Although alpha2-adrenoceptor agonists are widely used as postoperative sedatives in adults, the postoperative effects in pediatric patients with secondary pulmonary arterial hypertension (PAH) due to congenital heart disease are not well known. We experienced a case of successful ventilator weaning with continuous intravenous administration of dexmedetomidine (DEX) after surgical correction in a 46-month-old child with congenital ventricular septal defect (VSD) with severe PAH. She underwent VSD closure on cardiopulmonary bypass (CPB). After successful weaning from the CPB, hemodynamics and oxygenation were stabilized on DEX and nitroglycerin in the intensive care unit. The patient was successfully weaned from the ventilator 46 hours after surgery. The transthoracic echocardiogram two weeks after surgery showed a closed VSD with no residual shunt and trivial tricuspid regurgitation (Vmax = 2.5 m/sec) without PAH.
Administration, Intravenous ; Adult ; Cardiopulmonary Bypass ; Child* ; Child, Preschool ; Dexmedetomidine* ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular* ; Hemodynamics ; Humans ; Hypertension* ; Hypnotics and Sedatives ; Intensive Care Units ; Nitroglycerin ; Oxygen ; Tricuspid Valve Insufficiency ; Ventilator Weaning ; Ventilators, Mechanical ; Weaning

No abstract available.
Colon, Ascending*

Repairing large incisional hernia with abdominal wall reconstruction is a technically challenging problem for surgeons. We report our experience of large midline incisional hernia which was repaired successfully with components separation technique. A patient with incisional hernia, 35 x 20 cm in size, underwent operation following standard components separation technique. The aponeurosis of the external abdominal oblique muscle was longitudinally transected from the rectus sheath, and the external abdominal oblique muscle was separated from the internal abdominal oblique muscle. With further separation of the posterior rectus sheath from the rectus abdominis muscle, closure of the abdominal wall was attained without tension. The post-operative course was uneventful with minor wound seroma. The patient discharged safely, and no further complication in terms of recurrence and wound problem has occurred. Components separation technique could be a possible and effective treatment option for repair of large abdominal wall defect.
Abdominal Wall ; Hernia ; Hernia, Ventral ; Humans ; Muscles ; Rectus Abdominis ; Recurrence ; Seroma

Reiters syndrome is a clinical triad of arthritis, urethritis and conjunctivitis, but the characteristic mucocutaneous lesions occur frequently enough to be included in this syndrome. This applies to the initial stage of the disease, in which the arthritis usually, but by no means invariably, comes triad, i.e. after the urethritis and conjunctivitis have made their apperance. In most instances the arthritisis is of subacute onset, reaching its full intensity within a few weeks. Some 50% of the patients suffer from recurrences of the arthritis. The chronic progressive polyarthritis occuring in Reiters syndrome has severe functional and anatomical repercussions. Here we report a case of Reiters syndrome in a 14 year old middle school boy. He had classical characteristic triad of arthritis, nonspecific urethritis and conjunctivitis. He had pyuria in first fewdays after onset and the arthritis of acute onset, reaching its full intensity within two weeks and then followed by conjunctivits. A review of literature was included.
Arthritis ; Conjunctivitis ; Humans ; Male ; Pyuria ; Recurrence ; Urethritis

No abstract available.

No abstract available.
Education, Medical* ; Prospective Studies*

No abstract available.

No abstract available.
Transcription Factors*

No abstract available.

BACKGROUND: Leptin secreted from adipose tissue activates not only its receptors expressed abundantly in the brain, including arcuate nucleus but the POMC neurons resulting in secretion of a-MSH. Activation of MC4R by a-MSH increases the energy expenditure and decreases the food intake, and promotes the sympathetic activity. METHODS: To analyze the biologic activity of a-MSH analogues, the rat MC3R DNA and human MC4R DNA were stably and independently expressed in CHO cell lines, and we performed the competitive receptor binding assay with [125I]NDP-MSH as a radioligand and cyclic AMP accumulation assay following treatment with different ligands. a-MSH (Ac-Ser-Tyr-Ser- Met-Glue-His-Phe-Arg-Trp-Gly-Lys-Val-NH2) and its analogue a-MSH-ND (Ac-Nle-Asp-His-Dphe- Arg-Trp-Lys-NH2) were synthesized by Genosys Biotechnologies, Inc. The HPLC purified samples for NMR experiments were dissolved in 90% HO/10% DO or 99.9% DO solutions at pH 7.0 with concentrations of 2 mM in 50 mM sodium phosphate buffer. The NMR spectra were acquired on a Bruker AMX-500 or DMX-600 spectrometer in quadrature detection mode equipped with a triple-resonance probe with an actively shielded pulsed field gradient coil. RESULTS: The preferential order of binding activity for MC3R was NDP-MSH > a-MSH-ND > [Nle4]-MSH with IC50 values (nM) being 0.367 +/- 0.074, 47.20 +/- 9.08 and 106.70 +/- 32.25, respectively. For MC4R, the same preferential order, NDP-MSH (0.566 +/- 0.194) > a-MSH-ND (6.70 +/- 1.07) > [Nle4]a-MSH (238.30 +/- 30.47), were obtained. In c-AMP generation assay, the potency order of peptides was NDP-MSH > a-MSH-ND > a-MSH at both MC3R and MCAR. Accumulated food intake of Sprague Dawley rats was low significantly for six hours after injection of a-MSH-ND into peritoneum. a-MSH forms a hair pin loop conformation, whereas a-MSH-ND, a linear form of MTII, prefers a type I B-turn comprising residues Asp5-His6-DPhe7-Arg8 in NMR study. CONCLUSION: we developed in vitro and in vivo bioassay system to evaluate the biologic activity of a-MSH analogues and we also showed that the type I B-turn structure of a-MSH-ND might enhance the binding activity to MC4R.
Adipose Tissue ; Animals ; Arcuate Nucleus ; Biological Assay ; Biotechnology ; Brain ; CHO Cells ; Chromatography, High Pressure Liquid ; Cricetinae ; Cyclic AMP ; Diabetes Mellitus* ; DNA ; Eating ; Energy Metabolism ; Hair ; Humans ; Hydrogen-Ion Concentration ; Inhibitory Concentration 50 ; Leptin* ; Ligands ; Neurons ; Peptides ; Peritoneum ; Pro-Opiomelanocortin ; Rats ; Rats, Sprague-Dawley ; Sodium