Awareness of Hemophagocytic Lymphohistiocytosis as an Unusual Cause of Liver Failure in the Neonatal Period.

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome that predominantly affects infants from birth to 18 months of age, characterized by fever and multiorgan failure. Liver injury has been rarely reported as a presenting sign in the neonatal period. This study reports a case with HLH in the neonatal period who presented with acute liver failure. CASE PRESENTATION: Herein, a 3-day-old female newborn was admitted with cytopenia, increased liver enzymes, hypofibrinogenemia, and markedly elevated serum ferritin. Hemophagocytosis of bone marrow biopsy confirmed the diagnosis of HLH. The newborn was treated with HLH-2004 protocol, but she finally died from multiorgan failure. CONCLUSION: Growing awareness of HLH as a cause of liver failure in the neonatal period can be associated with early treatment and reduces mortality in this group of patients.

Lack of relationship between cord blood erythropoietin and intraventricular hemorrhage in premature neonates: a controversial result.

PURPOSE: The aim of this study was to assess the incidence and risk factors of intraventricular hemorrhage (IVH) as well as the role of cord blood erythropoietin (EPO) level in predicting the possibility of IVH in premature neonates. MATERIALS AND METHODS: This prospective study included 140 preterm neonates born at hospitals affiliated to Shiraz University of Medical Sciences from May 2014 to April 2015. Complete blood count and cord blood EPO level was measured after birth. Brain ultrasonography was performed at 3 and 7-10 days after birth in these newborns. RESULTS: Brain ultrasonography showed IVH in 8.57% (12/140) until the third day and 20% (28/140) at 7-10 days of life in premature neonates. Early gestational age, low birth weight, low Apgar score, and failure to give prenatal steroid were significant risk factors for developing IVH. The mean level of cord blood EPO was 20.95 ± 21.09 mIU/mL in premature newborns without IVH and 15.82 ± 17.11 mIU/mL with IVH. There was no correlation between the cord blood EPO and IVH in premature newborns. CONCLUSION: Antenatal steroids therapy should be encouraged among women at risk of premature delivery. Our results showed that the cord blood EPO was not correlated with IVH in preterm neonates and further research is required to assess this relationship.

Ultrasonographic Confirmation of Endotracheal Tube Position in Neonates.

OBJECTIVE: To compare endotracheal tube tip-to-carina distance obtained by ultrasonography vs. that obtained by chest X-ray in neonates. METHODS: After endotracheal intubation of 40 neonates, chest X-ray and, within one hour, ultrasonography was obtained for each patient for measurement of endotracheal tube tip-to-carina distance. RESULTS: Means of endotracheal tube tip-to-carina distances were not significantly different by both modalities (mean difference 0.157 cm, P= 0.06). In addition, an intraclass correlation was observed between them (r2= 0.61, 95% CI= 0.26, 0.79). CONCLUSION: Ultrasonography and chest X-ray are equally accurate for determination of endotracheal tube tip-to-carina in infants. As ultrasonography is more easily available and is safer than X-ray, it may be a better modality for confirming proper placement of endotracheal tube in neonates.

DRY CUPPING IN CHILDREN WITH FUNCTIONAL CONSTIPATION: A RANDOMIZED OPEN LABEL CLINICAL TRIAL.

BACKGROUND: As a common disease in pediatrics, constipation poses a high burden to the community. In this study, we aimed to investigate the efficacy of dry cupping therapy (an Eastern traditional manipulative therapy) in children with functional constipation. MATERIALS AND METHODS: One hundred and twenty children (4-18 years old) diagnosed as functional constipation according to ROME III criteria were assigned to receive a traditional dry cupping protocol on the abdominal wall for 8 minutes every other day or standard laxative therapy (Polyethylene glycol (PEG) 40% solution without electrolyte), 0.4 g/kg once daily) for 4 weeks, in an open label randomized controlled clinical trial using a parallel design with a 1:1 allocation ratio. Patients were evaluated prior to and following 2, 4, 8 and 12 weeks of the intervention commencement in terms of the ROME III criteria for functional constipation. RESULTS: There were no significant differences between the two arms regarding demographic and clinical basic characteristics. After two weeks of the intervention, there was a significant better result in most of the items of ROME III criteria of patients in PEG group. In contrast, after four weeks of the intervention, the result was significantly better in the cupping group. There was no significant difference in the number of patients with constipation after 4 and 8 weeks of the follow-up period. CONCLUSION: This study showed that dry cupping of the abdominal wall, as a traditional manipulative therapy, can be as effective as standard laxative therapy in children with functional constipation.

Attitudes of medical students and staff toward organ donation in cases of brain death: a survey at Shiraz University of Medical Sciences.

CONTEXT: Organ transplant is one of the most important management strategies for end-of-life patients. The demand for organs in patients awaiting transplant is increasing, and many of these patients die before a donor is found. OBJECTIVE: To determine the attitudes of medical students and staff at clinical institutions affiliated with a large medical university in the Eastern Mediterranean region toward organ donation in cases of brain death. PARTICIPANTS: A total of 500 medical students, physicians, and nurses recruited at hospitals and medical centers affiliated with Shiraz University of Medical Sciences in Shiraz, Iran.Design and Setting-Information about participants' demographic characteristics, knowledge of organ donation, and willingness to donate their own organs after death was collected by using self-administered questionnaires. RESULTS: Most participants (78%) had favorable attitudes toward donating their own organs after brain death. However, only about 25% of them carried an organ donation card. In addition to public media, the main sources of information about organ donation after brain death were their professors and textbooks. An association in charge of improving public awareness and facilitating the process of registration and issuance of donation cards appears to be necessary.

Low postprandial circulating inactive ghrelin: role of early satiety in undernourished children.

OBJECTIVE: To determine difference in the levels of circulating inactive ghrelin between undernourished and healthy children. METHODS: The present cross-sectional study was conducted in undernourished children from southwestern Iran, from July 2011 through July 2012. Postprandial inactive ghrelin levels were measured in 40 undernourished children and sex- and age-matched healthy controls by enzyme immunoassay. RESULTS: The levels of postprandial inactive ghrelin were considerably lower in undernourished compared to the healthy children (6.4 vs. 12.9, P < 0.001). Among the undernourished children, the level of inactive ghrelin was significantly lower in girls than in boys (5.8 vs. 7.1, P = 0.032). CONCLUSIONS: Thus, the levels of inactive ghrelin was found to be low in undernourished children compared to healthy children. Early loss of appetite might be a result of low circulating inactive ghrelin levels in the postprandial state in undernourished children.

Beneficial effect of cyproheptadine on body mass index in undernourished children: a randomized controlled trial.

OBJECTIVE: Cyproheptadine hydrochloride (CH) is a first-generation antihistamine which is used as an appetite stimulant. This study was designed to identify the role of CH therapy on weight gain, linear growth and body mass index in children with mild to moderate undernutrition. METHODS: Eighty-nine patients were enrolled. The present randomized, double-blinded controlled trial included 77 evaluable patients, aged 24-64 months with undernutrition. The patients were randomized to receive cyproheptadine with multivitamin, or multivitamin over a period of four weeks. The weight, height and body mass index were measured at the baseline, four weeks after intervention and four weeks after discontinuation. FINDINGS: A significant higher body mass index was observed among CH-treated patients after 8 weeks intervention with cyproheptadine compared with the control group (P<0.041). Mean weight gain after eight weeks was 0.11 kg in the control group and 0.60 kg in the CH group. There were no significant differences in changes of weight and height velocity across the study between CH-treated and control group at the end of study. CONCLUSION: In our study, cyproheptadine promotes increase in body mass index in children with mild to moderate undernutrition after four weeks treatment.

Clinical and genetic features in patients with cystic fibrosis in southwestern iran.

OBJECTIVE: Cystic fibrosis (CF) is a common autosomal recessive genetic disease caused by a mutation in the CF transmembrane conductance regulatory (CFTR) gene. This study attempted to identify the most common CFTR mutations and any correlations between certain mutations and the clinical presentation of the disease in CF patients in southwestern Iran. METHODS: Twenty nine common CFTR gene mutations were examined in 45 CF patients. FINDINGS: Chronic cough, intestinal obstruction, dehydration, heat exhaustion and steatorrhea were the most common early clinical symptoms among our patients. The most common mutation was ΔF508, with an allele frequency of 21%. The homozygous ΔF508 mutation was observed in eight patients (18%), and three patients (7%) were ΔF508 carriers. The 2183AA > G mutation was observed in four patients, one of whom was also a ΔF508 carrier. The R1162X mutation was detected in two patients. The G542X, R334W and N1303K mutations were detected each in one patient, the first of whom was also a ΔF508 carrier. CONCLUSION: Out of 45 patients, 27 (60%) had none of the CFTR gene mutations we tested for. The most frequent mutations in southwestern Iranian patients with CF should be identified by sequencing the entire CFTR gene in order to optimize the design of a diagnostic kit for common regional mutations.