1.T-large granular lymphocytic leukemia presenting as aplastic anemia: a report of five cases and literature review.
Xiao Xia LI ; Jian Ping LI ; Xin ZHAO ; Yuan LI ; You Zhen XIONG ; Guang Xin PENG ; Lei YE ; Wen Rui YANG ; Kang ZHOU ; Hui Hui FAN ; Yang YANG ; Yang LI ; Lin SONG ; Li Ping JING ; Li ZHANG ; Feng Kui ZHANG
Chinese Journal of Hematology 2023;44(2):162-165
2.The efficacy and safety of intravenous sucrose iron therapy for recurrent iron deficiency anemia.
Jing Qian LIU ; Xia Wan YANG ; Xu LIU ; Jing HU ; Xiang Rong HU ; Xiao Xia LI ; Yu Fei ZHAO ; Yi Meng SHI ; Bao Hang ZHANG ; Wen Rui YANG ; Guang Xin PENG ; Xin ZHAO ; Feng Kui ZHANG
Chinese Journal of Hematology 2023;44(5):408-412
Objective: To evaluate the efficacy and safety of intravenous iron supplementation in patients with recurrent iron deficiency anemia (IDA) . Methods: This retrospective analysis of 90 patients with recurrent IDA from May 2012 to December 2021 was conducted, comparing the efficacy and safety of the intravenous iron therapy group and the oral iron therapy group. Results: Among the 90 patients with recurrent IDA, 20 were males and 70 were females, with a median age of 40 (range: 14-85) years. A total of 60 patients received intravenous iron supplementation and 30 received oral iron supplementation. The hematologic response rates in the intravenous iron group were significantly higher than those in the oral iron group at 4 and 8 weeks after treatment [80.0% (48/60) vs 3.3% (1/30) and 96.7% (58/60) vs 46.7% (14/30), all P<0.001, respectively]. The median increase in hemoglobin levels was also significantly higher in the intravenous iron group than in the oral iron group [38 (4, 66) g/L vs 7 (1, 22) g/L at week 4 and 44.5 (18, 80) g/L vs 19 (3, 53) g/L at week 8, all P<0.001]. The intravenous iron group had a significantly higher proportion of patients who achieved normal hemoglobin levels than the oral iron group (55.0% vs 0 and 90% vs 43.3%, all P<0.001, respectively). Iron metabolism indicators were tested before and after 8 weeks of treatment in 26 and 7 patients in the intravenous and oral iron groups, respectively. The median increase in serum ferritin (SF) levels in the intravenous iron group 8 weeks after treatment was 113.7 (49.7, 413.5) μg/L, and 54% (14/26) of these patients had SF levels of ≥100 μg/L, which was significantly higher than the median increase in SF levels in the oral iron group [14.0 (5.8, 84.2) μg/L, t=4.760, P<0.001] and the proportion of patients with SF levels of ≥100 μg/L (P=0.013). The incidence of adverse reactions was 3.3% (2/60) in the intravenous iron group, which was significantly lower than that in the oral iron group [20.0% (6/30), P=0.015]. Conclusion: Intravenous iron supplementation is more effective for hematologic response, faster hemoglobin increase, and higher iron storage replenishment rates compared with oral iron supplementation in patients with recurrent IDA, and it is well tolerated by patients.
Male
;
Female
;
Humans
;
Adolescent
;
Young Adult
;
Adult
;
Middle Aged
;
Aged
;
Aged, 80 and over
;
Anemia, Iron-Deficiency/epidemiology*
;
Sucrose/therapeutic use*
;
Ferric Compounds/therapeutic use*
;
Retrospective Studies
;
Iron/therapeutic use*
;
Hemoglobins/therapeutic use*
3.Clinical and genetic characteristics of children with dopa-responsive dystonia caused by tyrosine hydroxylase gene variations.
Guang Yu ZHANG ; Zhi Jun CAI ; Xiao Li ZHANG ; Lei YANG ; Yi Zhe LI ; Liang Kui WEI ; Yang Ping ZHANG ; Ping Ping CHANG ; Deng Na ZHU
Chinese Journal of Pediatrics 2023;61(4):339-344
Objective: To explore the clinical and genetic characteristics of children with dopa-responsive dystonia (DRD) caused by tyrosine hydroxylase (TH) gene variations. Methods: Clinical data of 9 children with DRD caused by TH gene variations diagnosed in the Department of Children Rehabilitation, the Third Affiliated Hospital of Zhengzhou University from January 2017 to August 2022 were retrospectively collected and analyzed, including the general conditions, clinical manifestations, laboratory tests, gene variations and follow-up data. Results: Of the 9 children with DRD caused by TH gene variations, 3 were males and 6 were females. The age at diagnosis was 12.0 (8.0, 15.0) months. The initial symptoms of the 8 severe patients were motor delay or degression. Clinical symptoms of the severe patients included motor delay (8 cases), truncal hypotonia (8 cases), limb muscle hypotonia (7 cases), hypokinesia (6 cases), decreased facial expression (4 cases), tremor (3 cases), limb dystonia (3 cases), diurnal fluctuation (2 cases), ptosis (2 cases), limb muscle hypertonia (1 case) and drooling (1 case). The initial symptom of the very severe patient was motor delay. Clinical symptoms of the very severe patient included motor delay, truncal hypotonia, oculogyric crises, status dystonicus, hypokinesia, decreased facial expression, and decreased sleep. Eleven TH gene variants were found, including 5 missense variants, 3 splice site variants, 2 nonsense variants, and 1 insertion variant, as well as 2 novel variants (c.941C>A (p.T314K), c.316_317insCGT (p.F106delinsSF)). Nine patients were followed up for 40 (29, 43) months, and no one was lost to follow-up. Seven of the 8 severe patients were treated by levodopa and benserazide hydrochloride tablets and 1 severe patient was treated by levodopa tablets. All the severe patients responded well to levodopa and benserazide hydrochloride tablets or levodopa tablets. Although the weight of the patients increased and the drug dosage was not increased, the curative effect remained stable and there was no obvious adverse reaction. One severe patient developed dyskinesia in the early stage of treatment with levodopa and benserazide hydrochloride tablets and it disappeared after oral administration of benzhexol hydrochloride tablets. Until the last follow-up, motor development of 7 severe patients returned to normal and 1 severe patient still had motor delay due to receiving levodopa and benserazide hydrochloride tablets for only 2 months. The very severe patient was extremely sensitive to levodopa and benserazide hydrochloride tablets and no improvement was observed in this patient. Conclusions: Most of the DRD caused by TH gene variations are severe form. The clinical manifestations are varied and easily misdiagnosed. Patients of the severe patients responded well to levodopa and benserazide hydrochloride tablets or levodopa tablets, and it takes a long time before full effects of treatment become established. Long-term effect is stable without increasing the drug dosage, and no obvious side effect is observed.
Female
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Humans
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Infant
;
Male
;
Benserazide/therapeutic use*
;
Dystonia/genetics*
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Hypokinesia/drug therapy*
;
Levodopa/pharmacology*
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Muscle Hypotonia
;
Retrospective Studies
;
Tyrosine 3-Monooxygenase/genetics*
4.Characteristics of bone marrow compensatory erythropoiesis in hereditary spherocytosis.
Xiao Xia LI ; Yuan LI ; Xin ZHAO ; Guang Xin PENG ; Jian Ping LI ; Lei YE ; Wen Rui YANG ; Kang ZHOU ; Hui Hui FAN ; Yang YANG ; You Zhen XIONG ; Yang LI ; Lin SONG ; Li Ping JING ; Li ZHANG ; Feng Kui ZHANG
Chinese Journal of Hematology 2022;43(2):115-119
Objective: To reveal the compensatory features of bone marrow (BM) erythropoiesis in hereditary spherocytosis (HS) and to explore the effect of diferent hemoglobin levels on this compensation. Methods: Clinical and laboratory data of patients with HS were collected, and the peripheral blood absolute reticulocytes counts value was taken as the surrogate parameter to evaluate the ability of erythropoiesis compensation. BM erythropoiesis compensation in HS with diferent degrees of anemia were evaluated. Results: ①Three hundred and two patients were enrolled, including 115 with compensated hemolytic disease, 74 with mild anemia, 90 with moderate anemia, and 23 with severe anemia. ②Hemoglobin (HGB) was negatively correlated with serum erythropoietin in the decompensated hemolytic anemia group (EPO; rs=-0.585, P<0.001) . ③The median absolute reticulocyte count (ARC) of HS patients was 0.34 (0.27, 0.44) ×10(12)/L, up to 4.25 times that of normal people. The maximum ARC was 0.81×10(12)/L, about 10 times that of normal people. The median ARC of patients with compensated hemolytic disease was 0.29 (0.22, 0.38) ×10(12)/L, up to 3.63 times that of normal people. The median ARC of patients with hemolytic anemia was 0.38 (0.30, 0.46) ×10(12)/L, which was significantly higher than the patients with compensated hemolytic disease, up to 4.75 times that of normal people (z=4.999, P=0.003) . ④ ARC was negatively correlated with HGB in the compensated hemolytic disease group (rs=-0.177, P=0.002) and positively correlated with HGB in the decompensated hemolytic anemia group (rs=0.191, P=0.009) . There was no significant difference in the ARC among patients with mild, moderate, and severe anemia (χ(2)=4.588, P=0.101) . ⑤The median immature reticulocyte production index of the mild, moderate, and severe anemia groups was 13.1% (9.1%, 18.4%) , 17.0% (13.4%, 20.8%) , and 17.8% (14.6%, 21.8%) , respectively; the mild anemia group had lower index values than the moderate and severe anemia groups (P(adj) values were both<0.05) , but there was no significant difference between the latter groups (P(adj)=1.000) . The median immature reticulocyte count of patients in the mild, moderate, and severe groups was 5.09 (2.60, 7.74) ×10(10)/L, 6.24 (4.34, 8.83) ×10(10)/L, and 7.00 (3.07, 8.22) ×10(10)/L, respectively; there was no significant difference among the groups (χ(2)=3.081, P=0.214) . Conclusion: HGB can be maintained at a normal level through bone marrow erythropoiesis, while red blood cells are reduced in HS. However, once anemia develops, the bone marrow exerts its maximum erythropoiesis capacity and does not increase, regardless of anemia aggravation or serum EPO increase.
Bone Marrow
;
Erythropoiesis
;
Humans
;
Reticulocyte Count
;
Reticulocytes
;
Spherocytosis, Hereditary
5.Normative values of cervical sagittal alignment according to the whole spine balance: Based on 126 asymptomatic Chinese young adults.
Yan Chao TANG ; Wen Kui ZHAO ; Miao YU ; Xiao Guang LIU
Journal of Peking University(Health Sciences) 2022;54(4):712-718
OBJECTIVE:
To explore the normal distribution of cervical sagittal alignment and the relationship between cervical alignment and global spine balance in asymptomatic young adults.
METHODS:
A cohort of 272 asymptomatic Chinese adults (including 161 males and 111 females, with an average age of (23.2±4.4) years, ranging from 18 to 45 years) were prospectively recruited from November 2011 to December 2014. The C0-C2 angle, disk angles from C2-C3 to C6-C7, vertebral angles from C3 to C7, T1 slope, thoracic kyphosis (TK), lumbar lordosis (LL), pelvic incidence (PI), sacral slope (SS), C2-C7 sagittal vertical axis (C2-C7SVA), center of gravity of head to C7SVA (CGH-C7SVA), C7-S1SVA were measured and statistically analyzed. All the subjects were categorized with the Roussouly classification and the cervical morphologies were evaluated as lordotic, straight, sigmoid or kyphotic. Spinal sagittal alignment parameters were compared between different sexes and Roussouly classifications with independent student t test, analysis of variance (ANOVA) or Chi-square test. Correlations between cervical sagittal alignment and global spine sagittal alignment were calculated using the Pearson and Spearman correlation coefficient. Linear regression analysis was performed.
RESULTS:
Sixty-seven males and 59 females aged from 18 to 30 years old were included in the study. The mean value of C0-C7 was 26.0°±12.8°, composed of 15.2°±6.7° for C0-C2, 9.1°±12.1° for sum of disk angles from C2-C3 to C6-C7, and 1.4°±10.2° for sum of vertebral angles from C3 to C7. C2-C7SVA [(18.6±7.9) mm] and CGH-C7SVA [(22.9±12.3) mm]were offset ideally by C7-S1SVA [(-21.6±31.0) mm]. Males had a larger T1 slope (P < 0.05) and accordingly, a larger cervical lordosis C2-C7 (P < 0.01) and C0-C7 angle (P < 0.01) than females. Males had a smaller C7-S1SVA (P < 0.01) and accordingly, a smaller CGH-C7SVA (P=0.165) than females. Significant difference was found between cervical alignment of different Roussouly types (P < 0.01). In general, a larger LL was consistent with a set of larger TK, C2-C7angle, C0-C7 angle, and vice versa. There was no significant correlation between cervical morphology and the Roussouly classification (Chi-square=10.548, P=0.308). There was significant correlation between cervical alignment and T1 slope (P < 0.01), TK (P < 0.01). There was significant correlation between adjacent segmental angles from T1 slope up to C0-C2 angle (P < 0.05).
CONCLUSION
Normative values of each vertebral angle and disk angle were established. The cervical lordosis occurred mainly at C0-C2 and disk levels, which was influenced by parameters of other parts of the spine, such as T1 slope, TK and the Roussouly classification. There was significant correlation between adjacent disk angles.
Adolescent
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Adult
;
Cervical Vertebrae/diagnostic imaging*
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China
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Female
;
Humans
;
Kyphosis
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Lordosis/diagnostic imaging*
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Male
;
Thoracic Vertebrae
;
Young Adult
6.Mechanism of Bushen Huatan Prescription in Regulating Th17/Treg Balance in Ovariectomized Osteoporosis Rats
Zhang-kui TAN ; Guang-wen ZHOU ; Yan ZHANG ; Wei ZHU ; Xiao-xue WU ; Meng-xin XIONG ; Shi-yi HUANG ; Nan XIANG
Chinese Journal of Experimental Traditional Medical Formulae 2021;27(5):33-40
Objective:To study the effect of Bushen Huatan prescription on helper T cell 17 (Th17)/T regulatory cells (Treg) balance of immune T cell subsets in the prevention and treatment of postmenopausal osteoporosis. Method:Sixty 6-month-old female SD rats were randomly divided into sham operation group, model group, estradiol valerate group (0.184 mg·kg-1) and Bushen Huatan prescription low, medium and high groups (4.7, 9.4, 18.8 g·kg-1) according to the random number table. All the groups except the sham operation group received ovariectomy to make postmenopausal osteoporosis model. Intragastric administration was started 1 week after operation, and the rats in model group and sham operation group received equal volume of normal saline, once a day for 12 weeks. Microcomputed tomography (Micro CT) was then used to detect bone mass and microstructure of rats, the contents of Forkhead box protein (Foxp3) and retinoic acid related nuclear orphan receptor (ROR
7.Implications, spiritual characteristics and practical significance of Chinese schistosomiasis control culture
Wei-Cheng DENG ; Yue-Sheng LI ; Xiang-Hui CHENG ; Guang-Hui REN ; Hong-Bin HE ; Yi-Biao ZHOU ; Yue-Yun ZHANG ; Ben-Jiao HU ; Han-Qiu LIU ; Shi-Kui LU ; Sheng-Ming LI ; Xiao-Nong ZHOU
Chinese Journal of Schistosomiasis Control 2020;32(3):222-224
The culture of schistosomiasis control is specific in the history of Chinese culture. Broadly speaking, the culture of schistosomiasis control is a summary of specific social mood, social consciousness and material culture created by Chinese populations during the progress of schistosomiasis control since the founding of the People’s Republic of China. Narrowly speaking, the culture of schistosomiasis control is the spiritual culture that is jointly created and nurtured by schistosomiasis control workers since the founding of the People’s Republic of China. The spiritual features of Chinese schistosomiasis control culture are characterized by the patriotism and care about the people, the matter-to-fact attitude, the pioneering and enterprising spirit, and the spirit of sacrifice and dedication. The ultimate goal of the research on the culture of schistosomiasis control is to facilitate the achievement of the strategic goal of Healthy China 2030 as scheduled, accelerate the progress towards elimination of schistosomiasis, and to promote the sustainable development of schistosomiasis control in China.
8.Challenges and countermeasures of schistosomiasis control in Hunan Province in the new era
Sheng-Ming LI ; Wei-Cheng DENG ; Xiang-Hui CHENG ; Hong-Bin HE ; Yi-Biao ZHOU ; Jie ZHOU ; Ben-Jiao HU ; Han-Qiu LIU ; Shi-Kui LU ; Yue-Sheng LI ; Xiao-Nong ZHOU ; Guang-Hui REN
Chinese Journal of Schistosomiasis Control 2020;32(3):225-229
This paper describes the current epidemic characteristics and endemic status of schistosomiasis, analyzes the main challenges of schistosomiasis control and proposes the emphasis and interventions for future schistosomiasis control activities in Hunan Province, so as to provide insights into the elimination of schistosomiasis in Hunan Province.
9.Clinical characteristics of novel coronavirus cases in tertiary hospitals in Hubei Province.
Kui LIU ; Yuan-Yuan FANG ; Yan DENG ; Wei LIU ; Mei-Fang WANG ; Jing-Ping MA ; Wei XIAO ; Ying-Nan WANG ; Min-Hua ZHONG ; Cheng-Hong LI ; Guang-Cai LI ; Hui-Guo LIU
Chinese Medical Journal 2020;133(9):1025-1031
BACKGROUND:
The 2019 novel coronavirus (2019-nCoV) causing an outbreak of pneumonia in Wuhan, Hubei province of China was isolated in January 2020. This study aims to investigate its epidemiologic history, and analyze the clinical characteristics, treatment regimens, and prognosis of patients infected with 2019-nCoV during this outbreak.
METHODS:
Clinical data from 137 2019-nCoV-infected patients admitted to the respiratory departments of nine tertiary hospitals in Hubei province from December 30, 2019 to January 24, 2020 were retrospectively collected, including general status, clinical manifestations, laboratory test results, imaging characteristics, and treatment regimens.
RESULTS:
None of the 137 patients (61 males, 76 females, aged 20-83 years, median age 57 years) had a definite history of exposure to Huanan Seafood Wholesale Market. Major initial symptoms included fever (112/137, 81.8%), coughing (66/137, 48.2%), and muscle pain or fatigue (44/137, 32.1%), with other, less typical initial symptoms observed at low frequency, including heart palpitations, diarrhea, and headache. Nearly 80% of the patients had normal or decreased white blood cell counts, and 72.3% (99/137) had lymphocytopenia. Lung involvement was present in all cases, with most chest computed tomography scans showing lesions in multiple lung lobes, some of which were dense; ground-glass opacity co-existed with consolidation shadows or cord-like shadows. Given the lack of effective drugs, treatment focused on symptomatic and respiratory support. Immunoglobulin G was delivered to some critically ill patients according to their conditions. Systemic corticosteroid treatment did not show significant benefits. Notably, early respiratory support facilitated disease recovery and improved prognosis. The risk of death was primarily associated with age, underlying chronic diseases, and median interval from the appearance of initial symptoms to dyspnea.
CONCLUSIONS
The majority of patients with 2019-nCoV pneumonia present with fever as the first symptom, and most of them still showed typical manifestations of viral pneumonia on chest imaging. Middle-aged and elderly patients with underlying comorbidities are susceptible to respiratory failure and may have a poorer prognosis.
Adult
;
Aged
;
Aged, 80 and over
;
Betacoronavirus
;
China
;
Coronavirus Infections
;
complications
;
diagnostic imaging
;
Female
;
Fever
;
etiology
;
Humans
;
Male
;
Middle Aged
;
Pandemics
;
Pneumonia, Viral
;
complications
;
diagnostic imaging
;
Tertiary Care Centers
;
Tomography, X-Ray Computed
;
Young Adult
10. Clinical characteristics of novel coronavirus cases in tertiary hospitals in Hubei Province
Kui LIU ; Yuan-Yuan FANG ; Yan DENG ; Wei LIU ; Mei-Fang WANG ; Jing-Ping MA ; Wei XIAO ; Ying-Nan WANG ; Min-Hua ZHONG ; Cheng-Hong LI ; Guang-Cai LI ; Hui-Guo LIU
Chinese Medical Journal 2020;133(0):E002-E002
Background:
A novel coronavirus (2019-nCoV) causing an outbreak of pneumonia in Wuhan, Hubei province of China was isolated in January 2020. This study aims to investigate its epidemiological history, and analyzed the clinical characteristics, treatment regimens and prognosis of patients infected with 2019-nCoV during this outbreak.
Methods:
Clinical data from 137 2019-nCoV-infected patients admitted to the respiratory departments of nine tertiary hospitals in Hubei province from December 30, 2019 to January 24, 2020 were collected, including general status, clinical manifestations, laboratory test results, imaging characteristics, and treatment regimens.
Results:
None of the 137 patients (61 males, 76 females, aged 20-83 years, mean age 55 ± 16 years) had a definite history of exposure to Huanan Seafood Wholesale Market. Major initial symptoms included fever (112/137, 81.8%), coughing (66/137, 48.2%), and muscle pain or fatigue (44/137, 32.1%), with other, less typical initial symptoms observed at low frequency, including heart palpitations, diarrhea, and headache. Nearly 80% of the patients had normal or decreased white blood cell counts, and 72.3% (99/137) had lymphocytopenia. Lung involvement was present in all cases, with most chest computed tomography scans showing lesions in multiple lung lobes, some of which were dense; ground-glass opacity co-existed with consolidation shadows or cord-like shadows. Given the lack of effective drugs, treatment focused on symptomatic and respiratory support. Immunoglobulin G was delivered to some critically ill patients according to their condition. Systemic corticosteroid treatment did not show significant benefits. Notably, early respiratory support facilitated disease recovery and improved prognosis. The risk of death was primarily associated with age, underlying chronic diseases, and median interval from the appearance of initial symptoms to dyspnea.
Conclusions
The majority of patients with 2019-nCoV coronavirus pneumonia present with fever as the first symptom, and most of them still showed typical manifestations of viral pneumonia on chest imaging. Middle-aged and elderly patients with underlying comorbidities are susceptible to respiratory failure and may have a poorer prognosis.

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