scholarly journals Pediatric Hereditary Angioedema as a Cause of Acute Compartment Syndrome of the Hand and Forearm: A Case Report

Hand ◽  
2016 ◽  
Vol 12 (3) ◽  
pp. NP46-NP50
Author(s):  
Chelsea Venditto ◽  
Zachary Jager ◽  
John LoGiudice ◽  
Hani Matloub
Keyword(s):  
Compartment Syndrome ◽  
Upper Extremity ◽  
Hereditary Angioedema ◽  
Autosomal Dominant ◽  
Unknown Etiology ◽  
Laboratory Studies ◽  
Patient Diagnosis ◽  
Laboratory Results ◽  
Dominant Disease ◽  
Forearm Compartment Syndrome

Background: Compartment syndrome of the upper extremity is a surgical emergency that, when left untreated, can have dire consequences. Its causes are numerous, one of which is the uncommon entity hereditary angioedema, an autosomal dominant disease resulting in edema in a variety of potential locations, including the extremities. This is only the second time hereditary angioedema has been mentioned in the literature as a cause of compartment syndrome. Methods: We present a case of hereditary angioedema leading to hand and forearm compartment syndrome in a 13-year-old pediatric patient. Diagnosis of hereditary angioedema was made by our Rheumatology colleagues with physical exam and a thorough history, and confirmed by laboratory studies. Results: Our patient presented with compartment syndrome of the hand and forearm and underwent hand and volar forearm fasciotomies. She was subsequently worked up for hereditary angioedema with laboratory results confirming the diagnosis. She was discharged after a 5-day hospitalization with prophylactic C1-inhibitor therapy. Conclusions: Hereditary angioedema is a rare but known cause of compartment syndrome of the upper extremity, and must be considered when patients present with compartment syndrome of unknown etiology. This disease can be diagnosed by laboratory studies and symptoms can be controlled with medical therapy.

Starch-modified fillers for linerboard and paper grades: A perspective review

TAPPI Journal ◽  
2010 ◽  
Vol 9 (4) ◽  
pp. 31-36 ◽  
Author(s):  
YULIN DENG ◽  
PHIL JONES ◽  
LESLIE MCLAIN ◽  
ART J. RAGAUSKAS
Keyword(s):  
Filler Content ◽  
Product Platform ◽  
Laboratory Studies ◽  
The Past ◽  
Development Opportunity ◽  
Laboratory Results ◽  
Addition Rate ◽  
Institute Of Technology ◽  
Paper Product ◽  
Detrimental Impact

High-filler-content paper is a growing research and development opportunity in papermaking. These new products must address traditional paper product properties while providing papermakers with distinct product platform benefits. Over the past decade, a research team involving researchers from the Institute of Paper Science and Technology at Georgia Institute of Technology and from Imerys have significantly advanced the application of starch-encapsulated papermaking fillers. This review summarizes these accomplishments from initial laboratory studies to mill trials. Laboratory results have illustrated that starch-encapsulated fillers can facilitate a near-doubling of filler content over conventional levels at equal tensile and z-direction tensile (ZDT) values. Equally important is that the use of starch-encapsulated kaolin (SEK) filler has been shown to facilitate a doubling of filler addition rate without any detrimental impact on ring crush compared with control studies with filler. Pilot-plant and mill trials have shown that SEK can function as a fiber extender, reduce steam demand for drying by 10%, and increase papermaking speeds and production rates.

scholarly journals A family harboring an MLKL loss of function variant implicates impaired necroptosis in diabetes

2021 ◽  
Vol 12 (4) ◽  
Author(s):  
Joanne M. Hildebrand ◽  
Bernice Lo ◽  
Sara Tomei ◽  
Valentina Mattei ◽  
Samuel N. Young ◽  
...  
Keyword(s):  
Potential Role ◽  
Autosomal Dominant ◽  
Inflammatory Diseases ◽  
Diabetic Patients ◽  
Incomplete Penetrance ◽  
Loss Of Function ◽  
Healthy Sibling ◽  
Dominant Disease ◽  
Terminal Effector

AbstractMaturity-onset diabetes of the young, MODY, is an autosomal dominant disease with incomplete penetrance. In a family with multiple generations of diabetes and several early onset diabetic siblings, we found the previously reported P33T PDX1 damaging mutation. Interestingly, this substitution was also present in a healthy sibling. In contrast, a second very rare heterozygous damaging mutation in the necroptosis terminal effector, MLKL, was found exclusively in the diabetic family members. Aberrant cell death by necroptosis is a cause of inflammatory diseases and has been widely implicated in human pathologies, but has not yet been attributed functions in diabetes. Here, we report that the MLKL substitution observed in diabetic patients, G316D, results in diminished phosphorylation by its upstream activator, the RIPK3 kinase, and no capacity to reconstitute necroptosis in two distinct MLKL−/− human cell lines. This MLKL mutation may act as a modifier to the P33T PDX1 mutation, and points to a potential role of impairment of necroptosis in diabetes. Our findings highlight the importance of family studies in unraveling MODY’s incomplete penetrance, and provide further support for the involvement of dysregulated necroptosis in human disease.

scholarly journals Neonate born with ischemic limb to a COVID-19 positive mother: management and review of literature

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Shahana Perveen ◽  
Karmaine A. Millington ◽  
Suchitra Acharya ◽  
Amit Grag ◽  
Vita Boyar
Keyword(s):  
Differential Diagnosis ◽  
Compartment Syndrome ◽  
Upper Extremity ◽  
Preterm Neonate ◽  
Review Of Literature ◽  
Case Presentation ◽  
Ischemic Limb ◽  
History Of ◽  
Hand Amputation ◽  
Work Up

AbstractObjectivesTo describe challenges in diagnosis and treatment of congenital neonatal gangrene lesions associated with history of maternal coronavirus disease 2019 (COVID-19) infection.Case presentationA preterm neonate was born with upper extremity necrotic lesions and a history of active maternal COVID-19 infection. The etiology of his injury was challenging to deduce, despite extensive hypercoagulability work-up and biopsy of the lesion. Management, including partial forearm salvage and hand amputation is described.ConclusionsNeonatal gangrene has various etiologies, including compartment syndrome and intrauterine thromboembolic phenomena. Maternal COVID-19 can cause intrauterine thrombotic events and need to be considered in a differential diagnosis.

scholarly journals A novel TSC1 variant associated with tuberous sclerosis and sacrococcygeal teratoma

2020 ◽  
Vol 7 (1) ◽  
Author(s):  
Saba Ahmad ◽  
Luis Manon ◽  
Gifty Bhat ◽  
Jerry Machado ◽  
Alice Zalan ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Cellular Differentiation ◽  
Autosomal Dominant ◽  
De Novo ◽  
Autosomal Dominant Disease ◽  
Sacrococcygeal Teratoma ◽  
Dominant Disease ◽  
The Brain

AbstractTuberous sclerosis complex (TSC) is an autosomal dominant disease associated with tumors and malformed tissues in the brain and other vital organs. We report a novel de novo frameshift variant of the TSC1 gene (c.434dup;p. Ser146Valfs*8) in a child with TSC who initially presented with a sacral teratoma. This previously unreported association between TSC and teratoma has broad implications for the pathophysiology of embryonic tumors and mechanisms underlying cellular differentiation.

Laboratory and field measurements of HONO emissions from agricultural soils in Guangdong of China 

2021 ◽  
Author(s):  
Baobin Han ◽  
Peng Cheng ◽  
Yihang Yu ◽  
Wenda Yang ◽  
Zhilin Tian ◽  
...  
Keyword(s):  
Emission Rate ◽  
Agricultural Soils ◽  
Pore Space ◽  
Primary Source ◽  
Field Measurements ◽  
Laboratory Studies ◽  
Release Rates ◽  
Laboratory Results ◽  
Flux Measurements ◽  
Soil Flux

<p>Laboratory studies indicated that soil could produce considerable nitrous acid (HONO) emissions, which is the main primary source of hydroxyl radical (OH) in the troposphere. However, very few field observations of HONO emission from soil were reported. In order to relate laboratory results and field measurements, we measured HONO emissions from 7 representative agricultural soils (rice, vegetables, orchards, peanuts, potatoes, sugarcane and maize) in Guangdong under controlled laboratory conditions, and took flux measurements on 2 of them (rice and vegetables) by dynamic chambers in the field. Generally, release rates of HONO from the seven soils increased with temperature and varied with soil moisture, and the optimum release rates can be reached under specific values of water-filled pore space (WFPS), which is considered to be beneficial to nitrification. The seven soils' optimum release rates ranged from 1.24 to 43.19 ng kg<sup>-1</sup> s<sup>-1</sup>, and the Q<sub>10</sub> (It is defined as the multiple of the increase of soil gas emission rate when the temperature increases by 10℃) ranged from 1.03 to 2.25. Formulas were deduced from the lab results to express HONO emissions for every soil. Flux measurements on two soils varied around -1 to 4 ng N m<sup>-2</sup> s<sup>-1</sup>, and both showed similar diurnal variations with peaks around noontime and very low even negative values during nighttime. There were good correlations between HONO fluxes and soil temperature (R<sup>2</sup>=0.5). Furthermore, irrigation enhanced the HONO emission substantially. However, a large discrepancy existed between soil HONO emissions measured in lab and low HONO fluxes in field. More investigations are needed to explain the paradox.</p>

HEREDITARY ANGIOEDEMA – A PROBLEM AT THE INTERSECTION OF IMMUNOLOGY AND ALLERGOLOGY: ANALYSIS OF LITERATURE DATA AND DESCRIPTION OF A SERIES OF 34 CASES

2021 ◽  
Vol 100 (2) ◽  
pp. 49-57
Author(s):  
M.N. Guseva ◽  
◽  
E.I. Zinina ◽  
E.N. Suspitsyn ◽  
M.M. Kostik ◽  
...  
Keyword(s):  
Respiratory Tract ◽  
Hereditary Angioedema ◽  
Positive Family History ◽  
Clinical Manifestations ◽  
Upper Respiratory Tract ◽  
Upper Airways ◽  
Peripheral Edema ◽  
Dominant Disease ◽  
Characteristic Features ◽  
Head Neck

Hereditary angioedema (HAE) is a rare autosomal dominant disease characterized by isolated recurrent angioedema (AE) of various localizations: skin, the submucosa in the gastrointestinale tract, the respiratory tract. The characteristic features of edema in HAE are the absence of itching, skin flushing, accompanying urticaria, as well as the absence of pronounced effect of systemic corticosteroid therapy, systemic antihistamines therapy and adrenaline. The aim of our study was to present a descriptive characteristics of the group of patients with HAE and to access efficacy and safety of treatment. Materials and methods: the retrospective multicenter continuous nonrandomized uncontrolled study included data on 34 patients: 19 men (56%) and 15 women (44%) with HAE. The diagnosis of HAE was based on clinical and laboratorial data, according to the clinical guidelines of the Russian Association of allergologists and clinical immunologists on HAE (2014). Results The age range of patients at the time of inclusion in the study was 5–82 years, median – 38,8 (20,6; 46,9) years. Median age of first HAE episode was 12,5 (5,8; 16,3) years, ranged from 2 to 40, 22/28 (79%) of the patients manifested before 18 years. HAE was diagnosed at the median age of 30 years (17,5; 44,8) years, in 14,0 (9,0; 25,0) years after onset of the clinical manifestations. Most of the observations described have a positive family history of HAE (91%), in total 9 families were described. In 67% of families the disease manifested itself in two generations and in 33% of families – in three generations. At the onset of HAE peripheral edema was in 29/31 (94%) of the patients, and 22/31 (71%) had edema confined to the distal extremities. 7/31 (29%) patients had peripheral edema combined with edema of other locations. Abdominal attacks were in 6/31 (19%), head, neck and upper airways edema were in 7/31 (23%). The frequency of swelling of head, neck, upper respiratory tract increased statistically significantly from 23% (7/31) to 35% (7/20, p=0,003). 24 patients have recieved Icatibant treatment. No deaths were recorded against the background of this treatment. Conclusions HAE is diagnosed with significant time delays. Icatibant treatment is safe and effective.

OBSTRUCTIVE JAUNDICE IN INFANTS WITH NORMAL BILIARY TREE

PEDIATRICS ◽  
1954 ◽  
Vol 13 (4) ◽  
pp. 293-307
Author(s):  
RUTH C. HARRIS ◽  
DOROTHY H. ANDERSEN ◽  
RICHARD L. DAY
Keyword(s):  
Biliary Tract ◽  
Zinc Sulfate ◽  
Serum Bilirubin ◽  
Biliary Tree ◽  
Liver Cells ◽  
Unknown Etiology ◽  
Cholesterol Esters ◽  
Laboratory Studies ◽  
Bile Duct Proliferation ◽  
Information Exploration

Of 50 infants with persistent signs of biliary obstruction, not atresia, seen in the past 7 years, 16 had preceding hemolytic disease from ABO or Rh-incompatibility and 3 had acquired hemolytic anemia. One infant had probable serum hepatitis and 30 others had hepatitis of unknown etiology. All had a patent external biliary tract. Clinically these patients had jaundice, light stools and dark urine. All appeared in a good state of nutrition except those with hepatitis of unknown cause. The results of laboratory studies have been similar in these patients and have included a high direct serum bilirubin, usually negative cephalin flocculation, frequently elevated zinc sulfate turbidity and low cholesterol esters. The urine urobilin may be absent or increased in amount. Histologic studies of liver have shown the presence of multinucleated giant liver cells, much myelopoiesis and erythropoiesis, plugging of bile canaliculi and mild bile duct proliferation. Large amounts of yellow pigment have been found within the liver cells but this pigment has not taken the usual iron stains. Livers from patients with hepatitis of unknown etiology have shown more evidence of inflammation, cell necrosis and portal fibrosis than the others. Pertinent laboratory studies to help differentiate these cases from biliary atresia have included determinations of serum bilirubin, zinc sulfate turbidity, cholesterol and cholesterol esters and urine urobilin. Studies of maternal and infant blood factors and search for evidence of agglutination and increased production of erythrocytes are also important. Tests not found to be useful in differentiation are cholesterol esterase, alkaline phosphatase and prothrombin time. If diagnosis is impossible from clinical and laboratory information, exploration of the biliary tract with liver biopsy and diodrast cholangiography may be necessary. This procedure should be delayed until an adequate period of observation has elapsed and until the infant is over 4 months of age. Such a delay may result in spontaneous clearing of the jaundice with complete recovery.

Insidious Onset Compartment Syndrome of the Forearm in a Teenager: A Case Report and Review of the Literature

2021 ◽  
Vol 26 (03) ◽  
pp. 481-484
Author(s):  
Hidetoshi Iwata ◽  
Hideki Okamoto ◽  
Yohei Kawaguchi ◽  
Kojiro Endo ◽  
Yuji Joyo ◽  
...  
Keyword(s):  
Compartment Syndrome ◽  
Single Case ◽  
Case Reports ◽  
Upper Extremities ◽  
Review Of The Literature ◽  
Right Hand ◽  
Insidious Onset ◽  
Forearm Compartment Syndrome ◽  
Cash Register ◽  
Proximal Forearm

Compartment syndrome affecting the upper extremities is a relatively underreported event compared with compartment syndrome affecting the lower extremities. Moreover, insidious onset forearm compartment syndrome has been rarely reported and is usually limited to single case reports. We report a compartment syndrome of the forearm in a teenager. She hit her right proximal forearm lightly on the cash register, but there was no pain. However, the next day, she had difficulty in moving her right hand. Although she underwent electrotherapy, her right forearm gradually became swollen, and she felt numbness in the ring and little fingers of her right hand. Six day after the onset, she came to our hospital and underwent fasciotomy. There was no aftereffect, and very good functional recovery was obtained. All clinicians need to keep the case of forearm compartment syndrome in a young individual with a diffuse course, such as in this case in mind.

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