BACKGROUNDPreeclampsia (PE) is a serious idiopathic disease posing a threat to both mothers and fetuses' lives during pregnancy, whose main diagnostic criteria include hypertension with proteinuria. However, American College of Obstetricians and Gynecologists (ACOG) updated the diagnostic criteria for PE and reduced the diagnostic value of proteinuria for patients with PE. Qualitative analysis of the diagnostic value of 24-h proteinuria for patients with PE in China was conducted to evaluate the diagnostic criteria value in the latest ACOG guideline.

METHODSComplete clinical data of 65 patients with hypertensive disorder in pregnancy (HDP) were collected. All patients were delivered to and hospitalized in Renji Hospital. Adverse outcome was defined in case of the emergence of any serious complication for a mother or the fetus. A retrospective study was conducted according to ACOG guideline, to analyze the relationship between each diagnostic criteria of ACOG guideline and maternal and perinatal outcomes. Spearman correlation test was used to detect the association between each diagnostic criterion, its corresponding value, and the adverse pregnancy outcome. Logistic regression was performed to verify the result of Spearman correlation test.

RESULTSOf 65 HDP patients, the percentage of adverse pregnancy outcome was 63.1%. Adverse pregnancy outcomes constitute diversification. There were 55 cases with 24-h proteinuria value ≥0.3 g, of which the adverse outcome rate was 74.5%. While adverse pregnancy outcomes did not appear in the rest 10 HDP patients with proteinuria <0.3 g/24 h. The statistic difference was significant (P = 0.000). However, no significant difference was found in other criteria groups (impaired liver function: P = 0.417; renal insufficiency: P = 0.194; thrombocytopenia: P = 0.079; and cerebral or visual symptoms: P = 0.296). The correlation coefficient between 24-h proteinuria ≥0.3 g and adverse pregnancy outcomes was 0.557 (P < 0.005). Impaired liver function (P = 0.180), renal insufficiency (P = 0.077) and cerebral or visual symptoms (P = 0.118) were not related to adverse outcomes. The 24-h proteinuria value (HDP: r = 0.685; PE: r = 0.521), liver enzyme value (HDP: r = 0.519; PE: r = 0.501), and creatinine value (HDP: r = 0.511; PE: r = 0.398) were associated with adverse pregnancy outcomes both in PE and HDP, and the corresponding logistic regression equation can be produced.

CONCLUSIONSThe 24-h proteinuria value is still an important diagnostic criterion for PE, and deletion of 24-h proteinuria value from diagnostic criteria for severe PE was not recommended. The diagnostic criteria in ACOG guideline need to be verified in Chinese women.

Adult ; China ; Female ; Humans ; Middle Aged ; Pre-Eclampsia ; diagnosis ; Pregnancy ; Pregnancy Outcome ; Proteinuria ; diagnosis ; etiology ; Retrospective Studies ; Young Adult

We treated a 54-year-old woman who was suffering from membranoproliferative glomerulonephritis associated with a complete type of hydatidiform mole. The renal manifestations were proteinuria and hematuria. A renal biopsy, performed before gynecologic management, disclosed focal and segmental subendothelial deposits with a proliferation of the mesangial cell and showed irregularly thickened capillary loops by light and electronmicroscoy. Genralized edema, proteinuria and hematuria were completely recovered by suction and curettage of the hydatidiform mole with prophylactic chemotherapy. The clinical manifestation of earlier presented 3 cases have been the nephrotic syndrome. The common feature of them was a complete remission of the nephropathy after the removal of the hydatidiform mole. The relationship between the hydatidiform mole and glomerulonephritis remains unresolved at present. But we concluded that the hydatidiform mole might be a cause of glomerulonephritis in this case.
Case Report ; Diagnosis, Differential ; Edema/etiology ; Female ; Glomerulonephritis, Membranoproliferative/pathology ; Glomerulonephritis, Membranoproliferative/etiology* ; Hematuria/etiology ; Human ; Hydatidiform Mole/therapy ; Hydatidiform Mole/diagnosis* ; Hydatidiform Mole/complications* ; Middle Age ; Pregnancy ; Proteinuria/etiology ; Uterine Neoplasms/therapy ; Uterine Neoplasms/diagnosis* ; Uterine Neoplasms/complications*

OBJECTIVETo analyze the clinical features and gene mutations of 6 Chinese children with Dent's disease.

METHODThe clinical and laboratory data of 6 children with Dent's disease were summarized. CLCN5 gene was analyzed using PCR amplification and DNA sequencing.

RESULTAll the six patients presented with low molecular weight proteinuria and hypercalciuria, including 3/6 hematuria, 4/6 nephrocalcinosis, 3/6 hypophosphatemia, 1/6 rickets. Six mutations of the CLCN5 gene were revealed, including L594fsX595, R637X, R467X, IVS4-2A > G, S244L and V505G. The mutation L594fsX595, IVS4-2A > G and V505G was never reported before.

CONCLUSIONLow molecular weight proteinuria and hypercalciuria were the main clinical features of the six Chinese boys with Dent's disease. Dent's disease could be associated with a Bartter-like syndrome, which make the gene diagnosis more important.

Adolescent ; Child ; Child, Preschool ; Chloride Channels ; genetics ; Dent Disease ; complications ; diagnosis ; genetics ; Humans ; Hypercalciuria ; diagnosis ; genetics ; Male ; Mutation ; Pedigree ; Proteinuria ; diagnosis ; etiology ; genetics

Glomerulonephritis occurs as a rare form of renal manifestation in Plasmodium falciparum malaria. Herein, we report a case of falciparum malaria-associated IgA nephropathy for the first time. A 49-yr old male who had been to East Africa was diagnosed with Plasmodium falciparum malaria. Microhematuria and proteinuria along with acute kidney injury developed during the course of the disease. Kidney biopsy showed mesangial proliferation and IgA deposits with tubulointerstitial inflammation. Laboratory tests after recovery from malaria showed disappearance of urinary abnormalities and normalization of kidney function. Our findings suggest that malaria infection might be associated with IgA nephropathy.
Acute Kidney Injury/etiology/pathology ; Antimalarials/therapeutic use ; Creatinine/blood ; Glomerulonephritis, IGA/*diagnosis/*etiology ; Hematuria/etiology ; Humans ; Immunoglobulin A/*metabolism ; Malaria/*complications/drug therapy/*pathology ; Male ; Middle Aged ; Plasmodium falciparum/*isolation & purification ; Proteinuria/etiology ; Quinine/therapeutic use

OBJECTIVETo analyze the characteristics of proteinuria and glomerular lesions in Henoch-Schönlein purpura nephritis (HSPN) of adults.

METHODSThe pathologic results and clinical data of 103 HSPN patients were analyzed.

RESULTSPatients with mild or moderate proteinuria accounted for 40.8% and 37.9%, respectively. Patients with nephropathic proteinuria accounted for about 21.4%. Severer proteinuria resulted in higher blood pressure and increased cholesterol, and albumin decreased gradually (P < 0.01); meanwhile, total glomerular lesion, tubulointerstitial lesion, and vascular lesion also became severer (P < 0.01).

CONCLUSIONProteinuria may indirectly reflect the severity of glomerular lesions in HSPN of adults.

Adolescent ; Adult ; Aged ; Female ; Glomerulonephritis, IGA ; diagnosis ; etiology ; Humans ; Immunoglobulin A ; blood ; Immunoglobulin G ; blood ; Immunoglobulin M ; blood ; Kidney Glomerulus ; pathology ; Kidney Tubules ; pathology ; Male ; Middle Aged ; Nephritis ; diagnosis ; etiology ; Proteinuria ; etiology ; urine ; Purpura, Schoenlein-Henoch ; complications ; pathology ; Retrospective Studies

Fanconi Syndrome ; complications ; diagnosis ; therapy ; Female ; Humans ; Infant, Newborn ; Potassium ; therapeutic use ; Prognosis ; Proteinuria ; etiology ; Treatment Outcome ; Vitamin D ; therapeutic use

OBJECTIVEIdiopathic collapsing glomerulopathy (ICG) is a clinically and pathologically distinct variant of focal segmental glomerulosclerosis, which is characterized by proteinuria (often nephrotic range) and rapid progression to end-stage renal failure. The typical pathological changes are global/segmental glomerular collapse, hypertrophy and hyperplasia of podocytes and severe tubulointerstitial lesions. Most ICG patients who have been reported in previous published papers are adults. ICG in children is rare. The study aimed to analyze and investigate clinical manifestations, renal histopathological findings, treatment and outcomes of ICG in children.

METHODSData of two cases of ICG, a 7-year-old boy and a 12-year-old girl, were analyzed. Both of them were Chinese and Han. Clinical characteristics, results of laboratory tests, renal histopathological findings, treatment, outcomes and prognosis of the two children with ICG were retrospectively analyzed. Results were compared with published data.

RESULTSThese two children presented typical clinical features of nephrotic syndrome. The quantity of 24 hr urine protein was 7.6 g/d (0.47 g/kg x d for boy) and 10.67 g/d (0.35 g/kg x d for girl). Both of them had hypertension (blood pressure ranged from 130/90 to 150/110 mmHg) and hypercholesterolemia (15.4 mmol/L for the boy and 11.3 mmol/L for the girl). The serum albumin was 12 g/L for girl and 23 g/L for boy. The creatinine clearance rate gradually decreased from normal range to 30 ml/min for the girl. The histopathological changes in renal biopsy of them were focal segmental or global glomerular collapse, hypertrophy and hyperplasia of podocytes and severe tubulointerstitial lesions. These two cases were steroid-resistant and were treated with pulse intravenous methylprednisolone and pulse intravenous cyclphosphamade in one case, who rapidly progressed to end-stage renal failure and died half a year later. Another one was treated with cyclosporine. He showed continuous hypertention and heavy proteinuria for eight months.

CONCLUSIONICG in the 2 children was a severe disease which presented steroid-resistant nephrotic syndrome and rapidly progressive renal failure. The pathological characteristics was global/segmental glomerular collapse, hypertrophy and hyperplasia of podocytes and severe tubulointerstitial lesions. In children with ICG treatment was difficult and the prognosis was poor.

Child ; Disease Progression ; Female ; Glomerulosclerosis, Focal Segmental ; complications ; diagnosis ; pathology ; therapy ; Glucosinolates ; Humans ; Kidney ; pathology ; Kidney Failure, Chronic ; etiology ; Kidney Glomerulus ; pathology ; Male ; Nephrotic Syndrome ; etiology ; Proteinuria ; etiology ; Treatment Outcome

Animals ; Child ; Child, Preschool ; Chloride Channels ; genetics ; Dent Disease ; complications ; diagnosis ; genetics ; therapy ; Diuretics ; therapeutic use ; Humans ; Hydrochlorothiazide ; therapeutic use ; Hypercalciuria ; diagnosis ; genetics ; Mutation ; genetics ; Phosphoric Monoester Hydrolases ; genetics ; Proteinuria ; diagnosis ; etiology ; genetics

OBJECTIVEAnti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a disorder with poor prognosis. This study aimed to improve the diagnosis and treatment of ANCA associated vasculitis of children, to analyze the clinical features, pathological characteristics and the prognosis of children with ANCA-associated vasculitis.

METHODFifteen children with ANCA associated vasculitis who were hospitalized from 2003 to 2012 in our hospital were included. Their data of pre-diagnosis status, clinical manifestations, renal pathology, treatment and prognosis were reviewed retrospectively.

RESULTOf the 15 children, 11 were girls and 4 boys with a mean age of 10.7 years. Fourteen children were categorized as microscopic polyangitis. The time to diagnosis varied from 0.5 month to 40 months. Hematuria and proteinuria were revealed by urine analysis in all of them, only 6 children complained with gross hematuria or edema of oliguria. Decreased glomerular filtration rate was revealed in 13 children, 8 of whom had a creatinine clearance rate of less than 15 ml/(min·1.73 m(2)). Twelve children underwent renal biopsy, crescent formation was found in 11 children. Most of the crescents were cellular fibrous crescents or fibrous crescents. Six children were diagnosed as crescentic nephritis; the process of rapidly progressive nephritis was only observed in 2 children. Segmental glomerulosclerosis or global glomerulosclerosis were found in 10 children, 3 of them were diagnosed as sclerotic glomerulonephritis. Anemia and pulmonary injury were the most common extra renal manifestations. Other extra renal manifestations included rash, pain joint, gastrointestinal symptoms, abnormal findings of cardiac ultrasonography and headache. Eight children were treated with steroid combined with cyclophosphamide, 4 were treated with steroid and mycophenolate mofetil, 2 were treated with steroid, cyclophosphamide and mycophenolate mofetil, 3 children were treated with plasma exchange. Fourteen children were followed up for 0.5 month to 4 years. The renal function did not recover in children with creatinine clearance rate of less than 30 ml/(min·1.73 m(2)), who showed crescentic glomerulonephritis or sclerotic glomerulonephritis. The children who had creatinine clearance rate of more than 30 ml/(min·1.73 m(2))had better prognosis.

CONCLUSIONMore attention should be paid to ANCA-associated vasculitis among school age girls with anemia or pulmonary diseases. The renal damage was serious in children; however, the clinical manifestations were not obvious. Children with a creatinine clearance rate of less than 30 ml/(min·1.73 m(2)) had poor prognosis. Early accurate diagnosis is very important.

Adolescent ; Anemia ; etiology ; pathology ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; complications ; diagnosis ; pathology ; Antibodies, Antineutrophil Cytoplasmic ; blood ; immunology ; Biopsy ; Child ; Child, Preschool ; Creatinine ; blood ; Female ; Glomerulonephritis ; pathology ; Hematuria ; etiology ; pathology ; Humans ; Kidney ; pathology ; physiopathology ; Kidney Function Tests ; Male ; Nephritis ; diagnosis ; etiology ; pathology ; Prognosis ; Proteinuria ; etiology ; pathology ; Retrospective Studies

Various forms of hypogammaglobulinemia can occur in patients with autoimmune diseases and vice versa. We report a 13-yr-old boy with membranous nephropathy and common variable immunodeficiency. He presented with the nephrotic syndrome, pneumonia with bronchiectasis, and profound hypogammaglobulinemia. Renal biopsy showed diffusely thickened glomerular capillary walls with 'spikes' suggesting a membranous nephropathy. Secondary causes were ruled out by laboratory studies; however, heavy proteinuria persisted with steroid therapy. Cyclosporine and intravenous immunoglobulin were added, and the patient was discharged with decreased proteinuria. Hypogammaglobulinemia may have a deleterious impact on the immune dysregulation in some patients with membranous nephropathy.
Adolescent ; Bronchiectasis/etiology ; Common Variable Immunodeficiency/complications/*diagnosis/drug therapy ; Cyclosporine/therapeutic use ; Drug Therapy, Combination ; Glomerulonephritis, Membranous/complications/*diagnosis/drug therapy ; Humans ; Immunoglobulins/therapeutic use ; Immunosuppressive Agents/therapeutic use ; Injections, Intravenous ; Kidney/pathology ; Male ; Pneumonia/etiology ; Proteinuria/etiology ; Steroids/therapeutic use