Case study
Re-emergence of Diphtheria-evaluation
and outcome--- An institutional overview
Swagata Khanna1, Manabendra Debnath2,Manas P Das3- Guwahati (Assam)
Abstract:
Diphtheria was quite prevalent in the pre-immunisation era. But with the advent of proper immunisation the prevalence of
Diphtheria has gradually decreased&it is very rare in developed countries.Classical Diphtheria still persists in many parts of
India despite wide immunisation coverage. This study was carried out in the Department of ENT,Gauhati Medical
College&Hospital,Guwahati,Assam for a period of 4years from January2010toDecember2013 with the purpose(1)To study the
patients with membranes in throat & related deaths,(2)To find out the difficulties & constraints in diagnosis and treatment,(3)To
evaluate & formulate a proper institutional treatment protocol and outcome of treatment.A total of 70 patients with membranes in
throat were studied,45were suspected to be due to Diphtheria & of these 20patients died.It was found that inadequate
immunisation,late presentation to tertiary health centres,lack of ICU facilities, poor availability&high costs of essential drugs
like Anti-Diphtheric Serum & lack of proper institutional treatment protocol were the main reasons for such a scenario.We
formulated a definitive approach & proper treatment protocol for treating such patients.Thereafter,15patients although all cases
not proved by microbiological tests but with high clinical suspicion of Diphtheria were treated according to our institutional
treatment protocol&these patients recovered&survived and the efforts are still continuing.
Key words: Diphtheria, immunisation, Anti-Diphtheric Serum.
Introduction
Diphtheria was quite prevalent in the pre-immunisation era.
But with the advent of proper immunisation the prevalence of
Diphtheria has gradually decreased & it is very rare in
developed countries. Classical Diphtheria still persists in
many parts of India despite wide immunisation
coverage.Diphtheria is a highly contagious and potentially
life threatening bacterial disease caused by Corynebacterium
diphtheriae.In 2008, India contributed 6081 (86.66%) of the
7017 diphtheria cases reported globally. There were no
reports of outbreaks of diphtheria in Assam since last few
years, though sporadic cases were reported in UIP monthly
report, which were never investigated and documented. The
immunization coverage in Assam was 19.30% in 2006 (RHS),
which has improved to 67.60% in 2006-2007 [1]. But in spite
of all efforts the cases of membranous tonsillitis with high
suspicion of Diphtheria and related deaths are on the rise
recently.
Aim and objectives- The aim of this study is to find out the
cause of the sudden rise in Diphtheria cases with the
following objectives,to study the patients with membranes in
throat & related deaths,to find out the difficulties &
constraints in diagnosis and treatment,to evaluate &
formulate a proper institutional treatment protocol and
outcome of treatment.
Materials and methods
The study was carried out in the Department of ENT, Gauhati
1
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Medical College & Hospital, Guwahati for a duration of 4
years from 1st January 2010 to 31st December 2013. It is a
hospital based study.The data was collected from hospital
records since January 2010 onwards. All details regarding the
patients like Age, Sex, residential address, Diagnosis,
treatment given were studied. Also, death due to diphtheria if
any were also noted. The patients with membranous tonsillitis
those are admitted in our department, proper history regarding
the onset of the disease process, immunisation status,
associated symptoms like fever, odynophagia, cervical
lymphadenopathy, nasal blockage, respiratory distress etc
were evaluated.Proper general, local and systemic
examinations were carried out.The pseudo membrane over
the tonsils and soft palate is properly studied and also if there
is any bull neck appearance. Respiratory as well as cardiac
involvement is also examined properly.After proper history
and clinical examination, multiple throat swabs were sent for
smear for KLB and culture and sensitivity.Chest X-ray and
ECG on repeated occasions were done to rule out any cardiac
involvement.Benzyl Penicillin was started immediately in
required doses.Anti-Diphtheric Serum in required doses
according to the extent of the disease was started on high
clinical suspicion of Diphtheria.In 2 cases,emergency
tracheostomy was also done.The cases as far as possible was
notified to NRHM office for information regarding
immunization process.The data obtained were critically
analysed with the various data found in the State Programme
Professor & Head, 2Senior Resident, 3Resident, Department of ENT and Head and Neck Surgery,
Guwahati Medical College, Guwahati, Assam
National Journal of Otorhinolaryngology and Head & Neck Surgery, Vol. 2(11) No. 2, August 2014
Implementation Plan 2011-2012.
Results and observations
A total of 70 patients with membranes over tonsils,
oropharynx and nasopharynx were studied, out of which 45
patients were clinically suspected to be due to Diphtheria.
Only 9 out of these 45 were proven microbiologically.In our
study it is seen that in 2010, 50% patients diagnosed with
membranes in throat was suspected to be due to Diphtheria.
Out of these ,57.14% patients died due to Diphtheria and its
complications.In the year 2011, 57.14% patients with
membranes in throat were due to clinically suspected
diphtheria. Out of these, 25% died.Similarly, in the year 2012,
63.16% patients with membranes in throat were due to
diphtheria and 33.33% patients died.In 2013, 81.81% cases
with membranous tonsillitis were due to diphtheria and
55.55% patients succumbed to it. Also, there was a sudden
spurt in Diphtheria cases in the year 2013.
It is observed that the incidence of diphtheria cases was more
among Muslims compared to other religions.
Discussion
In 1613, Spain experienced an epidemic of diphtheria. The
year is known as "El Año de los Garotillos" (The Year of
Strangulations) in history of Spain[2]. Before 1826,
diphtheria was known by different names across the world. In
England, it was known as Boulogne sour throat, as it spread
from France. In 1826, Pierre Bretonneau gave the name to
disease as diphtérite (from Greek diphthera
"leather")describing the appearance of pseudomembrane in
the throat[3].In 1883, Edwin Klebs identified the bacterium
and named it Klebs-Loeffler bacterium. The club shape of
bacterium helped Edwin to differentiate it from other
bacteria. Over the period of time, it was called Microsporon
diphtheriticum, Bacillus diphtheriae and Mycobacterium
diphtheriae. Current nomenclature is Corynebacterium
diphtheriae.Friedrich Loeffler was the first one to cultivate
Corynebacterium diphtheriae in 1884. He used Koch's
postulates to prove association between Corynebacterium
diphtheriae and Diphtheria.In 1890, Shibasaburo Kitasato
and immunized guinea pigs with heat-treated diphtheria
toxin. Von Behring won the first Nobel Prize in medicine in
1901 for his work on diphtheria. Over the Christmas holiday
in 1891 in Berlin, the first attempt to cure a person of
diphtheria was made. It succeeded. ... it was the first
cure[4].Corynebacteria are Gram-positive pleomorphic
bacilli with a Chinese lettering-like appearance on Gram
stain. Nontoxigenic strains rarely cause more than local selflimiting disease but lysogenic conversion can occur at a site of
colonization, and this has been suspected to have happened in
several outbreaks[5][6]. The diphtheria toxin exerts a lethal
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effect on all human cells by inhibition of protein synthesis
with resulting pharyngeal damage and with absorption into
the bloodstream, subsequent myocardial and neurological
toxicity. Three biotypes of C. Diphtheriae exist: gravis, mitis
and intermedius types; the gravis and intermedius types are
associated with a higher rate of mortality which is probably
related to the amount of toxin production. Less commonly
Corynebacterium ulcerans can also be infected by this
bacteriophage and produce a Diphtheria-like
presentation[7].Diphtheria is a rare condition in countries
which have a routine childhood immunization programme,
but remains an important differential particularly in
immigrant populations in the diagnosis of acute laryngeal
infection in children. Diphtheria remains a significant public
health problem in the developing world. The causative
organism is Corynebacterium Diphtheriae. There are three
strains, gravis, intermedius and mitis; it is the gravis strain
which is responsible for major epidemics and for the high
mortality. The early clinical picture of upper respiratory tract
symptoms is due to the effects of the organism itself. Delayed
effects are due to the release of endotoxin. Diphtheria is a
disease particularly affecting young children; it is rare over
the age of ten years[8]. Initial symptoms are of pharyngitis
with sore throat and malaise. The child is feverish and on
examination there is a typical appearance of the pharyngeal
tonsils with necrosis and the development of a characteristic
grey pseudomembrane over the surface. This consists of
necrotic tissue, bacteria and a rich fibrinous exudate.Early
removal causes bleeding but the pseudomembrane may
separate more easily later in the course of the disease. There
may be a bull-neck appearance due to cellulitis and regional
lymphadenopathy[8]. Laryngeal diphtheria rarely occurs
without prior pharyngeal infection. After progressive
dysphagia and toxaemia, inspiratory stridor and a barking
cough develop; the cough is frequently paroxysmal and
exhausting.Death may follow owing to acute airway
obstruction or as a result of the later effects of the
endotoxin[8].The endotoxin can cause a toxic myocarditis in
the second week of the disease and this may be fatal.
Peripheral neuritis may also occur, palatal paralysis being the
most common effect of peripheral neuropathy and presenting
with nasal regurgitation of food and hypernasal
speech.Successful treatment depends on early diagnosis, and
the administration of high dose benzylpenicillin and antitoxin
(10,000 to 100,000 units depending on the severity of
infection). Airway management consists of removal of the
laryngeal membrane, administration of oxygen and
humidification, and endotracheal intubation or tracheostomy
if necessary. Systemic steroids may reduce the need for
National Journal of Otorhinolaryngology and Head & Neck Surgery, Vol. 2(11) No. 2, August 2014
airway intervention. Bed rest is recommended until the
danger of myocarditis is past[9].The EPI of WHO
recommends three doses of DPT vaccine starting at six weeks
of age with additional doses of diphtheria vaccine in countries
where resources permit. Many national immunization
programs, including the UIP in India offer two booster doses
at 18 months and between 54 to 72 months of age; after three
doses of primary vaccines, protective levels of antitoxin
develop in 94-100% of the children. However without booster
doses, over time toxoid induced antibody drops below
protective level[1].In Assam, the immunization status of DPT
from 2005 to beginning of 2010 was more or less stable but in
the year 2010-2011 there was a sudden dip in the
immunization of DPT which may have lead to the spurt in
cases of Diphtheria.Moreover, the overall immunization
status of Assam in the year 2010-11 up to Dec2010 is 65.07%,
also, a dip can be seen compared to earlier years.Also it is
observed in our above Result & Observation section that there
was sudden and gradual increase in Diphtheria cases 2012
onwards.After the above observations we put forward some
leading questions to ourselves like:What are the reasons of
this spurt in (?) Diphtheria cases?,What are the causes of
increase in death due to (?) Diphtheria? Is there a need of an
institutional protocol for treating (?) Diphtheria?,Above all
the main problem we have faced in those that cases where
throat swab results are negative for KLB. Going ahead with
all these leading questions in mind, several mortality
meetings were held in our Department where we critically
discussed most of the deaths due to (?) Diphtheria.After
critically analysing the cases it was seen that almost all the
cases that died suffered from (?)Diphtheria had incomplete
immunisation history or had not taken immunisation at
all.Moreover, the incidence was on the higher side in a
definite religious group as seen in the study.The reasons for
this inadequate immunisation could be patients hailing from
difficult areas, ignorance of the people regarding
immunisation and reluctance of some group of people or
communities to avail immunisation due to the false myth
persisting in them regarding immunisation. The sudden
increase in deaths due to (?)Diphtheria may be due to:Late
presentation of the patients in hospitals, An institutional
treatment protocol was formulated in consultation with
Department of Paediatrics:-Proper history specially
immunisation status,Isolation of the patient,Proper clinical
examination and early diagnosis,Vitals to be monitored
continuously, Proper resuscitation and ICU care if
required,Repeated throat swab for KLB and culture and
S e n s i t i v i t y, S e r i a l E C G t o r u l e o u t c a r d i a c
manifestations,Start Benzyl Penicillin empirically in required
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doses (varies from case to case),Start ADS on clinical
Suspicion in required doses (varies from case to case).Add
antibiotics depending on culture & sensitivity reports,Also to
add steroids depending on symptoms,Notify cases to the
hospital authority as well as NRHM office.After formulating
the protocol for treating Diphtheria cases in our department,
15 cases were treated according to the said protocol and
results are fruitful. The patients recovered and efforts are still
continuing in our department and we are trying to improve
ourselves more and more in the days to come.
References
1. Nath B, Mahanta TG: Investigation of an outbreak of
diptheria in Borborooah block of Dibrugarh district,
Assam. Indian J Community Med 2010, 35(3):436–438.
2. LAVAL, Enrique (March 2006). "El garotillo (Difteria)
en España (Siglos XVI y XVII)". Revista chilena de
infectología 23. doi:10.4067/S071610182006000100012. Retrieved 29 November 2012.
3. "Diphtheria”. Online Etymology Dictionary. Retrieved
29 November 2012
4. John M. Barry, The Great Influenza; The Story of the
Deadliest Pandemic in History (New York: Penguin
Books, c2004, 2005) p. 70.
5. Pappenheimer Jr AM, Murphy JR. Studies on the
molecular epidemiology of diphtheria. Lancet. 1983; 2:
923-6.
6. Wilson AP. Treatment of infection caused by toxigenic
and non-toxigenic strains of Corynebacterium
diphtheriae. Journal of Antimicrobial Chemotherapy.
1995; 35: 712-20.
7. Lipsky BA, Goldberger AC, Tompkins LS, Plorde
JJ.Infections caused by nondiphtheria
corynebacteria.Review of Infectious Diseases. 1982; 4:
1220-35.
8. “Diphtheria”.Paediatric Otorhinolaryngology Part 12.
Scott-Brown'D Otorhinolaryngology,Head and Neck
Surgery.p-1130.
9. Havaldar PV. Dexamethasone in laryngeal diphtheritic
croup. Annals of Tropical Paediatrics. 1997; 17: 21-3.
Address for correspondence
Dr. Swagata Khanna,
SWAGAT, J.P. Agarwalla road, Bharalumukh, Guwahati
District-Kamrup Metro, Assam 781009
Email:drswagatakhanna@gmail.com,
Cell : +91 9864094140
National Journal of Otorhinolaryngology and Head & Neck Surgery, Vol. 2(11) No. 2, August 2014