Kallman syndrome versus idiopathic hypogonadotropic hypogonadism at MR imaging.

Vogl, Thomas J.; Stemmler, J; Heye, Babette; Schopohl, Jochen; Danek, Barbara A.; Bergman, Cody; Balzer, J O; Felix, R

doi:10.1148/radiology.191.1.8134597

Cited by 73 publications

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“…Changes in the frontal basal regions have been consistently described in the rhinencephalon and contiguous cerebral cortical and bone structures by pathology, 5,6 conventional MR imaging, [7][8][9][10][11][12][13][14] and CT studies in patients with KS. 15 Increased volume of the corticospinal tracts and corpus callosum has been also depicted by conventional MR imaging, 28 suggesting the involvement of brain structures beyond the basal forebrain, though these findings have not been confirmed in larger series.…”

Section: Discussionmentioning

confidence: 99%

“…3,4 The failure of the migration process results in hypo-/aplasia of the rhinencephalon (olfactory bulbs and tracts) [3][4][5][6] and in altered gonadotropic axis function with low levels of sex hormones. Besides rhinencephalon abnormalities, distinctive KS neuroradiologic changes have been detected in the brain and bone structures of the anterior cranial fossa by conventional MR imaging [7][8][9][10][11][12][13][14] and CT studies. 15 The most known morphologic brain feature is the reduction in depth and length of the olfactory sulcus, which typically turns medially, opening anteriorly into the interhemispheric fissure.…”

mentioning

confidence: 99%

“…15 The most known morphologic brain feature is the reduction in depth and length of the olfactory sulcus, which typically turns medially, opening anteriorly into the interhemispheric fissure. [7][8][9][10][11][12][13] This sulcal abnormality is thought to be driven by the absence/hypoplasia of the olfactory bulbs and represents an intriguing model of genetically driven developmental brain abnormalities. Furthermore, a few case reports postulated the strict relationship between KS and midline brain abnormalities, such as corpus callosum agenesis and holoprosencephaly, 6,16 though pathologic and neuroimaging data are relatively scarce and often contradictory.…”

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confidence: 99%

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Brain Changes in Kallmann Syndrome

Manara

Salvalaggio

Favaro³

et al. 2014

American Journal of Neuroradiology

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BACKGROUND AND PURPOSE:Kallmann syndrome is a rare inherited disorder due to defective intrauterine migration of olfactory axons and gonadotropin-releasing hormone neurons, leading to rhinencephalon hypoplasia and hypogonadotropic hypogonadism. Concomitant brain developmental abnormalities have been described. Our aim was to investigate Kallmann syndrome-related brain changes with conventional and novel quantitative MR imaging analyses.

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Section: Discussionmentioning

confidence: 99%

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Brain Changes in Kallmann Syndrome

Manara

Salvalaggio

Favaro³

et al. 2014

American Journal of Neuroradiology

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“…5, patients with basal testosterone , 1.7 nmol/l and DT/hCG , 9 nmol/l must have an MRI investigation because it can reveal a pituitary or supra-pituitary tumor in patients with IHH. When there is evidence for Kallmann's syndrome (anosmia and/or family history), MRI is of no use since finding of olfactory bulb aplasia (33) would not change the management of the hypogonadism. For the moment, the majority of cases of IHH with normal MRI corresponds with idiopathic IHH for which hormonal investigations are still mandatory.…”

Section: Discussionmentioning

confidence: 99%

The human chorionic gonadotropin test is more powerful than the gonadotropin-releasing hormone agonist test to discriminate male isolated hypogonadotropic hypogonadism from constitutional delayed puberty

Degros

Cortet‐Rudelli²,

Soudan³

et al. 2003

European Journal of Endocrinology

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Objective: The effectiveness of biological investigations aiming at discriminating isolated hypogonadotropic hypogonadism (IHH) from constitutional delayed puberty (CDP) in male patients is still controversial. We revisited the diagnostic power of the basal serum testosterone level, the Triptorelin test and the human chorionic gonadotropin (hCG) test in a cohort of 33 boys with delayed puberty. Design: Boys were aged 14.2 to 26.2 years at referral. A 5-year-long clinical follow-up after the initial study allowed confirmation of the diagnosis. At the end of the follow-up period, IHH was found in 13 patients while the other 20 had normal spontaneous pubertal development (CDP). Results: At referral, a basal morning testosterone level . 1.7 nmol/l was observed in 55% of patients with CDP exclusively (predictive positive value (PPV) ¼ 100%; predictive negative value (PNV) ¼ 59%). For CDP, the PPV of the LH peak 3 h after Triptorelin was 100% by setting the upper threshold at 14 IU/l and the PNV was 72%. However, no lower threshold could discriminate IHH from CDP in the remaining patients with an LH peak 3 h after Triptorelin ,14 IU/l. In CDP patients, the PPV of the serum testosterone increment after hCG stimulation (DT/hCG) was 100% for values .9 nmol/l (PNV ¼ 72%). In IHH patients, the PPV of DT/hCG was 100% for values , 3 nmol/l (PNV ¼ 82%). Only 29% of the studied population had a DT/hCG between these lower and upper thresholds and therefore could not have been classified initially. Conclusions: (i) Dynamic testing for the diagnosis of delayed puberty is useful only when the basal testosterone level is lower than 1.7 nmol/l; (ii) in that case, the hCG test has better discriminating power than the Triptorelin test and appears as the best cost-effective investigation. It prevents useless and expensive investigations in about one-half of CDP patients with a basal morning testosterone level lower than 1.7 nmol/l.

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“…A rarely diagnosed Kallmann syndrome is characterized by anosmia caused by olfactory bulb hypoplasia [15].…”

Section: Olfactory Dysfunctionmentioning

confidence: 99%

Modern Principles of Allergic Rhinitis Therapy in Children

Вишнёва¹,

Намазова-баранова²,

Алексеева³

et al. 2014

Pediatr. farmakol.

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Modern principles of allergic rhinitis therapy in childrenAllergic diseases, the prevalence rate of which continues to grow, constitute a global issue for the world healthcare. Allergic rhinitis (AR) is a disease widespread among the children and adolescents, causing extensive socio-economic load as well as negative influence on the quality of life of both the patients and the members of their families [1−5]. Moreover, according to the results of the epidemiological studies, 10-40% of AR patients also suffer from bronchial asthma (BA). At the same time, almost 100% of BA patients, at least among the children, complain of AR manifestations. Constant sneezing, itching, rhinorrhea, nasal obstruction, cough and snoring, sleep disorders and fatigue negatively influence physical and psychological condition and social wellbeing, causing downslide in school performance [6]. Despite the presence of excruciating symptoms, the proven interconnection with and the influence on bronchial asthma, the issue of AR is often paid very little attention to, sometimes no more than a usual cold [5]. As a result, most of the pediatric patients either do not receive any adequate therapy they require, or are treated sporadically, taking palliative medications, falling out of modern treatment regimens. One of the reasons of this situation, which is not exclusively typical for our country, is the absence of guidelines on allergic rhinitis in children and its differences from the rhinitis in adults. The acute problem of choosing the correct treatment aiming at controlling the AR

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Kallman syndrome versus idiopathic hypogonadotropic hypogonadism at MR imaging.

Abstract: MR imaging demonstrates abnormalities of the rhinencephalon present in KS patients and occasionally absent in IHH patients.

Cited by 73 publications

References 0 publications

Brain Changes in Kallmann Syndrome

Brain Changes in Kallmann Syndrome

The human chorionic gonadotropin test is more powerful than the gonadotropin-releasing hormone agonist test to discriminate male isolated hypogonadotropic hypogonadism from constitutional delayed puberty

Modern Principles of Allergic Rhinitis Therapy in Children

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