Sirolimus treatment for neonate with blue rubber bleb nevus syndrome: A case report

BACKGROUNDBlue rubber bleb naevus syndrome (BRBNS) is a rare disease that usually presents with multiple venous malformations in the skin and gastrointestinal tract. Lesions located in the gastrointestinal tract always result in chronic gastrointestinal bleeding and severe anemia. The successful management of BRBNS with sirolimus had been reported in many institutions, due to its impact on signaling pathways of angiogenesis. However, the experience in treatment of neonates with BRBNS was limited.CASE SUMMARYA 38-day-old premature female infant born with multiple skin lesions, presented to our center complaining of severe anemia and hematochezia. Laboratory examination demonstrated that hemoglobin was 5.3 g/dL and contrast-enhanced abdominal computed tomography showed multiple low-density space-occupying lesions in the right lobe of the liver. She was diagnosed as having BRBNS based on typical clinical and examination findings. The patient was treated by transfusions twice and hemostatic drugs but symptoms of anemia were difficult to alleviate. A review of BRBNS case reports found that patients had been successfully treated with sirolimus. Then the patient was treated with sirolimus at an average dose of 0.95 mg/m²/d with a target drug level of 10-15 ng/mL. During 28 mo of treatment, the lesion was reduced, hemoglobin returned to normal, and there were no adverse drug reactions.CONCLUSIONThis case highlights the dosing regimen and plasma concentration in neonates, for the current common empiric dose is high.     

蓝色橡皮-大疱性痣综合征的围手术期护理

总结了1例蓝色橡皮-大疱性痣综合征围手术期的护理。术前给予充分的肠道准备和皮肤准备,术后加强管路的护理、造口护理,密切观察病情,加强并发症的观察和护理,采取早期完全胃肠外营养支持逐步过渡到肠内营养,加强健康教育,患者康复出院。     

Cluster headache with Brugada electrocardiogram pattern: a case report

Agents including the effect on sodium channel are restricted in patients with Brugada electrocardiogram (ECG) pattern or Brugada syndrome. On the other hand, many therapeutic agents for cluster headache (CH) block sodium channel. After recommended therapies without sodium channel blocking effect were failed in a 40-year-old male with CH whose ECG shows Brugada ECG pattern, clonazepam and codein phosphate, which are exceptional treatments for CH, were effective for severe unilateral orbital pain.     

血管内皮生长因子和碱性成纤维细胞生长因子缓释微球联合高压氧对超长随意皮瓣成活的影响

目的 探讨血管内皮生长因子（VEGF）和碱性成纤维细胞生长因子（bFGF）缓释微球联合高压氧对大鼠超长随意皮瓣成活率的影响.方法 采用改良的McFarlane皮瓣制作方法建立实验模型.模型制备后将SD大鼠40只随机分为4组（空白对照组、高压氧组、VEGF/bFGF微球组、高压氧与微球联合组）,每组各10只,分别给予不同的干预处理.术后7d计算各组皮瓣的存活面积比,取皮瓣中段组织计算新生微血管数量,免疫组化法检测VEGF表达的差异.结果 （1）存活面积百分比：术后7d,高压氧与微球联合组皮瓣的存活面积百分比为（89.54±3.23）％,VEGF/bFGF微球组为（73.54±4.57）％,高压氧组为（71.89±2.26）％,空白对照组为（50.36±2.37）％,4组经方差分析,差异有统计学意义（F=390.328,P＜0.05）,组间两两比较,高压氧与微球联合组显著高于其他实验组,差异有统计学意义（P均＜0.05）;而VEGF/bFGF微球组和高压氧组间则无统计意义（P＞0.05）.（2）新生微血管数目：高压氧与微球联合组、VEGF/bFGF微球组、高压氧组和空白对照组皮瓣Ⅱ区的新生血管计数分别为（35.14±4.21）、（23.34 ±2.53）、（25.22 ±2.73）、（17.37±5.73）个/mm2,4组经方差分析,差异有统计学意义（F=51.736,P＜0.05）,组间两两比较,高压氧与微球联合组显著高于其他实验组,差异有统计学意义（P均＜0.05）;VEGF/bFGF微球组与高压氧组间则无统计意义（P＞0.05）.（3）免疫组化检测：各组VEGF阳性量累积吸光度（A）值分别为78.39±19.12、52.42±13.59、49.84±12.93、29.24±10.35,4组差异有统计学意义（F=189.956,P＜0.05）,组间两两比较,高压氧与微球联合组显著高于其他实验组,差异有统计学意义（P均＜0.05）;而VEGF/bFGF微球组与高压氧组间差异则无统计意义（P＞0.05）.结论 VEGF和bFGF缓释微球联合高压氧可以促进皮瓣新生血管的增生,改善皮瓣血供,进而提     

Intraoral somatosensory abnormalities in patients with atypical odontalgia—a controlled multicenter quantitative sensory testing study

Intraoral somatosensory sensitivity in patients with atypical odontalgia (AO) has not been investigated systematically according to the most recent guidelines. The aims of this study were to examine intraoral somatosensory disturbances in AO patients using healthy subjects as reference, and to evaluate the percent agreement between intraoral quantitative sensory testing (QST) and qualitative sensory testing (QualST). Forty-seven AO patients and 69 healthy control subjects were included at Universities of Washington, Malmö, and Aarhus. In AO patients, intraoral somatosensory testing was performed on the painful site, the corresponding contralateral site, and at thenar. In healthy subjects, intraoral somatosensory testing was performed bilaterally on the upper premolar gingiva and at thenar. Thirteen QST and 3 QualST parameters were evaluated at each site, z-scores were computed for AO patients based on the healthy reference material, and LossGain scores were created. Compared with control subjects, 87.3% of AO patients had QST abnormalities. The most frequent somatosensory abnormalities in AO patients were somatosensory gain with regard to painful mechanical and cold stimuli and somatosensory loss with regard to cold detection and mechanical detection. The most frequent LossGain code was L0G2 (no somatosensory loss with gain of mechanical somatosensory function) (31.9% of AO patients). Percent agreement between corresponding QST and QualST measures of thermal and mechanical sensitivity ranged between 55.6% and 70.4% in AO patients and between 71.1% and 92.1% in control subjects. In conclusion, intraoral somatosensory abnormalities were commonly detected in AO patients, and agreement between quantitative and qualitative sensory testing was good to excellent.     

Body size dissatisfaction associated with dietary pattern,overweight, and physical activity in adolescents: A cross‐sectional study

This study analyzed the association of body size dissatisfaction with body mass index, dietary pattern, and physical activity in adolescents. A sample of 1074 participants between 10 and 17 years of age was recruited. Body size dissatisfaction was assessed with the silhouettes' scale. Dietary pattern, physical activity, and socioeconomic status were assessed with questionnaires. Body mass index was measured objectively. The prevalence of body size dissatisfaction was 77.0%, where 29.8% desired to increase body size and 47.2% desired to decrease body size. Adolescents who desired to increase body size were more likely to consume vegetables and sweets and less likely to be in the highest quartile of physical activity than satisfied adolescents. Adolescents who desired to reduce body size were more likely to report lower fruit and vegetable consumption than satisfied adolescents. Health actions aiming to reduce body dissatisfaction in adolescents should address improvement in dietary pattern and physical activity engagement, considering that desires to increase or decrease body size were differently associated in this study.     

Ovarian thecoma presenting with acute ovarian torsion in pregnancy; report of a rare case

Thecoma is a commonly benign ovarian tumor of the group “Sex cord‐stromal neoplasms.” This group represents <5 percent of ovarian tumors. Thecoma is extremely rare in pregnancy. Here, we describe a 17‐week pregnant woman presenting with acute ovarian pedicle torsion as a result of an ovarian thecoma.     

Single‐incision cholecystectomy in a patient with situs inversus totalis presenting with cholelithiasis: A case report

Laparoscopic cholecystectomy has become the gold standard for the treatment of cholelithiasis, and many reports of single‐incision laparoscopic cholecystectomy have been published in the past few years. Situs inversus totalis is a very rare condition, but the variant anatomy should not preclude a minimally invasive approach to surgery. We report a case of successful single‐port laparoscopic cholecystectomy in a patient with situs inversus totalis, describe the technical advantages, and review the literature.     

γδ TCR T lymphoblastic leukemia in a child presenting with marked hyperleukocytosis

γδ TCR T lymphoblastic leukemia is rare in children and should be differentiated mainly from hepatosplenic T‐cell lymphoma in this age group.     

Subcutaneous panniculitis‐like T‐cell lymphoma in a young girl presenting with periorbital edema and fever: A case report

Subcutaneous panniculitis‐like T‐cell lymphoma is a rare and highly malignant extra‐nodal lymphoma. It has a wide range of clinical presentations (such as periorbital swelling as in our case) and should be considered in the differential diagnosis of systemic lupus erythematosus, especially in children.     

Increased risk of all-cause,Alzheimer’s,and vascular dementia in adults with migraine in Korea: a population-based cohort study

BackgroundStudies investigating the association between migraine and dementia have reported inconsistent findings. This study aimed to evaluate whether patients with migraine have an increased risk of dementia compared to individuals without migraine.MethodsWe obtained data from the 2002–2019 Korean National Health Insurance Health Screening Cohort. Non-migraine controls were selected using a 1:1 risk-set matching with a time-dependent propensity score. The main outcome was the development of all-cause dementia, and the secondary outcome was the development of each cause of dementia (Alzheimer’s, vascular, mixed or other specified, and unspecified dementia). The incidence rate of dementia was calculated using Poisson regression, and the association between migraine and dementia was evaluated using Cox proportional hazards regression.ResultsAmong 88,390 participants, 66.1% were female, and the mean baseline age was 55.3 ± 9.4 years. During the study period, dementia cases were identified in 4,800 of the 44,195 patients with migraine and 3,757 of the 44,915 matched controls. The incidence rate of dementia was 139.6 (95% confidence interval [CI], 135.7–143.5) and 107.7 (95% CI, 104.3–111.1) cases per 10,000 person-years in patients with migraine and matched controls, respectively. Patients with migraine had a 1.30 (hazard ratio [HR], 1.30; 95% CI, 1.25–1.35), 1.29 (HR, 1.29; 95% CI, 1.23–1.35), 1.35 (HR, 1.35; 95% CI, 1.19–1.54), 1.36 (HR, 1.36; 95% CI, 1.00–1.83), and 1.30 (HR, 1.30; 95% CI, 1.17–1.45) times higher risk of developing all-cause dementia, Alzheimer’s dementia, vascular dementia, mixed or other specified dementias, and unspecified dementia than their matched controls, respectively.ConclusionOur results suggest that migraine is associated with an increased risk of subsequent dementia. Further research is warranted to confirm these findings and to reveal the underlying mechanisms.Supplementary InformationThe online version contains supplementary material available at 10.1186/s10194-022-01484-y.     

Endovascular treatment of an acute left middle cerebral artery >6 h post stroke in a patient presenting with dysphasia and dense right hemiplegia

This paper describes the case of a 32‐year‐old man presenting with dense right hemiplegia and global aphasia caused by an acute left middle cerebral artery infarct that underwent successful endovascular therapy after being determined ineligible for intravenous tissue plasminogen activator. Clot traversion and balloon disruption followed by intra‐arterial Alteplase resulted in successful re‐canalization of his middle cerebral artery at 7 h 30 min. At 3 months post stroke, the patient had moderately severe expressive dysphasia but was mobilizing independently with normal right upper and lower limb strength. In conclusion, the 3 month outcome suggests that the therapeutic time window for endovascular therapy might exceed 6 h post stroke.     

A C‐type lectin with a single carbohydrate‐recognition domain involved in the innate immune response of Tribolium castaneum

C‐type lectins are one of the pattern‐recognition proteins involved in innate immunity in invertebrates. Although there are 16 C‐type lectin genes that have been identified in the genome of Tribolium castaneum, their functions and mechanisms in innate immunity remain unknown. Here, we identified one C‐type lectin orthologue, TcCTL6 (TC003708), by sequencing random clones from the cDNA library of the coleopteran beetle, T. castaneum. TcCTL6 contains a 654 bp open reading frame encoding a protein of 217 amino acids that includes a single carbohydrate‐recognition domain. The expression of TcCTL6 was significantly induced by Escherichia coli, Staphylococcus aureus and stimulation with carbohydrates, including lipopolysaccharide and peptidoglycan. A binding assay suggested that the recombinant TcCTL6 not only bound to lipopolysaccharide and peptidoglycan but also bound to Gram‐positive (S. aureus, Bacillus subtilis and Bacillus thuringiensis) and Gram‐negative bacteria (E. coli and Pseudomonas aeruginosa) in the presence of calcium ions. Furthermore, when TcCTL6 was knocked down by RNA interference, four antimicrobial peptides (attacin1, attacin2, coleoptericin1 and coleoptericin2) were significantly decreased. These results demonstrate that TcCTL6 plays a vital role in the immune response towards pathogen infection by influencing the expression of antimicrobial peptides and the agglutination of bacteria in the presence of calcium ions in T. castaneum.     

Group C beta hemolytic Streptococci as a potential pathogen in patients presenting with an uncomplicated acute sore throat – a systematic literature review and meta-analysis

Objective: The pathogenicity of beta-hemolytic Streptococcus group C (GCS) in patients attending for an uncomplicated acute sore throat is unknown and it was the objective to clarify this.Design: Systematic literature review with meta-analysis. Setting Medline and Scopus were searched from inception to February 2019, with searches of reference lists, Subjects case-control studies stating prevalence of GCS in patients as well as healthy controls presented for children and adults separately. Studies including patients already treated with antibiotics and studies focused on patients with HIV, malignancy or immunosuppression were not included. Main outcome measures Pooled prevalence of GCS was compared between patients and controls using chi-square and was further explored by calculating the positive etiologic predictive value (P-EPV) showing the post-test probability of a link between a sore throat and the bacterial finding. P-EPV for GCS was compared with that for group A Streptococci (GAS) using figures from the same publications and patients.Results: Eleven studies were included. The prevalence of GCS among patients versus controls was similar in children (3.15 versus 2.87%, p = .44) but for adults higher in patients (11%) than in controls (5.6%) (p < .0001). The P-EPV for finding GCS in children with a sore throat was 9.3% (0.0–41%). The corresponding P-EPV for GCS in adults with a sore throat was 53% (36–67%) while the corresponding P-EPV for GAS in adults was 94% (90–96%).Conclusions: GCS do not seem associated with the uncomplicated acute sore throat in children but there is support for an association in adults being weaker than for GAS. A possible consequence is to ignore GCS in otherwise healthy patients at their first visit for an uncomplicated sore throat. This would enable a stronger focus on the use of modern point of care tests (POCTs) to detect GAS.

Key points

• There is no current consensus on the pathogenicity of group C beta-hemolytic Streptococcus (GCS) in patients attending for an uncomplicated acute sore throat.
• This systematic literature review concludes it is unlikely that GCS is involved in the uncomplicated sore throat in otherwise healthy children.
• This meta-analysis found a moderate link between GCS and the uncomplicated sore throat in adults.
• The link in adults between GCS and the sore throat is much weaker than the corresponding link for group A beta-hemolytic Streptococcus.