Multisystem Langerhans cell histiocytosis in an infant: A case report and review of literature

Langerhans cell histiocytosis (LCH) is a rare disease of childhood which originates from marrow-derived immature myeloid dendritic cells of skin and visceral organs with incompletely understood etiopathogenesis. An 11-month-old infant presented with fever, pallor, multiple erythematous, crusted, scaly hypopigmented macules, and shiny colored papules over scalp, forehead, and trunk along with hepatosplenomegaly. Persistent pancytopenia, punched out lesion on a brain scan, and multinucleated giant cells with eosinophilic cytoplasm admixture with eosinophils and lymphocytes on skin biopsy were seen. Immunohistochemistry was positive for CD1a and S100. The patient was treated with vinblastine and steroid, but unfortunately parents did not complete the therapy. A high index of suspicion is necessary to make timely diagnosis and therapy to minimize the frustration felt by parents/patients

Myocardial ischemia in neonate with perinatal asphyxia: Electrocardiographic, echocardiographic and enzymatic correlation

Objective: The objective of this study is to evaluate the presence and severity of cardiac involvement in asphyxiated neonates and toestablished which clinical and laboratory parameters (electrocardiogram [ECG], echocardiography [ECHO], and cardiac enzyme)are reliable predictors of myocardial ischemia. Design: Nested case–control study. Setting: Neonatology unit of tertiary careinstitute. Materials and Methods: The study group consist of 90 term neonates who had APGAR score <7 at 5 min as cases and90 term neonate who had APGAR score >9 at 5 min as controls. On the 2nd day of life (between 24 and 36 h), all neonate underwentECG, ECHO, and enzyme estimation (CK-MB). Result: The cases were divided into three groups according to SARNAT scale andGroup I consist 25, Group II - 54, and Group - III, 11 neonates. The mean gestational age was 36.97±0.35 and 37.12±0.46 week forcases and controls respectively while mean birth weight was 2.79±0.36 kg and 2.68±0.76 kg for cases and controls, respectively.ECG changes were present in 70 (77.7%) of cases. ECG changes of Grade 3 and 4 were present in only Group III neonates andall neonates with Grade 4 changes were died in 1st week of life. Fractional shortening (FS) decreased progressively from Group Ito Group III (p<0.001) and showed a significant difference between cases and controls as well as in different groups except incontrols versus Group I. Peak aortic velocity behaved similarly. Concerning mean acceleration, the significant difference wasobserved between control and cases (all 3 groups). CK-MB activity increased from Group I to Group III and statistically significantdifference was seen in controls and different Groups as well as in between different groups except in Controls versus Group I.Conclusion: Severe ECG changes (Grades 3 and 4), CK-MB elevation and reduced FS can be considered as reliable marker ofmyocardial ischemia in perinatal asphyxia

Macrosomia due to maternal obesity: A case report

Macrosomia, birth weight >4000 g is said to occur in <10% of the pregnancies and is associated with morbidity and mortality in both mother and baby. Although it is more common in diabetic mother, it is also associated with maternal obesity and high-body mass index. We describe the case of near-term male neonate who was delivered by cesarean section and birth weight was 5000 g who developed hypoglycemia and hyperbilirubinemia which was treated successfully

Neonatal Risk Factor for Seizures in Term Neonate: A Hospital - Based Case Control Study

Objective: Assessing the neonatal risk factors for seizure in first 72 hours of life in term neonate.Design: Case control study.Setting: Department of Paediatrics, Neonatology unit tertiary care centre Govt. Medical College, Nagpur.Method: A 210 cases i.e. term neonate with seizure within first 72 hours of life and 210 controls i.e. term neonate without seizure in first 72 hours of life were randomly selected as study subject over a period of two year. Seizures were defined as per standard definition. Data were collected regarding age of onset of seizure, birth weight, Apgar score at 1 minute and 5 minute, type of delivery. Appropriate investigation was done and neonates were followed up till discharge or deathResults: In73% neonate’s subtle seizure was most common and occurs within 48-72 hours of life. Neonates with low birth weight had 0.22 times more risk of seizure than normal weight. Birth asphyxia was most significant risk factor for seizure and had 4.66 times more risk of seizure (P<0.001). Presence of low Apgar score at 1 minute and 5 minute are found to be significant in univariate analysis but in multiple logistic regression analysis it is found that 5 minute Apgar score is more significant than 1 minute. Low Apgar score at 5 minute had 1.29 times more risk of seizure. In study population 7.1% neonates died in case group compared to 1.4% in control group which was statistically highly significant (OR 3.98, 95% CI 1.23-16.7, P<0.0095) and most common cause of death was severe birth asphyxia and sepsis.Conclusion: Early identification and timely intervention of neonatal risk factor may reduces the seizure in term neonate

Study of fetal malnutrition in full-term small for gestational age babies

Introduction: Fetal malnutrition (FM) is a risk factor for increased neonatal morbidities and mortalities worldwide. Clinician’s comes across the challenge to identify small for gestational age (SGA) babies whose health is endangered in utero due to a hostile intrauterine environment and to monitor and intervene appropriately. Objectives: This prospective observational study aimed to know the prevalence of FM and to study the occurrence of adverse perinatal events in term SGA babies. Materials and Methods: The studyenrolled 903 term SGA neonates. Clinical assessment of nutrition (CAN) score was applied and anthropometric evaluation was carried out between 24 and 48 h of birth. Babies with adverse perinatal events were treated according to standard hospital protocol. Results: The prevalence of FM in the present study was 34.21% in term SGA babies. All anthropometric parameters such as birth weight, length, head circumference (HC), and ponderal index (PI) were significantly lower in SGA babies with FM as compared to those without FM. There was significantly higher occurrence of FM in SGA babies. Risk of FM was 3.17 times higher in SGA babies with length <3rd centile and risk 2.07 times higher when HC was <3rd centile. The risk of FM was 4.27 times higher when PI <2.2. When PI was compared to CAN score for FM, it had low sensitivity and specificity. Conclusion: CAN score is the better index for the detection of FM and for predicting high risk of perinatal morbidity in term SGA babies. Our study demonstrates that predicting malnutrition is more meaningful than birth weight alone

Isolated congenital factor VII deficiency

Congenital factor VII (FVII) (proconvertin) is a rare autosomal recessive bleeding disorder. Bleeding manifestations and clinical findings vary widely, ranging from being asymptomatic to life-threatening bleeding. Intracranial bleeding is relatively less common with inherited FVII deficiency than with other coagulation disorders. We report a rare case of congenital FVII deficiency in an 11-year-old male child. The patient had recurrent subdural hemorrhages. The prothrombin time was markedly prolonged with a normal bleeding time, normal partial thromboplastin time and normal platelet count. Treatment consists of replacement therapy with fresh frozen plasma, prothrombin complex concentrates or plasma-derived FVII concentrates, and/or recombinant factor VIIa. Clinical heterogeneity is the hallmark of this disorder

Fracture of humerus in a newborn during cesarean section: A case report

Risk of birth injuries and long bone fractures are common in vaginal breech deliveries but have also occurred in the cesarean section. Here, we report the case of a term neonate who was admitted with swelling and paucity of movement of the right arm with a history of energetic traction during the emergency cesarean section. The diagnosis was confirmed by radiography and managed by plaster of paris slab. Obstetrician and pediatrician should remain vigilant regarding such rare complications so that these cases are not overlooked, as timely intervention and management are necessary to prevent disability and deformities in the future

Short-term safety and beneficial effects of hydroxyurea therapy in children with sickle cell disease

Introduction: Worldwide, sickle cell disease (SCD) is the most common hemoglobinopathy among which SS pattern is more common. Although hydroxyurea (HU) is approved by the Food and Drug Administration for the treatment of recurrent moderate-to-severe painful crises in pediatric sickle cell anemia, there is a fear of toxicities. Objectives: The objectives of the study were to evaluate the short-term safety and beneficial effects of low-dose HU therapy in SCD (SS pattern) children. Materials and Methods: This prospective cohort study enrolled 40 cases of severe SCD and started HU in a fixed dose of 10 mg/kg/day. During follow-up, cases were evaluated for compliance of HU, its toxic effects and adverse events from their histories, clinical examinations, and laboratory parameters. Furthermore, beneficial effects of HU therapy were evaluated by assessing blood transfusion rate, frequency of painful events, strokes, acute chest syndrome, avascular necrosis of femur, and estimation of hemoglobin F (HbF) level after 2 years of therapy. HU was discontinued temporarily if any toxicity or minor adverse drug events occurred during therapy and was restarted at the same dose after normalization of deranged laboratory parameters. Results: The clinical adverse drug events seen were nausea (8.33%), diarrhea (2.78%), and hematuria (2.78%). The most common hematological toxicity was anemia and thrombocytopenia. Renal and hepatic toxicities were transient in nature. The mean acute painful events and blood transfusion rate reduced significantly on HU therapy. It increased Hb and HbF level significantly in SCD children. Conclusion: HU is a safe drug without significant toxicity or adverse events in a dose of 10 mg/kg/day for short duration and it is beneficial in SCD (SS pattern) children in reducing acute painful events and decrease blood transfusion rate

Children’s and adolescents’ rising animal-source food intakes in 1990–2018 were impacted by age, region, parental education and urbanicity

Animal-source foods (ASF) provide nutrition for children and adolescents’ physical and cognitive development. Here, we use data from the Global Dietary Database and Bayesian hierarchical models to quantify global, regional and national ASF intakes between 1990 and 2018 by age group across 185 countries, representing 93% of the world’s child population. Mean ASF intake was 1.9 servings per day, representing 16% of children consuming at least three daily servings. Intake was similar between boys and girls, but higher among urban children with educated parents. Consumption varied by age from 0.6 at <1 year to 2.5 servings per day at 15–19 years. Between 1990 and 2018, mean ASF intake increased by 0.5 servings per week, with increases in all regions except sub-Saharan Africa. In 2018, total ASF consumption was highest in Russia, Brazil, Mexico and Turkey, and lowest in Uganda, India, Kenya and Bangladesh. These findings can inform policy to address malnutrition through targeted ASF consumption programmes.publishedVersio

Incident type 2 diabetes attributable to suboptimal diet in 184 countries

The global burden of diet-attributable type 2 diabetes (T2D) is not well established. This risk assessment model estimated T2D incidence among adults attributable to direct and body weight-mediated effects of 11 dietary factors in 184 countries in 1990 and 2018. In 2018, suboptimal intake of these dietary factors was estimated to be attributable to 14.1 million (95% uncertainty interval (UI), 13.8–14.4 million) incident T2D cases, representing 70.3% (68.8–71.8%) of new cases globally. Largest T2D burdens were attributable to insufficient whole-grain intake (26.1% (25.0–27.1%)), excess refined rice and wheat intake (24.6% (22.3–27.2%)) and excess processed meat intake (20.3% (18.3–23.5%)). Across regions, highest proportional burdens were in central and eastern Europe and central Asia (85.6% (83.4–87.7%)) and Latin America and the Caribbean (81.8% (80.1–83.4%)); and lowest proportional burdens were in South Asia (55.4% (52.1–60.7%)). Proportions of diet-attributable T2D were generally larger in men than in women and were inversely correlated with age. Diet-attributable T2D was generally larger among urban versus rural residents and higher versus lower educated individuals, except in high-income countries, central and eastern Europe and central Asia, where burdens were larger in rural residents and in lower educated individuals. Compared with 1990, global diet-attributable T2D increased by 2.6 absolute percentage points (8.6 million more cases) in 2018, with variation in these trends by world region and dietary factor. These findings inform nutritional priorities and clinical and public health planning to improve dietary quality and reduce T2D globally.publishedVersio