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urines of both parents and of two normal sibs weretested with negative results. The home lies inan isolated rural district. The parents are not

consanguineous.DETAILS OF THE FAMILY

Patients’ father.-A coachman, aged 63, physicallyhealthy, urine normal. He has recently been sufferingfrom the delusion that people are poisoning him. Hisfather had similar delusions for many years before hisdeath at the age of 84 ; his mother was healthy and diedaged 72.

Patients’ mother.—A hard-working woman of the

labouring class, aged 53, physically healthy, urine normal.A brother of hers has five healthy children, three of whomwere rather dull at school; a sister of hers has-two normalchildren. Her father (maternal grandfather of the patients)suffered from anæmia and had an enlarged liver ; he died

aged 52. The maternal grandmother was very healthyand lived to be 72.

Patients’ sibship.—(1) Female, died at 10 months in

convulsions.—(2) Male, aged 33, workman, very healthyand mentally normal.-(3) Female, aged 31, normal,is married and has a male child, aged 5, also healthy.-(4) Miscarriage at about the tenth week of pregnancy.-(5) Male, aged 24, won a scholarship to the secondaryschool-was fourth in the whole county ; healthy.-(6) Male, aged 22, healthy and intelligent.

(7) Male, aged 19, idiot, urine contains phenylpyruvicacid. Blood Wassermann reaction negative. The birthwas normal at full term and the child was, at an early age,recognised to be mentally defective. He had no convulsions.He has never walked or talked ; he cannot feed himselfand he is wet and dirty in habits ; he sits or lies with hislimbs contracted while he occupies himself with mannerismsof the fingers and with rocking movements of the trunk.He appears to notice a bright moving object. His headis small and brachycephalic (head measurements : length17-1 cm. ; breadth 14-3 cm. ; cephalic index 0-84). Thereis a pigmented patch of skin on the right side of the fore-head. The shoulders are broad but there is a markedkyphosis with depressed sternum ; the teeth are widelyspaced and the molars are decayed. He has long taperingfingers, thin legs, flat feet with partial syndactyly of thesecond and third toes. The testes are undescended

bilaterally and the penis is small. Deep reflexes in thelimbs are all very brisk ; there is bilateral extensor plantarresponse. The abdominal reflexes are present. Theforearms and legs show pronounced muscular wasting.The pupils react irregularly to light, there are signs ofold iritis with adhesions especially in the right eye, bilateralcataract, and small corneal opacities. He once had an

epileptic fit, when aged 14.(8) Male, aged 16, urine normal, works in a foundry,

intelligent and good at games, but scholastically tack-ward on account of visual refractive errors. At the ageof 15 years 2 months his score on the Stanford-Binet testswas only 9 years 8 months.-(9) Female, aged 13, urinenormal, healthy, mentality normal. At the age of 12

years 6 months her Stanford-Binet mental age was

11 years 3 months.

(10) Male, aged 5, idiot, urine contains phenylpyruvicacid ; unable to walk, talk, or stand, he can now crawl alittle but hardly uses his legs at all and sits tailorwise ;wet and dirty in habits ; brachycephalic (head measure-ments at the age of 2 years 1 month : length 6-2 cm. ;breadth 5-4 cm. ; cephalic index 0-87). He suffers from

constipation. The deep reflexes in the legs are very brisk ;plantar responses extensor. Fits started when he was afew months old; they consisted of twitchings associatedwith apparent loss of consciousness. They have continuedever since and often occur several times in one day.

COMMENTARY

The two cases described here are more severelyaffected mentally than those which were described byPollings. The epileptic fits in the second case are anovel feature but the kyphosis with broad shoulders,found in the first case, is supposed to be characteristic.Spasticity and skin pigmentation were found inseveral of the Swedish cases and signs of mentaldisease were noticed in relatives of some.

The discovery of this new special type of amentiaraises some interesting and important problems.It seems likely that the cause of mental deficiencyin these cases is an inborn metabolic error connectedwith the oxidation in the body of phenylalanine.The condition is, in some ways, analogous to

alkaptonuria 2 and both the diseases are probablyinherited as single Mendelian recessive characters.3The special association between abnormal metabolismand mental deficiency, however, which is character-istic of phenylpyruvic amentia, requires much

investigation.I am indebted to Sir F. Gowland Hopkins for providing

me with a specimen of pure phenylpyruvic acid for controlpurposes. The investigation was carried out under theauspices of the Medical Research Council and the DarwinTrust.

DUODENO-RENAL FISTULA

BY R. J. V. PULVERTAFT, M.D. Camb., M.R.C.P. Lond.DIRECTOR OF THE JOHN BURFORD CARLILL LABORATORIES,

WESTMINSTER HOSPITAL; READER IN PATHOLOGYIN THE UNIVERSITY OF LONDON

(WITH ILLUSTRATION ON PLATE)

THE close anatomical relationship between theduodenum, the right kidney, and the colon makespossible the development of fistulous communicationsbetween these organs. Duodeno-colic fistula has beenfound in relation to carcinoma of the hepatic flexure ;but duodeno-renal fistula must be of extreme rarity.In the following case it was made possible owing tothe coincidence of extreme scoliosis and renal calculus ;the organs were therefore brought mechanically intoabnormally close relationship.The patient was an unmarried woman of 28, with a

long history of tuberculous infection. At the age of 10she underwent a series of operations for tuberculousabscesses in relation to the left hip-joint, and at the ageof 12 superadded pyogenic infection necessitated amputa-tion of the left leg at the hip-joint ; this was performedby Mr. William Turner. She remained well for eightyears, and then returned with a recent history of painin relation to the amputation scar. She had then developeda very marked scoliosis with the convexity to the right.She was found to have an abscess in relation to the anteriorsuperior iliac spine on the left side ; several ounces of

pus were evacuated, which showed only S‘taphylococcusalbus no tubercle bacilli were found. Examination ofthe urine at this time showed pus and organisms thenidentified as gonococci. There were no T.B. Uretericcatheterisation by Mr. G. T. Mullally showed the rightkidney to be infected ; coliform bacilli were found, but noT.B. The patient was virgo intacta ; no cultural examina-tions of the organism were made ; and it is highly probablethat this infection was from the outset due to a coliformbacillus. The right kidney was normal.

She next reported eight years later ; the scoliosis wasnow extreme. Her symptoms were pain in the right sideof the abdomen of seven or eight weeks’ duration, and ofincreasing severity. She suffered from nausea after foodand general malaise ; there were no urinary symptoms.The bowels were very loose, and she had lost much weight.The amputation site was quite healthy. The leucocytecount showed 14,600 cells, with 71 per cent. polymorphs.The urine showed much pus and albumin; the onlyorganism found was Staphylococcus albus. The temperaturevaried between 99° and 1032°. The X ray examinationshowed multiple calculi in the right kidney (Fig. I. on Plate).There was considerable tenderness on the right side, andan operation was performed on the right kidney by Mr.William Turner. An incision was made from a little below

2 Dakin, H. D.: Oxidations and Reductions in the AnimalBody, Monographs on Biochemistry, 1922, p. 84.

3 Hogben, L., Worrall, R. L., and Zieve, I. : The GeneticBasis of Alkaptonuria, Proc. Roy. Soc. Edin., 1931-32, lii., 264.

25

the articulation of the twelfth rib with its vertebra towardsthe anterior superior spine. The kidney was opened alongits posterior border, and a quantity of foul-smelling pusevacuated. There was also a large perirenal abscess. One

large and many smaller calculi were removed. and fourdrainage-tubes were inserted, two draining the perirenalabscess and two the kidney. Immediately after the

operation all fluid given by mouth was found to escapewithin a short time from the renal drainage-tubes. Therewere severe rigors ; the patient died four days later.Examination of the large calculus showed it to be

irregularly shaped, with one large protuberance shapedlike the terminal phalanx of the thumb. It was soft andcrumbled readily, and consisted of calcium phosphateand calcium carbonate.At the post-mortem examination an old fibrotic

ulcer was found in the second part of the duodenum,

DUODENO-RENAL FISTULA

FIG. II.—Specimen showing the duodeno-renal fistula.

communicating directly with the pelvis of the rightkidney, to which the duodenum was closely adherent(Fig. 11.). This ulcer was about three-quarters of aninch in diameter, and circular ; its dimensions corre-

sponded closely with the thumb-like protuberanceon the phosphatic calculus. The extreme scoliosishad evidently forced the kidney into closer relationshipwith the duodenum, and calculus formation, whichis probably related in this case to the renal infection,had by pressure produced a fistula between theduodenum and renal pelvis. The orifice had, however,been blocked by the calculus, whose removalestablished a fistulous track from the duodenum tothe skin.The histology of the affected tissues revealed no

tuberculosis ; the process indicated chronic andacute pyogenic infection.My thanks are due to Mr. William Turner for

permission to publish this case, and to Mr. S. A.Sewell for his drawing.

ACROMEGALY AND SPLANGHNOMEGALY

BY FF. ROBERTS, M.D. Camb., M.R.C.P. Lond.PHYSICIAN IN CHARGE OF THE DOUTY X RAY CLINIC,

ADDENBROOKE’S HOSPITAL, CAMBRIDGE

(WITH ILLUSTRATIONS ON PLATE)

IN August, 1927, an unmarried woman of 28 years,was referred to me by Dr. J. R. C. Canney for bariummeal examination. She was 6 ft. 3 in. in height

and typically acromegalic in appearance. For some

years she had suffered from severe headache, butwithout any other symptom or sign of cerebral tumour.Some time previously she had had symptoms indicat-ing left hydronephrosis. On exploration the left

kidney was found to be enormously enlarged, but notstructurally abnormal. Recently she had been

complaining of vague abdominal pain.Barium meal examination revealed no abnormality.

The stomach was large but only in proportion to the sizeof the patient. In view of her headache I examined herskull with the remarkable result shown in Figs. I. and II. onPlate. It shows acromegalic hyperostosis and a large osteomawith a well-defined margin apparently growing from thefalx cerebri. In the frontal and right temporal regionsthe skull has undergone a change closely resembling

ACROMEGALY AND SPLANCHNOMEGALY

FIG. III.—Radiogram showing grossenlargement of the sigmoid.

Paget’s disease. Dr. A. Schuller, of Vienna, tells me thathe has seen acromegaly associated with Paget’s diseasein two cases. The sella, partly obscured by the thickeningof the right temporal bone, measures 14 mm. in the antero.posterior diameter and is thus within the normal size.It shows, however, some slight irregularity of its floor.In June, 1928, owing to persistence of the abdominal

pain, I made a second barium meal examination. Thistime the stomach was enormously enlarged. It was

actually too large to be radiographed on a 12 x 15 film. Ithen gave a barium enema with the result shown in Fig. III.Two pints of fluid were only sufficient to fill the enormouslydistended sigmoid, but the upper part of the large intestinewas equally distended for, being full of gas, its outlinewas clearlv visible.The patient died some months later. There was no

post-mortem examination.

To sum up, this was a case of acromegaly withunusual bony changes in the skull, together withgreat enlargement of the gastro-intestinal tract andat least one of the kidneys. The most interestingfeature was that the- enlargement of the stomach tookplace during the ten months which elapsed betweenthe two occasions on which I examined her.The association of acromegaly with enlargement

of the viscera is well recognised. Dr. Gordon Holmeskindly informs me that he has observed it, and thatin one case he described the colon as being the widthof his own thigh. Atkinson,l in a review of about1400 cases of acromegaly, writes: "A very rare

pathological condition—namely, enlargement of allthe organs-splanchnomegaly must be considered a

part phenomenon of acromegaly, as it has been met