Neonatal hypoglycemia represents an emergency of heterogeneous etiology. The occurrence of persistent hypoglycemia caused by hyperinsulinism has not been well established. Some authors claim that it may be more common than previously suggested. The diagnostic goal is to distinguish hyperinsulinemia from other causes of hypoglycemia because management strategies differ. The diagnosis of persistent hypoglycemia attributable to hyperinsulinism is made when insulin secretion is excessive or inappropriate (> 10 microIU/ml). Medical management includes frequent feeding, high hydrocarbon intake, glucagon, diazoxide, somatostatin or steroid treatment. In case of resistance to medical intervention, surgery consisting of subtotal pancreatectomy is performed to avoid neurological sequelae. However, pediatric organic hypoglycemia secondary to hyperinsulinism can be caused by either diffuse or focal pancreatic lesions. Differentiation between these two types of lesion is necessary since partial pancreatectomy can prevent diabetes. In this prospective study, pancreatic venous sampling (PVS) was evaluated for the preoperative localization of lesions in 25 children with hyperinsulinism and correlated with surgical, pathological and clinical outcome. PVS is the most accurate preoperative technique for localizing focal lesions in children. Besides being safe and effective, it has the great advantage of detecting focal secretion, thus reducing the need for extensive surgery.

SUMMARY Autism is thought to be associated with abnormal hemispheric specialization and left‐hemispheric dysfunction. Brain functional imaging using 133Xe‐SPECT (single photon emission computed tomography) was used to measure left/right asymmetry and absolute values of regional cerebral blood flow (rCBF) in 18 children with autism aged from four to 17 years and 10 age‐matched controls. All controls but only 10 children with autism were right‐handed. The left‐to‐right indices, both hemispheric and regional, were positive in controls, indicating higher left than right rCBF values, but were negative in patients with autism. This inversion was statically significant for total hemispheres, sensorimotor and language‐related cortex and was explained by a significant decrease of the left absolute rCBF values in these regions in the patients with autism. The inversion was independent of handedness, sex and age. These results confirm the existence of left‐hemispheric dysfunction in childhood autism, especially in the cortical areas devoted to language and handedness, leading to anomalous hemispheric specialization. RÉSUMÉ La tomographic par émission photonique dans l'autisme infantile: mise en évidence de l'absence d'asymétrie hémisphérique normale On considère souvent que l'autisme est associéà une specialisation hémispherique anormale et une dysfonction de L'hémisphère gauche. Une imagerie fonctionnelle cérébrale utilisant le SPECT avec le xénon133 (tomographic d'émission monophotoniquc) a été utilisée pour mesurer l'asymétrie gauche/droite et les valeurs absolues du debit sanguin cérébral régional (rCBF) chez 18 enfants autistes ages de quatre à 17 ans, et 10 contrôles appariés en âge. Tous les contrôles mais seulement 10 autistes étaient droiticrs. Les indices gauche/droit, à la fois hémisphériques et régionaux, étaient tous positifs chez les contrôles, indiquant des valeurs rCBF plus élévees à gauche qu'à droite, tandis qu'ils étaient négatifs chez les autistes. Cette inversion était statistiquement significative pour L'hemisphere entier, le cortex sensori‐moteur et les régions liées au langage et s'expliquait par une dimunution significative des valeurs absolues gauches du débit de ces régions chez les autistes. L'inversion était indépendante de la latéralité, du scxe et de L'âge. Ces résultats confirment L'existence d'une dysfonction de L'hémisphère gauche dans l'autisme infantile, spécialement dans les aires corticales spécialisés pour le langage et dc la commande manuelle, conduisant à une spécialisation hemisphérique anormale. ZUSAMMENFASSUNG Hint SPECT Darstellung bei kindlichem Aittismus: Verdacht auf das Fehlen eincr normalen Hemisphärenasymmetrie Es wird angenommen, daß Autismus mit abnormer Spezialisierung der Hemisphären und mit einter Dysfunktion der linken Hemisphäre verbunden ist. Es wurde eine Hirnfunktionsdarstellung mit 133‐Xenon SPECT (Einzelphotonenemissionskomputcrtomographie) durchgeführt, urn bei 18 Kindern mit Autismus im Alter von vier bis 17 Jahren und 10 altersentsprechenden Kontrollen die li...

Mitochondrial disorders have long been regarded as neuromuscular diseases only. In fact, owing to the ubiquitous nature of the oxidative phosphorylation, a broad spectrum of clinical features should be expected in mitochondrial disorders. Here, we present eight puzzling observations which give support to the view that a disorder of oxidative phosphorylation can give rise to any symptom in any organ or tissue with any apparent mode of inheritance. Consequently, we suggest giving consideration to the diagnosis of a mitochondrial disorder when dealing with an unexplained association of symptoms, with an early onset and a rapidly progressive course involving seemingly unrelated organs. Determination of lactate/pyruvate and ketone body molar ratios in plasma can help to select patients at risk for this condition.

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