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A T Piesowicz

Publications and source records attributed to A T Piesowicz.

9 recordsLinked to original sources

Limited joint mobility in children and adolescents with insulin dependent diabetes mellitus.

Joint mobility was studied in 70 children with insulin dependent diabetes mellitus aged 8-17 years, and the prevalence of limited joint mobility (LJM) was found to be 31% (22/70). This figure fell to only 7% (5/70) when an alternative method of assessment was used. A high number of non-diabetic, non-sibling controls (6/51 (12%] were found to have LJM. There was a trend towards an increasing prevalence of LJM with increasing age and duration of diabetes, but it was also found in patients with recent onset diabetes. A large proportion of prepubertal patients were noted to have LJM. No correlation was found between LJM and either short stature or diabetic control. There is a need for standardisation of the methods used to define and stage LJM in diabetic patients, and the significance of this clinical finding remains unclear.

Adolescent

Focal changes in the globi pallidi associated with neurological dysfunction in methylmalonic acidaemia.

Neurological abnormalities are common in patients with organic acidaemias and sometimes these are distinctive. Lesions in various parts of the brain are increasingly being recognised in patients with methylmalonic acidaemia. We present the cases of two patients with methylmalonic acidaemia who became acutely ill with marked metabolic acidosis. CT scan showed bilateral areas of low density involving the globi pallidi. One patient developed dystonic posturing of the upper and lower limbs with rigidity and bradykinesia following her recovery. Treatment with benzhexol and with levodopa and carbidopa had no sustained benefit. Her gait slowly improved but her dystonic posturing remained. The other patient developed marked truncal hypotonia and a variable increase in limb tone following the acute illness. These abnormalities have persisted. It is concluded that structural brain changes, and in particular symmetrical lesions in the globi pallidi, may follow acute illness in patients with methylmalonic acidaemia and be accompanied by neurological symptoms.

Adolescent

Pupillary size in children and adolescents with type 1 diabetes.

Pupillary adaptation to darkness was studied in 63 children and adolescents with Type 1 diabetes using a simple portable pupillometer. Results were compared with those in a group of age-related non-diabetic children and expressed as the ratio of the pupil diameter to the iris diameter (pupil diameter %). In the diabetic patients the pupil diameter % was 61.1 +/- 5.8 (44.4-71.9) % compared with 64.2 +/- 4.1 (53.2-72.6) % in the control subjects (p less than 0.001). Abnormal pupillary adaptation to darkness was found more commonly than abnormal heart rate variation in response to a variety of stimuli in the diabetic patients. Pupillary adaptation to darkness may be useful as an indicator of subclinical autonomic neuropathy in diabetic children.

Adolescent

Polycystic ovary syndrome in a virilised, premenarcheal girl.

A premenarcheal girl aged 12 years presented with an abdominopelvic mass and virilisation. A large ovarian cyst was removed at laparotomy. A histological diagnosis of polycystic ovarian syndrome was made, with no evidence of an associated masculinising tumour.

Child

Hyperprolinaemia.

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Amino Acid Metabolism, Inborn Errors