Pyoderma gangrenosum with pure red cell aplasia

Pyoderma gangrenosum is an inflammatory condition of the skin commonly associated with inflammatory bowel disease and rheumatoid arthritis, but also associated with various hematological malignancies. We describe its association with pure red cell aplasia in a four year old boy who presented with progressive skin lesions, fever and pallor, and improved with corticosteroid therapy.     

Fusobacterium osteomyelitis in a child with sickle cell disease

Children who have sickle cell disease are at increased risk for osteomyelitis caused by Salmonella spp. and Staphylococcus aureus. We report a case of anaerobic osteomyelitis caused by Fusobacterium nucleatum in a child with sickle cell disease. The infection did not resolve with antibiotic therapy alone, but was cured after surgical debridement and hyperbaric oxygen therapy.     

Hodgkin's disease in a child with sickle cell disease treated with hydroxyurea

Hydroxyurea (HU) is an oral drug that ameliorates the clinical course of sickle cell anemia by □increasing the levels of fetal hemoglobin and decreasing the adhesion of red cells to endothelium. Although HU has minimal short-term toxicity, few data are available about the long-term safety and the potential risk for carcinogenesis or leukemogenesis. An 8-year-old child with sickle cell / β0- thalassemia who received HU treatment for painful crises is described. Six months after the initiation of the HU treatment he developed Hodgkin's disease, lymphocyte predominance subtype. Chemotherapy induced a complete remission. After discontinuation of chemotherapy the painful crises recurred and bone marrow transplantation was decided at the age of 12 years. Two years after the bone marrow transplantation, the child is in complete remission without painful crises. Although the authors suggest that the development of Hodgkin's disease is a coexisting event, questions arise about the safety of HU treatment in childhood.     

Symptomatic pulmonary hypertension in a child with sickle cell disease

Pulmonary hypertension is a survival-limiting complication in adults with homozygous sickle cell disease (SS), but little is known about the development, course, or best treatment of pulmonary hypertension in children with SS. We report the clinical course and treatment of a child with SS-associated, hemolysis-driven pulmonary hypertension.     

Pyoderma gangrenosum in a six-month-old boy

Pyoderma gangrenosum (PG) is an uncommon, chronic ulcerative condition of the skin that was first described in 1930. It can occur in any age group, but only 4% of the patients are infants or children. An underlying systemic disease is present in approximately 50% of the patients with PG. The most common associations include inflammatory bowel disease, arthritis, lymphoproliferative disorders and chronic recurrent multifocal osteomyelitis (CRMO). PG has been reported in association with CRMO in only a few children whose ages were between 18 months and 12 years. We report a six-month-old boy who was diagnosed as CRMO based on his clinical examination and histological findings. This is the youngest case reported in the literature (under 12 months of age) with PG associated with CRMO.     

Gastric trichobezoar: abdominal mass in a child with sickle cell disease

Abdominal pain is a frequent occurrence among the pediatric population and can be a diagnostic challenge. Trichobezoar is a differential diagnosis that is often neglected. Different from previously reported cases, we present a 3-year-old girl with sickle cell disease with complaints of acute abdominal pain, suspecting sickle cell splenic sequestration. The child presented to the emergency department with sharp epigastric pain and an associated palpable upper abdominal mass. This case illustrates a large obstructing gastric trichobezoar and summarizes both the diagnostic modalities and treatment.     

Enuresis in children with sickle cell disease]

POPULATIONS AND METHODS: In order to analyze epidemiological and clinical characteristics of enuresis in children with sickle cell disease, and identify risk and aetiological factors, 987 subjects (aged 5-20 years) took part in a cross-sectional study. These children were divided into two groups: group 1 consisted of 456 homozygous children with sickle cell disease (haemoglobin SS), while group 2 consisted of 531 normal children. These groups were paired according to age and sex. RESULTS: The prevalence of enuresis was 50.9% in group 1 and 16.4% in group 2, and was significantly higher (P < 0.05) in the girls. The frequency decreased significantly between the ages of 5-20. At age 16, frequency of enuretic children with sickle cell disease remained statistically greater (15.9% vs. 8.0%). The frequency of wet nights was higher (P < 0.05) in group 1. In addition, anaemia crisis and painful crisis were related to prevalence of enuresis. There was a significant (P < 0.01) negative correlation between haemoglobin F percentage and prevalence of enuresis. CONCLUSION: Enuresis is frequent in children with sickle cell disease and its intensity is linked to severity of disease.     

Acute osteomyelitis in the child with sickle cell disease in a tropical zone: value of oral fluoroquinolones]

AIM: This study was designed to assess the efficacy and the safety of fluoroquinolones in their compassionate use for acute osteomyelitis in children with sickle cell disease in a tropical country. PATIENTS AND METHODS: This study was non comparative, including twelve children (eight SS, three SC and one SEzerothalassemia) treated for acute osteomyelitis with oral ciprofloxacin or ofloxacin because of the following reasons: financial inability to afford conventional parenteral beta-lactams therapy (nine patients), refusal of hospitalization (two patients), and failure of conventional treatment (one patient). RESULTS: The mean age of patients was 9.5 +/- 2.6 years. The long bones were the predominantly site. Salmonella species were present in 75% of cases, followed by other enterobacteriaceae (16.7%), and Staphylococcus aureus (8.3%). Successful outcome occurred in all cases after three to four-weeks of treatment and 45 days of plaster immobilization. Transient bilateral Achilles tendon tendinitis was noted in a five-year-old patient. CONCLUSION: In economically developing countries, oral fluoroquinolones may be a therapeutic alternative for acute osteomyelitis in patients with sickle cell disease particularly in cases of financial hardship or failure with conventional therapy.     

Hodgkin lymphoma in a child with sickle cell anemia

There are reports of patients with sickle cell disease who developed hematological malignancies but the relationship between these malignancies and sickle cell disease (SCD) is not yet defined. The co-existence of a hematological malignancy with SCD poses certain challenges for the management of each condition. We describe a 7-year-old boy with sickle cell anemia who developed Hodgkin's lymphoma and the challenges of management. He presented with a 4-year history of bilateral neck swelling and a 2-month history of weight loss and high-grade fever. Histology of a lymph node biopsy was consistent with mixed cellularity Hodgkin's lymphoma. He was treated with five cycles of Cyclophosphamide, Vincristine, Procarbazine and Prednisolone (COPP) and had complete clinical response. Chemotherapy was associated with an increase in frequency of painful crises and complicated by septicaemia. Blood transfusion needs were minimal; apart from the transfusion preceding the first cycle of chemotherapy, there was no need for further transfusion. Myelosuppression was not a problem in the patient; he responded well to antibiotics during the two episodes of septicemia without the use of hemopoetic growth factor. Patients with sickle cell anaemia who develop Hodgkin's lymphoma can be successfully treated with chemotherapy along with supportive management for crises and infections.     

Labyrinthitis ossificans in a child with sickle cell disease: CT and MRI findings

The association between sensorineural hearing loss and sickle cell disease has been described, and labyrinthine hemorrhage has been reported with sickle cell disease. We report the CT and MRI findings of labyrinthitis ossificans in a child with sickle cell disease who presented with sensorineural hearing loss. Labyrinthitis ossificans is associated with an infectious, inflammatory, or destructive insult to the membranous labyrinth; however, it has not been specifically described with sickle cell disease. Recognition of this condition is important because it affects both management and prognosis of this disease.     

Pyoderma gangrenosum, a nonintestinal manifestation of Lesniowski-Crohn's disease in a 15 years old girl

In the clinical course of Lesniowski-Crohn's disease in children, gastrointestinal symptoms are predominant. Extraintestinal manifestations are found rarely, contrary to adults. Pyoderma gangrenosum is hardly ever observed but frequently coexists with colon involvement. The aim of the study was a presentation of 15 years old girl with Lesniowski-Crohn's disease and predominant joint and skin symptoms in form of pyoderma gangrenosum. Skin lesions were shaped in solitary ulcerations with cyanotic undermined border localized on legs and buttocks. After steroid therapy these changes were healed with permanent blue scar. Gastrointestinal symptoms though slight were characteristic. The girl had stools with some fresh blood and perianal lesions.     

Echocardiographic aspects in pediatric patients with sickle cell disease]

BACKGROUND: Cardiovascular involvement is not well studied in children with sickle cell disease. The aim of this study was to evaluate the echocardiographic parameters of children with sickle cell disease. PATIENTS AND METHODS: We performed a transversal and case-control study including 80 subjects of six months to 16 years of age divided into four groups of 20 children each: heterozygous sickle cell disease, homozygous sickle cell anemia, anemia of other causes than sickle cell, and healthy children. All children had a complete physical examination, biological screening including hemogram and hemoglobin electrophoresis, chest x-ray, electrocardiogram, and Doppler echocardiogram. Data were compared using the chi 2 method and the Student's t-test. RESULTS: The mean age was 8.5 years and the sex-ratio was 1. Echocardiographic abnormalities were observed in seven patients with anemia unrelated to sickle cell, 15 children with heterozygous anemia and all the homozygous patients. The main abnormalities were: left ventricular enlargement (ten homozygous patients, one heterozygous subject, five of the patients with another cause of anemia), increased contractility of the heart (18 homozygous patients, seven heterozygous patients, five in the anemia group) and mild to moderate mitral or tricuspid regurgitation (12 homozygous, five heterozygous and five patients in the anemia group). One homozygous child had a dilated and hypokinetic cardiomyopathy with pulmonary hypertension. The parameters of left ventricular systolic function and left heart chamber dimensions were lower in the control group (P < 0.04). DISCUSSION: This study shows the frequency of heart chamber dilatation with left ventricular hyperkinesis usually described in the literature. The lack of case of chronic cor pulmonale may be due to the young age of our patients. One case of dilated and hypokinetic cardiomyopathy suggests that other causes than anemia should be considered, particularly myocardial ischemia, which could not be demonstrated because of our limited investigative methods. CONCLUSION: These results emphasize the frequency of the heart involvement in sickle cell disease, particularly in the homozygous type, and point out the importance of the cardiologic screening of these patients.