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An unusual cause of acute renal failure in a newborn: hydrometrocolpos

Here we report on an unusual cause of acute renal failure in a newborn: hydrometrocolpos due to imperforate hymen. Hymenotomy resolved all of the laboratory and clinical abnormalities of the patient and the baby was sent home healthy.

Proboscis lateralis: radiological evaluation.

Abstract. Proboscis lateralis is a rare craniofacial anomaly. It consists of a soft, trunk-like process that originates from the medial portion of the orbital roof and it may be accompanied by a number of facial and cerebral anomalies. The characteristics and relationship of the lesion to adjacent structures should be known, and associated anomalies should be detected before surgical correction. The patient should undergo neuroimaging studies besides clinical evaluation to obtain this information in detail. The neuroimaging findings of only one previous case are available in the literature. We report two cases of proboscis lateralis evaluated by CT and MRI before corrective surgery.

Is Vitamin D Deficiency a Risk Factor for Respiratory Distress Syndrome

BACKGROUND Previous studies have shown the relationship between in utero lung development and vitamin D [25(OH)D], but there have been no studies to investigate whether vitamin D deficiency is a risk factor for respiratory distress syndrome (RDS) in preterm babies. OBJECTIVES In this study, we investigated if 25(OH)D deficiency is a risk factor for RDS. METHODS One hundred fifty-two preterm newborns, born at 29 - 35 weeks gestational age, were included in the study following informed consent from the parents. Peripheral blood samples were collected within the first 24 hours of life and 25(OH)D levels were measured by liquid chromatography-tandem mass spectrometry. Demographic characteristics of the babies and the diagnosis of RDS were recorded. RESULTS In 64 % of preterm infants, 25(OH)D levels were compatible with severe deficiency (≤ 10 ng/mL), 33 % with moderate deficiency (10 - 20 ng/mL), and 3 % with mild deficiency (20 - 30 ng/mL). In none of the babies was a normal 25(OH)D level observed. Serum 25(OH)D levels were not correlated with gestational age. Respiratory distress syndrome was more common in preterm babies with severe (28 %) compared to mild-moderate 25(OH)D deficiency (14 %) (p < 0.05). CONCLUSIONS None of the preterm infants in this study had normal vitamin D level, which underlined the burden of vitamin D deficiency in pregnant women and their offspring. RDS was more common in severely vitamin D-deficient preterms. Determination of vitamin D status of the mothers and appropriate supplementation might be a valuable strategy to reduce RDS, in addition to antenatal steroids. Besides, since vitamin D is a regulatory factor in many organs during fetal development, long-term effects of in utero vitamin D deficiency warrant further studies.

Simplified gyral pattern with cerebellar hypoplasia in Sedaghatian type spondylometaphyseal dysplasia: A clinical report and review of the literature†

We report on a patient with Sedaghatian type spondylometaphyseal dysplasia (SSMD) who presented with metaphyseal dysplasia, congenital atrioventricular block, simplified gyral pattern, hypogenesis of corpus callosum, and severe cerebellar hypoplasia. We want to emphasize that in this rare congenital lethal skeletal dysplasia with unknown etiology, central nervous system malformations might be a major component of the disorder and should be evaluated in detail to possibly uncover the underlying pathophysiology.

Ciprofloxacin treatment in newborns with multi-drug-resistant nosocomial Pseudomonas infections

Objectives: To evaluate the efficacy and acute side effects of ciprofloxacin treatment in newborns who developed nosocomial Pseudomonas aeruginosa infection. Methods: Intravenous ciprofloxacin treatment was given to 30 newborns who developed nosocomial P. aeruginosa infection as proven by culture antibiogram results. Initial doses of 10 mg/kg/day were given and increased up to 40 mg/kg/day according to clinical response, laboratory and culture results. During therapy, complete white blood cell counts, urinalysis, liver and renal function tests were performed weekly. All patients were examined daily during treatment for possible symptoms of joint toxicity such as erythema and swelling. The patients were evaluated by general physical examination, with special attention to joints, 1 week after discharge. Results: Two of the patients (6.6%) died due to pseudomonas infection, but the bacteria were successfully eradicated in 28 patients (93.4%). Four patients died from other causes. No laboratory abnormality related to ciprofloxacin was observed during treatment. Swelling and hyperemia of the joints were not encountered during treatment and the 1-week period after discharge. Ciprofloxacin-resistant P. aeruginosa isolates were not grown during the study. Conclusion: Ciprofloxacin treatment is effective in life-threatening multi-drug-resistant P. aeruginosa infections.

Temporary Peritoneal Dialysis in Newborns and Children: A Single-Center Experience over Five Years

Aim: To evaluate the indications, complications, and outcomes of temporary peritoneal dialysis (TPD) in children with acute renal failure (ARF). Patients and methods: All patients undergoing TPD between February 2006 and January 2011 in a children’s hospital were included in the study. Patient characteristics, indications, complications, and duration of TPD (DPD), requirement of re-operation, length of stay, presence of sepsis, and outcome were recorded. Results: There were 21 newborns (14 prematures), 9 infants, and 9 children. The main nephrotoxic agents were gentamicin (n = 7), netilmisin (n = 5), vancomycin (n = 3), and ibuprophen (n = 3). Patients with multiorgan failure (n = 9) had significantly higher blood urea nitrogen (BUN) and creatinine levels than those without multiorgan failure (n = 30) [BUN: 94 ± 27.3 vs. 34.3 ± 4.9) and creatinine: 4.1 ± 0.8 vs. 1.9 ± 0.2)]. The mean DPD was longer in mature patients than in prematures (newborn: 3.7; children: 7.1). Nine complications were observed (23%) (leakage in three and poor drainage in six patients). Twenty-five patients (64.1%) responded to TPD treatment and were discharged, and 14 patients (10 newborns and 7 of them were premature) died (35.9%). Mortality rate was higher in prematures (n = 7) and patients with a history of nephrotoxic agent (n = 10). Conclusion: TPD is effective especially in neonates with ARF and it is a reliable alternative to the hemodialysis or other continuous renal replacement therapies but it is not free of complications. It has limited effects, particularly in patients with multiorgan failure.

Treacher Collins Syndrome associated with foot deformity and genital anomalies Ayak deformitesi ve genital anomali ile birliktelik gösteren Treacher Collins Sendromu

Treacher Collins sendromu, mandibulofasiyal disostozis olarak bilinen nadir bir sendromdur. Sendrom baslica yuz morfolojisini etkiler. Treacher Collins ile ilgili yayinlarin cogu sendromun fasiyal ozelliklerine odaklanmistir. Bu yazida mikropenis, kriptoorsidizm ve metatarsus adduktus deformitesine sahip Treacher Collins sendromlu bir yenidogan sunulmustur.

Joubert Sendromu; Olgu Sunumu

Joubert Sendromu; genellikle yasamin ilk ayinda ortaya cikan hipotoni, takipne-apne epi-zodlari ile karakterize anormal solunum paterni, anormal goz ve dil hareketleri, ataksi, mental-motor retardasyon ve bazi hastlarda bunlara ekstraserebral bulgularin eslik ettigi nadir gorulen bir hastaliktir. Anormal solunum paternine sahip hastalarda ayirici tanida dusunulmesi gerekir. Solunum sikintisi sikayeti ile getirilen ve Joubert Sendromu tanisi alan 40 gunluk bir erkek hasta literatur bilgileri isiginda tartisilmistir. Joubert syndrome is a rarely seen disease which genarlly presents in the first month of the life; characterized by hypotonia , tachypnea and apnea episodes. Furthermore; abnormal respiration pattern, abnormal eye and tongue movements, ataxia, mental - motor retardation can be found. Should be kept in mind that some extracerebral disorders may accompany in some cases It has to be considered in the differential diagnosis of patients with abnormal respiration pattern. A forty days old male who had tachypnea and had been diagnosed Joubert syndrome was discussed in the light of the literature.

Prune-Belly Sendromu: Olgu Sunumu

Prune belly sendromu abdominal kaslarin yoklugu, kriptorsidizm ve obstruktif uropati tri-adindan olusur. Bu yazida nadir gorulmesi nedeniyle prune belly sendromlu bir hasta sunulmus, literatur bilgileri gozden gecirilmistir. The Prune-Belly Syndrome: A Case Report The Prune belly syndrome consists of the triad absence of abdominal muscles, cryptorchidism and obstructive uropathy. A rare case of Prune belly syndrome was presented and the literature was reviewed.

Netilmisin Verilen Hiperbilirübinemili Yenidoğanlarda Retinol Bağlayıcı Protein Ve Beta -2-Mikroglobulin Düzeylerindeki Değişiklikler

Changes in Retinol Binding Protein and Beta-2-Microglobulin Levels in the Netilmicin Administered Newborns with Hyperbilirubinemia It is considered that hyperbilirubinemia affects renal functions in newborns. Increased levels of retinol binding protein (RBP) and J32 microglobulin in urine are used as indicators of proximal tubular dysfunction. The purpose of this study is to determine the changes in tubular functions in newborns with hyperbilirubinemia who were administered netilmicin prophlaxis. Sixty jaundiced newborns with gestational ages ranging from 38 to 42 weeks and with similar postnatal ages (in the first 10 days of life) were included in the study. Fifteen healthy newborns were selected as a control group. In newborns with indirect hyperbilirubinemia and in the control group, measuring blood urea nitrogen (BUN), serum creatinin, creatinin clearance (Ccr) , fractional excretion of sodium (FENa) serum and urine osmolarity, RBP and J32 microglobulin concentrations in urine, renal functions were examined. In this study, the results showed that the effect of indirect hyperbilirubinemia over RBP and J32 microglobulin concentrations in urine did not increase with prophlactic administration of netilmicin in newborn period. In addition, no difference was observed in the levels of RBP and j32 microglobulin in urine between nephrotoxic drug administered (group IA: Ampicillin 100 mg/kg/day I.M in four Yenidoganlarda, hiperbilirubineminin bobrek fonksiyonlari uzerine etkili oldugu dusunulmektedir. Idrarda, retinol baglayici protein (RBP) ve J32 mikroglobulin konsantrasyo-nundaki artis proksimal tubuler disfonksiyonun gostergesi olarak kullanilmaktadir. Indirekt hiperbilirubineminin, tubuler fonksiyona etkisinin nefrotoksik bir ilac olan netilmisin kullanimi ile siddetlenip siddetlenmiyecegini arastirmak amaciyla calismaya, gestasyonel yaslan 38-42 hafta arasinda degisen, indirekt hiperbilirubinemisi olan postnatal yaslan benzer (ilk on gun icinde) 60 yenidogan alindi. On bes saglikli bebek kontrol grubu secildi. Indirekt hiperbilirubenimili ve kontrol grubundaki yenidoganlarda kan ure azotu (BUN), serum kreatinini, kreatinin klirensi (Cer), fraksiyonel sodyum ekskresyonu (FENa), serum ve idrar osmolaritesi, idrarda RBP ve 32 mikroglobulin konsantrasyonu olculerek bobrek fonksiyonlari incelendi. Bu calismada; indirekt hiperbilirubineminin RBP ve J32 mikroglobulin uzerine etkisi, yenidogan doneminde profilaktik amacli verilen netilmisin ile artmadigi goruldu. Ayrica nefrotoksik ilac alan grupta (Grup IA:Ampisilin lOOmg/kg/gun I.M. dort doz + Netilmisin 5mg/kg/gun I.M. iki doz, Grup IB:Ampisilin lOOmg/kg/gun I.M. dort doz + Netilmisin 5mg/kg/gun I.M. tek doz), nefrotoksik ilac almayan gruba gore (Grup Ic:Ampisilin lOOmg/gun I.M. dort doz) idrarda RBP ve J32 mikroglobulin duzeyleri acisindan bir fark gorulemedi.