Data on four species of Longidorus Micoletzky, 1922 (Nematoda: Longidoridae) from southern and southeastern Iran, including description of a new species.

Four species of the genus Longidorus were recovered from southern (Bushehr province) and southeastern (Southern Khorasan province) Iran. The first species, L. paratabrizicus n. sp. represents a new member to the genus and is characterised by 4.8-5.6 mm long females with anteriorly flattened lip region separated from the rest of the body by depression, amphidial fovea pocket-shaped without lobes, tail conical, dorsally convex, ventrally almost straight with bluntly rounded tip and males in population. By having similar lip region and tail shape, the new species most closely resembles five species viz. L. artemisiae, L. globulicauda, L. patuxentensis, L. sturhani, and L. tabrizicus. It represents the cryptic form of the last species. The second species belongs to L. mirus, recovered in both southern and southeastern Iran, representing the first record of the species after its original description. As an update to the characteristics of this species, it's all juvenile developmental stages were recovered and described. The criteria to separate L. mirus from two closely related species, L. auratus and L. africanus, are discussed. The third species belongs to L. persicus, a new record in southern Iran. The fourth species, L. orientalis was recovered in high population density in association with date palm trees in Bushehr province. The phylogenetic relationships of the new species and recovered populations of L. mirus and L. persicus were reconstructed using two ribosomal markers and the resulted topologies were discussed.

Neurosurgical treatment of Nelson's syndrome.

Total bilateral adrenalectomy remains the definitive procedure for cure in Cushing's disease. It is complicated by the development of Nelson's syndrome, the treatment of which remains troublesome. We report the long-term follow-up, median 17 yr (range, 8-22 yr), of 13 patients (3 males and 10 females) treated with pituitary surgery for Nelson's syndrome at a median age of 35 yr (range, 21-67 yr). The presence of a pituitary mass lesion necessitated neurosurgery in all. Preoperatively, the median plasma ACTH level was 664 pmol/liter (range, 92-3665 pmol/liter); this fell to 29 pmol/liter (range, <2 to 1124 pmol/liter) postoperatively (P < 0.0005). Cutaneous hyperpigmentation was reduced in all and resolved in 11 patients. The pituitary tumor bulk was clearly reduced in 12 patients. There was no perioperative mortality. No patient developed a visual field defect attributable to surgery. New anterior pituitary hormone deficiency occurred in seven patients, and permanent diabetes insipidus occurred in five patients. At latest follow-up, the median plasma ACTH is 73 pmol/liter (range, <2 to 7759 pmol/liter); six patients have levels of less than 44 pmol/liter and also have a resolution of their pigmentation and no evidence of a recurrence of a pituitary mass lesion. We conclude that pituitary surgery is an efficacious treatment for mass lesions associated with Nelson's syndrome, has long-term benefit with minimal side effects, and must be considered in the management of this distressing complication.

Clinical, radiological and pathological features of patients with Rathke's cleft cysts: tumors that may recur.

Rathke's cleft cysts are cystic sellar and suprasellar lesions, characteristically lined by a single layer of ciliated cuboidal or columnar epithelium. In contrast, craniopharyngiomass, which are also cystic sellar and suprasellar lesions, are characteristically lined by stratified squamous epithelium with keratinization on a layer of connective tissue. The usual management recommended for Rathke's cleft cysts is simple surgical drainage with partial excision of the cyst wall. Recurrences of these cysts reportedly have been very rare. This retrospective study presents the details of 12 patients (6 females; median age 30 yr, range 21-58 yr) with Rathke's cleft cyst, referred to our department over a 15-yr period (1981-1996), an unusual feature being the recurrence of 4 (33%) of these lesions. Clinical, endocrine, radiological, surgical (10 transsphenoidal; 2 transcranial), and pathological details were recorded. Nine out of 12 patients (75%) were symptomatic; visual symptoms were the commonest, and 8 had visual field defects. The median duration of symptoms was 12 months (range 3-24 months). Three patients (25%) had panhypopituitarism, 2 of whom also had diabetes insipidus (17%). The cysts varied in size from 6 mm to 50 mm, 1 being entirely suprasellar. There were no pathognomonic clinical or radiological features to differentiate them from other pituitary lesions, although the presence of diabetes insipidus in 2 patients suggested that the lesion was not a pituitary adenoma. A definite histological diagnosis was possible in 8 patients; in 4, the diagnosis was presumptive. The median duration of follow-up was 30 months (1-168 months). Four patients (33%) showed reexpansion at 3, 6, 48, and 48 months after initial surgery, 3 of whom were symptomatic and required repeat surgery. Two of these patients were given postoperative external beam pituitary radiotherapy. Apparent recurrence of Rathke's cleft cysts after initially successful surgery in our series was higher than suggested by previous reports, and thus, long-term follow-up with pituitary imaging and neuroophthalmological assessment is essential. There are no specific characteristics of the cyst that predict recurrence. Ideal management of these cysts is unclear, but aspiration, followed by extensive excision of the cyst wall when possible, seems to be the best initial option. For recurrent symptomatic tumors, surgical resection is the treatment of choice. Considering the high recurrence rate with residual structural and functional dysfunction, the role of radiotherapy in preventing recurrence of these cysts needs careful evaluation with a larger study with a longer follow-up period.

Relative contributions of inferior petrosal sinus sampling and pituitary imaging in the investigation of children and adolescents with ACTH-dependent Cushing's syndrome.

Selective transsphenoidal microadenomectomy is the first line treatment of childhood Cushing's disease, with accurate preoperative localization of the corticotroph adenoma an important step in its investigation. Inferior petrosal sinus sampling (IPSS) for ACTH after CRH stimulation is a recognized investigation in adults, but there are few data in the pediatric age range. We report the relative contributions of IPSS and pituitary imaging in 11 patients, aged 10.7-18.8 yr, presenting with Cushing's disease. All underwent transsphenoidal surgery (TSS). IPSS was performed without complication. Sampling was from the inferior petrosal sinuses in 7 patients and the high jugular veins in 4 (patients 2, 4, 5, and 10). The central to peripheral ACTH (IPS/P) ratios were more than 2 (2.5-157.2) in 10 of 11 patients, confirming central ACTH secretion. In 3 patients with high jugular sampling, IPS/P ratio ranged from 2.5-21.1. In the fourth patient with high jugular sampling (IPS/P ratio, 0.95), a central adenoma was identified surgically, and the patient was cured after TSS. The interpetrosal sinus ACTH gradient (IPSG) was more than 1.4 (2.1-20.8) in 10 patients, indicating lateralization of ACTH secretion to the right side in 6 patients and to the left in 4. IPSG ratios were 2.1-8.5 in 3 patients with high jugular sampling. Pituitary imaging (computed tomography and or magnetic resonance imaging) was reported to identify an adenoma in 5 of 11 patients. At operation a tumor was visualized by the same surgeon in all 11 cases. In 9 patients with lateralization on IPSS, the correct side of the tumor was confirmed at surgery. In a 10th patient with a negative IPSG, a central tumor was present. Thus, IPSS gave a 91% prediction of correct tumor localization. In only 1 of 5 patients with an adenoma reported on pituitary imaging was this localization confirmed at surgery, a prediction rate of only 9%. After TSS, 8 patients were cured, 1 was in remission, and 2 required pituitary irradiation. In 73% of patients undergoing IPSS, localization of the adenoma was followed by surgical cure or remission. Pituitary scanning was therefore relatively unhelpful in localizing the adenoma. In experienced hands, however, IPSS was feasible in this age group, safe, and strongly predictive of the site of the adenoma, leading to a high rate of successful surgical outcome.

Linear growth and final height after treatment for Cushing's disease in childhood.

Cushing's disease is associated with growth failure in childhood and adolescence. Growth and final height were analyzed in 10 patients who were cured or in remission after treatment of Cushing's disease. Seven males and 3 females, aged 6.8-17.6 yr (bone age, 3.3-15.4 yr), had transsphenoidal surgery, which was combined with pituitary irradiation (4,500 cGy in 25 fractions) in 5 patients. At presentation, 5 patients were prepubertal (males), and 5 were pubertal (2 males and 3 females). The mean height SD score was -2.15 +/-1.26 (range, -0.21 to -4.32) compared with mean target height SD score of -0.43 +/- 0.58. Height velocity in 6 patients was subnormal (0.9-3.8 cm/yr). After treatment, short-term height velocity, over a mean interval of 0.57 yr, in 8 patients not receiving human GH (hGH) therapy, was variable (range, 0.8-7.6 cm/yr). GH stimulation tests (insulin tolerance test/glucagon) in 9 subjects showed peak GH levels of 0.5-20.9 mU/L. Eight were treated with hGH (14 IU/m2 wk), combined in 2 girls and 1 boy with a GnRH analog. After 1 yr of hGH, the mean height SD score had increased from -2.45 +/- 1.0 at initiation of hGH to -2.07 +/- 1.2 (P = 0.01). GH therapy was continued until final height or latest assessment. The mean final height SD score (n = 6) was - 1.24 +/- 1.38, and at the latest assessment the mean height SD score (n = 4) was - 1.52 +/- 1.33. Combining these 2 groups, the mean height so score was -1.36 +/- 1.29. The difference between final or latest height SD score and target height SD score was 0.93 +/- 1.13, i.e. less (P = 0.005) than the difference between height and target height SD score of 1.72 +/- 1.26 at presentation. In conclusion, catch-up and favorable long-term growth was seen after treatment for Cushing's disease. Posttreatment GH deficiency was frequent, and early hGH replacement may have contributed to the encouraging outcome.

Predictors of the outcome of surgical treatment in acromegaly and the value of the mean growth hormone day curve in assessing postoperative disease activity.

Acromegaly is associated with increased morbidity and mortality unless serum GH levels are persistently less than 5 mU/L ( approximately 2 ng/mL) after treatment. Transsphenoidal surgical resection is the best available treatment for restoring GH to such "safe" levels; however, criteria for the assessment of the response to treatment are not uniform. To determine the clinically most useful method of assessing disease activity postoperatively and identify predictors of a favorable response to surgical treatment, we have analyzed 67 patients with acromegaly who underwent transsphenoidal surgery between 1993 and 1998. We used three different definitions of a satisfactory or safe response: 1) a postoperative mean GH less than 5 mU/L obtained from averaging five serum GH values obtained throughout one day; 2) a random single GH less than 5 mU/L; or 3) a serum insulin-like growth factor I (IGF-I) level within the normal range. Relying on a single GH measurement alone, 9 of the 23 patients with a single postoperative mean GH level less than 5 mU/L obtained at least one GH value of more than 5 mU/L (false positive rate, 28%) and 8 of the patients with a postoperative mean GH value of more than 5 mU/L obtained a single GH value of less than 5 mU/L (false negative rate, 15%). Postoperatively, a significant increase in the fluctuation of random GH values around the mean was observed in patients who were rendered safe (coefficient of variation, from 26 +/- 2% to 53 +/- 6%; P < 0.001) compared with patients with persistence of inadequately controlled disease. However, 13% (3 of 23) of patients with mean postoperative GH levels of less than 5 mU/L had elevated serum IGF-I levels postoperatively, and 17% (8 of 44) of patients with mean serum GH levels more than 5 mU/L had postoperative IGF-I levels within the normal range. There was no difference in the rate of agreement between mean GH less than 5 mU/L and normalization of IGF-I in relation to the interval since operation when IGF-I levels were measured. Preoperative tumor size and pretreatment mean GH levels were the major determinants of the outcome of surgery, as patients who were rendered safe had significantly lower preoperative mean GH levels than patients who were not cured (median, 31 mU/L vs. 78.5 mU/L, P < 0.01). IGF-I levels were weakly correlated with tumor size and could not be used to predict the patients who would be rendered safe. Preoperative PRL levels were higher in patients who failed to achieve a surgical satisfactory outcome [498 mU/L (187-857) vs. 196 mU/L (136-315), P < 0.01]. In summary, although single random GH values and IGF-I values are both significantly correlated with mean GH levels, they should not be used as an alternative to averaging several GH values to assess disease activity, because of the pulsatile nature of GH secretion and the multiple factors that may influence serum IGF-I. Because significant discrepancies occur, particularly postoperatively, mean GH levels remain the more reliable indicator of surgical outcome and disease activity. As there is considerably more evidence relating long-term prognosis to serum GH levels than to IGF-I and discrepancies occur between GH levels and IGF-I, we suggest that mean serum GH levels and single IGF-I levels, measured early in the postoperative period, are currently the best biochemical guide to the adequacy of surgery and, hence, the need for further treatment.

A critical analysis of the value of simultaneous inferior petrosal sinus sampling in Cushing's disease and the occult ectopic adrenocorticotropin syndrome.

The clinical, biochemical, and radiological features of pituitary ACTH-dependent Cushing's syndrome (CS) [Cushing's disease (CD)] are often indistinguishable from those of occult ectopic ACTH-dependent CS (oEAS). We have evaluated, retrospectively, the results of simultaneous bilateral inferior petrosal sinus (IPS) ACTH sampling before and after CRH stimulation in 128 patients with ACTH-dependent CS: 107 patients with CD, 6 with oEAS, 1 with an adrenal adenoma, 1 with a pituitary gangliocytoma, and 1 with Nelson's syndrome; while, in the remaining 12, the source remains unclear. One hundred seven patients received human-sequence CRH (hCRH), and 11 received ovine CRH; another 6 patients underwent stimulation with desmopressin and hCRH, and 4 with desmopressin alone. A successful bilateral IPS catheterization and sampling (IPSCS) rate of 87.5% was obtained only after considerable experience had been gained. Sixty-nine patients with CD underwent successful bilateral IPSCS: the IPS-to-peripheral ratio of plasma ACTH value (IPS/P) rose from 9.5 +/- 1.4 to a maximum ratio of 55.8 +/- 7.5 in 67 patients, after CRH stimulation. The maximum ratio was obtained at 5 min in 60 of the 69 patients with CD; however, all 69 patients obtained a ratio of more than 2, at that time. In contrast, the 6 patients with occult ACTH-secreting neoplasms had a maximal IPS/P ratio of 1.3 +/- 0.16, and this did not change after CRH stimulation. A bilateral IPS/P ratio more than 2, obtained 5 min after CRH stimulation, had a sensitivity of 97% and a specificity of 100% in diagnosing CD. Two patients with proven active CD had an IPS/P ratio of less than 2. After successful bilateral IPSCS, the gradients between the IPS ACTH concentrations [IPS ACTH gradient (IPSG)] of more than 1.4, at 5 min after CRH stimulation, had a sensitivity of 83% in correctly lateralizing the pituitary microadenoma, compared with 72% sensitivity for magnetic resonance imaging (MRI) scanning. Furthermore, when IPSG and MRI findings were contradictory, IPSG was more often correct than MRI scanning. Although oEAS is a relatively uncommon cause of ACTH-dependent hypercortisolism (5.5% in our series), the accurate diagnosis of ACTH-dependent CS and localization of an intrapituitary microadenoma requires bilateral IPSCS with CRH stimulation, provided that the appropriate technical experience is available. hCRH is as effective a secretagogue as ovine CRH, and either may be used. The value of the combination of CRH and desmopressin stimulation requires more detailed investigation.

Diagnosis and selective cure of Cushing's disease during pregnancy by transsphenoidal surgery.

The diagnosis of Cushing's Disease during pregnancy is complex because the biochemical features are obscured by changes in the normal hypothalamo-pituitary-adrenal axis that occur during gestation. To date, treatment has not been successful and there is a high incidence of maternal and fetal complications. We report the case of a 24-year-old woman with Cushing's disease who presented during her 16th week of pregnancy. Diagnosis was confirmed by the finding of elevated serum and urinary free cortisol levels with loss of the normal circadian rhythm of serum cortisol. Cortisol levels failed to suppress after a low-dose dexamethasone test but suppressed after a high-dose test. There was an exaggerated serum cortisol and plasma adrenocorticotrophin (ACTH) response to corticotrophin-releasing hormone (CRH). Magnetic resonance (MR) scanning demonstrated a pituitary tumour and cure was effected by transsphenoidal surgery where tumour immunostaining for ACTH was removed. Postoperatively the patient made an uncomplicated recovery; serum cortisol and plasma ACTH levels were undetectable at 9 days following surgery and recovery of the hypothalamo-pituitary axis occurred at 99 days after surgery. Caesarean section was performed at 38 weeks of pregnancy and a healthy but small female infant was delivered. This case illustrates the biochemical features of Cushing's disease during pregnancy and is the first report of the use of CRH testing and MR scanning in this clinical situation. The cure by surgery and successful outcome for mother and infant, with preservation of normal anterior pituitary function, suggest that transsphenoidal surgery may be the treatment of choice.

Computerised axial tomography and magnetic resonance scanning in the Tolosa-Hunt syndrome.

A 50-year-old Asian male presented with a left sixth nerve palsy, left temporal pain, and rapidly deteriorating visual acuity in the left eye. A high resolution CT scan and magnetic resonance scan showed a left retro-orbital enhancing lesion extending from the lateral margin of the cavernous sinus on to the greater wing of the sphenoid and into the left orbit. Arteriography was normal. On high dose steroid therapy there was total resolution of the lesion. The value of imaging techniques in this condition is discussed.

Intraventricular fetus-in-fetu. Case report.

The authors present a case of an intraventricular 14-cm fetus in a 6-week-old infant who presented with enlarging head size. The entire intracranial fetus, with recognizable limbs, trunk, head, and exomphalos, was removed totally from the lateral and third ventricles, with excellent recovery in the patient, who is now 18 months old. The cytogenetical and tissue histological studies are reported. The embryology, operative procedure, and pathology of this condition are discussed and the literature briefly reviewed.

A brainstem stereotactic atlas in a three-dimensional magnetic resonance imaging navigation system: first experiences with atlas-to-patient registration.

OBJECT: The authors describe a computer-resident digital representation of a stereotactic atlas of the human brainstem, its semiautomated registration to sagittal fast low-angle shot three-dimensional (3-D) magnetic resonance (MR) imaging data sets in 27 healthy volunteers and 24 neurosurgical patients, and an analysis of the subsequent transforms needed to refine the initial registration. METHODS: Contour drawings from the atlas, which offer the 70th percentile of variation of anatomical structures, were interpolated into an isotropic 3-D representation. Initial atlas-to-patient registration was based on the fastigium/ventricular floor plane reference system. The quality of the fit was evaluated using superimposition of the atlas and MR images. If necessary, the atlas was tailored to the individual anatomy by using additional transforms. On average, the atlas had to be stretched by 2 to 6% in the three directions of space. Scale factors varied over a broad range from -8 to +19% and the benefit of visual interactive control of the atlas-to-patient registration was evident. Analysis of distances within the pons measured in the midsagittal MR imaging slices and the required scale factors revealed significant correlations that may be used to reduce the amount of user interaction in the coregistration substantially. In 70.6% of the cases, the atlas had to be shifted in a cranial direction along the brainstem axis (in 25.5% of cases 3-4 mm, in 45.1% of cases 1-2 mm). This was due to a more caudal position of the fastigium cerebelli on the MR images compared with the atlas. CONCLUSIONS: This observation, in conjunction with the variability of the height of the fourth ventricle in our MR imaging data (range 6.1-15.2 mm, mean 10.1 mm, standard deviation 1.8 mm) calls into question the role of the fastigium cerebelli as an anatomical landmark for localization within the brainstem.

Global and regional cerebral blood flow. Noninvasive quantitation in patients with subarachnoid hemorrhage.

Since inert gas washout techniques for cerebral blood flow (CBF) measurement are not applicable to asymmetrical pathological states where assumptions about constancy of distribution, volume, and partition coefficients cannot be held to be valid, a new approach to regional and global CBF is described. Regional volume and regional mean transit time are measured independently to give regional volume flow in ml/min. In a pilot study, this noninvasive, quantitative technique has been applied to 37 patients with various clinical responses to subarachnoid hemorrhage. Global CBF and clinical grading were significantly associated. Reduction of regional CBF and spasm of the relevant arterial supply were also significantly associated in these patients.

Paediatric brachytherapy. II. Brain implantation.

The case histories of four children with brain tumours, for whom stereotactic brachytherapy was indicated, are presented from the St Bartholomew's Hospital intracranial brachytherapy programme. The recent evolution of the computed-tomography-directed stereotactic technique is described and the uses of different radionuclides (198Au, 192Ir) are discussed. The future of brachytherapy for paediatric brain tumours is debated.

Results of the use of a new water-soluble contrast medium (metrizamide) in the posterior fossa of the baboon.

An investigation was made of the use of a new water-soluble contrast material (metrizamide) for posterior-fossa radiography in the baboon. The experiments showed that the concentration of metrizamide needed to produce adequate radiographs from sub-occipital injections into the posterior fossa always produced generalized seizures. Intraventricular injection produced excellent radiographs from metrizamide concentrations, which did not result in either clinical or EEG seizure activity. Seizures, when they occurred, were easily controlled with pentobarbitone and phenytoin. In all the experiments, ECG changes were found, which disappeared after some hours.

The microsurgical treatment of lumbar disc protrusion. Follow-up of 60 cases.

The technique and results of microsurgical techniques for lumbar disc protrusions in 60 patients are presented. All patients were followed up for a minimum of 2.5 years, and 91% had good or excellent results. The only patient with a recurrent disc sequestration had a good result after a second operation.

Computer-generated three-dimensional visualization of the trigeminal nuclear complex.

The spatial configuration of the trigeminal nuclear complex has been determined using new computer techniques aided by color graphic display facilities. Precise, statistical computer-generated drawings are provided of the three-dimensional anatomy of the nuclear complex and spinal tract of the fifth cranial nerve, as well as adjacent structures. Techniques for viewing these structures from variable directions and producing stereopairs are presented.

Delayed intracerebral haematomas in moderate to severe head injuries in young adults.

The incidence of delayed intracerebral haematoma formation in moderate to severely head-injured young adults who do not have signs of cerebral contusions on the initial post-trauma head CT scan has been studied. Thirty patients were reviewed and in 6 (20%) a delayed haematoma was diagnosed on a later CT scan, when signs of cerebral contusions were absent initially. Recurrence of intracranial hypertension within 3 to 4 h proved to be a significant indication for the development of these haematomas, despite the initial but inconsistent good response to osmotic diuretics. The average Glasgow outcome score was 3, including two mortalities. Cerebral perfusion pressure directly correlated with the outcome.

Balancing global city with global village.

The discourse on habitat and human settlements is increasingly dominated by that of the global, mega-city. If the aim of those of us in the human settlements field are to improve our understanding of and action on habitat and human settlements, this, often exclusive, focus is a mistake. Our habitat is better understood and acted upon as a network of interwoven settlements and surrounding countryside, large and small, themselves interwoven within our larger ecosystem. In this network the habitat of our smaller settlements (smaller cities, towns, villages) and rural areas and our global village also plays a critical role. The assumption can be challenged that urbanization and the mega-city are the critical issues of habitat and human settlements. Evidence suggests that with some rurbanization there is also counter urbanization, the ruralization of cities, and, perhaps most important, a growing urbanization as city-country interactions intensify. Habitat for all and habitat for a healthy, sustainable planet demands our openness to these new concepts and realities. It demands an integrated, balanced, approach which helps a wide range of living settlement nodes, large and small, to nurture each other. Global City and Global Village must go hand in hand. Our planetary and human health depends on it.

Chemical composition and general toxicity of essential oils extracted from the aerial parts of Artemisia armeniaca Lam. and A. incana (L.) Druce growing in Iran.

The essential oils of the aerial parts of A. armeniaca and A. incana, collected from Arasbaran area (East Azarbaijan province, Iran) were extracted by hydrodistillation and analyzed by GC-MS. In total, 16 and 40 constituents were identified and quantified in the oils of A. armeniaca and A. incana representing 80.5% and 84.6% of the oils, respectively. The essential oil of A. armeniaca was mainly composed of non-terpene hydrocarbons (24.8%). The major components of the oil were α-pinene (10.7%), nonadecane (10.0%), 6,10,14-trimethyl-2-pentadecanone (9.4%), spathulenol (7.8%) and Z-verbenol (5.8%). The essential oil of A. incana was dominated by oxygenated monoterpenes (41.6%), with camphor (20.4%), 1,8-cineol (10.3%), Z-verbenol (8.7%), ß-thujone (8.3%) and α-thujone (5.6%), as major components. The essential oils were also subjected to general toxicity assay using brine shrimp lethality method. The toxicity profile of both oils indicated some degree of toxicity in comparison with podophyllotoxin.

Use of intravenous etomidate to control acute psychosis induced by the hypercortisolaemia in severe paediatric Cushing's disease.

BACKGROUND: Psychosis secondary to paediatric Cushing's disease (CD) is extremely rare and presents a significant management challenge. METHOD: We report a 14.7-year-old CD patient with acute psychosis and self-inflicted injuries following failed transsphenoidal pituitary surgery. Her mental state rapidly deteriorated precluding medical therapy. RESULTS: Emergency intravenous low-dose etomidate infusion (3-3.5 mg/h) with dose titration according to the serum cortisol combined with a hydrocortisone infusion, in an intensive care setting, was effective in controlling the hypercortisolaemia. Her mental state improved with normalisation of her cortisol levels enabling oral administration of ketoconazole and bilateral adrenalectomy to be performed. CONCLUSION: This case illustrates the safe and effective use of a low-dose etomidate infusion in an unusual case of paediatric CD.