Approximately 10% of neonates with Down syndrome may develop a form of megakaryoblastic leukemia that usually disappear spontaneously during the first months of the life. Although it seems to have a benign course, it may also be lethal and severe in some cases, especially in the form of hydrops and/or cardiopulmonary failure. Herein, we report a male infant with Down syndrome who was admitted with respiratory distress due to severe pericardial effusion leading to pericardial tamponade in the first 2 weeks of life. Pericardiosentesis and pericardial tube replacement in combination with steroid therapy was performed. He responded well to these therapies and his leukemia resolved on the fourth month of life. This case suggests that severe pericardial effusion and pericardial tamponade may be life-threatening complications of transient leukemia of Down syndrome and also it may be managed successfully with appropriate treatments.

Key words familial Mediterranean fever, protracted febrile myalgia, streptococcus.Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent self-limited attacks of fever and inflammatory serositis. It occurs most often in the Mediterranean region, affecting mainly people of Jewish, Turkish, Arabic and Armenian ancestry. 1 FMF is associated with several types of vasculitis, including polyarteritis nodosa (PAN), HenochSchönlein purpura (HSP) and Behçet's disease. The clinical spectrum of FMF has recently been expanded and protracted febrile myalgia (PFM) is now a frequently recognized component in these patients. [2][3][4] PFM is characterized by severe paralyzing myalgia, high fever, abdominal pain, arthritis/arthralgia, and transient vasculitic rashes mimicking HSP. PFM develops usually in patients with known FMF, even under colchicine prophylaxis. Sometimes, FMF can be diagnosed with PFM as a first manifestation. [2][3][4] We describe here two children with FMF-associated protracted febrile myalgia. The first patient presented with PFM and was subsequently diagnosed as having FMF based on clinical features and genetic analysis. The second was a patient with known FMF. Case Reports Case 1An 8-year-old boy was admitted with abdominal pain, high fever, arthralgia and myalgia for two weeks. He was unable to walk without help due to the myalgia. His medical history revealed abdominal pain attacks with fever and/or recurrent febrile episodes every three to four months for the last two years. His parents were not relatives and there was no family history of autoimmune or rheumatic diseases.Physical examination showed normal anthropometric development, normal blood pressure (100/60 mmHg), fever (38.7°C), tachycardia, diffuse abdominal tenderness and severe muscular tenderness in all extremities. The rest of the physical examination was normal.Laboratory investigations revealed normal urine analysis, leukocytosis (17 600/mm 3 ) with neutrophil predominance (76%), thrombocytosis (598 000/mm 3 ), hyperglobulinemia (2.76 g/dL), normal serum glucose, urea, creatinine, electrolytes, transaminases and creatine phosphokinase (CPK). Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and antistreptolysin-O (ASO) were elevated to 98 mm/h, 260 mg/L (normal, <6 mg/L) and 509 Todd unit (normal, <166 TU), respectively. Serology for brucellosis, salmonellosis, hepatitis B (HBV), hepatitis C (HCV), toxoplasmosis and cytomegalovirus (CMV) were negative. Antinuclear antibody (ANA), anti-double stranded DNA (anti-ds DNA), C 3, C4, rheumatoid factor (RF), anti-cardiolipin IgG/IgM, perinuclear-antineutrophil cytoplasmic antibody (p-ANCA), and cytoplasmic-antineutrophil cytoplasmic antibody (c-ANCA) were normal. Blood, urine and throat cultures were negative for pathogenic bacteria. There was no occult blood in the stool. Chest X-ray and abdominal ultrasonography were normal.Severe abdominal pain persisted at the region of the epigastrium and an esophagogastroduodenoscopy was carried out. It r...

The aim of this study was to evaluate the importance of polymorphonuclear leucocyte (PMN) elastase as an early indicator and follow-up parameter in neonatal sepsis. Material and Methods: The study group consisted of forty patients with the diagnosis of sepsis and the control group included twenty newborn. Inclusion criteria were formerly sought in our subjects who were diagnosed using the Töllner scoring system and based on the clinical observations and laboratory findings. The results of white blood cell and platelet counts, immature/total neutrophil ratio, CRP and PMN elastase values were evaluated in both groups. Enzyme linked immunoassay methods were used to determine the PMN elastase levels. Results: The mean PMN elastase level was found to be 145.1±34.6 ng/mL in patients with neonatal sepsis and 75.5±9.8 ng/mL in healthy subjects (p<0.001). When the plasma PMN elastase levels were compared between both groups, the specificity was %96.3, sensitivity was %94.6, negative estimation value was %93.5 and positive estimation value was %95.1. Conclusion: These findings indicate that PMN elastase level is a major indicator for the early diagnosis of newborn sepsis.

Acute scrotal abscess is a rare condition in neonates. Most of these abscesses were reported to be unilateral and caused by Staphylococcus and Salmonella spp. Herein, we report a bilateral scrotal abscess in a preterm infant and Candida albicans was isolated from the scrotal fluid culture. To our knowledge, this is the first bilateral scrotal abscess in a preterm infant caused by C. albicans. Therefore, this organism must be suspected in differential diagnosis of acute scrotal abscess in neonates, especially in preterm infants.

Objectives The rehospitalization frequency/indications of low birth weight (LBW) preterms and the effect of rehospitalization on growth and neurodevelopment were investigated. Methods LBW preterms discharged from NICU were prospectively followed until the corrected age of 1 year. Infants rehospitalized after discharge were defined as the study group and those not rehospitalized as the control group. The frequency, duration and etiology of rehospitalization were investigated and the effects of neonatal complications, surgery and socio-demographic status on rehospitalization were assessed. Results The study and the control group included 113 and 217 infants, respectively. Infants in the study group were rehospitalized 247 times in total. Rehospitalization was significantly higher in the male gender (39.7% vs. 28.9%, p &lt; 0.05). Hyperbilirubinemia-anemia, anemia-surgery and pulmonary-other infections were the most common indications for rehospitalization in the 0–14 days, 15 days to 2 months and 2–12 months, respectively. Intrauterine growth had no impact on rehospitalization. Somatic growth and neurodevelopment were significantly delayed in the study group (p &lt; 0.05). Conclusion Birth weight and gestational week are the most important determinants of rehospitalization. Rehospitalized preterm infants have significant deficits in both somatic growth and neurodevelopment despite high-quality follow-up care.

Bu çalışmada yenidoğan yoğun bakım ünitemizin Nekrotizan Enterokolit (NEK) sıklığını, anne ve bebeğe ait risk faktorlerini ve bu faktörlerin NEK gelişimine ve mortaliteye etkilerini değerlendirmeyi amaçladık.

Abstractsgestation and/or < 1500 gram were included. Maternal and neonatal demographic data were all recorded. The plasma Ca, P, ALP and 25-OH vitamin D levels of mothers and infants were evaluated. The neonatal morbidities, duration of hospitalizati, on and total parenteral nutrition were also recorded. Infants were evaluated at postnatal 40th week. Bone mineralization was assesed by plasma Ca, P, ALP, urea, creatinine and GGT levels in combination with femur X-ray. Also, urine was collected to determine urinary Ca and P levels and tubulary phosphate reabsorption was calculated. Results No significant differences were detected between infants with and without osteopenia of prematurity in terms of maternal biochemical values. On the postnatal 40th week, infants with TPR>95% had significantly higher ALP and lower P levels compared with those who had lower TPR. The sensitivity, specificity, positive predictive value and negative predictive value of TPR ratio in diagnosis of prematurity of osteopenia were found to be 27.2%, 82.7%, 17.1% and 89.6%, respectively. Conclusion In conclusion, TPR ratio can be used as an ancillary diagnostic marker in addition to primary diagnostic tests in diagnosis of prematurity of osteopenia. Transient hypothyroxinemia without elevated thyroid-stimulating hormone (TSH) levels is common in prematurity, especially in verylow-birth weight (VLBW) infants. The transient hypothyroxinemia of prematurity (THOP) has been seen as a "benign" condition. Infants were classified as THOP by low thyroxine (T4) value without elevated TSH value (<20 µIU/mL). Primary hypothyroidism (PH) defined by low thyroxine (T4) and elevated thyroid-stimulating hormone (TSH) levels. Both of them can be seen at premature infants. HYPOTHYROXINEMIA VS HYPOTHYROIDISM IN VERY LOW BIRTH WEIGHT INFANTSRetrospectively we compared the premature infants born at ≤32 weeks who required thyroxine supplementation for THOP and hypothyroidism. 24 neonates required thyroxine supplementation for THOP and 18 neonates for PH were included the study between January 2008 and December 2010.There were no statistically differences in respect to demographic and prenatal characteristics between two groups. There was mild positive correlation between free T3, free T4 levels and gestational age. Median starting time of thyroxine supplementation was 13 days in PH and 21 days in THOP group (p=0.014). There were no statistically differences between groups in respect to birth-weight, hospitalization time, sepsis, NEC, PDA, and RDS rates. Although the THOP group started the thyroxine supplementation late, median weight of the neonates at discharge were significantly higher in THOP group (1774 vs 2070 p=0,018). Weight gaining per day after the thyroxine supplementation was significantly higher than the days before supplementation started (p=0.001).Infants who get enough calories but not satisfactory gaining weight should be screened for THOP and PH. VRE was isolated in 12 (1.6%) of the neonates. 6 of the neonates (50%) were born in public hospitals a...

Abstractspooled data was evaluated; of the NICU risk factors only mechanical ventilation had an impact on BAYLEY-II scores (p<0.05). Conclusion Knowing the rate of neurodevelopmental follow up of these patient group could be important when an early individualized management is needed. NEUROCOGNITIVE OUTCOME AT 3 YEARS IN EXTREMELY LOW BIRTH WEIGHT INFANTS (VLBW) WITH CEREBELLAR HEMORRHAGE (CH)