Appropriateness of pediatric hospitalization in a general hospital in Kuwait

To determine the rate of inappropriate pediatric admissions using the Pediatric Appropriateness Evaluation Protocol [PAEP] and to examine variables associated with inappropriateness of admissions. A prospective study was conducted in the Department of Pediatrics, Farwania General Hospital, Kuwait, to examine successive admissions for appropriateness of admission as well as several sociodemographic characteristics over a 5-month period [August 2010 to December 2010]. A total of 1,022 admissions were included. Of the 1,022 admissions, 416 [40.7%] were considered inappropriate. Factors associated with a higher rate of inappropriate admission included older age of patients and self-referral. The rate of inappropriate hospitalization of children was high in Farwania Hospital, Kuwait, probably due to the relatively free health care services, parental preference for hospital care, easy access to hospital services, and insufficient education about the child's condition

Arthrogryposis, renal tubular dysfunction and cholestasis [ARC] syndrome: a case report

Arthrogryposis, renal tubular dysfunctions and chloestasis [ARC] syndrome is a rare multisystem, usually fatal, autosomal recessive disorder. Awareness of this syndrome is growing with more reported cases over the last three decades. Many previously reported cases with similar association are now labeled as ARC syndrome. Although the genetic mutation is recently recognized, the diagnosis still depends on the clinical findings. Our patient is the first case of ARC syndrome to be reported from Kuwait. To the best of our knowledge this is also the first report of an Egyptian family with two siblings of ARC syndrome

Fulminating shigella encephalopathy [Ekiri syndrome]: a case report

Neurological manifestation, particularly seizures and encephalopathy, are common in childhood shigellosis. Fulminating shigella encephalopathy [Ekiri syndrome] is a rare form of shigella associated encephalopathy characterized by a rapid, severe and fatal course with few dysenteric symptoms. Brain edema is a common finding in patients presenting with severe shigella encephalopathy. Shiga toxin production is not essential for development of shigella-associated neurological symptoms. Early recognition and proper management of cases of severe shigella encephalopathy may help to improve the outcome. We are reporting the case of a six and half year old male child with severe fulminating shigella-encephalopathy [Ekiri syndrome] who made a partial recovery. Brain magnetic resonance image [MRI] findings of this patient are reported. To the best of our knowledge, brain MRI studies were not reported before in the pediatric population with Ekiri syndrome; moreover, this is probably the first case of Ekiri syndrome to be reported in the Arab population

Wolman disease in an Egyptian patient

Lysosomal acid lipase deficiency leads to accumulation of cholesteryl esters and triglycerides in different body tissues. This disorder is manifested in two clinical forms; cholesteryl ester storage disease which is a benign adult form and Wolman disease [WD], a fatal autosomal recessive form. We present an Egyptian infant with WD whose diagnosis was based on clinical, laboratory and imaging features. This is the first reported patient with WD from Egypt