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Biomedical subjects

G M Vujanic

Publications and source records attributed to G M Vujanic.

12 recordsLinked to original sources
PubMed2003

The constipated child: how likely is Hirschsprung's disease?

The incidence of Hirschsprung's disease (HD) was determined in children who presented with constipation to a specialist paediatric surgical unit. During a 5-year period, 355 rectal biopsies were performed on 182 neonates, infants and children presenting with chronic constipation or intestinal obstruction: 25 (14%) were diagnosed HD. One hundred and four patients had suction and 78 had full-thickness rectal biopsies. Haematoxylin-eosin (HE) staining and acetylcholinesterase (AChE) histochemistry was used. In 13 cases (8%) of suction and 2 cases (2.5%) of full thickness rectal biopsies, specimens were inadequate to diagnose HD. The mean age of all patients was 2.9 years and that of patients diagnosed with HD was 3.64 months. Nineteen patients with HD were diagnosed in the first month, 5 in 1-12 months and 1 at 4 years of age (Fig. 1). The authors found that along with onset of constipation convincing indications for rectal biopsy to exclude HD were as follows: those infants and children who do not pass meconium within 48 hours, have low intestinal obstruction of unknown cause, severe constipation, chronic abdominal distension and failure to thrive. A diagnostic accuracy of 94% was achieved with AChE histochemistry for suction rectal biopsy. After this review, referring paediatricians were advised that screening of other common organic causes of constipation with the least invasive investigations, including laboratory, dietary and paediatric gastroenterology advice, should be undertaken to avoid unnecessary rectal biopsy to exclude HD and related disorders.

Acetylcholinesterase↗
PubMed2001

Clinical impact of histologic subtypes in localized non-anaplastic nephroblastoma treated according to the trial and study SIOP-9/GPOH.

BACKGROUND: Histologic subtypes of standard histology Wilms' tumor (WT) and the effect of preoperative therapy on their clinical and histologic features, deserve to be analysed in respect to outcome to find an adequate baseline for therapy. PATIENTS AND METHODS: The German Society of Paediatric Oncology & Haematology enrolled patients from January 1989 to March 1994 for therapy according the International Society of Paediatric Oncology trial & study 9. Standardised preoperative therapy with dactinomycin and vincristine for 4-8 weeks was generally applied in patients between 0.5 and 16 years with localized renal tumors and imaging typical for WT. In 99.5% of cases representative material was sent for review to the Kiel Paediatric Tumour Registry. For prospective subtyping of 329 WT (258 after preoperative therapy, 71 with immediate surgery) modified Beckwith & Palmer criteria were used. Reduction in volume measured by imaging prior to chemotherapy and surgery was used to assess response (poor response: reduction < 40%; good response: reduction > or = 40%). RESULTS: There were 39% of patients treated with immediate surgery and 12.4% of patients with preoperative therapy in the age group up to 12 months. The difference in age (P = 0.022) was linked with different amounts of epithelial WT (15.5% vs. 3.1%), median age: 0.58 and 0.93 years. Due to the effect of chemotherapy the amount of other WT changed: stromal 0% to 14%, mixed 45.1% to 29.4%, blastemal 39.4% to 9.3%). After preoperative therapy 37.6% of WT were predominantly regressive, 6.6% completely necrotic. Poor response was frequent in differentiated WT (86% of stromal, 75% of epithelial WT) but none relapsed. In the other WT with viable tumor left after preoperative therapy > 70% had good response, poor response was a risk factor (P = 0.0057). CONCLUSIONS: Subtyping according modified Beckwith & Palmer can be used in WT after preoperative therapy to stratify postoperative therapy in future. A milder therapy could be tested in differentiated WT at low stages and an intensified in the others with viable tumor left and poor response, i.e., mainly blastemal WT.

Adolescent↗
PubMed2000

The treatment of Wilms' tumour: results of the United Kingdom Children's cancer study group (UKCCSG) second Wilms' tumour study.

The aims of the UKW2 study were: (1) to further refine treatment for stage I and II favourable histology (FH) patients; (2) to consolidate the UKW1 results for stage III FH patients; (3) to improve the outlook for patients with inoperable primary tumours and those patients with stage IV and unfavourable histology disease. Treatment consisted of primary nephrectomy, wherever possible, followed by chemotherapy and radiotherapy, as dictated by stage and histology. Treatment was refined successfully for stage I and II FH patients. The 4-year event-free survival for these two groups was 94% and 91%, respectively. Stage III FH patients had a 4-year event free survival of 84%. The outlook for patients with clear cell sarcoma of the kidney is as good as for patients with favourable histology, whilst that for patients with anaplastic or rhabdoid variants remains poor. The outlook for the majority of children with Wilms' tumour is now excellent.

Adolescent↗
PubMed1995

Value and quality of perinatal and infant postmortem examinations: cohort analysis of 400 consecutive deaths.

OBJECTIVES: To evaluate the contribution that perinatal and infant necropsy makes to clinical practice and to see how this might be influenced by the quality of the investigation. DESIGN: Cohort analysis, with data from the all Wales perinatal survey, of perinatal and infant deaths during 1993 of babies born to mothers usually resident in Wales. The clinicopathological classification of death based on clinical details was compared with the classification after necropsy. Similarly, cases in which necropsy yielded new information were identified. The quality of the necropsy was assessed by scoring six aspects of the examination. SUBJECTS: 400 consecutive deaths at 20 weeks of gestation to 1 year of age. MAIN OUTCOME MEASURES: Necropsy rate, effect of necropsy on clinicopathological classification, new information disclosed by necropsy, quality of necropsies, and the link between new information and quality of the necropsy. RESULTS: Necropsy was performed in 232 cases (58%). The clinicopathological classification was altered by necropsy in 29 cases (13%). New information was obtained in 60 cases (26%), and in 42 (18%) it disclosed the cause of death. The quality of necropsy was substantially higher when the main cause of death was detected than when nothing new was found. CONCLUSION: Necropsy is underused. Clinicians should be more positive about necropsies and realise how much clinically relevant information can be obtained from a good quality examination.

Autopsy↗
PubMed1994

The immunocytochemical demonstration of a relative lack of nerve fibres in the atrioventricular node and bundle of His in the sudden infant death syndrome (SIDS).

Whilst examining the variation with age of the nerve fibre content of the cardiac conduction system (CCS), using an immunocytochemical approach, it became evident that in two sudden infant death syndrome (SIDS) cases there was a selective lack of S100 positive nerve fibres in the atrioventricular (AV) node and His bundle. In the present study therefore, the examination of CCS with S100 was extended to a further five SIDS cases and three cases of sudden explained death. Also, in addition to S100--which selectively marks Schwann cells associated with both myelinated and non-myelinated nerves--PGP 9.5 (protein gene product) was used to reveal the presence of nerve axonal elements associated with the CCS. The results showed a uniform presence of S100 and PGP 9.5 positive nerve fibres in the sinoatrial (SA) node, the AV node and His bundle tissue of all three control cases. In contrast, five out of seven SIDS cases showed a uniform lack of staining with these markers in the AV node and His bundle tissue, whilst in the two remaining cases it was present in greatly diminished amounts. Staining in the SA node, although present in all seven cases, was reduced when compared with the control cases. This is the first time the CCS of SIDS cases has been studied with immunocytochemical markers of nerve elements. The overall results taken in conjunction with the epidemiology of SIDS suggest that the lack of AV node and His bundle innervation most probably reflects a delay in the development or maturation of the nerve elements of the CCS, similar to that noted for other parts of the central and peripheral nervous systems in SIDS.

Atrioventricular Node↗
PubMed1994

Systemic vasculitis complicating infantile autoimmune enteropathy.

An infant with unexplained sideroblastic anaemia and severe protracted diarrhoea due to autoimmune gut disease is reported. Despite treatment with an exclusion diet and immunosuppressive agents she developed a severe systemic vasculitic illness. Intensive treatment with plasmapheresis resulted in resolution of the vasculitic process but the child subsequently died after overwhelming septicaemia.

Anemia, Sideroblastic↗
PubMedDate not supplied

Juxtaposed cystic nephroma and Wilms' tumor.

We report a case of juxtaposed Wilms' tumor (WT) and cystic nephroma (CN) in a 21-month-old girl which gave rise to radiological diagnostic difficulty. Preoperative chemotherapy was given, resulting in marked tumor necrosis but the cystic nephroma remained untouched. Histological examination showed characteristic features of a triphasic WT and a CN; the two lesions were separated by a thick fibrous capsule. While everybody agrees that WT and cystic partially differentiated nephroblastoma (CPDN) are closely related, there are two opposite views about their relationship to CN. One is that CN may represent the final step in maturation of WT and CPDN. Other authors argue that there is no evidence to support this theory but believe CN might have something in common with nephrogenic rests. We suggest that the two lesions in the present case may have originated from two intralobar nephrogenic rests, which would strengthen the latter view.

Female↗
PubMedDate not supplied

New case of Beemer-Langer syndrome.

We present the case of a male infant born at 37 weeks gestation with multiple congenital anomalies, including hydrops fetalis, facial and visceral abnormalities, short ribs, and short limbs without polydactyly. We believe that this represents a further case of the Beemer-Langer syndrome, a relatively recently described form of lethal osteochondrodysplasia with an autosomal recessive mode of inheritance. This case also showed some less frequently described anomalies, including arachnoid cysts of the brain and short intestines.

Arachnoid Cysts↗
PubMedDate not supplied

Intrathyroidal thymic tissue: an autopsy study in fetuses with some emphasis on pathological implications.

Fifty-eight human thyroid glands obtained at autopsy from fetuses with proven retrosternal thymus were systematically studied for the presence of intrathyroidal thymic tissue. The latter was found in one thyroid lobe in each of three fetuses (5.1%). It was located in a subcapsular position in two cases (3.4%) and lying deep in thyroid tissue in one (1.7%). Our findings would support a IV-V pharyngeal pouch origin for some accessory thymic tissue and would provide an explanation of the histogenesis of intrathyroid thymomas.

Choristoma↗
PubMedDate not supplied

Cervical teratomas.

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Adult↗