Queen Mary's Hospital For Children

OBJECTIVE: As the clinical implications of Helicobacter pylori infection in children and adolescents continue to evolve, ESPGHAN and NASPGHAN jointly renewed clinical guidelines using a standardized evidence-based approach to develop updated recommendations for children and adolescents in North America and Europe. METHODS: An international panel of 11 pediatric gastroenterologists, 2 epidemiologists, 1 microbiologist, and 1 pathologist was selected by societies that developed evidence-based guidelines based on the Delphi process with anonymous voting in a final face-to-face meeting. A systematic literature search was performed on 8 databases of relevance including publications from January 2000 to December 2009. After excluding nonrelevant publications, tables of evidence were constructed for different focus areas according to the Oxford classification. Statements and recommendations were formulated in the following areas: whom to test, how to test, whom to treat, and how to treat. Grades of evidence were assigned to each recommendation based on the GRADE system. RESULTS: A total of 2290 publications were identified, from which 738 were finally reviewed. A total of 21 recommendations were generated, and an algorithm was proposed by the joint committee providing evidence-based guidelines on the diagnostic workup and treatment of children with H pylori infection. CONCLUSIONS: These clinical practice guidelines represent updated, best-available evidence and are meant for children and adolescents living in Europe and North America, but they may not apply to those living on other continents, particularly in developing countries with a high H pylori infection rate and limited health care resources.

Intussusception is one of the commonest causes of intestinal obstruction in infants and accounts for about 700 hospital admissions each year in England and Wales. Improved results of treatment have followed recent technological developments, which include ultrasonographic imaging and pneumatic reduction techniques. Most intussusceptions can be reduced successfully without the need for operation but close cooperation between surgeon and radiologist is essential. Mortality and morbidity rates from the condition have progressively declined in recent decades but avoidable deaths still occur.

Abstract Cyclic vomiting syndrome (CVS) was initially described in children but can occur in all age groups. Cyclic vomiting syndrome is increasingly recognized in adults. However, the lack of awareness of CVS in adults has led to small numbers of diagnosed patients and a paucity of published data on the causes, diagnosis and management of CVS in adults. This article is a state‐of‐knowledge overview on CVS in adults and is intended to provide a framework for management and further investigations into CVS in adults.

The inherited bone marrow failure syndromes are a heterogeneous group of disorders characterized by bone marrow failure usually in association with one or more somatic abnormality. The bone marrow failure (which can involve all or a single cell lineage) often presents in childhood but may not do so

Excessive weight gain occurred in a patient who was taking sodium valproate and phenytoin. The sodium valproate was therefore withdrawn but the rapid weight loss that ensued led to phenytoin intoxication. Hence a retrospective analysis was conducted of 100 children with epilepsy treated with sodium valproate. Fit control improved in 77 and was best in children with generalised epilepsy. None of the reported severe side effects, such as acute liver disease and pancreatitis, were encountered. Milder but troublesome side effects, however, occurred in 65 patients. The commonest was increased weight gain, which occurred in 44 cases. Others were transient gastrointestinal disturbances (20), lassitude (nine), transient hair loss (six), transient enuresis (seven), and aggressive behaviour (four).

1MEDICAL JOURNAL infection, but only in situations of special risk, such as the thoracic unit described and where a sufficiently high cross-infection rate has been reported.

IGF-I generation tests were developed over 20 yr ago and are currently used in differentiating GH insensitivity (GHI) from other disorders characterized by low serum IGF-I. Nevertheless, generation tests have never been adequately characterized, and insufficient normative data are available. One hundred and ninety-eight subjects [including normal subjects; subjects with GHI, GH deficiency (GHD), and idiopathic short stature (ISS); and heterozygotes for the E180 splice GH receptor mutation] were randomized to self-administration of either a high (0.05 mg/kg x d) or a low (0.025 mg/kg x d) dose of GH for 7 d. After a 2-wk washout period, they received the alternate dose. Samples were collected on d 1, 5, and 8 of each treatment period. In normal individuals, IGF-I generation was GH dependent at all ages, and little advantage was observed in using the higher dose of GH or extending beyond the d 5 sample. Some GHD patients had IGF-I levels, both baseline and stimulated, that overlapped levels in the verified GHI patients. Subjects heterozygous for the E180 GH receptor splice mutation did not show a decreased responsiveness to GH. ISS patients had low-normal IGF-I levels that did not stimulate beyond the baseline normative ranges for age. These data provide the first large scale effort to provide preliminary normative IGF generation data and evaluate the GH sensitivity of patients with GHI, GHD, and ISS.

A total of 115 children under 5 years who presented with a first symptomatic urinary tract infection and who had a 99mTc dimercaptosuccinic acid (DMSA) scan were studied to assess its value and compare the findings with those of other imaging techniques. Renal cortical defects were detected in 65 kidneys by DMSA scan, intravenous urogram, and ultrasound scan combined; 62 (95%) being seen on DMSA scan. The finding of reflux on micturating cystourethrography showed a highly significant correlation with renal defects seen on DMSA scanning, a less close but still significant correlation with abnormalities on intravenous urography, but none with ultrasound scan findings. The sensitivity of the DMSA scan in screening for all grades of reflux is estimated as 0.66, which is higher than that previously reported for the intravenous urogram or ultrasound scan. DMSA scans were less likely to miss grade 3 reflux than the other two methods. DMSA scans are more useful than other upper renal tract imaging techniques in detecting renal defects. Consideration should be given to their use as a first investigation in place of routine intravenous urograms. Ultrasound scans alone will overlook potentially serious urinary tract abnormalities.

Journal Article THE CENTRAL NERVOUS SYSTEM IN DUCHENNE MUSCULAR DYSTROPHY Get access VICTOR DUBOWITZ, VICTOR DUBOWITZ Department of Child Health, University of Sheffield, and The Department of Pathology, Queen Mary's Hospital for ChildrenCarshalton, Surrey Search for other works by this author on: Oxford Academic PubMed Google Scholar LEONARD CROME LEONARD CROME Department of Child Health, University of Sheffield, and The Department of Pathology, Queen Mary's Hospital for ChildrenCarshalton, Surrey Search for other works by this author on: Oxford Academic PubMed Google Scholar Brain, Volume 92, Issue 4, March 1969, Pages 805–808, https://doi.org/10.1093/brain/92.4.805 Published: 01 March 1969 Article history Published: 01 March 1969 Received: 26 March 1969

BACKGROUND: Hyperinsulinism (HI) due to dysregulation of pancreatic beta-cell insulin secretion is the most common and most severe cause of persistent hypoglycemia in infants and children. In the 65 years since HI in children was first described, there has been a dramatic advancement in the diagnostic tools available, including new genetic techniques and novel radiologic imaging for focal HI; however, there have been almost no new therapeutic modalities since the development of diazoxide. SUMMARY: Recent advances in neonatal research and genetics have improved our understanding of the pathophysiology of both transient and persistent forms of neonatal hyperinsulinism. Rapid turnaround of genetic test results combined with advanced radiologic imaging can permit identification and localization of surgically-curable focal lesions in a large proportion of children with congenital forms of HI, but are only available in certain centers in "developed" countries. Diazoxide, the only drug currently approved for treating HI, was recently designated as an "essential medicine" by the World Health Organization but has been approved in only 16% of Latin American countries and remains unavailable in many under-developed areas of the world. Novel treatments for HI are emerging, but they await completion of safety and efficacy trials before being considered for clinical use. KEY MESSAGES: This international consensus statement on diagnosis and management of HI was developed in order to assist specialists, general pediatricians, and neonatologists in early recognition and treatment of HI with the ultimate aim of reducing the prevalence of brain injury caused by hypoglycemia. A previous statement on diagnosis and management of HI in Japan was published in 2017. The current document provides an updated guideline for management of infants and children with HI and includes potential accommodations for less-developed regions of the world where resources may be limited.

Micrococcaceae isolated from the shunt, ventricles, and bloodstream of children with colonized ventriculo-venous shunts were classified within the scheme of Baird-Parker (1963). With one exception, all belonged to subgroup II of the genus Staphylococcus; tests were therefore devised for division within this subgroup, and results are reported in 30 cases from this and other hospitals. Skin and nasal staphylococci isolated from many of these patients were compared with those recovered from their shunts and blood. Evidence is offered for the occasional coexistence of more than one strain of staphylococcus in colonized shunts and in the bloodstream. Successive recolonization of replaced shunts was apparently not necessarily caused by the same type of staphylococcus. Nasal and skin micrococcaceae from many other babies, both in hospital and in parental care, from hospital staff and from adults selected at random from non-hospital sources, were similarly classified. The validity and significance of the findings are discussed.

rPENBRITIN," A NEW PENICILLIN BDICJOURNAL Experiment 6.-For comparative purposes, 10 sub- jects received two 250-mg. capsules of tetracycline and a similar procedure was adopted as in the previous experiments except that no half-hour specimen was taken. The results are set out in Table Serum TABLE VII.-Serum Concentrations and Urinary Excretion of Tetracycline After a Single 500-mg. Dose Sub-Dose Serum Concentration (pg. 'ml.) Urine ject mg. l h 2 h mg. in mg I hr. 2 hr. 4 hr. 6 hr. 6 hr.

Abstract. The efficacy of intravenous polyclonal immunoglobulin (IVIG) as adjunct therapy in sepsis has long been debated. Clinical trials have yielded contradicting results, in part due to the varying study design and varying microbiological aetiologies. In most trials, the study drug has been IVIG containing polyclonal IgG. However, in recent reports, the efficacy of IgM‐enriched IVIG as adjunct therapy in sepsis has been highlighted. Here we review studies on IgM‐enriched IVIG therapy in sepsis and we discuss the clinical efficacy in relation to microbiological aetiology and severity of sepsis. The results suggest that patients most likely to benefit from IgM‐enriched IVIG therapy are those with Gram‐negative septic shock.

Gastrointestinal issues are a major chronic problem in 80 to 90% of children with cerebral palsy and in children with neurodevelopmental disabilities who are at special risk of developing malnutrition because of uncoordinated swallowing, gastroesophageal reflux, and constipation. In addition to poor linear growth, there is a decrease in muscle strength and coordination, impaired cerebral function leading to decreased motivation and energy. Significant neurodevelopmental progress can be achieved with improved nutritional status. A multidisciplinary approach, with input from neurologists, gastroenterologists, nurses, occupational therapists, and dieticians, can make a major contribution to the medical wellbeing and quality of life of these children. Different neurological diseases ( eg, spinal dysraphism, syringomyelia, tethered cord syndromes) can give rise to gastrointestinal dysfunction and symptoms that may need different gastrointestinal or surgical management. The introduction of new drugs, including proton pump inhibitors and innovative endoscopic and surgical techniques in the management of gastroesophageal reflux disease and constipation also may have an impact on the treatment of neurologically handicapped children in the future.

An epidemiologic study by questionnaire was undertaken in Great Britain and the United States, to provide data on diabetes mellitus in Down’s syndrome. Among 20,362 patients with Down’s syndrome, 88 living diabetics were found. A high prevalence of diabetes in the population with Down’s syndrome, particularly In the younger age groups, was noted. In these age groups the prevalence of diabetes in the population with Down’s syndrome exceeded that of the general population by factors 6.8, 3.3, and 3.0. Insufficient data exist to determine the exact statistical significance of these ratios. Further investigation is necessary. Nevertheless, a definitive association between Down’s Syndrome and diabetes mellitus is suggested. The role of prediabetes, autoimmunity and genetics in this association is discussed.

Laboratory studies on clotrimazole showed that it had marked activity in vitro against all the Candida spp. and Cryptococcus spp. tested, against almost all strains of dermatophytes, and against Aspergillus spp and other fungal genera responsible for systemic mycoses; it had limited activity towards Gram-positive bacteria. The majority of Candida strains required MICs below 1 mug/ml and MCCs below 2 mug/ml.Serum, urine, and faecal assays of clotrimazole were made by microbiological methods on five children who received 100 mg/kg/day clotrimazole for several weeks. In-vitro sensitivity tests and biological fluid drug assays are also reported on specimens from 18 patients in other hospitals receiving clotrimazole for severe candidosis; several were renal transplant cases. Similar investigations are reported on specimens from 18 patients with pulmonary aspergilloses. The significance of low levels of the drug in body fluids, even after prolonged therapy, is discussed, and it is suggested that clotrimazole may be the first of a long series of imidazole derivatives with varying pharmacological and therapeutic properties.

The procedures evolved in the author's laboratory over the past 20 years for the microbiological assessment of antifungal drugs are described; methods are detailed for the estimation of the sensitivity of pathogenic fungi to therapeutic agents and for the assay of those agents in body fluids. The preparation and maintenance of stock reference solutions of the drugs, the culture media used, and the incubation temperature and time are discussed. Sensitivity tests by paper disc and by liquid titration for minimal inhibitory and cidal concentrations estimated are described, and the importance of standardized initial inocula is emphasized. Two groups of assay procedures are given, the liquid dilution and the agar diffusion methods, and suitable indicator organisms for both methods are named. The paper concludes with a discussion on the problem of differential assays when two antimycotic agents are in simultaneous clinical use.

BACKGROUND: Wheeze is a common symptom in infancy and is a common cause for both primary care consultations and hospital admission. Beta2-adrenoceptor agonists (b2-agonists) are the most frequently used as bronchodilator but their efficacy is questionable. OBJECTIVES: To determine the effectiveness of b2-agonist for the treatment of infants with recurrent and persistent wheeze. SEARCH STRATEGY: Relevant trials were identified using the Cochrane Airways Group database (CENTRAL), Medline and Pubmed. The database search used the following terms: Wheeze or asthma and Infant or Child and Short acting beta-agonist or Salbutamol (variants), Albuterol, Terbutaline (variants), Orciprenaline, Fenoterol SELECTION CRITERIA: Randomised controlled trials comparing the effect of b2-agonist against placebo in children under 2 years of age who had had two or more previous episodes of wheeze, not related to another form of chronic lung disease. DATA COLLECTION AND ANALYSIS: Eight studies met the criteria for inclusion in this meta-analysis. The studies investigated patients in three settings: at home (3 studies), in hospital (2 studies) and in the pulmonary function laboratory (3 studies). The main outcome measure was change in respiratory rate except for community based studies where symptom scores were used. MAIN RESULTS: The studies were markedly heterogeneous and between study comparisons were limited. Improvement in respiratory rate, symptom score and oxygen saturation were noted in one study in the emergency department following two salbutamol nebulisers but this had no impact on hospital admission. There was a reduction in bronchial reactivity following salbutamol. There was no significant benefit from taking regular inhaled salbutamol on symptom scores recorded at home. REVIEWER'S CONCLUSIONS: There is no clear benefit of using b2-agonists in the management of recurrent wheeze in the first two years of life although there is conflicting evidence. At present, further studies should only be performed if the patient group can be clearly defined and there is a suitable outcome parameter capable of measuring a response.

Forty five children presenting with a first proven urinary tract infection under the age of 5 years were studied by sequential 99mTc dimercaptosuccinic acid (DMSA) scans. Forty nine kidneys in 40 children had definite defects at presentation, and 39 (80%) of these defects were still present when the DMSA scan was repeated. Changes in the appearance of defects were independent of the presence or degree of reflux at presentation and of symptomatic recurrence of infection, though the combination of new infection and grade 3 reflux (reflux reaching the renal calices with distension) was associated with deterioration. No kidney with a relative DMSA uptake of less than 35% showed any improvement in its cortical defects. Only two kidneys that were initially without defects, in a single patient who had bilateral grade 3 reflux and breakthrough infections, developed defects on subsequent scans. The outcome after urinary tract infection is dependent on the effect of the first infection on the kidney. Occasionally children with grade 3 reflux develop damage during subsequent infections. More widespread use of DMSA scans should improve our understanding of the factors that determine the development of renal damage.

Forty-five patients have been treated surgically for obstructive azoospermia. Fifteen underwent reversal of vasectomy and 40% of the wives became pregnant. Thirty had epididymovasostomy, and in only 2 (6.5%) did the sperm count become normal, although a few poorly motile sperms appeared in the ejaculate in a further 4 patients. Congenital abnormalities of the vasa in 7 cases and post-inflammatory blocks in 4 cases were examples of obstructive azoospermia due to well defined causes. However, in half of the patients (15 cases) the cause was obscure although it was associated with sinusitis, bronchitis or bronchiectasis (Young's syndrome). The results of pulmonary function tests in 30 cases, and electron microscopic studies of cilia from epididymes (10 cases) and bronchial mucosa (2 cases) indicated that the basic abnormality might be malfunction of the microtubules which appeared to be ultrastructurally normal in most cases. One case appeared to be associated with dietary deficiency, and correction of diet coincided with a successful result of surgery.