Cyclic Neutropenia is a benign, unusual hematologic disorder characterized by regularly recurring episodes of severe neutropenia occurring approximately every 21 days. Beside neutrophils, monocytes, lymphocytes, platelets and reticulocytes all cycle with strict periodicity suggest that this disease should be viewed as cyclic hematopoiesis, not merely as cyclic neutropenia. During neutropenic periods, patients regularly experience aphthous stomatitis, fever, malaise, cervical lymphadenitis, cutaneous infections and occasional pneumonia and otitis media. The exact cause of cyclic neutropenia is unknown. But it is strongly suggested that cyclic neurtopenia is due to an abnormality in the regulation of early hematopoietic precursor cells. We have documented a case of cyclic neutropenia for a period of 2.5 years in a 11 year-old boy who had suffered from recurrent fever, stomatitis, gingival swelling, cervical lymphadenitis and skin infections at 3 weeks intervals since 5 years of age. A brief review of the related literatures is presented.
Child ; Fever ; Hematopoiesis ; Humans ; Lymphadenitis ; Lymphocytes ; Male ; Monocytes ; Neutropenia* ; Neutrophils ; Otitis Media ; Periodicity ; Pneumonia ; Reticulocytes ; Skin ; Stomatitis ; Stomatitis, Aphthous

No abstract available.
Adipose Tissue* ; Bone Density* ; Female ; Humans ; Middle Aged*

No abstract available.
Humans ; von Willebrand Disease, Type 1* ; von Willebrand Diseases

No abstract available.
Humans ; von Willebrand Disease, Type 1* ; von Willebrand Diseases

No abstract available.
Pulmonary Embolism*

PURPOSE: To evaluate the morphologic differential diagnosis of benign and malignant ductal breast tumors, as seen on US. MATERIALS AND METHODS: US findings in 29 pathologically proven cases of ductal breast tumor were retrospectively reviewed. All patients were female and their mean age was 42 years. Nineteen tumors were benign and ten were malignant, and all ductal or cystic lesions showed solid masses. According to the location of the mural nodule, we classified the sonographic appearance of these tumors into three types : intraductal, intracystic and amorphic. The intraductal type was divided into three subtypes: incompletely obstructive, completely obstructive and multiple mural nodules. For the intracystic type, too, three subtypes were designated : the intracystic mural nodule (mural cyst), intracystic mural nodule with the duct (mural cyst+duct) and intracystic multiple mural nodules. The amorphic type is defined as an atypical ductal tumor with the mural nodule extending into adjacent parenchyma. RESULTS: The margin of the duct or cyst was smooth in 68.4% of benign, and irregular in 90% of malignant ductal tumors. Internal echogeneity of the duct or cyst usually showed homogeneity in both benign and malignant tumors. 73.7% of tumors connecting the duct were benign and 50% were malignant. In benign tumors, 52.6% of mural nodule had an irregular margin, while in malignant tumors, the corresponding proportion was 100% ; both types usually showed heterogeneous hypoechogeneity. Among benign tumors, the most common morphologic type was the intraductal incompletely obstructive subtype (36.8%) ; among those that were malignant, the amorphic type was most common, accounting for 40% of tumors. No amorphic type was benign and no incompletely obstructive subtype was malignant. CONCLUSION: When ductal breast tumors are morphologically classified on the basis of sonographic findings, the intraductal incompletely obstructive subtype suggests benignancy, and the amorphic type, malignancy. The morphologic classification of ductal breast tumors based on sonography is therefore useful for the differential diagnosis of benignancy and malignancy.
Breast Neoplasms* ; Breast* ; Classification* ; Diagnosis, Differential* ; Female ; Humans ; Retrospective Studies ; Ultrasonography*

The syndrome of postural headache associated with low CSF pressure usually occurs after a dural puncture, myelography, or spinal anesthesia. However, it can also develop spontaneously or after minor trauma. Although the clinical and MRI findings of this syndrome are well described, radionuclide cisterno-graphic findings have not yet been clearly characterized. We report four patients presenting with postural headache associated with very low or unmeasurable CSF pressure. Three patients developed after minor trauma, while one developed spontaneously. Brain MRI revealed symmetric pachymeningeal enhancement in all four patients. Radionuclide cisternography demonstrated delayed migration of tracer to the cerebral convexity at 6 and 24 hours in three patients, and early soft tissue uptake suggestive of CSF leakage in one. We suggest that these radionuclide cisternographic findings are supportive evidences for the presence of intracranial hypotension and may provide useful information for appropriate treatment.
Anesthesia, Spinal ; Brain ; Headache ; Humans ; Intracranial Hypotension* ; Magnetic Resonance Imaging ; Myelography ; Punctures

Among the complications of Clostridium difficile (C. difficile) infection, rectal prolapse has been very rarely reported in children. We report a 29-month-old girl who presented with rectal prolapse complicated with C. difficile-associated pseudomembranous colitis following 3-week course of oral amoxicillin/clavulanic acid for treatment of acute otitis media. The patient complained of fever, abdominal pain and mucoid bloody diarrhea. She also showed a protruded and everted rectal mucosa with discrete white-yellowish exudative plaques. Abdominal CT scan revealed a diffuse wall thickening with mucosal enhancement of the rectosigmoid colon. Both stool culture and toxin assay for C. difficile were positive. Her symptoms were completely improved with oral metronidazole treatment. C. diffile-associated pseudomembranous colitis should be considered as a rare but possible cause of rectal prolapse in children who have recently received antibiotic therapy.
Abdominal Pain ; Child* ; Child, Preschool ; Clostridium difficile ; Colon ; Diarrhea ; Enterocolitis, Pseudomembranous* ; Female ; Fever ; Humans ; Metronidazole ; Mucous Membrane ; Otitis Media ; Rectal Prolapse* ; Tomography, X-Ray Computed

No abstract available.
Humans ; Male ; Nursing* ; Urinary Incontinence*

Omphalocele is a rare congenital malformation which is a herniation of the abdominal viscera into the base of the umbilical cord. We experienced a ruptured large omphalocele of short duration which contained of intestines, stomach, liver and speen without major of additional anomalies. He was repaired with the simple skin converage as a part of second staged operation. He had an eventful postoperative course but wad discharged on the 52th postoperative days with the good condition and with the plan of repair of ventral hernia about 1 year. Here, the literature on omphalocele was reviewed with the experienced case briefly.
Hernia, Umbilical* ; Hernia, Ventral ; Intestines ; Liver ; Skin ; Stomach ; Umbilical Cord ; Viscera