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Akyuz-Unsal AI, Key O, Guler D, Bekmez S, Sagus M, Akcan AB, Kurt-Omurlu I, Anik A, Oruc-Dundar S, Turkmen M. Retinopathy of prematurity risk factors: Does human milk prevent retinopathy of prematurity? Turk J Pediatr 2019; 61: 13-19. The aim of this study was to investigate the risk factors for Retinopathy of Prematurity (ROP) development and the potential effect of human breast milk among these factors. For this purpose, infants admitted to a tertiary referral clinic for ROP screening and treatment between April 2013 and May 2015, were included in this retrospective study. The demographic data, accompanying diseases, previous surgery, type of feeding and duration of human breast milk intake were recorded. According to the ROP screening examination results, infants were divided into two groups as those with ROP (infants at any stage of ROP) and those without ROP. Relationship between the risk factors and ROP were evaluated. The comparison of 221 infants without ROP and 99 infants with ROP; revealed that gestational age at birth, birth weight, mechanical ventilation support, bronchopulmonary and cardiac diseases, hydrocephaly, any previous surgery, infections, steroid treatment percentages were high and human breast milk intake percentage was low among infants with ROP. Mean breast feeding time for infants with ROP (3.81 ± 2.33 month) was shorter than the infants without ROP (5.51 ± 1.43 month) (p < 0.001). In logistic regression analysis, the duration of breast feeding was inversely related with ROP (OR 0.744; 95% CI 0.621-0.891; p < 0,001). These results suggest that gestational age at birth and accompanying diseases are the main risk factors for the development of ROP. As the duration of the breast feeding of the infants without ROP was longer than the infants with ROP; breast feeding may have a preventive effect on ROP development.

BACKGROUND Twin-to-twin transfusion syndrome (TTTS) is a significant cause of perinatal morbidity and mortality. Fetoscopic laser photocoagulation (FLP) is the optimal treatment option for twin-to-twin transfusion syndrome; but can cause central nervous system, extremity and intestinal system injury. CASE We report the case report of ileal atresia and severe cerebral infarction co-occurrence after fetoscopic laser photocoagulation treatment. It is uncertain as to whether ileal atresia occurred due to ischemia associated with TTTS, the treatment with FLP, or a combination of both. CONCLUSIONS Cases with prenatal ultrasonographic abnormalities after FLP should have a close assessment to detect bowel complications. Despite many developments in its management, TTTS remains an important risk factor for cerebral injury.

The crucial role of platelets in the permanent closure of the ductus arteriosus has recently been elucidated in an animal model; however, clinical studies investigating the impact of platelet count on ductal patency have conflicting results. Our aim is to compare platelet count, indices and serum platelet-derived growth factor levels in preterms with and without ductal patency. Preterms with a gestational age of 27-30 weeks followed up during a twelve-month period in the neonatal intensive care unit of Adnan Menderes University Hospital were enrolled. Infants underwent echocardiographic evaluation starting on the second day and subsequently assessed every other day until ductal closure was achieved, or upon clinical signs of reopening. Platelet-derived growth factor was measured on the second and fifth days of life. Eleven very low birth weight infants who subsequently required medical treatment for patent ductus arteriosus were compared with twenty-three infants with closed ductus. Although platelet count and indices were similar, median serum plateletderived growth factor levels on day 5 were significantly lower among babies who subsequently required medical treatment for ductal patency (874.6 vs 1099.6 pg/ml). The current study points out a possible association between serum platelet-derived growth factor levels and ductal closure. Our results suggest that platelet-derived growth factor may play a role in ductal closure independent from platelet count and might be used as an adjunct surrogate for prediction of future need for treatment for hemodynamically significant patent ductus arteriosus in preterm infants.

Pertussis or whooping cough is a vaccine-preventable disease that still remains a serious infection in neonates and young infants. The disease is particularly severe in infants less than three months old, who are often infected by their parents. Congenital nephrotic syndrome is a rare entity presenting within the first three months. It encompasses a heterogeneous group of entities with genetic, infectious and idiopathic etiologies. In this report we describe a newborn infant who presented with congenital nephrotic syndrome secondary to Bordetella pertussis infection.

Tubulointerstitial nephritis (TIN) refers to a group of heterogeneous diseases affecting the interstitial compartment of the kidney. It might be primary or can develop secondary to many urinary systemic diseases. Primary TIN develops mainly following drug usage, exposure to toxins, and also infections and humoral and cell-mediated immune reactions. In some patients, signs of systemic inflammatory reactions can be the first presenting symptoms. Histopathological evaluation reveals mononuclear cells and lymphocytes in the interstitium and tubuli. Acute and chronic TIN can resolve after elimination of the culprit destructive factors, as drugs, toxins and immune reaction. Combination of tubulointerstitial inflammation and uveitis is termed as tubulointerstitial nephritis-uveitis (TINU) syndrome. Uveitis might occur before, after, and also concomitantly with TIN. Herein, two adolescent cases of TIN and TINU, seemingly developed secondary to paracetamol and codeine phosphate use, are presented.

Chronic kidney disease (CKD) is a life-long condition associated with substantial morbidity and premature death due to complications from a progressive decrease in kidney function. Especially in children, early diagnosis and detection of the etiologic factors are important to improve their health outcomes. Familial juvenile hyperuricemic nephropathy (FJHN) is a rare autosomal-dominant disorder characterized by hyperuricemia with renal uric acid under-excretion and CKD. Genetic studies have revealed mutations in the uromodulin (UMOD) gene. Highlighting the importance of CKD in children, a 14-year-old girl with the rare diagnosis of FJHN is reported herein.

In this study, we aimed to investigate the relationship between renal P-glycoprotein (rP-gp) expression and response to corticosteroid therapy in childhood nephrotic syndrome (NS). Expression of rP-gp was evaluated prior to non-steroid immunosuppressive therapy in children with NS (Group 1), prior to any treatment in children with immunoglobulin (Ig)A nephropathy (Group 2), and during renal donation in healthy adults (Group 3); mesangial proliferation was evaluated in Groups 1 and 2. Total dosage of steroid was calculated in Group 1. The ratio of rP-gp (+) glomeruli was higher in Group 1 than Group 3 (33.8 ± 27.1% vs 4.7 ± 4.6%, p=0.000). There were no rP-gp (+) glomeruli in Group 2. The rate of mesangial proliferation was similar in Groups 1 and 2. There was no statistically significant correlation between rP-gp expression and total steroid dosage (r=0.455, p=0.160). Our findings showed that rP-gp expression is increased in patients receiving steroid therapy for NS regardless of the cumulative steroid dosage and mesangial cell proliferation.

Acute focal bacterial nephritis (AFBN) is a rare cause of interstitial bacterial nephritis. Ultrasound identifies AFBN as a hypoechogenic and hypoperfused parenchymal lesion, which requires its differentiation from renal abscess and tumor. Hematogenous spread or ascending infection arising from the lower urinary tract is thought to be involved in the pathogenesis of AFBN. Herein, a six-year-old healthy male patient, diagnosed using ultrasound and computerized tomography (CT) and treated with intravenous antibiotics, is presented. As a result, AFBN can be seen in healthy children without any history of reflux or urinary tract infection, and differentiation from renal abscess is important.

A preterm infant with isolated fetal ascites was admitted to the neonatal intensive care unit due to the appearance of respiratory distress at birth. An umbilical venous catheter (UVC) was inserted. Abdominal ultrasonography (US) showed localization of the catheter tip in the portal vein. It was removed and replaced with a newer one. UVC tip location was confirmed with X-ray. His condition had been improving until he worsened suddenly on the sixth day of life. US showed hepatic abscess and intraabdominal hemorrhage derived from the malpositioned UVC. A drainage catheter was inserted to the abscess and paracentesis was applied. Practitioners should be cautious about any signs of UVC complications, even if true localization of the catheter tip is proven at the first application. Furthermore, if it is difficult to decide whether the catheter tip is in the right location, confirmation with US can be considered.

Munchausen syndrome by proxy is a kind of child abuse in which affected children are often hospitalized for long periods and endure repetitive, painful and expensive diagnostic attempts. We present herein two toxicologically confirmed cases of Munchausen syndrome by proxy. Case 1 is a 16-month-old male who had fever, peripheral cyanosis, tremor, and reported cardiac arrest. Symptoms recurred in the hospital when the mother administered fluids. Toxicology detected 3.5 ng/ml mercury (Hg) in the fluid and 9.4 microg Hg/g creatinine in the urine. Case 2 is a 14-year-old female who had irregular blood findings and multiple hospitalizations. Serum analysis detected warfarin. Both mothers were transferred to psychiatric care. Munchausen syndrome by proxy should be suspected when clinical/laboratory findings are negative, illness descriptions are inconsistent, and frequent hospitalization yields no diagnosis. Psychiatric evaluation and toxicological analysis are recommended.

Secondary pseudohypoaldosteronism type 1 develops due to transient aldosterone resistance in renal tubules and is characterized by renal sodium loss, hyponatremia, hyperkalemia and high plasma aldosterone levels. Although many reasons are described, urinary tract infections and/or urinary tract anomalies are the most common causes. Although the cause of the tubular resistance is not known exactly, renal scar development due to obstruction and reduced sensitivity of mineralocorticoid receptors due to cytokines such as transforming growth factor (TGF)-beta are the possible mechanisms. It is seen especially within the first three months of life and the frequency decreases with age. The treatment is usually elimination of the underlying cause. In this article, we present four patients with several urinary tract anomalies and concomitant urinary tract infection who developed transient secondary pseudohypoaldosteronism.

Routine use of transcutaneous bilirubin (TcB) measurement in the newborn nursery could reduce costs, readmission rates for hyperbilirubinemia and the need for total serum bilirubin (TSB) measurements. The aim of this study was to examine the correlation between TcB measurement, as performed using BiliCheck, and TSB, measured with high-pressure liquid chromatography (HPLC) and with standard laboratory methods, and to determine the TcB cutoff points with desirable sensitivity and specificity values for various clinically relevant TSB levels by HPLC. Fifty-four infants ofor = 30 weeks of gestational age were enrolled in the study. Near simultaneous blood collection for TSB analysis by three methods--bedside bilirubinometer, diazo method and HPLC--and TcB measurement were performed. There was good correlation between TcB and HPLC-bilirubin (B) (r = 0.85), TSB by bilirubinometer and HPLC-B (r = 0.91) and TSB by diazo method and HPLC-B (r = 0.91). The cut-off limits providing a sensitivity of 100% for TcB measurements were TcBor = 9 mg/dl for HPLC-B17 mg/dl and TcBor = 8 mg/dl for HPLC-B15 mg/dl and HPLC-B13 mg/dl. Despite having good correlation with HPLC, BiliCheck showed worse performance than bilirubinometer and diazo method at various clinically relevant cut-off values. Since BiliCheck required relatively lower thresholds with false-positive results for having a sensitivity of 100%, it cannot be recommended as a complete substitute for serum bilirubin measurements.

Pediatric trauma is the leading cause of mortality and morbidity in children. A questionnaire was applied to evaluate physicians' attitudes and perception of pediatric trauma cost. Physicians working in the field of pediatric trauma (namely those who work in emergency services, pediatrics and pediatric surgery departments; who are consulted regarding pediatric traumas; and those for whom pediatric trauma patients constitute the majority of their patient spectrum) were enrolled in the survey. A questionnaire was administered to elicit the demographic data, features of pediatric trauma in their practice, self-perception of pediatric trauma knowledge, estimation of trauma severity, parameters used for pediatric trauma diagnosis, and perception of pediatric trauma score (PTS) and trauma cost. A total of 103 physicians responded to the questionnaire (median age: 30.8 +/- 5.6 years; range: 24-56). Of the 103 respondents, 49 were males (47.6%) and 54 were females (52.4%). Physicians responding to the questionnaire were specialists in Pediatrics (32.9%), Pediatric Surgeons (5.9%), Emergency Medicine Specialists (2.9%), and residents (47.6%) in those three disciplines in University Hospitals, Public Hospitals and Research Hospitals. Physicians reported falls (58.1%) as the most common cause of trauma, and noted head injuries with an incidence of 49.9% in their trauma practice. Physicians' self-perception of their pediatric trauma knowledge was questioned in three categories as: overall, diagnosis and treatment of trauma. They reported that their knowledge of pediatric trauma overall and regarding diagnosis and treatment was "efficient" at rates of 87.4%, 83.6% and 74.8%, respectively. However, while 76.7% of physicians perform radiological evaluations in all trauma patients, only 56.3% of them use laboratory tests routinely in diagnosis. Participants reported that cost of trauma was mostly affected by severity of trauma (49.5%) and least affected by the patient's sex (64.1%). They also believed that radiologic evaluations (66%) accounted for the largest portion of trauma cost and the cost of consultations (44.7%) for the smallest portion. In conclusion, we suggest that although most physicians were aware of cost factor in trauma, they did not consider trauma costs in diagnosis and management.

Traumatic injuries are the leading cause of mortality and morbidity during childhood. A retrospective study was performed to evaluate the impact of Pediatric Trauma Score (PTS) on burden of trauma in emergency care. Children admitted to the emergency room were retrospectively evaluated for age, sex, mechanism of injury, physical examination findings, and PTS. The cost of trauma was obtained by medical records. A total of 146 patients (male/female: 93/53) were enrolled. The median age was 6 (interquartile range: 3-9.25). Mechanism of injury was falls (74%), motor vehicle crashes (9.6%), non-vehicular accidents (7.5%), struck by/against (6.2%), and cuts and gunshots (2.1%). The median PTS was 10. In the evaluation of trauma burden, radiologic investigations accounted for 41%, consultations for 23.5%, laboratory investigations for 15.6%, emergency surgical interventions for 12.1%, and medical interventions for 6.8% of total trauma cost in emergency care. PTS showed no impact on burden of trauma in emergency care (p0.05). Total trauma cost was increased 2.1-fold in male patients, 2.6-fold in head injuries and 4.4-fold in abdominal injuries (p0.05). Pediatric Trauma Score had no effect on the burden of pediatric trauma in emergency care. The total cost of trauma was primarily affected by head injury and abdominal trauma. Higher costs may be related with routine radiological investigations in head and abdominal injuries.

We hypothesized that young children uncommonly acquire significant injuries due to household falls that necessitate medical attention. Data on recalled fall experiences were collected from parents of children younger than two years visiting the University Pediatrics Clinic. Statistical analysis was performed using Student's t test and chi square test. Of 573 children, 36% experienced no falls, while 64% experienced 782 falls. Eleven percent of falls occurred from a distance of three to five feet (approximately 90-150 cm). In 6% of all falls, medical care was sought. In 40% of this subcategory, examination was normal; in 33%, medical work-up revealed normal results; in 23%, injuries necessitated subsequent intervention. No fall led to neurological findings. This study supports that it is uncommon for household falls to cause injuries significant enough to require professional medical care. Even falls from a distance of greater than three feet result in only mild injury with no neurological findings.

Incontinentia pigmenti (IP) is a rare genetic multisystem disorder that may affect many organs including the skin, bone, eyes and the central nervous system. Central nervous system manifestations are seen in 30% of cases with seizures and mental retardation. Seizures occurring as the presenting sign of IP are rarely reported. We report a case of a female newborn with IP who had seizures on day 4 of life, which were followed in her second month by the development of the characteristic cutaneous changes for IP. With this case report, we would like to emphasize the need for inclusion of IP in the differential diagnosis of neonatal seizures.

A 17-year-old male presenting with chronic renal failure whose supporting clinical manifestations of the disease had appeared independently over a four-year period is reported. The renal biopsy specimen of the patient revealed tubulointerstitial nephritis and membranous glomerulonephritis. He never had hilar adenopathy, but maculopapular rashes, erythema nodosum, arthritis, chronic lymphocytopenia, hepatomegaly, splenomegaly, and lymphadenomegaly had been observed at different periods over four years. The presence of non-caseating granulomatous lesions in the liver biopsy accompanying uveitis verified the diagnosis of sarcoidosis. Low dose steroid was applied to this hepatitis-C carrier, and uveitis was suppressed. No recurrence has been observed in two-year follow-up.

Short rib-polydactyly syndrome (SRPS) is a group of rare, lethal skeletal dysplasias characterized by short ribs and limbs, polydactyly, hypoplastic thorax and visceral anomalies. Our case had coarsening of facial features, low-set ears, lobulated tongue, cleft palate, and hypoplastic epiglottis. Short proximal parts of upper limbs, bilateral postaxial polydactyly of hands, and bifid big toe with zygodactyly were additional findings. Chest was narrow. Ambiguous genitalia was noted but testicles were in scrotum. Choroid plexus cyst and coarctation of aorta were found in autopsy. Radiographies of the skull revealed occipital horn accompanied by prominent external occipital protuberance. The thoracic cage was narrow and elongated with short and iliac wings, pubic and ischial rami were were hypoplastic, and both acetabula were shallow and trident shaped. All tubular bones had wide and rounded metaphyses. Because clinical and radiological features of the four established subtypes are very similar, there are difficulties in the classification. We report an infant whose radiological, clinical and postmortem features were consistent with type IV SRPS (Beemer-Langer).

Horseshoe kidney is a relatively common renal anomaly with which many structural and developmental anomalies have been shown to be associated. However, there are only a few case reports regarding the association of membranous glomerulonephritis and focal sclerosing glomerulonephritis in patients with horseshoe kidneys. We report a girl who was evaluated for hematuria and proteinuria, and found to have horseshoe kidney. Renal biopsy demonstrated mesangioproliferative glomerulonephritis. She also had simple diffuse goiter.

Childhood tuberculosis might have unusual clinical presentation. A seven-year-old female patient was admitted with fever and pleural effusion. Her pneumonia resolved following 21-day treatment period. An atelectatic appearance remained on the right middle zone in her chest X-ray. Tuberculin skin test showed 13 mm induration. Triple drug antituberculosis treatment was started. Since atelectasis persisted on her follow-up radiograph one month later, bronchoscopy was performed which revealed a hemorrhagic polypoid mass occluding the right upper lobe anterior segment orifice. Surgical removal was performed by right upper lobectomy. The pathological diagnosis was necrotizing granulomatous infection suggesting tuberculosis. The patient has been well on follow-up after completing a nine-month course of antituberculous treatment.

We report a nine-year-old girl who presented with the clinical and laboratory findings of rapidly progressive glomerulonephritis. She was found to be positive for both pANCA and cANCA. However, renal histopathology revealed immune complex type of crescentic glomerulonephritis. Thus, although testing for ANCA is an important tool in the prediction of the subtype of crescentic glomerulonephritis, a renal biopsy is still required to establish the diagnosis.

Familial association of congenital camptodactyly and arthropathy without evidence of concurrent inflammation has an autosomal recessive pattern of inheritance. We describe four children born to consanguineous parents in two families with congenital camptodactyly and polyarthropathy which were misdiagnosed and treated as juvenile rheumatoid arthritis (JRA) for some time. The siblings in the second family also had fibrosing pleuritis. Histopathological examination of the synovial tissues of the children in the first family revealed synovial hypertrophy and presence of multinucleated giant cells with minimal inflammation and vasculitis. On the other hand, prominent fibrosis with no inflammation was present in the synovial tissue of the elder boy in the second family. Thus, while the children in the first family had the phenotypic characteristics of congenital familial hypertrophic synovitis, the latter siblings probably represent a form of the familial fibrosing serositis.

Dermatomyositis is the connective tissue disease with the least renal involvement. Although some renal findings like proteinuria, hematuria, pyuria, progressive renal insufficiency, and glomerular and tubular calcium deposits with arteriolar fibrosis have been described, glomerulonephritides have rarely been associated with dermatomyositis, especially in childhood cases. We describe a 10-year old boy with the clinical picture of dermatomyositis who underwent renal biopsy due to microscopic hematuria demonstrating membranous glomerulonephritis with Clq deposition. Children with "full-house" membranous glomerulonephritis with deposition of Clq and the other immunoglobulins have been reported to present in the future with the clinical findings of systemic lupus erythematosus. However, laboratory evaluation of our patient for systemic lupus erythematosus was negative at the present time. Thus, we think this case should be followed up closely with special attention to the possible clinical and laboratory findings of systemic lupus erythematosus.

Respiratory functions are affected during hemodialysis. Ultrafiltration rate, acid-base balance and the strength of respiratory muscles have been suggested as important factors in adults undergoing chronic hemodialysis. L-carnitine is crucial for energy producing utilization fatty acid and, possible amino acids. Carnitine treatment has been associated with hypertrophy of type I muscle fibers. Carnitine supplementation in nondialysis patients increases exercise tolerance. Eventually, administration of L-carnitine to adult hemodialysis patients improves exercise capacity, energy metabolism and muscle mass. The study was performed to investigate the chronic effects of L-carnitine treatment on respiratory functions in children receiving chronic hemodialysis therapy. Predialytic and postdialytic respiratory function tests were performed in ten children with end-stage renal disease before and after a three-month L-carnitine treatment period. The mean age was 12 +/- 4 years. L-carnitine was administered at a dose of 20 mg/kg intravenously at the end of each hemodialysis session. Mean predialytic serum carnitine value before and after the carnitine treatment period were 21.8 +/- 3 and 132.0 +/- 48.5 mmol/L, respectively, and the increase was significant (p0.05). Respiratory function tests performed just before the carnitine treatment period implied bronchospasm that was clinically vague and could only be detected by a significant decrease in FEV1/FVC, PEF and FEF25-75 values (p0.05). Nevertheless, at the end of the three-month carnitine therapy period these respiratory function parameters did not show any significant variation. Hence, it is implied that carnitine therapy might have prevented the subclinic bronchospasm that developed in children during hemodialysis.