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RHEUMATIC FEVER / RHEUMATIC

HEART DISEASE

PHILIPPINE SOCIETY OF PEDIATRIC CARDIOLOGY

Philippine Pediatric Society


Philippine Society of Pediatric Cardiology
Department of Health
Philippine Foundation for the Prevention and Control
of RF/RHD

Contributors

Santiago V. Guzman, M.D.


Wilberto L. Lopez, M.D.
Edgardo E. Ortiz, M.D.
Asuncion A. Reloza, M.D.
Luis M. Mabilangan, M.D.

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rheumatic fever/rheumatic heart disease cpm 3RD eDITION

Algorithm for the Management of Cases of Acute


Rheumatic Fever
1
Patient aged 5 to
15 years with joint
swelling, pain and
high temperature

Check whether

Keep patient
under
observation for
2 weeks
10

Meets the
Jones'
Criteria?

the streptococcal
infection was
treated

Classify the
case

11

13

12

Carditis?

Discharge
14

180

Send patient
to
hospital

If there is no
hospital, treat with
inflammatory drugs.
Let patient rest and
begin secondary
prevention.

FIGURE 1

15

Meets the
Jones'
Criteria?

Make the patient


rest and treat with
benzathine penicillin
until physician can
see patient.

Medical
Examination

Patient
sees a physician
within 48
hours?

Send for
consultation

Send for
registry

CPM 3RD EDITION

rheumatic fever/rheumatic heart disease

Algorithm for the Secondary Prevention


of Rheumatic Fever
1

3
by mouth for
10 days

Begin or
continue preventive
administration of
IM penicillin
every month

Continue preventive
regimen with a daily
dose erythromycin
250 mg twice daily

on evolution

11

10
Y Has reached
18 years
of age?

Keep an eye

Y Suspend the
prevention
treatment

13

Over 5 years
since acute
attack?

12

Maintain
preventive
regimen for
whole life

Keep an eye on
evolution

Continue preventive
regimen until 5
years have elapsed

14

15

Functionally
stable?
16

Has RHD?

erythromycin

Treatment with

Hypersensitive
to penicillin?

Patient with ARF in the


past and/or with RHD
seen by the physician for
secondary prevention

Continue
preventive
regimen

Send to
specialist for
evaluation

FIGURE 2

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rheumatic fever/rheumatic heart disease cpm 3RD eDITION

Algorithm for the Diagnosis and Treatment of Streptococcal Sore Throat


1

Child 5 to 15 years
of age with an acute
infection of the upper
respiratory tract
2

Consultation
3

Examination by
physician, nurse or
trained auxiliary

Apply the clinical


and/or bacteriological
diagnosis criteria
5

A single dose of
benzathine penicillin
or 10 days of oral
penicillin

Treatment
with
erythromycin
for 10 days

Allergic to
penicillin?

Health
education

10

Positive throat
culture
(Typical Strep
throat)

Discharge

FIGURE 3

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CPM 3RD EDITION

rheumatic fever/rheumatic heart disease

Manual of Operation of the Primary and Secondary Prevention of Rheumatic Fever-Rheumatic Heart Disease
INTRODUCTION
The RF/RHD Registry is one major component of the Program
for the Prevention and Control of RF/RHD, being spearheaded
by the Philippine Foundation for the Prevention and Control
of RF/RHD in coordination with the Department of Health
and other professional organizations and societies, such as the
Philippine Heart Association, Philippine Society of Pediatric
Cardiology and the Philippine Pediatric Society.
The RF/RHD Registry will serve at least two purposes. Firstly,
it will verify referred cases of RF/RHD, which will eventually
permit a long-term follow-up study of verified cases of RF/
RHD. Secondly, it will act as means through which secondary
prophylaxis will be administered.
SCREENING AND MANAGEMENT OF CASES OF ACUTE
RF
If the patient is a child aged between 5 and 15 who is suffering
from joint pain and high temperature, he must be sent to a
physician as soon as possible. If no physician is available,
the patient is advised to rest and symptomatic treatment is
given.
The physician should examine the patient before 48 hours
have elapsed and check whether he presents the symptoms
mentioned in the Jones' Diagnostic Criteria (Tables 1-3 and
Figure 1). If he does not, he should be kept under observation
for two weeks and then discharged if the diagnosis cannot be
established within the period. If the Jones' criteria are fulfilled,
the physician will check whether the streptococcal infection
has been treated and will classify the case as to presence or
absence of carditis.
If the patient is not suffering from carditis, he can be treated
by a general practitioner but whenever possible, he should
be admitted to a hospital as an in-patient for confirmation
of the diagnosis and for treatment. A patient with carditis
must always be admitted to a hospital for treatment and after
hospital discharge, must be on home rest for at least six (6)
weeks (Algorithm Fig. 1). Patient is also sent to a registry for
secondary penicillin prophylaxis (Algorithm Fig. 2).
Table 1: Diagnostic Criteria
Modified Jones' Criteria for Diagnosis of Rheumatic Fever
A. Major Criteria

B. Minor Criteria

1. Arthritis 1.
2. Carditis 2.

Fever
Arthralgia (pain in
joints without objective
findings)

3. Chorea 3. Increased sedimentation


rate and presence of C reactive protein or
leucocytosis
4. Erythema 4. Evidence of preceding

Marginatum beta-hemolytic strepto coccal infection
5. Subcutaneous 5. A previous history of

nodules rheumatic fever or the
presence of established
valvular disease
6. Prolonged P-R interval
in the ECG

Definite RF: Presence of two (2) major criteria, or of one (1)


major and two (2) minor criteria.
Historical Note: The Jones' Criteria for guidance in the
diagnosis of acute rheumatic fever were initially proposed by
T. Duckett Jones in 1944. Committees of the American Heart
Association subsequently modified (1955), revised (1965)
and now updated (1992). This modified criteria should only
be used if application of the updated criteria is not feasible
in the community.
Table 2: Jones' Criteria, Updated 1992
Major

Minor

Carditis
Minor
Polyarthritis Clinical
Chorea Fever
Erythema marginatum Arthralgia
Subcutaneous Nodules Laboratory
Elevated ESR
Positive CRP
Prolonged P-R interval

Evidence of previous Group A hemolytic Streptococcal


infection either:
Positive throat culture
Positive rapid streptococcal antigen test for Group A
streptococcal
Elevated ASO titer
Previous infection is indicated by increased anti- streptolysin
O or other streptococcal antibody and positive throat culture
for Group A streptococcus. Manifestations with a long latent
period, such as chorea and late-onset carditis, are exempted
from this last requirement.
Definite RF Diagnosis: Presence of two (2) major criteria, or one
(1) major and two (2) minor criteria. Plus evidence of previous
Group A -hemolytic streptococcal infection.
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rheumatic fever/rheumatic heart disease cpm 3RD eDITION

1. Arthritis: Joint symptoms may occur in as many as


75% of patients. These vary from a single joint pain,
to arthralgia and to arthritis with swelling, redness
and heating, mostly in the knees, ankles, elbows and
wrists-rarely in small joints with the characteristics of
being migratory. They disappear spontaneously without
leaving permanent deformities.
2. Carditis: Carditis is the most serious manifestation,
usually occurring in 40 to 50% of patients, which varies
from an equivocal diagnosis at onset to unequivocal
with an organic heart murmur, heart enlargement and
heart failure. Carditis is indicated by significant apical
systolic murmur, apical mid-diastolic murmur and
basal diastolic murmur as well as the friction rub of
pericarditis or a gallop rhythm. The patient may present
a chest pain, palpitations and dyspnea which may be
gradually increasing in variety.
3. Chorea: St. Vitus dance is less frequent, with the
characteristic involuntary movements which mainly
affect the hands and facial muscles, and disappear
during sleep (choreiform movements) with a marked
emotional lability.
4. Erythema Marginatum: Erythema Marginatum is a skin
manifestation which is seen in less than 10% of patients.
It usually affects the trunk because it is relatively rare,
it is often misdiagnosed clinically.
5. Subcutaneous nodules: Subcutaneous nodules are
small, non-tender, pea-sized nodules that appear
over the extensor surfaces of the joints. They are
uncommonly seen and when they do appear, it is often
in association with chronic rheumatic heart disease.
Table 4: Suggested Schedules of Anti-inflammatory
Therapy in Rheumatic Fever
Clinical Severity Treatment
Arthralgia or mild Analgesics only
arthritis, no carditis
Moderate or severe Aspirin, 90-100 mg/kg/day
arthritis; carditis with for 2 weeks; increased if
or without cardio- necessary, 60-70 mg/kg/
megaly, and without day for the subsequent
failure 6 weeks
*Carditis with failure; Prednisone, 40-60 mg/day,
with or without joint increased if necessary,
manifestation methyl prednisolone sodium
succinate IV in fulminating
cases; after 2-3 weeks.
Aspirin to be continued for
a month after
discontinuation of
prednisone

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Dosage of Prednisone:
Mild (without cardiomegaly) 40 mg/day
Moderate (with cardiomegaly) 60 mg/day
Severe (with cardiomegaly and hearth failure) 60
mg/day

Use ESR and ASO as criteria for evidence of progression of the


disease.
Prednisone to be tapered after 2-4 weeks based on patient
response.

From Philippine Foundation for the Prevention and Control of Rheumatic


Figure
A - Evidence
of a Recent Streptococcal Infection
Fever and
Rheumatic
Heart Disease

95%

100
PERCENT OF PATIENTS
WITH TITER .200 u./cc.

Table 3: Clinical Aspects of Rheumatic Fever and


Rheumatic Heart Disease

*Carditis:


80

90%
78%

60
40
20
0

ASO
ASO

only
or AH

ASO = Antistreptolysin O
AH = Antihyaluronidase
ASK = Antistreptokinase

ASO
or AH
or ASK

From Rheumatic Fever


Taranta, A. and
Markowitz, M. MTP
Press Limited Boston, 1981

A throat culture is not very helpful because by the time


signs and symptoms of acute rheumatic fever appear, the
culture is usually negative, especially if antibiotics have
been given for the preceding upper respiratory infection.
Streptococcal antibodies are more useful because they are
usually elevated by the time the rheumatic fever attack starts.
About 80% of patients with acute rheumatic fever have an
elevated antistreptolysin O (ASO) and the remaining 20% have
a rise in one or the other streptococcal antibody tests, (Figure
A).
Generally, streptococcal antibody levels begin to decline
within a month or two so that they are helpful only if the
patient is observed early in the course of the acute rheumatic
attack. Thus, in patients with insidious rheumatic carditis
that is discovered several months after onset, streptococcal
antibodies will usually have returned to normal levels. This
is also true in patients with Sydenham's chorea because this
manifestation may sometimes appear several months after the
streptococcal infection.
The ASO test is the best standardized and the most
frequently determined antibody test. The titers vary with

CPM 3RD EDITION

rheumatic fever/rheumatic heart disease

intensity of exposure to streptococcal infections which is


influenced by age and geographical area. Titers up to 2000 are
common in healthy school-age children so that only levels of
333 units or higher are considered abnormal.
*Markowitz and Gordis; W.B. Saunders, Philadelphia, 1972

Primary Prevention
Primary prevention is the treatment of upper respiratory
tract infection due to group A beta-hemolytic streptococci
to prevent an initial attack of acute rheumatic fever. Studies
have demonstrated that appropriate antibiotics, given early in
the course of streptococcal infection, essentially prevents the
development of rheumatic fever (Table 5).

For patients allergic to penicillin, oral erythromycin is an


accepted alternative. The recommended dose of erythromycin
is 250 mg 4 times a day. For children weighing less than 25 kg,
the recommended dose is 40 mg/kg of body weight per day
in 2-4 divided doses. The total daily dose should not exceed
1 gram.
SECONDARY PREVENTION OF RHEUMATIC FEVER OR
PREVENTION OF RECURRENCES
Secondary prevention will be prescribed for rheumatic fever
patients for all ages, but more frequently in school children
and young adults who have had one or more attacks of
rheumatic fever.

Primary prevention should be given equal importance as


secondary prevention because primary prevention has the
following advantages:

It is essential to check whether the patients are hypersensitive


to penicillin, a sensitivity test must be done before the first
injection.

1. Permits eradication of the disease;


2. Duration of treatment is brief;
3. Treatment is accepted because it concerns sick
individuals;
4. Prevents severe and irreversible heart damage from first
attack of RF;
5. Cost-effective if prevention is restricted to the major at
risk group - children aged 5-15 years compared to treating
cases of RHD for life.

* Those not hypersensitive to penicillin will be given a 21-28


days preventive dose of 1,200,000 u of benzathine penicillin
by the intramuscular route.
* In special circumstances or high-risk patients, the injection
may be given every 3 weeks (21 days).
* Those who are hypersensitive but not undergoing a
preventive regimen will have to be given an eliminatory
course of treatment initially with erythromycin by mouth
for 10 days: 125 mg four times a day for those under six years
and 250 mg four times a day for older patients.
* They will be maintained on Erythromycin 250 mg 2x daily,
as in the penicillin maintenance schedule.
* Patients who are not suffering from RHD and have only
had an attack of acute rheumatic fever will follow the same
treatment schedule for 10 years until they are 18 years of age,
whichever is longer (Algorithm Fig. 2).
* When they have fulfilled these two requirements, i. e. that
they should be 18 years of age or over and that 10 years
or more should have elapsed since the last acute attack,
prevention will be suspended and they will be kept under
surveillance.
* The preventive regimen will continue throughout life for
those with cardiac lesions, but care must be taken to ensure
that the condition remains stable. If symptoms of fatigue
appear and if there is a change of functional category, the
person concerned will again be sent to the physician for
examination.
* Patients who have undergone heart surgery will be included
in the registry to prevent bacterial endocarditis.
Rheumatic fever once diagnosed by appropriate criteria
is considered clinically active if any one of the following
features is found:

1. Joint symptoms
2. New organic murmurs
3. Changing heart size
4. Congestive heart failure (in the absence of long-standing

All physicians involved in child health care and primary


health workers such as municipal health officers, school
physicians and general practitioners should be encouraged
to detect and treat streptococcal pharyngitis (Tables 6-7 and
Algorithm Fig. 3).
Table 5: Recommended Treatment for Group A Streptococcal
Pharyngitis

Mode of

Antibiotics
Administration



Benzathine Intramuscular

Penicillin



injection)



Phenoxymethyl
Oral
Penicillin (V)

Dose
-1,200,000 units for
adults & children more
than 30 kg
600,000 units for
children weighing
less than 30 kg
(given as a single
-250 mg 3-4 times a
day for 10 days. Very
small children
weighing less than
20 kg may be given
125 mg 4 times a day.

Penicillin V (phenoxymethyl penicillin) is the preferred oral


formulation because of its more reliable absorption. Penicillin
G may also be used if available, although its absorption is
less predictable.

* Stollerman G. H., Rheumatic Fever and Streptococcal Infection: Grune


and Stratton 1975

185

rheumatic fever/rheumatic heart disease cpm 3RD eDITION

severe valvular disease).


5. Subcutaneous nodules
6. A sleeping pulse rate greater than 100/minute
7. Erythema marginatum
8. Chorea
9. A positive test for C-reactive protein
10. Unexplained fever with rectal temperature of 100.40F for
at least 3 consecutive days.

The patient when confirmed to have reactivation of RF will
be treated accordingly as outlined in Table 4*.
Administration of Secondary Prophylaxis
Antibiotic regimen for secondary prophylaxis of RF/RHD is
shown in Table 6. Benzathine penicillin shall be provided
free of charge for RF-RHD enrolled in the Registry. The
chemoprophylaxis may be administered at the source of referral
by trained primary health workers after initial injection in the
Referral Center has been found safe. Patients shall be advised
to see to it that their visits for injection of benzathine penicillin
shall be properly recorded in their appointment card. Also, a
Patient Data Record Form should be filled up and kept in the
unit where chemoprophylaxis is given.
The Philippine Foundation for the Prevention and Control
of RF-RHD DOH shall issue benzathine penicillin at the
Regional Registry Center. The quantity shall be based on
the list of registered cases submitted by the various Referral
Centers. Since the monthly administration of prophylaxis
shall be carried out at the peripheral level, the regional
hospitals should be responsible in sending the drugs to the
referral centers which shall in turn dispose the same to the
various units where the enrolled RF-RHD patients are being
followed-up.
With the private hospitals, the drug may be directly issued
to them at the RF-RHD Foundation Office at the Philippine
Heart Center.
Frequency and Duration of Prophylaxis
1. Low-risk group - every 4 weeks
1.1 Arthritis - minimum of 5 years (symptom/
recurrence free)
1.2
Carditis - minimum of 10 years or up to age

18, whichever is longer, if recurrence free and

non-high-risk group
2. High-risk group - every 3 weeks
2.1
Carditis with previous history of RF
2.2
Severe attack of carditis with or without
multiple valve involvement
2.3
Development of recurrence of RF despite
regular monthly benzathine penicillin.
2.4
Cardiomegaly
2.5
Congestive heart failure
Note: Diagnosis of carditis should be documented by echocardiography
when feasible.
* Patients dealing with children, working in health services or
circulating in population crowded areas are at higher risk of
contracting streptococcal infection and must continue the secondary
prophylaxis while working or living in these conditions.

186

2.6

RHD - every 4 weeks


2.6.1 Mild - up to age 25 (single valve, without
recurrences)* (Class II-III)
2.6.2 Severe - every 3 weeks up to age 25;
(decrease unless recurrences occur)
- every 4 weeks for life if recurrence
free (Class II-III)
- Class IV-Oral Prophylaxis recommended
2.6.3 RHD with surgical intervention - for
prolonged period/s for life.

Table 6: Antibiotic Regimens for Secondary (Continuous)



Prophylaxis for Rheumatic Fever and Rheumatic

Heart Disease Prevention
Mode of
Penicillin Erythromycin
Administration


Benzathine penicillin G

Single inj once a month*
Intramuscular
For children <30 kg,

give 600,000 units.

For children <30 kg

and adults, give

1,200,000 units.
Oral
Penicillin V
For children >25

For children, 250
kg and adults,

mg twice a day.
give 250 mg
twice daily.
For children <25
kg, 10 mg/kg
twice daily.

CPM 3RD EDITION

rheumatic fever/rheumatic heart disease

Table 7: Clinical Presentation of Streptococcal


Tonsillopharyngitis
Common Findings

Non-Strep
Symptoms

Pain on swallowing
Fever
Headache
Abdominal pain
Nausea and vomiting

Coryza
Hoarseness
Cough
Diarrhea

Signs
Tonsillopharyngeal erythema
Tonsillopharyngeal exudate
Soft palate petechiae
("doughnut" lesions)
Beefy red, swollen ovula
Anterior cervical adenitis
Scarlantiniform rash

Conjunctivitis
Anterior stomatitis
Discrete ulcerative lesions

Appendix 1
RF-RHD Registry Center
Code
Number Region
Center
00000
NCR
00101
Philippine Heart Center
00102
St. Luke's Medical Center
00103 UERMMC
00104
Phil. Children's Medical Center
00105
Quezon City General Hospital
00201 UP-PGH
00202 UST
00301
Makati Medical Center
00401
MCU
01000
Region I
San Fernando, La Union
02000
Region II Tuguegarao, Cagayan
03000
Region III San Fernando, Pampanga
04100
Region IV Lipa City
05100
Region V Naga
15200
Legaspi
05300
Sorsogon
06100
Region VI Iloilo
07100
Region VII Cebu
07200
Bohol
08000
Region VIII Tacloban, Leyte
09100
Region IX Zamboanga City
10100
Region X Cagayan de Oro City
11100
Region XI Davao City
12000
Region XII Cotabato City

References:
1. Rheumatic Fever. Markowitz and Gordis. W.B. Saunders,
Philadelphia, 1972
2. Rheumatic Fever and Streptococcal Infection. Gene H.
Stallerman. Grine and Stratton, New York, 1975
3. Rheumatic Fever. Angelo Toronta and Milton Markowitz.
MTP Press Limited, Boston, 1981
4. Prevention and Control of Rheumatic Fever in the
Community. Pan American Health Organization.
Washington, D.C., 1985
5. Rheumatic Fever and Rheumatic Heart Disease. B.L.
Agarwal. Arnold Publishers. Bombay, 1988
6. Streptococcal Sore Throat Rheumatic Fever/Rheumatic
Heart Disease. Achutti, Kaplan, Nordet and Vynckt.
UNESCO, WHO and ISFC. 1992
7. Treatment of Acute Strep Pharyngitis and Prevention of
RF: Statement for Health Professionals. Committee on
Rheumatic Fever, Endocarditis and Kawasaki Disease
of the Council on Cardiovascular Diseases in the Young,
American Heart Association Rajumi, et al. Pediatrics 95
(96) 4: 758-68
8. Guidelines of the Diagnosis of Rheumatic Fever.
Jones Criteria, 1992 update by Special Writing Group
of Committee on Rheumatic Fever. Endocarditis and
Kawasaki Disease of the Council on Cardiovascular
Diseases in the Young. American Heart Association, JAMA
92 October 21; (268) 15:2069-73

187

PHILIPPINE FOUNDATION FOR THE PREVENTION AND CONTROL


OF RHEUMATIC FEVER/RHEUMATIC HEART DISEASE
INITIAL REGISTRATION FORM
Rheumatic Fever Prophylaxis
Regional Registry Center:

Name of Patient:
Address:
School:
Year of Birth: [ ] [ ] [ ]

day month year
Source of Notification to Registry: [ ]
1 - Hospital in-patient department
2 - Hospital out-patient clinic
3 - Private physician
4 - Laboratory
Active Rheumatic Fever:
[ ] Diagnosis

Registration No.:


Date of Registration: [

Hospital No.: [
Sex: [ ] 1-Male 2-Female

] [
day

] [ ]
month year

5 - School health service


6 - Mass examination
7 - Rural Health Unit/Barangay Health Station
8 - Others, Specify:
1 - positive 2 - suspected 3 - negative 4 - not determined

Major Manifestation:
Minor Manifestation:
Evidence of previous Group A

Clinical
Laboratory
Beta-hemolytic Strep infection:

[ ] Carditis
[ ] Fever
[ ] Elevated ESR
[ ] Positive Throat Culture

[ ] Polyarthritis
[ ] Arthralgia
[ ] Positive CRP
[ ] Positive Rapid Antigen

[ ] Chorea
[ ] Prolonged P-R Test

[ ] Erythema Marginatum Interval
[ ] ASO Titer Elevation

[ ] Subcutaneous Nodules
[ ] Initial attack (1 - yes; 2 - no; 3 - not known)
[ ] Severity of heart damange

(0 - None; 1 - Mild; 2 - Moderate; 3 - Severe; 4 - not determined)
[ ] ASO Titer (1 - <200 units; 2 - 400 units; 3 - >800 units; 4 - not determined)
Chronic Rheumatic Cardiopathy:

[ ] 1 - positive 2 - suspected 3 - negative 4 - not determined

Diagnosis

[ ] Mitral Stenosis

[ ] Aortic Insufficiency

[ ] Mitral Insufficiency

[ ] Aortic Stenosis
[ ] Organic Lesion of the Tricuspid Valve
[ ] Heart Failure


Year of Initial Attack:
[ ]
[ ]
[ ]
Year of last Attack:
[ ]
[ ]

day month year
day month

[ ] Number of Recurrences

1 - One
2 - Two
3 - More

4 - Not known
5 - None
6 - Initial Attack

[ ] Preventive Regimen in Previous Year



1 - Regular Intramuscular

2 - Irregular Intramuscular

3 - Occasional Intramuscular

4 - Oral

PENICILLIN
5 - Sulphonamides
6 - Erythromycin
7 - Any Combination
8 - Initial Attack

9 - None

Source of Information:
Address: Telephone No.:

[ ]
year

CPM 3RD EDITION

rheumatic fever/rheumatic heart disease

Drugs Mentioned in the Treatment Guideline


This index lists drugs/drug classifications mentioned in the treatment guideline. Prescribing Information
of these drugs can be found in the Philippine Pharmaceutical Directory (PPD) 7th edition. Opposite the
brand name is its page number in the PPD 7th edition.

Erythromycin
Am-Erythromycin
42
Ditron
42
DLI-Erythromycin
43
Drugmakers
Erythromycin
43
Ery-Max
43
Erybron*
43
Erycin
43
Erythrocin/
Erythrocin DS
43
Erythrolan
NP9, 43
Ethiocin
43
Gentrocin
43
Ilosone
43
J. McKnoll Erythromycin
44
Macrocin
44
Pharex-Erythromycin
44
Sarazine
44
Sefavex
44
Servitrocin
45
UL Erythromycin
45
Pen G benzathine
Penadur 6-3-3/
Penadur L-A*
Phenoxymethylpenicillin
Centrapen
Cimpicillin
Megapen
Mipacin
Pentacillin
Sumapen
UL Phenoxymethyl
Penicillin K

52
48
48
51
52
53
54
55

189