Cerebral thrombosis associated with heterozygous factor V Leiden mutation and high lipoprotein(a) level in a girl with factor XIII deficiency.

A 5-year-old girl in whom the diagnosis of inherited factor XIII deficiency was established at the age of 1 day presented with cryptic tonsillitis along with drowsiness and an abrupt occurrence of getting left interior cross eyed. While an intracranial hemorrhage was expected, cerebral imaging studies surprisingly revealed multiple sino venous thrombosis. In prothrombotic screening studies she and her father were both found to be heterozygous for factor V Leiden mutation along with having elevated levels of lipoprotein(a). Low-molecular-weight heparin was started. Ventriculoperitoneal shunt was applied because of persistence of increased intracranial pressure. Thrombosis disappeared and blood flow was normalized by the end of 2 months and the patient was discharged on coumadin therapy as being well. We would like to report this unusual case and to discuss the possible effects of two major genetic prothrombotic risk factors on inherited bleeding tendency or vice versa.     

Comparison of Der p1-specific antibody levels in children with allergic airway disease and healthy controls

Although children, with allergic airway disease, who are sensitized to house-dust mite (HDM) are known to have increased levels of allergen-specific IgE and IgG, the association between the quantity of those immunoglobulins and the clinical features of disease is not yet well established. The purpose of this study was (i) to evaluate Der p1-specific IgA, IgG1, IgG4, and IgE levels of children with HDM-allergic asthma and allergic rhinitis and to compare it with that of healthy controls (ii) to assess the association with disease duration. A total of 73 patients were included. Of those, 58 had asthma (M/F: 27/31, mean age 7.9 +/- 2.7 yr) and 15 were diagnosed as allergic rhinitis (M/F: 8/7, mean age 10.1 +/- 4.0 yr) without asthma. Twenty-five (M/F: 13/12, mean age 9.5 +/- 4.2 yr) non-allergic children were included as healthy controls. Data on age at onset and duration of disease were recorded. Then, Der p1-specific IgA, IgG1, IgG4, IgE levels were measured in all of the 98 subjects by ELISA. Comparison of Der p1-specific antibody levels of patients and controls revealed that Der p1-specific IgG1, IgG4 and IgE levels of patients with asthma (p = 0.012, p = 0.021, p = 0.004, respectively) were significantly higher than healthy controls. Also, the ratio of Der p1-specific IgA/IgE was significantly lower in asthmatic children when compared with children with allergic rhinitis and controls (p = 0.029, p < 0.001, respectively). Der p1-specific IgG1, IgG4, IgE and IgA levels of asthmatic children with duration of disease of >or=4 yr were significantly higher than those with disease duration of <4 yr. IgA/IgE ratio was not significantly different in those two groups of asthmatics. We concluded that although all of the specific antibody levels increased with longer duration of asthma, IgA/IgE ratio remains to be low in asthmatic children allergic to HDM.     

EFFECT OF SHORT-COURSE, HIGH-DOSE STEROID THERAPY IN A CHILD WITH MYELODYSPLASTIC SYNDROME

High-dose methylprednisolone (HDMP) has been shown to induce differentiation of myeloid leukemic cells with a remarkable antileukemic effect in children with various subtypes of acute myeloblastic leukemia (AML). Here the beneficial effect of short-course HDMP therapy in a child with myelodysplastic syndrome (MDS) is reported. Oral methylprednisolone sodium succinate (Prednol-L) was administered at a single daily dose of 30 mg/kg for 5 days to a 4-year-old girl with refractory anemia with excess of blasts and hypocellular bone marrow before the initiation of chemotherapy. In addition to dramatic clinical improvement, the patient's white blood cell count increased from 2.3 ×10 9 /L to 5.0 ×10 9 /L, and peripheral blood blast cells disappeared 4 days after HDMP treatment. Repeated bone marrow aspirate 1 week after the initiation of HDMP disclosed increased cellularity with no blasts. Furthermore, short-course HDMP treatment stimulated the increase in the number of peripheral blood lymphocytes and CD3 + , CD4 + , CD8 + , CD19 + , CD34 + , and NK cells. Results obtained with HDMP from the previous studies and the present case suggest that high-dose methylprednisoloneis a promising agent in the treatment of MDS and it is recommended as an initial treatment especially for MDS children with hypocellular bone marrow at presentation.     

Transfer of T cells from intranasal ovalbumin-immunized mice ameliorates allergic response in ova-sensitized recipient mice.

Mucosal immunotherapy is suggested as a treatment strategy for tolerance induction in allergic diseases. The purpose of this study was to determine the effect of transferred splenic T cells from intranasal ovalbumin (OVA)-immunized mice to naive mice before sensitization on its impact of cytokine production and airway histopathology. BALB/c mice in group I received intranasal immunotherapy (days1-6), carboxylfluorescein succinyl ester (CFSE)-labeled splenocytes or splenic T cells were i.v. transferred to naive recipients (group II) before OVA sensitization. Acute murine asthma model was established by two i.p. OVA injections (days 21 and 28) and seven OVA nebulizations (days 42-48) in groups I, II and III. Groups III and IV served as asthma model and control, respectively. CFSE-labeled cells in splenocytes and lymph node lymphocytes, lung histopathology, IL-4, IL-10, and interferon (IFN) gamma cytokines of recipients were analyzed 24 hours after OVA nebulization challenge. CFSE-labeled T cells from group I were detected in spleen and regional lymph nodes of the OVA-sensitized recipients (group II). Smooth muscle and thickness of airways were less in intranasal OVA immunotherapy and OVA-sensitized recipients when compared with the asthma model (p < 0.05). Area of inflammation was significantly suppressed in OVA-sensitized recipients compared with the asthma model (p < 0.01). IL-10 and IFN-gamma levels in splenocyte supernatants were significantly increased in intranasal immunotherapy and OVA-sensitized recipients compared with asthma model and controls (p < 0.01). IL-4 levels were significantly less in intranasal immunotherapy group and the OVA-sensitized recipient group when compared with asthma the model group (p < 0.05). This study suggests that intranasal immunotherapy with allergens regulates T-cell responses and ameliorates airway histopathology in sensitized mice, hence, encouraging mucosal tolerance induction as a suitable treatment of allergic diseases.     

Effort-related compression of median and ulnar nerves as a result of reversed three-headed and hypertrophied palmaris longus muscle with extension of Guyon's canal.

We explored a swelling on the anterior surface of the right distal forearm of a 21-year-old right-handed male soldier and effort-induced symptoms of median and ulnar nerve compression that showed a reversed, three-headed and hypertrophied palmaris longus muscle with extension of Guyon's canal.     

Excision of an asymptomatic cervical intradural neurenteric cyst through the anterior approach: a study of two cases and a review of the literature

BACKGROUND: Spinal neurenteric cysts are very rare lesions, especially after the second decade of life. They account for 0.3% to 0.5% of all spinal tumors and occur most commonly in ventral locations. The cysts are usually removed via a posterior approach. PURPOSE: To present the clinical and radiologic results of patients with spinal neurenteric cysts who were treated via anterior approach procedures. STUDY DESIGN: This report is composed of two cervical neurenteric cyst cases that are compared with published studies. METHODS: We present two patients, 41- and 39-year-old women, each with a cervical intradural neurenteric cyst. Both of these patients had apparent neck pain without neurological deficit. MRI revealed neurenteric cysts located at C7 and C7-T1 levels. After anterior corpectomy, the intradural cysts were removed, and then fusion was performed. RESULTS: The postoperative period went well. The follow-up cervical MRI studies were performed at 3, 6, and 18 months postoperatively, and there were no abnormalities found. CONCLUSIONS: This study has led to the conclusion that although neurenteric cysts are rare in adults, they can still be present with only persistent neck pain and without neurological deficits. This may lead to misdiagnosis. The importance of MRI is not controversial in the early diagnosis. An anterior approach may be considered the first preference for surgical technique in patients with ventrally located neurenteric cysts.     

Relationship Between the Levels of Holotranscobalamin and Vitamin B<Subscript>12</Subscript> in Children

The purpose of this study was to evaluate the relationship between the plasma holoTC and serum vitamin B₁₂ in children and to identify a cutoff cobalamin values according to holoTC. One hundred and fifty-five children were enrolled into the study. All children were evaluated for hemoglobin, vitamin B₁₂, folate, ferritin and holoTC levels. Children were grouped as with low vitamin B₁₂ level (≤200 pg/mL, group I) and normal vitamin B₁₂ (>200 pg/mL, group II). Serum vitamin B₁₂, and holoTC levels were performed in each patient in the study. In 101 patients with low vitamin B₁₂ (group I) the mean holoTC was 21.74 ± 1.14 pmol/L. In 54 children with normal vitamin B₁₂ (group II) mean holoTC was 44.0 ± 2.7 pmol/L (p < 0.01). A ROC curve analysis was performed to delineate the optimum cut-off point for vitamin B₁₂ level and it was found to be 165 pg/mL with a sensitivity of 70% and specificity of 74%; the area under curve was 0.783 (p < 0.01). Our study displayed a positive correlation between vitamin B₁₂ and holoTC, and defined an optimum cutoff value for vitamin B₁₂ as 165 pg/mL. Further studies using the markers both MMA, tHcy and holoTC to confirm the findings are needed.     

Vertebral artery hypoplasia as an independent risk factor of posterior circulation atherosclerosis and ischemic stroke

Vertebral artery hypoplasia (VAH) is a frequent anatomical variation of vertebral arteries, with emerging evidence suggesting that it contributes to posterior circulation ischemia. However, the relationship between VAH and ischemic stroke remains unknown. Hence, this study aimed to determine the prevalence of VAH in patients diagnosed with acute ischemic stroke who were followed up in a neurology clinic and to determine if it can potentially be a risk factor for atherosclerotic stenosis in vertebrobasilar circulation.This retrospective study included 609 patients diagnosed with acute ischemic stroke between January 1, 2019 and January 1, 2020. Demographic of patients, risk factors, radiological and clinical characteristics were evaluated.Posterior circulation was very common in patients with VAH, and the most common locations of atherosclerotic stenosis were V1 and V4 segments of the vertebral artery and the middle segment of basilar artery. Analysis of the risk factors for atherosclerotic stenosis in patients with posterior circulation acute ischemic stroke suggested that VAH was an independent risk factor.Findings of the study suggest that VAH pre-disposes atherosclerotic stenosis in vertebrobasilar circulation, although its mechanism remains unknown. Hemodynamic parameters associated with atherosclerosis could not be measured in vivo. Thus, to better understand the underlying mechanism, conducting studies that examine blood flow parameters with high-resolution magnetic resonance angiography in patients diagnosed with acute cerebral ischemia patients with VAH is warranted.