Wilson's disease among children at King Hussein Medical Center

To describe the demographic characteristics and clinical presentation of 37 patients with Wilson's disease followed up at the Pediatric Gastroenterology Clinic. A specially designed data collection form was used to collect the relevant data; Medical history and a thorough clinical examination for patients who were diagnosed with Wilson's disease during the period between February 2000 and October 2010 at King Hussein Medical Center, Amman, Jordan was done. Laboratory investigations include ceruloplasmin level, liver enzymes, albumin, prothrombin time, partial thromboplastin time, international normalized ratio, complete blood count, urine analysis, abdominal ultrasound and liver biopsy. Simple descriptive statistics [frequency and percentage] were used to describe the study variables. A total of 37 patients diagnosed as Wilson's disease with age ranges between two and 13.5 years were included in this descriptive review. Out of 37 patients, 19 [51%] were males and 18 [49%] were females. Patients with affected siblings were 29 [78%]. Central nervous system involvement was found among 9 [24.3%] patients. The commonest presenting symptoms were jaundice [n=16, 43%], abdominal distension [n=13, 35%], fatigue and delayed school performance [n=12, 32.4%]. The most common clinical findings were hepatomegaly [n=26, 70%], jaundice [n=16, 43%], splenomegaly [n=14, 37.8%], Kayser-Fleischer ring [n=11, 29.7%], and lower limb edema [n=11, 29.7%] respectively. Low ceruloplasmin level was found in 34 [92%] patients, high liver enzymes in 23 [62%] patients, hemolytic anemia in 13 [35%] patients successively. Twenty-four hour urine collection average copper post D-penicillamine challenging test was above 230 micro g/dl. The most common ultrasound findings were hepatomegaly, abnormal echogenecity, splenomegaly and ascitis. Liver biopsies commonly showed liver fibrosis, however fatty liver changes, hepatosteatosis and liver cirrhosis were the least common finding. Family screening is needed once a child in the family is diagnosed. Full investigations to rule out Wilson's disease should be performed in any patient with unexplained elevation of liver enzymes, hepatomegaly, hemolytic anemia, jaundice or neurological/behavioral disturbances

Refractive errors among school children in Aqaba / Jordan

To assess the refractive errors among school children and to evaluate their eye condition, particularly visual acuity. This is a prospective survey that has been performed in Aqaba, at Princess Haya Hospital, eye clinic A total of 194 children were included: 166 came from public schools with an age range between [6-14] years. The rest were chosen from kindergarten schools with age range between [4-5] years. The period of the survey extended from August 2000 to February 2001 [one semester]. Only those with correctable refractive errors as a cause of low vision were included. More than half of the students [56%] noticed for the first time to have a refractive error were already above the age of 10 years. The highest percentage included those who had difficulty in seeing the blackboard in the classroom, followed by those watching television from a close distance. Only a quarter of these children were referred to the ophthalmologist by the school physician after routine medical checkup. Hypermetropia was more common among children 4-5 years. Myopia was noticed to be more common after the age of 6 years. This survey showed that there was a delay in presentation to ophthalmologists for refractive error correction, as well as lack of a routine ophthalmic school check -up, which is usually delayed till the fourth grade. We recommend yearly vision check to be started at the age of four years to prevent significant consequences of high refractive errors, like amblyopia, and to achieve a better school performance. A campaign for parental education for early signs of low vision detection is vital