Hemophagocytic lymphohistiocytosis (HLH) is an inflammatory condition that may run a rapid fatal course and calls for prompt diagnosis. Early intervention with steroids and other immunosuppressive drugs can contain the disease process and favours positive outcome. Ferritin ≥500 ng/ml is a HLH diagnostic criterion. We evaluated the diagnostic potential of admission ferritin, in children with HLH. Pediatric patients of a referral teaching hospital from Feb 2010-Oct 2013 having been investigated for ferritin on admission were included. HLH was confirmed when patients had clinical features and met 5/8 diagnostic criteria of the revised 2004 HLH guidelines. Ferritin was estimated on Cobas e411 by electrochemiluminiscence, with a measuring range of 0.5-2000 ng/ml. Dilutions were made when linearity exceeded and absolute values were reported. 905 on-admission ferritin investigations were reviewed out of which 346 values ≥500 ng/ml. Hyperferritinemia was seen in HLH/MAS (macrophage activation syndrome) [HLH group, median age 4 year 4 month, 59% male] and in systemic lupus erythematosus, sepsis, juvenile idiopathic arthritis, impending HLH, haemolytic anemias and malignancy [non-HLH group, median age 4 year 6 month, 60% male]. Of 346, 72 cases of hyperferritinemia were diagnosed with secondary HLH while one patient had primary HLH. 13/73 patients expired. The median ferritin level of the HLH group was significantly higher [6556 (2402-11,734) ng/ml] compared to non-HLH group [median 1175 (943-2000) ng/ml] ( < 0.0001). Receiver operator characteristics curve analysis revealed optimal admission ferritin of 3120 ng/ml as the cut-off with sensitivity of 70% and specificity of 88.9% for HLH diagnosis, exceeding the currently prescribed cut-off of 500 ng/ml. Hyperferritinemia below 3120 ng/ml has higher negative predictive value to rule out secondary HLH on admission in the study population of children predominantly diagnosed with infection associated HLH than the prescribed cut-off as per the 2004 guidelines. This may prove to be beneficial to alert physicians for prompt intervention which considerably decreases mortality in this often fatal condition.
Scrub typhus is an endemic disease of Asian Pacific region, affecting about 1 million people every year. Although it remarkably recovers with doxycycline, the mortality in untreated cases may be as high as 40 to 45%. Owing to the lack of diagnostic facilities for scrub typhus in most places, it is very important to recognize the clinical features. A typical eschar is pathognomonic of the disease. However, this is very rare to be found on the face, and hence can lead to confusions. We present the case of a boy who presented three eschars on the upper lip and was diagnosed as a case of scrub typhus. He responded dramatically with intravenous doxycycline and the eschars healed spontaneously over the next 2 to 3 weeks.
Background: Fever is the commonest presentation of pediatric patients attending emergency departments of all children’s hospital. The cause of fever may be acute bacterial infections or primary vasculitic disorders like Kawasaki disease or inflammation due to non-bacterial infections. The objective was to compare the performance of the four biomarkers ESR, CRP, Procalcitonin and IL-6 in predicting a diagnosis of sepsis/infection and find out a definite cut off value for the statistically most significant one.Methods: The authors conducted this prospective observational study at the indoors of a pediatric tertiary care referral center in India to find out a biomarker which can differentiate between infection and inflammation (vasculitis) in children admitted with fever and finally diagnosed as infection or inflammation (vasculitis).Results: Among ESR, CRP, Procalcitonin and Interleukin-6, only IL-6 showed statistical significance in differentiating infection from inflammation (vasculitis) analysed using the Receiver Operating Characteristic (ROC) curve and Mann-Whitney U test, sensitivity and specificity.Conclusions: IL-6 level 27 pg/mL or less at the time of admission indicates an infectious etiology while level more than this indicates towards a vasculitic cause.
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