Methemoglobin (MetHb) is an oxidation product of hemoglobin in which the sixth coordination position of ferric iron is bound to a water molecule or to a hydroxyl group. The most common cause of acquired MetHb-emia is accidental poisoning which usually is the result of ingestion of water containing nitrates or food containing nitrite, and sometimes the inhalation or ingestion of butyl or amyl nitrite used as an aphrodisiac. We herein report a case of MetHb-emia after ingestion of an aphrodisiac, later identified as dapsone by gas chromatograph/mass selective detector (GC/MSD). A 24-year old male was admitted due to cyanosis after ingestion of a drug purchased as an aphrodisiac. On arterial blood gas analysis, pH was 7.32, PaCO2 26.8 mmHg, PaO2 75.6 mmHg, and bicarbonate 13.9 mmol/L. Initial pulse oxymetry was 89%. With 3 liter of nasal oxygen supplement, oxygen saturation was increased to 90-92%, but cyanosis did not disappear. Despite continuous supplement of oxygen, cyanosis was not improved. On the fifth hospital day, MetHb was 24.9%. Methylene blue was administered (2 mg/kg intravenously) and the patient rapidly improved. We proved the composition of aphrodisiac as dapsone by the method of GC/MSD.
Administration, Oral ; Adult ; Antidotes/therapeutic use ; Aphrodisiacs/adverse effects* ; Case Report ; Cyanosis/drug therapy ; Cyanosis/chemically induced ; Cyanosis/blood ; Dapsone/adverse effects* ; Human ; Male ; Methemoglobinemia/drug therapy ; Methemoglobinemia/chemically induced* ; Methylene Blue/therapeutic use

Among the various complications of central venous catheterization, extravsscular migra tion of the catheter during fluid therapy is a rare condition to be encountered. We experienced a case in which an uneventful, successful insertion of subclavian catheter was followed at greater than 33 hours by massive mediastinal and bilateral pleural effusions, which resulted in hypotension, severe dyspnea, and cyanosis. The symptoms were re- lieved immediately after the bilateral thoracostomy and removal of the subclavian catheter. The chemical assay of the effusion was revealed glucoae-rich fluid given exogenously. The inferred cause was that postoperstive extravascular migration of the subclavian catheter probably resulted from both intensive respiratory physiotherspy and movement. We conclude that, although the successful placement of central line may be confirmed on insertion, a continuous reexamination of both function and location of the line is necessary to avoid the hazards of delayed diagnosis.
Catheterization, Central Venous ; Catheters* ; Central Venous Catheters ; Cyanosis ; Delayed Diagnosis ; Dyspnea ; Fluid Therapy* ; Hydrothorax ; Hypotension ; Pleural Effusion* ; Thoracostomy

Among the various complications of central venous catheterization, extravsscular migra tion of the catheter during fluid therapy is a rare condition to be encountered. We experienced a case in which an uneventful, successful insertion of subclavian catheter was followed at greater than 33 hours by massive mediastinal and bilateral pleural effusions, which resulted in hypotension, severe dyspnea, and cyanosis. The symptoms were re- lieved immediately after the bilateral thoracostomy and removal of the subclavian catheter. The chemical assay of the effusion was revealed glucoae-rich fluid given exogenously. The inferred cause was that postoperstive extravascular migration of the subclavian catheter probably resulted from both intensive respiratory physiotherspy and movement. We conclude that, although the successful placement of central line may be confirmed on insertion, a continuous reexamination of both function and location of the line is necessary to avoid the hazards of delayed diagnosis.
Catheterization, Central Venous ; Catheters* ; Central Venous Catheters ; Cyanosis ; Delayed Diagnosis ; Dyspnea ; Fluid Therapy* ; Hydrothorax ; Hypotension ; Pleural Effusion* ; Thoracostomy

Raynaud's phenomenon manifests as triphasic color change episodes of blanching, cyanosis, and reddening of the digits, induced by exposure to low temperature or emotional stress. It is a relatively common disorder, estimated to affect 5-10% of the general population and 20-30% of otherwise healthy women. Most cases of primary Raynaud's phenomenon also called Raynaud's disease, are mild and self-limited. Secondary Raynaud's phenomenon presents as a secondary manifestation of an underlying disease and are complicated by ulcerations and tissue necrosis. From March 1996 to August 1998, we experienced 4 patients with Raynaud's phenomenon. Two patients were diagnosed Raynaud's disease and the other two were secondary. Raynaud's disease responded to drug therapy and sympatetic ganglion block. Secondary Raynaud's syndrome was treated with vein graft and free tissue transfer. During postoperative follow-up of 33-49 months, both severity and symptomatic intervals were improved.
Cyanosis ; Drug Therapy ; Female ; Follow-Up Studies ; Ganglion Cysts ; Humans ; Necrosis ; Raynaud Disease ; Stress, Psychological ; Transplants ; Ulcer ; Veins

Pneumocystis carinii pneumonia is caused by Pneumocystis carinii. It usually occurs in premature or debilitated infants. Recently sporadic cases of human disease in patients who have been on long term steroid therapy, cytotoxic drug therapy, immunosuppressive drug were significantly increased. We recently experienced 35 cases of Pneumocystis carinii pneumonia in infants of an institution for foreign adoption in three epidemic period of Feb.1979, Mar. 1980, and Jan. 1980. The clinical review of 35 cases was made. Patients' age was between 1 to 4 months. Twenty-one cases (60%) occurred in 2-month-old infants. Many patients were included in poor weight gain and development. The common symptoms were tachypnea, cyanosis, restlessness, cough, diarrhea in order of frequency. The roentgenological findings were classified into three groups. normal finding, pulmonary emphysema only, and various forms of pneumonic infiltration. The roentgenological findings were somewhat characteristic. The most common finding (24 cases) showed streaky and mottled densities which began in both hill and were spreaded peripherally. The pneumonic infiltrations were spared peripheral lung, but progressed to total involvement. The prominence between alveolar and interstitial infiltration was almostly equal when patients were admitted. Nineteen cases (54%) showed pulmonary emphysema.
Cough ; Cyanosis ; Diarrhea ; Drug Therapy ; Humans ; Infant ; Lung ; Pneumocystis carinii ; Pneumonia ; Pneumonia, Pneumocystis ; Psychomotor Agitation ; Pulmonary Emphysema ; Tachypnea ; Weight Gain

OBJECTIVE: To evaluate the efficacy of volume therapy with sodium acetate Ringer solution during the perioperative period in children with cyanotic congenital heart disease (CHD). METHODS: The children who underwent elective surgery for cyanotic CHD admitted to Shanghai Children's Medical Center Affiliated to the Medical School of Shanghai Jiaotong University from September to December 2018 were divided into three groups according to random number table with the informed consent of their legal representatives. All of the children received volume therapy with infusion of sodium acetate Ringer solution intravenously upon anesthesia induction. The volume of infusion was calculated according to the "4-2-1" formula (group A, the rehydration volume was 4 mL×kg^-1×h^-1 for the first 10 kg body weight, 2 mL×kg^-1×h^-1 for the second 10 kg, and 1 mL×kg^-1×h^-1 for the third 10 kg and above), and the volume was increased by 50% or 100% in groups B and C, respectively. The intravenous infusion lasted for 30 minutes in all the three groups. Arterial blood gas analysis was performed before and 30 minutes after infusion to observe the acid-base status and electrolyte level. Pulse oxygen saturation (SpO₂), heart rate (HR), systolic blood pressure (SBP), diastolic blood pressure (DBP) before and 10, 20, 30 minutes after infusion, central venous pressure (CVP) at 30 minutes after infusion were recorded, as well as adverse events occurred after infusion. RESULTS: Twenty-six children with cyanotic CHD, 17 male and 9 female, aged from 1 to 36 months, body weight 3.6 to 16.0 kg, and America Society of Anesthesiologists (ASA) level of III or IV, were enrolled in the study. The pH value in group B at 30 minutes after infusion was significantly higher than that before infusion (7.35±0.05 vs. 7.32±0.06, P < 0.05), while no significant changes were found before and after infusion in the other two groups. The hematocrits (Hct) after infusion in three groups were significantly lower than those before infusion (0.433±0.141 vs. 0.473±0.146 in group A, 0.324±0.054 vs. 0.372±0.063 in group B, 0.363±0.097 vs. 0.418±0.111 in group C, all P < 0.01), indicating that all the children in the three groups achieved effective hemodilution. However, there was no significant difference in blood gas analysis before and after infusion among the three groups. The level of blood lactic acid (Lac) in all CHD children was decreased from (1.33±0.63) mmol/L to (0.98±0.36) mmol/L after infusion of sodium acetate Ringer solution, the serum Ca²⁺ concentration was decreased from (1.22±0.06) mmol/L to (1.19±0.06) mmol/L, and the serum Cl^- concentration was increased from (108.74±2.70) mmol/L to (109.77±2.54) mmol/L with the statistically significant differences (all P < 0.01). However, no significant difference was found in Lac or electrolyte levels before and after infusion among the three groups. There was no significant difference in vital signs before and after infusion among the three groups, but the period of infusion had an effect on SpO₂ (F = 5.998, P < 0.01), HR (F = 34.279, P < 0.01) and SBP (F = 4.345, P < 0.05). HR in groups A and C were significantly lower than those before infusion, and SBP in group A was decreased gradually with the prolongation of infusion time. The CVP value at 30 minutes after infusion in group B was higher than that in group A. No adverse reactions such as rash or anaphylactic shock occurred after infusion of sodium acetate Ringer solution in all children. CONCLUSIONS The perioperative volume therapy with sodium acetate Ringer solution in children with cyanotic CHD can effectively prevent the increase in Lac level and does not aggravate metabolic acidosis. The volume of infusion was well tolerated by all the children without disturbing the hemodynamic parameters.
Child, Preschool ; China ; Cyanosis/therapy* ; Female ; Fluid Therapy ; Heart Defects, Congenital/therapy* ; Humans ; Infant ; Infant, Newborn ; Infusions, Intravenous ; Male ; Perioperative Care ; Sodium Acetate/administration & dosage* ; Treatment Outcome

A full-term female infant was admitted at 5 hours after birth due to heart malformations found during the fetal period and cyanosis once after birth. Mmultiple malformations of eyes, face, limbs, and heart were noted. The whole-exome sequencing revealed a pathogenic heterozygous mutation, c.2428C>T(p.Arg810^*), in the BCOR gene. The infant was then diagnosed with oculo-facio-cardio-dental syndrome. He received assisted ventilation to improve oxygenation and nutritional support during hospitalization. Right ventricular double outlet correction was performed 1 month after birth. Ocular lesions were followed up and scheduled for elective surgery. The possibility of oculo-facio-cardio-dental syndrome should be considered for neonates with multiple malformations of eyes, face, and heart, and genetic testing should be performed as early as possible to confirm the diagnosis; meanwhile, active ophthalmic and cardiovascular symptomatic treatment should be given to improve the prognosis.
Female ; Humans ; Infant ; Infant, Newborn ; Male ; Abnormalities, Multiple/therapy* ; Cataract/genetics* ; Cyanosis ; Proto-Oncogene Proteins ; Repressor Proteins/genetics* ; Heart Defects, Congenital/genetics*

PURPOSE: Pompe disease (PD) is an autosomal recessive disorder caused by a deficiency of acid alpha-glucosidase resulting from pathogenic GAA variants. This study describes the clinical features, genotypes, changes before and after enzyme replacement therapy (ERT), and long-term outcomes in patients with infantile-onset PD (IOPD) and late-onset PD (LOPD) at a tertiary medical center. METHODS: The medical records of 5 Korean patients (2 male, 3 female patients) diagnosed with PD between 2002 and 2013 at Samsung Medical Center in Seoul, Republic of Korea were retrospectively reviewed for data, including clinical and genetic characteristics at diagnosis and clinical course after ERT. RESULTS: Common initial symptoms included hypotonia, cyanosis, and tachycardia in patients with IOPD and limb girdle weakness in patients with LOPD. Electrocardiography at diagnosis revealed hypertrophic cardiomyopathy in all patients with IOPD who showed a stable disease course during a median follow-up period of 10 years. Patients with LOPD showed improved hepatomegaly and liver transaminase level after ERT. CONCLUSION: As ERT is effective for treatment of PD, early identification of this disease is very important. Thus, patients with IOPD should be considered candidates for clinical trials of new drugs in the future.
alpha-Glucosidases ; Cardiomyopathy, Hypertrophic ; Child ; Cyanosis ; Diagnosis ; Electrocardiography ; Enzyme Replacement Therapy ; Extremities ; Female ; Follow-Up Studies ; Genotype ; Glycogen Storage Disease Type II ; Hepatomegaly ; Humans ; Liver ; Male ; Medical Records ; Muscle Hypotonia ; Republic of Korea ; Retrospective Studies ; Seoul ; Tachycardia

PURPOSE: Newborn infants with diarrhea, metabolic acidosis and dehydration may develop methemoglobinemia without exposure to oxidizing agents. This study was undertaken to investigate clinical features in the development of methemoglobinemia in newborn infants with diarrhea. METHOD: This study involved 16 newborn infants with diarrhea who were admitted to NICU at Dong San Medical Center between January 1995 and June 1996. We investigated the age of onset of methemoglobinemia, sex ratio, level of methemoglobin in the blood, feeding methods, clinical manifestations, arterial blood gas findings, the results of culture findings and the response to therapy. RESULT: 1) The age of onset was beyond the second week of life in most cases, no sex predilection was noted and formula feeding was used in all cases. 2) Methemoglobin level in the blood was 10.1-20.0% in 7 cases, 20.1-30.0% in 6 cases, 30.1-40.0% in 2 cases and above 40.1% in one case. 3) Clinical rnanifestations on admission: moderate to severe dehydration developed in all patients with diarrhea along with respiratory distress in 13 cases, cyanosis in 6 cases, fever was noted in 5 cases, vomiting in 5 cases, while 2 cases presented with abdominal distention. Combined diseases included metabolic acidosis in most cases (93.8%), hypokalemia in 6, failure to thrive in 3, chronic diarrhea in 2, hyponatremia in 2, necrotizing enterocolitis in 1 and hepatitis was found in one case. 4) In all cases, there were no pathogenic organisms found in either blood or urine cultures along with similar negative findings in stool cultures, and Rotazyme test results using ELISA method were negative. 5) Rehydration and correction of acidosis with sodium bicarbonate was accomplished in all patients and 11 cases with greater than 15% methemoglobin were treated with methylene blue, 2mg/kg as a 1% solution in normal saline. Response to methylene blue was indicated in 1 to 2 hours in all cases although there was a reoccurrence of methemoglobinemia after an initial response in 2 cases, they both responded favorably with retreatment with methylene blue. CONCLUSION: In all newborn infant with diarrhea, dehydration and metabolic acidosis, screening tests for early diagnosis of methemoglobinemia should be considered with prompt fluid replacement therapy.
Acidosis ; Age of Onset ; Cyanosis ; Dehydration ; Diarrhea* ; Early Diagnosis ; Enterocolitis, Necrotizing ; Enzyme-Linked Immunosorbent Assay ; Failure to Thrive ; Feeding Methods ; Fever ; Fluid Therapy ; Hepatitis ; Humans ; Hypokalemia ; Hyponatremia ; Infant* ; Infant, Newborn* ; Mass Screening ; Methemoglobin ; Methemoglobinemia* ; Methylene Blue ; Oxidants ; Retreatment ; Sex Ratio ; Sodium Bicarbonate ; Vomiting