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THALASSEMIA Clinical case presentation | THE WHITE ARMY | YouTubeToText
YouTube Transcript: THALASSEMIA Clinical case presentation
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Core Theme
This content is a detailed case presentation and discussion of a 12-year-old female patient with transfusion-dependent thalassemia, focusing on the diagnostic process, differential diagnoses, management strategies, and potential complications of the condition.
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Huh?
Okay. Yes, sir. Uh, good evening, ma'am.
Uh, I'm going to present a case on
palismia. U
here is a 12-year-old female patient
named Miss ABC hailing from Vidya Hubli,
Hindu by religion. The informant was her
mother, Mrs. XY Z. The information is
reliant and consistent. uh the date of
admission was 19th February 2020 and the
date of examination was 21 February 2020.
2020.
Okay. uh when you are giving demographic
data especially in cases of more like
ania uh actually uh we are supposed to
mention their community also uh in other
presentation actually we don't take the
religion of community much because here
community is very important because uh
the incidence of the carrier state and
the disease varies among the communities
right in the can you can be some
communities where it's where
this is very common
but tribal communities regions regions
tribal communities see especially if a
north nor if you take north India like
Punjabi Sindis it is very common so in
south India especially we are in talking
about Karnataka means in Karnataka it is
common among others GSB uh GSBs okay
this is uh it is like more of community
disases okay now the inter Community
marriages are increasing is there in
each and every community but these are
the incidents among these communities is
very common.
So it is better to mention the community
in the democracy.
Uh the chief complaints were she had
easy fat fatigability since 7 days and
increased palace since 5 days. Uh uh I
just want to make one comment before
starting. Okay. Uh one thing is these
are the chronic problems right? We know
like certain pediatric especially like
hemolytic anemia. Uh can you hear me
properly now?
Yes. Yes ma'am. Yes ma'am.
Okay. Hemolytic anemas uh cerebral paly.
Is it clear now? Can it can everyone hear?
hear?
Uh yes ma'am. Now it's clear.
Okay. Uh see certain pediatric problems
especially hemolytic anemia cerebral
plasty all these things will start from
the early ages right. Uh in two way you
can present the case. One thing is you
can tell the present uh the examiner
that the child has come for the
transfusion and doesn't have any
problem. uh since the history starts at
the age of six months I would like to
start from there. Similarly for cerebral
paly the incident happened at birth. So
I would like to start from birth history
like that you can start. Okay. Uh yes
and other is how you have presented. You
can uh uh tell easy fatigue, increased
paralysis like what is the present in
compliance and you can start but most of
the time talismia if it is well managed
they don't have any symptoms when they
come for transfusion that time you can
just start with the uh past history only
like you can tell them that these all
started at the age of five months or 6
months and since then uh it is going on
so I would like to start from there like
that you can continue. Okay.
Yes. Yes. Okay. Ma'am
ma'am shall I continue?
History of chief complaints. Patient is
a known case of hematological disorder
came for regular transfusion with
complaints of easy fatigability on doing
the daily activities which is relieved
on taking rest. This is not associated
with any other complaints. Patients
mother noticed parlor since 5 days which
is insidious in onset and progressive to
present stage in eyes and skin of
patient. No history of yellowish
discoloration of skin, yellow colored
urine or hematuria. No history of easy
bruising, bleeding gums. No history of
pain in legs, abdominal pain, headache,
convulsions. No history of bumps in
stool. No history of bone pain, weight
loss, etexia.
No history of blood loss in stool in
vomitus. No history of abdominal pain,
passing worms in stools or vomitus. No
history of pah. No history of diarrhea
or constipation. No history of fever
with chills and drivers or dark colored
urine. There's no history of oligura or
anura. There is no history of dysnia,
teipia or palpitation.
uh Dr. Niti uh I want you to explain why
you have asked all these things uh
negative history to parents. So child
has come with history of easy fatigue
liberty and palar right which is progressive.
progressive.
How what was running in your mind? Why
what why it happened to her?
Yes ma'am. Uh uh basically this is due
to anemia ma'am. Uh so to uh like to
distinguish whether it is a nutritional anemia
anemia
okay if it is anemia anemia [Music]
uh if it is due to anemia like it should
be severe right it is a progressive par
she's going into ability means HB would
have dropped to five or six otherwise
they don't manifest like this what made
her to wait till that like calcinium.
What is the ideal hemoglobin you would
like to maintain?
Yes, ma'am.
Ma'am, 9 to 10 g per percent.
Huh? 9 to 10.5 g% and you won't expect
any any of these symptoms at that point
at that level of hemoglobin. So
something would have happened to her
like especially here you should
concentrate. So there it is like some malised
malised
something would have happened like
infection that you have to highlight one
thing. Second thing is any other
complication can cause this kind of
picture in her
she could be having some cardiac
dysfunction due to uh iron overload.
Yes ma'am. These are the things which
you should keep in mind when the
calcinium patient comes with increasing
par easyetic
otherwise usually if they are taking
proper regular transfusion they don't
have this pal oretic they should not
have this easy or pal in between the
transfusions okay you are asking about
discoloration of skin yellow color urine
hematuria to rule out anyis
yes hemalysis
Okay. Easy bruising, gum bleeding, any
other like alastic crisis you would
wanted to rule out, right? Yes.
Yes.
Pain in leg.
Huh? Yes ma'am. No history of pain in
legs, abdominal headache or convolutions
for to rule out cell anemia. Ma'am.
Okay. Okay. Before that, I wanted uh you
to tell me what are the condition where
regular transfusion is required?
Uh ma'am uh thalisalcemia
major. Okay. Next.
Uh plastic anemia.
Eplastic anemia. Do you think uh child?
Okay. You have not told me yet the past
Malignant disorders like leukemia.
Uh see they will progress right? They
won't be allowed to receive recurrent
transfusions. They will have bleeding, hyposomal,
hyposomal,
high grade fever like they will progress
or either with treatment they should
improve or they'll they won't have this
uh this thing. Okay. Next.
Some hemorrhage severe hemorrhage.
Uh severe hemorrhage over a period of
years they will receive transfusions.
No ma'am like
at the time of hemorrhage.
Okay. So the important uh differential
diagnosis you should keep in mind when
the child of like child is receiving
recurrent transfusions from the engage
is one is ismia major other is uh
certain uh other hemolytic anemas like
severe hereditary sperocytosis severe
alastic anemia sorry severe cle cell
anemas that cle cell anemia who had CNS
manifestation in the past will receive
recurrent transfusions. Then inherited
bone marrow failure syndrome you would
have heard of diamond black pen anemia
and all they will receive recurrent
transfusion. Then other cause of
hemolytic anmas like enzyme like pyroate
deficiencies, GC3D deficiencies all
these people will receive recurrent
transfusions and uh other causes of
anemia like autoimmune hemolytic anemia,
nutritional anemia, iron deficiency, one
deficiency, colon cancer deficiency
transfusion maybe once or twice not
recurrent transfusions. Yes ma'am.
Uh I was mentioning alastic anemia. A
plastic anemia more than anemia. They
will have more of bleeding
manifestations and infections. Yes.
Yes.
For so long they won't sustain. They
will either die or they have to take
proper treatment and get okay. So other
rarely you can mention osteopetosis and
all. So these are the five to six uh
differentials you can give. uh if a
child with child comes with history of
recurrent transfusion regular uh
recurrent transfusions. Okay.
Yes ma'am.
Uhhuh. You can repeat now once what are
the differentials I told you.
Yes ma'am. Severe hereditary
sperocytosis or
severe hereditary sceroytosis. Okay.
Uh then cle cell anemia present with
presenting as CNS manifestation. cle
cell anemia more of they will have
crisis and other things but if they have
CNS event stroke we will start them on
regular transfusions to maintain the
okay okay next
then plastic anemia or pure red cellia
like diamond black
inher failure that is involving only the
red cell lineage okay yes
yes then
then
and ma'am once once or twice transfusion
for nutritional deficiency.
So these differentials you keep in mind
and uh pertaining to these you have to
ask for negative histories. Yes,
correct. You have asked for pain in
legs, abdominal pain, headache,
conversion. Okay. Next. Next you can do. Yes.
Yes.
No history of worms in stools for worms
which can cause hemorrhage and GI bleed
which can lead to anemia ma'am. Uh bone
pain weight loss kexia for malignancies.
I don't think so. You should mention
keexia in the hopi because it is not the
short term. Okay. Weight loss is enough.
Yes ma'am.
Other blood losses
or vomiters. No history of abdominal
pain passing worms in stool or vomitus
ma'am for uh worm causing GI bleed. No
history of fica.
This abdominal pain is very very very
important in hemolytic anemas especially
those who are requiring recurrent
transfusions. It could be due to many
causes. Okay. You should ask them where
is the proper how is like if they tell
abdominal pain in this patient is not
having but in case they have uh
abdominal pain where is the pain is it
left is it right there are multiple
reason to have reason for this abdominal
pain in hemolytic anemia one you have
mentioned sickle crisis painful crisis
they can have abdominal pain other thing
is massive spleenali can cause dragging
abdominal pain. Okay. Other thing is polytheasis.
polytheasis. Yeah,
Yeah,
they can have poly rightsided abdominal pain.
pain.
These are the important causes of
abdominal pain in and also sometimes uh
drug related they are on chilus right
that also can cause abdominal pain. So
most of the time if talismia patients
are made managed very well means they
don't have any of the symptom but these
can be there because of some
complication. Okay. History of p you
asked for history of pa you cannot ask
you can ask eating mud dirt like that
you can ask.
Yes ma'am.
Why diarrhea constipation?
Ma'am, diarrhea for malabsorption
syndromes and constipia.
Yes. And constipation for hypothyroidism
because hypothyroidism is associated
with anemia.
Uh no history of fever with chills and
drivers and dark color urine for malaria.
malaria. [Music]
[Music]
and no history of oligura or anura for
chronic kidney disease
which can lead to anemia and no history
of disna and palpitation it can it can
lead to complications like heart failure
so I have asked
okay uh I can see some messages in chat
box clotting factor deficiency
hemophilia and all though they have
recurrent bleeding uh their marrow is
perfectly healthy most of the time it
will compensate. They don't require like
blood transfusion for that. And now uh
we don't use any plasma for replacement
of the factors. We use the recominant
factors. Usually don't come with history
You can go to the past history.
Past history. A known case of
hematological disorder diagnosed at the
age of 6 months when child presented
with fever, loose stools, irritability
and decreased frequency of maturation
for 4 days. History of blood transfusion start
start
sorry I'm interrupting you. I thought I
should tell you now. Uh this is at the
age of 6 months. You told me so many uh
differentials which requires recurrent
blood transfusion, right? Yes.
Which one will present like uh can you
tell me at which disease will present at
the age of 6 months?
Ma'am, uh 3 to four months cle cell
anemia and 6 months talcemia. After 6
months talismia.
Okay. Okay. What is the classical
So they present mainly with the pain
crisis, right? We call this hand foot
syndrome. Okay. They can present with
per like peripheral bony pain, swollen
hands, swollen feet. That is a classical
picture as you mentioned around uh 6 to
8 months they will present. Okay. Other
thing is what other differential was
hereditary sperosyosis. So if it is a
severe hereditary sperosyosis
they usually present in newbra. Okay.
They presents with hemolysis and
neonatal jaundice requiring phototherapy
transfusions and all and other these
enzyos also will present in the early
life like neolyal period only they will
manifest if it is severe and mild form
of enzyopathies and scerosytosis can
present at any age but usually they
don't require recurrent transfusions. So
classically which presents at 6 months
which requiring mutant transfusion is
most likely is okay.
okay.
Yes ma'am.
Why at 6 months
ma'am? Because at 6 months the
hemoglobin F production decreases and
hemoglobin A production starts and
hemoglobin switch will happen at 6
months. Okay. They present phase 6
months. Okay. Continue.
History of blood transfusion started at
6 months once in 3 months for 2 years.
Then it is given once a month. Total
number of transfusions are 109. There is
no history of tuberculosis, chronic UTI
or jaundice. There is no history of PBS surgery.
surgery. Okay.
Okay. [Music]
[Music]
Treatment history.
Treatment history on tablet desperox not
on any other drugs. Okay. Here no. Uh so
uh age at uh age at first transfusion is
very very important. Okay. Uh 6 months
here like uh to differentiate calcium
major and calcia intermedia. If the
transfusion is started before less than
2 years of age then it is most likely to
be calcium major. More than two months
two years is calcia intermediate. That
is the test book definition. But we have
to follow the course of the patient to
tell whether he's major or intermediate.
Okay. Age of after that uh one more
thing which you have to uh tell at age
of presentation what was there any
trigger is it after any drug uh drug
intake was there any infectious trigger
that will give you some clue whether it
is enzyopathies. If they have some child
had some malaria they gave primock then
the child started having hemolyis
picture transfusions you know it could
be G6PD deficiency any trigger was there
at that four days uh had those things no
that is very very important you have to
ask h
yes ma'am
then uh you have to describe the
transfusion schedule in detail okay when
was the last transfusion how frequently
the child is receiving transfusion.
So many talismia child can 12 year old
can tell whether she's receiving how
much how many blood units one unit of
blood two bags of blood three bags of
blood and also certain any change in
pattern of transfusion in recent last
few months like increase in transfusion
requirement that will tell you child
might be going for hypersplenism. spleen
is big. It is getting consumed. The red
cells are getting consumed. That's why
requiring repeated transfusion. So
changing that pattern is also very
important. You have mentioned most of
the thing but like few things more you
have to do.
And next thing is about iron chilation
treatment history. Okay.
uh in that yeah you have mentioned she's
on deference rocks but when it was
started that is very important for
prognostication early trans early
chilation regular chilation will improve
their quality of life okay when it was
started and what was started was it
injection or the tablet and how is how
is it taken a way to take deferox it has
to be taken empty stomach dissolved in a
plastic container either in juice or
water and to be taken in empty stomach
in the morning once daily. Okay. If they
don't take if they use some steel spoon
steel cup to dissolve no it will chilate
the iron residual iron in the container
and it may not work well. So that is
very important. Now see uh you you know
it is a case of talisia almost. So you
have to be concentrating on the details
of the CMA H.
Yes ma'am.
Was the how is the compliance whether
taking uh regularly or not? Any other
medicine? Most of the patients will on
folic acid also. Yes.
Yes.
Some patients will take vitamin C,
hydroxyura, other medicines also. So
other medications also is important. Okay.
Okay.
Yes ma'am. And if the child is more than
10 years in the past history, you are
supposed to take some history regarding
any complications. Yes.
are guys please wait for some time I
will connect back some connection issues
we are having
Hello.
Can you hear me now?
Yes, ma'am. It's audible, ma'am.
Yes, ma'am.
Okay. Okay. Huh? You can continue now.
After the treatment history.
Yes, ma'am.
Birth history. Antiatal history. It was
a booked case. Anomaly scan was done.
Iron folic acid tablets taken. No
history of fever with rash. No history
of antipartum hemorrhage. No history of
exposure to radiation. No history of
multiple pregnancies. The inter
pregnancy interval was 4 years.
Okay. Which hemolytic anemia will
present in anti period.
M hydrox falic.
Yeah. Alpha Calismas can have
this patient. Okay.
Yes ma'am.
Ma'am, shall I continue?
Yeah. Yes.
Natal history. Uh cesarian section was
done at Kim at 10 months 16 days. Birth
weight was 3.5 kg. Cried immediately
after birth. No history of admission to
an ICU. No history of bleeding from c or
breastfeeding started within an hour
after birth. Postnatal history. No
history of neonatal jaundice.
Exclusively breastfed for 6 months and
continued for one year. Supplementary
food was given after 6 months.
Here in birth history as I've already
mentioned the neon genus which is the
most important history. Okay. So
normally especially for enzymeopathies
and hydrophocytosis
which can present in neonatal period and
they might require recurren repeated
translation in the
uh infancy like later in the child. Okay.
Okay.
Yes ma'am. Next.
Next.
Developmental history. Developmental
milestones achieved regularly. Good
scholastic performance. Immunization
history. immunized up to 5 years of age.
Okay. Uh this uh in the immunization
history I want you to be very very clear
about the uh immunization history you
have to take in detail for this child
because they are exposed to many of the
viruses due to recurrent transfusion. So
specifically you should ask hepatitis B
injection is taken or not? Hepatitis A also.
also.
And if they are if you take in the
history if they give any history of any
surgery especially spleentomies
then you should ask for any special
injection was given to prevent
infection. What would you like to give
if they are undergoing spleentomy?
Hemopilus influenza Bumoccoal and menal
hip penta sorry PCV and menal. So
imunization history should be little bit
elaborated because it is very important. Okay.
Shall I continue? Sure.
Sure.
Nutritional history morning uh required
is 1,900 kilo calorie and deficient was
five 520 calorie and 6 g of protein. So
percentage is 27% deficient in calories
and 18% deficient in proteins.
So in calismia
uh in the diet history you should ask
her what kitchen utensils they are
using. See so many people will use iron
utensils for cooking. Okay. So they are
already in iron overload status. They
should not use much of uh iron utensils.
Next thing most of the talismics will be
advised to take tea or citrus fruit
along with the meal. Okay. Whether they
are doing that or not is important. Why?
Why we have to give uh tea or citrus
fruit along with me?
Ma'am, tea inhibits iron absorption. So
to prevent iron overload.
Yeah, correct. So that history also you
should drink. Okay. That is important.
Yes. Of course the calorie protein
intake what is the 24-hour equal that
should be there along with that you
should take these two histories in the
diet history.
Personal history. She consumes a mixed
diet. Appetite is normal. Sleep is
sound. Bowel and bladder are normal and regular.
Oh,
Yes, ma'am.
Someone in the chat box is asking why
citrus fruit. Citrus fruit also vitamin
C also will reduce the iron absorption. Okay.
Okay.
Family history you can continue.
Yes ma'am. Second degree consynchronous
marriage married life 15 years. There is
no history of similar illness in any
other family member. There is no history
of surgeries in family members.
So uh it is automal recessive disorder.
So marriage is important. Similar
history in the family means what history like
like
ma'am like
jness or gallstones or repeated
transfusions and surgeries ma'am like
Okay.
And spleenctomy uh hy you should inquire.
inquire. Next
soio economic status. Father is farmer
studied till SLC. Belongs to upper lower
class according to KUS Swami
classification. Lives in a katcha house
with overcrowding and with good
ventilation. No open defication and
Next
ma'am shall I continue? Uh yeah uh I
would like to clarify one doubt on the
chat box that is why choleicusctomy
ma'am uh this is see uh we uh especially
in him anemia there is history there is
higher chance of developing pigmented
stones especially in heritary spiritsis
so they will they are known for more
more prone for gallstones so policy
Summary. Here is a 12 year. Yes ma'am.
Here is a 12-year-old female child who
is born to a secondderee consanguinous
marriage belonging to upper lower social
economic status who consumes a diet of
27% deficient in calories and 18%
deficient in protein. who is immunized
up to date with normal developmental
milestones and with past history of
known case of hematological disorder
came with complaints of ephetic ability
since one week and progressive palace
since 5 days.
Okay. uh past history of known case of
hematological disorder which is
requiring regular transfusion once every
month and uh oral tablet for
identulation. If you mention it will be
like it will what hematological disorder
you know now heological disorder which requires
requires uh
uh
blood trans
blood transfusion that is very important
you have to mention that now she has
come from easy ability since one week in
progressive valor could be due to
what is
um due to uh
that's why in the history whether she
has come late you have to ask was any
infection into current infection which
lead to disproportionate decrease in
hemoglobin level or any cardiac symptoms
are there. If you know that like what is
the reason you can tell here. Okay.
Yes ma'am.
It is due to the rel uh
uh delay like uh delayed presentation
because she couldn't come due to some
reason whatever she gives. If you
mention like your history summary will
be complete. Yes ma'am. Okay ma'am.
Yeah. Uh I would like to clarify one
more doubt here. Uh somebody has asked
uh can we start the history from 6
months. Yes of course you can start if
they don't have most of the talismic as
I mentioned they don't have any symptoms
when they come they will the minute you
go and ask they like you come for blood
transfusion no other problem. So you can
start from 6 months of age. You just
mentioned that here is a 12 year old
girl who is taking regular blood
transfusion since 6 months of age. The
history started since 6 months of age.
Hence I would like to start from the
initial history start with start from
there. Okay. That is that also is fair
you can continue like that. Okay.
General physical examination. Here is a
12 years old female child conscious,
cooperative and well oriented to time,
place in person. Examined in supine
position. Vital signs pulse 98 beats per
minute in right radial artery. Good
volume. Non-colapsing. No radioradraial
or radio delay. Respiratory rate 20 22
breaths per minute. Blood pressure 106
by 82 mm of Hg mm of mercury measured in
right brachial artery in sitting
position temperature is 98° F measured
ma'am shall I continue
head to toe examination hair is normal
frontal bossing is present parietal
bossing is present mala prominence is
present eye por is present nose flat
Nasal bridge indicates
indicates hemolytzing
hemolytzing
paral buzzing
prominent hemolytic faces classicalally
chipmunk faces we call okay next
uh teeth normal oral cavity good hygiene
no features of anemia nails normal skin
par no hyperpigmentation or fatigue
upper limb is normal eye normal No
microcornia. Tongue is normal. Lower
limb normal. No leg ulcers. Chest, spine
and back are normal. No, sinosis,
clubbing or generalized lymph nopathy.
Okay. Uh here uh your hemolytic pates.
Why you want to see the
hyperpigmentation? What it indicates?
Megaloblastic anemia or panconia anemia
can have hyperpigmentation.
Yeah. that they can have but it is not
your differential diagnosis at present. Right?
Right?
So iron overload itself can cause
generalized hyperpigmentation.
Okay. And what is this upper limb
normal? Lower limb normal. What what you
wanted to convey
ma'am upper limb I wanted to check for
absent radius or trial
and forearm anomalies hand analysis are
common in anemia
anemia
anemia anemia they will have a plastic
anemia kind of picture but diamond black
anemia they can have
thumb anomalies forearm anomalies with
just requiring recurrent transfusions S
okay with no platelet or WBC involvement
and also have you mentioned the spine
and back normal. Okay, that is
important. Any masses, extra medularary
hemtopoysis you are supposed to mention. Okay,
Okay,
that is your end of general physical
examination. Right? Yes.
Yes.
Here she is 12 year old. You are
supposed to mention Tanner stretching in
child like more than 10 years of age. Okay.
Okay.
Can you hear me?
Yes, ma'am. Ma'am, shall I continue?
Yeah. Yeah, that's what you're supposed
to mention tannis staging here.
What? Tanner staging. Okay, ma'am.
Okay. Because uh one of the complication
of talismia and iron overload is uh
endocrine dysfunction. H because of that
the most of them will have delayed puberty.
puberty.
Yes. So delight puberty means you have
to mention 12 year old okay still not a
age to all them as like if she has not
developed also it's okay tana stage one
you can mention t staging not in six
years five years that age group I'm not
mentioning this more than 10 years
mention the tanner stage okay in the
general physical examination
yes ma'am
anthropometry height is 42 cm and weight
is 40 kgs. Height is anthropometry
anthropometry
in talismia is very very important that
determines their adequacy of
transmission. How are they growing? Okay.
Okay.
So here you have to be little bit
elaborate like weight, height, upper
segment, lower segment because certain
chilator iron because of certain iron
chilator they can have short upper
segment. So you are supposed to take the
sitting height also. Okay.
Yes ma'am.
And uh I think within 50th uh as a
pediatrician within 19th cent within
50th cent will be very vague. Okay. You
have to mention whether between 5th to
10th 10th to 25th 25th to 50th. So uh if
I'm not wrong I think height is between uh
uh
uh fifth to 10th and weight is between
25th to 50th I think. So she is looks
like slightly short for her age and also
you have if she is short take if mother
and father is also present there take
their height also. Okay.
Because short
stature in a talismia child indicates
what? Or growth failure in a child
indicates what?
Growth retardation.
Ah they are not transuced uh adequately.
Okay. Uh other thing is they can have hypopetism.
hypopetism.
They can have growth hormone deficiency.
So they can short because of
anthropometry is very very very
important in
a little bit we elaborate on that is my
suggestion here.
systemic examination per abdominal
examination in inspection. Abdomen is
distended on left hypochondrium.
Umbilicus is central and averted.
Corresponding quadrants move equally
with respiration. Dilated veins are
present. No scars. Hernal orifificesis
are normal.
Palpation. There is no local rise of
temperature or tenderness. Deep
pulpation mass in right hypochondrium
moves with respiration. Firm
inconsistency. Smooth surface. Sharp
border 3 cm below right hostile margins.
Suggestive of liver. A mask in left
hypochondrium moves with respiration.
Non- tender firm in consistency. Smooth
surface. Sharp border of 8 cm below
coastal margin. suggestive of clean
spenic notch is not appreciated.
Okay. At this point in a caseia case
presentation for you definitely they
will ask two question. One is what are
the different ways of pulpating the
sleeve? You should know how you pulpate.
You can tell me
the pet side. It will be very good. We
can demonstrate. So this definitely will
how did you pulpate?
Okay. If it is very small,
how will you palpate
ma'am? By hooking method in left
subostal margin.
Yeah, you can right
the patient right lateral position and
see or you can go on the left side and
try to palpate by cooking method also.
You can do short.
Okay. Next they can ask you how do you
know it is clean or how do you
differentiate between
renal mass is balotable and it can be
bmanually palpated
spleen is not balotable or bmanually
palpated and if is very much enlarged we
can appreciate the knots
and get the swelling
also important like you can insert
finger between the margin and the mass
but not between the spleen and the margin.
margin.
Yes. These are the standard questions
the examiner will ask.
I I feel you know right how to palpate
the skin you have to from the right
right you have to go towards the left uh
uh margin.
Hello.
percussion. Liverpan is 14 cm. No free
fluids. Oscultation. Bowel sounds are
heard. Other system examination. CVS. No.
No.
You mention about the genet inspection.
I forgot.
No. No ma'am.
ma'am. Uh CVS no raise JBB S1 S2 herd no
m uh respiratory system normal
vesticular breath sounds CVS
if a child withia
has come with history of breath
like that. So you have to be little more
in detail.
So some cardia complication third
third
gallop is there or if the child is
having P2 that is showing corary
those two things are very important
and also any flow mas are there if she
has not transuced and taken her
uh CNS higher mental function is normal
no sensory or motor deficit or diagnosis
or case of hemolytic anemia probably not
in heart failure.
So that's why your history on the way
they are getting transuced the way they
are taking chilation is important
because while summarizing the history
and examination you are supposed to tell
like 12 year old female child presenting
with history of failure and easy
on examination having hemolytic faces paral
paral
and organomeagal is significant along
with some growth retardation. If puberty
is not a puberty, you have to mention if
any other complication is there you have
to mention. Then after mentioning that
you have to tell the case of calisthemia
major. Well transfused or under
transfused. She is definitely under
transfused because at the end of one
month she's coming with paral and she's
having already hemolytic faces and organism.
organism.
and some uh short stature also right?
Yes ma'am.
That means she is like under transfused.
So if a child is transfused well once
every week 3 to four weeks maintaining
the pre-transmission
hemoglobin of 9 to 10.5
uh they should not have extra medical
hematopois that means this liver
widening of the bones all these things
they should not have. So she is
definitely under transucated
or underated you have to mention looks
like if you have taken a history when
when was the chilation started whether
it was transation
is started at 8 years 10 years you
definitely know it will be not a good
chilation. So when it was ratted whether
she's taken regularly or not all those
history are there along with that your
physical examination can tell like if
they are in heart failure CCF they have
features of hypocarat
all these things tells there's some
endocrine thing going on that means she
is under chillated so you have to
calcium nature well transduced under
transduced or well chillated under
chilated or If any complication you are
supposed to uh
mention. Okay.
Yes ma'am. Yes ma'am.
Okay. And in diagnosis the terminologies
are important. So
they have now phenotype classification
depending on the severity of illness and
how often they require transfusion. So
now they are called transfusion
dependent talismia
uh transfusion uh non-transfusion
dependent talismia we call it as t dt n okay
okay
okay ma'am
you can understand you can see later in
the textbook also how terminologies
change it is not calisthenia majoria minor
minor
is TD NTD transcription depend dependent
non-transfusion dependent. Okay.
Okay.
Okay. Ma'am
that is transfusion dependent means they
cannot live without regular transfusion.
Life is sustained because of repeated transfusion
transfusion
dependent. They might require some
transfusions but they are not dependent
on the transfusion to sustain their
life. Okay. That is the terminology
which you have to use now latest. Okay.
Uh yes ma'am.
So you can summarize her history and
tell me the diagnosis correctly now.
Yes ma'am. Here is a 12 year old female
born with a consignment marriage. On
history she has easy fatigility since 7
days and increased parlor since 5 days.
uh she on examination she has hemolytic
faces and uh palar and on examination
she has uh extra medular features of
extra medillary hematopois like hippatosphenomegali
hippatosphenomegali
uh so my probable diagnosis is
not cardiac failure like you have told
so you have to mention not
yes and short stage which are important.
So she is what is the diagnosis
transfusion dependent calisthemia and
transduced underulated
presently having no features of
complication she might be having but she
doesn't have but if you attribute this
short stature to iron overflow then
definitely yes she's having short that
Yes ma'am.
Okay. Uh I just want you to tell me why
you want to keep that hemoglobin of 9 to
10.5. We can keep it at 12. No. Give
transfusion every 2 weeks.
Ma'am, it can lead to high output and
overload and cardiac failure.
Cardiac failure. Okay. No, it's like it
is they have seen there are there are
transfusion regimens you would have
right right hyper transfusion super
transfusion moderate transfusions okay
you can read this on theory paper theory
books they mentioned so this is called
moderate transfusion this is the HB
level they have seen the growth of the
child will be normal at this level and
they don't have uh extra medularary
hematopois is at this level and also
same time you are not pouring so much
blood into the body you are limiting the
iron overload. So in calisthenia the
iron overload is one is the transfusion
which you are giving iron can there is
no way out way out for the iron right
there is no mode of excretion for the
iron in the body. So you are giving
transfusion hyram getting accumulated
that also you can limit by keeping the
hemoglobin between 9 to 10.5. Okay. So now
now
now you have to now the transfusion
regimen which is uh which is uh
Uh somebody is asking chilation.
Chilation. So chilation uh you can tell
like whether adequately chilated or
under chilated you can tell by history
and examination. history you have to ask
that when it was started. Ideally it
should be started within 10 to 20
transfusion means if the child is
started transfusion at 6 months of age
by 2 to 3 years the cherry should be
started an iron chilation if and it
should be taken regularly and you can
ask them whether the blood test is being
done whether the doses are they are
increasing as the child as the child
grows you are supposed to increase the
dose whether it is done or not on
history you can ask all these things on examination
examination
You can check for the iron overload
features like generalized pigmentation
is there means they are iron overload
that means they are underulated. Other
things are if they have uh endocrine
dysfunction features like uh
hypothyroidism, short stature. Uh if the
child is 15 16 years old, still not
developed secondary sexual character
that means that they are having delayed
pupy that is due to either central or
per peripheral hypogonadism. All these
things indicates that they are
underated. If a 13year-old girl tanner t
if she has come means okay she is
getting chilated well and she is her are
working means you can tell us she is
So we can end the session here.
Yes ma'am. Don't think so. There's
life expectancy of major as ma'am.
Okay. Life expectancy is like if they
are well transfused maintaining their
pre-t transfusion novel globin 9 to 10.5
and well chilated uh 40 to 50 years what
they still that is good but most of the
time now uh we have durative option for
calisthenia people are going for
hemopoic stem cell transplant and they
and they want about management of this patient.
patient.
Okay. Uh for this patient, I would like
to go through her transfusion history.
Uh how uh how is she being transfused?
Okay. Uh whether she's maintained the
pre-transfusion hemoglobin 9 to 10.5, I
want to see. Okay. And
uh NI are you online still?
Yes. Yes ma'am.
Okay. H uh can you tell me what blood do
you want to give for this child? Mom
Mom
lucosite blood mom packed cells with
your lucco. Yeah, we we call it as luc
blood red blood red blood red blood red
blood red blood red blood red blood red
blood red blood cells with at least 75%
we'll be giving to reduce the volume of
blood which we are giving okay and we
will give at least 15 to 20 ml per kg and
and
over 4 to 6 hours transfusion we will
give and uh it should be repeated every
3 to four weeks to maintain the
pre-transfusion tumor. Next thing is uh
uh
uh her chilation chilation. What is she
on? What do those uh what was her last
feritin? I will check. Why? Why? Why we
have to check the feritin?
Iron overload.
Yeah, it is feritin indicates the total
body iron overload. But the very good
method to assess the exact iron overload
is T2 star MRI of the heart and liver. I
will do accordingly I will adjust for
chilation. Sometime we might have to go
for double chilation. What are the
chilating medicines? You know three
medicines which are used commonly desperoxamine
desperoxamine defirrone
defirrone and
and deferox
deferox deferox.
deferox.
So if she is very much so I will
optimize her iron chilation. I will see
I will go to the whatever defer what mg
per kg she is taking. I'll see she is
iron overload. I'll increase it to
maximum 40 mg per kg. is still required.
I will add one more lateration.
I will start. There's something called
shuttle hypothesis. What is shuttle hypothesis?
hypothesis?
I don't know ma'am.
Okay. This definite prone is there it
will intracellular
whichever inside the cells
and DF4 desperate will select the extracellular iron. When you give this
extracellular iron. When you give this combination of prone with
combination of prone with desperate you will have both
desperate you will have both intracellular the intracellular iron
intracellular the intracellular iron will be chilated by deserone and it will
will be chilated by deserone and it will be sent to the extracellular plasma. So
be sent to the extracellular plasma. So in plasma the teros will take care of
in plasma the teros will take care of that iron. So this is called sh
that iron. So this is called sh hypothesis. I will start them on I will
hypothesis. I will start them on I will start on dual chilation. Okay. Uh why
start on dual chilation. Okay. Uh why you want to give this luccoy depicted
you want to give this luccoy depicted cells?
Ma'am to prevent allergy. Yeah. So we have to take care of
Yeah. So we have to take care of transfusion related complications also.
transfusion related complications also. The most common transfusion related
The most common transfusion related complications are acute uh nonolitic
complications are acute uh nonolitic acute fbril illness which is due to the
acute fbril illness which is due to the neutrils which are present in the red
neutrils which are present in the red cell bag. Okay. By depleting the red
cell bag. Okay. By depleting the red cell uh you will prevent nonimmolic
cell uh you will prevent nonimmolic fibbral transfusion reactions and also
fibbral transfusion reactions and also there is to some extent you can prevent
there is to some extent you can prevent alohimization and CMV transfusion
alohimization and CMV transfusion especially if the child is planned for
especially if the child is planned for hemopolitic stem cell transplant which
hemopolitic stem cell transplant which is very very important they should not
is very very important they should not get autoimmunized they should not have
get autoimmunized they should not have okay and
okay and her nutrition which is deficit how we
her nutrition which is deficit how we have to take care of that nutrition.
have to take care of that nutrition. Next immunization if she is not
Next immunization if she is not immunized against hepatitis B definitely
immunized against hepatitis B definitely I'll go for it and I will vaccinate
I'll go for it and I will vaccinate against hepatitis A also then I will go
against hepatitis A also then I will go into her past history check what is the
into her past history check what is the blood requirement per year in ml per kg
blood requirement per year in ml per kg per year I will calculate if it is more
per year I will calculate if it is more than 250 ml per kg per year then we
than 250 ml per kg per year then we might have to consider
might have to consider because it is almost 8 cm
because it is almost 8 cm sorry
sorry which is huge. Okay.
These are the things we have to do for her. Uh someone is asking um best method
her. Uh someone is asking um best method to assess iron overload. See uh iron
to assess iron overload. See uh iron overload uh two important organs which
overload uh two important organs which we have to assess is the liver and the
we have to assess is the liver and the cardia. Now which is do biopsy is the
cardia. Now which is do biopsy is the gold standard but it is a invasive
gold standard but it is a invasive method. So since we have to go for
method. So since we have to go for noning technique which can be repeated
noning technique which can be repeated at interval is P2 star MRI of the MRI of
at interval is P2 star MRI of the MRI of the liver.
the liver. Okay, that will give us the uh iron
Okay, that will give us the uh iron overload status. And also while you have
overload status. And also while you have started you have taken the started the
started you have taken the started the chilation serum feritin may not
chilation serum feritin may not correlate later as the iron overall
correlate later as the iron overall reduces it may not correlate you may not
reduces it may not correlate you may not may not be a good indicator to follow up
may not be a good indicator to follow up later. So we have to have baseline MRI
later. So we have to have baseline MRI and annually you have to repeat the MRI
and annually you have to repeat the MRI to assess for
and also bone health which is very very important in
important in check calcium vitamin D phosphorus
check calcium vitamin D phosphorus levels is required vitamin D subate
levels is required vitamin D subate patient they are more prone for
patient they are more prone for osteoporosis
osteoporosis so it's required we have to start them
so it's required we have to start them on calcium disphosp
liver biopsy or MRI. Uh actually less 6 months it is MRI only. Now we can do it
months it is MRI only. Now we can do it for 5,000 but biopsy very
for 5,000 but biopsy very same cost but it is invasive.
uh par can be written in chief complaints like uh becoming excessively
complaints like uh becoming excessively white is very difficult to he usually
white is very difficult to he usually mention por in the uh uh chief
mention por in the uh uh chief complaints
so that's all of the doubts we guess ma'am so guys we had ma'am she's
ma'am so guys we had ma'am she's practicing hematoncologist pediatric
hematoncologist. So thank you Asha ma'am for your time.
So thank you Asha ma'am for your time. Thank you so much
Thank you so much and very well presented.
and very well presented. Yeah very good presentation Dr. Niti.
Yeah very good presentation Dr. Niti. Okay
Okay thank you. Thank
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