Cervical spinal canal narrowing and cervical neurological injuries / 中华创伤杂志(英文版)

Cervical spinal canal narrowing can lead to injury of the spinal cord and neurological symptoms including neck pain, headache, weakness and parasthesisas. According to previous and recent clinical researches, we investigated the geometric parameters of normal cervical spinal canal including the sagittal and transverse diameters as well as Torg ratio. The mean sagittal diameter of cervical spinal canal at C(1) to C(7) ranges from 15.33 mm to 20.46 mm, the mean transverse diameter at the same levels ranges from 24.45 mm to 27.00 mm and the mean value of Torg ratio is 0.96. With respect to narrow cervical spinal canal, the following charaterstics are found: firstly, extension of the cervical spine results in statistically significant stenosis as compared with the flexed or neutral positions; secondly, females sustain cervical spinal canal narrowing more easily than males; finally, the consistent narrowest cervical canal level is at C(4) for all ethnicity, but there is a slight variation in the sagittal diameter of cervical spinal stenosis (less than or equal to 14 mm in Whites, less than or equal to 12 mm in Japanese, less than or equal to 13.7 mm in Chinese). Narrow sagittal cervical canal diameter brings about an increased risk of neurological injuries in traumatic, degenerative and inflammatory conditions and is related with extension of cervical spine, gender, as well as ethnicity. It is hoped that this review will be helpful in diagnosing spinal cord and neurological injuries with the geometric parameters of cervical spine in the future.

Influence of degenerative changes of intervertebral disc on its material properties and pathology / 中华创伤杂志(英文版)

<p><b>OBJECTIVE</b>To investigate the material properties of normal and degenerated intervertebral discs (IVDs) and examine the effect of degenerative changes on IVD pathology.</p><p><b>METHODS</b>A computer-based online search was undertaken to identify English articles about material properties of IVDs published from January 1950 to 2011 in PubMed database. The retrieved keywords included material properties, intervertebral disc and degeneration. Based on the principles of reliability, advancement and efficiency, the obtained data were primarily examined, and the original source was retrieved to read the full-text. Repetitive articles were excluded. The data of material properties of normal and degenerated IVDs were summarized and analyzed by meta-analysis.</p><p><b>RESULTS</b>The data of Young's modulus, Poisson's ratio, shear modulus, hydraulic permeability and intradiscal pressure of normal and degenerated IVDs were obtained. Compared with normal IVDs, the Young's modulus and shear modulus of annulus fibrosus and nucleus pulposus were higher in degenerated IVDs, the Poisson's ratio was lower while the hydraulic permeability and intradiscal pressure were higher. Besides, the degeneration-related alterations in IVDs had an influence both on itself and other spinal structures, leading to diseases such as bulging disc, discogenic pain and spinal stenosis. Meanwhile, the heavy mechanical loading and injury indicated important pathways to IVD degeneration.</p><p><b>CONCLUSIONS</b>To a certain extent, the degenerative changes of IVD influence its material properties. And the degeneration-related alterations of composition can cause structural failure of IVDs, leading to injuries and diseases.</p>

Characteristics of repeated renal biopsy-proven primary focal segmental glomerulosclerosis in children / 中华儿科杂志

<p><b>OBJECTIVE</b>To analyze the characteristics of repeated renal biopsy-proven primary focal segmental glomerulosclerosis (PFSGS) in 8 children, and to reveal the relationship between clinical features and pathology, between the two times of renal biopsy pathology, and the indications for repeated renal biopsy.</p><p><b>METHOD</b>The records of cases who ever experienced renal biopsy in this hospital were reviewed, of whom 8 cases of repeated renal biopsy-proven PFSGS were enrolled. The clinical manifestations, the reason why they had renal biopsy again, the difference in renal pathological findings, between the two biopsies and their therapeutic response. The classification of focal segmental glomerulosclerosis (FSGS) was based on the new criteria suggested by D'Agati in 2004.</p><p><b>RESULT</b>Of the 8 cases, age of onset ranged from 1 to 12 years, all were diagnosed as nephrotic syndrome (NS), the age of first biopsy ranged from 1.1 to 15.0 years, and the follow-up period was 10 months to 14 years. The reason for repeated biopsy was poor therapeutic response, continuous heavy proteinuria, or the progressive renal dysfunction. Four cases had the both biopsies in this hospital, and the first renal pathology showed minimal change disease (MCD), mesangial proliferation, FSGS CELL type and FSGS GTL type. After the second biopsy, they were additionally treated with immunosuppressive agents or switched to another one, 2 cases with FSGS COLL type presented renal dysfunction or end stage renal disease (ESRD), 1 case who developed the disease at 1.4 years of age, presented renal dysfunction at 10 months follow-up. The remaining 5 cases acquired complete remission.</p><p><b>CONCLUSION</b>FSGS is a clinicopathological syndrome, NS predominates clinically. It often indicates pathologic transformation when the patients show poor therapeutic response or continuous heavy proteinuria without remission. Mesangial proliferation can convert into FSGS, and the subtype of FSGS can shift. FSGS COLL type and onset at young age may suggest poor prognosis.</p>

Clinicopathological study of 212 children with primary focal segmental glomerular sclerosis / 中华儿科杂志

<p><b>OBJECTIVE</b>To evaluate the correlation between clinico-pathological features and outcome of children with primary focal segmental glomerular sclerosis (FSGS).</p><p><b>METHOD</b>A total of 212 pediatric patients with D'Agati (2004) primary FSGS were included in this study between 1997 and 2008. According to FSGS histologic classification criteria, 5 pathologic variants were recognized: collapsing (COLL), cellular (CELL), glomerular tip lesion (GTL), perihilar, and not otherwise specified (NOS). Retrospective analysis of the therapeutic response, the relationship between the clinical efficacy and pathology and the outcome of the patients was made.</p><p><b>RESULTS</b>Of the 212 patients, 178 (83.9%) had nephritic syndrome (NS), 97 (45.8%) had simple NS, 81 (38.2%) had nephritis-type NS, GTL variants were mostly appeared to be nephritic syndrome (n = 28) and COLL variants were the fewest (n = 11). The difference between the two variants had statistical significance (P < 0.05). Fourteen cases (6.6%) had nephrotic proteinuria, 20 cases (9.4%) had proteinuria with micro-hematuria. According to histologic classification, NOS (n = 86, 40.6%) was the most common type; perihilar type was seen in 25 cases (11.8%); CELL was seen in 58 cases (27.4%), COLL in 12 cases (5.6%), GTL in 31 cases (14.6%). Chronic tubular injury was present in most cases. CEL variants were mostly found in the early infancy. GTL and NOS variants initially appeared to be responsive to steroids, but subsequently became resistant or frequently recurrent; CELL and COLL appeared to be primarily steroid resistant, GTL and COLL variants had statistically significant differences (P < 0.05). The patients were followed-up for 5 months to 10 years. A response to therapy was observed in 50%, COLL FSGS had the highest rate of ESRD; 2 years renal survival rates were 67%, 3 years were 41%.</p><p><b>CONCLUSIONS</b>FSGS is defined as a clinicopathologic syndrome manifesting proteinuria and focal and segmental glomerular sclerosis with foot process effacement. The location of the sclerosis within the glomeruli proved to have prognostic significance. Collapsing glomerulopathy is the most aggressive variant of FSGS. Compared with other variants, GTL variant may be the best type. Different histologic variants of FSGS have substantial differences in clinical features at the time of biopsy diagnosis and substantial differences in renal outcomes. Prolonged treatment of FSGS-NS with corticosteroids and immune suppressive agents may have some effects in achieving sustained remission and improve prognosis in children.</p>

Clinical and pathological characteristics of children with dense deposit disease / 中华儿科杂志

<p><b>OBJECTIVE</b>To analysis the clinical and pathological characteristics of children with dense deposit disease (DDD).</p><p><b>METHODS</b>12 Children diagnosed as DDD by electron microscope were enrolled in this study. The clinical and pathological data were analyzed.</p><p><b>RESULTS</b>Of the 12 cases, 7 were males and 5 females, mean age 9.1 +/- 3.9 (5-13) years at onset, the duration from onset to renal biopsy was 1 month to 5 years and the follow-up period was 1-9 years. All cases had heavy proteinuria >50 mg/(kg x d), and persistent microscopic hematuria with recurrent gross hematuria during the course. Seven cases had hypertension (> or = 140/100 mm Hg, 1 mm Hg =0. 133 kPa), 5 cases had transient or recurrent abnormal renal function, and mild to severe anemia were observed in 8 cases respectively. All the cases had lower serum C3 (0.15-0.55 g/L). Clinically, 10 cases were diagnosed as nephritic syndrome (one case had partial lipodystrophy at the same time), and 2 cases were diagnosed as acute nephritic syndrome. Immunofluorescence study showed intense deposition of C3 along GBM, TBM and the wall of Bowman's capsule in a ribbon-like pattern and in the mesangial regions as coarse granules in all the cases. Under light microscopy, 9 cases showed the feature of membrane proliferative glomerulonephritis (MPGN), 1 case with focal segmental glomerulosclerosis (FSGS), 1 case with endocapillary proliferative glomerulonephritis (EnPGN) and 1 case with proliferative sclerosis (PSGN). Crescents were seen in 3 cases. Under electron microscopy, ribbon-like or linear electron-dense intramembranous deposits were identified in the lamina dense of GBM, and often along TBM and the wall of Bowman's capsule. All patients showed steroid resistance. After methylprednisone treatment, some patients showed transient remission. During the follow- up stage of 1-9 years, 3 cases showed normal urinalysis, 5 cases showed partial remission, 2 cases progressed to end stage renal disease (ESRD) and 2 cases were lost.</p><p><b>CONCLUSION</b>DDD is an in dependently rare disease with pathological-clinical varieties. Children with DDD presented with persistently lower C3, heavy proteinuria, recurrent gross hematuria and anemia. The characteristic immunopathologic finding is intense deposition of C3 along the GBM. Under electron microscopy, ribbon-like or linear electron-dense deposits in the lamina dense of the GBM, TBM and the wall of Bowman's capsule. Electron microscopic examination to demonstrate the intramembranous dense deposits is definitive diagnosis, regardless of the finding of light microscopy. All of them showed steroid resistant. Patients with steroid and CTX treatment showed some clinical improvement of their urinalysis.</p>

Peritoneal equilibration test and results analysis in children undergoing chronic peritoneal dialysis / 中华儿科杂志

<p><b>OBJECTIVE</b>To explore the characteristics of peritoneal transport in children undergoing chronic peritoneal dialysis (PD).</p><p><b>METHODS</b>Peritoneal equilibration test (PET) was carried out 10 times in 6 children (aged from 2 to 14 years) who were maintained by continuous ambulatory peritoneal dialysis (CAPD), and the peritoneal solution transport rate was evaluated by the standards of Twardowski's and Pediatric Peritoneal Dialysis Study Consortium (PPDSC)'s criteria.</p><p><b>RESULTS</b>In this study, the initial PET was performed at (38.7 +/- 15.6) days following initiation of PD, the 4-hours of peritoneal creatinine clearance (4 h-D/P) and glucose absorption (4 h-D/D(0)) was (0.85 +/- 0.24) and (0.34 +/- 0.19), respectively. According to the standards of Twardowski's and PPDSC criteria, the peritoneal transport categories were divided into high transport (H) (6/10), high average transport (HA) (1/10), low average (LA) (3/10) for peritoneal solution transport, and H (3/10), HA (4/10), LA (1/10), low transport (2/10) for glucose absorption. No low transport type of solution was used in the patients. The coincidence rate of peritoneal creatinine and glucose transport types were 100% and 90% between the Twardowski's and PPDSC criteria, respectively. The different changes of peritoneal transport type were found in two patients with continuous PET. The value of 4 h-D/P increased after peritonitis episodes.</p><p><b>CONCLUSION</b>The results showed that the PET in 70% of CAPD children fell into high and high average transport categories elevated by PPDSC's and adult standards, no-sinusoid distribution. The peritoneal solute clearance was adequate in the children, but net water ultrafiltration was lower. Standard pediatric PET and its criteria are consistent with the adult criteria. The capability of peritoneal solute transport increased after peritonitis episodes.</p>

NPHS1 mutations in a Chinese family with congenital nephrotic syndrome / 中华儿科杂志

<p><b>OBJECTIVE</b>Congenital nephrotic syndrome (CNS) is defined as heavy proteinuria or nephrotic syndrome occurring before 3 months of age. It is characterized by early onset, resistance to steroid therapy and progressing to end-stage renal disease (ESRD). In recent years, several genes associated with CNS have been identified, such as NPHS1, NPHS2 and WT1. The mutations of these genes have been identified in the patients with CNS in Finland, other European countries, North Africa, North America, and Asia, respectively. However, the investigation of the above genes has not been performed in Chinese CNS patients. In this study, NPHS1 mutations in a Chinese family with CNS were detected and analyzed.</p><p><b>METHODS</b>There were two CNS patients in the investigated family. The proband, a 45-day-old boy, was born at fullterm and weighed 2700 g at birth. The placenta weighed 450 g. At the age of 10 days, generalized edema, proteinuria, hypoproteinemia, and hypoalbuminemia were found without renal insufficiency. The proband's sister, with the same phenotype and normal renal function, underwent renal biopsy at 5 years of age. Their parents and elder half-sister all had normal phenotypes. Genomic DNA samples were extracted from peripheral bloods of the proband, his family members and 50 unrelated, normal individuals. All 29 exons and exon-intron boundaries of NPHS1 were detected in the proband by polymerase chain reaction (PCR), direct DNA sequencing, and restriction enzyme analysis.</p><p><b>RESULTS</b>Three heterozygous mutations of NPHS1, namely, G928A (D310N), 1893-1900del 8 (CGAAACCG), and G2869C (V957L) were identified in the proband. These mutations involved exons 8, 14, and 21. The same genotype was found in the proband's sister who had the same phenotype, but was not detected in proband's elder half-sister who had normal phenotype. Fifty normal individuals had no these mutations. The proband's mother with normal urinalysis had G928A (D310N) heterozygous mutation, and the father with normal urinalysis had two heterozygous mutations of 1893-1900del 8 (CGAAACCG) and G2869C (V957L). At the same time, three types of single nucleotide polymorphisms (SNPs), E117K (rs3814995), S1105S (rs2071327), and IVS27+45c > t, were confirmed in the proband. Another variant, IVS8+68 a > g had also been found.</p><p><b>CONCLUSION</b>This is the first report about NPHS1 mutations in Chinese CNS kindred. These three heterozygous mutations of NPHS1 are novel genetic defects of CNS, which have not been described before.</p>

Clinical and Pathological Analysis of 91 Children with Lupus Nephritis / 实用儿科临床杂志

Objective To analyze the clinical and pathological characteristics of children with lupus nephritis(LN).Methods Ninety-one children with LN were diagnosed from 1993 to 2005,according to the clinical literature and renal pathology and the data were retrospectively summarized.Results Within the cohort of 91 children,there were 69 females and 22 males(female to male ratio 3.1).Most of the sick children were at the school age ranging from 6.0 to 15.5 years old.Nephrotic syndrome(44.0%) was the most common clinical manifestation.Fifty-nine renal biopsies were performed.Class Ⅳ LN(59.3%) was the most frequent pathological findings."A full-house pattern" on immunofluorescence was found in 72.0% of biopsies.The clinical and pathological manifestations of some children were atypical.There were 3 patients characterized by predominant deposits of immunoglobulin M(IgM),1 patient with predominant deposits of IgA,and 2 children with pauci-immune LN.Three children with class Ⅱ LN in our study presented with nephrotic syndrome.LN was initially controlled by aggressive treatment in 93.7% of thse patients.Relapses of nephritis covered 27.1% of them,mostly caused by the intermittent treatment.Conclusions The clinical and pathological manifestations of LN were variable.Some atypical LN was considered to be associated with the distinct pathogenesis.Most of LN could be controlled by aggressive treatment.Long and regular treatment is necessary to improve the prognosis of LN.

Clinicopathological analysis of IgA nephropathy with crescentic formation in childhood / 中华儿科杂志

<p><b>OBJECTIVE</b>To understand the clinical and pathological characteristics of IgA nephropathy (IgAN) with crescentic formation in children.</p><p><b>METHODS</b>Clinicopathological 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.</p><p><b>RESULTS</b>(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.</p><p><b>CONCLUSION</b>The 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%.</p>

Clinical and pathological characteristics of focal segmental glomerulosclerosis in children / 中华儿科杂志

<p><b>OBJECTIVE</b>To investigate the clinical and pathological characteristics of focal segmental glomerulosclerosis (FSGS) in children.</p><p><b>METHODS</b>The data of 38 children,aged from one and half to 15 years, 25 boys and 13 girls, with primary FSGS were studied retrospectively.</p><p><b>RESULTS</b>Majority of the cases in this study were school-aged children. The average age of initial onset was 8.9 +/- 3.68 years. The ratio of boys to girls was 1.92. The clinical manifestation included isolated proteinuria in 3 cases, proteinuria and hematuria in 1 and nephrotic syndrome in 34 (simple type in 16 and nephritic type in 18). Of 38 cases, 24 (63%) presented with hematuria, 11 (29%) with hypertension and 7 (18%) with decreased creatinine clearance. The pathologic classification included perihilar variant in 17 cases, peripheral variant in 14 and tip variant in 7. The predominant clinical feature of children with tip variant was simple type of nephrotic syndrome (86%). Microscopic hematuria was not common (29%). Blood pressure and renal function were normal. The children with diffuse mesangial hypercellularity superimposed on changes of FSGS (in 21 of 38 cases) were more likely to have hematuria (76%) and less simple nephrotic syndrome (30%). The initial treatment response to prednisone in 34 cases with nephrotic syndrome showed sensitive in 12 cases, resistant in 21 and unknown in 1. Transition from sensitive to resistant occurred in six of 12 children. Three of 4 cases with non-nephrotic syndrome showed no response and the remaining one had unknown response. It was found that 44% of children who received cyclophosphamide and 83% of children who received pulse methylprednisolone and pulse cyclophosphamide or cyclosporin A in addition to oral steroids had complete or partial remission. Correlation analysis showed that the level of proteinuria after treatment was correlated directly with renal tubulointerstitial lesion and renal function (Pr = 0.48, P < 0.05; Pr = 0.45, P < 0.05).</p><p><b>CONCLUSION</b>FSGS was common in school-aged children. The predominant presenting feature was nephrotic syndrome. Hematuria was common. Hypertension and renal insufficiency were less frequently seen. The renal biopsy showed multiple variants. Pulse methylprednisolone and pulse cyclophosphamide or cyclosporin A treatments showed relatively good response.</p>

Antineutrophil cytoplasmic autoantibody positive vasculitis induced by propylthiouracil: a case report / 中华儿科杂志

<p><b>OBJECTIVE</b>Propylthiouracil (PTU) as a drug used during the treatment of hyperthyroidism could induce antineutrophil cytoplasmic autoantibody-positive vasculitis. Here the author reported a childhood case of antineutrophil cytoplasmic autoantibody-positive vasculitis induced by PTU, which is rarely described.</p><p><b>METHODS</b>The diagnosis was made according to the symptoms, signs, serum markers and renal biopsy, and the relevant literature was reviewed.</p><p><b>RESULTS</b>The 12-year-old girl presented with gross hematuria, proteinuria, renal function damage [Ccr 52.46 ml/(min. 1.73 m(2))], positive antineutrophil cytoplasmic autoantibody (ANCA-MPO) (MPO ELISA 140%) and a vasculitis lesion in the renal biopsy sample. She had been treated with PTU for 5 years because of Graves disease. After the diagnosis, the PTU was withdrawn, and prednisone (40 mg/d) and cyclophosphamide (25 mg, Bid) were applied. Three weeks after the therapy with prednisone and cyclophosphamide the gross hematuria disappeared. Three months after the treatment the renal function returned to normal [Ccr 124 mg/(min.1.73 m(2))], and the titer of ANCA-MPO decreased from 140% to 57%.</p><p><b>CONCLUSION</b>PTU may induce antineutrophil cytoplasmic autoantibody positive vasculitis. A right diagnosis and treatment can improve its prognosis of the disease.</p>