Acquired cardiac hypertrophy with outflow tract obstruction in a patient with severe Takayasu arteritis

Takayasu arteritis with coronary artery involvement is rare and its association with secondary cardiac hypertrophy with severe outflow tract obstruction is not common. We describe a case of Takayasu arteritis, diagnosed 10 years ago, whose coronary artery involvement and obstructive cardiac hypertrophy are ascertained after our investigations.     

Multidisciplinary Management of Pediatric Hepatoblastoma: A 20-Year Single-Center Experience

Background:Hepatoblastoma is rare cancer that responds well to risk-based chemotherapy, and surgical treatment is needed to achieve complete remission and satisfactory survival rates in hepatoblastoma patients. In this study, we evaluated the clinical features and treatment outcomes of pediatric hepatoblastoma patients treated in our clinic.Methods:Eighteen patients with hepatoblastoma who were treated and followed up in our center between June 1999 and June 2020 were analyzed retrospectively. All patients were evaluated by a multidisciplinary team and managed using a risk-based protocol (SIOPEL-1 and SIOPEL-3).Results:The patients’ mean age at diagnosis was 38.33 ± 52.34 months. Sixteen patients (89%) received neoadjuvant chemotherapy, and 2 patients (11%) who underwent complete mass excision at diagnosis received adjuvant chemotherapy. After neoadjuvant therapy, the tumor was completely resected in 8 patients (45%), while liver transplantation was performed in 6 patients (34%) because complete resection of the tumor was not possible. Two patients died before surgical treatment. One patient relapsed with lung metastasis after salvage chemotherapy. She is alive without disease at 64 months. The mean follow-up time was 59.3 ± 49.8 months; 5-year overall and disease-free survival rates were 88.9% and 80.8%, respectively. The 5-year overall survival rate was 100% for both liver transplant and resected patients, whereas 5-year disease-free survival was lower in transplant patients (75% vs 100%, P < .001).Conclusion:Multidisciplinary follow-up is especially important for patients who may need liver transplantation. Some patients may benefit from new treatment options such as radiofrequency ablation and cyberknife treatment.     

Níveis de Tiol Sérico e Homeostase Tiol/Dissulfeto em Pacientes com Doença Valvar Mitral Reumatismal e em Sujeitos Saudáveis

BackgroundRheumatic mitral valve disease (RMVD) is the most common presentation of rheumatic heart disease (RHD). Inflammation and fibrosis processes also play significant roles in its pathogenesis. Recent studies showed that thiols and thiol-disulfide are promising novel oxidative stress markers.ObjectivesThe present study aimed to evaluate differences in the serum thiol and thiol-disulfide levels in patients with RMVD and the control group.MethodsNinety-two patients with RMVD were enrolled in the study. Fifty-four healthy subjects, age, and gender-matched with the study group, were also included in the study as a control group. This study investigated thiol levels in patients with RMVD and the control group. P-values lower than 0.05 were considered statistically significant.ResultsThe patients with RMVD presented higher systolic pulmonary artery pressure (SPAP) and left atrial (LA) diameter levels than the control group. Native thiol (407±83 μmol/L vs. 297±65 μmol/L, p<0.001) and total thiol (442±82 μmol/L vs. 329±65 μmol/L, p<0.001) levels were higher in the control group. Disulfide (16.7±4.9 μmol/L vs. 14.8±3.7 μmol/L, p=0.011) levels were higher in the group of patients with RMVD. A positive correlation was found between disulfide/native and disulfide/total thiols ratio with SPAP, LA diameter, and MS severity. Disulfide/total thiols ratio was significantly higher in patients with severe MS than with mild to moderate MS patients.ConclusionsTo the best of our knowledge, this is the only study of its kind that has evaluated thiol/disulfide homeostasis as a novel predictor, which was more closely related to RMVD and the severity of MS.     

The Effect of Renal Replacement Therapies on Serum Gastrointestinal System Hormones

Background. The kidney is a major site for the inactivation, degradation, and clearance of a variety of peptide hormones. It has been shown that the uremia increases or decreases gastrointestinal system (GIS) hormones. Moreover, studies investigating the serum GIS hormones levels in chronic renal failure (CRF) were conducted mainly in a particular period of the renal replacement therapy, and the changes caused by continuous ambulatory peritoneal dialysis (CAPD) and hemodialysis (HD) could not be fully demonstrated. In this study, we investigated the effect of CAPD and HD on serum GIS hormones (amylase, lipase, trypsinogen, and gastrin) levels in CRF patients who were diagnosed for the first time. Methods. Serum amylase, lipase, trypsinogen, and gastrin levels were measured in 36 patients who were just diagnosed with CRF, 22 patients with CAPD and 14 patients with HD. GIS hormones of these patients were measured before treatment and three months from the beginning of CAPD and HD treatment. As the control group, 20 normal healthy cases with well-matched age and gender were used. Results. The mean serum amylase, lipase, secretin, and gastrin levels were found meaningfully decreased according to the beginning values at third months of the CAPD and HD treatment. However, they were higher than control group. Conclusion. In patients receiving CAPD or HD as renal replacement therapy, GIS hormone levels were found to be lower, albeit higher than the healthy control group.     

Recurrent obturator abscess with spontaneous expulsion of the mesh after transobturator tape operation

The transobturator tape operation has been the most popular method of SUI surgery worldwide owing to its low complication rate and high success rate. However, erosions and abscesses secondary to transobturator tape have been observed. Here we report a 36-year-old woman referred to our unit with fever, persistent swelling in the left groin, difficulty in walking, and a tape that came through the vagina, 4 years after the transobturator tape operation. She had a history of ischiorectal abscess and rectovaginal fistula. A recurrent obturator abscess with fistula formation and spontaneous expulsion of the mesh was diagnosed. The patient underwent antibiotic therapy, incision through the fistula tract, drainage of the abscess, and removal of the necrotic material. Patients should be informed about risks of erosion and infection and that pain and foul smelling vaginal discharge might be the first signs of severe infectious morbidities after transobturator tape operation.     

Biologic and immunologic effects of preoperative trastuzumab for ductal carcinoma in situ of the breast

BACKGROUND:

Through this study, the authors sought to investigate the biologic and immunologic effects of preoperative trastuzumab in patients with ductal carcinoma in situ (DCIS) of the breast.

METHODS:

Patients with DCIS were enrolled in this open‐label phase 2 trial and tested for HER2. Trastuzumab was given by intravenous infusion (8 mg/kg). The patients then had surgery 14 to 28 days after treatment. Tissue and peripheral blood samples were obtained before therapy and at the time of surgery to examine residual disease and immunologic response.

RESULTS:

Median age of the 69 enrolled patients was 53 years, mean mammographic size of the DCIS lesions was 5.2 ± 1.2 cm, and 24 patients (35%) were found to have HER2 overexpression/amplification (12 received trastuzumab and 12 untreated patients provided tissue for blinded, controlled biomarker analyses). No overt histologic evidence of response was noted. No significant change in mean pretherapy staining for Ki‐67 (44.3 ± 3.4%) and cleaved caspase‐3 (2.6 ± 0.8%) was noted when surgical specimens from drug‐treated patient samples were compared with those not treated. Trastuzumab significantly augmented antibody‐dependent cell mediated cytotoxicity (ADCC) in 100% of patients; this was demonstrated to be mediated through CD56+ degranulating natural killer cells (P < .01). One patient developed a significant anti‐HER2 humoral CD4 T‐cell response.

CONCLUSIONS:

Single‐dose monotherapy with trastuzumab for patients with HER2‐positive DCIS does not result in significant, clinically overt, histologic, antiproliferative, or apoptotic changes, but does result in the ability to mount ADCC mediated through natural killer cells and may also induce T‐cell dependent humoral immunity. Further studies of trastuzumab for DCIS appear warranted. Cancer 2011. © 2010 American Cancer Society.     

Magnetic Resonance Spectroscopy of the Thalamus in Essential Tremor Patients

BACKGROUND AND PURPOSE: Although essential tremor (ET) is one of the most common movement disorders, its pathogenesis remains obscure. The ventral intermediate nucleus of the thalamus (VIM nucleus) is suggested to play an important role in the occurrence of disease. In this study, the authors investigated the presence of biochemical or metabolic alterations in the thalamus of patients with ET using magnetic resonance (MR) spectroscopy. METHODS: The study group included 14 patients with ET who suffered from tremor predominantly in their right arm and 9 healthy controls. All patients and controls were right handed. Following conventional cranial MR imaging, single voxel proton MR spectroscopy of the thalamus involving the VIM nuclei was performed bilaterally in both the patients with ET and controls. Metabolite peaks of choline (Cho), creatine (Cr), and Nacetylaspartate (NAA) were obtained from each spectroscopic volume of interest. The right and left thalamic NAA/Cr and Cho/ Cr ratios were compared first within the patient group and then between the control and patient groups. The differences in age and spectroscopic data between groups were assessed using the Mann-Whitney U test, whereas the comparison within groups between left thalamus and right thalamus was done by the Wilcoxon test. RESULTS: In patients with ET, the NAA/Cr ratio of the right thalamus was found to be significantly higher than the NAA/Cr ratio of the left thalamus (P= .02). However, NAA/Cr and Cho/Cr ratios were found to be similar (P> .05) when we compared the control and patient groups for the right thalamus and then the left thalamus. CONCLUSION: These data present preliminary evidence for metabolic alterations of the contralateral thalamus (namely, low NAA/Cr ratio) in ET patients with predominantly involved right arm. However, the series is small and further data are necessary to clear the subject adequately.     

Replantation after extended dry storage of avulsed permanent incisors: report of a case

Abstract –  A 15-year-old boy lost his maxillary right and left central incisor teeth in a bicycle accident. He was referred to our clinic 1 week after the injury. The crown-root integrities of both the teeth were not damaged. Although the teeth were stored under dry conditions for 1 week, reimplantation of the teeth was planned to retain the teeth in the mouth for as long a period as possible because of the patient's age. Following the debridement and sterilization of root surfaces in 2.5% NaOCl, root canals were prepared and filled with calcium hydroxide. Then, about 2 mm of the apexes were resected to ensure that the roots easily seated in the alveolar socket and the prepared cavities in root ends were obturated with the amalgam. The teeth were placed into their respective sockets and splinted temporarily. The root canal therapy was completed 5 weeks later. Ankylosis was observed radiographically after 10 months. The patient is now 23 years old and he is still able to use both the central incisors functionally. However, there is a pink appearance on the cervical buccal surface of left central incisor because of progressive replacement resorption. In this case, the new treatment plan is to perform a permanent restoration with dental implants following the extraction of both teeth. Even though the long-term prognosis is uncertain, this treatment technique has provided an advantage for the patient in his adolescent period by maintaining the height of alveolar bone and making the provision of an aesthetically acceptable permanent restoration at a later age possible.