To classify the patients regarding the disease severity, we used the
Shwachman Score (< 40 = severe; 41-55 = poor; 56-70 = average; 71-85 = good; and 86-100 = excellent).
In conclusion, paediatricians must be aware of, to include a few; causes and details of acute and chronic pancreatitis,
Shwachman Diamond syndrome, Johansson-Blizzard syndrome, congenital anomalies and cystic fibrosis in which the pancreas can be involved and hence warrants a specific clinical examination, investigation and management respectively.
The mean FEV1 of the subjects was 96.4% [+ or -] 20.3% of the predicted value and the mean
Shwachman score was 66.8/75 (SD = [+ or -] 9.6).
Harry
Shwachman of the Children's Hospital Medical Center in Boston along with Kopito of the Massachusetts Institute of Technology have shown that children with cystic fibrosis have only about ten percent of the normal concentrations of tightly bound calcium.
The median for
Shwachman score was 85 (range, 65-100).
"It's going to be my first time sharing a stage with all those acts,'' says Wade, whose real name is Matt
Shwachman. "I'm very excited to play my hometown for the show, with those acts, at Mechanics Hall.''
aeruginosa + 9 -
Shwachman score 75 - FEV1 (% predicted) 83 - MEF 25 (% predicted) 36.7 - VC (% predicted) 84.9 - [DELTA]F 508 (homozygous): homozygous for the AF 508 mutation; m: male; f: female; P.
Genetic diseases among the patients were Wilson's disease, muscular dystrophy, alpha-1-antitrypsin deficiency, Alagille syndrome, hereditary fructose intolerance, glycogen storage disease, ornithine transcarbamylase deficiency and
Shwachman's syndrome.
Furthermore, a number of inherited conditions, for example Down syndrome, Fanconi anemia, Bloom syndrome, Wiskott-Aldrich syndrome, dyskeratosis congenita, Werner syndrome,
Shwachman syndrome, Blackfan-Diamond syndrome, and Klinefelter syndrome, carry an increased risk of AML [5,6].
The occurrence of leukemia in patients with the
Shwachman syndrome.
In 1980, Goldberg,
Shwachman, and Isralsky noted that, "Although the medical outlook for cystic fibrosis has improved considerably, there is apparently a cultural lag between the new advances known to a group of specialists and the lack of acceptance by the rehabilitation community of this group of patients" (p.
He suffers from
Shwachman Syndrome, an illness so rare there are only 300 sufferers worldwide, only 30 to 40 families in Britain and only four children in the Midlands.