Annals of Tropical Paediatrics最新文献

Harlequin ichthyosis (HI) is a very rare severe form of autosomal recessive congenital ichthyosis, usually associated with stillbirth and early neonatal death. A newborn girl with HI is described. She presented in a critical condition with severe universalis hyperkeratosis, diffuse scales and deep erythematous fissures. She received preventive systemic antibiotics and hygienic nursing with skin and eye care, feeding and appropriate hydration. She was discharged at 28 days in good general condition.

A 3.5-year-old child with influenza B virus pneumonia developed pneumomediastinum and subcutaneous emphysema on the 3rd day of illness. Bronchoscopy demonstrated obstruction of the left main bronchus by mucopurulent sputum. Culture of the broncho-alveolar lavage yielded Stenotrophomonas maltophilia. After the respiratory complications resolved (11 days), the patient developed neurological symptoms and was diagnosed as acute disseminated encephalomyelitis (ADEM). Stenotrophomonas maltophilia was probably a factor in the development of pneumomediastinum. To our knowledge, this is the first case report of influenza virus infection with Stenotrophomonas maltophilia co-infection associated with spontaneous pneumomediastinum.

Alternating hemiplegia of childhood (AHC) is a rare neurological disorder which usually presents before 18 months of age and is characterised by recurrent alternating episodes of hemiparesis. A single effective treatment for this condition is yet to be established; flunarizine is currently the most widely used but with varying degrees of success. An 18-month-old child presented with AHC and treatment with a combination of topiramate and flunarizine made a significant difference in controlling the frequency and severity of the attacks. This possibly allowed a better developmental outcome than in most children with this condition. Topiramate combined with flunarizine for treating AHC has much potential for further research.

This review focuses on how to reduce the high mortality of severe acute malnutrition (SAM) in African hospitals. The World Health Organization's 1999 manual for physicians (protocol) has not resulted in case-fatality rates of under 5%, even in published research studies from Africa, far less in district and central hospitals which do not record case-fatality rates. It is suggested that the following eight changes to the protocol need to be considered if we are serious about reducing case-fatality rates in African hospitals: (1) use of low lactose, low osmolality milk feeds during the early stage of treatment, especially for HIV-exposed infants and diarrhoeal cases; (2) more cautious use of high carbohydrate loads (ORS, ReSoMal, sucrose and 10% dextrose) during initial stabilisation; (3) more careful grading up and down of feed volumes according the child's responses during the early rehabilitation phase; (4) rapid rehydration of children in shock with Ringer's lactate, as for well-nourished children, with closer monitoring for heart failure; (5) greater use of 3rd-generation cephalosporin and fluoroquinolone antibiotics (e.g. ceftriaxone, ciprofloxacin) to treat sepsis owing to resistant organisms; (6) consider adding glutamine-arginine supplements as gut-protective agents in addition to zinc and vitamin A; (7) the addition of phosphate to existing potassium and magnesium supplements for those at risk of the refeeding syndrome; and (8) introduce better tools for diagnosis and clearer management of combined HIV and tuberculous infections in infants. Many will argue that these suggestions are unaffordable or impractical. On the contrary, cases of SAM requiring hospital admission need to be allocated more resources, including better nursing care, better diet and better medication. Resources made available for other childhood inpatient services such as ID and HIV dwarf those for severe malnutrition. Of course, prevention is always a better investment, including improving breastfeeding rates, improving complementary feeding practices and using ready-to-use therapeutic foods (RUTF) or similar supplements for those failing to thrive in the community, but SAM is unlikely to disappear from our hospitals, and these children need to be better managed if we are serious about reducing mortality.

Background: In patients with sickle cell anaemia (SCA), recurrent episodes of sequestration, micro-infarction, ischaemia and necrosis within the renal cortex cause nephron damage. Micro-albuminuria results from compensatory mechanisms aimed at preserving the glomerular filtration rate.

Aims: To establish the prevalence of micro-albuminuria among children with SCA and to describe associated factors.

Methods: A cross-sectional study of patients aged 2-18 years with SCA was undertaken at the sickle cell clinic, Mulago Hospital, Kampala between November 2007 and April 2008. Haemoglobin was measured and urine dipstick was used to determine protein, glucose, red blood cells and nitrites, serum creatinine and albumin and urine creatinine and albumin. Binary logistic regression was performed to determine factors associated with micro-albuminuria.

Results: Of 305 children studied, 48·2% were male. The mean (SD) age of the study participants was 9·7 (4·9) years. The prevalence of micro-albuminuria (30-300 μg/mg) was 28·2% (86/305, 95% CI 23·1-33·3). Use of diclofenac (p = 0·01) and ibuprofen (p = 0·001) were found to increase the risk of micro-albuminuria only by bivariate analysis. By multivariate analysis, increasing age (p = 0·001), a higher number of blood transfusions (p = 0·001) and presence of urine nitrites (p = 0·031) were associated with a risk of micro-albuminuria, whereas high levels of haemoglobin (p = 0·018) were protective. There was no association between estimated glomerular filtration rate and micro-albuminuria.

Conclusions: The prevalence of micro-albuminuria among children with SCA is relatively high. SCA patients over 5 years of age should be screened for micro-albuminuria. Those with lower haemoglobin levels should be monitored closely because of its association with micro-albuminuria.

Background: No studies have assessed the diagnostic accuracy of clinical signs of electrolyte disturbances in children with dehydrating diarrhoea.

Aims: To assess the diagnostic accuracy and reliability of clinical signs previously reported to be associated with plasma sodium and potassium disturbances in children.

Methods: A cross-sectional analytical study of 476 children aged 6 weeks to 2 years, admitted to a rehydration unit in Cape Town, South Africa. The clinical signs were elicited on admission by one of 58 junior doctors. Operational definitions of clinical signs were provided, but no additional training was given. Admission plasma electrolyte levels were the reference standard. Likelihood ratios were the primary measures of diagnostic accuracy, with reliability expressed as weighted Kappa scores.

Results: Inter-observer agreement was generally poor, and confidence intervals were wide. None of the 18 signs studied had clinically meaningful diagnostic accuracy even for severe plasma sodium and potassium abnormalities.

Conclusions: None of the clinical signs assessed were useful in clinical practice. Additional training would improve the accuracy of the signs.

Cardiac involvement is an uncommon complication of echinococcosis. It is usually asymptomatic and may only be diagnosed incidentally. A 7-year-old boy was admitted with acute stroke and bullous and ulcerated skin lesions. He was diagnosed with cardiac echinococcosis complicated by systemic emboli to the central nervous system and superficial cutaneous arteries. In endemic areas, echinococcosis should be considered in the differential diagnosis of cardiac disease and unexplained cerebral embolism.

Objectives: In young infants, early development of symptomatic HIV infection increases the risk of morbidity and mortality. A prospective study was conducted over a 1-year period in a region with a high burden of HIV in order to describe the clinical presentation of HIV infection in infants aged between 0 and 59 days on attendance at hospital and the factors associated with the need for urgent hospital management.

Methods: Sick young infants presenting to the King Edward VIII Hospital, Durban between February 2003 and January 2004 were enrolled. After systematic evaluation by a primary health worker, an experienced paediatrician determined the primary diagnosis and need for urgent hospital management. Comparisons of these assessments were stratified by HIV status. Children were classified as HIV-uninfected (HIV ELISA-negative), HIV-exposed-but-uninfected (HIV ELISA-positive and HIV RNA PCR-negative), HIV-infected (HIV ELISA-positive and HIV viral load >400 copies/ml).

Results: Of 925 infants enrolled, 652 (70·5%) had their HIV status determined: 70 (10·7%) were HIV-infected, 271 (41·6%) HIV-exposed-but-uninfected, and 311 (47·7%) HIV-uninfected. Factors associated with an increased probability of being HIV-infected included if the mother had children from more than one sexual partner, if the infant had had contact with a tuberculosis-infected person or if the HIV-infected mother and/or her exposed infant failed to receive nevirapine prophylaxis. Signs of severe illness were more frequently encountered in HIV-infected than in HIV-exposed-but-uninfected infants, including the prevalence of chest in-drawing (20·3% vs 8·8%, p = 0·004) and severe skin pustules (18·6% vs 8·6%, p = 0·01). Among infants requiring urgent hospital management, observed or reported feeding difficulties and severe skin pustules were more common in HIV-infected than uninfected infants. More HIV-infected infants (12·9%) required hospitalisation than those who were HIV-exposed-but-uninfected (7·7%) or uninfected (7·4%). Primary diagnoses of pneumonia, sepsis or oral thrush were more frequently seen in HIV-infected than exposed-but-uninfected or HIV-uninfected children.

Conclusion: Early recognition and triaging of infants suspected of having HIV infection provides an opportunity for early diagnosis and treatment which could prevent the adverse impact of rapidly progressive HIV disease.

Background: The causes of oedematous vs non-oedematous childhood malnutrition (OM vs NOM) remain elusive. It is possible that inherited differences in handling oxidant stressors are a contributing factor.

Aims: To test for associations between polymorphisms in five genes and (i) risk of OM, a case-control study, and (ii) percentage cytotoxicity in peripheral blood mononuclear cells (PBMCs) exposed to hydrogen peroxide (H(2)O(2)), an in vitro cell challenge study.

Methods: Participants had been admitted previously for treatment of OM (cases, n = 74) or NOM (controls, n = 50), or were an independent set of healthy pregnant women (n = 47) who donated peripheral blood mononuclear cells. We tested for associations between genetic variation and outcome using single markers or a bivariate score constructed by counting numbers of deleterious alleles for each of 15 possible pairs of markers.

Results: In the case-control study there were no significant single-marker associations with OM. We did find that higher bivariate scores were associated with OM for the pair of NAD(P)H:quinone oxidoreductase 1 and catalase (odds ratio 2·00, 95% CI 1·05-3·82). In the cell challenge experiments, there were no significant associations with percentage cytotoxicity.

Conclusions: Variation in this small set of genes seems unlikely to have a large impact on either risk of OM or cytotoxicity after H(2)O(2) exposure. The use of larger sample sizes to test the effects of a much larger set of genetic variants will be required in order to determine whether genetic variation contributes to the risk of OM. Such studies have potential for improving our understanding of causal pathways in OM.

Tuberculous cerebellar abscess is a rare manifestation of central nervous system tuberculosis. An 8-year-old boy is described who presented with acute hydrocephalus and right hemiparesis owing to a cerebellar abscess.