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Use of Serum

Amylase

lsoenzymes

in Evaluation

of

Pancreatic

Function

Lynn M. Taussig, M.D., Robert 0. Wolf, D.D.S., Robert E. Woods, M.D., Ph.D., and

Richard J. Deckelbaum, M.D.

From the Department ofRe.rpiratory F,,nctic’n, The .Iontreal Children’s Hospital, .%lontreal, Quebec, Canada; and Oral .‘4edicine Branch, ,Vwional institute of Dent’al Research and Pediatric %feutholLun Branch, .\‘ational Institute of Arthritis, ,fetabolic, and Digestite Diseases. Bethesda, .%Iaryland

ABSTRACT. Amylase isoenzyme patterns were evaluated in serum, urine, and duodenal fluid from 19 patients with cystic

fibrosis (CF) and normal subjects. Two thirds of the CF

pa-tients with absent pancreatic enzymes lacked a senim pan-creatic isoamylase band, while the remainder had a marked-ly diminished pancreatic band when compared to the

sali-vary isoamylase. In normal sera the pancreatic band is equal

to or greater than the salivary band. In all patients with ab-sent enzymes the diagnosis of abnormal pancreatic function could have been made by evaluation of serum isoamyla.se patterns thereby avoiding duodenat intubation or collection of 72-hour stools for proteolytic enzyme activities. The pa-tients with normal pancreatic enzyme activities had normal

serum zymograms. No CF patient had a low total serum am-ylase concentration. Total duodenat fluid amylase levels may be misleading in the evaluation of pancreatic function since the amylase may be of salivary origin.

The advantages of this isoamylase method include (1)

sim-plicity and noninvasiveness, requiring only a small blood sample; (2) specificity for pancreatic function; and (3) inde-pendence of pancreatic enzyme medication. Pediatrics,

54:229, 1974, AMYLASE, ISOAMYLASES, AMYLASE 1SOENzYMES, PANCREAS, CYSTIC FiBROSIS.

function. Duodenal intubation with direct

mea-sumement of pancreatic secretions is the most ac-curate diagnostic test of pancreatic function.9 Un-fortunately, such intubation is often difficult to perform, uncomfortable for the patient, time

con-suming for patient and physician alike, and at

times unsuccessful.

Serum and urine alpha-amylase (hereafter

me-femmed to as amylase) can be separated by various methods into its isoenzymes (isoamylases).’#{176}” Such separation of isoamylases recently has been shown to be of benefit in assessing the functional development of the pancreas and salivary glands in human fetuses” as well as in studying

pancre-atic function in sporadic chronic 2

The present study was undertaken to determine if the diagnosis of partial or complete pancreatic insufficiency could be made in CF patients by

ex-amining the isoamylase patterns of serum and

urine for the presence or absence of pancreatic isoamylase.

MATERIALS AND METHODS

Documentation of partial or complete insuffi-ciency of exocrine pancreatic function in infancy and childhood is often very important in the diag-nosis and evaluation of such diseases as cystic fi-brosis (CF),’ hereditary pancreatitis,2 and the syn-drome of pancreatic insufficiency and bone mar-row dysfunction.3 Stool fat and starch excretion, fecal trypsin and chymotrypsin activity,47 serum carotene and cholesterol levels, and other absomp-tion and excretion tests”8 have been used to evalu-ate exocnine pancreatic function. These tests often lack the sensitivity and specificity needed to es-tablish a definite diagnosis of abnormal pancreatic

Serum, urine and duodenal fluid from 19

pa-tients with previously diagnosed CF’ were stud-ied. The patients were 3 weeks to 28 years of age

(Received October 9, 1973; revision accepted for publication January 31, 1974.)

L.N1.T. is a Clinical Fellow, National Cystic Fibrosis Re-search Foundation.

ADDRESS FOR REPRINTS: (L. NI. T.) Department of Pediatrics, Arizona Medical Center, Tucson, Arizona

(2)

(11 males and 8 females). The NIFI prognostic scores” for the 15 patients with pulmonary func-tion tests ranged from 58 to 93. Duodenal fluid was collected after a 12-hour fast by intubation with double lumen tubes placed tinder fluoroscopy. The pmoxinal part of the tube was left in the stom-ach to drain gastric secretions while the distal seg-ment (second lumen) was placed in the second and third portions of the duodenum. No duodenal fluid with a pH of less than 6.0 was accepted for study. Pmoteolytic enzyme (chymotrypsin, carboxypep-tidase B, and trypsin) activities were measured by

previously reported methods. ‘‘ Patients who

were taking supplemental pancreatic enzymes

stopped this therapy 48 to 72 hours before intuba-tion.

Control sema and urines were obtained from six male and six female white children,

7

to 16 years of age. Pamotid saliva and duodenal fluid were col-lected as previously described’1’8 from normal subjects and evaluated for comparative purposes.

Sera and urines from normal infants and adults

were evaluated in a previous study.h1

Total serum and duodenal arnylase

concentra-tions were measured by an amyloclastic method

based on timed iodine color reactions and

ex-pressed in Somogyi units.’9

A

B

C

D

+

Ftc. 1. Nornial isoamvlase patterns: A, dnodenal fluid (1).F.); B, saliva; C, seruni: I), urine. The major pancreatic

isoamy-lase is towards the cathode while the major salivary isoaIiy-lase is more anodic. Minor isoanylases froii each organ are

also evident. (For Figures 1 to 5, the interface between the

runlling and stacking gels at the right edge of each figure is

comparable to the origin. The stippled graphic represeilta-tion below each gel indicates isoamylase l)and width and

density.)

Isoarnylases of the various specimens were sep-arated according to the method of Wolf and Tay-1or,’ with modifications as described by Wolf and

Taussig.” Briefly, the isoamylases were separated

by

polyacrylamide gel electrophoresis employing

the standard 7% acrylamide separating gel and a

tmis-glycine reservoir buffer at pH 8.3. Sample volumes of 10 to 120il were used. Electmophoresis was carried out for 1.25 hours at a constant amper-age of 5 mA per gel at 0 C. After electrophomesis, the isoamylase bands were identified by squeezing

and incubating the gel columns between two

slides, one of which was coated with a starch agar film. Duodenal fluid and saliva samples were

incu-bated for 15 minutes at room temperature while

semuni and urine (which contain far less amylase) were incubated at 37 C in a moist box for six hours.

After incubation, the gels were removed and the starch slides were developed in an iodine solution. The areas of digested starch, indicating the loca-tions of the isoaniylases, appeared as clear areas against a blue background.

In addition to the evaluation described above,

serum and urine zymogmams were compared be-fore and after the ingestion of supplemental pan-creatic enzymes (Cotazym#{174}#{176})in two CF patients with pancreatic insufficiency. The patients had either discontinued or had taken the enzymes for at least 48 hours before samples were collected.

RESULTS

Normal Isoamylase Patterns (Fig. 1)

Normal pancreatic fluid found in the duodenum (Fig. 1, A) has one major and three to four minor

bands which are more cathodic. Normal saliva (Fig. 1, B) has one major and at least four minor

bands which are more anodic. Normal serum

(Fig. 1, C) has two major bands which are electro-phoretically identical to the major pancreatic and salivary bands, but in reduced concentration. Normal urine (Fig. 1, bottom right) reflects the serum pattern and, therefore, contains the major pancreatic and salivary bands. Occasionally the serum and urine zymogmams reveal additional minor isoamylases from both organs.

The isoamylase patterns of sera and urines from

all 12 control children were similar. These

subjects had major pancreatic and salivary isoen-zymes in both serum and urine. In all cases, the

pancreatic band was approximately equal to or

greater than the salivary band in width and

inten-sity both in serum and urine (Fig. 1, C and D).

Simi-lam results have previously been reported for

(3)

PANCREATIC ISOAMYLASES IN VARIOUS BODY FLUIDS

IN CYSTIC FIBROSIS PATIENTS

Requires Proteolytic

Pancreatic isoamylase

Total Serum

Oral Enzyme Pancreatic Duodenal Amylase

Patient Age Therapy Enzymes0 Fluid Serum Urine (Somogyi units)t

1 3weeks Yes N.D.S ND. 0 Low 23

2 3mo Yes ND. ND. 0 0 16

3 10 mo No ND. ND. 0 Low 88

4 3yr Yes 0 0 0 Low 100

5 7 yr Yes 0 0 0 Low 98

6 8yr Yes 0 0 0 Low 132

7 10 yr Yes 0 0 Low Low 140

8 l2yr Yes 0 0 0 Low 100

9 12 yr No Normal Normal Normal Normal 128

10 l4yr Yes 0 0 0 0 210

11 l5yr Yes 0 0 0 Low 83

12 l6yr Yes 0 0 0 Low 108

13 18 yr Yes 0 Low Low Low 94

14 l9yr Yes 0 0 0 0 65

15 20 yr Yes 0 0 Low Low 97

16 25 yr Yes 0 0 Low Low 96

17 25 yr No Normal Normal Normal Normal 147

18 26yr Yes 0 0 0 Low 133

19 28 yr Yes 0 0 0 Low 62

15-19: 10 to 140 Somogyi units; for patients 3-8, 14: 30 to 150 Somogyi units.

+

A

D.F.

B

Serum

C

Urine

D

Urine

FI;. 2. Isoamylase patterns in cystic fibrosis patients with ab-sent pancreatic proteolytic enzymes: A, duodenal fluid; B,

senim; C, urine (absent pancreatic isoamylase); D, urine (di-minished pancreatic band). Duodenal fluid and serum both

lack pancreatic isoamylase. TABLE I

#{176}Collected by duodenal intubation. Normal values for patients 1, 2, 9-13,

N.D. = not done.

fants and The intensity of the various iso- Total duodenal amylase concentrations were

amylase bands from each person correlated di- measured in eight of the patients listed in Table I.

mectly with the individual’s total serum amylase In six (all with absent proteolytic enzymes) no

am-concentration.

CF Patients: Duodenal Fluid

Sixteen of the 19 CF patients had duodenal

intubations; 14 of these had completely absent proteolytic enzyme activity in their duodenal fluid (Table I). Two patients had essentially nor-mal pmoteolytic activity (patient 9: trypsin, 126 units/ml; chymotrypsin, 1,800 units/ml; and car-boxypeptidase B, 217 units/ml; patient 1 7:

tryp-sin,

663

units/ml; chymotmypsin, 4,000 units/ml;

and camboxypeptidase B, 165 units/ml. Normal

values in this laboratory are 100 to 1,000 units/ml

for trypsin, 3,000 to 15,000 units/ml for chymo-trypsin, and 100 to 500 units/ml for carboxypep-tidase B).

(4)

A

B

C

+

D.

P.

Serum

Urine

Fic. 3. IsoaIvlase patterns fron patient L3. This patient

lacked pancreatic proteolytic enzymes lnt had a faint pan-creatic isoanivlase I)and in his duodenal fluid and serum:

A, duodenal fluid: B. serulli: (, urine.

A

B

C

FIG. 4. lsoa,nvlase patterns in a cystic fibrosis patient svith noriiial pancreatic enzyme activities (patient 9): A. duodenal

fluid; B, serum: C, urine. There is a norlnal paflcreatic isoani-ylase band in all three fluids. Salivary isoansylase is also

evi-dent in the duodenal fluid zvn4ogran.

+

Fi;. 5. Salivary isoainvlase in the dilodenal fluid of a cystic

fi-brosis patient with absent pancreatic enzymes. Total duode-nal fluid amylase activity s’as normal but only salivary iso-amylase could be identified. ‘s. normal duodenal fluid; B,

Pa-tient’s duodenal fluid: C, normal saliva.

ylase could be detected; zymograms also were

negative for amylase. The other two patients,

both

with normal proteolytic enzyme activity, had

nor-mal duodenal aniylase concentrations and

zymo-grams demonstrated the presence of pancreatic amnylase. In three additional young adult CF pa-tients (not shown in Table I), all with absent pro-teolytic enzymes, the duodenal fluid amylase

con-centmations were normal (84,000, 105,000, and

163,000 Somogyi units, normal being greater than 80,000 Somogyi units). However, zymograms me-vealed that only salivary isoamylase was present in these specimens (Fig. 5).

Serum Isoamylase

Pancreatic isoamylase was absent from the sema of ten of the 13 patients with absent pancreatic isoamylase in their duodenal fluid (Fig. 2, B). In the other three patients (7, 15 and 16) a faint

pan-creatic isoamylase band was found in the serum

(Table I). Patient 13, who had absent pmoteolytic

enzymes but a faint pancreatic isoamylase band in his duodenal fluid, also had a weak pancreatic band in his serum (Fig. 3, B). In these four patients,

the serum pancreatic band was markedly

dimin-ished in width and intensity when compared to

their seruni salivary isoamylase bands. Serum pan-creatic isoamylase bands were normal in the two

CF patients with normal pancreatic enzyme

1ev-els (Table I and Fig. 4, B). Total serum amylase concentrations for the CF patients were normal in all cases and even slightly elevated in one patient. Even the patients with no obvious pancreatic iso-aniylase in their sera had normal total serum am-ylase concentrations (Table I).

Urinary Isoamylase

The pancreatic isoamylase band was normal in

the urine from the two patients with normal

pan-creatic enzymes and normal serum pancreatic

.aniylase (Fig. 4, C). No pancreatic isoamylase could be found in the urine of three patients (2, 10 and 14) all of whom lacked pancreatic isoamylase in their serum (Fig. 2, C). In the remaining 14 pa-tients, zymograms of urine revealed pancreatic isoaniylase, but in all of these, the salivary band was always more prominent than the pancreatic band (Figs. 2, D and 3, C). Ten CF patients who did

not have serum pancreatic isoamylase had urine

pancreatic isoamylase. No patient with serum

pancreatic amylase lacked urine pancreatic amy-lase.

(5)

DISCUSSION

The separation of amylase isoenzymes by gel

electrophoresis has been shown’#{176}” to be reliable

and reproducible. Our data”’8 as well as that

from other studies’#{176}indicate that both pancreatic

and salivary amylase consist of one major and a

variable number of minor isoamylases. Normal

serum usually consists of the major pancreatic and salivary isoenzymes but occasionally minor bands derived from either organ may be found in addi-tion. Norby2#{176}has shown that duodenal fluid and salivary amylases are electrophometically

identi-cal to amylase found in pancreatic and pamotid

gland extracts, respectively. Approximately 4% of normal children do not have a serum salivary iso-amylase2l; however, isolated absence of the senun

pancreatic band in normal individuals has not

been reported. Urine zymogmams reflect serum

patterns and show the major and some minor

iso-enzymes. The data from our normal infants, chil-dren, and adults” are consistent with these find-ings as all had both major isoamylases in their sema and umines.

The present study indicates that patients with documented absence of pancreatic enzyme activi-ties have abnormal serum isoamylase patterns. In normal sera, the pancreatic isoenzyme is equal to or greater than the salivary isoamylase. Two thirds of the CF patients with absent pancreatic

proteo-lytic enzymes lacked serum pancreatic

isoamy-lase. The remaining third had extremely low

amounts of this isoamylase in their sera as

mdi-cated by the very weak serum pancreatic bands.

Therefore in all of these patients the diagnosis of grossly abnormal pancreatic function could have been made by evaluation of serum isoamylase pat-temns alone.

It should be noted that total absence of the pan-creatic isoamylase in serum is not necessary for the diagnosis of abnormal pancreatic function. Comparison of the pancreatic band to the salivary

isoamylase is also of value in documenting

de-creased pancreatic ftmction. The presence of se-rum pancreatic amylase in four patients with ab-sent duodenal pmoteolytic enzymes suggests that absence of pancreatic enzymes on routine

duode-nal intubation does not always imply total

de-struction of pancreatic acinam tissue. Shwachman

et 22 previously obtained similar results when

they reported that some CF patients with absent enzymes on routine duodenal intubation could ex-crete low levels of pancreatic enzymes after pan-creatic stimulation. The one patient in the present study who had absent proteolytic enzymes but

who also had a faint pancreatic amylase band in

his duodenal fluid supports previous studies2223

which indicated that mechanisms and inhibition of excretion of the various pancreatic enzymes in diseases of the pancreas are variable. Because of this variability, evaluation of serum isoamylases

should not replace duodenal intubation for

de-tailed evaluation of pancreatic function. Evalua-tion of serum isoamylase patterns may prove use-fii1, though, in the study of the progression of pan-creatic involvement in CF and other diseases.

The absence of pancreatic isoamylase in sera of some CF patients is due to pancreatic insufficien-cy per se and is not another unrelated abnormality in this disease, since CF patients with normal 1ev-els of pancreatic enzymes on routine duodenal

collection had normal isoamylase patterns in

serum, urine, and duodenal fluid. Although the ab-solute amount of pancreatic amylase in duodenal fluid increases with age,9 age does not affect the isoamylase patterns since infants, children, and adults have identical zymogmams as demonstrated in the present and previous studies.”

Urine zymograms were similar to the serum

patterns, although most patients had some urine

pancreatic isoamylase. In all of these cases,

though, theme was a reversal of the usual pattern in that the salivary band was always more prominent than the pancreatic band. Aw et al.’2 have shown that normal urine usually contains more

pancre-atic amylase than salivary amylase while urine

from patients with pancreatic insufficiency has less pancreatic than salivary amylase. The results of the present study confirm these observations.

Amylase is cleared by the kidneys and

concen-trated in the urine before excretion.24 Duane et a!. 24.23 have shown in baboons that the renal

clear-ance rate for pancreatic amylase is approximately 1.8 times that for salivary amylase. Therefore,

un-detectable amounts of pancreatic amylase in

serum might be concentrated by the kidneys and

thereby become evident on urine zymograms.

This could explain the apparent absence of

pan-creatic isoamylase in serum but its presence in

urine in some CF patients. These results indicate

that evaluation of serum isoamylase patterns is

more discerning and diagnostic than urine zymo-grams.

While duodenal intubation with direct mea-sumement of pancreatic secretions in the duodenal fluid is generally specific for pancreatic function,

determination of total duodenal fluid amylase

concentrations may be misleading. Three CF

pa-tients with absent proteolytic enzymes had nor-mal total duodenal amylase activity. However,

electmophoresis indicated that the amylase was

solely of salivary origin (Fig. 5). In some CF

(6)

stomach unaltered by gastric acid. Alternatively, although double lumen tubes were used, the pres-ence of salivary amylase in the duodenal fluid may represent a “contaminant,” a result of the intuba-tion.

This study suggests that the diagnosis of most

cases of abnormal pancreatic function can be

made by evaluation of serum isoamylases, thus

often avoiding the necessity for duodenal intuba-tion. Measurement of fecal fat may indicate the presence of steatorrhea but does not diagnose the etiology of the fat malabsomption. Therefore, the

examination of serum isoamylases should not

necessarily replace fecal fat measurements, but

should be used in conjunction with them in the

evaluation of a patient with suspected steatomrhea and malabsorption. Other tests of pancreatic func-tion such as quantitative measurements of fecal enzyme levels (chymotrypsin, trypsin) require dis-continuation of oral pancreatic enzyme supple-ments and timed (preferably three-day) stool col-lections. Ingestion of supplemental pancreatic

en-zymes

did

not change the serum and urine

zymo-grams, indicating that such medications do not

have to be discontinued before collecting speci-mens for isoamylase determinations.

This method of separation of serum isoamylases should be beneficial in other clinical situations than the diagnosis of total exocrine pancreatic

in-sufficiency. Lilibridge and Townes reported26 a

transient deficiency of pancreatic amylase in an infant with diarrhea. Lowe and May27 also me-ported a case of absent pancreatic amylase and di-minished trypsin activity. The true incidence of these “syndromes” is unknown, but a noninvasive technique, such as serum isoamylase determina-tion, may be beneficial in diagnosing more of these cases as well as in the evaluation of infantile diam-rhea. The evaluation of serum isoamylases would not be of benefit, though, in cases of isolated defi-ciencies of pancreatic proteolytic and lipolytic activity28 or in trypsinogen deficiency29; in these diseases, pancreatic amylase is normal.

REFERENCES

1. di Sant’Agnese, P. A.: The pancreas. in Nelson, %V. E., Vaughn, V. C., III, and McKay, R. J. (eds.):

Text-book of Pediatrics, ed. 9. Philadelphia: W. B. Saun-ders Co., 1969, p. 854.

2. Kattwinkel, J., Lapey, A., di Sant’Agnese, P. A., Ed-wards, W. A., and Hufty, M. P.: hereditary pan-creatitis: Three new kindreds and a critical review of the literature. Pediatrics, 51:55, 1973.

3. Shwachman, H., Diamond, L. K., Oski, F. A., and Khaw, K.-T. : The syndrome of pancreatic insufficiency and bone marrow dysfunction. J. Pediat., 65:645,

1964.

4. Barbero, C. J., Sibinga, M. S., Marino, J.M., and Seibel,

R.: Stool trypsin and chymotrypsin. Amer. J. Dis. Child., 112:536, 1966.

5. Banwell, J.C., Leonard, P. J., and Lobo, R. M. F.: Mea-surenient of trypsin and chymotrypsin activity in

stools to detect chronic pancreatic disease. Gut,

6:143, 1965.

6. Dyck, W. P.: Titrimetric measurements of fecal trypsin

and chyniotrypsin in cystic fibrosis with pancreatic exocrine insufficiency. AIller. J. Dig. Dis., 12:310,

1967.

7. Bonin, A., Roy, C. C., Lasalle, R., Weber, A., and Morin,

C. L.: Fecal chymotrypsin: A reliable index of exo-crine pancreatic function in children. J. Pediat.,

83:594, 1973.

8. Silverlnan, A., Roy, C. C., and Cozzetto, F. J.: Pediatric

Clinical Gastroenterology. St. Louis: C. V. Mosby

Co., 1971.

9. Hadorn, B., Zoppi, G., Shmerling, D. H., Prader, A.,

McIntyre, I., and Anderson, C. M.: uantitative as-sessment of exocrine pancreatic function in infants

and children. J. Pediat., 73:39, 1968.

10. Meites, S., and Rogots, S.: Amyta.se isoenzymes. CRC

Critical Reviews in Clin. Lab. Sd., January 1971,

p. 103.

11. Wolf, R. 0., and Taussig, L. Ni.: Human amniotic fluid

isoamytases :Funct jonal development of fetal pan-creas and salivary glands. Obstet. Gynec., 41:337,

1973.

12. Aw, S. E., Hobbs, J. R., and \Vootton, I. D. P.: Urinary

isoamylases in the diagnosis of chronic

pancreati-tis. Gut, 8:402, 1967.

13. Taussig, L. M., Kattwinkel, J., Friedewald, \V. T., and

di Sant’Agnese, P. A. : A new prognostic score

and clinical evaluation system for cystic fibrosis. J.

Pediat., 82:380, 1973.

14. Northrop, J. H., and Hussey, R. G.: A method for the

quantitative determination of trypsin and pepsin.

J. Gen. Physiol., 5:353, 1923.

15. Schwert, G. ‘V., and Takenaka, Y.: A

spectrophoto-metric deterniination of trypsin and chymotrypsin.

Biochim. Biophys. Acta, 16:570, 1955.

16. Folk, J.E., Piez, K. A., Carroll, \V. R., and Gladner, J.A.: Carboxypeptida.se B: IV. Purification and

charac-terization of the porcine enzyme. J. Biol. Chem., 235:2272, 1960.

17. Shapira, E., Arnon, R., and Russell, A.: Specific imniu-noassay for quantitative deterniination of human trypsin in intestinal content. J. Lab. Clin. Med.,

77:877, 1971.

18. Wolf, R. 0.. and Taylor, L. L. : Isozyme demonstration technique. Amer. J. Clin. Path., 49:871, 1968. 19. Somogyi, M.: Micromethods for the estimation of

dia-stase. J. Biol. Chem., 125:319, 1938.

20. Norby, S.: Electrophoretic non-identity of human sali-vary and pancreatic amylases. Exp. Cell Res.,

36:663, 1964.

21. Kamafyt, J., and Laxov#{225}, R.: Amyla.se heterogeneity.

Huniangenetik, 1:579, 1965.

22. Shwachman, H., Dooley, R. R., Guilmette, F., Patterson, P. R., \Veil, C., and Leubner, 11.: Cystic fibrosis of the pancreas with varying degrees of pancreatic

in-sufficiency. Amer. J. Dis. Child., 92:347, 1956.

23. Goldberg, D. M., and Wormsley, K. G.: The interrela-tionships of pancreatic enzymes in human

duode-nal aspirate. Gut, 11:859, 1970.

24. Duane, W. C., Frerichs, R., and Levitt, M. D.:

Distribu-tion, turnover and mechanism of renal excretion of

(7)

25. Duane, W. C., Frerichs, R., and Levitt, M. D.:

Simulta-neous study of the metabolic turnover and renal

ex-cretion ofsalivary amylase _125I and pancreatic

am-ylase _“I in the baboon. J.Clin. Invest., 51:1504, 1972.

26. Lilibridge, C. B., and Townes, P. L.: Physiologic

defi-ciency of pancreatic amylase in infancy: A factor in iatrogenic diarrhea. J. Pediat., 82:279, 1973.

27. Lowe, C. U., and May, C. D.: Selective pancreatic

defi-ciency: Absent amylase, diminished trypsin, and

normal lipase. Amer. J. Dis. Child., 82:459, 1951.

28. Townes, P. L.: Proteolytic and lipolytic deficiency of the exocrine pancreas. J. Pediat., 75:221, 1969. 29. Townes, P. L.: Trypsinogen deficiency disease. J.

Pedi-at., 66:275, 1965.

ACKNOWLEDGMENT

The authors extend their sincerest appreciation to Drs. Paul A. di Sant’Agnese, Pierre Beaudry and Mary Ellen Avery for their support and advice, and to Dr. Mimi Bel-monte for her assistance in referring her patients to us for study.

AN

ACCOUNT

OF

A MONSTROUS

CHILD

BY DR.

JOHN

BURTON,

PHYSICIAN

AT YORK

The case history below, published in 1747, dem-onstrates two things. First, that children could sum-vive in spite of serious congenital anomalies; and second, that physicians’ claims were not always accepted at face value.

-Wright, Spouse to a Ship Carpenter living at Kirk-thorp near Wakefield in Yorkshire, bore a Child that had no Parts of Generation proper either to Male or Female, there not being the least Appearance of such Organs at the Place where we should expect to find those Parts; the child in every other Part was made as is common, except about half Way betwixt the Navel and Os pubic there was a circular Orifice of about an Inch Diameter, in which was a spongious Substance resembling the end of the Glans Penis excoriated;

it did not project in the least from the Body, neither was it covered, but was quite bare, and very sore and tender. Through the several and almost innumerable Pores or On-fices of this spongious Body the Urine ouzed (sic) continually, and sometimes Blood, and at other times a reddish-coloured Serum. The Child lived to the Age of five Years or there-abouts, and died of the small Pox in November last. To the Truth of this I can bring many certificates, as well as living witnesses, if it was thought necessary.’

Noted BY T. E. C., Jr., M.D.

REFERENCE

1. Burton, J.: An account of a monstrous child. Medical Es-says and Observations (Edinburgh), 5: 1747, pp.

(8)

1974;54;229

Pediatrics

Lynn M. Taussig, Robert O. Wolf, Robert E. Woods and Richard J. Deckelbaum

Use of Serum Amylase Isoenzymes in Evaluation of Pancreatic Function

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1974;54;229

Pediatrics

Lynn M. Taussig, Robert O. Wolf, Robert E. Woods and Richard J. Deckelbaum

Use of Serum Amylase Isoenzymes in Evaluation of Pancreatic Function

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