Adrenal Cortical Insufficiency Research Articles

Long-diagnosed primary adrenal insufficiency in the setting of Werlhof’s disease. A clinical case report

Chronic adrenal insufficiency is an endocrine disease caused by insufficient secretion of adrenal hormones due to dysfunction of one or more links of the hypothalamic-pituitary-adrenal axis. The highest prevalence has been documented in Scandinavian countries: 15–22 people per 100,000 population, while other European countries report 10 cases per 100,000 population. Autoimmune adrenalitis is the most common cause of primary adrenal insufficiency in adults, and it can be either alone (40 %) or a component of autoimmune polyglandular syndromes (60 %). The etiologic factors of primary adrenal insufficiency include tuberculosis or AIDS. These causes of adrenal cortical insufficiency are particularly relevant in Ukraine. The article describes a clinical case of a patient with both primary adrenal insufficiency and Werlhof’s disease. The prevalence of adrenal insufficiency and etiologic factors of primary adrenal insufficiency are highlighted. A detailed differential diagnosis of primary adrenal insufficiency with other diseases that could cause clinical symptoms such as hypoglycemic states, severe weight loss, hypotension, severe muscle weakness, hyponatremia, hyperkalemia is performed. The basic principles of diagnosis, treatment, and prognosis in primary adrenal insufficiency are discussed. Despite typical, specific manifestations of adrenal insufficiency, diagnosis in real clinical practice is difficult. Awareness and vigilance of doctors regarding the symptoms of adrenal insufficiency is necessary. Late detection of chronic adrenal insufficiency can lead to complications. Timely diagnosis and treatment of chronic adrenal insufficiency improves disease prognosis and quality of life.

ODP019 A Case Study of the Association of Autoimmune Polyglandular Syndrome and Pulmonary Artery Hypertension

Abstract We report a patient with combined pulmonary arterial hypertension (PAH) and autoimmune polyglandular syndrome (APS) type 2. A 41 y/o woman with hypothyroidism presented for right heart (RHC) in the work up of PAH. Pt had a medical history of PAH diagnosed by transthoracic echocardiogram (TTE) and hypothyroidism. She reported symptoms of fatigue, shortness of breath with increased pigmentation of her skin progressive for several years. Preadmission lab found hyponatremia of 126 and potassium of 6.6. She was admitted and treated with IV insulin with D50, kayexalate, IV fluids. A random 11 AM cortisol was 0.57 mcg/dl N: AM: 6. 0–30. 0 mcg/dl; PM: 3. 0–16. 0 mcg/dl. Acosyntropin test was ordered. Pt failed the stimulation test (baseline 0.39, 30 min 0.49, 60 min 0.50, with an ACTH at baseline of 1423 pg/ml N: 7.2-63.3). Pt was started on hydrocortisone 20 mg AM/10 mg PM with fludrocortisone 0.1mg. On follow up at 2 weeks, her energy, mood, appetite and breathing tolerance had improved. Follow up RHC in 4 weeks found normalization of right heart pressures. APS is a collection of autoimmune diseases. Type I APS, caused by a defect in the autoimmune regulator (AIRE) gene, is diagnosed with two of three conditions, chronic mucocutaneous candidiasis, hypoparathyroidism, and adrenal cortical insufficiency. Type II APS (formally "Schmidt's Syndrome"), associated with HLA haplotypes DR3 (DQB*0201) and DR4 (DQB1*0302), is diagnosed with chronic autoimmune adrenal insufficiency with either Type 1 DM or chronic autoimmune thyroid disease (commonly Hashimotos but can be Graves). It typically presents later in life compared to APS I, age 30-40s. The association between PAH is rare but has been noted in several case studies. It has been associated with APS I in Korniszewski et al. and Bhansali et al. For APS II, the first association was reported by Barrou et al. of a patient with hypothyroidism, hypogonadism, and adrenal insufficiency who later developed PAH. Two cases reported by García-Hernández et al found PAH with positive adrenal antibodies without adrenal insufficiency. In 2009 Walid R. et al. reported a case of severe adrenal insufficiency (hypotension, hypokalemia) with PAH. She improved with steroids, but her PAH was present on TTE four years after treatment started. Pt in that case declined RHC. The patient in this case study proceeded with a RHC which did show normalization of right heart pressures, the first reported case to document normalization of PAH with replacement steroid treatment. Presentation: No date and time listed

345. CAMRELIZUMAB AS ADJUVANT THERAPY IN RESECTED ESOPHAGEAL SQUAMOUS CELL CARCINOMA: A PHASE 2 TRIAL

Abstract CheckMate 577 has confirmed that adjuvant immunotherapy significantly prolongs disease-free survival (DFS) in patients with resected (R0) stage II or III esophageal or gastroesophageal junction cancer who had received neoadjuvant chemoradiotherapy. However, adjuvant therapy for patients with different neoadjuvant therapies needs to be explored. Therefore, this study was designed to investigate the efficacy and safety of camrelizumab as adjuvant therapy in patients with resected (R0) esophageal squamous cell carcinoma (ESCC). This is an ongoing, non-randomized, open-label, phase 2 trial (NCT04533490) in China. ESCC patients (stage cT1b-T4aN0M0/cT1-T4aN + M0) aged 18–75 years with ECOG performance status of 0–1 was planned to be enrolled in cohort A (n = 42, with 6–12 weeks of neoadjuvant therapy) and cohort B (n = 43, without neoadjuvant therapy). Following R0 resection, adjuvant camrelizumab (200 mg Q2W) was given until tumor recurrence, metastasis, intolerable toxicity, or the completion of 12-month treatment. The primary endpoint was DFS. Secondary endpoints included overall survival, recurrence rate, 1/3/5-year DFS rates, and safety. At data cutoff (Apr 21, 2022), 17 patients were enrolled, with a median age of 65 years; 9 (53%) patients were males, and 12 (71%) had ECOG performance status of 0. There were 15 patients in cohort A (14 with neoadjuvant immunochemotherapy, and 1 with neoadjuvant chemotherapy) and 2 in cohort B. With a median follow-up of 5.5 months, DFS was not reached. A total of 14 (82.3%) patients had treatment-related adverse events, which were all grade 1–2. The most common events were reactive cutaneous capillary endothelial proliferation (11 [65%]), adrenal cortical insufficiency (3 [18%]), and interstitial pneumonia (2 [12%]). Adjuvant camrelizumab showed acceptable safety profile in patients with resected (R0) ESCC who mainly received neoadjuvant immunochemotherapy, and the efficacy was worth expecting. The study enrollment is ongoing, and further survival and safety data will be reported in the future.

Adrenal insufficiency of patients with graft versus host disease treated with extracorporeal photopheresis.

Graft versus host disease (GvHD) is a serious and common complication of allogenic haematopoietic stem cell transplant. Corticosteroids are considered the standard care for initial treatment of GvHD but a significant proportion of patients will need long-term steroid treatment for control of GvHD. Extracorporeal photopheresis (ECP) is a cell-based immunomodulatory therapy that is an accepted second line treatment in patients with steroid refractory, dependent or intolerant GvHD and has shown efficacy in allowing steroid dose reduction and discontinuation in this cohort of patients. Adrenal cortical insufficiency is defined by the inability of the adrenal cortex to produce sufficient amounts of glucocorticoids and/or mineralocorticoids leading to a severe and potentially life-threatening condition. The most common cause of drug-induced adrenal insufficiency is the suppression of the hypothalamic-pituitary-adrenal axis by exogenous glucocorticoid doses ≥5 mg prednisolone equivalent for more than 4 weeks. The aim of the study was to ascertain the number of patients with GvHD receiving ECP that are affected by adrenocortical insufficiency.

Adrenal Cortical Insufficiency

The clinical signs of adrenal cortical insufficiency (incidence, ca. 25 per million per year; prevalence, ca. 400 per million) are nonspecific, and misdiagnoses are therefore common. Glucocorticoid substitution therapy has been in use for 50 years but is not a wholly adequate treatment. Our understanding of this disease remains incomplete in many ways. We selectively searched the Medline database for publications on adrenal cortical insufficiency, with particular attention to studies from the year 2000 onward (search terms: "adrenal insufficiency" or "Addison's disease" or "hypopituitarism"). Hydrocortisone substitution therapy is often given in doses of 10-25 mg/day, timed according to the circadian rhythm. Gastrointestinal and other, febrile infections account for 30-50% of life-threatening adrenocortical crises. Such crises affect 8 of 100 persons with adrenal cortical insufficiency per year and must be treated by the immediate administration of glucocorticoids and fluids. When persons with adrenal cortical insufficiency are acutely ill or are otherwise under unusual stress, they may need additional amounts of hydrocortisone, often in the range of 5-10 mg but occasionally as high as 200 mg. The sustained administration of excessive amounts of steroid can shorten patients lives by several years. Inappropriate substitution therapy can cause other major medical conditions, such as metabolic syndrome and osteoporosis. Important measures for the prevention of adrenocortical crises include improved care by treating physicians, education of patients and their families, the provision of emergency identifying documents, and the prescription of glucocorticoid emergency kits.

Mutations in the genes encoding for leptin and its mediators: induction of obesity with various concomitant pathological conditions

g15384delG(G133fsX15), c.215T⇒C(p.L72S), c.309C⇒A(p.N103K), c.313C⇒T(p.R105W), c.422C⇒G(p.S141C), c.104_106delTCA, c.481_482delC⇒T, and c.135del13bp mutations in LEP in the homozygous state are known to be associated with the early onset of severe obesity and disturbances of many physiological functions. Similar pathological changes are induced by mutations in LEPR, such as G⇒A substitution in the splicing donor site, deletions of 4 and 11 base pairs in the 5'-terminus of cDNA, deletion of 66 bp in the CRH domain of LEPR, compound deletion of 1 base pair in the 5'-terminus/p.R612H, and missense mutations P316T, A409E, W664R, and H684P. Disturbances in LEP and LEPR expression in homozygous subjects are associated with severe obesity, retarded sexual development, insulin resistance, disordered secretion of adenohypophyseal hormones, and immunodeficiency. Mutations in LEP cause more serious pathological changes than LEPR mutations. In the homozygous state, mutations in POMC, such as 3804C⇒A, 6906delC, 6922insC, and compound heterozygous mutations 7134delG/7013G⇒T, 6996del/6851A⇒T, 3804CA/7100insGG are associated in man with morbid obesity and acute adrenal cortical insufficiency. In the heterozygous state, these mutations predispose to obesity in the absence of concomitant disorders. Mutations in MC4R are the most common cause of obesity. They are known to induce severe pathology in homozygous subjects but significantly milder disturbances of adipose tissue metabolism in heterozygotes. In both cases, obesity is not accompanied by serious disturbances of other functions.

Lower Serum Androstenedione Levels in Pre-Rheumatoid Arthritis versus Normal Control Women: Correlations with Lower Serum Cortisol Levels

Serum adrenal androgens (AAs), including androstenedione (Δ4A) and dehydroepiandrosterone sulfate (DHEAS), have been reported to be lower in female rheumatoid arthritis (RA) patients with early disease. Few data are available on hormonal status of women before the onset of clinical rheumatoid arthritis (pre-RA). A broad baseline panel of serum adrenal and sex steroids was compared in 36 female pre-RA to 144 matched cohort control (CN) subjects to determine differences in their mean values and in patterns of hormonal correlations. Study subjects having lower versus higher baseline serum cortisol levels than the total group's mean value were also analyzed separately to investigate differences in their hormonal levels and correlational patterns. In total subjects, mean (±SE) Δ4A level (nmol/L) was lower (P = 0.018) in 28 pre-RA cases (6.4 ± 0.40) versus 108 CN (7.8 ± 0.28). The significant (P = 0.013) difference was restricted to 9 pre-RA versus 53 CN subjects having lower cortisol levels (5.6 ± 0.73 versus 8.0 ± 0.42 nmol/L, resp.). In total subjects, no significant difference was found between study subjects in their bivariate correlations of the hormonal panel variables, unlike results found in the subgroups stratified by lower versus higher cortisol levels. A subgroup of pre-RA females may have relative adrenal cortical insufficiency, as reflected by lower Δ4A, especially observed among those subjects with lower cortisol levels.

Adrenal cortex disorders

In Brief Adrenal cortical hormones affect virtually every body system. By reviewing two complex disorders—adrenal cortical insufficiency (Addison disease) and adrenal cortical hypersecretion (Cushing syndrome)—nurses will be better prepared to recognize the wide-ranging effects on their patients' health and intervene appropriately.

Caring for patients with adrenal cortex disorders

In Brief Does your patient have adrenal insufficiency or oversecretion? Here's how to identify and help manage the problem, whether it's adrenal cortical insufficiency (also called Addison disease) or adrenal cortical hypersecretion (also called hypercortisolism and Cushing disease).

Pituitary FDG Uptake in a Patient of Lung Cancer With Bilateral Adrenal Metastases Causing Adrenal Cortical Insufficiency

A 64-year-old woman with history of lung cancer and left adrenal gland metastasis was referred for FDG PET/CT to assess the response to target therapy and local radiotherapy treatment. In addition to bilateral adrenal gland FDG uptake lesions, the PET/CT also showed focal FDG uptake in pituitary gland with standardized uptake value of 3.9. Adrenocorticotropic hormone serum level was 439 pg/mL (normal <46 pg/mL), and serum cortisol level was 6 μg/dL (normal range, 5-25 μg/dL). The image and serum test results suggested the diagnosis of lung cancer, with bilateral adrenal metastases causing adrenal cortical insufficiency with secondary pituitary gland hyperplasia.

Transsphenoidal surgery for Cushing disease: experience with 136 patients.

This is a retrospective study of 136 patients with Cushing disease treated with transsphenoidal microsurgery. To evaluate factors influencing immediate postoperative results and long-term outcomes. Data regarding clinical presentation, endocrine evaluation, imaging studies, surgical technique, immediate postoperative biochemical remission (IPBR), and long-term results were entered into a database and analyzed statistically. IPBR was based on biochemical evidence of adrenal cortical insufficiency and clinical evidence of such insufficiency. IPBR for the entire series was 83.4%. In microadenomas, IPBR was 89.8% with a mean immediate postoperative plasma cortisol (IPPC) of 2.1 μg/dL (range, <0.5-5.3). Positive magnetic resonance imaging (MRI) was associated with 18 times greater odds of finding microadenoma at surgery (P < .001) and with 4.1 times greater odds of IPBR (P = .07). In patients with a negative MRI, a positive inferior petrosal sinus sampling (IPSS) test was associated with 93% of IPBR (P = .004). IPBR in macroadenomas was 30.7%. Of patients followed for 12 months or longer, 34.8% required glucocorticoid replacement for the duration of follow-up. The mean follow-up in microadenomas was 68.4 months with a 9.67% incidence of recurrences. The estimated actuarial incidence of recurrences increased with the passage of time and IPPC of greater than 2 μg/dL was associated with higher incidence of recurrences, although without statistical significance (P = .08). In microadenomas, a positive MRI and positive IPSS test were associated with a higher incidence of IPBR. Recurrences increased with the passage of time, and an IPPC of greater than 2 μg/dL may be associated with higher incidence of recurrences.

Evaluation der hypothalamo-hypophysär-adrenalen Hormon-Achse bei Patienten mit chronisch-obstruktiver Atemwegserkrankung: Vergleich einer inhalativen mit einer systemischen Glucocorticoidtherapie

The action of inhalation and systemic treatment of chronic obstructive pulmonary disease by suppressing the hypothalamo-hypophyseal-adrenal axis was compared in patients with chronic obstructive pulmonary disease (COPD). Adrenocorticotropic hormone (ACTH) and cortisol concentrations were evaluated after a corticotropin-releasing-hormone (CRH)-test in 50 patients (aged 43 +/- 14 years) with chronic obstructive pulmonary disease (COPD) receiving inhalant glucocorticoid treatment (IGC), 61 patients (aged 54 +/- 11 years) with COPD on systemic glucocorticoid treatment (SGC) and 50 healthy volunteers (32 +/- 4 years). All 50 patients on IGC had normal CRH test results. 30 of 61 patients with SGC had decreased cortisol response (12 patients had no and 18 a reduced rise in cortisol). ACTH concentration was lower in patients on IGC than in the control group (basal ACTH 15.6 pg/ml and 24.5 pg/ml, respectively; after stimulation 40.3 vs 54.4 pg/ml, respectively). But systemic glucocorticoid treatment clearly caused suppression of basal (12.1 pg/ml) and stimulated (27.4 pg/ml) ACTH levels with correspondingly decreased cortisol levels (basal: 75.1 and 118.7 ng/ml [IGC], respectively, and after stimulation 128.5 and 225.9 ng/ml). Patients with COPD on inhalant glucocorticoid treatment have a clearly lower risk of adrenal cortical insufficiency than those on oral glucocorticoid treatment. But some suppression of ACTH secretion is demonstrable even in the former. Clinical significance of these findings seems unlikely. Development of adrenal cortical insufficiency need not be feared in patients treated with inhalant glucocorticoids.

마황약침이 부신피질기능부전증에 미치는 영향

Effects of mahwang(Ephedrae herba) aqua-acupuncture at sinsoo (B-23)and Jisil(B-52)on adrenal cortical insufficiency were investigated in dexamethasone treated rats. Concentration of serum cortisol was decreased in dexamethasone treated rats. However, these values showed a tendency to increase in mahwang(Ephedrae herba) aqua-acupuncture groups. Concentration of serum total protein was increased in dexamethasone treated rats. However, these values were decreased by the mahwang(Ephedrae herba) aqua-acupuncture. The portion of neutrophils was decreased and the portion of lymphocytes and eosinophils were increased in dexamethasone treated rats. However, in mahwang(Ephedrae herba) aqua-acupuncture groups, the portion of neutrophils showed a tendency to increase and the portion of lymphocytes and eosinophils showed a tendency to decrease. In dexamethasone treated rats, the weight of adrenal glands were decreased, however these values were increased in mahwang(Ephedrae herba) aqua-acupuncture groups.

The new biology enters the generalist pediatrician's office: lessons from the Human Genome Project.

1. Edward R.B. McCabe, MD, PhD* 1. 2. *Physician-in-Chief, Mattel Children’s Hospital at UCLA; Professor and Executive Chair, Department of Pediatrics, UCLA School of Medicine, Los Angeles, CA. Birth defects are the leading cause of infant mortality in the United States, representing more than 20% of all infant deaths. This infant mortality rate from birth defects exceeds that from sudden infant death syndrome, low-birthweight/short gestation, respiratory distress syndrome, and maternal complications. In addition, birth defects and genetic diseases represent major sources of morbidity for those who survive. As our ability increases to care effectively for those who have infectious diseases and other acute illnesses, individuals who have chronic illnesses due to genetic etiologies represent an increasing proportion of patients seen in the general pediatrician”s office. The Human Genome Project was initiated on October 1, 1990, and has a projected funding period of 15 years. The goal is to sequence the entire human genome, representing three billion base pairs that contain the coding sequences for approximately 75,000 genes. During the latter half of this century, investigations into the genetics of disease gathered increasing momentum. In addition to fundamental investigations into human genetics, technologic tools were developed that permitted large-scale genomic sequencing. These tools included the polymerase chain reaction (PCR), which permits amplification of hundreds of thousands or even millions of copies of DNA and requires only limited sequence data for its success; automated DNA sequencing, which allows increased sequence processing and decreased cost compared with manual methods; and improved information systems, which permit sophisticated analysis and assembly of the three billion base pairs of DNA in the human genome. Thus, the Human Genome Project represents the current chapter in our understanding, but it is neither the first nor the final chapter in this story. Once we know the sequences of all of the human genes, we must learn their functional roles in human development and disease pathogenesis. The Human Genome Project has been referred to as the “moon shot …

Polyglandular autoimmune syndrome type II: epidemiology and forms of manifestation

Polyglandular autoimmune syndrome (PAS), is characterized by the coexistence of several autoimmune diseases, affecting predominantly the endocrine glands. The juvenile form (PAS type I) is distinguished from the adult type II in which autoimmune thyroiditis, adrenal cortical insufficiency and diabetes mellitus type I predominate. The connections between epidemiological, clinical and immunological aspects were analysed and described in this study of patients with PAS type II. Among a total patient population of over 15,000 seen in our endocrinological university policlinic from 1992 through 1996, the clinical data of all 151 patients with PAS type II were analysed retrospectively. Of the 151 patients with PAS 37 were male (aged 16-74 years, median 42 years) and 114 female (aged 16-84 years; median 46 years), a prevalence of 1:100, 92 patients (61%) had diabetes mellitus type I (78% males, 55% females; p = 0.013), 50 (33%) had hyperthyroidism (32% and 33%; difference not significant), 49 had Hashimoto's thyroiditis (14% and 39%; p = 0.005) and 28 (18.5% had Addison's disease (14% males, 20% females; n.s.). Vitiligo was less common, occurring in 30 (20%), alopecia in 9 (6%), pernicious anaemia in 5 (5%) and hypogonadism in 8 (5%). The most common combination was diabetes type I with thyroiditis (33%), the time between manifestations of each disease being highly variable, diabetes was the first manifestation in half the cases. Partial cell antibodies were present in 62 patients (54%), and 52% had positive thyroid-associated antibodies without manifest signs of thyroid disease. PAS occurs relatively often among patients of an endocrinological centre, with a 3:1 female to male ratio. Because there is usually a long interval between the onset of the various endocrine diseases, regular monitoring of patients with an endocrine autoimmune disease is indicated, specific tests to be performed if additional immune diseases are suspected.

Assessment of adrenal function in women heterozygous for adrenoleukodystrophy.

Adrenoleukodystrophy (ALD) is an X-linked recessive disorder that destroys the white matter of the brain and is associated with adrenal insufficiency. The prevalence of adrenal dysfunction in 71 women carriers of the X-linked ALD gene was studied. These subjects were identified initially on the basis of being obligate carriers of the X-linked trait by pedigree analysis and were confirmed by plasma very long chain fatty acid levels consistent with a heterozygote status. One subject had well documented overt adrenal insufficiency, diagnosed and treated since age 9 yr. Among the remaining women, the mean serum 0800 h and 1 h post-ACTH cortisol concentrations [16 +/- 7 (+/-SD) and 34 +/- 8 micrograms/dL, respectively] were normal. All subjects had normal ACTH-stimulated serum cortisol levels, i.e. more than 20 micrograms/dL. However, 4 subjects (6%) had subnormal ACTH-stimulated aldosterone concentrations (mean, 9 +/- 6 vs. 42 +/- 16 ng/dL for other subjects; P = 0.001, by Mann Whitney rank sum test). Three of these women (75%) were taking nonsteroidal antiinflammatory agents (NSAIDs), whereas only 4 of 67 (6%) subjects with normal aldosterone responsiveness were NSAIDs users (P < 0.01, by Fisher's exact test). Thus, NSAIDs use was associated with increased risk of hypoaldosteronism (odds ratio, 50.2; 95% confidence interval, 3.3-266; P < 0.002). Three of these four women had symptoms consistent with mineralocorticoid deficiency. Serum sodium and potassium concentrations were normal in all subjects. Basal and metyrapone-stimulated plasma ACTH concentrations were also normal in adequately tested subjects with and without mineralocorticoid insufficiency. Five of eight subjects (63%) who underwent testing with synthetic ovine CRH (oCRH) had abnormalities. Three did not meet the criteria for adequate cortisol stimulation (i.e. > 20 micrograms/dL) and had peak ACTH levels greater than 30 pg/mL. Two other subjects had exaggerated ACTH responses with normal cortisol levels. There were no significant differences in the mean or median levels of very long chain fatty acid, C26:0, C24/22 ratios, or C26/22 ratios among the entire subject group, the subgroup with blunted aldosterone responses to ACTH, and the subgroup with blunted responses to oCRH (P > 0.05, by ANOVA and Kruskall-Wallis test for C26, C24/22 ratio, and C26/22 ratio). We conclude that 1) adrenal cortical insufficiency rarely develops in ALD heterozygotes; 2) isolated mineralocorticoid insufficiency can occur in ALD heterozygotes, as has been previously reported to occur with autoimmune and acquired immunodeficiency syndrome-related adrenal dysfunction; 3) ALD heterozygosity may predispose these individuals to NSAID-related hypoaldosteronism; and 4) a subclinical decrease in glucocorticoid reserve, as measured by oCRH testing, may be present in a majority of these women. Aldosterone levels should be included in the ACTH stimulation testing when seeking evidence of adrenal insufficiency in affected women. NSAIDs should be considered a risk factor for the development of hypoaldosteronism in women heterozygous for ALD.

Early detection of neonatal adrenal hemorrhage by ultrasonography.

To investigate clinical features and course of neonatal adrenal hemorrhage (NAH) from July 1992 to August 1993, fifteen babies suffering from NAH born at Veterans General Hospital-Taipei were included. Portable ultrasound scanner was used for initial screening. The frequency of NAH was approximately 0.35% which is lower than other reports. The distribution of their birth weight was 3 cases lower than 3000 gm, 3 cases over 4000 gm and 9 cases within 3000 to 4000 gm. Only one was premature and another one post-term. Four of them had a history of either prenatal or perinatal asphyxia. Only one patient had a palpable abdominal mass. On the sonographic findings, eleven had bleeding in the right-side, two in the left-side and two in both sides. Only one had adrenal calcification. During their early life, no patient developed adrenal cortical insufficiency or secondary profuse bleeding. Two patients had pathologic jaundice. Two patients had severe anemia. After conservative treatment, none of them developed any severe complication. In conclusion, with the aid of renal sonoscreening in the newborn, we can discover NAH early and give adequate treatment at once. Severe complications also can be prevented.

Acute onset of X-linked adrenoleukodystrophy mimicking encephalitis

We report the case of a 6-year-old boy with X-linked adrenoleukodystrophy (ALD). In view of the acute onset of vomiting, fever, and coma, encephalitis was initially suspected. However, brain magnetic resonance imaging demonstrated a pattern of demyelination that was consistent with ALD; this diagnosis was confirmed by the finding of elevated plasma very long-chain fatty acids levels. At presentation, the patient was hyponatremic. That this metabolic disturbance and the coma resolved within hours of the initiation of corticosteroid therapy suggests that the presenting symptoms were secondary to adrenal cortical insufficiency. Primary adrenal failure was confirmed by endocrinologic evaluation. Thrombocytopenia, hepatic transaminase abnormalities, anemia and leukopenia developed during the subsequent course of therapy with oleic acid and erucic acid.

Addison's Disease Secondary to Prostatic Carcinoma. A Case Report

Adrenal cortical insufficiency secondary to destruction of the cortex by a metastatic tumor is a rare condition. Addison's disease is usually caused by an autoimmune process or by a tuberculous infection. We report a case of adrenal insufficiency as the first clinical manifestation of a metastatic prostate carcinoma that occurred simultaneously with an active pulmonary infection by M. tuberculosis.

Contrasting effects of nitrofurans on plasma corticosterone in chickens following administration as a bolus or diet additive

Administration of furazolidone as a bolus dose (8-500 mg/kg), produced a decrease in plasma corticosterone in chickens. In contrast, addition of furazolidone or furaltadone to the diet (0.04% or above, 10 days), increased plasma corticosterone. Pre-treatment with a 200-mg/kg bolus of furazolidone or furaltadone did not affect pentobarbitone anaesthesia time in the birds. In chickens pre-treated with a nitrofuran in the diet, however, pentobarbitone anaesthesia time was significantly less than that in controls. Furaltadone in the diet, produced significant increases in the amount of cytochrome P-450 and the activity of aniline hydroxylase in the liver microsomes. It is suggested that nitrofurans given in the diet stimulated corticosterone biosynthesis in the adrenal glands and induced mixed-function oxidase activity in the liver. Nitrofurans given as a bolus did not produce these effects. Furazolidone (200 mg/kg) produced severe anorexia, which lasted 2 days in T-line birds. The anorexia seemed to be associated with tissue damage in the birds rather than the ensuing adrenal cortical insufficiency.