Infection by human herpes virus 8 (HHV-8) in childhood is common in the Mediterranean basin; however, classic Kaposi's sarcoma (KS) is exceedingly rare in children not infected with HIV and not receiving immunosuppression, with only 30 cases reported since 1960. We recently reported two children with autosomal and X-linked recessive primary immunodeficiencies underlying KS in a context of multiple clinical manifestations. These reports suggested that classic KS in otherwise healthy children might also result from inborn errors of immunity more specific to HHV-8. In this paper, we describe three unrelated Turkish children with classic KS born to first-cousin parents. The first patient, a girl, developed KS at two years of age with disseminated cutaneous and mucosal lesions. The clinical course progressed rapidly and the patient died within three months, despite treatment with vincristine. The other two children developed a milder form of KS at the age of nine years, with multiple cutaneous lesions. A boy treated with interferon alpha therapy for 12 months is now in full remission at age 14, two years after treatment. The second girl is currently stabilized with etoposide, which was begun four months ago. None of the three children had any relevant familial history or other clinical features. The occurrence of classic KS in three unrelated Turkish children, each born to consanguineous parents, strongly suggests that autosomal recessive predisposition may drive the rare occurrence of HHV-8-associated classic KS in children.
Brucellosis, although primarily a zoonotic infection, is also a threat for human health. Infection can be transmitted to humans through direct contact with infected animals, products of conception, or animal discharges, and through consumption of potentially infected milk, milk products, or meat. Human-to-human transmission is rare. There have been case reports of transmission via blood transfusion and bone marrow transplantation from infected donors. Sexual intercourse is a possible means of transmission. Neonatal infection can be acquired transplacentally or during delivery. This report describes a mother with brucellosis who probably transmitted the infection to her 3-month-old baby by breast milk.
Pancytopenia, although mainly reported in adults, has also been described in children with brucellosis. However, bone marrow hypoplasia is a rare feature of the infection. An 11-year-old boy was admitted with fever, vomiting, and abdominal pain of 10 days' duration. On physical examination, pallor and high fever were detected in the absence of lymphadenopathy and hepatosplenomegaly. His hemoglobin was 8.6 g/dL, white blood cell count 1,100/mm(3), neutrophil count 500/mm(3), platelets 56,000/mm(3), and reticulocytes 0.1%. Hypocellular bone marrow was found by aspiration, and bone marrow biopsy revealed hypocellularity. The agglutination titer was greater than 1/640. Trimethoprim/sulfamethoxazole was prescribed. His fever subsided and pancytopenia subsequently improved. Pancytopenia associated with brucellosis is attributed to hypersplenism, hemophagocytosis, and granulomatous lesions of the bone marrow, which is usually hypercellular. Bone marrow hypoplasia is rarely reported and should be kept in mind in the etiology of aplastic anemia in a country where brucellosis is frequently encountered.
PurposeDiagnosis of tuberculosis (TB) in children is more challenging than in adults. This study aimed to describe demographical, clinical and laboratory findings of children diagnosed with tuberculosis in Turkey, including the issues of contact tracing, culture positivity and forms of the disease.Materials and MethodsClinical and laboratory data of 51 children with a mean age of 8.0±4.6 years who were diagnosed with TB were retrospectively reviewed. Main diagnostic tools included tuberculin skin test, chest X-ray, sputum/gastric aspirate culture with sensitivity testing, and direct microscopy for acid-fast bacilli on available samples. Clinical characteristics and outcomes of the patients were examined.ResultsThirty-six (70.6%) children were diagnosed with intra-thoracic and 15 (29.4%) with extra-thoracic tuberculosis. Twenty-eight of the patients had a positive Bacillus Calmette-Guérin vaccine scar (28/51, 54.9%) and 23/51 (45.1%) had a positive tuberculin skin test. An adult TB contact was identified in 27 (52.9%) of the cases. On direct microscopy, acid-fast bacilli were found in nine (17.6%) patients and positive culture for Mycobacterium tuberculosis was found in 19 (37.3%). Drug resistance to isoniazid was detected in four (7.8%). One patient with nephrotic syndrome and miliary tuberculosis died during follow-up. All other patients responded well to the treatment.ConclusionFocusing on active contact tracing among all household contacts of tuberculous cases may be helpful in early identification and controlling childhood disease, even in regions with low disease prevalence. Adopting a suspicious and proactive approach in this particular age group is warranted.
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