[Acute adrenal insufficiency in the newborn]. / Insuffisance surrénale aiguë chez le nouveau-né

Neonatal acute adrenal insufficiency is a rare condition. Congenital adrenal hyperplasia with 21-hydroxylase defect appears to be the most frequent cause, but the neonatal screening has improved its potential severe outcome. The other causes and the various clinical presentations have been exposed, with a special reference to the salt-wasting syndrome. Among them, the severity of X-linked adrenal hypoplasia congenita (AHC) deserves special attention. Two other causes of adrenal hypoplasia have been recently discovered, i.e. a mutation of the SF-1 gene and the syndrome IMAGe. Adrenal insufficiency secondary to ACTH deficiency is often unrecognised despite the risk of severe seizures and hypoglycaemia with brain damage. Finally, the hormonal diagnostic testing and the main therapeutic approach by corticosteroids have been indicated. The aim of this work is to focus the attention of paediatricians who examine a newborn because the risk of delayed diagnosis and fatal outcome may be limited if the clinical symptoms are soon recognized.

[Idiopathic hypothalamic syndrome: retrospective study and literature review]. / Aspects cliniques des syndromes hypothalamiques idiopathiques: étude rétrospective et revue de la littérature.

UNLABELLED: Hypothalamic obesity is usually induced by tumoral or genetic alterations such as craniopharyngioma or Prader-Willi syndrome, respectively. However, few cases have been reported without recognized etiology, this syndrome is also called idiopathic hypothalamic syndrome. OBJECTIVES: To improve definition and frequency of complications associated with this syndrome. POPULATION AND METHODS: A retrospective cohort study was performed in French endocrine paediatric departments and was associated with a literature review. RESULTS: We report five cases of idiopathic hypothalamic syndrome. This syndrome is correlated with a high mortality (one of our five cases, 25% in the literature) by neurovegetative dysfunction (breathing or thermal alteration). Obesity began before six years old because of compulsive eating and resulted in social behaviour disorders. Abnormal endocrine secretions were characterized by early hyperprolactinemia, permanent but later somatotrope deficiency and 80% of thyreotrope deficiency. Puberty abnormalities included hypogonadotropic hypogonadism as well as precocious (one of our cases, three cases including literature) or normal puberty. Neurogenic hypernatremia and water and electrolytic disorders were also responsible of acute neurological alterations. CONCLUSION: This largest study ever reported of idiopathic hypothalamic syndrome emphasizes the need of a multidisciplinary coordination to provide the best care of these patients.

Description of microangiopathy in children with type 1 diabetes mellitus: a 19-year retrospective study.

BACKGROUND: The aim of the study was to estimate the outcome of patients with type 1 diabetes followed in a university hospital in the paediatric department and then in the adult diabetic department for at least 10 years. METHODS: We made a retrospective analysis of 50 patients (28 women and 22 men) with type 1 diabetes with disease duration of 19 +/- 6 years and analysed whether retinopathy and nephropathy had progressed, had remained unchanged or had improved or normalised. RESULTS: The mean age of diabetes onset was 8 +/- 4 years (1-16). Ketoacidosis revealed diabetes in 36% of the children. Mean HbA(1c) was 8.6 +/- 1.8%, and was over 8.5% in 34% of the patients. The mean age at onset of puberty (Tanner stage II) was 12 +/- 1 years in girls and 13 +/- 1 years in boys. Mean HbA(1c) was 7.9 +/- 1.2% during the year before onset of puberty and 8.7 +/- 1.1% in the following 3 years, corresponding to a 10% pubertal increase in HbA(1c). Retinopathy was seen in 50% of the patients at a mean age of 22 +/- 5 years, 15 +/- 6 years after onset of diabetes. Mean HbA(1c) was 9.7 +/- 1.6% in patients with proliferative retinopathy, 9.0 +/- 1.5% in patients with non proliferative retinopathy, and 8.1 +/- 1.3% in those without (p=0.02, proliferative versus no retinopathy, p > 0.05 non proliferative versus no retinopathy). Microalbuminuria was diagnosed in 26% of the patients. Mean HbA(1c) was 9.3 +/- 2.1% in patients with microalbuminuria versus 8.1 +/- 1.3% in those with normoalbuminuria (p=0.02). CONCLUSIONS: Glycemic control was similar in patients with non proliferative retinopathy and those without. Proliferative retinopathy and nephropathy were both related to the level of glycemic control.

Body composition, fasting leptin, and sex steroid administration determine GH sensitivity in peripubertal short children.

Serum IGF-I levels in GH-treated subjects demonstrate a wide range of responsiveness to GH. However, the factors influencing GH sensitivity are not well known. The aim of this work was 1) to test whether body composition (determined by dual energy x-ray absorptiometry) or factors related to body composition (fasting blood glucose, FFA, C-peptide, leptin, and insulin sensitivity determined by an insulin tolerance test) influence GH sensitivity; and 2) to study the effect of sex steroid priming on GH sensitivity. We measured serum IGF-I at baseline and 24 h after a single administration of GH (2 mg/m(2)) in 60 healthy prepubertal and early pubertal children (height, -2.1 +/- 1.0 SD score). GH sensitivity, as estimated by the increase in serum IGF-I after GH administration (difference between stimulated and baseline serum IGF-I = delta IGF-I), was also determined after a short-term administration of oral ethinyl E2 in girls and im T in boys. The serum IGF-I concentration was 297 +/- 114 microg/liter at baseline and increased to 429 +/- 160 microg/liter, corresponding to a 46 +/- 29% increase over the baseline value (P < 0.0001, stimulated vs. baseline serum IGF-I). delta IGF-I was not different between gender or pubertal stage. There were positive correlations (P < 0.001) between delta IGF-I and adiposity (total body fat, r = 0.62; trunk fat, r = 0.62), fasting leptin (r = 0.64), and C-peptide (r = 0.54), and a negative correlation with fasting FFA (r = -0.33; P < 0.05) even after adjustment for age, gender, and pubertal stage. These factors remained significant independent predictors of the absolute as well as the percent increase in serum IGF-I in multiple regression analyses. Priming with T and ethinyl E2 had a similar stimulating effect on the serum GH peak in response to the insulin tolerance test. In boys, serum baseline IGF-I increased by 60%, and delta IGF-I was similar after vs. before T administration. By contrast, in girls, serum baseline IGF-I was similar, and delta IGF-I was 60% less after vs. before ethinyl E2 administration. This study indicates that 1) GH sensitivity is determined by fat mass, serum fasting leptin, C-peptide, and FFA; and 2) oral ethinyl E2 and im T have divergent effects on the IGF-I response to a single administration of GH.

Growth and adult height in GH-treated children with nonacquired GH deficiency and idiopathic short stature: the influence of pituitary magnetic resonance imaging findings.

We analyzed the final height of 146 short children with either nonacquired GH deficiency or idiopathic short stature. Our purpose was 1) to assess growth according to the pituitary magnetic resonance imaging findings in the 63 GH-treated children with GH deficiency and 2) to compare the growth of the GH-deficient patients with normal magnetic resonance imaging (n = 48) to that of 32 treated and 51 untreated children with idiopathic short stature (GH peak to provocative tests >10 microg/liter). The mean GH dose was 0.44 IU/kg.wk (0.15 mg/kg.wk), given for a mean duration of 4.6 yr. Among the GH-deficient children, 15 had hypothalamic-pituitary abnormalities (stalk agenesis), all with total GH deficiency (GH peak <5 microg/liter). They were significantly shorter and younger at the time of diagnosis than those with normal magnetic resonance imaging, had better catch-up growth (+2.7 +/- 0.9 vs. +1.3 +/- 0.8 SD score; P < 0.01), and reached greater final height (-1.1 +/- 1.0 vs. -1.7 +/- 1.0 SD score; P < 0.05). Among patients with normal magnetic resonance imaging, there was no difference in catch-up growth and final height between partial and total GH deficiencies. GH-deficient subjects with normal magnetic resonance imaging and treated and untreated patients with idiopathic short stature had comparable auxological characteristics, age at evaluation, and target height. Although they had different catch-up growth (+1.3 +/- 0.8, +0.9 +/- 0.6, and +0.7 +/- 0.9 SD score, respectively; P < 0.01, by ANOVA), these patients reached a similar final height (-1.7 +/- 1.0, -2.1 +/- 0.8, and -2.1 +/- 1.0 SD score, respectively; P = 0.13). Pituitary magnetic resonance imaging findings show the heterogeneity within the group of nonacquired GH deficiency and help to predict the response to GH treatment in these patients. The similarities in growth between the GH-deficient children with normal magnetic resonance imaging and those with idiopathic short stature suggest that the short stature in the former subjects is at least partly due to factors other than GH deficiency.

Relationships between placental GH concentration and maternal smoking, newborn gender, and maternal leptin: possible implications for birth weight.

The control of fetal growth depends on multiple hormones, including both IGF-I and placental GH (PGH) in the mother, and IGF-I rather than pituitary GH (pitGH) in the fetus. Leptin, which is produced by adipocytes and syncitiotrophoblast cells, has also been thought to influence fetal growth by an as yet unknown mechanism. This study assessed the relationships between the GH-IGF-I axis in mothers and newborns, and maternal smoking, neonate gender, and maternal and fetal leptin. We collected blood in 87 mothers at the onset of labor and cord blood immediately after birth in their 87 healthy full-term newborns. GH concentrations were log(10) transformed, and data were expressed as the geometric mean (-1, +1 tolerance factor). PGH was lower in the 30 smoking mothers, as compared with the 57 nonsmoking mothers [18.2 (11.5; 28.6) vs. 27.0 (15.1; 48.2) microg/liter, P < 0.01]. Cord blood IGF-I was lower in neonates from smoking mothers (90 +/- 44 vs. 135 +/- 65 microg/liter, mean +/- SD, P < 0.01), consistent with their lower birth weight percentile (P < 0.01). A gender effect was observed for PGH, which was higher when the newborn was female, and for newborn pitGH and newborn leptin, which were, respectively, lower and higher in females, even after adjustment for birth weight and maternal smoking category (P < 0.05 for all comparisons). Multiple regression analyses identified maternal leptin as a negative predictor of PGH (P < 0.05) and newborn leptin as a positive predictor of newborn IGF-I (P < 0.05). Maternal smoking is associated to decreased maternal PGH and cord blood IGF-I concentrations. A sexual dimorphism for PGH, newborn pitGH, and newborn leptin exists at the time of birth, but its physiological significance remains to be studied. The relationships between maternal leptin and PGH and between cord blood leptin and IGF-I are consistent with the hypothesis that leptin could contribute to the control of fetal growth.

Macroorchidism due to autonomous hyperfunction of Sertoli cells and G(s)alpha gene mutation: an unusual expression of McCune-Albright syndrome in a prepubertal boy.

We report an unusual observation of a 3.8-yr-old boy with McCune-Albright syndrome (MAS) associated with abnormal prepubertal testis enlargement and no sexual precocity. Physical examination showed café-au-lait skin lesions, enlarged testes, prepubertal sized penis, and no pubic or axillary hair. Skeletal radiography disclosed fibrous dysplasia. The serum testosterone level was 0.58 nmol/L and remained below 1.4 nmol/L during the 4-yr follow-up. By contrast, serum inhibin B and anti-Mullerian hormone concentrations were abnormally increased up to 255 pg/mL (childhood range, 35--180) and 792 pmol/L (childhood range, 309--566), respectively. The LH response to a GnRH test was in the prepubertal range, whereas the FSH response was blunted. This abnormal hormone concentration profile indicates autonomous hyperfunction of Sertoli cells, with no evidence of Leydig cell activation. Testicular histology showed tubules with marked Sertoli cell hyperplasia and very rare germinal cells, and interstitial tissue containing mesenchymal cells but no mature Leydig cells. DNA sequence analysis from bone and testis tissues detected the known activating mutation in MAS that results in replacement of Arg by His at codon 201 of the G(s)alpha protein. Other endocrine tests showed excessive GH secretion and moderate adrenal androgen hypersecretion. These findings are consistent with the occurrence of an activating mutation of the G(s)alpha gene mainly expressed in Sertoli cells and weakly expressed or absent in Leydig cells. Abnormal prepubertal testicular enlargement extends the clinical spectrum of MAS, suggesting that determination of serum inhibin B and anti-Mullerian hormone should be considered in boys with this syndrome. This observation demonstrates the usefulness of detailed molecular and biological investigations in atypical cases of MAS.

[Precocious puberty in children adopted from foreign countries]. / Puberté précoce chez des enfants adoptés de pays étrangers.

AIMS: Precocious puberty has been more frequently observed in the population of children adopted from abroad. A study was therefore carried out to assess the prevalence of this early onset of puberty. POPULATION AND METHODS: In this study, 13 cases of precocious puberty have been examined in ten adopted girls and three adopted boys, and the clinical characteristics and other contributing factors have been described. In this study group, three of the cases were familial. In addition, a questionnaire was also completed by 99 French families with children adopted from abroad, and analyzed to determine the frequency of early pubertal development. The parameters included were age, weight and height at the time of adoption, date of onset of puberty, for the girls age at first menstruation, and current height and weight. RESULTS: It was determined that the 13 children had a very high growth recovery rate from the time that they arrived in France. For the period from time of adoption to the onset of puberty, mean height increased from -1.3 to +1.5 standard deviation score (SDS) and the mean weight-for-height factor increased from +1.2 to 1.9 SDS. The weight-height recovery rate following adoption seems to be the direct cause of early pubertal development in certain children, notably in those with a particularly rapid growth rate (between 6 years 6 months and 8 years 9 months for the girls, and between 8 and 10 years for the boys). In children adopted at an early age, a 'biological memory' seems to exist regarding the renutrition phenomenon which was instrumental in accelerating the onset of puberty some years after adoption. An analysis of the survey on the adoptive families showed that the frequency of precocious puberty was 44.9% in the group of 49 girls compared to only 8.6% in the group of 35 adopted boys, and that it mainly concerned children from Africa (57%), followed by those from South and Central America (57%), Asia (45%), and Eastern Europe (29%). CONCLUSION: A higher rate of precocious puberty was found in the adopted girls, with a significantly lower rate in the adopted boys. The etiological factors involved seemed to be mainly nutritional, and influenced by leptin and insulin-like growth factor 1 (IGF1) levels. The role of the latter and their interaction with other factors, particularly the ethnic aspect, remains to be determined via the study of a larger series of adopted children.

Relationship between glomerular hyperfiltration and ACE insertion/deletion polymorphism in type 1 diabetic children and adolescents.

OBJECTIVE: Glomerular hyperfiltration may predict diabetic nephropathy in type 1 diabetes, and some studies suggest that the ACE D allele is associated with diabetic nephropathy. The aim of this study was to examine a possible relationship between glomerular hyperfiltration and ACE insertion/deletion (I/D) polymorphism in type 1 diabetic children and adolescents. RESEARCH DESIGN AND METHODS: A cross-sectional study was conducted to examine the relationship between glomerular hyperfiltration and ACE (I/D) polymorphism in 76 type 1 diabetic children and adolescents without diabetic nephropathy (mean +/- SD: age 16 +/- 3 years; diabetes duration 7 +/- 4 years; age at diabetes onset 9 +/- 4 years; HbA1c 9.5 +/- 1.9%). Glomerular hyperfiltration (defined as a glomerular filtration rate [GFR] > or = 135 ml.min-1. 1.73 m-2 and by 51Cr-labeled EDTA plasma disappearance technique) and ACE I/D genotypes and plasma levels (enzyme-linked immunosorbent assay [ELISA] method) were determined. RESULTS: Of the patients, 29 (38%) displayed glomerular hyperfiltration. An association between glomerular hyperfiltration and ACE (I/D) polymorphism was observed (chi 2 = 7.09, P = 0.029) because of a reduced proportion of DD genotypes among patients with glomerular hyperfiltration (4 vs. 19; chi 2 = 6.03, P = 0.014) and not because of an excess of the II genotype (5 vs. 9; chi 2 = 0.04, P = 0.83). Age, diabetes duration, age at diabetes onset, and HbA1c were not different according to genotype. Patients with glomerular hyperfiltration had low plasma ACE levels, compared with those with normal glomerular filtration (457 +/- 157 vs. 553 +/- 186 micrograms/l; P = 0.027). CONCLUSIONS: These results suggest an unexpected association between glomerular hyperfiltration and ACE (I/D) polymorphism, characterized by a defect of the DD genotype among type 1 diabetic children and adolescents with glomerular hyperfiltration.

Three-year data from a comparative study with recombinant human growth hormone in the treatment of short stature in young children with intrauterine growth retardation.

Growth acceleration and bone maturation were studied for 3 y in 69 children with severe short stature and a history of intrauterine growth retardation (IUGR), to determine the effect of treatment with recombinant human growth hormone (r-hGH). The patients were enrolled in an open, multicentre trial and were randomly allocated to either the treated group (Group 1) or the control group (Group 2). The children in Group 1 were treated daily with 0.2 IU/kg/body weight (0.067 mg/kg) s.c., during 3 y and the children in Group 2 started the study with a 1-y observation period followed by a 3-y treatment period. At birth, their mean weight standard deviation score (SDS) was -2.5 and their mean length SDS -3.5. At baseline, the patients were prepubertal, non-GH deficient, with no known dysmorphic features. Mean age was 4.5 y, bone age was 3.3 y, height SDS was -3.4, height velocity (HV) SDS was -1.6, and body mass index SDS was -1.4. After 1 y of treatment, linear HV in Group 1 increased in comparison with the pre-treatment period (from 5.7 +/- 2.0 to 10.1 +/- 1.7 cm/y; p < 0.001) and with the first year of observation in Group 2 (p < 0.001). Increased HV was sustained during the second and third year of treatment and was significantly higher than at baseline. A similar growth pattern was seen during the 3 y of GH treatment in Group 2. Mean height SDS for chronological age increased by 2.0 +/- 0.7 in the two groups after 3 y of treatment. HV after 1 y of treatment was negatively correlated with growth velocity at baseline. Bone age remained retarded but increased with a mean of almost 4 y after 3 y of treatment in both groups. Even at a dose that is three times the replacement dose treatment with r-hGH was well tolerated. From these results, we conclude that r-hGH treatment over 3 y can induce sustained catch-up growth in young children with severe short stature and a history of IUGR. Long-term studies are needed to assess ultimate effects on final height.

A 5-year prospective study of growth hormone (GH)-deficient children treated with GH before the age of 3 years. French Serono Study Group.

The aim of the study was to assess the efficacy of GH therapy in GH-deficient children treated before the age of 3 yr. A noncomparative multicenter prospective study included 49 children (22 girls and 27 boys) with isolated GH deficiency (n = 19) or multiple pituitary hormone deficiency (n = 30) treated with daily s.c. injections (0.6 U/kg.week) for 3-5 yr. They were divided into two groups according to their height SD score for chronological age (CA) at the initiation of therapy: group A consisted of 8 patients presenting an initial height within the normal range (< 2 SD below the mean) followed for 2-5 yr, and group B consisted of 25 children followed for 5 yr among 41 patients with initial growth retardation. In group A, the mean height SD score increased from -1.1 +/- 0.6 to 0.35 +/- 1.0 SD (P < 0.001) in the first year and remained in the normal range throughout the following 4 yr. In group B after 4 yr of treatment, the mean height SD score for age had increased from -3.6 +/- 1.0 SD (time zero) to -0.9 +/- 1.2 SD. During the fourth year of therapy, the mean height gain of 0.2 +/- 0.2 SD was significant (P < 0.001). After 5 yr of treatment, a plateau was reached with a corresponding height SD score (CA) of -0.8 +/- 1.2 SD (95% confidence interval between -1.3 and -0.2 SD). This value remained significantly below normal for age (P < 0.001), indicating that catch-up growth was incomplete. Only four patients (16%) remained below -2SD for CA. The 5-yr height gain was negatively correlated with the height SD score at the start of treatment (r = -0.6; P < 0.005) and the first year height gain was the most predictive parameter. There was no significant influence of intrauterine growth retardation, body mass index and age at the start of treatment, or parental target height. Bone maturation was significantly retarded over CA by a mean value of 1.1 +/- 0.9 yr (P < 0.0001), with a mean bone age/CA ratio of 0.8 +/- 0.2 after a mean treatment duration of 5.1 +/- 1.1 yr. In conclusion, the rapid and almost complete return to normal height obtained in this study supports the need for GH treatment in early diagnosed GH-deficient children. The present dosage may be considered the minimum to obtain satisfactory catch-up growth ensuring a favorable outcome for these children. In addition, it allowed growth at a rate normal for age in patients diagnosed before growth retardation.

A curable cause of chronic idiopathic intestinal pseudo-obstruction in children: idiopathic myositis of the small intestine.

We report an intestinal pseudo-obstruction syndrome occurring in a 6-month-old infant girl suffering from acquired major and persistent abdominal distension, which 2 months later required ileostomy. Histologic examination of samples of the small intestine showed considerable inflammatory reaction in the muscular layers of the intestinal wall. Steroid therapy, begun as soon as the histologic results were known, resulted in recovery. In a similar case, reported previously, steroid therapy used after the lesions had already caused fibrosis and atrophy of the intestinal wall, proved ineffective. This particular form of pseudo-obstruction is classified as "idiopathic myositis of the small intestine." It is important to identify the condition because an early course of steroid therapy, before the appearance of fibrotic lesions, could improve the prognosis.

[Urinary tract abnormalities with prenatal diagnosis: neonatal management and outcome of 100 children born 1988-1990 at the Angers CHU (University Hospital)]. / Uropathies malformatives de diagnostic anténatal: prise en charge néonatale et devenir de 100 enfants nés entre 1988 et 1990 au CHU d'Angers.

UNLABELLED: Malformative uropathies diagnosed in utero are increasing in number. This work describes the decision strategy adopted in Angers concerning the neonatal handling of those abnormalities. PATIENTS AND METHODS: One hundred children born between 1988 and 1990, with prenatally suspected fetal uropathy, were included in the study and followed for a period of 3 years. In every case, an ultrasound scan was performed at birth. In cases with persistent abnormality, a voiding cystourethrography was done in the first week of life. An intravenous urograph and/or a nuclear renography were performed during the second month of life. RESULTS: Twenty-nine children were normal. Seventy-one were affected by 126 isolated or related uropathies; the most frequent ones were the ureteropelvic junction obstruction syndrome (48), ureterovesical junction obstruction (18) and multicystic kidneys (13). A vesicoureteral reflux was associated in 22% of cases. The diagnosis was perfectly correlated with the prenatal diagnosis in 50% of cases. Fourteen of the normal children had a later ultrasound scan control, between 2 and 9 months; later on, three of them showed a moderate ureteropelvic junction obstruction syndrome. Amongst the 48 ureteropelvic junction obstruction syndromes, 22% have been operated on. The others obstructive uropathies remained stable or spontaneously improved. DISCUSSION: These results require us to discuss as matter of priority the large number of spontaneously regressive prenatal hydronephrosis and the necessity to establish a consensus for the pre and postnatal pathological thresholds of the anteroposterior pelvic diameter, the interest in carrying out a voiding cystography after the birth when dilatation is confirmed, the interest of nuclear renography for the diagnosis and follow-up of obstructive uropathies and the absence of urgency for surgery. CONCLUSION: We propose a decision tree specifying the action to take when facing the diagnosis of a prenatal hydronephrosis. It could be a part of the reflection for the medical teams handling these uropathies is the aim of a consensual attitude which is now essential.

Growth factors and intrauterine growth retardation. II. Serum growth hormone, insulin-like growth factor (IGF) I, and IGF-binding protein 3 levels in children with intrauterine growth retardation compared with normal control subjects: prospective study from birth to two years of age. Study Group of IUGR.

The aim of this study was to describe serum GH, IGF-I, and IGF binding protein (BP) 3 levels at birth and during the first 2 y of life in intrauterine growth-retarded (IUGR) children and to correlate these hormonal values with auxologic parameters noted during this period to investigate their predictive value on the postnatal growth pattern. Three hundred and seventeen children were included at birth and studied for auxologic and biologic parameters at birth, 3 and 30 d, and 3, 6, 12, 18, and 24 mo of age. At birth, when analyzed according to gestational age, serum GH levels were increased (p = 0.0001) and serum IGF-I and IGFBP3 levels were decreased (p = 0.0001) in IUGR as compared with normal neonates. When two cohorts were established at birth as a function of the ponderal index (PI) (< or = or > 3rd percentile), serum IGF-I and IGFBP3 levels were found to be significantly reduced in the case of low PI. All parameters were within normal limits at 1 mo of age and remained normal thereafter. During the first 3 mo of life, a positive correlation was found between IGF-I increment and weight gain (r = 0.28, p = 0.002). None of the biologic parameters at birth were predictive either of later growth or of short stature at 2 y of age. In conclusion, low serum IGF-I and IGFBP3 levels at birth were related to fetal malnutrition and were not predictive parameters for later growth.

[A rare cause of severe diarrhea in children: pseudomembranous colitis]. / Une cause rare de diarrhée grave de l'enfant: la colite pseudomembraneuse.

BACKGROUND: Pseudomembranous colitis is a rare and serious complication of treatment by antibiotics. The case of a patient with a protracted pseudomembranous colitis followed by two relapses is reported. CASE REPORT: A 4 year-old boy was admitted after 18 days of profuse and feverish diarrhea. He had been given amoxycillin for 10 days, one and a half months previously. His temperature was 40 degrees C; he had abdominal pain and leucocytosis was 30,000/mm3. The situation rapidly improved with digestive rest and i.v. antibiotic therapy. Relapse of diarrhea together with bilious vomiting and acute abdominal pains required readmission three days after his discharge. Search for Clostridium difficile in stools remained negative. The diagnosis of pseudomembranous colitis was confirmed by sigmoidoscopy and intestinal biopsy. The patient was given parenteral nutrition for 3 weeks and vancomycin. The disease was complicated by anasarca related to severe protein-loosing enteropathy but evolution was finally favourable after a two month period. CONCLUSION: Pseudomembranous colitis remains a serious affection in childhood; its prognosis largely depends on the precocity of diagnosis and treatment.