Measurement of serum granulocyte colony-stimulating factor in a patient with congenital agranulocytosis (Kostmann's syndrome)

Article Abstract:

Granulocyte colony-stimulating factor (G-CSF) is a growth factor that regulates the formation of granulocytes, or granular white blood cells, such as neutrophils. The Kostmann's syndrome, also known as congenital agranulocytosis, is a birth-related disorder characterized by an abnormally low number of neutrophils. The cause of Kostmann's syndrome is not known, but may be related to a defect in the production or regulation of G-CSF, since patients with this disorder benefit from G-CSF therapy. The use of measurements of blood G-CSF levels in determining the cause, diagnosis and treatment of a patient with Kostmann's syndrome is illustrated. A case is described of a 12-month-old boy with Kostmann's syndrome and cavitary pulmonary disease, a lung disease characterized by the formation of cavities in lung tissue. The patient also had a history of inflammation of the conjunctiva, the membrane lining the eyelids and eyeball; inflammation of the connective tissue surrounding the eye cavity; inflammation of the lung; and ear infection since 10 days of age. The remnant of the umbilical cord, which connects the infant to the placenta, had not fallen off until three weeks after birth. The infant was diagnosed with an abnormal decrease in neutrophils (neutropenia) at four weeks of age. Analysis of the bone marrow showed delayed development of granulocytes, and blood levels of G-CSF were not detectable. Treatment with filgrastim, also known as G-CSF, increased the numbers of neutrophils to 10 billion cells per liter. These findings are consistent with evidence suggesting that Kostmann's syndrome results from a defect in the regulation or production of G-CSF. (Consumer Summary produced by Reliance Medical Information, Inc.)

Measurement, Drug therapy, Granulocyte colony-stimulating factor, Agranulocytosis, Granulocytopenia, Neutropenia

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Hemorrhagic shock and encephalopathy: clinical definition of a catastrophic syndrome in infants

Article Abstract:

In 1983, a condition that affected 10 infants in Great Britain was reported which was characterized by a rapid onset of shock, seizures, and fever. These symptoms were followed by coagulation of the blood, diarrhea, acidosis, and dysfunction of the kidneys and liver. The outcome of this disease was poor, with seven infants dying and the remaining infants suffering major brain damage. Other cases have also been reported, but some descriptions have lacked uniformity of clinical features. The condition has been termed hemorrhagic shock and encephalopathy (HSE), and it has been suggested that this represents a distinct clinical entity. This paper supports this idea, and has set out proposed diagnostic criteria for HSE to distinguish it from other similar childhood disorders. Nine infants with the same clinical signs and course were seen between 1980 and 1986 at a children's hospital in Virginia. All of the infants were less than six months old, four were black, and five were male. All but one of the infants were healthy prior to the onset of HSE. Three of the children died in spite of treatment, and all but one of the survivors were left with significant neurological abnormalities. Both the clinical and pathological characteristics were the same as those described for the 10 infants in the British study, with the exception of one infant with a fatty liver. It is thought that early elimination of other similar diagnoses will be facilitated by the recognition of this syndrome. (Consumer Summary produced by Reliance Medical Information, Inc.)

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The 'h' in hemorrhagic shock and encephalopathy syndrome should be 'hyperpyrexia'

Article Abstract:

An article by Chaves-Carballo et al. appearing in the October 1990 issue of the American Journal of Diseases of Children proposes the recognition of a condition in infants termed hemorrhagic shock and encephalopathy (HSE) syndrome. Although it is agreed that HSE should be given a distinct diagnostic category to separate it from other similar conditions, the term HSE is misleading. The name implies that the shock seen in this condition is the result of a hemorrhage such as that caused by trauma, ulcers, or esophageal varices. But in HSE, the bleeding occurs later in the shock stage of the condition, following the coagulation disorder that is typical of the disease. The causes of the coagulation disorder in HSE are not clear; it may be due to diffuse intravascular thrombosis or to disseminated intravascular coagulation, as Chaves-Carballo et al. propose. Patients with HSE also typically develop liver disease. The key feature in HSE seems to be hyperpyrexia, a very high fever, which is similar to that seen in heat stroke and malignant hyperthermia; the clinical findings of these conditions are very similar. A better understanding of the causes of hyperpyrexia in HSE syndrome is needed. The suggestion of Chaves-Carballo et al. to recognize HSE as a distinct disorder is sound, but a more appropriate term to classify this disorder is 'hyperpyrexia, shock and encephalopathy syndrome', rather than hemorrhagic shock and encephalopathy syndrome. (Consumer Summary produced by Reliance Medical Information, Inc.)

Fever in children, Childhood fever, editorial

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