Does renal transplant in children with LUTD improve their bladder function?

Much is still unknown about LUT function after receiving renal graft. Graft function was the main focus of different studies discussing the same issue. However, these studies ignored the effects of the graft on lower tract function and more demand for bladder cycling and growth of the child. Therefore, we aimed at evaluating the LUT function after RT into patients with LUTD. We enrolled a retrospective cohort of 83 live renal transplant children with LUTD. The 44 patients in Group (A) had a defunctionalized bladder, and the 39 patients in Group (B) had underlying LUT pathology . All patients had clinical and urodynamic evaluation of LUT functions at least 1 year after RT. We found that the improvement in patients with impaired bladder compliance was 73% in Group (A) and 60% in Group (B), with no statistically significant difference between the study groups. In Group (B), there was statistically significant worsening of MFP (8.4%) and mean PVR (79.9%) after RT. In Group (A), mild but stable significant improvement of all clinical and urodynamic parameters was observed. Serum creatinine was significantly worse in patients with pathological LUTD compared with those with defunctionalized bladder but without significant effect on graft survival. All LUT variables seemed to have no adverse effect on graft survival except for use of CIC and augmented bladder. Incident UTI independent of LUT variables accounted for 20% of graft creatinine change.     

Cough in children: when does it matter?

Cough is a common presenting symptom to paediatricians and paediatric respiratory physicians. The causes of chronic cough in childhood are significantly different from those in adults, and a different approach to diagnosis and management is needed. In most cases cause can be identified or a satisfactory label given. This review will cover the approach to cough in children and cover some specific causes of troublesome cough not discussed elsewhere in this mini-symposium.     

Developmental outcome of children with Robin sequence: How does the question arise?

Robin sequence (RS) is a heterogeneous congenital condition characterized by retrognathia, glossoptosis, upper airway obstruction, and very often, posterior U-shape cleft palate. Half the children with RS have an underlying syndrome, either identified (syndromic RS) or not (RS+). Long-term intellectual developmental outcome first depends on the underlying diagnosis and is often poor in syndromic cases. On the contrary, the rare studies that analysed the long-term developmental outcome of children with isolated RS who received effective treatment of their respiratory and feeding difficulties early in life, showed intellectual and academic results close to or within the normal range. Speech outcome in RS is often delayed with phonation disorders. Speech difficulties depend on intellectual level, hearing and velar function after palate repair. It affects most children with RS and deserves active monitoring and care.     

Wilms’ tumour in African children: Can an institutional approach improve outcome?

Background:

The poor outcome for patients with Wilms’ tumour (WT) in developing countries has been predicated on late presentation, poverty and low rate of chemotherapeutic access. This study aims to evaluate the effects of an institutionalised approach to improving outcome for patients managed in a tertiary hospital in Nigeria.

Materials and Methods:

Oncology records of children diagnosed with WT between 2009 and 2013 were analysed for therapy completion and other prognostic parameters. Ensuing data were then compared with those from other centres in Africa.

Results:

Compared with results from some local and African studies, the therapy completion rate was higher (60%) with a survival rate among this group being between 1 and 4 years. No patient was lost to follow-up because of unavailability or unaffordability of cytotoxic agents.

Conclusion:

This study shows that an institutionalised approach can help to improve access to anti-cancer drugs, reduce the rate of loss to follow-up and thus improve outcome. There is however need to improve on patient-doctor communication, form support groups and establish a WT registry.     

Congenital duodenal obstruction: does prenatal diagnosis improve the outcome?

Prenatal diagnosis of congenital duodenal obstruction (CDO) provides information about associated anomalies; helps plan the delivery, resuscitation, and neonatal surgery; and allows for appropriate family counseling. This report compares the outcomes of two groups of newborns: one with prenatal diagnosis of CDO (group I) and the other without (group II). Charts of the 23 newborns with CDO admitted to the Hospital of UNICAMP between 1993 and 2001 were retrospectively reviewed. Ten (44%) newborns had prenatal diagnosis of CDO. Among group I patients, the postnatal diagnosis was confirmed on the 1st day of life, whereas patients without prenatal diagnosis (group II) had the diagnosis of CDO confirmed at a mean age of 5.7 days (p=0.004). The mean ages at surgery, at total oral feeding, and at hospital discharge were also statistically lower among infants with prenatal diagnosis, and more complications occurred in group II patients. The earlier care could explain the statistically lower morbidity for patients with prenatal diagnosis, since they were able to undergo further investigation and surgical repair before any impairment to their clinical status could take place. We believe that prenatal diagnosis of CDO, associated with earlier surgery and adequate postoperative support, can provide lower morbidity, decrease the hospitalization period, and, therefore, decrease its costs to the state and to society.     

Anuria since birth: does it impact outcome of kidney transplant in infants?

Kidney transplantation (txp) in infants has recently made much progress but provides a unique challenge in infants anuric since birth. Little data exists on outcome of renal txp recipients with anuria since birth. Retrospective chart review was done for outcome of 27 children with wt ≤15 kg and they were divided into two groups: Group A (N=21) with urine output and Group B (N=6) anuric since birth had their urological complications and long‐term outcome compared. Median age at the time of txp 18 vs 23 months, mean wt 10.8 vs 11.8 kg, and mean ht 77 cm in both, mean follow‐up post‐txp: 9.4 vs 5.6 years, and neurological problems were noted in 48% and 33% in Group A and Group B. There was no graft thrombosis or post‐transplant lymphoproliferative disease and only two rejections. Anuric Group B were older, had more post‐txp urological surgeries (66% vs 19%) and UTIs (66% vs 38%) compared to Group A. The overall graft survival at 1, 5, and 10 years was 96%, 86%, and 70%; patient survival at 1, 5, and 10 years was 96%, 85%, and 85%. Long‐term graft outcomes in small children, anuric prior to txp, were excellent despite higher rates for UTIs and urological complications.     

Does diverting ileostomy improve the outcome in children with tuberculous small bowel obstruction requiring surgical intervention?

Introduction

Abdominal tuberculosis is fairly common in children. The most common clinical presentation is bowel obstruction. Depending upon the presentation, the intestinal obstruction can be either managed conservatively or by operative intervention. There are various options in patients who undergo operative treatment. This study was undertaken to analyze the results of operative intervention with and without ileostomy.

Materials and methods

This is a retrospective study carried out over a period of 10 years on 32 children who were operated for small bowel obstruction due to abdominal tuberculosis. The patients were divided into two groups (A: with ileostomy and B: without ileostomy). The relevant data and the defined outcome measures were statistically analyzed.

Results

A total of 32 children with tuberculous bowel obstruction requiring surgical intervention were studied. The patient of group A had mean duration of postoperative ileus for 2.55 days, restoration of enteral feeding within mean period of 3.55 days and had a primary hospital stay for a mean period of 9.0 days. These outcomes when compared with group B patients were statistically significant.

Conclusion

In children with bowel obstruction due to tuberculosis, diverting ileostomy decreases the morbidity by allowing early return of enteral motility, early institution of feeding and first-line ATT and decreasing the primary hospital stay.

     

Autonomic abnormalities in children with functional abdominal pain: coincidence or etiology?

BACKGROUND: There is increasing evidence that autonomic neuropathies may adversely affect gastrointestinal motility by involving the extrinsic nerves of the gut. The authors' hypothesize that functional abdominal pain in children is associated with generalized autonomic dysfunction. METHODS: The authors performed detailed autonomic testing in eight patients with functional abdominal pain, including deep breathing, Valsalva, tilting (to assess parasympathetic and sympathetic adrenergic function), and axon-reflex function and thermoregulatory sweat testing to assess sympathetic cholinergic function. Patients also completed a questionnaire regarding other autonomic symptoms. RESULTS: Results of autonomic testing were abnormal in seven patients. Parasympathetic function was normal in all, and the abnormalities were restricted to sympathetic cardiac, vasomotor, and sudomotor function. Abnormal results of axon-reflex testing in six were consistent with peripheral nervous system dysfunction. Five had decreased sweating over the abdomen, determined by thermoregulatory sweat testing. Five eight had nongastrointestinal autonomic symptoms, primarily palpitations and flushing. CONCLUSIONS: Functional abdominal pain in the current patients is associated with generalized dysfunction of the autonomic nervous system. This dysfunction can be peripheral or central in different individuals but seems to be restricted to the sympathetic branch. The known function of the sympathetic nervous system as the motility "brake" suggests that pain could be a manifestation of unmodulated peristalsis, resulting in abdominal cramps.     

Recurrent wheeze in children with Down syndrome: is it asthma?

Aim: To compare the prevalence of current wheeze in children with Down syndrome (DS), their siblings, and nonrelated population controls. Methods: This was a case–control study in which the International Study of Asthma and Allergy in Childhood questionnaire for respiratory symptoms was completed by parents for 130 children with DS, 167 of their siblings, and for 119 age‐ and sex‐matched control subjects from the general population. Results: Both wheeze ever and wheeze during the last 12 months was more commonly reported in DS than in their siblings or controls. The relative risk (RR) of current wheeze in DS was 2.8 (95% CI, 1.42–5.51) compared with siblings, and 2.75 (95% CI, 1.28–5.88) compared with controls. A doctor’s diagnosis of asthma was found in 3.1% in children with DS, in 4.2% in siblings and in 6.7% in controls. During 4‐years follow‐up, the diagnosis of asthma could not be confirmed in the 24 DS children with current wheeze, and atopy was found in none of them. Conclusion: Wheeze is common in children with DS. This is likely to be related to the factors specific for DS and probably unrelated to asthma.     

Does family functioning affect outcome in children with neurological disorders?

Children with neurological disorders are at increased risk of poor psychological and social adjustment. Medical factors are insufficient to explain the individual variation in outcome, so family variables have been considered. This review evaluates studies which have investigated the effect of family functioning on outcome in children with traumatic brain injuries, epilepsy and other neurological disorders. Methodological problems include the cross-sectional nature of many studies and inadequate control for confounding variables. There is good evidence that family functioning influences behavioural adjustment and adaptive functioning after traumatic brain injury. The role of family functioning in epilepsy and other neurological disorders requires further investigation. The development of effective interventions to improve family functioning may lead to important clinical benefits for children and their families.     

Long-term outcome in children with Guillain-Barré syndrome

OBJECTIVE: To determine the long-term sequelae and early predictors of sequelae for children with Guillain-Barré syndrome (GBS) after the introduction of intravenous gammaglobulin (IVIG).Study design A cross-sectional case series of children with GBS, at least 2 years after recovery, was performed. Manual muscle strength testing was done on 34 muscle groups. The functional independence measure was used to assess function. A chart review was completed, identifying acute factors that may predict long-term sequelae. RESULTS: Forty-seven children with GBS were identified, of whom 30 received IVIG. Persisting long-term muscle weakness with at least one muscle group at a grade 6 level (muscle moves the joint against gravity but only minimal resistance to an applied force) was found in 23% of cases. Each patient had a perfect score on the functional independence measure. Long-term muscle weakness was predicted by young age (P =.03) and a rapid progression to maximal weakness (P =.03). CONCLUSIONS: Despite the introduction of IVIG, 23% of children with GBS had evidence of long-term mild muscle weakness, with minimal impact on function. Young age and a rapid progression during the acute GBS period predicted long-term sequelae.     

Routine magnetic resonance imaging of the spine in children with Gaucher disease: does it help therapeutic management?

Background Routine magnetic resonance imaging (MRI) surveillance of bone marrow change in patients with Gaucher disease (GD) is recommended, but interpretation of imaging findings in the developing skeleton may be difficult.Objectives To assess the agreement between routine spinal MRI findings and clinical course in paediatric GD patients receiving enzyme replacement therapy (ERT).Materials and methods A retrospective institutional review of all GD patients on ERT who had had repeated routine spinal MRI in accordance with national recommendations (n=14) was carried out. Vertebral body bone marrow MRI T1 signal was assessed relative to intervertebral discs. MRI findings were then compared with recorded date(s) of ERT initiation and possible therapy changes.Results Nine patients had spinal MRI both before and after the start of ERT. MRI T1 marrow signal was normal in two and abnormal in three of the nine patients both before and after the start of ERT. Two of the nine patients had normalization and one had conversion from normal to abnormal T1 signal. Interpretation was uncertain in one. Seven episodes of treatment intensification occurred. MRI T1 marrow signal was normal before five and uncertain before two of the seven episodes.Conclusion Routine spinal MRI had low accuracy for predicting clinically indicated therapy intensification.     

Assessing bone health in children: who to test and what does it mean?

The foundation of bone health is established during childhood and adolescence. Unfortunately, pediatric bone health may be threatened in a variety of genetic and acquired disorders. Low bone mineral density (BMD) and/or fragility fractures may result. This paper addresses the pediatric indications for bone densitometry testing in children at risk for poor bone health and the challenges of interpreting the results. Dual energy x-ray absorptiometry (DXA) is the most commonly used of the bone densitometry methods. Most DXA software programs report BMD in terms of a T-score, which compares the results to adult norms. Z-scores, based upon age- and gender-matched reference data, should be used instead to avoid the mislabeling of a child as "osteoporotic." Delayed growth and maturation also complicate the interpretation of DXA findings in chronically ill patients. Although children with low BMD values may have an increased risk of fracture, a pediatric "fracture threshold" has not been established. Further research is needed to refine the indications for bone density testing in children and to aid in the interpreting the results.     

PET-CT in children: where is it appropriate?

The use of PET/PET-CT is a rapidly growing area of imaging and research in the care of children. Until recently, diagnostic imaging methods have provided either anatomical or functional assessment. The development of fused imaging modalities, such as PET-CT or PET-MRI, now provides the opportunity for simultaneously providing both anatomical and functional or physiological assessment. This review will discuss current established uses of PET-CT, possible uses and potential research investigations in the use of this modality in the pediatric population. The focus of this paper will be its use in children being treated for non-central nervous system and non-cardiac disorders.