Carbohydrate intolerance and frank diabetes mellitus (DM) are recognised features of cystic fibrosis (CF), but their cause has not been established. Damage to the islet cells due to pancreatic fibrosis is the most common explanation. The relationship between the genotype and the occurrence of diabetes mellitus has been examined in 21 cystic fibrosis patients attending our out-patient chest clinics. DNA samples were tested for the presence of the most common mutation (delta F508) and the results related to the degree of carbohydrate intolerance. We found that in CF patients over 18 years of age diabetes is more frequent among those homozygous for the delta F508 allele than the heterozygotes (P < 0.05). Those homozygous for delta F508 were diagnosed as CF at an earlier age than the heterozygotes (P < 0.05). Both the mean age and the female/male ratio were similar in the diabetic and non-diabetic groups. The prevalence of the delta F508 allele was similar to that expected in a random sample of East Anglian cystic fibrosis patients. These data suggest that the occurrence of diabetes mellitus in patients with cystic fibrosis is related to the genotype, which could be secondary to the way the genetic factors influence the degree of pancreatic disease and its rate of progression.

Pancreatic Disease

ΔF508

Cystic fibrosis-related diabetes

Heterozygote advantage

Compound heterozygosity

10.1111/j.1399-0004.1993.tb04445.x

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Citations (35)

Sleep disorders and response to treatment in the Acromegalic cardiovascular and respiratory outcomes with primary analogue therapy (ACROPAT) trial

Society for Endocrinology BES (2007)

Alison Webb Sam Pilsworth Sam O’Toole Chris Strey Shaumya Ariyaratnam

Sleep

Source

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Citations (0)

Comparison of the Bronchodilatation of Ipratropium Bromide and Fenoterol Nebuliser Solutions Administered Alone and in Combination

Respiration (1986)

John M. Shneerson

Bronchodilatation

Ipratropium bromide

Fenoterol

Ipratropium

β2 adrenergic receptor

Nebulizer

10.1159/000195144

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Citations (2)

Pulmonary artery pressure in thoracic scoliosis during and after exercise while breathing air and pure oxygen.

Thorax (1978)

John M. Shneerson

Pulmonary artery catheterisation was carried out in 25 scoliotics aged 13 to 67 years (mean 30.7). Each then performed a progressive exercise test breathing air, and 11 performed a similar test breathing pure oxygen. The mean pulmonary artery pressure (PAP) increased linearly with oxygen uptake (VO2) and with the work rate. The pressure responses have been described in terms of ther intercept and rate of rise of pressure (sPAP/VO2 and sPAP/work rate). sPAP/VO2 was unrelated to the anatomical features of the scoliosis, or to PaO2. It was inversely related to vital capacity, functional residual capacity, and total lung capacity. Inspiration of pure oxygen lowered the resting pressure by a mean of 3.2 mmHg but only decreased sPAP/work rate by 9%. The maximum pressure reached during exercise was diminished by a mean of 5.2 mmHg when pure oxygen was breathed. The mean pressures were shown to fall exponentially after exercise. The time constants were proportional to sPAP/VO2 and to the final pressure reached during exercise. Inspiration of pure oxygen did not effect the time constants but decreased the post-exercise pressure load by lowering the final pressure during exercise.

Work of breathing

Breathing gas

10.1136/thx.33.6.747

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Citations (21)

Handbook of Sleep Medicine.

Thorax (2001)

John M. Shneerson

Myositis associated with graft-versus-hostdisease (GVHD) typically presents with proximal muscle weakness, myalgias, and a raised creatinine phosphokinase (CPK) level.We report a case of a 51 year old man who developed respiratory muscle weakness five years after an allogeneic bone marrow transplant for multiple myeloma.His symptoms included tachypnoea, abdominal paradox, and orthopnoea.Pulmonary function tests revealed diminished vital capacity and maximal inspiratory and expiratory pressures.Serum CPK levels were raised and a peripheral muscle biopsy specimen was consistent with GVHD.He improved with immunosuppressive therapy.

Sleep

Sleep medicine

10.1136/thorax.56.1.86

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Citations (76)

The Effect of Noninvasive Intermittent Positive-Pressure Ventilation During Exercise in Severe Scoliosis

CHEST Journal (2002)

Martin Highcock Ian E. Smith John M. Shneerson

Mouthpiece

Respiratory minute volume

Respiratory Rate

Treadmill

10.1378/chest.121.5.1555

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Citations (20)