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Journal of Perinatology : Official... Oct 2023This study describes 3-year follow-up of 200 infants weighing ≥ 700 grams who underwent transcatheter patent ductus arteriosus (PDA) closure with the Amplatzer...
OBJECTIVE
This study describes 3-year follow-up of 200 infants weighing ≥ 700 grams who underwent transcatheter patent ductus arteriosus (PDA) closure with the Amplatzer Piccolo™ Occluder.
STUDY DESIGN
Between June 2017 and February 2019, 200 children were enrolled in this U.S. study (NCT03055858). PDA closure, survival, and device- or procedure-related events were evaluated. A total of 156 of the available 182 patients (86%) completed the study.
RESULTS
The implant success rate was 95.5% (191/200). At 3 years, PDA closure was observed in 100% (33/33) of patients. Survival was >95% with 9 reported deaths. No deaths were adjudicated as device- or procedure-related. Notable events included aortic obstruction (2) requiring stent placement and tricuspid regurgitation (5), for which no interventions were required.
CONCLUSIONS
This follow-up study demonstrates high rates of PDA closure, low serious complication rates, and survival > 95% at 3 years. The Amplatzer Piccolo™ Occluder is a safe and effective therapy for PDA treatment in premature infants.
CLINICALTRIALS
gov identifier: NCT0305585.
Topics: Infant; Humans; Child; Follow-Up Studies; Ductus Arteriosus, Patent; Prospective Studies; Cardiac Catheterization; Septal Occluder Device; Treatment Outcome
PubMed: 37587183
DOI: 10.1038/s41372-023-01741-1 -
Animals : An Open Access Journal From... Jul 2023Few studies have established the normal radiographic anatomical development of the donkey foal and, to date, no data are available for mules. Our aim was thus to...
Few studies have established the normal radiographic anatomical development of the donkey foal and, to date, no data are available for mules. Our aim was thus to evaluate the radiographic development of the fore digit and carpal joint in the mule foal from 0 to 3 months of age. Ten forelimbs of five healthy full-term mule foals were included. Radiographs of the fore digit lateromedial and dorsopalmar and the carpus dorsopalmar were performed weekly for the first month of age, and bi-monthly during the following two months. Fore digit growth plate closure times, morphological, angular, and linear radiographic parameters, and also carpal cuboidal bone mineralization were evaluated. Growth plates were graded as open, closing, and closed. Carpal bone appearance was graded as mature, slightly immature, or immature. Growth plate closure times showed the following: middle phalangeal distal physis (DP) closed at birth, and proximal physis (PP) started to close at around two months; first phalanx DP closed at seven days, and PP started to close at three months; and third metacarpal bone DP started to close at two months. Carpal bones were immature at birth. Distal phalanx (PD) was triangular at birth, palmar processes had developed by one month, and proximal sesamoid bones were trapezoidal by three months. The hoof wall, PD dorsal wall, and palmar angles values changed in parallel, and hoof capsule thickness increased. No PD remodeling evidence within the hoof capsule or variations in the hoof axis with growth were found. We identified a baseline for the interpretation of forelimb radiological features in mule foals. The specific features found were intermediate between those found in horses and donkeys.
PubMed: 37570226
DOI: 10.3390/ani13152417 -
Diagnostics (Basel, Switzerland) Aug 2023Patent ductus arteriosus (PDA) is frequent in preterm newborns, and its incidence is inversely associated with the degree of prematurity. The first choice of...
Association of the rs7557402 Polymorphism with Hemodynamically Significant Patent Ductus Arteriosus Closure Failure in Premature Newborns under Pharmacological Treatment with Ibuprofen.
Patent ductus arteriosus (PDA) is frequent in preterm newborns, and its incidence is inversely associated with the degree of prematurity. The first choice of pharmacological treatment is ibuprofen. Several genes, including , have been proposed as probable markers associated with a genetic predisposition for the development of PDA in preterm infants. 1 NG_016000.1:g.84131C>G or rs7557402 has been reported to be probably benign and associated with familial erythrocytosis by the Illumina Clinical Services Laboratory. Other variants of have been previously reported to be benign for familial erythrocytosis because they decrease gene function and are positive for familial erythrocytosis because the overexpression of is a key factor in uncontrolled erythrocyte proliferation. However, this could be inconvenient for ductal closure, since for this process to occur, cell proliferation, migration, and differentiation should take place, and a decrease in gene activity would negatively affect these processes. Single-nucleotide polymorphisms (SNPs) in and genes were searched with high-resolution melting and Sanger sequencing in blood samples of preterm infants with hemodynamically significant PDA treated with ibuprofen at the National Institute of Perinatology. The variant rs7557402, present in the gene eighth intron, was associated with a decreased response to treatment ( = 0.007, OR = 3.53). The SNP rs7557402 was associated with an increased risk of pharmacological treatment failure. A probable mechanism involved could be the decreased activity of the product of the gene.
PubMed: 37568921
DOI: 10.3390/diagnostics13152558 -
Turkish Archives of Pediatrics Sep 2023The timing of surgical patent ductus arteriosus ligation in preterm infants remains controversial. Early ligation (<2 or 3 weeks of life) benefits preterm infants with a...
OBJECTIVE
The timing of surgical patent ductus arteriosus ligation in preterm infants remains controversial. Early ligation (<2 or 3 weeks of life) benefits preterm infants with a lower incidence of bronchopulmonary dysplasia and necrotizing enterocolitis. We present patent ductus arteriosus ligation experience in premature infants who had been transported for closure to an outside centre other than the hospital they were hospitalized.
MATERIALS AND METHODS
We retrospectively evaluated 17 consecutive patients who had undergone surgery of premature infant patent ductus arteriosus closure during the period of March 2009-December 2020. Patent ductus arteriosus had been clipped in 17 patients.
RESULTS
The median birth weight and age were 930 g and 28 gestation weeks, respectively. The birth age of the sub-groups were A: ≤28 weeks and B: >28 weeks and birthweight were group I: ≤800 g and group II: >800 g. The median day of PDA ligation wasa 20 days, and patients with birthweight ≤800 g were ligated later than patients weighing >800 g. Two patients had intracranial hemorrhage, 6 had bronchopulmonary dysplasia, and 2 were dead. We found that exposure to large patent ductus arteriosus and low birth age in preterm babies was associated with longer hospitalization duration, preoperative mechanical ventilation time, and sepsis.
CONCLUSION
Infants exposed to moderate-to-large patent ductus arteriosus requiring intubation and resistant to medical therapy for more than 2 weeks should have surgical ligation as soon as possible.
PubMed: 37563951
DOI: 10.5152/TurkArchPediatr.2023.23066 -
Clinical Oral Investigations Sep 2023The aim was to provide epidemiological and clinical data on patients with orofacial clefts in Lower Saxony in Germany.
OBJECTIVE
The aim was to provide epidemiological and clinical data on patients with orofacial clefts in Lower Saxony in Germany.
MATERIALS AND METHODS
The records of 404 patients with orofacial clefts treated surgically at the University Medical Center Goettingen from 2001 to 2019 were analyzed in this retrospective study. Prevalence of orofacial clefts in general, orofacial clefts as manifestation of a syndrome, sex distribution, and prevalence of different cleft types was evaluated and associated with the need for corrective surgery, family history, pregnancy complications, and comorbidities.
RESULTS
The prevalence of orofacial clefts for Goettingen in Lower Saxony was 1:890. 231 patients were male and 173 were female. CLP was most common (39.1%) followed by CP (34.7%), CL (14.4%), CLA (9.9%), and facial clefts (2%). The left side was more frequently affected and unilateral cleft forms occurred more often than bilateral ones. Almost 10% of the population displayed syndromic CL/P. 10.9% of all patients had a positive family history regarding CL/P, predominantly from the maternal side. Pregnancy abnormalities were found in 11.4%, most often in the form of preterm birth. Comorbidities, especially of the cardiovascular system, were found in 30.2% of the sample. 2.2% of patients treated according to the University Medical Center Goettingen protocol corrective surgery was performed in form of a velopharyngoplasty or residual hole closure.
CONCLUSIONS
The epidemiological and clinical profile of the study population resembled the expected distributions in Western populations. The large number of syndromic CL/P and associated comorbidities supports the need for specialized cleft centers and interdisciplinary cleft care.
Topics: Pregnancy; Humans; Male; Infant, Newborn; Female; Cleft Lip; Cleft Palate; Retrospective Studies; Premature Birth; Sex Distribution
PubMed: 37542681
DOI: 10.1007/s00784-023-05187-9 -
Journal of the Canadian Academy of... Aug 2023Appropriate interventions for psychiatric conditions that commonly emerge during adolescence and early adulthood play a crucial role in modifying both acute risks as...
Appropriate interventions for psychiatric conditions that commonly emerge during adolescence and early adulthood play a crucial role in modifying both acute risks as well as long-term outcomes. Substance use disorder is a common comorbidity during the early stages of mood and psychotic disorders that further heightens acute risks and is considered a negative prognostic factor. New presentations of mood and psychotic symptoms with co-occurring substance use are inherently challenging to formulate due to the uncertainty surrounding the relative impact of multiple intrinsic and extrinsic factors. Given such uncertainty, it is natural for clinicians to rely on heuristics to guide assessment and management. These heuristics however may bring about premature diagnostic closure by favouring the primacy of substance use, which in turn can result in a missed window of opportunity for a timely and appropriate intervention. We caution clinicians against over-attributing early symptoms of mood and psychotic disorders to substances use alone.
PubMed: 37534116
DOI: No ID Found -
Children (Basel, Switzerland) Jul 2023Omitting the early closure of the cranial sutures in newly born children is not an uncommon practice. We describe the natural history of several unrelated children and...
BACKGROUND
Omitting the early closure of the cranial sutures in newly born children is not an uncommon practice. We describe the natural history of several unrelated children and adults from two unrelated families. These children were born with variable clinical manifestations: craniofacial asymmetry, ocular proptosis, floppiness, and progressive deceleration in cognitive development. None of these children underwent a cranial sutures assessment. False diagnoses of positional plagiocephaly, neonatal thyrotoxicosis, congenital muscular atrophy, and hydrocephalus were given to the parents. This sort of malpractice was the reason behind a sequence of devastating pathological events that occurred in the lifetime of these children and adults.
MATERIAL AND METHODS
This was a multigenerational study of two unrelated families. In total, we studied three children (aged 7-19 years) and three adults (aged 40-52 years) from two families. The children from the first family were referred to our departments because of pre-pubertal scoliosis, kyphoscoliosis, and early-onset osteoarthritis. Reading the clinical histories of these children signified apparent clinical misconceptions. For instance, craniofacial asymmetry was misinterpreted as positional plagiocephaly and treated by means of helmet molding therapy. Ocular proptosis was given the false diagnosis of neonatal thyrotoxicosis. Floppiness (hypotonia) was misdiagnosed as congenital muscular dystrophy. The index case from the second family showed progressive deceleration in his cognitive development, associated with signs of increased intracranial pressure. The only diagnosis was Dandy-Walker malformation. We documented every patient in accordance with the clinical and radiological phenotypic characterizations. The genotype characterization followed accordingly.
RESULTS
All patients in family (I) manifested a phenotype consistent to a certain extent with the clinical phenotype of Shprintzen-Goldberg syndrome (SGS), though the intensity of spine deformities was greater than has been described in the literature. The second family showed a constellation of Marfanoid habitus, craniosynostosis, increased intracranial pressure, hydrocephalus, Dandy-Walker malformation, seizures, and intellectual disability. The overall clinical phenotype was consistent but not fully diagnostic of craniosynostosis-Dandy-Walker-malformation hydrocephalus syndrome. The early closure of the sutures was totally different from one patient to another, including the premature closure of the metopic, coronal, squamosal, and sagittal sutures. One patient from family (II) underwent the implementation of a shunt system at the age of 3 years, unfortunately passing over the pre-existing craniosynostosis. In addition to skeletal deformities, a history of seizures and severe intellectual disability was recorded. The proband underwent chromosomal karyotyping, the FISH test, and whole-exome sequencing.
CONCLUSION
The purpose of this study was fivefold. Firstly, to gain a meticulous understanding in order to differentiate between positional plagiocephaly, hypotonia, and congenital exophthalmos and their connections to abnormal craniofacial contours was and still is our first and foremost concern. Secondly, we aimed to characterize craniosynostosis, seizures, intellectual disabilities, and hydrocephalus associated with Marfanoid habitus, which were clearly demonstrated in our patients. Thirdly, we aimed to address the imperative for interpretations of clinical and radiological phenotypes and relate these tools to etiological understanding, which is an essential basis for diagnosis in the majority of long-term pediatric admissions. Fourthly, we aimed to assess the impacts of the missed early closure by the pediatricians and pediatric neurologists, which added a heavy pathological burden on these patients and their families. Fifthly, we aimed to identify whether early and diligent recognition can assist in cranial vault remodeling via surgical intervention to halt premature cranial suture fusions and can possibly alter the devastating course and the complications of the synostosed sutures.
PubMed: 37508737
DOI: 10.3390/children10071240 -
Orphanet Journal of Rare Diseases Jul 2023Foramen magnum stenosis is a serious, and potentially life-threatening complication of achondroplasia. The foramen magnum is smaller in infants with achondroplasia,...
Foramen magnum stenosis is a serious, and potentially life-threatening complication of achondroplasia. The foramen magnum is smaller in infants with achondroplasia, compared with the general population, and both restricted growth in the first 2 years and premature closure of skull plate synchondroses can contribute to narrowing. Narrowing of the foramen magnum can lead to compression of the brainstem and spinal cord, and result in sleep apnoea and sudden death. There is a lack of clarity in the literature on the timing of regular monitoring for foramen magnum stenosis, which assessments should be carried out and when regular screening should be ceased. The European Achondroplasia Forum (EAF) is a group of clinicians and patient advocates, representative of the achondroplasia community. Members of the EAF Steering Committee were invited to submit suggestions for guiding principles for the detection and management of foramen magnum stenosis, which were collated and discussed at an open workshop. Each principle was scrutinised for content and wording, and anonymous voting held to pass the principle and vote on the level of agreement. A total of six guiding principles were developed which incorporate routine clinical monitoring of infants and young children, timing of routine MRI screening, referral of suspected foramen magnum stenosis to a neurosurgeon, the combination of assessments to inform the decision to decompress the foramen magnum, joint decision making to proceed with decompression, and management of older children in whom previously undetected foramen magnum stenosis is identified. All principles achieved the ≥ 75% majority needed to pass (range 89-100%), with high levels of agreement (range 7.6-8.9). By developing guiding principles for the detection and management of foramen magnum stenosis, the EAF aim to enable infants and young children to receive optimal monitoring for this potentially life-threatening complication.
Topics: Child; Infant; Humans; Adolescent; Child, Preschool; Foramen Magnum; Constriction, Pathologic; Achondroplasia; Sleep Apnea Syndromes; Spinal Cord; Bone Diseases
PubMed: 37501185
DOI: 10.1186/s13023-023-02795-2 -
Paediatrics & Child Health Aug 2023Acetaminophen has gained interest in the neonatal community for its use in the management of hemodynamically significant patent ductus arteriosus (HsPDA) in preterm...
OBJECTIVES
Acetaminophen has gained interest in the neonatal community for its use in the management of hemodynamically significant patent ductus arteriosus (HsPDA) in preterm infants. We conducted a systematic review of randomized controlled trials (RCTs) comparing the efficacy and safety of acetaminophen with indomethacin for the management of HsPDA in preterm infants.
METHODS
We searched PROSPERO, OVID Medline, OVID EMBASE, Wiley Cochrane Library (CDSR and Central), EBSCO CINAHL, and SCOPUS from inception to June 15, 2021. Bibliographies of identified studies were searched for additional references. Data were analyzed with Review Manager (RevMan) Version 5.3.
RESULTS
Four RCTs were identified, enrolling a total of 380 subjects. There was no difference between the interventions for the outcome of PDA closure after one course (RR 1.04 [95% CIs: 0.84, 1.29], -value 0.70) or after two courses of treatment (RR 1.01 [95% CIs: 0.92, 1.12], -value 0.77); and for the outcome of PDA ligation (RR 1.56 [95% CIs: 0.48, 5.12], -value 0.46). However, patients who received acetaminophen had lower rates of necrotizing enterocolitis (RR 0.37 [95% CIs: 0.14, 0.95], -value 0.04). There were no significant differences noted in the other clinical outcomes, that is, intraventricular hemorrhage, bronchopulmonary dysplasia, retinopathy of prematurity requiring treatment, and death. Two studies noted significant post-treatment elevation of serum creatinine and blood urea with indomethacin, as compared to none with acetaminophen use.
CONCLUSIONS
Acetaminophen has comparable efficacy to indomethacin for the outcome of HsPDA closure, with a better safety profile, that is, lesser rates of necrotizing enterocolitis and post-treatment azotemia noted with its use.
PubMed: 37484043
DOI: 10.1093/pch/pxac130