Humans ; Kidney ; metabolism ; pathology ; Kidney Failure, Chronic ; metabolism ; therapy ; Kidney Transplantation ; Leptin ; metabolism ; Renal Replacement Therapy

Animals ; Big Data ; Humans ; Kidney ; metabolism ; pathology ; Kidney Failure, Chronic ; metabolism ; pathology ; Natural Language Processing ; Renal Insufficiency, Chronic ; metabolism ; pathology

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

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*

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 ; Humans ; Kidney Failure, Chronic ; mortality ; pathology ; Peritoneal Dialysis ; Renal Dialysis ; Risk Factors

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 ; Case Report ; Femur/pathology ; Human ; Kidney Failure, Chronic/complications ; *Magnetic Resonance Imaging ; Male ; Osteosclerosis/*pathology ; Tibia/pathology

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 ; Female ; Follow-Up Studies* ; Hematuria ; Humans ; Hypertrophy ; Kidney ; Kidney Failure, Chronic ; Mass Screening* ; Nephrology ; Nephrons ; Pathology ; Urinalysis*

Adolescent ; Bone Diseases ; etiology ; Chronic Kidney Disease-Mineral and Bone Disorder ; complications ; pathology ; Facial Bones ; Female ; Humans ; Hyperparathyroidism, Secondary ; complications ; Kidney Failure, Chronic ; complications ; pathology ; Syndrome

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

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 ; Humans ; Kidney Failure, Chronic/*complications ; Middle Aged ; *Renal Dialysis ; Rupture, Spontaneous ; Spleen/*pathology ; Splenic Diseases/*complications/*pathology