Biomedical subjects
Publications and source records attributed to S Arsan.
The effect of oral administration of desmopressin (DDAVP) solution was investigated in a very low birth weight premature infant with central diabetes insipidus that was associated with grade four germinal matrix hemorrhage. As an alternative to the nasal route, long-term successful management resulting in favorable growth and development during infancy was achieved using the oral route.
TPIT is a highly cell-restricted transcription factor that is required for the expression of the propiomelanocortin (POMC) gene and for terminal differentiation of the pituitary corticotroph lineage. Its exclusive expression in pituitary POMC-expressing cells has suggested that its mutation may cause isolated deficiency of pituitary ACTH. We present a neonate with the diagnosis of congenital early onset isolated ACTH deficiency (IAD) associated with a loss of POMC function as a result of a missense mutation in the TPIT gene. A 5 day-old male infant was admitted for hypoglycemia, limpness and conjugated hyperbilirubinemia. Laboratory investigations indicated low plasma cortisol concentration (0.1 microg/dl) accompanying a very low ACTH (<5 pg/ml) concentration. An increase in plasma cortisol concentration following stimulation with low dose exogenous ACTH was observed. On replacement therapy with hydrocortisone (15 mg/m2/day orally), cholestatic jaundice and hypoglycemia resolved and subsequent normal growth (weight, height and head circumference, 25th, 10th and 50th percentile, respectively) and development was achieved without recurrence of hypoglycemic episodes.
The ophthalmo-acromelic syndrome of Waardenburg is an autosomal recessive trait comprising eye malformations ranging from true anophthalmia to mild microphthalmia with acromelic malformations. Some 29 affected individuals have been reported since Waardenburg's first report in 1935 [Waardenburg et al., 1961]. We report on a new case with bilateral anophthalmia and typical limb malformations. The patient also was found to have interruption of the inferior vena cava with azygos continuation as an additional finding. The previous reports are reviewed to elucidate the spectrum of the syndrome.
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We report the case of a 62-year-old male patient with left main coronary artery originating from the pulmonary trunk, severe mitral insufficiency, and huge right coronary artery aneurysm. He is the oldest such patient among those reported in the literature, surviving to the sixth decade without any anginal symptoms. He is also the first such case with such a huge and calcified right coronary artery aneurysm and a prominent collateral from the noncoronary circulation.
The disease of pulmonary vascular sling is a rare congenital condition. When it does occur it is often in conjunction with tracheal compression. The authors report herein a newborn with this anomaly presenting with a tracheal and esophageal compression.
The rate of evaporation from the skin (g/m2/h) was measured in 12 full-term and 16 preterm infants (gestational age 25-34 wk) both during incubator care and when nursed under a radiant heater. The method for evaporation rate measurement is noninvasive and based on determination of the water vapor pressure gradient close to the skin surface. Measurements were first made with the infant nursed in an incubator with a controlled environment with respect to humidity, temperature, and air velocity. The measurements in the term infants were performed at an ambient relative humidity (RH) of 50%, and in the preterm infants first at 50% and subsequently at 30-40%. Evaporation rate was then measured with the infant nursed under a radiant heater. In term infants, mean evaporation rate was 3.3 g/m2/h during incubator care (RH 50%) and 4.4 g/m2/h during care under the radiant heater. In preterm infants, the corresponding values were 15.5 g/m2/h in the incubator at RH 50%, 16.7 g/m2/h at RH 30-40%, and 17.9 g/m2/h under the radiant heater. It is concluded that the evaporative water loss from the skin depends on the ambient water vapor pressure, irrespective of whether the infant is nursed in an incubator or under a radiant heater. The higher rate of evaporation during care under a radiant heater is due to the lower ambient water vapor pressure and not to any direct effect of the nonionizing radiation on the skin.
From 1984 until 1994, 75 consecutive patients younger than 12 months of age were operated on for coarctation of the aorta. We retrospectively analyzed predictive factors for morbidity and mortality, and also interaction between surgical procedures and recoarctation. Surgical procedures were as follows: resection with a traditional end-to-end (E-E) anastomosis in 55 patients (73.3%), prosthetic patch aortoplasty (PPA) in 12 patients (16%) and subclavian flap aortoplasty (SFA) in 8 patients (10.7%). Early mortality was 9.33% (7 patients). Logistic regression analysis proved that age at operation, associated anomalies of heart, type of coarctation, aortic arch hypoplasia and pulmonary banding were independent predictors of hospital death. Late mortality occurred in 7 patients (10.3%). Associated anomalies of heart were an independent prognostic factor for late mortality. Actuarial freedom from recoarctation at 1 year was 91% [confidence limits (CL): 82% to 97%] and 5 years were 74% (CL: 67% to 86%). Immediate postrepair gradient was equal after E-E anastomosis and other procedures. We conclude that the treatment of first choice in the management of coarctation of the aorta in infants is E-E anastomosis.
A retrospective analysis of the records of 105 patients who underwent pericardiectomy from 1983 to 1993 was performed. Primarily, 40 patients (38%) had tuberculosis, 16 patients (15%) a malignancy, 12 patients (11.4%) uremia, and 11 patients (10.5%) had rheumatic disorders. Pericardiectomy was performed through midline sternotomy in all cases, 9 of them required cardiopulmonary bypass. On operation, the anterior pericardium was excised parallel to the phrenic nerves on both side. The early mortality rate was 10.5% (11 patients). Mean follow-up time was 5.8 +/- 2.1 years, ranging from 1 year to 11 years. Actuarial survival of the patients at 1 year and 5 years were 81.1% +/- 6.8% and 63.5% +/- 8.2%, respectively. We conclude that pericardiectomy using midline sternotomy with or without cardiopulmonary bypass can be performed safely and can lead to good functional results and long-term survival.
A case of spontaneous coronary artery dissection in a 26-year-old male with subsequent left-ventricular aneurysm is reported. A coronary bypass operation using left internal mammary artery combined with left-ventricular aneurysmectomy was performed successfully. This is the youngest case in the literature which describes a successful surgical treatment for a spontaneous coronary artery dissection with left-ventricular aneurysm.
Transcutaneous Doppler ultrasound measurements were made of the superior mesenteric artery of 25 term infants to correlate the intestinal blood flow with neonatal acid-base status as measured in umbilical artery blood and newborn condition as reflected by Apgar score. Compared with the babies whose umbilical artery pH was higher than 7.20 (peak systolic velocity: 84.1 cn/sn, mean velocity 48.6 cm/sn), the blood flow of superior mesenteric artery was significantly decreased in the newborn babies whose umbilical artery pH was less than 7.20 (peak systolic velocity 48.8 cm/sn, mean velocity = 32.6 cm/sn). Although no such relationship existed between Apgar scores and superior mesenteric artery blood flow indices, a significant linear correlation between superior mesenteric artery blood flow and umbilical artery pH was noted. A reduction of intestinal circulation was observed during umbilical cord blood acidemia and hypoxemia.
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A three-year-old girl was admitted to Hacettepe Children's Hospital due to sweating and poor feeding. Echocardiography demonstrated ostium primum atrial septal defect with a cleft mitral valve. After two months of medical follow-up with digitalis, she was subjected to open-heart surgery, and a primum-type atrial septal defect with double mitral orifice was found. To avoid creating acute mitral stenosis, the cleft was only partially sutured and the septal defect was closed with a patch. Her postoperative recovery was uneventful. It is emphasized that surgical intervention should be individualized in each case.
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We present a case of tetralogy of Fallot who had previously undergone a graded Brock-type operation. The post-operative development of pulmonary arteries and pulmonary trunk after the Brock operation were very suitable for total correction, which was then performed successfully. In contrast to shunt, in Brock-type operations enlargement of the outflow tract induces symmetrical growth of hypoplastic pulmonary arteries without the risk of acquired pulmonary obstruction or peripheral stenosis at the site of anastomosis. After palliative operations, the repair can be deferred until the pulmonary arteries are of a suitable size.
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