Adams–Oliver syndrome (AOS) is a rare congenital disorder with unknown etiology commonly presented with aplasia cutis and terminal limb defects. Central nervous and cardiopulmonary systems may also be affected. It is commonly inherited as an autosomal dominant disorder but autosomal recessive and sporadic cases have also been reported. Here, we present a 10-year-old boy with extensive aplasia cutis congenita and limb anomalies as well as mild pachygyria and focal acrania in neuroimaging. No other internal organ involvement was obvious in this patient. Family history was negative for this syndrome. AOS is a multisystem disorder, and so it is crucial to investigate for internal organ involvements.
Background: Hypertension has a growing trend all around the world among children. Evidences imply that inadequate sleep duration and its poor quality are related to hypertension. But there are only few studies to show this relationship in children. Objectives: The aim of this study was to investigate the correlation between sleep quality parameters and blood pressure (BP) changes in children. Patients and Methods: Eighty six patients aged 5-15 years old with the history of urinary tract infection were included in this study. They underwent 24-hour BP monitoring. In addition, the Pittsburg Sleep Quality index questionnaire was filled out and the data were compared with BP records. Results: After excluding duplicate cases and those with insufficient data, 76 children entered into study. Overall sleep quality was good in 48 and poor in 28 children. Mean diastolic BP load (P = 0.019), diastolic load awake-time (P = 0.045), mean systolic sleep-time (P = 0.022), non-dipper state (P = 0.009) were statistically different among groups. By dividing the children into two groups of good and poor sleeper, the parameters of BP were not different. In addition, there was no correlation between BP classifications and sleep latency, duration of sleep, sleep efficiency, sleep disturbance, day dysfunction due to sleepiness, and overall sleep quality score. Conclusions: Our study could not show any correlation between sleep quality and ambulatory BP monitoring parameters in children with abnormal BP.
Background: Seckel syndrome is a rare genetic disorder with autosomal recessive inheritance. It is characterized by dysmorphic features, intrauterine and postnatal growth restriction, microcephaly and mental retardation. Although cardiovascular complications are not prevalent in this syndrome, severe sinus bradycardia, hypertension and brain vasculopathy are reported. Here, for the first time, we describe a case of lower extremity arterial occlusion in a case of Seckel syndrome. Case Presentation: An 8-year-old girl with the characteristic features of Seckel syndrome was admitted to the children's hospital with the complaint of left lower extremity pain and a deep ulcer on her left shin. On examination, the left extremity was cooler than the other side, with a bluish color. Dorsalis pedis and popliteal artery pulses were not palpable on the left. A wound measuring 3 by 5 cm with gangrenous margins was visible on the anterior surface of the left leg. Severe stenosis in the left superficial femoral artery was reported on angiography. Considering the lean body of the patient, angioplasty was not possible and conservative wound care, analgesic drugs and aspirin were recommended. Conclusion: Clinicians should be suspicious of vascular complications in patients with Seckel syndrome, even in the absence of any other risk factors.
Background: Bystander cardiopulmonary resuscitation is an important predictor of out-of-hospital arrest prognosis in the pediatric population. The purpose of this study was to assess the effectiveness of two educational methods, a video module and Peyton model with manikin, in parent's education. Materials and Methods: We enrolled 140 subjects, 70 in each group. We assess their knowledge, attitude, and practice about pediatric basic life support (BLS) before and after two different types of educational interventions. Results: Mean attitude, knowledge, and practice score were significantly higher after educational intervention in both groups. Knowledge and total practice score were significantly higher in the Peyton group compared with DVD group ( P = 0.02 and 0.000, respectively). The rate of totally correct chest compression was 53% in Peyton/manikin group versus 24% in DVD/lecture group and the difference was meaningful statistically ( P = 0.0003). Conclusion: Any educational intervention has a significant effect on the Iranian parent's knowledge and practice about child BLS, but education through manikin can increase this impact.
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