Case managers

Background: Maternal phenylketonuria (mPKU) is a pathologic condition occurring in the fetus of a mother with PKU that is caused by prolonged elevated intrauterine blood phenylalanine (Phe) levels, which can lead to congenital abnormalities and mental retardation of newborns. Management of PKU during pregnancy can be challenging as protein substitutes may exacerbate nausea, vomiting, and gastrointestinal symptoms. Aim: To report the successful management of four PKU pregnant women. Methods: The patients were administered with prolonged-release amino acid supplementation and were recommended to follow a strict diet...

BACKGROUND: Left ventricular assist devices (LVAD) improve survival for patients with cardiac failure, but LVAD specific infections (VSI) remain a challenge with poorly understood predictive risk factors. Furthermore, the indications and utility of escalating medical treatment to surgical debridement and potential flap reconstruction are not well-characterized. STUDY DESIGN: A retrospective review of consecutive patients undergoing primary LVAD implantation at a tertiary academic center was performed...

BACKGROUND: Toxic shock syndrome (TSS) is a rare but potentially life-threatening disease caused by superantigen-producing Gram-positive bacteria such as Staphylococcus aureus and Streptococcus pyogenes. Staphylococcal TSS received special attention from 1978 to 1981, when an epidemic was observed associated with the use of hyper-absorbent tampons. Today the disease is rare and generally not related to menstruation, but can occur postpartum or in post-surgical wounds, intrauterine devices (IUDs), burns or other soft tissue injuries, mastitis or other focal infections...

The management of lymphatic fistulas following surgical procedures, in particular after inguinal lymphadenectomy, represents a significant clinical challenge. The current case report shows the novel use of the superficial circumflex iliac perforator (SCIP) pedicle vein for lymphovenous anastomosis (LVA) to treat a chronic inguinal lymphatic fistula in a 58-year-old male patient. This patient had developed a persistent lymphorrhea and wound dehiscence after a right inguinal lymph node biopsy performed for oncological reasons 1...

BACKGROUND: Post-oncologic surgical reconstruction of lower limbs in pediatrics remains a challenging topic. Microsurgical techniques allow reconstructions of large bony defects. The use of vascularized fibular flap with allograft has proven to be an ideal biologic construct. We aim to assess the success rate of this operation, including flap survival, bony union, weight-bearing ambulation, and complications in a long-term follow-up in our case series compared to the literature. PATIENTS AND METHODS: Our case-series includes 18 femoral resections (9 osteosarcomas, 8 Ewing sarcoma, and 1 desmoid tumor) and 15 tibial resections (10 osteosarcoma, 4 Ewing sarcoma, and 1 Malignant Fibrous Histiocytoma)...

Dens invaginatus (DI) is a developmental anomaly of the teeth characterised by the in-folding of the enamel into the dentin. Oehlers' Type III DI is the most serious form, in which the inherently invaginated channels communicate with periodontal and dental pulp tissue, increasing the risk of bacterial contamination. However, varying and complex anatomical features make diagnosis and treatment challenging. Conventional endodontic therapies promote healing by avoiding unnecessary interventions (e.g., surgical or other invasive treatments)...

Background: Tularemia is one of the most prevalent zoonoses across the world. Patients in Turkiye mostly contract the oropharyngeal form, acquired through drinking, or contact with microorganism-contaminated water. Methods: Patients with oropharyngeal tularemia aged under 18 years and diagnosed between January 01, 2017, and December 31, 2020, were evaluated retrospectively. Tularemia was diagnosed in patients with compatible histories, symptoms, clinical presentations, and laboratory test results. Results: The mean age of 38 children was 12...

OBJECTIVE: Endoscopic endonasal surgery is effective in the treatment of sinonasal cancers. However, in cases of well-differentiated locally advanced neoplasms as well as recurrences, the most appropriate treatment is debated. The purpose of this study is to report a mono-institutional experience on craniofacial surgery performed in a tertiary-care referral centre. METHODS: This was a retrospective analysis of 90 patients treated with transcranial and/or transfacial resection for sinonasal cancer between 2010 and 2020...

Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by hypothalamic dysfunction, hypotonia, cognitive deficits, and hyperphagia, primarily resulting from genetic abnormalities on chromosome 15. Among its varied manifestations, musculoskeletal issues, notably scoliosis, pose important challenges in management. This study aims to investigate differences in postural-motor development and spinal range of movement between preadolescents and adolescents with PWS, with and without scoliosis, while also exploring the potential impact of scoliosis on caregiving burden, an aspect yet to be thoroughly explored in existing literature...

OBJECTIVE: Medial epicondyle fractures are a common pediatric injury. When operative, cannulated partially threaded screws, with or without a washer, are commonly utilized. These implants may need to be removed after full healing if symptomatic. There is mixed evidence regarding the influence of a washer on rates of implant removal, and the influence of screw size has not been studied. We aim to determine the rate of symptomatic deep implant removal for each fixation type and identify factors associated with the need for removal...

The purpose of this study was to determine if women footballers have an increased lack of neuromuscular control of the knee joint after a concussion compared to a healthy cohort tested with tensiomyography (TMG). Forty-one female collegiate footballers were enrolled in this study from which there were 20 with a history of sports-related concussions (SRCs) and 21 control subjects. Results from the SRC group had significantly higher Tc (ms) (z = -5.478, p = 0.000) and significantly lower Dm (mm) (z = -3.835, p = 0...

There is increasing interest in using extended genetic sequencing (EGS) in newborn screening (NBS) for cystic fibrosis (CF). How this is implemented will change the number of children being given an uncertain outcome of CRMS/CFSPID (cystic fibrosis transmembrane conductance regulator (CFTR)-related metabolic syndrome/CF Screen Positive Inconclusive Diagnosis), probable carrier results, and the number of missed CF diagnoses. An international survey of CF health professionals was used to gather views on two approaches to EGS-specific (may reduce detection of CRMS/CFSID but miss some CF cases) versus sensitive (may increase detection of CRMS/CFSPID but avoid missing more CF cases)...

Properly selecting landfill sites for waste disposal is crucial for mitigating environmental and public health risks. Geographic Information Systems (GISs) and Artificial Intelligence (AI) techniques have emerged as valuable tools for identifying suitable landfill locations. This study presents a systematic mapping study (SMS) that investigates the usage of GIS and AI in landfill site selection. We searched six databases (IEEE Xplore, ACM Digital Library, Science Direct, Emerald Insight, Taylor & Francis Online and Web of Science) using predefined keywords related to landfills, GIS and AI...

OBJECTIVE: To comprehensively analyze reported cases of nasolacrimal squamous cell carcinoma (NLSCC), focusing on risk factors, treatment modalities, and outcomes. Additionally, investigate the impact of human Papillomavirus (HPV) status and histopathological subtypes' impact on prognosis. DATA SOURCES: Pubmed, Embase. REVIEW METHODS: We conducted a systematic literature review to identify relevant studies reporting cases of NLSCC. The review methods adhered to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines...

BACKGROUND: First-line treatment for symptomatic intracranial atherosclerotic disease (ICAD) is medical management; however, interventional approaches are increasingly considered for refractory disease. The Resolute Onyx in TIA management (ROTIA) study is a post-market evaluation of the Resolute Onyx (R-Onyx) drug-eluting stent in the treatment of recurrent transient ischemic attacks (TIAs) due to refractory ICAD (off-label use). METHODS: This is a single-center, retrospective case series of consecutive patients who underwent angioplasty and stenting with R-Onyx for treatment of recurrent TIAs due to refractory ICAD from October 2019 to November 2022...

BACKGROUND: Adenocarcinoma of the rete testis (AORT) is an extremely rare and aggressive tumor with a poor prognosis. Its etiology and pathological characteristics have not been extensively studied, making accurate diagnosis and appropriate management challenging. AORT, an invasive testicular tumor with a mortality rate of 46%, treatment typically involves radical orchiectomy, retroperitoneal pelvic lymph node dissection (RPLND), adjuvant chemotherapy, and/or ongoing monitoring, but the response to conventional radiation and chemotherapy is limited...

BACKGROUND: The objective of our study is to describe the clinical presentation, management, and outcome of a large cohort with nontuberculous mycobacteria (NTM) hand infection. METHODS: We reviewed the medical records of all adults (≥18 years) managed at the Mayo Clinic (Rochester, MN) for NTM hand infection between 1998 and 2018. RESULTS: Our cohort included 81 patients. The median age was 61.3 (interquartile range 51.7, 69.6) years; 39...

Though typically self-limiting, severe mpox infections have been treated with antiviral medications, most notably tecovirimat. Various reports exist of mpox progression despite tecovirimat treatment. Treatment resistance can be due to acquired mpox strain mutations, most often occurring in an immunocompromised host. We present the case of a male with AIDS who developed disseminated treatment-resistant mpox infection complicated by superimposed bacterial and fungal infections. His orthopoxvirus polymerase chain reaction result remained positive despite treatment with 4 weeks of oral tecovirimat and 3 doses of intravenous cidofovir...

BACKGROUND: Tuberculosis (TB) is recognized as a significant global public health concern. Still, there remains a dearth of comprehensive evaluation regarding the specific indicators and their influencing factors of delay for adolescents and young adults. METHODS: All notified pulmonary TB (PTB) patients in Jiaxing City were collected between 2005 and 2022 from China's TB Information Management System. Logistic regression models were conducted to ascertain the factors that influenced patient and health system delays for PTB cases, respectively...

BACKGROUND: No standardized criteria for surgical resection of parasagittal sinus meningiomas (PSM) have been established, and different surgical strategies have been proposed. The aim of the present study was to investigate the efficacy and safety of a "radical" surgical strategy in the treatment of PSM. METHODS: The clinical histories, radiological findings, pathologic features, and surgical records of 53 patients with PSM admitted by the same surgical team using the "radical" surgical strategy were retrospectively analyzed between 2018 and 2023...