Second branchial anomalies in children

AIM: The aim of this study was to evaluate the data of our patients who had been treated for second branchial anomalies in the last 10 years. Here we report our clinical experience in second branchial anomalies with a review of the literature. PATIENTS AND METHODS: We evaluated retrospectively the data of 14 patients, who had been operated on between 1994 and 2004 for second branchial anomalies, in relation to age, sex, complaint at application, diagnostic test, surgical procedures and histopathologic findings. RESULTS: The mean age of the patients (8 female, 6 male) was 5.3 years (range = 1.5-16). The anomalies were usually located on the left side of the neck (n = 6). There were only 3 cases with bilateral anomalies. The majority of the lesions were sinuses (93%). The most frequent clinical feature was the presence of persistent discharge from an external (cutaneous) orifice. All lesions were excised by performing a second step ladder incision. Eight of the lesions were removed under the guidance of 3/0 polypropylene suture. No postoperative complication or recurrence was observed during the follow-up period. CONCLUSIONS: Second branchial arches anomalies are the most common branchial anomalies. Sinuses are more frequently encountered in children. Definitive treatment for these lesions is surgical excision. A polypropylene suture can be inserted into the tract as a guide to prevent incomplete excision.     

Intracranial hemorrhage due to vitamin K deficiency after the newborn period

This study presents clinical and laboratory findings and outcome of infants with intracranial hemorrhage (ICH) due to vitamin K deficiency after the newborn period, and evaluates vitamin K prophylaxis. The hospital records of 19 infants with a diagnosis of ICH due to vitamin K deficiency after the newborn period, seen in our clinic in less than 4 years, were retrospectively evaluated. The mean age at onset of the symptoms was 49 ± 18 days. The most frequent presenting complaints were convulsion (58%), vomiting (47%), and irritability (47%). The most frequent examination findings were coma (74%), fontanel bulging (68%), and absence of pupil reaction (42%). The localizations of the ICHs were as follows: parenchymal (47%), subarachnoid (47%), subdural (42%), and intraventricular (26%). Four patients had used antibiotics and 1 patient had suffered diarrhea before the onset of the symptoms. One patient had a mild hepatic dysfunction that resolved spontaneously in a few weeks and its cause was not found. Mortality was observed in 6 (32%) patients. Ten patients were followed up for a mean period of 26.9 ± 22.6 months. The follow-up findings were developmental delay (40%), microcephaly (30%), epilepsy (30%), blindness (20%), strabismus (20%), spastic tetraparesis (10%), spastic hemiparesis (10%), growth retardation (10%), and hydrocephaly (10%). Three (30%) patients remained neurologically normal. Vitamin K deficiency leads to death and neurological defects. Vitamin K prophylaxis at birth is therefore a priority. In this series, hepatic dysfunction had been detected in only 1 patient. The authors speculate that additional vitamin K to breast-fed infants with liver problem, antibiotic use, diarrhea, etc. should be considered.     

Effects of coasting on the outcome of intracytoplasmic sperm injection-embryo transfer cycles

OBJECTIVES: To determine the effects of 'coasting' on the outcome of controlled ovarian hyperstimulation (COH) and intracytoplasmic sperm injection-embryo transfer (ICSI-ET). DESIGN: Retrospective study. SETTING: IVF Centre, Ozel Ege Tup Bebek Merkezi, Izmir, Turkey. SAMPLE: Twenty-six coasted and 52 non-coasted COH and ICSI-ET patients were enrolled in this retrospective study. METHODS: Coasted patients were enrolled consecutively during the study period, and two non-coasted controls were selected from our database for each coasted patient. Coasting was decided when serum oestradiol level was > or = 4000 pg/mL. Groups were compared using chi2 and Mann-Whitney U-tests for statistical analysis. MAIN OUTCOME MEASURES: Number of oocytes collected, metaphase II (MII) oocytes and cleaving embryos, the fertilisation rate and clinical pregnancy rate were the main outcome measures. RESULTS: Number of oocytes collected, number of MII oocytes, number of cleaving embryos, fertilisation rate and clinical pregnancy rate for the coasted and non-coasted groups were 15.5 +/- 5.2 and 14.0 +/- 7.1, 9.7 +/- 4.8 and 9.3 +/- 3.9, 6.8 +/- 3.9 and 5.8 +/- 3.1, 0.85 +/- 0.18 and 0.78 +/- 0.18, 13/26 and 24/52, respectively; these differences were not statistically significant. None of the patients in the coasted group were hospitalised for signs or findings of severe ovarian hyperstimulation syndrome. CONCLUSIONS: Coasting does not adversely affect the number and the function of mature oocytes and the clinical pregnancy rate.     

The effect of mid-luteal estradiol level on the outcome of ICSI-ET cycles

Methods We investigated if mid-luteal estradiol levels could predict the outcome in intracytoplasmic sperm injection embryo transfer (ICSI-ET) cycles (n=231). Pregnant and non-pregnant women were compared regarding their peak estradiol levels on human chorionic gonadotropin (hCG) injection day, and mid-luteal estradiol levels on the 7th day following oocyte recovery. Pregnancy rates of the groups that were designed according to the "peak/mid-luteal estradiol level" and the mid-luteal estradiol levels were also compared.Results Peak and mid-luteal estradiol levels in pregnant women were higher than in non-pregnant women in all patients, although the difference between peak and mid-luteal estradiol levels were similar in pregnant and non-pregnants. Pregnant women had higher mid-luteal estradiol levels in good responders, but the peak estradiol levels of pregnant and non-pregnant women were similar. In poor responders, pregnant and non-pregnant women were similar with respect to peak and mid-luteal estradiol levels. Both in all patients and good responders, women with mid-luteal estradiol levels <200 pg/ml had lower pregnancy rates than those with >2,000 pg/ml. Peak/mid-luteal estradiol ratios of pregnant and non-pregnant women were not significantly different in all patients, good responders and poor responders; although a tendency for a lower ratio in pregnants was encountered in good responders. Pregnancy rates of the groups according to the "peak/mid-luteal estradiol ratio" were similar; in all patients, good responders and poor responders.Conclusion A relation between the mid-luteal estradiol level and the outcome is encountered only in good responders.     

Hypercoagulability risk factors in children with minimal change disease and the protective role of protein-C activity

It is believed that thrombotic activity in nephrotic syndrome is due to an imbalance between procoagulant/thrombotic and anticoagulant/antithrombotic factors in plasma. The aim of this study was to investigate the hypercoagulability risk in childhood minimal change disease and to find possible protective mechanisms with respect to hemostasis. Twenty-six children with minimal change disease were enrolled in this study. All patients were evaluated during an attack and on remission. The control group consisted of 33 healthy children. During the attack period, prothrombosis parameters, total lipid, cholesterol, fibrinogen levels and platelet count increased significantly compared to levels in the remission period. This denotes that hyperviscosity increases thrombosis tendency. In the attack period, the significant increase of prothrombin fragments 1+2 which shows thrombin formation and thrombin-antithrombin complex which causes prothrombin activation, are an indication of increased thrombosis risk. Five patients with lupus anticoagulant present and 7 patients with activated protein-C resistance ratios carried an increased thrombosis risk. D-dimer level of fibrinolytic factors significantly increased during the attack period. These findings emphasize the existence of thrombotic activity causing the activation of the fibrinolytic system. The significant increase in protein-C activity in these patients represents one of the protective mechanisms against thrombosis. The decrease in tissue plasminogen activator and antiplasmin indicates the protective role of fibrinolytic activity. Consequently, an increase in the protein-C activity is one of the protective mechanisms. The fibrinolytic system also plays an important role in preventing thrombotic activity in these patients.     

A childhood case of intrascrotal neurofibroma with a brief review of the literature

Solitary neurofibroma unassociated with neurofibromatosis type I (NFI) arising within the scrotum is an extremely rare benign tumor. There are 8 cases reported in the literature with only 1 that occurred in childhood. In the current report the authors describe an additional adolescent patient, the second case encountered in childhood and the ninth case reported in the literature, together with a review of the related articles.     

Conjunctival Inverted Follicular Keratosis: A Case Report

Background We report a case of inverted follicular keratosis (IFK) on the conjunctiva. A few cases of IFK have been reported in the literature, but it appears that IFK on the conjunctiva has never been reported.Case A 21-year-old man was referred for evaluation of a conjunctival mass.Observations By slit-lamp biomicroscopy, an 8 × 5 × 3mm mass was observed over the nasal conjunctiva adjacent to the limbus in the left eye. An excisional biopsy of the lesion was carried out. The results of a pathologic examination showed IFK. During the 9-month follow-up period, the patient had no recurrence of the lesion.Conclusion IFK can be considered in the differential diagnosis of conjunctival masses as a rare specific lesion. Jpn J Ophthalmol 2004;48:497–498 © Japanese Ophthalmological Society 2004     

Color Doppler Imaging of Ocular Hemodynamic Changes in BehÇet’s Disease

Purpose A prospective evaluation of the ocular blood flow velocity in patients with BehÇets disease was carried out to determine its changes.Methods Subjects were divided into three groups: those with ocular involvement in BehÇets disease (group I), those without ocular involvement in BehÇets disease (group II), and a control group (group III). Twenty-seven eyes in group I and 28 eyes each in groups II and III were investigated. The blood flow in the central retinal artery (CRA), ophthalmic artery (OA), nasal posterior ciliary artery (NPCA), and the temporal posterior ciliary artery (TPCA) was measured using color Doppler ultrasonography (CDU) to determine the peak systolic velocity (PSV), end diastolic velocity (EDV), and resistivity index (RI). The mean blood flow velocity of the central retinal vein (CRV) was also measured. These results were then compared among the three groups of subjects.Results The PSV values of CRA in groups I and II were found to be significantly lower than those in the control group (P 0.001, P = 0.018, respectively). The PSV values of CRA in group I were found to be significantly lower than those in group II (P 0.001). The EDV values of CRA in groups I and II were found to be significantly lower than those in the control group (P 0.001, P = 0.034, respectively). The EDV values of CRA in group I were significantly lower than those in group II (P = 0.041). The PSV values of OA in group I were significantly lower than those in the control group (P = 0.002). The EDV values of OA in group I were significantly lower than those values in group II or the control group (P = 0.001 and P = 0.037, respectively). The PSV values of NPCA in group I were significantly lower than those in the control group (P = 0.007). The PSV values of TPCA in groups I and II were significantly lower than those in the control group (P 0.001, P 0.001, respectively). The EDV values of TPCA in group I were significantly lower than those values in group II or the control group (P = 0.014 and P = 0.003, respectively). There were no significant differences in the mean blood flow values of the CRV among all three groups (P 0.05).Conclusions There are significant reductions in the blood flow values of the orbital arteries in patients with BehÇets disease, and they are more evident in those with ocular involvement. This might be the result of occlusive vasculitis, which is frequently seen in the retinal vessels of patients with BehÇets disease. Jpn J Ophthalmol 2004;48:101–105 © Japanese Ophthalmological Society 2004     

Effect of ovarian stimulation with recombinant follicle-stimulating hormone,gonadotropin releasing hormone antagonists,and human chorionic gonadotropin on endometrial maturation on the day of oocyte pick-up

OBJECTIVE: To assess the effect of ovarian stimulation with recombinant FSH, GnRH antagonists, and hCG on endometrial maturation on the day of oocyte pick-up. DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENT(S): Fifty-five women undergoing controlled ovarian hyperstimulation for IVF/intracytoplasmic sperm injection (ICSI). INTERVENTION(S): [1] Ovarian stimulation with recombinant FSH, starting on day 2 of the cycle and GnRH antagonist, starting after a median of 6 days of recombinant FSH stimulation (range, 5-12 days); [2] hCG administration for ovulation induction; and [3] aspirational biopsy of endometrium at oocyte pick-up. MAIN OUTCOME MEASURE(S): Endometrial histology at oocyte pick-up by Noyes criteria. RESULT(S): Advancement of endometrial maturation (2.5 +/- 0.1 days) as compared to the expected chronological date was observed in all antagonist cycles at oocyte retrieval. Endometrial advancement at oocyte pick-up increased in line with values of LH at initiation of stimulation and the duration of recombinant FSH treatment before the antagonist was started. CONCLUSION(S): The higher the values of LH at initiation of stimulation and the longer the duration of recombinant FSH treatment before the antagonist is started, the more advanced the endometrial maturation at oocyte pick-up.     

Endocrine complications in patients with beta-thalassemia major

Thirty-seven patients with thalassemina major (TM) were studied to determine the extent and rate of endocrine complications. Mean haemoglobin and ferritin concentrations were 8.8 +/- 0.6 and 3,597 +/- 1,931, respectively. Provocation tests for growth hormone secretion were applied in patients with standing heights below the third centile and/or growth velocities below the 10th centile. Sexual maturation was assessed by using the criteria of Tanner. Glucose metabolism was assessed by fasting plasma glucose and glucose tolerance test. Basal thyroid function was measured and thyrotropin-releasing hormone tolerance test was carried out. Growth retardation was found in 40 per cent of patients and growth hormone deficiency was a prominent cause of growth retardation. Gonadal dysfunction was detected in 47 per cent of patients. Hypothyroidism was observed in 16 per cent and impaired glucose metabolism in 10.8 per cent patients. The high rate of endocrine disturbances indicates the importance of regular follow-up of thalassemia major patients with regard to endocrine complications of the disease.