Talaromyces marneffei infection in an HIV infected patient with hematological malignancy - first report from Turkey.

T.marneffei, encountered mostly in Southeast Asia, leads to a systemic infection, especially in immunocompromised individuals such as HIV-infected patients with low CD4 level. A 32-year-old male patient, residing in Hong Kong for the last two years, admitted with fever, cough, weakness, and weight loss. Physical examination revealed bilateral cervical and axillary multiple lymph nodes and hepatosplenomegaly. Screening of the pancytopenic patient revealed HIV infection. Histopathological examination of the cervical lymph node revealed plasmoblastic lymphoma. Blood and urine cultures remained sterile. Antiretroviral therapy was started. Fungal hyphae were detected in Gram staining of hemocultures taken in the third week due to ongoing fever, and antifungal therapy was started empirically. Red pigment around colonies on Sabouraud dextrose agar and microscopic appearance arose suspicion of Talaromyces spp. T.marneffei was identified by ITS 1-4 sequence analysis. Chemotherapy was started when fungemia was controlled. On the fifth day of chemotherapy, the patient's general condition deteriorated, broad-spectrum antibiotics were started and the patient was transferred to ICU. The cultures remained sterile and he expired five days later. In conclusion, although talaromycosis is not endemic in Turkey, it should be considered in patients with travel history to endemic regions and/or an underlying immunosuppressive disease such as HIV infection.

Soluble neuregulin-1 modulates disease pathogenesis in rodent models of Charcot-Marie-Tooth disease 1A.

Duplication of the gene encoding the peripheral myelin protein of 22 kDa (PMP22) underlies the most common inherited neuropathy, Charcot-Marie-Tooth 1A (CMT1A), a disease without a known cure. Although demyelination represents a characteristic feature, the clinical phenotype of CMT1A is determined by the degree of axonal loss, and patients suffer from progressive muscle weakness and impaired sensation. CMT1A disease manifests within the first two decades of life, and walking disabilities, foot deformities and electrophysiological abnormalities are already present in childhood. Here, we show in Pmp22-transgenic rodent models of CMT1A that Schwann cells acquire a persistent differentiation defect during early postnatal development, caused by imbalanced activity of the PI3K-Akt and the Mek-Erk signaling pathways. We demonstrate that enhanced PI3K-Akt signaling by axonally overexpressed neuregulin-1 (NRG1) type I drives diseased Schwann cells toward differentiation and preserves peripheral nerve axons. Notably, in a preclinical experimental therapy using a CMT1A rat model, when treatment is restricted to early postnatal development, soluble NRG1 effectively overcomes impaired peripheral nerve development and restores axon survival into adulthood. Our findings suggest a model in which Schwann cell differentiation within a limited time window is crucial for the long-term maintenance of axonal support.

Oral rehydration of infants in a large urban U.S. medical center.

A prospective randomized study of 100 well-nourished infants with acute gastroenteritis resulting in dehydration and acidosis was carried out at the Jackson Memorial Hospital, Miami from 1981 to 1983. Patients were randomly assigned to receive either standard intravenous therapy or oral rehydration. Infants in the latter group first received solution A containing 75 mEq/L sodium, 30 mEq/L potassium, 75 mEq/L chloride [corrected], 30 mEq/L bicarbonate, and 2 gm/dL glucose [corrected]. After ad libitum feeding for six hours, solution B containing 50 mEq/L sodium, 30 mEq/L potassium, 50 mEq/L chlorine, 30 mEq/L bicarbonate, and 3 gm/dL [corrected] glucose was given. With three exceptions (6%), oral rehydration was comparable to the intravenous regimen in clinical estimates of improvement, although the oral group had more stools in the first day. The oral group had faster correction of acidosis and a sustained rise in serum potassium concentration, whereas in the intravenous group the potassium concentration showed first a drop with a later increase, but levels were at all times below those in the oral group. Although potassium was given from the beginning of oral rehydration, and at a higher concentration than recommended by the World Health Organization, no hyperkalemia occurred. We concluded that oral therapy is safe, less expensive for patients, and more convenient for the medical and nursing staffs.