Dr.Subbulakshmi P S, Dr.Jayakumar.C, Dr.Vinitha Prasad Dr. Praveena N Bhaskaran, Dr.Suchitra Sivadas
AIMS, Kochi
7 month old girl presented with generalised edema of 1 week ,High grade intermittent fever of 4 days,high colored ,reduced urine output of 2 days and yellowish discolouration of eyes since 2 days.No periorbital puffiness was noted.No fast breathing/Loose or clay colored stools/vomiting/altered sensorium/abnormal movements/foul smelling urine/mucocutaneous bleeding.
At 4 months of age,child developed erythematous scaly with pruritus rashes ?Atopic eczema over the limbs and for which she was started on homeo treatment following which lesions started healing with hypopigmented patches.
Hypopigmented patches
Good weight gain was also noted during routine immunisation visits.No h/o oral thrush/ear infection/pneumonia. The child was exclusively breastfed till 6 months Growth velocity was decreased after 6 months. Complementary feeding was done with ragi and later the child received carrot and other fruit blends with ragi.
On examination, the child was febrile and irritable with other vitals being stable. Pallor +,Icterus +,Generalised Oedema + ,and local examination showed Cushingoid facies +, Bilateral non tender pitting pedal edema +,No eschar. The ba had grade 1 PEM, No stunting and Grade 1 wasting.
Systemically Hepatosplenomegaly ,Liver : 5 cm below RCM ,Span 9cm ,Soft with rounded borders,and Spleen 4 cm below LCM .
Labs Hb of 5gm%, Hyperleukocytosis with WBC of 65,000/cmm.Thrombocytopenia and Coagulopathy. Elevated inflammatory markers,Direct hyperbilirubinemia ,Transaminitis, Mild AKI and Low albumin levels.
USG Abdomen – Borderline hepatomegaly..No free fluid. URE done showed numerous pus cells Blood & urine c/s –showed Significant growth of Ecoli, Echocardiogram was Normal. Peripheral smear done showed microcytic hypochromic anemia with leukoerythroblastic picture with 2% blasts. With Bone marrow smear Immunophenotyping was done showed No definite evidence of acute leukaemia/bone marrow involvement B/T cell non-Hodgkin lymphoma.
She was started on IV Antibiotics and 2 units of PRBC ,Platelets were given. Albumin transfusions were given and dyselectrolytemia was also corrected fluid management.In view of direct hyperbilirubinemia with transaminits and altered coagulation profile and FFP transfusions & Vit K was given. Age specific immunoglobulin profile done and complement levels which were normal. The fever spikes came down and antibiotics were downgraded. Respiratory Viral panel done was negative. Tropical Fever Panel test done was negative. An ophthalmological evaluation was done and there was no cherry red spots or opacifications with normal macula and retina.
Differential diagnosis:
1. Acute leukaemia in favour child(Leucocytosis,pallor,HSM2% blasts in peripheral smear) against Well looking child
2. Kwashiokor in favour (Hepatomegaly,edema,sepsis)against No skin or hair changes
3. Metabolic disorders-GSD 1 (Edema,Hepatomegaly with renal involvement)
4. Secondary immune suppression and sepsis(due to use of alternative medications)
5. Primary immunodeficiency(Eczema and sepsis)
Kwashiokar with sepsis was ruled out as the child had weight gain and on complementary feeding practice
IEM(GSD I) was ruled out as there is no features of hypoglycemia,hyperuricemia or consanguity among the parents. Hematological malignancies like acute leukemia was ruled out as the child is well looking with normal uric acid and LDH with no history of recurrent fever or bone pain.and Bone marrow smear Immunophenotyping was normal
Primary immunological disorders were ruled out as the child was thriving well till 6 months of age with no history of recurrent infections. Secondary immune suppression is most commonly caused steroids as the child had a negative pre-op serology.
In view of history of usage of native medications for the skin condition following which the skin condition improved with appearance of cushingoid facies and sepsis,a possibility of steroids was suspected and a toxicology screening of blood & urine was done for the same and urine was found to be positive for steroids
Hence it was diagnosed to be a STEROID INDUCED TOXICITY with severe anemia and sepsis with AKI,Coagulopathy and direct hyperbilirubinemia.
POINTS IN FAVOUR OF THE DIAGNOSIS:
● Eczema improved following native medications which raises the suspicion of steroids.
● Futher,the development of sepsis with cushingoid features and MODS strongly suggests the possibility of rampant unsupervised steroids use
Serial monitoring of weight was done and it was found to be in improving trends. Child was started on oral iron and vitamin supplements. Parents were given dietary advices and also advised to stop native medications and proper treatment of eczema was initiated after dermatologist opinion. Child improved clinically and was discharged with hemodynamically stable vitals. Weight at the time of discharge -5.73 kg .
DISCUSSION:
It is high time to make it mandatory for all the indegenous medications to have proper labelling and to be subjected to quality control mechanisms to check for the proliferation of dispensing spurious drugs unqualifed/unregistered practitioners. Such measures can minimize the adverse health effects for the patients.