13 YEAR OLD BOY WITH MULTIPLE CLINICAL EVENTS
A 14 yr old male came to the opd with complaints of swelling in the left side of the abdomen since the past 15 days along with cold and cough since the past 10 days.
HOPI:
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 assymoptomatic till 2 years of age ,then she had shortness of breath and was rushed to hospital,where here condition detiorated 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 one year inspite of that she remained anemic ,she also underwent bone marrow biopsy twice .
According to parents she was given steriods also for one year before death .she never had jaundice or recurrent infections.
.Elder brother is 19 year 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 transfusion.
Pedigree chart :
Patient history :
He is the youngest of all .
He was born out of normal vaginal delivery with 4kgs birth weight ,cried immediately after birth .
He had jaundice at birth ,that resolved spontaneously .? Physiological jaundice.
He is Immunised as per schedule .
At 8 months of age patient had history of altered bowel habits - one week of loose stools and one week of normal consistency stools for few days later he was passing loose stools every day for 2-3 months ,during which he became very cachetic .
He got admitted in a hospital ,in the discharge summary - they mentioned as suspected case of celiac disease ,chronic diarrhea ,chronic malabsorption .
During the hospital stay he was given Iv antibiotic and 2FFP transfusions .He recovered in 3 months according to parents .
On further asking parents ,they told he used to have recurrent colds ,cough and fever .He used inhalers for 1 year during winters in childhood . Though he wasn't admitted at any point , and there was no history of pnuemonia.
At 9 years of age - parents noticed neck swelling and they took him to ENT doctor ,later referred to endocrinologist ,where he was diagnosed with hypothyroidism - intial TSH was -150 ,he was started with 150 mcg of thyronorm,later increased to 180 mcg .
patient also gives history of chronic itching over hands and foot since the age of 9 years .
At 12 years of age patient had chicken pox ,resolved over 10 days.
9 months ago
at age of 13 years - he came with history of yellowish discoloration of eyes since 9 days .
One episode of bilious vomiting .
He also complaints of yellow colored urine .
No pale colored stools .
No fever ,pain abdomen ,loose stools.
No cold , cough .
No history of small joint pains .
He was treated by a pediatrician for jaundice ,but as the bilirubin is increasing they referred here for further management .
On examination -
He is thin built .
Height -156 cm
Weight - 32kgs
Jaundice + ,pallor +
Per abdomen - moderate splenomegaly
Outside investigations -
There is significant drop in hemoglobin from 8.9 to recent HB -5,leucopenia , thrombocytopenia.
With raised bilirubin - both direct and indirect , normal enzymes .
Urine for bile salts and bile pigments + ve
HB electrophoresis - normal .
Serology for HiV ,HBSag , Hcv was negative
Dengue , Malaria ,widal was negative
At present:
He noticed a swelling in the left side of the abdomen since the past 15 days along with cold and cough since the past 10 days.
Pain is at left side of the abdomen and is of dragging type, non radiating.
General examination showed thin built and moderately nourished with massive splenomegaly
Assessment :
? Spectrum of Autoimmune diseases -
Thyroditis ,? AIHA
Chronic itching of both hands and limbs
? CVID
Plan - repeat CBP with peripheral smear ,LDH ,retic count ,coombs test .
Other routine investigations
Dermatology opinion .
Investigations in our hospital :
Hemogram showed -
Normocytic normochromic anemia with HB 5.1
WBC count -1700 ,neutrophils -39% ANC-663 ,Esoniphils -8% .
Platelet count -1.5 lakh
With few microcytes ,tear drop cells ,pencil forms.
Chest x-ray
2D Echo : https://youtu.be/7NAXqdO4ZDI
FEVER CHART :
27/08/22
Coombs test
AMC bed 3 14yr old male
Admit on 26/08/22
Day 3
S: no fresh complaints
O: pt is conscious, coherent,cooperative.
BP: 110/80mmhg
PR: 89
RR: 24
Temp: 99.2f
GRBS: 83mg/dl -8am
Spo2: 98%
RS: BAE+ clear
CVS: s1,s2 no added sounds
P/A: not tender
CNS:NAD
A: pancytopenia with massive splenomegaly
Thyroditis ,? , URTI
P: plan of treatment
1.Inj. Neomol 1gm IV SOS
2.Tab dolo 650 mg PO SOS
3.Tab. Levocetrizine 5mg PO SOS
4.Syrup ascoril 10 ml PO BD
5.tab. thyronorm 200mcg, PO OD
6.inj. optineruron 1 amp
7. BP temp charting 6th hourly
Xray 28/08/22
Admit on 26/08/22
Day 4
S: no fresh complaints
O: pt is conscious, coherent,cooperative.
BP: 110/70mmhg
PR: 84
RR: 22
Temp: 99.2f
Spo2: 98%
RS: BAE+ clear
CVS: s1,s2 no added sounds
P/A: not tender
CNS:NAD
A: pancytopenia with massive splenomegaly
Thyroditis ,? , URTI
P: plan of treatment
1.Inj. Neomol 1gm IV SOS
2.Tab dolo 650 mg PO SOS
3.Tab. Levocetrizine 5mg PO SOS
4.Syrup ascoril 10 ml PO BD
5.tab. thyronorm 200mcg, PO OD
6.inj. optineruron 1 amp
7. BP temp charting 6th hourly.
AMC bed 3 14yr old male
Admit on 26/08/22
Day 5
S: no fresh complaints
O: pt is conscious, coherent,cooperative.
BP: 110/80mmhg
PR: 82
RR: 22
Temp: 97.9f
Spo2: 98%
RS: BAE+ clear
CVS: s1,s2 no added sounds
P/A: not tender
CNS:NAD
A: pancytopenia with massive splenomegaly
Thyroditis ,? URTI
P: plan of treatment
1.Inj. Neomol 1gm IV SOS
2.Tab dolo 650 mg PO SOS
3.Tab. Levocetrizine 5mg PO SOS
4.Syrup ascoril 10 ml PO BD
5.tab. thyronorm 200mcg, PO OD
6.inj. optineruron 1 amp
7. BP temp charting 6th hourly
AMC bed 3 14yr old male
Admit on 26/08/22
Day 6
S: no fresh complaints
O: pt is conscious, coherent,cooperative.
BP: 110/80mmhg
PR: 80
RR: 20
Temp: 97.9f
Spo2: 98%
RS: BAE+ clear
CVS: s1,s2 no added sounds
P/A: not tender
CNS:NAD
A: pancytopenia with massive splenomegaly
Thyroditis ,? AIHA, URTI
P: plan of treatment
1.Inj. Neomol 1gm IV SOS
2.Tab dolo 650 mg PO SOS
3.Tab. Levocetrizine 5mg PO SOS
4.Syrup ascoril 10 ml PO BD
5.tab. thyronorm 200mcg, PO OD
6.inj. optineruron 1 amp
7. BP temp charting 6th hourly
Ugie
Case discussion:
https://pubmed.ncbi.nlm.nih.gov/2944377/
Three cases of idiopathic portal hypertension associated with Hashimoto's disease are described. All of the cases were middle-aged Japanese women showing splenomegaly, esophageal varices and pancytopenia in the absence of extrahepatic portal obstruction, and cirrhosis of the liver. Two patients were euthyroid with goiter, one of which revealed diffuse lymphocytic infiltration, obliteration of thyroid follicles, and fibrosis on histological examination of the thyroid; the third suffered from myxedema without goiter. Antithyroid microsomal antibody was positive in all patients and antithyroglobulin antibody was positive in none. These findings might imply an immunological role in the pathogenesis of idiopathic portal hypertension.
There is a general agreement that splenomegaly is a common feature in PAD but its consequences are not well understood [19]. Here, we confirmed our previous data [20] showing a spleen enlargement in 71/117 patients (61%) of our cohort. Spleen diameter was highly correlated with portal vein diameter (R2 = 0.5; P < 0.0001) (Figure 1) suggesting that an increased splenic venous flow secondary to splenomegaly could contribute to determining a condition of portal superflux. At ultrasound, 30 out of 117 patients (25.6%) had signs of portal vein enlargement but only 1/6 of these had portal hypertension/INCPH, with portal systemic collaterals (Figure 2). Longitudinal assessment of abdominal ultrasounds demonstrated that portal vein enlargement and splenomegaly slightly increased in the observation period
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3988706/
Thirty patients (28 CVID, 2 XLA) had portal vein enlargement detected by ultrasounds, an indirect index of portal hypertension. Four of these patients (3 CVID and 1 XLA) had esophageal varices (3 small and one large) at upper gastrointestinal endoscopy and one patient had portal vein collaterals detected by CT scan. None of the patients had portal hypertensive gastropathy. These five patients underwent liver biopsy, which excluded cirrhosis and thus they fulfilled the diagnostic criteria for INCPH [12]. In the remaining 25 patients with portal vein enlargement without other clinical or radiological signs of portal hypertension the liver biopsy was not performed for ethical reasons.
Discharge summary
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