Lipodystrophia Progressiva and Temporary Hydronephrosis
Authors: P. B. S. Fowler
Journal: BMJ (1955)

If the association of two diseases in a patient is not fortuitous their relationship to each other may throw light on the aetiology of one or both diseases. Law rence's (1946) study of a woman with lipodystrophia progressiva and insulin-resistant diabetes supports the concept of two types of diabetes mellitus and suggests the simple explanation that the elderly obese person develops diabetes because she is unable to convert any more carbohydrate into fat. In this paper the association of temporary hydro nephrosis with lipodystrophia progressiva is reported and the renal disorders which have been described with this disease are reviewed. It is suggested that in some of the case reports the renal changes may have been similar to those present in the patient described here. Gross  can develop in a week and dis appear completely if the obstruction is removed early enough, and the term  temporary hydronephrosis has been used to describe these transient changes (Fowler and Frankel, 1947). Ellis (1951), writing about lipodystrophia progressiva, mentions the association of this condition with renal disease. He makes a brief reference to the occurrence of oliguria in Gerhartz's case (1916). The daily urinary output of this patient was 400-1,100 ml., and it is difficult to believe that this is a significant oliguria. Klien (1921) reports on a patient with lipodystrophy and mentions polyuria. Recently a patient with this disease was shown at the Royal Society of Medicine (Tizard, 1954), and the summary of the case included the information that  she is said to drink and void excessive quantities of fluid. The glucose-tolerance test in this patient was normal, but full urinary investigations including a pyelo gram had not been done. Following the paper by Law rence (1946) on the association of lipodystrophy and diabetes, any patient with lipodystrophy complaining of thirst and polyuria may be examined for further evidence of diabetes mellitus only, as happened to our patient, when studied for a short period at another hospital. In 1932 two cases were reported in which lipo dystrophia progressiva and uraemia occurred in the same patient. They were both thought to have had chronic nephritis, but in neither did the diagnosis appear likely, nor was it confirmed. They both had unusual clinical features in common with the patient described in the present paper. Horton and Emmett (1932) report the case of a woman of 61 with a history of increasing nausea for one year with occasional attacks of vomiting. The patient was alert, and physical examination was essentially nega tive except for lipodystrophy affecting the legs. There were no symptoms or signs suggesting uraemia. On admission the blood urea was 400 mg. per 100 ml. After five weeks on a diet containing 25 g. of protein and 3-4 litres of fluid daily, the blood urea fell to 60 mg. per 100 ml. It was stated that pus cells, grade III, were present in the urine, l;ut no mention of urine culture was made. The renal lession was diagnosed as chronic diffuse glomerulonephritis after the possibility of poly cystic kidneys had been considered and dismissed, as no enlargement of the kidneys could be found on abdo minal palpation. Berger (1932) reported the case of a woman aged 26 who developed lipodystrophy at the age of 3years. Pyelitis occurred at 6 and another attack a year later. At the age of 19 the blood pressure was found to be raised and albumin was present in the urine. A diag nosis of acute exacerbation of chronic nephritis was made. Following a severe reaction to vaccination, the patient for a short time passed into uraemic coma. During the next seven years she remained well, and clinical examination at the end of this period was essen tially negative except for the lipodystrophy. The blood urea was then 21 mg. per 100 ml. Igersheimer (1948) described the case of a patient who had lipodystrophia progressiva, bilateral  with aberrant renal arteries, and other congenital abnor malities.