[Giant mesenteric lipoma in children: A case-report]. / Lipome mésentérique géant chez l'enfant : à propos d'un cas.

Mesenteric lipoma is an extremely rare disease in children. Fewer than 50 cases have been reported in the literature. Diagnosis is based on clinical examination, ultrasound, and computed tomography (CT). However, only the histological study of the specimen during laparotomy or laparoscopy can confirm the diagnosis. Thus, surgery, be it by laparotomy or laparoscopic, is both a means of exploration and treatment in mesenteric lipoma. We report on a case of giant lipoma of the mesentery in a 7-year-old girl presenting paroxysmal abdominal pain with a subocclusive syndrome lasting 1 week.

[Acute retention of urine secondary to a congenital diverticulum of the bladder]. / Rétention aiguë d'urine secondaire à un diverticule congénital bilatéral de la vessie.

Bladder diverticula in children are mostly congenital, frequently associated with vesicoureteral reflux. Their positive diagnosis is based primarily on retrograde urethrocystography. One complication of this condition is acute urinary retention. We report the case of a 4-month-old infant who presented acute retention of urine secondary to bilateral congenital bladder diverticulum and review the literature on this subject.

[Inflammatory tumor of the great omentum after ingestion of a bone fragment]. / Tumeur inflammatoire du grand épiploon après ingestion d'un fragment osseux.

The ingestion of a foreign body is one of the most common accidents in children. It comes in extremely varied clinical presentations in which radiological imaging plays an important role in diagnosis. Colonic perforations are particularly serious because of the major risk for septic fecal peritonitis. Management depends on the nature of the ingested object, its location, the child's age and the child's clinical status. We report a child presenting an inflammatory tumor of the omentum on an ingested bone fragment that had perforated the transverse colon.

Circulating microparticles from diabetic rats impair endothelial function and regulate endothelial protein expression.

AIM: Diabetes mellitus increases the risk of cardiovascular disease, which is accompanied by functional and structural changes in the vascular system. Microparticles (MPs) have been described as biological vectors of endothelial dysfunction in other pathologies. However, the molecular mechanisms underlying their formation and signalling are unclear. We investigated the role of MPs derived from streptozotocin (STZ)-induced diabetic rats in endothelial function. METHODS: Male Wistar rats were injected with STZ to induce diabetes, and MPs isolated from control or STZ-induced diabetic rats were characterized by dot blotting (assessed by CD62P detections), flow cytometry (assessed by annexin V detections) and ELISA. Carotid arteries from rats were incubated with MPs, and expressions of enzymes and endothelium-dependent relaxation were analysed. RESULTS: The circulating levels of MPs, particularly the levels of platelet-derived microparticles, from diabetic rats were higher than those present in controls. Endothelium-dependent relaxation induced by acetylcholine (ACh) was attenuated in carotid arteries from STZ-induced diabetic rats. Following the incubation of control carotid arteries with MPs isolated from STZ rats, ACh-induced endothelium-dependent relaxation was impaired, but MPs isolated from control rats had no such effect. Furthermore, the effect of MPs was mediated by a decrease in expression of endothelial nitric oxide synthase (eNOS) and the overexpression of caveolin-1. CONCLUSION: Circulating MPs isolated from STZ-induced diabetic rats induce endothelial dysfunction in carotid arteries and regulate protein expressions of eNOS and caveolin-1. These data advance our understanding of the deleterious effects of circulating MPs observed in disorders with diabetic complications.

[Colonic duplication revealed by intestinal obstruction due to fecal impaction]. / Duplication colique découverte à l'occasion d'une occlusion intestinale par fécalome.

Colonic duplications are very rare in children. With rectal duplications, they are the rarest locations of alimentary tract duplications, most often diagnosed in the first years of life. We report an unusual case of colic duplication with fecal impaction in a 9-month-old boy revealed by intestinal obstruction. We discuss the main diagnostic and therapeutic aspects of this malformation.

[Osteomyelitis of the mandible and dysosteosclerosis]. / Dysostéosclérose et ostéite chronique de la mandibule.

Dysosteosclerosis is a rare genetic disorder with a poor prognosis. It is an osteochondrodysplasia similar to osteopetrosis but it is typically characterized by platyspondyly and expanded metaphyses. It shows complications such as compression of cranial nerves, especially the optic nerve, hematologic complications, fractures following mild injury, abnormal dentition, neurological and psychological deterioration, in addition to osteomyelitis of the mandible. We report a new case of dysosteosclerosis complicated by osteomyelitis of the lower maxilla.

[Subcutaneous emphysema, pneumomediastinum, pneumopericardium, pneumorachis, and pneumoretroperitoneum revealing an unknown foreign body aspiration]. / Emphysème sous-cutané massif, pneumomédiastin, pneumopéricarde, pneumorachis et pneumorétropéritoine révélant une inhalation méconnue d'un corps étranger.

Subcutaneous emphysema develops from the spread of air essentially from the mediastinum into the subcutaneous tissue causing progressive distension and infiltration. Diagnostic and therapeutic delay expose the patient to massive air effusion and risk of compression of cervical and mediastinal structures. The initial mechanism is a breach in the tracheobronchial tree with air diffusion into the interstitial space and along the perivascular spaces toward the mediastinum, and then spread and dissection in the subcutaneous tissue. A tracheobronchial foreign body is a very rare cause of emphysema and is often localized. An association with a combination of epidural emphysema, pneumopericardium, or pneumoretroperitoneum is exceptional. Here, we present a unique case associating massive subcutaneous emphysema, pneumomediastinum, pneumopericardium, pneumorrhachis, and pneumoretroperitoneum in a 3.5-year-old child complicating an unrecognized aspirated foreign body. The extraction of the foreign body resulted in gradual regression of the symptoms and the disappearance of these emphysematous locations.

Indications and results of renal biopsy in children: A single-center experience from Morocco.

The contribution of renal biopsy (RB) is of major importance in the management of many renal diseases in children. Specific indications for performing biopsy in children include steroid-resistant nephrotic syndrome (NS) and secondary nephropathies. The aim of our study was to report the common histological varieties of kidney diseases in children in Morocco. In this retrospective and descriptive study, we included all renal biopsies performed in patients under 16 years in the Department of Pediatrics of Hassan II University Hospital, Fez, Morocco from July 2009 to December 2013. Biopsy samples without glomeruli and those with less than five glomeruli or repeat biopsies on the same patient were excluded from our study. We performed 112 RBs during this period; the average age at the time of RB was 10.05 ± 4 years and the sex-ratio was 1.07. The indications for RB were NS with hematuria and/or renal failure (RF) in 32.1%, active urinary sediment in 21.4%, isolated NS in 15.2%, RF in 13.4% and steroid-resistant NS in 10.7% of cases. Primary nephropathies represented 59.8% of cases, with a predominance of minimal change disease (MCD) seen in 40.2% of the cases. Secondary nephropathies accounted for 27.7% of the cases, with a predominance of lupus nephritis (11.6%), followed by Henoch-Schonlein purpura nephritis (6.2% of cases) and post-streptococcal glomerulonephritis (3.6%). There was one case of hepatitis B virus-associated membranous glomerulonephritis. Chronic glomerulonephritis accounted for 12.5% of the cases. Vascular and tubulo-interstitial nephritis were rare. Our study confirmed that primary glomerular nephropathy was the most common renal disease in children. The most common lesion was MCD. Secondary nephropathies were less frequent, with a predominance of lupus nephritis.

[Hypercalcemia complicating BCG lymphadenitis: case report]. / Hypercalcémie compliquant une bécégite locorégionale : à propos d'un cas.

One of the side effects of the BCG vaccine is a local infection that may spread to the regional lymph nodes causing lymphadenitis, which can resolve spontaneously without treatment. We report the case of an immunocompetent infant who developed lymphadenitis after administration of the BCG vaccine, complicated with persistent symptomatic hypercalcemia in spite of the usual treatment including corticotherapy. Antituberculous treatment was necessary to reduce this hypercalcemia.

Screening for childhood lead poisoning in the industrial region of Fez, Morocco.

The study objectives were to estimate lead poisoning prevalence among children living next to an industrial area, to compare it to that in a control population, and to establish clinical and biological follow-up of the poisoned children. This is a descriptive cross-sectional study including 150 children (exposed and unexposed) performed between January 2012 and April 2013. It was meant to determine blood lead levels (BLLs) in children considered to be an exposed population (EP N 90), living in the industrial area Ain Nokb Fez compared with BLLs of children of other areas belonging to the same city supposed to be unexposed [UP (N = 60)]. A sociodemographic questionnaire was obtained, and a blood lead analysis was performed. Clinical and biological follow-up has been performed of poisoned children. The sample consisted of 90 EP children with an average age of 6.82 ± 3.32 years and male-to-female sex ratio (SR) of 1.5 and 60 UP children with an average age of 6.45 ± 3.29 years and an SR of 1.2. Among the 150 children recruited, the average of BLLs was 58.21 ± 36 µg/L (18-202.3 µg/L). The average of BLLs in EP children (71 ± 40 µg/L) was statistically greater (p < 0.0001) than that registered in UP children (38 ± 13 µg/L). All poisoned children belonged to the EP group at a prevalence of 21.1 %. The clinical and biological examinations of poisoned children showed a few perturbations such as anemia, hypocalcaemia, and deficiencies in magnesium and iron. No renal disease or objective neurological disorders were observed. In the follow-up of the children with BLL ≥100 µg/L (19 cases). BLL monitoring showed a significant decrease in average of blood concentration ranging from 136.75 ± 32.59 to 104.58 ± 32.73 µg/L (p < 0.0001) and in lead poisoning prevalence (p < 0.001), which decreased to 7.8 % from 21.1. Our study showed a high prevalence of lead poisoning (21.1 %) in EP children. The relocation of the industrial site associated with corrective and preventive measures has contributed to a decrease of exposure and lead poisoning prevalence in the aforementioned population.

[Seizures revealing phosphocalcic metabolism abnormalities]. / Crises épileptiques révélant des anomalies du métabolisme phosphocalcique.

Hypocalcemia due to hypoparathyroidism produces a broad spectrum of clinical manifestations, but overt symptoms may be sparse. One unusual presentation is onset or aggravation of epilepsy in adolescence revealing hypoparathyroidism. This situation can lead to delayed diagnosis, with inefficacity of the antiepileptic drugs. We report five cases of adolescence-onset epilepsy with unsuccessful antiepileptic therapy, even with gradually increasing dose. Physical examination revealed signs of hypocalcemia, confirmed biologically. Full testing disclosed the origin of the seizures: hypoparathyroidism in three patients and pseudohypoparathyroidism in the other two. In four of five patients, computed tomography showed calcification of the basal ganglia, defining Fahr's syndrome. The patients were treated with oral calcium and active vitamin D (1-alphahydroxy vitamin D3). Seizure frequency progressively decreased and serum calcium levels returned to normal. These cases illustrate the importance of the physical examination and of routine serum calcium assay in patients with new-onset epileptic seizures in order to detect hypocalcemia secondary to hypoparathyroidism.

First report on the Moroccan registry of primary immunodeficiencies: 15 years of experience (1998-2012).

PURPOSE: Primary immunodeficiencies (PIDs) are a large group of diseases characterized by susceptibility to infections. We provide the first comprehensive report on PIDs in Morocco, the epidemiological, clinical, etiological and outcome features which have never before been described. METHODS: A national registry was established in 2008, grouping together data for PID patients diagnosed since 1998. RESULTS: In total, 421 patients were diagnosed between 1998 and 2012. Parental consanguinity was common (recorded for 43.2 % of patients) and the median time to diagnosis was 2.0 years. Overall, 27.4 % of patients were considered to have well defined syndromes with immunodeficiency (48 cases of hyper-IgE syndrome and 40 of ataxia-telangiectasia); 22.7 % had predominantly antibody deficiencies (29 cases of agammaglobulinemia and 24 of CVID); 20.6 % had combined immunodeficiencies (37 cases of SCID and 26 of MHC II deficiencies) and 17.5 % had phagocyte disorders (14 cases of SCN and 10 of CGD). The principal clinical signs were lower respiratory tract infections (60.8 %), skin infections (33.5 %) and candidiasis (26.1 %). Mortality reached 28.8 %, and only ten patients underwent bone marrow transplantation. We analyzed the impact on mortality of residence, family history, parental consanguinity, date of diagnosis and time to diagnosis, but only date of diagnosis had a significant effect. CONCLUSIONS: The observed prevalence of PID was 0.81/100,000 inhabitants, suggesting considerable underdiagnosis and a need to increase awareness of these conditions in Morocco. The distribution of PIDs was different from that reported in Western countries, with a particularly high proportion of SCID, MHC II deficiencies, hyper-IgE syndrome and autosomal recessive agammaglobulinemia. However, we have now organized a national network, which should improve diagnosis rates in remote regions.

[The contribution of transfontanellar ultrasound in the early diagnosis of neonatal cerebral venous thrombosis: report on three cases]. / Apport de l'échographie transfontanellaire dans le diagnostic précoce des thromboses veineuses cérébrales néonatales : à propos de trois cas.

The clinical aspects of neonatal cerebral venous thrombosis are polymorphic in their mode of onset, making diagnosis difficult. Transfontanellar ultrasound allows for the morphological and vascular exploration of intracranial contents. We report three cases of neonatal cerebral venous thrombosis diagnosed early by transfontanellar ultrasound. The patients were treated with low-molecular-weight heparin, and the outcome was favorable in all three newborns. Transfontanellar ultrasound has a variable sensitivity for the detection of thrombus, but it remains the first-line examination before any additional secondary exploration. The early diagnosis of neonatal cerebral venous thrombosis with transfontanellar ultrasound requires the implementation of emergency treatment.

The Fate of Patients Who Started Hemodialysis during Childhood or Adolescence: Results of an Interregional Moroccan Survey.

Hemodialysis is the most used renal replacement therapy for children in Morocco. The objective of this study was to determine the prevalence of patients who started hemodialysis in childhood and study their characteristics and specificities of their care. For this we conducted a multicentric descriptive cross-sectional study of all chronic hemodialysis patients who started dialysis in pediatric age, in hemodialysis centers in four of the sixteen regions of Morocco. We collected 2066 patients undergoing dialysis in 39 hemodialysis centers; from these, only 72 patients (3.48%) started hemodialysis in childhood. The average age of patients was 20.64 ± 6.5 years with a sex ratio of 1.9. Duration of dialysis was 78.2 ± 56 months. The cause of end stage renal disease was urological abnormalities in 18% of cases and glomerulopathy in 12.5% of cases; however, it remains unknown in half of the patients. Over 18 years, 74% of patients are without profession, it is active in 13% of cases, and pursuing studies are only in 13% of cases. Patients under 5 years and those with a low weight are rarely taken care of in chronic hemodialysis with little individualization of prescription. Greater attention should be paid to renal transplantation that is desired by the majority of these patients (92%).

Repair of isolated double-chambered right ventricle.

The finding of a double-chambered right ventricle (DCRV) is exceptionally rare as an isolated anomaly. It is a congenital cardiac anomaly in which the right ventricle is separated into two chambers, a proximal high-pressure chamber and a distal low-pressure chamber, by anomalous muscles or fibrous tissues in the right ventricular cavity. We report the case of a 6-year-old infant who was admitted for growth retardation. The patient was diagnosed with an isolated DCRV without any other associated congenital anomalies. The patient underwent a successful cardiac surgical procedure of enlargement repair; he was discharged in good clinical condition with a normal cardiac function.

[Jaundice and urinary tract infection in neonates: simple coincidence or real consequence?]. / L'ictère au cours de l'infection urinaire chez le nouveau-né : simple coïncidence ou véritable conséquence ?

UNLABELLED: In neonates, jaundice may be one of the initial symptoms related to urinary tract infection (UTI). The routine testing of the urine in jaundiced neonates is controversial. This study aimed to evaluate the related factors of neonatal infants with the initial presentation of hyperbilirubinemia and the final diagnosis of UTI by evaluating data that help diagnose UTI early in apparently healthy newborns with jaundice. PATIENTS AND METHODS: We retrospectively investigated the medical records of neonates who had been admitted for management of jaundice (n=26) and compared with neonates with jaundice but without UTI (n=26). RESULTS: There was a significant difference between the two groups in male gender and maternal conditions (prolonged rupture of membranes, maternal UTI). There was also a significant difference between the two groups in their age at the time jaundice started (4 ± 3 days vs 2 ± 1 days) in the UTI and non-UTI groups, respectively (P>0.05). The cases in the UTI group had significantly lower total bilirubin levels (183 ± 71 mg/l) vs (227 ± 40 mg/l) in the non-UTI group, but a higher indirect bilirubin rate than the non-UTI group (P<0.05). Type B blood group was more common in neonates with UTI (P<0.01). In the cases presented herein, none of the jaundiced infants with UTI presented conjugated hyperbilirubinemia. Therefore, urinary tests for UTI should not be absolutely excluded or neglected in neonates in the early stage with unconjugated hyperbilirubinemia. Performing urinary tests to exclude the possibility of coincidental UTI may be necessary for admitted jaundiced infants younger than if they have a high level of indirect bilirubin, especially in male newborns with group B blood and in the presence of maternal urinary infection.