Acute respiratory distress in Pena-Shokeir syndrome.

Pena-Shokeir syndrome is a rare, autosomal-recessive disorder that usually affects newborns. Its etiology is poorly understood. Pena-Shokeir syndrome is defined by camptodactyly, multiple ankyloses, pulmonary hypoplasia, and various facial anomalies.

Tumor necrosis factor and chemotherapeutic agents. Potentiation of cytotoxicity with interferon gamma.

This study examined combinations of the recombinant human cytokines, tumor necrosis factor alpha and interferon gamma, with doxorubicin and dactinomycin as well as other drugs on six squamous cell carcinoma cell lines of head and neck origin using the 3(4,5-dimethylthiazol-2-yl)2,5-diphenyl-tetrazolium bromide proliferation assay. Interferon gamma significantly enhanced the cytotoxicity of tumor necrosis factor alpha with dactinomycin on all six cell lines investigated, while in four of six cell lines the cytotoxicity of tumor necrosis factor alpha with doxorubicin was significantly augmented by interferon gamma. Additional experiments showed no effect with either cytokine in combination with cisplatin, fluorouracil, methotrexate, or etoposide.

Squamous cell carcinoma in an inverted papilloma of the buccal mucosa.

Inverted papillomas of the oral cavity are rare lesions. Although in the seven oral cases previously reported the lesions were benign, approximately 10% to 15% of inverted papillomas of the nasal cavity and paranasal sinuses develop or are associated with squamous cell carcinoma. This report presents a case of squamous cell carcinoma arising in an inverted papilloma of the buccal mucosa.

Cyclic neutropenia terminating in permanent agranulocytosis.

A patient with cyclic neutropenia was followed for 20 years. After 16 years she developed a permanent agranulocytosis, and lived for nearly four years without neutrophils in the peripheral blood. Neither prednisone, lithium nor leuco- and plasmapheresis had any effect on the neutrophil count.