Objective To explore the clinical features of invasive fungal infection in 27 children treated at nephrology department between 1999 and 2014.Methods Twenty-seven cases of invasive fungal infection at nephrology department were analyzed retrospectively.Results Candida urinary infection was found in 9 cases,pneumocystis carinii pneumonia were found in 8 cases,invasive pulmonary aspergillosis and urinary aspergillosis found in 4 cases and 1 case respectively,cryptococcus neoformans meningitis and pulmonary candidiasis found respectively in 2 cases,and pulmonary filamentous fungal disease was found in 1 case.These 27 cases showed different features of illness:10 primary nephrotic syndrome,7 secondary nephrotic syndrome,6 malformation of urinary development and 4 preterm birth ＜ 32 weeks gestational age with low birth weight.All of the cases had the history of taking antibiotics.Seventeen cases of them had used corticosteroid and cytotoxic drugs in a long-term.Sixteen cases had experienced invasive procedures.All children had fever in varying degrees,14 cases showed gasp,7 cases had progressive hypoxia and respiratory failure,and 5 cases developed into multiple organs failure.Chest computed tomographic (CT) imaging data showed diffuse ground-glass opacity with mosaic sign of pneumocystis carinii pneumonia(8 cases).The features of pulmonary aspergillosis included multiple nodules and cavity in bilateral lungs (4 cases).The radiologic findings showed patching lesions with indistinct edge and uncertained density of bilateral middle-lower lung fields with pulmonary candidiasis (2 cases).Full dose of antifungal drug was given to 23 cases of them,16 cases recovered completely,3 got better,4 cases died.Four cases gave up full dose antifungal therapy.Conclusions Long-term use of corticosteroid and cytotoxic drugs,use of broad-spectrum antibiotics,invasive therapies,such as indwelling centralvenous catheters,endotracheal intubation with mechanical ventilation,and preterm birth with low birth weight were risk factors in predicting invasive fungal infection.Chest CT findings were different among these cases.Even though not specific,the relatively differences were helpful to the differential diagnosis of these diseases.

Objective To analyze the clinical characteristics,prognosis and the risk factors in 48 children with lupus nephritis combined with renal insufficiency.Methods The clinical data from 48 pediatric lupus nephritis with renal insufficiency from January 2000 to January 2013 were retrospectively analyzed.Results Forty-eight cases showed renal insufficiency among 168 children with lupus nephritis,and the incidence rate was 28.6％.The age ranged from 5.8 to 16.2 years.The male to female ratio was 1.0 ∶ 2.2.Among 48 children,43 cases had hematuria,41 cases had heavy proteinuria,25 cases had anaemia and 23 cases had persistent hypertension.Totally 20 cases underwent renal biopsy,and among them,15 cases(75.0％) were diagnosed as diffuse proliferative lupus nephritis(class Ⅳ).The histological activity index was ≥7 in 13 cases and the histological chronicity index was ≥4 in 3 cases.Corticosteroid and/or cytotoxic drugs were used in all of 48 cases.Thirty-five cases had normal urine and renal function,5 cases had stable renal function with persistent proteinuria,4 cases developed into chronic renal failure and 4 cases died.Persistent hypertension (x2 =4.274,P =0.039),the time of starting therapy (x2 =28.830,P =0.000),and histologic chronicity index(P =0.008 8 by Fisher's exact probability test)were the prognostic factors.Conclusions Among pediatric lupus nephritis with renal insufficiency,class Ⅳ (diffuse proliferative lupus nephritis) is the most frequent finding.Persistent hypertension,the time of starting therapy,and histologic chronicity index are the prognostic factors.The outcomes of lupus nephritis with insufficiency can be improved by adequate and appropriate treatment.

Objective To analyze the clinical features of pediatric sepsis with initial symptoms of autoimmune hemolytic anemia(AIHA) and acute renal failure.Methods Ten cases of pediatric sepsis with initial symptoms of AIHA and acute renal failure were selected from January 2000 to January 2014 in Department of Nephrology,Beijing Children's Hospital Affiliated to Capital University of Medical Science.The clinical data were retrospectively analyzed and long-time follow-up was taken.Results There were 8 boys and 2 girls of the 10 cases.The ages ranged from 8 months to 12.8 years.There were 8 children less than 6 years old having a prevalence of 80％.There were sudden dark urine and progressive anemia in all the cases,jaundice in 9 cases,abdominal pain and vomiting in 6 cases.Urine volume decreased in 7 cases and anuria in 3 cases.Four cases showed disorder of consciousness and 2 developed shock.Respiratory failure and disseminated intravascular coagulation happened in 1 case respectively.In terms of pathogens,2 cases were infected by mycoplasma and 1 case was infected by hemolytic streptococcus.The rest of the pathogen was unknown.The Coomb's test was 3 +-4 + positive.Serum urea nitrogen and creatinine gradually increased.High level total serum bilirubin and unconjugated bilirubin were shown in 8 cases and low level complement in 7 cases.Bone marrow films showed hyperplasia in all the cases.Renal biopsy was taken in 3 cases and no microthrombus was found.Corticosteroid was used in all 10 cases for 6 months.Blood purification was taken in 8 cases.Respiratory support was used in 4 cases.Nine recovered and 1 case gave up treatment.Further follow-up was taken for 2.2-8.1 years.Eight cases had normal urine and blood routine test and renal function.Relapse happened in 1 girl 1 year later.One case lost follow-up.Conclusions Once sudden hemoglobinuria,anemia and progressively decreased urine output occur in sepsis cases,the clinicians should consider AIHA and acute renal failure.Based on active infection control,the early use of steroids and blood purification treatment can improve the prognosis.

Objective To explore the clinical feature of spontaneous bacterial peritonitis (SBP) in children with nephrotic syndrome (NS).Methods Eleven cases of SBP in children with NS from Department of Nephrology,Beijing Children's Hospital Affiliated to Capital University of Medical Science between January 2010 and June 2014 were analyzed retrospectively.The clinical features,laboratory data and efficacy of therapy were reviewed.Results In this study,12 episodes of primary peritonitis were detected in 11 patients.One patient had 2 attacks.There were 11 cases including 9 boys and 2 girls.The age of the patients ranged from 2 years and 11months to 14 years and 11 months.Nine cases of them received steroid therapy and/or cytotoxic drugs.Peritonitis was characterized by abdominal pain,mild abdominal pain occurred in 7 cases with slow onset.Ten cases had fever of varying degrees,8 cases had abdominal distention,5 cases had diarrhea and 4 cases had nausea and vomiting.Shock signs were present suddenly in 2 cases before therapy of antibiotics.Apparent edema and severe ascites were present in all cases.Five of them were presented with increasingly rapid severe ascites and showed no response to diuretic drugs.Blood investigation showed leukocytosis and high C-reactive protein.Serum albumin levels and IgG and CD4 ratio were very low in all cases.Ascites were like cloudy pus with raised neutrophil cells.Streptococcus pneumonia was positive in both blood culture and ascites culture in 2 cases.Cephalosporin was used empirically to all cases for 7 to 10 days in 9 cases and 4 weeks in 2 cases with positive blood culture.Paracentesis to release ascites fluid was performed in 5 cases with constant ascites.Eleven cases recovered from peritonitis completely.The proteinuria was negative in 2 cases after they recovered from peritonitis.Conclusions SBP was atypical in children with NS and sometimes patients might have to abdominal pain and rebound tenderness,which may be ignored and may induce deterioration suddenly and clinicians should be aware of it clearly.SBP could lead to relapse of NS and the cure of SBP by treatment could help the children recover from NS.

Mian-Zao-Er was collected from the bulbs of Scilla scilloides (Lindl. ) Druce, belonging to the Hyacinthaceae family. 17 compounds were obtained using various column chromatographies on macroporus resin (HPD100), silica gel, Sephadex LH-20 and ODS, as well as semi-preparative HPLC. Their structures were elucidated on the basis of physicochemical properties and spectral data as 2-hydroxy-7-methoxyscillascillin (1), scillascillin (2), 5,7-dihydroxy-3',4'-dimethoxyspiro 2H-1-benzopyran-7'-bicyclo[4.2.0 ] octa [1,3,5 ] -trien } -4-one (3), socialinone (4), 4-methylresveratrol (5), (E)-resveratrol (6), scillavoneA (7), 3,9-di- hydroeucomnalin (8), 3-(3-hydroxy-4-methoxybenzyl) -5,7-dihydroxychroman-4-one (9), (3R)-5,7,3'-trihydroxy-4'-methoxyspiro (2H-1-benzopyran-7'-bicyclo[4, 2, 0] octa [1, 3, 5]-trien} -4-one (10), scillabene A (11), 2-hydroxyscillascillin (12), 3-(4-hydroxybenzyl) -5,7-dihydroxychroman-4-one (13), 3-( 4-hydroxybenzylidene) -5, 7-dihydroxychroman-4-one (14), 3-( 4-hydroxybenzyl) -5-hydroxy-7,8-dimethoxychroman-4-one (15), 3-(4-hydroxybenzyl) -5-hydroxy-6, 7-dimethoxychroman-4-one (16), and 3-(4-hydroxybenzyl)-5,8-hydroxy-7-methoxychroman-4-one (17). Among them, compounds 3, 4, 6, 9, 13 and 15-17 were isolated from this plant for the first time.
Drugs, Chinese Herbal ; chemistry ; Flowers ; chemistry ; Isoflavones ; chemistry ; Mass Spectrometry ; Molecular Structure ; Scilla ; chemistry ; Stilbenes ; chemistry

Objective To understand the clinical characteristics, treatment effect and prognosis of children with methylmalonic acidemia (MMA) combined with renal damage, and to provide experiences about how to improve the level of diagnosis and treatment.Methods The medical records of children with MMA were collected from January of 2007 to December of 2013 in Beijing Children's Hospital Affiliated to Capital University of Medical Sciences,while the clinical manifestations,laboratory findings, imaging material, inspection results were analyzed, and the renal pathological gene analysis, treatment effect, and prognosis of MMA children with renal damage were studied, and follow-up was carried out for 3 months up to 7 years.Results Among the two hundred and ninety-six cases of MMA,28 cases (9.5％) with renal damage,including 19 boys and 9 girls, 18 patients with hyperhomocysteinemia.Their ages varied from 2 days to 13 years old when confirmed.The first symptoms of 16 cases were proteinuria, hematuria, and edema, 9 cases were complicated with acute renal failure,5 cases were diagnosed as hemolytic uremic syndrome,6 patients were diagnosed as nephrotic syndrome, receiving enough glucocorticoids treatment without improvement.The routine examination of the urine showed that proteinuria (241-3 060 mg/d), methylmalonic acid levels and urinary protein were positively correlated (r =0.982, P =0.003), as urinary β2-microglobulin [(360.386 ± 231.496) μg/L], retinol binding protein [(338.537 ± 243.293) μg/L] increased.Extrarenal clinical manifestations were the spiritual, mental and physical development retardations, seizures, anemia etc.Two cases had a positive family history.Renal pathology showed mesangial cells and mesangial matrix proliferation broadening, no electron dense deposits in mesangial area, renal tubular epithelial cell swelling degeneration, and immunofluorescence was negative.Gene analysis of 3 cases showed exon1:c.80A ＞ G,p.Q27R and exon1 :c.365A ＞ T,p.H122L/c.609 G ＞ A,p.W203X.Children with MMA were treated with vitamin B12 treatment.Two cases of multiple organ failure died during hospitalization, and 2 cases died after leaving hospital.After treatment, the level of urinary methylmalonic acid was significantly decreased, while nervous system symptoms improved significantly, and the mental state and the renal function were improved.Conclusions MMA may be associated with kidney damage,usually with severe renal tubular and glomerular impairment.Early diagnosis is the key,and timely treatment can effectively control the disease, improve the prognosis.

Objective To investigate the treatment of atypical hemolytic uremic syndrome (aHUS) in their acute phase and relate the prognostic factors.Methods Twenty-eight patients with aHUS treated in Department of Nephrology,Beijing Children's Hospital Affiliated to Capital Medical University from January 2011 to March 2015 were collected,the outcomes of the treatment were summarized.Results All the patients were given plasma exchange (PE)treatment,21 patients with hemodialysis (HD) treatment;13 patients with H factor (FH) antibody were further treated with corticosteroid therapy,and 1 patient was treated with the combination of mycophenolate mofetil.Evaluation of patients' indices were conducted at month 3.Out of the 28 patients,there were 13 patients with complete remission;of the 14 patients showed improvement,6 patients were complicated with hypertension and 4 patients with renal dysfunction.One patient,with ongoing hemodialysis,did not show restoration of renal function.There was no death in children.Statistical analysis showed that the acute phase serum creatinine levels were (430.0 ± 124.7) μmol/L and (265.6 ± 90.8)μmol/L for the group with remaining kidney injury and the group with complete remission,respectively,the difference was statistically significant(P ＜0.01).The levels of FH for the 2 groups were (206.5 ± 134.9) mg/L and(415.4-±214.1) mg/L,and the difference was statistically significant (P ＜ 0.01) as well.Statistical analysis showed that differences were no statistically significant between the 2 groups of patients on their age,platelets,complement C3,complement C4,hemoglobin and lactate dehydrogenase (all P ＞ 0.05).For the group with positive antibody,its average serum creatinine was (346.4 ± 112.5) μmol/L.For the group with negative antibody,its average serum creatinine was (297.0 ± 89.3) μmol/L.The FH for group with positive antibody and group with negative antibody were (307.4 ±187.3) mg/L and (379.8 ± 203.5) mg/L,respectively.The differences were not statistically significant (all P ＞0.05).Conclusions PE is an effective treatment for aHUS at acute phase,and corticosteroid and immunosuppressive therapy are effective for patients with positive anti-FH antibody.For patients with severe kidney injury at acute phase,the possibility of long-term kidney damage is high.Low concentration of serum FH indicates a poor prognosis.

The genus Scilla consists of 90 species widely distributed in Europe, Asia and Africa, one and its variant of which can be found in China Some species of the genus have been used in traditional medicine to treat various diseases related to inflammation and pain. Phytochemical studies have demonstrated the presence of triterpene and tritepenoid saponins derived from eucosterol, bufadienolides, alkaloids, stilbenoids and lignan in the plants of this genus. Various bioactivities such as antimicrobial, anti-inflammatory, antioxidant, anti-tumor and glycosidase inhibitory activities, have been reported. In this review, the advance of chemical constituents and pharmacological activities of the Scilla species are summarized for further development and utilization of the resource.
Animals ; Drugs, Chinese Herbal ; chemistry ; pharmacology ; Humans ; Scilla ; chemistry

Objective:To understand clinical characteristics, treatment effects and prognosis of children with methylmalonic acidemia (MMA) presented with hemolytic uremic syndrome(HUS).Methods:The medical records of children with MMA were collected in Beijing Children′s Hospital, Capital Medical University from January 2012 to January 2019, the clinical manifestations, laboratory, imaging material, inspection results, renal pathological, gene analysis, treatment effect, and prognosis of MMA children with renal damage were analyzed, and were followed-up for 1-7 years.Results:Thirty cases were diagnosed as MMA with secondary renal damage.Eight cases(26.67%) showed as MMA-HUS.Age was from 1 month and 14 days to 12 years and 10 months old.There were 4 males and 4 females.The concentration of urine methylmalonic acid increased by 10-62 times.All were combined with hyperhomocysteine(HCY). The level of serum methylmalonic acid(1.5-11.8 mg/L), propylene carnitine(6.33-9.77 μmol/L)and the ratio of propylene /ethylene carnitine (0.24-0.29)were increased.Manifested as the mental and physical development retardation, anemia, jaundice, renal dysfunction, platelet reduction, hematuria, proteinuria in 8 cases, hypertension in 6 cases, frequent vomiting and convulsions in 2 cases.Two cases had a positive family history.Renal pathology showed that mesangial cells and mesangial matrix proliferation broadening, electron dense deposits no mesangial area, renal tubular epithelial cell swelling degeneration, immunofluorescence was negative.Two cases were genetically analyzed. One case was a CblC type MMACHC compound heterozygous mutation[c.80A>G(p.Q27R); c.217C>T(p.R73X)] and CblX type HCFC1 heterozygous mutation [c.3757G>A(p.R1253C)] double mutation; 1 case was a CblC type MMACHC compound heterozygous mutation[c.365A>T(p.H122L); c.609 G>A(p.W203X)]. Children diagnosed were treated with vitamin B 12, etc.Four cases of children gave up.The others, after treatment, were improved. Conclusions:MMA-HUS might be associated with multiple organ failure.Early diagnosis was the key, timely treatment could effectively control the disease, improve the prognosis.It should be followed up for ever.

Objective To study the patients' clinical characteristics and prognosis when only C3 deposition exists in endocapillary proliferative glomerulonephritis and try to understand deeply the role of C3 in kidney damage deeply. Methods The patients who were diagnosed with endocapillary proliferative glomerulonephritis but only had C3 deposited in immunofluorescence(to avoid false positive,C3≥2 ﹢ was included)were selected from Beijing Children's Hospital Affiliated to Capital Medical University during November 2010 to October 2014. Their clinical manifestations,la-boratory examinations,treatments,prognosis,and pathological changes were analyzed,and literature review was performed. Their clinical characteristics and prognosis were summarized. Results There were 11 patients diagnosed with endocapil-lary proliferative glomerulonephritis which had only C3 deposition(≥2 ﹢ ). Nine of them had onset with acute nephritis syndrome(81. 8% ),and 2 cases presented recurrent paroxysmal gross hematuria(18. 2% ). Seven cases were diagnosed with acute post streptococcal glomerulonephritis(63. 6% ). Eleven cases' clinical manifestations were relatively severe, and the complement C3 was significantly lower than the normal(100. 0% ). Their light microscope showed capillary proli-ferative glomerulonephritis,and the electron microscope showed the immune complexes were deposited in the endothelium,the epithelium or the mesangial area. The patients received corresponding treatment respectively,and all the patients had good prognosis during following up of 7 months up to 39 months. Conclusions Streptococcus infection is a common cause in endocapillary proliferative glomerulonephritis with only C3 deposition. The clinical manifestations of some children are similar to post streptococcal glomerulonephritis but relatively severe. Only deposition of C3 without IgG may be involved in another complement activation mechanism.