Expanding the clinical spectrum in trichohepatoenteric syndrome.

Trichohepatoenteric syndrome (THES) is a very rare autosomal recessive genetic disorder, which is characterized by intractable diarrhea during infancy, dysmorphic features, immunodeficiency, and a failure to thrive. There are still significant difficulties for patients and clinicians in terms of the management of THES, even though its molecular basis has been uncovered in the last decade. In this article, we have presented two cases relating to siblings that have been diagnosed with the condition. Concerning one of the patients, we described a novel variation (c.2114 + 5G > A) in the TTC37 gene and a mild clinical course; meanwhile, the other one was clinically diagnosed with THES at 17 years of age, but they had seizures and died suddenly. These cases expand the spectrum of clinical findings in relation to THES.

Determinants of linear growth faltering among children with moderate-to-severe diarrhea in the Global Enteric Multicenter Study.

BACKGROUND: Moderate-to-severe diarrhea (MSD) in the first 2 years of life can impair linear growth. We sought to determine risk factors for linear growth faltering and to build a clinical prediction tool to identify children most likely to experience growth faltering following an episode of MSD. METHODS: Using data from the Global Enteric Multicenter Study of children 0-23 months old presenting with MSD in Africa and Asia, we performed log-binomial regression to determine clinical and sociodemographic factors associated with severe linear growth faltering (loss of ≥ 0.5 length-for-age z-score [LAZ]). Linear regression was used to estimate associations with ΔLAZ. A clinical prediction tool was developed using backward elimination of potential variables, and Akaike Information Criterion to select the best fit model. RESULTS: Of the 5902 included children, mean age was 10 months and 43.2% were female. Over the 50-90-day follow-up period, 24.2% of children had severe linear growth faltering and the mean ΔLAZ over follow-up was - 0.17 (standard deviation [SD] 0.54). After adjustment for age, baseline LAZ, and site, several factors were associated with decline in LAZ: young age, acute malnutrition, hospitalization at presentation, non-dysenteric diarrhea, unimproved sanitation, lower wealth, fever, co-morbidity, or an IMCI danger sign. Compared to children 12-23 months old, those 0-6 months were more likely to experience severe linear growth faltering (adjusted prevalence ratio [aPR] 1.97 [95% CI 1.70, 2.28]), as were children 6-12 months of age (aPR 1.72 [95% CI 1.51, 1.95]). A prediction model that included age, wasting, stunting, presentation with fever, and presentation with an IMCI danger sign had an area under the ROC (AUC) of 0.67 (95% CI 0.64, 0.69). Risk scores ranged from 0 to 37, and a cut-off of 21 maximized sensitivity (60.7%) and specificity (63.5%). CONCLUSION: Younger age, acute malnutrition, MSD severity, and sociodemographic factors were associated with short-term linear growth deterioration following MSD. Data routinely obtained at MSD may be useful to predict children at risk for growth deterioration who would benefit from interventions.

Rotavirus A Infections in Community Childhood Diarrhea in the Brazilian Semiarid Region During Postvaccination Era.

BACKGROUND: Rotavirus A (RVA) is one of the leading causes of acute gastroenteritis worldwide; however, few studies assessed RVA genetics with community surveillance. OBJECTIVES: This study aimed to investigate clinical data, genetic diversity, and coinfection patterns of RVA infections in children from 2 to 36 months old with or without community childhood diarrhea in the Brazilian semiarid region during postvaccination era. METHODS: We enrolled and collected socioeconomic/clinical information using a standardized questionnaire and fecal samples from 291 children. Viral RNA samples were extracted and analyzed using quantitative reverse transcription polymerase chain reaction to establish the diagnosis of RVA. Sequencing of VP7 and VP4 (VP8*) regions and phylogenetic analysis were performed. RESULTS: RVA-negative diagnosis was associated with children 24 to 36 months old with complete vaccination schedule. Genotype G1P[8] was the most prevalent (57%), whereas unusual genotypes including G1P[4], G2P[8], and G3P[9] were also detected. G1- and P[8]-positive samples showed high degrees of similarity with the vaccine strain. RVA coinfections were frequently observed, and enteroaggregative Escherichia coli was the most prevalent copathogen. CONCLUSIONS: These results demonstrate that genotype G1P[8] is the most prevalent strain. VP7 and/or VP8* gene segments arising from RV1 vaccine strain were documented in these children, suggesting shedding or herd vaccination. Moreover, our study indicates full vaccination is important for protection against RVA infections.

[Influential factors of iron deficient anemia among infants aged 8 months based on a case-control study].

OBJECTIVE: To study the status and influential factors of iron deficient anemia (IDA) among infants aged 8 months in Changsha City.
 Methods: A case-control study was performed in this research. The case group including 105 8-month-old infants definitely diagnosed as IDA based on standardized blood test. Four-times numbers of age- and genger-matched infants without IDA were selected as a control group. Chi-square test and conditional logistic regression was used to analyze the influential factors for IDA.
 Results: The incidence rate of IDA among infants aged 8 months in Changsha City was 14.7%. The risk factors were as follows: mother with anemia in late pregnancy (OR=3.540, 95% CI 1.898 to 6.601), mixed feeding within 6 months old (OR=1.682, 95% CI 1.099 to 2.574), artificial feeding within 6 months old (OR=4.162, 95% CI 1.343 to 12.896), complementary feeding before 6 months old (OR=1.423, 95% CI 1.022 to 1.982), complementary feeding at or after 7 months old (OR=4.415, 95% CI 2.150 to 9.064), recurrent respiratory tract infections within 8 months old (OR=2.878, 95% CI 1.224 to 6.764), and repeated diarrhea within 8 months old (OR=3.710, 95% CI 1.533 to 8.980).
 Conclusion: There is certain incidence rate of IDA among infants aged 8 months in Changsha City. To prevent the IDA among infants, we should treat mothers' anemia during pregnancy, advocate scientific feeding, encourage complete breastfeeding until 6 months old, add complementary food timely and reasonably, treat infants suffering from respiratory or digestive diseases actively.

Diarrhea in early childhood: short-term association with weight and long-term association with length.

The short-term association between diarrhea and weight is well-accepted, but the long-term association between diarrhea and growth is less clear. Using data from 7 cohort studies (Peru, 1985-1987; Peru, 1989-1991; Peru, 1995-1998; Brazil, 1989-1998; Guinea-Bissau, 1987-1990; Guinea-Bissau, 1996-1997; and Bangladesh, 1993-1996), we evaluated the lagged relationship between diarrhea and growth in the first 2 years of life. Our analysis included 1,007 children with 597,638 child-days of diarrhea surveillance and 15,629 anthropometric measurements. We calculated the associations between varying diarrhea burdens during lagged 30-day periods and length at 24 months of age. The cumulative association between the average diarrhea burden and length at age 24 months was -0.38 cm (95% confidence interval: -0.59, -0.17). Diarrhea during the 30 days prior to anthropometric measurement was consistently associated with lower weight at most ages, but there was little indication of a short-term association with length. Diarrhea was associated with a small but measurable decrease in linear growth over the long term. These findings support a focus on prevention of diarrhea as part of an overall public health strategy for improving child health and nutrition; however, more research is needed to explore catch-up growth and potential confounders.

Perceptions regarding oral rehydration solutions for the management of diarrhea in Guatemalan children: implications for diarrheal management in the Americas.

OBJECTIVE: To describe knowledge and perceptions regarding the use of oral rehydration solution (ORS) for the management of diarrheal diseases among formal and informal health care providers and community caregivers in the Guatemalan department of Santa Rosa, and to recommend strategies to increase ORS use for management of diarrhea in children. METHODS: From July to September 2008, in-depth, semi-structured interviews were conducted with formal health care providers; open-ended interviews were conducted with informal health care providers; and focus group discussions and pile sorting were carried out with community caregivers. RESULT: The study participants attributed episodes of diarrhea in children to culturally recognized or folk ailments (empacho, cuajo, and varillas) that are primarily treated by traditional healers. There were knowledge deficits about 1) dehydration as a manifestation of diarrhea, and 2) management of dehydration, including the use of ORS and the need to continue feedings during diarrheal episodes. Caregivers perceived bottled/ready-made ORS products and the more expensive over-the-counter antidiarrheal medications as superior to ORS packets in the treatment of diarrhea. CONCLUSIONS: In Guatemala, folk etiologies of disease differ from those of the biomedical establishment and influence the decisions made by caregivers when treating ill children, including those related to the use of ORS. Public health campaigns addressing the treatment and management of diarrheal diseases in Santa Rosa should recognize the ailments known as empacho, cuajo, and varillas and target them for ORS use by community caregivers as well as health care providers in both the formal and informal health sectors.

An unusual cause of secondary capillary leak syndrome in a child: rotavirus diarrhea.

Secondary capillary leak syndrome is characterized by loss of fluid and proteins to the interstitial space due to different causes, which are related to endothelial damage. Rotavirus is the most common pathogen of diarrhea in childhood, especially during the first years of life. This virus is generally responsible for severe diarrhea and electrolyte imbalance in children. Some complications can also occur during the course of the rotavirus diarrhea. An eight-month-old girl with rotavirus diarrhea admitted to our clinic with severe dehydration. After restoring the intravascular volume, hypoalbuminemia and generalized edema were seen in the recruitment phase of the treatment, which was attributed to secondary capillary leak syndrome. She was successfully treated with prednisolone and discharged from the hospital without any sequelae. Herein, we report an infant with rotavirus diarrhea complicated with secondary capillary leak syndrome, which is an unidentified complication of the disease. To our knowledge, this is the first such case in the literature.

Anaemia among Egyptian Children between 2000 and 2005: trends and predictors.

Anaemia remains the most prevalent nutritional disorder among women and children in the Middle East and North Africa region. We examined anaemia trends using data from the Egyptian Demographic and Health Surveys. Between 2000 and 2005, the prevalence of anaemia (defined as haemoglobin concentrations <11 g dL(-1) ) increased from 37.04% to over 52% among Egyptian children between 12 months and 36 months of age. We examined the associations of these changes with food consumption, vitamin A administration, recent illness, immunization status, socio-demographic factors and a child's anaemic status. Children under the age of 24 months who had recently been sick and those who resided in Upper Egypt were significantly more likely to be anaemic. Despite significant improvements in water and sanitation facilities, maternal education and asset-based household wealth, there were marked declines in the consumption of nutritive foods and increases in the prevalence of childhood diarrhoea between 2000 and 2005. Placing these analyses in the broader context of Egyptian economic trends suggests that the nutritional basket consumed by Egyptian households between 2000 and 2005 may have shifted towards less nutritive foods with lower costs per calorie, probably in response to economic difficulties and increasing food prices. Shifts in dietary consumption, in conjunction with increases in diarrhoea, are likely contributing to the rapid increase in childhood anaemia in Egypt between 2000 and 2005. National-level fortification efforts may be one way to combat rising levels of anaemia among Egyptian women and children.

Randomised controlled clinical trial of Lactobacillus sporogenes (Bacillus coagulans), used as probiotic in clinical practice, on acute watery diarrhoea in children.

OBJECTIVE: To assess the clinical efficacy of Lactobacillus sporogenes (Bacillus coagulans), as probiotic preparation, against dehydrating diarrhoea in children. METHODS: Double-blind, randomised, placebo-controlled, hospital-based clinical trial with children aged 6-24 months who had diarrhoea with some dehydration. Children received tablets of L. sporogenes (B. coagulans) or placebo (control group) and oral rehydration salt solution for correction of initial dehydration as well as maintenance therapy. Duration, frequency, volume of diarrhoea and intake of ORS of two groups were compared as outcome variables. RESULTS: One hundred and forty-eight children participated, of whom 78 (Study group) received L. sporogenes (B. coagulans) and 70 received placebo (Control group). Differences in recovery rate (P=0.2), duration (P=0.5), frequency (P=0.05), volume (P=0.1) of diarrhoea, intake of ORS (P=0.2) and other fluids (P=0.1) were not significant between both groups. Neither did a subgroup analysis of children who had rotavirus as sole enteropathogens show any significant differences in duration (P=0.5), frequency (P=0.6), volume (P=0.8) of diarrhoea, intake of ORS (P=0.8) and other fluids (P=0.8) among both groups. CONCLUSION: L. sporogenes (B. coagulans), as an adjunct to ORS, had no therapeutic impact on management of acute dehydrating diarrhoea of diverse etiology including rotavirus associated diarrhoea in children.

The duration of diarrhea and fever is associated with growth faltering in rural Malawian children aged 6-18 months.

Nutrition support programs that only focus upon better complementary feeding remain an insufficient means of limiting growth faltering in vulnerable populations of children. To determine if symptoms of acute infections correlate with the incidence of growth faltering in rural Malawian children, the associations between fever, diarrhea, and cough with anthropometric measures of stunting, wasting, and underweight were investigated. Data were analyzed from a trial where 209 children were provided with adequate complementary food and followed fortnightly from 6-18 months of age. Linear mixed model analysis was used to test for associations. Diarrheal disease was inversely associated with changes in height-for-age Z-score (HAZ), mid-upper arm circumference Z-score (MUACZ), and weight-for-age Z-score (WAZ). Fever was also inversely associated with changes in MUACZ and WAZ. These results suggest that initiatives to reduce febrile and diarrheal diseases are needed in conjunction with improved complementary feeding to limit growth faltering in rural Malawi.

Liver Pathology, Including MOC31 Immunohistochemistry, in Congenital Tufting Enteropathy.

Congenital tufting enteropathy (CTE) is a rare heritable cause of intractable diarrhea due to EPCAM mutation. Pathologic findings include intestinal villous atrophy, tufted discohesive tear-drop-shaped epithelium, and a normal brush border. In affected patients, absent intestinal epithelial cell adhesion molecule (EpCAM) expression results in loss of MOC31 immunostaining. CTE liver pathology has not yet been described. We identified CTE patients with liver biopsies and reviewed clinicopathologic material including MOC31 immunohistochemistry. Three CTE patients had 4 liver core biopsies (at ages 1, 5, 7, and 16 y), 2 for preintestinal transplant evaluation, and 2 (from a single patient) for pretreatment assessment of chronic hepatitis C; all had received parenteral nutrition (PN). All samples showed loss of biliary epithelial polarization and mild portal and lobular inflammation. Only the hepatitis C patient demonstrated fibrosis. One patient each had lobular neutrophilic microabscesses and macrovesicular steatosis. Proliferative ductular reactions were absent in CTE patients but present in all controls on PN for other reasons. MOC31 was absent in biliary epithelium and hepatocytes of all CTE patients; controls showed consistent strong membranous biliary epithelial and patchy membranous periportal hepatocyte staining. Our data show that, histologically, hepatopathy in CTE can be difficult to separate from comorbid disease including PN effect; however, the absent ductular reaction may be characteristic. MOC31 localization in the biliary epithelium and zone 1 hepatocytes of controls suggests these compartments of the liver might be most susceptible to effects of EpCAM deficiency. In addition, we validate the liver as suitable tissue for CTE diagnosis using MOC31 immunohistochemistry.

Evaluation of simple laboratory investigations to predict fatal outcome in infants with severe malnutrition presenting in an urban diarrhoea treatment centre in Bangladesh.

OBJECTIVE: To evaluate rapid and simple laboratory investigations to predict fatal outcome in infants presenting with diarrhoea and severe malnutrition. METHOD: Retrospective chart analysis of infants with severe malnutrition and diarrhoea with (cases) and without fatal outcome (controls) admitted to the Special Care Ward in Dhaka Hospital at ICDDR,B between May 2005 and April 2006. All infants (n=61) who underwent bedside blood glucose, full peripheral blood count, serum C-reactive protein (CRP), and serum electrolyte tests were included. RESULTS: In logistic regression analyses, after adjusting for all available potential confounders (abnormal WBC count, higher CRP level, hyponatraemia, hypokalaemia, hypocalcaemia, and hypomagnesaemia), cases (n=10) were significantly associated only with hypoglycaemia (measured using a portable bedside finger blood glucose test) (odds ratio 5.0, CI 1.1-23.0, P=0.039) on admission. CONCLUSION: A simple rapid bedside glucose test may be used to predict the outcome of diarrhoeal infants presenting with severe malnutrition.

Risk factors associated with severe underweight among young children reporting to a diarrhoea treatment facility in Bangladesh.

Protein-energy malnutrition (PEM) is a serious health problem among young children in Bangladesh. PEM increases childhood morbidity and mortality. Information is needed on the major risk factors for PEM to assist with the design and targeting of appropriate prevention programmes. To compare the underlying characteristics of children, aged 6-24 months, with or without severe underweight, reporting to the Dhaka Hospital of ICDDR,B in Bangladesh, a case-control study was conducted among 507 children with weight-for-age z-score (WAZ) < -3 and 500 comparison children from the same communities with WAZ > -2.5. There were no significant differences between the groups in age [overall mean+standard deviation (SD) 12.6 +/- 4.1 months] or sex ratio (44% girls), area of residence, or year of enrollment. Results of logistic regression analysis revealed that severely-underweight children were more likely to have: undernourished mothers [body mass index (BMI) < 18.5, adjusted odds ratio (AOR) = 3.8, 95% confidence interval (CI) 2.6-5.4] who were aged < 19 years (AOR = 3.0, 95% CI 1.9-4.8) and completed < 5 years of education (AOR = 2.7, 95% CI 1.9-3.8), had a history of shorter duration of predominant breastfeeding (< 4 months, AOR = 2.3, 95% CI 1.6-3.3), discontinued breastfeeding (AOR = 2.0, 95% CI 1.1-3.5), and had higher birth-order (> 3 AOR = 1.8, 95% CI 1.2-2.7); and fathers who were rickshaw-pullers or unskilled day-labourers (AOR = 4.4; 95% CI 3.1-6.1) and completed < 5 years of education (AOR = 1.5; 95% CI 1.1-2.2), came from poorer families (monthly income of Tk < 5,000, AOR = 2.7, 95% CI 1.9-3.8). Parental education, economic and nutritional characteristics, child-feeding practices, and birth-order were important risk factors for severe underweight in this population, and these characteristics can be used for designing and targeting preventive intervention programmes.

[Is oral rehydration with nasogastric tube more efficient than rehydration with spoon? Preliminary study in children with non-severe dehydration in Joseph-Raseta-Befelatanana Hospital, Madagascar]. / La réhydratation orale par sonde gastrique est-elle plus efficace que la réhydratation orale par cuillère ? Etude préliminaire chez l'enfant atteint de déshydratation non sévère à l'hôpital Joseph-Raseta-Befelatanana, Madagascar.

Since its recommendation by WHO, Oral Rehydration Solutions (ORS) contributed in reducing the rate of mortality due to acute gastroenteritis. In Madagascar, the rate of lethality imputed to gastroenteritis is about 3%. Rehydration can be performed either by using spoons which reliability is unsure because of parents' potential inobservance and child's refusal, or by nasogastric tube. The nasogastric tube may resolve these obstacles at the hospital. We realized a preliminary study to evaluate the efficacy and the safety of nasogastric tube and spoon administration of the ORS in rehydration of child with moderate post-gastroenteritis dehydration. This is an open comparative study realized in the Pediatric Unit at the Joseph-Raseta-Befelatanana Hospital from the 21 January to 21 May 2008. Main outcomes was the proportion of rehydrated children at 4 hours, other were: failure at 8 hours, duration and volume of SRO, side effects. Fifty-three children from 4-month to 4-year old among 1306 patients were recruited and forty-seven patients were included. Nine patients failed (4 children received ORS by spoon and 5 children by nasogastric tube). The use of spoon was more effective: 62.5% of the patients were rehydrated at the fourth hour versus 39.3% in nasogastric tube group (P = 0.04). Making debit constant presents difficulties in rehydration with nasogastric tube (44.4 %). Tolerance of nasogastric tube is generally good but 16.7% children get out their tube during this study. No ORS' inhalation was observed with both routes. No false passage or tube or ORS rejection was recorded in both techniques. This study shows that using spoon to rehydrate is more effective for the rehydration of moderate dehydration. The use of nasogastric tube needs more surveillance.

[Diarrheagenic Escherichia coli associated with acute diarrhea in children of Cumaná, Venezuela]. / Escherichia coli diarreogénica asociada a casos de diarrea aguda en niños de Cumaná, Venezuela.

To establish the prevalence of strains of diarrheagenic Escherichia coli (DEC) associated to acute diarrhea in children of Cumaná, Venezuela, stool samples were taken from 200 children aged < 5 years with acute diarrheal disease, and from 30 healthy children used as control. Isolation and bacterial identification was performed by conventional biochemical tests and stool cultures. The presence of pathogenic genes of each type of DEC was investigated by the technique of polymerase chain reaction (PCR), determining the genes eae and bfp (EPEC), st and lt (ETEC), ipaH and virF (EIEC) Stx1/Stx2 (STEC), aafII (EAEC) and daaE (ADEC). From 169 E. coli, isolates we determined by PCR 10.65% positive for EPEC (1.18% "typical", 9.47% "atypical"); ETEC (5.91%); EAEC (1.78 %), EIEC (0.59%). There were no statistically significant differences regarding the frequency of each "pathotype" in relation to age, but it did occur when related to the sex (p<0.05). The most relevant clinical features were: fever, vomiting and abdominal pain and the greatest percentage of children affected were of the working and marginal classes. These results shown that the strains of DEC are important etiological agents in acute infectious diarrhea in children of Cumaná.

A comparative study of the prevalence of zinc deficiency among children with acute diarrhoea in SouthWestern Nigeria.

BACKGROUND: Zinc deficiency has been associated with increased incidence, severity and duration of childhood diarrhoea. OBJECTIVE: The objective of the study was to determine the prevalence of zinc deficiency among under-five children with acute diarrhoea. METHODS: The study was a comparative cross-sectional study in which serum zinc levels were determined using atomic absorption spectrometry in under-five children with acute diarrhoea and in apparently healthy contols. Two hundred and fifty children with acute diarrhoea and 250 controls were studied at the Wesley Guild Hospital, Ilesa, Nigeria. RESULTS: The diarrhoea patients had a mean ± SD serum zinc level of 78.8 ± 35.6 µg/dl, while the controls had a mean of 107.3 ± 46.8 µg/dl. The mean serum zinc level was significantly lower in the patients than the controls (t = -7.66; p < 0.001). Furthermore, the prevalence of zinc deficiency was significantly higher among the patients (30.4% versus 12.4% in the controls; OR = 3.09; 95% CI = 1.94 - 4.90; χ2 = 24.08; p < 0.001). Low social class was associated with a significantly higher prevalence of zinc deficiency among the patients (p = 0.013). CONCLUSION: Zinc deficiency is significantly associated with diarrhoea among under-five children in the study community. Hence, routine zinc supplementation should be encouraged for the treatment of diarrhoea, and availability should be ensured.

Frequency of diarrhoea as a predictor of elevated blood pressure in children.

BACKGROUND: Diarrhoeal illness is a major public health problem for children worldwide, particularly among developing countries, and is a proxy condition for severe dehydration. It has been hypothesized that severe dehydration in the first 6 months of life could be associated with increased blood pressure later in life. This study aimed to explore whether frequency of diarrhoea is associated with elevated blood pressure in children in a setting with a high incidence of diarrhoeal disease. METHODS: The present study is a cross-sectional study of blood pressure among children from a longitudinal child diarrhoeal disease cohort in Lima, Peru. From 2001 to 2006, daily diarrhoeal surveillance was made. Children were revisited in 2006 and blood pressure was measured. Diarrhoeal exposures were evaluated in terms of total number of diarrhoea days, number of episodes of diarrhoea, persistent diarrhoeal episodes and by the quartiles of daily incidence and episode incidence of diarrhoea. RESULTS: The overall incidence of diarrhoeal episodes at age under 1 year was 4.35 (95% confidence interval: 3.79-4.98) and under 5 years was 2.80 (95% confidence interval: 2.69-2.92). No association was observed between the total number of diarrhoeal days, diarrhoeal episodes or diarrhoeal incidence rates with childhood blood pressure. There was weak evidence that hospital admission due to severe dehydration in the first year of life showed a gradient towards an increase in both, systolic and diastolic blood pressure. CONCLUSION: In the first study to date to examine the association in a setting with a high incidence of diarrhoeal disease, diarrhoeal frequency did not show an association with increased blood pressure. Our observations of elevated levels of blood pressure among those admitted into hospitals in the first year of life are in line with the original hypothesis of dehydration in early infancy and high blood pressure. However, the effect of episodes of severe dehydration on later blood pressure remains uncertain.

Infantile systemic hyalinosis presenting as intractable infantile diarrhea.

Infantile systemic hyalinosis is an autosomal recessive disease characterized by severe progressive flexion contractures, multiple recurring subcutaneous tumours, and gingival hypertrophy. It is caused by mutations in the gene encoding capillary morphogenesis protein-2 (CMG2). Here we report a Saudi infant with infantile systemic hyalinosis who presented with intractable diarrhea, and we review the literature emphasizing recent developments in the molecular genetics of this disease.

Factors associated with sclerema in infants with diarrhoeal disease: a matched case-control study.

AIM: To identify clinical and biochemical factors associated with sclerema in infants with diarrhoeal illness, and their outcome. METHODS: In this case-control study, we enrolled 30 infants with clinical sepsis with sclerema (cases) and another 60, age- and sex-matched infants with clinical sepsis but without sclerema (controls) from among those admitted to the special care unit (SCU) and longer stay unit (LSU) of the Dhaka Hospital of International Centre for Diarrhoeal Disease Research, Bangladesh (ICDDR,B) for their diarrhoeal illness from May 2005 through April 2006. Sclerema as the dependant variable while hypoxia, hypothermia, C-reactive protein (CRP) level, serum total protein and prealbumin level were the major independent variables compared in the analysis. Differences in proportions were compared by the chi-square test and differences of mean were compared by Student's t-test or Mann-Whitney test, as appropriate. RESULTS: The case-fatality was significantly higher among the cases than the controls (30% vs. 2%, CI 2.9-565.5). After adjusting for confounders, infants with sclerema were more likely to be hypothermic (OR 11.6, 95% CI 1.1-126.5), and have lower serum total protein (OR 1.12, 95% CI 1.04-1.21) and prealbumin (OR 1.5, 95% CI 1.1-2.3). CONCLUSION: Diarrhoeal infants having clinical sepsis presenting with hypothermia, lower serum protein and prealbumin are prone to be associated with sclerema.

Predictors of mortality in infants with sclerema presenting to the Centre for Diarrhoeal Disease, Dhaka.

BACKGROUND: Sclerema is an uncommon, life-threatening condition, usually of newborns, with a case-fatality rate ranging from 50 to 100%. Very little is known about factors influencing outcome. AIM: To identify clinical and biochemical predictors associated with fatal outcome of sclerema in infants with diarrhoea. METHODS: Thirty infants with sclerema admitted to the Special Care Unit of the Dhaka Hospital of ICDDR,B with diarrhoea from May 2005 to end April 2006 were studied prospectively. Nine infants who died (30%) were considered to be cases while the 21 who survived constituted the comparison group. Hypothermia, severe malnutrition, septic shock, serum ammonia and CRP levels were considered to be predictors of death. Differences in proportions were compared by the chi(2) test and mean differences were compared using Student's t-test or the Mann-Whitney test, as appropriate. RESULTS: The mean age of the 30 infants was 2.1 months (range 12 d to 8 m). Fatal cases were more likely than survivors to be associated with severe underweight, a positive blood culture and higher serum ammonia and serum CRP levels. After adjusting for possible confounders in logistic regression analysis, the likelihood of death was higher in infants admitted with septic shock or who developed it soon after admission (OR 17.96, 95% CI 1.5-0220.4, p=0.024). CONCLUSIONS: Sclerema is associated with a high fatality rate and scleremic infants with diarrhoea who present with septic shock are at a greater risk of death.