Currarino's triad: anorectal malformation, sacral anomaly and presacral mass

We report the case of a 3 year old boy with a combination of anorectal malformation, sacral agenesia and anterior meningocele (Currarino's triad) and provide a review of the literature of this rare syndrome.     

High HbA1c levels affect motility parameters and overexpress oxidative stress of human mature spermatozoa

The aim of this study is to investigate, by a validated in vitro model, the effect of diabetic plasma on ejaculated human spermatozoa. Plasma of 51 male diabetic patients (mean age 62.28 ± 9.28 years) was selected according to their HbA_1c level: low HBA_1c ≤ 5% (31 mmol/mol), moderate HBA_1c 6%–8% (42–64 mmol/mol) and high HBA_1c ≥ 10% (86 mmol/mol). The plasma was tested on eighteen normal semen samples by analysing gametes motility using a computer Sperm Class Analyzer® and their corresponding oxidative stress (OS) status using thiobarbituric acid-reactive substances assay. The results indicated that diabetic plasma affected all sperm motility parameters with high HbA_1c showing the most important deleterious effects. Low gametes' straight-line velocity was observed in high HbA_1c level, mainly after 20 min of co-incubation (8.78 ± 0.47 µm/s). Also, the highest lipid peroxidation (nmoles MDA/10⁸ SPZ) was observed in high HbA_1c values (0.92 ± 0.09), higher than those in spermatozoa treated with H₂O₂ (0.85 ± 0.04). Conclusively, a direct impact of diabetic plasma on spermatozoa is revealed with overexpression of OS as the underlying mechanism. These findings suggested that it is strongly recommended to control clinically the glycaemic level and OS in diabetic patients for the maintenance of male fertility.     

Thrombose portale après splénectomie chez l’enfant : à propos de 4 observations

Acute appendicitis can cause of portal vein thrombosis. Diagnosis of appendicitis can be difficult because its symptoms may be masked by those of acute portal vein thrombosis. We report 2 cases of appendicitis associated with acute portal vein thrombosis whose diagnosis was delayed by several months. The delayed diagnosis of acute appendicitis in the presence of acute portal vein thrombosis can be avoided by using spiral-computed tomography and careful investigation of signs of appendicitis.

Radiological analysis of hyaline membrane disease after exogenous surfactant treatment

Background. Exogenous surfactant treatment of hyaline membrane disease is known to modify the pattern of radiological changes on the chest radiograph. Objectives. To analyse and attempt to explain the radiological changes observed after exogenous surfactant treatment. Materials and methods. Thirty-nine premature infants with typical hyaline membrane disease. Results. Transient asymmetrical clearing with better aeration of the right lung in the absence of malposition of the tip of the endotracheal tube was observed in nine cases (23 %). This asymmetry was patchy in one case. It was due to a complication of mechanical ventilation in three cases [pneumothorax (n = 2) and pneumomediastinum (n = 1)]. In the other six cases, asymmetrical clearing could be related to the anatomical position of the right main bronchus, which facilitates distribution of surfactant to the right lung. However, the course of these premature infants was similar to that of infants with symmetrical chest radiological findings after treatment. Conclusions. Asymmetrical clearing of chest radiographs, sometimes patchy, after surfactant treatment requires exclusion of pneumothorax or infection but has no influence on clinical outcome. Received: 30 December 1997 Accepted: 15 June 1998     

Differential toxic effects of azathioprine, 6-mercaptopurine and 6-thioguanine on human hepatocytes

Thiopurines (azathioprine, 6-mercaptopurine and 6-thioguanine) are therapeutic compounds widely administered in the clinic for their multiple uses (autoimmune diseases, post-transplant immunosuppression and cancer). Despite these advantages, their therapeutic potential is limited by occasional adverse effects (myelotoxicity and hepatotoxicity) and by a relatively frequent lack of efficacy. Previous studies have demonstrated that azathioprine decreased the viability of rat hepatocytes. In order to investigate cytotoxic effects of thiopurines in human liver, we used primary human hepatocytes and a highly differentiated human hepatoma cell line, HepaRG, treated or not with azathioprine, 6-mercaptopurine and 6-thioguanine. In parallel, expression of the genes involved in the metabolism of thiopurines, glutathione synthesis and antioxidant defences was measured by quantitative PCR. We clearly demonstrate that human liver parenchymal cells were much less sensitive than rat hepatocytes to thiopurine treatments. The toxic effects appeared after 96 h of treatment while ATP depletion was observed after a 24 h incubation with azathioprine and 6-mercaptopurine. Toxic effects were more pronounced for azathioprine and 6-mercaptopurine, when compared to 6-thioguanine, and might explain glutathione synthesis and antioxidant enzyme induction only by these two drugs. Finally, we also demonstrate for the first time an up-regulation by azathioprine and 6-mercaptopurine of inosine monophosphate dehydrogenase which might have consequences on the de novo biosynthesis of guanine nucleotides and thiopurines metabolism.     

Primary hydatid cyst of the thigh: on seven cases

We report on seven cases that presented a benign soft-tissue mass in the thigh muscles. Ultrasonography provided the diagnosis of hydatid cyst. Total pericystecomy was performed. We point out the importance of preoperative diagnosis and the need for radical excision to avoid recurrence.     

Osteitis of the Fourth Metatarsal Caused by a Date Palm Thorn in a Child: Why the Dorsum of the Foot Is the Most Commonly Injured Site

The palms are frequent in the region of eastern Morocco. An insidious onset of a lytic lesion in the base of the fourth metatarsal caused by a date palm thorn in a 20-year-old patient is presented. An untreated embedded thorn can cause late complications, including periostitis or osteomyelitis. In most cases, removal of the foreign body is easy, and no surgical care is needed. If detected, these injuries can be treated without complications. In children, however, the diagnosis can be very easily missed, especially if the child has aphasia and deafness, which was present in our patient. These can influence communication between the child and family, such that the patient is unable to inform the family about what object pricked him (e.g., date palm thorn, toothpick, insect) and is unable to tell the family that the foreign object remains embedded. This major communication issue between the child and family can result in potentially avoidable complications such as osteitis. No other issues were present in our patient that could have caused a delay in diagnosis, such as fear of punishment or fear of medical treatment. We present a case of date palm thorn-induced periostitis of the base of the fourth metatarsal. Surgical exploration revealed a 2.5-cm palm tree thorn embedded in granulation tissue, forming an abscess. The lesion was successfully treated by curettage of the lesion and removal of the thorn embedded in the periosteum of the metatarsal base. The purpose of the present report was to explain the mechanism of this rare entity and the frequency of this lesion on the dorsal aspect of the foot rather than on the plantar. Despite the absence of a definite history of trauma, organic foreign material should be in the differential diagnosis of a lytic lesion of the bone, especially in patients who have a problem with deafness or aphasia.     

Influence of steatosis on progression of fibrosis and virological response in chronic hepatitis C cases

Background and study aimsHepatic steatosis seems to be frequently found histopathologically in chronic hepatitis C virus (HCV)-infected patients. The aim of this study is to determine the influence of steatosis on HCV disease severity (fibrosis) and to evaluate its impact on sustained virological response (SVR) to antiviral therapy.Patients and methodsFrom April 2008 to April 2010, 148 consecutive adults (87 females (59%) and 61 males (41%); mean age: 55.2 years) with HCV admitted for liver biopsy were included in this retrospective study. At least one element of metabolic syndrome was identified in all cases: Obesity (n = 44), hyperlipidaemia (n = 40), hypertension (n = 29) and diabetes (n = 21). Liver fibrosis was classified according to the Metavir score and hepatic steatosis described as following: S0: absent; S1: minimal (<30%); S2: moderate (30–60%); and S3: severe (>60%). Patients were divided into two groups: S0S1 group (absent or minimal steatosis) and S2S3 group (moderate to severe steatosis). Of the 148 patients, 53 were treated with pegylated interferon and ribavirin combination therapy.ResultsSteatosis was found in 40 patients (27%): S1 in 72.5%, S2 in 17.5% and S3 in 10% of cases. The distribution of patients according to the degree of fibrosis was as follows: in the S0S1 group, F1 = 12.4%, F2 = 36.5%, F3 = 21.1% and F4 = 21.1% and in the S2S3 group, F1 = 9%, F2 = 45.5%, F3 = 18.2% and F4 = 27.3%. There was no difference between the two groups regarding the degree of fibrosis (p ? 0.80). The rate of SVR was 64%: 63% in the S0S1 group and 75% in the S2S3 group. The difference was not statistically significant (p = 1).ConclusionSteatosis was found in 25% of cases. Liver steatosis in chronic hepatitis C is not a negative prognostic factor of response to combined antiviral therapy. These results must be confirmed by a large series of patients.