A Longitudinal Study of Atlanto-Dens Relationships in Asymptomatic Individuals with Down Syndrome

PEDIATRICS ◽  
1992 ◽  
Vol 89 (6) ◽  
pp. 1194-1198 ◽  
Author(s):  
Siegfried M. Pueschel ◽  
Francis H. Scola ◽  
John C. Pezzullo
Keyword(s):  
Down Syndrome ◽  
Clinical Symptoms ◽  
Age Groups ◽  
Atlantoaxial Instability ◽  
History Of ◽  
Child Development Center ◽  
Asymptomatic Individuals ◽  
Development Center ◽  
Over Time

This study was designed to investigate the natural history of atlantoaxial instability in individuals with Down syndrome and to determine whether significant changes in C1-C2 relationship are taking place over time. Although more than 400 patients with Down syndrome who are presently followed at the Child Development Center had cervical spine radiographic examinations in the past, only 141 patients who had serial radiological examinations and whose radiographs were available for reevaluation participated in this study. The results of our investigations revealed that there were only minor changes (1 to 1.5 mm) of atlanto-dens interval measurements over time in 130 (92%) patients with Down syndrome. Eleven patients (8%) had changes of atlanto-dens interval measurements between 2 and 4 mm over time; however, none of these patients had any clinical symptoms. The analyses of data obtained from several subgroups (males and females, various age groups, and patients with and without atlantoaxial instability) did not show any significant changes of atlanto-dens interval measurements of successively obtained radiographs. Our recommendations for and rationale of routine screening for atlantoaxial instability and follow-up examinations are discussed in detail.

CASE REPORT: RARE FORM OF HIRSCHPRUNG’S DISEASE

2020 ◽  
Vol 30 (5) ◽  
pp. 82-84
Author(s):  
Ilja Skalskis
Keyword(s):  
Down Syndrome ◽  
Case Report ◽  
Clinical Symptoms ◽  
Hirschsprung Disease ◽  
Distal Colon ◽  
Total Colonic Aganglionosis ◽  
Sphincter Function ◽  
Anal Sphincter Function ◽  
History Of ◽  
High Index Of Suspicion

Hirschsprung disease (HD) is a developmental disorder characterized by the absence of ganglia in the distal colon, resulting in a functional obstruction. Incidence of total colonic aganglionosis (TCA) is 1 in 500 000 and it accounts for 5-10% of all cases of HD. HD should be suspected in patients with typical clinical symptoms and a high index of suspicion is appropriate for infants with a predisposing condition such as Down Syndrome (DS), or for those with a family history of HD. The treatment of choice for HD is surgical, such as Swenson, Soave, and Duhamel procedures. The goals are to resect the affected segment of the colon, bring the normal ganglionic bowel down close to the anus, and preserve internal anal sphincter function. We present a clinical case report of TCA in a child with Down syndrome (DS) and review of literature.

Anterior approach levator plication for congenital ptosis, absorpable versus non absorpable sutures

2021 ◽  
pp. 112067212110053
Author(s):  
Moustafa Salamah ◽  
Ashraf Mahrous Eid ◽  
Hani Albialy ◽  
Sherif Sharaf EL Deen
Keyword(s):  
Randomized Study ◽  
Early Postoperative Period ◽  
Polyglactin 910 ◽  
Exclusion Criteria ◽  
Congenital Ptosis ◽  
History Of ◽  
Group A ◽  
Group B ◽  
Over Time

Purpose: To compare the efficacy of two different suture types in levator plication for correction of congenital ptosis. Subjects and methods: Prospective comparative interventional randomized study involving 42 eyes of 42 patients aged more than 6 years with congenital ptosis and good levator action. The exclusion criteria were as follows: bilateral ptosis, history of previous surgery, fair or poor levator action, and associated other ocular diseases. Patients were randomized into group A, in which double-armed 5/0 polyester Ethibond were used, and group B, in which double-armed 5/0 Coated Vicryl® (polyglactin 910) suture material we used. Outcomes including eyelid height and stability of eyelid height over time were compared with follow-up data. The MRD was 4.05 ± 0.36 mm and 3.95 ± 0.34 after 1 week for both groups A and B, respectively. At the end of study follow up period (24 weeks), the MRD was 3.60 ± 0.42 mm in group A, and 2.52 ± 0.85 mm in group B. Conclusion: No difference in eyelid height between two groups in early postoperative period, but the postoperative eyelid height was more stable over time in the 5/0 polyester Ethibond group (group A) than in the 5/0 Coated Vicryl® (polyglactin 910) group (group B).

scholarly journals Human Milk Antibodies Against SARS-CoV-2: A Longitudinal Follow-Up Study

2021 ◽  
pp. 089033442110301
Author(s):  
Hannah G. Juncker ◽  
M. Romijn ◽  
Veerle N. Loth ◽  
Tom G. Caniels ◽  
Christianne J.M. de Groot ◽  
...  
Keyword(s):  
Human Milk ◽  
Clinical Symptoms ◽  
Immunoglobulin A ◽  
Spike Protein ◽  
Enzyme Linked Immunosorbent Assay ◽  
Follow Up Study ◽  
Lactating Mothers ◽  
Milk Antibodies ◽  
Over Time

Background: Human milk contains antibodies against Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) following Coronavirus Disease 2019 (COVID-19). These antibodies may serve as protection against COVID-19 in infants. However, the evolution of these human milk antibodies over time is unclear. Research Aim: To elucidate the evolution of immunoglobulin A (IgA) against SARS-CoV-2 in human milk after a SARS-CoV-2 infection. Methods: This longitudinal follow-up study included lactating mothers ( N = 24) who had participated in the COVID MILK study. To assess the evolution of SARS-CoV-2 antibodies, serum and human milk samples were collected 14–143 days after the onset of clinical symptoms related to COVID-19. Enzyme-Linked ImmunoSorbent Assay was used to detect antibodies against the ectodomain of the SARS-CoV-2 spike protein. Results: SARS-CoV-2 antibodies remain present up to 5 months (143 days) in human milk after onset of COVID-19 symptoms. Overall, SARS-CoV-2 IgA in human milk seems to gradually decrease over time. Conclusion: Human milk from SARS-CoV-2 convalescent lactating mothers contains specific IgA antibodies against SARS-CoV-2 spike protein up to at least 5 months post-infection. Passive viral immunity can be transferred via human milk and may serve as protection for infants against COVID-19. Dutch Trial Register on May 1st, 2020, number: NL 8575, URL: https://www.trialregister.nl/trial/8575 .

Cochlear implantation in elderly patients: stability of outcome over time

2016 ◽  
Vol 130 (8) ◽  
pp. 706-711 ◽  
Author(s):  
O Hilly ◽  
E Hwang ◽  
L Smith ◽  
D Shipp ◽  
J M Nedzelski ◽  
...  
Keyword(s):  
Elderly Patients ◽  
Cochlear Implantation ◽  
Short Form ◽  
Age Groups ◽  
The Elderly ◽  
Long Term Results ◽  
Profound Hearing Loss ◽  
Younger Patients ◽  
Over Time

AbstractBackground:Cochlear implantation is the standard of care for treating severe to profound hearing loss in all age groups. There is limited data on long-term results in elderly implantees and the effect of ageing on outcomes. This study compared the stability of cochlear implantation outcome in elderly and younger patients.Methods:A retrospective chart review of cochlear implant patients with a minimum follow up of five years was conducted.Results:The study included 87 patients with a mean follow up of 6.8 years. Of these, 22 patients were older than 70 years at the time of implantation. Hearing in Noise Test scores at one year after implantation were worse in the elderly: 85.3 (aged under 61 years), 80.5 (61–70 years) and 73.6 (aged over 70 years;p= 0.039). The respective scores at the last follow up were 84.8, 85.1 and 76.5 (p= 0.054). Most patients had a stable outcome during follow up. Of the elderly patients, 13.6 per cent improved and none had a reduction in score of more than 20 per cent. Similar to younger patients, elderly patients had improved Short Form 36 Health Survey scores during follow up.Conclusion:Cochlear implantation improves both audiometric outcome and quality of life in elderly patients. These benefits are stable over time.

scholarly journals Chondrosternal Arthritis in Infant: An Unusual Entity

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Athina Nikolarakou ◽  
Dana Dumitriu ◽  
Pierre-Louis Docquier
Keyword(s):  
Clinical Symptoms ◽  
Therapeutic Option ◽  
White Blood Cells ◽  
Laboratory Data ◽  
C Reactive Protein ◽  
Reactive Protein ◽  
History Of ◽  
Local Tenderness ◽  
Wait And See

Primary arthritis of chondrosternal joint is very rare and occurs in infants less than 18 months of age. Presentation is most often subacute but may be acute. Child presents with a parasternal mass with history of fever and/or local signs of infection. Clinical symptoms vary from a painless noninflammatory to a painful mass with local tenderness and swelling, while fever may be absent. Laboratory data show low or marginally raised levels of white blood cells and C-reactive protein, reflecting, respectively, the subacute or acute character of the infection. It is a self-limiting affection due to the adequate immune response of the patient. Evolution is generally good without antibiotherapy with a progressive spontaneous healing. A wait-and-see approach with close follow-up in the first weeks is the best therapeutic option.

Indolent Neuroendocrine Tumor Involving the Bone Marrow

2003 ◽  
Vol 127 (4) ◽  
pp. 488-491
Author(s):  
Ellen Schlette ◽  
L. Jeffrey Medeiros ◽  
Miloslav Beran ◽  
Carlos E. Bueso-Ramos
Keyword(s):  
Bone Marrow ◽  
Neuroendocrine Tumor ◽  
Bone Marrow Biopsy ◽  
Clinical Symptoms ◽  
Eosinophilic Cytoplasm ◽  
Hydroxyindoleacetic Acid ◽  
History Of ◽  
Cardiac Valve Disease ◽  
Immunohistochemical Studies

Abstract We report a unique case of a patient with a neuroendocrine tumor localized to the bone marrow. The patient had a history of hairy cell leukemia, and the neuroendocrine tumor was detected in a bone marrow biopsy specimen obtained to assess response to 2-chlorodeoxyadenosine therapy. The neuroendocrine tumor was present as nodules that replaced approximately 15% of the bone marrow medullary space and was composed of round cells with fine chromatin, indistinct nucleoli, and relatively abundant, granular, eosinophilic cytoplasm. Histochemical stains showed cytoplasmic reactivity with Grimelius and Fontana-Masson stains, and immunohistochemical studies showed positivity for keratin and chromogranin. The histologic, cytochemical, and immunohistochemical features resembled a carcinoid tumor, and metastasis to the bone marrow was considered initially. The patient was asymptomatic without diarrhea, flushing, or cardiac valve disease. Serotonin production, assessed by the measurement of serum 5-hydroxyindoleacetic acid and substance P levels, was normal. Extensive clinical and radiologic work-up and endoscopy of the gastrointestinal tract to detect a primary site other than the bone marrow were negative. Follow-up bone marrow biopsy 7 years after the initial diagnosis was positive for persistent neuroendocrine tumor. The patient has not received any therapy specific for the neuroendocrine tumor and has had no clinical symptoms or evidence of progression after 9 years of clinical follow-up. We suggest that this neuroendocrine tumor may have arisen in the bone marrow.

Adult Victimization over Time

2018 ◽  
pp. 171-174
Author(s):  
Mary C. Zanarini
Keyword(s):  
Risk Factors ◽  
Sexually Abused ◽  
Female Gender ◽  
Physical Assault ◽  
Clinical Implications ◽  
Axis Ii ◽  
Borderline Patients ◽  
History Of ◽  
Over Time

At baseline, borderline patients reported higher rates of adult rape and physical assault by a partner than Axis II comparison subjects. Four risk factors were found to significantly predict whether borderline patients had an adult history of being a victim of physical and/or sexual violence before their index admission: female gender, a substance use disorder that began before the age of 18, childhood sexual abuse, and emotional withdrawal by a caretaker (a form of neglect). At six-year and 10-year follow-up, borderline patients reported higher rates of being verbally, emotionally, physically, and sexually abused or assaulted than did Axis II comparison subjects. However, each of these forms of abuse declined significantly over time. The clinical implications of these prevalence and predictive findings are discussed.

scholarly journals Spontaneous dyskinesia and parkinsonism in never-medicated, chronically ill patients with schizophrenia: 18-month follow-up

2002 ◽  
Vol 181 (2) ◽  
pp. 135-137 ◽  
Author(s):  
R. G. McCreadie ◽  
R. Padmavati ◽  
R. Thara ◽  
T. N. Srinivasan
Keyword(s):  
Natural History ◽  
Disease Process ◽  
Chronically Ill ◽  
Abnormal Involuntary Movements ◽  
Syndrome Scale ◽  
History Of ◽  
Chronically Ill Patients ◽  
Negative Syndrome ◽  
Over Time

BackgroundSpontaneous dyskinesia and parkinsonism have been reported in never-medicated patients with schizophrenia but there has been no previous study of the natural history of these conditions.AimsTo determine the prevalence of spontaneous dyskinesia and parkinsonism in a group of never-medicated, chronically ill patients with schizophrenia on two occasions separated by an 18-month interval.MethodDyskinesia was assessed by the Abnormal Involuntary Movements Scale using Schooler and Kane criteria for its presence; parkinsonism by the Simpson and Angus scale; and mental state by the Positive and Negative Syndrome Scale for schizophrenia.ResultsThirty-seven patients were examined on two occasions. Nine (24%) had dyskinesia on both occasions, 12 (33%) on one occasion and 16 (43%) on neither occasion. Twenty-one (57%) had dyskinesia on at least one occasion. Thirteen patients (35%) had parkinsonism on at least one occasion.ConclusionsSpontaneous dyskinesia and parkinsonism fluctuate over time. The former was found on at least one occasion in the majority of patients. It is an integral part ofthe schizophrenic disease process.

scholarly journals Prospective evaluation of autoimmune and non-autoimmune subclinical hypothyroidism in Down syndrome children

2020 ◽  
Vol 182 (4) ◽  
pp. 385-392 ◽  
Author(s):  
Giorgia Pepe ◽  
Domenico Corica ◽  
Luisa De Sanctis ◽  
Mariacarolina Salerno ◽  
Maria Felicia Faienza ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Subclinical Hypothyroidism ◽  
Heart Defects ◽  
Thyroid Autoimmunity ◽  
Overt Hypothyroidism ◽  
Sh Group ◽  
History Of ◽  
Group A ◽  
Group B

Objective To evaluate the prevalence and natural course of autoimmune and non-autoimmune subclinical hypothyroidism (SH) in Down syndrome (DS) children and adolescents. Design Prospective multicenter study. Methods For the study, 101 DS patients with SH (TSH 5–10 mIU/L; FT4 12–22 pmol/L), aged 2–17 years at SH diagnosis were enrolled. Annual monitoring of TSH, FT4, BMI, height, and L-thyroxine dose was recorded for 5 years. Thyroid autoimmunity was tested at diagnosis and at the end of follow-up. Results Thirty-seven out of 101 patients displayed autoantibody positivity (group A); the remaining 64 were classified as non-autoimmune SH (group B). Group A was characterized by higher median age at SH diagnosis and by more frequent family history of thyroid disease (6.6 vs 4.7 years, P = 0.001; 32.4% vs 7.8%, P = 0.001 respectively), whereas congenital heart defects were more common in group B (65.6% vs 43.2%, P = 0.028). Gender, median BMI (SDS), height (SDS), FT4, and TSH were similar in both groups. At the end of follow-up: 35.1% of group A patients developed overt hypothyroidism (OH) vs 17.2% of group B (P = 0.041); 31.25% of group B vs 10.8% of group A became biochemically euthyroid (P = 0.02); and 37.8% of group A vs 51.5% of group B still had SH condition (P = 0.183). Logistic regression suggested autoimmunity (OR = 3.2) and baseline TSH values (OR = 1.13) as predictive factors of the evolution from SH to OH. Conclusions In DS children, non-autoimmune SH showed higher prevalence and earlier onset. The risk of thyroid function deterioration over time seems to be influenced by thyroid autoimmunity and higher baseline TSH values.

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