Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “The discovery of novel GCK mutations represents a major strength of this study, as this gene exhibits one of the most heterogeneous mutational spectra among all MODY-related genes, with more than 600 distinct variants described worldwide [ 32 , 38 ].”
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “12 In this study, we reported an extremely rare case of simultaneous presentation of MRKH syndrome, left inguinal hernia, and left renal fusion with malrotation, and reported our experience in the diagnosis and treatment of such conditions.”
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “In this study, we reported an extremely rare case presenting with MRKH syndrome, inguinal hernia, left renal fusion, and malrotation.”
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “Whole-exome sequencing identifies a GREB1L variant in a three-generation family with Müllerian and renal agenesis: a novel candidate gene in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome.”
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “This study aimed to evaluate the genetic and clinical features of pediatric patients with MODY, to assess the prevalence of common and rare subtypes, and to report novel pathogenic variants identified in a Turkish cohort.”
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “The identification of novel GCK variants and the detection of rare subtypes further expand the mutational and phenotypic spectrum of pediatric monogenic diabetes.”
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “Moreover, data regarding novel mutations and rare MODY subtypes remain limited in the literature.”
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “The objectives were to define the clinical and phenotypic variability within this cohort, identify previously unreported (novel) mutations, and determine the prevalence of rare MODY subtypes.”
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “To the best of our knowledge, this is the first report on inverted SI of kidney cortex and medulla on fetal MRI.”
yesterday
Phenotype expansion
Signs & symptoms
conf 97%
A source describes a new or expanded observation: “We describe an additional patient with 2q12.1q14.1 microdeletion, showing MRKH and congenital hypothyroidism due to thyroid gland hypoplasia.”
yesterday
Phenotype expansion
Signs & symptoms
conf 97%
A source describes a new or expanded observation: “In this study, we report a second case of 2q12.1q14.1 microdeletion, involving PAX8 as a gene associated with Müllerian agenesis in a MRKH I and hypothyroidism.”
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “Emerging evidence suggests that extrarenal features in HNF1B -related disorders may also exhibit genotype-dependent patterns, much like the renal phenotypes [ 5 ].”
yesterday
Phenotype expansion
Signs & symptoms
conf 97%
A source describes a new or expanded observation: “We report five patients with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS), four of whom presented with precocious puberty and one with growth hormone deficiency (GHD.”
yesterday
Phenotype expansion
Research frontier
conf 97%
A source describes a new or expanded observation: “CONCLUSIONS Existing disease candidate gene prioritization methodologies mine biological and functional information about candidate genes, and we believe that our ToppGene Suite can complement these existing approaches by applying novel methods that mine mouse...
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “The most intriguing and novel finding in our study is the unexpected postnatal complete sonographic resolution of renal parenchymal hyperechogenicity, without evolution to cystic changes among unsuspected prenatal cases.”
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “Another novel finding among our patients is the coexistence of both types of HNF1B- associated kidney abnormalities, i.e., cystic kidneys and CAKUT, which presented sequentially in Patient 3.”
yesterday
Phenotype expansion
Signs & symptoms
conf 97%
A source describes a new or expanded observation: “Although MIEN1 is regarded as a novel gene involved in prostate cancer progression by enhancing cell migration and invasion, the relative functional significance and regulatory mechanisms of MIEN1 remain largely unknown.”
yesterday
Phenotype expansion
learning, speech and behaviour
conf 97%
A source describes a new or expanded observation: “Conclusions This is the first report of social anxiety disorder in ACC patients.”
yesterday
Phenotype expansion
learning, speech and behaviour
conf 97%
A source describes a new or expanded observation: “Discussion and conclusions Here we reported a 17-year-old Chinese juvenile who was suffering from ACC with comorbid social anxiety disorder, and accompanied by behavior problems, psychiatric symptoms.”
yesterday
Phenotype expansion
learning, speech and behaviour
conf 97%
A source describes a new or expanded observation: “That is, children with high behavioral inhibition (BI), which refers to caution, fear, low rate of approach, and passive withdrawal in novel situations [ 39 ], may evolve to social phobia in their growth.”
yesterday
Phenotype expansion
learning, speech and behaviour
conf 97%
A source describes a new or expanded observation: “In summary, this is the first report of an ACC patient combined with social anxiety disorder.”
yesterday
Phenotype expansion
learning, speech and behaviour
conf 97%
A source describes a new or expanded observation: “Prenatal identification of a novel mutation in the MCPH1 gene associated with autosomal recessive primary microcephaly (MCPH) using next generation sequencing (NGS): a case report and review of the literature.”
yesterday
Phenotype expansion
learning, speech and behaviour
conf 97%
A source describes a new or expanded observation: “36 Importantly, NGS performed on CVS samples has enabled the identification of rare monogenic disorders, including a novel MCPH1 mutation associated with autosomal recessive primary microcephaly, highlighting the value of early sampling combined with...
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “We report the case of a 60-year-old Japanese woman with genetically confirmed maturity-onset diabetes of the young type 5 [MODY5; HNF1B (hepatocyte nuclear factor 1B)-MODY] who developed amyotrophic lateral sclerosis (ALS), a progressive neurodegenerative disorder.”
yesterday
Phenotype expansion
Signs & symptoms
conf 97%
A source describes a new or expanded observation: “This disorder has an incidence of approximately 1 in 4500 newborn girls and the aetiology is poorly understood. we report on two patients affected by MRKH syndrome in which array-CGH analysis disclosed an identical deletion spanning 1.5 Mb of genomic DNA at...
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “Emerging evidence describes a "paradoxical" phenomenon where specific LoF mutations, initially causing hyperinsulinemic hypoglycemia in infancy, predispose individuals to glucose intolerance or frank diabetes in later life, often classified as Maturity-Onset...
yesterday
Phenotype expansion
kidney and urinary tract
conf 97%
A source describes a new or expanded observation: “Emerging evidence suggests that GLP-1 receptor agonists can mitigate ER stress and preserve remaining β-cell function, shifting the management paradigm from pure insulin replacement to disease-modifying therapy [ 43 ].”
yesterday
Frequency / statistic
Research frontier
conf 97%
A source reports “99.9%” in the context of general: “HiFi sequencing is 99.9% accurate, with occasional low-frequency false base calls.”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “0.005%” in the context of neurodevelopment: “Exome-wide burden analysis To evaluate the excess of rare, deleterious protein-coding variants in individuals with epilepsy, we performed burden analysis across the entire exome, at both an individual-gene and a gene-set level. "Ultra-rare" variants (URVs) wer”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “90%” in the context of neurodevelopment: “This resulted in 48,767 samples (~90% of initial) for the downstream burden analysis, which comprises 18,963 epilepsy cases (including 1,743 DEEs, 4,980 GGE, and 8,425 NAFE) and 29,804 controls.”
yesterday
Frequency / statistic
Research frontier
conf 97%
A source reports “0.1%” in the context of general: “To mitigate false positives, we used previously benchmarked filtering thresholds, where CNVs had to span >4 callable exons and had a site frequency <0.1% and a quality score >200.”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “000 individuals” in the context of neurodevelopment: “1 Epilepsy has a prevalence of 4-10 per 1,000 individuals worldwide, making it one of the most common neurological conditions.”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “979 individuals” in the context of neurodevelopment: “After stringent quality control (QC; Methods ), we included a total of 20,979 individuals with epilepsy and 33,444 controls without known neurological or neuropsychiatric conditions in our URV association analysis, roughly doubling the sample...
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “938 individuals” in the context of neurodevelopment: “We performed the association analyses separately for each epilepsy subtype - including 1,938 individuals with a diagnosis of a DEE, 5,499 with GGE, and 9,219 with NAFE - as well as for all epilepsy-affected individuals combined (including an...
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “94.1%” in the context of neurodevelopment: “The vast majority (128/136=94.1%) were DD/ASD genes, and only one gene - KDM6B (MIM: 611577) - was shared by all three NDD gene sets.”
yesterday
Frequency / statistic
Research frontier
conf 97%
A source reports “69.4%” in the context of general: “It is reported that the incidences of transient non-visualization, GB agenesis, biliary atresia, cystic fibrosis and chromosomal abnormalities of isolated NVFGB in western populations were 69.4%, 24.7%, 3.5%, 2.4% and 1.4%, respectively [ 6 ].”
yesterday
Frequency / statistic
Research frontier
conf 97%
A source reports “5.9%” in the context of general: “Qian et al. reported an overall detection rate of 5.9% for CMA-related BTS anomalies in foetuses [ 9 ].”
yesterday
Frequency / statistic
Research frontier
conf 97%
A source reports “15 individuals” in the context of general: “An additional genetic analysis was reported for 15 individuals with recurrent duplications and duplications larger than 1 Mb: three had single nucleotide variants of uncertain clinical significance, one had a pathogenic 15q11.2 deletion, and the remaining 11 h”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “88.9%” in the context of neurodevelopment: “The great majority of pediatric patients experienced early-onset epilepsy (8/9, 88.9%), with several cases of partial control or uncontrolled epilepsy (5/8, 62.5%).”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “3750 individuals” in the context of kidney: “We have therefore founded the Aetiology of renal and urinary tract anomalies defines Diagnostic Efficacy and Clinical Outcome (ArtDECO) consortium, a large translational research framework within the Netherlands that includes a data- and biobank with clinical,”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “384 cases” in the context of kidney: “To visualize this complexity of assigning genetic causality in CAKUT, Martino et al. calculated incredible estimates of 93,384 cases and 9,244,968 controls needed to identify genome-wide significant associations in a theoretical but feasible scenario that incl”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “21 patients” in the context of neurodevelopment: “However, Ricardo et al. used SPECT to analyze rCBF in areas such as the frontal, temporal, and parietal cortex in 21 patients with POE and 23 patients with epilepsy and found no differences [ 65 ].”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “8256 individuals” in the context of neurodevelopment: “A meta-analysis conducted in 2023, encompassing 8256 individuals diagnosed with schizophrenia and 7532 healthy individuals, revealed significantly reduced levels of glutamate in the medial frontal cortex, elevated levels of glutamine in the...
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “11%” in the context of neurodevelopment: “One study found that 11% of patients in two large epilepsy cohorts had antibodies against autoantigens, such as NMDA receptors, voltage-gated potassium channel complex protein, glycine receptors, and glutamic acid decarboxylase [ 145 ].”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “5.6%” in the context of neurodevelopment: “A systematic review of 58 valid articles showed that patients with epilepsy had a pooled odds ratio for risk of 7.8 for developing psychosis compared with controls, and the pooled prevalence was estimated to be 5.6% (95%CI: 4.8-6.4%).”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “7%” in the context of neurodevelopment: “In addition, the prevalence of psychotic disorders in temporal lobe epilepsy (TLE) is even higher, with an estimated 7% (95% CI: 4.9-9.1%) [ 6 ], whereas the probability of psychotic disorders in extra-TLE is about 4.3% [ 7 ].”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “1434 patients” in the context of neurodevelopment: “One study found that among 1434 patients with epilepsy, the prevalence of IIP was 2.2%, while the prevalence of postictal psychosis was 3.7%.”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “12 patients” in the context of neurodevelopment: “A 1-year follow-up of 12 patients with chronic interictal psychosis combined with intractable epilepsy showed that four patients worsened at 6 months and 1 year after treatment, two patients deteriorated at 6 months and improved at 1 year, three...
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “48.5%” in the context of neurodevelopment: “A review found that FN was mainly caused by the use of ASMs (48.5%), epilepsy surgery (31.8%), or vagus nerve stimulation (VNS) (13.6%).”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “2067 patients” in the context of neurodevelopment: “Naoto Adachi et al. studied 2067 patients with focal epilepsy for every six-month period after administration of a new drug (either ASMs or non-ASMs) between 1981 and 2015.”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “5.8%” in the context of neurodevelopment: “The incidence of psychosis was significantly increased with the use of second-generation ASMs, particularly topiramate (5.8%) and lamotrigine (3.7%).”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “50 patients” in the context of neurodevelopment: “A large cohort of 50 patients with POE and 50 patients with epilepsy examined the volume of the total hippocampus and each subregion and found that the volumes of the posterior hippocampus were reduced bilaterally in POE patients.”
yesterday
Frequency / statistic
Research frontier
conf 97%
A source reports “38%” in the context of general: “BRA and URA are common, reported in 38% and 36% of all cases, and BRA with FRAS1 in particular (48% of FRAS1 cases versus 33% of FREM2 cases) ( 45 ).”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “37%” in the context of kidney: “A study of 29 stillbirth fetuses with renal agenesis identified a heterozygous RET mutation in 7/19 (37%) of those with BRA and in 2/10 (20%) of those with URA ( 31 ), ranking RET among the most frequent genes reported in BRA.”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “10 cases” in the context of kidney: “Sex Renal agenesis has been reported to be more prevalent in males than females ( 8 ), although a more recent autopsy series of 10 cases of BRA yielded a male:female ratio of 1:1 ( 9 ).”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “2022 of 100” in the context of kidney: “When fetal cases are included in future genetic invesigations of CAKUT, BRA will predictably be evaluated more often, as illustrated by a large series published in 2022 of 100 fetuses with severe renal defects was examined by targeted next-generation sequencin”
yesterday
Frequency / statistic
Research frontier
conf 97%
A source reports “1 in 7,700” in the context of general: “BRA reportedly occurs in 1 in 7,700 fetuses to 30,000 live births ( 1 , 2 ) and although often seen in isolation, may be but one feature of a complex genetic syndrome affecting other organ systems.”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “18.2%” in the context of neurodevelopment: “For 113 VUS CNVs, inherited VUS CNVs dramatically reduced anxiety reflected by TOP rates in de novo (18.2%, 2/11), inherited (0/51) and unknown group (2.0%, 1/51).”
yesterday
Frequency / statistic
Research frontier
conf 97%
A source reports “5.2%” in the context of general: “In their study, Lin et al. reported a 5.2% VUS, which decreased to 1.9% (265/14073) after parent-of-origin tests 15 .”
yesterday
Frequency / statistic
Research frontier
conf 97%
A source reports “141 families” in the context of general: “A previous study including 141 families reported that 59.8% of fetal CNVs were maternal origin and 40.2% were paternal origin 14 .”
yesterday
Frequency / statistic
Research frontier
conf 97%
A source reports “37 individuals” in the context of general: “1 , 2 In our cohort of 37 individuals with 17q12DS, a 30-35% and 40-65% had an increased frequency of atopic disorders and recurrent sinopulmonary infections, respectively, raising the possible immune dysregulation.”
yesterday
Frequency / statistic
Research frontier
conf 97%
A source reports “52%” in the context of general: “Also, 52%, 43% and 65% subjects reported recurrent ear infection, sinusitis, and bronchitis, respectively ( Fig.”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “15 cases” in the context of diabetes metabolic: “The underlying ethiology was duodenal atresia in 15 cases (50%), duodenal web in 2 cases (6.6%) and annular pancreas in 13 cases (43.3%).”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “1 in 5000” in the context of diabetes metabolic: “Keywords: duodenal obstruction, annular pancreas, ultrasound, prenatal diagnosis, chromosomal abnormality Abstract Background and Objectives : Congenital duodenal obstruction (CDO) is a very rare anomaly with an incidence of 1 in 5000-10,000 live births.”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “1 in 20,000” in the context of diabetes metabolic: “The incidence of annular pancreas is approximately 1 in 20,000 live births [ 4 ].”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “40 percent” in the context of diabetes metabolic: “But it is crucial to emphasize that more than 40 percent of cases of annular pancreas lead to considerable duodenal obstruction and related complications [ 8 ].”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “13 cases” in the context of diabetes metabolic: “Thirty cases of CDO, in which both the diagnosis and ethiology were established, were categorized into two distinct groups based on their ethiology: CDO with annular pancreas (13 cases) and CDO without annular pancreas (17 cases).”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “46.2%” in the context of diabetes metabolic: “Fetal growth restriction was identified in six cases (46.2%) with annular pancreas and five cases (29.4%) without annular pancreas, with no significant difference between the groups.”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “100%” in the context of diabetes metabolic: “A study reported 100% accuracy in diagnosing annular pancreas prenatally in the case of double bubble and hyperechogenic band surrounding the duodenum ( Figure 2 ) [ 12 ].”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “43%” in the context of diabetes metabolic: “We found a 43% ratio of annular pancreas, which is slightly higher than previous studies.”
yesterday
Frequency / statistic
Research frontier
conf 97%
A source reports “63.9%” in the context of general: “Associated chromosomal abnormalities were reported in the literature over a wide range between 8 and 63.9% [ 9 , 18 , 21 ], mostly with trisomy 21.”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “77%” in the context of diabetes metabolic: “More interestingly in our study, CDO with annular pancreas was significantly more associated (77% vs.”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “11 cases” in the context of diabetes metabolic: “Laparoscopic diagnosis and treatment of neonates with duodenal obstruction associated with an annular pancreas: Report of 11 cases.”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “74 individuals” in the context of diabetes metabolic: “Sixty-nine of the 74 individuals with diabetes and a T1D polygenic score exceeding an externally validated cutoff for distinguishing T1D from not-T1D were confirmed to have T1D.”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “82%” in the context of diabetes metabolic: “The participants with diabetes were predominantly European ancestry (82%) and with T1D (65%).”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “5 individuals” in the context of diabetes metabolic: “Of those 5 individuals with scores above the optimal cutoff without a diagnosis of T1D, there was one individual with CFRD, two individuals with T2D, one individual with SIH, and one individual with diabetes in association with mitochondrial...
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “6.7%” in the context of diabetes metabolic: “Unclear diabetes diagnosis One prepubertal African ancestry individual presented with new-onset "T1D" with a hemoglobin A1c 6.7% ( Figure 3C ).”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “5 cases” in the context of diabetes metabolic: “Unlike other monogenic diabetes, about half of MODY5 cases are associated with a 17q12 deletion spanning approximately 1.5 Mb with variations from case to case encompassing 15 genes on average [ 4 , 5 ], including HNF1B [ 6 ], which explains the...
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “17.2%” in the context of diabetes metabolic: “At the age of 17 years, she was hospitalized due to elevated blood glucose levels with ketosis (blood glucose 29.2 mmol/l, HbA1c 17.2%, 3-hydroxybutyrate 6912 μmol/l).”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “9.9%” in the context of kidney: “In addition, she had hypomagnesemia (1.4 mg/dl) with inappropriately increased urinary excretion of magnesium (FEMg 9.9%).”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “6.9%” in the context of diabetes metabolic: “Since discharge, her blood glucose levels were well controlled (HbA1c ~ 6.9%) with a relatively small amount of insulin (total daily dose ~ 11 U/day) for a long time.”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “5 patients” in the context of diabetes metabolic: “However, MODY5 patients especially with microdeletion tend to be leaner [ 1 ], which may be responsible for her insulin sensitivity.”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “45 cases” in the context of kidney: “Bilateral renal agenesis/hypoplasia/dysplasia (BRAHD): postmortem analysis of 45 cases with breakpoint mapping of two de novo translocations.”
yesterday
Frequency / statistic
kidney and urinary tract
conf 97%
A source reports “127 families” in the context of kidney: “Establishing an algorithm for molecular genetic diagnostics in 127 families with juvenile nephronophthisis.”
yesterday
Frequency / statistic
Signs & symptoms
conf 97%
A source reports “45.2%” in the context of growth nutrition: “Methods We examined prevalence rates of obesity and MetS in 103 adults with a typical 22q11.2 deletion (45.2% male, at median age 30.0 (range 17-71) years) and compared these rates with population-based data.”
yesterday
Frequency / statistic
Signs & symptoms
conf 97%
A source reports “32.0%” in the context of growth nutrition: “Results Prevalence rates of generalized obesity (32.0%), abdominal obesity (51.5%), and MetS (33.0%) were significantly higher compared to a population-based cohort (15.7% ( P < 0.0001), 36.1% ( P = 0.002), and 15.2% ( P < 0.0001), respectively).”
yesterday
Frequency / statistic
Signs & symptoms
conf 97%
A source reports “43.5%” in the context of growth nutrition: “Two previous single-center cohort studies in adults with 22q11.2DS reported high prevalence rates of generalized obesity (43.5%; 90/202 Canadians and 38.5%; 20/52 Belgian, respectively), based on body mass index (BMI) [ 7 , 8 ], the most widely used and a quic”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “35.9%” in the context of neurodevelopment: “Participants were referred from three main sources: family medicine ( n = 38, 35.9%), intellectual disability medicine ( n = 34, 33.0%), and pediatrics ( n = 16, 15.5%).”
yesterday
Frequency / statistic
learning, speech and behaviour
conf 97%
A source reports “61.2%” in the context of neurodevelopment: “The most common reasons for referral were periodic 22q11.2DS-related assessments and health monitoring ( n = 63, 61.2%; including transition to adult care), and psychiatric symptoms and/or psychosocial problems ( n = 23, 22.3%).”
yesterday
Frequency / statistic
Signs & symptoms
conf 97%
A source reports “60%” in the context of growth nutrition: “Comparison with prevalence rates of obesity and metabolic syndrome in the general population To compare prevalence rates of obesity and MetS in the 22q11.2DS cohort with those in the general population, we used published data from Lifelines, a large Dutch popu”
yesterday
Frequency / statistic
Signs & symptoms
conf 97%
A source reports “38.5%” in the context of growth nutrition: “Obesity prevalence among Belgians, who are geographically and genetically closely related to the Dutch, was 38.5% (at a median age of 30 years).”
yesterday
Frequency / statistic
Signs & symptoms
conf 97%
A source reports “89%” in the context of hepatic: “In contrast, the primary rick factor for CCA in Northeast Thailand particularly in Khon Kaen is liver fluke infection ( Opisthorchis Viverrini ), which accounts for approximately 89% of all liver cancers in this region [ 5 ].”
yesterday
Frequency / statistic
Research frontier
conf 97%
A source reports “84%” in the context of general: “They reported the sensitivity of Arginase-1 and HepPar-1 to be 84% and 70%, respectively, while their specificity was 96% and 84%, respectively.”
yesterday
Contradicting evidence
kidney and urinary tract
conf 97%
A source reports no association or a negative finding: “If kidney cysts are not associated with disturbances in glucose metabolism, the term HNF1B -associated kidney disease is used.”
yesterday
Contradicting evidence
Research frontier
conf 97%
A source reports no association or a negative finding: “Overall, 17q12-related HNF1B whole gene deletions were the predominant type in our HNF1B pathogenic variant spectrum, with no significant difference in the proportion of inherited versus de novo pathogenic variants ( p = 0.198).”
yesterday
Contradicting evidence
kidney and urinary tract
conf 97%
A source reports no association or a negative finding: “Because EA has already been identified in patients with 17q12 duplication or HNF1B point mutation, we screened HNF1B (QMPSF and direct sequencing) in nine additional patients with EA and renal abnormalities but failed to identify any pathogenic variant.”
yesterday
Contradicting evidence
kidney and urinary tract
conf 97%
A source reports no association or a negative finding: “In our case, there was no evidence of hepatic enlargement or fibrosis on fetal imaging, and the family history (maternal renal cyst and paternal diabetes) was more suggestive of an HNF1B -related disorder.”
yesterday
Clinical trial
Research frontier
conf 85%
Screening for Genetic Forms of Diabetes in Convention of Care for Children and Adolescents With Diabetes (GENEPEDIAB) — COMPLETED. Sponsor: Université Catholique de Louvain.
yesterday
Clinical trial
Research frontier
conf 85%
Identifying Maturity-onset Diabetes of the Young in Emirati Patients — UNKNOWN. Sponsor: United Arab Emirates University.
yesterday
Clinical trial
Research frontier
conf 85%
Ovarian Function and Gynecological Profile of Patients Carrying a Pathogenic Variant of the HNF1B Gene (GYN-HNF1) — COMPLETED. Sponsor: University Hospital, Toulouse.
yesterday
Clinical trial
Research frontier
conf 85%
Switching From Insulin to Sulfonylurea in Diabetes Associated With Variants in MODY Genes — UNKNOWN. Sponsor: Haukeland University Hospital.
yesterday
Clinical trial
Research frontier
conf 85%
Online Study of People Who Have Genetic Changes and Features of Autism: Simons Searchlight — RECRUITING. Sponsor: Simons Searchlight.
yesterday
New publication
Genes & inheritance
conf 97%
TBC1D3 is a primate-specific gene family that has expanded in the human lineage and has been implicated in neuronal progenitor proliferation and expansion of the frontal cortex.
yesterday
New publication
Genes & inheritance
conf 97%
Author Contributions All authors contributed to patient phenotyping data and sample collection or to analyses.
yesterday
New publication
Genes & inheritance
conf 97%
Objectives The aim of the study was to evaluate the detection rate of genetic abnormalities in cases of foetal gallbladder (FGB) size abnormalities to determine whether these abnormalities justify prenatal diagnosis.
yesterday
New publication
learning, speech and behaviour
conf 97%
of WES info and statistics, [ 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 ]. Author Contributions L.M. and A.F. designed; L.
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New publication
Genes & inheritance
conf 97%
Background Chromosome 3q29 duplication syndrome is a rare chromosomal disorder with a frequency of 1:5000 in patients with a neurodevelopmental phenotype.
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New publication
kidney and urinary tract
conf 97%
A higher resolution version of the Graphical abstract is available as Supplementary information Supplementary Information The online version contains supplementary material available at 10.
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New publication
Genes & inheritance
conf 97%
Epilepsy is a prevalent chronic neurological disorder that can significantly impact patients' lives.
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New publication
Genes & inheritance
conf 97%
of the genetic etiologies known to be associated with this condition. Methods: A literature search was conducted as a scoping review employing OMIM, PubMed, and Cochrane to identify cases of BRA with known underlying genetic (chromosomal versus single gene) etiologies and those described in syndromes without any...
yesterday
New publication
Genes & inheritance
conf 97%
Background To determine the pathogenicity of a novel splicing variant in the SMARCC2 gene identified from a pair of adult male monozygotic twins with neurodevelopmental disorder, and to investigate the genotype-phenotype characteristics associated with SMARCC2 variants.
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New publication
kidney and urinary tract
conf 97%
A young man presented unconscious with severe hyponatraemia, hypokalaemia, hypomagnesaemia and metabolic alkalosis.
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New publication
Genes & inheritance
conf 97%
Low-penetrance pathogenic copy number variations (CNVs), variation of uncertain significance (VUS) CNVs and likely pathogenic CNVs present a challenge for prenatal diagnosis.
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New publication
Research frontier
conf 97%
Background The 17q12 deletion syndrome (17q12DS) is a heterozygous deletion of a 1.4 megabase‒spanning DNA sequence on chromosome 17.
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New publication
Genes & inheritance
conf 97%
statistics [ 45 ]. For instance, some methods clarify that summary statistics a few standard deviations above a genome-wide mean do not have p values [ 22 , 31 ], and equally so, no adjustment for multiple testing.
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New publication
kidney and urinary tract
conf 97%
Background and Objectives : Congenital duodenal obstruction (CDO) is a very rare anomaly with an incidence of 1 in 5000-10,000 live births.
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New publication
kidney and urinary tract
conf 97%
Background. Accurately classifying pediatric diabetes can be challenging for providers, and misclassification can result in suboptimal care.
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New publication
kidney and urinary tract
conf 97%
Maturity-onset diabetes of the young type 5 (MODY5), causally associated with loss-of-function of the HNF1B gene, is a rare form of monogenic diabetes that has been underdiagnosed in part because microdeletions of chromosome 17q12 encompassing the HNF1B gene cannot be detected by sequencing-based approaches, which...
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New publication
Genes & inheritance
conf 97%
Objective Copy number variations (CNVs) may contribute to medical conditions. However, research on the impact of individual CNVs on endocrine disease is limited.
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New publication
Genes & inheritance
conf 97%
Technological advances such as next-generation sequencing (NGS) have enabled high-throughput assessment of the human genome, supporting the usage of genetic testing as a first-line tool across clinical medicine.
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New publication
Genes & inheritance
conf 97%
Neonatal cholestasis can be caused by several conditions, with biliary atresia being the major cause. Genetic and endocrinological etiologies represent other possibilities, with most of them requiring a rapid diagnosis and a specific treatment.
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New publication
How it is diagnosed
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Hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) are primary liver cancers with overlapping histopathological features, making accurate diagnosis challenging.
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New publication
How it is diagnosed
conf 97%
Objective To discuss the application value of technologies such as chromosome microarray analysis (CMA) in fetuses with hydronephrosis and pyelectasis.
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New publication
Genes & inheritance
conf 97%
Congenital heart disease (CHD) is the most common birth defect, affecting 2-8% of newborns, with a marked impact on neonatal health.
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New publication
Genes & inheritance
conf 97%
Autism spectrum disorder (ASD) is a neurodevelopmental disorder that first develops in early childhood and is characterized by restricted interests, activities, and behaviors, as well as difficulties with social interactions and communication.
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New publication
Genes & inheritance
conf 97%
To evaluate the clinical utility of genetic testing via karyotyping, chromosomal microarray analysis (CMA), and exome sequencing in cases with corpus callosum abnormalities (CCA).
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