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Biomedical subjects

R P Zwierstra

Publications and source records attributed to R P Zwierstra.

At least 19 recordsLinked to original sources

[Surgical treatment of lymphadenitis caused by non-tuberculous mycobacteria in children].

A retrospective study was made of 43 consecutive cases of children with the diagnosis of non-tuberculous mycobacterial lymphadenitis treated in our surgical department between 1976 and 1992. In 16 of the 42 children the diagnosis was initially missed and these children elsewhere underwent incision and drainage resulting in a chronic sinus. In 37 children the disease was localized in the head-and-neck area, while in the other 6 inguinal or axillary lymph nodes were involved. All 43 children underwent a regional lymph node dissection with a partial parotidectomy in three. In 5 children recurrent disease developed within 4 months after dissection. The recurrences were all successfully treated with additional surgery. In none of the children did the dissection result in permanent damage of nerves, especially not of the facial nerve. The cosmetic results were good but depended on the possibilities of entering the neck through a cosmetically ideal incision. Early recognition of the disease is of the utmost importance for an adequate surgical and cosmetically desirable result. An aggressive surgical therapy is advocated and will not result in permanent damage provided the surgeon has the technical skill required for neck dissections.

Child

Bilateral split hand/foot malformation and inv(7)(p22q21.3).

A boy with typical tetramelic split hands and feet is described. In addition, there was a large arteriovenous malformation of the right arm. Chromosome studies showed a pericentric inversion of chromosome 7: 46,XY,inv(7)(p22q21.3). Inspection of the extremities and chromosome studies in the parents were normal. This case confirms the suggested localisation of a locus, important for early limb differentiation, on the long arm of chromosome 7, most probably in the chromosomal region 7q21.2-7q21.3. Previously reported cases are reviewed briefly.

Child, Preschool

[Comparison of iodine scintigraphy and computerized tomography in the localization of pheochromocytoma].

A comparison is made of the results of CT scanning and MIBG scintigraphy in the localization of phaeochromocytoma. In 21 out of 24 patients with clinically diagnosed phaeochromocytoma in the University Medical Hospital, Groningen in 1983-1990, MIBG scintigraphy provided accurate localization, while in 16 out of 18 patients with phaeochromocytoma who underwent CT scanning a correct localization was obtained. False negative results were mainly present when lesions were smaller than 2 cm. There were no false positive results. It is concluded that the sensitivity of the two methods in the localization of phaeochromocytoma is about equal. The use of one of the methods rather than of both is advised.

3-Iodobenzylguanidine

[Malrotation?].

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Digestive System Abnormalities

Teratogenic effects of transplacental transfusion of heterologous antisera simulated in an experimental model using in vitro whole rat embryo culture.

The effects of the transplacental transfusion of heterologous rabbit-anti-rat antiserum (RAR antiserum) and subsequent immunological interaction on the development of 9-10 days old rat embryos (stages 8-10 somites) were studied using an in vitro whole rat embryo culture. Transplacental transfusion was simulated by the embryonic intracardiac microinjection of approximately 0.5 microliter RAR antiserum, followed by an incubation period of 24 and 48 hours. All the tested embryos survived the incubation period. Embryos taken from the incubator after 24 hours showed signs of growth retardation and axial non-rotation, a delayed closure of the neural tube and ear vesicle, and a delayed formation of the foregut. They also had a moderate number of areas with local pathogenetic cell degeneration. Embryos taken from the incubator after 48 hours demonstrated signs of growth retardation and incomplete axial rotation. The formation of the foregut and closure of the neural tube was complete, with the exception of one embryo with a persisting open neuroporus posterior. All embryos displayed a considerable number of areas with local pathogenetic cell degeneration. The intracardiac injection technique is an elegant method to test the effects of teratogens administered directly into the embryonic circulation. The results demonstrate that heterologous antisera have teratogenic potential, believed to be due to an immunological reaction, with a particular sensitivity of the neurectoderm in 9-10 day old embryos.

Abnormalities, Drug-Induced

Enterocolonic fistula due to incarcerated inguinal hernia.

A 2 1/2-year-old boy presented severely underweight and with an extremely distended abdomen. He was born prematurely and at age 6 months had an incarcerated inguinal hernia, which had been treated conservatively. Laboratory investigations at the time of presentation showed evidence of bacterial overgrowth. Barium studies revealed a fistula between the jejunum and sigmoid colon. Resection of the fistula was followed by complete recovery. We suggest that the incarcerated hernia underlies this fistula. Until now, this series of events has not been published.

Barium Sulfate

Prognosis of extrahepatic biliary atresia.

We carried out a retrospective investigation of the 89 patients with extrahepatic biliary atresia born in The Netherlands during a 10 year period. Of these 89 patients 10 had a diagnostic laparotomy only. Eight patients had an anastomosis between the proximal bile duct and the intestine, and the remaining 71 had hepatic portoenterostomies. Bile drainage was re-established in 46 (65%). After successful hepatic portoenterostomy the development of cholangitis was the most important determinant of long term survival; five year survival was 54% in the 19 patients who had cholangitis and 91% in the 27 who did not. In the whole group of 71 patients the five year survival was 47%. Seventeen patients were at least 5 years of age at the time of writing, three of whom had had liver transplantation. Three patients have cirrhosis and hyperbilirubinaemia, and the other 11 have normal bilirubin concentrations and normal or slightly raised transaminase activities. To improve these results early surgical intervention in all children with extrahepatic biliary atresia is necessary, as are better methods of prophylaxis and treatment of cholangitis.

Anastomosis, Surgical

[Cystic dilatation of the choledochus. 9 cases].

A retrospective study of 9 cases of extra-hepatic biliary cyst is presented. Prior to admission to our hospital, 5 patients had been operated upon elsewhere. This initial operation proved to be of great importance. In 4 patients initial excision of the cyst was performed resulting in complete regression of symptoms, and no further surgery was required. In 5 patients the cyst was not excised initially and cysto-enterostomy was performed. Recurrence of symptoms and complaints, with major morbidity, occurred in all but one of these patients. After several operations, only final excision of the cyst definitively suppressed the symptoms, but in one patient the cyst could no longer be excised. Cysto-enterostomy results in recurrent symptoms and cholangitis and should be avoided. Early excision of extra-hepatic biliary cysts should be performed whenever technically feasible, not only to prevent these complications but also because of the risk of malignant degeneration related to the cyst.

Adolescent

['Glands'].

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Antitubercular Agents

Maternoembryonic transfusion and congenital malformations: an experimental study using rat embryos.

In an experimental study, using an in vitro whole rat embryo culture, the effects of a maternoembryonic transfusion and immunologic interaction on the development of ten-day-old rat embryos (stages 8 to 10 somites) has been studied. Transplacental transfusion has been simulated by embryonic intracardiac microinjection of 0.1 to 0.5 microL immunologically active rat serum. After an incubation of 24 and 48 hours, respectively, the embryos were killed. All tested embryos have survived the incubation period. On microscopic examination of the tested embryos those that were taken from the incubator after 24 hours showed no signs of pathogenic cell degeneration, while the embryos that were taken from the incubator after 48 hours all had localized lesions with pathogenic cell degeneration in one or multiple major structures. The neurectoderm and endoderm seem to be the most sensitive tissues in this period of organogenesis. The results suggest that immunologic reaction to transplacental transfusion of maternal serum may lead to congenital malformations.

Animals

Nesidioblastosis and endocrine hyperplasia of the pancreas: a secondary phenomenon.

Diffuse endocrine cell proliferation (nesidioblastosis) and islet cell hyperplasia are considered causes of organic hyperinsulinism but have not been distinguished (by histometric or immunohistologic methods) from the normally variable pancreatic islet cell population during development and in adults. Therefore, in this study morphologic, immunohistologic (to detect insulin, glucagon, somatostatin, and pancreatic polypeptide), and morphometric features were evaluated in 1) normal pancreases (from fetal to adult; n = 49); 2) pancreases from patients with nesidioblastosis (n = 5); and 3) tumor-associated pancreases (TAP) from patients with insulin-producing islet cell tumors (n = 8). The study of normal postnatal development revealed that all features of fetal development remain present after birth and that the diagnosis of any diffuse endocrine disorder should therefore be based essentially on quantitative histometric parameters (total endocrine area, islet size distribution, distribution of each endocrine cell type). With these parameters endocrine cell hyperplasia was demonstrated in TAP from adults due to increased numbers of A and D cells. However, in the cases previously diagnosed as pathologic nesidioblastosis, all parameters were within the normal range. Thus, nesidioblastosis does not appear to be a pathologic entity. Careful re-examination of the pancreases, prompted by these data, revealed small islet cell tumors in three of these five cases. It is concluded that the endocrine pancreas can react rapidly, both morphologically and functionally, to changes in hormonal feedback, e.g., islet cell tumors. Therefore, the observation of a diffuse islet cell disorder in a patient with hyperinsulinism should not be considered an indication that an islet cell tumor is not present.

Adult

Surgical treatment of organic hyperinsulinism in infancy. Surgical procedure in the absence of a demonstrable insulinoma and a peroperative diagnosis of nesidioblastosis.

This study consists of a review of 5 children operated on for organic hyperinsulinism. The diagnosis could be established by demonstrating the repeated presence of elevated serum insulin levels during hypoglycaemia and/or a rapid glucose disappearance. From our experience with this series and by a review of the literature the following data are relevant to the management of these children. Because of the risk of brain damage there is no justification for prolonged diagnostic and therapeutic trials. Surgical exploration of the pancreas is indicated without delay. When during exploration no localised lesion can be found, the diagnosis "nesidioblastosis" can be established by frozen section evaluation. Whether a diagnosis of nesidioblastosis has been established or not, a 90-95% subtotal pancreatectomy has to be performed subsequently, because this operation will cure most of the children with organic hyperinsulinism, has negligible complications and preserves normal exocrine and endocrine pancreatic function. A near-total pancreatectomy has to be performed without delay in patients with recurrent hypoglycaemia caused by persisting organic hyperinsulinism after the first operation.

Adenoma, Islet Cell

Undescended testes and puberty. Orchidopexia or orchiectomy.

In 35 consecutive patients who were over 11 years of age, surgical exploration was preformed for unilateral undescended testes. To evaluate the quality of spermatogenesis all testes were biopsied. In two patients with macroscopically normal testes a malignancy was detected, one seminoma and one carcinoma in situ with micro-infiltrative carcinoma. In all patients considerable loss of spermatogenesis could be demonstrated and in all but three patients severe degenerative changes were present. On the basis of this study we would consider orchiectomy rather than orchidopexia in patients with unilateral undescended testes presenting at puberty or later.

Adolescent