Disease Course and Treatment Response of Eosinophilic Gastrointestinal Diseases in Children With Liver Transplantation: Long-Term Follow-Up.

INTRODUCTION: To describe the clinical and laboratory profile, natural course, treatment outcome, and risk factors of posttransplant esophageal and nonesophageal eosinophilic gastrointestinal disorders (EGIDs). METHODS: All children (aged <18 years) who underwent liver transplantation, between 2011 and 2019, in a single transplant center with a follow-up period of 1 year or more posttransplant and with a history of posttransplant endoscopic evaluation were included in this study. RESULTS: During the study period, 89 children met the inclusion criteria. Patients were followed for a median of 8.0 years. A total of 39 (44%) patients were diagnosed with EGID after transplantation. Of these, 29 (33%) had eosinophilic esophagitis (EoE), and 10 (11%) had eosinophilic gastritis, gastroenteritis or enterocolitis. In comparison with the non-EGID group, patients with EGID were younger at transplant (P ≤ 0.0001), transplanted more frequently due to biliary atresia (P ≤ 0.0001), and had higher rates of pretransplant allergy (P = 0.019). In the posttransplant period, they had higher rates of mammalian Target of Rapamycin inhibitor use (P = 0.006), Epstein-Barr virus viremia (P = 0.03), post-transplant lymphoproliferative disease (P = 0.005), and allergen sensitization (P ≤ 0.0001). In regression analysis, young age at transplant, age at diagnosis, pretransplant atopic dermatitis, and post-transplant lymphoproliferative disease were associated with an increased risk of EGID or EoE. Laboratory abnormalities such as anemia (P = 0.007), thrombocytosis (P = 0.012), and hypoalbuminemia (P = 0.031) were more commonly observed in the eosinophilic gastritis, gastroenteritis or enterocolitis group than in the EoE group. Following treatment, most patients had symptomatic resolution at 3 months and histologic resolution at 6 months postdiagnosis. Among the patients who had 5 years of follow-up, none recurred. DISCUSSION: EGID is a common posttransplant diagnosis, which seems to affect patients who are transplanted earlier and who have pretransplant atopy. Posttransplant EGID is responsive to treatment, but as histologic remission occurs after symptomatic resolution, the decision to perform control endoscopy should be delayed.

Live donor liver transplantation for a child presented with severe hepatopulmonary syndrome and nodular liver lesions due to Abernethy malformation.

A 15-year-old boy first presented with severe lung lesions and hypoxia and he was considered as a lung transplant candidate. Upon evaluation, hepatopulmonary syndrome, multiple nodular liver lesions, and Abernethy type 1b malformation were diagnosed. The patient underwent successful right lobe live donor liver transplantation, and all of the symptoms disappeared soon after the transplant. He is currently alive and well with excellent liver and lung functions 4 years after surgery.

Velamentous and Furcate Cord Insertion with Placenta Accreta in an IVF Pregnancy with Unicornuate Uterus.

Velamentous and furcate cord insertion with concomitant placenta accreta is a very rare and life-threatening event of pregnancy for both the mother and the fetus. Obstetricians should be cautious about umbilical cord insertion and placental adherence abnormalities in pregnancies conceived by assisted reproductive technologies (ART) particularly in women with Müllerian anomalies.

Undifferentiated embryonal sarcoma of the liver in an adult patient: case report.

Undifferentiated embryonal sarcoma of the liver is a rare and aggressive tumor in adults, with an unfavorable prognosis. We present a 33-year-old female patient who was admitted with fatigue, weight loss and right upper abdominal pain. Magnetic resonance imaging of the liver revealed a bulky malignant mass with necrotic and cystic center, which nearly occupied the left lobe and invaded the middle-left hepatic vein and left portal vein. The patient was evaluated as inoperable, and in order to determine the histologic diagnosis, a Tru-cut biopsy was performed. Both histopathologic features and the immunocytochemical stainings revealed the diagnosis of hepatic embryonal sarcoma. Since the patient had no chance of surgery due to the advanced stage of the tumor and progressive hepatic failure, a combination chemotherapy was applied. Unfortunately, the patient did not respond to treatment at all and died in the second post-therapy week. Undifferentiated embryonal sarcoma of the liver is a highly chemo-sensitive tumor. Radical resection may be possible after combination chemotherapy in the childhood period, but since the disease is extremely rare in adults, an optimal treatment approach is still unknown.

Prenatal diagnosis of a 22q11 deletion in a second-trimester fetus with conotruncal anomaly, absent thymus and meningomyelocele: Kousseff syndrome.

A 30-year-old nulliparous woman was seen for a detailed ultrasound scan at 20 weeks of gestation. The scan revealed a male fetus with truncus arteriosus, membranous ventricular septal defect, absent thymus and sacral meningomyelocele. A 46,XY karyotype with a 22q11 deletion was detected. The parents chose to terminate the pregnancy. The pathological autopsy showed normal facial structures, minimal ventricular dilatation in the brain and a sacral meningomyelocele. Overlapping toes and a left claw-hand were also noted. An aplastic thymus with absent parathyroid glands was detected. The cardiac examination was consistent with the ultrasound diagnosis. The parental karyotypes were both normal. Kousseff syndrome is caused by a chromosome 22q11 deletion. It includes sacral meningomyelocele and conotruncal heart defects, unlike DiGeorge syndrome. Obstetricians should consider this a not so rare entity when they detect conotruncal abnormalities and a meningomyelocele as part of a 22q11.2 deletion syndrome.

Clinical results of the liquid-based cervical cytology tool, Liqui-PREP, in comparison with conventional smears for detection of squamous cell abnormalities.

OBJECTIVE: To compare the adequacy and efficacy of the liquid-based cytology tool Liqui-PREP (LP) with the conventional Papanicolaou smear (CS) test, for the screening of squamous cell abnormalities. METHODS: Data for 2,000 subjects screened with CS and 4,000 different subjects screened with the LP test were compared. RESULTS: LP showed significant decrease in the rate of unsatisfactory smears (P<0.01) and the detection rate for atypical squamous cells was significantly higher (P<0.01). The rate of low-grade squamous intraepithelial lesion was also higher, but this did not reach statistical significance. The number of high-grade squamous intraepithelial lesions detected was increased with LP, and the histological correlation of LSIL lesions showed a higher positive-predictive value. The coexistence of abnormal colposcopic findings with abnormal smear results was higher for LP (P<0.004). Furthermore, high-risk HPV-DNA detection was found to be increased in atypical LP smears than in normal LP smears. CONCLUSIONS: The liquid-based cytology tool LP detected more squamous cell lesions than CS. Also it reduced the number of unsatisfactory results due to enhanced cell visualization, and improved screening for HPV-DNA.

Adult tethered cord syndrome mimicking lumbar disc disease.

OBJECT: This paper reports four tethered cord cases who initially applied with lumbar disk disease symptoms. MATERIALS AND METHODS: All of them were investigated by magnetic resonance imaging (MRI) and somatosensory evoked potential (SSPE). In two patients, MRI revealed thick and fatty filum terminale, while in remaining two, the filum terminale appeared as normal in thickness. SSEP revealed pathological conduction values in all, and it was the main indicator for surgical sectioning in patients especially with normal MRI investigations. All patients had benefit from the surgical sectioning of the fila terminalia with either thick and fatty or normal appearance. CONCLUSION: A particular patient with lumbar disc disease symptoms having normal MRI should also be investigated for tight filum terminale. If there is no pathological appearance in MRI investigation (both in regard to disc herniation and thick and fatty filum terminale), SSEP investigation should be done to check whether a conduction block or delay is present, indicating cord tethering.

The efficacy of linear polarized polychromatic light on burn wound healing: an experimental study on rats.

We aimed to investigate the questionable effect of linear polarized polychromatic light on burn wound healing in rats. Two deep second-degree burn wounds on the backside of each of 21 Sprague-Dawley rats were created with a standard burning procedure by applying a heated plaque. Burned regions located right dorsolaterally and classified as group I lesions were treated with linear polarized polychromatic light + open dressing + antibacterial pomade, whereas group II lesions were located left dorsolaterally and treated with only open dressing + antibacterial pomade. Macroscopic evaluation was performed for determination of the completed wound closure rate, measurement of burn wound area, and investigation of macroscopic edema, hyperemia, and epithelialization. Histopathological evaluation included monitoring of epithelialization, vascularization, origination of granulation tissue, inflammatory cell response, and total histopathological score on days 7, 14, and 21 after burn creation. Macroscopic evaluation revealed more obvious epithelialization in group I lesions between days 6 and 15. The number of completely closed wounds was higher in group I than in group II on days 16 and 21. The average area of burn wounds was lower from day 5, hyperemia was less on days 2 to 17, and edema was less from day 4 to day 13 in group I lesions. Histopathological evaluation revealed a higher rate of epithelialization on day 7 and higher vascularization occurrence on day 21 in group I lesions. Linear polarized polychromatic light seems to be effective in the treatment of burn wounds and in the promotion of healing. This may be related to linear polarized polychromatic light stimulation of epithelialization and vascularization.

Multifocal mesenchymal hamartoma of the chest wall.

Chest wall hamartomas are extremely rare. Frequently mesenchymal hamartomas are presented as a single mass and contain some primitive mesenchymal elements such as chondroid and trabecular bone structures. A 60-year-old man presented to hospital with chest pain. Thirteen years earlier, his CXR and thoracic CT showed three masses on the right and two masses on the left, but he had not received any treatment thereafter. His CT showed the same masses present 13 years earlier, but they were bigger and right thoracotomy was undertaken. At thoracotomy, two sections of the mass in the right posterior mediastinum and one section of the mass in the right apex were excised. They had an occasional bloody appearance and contained small cystic areas, and some areas were extremely hard. Microscopic examination showed that the lesions consisted of mature adipose tissue, a large number of veins of different diameters and collagen tissue. Besides, primitive mesenchymal elements, lymphoid cell accumulations and trabecular bone structures were seen focally. Bilateral chest wall hamartomas are extremely rare and may be confused with malignancy.

Regenerative hyperplasia of follicular epithelium in chronic lymphocytic thyroiditis.

The thyroid gland is an endocrine organ composed of stable cells. It is well known that regenerative capacity of the thyroid tissue is minimal. Various degrees of morphologic alterations do occur in chronic lymphocytic thyroiditis (CLT), including Hashimoto's thyroiditis. Eighty-five CLT cases were analyzed for these morphologic alterations. Small, irregular, atrophic or hyperplastic thyroid follicles were seen adjacent to the lymphocytic infiltration. There was nuclear enlargement, loss of nuclear polarity in thyrocytes and intrafollicular thyrocyte proliferation in these follicles. We thought that the morphologic alterations in involved follicles could be due to regenerative hyperplasia with increased proliferative activity and basement membrane abnormalities. To examine this hypothesis we investigated Ki-67 and laminin immunoreactivity in the involved follicles adjacent to lymphocytic infiltration areas. The uninvolved follicles were used as controls. Immunopositivity of Ki-67 in involved follicles was significantly higher than that in uninvolved follicles (2.97% +/- 2.16 versus 0.83% +/- 1.63, P < 0.001). Laminin immunostaining indicated the destruction or irregular distribution of basement membrane in involved follicles. We conclude that the increased cell proliferation activity and basement membrane abnormalities in the follicles with morphologic changes adjacent to CLT occur in conjunction with regenerative hyperplasia.

L-carnitine inhibits ethanol-induced gastric mucosal injury in rats.

L-carnitine is a quaternary amine that is essential for the normal oxidation of long-chain fatty acids by mitochondria. It is known that L-carnitine and its derivatives prevent the formation of reactive oxygen species, scavenge free radicals and protect cells from peroxidative stress. Oxygen-derived free radicals and lipid peroxidation products play a critical role in the pathogenesis of ethanol-induced gastric mucosal injury. The aim of the present study was to determine the effect of L-carnitine on lipid peroxidation induced by ethanol in the rat stomach. In our study, gastric mucosal injury was induced by the intragastric administration of 1 ml of absolute ethanol. Test compounds were given to rats by gavage 30 min before the ethanol administration. The animals were killed 60 min after the administration of ethanol. The stomach of each animal was removed. Mucosal damage was evaluated by macroscopic examination, histological analysis and by measurement of lipid peroxidation and glutathione activity. The intragastric administration of ethanol induced hyperemia and hemorrhagic erosions in the rat stomachs. L-carnitine significantly prevented gastric ulcerogenesis induced by ethanol and decreased the ulcer index. Plasma and gastric lipid peroxidation that was increased significantly by ethanol was decreased after treatment with L-carnitine. Ethanol treatment decreased significantly the gastric glutathione levels, and pretreatment with L-carnitine increased them significantly. Based on these data, the beneficial effects of L-carnitine on ethanol-induced gastric mucosal injury may be attributed to its antiperoxidative effects.

Lymphoepithelioma-like carcinoma of the urinary bladder: a case report and discussion of differential diagnosis.

Lymphoepithelioma-like carcinoma (LELC) of the urinary bladder is very rare. It is mandatory to make differential diagnosis among lymphoma, chronic cystitis and LELC because of different therapeutic approach. A bladder tumor was found in a 90-year-old patient suffering from hematuria. After transurethral resection, undifferentiated tumor with prominent lymphoid infiltration was seen on light microscopy. Immunohistochemical examination demonstrated positive staining of tumor cells with cytokeratin (CK), epithelial membrane antigen and CK-20. We presented the case because of its rarity and related literature was reviewed.

Coexistence of Darier's disease and acrokeratosis verruciformis of Hopf.

Darier's disease and acrokeratosis verruciformis are inherited with the same pattern and they share similar clinical properties. We present a patient with both Darier's disease and acrokeratosis verruciformis. The patient had had brownish and skin-colored papules, initially on her face and neck, since the age of 25. Similar lesions had gradually spread to various parts of her body and lastly the dorsal aspect of her feet. On dermatological examination, brownish and skin-colored, 2 to 5 mm, keratotic papules were observed on her face, neck, both axillae, inframammary regions, and dorsa of the hands and feet. The nail examination revealed "V" shaped notches and longitudinal ridges on the fingers of both hands. The biopsy taken from the neck showed typical findings for Darier's disease. The biopsy from the dorsa of the left foot revealed the distinctive structures of acrokeratosis verruciformis. In this article the clinical and histopathological findings of these diseases are reviewed based on our patient.

Chondrosarcoma of the pubis imitating bladder tumor radiologically: a case report.

Chondrosarcoma is a rare tumor that more frequently involves the pelvic bones, the femur, and the humerus. The rareness of the tumor makes the diagnosis difficult. Malignant cartilage tumors typically have an unstable radiographic appearance. In this report we aimed to point out the difficulties of the radiological differentiation of the pelvic chondrosarcoma and bladder tumor.

The potential cardioprotective effects of amifostine in irradiated rats.

PURPOSE: The aim of this study is to determine the cardioprotective efficacy of amifostine. The study consists of researching the relationship between plasma brain natriuretic peptide levels and the electrical and morphologic changes in irradiated rats with or without amifostine. METHODS AND MATERIALS: Sixty Wistar albino rats were divided into 4 groups, and their hearts were given 15 Gy/fraction with (60)Co. In Groups I and II, the rats were killed after 24 hours to detect early effects; in Groups III and IV, the rats were killed 100 days after irradiation to detect late effects. Before irradiation, Groups I and III received 0.9% saline solution, whereas Groups II and IV received amifostine (200 mg/kg). Twenty rats were used as a control group. RESULTS: On the 100th day, mild myocardial degeneration was detected in 5 rats (33%) from Group III (no amifostine). This percentage was statistically different from that of Group IV (treated with amifostine) and the controls (p = 0.042). There was no statistically significant difference between the mean plasma brain natriuretic peptide values of the groups (p > 0.05). There was no significant difference in electrocardiographies between the groups. There was no correlation between continuous variables. CONCLUSION: In the amifostine group (IV) on the 100th day, there was no myocardial degeneration, suggesting that amifostine has a cardioprotective effect.

The fate of intramuscularly injected fat autografts: an experimental study in rabbits.

An experimental study was designed to assess the viability and revascularization of intramuscularly injected fat autografts. For the study, 18 rabbits were divided into two groups. In the first group, fat was injected intramuscularly (12 rabbits). Autologous fat was obtained from the inguinal area and subsequently injected into the thigh muscle. In the second group, physiologic saline was injected intramuscularly to determine the effects of cannulation and pressure on muscle tissue (6 rabbits). Fat autografts were performed on the right side of the animal, and the left side was used as the control. Scintigraphic imaging and histopathologic examination of the limbs were performed after injection of adipose tissue on days 15, 30, 45, 60, 90, and 120. On the technetium-99m ((99m)Tc) hexamethylpropylene amine oxime scintigraphy, whereas similar activity distribution was observed between the left and right thigh on days 15, 30, and 45, there was increased uptake at the right thigh on days 60, 90, and 120. This increased uptake indicates that there is viable fat tissue in this region. Histopathologic evaluation showed that microcysts resulting from degeneration of some adipocytes and inflammatory changes on day 15 additionally increased vascularity and fibrosis in some animals on day 30, as well as fibrosis, microcysties, and focal calcification areas in adipose tissue on day 45 and later. It was observed that adipose tissue survived in more than 50% of the graft area in all the animals. These findings show that fat autografts can survive in muscle tissue with less than 50% fibrotic change.

The effect of low-dose external beam radiation on extraneural scarring after peripheral nerve surgery in rats.

OBJECTIVE: Scar tissue is an inevitable result of peripheral nerve surgery. A variety of substances have been used to prevent epineurial scarring. In this study, the effect of low-dose radiation therapy on epineurial scarring was investigated. METHODS: Seventy-eight male Sprague-Dawley rats were studied. A total of 60 rats were subjected to one of three types of surgical procedure on the sciatic nerve, as follows: Procedure 1, external neurolysis (n = 20); Procedure 2, abrasive injury (n = 20); and Procedure 3, anastomosis (n = 20). On the left sciatic nerves, 700 cGy external beam radiation was administered 24 hours after surgery, and the right sciatic nerves served as a control group (surgery only). Eighteen animals without surgical intervention were used to establish the fibrotic effect of radiotherapy on normal nerves. A neurological examination was performed weekly. Six weeks after surgery, the extent of extraneural scarring was examined by gross microdissection by means of a numerical grading scheme and histological analysis. Cellular density and surface measurements of scar tissue were also evaluated. RESULTS: The dissection around the nerve was easier in rats treated with low-dose radiation compared with the control group. Furthermore, grading scores in both nerve adherence and nerve separability were significantly lower in treated nerves than in the control group (P < or = 0.05). Low-dose radiotherapy decreased the scores of cellular density and surface measurement of scar tissue (P < or = 0.05). In normal nerves, radiotherapy did not produce any fibrotic effects and the density of fibroblasts/fibrocytes was also very low. CONCLUSION: In the case of surgery or local trauma to peripheral nerve, the use of low-dose radiation therapy may be a safe method of limiting postoperative epineurial scar formation.

Thyroglossal duct cyst in hyoid bone: unusual location.

An atypically sited thyroglossal cyst in a 69-year-old woman is described in this report. The cysts may be located in the intralingual, suprahyoid, thyrohyoid or suprasternal region. The intrahyoid location is rare. The diagnosis was confirmed by computed tomography (CT). Surgical procedure should be indicated in intrahyoid thyroglossal duct cyst cases.

Thallium-201 SPECT in advanced non-small cell lung cancer: in relation with chemotherapeutic response, survival, distant metastasis and p53 status.

PURPOSE: The aim of this study was to evaluate the relationship between 201Tl tumor uptake, chemotherapeutic response, metastasis, p53 status and survival in non-small cell lung cancer (NSCLC). METHODS: A total of 23 patients underwent 201Tl SPECT. In 9 patients, 2nd 201Tl SPECT study were performed 1 week after the 3rd cycle of chemotherapy (ChT), and early (ER) and delayed (DR) tumor/normal lung ratios and retention indices (RI) were obtained. In 15 patients p53 status was assayed with immuno-histochemical staining. The patients were divided into subgroups after the 3rd cycle of ChT; responders [R(+) (n = 10)] and non-responders [R(-) (n = 13)], distant metastasis [(M1) n = 11] and [(M0) n = 12], and mutant p53 status [p(+) n = 7, p53(-) n = 8]. RESULTS: The differences for ER, DR and RI values between all of the subgroups were not statistically significant. ER and DR of responders decreased significantly after ChT; from to 2.46 to 1.36 (p = 0.04) and 2.29 to 1.53 (p = 0.04), respectively. In the non-responder group, both ER and DR slightly increased after ChT (p > 0.05). CONCLUSION: Our results suggest that in NSCLC, there was a weak correlation between higher 201Tl ratios and positive response to chemotherapy, absence of distant metastasis, and p53(-) status. Significant 201Tl uptake decrease after chemotherapy indicates that delayed 201Tl uptake can be used in evaluating the chemotherapeutic response.