Long-term follow-up results and treatment outcomes of children and adults with resistance to thyroid hormone alpha.

PURPOSE: Resistance to thyroid hormone alpha (RTHα) is a rare entity and has no specific treatment. To date, mostly levothyroxine has been used, but there is a lack of knowledge about the long-term outcomes of this treatment. We aimed to evaluate the long-term follow-up results and treatment outcomes of children and their parents diagnosed with RTHα. METHODS: Four children [the median (minimum-maximum) age at diagnosis, 4.5 (1.4-9.5) years] and three adults [age at diagnosis, 31.7 (28.0-35.3) years] from two families were included in the study, who had RTHα and followed up between 2014 and 2021. RESULTS: The median duration of treatment was 6.7 (5.9-8.0) years, and the levothyroxine dose at the final visit was 1.4 (1.2-2.2) and 1.9 (1.2-2.4) mcg/kg/day for adults and pediatric patients, respectively. Treatment ameliorated constipation in all patients with this complaint (n = 5). Normal mental functions were achieved and IQ scores improved in most children except one (age at diagnosis, 9.5 years). At the final visit, creatine kinase levels relative to the reference upper limit were significantly lower compared to the pre-treatment ratios [0.9 (0.2-1.3) vs. 1.3 (0.5-1.6), p = 0.028]. Anemia was present in five patients at diagnosis, which resolved in one adult patient but occurred in one child despite treatment (p = 0.999). A minimal pericardial effusion persisted in one pediatric patient. CONCLUSIONS: We demonstrated that constipation was ameliorated, neuromotor development of some children was improved, and creatine kinase levels were diminished with levothyroxine treatment in patients with RTHα, while some features including anemia did not resolve.

Early and midterm results of the retrograde transpopliteal approach as the first-line treatment for total occlusions of iliofemoral arteries.

OBJECTIVE: This study aims to present our early and midterm results regarding the use of the retrograde popliteal artery approach as the first-line treatment for patients with total occlusions of the iliac or femoropopliteal arteries. PATIENTS AND METHODS: Between July 2017 and July 2019, 84 patients underwent transpopliteal retrograde subintimal recanalization for iliac and femoral artery occlusive disease. RESULTS: The procedure was technically successful in 92.9% of the patients and had a complication rate of 5.95%. Complications, including stent thrombosis, dissection, and rupture, were treated successfully. No hematomas were observed at the puncture site. The primary patency rates at 6, 12, and 18 months were 86.9%, 82.1%, and 77%, respectively. CONCLUSIONS: With respectable early and midterm results, the retrograde popliteal artery approach can be considered a primary treatment option for iliac or femoropopliteal arteries' recanalization in selected patients.

Investigating the effects of two different pump heads (Centrifugal vs. Roller Pump) on hematological and immunological mechanisms.

BACKGROUND AND AIM: Technological developments and enhancement of knowledge level enable heart surgery with low mortality rates in most centers. On the other hand, increased systemic inflammatory response against cardiopulmonary bypass (CPB) plays a critical role in the development of postoperative complications. We aimed to compare the effects of centrifugal pump where it is claimed that blood is exposed to minimal trauma and roller pump techniques on inflammatory response and oxidant status during CPB. MATERIALS AND METHODS: : A total of 40 patients, who had coronary artery disease and underwent coronary artery bypass graft (CABG) surgery using either roller or centrifugal pump between June 2012 and June 2013 were enrolled in this study. Patients over 40 years old and without any known immunologic, infectious, or inflammatory incidents and hematological problems for the past 6 months were included in the study. Two study groups (Group R: roller pump group and Group C: centrifugal pump group) were created. During CABG surgery tumor necrosis factor (TNF) alpha, interleukin (IL)-6, IL-8, superoxide dismutase (SOD), catalase (CAT), and nitric oxide levels were measured before and after CPB. RESULTS: TNF alpha, IL-6, and IL-8 levels measured before and after CPB were found to be similar between groups. SOD, CAT and Nitric oxide levels were also similar between groups. After the CPB period, glutathione peroxidase enzyme activities in Group R measured after CPB were significantly lower than those measured in Group C. The platelet-activating factor (PAF) levels before CPB usage period were same in both groups, where PAF levels after CPB were found to be significantly higher in roller pump group than centrifugal pump group. At inter-group comparisons, the levels of PAF were same at each group before and after CPB. CONCLUSION: The study findings indicate that usage of the centrifugal pump does not have a clear superiority in terms of the effects on inflammatory response and oxidant status during CPB when compared to roller pump. Nevertheless, we believe that our results should be supported by further clinical and experimental studies.

Effects of kefir on ischemia-reperfusion injury.

OBJECTIVE: We aimed to investigate the effect of kefir on Ischemia-Reperfusion (I/R) injury on rats. MATERIALS AND METHODS: 24 male Sprague-Dawley rats between 250-350 g were selected. Rats were divided into three groups, and there were eight rats in each group. Rats were fed for 60 days. All of the rats were fed with the same diet for the first 30 days. In the second thirty days, kefir [10 cc/kg/day body weight (2 x 109 cfu/kg/day)] was added to the diet of the study group by gavage method. In all groups, lung and kidney tissues were removed after the procedure and rats were sacrificed. The biochemical and histopathological changes were observed in the lung and kidney within the samples. Serum urea, creatinine and tumor necrosis factor (TNF-α) were determined. RESULTS: Kefir + I/R groups was compared with I/R groups, a significant decrease (p < 0.05) was seen in Lipid peroxidation (MDA) levels of lung and renal tissues. Superoxide dismutase (SOD), Catalase (CAT) and Glutathione peroxidase (GSH-Px) activities of lung and kidney tissues decreased in I/R groups (p < 0.05). The enzyme activities in Kefir + I/R groups of renal tissues were significantly (p < 0.05) higher than I/R, not significantly different in lung tissues (p < 0.05). Kefir reduced the levels of serum urea, creatinine and TNF-α significantly. CONCLUSIONS:   This would be useful in this model against ischemia/reperfusion, and shows the protective effect of kefir in tissue and serum functions.

Effects of pleural opening on respiratory function tests in cardiac surgery: a prospective study.

PURPOSE: To show the effects on lung function of the opening pleura in patients undergoing cardiac surgery. SUBJECTS AND METHODS: 66 patients were included. Patients were allocated into two groups. In group 1 (n=21) pleura was intact, in group 2 (n=45) pleura was opened. Both groups were compared prospectively in terms of preoperative and on the post-operative 5th day pulmonary function tests (PFT), preoperative, postoperative first and fifth day arterial blood gas analysis, preoperative and postoperative first day mixt venous oxygen saturation, bleeding, operation periods, pulmonary complications, intensive care and hospital stay period and mortality. RESULTS: There was significant decrease in all PFT indicators on 5th post-operative day in group 2 (p < 0.01). Although there was a significant decrease in FEV1 on 5th post-operative day in group 1 (p < 0.001), other pulmonary functions parameters were not change significantly (p > 0.025). In group 2 much more decline in pulmonary function test parameters than group 1 were observed (p < 0.05). There was not statistically significant difference in blood gas analysis and mixed venous oxygen saturation values in group 1 (p > 0.05). But in group 2 except pH and PaCO2, other blood gas measurements were significantly decreased on the postoperative first and fifth day (p < 0.008). In group 2 except pH and PCO2, other parameters were less than the other Group (p < 0.01). The drained amount was still significantly higher in group 2 (p < 0.001). The frequency of the revision due to bleeding was observed much more in group 2. CONCLUSIONS: Protection of the integrity of pleura may have positive effects on pulmonary functions in cardiac surgery.

Technetium-99m hexamethyl propylenamine oxime lung clearance in the estimation of pulmonary hypertension in congenital heart disease: A preliminary comparative study with cardiac catheterization and pathology.

Thirty-six patients ranging in age from 7 months to 15 years and weighing from 5300 g to 49 kg (24 undergoing corrective surgery and 12 cases with reversed shunt and no operation) underwent technetium 99m hexamethyl propylenamine oxime (Tc-99m HMPAO) lung clearance study and the results were compared with catheterization and pathology. Patients were allocated into three groups with respect to pathological grading (Heath-Edwards' classification) and the results were correlated on the basis of pathology. In group I (grades I and II), Pearson correlation coefficient was 0.86 with pulmonary artery pressure (PAP), pulmonary vascular resistance (PVR), and Tc-99m HMPAO lung clearance (t1/2). Pearson correlation coefficients were 0. 863 and 0.88 in the second (grade III) and third group (with reversed shunt and no operation). There were statistically significant differences among the groups with respect to PAP, PVR, or t1/2. The results of radionuclide study (t1/2) were very well correlated within the groups with respect to hemodynamic parameters (PAP and PVR). Tc-99m HMPAO has potential as a highly sensitive indicator for detecting early and minimal microvascular lung injuries, and it may reflect accurate lung clearance and retention enabling an estimation of the state of pulmonary hypertension.

Fibro-osseous lesion of rib.

A 3 x 4 cm homogeneous opacity was incidentally detected over the first rib at routine chest radiography. Radionuclide bone scan identified a hot spot, not excluding malignancy and excision of the rib revealed fibro-osseous lesion. A review of the literature showed 12 cases of this extremely rare lesion.

The dose-dependent effects of L-carnitine in myocardial protection in normothermic ischemia.

L-Carnitine has been shown to improve the post-ischemic recovery of myocardial function and metabolic measurements that are reduced in the course of ischemia and reperfusion of the heart. In this study we used 40 male guinea-pigs in order to determine if the effect of L-carnitine which is used in the protection of the post-ischemic reperfused heart, is dose-dependent or not. All harvested hearts were perfused for 30 min on modified Langendorf apparatus with oxygenized Krebs-Henseleit solution. After this period, in (n = 10), 5 mmol and 10 mmol (group B, n = 10) of L-carnitine were added into a Krebs-Henseleit solution. After 20 min, perfusion was complete and the hearts were then exposed to normothermic ischemia for 20 minutes. Following the ischemia, hearts were reperfused with the same solutions for 30 min. In group C (n = 10), 10 mmol of L-carnitine was added into the solution at the post-ischemic reperfusion step. In the control group, the same procedures were performed without using L-carnitine. Matching was done according to the contractile force of the heart rate and the levels of malondialdehyde and adenosine deaminase. When 10 mmol L-carnitine was added into the perfusion solutions at the pre-ischemic period, the best results were obtained and myocardial damage was much less than the control group. The protective effects of L-carnitine in normothermic ischemia is dose-dependent and it must be given at the pre-ischemic period.

Mitral and aortic valve replacement using fresh unstented pulmonary and aortic homografts.

The use of prosthetic valves carries a high incidence of complications including thromboembolism, hemolysis, infection, impaired hemodynamic function and mechanical failure. Unstented homograft valves provide a good quality of life, particularly in terms of a reasonable freedom from these complications. This paper presents the first simultaneous replacement of the mitral and aortic valves using fresh unstented pulmonary and aortic homografts collected from the same donor. We believe that this technique will be especially useful in developing countries facing financial difficulties and problems with patient education.

The effects of thyroid hormones on the heart following global ischemia. A clinical and experimental study.

Cardiopulmonary bypass is associated with a reduction in plasma thyroid hormone concentrations in patients undergoing cardiac surgery. However, studies of the effects of cardiopulmonary bypass on thyroid function are limited and many studies report conflicting data concerning only the period of cardiopulmonary bypass. In this study, we tried to clinically determine the effects of cardiopulmonary bypass on concentrations of thyroid hormones by comprehensive thyroid function tests in 10 patients before and after surgery, and observed the benefits of triiodothyronine supplementation after global ischemia on myocardial function experimentally in guinea pigs. In patients undergoing surgery, concentrations of total triiodothyronine and free triiodothyronine decreased progressively on the institution of cardiopulmonary bypass and remained below normal levels until 24 hours postoperatively. In the guinea pig hearts studied in a Langendorf perfusion apparatus, T3 supplementation enhanced percentage recovery of ventricular contractile force, heart work and heart rate with respect to other groups receiving no T3 supplementation or T3 supplementation without any ischemic interval.

Endothelial function of human gastroepiploic artery in comparison with saphenous vein.

OBJECTIVE: Endothelial function is one of the important determinants of patency rate of a graft material used in coronary revascularisation. The aim of this study was to compare the endothelial reactivity of human gastroepiploic artery versus saphenous vein in response to various vasoactive substances. METHODS: Gastroepiploic artery and saphenous vein rings were mounted in an organ bath containing Krebs-Ringer bicarbonate solution aerated with 95% O2 and 5% CO2 at 37 degrees C. Endothelium dependent responses of acetylcholine, histamine, and bradykinin were examined on the precontracted rings of the vessels. The sensitivity of these two graft materials to the potent vasoconstrictor agent endothelin-1 was also compared. RESULTS: Acetylcholine, histamine, and bradykinin caused dose dependent relaxations in saphenous vein and gastroepiploic artery rings. These agonists were more effective in producing endothelium dependent relaxations in the artery than in the vein. Endothelium removal and pretreatment with nitro-arginine abolished the relaxations completely in saphenous vein but not in gastroepiploic artery, depending on the relaxing agent used. On the other hand, both graft materials showed nearly equal sensitivity to endothelin-1 (EC50 values; 3.5 x 10(-9) M in the vein versus 6.4 x 10(-9) M in the artery, p = NS) which was not affected by endothelium removal. CONCLUSIONS: Gastroepiploic artery exhibited more pronounced relaxation and a different response profile to endogenous vasoactive substances than saphenous vein. The demonstration of higher capacity of the artery to release vasoactive substances in response to various endogenous agents shows that gastroepiploic artery may have a better long term patency rate than saphenous vein.

Effect of right atrial appendicectomy on the release of atrial natriuretic peptide in humans.

The present study evaluated the effect of right atrial appendicectomy on the release of atrial natriuretic peptide (ANP) and subsequent changes in postoperative haemodynamics in 20 men undergoing coronary artery bypass graft surgery. The right atrial appendix was removed in ten patients and saved in ten. Serum ANP, sodium levels and urinary sodium excretion were measured before and on days 1, 7 and 30 after surgery. Haemodynamic parameters were monitored before surgery and on day 30. Serum ANP levels fell significantly in patients undergoing appendicectomy (P < 0.05); haemodynamic parameters were unchanged. Hence, appendicectomy reduced serum ANP levels in the short term, though these tended to rise again with time; cardiac function was not affected by lowered levels of serum ANP. Consequently, saving the appendix in right atrial appendicectomy improves natriuresis and may decrease diuretic requirement.

New technique to enlarge the aortic annulus.

A new technique is described to enlarge the aortic annulus and the supraaortic area by using the anterior mitral leaflet. This technique is used when it is necessary to replace both the aortic and mitral valves.

Dipyridamole induced myocardial recovery after global ischemia.

1. An experimental comparative study on isolated guinea pig hearts was carried out to determine the effect of dipyridamole added to the reperfusion solution on myocardial recovery after global ischemia. 2. After 20 min of normothermic ischemia two groups of solutions: (1) Krebs solution; (2) Krebs + dipyridamole 20 micrograms/l (10 experiments in each group) were used for reperfusion. 3. Postischemic myocardial functions (heart rate, ventricular contractility, heart work) and tissue enzymes (CPK-MB, LDH) were compared with their preischemic values. 4. Addition of dipyridamole 20 micrograms/l to reperfusion solution improved postischemic myocardial functions and decreased myocardial injury.

Horseshoe lung with left lung hypoplasia.

Horseshoe lung is an uncommon congenital malformation in which the bases of the right and the left lungs are fused to each other by a narrow isthmus posterior to the cardiac apex. So far 22 cases have been described: most of these were associated with right lung hypoplasia and the scimitar syndrome. A horseshoe lung anomaly with left lung hypoplasia is described.

Comparison of potassium and adenosine cardioplegia with or without verapamil in the isolated guinea pig heart.

1. The cardioprotective effects of adenosine cardioplegia and classical potassium cardioplegia with or without adding verapamil were investigated in isolated guinea pig heart. 2. Four different cardioplegic solutions were used to arrest the hearts which were previously perfused by Krebbs-Henseleit solution. (A) potassium 20 mMol/l. (B) Potassium 20 mMol/l + verapamil 1 micromol/l. (C) Adenosine 10 mMol/l. (D) Adenosine 10 mMol/l + verapamil 1 microgram/l. 3. Both of the adenosine-containing solutions shortened the arrest time and maintained better postischemic recovery according to the potassium cardioplegia. 4. A rapid cardiac arrest was observed when verapamil was added to cardioplegic solutions, on the contrary there was no significant effect on postischemic recovery.

A case of pericardial lipoma diagnosed by noninvasive techniques.

Intracardiac lipomas that consist of mature fat cells with fibrous and myxoid tissues are regarded as rather rare lesions. The diagnosis may frequently be established during surgical intervention or at autopsy. The patient presented is a 23-year-old male who had had no complaints. The lesion had been incidentally detected on x ray examination which had revealed an enlarged heart shadow. His physical examination revealed no abnormality except displacement of the heart to the right and sinus tachycardia. A solid mass derived from the pericardium was observed on CT scans and echocardiography. On CT scans the mass exhibited a density consistent with a lipoma. The patient was operated on and the mass, which was encapsulated and 19 x 17 x 10 cm in size, was removed. Histopathological examination revealed a lipoma.