17year old female with recurrent Respiratory tract infections,anemia,growth retardation
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Unit 1
Here is a case I've seen:
Admission under Unit 1 on 8/03/2021 History taken by PGs
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 wintersSince 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 CLDh/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
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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
Patient 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 RTI
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
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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)
544(raised)
Was prescribed with oral iron and B12 for 2weeks and she came back home
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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
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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 there 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 attenders 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)
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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 5/5 all 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
Patient was started on inj dexa, inj ceftriaxone, Tab Azithromycin (according to body weight)
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
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 ?
@PPM2 when she was admitted recently with hemolysis we didn't repeat the chest X-ray?
Can you share the last EMR summary of her discharge?
PPM 1 - She had to visit a nursing home in Nalgonda for another doctor to write her chest X-ray!
And they topped it up as usual with an HRCT of chest
@PA - Before treatment government DOTs centre do AFB test free and then they give medicines for free.
PPM 1 - Thanks for the chest X-ray. Doesn't appear to have been captured well?
Thanks!
This is better and while in retrospect this appears to have heralded her current pulmonary symptoms I guess we didn't focus on it as 1} it wasn't shared here for collective team cognition and 1} she didn't have pulmonary symptoms at that time.
Captured her chest X-ray better on our view box instead of yesterday's home picture held against the sun.
Findings suggestive predominantly of bilateral proximal bronchiectasis! ABPA!!!?
Even their spiral CT machine appears to be quite old looking at the HRCT films, also they haven't
mentioned any details of their hardware such as how many sliced spiral CT etc! This center may have been using a refurbished machine to make ends meet.
Noticed the point about delayed puberty made by our PG in her case report in 2021 and @PPM3 is examining her again now to reassess her current pubertal status.
Reminds us of another 19M with hypersplenism who also visited today @PPM4.
We realised 18 yrs is her Aadhar status and her real age is 21!
Also @PPM4 reports that her secondary sexual characteristics in terms of axillary pubic hair and breast development is minimal.
OBG note of her current sexual maturity
PPM 1 -
Found this written in her 2023 discharge summary while compiling all of them into one fresh case report with last few months updates👇
"TREATED EMPIRICALLY WITH ATT (RIFAMPICIN, ISONIAZID, PYRAZINAMIDE FOR 2 MONTHS)
EVEN AFTER WHICH SHE HAS BEEN HAVING RECURRENT COLD, COUGH AND HIGH GRADE FEVERS"
Age/Gender : 18 Years/Female
Address :
Discharge Type: Relieved
Admission Date: 08/03/2021 05:55 PM
Diagnosis
COMMON VARIABLE IMMUNODEFICIENCY WITH AUTOIMMUNE HEMOLYTIC ANAEMIA WITH RECURRENT RESPIRATORY TRACT INFECTIONS, GROWTH RETARDATION, DELAYED PUBERTY ? SECONDARY TO CHRONIC ANAEMIA AND SECONDARY AMENORHOEA.
Case History and Clinical Findings
A 17 year old lady presented to the emergency room with jaundice since 2 days.
She also reported exertional dyspnea, fatigue and pedal edema since 2 days. Her past history was also significant for recurrent childhood lung infections , ear and sinus infections.
She also has been having persistent anemia since early childhood. She also had significant developmental abnormalities with short stature and wasting and also delayed menarche.
Dietary history was insignificant and she did not have any bowel or bladder dysfunction.
Clinical examination
from head to toe revealed a patient with short stature and in mild respiratory distress. vitals :
at presentation were - PR - 150 BPM BP - 100/60 mm Hg
Temp - 98.4 F RR - 26/min
Spo2 - 98% on room air GRBS - 130 mg/dl
The patient had severe conjunctival pallor and lemon yellow scleral icterus.
She did not have any features of active sinusitis, tongue was pale and bald. cervical, axillary or inguinal lymph nodes were not palpable. Skin turgor was normal and no rashes were observed. Her nails were pale and showed Quincke's pulsations, consistent with high output state/failure. She also had cervical venous hum and prominent abdominal aortic pulsations.
Examination of feet showed pitting type pedal edema extending upto her ankles.
Systemic examination: was significant for hyperdynamic precordium, palpable P2 and raised JVP. Auscultaton revealed a loud P2 with a pansystolic murmur along the left sternal border accentuating on inspiration. She also had an S3 gallop, accentuating on inspiration.
Auscultation of the right femoral artery with occlusion of forward flow revealed a diastolic bruit. Respiratory system exam was significant for decreased intensity of breath sounds.
Per Abdomen examination showed moderate splenomegaly and mild hepatomegaly.
CNS exam was insignificant. After reviewing her past medical records and her current history and physical exam, she was diagnosed with Autoimmune Hemolytic Anemia with Acute High Output Heart Failure.
Course in the Hospital - The patient was admitted with severe anemia and jaundice leading to acute high output heart failure. Her labs showed a Hb of 3.2 gm% and Total Bilirubin of ( 8. 16 MG /DL ) and Direct Bilirubin ( 0.77 MG/DL ).
Samples for Direct Coomb's test returned positive with 4 + agglutination, consistent with her previous reports. At this point, she was diagnosed with Autoimmune Hemolytic Anemia. Causes of AIHA in the background of recurrent sino-pulmonary infections were reviewed and the patient appeared to have a primary immunodeficiency disorder. She also had a low gamma gap on LFT suggesting hypogammaglobulinemia.
Her CXR-PA and Right Lateral views showed Rt. Lower Zone Bronchiectasis, findings consistent with recurrent lower respiratory tract infections.
An ENT consult was taken for her past history of ear infections. She had bilateral subtotal tympanic perforations with no active discharge or conductive hearing loss. Her short stature and delayed menarche were also considered and we considered the possibility of autoimmune hypophysitis.
Review of literature showed that AIHA (reference 1) is the most common autoimmune manifestation of primary immunodeficiency disorders but hypophysitis was extremely uncommon. However the patient was fitting into this clinical picture also. Bone age of the patient was checked with x-rays of the bilateral wrists. The sequential bone age evaluation, using Gilsanz and Ratib's Atlas (reference 2) was done and her bone age was at 17 to 21 years, consistent with her chronological age.
An endocrine consult was taken - the case was discussed and it was attributed to chronic anemia and recurrent infections in childhood and not pituitary deficiency per se. Because her growth plates fused, increase in height cannot be possible. Hence, further evaluation of her short stature and delayed menarche was abandoned (prohibitive costs notwithstanding).
Blood transfusion was considered but the patient was in active hemolysis stage and when screening for donor blood, the patient's serum immediately hemolysed the donor blood. An extensive search in the blood bank was done and 1 packet of PRBC which showed no reaction was made available and transfused successfully. The patient reported good improvement in fatigue and appetite. She was already on steroids prior to admission and her doses were tapered to Tab Prednisone 30 mg in the morning and Tab Prednisone 20 mg at night. Antibiotics were considered and Tab Azithromycin 250 mg OD was started.
She also received a test dose for Ceftriaxone and after she showed no adverse reaction, Inj Ceftriaxone 500 mg BD was started safely. Serum Ig levels were sent for and all Immunoglobulins (IgG, IgM and IgA) returned significantly low. After excluding confounding factors, these values were consistent with a diagnosis of Common Variable Immunodeficiency (CVID). Futher, she did not receive iron supplements as she was at risk of secondary hemochromatosis due to chronic hemolysis. She received B12 and Folic acid therapy.
To summarise - She presented with severe hemolytic anemia and reccurent infections. She was diagnosed with CVID causing AIHA. CVID was diagnosed with severely depleted Ig levels. Hemolytic crisis was addressed with oral steroids and severe anemia was treated with 1 blood transfusion. Her hemolytic crisis waned gradually with spontaneous resolution of jaundice and rapid improvement in Hb and symptoms. Overall, the course of hospitalization was uneventful.
Treatment Strategies - Once the diagnosis of CVID was established, therapy with IVIg is usually initiated. We reviewed the literature on the efficacy of IVIg in CVID patients (reference 3) and found that - (summarised briefly)1. Reduced incidence of new respiratory tract infections. 2. Reduced antibiotic usage. 3. Reduced duration and number of hospital admissions. Because we found convincing benefits with IVIg therapy, we discussed with the patient regarding monthly IVIg injections. The patient's father agreed, however cost was proving prohibitive. We are therefore, referring this case to Osmania General Hospital so that the patient can avail IVIg injections free of cost.
REFERENCES -
1. https://twitter.com/AvrahamCooperMD/status/1370913647862632448?s=082.
2. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3955574/# ffn_ sectitle'By the age of 18 years, bone age cannot be computed from hand &wrist radiographs, therefore the medial end of the clavicle is used for bone age calculation in individuals aged 18—22 years'
3.https://pubmed.ncbi.nlm.nih.gov/22312934/
Investigation
2D ECHO - EF - 64 %
MILD TR + WIYH PAH AND TRIVIAL AR + /MR+ NO RWMA , NO AS/ MS
GOOD LV SYSTOLIC FUNCTION , NO PE CHEST X RAY -
TINY HYPERLUCENT AREAS MOTED IN RIGHT LOWER ZONE AND BILATERAL PERIPHERAL REGION LIKELY BRONCHIECTASIS CHANGES .
USG ABDOMEN -
MODERATE SPLEENOMEGALY. SERUM IgG - 332 MG/DL
SERUM Ig M - LESSTHAN 20 MG /DL
SERUM Ig A - LESSTHAN 10 MG/DL X RAY :
WRISTBONE AGE 17- 21 YEARS.
SCLEROSIS OF PROXIMAL AND MIDDLE PHALANGES - NON SPECIFIC FINDINGS.
Treatment Given(Enter only Generic Name)
1. TAB. VIT B12 AND FOLIC ACID PO /OD FOR ONEWEEK
2. BP/PR/TEMP/SPO2/GRBS MONITORING
3. Transfusion done with 1prbc
4. TAB. PREDNISONE 30MG morming and 20 mg at night PO /OD for 5 days 5.SYP. AMBROXYL PO / TID
5ML--5ML--5ML
6.TAB. AZITHROMYCIN 250 MG PO/OD for one week.
Advice at Discharge
1. TAB. PREDNISONE 40MG PO /OD
1--X--X
2. TAB. VIT B12 AND FOLIC ACID PO/OD FOR 2 WEEKS X---1---X
3. SYP. AMBROXYL PO / TID FOR 2 DAYS
5ML--5ML--5ML
4. NEEDS 10 GM IVIG/MONTH
BLOG LINK:
When to Obtain Urgent Care
IN CASE OF ANY EMERGENCY IMMEDIATELY CONTACT YOUR CONSULTANT DOCTOR OR ATTEND EMERGENCY DEPARTMENT.
Preventive Care
AVOID SELF MEDICATION WITHOUT DOCTORS ADVICE, DONOT MISS MEDICATIONS. In case of Emergency or to speak to your treating FACULTY or For Appointments, Please Contact: For Treatment Enquiries Patient/Attendent Declaration: - The medicines prescribed and the advice regarding preventive aspects of care, when and how to obtain urgent care have been explained to me in my own language
SIGNATURE OF PATIENT /ATTENDER
SIGNATURE OF PG/INTERNEE
SIGNATURE OF ADMINISTRATOR
SIGNATURE OF FACULTY
Discharge Date
Date: 22/3/21 Ward: FMW Unit:1
Admission Date: 17/11/2021 04:51 PM
Name of Treating Faculty
Diagnosis
ACUTE ON CHRONIC HEMOLYTIC ANEMIA SECONDARY TO ? VIRAL HEPATITIS CVID WITH AIHA
Case History and Clinical Findings
A 15 yr old female came to casualty with chief complaints of1- Yellowish discoloration of eyes since 5 days2- Vomitings since 3 days
HISTORY OF PRESENTING ILLNESS
Patient was apparently asymptomatic 5 days ago then she developedYellowish discoloration of eyesit wasassociated with dark coloured urineVomiting since 3 days , 3 episodes - Non bilious, non projectile , food as contentMosquito bites 2 days ago followed by rashes on B/L limbsNo history of fever, abdominal pain, cough, headache, constipation, diarrheaPAST HISTORYK/c/oCVID with AIHAadmitted in February and got dischargedIn detailPrevious historyduring her lastadmissionand her previous medical conditions (since birth) can be found in the case report link attached below - https://chandanavishwanatham19.blogspot.com/2021/03/17year-old-female-with- recurrent.html?m=1After the discharge there was h/o fall in hemoglobinPrevious h/o 4 blood transfusions doneIn August- 2 pint PRBS transfusion doneAfter August PRBC ,Since dischargePt is on IVIG, Oral steroids every monthStarted on TAB AZATHIOPRINE 50mg ODIn September - 1 pint PRBS transfusion doneIn september during her follow upShe is presently on TAB WYSOLONE 10mg OD,TAB AZATHIOPRINE 50mg ODBut according to attendants this time her yellowish discoloration is more severeK/C/OBronchial Asthmasince 10 yearsDiagnosed at Age 3Y , initially used Syrup , tablets and In 2016 at our hospital Inhaler was administered as the symptoms were severe and stopped using it 1 year later since symptoms subsided (Unknown medication)Tuberculosis diagnosed at Age 3Y - Used aTT medication for 6 monthsNot a K/c/o DM, Hypertension , EpilepsyPersonal history:Appetite -normalBowel movement- regularMicturition - normalNo known allergiesNo addictionsNo significant family historyGeneral examinationPallor+Icterus+No cyanosis,clubbing, lymphadenopathy and edemaTemp-98.6F.Pulse: 80bpmRespiration: 20 cpmBP - 100/60 mmHgSpO2
- 99% at RACVS:S1,S2 heardRS: BAE +,NVBS heardPer Abdomen - soft,non tender , Moderate splenomegalyCNS - NAD
Treatment Given(Enter only Generic Name)
1) Tab. Predinisolone 20 mg po/OD2) Tab.Azathioprine 50 mg po/OD3) Tab.Doxycycline 50 mg po
/BD4) Tab.Folvite 5 mg po/OD5) Tab Orofer -XT po/OD
Advice at Discharge
TAB PREDNISOLONE 20mg PO/ODTAB FOLVITE 5mg PO/ODTAB OROFER XT PO/ODTAB SHELCAL PO/ODCAP BIO D3 PO/OD ( Weekly once)CONTINUE IV IMMUNOGLOULINS TREATMENTSYP ARISTOZYME PO BD 1 -- X -- 1
Follow Up
REVIEW TO GM OP ON 26/11/21 WITH LFT , HEMOGRAM OR REVIEW SOS
When to Obtain Urgent Care
IN CASE OF ANY EMERGENCY IMMEDIATELY CONTACT YOUR CONSULTANT DOCTOR OR ATTEND EMERGENCY DEPARTMENT.
Preventive Care
AVOID SELF MEDICATION WITHOUT DOCTORS ADVICE, DONOT MISS MEDICATIONS. In case of Emergency or to speak to your treating FACULTY or For Appointments, Please Contact: For Treatment Enquiries Patient/Attendent Declaration: - The medicines prescribed and the advice regarding preventive aspects of care, when and how to obtain urgent care have been explained to me in my own language
SIGNATURE OF PATIENT /ATTENDER
SIGNATURE OF PG/INTERNEE
SIGNATURE OF ADMINISTRATOR
SIGNATURE OF FACULTY
Discharge Date
Date:22/11/21
Ward: MEDICAL WARD
Unit:3
Age/Gender : 18 Years/Female
Address :
Discharge Type: Relieved
Admission Date: 06/08/2022 02:15 PM
Name of Treating Faculty
Diagnosis
ACUTE GASTROENTERITIS WITH K/C/O CVI
Case History and Clinical Findings
PATIENT WAS APPARENTLY ASYMPTOMATIC SINCE 2 DAYS BACK AND THEN HE HAD CONSUMED OUTSIDE FOOD ON THURSDAY WITH
H/OLOOSE STOOLS SINCE 3 DAYS
NOT ASSOCIATED IWTH BLOOD IN STOOLS
H/O 2 EPISODES OF FEVER AND ONE EPISODE ON THURSDAY NIGHT ASSOCIATED WITH CHILLS AND RELIEVED ON MEDICATION
1 EPISODE ON FRIDAY MORNING
NO H/O ABDOMINAL PAIN AND VOMITING
NOT A K/C/O HTN , DM , TB , EPILEPSY , ASTHMA K/C/O CVLD WITH AIHA
NO SIGNIFICANT FAMILY AND DRUG HISTORY
GENERAL EXAMINATION : PATIE NT IS CONCIOUS , COHERENT , COOPERATIVE
NO PALLOR , ICTERUS , CYNAOSIS , CLUBBING , KOILONYCHIA , LYMPHADENOPATHY , EDEMA
BP - 100/60 MM HG HR - 106 BPM
TEMP - 98 F RR - 22 CPM
SYSTEMIC EXAMINATION CVS - S1 S2 HEARD
RS - BAE + CNS - INTACT
PA - SOFT , NONTENDER
Investigation
ULTRASOUND FINDINGS - BOWEL WALL EDEMA LIKELY INFECTIVE ETIOLOGY SPLENOMEGALY
STOOL C/S - PLENTY OF PUS CELLS SEEN NO OVA AND CYST SEEN
NORMAL INTESTINAL FLORA HEMOGRAM- HB-10.9
TLC-2000 UREA- 29
SERUM CREAT-0.1 N/K/CL-135/3.4/101 CA-7.3
RBC-3.46 PCV-32.7
Treatment Given(Enter only Generic Name)
1 IVF NS AND RL 50 ML /HR
2 TAB SPOROLAC PO TID
3 TAB PAN 40 MG PO OD
4 TAB DOLO 650 MG PO BD
5 PLENTY OF ORAL FLUIDS
6 ORS SACHETS IN 1 LIT WATER PO TID
7 BP AND HR , TEMP CHARTING
8 TAB OFLOX OZ PO BD
9 SYRUP POTCHLOR PO TID
Advice at Discharge
1 TAB DOXYCYCLINE 100 MG PO/OD FOR 5 DAYS
2 ORS SACHETS IN 1 LITER/SOS
3 TAB MVT PO/OD FOR 5 DAYS 5
4 ADEQUATE FLUID INTAKE
Follow Up
REVIEW TO GENERAL MEDICINE OP AFTER 1 WEEK
When to Obtain Urgent Care
IN CASE OF ANY EMERGENCY IMMEDIATELY CONTACT YOUR CONSULTANT DOCTOR OR ATTEND EMERGENCY DEPARTMENT.
Preventive Care
AVOID SELF MEDICATION WITHOUT DOCTORS ADVICE, DONOT MISS MEDICATIONS. In case of Emergency or to speak to your treating FACULTY or For Appointments, Please Contact: For Treatment Enquiries Patient/Attendent Declaration: - The medicines prescribed and the advice regarding preventive aspects of care, when and how to obtain urgent care have been explained to me in my own language
SIGNATURE OF PATIENT /ATTENDER
SIGNATURE OF PG/INTERNEE
SIGNATURE OF ADMINISTRATOR
SIGNATURE OF FACULTY
Discharge Date Date:11/8/22 Ward: general medicine Unit:6
Age/Gender: 18 Years/Female
Address:
Discharge Type: Relieved
Admission Date: 01/05/2023 03:25 PM
Name of Treating Faculty
Diagnosis
?AUTOIMMUNE HEMOLYTIC ANEMIA
?COMMON VARIABLE IMMUNODEFICIENCY WITH PRIMARY OPTIC ATROPY
Case History and Clinical Findings
17 YEAR OLD FEMALE K/C/O COMMON VARIABLE IMMUNODEFICIENCY WITH AUTOIMMUNE Hemolytic ANEMIA
C/O NYSTAGMUS SINCE 2 YEARS CAME FOR FOLLOW UP
HOPI:
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
PERSONAL HISTORY- DIET: MIXED APPETTITE:NORMAL
BOWEL AND BLADDER: REGULAR SLEEP: ADEQUATE
NO ADDICTIONS
GENERAL EXAMINATION-PATIENT IS CONSCIOUS,COHERENT,COOPERATIVE WELL ORIENTED TO TIME,PLACE,PERSON
MODERATLY BUILT AND NOURISHED
VITALS-
TEMPERATURE-AFEBRILE PR-66BPM
BP-90/60 RR-16CPM
SYSTEMIC EXAMINATION-
CVS-S1S2 HEARD, NO MURMURS RS-BAE+, NVBS HEARD
CNS-NFND, HMF INTACT
P/A-SOFT, NON TENDER, NO ORGANOMEGALY
OPTHALMOLOGY REFERRAL TAKEN ON 2/5/23: DIAGNOSIS: PRIMARY OPTIC ATROPHY
IMPRESSION:NO ACTIVE OPTHALMOLOGICAL INTERVENTION NEEDED
ENT REFERRAL TAKEN ON 2/5/23 I/V/O PT C/O NYSTAGMUS DIAGNOSIS: NYSTGMUS UNDER EVALUATION
ADVISED:
1. CIPLOX EAR DROPS 2 DROPS AT 8AM,2PM,8PM FOR 5 DAYS
2. T.LEVOCET 5MG BEFORE BED FOR1 WEEK
3. KEEP EAR DRY
4. OTRIVIN NASAL DROPS 3 DROPS AT 8AM,2PM,8PM FOR 5 DAYS
Investigation
USG: MILD SPLENOMEGALY MRI DONE ON 1/5/23 HEMOGRAM :1/5/23 HB:10.4GM/DL TC:11,000CELLS/CUMM
N/L/E/M:63/31/1/5
RBC COUNT:4.94MILLIONS/CUMM PLT:2.15LAKHS/CUMM
Treatment Given(Enter only Generic Name)
1. CIPLOX EAR DROPS 2 DROPS AT 8AM,2PM,8PM FOR 5 DAYS
2. T.LEVOCET 5MG BEFORE BED FOR1 WEEK
3. KEEP EAR DRY
4. OTRIVIN NASAL DROPS 3 DROPS AT 8AM,2PM,8PM FOR 5 DAYS
5. T.MVT PO/OD FOR 5 DAYS
Advice at Discharge
1. CIPLOX EAR DROPS 2 DROPS AT 8AM,2PM,8PM FOR 5 DAYS
2. T.LEVOCET 5MG BEFORE BED FOR1 WEEK
3. KEEP EAR DRY
4. OTRIVIN NASAL DROPS 3 DROPS AT 8AM,2PM,8PM FOR 5 DAYS
5. T.MVT.PO/OD FOR 5 DAYS
Follow Up
REVIEW TO MEDICINE OPD AFTER ONE WEEK REVIEW TO OPTHALMOLOGY OPD ON FRIDAY REVIEW TO ENT OPD AFTER 5 DAYS
When to Obtain Urgent Care
IN CASE OF ANY EMERGENCY IMMEDIATELY CONTACT YOUR CONSULTANT DOCTOR OR ATTEND EMERGENCY DEPARTMENT.
Preventive Care
AVOID SELF MEDICATION WITHOUT DOCTORS ADVICE, DONOT MISS MEDICATIONS. In case of Emergency or to speak to your treating FACULTY or For Appointments, Please Contact: 08682279999 For Treatment Enquiries Patient/Attendent Declaration: - The medicines prescribed and the advice regarding preventive aspects of care, when and how to obtain urgent care have been explained to me in my own language SIGNATURE OF PATIENT /ATTENDER
SIGNATURE OF PG/INTERNEE
SIGNATURE OF ADMINISTRATOR
SIGNATURE OF FACULTY
Discharge Date Date:03/05/2023 Ward: FMW Unit:1
Age/Gender: 18 Years/Female
Address:
Discharge Type: Relieved
Admission Date: 10/04/2024 11:26 AM
Diagnosis
HEMOLYTIC CRISIS PRECIPITATED BY RESPIRATORY INFECTION HIGH OUTPUT CARDIAC FAILURE SECONDARY TO SEVERE ANEMIA K/C/O AIHA
CVID ON IVIG THERAPY B/L OPTIC ATROPHY
ONYCHOMYCOSIS OF LEFT FINGER NAILS
Case History and Clinical Findings
PATIENT CAME WITH C/O YELLOWISH DISCOLOURATION OF EYES SINCE 8 DAYS FEVER SINCE 5 DAYS
COUGH SINCE 5 DAYS
HISTORY OF PRESENTING ILLNESS
PATEINT WAS APPARENTLY ALRIGHT UNTIL 8 DAYS AGO THEN HAD C/O YELLOWISH DISCOLOURATION OF EYES A/W WEAKNESS AND PALENESS OF LIPS.
C/O FEVER SINCE 5 DAYS LOW GRADE N/A/W CHILLS AND RIGORS INTERMITTENT WITH DIURNAL VARIATION AGGRAVATED ON NIGHTS AND RELIEVED ON MORNING A/W NAUSEA, LOSS OF APPETITE, HEADACHE.
C/O COUGH SINCE 5 DAYS PRODUCTIVE COUGH, GREEN COLOUR, SCANTY IN AMOUNT
,THICK IN CONSISTENCY, NON MUCOID DURING NIGHT TIME A/W SOB GRADE 3. N/C/O DARK/YELLOW COLOURED URINE,
N/C/O PAIN ABDOMEN LOOSE STOOLS, CONSTIPATION,VOMITING.
N/C/O HAIR LOSS,WEIGHT GAIN/WEIGHT LOSS, HEAT/COLD INTOLERANCE NO C/O CHEST PAIN ,PALPITATIONS,ORTHOPNEA,PND
NO C/O INCREASED/DECREASED URINE OUTPUT ;BURNING MICTURITION. PAST HISTORY
H/O RECURRENT INFECTIONS SINCE CHILDHOOD H/O IVIG SINCE 2020 MONTHLY
LAST TOOK IVIG ON 2/4/2024
N/K/C/O HTN,DM,ASTHMA,TB,EPILEPSY,CVA,CAD.
O/E
PATEINT IS C/C/C TEMPERATURE 99.1F PR 120BPM
RR 28CPM
BP 100/50MMHG SPO2 100% GRBS 104MG/DL
PALLOR : PRESENT ICTERUS : PRESENT
CLUBBING : PRESENT ON RIGHT HAND CYANOSIS : ABSENT
PEDAL EDEMA :ABSENT
GENERALISED LYMPHADENOPATHY :ABSENT JVP NOT ELEVATED
CVS S1 S2 HEARD , NO MURMUR
APICAL IMPULSE IN 5TH ICS MEDIAL TO MIDCLAVICULAR LINE PARASTERNAL HEAVE PRESENT
RS
TRACHEA CENTRAL
DYSPNOEA PRESENT ON WALKING AND TALKING BAE PRESENT;NVBS
B/L CREPTS IN IAA ,ISA,MA .
P/A
SOFT AND NON TENDER
NYSTAGMUS : SPONTANEOUS &EVOKED NYSTAGMUS+ ON DAY-6 SPLEEN: NOT PALPABLE
LIVER : NOT PALPABLE CNS:
RIGHT LEFT TONE: UL N N LL N N POWER
UL 5/5 5/5 LL 5/5 5/5 REFLEXES
BICEPS 2+ 2+
TRICEPS 2+ 2+
SUPINATOR 2+ 2+
KNEE 2+ 2+
ANKLE 2+ 2+
PLANTER FLEXION FLEXION ROMBERGS NEGATIVE TANDEM WALKING NEGATIVE
DVL: REFERAL I/V/O NAIL CHANGES
ADVICED :40%KOH MOUNT (NAIL CLIPPINGS) SHOWED NO FUNGAL ELEMENTS.
REVIEW REFERAL: O/E BLACKISH DISCOLOURATION OF NAILS WITH ONYCHOLYSIS AND PITTING NOTED OVER RIGHT HAND FINGER NAILS SPARING INDEX FINGER AND ONYCHOLYSIS OF RT SECOND TOE.
ADVISED: AMOROLFINE NAIL LAQUER LIA WEEKLY ONCE FOR 4 WEEKS. IMPRESSION B/L PRIMARY OPTIC ATROPHY, NO ACTIVE INTERVENTION REQUIRED. I/V/O OPTIC ATROPHY, VISUAL FIELD TESTING WAS DONE B
IMPRESSION: RESULTS ARE NOT RELIABLE AS PATIENT IS NON CO-OPERATIVE
Investigation
Haemogram: (10/04/24)
HAEMOGLOBIN:3.8gm/dl
TOTAL COUNT:7600cells/cumm NEUTROPHIL:66% LYMPHOCYTES:27% EOSINOPHILS:1% MONOCYTES:6% BASOPHILS:00%
PCV: 13.2VOL% MCV :125.7fl MCH :36.2pg MCHC :28.8%
RBC COUNT :1.05 millions/cumm PLATELET COUNT:3.2 lakhs/cu.mm SMEAR
RBC: Normocytic normochronic macrocytes seen
Haemogram: (12/04/24) HAEMOGLOBIN:3.9gm/dl
TOTAL COUNT:7000cells/cumm NEUTROPHIL:75% LYMPHOCYTES:22% EOSINOPHILS:01% MONOCYTES:02% BASOPHILS:00%
PCV: 13.4vol% MCV :122.9fl MCH :35.8pg MCHC :29.1%
RBC COUNT :1.09 millions/cumm PLATELET COUNT:3.3 lakhs/cu.mm SMEAR
RBC: Anisopoikilocytosis with microcytes normocytes macroovalocytes few tear drop cells
Haemogram: (15/04/24)
HAEMOGLOBIN:4.5gm/dl
TOTAL COUNT:9700cells/cumm NEUTROPHIL:80% LYMPHOCYTES:15% EOSINOPHILS:00% MONOCYTES:05% BASOPHILS:00%
PCV: 15.6vol% MCV :135.7fl MCH :39.1pg MCHC :28.8%
RBC COUNT :1.15 millions/cumm PLATELET COUNT:2.86 lakhs/cu.mm SMEAR
RBC: Anisopoikilocytosis with tear drop cell, pencil forms,microcytes,normocytes,macro ovalocutes
COMPLETE URINE EXAMINATION (CUE) 10-04-2024 12:24:PM COLOUR
Pale yellow APPEARANCE
Clear REACTION
Acidic SP.GRAVITY 1.010 ALBUMIN
Trace SUGAR
Nil
BILE SALTS
Nil
BILE PIGMENTS
Nil
PUS CELLS 3-4
EPITHELIAL CELLS 2-3
RED BLOOD CELLS
Nil CRYSTALS
Nil CASTS
Nil
AMORPHOUS DEPOSITS
Absent OTHERS
Nil
PERIPHERAL SMEAR10-04-2024 12:24:PM
RBC: Normocytic normochromic with macrocytes seen WBC: With in normal limits
PLATELET: Adequate
Imp: Normocytosis Normochromic Anemia LIVER FUNCTION TEST (LFT) 10-04-2024
12:24:PM Total Bilurubin5.23 mg/dl 1-0 mg/dl
Direct Bilurubin0.61 mg/dl 0.2-0.0 mg/dlSGOT(AST)26 IU/L 31-0 IU/L
SGPT(ALT)10 IU/L 34-0 IU/L
ALKALINE PHOSPHATASE98 IU/L 369-54 IU/
LTOTAL PROTEINS5.5 gm/dl
8.3-6.4gm/dl ALBUMIN3.95 gm/dl 5.2-3.5 gm/dl
A/G RATIO2.55RFT 10-04-2024 12:24:PM UREA20 mg/dl
42-12 mg/dl CREATININE0.6 mg/dl
1.1-0.6 mg/dl
URIC ACID4.2 mmol/L
6-2.6 mmol/L CALCIUM8.8 mg/dl 10.2-8.6 mg/dl
PHOSPHOROUS3.8 mg/dl
4.5-2.5 mg/dl SODIUM138 mmol/L 145-136 mmol/L POTASSIUM3.2 mmol/L.
5.1-3.5 mmol/L.
CHLORIDE102 mmol/L
98-107 mmol/L
HBsAg-RAPID10-04-2024 12:24:PM
Negative
Anti HCV Antibodies - RAPID10-04-2024 12:28: PM Non Reactive SERUM ELECTROLYTES (Na, K, C l) 11-04-2024 11:36:PM SODIUM139 mmol/L 145-136 mmol/LPOTASSIUM4.0 mmol/L 5.1-3.5
mmol/L CHLORIDE104 mmol/L98-107 mmol/L
LIVER FUNCTION TEST (LFT) 12-04-2024 08:32:AM Total Bilurubin4.41mg/dl, 1-0 mg/dl
Direct Bilurubin 0.61 mg/dl 0.2-0.0 mg/dl SGOT(AST)31 IU/L
31-0 IU/L SGPT(ALT)10 IU/L 34-0 IU/L
ALKALINE PHOSPHATASE81 IU/L 369-54 IU/L
TOTAL PROTEINS5.6 gm/dl
8.3-6.4 gm/dl ALBUMIN3.7 gm/dl 5.2-3.5 gm/dl
A/G RATIO1.94
RBS 97 MG/DL
RETICULOCYTE COUNT 0.9 ABSOLUTE RETICULOCYT COUNT:1.2 COOMBS TEST :DIRECT POSITIVE (4+) INDIRECT NEGATIVE
AUTO CONTROL POSITIVE (3+) SERUM IRON :34
SERUM MAGNESIUM:2.1 LDH:492
URINARY ELECTROLYTES :
SODIUM 156 MMOL/L POTASSIUM 7.8MMOL/L CHLORIDE 116MMOL/L PERIPHERAL SMEAR :
RBC :NORMOCYTIC NORMOCHROMIC WITH MACROCYTES WBC:NORMAL
PLATELET :ADEQUATE SERUM ELECTROLYTES: SODIUM 136 MMOL/L
POTASSIUM 4 MMOL/L
CHLORIDE 104 CALCIUM IONISED:1.06 CHEST X RAY :
IMPRESSION B/L PROMINENT BRONCHOVESICULAR MARKINGS 2D ECHO:
IMPRESSION:EF 65
MILD TR+,NO PAH,NO MR/AR NO RWMA
GOOD LV SYSTOLIC FUNCTION NO DIASTOLIC DYSFUNCTION NO PE/CLOTS
ULTRASOUND: MILD SPLEENOMEGALY SPLEEN SIZE :14 CM
KIDNEY SIZE :RIGHT -7.8*3.8CM LEFT -8*3.1 CM
ECG: NORMAL SINUS RHYTHM
12/4/24
LFT
TOTAL BILIRUBIN 4.41
DIRECT BILURUBIN 0.61 AST31
ALT 10
ALKALINE PHOSPHATASE 81
TOTAL PROTEIN 5.6
ALBUMIN 3.7
A/G RATIO 1.94
COURSE IN HOSPITAL :PATIENT IS K/C/O AIHA ,CVID ON IVIG THERAPY SINCE 2021 CAME WITH C/O FEVER , YELLOWISH DISCLOURATION OF EYES , COUGH SINCE 5 DAYS ON FURTHER EVALUATION AND INVESTIGATION SHOWED HB_3.8GM/DL, TLC-7600CELL/CUMM, PCVG-13.2, RETIC COUNT -0.9% , LDH -492, S.IRON -34 MICROG/DL, DIRECT COOBS TEST - POSITIVE (4+) AND PS SHOWING NORMOCYTIC NORMOCHROMIC ANEMIA , TB -5.23 , DB - 0.61, SODIUM-138MEQ/L, POTASSIUM - 3.2, CHLORINE-102 FOR WHICH POTASSIUM CORRECTION WAS DONE . I/V/O HEMOLYTIC CRISIS PRECIPATED BY RESPIORATORY INFECTION PATIENT WAS STARTED ON TAB PREDNISOLONE30MG PO/OD OD DAY 1 OF ADMISSION. REPEAT INVESTIGATION WAS DONE ON DAY 5 SHOWED HB-4.5GM, TLC-9700 WITH MICROCYTES, MACROCYTES WITH FEW TEAR DROP CELSS AND WAS STARTED ON INJ VITCOFOL 1ML(500MCG) ONCE DAILY .PATIENT HAD 1 FEVER SPIKE 103F ON DAY 6 OF ADMISSION AND STARTED ON TAB AZITHROMYCIN AND TAB AMOXICLAV I/V/O CONSOLIDATION ON CHEST XRAY .PATIENT IMPROVED AND HEMODYANMICALLY STABLE
.HENCE BEING DISCHARGED WITH FOLLOWING ADVICE
Treatment Given(Enter only Generic Name) TAB.PREDNISOLONE 30 MG PO/OD FOR 6 DAYS INJ.LASIX 20 MG IV/OD FOR 2 DAYS
TAB DYTOR PLUS I/2TAB PO/OD 1-0-0GIVEN IF SBP>100MMHG TAB DYTOR I/2TAB PO/OD 0-0-1 GIVEN IF SBP>100MMHG
TAB AMOXICLAV 625MG PO/BD X 2 DAYS TAB AZITHROMYCIN 500MG PO/OD X 2 DAYS INJ VITCOFOL 1ML(500MCG) IM ONCE DAILY MONITOR VITALS INFORM SOS
STRICT I/O CHARTING
Advice at Discharge
TAB PREDNISOLONE 30 MG PO/OD FOR 30 DAYS (FOLLOWED BY PLAN TO TAPER BASED ON RESPONSE))
TAB DYTOR PLUS 10/50 MG 1/2(8AM) 1/2(4PM)
TAB AMOXICLAV 625MG PO/BD X 3 DAYS TAB AZITHROMYCIN 500MG PO/OD X 3 DAYS INJ VITCOFOL 1ML(500MCG) IM ONCE DAILY
TAB ONDENSETRON 2MG CHEWABLE PO/BD X 3DAYS
AMOROLFINE NAIL LAQUER LIA WEEKLY ONCE FOR 4 WEEKS, OVER RT HAND FINGER NAILS AND RT 2ND TOE
Follow Up
REVIEW TO GENERAL MEDICINE OP AFTER 7 DAYS
When to Obtain Urgent Care
IN CASE OF ANY EMERGENCY IMMEDIATELY CONTACT YOUR CONSULTANT DOCTOR OR ATTEND EMERGENCY DEPARTMENT.
Preventive Care
AVOID SELF MEDICATION WITHOUT DOCTORS ADVICE,DONOT MISS MEDICATIONS.
In case of Emergency or to speak to your treating FACULTY or For Appointments, Please Contact: For Treatment Enquiries Patient/Attendent Declaration : - The medicines prescribed and the advice regarding preventive aspects of care ,when and how to obtain urgent care have been explained to me in my own language
SIGNATURE OF PATIENT /ATTENDER SIGNATURE OF PG/INTERNEE SIGNATURE OF ADMINISTRATOR SIGNATURE OF FACULTY
Discharge Date Date:17/4/24 Ward: GM
Unit:3
Age/Gender: 18 Years/Female
Address:
Discharge Type: Relieved
Admission Date: 22/10/2024 02:53 PM
Name of Treating Faculty
Diagnosis
AIHA: HEMOLYTIC ANEMIA ( PRECIPITATED BY RESPIRATORY TRACT INFECTION) HIGH OUTPUT CARDIAC FAILURE SECONDARY TO SEVERE ANEMIA
CVID ON IVIG THERAPY B/L OPTIC ATROPHY
Case History and Clinical Findings
COMPLAINTS OF COLD, COUGH, FEVER, SOB SINCE 1WEEK HOPI:-
PATIENT WAS APPARENTLY ASYMPTOMATIC 1WEEK BACK THEN SHE DEVELOPED COLD, COUGH WITH OUT SPUTUM AND DEVELOPED FEVER WHICH IS INTERMITTENT, MODERATE, NOT ASSOCIATED WITH CHILLS
H/O SOB GRADE-I(MMRC) NOT ASSOCIATED WITH ORTHOPNEA, PND NO H/O BLEEDING MICTURITION
NO H/O ABDOMINAL PAIN, BOWEL AND BLADDER ABNORMALITIES NO H/O BLEEDING MANIFESTATION
PAST HISTORY:-
N/K/C/O DM, HTN, ASTHMA, TB, EPILEPSY, CVA, CAD
GENERAL EXAMINATION: PALLOR PRESENT
ICTERUS PRESENT
NO EDEMA CYANOSIS CLUBBING LYMPHADENOPATHY BP: 100/60 MMHG
PR:110 BPM RR: 14 CPM
TEMPERATURE: 98 F
SPO2: 96 %
CVS: NO THRILLS, S1S2 +, NO MURMURS RS: BAE+,NVBS, DYSPNOEA PRESENT PER ABDOMEN:
SOFT , NON TENDER
NO ORGANOMEGALY FELT LIVER BORDERS PALPABLE CNS :NFND, E4V5M6
COURSE IN HOSPITAL:-
18 YEAR OLD FEMALE WAS BRUGHT TO OPD WITH C/O COLD, COUGH, SOB. ON VITALS AT ADMISSION BP-100/60MMHG, PR-110BPM, RR-14CPM, . ON TREATED WITH CORTICOSTEROIDS, ANTIHISTAMINES, COUGH SYRUP DIAGNOSED WITH AUTOIMMUNE HEMOLYTIC ANEMIA K/C/O HIGH OUTPUT CARDIAC FAILURE SECONDARY TO ANEMIA B/L OPTIC ATROPHY. PATIENT WAS IMPROVED CLINICALLY AND DISCHARGED IN HAEMODYNAMICALLY STABLE CONDITION.
Investigation
HAEMOGRAM ON 23/10/24:
HAEMOGLOBIN 2.8 gm/dl TOTAL COUNT 6,000 cells/cumm NEUTROPHILS 72 %LYMPHOCYTES 22 % EOSINOPHILS 02 % MONOCYTES 04 % BASOPHILS 00 % PCV 8.5 vol % M C V 112.7 fl M C H 36.7 pg M C H C 32.5 % RDW-CV 37.8 %RDW-SD 171.1 fl RBC COUNT 0.76
millions/cumm PLATELET COUNT 3.38 lakhs/cu.mm SMEARRBC Anisopoikilocytosis with microcytes, normocytes, macrocytes, macroovalocytes and few tear drops, target cells WBC normal counts PLATELETS Adequate in number and distribution HEMOPARASITES No hemoparasites seen IMPRESSION Dimorphic anemia ADVICED: Sickling test, OFT ,electrophrosis to rule out thehemolytic anemia
LIVER FUNCTION TEST (LFT) 23-10-2024: Total Bilurubin 5.20 mg/dl Direct Bilurubin 1.54 mg/dl SGOT(AST) 29 IU/L SGPT(ALT) 23 IU/L ALKALINE PHOSPHATASE 85 IU/LTOTAL PROTEINS 6.0gm/dl ALBUMIN 3.6 gm/dl A/G RATIO 1.50
LIVER FUNCTION TEST (LFT) ON 24/10/24Total Bilurubin 7.46 mg/dl Direct Bilurubin 1.56 mg/dl SGOT(AST) 28 IU/L SGPT(ALT) 17 IU/L ALKALINE PHOSPHATASE 75 IU/L TOTAL PROTEINS 5.2gm/dl ALBUMIN 3.74 gm/dl 5.2-3.5 gm/dlA/G RATIO 2.56 HAMOGRAM ON 24/10/24:
HAEMOGLOBIN 2.8 gm/dlTOTAL COUNT 10,800 cells/cumm NEUTROPHILS 62 %
LYMPHOCYTES 31 % EOSINOPHILS 01 % MONOCYTES 06 % BASOPHILS 00 % PCV 9.2 vol
%M C V 30.0 fl M C H 39.8 pg M C H C 30.6 % RDW-CV 38.1 % RDW-SD 200.3 fl RBC COUNT
0.70 millions/cumm ePLATELET COUNT 4.05 lakhs/cu.mm SMEARRBC Anisopoikilocytosis with microcytes ,normocytes , macrocytes , macroovalocytes few tear drop cells ,target cellsWBC normal counts on smear PLATELETS Adequate in number and distribution HEMOPARASITES No hemoparasites seen IMPRESSION Dimorphic anemiaADVICED: sickling test . OFT ,Electrophorosis to rule out hemolyticanemia
STOOL FOR OCCULT BLOOD 24-10-2024- positive (+ve)
RFT 24-10-2024:UREA 18 mg/dl CREATININE 0.7 mg/dl URIC ACID 6.6 mmol/L CALCIUM 8.5
mg/dl PHOSPHOROUS 3.0 mg/dlSODIUM 139 mmol/L POTASSIUM 3.5 mmol/LCHLORIDE 102
mmol/L
HEMOGRAM (25/10/24):
HAEMOGLOBIN 3.0 gm/dlTOTAL COUNT 14,000 cells/cumm NEUTROPHILS 52
%LYMPHOCYTES 42 % EOSINOPHILS 00 % MONOCYTES 06 % BASOPHILS 00 % PCV 9.9 vol
% M C V 141.1 fl M C H 42.9 pg M C H C 30.4 % RDW-CV 31.9 % RDW-SD 179.6 fl RBC COUNT
0.70 millions/cumm PLATELET COUNT 4.2 lakhs/cu.mm SMEARRBC Anisopoikilocytosis with microcytes ,normocytes , macrocytes , macroovalocytes few tear drop cells andtarget cellsWBC counts increased on smear PLATELETS Adequate in number and distributionHEMOPARASITES No hemoparasites seen IMPRESSION Dimorphic anemia with leucocytosisADVICED: Sickling test , OFT
, andElectrophorosis to rule out hemolyticanemia
LIVER FUNCTION TEST (LFT) 25-10-2024: Total Bilurubin 5.73 mg/dl Direct Bilurubin 1.18 mg/dl SGOT(AST) 25 IU/LSGPT(ALT) 11 IU/LALKALINE PHOSPHATASE 80 IU/L TOTAL PROTEINS 5.0
gm/dlALBUMIN 3.69 gm/dl A/G RATIO 2.82
28/10/24HEMOGRAM-Hb- 4.3, TLC-9,300,PLATELET COUNT- 3.19, PCV- 14.9, MCV-138.7 LFT- TB-4.43, DB-0.49,SGOT-33,SGPT-22,ALP-74
Treatment Given(Enter only Generic Name)
1. TAB.PREDNISOLONE 30MG PO/OD(I/V/O AIHA)
2. TAB.LEVOCET 5MG PO/OD(I/V/O PRURITIS)
3. TAB.ZOFER 4MG PO/OD(I/V/O NAUSEA)
4. SYRUP.ASCORYL D PLUS 10ML PO/TID ( I/V/O DRY COUGH )
5. CALOSOFT LOTION L/A TID
Advice at Discharge
1. TAB.PREDNISOLONE 30MG PO/OD(I/V/O AIHA) X 10 DAYS
2. TAB.LEVOCET 5MG PO/OD(I/V/O PRURITIS) X 3 DAYS
3. TAB.ZOFER 4MG PO/OD(I/V/O NAUSEA)
4. SYRUP.ASCORYL D PLUS 10ML PO/TID X 5 DAYS
5. CALOSOFT LOTION L/A TID
Follow Up
REVIEW TO GENERAL MEDICINE OPD AFTER ONE WEEK WITH HEMOGRAM AND LFT REPORT
When to Obtain Urgent Care
IN CASE OF ANY EMERGENCY IMMEDIATELY CONTACT YOUR CONSULTANT DOCTOR OR ATTEND EMERGENCY DEPARTMENT.
Preventive Care
AVOID SELF MEDICATION WITHOUT DOCTORS ADVICE, DONOT MISS MEDICATIONS. In case of Emergency or to speak to your treating FACULTY or For Appointments, Please Contact: For Treatment Enquiries Patient/Attendent Declaration: - The medicines prescribed and the advice regarding preventive aspects of care, when and how to obtain urgent care have been explained to me in my own language
SIGNATURE OF PATIENT /ATTENDER SIGNATURE OF PG/INTERNEE SIGNATURE OF ADMINISTRATOR SIGNATURE OF FACULTY
Discharge Date
Date: 25.10.2024
Ward:AMC Unit:I
23-11-2024
31-12-2024
PPM 1 - 👆any update on this report @PPM 3?
Was she started on ATT based on this.
Also I just noticed the name in the report that was pmmed to me by the patient advocate and I'm not sure now if it's of this patient.
Her current medicine update is:
PA - Please check the report sir. 5mg tab continued sir. One month completed.
PPM 1 - This is okay. Urine for 24 hour protein and creatinine test report is required.
PA - Is the test available in our hospital? Should we come to the hospital sir?
Are you available there?
PPM 1 - Yes this test is done in our hospital.
You will be provided a can for collection of urine test for 24 hr protein and creatinine.
Did the patient take any other tablet? Did you start TB medication?
PA - We are coming to the hospital sir.
PPM 1 - Okay you did not post in the group, we were confused.
We have prescribed the 24hr protein creatinine test for someone else.
PPM 3 - Yes sir
She is on ATT since 1 month.
PPM 1 - Alright wasn't aware of that and I guess the group may not have been aware too @PA2
👆2023 also on ATT? How long? @PPM3
PPM 3 - This is the NTEP identity card sir.
PPM 1 - Thanks for sharing
PPM 3 - She was not on any ATT prior to this sir.
Currently her complaints are she is having abdominal discomfort and tightness with icterus.
31-12-202
PPM 1 - This appears to be more of a conjugated hyperbilirubinemia and no hemolysis either in her hemogram!
👆How do we explain this write up in her 2023 discharge summary?
PPM 3 - Yes sir appears to be due to ATT
PPM 1 - 👍
What happened to the LPA report that they had registered to be sent as her CBNAAT was weakly positive?
👆 imaging was more suggestive of ABPA than Tuberculosis?
PPM 3 - Not sure about LPA report sir
As per the technician at the hospital the report will be given to attenders if it is significant
will try to trace her LPA report.
They will be coming tomorrow for admission sir.
PPM 1 - Ask them to hold the ATT for the time being.
PPM 3 - Okay sir.
01-01-2025
PPM 1 - Has anyone tried to verify this hypothesis that ATT induced hepatitis often happens to those where the diagnosis itself may not be tuberculosis? @PPM2.
PPM 2 - Vaguely remember a Ghanaian study and a score they calculated. Will try to find but unlikely.
PPM 1 - 👍
02-01-2025
PPM 1 - Reviewing her right now in OPD:
18F with ATT induced hepatitis DILI in a long term follow up patient of intermittent hemolysis on iv IG monthly since March 2021 and recent weakly positive CBNAAT after one week of increased cough.
Plan is to stop antitubercular therapy and repeat LFT after 10 days
On 30th before she collected her report of LFT after giving her blood sample she also received her monthly 10g of iv IG.
PPM 1 - 👆Her spleen is below the umbilicus although sliding down the left iliac foss without crossing over and barely touching our intern's finger tip exploring from the right.
PPM 1 - This is her chest X-ray from June.
PPM 4 -
PPM 1 - DILI should resolve once the drug is stopped.
PPM 4 - Any other drugs to be prescribed sir?
PPM 1 - None.
PPM 4 - Okay sir.
PPM 5 - Prominent splenic and portal vein - Portal HTN..?
Does she have it earlier..?
PPM 1 - She has massive splenomegaly and is very similar to the other boy with hypersplenism in this regard.
PPM 5 - 👍
16-01-2025
PPM 1 -
PPM 1 - OPD review of 18F with chronic intermittent hemolysis since childhood case reported earlier by different team members here.
Reviewed with fresh Chest X-ray which shows resolution of previous lung shadows and LFT also shows reduction of enzymes.
PPM 1 - Att was started on November 23 , 2024 and stopped on January 3, 2025
17-01-2025
PPM 1 - Reviewed again today in OPD just now and spoke with the rntep station in our college:
Yesterday's Sputum AFB single sample negative
Sending sputum CBNAAT now
In the general workflow of the rntep it's not done once the patient has taken ATT for a month or two but they appear to be accommodating her unique clinical complexity
NMC dynamic E log of this patient being regularly updated.
20-01-2025
PPM 1 - CBNAAT is negative this time as informed by RNTEP staff.
CBNAAT-Negative for MTB
Lab no-170
01-02-2025
PPM 1 - Thanks for sharing creatinine which is 0.33
And Bilirubin T is 7.3 and bilirubin D is 1.6
22-02-2025
PPM 1 - OPD bench to bedside right now: 18F came back after viral fever with a fear of TB as last time it was diagnosis but then she had to stop her antitubercular therapy abruptly after a month as she developed att induced hepatitis and then it wasn't restarted as the TB diagnosis itself was suspect! Her previous details are case reported here: https://pmc.ncbi.nlm.nih.gov/articles/PMC9685200/
[29-03-2025 11.29] PPM 1: Please share the patient's deidentified current blood reports here and let us know if she has any symptoms or was it done as a routine.
[16-06-2025 10:30] PPM 3: Patient had H/O fever recently and subsided now
Complaints of generalised weakness, fatigue and breathlessness since 2-3 days
In OPD now
[16-06-2025 10:32] PPM 1: Reaching OPD.
[16-06-2025 10:47] PPM 1: Again aadhar card 18 but going to be actually 22!
@PPM5 could feel her liver and spleen very well
[16-06-2025 10:52] PPM 1: Bilirubin 4.8 on 27/5/25, Hb 9.4, Rest were normal
[16-06-2025 11:00] PPM 1: Cough is intermittent only with teepi (sweet)and pulupu (sour) suggestive of reflux and she had been started on ATT outside from RNTEP on November 2024 for low doubtful AFB that had to be stopped after a few weeks when her bilirubin worsened.
[16-06-2025 11:14] PPM 1: @PPM4 any LLM leads to connect her childhood chronic autoimmune hemolytic anemia and her optic atrophy?
She's also been published in BMJ (again linked in the description box) but we didn't focus on this association at that time
[16-06-2025 15:54] PPM 4: Based on the WhatsApp discussion screenshot, here's a structured analysis and response addressing the query about connecting childhood chronic autoimmune hemolytic anemia and optic atrophy using LLMs:
### Key Clinical Elements from the Discussion:
1. *Patient History*:
- Periodic fever syndrome (likely PFAPA)
- Chronic autoimmune hemolytic anemia (since childhood)
- Optic atrophy + nystagmus
- Documented VEP (Visual Evoked Potential) abnormalities (delayed latency, poor reproducibility)
- Published case in BMJ previously
2. *Explicit Query*:
> "Any LLM leads to connect her childhood chronic autoimmune hemolytic anemia and her optic atrophy?"
—---
### How LLMs Can Assist in This Case:
1. *Literature Synthesis*:
- *Prompt Example for LLMs*:
"Identify published associations between chronic autoimmune hemolytic anemia (especially pediatric-onset) and optic atrophy/nystagmus. Include genetic syndromes, autoimmune disorders, or metabolic diseases linking these features."
- *Tools*: ChatGPT-4, Gemini, PubMed-optimized LLMs (e.g., BioBERT, SciBERT)
2. *Differential Diagnosis Generation*:
- *Possible LLM Output*:
- *Genetic Disorders*:
• Wolfram syndrome (DIDMOAD: Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, Deafness) - hemolytic anemia is rare but reported.
• Friedreich’s ataxia (optic atrophy + cardiomyopathy-associated hemolysis).
- *Autoimmune/Inflammatory*:
• SLE or APS (antiphospholipid syndrome) with CNS involvement.
• Neuromyelitis optica spectrum disorder (NMOSD) with hemolytic complications.
- *Metabolic*:
• Wilson’s disease (copper accumulation causing hemolysis + optic neuropathy).
3. *Mechanistic Hypotheses*:
LLMs can cross-reference pathways like:
- Chronic inflammation → oxidative stress → neuronal/optic nerve damage.
- Shared autoantibodies targeting red blood cells and retinal ganglion cells.
4. *BMJ Case Review Enhancement*:
Prompt:
"Re-analyze the published BMJ case [insert DOI/title] focusing on underexplored links between hemolytic anemia and optic atrophy using recent literature (2020-2025). Highlight diagnostic blind spots."
---
### Actionable Recommendations:
1. *Immediate LLM Utilization*:
- Use *Perplexity.ai* (with "academic" mode) to find case reports/pathophysiological links.
- Try *Gemini Advanced* for generating a diagnostic flowchart integrating VEP abnormalities.
2. *Clinical Next Steps*:
- Rule out *Wolfram syndrome* (check for diabetes/urine concentrating defects).
- Test for *aquaporin-4 antibodies* (NMOSD) and *anti-ganglioside antibodies*.
- Re-evaluate VEP with MRI orbits/brain to exclude demyelination.
3. *Collaborative Tools*:
- Share de-identified data via *CIELO* (Collaborative Interpretable Expert-Led Ontology) for AI-augmented diagnosis.
---
### Limitations & Caveats:
- LLMs may generate false associations; always validate outputs via PubMed/clinical expertise.
- Optic atrophy + hemolysis is highly atypical – consider novel mutations or undocumented syndromes.
- Prioritize genetic testing (whole-exome sequencing) given the chronic pediatric multisystem involvement.
Would you like me to generate specific LLM prompts or draft a referral note for further workup?
[16-06-2025 16:36] PPM 1: Let's try this query on perplexity
[16-06-2025 16:40] PPM 1: @PPM5 did your Ophthalmology PG friend evaluate her today for her optic atrophy and nystagmus?
[16-06-2025 16:41] PPM 1: Here's a similar patient where retro bulbar neuritis was reported 👇
https://journals.lww.com/ijog/fulltext/2023/03020/a_rare_case_of_evans_syndrome_presenting_as.67.aspx
[16-06-2025 16:44] PPM 4: Based on the provided case report, here are the key clinical takeaways regarding Evans Syndrome (ES) presenting as retrobulbar neuritis:
### 1. *Unprecedented Presentation*
This is the *first documented case* of ES manifesting as retrobulbar neuritis. ES typically involves autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia, but ocular involvement (especially optic neuritis) is exceptionally rare.
### 2. *Patient Profile & Clinical Features*
- *25-year-old female* with sudden painful vision loss in the right eye.
- *Past history*:
- Bilateral vision loss 5 months prior (misdiagnosed as nutritional optic neuropathy).
- Chronic menorrhagia (6 years) and iron-deficiency anemia requiring transfusions.
- *Systemic signs*: Pallor, clubbing, hepatosplenomegaly.
- *Ocular signs*:
- Severe vision loss (light perception OD, 20/200 OS).
- Relative afferent pupillary defect (OD), bilateral optic disc pallor.
- Prolonged P100 latency on VEP (OD).
### 3. *Diagnostic Workup*
- *MRI*: Bilateral optic atrophy + inflammatory changes in the right optic nerve (confirming retrobulbar neuritis).
- *Hematology*: Hemolytic anemia, positive direct Coombs test, splenomegaly.
- *Excluded other causes*: Negative for NMO antibodies, syphilis, TB.
- *Final diagnosis: **Primary Evans Syndrome* with retrobulbar neuritis.
### 4. *Treatment & Outcome*
- *Acute management*: IV methylprednisolone (1g × 3 days) → oral prednisolone taper.
- *Long-term*: Immunomodulatory agents for ES under physician supervision.
- Steroids halted further vision deterioration.
### 5. *Key Clinical Implications*
- *Ophthalmologists as first responders*: Patients with unexplained optic neuritis + systemic hematologic symptoms (e.g., anemia, bleeding) warrant evaluation for ES.
- *ES morbidity*: High relapse risk, treatment resistance, and potential mortality necessitate early multidisciplinary care.
- *Ocular associations*: While rare, ES can cause vision-threatening complications (retrobulbar neuritis, retinal hemorrhages, vascular occlusions).
### 6. *Recommendations from the Authors*
- Screen ES patients for optic nerve dysfunction.
- Report similar cases to refine understanding of ES-related ocular pathology.
> *In summary*: This case highlights ES as a differential in atypical optic neuritis, especially with concurrent hematologic abnormalities. Prompt steroids may preserve vision, but long-term immunomodulation is essential for systemic management.
For full details, refer to:
Shah VM, Hema VK, Kumar K. A rare case of Evans syndrome presenting as retrobulbar neuritis. Indian J Ophthalmol Case Rep. 2023;3(2):463-464. doi:10.4103/ijo.IJO_841_22.
[16-06-2025 16:44] PPM 1: Another similar patient although they put too much money on the association between her therapy and the atrophy. It's more likely due to ischemic optic neuropathy due to hemolysis and inflammation induced narrowing of the vessels to optic nerve👇
[16-06-2025 16:47] PPM 1: Possible lead to her optic neuritis and atrophy in association with her chronic hemolytic anemia 👇
[16-06-2025 16:48] PPM 4: Based on the case report of optic neuropathy in a hereditary xerocytosis patient with iron overload, your interpretation that ischemic optic neuropathy (rather than chelation toxicity) is the primary mechanism is well-supported. Here's a breakdown of the key evidence and pathophysiological rationale:
### 1. *Clinical and Diagnostic Findings Supporting Ischemic Mechanism*
- *Presentation*: Acute, painless, sectoral vision loss with inferior altitudinal defect—classic for non-arteritic anterior ischemic optic neuropathy (NAION) .
- *Ophthalmologic Signs*:
- Relative afferent pupillary defect (RAPD), optic disc swelling with pallor, and small cup-to-disc ratio (0.1)—known anatomical risk factors for NAION due to crowded discs impairing perfusion .
- MRI-confirmed bilateral optic nerve atrophy and diffuse brain hemosiderin deposition, indicating chronic iron-mediated vascular injury .
- *Exclusion of Alternatives*: Negative infectious/autoimmune serologies (e.g., syphilis, sarcoidosis), no compressive lesions, and absence of chelation-related retinopathy (e.g., bull's-eye maculopathy) .
### 2. *Iron Overload as the Primary Driver of Ischemia*
- *Hemolysis-Induced Vascular Damage*: Chronic hemolysis in hereditary xerocytosis promotes:
- *Oxidative stress*: Free iron catalyzes reactive oxygen species (ROS), directly damaging vascular endothelium and impairing optic nerve perfusion .
- *Inflammation*: Hepcidin upregulation from chronic hemolysis increases iron retention in tissues, perpetuating inflammation and vascular narrowing .
- *Hemosiderin Deposition*: MRI evidence of iron accumulation in the choroid plexus and optic nerves confirms direct tissue toxicity, disrupting microcirculation and causing ischemic injury .
### 3. *Chelation Therapy Unlikely as the Culprit*
- *Deferiprone Safety Profile: Unlike deferoxamine (linked to toxic retinopathy), deferiprone has no documented ocular toxicity in literature . The patient’s optic neuropathy occurred *despite switching to deferiprone.
- *Timing and Pathobiology*: Chelation toxicity typically causes gradual retinopathy (e.g., pigmentary changes), not acute NAION. The altitudinal defect aligns with vascular occlusion, not toxic/metabolic injury .
### 4. *Contributing Risk Factors*
- *Immunosuppressants*: Tacrolimus (used post-cardiac transplant) may exacerbate ischemia via endothelial dysfunction, though not primary .
- *Anatomical Susceptibility*: Crowded optic discs (cup-to-disc ratio 0.06) predispose to perfusion compromise during hemodynamic stress .
### Key Differentiators: Ischemic vs. Toxic Optic Neuropathy
| *Feature* | *Ischemic (NAION)* | *Chelation Toxicity* |
|---------------------------|--------------------------------|--------------------------------|
| *Onset* | Acute, painless | Gradual |
| *Visual Field Defect* | Altitudinal | Central/scotomatous |
| *Disc Appearance* | Swelling + pallor, small cup | Retinal pigmentary changes |
| *MRI Findings* | Hemosiderin deposition, atrophy| Often normal |
| *Mechanism* | Vascular occlusion | Photoreceptor/RPE damage |
### Conclusion
This case highlights *iron overload-induced ischemia* as the dominant mechanism:
- Chronic hemolysis and transfusions → iron-mediated oxidative stress + inflammation → vascular endothelial damage → impaired optic nerve perfusion → NAION.
- Deferiprone is an unlikely contributor, emphasizing that optic atrophy here is a consequence of *hemolytic/vascular pathology*, not chelation.
- *Clinical Implication*: In transfusion-dependent anemias, monitor for vascular optic neuropathy via OCT and visual fields, beyond ferritin levels .
> 💡 *Proactive Management Tip*: Address modifiable ischemia risk factors (e.g., nocturnal hypotension, anemia severity) and consider neuroprotective agents (e.g., antioxidants) alongside chelation .
[16-06-2025 16:50] PPM 1: Another association between chronic hemolytic anemia and optic neuritis atrophy👇
[16-06-2025 16:54] PPM 4: Based on the case reports and research findings, the association between chronic hemolytic anemia and optic neuropathy (including ischemic optic neuropathy and optic atrophy) involves multiple interrelated pathophysiological mechanisms. Here is a synthesis of key insights:
---
### ⚕ *1. Ischemic Mechanisms in Hemolytic Anemia*
- *Reduced Oxygen Delivery: Severe anemia (Hb <6 g/dL) compromises perfusion of the optic nerve head, particularly in anatomically vulnerable "crowded discs" with small cup-to-disc ratios. This hypoxia triggers **non-arteritic anterior ischemic optic neuropathy (NAION)*, characterized by acute, painless vision loss with altitudinal defects .
- *Thrombotic Microangiopathy: Hemolysis releases prothrombotic substances (e.g., free hemoglobin, ADP), promoting platelet aggregation and microvascular occlusion. Cases report NAION in **hereditary spherocytosis* and *sickle cell anemia* due to vascular sludging .
- *Hypotension-Induced Ischemia: Nocturnal hypotension or rapid blood pressure drops (e.g., during surgery or antihypertensive therapy) exacerbate ischemia. One case of **posterior ischemic optic neuropathy (PION)* occurred after aggressive BP management in hypertensive emergency .
---
### 🔥 *2. Inflammatory/Autoimmune Mechanisms*
- *Immune Dysregulation: Autoimmune hemolytic anemias (e.g., **Evans syndrome) generate antibodies targeting erythrocytes, platelets, and granulocytes. Cross-reactivity with optic nerve antigens can cause **retrobulbar neuritis*, presenting with painful vision loss, prolonged P100 latency on VEP, and inflammatory changes on MRI .
- *Complement Activation*: In cold agglutinin disease, IgM antibodies fix complement on RBCs, leading to membrane attack complex deposition on vascular endothelium. This causes inflammation and occlusion of optic nerve microvasculature.
---
### ⚖ *3. Iron Overload and Toxicity*
- *Oxidative Stress: Chronic transfusions in hemolytic anemias (e.g., **hereditary xerocytosis*) cause iron overload. Free iron catalyzes reactive oxygen species (ROS), damaging retinal pigment epithelium and optic nerve axons via lipid peroxidation.
- *Chelation Neurotoxicity: **Deferoxamine* (an iron chelator) is linked to toxic retinopathy and optic neuropathy. However, ischemia from iron-mediated vascular injury often predominates over direct drug toxicity.
---
### 🦠 *4. Infectious Triggers*
- *Parvovirus B19: In hereditary spherocytosis, acute infection can precipitate **aplastic crisis* with severe anemia. This may trigger NAION due to compounded hypoxia and endothelial damage .
- *Other Pathogens*: Viruses (e.g., West Nile, HIV), bacteria (e.g., syphilis), and parasites (e.g., malaria) cause optic neuropathy via direct invasion, immune complex deposition, or vasculitis .
---
### 👁 *5. Angioid Streaks as a Comorbid Risk*
- *Elastin Degradation: Chronic hemolysis (e.g., in **hereditary spherocytosis) increases matrix metalloproteinases, disrupting Bruch's membrane. **Angioid streaks* weaken choroidal vasculature, reducing optic nerve perfusion and increasing susceptibility to AION.
---
### 💊 *Clinical Management Implications*
| *Condition* | *Treatment Approach* | *Key Considerations* |
|------------------------|----------------------------------------------------------------------------------------|---------------------------------------------------------------|
| *Ischemic Optic Neuropathy* | Urgent anemia correction (transfusion), BP stabilization, avoid nocturnal hypotension | Avoid rapid Hb correction in sickle cell anemia (viscosity risk) |
| *Autoimmune Optic Neuritis* | IV steroids (methylprednisolone 1g/day), rituximab for refractory cases | Monitor for steroid-induced osteoporosis or hyperglycemia |
| *Iron Overload* | Chelation (deferiprone > deferoxamine), avoid rapid BP reduction | Regular ophthalmic screening with OCT/VEP |
---
### 📊 *Prognosis & Monitoring*
- *Ischemic Neuropathy*: Poor visual recovery in NAION (70% retain deficits) .
- *Inflammatory Neuritis*: Better response to steroids; atrophy may develop if delayed treatment .
- *Critical Screening*: Hemolytic anemia patients require biannual ophthalmic exams (acuity, fields, fundoscopy), Hb monitoring, and MRI for optic nerve inflammation/atrophy.
For further details, refer to the primary sources: [Evans syndrome with retrobulbar neuritis](https://journals.lww.com/ijog/fulltext/2023/03020/a_rare_case_of_evans_syndrome_presenting_as.67.aspx) and [AION in spherocytosis] (https://journals.sagepub.com/doi/abs/10.5301/ejo.5000205?download=true).
[16-06-2025 16:57] PPM 5: No sir
[16-06-2025 16:58] PPM 1: Didn't the patient return to the OPD?
[16-06-2025 16:58] PPM 5: Waited till now sir. They didn’t come
[16-06-2025 17:00] PPM 1: The patient advocate is in the group here. Please call him and check
[16-06-2025 17:04] PPM 5: Yes sir
Ophthal examination was done sir
But the PG'S who did the examination didn’t take any optic nerve pictures sir.
[16-06-2025 17.27] PPM 5: They have written. Pt is not willing for fundus examination sir
Today's Hemogram
Today's LFT
[16-06-2025 17.36] PPM 5: Should we admit this patient, sir??[16-06-2025 17.39] PPM 1: Yes if they are willing
[16-06-2025 17.39] PPM 1: Currently having another hemolytic crisis.
[16-06-2025 17:43] PPM 6: At her first admission she didn’t had optic atrophy or nystagmus sir. After her IVIG therapy for 9 months (roughly) she presented with nystagmus. Then on evaluation found to have increased blurring and then optic atrophy sir.
[16-06-2025 17:43] PPM 5: They didn’t not bring anything sir
They said they will stay if the patient condition is serious and in need of compulsory admission.
[16-06-2025 21:46] PPM 8: Patient got admitted Shifted to AMC
Current vitals:-
Bp-130/90
Pr-123
Rr-36
Spo2-96%
[16-06-2025 21:49] PPM 1: Thanks
[16-06-2025 21:50] PPM 1: Let's increase her current prednisolone from 5mg to 30 mg like last time when she was admitted for the same crisis.
[17-06-2025 15.09] PPM 1: @PPM7 @PPM8 what are the pointers to cardiac failure in this patient? What happened to the respiratory rate from yesterday evening to today morning?
[18-06-2025 14.35] PPM 1: UpdateS of SOAP
After pharmacological intervention for her presumed immune hemolysis with 6 times increase in her prednisolone dose
Nausea 100% reduced since yesterday
Fatigue no reduction
Appetite 60% improved
[18-06-2025 15:05] PPM 9: sir? Bone marrow aspiration report
[18-06-2025 15:08] PPM 9: her? Blood type
[18-06-2025 16:31] PPM 1: It's in the case report link available in the description box
[18-06-2025 16:32] PPM 9: sir did Blood typing done?
[18-06-2025 16:33] PPM 9: There are so many types of blood.
could be a different variant
like Bombay Blood group
just a hunch..
[18-06-2025 16:38] PPM 1: I'm sure if she's received so many blood transfusions since her childhood her typing was definitely done
[18-06-2025 16:38] PPM 9: Did we do it in our hsptl sir
[18-06-2025 16:39] PPM 9: And no blood group seems to match her
as said in report
[18-06-2025 16:43] PPM 1: That happens when a patient has autoimmune hemolytic anemia
[18-06-2025 16:44] PPM 1: Many times I'm sure
[18-06-2025 16:48] PPM 9: Oh okay sir But why didn't they add it in the report
[18-06-2025 16:49] PPM 1: Because we want everyone to learn to see and understand MRI images
[18-06-2025 16:49] PPM 9: Ohh okay sir
[18-06-2025 16:49] PPM 1: Oh that was for the other group patient!
[18-06-2025 16:52] PPM 9: 😅
[18-06-2025 16:53] PPM 9: I didn't see an MRI here that confused me.
[18-06-2025 19.59] PPM 9: I don't see a bone marrow aspiration study either
[19-06-2025 16.10] PPM 5: Today’s Hemogram report.
[19-06-2025 18:56] PPM 1: Let's hope the hemolysis has stopped
The Hb will take some time to rise
How's she feeling subjectively in terms of her fatigue and appetite @PPM7?
[19-06-2025 19:03] PPM 5: Yes sir she is feeling subjectively much better sir
[19-06-2025 19:05] PPM 1: Which parameters are better.
Yesterday @PPM7 identified three parameters which she noted in the file as shared above in an image
[19-06-2025 19:06] PPM 5: Nausea, appetite sir
[19-06-2025 19:13] PPM 1: Yesterday appetite was reduced by 60%
Fatigue was 100%
Today?
[19-06-2025 22:02] PPM 7: Today appetite was improved by 60% and fatigue reduced by 20 %. Sir
PPM 1: 👍
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