Reabilitação de dente com hipoplasia de turner: relato de caso / Rehabilitation of tooth with Turner's hypoplasia: Case report

Os defeitos de esmalte são alterações qualitativas ou quantitativas na estrutura dentária, que originam-se de fatores sistêmicos, locais ou genéticos. A hipoplasia de Turner é um defeito na espessura do esmalte localizado cuja etiologia decorre de um traumatismo ou infecção periapical presente no dente decíduo predecessor, afetando o desenvolvimento do dente permanente. O objetivo do presente estudo foi apresentar um caso clínico de paciente infantil com dente hipoplásico de Turner em pré-molar, que tornou-se não vital sem que houvesse lesão de cárie ou trauma adicional. Em razão das características clínicas e radiográficas do dente afetado, bem como do risco de cárie e do comportamento cooperador da paciente, optou-se pela reabilitação do elemento afetado por meio de tratamento endodôntico e de restauração semidireta em resina composta. Torna-se de fundamental importância o conhecimento da etiologia e a realização de um exame clínico e radiográfico minucioso visando ao diagnóstico precoce e à elaboração de um plano de tratamento adequado para todos os defeitos de desenvolvimento do esmalte, incluindo-se a hipoplasia de Turner, cujo tratamento dependerá da severidade da alteração, do comportamento do paciente e do risco de cárie. Sugere-se a realização de estudos que associem a microestrutura do esmalte hipoplásico com a ausência de vitalidade pulpar.

Enamel defects are qualitative or quantitative changes in the tooth structure originating from systemic, local, or genetic factors. Turner's hypoplasia is a defect in the thickness of the localized enamel whose etiology arises from trauma or periapical infection in the predecessor deciduous tooth, affecting the permanent tooth's development. The objective of the present study was to present a clinical case of a child patient with a hypoplastic Turner premolar tooth, which became non-vital without the occurrence of caries, or additional trauma. Due to the affected tooth's clinical and radiographic characteristics, the risk of cavities, and the patient's cooperative behavior, it was decided to rehabilitate the affected element through endodontic treatment and semidirect restoration in composite resin. It is of fundamental importance to know the etiology and carry out a thorough clinical and radiographic examination aiming at early diagnosis and the development of an adequate treatment plan for all enamel developmental defects, including Turner's hypoplasia, whose treatment will depend on the severity of the change, the patient's behavior and the risk of caries. Studies are suggested to be carried out that associate the microstructure of hypoplastic enamel with the absence of pulp vitality.

Clinical, laboratory and neuroimaging profile of patient's cohort with septo-optic dysplasia treated at a pediatric university hospital.

OBJECTIVES: Septo-optic dysplasia (SOD) is a relatively rare clinical condition. However, there has been a significant increase in its incidence over the years. Diagnosis is clinical and made when there are at least 2 components of the classic triad: Optic nerve hypoplasia (ONH), midline malformation, and pituitary dysfunction. This study aims to describe the clinical and complementary exam characteristics of patients with SOD. METHODS: A retrospective study of review of medical records of 48 patients cohort (24 female) with SOD followed to 2023. RESULTS: The average age at diagnosis was 3.90 ±â€¯3.85 years. Maternal age was ≤ 25 years at the time of delivery in 50% (24/48) of cases. Visual and developmental impairment was observed in 21 (43,7%) and nystagmus in 15 patients. Fourteen of them developed short stature. Regarding the diagnostic criteria for SOD: 92.6% (38/41) had ONH (78.9% bilaterally), 95.3% (41/43) had structural midline abnormalities, 85.7% (24/28) had hypothalamic-pituitary region alterations, and 73% had at least one hormonal deficiency, of which 2/3 had multiple pituitary dysfunctions. The most frequent deficiencies were thyroid-stimulating hormone and growth hormone, and the average age at diagnosis of the first dysfunction was 4.25 ±â€¯3.71 years. CONCLUSION: Clinical manifestations that most led to early suspicion were developmental delay, nystagmus and visual impairment. More than 1/3 of the patients had the complete triad and 2/3 developed multiple pituitary deficiencies, with TSH deficiency being the most prevalent followed by GH deficiency. Patients with ONH or midline structural changes should undergo endocrine evaluation.

Premaxillary Graft with Costal Cartilage in Rhinoplasty: A Technique to Support the Nasal Tip.

BACKGROUND: Rhinoplasty is an ever-evolving field, with innovative techniques continually being developed to enhance both aesthetic and functional outcomes for patients. Increasingly, research has focused on the integral role of the facial skeleton in providing nasal support and projection. Central to the structural integrity of the nose is the maxillary bone, which occupies a pivotal position in the midface. METHODS: The objective of this study is to assess the outcomes of patients who underwent rhinoplasty involving the placement of a premaxillary graft fashioned from costal cartilage. The study aims to evaluate the graft's tolerance, stability, and potential complications. The patient cohort comprised individuals who underwent open approach rhinoplasty with premaxillary insufficiency, necessitating the placement of a costal cartilage graft anterior to the nasal spine, performed by the same surgeon between 2021 and 2022. A total of 38 patients, consisting of 5 men and 33 women aged between 18 and 58 years, were operated on during this period. RESULTS: Consistent maintenance of tip support was observed across all cases. Among the 33 patients, 20 were randomly chosen for a comparative assessment of the nasolabial angle in preoperative and postoperative profile photographs, demonstrating a statistically significant improvement. No complications such as graft displacement, scarring, extrusion, or infections were reported. CONCLUSION: The use of a premaxillary graft with costal cartilage appears to be a viable, well-tolerated option with favorable long-term outcomes. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Effect of the Basilar Invagination (Type B) on Cervical Spine: A Case-control Study with MRI.

OBJECTIVE: To evaluate the effect of the basilar invagination (BI) type B on cervical spine. METHODS: The research protocol used head magnetic resonance imaging (MRI) exams from 41 participants with BI type B and 158 controls. The criterion for BI was the distance of the odontoid apex to Chamberlain's line (DOCL) equal to or greater than 7 mm. The clivus length (CLI), clivus canal angle (CCA), Welcker's basal angle (WBA), Boogaard's angle (BOA), upper cervical lordosis angle (UCL), and total cervical lordosis angle (CL) were evaluated. The descriptive analysis, group comparisons, and correlations between skull base and cervical spine parameters were performed at the 95% CI. RESULTS: Participants with BI type B showed shorter clivus length (CLI: 25.7 ± 7.3 mm); greater angulation of the skull base (WBA: 126.5 ± 10.4); greater inclination foramen magnum (BOA: 151.5 ± 14.5); decrease in the value of the CCA (131.6 ± 15); and greater angulations of UCL (17.9 ± 13.8) and CL (29.7 ± 19.9) in comparison to the control group (P < 0.05). Clivus length and CCA correlated inversely with UCL and CL, while BOA correlated directly with UCL and CL. The WBA did not correlate with CL (P < 0.05). CONCLUSIONS: The deformation of skull base in the BI of type B caused, on average, a hyperlordosis of almost 30° in the C2-C6 segment. This change was approximately 17° in the C2-C4, with the clivus hypoplasia being a risk factor for cervical hyperlordosis.

Focal dermal hypoplasia: The conflicting characteristics of prenatal and long-term follow-up images of skin anomalies.

To the best of our knowledge, this case presents the first prenatal magnetic resonance imaging diagnosis of focal dermal hypoplasia with long-term follow-up, with important discordance between the prenatal and postnatal imaging characteristics of the skin malformation.

Fetoscopic Tracheal Occlusion for Isolated Severe Left Diaphragmatic Hernia: A Systematic Review and Meta-Analysis.

Background: We aimed to conduct a systematic review and meta-analysis to evaluate the fetoscopic tracheal occlusion in patients with isolated severe and left-sided diaphragmatic hernia. Methods: Cochrane Library, Embase, and PubMed (Medline) databases were searched from inception to February 2024 with no filters or language restrictions. We included studies evaluating the outcomes of fetoscopic intervention compared to expectant management among patients with severe congenital diaphragmatic hernia exclusively on the left side. A random-effects pairwise meta-analysis was performed using RStudio version 4.3.1. Results: In this study, we included 540 patients from three randomized trials and five cohorts. We found an increased likelihood of neonatal survival associated with fetoscopic tracheal occlusion (Odds Ratio, 5.07; 95% Confidence Intervals, 1.91 to 13.44; p < 0.01) across general and subgroup analyses. Nevertheless, there were higher rates of preterm birth (OR, 5.62; 95% CI, 3.47-9.11; p < 0.01) and preterm premature rupture of membranes (OR, 7.13; 95% CI, 3.76-13.54; p < 0.01) in fetal endoscopic tracheal occlusion group compared to the expectant management. Conclusions: Our systematic review and meta-analysis demonstrated the benefit of fetoscopic tracheal occlusion in improving neonatal and six-month postnatal survival in fetuses with severe left-sided CDH. Further studies are still necessary to evaluate the efficacy of tracheal occlusion for isolated right-sided CDH, as well as the optimal timing to perform the intervention.

Diagnostic approach and treatment of ventricular septal defect associated with PDA, coarctation of aorta, hypoplastic aortic arch and multiple valvular heart disease in a tertiary center: An infrequent association.

Ventricular septal defect is the most common congenital heart disease in children and is associated with patent ductus arteriosus in 1%-7% of cases. The coexistence of both malformities with hypoplastic aortic arch and aortic coarctation is even rarer. We present the case of a 6-year-old girl referred to our hospital because of dyspnea on feeding, recurrent respiratory infections, poor weight gain, and a heart murmur. The image studies revealed a ventricular septal defect, patent ductus arteriosus, severe hypoplasia of the aortic arch with critical stenosis of the proximal portion, severe dilatation of the pulmonary artery and pulmonary, mitral, tricuspid, and aortic regurgitation. We will discuss the diagnostic approach and treatment in a tertiary reference center for patients with cardiovascular diseases.

Anatomy of vertebral artery hypoplasia and its relationship with clinical implications: a systematic review and meta-analysis of prevalence.

PURPOSE: The vertebral artery (VA) is a vital branch of the subclavian artery, coursing through the transverse foramina of the cervical vertebrae, and playing a crucial role in irrigating the posterior region of the arterial cerebral circle, also known as the Polygon of Willis. Among the various possible alterations that can affect the VA, vertebral artery hypoplasia (HAV) emerges as a significant variant. This study aims to discern the anatomical features of HAV and its correlation with the clinical conditions of the posterior cerebral circulation. METHODS: The databases Medline, Scopus, Web of Science, Google Scholar, CINAHL, and LILACS were searched until January 2024. Two authors independently performed the search, study selection, and data extraction. Methodological quality was evaluated with an assurance tool for anatomical studies (AQUA). Pooled prevalence was estimated using a random effects model. RESULTS: A total of 24 studies met the established selection criteria, with a total of 8847 subjects. In this study, 6 articles were included for the meta-analysis with a total of subjects. The average prevalence of VAH reported in each study was 11% (95% CI 10-12%); the studies had a heterogeneity of 41% based on the funnel plot and a low risk of bias. CONCLUSION: The prevalence of VAH is low, but in the presence of this condition, the changes are mainly in diameter rather than morphological. If it is present, some clinical safeguards must be taken to avoid complications such as stroke.

Isolated Apical Hypoplasia of the Left and Right Ventricle.

Isolated apical ventricular hypoplasia is an extremely rare congenital heart disease. We describe 2 cases, each affecting a different side, presenting with unique clinical and imaging characteristics not hitherto delineated in the literature.

Congenital Diaphragmatic Hernia Patients with Left Heart Hypoplasia and Left Ventricular Dysfunction Have Highest Odds of Mortality.

OBJECTIVES: To describe the scope of left ventricular (LV) dysfunction and left heart hypoplasia (LHH) in infants with congenital diaphragmatic hernia (CDH), to determine associations with CDH severity, and to evaluate the odds of extracorporeal membrane oxygenation (ECMO) and death with categories of left heart disease. STUDY DESIGN: Demographic and clinical variables were collected from a single-center, retrospective cohort of patients with CDH from January 2017 through May 2022. Quantitative measures of LV function and LHH were prospectively performed on initial echocardiograms. LHH was defined as ≥2 of the following: z score ≤ -2 of any left heart structure or LV end-diastolic volume <3 mL. LV dysfunction was defined as shortening fraction <28%, ejection fraction <60%, or global longitudinal strain <20%. The exposure was operationalized as a 4-group categorical variable (LV dysfunction +/-, LHH +/-). Logistic regression models evaluated associations with ECMO and death, adjusting for CDH severity. RESULTS: One hundred eight-two patients (80.8% left CDH, 63.2% liver herniation, 23.6% ECMO, 12.1% mortality) were included. Twenty percent demonstrated normal LV function and no LHH (LV dysfunction-/LHH-), 37% normal LV function with LHH (LV dysfunction-/LHH+), 14% LV dysfunction without LHH (LV dysfunction+/LHH-), and 28% both LV dysfunction and LHH (LV dysfunction+/LHH+). There was a dose-response effect between increasing severity of left heart disease, ECMO use, and mortality. LV dysfunction+/LHH + infants had the highest odds of ECMO use and death, after adjustment for CDH severity [OR (95% CI); 1.76 (1.20, 2.62) for ECMO, 2.76 (1.63, 5.17) for death]. CONCLUSIONS: In our large single-center cohort, patients with CDH with LV dysfunction+/LHH + had the highest risk of ECMO use and death.

The Effects of Radial Longitudinal Deficiency on Long-Term Use of the Thumb in Pediatric Patients Following Index Pollicization.

PURPOSE: To assess the effect of radial longitudinal deficiency on the function of pollicized digits as determined by the Thumb Grasp and Pinch (T-GAP) assessment. METHODS: We retrospectively evaluated 25 hands with thumb hypoplasia that underwent index finger pollicization. Patients were followed for an average of 10.4 years. Hands were divided by severity into two groups: no or mild radial longitudinal deficiency (RLD) (Group 1 = 16) and moderate to severe RLD (Group 2 = 9). We collected demographic information and completed physical examination measures, including hand strength, elbow, wrist, and hand range of motion, the Kapandji opposition score, active grasp span, and T-GAP total score. RESULTS: Patients with moderate to severe forms of RLD had stiffer long fingers, lower Kapandji opposition scores, and limited active and passive range of motion for elbow flexion, wrist ulnar deviation, and pollicized thumb interphalangeal flexion. They had shorter forearms, decreased active grasp span, and fewer thumb creases at the interphalangeal thumb joint. In addition, the T-GAP total score was significantly lower when comparing the two groups. Children with mild dysplasia were able to achieve 32% of age-matched normal grasp strength. Patients with more severe radial dysplasia averaged 17% less grasp strength compared with children with mild dysplasia. Patients with moderate to severe RLD also had lower T-GAP total scores and strength measurements if they had limited wrist ulnar deviation. CONCLUSIONS: Individuals with moderate to severe RLD have unique anatomical factors that affect outcomes after pollicization. These individuals use their thumbs for fewer activities, have weaker grasp, and retain more primitive grasp patterns compared with those who have milder forms of RLD. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic IV.

Infiltração resinosa em lesões brancas cariosas e não cariosas: relato de casos / Infiltración resinosa em lesiones blancas cariosas y no cariosas: Reporte de Casos / Resinous infiltration in carious and non-carious white lesions: Case Reports

Introdução: O processo de desmineralização proveniente da cárie leva à formação de manchas brancas que são a primeira manifestação visível da doença. Os infiltrantes resinosos surgem como uma alternativa para o tratamento dessas lesões não cavitadas, pois sãoresinas de baixa viscosidade e faz parte dos procedimentos que visam uma odontologia menos invasiva. Objetivo: Este estudo objetiva relatar a experiência clínica no uso de infiltrante resinoso, Icon, em lesões de mancha branca de etiologias cariosa e não cariosa em dois pacientes distintos, insatisfeitos com a estética do seu sorriso. Descrição do caso: Ao exame clínico foi observado nos pacientes com lesões brancas. No primeiro paciente, de 14 anos, verificou-se a presença da atividade de cárie e micro cavitações. Dessa forma, o plano de tratamento perpassou orientação de higiene oral supervisionada, aplicação de verniz fluoretado, Enamelast, semanal, adequação do meio com restaurações em resina, e só então o uso do infiltrante. A segunda paciente, de 11 anos, já possuía saúde bucal adequada e tinha queixa estética devido à lesão branca não cariosa, hipoplasia, cujo plano de tratamento foi à utilização do Icon apenas. Conclusões: Nos dois casos obteve-se melhoria estética considerável com este procedimento microinvasivo. Melhoria na saúde bucal, aliado à devolução da estética, pode ser observada com o uso de infiltrantes resinosos (AU).

Introduction: The process of demineralization resulting from caries leads to the formation of white spots that are the first visible manifestation of the disease. Resin infiltrants appear as an alternative for the treatment of these non-cavitated lesions, since they are low viscosity resins and are part of the procedures that aim at a less invasive dentistry.Objective:This study aims to report the clinical experience in the use of a resin infiltrant, Icon,in white spot lesions of carious and non-carious etiologies in two different patients who were dissatisfied with the esthetics of their smiles.Methodology: On clinical examination, white lesions were observed in both patients. In the first patient,14 years old,the presence of caries activity and micro cavitations was verified. Thus, the treatment plan included supervised oral hygiene guidance, weekly application of fluoride varnish,Enamelast, adaptation of the environment with resin restorations, and, onlythen, the use of the infiltrant, Icon. The second patient, 11 years old,already had adequate oral health and had an esthetic complaint due to a non-carious white lesion, hypoplasia, whose treatment plan consisted of the use of Icon only.Conclusions:In both cases considerable esthetic improvement was obtained with this microinvasive procedure. Improvement in oral health, combined with the return of esthetics, can be observed with the use of resin infiltrants (AU).

Introducción: El proceso de desmineralización resultante de la caries conduce a la formación de manchas blancas, que son la primera manifestación visible de la enfermedad. Losinfiltrantes de resina aparecen como una alternativa para el tratamiento de estas lesiones no cavitadas, pues se tratan de resinas de baja viscosidad y forman parte de los procedimientos que buscan una odontología menos invasiva. Objetivo:Este estudio tiene como objetivo relatar la experiencia clínica en el uso del infiltrante de resina,Iconen lesiones de mancha blanca de etiologías cariosas y no cariosas en dos pacientes diferentes, insatisfechos con la estética de sus sonrisas.Metodología: En el examen clínico se observaron lesiones blancas en ambos pacientes. En el primer paciente, 14 añosse verificó la presencia de actividad de caries y micro cavitaciones. De ese modo, el plan de tratamiento incluyó la orientación supervisada de la higiene bucal,la aplicación semanal de barniz de flúor (Enamelast), la adaptación del entorno con restauraciones de resina y, sólo después, el uso del infiltrante,Icon. La segunda paciente, 11 años,ya tenía una salud bucal adecuada y presentaba una queja estética debido a una lesión blanca no cariosa, hipoplasia, cuyo plan de tratamiento fue el uso exclusivo de Icon. Conclusiones: En ambos casos, se consiguió una mejora estética considerable con este procedimiento microinvasivo. La mejora de la salud bucal, unida a la recuperación de la estética, puede observarse con el uso de infiltrantes de resina (AU).

Extensive and deep granulomatous ulcers as an atypical manifestation of cartilage-hair hypoplasia syndrome: A diagnostic and therapeutic challenge.

Cartilage hypoplasia syndrome is a primary immunodeficiency disease characterized by short stature, hypoplastic hair and a variable degree of immunodeficiency. Noninfectious cutaneous granulomas represent an uncommon yet well-recognized manifestation within the spectrum of primary immunodeficiency diseases. However, cutaneous granulomas as a manifestation of cartilage-hair hypoplasia syndrome, are extremely rare. We present a case of a middle-aged man with cartilage hypoplasia syndrome featuring cutaneous granulomas, manifesting as chronic, extensive and deep cutaneous ulcers. The patient was treated with anti-TNF-alpha adalimumab with partial improvement. Our case underscores the broad spectrum of clinical manifestations associated with cartilage hypoplasia syndrome and adds new evidence to the potential therapeutic efficacy of anti-TNF-alpha drugs in its treatment.

Presentación de 11 pacientes con el diagnóstico de agenesia e hipoplasia pulmonar diagnosticados en un periodo de 24 años y revisión de la literatura / Presentation of 11 patients diagnosed with pulmonary agenesis and hypoplasia over a period of 24 years and a review of the literature

La agenesia pulmonar (AgP), la aplasia pulmonar (AP) e hipoplasia pulmonar (HP) son malformaciones congénitas poco comunes. En la AgP, no se desarrollan el bronquio ni el pulmón; en la AP, hay un bronquio rudimentario sin parénquima pulmonar; y en la HP, uno o ambos pulmones presentan un tamaño reducido debido a trastornos en su crecimiento. Las causas de la AgP, AP y HP pueden ser tanto primarias como secundarias, predominando estas últimas. Entre ellas se incluyen: oligohidroamnios, anomalías esqueléticas, enfermedades neuromusculares, hernia diafragmática, malformaciones vasculares, cardiopatías complejas, genopatías y cromosomopatías. El rango de manifestaciones varía desde pacientes asintomáticos hasta aquellos con dificultad respiratoria neonatal de leve a severa. Con el tiempo, algunos pacientes pueden experimentar neumonías recurrentes y progresar hacia una enfermedad pulmonar crónica (EPC). La imagenología juega un papel crucial en el diagnóstico. El pronóstico está fuertemente influenciado por la presencia de otras malformaciones congénitas. Generalmente, el enfoque terapéutico es conservador. Este artículo detalla la presentación clínica y la evolución a lo largo de 24 años de 11 pacientes diagnosticados con AgP o HP.

Pulmonary agenesis (AgP), aplasia (AP), and hypoplasia (HP) are rare congenital malformations. In AgP, there is no development of the bronchus or lung; in AP, a rudimentary bronchus is present without lung parenchyma; and in HP, one or both lungs are reduced in size due to growth disorders. The causes of AgP, AP, and HP can be either primary or secondary, with the latter being more common. Examples include oligohydramnios, skeletal anomalies, neuromuscular diseases, diaphragmatic hernia, vascular malformations, complex heart diseases, genopathies, and chromosomal disorders. The spectrum of manifestations ranges from asymptomatic patients to those with mild to severe neonatal respiratory distress. Over time, some patients may experience recurrent pneumonias and progress to chronic lung disease (CLD). Imaging studies are crucial for diagnosis. The prognosis primarily depends on the presence of other congenital malformations. The treatment approach is generally conservative. This article describes the clinical presentation and evolution over 24 years of 11 patients diagnosed with AgP or HP.

X-linked congenital adrenal hypoplasia: Report of long clinical follow-up and description of a new complex variant in the NR0B1 gene.

Adrenal hypoplasia congenita, attributed to NR0B1 pathogenic variants, accounts for more than 50% of the incidence of primary adrenal insufficiency in children. Although more than 250 different deleterious variations have been described, no genotype-phenotype correlation has been defined to date. We report a case of an adopted boy who reported the onset of an adrenal crisis at 2 weeks of age, requiring replacement therapy with mineralocorticoids and glucocorticoids for 4 months. For 3 years, he did well without treatment. At almost 4 years of age, the disorder was restarted. A long follow-up showed the evolution of hypogonadotropic hypogonadism. Molecular studies on NR0B1 revealed a novel and deleterious deletion-insertion-inversion-deletion complex rearrangement sorted in the 5'-3' direction, which is described as follows: (1) deletion of the intergenic region (between TASL and NR0B1 genes) and 5' region, (2) insertion of a sequence containing 37 bp at the junction of the intergenic region of the TASL gene and a part of exon 1 of the NR0B1 gene, (3) inversion of a part of exon 1, (4) deletion of the final portion of exon 1 and exon 2 and beginning of the 3'UTR region, (5) maintenance of part of the intergenic sequence (between genes MAGEB1 and NR0B1, telomeric sense), (6) large posterior deletion, in the same sense. The path to molecular diagnosis was challenging and involved several molecular biology techniques. Evaluating the breakpoints in our patient, we assumed that it was a nonrecurrent rearrangement that had not yet been described. It may involve a repair mechanism known as nonhomologous end-joining (NHEJ), which joins two ends of DNA in an imprecise manner, generating an "information scar," represented herein by the 37 bp insertion. In addition, the local Xp21 chromosome architecture with sequences capable of modifying the DNA structure could impact the formation of complex rearrangements.

Characterization of Three Cases of Primary Hypogalactia in Jalisco, Mexico.

INTRODUCTION: Human lactation should be taken into account as an important issue for the international agenda. Despite advances in lactation information and knowledge, insufficient milk production is still a concern for mothers and health practitioners, including International Board Certified Lactation Consultants and others. Primary hypogalactia, or insufficient milk production is uncommon, but should be considered when there is poor weight gain and decreased urine output in infants despite good latch-on and suckling, or anatomic differences in the physical exam of the lactating breast. MAIN ISSUE: This case series presents three cases illustrating insufficient milk production resulting in infants who experienced significant dehydration and poor weight gain. MANAGEMENT: Primary hypoplasia was diagnosed by means of a thorough interview and physical examination that entailed a consultation with a physician who was also an International Board Certified Lactation Consultant. CONCLUSION: Awareness of an infant's feeding needs and proper evaluation of a child's health status is paramount if health care providers are to identify the important factors contributing to breastfeeding problems. In some instances, breastfeeding goals cannot be achieved, and then the provider's role becomes support in coming to terms with persistent insufficient milk production, and coordinating appropriate supplementation to meet each baby's nutritional needs.

"NASCI case of the month: left ventricular apical hypoplasia".

Left ventricular apical hypoplasia is a rare congenital condition. It can cause nonspecific symptoms and can be accompanied by cardiac conduction system alterations such as bundle branch block, atrial flutter (AF) or atrial fibrillation. The diagnosis mostly is made by imaging.

Developmental Defects of Enamel: A Bibliometric Analysis of the Top 100 Most-Cited Papers.

BACKGROUND: Developmental defects of enamel (DDE) are a result of disturbances during formation and maturation of the enamel. Evaluating the most-cited DDE papers can provide important tools that point to the gaps and strengths of this important topic in dentistry. SUMMARY: This bibliometric study analyzed the 100 most-cited papers on DDE. Using a combined keyword search strategy, the 100 most-cited papers were selected in the Web of Science Core Collection. Papers that addressed any type of DDE were included. The extracted data were title, number of citations, study theme, authorship, journal, type of DDE, type of dentition (primary or permanent), type of diagnosis, study design, year, and country of publication. The bibliometric networks were generated through VOSviewer software. The 100 papers had a range from 78 to 459 citations. The main themes of studies were etiopathogenesis (53%), prevalence and incidence (22%), and diagnosis (8%). The authors with the highest number of citations were Goodman AH and Rose JC (459 citations). Most articles were published in dental journals (47%). The most studied types of DDE were fluorosis and amelogenesis imperfecta in the permanent dentition (47%). Observational (24%) and non-systematic reviews (24%) were the most common study designs and ranged from 1977 to 2019. The country with the highest number of publications was the USA (41%). KEY MESSAGES: Most of the top 100 DDE papers were about fluorosis and amelogenesis imperfecta, with top papers from three continents with English as the native language. This topic is of great importance in dentistry, and the need for further studies is highlighted, especially regarding the diagnosis and treatment of some DDEs.

Impact of Molar Incisor Hypomineralization on Oral Health-Related Quality of Life in Brazilian Schoolchildren Aged 8 to 10 Years

ABSTRACT Objective: To assess the impact of Molar Incisor Hypomineralization (MIH) and confounding factors on oral health-related quality of life (OHRQoL) according to the perception of 8 to 10-year-old children and their parents/caregivers. Material and Methods: A cross-sectional study including 403 students aged 8-10 years was carried out, in which OHRQoL was measured using the Child Perceptions Questionnaire administered to both children and parents/caregivers. The diagnosis of MIH was performed according to the previously proposed index. Dental caries experience, malocclusion, and sociodemographic factors were evaluated as confounders. Cluster analysis and Poisson regression with robust variance (p<0.05) were performed. Results: The prevalence of MIH was 13.4%. Parents/caregivers of children with MIH in incisors showed a higher impact prevalence in the emotional well-being domain (PR=1.92; 95%CI=1.16-3.19). Children with hypoplasia had a higher prevalence of negative impact on OHRQoL in the oral symptoms domain (PR=1.51; 95%CI=1.03-2.23). According to the perception of parents/caregivers, dental caries experience had a negative impact on the quality of life of students in the emotional well-being domain (PR=4.19; 95%CI=1.06-16.49) and in the total questionnaire score (PR=3.21; 95%CI=1.06-9.71). Conclusion: According to the perception of parents/caregivers, children with MIH in incisors showed a greater impact on OHRQoL. Additionally, the presence of hypoplasia affected the self-perception of OHRQoL in children, and caries experience influenced the OHRQoL of children, as perceived by parents/caregivers.

Characterization of enamel developmental defects in patients with orofacial clefts and their relationship to surgical procedures.

AIMS: This study aimed to characterize the prevalence of development defects of enamel (DDE) in patients with cleft based on the cleft phenotype and explore the relationship between surgical procedures and different types of DDE. MATERIAL AND METHODS: In this cross-sectional study, 290 standardized orthodontic documentation and medical records from a reference hospital were evaluated, which treated patients with: cleft lip (CL), cleft lip with alveolar bone involvement (CLa), cleft lip and palate (CLP), cleft palate (CP), cleft median (CM), and considering laterality as unilateral or bilateral. DDE was assessed using the Ghanim Index (2015). Information on surgical intervention periods was obtained from medical records. Statistical analyses were performed using prevalence ratio (PR) for DDE comparisons between cleft phenotypes and surgical procedures. RESULTS: The prevalence of DDE was 77.2%. Demarcated hypomineralization was associated with CP and CLP, while hypoplasia was associated with CLa, especially when bilateral. Hypoplasia was also associated with the labial adhesion surgery. CONCLUSION: Demarcated hypomineralization was the most common DDE in this population, and the cleft phenotype influenced the type of DDE manifested. The lip adhesion surgery increased the chances of hypoplasia manifestation. CLINICAL RELEVANCE: The type of DDE in patients with cleft depends on the cleft phenotype. Understanding this susceptibility enables the multidisciplinary team to monitor dental development, thus allowing early diagnosis and timely referral to the pediatric dentist and better prognoses.