Intranatal Torsion of Polydactyly: A Rare Event.

Polydactyly is one of the most common anomalies of hand and/or foot. Postnatal torsion of pedunculated polydactyly is a well known complication but intranatal torsion has been infrequently described in published literature. Here, we describe a case of pedunculated ulnar polydactyly which was gangrenous at birth due to intranatal torsion. Controversies surrounding the management of narrow pedicled pedunculated polydactyly by traditional method of suture ligation at base are also discussed.

Millennium development goals and child undernutrition.

Reduction in prevalence of underweight children (under five years of age) has been included as an indicator for one of the targets to eradicate extreme poverty and hunger (Goal 1) of the Millennium Development Goals (MDGs). The most recent MDG report of 2012 indicates that the target of reducing extreme poverty by half has been reached five years ahead of the 2015 deadline but close to one third of children in Southern Asia were underweight. In India, at the historical rate of decline the proportion of underweight children below 3 years, required to be reduced to 26% by 2015, is expected to come down only to about 33%. With barely 3 years left for achieving MDGs, the level of commitment to reduce child undernutrition needs to be gauged and effectiveness of current strategies and programmes ought to be reviewed. Undernutrition in children is not affected by food intake alone; it is also influenced by access to health services, quality of care for the child and pregnant mother as well as good hygiene practices. Would the scenario be different if child undernutrition was a part of Goal 4 of MDGs? What difference it would have made in terms of strategies and programmes if reduction in undernutrition in children underfive was a target instead of an indicator? It is time for nutrition to be placed higher on the development agenda. A number of simple, cost-effective measures to reduce undernutrition in the critical period from conception to two years after birth are available. There is a need for choosing nutrition strategies relevant in Indian context. Experiences from other countries should lead India toward innovative nutritional strategies to reduce underfive undernutrition in the country- that too on a fast track.

Serum soluble interleukin-2 receptor, interleukin-6 and tumor necrosis factor alpha as markers of celiac disease activity.

OBJECTIVES: To evaluate the markers of lymphocyte activation (sIL-2R, IL-6 and TNF α) in the peripheral blood of newly diagnosed patients with celiac disease (CD) and patients with CD on Gluten free diet (GFD) for at least 2 y. The markers were correlated with conventional serological tests Anti-tissue transglutaminase (Anti-TTG) used for diagnosis and follow up of the disease; wherever possible. METHODS: Thirty newly diagnosed cases of CD (on the basis of histopathology and serology) not on GFD were enrolled as Group 1 of the study. Thirty age and sex matched controls from the Pediatric Surgery OPD formed Group 2. Thirty cases of CD on GFD for at least 2 y (Group 3) were also enrolled in the study. Upper G.I. endoscopy was performed in all Group 1 patients and cytokine levels assayed by ELISA on serum obtained from all patients in Groups 1, 2, 3. RESULTS: Mean sIL-2R level in Group 1(1498.1+/-1234.31 pg/ml) and Group 3 (488.78+/-396.18 pg/ml) were significantly higher than the controls (336.27+/-218.67 pg/ml p < 0.05). Among the patients with CD, mean serum levels in Group 1 were higher than in Group 3 (p < 0.05). sIL-2R levels showed good correlation with tTg levels in Group 1 patients (p < 0.000, r = 0.69). Mean IL-6 levels in Group 1 were significantly higher (28.43+/-28.32 pg/ml) than Group 2(15.03+/-7.72 pg/ml p < 0.05) and Group 3(11.26+/-5.13 pg/ml p < 0.05). IL-6 levels were comparable between Groups 2 and 3 (p > 0.05).IL-6 levels showed good correlation with tTg levels in Group 1(p < 0.008, r = 0.471). Mean TNFα levels in Group 1(179.66+/-102.93 pg/ml), Group 2 (153.16+/-27.02 pg/ml) and Group 3 (166.67+/-28.95 pg/ml) were comparable (p > 0.05). TNFα levels showed poor correlation with tTg levels in Group 1 patients (p > 0.604, r = -0.099). CONCLUSIONS: sIL-2R and IL-6 levels have a good correlation with CD activity and can be used as reliable markers for detecting minimal transgression from GFD.

Hemophagocytic syndrome associated with visceral leishmaniasis.

The present paper reports a case of 6-year-old male child, suffering from pallor, fever and hepatosplenomegaly. A clinical diagnosis of enteric fever with a second possibility of malaria was considered. Laboratory findings included bicytopenia, hyperbilirubinemia and raised liver enzymes. Bone marrow examination revealed active hemophagocytosis. On extensive search few amastigote forms of Leishmania donovani were seen. Patient was negative for other viral, bacterial and malaria infections. The final diagnosis of hemophagocytic syndrome associated with visceral leishmaniasis was made. There was response of anti-Leishmanial treatment with improvement in clinical condition.

Catch-up growth in children with late-diagnosed coeliac disease.

Anthropometric parameters and catch-up growth were prospectively evaluated in fifty late-diagnosed children with coeliac disease aged 2.25-10 years after 1-4 years of adhering to a strict gluten-free diet (GFD). The anthropometric parameters were expressed as Z scores relative to National Centre for Health Statistics standards using Epi Info 2000 (weight-for-height Z score (WHZ) and height-for-age Z score (HAZ)). Catch-up growth was evaluated by repeated measures. ANOVA, overall significance by an F test and pair-wise comparisons for estimated marginal means using the least significant difference. At the time of enrolment, no significant difference was observed in WHZ and HAZ between children diagnosed before (group 1) or after (group 2) 4 years of age. On follow-up, HAZ was significantly higher in group 1 after the first and third years of the GFD (P=0.04 and 0.02, respectively), with a non-significant increase after completing 4 years of the GFD (P=0.22). Feeding the GFD resulted in an overall significant (F=3.99, P=0.011) increase in HAZ up to 4 years of follow-up. However, the catch-up in height was incomplete, with stunting in sixteen (55.4%) of twenty-nine children after 3 years and in seven (46.6%) of fifteen children after 4 years on the GFD. Pair-wise comparisons demonstrated a linear catch-up growth during the initial follow-up on GFD. Treatment with the GFD did not result in an overall significant increase in WHZ up to 4 years of follow-up (F=1.01, P=0.42). Our results suggest that, in children with late-diagnosed coeliac disease, treatment with a GFD leads to a normalisation of body mass and a significant but incomplete recovery in HAZ during 4 years of follow-up.

Serum prolactin in celiac disease.

Serum prolactin levels (SPL) were estimated in patients with celiac disease (CD), diagnosed as per ESPGAN criteria, on unrestricted gluten containing diet (group 1), as well as those consuming a gluten-free diet (GFD) (group 2). Forty-one children with CD, with 20 cases in group 1 (mean age 5.67 +/- 2.14, range 2.5-10.5 years) and 21 cases in group 2 (mean duration of follow-up 2 years, range 1-4 years), and 41 age- and sex-matched controls were studied. Hyperprolactinemia was defined as serum prolactin > 18 ng/ml in males and > 24 ng/ml in females. Upper gastrointestinal endoscopic biopsy was performed in both study groups for initial and follow-up evaluation. Hyperprolactinemia was detected in all the patients of group 1 and one patient of group 2 who had severe villous atrophy. The SPL in group 1 (mean 48.3 +/- 17.4; range 20-90 ng/ml) and group 2 (mean 18.3 +/- 6.9, range 10-39 ng/ml) was significantly higher compared with the controls (mean 9.3 +/- 4.5; range 2.4-20 ng/ml; p < 0.001). Among the patients with CD, mean SPL in group 1 was significantly higher than in group 2 (p < 0.001). In group 1, there was a positive correlation between SPL and duration of symptoms (p = 0.006, r = 0.768) and age of diagnosis (p < 0.001, r = 0.842). A positive correlation also existed in group 2 between SPL and degree of villous atrophy (p < 0.001, r = 0.71) and lamina propria infiltrate (p < 0.001, r = 0.568). Our results suggest that SPL has a significant correlation with activity of CD. Therefore serum prolactin estimation may provide an additional marker of disease activity in CD and may be a more viable option economically.

Clinical and nutritional profile of children with celiac disease.

This prospective study was aimed to evaluate the clinical and nutritional profile of children diagnosed as celiac disease (CD) as per the modified ESPGAN criteria, at the time of diagnosis and after institution of gluten free diet. Out of 65 enrolled cases of CD, 7 (10.8%) children did not follow a strict dietary compliance. Only 41 children with satisfactory dietary compliance on gluten free diet (GFD) who regularly attended the follow up for at least 6 months were evaluated for their nutritional and hematological status. Results were compared with age and sex matched controls. The mean age of diagnosis was 8.7 +/- 3.3 years. Diarrhea and failure to thrive were the most common presenting symptoms. At diagnosis, the nutritional and hematological indices were significantly lower in patients than in controls. Mean duration of follow up on GFD was 22 months (range 6-48 +/- 5.6 months). On follow up, height for age Z score was significantly lower, mean BMI was significantly higher, and weight for age Z score, weight for height Z score (%), mean triceps and biceps skin fold thickness, and mid arm circumference were comparable to controls. At diagnosis, 80% cases had microcytic hypochromic anemia and 20% had dimorphic anemia. On GFD for at least a period of more than 6 months, 19% had microcytic anemia and in 81% the hematological picture was normocytic normochromic. 60% cases had thrombocytosis at diagnosis in comparison to 2.3% after treatment. Institution of GFD leads to rapid improvement in clinical picture as well as most of the nutritional and hematological parameters.

Evaluation of invasive and non-invasive diagnostic modalities for Helicobacter pylori infection in children.

Invasive and non invasive tests for Helicobacter pylori performed on 31 children were evaluated as diagnostic modalities. Investigations included upper gastrointestinal endoscopy and endoscopic grasp biopsy (EGB) from antrum and corpus (for rapid urease test, impression smear, histology and culture), antral brushings, serum ELISA for IgG antibodies, rapid blood test, and IgG antibodies in unstimulated saliva. Our results suggested that amongst the invasive methods brush cytology was more sensitive than histology and impression smear. Best interpretation of urease test was possible at 4 hours incubation. Culture of EGB sample constitutes the most specific way to establish the diagnosis of infection but is not easy. Hence, non-invasive modalities like serum ELISA, rapid blood test and salivary ELISA can be used in children for the detection of H pylori infection.

Iron supplementation in children with celiac disease.

OBJECTIVE: To evaluate the effect of iron supplementation, in addition to gluten free diet (GFD), on hematological profile of children with Celiac Disease (CD). METHODS: Children diagnosed as CD as per modified ESPGAN criteria were prospectively evaluated for their hematological profile at the time of their enrolment and after consuming GFD for at least one year. The results were compared with age and sex matched controls. Evaluation of hematological profile included hemoglobin estimation, complete blood counts, peripheral blood smear examination, serum iron, total iron binding capacity (TIBC), and serum ferritin estimation. All the enrolled cases were given iron supplementation in addition to exclusion of gluten from their diet. Repeat intestinal biopsy was performed in all the cases after completing 1 year on GFD. RESULTS: Twenty one children (mean age 6.67 years, range 4-11 years) diagnosed as CD who completed at least one year of regular follow up on GFD (mean 1.5 years, range 1-2 years) were analysed for their hematological profile at the time of enrolment and after consuming GFD and iron supplementation. At the time of enrolment all the children had hemoglobin level <11 gm%, 78% had microcytic hypochromic anemia and 22% had dimorphic anemia, with lower mean MCV, MCH and serum ferritin levels, and a significantly higher mean TIBC as compared to controls (p<0.001). In the follow up evaluation of these cases on GFD, mean hemoglobin levels were comparable with controls but the cases continued to have lower mean MCV, MCH serum ferritin levels (p<0.05) and higher mean TIBC (p<0.05). Seven children had mild anemia. Serum ferritin levels showed a negative correlation with the grade of villous atrophy and lamina propria infiltrate. CONCLUSION: Our results suggest that iron deficiency anemia (IDA) is commonly associated with CD and iron deficiency state continues for a longer time even after excluding gluten from the diet and iron supplementation. Apart from offering them GFD rich in iron, early detection and treatment of IDA and prophylactic iron folic acid supplementation will go a long way to optimize their mental and psychomotor functions.

Cladosporium bantianum meningitis in a neonate.

Cladosporium bantianum meningitis has been reported mostly in adult farmers between 20 and 30 years of age. We report a 6-day-old male neonate who was admitted with fever, focal seizures and not accepting feeds. Initial investigations suggested a diagnosis of pyogenic meningitis but antibiotic therapy for 14 days did not result in any significant clinical improvement. Repeat CSF examination after 14 days suggested a diagnosis of C. bantianum meningitis which was supported by presence of multiple abscesses in the cerebral cortex on CT scan of the head and confirmed by CSF culture. Clinical response to antifungal therapy remained unsatisfactory.

Diagnostic modalities for gastroesophageal reflux.

OBJECTIVE: To evaluate commonly utilized diagnostic modalities to detecting Gastroesophageal Reflux (GER). METHODS: Sixty children aged 1-72 months (mean age 14.7 months) with symptoms suggestive of Gastroesosphageal Reflux (GER) were investigated and subjected to upper gastrointestinal endoscopy and esophageal biopsy (EB), gastroesophageal scintiscanning (GS) and 24 hour ambulatory pH monitoring. RESULT: GER was detected in 28 (46.7%) cases by one or more diagnostic modalities. Ambulatory 24 hour pH monitoring was positive in higher proportion (43.3%) of cases in comparison to other modalities, followed by EB (38.3%) and GS (30%). Considering 24 hour pH monitoring as the gold standard, esophageal biopsy was positive in 22/26 cases (84.6%) detected by 24 hour pH monitoring with a specificity of 97.1% as compared to 17/26 cases (65.4%) by gastroesophageal scintiscanning with a specificity of 97.1%. When compared with EB results, amongst various parameters measured during 24 hour pH monitoring, Reflux index (RI) ranked highest (sensitivity 95.6 % and specificity 89.2 %) followed by duration of longest episode > 20 minutes and Euler Byrne score. Oscillatory index, calculated from tracings of pH monitoring, even though ranked lower because of its low sensitivity helped to pick up 2 cases missed by EB and RI. CONCLUSION: Our results suggest that a combination of diagnostic modalities may be required to diagnose GER in young children. Ambulatory 24 hour pH monitoring appears to be the single best investigation and combining it with EB and/or GS can help to detect maximum number of cases.

Integrated Management of Childhood Illness (IMCI): a robust strategy.

Integrated Management of Childhood Illness (IMCI), a strategy fostering holistic approach to child health and development, is built upon successful experiences gained from effective child health interventions like immunization, oral rehydration therapy, management of acute respiratory infections and improved infant feeding. The core intervention of IMCI is integrated management of the five most important causes of childhood deaths-acute respiratory infections, diarrheal diseases, measles, malaria and malnutrition. Using a set of interventions for the integrated treatment and prevention of major childhood illnesses, the IMCI strategy aims to reduce death as well as the frequency and severity of illness and disability, thus contributing to improved growth and development. In health facilities, the IMCI strategy promotes the accurate identification of childhood illness (es) in the outpatient settings, ensures appropriate combined treatment of all major illnesses, strengthens the counselling of caretakers and the provision of preventive services, and speeds up the referral of severely ill children. The strategy also aims to improve the quality of care of sick children at the referral level. It also creates a scientifically sound link between the management guidelines at the community level and the management approach in a referral centre. The strategy also envisages actual situations when referral is not possible and offers the best possible options in such circumstances. In the home setting, it promotes appropriate early home care and care-seeking, improved nutrition and prevention, and the correct implementation of prescribed care. In addition to its focus on treatment of illness in the health facility as well as at home, it also provides an opportunity for important preventive interventions such as immunization and improved infant and child nutrition including breastfeeding. The IMCI strategy reduces wastage of resources and avoids duplication of efforts that may occur in a series of separate disease control programs. The essential pillars include improvement in the case management skills of health personnel, improvement in health systems, and improvement in family and community practices. IMCI has been introduced in more than 80 countries and 19 of them have already scaled up IMCI implementation Even though it is too early to relate the decrease in childhood mortality with the introduction of IMCI in these countries, there are several indirect indicators which endorse its validity as a comprehensive and effective strategy. IMCI has helped countries to revise and update their child health policies, streamline the essential drug lists for children, increase service utilization, improve quality of care and nutritional counselling, improve health systems and improved family and community practices.

Association of gastroesophageal reflux disease in young children with persistent respiratory symptoms.

Forty children aged between 3 months and 3 years (median age 14 months) with persistent respiratory symptoms beyond 4 weeks or recurrence of respiratory symptoms were investigated for gastroesophageal reflux (GER). Diagnostic tests included upper gastrointestinal endoscopy, oesophageal biopsy, gastroesophageal scintiscan and 24 h ambulatory oesophageal pH monitoring. GER was detected in 14 (35 per cent) of these patients; which included 38 per cent of the enrolled cases of recurrent bronchopneumonia, 40 per cent cases of reactive airway disease, and 22 per cent cases of persistent cough. Amongst the cases detected to have GER, the age of onset of respiratory symptoms was less than 1 year in 86 per cent of cases (p < 0.01), nocturnal symptoms of cough and wheeze were reported in 78 per cent (p < 0.05), and 86 per cent cases did not present with typical gastrointestinal symptoms (p < 0.01). Family history of asthma was absent in all cases of GER-related reactive airway disease (p < 0.01). Cases detected to have GER were followed for 3-6 months after starting anti-reflux therapy. A significant (p < 0.01) decrease was noticed in the number of further episodes in children with GER-related recurrent bronchopneumonia and reactive airway disease after starting anti-reflux therapy. Improvement was also noticed in nocturnal symptoms and nutritional status after anti-reflux therapy was started. Our results suggest that GER may be one of the possible contributing factors in any child with recurrent and persistent respiratory complaints. Early diagnosis and anti-reflux therapy in cases with GER-related respiratory complaints can result in significant improvement in symptoms.

Feeding problems in children with cerebral palsy.

OBJECTIVE: To determine the magnitude and extent of feeding problems in children with cerebral palsy (CP) and to evaluate the effectiveness of nutritional interventions. DESIGN: Prospective hospital based interventional study. METHOD: Children with cerebral palsy of either sex were enrolled randomly and their parents were interviewed for their perception about feeding problems, nutritional status and for their views about the expected outcome of feeding problems. Each case was assessed for feeding problems based on Gisel and Patrick feeding skill score; for nutritional status by measurement of weight, skinfold thickness (at biceps, triceps, suprailiac and subscapular), mid arm circumference and caloric intake; neurologically for type and severity of cerebral palsy and for developmental age by Gasell s developmental scale. Equal number of age and sex matched controls were included for comparison of nutritional status and developmental quotient. Various rehabilitation procedures were applied and their response was observed in the followup ranging from 3-10 months. RESULTS: One hundred children (76 boys and 24 girls) with cerebral palsy of mean age 2.5 years (range 1 to 9 years) and mean developmental age of 7.6 months (range 1 to 36 months) were included in the study. Oral motor dysfunction (OMD) was found in all cases and in each category. Spastic quadriplegic cerebral palsy (SQCP) and hypotonic patients had significantly poor feeding skill score (p < 0.001). Mean duration of feeding session was 31.5 minutes (range 10-60 minutes). Main food of children with cerebral palsy consisted of liquid and semisolid diet. Children with poor OMD were unable to take solid food. Cases with seizures had significantly more feeding problems than those without seizures (p < 0.001). Parental awareness about feeding problems of their children was significantly low and they overestimated the nutritional status of their children. Anthropometric indicators were significantly lower than controls (p < 0.001). Spastic quadreparesis, hypotonia and poor feeding skill score had negative effect on nutritional status. Thirty per cent parents of cerebral palsy patients were pessimistic about the possibility of any improvement in feeding problems. After nutritional rehabilitation, good improvement was seen in feeding problems, OMD and nutritional status. CONCLUSION: Nutritional status of children with cerebral palsy is poor due to summation of several factors. Therefore, they should be thoroughly assessed for feeding problems and nutritional status in order to start timely nutritional rehabilitation which can significantly improve their nutritional status and quality of life.