Long-term outcome of primary nephrotic syndrome.

One hundred and fourteen children with primary nephrotic syndrome were followed up prospectively for periods of between 5 and 14 years. Urine samples from 94 of them became protein-free during the initial 8-week course of prednisone, and the outcome for these children was good: 74 of them have been free of symptoms for at least 3 years, 18 have had relapses during the last 3 years, and only one child still has proteinuria. All these children have normal renal function and blood pressure. One child died accidentally. Twenty children did not respond to the initial prednisone treatment. Thirteen of them had remissions later, of whom 2 have had relapses during the last 3 years. Seven were totally resistant to prednisone 4 of whom died in renal failure, the remaining 3 have persistent proteinuria with normal levels of creatinine; one has high blood pressure too. Remission during the initial treatment indicated a good prognosis, but two-thirds of the initial non-responders also fared well.     

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目的探讨晚发型癫痫性痉挛患儿的临床和脑电图(EEG)特征、治疗反应及预后。方法对北京大学第一医院儿科2000年6月至2007年8月59例晚发型癫痫性痉挛患儿的临床和EEG资料进行回顾性研究,其中37例有随访,随访时间13～90个月,末次随访年龄为2岁6个月至12岁2个月(中位数60个月)。结果59例患儿癫痫起病年龄为12～98个月,中位年龄22个月。以痉挛发作起病36例(61.0%),以其他发作类型起病23例(39.0%)。17例(28.8%)在病程中有部分性发作,22例(37.3%)合并其他全面性发作形式,20例(33.9%)病程中仅有痉挛发作。EEG14例(23.7%)为典型高度失律;2例(3.4%)为一侧性高度失律;43例(72.9%)无典型的高度失律。总计22例(37.3%)表现为局灶性或一侧性临床、影像学和(或)EEG异常。44例(74.6%)为症状性癫痫,围生期脑损伤、中枢神经系统感染、脑结构异常为主要病因。14例(23.7%)符合晚发型West综合征,7例(11.9%)符合Lennox-Gastaut综合征。促肾上腺皮质激素(ACTH)治疗后近期无发作率32.0%,有效率58.0%,复发率为61...     

Extramembranous nephropathy in black South African children

Membranous nephropathy is the most frequent histological category among black children with nephrotic syndrome. In this study 31 African children with this condition are described. There were more boys than girls and the peak age was four to 11 years. The incidence of this histological category and clinical outcome in the African children were similar to these features in adults with membranous nephropathy. During a follow-up period of up to six years there was spontaneous remission in a third of patients, persistent proteinuria in just over a third (37.5%) and persistent relapse in under a third (29.2%). Hypertension occurred more frequently (19.3%) and spontaneous remission less often (33.3%) than in children with membranous nephropathy elsewhere. Hypertension, the lower remission rate and persistence of proteinuria during the course of the disease were similar to the disease seen in adults. Renal failure was not encountered in any patients. Steroids were of little value in the treatment of these children. Five children (16.2%) had associated infections. HBsAg was present in three of six children tested.     

Nebulised beclomethasone dipropionate in recurrent obstructive episodes after acute bronchiolitis.

Forty four children with recurrent obstructive episodes after acute bronchiolitis in infancy were treated with nebulised beclomethasone dipropionate or placebo for eight weeks in a randomised double-blind study. They were seen monthly for a year afterwards, and also if they had acute respiratory illnesses with or without bronchopulmonary obstruction. The two treatment groups were well matched. The children receiving active treatment had significantly fewer symptomatic respiratory illnesses and fewer episodes of bronchopulmonary obstruction during the follow up period. The children given placebo had significantly higher obstructive scores during the study period, and they were treated with inhaled beta 2 agonists and theophylline for longer periods of time during the follow up period. The results suggest that nebulised beclomethasone dipropionate may have prolonged effects on subsequent asthmatic symptoms after termination of treatment in children with recurrent obstructive episodes after acute bronchiolitis.     

Effects on growth of single short courses of fluoroquinolones

The aim of the study was to document the effects of short courses of fluoroquinolones given during an outbreak of multidrug resistant typhoid fever in southern Viet Nam on the growth of children over a period of two years. In a prospective cohort study, 326 Vietnamese children aged between 1 and 14 years were followed up for two years after receiving either ciprofloxacin (70 mg/kg given over 7 d) (n = 173) or ofloxacin (45-50 mg/kg given over 3-5 d) (n = 153) for suspected typhoid fever. Growth velocity and weight for height were compared with an age matched control group of children from the same locality (n = 223) who had not contracted typhoid or received any fluoroquinolones. In the ofloxacin and ciprofloxacin treated patients there was no evidence of acute joint toxicity, nor of any joint symptoms attributable to either of the fluoroquinolones. There was no difference in expected weight for height measurements between the three groups of children over the two year period. During the first year, height velocity in ciprofloxacin treated children was greater than in either ofloxacin treated children or untreated controls. Height velocity in the latter two groups was not significantly different. After two years height velocity was similar in the three groups. The results support the use of short course fluoroquinolone treatment in childhood typhoid, especially when caused by strains resistant to other antibiotics.     

Henoch-Schönlein purpura in north-eastern Turkey

AIM: To evaluate the epidemiological and clinical findings in children with Henoch-Sch?nlein purpura (HSP) admitted during a 10-year period, 1995 to 2004, and to compare them with series from other parts of the world. METHODS: The medical records of all children aged 17 years or less admitted with a diagnosis of HSP to the Department of Pediatrics of Karadeniz Technical University were evaluated retrospectively for epidemiological and clinical features. RESULTS: Of 116 children, 73 (63%) were boys. The mean (SD) age at presentation was 8.9 (3.7) years and one-third of them were older than 10 years of age. Over half the cases presented between September and January. All patients had the typical skin rash. Gastro-intestinal manifestations were seen in 64 (55.1%) and joint manifestations, common during the early course of the disease, in 73 (62.9%). Two patients required laparatomy, one for acute abdomen and the other for bowel resection owing to intussusception. Renal manifestations were observed in 36 (31%), all within 3 months of initial symptoms, and one patient (0.8%) with nephritic syndrome progressed to end-stage renal disease. Five patients had hypertension without urinary findings. Symptoms recurred in eight patients (6.9%) over a period ranging from 2 to 5 months after complete resolution of symptoms. There was a history of a preceding upper respiratory tract infection in 16 (13.7%) and a streptococcal infection was confirmed by throat culture in 12 of the 42 (28.5%) children at presentation. CONCLUSION: HSP is generally benign and self-limiting. Hypertension may be seen during the course of the disease without urinary findings. In this area, it seems to affect older children and there is a relatively lower incidence of renal manifestations.     

儿童脑脓肿的显微外科治疗体会

目的 儿童脑脓肿显微外科手术切除的治疗经验总结和分析.方法 回顾性分析2000年1月至2007年12月30例行显微外科手术切除治疗儿童脑脓肿的方法和疗效.结果 手术均全切除脓肿壁.随访21例治愈患儿,17例仍保持治愈,余4例未经影像复查但无异常临床表现.随访6例好转者,2例转为治愈,2例留有癫痫,1例留有左侧肢体偏瘫,1例死于先天性心脏病.结论 儿童脑脓肿的显微外科手术切除,应当选择适当的手术时机,并且要选择合适的手术入路,必要时行术中B超定位,完整剥离脓肿壁,能达到治愈的目的.     

Retrospective review of cystic fibrosis presenting as infantile liver disease.

The mode of presentation, clinical course, and outcome of 12 infants with cystic fibrosis and liver disease referred over an 18 year period were investigated retrospectively. Median age at presentation was 6.5 weeks (range, 5-12). Two thirds were boys. Conjugated hyperbilirubinaemia was the presenting symptom in 11 patients, and hypoalbuminaemia in one. Jaundice was cleared over a median period of 7.36 months. Eight patients had bile duct proliferation on liver biopsy and one required cholangiography to exclude biliary atresia. Classic histological features of cystic fibrosis were only present in two children biopsied at 8 and 18 months. Three patients had meconium ileus, including one infant with concomitant alpha(1) antitrypsin deficiency, who required early liver transplantation. All other patients had no signs of significant chronic liver disease during a median follow up of 42 months (range, 10-205). Children with cystic fibrosis and infantile liver disease have a good short and medium term prognosis.     

Non-convulsive status epilepticus.

The clinical, electrographic and reported neuropsychological features of 50 children with non-convulsive status epilepticus (NCSE) were reviewed and the children''s progress followed for one to five years. NCSE occurred in a variety of epilepsies, especially the Lennox-Gastaut syndrome. Clinical manifestations ranged from obvious mental deterioration to subtle changes. The condition had often been overlooked or misinterpreted and many children had experienced repeated episodes over long periods. Following diagnosis, immediate treatment was often not attempted or was not successful. Further episodes of NCSE occurred in the majority of children during the follow up period. Failure to recognise NCSE and to treat episodes promptly, and the high rate of recurrence, is of particular concern in view of fears that repeated exposure to this condition might be brain damaging. At least 28 children in the present series showed evidence of intellectual or educational deterioration over the period during which NCSE had occurred, although the exact cause was difficult to determine.     

Idiopathic thrombocytopenic purpura in childhood: Twenty years of experience in a single center

Background: Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder with a variable clinical course. Methods: A retrospective analysis was carried out of ITP patients presenting to a pediatric hematology‐oncology department during a period of 20 years, with a focus on treatment and outcome. Results: One hundred and twenty‐four cases were recorded (mean patient age, 8.4 years). Forty‐nine children (39.5%) had platelet counts <10 000/µL at diagnosis. No episode of severe bleeding was observed. Peak incidence was observed during spring and summer. Respiratory infections proceeded in 58% of cases. Treatment consisted of i.v. immunoglobulin (IVIG) in 93 children at four dosing schedules. Sixteen children received corticosteroids, 10 children received anti‐D immunoglobulin and 14 received no treatment. Recovery was observed in 67% of children on IVIG and in 50% on anti‐D globulin. Eight patients did not respond initially and received corticosteroids. Three children with refractory thrombocytopenia received anti‐CD20 (rituximab). Fourteen children (11%) had persistent/chronic disease. In 10 of them recovery was observed in 13 months–8 years. Splenectomy was performed in six children with resistant/chronic disease. Conclusion: ITP has a benign course in the majority of cases. Anti‐D globulin can effectively be used as an alternative first‐line treatment. Rituximab can successfully be used in refractory cases, while splenectomy has currently limited indications.     

Prolonged interferon alpha treatment in children with chronic hepatitis B

Interferon alpha has been used widely to treat hepatitis B virus infection in children. However, the overall initial response rates have been < 50% and several strategies have been attempted to improve this. The aim of this study was to evaluate the safety and efficacy of prolonged interferon alpha treatment in children who did not respond to a previous course of interferon alpha treatment. Twenty-seven children with chronic hepatitis B who had not responded to a 6-month course of interferon alpha 2a (5 MU/m2 body surface) thrice weekly subcutaneously continued to receive interferon alpha at the same dosage for another 6 months without a rest phase. The children were followed for 6 months after completing 12 months of therapy. All of them had HBsAg, HBV-DNA and HBeAg tested on completion of the first course. Six of the 27 (22.2%) cleared both HBV-DNA and HBeAg after completion of therapy and all six had a sustained response. Pre-treatment predictive factors were not significantly associated with treatment response. No adverse effect of interferon was seen during follow-up. We conclude that prolonged interferon treatment is well tolerated and leads to additional benefit.     

Short versus long initial prednisone treatment in steroid-sensitive nephrotic syndrome in children

A total of 184 children aged, 13 months to 11 years, suffering from their first attack of steroid-responsive nephrotic syndrome were included in a randomized study. They were treated according to three treatment protocols. All children received l-2mg of prednisone/kg body weight/day (up to 80 mg daily) for 4 weeks, and thereafter 1 mg/kg body weight/48 h for the next 4 weeks. Treatment was discontinued at this point in 44 children (protocol A); in 68 (protocol B) the dose was reduced by 25% each week, tapering off to 0 at the end of the third month, while in 72 children (protocol C), after the first 2 months of initial treatment the dose was reduced by 25% each month and tapered off to 0 by the end of the sixth month. All patients completed a 2-year follow-up period after withdrawal of prednisone. Treatment results were expressed as: percentage of children relapse-free within the first 6 months and 2 years after withdrawal of treatment, and average number of relapses per patient per year. The best results were obtained in children who had been treated for 6 months: 65.3% of them remained relapse-free within the first 6 months and 50% over the entire 2-year follow-up period; the number of relapses per patient per year in this group was 0.49. The respective values for children treated 2 and 3 months were: 36.4% and 32.4% for the 6-month period; 27.3% and 20.6% for the 2-year period; the numbers of relapses per patient per year were 0.79 and 0.77, respectively. The frequency of corticosteroid side effects such as transient hypertension and cushingoid obesity during the initial treatment, and growth retardation or recurrent "respiratory tract" infections observed during the following 2-year follow-up period, did not increase after prolongation of the initial treatment. Initial treatment period, steroid-sensitive nephrotic syndrome
J Ksiek, Department of Nephrology, Child Health Centre, 04-736 Warsaw, Poland     

Follow-up of children of drug-addicted mothers.

During a period of 2 years (1971-72) 19 newborn infants were admitted to hospital because their mothers were drug addicts. To evaluate the prognosis in these children, 17 were followed up by a social adviser, a psychologist, and a paediatrician. During the neonatal period 16 of the infants had withdrawal symptoms, for which 11 required medical treatment. One infant died of congenital malformations. Of the surviving 18 infants 14 were discharged to their mothers and 4 went to a children's home. During follow-up, which varied from up to 2 months to up to 2 years 8 months of age, 10 of the children had to be placed in a children's home for a period. No physical abnormalities were found in the children. Motor and perceptual development were normal in 12 but in 3 speech development was delayed. Five mothers ceased to take drugs after delivery and 2 had done so during early pregnancy. The pre- and perinatal complications and the undesirable environment in which the children grow up show the need for a comprehensive treatment programme.     

Pulmonary and mediastinal lesions in children with Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is an uncommon group of disorders affecting mainly children and young adults. In children, pulmonary involvement occurs mostly in the disseminated forms; isolated pulmonary lesions are unusual. A retrospective study was undertaken on a group of 42 children diagnosed with LCH over a 19-year period. Eight children (19 %) had radiological evidence of pulmonary involvement. The lung lesions were either present at the time of diagnosis or, when appearing during the course of the disease, always coinciding with exacerbation or recurrence of the disease in other sites. Lung involvement did not appear to be an unfavourable prognostic factor. However, the toxic effects of treatment on the lungs might lead to important pulmonary sequelae. Received: 3 February 1997 Accepted: 23 June 1997     

Intelligence and quality of dietary treatment in phenylketonuria.

The records of the Phenylketonuria Register were examined to determine the factors associated with early intellectual progress in children who had received a diet low in phenylalanine from soon after birth. A total of 1031 children were born between 1964 and 1980 and started treatment before they were 4 months of age, and 808 of them were followed up prospectively. In 263 children born between 1964 and 1971 (cohort 1), the mean intelligence quotient (IQ) at 4 years rose with year of birth from 24 points below revised IQ norms to 10 points below; in 545 born between 1972 and 1980 (cohort 2) there was no further rise in IQ and the mean remained eight points below the norms. IQ fell progressively by roughly four points for each four weeks'' delay in starting treatment, for each 300 mumol/l rise in mean phenylalanine concentrations during treatment, and for each five months during the first two years during which phenylalanine concentrations were below 120 mumol/l. Forty six children in cohort 2 had the most favourable treatment characteristics. Their mean IQ, after standardising for social class, was 112.6, and similar to the mean for the same group estimated by multiple regression, and to revised population IQ norms for the period during which the children were tested. The data suggest that many children who are treated early continue to suffer a mild degree of neurological impairment because of the difficulties in fully controlling the metabolic abnormality.     

Cutaneous hypersensitivity reactions due to thiacetazone in the treatment of tuberculosis in Zambian children infected with HIV-I.

Tuberculosis is one of the most common infections in Zambian adults and children infected with HIV. In Africa, cutaneous hypersensitivity reactions attributed to thiacetazone during treatment of tuberculosis in adults infected with HIV-I have been well documented. This study monitored adverse drug reactions during treatment for tuberculosis over an 18 month period (1 April 1990 to 31 October 1991) in 237 children with a clinical diagnosis of tuberculosis (125 boys and 112 girls; 88/237 (37%) infected with HIV-I) and 242 control children (149 boys and 93 girls; 26/242 (11%) infected with HIV-I). Twenty two (9%) of the 237 children with tuberculosis developed hypersensitivity skin reactions during the course of treatment. Adverse skin reactions were seen more often in children infected with HIV than in those who were not (odds ratio 11.65, 95% confidence interval 3.07 to 34.88). These represented 19 (21%) of 88 children infected with HIV and three (2%) of 149 children not infected with HIV. These skin reactions occurred after a period of treatment ranging between two and four weeks among 14 children receiving the HST (isoniazid, streptomycin, thiacetazone) regimen and eight children receiving the HSTR (isoniazid, streptomycin, thiacetazone, rifampicin) regimen. Twelve (55%) of the 22 children who reacted adversely to treatment developed the Stevens-Johnson syndrome. All 12 of these children with the Stevens-Johnson syndrome were infected with HIV. The mortality among these children who developed the Stevens-Johnson syndrome was 91% (11 of 12 died within three days of the onset of the reaction). No further reactions were observed in the 11 children who recovered from the cutaneous hypersensitivity reactions after thiacetazone was discontinued over a period of six months of further treatment of tuberculosis. The results of this study were in part responsible for the recommendations put forward by the World Health Organization to avoid the use of thiacetazone in the treatment of tuberculosis in children infected with HIV.     

Apnea states in infants, anxiety of parents and pediatricians, and home monitoring of respiration in children

In order to determine parental reasons of the use of home apnea/respiration monitors and to estimate the grounds of these decisions correspondence contact was achieved with the parents of 71 infants monitored by means of the APNEAL-1 device. Filled up questionnaires were the source of data regarding the families (structure, health practices, mothers' age and education, course of pregnancy and delivery), monitored infants (birth weight, Apgar score, apnea events) and the reasons of monitoring. Also opinions concerning monitor usefulness and performance were gathered. The majority of our families (> 97%) consisted of both parents, their socio-economic status was medium (52.11%) or good (46.48%) and they were residents of cities (88.73%). Cigarette smoking was present in 22 families (31%) with 13 smoking mothers (18.31%). Mothers' mean age was 29.11 years (SD 4.84), 45% of them had high school education and 36.62%-university education. As many as 45% of the pregnancies had a pathological course and 34.3% were terminated by a preterm delivery. Mean birth weight of monitored children was 2914.93 g (SD 971.4), mean Apgar score was 8.31 (SD 2.46). More than 60% of children were breast fed during the first six months of life, and 30% of them--during first year of life. Bed sharing was reported in only 8% of children. Apnea episodes were observed in 30 infants (42.25 of whole group), in 20 of them only during the neonatal period. More apnea episodes were present in premature infants (48% of premature versus 30% of full term infants). Only 12 children (16.9% of whole group) manifested apnea events during the monitoring period. However, almost all the parents (97%) had a high opinion of the role played by the monitor (mean monitoring time 7.12 months, SD 4.44). In 27 families (38%) a cause of monitoring was a preceeding apnea event. The decision of remaining parents was based exclusively on fear. This fear was justified only in a small number of families (previous SIDS victim, GER). Numerous children were monitored solely on the basis of information of apnea existence in children. Such a difficult to accept monitoring reason probably has its source in inappropriate family health education.     

Pancreatoblastoma in childhood: clinical course and therapeutic management of seven patients

BACKGROUND: To analyze the clinical course of pancreatoblastoma in children and to propose management and therapy. PROCEDURE: Retrospective review of seven cases of pancreatoblastoma treated in France over a 20-year period and literature review. RESULTS: Median age of patients was 6 years. The tumor was well encapsulated in one patient and had direct extension to adjacent tissues in two others. Three patients had regional lymph node involvement and one had liver metastases. The tumor arose in the head of the pancreas in three children, in the tail and body in two and in the tail and in the body, one each. Elevation of AFP serum levels was noted in four out of 6 cases. Five tumor resections were performed, one initially and 4 after neoadjuvant chemotherapy, and cisplatin plus doxorubicin seemed effective. Two children received post-operative irradiation because of incomplete resection. In all, four children are disease free with a median follow-up of 50 months ( range, 5--120 months) : one had a complete removal of tumor at diagnosis and no further treatment, three had unresectable tumor at diagnosis and received neoadjuvant chemotherapy with cisplatin and doxorubicin. One of them also received post-operative irradiation. CONCLUSIONS: Pancreatoblastoma is a curable tumor. Examination of serum AFP levels may be useful for diagnosis and to follow the course of the disease. Complete resection is the treatment of choice. However, tumor is often unresectable at diagnosis and preoperative chemotherapy is needed to reduce tumor volume. We suggest a regimen that include cisplatin and doxorubicin. In patients with incompletely resected disease, postoperative radiation may be indicated.     

Atypical presentations in children with post-neonatal tetanus in Ibadan, Nigeria

In a retrospective review of 73 children with post-neonatal tetanus seen over an 11-year period at University College Hospital, Ibadan, Nigeria, atypical portals of entry of the causative agent were recorded in nine and five developed unusual complications during the course of treatment for tetanus. Localized tetanus was encountered in seven children; this involved the cephalic region in one and one lower limb in each of the remaining six. Among these six children, the portal of entry of the organism included intramuscular injections into the buttock in five and an infected insect bite on the buttock in one case. Another unusual portal of entry, observed in two children, was through an impacted foreign body in the ear in the absence of otitis media. Arthritis of large joints was encountered in three children and two had episodes of severe bronchospasm which responded to bronchodilator treatment. The findings in this study significantly widen the reported spectrum of presentations and complications of tetanus in third-world children.