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INTRODUCTION: Congenital portosystemic shunts (CPSS) are rare vascular malformations that can lead to severe complications. With advanced imaging techniques, diagnosis is becoming more feasible occurring in fetal life. Different approaches have been adopted to manage these cases, with an increased utilization of interventional therapy recently. This cohort aims to describe the course of children diagnosed with CPSS and the impact of interventional therapy on the outcome. METHODS: Retrospective chart review was done for all patients who were diagnosed with CPSS in our institution between January 2006 and December 2015. RESULTS: Six patients were diagnosed with CPSS. During this period, 8,680 mothers carrying 9548 fetuses underwent fetal ultrasound examinations. Three patients were diagnosed antenatally at a median [IQ] gestational age of 33 [26-33] weeks, and three patients were diagnosed postnatally at 0, 2, and 43 months, respectively. At a median follow-up of 87 [74-110] months, 5 patients are alive; 4 of whom had received transcatheter closure for different indications, and one who had spontaneous resolution of her CPSS. One infant died at the age of 6 weeks secondary to sepsis. CONCLUSION: CPSS can result in significant complications in children. Interventional therapy is feasible at any age group, but long-term follow-up is warranted.
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OBJECTIVES: The use of growth hormone (GH) in idiopathic short stature (ISS) has been a subject of debate for the past two decades. We sought to assess the effect of GH on final height (FH) in patients with ISS in our region, which has a high consanguinity rate, and compare it to the effect observed in GH deficient (GHD) patients. METHODS: We conducted a retrospective chart review from 1 January 2005 to 31 December 2013 for patients with ISS or GHD from the local United Arab Emirates population who received GH treatment and were followed-up regularly in our clinic. The change in height Z-score at 12 months and FH were assessed within each group and between the two groups. RESULTS: Twenty-one patients with ISS and 29 patients with GHD were studied. There was a significant change in height Z-score at 12 months and FH in both groups (p < 0.001). The improvement in the ISS group was comparable to the response seen in GHD patients at 12 months (0.5±0.3 standard deviation score (SDS), and 0.5±0.4 SDS, respectively; p = 0.540). The effect on FH was better in ISS group than the GHD group of all etiologies (1.3±0.6 SDS vs. 0.9±0.7 SDS, respectively; p = 0.050), there was no difference between the ISS and the subgroup of idiopathic GHD (1.3±0.5 SDS and 1.2±0.8 SDS, respectively). CONCLUSIONS: In our local population, GH has a positive effect on the short-term growth and FH of children with ISS to the same extent that has been observed in children with idiopathic GH deficiency.
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Acute hemorrhagic edema of infancy is an uncommon benign cutaneous vasculitis. Despite its worrisome presentation, it carries good prognosis with rarely reported systemic involvement. Management of these cases has been an area of debate with majority of physicians adopting conservative modalities. We report a case that presented with classic triad of rash, low grade fever, and peripheral edema along with two rarely reported manifestations in literature: hemorrhagic lacrimation and epistaxis.
