ABSTRACT
OBJECTIVES: To derive objective values for the diagnosis of Hirschsprung disease (HSCR) from a comparison of the morphometric profile of large intestinal neuronal plexuses in normal perinatal autopsies and surgical specimens of HSCR. METHODS: A cross-sectional comparative study with 40 subjects each in (i) non-HSCR perinatal group encompassing neonates and stillborn babies beyond 30 weeks of gestation on whom autopsies were conducted and (ii) HSCR group comprising all patients clinicoradiologically diagnosed as HSCR. The morphometric assessment was done on hematoxylin-and-eosin-stained sections. KEY RESULTS: The morphometric profile in terms of average number of ganglia/linear mm of colon, interganglion distance, number of ganglion cells/ganglion, average ganglion cell length, ganglion cell nuclear area, ganglion cell nuclear diameter, nerve trunk thickness, and density has been outlined. On comparison with the neuroanatomically normal zone of HSCR, the cut-offs to identify hypertrophic nerve trunks (nerve trunk thickness of >37.85 µm) and reduced number of ganglia (number of ganglia/linear mm of colon <2.05 and interganglion distance of >229 µm) were derived. CONCLUSIONS & INFERENCES: The determined objective values, after testing on diagnostic rectal biopsies, may serve to formulate a diagnostic algorithm along with immunostaining for diagnosis of HSCR in colorectal specimens.
Subject(s)
Enteric Nervous System/pathology , Hirschsprung Disease/pathology , Intestine, Large/innervation , Intestine, Large/pathology , Neurons/pathology , Autopsy , Cross-Sectional Studies , Enteric Nervous System/cytology , Humans , Infant, Newborn , Intestine, Large/cytology , Prospective StudiesSubject(s)
Adenocarcinoma, Follicular/diagnosis , Cytodiagnosis , Diagnosis, Differential , Mandibular Neoplasms/diagnosis , Adenocarcinoma, Follicular/pathology , Aged , Biopsy, Fine-Needle , Female , Gingivitis/diagnosis , Gingivitis/pathology , Humans , Mandibular Neoplasms/pathology , Mandibular Neoplasms/secondaryABSTRACT
BACKGROUND: When a patient is steroid-dependant, a currently available strategy in chronic idiopathic thrombocytopenic purpura (ITP) is to follow a trial and error approach with any of the known drugs which has been found effective in the condition. OBJECTIVE: To evaluate the response of chronic ITP to dapsone, an inexpensive drug now reported to be effective in the disease. DESIGN: A controlled trial of abstinence and rechallenge type. SUBJECTS: Eight subjects with chronic ITP. INTERVENTIONS: Phase I - Intake of 100 mg of dapsone daily until response (in form of rise of platelet count in blood), Phase II - Above followed by drug abstinence, minimum for four weeks, and then rechallenge with the drug. MAIN OUTCOME MEASURES: Platelet counts during various phases viz during drug intake, withdrawal and rechallenge. RESULTS: Four (50%) patients responded to treatment. The mean pre-dapsone and post-dapsone platelet counts of blood were 29.6 x 10(9)/l and 142.5 x 10(9)/l respectively during the first phase of trial. The rechallenge was done in five patients following withdrawal of drug and the mean values of platelet count before and after rechallenge were 32.2 x 10(9)/l and 83 x 10(9)/l respectively. There was a remarkable response in two patients; one is now off the drug and the other on a maintenance dose of 50 mg of dapsone daily. CONCLUSION: Dapsone caused significant rise of platelet count in some patients of chronic ITP. It can be tried as an alternative to other second-line drugs in chronic ITP.
Subject(s)
Anti-Inflammatory Agents, Non-Steroidal/administration & dosage , Dapsone/administration & dosage , Purpura, Thrombocytopenic/drug therapy , Adolescent , Adult , Chronic Disease , Dose-Response Relationship, Drug , Drug Administration Schedule , Female , Follow-Up Studies , Humans , Inosine Triphosphate , Male , Purpura, Thrombocytopenic/diagnosis , Treatment OutcomeABSTRACT
Glanzmann's thrombasthenia is a well defined inherited disorder of platelet function characterized by qualitative and qualitative defect in cytoadhesive membrane protein, glycoprotein IIb-IIIa (the platelet fibrinogen receptor). From January 1990 to October, 1999, five patients who presented with mucocutaneous bleeding were detected to have Glanzmann's thrombasthenia. Clinical and laboratory spectrum of this rare disorder was studied which revealed heterogeneity of disease with respect to nature and severity of bleeding unpredictable by laboratory findings.
Subject(s)
Thrombasthenia/physiopathology , Adolescent , Blood Platelets/pathology , Child , Child, Preschool , Female , Hemorrhage , Hemostasis , Humans , Male , Platelet Aggregation , Platelet Glycoprotein GPIIb-IIIa Complex/metabolismABSTRACT
Ciprofloxacin, a broad-spectrum fluoroquinolone antibacterial agent, is generally considered to be a safe drug. However, occasionally it may have life-threatening complications. Two instances of bone marrow failure following use of ciprofloxacin are reported. In one case, the bone marrow reverted to normal following withdrawal of the drug. In the other case, the patient succumbed to irreversible bone marrow depression leading to severe thrombocytopenia and uncontrolled bleeding. This could have been an idiosyncratic reaction.
