Effect of growth hormone on final height in children with idiopathic short stature: a UAE, Eastern region experience

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

Neurally adjusted ventilatory assist [NAVA] mode as an adjunct diagnostic tool in congenital central hypoventilation syndrome

A full term female newborn was admitted to the neonatal intensive care unit [NICU] for continuous observation of apnea. Infant was noted to have apnea while asleep requiring intubation and mechanical ventilation. A video EEG was performed which demonstrated normal awake background without any seizure activity. Neurally adjusted ventilatory assist [NAVA] demonstrated the absence of electrical activity of the diaphragm [Edi] when the patient was in quiet phase of sleep. This finding on NAVA monitor raised the suspicion of central hypoventilation syndrome [CCHS] which was confirmed by genetic identification of the PHOX2B mutation

Congenital epulis

Epulis is a rare tumour, with female preponderance that is only seen in the newborns. It arises from the mucosa of the gingiva and protrudes out of the infant's mouth. It can potentially obstruct the airways and may require an EXIT [ex-utero intrapartum treatment] procedure which involves establishing an airway before the feto-maternal circulation is interrupted. We present a female newborn with such a mass, which was diagnosed antenatally. A multidisciplinary team including the neonatologist, anaesthesiologist and ENT specialist should be present in the delivery room to establish the airways, which may require an EXIT procedure. Recommended treatment is early surgical resection. Recurrences of the tumour and damage to future dentition have not been reported, suggesting that radical excision is not warranted

Neurally adjusted ventilatory assist mode used in congenital diaphragmatic hernia

A term baby with congenital diaphragmatic hernia [CDH] underwent surgical repair on the second day of life. Postoperatively; the oxygenation index increased to 85 despite high pressure ventilation with HFOV [high frequency oscillator ventilation] and inhaled nitric oxide therapy. Oxygenation index above 70 carries a mortality rate of 94% and merits starting extracorporeal membrane oxygenation [not available in the UAE]. A trial of neurally adjusted ventilatory assist [NAVA] on the 10th postoperative day was followed by a reduction of oxygenation index to 15 and marked improvement of the clinical parameters. The EAdi [electrical activity of diaphragm] signal was relatively weak [ +/- 5 micro volt] requiring augmentation witha high NAVA level [3 - 3.5]. The patient was successfully extubated after 3 weeks

Analysis of prognostic factors affecting response to treatment and survival in chronic myeloid leukemia patients

To evaluate the prognostic significance of the disease features at presentation in chronic myeloid leukemia [CML] patients. This is a retrospective study of sixty patients of CML and their follow up over 20 years. Ten clinical and laboratory features of the disease were evaluated for their prognostic significance. All patients received cytoreductive therapy. All patients were seen at King Abdul Aziz University Hospital and the National Guard Hospital in Jeddah, Saudi Arabia. The prognostic value of sex, age, white cell, basophils, promyelocytes and platelet counts, splenomegaly, bone marrow [BM] blast count, BM fibrosis and the presence of the Philadelphia chromosome, was assessed using log-rank tests. All variables significantly associated with survival univariately were included in a Cox regression. The time to either death or transformation from date of diagnosis was analyzed using a Kaplan-Meier survival curve. Splenomegaly greater than or equal to 13 cm and a bone marrow blast count of greater than 10% at diagnosis, were both found to be significantly associated with a high-risk of transformation or death in this population. Other presenting features studied, did not have a statistically-significant prognostic impact. The median duration of the chronic phase in the studied group of patients was 120 months. Splenomegaly and a high BM blast count were both associated with a risk of transformation or death in this population

Universal screening strategy for gestational diabetes mellitus: the experience of Tawam Hospital

To estimate the prevalence of gestational diabetes mellitus [GDM] and impaired glucose tolerance test [IGTT] in an Emirate population, study the incidence of various risk factors among patients with GDM and IGTT and evaluate the effectiveness of the universal screening for GDM in comparison with selective screening based on risks factors. We reviewed the data of all pregnant women who were diagnosed to have GDM or IGTT and delivered at Tawam Hospital, United Arab Emirates, between January 1 and December 31, 2005. Risk factors for GDM were reported as: [I] family history of diabetes mellitus [DM] in parents or siblings, [2] GDM or IGTT in previous pregnancy, [3] pre-pregnancy body mass index of >27 kg/m2, [4] previous delivery of macrosomic baby, [5] glucosuria, [6] previous unexplained intrauterine foetal death, [7] current polyhydraminos. The proportion of women without any risk factors was measured to assess the proportion of GDM patients who could be missed by selective screening. Out of 3650 women delivered during that period 268 had GDM and 44 had IGTT. Considering that only 82.3% of our pregnant women underwent screening for GDM, the prevalence was 8.9% [SE 0.5] for GDM and 1.4% [SE 1.2] for IGTT. Pre-pregnancy BMI > 27kg/ m2, family history of DM and previous GDM or IGTT were the commonest risk factors in the study subjects. Selective screening could miss 123% of women with GDM and 18.2% with IGTT. 15.2% of those with GDM needed insulin therapy. In conclusion the prevalence of GDM and IGTT is 8.9% [SE 0.5] and 1.4% [SE 1.2] respectively, obesity, family history previous GDM or IGTT were the commonest risk factors. Universal screening should be favored over selective screening for detection of gestational diabetes mellitus In such a high-risk population