Risk factors of congenital anomalies of the kidney and urinary tract (CAKUT): Exposure to mobile phones during pregnancy.

Background/aim: Congenital anomalies of the kidney and urinary tract(CAKUT) are the leading causes of childhood chronic kidney disease (CKD). The etiology of most of the cases is thought to be multifactorial. In this study, risk factors for CAKUT and the effect of mobile phone-related electromagnetic field (EMF) exposure during pregnancy were investigated. Materials and methods: Fifty-seven cases and 57 healthy controls under 2 years of age were included and their mothers were subjected to a questionnaire. Groups were compared for parents' demographics, pregestational (chronic disease, body mass index, use of the folic acid supplements) and antenatal variables (gestational disease, weight gain during pregnancy,) and exposures during pregnancy. To assess mobile phone-related radiation exposure, all participants were asked about their daily call time, the proximity of the phone when not in use, and the models of their mobile phones. The specific absorption rate (SAR) of the mobile phones and the effective SAR value (SAR × call time) as an indicator of EMF exposure were recorded. Results: Excess weight gain according to BMI during pregnancy was related to an increased risk of CAKUT (p=0.012). Folic acid use before pregnancy was protective for CAKUT (p = 0.028). The call time of mothers of the CAKUT group was significantly longer than the control (p = 0.001). An association was observed between higher effective SAR values and increased risk of CAKUT (p = 0.03). However the proximity of the mobile phone to the mother's body when not in use was not found as a risk factor. Conclusion: The etiology of CAKUT is multifactorial. Our results suggest that prolonged phone call and higher EMF exposure during pregnancy increases the risk of CAKUT in the offspring.

Gestational diabetes mellitus induces congenital anomalies of the kidney and urinary tract in mice by altering RET/MAPK/ERK pathway.

Gestational diabetes mellitus (GDM) presents a substantial population health concern. Previous studies have revealed that GDM can ultimately influence nephron endowment. In this study, we established a GDM mouse model to investigate the embryological alterations and molecular mechanisms underlying the development of congenital anomalies of the kidney and urinary tract (CAKUT) affected by GDM. Our study highlights that GDM could contribute to the manifestation of CAKUT, with prevalent phenotypes characterized by isolated hydronephrosis and duplex kidney complicated with hydronephrosis in mice. Ectopic ureteric buds (UBs) and extended length of common nephric ducts (CNDs) were noted in the metanephric development stage. The expression of Ret and downstream p-ERK activity were enhanced in UBs, which indicated the alteration of RET/MAPK/ERK pathway may be one of the mechanisms contributing to the increased occurrence of CAKUT associated with GDM.

Reproductive outcome of hysteroscopic metroplasty for women with T-shaped uterus: a retrospective study.

BACKGROUND: T-shaped uterus is a Müllerian malformation with unapparent clinical manifestations. Intrauterine adhesion and tuberculosis may lead to T-shaped uterus, too. Hysteroscopic metroplasty is a treatment option for T-shaped uterus, while the postoperative reproductive outcomes have not been thoroughly investigated. The aim of this study was to determine the reproductive outcome in Chinese women with T-shaped uterus who had hysteroscopic metroplasty with cold scissors. METHODS: This retrospective cohort study was conducted in the reproductive surgery unit of a university-affiliated hospital. One hundred and eleven patients with T-shaped uterus who underwent hysteroscopic metroplasty from Jan. 2017 to Sept. 2019 were followed-up by telephone in Apr. 2021. All patients received hysteroscopic metroplasty using microcissors, followed by estrogen-progesterone sequential treatment, with or without intrauterine device (IUD) implantation. According to whether they had had history of intrauterine operation, patients were divided into congenital group and acquired group. The main outcome measure was postoperative live birth rate. χ2 test and t test were used for comparison between groups. Cochran-Mantel-Haenszel test were used for stratified analysis. P < 0.05 was considered statistically significant. RESULTS: One hundred and eleven patients were included in total, with 46 in congenital group and 65 in acquired group. After hysteroscopic metroplasty, in the congenital group, the pregnancy rate increased from 28.3% to 87.0% (P < 0.001) and the live birth rate increased from 23.1% to 79.5% (P = 0.001); in the acquired group, the pregnancy rate slightly dropped from 98.5% to 72.3% (P < 0.001) while the live birth rate increased from 20.8% to 74.5% (P < 0.001). No statistically significant difference was observed in postoperative reproductive outcome indicators between the two subgroups except mode of conception. CONCLUSIONS: For both groups, hysteroscopic metroplasty may improve reproductive outcomes for patients with T-shaped uterus.

As a Müllerian malformation, T-shaped uterus is named for the shape of the uterine cavity. According to cause of the disease and patients' intrauterine operation history, T-shaped uterus can be divided into congenital and acquired types.This study was conducted in the reproductive surgery unit in a university-affiliated hospital. Data were collected from medical records, and patients were followed up via telephone.One hundred and eleven patients were included in this study, with 46 in congenital group and 65 in acquired group. Whether infertility/subfertility patients had had intrauterine operation history or not, their rates of giving live birth increased after the hysteroscopic metroplasty.In conclusion, hysteroscopic metroplasty is an effective intervention for T-shaped uterus patients with fertility intention.

Uterine synechiae after intrauterine device use: a case series.

PURPOSE: The intrauterine device (IUD) is one of the most effective and safe contraceptive methods. Substantial literature suggests an overall return to normal fertility following IUD removal. However, there are no studies to date that evaluate endometrial function specifically in nulliparous women after levonorgestrel IUD use. METHODS: We present three nulliparous women with a history of levonorgestrel IUD use who were evaluated for uterine dysfunction at the University of California, San Francisco Center for Reproductive Health. These patients had no other known risk factors or history of uterine manipulation, including prior uterine surgery, pelvic radiation, intrauterine infection, hypothalamic amenorrhea, or uterine anomaly. RESULTS: Upon evaluation, these patients were found to have uterine synechiae concerning for Asherman syndrome. All three patients were eventually able to conceive through assisted reproductive technology or natural conception. CONCLUSION: This case series is the first to suggest a possible effect of endometrial dysfunction on fertility resumption following levonorgestrel IUD removal in nulliparous patients. It is possible that a small subset of patients may be at risk for Asherman syndrome after IUD use. Larger prospective trials are needed to explore this possible association.

Posterior urethral valves masquerading as neuropathic bladder following sacrococcygeal teratoma resection.

Neuropathic bladder may be a co-associated morbidity in newborn babies following resection of a sacrococcygeal teratoma. We report a case of a male newborn showing features of incomplete urinary voiding requiring intermittent catheterisation after operation for bladder emptying. Videourodynamic assessment excluded neuropathic bladder and posterior urethral valves were demonstrated on micturating cystography. Urology outcomes have been excellent following curative valve ablation. This report highlights the crucial importance of being aware of the rare coexistence of lower urinary tract pathology in male babies with sacrococcygeal teratoma. Routine urodynamic assessment should be considered in all children following sacrococcygeal teratoma resection.

Definition, prevalence, clinical relevance and treatment of T-shaped uterus: systematic review.

OBJECTIVES: To summarize in a systematic review the current evidence regarding definitions, diagnosis, prevalence, etiology, clinical relevance and impact of surgical treatment for T-shaped uterus not related to diethylstilbestrol (DES) exposure, and to highlight areas on which future research should focus. METHODS: A search of PubMed, Scopus and EMBASE was performed on 9 April 2020 using the search terms 't-shaped OR t-shape OR infantile OR (lateral indentation) OR (diethylstilbestrol OR DES) AND (uterus OR uterine OR uteri) AND (anomaly OR anomalies OR malformation OR malformations)'. Additionally, the reference lists of the included studies were searched manually for other relevant publications. All studies presenting data on T-shaped uterus not associated with DES exposure and including at least 10 women were considered eligible. Studies regarding DES-related T-shaped uterus were excluded because DES has not been used since 1971. There were no restrictions on language, date of publication or status of publication. RESULTS: Of 2504 records identified by the electronic search, 20 studies were included in the systematic review. The majority of studies were of poor quality. In 11 of 16 studies reporting on the diagnosis of T-shaped uterus, the diagnostic method used was three-dimensional ultrasound. There is no consensus on the definition of T-shaped uterus, but the most cited criteria (4/16 studies) were of the European Society of Human Reproduction and Embryology and the European Society for Gynaecological Endoscopy (ESHRE/ESGE; 2013). The prevalence of T-shaped uterus varied from 0.2% to 10% in the four included studies reporting such data. With respect to etiology (except for DES), T-shaped uterus was considered a primary condition in three studies and secondary to adhesions in five and adenomyosis in one. T-shaped uterus was related to worse reproductive outcome based on subfertility (nine studies), miscarriage (seven studies), preterm delivery (two studies), ectopic pregnancy (one study) and repeat implantation failure (seven studies). Of the 12 studies that reported on the effects of surgical treatment of T-shaped uterus by hysteroscopic metroplasty, some mentioned an improvement in pregnancy rate (rates ranging from 49.6% to 88%; eight studies), live-birth rate (rates ranging from 35.1% to 76%; seven studies) and term-delivery rate (four studies) and a reduction in miscarriage (rates ranging from 7% to 49.6%; five studies) and ectopic pregnancy (one study). However, the evidence is of very low quality with serious/critical risk of bias toward overestimating the intervention effect. Some authors reported no complications related to the procedure, while others mentioned persistence of the dysmorphism (rates ranging from 1.4% to 11%; three studies), bleeding (1.3%; one study), infection (2.6%; one study) and adhesions (11.1% and 16.8%; two studies). CONCLUSIONS: The prevalence, etiology and clinical relevance, with respect to reproductive outcome, of T-shaped uterus remain unclear and there is no consensus on the definition and diagnostic method for this condition. Expectant management should be considered the most appropriate choice for everyday practice until randomized controlled trials show a benefit of intervention. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Assisted reproductive technologies and the risk of congenital urogenital tract malformations: A systematic review and meta-analysis.

A systematic review was conducted to evaluate the relationship between ART and the risk of urogenital tract malformations, especially hypospadias and cryptorchidism. Thirty-three papers were selected. Meta-analysis showed that ART was correlated with an increased risk of urogenital tract malformation in offspring (odds ratio (OR) = 1.61, 95% confidence interval (CI): 1.41-1.85), hypospadias (OR = 1.87, 95% CI: 1.47-2.40) and cryptorchidism (OR = 1.83, 95% CI: 1.54-2.18). Among offspring conceived by ART, multiple pregnancies appeared to result in a higher risk of urogenital tract malformation than singleton pregnancies (OR = 1.42, 95% CI: 0.99-2.04; P = 0.058). No significant difference in the risk of urogenital tract malformation was found between in vitro fertilization and intracytoplasmic sperm injection technologies. A sensitivity analysis showed that the above conclusions were relatively consistent. Conclusion: ART is correlated with an increased risk of urogenital tract malformations in offspring, especially hypospadias and cryptorchidism. Among ART offspring, multiple pregnancies may lead to a higher risk of urogenital tract malformations than singleton pregnancies, but more studies are needed for confirmation.

Roles for urothelium in normal and aberrant urinary tract development.

Congenital anomalies of the kidney and urinary tract (CAKUTs) represent the leading cause of chronic kidney disease and end-stage kidney disease in children. Increasing evidence points to critical roles for the urothelium in the developing urinary tract and in the genesis of CAKUTs. The involvement of the urothelium in patterning the urinary tract is supported by evidence that CAKUTs can arise as a result of abnormal urothelial development. Emerging evidence indicates that congenital urinary tract obstruction triggers urothelial remodelling that stabilizes the obstructed kidney and limits renal injury. Finally, the diagnostic potential of radiological findings and urinary biomarkers derived from the urothelium of patients with CAKUTs might aid their contribution to clinical care.

Pregnancy after mumps: a case report.

INTRODUCTION: Oophoritis, a complication of mumps, is said to affect only 5% of all postpubertal women. In this report, we present a case of a 31-year-old Iranian woman with amenorrhea and infertility due to an infantile uterus and atrophic ovaries associated with contracting mumps at a young age. She later successfully carried a healthy baby to term. CASE PRESENTATION: The patient was diagnosed with oophoritis when she was 8 years of age. She had no menses before treatment. The patient underwent a low-dose contraceptive treatment from age 19 until she was 31 years of age. During this period, the size of her uterus was constantly monitored, which revealed constant yet slow uterine growth. At age 31, Drospil (containing 3 mg of drospirenone and 0.03 mg ethinyl estradiol) treatment was initiated and administered for 3 months, which led to substantial uterine growth and menses. After her uterus had reached a mature size, the patient was referred to an assisted reproductive technology clinic. There she received a donor oocyte that was fertilized with the sperm of her husband. She had a successful low-risk pregnancy after the second embryo transfer. CONCLUSION: Low-dose contraceptive treatment containing progesterone, followed by Drospil, which includes both estradiol and progesterone, had a synergistic effect that led to the growth of the patient's uterus.

Renal abnormalities among children with sickle cell conditions in highly resource-limited setting in Ghana.

Sickle cell disease (SCD) is associated with progressive multi-organ failure especially, the brain and kidney and leads to high morbidity and mortality rate. The aim of this study was to determine the prevalence of renal abnormalities among children with SCD. This cross-sectional study recruited 212 sickling positive patients comprising of 96 Hb AS, 48 Hb SC, and 68 Hb SS phenotypes from the Pediatric Unit of Wassa Akropong Government Hospital, Wassa Akropong, Ghana. Early morning urine and venous blood samples were collected from each participant. Urinalysis was conducted and serum urea and creatinine levels were estimated. Estimate glomerular filtration rate (eGFR) was calculated using the Swartz equation. Classification of chronic kidney disease (CKD) was based on 'The Kidney Disease: Improving Global Outcomes (KIDIGO)' criteria. The mean age of the children were 7.90 years. Serum creatinine (p = 0.0310) and urea (p<0.0001) levels were significantly higher among Hb AS participants compared with Hb SS phenotype. The prevalent indicators of renal abnormalities were proteinuria (26.4%), urine granular cast (5.6%) and CKD (39.6%). Proteinuria, urine granular cast and CKD were most prevalent among Hb SS (47.1%, 11.8% and 73.5% respectively) compared with Hb SC (41.7%, 8.3%, and 45.8% respectively) and Hb AS (4.2%, 0.0%, and 14.5%) phenotypes, respectively. Sickle cell conditions were significantly associated with proteinuria (p<0.0001) and CKD (p = 0.0378). Children with Hb SS [aOR = 5.04, 95% CI (2.47-10.3); p<0.0001] and Hb SC [aOR = 3.14 95% CI (1.39-7.01); p = 0.0174] were at increased odds of developing CKD after adjusting for age, BMI and gender. Proteinuria and CKD are associated with sickle cell disease (Hb SC and Hb SS). Renal function should be routinely monitored for children with SCD.

CAKUT and Autonomic Dysfunction Caused by Acetylcholine Receptor Mutations.

Congenital anomalies of the kidney and urinary tract (CAKUT) are the most common cause of chronic kidney disease in the first three decades of life, and in utero obstruction to urine flow is a frequent cause of secondary upper urinary tract malformations. Here, using whole-exome sequencing, we identified three different biallelic mutations in CHRNA3, which encodes the α3 subunit of the nicotinic acetylcholine receptor, in five affected individuals from three unrelated families with functional lower urinary tract obstruction and secondary CAKUT. Four individuals from two families have additional dysautonomic features, including impaired pupillary light reflexes. Functional studies in vitro demonstrated that the mutant nicotinic acetylcholine receptors were unable to generate current following stimulation with acetylcholine. Moreover, the truncating mutations p.Thr337Asnfs∗81 and p.Ser340∗ led to impaired plasma membrane localization of CHRNA3. Although the importance of acetylcholine signaling in normal bladder function has been recognized, we demonstrate for the first time that mutations in CHRNA3 can cause bladder dysfunction, urinary tract malformations, and dysautonomia. These data point to a pathophysiologic sequence by which monogenic mutations in genes that regulate bladder innervation may secondarily cause CAKUT.

Radiographic abnormalities, bladder interventions, and bladder surgery in the first decade of life in children with spina bifida.

BACKGROUND: Spina bifida (SB) patients are at increased risk for hydronephrosis, bladder storage and emptying problems, and renal failure that may require multiple bladder surgeries. METHODS: We retrospectively reviewed patients born with SB 2005-2009, presenting to our institution within 1 year of birth. Outcomes at 8-11 years old included final renal/bladder ultrasound (RBUS) results, clean intermittent catheterization (CIC) use, anticholinergic use, surgical interventions, and final renal function. We excluded those without follow-up past age 8 and/or no RBUS or fluoroscopic urodynamic images (FUI) within the first year of life. Imaging was independently reviewed by four pediatric urologists blinded to radiologists' interpretation and initial findings compared with final outcomes. RESULTS: Of 98 children, 62 met inclusion criteria (48% male, 76% shunted). Median age at last follow-up was 9.6 years. Upon initial imaging, 74% had hydronephrosis (≥ SFU grade 1), decreasing to 5% at 10 years (p < 0.0001). Initially, 9% had ≥ SFU grade 3 hydronephrosis, decreasing to 2% (p = 0.13). CIC and anticholinergic use increased from 61% and 37% to 87% and 86%, respectively (p = 0.001 and p < 0.0001, respectively). With follow-up, 55% had surgical intervention and 23% had an augmentation. Of children with a serum creatinine/cystatin-C at 8-11 years old, one had confirmed chronic kidney disease (stage 2). CONCLUSIONS: Despite initial high incidence of hydronephrosis, this was low grade and resolved in the first decade of life. Additionally, the 8-11-year incidence of kidney disease and upper tract changes was low due to aggressive medical management.

Association of Sexually Transmitted Diseases With Cervical Ectopy: A Systematic Review.

OBJECTIVE: The aim of this study was to review the available literature to explore evidence indicating an association between cervical ectopy and sexually transmitted diseases, which could help in the decision to treat or not to treat this condition. METHODS: A review of the literature was conducted using the PubMed, EMBASE and clinicaltrials.gov databases on ectopy of the cervix using the terms "ectopy OR ectropium AND cervix" filtered only by language, without limit of date. A total of 71 studies were found in the initial selection, of which 56 were deleted by title, abstract, or full text. The remaining 15 articles were analyzed in this study. RESULTS: Cervical ectopy showed a positive association with human papillomavirus, human immunodeficiency virus, bacterial vaginosis, cervical epithelial atypia, postcoital bleeding, and desquamative inflammatory vaginitis. High-quality studies reported no association between ectopy and chlamydia infection. It was also not associated with gonococcal infection and herpes simplex. CONCLUSIONS: Cervical ectopy shows a probable association with increased acquisition of some sexually transmitted diseases. Additional studies are required to confirm the possible beneficial effects of treatment and to evaluate the possible complications of these treatments.

Incidental finding of Zinner syndrome in a Greek military recruit: a case report of a rare clinical entity.

BACKGROUND: Zinner syndrome represents a rare congenital malformation of the urinary tract. It comprises a constellation of Wolffian duct anomalies and is almost exclusively encountered as a classic triad of seminal vesicle cysts, ejaculatory duct obstruction and renal agenesis. Patients can be either asymptomatic or symptomatic. Recently, minimally invasive surgical techniques have emerged, superseding traditional surgery for select symptomatic cases. Our case highlights the finding of a rare clinical syndrome that was incidentally detected during a routine mass screening of military recruits in the Greek Armed Forces. CASE PRESENTATION: Herein, we present a case of a 19-year-old male who reported having a solitary right kidney when examined in a military training center of Northern Greece. No additional clinical information was available; thus, referral to a tertiary urology department for further investigation ensued. Imaging studies, namely, computed tomography and magnetic resonance imaging, revealed left renal aplasia, multiple left seminal vesicle cysts, and ejaculatory duct obstruction. Laboratory values and urinalysis were within normal range. Semen analysis was significant for cryptozoospermia. Our patient remained asymptomatic during the entire hospitalization. Long-term follow-up was recommended. Nevertheless, he declined further investigation and sought treatment in a private practice setting. CONCLUSIONS: This article aims to present the incidental diagnosis of a rare syndrome in a military setting. Population screening conducted in the armed forces permits the identification of undiagnosed diseases that warrant further investigation. To the best of our knowledge, this was the first report of Zinner syndrome in a military recruit and the second case cited of a Greek patient in the published literature. Regular follow-up is the key to timely intervention in conservatively managed cases.

Scimitar syndrome associated with aberrant right subclavian artery, diaphragmatic hernia, and urinary anomalies - case report and review of the literature.

Scimitar syndrome is a form of a partially or totally right pulmonary venous return to the inferior vena cava, which may associate variably right lung hypoplasia, right pulmonary artery hypoplasia, pulmonary sequestration together with the presence of aortopulmonary collaterals from the descending aorta towards the right lung. In many cases, there are also other cardiac anomalies associated. We present a unique association of a partially anomalous pulmonary venous return to the inferior vena cava with other vascular and thoracic anomalies: inferior sinus venosus and secundum atrial septal defect, retroesophageal right subclavian artery, obstructed accessory right bronchus, diaphragmatic hernia with ectopic liver, "S"-type thoracic scoliosis and malformations of the urinary tract (duplication of the right ureter and of the left basinet). The patient had a reimplantation of the "scimitar" vein to the left atrium and closure of the inferior sinus venosus and secundum atrial septal defect.

Solitary functioning kidney in children: clinical implications / Rim solitário em funcionamento em crianças: implicações clínicas

ABSTRACT Introduction: Children with solitary functioning kidney (SFK) are prone to develop long term problems, which are not well represented in the literature. The extent to which the presence of associated congenital anomalies of kidney and urinary tract (CAKUT) further de-stabilize renal function is to be addressed. Objective: This study was conducted to evaluate the etiology, presentation, presence of CAKUT, and renal damage in children with SFK. Methods: All children with SFK who presented to the department of pediatric surgery from March 2014 to May 2016 were included in the study. Children with malignancy were excluded from the study. Results: Of the 20 patients with SFK, 14 (70%) had primary SFK (8 with agenesis and 6 with multicystic dysplastic kidney), 6 (30%) belonged to secondary SFK group, among them 3 had pelviureteric junction obstruction, 2 had posterior urethral valves and 1 had vesicoureteric reflux. Eight (40%) had associated CAKUT, 4 (20%) were asymptomatic while 8 (40%) had UTI and 6 (30%) had hypertension. Ten (50%) patients had reduced glomerular filtration rate (GFR) suggesting compromised renal function. Conclusion: Children with SFK have high morbidity especially when associated with ipsilateral CAKUT. Long-term periodical follow up is essential in these patients to improve clinical outcome.

RESUMO Introdução: Crianças com rim solitário funcional (RSF) tendem a desenvolver problemas de longo prazo, que não são bem representados na literatura. Devemos abordar o quanto a presença de malformações congênitas do rim e do trato urinário (CAKUT) desestabiliza ainda mais a função renal. Objetivo: Este estudo foi realizado para avaliar a etiologia, apresentação, presença de CAKUT e dano renal em crianças com RSF. Métodos: Todas as crianças com RSF que se apresentaram no departamento de cirurgia pediátrica de março de 2014 a maio de 2016 foram incluídas no estudo. Crianças com doença maligna foram excluídas do estudo. Resultados: dos 20 pacientes com RSF, 14 (70%) tinham RSF primário (8 com agenesia e 6 com rim displásico multicístico), 6 (30%) pertenciam ao grupo RSF secundário, dentre eles 3 tinham obstrução da junção pelveuretérica, 2 tinham valvas uretrais posteriores e 1 refluxo vesico-uretral. Oito (40%) tinham ACRTU associado, 4 (20%) estavam assintomáticos, enquanto 8 (40%) tinham ITU e 6 (30%) tinham hipertensão. Dez (50%) pacientes apresentaram redução da taxa de filtração glomerular (TFG), sugerindo comprometimento da função renal. Conclusão: Crianças com RSF apresentam alta morbidade, especialmente quando associadas à CAKUT ipsilateral. O acompanhamento periódico a longo prazo é essencial nesses pacientes para melhorar o resultado clínico.

Effects of assisted reproductive technologies in neonates with indications for surgery (2007-2016).

PURPOSE: Recently, the number of births using assisted reproductive technologies (ART) has increased. An associated increase in the incidence of congenital malformations in babies conceived using this technology has also been reported. Therefore, we aimed to investigate the rate of malformations in babies with neonatal surgical diseases, who were conceived using ART. MATERIALS AND METHODS: Between January 2007 and December 2016, 1737 patients were admitted to our hospital. We analyzed the incidence of congenital cardiac diseases, genetic anomalies, and congenital anomalies of the kidney and urinary tract (CAKUT) in neonates conceived by ART. The χ2 test and logistic regression analysis were used to assess the odds ratios (ORs) for congenital malformations. A P-value < 0.05 indicated statistical significance. RESULTS: The OR for CAKUT was 16.94 for the first-birth neonates conceived using ART, [P < 0.05, AUC (area under the curve) = 0.86]. However, for non-surgery neonates, the OR for CAKUT was 5.99 (P = 0.15, AUC = 0.87), compared to 32.27 (P < 0.05, AUC = 0.93) for parallel conditions in surgery-neonates. CONCLUSION: Neonates conceived using ART are prone to develop CAKUT, which will need surgical treatment. Therefore, more management is necessary for associated malformations in these babies, particularly in cases with CAKUT.

Solitary functioning kidney in children: clinical implications.

INTRODUCTION: Children with solitary functioning kidney (SFK) are prone to develop long term problems, which are not well represented in the literature. The extent to which the presence of associated congenital anomalies of kidney and urinary tract (CAKUT) further de-stabilize renal function is to be addressed. OBJECTIVE: This study was conducted to evaluate the etiology, presentation, presence of CAKUT, and renal damage in children with SFK. METHODS: All children with SFK who presented to the department of pediatric surgery from March 2014 to May 2016 were included in the study. Children with malignancy were excluded from the study. RESULTS: Of the 20 patients with SFK, 14 (70%) had primary SFK (8 with agenesis and 6 with multicystic dysplastic kidney), 6 (30%) belonged to secondary SFK group, among them 3 had pelviureteric junction obstruction, 2 had posterior urethral valves and 1 had vesicoureteric reflux. Eight (40%) had associated CAKUT, 4 (20%) were asymptomatic while 8 (40%) had UTI and 6 (30%) had hypertension. Ten (50%) patients had reduced glomerular filtration rate (GFR) suggesting compromised renal function. CONCLUSION: Children with SFK have high morbidity especially when associated with ipsilateral CAKUT. Long-term periodical follow up is essential in these patients to improve clinical outcome.

Esophageal Atresia with or without Tracheoesophageal Fistula (EA/TEF): Association of Different EA/TEF Subtypes with Specific Co-occurring Congenital Anomalies and Implications for Diagnostic Workup.

BACKGROUND: Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) represents the most common developmental malformation of the upper digestive tract. It is classified into six subtypes according to the classification of Vogt, depending on anatomical variation of this malformation. Around 50% of the patients with EA/TEF present additional anomalies, which often influence, next to the EA/TEF subtype, the overall prognosis of EA/TEF newborns. Here, we investigated the association of the different EA/TEF subtypes with co-occurring congenital anomalies in EA/TEF patients and demonstrate their implications for postnatal diagnostic workup. MATERIALS AND METHODS: We investigated 333 patients of a large German multicenter study born between 1980 and 2012. After evaluation of all available clinical records, 235 patients were included in our analysis. We compared our results with existing data. RESULTS: The highest risk for co-occurring anomalies was seen in patients with most common Vogt 3b (p = 0.024), especially for additional gastrointestinal anomalies (p = 0.04). Co-occurring anomalies of the skin were significantly more common in patients with subtype Vogt 2 (p = 0.024). A significant correlation was observed for an impaired neurodevelopmental outcome and EA/TEF Vogt 3a (p = 0.041). Patients with EA/TEF showed a higher risk to present with any additional congenital anomaly compared with the general population (p < 0.001). CONCLUSION: Our results warrant thorough clinical workup for gastrointestinal anomalies especially in patients with Vogt 3b. Moreover, it might be necessary to focus on a thorough aftercare for neurocognitive development in patients with Vogt 3a. The here presented observations need to be confirmed by future studies.