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Table of Contents
Year : 2018  |  Volume : 1  |  Issue : 2  |  Page : 96-98

Severe abdominal pain in an adolescent: More than what meets the eye!

1 Division of Pediatric Nephrology, Sheikh Khalifa Medical City, Abu Dhabi, UAE
2 Department of Pediatrics, Christian Medical College, Ludhiana, Punjab, India

Date of Web Publication27-Dec-2018

Correspondence Address:
Dr Gurinder Kumar
Division of Pediatric Nephrology, Sheikh Khalifa Medical City, Abu Dhabi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/AJPN.AJPN_23_18

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Antiphospholipid syndrome (APLS) is a systemic autoimmune disorder characterized by venous or arterial thrombosis in the presence of persistent laboratory evidence of antiphospholipid antibodies. We report a case of a previously healthy, 14-year-old boy, who presented with abdominal pain and headache for one day. Magnetic resonance imaging of the brain revealed sinus venous thrombosis and multiple areas of arterial and venous infarction. Computed tomography of the abdomen showed smaller right kidney with multiple wedge-shaped non-enhancing cortical areas of renal infarction. A diagnosis of APLS was made following demonstration of antibodies to cardiolipin and beta-2 glycoprotein, and the child was managed conservatively with antihypertensive agents, anticoagulation, and immunosuppressants. Follow-up imaging after one year showed resolution of cerebral thrombosis and improvement in renal functions.

Keywords: Antiphospholipid syndrome, renal infarction, venous thrombosis

How to cite this article:
Kumar G, Chaudhry M, Mansour Mohamedfaris KM, Sohal A. Severe abdominal pain in an adolescent: More than what meets the eye!. Asian J Pediatr Nephrol 2018;1:96-8

How to cite this URL:
Kumar G, Chaudhry M, Mansour Mohamedfaris KM, Sohal A. Severe abdominal pain in an adolescent: More than what meets the eye!. Asian J Pediatr Nephrol [serial online] 2018 [cited 2021 Oct 22];1:96-8. Available from: https://www.ajpn-online.org/text.asp?2018/1/2/96/248637

  Introduction Top

Antiphospholipid syndrome (APLS) is an autoimmune multisystem disorder characterized by large or small vessel thrombosis in various organs associated with antiphospholipid or antiprotein/phospholipid complex antibodies. The diagnosis may be challenging due to myriad presentations and its rarity in the pediatric age group. The life-threatening variant, termed catastrophic APLS is characterized by multiorgan thrombosis developing over a short period.

  Case Report Top

A 14-year-old, previously healthy boy without any significant past history, presented with abdominal pain and headache for one day following an upper respiratory tract infection. Clinical examination revealed an irritable child of average built. The heart rate was 70/min, respiratory rate 22/min, and blood pressure 150/100 mm Hg, indicating stage 2 hypertension. The blood pressure was symmetrically elevated in all four limbs and all peripheral pulses were palpable. There was no skin rash, neurocutaneous markers or bruits. Neurological examination did not reveal any focal neurological deficits, cranial nerve palsies, meningeal signs or ataxia. Systemic examination was unremarkable.

Magnetic resonance imaging (MRI) of the brain was done in view of severe headache and showed sinus venous thrombosis [Figure 1] and multiple areas of arterial and venous infarction. Computed tomography of the abdomen with contrast showed relatively small-sized right kidney with multiple wedge-shaped nonenhancing cortical areas suggestive of renal infarction [Figure 2]. Echocardiography was normal, thus ruling out long-standing hypertension. Renal functions were deranged (creatinine 88 μmol/L and urea 15 mmol/L) while serum electrolytes were normal. Urine output was normal. In view of thrombosis in two major organs, thrombophilia workup was done, which showed prolonged aPTT that was not corrected with mixing study. Genetic analysis ruled out mutation in MTHFR, prothrombin, and factor V Leiden mutation. Protein C and S levels were normal. ADAMTS13 level was normal and ruled out thrombocytopenic purpura. ESR was elevated (36 mm/hr). Antiphospholipid antibodies (anticardiolipin and anti-beta-2 glycoprotein) were both positive, done twice 6 weeks apart. Cardiolipin IgG was 27.30 (normal < 10.00) units/ml. IgG and IgM beta-2 glycoprotein 1 antibodies were strongly positive (>40 U/mL each). Lupus anticoagulant was positive with titer 80 (normal 20–39) GPL units. Lupus workup was negative. Nuclear scan of the kidneys showed differential function of left kidney at 71% and that of right kidney at 29%. Based on involvement of two organs, a diagnosis of APLS was made. Histopathological confirmation of small vessel occlusion could not be done as renal biopsy was refused by family. He required therapy with multiple antihypertensives (amlodipine, labetalol, prazosin and hydralazine) initially. Anticoagulants (low molecular weight heparin followed by warfarin and then aspirin) and immunosuppressive agents (intravenous methylprednisolone followed by oral steroids and azathioprine) were started. Intravenous immunoglobulins was given at 0.4 g/kg/day for 5 days.
Figure 1: Magnetic resonance imaging of the brain showing right transverse sinus thrombosis

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Figure 2: Computed tomography of the abdomen showing right renal infarct

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The child showed clinical improvement on follow-up at 1 year. Repeat MRI of the brain has shown resolution of the infarcts. The titers of antiphospholipid antibodies also showed a falling trend on follow up at 1 year.

  Discussion Top

APLS is a systemic autoimmune disorder characterized by venous or arterial thrombosis in the presence of persistent laboratory evidence of antiphospholipid antibodies. Catastrophic antiphospholipid syndrome (CAPS) is the severe form defined by the development of thrombotic disease involving three or more organs within a week, in the presence of antiphospholipid antibodies and confirmation of small-vessel occlusion by histopathology.[1] It occurs in 0.8% of patients with APLS, and is associated with mortality in of 30% cases despite current treatment with anticoagulants and immunosuppressants.[2],[3] In view of involvement of two organs in the index case, a diagnosis of APLS was made. Small-vessel occlusion was not confirmed histopathologically which might have enabled the diagnosis of CAPS.

Most cases of CAPS occur in the context of underlying autoimmune disease. The usual organ affected in children include kidneys, lungs, and heart, followed by the brain. Data from CAPS registry (2014) reports 45 children of whom the majority had kidney (63%) and lung involvement (63%).[4] Infections are more often the precipitating factor for CAPS in pediatric as compared to adult population.[4]

The pathophysiology of APLS is based on the action of multiple autoantibodies against various antigenic targets. There is increased propensity for thrombus formation as a result of many contributory elements, including antiphospholipid-mediated activation of monocytes, platelets, and endothelial cells and antiphospholipid-induced perturbation of natural anticoagulant and fibrinolytic systems.

Lethal outcomes of this rare disease have been described in literature. Prasad et al. described a 7 year old girl, who presented with hemolytic anemia associated with antiphospholipid antibodies and later died because of CAPS.[5] Gru and Dehner also reported a 2-year-old girl with trisomy 21 presenting with insidious skin rash, which was rapidly followed by multiorgan failure and death.[6]

Renal involvement can lead to infarcts which can lead to chronic kidney disease, as in our child. However, in some cases, severe renal involvement can lead to mortality. Park et al. reported a 7-year-old girl who presented with multiple thrombotic episodes and rapidly progressive renal failure and had cerebral, femoral, and renal involvement.[7] Sharma et al. described a 12-year-old boy who presented with multiple thrombotic episodes and rapidly progressive renal failure.[8] Renal arterial thrombosis in a 2-year 6-month-old child due to antiphospholipid syndrome requiring nephrectomy has been described by Macedo et al.[9]

Early detection and treatment have been described to have favorable outcomes in children with CAPS. Diagnosis may sometimes be difficult as described by Canpolat et al. in a 14-year-old girl with CAPS, who was previously misdiagnosed as vasculitis related to parvovirus B19 infection.[10] Haskin et al. described three adolescents who were diagnosed with probable CAPS and had severe abdominal pain as the presenting symptom similar to our index child.[11]

Long-term anticoagulation (intravenous heparin followed by oral anticoagulants) and immunosuppressive agents (corticosteroids) are used in the management of CAPS. In severe cases, intravenous immunoglobulins at 0.4 g/kg/day for 4–5 days and/or plasma exchange are the mainstay of treatment. In our patient, anticoagulation, steroids, and IVIG resulted in successful resolution of the thrombosis of the brain. The case emphasizes that high index of suspicion can aid in early diagnosis and favorable outcomes among children with APLS. Symptoms of severe abdominal pain out of proportion to physical findings may be a clue to underlying ischemia in organs such as the kidney.

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Conflicts of interest

There are no conflicts of interest.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

  References Top

Asherson RA, Cervera R, de Groot PG, Erkan D, Boffa MC, Piette JC, et al. Catastrophic antiphospholipid syndrome: International consensus statement on classification criteria and treatment guidelines. Lupus 2003;12:530-4.  Back to cited text no. 1
Pengo V, Denas G. Diagnostics and treatment of thrombotic antiphospholipid syndrome (APS): A personal perspective. Thromb Res 2018;169:35-40.  Back to cited text no. 2
Cervera R, Rodríguez-Pintó I, Espinosa G. The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review. J Autoimmun 2018;92:1-1.  Back to cited text no. 3
Berman H, Rodríguez-Pintó I, Cervera R, Gregory S, de Meis E, Rodrigues CE, et al. Pediatric catastrophic antiphospholipid syndrome: Descriptive analysis of 45 patients from the “CAPS registry”. Autoimmun Rev 2014;13:157-62.  Back to cited text no. 4
Prasad N, Bhadauria D, Agarwal N, Gupta A, Gupta P, Jain M, et al. Catastrophic antiphospholipid antibody syndrome in a child with thrombotic microangiopathy. Indian J Nephrol 2012;22:310-3.  Back to cited text no. 5
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Gru A, Dehner LP. Catastrophic antiphospholipid syndrome in a child with trisomy 21. An acquired thrombopathy with a discussion of thrombopathies in childhood. Pediatr Dev Pathol 2010;13:178-83.  Back to cited text no. 6
Park JM, Shin JI, Shin YH, Kim DS, Lee JS, Kim MJ, et al. Catastrophic antiphospholipid syndrome in a 7-year-old girl. Clin Rheumatol 2007;26:1011-3.  Back to cited text no. 7
Sharma J, Karthik S, Rao S, Phadke K, Crasta J, Garg I, et al. Catastrophic antiphospholipid antibody syndrome. Pediatr Nephrol 2005;20:998-9.  Back to cited text no. 8
Macedo CS, Martinez RS, Riyuzo MC, Bastos HD. Renal arterial thrombosis and the antiphospholipid antibody syndrome: A case report. J Pediatr (Rio J) 2001;77:517-21.  Back to cited text no. 9
Canpolat N, Topal N, Civilibal M, Caliskan S, Sever L, Kasapcopur O, et al. A case of catastrophic antiphospholipid syndrome in an adolescent girl with parvovirus B19 infection. Clin Pediatr (Phila) 2008;47:593-7.  Back to cited text no. 10
Haskin O, Amir J, Schwarz M, Schonfeld T, Nahum E, Ling G, et al. Severe abdominal pain as a presenting symptom of probable catastrophic antiphospholipid syndrome. Pediatrics 2012;130:e230-5.  Back to cited text no. 11


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