Crescentic glomerulonephritis in children: a single centre experience

Background

Crescentic glomerulonephritis (CsGN) is characterized by crescents in 50% or more of glomeruli and clinically by a sudden and progressive decline in renal function.

Methods

We evaluated the etiology, clinical features, prognostic factors and long-term outcome of CsGN. Between January 2000 and December 2010, 45 children (26 girls, 19 boys) with biopsy-proven CsGN (>50% crescents) were investigated retrospectively.

Results

The mean age of the patients was 130.86±33.77 months. The mean duration of symptoms prior to diagnosis was 26±12 days (4-40 days). Most of the children had hypertension (62.2%), macroscopic hematuria (73.3%), oligoanuria (44.4%), edema (51.1%) and purpuric rash (40%) at presentation. The final clinical status of the patients was complete remission (n=21), partial remission (n=5) or chronic kidney disease (n=19). Adverse outcomes were significantly associated with a long duration between the onset of symptoms and treatment (P=0.038), the presence of oligoanuria (P=0.006), a severe decreased glomerular filtration rate (GFR <30 mL/min/1.73m²) and the need for dialysis (P=0.003) on admission, the ratio of crescents (>75%) (P=0.03), and the ratio of fibrous crescents (P=0.015).

Conclusion

The outcome of CsGN in children continues to be poor, and it should be treated as a renal emergency.

     

Sacrococcygeal teratoma: the 13-year experience of a tertiary paediatric centre

Aim: To describe the management, morbidity and mortality of infants admitted to a tertiary paediatric hospital in New South Wales, Australia with a diagnosis of sacrococcygeal teratoma (SCT). Methods: All neonates admitted to the neonatal intensive care unit with a SCT between January 1996 and December 2008 were included in this retrospective review. Data collected included maternal and neonatal demographics, time of diagnosis, tumour characteristics, surgical treatment, operative complications and outcomes. Results: Seventeen infants with a diagnosis of SCT were included in the study. Of these infants, the majority (70%) were born at term, and eight had a prenatal diagnosis made during the second trimester. Associated anomalies were detected in seven infants (41.8%), with renal anomalies being the most common. Tumour histology included mature (50%, n= 8), yolk sac tumour (18.75%, n= 3), immature (6.25%, n= 1) and mature with mixed elements (25%, n= 4). Recurrent disease occurred in two infants within 4–18 months of the primary resection, with one infant suffering a second recurrence. Only one child died prior to surgery, giving a survival rate of 94%, and mean age at follow‐up was 32 months. Long‐term sequelae found in four babies included revision of scar, vesicoureteric reflux, post‐surgical neurogenic bladder and osteotomy for hip dysplasia. Conclusions: The overall survival of neonatal SCT is high. While this is a small series, our results are consistent with the literature. Important components of management include timely diagnosis, multidisciplinary planning, long‐term follow‐up and intervention for functional sequelae.     

Congenital lumbar hernia: 20 years' single centre experience

Aim: Congenital lumbar hernia is an uncommon anomaly with only few cases reported in the English literature. This study was done to study the incidence, associated conditions, management and complications of congenital lumbar hernia repair in paediatric patients. Methods: Retrospective study of all patients over a period of 20 years (January 1990 to January 2010) was analysed. Results: A total of 18 patients (two bilateral hernias) were encountered in this series. All were in the age group of 1 day to 6 years. All the patients were operated by open technique. Primary repair was done in 14 patients and prosthetic meshplasty in two. Two patients could not be operated. The average follow‐up period was 3 years. There was no follow‐up loss in our study. There was no evidence of recurrence in this series. Conclusion: Up until now, the total number of cases is 68. The large size of hernia defect (>10 cm) may determine the use of prosthetic mesh repair. Proper surgical treatment, either primary repair or hernioplasty, provides adequate management of this condition.     

Acquired central diabetes insipidus in children: a 12-year Brisbane experience

OBJECTIVE: To study the clinical, endocrine and radiological features and progress of children presenting with acquired diabetes insipidus (CDI). METHODOLOGY: Chart review of children presenting because of CDI to Brisbane paediatric endocrine clinics between 1987 and 1999. RESULTS: Thirty-nine children (female/male ratio 21/18) aged 0.1-15.4 years (mean age 6.7 years) were identified. Aetiologies were head trauma or familial in eight cases (20.5%) each, central nervous system (CNS) tumours in five cases (12.8%), CNS malformations in four cases (10.2%), histiocytosis in three cases (7%) and hypoxia and infection in two cases (5.1%) each. Seven cases (17.9%) remain undiagnosed. Of the 32 (82%) cases with isolated anti-diuretic hormone deficiency at presentation, 24 cases (61.5%) experienced no further endocrine deficit. Additional endocrine deficits occurred mainly in the tumour or undiagnosed groups. On follow-up brain magnetic resonance imaging (MRI) scans in the seven undiagnosed cases, six patients had mild or no change and one patient had marked improvement of MRI findings. These changes occurred 10-48 months (mean 18 months) after presentation. CONCLUSIONS: Children without an aetiological diagnosis for the uncommon condition of acquired CDI require careful follow-up. More intensive investigation at presentation (e.g. estimation of cerebrospinal fluid human chorionic gonadotrophin) promises to lessen the number of such cases. Pituitary stalk biopsies should be reserved for those patients with progressive MRI changes. If these changes do not occur early, our experience suggests that follow-up MRI scans may need to be performed only yearly.     

Avoiding steroids in pediatric renal transplantation: long-term experience from a single centre

We report our experience in pediatric renal transplantation avoiding steroids whenever possible. Immunosuppression consisted of an initial induction with antithymocyte globulin followed by maintenance therapy with a calcineurin inhibitor and MMF. Steroids were only given to selected patients because of the primary disease, recurrence, rejection, or PTLD. Thirty-four transplants grafted into 32 recipients between 1995 and 2005 were followed for a median of 3.5 yr (range 1-9.8). All patients survived. Graft rejection occurred in 10 cases during the first year post-transplantation and graft survival at one, five, and seven yr was 97, 88 and 88%, respectively. Steroids were given to half of the patients (n = 16); in nine cases due to rejection. Only four patients (13%) were continuously on steroids. Calculated GFR at one to five yr post-transplant were 73, 74, 68, 64, and 70 mL/min/1.73 m(2). Unfortunately PTLD occurred in three patients, but all survived with functioning grafts. Accordingly, our findings indicate that steroid avoidance in pediatric renal transplantation is possible with good results with respect to acute graft rejection as well as long-term graft survival.     

Ovarian cancer in children and adolescents: A review of 15 cases

Fifteen patients, admitted between 1961 and 1975, from 8 to 19 years of age, with malignant ovarian tumors were reviewed. The histology was: dysgerminoma in 5, common “epithelial tumor” in 5, immature teratoma in 4, granulosa cell tumor in one. Of the 15 patients, 5 are alive and well, 4 of these had dysgerminoma, 1 had a papillary cystadenocarcinoma. Of 3 patients with dysgerminoma in Stage III, 2 are among the survivors. Dysgerminoma, in contrast with other types of germ cell tumors, may be curable even in advanced stage.     

Chronic neutropenia during childhood. A 13-year experience in a single institution

To evaluate the clinical course and characteristics of children with chronic neutropenia, we reviewed the charts of all such patients seen at our center during a 13-year period. A total of 50 patients with chronic neutropenia were identified. Three patients had documented congenital neutropenia, and two siblings had cyclic neutropenia. The remaining 45 children had chronic neutropenia of unknown origin. All children except two had a remarkably benign course despite markedly reduced granulocyte counts. Of six girls in this group who had abscess or cellulitis of the labia majora, it was a presenting manifestation in three. Resolution of neutropenia was documented in 23 (62%) of 37 patients for whom follow-up information was available, with a median duration of neutropenia of 19 months. No differences were evident between patients with positive antineutrophil antibody test results and those in whom the test yielded negative results or was not performed. Chronic neutropenia in childhood is a relatively uncommon entity, characterized by a benign course and eventual resolution in the majority of patients.     

Carcinoma of the thyroid in children: A 25-year experience

Over the past 25 years, 23 children with carcinoma of the thyroid have been treated at the Christie Hospital, Manchester. Twenty-one cases were well-differentiated carcinoma, and two were medullary carcinoma. They were all treated by resection, 14 with total thyroidectomy and 9 with lobectomy or subtotal thyroidectomy. Sixteen children also had surgery for nodal disease. Two children presented with lung metastases. Sixteen children received post-operative radiotherapy (4 external beam, 12¹³¹I). Median follow-up of 67 months (range 7–233), was the same for the 21 well-differentiated carcinomas and the whole group including the two medullary carcinomas. All 21 children with well-differentiated carcinomas are alive with no evidence of progressive disease. Two relapsed after total thyroidectomy, but both were salvaged, one with external beam radiotherapy, one with ¹³¹I. One child with medullary carcinoma died with progressive disease after 43 months, the other is alive, but with slowly progressive disease 145 months after diagnosis. Ten of 14 children experienced post-operative hypocalcaemia following total thyroidectomy, in 7 cases it persisted long-term. ¹³¹I and external beam radiotherapy were both well tolerated. The long-term results of treatment of well-differentiated carcinoma of the thyroid are excellent, but there remains disagreement over the extent of treatment required. Some authors believe the condition is multifocal and requires total thyroidectomy, others argue that lobectomy or subtotal thyroidectomy avoids the possible post-operative complications of total thyroidectomy and gives equal long-term cure rates. We agree with the latter view. Although a small series cannot be conclusive, we feel that our results are consistent with this. We also believe, that for children, radiotherapy can be reserved for relapse only, as long as regular follow-up is available. Med. Pediatr. Oncol. 29: 103–107, 1997. © 1997 Wiley-Liss, Inc.     

Chronic peritoneal dialysis in children: catheter related complications. A single centre experience

Despite advancements in catheter design and dialysis technique, catheter related complications still remain a common clinical problem in paediatric patients on chronic peritoneal dialysis (PD); in particular, infections are a common cause of patient’s morbidity and technique failure. In the present paper, data on 89 catheters implanted between January 1986 and December 2002 are reviewed to analyse the major causes of complications and/or PD failure and to ascertain their optimal management. A total of 89 catheters were implanted in 78 patients at the start of chronic PD: 26 in children under 2 years of age, 14 in children aged 2–5 years and 49 in patients over 5 years. Mean age of patients was 76.1 ± 73.0 months and median treatment time 14.5 ± 13.1 months. All catheters were surgically implanted and partial omentectomy was performed in 70% of cases. Straight Tenckhoff catheters were used in 70 cases (78%), curled ones in 19 (22%). Sixty-three catheters (71%) had two cuffs, 26 (29%) a single cuff. The entry-site was the midline in 34 patients (38%) and the paramedian line in 55 patients (62%). Catheter survival rate was 80% at 12 months, 62% at 24 months and 58% at 36 and 48 months, respectively. The incidence of catheter-related complications was one episode every 6.4 PD-months, and they were mainly represented by peritonitis (61%), exit-site infections and tunnel infection (ESI + TI: 23%), catheter obstruction (5%), dislocation (3.5%), leakage (2.5%). After the introduction of curled single-cuff catheters, a considerable reduction in the peritonitis incidence was observed during the last 7 years. A more prolonged catheter survival was observed in older children (>5 vs. <2 years: P < 0.05). Leakage was less common in catheters with paramedian entry-site compared with catheters implanted on the midline. In 7 out of 11 (64%) patients with catheter obstruction, omentectomy had not been performed. Single-cuff catheters had a lower infection-rate than double-cuff catheter (P < 0.01). Single cuff-curled Tenckhoff catheter can be considered the first choice catheter. Single cuff-catheters are not associated with an increase of infections. The surgical technique requires a strict adherence to a standardized procedure and a dedicated team, in order to obtain a reduction of the complications, a prolonged catheter duration and a better quality of life.     

Pediatric tracheostomy: a 13-year experience

Pediatric tracheostomy has been reported to be a surgical procedure with significant morbidity and mortality. The use of tracheostomy in airway management has changed over time as regards indication and outcome. A review of the last 13 years experience in our institution was carried out to focus on this group of patients and the recent trends in airway management. A retrospective analysis of hospital records was done and information collected with respect to age, gender, indication for tracheostomy, duration, complications, and follow-up. Thirty-nine tracheotomies were done in 36 patients, of whom males outnumbered females 2:1. The mean patient age was 41.6 months while nearly a third were newborns. The indications were congenital and acquired obstructive lesions. Apart from nine cases, all have been treated and decannulated. Follow-up ranged from 1 month to 8 years, and decannulation time from 48 h to 45 months. Home tracheostomy care was very well managed by the parents. One tracheostomy-related death was encountered. Complications were minor and transient and occurred post-decannulation in our series, in contrast to the major complications, both acute and chronic, reported in the literature. More neonates and infants are undergoing tracheostomy and surviving. Pediatric tracheostomy is a safe procedure with home care by parents feasible.     

Tuberculosis in children: a 13-year follow up of 1714 patients in a Belgian home care centre

From May 1970 to September 1983, 1714 children with different forms of primary tuberculosis were referred to the paediatric home care centre (Enfants soignés au Foyer, E.S.F.) of the Brussels University Hospital St.-Pierre. They were subdivided in five groups: asymptomatic (33%), symptomatic (28%), dubious tuberculous infections (35%), high-risk contacts (3%) and unestablished diagnosis (1%). They were aged from 10 days to 19 years, and 82% of them were migrants of low socio-economic level. Fifty percent of the symptomatic infections, mainly pulmonary, appeared in children under 3 years of age. An adult source of contamination was identified in 33% of the case (48% of the symptomatic children). Diagnosis was based on tuberculin screening with a 2IU intradermal test. Gastric aspirates yieldedMycobacterium tuberculosis in 15% of our patients, 11% of them showing resistance to one or more tuberculostatic drugs. Treatment was given to 1359 patients with excellent results. Therapy was shortened during the last 2 years of the study from 12 to 6 months for the asymptomatic patients and from 12 to 9 months for the symptomatic infections. Few complications were observed. Tuberculosis remains a serious cause of morbidity particularly in migrant children. Correct diagnosis and treatment of the disease is very important.     

Hodgkin's disease in children: A retrospective study of the 20-year experience (1968–1987) at a single institute

Between 1968 and 1987, 356 previously untreated consecutive children with Hodgkin's disease (HD), aged under 14, were treated at the Pediatric Department of N.N. Petrov Research Institute of Oncology (St. Petersburg, Russia). Prevailing among histologic subtypes were lymphocytic predominance and mixed cellularity (34 and 31.7%, respectively). Two-thirds of the patients (67.7%) were with Stages III-IV and one-third (32.3%) with Stages I-II. Radiation therapy (RT) as a primary method of treatment was used in 72 patients (20.2%), chemotherapy (CT) in 76 (21.4%), and combined (RT + CT) therapy (CMT) in 208 cases (58.4%). The patients were followed from 6.3 to 26 years (median follow-up was 13.7 years). For the whole group 5-, 10-, 15-, 20-, and 25-year actuarial survival (S) rates were 90, 80, 74, 73, and 73%, respectively. The corresponding event-free survival (EFS) rates were 63, 56, 56, 55, and 55%. The amelioration of the results was noted beginning with the 1973–1977 period, and for those treated in the last period (1983–1987) 5-year S and EFS rates were 93 and 68%, respectively. We conclude, that the modern treatment is also effective for our children suffering from HD. © 1995 Wiley-Liss, Inc.     

Management of choroid plexus tumours in children: 20 years experience at a single neurosurgical centre

OBJECTIVE: Tumours of the choroid plexus are rare tumours of neuro-ectodermal origin, accounting for less than 1% of all intracranial tumours. Most cases present in children less than 2 years of age. While choroid plexus carcinomas (CPC) are reported to have an extremely poor prognosis, choroid plexus papillomas (CPP) are generally regarded as benign tumours with a very favourable long-term outcome. Management dilemmas are associated with the choice of surgical procedure, tumour vascularity, the treatment of hydrocephalus and the value of adjuvant therapy. The objective of this study was to review our experience with this rare tumour over a 20-year period. METHODS: Patients were identified from the Great Ormond Street Neurosurgical Brain Tumour Database. Over a 20-year period (1979-1999), 34 children were identified with a choroid plexus tumour. There were 25 cases of CPP and 9 cases of CPC. A retrospective review of case notes, radiological imaging, operation reports and pathology was performed. RESULTS: The median age at presentation was 17 months (1-138) for CPP and 13 months (2-102) for CPC. There was no sex difference for CPP. However, 8 of the 9 CPCs were male (89%). A complete surgical resection was achieved in all 25 cases of CPP and in 3 cases of CPC (33%). The median survival for CPPs was 75.5 months (2-228), with a median follow-up of 73.5 months (2-228). The median survival for CPCs was 6 months (1-90), with a median follow-up of 6 months (1-90). CONCLUSION: With modern neurosurgical practise, a cure should be the aim for all children with CPP. There is no evidence that adjuvant therapy has any role in the primary management of these children. However, CPC still has an extremely poor prognosis, and the efficacy of adjuvant therapy remains to be established.     

Surgery in infants and children with testicular and paratesticular tumours: a single centre experience over a 25-year-period

Testicular and even more paratesticular tumours in children are rare. The aim of the study is to characterise the spectrum of these lesions with focus on the feasibility and effectiveness of testis sparing surgery. Twenty-four boys treated between 1980 and 2004 at the University Leipzig Medical Centre were evaluated. At presentation patients were between 5 months and 18 years old (median 23 months). Generally a high rate of malignant or potentially malignant tumours was observed. The majority of these tumours occurred in the first three years of age. The spectrum of testicular tumours comprised 13 germ cell tumours (6 yolk sac tumours, 6 teratomas, 1 embryonal carcinoma) and 4 sex cord stromal tumours (2 Leydig's cell, Sertoli's cell, granulosa cell). Both Leydig's cell tumours were endocrine active. Further on, we observed 3 boys with paratesticular rhabdomyosarcoma (RMS), and three with testicular and paratesticular metastases (Wilms' tumour, neuroblastoma, leukaemia). Serum alpha1-fetoprotein (AFP) was clearly elevated in 5 of 6 yolk sac tumours but remained within normal limits concerning the other entities. Human chorionic gonadotrophin was normal in all cases tested. During the observation period high inguinal orchidectomy was the surgical standard method. Dependent on tumour histology, stage and the recommended treatment schedule postoperative chemotherapy was added. Testis sparing surgery was performed in 3 boys with primary testicular tumours (2 Leydig's cell, mature cystic teratoma). Local relapses were not observed. Systemic relapses occurred in 3 cases (2 RMS, leukaemia). During a median follow up of 5 years all patients with primary testicular tumours survived event free. Meta-analysis of the recent literature revealed that testis sparing surgery is feasible and save in prepubertal boys after exclusion of a malignant tumour. If a testis sparing approach is planned, the following criteria are essential: 1. The presence of a well defined circumscribed nodule confirmed by imaging. 2. Normal levels of serum AFP and hCG. 3. The presence of sufficient healthy testicular parenchyma. However, the high rate of malignant or potentially malignant tumours suggests that high inguinal orchidectomy should remain the surgical standard of therapy.     

Imaging features of pancreatic tumors in children: 13-year experience at a pediatric tertiary hospital

Background

Pancreatic tumors are rare in children, resulting in limited information regarding their frequency and imaging appearances.

Objective

To review all pancreatic tumors seen in children over a decade at a large tertiary pediatric institution and to describe multimodality imaging findings.

Materials and methods

We conducted a retrospective review of imaging studies performed in children with pancreatic tumors between January 2000 and December 2012, analyzing imaging features on available US, CT and MR examinations. We reviewed patient charts for clinical features, management and final diagnosis.

Results

We included 23 children in this study. Of these, 12 had solid and papillary epithelial neoplasms (SPEN), 3 had neuroendocrine tumors, 3 had lymphoma, and 1 each had hemangioendothelioma, benign congenital cysts with adipose tissue, dendritic cell sarcoma, metastatic rhabdomyosarcoma, and lipoma. All children with SPEN were teenage girls. SPEN showed characteristic imaging features including well-defined margins with surrounding capsule, solid-cystic components and hemorrhage.

Conclusion

Pancreatic tumors are uncommon in children. SPEN is the most common tumor and is seen predominantly in teenage girls and shows characteristic imaging features.