OBJECTIVETo summarize the clinical characteristics, diagnosis, treatments and outcomes of perinatal autosomal recessive polycystic kidney disease.

METHODSThe clinical data of one case with infantile polycystic kidney disease diagnosed in perinatal stage and the reports of 11 cases seen in the past 15 years searched in Pubmed, OVID and Elsevier and CNKI, Wanfang database by using the polycystic kidney disease, infant, perinatal, autosomal recessive and case report as keyword were reviewed and analyzed.

RESULTSThe infant was characterized by huge kidneys, severe respiratory and renal compromise. The kidneys were symmetrically enlarged and highly echogenic by ultrasonographic examination and showed high-signal intensity on T2-weighted images by MRI. Histologic analysis showed pulmonary hypoplasia, numerous dilated and elongated tubular structures in the kidney and dilated intrahepatic biliary ducts. Among the 12 cases, 8 cases' presumptive diagnosis was made by prenatal ultrasound revealed enlarged kidneys and oligohydramnios. All cases suffered respiratory distress after birth, and 5 cases complicated pneumothorax. 6 cases died in neonatal stage because of respiratory failure.1 case died 2 m after birth because of renal failure. Five cases are alive and underwent dialysis, nephrectomy or renal transplant.

CONCLUSIONNewborn infants with perinatal autosomal recessive polycystic kidney disease often have poor outcome and died from respiratory and renal failure. Aggressive respiratory support and renal replacement therapy (including nephrectomy, dialysis and transplantation) may give these infants a favorable outcome.

Bronchopulmonary Dysplasia ; etiology ; pathology ; Fatal Outcome ; Humans ; Infant, Newborn ; Infant, Newborn, Diseases ; diagnosis ; genetics ; pathology ; Kidney ; pathology ; Male ; Perinatology ; Polycystic Kidney, Autosomal Recessive ; complications ; diagnosis ; genetics ; pathology ; Renal Dialysis ; Renal Insufficiency ; etiology ; pathology ; Respiratory Insufficiency ; etiology ; pathology ; Retrospective Studies ; Ultrasonography

OBJECTIVETo understand the clinical and pathological characteristics of IgA nephropathy (IgAN) with crescentic formation in children.

METHODSClinicopathological data of 29 children with IgAN accompanied by crescents were analyzed. These patients were divided into two groups according to the percentage of glomeruli affected by crescents more or less than 50%, and their data were compared.

RESULTS(1) CLINICAL FEATURES: all the patients had hematuria and proteinuria, and macrohematuria (86%) and proteinuria were also common, protein excreted in urine was more than 1 g per day in 76% of the patients. The patients with edema, hypertension, and renal insufficiency were less than fifty percent. Nine patients in Group A (glomeruli affected by crescents > or = 50%) were crescentic IgAN. Significantly more cases in Group A had persistent macrohematuria, hypertension and renal failure than in Group B (glomeruli affected by crescents < 50%) (P < 0.05), with especially severe proteinuria (P < 0.01). It was easy to find nephritic syndrome in Group A, and asymptomatic hematuria combined with proteinuria in Group B. (2) Renal pathology: the glomeruli were affected by crescents from 5% to 85%. There were 52% to 85% in Group A, and 5% to 40% in Group B. Most crescents were cellular. All the cases had a diffuse mesangial proliferation and tubular-interstitial injury to different degree. Three cases had crescentic IgAN. Glomerulosclerosis was significantly more often seen in Group A (P < 0.05) and tuft adhesion was more frequently seen in Group B (P < 0.05). (3) Immunofluorescence: All the patients presented deposition of IgA, IgM and C3. There were 45% specimens combined with the deposition of IgG. Five cases showed 'full house' (17%), four of them were in Group A. None had IgA deposition alone.

CONCLUSIONThe main clinical feature of IgAN with crescentic formation were hematuria combined with proteinuria, especially persistent gross hematuria and severe proteinuria. All of them showed diffuse mesangial proliferation and tubular-interstitial injury in morphology of kidney. Most of them had tuft adhesion. The main type of immunofluorescence were IgA + IgM and IgA + IgM + IgG deposition. Some showed 'full house' phenomenon. The clinical manifestation and renal lesions of IgAN with diffuse crescentic formation were worse than IgAN with glomeruli affected by crescents < 50%.

Adolescent ; Biopsy ; Child ; Child, Preschool ; Female ; Glomerulonephritis, IGA ; complications ; pathology ; Hematuria ; etiology ; Humans ; Hypertension ; etiology ; Kidney ; pathology ; Kidney Function Tests ; Male ; Prognosis ; Proteinuria ; etiology ; Renal Insufficiency ; etiology

No abstract available.
Bone Marrow/*metabolism/pathology ; Calcium Oxalate/chemistry/metabolism ; Humans ; Hyperoxaluria/*etiology ; Kidney Calculi/etiology ; Male ; Middle Aged ; Nephrectomy/*adverse effects ; Pancytopenia/*pathology ; Recurrence ; Renal Insufficiency/etiology ; Thyrotropin/blood

Antibodies, Antineutrophil Cytoplasmic ; blood ; Child ; Female ; Hematuria ; etiology ; Humans ; Kidney ; pathology ; physiopathology ; Kidney Function Tests ; Proteinuria ; etiology ; Renal Insufficiency ; etiology ; Vasculitis ; blood ; complications ; pathology

Humans ; Lymphoma, B-Cell ; complications ; pathology ; Lymphoma, Non-Hodgkin ; complications ; pathology ; Male ; Mesentery ; Middle Aged ; Peritoneal Neoplasms ; complications ; pathology ; Renal Insufficiency ; etiology

BACKGROUND/AIMS: Isolated cecal necrosis is a rare cause of the surgical abdomen. Its manifestation is similar to that of acute appendicitis. Thirteen cases, who were pre-diagnosed with acute abdomen and were finally diagnosed with isolated cecal necrosis after operation have been evaluated alongside with literature. METHODS: The records of 13 patients, who had isolated cecal necroses between 1995 and 2011 at Necmettin Erbakan University Meram Medical School's General Surgery Clinic (Turkey), were retrospectively evaluated. RESULTS: Eight of the patients were male, whereas 5 were female. Their mean age was 68.0+/-11.7 (range 51-84) years. All the patients had at least one accompanying disease the most frequent of which were heart failure and chronic renal failure. Ten patients had right hemicolectomy and ileotransversostomy, two had right hemicolectomy and ileostomy, and one had wedge resection to the cecum by the help of linear stapler. Mortality was seen in 5 patients (38%) in the early postoperative period. CONCLUSIONS: Isolated cecal necrosis should be considered in elderly patients with chronic diseases presenting with sudden right lower quadrant pains in the differential diagnosis. Isolated cecal necrosis may have a bad prognosis since it is seen in elderly patients with accompanying problems. Therefore, early diagnosis and immediate surgical management if necessary is important to reduce the risk of morbidity and mortality.
Age Factors ; Aged ; Aged, 80 and over ; Cecum/*pathology ; Colectomy ; Female ; Heart Failure/etiology ; Humans ; Ileostomy ; Male ; Middle Aged ; Necrosis/*diagnosis/mortality/surgery ; Renal Insufficiency, Chronic/etiology ; Retrospective Studies

No abstract available.
Adenocarcinoma/*secondary/surgery ; Chemotherapy, Adjuvant ; Colectomy ; Glomerulonephritis, Membranoproliferative/diagnosis/*etiology/therapy ; Hepatectomy ; Humans ; Liver Neoplasms/*secondary/surgery ; Male ; Middle Aged ; Paraneoplastic Syndromes/diagnosis/*etiology/therapy ; Renal Dialysis ; Renal Insufficiency/etiology ; Sigmoid Neoplasms/*pathology/surgery ; Treatment Outcome

Abnormalities, Multiple ; diagnosis ; pathology ; physiopathology ; Branchio-Oto-Renal Syndrome ; diagnosis ; pathology ; physiopathology ; therapy ; Child ; Deafness ; etiology ; physiopathology ; Diagnosis, Differential ; Ear ; abnormalities ; Female ; Humans ; Kidney ; abnormalities ; Renal Insufficiency ; etiology ; physiopathology ; therapy

OBJECTIVETo evaluate the alterations in renal function after radical nephrectomy (RN) and partial nephrectomy (PN) for renal cell carcinoma (RCC) and to determine the risk factors for the onset of postoperative renal function impairment.

METHODSWe assessed the renal function of 429 T1a RCC patients by investigating the time-dependent changes of the estimated glomerular filtration rate (eGFR) after surgery from August 2003 to August 2010. Univariate and multivariate regression models were used to determine the risk factors for the onset of an eGFR < 60 ml · min⁻¹ · 1.73 m⁻² function, and to evaluate the prognosis for the two groups.

RESULTSThe mean eGFR values (ml · min⁻¹ · 1.73 m⁻²) at postoperative 1, 7 days, 1, 3, 6, 12 and 24 months were 51.4 ± 12.6, 52.1 ± 17.8, 53.2 ± 19.5, 54.6 ± 20.2, 53.8 ± 16.6, 52.7 ± 22.3 and 51.5 ± 18.4 in the RN group and 69.6 ± 18.3, 70.3 ± 19.5, 71.5 ± 21.4, 76.2 ± 22.8, 75.4 ± 19.7, 74.3 ± 16.3 and 73.1 ± 23.2 in the PN group, respectively. The eGFR of the radical nephrectomy group was significantly lower than that of the partial nephrectomy group (P < 0.05). Multivariable analysis revealed that radical nephrectomy and age were risk factors for the onset of postoperative chronic renal dysfunction.

CONCLUSIONSRenal function recovered partially after partial and radical nephrectomy and is maintained constantly after 3 months. Surgical mode and age are risk factors for the onset of postoperative eGFR < 60 ml · min⁻¹ · 1.73 m⁻² impairment. Compared with radical nephrectomy, partial nephrectomy can preserve renal function and reduce the incidence of postoperative chronic renal dysfunction.

Age Factors ; Carcinoma, Renal Cell ; pathology ; physiopathology ; surgery ; Glomerular Filtration Rate ; Humans ; Kidney Neoplasms ; pathology ; physiopathology ; surgery ; Nephrectomy ; adverse effects ; methods ; Postoperative Complications ; physiopathology ; Postoperative Period ; Renal Insufficiency, Chronic ; etiology ; physiopathology ; Risk Factors

OBJECTIVETo study the long-term outcome of idiopathic IgA nephropathy and analyze its clinical and histological prognostic factors.

METHODSWe conducted a retrospective analysis of 495 cases of IgA nephropathy patients in our hospital from 1986 to 2002. Renal survival was analyzed by Kaplan-Meier method, univariate analysis and multivariate Cox regression analysis.

RESULTSThe cumulative renal survival rate was 85% at 10 years and 70.9% at 15 years. Univariate analysis found the following parameters at the time of biopsy to be significantly correlated with poor renal survival: serum creatinine > or = 133 micromol/L (P < 0.001), hypertension (P < 0.05), 24-hour urine protein excretion > or = 3 g (P < 0.001), histological subclass V (P < 0.001), and without the history of gross hematuria (P < 0.05). The Cox regression analysis showed that only the presence of hypertension (HR=7.75, 95%CI 1.02 to approximately 2.19) and serum creatinine level (HR=1.49, 95%CI 1.54 to approximately 38.90) were independent prognostic indicators.

CONCLUSIONSThe findings in Chinese IgA nephropathy patients are in agreement with the results of many other studies worldwide.

Adolescent ; Adult ; Child ; Creatinine ; blood ; Female ; Glomerulonephritis, IGA ; complications ; pathology ; physiopathology ; Humans ; Kidney ; pathology ; physiopathology ; Male ; Middle Aged ; Prognosis ; Regression Analysis ; Renal Insufficiency ; etiology ; pathology ; physiopathology ; Retrospective Studies ; Treatment Outcome