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PubMed1975

[Myelomonocytic leukemia with Philadelphia-positive and Philadelphia-negative cell lines in early childhood (author's transl)].

The authors report on an infant with myeloproliferative syndrome of the myelomonocytic type. The findings fulfilled the criteria for juvenile chronic myeloid leukemia except that there was no increase of fetal hemoglobulin and no depression of erythrocyte carbonic anhydrase I. In one half of the bone marrow cells a Ph1-chromosome was found. During the course of the disease the Ph1-positive clone was successively replaced by cell lines with different chromosomal aberrations. The relationship between the cytogenetic mosaicism and the clinical and laboratory findings is discussed.

Adult↗
PubMed2002

The epidemiology of type 1 diabetes in children in Philadelphia 1990-1994: evidence of an epidemic.

OBJECTIVE: To determine the epidemiology of type 1 diabetes in children in Philadelphia from 1990 to 1994, and to identify whether an epidemic occurred during that time period. RESEARCH DESIGN AND METHODS: This is a descriptive epidemiological study using a retrospective population-based registry in Philadelphia, PA, a city with large white, African-American, and Hispanic (Puerto Rican) populations. All hospitals in Philadelphia that admit children were identified. All charts meeting the following criteria were reviewed: 1) newly diagnosed type 1 diabetes, 2) children 0-14 years of age, 3) children residing in Philadelphia at the time of diagnosis, and 4) those diagnosed from 1 January 1990 to 31 December 1994. Standard type 1 diabetes registry data were abstracted from the charts. Ascertainment of the completeness of the hospital registry was validated by data from the Philadelphia School District. Communicable disease records were reviewed to identify epidemics from 1987 to 1995. RESULTS: A total of 209 cases were identified, and the combined hospital and school registry was determined to be 96% complete. The overall age-adjusted incidence rate in Philadelphia was 13.3/100,000/year. The highest rate by race continues to be in the Hispanic population (15.5). The incidence in African-American children has increased markedly (12.8), particularly in the 10- to 14-year age-group (22.9). An epidemic of type 1 diabetes occurred from January to June 1993, approximately 2 years after a measles epidemic in Philadelphia. CONCLUSIONS: The overall incidence of type 1 diabetes in Philadelphia is similar to other U.S. registries. The incidence in the Hispanic population continues to be among the highest of any U.S. ethnic group. The marked increase in incidence in the African-American population may be due in part to misclassification of cases actually having type 2 diabetes. The 1993 epidemic may have been due to beta-cell autoimmunity triggered by the measles virus.

Adolescent↗
PubMed1994

Mortality in a cohort of homeless adults in Philadelphia.

BACKGROUND: Homeless people are at high risk for death from many causes, but age-adjusted death rates for well-defined homeless populations have not been determined. METHODS: We identified 6308 homeless persons 15 to 74 years of age who were served by one or both of two agencies for the homeless in Philadelphia between January 1, 1985, and December 31, 1988. Using a data base that contained all deaths in Philadelphia and listings of all Philadelphia residents during the same period, we compared the mortality rate for this homeless population with the rate in the general population of Philadelphia. RESULTS: The age-adjusted mortality rate among the homeless was 3.5 times that of Philadelphia's general population (95 percent confidence interval, 2.8 to 4.5). The age-adjusted number of years of potential life lost before the age of 75 years was 3.6 times higher for the homeless people than for the general population (345 vs. 97 years lost per 1000 person-years of observation). Fifty-one of the 96 deaths of homeless persons (53 percent) occurred during the summer months. Mortality rates were higher among the homeless than in the general population for nonwhites, whites, women, and men. Within the homeless cohort, white men and substance abusers had higher mortality rates than other subgroups, but even homeless people not known to be substance abusers had a threefold higher risk of death than members of the general population. Injuries, heart disease, liver disease, poisoning, and ill-defined conditions accounted for 73 percent of all the deaths among the homeless. CONCLUSIONS: Homeless adults in Philadelphia have an age-adjusted mortality rate nearly four times that of Philadelphia's general population. White men and substance abusers are at particularly high risk. Matching cohorts of homeless people to death records is a useful way to monitor mortality rates over time, evaluate interventions, and identify subgroups with an increased risk of death.

Adolescent↗
PubMed1993

The epidemiology of type I diabetes in children 0-14 yr of age in Philadelphia.

OBJECTIVE: To determine the epidemiology of type I diabetes in children in Philadelphia, particularly with respect to racial differences. RESEARCH DESIGN AND METHODS: This is a descriptive epidemiological study using a retrospective population-based registry in Philadelphia, Pennsylvania, a city with large white, black, and Hispanic populations. All hospitals in Philadelphia that admit children were identified. All charts meeting the following criteria were reviewed: 1) newly diagnosed IDDM, 2) 0-14 yr of age, 3) residing in Philadelphia at the time of diagnosis, and 4) diagnosed from 1 January 1985 to 31 December 1989. Standard IDDM registry data were abstracted from the charts. Ascertainment of the completeness of the hospital registry was validated by two secondary sources: 1) diabetes camp records, 2) Philadelphia School District data. RESULTS: A total of 215 cases were identified, and the combined hospital, camp, and school registry was determined to be 93% complete. The overall age-adjusted incidence rate in Philadelphia was 13.4/100,000/yr. The highest incidence rate was in Hispanics (15.16), followed by whites (13.31), and blacks (10.95). CONCLUSIONS: The incidence of IDDM in Philadelphia is similar to what has been found in other U.S. registries. The unexpected finding was that the incidence in the Puerto Rican Hispanic population was the highest in the city. This is the first data reported on this population, and more studies are needed to elucidate the genetic and environmental causes of this high incidence of IDDM.

Adolescent↗
PubMed2001

A comparative, volumetric survey of airborne pollen in Philadelphia, Pennsylvania (1991-1997) and Cherry Hill, New Jersey (1995-1997).

BACKGROUND: Aeroallergen sampling provides information regarding the onset, duration, and severity of the pollen season that clinicians use to guide allergen selection for skin testing and treatment. OBJECTIVE: This atmospheric survey reports: 1) airborne pollen contributors in Philadelphia, Pennsylvania (1991 to 1997) and suburban Cherry Hill, New Jersey (1995 to 1997); 2) pollen onset, duration, and peak level; and 3) the relationship between airborne pollen and selected meteorologic variables. METHODS: At both locations, separated by 11 km. sampling was performed with a volumetric Rotorod Sampler (Sampling Technologies, Minnetonka, MN). RESULTS: In Philadelphia and Cherry Hill, respectively, 3-year average measurements included 75.0 and 74.2% tree pollen, 10.2 and 8.3% grass pollen, and 14.8 and 17.5% weed pollen. Prominent airborne pollen taxa were Acer, Quercus, Betula, Pinus, Cupressaceae, Poaceae, Ambrosia, and Rumex. The tree, grass, and weed pollen seasons extended from mid-March to mid-June, late April to mid-June, and mid-August to late September, respectively. A secondary Poaceae pollen peak occurred in September. There was a statistically significant correlation between simultaneous weekly average pollen levels in Philadelphia and in Cherry Hill (Acer, r(p) = 0.987, Quercus, r(p) = 0.645, Betula, r(p) = 0.896, Pinus, r(p) = 0.732, Cupressaceae, r(p) = 0.695, Poaceae, r(p) = 0.950, Ambrosia, r(p) = 0.903, and Rumex, r(p) = 0.572, P <0.001). Daily Poaceae pollen levels were positively influenced by same-day high temperature (r(s) = 0.333 in Philadelphia and r(s) = 0.426 in Cherry Hill, P < 0.05). Daily Ambrosia pollen levels were inversely influenced by same-day total precipitation (r(s) = -0.174 in Philadelphia and r(s) = -0.257 in Cherry Hill, P < 0.05). CONCLUSIONS: This is the first volumetric survey performed in either Philadelphia or Cherry Hill. Copious amounts of airborne pollen were seen from late April to early May and in early September. Pollen onset, duration, and year-to-year variability were similar at both sites. An awareness of local aeroallergen patterns is critical in the effective testing and treatment of atopic individuals.

Air Pollution↗
PubMed1999

Molecular cytogenetics investigation of the telomeres in a case of Philadelphia positive B-ALL with a single telomere expansion.

We have investigated a single telomere expansion in a case of acute lymphoblastic B-cell leukemia (B-ALL), where half of the cells in the bone marrow sample appeared with a Philadelphia chromosome. Comparing telomere sizes in Philadelphia-positive versus -negative cells, we found generally shorter telomeres in the Philadelphia-positive cells, but with an expansion of the telomere on the long arm of one chromosome 11 homologue. This expansion was also found in a minority of Philadelphia-negative cells. The telomeres in these cells were of the same overall size as the telomeres in the Philadelphia-negative cells without the 11q expansion. Together, these findings suggest that the order of events was: 11q telomere expansion, Philadelphia translocation, overall telomere shortening. The expanded 11q telomere contained the standard telomeric (AGGGTT)(n) repeat, but also variant repeat sequences. The single telomere expansion suggests a non-telomerase mechanism behind the expansion which may also explain the presence of variant repeats in the expanded telomere. The present case illustrates that telomere changes may occur at only some chromosome ends in a subset of cells. To reveal such changes, telomere morphology should be studied with in situ methodology.

Adult↗
PubMed1980

Hb S, Hb G-Philadelphia and alpha-thalassemia-2 in a Black family.

A Black family is described in which Hb S, Hb G-Philadelphia and alpha-thalassemia-2 determinants occurred in different combinations. The propositus was a healthy fullterm neonate who had 46% Hb G-Philadelphia and about 5% Hb Bart's in cord blood together with a relative microcytosis (MCV = 85 fl) and hypochromia (MCH = 28 pg). This is consistent with a diagnosis of Hb G-Philadelphia trait in association with a homozygous alpha-thalassemia-2 (alpha 0 alpha/alpha 0 alpha G; beta A/beta A). The mother and another son also had Hb G-Philadelphia in association with Hb S trait but with 37% Hb G-Philadelphia and with 39% Hb S. Hemotological and biosynthetic studies confirm the assignment of the alpha alpha/alpha 0 alpha G; beta A/beta S genotype in both and that of the alpha alpha/alpha 0 alpha; beta A/beta A genotype in the father. Despite this evidence for a moderate alpha chain deficiency in the propositus, the biosynthetic alpha/non-alpha value in the neonatal period was a high 1.2. Similar values were observed in 8 control cord blood samples if the incubation was not delayed longer than 3 hours after collection (alpha/non-alpha = 1.28 +/- 0.14). When the propositus was studied again, but at six months of age, the proportion of Hb G-Philadelphia in peripheral blood was unchanged, a marked microcytosis and hypochromia were observed, and a distinct deficiency of alpha chain synthesis (alpha/non-alpha = 0.56) was present.

Adult↗