[Multifocal tuberculosis in children: diagnostic traps]

Tuberculosis is a bacterial infection that occurs in endemic forms. This disease is still present in Morocco despite vaccination of 100%. Children, whether are immunocompetent or not are contaminated by positive adults-smear. The symptoms are often not too specific, which explains the severe forms. The authors report a case of multifocal tuberculosis. It concerns a year-old five-and-a-half- girl admitted for chronic iron deficiency anemia. The child has been followed-up for 5 months by iron deficiency anemia that unimproved the condition. No previous history of repeated infection, no tuberculosis contamination. The story of her disease began in April 2011 showing asthenia. Iron deficiency anemia required a treatment but without clinical or biological improvement after 5 months of treatment. One month prior to admission abdominal pain appeared, persistent fever and cough; all unimproved by the multiple prescribed outpatient antibiotic treatments. On admission, the child was pale, febrile at 38.5 with failure to thrive and signs of malnutrition; the abdomen was distended with HPSM. Pleuropulmonary examination showed discrete crackles [rales]. The lymph nodes are free. Apart from this long-term fever check-up showed microcytic hypochromic anemia [7.4g/l] and a biological inflammatory syndrome. According to physiological check-up, the research of BK in sputum X3 was negative, Tuberculin Skin Test [TST] and quantiferon test were negative. Abdominal ultrasound found a thickening of the colonic wall, with multiple infra-centimetric lymphadenopathies, a layer of intraperitoneal effusion, evoking thus lymphoma. The echocardiography showed an intra-pericardial effusion of non-compressive average size. Thoracic-abdominal-pelvic scan showed multiple nodules and micro-nodules of bronchiolar type and multiple necrotic mesenteric lymph nodes above and below the meso-colon, and a thickening of the ascending and transverse colons suggesting evolutive pulmonary and abdominal tuberculosis. Mini-laparotomy revealed an agglutination of loops and the presence of whitish granulations at the caecal region, with mesenteric lymph nodes. The anatomical-pathological study of the biopsies revealed peritoneal ileal appendicular the caecal region, with mesenteric lymph nodes. The anatomical-pathological study of the biopsies revealed peritoneal ileal appendicular caseo-follicular tuberculosis, with tuberculous lymphadenitis. The little girl has been put under antibuercular treatment following the severe multifocal tuberculosis scheme with a clear clinical and radiological improvement since D15 of treatment. Through this illustrative case, the authors stress the need to consider an iron deficiency anemia that is refractory to replacement therapy as a potential symptom of TB infection

[Ataxia reveals an inherrited deficiency of vitamin E]

Ataxia which is caused by deficiency of vitamin E is an inherited neurodegenerative disorder characterized by impaired ability of coordinate voluntary movements [ataxia] and rare peripheral neuropathy. The responsible gene is located on the long arm of chromosome 8. In the absence of replacement therapy, neurological signs worsen with loss of autonomy. The clinical picture is very similar to Freidreich's ataxia but there is a cure for deficiency of vitamin E. Ataxia. We report a case of a 14 years old patient of consanguineous parents hospitalized for chronic ataxia. The symptoms started 18 months earlier with a progressive gait disturbances. And balance and hand tremor, dysarthrie, without gastrointestinal symptoms. Clinical examination showed a thracolumbar scoliosis. And pes cavus, neurological examination noticed a kinetic ataxia, absence of tendon reflexes. There was no failure to thrive. The ophthalmologic examination was without abnormalities. The determination of vitamin E level in the blood was low up to 2 pmol/l [N: 16-53 pmol/l]. The child has a long term oral vitamin E treatment, and the evolution was marked after two years by an improvement of clinical signs, biological and radiological signs with stoppage of the disease progression. In any case of phenotype Freidreich chronic ataxia, we recommend performing a systematic determination of serum vitamin E level

[Niemann Pick type B disease]

Niemann Pick disease type B is a rare autosomal recessive lipid storage disorder, characterized by a partial deficiency of sphingomyelinase We report two cases one in a boy aged of 3 years old, and the other a 18-month-old girl. All of them have related parents. The circumstances of discovery were a hepato-splenomegaly without neurological signs and interstitial syndrome on chest radiograph. Laboratory tests showed a moderate hepatic cytolysis and microcytic hypochromic anemia. The bone marrow showed many cells overload Niemann-Pick type. Diagnostic confirmation was achieved by a decrease in cell lysosomal enzyme activity. It is a lysosomal disease characterized by visceral abnomality with preserved nervous system function. The current treatment of Niemann Pick disease is purely symptomatic awaiting the use of enzymatic replacement therapy

inflammatory pseudotumors of the colon

The inflammatory pseudotumors of the colon are very rare benign lesions, of unknown etiology and whose differential diagnosis is difficult with the adenocarcinoma. Young girl patient of 12 years old, presented with mass in the right iliac fossa, the preoperative assement was in favour of right colon malignancy with retro peritoneal extension. Right colectomy was done, histological examination confirmed the diagnosis of inflammatory pseudotumors of the colon. The inflammatory pseudotumors of the colon are benign tumor they can be the origin of local and general signs colonic localization is exceptional and etiology remains unknown. Surgery is necessary to obtain an histological diagnosis of the lesion and to eliminate malignant tumor