Potter's sequence is a rare fatal disorder that occurs in sporadic and autosomal recessive forms with an incidence of 1 in 4000 births. Babies born with this condition are either still born or die very early within the neonatal period. We report a case of Potter's sequence with the typical physical findings and histological findings.

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Linear nevus sebaceous syndrome is a multisystem disorder including nevus sebaceous, which is the hallmark of this syndrome, together with central nervous system, ocular and skeletal anomalies. We report a case of extensive skin lesions, CNS and eye anomalies in a full term infant who was conceived by intracytoplasmic sperm injection.

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Sirenomelia also known as the mermaid syndrome, is a rare congenital malformation of uncertain etiology. It is characterized by fusion of the lower limbs and commonly associated with severe urogenital and gastrointestinal malformations. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation.

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Background: Neonatal sepsis is a worldwide problem that presents a management challenge to care groups for neonates and infants. Early diagnosis and management can considerably decrease the risk of sepsis, and improve the outcome. Aim: The aim of the present study was to determine the incidence, causative pathogens, and the antibiotic sensitivity pattern for neonatal sepsis in Iran. Materials and Methods: A historical cohort study was conducted on 84 patients with neonatal sepsis who were admitted to the neonatal intensive care unit (NICU) wards of Baqiyatallah and Najmieh University hospitals in Tehran, between 2003 and 2006. Clinical, demographic and laboratory data was collected from medical records. Results: Among all the comprised neonates, 44 patients were diagnosed with early-onset sepsis, 23 cases with late-onset sepsis and others with nosocomial sepsis. The most common isolated pathogen in all groups was Enterobacter, and was responsible for 31.4%, 47.8% and 41.2% of the episodes of sepsis, according to the sepsis type mentioned above, respectively. Susceptibility of common sepsis related pathogens to imipenem and gentamycin gradually reduced over the years between 2003 and 2006. Total mortality and morbidity rates due to neonatal sepsis were estimated at 27.4% and 89.3%, respectively. Mortality following sepsis was found more in boys (Odds Ratio (OR)=4.897, Conifdence Interval (CI)=95%, P=0.031), and those with low birth weight (OR=4.406, CI: 95%, P=0.011). Higher sepsis related co-morbidity was found in neonates following cesarean delivery (OR=6.280, CI: 95%, P=0.025). Conclusion: It seems that the mortality rate in this study was lower than similar studies in Iran and other developing countries. This difference between the mortality rates of the centers in our study and others could be due to the high occurrence of Enterobacter infections in the latter and also high resistance of these pathogens to commonly used antibiotics such as β-lactams and aminoglycosides reported in other studies.

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7 days old baby was admitted in Kamla Raja Raja Hospital with complaints of swelling on upper limb and lower limb. Polydactly of fingers and toes were present. All systems were normal Lab investigation - Blood examination was normal Ultrasound of abdomen, Echocardiography and CT scan of brain - Normal.

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Bronchopulmonary dysplasia (BPD) is the major cause of pulmonary disease in infants. The pathophysiology and management of BPD changed with the improvement of neonatal intensive care unit (NICU) management and with the increase of survival rates. Despite the improvements made, BPD is still a public health concern, resulting in frequent hospitalizations with high rates of mortality, impaired weight and height growth, and neurodevelopmental disorders. Lung injury in the neonatal period has multiple etiologic factors - genetic, hemodynamic, metabolic, nutritional, mechanical, and infectious mechanisms - act in a cumulative and synergic way. Free radical (FR) generation is largely recognized as the major cause of lung damage. Oxidative stress (OS) is the final common endpoint for a complex convergence of events, some genetically determined and some triggered by in utero stressors. Inflammatory placental disorders and chorioamnionitis also play an important role due to the coexistence of inflammatory and oxidative lesions. In addition, the contribution of airway inflammation has been extensively studied. The link between inflammation and OS injury involves the direct activation of inflammatory cells, especially granulocytes, which potentiates the inflammatory reaction. Individualized interventions to support ventilation, minimize oxygen exposure, minimize apnea, and encourage growth should decrease both the frequency and severity of BPD. Future perspectives suggest supplementation with enzymatic and/or non-enzymatic antioxidants. The use of antioxidants in preterm newborns particularly exposed to OS and at risk for BPD represents a logical strategy to ameliorate FRs injury, but further studies are needed to support this hypothesis.

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Proteus syndrome is a rare hamartomatous disorder affecting multiple tissues and manifesting itself in a variety of ways. The understanding of the complete spectrum of clinical features, the natural clinical course of the disease and the proper management of such a rare but highly variable syndrome depend heavily on experiences gathered by previously reported cases. We present an unusually severely affected and rapidly progressive case of proteus syndrome in a neonate who presented with craniofacial hemihypertrophy, subcutaneous masses, capillary hemangioma, varicose veins, epidermal nevi and macrodactyly. The cranial ultrasonogram revealed unilateral hydrocephalus with partial obstruction of the foramen of monro.

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Background: Thrombocytopenia in hypoxic neonates admitted in NICU is a morbid condition encountered very commonly. Early-onset thrombocytopenia (<72 h) is most commonly associated with fetomaternal conditions complicated by placental insufficiency and/or fetal hypoxia. Chronic intrauterine hypoxia is the most frequent cause of early-onset thrombocytopenia in preterm neonates. Aims: In this study incidence and clinical impact of early thrombocytopenia in hypoxic neonates was investigated. Setting and Design: Neonatal intensive care unit of a tertiary level hospital attached to a medical college in Central India. A cross-sectional, observational hospital based study in hypoxic neonates for development of thrombocytopenia. Materials and Methods: 603 hypoxic newborns were evaluated for development of thrombocytopenia. 155 (25.07%) developed thrombocytopenia and were the cases. Non thrombocytopenic babies 448 (74.29%) served as controls. The two groups were compared for birth weight, sex ratio, gestational age, severity of asphyxia, platelet counts and mortality rate. Statistical Analysis: Descriptive statistics of continuous variable were expressed in mean and SD. P value less than or equal to 0.05 were statistically significant. Results and Conclusions: We found thrombocytopenia to be associated with male gender, prematurity and low birth weight. Most babies had mild to moderate thrombocytopenia. Mortality was higher in preterm thrombocytopenic babies as compared to term. We suggest screening for thrombocytopenia in all asphyxiated newborns, as hypoxia can lead to neonatal thrombocytopenia.

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Ectopia cordis is a rare congenital anomaly. Congenital anomaly scan can detect it at 18-23 weeks of gestation. Four chamber view of the heart in routine fetal anomaly scans at >18 weeks is the most effective technique to detect CHD prenatally.

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Objective: To compare the cord blood ghrelin level in (SGA) infants with the level in appropriate for gestational age (AGA) infants, and determine its relationship to anthropometric measurements at delivery. Materials and Methods: Fifty newborn infants (30 SGA newborns and 20 AGA infants) were included in the study and were subjected to complete clinical examinations, anthropometric measurement, and ghrelin assays. Results: The cord blood ghrelin level in SGA infants was significantly higher than that in AGA infants. Cord ghrelin level correlated negatively with gestational age, weight, length, and body mass index in SGA group. Conclusion: Cord ghrelin concentration increased in SGA infants due to state of prolonged undernutrition the source of ghrelin unknown may be from the mother placenta or fetal tissues.

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Chikungunya virus (CHIKV) infection manifesting in neonates is very rare. The prevalence of the entity was described only recently. We describe a neonate with chikungunya who presented with severe thrombocytopenia and features of multisytem involvement. Identification of this entity based on clinical and epidemiological background helps in appropriate management and aids in prognostication of the affected neonate.

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Mediastinal enteric cysts are relatively uncommon, and patients tend to present at a later age compared to those with duplications in other areas of alimentary canal. The tendency of enteric cyst to enlarge and produce airway obstruction is sufficient reason for early surgical removal. We report on a case of mediastinal enteric cyst in a neonate with respiratory distress for its early presentation and management. The embryological basis and anatomical issues relating to duplication cysts of the gastrointestinal tract is discussed.

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Objective: Phototherapy is the most common treatment used for severe jaundice. There is increasing evidence that phototherapy can directly affect the expression and function of cell surface receptors including adhesion molecules, cytokines, and growth factor receptors. The aim of this study is to investigate the effect of phototherapy use on the levels of interleukin (IL)-1α, IL-6, and tumor necrosis factor (TNF)-α as cytokine expressions from keratinocytes, and also white blood cell counts in the treatment of neonate with hyperbilirubinemia. Materials and Methods: We studied 32 term newborns with hyperbilirubinemia. Blood samples were obtained before and 72 h after phototherapy. Serum levels of IL-1α, IL-6, TNF-α, and WBC count were measured in the samples using appropriate methods. Results: Serum TNF-α at 72 h of exposure to phototherapy increased, while the levels of IL-1α and IL-6 at the same time were decreased. These changes were not statistically significant. WBC counts rose significantly with phototherapy. Conclusion: Phototherapy in term neonate does not affect cytokines' levels, but can raise peripheral WBC count.

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