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Biomedical subjects

H R Haak

Publications and source records attributed to H R Haak.

30 records · Page 2Linked to original sources

Should patients with medullary thyroid carcinoma undergo extensive lymph node (re)operation to improve long-term survival?

To investigate the role of primary and/or secondary modified radical neck dissection (MRND) in patients with medullary thyroid carcinoma (MTC), follow-up data on 43 patients operated on between 1972 and 1983 were analyzed retrospectively; patients included 16 with sporadic MTC (group 1) and 27 with multiple endocrine neoplasia type 2 (group 2). Primary surgical therapy consisted of (near-) total thyroidectomy and limited lymph node dissection; one patient in group 1 and two in group 2 underwent primary MRND. Positive nodes were found in nine patients of group 1 and in 12 of group 2. Calcitonin (CT) was raised postoperatively in 11 group 1 patients, who all died (four after additional MRND). Postoperative CT was normal in the four survivors of group 1. In group 2, post-operative CT was raised in 15 patients, including those who had MRND and in four apparently node-negative patients. Three of them underwent additional MRND, which was successful in one case. One patient in this group died of the disease; two patients developed elevated CT levels during follow-up, whereas in one, CT normalized spontaneously. In conclusion, the role of extensive lymph node surgery in patients with more than limited lymph node involvement or elevated postoperative CT levels remains to be established.

Adult↗

Correction of neutropenia associated with chronic lymphocytic leukaemia following treatment with granulocyte-macrophage colony-stimulating factor.

A patient with chronic lymphocytic leukaemia (CLL) and severe persisting neutropenia due to marrow infiltration of his leukaemia, developed bilateral Legionella pneumophila pneumonia for which he was treated with erythromycin, rifampin and ciprofloxacin. To increase the number of circulating polymorphonuclear neutrophils, the patient was treated with recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF) at a dose of 2 micrograms protein/kg bodyweight s.c./12 h. GM-CSF therapy resulted in a sustained rise of the neutrophil count from the fifth day of treatment onwards, without showing an effect on the number of circulating leukemic cells. The patient completely recovered from his pneumonia. It is suggested that the rise of the neutrophil count, due to GM-CSF, contributed to the improvement of the infection of this patient. Our observation illustrates that GM-CSF can be given safely to CLL-patients and that it can be used effectively in CLL patients with severe bacterial infections to restore neutropenia.

Granulocyte-Macrophage Colony-Stimulating Factor↗

Nuclear DNA content of medullary thyroid carcinoma in a large family with the MEN-2A syndrome.

Flow cytometry of medullary thyroid carcinoma (MCT) was performed in a large family with the MEN-2A syndrome. Of 15 family members with MCT five patients (10-27 yr) were without lymph node metastases. Six patients had a normal pentagastrin test after operation. All patients are alive and free of symptoms of MCT 6-9 yr after total thyroidectomy and an ablative dose of 131-I. In 12 of the 15 patients with MCT flowcytometry of paraffin-embedded tissue could be performed. The majority of all tumors (n = 9) were classified as peridiploid. Metastatic tumor, 6 years after thyroidectomy, in one of the patients was diploid. Only two MCT were clearly aneuploid. In one patient the tumor was tetraploid. We conclude that the majority of the MCT patients in this family with the MEN-2A syndrome have no or limited ploidy aberrations in their tumors, which correlates well with the favourable prognosis of familial MCT.

Adolescent↗

Prolonged bleeding time due to mitotane therapy.

After finding prolonged bleeding times in 2 patients treated with mitotane, we prospectively studied 7 patients with adrenocortical cancer on mitotane therapy. Before and 1 and 2 or more weeks after starting mitotane we determined the platelet counts, bleeding times and global coagulation parameters. All patients had a normal bleeding time before treatment. In 6 cases the bleeding time became prolonged (245-555 s). 4 patients exhibited platelet aggregation responses compatible with an aspirin-like defect. It is concluded that mitotane may cause a clinically relevant defect of platelet function.

Adolescent↗

Portal venous gas in a patient with diverticulitis.

Gas in the portal vein is a rare finding associated with a grave prognosis. We present a case of portal venous gas in a 34-year-old man with an abscess due to perforated-sigmoid diverticulitis and Escherichia coli sepsis. successfully treated with sigmoid resection and antibiotic therapy.

Abscess↗

Multiple endocrine neoplasia syndrome type 2: the value of screening and central registration. A study of 15 kindreds in The Netherlands.

Since 1975, 10 families with the multiple endocrine neoplasia (MEN)-2A syndrome and five with the MEN-2B syndrome, making a total of 101 patients, have been identified in The Netherlands. Twenty-three of the MEN-2A patients died before the start of the screening program. The average age of the patients whose death was due to pheochromocytoma (n = 11) or medullary thyroid carcinoma (n = 12) was 34.9 and 49.2 years, respectively. Eighty-seven patients with the MEN-2A syndrome and eight with the MEN-2B syndrome underwent thyroidectomy for C-cell hyperplasia and/or medullary thyroid carcinoma. Eighteen patients had signs or symptoms caused by MEN-2A (group A), 60 were relatives of these patients who had been found to be affected at the first screening of the family (group B), and nine relatives had had negative screening results that later became positive (group C). Five patients had signs or symptoms due to MEN-2B (group A) and three were relatives of these patients who had been found to be affected at the initial screening (group B). To assess the effect of screening, we compared these groups with respect to the occurrence of metastatic medullary thyroid carcinoma at thyroidectomy and the results of the postoperative calcitonin tests. Among the MEN-2A families, 72 percent of group A, 33 percent of group B, and none of group C were found to have metastatic medullary thyroid carcinoma at surgery. In the MEN-2B families, all five patients in group A and one of the three patients in group B had metastatic disease. The "cure rates" in these three groups with MEN-2A, as determined by stimulated calcitonin assessment, were 11, 57, and 100 percent, respectively. One of the five patients with MEN-2B in group A and two of the three patients in group B showed normalization of the stimulated calcitonin value after surgery. From these results, it may be concluded that screening can lead to the detection of medullary thyroid carcinoma in an earlier stage, which in turn may permit curative treatment and improvement of both prognosis and life expectancy. The need for supervision of affected families by central registration to promote periodic examination and to guarantee the continuity of such screening is discussed.

Adrenal Gland Neoplasms↗