Mehmet Sah Ipek

Therapeutic management of neonatal chylous ascites: report of a case and review of the literature

Congenital chylous ascites is a rare condition seen in the neonatal period and the data on pathogenesis and treatment modalities are limited. In this article, we report a case of neonate with chylous ascites and review the therapeutic management procedures on chylous ascites in childhood. We present our experience in the diagnosis and treatment of this condition.

NEONATAL PURPURA FULMINANS SECONDARY TO GROUP B STREPTOCOCCAL INFECTION

Purpura fulminans is an acute and frequently fatal disorder characterized by sudden onset of progressive cutaneous hemorrhage and necrosis due to dermal vascular thrombosis and disseminated intravascular coagulation. The authors present a neonate with extensive purpura fulminans due to group B streptoccoccal septicemia and evaluated the attributable clinical mortality and morbidity of this potentially lethal syndrome. Clinicians especially neonatologists should be aware that early-onset sepsis of group B Streptococcus in the newborn infant with purpura fulminans could be a cause of maternal carriage due to colonization of this pathogen microorganism.

Purpura fulminans in a newborn infant with galactosemia

An 11-day-old neonate presented with purpura fulminans and was subsequently diagnosed with galactosemia. Neonatal purpura fulminans occurs predominantly in patients suffering from inherited protein C deficiency or disseminated intravascular coagulation associated with septicemia. Hemostatic changes in patients with liver disease may result in bleeding or, rarely, thrombosis. We suppose that in the present patient, deficiency of protein C, secondary to liver disease, was responsible for the development of purpura fulminans. Treatment consisted of blood and blood products and galactose-free formula. The patient recovered with residual mild psychomotor retardation and the lesions with minimal scarring. In conclusion, galactosemia also should be kept in mind as an uncommon cause of purpura fulminans in newborn infants.

Alendronate for the treatment of hypercalcaemia due to neonatal subcutaneous fat necrosis

Dear Editor, We read with great interest the article by Mitra et al. [3] who described subcutaneous fat necrosis (SCFN) in a preterm infant after severe perinatal hypoxic injury complicated by hypercalcaemia. Mitra et al. [3] state that significant SCFN can develop in both preterm and term infants and preterm infants also develop significant complications including hypercalcaemia. Most patients can be treated successfully by hyperhydration, furosemide, corticosteroids, and also by citrate, calcitonin, and bisphosphonates [4, 5]. Among the bisphosphonates, both etidronate [4] and pamidronate [2] have been used in the treatment of hypercalcaemia due to SCFN of the newborn. However, the use of alendronate for this purpose has not been reported yet, as far as we know. Alendronate as a bisphosphonate is a potent inhibitor of osteoclast-mediated bone resorption. Previously, it has been used safely in patients with hypercalcaemia associated with vitamin D intoxication [1]. Recently, we successfully treated with alendronate sodium a patient with hypercalcaemia associated with SCFN after a severe perinatal asphyxic event. In our patient, the indurated erythematous plaques and nodules appeared on the back, upper arm and gluteal areas by postnatal day 7 (Fig. 1). When the lesions had almost disappeared on postnatal day 30, serum calcium level still reached a peak of 11.5 mg/dL with a urine calcium/urine creatinine ratio of 0.74 mg/mg. Laboratory testing showed serum parathyroid hormone level of 7.5 pg/mL (normal range, 15–65 pg/mL) and serum 25-hydroxyvitamin D level of 38.5 ng/mL (normal range, 10–40 ng/mL). Because of the clear clinical findings, a histopathological examination was not required. Renal ultrasonography showed bilaterally punctiform renal medullary hyperechogenicities. Despite adequate hydration, the patient’s serum calcium level remained high. Because the degree of hypercalcaemia was mild and there was nephrocalcinosis, furosemide treatment was not chosen in our patient. Moreover, corticosteroids were not considered because of their potential side effects. Hence, the bisphosphonate treatment was intended and then, because availability of oral preparation, the patient was given oral alendronate at a dosage of 5 mg/day. Oral alendronate treatment was discontinued on postnatal day 50 when the serum calcium level and urinary calcium/ creatinine ratio had returned to normal. During the alendronate therapy, no side effects were observed. In conclusion, patients with SCFN should be closely monitored for developing metabolic problems like hypercalcaemia. Moreover, alendronate can be used safely and effectively in the treatment of hypercalcaemia associated with SCFN. N. Hakan :M. Aydin (*) :A. Zenciroglu :N. Demirel : N. Okumus :M. S. Ipek Department of Neonatology, Dr. Sami Ulus Maternity and Children’s Hospital, Babur Street, no: 44 (06080) Altindag, Ankara, Turkey e-mail: dr1mustafa@hotmail.com

Incontinentia Pigmenti With Encephalocele in a Neonate: A Rare Association

Incontinentia pigmenti is a rare, X-linked dominant multisystem genodermatosis affecting ectodermal and mesodermal tissues. After the skin, the central nervous system is the second-most affected system. We report a neonate with incontinentia pigmenti and encephalocele, as a feature of the central nervous system involvement, to stress this uncommon association.

The role of antenatal factors on tibial speed of sound values in newborn infants

Objective: To evaluate the role of some antenatal factors on bone status of newborn infants. Methods: Tibial bone speed of sound (SOS) value was measured by quantitative ultrasound within 5 days after birth in 205 neonates whose gestational ages ranged between 31 and 40 weeks. The cohort was divided into two groups according to intrauterine growth curves, as small- (SGA) and appropriate-for-gestational age (AGA). All cases were also been evaluated by antenatal ultrasonography for oligohydroamniosis. Results: The mean SOS value was found significantly higher in SGA (n = 43) than AGA infants (n = 162) (p < 0.001). The mean tibial SOS value of infants with a history of oligohydroamniosis (n = 28) was also higher than those without oligohydroamniosis (n = 177) (p < 0.001). But, in SGA infants with a history of oligohydroamniosis (n = 17), the mean tibial SOS value was similar to those SGA infants without oligohydroamniosis (n = 26) (p > 0.05). Infants whose mothers had smoked during pregnancy (n = 18) had significantly higher tibial SOS values compared to those of whose mothers had not (n = 187) (p = 0.006). In addition, mean tibial SOS values were determined higher in male infants (n = 116) compared to female infants (n = 89) (p = 0.036). There was a significant correlation between tibial SOS values and gestational age (r = 0.178, p = 0.011). Conclusions: While creating reference curves of SOS values at birth, smallness for gestational age, maternal smoking and gender as well as gestational age should be taken into account.

Carmi syndrome with congenital heart defects

Carmi Syndrome With Congenital Heart Defects Mustafa Aydin,* Aysegul Zenciroglu, Ayhan Yaman, Utku Arman Orun, Nilufer Arda, Asuman Gurkan Colak, Nurullah Okumus, Mehmet Sah Ipek, and Serdar Ceylaner Department of Neonatology, Dr. Sami Ulus Maternity and Children’s Hospital, Ankara, Turkey Department of Pediatric Cardiology, Dr. Sami Ulus Maternity and Children’s Hospital, Ankara, Turkey Department of Pathology, Dr. Sami Ulus Maternity and Children’s Hospital, Ankara, Turkey Department of Dermatology, Dr. Sami Ulus Maternity and Children’s Hospital, Ankara, Turkey Intergen Genetic Disease Diagnostic Center, Ankara, Turkey

Changing pattern of Candida species in a neonatal intensive care unit

S ir : We were interested in an article which was posted 22 July 2010 ahead of print on the early on-line service of Medical Mycology [1]. While Yapar and colleagues noted in their report that Candida albicans was the most commonly recovered Candida species in 83 blood cultures, nonC. albicans Candida species, e.g., Candida tropicalis , Candida parapsilosis , Candida glabrata , were isolated in increasing frequency from adult patients. Collectively, the nonC. albicans Candida species were found to be associated with 43% of the cases of candidaemia in their study. The predictors of candidaemia included the presence of a urethral catheter, prior use of antibiotics, red blood cell transfusions and parenteral nutrition. Total parenteral nutrition was an independent risk factor for candidemia caused by C. albicans and nonC. albicans Candida species . We performed a retrospective, single centre study in the neonatal intensive care unit (NICU) of Ankara Sami Ulus Maternity, Childrens ’ Education and Research Hospital between January 2005 and December 2009 to assess the rate of invasive candidiasis among low birth weight premature newborns. During this period, 24 neonates with a mean gestational age of 30 weeks (range 24 – 36 weeks) and a mean birth weight of 1,455 g (range 780 – 2,470g) developed invasive candidiaemia, i.e., an overall incidence of 1.4% but with an increasing trend over the study period. All infections were acquired nosocomially and developed within a mean of 17.5 (range 6 – 53) days after birth. The four most frequent causative Candida species were C. parapsilosis (32%), C. pellucilosa (32%), C. albicans (22%) and C. glabrata (8%), that is nonC. albicans Candida species were the etiologic agents of 72% of the candidaemia cases in our series. C. albicans was the predominant species recovered between 2005 and 2008, whereas, nonC. albicans Candida species, particularly C. parapsilosis , were the most frequent species during the period 2008 – 2009. Risk factors for candidemia were total parenteral nutrition (87.5%), mechanical ventilation (62.5%), antimicrobial therapy (37.5%), central venous catheter insertion (29.2) and necrotizing enterocolitis (29.2%). The overall mortality due to candidemia in our patients was 4.2% (1 of 24 cases, Table 1).