Objective To investigate early clinical manifestations of osteogenic sarcoma to help establishment of an early diagnosis of the disease.Methods A total of 92 patients with osteogenic sarcoma in the extremities were admitted to our hospital from April 1984 to October 2002. Of the 92 patients, 71 (42 males and 29 females; averaged age 17.4 years, range 666 years; illness course 1-28 weeks) had a complete record of their medical history and examination. From their first medical visits, we obtained their clinical symptoms, physical sings, diagnoses, and duration of the delayed diagnoses. The patients were pathologically confirmed as having osteogenic sarcoma in the extremities, with the lesions located in the distal femur in 38 patients, proximal tibia in 22, proximal femur in 3, proximal fibula in 3, proximal humerus in 2, distal tibia in 2, and distalradius in 1. Results Of the 71 patients, 70 had a local pain and/or a palpable mass, 37 had a persistent pain with no difference between day and night, 23 had an intermittent pain, and 11 had a nocturnal pain. Of the 71 patients, 42 had an initial pain related to trauma, and 3 of the 42 patients had a pathologic fracture. The patients with the local mass had a delayed diagnosis of osteogenic sarcoma with a delayed duration of 1-14 weeks, averaged 4 weeks; however, the patients without the local mass had a delayed diagnosis of this disease, with a delayed duration of 3-30 weeks averaged 14 weeks. In the patients undergoing an X-ray examination at the first medical visit, the duration of the delayed diagnoses was 1-20 weeks, averaged 8 weeks, but in the patients without an X-ray examination at first, the duration was 4-30 weeks, averaged 16 weeks. Conclusion Intermittent and persistent pains and local masses are the most characteristic clinical manifestations in the early stage of osteogenic sarcoma. A history of trauma often helps to make a diagnosis of the disease. Carefulclinical examination and observation should be given to adolescent patients whohave a recurrent pain around the joint.
Objective To investigate the clinical manifestations, CT and prognosis of pontine hemorrhage. Methods Analyze the summarized clinical data of 21 patients with pontine hemorrhage retrospectively. Results The period of 46-70 years old was vulnerable to pontine hemorrhage,and hypertension was the major risky factor of it. The death rate of pontine hemorrhage inceases when the bleeding beyond 5 ml. Nine of the 21 patients survived. Conclution The prognosis was highly related to the bleeding amount, the position of bleeding and the complication.
ObjectiveTo explore the effects of cytokines on Febrile seizures (FS) in children with febrile seizures (Febrile seizures), febrile seizures duration and prognosis, and to explore the correlation between cytokines and the clinical manifestations and prognosis of FS. MethodsA retrospective analysis was performed on 121 children with FS (77 cases in the simple FS group and 44 cases in the complex FS group) who were treated in the pediatrics department of the Maternal and Child Health Hospital of Inner Mongolia Autonomous Region from January 2021 to October 2022 as the experimental group, including 71 males and 50 females, with a male-to-female ratio of 1.42:1, according to the type of attack (93 cases in the comprehensive group, 44 cases in the complex FS group). The focal group (28 cases) and convulsion duration (91 cases in <5 min group and 30 cases in ≥5 min group) were divided into groups, and 127 cases of children with fever but no convulsions were compared with the control group. In addition, 121 children with FS were followed up for 1 year by neurology specialist outpatient department and telephone follow-up. According to the follow-up, they were divided into the first course group, the relapse group and the secondary epilepsy group, so as to further explore the correlation between cytokines and the prognosis of children with FS. ResultsExperimental group compared with control group: Serum IL-1β (1.38 pg/mL), IL-2 (2.26 pg/mL), IL-4 (1.53 pg/mL), IL-6 (10.51 pg/mL), IL-10 (3.09 pg/mL), IL-12p70 (1.74 pg/mL), TNF-α (2.11 pg/mL), IFN-γ (46.56 pg/mL), IL-1β (1.38 pg/mL), IL-1β (1.26 pg/mL), IL-4 (1.53 pg/mL), IL-6 (10.51 pg/mL), IL-10 (3.09 pg/mL), IL-12P70 (1.74 pg/mL), TNF-α (2.11 pg/mL), IFN-γ (46.56 pg/mL). IFN-α (25.92 pg/mL) levels were higher, and the differences were statistically significant (P<0.05). There was no significant difference between the simple group and the complex group (P>0.05). <5 min group compared with control group: serum levels of IL-2 (2.32 pg/mL), IL-4 (1.53 pg/mL), IL-6 (9.65 pg/mL), IL-12p70 (1.74 pg/mL), TNF-α (2.11 pg/mL), IFN-γ (44.63 pg/mL), IFN-α (29.67 pg/mL) were higher, and the differences were statistically significant (P<0.05). Compared with control group, the levels of IL-2 (2.06 pg/mL), IL-6 (14.67 pg/mL), IL-12p70 (1.97 pg/mL), IFN-γ (58.56 pg/mL) and IFN-α (17.50 pg/mL) in ≥5 min group were higher, and the differences were statistically significant (P<0.05). ROC curve analysis showed that serum IFN-α had a high predictive value for FS onset, the cut-off point was 8.64pg/ml, and the sensitivity and specificity were 75.63% and 76.38%, respectively. There was no significant difference between the first course of disease group, relapse group and secondary epilepsy group. ConclusionSerum proinflammatory cytokines IL-1β, IL-2, IL-6, IL-12p70, TNF-α, IFN-γ, IFN-α and anti-inflammatory cytokines IL-4 and IL-10 are involved in the pathogenesis of FS. There was no correlation between the simplicity and complexity of serum cytokines. IL-2, IL-6, IL-12p70, IFN-γ, IFN-α were positively correlated with the duration of convulsion. When serum IFN-α>8.64 pg/ml, the possibility of FS attack increased.
ObjectiveTo summarize clinical electrophysiological features and efficacy of some of Anti-epileptic drugs(AEDs) of Juvenile myoclonic epilepsy (JME). MethodsClinical electrophysiological information of 101 outpatients with JME observed at Xuanwu Hospital from Jul. 2001 to Sep. 2014 was retrospectively analyzed, including the seizure types, trigger factors, electroencephalogram. We followed some of these patients and compared the efficacy between different AEDs. Result According to different seizure types, there are four subtypes: Myoclonus (MJ) only 11.88%, MJ+generalized tonic-clonic seizure(GTCS) 75.24%, MJ+GTCS+Absence(Abs) 11.88%, MJ+Abs 1.00%. Patients with typical ictal generalized poly-spike and waves (PSW) or spike and waves (SW) or spikes account for 96.80%. And 75.00% of patients have no MJ and 91.80% have no GTCS with valproic acid monotherapy. 65.00% and 88.24% of patients were seizure free of MJ and GTCS recpectively. But the difference of efficacy between these two drugs have no statistically significance. Sleep deprivation was the primary trigger factors, accounting for 16.83%. ConclusionJME has clinical heterogeinety, clinicians should fully understand the whole condition of JME individual, including their clinical manifestation, EEG features, reaction to AEDs, trigger factors, habitual patterns and so on, in order to help making individualized therapy.
ObjectivesTo compare the clinical features of combined pulmonary fibrosis and emphysema (CPFE) and idiopathic pulmonary fibrosis (IPF).MethodsEighty-three patients diagnosed as CPFE or IPF for the first time were retrospectively analyzed from June 2014 to July 2018 in Nanjing Drum Tower Hospital, including 47 patients in the CPFE group and 36 in the IPF group. The demographic characteristics, clinical manifestations, pulmonary function, cardiac ultrasound, blood gas analysis and prognosis of the two groups were compared.ResultsThe proportion of smokers in the CPFE group was higher than IPF group (P<0.05), but dyspnea was lower (P<0.05). The FVC, FVC%pred, FEV1, FEV1%pred and VC% of the CPFE group were higher than IPF group (P<0.05), while FEV1/FVC%pred in the IPF group was higher than CPFE group (P<0.05). DLCO/VA%pred of CPFE group decreased more significantly than IPF group (P<0.05), RV/TLC%pred of CPFE group increased annually, while decreased annually in IPF group (P<0.01). The RV%pred of CPFE increased annually, while that of IPF group decreased annually (P<0.05). There was no significant difference in arterial oxygen pressure and pulmonary artery pressure between the two groups. As for prognosis, the 1- and 3-year survival rate of the CPFE group were 87.9% and 73.8% respectively, those of the IPF group were 84.1% and 65.8% respectively, and no significantly difference was observed between two groups (P=0.95).ConclusionsCompared with IPF, patients with CPFE usually have more smokers, less proportion of dyspnea, almost normal lung volume, more rapidly decreased DLCO/VA%pred, and no significant difference in prognosis.
Objective To investigate the clinical manifestation and radiological features of unknownetiology ostearthritic disease in Shigeng village of Chongzhou city in Sichuan province so as to lay a basis for further study. Methods The data were analyzed retrospectively. The epidemiology investigation, physical examination, movement functional assessment, lab test and X-ray examination were performed on 116 villagers of 3 years and more than. Quantitative analysis on roentgenography was conducted. Results Sixty-four villagers had unknown ostearthritic disease，the prevalence was 55.2%；17 patients were limp, the prevalence of limp was 26.6%，onset age mainly focused under 40 years. Main manifestation of osteoarthritic disease was fixed pain of knee joint or hip joint, and bilateral pain was significantly more than single pain, but local fever and swelling were not obvious. Mild or moderate pain account for 73.5% of patients, and pain was aggravated after physical labor or movement and could be relieved after rest. Joint pain had no influence on joint movement function for long time, leading to a limp due to the joint functional disorders at late stage. Physical examination illustrated the flexion contracture of the hip and limitation of internal and external rotation. The Harris scores were 86.5±17.1(left hip) and 86.3±17.2. 46 (right hip), and the excellent or good result was achieved in 46 patients, fair and poor results in 18 patients. The average HSS scores were 88.4±9.3(left knee) and 88.2±11.0(right knee). The excellent or good result was achieved in 61 patients, fair and poor results in 3 patients. The X-ray films showed degenerative disorders of hip joint such as narrowing of the joint space (47.6%)，articular surface sclerosis and deformation (30.2%), shallow and increased density acetabulum, increased obliquity and insuffi cient coverage of the femoral head by the acetabulum, and increased femoral neck-shaft angles. Radiological features of knee joint were bony spur or bony bridge，osteoporosis，incomplete and thickening articular surface with sclerosis and deformation，degenerative disorders. Conclusion Main clinical manifestation of unknown osteoarthritic disease in Chongzhou city was fixed pain of bilateral knee joint or combined with hip joint pain at early stage，and functional limitation in hip joint at late stage. Main radiological feature was hip joint degeneration. To effectively control the osteoarthritic disease ofthis area, much more etiology researches should be done.
ObjectiveTo explore the clinical characteristics of different types of prolactinoma and the therapeutic effect of bromocriptine.MethodsThe medical records of patients with prolactinoma treated by bromocriptine from January 2010 to December 2016 were retrospectively analyzed, and the patients were followed up.ResultsA total of 106 cases of prolactinoma were included, in whom 67 were microprolactinomas, 31 were macroprolactinomas, and 8 were giant prolactinoma. There were differences in the distributions of gender and age, prolactin level, clinical manifestations and the effective dose of bromocriptine among the three groups (P<0.05). After the treatment of bromocriptine, the level of serum prolactin was restored to normal in 61 cases in microprolactinoma group, 26 cases in macroprolactinoma group and 6 cases in giant prolactioma group. For improvement of the main symptoms, there were 63 patients in microprolactinoma group, 27 in macroprolactinoma group and 6 in giant prolactioma group. Furthermore, the shrink or disappearance was achieved in 28 patients in microprolactinoma group, 23 in macroprolactinoma group, and 8 in giant prolactioma group. The statistical results showed no significant difference in normal prolactin level and improvement of symptoms among the three groups (P>0.05), but the reduction of tumor volume were statistically different (P<0.05).ConclusionsMicroprolactinomas and macroprolactinomas are mostly seen in childbearing-aged women with main manifestations of menstrual disorders and lactation, while giant prolactinomas are mostly seen in middle-aged men, with main manifestations of headaches and visual field disorders. Bromocriptine has a good effect on prolactin adenomas with various sizes. Therefore bromocriptine should be the first choice for different types of prolactinomas.
ObjectiveTo observe and investigate the related factors that might affect clinical features of familial exudative vitreoretinopaty (FEVR) patients. MethodsA retrospective chart study. From January 2012 and December 2021, 42 patients with 84 eyes with a diagnosis of FEVR from Department of Ophthalmology, Peking University People's Hospital were included in the study. The patients came from 42 separate families. There were 31 males and 11 females, with an average age of first diagnosis was 16.6±33.7 months. There were 21 patients referred from other hospitals for the fundus disease found in eye screening after birth, 21 patients were first seen in our hospital. There were 4 and 38 premature and full-term infants, respectively. Two patients with a positive family history of FEVR. All patients are FEVR stages 1-5. The wide-angle digital pediatric retinal imaging system after general anesthesia for fluorescein fundus angiography (FFA) examination were performed for patients aged ＜5 years. If patients ≥ 5 years old, routine FFA examination was performed. Sixty-eight first-degree relatives from 28 families undergo routine fundus examinations and FFA examination. Genetic examination was performed for 26 families, including 26 probands and 57 first-degree relatives. Genetic examination were performed on gene the coreceptor of low density lipoprotein receptor-associated protein 5 (LRP5), Wnt receptor coiled protein 4 (FZD4), Norrie disease (NDP), tetraporin 12 (TSPAN12), catenin β1 (CTNNB1) genes known to be involved in FEVR. The clinical features and the genotype of FEVR were observed in relation to the clinical phenotype. ResultsAmong the 42 patients, 13 patients were first observed by strabismus and nystagmus, with the median age of 12 months. Eight patients were complained non-chasing or vision-related symptoms. Among the 84 eyes, FEVR stage 1 or 2, 3 or 4, and 5 were 50 (59.5%, 50/84), 31 (36.9%, 31/84), and 3 (3.6%, 3/84) eyes, respectively. Among the 23 patients who were > 3 months at first diagnosis, 16 patients had at least one eye severer than stage 3 (69.6%, 16/23). Of the 68 first-degree relatives, 22 (32.4%, 22/68) had FEVR-like changes. Among the 26 families that underwent genetic detection, 13 families (50%, 13/26) of 16 variants of FEVR-related genes were detected, of which 10 mutations of LRP5 gene were the most common. There were 10 families with single gene mutations, including 6, 2 and 2 families of LRP5, FZD4 and CTNNB1 genes, respectively. One family of LRP5 gene mutations were compound heterozygous mutations, 1 family with LRP5 gene mutaition combined with NDP gene mutation, and 1 family with LRP5 and TSPAN12 gene mutation. Among the proband with FEVR pathogenic genes, 6 cases with similiar stage of both eyes, and 7 cases with inconsistent disease stages, and there was no obvious correlation between gene mutations and clinical phenotypes. ConclusionIn addition to the age of first diagnosis, no exact factors affecting the clinical manifestations of FEVR are found, and the association between clinical phenotypic and genetic heterogeneity still needs to be further explored.
Objective To improve the knowledge of pulmonary mucosa-associated lymphoid tissue (MALT)lymphoma. Methods A patient diagnosed as pulmonary MALT lymphoma was reported and related literatures were reviewed. Results The patient was a 58-year-old male,admitted due to intermittent fever,cough,sputum production,chest tightness and fatigue for 4 years.The patient was diagnosed as "pulmonary tuberculosis and tuberculous pleurisy" in other hospital and received anti-tuberculosis treatment for 3 years.The CT of the chest showed consolidation in the right middle lobe,right low lobe and left lower lobe with bronchial ventilation levy,miliary nodules in the right middle lobe,interstitial thickening,and right pleural effusion.Ultrasound guided lung biopsy pathology of the right lung showed diffuse small lymphocytes infiltration.The immunohistochemistry showed positive staining of CD20,CD79α and Vim,and weakly positive staining of Ki67(15%).Therefore,the patient was finally diagnosed pulmonary MALT lymphoma. Conclusions Pulmonary MALT lymphoma has no specific clinical manifestations,so is easy to be misdiagnosed as pulmonary tuberculosis,pneumonia or lung cancer.The patients with suspicious pulmonary MALT lymphoma should undergo percutaneous lung biopsy,transbronchial lung biopsy or open lung biopsy as soon as possible for immunohistochemistry staining to confirm the diagnosis.
Hydroxychloroquine is widely used in a variety of autoimmune diseases. However, long-term use of hydroxychloroquine can cause severe retinopathy, which has a complex pathogenic mechanism and diverse clinical manifestations, mainly manifested as photoreceptor and retinal pigment epithelial damage and irreversible vision loss. Identifying damage before retinitis pigment epithelium lesions preserve central vision, so early detection is crucial to slow disease progression and reduce vision loss. The development of multimodal imaging technology and the issuance of the latest treatment guidelines provide a powerful tool for the early screening and treatment of hydroxychloroquine retinopathy. Proficient in the latest guidelines for the treatment of hydroxychloroquine can better guide clinicians to do a good job in disease screening and management, recommend risks, safe dosages and appropriate screening procedures to patients and strengthen the prevention of hydroxychloroquine retinopathy, which will help save the vision of more patients and reduce the waste of medical resources.