69 Clinical Section 1015
Parents healthy; one other child, younger, who enjoys perfect health. No
similar case in family.
Blood examination on admission: Red cells 1,350,000 ; Hb. 19%; C.I. 0.7
white cells 2,200. (Polys. 44% ; lymphos. 39%.) Van den Bergh reaction,
negative direct and indirect. W.R. negative. Reds: fragility normal.
Splenectomy performed September 1931. Cells showed microscopically typical
nests of Gaucher cells, replacing some of the normal splenic tissue.
Great general improvement since operation and the anemia has disappeared. She
is mentally normal and likes playing games with other children. About eighteen
months ago she suffered from osteomyelitis of the right femur; Gaucher cells present
in the discharge; perfect recovery. There is slight kyphosis in the mid-dorsal
region.
Blood-count (23.2.34).-R.B.C. 4,400,000; Hb. 79%; C.I. 0-89; white cells,
19,000; platelets plentiful. Reds show no significant abnormality. No myelocytes.
Cushing's Basophil Pituitary Syndrome.-E. STOLKIND, M.D.
Female, aged 25, is suffering from hirsuties, high blood-pressure, attacks of
dyspncea and cough, headaches and migraine, mitral stenosis; obesity (limbs normal);
polycythawmia. Clinoid processes approximated; albumin, granular and epithelial
casts in urine; hair falling out.
Mother, who was not hairy, suiffered from migraine and died from nephritis.
Father and two brothers, healthy.
Patient had diphtheria at the age of 4, and rheumatic fever at the age of 12.
At 'about 16 hair began to appear, first on the upper lip, and then spreading to the
JUNE-CLIN. 2 *
1016 Proceedings of the Royal Society of Medtctne 70
face. At 17 it appeared also on the chest between the breasts, and later on the face,
shoulders, extremities, etc.; the pubic hair spread as far as the umbilicus. The
first menstrual period was at the age of 12 and subsequently has been regular, but
during the last months it lasts two days less and she loses much less. She married
in 1927 and has had three pregnancies: two premature births at six months at 21 and
22 years of age respectively, and a miscarriage of two months at 24; all without
any obvious cause. She has suffered from headaches since sbe was 12, and more so
since she was 15. They are mostly typical attacks of migraine with nausea and
vomiting, visual disturbances (sees various colours), etc. She had also headaches
without these symptoms.
Since August 1, 1933, there have been four attacks of dyspncea (two severe)
with difficult inspiration, coughing and frothy blood-tinged sputum. The first and
fourth attacks occurred during coitus. Lately she complained of pain in the left side
of the chest, left shoulder, and upper arm.
She is a dark, short, plethoric-looking, round-shouldered, fat woman; her height
is 4 ft. 11i in.; weight now 11 st. 2 lb. She says she was 10 st. 5 lb. in 1927;
13 st. in February 1932. The obesity is chiefly in the trunk, with pads of fat; the
extremities are spared. She has a high colour. The skin is dry. Many small dark
brown spots on the body. There are purplish strice atrophice on the abdomen,
back and thighs. Acrocyanosis of the legs with ecchymoses. Easily bruised.
Now shaves her beard and moustache several times a week. Much hair between
the breasts and an extension of the pubic hair upward to the umbilicus, a few hairs
over areolh round nipples, etc., on extremities and on the dorsum pedis. Lately she
has noticed that the hair on the temples is falling out. Dr. F. G. McCann found the
genital organs normal. She has mitral stenosis. Pulse, 90-120.
January 1933.-Albumin and a few granular and epithelial casts found.
August 1933.-Albumin in urine; blood-urea, 30 mgm. per 100 c.c.
Bilateral pyelography gave normal results.
During the last fourteen months blood-pressure 235-260 (systolic) and 150
(diastolic). Wassermann and Kahn reaction (blood) repeatedly negative.
Latest investigations.-X-ray (Dr. Martin Berry) : Heart horizontal and
enlarged. Sella turcica: clinoid processes rather approximated.
Nothing abnormal in the kidneys; no evidence of calcified tibial arteries.
Blood-count (Dr. Carnegie Dickson).-R.B.C. 6,150,000 per c.mm.; W.B.C. 14,200
per c.mm.; Hb. 102%; C'.I. 0 83.
Differential leucocyte count (of 200 counted): Polys. 76%; lymphos. 17 5%;
large monos. 4 0%; eosinos. 1 5%; basos. 0 5%. Films: Many R.B.C. 7 to 61 U,
or a little less.
Urine (catheter specimen): Urea, 0-8%; albumin, 0-08% (Aufrecht); occasional
degenerated cellular casts and a few epithelial cells, probably from collecting tubules
and very occasional polymorphs. Total quantity of urine in twenty-four hours
1,200c.c.,sp. gr. 1010-1020.
Urea concentration test: resting, 1-3%; after one hour, 1-6% urea; after two
hours, 75 c.c. urine and 1-7% urea. Blood-urea, 68 mgm. per 100 c.c. Urea
concentration factor, 19-1.
Blood-sugar test: fasting, 0-106%, it rose after 50 grm. of glucose to 0-181% in
one hour, and 0-135% in two hours; in urine 0-12% of sugar after two hours. In
this case the adrenal cortex, in all probability, is also affected.
Fundi (A. Sorsby). Marked arteriosclerosis (crushing). Right: occlusion of a
peripheral arteriole on temporal side; new vessel formation. Left: hiemorrhages
and exudates which are absorbing.
This is a case of Cushing's pituitary basophil syndrome. The final proof of this
diagnosis in cases with normal pituitary fossa is so far only possible after examination
of serial sections of the pituitary gland. There are no special pathognomonic symptoms
71 Clinical Section 1017
or signs of Cushing's pituitary basophil syndrome, which would distinguish it from
a genito-adrenal syndrome.
Dr. J. H. Douglas Webster is giving a short course of deep X-ray treatment to the
pituitary gland of my patient.
I am indebted to Dr. W. R. Reynell for facilities to investigate this case.
Two Cases of Dyspituitarism.-D. C. HARE, C.B.E., M.D.
(I) Cushing's basophil pituitary syndrome.
Chief symptoms and signs.- Attacks of severe headache and vomiting. Persistent
metrorrhagia. Hirsutism, male type, with loss of scalp hair. Skin of face greasy
with acne eruption. Adiposity of face, neck, shoulders, and trunk. Limbs normal.
Kyphosis. Polycythsemia with cyanosed extremities. Raised blood-pressure.
Pituitary fossa not enlarged.
History.-Miss R. L., aged 32, machinist. In good health till November 1931,
when she missed two menstrual periods and began to be languid and sleepless. After
an attack of tonsillitis in February 1932 the menses became gradually prolonged
lasting up to six weeks. The daily loss was not excessive at any time; during 1933
the average duration was from seventeen to twenty-one days with irregular intervals.
~~~~i- It~ -w|L"
FIG. 1.-Miss R. L. at age 24. FIG. 2.-At age 32.
Changes in appearance were noticed early in 1933. Gain in weight about half a
stone; the complexion, previously good, became spotty, the scalp hair broke off short,
and the beard began to g'row in April 1933. Headaches, to which she was always
liable, increased in frequency, severity and duration, vomiting was liable to occur on
waking or would follow the development of the headache. The appetite was very
good but not excessive. Thirst increased and nocturnal frequency of micturition
increased. Voice, no chainge noted. No history of htemorrhages or bru'isi'ng
obtained except a slight hiemoptysis in January 1934. Mentality and efficiency
unimpaired; patient continued at work until February 1934.
Past and family history.-Patient is the tenth of twelve children. She has had
no previous illnesses of importance. Menses previously regular. Family healthy.