The limitation of staged repair in the surgical management of congenital complex heart anomalies with aortic arch obstruction

OBJECTIVE: Severe aortic arch obstruction including an interrupted aortic arch in congenital complex heart anomalies remains a challenge in surgical management. METHODS: Treatment and outcomes in 75 consecutive patients who underwent an aortic arch repair as the first step of the staged repair protocol between 1975 and 2000 were reviewed. Their ages at repair ranged from 1 day to 8.5 months. RESULTS: Cross-sectional postoperative follow-up data were available in all the patients. The follow-up period ranged from 0 to 27.6 years (mean: 7.3 +/- 7.3 years). There were 20 postoperative hospital deaths (27%) and 7 late deaths. The Kaplan-Meier estimate of survival was 81.3% +/- 4.5% at 1 month, 68.0% +/- 5.4% at 1 year, 65.0% +/- 5.5% at 5 years, 63.1% +/- 5.7% at 10 years, 63.1% +/- 5.7% at 20 years. By Cox regression analysis, body weight of 2.5 kg or less is the only independent determinant of postoperative mortality (p = 0.04, multivariable odds ratio: 2.50, [95% confidence interval: 1.02-6.1]). The aortic arch morphology, the primary cardiac lesion, or date of operation did not reach a statistically significant level to show correlation with mortality. Reintervention to reconstruct the aortic arch was performed at 9 occasions in 8 of the 55 patients who survived the primary operation (14.5%). The Kaplan-Meier estimate of the reintervention-free rate was 91.3% +/- 4.2% at 5 years, 85.5% +/- 5.6% at 10 years, 75.6% +/- 8.2% at 20 years. Using multivariable Cox regression analysis, interrupted aortic arch (versus aortic coarctation) was the only independent predictor of a shorter time to reintervention (p = 0.001, multivariable odds ratio: 16.1, [95% confidence interval: 3.2-80.2]). CONCLUSIONS: The staged repair protocol was associated with significant limitations in patient survival and with the development of recurrent aortic arch obstruction. Thus, a primary repair protocol may serve as an alternate approach, especially in patients with low weight or with an interrupted aortic arch.     

Cyclic adenosine monophosphate production and contractile response induced by beta-adrenoceptor subtypes in rabbit urinary bladder smooth muscle

The spontaneous contractile force of muscle strips isolated from male rabbit urinary bladder dome [detrusor) and base (trigonal muscle) was dose dependently inhibited by isoproterenol, a non-specific beta-adrenoceptor agonist. The relaxant response to 10(-6) M isoproterenol in the detrusor muscle was completely blocked by butoxamine (10(-4) M), a selective beta-2-antagonist, and by propranolol (10(-6) M), a non-specific beta-antagonist, but not by metoprolol (10(-6) to 10(-4) M), a selective beta-1-antagonist. Relaxation of trigonal muscle induced by 10(-6) M isoproterenol was inhibited 30% by metoprolol (10(-5) M), 70% by butoxamine (10(-4)M), and 100% by propranolol (10(-6) M). Terbutaline, a selective beta-2-adrenoceptor agonist, also caused dose dependently a relaxant response in detrusor and trigonal muscle. The maximum relaxant responses to isoproterenol and terbutaline were significantly greater in detrusor than in trigonal muscle. Dobutamine, a relatively specific beta-1-adrenoceptor agonist, caused a small but significant relaxant response in trigonal, but no change in detrusor muscle. In trigonal muscle the relaxant response to dobutamine was less than that to terbutaline. Cyclic adenosine monophosphate accumulation in detrusor did not significantly increase after administration of dobutamine, but significantly increased after administration of terbutaline. On the other hand, not only terbutaline, but also dobutamine, markedly increased cyclic adenosine monophosphate accumulation in trigonal muscle.(ABSTRACT TRUNCATED AT 250 WORDS)     

Effects of ultraviolet B irradiation on lenticular riboflavin metabolism and high-molecular-weight-protein aggregation

We investigated the effects of ultraviolet B (UVB) irradiation on the formation of ester forms of riboflavin and of high-molecular-weight (HMW) protein aggregates and on lenticular riboflavin-binding capacity (LRBC). Esterification of riboflavin decreased as the duration of UV irradiation increased, suggesting the irradiation-induced denaturation of the apoenzyme of synthetases of ester forms of riboflavin. UVB irradiation of lens homogenate supplemented with riboflavin increased LRBC and the formation of HMW protein aggregates, while gamma-crystallin was decreased. These results are consistent with those of our earlier studies in which we obtained data suggesting that, upon exposure of rat lens homogenate to fluorescent light, photosensitized riboflavin may bring about cross-linking of lens protein. Our data demonstrate that the photosensitivity of lenticular riboflavin is increased by longer periods of UV irradiation.     

Toxicity of cadmium oxide instilled into the rat lung. I. Metabolism of cadmium oxide in the lung and its effects on essential elements

The metabolism of cadmium oxide (CdO, insoluble form) and cadmium chloride (CdCl2, soluble form) instilled intratracheally into the rat lung was investigated. CdO might be solubilized rapidly in the lung and consequently pulmonary clearance rate of CdO was not so different from that of CdCl2. At a dose of 5 micrograms Cd/rat about 20% of the dose was translocated to the liver within 12 h, whereas gradual and consistent accumulation of Cd was observed in the kidney up to 7 days. Both pulmonary clearance and translocation of Cd to the liver were accelerated with the dose of instilled CdO, however, Cd accumulated in the kidney was proportional to the dose. Lung weight was increased by the instillation of CdO. Lung essential elements such as S, P, Mg, Zn and Mn were not affected in the inflammatory-reparative proliferative process, but Cu content of unit lung weight was slightly decreased.     

Clinical similarities of hereditary progressive/dopa responsive dystonia caused by different types of mutations in the GTP cyclohydrolase I gene

OBJECTIVE—Hereditary progressive dystonia withpronounced diurnal fluctuation ((HPD)/dopa responsive dystonia (DRD))is a childhood onset dystonia which responds to levodopa. Variousclinical signs and symptoms of HPD/DRD have been recognised to date.Mutations in the GTP cyclohydrolase I (GTP-CH-I) gene were recentlyidentified as the cause of HPD/DRD. In the present study, the GTP-CH-Igene and the clinical features of eight HPD/DRD patients from sixfamilies were analysed to determine the correlationsbetween clinical expression and the mutations in the GTP-CH-I gene.
METHODS—The exons, exon-intron junctions, and anindispensable part of the 5' flanking region of the GTP-CH-I gene weresequenced in the eight clinically diagnosed patients with HPD/DRD andtheir asymptomatic parents.
RESULTS—Three independent mutations in theGTP-CH-I gene were found in three patients. One of the patients and herasymptomatic mother were heterozygous for a novel mutation at theinitiation codon. The three patients with dissimilar GTP-CH-I mutationsexhibited similar clinical features. The other five patients withnormal sequences presented several features not manifested by the three patients with the mutations. No mutation was found in the 5' flanking region of any patients or their parents.
CONCLUSIONS—A novel initiation codon mutation wasfound in a Japanese patient with HPD/DRD. The clinical manifestationscommon to the patients with HPD/DRD with a mutated GTP-CH-I gene werealso identified. Although focal manifestations of HPD/DRD associatedwith the mutations of this gene will be broadened, it is inferred thatthese clinical features are fundamental to HPD/DRD caused by mutationsin this gene.

     

Natural interferon enhances expression of placental alkaline phosphatase in human seminoma xenograft

The purpose of this study was to investigate the effect of natural interferon (IFN) on the expression of placental alkaline phosphatase (PLAP) in a human seminoma xenograft in severe combined immunodeficient mice. Mice were injected intramuscularly with 3 × 10⁵ U/mouse of IFN, twice a day, for five consecutive days. A significant increase in PLAP level of the xenografts followed IFN treatment. A radiolabeled anti-PLAP monoclonal antibody (MAb) was intravenously injected on the first day of IFN administration in order to determine if IFN has the potential to enhance the efficacy of an anti-PLAP MAb in the detection of seminoma. Enhanced retention of the anti-PLAP MAb was observed at 7 and 11 days after MAb administration. Thus, IFN treatment appears to have some effect on the efficacy of the anti-PLAP MAb in the detection of seminoma xenografts. Received: 26 September 1997 / Accepted: 23 April 1998     

Hepatocellular carcinoma: Efficacy of transcatheter oily chemoembolization in relation to macroscopic and microscopic patterns of tumor growth among 100 patients with partial hepatectomy

Purpose: To evaluate the efficacy of transcatheter oily chemoembolization (TOCE) for hepatoceliular carcinoma (HCC) on the basis of microscopic and macroscopic findings postembolization. Methods: HCCs ranging in size from 0.5 to 13 cm (mean 3.6 cm) were obtained from partial hepatectomies of 100 consecutive patients who had undergone TOCE between 20 and 246 days (mean 59.5 days) prior to surgery. The efficacy of TOCE was assessed on the basis of the necrotic to live cell ratio of the tumors. The microscopic pattern of tumor growth was grouped into expanding type (complete capsule formation) and replacing type (incomplete or no capsule). There were five types of macroscopic groupings: single nodule, single nodule with extranodular growth (SNE), contiguous and noncontiguous multinodular, and massive growth type. Results: Among 79 cases with the expanding type, 29 (37%) had 100% HCC necrosis, but none with 100% necrosis were in the replacing type. By macroscopic grouping, the efficacy of TOCE decreased from the single nodule type (50% of patients had 100% necrosis) to the SNE type (21%), and the other types (9%).     

Tracheal dilatation by halothane and enflurane in man

The effect of halothane and enflurane on tracheal tone were studied in 21 patients during the induction of anesthesia. Endotracheal tube cuff pressure was used to measure tracheal tone. Anesthesia, maintained by nitrous oxide 70% in oxygen, was supplimented with succinylcholine drip infusion to immobilize the patient. Ventilation was controlled by a Volume-preset ventilator. In the halothane group, the initial cuff pressure was 14.8 ± 1.3 (mean ± SE) cmH₂O but 10min after 0.15mg/kg of pancuronium injection, it increased to 21.7 ± 2.3cmH₂O (control). Ten min after inhalation of 0.75% of halothane, cuff pressure decreased to 14.7 ± 2.3cmH₂O (34 ± 11% decrease from the control value). In the enflurane group, the initial cuff pressure was 17.6 ± 1.8cmH₂O and it increased to 21.0 ± 1.7cmH₂O (control) 10min after pancuronium injection. Ten min after 1.7% of enflurane inhalation, cuff pressure decreased to 17.1 ± 2.3cmH₂O (23.9 ± 6% decrease from the control value). Halothane and enflurane produced similar tracheal dilatation in healthy individuals.(Yasuda I, Irimada M, Hirano T et al.: Tracheal dilatation by halothane and enflurane in man. J Anesth 2: 46–49, 1988)