Michael J. Chusid

The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.

Several closely related disease entities make up the idiopathic hypereosinophilic syndrome (HES). The syndrome is manifest by persistent and prolonged eosinophilia with organ damage. A group of 14 patients had hematologic, cardiac, and neurologic abnormalities attributable to this disease. Patient survival and response to chemotherapy was significantly better in this group than in previously reported patients. The etiology of HES remains unknown, as does the mechanism of tissue damage.

Correction of infantile agranulocytosis (Kostmann's syndrome) by allogeneic bone marrow transplantation

Allogeneic bone marrow transplantation has been unsuccessful as therapy for genetically determined bone marrow disorders. In patients prepared for transplantation with drugs alone long-term hematopoietic engraftment is not achieved due to the overgrowth of the infused donor bone marrow cells by residual recipient hematopoietic stem cells. Utilizing a combination of total body irradiation and antihuman thymocyte serum, the successful eradication of the abnormal hematopoietic stem cells of patients with the Wiskott-Aldrich syndrome and now infantile agranulocytosis has been achieved. Following preparation with total body irradiation and antihuman thymocyte serum a 20 month old patient with infantile agranulocytosis has complete donor hematopoietic and lymphoid engraftment one year after a histocompatible allogeneic bone marrow transplant. Prior to transplantation, this patient had no circulating or bone marrow granulocytes; following transplantation he has normal numbers of circulating granulocytes with normal in vivo and in vitro function. This therapeutic result demonstrates that genetic disorders of myeloid function can be corrected by allogeneic bone marrow transplantation following preparation with total body irradiation and antihuman thymocyte serum, and suggests that infantile agranulocytosis is due to an intrinsic defect of the pluripotent hematopoietic stem cell and not to a micro-environmental defect.

Clinical and epidemiologic characteristics of children hospitalized with 2009 pandemic H1N1 influenza A infection.

Background: In 2009, pandemic H1N1 influenza caused significant morbidity and mortality worldwide. We describe the clinical and epidemiologic characteristics of children and adolescents hospitalized for 2009 H1N1 infections in Milwaukee, Wisconsin from April 2009 to August 2009. Methods: We conducted retrospective chart reviews of hospitalized patients with laboratory-confirmed 2009 H1N1 infections. Data on financial burden associated with these infections were obtained and analyzed. Results: A total of 75 children hospitalized for 2009 pandemic H1N1 infections were identified; the median age was 5 years (range, 2 months–19.2 years); 56% were males; 56% were Non-Hispanic Blacks; and 75% had at least one underlying medical condition. Twenty-four percent had only upper respiratory symptoms. Bacterial coinfections occurred in 1.3%. All but one patient received antivirals, 80% of patients received antibacterials, 18.6% were admitted to the intensive care unit, 6% required mechanical ventilation, 2.6% required extracorporeal membrane oxygenation, and 2.6% died. The total charges incurred for H1N1 influenza hospitalizations were

Pulmonary Aspergillosis, Inhalation of Contaminated Marijuana Smoke, Chronic Granulomatous Disease

4,454,191, with individual charges being highest for children >12 years of age. Conclusions: The majority of children with pandemic H1N1 influenza-associated hospitalizations had uncomplicated illness despite the frequent presence of high-risk conditions in our patient population. Laboratory-confirmed 2009 pandemic H1N1 influenza hospitalizations resulted in substantial health care and economic burden during the first wave of the pandemic in spring 2009.

Chronic, Recurrent Multifocal Osteomyelitis Case Report and Review of the Literature

Excerpt Increased susceptibility to infection is a hallmark of chronic granulomatous disease of childhood (1), an inherited disorder in which phagocytic cells are unable to kill certain types of in...

Cryptosporidiosis in children during a massive waterborne outbreak in Milwaukee, Wisconsin: clinical, laboratory and epidemiologic findings

Chronic recurrent multifocal osteomyelitis (CRMO) is an unusual inflammatory process involving multiple osseous sites, often recurrently. The authors recently diagnosed CRMO in an 8-year-old girl who had complained of toe pain for several weeks. A number of other involved, asymptomatic areas were discovered by bone scan. Biopsy of the lesions did not reveal any pathogens. Antibiotics and inflammatory agents were not used, and the patient recovered. A review of previously reported cases of CRMO showed 1) that this entity affects children almost exclusively, 2) that from two to 18 sites, usually in the extremities, can be affected at one time, and 3) that about 20 percent of the patients develop a pustular rash on their palms and soles. Other than an elevated erythrocyte sedimentation rate, there are no consistent laboratory abnormalities, and cultures of affected bone are negative. Histopathology typically shows a chronic inflammatory response, sometimes with granulomas. Antiinflammatory agents and antibiotics offer little consistent beneficial effect. The disease is self-limited, and patients usually recover fully, although exacerbations may occur. In the proper clinical setting, CRMO should be considered, since recognition of this entity avoids costly and potentially harmful diagnostic and therapeutic interventions.

Mycoplasma hominis and Ureaplasma species brain abscess in a neonate.

During the spring of 1993 an estimated 403000 residents of the greater Milwaukee, Wisconsin area experienced gastrointestinal illness due to infection with the parasite Cryptosporidium parvum following contamination of the citys water supply. To define the clinical, laboratory and epidemiologic features of outbreak-associated cryptosporidiosis in children, medical and laboratory records for all children submitting stool samples to the microbiology laboratory of the Childrens Hospital of Wisconsin between 7 April and 13 May 1993 were reviewed retrospectively. Interviews with parents were also conducted to obtain additional clinical history. Cryptosporidium, as the sole pathogen, was identified in stools from 49 (23%) of the 209 children enrolled in the study. Children with laboratory-confirmed cryptosporidiosis were more likely to live in areas of Milwaukee supplied with contaminated water (RR = 1.92, CI = 1.19-3.09), to be tested later in their illness (P < 0.05), to have submitted more than one stool specimen (P = 0.01), to have an underlying disease that altered their immune status (RR = 2.78, CI = 1.60-4.84), and to be older than 1 year of age (RR = 2.02, CI = 1.13-3.60). Clinical illness in these patients was more prolonged and associated with weight loss and abdominal cramps compared with Cryptosporidium-negative children. In the context of this massive waterborne outbreak relatively few children had documented infection with Cryptosporidium. If many children who tested negative for the parasite were truly infected, as the epidemiologic data suggest, existing laboratory tests for Cryptosporidium were insensitive, particularly early in the course of illness.

Kimura’s disease: an unusual cause of cervical tumour

We report an infant with a mixed Mycoplasma and Ureaplasma brain abscess who was successfully treated with intravenous doxycycline and erythromycin. Therapeutic concentrations of doxycycline were demonstrated in cerebrospinal fluid. This report is evidence of the potential for Mycoplasma and Ureaplasma to produce focal central nervous system infection, as well as meningitis, in neonates and that use of doxycycline can be efficacious in the therapy of such infections.

Eosinophilic leukemia. Remission with vincristine and hydroxyurea.

An 11 year old Chinese boy developed a unilateral cervical mass associated with pronounced eosinophilia and a marked increase in IgE concentrations. A biopsy sample showed massive eosinophilic tissue infiltration consistent with Kimura’s disease. This disorder should be suspected when the clinical triad of painless unilateral cervical adenopathy, hypereosinophilia, and hyper-IgE is present, particularly in male Asian patients.

Epidemiologic markers of pediatric infections caused by coagulase-negative staphylococci.

A 40 year old man with eosinophilic leukemia was treated with a variety of chemotherapeutic agents. A long-term remission, unusual for eosinophilic leukemia, was maintained with chronic hydroxyurea therapy after induction with vincristine. Hydroxyurea and vincristine may offer an effective mode of therapy for this disease.