No effect of patent foramen ovale on acute mountain sickness and pulmonary pressure in normobaric hypoxia.

NEW FINDINGS: What is the central question to this study? Is there a relationship between a patent foramen ovale and the development of acute mountain sickness and an exaggerated increase in pulmonary pressure in response to 7-10 h of normobaric hypoxia? What is the main finding and its importance? Patent foramen ovale presence did not increase susceptibility to acute mountain sickness or result in an exaggerated increase in pulmonary artery systolic pressure with normobaric hypoxia. This suggests hypobaric hypoxia is integral to the increased susceptibility to acute mountain sickness previously reported in those with patent foramen ovale, and patent foramen ovale presence alone does not contribute to the hypoxic pulmonary pressor response. ABSTRACT: Acute mountain sickness (AMS) develops following rapid ascent to altitude, but its exact causes remain unknown. A patent foramen ovale (PFO) is a right-to-left intracardiac shunt present in ∼30% of the population that has been shown to increase AMS susceptibility with high altitude hypoxia. Additionally, high altitude pulmonary oedema (HAPE) is a severe type of altitude illness characterized by an exaggerated pulmonary pressure response, and there is a greater prevalence of PFO in those with a history of HAPE. However, whether hypoxia per se is causing the increased incidence of AMS in those with a PFO and whether a PFO is associated with an exaggerated increase in pulmonary pressure in those without a history of HAPE is unknown. Participants (n = 36) matched for biological sex (18 female) and the presence or absence of a PFO (18 PFO+) were exposed to 7-10 h of normobaric hypoxia equivalent to 4755 m. Presence and severity of AMS was determined using the Lake Louise AMS scoring system. Pulmonary artery systolic pressure, cardiac output and total pulmonary resistance were measured using ultrasound. We found no significant association of PFO with incidence or severity of AMS and no association of PFO with arterial oxygen saturation. Additionally, there was no effect of a PFO on pulmonary pressure, cardiac output or total pulmonary resistance. These data suggest that hypobaric hypoxia is necessary for those with a PFO to have increased incidence of AMS and that presence of PFO is not associated with an exaggerated pulmonary pressor response.

Child Protective Services Referral in a Cleft Lip and/or Palate Population: Assessment of Prevalence, Indications, and Outcomes.

OBJECTIVE: To determine the prevalence of, reasons for, and outcomes related to Child Protective Services (CPS) referral in an isolated and syndromic cleft lip/palate population. DESIGN: Retrospective cohort study. SETTING: Tertiary Children's Hospital. PATIENTS: Any patient <18 years of age attending the multidisciplinary cleft team for care at our institution with a history of referral to CPS by the cleft team during the study period 2009 to 2014. MAIN OUTCOME MEASURES: The number of children with CPS referrals, reasons for CPS referrals, outcomes of CPS referrals, associated psychosocial risk factors potentially predictive of CPS referral; demographics and cleft-related surgical history was also reviewed for each patient. RESULTS: Of 1392 patients, 25 (1.8%) were identified with a history of referral to CPS. Average age at referral was 11 months; 76.0% of patients were <1 year of age. Most referrals (64.0%) were directly associated with issues related to cleft care. Identified psychosocial risk factors included financial strain, mental illness/cognitive disability, transportation issues, and inadequate social support. Nine families ultimately lost custody of their children temporarily (n = 5) or permanently (n = 4). CONCLUSIONS: Cleft team family referral to CPS involves long-term patient care challenges requiring maximal medical and social support. Families are most commonly referred for issues related to medical neglect, which can lead to failure to thrive, delays in care, and ultimate removal from the home. Identifying families with known psychosocial risk factors and providing increased support may potentially help avoid referrals to CPS.

Malignant Granular Cell Tumor of the Lower Extremity in an Adolescent Male.

Granular cell tumors (GCTs) are extremely rare soft tissue tumors, with only 2% of tumors being malignant. Malignant GCTs are more often seen in women between the ages 40 and 60. There has been no case reported of a malignant GCT in a pediatric patient. We present a case of a 14-year-old male who presented with a large mass in his left lower extremity. After being biopsied, the mass was diagnosed as a malignant GCT. The tumor was completely excised with wide margins and close follow-up with the patient continued.

Roles of metabotropic glutamate receptor 8 in neuropsychiatric and neurological disorders.

Metabotropic glutamate (mGlu) receptors are G protein-coupled receptors. Among eight mGlu subtypes (mGlu1-8), mGlu8 has drawn increasing attention. This subtype is localized to the presynaptic active zone of neurotransmitter release and is among the mGlu subtypes with high affinity for glutamate. As a Gi/o-coupled autoreceptor, mGlu8 inhibits glutamate release to maintain homeostasis of glutamatergic transmission. mGlu8 receptors are expressed in limbic brain regions and play a pivotal role in modulating motivation, emotion, cognition, and motor functions. Emerging evidence emphasizes the increasing clinical relevance of abnormal mGlu8 activity. Studies using mGlu8 selective agents and knockout mice have revealed the linkage of mGlu8 receptors to multiple neuropsychiatric and neurological disorders, including anxiety, epilepsy, Parkinson's disease, drug addiction, and chronic pain. Expression and function of mGlu8 receptors in some limbic structures undergo long-lasting adaptive changes in animal models of these disorders, which may contribute to the remodeling of glutamatergic transmission critical for the pathogenesis and symptomatology of brain illnesses. This review summarizes the current understanding of mGlu8 biology and the possible involvement of the receptor in several common psychiatric and neurological disorders.

Prosthetic temporomandibular joint reconstruction in a cohort of adolescent females with juvenile idiopathic arthritis.

BACKGROUND: Temporomandibular joint (TMJ) arthritis and involvement is commonly seen in Juvenile Idiopathic Arthritis (JIA). Therapy includes conservative measures, but also includes intraarticular corticosteroid injections (IASI) and systemic immunosuppressive therapy. Despite aggressive medical therapy, some patients develop arthritic changes and frank TMJ ankylosis that can result in persistent pain and limitation in range of motion (ROM). A surgical option is prosthetic TMJ replacement with concurrent correction of dentofacial deformities, which can be performed simultaneously. The objective of this study was to evaluate the outcomes of prosthetic TMJ replacement in a cohort of adolescent females with JIA and severe TMJ involvement. METHODS: This is a retrospective case series that took place at one tertiary care center. Patients with a diagnosis of JIA who also underwent alloplastic TMJ replacement were identified through electronic medical record system (EMR) and reviewed. Chart review included analysis of all documents in the EMR, including demographic data, JIA history, surgical complications, ROM of TMJ measured by maximal incisal opening in millimeters (mm) and TMJ pain scores (4-point Likert scale: none, mild, moderate, severe) obtained pre- and postoperatively. RESULTS: Five female patients, ages 15-17 year when TMJ replacement was performed, had nine total joints replaced with a post-operative follow-up period of 12-30 months. All patients had polyarticular, seronegative JIA and were treated with IASI and multiple immunosuppressive therapies without resolution of TMJ symptoms. One patient had bilateral TMJ ankylosis. Three of the five patients demonstrated significant dentofacial deformities, and all underwent simultaneous or staged orthognathic surgery. All patients had improvement in TMJ pain with most (80%) reporting no pain, and all had similar or improved ROM of their TMJ postoperatively. There was one delayed postoperative infection with Cutibacterium Acnes that presented 15 months after surgery and required removal and reimplantation of prosthesis. CONCLUSION: The sequelae of TMJ arthritis and involvement from JIA in the adolescent population can be difficult to treat. Current medical therapy can be successful, however, in select cases that develop chronic changes in the TMJ despite extensive medical therapy, early results show that prosthetic joint replacement maybe a reasonable surgical option. With prosthetic joint replacement pain levels were reduced and range of motion was maintained or improved for all patients.