Haemophagocytic Lymphohistiocytosis in a Newborn Infant Presenting with Cholestasis: Case Report

Hemophagocytic lymphohistiocytosis (HLH) is a rare, fatal disease. Neonatal cholestasis exhibits symptoms similar to those seen in several newborn diseases. HLH is rapidly fatal; therefore, an effective and prompt differential diagnosis is vital. A 10-hour-old newborn with icterus was referred to our clinic. Laboratory examination revealed direct bilirubinemia and pancytopenia, and cholestasis developed. HLH findings were observed in the bone marrow aspiration. Induction chemotherapy as described in the HLH-2004 protocol was initiated. Despite notable improvement in clinical signs and laboratory findings, the infant died, probably due to sepsis, one week after start of chemotherapy HLH should be kept in mind in the differential diagnosis of all cholestatic patients with recently developed cytopenia. For definitive diagnosis of HLH, clinical signs and laboratory findings of the patient should be evaluated, hyperferritinemia and hypertriglyceridemia should be searched and bone marrow aspiration materials should be examined carefully

Influence of smoking on human milk tumor necrosis factor-alpha, interleukin-1 beta, and soluble vascular cell adhesion molecule-1 levels at postpartum seventh day

WOS: 000271811000011PubMed: 19438832Background: The aim of this study was to investigate the effect of maternal smoking during pregnancy on human milk interleukin-1 beta, tumor necrosis factor-alpha (TNF-alpha) and soluble vascular cell adhesion molecule-1 levels at the postpartum seventh day. Methods: Forty-four mothers (age range: 21-34 years) were enrolled in the study. Mothers were interviewed and classified according to their smoking status into one of two groups: the smoking mothers (n = 21) and the nonsmoking mothers (n = 23). Results: There were no significant differences between study groups with respect to human milk interleukin-1 beta (P = 0.12) and soluble vascular cell adhesion molecule-1 levels (P = 0.83). However, TNF-alpha levels were found to be significantly lower in the smoking mothers compared with the controls (P = 0.002). Conclusion: This study shows that maternal smoking during pregnancy affects the levels of TNF-alpha in milk. The protective effect of human milk against infections seems to be impaired in smoking mothers

Early abdomino-perineal pull-through vaginoplasty

Vaginal agenesis is known as one of the Mόllerian anomalies. Mόllerian anomalies occur during gonadal development and differentiation, and may lead to complex outcomes. McKusick-Kaufman syndrome (MKS) is an autosomal recessive multiple malformation syndrome characterized by Mόllerian anomalies with hydrometrocolpos (HMC) and postaxial polydactyly (PAP). We report a case of a female newborn that developed hydronephrosis and respiratory distress due to compression of the cystic mass and underwent surgery on postpartum day 3. Pull-through vaginoplasty was performed with an abdomino-perineal approach. We report the unique treatment approach in this case

Assessment of aflatoxin M1 in maternal breast milk in Eastern Turkey

Breastfeeding may be considered as a risk factor for aflatoxin M1 (AFM1) exposure in early infancy. Hence, AFM1 levels in maternal breast milk (MBM) and the correlation between moldy cheese consumption of lactating mothers and infant exposure to AFM1 were investigated in this study. MBM samples from 73 lactating women randomly selected in hospitals located in Eastern Turkey were analysed for the presence of AFM1 using competitive ELISA. Out of 73 lactating mothers, 44 of them had the habit of cheese consumption (at least once a week), while remaining 29 had no such habit. AFM1 was detected in MBM of 18 out of 73 samples (24.6%); 12 MBM of 44 lactating mothers with moldy cheese consumption habit (27.2%) and 6 MBM of 29 mothers with no such habit (20.6%) with the range of 1.3-6.0 ng/l. None of the samples exceeded the limit set by EU and Turkish legislations. Moldy cheese consumption habit of lactating mothers exhibited no significant correlation with the presence of AFM1 in their milk (p > 0.05). The results indicated that the relative risk of infant AFM1 exposure via MBM of moldy cheese consuming mother was not higher than MBM of mothers with no such habit. (C) 2014 Elsevier Ltd. All rights reserved

Perinatal Chicken Pox (Varicella Zoster Virus) Infection

Chickenpox is due to infection with the varicella zoster virus (VZV), a human alphaherpervirus found worldwide. Classically, the cinical disease is a febrile illness with a pruritic vesicular rash. Maternal chickenpox between 5 days before delivery to 2 days after delivery (perinatal varicella) can cause severe and even fatal illness in the newborn. A 7-day old girl baby presented on day 4 of postnatal with the complaints of widespread vesicular rash and non-suckling. Mother of the baby also had a similar eruption four day prior to delivery, which was clinically characteristic of varicella. Considering history and clinical presentation, a diagnosis of perinatal chickenpox was considered and the baby was treated with acyclovir which she responded and recovered. Herein, the clinical feasures and treatment of chickenpox infection in the perinatal period have been emphasized with this case report. [Cukurova Med J 2013; 38(2.000): 311-314

A CASE OF DILATED CARDIOMYOPATHY DUE TO HYPOCALCEMIA IN NEWBORN

Dilation of one or both ventricles and systolic dysfunction occur dilated cardiomyopathy. Hypocalcemia is a rare cause of reversible heart failure and dilated cardiomyopathy. A seventeen-day old male infant was brought to our clinic with complaints of poor feeding and convulsion. On physical examination, the patient had worse general appearance and was apathetic. Examination of cardiovascular system revealed 2/6 diastolic murmur and signs of congestive heart failure. The value of serum calcium was 4.8 mg / dL. Cerebrospinal fluid examination was unremarkable. Echocardiographic evaluation showed enlarged heart chambers and globally hypokinetic left ventricular wall. Left ventricular ejection fraction was 39% and shortening fraction was 17%. After the treatment of calcium gluconate, a clinically dramatic improvement was observed. On the second day of hospitalization, the echocardiographic examination was normal. Therefore, we suggest that sermon calcium level in newborn with cardiomyopathy should be measured

Prognostic value of amplitude-integrated electroencephalography in neonates with hypernatremic dehydration

Conclusion: The follow-up of newborns after discharge is key to determine the risks associated with hypernatremic dehydration. Our results suggest that hypernatremic dehydration had no impact on the long-term outcome. In addition, continuous aEEG monitoring could provide information regarding early prognosis and mortality

Phototherapy causes a transient DNA damage in jaundiced newborns

In this study, we aimed to clarify the following questions: 1) Does phototherapy (PT) cause genotoxicity in full-term newborn babies undergoing PT as a result of neonatal jaundice?, 2) if genotoxic effect occurs, is there any relationship between the duration of PT and genotoxicity?, and 3) is genotoxic effect temporary or not? The frequency of sister chromatid exchange (SCE) was determined in jaundiced newborns before, during, and after phototherapy, then determined again in childhood (approximately 3.5 years old). Mean frequency of SCE of 22 full-term jaundiced babies significantly increased during the PT procedure and in every single day, compared to the previous day, in comparison to the pre-PT basal value (6.20 +/- 0.57;); mean SCE frequencies at 24, 48, 72, and 96 hours were 7.75 +/- 0.40, 8.16 +/- 0.47, 8.50 +/- 0.40, and 9.36 +/- 0.55, respectively (all P-values < 0.01). In childhood, no significant difference was found between the mean SCE value (4.9 +/- 0.9) of 20 of 22 children, who received PT in the neonatal period, and the mean SCE value (4.7 +/- 0.6) of 20 coevaluated healthy children (P = 0.40). This study demonstrates that the negative effect of PT on SCE is a temporary effect

Repeated Courses of Oral Ibuprofen in Premature Infants with Patent Ductus Arteriosus: Efficacy and Safety

BACKGROUND: There are limited data about the results of repeated oral ibuprofen (OIBU) treatment. This study aimed to describe patent ductus arteriosus (PDA) closure rates and adverse events after repeated courses of OIBU in premature infants with PDA. METHODS: Preterm infants with hemodynamically significant (hs)PDA were enrolled in the study. If the first course of OIBU treatment failed, a second and, if required, third course was administered. RESULTS: A total of 100 patients received OIBU. In six patients, treatment could not be completed due to death (n=3) and side effects (n=3). In three patients, adverse effects related to OIBU (thrombocytopenia and impairment of renal function) developed during the first course. During the second and third courses, no new adverse event occurred. After all courses, the PDA closure rate was determined as 88%. The rate was 71% after the first course, 40% after the second course, and 35% after the third course. Although the second course resulted in a significant increase in the closure rate (p0.05). The mean postnatal age at the start of the first dose of OIBU was not significantly different among the responders and non-responders to the first course (p>0.05). Clinical characteristics did not affect the closure rate significantly. The number of courses did not have a significant effect on death, when gestational age and birth weight were used as covariates [p=0.867, Exp(B)=0.901, 95% confidence interval=0.264-3.1]. CONCLUSION: A second course of OIBU seems effective and safe for use in preterm infants with hsPDA. Although a third course of OIBU results in PDA closure in some additional patients, the difference is not significant. Thus, surgical ligation should be considered after the second course, especially in patients with signs of severe heart failure