TREATMENT OF ASTHMA: Somatic and Psychiatric

Various conditions of the adrenal gland are amenable to surgical treatment. Removal of a pheochromocytoma is almost always indicated when the tumor is diagnosed. The results of extirpation have been excellent in cases in which patients were operated upon before the onset of chronic hypertension. Removal of the “nerve cell” tumors of the adrenal is indicated if metastasis cannot be demonstrated.Hypofunction of the adrenal cortex may be partially alleviated by the repeated implantation of pellets of desoxycorticosterone acetate. Hyperfunction of the adrenal cortex causes a variety of clinical manifestations depending upon which of the numerous hormones are affected. Removal of a cortical tumor alleviates these symptoms. These tumors are malignant in more than 50 per cent of cases, and recurrence is frequent. Bilateral hyperplasia of the glands rather than a tumor may be present. In such circumstances, resection of 95 per cent of the adrenal tissue is effective in controlling the symptoms of the disease.Total bilateral excision of the adrenals is, at present, under investigation as a means of treatment for a variety of conditions.     

无功能垂体瘤的术后评估

垂体瘤是常见的颅内良性肿瘤,患病率高,预后较好。在我国,其发病率仅次于胶质瘤和脑膜瘤。无功能垂体瘤约占垂体瘤22.5%,手术是其首选的治疗方式,以解除肿瘤的占位效应,减少对正常垂体组织的压迫。然而手术本身可能引起垂体功能减退,术后应对患者垂体功能的监测并予以处理。同时,由于手术及患者个体身体、心理原因,患者术后的生活质量也越来越受到重视。本文综述了无功能垂体瘤患者术后各内分泌轴功能的临床检测方法,以帮助临床医生对术后患者的垂体功能进行评估并重建;同时对术后患者生活质量的评估加以讨论,帮助指导患者恢复生理及心理健康。     

CUSHING'S SYNDROME

Sixteen cases of verified Cushing''s syndrome, and twelve cases of probable Cushing''s syndrome were reviewed and data on them were compared with various reports on Cushing''s syndrome in the literature.The diagnosis hinges upon a high index of suspicion, and one or several of the major criteria may be lacking.Ultimate establishment of correct diagnosis should be based largely on the clinical features, although stimulation and suppression tests may help to confirm a clinical diagnosis.In well-established clinical cases, with borderline laboratory confirmation, exploration may be justified, especially if tests fail to identify a specific cause.In cases of adrenal cortical tumor, all pathological tissue should be removed if possible, with great care to support and stimulate the remaining atrophic adrenal gland during and following operation.In cases of bilateral adrenal cortical hyperplasia, the problem is one of how much to remove. At present most investigators advocate radical subtotal resection, leaving less than 10 per cent of one side.     

Leucine-induced hypoglycemia in a family: effect of diphenylhydantoin, oxprenolol, and diazoxide

The present report describes a mother and 2 children with leucine-induced hypoglycemia (LIH). Hypoglycemic episodes following high-protein meals first appeared at age 4-7 months. Leucine-stimulation tests triggered marked hyperinsulinism and hypoglycemia in the children and a milder but abnormal response in the mother. To evaluate the therapeutic effects and to study the mechanism of hyperinsulinism in LIH, the leucine test was repeated under treatment with diphenylhydantoin, oxprenolol (a beta-blocker), and diazoxide. Diazoxide abolished hyperinsulinism; diphenylhydantoin did not affect the response to leucine; and oxprenolol, tested in the mother only, increased hyperinsulinism and hypoglycemia. Our results indicate that LIH is an autosomal dominant disorder; LIH may persist into adulthood with milder clinical symptoms and chemical response to leucine; diazoxide is the treatment of choice in LIH. Considering the effects of the three agents on stimulated release of insulin, it is concluded that leucine triggers hyperinsulinism by a mechanism different from that of glucose and beta-adrenergic receptors.     

Deoxycorticosterone-Producing Adenoma Concomitant with Aldosterone-Producing Microadenoma: A Challenging Combination

ObjectiveTo describe the challenging case of a 59-year-old male with a deoxycorticosterone (DOC)-producing adrenal adenoma concomitant with an aldosterone-producing microadenoma.MethodsWe measured the patient’s aldosterone and progesterone levels during adrenal venous sampling (AVS). The steroidogenic enzyme expression was studied with in situ hybridization (ISH). Steroids profiles were determined in the peripheral serum obtained before and after the operation, as well as in the main adrenal tumor.ResultsThe patient was diagnosed with primary aldosteronism (PA) based on typical clinical findings. He had an adrenal tumor located at the lower pole of the left adrenal gland. The aldosterone concentration in the adrenal vein proximal to the adrenal tumor was higher than that of the ipsilateral adrenal vein distal to the tumor during the AVS. Progesterone was only elevated in the adrenal vein proximal to the tumor, suggesting that the tumor produced steroids other than aldosterone. The postoperative findings revealed that the main tumor was accompanied by 2 microadenomas. The main adrenal tumor was diagnosed as a DOC-producing adenoma, and one of the microadenomas was diagnosed as aldosterone-producing based on the ISH and the determination of the steroid profiles.ConclusionsConcomitant PA masked the key findings of a DOC-producing tumor; the suppression of aldosterone in this patient. Multiple sampling in the adrenal vein considering the location of the adrenal tumor provided a clue to the diagnosis. Progesterone measurement during AVS is easy and may be useful in diagnosing rare adrenal tumors that produce intermediate products in adrenal steroid biosynthesis. (Endocr Pract. 2014;20:e171-e175)     

EXTRACUTANEOUS MANIFESTATIONS OF KAPOSI'S SARCOMA—A Systemic Lymphoblastoma

Kaposi''s sarcoma may affect any system of the body. Serious difficulties occur only when the heart, lungs or gastrointestinal tract are affected. Usually, involvement in other viscera causes no clinical symptoms.This neoplasm is thought to be a low-grade lymphoblastoma. This idea of relationship is based on clinical and histologic association of Kaposi''s sarcoma with the lymphoblastomas more commonly than would be anticipated from the rarity of the conditions under consideration. This concept is strengthened by the occasional seeming mutation of Kaposi''s sarcoma into a lymphoblastoma. The associated reticuloendothelial hyperplasia in Kaposi''s sarcoma is another link in the evidence of relationship.     

The clinical spectrum of phosphomannose isomerase deficiency,with an evaluation of mannose treatment for CDG-Ib

Phosphomannose isomerase (PMI) deficiency or congenital disorders of glycosylation type Ib (CDG Ib) is the only CDG that can be treated. Despite variable severity leading to dramatically different prognoses, clinical presentation is relatively homogeneous with liver and digestive features associated with hyperinsulinism and inconstant thrombosis. A feature of CDG is that coagulation factors are decreased. In our experience, mannose given orally at least 4 times per day not only transformed lethal CDG Ib into a treatable disease, but also improved the general condition and digestive symptoms of all reported patients but one. Liver disease, however, still persisted. Heparin can be used as an alternative to mannose in certain patients, particularly in the treatment of enteropathy.     

甲状腺肿瘤细胞核DNA含量测定对病理诊断的意义

应用真彩色医学图像分析技术, 对９０ 例甲状腺肿瘤（其中甲状腺腺瘤１０ 例, 不典型腺瘤１５ 例,乳头状腺癌２５ 例, 滤泡癌１５ 例, 髓样癌１５ 例, 未分化癌１０ 例） 细胞核ＤＮＡ含量进行了分析。结果显示,甲状腺腺瘤组与各型甲状腺癌比较均有显著性差异（Ｐ＜ ００１）,甲状腺腺瘤组同不典型腺瘤组比较无统计学意义（Ｐ＞ ００５）。甲状腺癌随组织分化程度的不同, ＤＮＡ 含量明显增加, 多为高倍异倍体细胞, ＤＮＡ直方图明显右移, 峰值主要位于≥５Ｃ处; 甲状腺腺瘤组ＤＮＡ含量较低, 多为低倍整倍体细胞, ＤＮＡ 直方图峰值位于２Ｃ－ ４Ｃ处; 不典型腺瘤组ＤＮＡ含量介于上述二者之间, ＤＮＡ 直方图逐渐右移。表明ＤＮＡ倍性程度与肿瘤的增殖程度呈正相关, 高倍异倍体细胞随肿瘤恶性程度的增高而增多。作者认为ＤＮＡ原位图像定量分析可为甲状腺肿瘤的诊断、分级及早期发现癌变趋势提供一个可靠的参考指标     

THE TREATMENT OF MEDIASTINAL TUMORS

Because there can be no certainty of diagnosis without it, thoracotomy is indicated in cases in which there is roentgenographic evidence of mediastinal tumor. “Watchful waiting” is considered unwise because the tumor may be malignant when first observed or may later undergo malignant change. Even if the lesion is and remains benign, an increase in the size of the mass may cause obstructive pressures necessitating operation ultimately. Moreover, infection and inflammation may develop at the site during prolonged observation, making operation more difficult.The hazards of thoracotomy, which have been greatly reduced in the last few years, are less than the hazards of waiting.     

恶性消化道肿瘤患者的治疗进展

姑息治疗是是世界卫生组织(WHO)定义的癌症基本处理原则之一,消化道恶性肿瘤的姑息治疗与患者发病率、死亡率提高和治疗成本增加密切相关。根据患者指征将急诊或姑息性治疗分为两类。包括肿瘤相关并发症与其他在治疗过程中可能出现的症状。虽然存在非手术、内镜及介入治疗方式多种治疗方法,但手术治疗仍是应用最多且最有效的方法。患者治疗方案的选择需要考虑多种因素,包括患者总体预后、体力状态等。西医治疗对改善症状、维持并延长生命更有效。而中医治疗对改善患者症状,加速疾病好转,提高患者生活质量的作用也非常重要。     

Unexpected Clinical Course During Treatment of a TSH-Secreting Pituitary Adenoma

ObjectiveTo report the rare occurrence of a patient with thyrotropinoma that transitioned into a secretory thyro-somatotroph adenoma during medical treatment with somatostatin analogue.MethodsWe report the case of a patient with a thyrotroph pituitary adenoma who developed de novo evidence of growth hormone cosecretion following one year of successful medical treatment.ResultsA 78-year-old woman was diagnosed with a thyroid stimulating hormone (TSH) secreting pituitary macroadenoma (TSHoma) based on classical clinical and biochemical features. There was no clinical or biochemical evidence of growth hormone (GH) cosecretion. She declined surgical resection and was treated with primary medical therapy, octreotide long acting repeatable (LAR), to which she had an antitumor and antisecretory response; however, following 12 months of successful medical treatment she developed de novo hypersecretion of growth hormone despite involution of the tumor mass. TSH-secreting pituitary adenomas may rarely become plurihormonal during apparently successful medical treatment. This may represent an unusual form of secondary resistance to somatostatin analogue or the rarer phenomenon of tumor transformation into a secretory thyro-somatotroph adenoma.ConclusionThe unexpected clinical course of this case highlights the need for careful long-term surveillance in patients with TSH secreting pituitary adenomas. (Endocr. Pract. 2013;19:e88-e91)     

ANALYSIS OF ABSENTEEISM IN INDUSTRY

There are many nonmedical factors that contribute to employee absenteeism in industry. An employee''s total life situation or total environment may be a causative factor in excessive “sick absenteeism.” In many instances the cure for “abnormal” sickness absenteeism is within the province of supervisory personnel, who should look upon abuse of sick leave benefits among employees as morale problems and as evidence of possible maladjustment to the demands of the job or the industry. There are, however, many problems in mental and physical health affecting absence rates in which preventive psychiatry and medicine can make greater contributions. Even truancy and malingering may sometimes be conditions requiring professional medical care.The role of a private physician in determining and certifying the true state of a patient''s health is a most important one economically to industry and the community. The total problem of absenteeism for sickness, as it exists in industry today, points up the need for the most effective cooperation and communication possible between industrial and private physicians. Since no more than 25 per cent of the total work force is employed in industries having in-plant medical programs, the burden of responsibility for the control of absenteeism for sickness rests mainly with private practitioners.     

Psychological impact of adjuvant chemotherapy in the first two years after mastectomy.

Psychological symptoms were assessed over two years in a randomised trial of three forms of treatment given to women after mastectomy for stage II breast cancer. The treatments were: three weeks'' radiotherapy; one year''s adjuvant chemotherapy with cyclophosphamide, methotrexate, and 5-fluorouracil; and radiotherapy followed by chemotherapy. Analysis of the results on an intention to treat basis showed no substantial differences in depression or anxiety among groups at one, three, or six months after the operation. At 13 months, however, patients who had been allocated chemotherapy had significantly more symptoms, especially depression, than control patients treated with radiotherapy alone. Conditioned reflex nausea and vomiting increased considerably during the second six months of chemotherapy and persisted for up to a year afterwards. The psychological morbidity of adjuvant chemotherapy could be substantially reduced if courses of treatment were restricted to about six months.     

DIHYDROGENATED ALKALOIDS OF ERGOT IN TREATMENT OF PERIPHERAL VASCULAR DISEASES

The dihydrogenated alkaloids of ergot, dihydroergocornine (DHO 180) and an equal mixture of dihydroergocornine, dihydroergocristine and dihydroergokryptine known as CCK 179 have been found to be therapeutic adjuncts in the medical treatment of peripheral vascular diseases. Their action is primarily that of adrenergic blockage, although depression of the brain stem is to be considered.The mixture of alkaloids (CCK 179) was found to be more effective than a single alkaloid, dihydroergocornine (DHO 180). A greater number of patients were benefited, relief of symptoms was greater and the dosage easier to establish. A favorable therapeutic response of clinical significance with the mixture was obtained in approximately 60 per cent of all cases investigated. It was of greater benefit in the organic occlusive diseases, where a larger percentage of favorable responses was obtained than in the purely vasospastic disorders.Orally and subcutaneously, CCK 179 exhibited vasodilating properties which compared favorably with paravertebral and peripheral nerve block, reflex heat, alcohol and sympathectomy. Surface temperatures were elevated, oscillometric readings increased and tolerance to cold increased in a statistically significant number of cases. Effects of sympathectomies were frequently enhanced. Following subcutaneous administration, increased surface temperatures of the extremities of one to two hours'' duration were obtained in 90 per cent of all cases.Paresthesias, nocturnal cramps and intermittent claudication were improved. A sense of well-being was occasionally exhibited.Blood pressure and pulse rates were rarely affected. Blood pressure was lowered in normotensive patients, but was rarely changed in hypertensive patients.Symptoms of overdosage appeared after two to three months of continuous therapy. These were manifested by lowered surface temperatures, decreased tolerance to cold, return of intermittent claudication and occasionally by vague general discomfort. These symptoms disappeared on cessation of therapy. Improvement frequently followed. In only one case was there an immediate reaction. Following subcutaneous administration of CCK, blood pressure and pulse rate increased and oscillometric readings and surface temperatures decreased.Frequent courses of therapy with interruptions were necessary for maintenance of improvement.     

Operations on the adrenal glands

Various conditions of the adrenal gland are amenable to surgical treatment. Removal of a pheochromocytoma is almost always indicated when the tumor is diagnosed. The results of extirpation have been excellent in cases in which patients were operated upon before the onset of chronic hypertension. Removal of the "nerve cell" tumors of the adrenal is indicated if metastasis cannot be demonstrated. Hypofunction of the adrenal cortex may be partially alleviated by the repeated implantation of pellets of desoxycorticosterone acetate. Hyperfunction of the adrenal cortex causes a variety of clinical manifestations depending upon which of the numerous hormones are affected. Removal of a cortical tumor alleviates these symptoms. These tumors are malignant in more than 50 per cent of cases, and recurrence is frequent. Bilateral hyperplasia of the glands rather than a tumor may be present. In such circumstances, resection of 95 per cent of the adrenal tissue is effective in controlling the symptoms of the disease. Total bilateral excision of the adrenals is, at present, under investigation as a means of treatment for a variety of conditions.     

Metanephric adenoma.

In a recent survey of more than one hundred childhood renal tumors in our Laboratory files, we identified a unique case characterized by an unusual degree of differentiation and cell maturity. Histologically this case was notable for an orderly array of small and uniformly-packed tubules with a rosette-like configuration. The nuclei were oval, smooth and of a bland appearance. Mitoses were absent. Many glomerular figures were intermingled. This renal tumor picture is somewhat different from that known as tubular Wilms' tumor because of the well-differentiated adenomatous pattern and the absence of any blastema. The term metanephric adenoma is suggested for this tumor, which may represent the benign counterpart of Wilms' tumor.     

Prolactin-Secreting Pituitary Adenomas

Prolactin-secreting pituitary adenoma is a common cause of gynecologic problems that include oligomenorrhea, infertility, amenorrhea and galactorrhea. Diagnosis requires a combination of endocrine testing and radiologic evaluation. The diagnosis of macroadenomas is usually straightforward and these large tumors may be associated with mass effects such as severe headache, nerve palsies or visual changes. Microadenomas may be more subtle in presentation, and the diagnosis of hyperprolactinemia without radiologic evidence of a tumor frequently is problematic. The management of prolactin-secreting adenoma remains controversial, with no clear consensus or indication for surgical versus medical treatment. Surgical intervention is a realistic option for those patients who have access to an experienced neurosurgeon and who have tumor characteristics that offer a reasonable hope for cure. Many questions remain to be answered, including the cause, natural history of development and the optimum treatment for individual cases.     

PRIMARY CARCINOMA OF THE DUODENUM

As the only hope for patients with malignant disease of the duodenum depends upon early diagnosis and prompt and adequate operation, suspicion must be alert even though the condition is relatively rare. The incidence is highest in persons between 50 and 70 years of age, and two or three times as high in males as in females.The onset is insidious. The patient usually gives a history of fairly good health and no other related symptoms until about a year before diagnosis. Early symptoms are loss of appetite, loss of weight, and moderate pain in the right upper quadrant of the abdomen, sometimes associated with epigastric fullness which is relieved by belching. Vomiting and constipation are late symptoms. There may be occult blood in the stools, moderate anemia in some cases, and frequently jaundice.The radiological findings are irregularity of the mucosal pattern in the region of the tumor and often constriction of the involved portion of duodenum.A report is made herein upon four cases of primary carcinoma of the duodenum observed at one hospital in a period of only a little more than two years.     

The genetics of neonatal hyperinsulinism

Congenital hyperinsulinism (CHI) is the most important cause of persistent hypoglycaemia in the neonate and infant. It is a clinically and genetically heterogeneous entity. The clinical heterogeneity is manifested by severity ranging from extremely severe life-threatening disease to very mild clinical symptoms which may even be difficult to identify. Furthermore, clinical responsiveness to medical and surgical management is extremely variable. Two histopathological forms have been described: a diffuse form of CHI and a focal form of CHI. Recent discoveries have begun to clarify the molecular aetiology of the disease and therefore the mechanisms responsible for its clinical heterogeneity are becoming clearer. Mutations in four different genes have been identified in patients with CHI. Most cases are caused by mutations in genes coding for either of the two subunits of the beta-cell K(ATP) channel (ABCC8 and KCNJ11). In the diffuse form of CHI, the hyperinsulinism is due to a recessive mutation of both alleles of these genes (rare dominant mutations have been described). In the focal form of CHI, two events intervene: first, the inheritance of a paternal ABCC8/KCNJ11 mutation; second, the focal reduction to homozygosity of the mutation during pancreatic development by a localized loss of the maternal 11p15 region. Others cases of CHI are due to rare mutations in the beta-cell enzymes glucokinase (only one family described) and glutamate dehydrogenase in hyperammonaemia-associated hyperinsulinism. However, in as many as 50% of cases, no genetic aetiology has yet been identified.     

RETROPERITONEAL TUMORS IN CHILDREN—Roentgen Diagnosis

A study was undertaken to determine whether there are any features of retroperitoneal tumors in children that might be demonstrated on roentgenograms to aid in identifying them preoperatively. Study was limited to Wilms'' tumor of the kidney and neuroblastoma.Calcification was found in 57 per cent of the neuroblastomas and in only 12 per cent of Wilms'' tumors. Calcifications in neuroblastomas differed from those in Wilms'' tumors. Calcification in neuroblastoma was more frequent in older children than in the younger ones.The kidney was frequently displaced by both types of tumor. However, the neuroblastoma always displaced the kidney downward, or downward and slightly outward.In most instances, the Wilms'' tumor also displaced the kidney downward and outward, but in some instances upward and medially. This, of course, depended upon the site of origin of the tumor.There was a distortion of the intrarenal structures in 75 per cent of the cases of neuroblastoma and in 71 per cent of the cases of Wilms'' tumor.