Liver transplantation from a live donor to a patient with maple syrup urine disease: Two case reports

Liver transplantation were reported in patients with classic maple syrup urine disease in the literature. Branched chain alpha keto acid dehydrogenase activity can be improved in patients after transplant, and a protein-restricted diet is usually not needed. The first patient was a boy aged 2,5 years who presented with frequent ketosis attacks and epileptic seizures, and the second patient was an 11-month-old boy who also presented with frequent ketosis episodes, both despite adherence to diet therapy. Both patients received liver transplantations from live donors. A low protein diet was no longer required and no decline in cognitive functions was observed in either patient in the follow-up. We wanted to present these cases to show that despite a normal diet, plasma levels of branched- chain amino acids remained normal without any decline in cognitive function after liver transplantation in patients with classic maple syrup urine disease patients.

A Case of laryngeal atresia accompanied with persistent pharyngotracheal ductus

Laryngeal atresia is generally a fatal congenital anomaly with an incidence of 1: 50,000 births. This congenital anomaly is a condition of multifactorial inheritance, in which the fetus has a dilated trachea, enlarged echogenic lungs, an inverted or flattened diaphragm, fetal hydrops, and ascites. Diagnosis is usually made when there is failure to perform endotracheal intubation in a neonate with severe respiratory distress and absence of audible cry. Here, we present a very rare case of a newborn with laryngeal atresia who had respiratory distress and was sustained for the first few minutes of life using partial ventilation via a persistent pharyngotracheal duct. We would like to draw the attention of all physicians to this issue by reporting a rare fatal case of a newborn with a congenital presentation.

Distribution of childhood skin diseases according to age and gender, a single institution experience

Studies on pediatric skin diseases in Turkey are not yet sufficient. It was aimed to characterize the prevalence of skin diseases, age and gender distribution in children living in the Erzincan region.

National Control of Tuberculosis: Does Primary Health Care System Play a Crucial Role in the Fight Against Tuberculosis?

To analyze the tuberculosis control studies in a primary health care center and to observe the changes throughout the years.

Evaluation of Noncystic Fibrosis Bronchiectasis Using Clinical and Radiological Scorings in Children

The aim of this study was to evaluate radiological, clinical, and demographic data of patients with noncystic fibrosis bronchiectasis and to compare high-resolution computed tomography (HRCT) scores based on the demographic and clinical characteristics.

Authors: Sapiens H, Consilius H, Laborem H, Mutuus H, Exercitationa H, Parasitorum H, Gloria H

Needs Assessment Kompetensi Pelengkap Mahasiswa Pendidikan Islam Anak Usia Dini Berdasarkan Kebutuhan Pengguna

The purpose of this study is to identifying competences of students of Department of Pendidikan Guru Raudhatul Athfal in Tarbiyah and Teaching Faculty UIN Maulana Malik Ibrahim Malang that needed by users. This study using needs assessment approach for finding competences of student that needed by users. This study used a quantitative approach and the type of this study was a survey research (quantitative descriptive). Data was collected by using questionnaires and interviews. The result of this study are competences that need to be owned by the students based on priority needs. They are as follows: (1) Factor 1 consist of skills related to the skills intra-personal namely control emotions, affection, self confidence, work hardly, motivation, discipline, never surrender, diligent, initiative, creativity, adaptability, ability of IT, (2) Factor 2 consist of skills related to the skills of interpersonal namely the ability of Arabic, teamwork, ability 0f english, proficiency, leading a team, written communication, public speaking, verbal communication; (3) Factor 3 consists of skill related to technical skill that are praktik ibadah, hafalan surat pendek, skill of arts, baca tulis al-Qur’an, skill of music, skill of dancing, doa-doa harian.

RANCANG BANGUN TIMER KONTROL ALAT LAMPU ULTRA VIOLET STERILIZER DILENGKAPI SISTEM BUZZER

Ecofriendly and simple synthesis of pyrano[3,2-c]quinolone in water via an efficient one-pot three-component reaction

Pyrano[3,2-c]quinolones are commonly found in alkaloids, manifesting diverse biological activities. In this work, 2-amino-6-methyl-5-oxo-4-substituted-5,6-dihydro-4 H -pyrano[3,2-c]quinoline-3-carbonitriles and ethyl 2-amino-4-(substituted)-6-methyl-5-oxo-5,6-dihydro-4 H -pyrano[3,2-c]quinoline-3-carboxylates have been synthesized efficiently from reaction of 4-hydroxy-1-methylquinolin-2(1 H )-one, aldehydes and active methylene nitriles in one-pot three component reaction in aqueous medium, containing catalytic amount of ethanolamine resulting in 70-95% yields.

Synthesis and characterization of new 4-aryl-2-(2-oxopropoxy)-6-(2,5-dichlorothiophene)nicotinonitrile and their furo[2,3-b]pyridine derivatives: Assessment of antioxidant and biological activity

A new series of furo[2,3- b ]pyridine derivatives bearing aryl substituents were synthesized in two steps, where, the cyano-(2 H )-pyridones (1a-l) were converted to the corresponding nicotinonitriles (2a-l), followed by the Thorpe-Ziegler ring cyclization to the furo[2,3- b ]pyridine derivatives (3a-l). All new compounds were characterized by 1D-NMR experiments ( 1 H and 13 C) and 2D-NMR experiments (COSY, HMBC and HSQC), as well as ESI-MS and HR-ESI-MS data. The new compounds were screened for their antioxidant activities by 2,2-diphenyl-1-picryl-hydrazylhydrate (DPPH) free radical assay. The highest radical scavenging effect was observed for nicotinonitriles 2d, 2h and 2l and furo[2,3- b ]pyridines 3b, 3f and 3j by methanolic solvent at 4.0 mg/mL concentration. Remarkably, all nicotinonitriles and furo[2,3- b ]pyridine exhibited a significant radical scavenging activity after 24 and 48 hours compared with 0.5 hour.