Cranial chordomas are extremely rare in childhood with only 25 cases having been reported in the first decade of life. A 6-year-old female child with cranial chordoma is reported. Literature on the subject is reviewed, with special reference to the management, histopathological features and prognosis in childhood chordomas as compared ...

Few chemicals in the environment have been implicated as causes of isolated thrombocytopenia, and the evidence is usually less than convincing because the patients were not rechallenged with the chemical in vivo. In the present paper, a child is reported with the onset of thrombocytopenia in temporal association with environmental ...

The use of prostacyclin infusion in thrombotic thrombocytopenic purpura is consistent with the hypothesis that patients may lack a plasma factor stimulating prostacyclin production. However, prostacyclin therapy, alone or in combination with aspirin, dipyridamole, steroid and plasmapheresis, failed in many cases. We here describe the case of a patient who ...

We report a case of pauci-immune, necrotizing and crescentic glomerulonephritis in a 10-year-old child initially thought to have Henoch-Schönlein purpura. The diagnosis of a Wegener's granulomatosis-microscopic polyarteritis disorder was made on the basis of clinical presentation and a positive anti-neutrophil cytoplasmic autoantibody (ANCA). This case illustrates the usefulness of the ...

Six cases of malignancy-associated thrombotic microangiopathy and eight cases of idiopathic microangiopathy have been studied by renal biopsy. All patients of both groups had mild to severe renal impairment and microangiopathic hemolytic anemia. The renal lesions were histopathologically identical in the two groups. The most characteristic abnormalities were glomerular mesangiolysis ...

The association between thrombotic thrombocytopenic purpura (TTP) and autoimmune hematological conditions is reported in 2 patients. In a 35-year-old man, acute autoimmune hemolytic anemia (AIHA) was diagnosed in 1960; until 1965 he was free of disease, when he abruptly developed TTP and failed to respond to blood transfusions and corticosteroids. ...

Henoch-Schonlein purpura is a clinical diagnosis, based on a presentation including rash, arthralgia/arthritis, and abdominal pain, often with renal complications. A rare complication is intussusception, presumably initiated by an edematous vasculitic leading point in the small bowel's mucosal surface. A case is described involving a 3-year-old male, with a brief ...

This report describes two cases of Henoch-Schönlein purpura with unusual lymph node pathology. The patients presented with severe colicky abdominal pain mimicking acute appendicitis but a normal appendix was removed in each case. Enlarged mesenteric lymph nodes were removed, with a clinical suspicion of Yersinia infection. Cultures and serology were ...

The case of a 20-year-old girl who had morphea with bullous lesions is reported. Two bullae appeared on an indurated patch of morphea on the right side of the trunk. A half-moon-shaped lesion was localized along the superior border of the patch and the other followed the anterior border and ...

In 1987, Washington became the first state to require that infection with Escherichia coli serotype O157:H7 be reported. In the first year of surveillance, 93 cases were reported, yielding an annual incidence of 2.1 cases per 100,000 population. The median age of case patients was 14 years (range, 11 months ...

Thrombotic microangiopathy (TMA) is a serious toxicity associated with a small number of antineoplastic agents. A case report of a patient with probable cisplatin and bleomycin-induced TMA is presented. The basic triad of symptoms that occurs in the TMA syndrome include microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. Cardiovascular, pulmonary, ...

Charts from 2929 consecutive parturients were reviewed. Twenty-four had platelet counts less than 100,000/microL in the peripartum period. Seventeen of the 24 had predisposing causes for thrombocytopenia, including preeclampsia (nine), immune thrombocytopenia purpura (two), infection (three), placenta accreta (one), abruption (one), and excessive surgical bleeding (one). Seven had asymptomatic thrombocytopenia ...

An association between bullous pemphigoid and internal malignant neoplasms has been reported by many authors, primarily in case report formats. Stone and Schroeter, however, compared cases of bullous pemphigoid with age and sex-matched control patients with contact dermatitis and psoriasis and found no evidence of an increased association between malignant ...

ABPA is more common than was once suspected, while it remains likely that ABPF is rare. The spectrum of ABPA ranges from mild asthma to end-stage fibrotic lung disease. The episodes of chest roentgenographic infiltrates may be associated with little symptomatology and their detection is made by serial measurement of ...

A case of reticular erythematous mucinosis associated with chronic idiopathic thrombocytopenic purpura and circulating immune complexes is described. We compare reticular erythematous mucinosis with the similar plaquelike cutaneous mucinosis. We discuss the apparent photosensitivity of reticular erythematous mucinosis and its possible relationship with altered states of immune function such as ...

Hypergammaglobulinemic purpura is a rare syndrome characterized by long-standing recurrent purpura of the lower limbs, polyclonal hypergammaglobulinemia, increased erythrocyte sedimentation rate and high titer rheumatoid factor positivity. The syndrome can occur isolated or in association with a systemic disease. Few cases have been described in childhood. We report two cases ...

It is hoped that recently released low osmolality radiocontrast agents (RCAs) will have a lower nephrotoxic potential, since dye nephropathy may be related to hyperosmolality. We report here 2 cases of typical oliguric dye nephropathy in patients without risk factors for dye nephropathy. These cases show that these new agents ...

The cases of seven patients between the ages of seven days and three years with purpura fulminans have been reviewed. Causative agents in these new-borns were Escherichia coli, Enterobacter, and Staphylococcus; in older children they were varicella and E. coli. The clinical findings, medical and surgical interventions, and outcomes of ...

Intracranial haemorrhage (ICH), a rare complication of idiopathic thrombocytopenic purpura (ITP), described only once previously in an adult, is usually fatal. We report a previously healthy 26 year old woman with chronic ITP in whom spontaneous ICH developed. The eventual favourable outcome in this case despite severe initial neurological deficit ...

This case studies the clinical, laboratory, and pathologic findings observed in thrombotic thrombocytopenic purpura (TTP). Although TTP is a well-recognized syndrome, it is frequently undetected antemortem and represents a rare cause of sudden death. We recently were involved in a case of TTP in which an 18-year-old woman with no ...

Simulium bite reaction is described; the lesions were present mainly on the lower legs as palpable purpura, which persisted for 2 weeks with marked oedema and considerable discomfort. Simuliids are tiny bloodsucking flies, popularly known as blackflies or buffalo flies, belonging to the dipterous family Simuliidae. They occur world-wide, breed ...

A new case of a preeclamptic who developed the diagnostic criteria for the hemolytic uremic syndrome and who was treated with plasmapheresis is presented. Fourteen case reports of gravidas with preeclampsia treated by exchange plasmapheresis with fresh frozen plasma for maternal indications are reviewed. Only four cases had been reported ...

Sporadic cases and occasional outbreaks of hemorrhagic colitis recently have been associated with the rare Escherichia coli serotype O157:H7, which is now recognized as an important identifiable cause of bloody diarrhea in patients in whom more common gut pathogens cannot be detected. The authors report such a case in a ...

Eighteen therapists-in-training working with outpatient transfer cases were interviewed by the author. Seven major factors which impacted upon the trainees' treatment of these cases were identified. Two case studies are presented to illustrate the interactive nature of these factors. The implications for supervision and future research are discussed.

There have been a few reports suggesting the association between glomerulonephritis (GN) and ankylosing spondylitis (AS). The reported glomerulonephritides include IgA nephropathy, mesangial proliferative GN and membranous nephropathy. From January 1983, through December 1984, we observed 5 cases of GN among 116 cases of definite AS. Three of them were ...

There are only a few reports of the association of immune-complex glomerulonephritis with bullous pemphigoid. The occurrence of immune-complex nephritis with linear IgA bullous disease has not been previously reported. We report two cases, one a case of bullous pemphigoid and the other a case of linear IgA bullous disease, ...

We report a case of mesangial glomerulonephropathy associated with decreased circulating C4 in a young man with recurrent microscopic hematuria and one null gene at the C4B locus. Mesangial deposits moderately reactive with anti-C4 and weakly reactive with anti-C3 and anti-IgA were found on renal biopsy. No evidence was found ...

We report a case of a sibling pair with neonatal alloimmune thrombocytopenic purpura. Serial antepartum platelet alloantibody quantitation by an enzyme-linked immunoabsorbent assay revealed rising antibody titers during advancing gestation. We discuss the implications of this finding in the antepartum diagnosis of neonatal alloimmune thrombocytopenic purpura, a rare, but frequently ...

Linear IgA bullous dermatosis (LABD) can mimic bullous pemphigoid (BP) and/or dermatitis herpetiformis (DH) both clinically and histologically. LABD, however, can be distinguished from BP and DH by direct immunofluorescent (IF) demonstration of linear IgA deposits along the basement membrane zone. A retrospective study of 234 cases of BP, 27 ...

Two patients who developed thrombocytopenia while on Tiopronin and gold salts respectively were HLA typed. Their common haplotype was A25(10), B8, DR3. A survey of the literature showed that the association between DR3 and the sudden onset form of thrombocytopenia is striking. A genetic predisposition, besides other unknown factors, seems ...

We present the rare occurrence of an inhibitor of factor VIII procoagulant arising in a patient with mild haemophilia A and rheumatoid arthritis. The inhibitor was transient and behaved like a low titre, type II factor VIII procoagulant inhibitor similar to previously reported cases (Biggs et al, 1972b). In vitro ...

Three patients developed prominent neurologic symptoms and signs associated with Schoenlein-Henoch purpura. A 7 1/2-year-old boy was seen with status epilepticus after a 2-week history of generalized headaches, irritability, and intermittent colicky abdominal pain. A left hemiparesis and a left homonymous hemianopia with a right gaze preference that were present ...

Autoimmune thrombocytopenic purpura associated with Kaposi's sarcoma in a 42-year-old male from the south of Saudi Arabia is reported. This association has not previously been reported in the medical literature. The occurrence of Kaposi's sarcoma in renal transplant recipients and in those suffering from acquired immune deficiency syndrome has been ...

Acute thrombotic thrombocytopenic purpura (TTP) is a rare, usually fatal, disease characterized by widespread deposition of microvascular occlusive thrombi of platelets and fibrin. Although its exact etiology is unknown, numerous case reports in the medical literature have linked TTP with a variety of medical conditions, including systemic infections, vaccinations, pregnancy, ...

Buprenorphine is a powerful new analgesic agent with agonist and antagonist opiate receptor activity. Its withdrawal symptoms have been reported as being mild; however, its potential for abuse is not known. A case of buprenorphine abuse is reported, in which the patient's history and his response to naloxone suggest that ...

Aetiological factors predisposing to cerebral palsy were investigated in 190 children with this condition seen in Riyadh , Saudi Arabia since January 1980. Ninety-four (49.5%) of the cases were natal in origin, 63 (33.1%) prenatal and 33 (17.4%) postnatal. Anoxia was the most common aetiological factor, accounting for 38 cases ...

The hemolytic-uremic syndrome (HUS) following dysentery caused by S. dysenteriae Type 1, characterized by microangiopathic hemolytic anemia and acute renal insufficiency, is clinically similar but not identical to the idiopathic HUS. We studied renal necropsy specimens of nine children who died of HUS following shigellosis by light and immunofluorescent microscopy ...

Henoch-Schoenlein purpura is a haemorrhagic syndrome, rare in the adult but well known in paediatric pathology. The clinical manifestations are usually: arthralgia, skin rash, renal and gastrointestinal involvement. Although the small bowel is more frequently involved, cases of oesophageal, gastroduodenal and colo-rectal localization have been rarely reported. We describe endoscopic ...

100 cases of alopecia areata are examined and the possible relationship with the presumed etiological factors (atopy, autoimmunity and psychosomatics) are looked for. When atopy or autoantibodies are present alopecia areata always runs a serve course. It is, however, impossible to establish a definite etiological relation between atopy or autoimmunity ...

The monocyte function of 112 specimens from 42 children with idiopathic nephrotic syndrome (INS) aged from 2 to 17 years was studied by the methods of nitroblue tetrazolium (NBT) reduction, phagocytosis of immunobeads (IB) and yeast cells, chemotaxis and acid alpha-naphthyl acetate esterase (ANAE) staining. The dissociation between phagocytosis and ...

During the course of a review of our patients with bullous pemphigoid (BP), we found that one of our patients had a nephew with the disease. This report presents the cases and suggests that genetic factors may play a role in the pathogenesis of BP. To our knowledge, the occurrence ...

Immunofluorescence studies (direct and indirect) were done on 48 cases of pemphigus and 55 cases of bullous pemphigoid. All cases of pemphigus had in vivo and in vitro IgG intercellular antibodies except for two patients whose sera were not sent for pemphigus antibodies. 47 (85.4%) of our pemphigoid patients had ...

The origin of the two linguistically distinct recurrent utterance types--real word recurrent utterances (RWRUs) and non-meaningful recurrent utterances (NMRUs) - is discussed. It is argued that the origins of RWRUs may have more to do with post-onset factors than with activities being pursued at the time of onset, and the ...

The youngest case with spontaneously acquired inhibitor to factor VIII is reported. A 7-year-old girl without any previous disorders presented with a hemarthrosis in the left knee and developed a rapidly expanding hematoma on the right arm, shoulder and chest after an initial infusion of factor VIII concentrate (1,000 units). ...

A 5-year-old girl suffering from Henoch--Schonlein purpura developed severe abdominal pain accompanied by vomiting and fever. Concomitantly, the serum amylase level became elevated and leukocytosis developed, with a shift to the left. A diagnosis of pancreatitis complicating Henoch--Schonlein purpura was made. This rare complication is presented, along with a review ...

Attention has recently focused on the need to build up a corpus of case observations relating to the function of delayed echolalia in various handicapping conditions but especially in early childhood autism. This present article offers six functional categories as an aid to clear reporting of observation and, thus clarification ...

This paper describes a woman with pseudopseudohypoparathyroidism (PPHP) who had intractable lymphoedema and also abnormal dermatoglyphics. The findings were quite distinct from those in two previously reported cases of pseudohypoparathyroidism (PHP). Dermatoglyphics may prove useful in the diagnosis of PPHP but may also provide a clue to the factors which ...

Penicillamine-associated bullous eruptions share with spontaneously occurring pemphigus intraepidermal acantholysis, epidermal intercellular deposition of immunoglobulin, and circulating serum antibody against the intercellular regions of the epidermis. We report the case of a penicillamine-associated bullous eruption in which there were some of the histologic features of pemphigus, but none of the ...

Intracranial hemorrhage is a rare but life-threatening complication of childhood idiopathic thrombocytopenic purpura. We present three cases of this complication encountered at our institution, in addition to a tabulation of 15 previously reported cases. Prevention, diagnosis, and management of intracranial hemorrhage in idiopathic thrombocytopenic purpura are discussed. The importance of ...

We report a case of presumed autoimmune thrombocytopenic purpura in a patient with squamous cell carcinoma of the neck. A review of the literature on the association of autoimmune thrombocytopenic purpura and solid tumors is presented. Autoimmune-like thrombocytopenia should be considered when patients with head and neck carcinoma present with ...