Recurrent Cushing's syndrome due to recurrent adrenocortical tumor--fragmentation or tumor in ectopic adrenal tissue?

A 33-yr-old woman was found to have Cushing's syndrome due to a left adrenal cortical tumor. The tumor and the surrounding adrenal gland were removed intact and in toto. Four years later, she noticed recurrent symptoms of Cushing's syndrome, and 6 yr postoperatively, an adrenal tumor was demonstrable on computed tomography. Fourteen years after the initial procedure, a left adrenal tumor, presumably arising in ectopic adrenal tissue, was removed with relief of her symptoms of Cushing's syndrome. The site and functional capacity of ectopic adrenal tissues are reviewed.     

A young woman with metabolic acidosis and recently discovered IDDM without ketonuria. A rare autoimmune (?) combination of hypothyroidism, diabetes mellitus and distal renal tubular acidosis

A case of a 29-year-old woman with a multiple autoimmune disorder is reported. She had a history of hypothyroidism since the age of 18. She was admitted to hospital due to hyperglycaemia. At admission she had hyperglycaemia, metabolic acidosis, but no urinary ketone bodies. Further laboratory studies revealed that the acidosis was due to distal renal tubular acidosis rather than diabetic ketoacidosis (although the patient had type 1 diabetes mellitus). Blood tests revealed antibodies to glutamic acid decarboxylase (GAD-65; associated with type 1 diabetes mellitus), thyroid and adrenal tissue, and gastric parietal cells. The patient had not developed pernicious anaemia or Addison's disease. The multiple positive antibody titres in this patient indicate that the diabetes, hypothyroidism and distal renal tubular acidosis are part of an autoimmune syndrome.     

Bilateral pheochromocytomas with von Hippel-Lindau's disease: a case report

A case of bilateral pheochromocytomas with von Hippel-Lindau's disease (VHL) is reported. A 46-year-old woman who had hyperglycemia was admitted to our hospital because of abdominal tumors. Her elder sister and niece had been diagnosed as VHL. Ultrasonography, CT, and MRI revealed bilateral adrenal tumors. Noradrenaline levels in serum and urine were elevated and 131I-MIBG scintigraphy showed accumulation in bilateral adrenal glands. Moreover, she had bilateral renal cysts and cerebellar hemangioblastoma. Bilateral adrenalectomies were performed and pathological diagnosis was pheochromocytoma. This is the seventh case of bilateral pheochromocytomas with VHL reported in Japan.     

Postpartum thyroiditis and Basedow disease

BACKGROUND: The postpartum period is characterized by a rebound in autoimmunity secondary to immune tolerance induced by pregnancy, creating favorable conditions for flare up of Graves' disease or autoimmune thyroiditis. Postpartum thyroiditis is a recognized clinical entity. CASE REPORT: Six years after onset of Graves' disease treated with antithyroid drugs, a 25-year-old woman had a high serum level of antithyroperoxidase antibodies a few months before she became pregnant. Six weeks after delivery, she developed signs of hyperthyroidism and goiter. The diagnosis of postpartum thyroiditis was retained. Her condition regressed spontaneously to euthyroidism then hypothyroidism. DISCUSSION: The therapeutic options involved underline the importance of distinguishing between Graves' disease and postpartum thyroiditis. The diagnosis of postpartum thyroiditis is based on history taking, clinical findings, and laboratory tests, especially isotope uptake.     

Autoimmune oophoritis. An incidental finding

A case of autoimmune oophoritis is reported. A 41-year-old woman had a total abdominal hysterectomy and bilateral salpingo-oophorectomy for menorrhagia, polymenorrhoea and cystic ovaries. The diagnosis of autoimmune oophoritis was not suspected clinically, and was an unexpected histological finding in the ovaries. The gross and histological appearances of this rare condition are described, and the lymphoid infiltrate characterised by immunocytochemistry. Recognition of this condition by pathologists is important, as there is an associated risk of developing other autoimmune disease, even some years later, necessitating close patient follow-up. In this case serum auto-antibodies to adrenal cortex were detected, indicating a subsequent risk of Addison's disease.     

The prevalence of Addison's disease in Coventry, UK

The prevalence of Addison's disease (chronic adrenal failure) has not been widely investigated and is usually given as 39 in a million. We conducted a prevalence study using a postal survey of general practitioners in Coventry. Three quarters (139/188) replied, representing 79/85 (93%) of the practices. Thirty cases of Addision's disease were found from a total patient list of 323852, of which a third were tuberculous in origin and two-thirds non-tuberculous (12/30 autoimmune, 8/30 unclassified). We conclude that Addison's disease is 2.4 times more common than previously reported. The tuberculous group was older, 65 vs 52 years (p < 0.05), and had had the disease for longer than the non-tuberculous group, 20 vs 12 years (p < 0.05). There was no significant difference in the age at diagnosis.     

Successful treatment with plasmapheresis, cyclophosphamide, and cyclosporin A in type B syndrome of insulin resistance. Case report

CASE HISTORY: A woman born in 1949 was diagnosed in 1990 with systemic lupus erythematosus. She was treated with prednisolone, and < 1 year later she presented with marked hyperglycemia. Large doses of insulin were given four times per day. Even though the patient was thin (BMI 17.4 kg/m2), very little improvement was seen. INVESTIGATIONS AND TREATMENT: Serum insulin levels were high, and a euglycemic clamp investigation confirmed severe insulin resistance. The patient's serum contained insulin receptor antibodies inhibiting insulin binding, and thus the patient had a type B syndrome of insulin resistance. After diet and exercise, glycemic control stabilized and insulin treatment was withdrawn. However, in late 1993 she was in a catabolic and hyperglycemic state even though prednisolone doses were increased and azathioprin was added. In early 1994 she was treated with plasmapheresis and cyclophosphamide i.v. Subsequently, cyclosporin A was started as a maintenance therapy in addition to azathioprin. There was a rapid and sustained clinical improvement. Since late 1994 and onward, there is no sign of diabetes or glucose intolerance and there are no demonstrable insulin receptor antibodies in the patient's serum. DISCUSSION: Severe type B insulin resistance may respond favorably to treatment with plasmapheresis and cyclophosphamide followed by cyclosporin A in combination with azathioprin.     

Massive adrenal hemorrhage secondary to metastasis of lung cancer

Hemorrhagic adrenal metastasis from lung cancer is extremely rare, although adrenal involvement is common in widely disseminated cancer. We report a case of massive adrenal hemorrhage secondary to metastasis of lung cancer. A 47-year-old female was treated by left upper lobectomy and mediastinal lymph node resection for an adenocarcinoma with intrapulmonary metastasis in the left upper lobe. Eight months later, she presented with right flank and back pain, and abdominal ultrasonography and computed tomography showed a right solitary adrenal tumor with massive hemorrhage. The tumor was not resectable and partially responded to chemotherapy. A massive adrenal hemorrhage, secondary to metastasis of lung cancer, presents with nonspecific clinical signs and symptoms. In lung cancer patients with an acute flank or back pain, hemorrhagic adrenal metastasis should be considered in the differential diagnosis.     

Idiopathic hypoparathyroidism--a rare disease?

Five patients with nonfamilial idiopathic hypoparathyroidism were observed in a peripheral hospital. There was no association with other autoimmune disorders such as hypothyroidism, adrenal insufficiency or pernicious anemia. Only in one patient with tetany was the diagnosis clinically obvious; all the others presented with unusual clinical symptoms. These manifestations of chronic hypocalcemia are presented, as well as the diagnostic workup and therapeutic management. We suggest that idiopathic hypoparathyroidism is not a very rare disease, but one which is often missed because of the unusual clinical picture.     

Adrenalectomy for metastatic disease to the adrenal glands

A policy of supportive treatment is frequently adopted for patients with metastatic disease to the adrenal glands. This study reports an experience with adrenalectomy for adrenal metastasis. Between 1983 and 1993, adrenalectomy was performed in 52 patients for metastasis to the adrenal glands. Survival was calculated by the Kaplan-Meier method and compared with the log rank test. Primary tumour sites included kidney (n = 15), lung (n = 11), colon (n = 7), unknown (n = 5), stomach (n = 3), melanoma (n = 3) and other (n = 8). Adenocarcinoma (69 per cent) was the most common histological cell type. Thirty-two patients were asymptomatic on initial evaluation. Symptomatic adrenal pain relief was achieved in 11 of 13 patients. Overall survival rates were 73 per cent at 1 year and 40 per cent at 2 years. Patients with potentially curative resection had better survival than those who had a palliative procedure. Patients with adrenal metastases due to adenocarcinoma had improved survival compared with that in those with other histological cell types. Although long-term survival is generally poor, highly selected patients with adrenal metastasis (symptomatic disease or adenocarcinoma) may benefit from surgical resection.     

Evans'' syndrome associated with Graves'' disease

A 36-year-old woman who had had Graves' disease for 6 years was admitted with severe thrombocytopenia. Evans' syndrome was diagnosed. The patient's family history showed multiple cases of Graves' disease but no cases of Evans' syndrome. Both conditions in this patient improved with corticosteroid and thiamazole therapy. Several autoimmune antibodies were found, but a common autoimmune mechanism was not clearly shown. Although the combination of Graves' disease and Evans' syndrome had not occurred previously in her family, genetic factors may play an important role in the pathogenesis of both conditions.     

Laceration of a benign adrenal adenoma mimicking a splenic rupture

Lacerations of adrenal tumors are very rare events and have been described for myelolipoma, pheochromocytoma, and cortisol-producing adenoma. We report on a patient who was admitted with suspected splenic rupture. Computed tomography showed a mass 14 cm in diameter adjacent to the spleen, but selective angiography revealed blood supply by the left suprarenal artery. A ruptured adrenal tumor was therefore diagnosed and resected. No hormone production was detected. Histologically a benign adrenal adenoma was found. Frequency, diagnosis and therapy of adrenal masses are discussed.     

Diabetes mellitus-associated glycogen storage hepatomegaly: report of a case and review of the Japanese literature

A huge hepatomegaly was seen in a 30-yr-old female diabetic who was treated with high dose of insulin for her uncontrollable food ingestion. The liver function at the peak of the hepatic enlargement showed a moderate increase of transaminases, alkaline phosphatase, and gamma-glutamyl transpeptidase. The histology of the enlarged liver revealed PAS-positive granules in enlarged hepatocytes, indicating the presence of massive glycogen storage. On admission, she was maintained under a calorie-restricted diet and received approximately 15 to 20 units per day of insulin supplement. At one month after admission, a marked shrinkage of her enlarged liver and restoration of normal liver function were observed concomitantly with the return of fair control of her blood sugar levels. One year later, she had an episode of diabetic ketoacidosis which subsequently was treated with a continuous low-dose infusion of insulin; however, she showed neither hepatomegaly nor liver dysfunction during this episode. There have been 20 cases reported of Japanese diabetics with marked hepatomegaly, in whom the vigorous treatment of diabetic ketoacidosis with insulin seemed to be a trigger of the enlarged liver. This has occurred mostly in patients with insulin-dependent diabetes mellitus. We present a case of non-insulin-dependent diabetes mellitus with glycogen storage hepatomegaly, presumably due to excessive insulin supplements. This suggests that glycogen storage hepatomegaly in diabetics may not be only due to an acute restoration from diabetic ketoacidosis, but may also be due to an overinsulinization in an attempt to maintain a euglycemic condition in spite of excess food intake.     

Kinetics of populations of enterocytes of experimental tumors of the large intestine in rats

To determine whether somatostatin, an inhibitor of glucagon and growth hormone secretion, might be useful as an adjunct to insulin the management of diabetic hyperglycaemia, seven insulin-requiring diabetic men were given somatostatin (100 microgram/h, IV) continuously for 3 days after their diabetes had been treated intensively by diet and insulin on a metabolic ward. During infusion of somatostatin and despite reduction in average insulin dose exceeding 50%, there was improvement in diabetic control as assessed by postprandial hyperglycaemia, 24-h glycosuria and the average daily serum glucose level and its fluctuation; when somatostatin was discontinued, but insulin doses held constant, diabetic control rapidly worsened. No adverse effects were observed. These results indicate that somatostatin plus insulin can be a more effective regimen than insulin alone in controlling diabetic hyperglycaemia. A longer acting and more selective somatostatin preparation may prove useful as an adjunct to insulin in the management of diabetes.     

Metabolic derangements in nonmodulating hypertension

A positive association exists between insulin resistance, dyslipidemia, and hypertension, specifically salt-sensitive hypertension. A subgroup of salt-sensitive normal and high renin hypertensives called nonmodulators (NM) manifest an inability to modulate the adrenal and renal blood flow responses to a change in dietary sodium. Therefore, we tested the hypothesis that the NM subgroup would be insulin resistant and dyslipidemic when compared with normal and high renin hypertensives, in whom modulation is intact (M). Forty-six nondiabetic hypertensive individuals were evaluated and their modulation status defined by either renal or adrenal criteria. Fasting blood was drawn for measurement of several metabolic factors. Since the NM group had a greater body mass index (BMI) it was subdivided into a "lean" subgroup that matched the BMI of the M group. The fasting insulin levels in both the total NM and lean NM groups was significantly higher than in the M group (P = .013 and .04, respectively). There were no differences in age, blood pressure, or plasma/serum levels of glucose, triglycerides, total cholesterol, or potassium. NM had elevated fasting insulin levels compared to M, compatible with an insulin resistant state, but this insulin resistance are dissociable in the hypertensive population.     

Encephaloduroarterio-synangiosis in a child with sickle cell anemia and moyamoya disease

We report a black girl with sickle cell anemia. On prophylactic exchange transfusion protocol, she experienced cerebrovascular accidents at 3 and 3.5 years of age, both associated with transient right hemiparesis. At 7.5 years of age, she presented with a partial motor seizure and a left hemiparesis. A cerebral angiogram demonstrated stenosis at the origins of both middle and anterior cerebral arteries bilaterally with extensive basal collateralization. She underwent uncomplicated bilateral encephaloduroarteriosynangiosis (EDAS) procedures using both superficial temporal arteries. At age 9 years, the patient presented with a severe headache and tunnel vision secondary to a stenosis of both posterior cerebral arteries. She underwent bilateral EDAS procedures using both occipital arteries. No complication was encountered. Postoperative cerebral angiogram demonstrated impressive neovascularity at the sites of all four EDAS procedures. Different treatment options of moyamoya disease are discussed.     

Long-term selective IgG immuno-adsorption improves Rasmussen's encephalitis

We report that long-term selective immunoglobulin G immunoadsorption by protein A (PAI) improved seizure frequency and neuropsychological deficits in a 16-year-old patient with severe treatment-resistant Rasmussen's encephalitis (RE). Clinical improvement correlated with reduction of antiglutamate receptor 3 antibodies. The efficacy of PAI in our patient supports the autoimmune hypothesis of RE and suggests its application to avoid, or at least delay, functional hemispherectomy in selected cases.     

Liver disease in polyglandular autoimmune disease type one: clinicopathologic study of three patients and review of the literature

We report the findings from liver biopsies of three patients with polyglandular autoimmune disease type 1. The livers in two patients had histologic features of chronic hepatitis that eventuated in bridging fibrosis and cirrhosis over a period of several years. Nonspecific lobular and portal tract inflammation was present in the liver biopsy of the third patient. Although these patients did not have liver-specific autoantibodies, the liver disease in polyglandular autoimmune disease type 1 possibly represents an expression of autoimmune hepatitis.     

Plasma insulin levels in coronary artery disease

Seventy two consecutive patients without a history of diabetes and normal fasting plasma glucose were included in this study of insulin levels. Standard oral glucose tolerance test with 75 gm glucose and fasting and two hour insulin levels were estimated in all patients. Coronary artery disease (CAD) was confirmed or excluded by selective coronary arteriography. In 20 patients, CAD was diagnosed by electrocardiographic (ECG) and clinical evidence of earlier myocardial infarction. Mean fasting plasma insulin was 31.40 +/- 22.2 IU/dl in the CAD positive and 32.3 +/- 13.6 IU/dl in the CAD negative group. The mean two hour plasma insulin was 274.6 +/- 301.1 IU/dl in the CAD positive and 104.8 +/- 74.9 IU/dl in the CAD negative group (p < 0.04). Two hour plasma insulin levels were significantly higher in patients with atherosclerotic coronary artery disease. It is concluded that the estimation of a two hour plasma insulin level after 75 gm of glucose load, could help differentiate CAD from normals.     

The polycystic ovary syndrome associated with ovarian tumor

Case report of a girl with PCOD (polycystic ovarian disease) and Sertoli-Leydig ovarian tumour. A sixteen-year old girl was clinically, endocrinologically, echosonographically and laparoscopically examined, and polycystic ovarian disease was diagnosed. After a two-year period she was reexamined: normal adrenal function was confirmed and left ovarian tumour was discovered echosonographically. Therefore, she was operated on and adnexectomy of the left ovarian tumour and biopsy of the right ovary were done. The histopathologic analysis revealed the Sertoli-Leydig tumour of the left ovary and polycystic degeneration of the right ovary. In conclusion, because of the greater frequency of ovarian tumours in women with PCOD, the permanent follow-up of women with PCOD is necessary.