Langerhans' Cell Histiocytosis Masquerading as Caroli's Disease.

Langerhans' cell histiocytosis is an uncommon disease in children with varied clinical presentation. Multisystem form of this disorder usually affects organs like the bones, skin, liver, spleen, lungs, and the central nervous system. We describe here the clinical details of a 2-year-old girl with involvement of unusual sites like the parotid glands and the nails. This child also had multiple cystic lesions in the liver leading to a misdiagnosis of Caroli's disease. Knowledge about the uncommon manifestations of this rare disorder helps in early diagnosis and treatment.

Acute Motor Axonal Polyneuropathy Following Mumps Infection in a 9-Year-Old Girl.

A 9-year-old girl presented with lower motor neuron type of paralysis involving limbs, trunk and multiple cranial nerves (7, 9 and 10) with preceding history of mumps 1 week before the onset of weakness. There were no features to suggest either a meningitis or encephalitis in the child. Cerebrospinal fluid showed hypoglycorrhachia and mild protein elevation; magnetic resonance imaging of the brain was normal. Nerve conduction study showed motor axonal neuropathy. Serology for mumps IgM was positive, consistent with a diagnosis of post-mumps acute motor axonal polyneuropathy. The girl made a complete recovery within 3 weeks.

Clinical Profile and Outcomes of Children with Acute Liver Failure in a Tertiary Care Center in South India: A Retrospective Study.

Purpose: In this study, we investigated the clinical profile, survival at discharge, and proportion of children with acute liver failure (ALF) meeting the criteria for, yet surviving without, liver transplantation (LT). Methods: Medical case records of children aged >28 days to ≤15 years over a period of 7 years, identified from pediatric admission and discharge registers, were screened. Children satisfying the criteria for ALF were included in this study. Results: A total of 71 records meeting the pediatric ALF (PALF) criteria were included. The survival rate at discharge was 61% (n=44). A considerable proportion of children satisfied the King's College Criteria (KCC) (56.3%) and the European Association for the Study of the Liver (EASL) criteria (7%) for LT at admission. Nonetheless, the survival rate in the absence of LT was 42.5% in children who satisfied the KCC and 20% in those who met the EASL criteria. Infection (29.5%) and paracetamol overdose (19.7%) were the major identifiable causes of PALF. Hepatitis A was the most common infection identified. No significant predictors of poor outcomes were identified in multivariable analysis. Conclusion: Our study highlights the changing survival rates and the clinical and etiological profiles of patients with PALF. In areas with poor access to LT services, survival in these children could be improved through early referral to centers with adequate intensive care facilities. Preventing ALF and referring patients to LT services are paramount to reducing mortality.

Second Dose of Scorpion Antivenom in Children with Indian Red Scorpion (Mesobuthus tamulus) Sting Envenomation.

OBJECTIVE: To study the effect of the second dose of scorpion antivenom in children with non-resolving or worsening scorpion sting envenomation. METHODS: 72 children aged ≤12 years with scorpion sting envenomation grade 2 and above were enrolled. 61 received the first dose of three vials of antivenom at admission (group A). Children with persistent/worsening envenomation at 6 hours received the second dose (group B). The time required for resolution of autonomic symptoms, myocardial dysfunction, predictors of the second dose and side effects were studied. RESULTS: The mean time taken for resolution of autonomic symptoms were similar in GroupA and B (4.1 vs. 5.3 hours, P=0.452), and of myocardial dysfunction was shorter in Group A (10.8 vs. 37.6 hours, P=0.019). On regression analysis, abnormal echocardiography at admission was found to be a significant predictor of the second dose (OR=27.6, 95% CI, 4.7-162.5; P=<0.001). CONCLUSION: Children with severe scorpion sting envenomation with abnormal echocardiography may require a higher dose of scorpion antivenom.