Insufficiency In Association Research Articles

Three-step technique for implantation of rotating hinge knee prostheses : Demonstration using the Enduro prosthesis.

Implantation of an axis-guided knee prosthesis with consideration of the joint line and balanced flexion and extension gap to reduce the mechanical load on the prosthesis axis and to improve the movement of the prosthesis. Distinct unilateral instability due to ligament insufficiency in association with knee prostheses. Primary implantation of axis-guided knee prostheses with pronounced axial malalignment (>20-25° valgus or varus malalignment) or/and severe flexion contracture > 40°. Replacement of prosthesis with nonsignificant imbalance between flexion and extension gap. Clearly unstable flexion gap such that the extension gap cannot be adjusted appropriately. Significant femoral bone defects requiring distal femoral replacement prostheses. Reconstruction of the tibial platform with the trial prosthesis with regard to height in relation to the tip of the fibula. Determination of the femoral prosthesis size. Adjustment of the anteroposterior (AP) cutting block of the selected size, referenced anteriorly and in correct rotation. Determination of the flexion gap with spacers using the stable side of the collateral ligaments. Positioning the distal cutting block and determination of the height of the extension gap. Moving the distal cutting block until the same height of spacer as used in the flexion gap fits into the extension gap, taking into account the stable side of the collateral ligaments. The extent of the displacementD1 is noted. Reverse displacement of the distal cutting block by the distanceD2 until asatisfactory bony contact surface for the femoral component in the distal femur can be created. The thickness of the required distal augments on the following chamfer-cutting guide (4-in‑1 cutting block) and thus on the femoral prosthetic component is the sum of D1 + D2. The chamfer-cutting guide (4-in‑1 block) with distal augments of the calculated height (D = D1 + D2) is placed in position. Definition and preparation of the posterior contact surface with positioning of any necessary posterior augments. Completion of the femoral preparation taking into account the distal and posterior augments. Assembly of the trial prostheses with atrial inlay having the height of the spacers used. Implantation of the selected prosthesis components. Thrombosis prophylaxis, physiotherapy under full weight-bearing and mobility exercises. After implantation of 104 axis-guided Enduro™ knee prostheses (Aesculap AG, Tuttlingen, Germany) (73knee prosthesis revisions and 31 primary implantations), the Knee Society Score increased from 42.8 ± 18.8 preoperatively to 84.8 ± 13.9 after 24months. Complications comprised one deep vein thrombosis and one periprosthetic infection.

Influence of fetal acidemia on fetal heart rate analyzed by computerized cardiotocography in pregnancies with placental insufficiency

Objective: To determine the influence of fetal acidemia on fetal heart rate (FHR) parameters analyzed by computerized cardiotocography (cCTG) in pregnancies with placental insufficiency.Methods: This was a cross-sectional study of 46 pregnancies with placental insufficiency between 26 and 34 weeks gestation by abnormal umbilical artery Doppler [pulsatility index (PI) > 95th percentile].Results: Twenty fetuses had acidemia at birth, pH < 7.20 (43.5%) and 26 had normal pH (56.5%). In the analysis of FHR parameters, fetal movements (FM) per hour was significantly lower in the group with acidemia (median = 2) when compared with the group with normal pH (median = 15, p = 0.019). The values of pH correlated positively with FM (ρ = 0.35; p = 0.019, 95% CI: 0.061 to 0.577) and basal FHR (ρ = 0.37, p = 0,011, 95% CI: 0.090 to 0.597) and negatively with the ductus venosus (DV) PI for veins (PIV) z-score (ρ = −0.31, p = 0,036, 95% CI: −0.550 to −0.021). Logistic regression showed that the DV PIV z-score (p = 0.0232) and basal FHR (p = 0.0401) were independent variables associated with acidemia at birth.Conclusions: The present results suggest that cCTG parameters may be useful in the management of cases with early onset placental insufficiency in association with Doppler velocimetry assessment, and that basal FHR and DV-PIV are most clearly in association with acidemia at birth.

Adrenal insufficiency in association with congenital nephrotic syndrome: a case report

Adrenal disorders in patients with congenital nephrotic syndrome (CNS) have seldom been reported, and the mechanisms that could explain this association are not known. The follow-up of a male infant diagnosed with CNS and primary adrenal insufficiency in his first year of life is the object of this paper.

Concomitant renal insufficiency and diabetes mellitus as prognostic factors for acute myocardial infarction

BackgroundDiabetes mellitus and renal dysfunction are prognostic factors after acute myocardial infarction (AMI). However, few studies have assessed the effects of renal insufficiency in association with diabetes in the context of AMI. Here, we investigated the clinical outcomes according to the concomitance of renal dysfunction and diabetes mellitus in patients with AMI.MethodsFrom November 2005 to August 2008, 9905 patients (63 ± 13 years; 70% men) with AMI were enrolled in a nationwide prospective Korea Acute Myocardial Infarction Registry (KAMIR) and were categorized into 4 groups: Group I (n = 5700) had neither diabetes nor renal insufficiency (glomerular filtration rate [GFR] ≥ 60 ml/min/1.73 m2), Group II (n = 1730) had diabetes but no renal insufficiency, Group III (n = 1431) had no diabetes but renal insufficiency, and Group IV (n = 1044) had both diabetes and renal insufficiency. The primary endpoints were major adverse cardiac events (MACE), including a composite of all cause-of-death, myocardial infarction, target lesion revascularization, and coronary artery bypass graft after 1-year clinical follow-up.ResultsPrimary endpoints occurred in 1804 (18.2%) patients. There were significant differences in composite MACE among the 4 groups (Group I, 12.5%; Group II, 15.7%; Group III, 30.5%; Group IV, 36.5%; p < 0.001). In a Cox proportional hazards model, after adjusting for multiple covariates, the 1-year mortality increased stepwise from Group III to IV as compared with Group I (hazard ratio [HR], 1.96; 95% confidence interval [CI], 1.34-2.86; p = 0.001; and HR, 2.42; 95% CI, 1.62-3.62; p < 0.001, respectively). However, Kaplan-Meier analysis showed no significant difference in probability of death at 1 year between Group III and IV (p = 0.288).ConclusionsRenal insufficiency, especially in association with diabetes, is associated with the occurrence of composite MACE and indicates poor prognosis in patients with AMI. Categorization of patients with diabetes and/or renal insufficiency provides valuable information for early-risk stratification of AMI patients.

Paricalcitol aggravates perivascular fibrosis in rats with renal insufficiency and low calcitriol

Cardiovascular complications are a major problem in chronic renal failure. We examined the effects of plasma calcium, phosphate, parathyroid hormone (PTH), and calcitriol on cardiac morphology in 5/6 nephrectomized rats. Fifteen weeks after nephrectomy rats were given a control diet, high-calcium or -phosphorus diet, or given paricalcitol treatment for 12 weeks. Sham-operated rats were on a control diet. Blood pressure, plasma phosphate, and PTH were increased, while the creatinine clearance was reduced in remnant kidney rats. Phosphate and PTH were further elevated by the high-phosphate diet but suppressed by the high-calcium diet, while paricalcitol reduced PTH without influencing phosphate or calcium. The high-calcium diet increased, while the high-phosphate diet reduced plasma calcium. Plasma calcitriol was significantly reduced in other remnant kidney groups, but further decreased after paricalcitol. Cardiac perivascular fibrosis and connective tissue growth factor were significantly increased in the remnant kidney groups, and further increased in paricalcitol-treated rats. Hence, regardless of the calcium, phosphate, or PTH levels, cardiac perivascular fibrosis and connective tissue growth factor increase in rats with renal insufficiency in association with low calcitriol. Possible explanations are that aggravated perivascular fibrosis after paricalcitol in renal insufficiency may be due to further suppression of calcitriol, or to a direct effect of the vitamin D analog.

Prevalence and management of ovarian venous insufficiency in the presence of leg venous insufficiency

The correlation between ovarian venous insufficiency and lower limb venous insufficiency remains poorly understood. Clinically, incompetent ovarian veins in association with lower extremity varicose veins are suspected when leg varicose veins are found in atypical distributions. Such distributions include upper lateral or posterior thigh, on the buttocks, crossing the inguinal ligament, and also in the vulval or perineal regions. The aim of this study was to determine the prevalence of ovarian venous insufficiency in those with clinically suspicious varicose veins, and to assess the effectiveness of ovarian venous embolization/ligation in treating this condition. Between June 2001 and December 2004, 424 female patients with lower limb superficial venous insufficiency were seen by a single vascular surgeon. These patients were clinically assessed, and those with atypical varicose veins were investigated with venous duplex examination and magnetic resonance imaging (MRI) venography. Patients with proven ovarian venous insufficiency were offered venography with a view to embolization or laparoscopic ligation. A total of seven patients were clinically suspected of having ovarian venous insufficiency, of which three had recurrent varicose veins (42.9%). Of these, six were confirmed on MRI venography with the left side being more affected than the right; one of them had an occluded vena cava, three were treated by embolization, and two had laparoscopic ligation. The prevalence of clinically detectable ovarian venous insufficiency in association with lower extremity varicose veins is in the region of 1.65%. Compared with the estimated prevalence of incidental ovarian venous insufficiency of 10-47%, this suggests that only a minority of incompetent ovarian veins will present with clinically detectable lower limb venous insufficiency. In our opinion, patients with signs suggestive of ovarian venous insufficiency in association with lower limb venous insufficiency should have their ovarian insufficiency controlled prior to embarking on limb venous surgery.

Novel compound heterozygote mutations (H234Q/R1206X) of the ADAMTS13 gene in an adult patient with Upshaw–Schulman syndrome showing predominant episodes of repeated acute renal failure

Unlike acquired thrombotic thrombocytopenic purpura or haemolytic uraemic syndrome, which are often intractable, thrombotic microangiopathy in patients with Upshaw-Schulman syndrome (USS)--a congenital deficiency of von Willebrand factor-cleaving protease (ADAMTS13) activity--responds very well to plasma infusion and does not even require plasma exchange. However, the symptoms significantly vary in each individual and thus clinicians often overlook this diagnosis. A 31-year-old adult male patient with thrombotic microangiopathy, which was complicated with repeated episodes of acute renal failure, is reported. We suspected that the patient had USS and performed assays of ADAMT13 activity and its inhibitor, followed by ADAMTS13 gene analysis of the patient and his parents. The patient had extremely low ADAMTS13 activity and has no inhibitors of ADAMTS13. Through an ADAMTS13 gene analysis of this family, we found two novel mutations responsible for the disease: a missense mutation in exon 7 [702 C --> A (H234Q)] from the father and a nonsense mutation in exon 26 [3616 C --> T (R1206X)] from the mother. Our experience appears to indicate the importance of assays of ADAMTS13 activity and its inhibitor in patients who have episodes of renal insufficiency in association with thrombotic microangiopathy, for diagnosis and choice of treatment.

Neutrophil and macrophage activation and anaphylatoxin formation in orthotopic liver transplantation without the use of veno-venous bypass.

Activation of neutrophils and activation of complement may be an aetiologic factor behind circulatory insufficiency in association with reperfusion of the grafted liver. Neutrophil and macrophage activation (determined as PMN elastase and neopterin release) and complement activation were evaluated in 15 consecutive patients undergoing orthotopic liver transplantation without the use of veno-venous bypass. The PMN elastase concentrations were increased at the end of the anhepatic phase, 2, 5 and 30 min after start of reperfusion and 6 and 24 h postoperatively. There were significantly higher PMN elastase concentrations in patients with circulatory instability (postreperfusion syndrome) compared with those without postreperfusion syndrome. The neopterin concentration was increased 2 min after the start of reperfusion and remained elevated until 6 h postoperatively. The plasma complement C3a concentrations were increased at the end of the anhepatic phase and 2, 5 and 30 min after the start of reperfusion. The plasma C3a levels were higher in patients with postreperfusion syndrome compared to those without. Activation of neutrophils and macrophages and of the complement cascade with the formation of biologically active substances may be one explanation for the circulatory instability often seen in patients undergoing orthotopic liver transplantation.

Fetal and transient neonatal right heart dilatation with severe tricuspid valve insufficiency in association with abnormally S‐shaped kinking of the ductus arteriosus

Alterations of the ductus arteriosus, e.g. occlusion, constriction or aneurysm, can influence the fetal and neonatal circulation. A case of fetal right atrial and ventricular dilatation, severe tricuspid valve insufficiency and transient pulmonary valve regurgitation at 32 weeks of gestation is reported. Structural heart defects and extracardiac diseases (e.g. high output cardiac failure in arteriovenous shunts and anemia) were excluded by Doppler echocardiography and fetal blood sampling. An abnormally S-shaped kinking of the ductus arteriosus with increased systolic and diastolic flow velocities in the distal part was demonstrated. At 35 weeks' gestation, Cesarean section was performed, because of increased tricuspid valve insufficiency, and a healthy boy was born. After birth, right heart dilatation and tricuspid valve insufficiency decreased. The cardiac changes in relation to the anatomical abnormality of the ductus arteriosus are discussed.

Rapid onset of blindness due to meningeal carcinomatosis from an oesophageal adenocarcinoma

We report a 49 year old man who presented with left leg weakness, frontal headache, impaired concentration and dysphagia. He was thought to have a benign oesophageal stricture on barium swallow and oesophagoscopy though this was not initially biopsied. During admission his vision deteriorated so that he could only recognize bright light. Repeat oesophagoscopy demonstrated an oesophageal adenocarcinoma. The diagnosis of meningeal carcinomatosis was confirmed at post-mortem. Sudden bilateral blindness is a common feature of meningeal carcinomatosis but, as this is rare, it is not commonly considered in the differential diagnosis of visual loss. The visual loss can be explained by vascular insufficiency in association with tumour cuffing of the subarachnoid space of the optic nerve sheath, by neuronal toxins, or other, as yet unknown, mechanisms.

Doppler assessment of the pulsatility index of the middle cerebral artery during constriction of the fetal ductus arteriosus after indomethacin therapy

To investigate the effects of constriction of the ductus arteriosus on the pulsatility index of the middle cerebral artery, maximum velocity waveforms were obtained in 13 fetuses (one set of twins) whose mothers were treated with indomethacin for preterm labor (n = 9) or polyhydramnios (n = 3). Eleven of the fetuses manifested ductal constriction within 48 hours of therapy, whereas two fetuses had constriction after 1 week of therapy. Six of the 13 fetuses also manifested tricuspid insufficiency in association with constriction of the ductus arteriosus. All abnormal cardiac changes resolved in utero after discontinuation of indomethacin. No difference in the pulsatility index values of the middle cerebral artery was observed in the fetuses with ductal constriction but without tricuspid regurgitation (n = 7) when the values were compared with those obtained in absence of ductal constriction (1.87 +/- 0.37 vs. 1.88 +/- 0.33). In the fetuses that manifested both ductal constriction and tricuspid insufficiency (n = 6), the pulsatility index values in the middle cerebral artery were significantly lower in the presence of ductal constriction when compared with the values obtained in the absence of ductal constriction (2.22 +/- 0.26 vs. 1.57 +/- 0.34). These results indicate that a response to indomethacin sufficient to cause both ductal constriction and tricuspid insufficiency decreases the pulsatility index of the middle cerebral artery.

Survival in atrioventricular discordance.

Limited information is available concerning the long-term survival of patients with atrioventricular discordance, ventriculoarterial discordance, and two ventricles (corrected transposition). The long-term follow-up of 107 patients examined at the Mayo Clinic over a 30-year period between 1951 and 1981 was reviewed. Overall survival from the date of Mayo Clinic diagnosis was 70% at five years and 64% at ten years. Associated variables were analyzed for their effect on survival including sex, age at diagnosis, presence of ventricular septal defect, pulmonary stenosis, dextrocardia, left atrioventricular valve insufficiency, and complete heart block. There was no significant difference between those with and without a ventricular septal defect (VSD). Pulmonary stenosis was protective when a VSD was present but was not a significant predictor of long-term survival. The only variable that consistently correlated with decreased survival was left atrioventricular valve insufficiency (p less than 0.04 for univariate and stepwise Cox, and p = 0.08 for multivariate analysis). A logistic model for survival after open-heart surgery failed to identify any significant variable. We conclude that the presence of left atrioventricular valve insufficiency in association with atrioventricular discordance significantly alters the long-term outcome. Atrioventricular valve replacement should be considered in such patients when insufficiency becomes hemodynamically significant.

Management of the tortuous internal carotid artery

The tortuous internal carotid artery as the basis of cerebral vascular insufficiency is a controversial entity, as such lesions frequently occur without neurologic symptoms. However, some tortuous vessels are associated with significant carotid atherosclerotic disease requiring a specific surgical approach. This formed the basis of our report. Symptoms of cerebrovascular insufficiency in association with a tortuous internal carotid artery was encountered in 27 of 853 carotid endarterectomies over a 22 year period. Stenosis of hemodynamic significance was noted in nine patients and irregularities or ulceration were noted in the rest. With the standard technique of carotid endarterectomy, removal of plaque from a tortuous vessel leads to loss of the stenting effect provided by the plaque such that angulation occlusion is likely to occur. The optimal reconstruction in our experience is eversion endarterectomy by transection and lower reimplantation of the internal carotid artery on the common carotid artery. Our experience with 17 such procedures in 15 patients has been presented.

Nephrotic syndrome and renal insufficiency in association with amyloidosis: A correlation between structure and function

The following results were obtained by correlating light and electron microscopic findings from 85 cases of glomerular renal amyloidosis with clinical parameters: 1. Amyloid masses deposited in the glomeruli do not represent an effective filtration barrier, i.e., the formation of primary urine is not significantly influenced even by extensive amyloid masses in the glomeruli; protein retention is first observed when the glomerular capillaries are almost totally obliterated. 2. Once the nephrotic syndrome has developed in association with glomerular renal amyloidosis, it shows no tendency for remittance, despite progressing renal insufficiency. 3. The reason for this persistance of the nephrotic syndrome, despite increasing renal insufficiency, is a progressive reduction in the capacity of the tubules for protein reabsorption in the presence of increasing interstitial fibrosis of the kidney. 4. Interstitial fibrosis of the kidney cortex leads to increasing impairment in the oxygen and energy supply to the tubule cells together with considerable functional deterioration which, in addition to other metabolic disturbances, also results in a reduced capacity for protein reabsorption.

Aortic insufficiency in association with juvenile ankylosing spondylitis

vitro m e a s u r e m e n t o f 22Na influx into red blood cells will establish the diagnosis o f Liddle syndrome pr ior to therapy in pat ients who have hyper tens ion and hypokalemia wi thou t demons t r ab l e renal or adrenal pathology. The demons t r a t i on o f increased t ranspor t o f sodium into erythrocytes and the absence of mineralocor t icoid excess suppor t the view that Liddle syndrome is due to a general ized m e m b r a n e abnormal i ty .

Persistent pulmonary hypertension of the newborn infant

Persistent pulmonary hypertension of the newborn infant can be difficult to distinguish from other cardiopulmonary causes of cyanosis during the newborn period. Infants with PPHN have cyanosis, tachypnea, acidemia, normal pulmonary parenchymal markings on the chest radiography, and anatomically normal hearts. We have identified and treated 11 infants and have noted several signs and symptoms not previously emphasized. These are cineangiocardiographic evidence of atrioventricular valve insufficiency in association with systolic murmurs and slow ventricular emptying, apnea, hypocalcemia, only a small rise in abdominal aortic blood oxygen tension during breathing of 100% oxygen, and no response to continuous positive airway pressure. Right-to-left shunting through the patent ductus arteriosus was documented in nine infants: in all six of those in whom simultaneous temporal and abdominal aortic blood oxygen tension measurements were made; in three by means of cardiac catheterization. Ten infants survived after variable courses and treatments which makes it difficult to ascribe improvement to any one therapy. The distinct increase in blood oxygen tension with tolazoline HCl and curare in some instances is discussed.

Effect of compromising right ventricular function in left ventricular failure by means of interatrial and other shunts: Right ventricular success versus left ventricular failure

The immediate advantages of a left to right interatrial shunt in the presence of a high left atrial pressure are evident from the experiments presented. The resultant reduction in left atrial pressure is shown to be proportional to the size of the shunt and to the initial height of left atrial pressure. When the initial left atrial pressure was high, this reduction in pressure was along the plateau of a ventricular function curve and was, therefore, associated with only a small drop in systemic flow. When shunt flow averaged 48.9 ml./min. per kg. and the ratio of shunt flow to systemic flow was 1.16, average left atrial pressure fell 30.7 per cent, from 18.1 mm. Hg to 12.5 mm. Hg; systemic flow fell only 5.3 per cent. Evidence that the right ventricle is well able to tolerate the increased flow burden is provided by the minor rise in right atrial pressure, which never exceeded 2 mm. Hg. A shunt from the pulmonary artery to the right atrium is shown to be similar in action, with a somewhat smaller effect on left atrial pressure for reasons which are discussed. The results reported are of acute experiments, and provide no information on the possible long-term benefits of a left to right interatrial shunt in left ventricular failure. However, situations analogous to this experimental preparation occur in nature in the form of Lutembacher's syndrome, the syndrome of partial anomalous pulmonary venous drainage with mitral stenosis, and tricuspid stenosis or insufficiency in association with left-sided heart lesions. These clinical syndromes are reviewed and the beneficial effect of the intracardiac or extracardiac shunt on high left atrial pressure discussed. It is further suggested that these syndromes support the view that the results of the studies presented are not mere artifacts of acute experiments but that a true reduction of left atrial pressure is likely to persist after blood pressures and flows have equilibrated. Finally, a mechanism of action of these shunts is postulated which relates the left atrial pressure to the characteristics of the right ventricular pump.

Mitral insufficiency in association with the syndrome of idiopathic hypercalcema of infancy

of the 4 patients with SSPE (Table I I ) . Normal levels for individuals in the age group are < 8 to 64 with the complement fixation method and < 8 to 256 with the hemagglutination inhibition technique. None of the parents or siblings of these patients showed unusually elevated titers with either the CF or H I methods. Three pairs of siblings in 3 separate families had clinical measles during the same year as the affected patients. One sibling had live measles immunization at age 3 years.

Pancreatic Insufficiency as the Presenting Feature of Hyperparathyroidism

The fifth reported case of exocrine pancreatic insufficiency in association with a parathyroid adenoma is described. The patient presented with severe malabsorption syndrome in which diarrhea, weight loss, steatorrhea, and hypoproteinemia were prominent. She had no symptoms of pancreatitis, renal disease, or bone disease. The malabsorption syndrome was effectively treated by oral administration of pancreatic enzymes. Subsequently, the presence of hypercalcemia and increased alkaline phosphatase activity in the serum led to the discovery of a parathyroid adenoma. After removal of the adenoma, the patient became euparathyroid. A secretin test of pancreatic function was still abnormal 2 months after surgery, but the degree of steatorrhea may have been somewhat less than it had been before surgery. It is suggested that in this patient hyperparathyroidism may have led to chronic pancreatitis and consequent injury and insufficiency. Hyperparathyroidism should be considered among the causes of pancreatic insufficiency.

Adrenocortical insufficiency in infants with the adrenogenital syndrome: A clinical and pathologic study of four cases

<h2>Summary</h2> This paper deals with clinical, chemical, and pathologic observations on fourinfants who presented the picture of adrenal cortical insufficiency in association with bilateral adrenal hypoplasia as shown at autopsy. The condition was found in 0.52 per cent of autopsies done on infants under1 year of age, indicating a higher incidence than is suggested by the literature. The familial pattern which was apparent in two of our patients remains unexplained and requires endocrinologic studies of the mothers of such infants. In our own group and in all eases thus far reported the patients were either female pseudohermaphrodites with a characteristic deformity of the genitalia or else true males. In the latter, external manifestations of virilism are not apparent in early infancy. The presenting clinical features were gastrointestinal symptoms, especiallydiarrhea, vomiting, and dehydration. Serial chemical studies performed in two cases showed a lowering of the"total base," bicarbonate and chloride values in the plasma. The blood sugar levels were found normal in three patients. Anatomic and histologic examination of the adrenals showed diffuse hyperplasia of the cortex with apparent replacement of the zona glomerulosa by eosinophilic cells which often contained fuchsinophil granules. A correlation of these findings with the clinical manifestations is suggested. The physiologic disturbance appears to be one of overproduction with respect to N hormone and underproduction of substances regulating salt and water metabolism. The apparently intact carbohydrate metabolism differentiates these patients from those with true Addison's disease as regards the manifestations of adrenal insufficiency. Further studies of the carbohydrate metabolism in such patients are needed. It is suggested that the administration of DOCA and salt may be a complete substitution therapy in patients exhibiting this syndrome.