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Peutz-Jeghers Syndrome - JMI ArticlesArticles from our collection about Peutz-Jeghers Syndrome published about childrenChildhood Title: Gastroduodenal intussusception due to Peutz-Jeghers syndrome in infancy Authors: Boseto F;Shi E;Mitchell J;Preddy J;Adams S; Journal: Pediatr Surg Int Date: 2002 Mar Abstract: A case of Peutz-Jeghers Syndrome (PJS) presenting in infancy with gastric-outlet obstruction is described. PJS may become symptomatic at any age and should be suspected when there are obstructive symptoms or gastrointestinal blood loss in a baby with a positive family history. Contrast studies and endoscopy are useful in diagnosis and surveillance. Treatment requires a combination of endoscopy and laparotomy/laparoscopy. Because of the reported cancer risk, we recommend surveillance from the age of diagnosis Link: Click for Pubmed record Title: Peutz-Jeghers colonic tumour in a newborn Authors: Al Faour A;Vrsansky P;Abouassi F;Dabbagh H;Gross P;Retbi JM; Journal: Eur J Pediatr Surg Date: 2002 Apr Abstract: The authors refer a case report of the Peutz-Jeghers tumour localised in transverse colon found during a systematic clinical examination of the 4-day-old newborn, without any signs of intestinal occlusion. The end-to-end colic resection was made. Post-operative course was without any complications. The observation is rarely due to the tumour's atypical localisation as well as its presence in the newborn Link: Click for Pubmed record Title: Gastrointestinal polyps in children: advances in molecular genetics, diagnosis, and management Authors: Corredor J;Wambach J;Barnard J; Journal: J Pediatr Date: 2001 May Abstract: Link: Click for Pubmed record Title: Feminizing Sertoli cell tumors in boys with Peutz-Jeghers syndrome Authors: Young S;Gooneratne S;Straus FH;Zeller WP;Bulun SE;Rosenthal IM; Journal: Am J Surg Pathol Date: 1995 Jan Abstract: We report the pathology findings in two cases of multicentric Sertoli cell testicular tumors in two young boys with probable Peutz-Jeghers syndrome. Four cases of such tumors occurring in boys with Peutz-Jeghers syndrome were previously reported. Each of the two boys reported in this paper had prominent gynecomastia, rapid growth, and advanced bone age. Serum levels of estradiol were markedly elevated. Anti-mullerian hormone was measured in the serum of one of the boys and was in the normal range for age. Bilateral orchiectomy was performed in each case because the neoplastic growth would most likely result in sterility, and curtailment of height potential was threatened from continued elevation of estradiol levels. Microscopically, greatly enlarged seminiferous tubules packed with ovoid Sertoli-like cells were present. Prominent eosinophilic basement membrane surrounded the tubules and intersected between the cells, forming hyalinized ovoid globules and microcalcifications. Ultrastructure revealed lamination of basement membranes surrounding adjacent cells, ovoid cells with abundant cytoplasm, and limited smooth endoplasmic reticulum. Studies of testicular tumor tissue from both cases revealed increased transcription of the aromatase cytochrome P450 gene using promoter II, the promoter directing aromatase expression in the normal ovary and testis. The levels of transcripts were comparable to corpus luteum, thus resulting in increased estrogen synthesis. Transcripts specific for placental-type aromatase promoters (I.1 and I.2) were not detected in significant levels in these tumors Link: Click for Pubmed record Title: Bilateral asynchronous Sertoli cell tumor in a boy with the Peutz-Jeghers syndrome Authors: Niewenhuis JC;Wolf MC;Kass EJ; Journal: J Urol Date: 1994 Oct Abstract: A rare type of Sertoli cell tumor with features of a large cell calcifying Sertoli cell tumor and a sex cord tumor with annular tubules developed in a boy with the Peutz-Jeghers syndrome. A similar tumor had been removed from the contralateral testicle 9 years previously. The clinical and pathological findings in our case are compared to 3 similar cases reported in the literature Link: Click for Pubmed record Title: Peutz-Jeghers syndrome in children: report of two cases and review of the literature Authors: Tovar JA;Eizaguirre I;Albert A;Jimenez J; Journal: J Pediatr Surg Date: 1983 Feb Abstract: In this article, we report two new cases of Peutz-Jeghers syndrome in children and review the literature over the past twenty years. This series of 70 cases demonstrates that the clinical pictures observed in children are similar to those of adults. Rectal prolapse or extrusion of polyps can be the clue to the diagnosis at an early age, even in the absence of pigmentation, which can appear later. Gastroduodenal polyps were strikingly frequent in the less than or equal to 16-yr-old group (62%) a circumstance that can create operative difficulties. Five out of the 70 patients (7.14%) had tumors during childhood (two gastrointestinal adenocarcinomas, two ovarian and one testicular neoplasms). A higher risk of tumor development in these patients does exist either as a result of degeneration of the polyps or of a genetic predisposition. Whenever operation becomes necessary, a very cautious approach must be advised in order to preserve as much intestinal length as possible in these patients, who have a lifelong disease which may require repeated operations Link: Click for Pubmed record Title: Jejunal carcinoma in a child Authors: Cordts AE;Chabot JR; Journal: J Pediatr Surg Date: 1983 Apr Abstract: A case of Peutz-Jeghers Syndrome in an 8-yr-old girl with an adenocarcinoma arising in the duodenum and jejunum is herein reported with a 9-yr follow-up. This carcinoma fulfills histologic criteria for malignancy in the Peutz-Jeghers Syndrome. We suggest that this represents a documented case of malignancy arising in a Peutz-Jeghers polyp Link: Click for Pubmed record Title: Peutz-Jeghers polyps causing colocolic intussusception in infancy Authors: Howell J;Pringle K;Kirschner B;Burrington JD; Journal: J Pediatr Surg Date: 1981 Feb Abstract: Link: Click for Pubmed record Title: Sporadic Peutz-Jeghers syndrome in early childhood: a diagnostic dilemma Authors: Yosowitz P;Hobson R;Ruymann F; Journal: Am J Dis Child Date: 1974 Nov Abstract: Link: Click to read the article Title: PEUTZ-JEGHERS SYNDROME IN CHILDHOOD: UNUSUAL RADIOLOGICAL FEATURES Authors: G. KEEN, M. A. MURRAY Journal: BRITISH MEDICAL JOURNAL Date: 1962 Abstract: FAMILIAL INTESTINAL POIYPOSIS, ASSOCIATED. WITH A CHARACTERISTIC CIRCUMORAL SPOTTY PIGMENTATION, WAS FIRST RECOGNIZED BY PEUTZ (1921) IN HOLLAND. JEGHERS ET AL. (1949) DESCRIBED A FURTHER 10 CASES, AND DORMANDY (1957) MADE AN EXTENSIVE REVIEW OF THE LITERATURE, AFTER DESCRIBING 21 CASES OF THE CONDITION IN FIVE FAMILIES. Link: Click to read the article Title: GASTROINTESTINAL POLYPOSIS WITH MUCOCUTANEOUS PIGMENTATION IN CHILDREN (PEUTZ-JEGHERS SYNDROME) Authors: JAMES E. WENZL, LLOYD G. BARTHOLOMEW, GEORGE A. HALLENBECK Journal: PEDIATRICS Date: 1961 Abstract: THE ASSOCIATION OF MUCOCUTANEOUS PIGMENTATION WITH INTESTINAL POLYPOSIS WAS FIRST REPORTED BY PEUTZ IN 1921. THE CLINICAL SIGNIFICANCE OF THIS ENTITY WAS NOT WIDELY APPRECIATED UNTIL 1949, WHEN THE EXCELLENT SUMMARY BY JEGHERS ET AL. APPEARED IN THE AMERICAN LITERATURE. Link: Click to read the article Title: DUODENAL POLYPOSIS ASSOCIATED WITH MUCOCUTANEOUS MELANOSIS (PEUTZ-JEGHERS SYNDROME) Authors: ARTHUR S. TUCKER, ROBERT P. BOLANDE Journal: AMERICAN JOURNAL OF DISEASES OF CHILDREN Date: 1960 Abstract: INTESTINAL POLYS ARE RELATIVELY COMMON IN CHILDREN, BUT THOSE WHICH OCCUR IN CONJUNCTION WITH MELANOTIC PIGMENTATION AT THE RNUCOCUTANEOUS JUNCTIONS ARE SEEN MUCH LESS FREQUENTLY. PEDIATRICIANS WHO EXAMINE CHILDREN WITH INTESTINAL BLEEDING AND FIND SMALL FOCI OF DARK PIGMENT ON THE LIPS AND ADJACENT CUTANEOUS AND MUCOSAL SURFACES SHOULD BE ALERT TO THE POSSIBILITY OF THE PRESENCE OF INTESTINAL POLYPS AS REPORTED BY PEUTZ, IN 1921, AND BY JEGHERS, IN 1944. Link: Click here to read the article
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Jeghers Medical Index, St. Elizabeth Health Center, 1044 Belmont Ave., Youngstown, OH 44501
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