A 13 YEAR OLD BOY WITH YELLOWISH DISCOLORATION

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This is a case of a 13 year old boy who came to OPD with the chief complaints of- 

Yellowish discoloration of the eyes since 9 days.

Yellow coloured urine since 9 days.


Patient history


Youngest child

Normal vaginal delivery

Birth weight: 4.5 kg


At birth:


Patient had jaundice and it resolved spontaneously (physiological jaundice).

Immunised as per schedule.


At 8 months:


He had altered bowel habits - loose stools for a week and another week he had normal consistency stools, later he had loose stools every day for 2-3 months during which he became cachectic.

He got admitted in hospital and they mentioned as suspected case of celiac disease,chronic diarrhoea,chronic malabsorption.

He was treated with IV antibiotics and 2FFP transfusions. 

He recovered within 3 months.


He also had recurrent attacks of cold,cough and fever.He used inhalers for 1 year.

No h/o pneumonia.


At 9 years:

Parents noticed neck swelling and took him to ent doctor, who then further referred him to an endocrinologist and then diagnosed with hypothyroidism .(Initial TSH levels-150)

He was started with THYRONORM 150mcg later increased to 180mcg


He also had chronic itching over hands and foot since the age of 9 years


At 12 years


He had chickenpox and resolved within 10 days


At 13years (Now)


He came with complaints of yellowish discoloration of eyes ,dark yellow colored urine since 9 days . Also had one episode of greenish yellow coloured vomiting which was non projectile, non foul smelling and contained bile, food particles.


No history of fever,chills,abdominal pain,loose stools, cold ,cough, joint pains.

He was treated by a paediatrician for jaundice but as bilirubin is increasing they referred here for further management.




Post inflammatory hypopigmentary patches-








Daily routine of patient

Patient stays in the hostel, gets up at 5:30 am, takes tab. THYRONORM empty stomach before brushing his teeth, takes his breakfast, goes to school at 9am takes his lunch at 12 noon, dinner at 7:30 pm, sleeps at 9 pm, uses lotion for allergy both in the morning and before going to bed.

 


FAMILY HISTORY


He  is a 4th order child ,born out of grade 4 consanguinous marriage


He has 2 elder brothers and 

one elder sister - who expired at 5 years of age


His sister was the first child and she was asymptomatic till 2 years of age ,then she had shortness of breath and was rushed to hospital,where her condition deteriorated and got admitted .They were told that she had splenomegaly and her blood counts were decreasing  . She was given multiple blood transfusions every 25 days for an year inspite of that, she remained anemic. She also underwent bone marrow biopsy twice.


According to parents she was given steroids also for one year before death .she never had jaundice or recurrent infection


His elder brother is 19 years old and second brother is 16 years old


Second one had history of fever ,white coloured loose stools at the age of 5 years for which they went to nalgonda hospital .He was given some medications and it got resolved .But he was said to have anemia ,and he recovered according to parents with some medications.There was no jaundice and no history of blood transfusions.

H/o scabies in the family 2 years ago, 

His second brother got affected first after which it spread to the patient, then his grandmother and then to his mother. They detected scabies in his mother, then rest of family members were diagnosed to have scabies for which they got treated. 


PERSONAL HISTORY

Diet:mixed

Appetite:normal

Sleep:normal

Bowel and bladder movements:regular

No addictions


TREATMENT HISTORY

Dexamethasone valerate skin cream

Dermadew lotion


GENERAL EXAMINATION

Patient is conscious, coherent and cooperative well oriented to time place and person.

Built - thin

Height- 156cm            Weight- 32kg

BMI-13.15 kg/m2 (severely underweight)

Pallor - present

Icterus - present

Cyanosis, Clubbing, Lymphadenopathy, oedema - Absent














VITALS-

Temp : afebrile(98.6°F)

PR :90bpm

RR :22cpm

BP :120/70mmhg


SYSTEMIC EXAMINATION

CVS : S1,S2 heard. Shift in apex beat to 4th ics

RS : BAE+ NVBS heard

CNS : NFND

P/A : Moderate splenomegaly palpable upto the left lumbar region


INVESTIGATIONS:


Blood grouping: B+ve 


Complete blood picture:

6/1/22







Hb - Decreased

TLC- decreased      

Eosinophils - slight increase

PCV- reduced

RBC- reduced

RDW-CV - increased


9/1/22

Improved from the date of admission

Eosinophil count - normal









Peripheral smear

Normocytic normochromic blood picture with few microcytes, tear drop cells, pencil forms


Leucopenia.


Hb electrophoresis: normal




Ultrasonography: splenomegaly



Thyroid profile

T3 : 61ng/dl

T4 : 8.66ng/dl

TSH : 91.85mIU/l

Anti TPO antibodies- 77.1


Coombs test

Direct coombs test : positive

Indirect coombs test : negative


Coagulation profile

BT, CT, aPTT, PT - normal.


Liver function tests

6/1/22

Total bilirubin - 6.49   

Direct bilirubin - 0.52 

AST (SGOT) - 19  

ALT (SGPT) - 10 

ALP - 274 

Total Protein - 5.5   

Albumin - 3.8  


9/1/22







Total bilirubin- high

Direct bilirubin- slightly high

Indirect bilirubin- very high



PROVISIONAL DIAGNOSIS:

SPECTRUM OF AUTOIMMUNE DISEASES-

AUTOIMMUNE THYROIDITIS.

AUTOIMMUNE HEMOLYTIC ANEMIA

CVID?


TREATMENT:

TAB.METHYLPREDNISOLONE 32mg PO/OD

TAB.THYRONORM 200micrograms PO/OD

PHYSIOGEL LOTION

TAB. ATARAX 10mg




Monitor vitals for every 4hrs.








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