ABSTRACT
Patent ductus arteriosus [PDA] is an extremely common occurrence in very premature infants. Untreated symptomatic PDA may be associated with chronic lung disease. PDA has a major role in neonatal mortality and morbidity. We compared the efficacy and safety of oral versus intravenous ibuprofen for the pharmacological closure of PDA in low birth weight [LBW] preterm infants. A randomized, single-blinded, controlled study was performed on premature neonates at the neonatal unit, University Hospital for Obstetrics and Gynecology "Koço Gliozheni", Tirana, Albania from January 2010 to December 2012. The study enrolled 68 preterm infants with a confirmed and significant PDA. The preterm infants received either intravenous or oral ibuprofen randomly as an initial dose of 10 mg/kg, followed by 5 mg/kg at 24 and 48 h. 36 patients were treated with oral ibuprofen and 32 with intravenous ibuprofen during this period. After the first course of the treatment, the PDA closed in 30 [83.3%] of the patients assigned to the oral ibuprofen group versus 23 [71.8%] of those enrolled in the intravenous ibuprofen group [P=0.355]. 15 patiens needed a second treatment course and they all [100%] had clinical signs of infection and positive blood culture. There was no reopening of the ductus after the closure. Our data indicate that, for LBW infants, the rate of early ductal closure was comparable and the adverse effects were fewer with oral ibuprofen in comparison to the intravenous route. Association of PDA with perinatal infection has a negative impact in pharmacological closure of the ductus, increasing the need for a second course of treatment or for surgery
ABSTRACT
Gaucher's disease is a rare genetic disorder that results in the accumulation of cerebrosides in the liver, spleen, kidneys, lungs, brain and bone marrow. The deficiency of the specific lysosomal enzyme glucocerebrosidase is considered as causative factor. The first effective treatment for the disease, the drug Ceredase, approved in 1995, was replaced in 2001 by the drug Cerezyme. During the period 2004-2009 in our service 11 children were hospitalized and treated for Gaucher's disease: 9 children with type 1, and 2 children with type 3 of the disease. The enzymatic examinations of the biomarker chitotriosidase were performed in Sahlgren's University Hospital, Molndal Sweden; the DNA analysis was performed in the Children's Hospital and Regional Medical Center, Seattle, USA. We are presenting the biological and genetic molecular data of the children. In our case series, one year after the treatment started, the hemoglobin level was normalized; the platelet count was normalized in 7 patients after one year of treatment, and in 9 patients after two years of treatment. The hemorrhagic syndrome stopped after 6 months of treatment. Chitotriosidase values decreased 10-20 times the initial value, after one year of treatment and in one case the value reached the normal range. The treatment with Cerezyme has also improved the visceral and biological signs. Anomalies of the oculomotricity were less sensitive to the treatment. According to our experience, Chitotriosidase is a sensitive and specific marker in diagnosing and monitoring Gaucher's disease. The enzyme replacement therapy through Cerezyme is an effective and safe treatment of Gaucher's disease. Blood signs [anemia, platelet count]; visceral signs [splenomegaly, hepatomegaly] as well as bone involvement showed decisive improvement under the therapy
Subject(s)
Humans , Male , Female , Gaucher Disease/therapy , Child , Cerebrosides , Glucosylceramidase , Enzyme Replacement TherapyABSTRACT
The goal of this study was to investigate all the potential routes to Child/Adolescent Psychiatry Clinic-University Hospital Center [CAPC-UHC] in Tirana. The article provides demographic data, as well as further information on the types and amounts of services children/adolescents received during the process of seeking help related to different diagnoses. The study was conducted in CAPC-UHC in Tirana, during September 2006-September 2007. Data were collected from 162 children and their parents using Strengths and Difficulties Questionnaire [SDQ] and Pathways Encounter Form. The sample consisted of 53.1% [86] males and 46.9% [76] females. The mean age was 9.5 + 4.4 years. Out of the total number of cases that sought care to CAPC; 55, 6% were referred by parents themselves, while the rest were referred by others. There was a significant effect of gender to intervals from the onset of problem to the first career [F = 10.803, p=0.001], as well as a significant effect of gender to total time intervals from the onset till the specialist of child mental health problem [F = 6.742, p=0.01]. This is the first study investigating the help seeking process to psychiatric care in CAPC Tirana-Albania and may serve as a good start in generating evidence based on child/adolescent mental health service. Further multicentre studies will enhance the values of the findings, since the present study was performed in a single service, and in a setting lacking previous works with similar scope that could have served as references