Combination of myasthenia gravis and HELLP Syndrome in pregnancy: case report and literature review

Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction. Preeclampsia/HELLP Syndrome is a potentially life-threatening pregnancy complication. The combination of HELLP Syndrome and MG is challenging because the preferred treatment regimens for both conditions generally contradict each other. Our aim is to describe the management options when these two diseases occur simultaneously. We present a case in which a woman with an established diagnosis of MG developed HELLP Syndrome at 31 weeks gestation. Magnesium sulfate prophylaxis was not utilized because of the patient’s MG diagnosis. A cesarean delivery was performed. Reported cases with combined diagnoses of MG and preeclampsia/HELLP Syndrome are reviewed and adjustments to treatment plans are discussed. Management of such patients should be done with a multidisciplinary approach in advanced medical centers with careful consideration of the medications used

Acute pancreatitis in children: A single center experience over ten years

Acute pancreatitis (AP) is an inflammatory disease characterized by sudden onset abdominal pain together with elevation of pancreatic enzymes and radiographic changes. Increased incidence of AP in children have been reported in recent reports. In this study; we aimed to analyze the demographic characteristics, etiology, outcome and incidence of AP among hospitalized children in our center. Medical records of the children with AP since January 2005 were analyzed from hospital files (N=63). Major etiologies were systemic diseases (14.3%), trauma (11.1%), cholelithiasis (9.5%); 54% (N=34) of the patients had mild AP, while 28.6% (N=18) had moderately severe AP and 17.4% (N=11) had severe AP. Organ dysfunction was found in 11 patients (17.4%) at initial examination. During the follow-up period (68.1 +/- 24.3 months), 10 patients (15.9%) experienced 24 recurring AP (RAP) attacks. Male gender, presence of local pancreatic or systemic complications at initial attack, metabolic and hereditary diseases were associated with the increased risk of RAP (p<0.05 for all). The mortality rate associated with AP was 4.84%. There was an increase in the incidence of AP since 2010 (9.57 in 2009-2010 vs. 39.17/10,000 patients in 2015-2016 years; p=0.0002; OR: 4.1) among the hospitalized patients. Our results indicate that AP is a mild disease in children and the incidence is increasing among hospitalized children. Male gender, presence of local pancreatic or systemic complications at initial attack, metabolic diseases and hereditary diseases were associated with the increased risk of RAP

Urinary tract infections in neonates with unexplained pathological indirect hyperbilirubinemia: Prevalence and significance

Background: It is controversial to test for urinary tract infection (UTI) in patients with unexplained indirect hyperbilirubinemia in the first 2 weeks of life. We aimed to study the prevalence and significance of UTIs in such neonates who were requiring phototherapy. Methods: Subjects were 2- to 14-day-old neonates with indirect bilirubin levels above phototherapy limit with no other abnormality in their bilirubinaemia-related etiologic workup. UTI was diagnosed by 2 consecutive positive cultures obtained by catheterisation, documenting growth of >10,000 colonies of the same microorganism with consistent antibiograms. The UTI (+) patients were evaluated by renal ultrasonography (US), and some were followed up for possible recurrent UTI. Results: 262 neonates were included in the study. UTI prevalence was 12.2%, and bacteraemia was 6.2% among UTI (+) patients. The two most common pathogens (81.2%) were Escherichia coli and Klebsiella. pneumonia. All UTI (+) patients had undergone US, revealing 12.5% pelvicaliectasis, other 12.5% increased renal parenchymal echogenicity, 3.1% concurrent pelvicaliectasis and increased renal parenchymal echogenicity. 53.1% of UTI (+) patients had undergone follow-up, after which 23.5% recurrent UTI were found at the end of a mean of 52 months. Conclusion: We suggest that the neonates with unexplained pathological jaundice should be tested for possible UTI. Consequently, all newborns with UTI shall be evaluated by the urinary US and followed up for recurrent UTI

Karaciğer fibrokistik hastalıklarının değerlendirilmesi tek merkez deneyimi

Amaç: Karaciğerin fibrokistik hastalığı (KFKH), intrauterin dönemden adölesan yaşa kadar geniş bir yaş aralığındagörülebilen, multisistemik bir hastalıktır. Çalışmadaki amacımız kliniğimizde KHFK olan hastaların, başvuru semptomları,klinik-laboratuvar bulguları, tedavi yaklaşımı ve takip sonuçlarını değerlendirmektir.Gereç ve Yöntemler: Ocak 2008-Aralık 2019 yılları arasında, Çocuk Gastroenteroloji, Hepatoloji ve Beslenmepolikliniğinde KFKH nedeniyle takipli olan hastaların demografik özellikleri, klinik-laboratuar bulguları, tedavi yaklaşımlarıve son durumları geriye dönük olarak incelendi.Bulgular: Otuz dokuz hastanın (56.4 erkek, ortanca yaş 5 yıl 3 ay, yaş aralığı: 10 gün-6.8 yıl) sekizinde (20.5) Carolihastalığı (CH), 16’sında (41) konjenital hepatik fibrozis (KHF), 15’inde koledok kisti tespit edildi. En sık başvuru şikayetisarılık (n=8, 20.5), kronik karın ağrısı (n=6, 15.4) ve splenomegali (n=4, 10.3)’dü. Hastaların sekizi (20.5) böbrektekist tespit edildikten sonra yapılan incelemelerde, yedisi (17.9) intrauterin dönemde, ikisi (5.1) insidental olarak tespitedilmişti. Otozomal resesif polikistik böbrek hastalığı (ORPBH) olan altı hastada PKHD1 gen mutasyonu saptandı. On sekizhasta (46.2) opere edildi (karaciğer nakli, sol lob segmental hepatektomi, mezokavalşant, böbrek nakli, kistektomi).Yirmi beş hastada (64.1) ekstrahepatik tutulum mevcuttu [ORPBH (n=18), mental motor retardasyon (n=2, birindemetokromatik lökodistrofi, diğerinde Arnold Chiari malformasyonu), nefrokalsinozis (n=1), juvenil nefronofitizis (n=1),akut pankreatit (n=1), pulmoner hipoplazimetakarpal distal falanks hipoplazisi (n=1) ve medüler sünger böbrekpinealkist (n=1)]. Takip edilen 39 hastanın altısında portal hipertansiyon, beşinde kronik böbrek yetmezliği (12.8), dördündekompanse kronik karaciğer hastalığı (10.3) gelişmiş olup iki hastaya dekompanse siroz nedeniyle karaciğer nakli, birhastaya son dönem böbrek yetmezliği nedeniyle böbrek nakli yapıldı.Sonuç: Karaciğerin fibrokistik hastalıklarında, morbidite ve komplikasyon riskinin yüksek olması nedeniyle erken tanı,düzenli takip ve tedavi önemlidi

AUTOSOMAL-DOMINANT POLYCYSTIC KIDNEY DISEASE IN CHILDREN: PRELIMINARY RESULTS OF A TURKISH NATIONAL, MULTICENTER STUDY

WOS: 000443998400522

The predictive nature of uterocervical angles in the termination of second trimester pregnancy

Aim: To establish how useful and the predictive capacity of uterocervical angles (UCA) in the termination of second trimester pregnancies. Material and methods: This prospective cohort study was conducted at a tertiary center with a total of 120 singleton pregnancies delivered between 14 and 24 gestational weeks. Before the beginning of misoprostol induction, patients were screened for both cervical length (CL) and uterocervical angles (UCA). The UCA is defined as an angle constructed by the measurement of the cervical canal and lower uterine segment. The study population was subdivided into four groups; successful and failed terminations at the end of 24 hours of induction and successful and failed terminations at the end of 48 hours of induction. We decided to further evaluate our study population based on their UCAs, and placed them into four categories; UCA >= 95 degrees, UCA = 105 degrees, and UCA Results: In the 24-hour time frame group, the mean UCA was 105.50 +/- 15.38 degrees in the successful termination group and was 100.22 +/- 11.12 degrees in the failed group (p = .001). In the 48-hour time frame group, the mean UCA was 104.19 +/- 13.51 degrees in the successful termination group and was 93.52 +/- 7.84 degrees in the failed group (p = .007). The mean hour of induction was shortest in the UCA >= 105 degrees group. Conclusions: Regardless of the time frames, patients who had successful terminations had a broader angle, less amount of misoprostol use and shorter duration of induction as compared to the failed termination groups. What do the results of this study add? The uterocervical angle has never been measured in second trimester pregnancies to predict the timing of termination. Our study demonstrated the useful application of this ultrasonographic finding in the prediction of successful second trimester terminations

Atypical Hemolytic Uremic Syndrome in Children Aged < 2 Years

Agbas, Ayse/0000-0002-3658-8622; Ozcakar, Zeynep/0000-0002-6376-9189; Cakar, Nilgun/0000-0002-1853-0101WOS: 000437355600002PubMed: 29533929Background: There are limited data on infants with atypical hemolytic uremic syndrome (aHUS). The aim of this study was to determine the clinical and laboratory features, and to evaluate treatment modalities and outcomes in infants with aHUS. Materials and Methods: Relevant data on patients with onset of aHUS at age <2 years were obtained from the Turkish Pediatric aHUS Registry. Results: Among the 146 patients included in the Registry, 53 (36%) (23 male and 30 female) were enrolled for the study. Age at disease onset was <= 1 year in 29 of the patients. In all, 21 (40%) of the patients developed neurological symptoms. Disease-causing muta tions were noted in 14 (36%) of the 39 patients in which genetic analysis was performed. Plasma therapy was performed in 42 (79%) patients; eculizumab therapy was administered to treat the first episode of aHUS in 33 (62%) patients and in 5 patients as the first- line therapy. In total, 38 (72%) patients received renal replacement therapy (RRT), 3 (6%) died due to acute illness, and 4 (8%) were discharged from hospital with RRT. Follow-up visit data were available for 46 patients and the median duration was 23 months (range 3-129 months). End-stage renal disease developed only in 1 patient. Proteinuria and hypertension persisted in 17 (37%) and 20 patients (44%) respectively. Eculizumab treatment was continued in 25 of the 39 patients during the follow-up period. Conclusion:One-third of the aHUS patients had disease onset during infancy. The prognosis of this life-threatening disease seems to get better with improved treatment modalities. (C) 2018 S. Karger AG, BaselHacettepe University Scientific Research and Development OfficeHacettepe University [010A101009]; Alexion Pharmaceuticals, Inc.Fukuda Foundation for Medical TechnologyKayamori FoundationThis study was supported by Hacettepe University Scientific Research and Development Office (grant No.: 010A101009). The article processing charge of this manuscript has been granted by the Alexion Pharmaceuticals, Inc

Could Pyelonephritic Scarring Be Prevented by Anti-Inflammatory Treatment? An Experimental Model of Acute Pyelonephritis

Objectives. This study aimed to demonstrate if the addition of anti-inflammatory treatment to antibiotic therapy shows any superiority to the treatment with antibiotic only. Methods. Forty-nine Wistar rats were divided into 7 groups. Pyelonephritis was performed by E. coli injection to upper pole of kidneys except control group. Group 2 was not treated. Ceftriaxone, ketoprofen, “ceftriaxone + ketoprofen,” methylprednisolone, and “ceftriaxone + methylprednisolone” were given in the groups. The technetium-99m-dimercaptosuccinic acid scintigraphies were performed in 3rd day to detect pyelonephritis and 10th week to detect renal scarring. All kidneys were also histopathologically evaluated. Results. When 3rd day and 10th week scintigraphies were compared, initial 2.00 ± 0.30 point pyelonephritis score resulted in 0.71 ± 0.36 renal scar score in “ceftriaxone + ketoprofen” group (P=0.039). Initial 2.00 ± 0.43 point pyelonephritis score resulted in 0.86 ± 0.26 renal scar score in “ceftriaxone + methylprednisolone” group (P=0.041). Renal scar score was declined in “ceftriaxone + ketoprofen” group and “ceftriaxone + methylprednisolone” group compared with no-treatment group on 10th week of the study (P=0.026, P=0.044). On histopathological evaluation, it was seen that renal scar prevalence and expansion declined significantly in “ceftriaxone + ketoprofen and ceftriaxone + methylprednisolone” (P=0.011, P=0.023). Conclusion. It was evidenced that ceftriaxone treatment in combination with ketoprofen or methylprednisolone declined scar formation in scintigraphic and histopathologic examinations of the kidneys

Reference Ranges of Serum Blood Urea Nitrogen, Creatinine Concentration and Ultrasonographic Measurement of the Kidneys in Term Healthy Newborns in the Neonatal Period

Objective: Acute kidney injury is an important problem in neonates. We conducted a cross-sectional prospective study to determine normal serum blood urea nitrogen, creatinine reference ranges and ranges of ultrasonographic measurement of kidneys in healthy term newborns. Study Design: Blood samples were collected from total 357 healthy newborns at birth (n=45), 1st (n=30), 3rd (n=61), 7th (n=34), 10th (n=132), 14th (n=36), and 28th (n=19) days of life. Renal ultrasonographic was performed by the same two radiologists on 81 newborns aged 10 days. Results: Serum blood urea nitrogen and creatinine concentrations have reached to the highest level at the first day of life and have returned to cord level at the third day of life. There were gradually decrease in serum blood urea nitrogen and creatinine levels after the first day of life. There were significant difference in both right and left renal length, width, and volumes in terms of gender and these parameters were statistically higher in boys than girls (p<0.05). Birth weight of the boys (3548±539g) was statistically higher than girls (3307±405 g) (p=0.028). There was a positive correlation between birth weight and right (r=0.38, p=0.000) and left kidney volumes (r=0.44, p=0.000). Conclusion: Our findings showed that measured blood urea nitrogen and creatinine levels changed in accordance with postnatal days and there was a positive correlation between kidney volume and birth weight of newborns. We concluded that these findings are important for evaluation of acute kidney injury and for screening of for urinary tract anomalies in neonate