What is DIOS?

Discussion Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane receptor (CFTR). It is found in the epithelium of the bronchi, intestine, pancreatic duct and biliary tree. It regulates chloride, bicarbonate and water secretion. The heterozygous state helps prevent against secretory diarrhea, but the homozygous state causes thickened secretions in the hollow tubes of the lungs and digestive tract. There are multiple mutations (> 2000) which have been currently classified into classes depending on their protein production and activity. CF patients generally are living longer with improved morbidity because of new treatments including lung transplantation but they also are at higher risk of having gastrointestinal problems over their lifespan. Gastrointestinal problems in patients with CF include: Pancreas Second most frequent organ affected after the lung Concentrated pancreatic juice and thickened secretions can increase obstruction leading to acute or chronic injury and inflammation Pancreatic insufficiency Most (~90%) patients have exocrine pancreatic insufficiency Greasy stools, abdominal bloating, flatulence, diarrhea and poor weight gain are symptoms Treatment is pancreatic enzyme replacement therapy (PERT), proton pump inhibitors are used sometimes also Fat soluble vitamins (A, D, E, and K) can occur and usually are also supplemented Poor nutrition Patients have increased nutrition needs because of the ex...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news

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His group will study how abnormal calcium signals may contribute to human diseases such as allergies and asthma. (read more)
Source: Environmental Factor - NIEHS Newsletter - Category: Environmental Health Source Type: news
Neonatal brachial plexus palsy (NBPP) is a birth injury that can cause severe functional loss in the affected limb. The purpose of this study was to determine the temporal changes in the national incidence of this condition and whether associated risk factors have changed over time. Children born via vaginal delivery were identified in the Kids’ Inpatient Database (KID) from 1997 to 2012, and those with NBPP were identified. The trend in incidence and risk factors were assessed through the study period. The nationwide incidence of NBPP decreased during the study period. Infants with shoulder dystocia, fetal macrosomi...
Source: Journal of Pediatric Orthopaedics B - Category: Orthopaedics Tags: Upper Limb Source Type: research
Defects occurring in the femoral–fibular–ulnar developmental field are believed to cause the cluster of anomalies seen with femoral, fibular and ulnar limb deficiencies. Upper limb function must be considered in the management of lower limb deficiencies. The purpose of this study is to determine the frequency and type of upper extremity anomalies found in children with femoral and/or fibular deficiency. A retrospective review of 327 consecutive patients with the diagnosis of femoral and/or fibular deficiency was performed using existing records and radiographs. Characteristics of those with and without upper ex...
Source: Journal of Pediatric Orthopaedics B - Category: Orthopaedics Tags: Upper Limb Source Type: research
This study proposed a classification system and a possible mechanism for stress fractures of the first rib using three-dimensional computed tomography (3D-CT). The data of 10 stress fractures of the first rib in adolescent athletes obtained using 3D-CT between 2007 and 2018 were reviewed. Fractures of the first rib were classified according to the location and type of the fracture line. Stress fractures of the first rib were classified into three types: type 1, center-transverse fracture; type 2, center to posterior-oblique fracture; and type 3, posterior-transverse fracture. There were three type 1 fractures, five type 2 ...
Source: Journal of Pediatric Orthopaedics B - Category: Orthopaedics Tags: Upper Limb Source Type: research
The Langenskiöld classification is the most commonly utilized classification system for the radiological features of Blount’s disease. Although there is only a single study found on the interobserver variability and none found on the intraobserver variability, it is commonly used for prognostication and guiding management decisions. The aim of this study was to determine the reliability and reproducibility of the Langenskiöld classification. A retrospective review of radiographs was done of patients treated for infantile and juvenile Blount’s disease at Chris Hani Baragwanath Academic Hospital from 20...
Source: Journal of Pediatric Orthopaedics B - Category: Orthopaedics Tags: Leg & Foot Source Type: research
The objective of this study is to provide an overview of the available hypotheses on the aetiology of Blount’s disease since its first description and assessment of the available level of evidence, the quality of evidence and the occurrence of bias supporting these individual hypotheses. A systematic search according to the PRISMA statement was conducted using PubMed, MEDLINE, EMBASE and the Cochrane Library using a broad combination of terminology to ascertain a complete selection. Proper MESH search criteria were formulated and the bibliographic search was limited to English and Dutch language articles. Articles wi...
Source: Journal of Pediatric Orthopaedics B - Category: Orthopaedics Tags: Leg & Foot Source Type: research
Estimating the appropriate tendon length and associated skin incision needed to achieve a plantigrade foot without compromising function are essential steps in open Achilles tendon lengthening. Here we describe a technique using basic surgical instruments referencing anatomic landmarks without the need for radiographs. The center of ankle rotation in the sagittal plane is found referencing the tip of the medial malleolus. This point is translated to the plantar surface of the foot, and a straight instrument in line with the foot is used to make a mark at the posterior calcaneal fat pad. The straight instrument is then rota...
Source: Journal of Pediatric Orthopaedics B - Category: Orthopaedics Tags: Leg & Foot Source Type: research
Recent literature suggests that adults do not necessarily develop osteoarthritis if they have an osteochondral lesion of the talus (OLT). The purpose of this study was to determine if children with OLT are at risk to develop any radiographic evidence of early joint degeneration despite treatment efforts. Fifty-six ankles were identified over 6 years with stable OLT that underwent operative treatment. Preoperatively, each lesion was classified according to Berndt-Hardy for radiographs, Hepple for MRI, and Ferkel and Sgaglione for computed tomography. Radiographs were also evaluated for physeal status, OLT characteristics (l...
Source: Journal of Pediatric Orthopaedics B - Category: Orthopaedics Tags: Leg & Foot Source Type: research
Publication date: Available online 31 May 2020Source: Obstetrics and Gynecology Clinics of North AmericaAuthor(s): Sydney M. Thayer, Jamie O. Lo, Aaron B. Caughey
Source: Obstetrics and Gynecology Clinics of North America - Category: OBGYN Source Type: research
Conclusion: This study showed positive association between depression and IL-1 β , IL-9 in T2DM patients. Additionally, the diabetic patients have poorer quality of life, which is further worsened by the presence of depression. Thus, routine assessment for the presence of depression is suggested in T2DM patients.RESUMO Introdu ção: O diabetes e a depressão comórbidas são condições crônicas prevalentes que afetam negativamente a qualidade de vida (QdV). A inflamação tem sido considerada como um mecanismo integral em pacientes com diabetes e depress&at...
Source: Arquivos de Neuro-Psiquiatria - Category: Neurology Source Type: research
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