Case summary
11 years female child came to our OPD with complaints of 4 episodes of sudden loss of consciousness,what the parents called,fainting attacks 10 months back.
On all occasions,there was no abnormal body movement and each episode lasted for less than a minute.
The child was completely normal after the episodes.
In the last 3 months ,the parents noticed easy fatiguebility in the child.
The child was experiencing breathlessness on little exertion.
ON clinical examination
There was no clubbing,respiratory rate and pulse rate were normal,SPo2 on room air was 98%
On chest auscultation,breath sounds were normal and there was no adventitious sound.
ON auscultaion of heart-P2 was loud
INVESTIGATION;Chest X-Ray was normal,Echocardiography revealed enlarged right atrium and right ventricle and bulging of ineratrial septum towards left atrium.
Pulmonary artery pressure was 60 mm of Hg.
CT Pulmonary angiography revealed filling defects in pulmonary arteries supplying to the right upper lobe of the lung at multiple sites and few sites on the left lung also.
Pulmonary trunk was dilated and heart chambers of right side were enlarged.
On further investigations-Factor V Leiden,Protein C,Protein S were negative but ANA was positive(1:320).
Anti ds antibody,anti smith antibody and ENA(Extractable nuclear antigen) were negative.
Lupus anti coagulant elevated,anti phospholipid antibodies in the form of anticardiolipin antibody and anti beta 2 glycoprotein 1 antibodies on two occasions ,3 months apart were positive.
DIAGNOSIS
According to EULAR 2006(,European Alliance of Association of Rheumatology) criteria the diagnosis was made of anti phospholipid syndrome–APS
TREATMENT:
Enoxaperin 1 mg /kg/dose 12 hourly was started for pulmonary thrombosis along with Sildenafil 12.5 mg three times daily for pulmonary hypertension.
Discussion:
we present a case of a female child with pulmonary hypertension due to pulmonary thrombosis with antiphospholipid syndrome.
It is important to recognise the association of Pulmonary thrombosis with Antiphopholipid syndrome(APS)
Pulmonary hypertension is dianosed if Pulmonary artery pressure is more than 25 mm of Hg IN CHILDREN > 3 months.
Symptoms include fatigue,exertional dyspnea and syncope.
Treatment includes PED5 inhibitor like sildenafil
It is not uncommon in adults but rare in children with frequency of less than 1 per million children.
APS is diagnosed when at least one clinical condition like thrombosis or morbidities of pregnancy and at least one laboratory finding (Anti lupus anticoagulant ,Antiphospholipid antibody) are positive on 2 separate occasions 12 weeks apart.It is more common in female and treatment is anticoagulants for thrombosis.
In children mostly it is secondary to autoimmune diseases and 20% primary APS develop lupus.
CONCLUSION: Since the association of APS with PH is rare ,it will be beneficial to report such case as early recognition and treatment may improve the outcome
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