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Ginekologia Polska 2021The rusty pipe syndrome is an uncommon condition. It is characterised by suddenly painless, bilateral bloody nipple discharge with no visible evidence of mechanical...
The rusty pipe syndrome is an uncommon condition. It is characterised by suddenly painless, bilateral bloody nipple discharge with no visible evidence of mechanical injuries within the breasts. It resolves spontaneously with no additional medical intervention. If the problem persists for more than 5-7 days, further investigations should be made to exclude other pathologies. In the available literature, there is no clear explanation of the condition. Many authors agree that the condition may be caused by the structure of blood vessels and may depend on changes therein that occur during stage I and II lactogenesis. In most cases, it is recognised during breast milk expression, when the colour of milk is different than normally.
Topics: Breast; Breast Feeding; Female; Humans; Syndrome
PubMed: 35014016
DOI: 10.5603/GP.a2021.0188 -
Surgical and Radiologic Anatomy : SRA Oct 2019Here, we present a description of Poland syndrome from the second session of the Anatomical Society (Paris, France) on 11 December 1803 of congenital mammary absence and...
Here, we present a description of Poland syndrome from the second session of the Anatomical Society (Paris, France) on 11 December 1803 of congenital mammary absence and muscular atrophy on the right side. This case report predates the first official description of the disease published by Alfred Poland in Guy's Hospital Reports (London, 1841). Consequently, perhaps would it be necessary to do justice to its French discoverer, and to name from now on this nosological entity the "syndrome of Marandel"?
Topics: Female; Humans; Anatomy; Breast; France; History, 19th Century; Pectoralis Muscles; Poland Syndrome; Societies, Scientific
PubMed: 30937564
DOI: 10.1007/s00276-019-02232-9 -
Respirology Case Reports Mar 2024The key feature of Poland syndrome is asymmetry in the chest wall. Clinicians should be alert to abnormalities of the chest wall as well as the lungs if there is...
The key feature of Poland syndrome is asymmetry in the chest wall. Clinicians should be alert to abnormalities of the chest wall as well as the lungs if there is abnormal chest radiograph lucency.
PubMed: 38504769
DOI: 10.1002/rcr2.1326 -
Joint Diseases and Related Surgery 2021Radioulnar synostosis is a rare disease which causes supination and pronation restriction as a result of osseous or fibrous connections between the radius and ulna....
Radioulnar synostosis is a rare disease which causes supination and pronation restriction as a result of osseous or fibrous connections between the radius and ulna. Radioulnar synostosis includes both congenital and post-traumatic types. Post-traumatic radioulnar synostosis can be seen in the proximal, middle, and distal part of the forearm, depending on the location of the trauma. Congenital proximal radioulnar synostosis occurs as a result of a separation defect between the radius and ulna in the embryonic period. In the presence of congenital proximal radioulnar synostosis, the patient should be evaluated for accompanying syndromes and possible developmental anomalies. In this report, we present a rare case of both proximal and distal radioulnar synostosis. Hypoplasia of the right pectoral muscle mass, hypoplastic appearance of the right nipple, presence of proximal and distal radioulnar synostosis in the right forearm, and accompanying symbrachydactyly suggested Poland syndrome. To the best of our knowledge this is the first case of congenital proximal and distal radioulnar synostosis with Poland syndrome.
Topics: Humans; Poland Syndrome; Radius; Synostosis; Ulna
PubMed: 34842120
DOI: 10.52312/jdrs.2021.100 -
Journal of Clinical Medicine Sep 2021Poland syndrome is a rare, challenging combination of chest wall and breast deformities for reconstructive surgeons and selecting the treatment can prove difficult. This...
INTRODUCTION
Poland syndrome is a rare, challenging combination of chest wall and breast deformities for reconstructive surgeons and selecting the treatment can prove difficult. This study aims to help surgeons in choosing the best viable option for treatment by sharing our institutional experience and proposing a guiding algorithm.
METHODS
A retrospective analysis of all patients with Poland syndrome undergoing treatment for breast and chest wall deformities at a single institution between December 2011 and May 2020 was performed. Medical charts were reviewed to allow for a description of patient demographics, treatment modalities and complications. A treatment algorithm to aid in selecting the adequate reconstructive option based on our institutional experience was formulated.
RESULTS
A total of 22 patients (six male, 16 female) were identified who received treatment for Poland Syndrome related deformities. Nine received microsurgical free flap reconstruction (three Deep Inferior Epigastric Perforator flaps, six Transverse Myocutaneous Gracilis flaps), two received reconstruction with a local flap (two Latissimus dorsi flaps), nine received implant based reconstruction, and two were treated with autologous free fat transfer only (17 in combination with other surgical methods).
CONCLUSION
Free flap reconstruction with the TMG flap is a valid option for patients with low Body Mass Index (BMI), while Deep Inferior Epigastric Perforator flaps should be considered for patients with a higher BMI. Autologous free fat transfer proves to be a safe and efficient treatment option in mild cases of Poland syndrome for male and female patients, in combination with or without implant based reconstructive surgery. Multicentre studies should be conducted to achieve higher case numbers of this rare disease and support clinical decisions with more data.
PubMed: 34640539
DOI: 10.3390/jcm10194515 -
BMJ Case Reports May 2021Poland's syndrome (PS) is a rare developmental anomaly that can manifest mild (pectoralis muscles involvement) to severe deformities (rib hypoplasia and hand...
Poland's syndrome (PS) is a rare developmental anomaly that can manifest mild (pectoralis muscles involvement) to severe deformities (rib hypoplasia and hand deformities). We report a case of 69-year-old man who presented to the emergency department with a traumatic chest injury after a fall. It was initially thought to have a significant chest injury as the trauma survey revealed a palpable defect and tenderness in the right anterior chest wall. There was also a symbrachydactyly deformity in the right hand. CT of the chest showed lack of right pectoralis muscles, which were consistent with PS. This case highlights the importance of gathering detail history in adult trauma patients such as congenital disorder especially in the presence of bony deformity. With possibilities of several traumatic conditions in trauma patients eliminated, one can expand the non-traumatic differential, keeping in mind the possibility of a congenital disorder that can mimic traumatic chest injury.
Topics: Adult; Aged; Humans; Male; Pectoralis Muscles; Poland Syndrome; Syndactyly; Thoracic Injuries; Thorax
PubMed: 34059541
DOI: 10.1136/bcr-2020-241408 -
Indian Pediatrics Apr 2022
Topics: Brugada Syndrome; DiGeorge Syndrome; Electrocardiography; Humans
PubMed: 35410974
DOI: 10.1007/s13312-022-2505-6 -
Reproductive Toxicology (Elmsford, N.Y.) Jan 2024Bisphenols (BPs) have become a chemical group of special interest due to their ability to interfere with the endocrine system and their ubiquitous presence in the...
Bisphenols (BPs) have become a chemical group of special interest due to their ability to interfere with the endocrine system and their ubiquitous presence in the environment. As some of them possess mild estrogenic and anti-androgenic effects, they might be associated with the diagnosis of polycystic ovary syndrome (PCOS). Acting on multiple tissues, BPs exposure may lead to metabolic derangements characteristic for metabolic syndrome (MetS). Therefore, the aim of this study was to determine the potential relationship between exposure to some BPA analogues and features of the MetS in women with PCOS. Serum BPE, BPC, BPG, BPM, BPP, BPZ, BPFL, and BPBP concentrations did not differ significantly between the PCOS (n = 135) and the control subjects (n = 104). However, women whose serum BPM and BPP concentrations were in the highest tertile were more likely to be diagnosed with PCOS (adjusted OR; [95%CI] 0.43; [0.20; 0.89], P < 0.001 and 0.56; [0.27; 0.96], P = 0.049, consequently). Serum concentrations of BPs were not associated with the MetS diagnosis in the PCOS group. There was a negative correlation between the concentrations of serum BPBP and total serum cholesterol (r = - 0.153; P = 0.019), BPE and serum testosterone (r = - 0.160; P = 0.014) as well as BPC and HDL-cholesterol (r = - 0.138; P = 0.036). There was a positive correlation between the concentrations of BPP and serum triglycerides (r = 0.138; P = 0.036). Our results point to the potential association between exposure to BPM, BPP, and the diagnosis of PCOS, along with the impact of BPBP, BPE, BPC, and BPP on the metabolic features of the MetS.
Topics: Female; Humans; Polycystic Ovary Syndrome; Metabolic Syndrome; Phenols; Cholesterol; Benzhydryl Compounds
PubMed: 37984601
DOI: 10.1016/j.reprotox.2023.108511 -
Journal of Oral and Maxillofacial... Jan 2022The authors' aim was to review the literature in terms of the etiology of the syndrome, the frequency of Silent Sinus Syndrome (SSS) and surgical procedure, as well as... (Review)
Review
PURPOSE
The authors' aim was to review the literature in terms of the etiology of the syndrome, the frequency of Silent Sinus Syndrome (SSS) and surgical procedure, as well as to present their own experience.
METHODS
The authors used PubMed, Medline, and Science Direct websites to find and review the most significant papers related to SSS. The case reports of SSS published between 2010 and 2020 were reviewed. A retrospective case review of 8 patients with SSS treated at the authors' departments was done.
RESULTS
The silent sinus syndrome has been reported in both children and adults. It is relatively rare and should be differentiated from congenital sinus hypoplasia or atelectasis. It most often affects the maxillary sinus. SSS is usually diagnosed when facial asymmetry or vision problems occur. Late diagnosis requires endoscopic sinus surgery, involving orbital wall reconstruction. The etiology of the syndrome, including the role of bacterial flora found in the sinuses, is unclear.
CONCLUSION
Early diagnosis of SSS enables avoiding orbital complications and limits surgical intervention to endoscopic surgery. Further research into bacteriology may help to understand the pathophysiology of the silent sinus syndrome.
Topics: Adult; Child; Early Diagnosis; Enophthalmos; Humans; Maxillary Sinus; Paranasal Sinus Diseases; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 34597532
DOI: 10.1016/j.joms.2021.08.166 -
Italian Journal of Pediatrics Oct 2020Shwachman-Diamond syndrome (SDS) is a rare genetic, multi-systemic disease characterized by exocrine pancreatic insufficiency, immune deficiency, bone marrow failure and...
BACKGROUND
Shwachman-Diamond syndrome (SDS) is a rare genetic, multi-systemic disease characterized by exocrine pancreatic insufficiency, immune deficiency, bone marrow failure and skeletal abnormalities. Most patients present with failure in somatic development and short stature, but systematic data concerning those features are limited. The aim of the study was to assess the prevalence of failure in somatic development in the children with SDS.
METHODS
An analysis of anthropometric measurements of 21 patients (14 girls and 7 boys),aged 2 to 17 years (mean age 6.3 years) with SDS diagnosed in The Children's Memorial Health Institute in Warsaw, Poland was performed. The patients were measured using a Holtain Limited stadiometer, an electronic scale, a Harpenden anthropometer, a metric tape and a spreading caliper. The assessed anthropometric parameters were expressed as standard deviation scores in relation to the reference values in Poland, suitable for sex as well as calendar and growth age.
RESULTS
A total of 66 measurements was collected and analyzed with a median number of 3 observations per patient. The group of boys presented with a significantly lower height (- 3.0 SD, p < 0.0001) and BMI (- 1.4 SD, p < 0.00001), and in the relation to the growth age a lower weight (- 1.0 SD, p < 0.001) as well as a smaller chest width (- 0.9 SD, p < 0.05), hip width (- 0,5 SD, p < 0,05) and lower limb length (- 0,5 SD, p < 0,05). The group of girls also showed significantly lower height (- 2.6 SD, p < 0.00001) and BMI (- 0.8 SD, p < 0.00001), and in relation to the growth age, lower weight (- 0.5 SD, p < 0.001) as well as decreased width of the chest (- 1.7 SD, p < 0.0001) and shoulder (- 1.0 SD, p < 0.001) were observed. Boys and girls were also characterized by significantly decreased circumference and width of head, additionally, girls had also smaller head length.
CONCLUSIONS
Patients with SDS have abnormal somatic development. Both boys and girls are characterized by short stature, decreased weight, BMI, leg length, chest width as well as circumference and width of head. Anthropometric measurements provide important data on the process of growth and body proportions in children with SDS.
Topics: Adolescent; Anthropometry; Child; Child Development; Child, Preschool; Female; Humans; Infant; Male; Poland; Shwachman-Diamond Syndrome
PubMed: 33046118
DOI: 10.1186/s13052-020-00919-z