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Biomedical subjects

C M Kullendorff

Publications and source records attributed to C M Kullendorff.

At least 37 records · Page 2Linked to original sources

Clonal karyotypic evolution in a pediatric neurofibrosarcoma.

A retroperitoneal neurofibrosarcoma infiltrating the spine of a 2-year-old boy was investigated cytogenetically three times over a 5-month period. The first sample, from a diagnostic fine-needle aspiration biopsy, had a supernumerary i(1)(q10) as the sole clonal aberration; two cells showed monosomy 18 in addition to the isochromosome. The second sample, obtained at tumor resection 3 weeks later, had the karyotype 47,XY, +i(1)(q10), -18, +21/45,XY, -18. After 5 months, a large local recurrence was resected. The chromosome analysis showed further clonal evolution: 45,XY, +1,der (1;11)dic(1;11)(q44;q13)i(1)(q10), inv(6)(p21q12), -17. The findings indicate that formation of i(1)(q10) and loss of chromosome 18 may be early genetic events in neurofibrosarcoma development.

Child, Preschool↗

Choledochal cyst in childhood.

During a two years period, four children were operated on because of choledochal cysts. All four patients were girls aged 9, 9, 8 and 6 years at operation. The symptoms at presentation were mainly episodic abdominal pain, mild jaundice and tenderness under the right costal curvature. Ultrasonography of the biliary tract revealed a cystic dilatation of the choledochus. Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the cystic dilatation and, importantly, delineated the junction of the pancreatic duct. The surgical procedure in all four patients consisted of complete excision of the choledochal cyst and extrahepatic biliary tree combined with a Roux-en-Y hepaticojejunostomy. The postoperative course of all patients was uneventful. Ultrasonography of the abdomen should be performed liberally in case of biliary tract symptoms in children.

Anastomosis, Roux-en-Y↗

DMSA scintigraphy in renal duplex system.

During a 4-year period 40 children (13 boys and 27 girls) with renal duplex system were examined with DMSA scintigraphy. The age at the investigation varied from 18 days to 9 years, mean 2.5 years. Duplicated system existed in 52 kidneys, bilaterally in 12 cases, right-sided in 10 and left-sided in 18 cases. The renal DMSA scintigraphy of the 52 duplicated kidneys revealed insignificant parenchymal damage in 19 kidneys. Moderate parenchymal damage existed in 10 kidneys. In the remaining 23 kidneys, severely deteriorated function of the upper moiety was found in 15 cases and of the lower pole in 9 cases, as both moieties were affected in one kidney. The function of the severely deteriorated moiety varied between 0% and 14%, mean 5.7% of the total renal function. All the 23 cases with severely deteriorated function were operated upon, 22 with heminephrectomy and one with nephrectomy. The assessment of parenchymal function of the separate parts of the kidney with duplex system by DMSA scintigraphy is of great value in the decision of management and surgical procedure.

Child↗

Elective testicular biopsy at the end of maintenance treatment for acute lymphoblastic leukemia. A prospective study.

Open testicular wedge biopsy has electively been performed prior to completion of maintenance therapy for acute lymphoblastic leukemia. 25 boys during 6 years were included in this prospective study. Three boys developed testicular enlargement while on maintenance therapy and biopsies confirmed testicular leukemia. The remaining 22 boys were biopsied at termination of maintenance therapy. Occult testicular relapse was revealed on histologic examination in only one boy with normal testicular size. During the follow-up period, one boy developed overt testicular relapse two months after negative biopsy. These results will not justify electively performed routine biopsy prior to termination of chemotherapy. Instead, careful and minute clinical examination of the testicles, estimating their size and consistence, should be performed regularly at short intervals.

Adolescent↗

Abdominal manifestations of non-Hodgkin's lymphomas.

25 children (18 boys and 7 girls) were treated for non-Hodgkin's lymphomas (NHL) during 1982-1990. The age at diagnosis was 2-15 years. Five of the 25 children died. Of the 25 children 6 patients had the diagnosis of intraabdominal NHL. The presenting abdominal symptoms for these 6 patients were abdominal pain in 4 cases, nausea 3, abdominal distention 2, icterus 1 and diarrhoea 1. Four of the 6 patients with abdominal NHL underwent laparotomy but the diagnosis was unknown before surgery. The indication for explorative laparotomy was in all cases a tumor of unknown etiology combined with ascites in 2 cases and icterus in 1 case. At surgery there was a tumor localized around the porta hepatitis in 1 case and in the right ovarium in 1 case. Furthermore, spread infiltrative growth of tumor was found in 3 cases, with infiltration of the peritoneum in 2 of these cases. A specimen for peroperative histological examination was taken and gave the conclusive diagnosis of NHL in 4 of 6 cases. Staging laparotomy, palliative tumor resection and radical surgery are preferably avoided. For proper treatment an adequate biopsy is important. The management of children with NHL is a multidisciplinary approach.

Abdominal Neoplasms↗

Roentgen stereophotogrammetric short-term analysis of growth rate in children operated for Crohn's disease.

Roentgen stereophotogrammetry is a new method for short term analysis of growth rate. Growth is measured over the growth zone in the distal fibula and is a fraction of the total statural growth. Normal growth rate in the distal fibula is about 15 micrograms/day at a total growth rate of 5 cm/year. The method was used to follow growth six months before and six months after surgery in six growth retarded children with Crohn's disease. Growth rate increased significantly (p less than 0.01) within three months after surgery from 5.8 microns/day before operation to 15.8 microns/day three months after operation and six months postoperatively had reached 18.0 micron/day. Improved growth could be recorded as early as four weeks after operation. The Roentgen stereophotogrammetric analysis (RSA) gives a unique possibility to follow short-term changes in growth rate in children with disturbed growth and to use growth rate as a quick assessment of response.

Adolescent↗

Diagnosis and treatment of ovarian cysts in children.

Seventeen girls were treated following the diagnosis of ovarian cysts. Four patients were operated on within the first 6 months of age and the other 13 patients were 10-15 years old at the time of diagnosis. Antenatal diagnosis was made in 3 cases. The presenting symptoms in the infants were distended abdomen in 3 cases, abdominal pain in 1 and vomiting in 1. In the older children the presenting symptoms were abdominal pain in 12, vomiting in 5 and elevated temperature in 6. Preoperative ultrasound was performed in 5 patients, 3 neonates and 2 older children. Sixteen of the 17 girls were operated on. The indication for surgery was an ovarian cyst with complication in the infants and in the older children the suspicion of acute appendicitis. The operative procedure was cyst uncapping in 7 cases, salpingo-oophorectomy in 4, ovarian resection in 2, ovarian fixation only in 1 and no ovarian intervention in 2. Asymptomatic infants with an ovarian cyst less than 4 cm in diameter can be managed conservatively. Surgery can be recommended after documented change of the cyst on ultrasound, large cysts giving rise to symptoms or presentation of an acute abdomen. Salvage of variable ovarian tissue is desirable.

Adolescent↗

Surgery in unilateral multicystic kidney.

During a 15-year period 29 patients were treated for unilateral multicystic kidney. The diagnosis was obtained from antenatal ultrasonography in 17 cases, investigation of an abdominal mass at routine palpation in 10, UTI investigation in 1 and uraemia investigation in 1. Eleven patients had associated malformations. Further investigations included ultrasonography, intravenous pyelography, micturition uretrocystography and in a few cases computed tomography and Dimercapto-succinic acid (DMSA) scan. Percutaneous pyelography was performed in 9 cases. Twenty-six children were operated on and 3 were not operated, of which 1 with severe cardiac malformations, died. The age at operation was under 6 months in 14 cases and between 6 and 12 months in 8. Four children developed hypertension preoperatively but the hypertension disappeared within 1 week postoperatively in all cases. The indication for surgery in unilateral multicystic kidney seems to be clear if the patient is symptomatic. In the asymptomatic patient, the complications of a remaining multicystic kidney like hypertension, malignancy, infection and pain suggest a surgical approach at around 6 months of age.

Female↗

Influence of renal sympathectomy, sodium depletion and prostaglandin synthetase inhibition on prostaglandin production and [3H]PGE2 binding characteristics in rat kidney.

The effects of renal sympathectomy (unilateral, renal microsurgical denervation), sodium depletion (hypovolaemia) and prostaglandin synthetase inhibition on the rate of prostaglandin synthesis and [3H]PGE2 binding characteristics were studied in the rat kidney. The intrarenal rate of prostaglandin synthesis was measured by monitoring the urinary excretion of 6-keto-PGF1 alpha, the stable hydration product of prostacyclin. Dietary sodium restriction was associated with a 99% decrease in urinary sodium excretion (P less than 0.001) and a 17% decrease of urine volume (n.s.). Renal denervation or sodium deprivation changed neither the rate of excretion of 6-keto-PGF1 alpha nor the density or affinity of [3H]PGE2 binding sites as compared to control. However, in sodium-depleted rats, prostaglandin synthesis inhibition, induced by naproxen, decreased the urinary excretion of 6-keto-PGF1 alpha by 40% (P = 0.011) and increased the number of [3H]PGE2 binding sites by almost 30% (P = 0.031) with no change in binding affinities as compared with sodium-depleted controls. In contrast, sulindac was not able to suppress the renal synthesis and excretion of 6-keto-PGF1 alpha, and did not modulate the [3H]PGE2 binding characteristics. The lack of effect on the excretion of 6-keto-PGF1 alpha and on the [3H]PGE2 binding characteristics supports the view that sulindac spares renal prostaglandin synthesis.

Animals↗

Late surgical problems in children born with abdominal wall defects.

During a 17 year period 55 patients with abdominal wall defects were treated. A questionnaire concerning late surgical problems was distributed to the parents of the 47 surviving children and 44 (94%) answers were received. The mean follow up time was 5.4 years. There was no mention of remaining problems regarding 16 of the 28 omphalocoele patients and 10 of the 16 gastroschisis patients. Postoperative abdominal wall hernia was reported in 7 cases with omphalocoele and in 6 with gastroschisis; postoperative intestinal stoma occurred in 1 child with omphalocoele associated with anal atresia, and in 1 with gastroschisis and postoperative intestinal obstruction in 4 cases with omphalocoele and in 1 with gastroschisis. The other complaints related to abdominal pain, cryptorchidism, constipation and difficulties with care of the intestinal stoma. No difference in results was found between the two types of closure of the abdominal wall defects irrespective of the primary treatment. All the remaining problems could be corrected and the long term results in both conditions were good.

Abdominal Muscles↗

Distribution of renal parenchymal damage on 99m-Tc DMSA scintigraphy in vesico-ureteric reflux.

During a 2 year period renal scintigraphy was performed in 64 children prior to surgery for vesico-ureteric reflux (VUR). In total 126 kidneys were examined. Renal scintigraphy was performed 3 hours after intravenous injection of 99m-technetium labelled dimercaptosuccinic acid (DMSA). The renal parenchyma was assessed as normal in 64 kidneys and abnormal in 62. Renal parenchymal damage was revealed in the upper pole in 42 cases, the middle lateral part in 27, the lower pole in 47 and the middle medial part in 25. The whole kidney was affected in 21 cases. Damage within one or two poles was present in 59 of the 62 kidneys with parenchymal damage. Renal scintigraphy is regarded a sensitive technique for detection of renal parenchymal damage. The DMSA scintigraphy can reveal even minor scars. It can be recommended as a routine investigation in evaluating children with VUR.

Child↗

Diagnostic cyst puncture of multicystic kidney in neonates.

In the neonatal period it is important to differentiate hydronephrosis from cystic disease of the kidney, since treatment of these entities differ. Early surgery in hydronephrosis may be indicated to salvage kidney function. We studied a group of 29 infants with renal cysts or hydronephrosis. In 10 cases some doubt about diagnosis remained after thorough diagnostic imaging. These infants were examined using percutaneous puncture of the kidney to verify the suspected diagnosis of multicystic renal disease. The studies were performed using local anesthesia and sedation. Ultrasonography was used for puncture and contrast medium was injected during fluoroscopy. The cysts communicated in 7 out of 10 cases, and a true renal pelvis was never seen. Irregular tubular structures joining the cysts were identified in 7 cases and seem to be characteristic of the multicystic dysplastic kidney. One instance of the hydronephrotic type of multicystic kidney was found at surgery. Large size of the cysts can make diagnosis difficult. Percutaneous puncture was successful and gave the diagnosis in all cases. No complications ensued.

Diagnosis, Differential↗

Renal parenchymal damage on DMSA-scintigraphy in pelviureteric obstruction.

During a 1.5 year period 21 children were investigated with 99-m-technetium dimercaptosuccinic acid (DMSA) before operation for hydronephrosis due to pelviureteric obstruction. The age at investigation was 0.2-11.5 years. Fourty-two kidneys were examined. Hydronephrosis existed on the right side in 8 cases, left side in 9 and bilateral in 4 cases. Seventeen kidneys had no obstruction. The scintigraphy was interpreted as normal in 19 kidneys. Decreased isotope uptake was found in 23 kidneys and localized to the upper pole area in 19 kidneys, middle-lateral part in 7, lower pole area in 15 and the middle-medial part in 12 kidneys. There were no predominance for any part of the kidney to be affected by parenchymal damage. In 8 children investigated before the age of 1 year, 4 of 10 hydronephrotic kidneys revealed normal DMSA scintigram. DMSA scintigraphy delineates functioning renal parenchyma. DMSA scintigraphy delineates functioning renal parenchyma. It can be recommended as a routine method for evaluation of the renal parenchyma before surgery and for follow up studies in all ages of childhood.

Child↗

Influence of renal denervation on vascular responsiveness of isolated rat intrarenal arteries.

Microsurgical renal denervation of the rat has been reported to increase blood loss and bleeding time after a standardized kidney resection. To investigate the vascular effects of denervation, isolated intrarenal arteries were studied using sensitive 'isometric' recording equipment. Four pieces of evidence were obtained to indicate an effective functional denervation I week after renal nerve transection: (i) Phentolamine reduced the K+-induced contraction in controls but not in denervated arteries. (ii) The K+-induced contraction was significantly smaller in denervated than in control arteries. (iii) Noradrenaline (NA) was a significantly more potent vasoconstrictor (4 x) in denervated than in control arteries. (iv) Cocaine increased the NA sensitivity in control arteries (3 x), whereas it failed to do so in denervated vessels. Vasopressin, 5-hydroxytryptamine (5-HT), NA (in the presence of cocaine), prostaglandin F2 alpha (PGF2 alpha) and dopamine (DA) produced concentration-dependent contractions in the mentioned order of potency. Denervated arteries were found to be about two to three times more sensitive to the vasoconstrictors than control arteries. Angiotensin I and II had no contractile effect in any of the vessel segments examined. Indomethacin-pretreated arteries also failed to respond to angiotensin II. Neuropeptide Y produced only weak contractions and failed to influence the NA concentration-response relationship in either control or denervated arteries. In conclusion, renal denervation caused a general supersensitivity of the vascular smooth muscle cells to both circulating and non-circulating vasoconstrictors. Our results cannot explain the increased blood loss and bleeding time seen after denervation, but rather support the view that the enhanced bleeding was caused by an interrupted vasoconstrictor influence of the sympathetic nerves.

Angiotensins↗

Urinary bladder innervation in children.

Muscle strips from the fundus, trigone, and distal ureter obtained from children at operation for vesicoureteric reflux were studied using histochemical and immunohistochemical methods, and electrical nerve stimulation in an organ bath. A rich supply of cholinergic nerves was found and the transmitter causing contraction of the detrusor muscle was regarded as being acetylcholine. The adrenergic innervation was very sparse except around the ureteric orifices. No contractile alpha-adrenoceptors could be detected but beta-receptor-mediated relaxation was found. The type was not beta 1 or beta 2, suggesting a third type of beta-receptor. Peptidergic nerves containing vasoactive intestinal peptide (VIP) were demonstrated in a few nerve terminals. No nerves containing enkephalin, somatostatin, or substance P were found. VIP affected the detrusor muscle, indicating a possible role as a modulator of transmitter action. Imipramine, used for enuresis, had no anticholinergic effect on the bladder in the doses used clinically. The anticholinergic and calcium antagonistic drug terodiline inhibited all muscle activity, making it suitable for treatment of diurnal enuresis.

Acetylcholinesterase↗

The effect of intraportal six-hydroxy-dopamine on hemorrhage after standardized liver trauma in rats.

Sympathetic denervation of the liver was accomplished by intraportal injection of 6-hydroxy-dopamine in rats. The animals were subjected to standardized liver trauma 1 week later. Blood loss and duration of bleeding from the cut raw surface were more than doubled in the denervated animals. Mean arterial blood pressure was significantly lower in the denervated animals, but stable during hemorrhage in both denervated and innervated animals. Platelet aggregation and the intrinsic coagulation system were not affected by the chemical denervation. Hepatic norepinephrine was almost eliminated by the denervation procedure. These findings suggest that the liver nerves at the porta hepatis should be preserved during hepatic resections.

Animals↗