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J Zeller

Publications and source records attributed to J Zeller.

At least 55 records · Page 3Linked to original sources
PubMed1994

[Persistent müllerian duct syndrome (males with uterus): a pediatric problem].

BACKGROUND: The persistent müllerian duct syndrome (PMDS) is characterized by the persistence of the uterus and Fallopian tubes in otherwise normally virilized boys. Its diagnosis is usually made during a surgical procedure for inguinal hernia or cryptorchidism. We report six recent cases of PMDS, in which we have studied anti-Müllerian hormone (AMH) serum levels. CASE REPORTS AND METHODS: Six boys including three brothers were operated on for cryptorchidism or inguinal hernia. Surgical exploration showed persistence of the uterus and Fallopian tubes in patients having normal 46, XY karyotype and male gonads. The AMH serum levels were measured by Elisa and the AMH gene by single strand conformation polymorphism of PCR products. RESULTS: The three brothers showed a mutation in the AMH gene which leads to the replacement of leucine by proline at position 70 and to a defect in AMH production. In two other patients, serum AMH values were normal, no mutation on the AMH gene was found, and end-organ insensitivity was suggested to explain the persistence of müllerian derivatives. In the last patient, although AMH serum levels were very low due to a progressive degeneration of testicular tissue, molecular analysis of the AMH gene suggested that end-organ resistance might be the cause of the persistence of müllerian ducts. CONCLUSION: PMDS is not extremely rare. Many diagnostic mistakes are made which could be prevented by performing pelvic or inguinal ultrasonography before surgical treatment of bilateral cryptorchidism or irreducible inguinal hernia. Prognosis depends upon the integrity of the testicular tissue, sometimes compromised for yet unexplained reasons, and upon the successful correction of cryptorchidism, which is complicated by the close anatomical relationship between the vasa deferentia and the Müllerian derivatives.

Adolescent↗
PubMed1994

[Neonatal hyperglycemia and diabetes mellitus].

BACKGROUND: Hyperglycemia in the neonate may be permanent or more frequently transient. Its treatment is still debated. PATIENTS AND METHODS: The files of 19 infants with hyperglycemia were retrospectively analysed. Their birth-weights were more than 900 g and their gestational ages more than 27 weeks. Plasma glucose, insulin, C-peptide, islet-cell and insulin autoantibodies were measured in each patient. RESULTS: Blood glucose ranged from 1.2 to 10 g/l (mean: 5.05 +/- 1.0), between the second hour and the 60th day of life. Four infants presented with permanent neonatal diabetes mellitus: they were small for gestational age. Hyperglycemia was noted from the first day of life. C-peptide levels were less than 0.1 pmol/ml and autoantibodies were absent. Two sibs had hypothyroidism, one patient had unclassifiable chronic diarrhea plus renal disease, the fourth patient had ventricular septal defect. The 15 other patients presented with a transient hyperglycemia that appeared lately (6.1 +/- 3.4 day of life). Hyperglycemia was induced by glucose infusion in five patients, by potentially hyperglycemic drugs in five others. C-peptide levels ranged from 0.01 to 0.76 pmol/ml (mean 0.29 +/- 0.11). One patient and his mother had insulin antibodies. Another patient had congenital heart abnormalities. Hyperglycemia required insulin therapy in 17 cases (four with permanent and 13 with transient hyperglycemia). CONCLUSION: There are no clinical or biological features permitting foresee the duration of neonatal hyperglycemia. Each patient with birth-weight below 2,500 g should be given insulin when glucosemia remains above 1.3 g/l and his weight does not increase.

Diabetes Mellitus, Type 1↗
PubMed1993

The persistent müllerian duct syndrome: a rare cause of cryptorchidism.

The persistent Müllerian duct syndrome is characterized by the retention of Müllerian derivatives in patients otherwise normally virilized. Clinically, the persistence of uterus and tubes leads either to cryptorchidism or inguinal hernia, depending on whether or not the Müllerian derivatives can be mobilized during testicular descent. The condition is usually discovered at surgery, however preoperative sonography could allow the diagnosis to be made preoperatively. The molecular basis of the persistent Müllerian duct syndrome is heterogeneous, and is reflected by wide variations in the serum concentration of anti-Müllerian hormone. Some cases are apparently due to end-organ resistance, and are associated with normal serum levels of the hormone. Others, characterized by absent or low hormone concentrations, can be explained by mutations of the gene coding for anti-Müllerian hormone, which are distributed along the whole length of the coding region.

Adolescent↗
PubMed1993

[Localizing of Langerhans islets adenoma by transhepatic portal catheterization].

BACKGROUND: The procedures used to locate pancreatic endocrine tumors have only limited success in infants and children in whom the nodules may be small. Portal catheterization may therefore be useful. CASE REPORT: A child aged 6 yrs 4 months was admitted because of several recent episodes of pallor and sweating associated with hypoglycemia. Further investigation showed moderate hyperinsulinemia and low blood levels of ketone bodies and branched amino-acids after a 15 hr fast. Celiac angiography was normal. The hypoglycemic episodes recurred despite treatment with diazoxide for 6 months. A transparietal portal catheterization was therefore performed. Selective pancreatic venous sampling showed high concentrations of insulin in two small veins draining one part of the head of the pancreas (117 and 89 microU/ml). The head of pancreas was removed 16 months later. Extemporaneous examination revealed an adenoma 0.8 cm in diameter. This patient has completely recovered, 8 months after surgery. CONCLUSION: Transparietal portal catheterization can detect pancreatic areas with high insulin secretion. It may also help the interpretation of celiac angiographs in children, as diagnosis may be obscured by the normal rich vascularity of the pancreas in these patients.

Adenoma, Islet Cell↗
PubMed1993

[Pathology of autografts of the nipple in mammaplasty. Apropos of 2 original cases].

Autologous nipple graft is a classical technique in nipple reconstruction. The authors present two cases of deferred development of specific lesions in the grafted nipple, including one historical case of Paget's disease. After reviewing the current state of knowledge concerning Paget's disease of the nipple and in the light of these two cases, the authors discuss the histogenesis of this disease and the pathology of the grafted nipple in reconstructive surgery.

Adult↗
PubMed1993

[Basal cell carcinoma of the nose].

UNLABELLED: The purpose of this study of 81 patients with basal cell carcinoma (BCC) of the nose was to present the oncological and cosmetic results of surgical treatment and compare these results with those of other possible treatments. MATERIAL AND METHOD: We report a series of 81 cases of histologically proven BCC of the nose located chiefly on the alae nasi and on the lower end of this organ; 42 p. 100 of the tumors had previously been treated and had recurred. The patients' mean age was 63 years, and the shortest follow-up was 3 years. Excision of the tumor under simple or reinforced local anaesthesia was complete in 88 p. 100 of the cases, incomplete or borderline in 12 p. 100 and systematically repeated. Extemporaneous histological examination was performed in 18 p. 100 of the cases. The operative lesion was repaired with a graft or a flap. There was no postsurgical treatment. RESULTS: The recurrence rate was 4 p. 100 with a minimum follow-up of 3 years. The cosmetic result was good in 78 p. 100 of the patients. DISCUSSION: Numerous treatments have been used against BCC of the nose, the results, advantages and disadvantages of each of these treatments are given below: 1. Cryosurgery. The problem with this method is that it is relatively difficult to perform and requires reliable operators. The cure rate is similar to that of other treatments. 2. Chemotherapy is not frequently used. 3. Electrocoagulation. Contrary to the conventional excision, this method precludes all histological controls, and the common idea of good oncological results is now being revised. 4. Radiotherapy. The recurrence rate varies from 7 to 11.8 p. 100 with fair cosmetic results. It requires numerous sessions, cannot be repeated in case of recurrence and complicates the surgical treatment. In addition, there is a long-term risk of radiodystrophy. 5. Curietherapy by local implantation of 192Iridium has a recurrence rate of 2.5 to 7 p. 100. This treatment requires hospitalization and is costly. It is indicated in cases of complex surgery, difficult conventional radiotherapy and above all, sclerodermatous BCC. 6. Surgery has a recurrence rate which varies from 3.7 to 12.6 p. 100. Recurrences are due to insufficient excision, and this underlines the value of histological control which can be done only at surgery. It is a rapid and ambulatory treatment, usually performed under local anaesthesia. The cosmetic result is good (85 to 90 p. 100) and the patients must be made aware of this preoperatively with some degree of reliability, depending on age, location, size of BCC and on the surgical technique utilized. CONCLUSION: Surgical treatment of nasal BCC is safe, effective and rapid, with good oncological and cosmetic results, and it has many advantages over the other treatments.

Adult↗
PubMed1993

[The use of actapulgite for the treatment of acute diarrhea in infants and children. Review of pediatric studies].

Epidemiological data on diarrhea due to Rotavirus are similar enough across different regions of the world to allow the pooling of clinical studies of Actapulgite for the treatment of acute diarrhea in infants and children in France and Africa. A total of 7,616 infants and children were entered into these open or placebo-controlled trials. Most patients were under two years of age, results of the analysis confirmed the antidiarrheal efficacy and safety of Actapulgite.

Acute Disease↗
PubMed1990

Non-AIDS-associated Kaposi's sarcoma (classical and endemic African types): treatment with low doses of recombinant interferon-alpha.

In the treatment of the classical and endemic African forms of Kaposi's sarcoma (KS), radiation therapy and chemotherapy have been widely used with varying degrees of success and morbidity. We here report our preliminary experiences with low doses of recombinant interferon alfa-2b (rIFN alpha-2b) in the treatment of these types of KS, non-linked to the acquired immunodeficiency syndrome (AIDS). Ten consecutive patients (eight patients with classical and two with endemic KS) with a median age of 62 years were treated long-term with 5 X 10(6) units of rIFN alpha-2b (Introna) given subcutaneously three times weekly for at least 6 months. Of the 10 patients, six presented with cutaneous disease only, and four had additional visceral involvement. After 6 months of treatment, seven of the 10 patients had a major response of the cutaneous lesions, and three patients showed stable skin diseases. Of the four patients with additional visceral disease, one patient showed a complete regression of an intramyocardial tumor involving the right atrium and ventricle, whereas in the three other patients stabilization of the visceral lesions with marked symptomatic improvement occurred. On the whole, the long-term results over a median duration of 12 months (range, 7 to 30) are also satisfactory: IFN-alpha continued to control KS in all patients. The treatment was generally well tolerated; no serious side effects were observed. Our preliminary data suggest that low-dose rIFN alpha-2b regimens are effective in classical and endemic African KS. However, further studies are needed to establish the exact role for IFN-alpha as alternative to radiation and chemotherapy.

Humans↗