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PUBLIC

HEALTH

CHARACTERISTICS

OF PATIENTS

WITH

CYSTIC FIBROSIS

DISCHARGED

FROM

HOSPITALS

IN I 957: ESTIMATES

FOR

THE

UNITED

STATES

Elizabeth R. Kramm, Ph.D., M.P.H., Marian M. Crane, M.D., Morton L. Brown, B.A.,

and Monroe G. Sirken, Ph.D.

U. S. Department of Health, Education, and W7elfare

ADDRESS: (E.R.K.) Children’s Bureau, Department of Health, Edimcation, and \Velfare, \Vashingtomi 25, D.C.

PEDIAmlimes, Jtmlv 3961

CONTRIBUTOR’S

SECTION

C

YSTIC FIBROSIS of the pancreas is recog-nized as a generalized hereditary

dis-ease characterized by dysfunction of many

of the exocrine glands. Tile clinical findings are vell documented in tile literature.14

Much less is known about the incidence28 and prevalence of this disease because of the relatively recent recognition of it as a specific entity and the wide range of its clinical manifestations and also because of past difficulty in establishing a diagnosis

based on sound laboratory evidence.

Recently the Children’s Bureau, Depart-ment of Health, Education, and Welfare, in co-operation with the National Office of Vital Statistics, planned a series of studies designed to obtain information on the mci-dence and prevalence of diagnosed cases. A national survey of hospitalized cases, con-ducted in January and February of 1959,

was the first of tile series. From the survey information, estimates of the number of pa-tients with cystic fibrosis cared for in

hos-pitals, the ntmmber of discharges for these

patients, and the number of discharges that

were due to death, were derived for each of the years 1952 through 1957. These estimates together with a discussion of the survey de-sign and procedures and their effect on the findings were presented in a previous re-port.9

As described previously, nonpsychiatric

hospitals in tile continental United States

were sampled from a listing of hospitals

in the 1958 Directories of the American

Hospital and of the American Osteopathic Hospital Associations. The sample in-eluded all 296 hospitals approved for pedi-atric residency and a stratified sample of 320 other hospitals selected with probability roughly Pr0P0rtinate to the number of hospital beds. Queries to these iiosl)itals brought responses from which the follow-ing national estimates were derived: In

1957 approximately 2,500 patients with a

diagnosis of cystic fibrosis were discharged from hospitals. Slightly less than half (1,180) of these patients were reported by hospitals approved for a pediatric residency. The number of discharges for that year totaled about 3,200, indicating repeated

hospitalization for many patients. Fourteen

per cent, or about 360 patients, were dis-charged by death. From 1952 to 1957 the number of hospitalized patiemits with the

(lisease increased by about 50%. The numl)er of discharges showed a corresponding

in-crease. Tile estimated number of (leaths in

1957 was approximately 20% greater than

the estimated number in 1952, but this

dif-ference may he attributable to sampling

error.

The present report covers information

gathered in the same survey, bimt it is con-fined to an analysis of some characteristics

of the 2,500 patients estimated to have been

hospitalized for cystic fibrosis in 1957. Data

(2)

patients were analyzed according to whether the patient was discharged from a pediatric residency or other type hospital. Hospitalization rates for patients by geo-graphic region, fatality data and informa-tion on repeated hospital admissions were

also analyzed.

Heretofore, information about age, sex and mortality distribution of patients with

cystic fibrosis has been based on studies

of relatively small groups of patients seen in a few leading clinics in the coun-tr24’ 10-12 The present paper reports on

data that for the first time are based on a sample of hospitals distributed throughout the nation.

METHODS

In the earlier report estimates of the num-ber of persons, discharges and deaths were based on responses from hospitals giving complete and consistent data for the

6-year period, 1952-1957. Sample data were

then inflated by appropriate ratios to give national estimates. The distributions pre-sented in this report are derived from

sam-TABLE I

‘tMpLING Emuoom6s FOR ESTIMATED NUMBER OF

ITmF:xTs WITh CYSTIC FIBitosms, BY

GE0-Gn%Pmmmc REomoNs AND BY AGE GRoups

.

Region. and Age

(;T0UJ8 . 1108J)ztals (no.) . Etzmate1 Number of Patients Sampling rrror of Estimate All hospitals Totalpatient.s Total deaths Regiomms Northeast North Central South \Vest

Age gros (yr)

1 1- 4 .5- 9 10-19 >19 616 616 189 1.58 176 93 616 616 616 616 616 2,500 860 737 969 563 231 778 743 609 S5 115 10 10 10 0 0 10 15 15 15 30 80

pie data for the year 1957 and are similarly adjusted to compensate for both nonres-pondent hospitals and those reporting in-complete and inconsistent information for that year. Sample data are based on reports

from 93% of the hospitals queried.

Estimates presented in this paper are sub-ject to sampling errors, as they are based on reports made by a sample of hospitals. In the earlier paper it was shown that for the total sample of hospitals the sampling error was about 10% for both the estimated num-ber of patients with cystic fibrosis and the number of deaths from the disease in 1957.

For hospitals exclusive of those approved for pediatric residency, the sampling error was approximately 20% for patients and 30% for deaths. Table I shows approximate sam-pling errors, in per cent, for the more im-portant estimates presented in this report. The chances are about 95 in 100 that differ-erences due to sampling variability between the estimate based on the sample of hospi-tals and the figure that would have been obtained from a survey of all hospitals are less than twice the sampling error.

FINDINGS

On the basis of survey data, it is esti-mated that about 2,500 patients were dis-charged from hospitals in the nation in 1957. This estimate excludes multiple dis-charges of the patient from the same hospi-tal during the year but makes no provision for excluding duplicate reporting of the same patient from more than one hospital.

Geographic Distribution

About 70% of the estimated 2,500 patients with cystic fibrosis were discharged from hospitals located in the Northeast and

(3)

dis-ESTIMATED NUMBER AND PERCENTAGE DISTRIBUTION OF PATIENTS WITh CYsTIc FIBRosIS BY TYPE

OF HOSPITAL AND BY AGE GROUPS

Age GroUps HospitaLs (no.) ___________ hospitals (%)

----

----

-All

Residency Other

:i:;

L_:

<1 1- 4 5- 9 10-19 >19 Total 778 743 609 55 I 15

l,500

35

416

84

I 13

15

1 ,180

46 37 35 14 100 I ,30 31.1 9 .7 Q4 .4 10. 4.6 100.0 9 .8 35.3 4 .0 9.6 1.3 bOo 3 .3 24.6 10.7 7.6 100.0 SURVEY OF CYSTIC FIBROSIS

TABLE II

PERCENTAGE DISTRIBu’rIoN OF THE ESTIMATED

Nuii-BER OF PATIENTS WITH CYSTIC FIBROSIS, BY

TYPE OF HOSPITAL AND BY REGION

Regions

Distribidion of Patients (%)

All Ho,-pitaL Pediatric R Hospitals Other Hospitals Northeast NorthCentral South West Total(%) 9.5 38.8 2.5 9. 100.0 30.7 14.9 100.0 8.3 48. 19. 4.3 100.0

Total (no.) (,500) (1,180) (1,30)

charged from the latter group were from the North Central region and as few as 4% in the West.

Age Distribution by Type of Hospital

About 85% of all hospitalized patients with cystic fibrosis were children under 10 years of age (Table III). Almost a third (778) of the group were infants. An esti-mated 115 patients (about 5%) were in the

age group 20 years and over. The age dis-tribution of patients was approximately the same for both types of hospitals queried, except for the oldest age group. Almost 8% of patients reported by non-pediatric res-idency hospitals were aged 20 years or

more, whereas in pediatric residency hos-pitals there were only 1% in that age proup.

Rates for Hospitalization

For every 100,000 children in the popula-tion who were under 21 years of age, roughly 4 were hospitalized for the disease

during 1957. As the age of the children

in-creased, the ratio of hospitalized cases to the population in the corresponding age group decreased. For every 100,000 infants liveborn in 1957, approximately 18 were hospitalized for cystic fibrosis#{176}; about 8 per 100,000 in the child population under 5 years of age were hospitalized; for children 5 to 9 and 10 to 20 years of age, the rates

were about 3 and 1, respectively (Table IV). There was a significant regional variation

in rates for hospitalization due to cystic

fi-brosis. Whereas for both the Northeast and North Central regions there were about 5 patients under 21 years of age hospitalized

per 100,000 children under 21 years in the

total population, there were only about 2 cases per 100,000 population in the same age group for both the South and the West (Fig. 1).

For each of the four regions, the ratio of hospitalized patients with cystic fibrosis to child population was highest in tile age group under 5 years. In the Northeast 11 children were hospitalized for cystic fibrosis for every 100,000 in the population under 5 years of age; in the North Central region

* In 1957 the number of live births adjusted

for under-registration was 4,308,0O0.’

(4)

R.egu)ns Age Groups

(yr) (‘it’ilian Population

Patients

(no.)

Rate per ffXikX.

Population

TABLE IV

ESTIMATED NUMBER OF PATIENTS UNDER 21 YEARS OF AGE HOSPITALIZED FOR CYSTIC FIBROSIS PER

100,000 ESTIMATED POPULATION UNDER 21 YEARS OF AGE, BY REGIONS AND AGE GROUPS

AllRegions <21 65,294,000* 2,413 3.7

< 5 19,153,000 1,521

.5- 9 17,991,000 609

10-2() tS,bSO),000) 283

7.9 3.4

1.0

Northeast <21 14,811,000t 689 4.6

< 5 4,299,000

5-20 bO),.512,000

488 201

11.4 1.9

North (‘entral <21 19,166,000f 938 4.9

< 5 5,755,000

5-20) 13,411,000

565

373

9.8

.8

Soliti) <21 21,847,000t 556

< .5 6,180,000

5-20 15,667,000

338

218

5.3

1.4

Vest <21 9,471,000t 230

< 5 2,919,000

5-20 6,552,000

130

100

4.4

1.5

* ITrlite(l States Bureau of Census, Current Population Reports, Population Estimates, Series P-25, No. 193, February 16, 1959, Table 3, p. 12.

t Ibid., Population Estimates, Series P-25, No. 194, February 16, 1959, Table 1, p. 4. (Population estimates for age groups 5-9 and 10-20 years not available by regions.)

Differences betweell hospital rates in each of the high regions (Northeast and North Central) and each of the low regions (South and West) are significantly different at the 0.05 probability level.

tilere were 10 children; in the South, 5, and in the West, 4 children.

Fatality

About 14% (360) of all patients hospitalized for cystic fibrosis were discharged by death. Table V shows that among infants the case fatality rate was only slightly higher than among older children. Approximately 18 infants with the disease died for every 100

hospitalized for the disease. Among

chil-dren in age groups 1 to 4 years and 10

years and older, there were about 12

fatali-ties in each age group for every 100 hospi-talized; among children 5 to 9 years of age

the case fatality rate was 14. About 40% of

all fatal cases were among infants, almost 25% were among children in the age group 1 to 4 years and about the same proportion in the group 5 to 9 years.

Up to 10 years of age the number of fatal cases among females slightly exceeded the number among males, even though the total number of cases (alive and dead) in each age group among males exceeded that of females according to survey data (Table VI).

Repeated Hospital Discharges

Of the 2,500 patients discharged from hospitals in 1957, approximately 35% had

(5)

hospi-Rate per 100, 000 estimated population under 21 years

4.6 - 5.0

t\\\I

2.0 - 2.5

FIG. 1. Distribution of patients under 21 years of age hospitalized for cystic fibrosis per 100,000 population under 21 years of age, by regions of the United States, in 1957. (Source, Table 1V)

CASE FATALITY RATES FOB i’ATIENTS HOSPITAI.IZEI)

FOR CYSTIC FIBIt0SIS, BY AGE GROUPS

Age Group Cases i)eaths Deaths per

(yr) (no.) (,io.) 100 Cases

<1 778 lW 18.

1-4 743 88 11.8

5-9 609 85 14.0

>9 370 45 12.2

Total 2,500 360 14.4

SURVEY OF CYSTIC FIBROSIS

tal reporting the case either in the same

year or in some prior year. About 6% of all

patients were discharged at least three times during 1957 from the same hospital, and a few of these as frequently as seven times in the single year. Some patients may have been discharged from more than one hospital during 1957, but this type of in-formation was not available from the survey data.

For patients discharged by death, re-peated hospital admissions were

consider-ably more frequent than for those

dis-charged alive. Fifty per cent of the 360

pa-tients who died had multiple hospital admissions in contrast to only about 33% of the 2,140 patients discharged alive (Table VII). The association of repeated discharges from the hospital and death is more striking when examined according to age groups.

Thirty-eight per cent multiple admissions

among the 142 fatal cases in infants is in contrast to 22% of the 636 infants discharged alive. In the age group 1 to 4 years, 57% of the 88 patients with fatal cases were

ad-mitted two or more times to the same hos-pital, whereas among 655 children

dis-charged alive, about 34% were hospitalized

two or more times. Similarly, in the age group 5 years and older, 59% of the 130 pa-tients with fatal cases were hospitalized two or more times in contrast to about 40% of

tile 849 patients discharged alive.

COMMENT

The analysis of characteristics of patients with cystic fibrosis, discharged from

(6)

TABLE VI

ESTIMATED NUMBER OF MALES AND FEMALES WITH CYSTIC FIBIt0SIS DISCHARGED AuvE

AND BY DEATH FROM HOSPITALS, BY AGE GROUPS

Total AgeGroup

(ii’S) Males Females

Alive

-_____

---Males Females

Dead

---______

Males Female8

<1

1-4

5-9 >9

Total

433

378

343

221

1 ,375

345

365 266 149

1,125

367 337

314

182

1,200

269 318 210

143

940

66

41

29

39

175

76 47

.56

6

185

tals in the United States in 1957, provides the following two kinds of information: 1) data on the regional distribution of cases in the country that need corroboration and an investigation of variables contributing to that distribution and 2) data on age and

fa-tality distributions and the number of

ad-missions to the hospital per patient, which roughly confirm current concepts.

In the use and interpretation of the find-ings, cognizance must be taken of the fact that all frequency distributions are based on estimates of the number of hospitalized patients and of the number of deaths from the disease among these patients during a single calendar year. Although discussions with physicians indicate that most patients with cystic fibrosis are hospitalized at least once during the period of diagnostic study, this procedure varies. The extent to which patients are never hospitalized for the dis-ease is not known. It is known that many patients are given a diagnosis and are treated for the condition in the clinic with-out admission to the hospital. During any single year there will be many previously diagnosed cases that do not require hospi-talization. Still other cases are completely missed.

Regional variation in rates for hospitali-zation because of cystic fibrosis and the dis-tribution of hospitalized cases may not be indicative of real differences in the preva-lence of the disease. Further studies, based on a sample of all cases in the total popu-lation, are needed.

Differences in medical awareness of the disease and of diagnostic facilities may be influencing factors contributing to what appears to be a higher prevalence in the Northeast and North Central regions of the United States in contrast to a lower preva-lence in the South and West. Again, re-gions of apparent higher prevalence may reflect the movement of families having children with cystic fibrosis into regions where there has been medical specialization in the treatment of patients with the dis-ease. The survey questionnaire did not re-quest information on the usual place of resi-dence of families. According to one special-ist treating large numbers of cases, however, almost all patients under his care had al-ways resided within a two-to-three-hun-dred-mile radius of the hospital or clinic where treatment was being given.

The wide variation in the regional dis-tribution of cases in “other” hospitals in contrast to that in pediatric residency hos-pitals may be due to differences in the type of hospital services available in different areas of the country for the diagnosis of the disease or, again, may reflect sampling van-ability.

Whether or not environmental factors

play any role in contributing to the higher

prevalence in one section of the country in contrast to another is not known. Carter’4 denies the influence of environmental fac-tors; Andersen’s is not willing to eliminate their possible role.

(7)

Age Group

(1ff)

Discharges Total Alive Dead

(no.) -

-%

a contributing factor in the contrasting re-gional variation. Information on the race and nationality of patients was not avail-able from the survey. Based on clinical ex-penience, however, it is generally accepted that cystic fibrosis is chiefly a disease of the Caucasian 12, 1618 It is rarely seen in

the American and until recently had not been reported in the African Negro or among members of the Mongolian or

oriental races. Senecal et al.20 observed two cases in African infants at Central African Hospital, Dakar; Salam” reported a case in an Arab child. The lower incidence of the disease among Negroes may have contrib-uted to the lower prevalence in the South, as in that region about 20% of the

popula-tion (1950 census) was classified as Negro

as contrasted with 5% for the Northeast and

North Central regions and 3% for the

West.’2 Racial predilection does not explain the low prevalence in the West. The rela-tively large numbers of oriental persons and American Negroes, particularly in Califor-nia, are still too few to have an effect on the regional prevalence.”

Shwachman et al. observed a high pro. portion of the patients with cystic fibrosis from French Canadian, Irish and English stock who were seen in his clinic at Chil-dren’s Medical Center, Boston. Whether this is simply because tile population from which his patients were drawn contained a high

proportion of people of this stock23 would

need further investigation.

The age distribution of patients with cystic fibrosis discharged from hospitals in the nation roughly approximates that of clinic groups of patients reported on in the literature. Even though the number of

TABLE VII

PERCENTAGE DISTRIBUTION OF THE ESTIMATED NUMBER OF PATIENTS WITH CYSTIC FIBII0SIS l)ISnAlIwu AI.IvE AND BY DEATH FROM HOSPITALS IN 1957 BY NUMBER OF TIMEs EVER I)IscHAuG:o

FROM THE SAME HOSPITAL, BY AGE GROUPS

Patients Discharged from Hospital*

No. % No. % No.

All age groups 2 ,500 100.0 2,140 100 .0 360 100.0

1

>1

65.0

35.0

67.5

32.5

49.7

50.3

<1 778 100.0 636 100.0 142 100.0

1 >1

75.1 24.9

78.0 22.0

62.0 38.0

1-4 743 100.0 655 100.0 88 100.0

1

>1

63.7

36.3

66.4

33.6

43.2

56.8

>4 979 100.0 849 100.0 130 100.0

1

>1

57.9

42.1

60.5

39.5

40.8

59.2

* Information on the number of discharges per patient in 1957 was either not reported or the accuracy of this

information was doubtful for about 10% of patients. Tabulated frequencies on repeated hospital discharges are,

(8)

cases in the age group 20 years and over may be an overestimate due to misdiag-nosis, survey findings corroborate current clinical data showing that cystic fibrosis is not the uniformly fatal disease of infancy or

early childhood that it was formerly

con-sidered to “, 2427 Such beliefs

origi-nated from the early experience of clinicians who differentiated the disease entity over

two decades ago in studies of necropsy find-528, 29 A review of survey data for

pa-tients aged 20 years and over showed that for about two-thirds of them (almost 3% of

the 2,500 cases) there was no apparent

reason for questioning the correctness of the diagnosis. For example, the report from one reliable hospital source indicated that

cystic fibrosis was diagnosed at necropsy in

a female aged 41 years.

At Babies Hospital, New York, almost 20% of 550 patients seen over a 19-year period survived beyond the age of 10 years; 85 were still living at the time of the report, and the oldest was aged 24 years.25’26 Cald-well and McNamara” presented the case of a 24-year-old woman with diagnosis proven on the basis of present-day standards. Shwachman and KulczyckP’ reported on several adult patients, one of whom was a 33-year-old father of three children, and Marks and Anderson32 discussed the case of a patient aged 46 years “believed to have fibrocystic disease of the pancreas.” Because of improved diagnostic methods, the more frequent recognition of mild cases, and the application of effective therapeutic meas-tires, it is possible that an increasing number of patients with cystic fibrosis may be ex-pected to live to adulthood.

The reported ages at which patients died in hospitals participating in the survey fur-ther confirm clinical observations that, on the average, patients are surviving longer than formerly. In 1955 Shwachman et al.

reported on the age at death of 99 patients

(

exclusive of meconium ileus) studied at Children’s Hospital, Boston, over a 13-year period. Almost 45% of the Boston group died during infancy and another 45%

be-tween the ages of 1 and 5 years. The re-maining number of fatal cases were in the age group 5 years and over. In the hospital

survey 40% of all fatal cases were among

infants and only about 25% were in the age group 1 to 4 years. The case fatality rate for infants with cystic fibrosis was only slightly higher than rates for children in older age groups.

Observation of large numbers of patients in clinics has indicated that cystic fibrosis occurs with about the same frequency in males and Based on survey

data, the number of males hospitalized for cystic fibrosis in 1957 exceeded the number of females except among fatal cases under 10 years of age. Differences in the sex dis-tnibution of both cases and deaths might well be due to sampling error.

The analysis of the death certificates for 1958 for the nation reveals findings broadly consistent with those of the survey data. For the first time, deaths due to cystic fibrosis were coded separately instead of being included in the residual category, “other diseases of the pancreas.” These data are now available in the annual publication of the National Office of Vital Statistics.” Of the 550 deaths attributed to the disease in 1958, almost 83% (456) occurred in in-stitutions. Slightly less than 5% were

clas-sified as in Negroes, and only two deaths were attributed to “other” races. The age distribution approximated that shown for fatal cases by survey data. Of the 550

deaths, 33% were among infants, 34% among children in the age group 1 to 4 years and

(9)

as-certain whether or not there might be a relationship between sex and greater risk of death in very young children.

Hospitalization of patients with cystic fibrosis is not necessarily indicative of the seriousness of the child’s condition. Fre-quently inpatient care is needed at the time a diagnosis is being established or con-firmed. This is particularly true for patients who live at a distance from the hospital. Also, hospitalization for establishing or con-firming a diagnosis or for treatment is de-pendent to some extent on the availability and quality of outpatient care, on the policy of the hospital, or on preference of the phy-sician caring for the child. In the present

study about two-thirds of the patients were

discharged only one time from the hospital reporting the case. The remaining third had repeated discharges from the same hospital. As might be expected, the proportion of patients having repeated admissions to the hospital increased progressively from those in the youngest age group to those in the oldest. This trend was more pronounced among cases terminating in death and por-trays the typically progressive severity of

this disease as differentiated from most other childhood diseases.

Clinical experience shows that the domi-nant feature in cystic fibrosis is the pulmo-nary 619, 25, 29, 33 Di Sant’

16 and di Sant’ Agnese and

Vi-daurreta’ observed that pulmonary insuffi-ciency occurred in almost all 550 patients with cystic fibrosis observed at Babies Hos-pital in New York. The respiratory disease was severe in the average case and ac-counted for over 90% of the 300 or so deaths. Fatal termination showed individual vania-tion but, on the average, death occurred within 2 or 3 years following the onset of the pulmonary involvement.

SUMMARY

Up to the present time accumulated cx-penience with cystic fibrosis has been based on intensive study of relatively small groups of patients treated or seen for the disease in

a few leading clinics in the country. This paper presents, for the first time, charac-tenistics of patients with cystic fibrosis from a sample of hospitals throughout the con-tinental United States. Estimates are based on sample data for 1957. The more impor-tant findings follow.

Almost 70% of the estimated 2,500 pa-tients with cystic fibrosis were discharged from hospitals in the Northeast and North Central regions of the United States. Only 9% of all cases were reported by hospitals in the West.

Among hospitals approved for pediatric residency, discharges were more equally distributed between the four regions than among other types of hospitals. Almost half of the estimated 1,320 patients in non-pedi-atnic residency hospitals were in the North Central region alone.

Eighty-five per cent of hospitalized pa-tients were under 10 years of age; approxi-mately 30% were infants. About 5% were aged 20 years or more. In hospitals ap-proved for pediatric residency, slightly over 1% of patients were aged 20 years or more, whereas, in other types of hospitals, 8% were were in the oldest age group.

For every 100,000 children in the

popula-tion under 21 years of age, about 4 were hospitalized for cystic fibrosis in 1957. Among children under 1 year of age, the rate for hospitalization due to cystic fibrosis

was 18 per 100,000 live births in 1957; the

rate was 8 per 100,000 in the child popula-tion under 5 years of age; for children 5 to 9 and 10 to 20 years, rates were about 3 and 1 respectively.

There was a significant regional variation in rates for hospitalization because of cystic fibrosis. Whereas in both tile Northeast and North Central regions there were about 5 patients under 21 years of age per 100,000 in the population under 21 years, there were only about 2 cases per 100,000 popula-tion in the same age group in both the South and West.

(10)

PUBLIC HEALTH

population was highest in tile age group under 5 years. In the Northeast there were 11 children for every 100,000 in the popu-lation under 5 years of age hospitalized for cystic fibrosis; in the North Central region there were 10 children; in the South there were 5, and in the West, 4 children.

Almost 15% of the 2,500 hospitalized pa-tients with cystic fibrosis were discharged

by death. Of the 360 patients with fatal cases, about 40% died during infancy; a!-most 25% died at ages 1 to 4 years; about the same proportion died at ages 5 to 9

years.

The ratio of deaths from the disease per 100 cases hospitalized during the year was

18 for infants, 12 for children in the age

group 1 to 4 years, 14 for children 5 to 9

years, and 12 for those aged 10 years or

more.

According to survey data, a larger num-ber of males than females in each age group

were hospitalized for cystic fibrosis. Among fatal cases under 10 years of age, however, the number of females slightly exceeded the

number of males.

More than one-third of the 2,500 pa-tients had been previously discharged from tile hospital reporting the case either in the same year or in some prior period. About 6% of all patients were discharged from the same hopsital at least three times during

1957.

Among the 360 patients discharged by death, over half were discharged two or more times during their lifetime from the hospital reporting the case. Among the 2,140 discharged alive, only about a third were reported to have multiple discharges from tile same hospital.

REFERENCES

1. Andersen, D. H. : Cystic fibrosis of the

pan-creas. J. Chron. Dis., 7:58, 1958.

2 I)i Sant’Agnese, P. A., and Vidaurreta, A. M.: Cystic fibrosis of the pancreas. J.A.M.A.,

172:2065, 1960.

3. Fibrocystic Disease of the Pancreas: Report

of the Eighteenth Ross Pediatric Research

Conference held in Iowa City, Sept.

30-Oct. 1, 1955. Columbus, Ohio, Ross Labora-tories, 1956.

4. Shwachman, H., Leubner, H., and Catzei, P.:

Mucoviscidosis, in Advances in Pediatrics,

Vol. 7, edited by Levine, S. Z. Chicago, Yr. Bk. Pub., 1955, pp. Z49-323.

5. Andersen, D. H., and Hodges, R. G. : Celiac

syndrome: V. Genetics of cystic fibrosis of the pancreas with a consideration of etiol-ogy. Amer. J. Dis. Child., 72:62, 1946.

6. Bodian, M. : Fibrocystic Disease of the

Pan-creas. New York, Grune, 1953.

7. Goodman, H. 0., and Reed, S. C.: Heredity

of fibrosis of the pancreas: possible muta-tion rate of the gene. Amer. J. Hum. Genet.,

4:59, 1952.

8. Steinberg, A. G., and Brown, D. C. : On the

incidence of cystic fibrosis of the pancreas.

Amer. J. Hum. Genet., 12:416, 1960.

9. Sirken, M. C., et al.: A national hospital

sur-vey of cystic fibrosis. Public Health Rep.,

74:764, 1959.

10. Craig, J. M., Haddad, H., and Shwachman,

H. : The pathological changes in the liver

in cystic fibrosis of the pancreas. Amer. J.

Dis. Child., 93:357, 1957.

I 1. Di Sant’Agnese, P. A.: Recent observations

on pathogenesis of cystic fibrosis of the

pancreas. PEDIATRICS, 24:313, 1959. 12. Shwachman, H., and Kulczycki, L. L. :

Long-term study of one hundred five patients

with cystic fibrosis. Amer.

J.

Dis. Child.,

96:6, 1958.

13. U. S. Dept. of Health, Education, and

Wel-fare. National Office of Vital Statistics. Vital

Statistics-Special Reports. 50: 16, 1959,

Table 1.

14. Carter, C. 0. : Familial incidence, in Bodian, XI. : Fibrocystic Disease of the Pancreas.

New York, Grune, 1953, Chapt. 3.

15. Andersen, D. H. : Cystic fibrosis of the pan-creas, in Pediatrics, Ed. 12, edited by Holt,

L. E., Jr., and McIntosh, R. New York, Appleton, 1953, pp. 509-515.

16. Di Sant’Agnese, P. A. : Cystic fibrosis of the

pancreas. Amer. J. Med., 21:406, 1956.

17. Lowe, C. U., May, C. D., and Reed, S. C.:

Fibrosis of the pancreas in infants and

chil-dren : a statistical study of clinical and

hereditary features. Amer. J. Dis. Child., 78:349, 1949.

18. U. S. Dept. of Health, Education, and

Wel-fare. National Office of Vital Statistics. Vital Statistics of the United States, 1958, Vol. 2, Washington, D.C., Government Printing

Office, 1960, Tables 73, 80.

19. Zuelzer, W. W., and Newton, W. A., Jr. : The

pathogenesis of fibrocystic disease of the

(11)

reference to the pulmonary lesions.

PimIAT-RICS, 4:53, 1949.

20. Senecal, J., et al.: Deux cas de fibrose kystique

du pancreas chez l’enfant Africain r#{233}alisant

le syndrome du kwashiorkor. Bull. Medic

A.O.F., 11:95, 1954.

21. Salam, M. Z. : Cystic fibrosis of the pancreas in an oriental child. Ann. Paediat. (Basel),

190:2,52, 1958.

22. U. S. Department of Commerce. Bureau of

Census. Statistical Abstract of the United

States, 1957, Ed. 78, Washington, D.C.,

Government Printing Office, Tables 26, 27.

23. U. S. Department of Commerce. Bureau of

Census. United States Census of Popula-tions: 1950-Special Reports, Vol. 4,

Wash-ington, D.C., Government Printing Office,

1954, Table 13.

24. Di Sant’Agnese, P. A. : Fibrocystic disease of

the pancreas: a generalized disease of exo-crine glands. J.A.M.A., 160:846, 1956.

25. Di Sant’Agnese, P. A., and Andersen, D. H.:

Cystic fibrosis of the pancreas in young

adults. Ann. Intern. Med., 50: 1321, 1959. 26. McIntosh, R.: Cystic fibrosis of the pancreas

in patients over ten years of age. Acta

Paediat., 43(Suppl. 100):469, 1954.

27. Royce, S. W. : Cardiac and pulmonary

corn-plications in fibrocystic disease of the pan-creas, in Fibrocystic Disease of the Pancreas,

Report of the Eighteenth Ross Pediatric

Re-search Conference held in Iowa City, Sept.

30-Oct. 1, 1955. Columbus, Ohio, Ross

Lab-oratories, 1956. pp. 79-86.

28. Andersen, D. H. : Cystic fibrosis of the

pan-creas and its relation to celiac disease: a

clinical and pathologic study. Amer. J. Dis.

Child., 56:344, 1938.

29. Farber, S. : Pancreatic function and disease

in early life: V. Pathologic changes

asso-ciated with pancreatic insufficiency in early

life, Arch. Path., 37:238, 1944.

30. Di Sant’Agnese, P. A. : Cystic fibrosis of the pancreas. Conn. Med. J., 22:445, 1958.

31. Caldwell, D. M., and McNamara, D. H.:

Fibrocystic disease of the pancreas and dia-betes in an adult with unusual pulmonary manifestations. Calif. Med., 89:280, 1958.

32. Marks, B. L., and Anderson, C. M. :

Fibro-cystic disease of the pancreas in a man

aged 46. Lancet, 1:365, 1960.

33. Di Sant’Agnese, P. A. : The pulmonary

mani-festations of fibrocystic disease of the pan-creas. Dis. Chest, 27:654, 1955.

GROWTh DLcNosIs: Selected Methods for

Interpreting and Predicting Physical De-velopment from One Year to Maturity,

Leona M. Bayer and Nancy Bayley. Chi-cago, University of Chicago Press, 1959,

241 pp., $10.00.

The authors of this important book gained their unique experience and data in longitud-ma! studies of the growth of healthy children

in California. They here present to us chosen

fruits.

Published data on child growth rates are so rare that when they appear in a work of this kind we are particularly anxious to learn how we may best use the benefits of such careful labors. The book does live up to our expecta-tions by being practical and by describing a battery of measures and ratings of the size, shape and maturity of children. The most

corn-plicated measure is a roentgenogram of the

hand and wrist, and the most bothersome is a body-build photograph-hardly too much to ask when confronted by a “growth problem.” The aim is for physicians and others to be able to define a child’s status of maturity and growth

relative to specific norms-thus “growth

diagno-sis” and the intriguing possibilities of

predic-tion of adult size.

There is a particularly interesting chapter describing the authors’ method of assessing the physical masculinity or feminity of the child; but the illustrations denoting the method of rating sexual maturation are not quite up to the general high standard of the book. Practi-cal throughout, over half the book is made up of actual case histories plotted onto gain or

increment charts (a rare jo’), with photographs,

and lucid accompanying descriptions and illus-trations showing various growth patterns of wide diversity, and over long periods of time.

This book will probably not be most useful

to the buss’ “general pediatrician”; but there is

no question that it will for many ‘ears be a

valued and well-thumbed reference book for all who are especially interested in child growth,

and for human biologists.

The organization and the publication are of

a high standard, from the printing to the

fas-cinatmg dust-sheet.

(12)

1961;28;128

Pediatrics

Elizabeth R. Kramm, Marian M. Crane, Morton L. Brown and Monroe G. Sirken

UNITED STATES

FIBROSIS DISCHARGED FROM HOSPITALS IN 1957: ESTIMATES FOR THE

PUBLIC HEALTH: CHARACTERISTICS OF PATIENTS WITH CYSTIC

Services

Updated Information &

http://pediatrics.aappublications.org/content/28/1/128

including high resolution figures, can be found at:

Permissions & Licensing

http://www.aappublications.org/site/misc/Permissions.xhtml

entirety can be found online at:

Information about reproducing this article in parts (figures, tables) or in its

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(13)

1961;28;128

Pediatrics

Elizabeth R. Kramm, Marian M. Crane, Morton L. Brown and Monroe G. Sirken

UNITED STATES

FIBROSIS DISCHARGED FROM HOSPITALS IN 1957: ESTIMATES FOR THE

PUBLIC HEALTH: CHARACTERISTICS OF PATIENTS WITH CYSTIC

http://pediatrics.aappublications.org/content/28/1/128

the World Wide Web at:

The online version of this article, along with updated information and services, is located on

American Academy of Pediatrics. All rights reserved. Print ISSN: 1073-0397.

References

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