P.4.7 The natural history of myotubular myopathy, summary of the first year of enrollment

Abstract BACKGROUND Despite reducing mortality, the use of intravenous (IV) corticosteroids has not significantly reduced inpatient colectomy rates among hospitalized patients with ulcerative colitis (UC). Whether the occurrence of colectomy, estimated at 20–30% in this population in the prebiologic era, remains elevated is unknown. Methods A retrospective cohort study was conducted among health plan members of an integrated health care delivery organization that serves approximately 4.0 million members in urban, suburban, and semi-rural regions throughout Northern California. The study population consisted of 524 health plan members age >18 years of age with a 1) diagnosis of ulcerative colitis (ICD-9 code 556; ICD-10 code K51); 2) first hospitalization between 2009 and 2017 coded with ulcerative colitis as the primary cause for admission; 3) no prior hospitalizations for UC; 4) at least 1 year of continuous membership prior to the index hospitalization; 5) No dispensing of biologic therapy (infliximab, adalimumab, golimumab, vedolizumab) recorded prior to hospitalization; 6) No record of Clostridium difficile or cytomegalovirus infection during the hospitalization or 30 days prior. The primary outcome was the occurrence of colectomy during the index hospitalization and up to 12 months after hospitalization. Additional outcomes assessed were trends in colectomy over this time period and predictors for colectomy during the index hospitalization. Results A total of 5.3% of UC patients underwent colectomy during the index hospitalization (95%CI 3.3–7.3%) and 11.9% underwent colectomy within the first year after hospitalization (95% CI: 9.1–14.6%). Inpatient colectomy declined from 6.5% (95%CI: 2.5–10.6%) in 2009–2010 to 3.4% (95%CI: 3.3–7.3%) in 2015–2017. Colectomy in the first year after admission declined from 15.7% (95%CI 10.0–21.4%) in 2009–2011 to (10.1%; 95% CI: 5.4–14.7) in 2015–2017. A total of 16.3% of patients received infliximab in the hospital and 36.3% received within the first year of admission. In a multivariable model, the 2015–2017 cohort experienced a 64% reduction in inpatient colectomy compared to the 2009–2011 cohort (OR 0.36; 95%CI: 0.11–1.14, p=0.082) and a 54% reduction in colectomy in the first year (OR 0.46; 95%CI: 0.22–0.96, p=0.038). A prior study between 1998–2004 in this same population showed colectomy rates of 20% during the index hospitalization and 30% at 1 year. Conclusion Colectomy within the first year after hospital admission for UC has declined over time in the modern era of biologics with a magnitude of colectomy that is lower than the historical rate of 20–30%. Our data imply cause for optimism that the natural history of colectomy in acute severe UC may be different and modifiable in the modern biologic era compared to the past.

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Natural history of premature thelarche: review of 60 girls

Paediatrica Indonesiana ◽

10.14238/pi41.6.2001.279-83 ◽

2001 ◽

Vol 41 (6) ◽

pp. 279 ◽

Cited By ~ 1

Author(s):

Jose R. L. Batubara ◽

Adji Suranto ◽

Sudigdo Sastroasmoro ◽

Bambang Tridjaja ◽

Aman B. Pulungan

Keyword(s):

Natural History ◽

Practical Knowledge ◽

Central Precocious Puberty ◽

Daily Practice ◽

Bone Age ◽

Breast Development ◽

First Year ◽

Premature Thelarche ◽

History Of ◽

One Year

In Indonesia report on the natural history of premature thelarche is very limited. Daily practice requires physicians to have some basic practical knowledge, among others the natural history of premature thelarche, in order to manage these patients properly. We reviewed data of 85 premature thelarche patients who visited our department from January 1989 until December 1998. Only 60 patients met the study criteria. The mean chronological age of the patients at diagnosis was 43.4 months. About half of these patients (31/60) were diagnosed before they were 2 years old. Half of the patients had bilateral breast involvement. The hormonal pattern showed 24/48 follicle stimulating hormone predominant-response. Most patients (33/47) showed normal plasma estradiol level. Bone age analysis was normal in 46/57 patients, and only 9 showed accelerated bone age. Pelvic ultrasonography showed prepubertal reproduction organs in 26/35. Vaginal smears showed signs of estrogenization with various degree of stimulation in 13 patients. At the end of observation the outcome of premature thelarche were: 31 regressed, 19 persisted, 6 had progressive breast development and 4 progressed to central precocious puberty. The initial clinical and laboratory characteristics of those who developed CPP varied. Among 31 premature thelarche patients who regressed, 21 had onset of breast enlargement before age of 2 years. In most of the regressed patients (20/31), regression occurred completely within the first year. Most premature thelarche patients with onset before 2 years will regress within one year after diagnosis.

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Retrospective Analysis of the Natural History of Atopic Dermatitis Occurring in the First Year of Life in Korean Children

Journal of Korean Medical Science ◽

10.3346/jkms.2012.27.7.723 ◽

2012 ◽

Vol 27 (7) ◽

pp. 723 ◽

Cited By ~ 8

Author(s):

Younghee Chung ◽

Jung Hyun Kwon ◽

Jihyun Kim ◽

Youngshin Han ◽

Sang-Il Lee ◽

...

Keyword(s):

Atopic Dermatitis ◽

Natural History ◽

Retrospective Analysis ◽

First Year ◽

Korean Children ◽

History Of ◽

First Year Of Life

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Paroxysmal nocturnal hemoglobinuria: natural history of disease subcategories

Blood ◽

10.1182/blood-2008-01-133918 ◽

2008 ◽

Vol 112 (8) ◽

pp. 3099-3106 ◽

Cited By ~ 195

Author(s):

Régis Peffault de Latour ◽

Jean Yves Mary ◽

Célia Salanoubat ◽

Louis Terriou ◽

Gabriel Etienne ◽

...

Keyword(s):

Natural History ◽

Paroxysmal Nocturnal Hemoglobinuria ◽

Immunosuppressive Treatment ◽

Survival Rates ◽

Time Dependent ◽

First Year ◽

Prognosis Factors ◽

History Of Disease ◽

History Of

AbstractThe natural history of paroxysmal nocturnal hemoglobinuria (PNH) clinical subcategories (classic PNH and aplastic anemia [AA]/PNH syndrome) is still unknown. We retrospectively studied 460 PNH patients diagnosed in 58 French hematologic centers from 1950 to 2005. The median (SE) follow-up time was 6.8 (0.5) years. The median survival time (SE) was 22 (2.5) years. We identified 113 patients with classic PNH, 224 patients with AA-PNH syndrome, and 93 (22%) intermediate patients who did not fit within these 2 categories. At presentation, classic PNH patients were older, with more frequent abdominal pain and displayed higher levels of GPI-AP–deficient granulocytes. A time-dependent improved survival was observed. In classic PNH, diagnoses before 1986 (hazard ratio [HR]: 3.6; P = .01) and increasing age (P < .001) were associated with worse survival prognoses, whereas use of androgens within the first year after diagnosis was protective (HR, 0.17; P = .01). Transfusions before 1996 (HR, 2.7; P = .007) led to lower survival rates in patients with AA-PNH syndrome, whereas immunosuppressive treatment was associated with better outcomes (HR, 0.33; P = .03). Evolution to thrombosis affected survival in both subcategories (classic PNH: HR, 7.8 [P < .001]; AA-PNH syndrome: HR, 33.0 [P < .001]). Evolution to bicytopenia or pancytopenia for classic PNH (HR, 7.3, P < .001) and malignancies for AA-PNH syndrome (HR, 48.8; P < .001) were associated with worse outcomes. Although clinical presenta-tion and prognosis factors are different, classic PNH and AA-PNH syndrome present roughly similar outcomes, affected mainly by complications.

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The Natural History of Weight Percentile Changes in the First Year of Life

JAMA Pediatrics ◽

10.1001/jamapediatrics.2014.345 ◽

2014 ◽

Vol 168 (7) ◽

pp. 681 ◽

Cited By ~ 6

Author(s):

William E. Bennett ◽

Kristin S. Hendrix ◽

Rachel T. Thompson ◽

Aaron E. Carroll ◽

Stephen M. Downs

Keyword(s):

Natural History ◽

First Year ◽

History Of ◽

First Year Of Life ◽

Weight Percentile

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Feeding, reproduction and growth in the crowned snake

Amphibia-Reptilia ◽

10.1163/156853898x00214 ◽

1998 ◽

Vol 19 (3) ◽

pp. 311-318 ◽

Cited By ~ 27

Author(s):

Otavio A.V. Marques ◽

Giuseppe Puorto

Keyword(s):

Seasonal Variation ◽

Natural History ◽

Rainy Season ◽

Sexual Maturity ◽

Southeastern Brazil ◽

First Year ◽

Main Prey ◽

Significant Seasonal Variation ◽

Body Sizes ◽

History Of

AbstractThe crowned snake Tantilla melanocephala is a small fossorial sonorine snake, widely distributed through South America. Dissection of 186 specimens, combined with observations of captive specimens, provided information on the natural history of this species in southeastern Brazil. Females attained larger body sizes than males. Apparently T. melanocephala forage at night for active prey. Centipedes of the genus Otostigmus were the main prey item. These centipedes are subdued by injection of venom. Data from preserved specimens showed no significant seasonal variation in the number of collected snakes. Reproduction seemed to be highly seasonal with vitellogenesis occurring from onset to the middle of the rainy season and hatching at the end of the rainy season. Clutch size ranged from one to three and was correlated with female body length. Neonates measured 10-12 cm snout-vent length and juveniles attained about 17.5 cm SVL during the first year. Apparently males attain sexual maturity at an age of about 10 months and females at about 20 months.

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