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Henoch Schönlein purpura (HSP) with acute rheumatic fever (ARF) is a rare entity and only few cases have been reported so far. In all previously reported cases with HSP and ARF, patients initially presented with purpuric rash, arthralgia/arthritis, or abdominal pain and later diagnosed as ARF. We report an 11-year-old girl with features of both ARF and HSP. She initially presented with arthralgia and murmur. Echocardiography showed mild to moderate mitral regurgitation. Later, the clinical course was complicated by purpuric rash and abdominal pain. She was treated conservatively with IM penicillin, acetylsalicylic acid and oral prednisolone. Our patient is the first patient with HSP and ARF who initially presented with features of ARF. A review of literature revealed a limited number of cases of HSP associated with ARF (14 cases including the present case); and that the response to treatment in cases suffering from ARF associated with HSP was good; but one should also be aware of serious cardiac complications in HSP patients which may be fatal.

Streptococcus pneumoniae is a common pathogen responsible for pulmonary infections and the leading cause of mortality and morbidity in patients with particularly B cell immunodeficiencies. Antibody production is the principal protective immune response against S. pneumoniae and measurement of the production of antipolysaccharide antibodies is important in the evaluation of B cell deficiencies. We quantified serotype-specific immunoglobulin G antibodies against seven common pneumococcal serotypes before and three weeks after unconjugated vaccine in 416 patients with recurrent respiratory tract infections; fifty-five (13%) of whom showed impaired antibody response. We could evaluate 41 of these 55 patients for their particular clinical features. Specific antibody deficiency, was diagnosed in 10 of these patients, common variable immunodeficiency in 18, ataxia telangiectasia in 10 and other antibody deficiencies in 7 (transient hypogammaglobulinemia in 4, IgG subclass deficiency in 1, partial and selective IgA deficiency in 1) patients. Evaluation of the antibody response to polysaccharide antigens should be considered early on in patients with recurrent respiratory infections and required particularly for the diagnosis of specific antibody deficiency and the decision of the appropriate treatment approaches.

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Background: Dietary copper restriction in Wilson’s disease is recommended mostly for 1 year or until showing normal liver enzymes. Little is known about the effect of long-term copper restriction on copper and nutritional status in the body. The relationship between daily copper consumption and serum and urine copper parameters, liver enzymes, and dietary contents was investigated. Methods: In this study, 32 pediatric Wilson’s disease patients who had been on treatment at least for 12 months were included. Clinical features, liver enzymes, serum total copper concentrations, non-ceruloplasmin bound copper concentrations, adjusted copper concentrations, 24-hour urine copper excretions, and macro- and micronutrient consumptions were analyzed. Results: In total, 27 patients reported following copper-restricted diets, while daily copper consumption was low only in 7 patients (21.9%). Total copper concentrations and non-ceruloplasmin-bound copper concentrations were low at 78.1% and 53.1%, respectively. All but one adjusted copper concentration were within normal limits. Total copper concentrations, adjusted copper concentration, and non-ceruloplasmin-bound copper concentrations correlated with each other but none correlated with urine copper excretions. Daily copper consumption was inversely correlated with total copper concentrations ( P = .041, r = –0.363) but not correlated with non-ceruloplasmin-bound copper concentrations and adjusted copper concentrations. There was no relationship between liver enzymes and daily copper consumption and serum and urine copper parameters. High fat consumption with low fiber and vitamin B6 was more common in low daily copper consumption group ( P = .033, P = .029, P = .007, respectively). Conclusions: Daily copper consumption may be the least effective or non-effective factor on liver enzymes in Wilson’s disease. Prolonged copper restriction may result in unintentional dietary imbalance. Avoidance of undernutrition and high-fat meals, as well as enrichment of the meals with vitamin B6 and fiber, should be encouraged during copper-restricted diets.

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Giriş: Endoskopik retrograde kolanjiopankreatografi (ERCP) endoskopi ile fluoroskopiyi birlikte kullanan pankreas ve safra yolları hastalıklarında tanı ve tedavi imkanı sağlayan işlemdir. Gereç ve Yöntem: Hacettepe Üniversitesi Tıp Fakültesi Gastroenteroloji Endoskopi Ünitesinde Kasım 2017 -Nisan 2020'de ERCP yapılan 46 çocuk hastanın verileri retrospektif incelendi. Bulgular: Yirmi üç hastada biliyer, 23'ünde pankreatik endikasyonla olmak üzere 46 hastaya (29 K, %63,04) toplam 77 (1-5) ERCP işlemi yapıldı. Yaş ortalaması 10,3±4,07 (2,08 -17) yıldı. En küçük hasta 12,6 kg idi. ERCP endikasyonları; 16 hastada koledok taşı şüphesi (%34,8), 9 hastada kronik pankreatit (KP) ve ağrı (%19,6), 8 hastada rekürren akut pankreatit atakları (RAP, %8,7), 4 hastada KP ve RAP (%8,7), 3 hastada karaciğer nakli ve kolestaz (%6,5), 2 hastada benign biliyer darlık (BBD) (%4,3) ve 1 hastada sarılık nedeniyle hastalık ekartasyonu için (%2,2), 1 hastada profilaktik pankreatik stent uygulanması (%2,2), 1 hastada safra yolu ile ilişkili kist hidatik (%2,2) ve 1 hastada pankreas fistülü (%2,2) idi. ERCP'de, 14 (%30,4) KP, 7 (%15.2) koledok kisti, 11 (%23,9) koledok taşı, 4 (%8,7) Oddi sfinkter disfonksiyonu, 3 (%6,5) BBD, 3 (%6,5) anastomoz darlığı, 1 (%2,2) pankreas fistülü, 1 (%2,2) safra yolu ile ilişkili kist hidatik tanıları konuldu. Hastaların 6'sında (%13) anormal pankreatikobiliyer bileşke ve 5'inde (%10.9) pankreas divisium tespit edildi. Hastaların 37'sine (%80,4) endoskopik sfinkterotomi, 10'una (%21,7) dilatasyon yapıldı. 22'sinden (%47,8) taş çıkarıldı ve 27'sine (%58,6) stent takıldı. Pankreatit, kanama, perforasyon gibi major komplikasyon olmadı. Sonuç: Pediyatrik yaş grubunda ERCP'nin deneyimli kişiler tarafından yapıldığı taktirde çocuklarda oldukça güvenilir, etkili bir tanı ve tedavi yöntemidir.