Henoch-Schönlein purpura with hypocomplementemia in children

BACKGROUND: The clinical course and prognosis of Henoch-Schonlein purpura (HSP) associated with hypocomplementemia are not clear. METHODS: The clinical findings of 10 children with HSP and hypocomplementemia were studied. RESULTS: Purpuric rash in all patients, abdominal pain in five, and arthralgia in nine were noted. The findings in HSP were not different from others with HSP. In eight patients, infection preceded hypocomplementemia. Serum levels of CH50, C3 or C4 were depressed variously. Complement levels returned to normal within 5 weeks in all patients. Antistreptolysin-O (ASO) titer was elevated in all patients and nephritis occurred in eight patients. Six patients had generalized edema and hypertension. Macroscopic hematuria occurred in two patients and heavy proteinuria in five patients. One patient was diagnosed as having poststreptococcal acute glomerulonephritis (PSAGN) combined with HSP nephritis according to renal biopsy findings. In three of eight patients with nephritis, abnormal urinary findings continued for more than 1 year. CONCLUSIONS: Hypocomplementemia in children with HSP was transient and was not related to severity of HSP. Incidences of elevated ASO titer and nephritis were high. The nephritis resembled PSAGN during the acute stage and long-term clinical courses varied. These findings suggest PSAGN may be associated with HSP nephritis.     

Pulse methylprednisolone therapy in children with membranoproliferative glomerulonephritis

The effects of pulse methylprednisolone (PM) therapy were studied in 15 patients (aged 3–14 years) with biopsy proven membranoproliferative glomerulonephritis (MPGN). Patients were treated with intravenous PM 30 mg/kg (max 1 g) given over 30 min every other day for a mean of 9.8 days (3–15 days). Oral prednisolone therapy was continued at a dose of 1 mg/kg/24 h for 1 month and subsequently tapered off the following month. Eight patients had hematuria and six had medically controlled hypertension. Serum C₃ levels were low in 11 patients and all of the patients had proteinuria. Following PM therapy proteinuria was significantly reduced from 2602.9 ± 1852.5 mg/24 h to 1871.2 ± 2090.8 mg/24 h (P < 0.05) and at final evaluation, proteinuria was 774.33 ± 1225.67 mg/24 h which was significantly lower than pre- and post-PM therapy values (P < 0.05). Serum creatinine levels were high in five patients before PM therapy and remained high in one of the patients who progressed to end-stage renal failure. After PM therapy, high serum creatinine levels normalized in three patients and was reduced, but still above normal, in one. One patient, with initially normal serum creatinine, had elevated levels afterwards. Nine of the patients were considered responsive and six non-responsive according to our tentatively defined criteria. Mean follow-up period was 27.4 ± 24.1 months (6–84 months). Three patients were lost for follow-up, and 12 were re-evaluated. At final evaluation, all of the patients except one with end-stage renal failure had normal creatinine levels. There was no correlation between the clinical and laboratory features at onset and the outcome of the disease in the responder or non-responder patients. Results of our study show that PM therapy reduced proteinuria and may affect renal function positively in patients with MPGN. However, a prospective controlled study with repeat biopsies is required to draw a conclusion.     

Characteristics of eighth-grade students who initiate self-care in elementary and junior high school

The purpose of this paper is to examine whether children who care for themselves for longer periods of time are at increased risk of poor grades, truancy, anger, family conflict, stress, risk-taking, and peer influences (in addition to the increased risk of substance use previously reported). Demographic characteristics of eighth-grade students who initiate self-care in junior high school are compared with those initiating self-care in elementary school. Further, increased risks for those initiating self-care in elementary school are examined. Over two thirds of the respondents (67.8%) cared for themselves after school without adult supervision at some time during the week; 23.5% for 1 to 4 hours per week, 15.7% for 5 to 10 hours per week, and 28.6% for 11 or more hours per week. Of those in self-care, 48.5% initiated self-care during elementary school and 51.5% during junior high school. Students who were in the highest category of self-care (greater than or equal to 11 hours per week) vs those in self-care zero hours per week were 1.5 to 2 times as likely to score high on risk-taking, anger, family conflict, and stress, to be more likely to see their friends as their major source of influence, and to attend more parties. The self-reports of academic grades did not differ. The grade of initiation of self-care (elementary vs junior high school) conferred additional risk for drinking alcohol (odds ratio = 1.4), risk-taking tendencies (odds ratio = 1.5), and attending parties (odds ratio = 1.6).     

Outcome of idiopathic membranoproliferative glomerulonephritis in children

The aim of this multicentre study was to analyse the long-term outcome of idiopathic membranoproliferative glomerulonephritis (MPGN) according to histological type and to the presence of C3 nephritic factor. Fifty patients aged 2 14 years at the onset of the study were followed over 2 20 years; 26 patients had MPGN type I, 17 had type II and 7 had type III. Treatment was variable. At the last observation, 30 patients had reached terminal and four pre-terminal renal failure. The median survival probability until renal death was 15.3, 8.7 and 15.9 years for disease types I, II and III respectively (difference between MPGN types I + III versus type II: p = 0.013). The presence of an initial nephrotic syndrome was associated with a more rapid progression ( p = 0.018). C3 nephritic factor was of no prognostic value. We conclude that the outcome of MPGN mainly depends on the histological type observed.     

Complement analysis in children with idiopathic membranoproliferative glomerulonephritis: A long-term follow-up

Fifty children with idiopathic membranoproliferative glomerulonephritis (MPGN), aged 2–14 years at apparent onset, were monitored for the presence of C3 nephritic factor (C3 NeF) and signs of complement activation in serum. In addition, C3 allotyping was performed in 32 patients. Observation time ranged from 2 to 20 (median 11) years. C3 NeF activity was detected at least once in 60% of the patients (in 11 of 26 with type I, in 15 of 17 with type II, and in four of seven with type III). C3 NeF-positive patients had significantly reduced levels of CH50 and C3 and elevated levels of C3dg/C3d. During follow-up, C3 levels were persistently normal in 62% of the patients with MPGN type I and in 43% with type III but in only 18% with type II. C3 allotype frequencies differed from those found in healthy controls with a significant shift to the C3F/C3FS variants in C3 NeF-positive patients. C3b(Bb)P as a marker for alternative pathway activation was not increased in C3 NeF-positive patients. Despite the presence of C3 NeF activity, C3 levels remained normal in six patients throughout the observation period. C3 NeF became undetectable in six patients, whereas seven developed C3 NeF activity during follow-up. There was no significant difference in renal survival probability in patients with or without C3 NeF activity. Neither C3 variants nor continous low C3 or low CH50 levels had any prognostic value for the clinical outcome. No factor H deficiency was detected.     

上海市中小学生尿液筛查的探索

目的探讨上海市中小学生血尿(隐血)和/或蛋白尿的检出率并评价筛查的方法和意义。方法2003～2005年,分别采用单一尿液试纸目测法(A)或尿液试纸机测法联合尿沉渣镜检/磺基水杨酸法(B)检测上海市46171名中小学生晨尿标本中的血尿(隐血)和蛋白尿。部分(27796例)一次尿检阳性者于10～14d后留尿复检。观察结果分三组(单纯血尿、单纯蛋白尿、血尿合并蛋白尿)随访。结果①46171名学生一次尿液标本阳性检出率>5.00%;27796名学生中二次尿液标本检测均阳性者占1.00%。不同筛查方法的阳性检出率不同。留尿一次检测,B方法较A方法阳性率低,特异度高;不论何种方法,留尿二次的阳性率均较留尿一次显著降低,特异度增高。②留尿一次,A、B方法的直接成本各约1.70元/人、2.90元/人;阳性者再次留尿检测的直接成本分别不超过1.90元/人、3.00元/人。A方法较B方法简便经济。A方法 留尿二次检测是既经济简便又有效提高特异度、降低假阳性率的筛查方法。③徐汇区初中生阳性检出率较小学生显著升高(P<0.05),12岁年龄组阳性检出率较其他各年龄组高;静安、杨浦区中小学生间差异无统计学意义。④10个月以上不完全随访确诊IgA肾病2例。结论中小学生尿液筛查是早期发现泌尿系统疾病的简便方法,阳性检出率需引起重视;选择最佳筛查年龄段优化成本效益值得进一步探讨;提倡多单位合作以建立尿液筛查全市网络。     

Mass screening for early detection of congenital kidney and urinary tract abnormalities in infancy

BACKGROUND: Recent widespread use of ultrasound has led to new efforts at screening for congenital kidney and urinary tract abnormalities. However, a standard screening methodology, criteria defining abnormalities, and follow-up procedures remain to be established. In order to establish screening criteria for these abnormalities, we performed a preliminary study in 800 1-month-old infants using provisional methods and criteria. METHODS: Based on the results of preliminary study, we screened 2700 1-month-old infants in a prospective study using the criteria of renal size (longitudinal diameter or=60 mm, or a difference between sides of >or=10 mm), and of pelvic dilatation (Society for Fetal Urology [SFU] grade 2 or higher) as positive at the first ultrasound screening. We used the SFU grading system instead of anteroposterior pelvic diameter measurements for pelvic dilatation. RESULTS: One hundred and twelve (4.1%) of the 2700 infants had abnormalities at the first ultrasound screening, while 18 (0.67%) had congenital kidney and urinary tract abnormalities on further examination. Use of the SFU grading system enabled us to reduce the false-positive rate at first screening, while maintaining a high diagnostic rate. The abnormalities consisted of ureteropelvic junction obstruction in seven infants, megaureter in two, hypoplastic kidney in four, vesicoureteral reflux in six (three were accompanied by hypoplastic kidneys or multicystic dysplastic kidney), multicystic dysplastic kidney in one, and horseshoe kidney in one. CONCLUSION: These results indicate that our screening methods and criteria are useful variables for detecting congenital kidney and urinary tract abnormalities.     

Analysis of renal biopsies performed in children with abnormal findings in urinary mass screening

Background: School urinary mass screening tests are performed to make early diagnosis and provide proper treatment for chronic renal diseases. However, very few systemic analyses or studies have been reported regarding final diagnosis made on children with abnormal urinary screening results. Aim: To study the cases of renal biopsy in children detected in urinary screening. Methods: We retrospectively analysed 461 cases of renal biopsy performed on children referred to us with abnormal school urinary mass screening results who satisfied indications for renal biopsy. Results: Pathologically abnormal findings were observed in 285 (61.8%) patients. Thin glomerular basement membrane disease was detected in 127 (27.5%) cases and IgA nephropathy in 121 (26.2%) cases. Among those 461 children, microscopic haematuria was observed in 289 (62.7%) patients, proteinuria in nine (2.0%), and both in 163 (35.4%). In addition, a statistically higher rate of pathological abnormalities on renal biopsy was noted in the group with microscopic haematuria combined with proteinuria and also in cases with more severe haematuria.

Conclusion: School urinary mass screening has greatly contributed to diagnosing chronic renal diseases. Continuous medical observation is required when abnormal urinalysis is observed, and a more aggressive medical approach such as renal biopsy should also be performed if necessary.     

Cardiac abnormalities in children with hyperthyroidism

Summary The cardiac status of 18 hyperthyroid (HT) children (9 black and 9 white) was evaluated by echocardiography. Mitral regurgitation (MR) was diagnosed clinically in 33% (6 of the 9 blacks). None of the 9 white children had MR. Left ventricular end-diastolic diameter (LVEDD) and volume (LVEDV) did not differ from the predicted normal (PN) based on body surface area and heart rate, except in those with MR where increased LVEDD and LVEDV were noted (p<0.02). LV mass was +1.75 standard deviations (σ) of the PN (p<0.01), due to increased wall thickness or LVEDV. Left ventricular output (LVO) was +0.35σ PN (p=ns); however, when compared to that of normal children, LVO of HT was higher (p<0.001) due to the increased heart rate. Enhanced left ventricular contractility was suggested by increased rate of dimensional change during ejection (peak dD/dt-syst), with a mean value of −11.39 cm/sec as compared to the normal of −9.54 cm/sec (p<0.01). A linear multivariate regression equation differentiated the cardiac status of HT from that of normal children. Following treatment to euthyroid state, MR disappeared in 2 and became less in 4 patients. LVO, LV mass, and peak dD/dt-syst also became less. Significant cardiac changes occur in children with hyperthyroidism, which may be reversible in part after euthyroidism is restored. Supported in part by a grant (RR 305) from the General Clinical Research Centers Program of the Division of Research Resources, National Institutes of Health.     

Two children with obesity‐related glomerulopathy identified in a school urinary screening program

The incidence of obesity‐related glomerulopathy (ORG) has increased over the last decade, but there have been few reports on ORG in Japanese children. Reported herein are two children with ORG identified on school urinary screening (SUS). Patient 1 was a 12‐year‐old boy in whom proteinuria was first detected on SUS. His body mass index (BMI) was 33.8 kg/m² and he had hypertension and hyperuricemia. Patient 2, a 10‐year‐old boy, also had proteinuria identified on SUS. His BMI was 34.8 kg/m², and he had fatty liver, hyperuricemia, and hypercholesterolemia. Both were diagnosed with ORG based on obesity, proteinuria, and renal pathological findings. After treatment, including candesartan, food restriction and physical exercise, urinary protein excretion was decreased in both cases. We believe that such school urinary screening programs may be effective for the early identification and treatment of children with ORG.     

Prevalence of the metabolic syndrome in elementary school children

AIM: To determine the prevalence of the metabolic syndrome (MS) and its related components in elementary-aged school children. METHODS: Three hundred and seventy-five 7-9-y-old boys (n=182) and girls (n=193) in the eastern Kansas area served as participants. Criteria for the MS were the presence of three or more of the following components: 1) central obesity (waist circumference>or=90th percentile in males and females); 2) elevated triglyceride concentrations (>or=1.13 mmol/l); 3) low HDL-C concentrations (or=90th percentile, age and gender specific); or 5) elevated fasting glucose levels (>or=6.10 mmol/l). RESULTS: The prevalence of the MS in this sample was 5%, and was similar across gender and race. Fifty percent of the children had no components and 15% had at least two components, with elevated blood pressure the most common component (37%). CONCLUSION: The MS is prevalent even in young children at rates similarly reported in adolescents.     

Social involvement of children with autism spectrum disorders in elementary school classrooms

Background: Children with autism spectrum disorders (ASD) are increasingly included in general education classrooms in an effort to improve their social involvement. Methods: Seventy‐nine children with ASD and 79 randomly selected, gender‐matched peers (88.6% male) in 75 early (K‐1), middle (2nd–3rd), and late (4th–5th) elementary classrooms across 30 schools completed social network surveys examining each child’s reciprocal friendships, peer rejection, acceptance, and social involvement. Results: Across grade levels, peers less frequently reciprocated friendships with children with ASD than students in the matched sample. While children with ASD were not more likely to be rejected by peers, they were less accepted and had fewer reciprocal friendships than matched peers at each grade level. Although 48.1% of children with ASD were involved in the social networks of their classrooms, children with ASD were more likely to be isolated or peripheral to social relationships within the classroom across all grade levels, and this difference is even more dramatic in later elementary grades. Conclusions: In inclusive classrooms, children with ASD are only involved in peers’ social relationships about half of the time, and appear to be even less connected with increasing grade level. Promoting children with ASD’s skills in popular activities to share with peers in early childhood may be a key preventive intervention to protect social relationships in late elementary school grades.     

Multiple associated endocrine abnormalities in a patient with pseudohypoparathyroidism type 1a

A girl with type 1a pseudohypoparathyroidism (PHP) presented several hormonal abnormalities. Although she had eluded neonatal thyroid screening, she was diagnosed as having hypothyroidism at the age of 5 months. Thereafter, a diagnosis of PHP was made on the basis of skeletal features of Albright osteodystrophy and lack of both cyclic adenosine monophosphate (c-AMP) and phosphaturic responses after parathyroid hormone (PTH) infusion. The basal levels of luteinizing hormone (LH) and follicle stimulating hormone (FSH) were higher than normal and showed exaggerated responses to luteinizing hormone-releasing hormone (LH-RH). There was no growth hormone (GH) response to arginine infusion, and the prolactin (PRL) response after thyrotropin-releasing hormone (TRH) infusion was also impaired. The stimulating guanine nucleotide-binding protein (Ns) activity of the erythrocytes was reduced to 66.9%. The skeletal age was not delayed at the age of 5 months in spite of the hypothyroid state, and it advanced following thyroxine and vitamin D treatments.Abbreviations PHP pseudohypoparathyroidism - c-AMP cyclic adenosine monophosphate - PTH parathyroid hormone - LH luteinizing hormone - FSH follicle-stimulating hormone - LH-RH luteinizing hormone-releasing hormone - GH growth hormone - PRL prolactin - TRH TSH-releasing hormone - Ns stimulating guanine nucleotide-binding protein - TSH thyroid stimulating hormone - Pi/cr phosphate/creatinine ratio     

Incidence of urological anomalies in children with urinary tract infections in India

A total of 2,970 infants and children with symptoms suggestive of urinary tract infection (UTI) were investigated. Diagnosis was based on clinical, microbiological, radiological, and sometimes endoscopic investigations. Quantitative culture of an uncontaminated sample of urine is the most sensitive screening test. An additional 548 children had positive physical findings such as enlarged kidneys and/or bladder and were excluded from this study. There were 2,970 children with urinary symptoms but no definite positive physical findings, and this group is discussed in detail; 73% (2,168) were boys and 27% (802) were girls. The commonest presenting symptoms were frequency of micturition, enuresis, and straining on voiding. One hundred sixty-six had significant bacteriuria and were fully investigated. Only 148 children completed the investigations and treatment; 130 had an underlying structural abnormality causing stasis of urine. It is well known that removal of the cause of the stasis will help to prevent further infections in most patients, and also reduces the effects of back-pressure on the upper tracts. This study illustrates: (1) the importance of proper urine culture technique; (2) the high incidence (88%) of structural abnormalities causing UTI in children; and (3) the importance of investigating all children with proven UTI to determine the cause of stasis. In India, the patterns of UTIs and their causes are markedly different from those published in the English literature.     

Urinary screening and urinary abnormalities in 3‐year‐old children in Japan

In Japan, urinary screening for preschool children has been obligatory since 1961. The system was reconsidered and has been under review since 2012, because many problems in the system had been identified, and its usefulness was uncertain. In the process, the following were analyzed: (i) frequency of urinary abnormalities identified on screening; (ii) diseases identified from urinary abnormalities; (iii) clinical course of children found to have urinary abnormalities; and (iv) screening for asymptomatic urinary tract infection (UTI) as a way of screening for congenital anomalies of the kidney and urinary tract. A computerized literature search was conducted, and study reports issued by the Ministry of Health, Labour and Welfare study group, and data of Akita City and Chiba City were reviewed. The prevalence of abnormal results at the first urinalysis was high, but at the second urinalysis the prevalence decreased in the range 1/6–1/20. The prevalence of tentative diagnosis at the third urinalysis was similar to the school urinary screening results. Serious illness was not found in children who had hematuria alone. In contrast, diseases requiring immediate attention were found in children with proteinuria, although the prevalence of proteinuria was not high. The dipstick method for leukocyturia was inefficient. The importance of two consecutive urinalyses before detailed examination, the lack of usefulness of screening for hematuria in 3‐year‐old children, and the importance of proteinuria were confirmed. Screening for asymptomatic UTI using urinary leukocytes was very inefficient.     

营养支持对高营养风险患儿临床结局的影响

目的探讨营养支持对有高营养风险患儿临床结局的影响。方法应用改良的儿科营养不良筛查评分法(STAMP),对连续入院的1 296例患儿进行营养风险筛查评分,并分析营养支持对临床结局的影响。结果 1 296例住院患儿中,379例STAMP评分≥4分,高营养风险检出率为29.24%。304例患儿纳入进一步分析,其中85例(27.96%)有营养支持。营养支持患儿中,37例肠外营养支持(PN),23例肠内营养支持(EN),25例PN和EN联合应用;EN、PN、PN和EN联合应用比较,人均和日均营养支持费用的差异有统计学意义(P均0.001);EN的人均和日均费用均为最低。与无营养支持患儿比较,营养支持患儿感染发生率有所降低,但差异无统计学意义(P=0.095);无营养支持患儿抗感染治疗费用高于有营养支持患儿,抗生素治疗费用也明显高于有营养支持患儿,差异均有统计学意义(P均0.001)。无营养支持、EN、PN、PN+EN四组间住院费用差异有统计学意义(P=0.003),而四组间住院时间差异无统计学意义(P=0.213)。结论改良的STAMP评分作为儿科患者营养风险筛查工具简单实用;不同的营养支持方式费用存在差异;营养支持可有效改善临床结局,特别是减少感染和降低抗感染治疗费用。