Skeletal muscle pathology in endurance athletes with acquired training intolerance.

BACKGROUND: It is well established that prolonged, exhaustive endurance exercise is capable of inducing skeletal muscle damage and temporary impairment of muscle function. Although skeletal muscle has a remarkable capacity for repair and adaptation, this may be limited, ultimately resulting in an accumulation of chronic skeletal muscle pathology. Case studies have alluded to an association between long term, high volume endurance training and racing, acquired training intolerance, and chronic skeletal muscle pathology. OBJECTIVE: To systematically compare the skeletal muscle structural and ultrastructural status of endurance athletes with acquired training intolerance (ATI group) with asymptomatic endurance athletes matched for age and years of endurance training (CON group). METHODS: Histological and electron microscopic analyses were carried out on a biopsy sample of the vastus lateralis from 18 ATI and 17 CON endurance athletes. The presence of structural and ultrastructural disruptions was compared between the two groups of athletes. RESULTS: Significantly more athletes in the ATI group than in the CON group presented with fibre size variation (15 v 6; p = 0.006), internal nuclei (9 v 2; p = 0.03), and z disc streaming (6 v 0; p = 0.02). CONCLUSIONS: There is an association between increased skeletal muscle disruptions and acquired training intolerance in endurance athletes. Further studies are required to determine the nature of this association and the possible mechanisms involved.

Hepatoblastoma--evolution of management and outcome and significance of histology of the resected tumor. A 31-year experience with 40 cases.

BACKGROUND/PURPOSE: The aim of this study was to retrospectively evaluate and compare the clinical features, treatment strategy, pathology, and outcome of all patients with hepatoblastoma treated at an African hospital over a 31-year period (1970 to 2001). METHODS: Forty patients with hepatoblastoma were divided into 3 groups according to the treatment given. Group I (1970 to 1983, 14 patients) had no protocol therapy; group II (1984 to 1988, 6 patients) received protocol treatment according to Children's Study Group (CCSG) guidelines; group III (1989 to 2001, 20 patients) received SIOPEL protocol therapy. All available clinical, surgical, radiologic, and pathologic data were reviewed and analyzed. RESULTS: Overall patient survival was as follows: group I, 14%; group II, 50%, and group III, 80%. Deaths in group II were caused by chemotherapy-induced immunosuppression only. Prognostic data for group III showed that all tumor-related deaths could be predicted by identifying multifocal disseminated growth patterns (P =.001) or vascular invasion (P =.001) in resected tumors. Of the 40 diagnostic tumor biopsies performed, 2 significant complications (1 death, 1 intraperitoneal tumor seeding) occurred. Histologic criteria evaluating these biopsies were not predictive of overall survival. CONCLUSIONS: The introduction of protocol therapy has resulted in a marked improvement in survival. Immunosuppression-related sepsis in our setting resulted in unacceptable mortality in patients treated according to CCSG guidelines. A diagnostic biopsy in hepatoblastoma is of value but not without complications. Preoperative chemotherapy followed by complete surgical excision according to International Society of Paediatric Oncology guidelines yields excellent results with a current survival rate of 80%.

Interdigitating dendritic cell sarcoma: a report of four paediatric cases and review of the literature.

AIMS: To report a series of four paediatric cases of interdigitating dendritic cell sarcoma (IDCS) and add to the known extranodal sites of occurrence for this tumour. Neoplasms derived from interdigitating dendritic cells are rare, with only 33 cases being reported in the literature (Medline search). These tumours usually occur in lymph nodes in the adult population. METHODS AND RESULTS: The patients were a 10-year-old girl with a large soft tissue mass bulging into the left chest, a 12-year-old girl with a right paraspinal mass, a 21-month-old boy with generalized lymphadenopathy and hepatosplenomegaly and a 6-year-old girl with a large bladder mass. Paraffin blocks and haematoxylin and eosin slides were available in all cases. In addition, immunohistochemistry and electron microscopy were performed. A diagnosis of IDCS was made in all cases. CONCLUSION: The diagnosis of IDCS can rarely be entertained on clinical information alone. Microscopically, there is a wide spectrum of features. Thus, immunohistochemistry and electron microscopy are crucial in making the diagnosis. The differential diagnosis includes inflammatory pseudotumour, follicular dendritic cell sarcoma, true histiocytic lymphoma, malignant Langerhans cell histiocytosis, anaplastic large-cell lymphoma, melanoma, and a range of sarcomas. IDCS displays aggressive behaviour and approximately half of the patients die of the disease.

An enteric duplication cyst of the pancreas causing abdominal pain and pancreatitis in a child.

A rare case of a gastric duplication in the tail of the pancreas in a child presenting with recurrent abdominal pain and evidence of pancreatic calcification suggesting pancreatitis was cured by excision of the cyst and adjacent pancreas. Congenital causes of pancreatitis are rare, but are curable with surgery. These lesions are often misdiagnosed, and patients may be subjected to inappropriate surgery. Imaging is sensitive in the detection of such lesions, but the lack of specific features necessitates an index of suspicion. Similar cases have been described previously, but the pancreatic tail is rarely involved.

Improved prognosis of Epstein-Barr virus associated childhood Hodgkin's lymphoma: study of 47 South African cases.

AIM: To study the distribution of Hodgkin's lymphoma in South African children and report the incidence of Epstein-Barr virus (EBV) as regards age, race, sex, and histological subtype; to investigate whether EBV is relevant to survival. METHODS: Immunohistochemistry (IHC) and in situ hybridisation (ISH) to detect EBV were performed on 47 South African children with classical Hodgkin's lymphoma, ranging in age from 3 to 14 years and coming from different ethnic backgrounds. The correlation between the presence of the virus and clinical outcome was assessed. RESULTS: The nodular sclerosing subtype predominated, comprising 89% of cases; the remaining 11% were of the mixed cellularity subtype. EBV was present in 68%. Full clinical data were available for 36 cases; EBV positive patients presented with less aggressive symptoms at diagnosis and had a significantly longer median survival than EBV negative patients. CONCLUSIONS: The distribution of EBV in South African childhood Hodgkin's lymphoma follows a pattern intermediate to that of industrialised and non-industrialized countries. Furthermore, our data suggest that there is an association between poor prognosis and the non-detection of EBV products in South African childhood Hodgkin's lymphoma.

Intrahepatic fetus-in-fetu: a case report and review of the literature.

The authors report the third case since 1964 of fetus-in-fetu presenting at Red Cross Children's Hospital. A fetal liver abnormality was detected during routine antenatal ultrasound scan, and the postnatal diagnosis of an intrahepatic, multiple fetus-in-fetu was made with abdominal ultrasound scan, plain radiograph of the abdomen and computed tomography. The mass was removed surgically by hemihepatectomy when the patient was 4 months of age. Imaging and the macroscopic and microscopic appearances of the abnormality confirmed the diagnosis and amplified it to include an adjacent teratoma. Multiple small subcapsular and perimass vascular hamartomas were coincidentally discovered in the liver. Two of the 3 nodules within the amniotic-lined cavity of the intrahepatic mass individually comply with the accepted criteria for fetus-in-fetu, and the third nodule has the accepted features of a benign teratoma. The existence of multiple fetus-in-fetu and fetus-in-fetu combined with a teratoma, is recorded in the medical literature. The imaging as well as the macroscopic and microscopic features are illustrated.

Sacrococcygeal germ-cell tumours--the Red Cross War Memorial Children's Hospital experience, 1980-1996.

OBJECTIVE: To document the experience of Red Cross War Memorial Children's Hospital in the treatment of sacrococcygeal germ-cell tumours. PATIENTS: Twenty-seven patients with sacrococcygeal germ-cell tumours were treated in our hospital from 1980 to 1996. DESIGN: A retrospective review of these patients' records was undertaken. RESULTS: There were 19 female and 8 male patients. Seventeen (63%) presented in the neonatal period, 13 on the first day of life. Complete surgical resection of the tumour was achieved in all patients with mature or immature teratomas (20 patients) and in 2 neonates with malignant tumours. The first of these 2 neonates, with a malignant teratoma, was not given chemotherapy and remains well 10 years later. The second, with a yolk-sac tumour, also received no initial chemotherapy. He relapsed at the age of 9 months and was successfully treated with repeat excision and chemotherapy. All 5 patients first diagnosed after the age of 1 year had malignant tumours. These patients had incomplete surgical resection (3) or biopsy only (2), and 3 were successfully treated with chemotherapy. One patient relapsed with yolksac tumour after initial complete resection of a mature teratoma. She was successfully treated with repeat surgery and chemotherapy.

The 'worn-out athlete': a clinical approach to chronic fatigue in athletes.

Chronic fatigue in the athletic population is a common but difficult diagnostic challenge for the sports physician. While a degree of fatigue may be normal for any athlete during periods of high-volume training, the clinician must be able to differentiate between this physiological fatigue and more prolonged, severe fatigue which may be due to a pathological condition. As chronic fatigue can be the presenting symptom of many curable and harmful diseases, medical conditions which cause chronic fatigue have to be excluded. The clinician must then be able to differentiate between chronic fatigue associated with training or chronic fatigue from other medical causes, and also between the chronic fatigue syndrome and the overtraining syndrome. Once the clinician has excluded all of the above medical conditions which cause chronic fatigue in athletes, a significant proportion of fatigued athletes remain without a diagnosis. Novel data indicate that skeletal muscle disorders may play a role in the development of symptoms experienced by the athlete with chronic fatigue. The histological findings from muscle biopsies of athletes suffering from the 'fatigued athlete myopathic syndrome' are presented. We have designed a clinical approach to the diagnosis and work-up of the athlete presenting with chronic fatigue. The strength of this approach is that it hinges on the participation of a multidisciplinary team in the diagnosis and management of the athlete with chronic fatigue. The athlete, coach, dietician, exercise physiologist and sport psychologist all play an important role in enabling the physician to make the correct diagnosis.

Pluripotential melanoblastoma, a unifying concept on malignancies arising in congenital melanocytic nevi: report of two cases.

Congenital melanocytic nevi are benign lesions present at birth and considered to be caused by a maldevelopment of the neural crest. The malignant potential of the congenital melanocytic nevi have been extensively addressed by several authors, and malignant melanoma is the most frequent neoplasm arising in these lesions. The present report describes two patients with congenital melanocytic nevi in which malignant melanoma with undifferentiated areas showing rhabdomyoblastic differentiation developed. The findings suggest that these mixed neoplasms may be recapitulating the differentiation potential of the ectomesenchyme-neural crest cells. We advocate the term "melanoblastoma" when referring to them.

Congenital stenotic arteriopathy with medial dysplasia of aorta, pulmonary artery, and their major branches.

This report describes the morphological findings in a young child with congenital stenotic arteriopathy who died suddenly following arteriography. Hyperplasia of all of the medial components had produced severe thickening of the wall of the aorta (mean number of lamellar units = 133 in the thoracic aorta and 125 in the abdominal aorta), the pulmonary artery, and their major proximal branches, resulting in significant luminal narrowing. Bilateral renal artery stenosis, attributable mainly to intimal longitudinal smooth muscle hyperplasia associated with fibroelastosis, was the cause of her systemic hypertension. The left ventricle showed healed subendocardial infarction.

Idiopathic annular submitral aneurysm associated with Takayasu's aortitis. A report of two cases.

The pathologic findings in two patients with idiopathic annular submitral left ventricular aneurysms and coexistent Takayasu's aortitis are documented. The evidence of chronic persistent myocarditis in one patient and marked myocardial fibrosis in the other supports two theoretical possibilities: first, the aneurysms may have an inflammatory etiology and, second, a common inflammatory process may have accounted for both the myocardial and the aortic lesions.

Hydatid infestation of the brain: difficulties with computed tomography diagnosis and surgical treatment.

The radiological, surgical and pharmacological management of 11 children with cerebral hydatid disease is presented with special emphasis on the varying CT and MRI appearances and the surgical difficulties we have encountered. All but 2 had enhanced CT scanning and 2 had an MRI. The typical CT appearance of a large non-enhancing cyst of CSF density with minimal oedema was only seen in 3 children. Atypical appearances included irregularity of the cyst wall contour (2), enhancement of the surrounding rim (3), isodensity or heterogeneity of the cyst content (4), surrounding oedema (4) and globular as opposed to curvilinear calcification (2). Complete intact cyst removal was achieved in 3 patients. The reasons for puncturing the cysts or rupturing them at operation were failure to make a definitive pre-operative diagnosis (5), dense adhesions to the skull, dura or falx (3) and the misdiagnosis of an arachnoid cyst and the subsequent placement of a cystoperitoneal shunt (1). Pathological examination suggests that the degree of the inflammatory response to the ectocyst may determine the enhancement characteristics and the ease of surgical removal. There was 1 recurrence which responded well to four 28-day treatment cycles of albendazole.

Morphologic features of the myopathy associated with chronic renal failure.

Our previous studies indicate that impaired function of skeletal muscle limits the exercise tolerance of patients with end-stage renal failure who are either maintained on dialysis or undergo renal transplantation. To study the morphology of the condition, muscle biopsies were performed on eight patients with renal failure-associated myopathy. Control samples were taken from seven healthy athletes undergoing knee surgery and from five otherwise healthy but untrained subjects. Tissues were examined by routine light and transmission electron microscopy. Histochemical staining of frozen sections for myosin adenosine triphosphatase and quantitative computer-assisted morphometry of the fiber type and size was performed. The mean (+/- SD) size for type I fibers in patients was 61.2 +/- 11.8 microns, while type II fibers measured 46.7 +/- 11.4 microns. The mean percentage of type II fibers was 67% +/- 12%. These values are within the normal population range and were not different from controls. Significant changes were found on light microscopy of patient samples. These included fiber splitting, internalized nuclei, nuclear knots, moth-eaten fibers, fiber degeneration and regeneration, increased content of lipid droplets, and fiber-type grouping. Electron microscopy showed a large variety of nonspecific abnormalities, including mitochondrial changes, Z-band degeneration, myofilament loss, and accumulation of intracellular glycogen. Ten of 12 control subjects showed no such changes; minor abnormalities were noted on both light and electron microscopy in the remaining two subjects. Muscle oxidative capacity (19.5 +/- 5.1 microL O2/min) for patients with end-stage renal failure was not different from values for those who had undergone renal transplantation, but was lower than values found in trained athletes.(ABSTRACT TRUNCATED AT 250 WORDS)

Colorectal carcinoma in children.

An increasing incidence of colorectal carcinoma has been noted at this institution. We report seven children with colorectal carcinoma. The average delay between onset of symptoms and diagnosis was 41/2 months, and in five patients distant metastases were present at the first operation. Initial symptoms were ignored in all cases and in only one was the serious nature of the condition realized at first presentation. In five lesions the histology was mucin secreting adenocarcinoma, a poor prognostic variant. All seven died on average 11 months after diagnosis. These three factors--delay in diagnosis, advanced stage of disease, and poorly differentiated histology--contribute overall to a poor prognosis in the young.

The congenital bregmatic dermoid: an African cyst?

Thirty-five children with bregmatic dermoids were operated on at the Red Cross War Memorial Children's Hospital and Groote Schuur Hospital between 1969 and 1990. Most children were under 1 year of age; 20 were males and 15 females. Thirty-one were African or of African descent, two were Indian and two Caucasian, an ethnic distribution which roughly approximates the ethnic ratio of patients attending our hospital. Most were investigated with skull X-rays and CT scan, but MRI was performed where further clarity was needed. Other lesions mimicking bregmatic dermoids included four haemagiomas, two hamartomas, two lipomas and one encephalocoele through the anterior fontanelle. One bregmatic lipoma was associated with a lipoma of the corpus callosum. All bregmatic cysts were removed through a simple elliptical incision and on connection with the central nervous system or its coverings was encountered in any patient. The pathology in all specimens reviewed (75%) revealed skin appendages to be present in the cyst wall. No epidermoids were found in this series. This series is the largest to date and adds another 31 patients of African descent to the accumulated literature which suggests that although universally distributed, the bregmatic dermoid still appears to be more common in Africans or their descendants.

The role of chorioamnionitis and prostaglandins in preterm labor.

Spontaneous preterm labor remains a significant cause of high morbidity and mortality in the newborn. Chorioamnionitis with an associated rise in prostaglandins (PGs) is thought to be one of the factors responsible for the onset of preterm labor. In this study, 52 placentas and membranes from gestations of 35 weeks' or fewer were collected. Tissue samples of membranes and placentas were incubated in pseudoamniotic fluid, and the levels of PGs and leukotriene B4 were assayed. Swallowed amniotic fluid aspirated from the infants' stomachs was analyzed for PGs and examined microscopically for the presence of degenerate neutrophilic polymorphonuclear leukocytes (pus cells) and bacterial organisms. Prostaglandins E and F and leukotriene B4 production were significantly higher in the membranes and placentas with chorioamnionitis than in those without. Although the comparison of PG levels in the gastric fluid of infants with and without chorioamnionitis did not reach statistical significance, there was a trend toward higher levels of PGs with chorioamnionitis. Prostaglandin E levels from membranes and placentas and PGF from placentas were significantly elevated in the gastric fluid of infants with pus cells and organisms. These findings support the hypothesis that chorioamnionitis may initiate preterm labor.