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Nuclear protein in testis (NUT) carcinoma is a rare neoplasm arising mainly from midline structures. It is an aggressive type of carcinoma associated with poor survival despite the use of multiple treatment modalities. Here, we present a case of a 17-year-old paediatric patient with NUT carcinoma of larynx, which is even rarer among all reported cases. The patient underwent surgery followed by radiotherapy and systemic treatment and he died 15 months after the diagnosis. The management of this rare disease requires further investigation.

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OBJECTIVES: We aimed to determine whether the presence of Hürthle cells altered the distribution of categories in the Bethesda system for reporting thyroid cytopathology, or the expected neoplastic and malignant outcome. METHODS: Fine needle aspiration (FNA) cytology reports of Hürthle cells in a 2-year period were evaluated. The distribution of Bethesda system categories and the outcome at partial or complete thyroidectomy were compared for FNAs with and without Hürthle cells. RESULTS: Of 895 adequate FNAs with Hürthle cells, 764 (85.4%) were classified as benign, 86 (9.6%) as atypia of undetermined significance/follicular lesion of undetermined significance (AUS/FLUS), 32 (3.6%) as follicular neoplasm/suspicious for follicular neoplasm (FN/SFN), 12 (1.3%) as suspicious for malignancy (SFM) and one (0.1%) as malignant. Of 10 359 adequate FNAs without Hürthle cells, 9707 (93.7%) were classified as benign, 412 (4.0%) as AUS/FLUS, 77 (0.7%) as FN/SFN, 93 (0.9%) as SFM and 70 (0.7%) as malignant. The distribution of categories in FNAs with and without Hürthle cells was significantly different (P < 0.001) as a result of a decrease in benign and an increase in AUS/FLUS and FN/SFN categories. Among 128 patients with and 582 without Hürthle cells undergoing surgery, the overall neoplastic and malignancy rates were higher in the former than in the latter group (27.3% versus 14.9%, P < 0.001; 21.1% versus 11.7%, P = 0.003; respectively). Although neoplastic and malignant rates were higher in the group with than without Hürthle cells in all categories, the differences were only significant for a neoplastic outcome of benign cytology (15.1% versus 6.0%, P = 0.0013) and a malignant outcome of FN/SFN cytology (63.6% versus 21.9%, P = 0.0108). CONCLUSIONS: We found that the rates of AUS/FLUS and FN/SFN categories in the Bethesda system were higher when Hürthle cells were present. After surgery, neoplastic and malignant outcomes were significantly higher in the Hürthle cell group.

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PURPOSE: The purpose of this study is to evaluate the role of apoptosis in the pathogenesis of blepharoptosis. PATIENTS AND METHODS: Forty-five eyelids of 43 consecutive patients (16 female, 27 males) that underwent levator resection surgery for ptosis correction were included in the study. Twenty-six of the eyelids had congenital myogenic ptosis and 19 had aponeurotic ptosis. Levator palpebrae superioris function and height of the vertical palpebral fissure were measured in all patients. After levator resection surgery, the distal part of the levator aponeurosis was fixed and sent for evaluation. Apoptotic cells were detected using Apop Tag Plus Peroxidase In Situ Apoptosis Detection Kit. RESULTS: The mean levator palpebrae superioris function was 8.4 mm (range 5-10 mm) in congenital ptosis group and 12.1 mm (range 10-17 mm) in the aponeurotic ptosis group. The mean height of the vertical palpebral fissure in patients with congenital ptosis and aponeurotic ptosis were 6.5 mm (range 5-9 mm) and 6.1 mm (3-9 mm), respectively. The mean apoptotic index of congenital ptosis and aponeurotic ptosis were 27.3 (16-39) and 29.8 (18-41), respectively. There was no statistically significant difference between congenital and aponeurotic ptosis groups in a mean apoptotic index (P<0.05). Apoptotic index was not correlated with age, levator palpebrae superioris function, palpebral fissure height, and lid crease height in two groups. CONCLUSION: We found no statistically significant difference between two subtypes of blepharoptosis regarding apoptosis. According to this study, apoptosis seems to have no significant role in the development of aponeurotic blepharoptosis.

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OBJECTIVE: To report our experience of atypia of undetermined significance (AUS)/follicular lesion of undetermined significance (FLUS) rate and outcome. METHODS: Among 7658 patients with 19 569 nodules, 524 (2.7%) nodules were diagnosed as AUS/FLUS on fine needle aspiration (FNA). After exclusion of patients with simultaneous nodules that were suspicious for follicular neoplasm or malignancy or that were malignant, 368 (4.8%) patients were diagnosed as AUS/FLUS. The outcome of 146 patients who had undergone surgery or repeated fine needle aspirate at the time of preparation of this study was evaluated. The original FNAs were matched to repeated FNAs and thyroidectomy or diagnostic lobectomy specimens. RESULTS: Seventy-two (19.6%) of the 368 patients had directly undergone surgery, either a lobectomy or a thyroidectomy: of these, 27 (37.5%) had neoplastic nodules (21 were malignant). Seventy-four (20.1%) of the 368 patients had repeat FNA. On second FNA, 47 of 74 (63.5%) were benign, three were suspicious for follicular neoplasm, one was malignant and 23 (31.1%) were non-diagnostic. Four patients had a third FNA: two were AUS/FLUS, one was malignant and one non-diagnostic. One patient had a fourth FNA, which was diagnosed as AUS/FLUS. Sixteen (21.6%) of 74 patients with repeat FNA had surgery: three of these had neoplastic nodules (two were malignant). Overall, 88 of the 368 (23.9%) patients had a thyroidectomy of which 30 (34.1%) were neoplastic and 23 (26.1%) malignant. The neoplastic rate for patients who were once diagnosed with AUS/FLUS was 8.2% and the malignancy rate 6.3%. The malignancy rate for patients on follow-up at the time we prepared the study was 15.7% (23/146); 222 remained on follow-up without surgery or repeat FNA or were managed elsewhere. CONCLUSIONS: Although in this category repeat FNA is expected rather than excision, we suggest evaluation of all AUS/FLUS patients in multidisciplinary meetings to decide management and recommend follow-up of all patients with this diagnosis.

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Zinc (Zn) plays an important role in the maintenance of immune functions, including cellular/humoral immunity, and in the prevention of oxidative injury. Therefore, the maintenance of a normal Zn status may be important in bone marrow transplantation (BMT) patients. Serum Zn levels were determined in 35 children during the BMT period. In addition, as Zn-related factors, serum Cu levels and alkaline phosphatase (AP) activity were also measured. There was a significant decrease in Zn and AP values during the immediate post-transplant period (lowest at day +7) when compared to pre-BMT levels (p <.01). The patients who developed hypozincemia were more likely to be transplanted for a diagnosis of malignant disorder and were younger, and adverse events appeared to occur more frequently. This preliminary study suggests that maintaining a normal Zn status may be important in BMT patients and that Zn deficiency may be a risk factor for adverse events.

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Chelation therapy with desferrioxamine (DFO) can be an important problem for patients with thalassaemia major (TM). In an effort to find a solution to this problem, a new delivery system based on a disposable and lightweight device was developed by Baxter. The aim of study was to investigate the compliance of patients and the effects and side effects of two different infusors. The 26 patients using infusors were divided into 2 groups, who were infused with DFO for 48 h (group A) and for 120 h (group B). The 24-h mean urinary iron and zinc excretion in groups A and B were statistically significant at the beginning and end of therapy (p < 0.001). Serum ferritin levels decreased in both groups compared with control (p < 0.001). No systemic reactions were observed either during or at the end of treatment. The compliance and use of infusors were 97% in group A and 72% in group B. In conclusion, better compliance was achieved and there were fewer local complications in group A than in group B. Subcutaneous infusion of DFO with both devices may be ideal for patients who have compliance problems and cannot be treated using portable pumps. The device is new and expensive, but excellent and effective for long-term DFO infusion.

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Reports of complications associated with local anesthesia in ophthalmic surgery have increased conspicuously in recent years. Sub-Tenon's capsule anesthesia for anterior segment surgery avoids the risks of retrobulbar and peribulbar injections. This study compared sub-Tenon's and retrobulbar anesthesia. Patients undergoing various anterior segment surgery were randomly assigned to sub-Tenon's or retrobulbar anesthesia; 300 were operated with 1-quadrant sub-Tenon's anesthesia (1-QST) and the results were compared with 270 patients operated with retrobulbar anesthesia. Retrobulbar anesthesia consisted of a 2.5 ml injection of a 1:1 mixture of 2% lidocaine without epinephrine and 0.5% bupivacaine. Sub-Tenon's anesthesia involved direct transconjunctival infiltration of the same local anesthetic directly into the sub-Tenon's space, in the inferior-nasal quadrant, using a blunt 23-gauge cannula. Patients undergoing various anterior segment surgery procedures were randomly assigned to 1-QST or retrobulbar anesthesia; 300 patients were operated with 1-QST and the results were compared with 270 patients operated with retrobulbar anesthesia. Preinjection mean (+/- SD) IOP wer 12.9 +/- 3.7 mmHg in the retrobulbar and 13.4 +/- 3.2 mmHg in the 1-QST patients. Preoperative intraocular pressures were 8.7 +/- 3.0 mmHg in the retrobulbar and 9.2 +/- 3.2 mmHg in 1-QST patients. Pre- and postinjection IOP for retrobulbar and 1-QST patients were similar. Pain scores for delivery of the anesthetic, using a numerical rating scale, produced a median score of 1 for 1-QST and 2 for the retrobulbar technique. For the subsequent operative procedure, the median score was 1 for 1-QST and 2 for the retrobulbar patients. Complete akinesia was achieved in 41% with 1-QST and in 69% of retrobulbar patients. 1-QST patients with incomplete akinesia most often had lateral muscle function which did not interfere with the operation. We found the use of a blunt cannula to deliver anesthetic into the sub-Tenon's space as a simple, safe and effective alternative approach to traditional retrobulbar anesthesia in anterior segment surgery.

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This study has been undertaken to find out whether urinary zinc excretion, which is already increased in patients with thalassemia, is further increased by usual and high doses of desferrioxamine (DF). A total of 11 beta-thalassemia major patients were included. DF infusions have been performed with doses, either 50 mg/kg or 150 mg/kg. Nine age and sex matched normal children were taken as the control group. The mean basal-Zn excretion of the patients was significantly higher than the mean Zn excretion observed in controls. No significant difference is observed between the mean Zn excretion obtained on different doses of DF. However, they are both significantly higher than the mean basal-Zn levels of the controls.

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