2.Big Data Research in Chronic Kidney Disease.
Xiao-Xi ZENG ; Jing LIU ; Liang MA ; Ping FU
Chinese Medical Journal 2018;131(22):2647-2650
3.Long-term auditory monitoring in children with Alport syndrome based on different degrees of renal injury.
Lining GUO ; Wei LIU ; Min CHEN ; Jiatong XU ; Ning MA ; Xiao ZHANG ; Qingchuan DUAN ; Shanshan LIU ; Xiaoxu WANG ; Junsong ZHEN ; Xin NI ; Jie ZHANG
Journal of Clinical Otorhinolaryngology Head and Neck Surgery 2024;38(1):44-49
Objective:To investigate long-term auditory changes and characteristics of Alport syndrome(AS) patients with different degrees of renal injury. Methods:Retrospectively analyzing clinical data of patients diagnosed AS from January 2007 to September 2022, including renal pathology, genetic detection and hearing examination. A long-term follow-up focusing on hearing and renal function was conducted. Results:This study included 70 AS patients, of which 33(25 males, 8 females, aged 3.4-27.8 years) were followed up, resulting in a loss rate of 52.9%.The follow-up period ranged from 1.1to 15.8 years, with 16 patients followed-up for over 10 years. During the follow-up, 10 patients presenting with hearing abnormalities at the time of diagnosis of AS had progressive hearing loss, and 3 patients with new hearing abnormalities were followed up, which appeared at 5-6 years of disease course. All of which were sensorineural deafness. While only 3 patients with hearing abnormalities among 13 patients received hearing aid intervention. Of these patients,7 developed end-stage renal disease(ESRD), predominantly males (6/7). The rate of long-term hearing loss was significantly different between ESRD group and non-ESRD group(P=0.013). There was no correlation between the progression of renal disease and long-term hearing level(P>0.05). kidney biopsies from 28 patients revealed varying degrees of podocyte lesion and uneven thickness of basement membrane. The severity of podocyte lesion was correlated with the rate of long-term hearing loss(P=0.048), and there was no correlation with the severity of hearing loss(P>0.05). Among 11 cases, theCOL4A5mutationwas most common (8 out of 11), but there was no significant correlation between the mutation type and hearing phenotype(P>0.05). Conclusion:AS patients exhibit progressive hearing loss with significant heterogeneity over the long-term.. THearing loss is more likely to occur 5-6 years into the disease course. Hearing abnormalities are closely related to renal disease status, kidney tissue pathology, and gene mutations, emphasizing the need for vigilant long-term hearing follow-up and early intervention.
Male
;
Child
;
Female
;
Humans
;
Nephritis, Hereditary/pathology*
;
Retrospective Studies
;
Kidney
;
Deafness
;
Hearing Loss/genetics*
;
Kidney Failure, Chronic/pathology*
;
Mutation
4.A Case of Congenital Nephrotic Syndrome due to Diffuse Mesangial Sclerosis.
Jung Jin YU ; Hae Il CHEONG ; Hyun Soon LEE ; Yong CHOI ; Dong Kyu JIN
Journal of the Korean Pediatric Society 1998;41(3):415-419
Diffuse mesangial sclerosis (DMS) is one of the underlying pathology of congenital and infantile nephrotic syndrome. Infants with DMS develop nephrotic syndrome before 2 years of age and progress to end stage renal disease within 3 years of age. The authors experienced a case of isolated DMS in a 4-month-old male infant who had nephrotic syndrome for 1 month. The diagnosis was confirmed on the basis of clinical, laboratory, pathological and molecular genetic findings. This is the 3rd case report of DMS in our country and the 1st case report of isolated DMS confirmed by molecular genetic study.
Denys-Drash Syndrome
;
Diagnosis
;
Humans
;
Infant
;
Kidney Failure, Chronic
;
Male
;
Molecular Biology
;
Nephrotic Syndrome*
;
Pathology
;
Sclerosis*
5.Associations of mean arterial pressure levels with mortality in patients with peritoneal dialysis.
Duo LYU ; Xishao XIE ; Xiaohui ZHANG ; Jianghua CHEN
Journal of Zhejiang University. Medical sciences 2019;48(2):180-185
OBJECTIVE:
To investigate the associations between mean arterial pressure (MAP) and mortality in patients with peritoneal dialysis (PD).
METHODS:
A total of 1737 patients with terminal renal diseases under PD in the First Affiliated Hospital of Zhejiang University from 2008 to 2016 were enrolled. Patients were followed up for 33.0(19.3, 52.4) months. The mean arterial pressure over the first 3 months of PD therapy were calculated. All-cause death and cardiovascular death were assessed using Cox regression models adjusted for demographics, laboratory measurements, comorbid conditions and antihypertensive medications.
RESULTS:
During the follow-up, 208 patients died, among which 95(45.7%) patients died of cardiovascular causes. Compared with patients with MAP >95-<120 mmHg, patients with MAP ≤ 95 mmHg were associated with significantly higher risk of all-cause death (=1.40,95%:1.01-1.93,<0.05); patients with MAP ≥ 120 mmHg were associated with significantly higher risk of all-cause (=2.12,95%:1.32-3.40, <0.01) and cardiovascular morality (=2.55, 95%:1.38-4.70, <0.01). MAP presents a U-shaped association with all-cause mortality and a J-shaped association with cardiovascular mortality.
CONCLUSIONS
Both high MAP and low MAP are associated with higher risk of mortality in PD patients.
Arterial Pressure
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Humans
;
Kidney Failure, Chronic
;
mortality
;
pathology
;
Peritoneal Dialysis
;
Renal Dialysis
;
Risk Factors
6.MR Imaging of Medullary Streaks in Osteosclerosis: A Case Report.
Hak Soo LEE ; Kyung Bin JOO ; Tae Soo PARK ; Ho Taek SONG ; Yong Soo KIM ; Dong Woo PARK ; Choong Ki PARK
Korean Journal of Radiology 2000;1(3):172-174
We present a case of medullary sclerosis of the appendicular skeleton in a patient with chronic renal insufficiency for whom MR imaging findings were char-acteristic. T1- and T2-weighted MR images showed multiple vertical lines (medullary streaks) of low signal intensity in the metaphyses and diaphyses of the distal femur and proximal tibia.
Adult
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Case Report
;
Femur/pathology
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Human
;
Kidney Failure, Chronic/complications
;
*Magnetic Resonance Imaging
;
Male
;
Osteosclerosis/*pathology
;
Tibia/pathology
7.A Four-year Follow-up Case of Oligomeganephronia Detected Early by School Screening Urinalysis.
Ju Yeon HAM ; Nam Hyang KOO ; Ki Soo PAI ; Hyun Yi LIM ; Kee Hyuck KIM
Journal of the Korean Society of Pediatric Nephrology 2003;7(2):223-228
Oligmeganephronia is congenital hypoplasia of kidney with renal pathology showing very small number of nephrons with compensatory hypertrophy of the remaining glomeruli. A 7- year-old girl was referred to our nephrology clinic due to hematuria detected on school screening urinalysis and diagnosed as chronic renal failure and oligomeganephronia on renal biopsy. We are reporting the clinical and histomorphometric changes for the four years follow-up with review of literatures.
Biopsy
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Female
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Follow-Up Studies*
;
Hematuria
;
Humans
;
Hypertrophy
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Kidney
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Kidney Failure, Chronic
;
Mass Screening*
;
Nephrology
;
Nephrons
;
Pathology
;
Urinalysis*
8.A case of a 13-year old girl with Sagliker syndrome.
Jishi LIU ; Ying JI ; Hua LUO ; Li WU ; Wei ZHANG ; Hao ZHANG
Chinese Journal of Pediatrics 2014;52(8):634-635
9.Morphological characteristics of spermatozoa before and after renal transplantation.
Long-Gen XU ; Shi-Fang SHI ; Xiao-Ping QI ; Xiao-Feng HUANG ; Hui-Ming XU ; Qi-Zhe SONG ; Xing-Hong WANG ; Zong-Fu SHAO ; Jun-Rong ZHANG
Asian Journal of Andrology 2005;7(1):81-85
AIMTo investigate the changes of the spermatozoa ultrastructures before and after renal transplantation in uremic patients.
METHODSThe sperm of five uremic patients before and after transplantation and four healthy volunteers were collected and examined by scanning electron microscopy.
RESULTSAbnormal spermatozoa were found in patients pre-transplantation; abnormalities included deletion of the acrosome, absence of the postacrosomal and postnuclear ring, dumbbell-like changes of the head, tail curling, and absence of the mitochondrial sheath in the mid-segment. After renal transplantation, most of the spermatozoa became normal.
CONCLUSIONThere are many abnormalities with regard to the appearance and structure of the head, acrosome, mitochondria and tail of the spermatozoa in uremic patients. The majority of the spermatozoa returned to normal after renal transplantation, but a few still presented some abnormalities possibly relating to the administration of immunosuppressants.
Acrosome ; pathology ; Adult ; Case-Control Studies ; Humans ; Kidney Failure, Chronic ; complications ; Kidney Transplantation ; Male ; Microscopy, Electron ; Renal Dialysis ; Sperm Head ; pathology ; Sperm Tail ; pathology ; Spermatozoa ; pathology ; ultrastructure
10.Spontaneous Splenic Rupture in a Hemodialysis Patient.
Hyun Jung KIM ; Gyeong Won LEE ; Dong Jun PARK ; Jong Deog LEE ; Se Ho CHANG
Yonsei Medical Journal 2005;46(3):435-438
Spontaneous splenic rupture (SSR) in a patient undergoing hemodialysis has been described as an extremely rare and potentially fatal complication. We report here spontaneous splenic rupture in a 52-year-old woman undergoing regular hemodialysis for end-stage renal disease (ESRD). She complained of colicky abdominal pain in the left upper quadrant area and dizziness when she assumed an upright posture. Her vital signs revealed low blood pressure and tachycardia, which was suggestive of hypovolemic shock. Abdomen CT scan showed splenic hematoma and hemoperitoneum. However, she had no history of any event triggering the splenic rupture. An exploratory laparotomy showed a ruptured spleen and an emergency splenectomy was performed. We suggest that spontaneous spleen rupture may be attributed to uremic coagulopathy and heparin-induced coagulopathy.
Female
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Humans
;
Kidney Failure, Chronic/*complications
;
Middle Aged
;
*Renal Dialysis
;
Rupture, Spontaneous
;
Spleen/*pathology
;
Splenic Diseases/*complications/*pathology