[Social participation after childhood craniopharyngioma].

Unlabelled: Craniopharyngioma is a rare, benign central nervous system tumor, which may be a source of multiple complications, from endocrinology to vision, neurology and neurocognitive functions. This morbidity can lead to reduced participation in life activities, as described in the International Classification of Functioning, Disability, and Health. The primary objective of this study was to measure participation in life activities in a population of children and young adults affected by childhood craniopharyngioma, using the LIFE-H questionnaire (Assessment of Life Habits), validated as a social participation measurement tool in various pediatric disabilities.

Age at Birth of First Child and Fecundity of Women Survivors of Childhood Acute Lymphoblastic Leukemia (1987-2007): A Study of the Childhood Cancer Registry of the Rhône-Alpes Region in France (ARCERRA).

We studied the fecundity of 174 successive ALL (1987-2007) in females of the Childhood Cancer Registry of the Rhône-Alpes Region (ARCERRA) with a median age at follow-up of 25.6 years (18.0-37.

Pineal region tumors: Clinical symptoms and syndromes.

The present paper investigates the clinical picture and the different clinical signs that reveal pineal region tumors or appear during the course of the follow-up. Biological malignancy and tumor extension determine the semiology and its setting up mode. Typical endocrine signs, dominated by abnormal puberty development, are frequently a part of the clinical scene.

Mini-invasive surgery for adrenocortical carcinoma in children: is it safe?

Laparoscopy in adrenocortical carcinoma is controversial due to high risk of local recurrence in case of per-operative spillage. We wanted to report the case of a successful laparoscopic transperitoneal adrenalectomy performed in a 3-year-old girl presenting a 5.5-cm adrenocortical carcinoma.

[Papillar thyroid cancer: a rare case of a second primary tumor in retinoblastoma].

Retinoblastoma is the most common primary cancer of the eye in children. The prognosis for survival is excellent. The current therapy includes an improved survival rate and decreased iatrogenic sequelae.

Final height and body mass index after fractionated total body irradiation and allogeneic stem cell transplantation in childhood leukemia.

Impaired linear growth has been reported in patients treated during childhood with allogeneic stem cell transplantation and fractionated total body irradiation (fTBI). The objective of this study was to determine the final height and body mass index (BMI) achieved. Forty-nine patients with leukemia were included and surveyed for more than 5 years.

Optimization of growth hormone dosing in children born small for gestational age: an open-label, randomized study of children during the fourth year of therapy.

Background: Optimal dosage for growth hormone (GH) therapy in short, prepubertal children born small for gestational age (SGA) is controversial.

Methods: SGA OPTIMIS (NCT00249821) is a multicenter, open-label, parallel-group, pilot study of short children born SGA who had received recombinant human GH (r-hGH) (57 μg/kg/day) for 3 years. Children were randomized 1:1 to receive either 57 or 35 μg/kg/day r-hGH during year 4.

Sporadic and genetic forms of paediatric somatotropinoma: a retrospective analysis of seven cases and a review of the literature.

Background: Somatotropinoma, a pituitary adenoma characterised by excessive production of growth hormone (GH), is extremely rare in childhood. A genetic defect is evident in some cases; known genetic changes include: multiple endocrine neoplasia type 1 (MEN1); Carney complex; McCune-Albright syndrome; and, more recently identified, aryl hydrocarbon receptor-interacting protein (AIP). We describe seven children with somatotropinoma with a special focus on the differences between genetic and sporadic forms.

Growth hormone treatment before the age of 4 years prevents short stature in young girls with Turner syndrome.

Objective: Adult height deficit seen in Turner syndrome (TS) originates, in part, from growth retardation in utero and throughout the first 3 years of life. Earlier diagnosis enables earlier therapeutic intervention, such as with recombinant human GH (r-hGH), which may help to prevent growth retardation. In this open-label, multicentre phase III study, we investigated efficacy and safety in r-hGH treatment in young girls with TS.

Improvement of diencephalic syndrome after partial surgery of optic chiasm glioma.

A 10-month-old male presented with sudden growth failure and cachexia. MRI showed a chiasma of the hypothalamic mass. Biopsy was avoided due to operative risks.

Craniopharyngiomas: our experience in Lyon.

Objective: We reviewed our experience in surgical treatment of craniopharyngiomas. Surgical treatment of craniopharyngiomas in children represents a challenge for neurosurgeons because it presents a different set of surgical problems. Results are controversial and debates concerning strategies to ameliorate the rate of success and to decrease the rate of morbidity and mortality are ongoing.

Antileukemic and long-term effects of two regimens with or without TBI in allogeneic bone marrow transplantation for childhood acute lymphoblastic leukemia.

Between September 1986 and June 1997, 24 children with high-risk ALL in CR1 were allografted after TAM (fractionated TBI, high-dose Ara-C, and melphalan; n = 10) or BAM protocol (busulfan, high-dose Ara-C, and melphalan; n = 14). The EFS for transplants from sibling donors was 33% with TAM and 62% with BAM (P = 0.148).

Intracystic chemotherapy with bleomycin in the treatment of craniopharyngiomas.

Object: Since bleomycin has not yet been used very frequently in the treatment of patients with craniopharyngioma, it seemed important to document the course of a series of such patients treated with this preparation.

Methods And Results: Local chemotherapy with bleomycin was performed in 24 patients (20 children and 4 adults), 16 of whom presented with cystic or mixed (solid/cystic) craniopharyngioma and 8, with recurrent cystic craniopharyngioma. The drug was administered through an Ommaya reservoir, which was placed either by using a direct surgical approach (6 patients) or a stereotactic approach (16 patients), or with endoscopic assistance in patients with hydrocephaly (2 patients).

Hypothalamic hamartoma: the role of surgery in a series of eight patients.

Hypothalamic hamartoma are rare lesions. We report a new series of eight patients treated for precocious puberty (six cases) or gelastic seizures (two cases). Surgical resection was total in four cases (three pediculated and one sessile).

[Ventriculostomy of the third ventricle and diabetes insipidus].

We report the case of a 2-year-old child who sustained a permanent diabetes insipidus following a third ventriculostomy for hydrocephalus from a Dandy-Walker syndrome. Ventriculostomy, used for therapy of non-communicating hydrocephalus, can cause complications such as diabetes insipidus. The latter has rarely been reported and is usually transient.

Is the response to rhGH in haemodialysis patients less effective than in patients with chronic renal failure? Société de Néphrologie Pédiatrique.

A total of 82 prepubertal children with chronic renal insufficiency and with (HD, n = 28) or without (CRI, n = 54) haemodialysis were treated with rhGH, 1 i.u./kg/week.

[Vulvitis in young girls: value of cleansing base with concentrated colloidal oat extract].

Vulvitis is a frequent infection in young girls. Fourty prepubertal children with recurrent vulvitis were submitted, besides usual local hygiene rules, to a twice daily toilet with a cleansing base containing colloidal oat extract (Emulave fluid). It was associated with a colouring product in case of severe local inflammation during the first days of treatment.

[Study of the dimensions of the penis from birth to adult age and as a function of testicular volume].

Year to year mean +/- 2 standard deviation values of the length and circumference of the penis from birth to 18 years have been determined on a population of white French children and adolescents of European origin. In addition, curves relating the length and the circumference of the penis with the testicular volume are reported. These curves appear to be useful for an appropriate evaluation of the size of the penis in the adolescents where large variations of the pubertal development are observed.

[Virilizing ovarian tumor in an adolescent].

This clinical case describes a 13 year-old pubertal girl suffering from secondary virilization, amenorrhea, and abdominal mass. Plasma testosterone and androstenedione levels were markedly elevated. Upon surgery, an ovarian tumor containing 5 l of liquid was removed.

[Benign vulvovaginal tumefactions in children. General review illustrated by a series of 57 personal cases].

There are numerous etiologies of benign vulvo-vaginal tumefactions in children. However, the etiological diagnosis can usually be based on clinical data after careful examination. Para-urethral cysts are the main cause of labial and vestibular tumefactions; they are generally cured by simple puncture.

[Isolated metrorrhagia in a prepubertal child caused by functional ovarian cyst].

The authors report the case of a 10-year-old girl, without any sign of pubertal development, who showed repeated metrorrhagia, related to a functional ovarian cyst. Cyproterone acetate therapy gave only a transient remission of the metrorrhagia. A complete remission was obtained with a 6 months treatment with an LHRH analog.

[Treatment of purulent leukorrhea in young girls].

The case of a prepuberal girl complaining of a more or less purulent genital discharge, eventually associated with bleeding, evokes a vulvovaginitis. Vulvovaginitis is much less frequent than vulvitis. When a vulvovaginitis is resistant to medical treatment or is recurrent, an endoscopic examination is required in order to eliminate the presence of a vaginal foreign body.