The management of acute hyperkalaemia in neonates and children

This review article describes the pathophysiology and common aetiologies of hyperkalaemia including pseudohyperkalaemia, renal impairment, medication, rhabdomyolysis and aldosterone deficiency. Two clinical cases are used to describe symptoms (mainly muscle weakness and arrhythmias) and illustrate different management options. An approach to management including relevant investigations and interpretation of ECG changes is described. Emergency drug treatments are outlined and the effectiveness of individual therapeutic methods in reducing the potassium concentration described. Chronic management is mentioned but is outside the scope of this article. Hyperkalaemia is a rare but potentially life threatening emergency. It is a manifestation of a disease and therefore the incidence in children is not known. Quick and effective intervention may be necessary and clinicians must be adept at managing this condition. This overview provides two clinical scenarios and summarises aetiologies, investigations and management.     

Management of acute renal failure in the newborn

Acute renal failure is common in the neonatal period. It is usually manifest by abnormal biochemistry and decreased urine output (<1 ml/kg/h), but non-oliguric renal failure is also common. A detailed understanding of the common pathophysiological mechanisms is rarely needed but an understanding of the common aetiologies (pre-renal, renal and post-renal) will enable the clinician to approach the problem in a logical manner. A standard approach to fluid and electrolyte management is described, along with a practical approach to the investigation and management of renal failure. A working understanding of the principles of peritoneal dialysis is important and a brief overview of the role of haemodialysis in neonatal renal failure is provided.     

A structured approach to the child with precocious puberty

Precocious puberty is the appearance of physical and hormonal features of puberty before 8 years (girls) or 9 years (boys). Untreated it limits adult height and has potential psychosocial implications. It is a fairly common referral to secondary care clinics and is often seen by general paediatricians in the first instance in the UK. As an outpatient presentation it can cause anxiety owing to the breadth of potential aetiologies and concerns about missing a rare sinister cause. This short article offers a structured approach to precocious puberty to help identify any sinister causes and determine what steps you should take next.     

Paediatric acute liver failure: a practical approach

Acute liver failure (ALF) in the paediatric patient is a multisystem complex disorder, which occurs in the absence of chronic liver disease. Globally, viruses remain a common cause but drugs, metabolic and autoimmune conditions are important triggers. In up to half of cases no specific cause is identified. The definition entails a coagulopathy with a Prothrombin time (PT) ≥ 15seconds or International Normalized Ratio (INR) ≥ 1.5 not corrected by vitamin K in the presence of hepatic encephalopathy (HE) or a PT ≥ 20 or INR ≥2 or above regardless of HE. HE can be difficult to recognize in children and is defined differently than HE in adults. Timely recognition of ALF improves outcomes and allows time to undertake investigations, provide supportive therapy and arrange transfer to a specialist paediatric liver centre with transplant capacity. The purpose of this article is to review the aetiologies of ALF in children and outline an approach to investigation, management and treatment.     

Ascites and encephalopathy in chronic liver disease

Ascites and particularly encephalopathy in the setting of chronic liver disease are traditionally thought to be poor prognostic markers of end stage liver disease. Diagnostic and therapeutic approaches to these complications are based on experience with adult patients, however, differences in the spectrum of aetiologies causing end stage liver disease together with the relative resistance of the pediatric patient to encephalopathy impact on the management of such patients. The following is a review of current thinking in the aetiology and management of ascites and encephalopathy in the setting of chronic liver disease in children.     

Investigation of chronic diarrhoea in infancy

Diarrhoea in infants and young children is defined as > 200 g/day of stools, and occurs when there is an imbalance between intestinal fluids absorption and secretion. This may be caused by either a decreased absorption (osmotic diarrhoea) or an increased secretion (secretory diarrhoea). Chronic diarrhoea defines intestinal loss of water and electrolytes with increased stool frequency, reduced consistency and larger volume over more than 14 days. This disorder in children shows a wide range of aetiologies depending on the age. The knowledge of common and rare aetiologies is important to optimize the diagnostic approach. A stepwise approach, starting with a comprehensive history, physical examination, inspection and collection of stool samples, helps to devise appropriate diagnostic and therapeutic management. In this article we discuss the pathophysiology, aetiology and possible approach to chronic diarrhoea in infancy.     

Non-organic failure to thrive complicated by benign intracranial hypertension during catch-up growth

Severe non-organic failure to thrive associated with physical and emotional abuse including food deprivation was diagnosed in a 9-y-old boy. Rapid catch-up growth (weight and height) followed change of carer. Recovery of poor growth hormone response to clonidine stimulation was associated with benign intracranial hypertension accompanied by headaches and vomiting. Possible mechanisms are discussed.     

Clicks,Whoops, and Honks

5 children are described with the midsystolic click—systolic murmur syndrome, sometimes accompanied by honks or whoops. The characteristic variation in auscultatory findings with posture is shown. The theory as to the mechanism of production of these findings is described, together with their aetiologies, prognosis, and treatment.     

Demystifying neurological investigations: a practical guide for the child with seizures

The epilepsies of childhood are a markedly heterogeneous group of diseases with different presentations, outcomes and aetiologies. There is no single, gold-standard investigation that will unequivocally diagnose an epilepsy. The range of aetiologies includes genetic, structural brain malformations and metabolic; in many, there may be a combination of causes. Despite major advances in neuro-imaging and genetics, a cause will not be identified in approximately 40% of all children with epilepsies. This article outlines the available investigations and provides a practical and structured approach on their most effective use in the childhood epilepsies.     

Cardiomyopathy in newborns and infants: a broad spectrum of aetiologies and poor prognosis

Aim: This study set out to describe the initial clinical findings, morbidity, mortality and aetiology of infant cardiomyopathy focusing on potential risk factors for an adverse outcome. Methods: We retrospectively analysed clinical and laboratory findings of all patients diagnosed at our institution from 1995 to 2004 with cardiomyopathy within their first year of life. Results: Of the 35 patients, cardiomyopathy was classified as dilated in 18, hypertrophic in 14 and unclassified in 3. The aetiologies were genetic syndromes (8), metabolic diseases (5), familial isolated cardiomyopathy (3) and myopathy (1). During a median follow‐up of 1.5 years (range 0–9 years), 13 patients died from progressive heart failure and two underwent heart transplants. Estimated survival and freedom from transplant was 69, 66, 58 and 50% after 0.5, 1, 2 and 6 years, respectively. Patients with severe heart failure symptoms within the first month of life had significantly worse outcomes than patients without heart failure symptoms. Conclusion: High morbidity and poor prognosis result through progressive heart failure. Aetiology and clinical course are especially heterogeneous in infants. The most commonly identified aetiologies are genetic syndromes and metabolic diseases. A multidisciplinary approach is recommended for defining the aetiology and developing individual treatment strategies.     

Improving the management of atopic disease.

Asthma, wheeze, eczema, and, to a certain extent, rhinitis are very common conditions among children. The prevalence of allergic disease in the general population has increased alarmingly over the past 25 years, particularly in Western industrialised countries. However, it is important to remember that the symptoms often associated with allergy can have other aetiologies. Evidence suggests that in most circumstances, only 30-40% of chronic allergic-type symptoms are due to allergy. Accurate diagnosis of the presence of allergy is therefore an important issue, particularly given the interventions that such a diagnosis may initiate. In this review, we examine management options for allergy, provide the evidence as to what proportions of patients with common allergic-type symptoms are actually allergic, and list other causes of such symptoms. The importance of allergy testing and the options available are described, particularly with reference to the role of the non-allergist.     

Developmental Therapy in the Neonatal Intensive Care Unit

The skill and knowledge requirements for physical therapists and occupational therapists practicing in the neonatal intensive care unit (NICU) setting are discussed as a highly specialized, advanced level of pediatric practice. The characteristics of the NICU population at risk for later developmental problems and the potential effects of environment are examined. The design of this program's physical/occupational therapy team approach is presented, including areas of overlap and role differences. Criteria for developmental therapy, general program goals and evaluation procedures are outlined. Treatment considerations and selected intervention strategies, using a Neurodevelopmental Treatment (NDT) approach, are described in the areas of handling, positioning, sensory stimulation, oral feeding techniques and intervention with parents.     

Improving the management of atopic disease

Asthma, wheeze, eczema, and, to a certain extent, rhinitis are very common conditions among children. The prevalence of allergic disease in the general population has increased alarmingly over the past 25 years, particularly in Western industrialised countries. However, it is important to remember that the symptoms often associated with allergy can have other aetiologies. Evidence suggests that in most circumstances, only 30-40% of chronic allergic-type symptoms are due to allergy. Accurate diagnosis of the presence of allergy is therefore an important issue, particularly given the interventions that such a diagnosis may initiate. In this review, we examine management options for allergy, provide the evidence as to what proportions of patients with common allergic-type symptoms are actually allergic, and list other causes of such symptoms. The importance of allergy testing and the options available are described, particularly with reference to the role of the non-allergist.     

Update on treatment of childhood bronchiectasis unrelated to cystic-fibrosis

There are multiple aetiologies for childhood bronchiectasis unrelated to cystic fibrosis. Some of these aetiologies, such as those predisposing to recurrent lung infections, e.g. immunodeficiencies, require treatment of the underlying condition and disease-specific pulmonary pathogens within the airway. Regardless of aetiology, the treatments for bronchiectasis include antibiotics, airway clearance regimens, immunizations to prevent infections, and in some cases asthma therapies. The grade of evidence for specific treatments is low with few randomized controlled trials in children. Extrapolations of care provided to adults with bronchiectasis and patients with cystic fibrosis may not always be justified. Comprehensive care programs for children with bronchiectasis have demonstrated clinically relevant improvements over 2-7 year periods. Multi-center research is needed to rigorously evaluate current treatment practices for children with this disorder.     

The rule of four: a systematic approach to diagnosis of common pediatric hematologic and oncologic disorders

The “Rule of Four” facilitates a rapid and focused approach to the diagnosis of the common hematologic and oncologic disorders encountered in general pediatric practice. This system relies on four recurrent but different clinical entities or laboratory tests relevant to the diagnosis of children with anemia, excessive bleeding or clotting, and common malignancies. For each disorder, there is a discussion of a variety of four lab tests or factors pertinent to a differential diagnosis.     

Bilateral acromial fractures in a neonate with epileptic encephalopathy

We present a case of a newborn male with seizures who on subsequent radiographs was shown to have bilateral acromial fractures. These are uncommon fractures and are described in association with nonaccidental injury (NAI). However, in this case NAI was thought unlikely due to continuous hospitalisation of the boy. The possible aetiologies are discussed.     

Cardiomyopathy in children in Ahmedabad

A clinical study and follow up of 20 children with cardiomyopathies upto age of 16 years are presented. The DCM was most common variety followed by RCM and HCM in pediatric age group. SHMD presenting with cardiomyopathy were common in infancy and early childhood. Cardiomyopathies presented most frequently between 2–5 years and 10–16 years age group with DCM having almost equal distribution. Clinical presentation of various types is described, despite of vigorous decongestive and vasodilator treatment in advanced cases, course was rapidly downhill and prognosis is poor in general.     

Adolescents and contraception. What should the pediatrician know? (Part one)]

Many adolescents are sexually active and reproductive health is an important aspect of adolescent medicine. However, pediatricians are often uncomfortable with the issues of sexuality and contraception, for which they have not been particularly trained. The general purpose of this article is to increase pediatricians' sense of competence with adolescents, particularly when having to deal with or counsel on such a sensitive issue as contraception. This first of two parts is an updated review of the relevant contraceptive methods available, mainly condoms and various contraceptive pills, but also less-prescribed method like long-term progestins, as well as the recently debated emergency contraception. Each of these methods is described in its context of use at adolescence.     

Paediatric bronchiectasis in the twenty-first century: experience of a tertiary children's hospital in New Zealand

OBJECTIVE: Despite its decline in developed countries, bronchiectasis appeared to be a common diagnosis in Auckland, New Zealand children. The aims of this study were: to document the number of children in Auckland with bronchiectasis, their severity, clinical characteristics and possible aetiologies; to assess whether there was a relationship between ethnicity and poverty; and to estimate a crude bronchiectasis prevalence rate for New Zealand. METHODS: A retrospective review of the case histories of all children attending a tertiary children's hospital in Auckland with bronchiectasis diagnosed by high-resolution chest computed tomography (CT) scan, during the period 1998-2000 was undertaken. Data collected included patient demographics, number of hospitalizations pre- and post-diagnosis, lung function tests, radiology and investigations. The New Zealand deprivation 1996 index was applied to the data to obtain a measure of socio-economic status. RESULTS: Bronchiectasis was found to be common, with an estimated prevalence of approximately one in 6000 in the Auckland paediatric population. It was disproportionately more common in the Pacific Island and Maori children. In Pacific Island children, bronchiectasis not caused by cystic fibrosis was nearly twice as common in the general population than cystic fibrosis. Socio-economic deprivation and low immunization rates may be significant contributing factors. The bronchiectasis seen was extensive. Ninety-three percent had bilateral disease and 64% had involvement of four or more lobes on chest CT scan. A wide range of comorbidities and underlying aetiologies were evident. CONCLUSIONS: Paediatric bronchiectasis in Auckland, New Zealand, is common but underresourced. Only the most severe cases are being recognized, providing a significant challenge for paediatric health professionals.     

The Use of Diagnostic Systems for Tuberculosis in Children

Effective management of tuberculosis (TB) in children and important data of disease burden continue to rely on a clinical approach to diagnosis, as diagnosis of childhood TB is not confirmed in the majority. Many diagnostic scoring systems have been developed to aid with diagnosis. This article reviews the use and evaluation of these approaches. The diagnostic systems are often closely related and all rely on the well-known clinical features associated with TB disease in children. The scoring systems are not well validated and validation is limited by the lack of a gold standard for comparison. When they have been validated, some systems perform reasonably well but may bias to identify the most obvious clinical cases. They perform less well in important sub-groups that pose the greatest diagnostic challenge and are at greatest risk for poor outcome, such as the young, malnourished or HIV-infected. There is marked variation in performance between these diagnostic approaches. The better validated systems may have a role as a screening tool in some settings, but this would need careful consideration as to the most useful and safest approach. More attention is being given to improving diagnosis and management of child TB, including within National TB Programmes. Research with new diagnostics should include children so that there is less reliance on clinical features alone. However, the clinical approach will continue to be relevant and so it is important to strive to improve the diagnostic approach to TB in children, and to validate the approach in different settings.