[Cervical lymphadenitis caused by non-tuberculous mycobacteria in children with different residence].

Objective: Atypical mycobacteria form a heterogeneous group. Although more than 140 species have been identified, only 25 of them are considered responsible for infection in humans. The most frequent manifestation of the disease in immunocompetent children is the cervical lymphadenitis.

Development of Haemophilia Treatment in the Eastern Part of Germany over the Last Decade in the Kompetenznetz Hämorrhagische Diathese Ost (KHDO).

Introduction:  In 2005 the Kompetenznetz Hämorrhagische Diathese Ost published epidemiologic data about patients with haemophilia A (HA) and haemophilia B (HB) in the eastern part of Germany. This study provides data about the development of treatment in these patients over the past 10 years.

Methods:  Data from 12 haemophilia centres in eastern Germany were retrospectively collected for the year 2015 from patients' records.

Spontaneous omental bleeding in a 20-year old patient with hemophilia A. A rare cause for emergency laparotomy.

Spontaneous intraabdominal hemorrhage is a very rare event even in patients with bleeding disorders like hemophilia. Nevertheless this rare case must be considered in patients with coagulopathies presenting with abdominal pain. Prompt radiologic imaging and surgical consultation are of highest priority.

Small part ultrasound in childhood and adolescence.

Small-part sonography refers to the display of small, near-surface structures using high-frequency linear array transducers. Traditional applications for small part ultrasound imaging include visualization and differential diagnostic evaluation in unclear superficial bodily structures with solid, liquid and mixed texture, as well as similar structures in nearly superficial organs such as the thyroid glands and the testes. Furthermore indications in the head and neck regions are the assessment of the outer CSF spaces in infants, the sonography of the orbit, the sonography of the walls of the large neck vessels, the visualization of superficially situated lymph nodes and neoplasms.

Desmopressin testing in haemophilia A patients and carriers: results of a multi centre survey.

Introduction: Desmopressin (DDAVP) testing (DT) in patients (pts) with haemophilia A (HA) and carriers (CHA) is up to now not standardized. This prompted us to evaluate results of DT carried out between 1996 and 2011 in centres of the Competence Network Haemorrhagic Diatheses East.

Patients And Method: An increase of the factor VIII activity (FVIII) above 50% or at least the two fold of initial values within 120 min after DDAVP was defined as complete response (CR).

[Desmopressin testing in children with von Willebrand syndrome in haemostaseologic centers of Saxonia, Saxonia-Anhalt and Thuringia].

The influence of desmopressin on hemostasis is mediated by the release of von Willebrand factor and of coagulation factor VIII from vascular endothelium. The necessity of testing desmopressin effectiveness on hemostasis is a matter of controversy and the performance of the test is not yet standardized. For this reason the desmopressin tests in 114 children with von Willebrand syndrome (type 1, n=98; type 2A, n=12; type 2M, n=2; type 2N, n=2) carried out in 7 paediatric haemostaseologic centers were retrospectively analyzed.

[Incidental finding of an acute appendicitis in a premature newborn with haematochezia].

In newborns, acute appendicitis is a very rare condition associated with significant lethality. Due to mostly non-specific symptoms, it is difficult to find the correct diagnosis preoperatively. Interestingly, rectal bleeding as a clinical sign in neonatal appendicitis is very uncommon.

[Multicentric extra-abdominal fibromatosis: a rare case].

Extra-abdominal aggressive fibromatosis is a benign fibroblastic neoplasia with an infiltrative nature and a high tendency of local recurrence. Here, we report on a very rare case of multicentric fibromatosis. Low complaints led to considerable size of the tumours.

[Epitheloid leiomyosarcoma of the mouth in childhood].

A six-year-old girl is presented with an increasing mass involving the anterior vestibule and the floor of the mouth. Histologic and immunohistochemical examination revealed a poorly differentiated epitheloid leiomyosarcoma with destruction of the mandible. According to the CWS-96-study the patient underwent preoperative chemotherapy followed by complete resection of the mandibular body with the surrounding soft tissues.

Chronic natural killer cell lymphocytosis is associated with elevated cytotoxic activity of natural killer cells.

The authors describe a 16-year-old girl who has suffered from chronic natural killer cell lymphocytosis (CNKL) for 12 years. From age 4 years, she has shown a persistent lymphadenopathy and lymphocytosis. Clinically, she developed allergic skin involvement, thrombocytopenia, and peripheral polyneuropathy.

Familial urticaria pigmentosa associated with thrombocytosis as the initial symptom of systemic mastocytosis and Down's syndrome.

Most cases of urticaria pigmentosa are confined to the skin, but visceral involvement and/or haematological abnormalities have been observed. It is still a matter of debate whether all forms of mastocytosis are true neoplasias or reactive hyperplasias. Familial inheritance of urticaria pigmentosa is rare.

Influenza B pneumonia with Staphylococcus aureus superinfection associated with parvovirus B19 and concomitant agranulocytosis.

An 11-year-old patient with anamnestic fever for 3 days and signs of upper respiratory tract infection underwent fulminant Staphylococcus aureus pneumonia with concomitant agranulocytosis. From autopsia influenza B virus and parvovirus B19 were detected by nucleic acid amplification technique (NAT). Specific IgG but no IgM points to preexisting parvovirus B19 infection.

von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP.

Thrombotic thrombocytopenic purpura (TTP) is caused by the persistence of the highly reactive high-molecular-weight multimers of von Willebrand factor (VWF) due to deficiency of the specific VWF-cleaving protease (VWF-CP) ADAMTS13, resulting in microangiopathic disease. The acquired form is caused by autoantibodies against VWF-CP, whereas homozygous or compound heterozygous mutations of ADAMTS13 are responsible for recessively inherited TTP. We investigated 83 children with hemolytic or thrombocytopenic episodes with or without additional neurologic symptoms or renal failure.

[Postoperative haemorrhagia in a girl with congenital factor XI deficiency - successful treatment with desmopressin (DDAVP, Minirin(R))].

Unlabelled: The rare factor XI deficiency is associated with different profuse bleeding without correlation to the severity of reduction of factor XI. Accordingly, traumata or surgical procedures may cause unexpected excessive bleeding in asymptomatic patients. After surgery of a nine-year-old girl with factor XI deficiency (8 per cent) profuse bleeding occurred which could only be stopped after infusion of desmopressin.

Gliomatosis cerebri: post-mortem molecular and immunohistochemical analyses in a case treated with thalidomide.

Gliomatosis cerebri (GC) is a rare tumor of the central nervous system (CNS) characterized by widespread diffuse infiltration of the brain and spinal cord by neoplastic glial cells. We report the case of a 17-year-old boy with a bioptically diagnosed fibrillary astrocytoma. The administration of thalidomide, which was suggested to be beneficial in the treatment of human cancers, had no substantial clinical effect on our patient.

[Factor VIII inhibitors in patients suffering from severe haemophilia A: problems of "very low" responders].

Without recognition of any inhibitor until now, low concentrations of factor VIII inhibitors (< 1 Bethesda unit (BU)/mL plasma) can be occasionally measured in patients with severe haemophilia A. The existence of so-called "very low" responders is assessed contradictorily due to a methodically caused inhibitor increase. Plasma from 10 patients with severe haemophilia A was incubated with human plasma or animal plasma from pig, cattle, or cat and assayed for factor VIII inhibitors.

An approach to study the viral safety of plasma-derived products in previously treated, non-infected patients.

Using the polymerase chain reaction (PCR), we designed a study concept to evaluate the safety of plasma derivatives in previously treated patients who are non-infected by the specific viruses studied. Several product lots can be studied in a single patient, with a study period for each lot of 3 months. In the present study 19 patients were included for treatment with Baxter Hyland Immuno's PCR-screened factor VIII concentrate Immunate (n=7), factor IX concentrate Immunine (n=10), the by-passing agent FEIBA plus Immunine (n=1), and the protein C concentrate Ceprotin (n=1).

Molecular biology and clinical manifestation of hereditary factor VII deficiency.

Inherited factor VII (FVII) deficiency is a rare autosomal recessive disorder. Mutations and polymorphisms of the FVII gene were characterized in more than 40 unrelated patients with FVII deficiency. Among the 29 different mutations, the most frequent were Ala294 Val, Ala294Val;404delC, IVS7+7, and Val281 Phe.

Hepatic and retroperitoneal tumor resection for late metastases of a Wilms' tumor in an adult patient--a case report.

Hepatic metastases after a Wilms' tumor in adult patients are seen extremely rarely. A 21 year-old male patient developed liver metastases 13 years after resection of a primary left extrarenal Wilms' tumor. In this case, without any other metastases, extended right curative hepatic lobectomy was performed.

[Treatment concepts in osseous manifestations of Langerhans cell histiocytosis].

Introduction: Patients with Langerhans Cell Histiocytosis (LCH or Eosinophilic granuloma) were assessed from the orthopaedic point of view to give recommendations for the management of the disease.

Material And Methods: The results of 36 cases of histologically proven bony manifestations out of 48 treated cases were reviewed. A retrospective analysis of our treated cases with bony manifestations of LCH between 1970 and 1995 was performed.

von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome.

Background: Thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome are severe microvascular disorders of platelet clumping with similar signs and symptoms. Unusually large multimers of von Willebrand factor, capable of agglutinating circulating platelets under high shear stress, occur in the two conditions. We investigated the prevalence of von Willebrand factor-cleaving protease deficiency in patients with familial and nonfamilial forms of these disorders.