Osteogenesis imperfecta type V: A repost of two Filipino families and review of literature

Osteogenesis imperfecta (Ol) type V is distinct Ol phenotype that has recently been described. Patients with this phenotype present characteristically with interosseous membrane calcification and hyperplastic callus formation. We present the clinical and radiographic characteristics of two Filipino families diagnosed to have Ol type V. Through this review of cases, we aim to educate healthcare providers by highlighting salient clinical and radiographic features to aid in the recognition of this specific Ol phenotype, difficulties in diagnosis, current practices in management and fracture prevention, and issues in genetic counseling.

The clinical outcome and antibiotic sensitivity pattern of Enterobacter spp. culture positive neonates admitted at Cebu Doctors' University Hospital - Neonatal Intensive Care Unit (2005-2008)

@#Objectives: This aim of this research is to determine the clinical outcome of neonates with Enterobacter spp. positive blood culture and the antibiotic sensitivity pattern of these isolates at Cebu Doctors' University Hospital Neonatal Intensive Care Unit. Methods: This descriptive, cross-sectional, retrospective study retrieved the list of neonates admitted at Cebu Doctors' Hospital - Neonatal Intensive Care Unit (CDUH-NICU) from January 2005 to December 2008 whose bloods were taken for culture. The antibiotic sensitivity patterns of the Enterobacter positive cases were reviewed, along with broad-spectrum cephalosporin (BSC) resistant and multiresistant Enterobacter spp. (MRE) determination and clinical outcome. The relationship between outcome and MRE was analyzed using the Pearson Chi-square test. Conclusion: Most isolates were BSC-resistant while 24% were MRE. Enterobacter was most sensitive to Imipenem, Meropenem, Cefepime, and Piperacillin-Tazobactam.

Intravenous pamidronate treatment in Filipino children with moderate to severe osteogenesis imperfecta

Objective. To present preliminary data on the effects of intravenous pamidronate in children with moderate to severe Osteogenesis Imperfecta (OI). Methods. This is a restrospective study wherein a review of medical records and available serial radiographs of children (N=14) with moderate to severe IO started on pamidronate from 2006 to 2010 was done. Results. Two children have IO Type I, 8 have IO Type III and 4 have IO Type IV. At baseline, 2 had normal height, 8 had height less than minus 2SD and the rest with less than minus 1SD. Twelve out of 14 had vertebral compression fractures. Mean age at start of pamidronate was 5.4 years (range 0.5-11 years). First infusion fever in five patients and transient generalized macular rash in one child were noted. Serum calcium and phosphorus levels were normal at baseline and remained stable. Based on parental report, improvement of motor function was noted. In the 10 children who had at least a year of treatment, long bone fractures decreased from mean annualized fracture rate of 2.6 at baseline to 0.9. In patients with vertebral compression fractures, serial radiographs showed improvement of vertebral shape. Conclusion. This preliminary study shows that treatment was generally well tolerated and led to decrease in long bone fractures, improved vertebral shape and improved function.

Establishment of the Philippine birth defects surveillance

Congenital anomalies have been in the top ten causes of infant mortality in the Philippines for the past 50 years. Since there is no synthetic surveillance for the congenital anomalies in our country, there are no specific programs directed toward better understanding of this group of patients. The birth defects surveillance project was convinced in 008 to develop a system of reporting of newborns with birth defects at the hospital and community setting. The lead agencies for this project are the University of the Philippines Manila-National Institutes of Health and the department of Health (DOH). This paper describes the establishment of the Philippine Birth Defects Surveillance.