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  • ISSN: 2373-9312
    Volume 4, Issue 3
    Case Series
    Solmaz Celebi*, Sefika Elmas Bozdemir, Mustafa Hacimustafaoglu, and Ozgur Taskapilioglu
    Intracranial hydatid disease is rare, with reported incidence of 1-2% of all cases with hydatid disease and most of these cases present in childhood. In this report, we describe four children with primary hydatid disease of the brain. All four patients were diagnosed with cranial Magnetic Resonance Imaging and in direct hemagglutination test for Echinococcus granulosus. Of these four children, three had cysts in cerebral localization and one in cerebellar localization. Two children had multiple and one of them had recurrent cerebral hydatid disease. All patients received albendazole treatment. While three patients did well after surgical excision, a ventriculo peritoneal shunt was placed in one. Also, this child was operated for duramater defect. This report shows that primary hydatid disease of brain is still a difficult problem despite all advances in diagnostic methods and surgical techniques.
    Case Report
    Mohamed Abdellatif*, Asad Ur Rahman, Mariam Mathew, Nihal Al Riyami, and Khalid Althily
    The frequency of NIHF (Non-immunological hydrops fetalis) and congenital ascites associated with LSD (Lysosomal storage disorders) is exceptionally rare. The reported incidence worldwide is about 1% with very limited reports from the MENA (Middle East and North Africa) region despite high incidence of consanguinity in the area. We report a case of NIHF with subcutaneous edema and congenital ascites as the main presenting sign which was diagnosed antenatally; subsequently LSD was diagnosed by enzymatic studies of amniotic fluid cultured cells after ruling out the most common conditions associated with fetal hydrops. A baby girl was delivered by spontaneous vaginal delivery at 33 weeks of gestation with generalized hydrops noted on physical examination. Diagnostic work up including lysosomal enzymes study confirmed the diagnosis of GM1 ganglisidosis. MRI (Magnetic resonance imaging) of the brain confirmed focal areas of hyper intensity with perivasculardistribution consistent with dilated VR (Virchow Robin) spaces. LSD are serious and genetic conditions with poor outcome so efforts should be made to institute the best screening strategy in the MENA region because of the high rate of intermarriages.LSD should be considered in the diagnosis of NIHF which is essential for genetic counseling as there is a high chance of recurrence considering the high degree of consanguinity in the region.
    Nikhil Sonthalia*, Jain SS, Pawar VB, Zanwar VG, Surude RG, Rathi PM, Munde KK, and Sandeep Bavdekar
    We describe a case of two-year-old boy presenting with debilitating pruritus, patchy alopecia and jaundice since the age of 6 months. On evaluation he had intrahepatic cholestasis with persistently raised serum alkaline phosphatase, normal Gamma glutamyl transferase and raised serum bile acid levels. His liver biopsy showed bland cholestasis and electron microscopy showed granular bile suggestive of Progressive familial intrahepatic cholestasis (PFIC) type I. Medical therapy with ursodeoxycholic acid, cholestyramine, and rifampicin with nutritional modification was successful in alleviating the symptoms and correcting the nutritional status. At present his liver function had stabilized at 1 year follow up and there is no need for liver transplant in him. To our knowledge this is only the twelfth case of Progressive familial intrahepatic cholestasis type reported from India. Successful medical therapy of PFIC has been rarely described in tropical countries including India. Most of the data in the literature describe poor response to medical treatment alone and need for surgical biliary diversion and liver transplant in these patients. Liver transplant has guarded prognosis in patients with PFIC I if extrahepatic manifestations are present at the onset. Recurrence of PFIC after a successful Liver transplant is a possibility due to all immunization of recipient against the effected protein. Our experience suggests that if instituted early medical therapy alone may obviate the need for liver transplant in a subset of patients with PFIC. Further molecular testing is required to identify this sub-group of patients with favorable prognosis.
    Shannon N. Foster*, Vincent Mysliwiec, Emerson Wickwire, and Matthew S. Brock
    Nighttime use of electronic media (EM) including smartphones and tablets is reported to have detrimental effects on sleep. Survey-based studies demonstrate that EM use near bedtime and in bed is associated with shortened sleep duration and insomnia in adults and children. This report describes the case of a 3-year old boy who repeatedly used a smartphone while undergoing an attended in-lab polysomnogram (PSG). The child’s PSG results provide objective evidence supporting the adverse effects of EM on sleep quality and suggest a potential pathway for development of chronic insomnia. Providers should ask about EM use in young children with complaints of insomnia or restless sleep.
    Mini Review
    Ninh Doan*, Ha S. Nguyen, Mohit Patel, Saman Shabani, Karl Janich, and Andrew Montoure
    Gunshot wound to the head (GSWH) has a mortality rate of 20-90% in adults and 20-65% in the pediatric population. Due to the high rates of mortality and morbidity, the management of these patients remains a topic of high interest. Here, we present the current data on and management of GSWH in the pediatric population. The St. Louis scale for pediatric GSWH can be utilized to stratify risks and guide clinical decisions. However, it is important to recognize that pediatric brains may still have the potential for neurological plasticity and can still benefit from aggressive measures despite severe GSWH, especially in patients with bifrontal GSWH injuries.
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