Limited vertical skin incision for median sternotomy.

The cosmetic deformity of the vertical skin incision for median sternotomy was decreased by using a mechanical traction system to increase exposure at the superior margin of a shorter wound. The limited vertical skin incision did not impede technical surgical maneuvers and produced a scar that was more acceptable than submammary incision or right anterior thoracotomy. The limited skin incision is especially useful in young women with congenital heart disease.     

Occipital horn syndrome: report of a patient and review of the literature

We report an 18-year-old boy with occipital horn syndrome and we review the 20 cases previously published with this syndrome. The distinctive features common to all patients were unusual facial appearance, skeletal abnormalities, chronic diarrhea and genitourinary abnormalities. The skeletal abnormalities included occipital horns, short, broad clavicles, deformed radii, ulnae, and humeri, narrowing of the rib cage, undercalci-fied long bones with thin cortical walls and coxa valga. Occipital horn syndrome is inherited in an X-linked recessive fashion. Our analysis indicates that occipital horn syndrome is associated with a recognizable characteristic phenotype.     

Inhibitory effects of nisoldipine and saralasin on angiotensin II-induced antidiuresis in anesthetized dogs

Inhibitory effects of the calcium channel blocker nisoldipine on angiotensin II-induced antidiuresis were investigated in anesthetized dogs, and the findings were compared with those of saralasin. Intrarenal arterial infusion of 10 ng/kg/min angiotensin II resulted in marked decreases in renal blood flow (RBF) and urine formation, with a relatively moderate decrease in glomerular filtration rate. There were marked reductions in the fractional excretion of lithium, which is used as an index of the fractional proximal excretion of sodium, and the fractional distal excretion of sodium. Nisoldipine (50 ng/kg/min) administered intrarenally produced a partial inhibition on the decreased response of RBF to angiotensin II. The peptide-induced decreases in urine flow, urinary excretion of electrolytes and fractional excretion of electrolytes were abolished by nisoldipine. In contrast, when saralasin was administered intrarenally at 10 ng/kg/min, a dose which could partially inhibit the angiotensin II-induced decrease in RBF to the same extent as seen with nisoldipine, the antagonist attenuated, but did not abolish, the antidiuretic action of angiotensin II. Significant decreases in urine formation by angiotensin II were observed, even in the presence of saralasin. These results suggest that nisoldipine, unlike saralasin, preferentially interferes with the stimulatory effect of angiotensin II, as related to the renal tubular reabsorption of sodium and water.     

Internal jugular vein catheterization in infants undergoing cardiovascular surgery: an analysis of the factors influencing successful catheterization.

Central venous catheterization for pressure monitoring and drug administration is often important in the anesthetic management of infants undergoing cardiovascular surgery. We examined the effects of patient age, weight, and central venous pressure and the experience of the anesthesiologist on the rate of successful catheterization and catheterization time of the internal jugular vein (IJV) in a prospective study. We studied 106 infants undergoing IJV catheterization for cardiovascular surgery over a 7-mo period at our institution. We catheterized the IJV by the high approach. The direct venipuncture or the Seldinger method was used according to the patient's weight. Overall successful catheterization rate was 97.2%, and the average catheterization time was 353 +/- 21 s (mean +/- SEM). Complications included arterial puncture in 12 cases (11.3%), hematoma formation in four cases (3.8%), and catheter malposition in two cases (1.9%), but pneumothorax was not observed. When a patient was younger than 3 mo or weighed less than 4.0 kg, successful catheterization rate decreased significantly to 81.3% and 78.6%, respectively. Catheterization time was inversely correlated with both age and weight, whereas central venous pressure did not affect either successful catheterization rate or catheterization time. We were unable to demonstrate that the experience of the anesthesiologist plays a significant role in the success or complication of the catheterization procedure. Our results indicate that IJV catheterization by the high approach is a reliable and useful technique in infants, and that the weight and age of the patient significantly influence the rate of successful catheterization.     

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.     

Dysferlin mutation analysis in a group of Italian patients with limb-girdle muscular dystrophy and Miyoshi myopathy

Mutations in the dysferlin gene (DYSF) on chromosome 2p13 cause distinct phenotypes of muscular dystrophy: limb-girdle muscular dystrophy type 2B (LGMD2B), Miyoshi myopathy (MM), and distal anterior compartment myopathy, which are known by the term 'dysferlinopathy'. We performed mutation analyses of DYSF in 14 Italian patients from 10 unrelated families with a deficiency of dysferlin protein below 20% of the value in normal controls by immunoblotting analysis. We identified 11 different mutations, including eight missense and three deletion mutations. Nine of them were novel mutations. We also identified a unique 6-bp insertion polymorphism within the coding region of DYSF in 15% of Italian population, which was not observed in East Asian populations. The correlation between clinical phenotype and the gene mutations was unclear, which suggested the role of additional genetic and epigenetic factors in modifying clinical symptoms.     

The effect of dietary protein source on plasma cholesterol level and fecal steroid excretion in obese mice

Both goldthioglucose (GTG)-treated and the genetically obese (C57BL/6J ob/ob) mice were fed semisynthetic diets containing either soy protein isolate (SPI) or casein as a protein source, or laboratory chow. In GTG-induced obese mice, the plasma cholesterol level correlated positively with their body weight. The level was highest in mice fed high-fat diet, and lowest in ones fed laboratory chow. No difference was observed between SPI and casein groups whether the diet was low-fat or high-fat. Thus, in the GTG-treated mice, SPI did not have a hypocholesterolemic effect while dietary fat had a hypercholesterolemic effect, and laboratory chow contained some component(s) which can lower the plasma cholesterol level. Both neutral and acidic steroid contents in feces of the SPI group were not different from those of the casein group, and both groups of mice excreted a smaller amount of steroids than mice fed laboratory chow. Results of essentially the same tendency were obtained with normal mice regarding the effects of SPI and casein, although the degree of hypercholesterolemia was lower in high-fat-fed normal mice than in similarly fed GTG-treated mice. These results strengthened the inverse correlation between the amount of fecal steroids and the plasma cholesterol level upon feeding various proteins, indicating that the former is one of the important factors that determine the latter. The ob/ob mice showed a marked hypercholesterolemia irrespective of the kind of diet. The amount of fecal steroids was highest in the laboratory chow group and lowest in the casein group. This indicates that some factor(s) other than fecal steroid excretion is dominantly responsible for their hypercholesterolemia.     

Effects of hypothermal and circulatory arrest on the auditory brainstem response during operation in children

Twenty-two children were monitored by the auditory brainstem response (ABR) during the open chest and/or open heart surgery for cardiac anomalies under the extracorporeal circulation (ECC) with moderate to deep hypothermia. These patients were divided into two groups, namely, the ECC only group (group A: 9 cases) and the total circulatory arrest (TCA) group (group B: 13 cases). The mean age of group A was 36 months and group B was 6.1 months. In group A, moderate hypothermia was conducted with the rectal temperature ranging from 25 degrees C to 30 degrees C, and in group B profound hypothermia was conducted lowering the temperature to 18 degrees C. The ABR recorded at the following points, namely; before inducing anesthesia, before lowering the body temperatures, during the cooling process, at the time of TCA, and during the rewarming process. Accompanying the decrease in body temperature the peak latency of waves I, III and V were markedly prolonged. When the rectal temperature fell to 23 degrees C, the peak latency of each wave was prolonged to about 150% of their precooling values at 36 degrees C. When it fell to 22 degrees C, the ABR disappeared entirely in 16 of 22 cases, and in remaining 6 cases, only I wave was detected. During rewarming, in both the A and B groups, at 24 degrees C, the wave of ABR started to reappear beginning with I waves, and on reaching 26 degrees C, I, III and V waves from became detectable. The peak latency of all waves at rectal temperature of 33 degrees C recovered to almost the same values as these at 36 degrees C.(ABSTRACT TRUNCATED AT 250 WORDS)