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Onrust S - - 1999
Maternal mortality in developed countries does not seem to have decreased during the past decade, despite good prenatal care. Hypertensive disorders of pregnancy are the main cause of maternal mortality in most countries. In more than half of these cases, the HELLP syndrome is involved. In this article attention is ...
Clarke D - - 1998
Six people with Prader-Willi syndrome (PWS) who developed psychoses are described. Along with other literature reviewed in the present paper, the results imply an association between PWS and psychotic symptoms. Genetic studies were possible in five cases and SNRPN expression was examined in three cases. Maternal uniparental disomy and 15q11q13 ...
Haasbeek J F - - 1998
This report recounts an unusual case of compartment syndrome of the leg and foot resulting from hanging on the side of a toddler's bed in a 11/2-year-old boy. Parents apparently found the patient on the side of the bed in the morning. Presentation to hospital revealed obvious tense compartments of ...
Yokoyama W - - 1998
We report the first case of the syndrome of periodic adrenocorticotropin (ACTH) and vasopressin (ADH) discharge associated with focal glomerulosclerosis. Approximately 30 cases of this syndrome have so far been reported in Japan, but no cases associated with renal dysfunction have yet been reported. The patient, a 10-year-old Japanese boy, ...
Hoshino T - - 1998
We present a case of prune belly syndrome in a 12-week fetus whose previous anomaly scan at 10 weeks had been normal. The ultrasound diagnosis was based on the findings of a lower abdominal cystic echo caused by abnormal dilatation of the bladder. Termination was performed at 14 weeks and ...
Reade B - - 1998
There are various osseous pathologies that afflict the tarsal navicular. These range from stress fractures and coalitions to overt fractures. In certain instances there is an interruption of its vascular supply. If this occurs in childhood, it is called Koehler's disease. Spontaneous osteonecrosis of the navicular in the adult is ...
Harrison M - - 1998
A case is described in which a child appeared for evaluation with marked gingival overgrowth, facial dysmorphism, and abdominal defects consistent with prune-belly syndrome. The relationship between this case and other reports of gingival enlargement are discussed. Coincidence of the oral, facial, and abdominal abnormalities has not been previously reported.
Wilson C - - 1998
We report on two sibs with high myopia, vitreoretinal degeneration (VRD), and occipital encephalocoele or scalp lesion. We review the literature on Knobloch syndrome, discuss possible causes, and suggest a possible involvement of mesoderm in the morphogenesis. One case presents with very early onset of severe eye disease, whereas the ...
Burnstein Y - - 1998
PURPOSE: To report a case of glaucomatocyclitic crisis (Posner-Schlossman syndrome) in a child. METHOD: Case report. A 13-year-old boy presented with decreased vision, photophobia, halos, and pain in the right eye. RESULT: Findings in this child were consistent with a diagnosis of Posner-Schlossman syndrome. CONCLUSION: Glaucomatocyclitic crisis can occur in ...
Assumpcão Júnior F B - - 1998
The author described a male patient, age 13 years and 5 months, with behavioral profile meeting the criteria of the autistic syndrome by the DSM-III-R (APA, 1989) and DSM-IV (APA, 1995). After clinical and laboratory examinations, it was found that he had an abnormal karyotype, 46 XY, R(22). The need ...
McTaggart S J - - 1998
Haemolytic uraemic syndrome secondary to infection with neuraminidase producing Streptococcus pneumoniae is well recognised, but was previously considered to be rare. This case report describes the course of a 9-month-old male with pneumococcal pneumonia, T activation and haemolytic uraemic syndrome. The clinical features of three other cases treated in Southeast ...
Grossmann E - - 1998
The authors analyzed, through a review of the literature, Eagle's Syndrome which is a complex entity which presents common symptoms varying from an aching sensation in the throat to dysphagia and pain when rotating the head. Diagnosis and treatment are the main topics discussed relative to this abnormality which involves ...
Goldfarb J - - 1998
Munchausen syndrome by proxy is a form of abuse, usually of a child by a parent, in which a factitious illness is reported or produced in the child, resulting in unnecessary medical evaluations and treatments. A dramatic case of a 17-month-old infant with recurrent polymicrobial bacteremia prompted a review of ...
Pinnola G C - - 1998
Terson's syndrome is characterized by the presence of a subarachnoid hemorrhage accompanied by retinal and vitreous hemorrhage leading to a not very favorable prognosis. We describe a case with a good outcome, probably because of a early diagnosis and medical intervention. We emphasize the routine optician's check up as very ...
Recupero S M - - 1998
The association of Sturge-Weber syndrome with naevus of Ota is an infrequently reported phenomenon and there are only four previously described cases in the literature. In this paper we briefly review the literature regarding the coexistence of vascular and pigmentary naevi and present an additional patient with the association of ...
Anderson C A - - 1998
A woman developed the delusional syndrome of erotomania 4 years after subarachnoid hemorrhage from a ruptured basilar artery aneurysm. The authors review the literature on erotomania in association with neurologic and medical conditions. Although no cause-and-effect relationship can be established between the neurologic event and erotomania in this patient, the ...
Yetkin I - - 1998
The case of a 21-year-old woman with findings consistent with Costello syndrome (CS) is reported. Most previously reported cases of CS were in young children. In addition to the classic features, the patient had endocrine disturbances including secondary amenorrhea, goiter, and adrenal insufficiency. This last abnormality may be the cause ...
Witztum E - - 1998
A case report is presented of a koro-like syndrome in a 34-year-old Bedouin man living in the Negev desert in Israel. The patient's disturbance is accompanied by voyeurism and the onset appears to be related to his sexual practices. This is the first reported case among Bedouins, who are an ...
Chabchoub A - - 1998
The author report a new case of the syndrome described by Antley and Bixler. It concerns a female new-born who presents a craniosynostosis with brachycephaly, midface hypoplasia, unilateral choanal atresia, multiple joint contractures and arachnodactyly. There was no bowing of the femora and only the radius and the ulna were ...
Ruscazio M - - 1998
We present the clinical and postmortem findings of the first photographically documented case of asplenia and interrupted inferior vena cava and the anatomic findings of 5 previously reported cases. A brief review of the various hereditary patterns of visceral situs abnormalities suggests that, at least in some cases, the asplenia ...
Pandolfi C - - 1997
Vascular damage is a well known cause of hypopituitarism since Sheehan's report of postpartum pituitary necrosis; it has subsequently been reported that also sickle-cell anemia, eclampsia, pituitary apoplexy and other pathologies may induce failure of the anterior hypophysis through this mechanism. The antiphospholipid syndrome (APS) is characterized by widespread arterial ...
Viera A J - - 1997
Using an illustrative case, this report reviews the entity of primary upper-extremity deep venous thrombosis, also known as Paget-Schroetter syndrome. The entity of Paget-Schroetter syndrome is important to military physicians because of its occurrence in young, healthy, active individuals such as those in our active duty population. A review of ...
Pirayesh A - - 1997
Vasculitis in patients with the myelodysplastic syndrome (MDS) is a rare phenomenon. We describe a patient who presented with necrotic lesions of his toes, which proved to be the result of immune complex mediated vasculitis. This unusual combination of vasculitis and MDS prompted us to review the literature. Forty-four cases ...
Ak┼čit S - - 1997
We describe the clinical features of Robinow syndrome in the children of four Turkish couples. All the patients had cardinal features of this condition, such as short stature, frontal bossing, hypertelorism, short upturned nose with anteverted nares, micrognathia, mesomelic shortening of the forearms, vertebral and costal anomalies and hypoplastic genitalia. ...
Dams E T - - 1997
Investigation of the cytokine profile in a 26-year-old man, suffering from combined immunodeficiency with hypereosinophilia, revealed high levels of interleukin-4 and interleukin-5 and relatively low levels of interleukin-2 and interferon gamma, consistent with a T-helper type 2 pattern, as has been reported in Omenn's syndrome. However, some distinct clinical and ...
Stabile B E - - 1997
BACKGROUND: The 1955 landmark publication by Zollinger and Ellison characterized the gastrinoma syndrome and presented a hypothesis for the pancreatic endocrine origin of the gastric hypersecretion and dramatic ulcer diathesis. METHODS: A detailed review of the literature was conducted to ascertain the clinical and pathologic spectrum of gastrinoma reported prior ...
Kaufmann K - - 1997
Apert Syndrome is characterized by craniosynostosis, bilateral syndactyly, and midfacial hypoplasia. Although it was first described by Wheaton in 1894, it was first diagnosed prenatally only a decade ago-with only five cases reported in the literature. A sixth case is reported here. Prenatal diagnosis of Apert syndrome is reviewed.
Dabrowski G P - - 1997
Takayasu's arteritis, also known as pulseless disease, is an unusual cause of symptomatic peripheral vascular disease. It was first reported in 1908 by a Japanese ophthalmologist who described retinal arteriovenous shunts which appeared in a wreath-like distribution around the optic disc and microaneurysms of the retinal vessels in a 19-year-old ...
Ramina R - - 1997
OBJECTIVE AND IMPORTANCE: Our objective was to study the diagnosis and management of this rare condition. A review of the literature concerning chondrosarcomas related to Maffucci's syndrome is reported. Cause and management are discussed. CLINICAL PRESENTATION: We report a case of Maffucci's syndrome associated with a cranial base chondrosarcoma. To ...
Blackburn W D WD - - 1997
The term eosinophilia myalgia syndrome (EMS) was coined in 1989 after a cluster of cases with symptoms of incapacitating myalgias and eosinophilia were reported. This syndrome has been only defined as varying degrees of myalgias and peripheral eosinophilia. Case reports of EMS are protean and do not show a consistent ...
Cullen A - - 1997
The Marshall-Smith syndrome is characterised by a triad of facial dysmorphism, failure to thrive and accelerated osseous maturation. We report a further case of this rare syndrome with the unusual but previously reported complication of laryngeal hypoplasia and review the associated laryngeal anomalies that have been reported to date. Severe ...
Goh K Y - - 1997
Cloverleaf skull or kleeblattschadel usually occurs as a result of premature closure of the cranial sutures, with accompanying hydrocephalus, proptosis, cranial base and midface hypoplasia. The incidence of this abnormality is rare, with less than 130 reported cases in the literature, and is associated with various congenital syndromes. Conventional surgical ...
Chan P S - - 1997
The authors present a case of a lower leg compartment syndrome that developed after a regional chemotherapy technique was used for recurrent melanoma of the foot in a 74-year-old woman. The diagnosis was based on the results of physical examination, with confirmation by intracompartmental pressures. Prompt consultation of orthopedic surgeons ...
Stern J S - - 1997
Coincident cases of Tourette syndrome and Pervasive Developmental Disorders increasingly have been reported in the literature. Coincident cases currently number around 90. A minority of cases have been associated with cases of Tourette syndrome and tics in other family members; this has led some authors to suggest that Tourette syndrome ...
Grateau G - - 1997
We report nine cases of haemophagocytic syndrome (HS) in patients with human immunodeficiency virus infection, and review the 17 cases described to date in the literature. In 21 of 26 cases, HS developed during an advanced stage of immunodeficiency. Clinical and haematological signs are not specific in this setting, and ...
Garcia-Cruz D - - 1997
The hypertrichosis and osteochondrodysplasia syndrome is a rare entity with clinical findings including macrosomia at birth cardiomegaly. Autosomal recessive inheritance is presumed based on the report of two affected sibs born to healthy parents. Here we report on four new patients with their follow-up data, as well as on one ...
El Aun M - - 1997
We report a case of a child with symptoms and signs of Bartter's syndrome and nephrocalcinosis. This report discusses the manifestations and management of this rare disease at a very early age.
Dhodapkar K M KM Department of Pediatric and Adolescent Medicine, Mayo Clinic Rochester, MN 55905, - - 1996
In this article, we report a case of Leuconostoc bacteremia in a 7-month-old infant who had short-gut syndrome after a gastroschisis repair and who was dependent on total parenteral nutrition through a central venous catheter. The organism was initially misidentified as viridans group streptococcus. Detection of vancomycin resistance led to ...
Amoah G B - - 1996
Landry-Guillain-Barre-Strohl Syndrome (LGBS) is a rare occurrence in pregnancy with only 38 previously reported cases in the literature. A case of a 26 year old Ghanaian woman who presented at thirty weeks gestation with limb weakness, facial diplegia, dysarthria, dysphagia and CSF cytoalbuminic dissociation and who made complete recovery before ...
Akers D L DL - - 1996
Iliac vein compression syndrome is a clinical condition that occurs as a result of compression of the left iliac vein between the right iliac artery and the fifth lumbar vertebrae. Patients usually have marked edema of the left leg. We report a case of a 16-year-old man who sought medical ...
Prasad R A - - 1996
Alport's syndrome (hereditary nephritis with deafness), is an uncommon disease and is seen very infrequently in India. We report a fatal case in a young girl with characteristic ultrastructural changes in the kidney thus emphasising the exception noted in the observation that females have a better prognosis compared to males.
Jan J S - - 1996
Hepatopulmonary syndrome (HPS) is the triad of liver disease, pulmonary vascular dilatation, and abnormal arterial oxygenation. We report a case with progressive orthodeoxia and platypnea who was proven to have HPS after serial investigations, including autopsy. This 61-year-old male is a case of alcoholic liver cirrhosis without any documented cardiopulmonary ...
Sukumar S - - 1996
Although Gerstmann's syndrome has been well documented since it was characterised in the latter half of last century, there has not been much literature on it in the last few years. We present a classical case in a patient who was admitted into hospital for an unrelated problem. We conclude ...
Lowe P M - - 1996
A case of pemphigoid gestationis occurring in a 40 year old female who developed HELLP syndrome during her fourth pregnancy is reported. Seven days after emergency Caesarean section for pre-eclampsia, she developed a pruritic blistering eruption which clinically resembled pemphigoid gestationis. The diagnosis was supported by skin histology and immunofluorescence. ...
Leonard A - - 1996
Two children who had the Rothmund-Thomson syndrome and developed osteosarcoma are reported. The 10 previously reported cases are reviewed. The osteosarcomas developed at a younger age than normally expected and 66% occurred in the tibia/fibula. Four of the five patients for whom information was available showed undue sensitivity to cancer ...
Seker B - - 1996
We report a trichobezoar extending from the stomach through the entire length of the small intestine to the caecum. A case history of a 6-year-old girl with trichobezoar is described. She is the ninth patient with Rapunzel syndrome in the literature. The epidemiology, clinical features, complications, treatment and literature are ...
Gütling E - - 1996
We investigated five patients with a locked-in-syndrome (LIS) and reported the clinical, electrophysiologic, neuroradiologic, and neuropathologic findings. EEG reactivity was present in two and absent in three cases. Somatosensory evoked potentials (SEP) varied from unilaterally normal to bilaterally absent. We conclude that there is no specific pattern of SEP abnormality ...
Meese M A - - 1996
Periostitis in the athlete is a common overuse clinical entity often found in the lower extremity. An analogous overuse syndrome also may occur in the upper extremity. These 2 case reports describe the history, physical findings, radiographic changes, and treatment for periostitis of the upper extremity rarely seen in the ...
Witzum E - - 1996
BACKGROUND: Stress has been associated with the course of Tourette's syndrome (TS). METHODS: The case report presented aims to emphasize the effect a severe traumatic experience has on the expression of TS. RESULTS: Following an intense traumatic experience, combat-related, a relatively mild case of TS became intractable. CONCLUSION: The combination ...
Schepis C - - 1996
We describe two boys affected by Down syndrome (DS), who showed milia-like idiopathic calcinosis cutis (MICC). The clinical diagnosis was confirmed by histological examination. All reported cases are reviewed and compared. Syringeal structures play a significant part in the pathogenesis of this dermatosis. MICC appears to be a poorly recognized ...
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