OMENS-Plus: Analysis of Craniofacial and Extracraniofacial Anomalies in Hemifacial Microsomia

1995 ◽  
Vol 32 (5) ◽  
pp. 405-412 ◽  
Author(s):  
Joan E. Horgan ◽  
Bonnie L. Padwa ◽  
Richard A. Labrie ◽  
John B. Mulliken

This review of 121 patients with hemifacial microsomia (HFM) revealed that 67 (55.4%) had extracraniofacial anomalies. Sixteen patients (13%) had one extracraniofacial anomaly and 51 patients (42.4%) had anomalies of multiple organ systems. There was no gender or side predominance in the cohort with the HFM “expanded spectrum.” Central nervous system (CNS), cardiac, and skeletal anomalies were “associated” (i.e., had frequencies of 10% or more). Pulmonary, gastrointestinal, and renal deformities were equivocally associated. Statistical analysis indicated significant associations between several orbital, mandibular, ear, neural, and soft tissue (OMENS) variables and extracraniofacial anomalies. Patients with extracraniofacial structural defects had higher OMENS grades for individual craniofacial anatomic categories. Furthermore, patients with expanded spectrum had higher total OMENS scores. The frequency of cardiac anomalies (26%) supports the model of neural crest involvement in the pathogenesis of both hemifacial microsomia and conotruncal defects. The majority of the heart defects in this study were of either the outflow or septal type. We propose that the OMENS classification system for craniofacial anomalies of HFM be expanded to OMENS-Plus (+) to designate the presence of associated extracraniofacial anomalies.

2019 ◽  
Vol 5 (3) ◽  
pp. 70 ◽  
Author(s):  
James Riddell ◽  
L. Joseph Wheat

Histoplasmosis is an endemic fungal infection that may affect both immune compromised and non-immune compromised individuals. It is now recognized that the geographic range of this organism is larger than previously understood, placing more people at risk. Infection with Histoplasma capsulatum may occur after inhalation of conidia that are aerosolized from the filamentous form of the organism in the environment. Clinical syndromes typically associated with histoplasmosis include acute or chronic pneumonia, chronic cavitary pulmonary infection, or mediastinal fibrosis or lymphadenitis. Disseminated infection can also occur, in which multiple organ systems are affected. In up to 10% of cases, infection of the central nervous system (CNS) with histoplasmosis may occur with or without disseminated infection. In this review, we discuss challenges related to the diagnosis of CNS histoplasmosis and appropriate treatment strategies that can lead to successful outcomes.


2013 ◽  
Vol 2013 ◽  
pp. 1-2
Author(s):  
Syed Hassan ◽  
Fatima Khalid ◽  
Zaid Alirhayim ◽  
Syed Amer

Lithium toxicity is known to affect multiple organ systems, including the central nervous system. Lithium levels have been used in the diagnosis of toxicity and in assessing response to management. There is evidence that nonsteroidal anti-inflammatory medications (NSAIDs) can increase lithium levels and decrease renal lithium clearance. We present a case of lithium toxicity, which demonstrates this effect and also highlights the fact that lithium levels do not correlate with clinical improvement, especially the neurological deficit.


Author(s):  
Samaneh Nakhaee ◽  
Christopher Hoyte ◽  
Richard C. Dart ◽  
Masoumeh Askari ◽  
Roland J. Lamarine ◽  
...  

Abstract Aims As an analgesic that acts upon the central nervous system (CNS), tramadol has gained popularity in treating moderate to severe pain. Recently, it has been increasingly reported as a drug of misuse with intentional overdoses or intoxications. This review focuses on tramadol intoxication in humans and its effects on different systems. Subject and method This narrative review provides a comprehensive view of the pharmacokinetics, mechanism of action, and incidence of tramadol toxicity with an in-depth look at its side effects. In addition, the main approaches to the management of tramadol poisoning are described. Results Tramadol poisoning can affect multiple organ systems: gastrointestinal, central nervous system (seizure, CNS depression, low-grade coma, anxiety, and over time anoxic brain damage), cardiovascular system (palpitation, mild hypertension to life-threatening complications such as cardiopulmonary arrest), respiratory system, renal system (renal failure with higher doses of tramadol intoxication), musculoskeletal system (rhabdomyolysis), endocrine system (hypoglycemia), as well as, cause serotonin syndrome. Seizure, a serious nervous disturbance, is more common in tramadol intoxication than with other opioids. Fatal tramadol intoxications are uncommon, except in ingestion cases concurrent with other medications, particularly CNS depressants, most commonly benzodiazepines, and ethanol. Conclusion With the increasing popularity of tramadol, physicians must be aware of its adverse effects, substantial abuse potential, and drug interactions, to weigh its risk–benefit ratio for pain management. Alternative therapies might be considered in patients with a previous overdose history to reduce risks for adverse outcomes.


2017 ◽  
Vol 9 (3) ◽  
Author(s):  
Erna Suparman ◽  
Eddy Suparman

Abstract: Secondary amenorrhea occurs when a woman in reproductive age who has experienced menstruation, at a sudden stops menstruating for at least three consecutive months. The basic principle underlying the physiology of menstrual function is composed of multiple organ systems with their appropriate compartments in which the menstrual cycle depends on, as follows: compartment I, disorders of the uterus; compartment II, disorders of the ovary; compartment III, disorders of the anterior pituitary; and compartment IV, disorders of the central nervous system (hypothalamus). Finding the cause of secondary amenorrhoea can be done by doing some tests or trials. Determination of the location of the specific anatomical defect is useful to obtain appropriate treatment according to the cause of amenorrhea.Keywords: secondary amenorrhoeaAbstrak: Dikatakan amenorea sekunder bila seorang wanita usia reproduktif yang pernah mengalami haid, tiba-tiba haidnya berhenti untuk sedikitnya 3 bulan berturut-turut. Prinsip dasar yang mendasari fisiologi dari fungsi menstruasi memungkinkan penyusunan beberapa sistem kompartemen yang tepat di mana siklus menstruasi bergantung, yaitu: kompartemen I gangguan pada uterus, kompartemen II gangguan pada ovarium, kompartemen III gangguan pada hipofisis anterior, dan kompartemen IV gangguan pada sistem saraf pusat (hipotalamus). Gangguan ini sering berhubungan dengan keadaan stres (wanita pengungsi, dipenjara, hidup dalam ketakutan), atlit wanita, atau anoreksia nervosa dan bulimia. Mencari penyebab amenorea dapat diperoleh dengan melakukan beberapa uji atau percobaan. Penentuan lokasi defek anatomis spesifik sangat bermanfaat untuk mendapatkan penanganan yang sesuai dengan penyebab amenore.Kata kunci: amenorea sekunder


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Guojia Du ◽  
Yandong Li ◽  
Pan Wu ◽  
Xin Wang ◽  
Riqing Su ◽  
...  

Abstract Background To explore central nervous system (CNS) involvement in this disease, from the perspectives of diagnosis, treatment, and misdiagnosis Methods Twenty-eight patients with CNS echinococcosis were included in this retrospective study, including 18 males (64.3%) and 10 (35.7%) females. The average age of all the patients were 23.5 years (ranged 4–60 years). Twenty-three (23) patients (82.1%) received the first surgical resection in our hospital. Five (5) patients (17.9%) gave up surgical treatment for multiple-organ hydatidosis and previous surgery history at other hospitals, and albendazole was applied for a long-term (3–6 months) adjunct therapy for the 5 patients. The average follow-up time was 8 years. Results For the 28 patients, 23 cases received surgical treatments, and the diagnosis was confirmed by pathological examinations. The diagnosis of 4 cases of brain echinococcosis and 2 cases of spinal cord echinococcosis could not be confirmed, resulting in a misdiagnosis rate of 21.4% (6/28). For the pathological examination, a total of 17 cases were infected with Echinococcus granulosus (including 2 cases of spinal cord echinococcosis), and 6 cases were infected with Echinococcus alveolaris. Conclusion The diagnosis should be specifically considered in endemic regions. The clinical features of CNS hydatidosis were intracranial space-occupying lesions. For the treatment, the surgical removal of cysts should be necessary. In addition, the adjuvant therapy with drug and intraoperative prophylaxis is also suggested. The misdiagnosis may have resulted from atypical clinical features and radiographic manifestations, as well as the accuracy of hydatid immunologic test.


Author(s):  
C. Welzl ◽  
A.L. Leisewitz ◽  
L.S. Jacobson ◽  
T. Vaughan-Scott ◽  
E. Myburgh

This study was designed to document the systemic inflammatory response syndrome (SIRS) and multiple-organ dysfunction syndrome (MODS) in dogs with complicated babesiosis, and to assess their impact on outcome. Ninety-one cases were evaluated retro-spectively for SIRS and 56 for MODS. The liver, kidneys, lungs, central nervous system and musculature were assessed. Eighty-seven percent of cases were SIRS-positive. Fifty-two percent of the cases assessed for organ damage had single-organ damage and 48 % had MODS. Outcome was not significantly affected by either SIRS or MODS, but involvement of specific organs had a profound effect. Central nervous system involvement resulted in a 57 times greater chance of death and renal involvement in a 5-fold increased risk compared to all other complications. Lung involvement could not be statistically evaluated owing to co-linearity with other organs, but was associated with high mortality. Liver and muscle damage were common, but did not significantly affect outcome. There are manysimilarities between the observations in this study and previous human and animal studies in related fields, lending additional support to the body of evidence for shared underlying pathophysiological mechanisms in systemic inflammatory states.


2021 ◽  
Author(s):  
Bashaer Abu Khatir ◽  
Gordon Omar Davis ◽  
Mariam Sameem ◽  
Rutu Patel ◽  
Jackie Fong ◽  
...  

Tuberin is a member of a large protein complex, Tuberous Sclerosis Complex, and acts as a sensor for nutrient status regulating protein synthesis and cell cycle progression. Mutations in the Tuberin gene, TSC2, lead to the formation of tumors and developmental defects in many organ systems, including the central nervous system. Tuberin is expressed in the brain throughout development and levels of Tuberin have been found to decrease during neuronal differentiation in cell lines in vitro. Our current work investigates the levels of Tuberin at two stages of embryonic development in vivo, and we study the mRNA and protein levels during a time course using immortalized cell lines in vitro. Our results show that Tuberin levels remain stable in the olfactory bulb but decrease in the Purkinje cell layer during embryonic mouse brain development. We show here that Tuberin levels are higher when cells are cultured as neurospheres, and knockdown of Tuberin results in a reduction in the number of neurospheres. These data provide support for the hypothesis that Tuberin is an important regulator of stemness and the reduction of Tuberin levels might support functional differentiation in the central nervous system. Understanding how Tuberin expression is regulated throughout neural development is essential to fully comprehend the role of this protein in several developmental and neural pathologies.


Neurosurgery ◽  
1991 ◽  
Vol 29 (4) ◽  
pp. 612-616 ◽  
Author(s):  
Hendrikus G.J. Krouwer ◽  
John Vollmerhausen ◽  
Joel White ◽  
Michael D. Prados

Abstract A case is reported in which a desmoplastic medulloblastoma metastasized to the pancreas and to the surrounding soft tissues but did not recur locally or disseminate within the central nervous system. Multidrug chemotherapy and local radiation therapy resulted in a complete remission. In all four previously reported cases of medulloblastoma metastasizing to the pancreas, the diagnosis was not made until the postmortem examination, and all of these patients also had extensive metastases in other organ systems. Modification of the Weiss criteria defining extraneural metastases from tumors of the central nervous system is suggested.


2019 ◽  
Author(s):  
Bryan Cook ◽  
Krystina Geiger ◽  
Megan Barra

Opioid agonists are frequently used to provide anesthesia in combination with sedatives and hypnotic agents and manage postoperative acute pain. There are many different opioid agents available that differ in their potency, onset and duration of action, metabolism, drug interactions, and side-effect profile. All opioids have distinct effects upon various organ systems, including central nervous system depression, respiratory depression, and decreased gastrointestinal motility. Fentanyl and fentanyl-derived agents (alfentanil, sufentanil, remifentanil) are most frequently used in the intraoperative period due to their quick onset and duration of action, allowing them to be easily titrated and discontinued at the completion of a procedure. Oral opioids with moderate durations of action, such as oxycodone, hydrocodone, and morphine, are commonly used for acute pain management in the postoperative setting. When oral analgesics cannot be used, intravenous patient-controlled analgesia is another option for pain management. This review contains 5 figures, 11 tables, and 59 references. Key Words: analgesia, anesthesia, central nervous system depression, fentanyl, morphine, opioid agonist, pain management, patient-controlled analgesia, perioperative, respiratory depression


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