[Cerebral venous thrombosis complicating a severe dehydration in the infant about a case report]

Cerebral venous thrombosis [CVT] is a rare vascular pathology in the infant characterized by a clinical and etiologic diversity. We report the case of CVT secondary to a severe deshydratation. It is about a one month old infant without notable pathological antecedents which presented 5 days before its hospitalization a refusal of feeding, a disorder of behavior and somnolence. The initial assessment showed a urinary infection with negative gram bacillus and a functional renal insufficiency. In front of the non improvement under treatment and the aggravation of the renal insufficiency, the patient was transferred to our formation. At admission the examination found an apyretic, icteric and hypertonic infant with dehydration of more than 15%and a convex anterior fontanella. Transfontanellar echography showed signs of cerebral haemorrhage and the CT-scan revealed a wide cerebral thrombosis. In spite of the rehydration and the antibioc, the patient deceased following a bradycardia with haemodynamic instability non responding to vaso-active drugs. The TVC is an unusual complication of deshydration in the infant. Through this observation the authors discuss the mechanisms of the TVC

[Peritoneal tuberculosis in children about a 42 cases]

Peritoneal tuberculosis is a common affection in the developing countries. Its frequency in the industrial countries is substantially increasing mainly due to AIDS pandemiy. In Morocco it occupies the third position after pulmonary tuberculosis and gangional tuberculosis. It represents 75%of abdominal localisations. In this study, we report 42 cases that were hospitalized at the pediatrics department at the Hassan II University Hospital of Fez, Morocco, Our patients, 22 boys and 20 girls, were aged between 1.5 and 14 years old with an average age of 7.7 years. They were vaccinated with BCG in 97%of cases. Twelve cases were due to contagion. The clinical profile was dominated by ascitis, abdominal pain sometimes with resemblance to surgical pathologies and acute fever. Association with other tuberculosis affections, mainly pleural tuberculosis, was observed in 17 cases. In 26 kids diagnosis was confirmed with histology. The scheme and the duration of anti-bacilli treatment varied depending on cases and the coarse was good in general. Based on these observations we can con that the incidence of peritoneal tuberculosis is still very high in Morocco, since the frequency of contagion is very important the screening remains obligatory, the delay of diagnosis is due to the clinical polymorphism and to the lack of bacteriological evidence

[Ecthyma gangrenosum in the infant about 3 cases]

Ecthyma gangrenosum [EG] is one of the most dangerous bacterial cutaneous infections in the infant. The Pseudomonas Aeruginosa is frequently responsible. The skin localization is often associated with a bacteriemia, rares cases of primitive cutaneous localisation were described. The evolution is often fatal in case of immunodeficiency situation. The authors report 3 observations of ecthyma gangrenosum 2 months, 7 months and 18 months old. They all presented localised cutaneous erythema followed one week later by oedematous plates. These lesions evolved to necrosis and ulceration. The fever was absent in all patients and only one had a deteriorated general state. A high lymphocyte count was found in all patients. The inflammatory syndrome was inconstant. There were not any other localizations of the infection. The 3 patients were treated with cephalosporin of 3rd generation, associated in 2 cases with the gentamycin. The evolution was favourable. The early recognition of the lesions of EG and the institution of an adequate treatment often allows a favourable result in this affection

[ acute liver failure [ALF] in children - about 55 cases]

The acute liver failure [ALF] in children has multiple etiological factors.. In our context, the infectious, especially viruses, are in the first rank. In this study, the authors report 35 cases of ALF, diagnosed and treated in our unit, between January the 1st 2003 to february 28[th], 2006 for analyzing the clinical, biochemical characteristics, the evolution as well as the treatment. The age of the patients varies between 2 months to 14 years old. Clinically, the jaundice was noted at 57% of the patients, are bleeding 28%, a hepatomegaly 41% with cirrhotic once at 17% and a generalized edema at 34%. A hepatic encephalopathy was noted in 11% of the patients. On the biological parameters: the rate of prothrombin [RP] was lower than 50% in 82% of cases, the hepatic cytolysis was noted in 88% of cases, the hypoalbuminemia in 40% and low blood glucose in 43% of the patients. Concerning etiologies, the infectious origin accounts for 45%, followed by Wilson disease with a rate of 19%. The treatment is in all cases symptomatic; it was etiologie each time that a cause was identified. The death of 3 patients is deplored. The infectious origin remains the dominant cause in our department, represented primarily by hepatitis A and the leishmaniosis, followed by the Wilson's disease. The high mortality must encourage rapid evaluation and selection of the patients candidates to liver transplant

[ prune belly syndrome - about 2 cases]

The Prune Belly syndrome, or aplasia of the abdominal wall muscles is a rare syndrome. It occurs in 1/40.000 children and almost exclusively in boys. It is characterized by the association of an absence of the abdominal wall musculature, a cryptorchidism and severe anomalies of the urinary tract. The treatment of this syndrome is difficult. We bring back two typical observations. It is about two newborns, the first was admitted at 8 hours of life and the second at 11 days of life for an abdominal distension with a total haematuria without anuria. The clinical examination found a spread and expansive stomach, a crumbled cutaneous coating and two voussures in the flanks, a bilateral cryptorchidism and an overflowing miction. The first patient had a minimal renal insufficiency and a low prothrombine time. In the second patient the biologic balance was normal and the UBCE was negative. The abdominal ultrasonography showed a bilateral hydronephrosis. The IVU showed an important hydronephrosis with the presence in the first case of a bilateral anomaly of the uretero-pelvic junction. The UCG in the second patient revealed a bilateral vesicoureteral reflux. The Prune Belly syndrome was evoked. We decided not to operate the patients and to supervise the renal function and to maintain the urines sterile. The first patient received K1 vitamin. The evolution was marked by the disappearance of the haematuria. The first patient died at 40 days of life, the second didn't return to consultation. The interest of these observations is to recall the clinical, radiological and evolutionary aspects of this syndrome and to raise the importance of the antenatal diagnosis

Ritscher-Schinzel syndrome: a case report

The 3C syndrome [cranio-cerebello-cardiac], or Ritscher-Schinzel syndrome, is rare. Only about thirty cases have been described in the literature. Transmitted on autosomic recessive mode, it associates a characteristic cranio-facial dimorphism, a cerebella vermis hypoplasia, cardiac malformations, a severe psychomotor retardation, and others anomalies such as ocular and renal. Authors report a new typical case characterized by its clinical symptoms. A four year-old child, from first degree consanguineous parents, is hospitalized for cyanosis with respiratory distress. The clinical examination finds a facial dimorph, a total hypotonia, a poor growth, a psychomotor retardation, a bilateral cryptorchidy and a camptodactyly of the 5th finger. The echocardiography shows a Fallot tetralogy in its complete form and the cerebral scanner shows Dandy-Walker lesions and hydrocephaly. The treatment was symptomatic. The Ritscher-Schinzel syndrome is a rare and serious disease. The postnatal treatment is difficult. The prognosis is often reserved. The gene identification would allow antenatal diagnosis, eventual pregnancy interruption and genetic advice

Qsteite tuberculeuse [a'propos d'une forme particulie're de l'enfant: spina ventosaa]

Bone tuberculosis is an uncommon disease. Its evolution is chronic. We report one case in a 16 month old child who has a particular form: spina ventosa, which lies on the small bones of hands and feet. It makes a gap in bone's centre and widens it as a spindle. We propose to analyze the symptoms and X-Ray of this disease, and stress bone biopsy and bacteriologic research of all bone's ragging lesion