Type I retinopathy of prematurity in infants with birth weight less than 1251 g: incidence and risk factors for its development in a nursery in Kuwait

To report the rate of acute retinopathy of prematurity [ROP] and Type I ROP among infants with birth weight [BW] <1251 g and identify the risk factors for the development of Type I ROP. A retrospective review of ROP records of infants with BW <1251 g was performed to identify infants with acute ROP and Type I ROP. Infants with Type I ROP were compared with those without Type I ROP to assess the risk factors for the development of Type I ROP. P < 0.05 was statistically significant. Multivariate analysis was performed and odds ratio [OR] and 95% confidence intervals [CI] were calculated. Among the 207 infants with BW <1251 g, acute ROP occurred in 154 infants [74.4%] and Type I ROP in 95 eyes of 50 infants [24.4%]. The numbers of infants with BW <750 g and BW <1000 g were 19.3% and 58.4%, respectively, and the incidences of Type I ROP were 50% and 36.4%, respectively, among them. Forty-four [46.3%] eyes were treated at stage 2+ ROP in zone I or II. All the eyes treated for Type I ROP showed complete regression. Gestational age at birth [OR 0.657, 95% CI: 0.521-0.827; P< 0.000l] and number of ventilated days [OR 1.017, 95% CI: 1.005-1.029; P= 0.006] were identified as independent risk factors for the development of Type I ROP. The rate of Type I ROP in this study is higher than that in previous studies due to the higher number of infants with BW <1000 g in our cohort and the treatment of more eyes with stage 2+ ROP. However, all the treated eyes had a favorable outcome. Gestational age at birth and number of ventilated days were independent risk factors for the development of Type I ROP

Reticular dysgenesis: a very rare and fatal immunodeficiency disease

Human severe combined immunodeficiency [SCID] represents a group of rare, often fatal, congenital disorders characterized by little or no immune response. Reticular dysgenesis [RD] is the most severe form of inborn SCID. Until recently, its genetic basis was unknown. It is only in 2008, that a gene defect has been described. We present here, a unique hematological phenotype of RD with a dramatic clinical course. The paucity of such cases in the literature, merits this report

Fatty acid oxidation in Kuwait: a case series and review

Fatty acid oxidation disorders [FAOD] are a group of inherited Inborn Error of Metabolism [IEM] in which specific enzyme defect in fatty acid metabolic pathway will lead to accumulation of fatty acids and decrease in cell energy metabolism. This paper will highlight the clues that can be obtained from history, clinical examination, and simple bedside tests characteristic for fatty acid oxidation disorders [fig 1] in order to raise the index of suspicion of these disorders among all pediatricians working in a society with high consanguineous marriage rate, such as Kuwait. The records of 15 patients diagnosed as FAOD were retrospectively reviewed. The final diagnosis was very long chain acyl-CoA dehydrogenase deficiency [VLCAD] in ten patients and long chain 3-hydroxyacyl-CoA dehydrogenase deficiency [LCHAD] in five patients. Eighty percent had a positive family history of either a previous sudden unexplained infant death or a similar diagnosis in a first-degree relative. History of consanguinity was positive in eleven patients [73%]. Diagnosis of FAOD can be difficult because patients tend to develop symptoms only during prolonged fasting and intercurrent acute illnesses. In our series seven patients presented with acute illness and four patients presented with respiratory distress, four had heart failure due to cardiomyopathy and another two presented with convulsions due to severe metabolic acidosis. Physical findings are usually non-specific, eight [53%] had hepatomegaly and four [27%] had marked hypotonia. Our group of 15 cases accumulated within 3 years is inordinately large and suggest that Kuwait provides a promising venue in which to study the biochemical and molecular genetics of FAOD

newborn requiring selective left bronchial intubation Case report

Pulmonary interstitial emphysema [PIE] is an iatrogenic pulmonary condition of the premature infant with immature lungs. PIE occurs almost exclusively with mechanical ventilation. We report a case of preterm newborn 26 weeks gestation who developed PIE in the right lung while on advanced nasal CPAP ventilation mode and managed by selective left bronchial intubation and mechanical ventilation