Hard X-Ray flux upper limits of central compact objects in supernova remnants

We searched for hard X-ray (20–300 keV) emission from nine central compact objects (CCOs) 1E 1207.4−5209, 1WGA J1713−3949, J082157.5−430017, J085201.4−461753, J1601−5133, J1613483−5055, J181852.0−150213, J185238.6+004020, and J232327.9+584843 with the INTEGRAL observatory. We applied spectral imaging analysis and did not detect any of the sources with luminosity upper limits in the range of 1033-1034 ergs/s in the 20-75 keV band. For nearby CCOs (< 4 kpc) the upper limit luminosities are an order of magnitude lower than the measured persistent hard X-ray luminosities of AXPs. This may indicate that the central compact objects are low magnetic field systems with fallback disks around them

Hard X-Ray flux upper limits of central compact objects in supernova remnants

We searched for hard X-ray (20–300 keV) emission from nine central compact objects (CCOs) 1E 1207.4−5209, 1WGA J1713−3949, J082157.5−430017, J085201.4−461753, J1601−5133, J1613483−5055, J181852.0−150213, J185238.6+004020, and J232327.9+584843 with the INTEGRAL observatory. We applied spectral imaging analysis and did not detect any of the sources with luminosity upper limits in the range of 1033-1034 ergs/s in the 20-75 keV band. For nearby CCOs (< 4 kpc) the upper limit luminosities are an order of magnitude lower than the measured persistent hard X-ray luminosities of AXPs. This may indicate that the central compact objects are low magnetic field systems with fallback disks around them

Comparative effectiveness of drugs used to constrict the patent ductus arteriosus: a secondary analysis of the PDA-TOLERATE trial (NCT01958320).

ObjectiveTo evaluate the effectiveness of drugs used to constrict patent ductus arteriosus (PDA) in newborns &lt; 28 weeks.MethodsWe performed a secondary analysis of the multi-center PDA-TOLERATE trial (NCT01958320). Infants with moderate-to-large PDAs were randomized 1:1 at 8.1 ± 2.1 days to either Drug treatment (n = 104) or Conservative management (n = 98). Drug treatments were assigned by center rather than within center (acetaminophen: 5 centers, 27 infants; ibuprofen: 7 centers, 38 infants; indomethacin: 7 centers, 39 infants).ResultsIndomethacin produced the greatest constriction (compared with spontaneous constriction during Conservative management): RR (95% CI) = 3.21 (2.05-5.01)), followed by ibuprofen = 2.03 (1.05-3.91), and acetaminophen = 1.33 (0.55-3.24). The initial rate of acetaminophen-induced constriction was 27%. Infants with persistent moderate-to-large PDA after acetaminophen were treated with indomethacin. The final rate of constriction after acetaminophen ± indomethacin was 60% (similar to the rate in infants receiving indomethacin-alone (62%)).ConclusionIndomethacin was more effective than acetaminophen in producing ductus constriction

A Patient with Proopiomelanocortin Deficiency: An Increasingly Important Diagnosis to Make

Proopiomelanocortin (POMC) deficiency is a rare monogenic disorder with early-onset obesity. Investigation of this entity have increased our insight into the important role of the leptin-melanocortin pathway in energy balance. Here, we present a patient with POMC deficiency due to a homozygous c.206delC mutation in the POMC gene. We discuss the pathogenesis of this condition with emphasis on the crosstalk between hypothalamic and peripheral signals in the development of obesity and the POMC-melanocortin 4 receptors system as a target for therapeutic intervention

A national survey on use of less invasive surfactant administration in Turkey

Background. The aim of the study was to assess the rate of utilization, policy of premedication, technique, equipment, experience on safety and efficacy for less invasive surfactant administration or minimally invasive surfactant therapy (LISA/MIST) use in Turkey. Methods. An online survey was designed and distributed via Google Forms tool to 350 neonatologists from 173 units through NICU-Turk mailing list of the Turkish Neonatal Society. Participants were asked to answer the survey for their own neonatal intensive care unit (NICU). Results. LISA/MIST use rate was 81.6% among 87 NICUs which responded (response rate was 50.2%). LISA was used regularly in 23 of the units (26.4%), occasionally in 35 (40.2%), rarely in 12 (13.8%), and only for clinical trials in 1 (1.1%). LISA/MIST has been never applied in 16 units (18.4%). Conclusions. LISA/MIST is widely used in Turkey similar to several regions in Europe but unlike the USA. Future studies are expected to further clarify some questions about LISA/MIST procedure, especially on its efficacy and safety

Rare Types of Congenital Adrenal Hyperplasias Other Than 21-hydroxylase Deficiency

Although the most common cause of congenital adrenal hyperplasia (CAH) worldwide is 21-hydroxylase deficiency (21-OHD), which accounts for more than 95% of cases, other rare causes of CAH such as 11-beta-hydroxylase deficiency (11&#946;-OHD), 3-beta-hydroxy steroid dehydrogenase (3&#946;-HSD) deficiency, 17-hydroxylase deficiency and lipoid CAH (LCAH) may also be encountered in clinical practice. 11&#946;-OHD is the most common type of CAH after 21-OHD, and CYP11B1 deficiency in adrenal steroidogenesis causes the inability to produce cortisol and aldosterone and the excessive production of adrenal androgens. Although the clinical and laboratory features are similar to 21-OHD, findings of mineralocorticoid deficiency are not observed. 3&#946;-HSD deficiency, with an incidence of less than 1/1,000,000 live births, is characterized by impairment of both adrenal and gonadal steroid biosynthesis very early in life, with inadequate virilization in boys and varying degrees of virilization in girls. It may present with salt wasting crisis or delayed puberty in both genders. While 46,XY disorders of sex development is frequently observed in boys with 17-hydroxylase deficiency, immature pubertal development and primary amenorrhea are observed in girls due to estrogen deficiency throughout adolescence. Patients with LCAH, which develops due to steroidogenic acute regulatory protein deficiency, typically present with salt wasting in the first year of life. It is characterized by complete or near-complete deficiency of adrenal and gonadal steroid hormones and progressive accumulation of cholesterol esters in the adrenal gland

Efficacy and safety of Saccharomyces boulardii in amebiasis-associated diarrhea in children

The efficacy and safety of adding Saccharomyces boulardii to antibiotic treatment for amebiasis-associated acute diarrhea in children were assessed in this study. Forty-five children in Group I received only metronidazole per oral for 10 days while 40 patients in Group II received S. boulardii in addition to the same medication. The major outcomes investigated were duration of acute and bloody diarrhea, frequency and consistency of stools, resolution time of the symptoms, and the tolerance and side effects of the treatment regimens. The median duration of acute diarrhea was 5 (1-10) days in Group I and 4.5 (1-10) days in Group II (p=0.965). The median number of stools on follow-up and duration of bloody diarrhea, fever, abdominal pain and vomiting were similar in the two groups. S. boulardii was well tolerated by the children and no side effects were recorded. Addition of S. boulardii to antibiotic treatment of amebiasis-associated acute diarrhea in children does not seem to be more effective than metronidazole treatment alone

Two Siblings with Isolated GH Deficiency Due to Loss−of−Function Mutation in the GHRHR Gene: Successful Treatment with Growth Hormone Despite Late Admission and Severe Growth Retardation

Patients with growth hormone releasing hormone receptor (GHRHR) mutations exhibit pronounced dwarfism and are phenotypically and biochemically indistinguishable from other forms of isolated growth hormone deficiency (IGHD). We presented here two siblings with clinical findings of IGHD due to a nonsense mutation in the GHRHR gene who reached their target height in spite of late GH treatment. Two female siblings were admitted to our clinic with severe short stature at the age of 13.8 (patient 1) and 14.8 years (patient 2). On admission, height in patient 1 was 107 cm (−8.6 SD) and 117 cm (−6.7 SD) in patient 2. Bone age was delayed in both patients (6 years and 9 years). Clinical and biochemical analyses revealed a diagnosis of complete IGHD (peak GH levels on stimulation test was 0.06 ng/mL in patient 1 and 0.16 ng/mL in patient 2). Patients were given recombinant human GH treatment. Genetic analysis of the GH and GHRHR genes revealed that both patientscarried the GHRHR gene mutation p.Glu72X (c.214 G>T) in exon 3 in homozygous (or hemizygous) state. After seven years of GH treatment, the patients reached a final height appropriate for their target height. Final height was 151 cm (−1.5 SD) in patient 1 and 153 cm (−1.2 SD) in patient 2. In conclusion, genetic analysis is indicated in IGHD patients with severe growth failure and a positive family history. In spite of the very late diagnosis in these two patients who presented with severe growth deficit due to homozygous loss−of−function mutations in GHRHR, their final heights reached the target height

Low hemoglobin A1c levels in a patient with diabetic ketoacidosis: Fulminant type 1 diabetes mellitus

Fulminant type 1 diabetes mellitus (FT1DM) is a clinical condition that is characterized by remarkably rapid and complete pancreatic β-cell destruction, rapid onset of hyperglycemic symptoms followed by ketoacidosis. In most cases this process takes a few days. Although rare, there have been clinical manifestations with a prolonged progress that lasts longer than one week. This study focused on the case of a 35-monthold boy who was referred to our clinic with the diagnosis of diabetic ketoacidosis, and later had a modest elevation in hemoglobin A1c (HbA1c) levels (6.7 %) incompatible with his significantly elevated blood glucose levels. The autoantibodies against pancreatic β-cells were negative. On the basis of these above mentioned findings, our patient was then diagnosed with fulminant type 1 diabetes mellitus. If patients with diabetic ketoacidosis have no elevation in HbA1c levels, they should be assessed for possible clinical factors that can lead to lower detectable levels of HbA1c. Furthermore, FT1DM which is characterized by very rapid and potentially fatal progression should be considered as a differential diagnosis in these patients