Kawasaki disease manifesting with acute cholangitis. A case report.

A 3-year-old boy, who developed the signs and symptoms characteristic of Kawasaki disease, is described. The child also had an 8 cm tender hepatomegaly. Hydrops of the gallbladder could not be shown. Liver biopsy showed marked infiltration of inflammatory cells, including neutrophil and eosinophil leucocytes in the portal tracts involving the periphery of the portal arteries and veins, and acute inflammation of the bile ducts with neutrophil and eosinophil infiltration of the walls. Overt cholangitis has been described only once before in Kawasaki disease, when a viral agent was suggested as being important in the pathogenesis. Although the clinical and laboratory findings in cases of Kawasaki disease clearly suggest an acute infection--as they did in this case--no aetiological agent has yet been incriminated. The possibility of a drug-induced auto-allergic or hypersensitivity state is considered. Evidence for such a state includes a history of drug administration, pathological findings similar to peri-arteritis nodosa--a condition often associated with a hypersensitivity state--the presence of eosinophils in the lesions and a response to treatment with aspirin, a drug known to ameliorate hypersensitivity states.

Immunological disorders--the evolution of an hypothesis.

In honouring the memory of Dr Alwyn Zoutendyk, a respected member of the staff of the South African Institute for Medical Research, attention is called to the studies of the immunological disorders. While investigating serum hepatitis affecting soldiers of the US army following the administration of yellow fever vaccine, an antigen similar to that later called the Australia antigen, now hepatitis B surface antigen, was found in the acute phase serum and the corresponding antibody was found in convalescence. This finding and subsequent studies suggested there was a group of disease, which we called the hyperreactive auto-allergic disorders, of which examples were to be found in every system. The obverse of these we called the hyporeactive immunologically deficient disorders resulting from defects of the cell or serum components of the immunological reactions, of which many examples have also been found.

Severe tick-bite fever in young children. A report of 3 cases.

Tick-bite fever in young children is usually a mild illness with few complications and no mortality. That it may assume a severe form is illustrated by the occurrence of 3 cases admitted to Johannesburg Hospital within 1 week in which 2 patients, 3 and 5 years old respectively, had severe involvement of the central nervous system with epileptiform fits and deep coma and loss of power of speech on recovering consciousness. The third patient, aged 5 years, developed an incipient haemorrhagic state as well as serious involvement of the central nervous system. The severe form of the disease in these patients appears to have been due to unusually virulent strains of Rickettsia conorii, probably acquired from dog ticks in their homes. All patients recovered on appropriate treatment. Two regained their normal speech after many weeks while the third patient's incipient haemorrhagic state rapidly resolved.

Clinical aspects of African viral hemorrhagic fevers.

Three hemorrhagic fevers occur in southern Africa: Rift Valley fever, Marburg virus disease, and Crimean-Congo hemorrhagic fever. The patient's history of travel in Africa, visits to rural areas, contact with sick animals or their carcasses, or contact with a tick-infested environment or tick bites is important. Rift Valley fever is characterized by an incubation period of approximately 3 or 4 days, sudden onset of fever with a biphasic course, and signs and symptoms of liver and kidney disorder. The commonest complication is retinitis with a central scotoma. Severe cases may develop a hemorrhagic state, which may be fatal. Marburg virus disease was studied in two Australian students after a tour of Rhodesia (now Zimbabwe) and in a nurse who cared for them. The incubation period of approximately 7 days is followed by sudden onset of fever (typically lasting 7 days) and the appearance of a maculopapular petechial rash on the 5th day. A hemorrhagic state develops about the same time and may be fatal. Crimean-Congo hemorrhagic fever is widespread in South Africa; it may be transmitted by tick bite of the species Hyalomma, by contact with the tissues of animals, or by contact with infected patients.

The clinical manifestations of Rhodesian trypanosomiasis: an account of cases contracted in the Okavango swamps of Botswana.

Over 100 years ago, David Livingstone reported the presence of tsetse flies in the Okavango swamps in northern Botswana. They have persisted in the region and recently have been responsible for many cases of Rhodesian sleeping sickness caused by Trypanosoma rhodesiense in visitors to the area. The clinical manifestations in illustrative cases of this disease are described. One patient who refused treatment died five months after being infected. One patient died of encephalopathy complicating treatment with Melarsoprol (Mel B) and one died in a hemorrhagic state associated with a heavy parasitemia early in his illness. Most patients treated early respond well to treatment with specific drugs, usually Suramin, and are cured. In those with involvement of the central nervous system the treatment required is more hazardous, but usually is effective in curing the patient.

Psittacosis in the RSA.

Patients usually provisionally diagnosed as having typhoid fever or pneumonia are regularly admitted to the Rietfontein Fever Hospital suffering from psittacosis. The main symptoms are intense headache, chills and fever and an irritating non-productive cough. Later most patients develop signs of pneumonitis most clearly seen on radiographic examination. An important clue to the diagnosis is a history of contact with birds, most often budgerigars and more recently cockatiels. The diagnosis may be confirmed by the isolation of Chlamydia psittaci, the causative organism, but more usually reliance is placed on the results of serological tests revealing the development of chlamydial antibodies. None of the patients in this series developed serious complications, but if not treated psittacosis sufferers may develop severe pneumonitis, hepatitis and gastro-enteritis; the mortality rate is up to 20%. A rare but fatal complication is chlamydial endocarditis, presenting with the signs and symptoms of subacute bacterial endocarditis, but giving repeated negative blood cultures. The illness responds specifically to treatment with tetracycline antibiotics within 48 hours. Chlamydial infections are widespread among avian species. In the RSA most cases of psittacosis have resulted from contact with budgerigars and cockatiels, but outbreaks have been associated with imported batches of birds including South American parrots and Australian finches, emphasizing the need for vigilance at seaports.

Correlation of the distribution of Rickettsia conorii, microscopic lesions, and clinical features in South African tick bite fever.

Three South African patients with severe Rickettsia conorii infection had complicated courses of illness with 2 fatal cases and 1 with gangrene of multiple digits. Immunofluorescent organisms of R. conorii were demonstrated in vascular endothelium of brain, leptomeninges, renal glomerular arterioles and capillaries, renal arteries and veins, myocardial capillaries and arteries, pulmonary alveolar capillaries, pancreatic septa, splenic arterioles, and dermis. Rickettsiae were also observed in hepatic sinusoidal lining cells, splenic and lymph node macrophages, and the blood vessels of the partially viable zone of the amputated digits. Pathologic lesions included cerebral and cerebellar perivascular mononuclear leukocytes, mild mononuclear leptomeningitis, glomerular arteriolitis, vascular and perivascular mononuclear cell-rich inflammatory foci in the kidney, pancreas, skin, and myocardium, hepatocellular necrosis, and pulmonary edema. The sites of lesions and rickettsiae showed strong topographical correlation. Thrombi and hemorrhage occurred in a minority of the sites of vascular injury. Rickettsiae were the apparent direct cause of meningoencephalitis, peripheral gangrene, and other foci of vascular injury. Fatal R. conorii infection with disseminated organ involvement emphasizes the pathogenic potential of this disease.

Treatment of tick-bite fever with erythromycin.

Because of the occasional need for an alternative drug for the treatment of tick-bite fever, a study of the value of erythromycin for the variety of tick-borne typhus fever occurring in southern Africa was undertaken. In guinea-pigs erythromycin 125 mg/kg for 5 days largely prevented the fever and other signs of the infection, but did not prevent the serological reaction as determined by the rickettsial complement fixation test. A series of 17 human patients were treated for 4 days with erythromycin 500 mg 6-hourly for adults and 30-50 mg/kg/d in 4 divided doses for children. Eleven patients appeared to respond favourably. It was concluded that erythromycin does not have as specific a value as tetracycline in the treatment of tick-bite fever, but that when tetracycline is contraindicated erythromycin is a useful alternative drug.