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Objective: Drowning is a leading cause of unintentional injury-related death in low- and middle-income countries (LMICs). This study was undertaken to know the epidemiology, intensive care needs and predictors of outcome of children with drowning., Methods: Records of children below 12 years admitted with drowning to the emergency room and/or paediatric intensive care unit (PICU) of a tertiary care hospital in North India were retrospectively analysed. 'Favourable outcome' was defined as normal neurological status at discharge (normal cognition and no motor deficits) and 'unfavourable outcome' as death or abnormal neurological status at discharge. Multivariable analysis was done for predictors of unfavourable outcome., Results: Twenty-seven children were analyzed, 14 (51.8%) were boys. Median (IQR) age was 18 months (12-30). The median (IQR) duration of submersion was 4 min (3-9). Six children (22.2%) presented in pulseless arrest, and 7 (43.7%) had both hypoxaemia (saturation <94% on room air) and encephalopathy (GCS <13) at admission. Ten children (37%) were transferred to PICU; principal indications being hypoxic ischaemic encephalopathy (HIE) (n = 6) and ventilation (n = 4). One child died, four survived with sequalae. Predictors of unfavourable outcome on univariable analysis were hypoxaemia, or pulseless arrest at admission, HIE and need for mechanical ventilation; none of these could predict outcome on multivariable analysis., Conclusion: Hypoxaemia or pulseless arrest at admission, HIE and need for ventilation predict unfavourable outcome. Appropriate on-site resuscitation and early stabilization may improve outcome in children with drowning in LMIC., (© The Author(s) [2019]. Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Background: Loop-mediated isothermal amplification (LAMP) assay is a novel molecular diagnostic technique that can be used for the diagnosis of tuberculosis (TB). It is most suited for developing countries as it is rapid, inexpensive, highly sensitive, requiring minimal infrastructure, training and manpower. Studies in pediatric TB are lacking. We evaluated LAMP in the diagnosis of pediatric pulmonary TB., Methodology: This was a cross-sectional analytical study conducted at a tertiary care teaching hospital in North India from July 2014 to June 2015 involving 60 children with suspected pulmonary TB. Respiratory specimens (sputum, gastric lavage, bronchoalveolar lavage and/or endotracheal aspirates) were collected and subjected to BACTEC MGIT 960 culture, IS6110 PCR, and LAMP assay targeting IS6110 gene of Mycobacterium tuberculosis., Results: Thirty seven children had confirmed and probable TB according to the composite reference standard (CRS). Among all the 3 tests used for diagnosis of Pulmonary TB, LAMP had highest sensitivity (37.8%) followed by PCR (27%), and culture (21.6%) when compared against the predefined CRS. Culture had maximum specificity of 100%; and PCR, and LAMP had specificity of 95-96%. The sensitivity, specificity, PPV, and NPV of LAMP against culture as reference standard were 75%, 72.4%, 42.9%, and 91.3% respectively. Similarly sensitivity, specificity, PPV, and NPV of PCR against culture as reference standard were 75%, 86.2%, 60%, and 86.2% respectively. On combining LAMP with culture, sensitivity increased to 45.7% (7.8% increase, p = 0.04)., Conclusion: We noted that LAMP had highest sensitivity when compared to culture and PCR and comparable specificity., (Copyright © 2020 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.)

We report the case of an eight-year-old boy who presented with an acute encephalitis and was confirmed to have Japanese encephalitis (JE). In addition, we found the vesicular stage of neurocysticercosis (NCC). The co-occurrence of JE and NCC was thought to be synergistic as there is some evidence that in presence of NCC, the neuroinvasiveness and virulence of JE is greater and associated with poor outcome.

First reported in China, the 2019 novel coronavirus has been spreading across the globe. Till 26 March, 2020, 416,686 cases have been diagnosed and 18,589 have died the world over. The coronavirus disease mainly starts with a respiratory illness and about 5-16% require intensive care management for acute respiratory distress syndrome (ARDS) and multi-organ dysfunction. Children account for about 1-2% of the total cases, and 6% of these fall under severe or critical category requiring pediatric intensive care unit (PICU) care. Diagnosis involves a combination of clinical and epidemiological features with laboratory confirmation. Preparedness strategies for managing this pandemic are the need of the hour, and involve setting up cohort ICUs with isolation rooms. Re-allocation of resources in managing this crisis involves careful planning, halting elective surgeries and training of healthcare workers. Strict adherence to infection control like personal protective equipment and disinfection is the key to contain the disease transmission. Although many therapies have been tried in various regions, there is a lack of strong evidence to recommend anti-virals or immunomodulatory drugs.

Background: Sepsis-induced myocardial dysfunction has implications on outcome. For lack of echocardiography in resource-limited settings, myocardial biomarkers may be an alternative monitoring tool., Objective: This study was planned to explore the longitudinal behavior of creatine phosphokinase-MB (CPK-MB) in children with sepsis with and without shock, and its correlation with clinical and echocardiographic parameters over the first 10 days., Design: Prospective observational study., Setting: Tertiary care hospital in a lower-middle-income economy of South Asia., Patients: Children (3 months to 12 years) with nonshock sepsis (NSS) ( n = 40) and septic shock survivors (SSSs) ( n = 40) after optimal resuscitation. Patients with catecholamine refractory shock, preexisting heart disease, and cardiorespiratory event within the past 1 month were excluded from the study., Measurements and Main Results: Pediatric logistic organ dysfunction (PeLOD) score, vasoactive inotrope score (VIS), CPK-MB, and echocardiographic measures of myocardial function were recorded on days 1, 3, 7, and 10. Echocardiography was repeated at 1 month. Both groups were similar at baseline. The SSSs had higher CPK-MB (180 vs 53 IU/L; p < 0.001) and PeLOD score (2 ± 0.4 vs 11.7 ± 5.1, p < 0.001) on day 1 compared to the NSS children. More than half of the SSS and none of the NSS patients had myocardial dysfunction. Reduction in CPK-MB over 10 days correlated well with improvement in PeLOD ( p < 0.01), VIS ( p = 0.04), and echocardiographic measures of myocardial dysfunction ( p < 0.05) among SSSs. At 1 month follow-up, all had normal echocardiography., Conclusion: The SSSs had markedly elevated CPK-MB, and its fall paralleled the improvement in clinical status and myocardial dysfunctions. The CPK-MB could be a potential monitoring tool for septic cardiomyopathy in resource-limited settings., How to Cite This Article: Baranwal AK, Deepthi G, Rohit MK, Jayashree M, Angurana SK, Kumar-M P. Longitudinal Study of CPK-MB and Echocardiographic Measures of Myocardial Dysfunction in Pediatric Sepsis: Are Patients with Shock Different from Those without? Indian J Crit Care Med 2020;24(2):109-115., Competing Interests: Source of support: Nil Conflict of interest: None, (Copyright © 2020; Jaypee Brothers Medical Publishers (P) Ltd.)

Kerosene poisoning is one of the most common accidental poisoning in children in developing countries due common use of kerosene in house-hold and unsafe storage practices. Aspiration pneumonitis is the most common manifestation of kerosene ingestion due to its low viscosity, high volatility, and low surface tension. The treatment of aspiration pneumonitis due to kerosene poisoning is symptomatic including oxygen support, respiratory monitoring, and careful monitoring of fluid balance. Children with severe respiratory distress and hypoxemia unresponsive to supplemental oxygen and/or severe central nervous system involvement require early intubation and mechanical ventilation. Transfer to the pediatric intensive care unit (PICU) is required at this stage. Emesis, gastric lavage, and administration of activated charcoal are contraindicated due to risk of aspiration. There is no clear benefit of using corticosteroids or prophylactic antibiotics. Asymptomatic children should be kept under observation for atleast 6 hours after exposure. The mortality rate is low and death occurs due to pneumonitis. Camphor is used in house-hold items including vaporized or topical cold preparations, liniments, moth repellents, for performing rituals in religious ceremonies, and in antimicrobial preparations. Camphor poisoning is not very common in childhood. Even small doses of camphor can cause serious toxicity and is potentially fatal. The onset of action is very rapid (5-15 minutes). The common manifestations are confusion, restlessness, delirium, and hallucinations, muscle twitching, myoclonus, ataxia, hyperreflexia, fasciculations, and seizures. Seizures are common and serious complication in camphor toxicity. The treatment is supportive including decontamination, gastric lavage, activated charcoal, and seizure control. Naphthalene is a major constituent of mothballs which are commonly used in household to protect clothes from moths. Though the poisoning with naphthalene is uncommon in children, most of the cases with naphthalene poisoning occur in developing countries where mothballs are still commonly used. The manifestations of naphthalene toxicity are predominantly due to acute intravascular hemolysis leading to anemia, hemoglobinuria, methemoglobinemia, and acute kidney injury (AKI). The treatment of naphthalene toxicity is supportive in form of transfusion of the packed red blood cells, monitoring of fluid and electrolyte balance, administration of alkalis in presence of hemoglobinuria, and renal replacement therapy. Prevention is better than cure. The strategies should be adopted to prevent children being exposed to these toxic compounds in the house-hold. Safe storage of toxic compounds away from the reach of children, avoiding storing kerosene in cold drink and beverage bottles, community education, provision of electricity in rural areas, safe cooking practices, and parental supervision are important interventions to prevent accidental poisoning among children., How to Cite This Article: Kumar S, Kavitha TK, Angurana SK. Kerosene, Camphor, and Naphthalene Poisoning in Children. Indian J Crit Care Med 2019;23(Suppl 4):S278-S281., Competing Interests: Source of support: Nil Conflict of interest: None, (Copyright © 2019; Jaypee Brothers Medical Publishers (P) Ltd.)

Objectives: To study the clinico-laboratory profile and outcome of children with severe dengue and dengue-associated hemophagocytic lymphohistiocytosis (HLH)., Methods: In this retrospective study, 22 children with laboratory confirmed severe dengue admitted to pediatric intensive care unit (PICU) were enrolled. Clinical features, laboratory parameters, and outcome were noted and compared between cases fulfilling HLH-2004 criteria and those without HLH., Results: Median (IQR) age was 8 (5-10.3) y. Fever was present for mean (SD) duration of 5.3 (2.1) d. Vomiting, respiratory distress, pain abdomen and hepatomegaly were other clinical features. Thrombocytopenia, anemia and elevated serum transaminases were noted in 91%, 41% and 30% respectively; coagulopathy and hypoalbuminemia were seen in 36% each. Half (n = 11, 50%) had dengue shock syndrome. Acute respiratory distress syndrome (ARDS) (n = 7, 32%) and acute kidney injury (AKI) (n = 6, 28%) were other major organ dysfunctions. Mean (SD) duration of PICU stay was 3.6 (1.5) d with 13.6% mortality. HLH was noted in 7 (32%) cases at a median (IQR) hospital stay of 5 (2-8) d. Children with HLH had significantly higher Pediatric Index of Mortality 2 (PIM 2) score at admission and higher frequency of pain abdomen, anemia, hypoalbuminemia, elevated alanine aminotransferase (ALT) and ARDS. Length of PICU stay (5.1 vs. 2.9 d) and mortality (28.6% vs. 6.7%) were higher in HLH group, however the difference was not statistically significant. Steroids were used in 4 cases with HLH and all survived, whereas among 3 who did not receive steroids, 2 died (p = 0.23)., Conclusions: Severe dengue presents with life-threatening organ dysfunctions. HLH is increasingly recognized in dengue infection and maybe considered as a differential diagnosis in children with lower hemoglobin, hypoalbuminemia, elevated ALT and severe organ dysfunction.

Disorders associated with multiple vertebral segmentation defects may have additional rib anomalies in form of absence or hypoplastic ribs, fanning of ribs, etc. Spondylocostal dysostosis is genetic disorder with abnormal vertebral segmentation and rib anomalies. Diagnosis is often delayed because of non-familiarity with the characteristic features. There are six genes identified for spondylocostal dysostosis, of which SCDO5 is responsible for autosomal dominant form of the disorder. Retrospective study was conducted in Genetic and Metabolic unit of a tertiary hospital in north India over a period of 9 years. Twenty patients with a clinical diagnosis of congenital scoliosis were identified, and reviewed. Three patients were discussed in an earlier report and 11 subsequent patients, are described in this case series here. The median age at presentation was 34 months. The patients showed hemivertebrae, vertebral fusion, fusion of ribs, fanning of ribs. Hydrocephalus/ventriculomegaly was found in three cases and diastematomyelia was identified in one case. Other associated anomalies included corpus callosal agenesis, club foot and capillary malformation. One parent showed rib/spinal defects in two cases. Further studies are needed to characterise the phenotype and genetic basis of scoliosis in Indian patients.

Objectives: The purposes of this paper are to study clinicobacteriological profile, treatment modalities and outcome of pediatric empyema thoracis and to identify changes over a decade., Design: This is a retrospective study., Setting: Department of Pediatrics of a tertiary care hospital in North India., Patients: We enrolled 205 patients (1 month-12 years) of empyema thoracis admitted over 5 years (2007-11) and compared the profile with that of a previous study from our institute (1989-98)., Results: Pleural fluid cultures were positive in 40% (n = 82) cases from whom 87 isolates were obtained. Staphylococcus aureus was the most common isolate (66.7%). Methicillin-sensitive S. aureus accounted for 56%, Methicillin-resistant S. aureus (MRSA) 10% and gram-negative organisms 18.3% of isolates. Intercostal drainage tube (ICDT) was inserted in 97.5%, intrapleural streptokinase was administered in 33.6%, and decortication performed in 27.8% cases. Duration of hospital stay was 17.2 (±6.3) days, duration of antibiotic (intravenous and oral) administration was 23.8 (±7.2) days and mortality rate was 4%. In the index study (compared with a previous study), higher proportion of cases received parenteral antibiotics (51.7% vs. 23.4%) and ICDT insertion (20.5% vs. 7%) before referral and had disseminated disease (20.5% vs. 14%) and septic shock (11.2% vs. 1.6%), less culture positivity (40% vs. 48%), more MRSA (10.3% vs. 2.5%) and gram-negative organisms (18.4% vs. 11.6%), increased use of intrapleural streptokinase and surgical interventions (27.8% vs. 19.7%), shorter hospital stay (17 vs. 25 days) and higher mortality (3.9% vs. 1.6%)., Conclusions: Over a decade, an increase in the incidence of empyema caused by MRSA has been noticed, with increased use of intrapleural streptokinase and higher number of surgical interventions., (© The Author(s) [2018]. Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

CNS infections in children are medical emergency and are associated with high mortality and morbidity. For diagnosis, a high index of suspicion is required. Clinical assessment should be supplemented by laboratory investigations including CSF Gram stain and cultures, blood culture, PCR on CSF, serological tests, and imaging. Commonly associated life threatening complications include coma, seizure, raised intracranial pressure (ICP), focal deficits, shock, respiratory failure, and fluid and electrolyte abnormalities. Immediate management should first address control of airway, breathing and circulation; protocolized management of raised ICP and status epilepticus; maintaining adequate intravascular volume; and close monitoring for early detection of complications. Appropriate antimicrobial agents should be administered promptly according to the suspected pathogen. Clinical evaluation, laboratory workup, specific antimicrobial therapy, supportive treatment, and management of associated complications should go hand in hand in a protocolized way for better outcome.

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Objectives: To evaluate the effect of probiotics on cytokines in children with severe sepsis., Design: Randomized, double-blind, placebo-controlled trial., Setting: ICU of a tertiary care teaching hospital in North India., Patients: Children 3 months to 12 years old with severe sepsis., Interventions: Enrolled children were randomized to probiotic (n = 50) and placebo (n = 50) groups. Probiotic group received VSL#3 (Danisco-Dupont USA, Madison, WI) (Lactobacillus paracasei, L. plantarum, L. acidophilus, L. delbrueckii, Bifidobacterium longum, B. infantis, B. breve, Streptococcus salivarius; maltose and silicon dioxide), and placebo group received maltose and silicon dioxide. Dose was 1 sachet twice daily for 7 days. Blood was collected on days 1 and 7 for estimation of interleukin-6, interleukin-12p70, interleukin-17, tumor necrosis factor-α, interleukin-10, and transforming growth factor -β1. "Primary outcome": Change in cytokine levels in probiotic and placebo groups from day 1 to 7. "Secondary outcomes": Sequential Organ Failure Assessment score, healthcare-associated infections, ICU stay, and mortality., Measurements and Main Results: On day 7, probiotic group had significantly lower levels of proinflammatory cytokines (interleukin-6 [80 vs 186 pg/mL, p = 0.001]; interleukin-12p70 [44 vs 79 pg/mL, p = 0.001]; interleukin-17 [217 vs 293 pg/mL, p = 0.01]; and tumor necrosis factor-α [192 vs 348 pg/mL, p = 0.01]) and higher levels of antiinflammatory cytokines (interleukin-10 [320 vs 240 pg/mL, p = 0.02] and transforming growth factor-β1 [311 vs 221 ng/mL, p = 0.01]) than placebo group. From day 1 to 7, probiotic group showed significant decrease in proinflammatory cytokines (interleukin-6 [196-80 pg/mL, p = 0.001]; interleukin-12p70 [71-44 pg/mL, p = 0.01]; interleukin-17 [258-217 pg/mL, p = 0.01]; and tumor necrosis factor-α [347-192 pg/mL, p = 0.001]) and increase in antiinflammatory cytokines (interleukin-10 [198-320 pg/mL, p = 0.001] and transforming growth factor-β1 [216-311 ng/mL, p = 0.001]) as compared to placebo group. Sequential Organ Failure Assessment score on day 7 was significantly less in probiotic group (1 vs 3). There was a nonsignificant trend toward lower incidence of healthcare-associated infections (14% vs 20%) and duration of ICU stay (6.5 vs 9 d) in probiotic group. Mortality was similar in two groups., Conclusions: Probiotics supplementation for 7 days resulted in significant decrease in proinflammatory and increase in antiinflammatory cytokines in children with severe sepsis.

Competing Interests: There are no conflicts of interest.

Objective: To assess pulmonary functions of children who received mechanical ventilation for acute hypoxemic respiratory failure., Design: Longitudinal study., Setting: PICU and Pediatric Pulmonology Clinic of a tertiary care teaching hospital in North India., Patients: All children, 5-12 years old, ventilated for acute hypoxemic respiratory failure in PICU from July 2012 to June 2013 and survived., Interventions: The baseline admission variables recorded were as follows: age, sex, duration of illness, primary diagnosis at admission, Pediatric Risk of Mortality III score, lung injury score, mechanical ventilation parameters, oxygenation indices, and duration of PICU stay. The children were followed up twice, at 3 and 9-12 months, after discharge from PICU and evaluated for any residual respiratory symptoms and signs, pulse oximetry, chest radiograph, 6-minute walk test, peak expiratory flow rate, and spirometry. Age, sex, duration of illness, primary diagnosis, Pediatric Risk of Mortality III score, lung injury score, mechanical ventilation parameters, oxygenation indices (PaO2/FIO2 ratio and oxygenation index), and duration of PICU stay were recorded from patient records., Measurements and Main Results: Twenty-nine children (25 boys and four girls; mean [SD] age, 8.4 [2.4] yr) were followed up at 3.5 (± 1.2) and 10.6 (± 2.7) months after discharge from PICU. Recurrent respiratory symptoms were noted in 37.9% patients (11/29) during first and in none during second follow-up. None had limitation of physical activity or need of supplemental oxygen. Chest examination was normal in all, except one during first follow-up, but 13.8% (4/29) had abnormal chest radiograph during first follow-up. Nearly all children could perform 6-minute walk test although mean distance walked increased significantly from first (352 ± 66.7 m) to second follow-up (401 ± 60.7 m; p = 0.002). Abnormal spirometry was seen in 82.7% (24/29) versus 18.5% (5/27) children during first and second follow-up visits, respectively (p = 0.0001). Most cases had restrictive abnormality (58.6% vs 11.1%; p = 0.002) during first and second follow-up, respectively. There was no correlation between pulmonary functions and lung injury scores, oxygenation indices (PaO2/FIO2 ratio and oxygenation index), and mechanical ventilation parameters., Conclusions: Significant number of children ventilated for acute hypoxemic respiratory failure had subclinical pulmonary function abnormality, without limiting physical activity, which improved over time. Further research on this topic with a larger sample size and patient categorization according to recent pediatric acute respiratory distress syndrome definition is needed.

Objectives: To evaluate pediatric intensive care unit (PICU) needs, outcome and predictors of mortality in post-neonatal tetanus., Materials and Methods: Review of 30 consecutive post-neonatal tetanus cases aged 1 months to 12 years admitted to a PICU in north India over a period of 10 years (January 2006 to December 2015)., Results: Chronic suppurative otitis media was the commonest portal of entry. All received tetanus toxoid, human tetanus immunoglobulin (HTIG) and appropriate antibiotics; 7 (23.3%) received intrathecal HTIG. Common complications were respiratory failure, rhabdomyolysis, autonomic dysfunction, acute kidney injury and healthcare-associated infections. PICU needs were as follows: ventilation; benzodiazepine, morphine and magnesium sulfate infusion; neuromuscular blockers, inotropes, tracheostomy and renal replacement therapy. Mortality rate was 40%; severity Grade IIIb, autonomic dysfunction, use of vasoactive drugs and those who did not receive intrathecal HTIG were significantly associated with mortality., Conclusion: Post-neonatal tetanus is associated with high mortality, and PICU needs include management of spasms, autonomic dysfunction and complications and cardiorespiratory support., (© The Author [2017]. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com)

Competing Interests: There are no conflicts of interest.

Competing Interests: There are no conflicts of interest.

Hypoparathyroidism leading to status dystonicus is rarely reported in literature. The authors present an 8-y-old girl with idiopathic familial hypoparathyroidism who presented with status dystonicus. She was managed successfully with midazolam infusion, calcium and vitamin D supplementation, and oral anti-dystonia drugs.

Background: The data on the prevalence of vitamin D deficiency (VDD) in apparently healthy children from India is limited., Objective: To assess the prevalence of VDD in apparently healthy children., Design: Prospective study., Setting and Subjects: Apparently healthy children from the age groups of 3 months-12 years, from the upper socioeconomical status (USES), attending the outpatient department (OPD) of a private pediatric hospital in Chandigarh, India, for minor ailments were enrolled over a period of 6 months (March-August 2013)., Methods: Plasma levels of 25-hydroxyvitamin D [25(OH)D] were measured by competitive electrochemiluminescence immunoassay., Results: In total, 338 children (188 boys, 150 girls) with mean age of 3.31 years were enrolled. The percentages of children with deficient, insufficient, and sufficient levels of 25(OH)D were 40.24%, 25.44%, and 34.32%, respectively. Clinical signs of VDD were seen in only 8.53% of the children. The mean (±SD) levels of 25(OH)D were 27.48 (15.99) ng/mL. On univariate analysis, deficient levels of 25(OH)D were associated with relatively younger age group, female sex, failure to thrive, exclusive breastfeeding, inadequate sun exposure, and no vitamin D supplements., Conclusion: A high prevalence of clinical and biochemical VDD was noted in apparently healthy children belonging to the USES.

Background: Data on the micronutrient levels in children with cystic fibrosis (CF) are not available from developing countries, wherein the nutritional profile of children is quite different from that of Western countries., Methods: Levels of fat-soluble vitamins (A, D, and E) and trace metals (iron, copper, and zinc) were measured in 27 CF cases and 27 controls., Results: CF cases had significantly low levels of all studied micronutrients compared with controls, and the levels were even lower in cases with exacerbation than in stable CF cases. Prevalence of deficiency of vitamin D, vitamin E, iron, copper, and zinc was significantly higher in cases than in controls, whereas vitamin A deficiency was almost equal in both the groups., Conclusion: The prevalence of deficiency of vitamins A, D, and E and iron, copper, and zinc was high in CF cases, and their levels were significantly lower in cases than controls. CF cases should be regularly monitored for these micronutrients, and appropriate supplementation should be considered.

Objective: Proteus syndrome (PS) is characterized by patchy or segmental overgrowth and hyperplasia of multiple tissues and organs, along with susceptibility to development of tumors. Very few cases are reported in literature from developing countries. Due to certain overlapping features with other overgrowth syndromes, diagnosis is usually delayed. Our aim was to describe clinical profile of this rare condition in six patients., Materials and Methods: Retrospective case sheet review of patients followed in a Pediatric Genetic and Metabolic clinic at a tertiary care institute of North India with a diagnosis of hemihypertrophy/overgrowth syndrome., Results: Six cases presented with asymmetric overgrowth and peculiar features suggestive of PS were included in this study. Age at presentation was 2 months to 10 years; two were males and four were females. Hemihypertrophy was noticed in only one case at birth, and focal overgrowths in rest of other patients were seen later during childhood., Conclusion: Due to certain overlapping features with other overgrowth syndromes, diagnosis of PS is usually delayed. Pediatricians are the first persons who come across such patients and they should be aware about this rare condition.

Neural tube defects are common congenital malformations that could be apparent at birth or manifested in later stages of life. Morbidity is high in anencephaly, whereas in spina bifida, there are neurological and motor disorders. These defects deserve paramount importance in clinical dentistry. Latex allergy, dental caries, difficulty in mouth opening, and sitting in a dental chair are common problems. There is a high risk of anaphylactic response during anaesthesia. There could be associated craniosynostosis causing maxillary deficiency, and malformed sella turcica might be seen. An association of the defects has been linked with orofacial clefts and Down syndrome., (© 2012 Wiley Publishing Asia Pty Ltd.)

Hypoglycemia is the usual feature of commonly occurring organic acidemias. Organic acidemias manifesting as hyperglycemia or diabetic ketoacidosis are rare and only a few cases have been reported. We report a 13-month-old boy who presented with vomiting, dehydration, coma, hyperglycemia, high anion gap metabolic acidosis and ketosis, mimicking diabetic ketoacidosis (DKA). Treatment with parenteral fluid, electrolytes, and insulin infusion resulted in an improvement in hyperglycemia, but persistence of metabolic acidosis and lack of improvement of neurologic status led us to suspect an organic acidemia. Urinary organic acid analysis revealed increased methylmalonic acid levels. In addition, hyperhomocysteinemia and homocystinuria were also noted in presence of normal vitamin B12 levels. This confirmed the diagnosis of cobalamin metabolism defect leading to combined methylmalonic aciduria and homocystinuria. There was some improvement in neurologic status and metabolic parameters after treatment with low-protein diet, vitamin B12, folic acid, and L-carnitine, but he ultimately succumbed to polymicrobial nosocomial sepsis. The entire MMACHC gene of the patient was sequenced and no mutations were identified. This is probably the first case report of cobalamin intracellular metabolism defect (CblC/CblD/CblF/CblJ or ABCD4) presenting as diabetic ketoacidosis.