Adrenal Ultrasonography Research Articles

Non-classical 11β-hydroxylase deficiency caused by a novel heterozygous mutation: a case report and review of the literature.

11β-hydroxylase deficiency (11β-OHD) constitutes a rare form of congenital adrenal hyperplasia (CAH), typically accounting for ~5-8% of CAH cases. Non-classical 11β-OHD is reported even more rarely and frequently results in misdiagnosis or underdiagnosis due to its mild clinical symptoms. A clinical, biochemical, radiological, and genetic study was conducted on a 9-year-old girl presenting with mild breast development, axillary hair growth, and advanced bone age. Additionally, a comprehensive review and synthesis of the literature concerning 11β-OHD were conducted. The patient presented with breast enlargement, axillary hair development, and accelerated growth over the past year. Laboratory tests revealed levels of cortisol, luteinizing hormone, testosterone, and progesterone that were below normal. A gonadotropin-releasing hormone (GnRH) stimulation test suggested the possibility of central precocious puberty. Radiologic examination revealed a 2-year advance in bone age, while bilateral adrenal ultrasonography showed no abnormalities. Her mother exhibited hirsutism, while her father's physical examination revealed no abnormalities. Whole-exon genetic testing of the child and her parents indicated a heterozygous mutation of c.905_907delinsTT in exon 5 of the 11β-hydroxylase gene (CYP11B1) in the child and her mother. This mutation resulted in a substitution of aspartic acid with valine at amino acid position 302 of the coding protein. This frameshift resulted in a sequence of 23 amino acids, culminating in a premature stop codon (p.Asp302ValfsTer23). A review of the previous literature revealed that the majority of heterozygous mutations in 11β-OHD were missense mutations, occurring primarily in exons 2, 6, 7, and 8. The most common mutation among 11β-OHD patients was the change of Arg-448 to His (R448H) in CYP11B1. Furthermore, bioinformatics analyses revealed that heterozygous mutation of c.905_907delinsTT had deleterious effects on the function of CYP11B1 and affected the stability of the protein, presumably leading to a partial impairment of enzyme activity. The results of the in vitro functional study demonstrated that the missense mutant (p.Asp302ValfsTer23) exhibited partial enzymatic activity. We report a novel heterozygous mutation of CYP11B1 (c.905_907delinsTT), enriching the spectrum of genetic variants of CYP11B1. This finding provides a valuable case reference for early diagnosis of non-classical patients with 11β-OHD.

Neonate With Jaundice

A 3-day-old male infant presented to the emergency department for jaundice. He was born at 39 weeks of gestation via spontaneous vaginal delivery, with a weight of 3,056 g and Apgar scores of 8 and 9. The mother was 35 years old, and this was her second child after an uneventful pregnancy. He was breastfed 10 times per day, with 4 daily diaper changes. On examination, his vitals were normal, and he interacted well. He had icteric skin over the face, trunk, and legs, with subtle hyperpigmentation of the axilla and scrotum (Figure 1). Initial data demonstrated sodium levels of 133 mEq/L and potassium levels of 5.9 mEq/L. The pediatric emergency physician performed abdominal point-of-care ultrasonography (Figure 2).Figure 2Emergency abdominal ultrasonography shows an enlarged left adrenal gland above the left kidney apex (left panel arrowhead), with a highly coiled-cerebriform appearance resembling cerebral gyri in the suprarenal fossa (left panel arrow). The right adrenal gland was wrinkled and wavy, located above the right kidney apex (right panel arrowhead). Normal thin central echogenic stripe was absent, replaced by diffuse stippled echogenicity surrounded by a hypoechoic rim (right panel arrow).1Al-Alwan I. Navarro O. Daneman D. et al.Clinical utility of adrenal ultrasonography in the diagnosis of congenital adrenal hyperplasia.J Pediatr. 1999; 135: 71-75Abstract Full Text Full Text PDF PubMed Scopus (47) Google ScholarView Large Image Figure ViewerDownload Hi-res image Download (PPT) Congenital adrenal hyperplasia. The patient progressed to a sodium level of 131 mEq/L and a potassium level of 6.3 mEq/L the following day. On day 6, screening showed 17-hydroxyprogesterone 65 ng/dL. Additionally, physicians administered fludrocortisone and cortisone acetate with normalization of electrolyte derangement. Final data confirmed 21-hydroxylase deficiency, salt-wasting type congenital adrenal hyperplasia: with 17-OH progesterone levels of 21.9 ng/mL (reference range, 0.49-4.1 ng/mL), and adrenocorticotropic hormone levels of 585.8 pg/mL (reference range, 10-70 pg/mL). Infants with congenital adrenal hyperplasia present perinatally with ambiguous genitalia in females and life-threatening adrenal crisis in males. Although neonatal screening for 21-hydroxylase deficiency minimizes delays in diagnoses, one-third of infants with congenital adrenal hyperplasia were symptomatic when diagnosed,2Pang S.Y. Wallace M.A. Hofman L. et al.Worldwide experience in newborn screening for classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency.Pediatrics. 1988; 81: 866-874PubMed Google Scholar with the most severe manifestation being cardiac arrest secondary to hyperkalemia.3Agarwal S. Deshpande G. Agarwal D. et al.Sudden cardiac arrest in a neonate with congenital adrenal hyperplasia.Pediatr Cardiol. 2005; 26: 686-689Crossref Scopus (3) Google Scholar In experienced hands, adrenal ultrasonography has a sensitivity of 92% and specificity of 100% for diagnosing congenital adrenal hyperplasia1Al-Alwan I. Navarro O. Daneman D. et al.Clinical utility of adrenal ultrasonography in the diagnosis of congenital adrenal hyperplasia.J Pediatr. 1999; 135: 71-75Abstract Full Text Full Text PDF PubMed Scopus (47) Google Scholar and is a useful adjunct for the rapid diagnosis of adrenal crisis in male infants with congenital adrenal hyperplasia.

Utility of a corticotropin-releasing hormone test to differentiate pituitary-dependent hyperadrenocorticism from cortisol-producing adrenal tumors in dogs.

BackgroundHyperadrenocorticism (HAC) is a common endocrine disorder in dogs; however, there are no reports on the use of the corticotropin‐releasing hormone test (CRHT) to differentiate between pituitary‐dependent hyperadrenocorticism (PDH) and cortisol‐producing adrenal tumors (CPATs), both causative of HAC.ObjectivesTo evaluate the usefulness of CRHT as a tool to differentiate between PDH and CPAT in dogs and to determine the reference intervals for CRHT in healthy, PDH, and CPAT dogs.AnimalsDogs diagnosed with PDH (n = 21), CPAT (n = 6), and healthy beagle dogs (n = 33).MethodsThis prospective study included dogs with a definitive diagnosis of PDH and CPAT and healthy beagle dogs, in which CRHT was performed, were prospectively evaluated. We investigated the correlations of CRHT (endogenous adrenocorticotropic hormone [ACTH] concentration, endogenous ACTH concentration [EAC], and poststimulation ACTH concentration [PAC]) with pituitary‐to‐brain ratio (PBR) (in PDH) and with indices of adrenal ultrasonography (smaller and larger adrenal gland dorsoventral thickness in PDH and CPAT).ResultsFor EAC, the area under the curve (AUC) was 0.95, with a cutoff value of 26.3 pg/mL (sensitivity: 90.62%, specificity: 87.50%). The AUC for PAC was 0.96 with a cutoff value of 54.5 pg/mL (sensitivity: 100.00%, specificity: 66.67%). The 95% reference interval for CRHT in healthy (control) dogs ranged 5.00 to 79.8 pg/mL (1.10‐17.57 pmol/L) for EAC, and 1.92 to 153.42 pg/mL (0.42‐33.78 pmol/L) for PAC. There was no significant correlation between PBR and CRHT, nor adrenal size and CRHT.Conclusions and Clinical ImportanceCRHT appears to be a rapid and reliable test for differentiating PDH from CPAT in dogs.

Adrenal Gland Ultrasonographic Measurements and Plasma Hormone Concentrations in Clinically Healthy Newborn Thoroughbred and Standardbred Foals.

Simple SummaryThe hypothalamic–pituitary–adrenal axis regulates many physiologic and metabolic functions and plays a central role in the inflammatory response to illness. Clinically, hypothalamic–pituitary–adrenal axis function can be evaluated by combined assessment of adrenal hormones, adrenocorticotropin plasma concentrations and ultrasonographic examination of the adrenal glands in other species. Multiple individual variables have been demonstrated to affect ultrasonographic measurements of adrenal glands in several species. Ultrasonographic measurements of the adrenal glands and plasma concentration of adrenocorticotropin, cortisol, dehydroepiandrosterone, progesterone, aldosterone and adrenocorticotropin/adrenal hormone ratios were assessed and compared between Thoroughbred and Standardbred healthy neonatal foals. The effect of age, weight and sex on ultrasonographic measurements of the adrenal glands was also investigated. The width of whole adrenal glands and the medulla differed significantly between Thoroughbreds and Standardbreds. Cortisol and adrenocorticotropin were, respectively, higher and lower in Thoroughbreds than in Standardbreds. The cortisol/dehydroepiandrosterone ratio was higher in Thoroughbreds than in Standardbreds. The results of this study provide reference ranges for healthy neonatal Thoroughbred and Standardbred foals and suggest the presence of breed-related differences in ultrasonographic measurements, plasmatic cortisol and adrenocorticotropin concentrations. The higher cortisol/dehydroepiandrosterone ratio of Thoroughbred foals may suggest a different response to stress and environmental stimulation between the two breeds.Adrenal hormones, ACTH plasma concentrations and the ultrasonographic evaluation of the adrenal glands are considered complementary in clinical evaluations of the hypothalamic–pituitary–adrenal (HPA) axis function in several species. In dogs, age, size and weight have a significant effect on the ultrasonographic size of the adrenal glands. In neonatal foals, ultrasonographic evaluation and measurements of the adrenal glands have been demonstrated to be reliable; however, the effect of individual variables on ultrasonographic features has not been investigated, and the clinical usefulness of adrenal gland ultrasonography is still not known. The aims of this study were: (i) to provide and compare adrenal glands ultrasound measurements in healthy newborn Thoroughbred and Standardbred foals, and assess any effect of age, weight and sex on them; (ii) to assess and compare ACTH and steroid hormone concentrations in healthy neonatal foals of the two breeds. Venous blood samples and ultrasonographic images of the adrenal glands were collected from 10 healthy neonatal Thoroughbred and 10 healthy neonatal Standardbred foals. Ultrasonographic measurements of the adrenal glands were obtained and adrenocorticotropin (ACTH), cortisol, dehydroepiandrosterone (DHEA), progesterone (P4) and aldosterone plasma concentrations were assessed. The ACTH/cortisol, ACTH/progesterone, ACTH/aldosterone, ACTH/DHEA and cortisol/DHEA ratios were calculated. A significant positive correlation was found between the height of the right adrenal gland and the foal’s weight; the width of the right and left adrenal gland and the medulla was significantly lower in Thoroughbreds than in Standardbreds. Cortisol and ACTH plasma concentrations were significantly higher and lower, respectively, in Thoroughbreds compared with Standardbreds. The cortisol/DHEA ratio was significantly higher in Thoroughbreds. This study provides reference ranges for neonatal Thoroughbred and Standardbred foals, and suggests the presence of breed-related differences in ultrasonographic adrenal gland measurements, plasmatic cortisol and ACTH concentrations. The higher cortisol/DHEA ratio detected in Thoroughbred foals could suggest a different response to environmental stimulation in the two breeds.

MON-LB036 Uncommon Findings in Beckwith-Wiedemann Syndrome

Introduction: Beckwith-Wiedemann syndrome (BWS) is characterized by variable phenotypes that might include overgrowth, macroglossia, abdominal wall defects, neonatal hypoglycemia, hemihypertrophy and predisposition to embryonal tumours.Some features are more specific, including adrenal cytomegaly. BWS increases risk for several tumors, including adrenocortical tumor (ACT). Some protocols recommend screening with adrenal ultrasonography and serum DHEAS every 4–6 months. Case report: A 1-month-old male, preterm (34 weeks 4 days), large for gestational age (weight 3670g), presented with macrosomia, macroglossia, umbilical hernia and auricular pits, with clinical diagnosis of BWS. In newborn screening 17 alpha hydroxyprogesterone (17OHP): 70,15 ng/mL (<15ng/mL) and 66,32 ng/mL in second sample. Presented basal cortisol 11.4 mcg/L (6,70 a 22,60 µg/dL), normal electrolytes, dehydroepiandrosterone sulphate (DHEAS) > 1000 mcg/dL (30,0 - 250,0 mcg/dL), testosterone 637 ng/dL (60-400 ng/dL), leading to ACT suspicion. No suppression of cortisol (2,8 mcg/dL) after dexamethasone 10 mcg/kg. Urinary metanephrines were normal, plasmatic normetanephrine 554 pg/mL (<196 pg/mL) and plasmatic norepinephrine 2094 pg/mL (< 420 pg/mL). Abdominal CT showed increased adrenal glands, with normal contrast uptake. MRI and PET/CT DOTA-68Ga did not identify tumors. Eight months later: 17OHP 0,88 ng/mL (0,8-5ng/mL), DHEAS 37 mcg/dL (<30,0 mcg/dL), testosterone 13 ng/mL (< 30 ng/dL), normal urinary metanephrines and plasmatic catecholamines. Conclusion: In this case congenital adrenal hyperplasia was ruled out and ACT was suspected. Imaging did not identify tumors and biochemical findings (hypercortisolism and high androgens levels) had progressive improvement. Bilateral adrenal hyperplasia may be present in BWS as a result of fetal adrenal gland delay maturation. Permanent adrenal cortex cytomegaly may be responsible for hypercortisolism. Transient cortex persistence can lead to androgens elevation and produces large amounts of DHEA, that could explain DHEAS elevation similar to that in ACT.Bilateral adrenal hyperplasia is described as a feature of BWS, but, to our knowledge, there is no similar case in literature with these biochemical findings.

Retrospective Study of Adrenal Gland Ultrasonography in Dogs with Normal and Abnormal ACTH Stimulation Test

Retrospective Study of Adrenal Gland Ultrasonography in Dogs with Normal and Abnormal ACTH Stimulation Test

Ultrasonographic adrenal gland findings in healthy semi-captive cheetahs (Acinonyx jubatus).

Cheetahs in captivity are believed to suffer from stress predisposing them to poor health. To date fecal glucocorticoids have been used as a non-invasive indicator of chronic stress. This study examines, the feasibility of transabdominal adrenal gland ultrasonography in cheetahs and determined normal adrenal measurements that can potentially be used as a more reliable indicator of chronic stress and/or adrenal function. Thirty-three adult cheetahs, aged between 2 and 13 years, accommodated in large off-display camps were examined over 9 days under general anesthesia. The adrenals were readily identified, with the right adrenal being more difficult to find and measure than the left, and were smaller than those expected in similar sized dogs. The left adrenal was shorter and slightly more oval shaped than the right with a length and cranial pole width at a 95% prediction interval of 16.3-22.4 and 4.1-8.7 mm. The same measurements for the right adrenal were 16.8-26.2 and 3.4-10.8 mm, respectively. Corticomedullary ratios were larger for the left adrenal. When corrected for body size, females had significantly longer and greater left adrenal corticomedullary ratios than males. Adrenal measurements did not correlate with left renal length, body size measurements, or enclosure size. Measurements that increased with age included the cortical and total adrenal widths. Adrenal ultrasonography offers potential benefits in assessment of individual cheetah adrenal pathology or the evaluation of stress induced adrenomegally especially in combination with other evaluations such as non-invasive fecal glucocorticoid analyses. Zoo Biol. 35:260-268, 2016. © 2016 Wiley Periodicals, Inc.

Renal and Adrenal Ultrasonography: a valuable diagnostic tool in the salt-wasting infant.

The major differential diagnosis in 'salt-wasting' infants (characterised by hyponatraemia and hyperkalaemia) is that of an adrenal or renal disorder. Appropriate management relies on rapid diagnosis, but existing guidelines do not highlight the role of ultrasonography. We describe how ultrasound may lead to a more rapid diagnosis in disorders of sex development (DSD) and other potential 'salt-wasting' infants. Ultrasonography as a diagnostic tool in infants with salt-wasting or DSD needs to be more widely recognised.

Imaging of disorder of sex development

BACKGROUND AND OBJECTIVESDisorders of sex development (DSDs), formerly termed ambiguous genitalia, are a group of conditions where the external genitalia appear abnormal. It represents a true medical and social emergency that needs a multidisciplinary team approach for elucidation. The pediatric radiologist plays an important role in defining the genital anatomy that remains one of the most important factors in sex determination, in addition to chromosomal analysis.DESIGN AND SETTINGSA retrospective study, conducted between January 1995 and December 2010.MATERIALS AND METHODSImaging studies (ultrasound, genitogram, and/or magnetic resonance imaging) were performed in patients with ambiguous genitalia, confirmed by chromosomal analysis and appropriate hormonal investigations.RESULTSThe sensitivity of ultrasound is 89.5%, while its specificity reaches 100%. Retrograde genitogram is more invasive and less sensitive, as the yield of determining the presence of a uterus ± vagina is 84.2%. However, magnetic resonance imaging (MRI) is more sensitive for gonadal tissue identification. Its specificity reaches up to 100% and can provide detailed internal structures (uterus, fallopian tubes, and gonads).CONCLUSIONUltrasound examination is still the main modality of choice for screening patients with ambiguous genitalia. It is cheap and readily available everywhere. In addition to elucidating the uterus in 89.5%, it can also give more information on the adrenal glands. However, genitography is good—84.2% in elucidating genital anatomy—but it gives no information of the gonads and it is time consuming and invasive. MRI is helpful in cases with equivocal ultrasound, reaches 100% in elucidating internal extractor, and therefore could be reserved for that.

Abdominal Aortic Chondrosarcoma in a Dog

A 14-year-old, mixed breed dog was presented with acute pain and paresis of the hindlimbs. Ultrasonography revealed an intraluminal mass and an abrupt halt of blood flow signal in the distal abdominal aorta. The mass had homogeneous hyperechoic echotexture compared with blood flow. Although clinical presentation suggested a thromboembolism and pituitary dependent hyperadrenocorticism was suspected as a predisposing cause based on adrenal function tests and ultrasonography, an aortic chondrosarcoma originating from the distal abdominal aorta was diagnosed with histologic examination. Primary aortic sarcoma is extremely rare, and extraskeletal chondrosarcoma is only reported in 2 cases previously. Aortic neoplasm should be included in differential diagnosis when an intraluminal aortic mass is observed on ultrasonography and acute paresis of hindlimbs is shown.

A Case Study of Virilizing Adrenal Tumor in an Adolescent Female Elite Tennis Player—Insight Into the Use of Anabolic Steroids in Young Athletes

A 14-year-old Caucasian girl was referred to the endocrine clinic for evaluation of voice deepening, facial hirsutism, and acne starting 2 years previously. She had been a competitive tennis player since age 7 years, practicing for 4-6 hours daily. On physical examination she was noticed to have a masculine appearance with mild facial acne and moderate hirsutism. Tanner stage was 1 for breast tissue and 5 for pubic hair. Her androgen levels (testosterone, androstenedione, dehydroepiandrosterone sulfate) were extremely elevated. Adrenal ultrasonography revealed a round left 4.6 × 5.3-cm adrenal mass. Laparoscopic left adrenalectomy was performed. The histologic findings were compatible with a benign adrenocortical tumor. Postoperatively, androgen levels dropped to within the normal range. Breast development proceeded normally, menarche occurred 2 months after tumor resection, and menses has been regular since then. Muscle strength of the dominant and nondominant upper and lower extremities was measured 1 month before surgery and 1 year later, using an isokinetic dynamometer (Biodex Systems II, Biodex, Shirley, NY, USA). There was no significant decrease in overall muscle strength after removal of the virilizing tumor and the marked drop in circulating androgens. In addition, the patient maintained her age category, number 1, national tennis ranking. The results suggest that even extremely high levels of tumor-related circulating androgens had no evident effect on muscle strength and competitive performance in a female adolescent tennis player. The lack of beneficial effect on performance in adolescents, combined with the potentially hazardous side effects of anabolic steroids, suggests that teenage athletes should avoid their use.

Ultrasonographic evaluation of adrenal glands in dogs with primary hypoadrenocorticism or mimicking diseases

The adrenal glands of 30 dogs with primary adrenal insufficiency (hypoadrenocorticism) were measured ultrasonographically and compared with those of 14 healthy dogs and those of 10 dogs with diseases mimicking...

Ultrasonography Criteria for Differentiating ACTH Dependency from ACTH Independency in 47 Dogs with Hyperadrenocorticism and Equivocal Adrenal Asymmetry

Adrenal ultrasonography (US) in dogs with hyperadrenocorticism (HAC) is commonly used to distinguish adrenocorticotropic hormone (ACTH)-independent (AIHAC) and ACTH-dependent hyperadrenocorticism (ADHAC). To date, no cut-off values for defining adrenal atrophy in cases of adrenal asymmetry have been determined. Given that asymmetrical hyperplasia is sometimes observed in ADHAC, adrenal asymmetry without ultrasonographic proof of adrenocortical tumor such as vascular invasion or metastasis can be equivocal. The purpose of this study was to compare adrenal US findings between cases of ADHAC and AIHAC in dogs with equivocal adrenal asymmetry (EAA), and to identify useful criteria for their distinction. Forty dogs with EAA were included. Ultrasound reports of HAC dogs with adrenal asymmetry without obvious vascular invasion or metastases were reviewed. Dogs were classified as cases of ADHAC (n = 28) or AIHAC (n = 19), determined by plasma ACTH concentration. The thickness, shape, and echogenicity of both adrenal glands and presence of adjacent vascular compression were compared between AIHAC and ADHAC groups. The maximal dorsoventral thickness of the smaller gland (SDV) ranged from 2.0 to 5.0 mm in AIHAC and from 5.0 to 15.0 mm in ADHAC. The 95% confidence intervals for estimated sensitivity and specificity of a SDV cut-off set at 5.0 mm in the diagnosis of AIHAC were 82-100 and 82-99%, respectively. Other tested US criteria were found to overlap extensively between the 2 groups, precluding their usefulness for distinction. In EAA cases, an SDV ≤5.0 mm is an appropriate cut-off for AIHAC ultrasonographic diagnosis.

Sonography of adrenal gland diseases in the newborn infant

Diseases and morphological changes of the adrenal gland play an important role especially in the newborn, because malformations, congenital errors of metabolism, connatal tumours and birth injuries become symptomatic in this period of life. In this paper, the possibilities of neonatal adrenal ultrasonography for diagnosis and in the aspect of differential diagnosis are discussed. The characteristic sonographic appearance is the thin echogenic core surrounded by a thick transonic zone. Adrenal haemorrhage as well as the neuroblastoma disturb these structures and show their own characteristic pattern. In congenital adrenal hyperplasia the organ is enlarged in a fold-like manner. In agenesis or displacement of the kidney the adrenal gland presents as an elongated mass in the renal fossa and on the psoas muscle.

Use of endogenous ACTH concentration and adrenal ultrasonography to distinguish the cause of canine hyperadrenocorticism.

Twenty-nine dogs were diagnosed with hyperadrenocorticism (HAC). A single determination of endogenous plasma adrenocorticotropic hormone (ACTH) and adrenal ultrasonography were used in a prospective study to differentiate between pituitary-dependent HAC (PDH) and adrenal-dependent HAC (ADH). In 27 out of the 29 dogs (93 per cent), both endogenous plasma ACTH concentrations and adrenal ultrasonography indicated the same cause of HAC. Twenty-one of the 29 cases (72 per cent) were shown to be pituitary-dependent; all had plasma ACTH concentrations of greater than 28 pg/ml (reference range 13 to 46 pg/ml) and both adrenal glands were ultrasonographically of similar size and of normal shape. All 21 cases responded well to mitotane therapy. Six cases (21 per cent) were shown to be adrenal-dependent; all had plasma ACTH concentrations below the limit of the assay (<5 pg/ml) and the presence of an adrenal mass on ultrasonography. The sensitivity and specificity of adrenal ultrasonography and endogenous ACTH determinations to identify the cause of HAC were demonstrated to be 100 per cent and 95 per cent, respectively, for ADH. These discriminatory tests are more accurate than published figures for dexamethasone suppression testing.

CAH: Diagnostic Utility of Adrenal Ultrasonography

CAH: Diagnostic Utility of Adrenal Ultrasonography

Clinical utility of adrenal ultrasonography in the diagnosis of congenital adrenal hyperplasia

Rapid, accurate diagnosis of congenital adrenal hyperplasia (CAH) is essential in the neonate with ambiguous genitalia, life-threatening salt loss, or both. We aimed to determine the accuracy of adrenal ultrasonography in the diagnosis of CAH in a retrospective analysis of 52 children with ambiguous genitalia or salt-losing crises. Adrenal ultrasounds were interpreted as follows: “normal” if the adrenals were normal in size (adrenal limb width <4 mm), had a smooth surface, and a central echogenic stripe with a hypoechoic rim, and “abnormal” if they were increased in size (limb width >4 mm), had a lobulated or cerebriform surface, or showed abnormal echogenicity. Group 1 consisted of 25 neonates and infants with CAH; group 2, 19 children with conditions other than CAH; and group 3, 8 with treated CAH: 7 receiving replacement therapy and 1 whose mother received glucocorticoids during pregnancy. In all children in groups 2 and 3, adrenal ultrasounds were read as normal. In group 1 adrenal ultrasonography was normal in 2 (8%) and abnormal in 23 (92%). Thus adrenal ultrasonography has a sensitivity of 92% and a specificity of 100% for diagnosing CAH. Adrenal ultrasonography is a highly sensitive and specific adjunct in the diagnosis of CAH. The presence of enlarged, lobulated adrenals with stippled echogenicity is invariably associated with CAH. (J Pediatr 1999;135:71-5.)

The Efficacy of L‐Deprenyl in Dogs with Pituitary‐Dependent Hyperadrenocorticism

Ten dogs with pituitary‐dependent hyperadrenocorticism (PDH) received 2 mg/kg of L‐Deprenyl once daily for 6 months. Monthly patient assessment consisted of evaluation of the owner's daily observation protocol, a standardized owner questionnaire, physical examination, CBC, biochemical profile, determination of the urine cortisol/creatinine ratio (UC/C), low‐dose dexamethasone suppression (LDDS) test, corticotropin releasing hormone (CRH) test, and adrenal ultrasonography. At the beginning and the end of the study, an adrenocorticotropic hormone (ACTH) stimulation test and computed tomography also were performed. Two dogs developed neurologic signs and 2 dogs developed acute pancreatitis. An increase in activity, decrease in polyphagia, and decrease in panting were reported by 6, 4, and 2 owners, respectively. Seven owners believed that water intake decreased, but this was confirmed in only 3 dogs. Water intake increased in 2 dogs and remained unchanged in 5 dogs. The condition of the hair coat and skin improved in 2 dogs, worsened in 3, and remained unchanged in 5. Urine specific gravity, urine osmolality, ACTH test results, UC/C, and adrenal gland size did not change significantly throughout the study. In 4 of 8 dogs, LDDS was abnormal at the start of the study but normal at the end of the study, and in 2 dogs, the opposite occurred. Marked individual variation was noted in the CRH test, with a tendency for smaller increases in ACTH toward the end of the study. A marked increase in hypophyseal tumor size occured in 4 dogs. Treatment with L‐Deprenyl resulted in improvement, deterioration, and stagnation of clinical signs in 2, 4, and 4 dogs, respectively. The results of this study indicate that L‐Deprenyl cannot be recommended as the sole treatment for canine PDH.

Serial Adrenal Ultrasonography in Normal Neonates

Serial Adrenal Ultrasonography in Normal Neonates