in a

respects. D. have

daily milk were

vitamin might

other

CASE REPORT
D. W.,
to the 19, rhea breathing She
when,

a bearing

on the

present

The
was time rapid, January, admitted on June of diarlabored 1952, 40 been years in

father,
old, good

41
both health. There

years
of was There

old,
Jewish

and
had

the
been

mother,
had no other of

a 12-year-old Hospital with 5 days of 3 days apparently the duration

white for chief duration. well weeks the

girl, first complaints and until later

extraction, family history

Jewish of was
at

1953,

pregnancies. convulsive

no

disorder.

On
critically well

admission
ill. nourished; She

the
was the

patient
fairly vell temperature

appeared
developed was Pulse She to simple and alopecia of

to

be
and

the

age
Three

of 103 years, and had

this, and 4 convulsive occurred,

she
she

became
developed

ill

38.3#{176}C., and apcommarked of the

with

pertussis.

a transient
sive episodes. therapy with started. a rather

hemiparesis
Following phenobarbital nature

2 brief

convul-

and the respirations blood pressure were peared mands. diaphoresis. stuporous There was Definite but

were 68/mm. unobtainable. responded some areas cyanosis

anticonvulsant Tridione#{174} was seizures the last of in

Subsequently bizarre

March,
results time

1953.
were both

An electroencephalogram
examination been normal. said to have of mild

and

the
One

scalp were noted and tufts be pulled out. Examination strated of the ately heart mal. below There sponse bilateral retinal injected. at The the was to this edge costal papilledema vessels. The time of the was margin The lungs liver

of hair could easily of the fundi demonwith pharynx were clear to palpable the right and patient no narrowing was moderand be

of an ophthalmologic

at this

month
showed

later
evidence

a in

second April,
second

electroencephalogram
epilepsy. Shortly

the
nor3 cm. side. rewas,

considered was on

thereafter,
mumps. On

1953,
day

she
of the

developed
illness her

the

temperature rose to 38.9#{176}C. and she plained of headache, became lethargic, suffered 3 attacks of carpal spasm, each
From Hospital
0

comand lastJewish

generalized areflexia, painful stimuli. The

however,
tarily.

able was
for

to move withdrawn
chemical were

all 4 extremities at
analyses. as

volunof
results sugar

the Department of Brooklyn,

ADDRESS:

of Pediatrics, Brooklyn, New

The Johns Hopkins

The York.

Blood
mission the

the
The follows:

time

adof 186

PRESENT

Hospital,

determinations

Baltimore,

Maryland.
848

mg./100

ml.;

sodium

125,

potassium

5.13,

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CLINICAL
carbon chloride The tomaticahly travenous antibiotics.
sure rose to

NOTES
hospital presence
sinus

849 day yielded with showed the a rate low first suggestion was The of the a regular

dioxide 91 mEq./l. initial state with fluids, Under

normal

combining of shock

power was

15.0, treated and blood patient

and sympof inwith presbe-

of hypocalcemia. rhythm

There of 97/mill. voltage.

The

QRS

the administration including plasma, this regimen

levels

complexes

the tile

terval measured measured 0.38

0.20 sec.

sec.

0.18 sec. The Q-T The S-T segment measured

with a normal value

P-R ininterval of pre-

and A

as compared

came clear action,

and

more fluid and chlorides.

responsive. with no normal

cells, values

spinal negative for

tap yielded Pandy reprotein, sugar,

0.106
changes cordial
tion.

sec.
leads

There
RS-T provided

were
and
00

no

other
additional

remarkable
and tile informa-

in the

T waves

A roentgenogram
after markedly reaching effusion zation admission ported frequency
4

of the
to
tile

chest

the

day
a

On
stek time of

the
sign

fourth
was
of

hospital
elicited
calcium

day
for
in

admission

hospital

revealed

the and

a positive Chvofirst time. The

enlarged heart with the left almost to the lateral rib cage. was was as 5/sec.
was

border Pleural Digitali-

concelitratiOn

the
the 6.1

serum
mg.

was

3.9

mg.,/100 phosphorus

ml.

at that concentration /100 ml.

present begun. On

on the the

right second

side. day

inorganic

following resome

Other
same

chemical
time showed

determinations
the total protein sodium carbon of the

done
to

at
be

the
7.0

an electroencephalogram showing waves with of 2 to 3/sec. activity no significant from occurring

was a predominant with intermittently.

all areas

gm.,/100 ml., 4.21 mEq.,/l., 21.9 the mEq., remainder

131 mEq./l., potassium dioxide combining power 89 40 mEq./l. ml. of During a 10 were hospital per adday day

to

I. and

chloride

There tude focal spike seen. dence ing An

asymmetry in nor the

of amphiwas there a tracings. No

from area

either hemisphere of abnormality

potentials nor The impression of a diffuse both hemispheres. electrocardiogram

paroxysmal was that

activity were there was cviaffecton the third

cent solution of calcium ministered intravenously. On the morning of the 3.6 twenty concentratioll mg./100 was ml. ml. 4.3

gluconate the fifth

electrical also

disturbance taken

of calcium and that mg.lOO

cent

in the serum was of inorganic phosml. solution One hundred of calcium

1,horus

of a 10 per

FIG.

1. Roentgenogranis
B (Right).

of

the After

shoulder. 10 months

A (Left). Before therapy, showing marked of treatment, showing partial recalcification.

deniineraiization

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850

SCHULMAN

IDIOPATHIC

HYPOPARATHYROIDISM

11G.

2.

Roe

tgcnogranis

alization.
showing

Arrows
I)artial

of the lumbar spine. A (Left). indicate widening of the intervertebral recalcification.

Before spaces.

therapy, B (Right).

demonstrating marked deminerAfter 10 months of treatment,

gluconate was 6 gm. of calcium thera1w

an(1 the
tile

administered chloride

intravenously orally. In spite remained marked survey 2A) was was

and of this positive carpal of the obtained

A gastrointestinal
small amounts fat cent stool citric were bowel of fat concentrations of dry weight collections. acid tolerance, normal
many examinations
OIl

study
pattern.

revealed
Excretion of

a normal
normal

Chvostek
patient

sign developed

was of

indicated 2.5 per respectively Tests of and limits.

of

by cent

the and

finding 3.6

of per

spasm. The
osseous

first
system

roentgenographic (Figs. 1A and

in two 24-hour glucose tolerance, vitamin the urine Normal

of

A tolerance gave the of norvalues blood normal

on the fifth
tion of the of
ill

hospital
entire several
both

day.
bony of
lenses

Marked
dorsal of diffusely of

demineralizanoted showed scattered, variable lenses size. opacities for with vertebrae. the

all within results urea

structure the examination

Routine
mal for were pvelogram

collapse presence sui)capsular, There extending variable

present

occasions.
concentration

Ophthalmoscopic whitish were incipient from distances

both
ill

nitrogen

repeatedly outlined

obtained. a urinary

An tract

intravenous

deposits periphery

wedge-shaped of the

the

configuration. In order dence of normal renal

to obtain further cvifunction an ammonium

toward the the anterior cortex. were to respond of the

center. They were and the posterior fine,

beneath

portions vacuolar posterior The injections strated (Fig. 3).

of the opacities capsule. ability of by means

Diffuse, found to extract Ellsworth-Howard the

white the

chloride loading test was performed. Control determinations of urinary excretion of electrolytes were followed by the oral administration of 300 mEq. After
of

of the of the

ammonium tile arc of acidic were therapy shown urine test

chloride and again

every basic determined. I. as

24 com-

hours. intravenous was demontest1

ponents

3 days

parathyroid

The values

results The entire for

in Table as well phosphorus,

course calcium,

the and

inorganic

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CLINICAL
Io

NOTES
of calcium had not risen appreciably above

851
the

ML
Parathormone
intraveouslq

80

initial level in spite of intensive treatment with calcium gluconate administered intravenously. At this point treatment with parathyroid cxtract and vitamin D was begun. After 14 days of therapy 600,000 units of vitamin D (given
as Drisdol#{174}) and had been given. 10.0 ml. During ml. and of parathyroid this period the extract con-

70

M G.

P
H

60

centration
to 5.7

of calcium
mg./100

in the
the

serum

rose

slowly
of

concentration

0 S

SO

inorganic mg./100
abated diminished. and

phosphorus to ml. The tetanic

the signs

a maximum symptoms

of 9.6 gradually

P
H

of cardiac day of

decompensation hospitalization

0 R U S

On
30

the

twenty-fourth

a brief course of treatment with dihydrotachysterol was started. The child was given 3 ml. of after phorus this the in material last the dose, serum daily the for level had 4 risen days. to Five a value days phosof of inorganic

20

10-rh J

2.
TIME

.3
IN

4
HOURS

FIG.

showing lowing extract. alkaline urinary

3. Results of the Ellsworth-Howard test the increased excretion of phosphorus folintravenous administration of parathyroid

11.4 mg./100 ml. The level of calcium was not appreciably affected. On the forty-eighth day of hospitalization she was placed on a regimen consisting of a diet
high in calcium
and low in phosphorus sup-

phosphatase Sulkowitch

in reaction

the are

serum, shown

and

the

phemented by a daily oral intake of 200,000 units of vitamin D and 1.5 gm. of calcium lactate. When this was instituted the serum level of calcium was 6.4 mg./100 ml. and the
level of inorganic phosphorus 8.2 ml. mg./100 but the ml.

4. On
had

the

ninth
carpal

hospital
spasm

day
and

the
the

in Figure patient still

serum level

Three
phorus

days
rose

later
to 9.8

the

level

of inorganic

phoslevel

marked

mg./100

TABLE
AMMONIIM CHLORIDE

I
LOADING TEST*

Daily Before A rnmonium

Urinary

Excretion After
A mmonium Before

1Th)od

Plasnia

Values
lfter

Ammonium (liloride

lmmoninm Chloride

Chloride
\olulue Titrable acidity 320 U24 .8 ml. mEq.

(hloride
1400

ml. 7.35
mg. mg.
mg.

pH

6.49 7.4 265

mg.

4.94
217 I 120
1795

7.2.5 ml.
1111.

Calcium Phosphorous
Sodium

mg. ing.

5.9 mg./100 0 .5 mg./100

131

6.5

nig./l00

378
1250
31

mEq./l.
mEq./I.

Potassium Chloride
Ammonia

rug.
ruEq.

3225 241

mg.
mEq.
mEq.

4 .49
99

inEq./I. mEq./I.
of

9 . 0 mg./Io0 130 niEq./l. 3 .0 mEq./I. 94 .5 inEq./1.

19.9 mEq./1. the

lid. 1111.

31 mEq.

L51

Carbon
*

dioxide
chloride,
during 300 mEq./24 period hours, was
was

22.1

Amnioniuni

administered
with that

for a period
found during

S days,
a control

following period.

which

excretion

of

substances

a 24-hour

compared

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852

SCHULMAN
$2

IDIOPATHIC

HYPOPARATHYROIDISM

CALCIUM

M0%

pHospIiog.us
M&% 8

ALKALINE

PHO.SP1-$ATASE
44

URINARY

5ULIWWITCh

TREATMrNT

aoc:E:J
.TL-

r-_.
OJOOL

IL.__..J 1,
o&Isoo_

40

20

50

40

30

To

8b DAY AFTER D%SCHARGE

phospilorns, alka1in

HO5PITAL
FIG.

DAY
values for serum calcinm,

4.

Course

of

the

patient

silowing

serum

inorganic

phosphatase of 45 the calcium ml. serum per

(in Bodansky remained day, the reached was When

units), unchanged. thereupon

and

urinary

Suikowitcll and

reaction vitamin

in relation A, and the

to various results were

forms of various all within

of therapy. cheminormal

Amphogel#{174}, added mg./100 to the in ml., of calcium

cal tests

of liver

function

therapy.

concentration a value of 7.8

limits. Amounts the urine were Urinary estrogen

of 17-ketosteroids 2.6 and 1.96 determinations

excreted in mg./24 hours. were negative.

the
tive

urinary
for

the

Sulkowitch first time.

reaction Vitamin

became posiD administra8

The
a short hair has cium in the nails
nails

alopecia
time since after

ceased
grown

to be progressive
of therapy As the level the proximal the nail beds have is no evidence in the bones. from back.

within
and the of calends of and new remained presthe hos-

tion
units

was discontinued after had been administered Three days after

a total of 1,500,000 in a period of of the vitamin

institution rose, from

days.

omission

supplement reached
the

the level

concentration of of 11.0 mg./100

of inorganic

calcium ml. and

the serum separated in. but the was time of calcium The

grew At there

cataracts of discharge

concentration

phosphorus

unchanged
cot.

papilledema no definite

longer

dropped to 6.5 mg./100 ml. Coincident with improvement in the serum calcium an electroencephalogram 1 month after admission demonstrated
turn of normal wave patterns.

level of about a re-

pital

of increased

deposition

The
1953, days.
was

child
after An
observed

was
a

discharged
of diet and
out-patient tile

on
was her

September
of without course given further

5,
79

The

similarly returned
gram, on

found gradually the of risen

roentgenographically to normal. The other the to Q-T normal hand, interval levels.

heart was to have ehectrocardiothe abcal-

period
vitamins in

hospitalization

unrestricted

supplementary

despite

department.

sence
cium

of clinical
had

symptoms,

showed
until

persistent
the

The

serum

prolongation

reached after action (given At this

level of calcium gradually fell and a low point of 5.0 mg./100 ml. 42 days The became Ertron#{174}) 120 the mi/day effect was urinary negative. 50,000 were not Sulkowitch Vitamm units/day again satisfactory started. and reD and

discharge. again as time

After
consistently

the

initial
electrolytes

episode normal
amino

the limits.
acids,

concentrations
serum total remained

of all of the levels

in the

within
of magnesium,

The

serum
protein

Amphogel#{174}

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CLINICAL
on
tile

NOTES

853

the

eighty-fourth
of vitamin

day

following

admission increased serum to cal-

dosage

D
effect During content levels

was

100,000
cium

units/day. level

The

on the this of
and

inorganic remained of alkaline for

January

was minimal. phosphorus at rather phosphatase the first 29, the time. 1954, vitamin high

period the the serum the

content

became 147 D

significantly leaving was condosage increased to of 200,000

elevated

On
the
taming

hospital,

days after preparation

changed was to

to a special aqueous dispersion 100,000 units/mI. * The initial units/day. 167,000, This and was finally later

100,000 133,000,

ullits/day. the serum

and
wise

The concentration remained persistently urinary Sulkowitch negative. asymptomatic Since discharge gained 5). work the in the severely lB has evidence vertebral affected and 2B). weight been and periods Menstrual roentgenograms bodies, portions The

calcium in at low levels was

patient

the

reaction except from appears

likeIlas

consistently completely difficulty. she school Recently definite has (Fig.

been visual pital robust and strated

for her the hosmore

begun

have have which of the

satisfactory. demonmost were skele-

of recalcification,

apparent the most ton (Figs.

DISCUSSION
There establishes roidism. of tions a Ilumber together The of hypocalcemia with is no the pathognomonic diagnosis classification requires associated evaluation of of clinical of finding hypoparathya given manifestaappropricase of which

consideration

ate
erally

laboratory
employed

data.
for are low by renal

The
the
those

criteria
diagnosis
of

most
of

genhypoet a!. high the en-

)1ratl1yr0idism which include, serum hone absence inorganic texture of

Drake
and and Other levels,

serum roentgenogram, insufficiency.

calcium

phosphorous

normal

teria
include

which
absence

have
of absence and

been
cllronic of

added
rachitic response

to

this

hist
in

gastrointestinal changes to parathv-

disturbance,
tile

bones, extract.

normal

roid

In
our

attempting 1)atient we
known to

to arrive at a diagnosis reviewed the conditions

cause hypocalcemia. Inc.

in that
The
FIG. IflOllths

are
a

Supplied

by Endo

Products,

time

5. Photograph after discharge she was clinically

of the from the well.

patient hospital.

taken At

S this

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854 absence renal of conditions

SCHULMAN base-losing was nephritis shown,

IDIOPATHIC and other a con-

HYPOPARATHYROIDISM deminerahization ated diagnoses, not conclusive,

genographic

first,

by

sistently negative Sulkowitch reaction in the urine during the period before therapy was instituted. Further evidence against a renal test origin the results renal
of

because laboratory incomplete

studies, and

of other associresults which are reports of roentother factors.

However,
is at least

they
tile

serve
possibility

to indicate
of with

that
association

there
of

for

the

condition

was chloride demonstrated of

obtained loading satand patient concomichanges

l)y means
isfactory
acidification

of an ammonium of which synthesis the

hypoparathyroidism

demineralization

of bone. Of
tion instances

ammonia The

considerable
is the fact of demineralization that

interest
there are

in
now

this

connec-

urine.

2 reported

furthermore tant urinary found there

aly.

showed none of the or blood chemical

pseudohypoparathyroidism of the

with
In each

with chronic renal disease, nor was evidence of an anatomical renal anomSprue, or a sprue-hike syndrome, was

the

diagnosis The

was abnormal
In

established of response
is similar the to former

by the to parain
to that there

demonstration thyroid
of is

of a lack

extract.

metabolism

ruled
of fecal

out
fat

by the
on

finding

of a normal
a normal

content
vitamin

pseudohypoparathyroidism hypoparathyroidism. an inability and in to respond

2 occasions,

A absorption

curve,

as well small studies.

as by bowel Rickets,

the pattern either

presin of by of in-

parathyroid

ence of a normal roentgenographic the the simple type resistant due or of the organic value absence rachitic long last mated
response

hormone

the of

later the bony

there hormone. why our

is

de-

creased exhibited rather

density

production generalized than

found

to vitamin type, was of an elevated

D deficiency eliminated level

In attempting the
ill

to explain normal the

of

patient bone

demineralization or increased of cases struck by

which

demonstration

phosphorus in the serum, a normal for alkaline phosphatase, and the of roentgenographic evidence of changes at the ends of the growing was of
administration

vast

of
the

hypoparathyroidism
extreme degree

majority we were cases

Steinberg level lowest

hypocalcemia by in value

was
only 3.6

present.
1 had mg./100

In
collected a serum ml.

the

52

of hypopara1952 as reas low

bones. Pseudohypoparathyroidism, possibility to be considered, by the

following

the elimof

thyroidism

calcium The next

demonstration
the

a normal

corded
determinations

was patient

4.2 were

parathyroid The fact usual criteria hypoparathyroidism bones to the

disease.

extract. that for our the density of

and

of the

mg./100 serum 3.9 there and was the of

These lowest

ml. The first level of calcium 3.6 mg./100 in addition first 23.8,
may

patient diagnosis except lends

fulfils

all

of the

in our

ml. an

of idiopathic that requiring some interest in

mention

As a consequence,
extraordinarily low

calcium-phosphorous

of normal question
Albright

product.
ations of

The gave
respectively. with

values products
the

for
and

3 determinin the

demineralization
Reifenstein but do reveals not cite

this in A
of

calcium

phosphorus

patient
23.1

15.5,
be

and
com-

demineralization
hypoparathyroidism,

as a possible clear-cut
the literature

occurrence
specific

pared

calcium-phos-

cases
survey

with
of

evidence associated

of
few

this.
cases

phorus the

products patients

of reported

30.6, by

32.0, Steinberg

and

32.4

in

to have There of pain whom products number was

hypoparathyroidism creased Leonard,6 Eaton published and bone density. et Emerson Haines,9 reports with to accept

with and Sutphin et

deScott,5 al.,

Cantor al.,

had normal are several tients quite were with low found, in

skeletal roentgenograms. other cases in tile literature normal roentgenograms calcium-phosphorus but the overwhelming which be osseous calcification normal had products.

and Klatskin1#{176} have of patients with hypobony all demineralization. of these cases with bony as

parathyridism It is difficult examples

of cases

of hypoparathyroidism

considered to calcium-phosphorus

much higher On the other

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CLINICAL
hand, the case of Eaton and Haines,9 and

NOTES tions work led that to the an he a reduction heart could interesting in the perform. series amount

855 of

to cited had for

lesser above quite the low

extent as values

the showing

case

of

Klatskin,#{176} calcium and The

Orang&5 of studies

deminerahization, products.

performed in which transfused citrated compensation

simultaneously

for serum

calcium-phosphorus

exsanguinated them. When at a certain occurred.

administered,

dogs and then he administered rate If cardiac

or

low figures obtained tllerefore led us to sional appearance tion

may

in our patient have conclude that the occaof bony demineralizaof hypoparathyroidism
of hypodevelopment

blood

cardiac calcium
if heparin-

dewas

in
result

the of

course
from the

ized tion citrate

fusion,

blood failed

was to

used, occur.

decompensasame amount of transsoiuin These a clear-cut due in

The

calcemia

unusual

severity.

The exact mechanism of normal calcification of bone is still poorly understood. Likewise the relationship between parathyroid hormone unsettled.
to us assume required

present if given without cardia

in the blood used for in a very concentrated blood, was seem equally to offer effective decompensation. cardiac failure ion concentration
is very little

tion

producing experiments

and
that for

resorption
there is laying a

of it would and exceed

not be

bone seem

remains logical
minimal of bone.

would

However,

certain down

example of mammalian to decreased calcium the in

no in

concentration If the values

bone

of calcium
the

of phosphorthe necessary low

to

blood.
Surprisingly, there mention of
1

do the

not
will

the
human

literature
beings

of
in

cardiac
the

embarrassment
presence severe

minimum,

deposited

matter Failure
of bone an

what
equally would

cause
impaired

for of bone

the
ability

values. absence
resorb We

hypocahcemia.

Lachmann,i

of deposition
result in

in the

gun,18 gested The on a would

physical is

and this

Evans for

and

EhlioV9

have

all

Hegsug-

relationship. cardiac decompensation our patient Numerous

physi-

demineralization.

evidence hypocalcemic seem to

examinations

believe that demineralization tient.

able

this

is the explanation of bone found found bones and as the

of evidence

in
that

for our
she

the pa-

be

basis in conclusive.
by competent

We

have

furthermore

unmistakelevation calciumof
D.

roentgenologic

able to recalcify her of the serum calcium phosphorus

apy with

after of the result

vitamin

cians failed
ease.

before the onset of the to reveal any evidence

Upon recovery from the

present illness of cardiac disacute episode

products
large doses produces in by to It the lie

therthat
The

all evidence

of cardiac

disease

disappeared in this Fluorothe

in size

It
changes

is

well-accepted

clinical
certain electrocardiogram.

fact

and the patient has respect for a period scopically

heart has

remained well of 10 months.

normal

hypocalcemia

definite

and
been

roentgenographically
consistently

mechanism is the believed neuromuscular

which
in

these
an

changes
interference to follow

occur
with that if

and contour. heart sounds

pressure and

There are no murmurs, and the are of good quality. The blood
the pulse rate have been than the no remained electrohypocal-

transmission would appear

of electrical

impulses.

within
is lyte now

normal
normal. disturbance,

limits.
There

The
has other

electrocardiogram

hypocalcemia of severity

attained interference

a sufficient with the

degree neuroimpulses cardiac hypothe-

muscular transmission of electrical might result in clinically apparent

dysfunction. There is ample and

experimental

cemia, to account for the cardiac difficulty present during the acute illness. It is believed that wider appreciation of
hypocalcemic heart disease in human beings may
cases.

evidence
sis. McLean

in animals
and

to support
Hastings13

this decreasing below

heart

Salter

and

lead The

to

the

recognition however, a rare

of

additional is that the

Runels1 calcium
optimal

have ion
point

noted concentration
in isolated

that
frog

the a certain
prepara-

likelihood,

condition will remain seem that hypocalcemia

one. It would ordinarily first

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856 exhibits its neuromuscular

symptoms

SCHULMAN deleterious effects excitability

so compelling

IDIOPATHIC in increased with clinical

relief

HYPOPARATHYROIDISM
idiopathic hypoparathyroidism. Canad. M. A. J., 47:551, 1942. Leonard, M. F. : Chronic idiopathic hypoparathroidism with superimposed Addisons disease in a child. J. Chin. Endocrinol., 6:493, 1946. Emerson, K., Jr., Walsh, F. B., and Howard, J. E. : Idiopathic hypoparathyroidism; a report of two cases. Ann. mt. and McCune, in siblings) idiopathic hypoparathyroidism associated with moniliasis. J. Clin. Endocrinol.,

as to demand

6.

before have the

the

secondary opportunity

effects to develop.

on

the

heart

7.
CONCLUSIONS
Marked deminerahization of
tile

entire

skeleton chronic
is postulated directly

occurred idiopathic
that related to

in a 12-year-old hypoparathyroidism.
the the demineralization

girl

with It
was

Med., 14:1256, 1941. 8. Sutphin, A., Albright, F., D. J. : Five cases (three

of

unusual

severity cardiac

of
9.

3:625,

1943.

the

hypocahcemia. The patient

also There

manifested was and with the no

deof

compensation. organic improved calcium. decompensation calcemia.

evidence

heart disease coincident It is postulated was

the cardiac status the rise in serum that result the of cardiac hypo10.

Eaton, L. McK., and Haines, S. F. : Parathyroid insufficiency with symmetrical cerebral calcification. Report of three cases, ill OIl of which the patient was treated with dihydrotachvsterol.

J.A.M.A.,
Klatskin,
vitamin

113:749,
C. : On
D2

1939.
the
action in of crystalline parachronic

(calciferol)

ACKNOWLEDGMENTS
The mm for their authors Kramer advice and are to and indebted Dr. suggestions. to Bernard Dr. BenjaBenjamin

REFERENCES 1. Ellswortil,
OIl

R., and Howard, J. E. : Studies the physiology of the parathyroid glands. VII. Sonic responses of normal human kidneys and blood to intravenous parathyroid extract. Bull. Johns Hopkins Hosp., 55:296, 1934.

thyroid tetany. J. Clin. Investigation, 17: 431, 1938. 1 1. Peterman, \I. C., alld Carvey, J. L.: Pseudohypoparathyroidism ; case report. J. Lab. & Clin. Med., 33:1620, 1948. 12. Reynolds, T. B., Jacobson, G., Edmonson, H. A., Martin, H. E., and Nelson, C. H.: Pseudohpoparathyroidism : Report of a case showing bony demineralization. J. Clin. Endocrinol., 12:560, 1952. 13. McLean, F. C., and Hastings, A. B. : A biological method for the estimation of calcium ion concentration. J. Biol. Chem., 107:337, 1934. 14. Salter, \V. T., and Runels, E. A. : A nomogram for cardiac contractility involving

Drake, T. C., Aibright, F., Bauer, W., and Castleman, B. : Chronic idiopathic hypoparathvroidism; report of six cases with autopsy findings in one. Ann. Int. Med., 12:1751, 1939. 3. Steinberg, H., and Waldron, B. R. : Idiopathic hypoparathyroidism : An analysis of fifty-two cases, including the report of a new case. Medicine, 31 : 133, 1952. 4. Albright, F., and Reifenstein, E. C. : The Parathyroid Glands and Metabolic Bone

2.

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calcium, potassium and digitalis-like drugs. Am. J. Physiol., 165:520, 1951. Orange, M. : Personal communication to the author. Lachmann, A. : Hypoparathyroidism in Denmark; clinical study. Acta med. scalldinav., suppl. 121, pp. 1-269, 1941. Rose, E. : Hypoparathvroidism. Clinics, 1: 1179, 1943. Hegglin, B. : Herz und Hypokalzamie. Helvet. med. acta, 6:584, 1939. Evans, J. A., and Elliot, F. D. : Multiple
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IDIOPATHIC HYPOPARATHYROIDISM WITH BONY DEMINERALIZATION AND CARDIAC DECOMPENSATION Jerome L. Schulman and Harold Ratner Pediatrics 1955;16;848-856
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