Kawasaki disease: About a case in Senegal

Kawasaki disease is an acute multisystemic vasculitis. We report the case of a 10-month-old boy received for a fever of 38.7° Celsius persisting on usual antipyretics for 10 days and bilateral conjunctivitis. The patient had bilateral serous rhinorrhea, polymorphic rash with diffuse perineal erythema, bilateral angular cheilitis, erythematous throat, plus perioral, periorbital and trunk desquamative lesions. There was microcytic hypochromic anemia at 9.5g/dL, thrombocytosis at 760x103/mm3, hyperleukocytosis at 28.27x103/mm3, positive C-reactive protein at 58 mg/L and an elevated sedimentation rate at 88 mm at the second hour. Aspartate amino transferase acid (AST) was elevated to 30 IU/L and alanine amino transferase acid (ALT) to 45 IU/L. Gamma glutamate transferase (γ-GT) was elevated at 488mg/dl. Cytobacteriological examination of the urine indicated aseptic pyuria. Cardiac Doppler ultrasonography showed low-grade pericarditis without coronary involvement. In the presence of prolonged fever > 5 days: bilateral conjunctivitis, oropharyngeal involvement, polymorphic rash, CRP >30mg/dL, VS >40mm/h, thrombocytosis, elevated transaminases and gamma GT, aseptic pyuria and pericarditis, we retained the diagnosis of Kawasaki disease in its incomplete form. The patient had been treated with acetylsalicylic acid 50mg/kg/24h. The evolution was favorable with stable apyrexia, regression of mucocutaneous lesions and normalization of cardiac ultrasound.

Infantile Cortical Hyperostosis: Report of a Case with Observations on Clinical Manifestations, Radiology, and Pathology with a Late Follow-Up of Eight Years

Purpose. The purpose of our study was to investigate clinical manifestations, roentgen images, histopathological studies, and evolution of the disease in patient displaying infantile cortical hyperostosis.Methods. Roentgenograms were made to evaluate a neonatal patient presenting multiple soft-tissue swellings. The initial radiographs insinuated that the disease had been present for some timein utero. Bone puncture biopsy of the tibia for histopathological observation and diagnosis conclusions was performed.Results. The disease was demonstrated radiographically by massive cortical diaphyseal thickening and also extensive periosteal new bone formation surrounding several bones. Results in blood count were as follows: discrete anemia, moderate leukocytosis, and elevated sedimentation rate. Histological pattern of tissue removed from tibia showed lamellar cortical bones and hyperplasia. Biopsy studies disclosed no evidence of neoplasia as well as of bacterial infection.Comments. Clinical manifestations in a neonatal patient displaying infantile cortical hyperostosis have gradually decreased. Radiograph findings have demonstrated complete recovery of bones manifested by the disease. The pathologic findings are in accordance with previous microscopic examination summarized by the literature. Total patient cure, without sequels, could be demonstrated.

Yersinia enterocoliticaInfection Simulating Lymphoproliferative Disease, after Liver Transplant

We describe a 14-year-old girl, who was 13 y after liver transplantation for biliary atresia with an unremarkable postoperative course. She presented with fever of up to 40°C, extreme fatigue, malaise, anorexia, and occasional vomiting. On physical examination the only finding was splenomegaly. Lab results showed hyperglobulinemia and an elevated sedimentation rate. Liver function tests were normal except for mild elevation ofγGTP. Abdominal U/S and CT demonstrated an enlarged spleen with retroperitoneal and mesenteric lymph nodes enlargement. An exhaustive evaluation for infectious causes, autoimmune conditions, and malignancy was negative. A full recovery after 5 months prompted testing for self-limited infectious etiologies.Yersinia enterocoliticainfection was diagnosed.

Juvenile Sjögren’s syndrome: Case report

Introduction. Sj?gren?s syndrome (SS) is an autoimmune disease of unknown etiology, clinically manifested by dry eyes (xerophthalmia) and dry mouth (xerostomia). In childhood SS is a rare disease, clinically atypically or asymptomatic and is often unrecognized. We report a girl with asymptomatic, juvenile form of primary Sj?gren?s syndrome (JSS). Case Outline. A 13?year?old girl was initially observed for several months due to elevated sedimentation rate (ESR 75?90 mm/h) without signs of inflammation or other symptoms and disease signs. Subjective symptoms of dryness of the eyes and mouth were absent at the beginning. Ophthalmologic examination demonstrated hypolacrimia although the patients had no subjective signs of xerophthalmia. Ultrasonography (US) revealed mild enlargement and heterogeneity of large salivary glands parenchyma. Increased rheumatoid factor (RF), anti SS?A/Ro, anti SS?B/La antibodies were found in serum. Ophthalmologic examination demonstrated decreased lacrimation. JSS was confirmed on the basis of ophthalmologic examination, immunological tests, histological findings of biopsy of small and US of major salivary glands. During a 12?years follow?up period systemic or extraglandular manifestations of JSS and other autoimmune diseases were not observed. Conclusion. Our experience suggests that in the differential diagnosis of unexplained elevated ESR the primary form of JSS should be also taken into consideration. Ultrasonographic changes of major salivary glands in the absence of symptoms of xerostomia point out that this noninvasive method has an important role in the diagnosis and management of patients with JSS.

Clinical characteristics of idiopathic ulcerative colitis in children

Introduction. Idiopathic ulcerative colitis (IUC) represents a rare disease of childhood. It usually occurs at age over 10 years, and below that exceptionally rarely. Objective. The aim of the paper was to analyze the clinical signs, symptoms and therapeutic procedures in children with IUC. Methods. The aims of the paper were based on a sample of 17 children (11 male and 6 female, mean age 11.90?3.50 years; range 3.8-17.5 years) with IUC. The disease diagnosis was based on characteristic endoscopic and pathohistological findings. Results. The basic signs of the disease involved chronic mucosal haemorrhagic diarrhoea which was confirmed in 16 of 17 patients, with body weight deficiency (10), recurrent abdominal pain (6), fever (5), slowed-down maturation (5), marked anorexia (4), and tenesmus (3). Two patients had recurrent aphthous stomatitis, 2 anal fissures, 2 arthralgia, one autoimmune hepatitis and one pyoderma gangrenosum. None of the children had longitudinal growth retardation. Elevated sedimentation rate and C-reactive protein in blood were registered in 11, sideropenia in 10, anaemia in 6 and hypoalbuminemia in 3 patients. The remission of proctosigmoiditis and left-sided colitis was achieved with aminosalicylates, and of pancolitis with the combination of aminosalicylates and glucocorticoids, except in cases of steroid-dependent colitis, which additionally required azathioprine. Conclusion. The main signs of IUC in children are chronic mucous haemorrhagic diarrhoea, body weight loss and sideropenic anaemia, while the basic therapy consists of aminosalicylates, and in more severe cases of the disease the initial use of glucocorticoids and later azathioprine.

LEFT ATRIAL MYXOMA IN A CHILD

A 9-year-old female presented with mitral regurgitation, heart failure, fever, abnormal serum proteins, elevated sedimentation rate, and elevated antihyaluronidase titer. At first thought to have rheumatic fever, she initially responded favorably to medical therapy which included corticosteroids. Evidence of progressive disease led to cardiac catheterization and angiography which demonstrated a left atrial myxoma which was successfully excised. Review of the literature reveals that 23 children with left atrial myxomas have been reported. These may present with embolic, obstructive, or constitutional symptoms. Most children have clinical evidence of mitral regurgitation. Associated heart failure, anemia, elevated sedimentation rate, elevated antihyaluronidase titer and initial favorable response to medical therapy often lead to the incorrect diagnosis of acute or chronic rheumatic heart disease. In these patients, increased endogenous hyaluronic acid excretion from tumor cells may lead to increased hyaluronidase and antihyaluronidase activity, abnormal serum proteins and elevated sedimentation rate.

TAKAYASU'S DISEASE (PRIMARY AORTITIS) IN CHILDHOOD

This is a case report of a 7-year-old child with the onset of the manifestations of Takayasu's disease (primary aortitis) at the age of 7 years. She exhibited a "prepulseless" stage with systemic symptoms of fever, fatigue, and weight loss. This early stage was also characterized by arthralgias and cardiac enlargement with laboratory findings of an elevated sedimentation rate and moderate leukocytosis. Within several months of the onset of the symptoms, she exhibited a "pulseless" or occlusive stage of the disease with absent arterial pulses of the arms and transient diminution of the carotid artery pulsations bilaterally. During this stage of her illness, she developed aneurysmal dilatation of the ascending aorta. Over a 3-year period she became asymptomatic with the disappearance of her anemia. Biplane angiocardiography demonstrated marked dilatation of the ascending aortic arch with associated discrete aneurysmal changes in the lower thoracic aorta. The patient recovered from her illness and remains asymptomatic 8 years following the onset of symptoms. A review of the literature is presented which emphasizes the rarity of this disease in children in this country. Since aortitis may involve the aorta at any level, it is suggested that the name "Takayasu's disease" be extended to include those cases with involvement of the thoracic and abdominal aorta in addition to those with the classical picture of aortic arch involvement and absent pulses in the upper extremities.