Jonathan C. Darling

Central Venous Catheter Tip in the Right Atrium: A Risk Factor for Neonatal Cardiac Tamponade

Fatal cardiac tamponade is a well recognised complication of the use of central venous catheters in neonatal patients. There is controversy over optimum catheter tip position to balance catheter performance against risk of adverse events. We report a series of five cases of tamponade occurring in one neonatal unit over a 4-year period, related to catheter tip placement in the right atrium. Right atrial catheter angulation, curvature or looping (CA) was present in all five cases on plain radiograph. It was infrequently seen in other patients over the same period. Review of the literature indicates that CA was present in 6 of the 11 previous cases where the presence or absence of CA can be determined. Where right atrial catheter tip placement is accepted, clinicians should be aware of this characteristic catheter configuration, which is a major risk factor for cardiac tamponade. We recommend that catheter tips should not be placed in the right atrium to avoid risk of tamponade.

Dietary management of acute diarrhoea in children: effect of fermented and amylase-digested weaning foods on intestinal permeability.

BACKGROUND There is a strong relationship between diarrhoea, malnutrition, and intestinal integrity. To investigate the effect of different dietary-treatment on intestinal permeability during acute diarrhoea, 87 Tanzanian children aged 6-25 months were recruited to this study when admitted to hospital. METHODS Children with acute diarrhoea were rehydrated and then randomly assigned to one of three dietary treatment groups: a conventional low-energy density porridge, a high-energy density amylase digested porridge (AMD), or a high-energy density amylase digested and then fermented porridge (FAD). Lactulose/mannitol permeability tests were performed on admission, at 3 days, and at follow-up 2 and 4 weeks after discharge. The lactulose/mannitol (L/M) ratios were compared between dietary treatment groups and to a group of age-matched, healthy control subjects. RESULTS Children with diarrhoea had higher L/M ratios (geometric mean 0.85, 95% CI 0.68-1.05) compared with control subjects (0.14, 0.12-0.17) on admission. There was a significant difference in the change in L/M ratio between admission and 3 days between dietary treatment groups in favour of the FAD group (p < 0.05). CONCLUSIONS Dietary treatment and intestinal damage at admission explain 13.5% of the variation in L/M ratio, but when age at admission and age at weaning are included as covariants, 21.9% is explained. FAD porridge seems to be more effective in the treatment of intestinal permeability than AMD or conventional porridge. Urinary lactose concentrations in spot urine samples taken prior to the permeability test were also measured. There was a significant correlation with the L/M ratio (correlation coefficient = 0.62, p < 0.001).

Cutaneous granulomas associated with primary immunodeficiency disorders

Cutaneous granulomas are uncommon in primary immunodeficiency disorders. We report cutaneous granulomas in a child with ataxia telangiectasia (AT) and compare the clinical course with similar lesions in an adult with common variable immunodeficiency (CVI). A 4‐year‐old female with AT developed cutaneous granulomas as erythematous plaques. The largest lesion appeared on her left cheek and continued to progress despite treatment with topical and intralesional steroids. Disease control was obtained initially with oral antibiotics and low‐dose oral steroids. On cessation of oral steroids, significant relapse of the facial granuloma occurred. Pulsed and then oral steroids were required to stop the disease process leaving significant scarring. The second case is of cutaneous granulomas in a 66‐year‐old man, with CVI, who presented with an erythematous reticulate rash on the legs. We consider it useful to report this patient here as disease control was obtained in a similar way with systemic immunosuppression. In this patient a combination of oral steroids and azathioprine was used. These cutaneous granulomas are thought to be a manifestation of immune dysregulation. No infectious cause has been found so far. We recommend the use of broad‐spectrum antibiotics in conjunction with systemic steroids for progressive granulomas, as these patients are immunosuppressed and infection with an unidentified organism cannot be excluded.

UK health professionals' attitudes and knowledge regarding Human Papillomavirus (HPV) vaccination: A West Yorkshire Study

Aim:  To investigate the willingness of clinicians to recommend human papillomavirus (HPV) vaccination, the strength of support for a national HPV vaccine programme and to determine which factors, if any, affected these.

Improved energy intakes using amylase-digested weaning foods in Tanzanian children with acute diarrhea.

Summary Amylase from germinating cereal grains enables the preparation of porridge with a higher energy density than conventional weaning foods. This food can be combined with fermentation, which inhibits pathogen growth. These food technologies are inexpensive, can be implemented at the household level, and are therefore particularly appropriate for use in developing countries. In a controlled clinical trial, 75 children aged 6–25 months admitted to hospital with acute diarrhea were rehydrated and then randomly allocated to three corn porridge dietary groups: conventional, amylase-digested (AMD), and fermented and amylase-digested (FAD). The study diets were given ad libitum five times daily, and all intakes except breast milk were weighed. Mean daily energy intakes over 4 days in the conventional AMD, and FAD groups, respectively, were 32.4 (95% CI 28.7–36.6), 46.0 (CI 39.6–53.4), and 37.3 (CI 31.8–43.9) kcal/kg/day. The energy intake in the AMD group was 42% higher than the conventional group (p = 0.003). There were no significant differences between the groups for duration of diarrhea, frequency of stooling, or vomiting. Starch digestion using amylase from germination is an effective way of improving energy intake in children with acute diarrhea.

A child with laryngo-onychocutaneous syndrome partially responsive to treatment with thalidomide

Laryngo‐onychocutaneous syndrome (LOCS) is a condition characterized by erosive or ulcerative skin lesions associated with excessive granulation tissue, at sites of trauma such as the digits, elbows and knees. Similar lesions can occur within the conjunctival mucosa, leading to corneal scarring and blindness. The main complications, however, occur in the respiratory tract, where a similar process of erosions and subsequent formation of granulation tissue causes airway obstruction which may lead to premature death. LOCS is now believed to be a nonblistering variant of junctional epidermolysis bullosa and to date there are no efficacious treatments available. We report a 16‐year‐old girl with LOCS who failed to respond to methylprednisolone and cyclophosphamide, but had a partial response to oral thalidomide with marked decrease in granulation tissue and tracheal secretions. Interruption of treatment resulted in prompt resurgence of the granulation tissue which was again controlled by reintroduction of thalidomide. We propose that in the absence of effective therapies for LOCS, a trial of thalidomide in these patients should be considered.

Placement of neonatal central venous catheter tips in the right atrium: a practice to be avoided?

Editor—Following the recent media interest in pericardial tamponade complicating the use of percutaneous central venous catheters in neonatal patients, we wish to alert readers to our experience. Our previous policy was to accept right atrial placement of percutaneous central venous catheter tips. This was in line with published recommendations1-3 and is still considered acceptable practice in some …

Primary school children in a large-scale OSCE: Recipe for disaster or formula for success?

Background: Many medical schools have moved to large end-of-year Objective Structured Clinical Examinations (OSCEs) in which it is difficult to involve children as patients. It is nevertheless important to assess student competencies in clinical examination of children. Methods: We set up a partnership with a local primary school, where children aged 8–11 years have assisted with our OSCE annually from 2007 to 2012. Approximately 30 children attend each exam, and are distributed between 14 simultaneous stations, each part of a 20-station circuit. Approximately 280 candidates complete the same paediatric station (e.g. cardiovascular examination) in one morning. Evaluation: A total of 160 children took part in the exams over this period, and of 129 (80.6%) who filled a questionnaire: 99.2% agreed that they ‘had enjoyed taking part in the exam’; 100% ‘thought it was a good experience’; and 96.1% ‘thought that it was well organised’. Parent and teacher feedback has been overwhelmingly positive. Conclusion: We conclude that it is feasible to involve school children in a large-scale OSCE. A school – medical school partnership is mutually beneficial, improving assessment of important paediatric clinical skills, while providing a positive experience for children who participate.

Laryngo-Onycho-Cutaneous Syndrome

Laryngo-onycho-cutaneous syndrome (LOC) is a rare autosomal recessive genodermatosis characterized by altered cry at birth, skin erosions, nail abnormalities, and excessive granulation tissue in the conjunctivae and larynx. Shabbir first described this condition in 1986 in several affected individuals born to consanguineous families from the Punjabi regions of India and Pakistan. In 2003, the molecular basis of LOC syndrome was elucidated with the discovery of a unique mutation affecting the N-terminus of the α3 chain of laminin-332. In 2008, LOC syndrome was reclassified as a variant of junctional epidermolysis bullosa-other (JEB-O) and termed JEB-LOC. In this chapter, we provide an overview of the clinical features, pathogenesis, and management of LOC syndrome.

Acute phase proteins as markers of systemic illness in acute diarrhoea

Fifty‐seven Tanzanian children, 6–25 months, hospitalized with acute diarrhoea were grouped according to whether there was clinical evidence of systemic infection (SI) (n= 35) or not (n= 22). Serum acute phase proteins were measured in samples taken within 48 h of admission. Means for C‐reactive protein (CRP) and serum amyloid A (SAA) were significantly higher in children with SI compared to those without (geometric means (95% CI); CRP, mg/l: 22.1 (13.6‐35.5) vs 7.4 (4.4‐12.4); SAA, mg/l: 12.2 (6.8‐22.1) vs 4.9 (2.5‐9.7)). Levels of o1‐acid glycoprotein were similar in both groups (1.16 g/l (0.95‐1.43) vs 1.04 (0.83‐1.29), respectively). CRP ≥ 30 mg/l had a positive predictive value of 95%, and specificity of 96% for correctly identifying SI, but a low sensitivity (51%) and negative predictive value (55%). Clinical outcome of diarrhoea was worse in children with SI: more needed intravenous fluids (23% vs 5%), the duration of diarrhoea was longer (59.4 vs 34.2 h) and mortality was higher (6% vs 0%). APPs were not found to be useful markers of systemic illness in acute diarrhoea in this population.