Levodopa/carbidopa intestinal gel infusion and weight loss in Parkinson's disease.

BACKGROUND AND PURPOSE: Weight loss (WL) is a frequent yet under-recognized complication of levodopa/carbidopa intestinal gel (LCIG) infusion, as well as a milestone of Parkinson's disease (PD) disability progression. The complex association between WL, poor nutritional status, motor complications and PD progression, however, remains unclear. METHODS: Consecutive consenting patients with PD treated with LCIG (n = 44; PD duration, 18.3 ± 6.5 years) were enrolled in an open-label observational study assessing the extent of WL occurring during LCIG treatment. As secondary aims, we correlated the nutritional status, as detected by the Mini Nutritional Assessment, with the severity of motor symptoms [Movement Disorder Society Unified Parkinson's Disease Rating Scale part III], motor complications (Unified Parkinson's Disease Rating Scale part IV), activities of daily living (Schwab and England scale), cognitive impairment (Mini Mental State Examination), depression (Beck Depression Inventory), difficulties in feeding (Edinburgh Feeding Evaluation in Dementia Questionnaire) and levodopa equivalent daily dose (LEDD). RESULTS: There was an average WL of 9.9 ± 10.5% (7.6 ± 7.1 kg) over an LCIG treatment period of 51.6 ± 28.5 months. The extent of WL correlated with the percentage of the waking day spent with dyskinesia (P < 0.05). The nutritional status correlated with motor symptom severity (P < 0.01), dysphagia (P < 0.01) and LEDD (P < 0.01). CONCLUSIONS: Weight loss may occur in patients with PD undergoing LCIG in correlation with the percentage of the waking day spent with dyskinesia. Regardless of the extent of WL, the nutritional status correlated with higher LEDD, as well as with indices of disease progression, such as motor symptom severity and dysphagia.

A survey on Prader-Willi syndrome in the Italian population: prevalence of historical and clinical signs.

Clinical criteria for the diagnosis of Prader-Willi Syndrome (PWS) were established by consensus in 1993 (Holm et al.). Specific molecular testing is now available and the purpose of diagnostic criteria has shifted to identify individuals to test, thus avoiding the expense of unnecessary analysis. The aim of this study was to find clinical indicators to select patients with suspected PWS for laboratory testing. We analyzed the prevalence of clinical signs and symptoms in 147 genetically diagnosed Italian patients with PWS (67 males and 80 females), aged from 9 months to 34.6 years (13.6 +/- 8.3 years), using the consensus diagnostic criteria, and according to age, sex and type of genetic abnormality. The prevalence of several clinical features changed significantly with age, but very few with sex. According to genetic subtypes (deletion vs UPD), only hypopigmentation and acromicria were more frequent in patients with deletion. Some criteria considered as minor or supportive by Holm et al. have higher prevalence than some major criteria. In conclusion, in order to identify patients with suspected PWS to submit to laboratory testing, we recommend a classification of clinical criteria according to age, giving more attention to those so-called minor or supportive criteria.

Physiological role for cholecystokinin in reducing postprandial hyperglycemia in humans.

It is known that the ingestion of glucose alone causes a greater increase in plasma glucose levels than ingestion of the same amount of glucose given with other nutrients. Since physiological plasma concentrations of cholecystokinin (CCK) prolong gastric emptying, it is proposed that after a meal, CCK may modify plasma glucose levels by delaying glucose delivery to the duodenum. To evaluate the effect of CCK on oral glucose tolerance, plasma CCK, insulin, and glucose levels and gastric emptying rates were measured in eight normal males before and after the ingestion of 60 g glucose with the simultaneous infusion of either saline or one of two doses of CCK-8 (12 or 24 pmol/kg per h). Gastric emptying rates were measured by gamma camera scintigraphy of technetium 99m sulfur colloid and plasma CCK levels were measured by a sensitive and specific bioassay. Basal CCK levels averaged 1.0 +/- 0.1 pM (mean +/- SEM, n = 8) and increased to 7.1 +/- 1.1 pM after a mixed liquid meal. After glucose ingestion, but without CCK infusion, CCK levels did not change from basal, and the gastric emptying t1/2 was 68 +/- 3 min. Plasma glucose levels increased from basal levels of 91 +/- 3.9 mg/dl to peak levels of 162 +/- 11 mg/dl and insulin levels increased from 10.7 +/- 1.8 microU/ml to peak levels of 58 +/- 11 microU/ml. After glucose ingestion, with CCK infused at 24 pmol/kg per h, plasma CCK levels increased to 8 pM and the gastric emptying t1/2 increased to 148 +/- 16 min. In concert with this delay in gastric emptying, peak glucose levels rose to only 129 +/- 17 mg% and peak insulin levels rose to only 24.2 +/- 4.2 microU/ml. With CCK at 12 pmol/kg per h, similar but less dramatic changes were seen. To demonstrate that endogenous CCK could modify the plasma glucose and insulin responses to oral glucose, oral glucose was given with 50 g of lipid containing long-chain triglycerides. This lipid increased peak CCK levels to 3.7 +/- 0.9 pM. Concomitant with this rise in CCK was a delay in gastric emptying and a lowering of plasma glucose and insulin values. To confirm that CCK reduced hyperglycemia by its effect on gastric motility, 36 g glucose was perfused directly into the duodenum through a nasal-duodenal feeding tube in four subjects. With duodenal perfusion of glucose, there was no change in plasma CCK levels, but plasma glucose levels increased from basal levels of 93+/-5 to 148+/-6 mg/dl and insulin levels rose from 10.6+/-3.5 to 29.5+/-5.2 microU/ml. When CCK was infused at 24 pmol/kg per h, neither the plasma glucose nor insulin responses to the duodenal administration of glucose were modified. Thus we conclude that CCK, in physiological concentrations, delays gastric emptying, slows the delivery of glucose to the duodenum, and reduces postprandial hyperglycemia. These data indicate, therefore, that CCK has a significant role in regulating glucose homeostasis in human.

Effects of a novel cholecystokinin (CCK) receptor antagonist, MK-329, on gallbladder contraction and gastric emptying in humans. Implications for the physiology of CCK.

To explore the physiology of cholecystokinin (CCK) in humans, we investigated the effect on gallbladder contraction and gastric emptying of a recently developed CCK receptor antagonist, MK-329. In a double-blind, four-period crossover study eight subjects received single doses of 0.5, 2, or 10 mg MK-329, or placebo, followed by an intravenous infusion of CCK-8 (30 pmol/kg.h). In placebo-treated subjects gallbladder volumes decreased on average to 43% of initial volumes after 2 h of CCK infusion. MK-329 caused a dose-dependent inhibition of CCK-stimulated gallbladder contraction with 10 mg producing complete blockade (P less than 0.01, cf. placebo). Gallbladder contraction and gastric emptying rates after a mixed meal were then measured in a two-period crossover study. Subjects received placebo or 10 mg of MK-329 2 h before eating. Gastric emptying of both solids and liquids was measured simultaneously by gamma scintigraphy. In placebo-treated subjects plasma CCK levels increased postprandially to 2.3 pM, gallbladder volumes decreased 68.4 +/- 3.8% (SE), and the times for 50% emptying of liquids and solids from the stomach were 58 +/- 10 and 128 +/- 8 min, respectively. In MK-329-treated subjects there was a marked elevation in peak CCK levels to 13.8 pM (P less than 0.01, cf. placebo), and gallbladder contraction was completely inhibited. Solid and liquid emptying rates were unaffected. These findings demonstrate that (a) MK-329 is a potent, orally active antagonist of CCK in humans, and (b) CCK is the major regulator of postprandial gallbladder contraction. These data also support the concept of negative feedback regulation of CCK secretion and suggest that mechanisms other than CCK play a dominant role in the regulation of postprandial gastric emptying rates.

Prednisone treatment in newly diagnosed type I diabetic children: 1-yr follow-up.

Thirty-one children suffering from type I diabetes mellitus were arranged at onset of the disease in two different groups. Group 1 was treated with oral prednisone (60 mg X m-2 X day-1 for 14 days, 30 and 15 mg X m-2 X day-1 for 7 days). Group 2 matched the control group. All patients were treated with continuous subcutaneous insulin infusion for the first 15 days of treatment, and then with two daily injections of a mixture of intermediate- and fast-acting insulin. All subjects were followed for 1 yr. Group 1 required more insulin than group 2 after 30 days (1.5 +/- 0.3 vs. 0.6 $ 0.2 U X kg-1 X day-1, P less than .001) and after 60 days (0.8 +/- 0.1 vs. 0.5 +/- 0.06 U X kg-1 X day-1, P less than .001). After 3 mo, both groups reached the lowest mean stable HbA1 level (8.4 +/- 0.4 and 8.3 +/- 0.4% group 1 and 2 respectively). Between the 2nd and 9th mo of follow-up, mean postbreakfast C-peptide concentration increased in both groups. The highest levels of fasting C-peptide were reached by group 1 after 90 days (0.77 +/- 0.32 nM) and group 2 after 60 days (0.34 +/- 0.09 nM). The largest partial remission (C-peptide 0.3 nM, insulin requirement less than 0.5 U X kg-1 X day-1 and no glycosuria) was observed in group 1 after 180 days (5 of 16 patients) and in group 2 after 60 days (5 of 15 patients).(ABSTRACT TRUNCATED AT 250 WORDS)

Reduced postprandial cholecystokinin (CCK) secretion in patients with noninsulin-dependent diabetes mellitus: evidence for a role for CCK in regulating postprandial hyperglycemia.

The plasma cholecystokinin (CCK) response to a test meal was studied in 16 control subjects and 15 patients with noninsulin-dependent diabetes mellitus (NIDDM). Basal CCK levels were approximately 1 pmol in both groups. However, after the test meal, plasma CCK levels were 2-fold greater in the controls when compared to the diabetics. In controls, CCK levels maximally increased by 5.6 +/- 0.8 pmol (mean +/- SEM) 10 min after feeding, whereas in the NIDDM patients this value was 1.9 +/- 0.6 pmol (P < 0.001). After the test meal, the normal subjects showed no postprandial rise in blood glucose, whereas the diabetic patient showed a rise of 2.6 +/- 0.7 mmol. To determine whether the decreased CCK levels may have been related to the postprandial hyperglycemia, 7 diabetic subjects were infused with CCK. With this CCK infusion, postprandial glucose levels did not rise. These data suggest, therefore: 1) a role for cholecystokinin in regulating postprandial hyperglycemia in man, 2) abnormalities in CCK secretion occur in NIDDM and may contribute to the hyperglycemia seen in this disease.

Regulation of pancreatic endocrine function by cholecystokinin: studies with MK-329, a nonpeptide cholecystokinin receptor antagonist.

A cholecystokinin (CCK) receptor antagonist, MK-329, was used to explore the physiological role of CCK in regulating pancreatic endocrine function in humans. The ability of CCK to increase plasma pancreatic polypeptide (PP) concentrations and blockade of this effect with MK-329 were evaluated in a double blind, balanced, four-period cross-over study. Eight subjects received single oral doses of 0.5, 2, or 10 mg MK-329 or placebo, followed by an iv infusion of CCK-8 (34 ng/kg.h). In placebo-treated subjects, PP increased from basal levels of 70 +/- 15 (+/- SE) to peak values of 291 +/- 58 pg/mL after CCK infusion (P less than 0.05 compared to basal). This increase in plasma PP concentration was inhibited in a dose-dependent fashion by MK-329, with 10 mg antagonizing the stimulatory effect of CCK infusion by nearly 80%. Second, the effect of MK-329 on meal-stimulated pancreatic endocrine responses was evaluated by giving placebo or 10 mg MK-329 2 h before ingestion of a mixed meal. Eight subjects were treated in a randomized two-period cross-over fashion. With placebo treatment, peak postprandial plasma insulin, glucagon, and glucose concentrations were 101 +/- 8 microU/mL, 195 +/- 15 pg/mL, and 150 +/- 10 mg/dL, respectively (all P less than 0.05). The integrated PP response following the meal was 56.3 +/- 11.1 ng/mL.minute. With MK-329 treatment, the integrated PP concentration was reduced to 33.9 +/- 2.2 ng/mL.min (P less than 0.05 compared to placebo treatment). Mean postprandial insulin, glucagon, and glucose concentrations did not differ between placebo and MK-329 treatments. We conclude that CCK receptor blockade with 10 mg MK-329 does not alter plasma insulin, glucagon, or glucose responses to a mixed meal. However, the observation that physiological concentrations of CCK increase plasma levels of PP, and the finding that CCK receptor blockade selectively attenuates the postprandial increase in plasma PP concentrations support a physiological role for CCK in regulating PP secretion.

Peculiar body composition in patients with Prader-Labhart-Willi syndrome.

Prader-Labhart-Willi syndrome (PWS)-characterized by severe obesity, short stature, hypogonadism, and muscle hypotonia-appears to be an interesting model for body-composition abnormalities. Twenty-seven PWS patients (15 males and 12 females) aged 6-22 y underwent total-body analysis by dual-energy X-ray photon absorptiometry (DXA). For each PWS patient two age- and sex-matched control subjects were studied: one obese subject with a relative body weight (RBW > 120%) and body mass index (BMI) similar to that of the patient and one normal-weight subject (RBW < 120%). Percentage body fat was significantly greater in PWS patients than in obese subjects (47.4 +/- 7.2% compared with 41.9 +/- 9.9%, P < 0.0001) and the same difference was evident for arms and legs but not for the trunk. Lean mass was significantly lower in PWS patients (26.4 +/- 8.2 kg) than in normal-weight subjects (32.9 +/- 10.2 kg) and even more so than in obese subjects (40.3 +/- 13.2 kg) (P < 0.0001). The most affected regions were limbs; thus, the ratio of lean mass in the trunk to that in the limbs was significantly higher in PWS patients (1.19 +/- 0.15) than in obese (1.07 +/- 0.13) and normal-weight (1.07 +/- 0.09) subjects (P < 0.002). The ratio of fat mass to lean mass was significantly higher in PWS patients than in obese subjects (0.90 +/- 0.32 and 0.74 +/- 0.27, P < 0.05). Bone mineral content (BMC) was significantly lower in PWS patients (1503 +/- 46 g) than in normal-weight (1876 +/- 677 g) and obese (2322 +/- 773 g) subjects (P < 0.0001); this difference was most pronounced in the limb region. Bone mineral density (BMD) in PWS patients (0.993 +/- 0.116 g/cm2) did not differ significantly from that of normal-weight subjects (1.033 +/- 0.147 g/cm2) but was significantly lower than that of obese subjects (1.154 +/- 0.139 g/cm2). The influence of age on body composition was assessed by comparing two age subgroups (< 12 y, n = 10; and > or = 12 y, n = 17). The older PWS patients had higher adiposity, lower BMC, and dramatically lower BMD. Also, the lean mass deficit increased with age so that the ratio of fat mass to lean mass was close to 1. In conclusion, PWS patients showed a peculiar body composition, to some extent similar to that found in subjects deficient in growth hormone or even to sedentary and elderly people. These results suggest the importance of an accurate analysis of body composition in PWS patients.

Influence of body composition on bone mineral content in children and adolescents.

Excess fat and fat-free mass have been extensively described in obese children, whereas few data about bone mineral content (BMC) variations are available in children. Dual-energy X-ray absorptiometry (DXA) allows a direct and accurate measurement of three body compartments (fat, lean, and BMC), subdivided into three regions (arms, trunk, and legs). The aim of our study was to evaluate the influence of body compartments on total BMC (TBMC) and regional BMC (RBMC) in obese and normal-weight subjects. Sixty-five obese and 50 normal-weight children and adolescents (age range: 5-18 y relative body weight: 160 +/- 23% and 101 +/- 12%, respectively), matched for sex and pubertal stage underwent a DXA total-body analysis. Obese subjects had significantly greater fat and lean compartments than normal-weight subjects (P < 0.0001). TBMC was larger in obese children (1930 +/- 670 g compared with 1480 +/- 490 g, P < 0.0001) as was RBMC (arms: 182 +/- 81 g compared with 151 +/- 65 g; trunk: 560 +/- 223 g compared with 433 +/- 169 g; legs: 788 +/- 341 g compared with 539 +/- 231 g, P < 0.0001). We found lean mass to be the best correlate with TBMC (r = 0.91 in obese and 0.94 in normal-weight children). Multiple-regression analysis confirmed lean mass as one of the major determinants of TBMC and RBMC in children. However, differences in TBMC and RBMC were no longer present after correction for age, sex, and body-composition variables. There were no differences in TBMC and RBMC between obese and normal-weight children after correction for the confounding variables age and sex.

Relative expansion of extracellular water in obese vs. normal children.

This preliminary communication reports data regarding the distribution between intracellular (ICW) and extracellular (ECW) water compartments in a group of 21 prepubertal young obese children of both sexes in comparison with a group of 18 normal children weight matched for age. Our data indicate that obesity is associated with a highly significant relative expansion of extracellular water (ECW/ICW = 0.61 +/- 0.19 and 0.76 +/- 0.09 in control and obese subjects, respectively; P < 0.0015). This observation, which has been already reported in adult women, suggests that some disturbances of water homeostasis have an early onset and stress the need for an early control of energy imbalance in children. These findings are of great concern also in the field of human body composition, suggesting the opportunity for a critical reevaluation of the assumed constancy of some human body characteristics. Body composition methodologies developed for "normal" populations would require adjustment for use in the obese population, since a considerable error would be introduced.

Body composition during GH treatment in Prader-Labhardt-Willi syndrome.

Prader-Labhardt-Willi syndrome (PLWS) is a model to study GH secretion, body composition and consequences of GH therapy. Twenty-seven patients were studied by dual-energy X-ray absorptiometry (DXA) and were each compared to two age- and sex-matched controls (obese and normal weight). Fat mass (FM) was significantly greater in PLWS than in patients with simple obesity; lean body mass (LM) and bone mineral content (BMC) were significantly lower compared to both controls. The peculiar body composition of PLWS patients seems to be similar to that found in GH deficiency. In six PLWS children treated with GH, LM increased after 6 months (p<0.02) up to 12 months (p<0.03); FM decreased in 5/6 patients. Obese adult PLWS patients treated with GH for 6 months showed a reduction in adiposity; LM increased significantly only in the leg compartment. Abdominal CT scan did not show a significant reduction of intrabdominal fat area. In conclusion, GH therapy might improve final stature and exert a positive influence on body composition in patients with PLWS.

Multiple insulinomas of the pancreas: a patient report.

Whereas there is general agreement about the biochemical diagnosis of hyperinsulinism, the value of imaging to differentiate diffuse from focal pancreatic lesions is still a matter of debate. We describe a case of multiple adenomas of the pancreas in an eleven year-old boy. The source of hyperinsulinism was detected by pancreatic ultrasound examination and confirmed by MRI as a single adenoma of the pancreas. These radiological exams did not identify three other pancreatic adenomata. Our report outlines the difficulties in anatomically localizing the source of excessive insulin secretion in cases of hyperinsulinemic hypoglycemia.

GH secretion in Prader-Labhard-Willi syndrome: somatotrope responsiveness to GHRH is enhanced by arginine but not by pyridostigmine.

Low somatotrope responsiveness to secretagogues has been reported in patients affected by Prader-Labhard-Willi Syndrome (PLWS). In normal subjects, GH response to GHRH is known to be greatly potentiated to the same extent by pyridostigmine (PD) or arginine (ARG) which probably act via inhibition of hypothalamic somatostatin release. To clarify somatotrope responsiveness in 7 PLWS patients, we studied GH response to GHRH alone and to GHRH combined with PD or ARG. Eight normal short children were studied as controls (NC). GH response to GHRH in PLWS was lower than in NC (AUC: 615 +/- 205 micrograms/l.h, vs 1271 +/- 333 micrograms/l.h, p < 0.02). In NC, the GHRH-induced GH rise was potentiated to the same extent by PD or ARG. In contrast, in PLWS PD failed to increase the GH response to GHRH (AUC: 615 +/- 205 micrograms/l.h vs 621 +/- 176 micrograms/l.h, n.s.) which was enhanced by ARG (AUC: 615 +/- 205 micrograms/l.h vs 1633 +/- 425 micrograms/l.h, p < 0.02). However, the GH response to GHRH + ARG in PLWS was lower than in NC. In conclusion, our results demonstrate that in PLWS the low somatotrope responsiveness to GHRH is not enhanced by cholinergic potentiation while it is increased by arginine.

[Prader-Willi syndrome]. / La sindrome di Prader-Willi.

Prader-Willi syndrome (PWS) is the most frequent cause of secondary obesity, characterized by neonatal hypotonia, dysmorphic facies, acromicria, hypogonadism, stunted growth, obesity, behavioural disturbances and cognitive impairment. Clinical diagnosis is confirmed by alteration of imprinted genes on the proximal long arm of chromosome 15 (15q11-13) for deletion, translocation, uniparental disomy for maternal chromosome 15 or imprinting center defect. Methylation test is the most reliable test for diagnosis. This issue explains diagnostic tests, clinical, metabolic, endocrinological features, and the most frequent complications observed in this syndrome. Precocious diagnosis and multidisciplinary approach allow in these patients to prevent the severe obesity and linked complications.

[Expectations of nursing managers and assistants as to the managerial style of an executive director of a teaching hospital]. / Expectativas de gerentes e assessores de enfermagem quanto ao estilo gerencial do diretor executivo de um hospital de ensino.

The present study focussed the expectations of 13 nurses from a University hospital regarding to the executive director's managerial style. The Managerial Grid of BLAKE & MOUTON (1987) was used as a theoretical reference and a questionnaire was applied based on the Grid & Leadership in Nursing Instrument elaborated by TREVIZAN (1993). Results evidenced that the most expected style corresponds, considering the Managerial Grid, to "team management", or 9.9. The second style was the "management of men organization", or 5.5. Authors concluded that there are important expectations for these nurses related to a management that enhances values such as trust, respect, commitment, personal investment and team work in order to achieve the organization goals.

The clinical and molecular characterization of patients with dyshormonogenic congenital hypothyroidism reveals specific diagnostic clues for DUOX2 defects.

CONTEXT: Mutations in the DUOX2 gene have been associated with transient or permanent congenital hypothyroidism due to a dyshormonogenic defect. OBJECTIVE: This study aimed to verify the prevalence of DUOX2 mutations and the associated clinical features in children selected by criteria supporting a partial iodide organification defect (PIOD). PATIENTS AND METHODS: Thirty children with PIOD-like criteria were enrolled and genotyped. A detailed clinical characterization was undertaken together with the functional analysis of the DUOX2 variations and the revision of the clinical and molecular data of the literature. RESULTS: In this large selected series, the prevalence of the DUOX2 mutations was high (37%). We identified 12 missense variants, one splice site, and three frameshift DUOX2 mutations. Functional analyses showed significant impairment of H2O2 generation with five missense variants. Stop-codon mutants were shown to totally abolish DUOX2 activity by nonsense-mediated RNA decay, exon skipping, or protein truncation. DUOX2 mutations, either mono- or biallelic, were most frequently associated with permanent congenital hypothyroidism. Moreover, the present data suggested that, together with goiter and PIOD, the most significant features to select patients for the DUOX2 analysis are the low free T4 and the high TSH concentrations at the first postnatal serum sampling, despite borderline blood spot TSH. Interestingly, the analysis of previously described DUOX2 mutated cases confirmed the validity of these findings. CONCLUSIONS: The defects in the peroxide generation system are common among congenital hypothyroidism patients with PIOD. The most robust clinical parameters for selecting patients for DUOX2 analysis have been identified, and several DUOX2 variants have been functionally characterized.

[Diagnosis and differential diagnosis of obesity in childhood]. / Diagnosi e diagnosi differenziale dell'obesità in età pediatrica.

About 2-3% of "essential" obesity in pediatric age is of endocrine or genetic origin (secondary obesity). The clinical picture of these forms is almost always characteristic; however, some patients affected by secondary obesity can present with an incomplete or atypical aspect. The aim of this review is to offer the pediatrician useful indications to correctly diagnose children presenting with obesity. It is advisable to make a careful anamnesis and an accurate medical examination in order to ascertain the causes that may have contributed to the onset and increase of weight gain. Obesity associated with mental retardation, short stature, cryptorchidism or hypogonadism, dysmorphism with facies sui generis, ocular or uditive defects, might suggest a genetic origin. Prader-Willi syndrome is the most frequent of these disorders and it is due to an alteration of chromosome 15 of paternal origin. These patients have to undergo the methilation test (easy and low cost genetic research) in order to confirm the clinical suspicion. Endocrine alterations, that play a pathogenic role in pediatric obesity (i.e., hypothyroidism, hypothalamic-pituitary diseases, pseudohypoparathyroidism), are rare. Early treatment of hormonal dysfunction generally allows to ameliorate or normalize the weight gain. In absence of specific clinical manifestations or lacking a significant clinical history, no endocrine test is required. The family pediatrician should require some routine hematochimic tests, in order to evaluate the possible presence of hyperlipidemia and/or glycometabolic complications. An oral glucose tolerance test is necessary only for patients presenting with serious weight gain, acanthosis nigricans, and for those with a family history of diabetes. In the most serious cases, a careful cardiovascular and respiratory evaluation should be performed. Children with a suspicion of secondary obesity have to be submitted to an endocrinologist, for a correct diagnosis and a specific treatment. However, the family pediatrician's assistance is essential during the follow-up period, in order to assure the patient and his/her family a proper assistance.

A case of familial nesidioblastosis: prenatal diagnosis of foetal hyperinsulinism.

Persistent neonatal hyperinsulinaemic hypoglycaemia due to nesidioblastosis is a rare condition probably transmitted by an autosomal recessive inheritance. Recurrent hypoglycaemic episodes become evident after birth and cause severe neurological damage without intensive treatment. The intrauterine detection of hypoglycaemia and hyperinsulinism in newborns subsequently diagnosed as affected by nesidioblastosis has not yet been reported. We describe a case of familial nesidioblastosis in which an intrauterine diagnosis could be suggested by high levels of insulin and C-peptide and low values of glucose in the amniotic fluid.