17year old female with recurrent Respiratory tract infections,anemia,growth retardation

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Unit 1


Dr. Charan PGY1

Dr. Chandana PGY1

Dr. Sushmitha PGY2

Dr. Adithya PGY3

Dr. Praneeth PGY3


Dr.Hareen (SR MD gen med)

Dr. ARJUN (Asst. Prof.gen med)


Dr. RAKESH BISWAS HOD




Here is a case I've seen:


Admission under Unit 1 on 8/03/2021 History taken by


Dr.Chandana Vishwanatham

Dr. Sai charan 

Dr.Susmitha


15year old female who is the first child of a consanguinous married couple

her mother expired during the birth of 3rd child

She has 2 younger brothers who are apparently alright with no health related issues


Since childhood she has been having recurrent Respiratory tract infections(She almost always had cold,fever,cough with sputum) aggrevated during winters

Since 4yrs she is complaining of yellowish discoloration of eyes on and off not preceeded by fever with no h/o pruritus,No history s/o CLD

h/o easy fatigability , generalized weakness since 4years associated with loss of appetite,progressed to great extent in the last 2 to 3months

h/o short stature,failure to gain weight appropriate for her age and delay in secondary sexual characters

Attained menarche in 2020 May had regular cycles of 4months and then  2months of ammenohrea,2months of regular cycle and then followed by amenorrhea till now.


4years back(2016) she had h/o jaundice and anemia for which one blood transfusion was done,her jaundice subsided slowly and she never had jaundice for the next 3yrs

2020 october she again developed yellowish discoloration of eyes which subsided on its own

2021 jan yellowish discoloration of eyes recurred and not subsided till now

h/o 2 blood transfusions in feb 2021

No h/o 

No proper immunization history or documents available

**************************************************

Evaluated in various hospitals since childhood


since the age of

2 years(9/2005),7.3kgs wt, started having fever,cold ,cough with expectoration,during which her x ray showed right middle lobe consolidation,hb 8.8,TLC,plts Normal,CRP strongly positive,smear for MP+

Diagnosis:Failure to thrive with recurrent RTI's

Treatment:cefuroxime drops and treated empirically with ATT(Rifampicin, isoniazid,pyrazinamide for 2months)


even after which she has been having recurrent cold, cough and high grade fevers

23/3/2006:LRTI ,CSOM diagnosed and treated

22/8/2006:B/L bronchopenumonia treated with syp.cefpodoxime

11/09/2006:cough,cold,fever 

13/9/2006(Age 3yrs):Monteux test was done :positive with Erythema 12mm,induration 12mm and was started on ATT again for 2months

Hb 10.8,Tlc ,plts normal

AEC 408


4/1/2007(age 4yrs ,Wt 10.5kgs):Her symptoms did not resolve and continued with ATT for 4more months

15/6/2007:cold,cough,fever (102F)returned

also gave history of itchy lesions over hands and was diagnosed with Scabies and treated with ivermectin,permite cream

20/10/2007:Acute LRTI treated with antibiotics



27/05/2009and 11/07/2009:acute LRTI treated with antibiotics 

7/9/2009:Hb 8.5,RBC 3million,AEC 476,


11/11/2010:LRTI


6/4/2011(Age 8yrs,Wt 11kgs) :LRTI treated with Iv antibiotics and inj deriphylline

serum ADA 10(normal <30)

12/10/2011:CT chest:Few prominent bronchi left lower lobe,B/L ground glass opacities

USG :Borderline spleenomegaly

10/12/2011 :LRTI

14/10/2011: Recurrent RTI,Failure to thrive, Protein energy malnutrition

Their differentials were 1.PEM with kochs,2.PEM with bronchiolitis,3.PEM with ?Enteric fever,4PEM with ARI/Hyperactive airway disease

Hb 10.8,WBC , platelets normal,MCV 68,McH 20.5,MCHC 30.5

serology negative

RFT,SGPT,ALP normal




2012 to 2014:5episodes of LRTI (Fever,cold,cough with sputum)

age 9yrs wt 13kgs

age 11yrs wt 16kgs

2/10/2014:Was having lesions on sides of neck and was treated with Acyclovir for Herpes




11/4/2016**:For the first time along with fever,cold and cough pt developed yellowish discoloration of eyes

Hb :9.0,RBC:3.1,TLC and platelet normal

TB 2.1mg/dl

1PRBC transfusion was done

Treated as ?Viral hepatitis


5/07/2016:cough+,chest x ray:Interlobar effusion upper and middle lobe, prominent brinchivascular markings? Bronchitis

9/8/2016,11/10/2016:LRTI




12/03/2017(13yrs 23kgs):LRTI,chest x ray :B/L upper lobe consolidations,hb 10.2,CUE:pus cells 19-20,alb trac

15/8/2017,7/10/2017,18/10/2017: Recurrent LRTIs

31/10/2017:Hb 10.6,TLc platelet normal



*************************************************

13/11/2017

For the first she visited our Hospital with c/o cough since 10days,sputum,SOB grade 1

In the background of recurrent LRTI,2D echo was done

2D echo in our hospital showed Moderated SA VSD( left to right shunt),EF 68%,Good LV systolic function,trivial TR

hb 11,TLC 6200,plt 5lakhs

pt was immediately taken to cardiologist


2D echo at an outside corporate hospital:normal sized chambers,No RWMA,Normal LV/RV function,

nothing mentioned about VSD

so she came back to our hospital and was in follow up with dept of TB and chest for 2 yrs till the end of 2018 being treated for allergic brinchitis

She was prescribed with various medicines for her Recurrent LRTIs(symbecort inhalers,moteleukast,aphylline tabs,Tab ferreo XT etc) and nothing helped



8/7/2019 to October 2019(age 14yrs wt 24kgs): They stopped coming to our hospital and went to other local hospitals for RTIs

Human growth hormone was done:4.53ng/ml (which is normal)

USg moderate spleenomegaly

hb 8.1,TB 2.1mg/dl


30/5/2020:loss of appetite started and on and off pain abdomen(subsided later) upon her background of LRTI continued

16/08/2020 :Hb 8.1,RBC 3.7million,MCV 77,MCH 27.1,TB 3.9mg/dl

5/10/2020:Her yellowish discoloration of eyes recurred lasted for about 1 month and subsided on its own(TB 4.4mg/dl-->1.2)



18/1/2021:she again developed yellowish discoloration of eyes ,TB 3.9,direct 2.6,indirect 1.3, with mild spleenomegaly on USG

?Gilbert,? Hemolytic anemia

24/1/2021 

upper GI endoscopy done:Normal

26/1/2021

Hb 5.6,RBC dropped to 1.2M***,MCV 141,MCH 36,Dimorphic picture shows macrocytic,normochromic ovalocytes ,tear drop cells and 7-8 rbcs/100wbcs


High performance liquid chromatography

HbA 87.4%

HbF 0.6%(<1 is normal)

HbA2 :3(normal 2-3.5)


serology negative


urine reactive for bile salts and bile pigments

Hb 6.0,RBC 1.9M,TB 3.1


**************************************************

She was taken to NIMS admitted under gastroenterology from 4/2/2021 to 13/2/2021


Hb 9.6

TLC plts normal

retic count 4%

retic index 1.85%

RFT normal

coombs DCT,ICT negative

thyroid function tests normal

ANA immunofluorescence negative

IgG TTG negative

anti endomyseal ab negative

G6PD 30.3 (normal)

LDH 544(raised)


Was prescribed with oral iron and B12 for 2weeks and she came back home

*************************************************

 After 4days 17/2/2021 her generalized weakness,loss of appetite and jaundice has aggrevated for which she was admitted in local hospital where 2 blood transfusions were done

Before admission:Hb 6.5,TB 3.9,RBC 2M

At the time of discharge(20/2/2021)Hb 10.0,TB 2.2,RBC 4M

Total protein :6mg/dl



**************************************************


After 4days she was taken to a top govt hospital with drop in Hb again post transfusion (Hb 9.3,RBC 3M,plt 2L,DCT 3+)

1/3/2021 In osmania pt was adviced for vit B12 levels,folic acid levels,osmotic fragility test,serum ferritin,hb electrophoresis again


From ther they went to a top corporate hospital where pt was diagnosed with ?AIHA 

adviced for clinical exome sequence,they gave the sample for sequencing ,it takes one month for the report(report awaited)

and was started on Tab Wysolone 20mg OD from march 1st 2021 and was asked to review after 2 weeks

but in the meanwhile pt and her attendors felt that her jaundice and her generalized weakness progressed suddenly and was brought to our hospital.


Hemoglobin chart from childhood



Bilirubin chart from 2016(no previous values available)



**************************************************


O/E pt short thin built,Wt 28kgs

Ht 140cms






gen exam:Pallor+,Icterus+,




No lymphadenopathy,pedal edema

JVP raised


RS:

chest expansion 1.5cms which appeared a little less on right side

dull note on percussion of Rt ISA,all other lung fields resonant

Auscultation:BAE+,?Bronchial BS rt ISA and decreased Air entry rt ISA


P/A

shape normal

umbilicus central , inverted

no increase in local temp,no tenderness

Mild spleenomegaly+

No hepatomegaly

BS+

No free fluid


CNS

HMF intact

Motor

Power 5/5all 4limbs

Reflexes.   r.       L

B.                1+.      -

T.                 1+.      2+ 

K.                 2+.      2+

A.                 -.         -

Plantar.      F.         F

Sensory :Normal

Cerebellar function tests normal


Diagnosis:

Autoimmune hemolytic anemia

?Common variable immunodeficiency syndrome

Recurrent RTIs

Indirect hyperbilirubinemia

Failure to thrive


hb 3.2

tlc 8,200

plt 3.0l

RBC 0.8M**

MCV 116

MCH 37

MCHC 31

RDW 25(increased)


TB 8.16

DB 0.77

AST 43

Alt 23

ALP 113

Tp 5.9

Alb 4.6

A/G 3.4

narrow gamma gap is noted



RFT normal


PBS macrocytes,macroovalocytes,anisopoikilocytosis with hypochromia




coombs:

DCT 4+(positive)

ICT 1+

Auto control 3+


[The direct Coombs test is used to detect antibodies (IgG or C3) that are stuck to the surface of red blood cells. 

The indirect Coombs test looks for free-flowing antibodies against certain red blood cells. It is most often done to determine if you may have a reaction to a blood transfusion.
 The transfusion dept has added the centrifuged and suspension of pts rbcs 50micrograms to the kit which they are provided with.It has coated anti human antibodies in it anta.after adding pts suspended rbcs to the kit they again centrifuge it and incubate it..after some time grade it accordingly]


Chest x ray day1







?Delayed hemolytic transfusion reaction
?Common variable immunodeficiency
Auto immune hemolytic anemia
? Hypopitutarism


Day 2

17year old female
? Delayed hemolytic transfusion reaction
?CVID with AIHA
Recurrent Respiratory tract infections
Short stature,delayed puberty (?hypopitutarism)

C/O cough on and off
No fever spikes


O/E 
Pt c/c
PR 130bpm
RR 40/min
BP 100/60
cvs s1s2+
Rs BaE+, decreased air entry rt ISA
P/A soft NT spleenomegaly


Pt was started on inj dexa,inj ceftriaxone,Tab Azithromycin (according to body wt)

Sample sent for serum immunoglobulin levels

Transfusion done with 1prbc after testing many bags from blood bank,they provided us with best compatible possible that is on gel card method 1+,as her blood is incompatible with many other blood bags





Day 3


17year old female
?CVID with AIHA
Recurrent Respiratory tract infections
Short stature,delayed puberty (?hypopitutarism)

C/O cough on and off
No fever spikes
No loose stools

O/E 
Pt c/c
PR 120bpm
RR 34/min
BP 90/60
cvs s1s2+
Rs BaE+,diffuse fine crepts+
P/A soft NT spleenomegaly

Inj dexa 2mg BD
Inj ceftriaxone 500mg Iv BD
Tab Azithromycin 250mg PO Od
Tab vit b12+folic acid Po od
Tab lasix 20mg PO BD

Chest x ray and x ray wrist to be done 
Transfusion done with 1Prbc










Questions around the pt

1.Efficacy of IV Ig in CVID?
2.Can her immunization be optimised now?
3.Could it be delayed hemolytic transfusion reaction during presentation (transfusion done last month)?
4.Cause of her short stature and delayed puberty?(? hypopitutarimsm,? secondary to chronic anemia?)
5.could it be simple IgG deficiency?
6.Could it be chronic granulomatous disease?
7.Alternatives for transfusion -leuckocyte reduction before transfusion?saline wash of donor rbcs ??






COMMON VARIABLE IMMUNODEFICIENCY



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