Characterization of adipocytes derived from fibro/adipogenic progenitors resident in human skeletal muscle.

A population of fibro/adipogenic but non-myogenic progenitors located between skeletal muscle fibers was recently discovered. The aim of this study was to determine the extent to which these progenitors differentiate into fully functional adipocytes. The characterization of muscle progenitor-derived adipocytes is a central issue in understanding muscle homeostasis. They are considered as being the cellular origin of intermuscular adipose tissue that develops in several pathophysiological situations. Here fibro/adipogenic progenitors were isolated from a panel of 15 human muscle biopsies on the basis of the specific cell-surface immunophenotype CD15+/PDGFRα+CD56-. This allowed investigations of their differentiation into adipocytes and the cellular functions of terminally differentiated adipocytes. Adipogenic differentiation was found to be regulated by the same effectors as those regulating differentiation of progenitors derived from white subcutaneous adipose tissue. Similarly, basic adipocyte functions, such as triglyceride synthesis and lipolysis occurred at levels similar to those observed with subcutaneous adipose tissue progenitor-derived adipocytes. However, muscle progenitor-derived adipocytes were found to be insensitive to insulin-induced glucose uptake, in association with the impairment of phosphorylation of key insulin-signaling effectors. Our findings indicate that muscle adipogenic progenitors give rise to bona fide white adipocytes that have the unexpected feature of being insulin-resistant.

Prospective study on the prevalence and associated risk factors of cryptorchidism in 6246 newborn boys from Nice area, France.

To assess the incidence and risk factors of cryptorchidism in Nice area. A 3-year prospective study was conducted at two maternity wards involving neonatal screening of boys born ≥34weeks of amenorrhoea. Methodology was strict with examination at birth, 3 and 12months by the same paediatrician. Two strictly matched controls were included for each case. Information on child and parents (medical history, pregnancy, lifestyle) was recorded using medical chart and self-administered questionnaires. A total of 102 of 6246 boys were born with cryptorchidism (prevalence 1.6%, 95 included). Half of them were still cryptorchid at three and 12months with, however, 10% of secondary re-ascent (recurrent cryptorchidism) at 12months, justifying long-term follow-up. Cryptorchidism at birth was associated with instrumental delivery, inguinal hernia and urogenital malformations, particularly micropenis and paternal history of cryptorchidism. Our results suggest that maternal exposure to anti-rust or phthalates could be a risk factor, whereas eating fruits daily seemed somewhat protective. Prevalence of cryptorchidism in our area is on the lower bracket compared with other countries, and is associated with both familial and environmental risk factors.

[Abdominal aortic injury in a child: seat-belt syndrome]. / Traumatisme de l'aorte abdominale : « le syndrome de la ceinture de sécurité ¼

Traumatic injuries of the abdominal aorta are rare, especially in the pediatric population, but they must be sought when the clinical signs are associated with a severe traumatic context. We report the case of a 7-year-old child who presented with a partial transection of the abdominal aorta following a traffic accident. The optimal management is based on the detection of the less striking but suggestive clinical signs, such as a belt hematoma in the abdominal wall or a decreased pulse in the lower limbs, as well as a systematic precise X-ray examination, and an urgent surgical intervention. The risk is complete rupture, whose time of occurrence after the accident is unpredictable. The abdominal aorta is anatomically protected, so injury in children is traditionally associated with seat belt wear. Lesions are related to the shearing of the abdominal aorta between the spine and the seat belt.

[Noninvoluting congenital hemangioma: 2 cases]. / Hémangiome congénital non-involutif: 2 cas.

INTRODUCTION: Hemangiomas (or immature hemangiomas) are characterized by a stereotyped 3-phase evolution: proliferation, stabilization and regression. The rare congenital hemangiomas are present at birth and regress spontaneously more rapidly. However, certain congenital hemangiomas, described recently as "noninvoluting congenital hemangiomas", evolve differently and do not regress. We report two cases. OBSERVATIONS: Two adolescents aged 16 and 17 were born with a congenital hemangioma. Its evolution during childhood was marked by the progressive collapse of the lesion and lightening of the skin. After stabilizing, a round, centrally involuted lesion persisted with numerous telangiectasia on the surface and peripheral varicosities. The lesion was hot but no pulsation or murmur were observed. Doppler sonography revealed a rapid flowing lesion, limited to the cutaneous areas. Magnetic resonance imaging, conducted in one case, showed a hypersignal area limited to the skin. DISCUSSION: Noninvoluting congenital hemangiomas can be differentiated from classical congenital hemangiomas by their partial regression, stereotyped clinical aspect and a certain activity after stabilization. All the clinical, histological and imaging data support a vascular malformation with proliferative component, rather than a true hemangioma. Whenever possible, treatment is surgical. Knowledge of the existence of this type of angioma is important, and the dermatologist should be careful when reassuring parents of children presenting with congenital hemangioma.

ALK probe rearrangement in a t(2;11;2)(p23;p15;q31) translocation found in a prenatal myofibroblastic fibrous lesion: toward a molecular definition of an inflammatory myofibroblastic tumor family?

A prenatal tumor located in the lumbar paravertebral area was discovered during a routine ultrasound examination at 32 weeks of pregnancy and surgically removed at 4 months of life. The histopathological diagnosis was first suggested to be an infantile desmoid fibromatosis. The tumor karyotype showed a three-way translocation involving both chromosomes 2 and a chromosome 11, t(2;11;2)(p23;p15;q31). Fluorescence in situ hybridization with a probe flanking the ALK gene at 2p23 demonstrated a rearrangement, as previously described in inflammatory myofibroblastic tumors (IMTs). In light of the genetic analysis, the histopathological diagnosis was revised to IMT, although inflammatory cells were scarce. IMTs are pseudosarcomatous inflammatory lesions that primarily occur in the soft tissue and viscera of children and young adults. Our report describes for the first time the occurrence of IMT during prenatal life. The ALK rearrangement may represent the molecular definition of a subgroup of mesenchymal tumors, not always with complete morphological features of IMT, similar to the model of EWS rearrangement in the Ewing sarcoma family of tumors.

[Value of thoracoscopy in purulent pleuresies in children younger than four years]. / Intérêt de la thoracoscopie dans les pleurésies purulentes de l'enfant de moins de quatre ans.

UNLABELLED: Video-assisted thoracoscopic surgery is widely performed in adults but there are few publications concerning the paediatric population. The objective is to effect optimal adhesiolysis of post-pneumonic loculated empyema with lower morbidity. PATIENTS AND METHODS: Over a 4-year period we used thoracoscopic debridement in five children younger than 4 years of age with loculated thoracic empyema. All patients failed initial treatment, including antibiotics and chest tube drainage. Early sonographic evaluation of the empyema organization guided the most appropriate moment for the intervention. The average duration of tube drainage after thoracoscopy was 4 days (range: 1 to 7 days). RESULTS: All patients made an uneventful postoperative recovery. At a follow-up visit 1 month after discharge, the children were clinically asymptomatic; however, some degree of pleural thickening was still visible on chest X-rays. CONCLUSION: In skilled hands, thoracoscopy is a safe procedure for post-pneumonic empyema in young children, providing a rapid clinical and radiological recovery with a good cosmetic result.

Partial splenectomy in a child with human immunodeficiency virus-related immune thrombocytopenia.

Immune thrombocytopenia (IT) is a frequently occurring disease in childhood and a well known complication of HIV infection. Splenectomy is a part of the treatment strategy for severe chronic IT. However, overwhelming infections after splenectomy have limited its use, especially in young children. A 7-year-old child with maternal-fetal HIV-1 infection and related thrombocytopenia underwent splenectomy after previous treatment failed to improve her platelet count. Approximately 75% of the spleen was removed. The postoperative period was uncomplicated, and the platelet count increased significantly to greater than 500,000/mm3. Ultrasonographic examination performed 3 months later showed a stable volume of the spleen stump (40 x 40 x 20 mm) with effective "vascularization." The platelet count 12 months after surgery showed a sustained increase greater than 150,000 cell/mm3. Subtotal splenectomy may be a safe and effective alternative for patients with HIV and immune thrombocytopenia.

Increased urinary catecholamines in a hypertensive child with renal artery stenosis.

We report a hypertensive child with renal artery stenosis who exhibited increased urinary excretion of norepinephrine (NE) and normetanephrine (NMN), while vanillylmandelic acid (VMA) excretion was within the normal range. The NMN values prompted us to investigate the patient for pheochromocytoma; for this purpose, NE was determined by plasma catecholamine assays in venous samples obtained by catheterization. The moderately increased NE levels could not be localized to any particular sampling site. Arteriography demonstrated right renal artery abnormalities. Following right nephrectomy with preservation of the right adrenal gland, arterial blood pressure returned to normal. The cause of increased NMN excretion without a concomitant rise in VMA during hypertension is discussed.

[Pseudo-tumoral circumscribed myositis ossificans of the thoracic wall. Contribution of ultrasonography, x-ray computed tomography and MRI]. / Myosite ossifiante circonscrite pseudo-tumorale de la paroi thoracique. Apport respectif de l'échographie, de la tomodensitométrie et de l'IRM.

The authors report the main clinical and radiographic aspects of a circumscribed myositis ossificans in a 5 year old child located in the thoracic wall, near the sternum, stimulating a malignant tumor. CT scan was more contributive than MRI, both for diagnosis and survey, because it shows better the progressive peripheral calcification typical of the lesion.

[Meconium peritonitis. The value of prenatal diagnosis]. / Péritonites méconiales. Intérêt du diagnostic prénatal.

Over an eight year period (1980-1987), fifteen cases of meconium peritonitis have been studied. As it has been reported, this pathology is still very rare. Ten pregnancies have been followed on by multiple échographies and the prenatal diagnosis of meconium peritonitis, suspected as early as 24 weeks of amenorrhea, has been established in ten cases. Among these, eight infants have been operated on before twelve hours of life, and for the ninth, surgical treatment was not advocated. The most frequent cause of the pathology is a perforation above a bowel obstruction. Two had cystic fibrosis. Of the 15 infants with meconium peritonitis, nine survived, and among these, one with cystic fibrosis and the one that has not been operated. Prenatal diagnosis in meconium peritonitis is of major interest in taking care early infants that will need an urgent surgical operation in most cases.

[Rhabdomyosarcomas discovered in newborn infants. Study of markers of muscle differentiation]. / Rhabdomyosarcomes de découverte néonatale. Etude des marqueurs de différenciation musculaire.

Rhabdomyosarcomas were observed at birth in two premature infants. The evolution of these tumors was both rapid and fatal. One case was a botryoid sarcoma of the bladder and the second was a cervical alveolar sarcoma of Riopelle and Thériault. A cytological differentiation of the tumor was observed in the first case spontaneously and in the second following chemotherapy. In the second case histology, immunocytochemistry and a novel biochemical analysis of the contractile proteins demonstrated this differentiation of the tumor. The cellular reactivity varies according to the degree of muscular differentiation that can be defined either by studying the type of intermediate filaments present or the expression of specific muscle contractile proteins.

Trifid pelvis and controlateral bifid ureter with bilateral ureteropelvic junction obstruction.

A 20-month-old boy with a trifid pelvis and ureteropelvic junction obstruction on one side and incomplete duplication of the ureter with ureteropelvic junction obstruction on the lower pole of the other kidney is described.

[Esophageal atresia in newborn infants weighing less than 2,000 grams. Apropos of 37 case reports]. / Atrésie de l'oesophage chez les nouveau-nés de moins de 2,000 grammes. A propos de 37 observations.

In a consecutive series of 275 infants with esophageal atresia seen at Saint-Vincent-de-Paul's Hospital (Paris) between 1971 to 1987, the authors report the experience with 37 infants weighed under 2,000 g (13.45%). 19 had severe additional malformations (51.35%) and 10 (27%) had a respiratory distress syndrome. As Abrahamson in 1972 (3), Cozzi an Wilkinson in 1975 (4), Rickham in 1981 (5) reported, according to the criteria suggested in 1962 by Waterston (1), survival rate are related to additional congenital anomalies and initial respiratory distress (pulmonary dysmaturity or pneumonia) but seems more related to maturity (small-for-date babies) than to birth weight. The authors recommend to perform a primary division of the tracheo-esophageal fistula and end-to-end esophageal anastomosis whenever possible.

[Laparoschisis. Hypotrophy and intestinal lesions]. / Laparoschisis. Hypotrophie et lésions intestinales.

37 cases of gastroschisis were operated on at Saint-Vincent-de-Paul's Hospital from january 1983 to july 1987. They were more frequently premature by birth weights (70% below the 10 th percentile), than by period of gestation (40%). The comparison of their weights plotted on the centile charts with 28 neonates with omphaloceles and 25 neonates with upper intestinal atresia (esophageal atresia without tracheoesophageal fistula and complete duodenal and proximal jejunal atresia) shows that the 2 groups of laparoschisis and anomalies of the alimentary tract are quite similar. The authors suggest that the amniotic fluid may play a role in the fetal growth so that the alteration of the herniated bowel is responsible of the fetal growth retardation observed in gastroschisis.

[Congenital diaphragmatic hernia. 120 neonatal cases. Preliminary study]. / Hernies diaphragmatiques congénitales: 120 cas néo-nataux. Etude préliminaire.

120 newborns with a congenital diaphragmatic hernia (CDH) were treated during the first day after birth in the Department of Pediatric Surgery at Saint Vincent de Paul's Hospital from 1980 to 1987. The overall survival rate was 40%. The influence of prenatal diagnosis, clinical and anatomical aspects is analysed. The side and the size of the defect, the intra-thoracic liver, the intestinal malrotation and the abdominal hypoplasia are significant prognosis factors. This preliminary study suggests that the natural history of CDH may be responsible of two different diseases: a disease with an early visceral intra-thoracic growth, and a severe lung hypoplasia; another one with a secondary herniation of the intra-abdominal mass, and a better prognosis.

[Blunt injuries of the pancreas in children. Diagnostic and therapeutic approach apropos of 8 cases]. / Traumatismes fermés du pancréas de l'enfant. Approche diagnostique et thérapeutique à propos de 8 cas.

From 1970 to 1985, eight severe blunt pancreatic traumas were admitted. There is significant difference in morbidity between early pancreatectomy with or without splenectomy (a mean hospital stay of 23 days, low loss of weight) and initial simple external pancreatic drainage with delayed partial pancreatectomy or pancreato-cystojejunostomy Roux-en-Y (mean hospital stay of 45 days, 24% loss of weight, one death two years later). Thus it seems essential to diagnose the pancreatic injury and particularly rupture of the pancreatic duct. Aiming to evaluate these lesions, biology, ultrasonography or computerized axial tomography proved insufficient. As a result, in the last two patients, an endoscopic retrograde pancreatography was performed confirming total transection of the pancreatic duct, one case with fistula, the other with a contrast fluid stop. The surgical approach was guided by these X-ray findings and a distal pancreatectomy performed preserving spleen. The authors propose the following protocol: endoscopic pancreatography if an evident improvement in recent pancreatic injury is not obtained in 48 hours, or in the case of former complicated pancreatic trauma; in the event of total rupture of pancreatic duct, operation should be carried out: abdominal exploration guided by the X-rays findings, distal pancreatectomy or, rarely, repair of the pancreatic duct; when no pancreatic duct lesion is found, but ultrasonographic blunt trauma patent, the surgical decision depends on the amount of peripancreatic reaction: medical treatment or external drainage; when lesion of the head of pancreas is detected, conservative treatment is to be preferred to pancreatoduodenectomy.

[An aid toward decision in congenital hip dislocation. Value of decision trees in the determination and treatment of hips at risk (preliminary study)]. / L'aide à la décision dans la luxation congénitale de hanche. Apport des arbres de décision dans la détermination et le traitement des hanches à risque (étude préliminaire).

Well established procedures exist for treatment of congenital dislocation of hip, whereas unanimity is lacking with respect to management of a hip at risk during the neonatal period. Based on a homogeneous series of 720 case-reports of neonates at risk a decision tree has been developed to determine essential risk factors and to demonstrate preferential pathways when evaluating appropriate therapy.

[Testicular exploration by Doppler ultrasonography in children. Importance, indications, limitations. Apropos of 90 cases]. / Exploration testiculaire par ultrasonographie Doppler chez l'enfant. Intérêt, indication, limites. A propos de 90 cas.

The authors evaluate the testicular blood flow by Doppler stethoscope in 90 infants and children; they tell two indications: In emergency the most common is "acute scrotal swelling": 78 cases, 4 false negative are reported in diagnosis of torsion of the spermatic cord; the limit of the Doppler examination are the possibility of a interepididymo-testicular torsion and, above all, the inflammation of the scrotal wall. If Doppler examination are in agreement, unnecessary surgical explorations must be avoided. Outside emergency, Doppler examination may be help-full to evaluate varicocele and to control some surgical operation involving spermatic cord (12 cases).

[Esophageal plasty in children. Retrospective study of 22 colonic transplants. Current orientation]. / Plasties oesophagiennes chez l'enfant. Etude rétrospective de 22 transplants coliques. Orientation actuelle.

Esophageal replacement using transverse colon with left colic pedicle is reviewed. 18 were done for caustic stenosis with one death, 4 for atresia without mortality. For caustic stenosis the authors advocate early transplant since the third month if dilatations failed. Improvement of technique using staplers prevented any complication in the last 8 patients. These good results allowed the authors to switch to isoperistaltic gastric tube. In esophageal atresias, the authors did not find any indication of replacement in the 12 last years.