Weitere Untersuchungen über Polycythaemia vera im Kindesalter

Wieland, E.

doi:10.1007/bf02248294

Cited by 7 publications

(2 citation statements)

References 2 publications

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“…Wieland (46), cited and agreed with this classification, but saw no good reason to distinguish group (i) from polycythaemia vera in adults. Subsequent authors have however done so on account of the benign course of the familial condition: Spodaro and Forkner (24) used the name 'benign familial erythrocytosis' and Knock and Githens (42) reporting a sporadic case, called it 'primary erythrocytosis'.…”

Section: Tabu 11mentioning

confidence: 92%

Familial Erythrocytosis

Mg¹,

Lawrence²,

Lander³

et al. 1968

Acta Haematol

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Section: Tabu 11mentioning

confidence: 92%

Familial Erythrocytosis

Mg¹,

Lawrence²,

Lander³

et al. 1968

Acta Haematol

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“…In many, the data presented suggest that the erythro¬ cytosis was due to an underlying hypoxic cause. In the family described originally by Engelking,*'7 later by Wieland,27'28 and finally by Hottinger,12 five of the six cases had clubbing of the fingers. A similar find¬ ing was noted by Kretschmer 14 in his two families.…”

Section: Commentmentioning

confidence: 99%

Primary Erythrocytosis of Childhood

Knock¹,

Githens²

1960

Arch Pediatr Adolesc Med

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Erythrocytosis (an increase in the total red cell mass)* in childhood is usually secondary to cyanotic congenital heart disease. Occasionally it occurs with pulmonary disease, methemoglobinemia, brain tumors, Cushing's disease, or renal adenoma. When there is an accompanying increase of white blood cells and platelets, the condition is termed polycythemia vera; this is extremely rare in children. Occasionally the condition may be familial with no elevation of white blood cell or platelet count and with no underlying cause or associated condition discernable. It is the purpose of this paper to present a case of primary erythrocytosis in a 6-year-old child whom we have been able to investigate. Report of CaseThe patient, a 6-year-old boy, was first seen in the Congenital Heart Disease Clinic of the University of Colorado Medical Center on Jan. 22, 1957. He had been referred because of cyanosis and decreasing tolerance for exercise. Pregnancy and delivery had been normal, as had growth and development. The mother stated that he had been "bluish" since infancy and that this appearance had become more marked recently. He had oc¬ casional mild headaches and was unusually sleepy ; otherwise he had been quite well except for several attacks of asthma, generally mild, during the previ¬ ous two years.Family history revealed that his father was well except for occasional "asthma and hay fever." His mother and three siblings were all well.On physical examination the patient was found to be a well-developed, well-nourished white boy, bright and alert. The pulse was 90, respirations 18, and the blood pressure 110/80. There was a reddish-blue appearance to the skin, also noted in the nailbeds, lips, and oral mucosa. The bulbar conjunctivae appeared injected. The remainder of the examination was normal. No heart murmur was heard, and there was no cardiomegaly. The lungs were normal to auscultation and percussion.There was neither hepatosplenomegaly nor club¬ bing.The routine laboratory studies showed a hemo¬ globin of 27.8 gm. %, a red blood cell count of 9.25 million, hematocrit 78%, and a white blood cell count of 8,300 with 52% neutrophils, 46% lymphocytes, and 2% monocytes. Platelets num¬ bered 203,500/cu. mm., and the reticulocyte count was 1.2%. Other studies revealed a serum iron of 32/ig/lOO ml. and a latent iron-binding capacity of 371j[ig/100 ml. Hemoglobin electrophoresis showed only normal adult hemoglobin (AA). Fetal hemoglobin constituted 1.5% of the total hemoglobin. Serum uric acid, serum bilirubin, and serum proteins were all within normal limits. The urine showed no abnormalities.Since this patient showed an obvious erythrocy¬ tosis, studies were carried out to determine whether there was any underlying condition which would justify the diagnosis of secondary polycythemia. (As mentioned above, physical findings were nega¬ tive except for the plethora.) Further studies in¬ cluded an electrocardiogram, chest x-rays, and

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Erbpathologie des Blutes und der blutbildenden Organe

Gänsslen¹

1940

Erbbiologie Und Erbpathologie Körperlicher Ƶustände Und Funktionen II

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Weitere Untersuchungen über Polycythaemia vera im Kindesalter

Cited by 7 publications

References 2 publications

Familial Erythrocytosis

Familial Erythrocytosis

Primary Erythrocytosis of Childhood

Erbpathologie des Blutes und der blutbildenden Organe

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