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Biomedical subjects

J M Guys

Publications and source records attributed to J M Guys.

At least 55 records · Page 3Linked to original sources

[Kidney injuries in children].

Over the past 4 years 18 children were observed at the La Timone-Enfants Hospital in Marseille with renal lesions consequent to nonpenetrating abdominal traumas. Hematuria was reported in 14 cases (7 macroscopic and 7 microscopic) and all cases suffered from abdominal and/or lumbar pain. In relation to the diagnostic iter used: 18 echographies, 15 urographies, 13 TAC, 2 ascendant pyelographies, 2 scintigraphies with DMSA, 1 echo-Doppler and one angiography were performed. Renal lesions were classified as: 8 type 1, 5 type 2, 4 type 3, 1 type 4. Four cases were operated and one case underwent percutaneous drainage of an urohematoma. Following an analysis of these case and a revision of the literature, the authors emphasise that the evolution and progress in the diagnosis and treatment of this important aspect of traumatic pathology in children enables an almost complete recovery of kidney function in almost all cases.

Abdominal Injuries↗

Pancreatic function and congenital duodenal anomalies.

Six children operated on for congenital anomalies of the duodenum were investigated to find out if pancreatic dysfunction was associated with the duodenal malformation, even in the absence of clinical evidence of pancreatic insufficiency. None of the children had diarrhea and none requested nutritional support. Pancreatic function was assessed by enzyme activities (lipase, trypsin, and chymotrypsin) bicarbonate and calcium measurements in pancreatic juice obtained through a nasoduodenal tube under stimulation by secretin and cerulein. Results showed no significant modification in hydro-electrolytic secretion, but impairment of enzymatic secretion was seen. The physiopathological relationship between duodenal anomalies and pancreatic dysfunction is discussed.

Child, Preschool↗

[Unusual intrathoracic tumor in children: lipoma].

BACKGROUND: Intrathoracic lipomas are rare, often asymptomatic and, in this case, discovered by chance on X-ray. CASE REPORT: A 3 year-old girl was admitted because chest X-rays prescribed for a persistent cough showed a mass in the right axillary area. The CT scan showed a mass with density of -80 Hounsfield units, characteristic of adipose tissue. There was a slight rib change in contact with the mass, but radionuclide bone scan showed no area of increased uptake. A chest X-ray performed 3 years earlier had shown such a mass, but it was not recognized at that time. Surgery demonstrated that the mass was extrapleural in location and adhering to the rib which was partly excised. Histologic examination showed the presence of mature adipocytes, but no malignant cells. CONCLUSION: The fatty component of an intrathoracic tumor is easily recognized with CT scan. Erosion of bone in contact with it has been reported.

Age Factors↗

Splenic hemangioma--report of three pediatric cases with pathologic correlation.

Splenic hemangioma is a very rare neoplasm in children. We report three cases of splenic hemangioma, two of solitary hemangioma and one of hemangiomatosis. Two patients had hematologic disorders related to hemangioma. Imaging findings of this entity are presented and discussed with emphasis on pathologic correlation.

Child, Preschool↗

Surgical management of duplex ureteroceles.

We reviewed the cases of 95 children with duplex ureteroceles treated in this department over an 18-year period. There were 101 ureteroceles (6 bilateral). Diagnosis and treatment were analyzed. Special attention was paid to newborns screened in utero. We always strove to preserve functional renal tissue whenever possible. In keeping with this goal, three surgical techniques were used: (1) upper pole heminephrectomy; (2) ureterocele excision, bladder neck reconstruction, and ureter reimplantation with or without cutaneous ureterostomy of the upper pole ureter; and (3) endoscopic ureterocele incision. Follow-up studies using x-ray and radionuclide imaging demonstrated satisfactory renal function in 86.6% of patients. These findings support a conservative approach to ureteroceles using endoscopic ureterocele incision as the primary treatment. Lower urinary tract reconstruction may be associated in cases involving urinary tract infection, obstruction or incontinence. Upper pole heminephrectomy should be performed only after functional evaluation following ureterocele incision or cutaneous ureterostomy.

Adolescent↗

[Pseudo-tumoral form of urinary bilharziasis in a child].

A tumor-like form of urinary schistosomiasis is reported. Diagnosis was established intraoperatively. The finding of hematuria and of a stay in an area where schistosomiasis is endemic suggested the diagnosis but specific investigations were negative. Cystoscopy revealed a budding, hemorrhagic tumor lying against the posterior aspect of the bladder with no other lesions suggestive of schistosomiasis. The true prevalence of these tumor-like forms of urinary schistosomiasis is analyzed. Emphasis is put on the need for using several investigations to reach the correct diagnosis.

Child↗

[Drash syndrome].

The authors report one case of Drash syndrome: association of Wilms' tumor, nephropathy and genital abnormalities. The definition, prognosis and origin of this triad are discussed.

Cadaver↗

[Hydatid cyst of the kidney in the child].

Three pediatric cases of hydatic cyst of the kidney are reported. This is a very rare condition in children. Usually, the presentation is a cystic space-occupying lesion of the kidney. Several diagnostic methods are available but it is stressed that ultrasonography may be sufficient. Surgical treatment is still necessary.

Child↗

Epidemiological study of congenital diaphragmatic defects with special reference to aetiology.

Congenital diaphragmatic defects (CDD) are easily accessible to ultrasonographic diagnosis. In spite of progress in the management of prenatally detected cases, the mortality rate for CDD remains high. The prognosis depends mainly on the severity of fetal lung hypoplasia but is also linked to the associated malformations. We report on 77 cases of CDD ascertained between 1982 and 1988 from 136,161 consecutive births in the Bouches du Rhône area. The spontaneous perinatal mortality rate was 61% with 28 early post-natal deaths and 14 stillbirths. Eight pregnancies were terminated after prenatal diagnosis. The diaphragmatic defect was associated with other congenital anomalies in 33 cases, more often among stillborn (92.8%) than liveborn infants (23.6%). A chromosomal abnormality was present in 9 cases representing 11.6% of all CDD and in 27.2% of cases with other anomalies. A Mendelian disorder was present in 9 cases (eight Fryns syndrome and one Fraser syndrome). This study underlines the necessity of a systematic work up of prenatally diagnosed cases, including fetal karyotyping and analysis of associated malformations in order to adapt the management of the pregnancy and delivery to the prognosis.

Abnormalities, Multiple↗

Management of pediatric laryngotracheal stenosis.

In the past 6 years, 40 children underwent surgery for laryngotracheal stenosis; 32 by the external approach and 8 by endoscopic CO2 laser. Twenty-seven children (67%) were less than 5 years old at the time of treatment and 80% of the stenoses (n = 32) corresponded to an etiology that is secondary to endotracheal intubation and/or tracheotomy. By grading the stenoses according to the amount of narrowing of the lumen, the authors emphasize the interest of conservative treatment (endoscopic for grade I [less than 70%, n = 8], and treatment by external surgical methods for grade II [70% to 90%, n = 13], grade III [90% to 99%, n = 14], and grade IV [total obstruction, n = 5]). At this time, the most commonly used technique is laryngotracheoplasty with costal cartilage interposition. In this series, 88% of the patients were successfully decannulated. As for the treatment of stenosis in infants, the authors describe their recent experience of laryngotracheofissure in 7 patients as an alternative to either tracheotomy in cases of difficult extubation or laryngotracheoplasty when the child is underweight.

Adolescent↗

A novel technique for reconstruction of the abdominal wall in the prune belly syndrome.

There is currently widespread enthusiasm for abdominal wall reconstruction in patients with the prune belly syndrome. We have devised an operation that appears to offer some advantages over those proposed by Ehrlich and Randolph. The technique preserves the umbilicus, and thickens and strengthens the anterior abdominal wall. By narrowing the waist, it also produces a better cosmetic appearance. After full thickness resection of a varying amount of skin from the central abdomen, the anterior wall is sutured in double-breasted fashion, thus, preserving all vascularization and the umbilicus. Since 1969 we have successfully performed this procedure on 9 prune belly patients including 1 girl. The results were excellent in terms of duration and cosmetic appearance.

Abdominal Muscles↗

Esophageal atresia, tracheomalacia and arterial compression: role of aortopexy.

Between 1985 and 1990, we treated 46 cases of esophageal atresia in the pediatric surgery department in Marseille. In 17 of these patients atresia was associated with tracheomalacia. The most common respiratory manifestations were acute apneic attacks, cyanotic spells and recurring pneumopathy. The underlying cause was arterial compression of the airways by the innominate artery in 6 cases, the aortic arch in 2 cases, and a common origin of the innominate and left carotid arteries in 2 cases. Eleven patients underwent surgery to relieve arterial compression: suspension of the aorta from the under surface of the sternum (10 cases) and reimplantation of the innominate artery (1 case). Results in terms of tracheal caliber and symptomatic relief were excellent in 10 cases including 1 case requiring redo, and poor in 1 case. For diagnosis, our experience shows the importance of endoscopy, nuclear magnetic resonance and recognition of gastroesophageal reflux which is often associated (9/11). The role of tracheomalacia is discussed. On the basis of our results and those previously reported, aortopexy appears to be an effective surgical technique in patients with esophageal atresia presenting respiratory complications due to arterial compression.

Airway Obstruction↗

[The role of dialysis in the treatment of terminal renal insufficiency in children].

In order to analyze the place of dialysis in a hemodialysis/transplantation program, the duration of each treatment modality, mortality rate and quality of inclusion in the social network were studied. Complications which arose during hemodialysis were evaluated by comparing the 1970's and the 1980's. Sixty children with terminal renal failure, aged 3 to 15 years, were entered in a hemodialysis/transplantation program between May 1971 and December 1988. Patients were followed up until December 1989. Among the 47 (78%) survivors at the end of the follow-up period, 25 had a functioning renal transplant and 22 were undergoing dialysis. Among the 13 deaths, 7 occurred during renal transplantation or immediately after loss of the transplant and 6 occurred under dialysis. Mean duration of treatment, including both dialysis and transplantation, was 7 years 11 months. Mean time spent under dialysis was 4 years 9 months. Time spent with a functioning transplant was 3 years 10 months for the 46 transplant recipients. Mean time spent on the transplant waiting list fell from 3 years 6 months before 1980 to 2 years after 1980. Virtually no cases of renal osteodystrophy, acute arterial hypertension or hepatitis B were seen after 1980 as a result of the use of higher-potency vitamin D derivatives, recent antihypertensive drugs including ACE inhibitors, and the Hevac B vaccine. Similarly, safety and patient comfort during dialysis improved substantially, as well as the quality of rehabilitation. Growth remained a significant problem although improvements can be expected to occur in the near future. Hemodialysis is an indispensable complement to transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Disseminated intravascular coagulation masking neonatal hemophilia].

Most infants with hemophilia have no bleeding in the neonatal period even if birth trauma occurs. The explanation for this lack of bleeding in the first few days of life in most hemophiliacs is unknown. Maternal factors VIII and IX fail to cross the placenta and cannot, therefore, protect the neonate. There have, however, been an increasing number of reports of severe neonatal bleeding in hemophiliac neonates. Herein, a case of severe neonatal bleeding responsible for hypovolemic shock and disseminated intravascular coagulation masking the hemophilia and delaying its diagnosis is reported. Transfusion of twice the total globular mass and exchange-transfusion were required. Hemorrhagic gastric necrosis occurred, requiring subtotal gastrectomy. The diagnosis of severe hemophilia A (factor VIII = 1%) was established only at 17 days of age. At the age of five months, the child developed a dumping syndrome which improved under appropriate dietary therapy and finally resolved. Outcome was favorable and at the evaluation at two years of age the child was leading a normal life. This case underlines the difficulty of the diagnosis of hemophilia at birth. When there is no family history of bleeding, the diagnosis of hemophilia is usually missed in the neonatal period and established only later or retrospectively. Factors VIII and IX should consequently be measured in male neonates with unusual bleeding and an increased activated partial thromboplastin time, even if disseminated intravascular coagulation is present. Prompt diagnosis and initiation of specific therapy may lessen acute morbidity and prevent long-term sequelae in affected infants.

Disseminated Intravascular Coagulation↗

Multiple aneurysms in a seven-year-old child.

Arterial aneurysms, rarely encountered in childhood, pose difficult etiologic and therapeutic problems. We report the case of a seven-year-old Algerian girl with aortoiliac, visceral, and limb aneurysms revealed by a tumefaction in the left popliteal fossa. Aortic rupture subsequently occurred. The aneurysms were resected with subsequent prosthetic or vein graft replacement. Three years later, clinical and functional results of the grafts were satisfactory. Possible etiologies include inflammatory arteritis, dysplasia, or idiopathic primary aneurysms.

Aneurysm↗

[Retrospective study on abortions of malformed fetuses carried out during a 5-year period in the department of Bouches-du-Rhône].

The authors report a retrospective study of therapeutic abortions for antenatally diagnosed abnormalities, 146 fetuses are concerned during a five years period, an anatomo-pathological examination have been performed in all cases, malformations of central nervous system are the most frequent (62). 26 urologic abnormalities are especially detailed. There are 10 isolated urologic abnormalities, and 16 abnormalities multiples. All fetuses had renal lesions which could justified an intervention but the declaration at registration of congenital birth defects is not obligatory. A multidisciplinary approach to prenatal diagnosis and congenital birth must still developed for a better understanding.

Abortion, Induced↗

[Laryngotracheal stenosis in children. Current aspects of the surgical treatment and prevention].

In the past 6 years, 40 children underwent surgery for laryngotracheal stenosis; 32 by the external approach, 8 by endoscopic CO2 laser. Twenty-seven children (67%) were less than 5 years old at the moment of treatment and 80% of the stenoses (n = 32) corresponded to an etiology that is secondary to endotracheal intubation and/or tracheotomy. By grading the stenoses according to the amount of narrowing of the lumen, the authors emphasize the interest of conservative treatment (endoscopic) for grade I (less than 70%, n = 8), and treatment by external surgical methods for grade II (70%-90%, n = 13), grade III (90%-99%, n = 14), and grade IV (total obstruction, n = 5). At this time, the most commonly used technique is laryngotracheoplasty with costal cartilage interposition. In this series, 88% of the patients were successfully decannulated. As for the treatment of stenosis in infants, the authors describe their recent experience of laryngotracheofissure in 7 patients as an alternative to either tracheotomy in cases of difficult extubation or laryngotracheoplasty when the child is underweight.

Adolescent↗