A clinico-pathological study of Cushing's syndrome at the University Hospital of the West Indies and a review of literature

Cushing's syndrome is an uncommon but important disease. Twenty-one confirmed cases of spontaneous Cuching's syndrome were documented at the University Hospital of the West Indies over a 21-year period. They were predominantly young females (F:M ratio of 17:4; mean age 25 years and three months). The commonest presenting symptoms were amenorrhoea (41 percent) and obesity (19 percent). Common clinical features were cushingnoid features (95 percent), hypertension (76 percent) and hirsutism (82 percent). Twenty-nine per cent had frank hyperglycaemia. Cushing's syndrome was due to Cushing's disease in 10 cases, adrenal adenoma in 3 and adrenal carcinoma in 2 cases. In 4 cases with presumed adrenal hyperplasia, the histology was either unavailable or was not consistent with the diagnosis. Two cases appear now to have had ectopic ACTH syndrome. Adrenalectomy was the commonest treatment offered. There were no intra-operative or post-operative deaths but recurrence was common after subtotal adrenalectomy in Cushing's disease. Twenty-seven per cent of the patients developed Nelson's syndrome, which was fatal in 50 per cent. Long-term hormone replacement therapy was unnecessary after surgery for adrenal adenomas. Treatment of Cushing's syndrome was well tolerated by the patients. (AU)

The neurological complications of diabetes mellitus: a clinical classification - abstract

Diabetic neurological disorders affect a significant proportion of our diabetic population in the Caribbean and world-wide. With the increasing life expectancy of today's diabetic population so has the incidence of these neurological disorders increased. Even without this, however, with early diagnosis it has been found that neurological lesions can occur early in the disease and in some instances be the presenting manifestation of hyperglycaemia. Over the ensuring years an increasing numbers of neurological syndromes have been identified affecting both somatic and autonomic nervous systems. These classifications have used either anatomical location, symmetrical or asymmetrical involving, focal or generlaised, functional disturbances, and have even been based on possible aetiological factors, recognisable clinical syndromes, type of nerve fibre implicated, pathological, and clinical course of the disorder, etc. In clinics and institutions where investigative tools such as electromyography, nerve conduction studies and the more refined biochemical modulations are available, classification of the syndromes on an anatomical or aetiological basis can be defined, but in settings such as ours and in institutions not having access to sophisticated neuro-investigations, clinicians have to rely on their clinical knowledge to specify and identify these neurological syndromes. In order to better identify these neurological lesions, we at the University of the West Indies. (Drs. C. McIver, E. Morrison, D. Kelly and R. Richards) began an attempt to identify and describe the neurological lesions and their prevalence in the diabetic population attending the Diabetic Clinic at the University Hospital of the West Indies between 1967 and 1969. Based on these clinical assessment we were able to identify a number of clinical presentations of neurological disorders in our diabetic subjects. We found that diabetic neuropathy or neurological disorders were present in 69 percent of the diabetic population, with neuropathy being present in 59 percent of the male and 36 percent of the female population attending the clinic over the period of assessment. Again, it was found that 12 percent - 15 percent of patients presenting with a history of a few months to two years had clinical features of diabetic neuropathey which at times could be ameliorated by normalisation of their hyperglycaemia (AU)

Clinical profile of diabetes mellitus in Jamaica (phasic insulin dependence)

A retrospective study of 516 patients attending the Diabetic Outpatient Clinic at the University Hospital of the West Indies revealed that 13.9 percent were insulin-dependent, 12.9 percent required intermittent insulin therapy (phasic insulin dependent), and 1.8 percent required combined insulin and biguanide therapy for adequate control; 38.4 percent needed sulphonylurea plus biguanide therapy, 21.2 percent a sulphonylurea preparation alone, 8.7 percent biguanide alone and 3.1 percent diet alone. There were 40.6 percent males and 59.4 percent females with an age range of 2 to 70 years (modal age group 61-70 years). It was noted that most patients had their disease for 10+ years before being first seen at a tertiary health care centre when complications such as gangrene (6.8 percent), neuropathy (4.0 percent), retinopathy (2.1 percent), ischaemic heart disease (1.9 percent) and nephropathy (1.2 percent) had already been present. To make an impact on the loss of productive man-hours due to diabetes mellitus, there needs to be an assessment of the non-tertiary health care, and for appropriate intervention programmes to be undertaken (AU)

The syndrome of insulin resistance, acanthosis nigricans and an autoimmune disorder in diabetes mellitus treated with immunosuppressants

Insulin resistance in diabetes mellitus associated with acanthosis nigricans and an underlying autoimmune disorder has, so far, been described in the world literature only in 25 patients. This is another such patient to be described, and antibodies were demonstrated against the insulin receptor sites in monocytes. The insulin refractoriness was treated successfully by immunosuppressant therapy and, after one year, the autoimmune condition subsided. The patient continues to do well and achieved euglycaemic control on a diabetic diet alone (AU)

Cushing's syndrome and its differential at the University Hospital of the West Indies - abstract

Crushing's syndrome is a rare condition with a potential for morbidity and mortality. During a 20-year retrospective review of patients with the diagnosis confirmed at the University Hospital, 20 cases were seen. There were 5 men and 15 women with an age range of 15 to 60 years and a mean age of 27 years. Sixty per cent of cases were due to hyperplasia, 15 percent to adenoma, 10 percent to carcinoma and 5 percent(1 case) was of ectopic origin in a patient with bronchogenic carcinoma. The zona fasciculata was involved in 90 percent of cases of hyperplasia. The clinical features of these black patients were as described elsewhere but we noted hypopigmentation alone in 45 percent of patients, with hyperpigmentation in 33 percent. One female patient, age 38, had a 13-year history of hypertension and later developed psychiatric symptoms. A 30-year-old man, who had severe hypertension of recent onset, developed congestive cardiac failure, and an adenoma compressing the renal artery was found at laparotomy. Difficult to control diabetes mellitus in a 27-year-old female was followed 3 years later by the development of cushionoid features. The mean time interval between the onset ofsymptoms and the confirmation of the diagnosis was 2 years 8 months. Serum cortisol levels with loss of diurnal variation and the dexamethasone suppression tests are still quite useful in confirming the diagnosis. Urinary cortisol and serum ACTH levels are not available here. Venography is the commonest and better-used method for localisation. Ultrasonography recently available has so far not been found to be helpful. Sixteen patients underwent successful surgery; two were scheduled for surgery, and one refused. Three patients developed Nelson's syndrome 1 to 4 years after surgery and two of them died, one in the perioperative period after transphenoidal resection of the pituitary tumor. Twenty-seven patients investigated for cushing's Syndrome during the same study period showed the cause to be simple obesity in 21 and Stein-Leventhal Syndrome in 6. It is concluded that blacks in Jamaica with cushing's syndrome present with similar clinical features as elsewhere, except for hypopigmented skin lesions. The zona fasciculata site involved. The serum cortisol and dexamethasone suppression tests are satisfactory in establishing a diagnosis (AU)

Altered thyroid status: a possible cause of growth retardation in children with congenital heart disease

To investigate the possibility that growth retardation in children suffering from congenital heart disease (CHD) may be due to altered thyroid function, we studied 27 growth-retarded children with CHD (7 cyanotic, 20 acyanotic) and compared the results with thyroid functions of 10 healthy normal-growing children of the same age group (control). Serum tri-iodothyronine (T3) and throxine (T4) levels were significantly lower in cyanotic CHD; however, serum thyrotropin (TSH) levels were not affected. In acyanotic children, thyroid function was essentially normal. These findings suggest that subnormal levels of T3 and T4 with normal TSH might be a homeostatic response to chronic hypoxia in cyanotic CHD and may contribute to growth retardation in these children (AU)

Periarteritis nodosa and pregnancy

Pregnant patients with periarteritis resulted in maaternal death in 7 of 8 cases that have been reported. In the present case, periarteritis was in remission throughout the pregnancy; the patient was thus the second known maternal survivor. The infant also did well. It is suggested that pregnancy probably does not have as direct an effect on the course of the disorder as appears from the outcome of the previous case. Extreme caution must prevail, especially as diagnosis is often difficult and experience so limited (AU)

Some social factors related to control of diabetes mellitus in adult Jamaican patients

A population of 103 adult diabetic patients was interviewed at the Outpatient Clinic for diabetic patients of the University Hospital of the West Indies to examine the effects of severity of the illness and social variables, such as facilities at home

Foot ischaemia - a need for early rehabilitation

Ischaemia of the foot usually affects the elderly. The correlative study based on clinical data and pathological study has shown that maturity onset, diabetic foot ischaemia is usually due to severe arteriosclerosis. Because of the late presentation, most patients show end-stage obliterative vascular changes when first hospitalized which makes reconstructive surgery impossible. By early referral of all diabetic patients with infection, the number of below-knee or above-knee amputations can be reduced. Early prosthetic fitting is essential for the early rehabilitation of the patient. This paper was presented in part at the Commonwealth Caribbean Medical Research Council Meeting held at Bridgetown, Barbados in April, 1978. (AU)

Primary hyperparathyroidism at the U.H.W.I

Twenty-two cases of primary hyperparathyroidism were seen at the University Hospital of the West Indies during the twelve year period, January, 1966, to December 1977. Three groups of patients were identified depending on the main symptom complex at presentation: (1) renal calculi (2) bone pain and (3) symptoms of hypercalcaemia. All cases had elevated serum calcium levels. Twenty-one patients were found to have a single parathyroid adenoma at surgery and there was one case of hypersia. No cases with the multiple endocrine adenoma syndrome were identified. The pathology of the disease is described, aspects of diagnosis are outlined and methods of tumour localization are discussed (AU)

Haemophilus influenzae as an agent of urinary tract infection

Two cases of urinary tract infection due to H. Influenzae, an unusual urinary pathogen, are described. One was an adult female who presented with endometritis; the other was a male infant who had posterior urethral valves. Increased awareness of the potential existence of this at this site is a prequisite to diagnosis since it does not grow on the conventional media used for urine cultures (AU)

The metabolic effects of fenfluramine (ponderax) on obese diabetics - abstract

A trial of Ponderax in the management of refractory obesity in diabetic patients was conducted at the University Hospital of the West Indies over a 14-month period. The duration of the exhibition of the drug was six to fourteen months. Sixty-one patients were selected for the trial. They consisted of patients more than 25 percent in excess of ideal body weight in whom all methods of weight reduction had failed. Of the 61 patients 48 completed the trial, 13 defaulted or did not attend regularly enough to make assessment meaningful. There were 42 women and 6 men with an age range of 17-71 years. At the onset of the trial the drug therapy of the 48 patients was as follows: Diet alone - 8 patients, Diet + Diabinese - 15 patients, Diet + Metformin - 5 patients, Diet + Diabinese + Metformin - 12 patients, Diet + Insulin - 6 patients, Diet + Insulin +Metformin - 2 patients. All the patients had difficulty in the control of their diabetes and had 2-hour post prandial blood sugar levels in excess of 251 mgm percent on no treatment, and 153 mgm percent on their standard therapy. The dose of Ponderax given to the patients was as follows: 60 mgm/day - 5 patients, 80 mgm/day 10 patients, 100 mgm/day - 2 patients, 120 mgm/day - 22 patients, 140 mgm/day - 2 patients, 160 mgm/day - 8 patients. Of the 48 patients who completed the trail, the mean blood sugar level fell to 92 mgm percent on Ponderax and their usual therapy. Interestingly, in ten patients the dose of Diabinese had to be either reduced or stopped. In two patients taking insulin, there was a fall in the insulin requirements. Six patients taking metformin alone or metformin and diabinese had to habe a reduction in their dosage. 5 patient lost no weight, although blood sugar control improved, 10 patients lost less than 5 pounds, 9 patients lost less than 10 pounds, 9 patients lost less than 15 pounds, 4 patients loss less than 20 pounds and 11 patients loss more than 25 pounds. 25 percent of patients lost more than 10 pounds in weight. Weight loss lagged behind blood sugar and diabetic control by a mean of six to eight weeks. Serum Insulin levels were measured in 16 patients. In 11 of these patients there was a mean rise in the 2-hr. p.p. Insulin levels of 35u Units/ml. In 4 patients serum insulin levels fell by 24 units and in 1 patient there was no change. There was a considerable fall in blood pressure in all patients. The fall was greatest in the hypertensive and least in the normotensive patients. Side effects seen postural hypotension (36 patients), diarrhea (24), drowsiness (42), skin rash (1), depression (3). The 3 patients who suffered depression had a previous history of psychiatric disturbances and had stopped taking the Ponderax abruptly (AU)

Diabetic neuropathy: its incidence and relationship to control - abstract

The reported incidence of diabetic neuropathy varies from as little as 5 percent to as high a 90 percent in some areas. It has also been stated that most diabetic patients develop neurological and angiopathic lesions 8-10 years after the onset of their disorder. We analysed 1,500 diabetic patients attending the Diabetic Clinic between 1961 and 1972 at the University Hospital of the West Indies. The results showed that the sex ratio was 2.8:1 females: males and of the 1,500 patients examined 885 (59 percent) had symptoms suggestive of neuropathy. 568 (64.6 percent) of the patients were female and 297 (35.4 percent) were male. Thus there was a slightly higher incidence of neuropathy in the male than in the female diabetics. The relationship of neuropathy to the duration of the diabetes showed that 74.3 percent of the patients developed neuropathy within 10 years of the onset of diabetes and 12 percent presented with neuropathy. It is of interest to note that 62.8 percent of the male population of diabetic had impotence as an autonomic disorder, and that 5 percent of patients had bladder dysfunction. The complications of diabetic neuropathy leading to perforating ulcers and amputations are discussed. The relationship of diabetic neuropathy is presented (AU)