[Six cases of actinomycosis]

Actinomycosis is a rare granulomatosis infection caused by anaerobic bacteria; Actinomyces. There are many locations of actinomycosis and they can simulate an inflammatory or malignant process or pyogenic infection. We report 6 cases of actinomycosis. The diagnosis was confirmed by an anatomopathologic exam. The evolution was marked by an improvement after prolonged antibiotic treatment. This study shows the difficulties in the diagnosis of actinomycosis and the importance of systematic histopathological examination

[Persistent hyperinsulinemic hypoglycemia [HHI] of infancy: about 3 cases]

Hyperinsulinaemic hypoglycaemia [HHI] is the most frequent aetiology of recurrent severe hypoglycaemia in newborn and infant. This pathology arises the problem of early medical and surgical management to prevent brain damage. The aim of this work is to stress on the treatment emergency, to underline difficulties to distinguish damage. The aim of this work is to stress on the treatment emergency, to underline difficulties to distinguish between focal forms and diffuse forms, and to discuss our results with literature data. Our work is a retrospective study of 3 observations of symptomatic newborn HHI. Due to failure of glucose supplementation and medical treatment based on diazoxide and hydrocortisone, the 3 patients were operated between 50 and 107 days of life. Surgical exploration did not find any macroscopic pancreatic abnormality and intervention consisted of subtotal pancreatectomy [90-95 percent]. Anatomopathological study showed diffuse form in 2 cases and focal form in one case. After a follow up of 3 months to 2 years and a half the 3 patients have a normal glycaemia with no need to medical treatment. Yet 2 patients present brain damages .HHI is an emergency which needs a rapid medical management. Medical treatment failure should lead quickly to surgery to prevent neurological complications

Congenital cutis laxa: report of a case

Congenital cutis laxa is a rare hereditary heterogeneous group of disorder of elastic tissue. It is characterized by abnormally loose skin with a reduction in elastic tissue throughout the dermis. Depending on the mode of transmission and phenotypic expression, congenital cutis laxa has been classified in four types: autosomal dominant; autosomal recessive: types I and II and X-linked recessive. We report a new born girl with the autosomal recessive form of congenital cutis laxa associated with visceral abnormalities. The out come for this form was fatal. The most molecular basis for CCL were known but the therapeutic options are still limited

[Ileal prolapse through patent omphalomesenteric duct. A review of six cases]

Meal prolapse through the umbilicus is a severe complication of patent omphalomesenteric duct which exposes to the risk of intestinal necrosis in case of diagnostic or therapeutic delay. This is a retrospective study of six cases of ileal prolapse complicating a patent omphalomesenteric duct observed between 1986 and 2004 in the pediatric surgery department of Monastir. The mean age of the six patients was 67 days. The diagnosis was performed on the observation of an out-f low of intestinal liquid or stools through the umbilicus associated to an ileal prolapse. Five patients were treated by intestinal resection and anastomosis and the sixth had a wedge-shaped resection of the omphalomesenteric duct. One patient died from a severe sepsis. The evolution was favourable for the other patients. The ileal prolapse complicates 50 to 80 per cent of the umbilical enteric fistula. It exposes to the risk of occlusion and intestinal necrosis. So, the treatment must be urgent and consists on the resection of omphalomesenteric duct with ileal anastomosis.This treatment can be done by classic surgery or, more recently, by coelioscopy. The mortality can reach 33 per cent in the event of diagnostic or therapeutic delay. The ileal prolapse is a rare form of the patent omphalomesenteric duct which must be diagnosed and treated quickly to ovoid complications

[Obstructive anuria due to fungus balls. Report of 3 cases]

Fungus balls as a cause of upper urinary tract obstruction are rare, with less than 60 cases reported in the literature. We herein describe three cases or secondary anuria caused by Candida infection of the upper urinary tract. The first case was observed in a patient with a transplanted kidney, the second in a diabetic patient and the third in a. patient suffering from chronic Kidney failure. The treatment consisted of urinary drainage, identification of the infectious organism and appropriate antifungal treatment.

[Breast tuberculosis: study of 15 cases]

Mammary tuberculosis is a rare disease especially seen in underdeveloped countries. Describe the epidemiological, clinical parameters of mastitis tuberculosis and determinate the interest of imagery and histology in diagnosis. Fifteen cases of tuberculous mastitis diagnosed at the Infectious Diseases and Gynaecology departments of F. Bourguiba hospital during the period from January 1988 to March 2006. The mean age of our patients was 29.2 years [21 - 56 years]. History of tuberculosis was found in 33.3% of the cases. Sixty percent presented with fever and 73.4% complained of skin abscess. We noted symptoms of tuberculosis impregnation in 53.3% of cases. The fine needle aspiration achieved for all patients was negative in 14 cases and brought back the caesium in the other. The diagnosis has been made, for all our patients, after histological study when we found typical tuberculosis lesions on pieces of tumorectomy or biopsy. All patients had an anti-tuberculosis medical treatment. The median duration of treatment was 10 months [9 -18 months]. Although tuberculosis of breast is extremely rare, it should be kept in mind particularly in undeveloped countries. In front of symptoms evoking tuberculosis, biopsies must be done to eliminate an eventual cancer

[Lung abscess revealing a congenital cystic adenomatoid lung malformation]

Congenital cystic adenomatoid lung malformations [CCAM] are, todays, more frequently diagnosed during the pregnancy thanks to progresses in ultrasonographic explorations. On occasion, CCAM may remain asymptomatic and be discovered after being complicated by infection. This was the case of our patient. Amani _ was delivered by spontaneous vaginal delivery at term after a well-controlled pregnancy. She had good staturo-ponderal and psychomotor developments. When she was eight-year-old, she was treated for right pneumonia. Three years later, she was admitted in a general pediatrie unit because of right lower lobe abscess. Thoracic computed tomography, indicated since the infection was recurrent in the same lobe and after the constatation of a persistent cystic feature on chest radiograph, revealed multiple cystic lesions compatible with CCAM. The anatomopathological study of the surgically removed lobe concludes for a cystic adenomatoid lung malformation type I. Cystic adenomatoid lung malformations are frequently revealed by recurrent infections in old children. Thoracic computed tomography is very interesting for the diagnosis when pneumonia relapses in the same site

[Mature retrovesical cystic teratoma in the adult. Case report]

A teratoma is a tumor with components resembling normal derivatives of the three germ layers [ectodermal, endodermal and mesodermal]. In most cases it is located in the testis and in the sacrococcygeal region. Diagnosis mainly depends on ultrasonography and MR imaging and will be confirmed histologically. Treatment consists of surgery, aiming at complete excision of the tumor. The prognosis varies according to the age of the patient and the location of the tumor. We herein present a new case of a retro-vesical cystic teratoma in a 24-year-old patient and describe the classification, treatment and prognostic factors related to this type of tumor

[Partial cystectomy for primary neurosarcoma of the urinary bladder: a case report]

Genitor-urinary sarcomas are rare. Leiomyosarcomas and rhabdomyosarcomas are the histological types most frequently observed. The nervous origin of these sarcomas is exceptional. Early diagnosis and a complete resection of the tumor are necessary to improve the prognosis. We herein report the case of a primary neurosarcoma of the urinary bladder treated by partial cystectomy. At 9 years follow-up the patient is disease-free and has completed two pregnancies without any adverse events

[Atypical haemolytic uremic syndrome associated with steroid-resistant idiopathic nephrotic syndrome]

The association of atypical hemolytic uremic syndrome [HUS] with idiopathic nephrotic syndrome has been exceptionally described and its physiopathology is not yet understood

Observation: We report the observation of a four year and two month old boy descended of consanguineous parents which have since the age of three years a steroid-resistant idiopathic nephrotic syndrome with minimal glomerular lesions treated favorably by cyclosporine A during 12 months but whose ulterior evolution was marked by relapse of the disease and after a few months by the installation of an atypical hemolytic uremic syndrome and afterwards a terminal renal insufficiency

Conclusion: Idiopathic nephrotic syndrome may constitute the terrain of an atypical HUS. Its physiopathology, complex and not yet clarified, does probably incriminate an infectious factor and/or a genetic predisposition that remains to confirm

[Post infectious extracapillary glomerulonephritis: about 8 paediatric cases]

We reported 8 cases of post infectious extracapillary glomerulonephritis enrolled in the pediatrics department of Sousse during 10 years, diagnosed histologically. It is about 4 girls and 4 boys aged 3 at 13 years. Clinically, all patients had oedema and haematuria. Hypertension was present in 6 cases; 7 children were anuric. All patients have renal failure, associate at nephrotic syndrome in 6 cases. The ASLO titres are elevated in 7 patients. Renal biopsy showed diffuse crescents in 6 cases and circumferential cresents in 3. 7 patients well treated by corticoids and immunosupresseurs. The outcome is good in 4 cases

[Rectal duplications. Report of two cases and literature review]

Rectal duplications are very rare and pose sometimes diagnostic problems. We report 2 cases of rectal duplication among 32 alimentary tract duplications [6%] observed over 17 years period. The 2 patients were female and were hospitalized respectively at the age of 20 days and one month. In the first case the diagnosis was early in front of a mass prolapsed to the anus. In the second case, the diagnosis was posed at age the 6 months after iterative interventions for perished abscess and anal dents. The treatment consisted on a subtotal resection of the malformation in the first case and a lowering of the colon after iterative interventions for recidivant anal dents. Evolution was favorable in the two cases with a follow up of 4 and 10 year. Although it's rare, the diagnosis of rectal duplication must be evoked in front of any crowned pre sacral mass. Only an early diagnosis and treatment make it possible to prevent the complications and the later evolution towards the degeneration

[Out come of steroid resistant and steroid dependant nephrotic syndrome traeted by cyclosporine]

We study retrospectively 9 cases. We colliged 5 boys and 4 girls aged between 2 years 3 months and 13 years how have steroid resistant nephrotic syndrome in 6 cases and steroid dependant nephrotic syndrome in 3 cases.The renal biopsy show minimum lesion in 2 cases, segmental glomerular sclerosis in 6 cases, and proliferative glomerulonephritis in one. All this patients received cyclosporine.The out come is good in 5 cases: 4 enfants have complete remission, one have tow relapse steroide sensible. Two enfant were ciclodependant and need prolangation of the treatement and two were resistant

[Detection of EBV by PCR in fresh and paraffin-embedded naso pharyngeal carcinoma biopsies]

The nasopharyngeal carcinoma [NPC] is frequent in Tunisia. It's the second ORL cancer of men after the larynx one. To analyse the NPC characteristics in our population, we determined the frequency of EBV infection in 47 paraffin-embedded and 6 fresh NPC biopsies. We first extracted the DNA from tumoral tissus and then amplified viral sequences by PCR to detect and to type the infecting virus [EBV-A or ABV-B]. Our results showed that amplifiable DNA has been obtained from 34/47 paraffin-embedded NPC biopsies while 13/47 of the others biopsies contained degraded and not amplifiable DNA. All the fresh biopsies allowed to obtain DNA with good quality. The EBV infection frequency in paraffin-embedded NPC biopsies is 35% while EBV is detected in all fresh biopsies [6/6]. Our analyse also showed that the EBV-A is predominant in our population compared to EBV-B as it was shown in most countries of the world. This study clearly shows that PCR results obtained with paraffin-embedded NPC biopsies are divergeant from those obtained with fresh biopsies. Because of DNA degradation in paraffin-embedded NPC biopsies, the biology molecular results from that kind of samples is criticable. Moreover, the results obtained from fresh NPC biopsies confirmed the quasi-constant association of EBV with undifferenciated carcinoma nasopharyngeal type

[ abdominal cystic lymphangioma in childhood: a report of 12 cases]

Cystic abdominal lymphangioma is a rare malformative tumor, often discovered in childhood. The aim of this work is to study diagnostic and therapeutic aspects of this malformation. We report a retrospective study of 12 cases treated at the pediatric surgery department of Monastir over a 19-year period. The average age of the patient was 4 years. Diagnosis was suspected prenatally in only 1 case. Clinical signs have no specificity. Ultrasonography [11 cases] suggested the diagnosis in 10 cases. Computed tomography was performed in 4 cases. All patients underwent surgery. Lymphangioma was intraperitoneal in 11 cases and retroperitoneal in 1 case. Total resection was possible in 9 cases. Pathology was confirmed diagnosis in all cases. Postoperative outcome was uneventful for all patients. Because of no specificity of its signs, lymphangioma had many diagnostic difficulties. Ultrasonography constitutes the most important diagnostic imaging. The treatment is surgical and total resection is wished, which will be correlated with an excellent prognosis

[ malignant adrenocortical carcinoma of the child to a comment]

Malignant corticossurrenaloma is very rare in childhood. It occurs more often before the age of 5 years. Its pathogeny remains unknown but a genetic predisposition was suggested. The tumor is usually of secreting type and revealed by virilisation and/or Cushing syndrome, seldom by feminization and exceptionally by Conn syndrome. We report the case of a 7 years old girl who presented with a hetero-sexual precocious pseudo-puberty associated to a Cushing syndrome. The clinical examination found an abdominal mass. Hormonal investigation confirmed androgen and gluco-corticoid hypersecretion. Malignancy of the tumor was evoked by MRI. Evolution was marked by a tumoral rupture which required a laparotomy in emergency. The anatomo-pathological examination confirmed the diagnosis. In spite of a favorable immediate post operative evolution, the child died eight days later. Through this observation, we discuss the clinical, biological, therapeutic and evolutive aspects of this affection

[Acute post-streptococcal glomerulonephritis and pneumonia. About 6 cases]

Acute post-streptococcal glomerulonephritis [GNA] typically occurs 7-14 days after streptococcal infection, however some children can develop GNA concomitantly with bacterial pneumonia. ONA and pneumonia is a rare association, its often underestimated, its etiopathogenie remains still not clear. The purpose of this study is to analyse the etiopathogeny, clinical course and the outcome of this association. It is about 6 children hospitalized for GNA and pneumonia among 417 cases of GNA admitted between 1982 and 2003. All patients had at the time of admission abnormal urianalysis associated with respiratory manifestations. The evolution was favorable at 5 patients while the last one presented an empyema, nephrotic syndrome and died by pulmonary emboly. In conclusion the presence of a renal symptomatology at a child with pneumonia should be evaluated for concomitant GNA. This glomerulonephrite has generally a good prognosis, but sometimes specific therapeutic interventions are necessary to avoid the risk of renal failure