Emergent presentation of Langerhans cell histiocytosis in a pediatric patient: Acute cerebellar involvement causing obstructive hydrocephalus requiring posterior fossa decompression.

Langerhans cell histiocytosis (LCH) is a disorder of the monocyte-macrophage system that can be unifocal or systemic. Here, we present a pediatric case who initially presented with osseous LCH but again presented 6 years later emergently with cerebellar symptoms, cerebellar mass and obstructive hydrocephalus. Patient underwent biopsy of the cerebellum which was path proven intracranial LCH.

Sjögren-Larsson syndrome: definitive diagnosis on magnetic resonance spectroscopy.

Sjögren-Larsson syndrome (SLS) is a rare autosomal-recessive neurocutaneous disorder comprising a triad of ichthyosis, mental retardation, and spastic diplegia or quadriplegia. It has rarely been reported in Asian and Indian populations. We report the case of an Indian patient with SLS who presented with the classical clinical triad and demonstrated characteristic findings on magnetic resonance spectroscopy. In resource-restricted settings where enzymatic and genetic analyses are not available, magnetic resonance spectroscopy serves as a useful adjunct in confirming the diagnosis of SLS.

Efficacy and safety of sofosbuvir-based therapy for chronic hepatitis C infection in "real-life" cohort.

BACKGROUND: The safety and efficacy of sofosbuvir-based treatment (sofosbuvir and ribavirin with or without pegylated interferon-α) for hepatitis C virus (HCV) infection has been established in clinical trials. However, there is limited data regarding safety and efficacy of sofosbuvir-based treatment for HCV infection in a "real-life" cohort. We describe our experience with sofosbuvir-based treatment for HCV infection in a real-life cohort. METHODS: This was a prospective, nonrandomized and observational study at a tertiary care centre in Surat, India. The primary end-point was proportion of the study patients who achieved a sustained virological response 12 weeks after cessation of treatment (SVR 12). Secondary end-points of the study include SVR 4, virological relapse and appearance of adverse events. RESULTS: A total of 107 patients with chronic HCV who received sofosbuvir-based treatment were included in the study. During study period, two patients died due to severity of liver complications. Hence, overall rate of SVR 4 and SVR 12 was 98.1 % (n = 103/105) and 94.3 % (n = 99/105), respectively. Among 67 patients with HCV genotype-3 infection, the SVR 12 rate was 92.5 % (n = 62/67), and among 38 patients with HCV genotype-1 infection, the rate of SVR 12 was 97.4 % (n=37/38). A total of 32 (29.9 %) patients reported adverse events during the course of sofosbuvir-based treatment. None of the patient discontinued treatment due to adverse event. CONCLUSIONS: Sofosbuvir-based treatment is safe and efficacious in clinical practice in Indian patients with HCV genotype-1 and genotype-3 infection.

Patient education cards for skin cancer detection and treatment.

Over 1.3 million cases of skin cancer occur annually in the USA, but no uniform or evidence-based screening guidelines exist. During a 1-year intervention, a patient education tool was distributed to patients. Billing records were utilized to compare detection and treatment rates for cancerous and precancerous skin lesions during the intervention to a historical control. No significant change in detection rates was demonstrated (RR = 1.0, p = 0.88). However, the number of procedures performed for malignant and pre-malignant skin lesions increased significantly (RR = 2.4, p < 0.0001). The patient education tool created clinically significant behavioral change at a low monetary and time cost.