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At least 1,153 records · Page 64Linked to original sources
PubMed1996

Pituitary Corticotroph Adenoma in a Woman with Long-Standing Addison's Disease: A Histologic, immunocytochemical, Electron Microscopic, and In Situ Hybridization Study.

A 64-year-old woman with long-standing Addison's disease owing to destructive immune adrenalitis presented with hyperpigmentation and progressively increasing blood adrenocorticotrophic hormone (ACTH) levels. Magnetic resonance imaging demonstrated a pituitary microadenoma, which was removed by transsphenoidal surgery and investigated by histology, immunocytochemistry, transmission electron microscopy, and in situ hybridization (ISH). The morphologic studies revealed a basophilic, periodic acid-Schift (PAS)-positive pituitary adenoma immunoreactive for ACTH and B-endorphin and in several cells for a-subunit. By transmission electron microscopy, the tumor was a densely granulated corticotroph adenoma, which, by ISH, expressed pro-opiomelanocortin (POMC) mRNA. The lack of corticotroph hyperplasia in the nontumorous adenohypophysis was an intriguing finding. Corticotroph adenomas in patients with long-standing Addison's disease were very rarely examined by morphology. Our report includes a detailed morphologic analysis and is the first demonstration of POMC mRNA in the tumor cells using ISH. The question of whether the adenoma was related to increased secretory activity secondary to protracted hypocorticism or developed independently unrelated to deranged endocrine homeostasis remains unresolved. The lack of corticotroph hyperplasia in the nontumorous adenohypophysis favors the interpretation that hypothalamic stimulation played no major role in adenoma formation in our case.

Journal Article↗
PubMedDate not supplied

Congenital adrenal hyperplasia: experience at intersex clinic, AIIMS.

During 1981-88, 63 cases of female pseudohermaphroditism (FPH) were seen at the Intersex clinic at AIIMS, of whom 34 (54%) were diagnosed as due to congenital adrenal hyperplasia (CAH). Though ambiguity was present at birth in most cases, only one child was brought immediately after birth, while 14 presented after one year. Family history of affected siblings and fetal wastage was present in 10. Salt wasting symptoms were present in 13 (38.2%), evidence of early virilization in 10 (29.4%) and generalised hyperpigmentation in 7 (20.6%). Clitoromegaly was present in 30 children with labial fusion in 10 and scrotalisation of labia in 6. The urogenital opening was single in 25 (73.5%). Buccal smear was positive for sex chromatin in 19. Chromosomal pattern showed 46 XX in 33. Dyselectrolytemia was present in 16 children. Bone age was advanced in all. Adrenal hyperplasia could be documented in 3 on CT scan. All the girls were put on hydrocortisone or prednisolone, and fluodrocortisone was given only to children with salt wasting CAH. Children with CAH are being brought to medical attention much too late and investigative and therapeutic facilities are grossly inadequate. There is a need to educate primary care physicians for early case detection and provide minimum diagnostic and therapeutic facilities in regional centres.

Adrenal Hyperplasia, Congenital↗
PubMedDate not supplied

ACTH 1-17 effects in chronic obstructive bronchitis.

The authors have tested the effectiveness of ACTH 1-17, administered in a single dose of 100 micrograms intramuscularly at 07(00) for 10 consecutive days, in 10 male patients suffering from chronic obstructive bronchitis with different ventilatory impairment. The subjects performed every 4 h (02(00), 06(00), 10(00), 14(00), 18(00), 22(00] for 14 consecutive days (3 days of wash-out and 10 days of treatment) spirometric tests with recording of 8 ventilatory parameters and of oral temperature. The day before and after treatment plasma cortisol levels were also determined 6 times a day. After the period of treatment 4 subjects improved, 4 remained stationary and 2 worsened as concerns the ventilatory functions, while 7 perceived a better general condition. The normal circadian synchronization of the respiratory function of the group, with acrophase in the afternoon, was maintained and the temperature rhythm was not influenced. The circadian rhythm of plasma cortisol showed a significant mesor reduction after treatment with a slight advance in acrophase indicating a partial inhibition of the hypothalamo-hypophyseal axis. The success of treatment was independent of the severity of the ventilatory impairment and the subjects showed different patterns of response both quantitatively (probably related to the dosage) and temporally (immediate or delayed). Hyperpigmentation of the skin owing to the intrinsic melanotropic activity of the peptide occurred in 2 subjects. As a side effect, weight increase was apparent in 3 subjects.

Adrenocorticotropic Hormone↗
PubMed1998

[POEMS syndrome. A rare variant of osteosclerotic multiple myeloma with polyneuropathy, organomegaly, endocrinopathy, M-gradient and skin lesions].

BACKGROUND: A rare multi-organ involvement in plasma-cell dyscrasias has been named POEMS-syndrome: it is a synopsis of monoclonal gammopathy (M-gradient), osteosclerotic bone lesions, peripheral polyneuropathy, organomegaly, endocrinopathy and skin lesions. CASE REPORT: A patient is presented who had a classical manifestation of this disease known mainly in Japan. A monoclonal IgA-lambda-gammopathy was determined as cause of a gradually progressive polyneuropathy. The patient had a hypergonadotropic hypogonadism, hyperprolactinaemia, and sclerotic bone lesions. In addition, he showed a changing organomegaly, and hyperpigmentation of the skin. CONCLUSION: As yet, aetiology and pathophysiology are not fully understood. Irradiation or surgical resection of one or several osteosclerotic bone lesions may improve the polyneuropathy or may even lead to a complete remission of all symptoms. Thus, monoclonal immunoglobulins should be searched for in any unclear polyneuropathy, as should be for other symptoms of the POEMS-syndrome.

Diagnosis, Differential↗
PubMed1999

Complete remission of Nelson's syndrome after 1-year treatment with cabergoline.

In this case report we demonstrated that treatment with the long-acting D2 receptor agonist cabergoline for 1 year induced normalization of plasma ACTH levels and disappearance of the pituitary tumor in a patient with Nelson's syndrome. A young man underwent bilateral adrenalectomy and subsequent pituitary irradiation for Cushing's disease after unsuccessful neurosurgical treatment. Thereafter, he was given cortisone acetate replacement at the dose of 62.5 mg a day. Fifteen months after pituitary irradiation, he developed Nelson's syndrome, having skin hyperpigmentation, high plasma ACTH levels (376 ng/l) and a pituitary microadenoma (5 mm) documented at magnetic resonance imaging (MRI) of the pituitary region. After 6 months of cabergoline treatment, given at the dose of 1 mg a week, plasma ACTH levels were significantly decreased (from 376 to 113 ng/l) but they were not normalized. Cabergoline dose was then increased up to 2 mg a week. Six months later plasma ACTH levels were normalized (22 ng/l) and MRI demonstrated the disappearance of the pituitary adenoma. In order to investigate on the direct effect played by cabergoline treatment on the remission of Nelson's syndrome, the treatment was withdrawn. Plasma ACTH levels significantly increased (119 ng/l) after 3 months of treatment withdrawal. At the last follow-up, during cabergoline treatment at the dose of 2 mg/week plasma ACTH levels were normalized (40.4 ng/l). This case demonstrated that cabergoline treatment is able to induce the remission of Nelson's syndrome and may be a valid therapeutic alternative in this syndrome.

Adenoma↗
PubMed2002

Abnormalities of GH secretion in a young girl with Floating-Harbor syndrome.

We present a 9.1-year-old girl of Calabrian (Italy) ancestry, with clinical features (cranio-facial dysmorphism, short stature with delayed bone age and speech delay) suggesting the diagnosis of Floating-Harbor syndrome (FHS). Physical examination showed: height 113.9 cm (-2.9 SD), with a parent's target of 156.2 cm (+1.0 SD), weight 20.7 kg, BMI 16.0 (-0.04 SD), and many phenotypic abnormalities: long eyelashes, large bulbous nose with broad nasal bridge, short philtrum, moderately broad mouth, tooth folding and malocclusion, posteriorly rotated ears, low posterior hair line, short neck, clinodactyly of the 5th finger and hyperextensible finger joints. Diffused hyperpigmentation and hypertrichosis with sporadic pubic terminal hairs, but neither clitoromegaly nor other signs of hyperandrogenism and/or precocious puberty, were observed (T1, P1). Carpal bone evaluation showed a delayed bone age (TW2: 5-5/10, - 3.6 yr) and the statural age/bone age ratio was 1.1. Other dysmorphic syndromes were excluded on the basis of clinical evidence, also evaluated by a computer-assisted search (P.O.S.S.U.M. version 3.5, 1992). Analysis of chromosome 22 by the FISH method, using specific probes Cos29 and Tuple1, excluded microdeletions in the region 22q11.2, typical of Velo-cardio-facial syndrome. In this case, we report the impairment of serum GH responsiveness (GH baseline values: 0.2-1.9 ng/ml) to the administration of oral 150 microg clonidine [peak 4.7 ng/ml, normal values (nv)>10 ng/ml] and oral 4 mg dexamethasone (8.1 ng/ml, nv>10 ng/ml). Moreover, the evaluation of spontaneous 24-h GH secretion (Carmeda AB, Stockholm, Sweden) showed low mean GH levels (1.75 ng/ml, nv>3.0 ng/ml), with a maximum sleep-related peak of 2.8 ng/ml. Serum IGF-1 values were in the low-normal range (80-176 ng/ml, nv 133-626 ng/ml). While in FHS the cranio-facial features minimize with advancement of age, the impairment of growth velocity is permanent and results in severe dwarfism. In our case, treatment with recombinant GH (0.10 U/kg/day), administered by a needle-free device, induced a dramatic increase of growth velocity, increasing the height from -2.8 to -1.9 SD after 18 months, thus indirectly confirming a role of GH deficiency in the pathogenesis of FHS dwarfism.

Abnormalities, Multiple↗
PubMed2006

Six cases of ectopic ACTH syndrome caused by thymic carcinoid.

The ectopic ACTH syndrome (EAS) caused by thymic carcinoid is rare and its diagnosis remains a challenge to the endocrinologist. Here are six cases of EAS with a typical Cushing habitus accompanied by hyperpigmentation and hypokalemia. For all six patients, the high dose (8 mg) dexamethasone suppression test (HDDST) showed lack of suppression, computed tomography (CT) scanning documented anterior mediastinal masses, and the mediastenal tumors removed were confirmed as ACTH secreting thymic carcinoids by positive ACTH and NSE staining. Our data indicate that HDDST chest radiologic imaging and other laboratory examinations will greatly assist in diagnosing the thymic carcinoid-induced EAS at an earlier stage, which will significantly improve the long-term survival of the patient.

ACTH Syndrome, Ectopic↗
PubMed1987

Isolated glucocorticoid deficiency.

A three-month-old boy with hypoglycemic episodes, feeding problems and hyperpigmentation is described. Hormone assays revealed elevated serum ACTH values and low levels of plasma cortisol that did not rise after exogenous ACTH administration; blood pressure, serum electrolytes and earlier plasma aldosterone and renin activity were in the normal range. These data suggest a diagnosis of an isolated glucocorticoid deficiency, secondary to unresponsiveness to ACTH. Replacement therapy with glucocorticoids was highly effective. Despite replacement therapy, during follow-up he had an increase in basal plasma renin activity with aldosterone concentration normal. In our patient, a mineralcorticoid deficiency might eventually develop; therefore, the selective glucocorticoid deficiency might also be part of a progressive defect involving the glomerulosa too.

Aldosterone↗
PubMed2004

Clinical manifestations and hormonal profile of two women with Cushing's disease and mild deficiency of 21-hydroxylase.

The development of Cushing's disease among patients with deficiency of 21-hydroxylase has not been observed to date. The clinical manifestations and the hormonal profile of this exceptional association are herein described through the study of two cases. The first one was a 39-yr-old woman who had undergone non-curative transsphenoidal surgery for a pituitary-dependent Cushing's syndrome 12 yr before. She showed hypertension, central obesity, severe hirsutism, alopecia and hyperpigmentation. Urinary excretion of cortisol was normal, but ACTH levels were very high and hormonal dynamic studies (cortisol circadian rhythm, insulin-induced hypoglycemia and dexamethasone suppression tests) revealed the qualitative disturbances that characterize Cushing's disease. Serum concentrations of androstenedione, free testosterone and 17-hydroxyprogesterone were clearly increased. Reexamination of the tissue samples from previous surgery confirmed the presence of an ACTH-producing pituitary adenoma. CYP21 gene analysis found the splicing 655G mutation at intron 2 and the V281L mutation at exon 7. The second case was a 21-yr-old woman who was diagnosed with pituitary ACTH-dependent Cushing's syndrome according to unequivocal clinical and laboratory findings. However, hirsutism was particularly severe and both serum androgens and 17-hydroxyprogesterone were elevated. The patient was heterozygote for a large conversion of CYP21 gene. In these cases, the clinical and biochemical expression of Cushing's syndrome was determined by the different severity of 21-hydroxylase deficiency and the subsequent residual ability of adrenal cortex to synthesize cortisol.

Adenoma↗
PubMed1994

Long-term treatment of Nelson's syndrome by octreotide: a case report.

Medical management of Nelson's syndrome by drugs such as bromocriptine, sodium and magnesium valproate has provided disappointing or, at least, controversial results. We report here on the results of long-term (2 yr) treatment with the somatostatin analogue octreotide (300 micrograms daily sc) in one patient affected by Nelson's syndrome occurring after bilateral adrenalectomy for Cushing's syndrome. During treatment, skin hyperpigmentation and serum ACTH levels decreased dramatically and a slight (about 10%) reduction in tumor size, as assessed by computerized tomography, was also observed. These results suggest that octreotide may be useful for the medical management of Nelson's syndrome.

Adrenocorticotropic Hormone↗
PubMed1985

Evidence for ectopic ACTH production years after bilateral adrenalectomy for Cushing's syndrome: in vivo and in vitro studies.

Three patients presented with hyperpigmentation and high plasma levels of ACTH 4-5 years after bilateral adrenalectomy for Cushing's "disease". X-rays and ct-scan of the lungs showed a small pulmonary mass in all. ACTH levels (1100-2000 pg/ml) were not suppressible by high doses hydrocortisone. A carcinoid tumor was removed in two cases and a chemodectoma in the third. Evidence for ACTH secretion by these tumors was provided by both decrease of ACTH levels after surgery and/or in vitro studies. Both carcinoid cultures showed a basal ACTH production, which was clearly increased by LVP (10(-7) M) and CRF (10(-7) M). Our findings in vitro studies suggest the presence of specific receptor sites for physiological stimuli (LVP and CRF) in tumor cell membranes.

Adrenalectomy↗
PubMed2002

[Congenital bullous poikiloderma (Kindler syndrome)].

We report on an 18 years old female patient who presented with synechia of the labia. History reveals congenital blistering with acral localization and photosensitivity in childhood. At present, the patient showed wide-spread poikoloderma with reticulate hyperpigmentation, xerosis, proximal synechia between fingers and toes and absence of dermatoglyphics, suggestive for congenital bullous poikiloderma (Kindler syndrome). The diagnosis was confirmed by histology and electron microscopy. Kindler syndrome is a rare, autosomal recessive disorder with synechia of mucosal areas being the presenting symptom.

Adolescent↗
PubMed2001

[Successful symptomatic tazarotene treatment of juvenile acanthosis nigricans of the familial obesity-associated type in insulin resistance].

An 11-year-old boy suffering from morbid obesity since infancy developed at age 9 a progressive brown-black hyperpigmentated and hyperkeratotic eruption in the neck and axillary region with minor involvement of both groins. Based on this clinical picture, and confirmed by histopathology, we diagnosed acanthosis nigricans. Following a thorough endocrinological examination and because the patient's obese mother showed similar skin lesions, the disease was subclassified as a familial obesity-associated type of acanthosis nigricans associated with insulin resistance. In a right-left-comparison the affected skin of one body side was treated with tazarotene 0.05% versus urea 10%, once daily each. A great benefit for the tazarotene-treated over the opposite side could already be seen after three weeks which was also verified by dermatohistopathology. Three months after topical tazarotene treatment had been extended to both sides, the residual lesions were significantly improved. The highly satisfying, good result has been maintained up to now by a continuous topical retinoid treatment over 18 months, usually with an interval application regimen, i.e., 3 x per week.

Acanthosis Nigricans↗
PubMed1994

[Atrophodermia linearis Moulin. A new disease picture, following the Blaschko lines].

Many different congenital and acquired skin diseases are seen along Blaschko's lines. In 1992, Moulin et al. [40] described five patients affected with a new clinical entity characterized by acquired atrophic band-like skin lesions showing hyperpigmentation. We report on a further patient with this skin disease. Characteristic features are hyperpigmented atrophoderma arranged in a pattern following the lines of Blaschko, with no preceding inflammation and no subsequent induration or scleroderma. The lesions usually appear during childhood or adolescence, but sometimes in young adults. For this skin disease we propose the term 'linear atrophoderma of Moulin'.

Adult↗
PubMed1995

[Nevi flammei in childhood and adolescence. Treatment with the flashlamp-pumped pulsed dye laser].

In 77 patients with port-wine stain under 18 years of age we performed 100 test treatments with the flashlamp-pumped pulsed dye laser (585 nm, 450 microseconds, 5 mm beam diameter). In 54% of cases we could obtain excellent or good lightening with one test treatment. In only 9% of cases did the patients show bad or no response to laser therapy. In the following treatment of larger areas (n = 125), the rate of good or excellent lightening was even higher (60%). Side effects were infrequent and consisted mostly of transient hyperpigmentation. In contrast to argon laser therapy, the flashlamp-pumped pulsed dye laser is safe and effective for treatment of port-wine stains in childhood and adolescence. Our results and the good results reported in the literature confirm the flashlamp-pumped pulsed dye laser as the laser of choice for port-wine stain treatment in young patients.

Adolescent↗
PubMed1995

[Dermatomyositis-like skin changes with long-term hydroxyurea (Litalir) therapy].

We report on dermatomyositis-like adverse cutaneous reactions following long-term maintenance therapy with hydroxyurea in two patients suffering from chronic myelogenous leukaemia (CML). In addition to non-specific side effects, such as xerosis, pruritus and hyperpigmentation, both patients presented with more specific skin changes, i.e. erythematous lesions, scaling, and partially atrophic areas distributed in a linear fashion on the dorsal aspects of the hands and fingers. In addition, teleangiectatic erythema of the face was present in both patients, and this was associated with oedema of the eyelids in one patient. Despite these dermatomyositis-like features there were no clinical signs of muscular involvement, and muscle-specific enzymes were within normal ranges. Skin biopsy specimens revealed an interface dermatitis characterized by a lichenoid cell infiltrate, vacuolar alteration of basal cells, necrotic keratinocytes within the spinous zone, focal hypergranulosis, ortho-hyperkeratosis and telangiectases in the upper part of the dermis. Analogous histopathological findings have been documented in lichen planus-like skin changes on the hands following hydroxyurea therapy. It seems doubtful whether there are actually any major differences between those skin changes described as dermatomyositis-like and those interpreted as lichen planus-like in patients receiving long-term hydroxyurea therapy.

Aged↗
PubMed1996

[2 unusual cutaneous T-cell lymphomas with extracutaneous involvement].

In this paper we present two patients with T-cell lymphomas, who presented with unique skin lesions, but later developed extracutaneous involvement. The first patient showed marked edematous infiltration of the trunk and legs as well as grouped, umbilicated red-to-blue papules limited to the body folds. The second patient had lesions reminiscent of poikiloderma vascular atrophicans, with erythema, teleangiectasia, hypo- and hyperpigmentation, and bizarre scarring, followed by development of papules and nodules. The first patient also had marked leukocytosis and lymph node enlargement. Histology, immunohistology, and immunophenotyping revealed an unclassifiable CD8+, alpha, beta +, CD10+ cutaneous T cell lymphoma with leukemic involvement. Unclassifiable cutaneous T-cell lymphoma was also diagnosed in the second patient: it was associated with a monoclonal proliferation of T-cell receptor V gamma 9, delta + large granular T lymphocytes and finally developed into frank, acute pre-T lymphatic leukemia.

Adult↗
PubMed1996

[Selective photothermolysis of superficial varicose veins telangiectasias of the lower extremity].

Leg venectasia is a common problem. Although there are a variety of therapeutic modalities, none of them gives satisfactory results. The effects of sclerotherapy in the treatment of telangiectasia with a small diameter are poor. Electrocoagulation and argon laser treatment often result in scarring. We studied the effectiveness of the flashlamp pumped pulsed dye laser at a wavelength of 585 nm in the treatment of leg venectasia. After repeated treatments using this laser system, only 30% of the vessels that looked blue on clinical examination could be removed; we obtained better lightening of smaller red, telangiectatic vessels with a diameter up to 0.4 mm. However, hyperpigmentation frequently developed after treatment. In post-treatment biopsies, occlusion of the vessels was found. Based on these results, we recommend a combined therapy for leg venectasia. Larger diameter vessels should be treated with sclerotherapy, while finer telangiectasia can be treated with the flashlamp pumped pulsed dye laser.

Adult↗