Coexistence of Kasabach-Merritt Syndrome and placental chorioangioma in a premature infant.

Kasabach-Merritt syndrome is a rare life-threatening clinical presentation in neonatal period. it is characterized by giant hemangioma and serious thrombocytopenia. The diagnostic criteria include: 1) hemangiomas on skin, 2) thrombocytopenia or coagulopathy, 3) hemangioma on internal organs diagnosed by ultrasonography, computed tomography or magnetic resonance imaging, and 4) excluding reasons, such as idiopathic thrombocytopenic purpura or hypersplenism.Placental chorioangiomas are the most widespread non-trophoblastic benign tumor-like lesions of placenta. The clinical signs are associated with tumor size. Chorioangiomas larger than 4-5 cm may lead to various maternal and fetal complications.Here, a female premature infant was diagnosed with placental chorioangioma and liver hemangioma during antenatal period. She developed heart failure secondary to non-immune hydrops fetalis in the neonatal period. The atypical giant hemangioma and coagulopathy suggested the diagnosis of Kasabach-Merritt syndrome. The macroscopic and histopathological examination of the placenta confirmed the diagnosis of chorioangioma. The patient died due to purpura fulminans despite the treatment with prednisolone and propranolol that was started on the second day of life. We are presenting this rare case where placental chorioangioma leading to non-immune hydrops fetalis co-existed with Kasabach-Merritt syndrome.

Procalcitonin measurement at 24 hours of age may be helpful in the prompt diagnosis of early-onset neonatal sepsis.

BACKGROUND: The clinical signs of early-onset neonatal sepsis (EONS) are nonspecific and indistinguishable from those of noninfectious disorders. The early diagnosis of EONS is difficult, but is essential to improve outcomes. The aim of this study was to determine the diagnostic value of procalcitonin (PCT) at birth and at 24h of age in the prompt diagnosis of EONS. METHODS: The patient group consisted of neonates with a Töllner score of ≥ 10 or a Töllner score of 5-10 but with the presence of prolonged rupture of the membranes (> 18 h) or chorioamnionitis or maternal fever (n=171). The control group (n=89) comprised neonates admitted to the neonatal intensive care unit for different disease entities. Procalcitonin levels at birth (first) and at 24h of age (second) were measured for each neonate in both of the study groups. RESULTS: There was no difference between the two groups in terms of gender, birth weight, or gestational age. The mean (min-max) first PCT level was 0.48 (0.07-3.48)ng/ml in the controls and 0.51 (0.09-28.6)ng/ml in patients. The mean (min-max) second PCT level was 1.72 (0.21-18.23)ng/ml in the controls and 16.17 (0.17-100)ng/ml in patients. There was no statistically significant difference in PCT levels between the patient and control groups at birth. However, at 24h of age, PCT levels were significantly higher in the patient group than in the control group (p<0.001). Serum PCT levels in controls at 24h of age were slightly increased compared to levels at birth, but as a normal reaction. PCT thresholds for the diagnosis of sepsis were 0.59 ng/ml at birth (sensitivity 48.7%, specificity 68.6%) and 5.38 ng/ml at 24h of life (sensitivity 83.3%, specificity 88.6%). CONCLUSIONS: In EONS, PCT measurements at birth may initially be normal; a serial PCT measurement at 24h of age may be more helpful for an early diagnosis. During the first 24h of life PCT is a more sensitive marker of infection than C-reactive protein. Further studies are needed to confirm our findings.

Ischaemia-modified albumin levels in newborn jaundice before and after phototherapy.

The aim of our study was to assess the effect of phototherapy (PT) on ischaemia-modified albumin (IMA) and malondialdehyde (MDA) levels in hyperbilirubinemic full-term newborns. The study was performed on 36 full-term infants exposed to PT. The babies were aged 3 to 13 days. IMA and MDA levels of the babies were determined before and after PT, by a colorimetric assay. IMA levels before and after PT were found as 0.424 ± 0.290 and 0.531 ± 0.262 absorbance units, respectively. Although IMA levels after PT were slightly higher, the difference was not statistically significant (p > 0.131). MDA levels before and after PT were found as 8.4 ± 1.8 µmol/l and 9.4 ± 1.5 µmol/l, respectively. Serum MDA concentrations were significantly higher after PT than before PT (p < 0.000). In previous studies, conflicting findings have been reported about the effect of PT on oxidant and antioxidant systems. However, we have found no study investigating IMA levels in hyperbilirubinaemia in newborns before and after PT. Our results shows that PT does not affect IMA levels significantly. IMA increases as a result of oxidative stress. We believe that the lack of significant difference between our IMA levels before and after PT may resulted from hyperbilirubinaemia, which has antioxidant effect.

Freeman-Sheldon (whistling face) syndrome with hyperpyrexia in the newborn: case report.

Freeman Sheldon syndrome (FSS) is a rare, multiple congenital contracture syndrome that is relatively well-known, since affected children have a striking appearance. This entity was historically referred to as the "whistling-face syndrome". Malignant hyperthermia and hyperpyrexia have been documented in FSS after general anesthesia related to the neuropathy. We report a male neonate with FSS and hyperpyrexia without anesthesia. To our knowledge, our patient is the first in the literature with hyperpyrexia in the newborn period without anesthesia.

Testicular changes in newborn rats exposed to phototherapy.

In this study we investigated the long-term effects of 72-h continuous phototherapy on the reproductive system of newborn rats. The animals' weight, fertilization rates, and number of newborn and histopathological changes in the gonads in a normal group not exposed to phototherapy and in the test animals were compared. At the age of 24 weeks there were no significant differences between the two groups, apart from the histology of the testicles of the male rats who were exposed to the phototherapy. The study group showed a significantly reduced diameter of the seminiferous tubules when compared to the controls (P < 0.001). It can be postulated that phototherapy may cause histological degenerative changes in the structure of the rat's testes, even though there were no changes in fertilization rates. Further studies are necessary to reveal the effects of phototherapy on humans and to determine the effects, if any, on fertility.