The Effectiveness of Sirolimus Treatment in Two Rare Disorders with Nonketotic Hypoinsulinemic Hypoglycemia: The Role of mTOR Pathway

Nonketotic-hypoinsulinemic hypoglycemia (NkHH) is a very rare problem charcterized by increase in glucose consumption without hyperinsulinism. This disorder has mainly been reported in cases with AKT2 mutation and rarely in cases with PTEN mutation. In cases with PTEN or AKT2 mutation, there is no effective therapy other than frequent feeding to counter hypoglycemia. The mammalian target of rapamicin (mTOR) inhibitor, sirolimus, has been used in hyperinsulinemic hypoglycemia that was unresponsive to other medical treatment. In the insulin signaling pathway, both AKT2 and PTEN function upstream of mTOR. However, the role of Sirolimus on hypoglycemia in AKT2 and PTEN mutations is unknown. Case 1: Six month-old female with AKT2 mutation [c.49G>A (p.E17K)] and evidence of NkHH. Frequent feeding was unsuccesful in correcting hypoglycemia and her proptosis continued to worsen. Sirolimus treatment was started at three years of age. Subsequently, blood glucose (BG) levels increased to normal levels. Case 2: In a male with PTEN mutation (p.G132V (c.395G>T), persistent NkHH started at 16 years of age (fasting BG: 27 mg/dL, fasting insulin 1.5 mmol/L, while ketone negative). Sirolimus treatment was started and hypoglycemia was succesfully controlled. NkHH is a very rare and serious disorder which is challenging, both for diagnosis and treatment. Additionally, AKT2 and PTEN mutations may result in NkHH. Sirolimus treatment, through mTOR inhibition, appeared to be effectively controlling the peristent hypoglycemia and may be a life-saving therapy in this NkHH due to AKT2 and PTEN mutations.     

Incremental Value of Three‐Dimensional Transesophageal Echocardiography over Two‐Dimensional Transesophageal Echocardiography in the Assessment of Lambl's Excrescences and Nodules of Arantius on the Aortic Valve

In this retrospective study, we identified 7 cases where Lambl's excrescences were identified by two‐dimensional transesophageal echocardiography (2DTEE) and also had live/real time three‐dimensional transesophageal echocardiography (3DTEE) studies available for comparison. We subsequently assessed them for the presence of Lambl's excrescences (LE) and nodules of Arantius (NA) on the aortic valve. After their identification, we qualitatively and quantitatively organized our findings by number, cusp location, measurements, and orientation if applicable. A greater number of LE was found by 3DTEE than 2DTEE (19 vs. 11, respectively). In all 3DTEE studies, their cusp attachment site, their x‐, y‐, and z‐axis measurements, and orientation were clearly visualized and described. Only 3DTEE studies provided confident visualization of the cusp attachment sites. Similarly, a greater number of NA was found by 3DTEE than 2DTEE (21 vs. 5, respectively). The triad of NA was visualized in all 3DTEE studies and each was described using its x‐, y‐, and z‐ axis measurements. Only three 2DTEE studies provided reliable identification of the NA. In conclusion, we present further evidence of the incremental value of 3DTEE over 2DTEE in the qualitative and quantitative assessment of cardiac structures including LE and NA on the aortic valve.     

Comparison of biomarkers of oxidative stress, 8-isoprostane,advanced oxidation protein products,and 8-hydroxy-2′-deoxyguanosine and pro-apoptosis,cytokeratin 18 M30, in women with normal glucose tolerance and gestational diabetes mellitus

International Journal of Diabetes in Developing Countries - We aimed to compare the values of selected biomarkers of oxidative stress, 8-isoprostane (8IsoP), advanced oxidation protein products...     

Acute generalized exanthematous pustulosis induced by iodixanol (Visipaque): a serious reaction to a commonly used drug

Acute generalized exanthematous pustulosis (AGEP) is an acute sterile pustular eruption most commonly induced by medications. Although antibiotics are the most commonly accused drugs in AGEP, non-antibiotic agents may also cause this disease. We present a case of AGEP following use of iodixanol for coronary angiography in a 61-year-old woman. Given the wide use of this substance in cardiology, clinicians should be aware of this potential complication.     

Type of anesthesia affects neonatal wellbeing and frequency of transient tachypnea in elective cesarean sections

Objective: We aimed to assess whether the type of anesthesia in cesarean section (C/S) (spinal anesthesia, SA versus general anesthesia, GA) has an effect or not on umblical vein blood gas analysis and APGAR scores of term neonates and development of transient tachypnea of the newborn (TTN).

Methods: The data of 172 procedure (85, GA versus 87, SA) were collected retrospectively. Results of umblical vein blood gas analysis, APGAR scores at first and fifth minutes and presence of TTN from in-hospital files’ of neonates were examined.

Results: Neonates in the SA group had significantly higher first and fifth minute APGAR scores (8, 7 versus 9, 2, p?p?=?0.017, respectively). The pH value of umblical vein samples were higher (7.30?±?0.05 versus 7.32?±?0.05, p?=?0.029) and pO₂ and SaO₂ levels were significantly lower in the SA group (34.8?±?13.8?mmHg versus 27.6?±?14.5?mmHg; p?=?0.001 and 56.6%?±?18.7 versus 49.8%?±?21.4; p?=?0.029, respectively) as compared to the GA group. Thirteen neonates in the GA group (15.3%) and five in the SA group (5.7%) were diagnosed as TTN (p?=?0.048).

Conclusion: In our study, considerable determinants of fetal wellbeing was stated to be higher in C/S performed under SA in comparison to GA. Furthermore, our findings favor SA for avoidance of TTN.     

A new risk factor for neonatal vancomycin-resistant Enterococcus colonisation: bacterial probiotics

Objective: Vancomycin-resistant Enterococcus (VRE) colonisation can be controlled with strict adherence to infection control measures. We describe a VRE outbreak coincident with bacterial probiotic trial. Relationship between probiotic and VRE colonisation, and other possible risk factors were investigated.

Methods: Two hundred and ten infants with gestational age less than 32 weeks had been randomised for a trial with probiotic preparation containing Lactobacillus casei, Lactobacillus rhamnosus, Lactobacillus plantarum, Bifidobacterium lactis, fructooligosaccharide, galactooligosaccharide, colostrums and lactoferrin (NBL probiotic ATP®; Nobel, Istanbul, Turkey) between February 2012 and August 2013 when a VRE outbreak also took place. The existence of a relationship between this probiotic preparation and VRE colonisation was investigated.

Results: The begining and end of the outbreak were coincident with the beginning and end of the probiotic trial. Demographic and clinical features of neonates did not differ between VRE colonised (n?=?94) and non-colonised infants (n?=?116) except for vancomycin (p?=?0.012) and probiotic (p?<?0.001) use.

Conclusions: Probiotic and vancomycin exposure were significant risk factors for VRE colonisation. The acquisition and transfer of resistance genes of bacteria may be mediated by probiotics. Therefore, the safety of probiotics is a concern and should be investigated further.     

Burkitt lymphoma with unusual presentation: Acute pancreatitis

Pancreatitis due to malignant infiltration is an uncommon condition in childhood. Pancreatic lymphomas constitute <2% of all non‐Hodgkin lymphomas. Only six reported cases with various clinical presentation have been documented in the literature. Described herein is the case of a nine‐year‐old boy with abdominal pain, jaundice, emesis, weight loss, diarrhea, who developed hyperlipidemia and cholestasis. Pancreatitis was suspected due to high amylase and lipase. Computed tomography and magnetic resonance cholangiopancreatography showed diffuse enlargement of the pancreas. This sausage pancreas imaging was suggestive of autoimmune pancreatitis, but the patient was diagnosed with Burkitt lymphoma on bone marrow aspiration, and rapidly improved with chemotherapy. Burkitt lymphoma should be kept in mind when patients present with pancreatitis, especially with diffuse enlarged pancreas.     

Bronchopericardial fistula, an unusual complication of oxytetracycline sclerosis therapy

Here we report a rare case of bronchopericardial fistula following intrapericardial instillation of oxytetracycline. A 63-year-old female patient was admitted for management of malignant pericardial effusion secondary to right-sided bronchogenic carcinoma. Medical therapy and recurrent percutaneous catheter drainage failed in resolving the problem, so subxiphoid pericardiostomy and drainage tube insertion was performed. There was no decrease in the drainage so we decided to perform pericardial sclerosis by intrapericardial tetracycline instillation. After the second time oxytetracycline instillation, the patient developed respiratory arrest with hemodynamic instability. A huge amount of yellow frothy secretion aspirated through the endotracheal tube. The presence of tetracycline in the bronchial secretion was proved by microbiological methods. The hemodynamic status of the patient deteriorated rapidly and despite all resuscitation measures we lost the patient within a few hours.     

Synthesis, biological evaluation and 2D-QSAR analysis of benzoxazoles as antimicrobial agents

A new series of 5(or 6)-nitro/amino-2-(substituted phenyl/benzyl)benzoxazole derivatives were synthesized and evaluated for antibacterial and antifungal activities against Staphylococcus aureus, Bacillus subtilis, Klebsiella pneumoniae, Pseudomonas aeruginosa, Escherichia coli, Candida albicans and their drug-resistant isolate. Microbiological results indicated that the synthesized compounds possessed a broad spectrum of activity against the tested microorganisms at MIC values between > 400 and 12.5 microg/ml. The results against B. subtilis, P. aeruginosa, drug-resistant B. subtilis, drug-resistant E. coli, and C. albicans isolate for these kinds of structures are quite encouraging. The 2D-QSAR analysis of a set of newly and previously synthesized benzoxazoles tested for growth inhibitory activity against B. subtilis ATCC 6633 was performed by using the multivariable regression analysis. The activity contributions for substituent effects of these compounds were determined from the correlation equation for predictions of the lead optimization.